¢ Tenderness is maximum at the free  edge of the nail. Pulp and distal parts  of fingers are relatively painless. It 1s  treated by “V” excision of a portion  of nail and opening of the abscess  cavity with antibiotic cover (Fig. 4.8).    These spaces are loose when compared to terminal pulp  space. They are filled with fibrofatty tissue.    ¢ Swelling is tender and indurated. Finger is held in the  position of flexion.  e Treated by transverse incision and drainage of the pus.    ¢ Web spaces are the triangular spaces in between the four  divisions of the palmar aponeurosis. They are 3 in number  (Fig. 4.10). Thumb has no palmar aponeurosis. They are  filled with subcutaneous fat and posteriorly covered by  metacarpal bones.    e Pain and swelling of palm in the region of web space.    Under anaesthesia, a transverse skin incision 1s made and the  pus 1s drained (Fig. 4.11). The cavity is treated like any other  abscess cavity. The skin edge is trimmed in such a way as to  leave a diamond-shaped opening to get better drainage.   ¢ Midpalmar space is the space behind the palmar aponeurosis  and in front of the metacarpal bones.   e Since palmar fascia is thick, strong and unyielding, pus  collects deep to palmar fascia. If it is due to penetrating  injuries, it collects in the subcutaneous plane like collar-  stud abscess. In the centre of palm, there is no subcutaneous  tissue. Hence, pus collects beneath the thick dermis.    ¢ Apex of the triangular palmar aponeurosis is continuous  with flexor retinaculum and palmaris longus tendon.    ¢ Distally, it forms 4 longitudinal digital bands and attaches  to bases of proximal phalanges.    ¢ Two fibrous septa extend from medial and lateral margins  of palmar aponeurosis. They are medial and lateral fibrous  septa. The septum is attached to 5th and 3rd metacarpals  respectively.   ¢ Deeper to flexor tendons, digital arteries and nerves lies  the midpalmar space.   ¢ The midpalmar space is continuous with anterior  compartment of the forearm via carpal tunnel. This space  is called ‘space of Parona’ (Fig. 4.13).    ¢ Obliteration of normal concavity of the palm  ¢ Gross oedema of the dorsum of the hand  ¢ Extreme tenderness in midpalmar space    ¢ Fingers are held in flexion at the metacarpophalangeal (MP)  joint because the palmar aponeurosis gets relaxed in this  position. MP joint movements are painful.    Thus, swollen palm, oedema of the dorsum of the hand,  flexed attitude of MP joint and separated fingers give  the picture of a frog hand.    ¢ Under anaesthesia, a transverse crease Incision 1s made and  once the palmar aponeurosis is seen, it is split /ongitudinally  in the direction of the fibres to avoid damage to nerves and  vessels,    'Henry Hamilton Bailey lost his left index finger because of acute  tenosynovitis.    e Similarly, there 1s tenderness over lateral side, over th:  flexor pollicis longus sheath when radial bursa is involvet  (Figs 4.15 and 4.16).    e Under anaesthesia, multiple incisions may have to be giver  to decompress the flexor tendon sheaths, so as to relieve  tension to drain the pus, exudate, etc.   e [nsevere cases a small plastic catheter should be introducec  into synovial bursa and it should exit by counter incisior  in the palm for antibiotic irrigation.   e These two are communicating in 80% of the cases. In 25%  of cases, the flexor tendon sheath of the thumb 1s  communicating with radial bursa and of the little finger  with ulnar bursa.   ¢ Thus, infection of the fingers (flexor tendon sheath) can  involve the entire hand.   ¢ The synovial sheaths of flexor tendons extend from the  bases of the terminal phalanges to the heads of the  metacarpal bones.   ¢ MP joint movements are not painful: This sign  differentiates suppurating tenosynovitis from deep palmar  abscess.   Fig. 4.16: Grossly swollen hand in a diabetic patient. It was looking  dangerous, but responded to conservative line of treatment   . Spread of infection to space of Parona. It is the space deep    to the flexor profundus and superficial to pronator quadratus  in the lower end of forearm. Patients present with swelling  of the forearm along with gross oedema of the hand.   In addition to the treatment mentioned above, a separate  incision may have to be given in the lower forearm for a  better drainage of the pus.   Cloxacillin 500 mg, 6th hourly for 7-10 days, with  metronidazole 400mg, 8th hourly for 7-10 days. Otherwise,  higher antibiotics such as cephalosporins may have to be  given.   Physiotherapy to decrease the stiffness of the fingers.  Tetanus prophylaxis in high-risk patients.    Position of function  The hand is held as if holding a cup or a glass    Extension at wrist: 25 degrees  Flexion at metacarpophalangeal joint 60 degrees  Flexion at interphalangeal joint 10 degrees    Flexion at distal interphalangeal joint 5 degrees  Thumb in alignment with forearm (Fig. 4.17)    It refers to chronic granulomatous lesions of the foot  involving skin and subcutaneous tissues. The disease not  only involves skin and subcutaneous tissue but also the  deeper structures such as bones resulting in osteomyelitis.  Gross thickening of subcutaneous tissue results in  convexity of the instep of the foot that is characteristic of  this condition (Fig. 4.18). Chronic suppuration, multiple  sinuses, sulphur granules in the discharge are characteristic  of mycetoma pedis (Fig. 4.19).   Bare foot walking which results in repeated minor trauma,  implants the organisms within subcutaneous tissue. It starts  as a pale, painless single nodule. Later multiple nodules  develop and rupture resulting in multiple sinuses.   Fig. 4.18: Flattening of the foot (Courtesy: Prof Madakatti, HOD  Surgery, Karnataka Institute of Medical Sciences, Hubli, Karnataka)    1. Bacterial mycetoma: It is due to Nocardia madurae or  due to actinomyces. These organisms are normally present  in the soil.    ¢ Sulphur granules in the discharge, plain radiograph of the  foot (Fig. 4.20).    1. Broad-spectrum antibiotics to treat secondary infection  along with dapsone 100 mg, twice daily is the choice.  Treatment may have to be continued for 1-2 years.   3. Amputation may be necessary, in refractory cases, to get  rid of a deformed, diseased limb.    ¢ [tis also described as embedded toe nail. Exact aetiology  is not clear. However, a few patients have family history  of this condition. Trimming the nail too much may result  in ingrowing toe nail.   Fig. 4.20: Plain radiograph of the foot showing extensive  rarefaction of the bones  ¢ As the nail grows inside, some degree of infection sets in.    resulting in development of granulation tissue which starts  pouting. The condition is painful, disturbing and unsightly.    ¢ Conservative: Dressing with antiseptic agents such as  iodine. Copper sulphate can be applied to treat extra  granulation tissue. Appropriate antibiotics are given.    ¢ Surgical: Under local anaesthesia, a portion of the involved  nail upto the base 1s removed followed by application of  phenol to the growing point of the nailbed at its base. It  takes about 10—15 days for complete healing.    ¢ Zadik’s or Fowler’s operation: The principle of this  radical procedure is to expose the lateral spike and germinal  matrix. This is achieved by incising the skin in lateral margin  and root of the nail.   e Surgical anatomy of palmar fascia and aponeurosis has  been added   Figs 4.21 and 4.22: Very severe dorsal space infection in a diabetic patient. Observe the constriction effect  caused by bangle. They need to be removed in all cases of hand infections   Fig. 4.25: Wet gangrene of the hand (Courtesy: Dr Maruthu  Pandyan, Government Medical Collage, Madurai)    (All the photographs in this chapter are contributed by Dr P Sreenivasa, Postgraduate student (2000-2003) and Prof. Mahimanjan  Singh, Head of the Department of Surgery, Govt Medical College, Mysore, Karnataka)   A. Chronic paronychia is caused by fungal infections  B. It is a deep infection   C. Osteomyelitis of distal phalanx will not occur due to  extensive collaterals   B. Space deep to flexor digitorum superficialis  C. Space deep to flexor profundus   C. Tetanus prophylaxis in high-risk patient  D. Pencillins are the drug of choice    Actinomycosis, leprosy, syphilis and AIDS are the chronic  diseases discussed in this chapter. Actinomycosis is a rare  disease, leprosy is of more interest to the skin specialists and  AIDS is an interesting topic to all clinicians. Hence, only  relevant aspects of each of these diseases as far as general  Surgeons are concerned are being discussed here.    Actinomycosis 1s caused by Actinomyces israelii, an anaerobic,  gram-positive branching filamentous organism (ray fungus).  Normally present in the oral cavity, tonsillar crypts and dental  cavities, they become pathogenic in the presence of trauma.  Three types of actinomycosis which are of some interest to  general surgeons have been discussed here.    e [tis common in patients with poor oral hygiene, bad caries  tooth, etc.   ¢ The organisms produce a subacute or chronic inflammation  for many months to years and produce lumpy jaw (Key  Box 5.1 for differential diagnosis).   ¢ Eventually cheek, mandible, jaws and salivary glands are  involved resulting in suppuration.    ¢ Extensive induration (marked induration) of lower jaw  (mandible) and gums gives consistency of bone.   ¢ The discharge contains sulphur granules which are gram-  positive mycelia surrounded by gram-negative clubs.   ¢ Over a period of years, it produces actinomycosis of lung  with involvement of pleura. Later it involves the chest wall,  resulting in multiple discharging sinuses.   ¢ There may be associated empyema and can easily spread  to liver.    ¢ It commonly occurs after surgery when there 1s mucosal  injury or discontinuity, e.g. after appendicectomy.    DIFFERENTIAL DIAGNOSIS  e Jaw tumour  e Osteomyelitis of jaw  ¢ Malignancy of oral cavity   ¢ The organisms which are normally present in the gut slowly  migrate into pericaecal tissue, then into the soft tissue,  subcutaneous tissue, and produce subacute or chronic low  grade inflammation.   ¢ Once the portal venous radical gets involved, spread to the  liver occurs.   ¢ 3-6 months later, fever and swelling in right iliac fossa  appears. Fever is probably due to pyaemia.   ¢ Onexamination, there is amass in the right iliac fossa which  is indurated, nodular and fixed.   ¢ Late stages produce multiple discharging sinuses,  sometimes faecal matter and sulphur granules. Unlike  tuberculosis, the lymph nodes are not enlarged.   ¢ Inj. crystalline penicillin 10 lakh units once a day for  6-12 months. Tetracycline and lincomycin are the other  alternatives.   e Sinuses in the jaw may have to be excised and osteomyelitis  has to be curetted out.   ¢ Actinomycosis of the right iliac fossa may need right  hemicolectomy.    LEPROSY (HANSEN'S DISEASE) _  ¢ Leprosy is caused by Mycobacterium leprae, an acid-  fast bacillus. Poverty, poor hygiene and population  (overcrowding) facilitate the spread of the disease.    e The disease 1s contracted in childhood or adolescence, but  it manifests after a latent period of 2—5 years.    ¢ Nasal secretions are the main source of infection but active  ulcers, sweat also contain lepra bacilli.   ¢ Leprosy predominantly affects skin, upper respiratory  tract (nasal cavity) and nerves. Thus, characteristic lesions  of leprosy include an anaesthetic patch of skin, thickened  nerves, a deformed leonine face and collapsed nose.    I. Tuberculoid leprosy: It occurs in patients with good  immunity with strong tissue response.   2. Lepromatous leprosy: It occurs in patients with poor  immunity with poor tissue response.   3. Borderline leprosy: It can be borderline lepromatous or  borderline tuberculoid leprosy depending upon the immune  response.    1. Lepromatous and borderline lepromatous leprosy  (Multibacillary disease)  e 3-drug regimen is the most ideal treatment.  Dapsone 100 mg/day,    Rifampicin 600 mg once monthly, period of 2  supervised. years. Skin  Clofazimine 300 mg once monthly, | Seat should be  supervised. negative.    2. Tuberculoid and borderline tuberculoid leprosy  (Paucibacillary disease)  ¢ Dapsone 100 mg daily.  ¢ Rifampicin 600 mg once a month,  supervised.   ¢ Face: It is involved in lepromatous leprosy and 1s described  as leonine facies with multiple nodules over the face,  pigmentation, /oss of lateral portion of the eyebrows  (madarosis), collapse of bridge of the nose due to destruction    Localised. Anaesthetic, hypopigmented, raised  skin patch. Early nerve damage, nerve thickening  is a characteristic feature. Involvement of face   Generalised. Erythematous multiple macular  rashes. Nerve involvement is usually not seen.  Leonine facies, collapse of the bridge of the nose  are characteristic.   Fig. 5.2: Autoamputation of the toes in a case of leprosy    of nasal cartilages (warm and moist area) and paralysis of  facial nerve.   Hands: Involvement of ulnar nerve at the elbow and median  nerve at wrist gives rise to ‘claw hand’.   Foot: Posterior tibial nerve is involved at the ankle leading  to clawing of the toes. Foot drop occurs when lateral  popliteal nerve below the knee joint is involved.    Leonine  facies,  collapse of  the bridge  of the nose  are a few  other  features of  lepromatous  leprosy   A 28-year-old lady complained of swelling in the posterior  triangle of 8 months duration. It was a bit irregular with  restricted mobility and it was tender. 3 candidates offered  nonspecific lymphadenitis as a diagnosis. Only one candidate  thought of nerve abscess. The clue was thickened nerve above.  It was a case of Hansen disease (Fig. 5.4).    A young man, 33 years of age, presented to the outpatient  department with inability to use his right hand for the last 6  months. He said he could not work, or hold objects.  Examination revealed atrophy of small muscles, specially  hypothenar muscles and interossei muscles. A 2.cm ‘skinulcer’  was also noticed. On careful questioning, he said he also had  loss of sensation. Further examination revealed a 4 cm x 3  cm oval swelling on the medial side of right arm. It was firm.  Some fluctuation could be elicited—   This is a case of pure neuritic leprosy with nerve abscess usually  caused by Mycobacterium leprae. |t is an uncommon manifestation.  No other peripheral nerve was involved. Differential diagnosis will  be ulnar nerve schwannoma.   e Presents as oval fluctuant swelling on the medial side of  arm   e Granuloma is common. Progression to abscess occurs in  tuberculoid leprosy.   e Schwann cell is affected. Slowly whole endoneurial zone  is occupied by endothelial cells.   e Treatment: Incision, drainage, excision of granulomatous  mass followed by treatment for leprosy.   3. Temporalis muscle flap to the upper eyelid to prevent  exposure keratitis.    ¢ Claw hand can be corrected using extensor carpi radialis  brevis muscle (Paul Brand’s procedure).   ¢ Foot drop can be corrected by using tibialis posterior  muscle tendon transfer (Ober’s and Barr’s procedure).    ¢ Because of involvement of the nerves, sensations are  impaired or lost. As a result of this, ulcers on the fingers,    a deep penetrating, perforating ulcer over sole of the foot  and even autoamputation of toes can occur.    Nonhealing ulcer over the sole of foot is corrected by applying  a POP (plaster of Paris) posterior slab. It takes off the pressure  and thus the ulcer heals. If calcaneus is involved due to  osteomyelitis, the bone has to be curetted out followed by  regular dressings.    e This is asexually transmitted disease caused by Treponema  pallidum. It is a delicate spiral organism (spirochaete).   ¢ Syphilis is infective only in its early stage. Early lesions  are predominantly situated in moist areas such as genitalia  and oral cavity.    1. Primary syphilis: Classically, a genital chancre occurs in  the penis or vulva after 3-4 weeks of sexual exposure.   e This chancre is shallow, indurated, painless ulcer called  Hunterian chancre. Associated inguinal nodes which are  shotty, multiple, nontender clinch the diagnosis (Fig. 5.7).   e Extragenital chancres can occur in the lips, tongue,  nipple, etc. They produce large enlargement of the  corresponding lymphnodes. Chancres in the rectum and  perianal region are common in homosexuals. They are  painful and resemble anal fissures.   ¢ Demonstration of Treponema pallidum in the clear  exudate from the lesion by dark field microscopy  confirms the diagnosis.    2. Secondary syphilis: It appears after 6-12 weeks of  spirochaetaemia.  ¢ Itis characterised by bilateral, symmetrical, coppery red  rashes which are generalised.  The rash is macular or papular, never vesicular. Papules  on moist sites such as vulva and perineum enlarge to  form condylomata lata—fleshy wart-like growths.  ¢ Small superficial ulcers in the mouth join to form snail  track ulcers.  ¢ Generalised lymphadenopathy involving epitrochlear and  occipital nodes can occur.  ¢ Moth-eaten alopecia, iritis, bone and joint pains.  3. Latent syphilis: If secondary syphilis is not treated, 1t will  develop into latent syphilis. There are no signs but serum  tests are positive.    It is also called tertiary syphilis. It basically affects vessels  causing inflammatory reactions and the end result is as follows:  ‘Endarteritis obliterans’— tissue necrosis — ulcers or  fibrosis.   This stage develops after 5—15 years of primary syphilis.  It causes neurosyphilis and cardiovascular syphilis. Lesser  form, a benign lesion is called gumma.Gumma is a syphilitic  hypersensitivity reaction consisting of granuloma with central  necrosis and sloughing.    ¢ Typically it 1s a subcutaneous swelling.  ¢ Affects midline of the body, e.g. posterior 1/3 tongue,  sternum, over the sternoclavicular joint.    ¢ Edges are punched out when the gumma ulcerates.  ¢ Floor contains wash leather slough.  ¢ On healing, it leaves a silvery, tissue paper scar (thin scar).    Gumma can also involve bone, testis and liver (ovary is  not involved).   1. Primary and secondary syphilis are treated by injection  procaine penicillin 10 lakh units IM x 14 days.   2. In late syphilis: Treatment is continued for a period of  21 days. With the current effective treatment of syphilis, it  is highly unusual to find late cases now.    ¢ Acquired Immunodeficiency Syndrome (AIDS) is the end  stage of a progressive state of immunodeficiency.  Causative organism: Human immunodeficiency virus (HIV).    Prophylactic measures to be adopted by surgeons  (healthcare workers) while treating AIDS patients  (universal precautions) (Key Box 5.4)   ¢ Gloves! must be worn when examining any patient with  open wound.    ¢ Reusable instruments such as endoscopes are cleaned with  soap and water and immersed tn glutaraldehyde.    ¢ No surgical procedure involving sharp instruments is  performed in the OPD.   e Operating table is covered with a single sheet of polythene.   e Staff with abrasions or lacerations on their hands are not  allowed inside the theatre.   ¢ Staff who enter the theatre must wear shoe covers, gloves,  disposable, water-resistant gowns and eye protection.    'Gloves were introduced by William Halsted to protect his nurse’s hands  from the harmful effects of carbolic acid (the nurse became his wife).    involved withthe operation (surgeon, assistant, scrub nurse)  (Figs 5.8 to 5.10).   . In the event of needlestick injury, allow the part to bleed  and wash the part thoroughly in tap water.   . AZT—zidovudine, lamivudine and indinavir should be  given to health workers following exposure of susceptible  areas to infected material from AIDS patients.    Anorectal disease is the most frequent reason for surgical  treatment in HIV-positive patients. This 1s common tn  homosexuals in whom AIDS 1s also common. They have  been grouped together as “A/DS anus syndrome”.  Anorectal disease can be classified into:   A 23-year-old-lady, carrying a 6-month-old child, presented  to us with severe dysphagia and odynophagia. Endoscopy  revealed extensive, unusual oesophageal ulcers which  prompted us to conduct HIV test. It was positive. We called  her husband and wanted to convey the message to him. Before  we conveyed, he said, ‘I am HIV-positive, my wife is HIV-  positive and even my child is HIV-positive!!!’   It is a type of colitis and produces abdominal pain, cramps,   ¢ Biliary tract infection by Cryptosporidium can cause  acute cholecystitis. AIDS-related sclerosing cholan-  gitis can occur resulting 1n right upper quadrant pain.   ¢ Abdominal lymphoma with involvement of liver and  spleen also causes abdominal pain.   ¢ Severe abdominal pain, may be due to perforation of  small or large bowel which should be treated as an  emergency.    Lymphomas and Kaposi's sarcoma, etc. They are due  to reduced cellular immunity due to following reasons:  1. Immunosuppression used in organ transplantation   5. Kaposi’s sarcoma can affect skin surface, gastro-  intestinal tract from mouth to anus, lungs, etc. It  presents as pigmented multifocal skin lesions.    abdominal and extranodal involvement including lung,  bone marrow and liver are common.    ¢ Risk of HIV transmission from the patient to a surgeon 1s  very low but it is dangerous. In US, by December 31, 2001,  only 6 cases of surgeons with HIV seroconversion from a  possible occupational exposure 1s documented by Centre  for Disease Control and Prevention (CDC) (out of a total  of 4,70,000 reported cases of HIV).   ¢ Routine use of barriers (gloves and goggles), washing  hands before and after examination of patient and careful  handling of sharps and needles will greatly reduce  transmission of the disease.    e Most of the diseases in this chapter are not of much  interest to us other than HIV. | have given a new Key  Box—FACE MASK. Detailed drug treatment of HIV is  in medical books.   C. Surgery may be required for abdominal actinomycosis  D. Ceftriaxone can be used for pulmonary actinomycosis   C. Indurated ulcer with large rubbery inguinal nodes  D. Indurated, single, painless ulcer   B. It is a gram-negative organism  C. Visible in dark field illumination  D. It does not have helical structure   C. It can be transmitted by blood transfusion and organ  transplant    Leg ulcers are one of the important topics in surgery. They  can occur in children, adults and the elderly. No age is spared,  no Sex 1s spared. Varying aetiological factors and presence of  complicated systemic diseases make the treatment of ulcers  very difficult. Chronic ulcers in old people definitely cause  considerable morbidity and diabetic ulcer of the leg can cause  life-threatening complications such as diabetic ketoacidosis  and septicaemia. Hence, it is necessary to do a careful clinical  examination of the ulcer to arrive at the diagnosis and plan  for appropriate treatment.   Anulcer is a discontinuity of the skin or mucous membrane  which occurs due to microscopic death of the tissues. Thus,  ulcer can occur anywhere in the body (skin), oral cavity, penis  (mucous membrane) or in the duodenum, intestine, etc. In  this chapter, lower limb ulcers will be discussed.    Ulcers can be classified based on the pathology or clinical  features.    1. Traumatic: Trauma can be mechanical. This is the most  common cause of leg ulcer. It can be physical trauma    due to burns or radiation. It can also be due to chemicals  such as acids.  . Venous ulcers: They include varicose ulcers and post-  thrombotic ulcers which can occur following deep vein  thrombosis.  . Arterial ulcers: Following are a few examples of arterial  ulcers:  ¢ Buerger’s disease—common  ¢ Atherosclerotic vascular disease—common  ¢ Vasospastic disorders such as Raynaud’s disease—  uncommon   ¢ Patients with rheumatoid arthritis can develop leg or  foot ulcers due to vasculitis.    e e e e e e e e e e e e e e e e e e e e e e e e e    1. Traumatic 2. Venous  3. Arterial 4. Neurogenic—trophic  5. Tropical 6. Diabetic    ¢ Paraplegia, meningomyelocoele, posterior tibial nerve  injury, tabes dorsalis are the other causes.  5. Tropical ulcer: It 1s a rare ulcer due to malnutrition  associated with infection caused by Vincent’s organisms.  6. Diabetic ulcer foot or diabetic ulcer leg  7. Blood dyscrasias: Sickle cell anaemia, thalassaemia,  leukaemia, etc. can produce recurrent ulcerations in the    There are due to specific type of organisms, e.g. tubercular  ulcers, syphilitic ulcers, actinomycotic ulcers.    Malignant ulcers are squamous cell carcinoma, basal cell  carcinoma, malignant melanoma. Malignant ulcers are  discussed in Chapter 11.   ¢ Arterial ulcer: Tip of the toes', dorsum of the foot.   ¢ Long saphenous varicosity with ulcer: Medial side of  the leg.   e Short saphenous varicosity with ulcer: Lateral side of  the leg just above the lateral malleolus.    2. Floor of the ulcer: This is the part of the ulcer which 1s  exposed or seen.  ¢ Red granulation tissue: Healing ulcer (Fig. 6.1)  ¢ Necrotic tissue, slough: Spreading ulcer (Fig. 6.2)  ¢ Pale, scanty granulation tissue: Tuberculous? ulcer.  ¢ Wash-leather slough: Gummatous ulcer  ¢ Part of the bone: Neuropathic ulcer  ¢ Nodular: Epithelioma  ¢ Black tissue: Malignant melanoma    Induration at the base, edge and surrounding area  Smell can be present    3. Discharge from the ulcer  e Serous discharge: Healing ulcer  ¢ Purulent discharge: Spreading ulcer  ¢ Bloody discharge: Malignant ulcer  ¢ Discharge with bony spicules: Osteomyelitis  ¢ Greenish discharge: Pseudomonas infection  4. Edge: This is between the floor of the ulcer and the margin.  The margin is the junction between the normal epithelium  and the ulcer. \trepresents the areas of maximum cellular   'When there is a block in the pipelines supplying water, distal houses suffer the maximum. Is it not?    activity. If destruction dominates as in spreading ulcers,  the edge is inflamed, oedematous and angry-looking (stage  of extension). When ulcer shows evidence of healing, the  edge will be bluish due to granulation tissue covered with  thin epithelium (stage of transition). In a healed ulcer, the  outermost part of the edge is whitish due to fibrosis (stage  of repair)—for types of edge (Fig. 6.3).   ¢ Sloping edge is seen in all healing ulcers such as    ¢ Punched out edge is seen in gummatous ulcers and  trophic ulcers. Gummatous ulcers have punched out  edge due to endarteritis obliterans caused by syphilitic  organisms. Chronic nonhealing ulcers may also have  punched out edges (Fig. 6.5).    ¢ Undermined edge is seen in tuberculous ulcers,  probably due to more destruction of subcutaneous tissues  than the skin. The edge !s classically thin and bluish in  colour (Fig. 6.6).   ¢ Raised edge (beaded edge) is seen in rodent ulcers or  basal cell carcinoma (Fig. 6.7).    ¢ Everted edge (rolled out) is diagnostic of squamous  cell carcinoma. The edge grows very rapidly and it  occupies the normal skin and thus gets averted (Fig. 6.8).    Fig. 6.7: Elevated edge—basal cell carcinoma (Courtesy: Prof  Vidyadhar Kinhal, HOD, Surgery, VIMS, Bellary, Karnataka)   5. Surrounding area  ¢ Thick and pigmented: Varicose ulcer.  ¢ Thin and dark: Arterial ulcer.  ¢ Red and oedematous: Spreading ulcers like diabetic  ulcer.  ¢ Scar around the ulcer: Marjolin’s ulcer.    1. Edge: Induration (hardness) of the edge is characteristic  of squamous cell carcinoma. Some degree of induration  can also be seen in chronic ulcers and long-standing  varicose ulcers. Induration occurs due to extensive fibrosis.  It is said to be a host defense mechanism. By causing  fibrosis, lymphatic spread is delayed. Tenderness of the  edge is characteristic of infected ulcers and arterial ulcers.    2. Base: /t is the area on which ulcer rests. Pick up the ulcer  between thumb and index finger and tissues beneath can  be appreciated. If the ulcer cannot be lifted up, the base  cannot be made out. The base can be tendons, muscles or  bone depending upon the site of ulcer. Marked induration  at the base is diagnostic of squamous cell carcinoma.  Hunterian chancre is a benign ulcer and produces  significant induration. Hence, itis also called a hard chancre  (Key Box 6.2).    3. Mobility: Gentle attempt is made to move the ulcer to know  its fixity to the underlying tissues. Malignant ulcers are  usually fixed, whereas benign ulcers are not.    4. Bleeding: Malignant ulcer is friable like a cauliflower. On  gentle palpation, it bleeds. Granulation tissue as in a  healing ulcer also bleeds’.    5. Surrounding area: Thickening and induration is found in  squamous cell carcinoma. Tenderness and pitting on  pressure indicates spreading inflammation surrounding the  ulcer.    1. Regional lymph nodes  ¢ Tender and enlarged: Acute secondary infection  ¢ Nontender and enlarged: Chronic infection   e The base and the surrounding area should be examined  for induration.    ¢ Nontender and hard: Squamous cell carcinoma  ¢ Nontender, large, firm, multiple: Malignant melanoma    2. Peripheral vessels: Detailed examination of periphera  vessels 1S discussed under peripheral vascular disease  However, dorsalis pedis, posterior tibial, popliteal an  femoral arteries should be palpated in cases of lower lim!  ulcers. Presence of weak pulses or absent pulses indicat:  peripheral vascular disease.    3. Sensations: Loss of vibration sense and loss of ankle jerk  occurs early in cases of diabetic neuropathy. Later, toucl  and pain are lost. Jotally anaesthetic feet are characteristic  of leprosy.   4. Function of the joint: Movements of the involved join  are restricted either due to pain, involvement of the joint o}  due to infiltration into the joint by malignant ulcers.    5. Varicose veins: If present, it is most probably a varicose  ulcer. However, A-V fistula can present as distal ulcers.  with arterialisation of veins and a continuous murmur.    I. Central nervous system (CNS) and spine in neuropathic  ulcers. There may be gibbus as in cases of TB spine or  operated scar due to myelomeningocoele, etc. See clinical  notes.   An 18-year-old girl with a nonhealing trophic ulcer was  examined by a postgraduate student. He gave a diagnosis of  trophic ulcer (neuropathic ulcer) due to leprosy as first  diagnosis followed by polyneuropathy. He failed. It was a  case of myelomeningocoele and the candidate had not  examined the spine! The patient had an operated  myelomeningocoele.    2. Splenomegaly in blood dyscrasias such as in early stages  of sickle cell anaemia.    ¢ Low Hb% is found in chronic ulcer. It 1s either nutritional  or due to frequent blood loss during dressings as in  diabetic ulcer.    'Granulation tissue is made up of capillaries and fibroblasts. Hence, it gives rise to fresh blood loss.   e Tenderness, local rise of temperature, bleeding on touch,  consistency of the ulcer, edge, surrounding area—  oedema, mobility.    . Urine and blood examination to rule out diabetes  . Chest X-ray: PA view to rule out pulmonary tuberculosis  . Pus for culture/sensitivity  . Doppler/duplex scan/lower limb angiography in cases  of arterial diseases  6. X-ray of the part to look for  ¢ Osteomyelitis—common in diabetic ulcers  ¢ Periostitis tibia—common in varicose ulcers  7. Biopsy: Nonhealing/malignant ulcers    It can be discussed under the following headings  1. Treatment of spreading ulcers    After obtaining pus culture/sensitivity report, appropriate  antibiotics are given. Many solutions are available to treat the  slough, such as hydrogen peroxide and Eusol!.   ¢ Hydrogen peroxide (diluted) when poured over the wound,  liberates nascent oxygen which bubbles out and helps in  separating the slough. Eusol also separates the slough.  There are reports that H,O, and Eusol can cause more  damage. Hence, they are no longer used.   ¢ Partially separated slough needs to be removed daily or on  alternate days, in the wards.   e Excessive granulation tissue or pouting granulation tissue  (proud flesh) needs to be decapitated by excision or by  application of copper sulphate or silver nitrate solution.   Thus, by repeated dressings, slough gets separated and  discharge becomes minimal, resulting in a healing ulcer  with healthy red granulation tissue. Management thereafter  is like that of a healing ulcer.    ¢ Regular dressings are done for a few days with antiseptic  creams such as liquid iodine, zinc oxide or silver  sulphadiazine preparation.  © A swab ts taken to rule out the presence of Streptococcus  haemolyticus which ts a contraindication for skin grafting.  ¢ Ifthe ulcer is small, it heals by itself with epithelialisation  from the cut edge of ulcer.  ¢ Ifthe ulcer is large, free split skin graft is applied as early  as possible (Key Box 6.4).  ADVANTAGES OF SPLIT SKIN GRAFT y  e Wound healing occurs fast  e Secondary infection is avoided because of early skin cover   e Infrared radiation, short-wave therapy, ultraviolet rays  decrease the size of the ulcer.   ¢ Chorion helps in formation of granulation tissue. These  ulcers ultimately may require skin grafting (Fig. 6.9).   Fig. 6.9: After epidermal growth factor for 4 days, wound became  very vascular    They can occur anywhere in the body. However, they are more  common where skin is closely applied to bony prominences,  e.g. shin, malleoli, over which there are no muscles. They are  usually single, very painful ulcers of healing type (Fig. 6.10).    Classes of wound dressings, debridement agents and skin replacements currently available    comments  1. Debriding agents H,O, releases nascent oxygen Destroys anaerobic bacteria; heat generated causes Both these are not favoured  * Hydrogen peroxide — which bubbles out and slough vasoconstriction and haemostasis, frothing brings today (Eusol is Edinburgh  comes to surface. debris to the surface University Solution)  ¢ Eusol Hypochlorite solution Mild debriding action  2. Polymeric films Plastic (polyurethane); Allows water vapour permeation; adhesive Opsite; Tegaderm  semipermeable  3. Hydrocolloid Hydrophilic colloidal particlesand Absorbs fluid; necrotic tissue autolysis; little Puoderm, Intrasite  dressings adhesive. It is impermeable to adherence; occlusive forms complex structures with  fluids and bacteria water and aids in atraumatic removal of the    dressing, hydrocol allows a high rate of evaporation  without compromising wound hydration    4. Alginates Polymer gel contains mannuronic Absorbs exudates, nonadherent, nonirritating, Ajgisorb, Sorbsan should  acid and glucuronic acid— _ requires a cover dressing (permeable) not be used in presence of  Seaweed polymer that forms a gel Along with silver', antimicrobial action against hepatic or renal impairment  when it absorbs fluid MRSA and pseudomonas.  5. Miscellaneous  * Gauze Woven cotton fibres Permeable with desiccation; debridement; painful  removal  ¢ Tulles  6. Medicated dressings Medications Increased epithelialisation by 25-30% Zinc oxide, Neomycin,  Bacitracin, Zinc  * Impregnated gauzes Fine mesh fabric (silicone, nylon) Nonadherent; semipermeable Biobrane II  with dermal porcine collagens)  7. Platelet-derived Stimulates growth of cells and angiogenesis; plemin, Regen-D  growth factor (PDGF) | Acts through tyrosine increases granulation tissue; stimulation of repair—  8. Endothelial-derived kinase receptor used when blood supply is good, classically  growth factor (EDGF) neuropathic nonhealing ulcers of diabetics Costly   ¢ Footballer’s ulcer is the name given to those nonhealing  ulcers which occur in the leg over the shin due to direct  trauma caused by the football. Sometimes, these ulcers may  take a long time to heal. If not treated properly, it gets  adhered to the bone.   * Located on the medial side of lower one-third of the leg in  cases of long saphenous varicosity and on the lateral aspect  of the leg in short saphenous varicosity.    They are very painful and occur in young patients who have  Buerger’s disease or occur in elderly patients due to  atherosclerotic vascular disease. It commonly occurs on the  tips of toes and fingers (Figs 6.12 and 6.13). The ulcer is dry,  deep and penetrates deep fascia. Evidence of chronic ischaemia  in the rest of the foot clinches the diagnosis (Table 6.4 for  differences between arterial and venous ulcers).    This type of ulcer develops in an anaesthetic limb. The causes   ¢ Ulcer develops on the pressure points such as beneath heel,  beneath the first and fifth metatarsals and gluteal region  (decubitus ulcer). It develops as a callosity, gets infected,  Suppurates and leaves a central hole discharging pus. Slowly,  it burrows deep inside, may involve bone and cause  osteomyelitis. Hence, it is also called perforating ulcer.   ¢ Trophic ulcers are caused by inadequate blood supply,  malnutrition and neurological deficit. They are also included  in this group.    ¢ Immobilisation of the foot in a plaster of Paris posterior  slab with a walking boot almost cures the ulcer within  2—3 weeks, provided the primary disease 1s also controlled,  e.g. leprosy. If the ulcer is nonhealing with slough, initial  management should include desloughing agents and  surgical removal of the slough.    The infection is caused by Vincent’s organisms like  bacteroides, B. fusiformis and Borrelia vincentii. It starts as a  pustule with extensive inflammation. The pustule bursts and  the ulcer spreads rapidly and causes destruction of surrounding  tissue. Hence, it is also called phagedenic ulcer’. The edges  are undermined, floor contains slough, and there 1s copious  seropurulent discharge. Healing 1s delayed for days to a month.  Metronidazole may be quite useful in bringing down the  inflammation. Broad-spectrum antibiotics may also be required  in cases of secondary infections. If healing takes place, it leaves  behind a scar.    It occurs due to deep vein thrombosis. It may affect calf veins  or it may be due to femoral vein thrombosis. It is an example  of venous ulcer or gravitational ulcer.    'Phagedenic (to eat). Rapidly spreading, ulcerative, destructive lesion.  It can occur in the oral cavity and also over the penis.   ¢ Due to increased hydrostatic venous pressure, the part 1s  significantly indurated (cyanotic induration), pigmented and  thickened with a rise in local temperature.   ¢ Homan’s sign: It is positive in calf vein thrombosis. It 1s  elicited by forcible dorsiflexion of the foot with the knee  extended causing pain in the region of calf.   ¢ Moses’s sign: Squeezing of the calf muscles from side to  side also produces pain.  These two signs are positive in acute cases.   With conservative treatment for a few days to a few weeks,  veins may get recanalised and the ulcer may heal. The  treatment can be very difficult (see Chapter 10 on varicose  veins).   ¢ Atherosclerosis is also a precipitating factor even though  all peripheral pulses are usually present.   e It occurs due to sudden obliteration of end-arterioles of  the skin on the back or outer side of calf region.   e Severe pain, ischaemic patch of skin which later develops  into a deep punched out nonhealing ulcer 1s a clinical  feature.    Students should not offer these ulcers as clinical diagnosis. They are  rare ulcers, with rare clinical interest.    These ulcers exclusively occur in young females and occur  in the lower third of leg and ankle region.   Usually seen in those patients who are obese with thick  ankles and abnormal amount of subcutaneous fat.   It begins with erythematous purplish nodules (hence the  name erythrocyanosis frigida) on the calves which later  rupture producing nonhealing ulcer.   Aetiology of these ulcers is not clear. It is supposed to be  due to ischaemia of lower leg due to spasm of branches of  posterior tibial and peroneal arteries. These vessels are  abnormally sensitive to hot and cold weather similar to  Raynaud’s disease. In some cases, tubercle bacilli have  been isolated, with ulcers responding to antituberculous  treatment.   These ulcers are managed conservatively.  Sympathectomy may be beneficial in those patients who  are hypersensitive to weather changes.    Diabetic patients are more prone for development of ulcers in  the foot because of the following reasons:  1. Neuropathy’: It commonly manifests after about 10 years    of diabetes. Neuropathy can be distal and diffuse with a  stocking type of distribution. Nerve damage is due to  formation of sorbitol from sugar. Sorbitol causes  demyelination of large fibres. New arteriovenous  communications open beneath skin, diverting nutrient flow  away from it. This ischaemic tissue is vulnerable for  infection causing nerve ischaemia. Loss of vibration sense  and deep tendon reflexes occur early. Later, joint position,  touch, pain and temperature sensations are lost. Asa result  of this, trophic ulcer develops. It progresses and can  penetrate deeper and deeper. Very often the patient is  unaware of this.  ¢ Diabetic neuropathy of the tibial nerve is dangerous.  Clawing of the toes and hammer toe result due to  paralysis of the intrinsic muscles of the foot. Sensation  is absent over the entire sole of the foot due to  involvement of medial and lateral plantar nerves. These  two factors predispose to the pressure sore over the  plantar surface of the head of the metatarsals.  ¢ Charcot’s arthropathy (joints) is a form of arthritis seen  In neuropathic foot and leads to severe defortnity.  ¢ Autonomic neuropathy leads to absence of sweating  and gives rise to anhydrotic skin (Key Box 6.6).    'Burning or tingling in plantar aspect of forefoot radiating to toes—  Morton’s interdigital neuroma.   DM of more than 10 years duration  Blood glucose levels not controlled  Peripheral neuropathy   DIABETIC NEUROPATHY Y  Loss of vibration and touch  Loss of pain and temperature  Dead like feeling in the feet  Sensation of walking on the ‘sand’  Susceptible for repeated trauma    Nylon monofilament test is routinely used to test neuro-  pathy by pressing in on the foot/sole    configuration of foot and change in gait. Foot becomes  shorter and wider. Longitudinal arch becomes flat. New  pressure points develop resulting in ulcers in the foot  (Key Boxes 6.6 and 6.7).   ¢ In ketoacidosis, granulocyte mobilisation is impaired  and chemotaxis 1s also reduced. Thus, they are suscep-  tible for polymicrobial and fungal infection.   Loss of muscle strength  Loss of curvature of foot  Loss of normal joint position  Loss of elasticity of skin    3. Atherosclerosis: Diabetic angiopathy involving major ves-  sels results in ischaemia of the foot (macroangiopathy).  They have accelerated atherosclerosis. In addition, it also  produces small vessel disease in the form of nonspecific  thickening of the basement membrane. It 1s described as  microangiopathy (Key Box 6.8).   Thus, neuropathy or microangiopathy singly or in  combination with secondary infection favours the development  of diabetic ulcer. Ulcer starts due to minor trauma such as  thorn prick, trimming of the nail or due to shoe bite. It may  also start as a callosity in the sole of the neuropathic foot.  Hence, utmost care should be taken to protect foot.   e Collateral circulation is reduced in diabetics  e Atherosclerotic plaque causes increased ulceration and  cracking    e More severe the diabetes, more severe will be peroneal  occlusive disease   1. Following an injury or due to infection, an ulcer develops  along with swelling and oedema of the leg—Stage of  cellulitis.   3. Secondary infection caused by mixed organisms along with  anaerobes and nonclostridial gas-forming organisms  produce multiple abscesses—Stage of abscesses.    Fig. 6.16: Various stages of infection of the leg in diabetic patients    1. Complete blood picture usually demonstrates high total  count with low Hb% (infection).   4. X-ray of the foot to rule out osteomyelitis which may be  the cause for chronicity of the ulcer (Fig. 6.17).   5. Liver function test (LFT), ECG, chest X-ray, blood urea,  serum creatinine as a routine in diabetic patients (remember  diabetes is a systemic disease).   6. Lower limb arterial duplex scan is an important  investigation to check the patency of vessels.  ¢ Turbulent blood flow (normal ts laminar flow wider  spectrum frequency and loss of diastolic forward flow ts  typically seen in diabetic foot ulcer.   7. Hand held Doppler and measurement of ankle brachial  index (ABI) is a bedside test.   Grade 2: Ulcer extension  a. Involves ligament, tendon, joint capsule or fascia  b. No abscess, no osteomyelitis   Various types of surgery for diabetic ulcer of the foot  Care’ of the patient as a whole    Control of diabetes: It is an important part of the treatment  of diabetic ulcer of the foot. Diabetes precipitates infection  which worsens the diabetic status. These ulcers are better  managed, at least in the initial period using insulin rather  than oral antidiabetic drugs. Inj. plain insulin is given 3-4  times/day depending upon the requirement. With availability  of glucometer and estimation of GRBS, sliding scale method  based on urine sugar estimation has become obsolete.    available, appropriate antibiotics are started. Commonly  gram-positive, gram-negative and anaerobic infection exist.  Triple antibiotics may have to be continued for a long time  depending upon the nature, type and severity of infection.  Presence of high grade fever with chills and rigors  suggests development of multiple abscess pockets that  need to be drained rather than indiscriminate change and  usage of antibiotics. \f infection 1s not controlled properly,  ketoacidosis can occur.    a nonhealing ulcer. Hence, initial treatment is debridement/  dressings or 1odine solution and when the ulcer 1s converted  into a healing ulcer, with pink granulation tissue, a split  skin graft is applied. Small ulcers heal by granulation tissue  (Fig. 6.18). Dakin’s solution is a chlorine releasing agent.  It 1s bactericidal and it loosens necrotic tissue. Collistin  solution contains 75 mg colistin in 50 ml normal saline,  can be used if Pseudomonas and anaerobic organism are  present.    Why do diabetic wounds / ulcers take  a long time to heal (Delayed healing)?    . Care of the patient as a whole: Recovery and healing of    diabetic ulcer of the foot may range from a few weeks to a  few months. During this period there are various other  aspects to be looked after apart from infection and insulin  (Table 6.6).    ¢ Ankle brachial pressure index is misleading because of  calcified vessels (incompressible).   ¢ Duplex ultrasound assesses both anatomical and  functional abnormality. If there is stenosis, peak systolic  velocity ratio will be greater than 2 across arterial lesion.   ¢ For short stenotic lesions, balloon angioplasty with or  without stent placement is the treatment.   ¢ Even after successful surgery, amputation rate is about  35%. 2 year patency rate is around 70%.   Multiple fasciotomy/decompression/debridement  Spreading cellulitis/necrotising fasciitis Amputation below knee or above knee.  Spreading cellulitis with gangrene Revascularisation, infrainguinal bypass    'Even a small diabetic ulcer should be treated properly. Otherwise a patient may have to ‘pay through his foot’ for it.  -This is an important aspect often forgotten by the treating physicians and surgeons.  Unfortunately in some patients, in an attempt to save the limb, all these surgeries will be done and at last, they may end up with amputation of the    Nutritional factors  A bedridden patient may have difficulty in passing urine, situation  worsened by pre-existing benign prostatic hypertrophy   Chest infection such as pulmonary tuberculosis or static pneumonia  Development of bedsores   More sensitive to carbohydrate metabolism, protein depletion and  changes in water and electrolyte metabolism   Catheterisation (Foley’s catheter) under aseptic measures, frequent  change of catheter and catheter care   Frequent checking of Hb%, total proteins, blood glucose and  electrolyte levels, etc.    7. Vaccum assisted closure (VAC) therapy (Figs 6.19 and  6.20): It is also called vacuum therapy, vacuum sealing or  topical negative pressure therapy. It 1s used for drainage  to remove blood or serous fluid from a wound or operation  Site.   ¢ Foam with an open-cell structure 1s introduced into the  wound and a drain 1s kept and brought out by lateral multiple  holes. The entire area is then covered with a transparent  adhesive membrane, which is firmly secured to the normal  healthy skin around the wound margin. The drain tube 1s  connected to a vacuum, fluid is drawn from the wound  through the foam into a bag (Fig. 6.19).   ¢ The plastic membrane prevents the inflow of air and  bacteria. It also allows a partial vacuum to form within the  wound, facilitates the removal of fluid.    ¢ Negative pressure assists with removal of interstitial fluid,  decreasing localised oedema and increasing blood flow.   ¢ Mechanical deformation of cells occur and it results in  protein and matrix molecule synthesis, which increases the  rate of cell proliferation.    ¢ Equipment: Microprocessor-controlled vacuum unit that  is capable of providing controlled levels of continuous or  intermittent sub-atmospheric pressure ranging from 25 to  200 mmHg.    ¢ Venous ulcers, diabetic ulcers, large ulcers following  necrotising fasciitis and any chronic ulcers.    ¢ Soft tissue injuries including sacral pressure ulcers, acute  traumatic soft tissue defects and infected soft tissue defects  following rigid stabilisation of lower limb fractures.    ¢ Treatment of burns and wounds in the perineum, hand or  axilla.    8. Limited access dressing (LAD): A simple plastic sheet  covers the wound. Intermittent suction 1s applied. It is  cheaper when compared to VAC (Fig. 6.20).    See Figs. 6.21 to 6.31 for various aspects of diabetic ulcer  foot.    Fig. 6.21: Cellulitis leg Fig. 6.22: Clawing of toes Fig.6.23: Wet gangrene with abscess foot    ¢ Never walk barefoot, preferably use microcellular rubber  shoes which are not only soft, but also allow oxygenation.   ¢ Keep the foot dry after proper cleaning of the foot.   ¢ Paring of the nails and trimming should be done carefully.  If infection sets in, consult the physician at the earliest.   ¢ Avoid applying herbal/local medicines or lotions to a corn.  Consult the surgeons for treatment of the corn.   ¢ Proper and regular control of diabetes by diet and frequent  measurement of blood sugar.   ¢ Since these patients have peripheral neuropathy, they will  not be able to appreciate temperature of water. Pouring  hot water over the foot can cause burns without the patient  being aware of it. Hence, a bystander has to check the  temperature of water before being used by the patient.  Thus, the best way to avoid complications related to   diabetes is by controlling the sugar levels with diet alone or    PUBLIC EDUCATION IN  DIABETIC PATIENTS  Barefoot walking should be avoided  Avoid herbal/local ointment application  Regular and Rigorous control of diabetes with diet  Exercises  Foot care—dry, frequent cleaning and corn care  Oxygenation to toes/foot, proper shoes (MCR)  O Do not consult Others  Trimming of nail should be done carefully   Pressure ulcers or bedsores are a serious and frustrating  complication for the paralysed, debilitated or comatose patient  confined to a bed or wheel chair. These ulcers form when soft  tissue is compressed between a bony prominence such as the  ischium, sacrum or trochanter and a supporting structure such  as the bed or wheel chair (Figs 6.32 and 6.33).    The growing incidence of spinal cord injuries due to  automobile accidents and increased numbers of debilitated  geriatric patients admitted to hospitals have drawn more  attention to the problem of pressure ulcer prevention and  treatment. Pressure ulcer 1s usually the most important factor  that delays rehabilitation of the paraplegic or quadriplegic  patient.    Most important factor is pressure, other factors being  paralysis, paresis, shearing forces, malnutrition, anaemia,  advanced age and infection. Lack of protective sensation in  comatose, debilitated patients, prevents them from changing  posture. The localised pressure continues and skin ulcer  develops. Initially there is tissue anoxia and cell death. Later  active inflammation and vasodilatation occurs resulting in  reactive hyperaemia. If pressure is removed allowing tissue    perfusion and thus wash out of toxic byproducts, initial damage  may be reversible. If not, permanent damage will occur. This  can happen in one to six hours.    ¢ Erythema, oedema and punctate haemorrhage. Moist  irregular ulceration with surrounding erythematous halo.    2. Late superficial ulceration  ¢ Full thickness skin ulceration  ¢ Spreading necrosis of subcutaneous tissue  ¢ Deep inflammatory response spreads in cone-shaped  fashion to deeper tissues.    ¢ Cicatrization of rolled ulcer edges  ¢ Eschar at base of ulcer  ¢ Spread of inflammation and bacterial invasion    There is no such thing as a small pressure ulcer. The  visible skin wound is merely the “tip of the iceberg”. 70% of  the ulcer is below the skin. Pressure is transmitted in a cone-  shaped or pyramidal manner from the skin through each layer  of tissue to the bony prominence, so that a cone of tissue  destruction is created. The point of the cone is at the skin  surface, and base 1s formed by the larger undermined defect  overlying the bone.    Pressure ulcers can be avoided by meticulous skin care and  relief of pressure over bony prominence (Key Box 6.11).   by secondary intention. It will take many weeks to heal.  2. Deep ulceration or large superficial ulceration   SKIN CARE  1. Regular, periodical skin inspection, especially over the  bony prominences.    3. Keeping the skin clean and dry: Moist areas lead to  maturation. Fine talcum powder may be applied to areas  where moisture tends to develop. It must be dusted every  day after drying the skin.   5. Care of perineum and genitalia, especially in patients  with incontinence.   6. Clothing and bedding must be wrinkle-free, made of porous  absorbent material to allow air circulation and avoid  accumulation of perspiration.    7. Pressure relief—in bedridden patients.  a. Frequent change of posture round the clock every  2 hours.  b. Avoid localised pressure by proper body alignment.  c. Use of air or fluid-filled floatation mattresses also  lessens risk of ulcer formation.  d. Patient and patient's family education.    3. Education of patient and patient attenders to prevent  pressure sores.    ¢ A maggot is the larva of a fly such as house fly. In practice,  any neglected wound on the body specially legs can get  contaminated with maggots. Within I—2 days, there can be  acolony of maggots (Fig. 6.34).   ¢ It is a type of biotherapy involving introduction of live,  disinfected maggots into nonhealing wounds.   ¢ The ugly look and feeling of a crawling creature on the  body drives the patient to reach the hospital in our country.  Maggots can be removed by using turpentine.   ¢ Maggot therapy may also reduce the need for antibiotics in  people with complex, chronic wounds.    ¢ Maggot therapy has been shown to accelerate debridement  of necrotic wounds.    ¢ Maggots inhibit and destroy a wide range of pathogenic  bacteria including methicillin-resistant Staphylococcus  aureus (MRSA), group A and B streptococci, and gram-  positive aerobic and anaerobic strains. Maggot therapy  therefore represents a cost-effective method for managing  MRSA infection.    ¢ Maggot therapy may also reduce the need for antibiotics 1  people with complex, chronic wounds.    Aldose reductase is the first enzyme in the sorbitol-aldosi  reductase pathway responsible for the reduction of glucose t¢  sorbitol, as well as the reduction of galactose to galactitol  Too much sorbitol trapped in retinal cells, the cells of the lens  and the Schwann cells that myelinate peripheral nerves cat  damage these cells, leading to retinopathy, cataracts anc  peripheral neuropathy, respectively. Aldose reductas«  inhibitors, are currently being investigated as a way to preven  or delay these complications.   e More details have been written about pathogenesis and  management of diabetic foot. Dozens of varieties of  wound dressings are available in the market in case of  nonhealing ulcers/wounds, a list of which has been  included.    e Povidone iodine is also toxic to fibroblasts. It increases  risk of wound infection.    Gummatous ulcer has following features except:  A.Commonly seen in the subcutaneous tissues    Characteristic feature of venous ulcer in the leg is:  A. Deep painful ulcer    Following are true for usage of platelet derived growth  factors in wound dressings except:   C. It is also an example of phagedenic ulcer  D. Metronidazole is helpful   A.It reduces the pain of intermittent claudication  B. It is a phosphodiesterase inhibitor    Lower limb ischaemia  Collateral circulation  Clinical features  Clinical examination  Management  Differential diagnosis  Acute arterial occlusion  Critical limb ischaemia    Walking is a fundamental human requirement. Peripheral arterial CAUSES OF LOWER LIMB ISCHAEMIA (Table 7.1)    disease (PAD) is a main cause of disability. Majority of the  individuals are conscious about chest pain caused by  myocardial ischaemia and arrive at a hospital early. However,  patients present relatively late to the hospital with lower limb  ischaemia (Figs 7.1 and 7.2). The disease, even though benign,  is not curable totally, thus causing financial, social and    Other rare causes of lower limb ischaemia include popliteal  entrapment syndrome and cystic medial degeneration. One  should not forget that diabetes mellitus is also one of the  common causes of peripheral vascular disease in elderly  patients. While treating a patient with atherosclerotic disease,  care of diabetes is equally important.   ¢ Other risk factors: This include hypercholesterolemia  (> 200 mg/dl), and hypertriglyceridaemia, hypercoagulable  state (polycythaemia) and hyperhomocysteinaemia.   ¢ Known diabetes: Specially type 2 diabetes mellitus—  increases the risk by two to four times.    It increases the risk of developing PAD by 7%  Homocysteine levels more than 15 utmol/I   Prevention is by eating foods containing B,, By and B,>  and with folate, such as potato, green vegetables, fish, etc.  It is also a strong risk for myocardial infarction in young  patients.    Collateral circulation is present in most of the organs.  Hence, even if a major vessel 1s occluded, the organ can  still survive provided collaterals are well-developed.  Chronic ischaemia caused by TAO or atherosclerosis,  allows sufficient time for collaterals to develop. Hence,  necrosis or gangrene that occurs 1s minimised. Thus, limbs  often survive (Figs 7.3 and 7.4).   In acute ischaemia caused by thrombus or embolism,  there is no time for collaterals to develop. This results in  gangrene of the limb, in untreated cases.    Pain in the limb is the chief symptom of lower limb  ischaemia. It is a severe cramp-like pain, due to ischaemia  of the muscles brought on mainly by exertion. It is called  intermittent claudication (Key Box 7.1).   Rest pain’: |t is an intractable type of pain usually felt in  the foot (instep), toes, etc. It is an indication of severe    GRADES OF INTERMITTENT  CLAUDICATION—BOYD’S CLASSIFICATION  Gradel: The patient walks for a distance, gets the pain,  continues to walk and the pain disappears. As a  result of ischaemia, anaerobic metabolism takes  place, which produces substance P, lactic acid,  etc. These produce vasodilatation and the pain    Grade Il: The patient walks for a distance, gets the pain and    Grade Ill: The patient walks and gets the pain. He has to take  rest. This grade indicates severe muscle ischaemia.    In late stages: Pain at rest is due to ischaemia of nerves” in  addition to ischaemia of the muscles.    *Cry of the dying nerves, due to involvement of vasa nervosum    Pain is due to ischaemic neuropathy involving small unmyelinated A  delta and C sensory fibres.    ischaemia of the foot with impending gangrene. Typically,  a patient with rest pain sits on the bed, holds his foot with  both hands or may hang the foot out of bed. This gives  him some kind of relief. Rest pain is worse at night time’.  It may lead to suicidal tendency.    ¢ Claudication distance refers to the distance a patient is  able to walk before the onset of pain. A patient with severe  claudication may not be able to walk even a few yards.    e Site of claudication depends upon the level of arterial  occlusion (Table 7.2).    ¢ Also see Table 7.3, for other causes of pain in the  leg.    2. Nonhealing ulcer is the next common presenting  symptom. It 1s usually precipitated by a minor trauma  and it occurs in the most distal part of the body such as  the tip of toes. Ischaemic ulcers are deep and very pain-  ful.    'One of our TAO patients with sleepless nights due to rest pain committed suicide by jumping from the 2nd floor of the hospital.   KEY BCX 7.2  Intermittent claudication in a young patient can be due >  to some rare causes such as SUPERFICIAL FEMORAL ARTERY    ¢ Popliteal artery entrapment due to abnormal origin of STENOSIS OR OCCLUSION  gastrocnemius muscle e It is the most common cause of intermittent claudication  © Cyst in the media of the popliteal artery e Usually calf muscles are affected    profunda femoris is involved.  3. Some patients present with gangrenous patches of skin    or subcutaneous tissue. Gangrene affects distal parts such  as toes. However, gangrene is minimal because of  collaterals (Fig. 7.5).   4. History of bilateral gluteal claudication with impotence  can occur In a young patient due to a saddle thrombus at 5  the bifurcation of aorta. It is called Leriche’s syndrome.    Impotence is due to failure to achieve an erection due to . .  paralysis of L1 nerve. 6. Majority of patients with peripheral vascular disease are   ¢ Gluteal claudication is confused for sciatica and many smokers. TAO occurs exclusively in male smokers’.  patients are referred to orthopaedic department. Sciatica  causes neurogenic claudication which is present even    e Single stenosis less than 3 cm is treated by Percutaneous  Transluminal Angiography (PTA)    at rest and is aggravated on movements of the spine. Cauda equina claudication or pseudoclaudication is due  Causes of neurogenic claudication are slipped disc, to compression of cauda equina.  fracture vertebrae, tuberculosis of spine, etc.    ¢ Rutherford also categorised chronic lower limb ischaemia  into 6 different categories starting from asymptomatic case  to a major tissue loss.    ¢ Claudication has been classified as mild, moderate and  severe depending upon treadmill response and ankle  pressure (AP)    '4 26-year-old female, nonsmoker patient presented to the hospital with ischaemic features of the right upper limb. All causes of upper limb  ischaemia were ruled out (Raynaud’s, cervical rib, etc.). On careful questioning, she admitted to using SNUFF DIPPING for 10 years (snuff  contains nicotine).    The findings are appreciated better if a comparison 1s made  with the opposite limb. Evidence of chronic ischaemia of the    ¢ Attitude of the limb: Very often, the patient holds the calf    muscles or dorsum of foot.  ¢ Flattening of the terminal pulp spaces of toes  ¢ Fissures, cracks in between the toes  ¢ Ulceration of toes, interdigital ulcers  ¢ Brittle, flat and ridged nails, shiny skin  ¢ Loss of hair and subcutaneous fat    ¢ The limb may appear more dark 1n dark-skinned patients  or markedly pale in fair-skinned patients with vasospastic    1. Ulcer: Examination should be done as described in  Chapter 6. Ischaemic ulcers are very tender.    2. Gangrene: It is described according to its size, shape and  extent. In dry gangrene, the part is dry and mummified or  shrunken. Features of dry gangrene are summarised in Key  Box 7.3.    ¢ Gangrene is usually dry with a clear line of demarcation.    [t indicates the junction of dead and living tissue. Since the  blood supply to the muscle is better, usually the line of  demarcation involves skin and subcutaneous tissue. Line  of demarcation is very well appreciated in senile gangrene  where it can be skin, muscle or bone-deep.    cardiac output at rest, reactive dilatation of skin vessels to  warmth and increased attention of the patient to the leg.    'It is better to palpate the entire limb from the thigh downwards.  “Ischaemic limb is like irritable personalities.    ¢ Ischaemic limb is cold: Careful palpation from above  downwards will reveal the change in temperature from  warm to the cold area. Temperature changes are  appreciated better with the dorsum of the hand which  is more sensitive as it has a lot of cutaneous nerve  endings.   ¢ Tenderness: It is tender due to the presence of  inflammation.    ¢ Pitting oedema can be due to thrombophlebitis or due  to nonfunctioning of limb.    ¢ Feel pulses in all four limbs and head and neck.    ¢ In a similar manner, upper limb pulses, head and neck    Disappearing pulse: When collateral circulation is very good  peripheral pulses may be normal. However, when the patien  is asked to exercise, the pulse may disappear. Exercise  produces vasodilatation below the obstruction and arteria  inflow cannot keep pace with increasing vascular space  pressure falls and the pulse disappears.    1. Buerger’s postural testis relevant in fair-skinned patients.  The patient (supine) is asked to raise his legs vertically  upwards keeping the knees straight. In cases of chronic  ischaemia, marked pallor develops within 2—3 minutes.  The angle at which pallor develops is Buerger’s angle of  circulatory insufficiency. In ischaemic limb, pallor    2. Capillary refill test: Apply pressure over the tip of the  terminal pulp space for a few seconds and release the    3. Popliteal artery, a continuation of  femoral artery, extends from the hiatus  in adductor magnus to the fibrous arch  in soleus. It is about 20 cm long    1. Subclavian artery arises from the arch  of aorta on the left side and  brachiocephalic on the right side    2. Common carotid artery arises from  arch of aorta on the left side and from  brachiocephalic artery on the right side    3. Superficial temporal artery is the  terminal branch of the external carotid  artery    At the level of ankle joint lateral to extensor  hallucis longus. It should not be felt distally  where it dips into the plantar space    In between the medial malleolus and medial bor-  der of the tendoachilles    [t is felt in the prone or supine position with knee  flexed. It is felt against lower end of femur or  against tibial condyles    It is felt midway between anterior superior iliac  spine and pubic tubercle, just below the inguinal  ligament in the upper thigh    It is felt in the supraclavicular region in the pos-  terior triangle against the first rib    It is felt against the carotid tubercle of sixth cer-  vical vertebra (C6) in the carotid triangle (at the  upper border of the thyroid cartilage)    It is felt in front of tragus of the ear against the  zygoma    For circulation of the foot, any one of these  arteries is sufficient    Dorsalis pedis, posterior tibial and popliteal  artery are usually not palpable in TAO patients    Carotid artery bifurcates at the upper border of  the lamina of thyroid cartilage (C3 vertebra)    This ts involved in temporal arteritis, a type of  giant cell arteritis    'A thorough clinical examination of pulses includes not only lower limb vessels but also head, neck and upper limb vessels.    ¢ The test can also be done in the ischaemic foot by asking  the patient to sit up and hang his legs down and observe  for colour changes. The time taken for the ischaemic foot  to become pink is described as capillary filling time. This  is prolonged in an ischaemic foot.    |. Systolic bruit over the femoral artery can be heard in  atherosclerotic occlusion of iliofemoral segment, due to  turbulence created by the blood flow.    2. Auscultation of the heart to rule out mitral stenosis (mid-  diastolic murmur, loud |st heart sound).   Fig. 7.9: Auscultation over the  femoral artery to look for bruit    Fig. 7.8: Auscultation of the  heart to rule out valvular heart  diseases   An eighteen-year-old girl was kept in the MBBS examination  with ischaemia of lower limb of 6 days duration. The  candidate offered collagen vascular disorder as the first  diagnosis and failed.   He had missed the cardiac history and findings totally by  not auscultating the heart. It was a case of mitral stenosis  with acute embolic gangrene of the lower limb.    ¢ Even though there are many causes of lower limb ischaemia,  thromboangitis obliterans (TAO) and atherosclerotic  vascular disease are the commonest causes. Hence, they  have to be considered first before giving other diagnosis.   ¢ Atherosclerotic vascular disease is the commonest cause  of lower limb ischaemia. It can manifest as simple ulcer to  massive gangrene.    CHECKLIST OF FEATURES OF CHRONIC  LOWER LIMB ARTERIAL OCCLUSION  e Cold and numbness  e Limb elevation: Slow venous refilling. Line of demarcation  present  e Altered/diminished sensation  e Ulcerations  e Dead toe—gangrene—Dried and mummified  e Intractable pain—rest pain  e Cracks, fissure—interdigital  e Arterial pulsations decreased or absent  e Thrill/oruit may be present  e Intermittent claudication  e Oedema—lf thrombophlebitis or cellulitis occurs  e Narrow calf muscle girth—muscle atrophy   e Occlusion of small- and medium-sized vessels,  superficial thrombophlebitis and Raynaud’s pheno-  menon constitute the ‘triad’ of TAO.   e Distal, infrapopliteal, segmental occlusion with skip lesions  and corkscrew collaterals in angiogram.   Key words can be remembered as PRISON  Progressive, Inflammatory, Segmental, Occlusive, Nonathero-  sclerotic    1. Complete blood picture: Anaemia definitely delays wound  healing and it also decreases tissue perfusion. High total  count indicates secondary infection.  ¢ Elevated platelet count suggests risk of thrombosis.   . Excessive smoking produces increased levels of  carboxyhaemoglobin which damages these vessels.    Atherosclerosis  Around 50 years and above  Females are also affected  1. Atherosclerosis is a rich man’s disease, who is  usually a smoker, diabetic and hypertensive.  2. Strong family history is also present in a  few cases.  3. Consumption of high fat diet leading to obesity,    Diffuse inflammatory reaction involving all three coats  of vessel (panarteritis) causing a thrombus, resulting  in occlusion of lumen (obliterans). Polymorphs, giant  cells and micro-abscesses are found within the  thrombus. In severe cases, vein and nerve are bound    Small- and medium-sized vessels such as dorsalis  pedis, posterior tibial, popliteal are commonly involved.    Deposition of lipid-rich atheromatous plaque in the  intima is the hallmark of atherosclerosis. Plaques  tend to be more in lower abdominal aorta, coronary  arteries, etc. Plaques may undergo calcification,  ulceration and thrombosis, dislodge cholesterol  emboli or may weaken the media and produce  aneurysm.    Medium-sized and large vessels such as aorta,  common iliac, femoral, common carotid, arteries are    involved.  6. Upper limb involvement Not uncommon Rare  7. Nature of vessel wall Not thickened Thickened  8. Blood pressure Normal in the normal limb and low in diseased limb. | Hypertension is commonly present.  9. Superficial? migrating Seen in about 30% of cases of TAO. Veins of lower Not seen  thrombophlebitis limb are involved and are tender and thickened.  10. Raynaud’s phenomenon Can be present Not seen  11. Auscultation— Bruit is not heard. Bruit can be present as in aortoiliac disease.  femoral artery  12. Angiography Cork-screw pattern of vessels Shows site of block   ¢ Fasting blood glucose or and glycosylated haemoglobin  HbAjc 1s important test as it reflects the duration of  diabetes.    Protein C deficiency is identified as a risk factor for arterial  thrombosis especially in patients who will be treated with  heparin.   Antiphospholipid antibody (APLA) syndrome (APLS) 1s  also called Hughes syndrome. It is an autoimmune  hypercoagulable state resulting in thrombosis of veins (deep  vein thrombosis), thrombosis of artery (stroke) and  pregnancy related complications. Treated by aspirin and  heparin.    . Homocysteine levels more than 15 pmol/|  . Doppler ultrasound blood flow detector:    This test is based on Doppler principle. An ultrasound signal  is beamed at an artery and the reflected beam is picked up  by areceiver. Frequency changes of the beam due to moving    'From MBBS examination point of view, TAO and atherosclerotic vascular disease are to be differentiated.  “Migrating thrombophlebitis is also seen in pancreatic malignancy where it is called Trousseau’s sign.   Fig. 7.11: Handheld Doppler checking the dorsalis pedis artery Fig. 7.13: Colour Doppler showing atherosclerotic narrowing    blood are converted into audio signals which can be heard | |  by using a probe. Thus, Doppler probe can be used to detect |  the pulse even when the pulse is clinically not palpable  (Figs 7.10 to 7.13).    ¢ To measure BP (blood pressure) in ischaemic limb.  ¢ Remeasuring BP in lower limb after exercise to    a combination of Doppler with B mode ultrasound (Fig.  7.14). With the availability of colour Duplex, the  direction of blood flow can be assessed. Red colour  means direction of flow towards transducer and blue  means away (Key Box 7.8).    DUPLEX IMAGING SCAN yy  It yields both anatomic and blood flow information  No nephrotoxic contrast agent is used  It gives a triphasic wave pattern systolic, diastolic and  elastic recoil  Elastic recoil is absent in calcified arteries  Thus biphasic and monophasic wave patterns indicate ‘PAD’  Overall sensitivity of 92% and specificity of 99% in occlusive  cases  Limiting factors are extensive ulcers, calcification and  oedema of leg    ¢ It is not usually indicated in TAO patients where direct  arterial surgery is not done. However, a few cases of    presenile atherosclerosis who are misdiagnosed as TAO  will require angiography to locate the site of obstruction  which is suitable for an arterial reconstruction.  Angiography is indicated in patients with atherosclerotic    vascular disease, to know the exact site of block, type of  obstruction, to define the collaterals, so as to plan for    Small vessel bypass is becoming popular today as in  selected cases of diabetes and atherosclerosis.    Fig. 7.15: Angiography showing block in the common iliac arter  on the right side (conventional)    Fig. 7.17: Distal formation at common femoral artery  (Courtesy: Dr Chandrakanth Shetty, Prof of Radiology and  Imaging, KMC, Manipal)   ¢ This is done in unilateral obstruction. An incision is made  in the upper thigh to expose the femoral artery on the  normal! side. A Seldinger needle and guide wire are used  to introduce arterial catheter and radiopaque dye is  introduced after placing the catheter into the aorta. It  visualises the entire aortoiliac segment and below.   ¢ [tis indicated when obstruction is bilateral, both femoral  pulses are not palpable, clinically manifesting as bilateral  lower limb ischaemia. Aorta is directly punctured from  behind (translumbar) by using ultrasound image  intensifier.  Results: Arteriography establishes the site of block and  nature of collaterals.   ¢ Haemorrhage from the puncture site which needs to be  stopped by pressure packing.   ¢ Infection: Digital subtraction angiography (DSA)—  This can be done by arterial or venous route. The arterial  route is preferred.  DSA gives excellent pictures in carotid and large  central vessels. However, the peripheral vessels may not  reveal adequate information. Thus, conventional  arteriography still hasan important role in atherosclerotic  vascular disease.   8. Magnetic Resonance Angiography (MRA) (Figs 7.16 and  Tl)  e It is more popular than arteriography because no arterial  puncture and no contrast induced nephropathy.   9. CT Angiography: It is good for above knee vessels  compared to below knee (Figs 7.18 and 7.19).   10. Carbon dioxide angiography with gas infusion also has  been found useful in all sized vessels and 1s well tolerated.    ¢ Femoral pulse is palpable. Hence, easy to locate the artery and introduce the catheter.  ¢ Ifathrombus occurs in the diseased limb due to angiography, it will worsen the ischaemia, may lead to gangrene.  Because of these complications, angiography should be done carefully only when it is indicated.    Fig. 7.20: Extensive narrowing of popliteal artery and branches  due to atherosclerosis    e [nall patients with peripheral vascular disease, following  general measures must be taken which will help in better  perfusion of the lower limb tissues.   * Anaemia must be treated with haematinics and if necessary,  blood transfusion. If ejection fraction is low, drugs are  given to improve cardiac output.    As already discussed, the pain is very severe and distressing.  Some amount of pain relief can be obtained as follows:    I. Analgesics: Simple analgesics may not help these patients.  ¢ Tramadol (50 mg) one tablet, 3 times a day can be used.  ¢ Ketorolac 10-20 mg one tablet, 3 times a day can be    Decrease blood pressure to < 130/85 mmHg  Glycosylated haemoglobin level of < 7%   e To arrest the progression of the disease  e Role of vasodilators    ¢ Narcotic analgesics can be used judiciously in cases with  rest pain.   2. Buerger’s position by elevating head-end of the bed, causes  venous congestion and reflex vasodilatation.   3. Buerger’s exercises by elevation and dependency of the  limb for a few minutes.   4. Heel raise by raising the heels of shoes by 1-2 cm,  claudication distance can be increased as it decreases work  load on the calf muscles.    « Stop smoking: This is more beneficial in TAO patients  than atherosclerotic patients.   * Diet: Avoid fatty food to reduce serum cholesterol. It 1s  more useful in patients with hyperlipidaemia.    * Vasodilators have not been found to be useful in  atherosclerotic vascular disease.   * Some degree of reduction of pain and healing of cutaneous  ulcers has been found in TAO patients.    ¢ It can be used to treat intermittent claudication  ¢ Pentoxifylline improves blood flow through peripheral  blood vessels. It can also be used to increase sperm motility    Smoking cessation can decrease 10-year  mortality rate from 54% down to 18%    Slow release niacin is emerging as an  important therapy in patients with  dyslipidaemia    when viable sperms are immotile and are being used in  intracytoplasmic sperm injection (ICSI).   Pentoxifylline improves red blood cell deformability (alters  the shape of RBCs) reduces blood viscosity and decreases  the potential for platelet aggregation and thrombus  formation and reduces inflammation and innate immunity.  Dose : 400 mg 3 times a day   It may take 4-8 weeks for the benefit to the patient    Prostacyclin (also called prostaglandin I, or PGI,) is a  prostaglandin member of the family of lipid molecules  known as eicosanoids.   Prostacyclin (PGI,) chiefly prevents formation of the  platelet plug involved in primary hemostasis (a part of blood  clot formation).   It is also an effective vasodilator. Hence used in peripheral  arterial diseases.   Dose: 2 ng/kg/min IV, to start with and increased by 2 ng/  kg/min every 15 minutes as tolerated to a maximum of  16 ng/kg/min intravenous.    It acts by producing vasodilatation of the blood vessels of  the lower limb.   In this procedure, 5 ml of phenol in water 1s injected beside  the bodies of 2nd, 3rd and 4th lumbar vertebrae.   The effect of the drug can be judged immediately by feeling  for warmth in the feet.   It helps in healing of ulcers and may improve rest pain  probably by interfering with afferent sensory circuits.    . Lateral injection by using a lumbar puncture needle.  . Injection should be in front of lumbar fascia which    . Avoid injuries to aorta and inferior vena cava.  . Procedure is to be done under X-ray control (screening)    Since phenol has replaced lignocaine because of its long  lasting effect, it is also called phenol sympathectomy.    1. Genitofemoral nerve  2. Tendon strip of psoas muscle  3. Lymphatic chain and fatty tissue   Lumbar sympathetic trunk is identified in the paravertebral  gutter lateral to the psoas muscle as a cord-like structure.  2nd lumbar ganglion is large and has white rami  joining it.   Sympathetic trunk is divided below the first lumbar vertebra  and removed up to the 4th lumbar vertebra.    This is a preganglionic sympathectomy because fibres  supplying the vessels of the limb have their cell stations in  the sacral ganglia which are not disturbed.    * By depriving the sympathetic nerve supply to lower limb  blood vessels, vasomotor tone is reduced so that some  amount of vasospasm 1s reduced. Thus, rest pain improves,  minor ulcerations heal due to cutaneous vasodilatation.  However, the duration of effect of lumbar sympathectomy  is not clear.  ¢ Both sides can be done in one sitting. However, during  bilateral operation, the Ist lumbar ganglion on one side  should be spared since removal of both ganglia may cause  sterility due to paralysis of ejaculatory mechanism.  ¢ One should be careful not to damage lumbar veins' which  join inferior vena cava.  2. Omentoplasty has been tried in TAO patients. In most of  these patients vasodilator therapy and lumbar sympathec-  tomy has been done with almost no relief of symptoms. In  such cases, before a merciful amputation is done, omento-  plasty 1s attempted.  ¢ By careful division of vascular arcade of omentum, it  can be lengthened based on one of the epiploic arteries,  brought out of the laparotomy incision, tunnelled in the  subcutaneous plane and can be brought up to calf or even  to the ankle joint level in some patients.   3. Conservative amputations should be done if the toes are  gangrenous.   4. Below knee amputation? is the last resort. It is indicated  in severe rest pain cases where all other modalities of  treatment have failed. Risk of amputation after ten years  of the disease is around 10%.    ci \  MAJOR RISK FACTORS FOR  ATHEROSCLEROSIS  Lipids: Dyslipidaemia  Inhalation of tobacco: Smoking  Pressure: Hypertension  Insulin deficiency: Diabetes  Disordered metabolism: Hyperhomocystinaemia  Remember as LIPID    e Presence of diabetes e Angina pectoris  e Chronic smoking e Fitness for anaesthesia  Vi. Surgery in atherosclerotic vascular disease    ¢ The decision to revascularise the limb is taken after an  angiography. The success of reconstruction depends upon  a number of factors. They are given in Key Box 7.14.    ¢ Intermittent claudication alone is not an indication  for surgery. Rest pain and pregangrenous changes in  the limb are definite indications for reconstruction with  accepted mortality and morbidity.    ¢ Surgery can be classified into surgery for aortoiliac  disease and surgery for iliofemoral stenotic disease.    | Aortoiliac disease  It is treated by bypass grafts or endarterectomy.    ¢ Usually, it is bilateral and is treated by using aortobifemoral  graft to bypass the stenosis. The graft, made from either   Figs 7.21 and 7.22: Aortobifemoral graft (Courtesy: Dr Ganesh  Kamath, Prof. and Head of Cardiothoracic Surgery, KMC, Manipal)    teflon or dacron, is used. It is also called Y-graft or trousel  graft. It has commonly 16 mm trunk and two 8 mm  diameter limbs (Figs 7.21 and 7.22).    A. Open endarterectomy: An arteriotomy is done first and  the diseased intima, atheromatous plaque and the thrombus  are removed. An arteriotomy incision can be closed directly  or a vein patch graft is used to close the defect, so as to  avoid narrowing.    B. Closed endarterectomy is indicated in a longer-diseased  segment. In this procedure, after an arteriotomy, a wire  loop is used to strip out a core of atheroma by introducing  it through the lower arteriotomy and removing the  atheromatous plaque from the upper end.    However, results of bypass graft are better than  endarterectomy. The modern tendency ts to do bypass graft.    ¢ This can be repaired by a bypass graft which is sutured  to the normal common iliac artery above and to the normal  femoral artery below (Figs 7.23 and 7.24).   *  Ifpatients are unfit for a major vascular bypass, angioplasty  can be done. Ideal for short segment stenosis.   ¢ In this procedure, a balloon catheter is inserted into the  artery, inflated and its correct position is confirmed by  radiopaque markers which are present tn the balloon. By  inflation and dilatation technique 2—3 times, the stenosed  segment can be dilated (Figs 7.25 to 7.31).   Figs 7.23 and 7.24: lliofemoral graft by using reversed saphenous  vein   Cr ee ee ee ee ee | / ——s  a.  SUMMARY OF THE  REVASCULARISATION SURGERY (Figs 7.25 to 7.31)  Aortoiliac disease Aortobifemoral or aortofemoral  graft and endarterectomy    * Any arterial stenosis can be dilated in angioplasty.  * The technique can be repeated if stenosis recurs.    *. The procedure is done under local anaesthesia and is  indicated in poor-risk patients.    ¢ Ideally suitable for iliofemoral segment, not suitable for  stenosed vessels below knee.    — Internal dissection, distal embolisation, thrombosis and  even rupture of vessel can occur.    * This is treated by using a graft extending from the femoral  artery above, to the popliteal artery below.    ¢ Reversed long saphenous vein is better than other grafts  because it is less thrombogenic.    * Dacron graft, polytetrafluoroethylene graft (PTFE),  human umbilical vein graft are the other grafts.    * Significant occlusion of profunda is demonstrated by  oblique views in an arteriography. If there are no significant  vessels available below the stenosis for reconstruction,  profundoplasty 1s considered. It is done by using a patch  of Dacron or vein to widen the origin of this vessel after  doing endarterectomy.    ¢ Sudden occlusion of an artery is commonly due to emboll.  The source of emboli is from the heart or from atheroma.  Increased incidence of road traffic accidents, fall or war  injuries are other causes. Trauma to the artery also produces  occlusion.    Embolic occlusion  ¢ This occurs commonly in the peripheral arteries such as  the common iliac, femoral and popliteal.    * An embolus is a foreign body to the bloodstream, gets  lodged in a vessel and produces obstruction. It manifests   A 75-year-old lady, known case of polycythaemia vera,  presented to the hospital with multiple gangrenous patches  of skin in the upper limbs and in the lower limbs. Conservative  surgery was attempted by debriding gangrenous portion of  the skin. Polycythaemia is also one of the causes of gangrene.    clinically as severe ischaemia or gangrene, resulting in  critical limb ischaemia (CLI). See clinical notes above    It is defined as persistently recurring ischaemic rest pain  requiring regular, adequate analgesia for more than 2 weeks  or ulceration or gangrene of foot or toes with an ankle pressure  of < 50 mmHg or a toe systolic pressure of < 30 mmHg.    ¢ Atherosclerotic vascular disease, thromboangitis  obliterans, acute embolic ischaemia and even diabetes, etc.  at some stage can present as CLI.    — Pain  — Pallor  — Paresis  Pulselessness  — Paraesthesia  Pain is severe, unbearable, burning or bursting type.  2. Limb is pale, cold and superficial veins are collapsed.  Paresis: Depending on the level of occlusion, the function    4. Pulselessness: Characteristically, peripheral pulses below  the level of embolism are not palpable.   Fig. 7.29: Mid-SFA showing narrowing Fig. 7.30: Balloon angioplasty Fig. 7.31: Postangioplasty image showing  restoration of lumen of mid-SFA    (Courtesy: Prof Chandrakanth Shetty and Dr Praharsha, Resident, Department of Radiodiagnosis and Imaging, KMC, Manipal)   Cyclo-oxygenase Mannitol, NSAID, steroid  inhibitors vitamin E, etc. calcium channel blockers    Fig. 7.33: Critical limb ischaemia (CLI) Fig. 7.34: CLI due to diabetes and atherosclerosis    ¢ If left untreated, necrosis of the muscles followed by  gangrene of the limb can occur within a few hours  (6—24 hours).    Intravenous drug abuse remains a major risk factor for  endocarditis and embolic complications.    Peripheral circulation should be assessed by Doppler  ultrasound, whichis an excellent noninvasive investigation  to judge the severity, level, position and length of  superficial femoral artery stenosis.   ¢ First, the balloon catheter is introduced percutaneously  over a guidewire across the lesion. Under fluoroscopic  control, the balloon 1s dilated until satisfactory widening  of the lumen is achieved.   ¢ Immediate intravenous infusion of heparin (10,000 IU)  is necessary to reduce the extension of the thrombo-  embolism.   removal of clot or by using a Fogarty balloon catheter to    ¢ Embolectomy: Under local or general anaesthesia, a  transverse incision Is given over common femoral artery.  A Fogarty catheter is introduced through the incision for  about 1-2 cm and the balloon 1s inflated.   Try to find out the cause  (History, examination, unilateral or bilateral   . Intestine Superior mesenteric artery Postprandial pain, shock Intestinal ischaemia/gangrene  Kidney Renal artery Pain in loin, haematuria Renal ischaemia    ¢ The catheter 1s then withdrawn and emboli removed.  The procedure is repeated until bleeding occurs. The  catheter can be passed by the ankle and distal embol1  also can be removed. Postoperatively, anticoagulants are  continued.   ¢ Intra-arterial thrombolysis: A catheter is passed into  the ‘clot’ after doing arteriography and t-PA (tissue  plasminogen activator) is infused through catheter.  Repeat angio check films are taken to see the results such  as lysis of clot, revascularisation, etc.    IIl. Thrombolysis  ¢ It is indicated in acute or acute on chronic ischaemia.  ¢ A fine lysis catheter is passed percutaneously into  blocked vessel.    ¢ Streptokinase is infused at a rate of 5000 IU at  10 ml/hr.    ¢ It will reopen the occluded lumen within 24 hours.  ¢ Repeat angiogram is necessary to check for patency.    ¢ These can affect the popliteal artery, femoral artery, iliac  artery, etc. 70% of peripheral aneurysms affect popliteal  artery, and two-thirds of them are bilateral.   ¢ However, students should realise that aneurysms are  uncommon (rare) causes of lower limb ischaemia. Early  diagnosis and effective treatment are essential to save the  limb. Aortic aneurysms are discussed in Chapter 37 under  abdominal mass.   ¢ Pulsatile, tense, cystic, fluctuant swelling behind the knee,  in the popliteal fossa and in the line of popliteal artery.   ¢ Proximal compression test: On occluding the femoral  artery proximally, the swelling may diminish in size.    In all cases of popliteal aneurysm, please search for  femoral and aortic aneurysm.    ¢ Duplex ultrasonography is the investigation of choice  which can measure diameter and determine extent of mural  thrombus.   ¢ Angiography can demonstrate the extent of involved  segment to look for patency and quality of runoff vessels.    1. Thrombosis causes severe acute ischaemia of the lower  limb (incidence: 40%).    4. Compression on the popliteal vein causes pain, tenderness  and swelling of the leg.    5. Compression on the lateral peroneal nerve causing foot  drop, due to paralysis of the peronei and the extensors of  the foot.    ¢ Proximal and distal ligation of the artery followed by  reversed saphenous vein bypass graft is the treatment of  choice. This method results in total obliteration of the sac  with revascularisation of the limb.    ¢ Excision of the sac is better avoided because of chances  of injury to the popliteal vein and nerves. Lateral popliteal  nerve injury causes foot drop and tibial (medial popliteal)  nerve injury causes thinning of the calf region, inability to  plantar flex the ankle and clawing of the toes due to  paralysis of the intrinsic muscles of the foot.    It affects those who do not use footwear or walk barefooted.  It starts as a fissure at the level of interphalangeal joint of a  toe, usually fifth. Repeated trauma of minor degree may be  present. The tissue becomes a fibrous band resulting in tight  constriction and necrosis. If it continues, it may culminate in  autoamputation.   ¢ The division of band or early Z-plasty may be needed to    e It occurs due to too much of exposure to cold weather.   ¢ High altitudes with excessive cold precipitates vasospasm  and damage to the blood vessel wall. It causes sludging of  blood and thrombosis.   e Malnutrition, ageing process are the other precipitating  factors. Severe burning pain, discolouration of foot, develop-  ment of blisters suggest gangrene 1s imminent (Fig. 7.39).   e Paravertebral injection into the sympathetic chain helps in  a few patients.    ¢ This dangerous event follows revascularisation of limbs,  resulting in acute compartment syndrome with com-  partmental pressure exceeding capillary pressure (30  mmHg) (Fig. 7.39).    ¢ Most of the injury is believed to be due to O, derived free  radicals. The most important ones include superoxide  radical, hydrogen peroxide and hydroxy] radical.    e Diagnosis is clinical as suggested by severe pain in the limb.  oedema of the leg and muscle tenderness.   e Raised intracompartmental pressure measured by transducer  cannula will help in the diagnosis.    Often a potentially lethal condition which occurs due to  blockage of major arteries by aggregation of chylomicrons.    Respiratory failure, confusion, petechial haemorrhages:  Triad of fat embolism syndrome. Early diagnosis and  prophylactic administration of oxygen to patients at risk  is recommended.    ¢ Pulmonary: Central cyanosis, tachypnoea, right heart  failure, froth in mouth and nostrils. Respiratory distress  is the most common presenting feature.   * Retinal artery: Striate haemorrhages, fluffy exudates on  fundoscopic examination, cotton wool spots.   * Cutaneous: Petechial rashes in the nondependent parts of  the body—chest, axilla and conjunctiva.   ¢ Free fatty acids (FFA) released at the time of trauma or  breakdown of fat in the lung directly affect pneumocytes  resulting in acute respiratory of distress syndrome (ARDS).    * Arterial blood gases will show hypoxaemia and respiratory  alkalosis from hyperventilation.    AIR EMBOLISM  Definition  It is a potentially fatal condition that can occur due to    blockage of pulmonary artery by large volume of air in the  venous circulation (Key Box 7.18).    * Neck surgery during which a large vein is inadvertently  opened and the patient is in head-elevated position (thyroid  surgery) or sitting position (posterior fossa surgery).   « Rapid infusion of intravenous fluids where emptying of  the bottle and infusion set may go unnoticed and a fresh  bottle is connected and infused without eliminating air from  the infusion set.    Formation of air lock within pulmonary artery and right heart  failure.   * Position the patient head down (Trendelenburg position)  so that air entrainment 1s stopped.   * Turn the patients on their left side so that air will float to  ventricular apex reducing its entry into the pulmonary  artery.    ¢ Peripheral artery occlusive disease is commonly divided  into stages as introduced by Dr René Fontaine in 1954.  ¢ Fontaine classification has defined the severity of chronic  ischaemia as:  Stage 1: Asymptomatic  Stage 2: Intermittent claudication limiting lifestyle  Stage 3: Rest pain due to ischaemia  Stage 4: Ulceration or gangrene due to ischaemia  ¢ Some have also classified stage 2 into A and B:  A — Intermittent claudication > 200 metres  B — Intermittent claudication for < 200 metres    e It has been described previously in literature as a rare  clinical entity. It was first described by Jonathan Hutchinson  in 1891 as multiple extremity ischemia and was termed  “symmetrical peripheral gangrene’.   ¢ Possible aetiological factors include sepsis, DIC and use  of vasopressor agents. The possible responsible drug 1s  noradrenaline (Fig. 7.40).   ¢ Patients in ICU also have multiple arterial punctures for  securing intra-arterial line for invasive monitoring in the  ICU. The arterial punctures can be a possible aetiological  factor.   ¢ Sepsis in the presence of sluggish blood flow has been  described to be responsible for development of symmetrical  peripheral gangrene (Fig. 7.41).   ¢ Vasospastic conditions, small vessel obstruction and very  low cardiac output states (perfusion pressure falling to  35—60 mmHg) may contribute.    Please note: Aortic aneurysm is discussed in Chapter 44 under cardiothoracic surgery.    Medical conditions such as diabetes mellitus, malignancy,  protein C or S or antithrombin III deficiency are also  contributing factors.   The usual manifestation is pallor or cyanosis, coldness and  pain in the extremity. They become erythematous and there  is dusky discolouration of skin, bullae or blisters followed  by gangrene.   Pulses may be intact in the early stages and large vessels  are often spared. Low flow states result in occlusion of the  microcirculation of the affected parts.   The first line of management of this gangrene 1s immediate  discontinuation of vasopressors as soon as discolouration  is seen.    and anticoagulation for DIC are the suggested measures.  Local debridement and secondary skin grafting have been  unsuccessful.   Amputation should be considered only after a clear line of  demarcation develops. The condition has a high mortality  rate of 40%.   e A few clinical photographs and Key Boxes have been  added. More details about medical management of  arterial disease is added.   A. Dorsalis pedis and posterior tibial are often affected A. Respiratory failure, confusion, petechial haemorrhages   B. Ankle pressure is less than 70 mm of Hg  C. Toe systolic pressure is less than 30 mmHg  D. Absent pulses   B. It is the most common type of peripheral aneurysm  C. It is always unilateral   e Thoracic outlet syndrome  e Axillary vein thrombosis  e Vasculitis syndrome    Upper limb ischaemia (ULI) 1s a well recognised clinical entity  as lower limb ischaemia, though it is less common. There are  certain specific conditions which are responsible for ULI such  as cervical rib, Raynaud’s disease, etc. It is also important to  note that reconstructive surgery 1s rarely done in the upper  limb when compared to lower limb. However, students should  be able to identify the ischaemic features in the upper limb  early and refer the case to a suitable specialist, so that it can  be treated promptly and adequately, thereby avoiding a tragedy  such as loss of the limb.    1. Raynaud’s disease and Raynaud’s syndrome  2. Embolic causes  3. Thoracic outlet syndrome (Fig. 8.1)  4. Trauma  5. Buerger’s disease  6. Axillary vein thrombosis  7. Vasculitis syndromes  e Takayasu’s arteritis, giant cell arteritis, polyarteritis  nodosa  ¢ Systemic sclerosis—scleroderma—CREST syndrome   * Jt occurs in young women, commonly.  ¢ Upper limb is more involved than lower limb    Fig. 8.1: Gangrene of the tip of fingers due to cervical rib    Commonly seen in western countries in white-skinned  people. Cold climate is possibly a precipitating factor. It  was first described by Raynaud as bilateral episodic digital  ischaemia of the upper limb on exposure to cold and  emotions. It is also referred to as primary Raynaud’s  phenomenon.   Raynaud’s phenomenon 1s the blanket term used to describe  cold-related digital vasospasm (see pathophysiology).  Raynaud’s phenomenon is subdivided into Raynaud’s  syndrome wherethere 1s an associated disorder and primary  Raynaud’s disease where there 1s none.   CREST syndrome: Calcinosis circumscripta, Raynaud’s  phenomenon, (O) Esophageal defects, Sclerodactyly,  Telangiectasia.    On exposure to cold', some kind of discomfort and colour  changes are observed. This is due to abnormal sensitivity of  the arterioles to cold. Three stages have been described.   1. Stage of syncope: Arterioles undergo constriction as an  abnormal response to cold. As a result of this, the part  becomes blanched and severe pallor develops.   2. Stage of asphyxia: After a brief period of vasoconstriction,  capillaries dilate, filling with deoxygenated blood resulting  in bluish discolouration of the part (cyanosis).   3. Stage of recovery or stage of rubor: As the attack passes  off, relaxation of the arterioles occurs, circulation improves  and redness occurs. Because of dilatation of capillaries,  red engorgement of the part occurs, which causes tingling,  burning or bursting pain in the fingers.   e Pallor, cyanosis and rubor are the colour changes occurring  during the attack along with pain.   ¢ In a few patients, because of recurrent attacks, gangre-  nous patches occur on the tip of the fingers (superficial  necrosis).   ¢ TAO affecting the upper limb usually affects male smokers.  Peripheral pulses are feeble or weak.    'Cold refers to temperature—cold climate (winter), cold environment  (refrigerator), or cold substance like cold water or ice. Erythromelalgia  is a condition wherein heat provokes an attack of burning pain of hands  and feet.   ¢ Calcium antagonists such as nifedipine 10-20 mg, two times  a day may be beneficial.   e In this operation, sympathetic trunk from the lower half of  the stellate ganglion to just below the 3rd thoracic ganglion  1S removed.    e All rami communicantes associated with the 2nd and 3rd  ganglia are removed.    ¢ Nerve of Kuntz, a grey ramus which springs from the 2nd  thoracic ganglion to the Ist thoracic nerve ts also divided.   ¢ Sympathectomy raises the threshold at which spasm  occurs (but the effect seems to be temporary). However,  the severity of the disease is reduced.   INDICATIONS FOR  CERVICAL SYMPATHECTOMY  e Raynaud's disease  e TAO  e Hyperhidrosis  e Cervical rib  e Causalgia   COMPLICATIONS OF  CERVICAL SYMPATHECTOMY  e Perforation of pleura causing pneumothorax  e Lymph fistula due to injury to thoracic duct  e Horner's syndrome  e Injury to the accessory nerve  e Haemorrhage   OTHER VASOSPASTIC DISORDERS  Acrocyanosis  e Women with cyanosis of hands and feet  e Cutaneous vasoconstriction is the cause  Livedo reticularis  e Spasm of arterioles and dilatation of venules  e Worsening by cold  e May be associated with systemic lupus erythematosus  (SLE)  Erythromelialgia  e Burning sensation of hands and feet due to heat    e Thoracic outlet is a tight space with bony structures all  around (Key Box 8.5) such as manubrium sternum in the  front, spine posteriorly and first rib laterally.    * At the root of the neck, brachial plexus and subclavian  artery pass through scalene triangle into the axilla.    ¢ Scalenus triangle is the posterior compartment of  costoclavicular space. The division into anterior and  posterior compartments Is by scalenus anticus. The anterior  compartment contains subclavian vein.    INTRODUCTION TO THORACIC OUTLET  e Tight space with bones all around  e Brachial plexus and subclavian artery are chief contents  e Scalenus anticus muscle is the muscle for landmark  e Vascular compression is more dangerous  e Neurological symptoms are often undiagnosed    ¢ Boundaries of scalene triangle (Fig. 8.2)  Base : First thoracic rib  Anteromedially : Scalenus anticus  Posterolaterally : Scalenus medius    « Ifthe base (the first thoracic rib) is raised by interposition  of cervical rib or any other causes (Key Box 8.6), it results  in compression of the subclavian artery.    ¢ Due to slow compression, artery distal to the compression  dilates due to Jet-like effect and turbulence of blood flow.  This 1s described as poststenotic dilatation (Venturi  effect) (Fig. 8.3 and Key Box 8.7).    « [In this dilated segment, small multiple thrombi develop,  which, when dislodged, result in emboli and distal  ischaemia. Vascular symptoms are strictly unilateral.    This is an extra rib present in the neck in about 1—2% of  the population.   It is the anterior tubercle of the transverse process of the  7th cervical vertebra that attains excessive development  and results in cervical rib.    Type! The free end of the cervical rib is expanded into a  hard, bony mass which can be felt in the neck.  Type If Complete cervical rib extends from C7 vertebra   Type III Incomplete cervical rib, which is partly bony, partly  fibrous.   Type IV A complete fibrous band which gives rise to  symptoms but cannot be diagnosed by X-ray.    Common in young females. Even though congenital.  symptoms appear only at or after puberty. This is because  of the development of shoulder girdle muscles and sagging  of the shoulder which narrows the root of the neck. Nerve  roots C8, T] are stretched by completion of growth around  25 years.    Dull-aching pain in the neck is caused by expanded bony  end of cervical rib.   — Claudication pain is apparent when the arm with muscle  wasting 1s used. Low temperature, pallor, excessive  sweating (vasomotor disturbances), splinter haemor-  rhages, ischaemic ulcers in fingers and gangrene of the  skin of the fingers are the other features. Peripheral pulses  may be absent/feeble. Oedema and venous distension  are very rare. These are called vascular symptoms of  cervical rib.    Features of ulnar nerve weakness (lower nerve roots  involvement, mainly first thoracic nerve) manifest as  tingling and numbness, or paraesthesia in the distribution  of C8, T!. The following are the tests which confirm ulnar  nerve weakness. It includes sensory disturbances and motor  disturbances (performing fine action—writing, buttoning,  etc.)   A. Card test!: The patient is asked to hold a thin paper or  a card in between the fingers. In cases of ulnar nerve  paralysis, due to weakness of interossei muscles, the  patient will not be able to hold the card tightly (Fig. 8.5).   B. Froment’s sign: Patient is asked to hold a book between  the hand and the thumb. In cases of ulnar nerve  paralysis, since the adductor pollicis is paralysed, there  is flexion at the distal interphalangeal joint of the thumb.    This is because flexor pollicis longus, which is  supplied by median nerve, contracts (Fig 8.6).    Adson’s test: Feel radial pulse, ask the patient to take deep  inspiration and turn the neck to the same side. The pulse  may disappear or it may become feeble. This test indicates  compression on subclavian artery (Fig. 8.7).    Hyperabduction test (Halsted test): This test is done to  rule out hyperabduction syndrome caused by pectoralis  minor. The radial pulse becomes weak on hyperabduction  due to angulation of axillary vessels and brachial plexus,  which gets compressed between pectoralis minor and its  attachment to the coracoid process.    Military attitude test: When shoulders are set in backward  and downward positions the radial pulse becomes weak.  This is due to the compression of subclavian artery between  the clavicle and the first rib. This is seen in costoclavicular  syndrome.    Allen’s test: Ask the patient to clench his fist tightly and  compress the radial and ulnar arteries at the wrist with the  thumbs. Wait for 10s and ask the patient to open his hands.   Pallor can be seen in the palm. Now release pressure on  the radial artery and watch for the blood flow. Repeat test  for ulnar artery. If there is occlusion of either artery, colour  changes occur in the fingers slowly (Fig. 8.8).   Elevated arm stress test—EAST (Roos’): Patient is asked  to abduct the shoulders to 90 degrees and to flex the elbow.  Then he is asked to pronate/supinate forearms continuously.  Appearance of symptoms suggests thoracic outlet syndrome.    A hard mass may be visible or palpable in the root of the  neck (Type 1).  On palpation of supraclavicular region, a thrill and on    auscultation, a bruit can be heard in cases of poststenotic  dilatation.    A patient who presents with a few neurological symptoms and  signs in the upper limb with a cervical rib may be having some  other causes for those symptoms. Hence, it 1s important to  exclude other causes.    Cervical spondylosis: This should be considered as a  possibility in patients above the age of 40 years.    such as myxoedema, rheumatoid arthritis and malunited  Colles’ fracture. Predominant features of median nerve  involvement, more so In menopausal women gives a clue  to the diagnosis.    . X-ray neck may show a cervical rib (Types I, II and III).    Interestingly Type IV variety, a fibrous band which cannot  be diagnosed by X-ray or by any other investigation, usually  gives rise to symptoms (Fig. 8.9).   ¢ Presence of vascular symptoms and signs are the definite  indications for surgery.   e¢ Excision of cervical mb including periosteum is called  extraperiosteal excision of cervical rib (so that it will not  regenerate). This is combined with cervical sym-  pathectomy if vascular symptoms are predominant.   e Ifthere is a thrombus 1n the subclavian artery, it is removed  and the artery 1s repaired (Fig. 8.11).    2. Cervical disc protrusion and spinal cord tumours may have  predominant neurological features and thus may mimic  cervical rib. When in doubt, ask for MRI.    I. Conservative  e Patients with mild neurological symptoms are  managed by shoulder girdle exercises or correction of faulty    ¢ At exploration, if cervical rib is not found, scalenus anterior  muscle is divided. This is called scalenotomy (Key Box  8.9). If hyperabduction syndrome is diagnosed, pectoralis  minor is divided from its insertion into the coracoid  (€jjnical features    e [t is a panarteritis involving aortic arch and its branches.  Subclavian artery is involved in 85% of the cases.    IF CERVICAL RIB IS NOT FOUND  e Scalenotomy  e Division of pectoralis minor  e Extraperiosteal resection of the first rib    from the dominant hand Thrombosis and stenosis can occur later.  * Hypertrophy of subclavius muscle also can cause    ¢ Hypertension is common in 50% of the cases due to renal  artery involvement.   e Visual disturbances can occur due to involvement of retinal  arteries. Late blindness can occur.   ¢ Thrombolysis or if necessary venotomy, removal of the Ne  thrombus and Ist rib (if it is the cause of obstruction) are VASCULITIS SYNDROMES  the treatment modalities. ¢ Aetiology is inflammatory or immunological.    * Axillary vein thrombosis is also a complication of axillary — « Uncommon causes of upper and lower limb ischaemia.  block dissection specially where extensive nodal dissection .    Women are affected more than men.  e Multiple small vessel involvement.  e Symptoms are confusing, depending upon organ involve-    TAKAYASU’S ARTERITIS (PULSELESS DISEASE) involves the limbs.  : e Steroids are useful in controlling the disease.  ¢ Itis of unknown aetiology.    ¢ C-reactive protein 1s elevated as part of acute phase response  (nonspecific).   ¢ Duplex-Doppler ultrasound, MR angiography can  diagnose the site of obstruction and blood flow pattern.    e Very early cases benefit with steroid therapy, e.g. tablet  prednisolone 30 to 50 mg/day (anti-inflammatory effect).  Cyclophosphamide can be tried when other measures fail  (immunosuppressive effect).   ¢ Elderly women presenting with severe headache is the  common presentation.    ¢ Biopsy of the temporal arteries will reveal giant cell  granuloma, comprising mainly CD4+ T lymphocytes.    ¢ This includes microscopic polyarteritis (commonly) and  polyarteritis nodosa (less often).   ¢ Ischaemia of the lower limbs and upper limbs can occur  due to involvement of small vessels.   ¢ Involvement of renal arteries causes loin pain, haematuria  and hypertension.    ¢ Earlier called collagen vascular disorder because of obstruc-  tion of the small vessels by collagen deposition.   ¢ Ischaemic changes occur in the fingers and toes. Necrosis  and ulceration are common.   ¢ Raynaud’s symptoms can be controlled using calcium  channel blockers and nitrates.    Definition  Macroscopic death of tissue with superadded putrefaction. It  affects the limbs, intestines, appendix, etc. In this chapter    differential diagnosis of causes of gangrene of the limbs is  considered (Figs 8.14 to 8.19).    Rest pain, colour changes at rest and with exercise, oedema,  hyperaesthesia, skin ulcerations are due to inadequate blood  supply to the limb. These changes are described as  pregangrenous changes tn the limb.    . Physical causes: Sunrays, radiation, corrosive acids  . Drugs: Ergotamine  . Diabetic gangrene   Fig. 8.14: Peripheral gangrene due to sepsis and vasopressors Fig. 8.17: Left medial 2 fingers gangrene due to TAO   Fig. 8.15: Emboli at the brachial artery resulting in massive  gangrene of the hand (wet gangrene)    e A part which is gangrenous ts a dead portion of the body.  It has no arterial pulsations, venous return or capillary  filling.   ¢ The colour initially will be pale and later it changes to dusky  grey and finally black. The black colour is due to  disintegration of haemoglobin and formation of iron  sulphide.   e The gangrenous part has to be treated with surgical excision  or debridement, which may amount to either disarticulation  of the toe or even an amputation.   e The gram-positive, gram-negative and anaerobic organisms  multiply in this segment and can produce septicaemia.  Thus, this may precipitate multiorgan failure including  renal failure, adult respiratory distress syndrome (ARDS),  cardiac failure, etc.    e Loss of pulsation  e Loss of temperature  e Loss of function    Basically there are two types—dry gangrene and wet gangrene.  They are compared in Table 8.1.   — Major diseases such as diphtheria, whooping cough,  typhoid, measles and kala azar.   ¢ Occasionally, as the disease progresses, whole thickness of  the cheek may be lost.   3. Appropriate antibiotics: Metronidazole 400 mg three times  a day tor 7-10 days.    1. Fibrosis causing restriction of the movement of jaw.  2. Septicaemia, toxaemia and death.   ¢ Persistent cyanotic discolouration of hands when exposed  to cold is a feature.   ¢ This is brought about by intermittent spasm of small  peripheral vessels. Commonly affects hands, rarely feet also.   ¢ Abuse of the drugs is an important cause of gangrene in the  modern days.   e Inadvertent injection of drugs into artery can lead to  thrombosis of the artery resulting 1n acute 1schaemia—  commonly in the brachial artery.   Sudden occlusion of the arteries —  Large area is affected due to absence of collaterals  Wet, turgid, swollen, oedematous  Absent  May be present  Foul odour due to sulphurated hydrogen produced by  putrefactive bacteria  Usually present  Emboli, ligatures, crush injuries   Emergency treatment in symptomatic cases include  heparinisation and infusion of dextran.    Inadvertent intra-arterial injection of thiopentone into  one of the high divisions of brachial artery, (congenital  anomaly) usually the ulnar, will result in severe burning  and blanching of the hand (Figs 8.20 and 8.21).   If this complication is noticed following steps (measures)  have to be taken immediately.    After the injection, initial signs and symptoms occur very  fast (within 15—20 seconds). It consists of intense forearm  pain and mottling of the skin on his hand.   Minutes later, discolouration and nail bed pallor became  evident. Approximately 3 to 4 hours later, the symptoms  had progressed to paraesthesias and pronounced hand  weakness.   Rapid development of signs indicative of necrosis (by the  eighth day) required the patient to undergo fasciotomies,  multiple debridements, and 4 skin grafts for cosmesis.  Step 1: If iatrogenic, maintain the intra-arterial catheter in  place—do not remove it   Step 2: Identify the progress of the disease—colour  changes, necrosis, gangrene   Step 6: Perform specific interventions—angiogram, intra-  arterial thrombolysis, vasodilators, prostacyclines, sym-  pathectomy, corticosteroids    ¢ Ergot preparations are used by patients with migraine over  a long period.   ¢ Ergotamine gangrene occurs in those who eat bread infected  with Claviceps purpurea. Example: Dwellers on the shores  of the Mediterranean Sea and the Russian Steppes.    ¢ Development of symptoms in either brain or in the arm  because of subclavian artery obstruction is referred to as  subclavian steal syndrome (Key Box 8.14).   ¢ [tis most commonly used to describe reversed flow in the  vertebral artery ipsilateral to proximal subclavian artery  stenosis or occlusion.   e Arm symptoms—fatigue, pain with exercise, para-  esthesias, coolness and heaviness on the affected side   e Asymmetrical radial pulses or difference of 20 mmHg  systolic pressure between the upper limbs.    e A few Key Boxes and figures have been added.  e Subclavian steal has been discussed.   e A few vasospastic diseases have been added.  e inadvertent intra-arterial injection   1. Which of the following is usually not a feature of   D. Pallor, cyanosis and rubor are the colour changes  during the attack    A. Scalenus anticus  B. Scalenus medius  C. Scalenus posterior  D. Levator scapulae    . Following is the treatment of choice for cervical rib  with ischaemia:   C. Excision of the rib with division of pectoralis minor  D. Excision of rib with division of scalenus anticus muscle   D. It continues as axillary artery at the medial border of  first rib    Compression of the subclavian artery is detected by  following clinical test:    Which is the test to detect the dominant arterial  supply in the hand circulation?    Lymphatics and lymph vessels play the role of draining the  waste fluid from the body. Hence, they are vulnerable for  various infections. The lymphatics are connected to a group  of lymph nodes and then drain into the veins. Hence, infections  of the lymphatics give rise to enlargement of lymph nodes.   In this chapter, significant surgical diseases affecting the  lymphatics and lymph nodes are discussed.    compartment and subcutaneous tissues results in enlargement  of the limb. It is protein-rich interstitial fluid.   ¢ Scrotum: Elephantiasis of the scrotum is caused by filarial  organism (Wuchereria bancrofti).    ¢ To return protein-rich fluid to circulation through  lymphaticovenous junctions in the jugular area.    e This fluid includes water, electrolytes, low molecular weight  substances such as polypeptides, growth factors and  cytokines.   ¢ It also includes macromolecules such as fibrinogen,  albumin and globulin.   ¢ Transport of cholesterol, long chain fatty acids,  triglycerides and fat soluble vitamins (A, D, E, K) by  intestinal lymph into circulation. They bypass liver and enter  circulation through cisterna chyli and thoracic duct into the  left internal jugular vein (lymph from lower limbs, abdomen  and left arm).   ¢ Right lymphatic duct drains into right internal jugular vein  (lymph from head and right arm).    ¢ Lymph flow is largely due to intrinsic lymphatic  contractility by lymphangions (segment of lymphatics).  ¢ Transient increase in interstitial pressure secondary to  exercise and limb movements also helps to a certain extent.  ¢ Valves prevent reflux in the lymphatics (Fig. 9.3).    ¢ A few cases which occur later in life may be due to  unnoticed factors—repeated bacterial and fungal infections,  trauma to feet, etc.   ¢ Loss of venoarteriolar reflux (VAR) which protects lower  limb capillaries from excessive hydrostatic forces in the  erect posture, occurs in advancing age. Diseases such as  diabetes and chronic venous insufficiency may also  contribute.    I. Hereditary: Associated with syndromes such as Turner’s  (XO), Klinefelter’s (XXY), Down’s (Trisomy 21).    ¢ Lymphoedema develops between puberty and middle age  (50 years). It may involve arms also.  ¢ A few are inherited in an autosomal dominant manner.    Lymphoedema leads to an impairment of immune  surveillance and predisposes to other malignancies (Key  Box 9.2).    Fig. 9.4: Congenital lymphoedema—observe the right leg. One  attempt has been made to decrease lymphoedema by excision    Milroy’s disease is a type of lymphoedema congenita whicl  runs in families.    Depending upon the time at which lymphoedema appears  it can be classified as follows:    Birth Lymphoedema congenita.  Puberty - Lymphoedema praecox.  Later life - Lymphoedema tarda.   ¢ Initially, it causes lymphangitis which clinically presents  with high grade fever, chills and rigors, red streaks in  the limb, tenderness and swelling of the spermatic cord  and scrotum (Table 9.1).   ¢ The lymph nodes are swollen and tender. Retro-  peritoneal lymphangitis produces acute abdominal pain.   ¢ Due to such repeated infections, fibrosis occurs resulting  in lymphatic obstruction. Later, this gives rise to  lymphatic dilatation. Lower limb lymphatics are dilated  and tortuous (lymphangiectasis).   ¢ To start with, lymphoedema is pitting in nature and  progressively becomes nonpitting in nature. Lymph  (protein) provides good nourishment for fibroblasts.   ¢ After repeated infections, the skin over the limb becomes  dry, thickened, thrown into folds and even nodules that  break open and result in ulcers, Hence, it 1s called  ‘elephant leg’. Lack of nutrition and infection precipitates  lymphoedema. Oedema 1s also due to reflux of lymph    Lymphangiosarcoma (Stewart-Treves syndrome)—  sarcoma arising in a lymphoedema after treatment of  carcinoma breast    from para-aortic vessels into the smaller lymphatics  draining the lower limb. Subcutaneous tissue ts grossly  thickened. The presence of deep fascia prevents  involvement of the deep muscles of the lower limb (Key  Box 9.3).   Skin on the dorsum of the toes cannot be pinched because  of subcutaneous fibrosis—Stemmer’s sign   In early cases, it pits on pressure. Later, oedema does not  pit and does not reduce even with elevation.   Advanced cases: Chronic eczema, fungal infections  (dermatophytosis) and nails (onychomycosis), fissuring and  warts.   However, once ulcers start, recurrent bacterial infections  are common, thus worsening the disease process.    Grade IV -— Oedema-nonpitting: Not relieved, warty  projections, elephantiasis, lymphorrhoea present.    1. Filariasis is the most common cause of elephantiasis of  the leg in endemic areas such as coastal Kamataka, coastal  Andhra Pradesh, Tamil Nadu, etc. (Table 9.3).   2. Congenital A-V fistula can present with unilateral  gigantism of the leg. Dilated veins, continuous murmur,  gigantism, nonhealing ulcer in the leg in a young boy give  the clue to the diagnosis.   Unilateral, begins distally, spreads proximally  Capillary haemangioma may be present  Regional lymph nodes are absent    3. Elephantiasis neuromatosa of the leg can cause diffuse  enlargement of the leg. The leg is tender on palpation with  soft to firm diffuse swelling.    By and large, no investigations are done in secondary  lymphoedema. In selected cases of primary lymphoedema,  investigations can be done but are largely of academic interest.  Comparison of primary and secondary lymphoedema is  shown in Table 9.3.  Causes of lymphoedema are shown in Key Box 9.4 and  Table 9.4.    Lymphangiography is an investigation wherein a dye 1s  injected into the lymphatics and the entire draining lymphatics  and lymph nodes are visualised.    2. In cases of lymphoma, to detect pelvic nodes, para-aortic  nodes, etc. but now CT scan is preferable.   Chronic lymphadenitis  2. Surgery Lymph node block dissections (inguinal or axillary)  3. Lymph node obstruction Postradiotherapy large nodes—metastasis lymphoma  4. Tissue damage Burns scarring  5. Venous diseases Chronic venous insufficiency, venous ulcer  6. Endocrine Pretibial myxoedema    Fig. 9.8: Acase of carcinoma of the breast  presented with lymphangitis    ¢ 5-10 ml of methylene blue (patent blue) is injected into the  web spaces intradermally between the toes. This delineates  the lymphatics of the dorsum of the foot which are  identified. Then, an oily dye such as “ultra fluid lipoidol”  is injected (10-15 ml).   ¢ It may take 12-24 hours to delineate inguinal nodes and  para-aortic nodes.    e Metastases appear as irregular filling defects in the lymph  nodes.   e It may demonstrate hypoplasia or hyperplasia as in primary  lymphoedema.   e If there is obstruction, the dye may return back (dermal  back flow).    1. Congenital hyperplasia (10%): This ts the condition  Wherein lymphatics are increased in number. These  megalymphatics are defective resulting in chylous ascites,  chylothorax and end in intestines resulting in protein loss  (protein-losing enteropathy). Being congenital, it manifests   Recurrent lymphangitis  Lymphadenitis  Lymphatic destruction  Excision of lymph nodes as part of treatment  Scarred and obliterated lymphatics    at birth, 1s progressive, the whole leg 1s involved and shows  variable response to compressive therapy.  . Distal obliteration (80%) (Key Box 9.5)  3. Proximal obliteration (10%)  e It is less common  ¢ Obstruction is at aortoiliac or inguinal nodes region.  ¢ Whole leg/thigh is involved (Fig. 9.11).  ¢ No family history  ¢ Rapid progression and poor response.    Complications of l_ymphangiography  1. Lymphangitis and toxaemia  e It 1s not being done routinely nowadays, because of  availability of ultrasound, CT scan and other noninvasive  investigations.    2. Rapid infusion is to be avoided for the fear of lipid    Ithasa sensitivity and specificity of 92% and 100% respectively.  It has replaced lymphangiography.   Radiolabelled (technetium 99m) colloid is injected into web  space between 2nd and 3rd toes or fingers. Limb is exercised  periodically and images are taken.   If there 1s abnormal accumulation of tracer with collaterals,  it is a sign of lymphoedema.   MRI and CT scan are the latest investigations in addition  to lymphangiography for the evaluation of gross swelling  of the limb.    e Graduated pressure highest at ankle (100%) to lowest in the    e Itrequires about 40-60 mmHg to leg and 30-40 mmlbg to arm.  e MLLB should be worn at the start of the day and removed at    The aim is to get a graduated pressure in applying. If it is  applied wrongly, it may result in increase in lymphoedema.    ¢ Massage: Manual lymphatic drainage (MLD)  ¢ It aims at draining fluid and protein from ISF space.  ¢ It also stimulates lymphangion contraction.   ¢ Moisturiser or liquid paraffin should be applied for dry  skin   ¢ Hyperkeratosis should be treated by keratolytic agen:  such as 5% salicylic acid.    ¢ Painis multifactorial. It can be due to swelling, infection,  involvement of nerve or bone, etc.   ¢ However, it is often psychosomatic and affected by mood  and morale.   ¢ Oral or injectable penicillin and flucloxacillin or  clindamycin are the drugs of choice.    ¢ Oxerutins are the drugs used for venous disease. These  are not yet licensed in the UK but are used in India.    ¢ They help by reducing capillary permeability, improve  microcirculatory perfusion, stimulate interstitial  macrophage proteolysis and reduce erythrocyte and  platelet aggregation.   ¢ Diuretics: 20 mg of furosemide every day/alternate days.  This helps in early cases of lymphoedema.   ¢ Antifilarial treatment: Diethy] carbamazine citrate  (DEC) 100 mg 3 times/day for 21 days with every attack  of lymphangitis and once in 6 months.   ¢ Warfarin has been used in reducing lymphoedema due  to filariasis. It acts by enhancing macrophage activity  and extralymphatic absorption of interstitial fluid.    1. Swiss-rol! operation (Thompson’s): In this a skin flap is  raised containing dermis and it ts buried into the deep tissues  (close to vascular bundle). This is a dermal flap prepared  by denuding epidermis (Fig. 9.12).   2. Charles excision operation: It is indicated in primary  lymphoedema. It is performed for extensive swelling and  skin changes (Fig. 9.13).   A B C  Figs 9.14AtoC: (A) Node to vein, (B) Lymphatics  threaded into a vein and (C) Microvascular  anastomosis    e In this operation, thickened, diseased skin and  subcutaneous tissue are excised till the healthy underlying  structures are seen followed by split skin grafting.   ¢ The skin has dermal lymphatics which are never  involved in filariasis. Thus, the subcutaneous lymph may  flow via dermal lymphatics.    3. Nodovenous shunt: Dilated, enlarged lymph node tn the  inguinal region is anastomosed to a vein nearby, e.g. long  saphenous vein or femoral vein (Fig. 9.14).    Thus, these are 3 types of surgery commonly done for  filarial leg (Fig. 9.15). There are many other surgeries which  are of historical interest. However, the results of surgery for  filarial leg are disappointing. Many patients develop intractable  ulcers and wetting of the limb due to loss of protein. The wound  gets secondarily infected resulting in sepsis, recurrent  lymphangitis, etc. As a last resort many patients beg for  amputation, to get rid of the ‘useless limb.’ After amputation  the limb can be fitted with prosthesis.    Large atypical cells (Reed-Sternberg cell)  Express CD15 and CD30 (20 and 45 negative)    ¢ Plantar oedema develops and spreads rapidly.  ¢ Itisa preventable condition and wearing of shoes can slow  down the progression.   ¢ Superficial venous thrombophlebitis (SVT) and deep vein  thrombosis (DVT) can lead to lymphatic destruction and  secondary lymphoedema. Lymphoedema can predispose to  DVT.    Definition  This is amalignant neoplasm of lymphoreticular system. Thus,  it can involve lymph nodes, spleen and liver.    ¢ History of infectious mononucleosis increases risk of HL  two to three fold. Epstein-Barr virus (EBV) may be a  causative agent.    ¢ The disease starts usually in one of the lymph nodes as a  painless swelling.   ¢ Commonly, it involves the left supraclavicular region. The  nodes are enlarged without matting. It spreads to other  nodes in a downstream lymphatic drainage (contiguous  spread).    I. Lymphocyte predominance, nodular (both Hodgkin’:  lymphoma and low grade B cell lymphomas)    However, classical HL and nodular lymphocyte predominant  (HL)—LPHL is now more practically used classification  (Table 9.5).    ¢ Age: Bimodal distribution. First peak in the 3rd decade and  second peak after 50 years.    e It presents as generalised lymphadenopathy. Generalised  lymphadenopathy means when more than one group of  lymph nodes are enlarged and are significant.    + Disease starts in the left posterior triangle as a group of  lymph nodes with a ‘bunch of grapes’ appearance . This is  seen in about 80% of cases. By means of contiguous and  centripetal spread, other lymph nodes in the neck, axillary,  mediastinal, para-aortic and inguinal lymph nodes get  enlarged (Figs 9.16 and 9.17).    ¢ The nodes are firm (India rubber consistency) without  matting. In advanced cases and in poorly differentiated  variety, matting can occur.    ¢ Abdominal pain can occur due to hepatosplenomegaly,  which are smooth and firm with round borders.    ¢ Multiple bony pains can occur due to secondary deposits,  especially in the lumbar vertebrae. The secondary deposits  are usually osteoblastic giving rise to ivory vertebrae.   mediastinal nodes. This 1s tested by asking the patient to  Fig. 9.16: Cervical lymphadenopathy due to Hodgkin’s lymphoma. raise the hand above the head. Engorgement of the veins  Lymph nodes are firm or rubbery in consistency indicates obstruction and the test 1s said to be positive  (Pemberton’s test).    eoclUlUtlmlmUCOUlCCUCOU 8 e e e e e e e e e e e e e e e e   e Most dramatic response to the chemotherapy/radiotherapy  with a cure rate of 90% in early Hodgkin’s and 60% in  advanced stage.   e Even in patients with mediastinal bulky lymphoma, symptoms  of cough, chest pain, dyspnoea or SVC obstruction is not  common.   ¢ Mediastinal mass ratio (MMR), defined as the ratio of  maximum transverse diameter of mediastinal mass to the  maximum transverse intrathoracic diameter, must be  calculated. MMR greater than 0.33 by chest X-ray or 0.35 by  CT predicts a worse prognosis.    e Para-aortic nodes (Key Box 9.8) are felt in the umbilical  region, more so on the left side. Its clinical features are:  — Nodular, firm to hard mass, fixed mass PEARLS OF WISDOM    Generalised pruritus occurs in 25% of patients with  Hodgkin’s lymphoma. It can be the only presenting    'Mycosis fungoides: It is not a fungal infection but is caused by non-Hodgkin’s lymphoma with infiltration of the skin with malignant lymphocytes.  Dermatitis and papular rashes are common which progress to tumour formation. It is a variety of cutaneous T cell lymphoma.    | Involvement of a single lymph node region or lymph  node structure  ll Involvement of two or more lymph node regions on  the same side of the diaphragm  HI Involvement of lymph node regions or structures on  both sides of the diaphragm  IV Involvement of extranodal sites beyond “E” sites   B—Fever, weight loss > 10% over 6 months, or night sweats  E—Involvement of a single extranodal site contiguous or proximal  to known nodal site    . Chest X-ray 1s taken to rule out mediastinal lymph nodes,   ¢ However, CT scan of the abdomen ts better to define  para-aortic nodes when there is minimal enlargement  (0.5 cm).    . CT scan  ¢ CT scan is the investigation of choice for staging.  ¢ CT scan of chest, abdomen, pelvis is mandatory.  ¢ CT of the neck ts optional.  ¢ CT will also calculate MMR (see to mediastinal  lymphoma)  ¢ CT scan will also help assess renal function and can detect  back pressure hydronephrosis.  . Lymph node biopsy: Incision biopsy is done and a neck    node is usually removed. Fine needle aspiration cytology  (FNAC) may give the diagnosis but a definite histological  pattern cannot be made out by FNAC. A trucut biopsy can  also give the diagnosis.    . ABVD 4 to 6 cycles  — Doxorubicin 25 mg/m? IV, days 1 and 5  — Bleomycin 10 mg/m? IV, days | and 5  — Vinblastine 6 mg/m? IV, days | and 5  ~— Dacarbazine 375 mg/m? IV, days | and 5   ¢ Lung cancer, breast cancer, melanoma, soft tissue  sarcoma, thyroid cancer can develop after 30 years.    Highrisk patients have bulky disease. There are many othe  regimens available. Students can refer to medical oncology  manuals.   Procarbazine 100 mg orally, for | to 10 days.  Prednisolone 15 mg 8th hourly orally, for | to 10 days.  Minimum of 6 cycles or at least 2 extra cycles after attaining  complete remission should be given. In spite of stage III  and stage IV disease, survival of 10 years with disease-free  interval is about 80% (Key Box 9.10).   ¢ 60 to 70% of patients with advanced stage Hodgkin’s  lymphoma can be cured with 6 cycles of ABVD chemo-  therapy which ts superior to older MOPP.    4. LPHL  ¢ Involved field RT alone is for treatment of early stage  LPL.  ¢ Stage III and stage IV are treated by combination  chemotherapy.    5. Recurrent Hodgkin’s disease  ¢ All patients who are less than 70 years, who relapse after  therapy can be considered for autologous haemato-  poietic cell transplant (HCL).  ¢ It should be remembered that drug therapy and HCL can  be offered to those who are fit patients only and toxicity  of all the drugs should be explained to these patients.    Extended mantle field radiotherapy can cause carcinoma  of the breast. Pelvic radiotherapy (inverted Y) can cause  infertility. Hence, they are not commonly used now.   2. HL decreases the cell-mediated immunity. It causes  depressed CD4+/CD8+ ratio. They are vulnerable for  opportunistic infections—mycobacteria, herpes zoster,  cytomegalovirus.    With availability of CT scan, ultrasound and lympho-  scintigraphy, all the details of involvement of intra-  abdominal viscera or pathology can be detected. Thus,  staging laparotomy is not done any longer.   5. Intravenous pyelography: Renal function can be easily  assessed by CT scan.    ¢ RNA viruses: Human immunodeficiency virus (HIV)  produces AIDS. These patients can develop high grade  B cell lymphoma.    ¢ DNA viruses: Epstein Barr viruses (EBV) can produce  Burkitt’s lymphoma.  3. Bacteria  ¢ H. pylori: Gastric extranodal marginal zone B cell  lymphomas of MALT type.  4. Immunodeficiency states  ¢ AIDS  ¢ Organ transplantation    I. B cell NHL: Small lymphocytic lymphoma, follicular  lymphoma, Burkitt’s lymphoma (Key Box 9.11).   Il. T cell NHL: Cutaneous T cell lymphoma, mycosis  fungoides and Sezary syndrome, lymphoblastic lymphoma.    It depends on following factors: * Complete blood count  « Grade of tumour ¢ Liver function tests including LDH  ¢ Stage of disease  — Stages I and II: Low grade—radiotherapy  — Stages I and I]: Intermediate and high grade—  chemotherapy  — Stages III and [V: Chemotherapy    LDH is an important prognostic factor and aggressive  therapies are required for patients with increased LDH.   Fig. 9.21: Advanced case of non-Hodgkin's lymphoma with Fig. 9.22: Massive cervical lymph node enlargement. Observe the  involvement of groin nodes, testicle and skin deposits in the thigh scar of the lymph node biopsy on the opposite side, reported as normal   Fig. 9.23: Bilateral lymph nodes in the upper Fig. 9.24: Preauricular, postauricularand Fig. 9.25: Epitrochlear lymph node  neck facial lymph nodes enlargement   ¢ Core needle biopsy, lymph node biopsy and bone marrow  biopsy.   ¢ Immunohistochemistry is required for subclassification.  Examples: Small lymphatic lymphoma (CD5+, CD23+).    If Waldeyer’s ring is involved, an upper GI scopy should  be considered since the incidence of gastric involvement  in such cases is increased.    ¢ Combination chemotherapy with COPP (cyclophos-  phamide, oncovin, procarbazine, prednisolone) or ABVD  with or without rituximab is the treatment of choice.    ¢ Indolent lymphomas can also be treated with same regimen  with or without involved field RT (IFRT).   In 40% of cases, iliac crest is involved by the time of diagnosis  Absent    ¢ Gastric MALT lymphomas may completely regress with  therapy against #7. pylori with appropriate antibiotics and  proton pump inhibitors.    e [tis a type of high grade non-Hodgkin’s lymphoma first  described by Burkitt affecting jaw bone (maxilla, mandible).   e Itis caused by Epstein-Barr virus (EBV) which multiplies  in presence of heavy malarial infestation.    ¢ Endemic: Parts of Africa and other tropical locations. It is  associated with EBV. Affects jaw and orbit. It has good  prognosis.   ¢ Sporadic: Throughout the world. It is not associated with  EBV. It affects abdomen and GI tract rather than bones,  has poor prognosis.    e Endemic cases respond well to cyclophosphamide.  ¢ Sporadic cases need to be treated with combination  chemotherapy.    It is also a type of cutaneous T cell lymphomas. Here, skin  involvement is manifested clinically as a generalised  exfoliative erythroderma along with an associated leukaemia  of Sezary cells.   These cells are characterised by cerebriform nucleus. These  are indolent tumours having good survival time.    e It 1s the commonest manifestation of rupture of lymph  vessels due to filariasis (others being chylocoele, chylous  ascites and chylothrax).   e It occurs when intestinal lymphatics are obstructed by  filarial fibrosis and the lymphatics are diverted to renal  lymphatics.   ¢ The dilated and tortuous lymphatics, due to high pressure,  rupture into the renal pelvis and ureter leading to chyluria.  Passage of large amount of chyle results 1n the loss of protein  and fat resulting 1n malnutrition. Such patients are also  debilitated with loss of 1mmunity.   ¢ Oral ingestion of fat labelled with Sudan red III turns the  urine pink in these cases.   e It 1s treated by diethyl carbamazine, bed rest, elevation of  foot end of bed and administration of high protein diet.   ¢ Cytokeratins are expressed by epithelial cells and  cytokeratin positivity suggests carcinoma.   e¢ Similarly, endothelial cell markers, lymphoid markers,  neuroendocrine markers or melanoma markers are  available.    e Thus, immunohistochemical stains can detect all types and  can confirm malignancy. $100 and actin stains can be used  to identify myoepithelial cell layer in a duct or gland.    e JHC may also help in selection of treatment and prognost!  predictions. Examples: Carcinoma of the breast 1s assesse  for oestrogen receptor, progesterone receptor and HER  status.    ¢ IHC can also detect infections such as cytomegaloviru  (CMV), Epstein-Barr virus (EBV), etc.    ¢ IHC can also be used to detect abnormal accumulation c  proteins or amyloid.    ¢ Autologous transplants (which use stem cells from th:  patient ) have the risk of reintroducing lymphoma cells bac!  into the patient after treatment.   ¢ Drugs to eliminate graft-versus-host disease in allogener  (donor) transplants. This work revolves around altering thi  transplanted T cells so that they will not react with the  recipient’s normal cells but still kill the lymphoma cells.   ¢ Targeted therapies: New drugs are—bortezomit  (Velcade), romidepsin (Istodax), and temsirolimus (Torisel)   ¢ Antibiotics: Gastric MALT lymphoma, which 1s linked tc  infection by the bacteria Helicobacter pylori, can often be  treated with triple therapy. Doxycycline also 1s helpful.   ¢ Monoclonal antibodies: “Immunotherapy for non-  Hodgkin lymphoma.” Rituximab ts most often given for <¢  short period in follicular lymphoma. Other similar drugs  such as ibritumomab and tositumomab, epratuzumab, whict  targets the CD22 antigen on certain lymphoma cells, anc  obinutuzumab, which targets the CD20 antigen.   ¢ Immunotoxins: They act as homing devices to deliver the  toxins directly to the cancer cells. One example of this 1s  brentuximab vedotin (Adcetris), which 1s made up of an  antibody to CD30 that 1s attached to a cell poison. It has  been shown to help treat patients with anaplastic large cell  lymphoma (ALCL) that 1s not responding to treatment with  chemotherapy.    . Which of the following is not true regarding function  of the lymphatic system?    D. It does not accompany neurovascular bundles in central  nervous system   C. Skin on the dorsum of the toes cannot be pinched  D. Buffalo hump on the dorsum of the foot   . Following is true for filarial elephantiasis of leg except:  A. It is caused by Wuchereria bancrofti   . Majority of the primary lymphoedema is due to:  A. Congenital hyperplasia of lymphatics    Which of the following is true in lymphoedema?  A. Dermal lymphatics are never involved in filariasis  B. Oxerutins are not used in lymphoedema    15. Following are causes of epitrochlear enlargement C. It can also be used to treat follicular lymphomas    exCaps D.It has no role in transplant rejections  A. Hodgkin’s lymphoma  B. Non-Hodgkin's lymphoma  C. Secondary syphilis A. It is associated with Epstein-Barr virus  D. Cat-scratch fever    B. It is used to treat leukaemia D. It gives starry sky appearance in histological sections    ANSWERS  1D 2C 3 D 4C 5 D 6B 7D 8A 9A 10 A  11 C 12 D 13 D 14 D 15 A 16 D 17 B    e Surgicalanatomy of venous system of legs  e Anatomy of the long saphenous vein    Varicosity is the penalty for verticality against gravity. This  is the common statement made in lecture classes. The blood  has to flow from the lower limbs into the heart against gravity  because of the upright posture of human beings. In many cases,  varicose veins are asymptomatic. Raised intra-abdominal  pressure also precipitates varicose veins, more commonly  in females due to repeated pregnancy. The complications  of varicose veins are responsible for hospitalisation of the  patient.    Definition  Dilated, tortuous and elongated superficial veins of the limb  are called varicose veins.    They occur as a result of congenital weakness in the vein  wall due to defective connective tissue and smooth muscle.  It can also be due to muscular weakness or due to congenital  absence of valves (Key Box 10.1).   Very often, the valve at the saphenofemoral (SF) junction  1s incompetent/absent. The valves can also be absent where  the superficial veins join the deep veins.  Klippel-Trénaunay syndrome is a congenital venous abnor-  mality wherein superficial and deep veins do not have any  valves. It is also called valveless syndrome (Key Box 10.2).  Primary varicosity can also be genetic. Some patients inherit  abnormalities in the FOXC2 gene. These factors, in addition  to prolonged standing (agriculturists, traffic police, hotel  workers), contribute to the development of varicose veins.   TO VARICOSE VEINS  Height: Tall individuals suffer more  Weight: Obesity may weaken vein wall  Occupation: Hotel workers, policemen, shopkeepers,  tailors  Side: Left is affected more than the right  Age and sex: Not very clear    e Characterised by nevus flammeus (portwine stain), venous  malformations, lymphatic malformations and soft tissue  hypertrophy of the affected limb.    e Patients can complain of pain heaviness in the limbs,  difficulty in walking due to abnormal length of the limb.    e Excision of veins, sclerotherapy, laser treatment for port-  wine stain are few methods to treat the veins.    ¢ Women are more prone for varicose veins because of the  following reasons:    — Pills (oral contraceptive pills) alter the viscosity of blood.  — Progesterones dilate vessel wall.    ¢ Congenital arteriovenous (AV) fistula increases blood  flow and increases venous pressure.    ¢ Deep vein thrombosis can occur as a result of road traffic  accidents, postoperatively, etc. This can lead to destruc-  tion of valves resulting in varicose veins.    It starts in the foot from the tributaries of dorsal venous arch,  permits reverse flow through its competent valves, ascends in  front of medial malleolus and runs along the medial side of  the leg. It then ascends in the thigh and ends at the  saphenofemoral junction (SF) by joining the femoral vein,  which is 1% inches (4 cm) below and lateral to the pubic  tubercle. It has 15 to 20 valves. Absence of valves results in  varicose veins (Key Box 10.3 and Fig. 10.1).    e As the name suggests, they are in the superficial fascia  and are often visible (saphenous means easily seen).    e The middle coat of these veins consists mostly of smooth  muscle.    e The middle coat is also thicker than that of other  veins.    e Normal blood flow is from superficial to deep system of  veins.    1. Lateral superficial femoral vein  2. Medial superficial femoral vein  3. Transverse suprapatellar vein  4. Transverse infrapatellar vein   2. Posterior arch vein lies parallel to and behind the main trunk  of long saphenous vein. It anastomoses with small venous  arches connecting the medial perforating veins.    These tributaries connect the long saphenous with short  saphenous veins (SSV). They are also called communicators.    These are the veins which connect long saphenous vein with  deep system of veins. Since they perforate deep fascia, they  are called perforators. There are 5 constant perforators in the  lower limb on the medial side.    ¢ Leg perforators: They are 3 in number. The lowest  perforator is situated below and behind the medial  malleolus. The middle perforator is 10 cm above the tip of  the medial malleolus. The upper perforator is 15 cm above  the medial malleolus.    A knowledge of perforators forms the basis of multiple  tourniquet test. Most of the perforators are provided with  valves. Weakness of these valves or damage to valves results  in varicosity.    This comprises the femoral and the popliteal veins, veins or  venae comitantes accompanying anterior tibial, posterior tibial  and peroneal arteries and valveless veins draining the calf  muscles (soleal venous sinus).    ¢ It is a high pressure system, well-supported by powerful  muscles.   ¢ They are connected to superficial veins by means of  perforators.   ¢ It is the powerful calf muscle contraction that returns the  blood to the heart.   ¢ The deep veins are also provided with valves (Fig. 10.3).    Blood is returned to the heart from the lower limbs by the   ¢ Calf muscle pump: It is the alternate contraction and  relaxation of the muscles of the leg (the major factor). The  pressure within the calf compartment rises to 200—300  mmHg during walking.   ¢ Competent valves (unidirectional) in the leg (Key Box  10.3): When these valves are absent or weak, perforator  incompetence develops resulting in varicose veins.   e Long saphenous vein : 10-14 valves  e Short saphenous vein : 1 valve    ¢ JVis-a-tergo of the circulation, that is the pressure transmitted  from the arterial tree passes the capillary bed to the venous  side. This helps in return of blood to heart at resting position.    1. Telangiectasia: It means a confluence of dilated intradermal  venules of < 1 mm in diameter.   2. Reticular veins: Dilated subdermal veins of 1—3 mm in  diameter.    Ankle flare: Dilated group of reticular (corona  phlebectasia) veins near medial malleolus.    Saphena varix: A dilated long saphenous vein near the  termination (saphenofemoral junction) in the groin  which is soft and reducible on lying down and elevation of  leg.   Atrophic blanche: Atrophic skin with pigmentation and  reticular veins—seen tn the ankle region.    Majority of the patients present with dilated veins in the  leg. They are minimal to start with and at the end of the  day they are sufficiently large because of the venous  engorgement.   Dragging pain in the leg or dull ache ts due to heaviness.  Night cramps occur due to change in the diameter of veins.  Aching pain Is relieved at night on taking rest or elevation  of limbs.    Sudden pain in the calf region with fever and oedema o  the ankle region suggests deep vein thrombosis (DVT)  Some patients with DVT may be asymptomatic.    Interestingly, pain due to varicose veins is relieved on  exercise in contrast to pain due to arterial diseases, which  get worse on exercise.    Inspection (should be done in standing position)  ¢ Dilated veins are present in the medial aspect of leg anc    the knee. Sometimes they are visible in the thigh also (see  next page for clinical classification) (Figs 10.4 to 10.10).    Single dilated varix at SF junction is called saphena varix.  It is due to saccular dilatation of the upper end of long  saphenous vein at the saphenous opening (Tables 10.1 and  Oe 2    See Table 10.2  for description  of various  grades of  chronic lower  extremity  venous disease   C Clinical signs (grade 0-6): A for asymptomatic  or S for symptomatic   A Anatomic distribution (superficial, deep or  perforator, alone or in combination)    'This classification is from International Consensus Committee on Chronic  Venous Diseases.    ¢ A localised, dilated segment of the vein, if present, is an  indication of a blow out. It signifies underlying perforator  (Fig. 10.11).    ¢ Ankle flare is a group of veins near the medial malleolus.    ¢ Complications such as ulceration, bleeding, eczema and  dermatitis may be present.    Smaller varicosities are called reticular veins. They are  usually 1-3 mm in diameter.    ¢ First palpate along the whole length of vein. Look for  tenderness. If present, it indicates thrombophlebitis.    e Vein which is thrombosed will feel as a firm/hard nodule.    Clinical classification of chronic lower extremity  venous disease (Figs 10.4 to 10.10)   4 Skin changes ascribed to venous disease (e.g. pigmenta-  tion, venous eczema, or lipodermatosclerosis)    Cough impulse test (Morrissey’s test): This test should  be done in the standing position. The examiner keeps the  finger at SF junction and asks the patient to cough. Fluid  thrill, an impulse felt by the fingers, is indicative of  ‘saphenofemoral incompetence’.    Trendelenburg test: This test is done in 2 parts (Figs 10.13   ¢ Method: The patient is asked to lie on the couch in the  supine position. The leg is elevated above the level of  heart and the vein emptied. SF junction 1s occluded with  the help of the thumb (or a tourniquet) and the patient 1s  asked to stand.    Fig. 10.15: Trendelenburg test Part Il—slow filling is seen; Figs  10.16A and B: Multiple tourniquet test (3 or 4) 5.    Trendelenburg I: Release the thumb or tourniquet  immediately. Rapid gush of blood from above  downwards indicates saphenofemoral incompetence.  Trendelenburg II: The pressure at the SF junction is  maintained without releasing the thumb or tourniquet.  The patient is then asked to stand. Slow filling of the  long saphenous is seen. It is due to perforator  incompetence (retrograde flow of blood).    3. Multiple Tourniquet test: It is done to find out exact site  of perforators (Fig. 10.16).    ¢ Method: The patient 1s asked to lie supine on the coucl  The vein 1s emptied by elevation. As the name suggest  3—5 tourniquets (multiple) can be applied. However,  more tourniquets are applied, the exact localisation  the perforators can be made out but it is not practica  There are mainly ankle, knee and thigh perforator:  Hence, four tourniquets can be applied at various level  as mentioned below.    ¢ Ist Tourniquet: At the level of saphenofemoral junctio  (SF junction).   ¢ 2nd Tourniquet: At the level of middle of the thigh, t  occlude perforator in the Hunter’s canal.    ¢ 4th Tourniquet: Palm breadth (lower third of the leg  above medial malleolus/ankle.    Inference: Appearance of veins between first and secon  tourniquets indicates incompetence of thigh perforators  between second and third indicates incompetence of kne«  perforators and below the fourth tourniquet indicate:  incompetence of ankle perforators. Most commonly, below  knee and ankle perforators are incompetent.   On releasing the tourniquets one by one from below  upwards, sudden retrograde filling of the veins occurs.    Schwartz test: It is done with the  patient in the standing position.  Place the fingers of the left hand  over a dilatedsegment of the vein  and with the right index finger tap  the vein below. A palpable  impulse suggests a superficial  column of blood in the vein and it  also suggests incompetence of the  valves in between the segment of  the vein (Fig. 10.17).    Modified Perthes’ test': It is  done to rule out deep vein  thrombosis. The patient is asked  to stand, the tourniquet is applied Fig. 10.17: Schwartz  at SF junction and he is asked to _ test (shouldbe done in  have a brisk walk. the standing position)   Inference: If the patient complains of severe pain in calf  region or if superficial veins become more prominent, it  1s an indication of deep vein thrombosis and is a contrain-  dication for surgery.    6. Fegan’s method (test): It is done to detect the site of  perforators. The patient is asked to stand. The varicosity  is marked with methylene blue and he 1s asked to lie down.  The leg is elevated to empty the vein and the vein 1s  palpated throughout its course. The defects in the deep  fascia have a circular, buttonhole consistency.   Look for inferior vena caval obstruction in the form of dilated    At the end of clinical examination, you should be ready to  answer following questions:  1. Which system is involved?  ¢ Medial veins—LSV—long saphenous vein  e Lateral veins—SSV—short saphenous vein  2. Is SF junction incompetent?  Yes—Trendelenburg I 1s positive  No—Trendelenburg I is negative  3. Is there perforator incompetence?  Yes—Trendelenburg II is positive  No—Trendelenburg II 1s negative  4. Which group of perforators are incompetent?  According to the results of multiple tourniquet test, mostly  ankle and below knee  5. Is there deep vein thrombosis?  Yes—Perthes’ test is positive  No—Perthes’ test is negative  6. Is there any abdominal mass?  Pelvic tumours  7. Any complications are present?  Eczema, dermatitis, ulcer, etc.  8. Is it unilateral or bilateral?  Use hand-held Doppler    1. Hand held Doppler is the first, minimum level  investigation to be done before treating a patient with  venous disease.  ¢ When the blood flows, the wave emits a signal — the  Doppler signal.   ¢ Biphasic signal: Gently squeeze calf muscles and the  flow can be assessed by Doppler probe in the SF  junction.   ¢ Itcan also pick up accessory long saphenous vein in the  groin.    e This investigation is carried out with the patient standing/  sitting.   e Incompetence of SFJ and saphenopopliteal junction (SPU)  can be assessed by this method—reflux.    e Gentle squeezing of calf muscles helps in detecting  saphenopopliteal incompetence.    2. Duplex ultrasound imaging: In this investigation high  resolution B-mode ultrasound imaging and Doppler  ultrasound are used (Figs 10.18 to 10.20).   Fig. 10.19: Popliteal vein thrombosis  (Courtesy: Dr Rajgopal, Head, Department of Radiology and  Imaging, KMC,Manipal)    ¢ It helps in getting images of veins, measure flow in all  lower limb veins.   ¢ Origin of venous ulcers and varicose veins can also be  assessed.    Retrograde flow in the veins can be demonstrated by  compression, release and valsalva manoeuvre.    3. Venography: Both ascending and descending veno-  graphies can be done in a case of deep vein thrombosis. It  Is an invasive procedure and risk of spreading infection  and septicaemia is present. Duplex ultrasonography has  largely replaced this investigation (rarely done now).  Varicography refers to injecting contrast into surface veins  (indicated in recurrent varicose veins).    4. Plethysmography: They are based on measurement of  volume changes in the leg. By placing a light-emitting  diode above medial malleolus and patient performing tip-  toe movements, venous recovery time can be measured.  This is called photoplethysmography (PPT).    With the advent of venous duplex scan, venography and  plethysmography are rarely done.    Elastic compression bandage and elevation of leg. It forms  the fundamental steps in treating varicose veins. This can be  advised in asymptomatic cases of varicose veins and ir  secondary varicose veins.    ¢ Usually 20-30 mmHg stockings are sufficient.  ¢ It should be worn from ankle to below knee.  ¢ It should be worn during working hours (entire day).    ¢ Should be removed while lying down but legs should be  kept elevated.    ¢ Above knee stockings should never be prescribed as  they are difficult to put on and tend to roll down.    Inner layer: \t contains roller gauze bandage impregnated  withcalamine, zinc, glycerine, etc. with graded compression.    ¢ Varicose veins are marked in the standing position. The  veins are punctured with a needle attached to a syringe  containing sclerosant agent and the patient is asked to   COMPRESSION SCLEROTHERAPY  e Endothelial cells in the vein wall are damaged  e Effective sclerosant  e Empty vein  e Elastic compression stockings    lie down. 3% sodium tetradecyl sulphate or 1-2 ml  of ethanolamine oleate or hypertonic saline 1s injected  into the column of vein. Aseptic thrombosis occurs and  when fibrosis occurs, the vein shrinks. Tight elastic  compression bandage is applied. Success of sclerotherapy  depends upon effective sclerosant, injection into an  empty vein and compression followed by exercise.    Injection sclerotherapy ts useful in varicose veins less  than 3 mm in diameter. Large veins require surgery.    ¢ It is popular in a few centres in the UK only.    ¢ Foam sclerosant C (Polidocanol) is used. It 1s prepared by  air mixing technique with sclerosant.    ¢ LSV should be compressed in the upper thigh to prevent  foam from entering into femoral vein.    ¢ Elevate the leg to prevent foam from entering into calf veins.   ¢ Escape into deep veins: Deep vein thrombosis  ¢ Entering into brain (air): Headache and stroke.   FOAM SCLEROTHERAPY  e Used to treat small and medium sized varicose veins.    e Sclerosant is mixed with air or preferably carbon dioxide  plus oxygen.   e Carbon dioxide plus oxygen is soluble in blood and hence  safe.   e Foam causes inflammation of vein wall, obliteration of  venous lumen and vein occlusion.   e Complications include cutaneous ulceration, thrombo-  phlebitis, deep vein thrombosis and allergy.   e Foam displaces blood (unlike sclerosant which mixes with  blood), requires small quantity to be in touch with vein wall.    Introduction: It is a minimally invasive, outpatient procedure  using laser fibre to ablate varicose veins. This gives excellent  cosmetic and functional results.    Pass a catheter over the wire and position  i it to 1 cm distal to SF junction    * Not ideal for smaller veins, tortuous veins and  thrombophlebitic veins    ¢ [tis also a minimally invasive technique of ablation of the  veins by using a bipolar catheter at a temperature of 85—  120°C, using a power of 2—4 W.    ¢ The position of the patient, accurate marking of the veins,  tumescent anaesthesia, are similar to that of EVLA.    ¢ However, the vein is cannulated with a 7F sheath and  catheter is not positioned within 2 cm of the SF junction.    ¢ Segment of the vein over 7 cm long can be ablated.    6. Surgery  1. Trendelenburg’s operation (Figs 10.22 to 10.27)  ¢ An inguinal incision is made, long saphenous vein  identified and the 3 tributaries are ligated. Long  saphenous vein is ligated close to the femoral vein  juxtafemoral flush ligation.   You can see in this  picture the prominent  veins are marked  (Fig. 10.23).   Fig. 10.26: One segment of the vein is isolated followed by ligation    , Femoral at both ends and if it is very tortuous, it is excised  vein    Fig. 10.24: Trendelenburg operation. Long saphenous vein should  never be ligated in the groin unless you demonstrate   5. Demonstrate femoral vein for a centimetre both above and  below the SF junction.   ¢ An incision is given on the medial side of the upper leg  and long saphenous vein 1s isolated. The lower end is  ligated and the vein incised. A long metallic stripper is    introduced within the vein and brought out from the long  saphenous vein below knee incision. A metallic head is  connected to the stripper and the vein 1s avulsed. Tight  crepe bandage is applied, inguinal incision sutured and  the limb is elevated (retrograde avulsion).   ¢ During the procedure, the perforators get avulsed from  the long saphenous vein and get thrombosed. Hence,  this procedure is called ligation with stripping operation.  It is indicated in cases of saphenofemoral incompetency.   ¢ Once stripping is done, small incisions are given where  the tributaries are marked. The vein is cleared of  subcutaneous tissue and is avulsed.    ¢ In this operation, the perforators are identified deep to  deep fascia and are ligated subfascially. This is indicated  in cases of perforator incompetence with saphenofemoral  competence. This can also be done using an endoscope.   ¢ Small port incisions are made in the skin of calf region,  deepened through the fascia.   ¢ Carbon dioxide insufflation is done. A balloon expander  may also be used to distend the subfascial plane.   ¢ The procedure is simple, quick with least morbidity and  is becoming popular.    By using ultrasound control, an ablation catheter 1s inserted  into saphenofemoral junction and slowly withdrawn.   This procedure has the advantage of lesser incidence of  thigh haematoma and pain.   Complications include deep vein thrombosis, recurrence  and damage to overlying skin.    In this method, veins are identified by subcutaneous  illumination, followed by injection of large quantities of  fluid.    Eczema and dermatitis: It occurs due to extravasation and  breakdown of RBCs in the lower leg. It gives rise to itching  which precipitates varicoseulcer. It is treated by application  of zinc oxide cream or silver sulfadiazine cream (stasis  dermatitis) (Figs 10.29 and 10.30).    changes in the lower leg associated with varicose veins such  as thickening of subcutaneous tissue, indurated wood  like feel, pigmentation, etc. It is due to increased venous  pressure resulting in capillary leakage with extravasation  of blood and fibrin into surrounding tissues. Blood is broken  down and haem is released. This combines with iron giving  rise to haemosiderin which ts responsible for pigmentation.  Classically this affects gaiter area of leg just above the  malleoli (Figs 10.30 and 10.31).    3. Haemorrhage: It occurs due to trauma or eczema. It can  be controlled by elevation of the leg and crepe bandage  application. Do not try to catch bleeders with artery forceps.    . Thrombophlebitis: It refers to inflammation of a  superficial vein. The vein is tender, hard and cord-like. The  skin 1s inflamed and pyrexia 1s usually present. It is treated  by bedrest, elevation, crepe bandage, antibiotics and anti-  inflammatory drugs (Key Box 10.10).    5. Venous ulcer: It is also called gravitational ulcer (Figs  10.32 to 10.35). Precipitating factors are venous stasis and  tissue anoxia. Deep vein thrombosis 1s also an important  cause of venous ulcer wherein valves are either destroyed  or incompetent due to damage. Sustained venous pressure  results in extravasation of cells, activation of capillary  endothelium resulting in release of free radicals. These  free radicals cause tissue destruction and ulceration.  Lipodermatosclerosis, tissue anoxia are the other factors.  Following hypotheses may explain genesis of varicose  ulcers.    ¢ The combination of capillary proliferation and  inflammation in the form of presence of macrophages Is   Fig. 10.33: Varicose vein with venous ulcer (Courtesy: Dr Maruthu  Pandyan, Government Medical College, Madurai, Tamil Nadu)    a major factor 1n the development of venous ulcer. As a  result of chronic inflammation, perivascular cuff!  develops around the capillaries. This perivascular cuff  is made up of many connective tissue proteins including  fibrin, collagen IV and fibronectin. Slowly, venous ulcer  develops.    ¢ Venous hypertension causes trapping of leucocytes.  These leucocytes become activated and release  proteolytic enzymes thus causing damage to capillary  endothelium.    Whatever be the exact mechanism of ulceration,  ambulatory venous hypertension is the only accepted  cause of ulceration (Fig. 10.34).    Fig. 10.34: Gaiter areas are those areas where skin changes  and venous stasis ulcer occurs due to ambulatory venous  hypertension and incompetent perforators. Damage to capillaries  results in protein-rich fluid and blood extravasation   Fig. 10.36: Elastic crepe bandage for varicose ulcer. Elevation of  the leg is the most important factor for healing of the ulcer    Fig. 10.35: Venous ulcer (Courtesy: Dr Maruthu Pandyan, Prof of 1. Should take rest with leg elevated above heart level  Surgery, Government Medical College, Madurai, Tamil Nadu) 2. Should receive antibiotics if infection is present  Neagunes of Wenousallecnieic Ae) 3. Should apply wound gener with suitable dressings  : 4. Should apply compression stockings  * Typically, ulcers are situated Just above the medial 5. Should do venous Doppler to rule out deep vein thrombosis  malleolus. . . or thrombophlebitis  ° The ulcers are oval, small, painless, superficial with 6. Should consider surgery only when infection is under  pigmentation all around. control  ¢ Dilated veins above the ulcer give the clue to the 7. Should ligate long or short veins if they are incompetent  diagnosis (Key Box 10.11). 8. Should ensure that all perforators are ligated   3. White cell trapping.  4. Reactive oxygen species are increased and they generate    free radicals leading to tissue damage. SHORT SAPHENOUS  5. Inhibition of growth factors leading to poor repair. VARICOSITY    They are uncommon causes of  varicosity in the leg. Short saphenous  vein originates from the lateral part  of the dorsal venous arch and ends in  the popliteal vein in the popliteal fossa  (Fig.. 10.37). Incompetence of  saphenopopliteal valve results in short  saphenous varicosity. It produces  prominent veins on the lateral aspect  of the leg with or without ulceration. ite  They are treated by ligation of the Fig. 10.37: Short  ae saphenous vein and  short saphenous vein in the popliteal 4, perforators  fossa (Table 10.3).   ¢ Active exercises should be taught to the patients  (to contract calf muscles).   6. Calcification can be seen in the walls of the vein.   7. Periostitis of the tibia can occur due to the ulcer situated   on the medial surface of the leg. Due to involvement of Ligation of saphenopopliteal junction   the periosteum, the ulcer gives rise to severe pain. ¢ Preoperative ultrasonographic marking is very essential.  8. Equinovarus deformity occurs due to improper habit of | * Vein should be ligated deep to deep fascia.    e It can be stripped up to mid-calf so as to avoid injury to  sural nerve.    e It is important to close the deep fascia to avoid unsightly  cosmetic bulge behind the knee.    It is also called phlebothrombosis. It is an acute thrombosis of  deep veins. Deep vein thrombosis is very common in the  western countries, the exact cause of which is not known.  Postoperative immobilisation, pressure on the calf muscles,  sluggish blood flow and prolonged bed rest are the various  factors which precipitate deep vein thrombosis. Commonly,  it affects venous sinuses in the soleal muscles. It is a common  starting place. It can also involve pelvic veins. Various factors  responsible for deep vein thrombosis can be remembered as  THROMBOSIS (Key Boxes 10.12 and 10.13).    Upper limb DVT is  rare. Can occur  due to trauma or  surgery.   Long saphenous vein Short saphenous vein  1. Origin Medial part of dorsal venous arch Lateral part of dorsal venous arch  2. Location Front of medial malleolus Behind lateral malleolus  3. Relation with nerve Saphenous nerve Sural nerve  4. Number of valves 15-20 valves 1 valve  5. Termination Saphenofemoral junction Saphenopopliteal junction    Clinical features  ¢ The maximum incidence occurs on the 2nd day anc  5th-6th days in the postoperative period.    ¢ First complaint is usually oedema, erythema, dilated veins  of the leg (Figs 10.38 to 10.41).    e Dull-aching or nagging pain in the calf muscles 1s present.  ¢ Superficial blebs in the skin.  ¢ Low grade fever with increased pulse rate is characteristic.    ¢ Phlegmasia alba dolens refers to white leg. It occurs when  the thrombus extends from calf region to iliofemoral vein.    ¢ Phlegmasia coerulea dolens refers to blue leg with loss of  superficial tissues of the toes.    1. Homan’s test: Forcible dorsiflexion of foot results in severe  pain in the calf region.    2. Moses’ test (Ideally should not be done for fear of  embolism): Tenderness over calf muscle on squeezing the  muscle from side to side.    1. Doppler study: It is ideal for femoral vein thrombosis or  when thrombus extends into popliteal vein. Normal  femoral vein gives a wind storm sound which completely  disappears at the end of inspiration. No sound is heard if  there is femoral thrombosis (Key Box 10.14).    dye into dorsal venous arch with an inflatable cuff both  above the ankle and above the knee. Clot appears as a  filling defect. However, venography is not routinely done  because it is expensive and invasive.    DUPLEX SCANNING IN DEEP VEIN  THROMBOSIS (B-MODE)  e Vein is larger than normal because of occlusion.  e Not completely compressible.  e Lacks respiratory variation.  e Does not show flow augmentation with calf compression.  e May have collateral flow.   Fig. 10.41: Grossly swollen leg, pigmentation, eczema and  secondary infection in a case of DVT   ¢ Warfarin, an oral anticoagulant is started 2—3 days before  heparin 1s withdrawn because of the slow onset time of  warfarin. Treatment with warfarin should continue for  6 to 12 months. Repeat duplex scanshould bedoneto look  for recanalisation of the veins. The dose of warfarin    Fig. 10.39: Acute DVT: Grossly swollen, oedematous leg. Skin is with prothrombin time and INR. International  red and warm with superficial blisters Normalized Ratio (INR) should be between 2.0 and 3.0.    3. Low molecular weight heparin (LMWH): It is given once  or twice a day, in the form of injection. No blood monitor-  ing is required. Incidence of bleeding 1s less with LMWH.   4. Inferior vena caval filters: They can be inserted  percutaneously via femoral vein in patients wherein lytic  therapy 1s contraindicated.   IVC filters are more commonly indicated in patients with  recurrent DVT with symptomatic pulmonary embolism.    5. Surgery is not done regularly. However, in chronic cases  venous bypass has been attempted with moderate success.  ¢ Palma operation is done in iliofemoral thrombosis  wherein common femoral vein below the block 1s  anastomosed to the opposite femoral vein using long  saphenous, from the opposite side  ¢ May-Husni operation wherein popliteal vein is  connected to long saphenous vein above.    Observe the differences  between acute DVT and  chronic DVT. Such swollen  legs have been mistaken for    varicosity in a case of chronic DVT 3. Secondary varicosity and nonhealing ulcer.    e Low risk : Young patients  Minor illness  Operation < 30 minutes  e Moderate : > 40 years  risk Debilitating illness  Major surgery  e High risk : > 50 years, medical conditions—MI,   ¢ Low dose heparin 5,000 units subcutaneous, 2 hours before  surgery and 24 hours after surgery, and then every 12 hours  for 5 days is given, during major surgeries such as  cholecystectomy, abdominoperineal resection, etc.   ¢ Intermittent pneumatic compression of the calf  throughout the operation, maintains the blood flow in the  lower limbs. Inflation pressure is around 30-50 mmHg.   ¢ Dextran 40 inhibits sludging of red blood cells and platelet  aggregation.   ¢ Important causes of recurrence 1s failure to ligate the LSV  at SF junction, failure to ligate tributaries at SF junction,  and a possible accessory long saphenous vein (Fig. 10.42).   ¢ Re-surgery can be done. However, wound infection, woum  gaping, lymph leak are more common.    Few premenopausal patients complain of dul] aching/severs  pain in the pelvis. Clinical examination may reveal tendernes:  in the hypogastrium. A few salient features given below car  be remembered by mnemonic—PELVIC  P Premenopausal patients  E Excessive micturition, excessive bleeding during  menstruation  IL. Leg varicosity in atypical sites such as in the thigh  V_ Varicosity of ovary, vulva, pelvic veins cause for pelvic  pain  { Increased on standing  C_ Chronic pelvic pain, which is noncyclical    ¢ Ultrasound, CT scan and MRI may be necessary to rule  out pelvic pathology   ¢ ECG may show in SI Q3 T3 pattern (S wave in lead I and  Q and inverted T waves in lead III).   e New/alternate treatment of varicose veins in the form  of endolaser ablation, radiofrequency ablation have  been given.   e Recurrent varicose vein, pelvic congestion syndrome  and new signs of varicose veins in the duplex scan  have been added    . Which of the following statements is false in long  saphenous vein anatomy?   D. Posterior arch vein is not a tributary of long saphenous  vein    . Trendelenburg test is done to find out:  A. Sapheno-popliteal in competency    A. Saphenopopliteal in competency  B. Saphenofemoral incompetency  C. Deep vein thrombosis    Characteristic feature of venous ulcer in the leg is:  A. Deep painful ulcer    Plethysmography is based on measurement of which  of the following?  A. Volume changes  C. Sound changes   D. Useful in veins with less than 3 mm 1n diameter   B. Hepatoma larger than 6 cm are often indication for  treatment   D. Nerves and cardiac muscles are not stimulated during  the treatment    The skin, the outermost coat of the human body, functions as  a protective cover against various insulting agents such as  ultraviolet radiation of sunlight, excessive heat and various  chemical agents.    Hence, no wonder it 1s one of the commonest cancers in  elderly patients. However, more than 90% of skin tumours  are curable because they are diagnosed early and easily (unlike  intra-abdominal malignancies). Many of them are low grade  cancers.    ¢ Among skin cancers, about 70% are basal cell carcinomas,  20% are squamous cell carcinomas and 5% are melano-  carcinomas.    e Other rare skin cancers are sebaceous carcinomas, dermato-  fibrosarcomas, etc. In this chapter, only common malignant  skin tumours are discussed.    e Also some common skin lesions such as corn and wart are  also discussed in this chapter.   I. Chronic irritation to the skin can occur due to various  chemicals such as dyes, tar and inorganic arsenic, which  contain various carcinogens. Coal tar contains polycyclic  aromatic hydrocarbons such as benzopyrenes which are  carcinogenic-arsenic, soot, mineral oil.   2. Solar keratosis (actinic keratosis): Prolonged exposure  to sun rays can cause hyperkeratosis of the skin which 1s  called solar keratosis. Skin changes occur due to  accumulated effect of ultraviolet rays over a period of many  years. Ultravioletrays are also present in phototherapy given  in the treatment of psoriasis (PUVA therapy—Psoralen  Ultra Violet-A). Common sites: Back of hands, face, rim  of ears.  ¢ Age group: Middle age, more than 50 years.  ¢ Clinically the lesion is an irregular, firm and irritating  patch which is flat or raised, better felt than seen.   ¢ Malignancy should be suspected when the lesion  becomes indurated, when a nonhealing ulcer develops,  when the central crust is shed or when regional lymph  nodes are palpable.    4. Radiodermatitis: Increased incidence of skin cancer was  found in persons who worked in the radiology department  initially. Now, the incidence is less due to the usage of  protective gear. Radiation change in the skin may vary from  a simple erythema initially to atrophy or hyperpigmentation.  Later, this lesion changes into squamous cell carcinoma.    5. Bowen’s disease is an intraepidermal carcinoma. Chronic  solar damage, inorganic compounds and human papilloma  virus (HPV 16) are possible aetiological factors. It is rare  and occurs in middle-aged patients. It occurs on the skin of  the trunk as scaly, erythematous plaques which are often  multiple. They are brownish patches with raised margin.  Microscopically, large clear cells are found (these cells are    3. Chronic scar: Squamous cell carcinoma which develops also found in Paget’s disease of the nipple). Topical  in a scar tissue 1s called Marjolin ulcer (Fig.11.1). Burns application of 5-FU and/or imiquimod is an effective  scar is the commonest cause of Marjolin ulcer followed treatment (Figs 11.2 to 11.4).   ¢ Marjolin ulcer differs from squamous cell carcinoma by  following characteristics (Table 11.1).    7. Autosomal recessive disorders: In this group, one or more  of the DNA repair enzymes are defective or deficient. As a  result of this, sites exposed to sun are vulnerable for the  development of various skin cancers. Xeroderma    us) Comparison of Marjolin ulcer and squamous cell carcinoma  Marjolin ulcer Squamous cell carcinoma  Grows very slowly because of scar tissue Grows slowly  It is painless as scar does not contain nerves It can be painful if it infiltrates the nerve fibres    Lymphatic metastasis does not occur because lymphatics are | Lymphatic metastasis is the chief method of spread  destroyed or occluded    Surgery cures the disease, radiotherapy is not very useful Both surgery and radiotherapy are used    Fig. 11.3: Albinism with squamous cell carcinoma of the right elbow  region—patient also has photophobia (Courtesy: Dr Satish  Deshmukh and Dr Murtaza Akhtar, NKP Salve Medical College  and Research Center, Nagpur)   ¢ Previous diagnosis of cutaneous carcinoma increases the  risk to 35% at 3 years and 50% at 5 years.    It is the most common malignant skin tumour. It arises from  basal cell of the pilosebaceous adnexa and occurs only on the  skin. Generally, it 1s a slow-growing neoplasm which can  present as an ulcer of many years duration. Metastasis and    death from this disease ts extremely rare. In some cases, it  can present as locally penetrating, ulcerative and destructive  lesion.    Majority of the lesions are found  on the face above a line from lobule  of the ear to the angle of mouth.    e Inner canthus of the eye  ¢ Outer canthus of the eye  ¢ Eyelids    Basal cell carcinoma cannot occur in the mucosal  surfaces which do not have pilosebaceous adnexa, e.g.  cervix, lips, tongue.    ¢ Ultraviolet rays: Basal cell carcinoma is common in  Australia! and New Zealand because of ultraviolet rays.   e Fair skin is vulnerable for the development of basal cell  carcinoma.    'This may be the reason why Australian and New Zealand cricket players apply some protective cream to the potentially risky sites while playing   Fig. 11.6: Basal cell carcinoma Fig. 11.7: Basal cell carcinoma  involving the right ear    A brother and sister with xeroderma pigmentosum developed 33  and 26 skin malignancies respectively which included basal cell  Carcinoma, squamous cell carcinoma and malignant melanoma,  since the age of 5 till 22 years. Eventually the boy died at 22 years  of age (Fig. 11.6). This is the sad part of this distressing and  frustrating disease. | have not seen this girl since 12 years now    Arsenic, once used in skin ointments, also increases the  risk of basal cell carcinoma.    The most commonclinical presentation is an ulcer that never  heals (Fig. 11.9). Sometimes healing takes place with  scabbing and later it breaks down and forms ulcer again.  The ulcer has a raised and beaded edge, may be indurated  and bleeds on touch. The base can be subcutaneous fat or  deeper structures like muscle or bone depending upon  invasion.   When the /esion is big, it is called rodent ulcer (Figs 11.10  and 11.11).   Fig. 11.10: Pigmented large destructive  BCC (Courtesy: Dr Vidyadhar Kinhal,  HOD, Surgery, VIMS, Bellary, Karnataka) slough    Scabbing occurs only in benign ulcers. Basal cell  carcinoma ts the only malignant ulcer which shows  scabbing.    It can also present as a painless, firm, nodule, which 1s  pigmented with fine blood vessels on its surface (Key Box  11.1).   Fig. 11.11: Lateral view of the same patient  showing elevated edge, few areas of scab and   ¢ Rarely, it can be nodulocystic variety which does not show  fluctuation.   ¢ Field fire rodent ulcer is a rapidly growing rodent ulcer  with destruction and disfigurement of the facial skin. It has  an advancing edge with healed central scar.   ¢ More aggressive infiltrative form is Morpheaform and  more common form is nodulo-ulcerative form.    ¢ Keratoacanthoma: It occurs only in the face. The edge  may be raised with ulceration, thus resembling basal cell  carcinoma.   ¢ Malignant melanoma: Pigmented basal cell carcinoma  may be mistaken for malignant melanoma.    e It spreads by local invasion. Even though it is slow-  growing, it slowly penetrates deep inside, destroys the  underlying tissues such as bone, cartilage or even eyeball.  Hence, the name rodent ulcer. It does not spread by  lymphatics because the large size of tumour emboli. Blood  spread is extremely rare.   e With peripheral palisade layer of columnar cells. Cell nests,  keratinisation and mitotic figures are absent.    Fig. 11.12: Palisading islands of basaloid cells seen in BCC  (Courtesy. Dr Laxmi Rao, HOD, Pathology, KMC, Manipal)   T1 ~=Tumour < 2 cm in greatest dimension with less than 2  high-risk features   T2 Tumour > 2 cmin greatest dimension (or) tumour of any  size with 2 or more high-risk features.   N2 Metastasis in a single ipsilateral lymph node > 3 cm but  <6 cm; or B/L or contralateral lymph nodes none > 6 cm  in greatest dimension   N3 Metastasis to lymph node > 6 cm in greatest dimension    Wedge biopsy from the edge of the ulcer. The edge is selected  because of the following reasons:  ¢ Edge is the growing part, malignant cells are numerous  ¢ Centre has slough or scab which may not reveal  malignancy  ¢ Comparison with normal skin is possible.    ¢ Basal cell carcinoma responds well to radiation. Surgical  excision also cures the disease.   ¢ Surgery is the first line of treatment for basal cell carcinoma.   * Very close to the eye, adherent to the cartilage or bone   Wide excision is done in all cases: This means excision of  the growth with at least 3-4 mm of healthy margin all around  including at the base. The resulting defect is closed by:   a. Primary suturing of the defect if the lesion is small  (Figs 11.18 to 11.20)   b. Skin grafting if defect is big as in neck or dorsum of  hand   c. Rotation flaps as in face for better cosmetic effect  (Figs 11.13 to 11.16).   Mohs’ micrographic surgery: It is a special surgical  technique which involves excision of skin cancer under  microscopic control. It minimises recurrence and maximises  conservation of surrounding normal tissue.    The technique offers complete evaluation of the lateral and  deep margins of the tumour excision.    Better cosmetic results since minimal amount of normal tissue  is removed.    II. Radiation is indicated in elderly patients who have an  extensive lesion which requires a complicated plastic  reconstruction. Dosage: 4000-6000 cGy units. Radiation  chondritis and osteitis are the complications (see Fig. 11.17  for summary of the treatment of basal cell carcinoma).    III. Other forms of treatment for basal cell carcinoma  ¢ Small and superficial: Curettage with electrodesiccation    (Courtesy: Dr Balakrishna Shetty, Professor of Surgery, KS Hegde Medical Academy, Deralakatte, Mangalore, Karnataka)   e Useful in superficial BCC that are confined to epidermis  and papillary dermis.   e Indication in high risk BOC—large (> 2 cm), located near  eye, nose, ear.    2. Skin graft  ¢ Skin graft does not look like normal skin. However, it  provides a good skin cover  ¢ Cannot be used across a joint  ¢ When aesthetic result is important.    ¢ Cortical bone without periosteum  ¢ Cartilage without perichondrium  ¢ Tendon without paratenon    ¢ Rotation flap: \t 1s a semicircular flap of skin and  subcutaneous tissues which rotates about a pivot point  into the defect to be closed. Small triangle of skin can  be removed to facilitate rotation.    ¢ Advancement flap: Defect is closed by stretching the  skin. It can be single, double, or V-Y advancement flap.   ¢ Transposition flap: Bilobed and rhomboid flap are few  examples. A rectangular piece of skin and subcutaneous  tissue 1s rotated (details below).    1. Healing by secondary intention  Simplest method: Suitable only if defect is < 1 cm,  superficial defects and located in cosmetically  inconspicuous sites.  Disadvantage: Not always cosmetically acceptable, might  result in contour irregularity, distortion of surrounding  structure and unstable coverage.    ¢ Possible only if excessive tension and distortion can be  avoided.    3. Skin grafts  ¢ Cosmetically acceptable but does not give colour and  contour match.    ¢ Preferred if patient has high risk of local recurrence such  as malignant melanoma.    6. Free flap  Radial artery based free forearm flap, fibular free flap    Advancement flaps  ¢ \V-Y flap  ¢ Good for defects on medial cheek and ala of nose.  A. V-shaped incision is made and then advanced to cover  the defect and later sutured in a ‘Y’ shape.    2. Bilobed flap: Banner flap uses a single lobe. Instead, in  this flap, two lobes are created and then rotated.   ¢ Used when defect is large enough not to be closed with  banner flap.    3. Rhomboid flap: Geometric modification of Banner’s  flap. It can be used for medium-sized defects (details in  page 149).   ¢ Causes minimal loss of function in lower lip.  Modification of Abbe flap like reverse Abbe flap can be  used for lower lip defects also.    ¢ Rotates the upper lip to lower lip  ¢ Disadvantage: Produces a round commissure and loss  of normal taper of vermilion.    ¢ Medial advancement of cheek tissue to create a new  lower lip.   It is the second common malignant skin tumour after basal  cell carcinoma. It arises from prickle cell layer of the  Malpighian layer of the skin. It usually affects elderly males.  All the premalignant conditions listed earlier apply to this  condition. It can also occur de novo in the skin. Basosquamous  carcinoma is the term applied when squamous cell carcinoma  arises in a pre-existing basal cell carcinoma. It is interesting  to note that a variety of names have been given to squamous  cell carcinoma when it occurs in different places. They are  given in Table 11.2.   Kangri is the name given to the pot containing hot charcoal  which is applied to the abdominal wall because of excessive  cold in Kashmir (India)   Seen in chimney sweepers due to prolonged irritation by  chemicals such as tar or pitch in    ¢ Most lesions are preceded by actinic keratosis. Such lesions  grow slowly and are locally invasive without metastasis. If  not from keratosis, lesions are more aggressive and  metastatic.   ¢ Typically, it is an ulcerative or cauliflower-like lesion  (Fig. 11.21).   ¢ The base is indurated and it may be subcutaneous tissue,  muscle or bone.   ¢ The floor contains cancerous tissue which looks like  granulation tissue. It is pale, friable, bleeds easily on touch  (Figs 11.23 to 11.27).   ¢ Mobility is usually restricted due to infiltration of  underlying structures. In very early cases, ulcer can be  moved along with skin over the underlying structures.   ¢ Regional lymph nodes such as inguinal lymph nodes (both  vertical and horizontal groups) can get enlarged when  Squamous cell carcinoma affects lower limb or abdominal  wall. Hard lymph nodes are suggestive of secondaries.    1. Local spread occurs by infiltration into the surrounding  tissues. Depending upon the site, various structures can be  involved. Some examples are:  ¢ Tendon involvement in the dorsum of the hand.  ¢ Muscle involvement in the abdominal wall.  ¢ Bone involvement, e.g. tibia in carcinoma developing 1n    2. Lymphatic spread is the chief method of spread even  though it occurs relatively late. Regional nodes are involved  first.  ¢ Nodes which are soft to firm and tender are due to  secondary infection.   ¢ Nodes which are hard, nontender, with or without fixity  are due to secondary deposits.    ¢ Lined by stratified squamous epithelium as in oral cavity and  oesophagus   ¢ Lined by columnar epithelium, wherein squamous metaplasia  occurs, such as gall bladder, bronchus    * A wedge biopsy from the edge of the ulcer or growth 1s  taken. However, in proliferative lesions punch biopsy can  also be taken.   * Microscopic picture: Eighty per cent of these cancers are  well differentiated (Key Box 11.5). It 1s characterised by  central structureless mass of keratin surrounded by normal  looking squamous cells which are arranged in concentric  manner like onion skin. This whole appearance is called  epithelial pearl or cell nest (Fig. 11.28). In 20% of cases,  cells are undifferentiated with numerous mitoses, without  keratinisation.    |. Well differentiated 75% keratin pearls  Il. Moderately differentiated 50% keratin pearls  Ill. Poorly differentiated 25% keratin pearls  IV. Anaplastic < 25% keratin pearls   Fig. 11.26: A large epithelioma of the scalp—biopsy proved  Squamous cell carcinoma Fig. 11.27: Bowen's disease with squamous cell carcinoma   Fig. 11.28: Classical keratin pearls—well-differentiated squamous  cell carcinoma (Courtesy: Dr. Laxmi Rao, Head, Department of  Pathology, KMC, Manipal)    Treatment can be classified as treatment of the primary and  treatment of the secondaries.    ¢ [tinvolves removal of growth along with | cm of normal  healthy tissue from the palpable indurated edge of the  tumour, in high-risk cases.   ¢ When the lesion has involved deeper tissues such as  muscles, cartilage or bone    Reconstruction: After wide excision, the defect can be closed  primarily, with split skin graft or with a flap to reconstruct the    Fig. 11.30: Wide excision followed by  skin grafting on 3rd year follow-up    part depending upon the extent of resection. If the growth 1  fixed to tibia, below knee amputation is the treatment.   |. Patients unable or unwilling to undergo surgical treatmen  for primary lesion    |. Thirty to forty per cent of the enlarged regional nodes are  due to secondary infection. Once the primary is treated anc  antibiotics are given, lymph nodes may regress. In such  cases, ‘wait and watch policy’ is observed.   2. If lymph nodes do not regress or are hard and mobile, FNAC  can be done to confirm the diagnosis followed by radical  block dissection. Thus, squamous cell carcinoma of the leg  requires inguinal block dissection.   3. If lymph nodes are hard and fixed to the femoral vessels,  palliative radiotherapy is given. Even in advanced fungating  lesions, the response rate to radiotherapy is reasonably good.    ¢ The superficial group of nodes consisting of a horizontal  chain which lies below inguinal ligament and a vertical  chain which lies along upper 5—6 cm of long saphenous  vein. These two groups of nodes form the letter T.   ¢ The deep glands are located alongside the proximal end of  the femoral vein, and one lying within femoral canal.   ¢ Fat, fascia, lymphatics are cleared from 2 cm above the  inguinal ligament up to 2 cm below saphenofemoral  junction. The medial clearance is important up to femoral  canal. 8-10 cm long saphenous vein near its termination 1s  removed to facilitate lymph node clearance.    e The saphenous veincan be preserved, nodal tissue is removed  from the vessel circumferentially; otherwise, it is sacrificed.    Fixity to muscle, veins, fascia and bones is nota  contraindication for block dissection. Carotid and  femoral artery infiltration makes it inoperable.   Fig. 11.31: Large penetrating baso-squamous cell carcinoma with  destruction of the left ear and infiltrating mastoid process and  external ear   ¢ Femoral blow-out > Sartorius muscle slide to cover  femoral vessels at the end of surgery    Wide excision  followed by  reconstruction  (a) Split skin grafting  (b) Flap  reconstruction    e Invasion: Deeper the lesions or SCC less than 2 mm,  metastasis is unlikely. If it is greater than 6 mm—15% SCC  may have metastasised    A patient who had undergone wide excision and SSG  for a growth behind the knee, presented with oedema  of the leg. The MBBS student could not explain oedema.  He said the cause is nutritional. Groin had 3-4 hard  nodes.    These are also called pigmented naevi which are composed of  modified melanocytes derived from the neural crest. All naevi  have excess melanin pigmentation because of which they are  tan brown or black in colour. They are located in the basal  layer of the epidermis. They are benign. They are of following    1. Junctional naevus: Located within the epidermis at  the dermoepidermal junction. They are common in  children. They appear as tan brown to black macules.  They are smooth, flat and hairless moles. As they enlarge,  they become slightly raised and can evolve into an  intradermal or a compound naevus. It has no malignant  potential.    Junctional naevus commonly occurs on the palm, sole,  digits and genitalia.    2. Compound naevus: As the mole enlarges, naeval cells  also appear in the dermis along the intraepidermal cells.  Such moles are described as compound naevi. These are  found usually in adolescents and are usually benign  (Fig. 11.34).    3. Intradermal naevus: It is the most common mole in adults.  Because of its deep seated nature, it appears blue. Hence,  the name blue naevus. It is seen on the scalp and face. It  contains hair and does not become malignant.    4. Spitz naevus: Commonly occur on face and legs. Grows  rapidly initially and then remains static. It appears as reddish  brown nodule—occasionally deeply pigmented.    5. Spindle cell naevus: Occur in women. Common site ts the  thigh. It has a malignant potential.    6. Congenital pigmented naevus which is present at birth,  has a greater potential for malignant change. It can  involve extensive areas of the skin (Giant).    ¢ It is an example of hamartoma of naevomelanocytes.  Involves extensive areas of the skin.    e Naevus cells are present not only in epidermis but also  in the subdermal fat and muscle    ¢ Risk of malignant melanoma is about 10%  ¢ Malignant melanoma tends to be axial    7. Dysplastic naevi are different from acquired naevi in the  following ways:   It is a malignant tumour arising from pigment-forming cells  (melanoblasts) which are derived from the neural crest.  Melanoblasts and melanocytes convert dihydroxyphenyl-    It is a potentially curable tumour in early stages. If left  untreated or if not treated properly, it disseminates rapidly,  showers the body with tumour emboli and offers a very painful  death.   INTERESTING ‘MOST’ FOR y  MALIGNANT MELANOMA  Most benign form of melanoma is lentigomaligna  melanoma.  Most common form of melanoma is superficial spreading  melanoma.  Most malignant type of melanoma is nodular melanoma.  Most reliable independent prognostic indicator in malignant  melanoma is tumour thickness based on Breslow  classification.  Most of them (majority) arise from pre-existing moles.  Most of the melanoma are pigmented. However,  pigmentation is not mandatory for diagnosis of melanoma.   Remaining cases occur in upper extremities, genitalia,  choroid of the eye!.    people. There is a linear correlation between intensity of  exposure to sunlight and malignant melanoma in white-  skinned people’. White-skinned people who live close to   Early childhood burns and eyes blue, red hair  Naevus: GCPN and total number > 20 naevi  Tendency to freckle    the equator have increased tendency of developing  malignant melanoma. The highest incidence is found in  Queensland (Australia). For the same reasons, malignant  melanoma is common in the United Kingdom, North  America and Australia.    females. The higher incidence of the disease is found during  reproductive age period. Even though oestrogen and  progesterone receptors are found 1n malignant melanoma  in some patients, their true role is not yet established. Leg  is the commonest site in females.    In general, the incidence of melanoma ts higher in men  than in women. The incidence of melanoma is 1.7 times  higher for women than men before 39 years of age. After  70 years of age, the incidence of melanoma ts 2.2 fold higher  for men than women.   ¢ Multiple, large > 5 cm, atypical dysplastic nevus in areas  covered by clothing.    patients with familial dysplastic naevus syndrome.  The disease 1s also common in individuals with Celtic race  who give family history of malignant melanoma (3—5%).    arise in a pre-existing mole, remaining 1/3rd arise de novo  in the normal skin. Malignant change occurs in the  junctional or compound naevus. Malignancy should be  suspected when following changes occur in a mole (Key  Box 11.10).    7. Increased incidence of malignant melanoma has been  found in patients with renal transplantation and leukaemia  as a result of immunosuppression.    Microscopic picture: Anaplastic, pigment laden melanocytes  confined to epidermis. The cells which have vacuolated  cytoplasm (Paget’s cells resembling those seen in Paget’s  disease of the breast) are found. Cells also invade the dermis.  Along with pigment-laden macrophages, dermal infiltration  of lymphocytes! may be present. Rarely, anaplastic  melanocytes do not form pigment (amelanotic melanoma).    All melanomas (except nodular) show radial growth  initially in the form of intraepidermal growth. However,  nodular melanoma has vertical growth phase thus involving  dermis leading to nodule formation. This has poor prognosis.   Level 3. Tumour reaches the interface between papillary  dermis and reticular dermis    Breslow described staging depending upon the maximum  thickness at the centre of the lesion.    was an increase in the size and oozing from the lesion    Fig. 11.40: Malignant melanoma with malignant cells showing dark  melanin pigment inside. Second picture is a close-up view  (Courtesy: Dr Laxmi Rao, HOD, Pathology, KMC, Manipal)    Name Nature Behaviour  Lentigo malignant melanoma Arises from Hutchinson’s melanotic freckle Least common (5%)   Occurs in old people on face and temporal region Least malignant  Superficial spreading Any part, more 1n the trunk Most common (70%)  Nodular Any part, more in the leg Most malignant, invasive (15-30%)  Acral lentiginous Located on the palm, sole, digits Presents late, aggressive (4-8%)  Amelanotic Difficult to diagnose Rare, nonpigmented (5%)   Stage II: 0.76 to 1.5 mm ACRAL LENTIGINOUS (Fig. 11.43)  Stage IIT: 1.51 to 3.0 mm ¢ Least common subtype and very aggressive  Stage IV: More than 3 mm ¢ Popularly called hand and foot melanoma    e This subtype occurs in dark-skinned people (21—22%).  e In subungual region—blue black discolouration of  ma posterior nail fold (most common on great toe or thumb).  Clinical features e Pigmentation in proximal or lateral nail folds (Hutchinson's  ¢ Malignant melanoma can present as changes in the pre- sign)   existing mole which are already described (Key Box 11.11).  ¢ The patient can present as a nonhealing ulcer of the sole of AMELANOTIC MELANOMA (Fig. 11.44)    ¢ Typically, the ulcer is pigmented (Fig. 11.41). In 10% In all cases of suspected melanomatous skin lesions  of patients, pigment is absent. They are called amelanotic examine axillae, scalp, soles, genitalia, oral cavity and  melanoma. interdigital webs also.   The lesion moves with the skin and is usually not fixed  to underlying structures.    ¢ Satellite nodules (within 5 cm of the primary) may be —- _Tymour Node  found surrounding the lesion which are due to spread TO No tumour NO—no nodes  through intradermal lymphatics (Fig. 11.47). Such Tis in situ tumour N1a—one node  patients willhave greatly enlarged, firm, nontender nodes. T 1a < 1mm, level Ill, level III micrometastasis  ¢ In-transit lesion—disease found in the dermis or no ulceration N1b—two nodes  subcutaneous tissue more than 2 cm away from the T 1b < 1mm, level IV macrometastasis  primary melanoma but before the regional lymphnode __ with ulceration N2a—2 or 3 nodes  basin (Figs 11.45, 11.46 and 11.48) T2a 1-2 mm no ulceration micrometastasis  See Key Box 11.12 for acral lentiginous variety. T2b 1—2 mm with ulceration N2b—2 or 3 nodes  macrometastasis  T3a 2—4 mm No ulceration  a aC a a oe ee ee en ee ae a re i ir i T3b 2—4 mm with ulceration N2c—no nodes but satellite  : or in-transit lesions  et de esh Lahey T4a > 4mm no ulceration  N3—4 more nodes; nodes  ABCDE OF EVALUATING T4b > 4mm with ulceration —_—_ with satellite or in-transits,  A CHANGING MOLE M—Metastasis matted nodes  A Asymmetry: One half does not match the other MO—no blood spread  B Border irregular: Ragged or blurred M1a—skin, subcutaneous tissue, distant node—normal LDH  C Colour variation: Tan, black, brown M1b—lung spread—normal LDH  D Diameter: > 6mm M1c—other viscera or distant spread and increase in LDH with   Fig. 11.46: Same patient  as in Fig. 11.45 presented  to the hospital with in-transit  deposits    Fig. 11.45: Observe the foot,  little toe has been amputated  five years back for malignant  melanoma   Stage 0 Tis NO Mo  Stage IA Tla NO Mo  Stage IB Tib NO Mo   T4a No Mo  Stage IIC T4b NO Mo  Stage III Any T >Nt1 MO  Stage IV Any T Any N M1    *Clinical staging includes microstaging of the primary melanoma and  clinical/radiologic evaluation for metastases. By convention, it should  be used after complete excision of the primary melanoma with clinical  assessment for regional and distant metastases    Fig. 11.47: Malignant melanoma operated—recurrence after  8 months with satellite nodule. It is important that the first surgery  should be the best surgery. Every attempt should be made to do a  three-dimensional excision    Fig. 11.48: Whole leg is studded with tumour. In-transit deposits  from malignant melanoma of the foot   . Satellite nodules and in-transit deposits are grouped into  stage III disease.    * Pigmented basal cell carcinoma  * Histiocytoma (sclerosing angioma)  ¢ Naevus, Kaposi’s sarcoma, cavernous haemangioma.    1. Local spread occurs mainly by continuity and contiguity.  Satellite nodules are due to local and lymphatic spread,  situated within 5 cm of the primary lesion. Malignant  melanoma rarely infiltrates the deep fascia unless and until  a ‘blunder biopsy’ is done. Inadequate local excision can  result in local recurrence later. Hence, initial surgery  Should attempt at cure.   2. Lymphatic spread is the principal mode of spread.  Regional nodes get involved very early in malignant mela-  noma, altering the prognosis. Thus, nodes can get enlarged  to a large extent even when the lesion looks innocent. Spread  occurs both by permeation and embolisation. Permeation  produces satellite nodules and in-transit nodules which  develop between primary and secondaries. Embolisation  occurs rapidly and early producing massive regional nodes  (Key Boxes 11.13 and 11.14).    Metastasis in lung  Metastasis in liver  Metastasis in brain  Metastasis in bone  Metastasis in bowel   e It is the single-most important prognostic index and it  presents stage Ill disease.   e Number of nodes is more important than size of nodes.   e Lymph node metastasis proceeds as an orderly process.  Thus, evolved the concept of the first node to get  involved—sentinel node (SN).   e Elective lymphadenectomy is not indicated in thin  melanoma (1 mm)   e In thick melanoma, do a sentinel node biopsy. If positive,  proceed to complete lymphadenectomy (block dissection).    In-transit metastasis appear in the skin as intracutaneous  metastasis. They are thought to be due to melanoma cells  trapped in lymphatic vessels.    3. Blood spread occurs relatively early and it causes  secondaries in liver, lung, brain and bones producing  miserable, pathetic situations. They are summarised in  Table 11.5.   A 55-year-old lady presented to the hospital with enlarged  inguinal lymph nodes. Clinical examination of the leg  revealed nodules in the leg with firm to hard enlarged nodes  in the inguinal region. On careful observation, the little toe  was missing. On questioning, the patient admitted that the  toe had been amputated elsewhere five years back for a  painless blackish lesion (Figs 11.45 and 11.46).    ¢ There is no specific investigation except an excision biopsy  of the lesion. Excision with | cm of the margin 1s all that 1s  required. Incisional biopsy is avoided because of the  following reasons:    — It may injure the deep fascia and it may open up a new  plane of spread.   — It does not allow the pathologist to perform a detailed  histological examination (done tn large lesions).    ¢ Nonspecific investigations to look for metastasis are:  — Chest X-ray—cannonball secondary  — CT scan ts better, however, it 1s costly (Fig. 11.49)  — Ultrasound abdomen—secondaries tn liver  — X-ray of involved bone—osteolytic lesions  ¢ FNAC of the regional lymph nodes  — Fine needle aspiration of groin nodes is helpful in  detecting the spread and to stage the disease (Figs 11.50  and 11.51).  e A large ulcerated lesion can be subjected to histopathology  by punch biopsy or even incision biopsy.  ¢ Serum LDH levels increase indicate metastatic disease.    Fig. 11.49: Lung metastasis in CT of the chest. It was not visible  in X-ray chest   Fig. 11.50: Hugely enlarged inguinal lymph nodes—the nodes  can be firm to hard. Sometimes, they are soft due to degeneration    Surgery ts the main modality of treatment available for  malignant melanoma. All other modalities of treatment  are only palliative and supportive.    1. Excision biopsy-wide excision: Asmall lesion of | mmcan  be excised even under local anaesthesia with | cm of healthy  margin around (narrow excision). Defect can be closed by  primary suturing. While excising the tumour, it is better not  to handle the tumour. It is possible to remove the tumour by  strictly adhering to the principle of “No touch’ technique  (Figs 11.52 and 11.53). Wide excision 1s based on principle—  centritugal spread of melanoma.    * Desmoplastic melanoma: Has high rate of recurrence,  hence wide margin of excision Is required.    2. Clarke’s level II lesions are managed by a wider excision  along with 2 cm of healthy margin around. Resulting defect  is closed by split skin grafting (Fig. 11.54).   3. Subungual malignant melanoma is treated by amputation  of the digit.   4. Malignant melanoma of the choroid has good prognosis.  Choroid does not have lymphatic drainage. However,  metastasis can occur through haematogenous route even  after many years. It 1s treated by enucleation of the eye.    Fig. 11.54: Local recurrence after 2 years probably because of  inadequate local excision—you can see the previous skin graft    2. If lymph nodes are away, radical block dissection is done.  Example: For a lower limb malignant melanoma, inguinal - Sentinel lymph node mapping: Isosulfan blue is  nodes along with iliac nodes are removed. This 1s called injected intradermally and the node that gets stained is  Ilioinguinal block dissection. If these group of nodes are identified and sent for frozen section (Haematoxylin and  positive at frozen section, lymph node clearance should Eosin stains and immunohistochemical technique). If the  include lymph nodes of obturator vessels. This is called node is positive, regional lymphadenectomy is done    Ilio-obturator block dissection (Figs 11.56 and 11.57). even when nodes are clinically not palpable. At least    20-30% of the patients who are in Stage I will be  identified as Stage III after sentinel node biopsy. There  is a definite survival advantage in patients who  undergo sentinel node biopsy (SNB) and regional  lymphadenectomy when the nodes are not palpable.    3. If lymph nodes are enlarged, hard and fixed, palliative  radiotherapy is given.    * The aim of treating this group of patients is only to afford  a reasonable palliation. More than 50% of patients, who  have metastasis in the regional nodes are dead by the end  of one year.   ¢ Patients who have been deemed inoperable due to severe  medical co-morbidities. 5-year survival is 70% but local  recurrence is unacceptablly high   ¢ Adjuvant radiation to regional lymph node basin for  — multiple +ve lymph nodes  — | or more metastatic lymph nodes larger than 3 cm  — Extracapsular extension  — Recurrent regional disease may benefit from adjuvant  radiation.    2. Systemic chemotherapy—dacarbazine (DTIC-dmethy]  triazenyl imidazole carboxamide) is the standardagent. The  response rate 1s around 20-30%. Cisplatin, Vinblastine,  Bleomycin are also used in combination. The addition of  immunomodulators such as BCG or Levamisole to chemo-  therapy have been tried.    ¢ Temozolomide is an oral analogue of dacarbazine. It  achieves significant CNS penetration. It may decrease  incidence of cerebral metastasis.    3. Immunotherapy  ¢ Interferon alfa-2b (Key Box 11.15)  ¢ Patients with locally advanced, recurrent, nodal, in-transit    (Fig. 11.58) or satellite disease should be considered for  adjuvant high dose IFN alfa-2b.   INTERFERON-o  e It has stimulatory effect on natural killer cells (NK)  e It has anti-angiogenic activity  e It is toxic—weight loss and myelosuppression can occur  e [L,—can cause capillary leak syndrome—hypotension and  renal failure.    ¢ Monoclonal antibodies: These are directed against  antigen, expressed on the surface of melanoma cells.  These antibodies are similar to 1gG3 activate the immune  system.   ¢ Cutaneous nodules can be managed by surgery or CO,  laser excision.   ¢ Intralesional injection of BCG has caused regression of  the cutaneous nodules in some patients.    4. Hormone treatment: Antioestrogens such as tamoxifen  have been tried in the systemic disease with 15-20%  response rate.    5. Isolated limb perfusion: This is tried when there are  extensive in-transit deposits in the limb (Fig.11.59) or  recurrent disease in the limb. Melphalan is the drug of  choice. A tourniquet is applied first, femoral vein and artery  are cannulated, the blood which comes out is passed  through a pump and oxygenator, into which a high dose  of Melphalan or DTIC ts given. Input temperature is kept  at 41°C. Therapy is aimed at controlling local disease in  the limb and to give a better functional limb even in  presence of metastasis.   MATHEMATICS IN  MALIGNANT MELANOMA  e 1mm: Thin melanoma  e 1-4: Intermediate  e 4 and above: Thick melanoma  e >6mm diameter of a mole: Suspect melanoma  e §5cm within primary—satellite nodule  e 8 to 12%: Familial variety  e 20 times more common in whites  e 50 to 60%: Arise from benign naevus  e 70%: Superficial spreading melanoma   * Common sites are—trunk, flexor region of limbs. It  presents as nodular (bosselated) ulcerative lesion of many  years duration.   ¢ Treatment is by local wide excision followed by primary  closure or skin grafting.   Aetiology Chronic irritation Ultraviolet rays, fair skin Ultraviolet rays, fair skin, pre-existing mole  Site Trunk, leg, hand, oral cavity Tear cancer Head and neck, face, digits, palm and sole  Types Ulcerative or cauliflower Nodular or ulcerative Nodular or ulcerative    e This neoplasm arises from proliferating capillary vessels  and perivascular connective tissue cells.   e Multiple, purplish nodules appear in the limb, which  ulcerate and bleed. This is its characteristic feature.   e Few examples are syringoma, hidradenoma, trichoepithe-  lioma and sebaceous carcinoma.   e They have to be kept in mind as a differential diagnosis  for malignant skin tumours. Details of a few skin lesions    e Holocrine: Entire cell dies or disintegrates to liberate  secretion, e.g. sebaceous gland.   e Apocrine: Only the luminal part of the cell disintegrates,  cell regeneration takes place from the nucleus and basal  portion, e.g. mammary gland.   e Merocrine: Secretion is discharged without destruction of  the cells. Most of the glands belong to this type.    Fig. 11.61: Turban tumour (Courtesy: Dr  Sreejayan, Professor of Surgery, Calicut   * tis painless swelling in the skin with central dark brow  core. After initial rapid growth of 2--4 weeks, spontaneou  regression occurs in 24 hours. After separation of th  central core, lump diminishes in size leaving a dee|  indrawn scar.   ¢ Keratoacanthoma if associated with sebaceous carcinom:  and visceral malignancy (colon cancer) constitutes Muir.  Torre syndrome (Fig. 11.62).    It is the blanket term used to describe a tumour occupying  the whole of the scalp resembling a turban.    TURBAN TUMOUR  e Very, very rare  Types  e Multiple cylindromata  e Multiple nodular basal cell carcinoma  e Hidradenomata  e Plexiform neurofibromatosa of scalp   Treatment includes excision and reconstruction by skin  grafting or rotational flaps.    It is a popular painful lesion in the plantar surface of the  foot (sole of the foot).    It affects the plantar surface of toes and sole of the feet.  Corn develops due to intermittent pressure over a limited  area.   Basically it is a localised hyperkeratinisation of the skin  with a hard central core.   It is a cone-shaped lesion with broad surface and narrow  at deeper plane.    Diabetic patients have to be carefully explained the  consequences of a ‘mistreated’ corn. Sensations and  pulsation have to be checked in those patients.  Symptomatic corns have to be excised. Excision of a good  cone-shaped tissue is necessary for a permanent cure.  Otherwise, recurrence can occur.    A wart 1s a rough excrescence on the skin  Papillomaviruses are responsible for this   Common sites: Fingers, feet, genitalia, beard area, etc.  Veneral warts: They are also called papilloma acuminata.  They can occur in the anal region, perineum and in the  coronal sulcus of the penis. Some of the warts may regress  spontaneously. Fulguration with diathermy is the  treatment.    It is derived from neuroendocrine cells which function as  touch receptors.    Sun-affected areas such as head and neck regions are  involved probably due to ultraviolet rays.   e Treatment of basal cell carcinoma has been discussed  in more detail including CO, laser excision. Recons-  truction with various flaps have been discussed, in more  detail.   e A few lines have been added for malignant melanoma  including c-interferon.    . Which of the following statements is false about basal  cell carcinoma?   A. It starts as a nonhealing ulcer of many months duration  B. Outer canthus of the eye 1s the common site    . Following are true for basal cell carcinoma except:  A. It is the most common malignant tumour   A.Metastasis is unlikely if the lesion is more tha  10 mm   C. Melanocyte stimulating hormone is released fron  intermediate lobe of pituitary gland    Following are true for malignant melanoma except:  A. Spindle cell naevus has high malignant potential    Which one of the following treatment is recommended  in 2 cm melanoma leg with inguinal nodes?    17. Which one of the following drugs used to decrease 19.    Alpha-interferon has following actions except:  A.It has stimulatory effect on natural killer cells  B. It has anti-angiogenic activity   B. Paclitaxel is the drug of choice with a 75% effective  rate    7 C 8 D 9 C 10 A  17 G 18 & 19 C 20 D    A. Arterial haemorrhage: Bright red in colour, jets out.  Pulsation of the artery can be seen. It can be easily  controlled, as it 1s visible.   B. Venous haemorrhage: Dark red in colour. It never jets out  but oozes out. It is nonpulsatile and difficult to control  because vein gets retracted.   B. Reactionary haemorrhage: Occurs after 6—12 hours of  surgery. Hypertension in the postoperative period, violent  sneezing, coughing or retching, are the usual causes, e.g.  superior thyroid artery can bleed after thyroidectomy, if  the ligature slips. Hence, it is better to /igate it twice.   C. Secondary haemorrhage: Occurs after 5—7 days of surgery.  It is due to infection which eats away the suture material,  causing sloughing of vessel wall, e.g. bleeding after 5—7  days of surgery for haemorrhoids.    A. Acute haemorrhage: Occurs suddenly, e.g. oesophageal  variceal bleeding due to portal hypertension.    B. Chronic haemorrhage: Occurs over a period of time, e.g.  haemorrhoids/piles or chronic duodenal ulcer, tuberculous  ulcer of the 1leum, diverticular disease of the colon.   B. Internal haemorrhage/concealed haemorrhage, e.g. splenic  rupture following injury, ruptured ectopic gestation, liver  laceration following injury.    A loss of more than 30-40% blood volume results in a fall in    A patient who underwent subtotal thyroidectomy for toxic  goitre was shifted to the postoperative intensive care unit.  Within 10 minutes, the nurse came to inform the surgeon that  45 ml of blood was collected in the “Redivac” suction bottle.  The dressing was opened and there was no large collection  of blood in the surgical wound. The presence of a large  haematoma requiring drainage was thus ruled out. The blood  pressure (BP) which was previously under control had shot  up to 210/110 mmHg postoperatively, possibly due to pain.  Careful monitoring and treatment reduced the BP to 140/90  mmHg. After 24 hours, the drainage was only 100 ml. The  incision did not need re-exploration. This case illustrates  reactionary haemorrhage due to hypertension.    to haemorrhagic shock. The evolution of haemorrhagic  shock can be classified into four stages.    When blood loss is less than 750 ml (< 15% of blood  volume), it can be called mild haemorrhage.   60-70% of blood volume is present in the low-pressure  venous system (capacitance vessels). Ten per cent of the  blood volume ts present in the splanchnic circulation.  When there 1s blood loss, peripheral venoconstriction takes  place and compensates for the loss of blood volume by  shifting some blood into the central circulation. Some  amount of correction of blood volume also occurs due to  withdrawal of fluid from the interstitial spaces.   Apart from a mild tachycardia and thirst, there may be no  other symptom or sign suggesting hypovolaemia. The blood  pressure, urine output and mentation are all normal in  Class I shock.    Loss of 800-1500 ml (15-30% of blood volume) results in  moderate (Class IT) shock.    Peripheral venoconstriction may not be sufficient to main-  tain the circulation. Hence, adrenaline and noradrenaline  (catecholamines) released from the sympathoadrenal system  cause powerful vasoconstriction of both arteries and veins.    Increased secretion of ADH causes retention of water and  salt. Thirst increases.   Clinically, the patient shows a heart rate of 100-120 beats/  minute and an elevated diastolic pressure. The systolic  pressure may remain normal. Urine output 1s reduced to  about 0.5 ml/kg/h and the capillary refill 1s more than the  normal 2 seconds. Extremities may look pale and the patient  is confused and thirsty.    Loss of 1500-2000 ml (30-40% of blood volume) produces  Class III shock. All the signs and symptoms of Class II  haemorrhage get worse.    The patient’s systolic and diastolic blood pressures fall and  the heart rate increases to around 120 beats/minute. The  pulse is thready.   The respiratory rate increases to more than 20/minute. Urine  output drops to 10 to 20 ml/hour. The patient appears pale  and 1s aggressive, drowsy or confused.    A blood loss of more than 2000 ml (> 40% of blood volume)  results in Class IV shock. The peripheries are cold and  ashen.   The pulse is thready and more than |20/minute. The blood  pressures are very low or unrecordable.   The patient may be moribund.  If persistent, can damage other organs, e.g.    GIT: Mucosal ulcerations, upper GI bleeding, absorption  of bacteria and toxins, bacterial translocation and  bacteraemia    permeability, interstitial oedema, increased arteriovenous  shunting results in acute lung injury (ALI).    Hospitalisation  Care of all critically ill patients begins with A, B and C.  A: Airway, B: Breathing, and C: Circulation.    Oxygen should be administered by face mask to all patients  who are In shock but are conscious and are able to maintain  their airway.   If unconscious, endotracheal intubation and ventilation with  oxygen may be necessary.    Intravenous access: Urgent intravenous administration of  Ringer lactate to restore blood volume to normal. If there  has been massive blood loss as in Class IV shock or the  patient is anaemic, blood transfusion ts indicated. Colloids  such as gelofusine or 5% albumin may also be used. The  use of hetastarch may be associated with increased rate of  acute kidney injury and mortality, andhence better avoided.  Investigations: Blood 1s collected at the earliest opportunity  for routine investigations as well as for blood grouping and  cross-matching.   Cross-matched blood is usually given: If the haemorrhage  is life-threatening, uncross-matched, O —ve packed cells  may be transfused into the patient.   Use of inotropes and vasoconstrictors 1s not indicated as  they may harm tissue perfusion.   However, if inotropes have been started as a life-saving  measure, an attempt should be made to wean them as soon  as the volume status is corrected and the patient ts stable.    To control bleeding from nose and scalp: Packing using  roller gauze with or without adrenaline to control bleeding  from nose.   ¢ Bleeding from vein: Middle thyroid vein during thyroidec-  tomy, lumbar veins during lumbar sympathectomy can be  controlled using pressure pack for a few minutes.   ¢ Sengstaken tube is used to control bleeding from oesopha-  geal varices—internal tamponade.   ¢ Elevation of the head-end reduces venous bleeding in  thyroidectomy—anti-Trendelenburg position.   ¢ Sedation to relieve anxiety—Midazolam in titrated doses  of 1-2 mg intravenously may be given.   ¢ Too tight: Arterial thrombosis can occur which may result  in gangrene.   ¢ Too long (duration of application): Gangrene of the limb.  Hence, when a tourniquet is applied, the time of inflation  should be noted down and at the end of 45 minutes to an  hour, it has to be deflated at least for 10 minutes and  reinflated only if necessary.   ¢ Application of artery forceps (Spencer Well’s forceps) to  control bleeding from veins, arteries and capillaries.   ¢ Application of bone wax (Horsley’s wax which is bee’s  wax In almond oil) to control bleeding from cut edges of  bones.    ¢ Surgical procedure: Splenectomy for splenic rupture  hysterectomy for uncontrollable postpartum haemorrhage  laparotomy for control of bleeding from ruptured ectopi  pregnancy.   Shock is defined as an acute clinical syndrome characterise  by hypoperfusion and severe dysfunction of vital organs  There is a failure of the circulatory system to supply blood 11  sufficient quantities or under sufficient pressure necessan  for the optimal function of organs vital to survival.    ¢ Hypovolaemic shock  ¢ Cardiogenic shock  e Distributive shock  ¢ Obstructive shock    The primary problem ts a decrease in preload. The decreased  preload causes a decrease in stroke volume.    Clinical features depend on the degree of hypovolaemia.  Severe (Class III or IV) shock results in tachycardia, low blood  pressures and decreased urine output.    The peripheries are cold and the patient may be confused  or moribund (see pathophysiology of haemorrhagic shock).    ¢ The primary goal is to restore the blood volume, tissue  perfusion and oxygenation to normal as early as possible.  ¢ Replace the lost blood volume.    'In MS examination, a candidate was asked to examine a case of radial nerve palsy. The patient had an injury to the wrist 4 months back. The cut  flexor tendons had been sutured. The candidate could not correlate the radial nerve palsy to the injury at the wrist. He failed! It was a case of    ¢ Crystalloids: \f crystalloids are used to replace blood loss,  2—3 times the volume lost needs to be given. Ringer lactate  is the crystalloid of choice. Large volumes of saline infusion  can cause hyperchloraemic metabolic acidosis. 5% dextrose  is not used to expand the intravascular volume as it is  hypotonic once the dextrose is metabolised.   ¢ Colloids: When colloids are used to replace lost blood  volume, a volume equal to the lost volume may be given.   ¢ Crystalloids are preferred in the initial phase of  resuscitation. If large volume of blood 1s lost, colloids such  as gelofusine or 5% albumin may be given in order to  reduce the amount of volume to be infused.   ¢ Blood transfusion may be needed if large amount of blood  is lost (> 40% of blood volume) or if the patient is anaemic  (Hb < 8 g%).    The blood flow is reduced because of an intrinsic problem in  the heart muscle or its valves. A massive myocardial infarction  may damage the cardiac muscle so that there is not much  healthy muscle to pump blood effectively. Any damage to the  valves, especially acute may also reduce the forward cardiac  output resulting in cardiogenic shock.    ¢ The primary problem is a decrease in contractility of the  heart. The decreased contractility causes a decrease in  stroke volume.   ¢ Left ventricular pressures rise as forward cardiac output  reduces. The sympathetic nervous system 1s activated and  consequently, systemic vascular resistance increases.   ¢ Clinically, the patient presents with tachycardia, low blood  pressures and decreased urine output.   ¢ The jugular venous pulse may be raised, a S; or S, gallop  may be present.   ¢ The lung fields may show bilateral extensive crepitations  due to pulmonary oedema.   ¢ The peripheries are cold and the patient may be confused  or moribund.   ¢ Oxygenation can be improved by administering oxygen,  either by face mask or by endotracheal intubation and  ventilation as necessary.    ¢ Vasodilators such as nitroglycerine may dilate the coronary  arteries and peripheral vessels to improve tissue perfusion.  Lowering systemic vascular resistance reduces impedance  to forward cardiac output (afterload). However, the patient  must be monitored closely to avoid excessive drops in blood  pressure due to these drugs.   ¢ Intra-aortic balloon pump or ventricular assist devices  may be used to augment cardiac output.   ¢ If hypotension continues to be unresponsive, revas-  cularisation (surgical or interventional) or valve replace-  ments may be considered on an emergency basis.    In distributive shock, the afterload 1s excessively reduced  affecting circulation. Distributive shock can occur in the  following situations:    ¢ Sepsis is the response of the host to bacteraemia/  endotoxaemia.   ¢ It may be produced by gram-negative or gram-positive  bacteria, viruses, fungi or even protozoal infections.   e Severe sepsis can result in persistent hypotension despite  adequate fluid resuscitation and is called septic shock.   ¢ Local inflammation and substances elaborated from  organisms, especially endotoxin, activate neutrophils,  monocytes and tissue macrophages. This results in a  cascade of proinflammatory and anti-inflammatory  cytokines and other mediators such as IL-1, IL-8,  [L-10, tumour necrosis factor-alpha, prostaglandin FE,  endogenous corticosteroids and catecholamines.   ¢ Effects of this complex mediator cascade include cellular  chemotaxis, endothelial injury and activation of the  coagulation cascade (Key Box 12.2).    ¢ These substances produce low systemic vascular  resistance (peripheral vasodilatation) and ventricular  dysfunction resulting in persistent hypotension.   ¢ Generalised tissue hypoperfusion may persist despite  adequate fluid resuscitation and improvement in cardiac   e Inflammatory response in sepsis activates tissue factor which  in turn activates coagulation   e Lowered levels of natural anticoagulants such as protein C,  protein S and antithrombin III    output and blood pressures. This is due to abnormalities in  regional and microcirculatory blood flow. These  abnormalities may lead to cellular dysfunction, lactic  acidosis (anaerobic metabolism) and ultimately, multi-  organ failure.    ¢ Early phases of septic shock may produce evidence of  volume depletion such as dry mucous membranes and cool,  clammy skin.    e After resuscitation with fluids, however, the clinical picture  is typically more consistent with hyperdynamic shock.  This includes tachycardia, bounding pulses with a widened  pulse pressure, a hyperdynamic precordium on palpation  and warm extremities.    e Signs of possible infection include fever, localised erythema  or tenderness, consolidation on chest examination,  abdominal tenderness, guarding, rigidity and meningismus.    ¢ Signs of end-organ hypoperfusion include tachypnoea,  cyanosis, mottling of the skin, digital ischaemia, oliguria,  abdominal tenderness and altered mental status.    e Often, a definitive diagnosis cannot be made on the basis  of initial findings, on history taking and physical  examination and empirical treatment for several possible  conditions commences simultaneously.    ¢ Removal of the septic focus is an essential step and a  priority in the treatment of septic shock, e.g. resection of  gangrenous bowels, closure of perforation, appendicectomy.    e Early (preferably given in the first hour of arrival) empirical  antibiotic therapy to treat the precipitating infection.    ¢ Supportive care: Oxygenation and if necessary,  endotracheal intubation and mechanical ventilation should  be administered.    ¢ Intravenous fluids: Restoration of intravascular filling  pressures must be done using crystalloids, colloids and  blood as necessary. Crystalloids such as isotonic saline  or Ringer’s lactate may be used. Large amounts may be  required and may contribute to tissue oedema. Colloids  restore intravascular volume faster and remain longer in  the central circulation. However, they are expensive and  are more often used in patients where there is a high-risk  of pulmonary oedema due to cardiac dysfunction and thus,  may not tolerate large volume of fluids. Avoid hetastarch  as it may increase risk of renal injury and mortality. Blood  transfusions may be required to maintain the patient’s  haemoglobin levels at 8-10 g%.    ¢ Vasoactive agents such as norepinephrine to produce  vasoconstriction and raise the systemic vascular resistance  to normal may be used. Dopamine, dobutamine or adrenaline  may need to be added. Vasopressin infusion may be useful  in patients with refractory shock. All these potent drugs are  given as infusions under careful and continuous monitoring    of blood pressure as well as cardiac filling pressures (central  venous pressures).    No matter what antibiotics you use, unless you remove the  septic focus by surgical drainage of pus or resection of  gangrene, etc. patients in septic shock do not improve.   e Early and aggressive resuscitation, supportive care and close  monitoring in intensive care unit (ICU).    Occurs on exposure to an allergen the patient is sensitive to. It  may be pollen, foodstuffs, preservatives in the food or a  medication. The anaphylactic shock that occurs in the hospital  is usually due to some drug, e.g. the patient is allergic to  penicillin. Latex allergy is being increasingly recognised.  The reaction may be in the form of mild rashes with or  without bronchospasm or it may be a full blown anaphylactic  shock wherein the patient presents with rashes, generalised  oedema including laryngeal oedema, bronchospasm and  hypotension and if not treated in time, cardiac arrest.   ¢ If facilities exist, take a 10 ml sample of blood to analyse  for serum tryptase levels. If raised, they confirm  anaphylactic reaction.    Causes: High spinal cord injury, vasovagal shock  Features: Hypotension without tachycardia that can  deteriorate to produce shock and cardiac arrest.    ¢ Headache, profound weakness, fatigue, slow and lethargic  movement, joint pain.   RISK FACTORS FOR ADRENAL CRISIS  e Infection  e Trauma or surgery  e Adrenal gland or pituitary gland injury  e Premature termination of treatment with steroids such as  prednisolone or hydrocortisone.    ¢ Nausea, vomiting, abdominal pain, high fever and chills  ¢ Low blood pressure, dehydration, rapid heart rate and  respiratory rate, confusion or coma.    It can be due to cardiac tamponade or due to tension  pneumothorax.    ¢ In obstructive shock, there is impedance to either inflow or  outflow of blood into or out of the heart.    A 54-year-old lady was admitted to the casualty with low  blood pressures and dyspnoea since one day. She had a  history of fever, vomiting and diarrhoea since 3—4 days, was  treated in a local nursing home and when she got worse,  was referred to our hospital. In spite of fluid therapy,  profound hypotension persisted and within half an hour of  arrival to the casualty, she suffered a cardiopulmonary  arrest.    Her trachea was immediately intubated, cardio-  pulmonary resuscitation (CPR) given and was Shifted to the  intensive care unit for further management. She required  high doses of dopamine, adrenaline and noradrenaline to  maintain blood pressures. A blood gas analysis showed  severe metabolic acidosis (pH = 7.02, PaCO7 = 35 mmHg  and HCO; = 12 mmol/L).    Considering the history, a diagnosis of septic shock was  made when she continued to have hypotension even after  her central venous pressures were normal. Peritoneal  dialysis was done as she was in oliguric renal failure.  Haemodialysis was not possible as she was hypotensive and  on inotropes. A search for septic focus was initiated. An  ultrasound abdomen showed dilated kidney and obstructed  urinary system.    A double J stenting of the ureter, which was done to  relieve the obstruction, drained pus. Once the pus was  drained, appropriate antibiotics were given and with  continued cardiorespiratory support, she showed steady  improvement. She was gradually weaned off the ventilator  and inotropes, and was discharged from the hospital five  weeks later. At discharge, she was fully conscious, stable,  ambulant and very grateful to the medical fraternity.    This case illustrates the importance of resuscitation,  cardiorespiratory support, removal of septic focus and  antibiotics in the treatment of septic shock.    ¢ In cardiac tamponade, the pericardium ts filled with blood  and hampers venous filling as well as outflow.   ¢ The filling pressures of the left-sided and right-sided  chambers equalise.   ¢ The central venous pressure is high and the blood  pressure is low.    ¢ The patients also have pulsus paradoxus where there is 10%  decrease in systolic blood pressure with inspiration.   ¢ Relief of obstruction, drain the pericardial cavity as early  as possible.   ¢ Rupture of emphysematous bulla in a patient with chronic  obstructive pulmonary disease.   ¢ No air entry on the side of pneumothorax, hyper-resonance  to percussion    ¢ A wide (large) bore needle/cannula must be inserted into  the pleural cavity to drain the air. The needle is inserted in  the midclavicular line in the 2nd intercostal space on the  affected side. This is followed by insertion of a tube  thoracostomy.    A massive pulmonary embolus is a differential diagnosis  for obstructive shock.    Hyperbaric oxygen is administered to patients when their own  haemoglobin is unable to carry adequate oxygen or when  hyperoxia is required. Hyperbaric O, is administered using  either a monoplace (single patient) or multiplace compression  chamber.    1. Carbon monoxide poisoning: Hyperbaric O, at 2—3 atm  is given to increase the O, dissolved in plasma (5—6 ml/dl  at 3 atm, whereas it is 0.3 ml/dl at 1 atm) which is sufficient  to meet the O, requirement of the body even in the absence  of normal haemoglobin (severe CO poisoning).   2. Infections such as gas gangrene: Hyperoxia suppresses the  growth of anaerobic organisms.   5. Decompression sickness and air embolism: Hyperbaric  oxygen reduces the size of air bubbles and rapidly eliminates  nitrogen from the air bubbles.    Untreated pneumothorax: Can expand with hyperbaric O,  resulting in life-threatening tension pneumothorax.   ¢ One of the desirable requirements while treating patients  in shock includes monitoring of CVP.   ¢ CVP is a fair indicator of blood volume. Thus, in shock.  measurement of CVP is useful so as to plan proper fluid  management.   ¢ CVP is also affected by contractility of the right ventricle,  intrathoracic pressure and intrapericardial pressure.    ¢ Internal jugular vein (IJV) or subclavian vein are preferred  to gain access to central veins (Key Box 12.5).   ¢ A 16cm long IV catheter is introduced into the central vein  with the patient supine, head down and neck rotated to the  opposite side. Head down position helps in engorging the  vein. Seldinger’s technique is employed and the catheter is  advanced up to the junction of superior vena cava and right  atrium.   ¢ The patency and position of the catheter is confirmed by  lowering the saline bottle to check free flow of blood into  the connecting tube.   ¢ The tube is connected to a saline manometer. Readings of  the saline level are taken with the ‘zero reference point’ at  the midaxillary level if the patient is in supine position  or at the manubriosternal joint, if he is in the semireclining  position (45°). An electronic pressure transducer may be  used for greater accuracy.   ¢ CVP must be measured at end-expiration as it may change  with phases of respiration.    1. If CVP is low, venous return should be supplemented by  IV infusion, as in cases of hypovolaemic shock.   2. When CVP is high, further infusion of fluids may result in  pulmonary oedema.    ACCESS TO RIGHT HEART/GREAT VEINS  e Internal jugular vein  e Subclavian vein  e Median cubital vein  e External jugular vein    ¢ This is a better indicator of circulatory blood volume and  left ventricular function.   e [t is measured by a pulmonary artery balloon floatation  catheter—Swan-Ganz catheter.    Swan-Ganz catheter 1s introduced into the right atrium through  a central vein (RIJV usually). The catheter has a balloon near  its tip which is inflated with air. The catheter is advanced and  pressure tracing is monitored. Its entry into the right ventricle  and pulmonary artery is identified by changes in pressure  tracing. With further advancement, the pressure tracing  becomes flat, when the balloon gets wedged in a small branch  to give pulmonary capillary wedge pressure (PCWP). When  the balloon is deflated, the pulmonary artery pressure is  obtained. Normal values: PCWP: 8-12 mmHg. PAP: systolic  25 mmHg; PAP diastolic 10 mmHg.    Note: CVP reflects right atrial pressures only. It is low in  hypovolaemia and, high in hypervolaemia and in right  ventricular failure.    PCWP is a better indicator of left ventricular pressures  and is a preferred monitor in cardiogenic shock. PCWP is low  in hypovolaemia and high in hypervolaemia as well as in left  ventricular failure.   Note: The use of pulmonary artery catheter has declined  significantly due to increased complications and unfavourable  risk-benefit ratio. An echocardiogram 1s noninvasive, provides  a lot more information and is preferred in the treatment of  cardiogenic shock.    Administration of whole blood or its components into a patient  is often necessary for various reasons. Since the only available  source of blood is by voluntary human donation, and that  source is scarce, whole blood is separated into 1ts components,  namely packed red blood cells, fresh frozen plasma, platelets  and cryoprecipitate. Some coagulation factors may be isolated  and separately stored, e.g. Factor VIII for administration into  haemophiliacs. Packed red cells are the commonest blood  products used. Their transfusion increases the oxygen carrying  capacity of blood. Transfusion of 250 ml of packed cells raises  haemoglobin by 1 g% 1n an adult.    I. Packed red cells are used to replace acute and major blood  loss as in:  ¢ Haemorrhagic shock  ¢ Major surgery—open heart surgery, gastrectomy  e Extensive burns  I]. Packed red cells are also used to treat anaemia due to:  e Extensive burns  ¢ Chronic blood loss as in haemorrhoids, bleeding  disorders, chronic duodenal ulcer, etc.  ¢ Inadequate production as in malignancies, nutritional  anaemia   III. To replace platelets in thrombocytopaenia, fresh frozen  plasma in vitamin K deficiency unresponsive to vitamin  K replacement as in liver disease or to reverse effects of  warfarin, cryoprecipitate to replace fibrinogen in patients  with disseminated intravascular coagulation.   IV. Whole fresh blood administration is described in massive  trauma with heavy blood loss, the rationale being that the  patient rapidly loses all components of blood and so all  components need to be replaced. Thus, it makes sense to  administer whole blood rather than individual  components. However, many blood banks are reluctant  to allow whole blood transfusion.    GUIDELINES: WHEN TO TRANSFUSE  1. Blood loss > 20% of blood volume  2. Haemoglobin < 8 g/dl    3. Haemoglobin < 10 g/dl in patients with major cardiovascular  disease (e.g. ischaemic heart disease)    Packed red blood cells (PRBC): When whole blood is  centrifuged, red blood cells settle down and the platelet rich  plasma remains supernatant. The plasma is transferred to  another bag while preservative is added to red cells and the  bag is sealed. Each bag of packed cells contains approximately  250-300 ml with a red cell concentration of 70%. Red cell  transfusion 1s required for patients whose haemoglobin 1s < 7  g%, as in patients undergoing chemotherapy, major surgery,  delivery, trauma, grossly anaemic neonates and infants or in  patients with sickle cell anaemia, especially in sickle cell crisis.  A higher transfusion threshold (9-10 g%) may be used for  patients with cardiac disease. Each unit of packed cells should  increase haemoglobin by | g% and haematocrit by 3%.    The red cells may be leucodepleted (white blood cells  removed) for use in patients requiring multiple transfusions  to prevent development of antibodies to leukocytes or in those  who are known to react to leukocytes in the past.   The rate of blood or blood product administration depends  on the quantity and speed at which it is lost from the body.  Slow transfusion is indicated in patients with cardiac disease,  renal dysfunction, severe chronic anaemia and in paediatric  patients.   In semi-emergent situations, e.g. treatment of low  haemoglobin in patients awaiting surgery or delivery, each  bag of packed cells may be transfused slowly. The initial  25 mlican be given slowly to check whether the patient tolerates  it. Thereafter, it can be transfused at a rate of 3-4 ml/kg/h  (approximately 1.5 h for a bag of red cells). The red cells  must be transfused within four hours of removing from  refrigerator as bacterial growth may be promoted after that.   In emergent situations, the red cells may be transfused much  faster in order to keep up with the loss. When large amounts  are transfused in a short period (see section on massive  transfusion), effects of other components such as citrate  become significant.    Packed red blood cells must be both ABO and Rh  compatible unless the patient has life-threatening massive  bleeding, in which case O —ve packed cells may be transfused.    Platelets: The bag containing platelet rich plasma 1s again  centrifuged to express off the plasma so that the bag with the  remaining platelets can be sealed off. Each unit (50 ml) should  contain at least 5.5 x 10!® platelets (platelet concentrate) and  each unit should elevate the platelet count by 5—10,000 cells/  cu mm in a 70 kg person. Platelets may be transfused pro-  phylactically or for therapeutic purposes.   Prophylactic platelet transfusion may be done when platelet  count is < 10,000 cells/cu mm 1n oncology patients. In patients  who are at high-risk of alloimmunisation, e.g. leukaemia, the  threshold for platelet transfusion is even lower at 5000 cells/  cu mm, whereas in patients with clinical instability, the  threshold may be raised to 20,000 cells/cu mm. Patients who  need to undergo major surgery or requiring invasive procedures    such as spinal anaesthesia, liver biopsy, etc. generally nee  their platelet count elevated to > 50,000 cells/cu mm. However  patients requiring surgery in critical areas such as neurosurgen  or ophthalmic surgery will need their platelet count raised t¢  > 1,00, 000 cells/cu mm.  Therapeutic platelet transfusion is required in patients whc  are known to be thrombocytopaenic and are actively bleeding  (platelet count < 50,000 cells/cu mm). The dose of platelet:  may be calculated as follows: One unit of platelets (60 ml) fo:  every 10 kg body weight.  Platelets must be transfused within four hours o  commencement of the infusion. ABO compatibility is no  required for platelets. Filters of 170-260 uw must be used tc  transfuse platelets as with other blood components.  Fresh frozen plasma (FFP): The remaining plasma (200-  230 ml) may be stored in a frozen form (called fresh frozer  plasma) at —18°C for one year. It needs to be thawed over hal!  hour before use. Fresh frozen plasma transfusion 1s indicatec  in patients with prolonged INR > 1.5 and are bleeding o1  require surgery. FFP provides coagulation factors to those  patients who are actively bleeding and those on warfarin. Fresh  frozen plasma is administered in a dose of 10—20 ml/kg body  weight. FFPs must be ABO compatible. FFP must be  transfused as soon as possible after thawing and definitely  within 24 hours of thawing. A hanging unit must be transfused  within six hours of commencement of transfusion due to risk  of bacterial contamination.  Cryoprecipitate: The fresh frozen plasma may be further  treated to produce cryoprecipitate rich in fibrinogen. Trans-  fusion of cryoprecipitate (each bag contains 20 ml) 1s indicated  in patients whose fibrinogen levels are < 1 g%, e.g.  disseminated intravascular coagulation (DIC). The dose 1s 0.2  units/kg body weight but usually 10 units are transfused  initially and repeated as necessary.  Important points to remember before transfusion of blood  products:  ¢ Always obtain informed consent from patients or immediate  relatives (in emergent situations) prior to transfusion.  ¢ Recheck the patient’s blood group and of the donor blood,  preferably along with one other qualified person to ensure  that the correct bag is being transfused to the patient. The  patient’s hospital number and the blood bag number should  also be checked. Both people must sign on the transfusion  report.   ¢ Visually check the blood bag for any obvious abnormalities  such as very dark blood, visible clots and if present, do not  transfuse but return to blood bank. Check the date of  collection and date of expiry before transfusion. The storage  shelf life of red blood cells depends on the preservative  added. RBCs stored in ACD (acid-citrate-dextrose), CPD   mannitol) solutions have a shelf-life of 21, 28, 35 and 35    ACD (acid-citrate-dextrose) 21  CPD (citrate-phosphate-dextrose) 28  CPDA (citrate-phosphate-dextrose-adenine) 35  SAGM (saline-adenine-glucose-mannitol) 35   Whole blood transfusion is not advisable for routine use  and is also not available from blood banks. However, there  1S some evidence in the literature that fresh whole blood  transfusion may be helpful in patients with massive trauma  as all blood components are replaced simultaneously  matching the loss. However, this is difficult practically as  the blood products still need to be screened for various  antigens and infections.   Other blood products available are fibrinogen concentrate  (high risk of hepatitis), Factor VIII and Factor IX  concentrates (for use in haemophilia and Christmas disease  respectively) and Factor VII concentrate (for use in  disseminated intravascular coagulation—DIC).   ¢ Majority of cases are due to technical errors like sampling,  labelling, dispatching.   ¢ Oliguria is due to products of mismatched blood transfusion  blocking the renal tubules. It results in acute renal tabular  necrosis.    ¢ Stop the blood. Send it to blood bank and recheck.   ¢ Dhiuresis with furosemide 20-40 mg IV or injection mannitol  20% 100 ml IV to flush the kidney.   ¢ Use of 20-40 mm filter or leukocyte-depleted blood avoids  it.    ¢ Occurs due to allergy to plasma products; manifest as chills,  rigors and rashes all over.   ¢ They subside with antihistaminics such as chlorpheniramine  maleate 10 mg IV.    It 1s a rare complication, occurring within six hours of a  transfusion due to the presence of antileukocyte antibodies in  the transfused plasma causing patient’s white cells to aggregate  In the pulmonary circulation. This leads to degranulation of  leukocytes causing increased capillary permeability and  noncardiogenic pulmonary oedema. The symptoms may vary  from mild dyspnoea to full blown acute respiratory distress  syndrome. Proper supportive therapy will see that it resolves  within 24—48 hours.    d. Congestive cardiac failure (CCF)  CCF can occur if whole blood 1s transfused rapidly in  patients with chronic anaemia.    ¢ Slow transfusion, injection furosemide 20 mg IV  ¢ Packed cell transfusion is the choice in these patients.    Serum hepatitis, 4/DS, malaria, syphilis are dangerous  infectious diseases which can be transmitted from one patient  to another through blood transfusion. The danger is increased  in cases of multiple transfusions and in emergency situations.  “Prevention ts better than cure”. Hence, it is mandatory to  screen the blood for these diseases before transfusion.    Definition: Massive blood transfusion has been variously  defined—replacement of > | blood volume (or > 10 units of  packed cells) in 24 hours, half the patient’s blood volume in  six hours, > 4 RBC units in one hour with ongoing need for  transfusion, 500 ml over 5 min or even blood loss > 150 ml/  min with haemodynamic instability and need for transfusion.  Massive blood loss may occur with trauma, postpartum  haemorrhage or during major surgeries.   MASSIVE BLOOD TRANSFUSION  1. > 500 ml over 5 minutes  2. > 1/2 the patient’s blood volume in 6 hours  3. > the whole blood volume in 24 hours    Massive transfusion protocol: In patients with massive  bleeding and anticipated to require massive transfusion, the  bloodbank should be intimated to activate massive transfusion  protocol (MTP). A blood sample must be sent for cross  matching with an initial request for 4 units of O —ve red blood  cells. After this, if MTP is requested, the blood bank releases  blood products in different ‘boxes’ (Table 12.2).   The blood products are obtained box by box as necessary.  If the bleeding stops, the blood bank should be intimated  immediately so that MTP can be terminated. If bleeding  continues, box three and four are alternately requested for. In  such patients regular and half hourly measurements of acid-  base status, haemoglobin, platelets, prothrombin time (PT),  activated partial thromboplastin time (aPTT), fibrinogen and  serum calcium are required with an aim to normalise their  values.    ¢ Occurs in massive blood transfusion wherein all factors of  coagulation are used up resulting in a bleeding disorder.   ¢ Itis treated by replacement with fibrinogen (cryoprecipitate)  and other clotting factors.   This concept originated to avoid transfusion reactions which  can develop when homologous blood is used.  ¢ Here, patient’s own blood is used.   PRBC FFP  One 2 2  Two 4 4  Three 4 4  Four 4 4   ¢ Cases suchas surgery for thyrotoxicosis or abdominopelvi  resection wherein one can expect 1—2 units of blood los:  Just before surgery, 1—2 units of blood are removed an  retransfused after the procedure.    Blood which was lost during surgery is collected, mixed wit  anticoagulant solution, washed and reinfused. This can be don  provided surgery does not involve severe infection, bowe  resection or malignancy, revision total hip replacement.    These are colloidal solutions used to restore normal blooc  volume in emergency situations, e.g. polytrauma with severe  haemorrhage, massive GI bleed and shock.    Packed cells and fresh frozen plasma transfusion should  not be used to treat malnutrition.   Packed red cells  ¢ Prepared by centrifugation of whole  blood and removing plasma J  e Used to correct chronic anaemia  ¢ Economical and safe    ¢ Severe reactions with urea-linked gelatin, e.g. Haemaccel  (1:2,000) but less with succinylated gelatin, e.g.  gelofusine (1:13,000)  3. Dextran 40  ¢ Reduces viscosity and red cell sludging  ¢ May affect renal function and coagulation  4. Hydroxyethyl starch  ¢ Derived from starch  e Plasma expansion lasts for over 24 hours  ¢ Maximum dose—20 ml/kg  ¢ Large doses may interfere with coagulation  ¢ Incidence of severe reactions (1:16,000).  ¢ Use in shock is associated with increased risk of renal  injury and mortality.    This is the commonest bleeding disorder which occurs due to  X-linked genetic disorder of coagulation.   1. Haemophilia A: This results from a reduction of factor  VIII (antihaemophilic factor) and is carried by a recessive  gene.   2. Haemophilia B: This results due to deficiency of factor  [X.   ¢ A haemophilic patient’s daughters will be carriers but all  sons will be normal.   ¢ Thus, a carrier woman has 50% chance of producing  haemophiliac male or a female carrier.   ¢ The level of coagulation factor VIII in the blood may be  less than 1% of that in a normal individual.    e Large joints such as knees, elbows, ankles, wrists are  affected.   ¢ Spontaneous bleeding 1s common. It may also occur due to  minor trauma.   ¢ Repeated bleeding may result in permanent damage to the  articular surfaces resulting in deformity of the joints.    ¢ Calf muscle and psoas muscle haematomas are common  resulting in contraction and fibrosis of muscle, muscle pain  and weakness of limb.    Administration of factor VIII concentrate by intravenous  infusion is the treatment whenever there is bleeding.   It should be given twice daily since it has half-life of  12 hours.   Any major surgical procedure should be carried out only  after raising factor VIII: C levels to 100% preoperatively  and maintained above 50% until healing occurs.    Cerebral haemorrhage used to be the most common cause  of death. However, today HIV infection seems to be the  most common cause of death.   Hepatocellular carcinoma, cirrhosis due to HIV and HCV  also are the other causes (due to repeated blood transfusions).    Incidence: One in 30,000 males. The inheritance an  clinical features are identical to haemophilia A.    In vWD, there is defective platelet function as well as facto  VIII: C deficiency and both are due to deficiency o  abnormality of vWF.    Epistaxis, menorrhagia and bleeding following mino:  trauma or surgery 1s common.    Treatment is similar to mild haemophilia. Thus, DDAVF  or IV infusion of factor VIII: C or vWF surgery.    . Which of the following statements is false in  management of haemorrhagic shock?    . Which solution is ideal in the initial management of  shock?    . Which one of the following catheters does not have  balloon at the tip?  A. Swan-Ganz    Packed red cells are used to treat:  A.Chronic anaemia B. Renal failure  C. Tetanus D. Gas gangrene    Haemolytic reactions due to mismatched blood  transfusion include the following except:    Which one of the following is decreased in disseminated  intravascular coagulation?   A. First line of treatment in hypovolaemic shock  B. Does not carry risk of transfusion hepatitis    In haemorrhagic shock, when both the systolic and  diastolic blood pressures are normal, the patient is    Tension pneumothorax is one of the reasons for which  of the following types of shock?    The central venous pressure is high in the following  type of shock:    The route of choice for administration of adrenaline  in anaphylactic shock is:    The blood product of choice in bleeding patients with  a fibrinogen concentration of < 100 mg/L:    ANSWERS  B 2B Sub) 4D 5 D 6 B 7D 8 C 9 D 10 A  12D 3D 14 B 15 C 16 C ae) 18 "B 19 C 20 A  B 22 C 23 B 24 B a5 °B 26 D 27D 28 A 295 30 D    Definition  Burns is a type of coagulative necrosis caused by heat,  transferred from the source to the body.    Frostbite which occurs in cold countries 1s also a coagulative  necrosis but it is caused by extreme degrees of cold. Scald isa  burn caused by moist heat (steam). Burns never occur at  temperatures less than 44°C.    Although the exact mechanism of the postburn microvascular  changes and hypovolaemia leading to low cardiac output and  poor tissue perfusion has not been determined, the following  mechanisms have been proposed:   1. Increased capillary permeability leading to fluid and  protein leakage from the intravascular space.   2. Decreased plasma oncotic pressure due to hypo-  proteinaemia resulting from loss of protein from the  intravascular space.   3. Increased capillary hydrostatic pressure due to  vasoconstriction or partial blockage of vessels with  aggregate of cells and platelets.    4. Reduced clearance of fluid and protein from the  interstitial space by lymphatic ducts due to blockage by  platelet aggregates and fibrin clots.   6. Increase in osmotic pressure in the burned tissue leading  to further fluid accumulation.    ¢ Chemical mediators released from the site of injury are  responsible for the development of typical inflammatory  response. This results in rapid and dramatic oedema  formation.   ¢ The activated complement cascade system facilitates  liberation of various permeability factors such as  histamine, prostaglandins (PGF-1, PGF-2, PGF-2q@) and  thromboxane.    e Maximum water reabsorption by release of ADH from  posterior pituitary, as a compensatory mechanism.    e Acute tubular necrosis (ATN) can occur due to toxins.  e Injury to kidney can occur due to myoglobin.    and reduced immunoglobulin synthesis results in a  decreased concentration of all individual immuno-  globulin levels and triggering of complement cascade.    Septic shock: Due to infection by micro-organisms.  Infection commonly occurs from cross infection. GI  system, genitourinary system, respiratory system, IV  cannula site are various other portals of entry of infection  (Pig: 13.1).   Endotoxins: Lipopolysaccharides and endogenous  chemical mediators (cytokines) such as TNF-@ and  interleukins are activated resulting in systemic  inflammatory response syndrome and multiorgan failure.   AIRWAY oedema in burns  lt is due to inhalation injury  Direct injury from hot air/smoke  Inflammatory response to burns  Oedema from resuscitation fluids   ¢ Flow: Hypermetabolism, high cardiac output, hyper  glycaemia, increased heat production.    ¢ Lipolysis: It is increased and fatty acids are released. The\  are re-esterified into triglycerides resulting in fa  accumulation in the liver.   ¢ Proteolysis: The result 1s increased production of urez  which 1s excreted in the urine. As a result of this, there 1:  increased efflux of amino acids from skeletal muscle poo  including alanine and glutamine.  Significance: Burns patient requires more than | g/kg/day  proteins.    ¢ Any patient exposed to smoky fire should receive 100%  oxygen via a non-rebreathing mask. If he is unconscious,  endotracheal intubation should be performed.    Clothing and jewellery should be removed because the  swelling begins immediately.    ¢ Small burns can be managed by cool water. Do not use  ice-cold water.    Allergy  1. First aid: Cold water bath should be given immediately. Medications  This takes away the heat, stabilises mast cell, thus Past medical history  decreasing the release of histamine and reduces oedema. Last meal    2. Careful history-taking AMPLE (Key Box 13.3). The time Events regarding injury  Since sustaining the injury, the type of heat source,  location and circumstances of the burn should be recorded.    3. Hospitalisation and admission in a burns ward with air- 9. Replacement of fluid volume is done using anyone of    3 rations in 12 hours, 2 rations in next 12 hours and  causes severe degree of pain.    ] ration in next 12 hours.  ¢ Full thickness burns: Involvement of full thickness of Example: 40% burns patient weighing 60 kg.    5. Assessment of extent of burns in terms of body surface Ist 12 hours : 3600 ml fluid  area (BSA): It is calculated by a Rule of 9 “Rule of 2nd 12 hours : 2400 ml fluid  Wallace” 3rd 12 hours : 1200 ml fluid    - Burns of posterior trunk = 18% ¢ In any patient with burns above 20%, packed red cells are    ¢ Burns of the lower limbs: 18 x 2 = 36% (front and back given in the 2nd ration (after 12 hours).  of each lower limb is 9%)   8. A Ryle’s tube is passed. A burn patient can develop “acute ° After 36 hours, depending upon the urinary output and  stress ulcers” called acute peptic ulcers or Curling ulcers. depending on the requirements of the body, about 2—3 L of  Hence, to prevent bleeding, a cold stomach wash is given fluid are given per day.  through the Ryle’s tube. Antacids and H, receptor blockers b. Parkland’s formula: 4 ml/%/kg in the first 24 hours.  such as Ranitidine 150 mg twice a day are also given. 1/2: In 1st 8 hours, 1/4: Next 8 hours each.    Ist 24 hours: 2 ml/% of burns/kg (deficit) + maintenance  (2500 ml).    2nd 24 hours: 50% of the deficit in Ist 24 hours +  maintenance (2500 ml in adults).    Rate of administration: 50% in the first 8 hours after injury  and 50% in the next 16 hours.    10. Broad-spectrum antibiotics are given against gram +ve,  gram —ve and anaerobic organisms.    ¢ To treat septic shock, higher antibiotics such as  cephalosporins have to be given.    PEARLS OF WISDOM  DO NOT GIVE COLLOID itn first 24 hours.    11. Narcotic analgesics such as injection morphine 10-12 mg  IM or injection pethidine 60—75 mg IM 1s given to relieve  the pain. (If the veins are not visible, a “cut down” needs  to be done, so that the infusion cannula can be maintained  for a longer time.)    1. Clean the wound with antiseptic agents such as savlon,  1odine.    Open method: Exposure line of management: If facilities  available are good, with a fumigated ward, the wound can  be left open to the atmosphere after applying Si/ver  Sulfadiazine cream.    Closed method: Alternately, after applying silver  sulfadiazine, 1% silver nitrate and mefenide cotton rolls  are applied, over which a bandage ts applied. This 1s called  closed method. Advantages of closed method are less pain,  reduced infection and sogging, and better medication.    3. Surgery: Tangential excision followed by grafting 1s done  after about 48-72 hours in which the burn wound ts excised  tangentially till fresh bleeding occurs. This is followed by  skin grafting. Skin is taken from the remaining normal area,  or can be obtained from skin bank. A mesh expander and  cell culture may also be used.    Advantages of tangential excision  1. It prevents secondary infection and septicaemia.    2. Decreases the hospital stay (2-5 weeks 1s the usual time  taken for eschar of III° burn to separate. This is reduced by  tangential excision).   2. Choking, suffocation due to respiratory burns especially 11  burns of head and neck.   3. Septicaemic shock is the most common cause of death.  Mnemonic: Look for 6 Ps  Pain, Pallor, Pulselessness, Paraesthesia, Paralysis  Poikilothermia (in the affected part).  e If these are present, it indicates compartment syndrom:  ¢ It needs to be treated by escharotomy.    FACTORS AFFECTING OUTCOME  e Extremes of age  e Depth of burns  e Inhalation of noxious fumes  e Cardiopulmonary disease   e Split skin graft is usually harvested using Humby’s knife  (Fig. 13.7). Drum dermatome or a power dermatome may  also be used.    Consists of epidermis and full thickness of dermis.  Harvested using ordinary scalpel.   Used for small uncontaminated wounds produced after  excision of skin lesions or after release of skin  contractures (lower eyelids, fingers).   Donor area needs primary suturing or split skin graft  for healing and hence limits the size of the graft.  Unlike split skin grafts, full thickness grafts do not  contract and retain their colour. Hence, they are  cosmetically superior.    Post-traumatic (e.g. avulsion and degloving injury)  Post-surgical (e.g. excision of tumours, excision of burn  wound)    ¢ Infection by beta-haemolytic streptococci. They produce  fibrinolysin which dissolves fibrin.   ¢ Presence of an infected wound with copious discharge in  the vicinity.   e Avascular wounds: With exposed bare bone without  periosteum, exposed tendon without paratenon and  exposed cartilage without perichondrium.    Donor area of split skin graft heals by epithelialisation from  the adnexal remnants of dermis, pilosebaceous follicles and/  or sweat gland apparatus. Complete healing of donor area  occurs by 8-10 days.    1. Wound should be free from infections such as Streptococci  and Pseudomonas    . Vascular wounds, e.g. wounds with healthy granulation.  . Wound should be thoroughly debrided.   . Haemostasis must be achieved before placing the graft.  . Close and immobile contact between graft and the wound.  . Recipient area should be immobilised with POP slab.    ¢ The processes which result in reattachment and  revascularisation of the graft to the bed are collectively  referred to as “take” of graft.   ¢ The graft initially adheres to its new bed by fibrin.  Revascularisation starts by 48 hours and 1s completed by  4—5 days. This is achieved by the outgrowth of capillary  buds from the recipient area to unite with those on the deep  surface of graft. For the first 2 days after grafting, the skin  graft derives its nutrition from the wound by the process of  serum imbibition/plasmatic circulation.    Flap is a block of tissue transferred from donor to recipient  area along with its vascularity.    ¢ To cover defects/wounds where free skin graft cannot be  used, e.g. exposed bare bones, bare tendons, bare  cartilage.   ¢ In wounds with soft tissue loss, where future reconstructive  surgery 1s contemplated.    These are broadly classified into Pedicled flaps and Free flaps.  |. Pedicled flaps    Pedicle or the base remains attached to the donor site during   its transfer to the recipient area (Figs 13.8 to 13.10). Pedicled   2. Pectoralis major myocutaneous (PMMC) flap, delto-  pectoral (DP) flap for head and neck defects, transverse  abdominis myocutaneous (TRAM) flap for breast  reconstruction.  Regional flap, e.g. PMMC, DP for head and neck defects,  TRAM for breast reconstruction.   3. Distant flaps, e.g. groin flap, subaxillary flap for hand  defects.   Fig. 13.9: Latissimus dorsi flap  used to cover the defect in the arm  following radical excision of a  sarcoma    These are completely detached from the donor area before  being transferred to the recipient area. The vascularity of the  flap at the recipient site 1s immediately restored by  anastomosing the vessels of the flap with the vessels at the  recipient area using microvascular techniques.    ¢ Forehead flap: Entire forehead skin can be raised based  on anterior branch of superficial temporal artery. It bears  an unsightly scar of the donor site. Median (Indian)  forehead flap based on supratrochlear vessels is a very  useful flap in reconstructing defects over nose.    ¢ Deltopectoral (DP) flap: It is supplied by upper 4  perforating branches of internal mammary artery and 1s  used to reconstruct detects of neck and lower face. After  about 4 weeks, the flap is divided and the base is returned  to the chest wall.    ¢ Pectoralis major myocutaneous (PMMC) flap: Pectoral  branch of thoracoacromial artery is the pedicle of this flap.  It is the ideal pedicled flap for reconstruction of head and  neck defects following ablative surgeries for various head  and neck cancers. Hence, it 1s described as a workhorse  among the flaps. Osteomyocutaneous PMMC flap by  including Sth or 6th rib can be used for mandibular  reconstruction.    ¢ Latissimus dorsi flap (LDF): As a myocutaneous flap,  based on thoracodorsal vessels can be used for  reconstruction of the lower half of face, neck, breast, chest  wall, axilla, and upper arm.    Using operating microscope and microvascular techniques, it  is possible to do a free tissue transfer of tissues such as skin,  muscle, bone, intestine, omentum, etc. The procedure involves  anastomosis of vessels of the flap to vessels at the donor site,  e.g. latissimus dorsi muscle myocutaneous flap, radical artery  forearm flap, gracilis flap and free fibula flap.    The most important factors taken into consideration while  assessing the damage caused by the passage of electrical  current through the body are: The nature of electric current  and the resistance of body tissues through which the electric  current is passing.   ¢ When a portion of the body comes in contact with a live  electric wire, the actual point where the electric current  enters the body is known as point of entry and where it Is  grounded as the point of exit. The most important factor  which decides the severity of injury is the “voltage” of  current.   e The resistance offered by the tissues to the flow of current  leads to the conversion of electrical energy to thermal  injury and causes tissue damage.    ¢ True electrical injury: The burns is as a result of heat  generated due to the passage of current.   ¢ Flame burns: It is due to an electrical flash or spark.   ¢ Arc burns: Localised injury due to intense heat at the  termination of current flow. It occurs when it jumps the  gap between the source and the conductor, e.g. flexor aspect  of joints.    a. Low voltage injuries (less than 1000 volts of current)  (1) Flash burns (11) Contact burn.    b. High voltage injuries (more than 1000 volts of current)  (1) Flash (11) Arc or contact  1. Punctate  2. Extensive  3. Extensive with vascular impairment of extremities.  ¢ Compartment syndrome and dry gangrene   b. Acute central nervous system complications such as  transient loss of consciousness, agitation, and confusion.  Rarely, hemiplegia, aplasia, convulsion and memory  defects can occur.   e. Injuries to other internal organs due to fall or electric  current.    1. Delayed spinal cord injury  2. Delayed peripheral neuropathy  3. Optic nerve atrophy, cataract    SUMMARY OF MANAGEMENT OF  ELECTRICAL INJURY  e Initial resuscitation as in thermal burns  e Low voltage injury  Superficial—conservative  Deep dermal—tangential excision and split skin graft  Contact burns—excision with SSG/flap  e High voltage injury  Flash burns: Superficial—conservative  Deep dermal: Tangential excision and split skin graft    e Extensive injury without vascular compromise: Wider  excision with split skin graft or flap Cover   ¢ Chemicals are a relatively uncommon cause of burns. The  chemicals used in industry, science laboratories and at home  are the usual agents.   ¢ The tissue damage in chemical burns is mainly due to  prolonged contact period and effects of systemic absorption.    1. Acids +: Hydrochloric acid, sulphuric acid, nitric acid,  hydrofluoric acid, phenol (carbolic acid), oxalic    2. Alkalis : Sodium hydroxide, potassium hydroxide,  ammonium hydroxide, lithium, barium and  calcium hydroxide    ¢ Acid causes coagulative necrosis of the skin due to rapid  conversion of protein to coagulum salt of the acid. The  coagulated eschar prevents these acids to penetrate deeply.  Activity in the tissues continues for a long time.   ¢ Alkalis are corrosive agents and produce extensive  denaturation of tissue proteins. These produce more tissue  destruction than acids.    ¢ Early irrigation of injured area with large volume of water  or running water (with few exceptions) is the main focus in  the first aid management of chemical injuries. Hydrotherapy  mechanically cleans the area reducing the concentration of  chemicals and duration of contact. Earlier the hydrotherapy  is started, more is the benefit obtained.    e Since the absorption of phenol increases with dilution,  surface phenol is removed by solvent polyethylene glycol  before hydrotherapy is started.    Achieved in a similar way as in any other thermal injury   ¢ Primary excision and split skin grafting, of all acid and  alkali burns, if done before 10 days.   ¢ Grafting of granulating wound, if the patient presents  late, with wound infection. This takes much longer (1%  months) to heal compared to thermal burns (3 weeks).    Very common in acid and alkali burns. Severe blepharospasm  and forceful rubbing increases the severity of injury. Various  sequences of events are:    ¢ If perforation does not occur, corneal opacity and heavy  vascularisation occurs. Quick, thorough and prolongec  lavage of cornea with water is the most important measure  in the first aid treatment. Systemic and topical steroid:  minimises inflammation and scarring of cornea.    ¢ This is one of the important requirements wherein large  surface area of burns and skin is lost.   ¢ An ideal substitute must be affordable, permanent, provide  normal pigmentation, resist scar formation and grow with  developing children.   — Dermal substitutes: They allow for creation of a  ‘neodermis’. They are formed from patient’s own  mesenchymal cells. Once ‘neo-dermis’ is formed, split  thickness skin graft is applied. Thus, the burns site is  closed quickly with less scarring.   — Another cultured skin is a biologic dressing from  cultured neonatal keratinocytes and fibroblasts. They  are all very expensive.    It refers to usage of external water (may be very cold) to run   1. Immersion hydrotherapy. It is performed in tubs called  hydrotanks or burns tanks. Tanks should be disinfected after  usage. Water used Is sterile.   2. Shower hydrotherapy: Depending upon severity of burns,  the shower can be either in supine or sitting position.  Showering immediately rinses away dead skinand bacteria.   ¢ Sedation or general anaesthesia is required as it can be very  painful.   3. Acute tubular necrosis can occur in burns due to the A. Split skin graft    4. Following metabolic response to burns occur except: 9. Following are true for pectoralis major myocutaneous  A. Gluconeogenesis is increased flap except: .  B. Lipolysis is increased A. It is the flap for head and neck reconstruction  C. Catecholamines are increased B. Based on pectoral branch of thoracoacromial artery  D. Proteolysis is decreased C. It is an example of pedicle flap  D. It is a free flap  5. Tangential excision refers to:    All photographs and the entire text in this chapter are contributed by Professor Pramod Kumar, Head of the Dept. of Plastic Surgery, KMC,  Manipal, and Dr Bhaskar KG, Senior Consultant, Dept. of Plastic Surgery, Medical Trust Hospital,Cochin, Kerala.    Human blood has a hydrogen ion concentration [H"] of 35 to  45 nmol/L andit 1s essential that its concentration is maintained  within this narrow range. Hydrogen ions are nothing but  protons which can bind to proteins and alter their  characteristics. All the enzymes present 1n the body are proteins  and an alteration in these enzyme systems can change the  homeostatic mechanisms of the body. Hence, a disturbance in  acid—base balance can result in malfunction of the various  organ systems.    pH notation is a more common method of expressing the  hydrogen ion concentration. It is defined as the negative  logarithm to base 10 of the [H*] expressed in mol/L. pH of  blood = 7.4    What is an acid? What is a base? What is a buffer?    ¢ An acid ts a substance that dissociates in water to produce  H*.   ¢ A buffer is a combination of a weak acid and its conjugate  base. By combining with a strong acid or a strong base,  they produce the corresponding salt and a weak acid or a  weak base respectively.    For example: A weak acid such as carbonic acid with its  conjugate base, sodium bicarbonate Is called the bicarbonate/  carbonic acid buffer system. When a strong acid such as  hydrochloric acid is added to the solution, it combines with  the weak alkali (sodium bicarbonate) to form sodium chloride  and carbonic acid. When a strong base such as sodium  hydroxide is added, it combines with the weak acid (carbonic  acid) to form sodium carbonate and water. Thus, a strong  acid and a strong base are converted into a weak acid  and a weak base by the bicarbonate/carbonic acid buffer  system.    The hydrogen ion concentration of blood is maintained  within narrow limits because of the presence of buffers in the  body. These natural buffers are of two types: Extracellular  and intracellular.    ¢ The extracellular buffers are bicarbonate/carbonic acid  buffer system, phosphate buffer system and plasma proteins.  The intracellular buffers are haemoglobin and other  proteins.    * The most important buffer system in the body is the  bicarbonate—carbonic acid buffer system. This 1s because  of the ability of the body to maintain or alter the  concentrations of its two components separately. The  concentration of carbonic acid is regulated by respiration  wherein the excess carbonic acid 1s eliminated as carbon  dioxide by the lungs. The bicarbonate concentration is  independently regulated by the kidneys.    The hydrogen ion concentration is proportional to the  concentration of buffer systems of the body. The hydrogen  ion concentration, carbonic acid levels and the bicarbonate  levels of blood are related according to the following equation:  , HCO; (mmol/L)   Where K = constant. This equation is called the Henderson  equation.   The amount of carbonic acid in the blood is directly  proportional to the partial pressure of carbon dioxide in  the blood. Thus, the carbonic acid concentration 1s a product  of the partial pressure of carbon dioxide in blood times its  solubility coefficient.   [H,CO,] = a PCO,, where a = solubility coefficient of  carbon dioxide in blood and PCO, is the partial pressure of  carbon dioxide in blood.   oa = 0.03 ml/mmHg/100 ml blood and normal PCO, = 40  mmHg   K = 800 for the carbonic acid/bicarbonate buffer system.  The normal bicarbonate level of blood is 24 mmol/L.    When expressed in logarithmic form, the Henderson  equation Is written as follows:    This logarithmic version of Henderson equation is called  the Henderson-Hasselbalch equation.   The pK, (negative logarithm of the constant K) of the  carbonic acid/bicarbonate buffer system is 6.1.   It is important to appreciate that the [H*] and pH are  inversely related. When the [H‘] rises, the pH decreases and  vice versa.   The normal pH of blood 1s 7.35—7.45. Acidosis is defined as a  pH less than 7.35. Conversely, when the pH 1s more than 7.45,  alkalosis is said to exist. Acidosis and alkalosis are of two  types each: respiratory and metabolic.   An increase in carbon dioxide (CO,) levels increases  the plasma |H*| and decreases the pH (respiratory  acidosis). Similarly, a decrease in plasma carbon dioxide levels  reduces the [H*] and increases the pH (respiratory alkalosis).  A decrease in [HCO, ] reduces the pH and is called metabolic  acidosis. Similarly, an increase in [HCO, ] increases the pH  and produces metabolic alkalosis.   The pH is regulated in the human body mainly by two  organs: the respiratory system and the renal system.   The arterial carbon dioxide levels are regulated by the  respiratory system. Any increase in carbon dioxide levels  stimulates the respiratory centre in the medulla thus  augmenting respiration, alveolar ventilation and elimination  of extra CO, levels. A decrease in CO, levels may reduce the  stimulus to breathe and cause hypoventilation. This response  is limited by hypoxia as the hypoxic drive stimulates the patient  to maintain respiration. Respiratory response to changes in  CO, level occurs very fast.   The plasma bicarbonate levels are regulated by the kidneys.  Any decrease in [HCO, ] stimulates the kidney to retain and  synthesise bicarbonate. High [HCO, ] results in elimination  of more bicarbonate in urine. In general, the pulmonary  response to a change in acid—base status is faster and occurs  immediately. However, renal regulation takes time, a few hours  to days. Kidneys filter and reabsorb all the bicarbonate in the  urine. When necessary, kidneys can also produce extra  bicarbonate through the glutamine pathway.    When an acid—base disorder occurs, the initial disturbance that  occurs 1s termed the primary disorder. The body attempts to  normalise the pH by certain compensatory mechanisms  resulting 1n a secondary disorder, e.g. primary metabolic  acidosis results in an increase in hydrogen ions and a  consequent decrease in bicarbonate ions. To compensate for  this, the patient hyperventilates and reduces the arterial carbon  dioxide levels, thus moving the pH back to normal  (compensatory respiratory alkalosis).   e Metabolic acidosis  e Respiratory acidosis  e Metabolic alkalosis  e Respiratory alkalosis    e Respiratory alkalosis  e Metabolic alkalosis  e Respiratory acidosis  e Metabolic acidosis    This disorder occurs when the patient’s ability to maintain  minute ventilation 1s compromised. This may be acute or  chronic in origin. The causes may be classified as follows:    ¢ Central nervous system: Central nervous system  depression due to trauma, tumour, infections, ischaemia or  drug overdose. Spinal cord injuries, especially cervical or  high thoracic can cause respiratory muscle paralysis.    ¢ Peripheral nervous and muscular system: Guillain-Barré  syndrome, tetanus, organophosphorus poisoning,  poliomyelitis, myasthenia gravis.   ¢ Primary pulmonary disease: Asthma, chronic obstructive  pulmonary disease, acute respiratory distress syndrome,  pneumonia.    ¢ The features of the underlying problem predominate the  clinical picture.   ¢ If acute, hypoxia and hypercarbia result in tachycardia,  hypertension, arrhythmias, confusion, drowsiness and  coma. The hypoxia, if untreated, can be fatal.   ¢ If gradual in onset, as in chronic obstructive pulmonary  disease (COPD), the patient’s kidneys may compensate by  retaining bicarbonate resulting in compensatory metabolic  alkalosis. Arterial blood gas analysis in these patients  typically shows low PaO., high PaCO,, high bicarbonate  levels and a near-normal pH.   ¢ Maintenance of oxygenation and ventilation using  mechanical ventilatory support till recovery of the primary  problem occurs.    This occurs due to an increase in minute ventilation. This  increase can be sustained only in abnormal conditions. This  may be acute or chronic in origin.   ¢ It may also occur secondarily as a compensation to primary  metabolic acidosis   ¢ Acute severe hypocarbia (PaCO, < 20 mmHg) may cause  cerebral vasoconstriction, reduced cerebral blood flow,  confusion, seizures and tetany.   ¢ The alkalosis and consequent hypokalaemia can also cause  cardiac arrhythmias.    Causes  This is associated with a decrease 1n bicarbonate ions due to  one of two reasons:  1, Overproduction or retention of nonvolatile acids in the  body, as in  ¢ Diabetic ketoacidosis  ¢ Lactic acidosis  ¢ Salicylate poisoning, methanol poisoning  ¢ Renal failure  2. Loss of bicarbonate tons from the body as in  ¢ Diarrhoea  ¢ Intestinal fistulae   * Deep, gasping type of respiration seen in diabetic  ketoacidosis is called Kussmaul’s respiration.   ¢ Adequate ventilation must always be ensured in all these  critically ill patients.   ¢ If pH < 7.1 and the patient 1s unstable, may administer  sodium bicarbonate. The chances of life-threatening  arrhythmias are reduced when pH 1s > 7.2.    Bicarbonate requirement (mmol) = Body weight (kg) x base  deficit (mmol/L) x 0.3    (Each ml of 8.4% NaHCO, solution contains | mmol of HCO .  Each ml of 7.5% NaHCO, solution contains 0.9 mmol of  HCO; ).   Half the calculated dose of bicarbonate should be given  slowly and should be followed up with repeat blood pH  measurements as required.    The law of electroneutrality states that the total number of  positive charges must be equal to the total number of negative  charges in the body fluids. Thus, cations (positively charged  ions such as sodium and potassium) must produce a charge  exactly balanced by anions. However, the concentrations of  only sodium, potassium, chloride and bicarbonate ions are  routinely measured in clinical practice. The sum of the  measured cations (Na*, K*) exceeds the sum of measured  anions (CI and HCO, ) producing a ‘deficit’ called the “anion  gap”. The normal anion gap is 9-14 mmol/L. This gap is due  to the presence of unmeasured anions in the body.    Since the extracellular concentrations of potassium is small,  it is often ignored in the calculation of anion gap. The equation  may be written as follows:    Anion gap = ({[Na*] + [K*] )—( [Cl] + [HCO, })    Anion gap may be used to distinguish the cause of metabolic  acidosis.    e Anion gap is increased (> 14 mmol/L) in metabolic  acidosis due to anincrease in fixed acid load. These acids  react with the bicarbonate ions in the plasma lowering its  concentration. The anion portion of the fixed acid is not  measured in the laboratory and contributes to the  ‘unmeasured anion’ concentration, thus increasing the anion  gap.   ¢ Anion gap remains unchanged in metabolic acidosis due  to loss of bicarbonate ions as the lost bicarbonate ions are  replaced by chloride ions. This type of metabolic acidosis  is also called “hyperchloraemic acidosis”.   This may be either due to loss of acid from the body or   ¢ Loss of gastric hydrochloric acid as in vomiting, prolonged  nasogastric drainage   ¢ Excessive loss of H™ from kidneys in exchange for K* in  severe hypokalaemia   ¢ Excessive exogenous administration of alkali, e.g.  indiscriminate use of NaHCO,, antacid abuse   ¢ Retention of bicarbonate in exchange for loss of chloride  ions as in diarrhoea.    It is one of the common acid—base disorders in the intensive  care unit. The underlying problem gives a clue to the cause of  metabolic alkalosis. When severe, can cause hypoventilation  and seizures. Associated hypokalaemia can cause arrhythmias  and contribute to difficulty in weaning patients off a ventilator.    e Rarely, as in life-threatening metabolic alkalosis (pH > 7.7),  rapid correction may be necessary and may be achieved by  administration of H* in the form of dilute hydrochloric acid  or ammonium chloride.    Analysis and conclusion of arterial blood gas (ABG) report  must always be done in conjunction with history and clinical  examination. ABG analysis is done to assess:    The partial pressure of oxygen in arterial blood (PaO,) of a  normal, healthy, young adult is usually 90-100 mmHg. An  increase in inspired oxygen concentration (FIO,) is expected  to increase the PaO,. The expected PaO, of a normal person  may be estimated rapidly using the following formula:    For example: A person breathing 40% oxygen is expected to  have a PaO, of 40 x 5 = 200 mmHg.   In patients with diseased lungs and increased shunt fraction  (not ventilated but perfused alveoli—‘‘wasted perfusion’), the  PaO, will not rise at the same rate as in a normal person. As  the shunt fraction increases, the rate of rise in PaO, reduces.  If shunt fraction exceeds 40-50%, there may not be any rise  in PaO, with an increase in FIO,. Since the PaO, depends on  the FIO, it is important to remember to relate the PaO, to the  inspired oxygen concentration whenever oxygenation status  of an individual is to be assessed.    Low PaQ, responds to simple oxygen therapy if shunt  fraction is 30% or less. If large shunts are present, measures  must be taken to improve ventilation of the lungs, if necessary  use endotracheal intubation and mechanical ventilation so that  the shunt fraction is reduced.    A PaO, less than 60 mmHg can be life-threatening  [corresponding to an arterial oxygen saturation, SpO, of 90%    as measured by a pulse oximeter and an attempt should  always be made to keep a patient’s PaO, (SpO,) above this  level]. Exceptions to this may be an individual acclimatised  to low PaO, such as at high altitudes, cyanotic congenital heart  disease or chronic obstructive pulmonary disease.    Nomograms are available to calculate the shunt fraction  but an easy bedside assessment of oxygenation can be made  using a PaO,/FIO, ratio (Key Box 14.3). The ratio can be used  to assess Oxygenation and to evaluate the response to therapy.  A pulse oximeter is helpful to assess whether the patient’s  oxygenation Is life-threatening or not. A saturation of 98—100%  may be reassuring. However, subtle changes in the oxygenation  status may be missed if the patient is breathing high  concentrations of oxygen. This 1s because of the sigmoid shape  of the oxygen dissociation curve where the SaO, will be 99-  100%, whether the PaO, is 100 mmHg or 500 mmHg. Hence,  an arterial blood gas analysis must always be obtained  whenever a doubt exists about the oxygenation status of  the patient.   PaO./FIO, STATUS OF OXYGENATION  500 Good  250-500 Acceptable  100-250 Poor  < 100 Critical  Ventilation    The normal PaCO, is 35-45 mmHg. The PaCO, must always  be related to the alveolar ventilation of the patient. The minute  volume of a normal healthy adult at rest would be 100 ml/kg/  min. Sixty to sixty-five per cent of this actually ventilates the  alveoli, the rest 1s dead space ventilation.    If the alveolar ventilation decreases (either due to a decrease  In minute volume or an increase in dead space ventilation),  the arterial PaCO, will rise. On the other hand, the arterial  PaCO, may remain normal but the patient’s minute volume  may have increased.    Do not assume that the patient must be well when the  PaCO, is normal. A clinical examination of the patient is  necessary to rule out respiratory distress (dyspnoea,  tachypnoea, active accessory muscles of respiration, tracheal  tug, flaring of alae nasi, etc.). The patient may not be able to  sustain this increased levels of ventilation for a prolonged  period of time, is likely to get exhausted and may require  mechanical ventilatory support.    The assessment of acid—base status must be done in three steps  and in the following order.    a. Assess the pH first: Normal pH—7.35 to 7.45. If the pH 1  less than 7.35, the patient has acidosis and if it is more thai  7.45, the patient is alkalotic. The direction of change in pt  shows the primary disorder. This is because the  compensatory mechanisms never overshoot the requiremen  of reaching the normal pH.    b. Assess the PaCO, next: Is the PaCO, normal? The norma  PaCO, 1s 35-45 mmHg. If the pH 1s abnormal but the PaCO.  normal, it suggests a metabolic disorder. However, the body  usually tries to compensate for a change in pH. The  respiratory compensation ts early and fast.    If the change in pH and PaCO, are in opposite directions  (one 1s Increased and the other decreased), the primary  disorder is respiratory. If the change in pH and PaCO, are  in the same direction (both are increased or decreased), the  primary disorder must be metabolic.    For example, if the pH is 7.2 and the PaCO, is 60  mmHg, the decrease in pH suggests acidosis. The PaCO,  has moved in the opposite direction (increased) and suggests  respiratory acidosis. Since the direction of change of pH 1s  towards acidosis and there is respiratory acidosis, 1t must  be a primary respiratory disorder. Similarly, if the pH 1s  alkalotic and the PaCO, 1s low, it suggests primary respira-  tory alkalosis.    The arterial carbon dioxide concentration (PaCO>) and  pH change in opposite directions. The extent and direction  of change in pH due to a change in PaCO, depends on  whether the disorder 1s acute or chronic and the amount of  compensation. To analyse the disorder, the ratio of change  in pH (ApH) to change in PaCO, (APaCO,;) can be used.  ApH represents the difference between the normal pH (7.4)  and the observed pH. PaCO, represents the difference  between the normal PaCO, (40 mmHg) and observed PaCQ .    A change in PaCQO, also changes the serum bicarbonate  concentration. A rule of 1-4, 2—5 (given below) can be used  to remember these changes (Key Box 14.5).   ¢ Acute respiratory acidosis 10mmHgT 71 mmol/L  ¢ Chronic respiratory acidosis 10 mmHg TT 4 mmol/L  e Acute respiratory alkalosis 10mmHgl J2 mmol/L  ¢ Chronic respiratory alkalosis 10 mmHg l J 5 mmol/L    c. Assess the bicarbonate level last: The normal plasma  bicarbonate level 1s 22—26 mmol/L. An examination of the  bicarbonate level after assessment of pH and PaCO,  confirms the acid—base disorder. If the change in pH and  the bicarbonate level is in the same direction, the disorder  is primarily metabolic and vice versa. If the bicarbonate  level 1s normal but the pH is acidotic, the disorder is of  respiratory origin.   I. A 30-year-old man was admitted to the ICU with history of  consumption of organophosphorus poisoning 4 hours ago.  On admission, he is drowsy, breathing 60% oxygen by face  mask and has a bradycardia. A blood gas analysis taken  halfan hour later shows a PaO, = 100 mmHg, PaCO, = 60  mmHg and a pH of 7.24.   Oxygenation: The PaO, on 60% oxygen should have been  about 300 mmHg. The PaO,FIO) ratio in this patient is  100/0.6 = 167. Thus, although the PaO» is adequate to  sustain life, the patient s oxygenation status is poor.  Ventilation: Raised PaCO)> suggests respiratory acidosis.  Acid—base status: The pH shows acidosis. The pH has  decreased, whereas the PaCO, has increased. Hence, the  patient has primary, uncompensated respiratory acidosis.    2. A 60-year-old man, a known diabetic since the last 15 years  is admitted with diabetic ketoacidosis. He required  endotracheal intubation and ventilation with 60% oxygen.  An arterial blood gas analysis shows the following:    PaO, = 60 mmHg, PaCO, = 28 mmHg, pH = 7.14 and  [HCO ; ] = 12 mmol/L    Analysis  Oxygenation: The PaO, on 60% oxygen should have been  about 300 mmHg. The PaO,/FIO, ratio in this patient is  60/0.6 = 100. Thus, although the PaO), is just adequate to  sustain life, the patient’s oxygenation is poor.  Ventilation: Low PaCQO) suggests respiratory alkalosis.    Changes in serum bicarbonate concentration is  accompanied by compensatory changes in PaCO, (Key Box  14.6).    It must be remembered that these are general guidelines  applicable to patients who are breathing spontaneously. These  are useful for rapid bedside assessment of acid—base status.  Occasionally, the patients can present with different combina-  tions of acid—base disorders such as a mixed respiratory and  metabolic alkalosis, or a mixed respiratory and metabolic  acidosis. Such disorders are common in patients who are  receiving mechanical ventilation in the intensive care unit.    The pH changes with a change in PaCO,, the extent of  which depends on whether the change Is acute or chronic.   Metabolic acidosis: Expected change in PaCO,  = {serum [HCO, ] x 1.5}+8+2   Metabolic alkalosis: Expected change in PaCO,  = {[HCO, ] x 0.7]} + 20+5   Acid—base status: The pH shows acidosis. The pH has  decreased, whereas the PaCO, has also decreased. Hence,  it is not respiratory acidosis and must be metabolic. The  bicarbonate levels are far below normal and suggests a  primary metabolic acidosis. The low PaCO, suggests  secondary respiratory alkalosis. The patient has primary  metabolic acidosis with partial compensation.   3. A 65-year-old man with a 40-year history of smoking, posted  for elective herniorrhaphy was sent to the pre-anaesthetic clinic  for evaluation. Since, he gave history of poor exercise tolerance  as evidenced by breathlessness even on mild exertion, and  clinical examination revealed presence of COPD, an arterial  blood gas analysis was done while the patient breathed room  air. The reportshoweda PaO, = 55 mmHg, PaCO, = 60 mmHg,  DH = 7.34 and [HCO; ] = 30 mmol/L.   Oxygenation: The PaO/FIO) ratio is 55/0.21 = 262. Thus,  although the PaO,/FIO), ratio seems adequate, the actual  PaO) is less than 60 mmHg and suggests hypoxaemia.  Ventilation: High PaCO) suggests respiratory acidosis.  Acid—base status: The pH shows acidosis. The pH has  decreased, whereas the PaCQ) is high. Hence, itis respiratory  acidosis. The bicarbonate levels are high and suggests  metabolic alkalosis. Since, the pH is acidotic but near normal,  the patient must be having primary respiratory acidosis with  compensatory metabolic alkalosis. He has fully compensated  respiratory acidosis. This picture of chronic hypoxaemia and  hypercarbia is typical of patients suffering from severe chronic  obstructive pulmonary disease.   When the serum bicarbonate levels change, there is a change  in the arterial carbon dioxide levels also.    Whether a process Is respiratory, metabolic or both, whether  acute or chronic may be deduced from the history and physical  examination of the patient. Various nomograms and formulae  are available to evaluate these situations.    A good understanding of the physiology of fluids and  electrolytes 1s fundamental to the practice of surgery. Most  surgical conditions are associated with changes in this balance  and it is only appropriate that these are identified and treated  effectively.    The human body consists of about 50-70% liquids and  30-50% solids by weight. The liquid portion varies with age,  sex and body habitus. The variation is the result of individual  differences in the fat content of the body which contains very  little water. Hence, thin individuals have greater total body  water (TBW) content as compared to obese individuals.  Similarly, the TB W is about 50% in women and 60% in men.  Neonates have up to 80% TBW. Of this total body water,  intracellular water constitutes 40% of body weight (2/3 of  TBW) and the extracellular portion, 20% of body weight  (1/3 of TBW). The interstitial fluid and plasma portions of  extracellular fluid constitute 15% and 5% of body weight  respectively (Fig. 14.1).    These fluid compartments are separated by semipermeable  membranes allowing their fluid composition to be maintained  within distinct limits. Table 14.1 shows the composition of  the intracellular and extracellular fluid compartments. The  composition of the intracellular compartments may vary  according to the tissue, e.g. fat contains very little water.    The tonicity of plasma is determined by the solutes, sodium  and its corresponding anions, chlorides and bicarbonate,  together with substances such as glucose, urea and proteins.  These particles are osmotically active and hence, tonicity is  described in terms of osmolality (mOsm/kg HO).    Osmolarity is concentration of a solution in terms of  osmoles (or mosmoles) of solute per litre of solution (solute  + water). Osmolality is concentration of a solution in terms  of osmoles (or mosmoles) per kilogram of solvent.  Osmolality is independent of the temperature of the solution  and volume of the solute. Hence, osmolality is the preferred  term in clinical practice.    I. By using the depression of freezing point of the solution:  A solution of | Osm/kg freezes at —1.86°C. Normal plasma  freezes at —0.54°C.    2. By estimating the solute concentration: Osmolality can  be estimated from the concentration of major solutes of  plasma.    Example: If a patient’s sodium concentration is 140 mmol/L,  blood glucose concentration 1s 180 mg% and blood urea is 30  mg%, his plasma osmolality can be calculated as follows:    Osmolality = 2 = [140] , 180 , 30  18 6  = 280 + 10+ 5=295 mOsm/kg    From the equation, it is evident that sodium contributes the  most to the osmolality of plasma.    A change in osmolality is usually due to changes in sodium.  The normal range of plasma osmolality is 285-300  mOsm/kg.    The plasma proteins normally do not pass out of the capillaries  into the interstitium. These raise the plasma osmotic pressure  above that of the interstitial fluid by an amount referred to as  colloidal osmotic pressure (plasma oncotic pressure). The  normal plasma colloidal osmotic pressure is 25 mmHg.  Albumin ts responsible for 75% of this oncotic pressure.    The body has mechanisms to regulate and maintain the  volume of fluids, their concentration and composition within  narrow limits to maintain homeostasis. Hence, a systematic  assessment of fluid status of a patient involves the assessment  of body fluid volume, its concentration and its composition in  that order.    I. Regulating the volume of liquid ingested: When the  extracellular fluid volume reduces, the thirst centre in the  hypothalamus is stimulated which encourages the person  to ingest more water.    2. Regulating the volume of urine excreted: This is regulated  by plasma antidiuretic hormone (ADH). A reduction in  plasma volume releases ADH from the posterior pituitary  which in turn acts on the ADH receptors in the collecting  tubules of the kidney. This results in increased reabsorption  of water and reduced production of urine. ADH release may  also be stimulated by increased plasma osmolality and  angiotensin.    A decrease in the circulating volume ts called hypovolaemia  and an increase, hypervolaemia.    This is common in surgical patients. The assessment of acute  loss of blood volume is detailed in Chapter 12. The reduction in  blood volume due to loss of water can be in the following ways:    b. Skin and lungs—0.5 ml/kg/h normally, increases by 12%  for every [°C rise in temperature   2. Examination: Thirst, dryness of mucosa, loss of skin  turgor, orthostatic hypotension, tachycardia, reduced jugular  venous pressures and decreased urine output in the presence  of normal renal function. Dehydration can be classified as  given in Table 14.2.    Loss of body  dehydration weight   Mild 5 Reduced skin turgor, sunken eyes,  dry mucous membranes  Moderate 10 Oliguria, hypotension and  tachycardia in addition to the above  Severe 15 Profound oliguria and    Haemoconcentration leads to falsely elevated haemoglobin,  packed cell volume estimations and increased blood urea  concentration. The kidneys reabsorb more water than usual  leading to increased urine osmolality (> 650 mOsm/kg).   2. Retention of water in abnormal conditions such as cardiac,  renal and hepatic failure   3. Absorption of irrigation fluid as during transurethral  resection of prostate using distilled water.   ¢ Physical examination: Distended neck veins, pedal oedema,  body weight gain    The development of these signs depends on the rate and  volume of fluid overload, renal function and cardiovascular  reserve.   Water constitutes the major component of all body fluids but  the composition varies with the fluid compartment. The most  abundant cation of extracellular fluid is sodium and is the prime  determinant of ECF volume. Ninety per cent of the ECF  osmolality 1s due to sodium.    The human body has no known mechanism to regulate  sodium intake. The body regulates sodium output by:    Addition or loss of water produces a change in the  concentration of the solute. The quantity of solute relative to  the volume of water is thereby increased (ECF ts concentrated)  or decreased (ECF is diluted) with loss or addition of water  respectively. Changes in concentration are generally changes  in water balance rather than changes in sodium regulation.  Since the changes in volume and concentration are  interdependent and the changes in water content are not easily    measured, an estimate of the fluid volume and concentration  is usually made by using the measured sodium levels and serum  osmolality.    Hyponatraemia 1s defined as a sodium level less than  135 mmol/L. Itmay occur as a result of water retention, sodium  loss, or both. True hyponatraemia is always associated with  low plasma osmolality. It may be associated with expanded,  contracted or a normal extracellular volume.    Assessment of hypovolaemia should begin with an estimation  of the extracellular fluid volume (clinically and 1f necessary,  using central venous catheters). Thus, true hyponatraemia can  be of three types: hypervolaemic hyponatraemia, hypo-  volaemic hyponatraemia and normovolaemic hyponatraemia  (Fig. 14.2).    Hypervolaemic hyponatraemia may be associated with clinical  features of hypervolaemia such as oedema. Acute  hypervolaemia (e.g. TURP syndrome—acute absorption of  hypotonic fluids into the intravascular compartment) may  result in cerebral oedema and pulmonary oedema. As plasma  osmolality decreases, water moves from the extracellular space  into the cells leading to oedema. The expansion of brain cells  is responsible for the symptomatology of water intoxication:  nausea, vomiting, lethargy, confusion, restlessness, etc. If  severe ([Na’] < 100 mmol/L), it can result in seizures and  coma. Chronic hypervolaemia as in congestive cardiac failure,  cirrhosis and nephrotic syndrome may manifest with pedal  oedema and elevated jugular venous pressures till de-  compensationoccurs. The urinary sodium concentration Is less  than 15 mmol/L.    Acute hyponatraemia (duration < 72 h) can be safely  corrected more quickly than chronic hyponatraemia. The  following factors must be evaluated: patient’s volume status,  duration and magnitude of the hyponatraemia and the degree  and severity of clinical symptoms.    Fluid restriction, diuretics and correction of the  underlying condition may be adequate in most cases. A  combination of intravenous normal saline and diuresis with a  loop diuretic (e.g. frusemide) also elevates serum sodium  concentration.    Acute symptomatic hyponatraemia Is a medical emergency.  It should be treated with hypertonic saline (1.6% or 3%).  Concomitant use of loop diuretics increases free water  excretion and also decreases the risks of fluid overload.    The sodium concentration must be corrected to relieve  symptoms and to a concentration of 125 mmol/L. Patients  who are acutely symptomatic, the treatment goal 1s to increase  the serum sodium by approximately 1—2 mEq/L/h until the  neurologic symptoms subside. The correction should be slow  and over a period of 12—24 hours with frequent checks of  sodium concentration (every 2-4 h) to avoid overcorrection.    Avoid an absolute increase in serum sodium of more than  15—20 mEq/L in a 24-hour period. If sodium correction is  undertaken too rapidly, the resulting osmolality changes in  the extracellular fluid can cause central pontine myelinolysis.  This condition is serious and can be irreversible.    The following equation can aid in the estimation of a  sodium deficit to help determine the rate of saline infusion:   A litre of normal saline (0.9%) contains 154 mEq sodium  chloride (NaCl) and 3% saline 500 mEq NaCl. In chronic  severe hyponatraemia (1.e. serum sodium <115 mEq/L), the  rate of correction should be slow and should not exceed  0.5—1.0 mEq/L/h, with a total increase not to exceed 10 mEq/  L/day.    Hypovolaemia corrected inappropriately with hypotonic fluids  such as 5% dextrose may result in hyponatraemia. The  hypovolaemia may be due to renal causes such as diuresis or  a salt-losing kidney. The urinary concentration of sodium 1s  more than 20 mmol/L in these patients. Extrarenal loss of  volume as in diarrhoea, vomiting or 3rd space loss may result  In urinary concentration less than 20 mmol/L. All these are  termed depletional syndromes and require saline infusion.    Based upon the volume status, administer isotonic saline to  patients with hypotonic hyponatremia who are hypovolaemic  to re-expand the contracted intravascular volume.    Occasionally, hyponatraemia may exist with normovolaemia.  In such situations, the plasma osmolality must be estimated.  If itis low, renal failure or the syndrome of inappropriate ADH  secretion (SIADH) may be considered.    For patients who have hypotonic hyponatraemia and are  normovolaemic (euvolaemic), asymptomatic, and mildly  hyponatraemic, water restriction (1 L/day) is generally the  treatment of choice. For instance, a fluid restriction to | L/  day is enough to raise the serum sodium in most patients.  This approach is recommended for patients with asymptomatic  SIADH.    Pharmacological agents can be used in some cases of more  refractory SIADH, allowing more liberal fluid intake.  Demeclocycline is the drug of choice to increase the diluting  capacity of the kidneys by achieving vasopressin antagonism  and a functional diabetes insipidus.    Occasionally, the hyponatraemia ts only an apparent one due  to the accumulation of other solutes such as glucose, urea,  plasma proteins or lipids. The plasma osmolality 1s either high  or normal in these patients. Such hyponatraemia ts called  pseudohyponatraemia.    Serum osmolality is governed by contributions from all  molecules in the body that cannot easily move between the  intracellular and extracellular space. Sodium ts the most  abundant electrolyte but glucose, urea, plasma proteins and  lipids are also important. Normally, their concentrations are    small and contribute to the plasma osmolality only to a small  extent. However, when the concentrations of these molecules  increase to very high levels, the relativeconcentration of sodium  In unit volume of serum may reduce. The actual amount of  sodium is normal in these patients and hence the term pseudohy-  ponatraemia. High blood sugar level or uraemia leads to higher  plasma osmolality but high plasma protein or lipid levels 1s  associated with normal plasma osmolality.    The treatment of pseudohyponatraemia mainly involves  treatment of the cause and supportive therapy.    Hypernatraemia is defined as a plasma sodium concentration  of more than 150 mmol/L and may result from pure water  loss, hypotonic fluid loss or salt gain.    The hypertonicity of plasma leads to cellular dehydration.  Clinical evidence of dehydration may not be apparent until  10-15% of body weight has been lost. Rehydration should be  slow to prevent cerebral oedema.    The diagnosis can be established by measuring plasma and  urine osmolalities and urine output.    Treatment  !. Administration of water orally/nasogastric tube  2. Administration of IV fluid—5% dextrose or 0.45% saline    3. Change in serum sodium should not be more than  2 mmol/L/h. Rapid rehydration can cause cerebral oedema.    The normal potassium level is 3.5—5.5 mmol/L. Hypokalaemiz  and hyperkalaemia are two clinically important disturbances.    This is defined as a plasma concentration of potassium less  than 3.5 mmol/L.   e Renal causes: Diuretics, renal artery stenosis, diuretic  phase of renal failure   ¢ Potassium supplements, in the form of milk, fruit juice,  tender coconut water.   ¢ Ifthe patient cannot take orally or the hypokalaemia 1s  severe, Intravenous potassium chloride 1s usually given at  a rate of 0.2 mmol/kg/h. If there are life-threatening  arrhythmias, it may be given at a rate not exceeding 0.5  mmol/kg/h under electrocardiographic monitoring and  serial measurements.    Treatment of hyperkalaemia  1. Calcium gluconate (10%): 10-30 ml.  2. Sodium bicarbonate: |—2 mmol/kg over 10—15 minutes.    ¢ Magnesium is the second most abundant intracellular ion.  The normal intracellular concentration of magnesium Is  about 26 mEq/L and extracellular concentration is |.5—2.5  mEq/L. Almost 60% of the magnesium is deposited in the  skeleton. Magnesium 1s required as a cofactor for several  important enzymatic reactions including the phosphoryla-  tion of glucose within cells and the use of ATP by contracting  muscle fibres.   ¢ A daily dietary intake of 0.3-0.4 g (approximately  20-30 mEq) ts required. The proximal convoluted tubule  reabsorbs magnesium very effectively.    ¢ Calcium is the most abundant mineral in the body. Ninety-  nine per cent is deposited in the skeleton. In addition,  calcium 1ons are important for the control of muscular and    A 21-year-old lady was found to be collapsed as she was  feeding her 15-day old baby in the nephrology ward. On  arrival, the cardiac arrest response team found her to have  ventricular tachycardia without pulse. Cardiopulmonary    resuscitation was given and she was shifted to the intensive  care unit after return of spontaneous circulation.  Investigations showed that her potassium level was 1.6  mmol/L. She had been admitted to the nephrology unit for  postpartum acute renal failure. She had been dialysed three  times following which she had gone into the diuretic phase  of recovery from acute renal failure. She was putting out  about 5 litres of urine per day in the last two days. Her  hypokalaemia was corrected over 2—3 days. She recovered  completely and could be discharged from the ward in  5 days time.   neural activities, in blood clotting, as cofactors for  enzymatic reactions and as second messengers.    ¢ Calcium homeostasis reflects a balance between reserves  in the bone, rate of absorption across the digestive tract,  and rate of loss from the kidneys. The hormones parathyroid  hormone (PTH), vitamin D and calcitonin maintain calcium  homeostasis in the ECF. Parathyroid hormone and vitamin  D raise Ca** concentrations and calcitonin lowers it.    ¢ Calcium absorption from the digestive tract and reabsorp-  tion along the distal convoluted tubule are stimulated by  PTH from the parathyroid glands and calcitriol from the  kidneys. The average daily requirement of calcium in an  adult is 0.8—1.2 g/day.    Hypercalcaemia exists when the Ca** concentration of the ECF  is above 11 mg%.    Severe hypercalcaemia (12—13 mg%) causes symptoms such  as fatigue, confusion, cardiac arrhythmias, and calcification  of the kidneys and soft tissues throughout the body.    Hypocalcaemia exists when calcium level is < 9 mg%.  However, the serum calcium level should be related to the  albumin levels. Half the serum calcium is bound to albumin  and as albumin levels become low, this bound fraction is lower  leading to a low total serum calcium concentration. Free tonic  calcium is important for the electrical activity of the nerves  and muscles and 1s more reliable (Normal: |.0—1.4 mmol/L).    Muscle spasms, stridor, generalised convulsions, myocardial  depression, cardiac arrhythmia and osteoporosis.   CAUSES OF HYPERCALCAEMIA  e Hyperparathyroidism  e Malignant cancers of the breast, lung, kidney or bone marrow    CAUSES OF HYPOCALCAEMIA  e Hypoparathyroidism  e Vitamin D deficiency  e Chronic renal failure    A patient undergoing surgery needs intravenous fluids to  replace volume deficit acquired during starvation, normal  maintenance for the duration of surgery and volume lost during  surgery. Depending on the extent of dissection, fluid  accumulates in these tissues in the form of oedema (third space  losses). In addition, blood loss also needs to be replaced.    Perioperative fluid therapy in a patient whose body  homeostasis is normal   1. Fluid requirement during starvation: Patients awaiting  anaesthesia and surgery need to be kept fasting for a few  hours prior to and after the surgery. Except in very minor  surgery, fluid lost during this period needs to be replaced.  This volume is calculated at the rate of 2 ml/kg/h for the  number of hours of fasting and is then replaced over  2-3 h.   2. Maintenance requirement: The average daily requirement  of water for an average-sized adult 1s 2000 ml. In general,  a volume of 30-35 ml/kg/day meets the daily maintenance  needs. This is calculated as 2 ml/kg/h of surgery.    3. Third space losses:  ¢ 4 ml/kg/h for surgery with minimal dissection, e.g.  herniorrhaphy  ¢ 6 ml/kg/h for surgery associated with moderate  dissection, e.g. gastrojeyjunostomy and vagotomy  ¢ 8 ml/kg/h for surgery associated with large amount of  dissection, e.g. Whipple’s procedure.    4. Blood loss is replaced by compatible blood transfusion  (homologous or autologous), if the haematocrit falls below  25%. Blood loss is replaced with an equal amount of  colloids or three times the volume with crystalloids if the  haematocrit 1s > 25% in an otherwise healthy individual.  Crystalloids are electrolyte solutions that distribute  themselves throughout the body water and hence, a larger  volume needs to be given.    A patient undergoing surgery should receive fluid deficit  due to starvation + maintenance fluids + third space losses +  replacement of blood loss (as detailed above). Adequacy of  fluid replacement should be checked with haemodynamic  stability and urine output in major procedures. When very large  fluid shifts are expected (oncologic surgeries), and the patient  has compromised cardiac status or has renal insufficiency, it  may be necessary to monitor fluid status using central venous  pressure monitoring.    In the evaluation of a patient with a suspected problem tr  fluid and electrolyte or acid—base balance, careful sequential  analysis of the volume, concentration and composition (in that  order) followed by appropriate therapy protects the patient  from severe, perhaps fatal errors in management.    These can be broadly divided into three groups: crystalloids,  colloids and special purpose solutions.    Crystalloids: These are essentially solutions of electrolytes  in water, e.g. Ringer lactate. Some also contain dextrose, e.g.  dextrose saline, 5% dextrose and paediatric maintenance  solutions. They vary in the content of different electrolytes.    Colloids: These are solutions of large molecules which tend  to remain in the intravascular compartment, e.g. gelatine,  hetastarch, pentastarch, dextran 40, dextran 70. They are all  plasma expanders since the molecules tend to exert osmotic  forces and draw fluid from the interstitial compartment into  the intravascular space. The colloids vary in their magnitude  of volume expansion and duration of action. Dextran 40  reduces viscosity of blood and maintains blood rheology better.  Hence, it is used as continuous infusion after microvascular  surgery. It also reduces platelet aggregation which along with  reduced viscosity helps in maintaining blood supply to free  flaps and vascular grafts.    Volume deficits and losses are usually replaced using  Ringer lactate. However, if the patient has disturbances of  concentration and composition, other solutions may need to  be given.    Sodium bicarbonate: It is available as 7.5% (0.9 mEq/ml)  and 8.4% (1 mEq/ml) of sodium bicarbonate.   ¢ Alkalosis with a shift of oxygen dissociation curve to the  left (increased affinity of haemoglobin to O,, reducing its  unloading)    ¢ Circulatory overload leading to cardiac failure  ¢ Carbon dioxide load leading to respiratory failure    Mannitol (10 and 20%): It is an osmotic diuretic. Its main  use 1s to reduce intracranial pressure by producing diuresis.  It is also used to reduce intraocular pressure. Mannitol  expands intravascular volume initially by drawing fluid from  the interstitium. This 1s followed by diuresis. It should be  used with caution tn patients with cardiac failure, renal failure,  etc.    Hypertonic saline (1.6, 3, and 5%): These solutions are  available to treat hyponatraemia.    Albumin: 5% albumin is used as a plasma expander. 20%  albumin can be used to replace lost albumin in severe  hypoalbuminaemia in addition to plasma expansion.    Cells can perform their function only when they get nutrients  and oxygen. When the nutrients are metabolised in the cells,  the tissues get energy to perform their physiological  functions. Gastrointestinal system (GIT) is the source of  supply of nutrients (energy) to all the tissues. It has to supply  nutrients on a day-to-day basis because the body has limited  expendable reserves (stores). Lack of nutrients results in  energy crisis.   In critical care units, priority is given to treatment of  hypoxia, haemorrhage, haemodynamic instability, fluid,  electrolyte, acid-base imbalance, and sepsis. These deserve  their priority, but hyponutrition and the consequent energy  crisis should not be ignored.    e Pathophysiology of GI failure—autocannibalism: When  GIT fails, the stores are consumed to supply energy.  Glycogen stored in the liver undergoes glycogenolysis and  gets depleted in 24 to 48 hours. Normal physiological  functions of the organs of an average adult at rest (BMR/  Resting energy expenditure—REE) need about 20 kcal/kg/  day. Therefore, when GIT fails, the fat in the adipose tissue  and protein in the muscles and viscera are mobilised and  metabolised to supply and sustain REE. This is called  autocannibalism (eating one’s own tissues to survive). It  weakens the muscles (e.g. respiratory and cardiac muscles),  viscera (liver, kidneys, etc.) and immune system, resulting  in increased morbidity and mortality.   ¢ Patients receiving intravenous (IV) fluids are  semistarving: 500 ml of 5% dextrose containing 25 g of  dextrose provides about 100 kcal (each gram of  carbohydrate provides about 4 kcal), and therefore an adult  taking nil by mouth (NBM), receiving 4—5 bottles of 5%  dextrose/day gets about 400-500 kcal; the balance amount  of REE (for a 50-kg individual, REE = 50 x 25 = 1250 kcal:   e Fasting in healthy persons versus fasting in patients:  When resting healthy persons are fasting, the metabolic  rate drops to basal level. Although critically ill patients who  are kept NBM are also resting, their basal metabolic rate is  accelerated (hypermetabolic) and their REE is increased  proportionate to the level of stress due to injury or illness.   ¢ Assessment of nutritional status: A healthy adult can  withstand semistarvation (receiving IV fluids only) for  about 4 to 5 days without ill-effects. Beyond that they need  nutritional support in order to prevent the adverse effects  of autocannibalism. Many patients are nutritionally depleted  at the time of admission and will need nutritional support  much earlier.    ¢ Weight loss is an important indicator of nutritional status.  A recent h/o 20% weight loss indicates mild, 20 to 40%  moderate and more than 40% severe undernutrition. Body  mass index (BMI) is another easily calculated indicator.  Mid-arm circumference, triceps skin fold thickness are  anthropometric indices that are useful. Low serum albumin  (3-3.5 g%—mild, 2 g%—moderate and < 2 g%—-severe  undernutrition) 1s not a sensitive indicator because of the  long half-life and a large pool size. Prealbumin, retinol-  binding protein and transferrin have shorter half-life and  smaller pool size and are more sensitive indicators.    ¢ How much nutrition to be given: Resting energy  expenditure (REE) can be more accurately calculated by  the Harris-Benedict equation. The REE ~* stress factor  equals total caloric requirement. The stress factor depends  upon the severity of illness, injury or operation and ranges  from 1.2 to 2.0. For practical purposes, it is easier to  remember that the adult REE is 20 kcal/kg/day and it  increases to 25, 30, and 40 kcal/kg/day 1n mild, moderate  and severe stress.    e Sixty per cent of the total calories should come from  carbohydrates and 40% from fats. These calories are  nonprotein calories. Calories obtained from proteins should  not be taken into account for calculating the energy needs  because they are building blocks tn tissue repair and are  not meant for burning for calories. The protein requirement  of the resting adult 1s 0.8 g/kg/day, and it increases to 1.1,  1.5, and 2.0 g/kg/day in mild, moderate and severe stress.  Recommended daily allowances of vitamins, minerals and  trace elements are added to the formulations. The daily  intake and output of fluids should be balanced.    e¢ Nutrition can be administered by either the enteral or  parenteral route.    It is paradoxical that sick patients who need to eat more to  meet the increased metabolic demands are often unable to eat.   They have anorexia, nausea, vomiting and altered sensorium.  Oral feeding is impossible in patients with faciomaxillary  injuries or those on ventilators. In many of them, the intestines  are working. Enteral route is best for providing nutrition. Hence  the dictum, “When the gut is working, use it’’.  e Enteral Access: Following are the routes to introduce  nutrients into the GIT (Table 14.3):  ¢ Nasogastric feeding: When the stomach emptying is  normal and swallowing is impossible or contraindicated,  nasogastric feeding (Ryle’s tube) provides nutrition.  ¢ Nasojejunal feeding: |n gastric stasis, feeding can be  given through a nasojejunal (NJ) feeding tube introduced  either blindly, or under radiologic or endoscopic guidance  to place its tip in the jejunum (postpyloric).  ¢ Feeding gastrostomy: By open method or by  percutaneous endoscopic gastrostomy (PEG) when RT  or NJ tube insertion is impossible.  ¢ Percutaneous Endoscopic Gastrostomy (PEG): With the  help of an endoscope, a gastrostomy tube is placed ina  retrograde manner and brought out through a skin  incision. Itis technically very easy andcan be done under  local anaesthesia. It has replaced feeding gastrostomies  (open method). It is popular nowadays (Fig. 14.3).  Complications include colonic perforation, sepsis,  bleeding, wound infection, etc.  ¢ Feeding jejunostomy: After major/complex operative  procedures on the oesophagus, stomach and pancreas, a  feeding jejunostomy is frequently established.    e What to feed: A number of preparations are commercial!  available but most cost-effective ones are the blenderise:    kitchen feeds. Enteral feeds are hyperosmolar and provid  1.2 to 2.0 kcal/ml.    Polymeric feeds: These are commonly prepared in thi  kitchen. Liquid and powder preparations ari  commercially produced. These contain polysaccharides  polypeptides and oils. Soups of dal, vegetables anc  chicken are examples of polymeric feeds.   Elemental feeds: These are predigested in vitro anc  contain oligosaccharides, oligopeptides and mediun  and long chain triglycerides (MCT and LCT). They  are useful in patients with irritable bowel disease anc  short bowel.    Disease-specific feeds: The composition of the feeds  needs to be altered in certain disease states. Renal  failure—low protein, low/ no electrolytes; Hepatic  failure—more branched chain amino acids (BCAA) and  less aromatic amino acids; Respiratory failure—more fats  (55% cal) and less carbohydrates.    a syringe or pumped into the tubes either continuously  or intermittently. To start with 50 ml every two hours on  the first day and if tolerated may be increased gradually  to 200 ml every two hours until the target is reached.   Easy, quick, cheap method Indicated when RT cannot be passed, e.g. Indicated after major oesophageal  Indicated in stroke, comatose patients, etc. inoperable carcinoma oesophagus, stricture surgeries, high duodenal fistulae  Chances of aspiration are high. Hence, 30° Malecot’s catheter is introduced into the A Ryle’s tube is introduced into the  propped up position is recommended stomach and kept in place using a purse string jejunum under vision (during surgery) and    |. The integrity of gut mucosa depends on provision of  nutrients into the gut lumen. If the fasting period  exceeds more than a few hours, the gut mucosal cells  start disintegrating and the villi get destroyed. This may  permit the intestinal bacteria to enter the circulation  leading to sepsis. Translocation of bacteria from the  intestines into the circulation has been identified as the  ‘motor of multiorgan failure’.    2. Use of natural route of nutrition requires less nursing  supervision.    3. Infection rate is lower with enteral nutrition.  4. Greater insulin response is seen with enteral nutrition.  5. There is a lower tendency to retain salt and water.    ¢ Complications of enteral nutrition: Nausea, vomiting,  abdominal distension and diarrhoea are common. However,  intractable diarrhoea should be investigated for Clostridium  difficile infection. Electrolyte imbalance, hyperosmolar  coma, refeeding syndrome and aspiration are other  problems. Tube clogging, displacement, leak and erosion  are mechanical complications.    Glutamine is an essential nutrient for the maintenance of  integrity of intestinal cells and there is evidence to show that  its inclusion in enteral feeds enhances patient immunity.    When enteral nutrition is not possible for more than a few  days, parenteral nutrition (PN) may need to be considered.  When all nutrition is done by the parenteral route, it 1s termed  total parenteral nutrition. Partial parenteral nutrition may be  given to supplement inadequate enteral nutrition.    Parenteral nutrition (PN) to GI failure is like dialysis to  renal failure and ventilator support to respiratory failure. When  enteral feeding is impossible, parenteral nutrition should be  given. Prolonged ileus, intestinal obstruction, malabsorption,  short gut, inflammatory bowel disease, high output intestinal  fistulae are some common indications for PN.    1. Dextrose (10, 20, 25 and 50%), amino acid (5 and 10%)  and lipid (10 and 20%) solutions in separate bottles.    2. Dextrose + amino acid solutions  3. All in one solutions.    One gram of glucose provides 4 kcal. The entire energy  requirement of the day can be given in the form of glucose.  This needs to be administered in a concentration of 50%, so  as not to exceed the daily fluid requirement. 1000 ml of 50%  dextrose contains 500 g of dextrose providing 2000 kcal.    ¢ Hyperglycaemia must be prevented by addition of  appropriate amount of insulin.   ¢ Increased CO, production may increase work of breathing  and results in difficulty in weaning patients from ventilator.   ¢ Infusion of dextrose-containing solutions do not take care  of daily protein and other nutrient requirements of the body.  Thus, parenteral nutrition should not be given using    Lipids  One gram of fat provides 9 kcal. Since, it is a more    concentrated form of energy, half the daily caloric requirement  can be given using 10 or 20% lipid emulsions.   ¢ Its clearance from plasma may be delayed with impaired  liver function.    The average protein requirement is | g/kg/day. Amino acid  replacement solutions are available as 10 and 20% solutions.  The calorific value of proteins should not be counted as they  are the building blocks of the body. They are not infused to be  metabolised for energy.    Vitamins are given separately. Trace elements are given  weekly to patients on long-term PN. One ampule of water-  soluble vitamins must be infused daily, over a period of time  exceeding 30 minutes to avoid urinary loss. Folic acid,  vitamins B,, K, A and D also need to be given once a week.   The total volume of PN should be 2000-3000 ml/day.  Alternately, commercially available PN solutions which are  well-balanced with all nutrients can be used. Although expen-  sive, good nutrition may reduce the morbidity and duration of  hospital stay of the patient thus proving cost-effective.    1. Peripheral vein: Solutions with less than 800 mOsm/L may  be administered through a peripheral vein. This is suitable  for short-term PN.    2. Central vein: Either internal jugular or subclavian vein is  cannulated. PN solutions with higher osmolality must be  given through a central vein.    3. Peripherally inserted central vein catheter: This also may  be used for short-term purposes.   4. Subcutaneously implanted central vein catheter ports  are especially suitable for long-term, domestic or  ambulatory PN.    The central venous access should be dedicated to PN  and should not be used for administration of drugs or  other fluids. The line should be handled with strict  asepsis.    ¢ The solutions can be gravitated but the rate of infusion 1s  better controlled if given through pumps. Smaller volumes  are given initially and is gradually increased to reach the  target volume/day. Absolute aseptic precautions are  observed while handling the catheters and the PN  formulations, since central vein catheter infection is a  dangerous complication. Central vein catheter should be  used exclusively for administering PN solutions and should  not be used for any other purpose.   Aim  1. To identify excess or deficiency of individual nutrients.  2. To identify complications.    Daily  ¢ Blood sugar, serum electrolytes, blood urea and serun  creatinine.    1. Technical complications: Injury to subclavian/carotid  artery, brachial plexus, haemo or pneumothorax.    2. Catheter-related: Central line sepsis is the most dangerous,  at times, life-threatening and yet, preventable complication  and its incidence is a measure of patient safety. It may not  be possible to control sepsis with antibiotics alone without  removing the central catheter. Thrombosis and catheter  clogging are other problems.    3. Gut mucosal atrophy: Patients on total parenteral nutrition  develop atrophy of the intestinal mucosa which loses its  barrier function and becomes permeable to bacteria. The  consequent bacterial translocation leads to sepsis and  multiorgan dysfunction syndrome (MODS). This is not seen  In patients on partial PN supplemented with EN.    4. Cholestasis: Some patients on long-term TPN develop  cholestasis, jaundice and gallstones which resolve on  starting oral/EN.    Assured delivery of nutrients, accurate and rapid correction  of fluid, electrolyte and acid—base imbalances are the merits  of PN but it is complicated and expensive.    Nutrition is an important component of the care of the  surgical or critically ill patient. Malnutrition can be  fatal. Overnutrition can also be harmful. Provision of  services of a physician, dietician, microbiologist and  good nursing care are vital to patient recovery.   . The most important buffer system in the plasma is:  A. Phosphate buffer system   . pH is the negative logarithm to the base 10 of hydrogen  ion concentration expressed in __ ;    . The solubility coefficient of carbon dioxide in plasma  is mli/mm Hg/dL    . The following is true in primary metabolic acidosis  with secondary respiratory alkalosis:    A. pH and PCO, are decreased  B. pH decreases but PaCO, increases  C. pH and PaCO, are increased  D. pH increases but PaCO, decreases    . The following is one of the causes of metabolic acidosis  with increased anion gap:   . The following may be a cause of respiratory alkalosis:  A. Morphine overdose    Metabolic acidosis with normal anion gap is also called:  A. Hyperchloraemic acidosis    In chronic hyponatraemia, the sodium concentration  Should be increased at a rate not exceeding  mmol/L/h.    The following is one of the drugs used to treat  hyperkalaemia:    11 A 12 B 13 A 14 D 15 A 16 D 17 B 18 B 19:G 20 D    This is a benign tumour arising from skin or mucous  membrane. It 1s characterised by finger-like projections with  a central core of connective tissue, blood vessels, lymphatics  and lining epithelium (Fig. 15.1). It can be called Hamartoma  or a skin tag. It is an example of overgrowth of fibrous tissue  (Key Box 15.1). It can be pedunculated with narrow base or  broad base.    Acrochordons (skin tags) are fleshy, pedunculated  masses located on the axillae, trunk and eyelids.    e Keloid : Page 8  e Desmoid tumour : Page 910  e Hypertrophic scar ; Page 8    a. Squamous papilloma occurs in the skin, cheek, tongue,  Ste:  ¢ Soft papillomas are squamous papillomas. They are seen  in elderly patients on the eyelid as small, soft, brownish  swellings.  ¢ Squamous papilloma can also be congenital,  sometimes multiple in number and can be sessile or  pedunculated.  b. Basal cell papilloma (seborrhoeic keratosis) 1s seen on the  trunk of elderly patients as brownish elevated patch of skin  and gives a semitransparent, oily appearance.    a. Transitional cell papilloma in the urinary bladder as a cause  of haematuria.   b. Columnar cell papilloma in the rectum as a cause of  mucous diarrhoea.    1. Skin papilloma can get secondarily infected resulting in  pain and swelling.   3. Papilloma in the breast, rectum, tongue and gall bladder  can undergo malignant change.    Fibroma is a benign tumour, consisting of connective tissue  fibres only. Clinically, it presents as a firm, subcutaneous  swelling. However, a true fibroma is rare. They are combined  with neural elements, muscle tissue or fatty tissue.   2. Hard fibromas: More fibrous tissue  ¢ Neurofibroma: Fibroma mixed with nerve fibres  ¢ Fibrolipoma: Fibroma mixed with fat    ¢ Myofibroma: Fibroma mixed with muscle fibres  ¢ Angiofibroma: Fibroma mixed with blood vessels    They are treated by excision because of the possibility oi  developing into a sarcoma.    Lipoma is a benign tumour arising from fat cells of adult type.  It 1s also called ‘universal tumour’ because it can occur  anywhere in the body where there is fat (Key Box 15.2).    ¢ This is a single, soft, slow-growing, painless and semi-  fluctuant swelling (Figs 15.4 and 15.5).   Fig. 15.5: Giant lipoma in the chest wall (Courtesy: Prof Rajiv  Shetty, HOD, Bangalore Medical College, Medical Superintendent,  Bowring Hospital, Bangalore)    'Students should not give the diagnosis of fibroma because in majority of cases, it is neurofibroma or fibrolipoma.   Fig. 15.7: Retroperitoneal lipoma. It can grow to large  dimensions. Mostly asymptomatic, and hence patients come late  to the hospital   Surface 1s lobular. Lobulations are better appreciated with  firm palpation of the swelling. Due to the pressure, lobules  bulge out between the fibrous tissue strands.   The edge slips under the palpating finger which ts a  pathognomonic sign of lipoma.    Fig. 15.8: Mediastinal lipoma. The  swelling was deep to pretracheal  fascia and partly substernal. It was  soft and lobular—confused for com-  pressible swelling. Candidate offered  haemangioma as_ diagnosis.  (Courtesy: Dr Salim, HOD, Surgery,  Trivandrum Medical College, Kerala  (PG exam case 2009)   Fig. 15.9: Lipoma forehead.  An undergraduate gave the  diagnosis as dermoid cyst.  You see the location. It is not  the classical site of dermoid  cyst and the ‘slip sign’ was  positive. Even though face is  not a common site, lipoma  can occur in this location.    Commonly present as a subcutaneous swelling. It 1s freely  mobile. The flank 1s the commonest site. Shoulder region,  neck, back, upper limbs are the other common sites.  (For various locations see Table 15.1, Figs 15.8 and 15.9.)  Some lipomas from the chest wall can be of large size.   Dimpling sign: Fibrous bands connect a lipoma to the  skin. When the skin moved, a dimple appears on the skin.    Such lipomas are multiple and very often tender because  of nerve elements mixed with them. Hence, they are called  multiple neurolipomatosis. Dercum s disease \s one example  of this variety (Adiposis dolorosa) wherein tender,  lipomatous swellings are present 1n the body, mainly the  trunk.    Diffuse variety is a rare type of lipoma. It 1s called  pseudolipoma. It is an overgrowth of fat without a capsule.   3. Subsynovial, Swelling in relation to knee joint, elbow joint  intra-articular (Figs 15.12 and 15.13)   4. Intermuscular Swelling of the thigh. On contraction  Thigh of the muscles, it becomes more  Shoulder region firm due to transmitted pressure    An incision is given over the swelling. Dissection is carried  out all around, separating it from underlying tissues and it  is excised (Fig. 15.11).    . Small lipoma can be removed by incising the skin followed   ¢ The swelling becomes vascular and red coloured with  dilated veins over the surface.   ¢ Liposarcoma spreads via blood. It rarely spreads via  lymphatics. Metastasis in the lung can rarely occur from  liposarcoma producing multiple chest secondaries.   ¢ Liposarcoma is treated by wide excision followed by  reconstruction either by split skin graft or by flaps. In  the thigh, sometimes radical surgery may amount to  compartmental excision. Chemotherapy and radiotherapy  can also be used but the benefit is doubtful (see page 663).   Since 3 months, it is  rapidly growing, observe  dilated veins and shiny  skin. There is local rise  of temperature. These  features are suggestive  of sarcoma.    Fig. 15.10: Pedunculated lipoma of the back of 15 years duration  with features suggestive of sarcomatous change   4. Intussusception—due to submucosal lipoma of terminal  ileum is an abdominal emergency.   5. Saponification (see Key Box 15.3 and Figs 15.12 and  15.13)    A 32-year-old man presented with gross swelling of the right  leg. He had seen two surgeons earlier who had told him that  he had deep vein thrombosis but no treatment was offered.  Examination revealed an obvious mass which was palpable  in anteromedial and posterior compartment. MRI revealed  an intermuscular mass. At exploration, an intermuscular  lipoma weighing 700 g was excised (Figs 15.12 and 15.13).    They are uncommon benign tumours which arise from  sympathetic nervous system or spinal cord. They can be  classified into true neuromas and false neuromas.    1. Ganglioneuroma: It consists of ganglion cells and nerve  fibres of sympathetic chain. They are slow-growing  tumours. When present in the neck as a parapharyngeal  mass, it can cause dysphagia. These tumours can occur in  the neck, retroperitoneum or mediastinum. Excision of the  tumour Is the treatment (see clinical notes).    2. Neuroblastoma: It consists of poorly differentiated cells.  It occurs in young children. It is interesting to know that  this tumour can undergo spontaneous regression.    3. Myelinic neuroma: It is very rare. It arises in relationship  with spinal cord made up of myelinic fibres.    ¢ Does not contain any ganglion cells.  All these three tumours are called true neuromas.    These tumours arise from the connective tissue of the sheath  of nerve endings.    They occur following nerve injuries, lacerations or after  amputation. They are of two types:    An 18-year-old engineering student who had backache was  examined by an orthopaedician and referred to general    Fig. 15.12: Compare both  the legs. Local gigantism  can also be caused by  extensive lipomatosis involv-  ing the leg. This was treated  initially as filariasis    Fig. 15.13: Soleus muscle is cut,  lipoma is Seen coming out of the  deeper plane. Luckily no major  neurovascular bundle was invol-  ved. The patient had a smooth  recovery    surgery. CT scan of the abdomen revealed mass in the  paraspinal region in the retroperitoneum. Laparotomy and  excision of the mass was done. It was a ganglioneuroma.  Paraspinal region is one of the common sites of  ganglioneuroma.    I. End-neuroma occurs after amputation due to proliferation  of nerve fibres from the distal cut end of the nerve. This  produces a bulbous swelling. If it 1s caught in the suture  line or due to pressure of the prosthesis, 1t produces severe  neuralgic pain. To avoid this, when an amputation 1s being  done, the nerve is pulled downwards and cut as high as  possible so that it retracts upwards (Fig. 15.14).    2. Lateral neuromas occur due to partial injury to the nerve  on the lateral aspect (Fig. 15.15).    It is a benign tumour arising from the connective tissue of the  nerve sheath. Typically, it produces a fusiform swelling in the  direction of the nerve fibres. The tumour contain both neural  (ectodermal) and fibrous (mesodermal) elements.    Commonly affects the peripheral nerves such as ulnar nerve,  median nerve or cutaneous nerves. Occurs in adults.   e Tingling and numbness, paraesthesia in the distribution of  the nerve, specially when the nodule 1s compressed.   ¢ Smooth surface, with round border. The swelling moves at  right angles to the direction of nerve fibres. Vertical mobility  is absent.   ¢ Being a subcutaneous swelling, the skin can be lifted up.    1. Neurofibroma, 2. Metastatic nodule, 3. Cutaneous T cell  lymphoma, 4. Chronic abscess    ¢ It is treated by excision.  ¢ In most of the cases, excision 1s easy as the tumour is we  encapsulated.    e Thisis an autosomal dominant disorder transmitted by bot!  sexes. The whole body is studded with cutaneous nodule  of varying sizes. They are soft and nontender.   ¢ Coffee brown pigmentation 1s characteristic of this conditio1  (cafe au lait spots, Fig. 21.). Cafe au lait spots can bi  associated with involvement of cranial nerves—VIIIth nerv:  (auditory nerve) acoustic neuroma—a cerebellopontin  angle tumour. Popularly called vestibular schwannoma.   ¢ Type I! is caused by gene mutation on chromosome 17.   ¢ The presence of skin pigmentation 1s an indication of the  common neuroectodermal origin of nerve sheath cells anc  melanocytes.   Fig. 15.16: von Recklinghausen’s with plexiform neurofibromatosis  (Courtesy: Dr Prashanth Shetty, Professor, Dept. of Surgery, KMC,  Manipal)    It may be associated with phaeochromocytoma (high blood  pressure).  Sarcomatous changes do occur    Record the blood pressure in all cases of von  Recklinghausen’s disease because it may be associated  with phaeochromocytoma.    pathognomonic of neurofibromatosis type 2, a syndrome  resulting from chromosome 22 mutation. It 1s also associated  with increased incidence of meningiomas and gliomas.   In this condition, the branches of 5th cranial nerve are  commonly affected. It can also involve the peripheries.  The affected part is grossly thickened due to fibro-  myxomatous degeneration.   — Tingling paraesthesia in the distribution of Vth nerve,  especially ophthalmic division.   — When it attains a huge size, it can obstruct the vision.  As it grows bigger in size, it hangs in front of the neck,  as a grossly thickened pendulous fold of skin.    This condition affects the limbs. It represents an advanced  stage of plexiform variety. Gross thickening of subcuta-    This refers to the plexiform lesions mainly found in the  neck as a thickened, coiled single mass.    Fig. 15.19: Plexiform neurofibromatosis  (Courtesy: Dr Rohit Jain, Asst. Professor,  Department of Surgery, KMC, Manipal)    Commonest site 1s the acoustic nerve. However, vagus  nerve 1s the most common peripheral site (Fig. 15.22).    They can be single or multiple and present with a fusiform  swelling in relationship with the nerves.    They can also arise from a peripheral nerve. Sensory  branches are affected more frequently (Table 15.2).   Fig. 15.20: Elephantiasis neuromatosa  (Courtesy: Dr Balakrishna Shetty, Profof Surgery,  KS Hegde Medical Academy, Mangalore)    ¢ Excision of the tumour can be done without sacrificing the  nerves because the tumour is well encapsulated anc  displaces the nerve.    ¢ It 1s a tumour-like developmental malformation of the  tissues of a particular part of the body wherein it is arranged  haphazardly.   ¢ Hamartoma ts a Greek word which means fault or misfire.  It is not a clinical diagnosis.   Fig. 15.21: The café au /ait spots. More than five such spots will   appear by early life (Courtesy: Dr Prashanth Shetty, Professor, Characteristic features   KMC, Manipal) ¢ Being a developmental anomaly, they are seen at birth or  in early childhood.   ¢ Being a malformation (not a tumour), it does not have  a capsule.    ¢ Excision is not only curative but also gives a correct  diagnosis.   ¢ Care should be taken when it contains vascular tissue such  as haemangioma or neural tissue as in cases of neuro-  fibroma.   ¢ Facial nerve and its branches may be damaged while  excising hamartomatous lesions over the face.   Kasturba Hospital, Manipal. Initially it was diagnosed as solitary ° Remnant of notochord (origin)  nodule of the thyroid gland but it was not moving with deglutition) * Sacrococcygeal region (common site)    ¢ They are of two types: Carcinoma and sarcoma. Carcinoma  arises from epithelium—ectodermal, endodermal or  mesodermal in origin.   ¢ Sarcomas arise from soft tissues or bone and are derived  from mesoblast or mesenchymal tissues.   ¢ It may be observed that mesoderm can give rise to  carcinoma and mesenchymal sarcoma also.    1. Local spread: Generally, local spread occurs into adjacent  structures. Few examples are given:    2. Lymphatic spread: [tis one of the most important features  of carcinoma (Key Box 15.5). As you complete reading  this book, you will come across many cases and many  examples of lymphatic spread of malignant tumours. A few  sarcomas also spread by lymphatics (see page 225).  Different types of lymphatic spread are given below:   ¢ Permeation: Refers to tumour cells travelling along  the lymphatic vessel, e.g. carcinoma tongue with sub-  mandibular node enlargement (Fig. 15.23C).    counterparts  2. Undifferentiated (anaplasia) Loss of structural and functional differentiation  3. Dysplasia Loss in the uniformity, loss in the architectural    ¢ Retrograde lymphatic spread: When main lymphatic  pathway 1s blocked retrograde spread can occur and a  node in an unusual location may get enlarged, e.g. Irish  node (left axillary node enlargement in carcinoma  stomach).    3. Haematogenous spread: This is most important method  of spread of sarcomas. Also, a few malignancies such as  renal cell carcinoma, follicular carcinoma thyroid,  carcinoma prostate, carcinoma breast (Fig.15.23D) and  malignant melanoma commonly spread by blood. Bony  metastasis and lung metastasis results from blood spread.  Bone metastasis can vary from mild form with only bone  pain to severe form with quadriplegia/pathological fracture.    4. Transcoelomic spread: Spread through peritoneal cavity  by dislodgment of malignant cells, e.g. Ca stomach with  Krukenberg’s tumour—Bilateral bulky ovarian metastasis,  commonly seen in premenopausal patients (Fig. 15.23E).    5. Seeding (Fig. 15.23F): A few examples are given below:  ¢ Cancer of lower lip spreading to upper lip, also called  kiss cancer. Other example: Cancer of vulva.  ¢ Incision and ‘port’ site metastasis (port refers to  laparoscopic port).   Certain cancers produce some specific clinical syndrome  (symptom complexes other than cachexia) which cannot bi  explained by their local or distant spread or by the hormone  produced by the tissue of origin of these tumours. These art  called Paraneoplastic syndromes (Key Box 15.6).   Just to give an example, hypercalcaemia due to skeleta  metastasis from carcinoma breast is not considered as PNS  but if it occurs without skeletal metastasis, it is considered a:  PNS.   IMPORTANT FEATURES OF PNS  e Incidence : 10-15% of patients with cancer    e |t may be the earliest manifestation (primary can be occult)  e Bronchogenic cancer and breast cancer are most  commonly associated with PNS.    e Hypercalcaemia and Cushing’s syndromes are the  most common clinical syndromes associated with  PNS.    e PNS can be a major clinical problem and can be treated.    1. Tobacco is the most important factor 1n the development  of lung cancer, upper respiratory tract cancer,  gastrointestinal tract and genitourinary tract cancer.  Carcinoma pancreas is found more commonly in smokers.  Passive smokers also have increased incidence of  development of cancers.    ACTH or ACTH-like substance  ADH or atrial natriuretic factor  Insulin or insulin-like substance  PTH like substance    3. Bone and soft tissues  * Hypertrophic osteoarthropathy  and clubbing of fingers  4. Vascular  * Venous thrombosis    2. Alcohol: Smoking with alcohol increases the permeability  of the upper digestive tract mucosa and respiratory mucosa  to the carcinogens. Thus, they increase the incidence of  cancer. Hepatocellular cancer is commonly found in  alcoholic cirrhotic liver.    3. Ionising radiation: Atomic bomb blasts in Japan have  definitely resulted in increased number of cases of breast  cancer in premenopausal women and leukaemia in children.    4. Ultraviolet radiation: Causes all types of skin cancers.  5. Genetic causes (Key Box 15.7)  6. Hereditary causes  ¢ MEN syndrome: Medullary carcinoma thyroid  (Multiple Endocrine Neoplasia)  ¢ FPC: Colonic cancer (Multiple)  (Familial Polyposis Colt)    1. Xeroderma pigmentosa : Skin cancer  2. Bloom’s syndrome : Acute leukaemia, various  cancers    ¢ Li-Fraumeni syndrome: Familial breast cancer.  ¢ Retinoblastoma.  7. Dietary factors  ¢ Red meat: Carcinoma colon, carcinoma breast.  ¢ Fat: Carcinoma breast, carcinoma colon.  ¢ Smoked, charred fish: Carcinoma oesophagus,  carcinoma stomach.  8. Chemicals  ¢ Benzanthracenes: Skin cancer when painted on the skin  ¢ Benzopyrenes: Lung cancer  ¢ B-naphthylamine: Bladder cancer  ¢ Nitrosamines and amides: Cancer stomach  ¢ Aflatoxin B: Hepatocellular carcinoma  ¢ Asbestos: Lung cancer  9. Viral factors  ¢ Human T-cell leukaemia virus type 1 (HTLV-1): T cell  leukaemia/lymphoma. (RNA virus)  ¢ Human papillomavirus (HPV)  Cancer cervix, cancer urogenital region  ¢ Epstein-Barr virus: Burkitt’s lymphoma.    These are malignant tumours arising from soft tissues. Thus,  they can occur in any part of the body. Examples are given in  Table 15.6.    These are malignant tumours which are fatal if untreated  or mistreated. Most of them occur in young patients as  painless lumps. CT scan, MRI and incision biopsy (details  later) are key investigations. Early diagnosis and curative  resection have a major role in the management of soft tissue  sarcomas. In addition to TNM staging, pathological grading  of the tumour has been included GTNM staging.    Genetic factors: Genetic mutations/gene rearrange-  ments have been implicated in the pathogenesis of STS.  Two genes that are most relevant to soft tissue tumours are  retinoblastoma (Rb) tumour suppressor gene and p53  tumour suppressor gene. Various familial syndromes are  associated with STS such as Li-Fraumeni syndrome,  Gardner’s syndrome and von Recklinghausen’s disease.  Several oncogenes have been identified.    incidence of STS is reported in patients who are treated for  cancer of the breast, cervix and ovary by radiation.    factor for development of STS. Others believe that trauma  draws attention of the patient to the STS.   1. Grade (G)  G1 — Well differentiated  G2 — Moderately differentiated  G3 — Poorly differentiated  G4 — Undifferentiated    2. Primary Tumour (T)  a: Superficial tumour  b: Deep tumour  T1: Tumour < 4—5 cm in greatest dimension  T2: Tumour > 5 cm in greatest dimension  3. Regional lymph nodes (N)  NO : No nodal metastasis  Ni : Regional lymph node metastasis present.  4. Distant Metastasis (M)  MoO : No distant metastasis  M1: Distant metastasis present  G — TNM staging system  Stage grouping    ¢ Routine blood tests  ¢ Chest radiography: Presence of cannon ball metastasis  alters the staging, treatment policy and prognosis.    If STS is more than 5 cm (12), computed tomography  (CT) of the chest should be considered.    ¢ CT scan is useful in evaluating retroperitoneal sarcomas.  It can define structures, infiltration into neighbouring  structures, hydronephrosis, etc. CT can guide a core biopsy  also.   4. Presentation Nonhealing ulcer, cauliflower-like growth with Fleshy mass, red and vascular, dilated veins over the  everted edges and induration. Fixity 1s a late fea- surface, local rise of temperature. Ulceration is a late  ture feature   5. Spread Lymphatic spread is very common, both by em- Blood spread occurs very early and results in cannon  boli and permeation. Blood spread does occur as ball secondaries in lung. Rhabdomyosarcoma, synovial  in renal cell carcinoma, thyroid carcinoma, breast sarcoma, epithelioid sarcoma. Malignant fibrous his-    6. Microscopy Cell nests or epithelial pearls are seen in well- Malignant cells resemble their cel! of origin. Thus,  differentiated cancers spindle-shaped cells are found in fibrosarcoma   7. Treatment Surgery is the main treatment of ectodermal Surgery is the main modality of treatment. Radio-  cancers, surgery or radiotherapy for ectodermal therapy and chemotherapy are also beneficial when the    ¢ MRI is the investigation of choice when STS occurs in  extremities to delineate muscle groups, bones, vascular  structures, etc.  ¢ Biopsy: FNA  — Acceptable first investigation to prove the diagnosis.  — Useful to detect metastatic disease  — To detect local recurrence  — Ideal for superficial lesions  — Disadvantage: Cannot assess tumour grading. Tissue  is not sufficient for diagnostic tests.  ¢ Core needle biopsy  — Safe and accurate  — Tissue is sufficient for grading, electron microscopy  and flow cytometry  — With CT guidance, core biopsy can be taken from deeper  structures also.  ¢ Incisional biopsy  — When core biopsy tissue is not adequate, incisional biopsy  is indicated (Key Box 15.9).   e Incision should be oriented longitudinally in STS of  extremities to facilitate removal of biopsy site, scar and  tumour en bloc    The aim is to achieve local control and to treat metastasis  including subclinical metastasis, thus trying for a cure.   Surgery is the first line of treatment varying from a wide  local excision to amputation or disarticulation (5%), when it  occurs in the extremities. Low-grade tumours can be  treated on 2 cm wide excision of surrounding normal tissue  and high-grade tumours by 4 cm margin (Key Box 15.10).  Small tumours less than 5 cm have not been associated  with recurrence. Hence, radiotherapy may not be required  but if grade is high, RT is required (Key Box 15.11).    High-grade tumours have high potential of metastasis. Hence,  combination chemotherapy is to be considered before or  after surgery.   The most favoured combination chemotherapy drugs  include Mesna, Adriamycin, Ifosfamide and Dacarbazine  (MAID).   Fig. 15.24: A 24-year-old lady underwent excision of a swelling  diagnosed as lipoma in a peripheral hospital which was reported  as sarcoma   Fig. 15.27: Alarge (8 cm) liposarcoma of the thigh being explorec  with a longitudinal incision of the thigh. He had undergone an  incision biopsy earlier. The biopsy site is removed along with the  skin    Dr Ankur Sharma, Asst Professor, Dept. of Surgery, KMC, Manipal) = excision. No nodes were palpable in the leg    SURGERY FOR SOFT TISSUE SARCOMA  Surgery is the primary and most effective therapy  Local wide excision with 2 cm to 4 cm of Surrounding normal  tissue should be removed. 2 cm for low grade and 4 cm  for high grade tumour.   Not to dissect along pseudocapsule which is composed  of tumour cells.   lf necessary, excision should include nerves and vessels  followed by nerve grafts and arterial reconstruction.   STS rarely involve bones and skin. Hence, wide resections  of these structures are infrequently necessary.   If amputation can be avoided by giving preoperative  radiotherapy, it is preferred first. it is then followed by wide  excision/compartmental excision and postoperative  radiotherapy. Dose of pre- and postoperative radiotherapy  is 50-60 Gy given in 25 fractions. Brachytherapy is also given.  Margin negative resection should be the aim.    Almost 80% of STS metastasise to lungs within 2—3 years  of the diagnosis.   Prognosis depends on metastatic STS, grade of the tumour,  size of the tumour, margins after resection and anatomical  location.   If pulmonary metastasis 1s resectable, 30% survival may  be expected at 3 years.   They are generally large at the time of diagnosis, e.g.  retroperitoneum. It results in gross swelling, which is firm  to hard (more than 50% will be of > 20 cm size) (Key Box  13.12)    RADIOTHERAPY  It is not the first modality of treatment in STS.  Radiotherapy is given after margin negative resection.  Surgery + RT has resulted in local control rates up to or  more than 90%.    External beam radiation therapy (electrons, protons or  neutrons) is given with a margin of 5 to 7 cm or even more  depending on size of tumour. Dose is 50 Gy given in 25  fractions—as preoperative dose.   In brachytherapy, multiple catheters are placed in tumour  resection bed. Catheters are loaded with seeds containing  lridium—192.    Dose of brachytherapy is 42-45 Gy to tumour bed over  4-6 days.   Short period of treatment time and less systemic toxicity are  advantages of brachytherapy.    The compression on blood vessels may result in oedema of  the limbs when it occurs in retroperitoneum.  Well-differentiated myxoid liposarcomas are notoriously  known to recur many times before spreading to lungs.  Hence, prognosis is good (Fig. 15.42).   Pleomorphic and lipoblastic liposarcomas tend to be of  higher grade and often present with metastasis.   RETROPERITONEAL SARCOMA (RPS)  Most common FPS is liposarcoma or leiomyosarcoma.  They constitute 15% of adult soft tissue sarcomas.    May present as large masses of more than 20 cm at the  time of presentation.    Progressive abdominaldistension, pedal oedema, young age  and firm to hard retroperitoneal mass clinches the diagnosis.    CT scan of chest and abdomen followed by FNAC/True cut  biopsy for histology/grade of the tumour.    It is a malignant tumour of mesenchymal tissue (fibrous  tissue). This is the recent nomenclature of sarcoma.    Fibrosarcoma or pleomorphic rhabdomyosarcomas are  included under this. Most of the so-called fibrosarcomas  are presently included under MFH (Fig. 15.31).   These are high-grade tumours that lack differentiation.  It can also arise from bone.   Locations: Retroperitoneum, trunks and limbs (inter-  muscular septae of adductors, scapulohumeral and pectoral  muscles).    Common in elderly patients (50 years) but can occur at any  age.   Slow-growing, firm to hard mass with restricted mobility.  As the tumour ts locally invasive, it infiltrates the muscles  and adjacent structures. Thus, it can cause muscle weakness  or pain, etc.   Spread: Local spread is common. Distant metastasis by  blood is late (lungs). Lymph node metastasis Is rare.   Like other sarcomas, dilated veins, local rise of temperature,  restricted mobility and hardness will clinch the diagnosis.  MRI is the investigation of choice to know the extent of  the disease (Figs 15.32 to 15.35).    Any rapidly growing tumour in the region of joint or near  the tendons in young patients (20-40 years), synovial  sarcoma 1s to be considered.   In addition to the local and blood spread, it also spreads  by lymphatic route.   Figs 15.32 to 15.35: T1- and T2-weighted (W) axial MRI. lsointense  to hyperintense mass lesion noted in the anterior compartment of  right thigh. Sagittal T2-W MRI shows supero-inferior extent of the  lesion    ¢ Plain X-ray: It may show characteristic calcification.  ¢ [tis aggressive, with high rates of recurrence.  ¢ In the G-TNM staging system, they are grade 3.   ¢ They arise from skin and subcutaneous tissue rather than  deeper tissues.   ¢ Most of them occur in the head and neck, breast and liver.   ¢ Surgery (excision) followed by radiotherapy/combination  chemotherapy may have to be given.    ¢ Itis the most common soft tissue sarcoma seen in children,  even though they are rare (under the age of 15).   ¢ Sites: Head and neck (30%), genitourinary system (25%),  extremities (20%).   Fig. 15.42: Six times recurrent myxoid liposarcoma since 8 years.  It has good prognosis (Courtesy: Dr Shashi, HOD, Surgery, Calicut    Fig. 15.38: Swelling in the  elbow region for two years  duration presented to the  hospital with bleeding and  fungation—advanced case of  epithelioid sarcoma   Fig. 15.39: Ulceratedlesion in the  elbow region of two years duration  presented to the hospital with  bleeding—a case of dermatofibro-  sarcoma protuberans    Fig. 15.43: Spindle cell sarcoma (Courtesy: Dr Laxmi Rao, Head  of the Dept. Pathology, KMC, Manipal)    ¢ Complete tumour resection should be the aim. Chemo-  therapy and RT are also used.    ¢ Vulnerable section of people include Jews, immunocom-  promised patients such as transplant recipients and AIDS.   ¢ Typical sites: Legs. Other sites include chest, arm, neck in  epidemic form (Africa).   ¢ It manifests with purplish to red subcutaneous nodules in  the leg followed by ulceration and bleeding.   ¢ Combination chemotherapy with doxorubicin, etoposide  and interferon have been used to control the disease.   e Wide excision should be the aim with negative margin to  prevent local recurrence.   ¢ Mohs’ micrographic surgery such as basal cell carcinoma  has been advocated to get negative margin and thus to get  a low recurrence rates.   In undergraduate clinical examination, students are advised  to offer soft tissue sarcoma as the diagnosis. When asked the  possible type, they only give a possible histological type based  on various clinical features mentioned above. Ask following  questions to yourself to get ready for the clinical exams.    1. Is it soft tissue sarcoma? Tumour arising from soft tissue,  dilated veins, reddish skin, increase in local temperature,  firm to hard, rapidly growing swelling, with late  involvement of skin (carcinoma starts in the skin) (Key  Box 15.13).   2. What is the age of the patient?  ¢ In children — Rhabdomyosarcoma   ¢ Head and neck — Angiosarcoma  Rhabdomyosarcoma  Osteogenic sarcoma (jaw)  Synovial sarcoma (Fig. 15.25)  Epithelioid sarcoma (Fig. 15.38  Clear cell sarcoma   ¢ Epithelioid sarcoma  5. Has it spread to lungs or liver?   e Most common soft tissue sarcoma in adults is MFH (28%).   e Most of the extremity soft tissue sarcoma metastasise to  lungs.   e Most of the retroperitoneal soft tissue sarcoma  metastasise to liver.   e Most important prognostic factor for soft tissue sarcoma is  size and grade of the primary tumour.   e Most accurate imaging modality for soft tissue sarcoma is  MRI.   e Most effective modality of treatment is surgery.  e Most effective chemotherapeutic drugs are doxorubicin,  dacarbazine and ifosfamide.    CLINICAL NOTE!  A 34-year-old nondiabetic male patient had a nonhealing ulcer  great toe (RT) of 6 months duration. History of minor trauma  was present. Examination revealed ulceration, thickening and  induration with ‘mass’ lesion. It was MBBS exam case. The  student offered osteomyelitis, foreign body granuloma and  tuberculous synovitis as diagnosis. He qualified but he was asked  to examine the groin. The patient had 3 hard nodes in the groin.  The final diagnosis—Synovial cell sarcoma with metastasis in  the groin nodes—the student had not examined the groin!    . Which one of the following does not have a capsule?  A. Hamartoma B. Schwannoma  C. Fibroadenoma D. Branchial cyst    . Which one of the following is commonly associated  with paraneoplastic syndrome?    . Which one of the following condition is more commonly  associated with paraneoplastic syndrome?    Polycythaemia is a paraneoplastic syndrome seen in  which condition?  A. Hepatoma  C. Apudoma    Precautions to be taken while doing a open biopsy in  soft tissue sarcoma include following except:    Following are true for the treatment of soft tissue  sarcomas except:    ANSWERS  10 2A 3 A 4D 5 D 6D 7A 8 D 9C 10 BD  11A 12 0 13 D 14D 15 B he} D 17 A    e Swellings in the carotid triangle  e Swellings in the posterior triangle    A cyst is a swelling containing fluid. True cysts are lined by  endothelium or epithelium. They contain clear serous fluid,  mucoid material, pus, blood, lymph or toothpaste like material.   The false cysts do not have lining epithelium. They can be  degenerative cysts as in the case of tumours which undergo  tumour necrosis or tumour degeneration, or merely a collection  of fluid which is walled off by coils of bowel as in tuberculous  encysted ascites or an exudation cyst as in pseudopancreatic  cyst.    Classification of cyst  I. Congenital cyst  ¢ Sequestration dermoid cyst  ¢ Branchial cyst  ¢ Thyroglossal cyst  ¢ Lymphangioma  ¢ Cysts of embryonic remnants: Cyst of urachus, vitello-  intestinal duct cyst  Il. Acquired cyst  ¢ Retention cyst: Sebaceous cyst, galactocoele, spermato-  coele, Bartholin’s gland cyst  ¢ Distension cyst: Thyroid cyst, ovarian cyst  ¢ Exudation cyst: Hydrocoele  ¢ Degenerative cyst: Tumour necrosis    Students are requested to follow the standard practice of  examination of the swelling in the form of inspection, palpation,  percussion and auscultation in the clinical examination. Some  Important tests for cystic swellings are given in the next page.    1. Location: Most of the congenital cystic swellings have a  typical location wherein diagnosis can be made with fair  accuracy. A few examples are as follows:  ¢ Branchial cyst occurs at the junction of upper one-third  and lower two-thirds of the sternocleidomastoid muscle  whereas opening of branchial fistula occurs at junction  of upper two-thirds and lower one-third of sterno-  cleidomastoid muscle.   ¢ Dermoid cyst: Midline, outer or inner canthus of the  eye    Fig. 16.1: Fluctuation should be Fig. 16.2: Transillumination  elicited using both hands and in test: It should be done in a  both directions dark room   ¢ Subhyoid bursitis: Transverse oval cystic swelling in  the midline of the neck.   ¢ Thyroglossal cyst: Vertically placed oval swelling in  the midline of the neck.    Surface: Almost all the cystic swellings in the skin and  subcutaneous tissue have smooth surface.   ¢ Tensely cystic: Ganglion, tensely cystic swellings in the  neck may feel firm or solid, e.g. tense thyroid cyst. Cyst  in the breast may feel firm or hard.   ¢ Yielding in cases of lipoma, as fat at body temperature  behaves like fluid (pseudofluctuation).   ¢ Cross fluctuation for swellings having two components  connected to each other, e.g. plunging ranula.   as a rule, they should have free mobility. However, this is   ¢ Branchial cyst: Restricted mobility is due to its  adherence to the stemomastoid muscle.   ¢ Thyroglossal cyst: Transverse mobility 1s absent because  the cyst 1s tethered by remnant of the thyroglossal duct.   ¢ Sebaceous cyst: Limited mobility due to the adherence  to the skin.    communication with a cavity or with tissue spaces give the  positive sign of compressibility. Thus, a steady pressure is   ¢ Almost all significant cystic swellings in the neck are  deep to deep fascia. Thus, contracting sternomastoid for  laterally placed swellings and bending the chin against  resistance for centrally placed swellings must be done  to define the plane of swelling.   ¢ Subcutaneous swellings become more prominent when  the underlying muscles are contracted as 1n limbs.   ¢ Swelling due to semimembranous bursitis, almost dis-  appears on flexion of knee and becomes more prominent  on extension of the knee.   ¢ Sebaceous cysts are attached to the skin at the site of  punctum.    a. Expansile: Aneurysms are characterised by expansile  pulsations. When two fingers are placed over the swelling  on the sides, the fingers are not only elevated but are  also separated. Popliteal aneurysms typically give this sign  (Key Box 16.3).   b. Transmitted: When the swelling Is situated over a vessel,  the fingers are raised but not separated, e.g. pseudo-  pancreatic cysts. When the swelling pushes the vessel  anteriorly, transmitted pulsation can be obtained, e.g.  cervical rib pushing the subclavian artery.   c. Pulsation can also be present 1n vascular tumours such  as osteogenic sarcoma or secondaries from carcinoma  thyroid, etc.   RULES OF ELICITATION OF FLUCTUATION y  ¢ Mobile swelling has to be fixed.   e With the index finger and thumb of one hand the swelling is  pressed—these are ‘active’ fingers and the impulse is  received by the thumb and index finger of the other hand  (passive fingers).   e Fluctuation should be elicited in both directions, as fleshy  muscle in the thigh can be fluctuant across but not in the  longitudinal direction.   e When swelling is smaller than 2 cm in size, Paget’s test is  done. Cystic swellings feel soft in the centre and firm at the  periphery. Solid swellings feel firm at the centre than  periphery.   e Thrill and bruit are present and distal pulses may be weak.   EFFECTS OF ANEURYSM: TIPS &  e Thrombosis, [schaemia  e Pressure, Skin changes    1. Thrombosis: It 1s one of the common effects of aneurysm  particularly aortic and popliteal resulting in ischaemia in  the distal territory.    2. Ischaemia: Distal parts may become gangrenous and or  can have ischaemic ulcers or claudication.    a. Effect on bone: Erosion of the vertebral body as in aortic  aneurysm. This does not happen in TB spine.    b. Effect on nerves: Popliteal aneurysm can give rise to  foot drop due to pressure on lateral popliteal nerve.    c. Effect on the veins: Results in congestion and oedema  of the leg.    4. Skin changes may be in the form of oedema and redness.    . Ovarian cachexia: Large ovarian tumour with pedal  oedema, anorexia, loss of weight, lordosis.    This 1s a cyst lined by squamous epithelium containing  desquamated cells. The contents are thick and sometimes  toothpaste-like which 1s a mixture of sweat, sebum and  desquamated epithelial cells and sometimes even hair.    ¢ They occur along the line of embryonic fusion, due to  dermal cells being buried in deeper plane.   ¢ The cells which are sequestrated in the subcutaneous plane  proliferate and liquefy to form a cyst.   ¢ As it grows, it indents the mesoderm (future bone) which  explains the bony defects caused by dermoid cyst in the  skull or facial bones.   ¢ Even though they are congenital, they manifest as a swelling  during childhood or later in life. Often they can be mistaken  for lipoma and sebaceous cysts (Fig. 16.5).   ¢ They can occur anywhere in the midline of the body or the  face (Key Box 16.5)    |. External and internal angular dermoid cyst: At the fusion  lines of frontonasal and maxillary processes (Fig. 16.6).   . Median nasal dermoid cyst: At the root of the nose at  the fusion lines of frontal process (Fig. 16.7).    Pinna is formed by the fusion of 6 cutaneous tubercles.  Both preauricular and postauricular dermoid cysts occur  because of failure of fusion of one of the tubercles with  the others as they form pinna.    e The face is developed from 5 processes—2 maxillary,  2 mandibular and 1 frontonasal process.   e Dermoid cyst occurs in the line of embryonic fusion of these  processes.    ¢ Though congenital, the cyst manifests in childhood or  during adolescence. A few cases also manifest in 30-40  years age group.   ¢ The underlying bony defect gives the clue to the diagnosis.   ¢ Classical location of the cyst (along the line of fusion) is a  feature of sequestration dermoid cyst.   ¢ Following a sharp injury, few epidermal cells get  implanted into the subcutaneous plane. There, they develop  into an implantation dermoid cyst. Hence, it is typically  found in the fingers, palm and sole of the foot. As the cyst  develops in the areas where the skin is thick and keratinised,  it feels firm to hard in consistency.    ¢ Teratoma Is a tumour arising from totipotential cells. Thus,  it contains ectodermal, endodermal and mesodermal  elements—harr, teeth, cartilage, bone, etc.    Fig. 16.12: Ovarian teratomatous dermoid cyst. Usually  they are bilateral—large ones with pedicles are  vulnerable for torsion (Courtesy: Dr Rajesh Bhakta,  Associate Professor, Dept. of OBG, KMC, Manipal)   A 24-year-old female patient was admitted with acute lower  abdominal pain of 2 days’ duration. There was guarding and  rigidity of the abdominal wall with rebound tenderness. Per  vaginal examination was normal. At exploration, there was  a twisted ovarian teratoma on the left side with gangrene. It  was excised. The opposite ovary, on careful examination  revealed a small teratomatous dermoid cyst which could be  enucleated. The significance of this case report lies in the  fact that both ovaries should be examined in cases of ovarian  dermoid cysts.   ¢ They arise from ectodermal tubes. A few examples are  thyroglossal cyst, post-anal dermoid cyst.    e This is popularly called sebaceous cyst. It is a misnomer.  This occurs due to obstruction to one of the sebaceous ducts,  resulting in accumulation of sebaceous material. Hence,  this is an example of retention cyst.   ¢ Sites: Scalp, face, back, scrotum, etc. Jt does not occur in  palm and sole, where sebaceous glands are absent. In the  back, scalp and scrotum (Figs 16.15 to 16.19), multiple  cysts are often found.    ¢ They are slow-growing and appear in early adulthood or  middle age.    ¢ Hemispherical or spherical swelling located in the dermis.  A dark spot in the centre (punctum) filled with keratin is a  diagnostic feature of this cyst. The punctum indicates  blockage of the duct (Fig. 16.14).   ¢ In 20-30% of cases, instead of opening into the skin,  sebaceous duct opens into the hair follicle. Hence, punctum  is not seen.   ¢ It has a smooth surface, round borders, soft and putty  consistency and is nontender.   ¢ The cyst can be moulded into different shapes which is  described as sign of moulding.   ¢ The swelling is mobile over the deep structures, and the  skin is free all around except an area of adherence at the  site of punctum.   ¢ In the scalp, loss of hair is a feature over the swelling  because of constant slow expansion of the cyst.    ¢ Incision and avulsion of cyst with the wall. Very often,  during dissection, the cyst wall ruptures. Care should be  taken to excise the entire cyst wall. If not, recurrence can  occur.    ¢ When it is small it can be excised along with the skin.    1. Infection can occur due to injury or scratch resulting in an  abscess. The cyst will be tender, red and warm to touch. It  should be treated like an abscess by incision and drainage.  After one to two months, the cyst can be excised.   2. Sebaceous horn results due to slow drying of the contents  which are squeezed out, specially if a patient does not wash  the part. Thus, it is not common to find a large sebaceous  horn nowadays because of better ways of living and  sanitation (Fig. 16.20).    Fig. 16.16: Multiple seba- Fig. 16.17: Multiple seba- Fig. 16.18: Strawberry Fig. 16.19: Sebaceous cyst on the   ¢ Location Midline of the body, along the line of fusion Face, scalp, scrotum, back    5. Rarely, basal cell carcinoma can arise in a long-standing  sebaceous cyst.    'Pott’s puffy tumour refers to osteomyelitis of the frontal bone with oedema of scalp secondary to frontal sinusitis. This and Cock’s peculiar  tumour are favourite viva questions.    Meibomian cyst: They are epidermal cysts found on the  free edge of eyelid. Chronic Meibomian cyst is called  chalazion.    [It is a tense, cystic swelling and occurs due to myxomatous  degeneration of the synovial sheath lining the joint or tendon  sheath. They are common around joints because of abundant  fibrous tissue. They contain gelatinous fluid.    ¢ The dorsum of the hand is the common site, at the  scapholunate articulation.   ¢ A round to oval swelling in the dorsum of the hand, with  smooth surface and round borders. Skin over the swelling  is normal.   e The swelling is tensely cystic and fluctuant. Trans-  illumination is negative. It is mobile in the transverse  direction.   ¢ When the tendons are put into contraction, the mobility of  the swelling gets restricted.   ¢ Ganglion is not connected with the joint space. Sometimes,  it gives an impression of becoming small due to slipping  away between bones.   2. Aspiration of the ganglion and injection of sclerosants may  reduce the size of ganglion.   3. Sometimes, rupture of the cyst due to trauma may result in  permanent cure.   4. Surgical excision can be done. However, recurrence rate  is high.    1. Implantation dermoid cyst, when it occurs in the feet or  hand.   2. Exostosis of the bone, has to be considered if swelling is  very hard.    ¢ Tuberculous tenosynovitis of the tendon sheaths affecting  the flexor tendons. This is a common cause in India (Key  Box 16.7)    ¢ Rheumatoid arthritis with involvement of multiple joints  causing thickening of synovial membrane—common cause  in Western countries.    ¢ Asa result of tuberculous tenosynovitis, typical caseous  material collects within the flexor tendon sheaths. The  tendons get matted, a swelling develops in the palm and  another swelling develops in lower aspect of forearm. The  thickening of synovial membrane, fibrin particles in the  fluid and melon seeds, are characteristic of this condition.   ¢ Soft, cystic, fluctuant, transillumination—negative swelling  situated above and below the flexor retinaculum.   ¢ Cross fluctuation test between these two swellings is  positive, which is diagnostic of compound palmar ganglion.   ¢ Restricted mobility of the fingers due to matting of the  tendons.    1. The ESR may be increased if it is due to tuberculosis.   2. Aspiration of the swelling and fluid can be sent for acid-  fast bacilli.    1. Antituberculous treatment (ATT) in case of tubercular  pathology. If the response rate is not satisfactory—  exploration, decompression, synovectomy and release of  matted tendons is the treatment.   2. Control of rheumatoid arthritis, with complete excision  of the synovial sheath, in cases due to rheumatoid arthritis.   ¢ Decompression or synovectomy may be required in both  conditions mentioned above.   ¢ This is also called glomangioma or angioneuromyoma.  ¢ Glomus ts a specialised organ.    Abundant arteriovenous anastomosis surrounded by large clear  cells (glomus cells) and medullated and non-medullated nerve  fibres in between the cells is characteristic of glomus.    1. Typical site: Under the nail beds of hands and feet.   2. It is purple red in colour, usually single, the size does not   4. Excruciating pain either at rest or on movement of the finger  or on pressure 1s pathognomonic feature of this tumour.  Pain is due to compression of the nerve fibres by dilated  glomus vessels.   Figs 16.21 and 16.22: Glomus tumour: The most painful condition  in the finger (Courtesy: Prof Bhaskarananda Kumar and Dr Anil  Bhat, Department of Orthopaedics, KMC, Manipal)   Anatomical site  1. Prepatellar bursa  In front of patella tendon (infrapatellar)    Under the insertion of tendons of sartorius, gracilis and semiten-  dinosus muscle    5. Between the tendon of the semimembranosus and the medial  condyle of tibia    GLOMUS TUMOUR o  e Rare and benign tumour  e The most painful tumour  e The smallest benign tumour, does not turn malignant  e Nail bed is the commonest site  e Histologically, it is an angioneuromyoma  e It is radioresistant  e Excision gives permanent cure  e Function of glomus is concerned with heat regulation.   2. Granuloma pyogenicum: Mild pain, bleeds on touch and  evidence of infection is present.    ¢ Bursa means a sac ora sac-like cavity containing fluid lined  by endothelium. It is meant to reduce the friction between  tendons of the muscle and the bone.    ¢ Bursitis refers to inflammation of a bursa resulting in  accumulation of excessive fluid inside the bursa. This results  in a swelling in the anatomical sites of normal bursa.    ¢ The causes of chronic bursitis includes constant pressure,  constant irritation or minor injuries.    Bursa anserina (extension of the bursa along the sides of tendon-  resembles goose’s foot)    ¢ Bursitis produces a soft, cystic, circumscribed or oval  swelling with fluctuation.    ¢ As majority of bursitis contains inflammatory fluid, they  do not show transillumination.    ¢ Excision is indicated only in the presence of symptoms such  as pain or complications mentioned above.   SEMIMEMBRANOSUS BURSA (Figs 16.23 to 16.25) Fig. 16.24: Constant pressure on the lateral aspect of foot   This is the commonest  swelling in the popliteal  Space. It presents as a  tensely cystic swelling  when the knee is extended  and it becomes flaccid on  flexion of the knee. It is not  compressible as it does not  communicate with the joint.   The differential diag-  nosis for semimembrano-  sus bursitis is Morrant-  Baker’s cyst, which is a  herniation of the synovial  membrane. The differences  between these two swellings Fig. 16.23: Popliteal fossa swelling  are given in Table 16.3.   Adventitious bursae  e This refers to a cyst which develops in an anatomical area  where no bursa is present. These also occur due to constant    pressure or friction. They are summarised below. Figs 16.25A and B: (A) Semimembranosus cyst and (B) cyst  1. Tailor’s ankle: Above the lateral malleolus disappears on flexing    Semimembranosus bursa Baker’s cyst  1. Aetiology Friction or pressure Rheumatoid or osteoarthrosis of knee joint  2. Age Young patients Middle aged  3. Location in the popliteal fossa Higher up and more medial Below and midline  4. On flexion of the knee Disappears Increases  5. On extension of the knee Appears and is tense (Figs 16.25A and B) Diminishes  6. Patellar tap Absent Present  7. Compressibility Absent Present partially  8. Knee movements Normal Restricted    2. Porter’s shoulder: Between clavicle and skin  3. Weaver’s bottom: Between gluteus maximus and ischial  tuberosity  4. Bunion: Between prominent head of the first metatarsal  and skin due to hallux valgus  ¢ The complications and treatment of adventitious bursae are  similar to chronic bursitis (Fig. 16.24)    These are the cystic swellings containing clear fluid  characterised by fluctuation and transillumination.   ¢ Failure of one of the lymphatics to join the major lymph  sac of the body results in a lymphangioma. Hence, it occurs  in places where lymphatics are abundant.   ¢ They are dilated lymphatics that project onto the skin  surface.   ¢ Common sites: Posterior triangle of the neck, axilla,  mediastinum, groin, etc. (Key Box 16.8).   ¢ In the neck, it 1s called cystic hygroma of the neck. As the  sac has no communication with lymphatics by the time  swelling appears, the lymph is absorbed and 1s replaced by  thin watery fluid (mucus) secreted by endothelium. Hence,  it is also called hydrocoele of the neck.   ¢ When it is largely confined to subcutaneous plane, it is  called cystic hygroma.   Fig. 16.26: Lymphangioma involving chest wall,  neck. The two were different swellings   LYMPHANGIOMA SITES  e Jugular lymph sac Neck  e Posterior lymph sac Groin  e Cisterna chyli Retroperitoneum    1. Lymphangioma circumscriptum: If it 1s less than 5 cm  across.    3. Lymphoedema ab igne: If they form a reticulate pattern  of ridges.    ¢ Usually, cystic hygroma presents during infancy or early  childhood. Occasionally, present since birth and rarely  before birth. They can also present as small vesicles.    ¢ When the child cries or strains the swelling increases  in size and becomes prominent due to increased intra-  thoracic pressure which is transmitted through root of the  neck.    ¢ Soft, cystic, fluctuant, partially compressible swelling.  Lymphangioma is a multilocular swelling consisting of  aggregation of multiple cysts. These cysts may inter-  communicate and may occasionally insinuate between  muscle planes. Hence, it gives the sign of compressibility!  However, complete reducibility is not a feature.   TRANSILLUMINATION TEST Ys  Should be done in a dark room  Avoid surface transillumination    Surgical excision is the treatment of choice. All the loculi  or cysts should be removed. Careful search has to be made  for the extension of lymphangioma through the muscle  planes so as to avoid recurrence (Figs 16.29 and 16.30).  Sclerotherapy was being used earlier for lymphangioma.    2. Lipoma: This 1s a soft lobular swelling with fluctuation  because fat behaves like fluid at body temperature. However,  the edge slips under the palpating fingers. Both trans-  illumination and compressibility tests are negative with  lipoma.    1. In neonates and infants, lymphangioma can cause difficulty  in breathing due to its large size.   3. Lymphangioma in the mediastinum can give rise to  dyspnoea, dysphagia due to compression on the trachea/  oesophagus.   in the floor of the mouth called glands of Blandin and Nuhn.  The word ranula is derived from the resemblance of the    1. Haemangioma: Posterior triangle of the neck 1s one of the  common sites for haemangioma. Haemangioma Is soft,  cystic and fluctuant but transillumination 1s negative and  the sign of compressibility is positive. Fig. 16.31: Ranula in the floor of the mouth   ¢ Plunging ranula: \t is an intraoral ranula with cervical  extension, where it passes on the side of mylohyoid muscle  and produces a swelling in the submandibular region. Thus,  one swelling in the floor of the mouth and the other tn the  neck gives rise to plunging ranula. The diagnosis is  confirmed by cross-fluctuation test (Key Box 16.10).    rr ==  CROSS-FLUCTUATION TEST  e Indicated when a cyst has two interconnected components   e Gentle pressure on one component, impulse felt on the other  component   e Plunging ranula, compound palmar ganglion, iliopsoas  abscess, hydrocoele en bisac will give a cross-fluctuation  test.    1. Complete excision of the ranula is the treatment of choice  in plunging ranula. Since the cyst wall is very thin, it should  be carefully dissected and removed.  2 Marsupialisation is indicated in simple ranula. The ranula  is incised and the wall of the cyst is sutured to the mucosa  of the floor of the mouth, so as to leave an opening to the  exterior (Marsupials, e.g. kangaroo).  ¢ After 5—10 days, the cyst gets collapsed, fibrosis occurs  and the entire cavity gets obliterated.   ¢ Marsupialisation avoids surgical dissection and  chances of injury to the submandibular duct.   ¢ Plunging ranula can be excised by intraoral approach.  Once the intraoral dissection 1s completed the cervical  extensioncan be mobilised by the same incision dissecting  close to the cyst wall. However, rupture and chances of  leaving behind a portion of the cyst wall are high.    ¢ Sublingual dermoid cyst is a thick-walled cyst, whitish in  colour and not transilluminant.  ¢ Mucus cyst    1. Rupture of the cyst decreases the size but it can reappear at  a later date.   2. When the swelling is big, the tongue is pushed upwards  and may cause difficulty in speech or swallowing.    Meningocoele is a herniation of the meninges through a weak  point in the spine (neural arch) where the bony fusion has not  taken place effectively (Fig. 16.33 and Key Box 16.11). The  swelling is covered by pia mater and arachnoid mater without  a dural covering. The swelling contains cerebrospinal fluid  (CSF). Meningocoele is an example of spina bifida cystica.   * Soft, cystic, fluctuant with brilliant transillumination  are the typical features of the swelling.   ¢ When the child cries or coughs, an expansile impulse ts  present.   ¢ On palpating the edge of the swelling, a bony defect is  usually found.   MENINGOCOELE: SITES  e Lumbosacral: The commonest  e Occipitocervical: Second common  e Root of the nose: Rare    ¢ CT scan is done to look for hydrocephalus. If it is present,  a ventriculoperitoneal shunt is done which will reduce the  meningocoele.    ¢ Excision of the meningocoele should be done as early  as possible to prevent the rupture and secondary infection.    ¢ Skin covering the swelling is very thin and so is prone to  ulceration. Due to ulceration, secondary infection and  meningoencephalitis can occur.   EXCISION OF MENINGOCOELE  e Surgery: As early as possible after birth  e Early closure prevents infection  e Transverse elliptical incision  e Excision of the sac  e Closure of the defect by plication  e Approximation of the muscles    ¢ In this condition, the neural arch is defective posteriorl:  There is no visible swelling.   e It can be suspected when there is a tuft of hair, lipom:  naevus, pigmented patch of skin overlying the lumbosacrz  region.   ¢ Child is normal at birth. Neurological symptoms such a  weakness, sciatica-like pain may start appearing at pubert  (neurogenic talipes equinus—club foot).   ¢ Surgical excision of the membrane gives permanent cur  to the patient, if there are symptoms.    ¢ Protrusion of meninges, with nerve root of spinal cord  or disordered spinal cord results in meningo-  myelocoele.  ¢ Neurological deficit such as foot drop, talipes, trophic  ulcer of the foot (S, root) may be present.  ¢ Surgical excision may be followed by residual  neurological deficit.  3. Syringomeningomyelocoele  e In this condition, in addition to the meninges, the central  canal of spinal cord is also herniated out.  ¢ Most of the children are stillborn.  ¢ Very difficult to treat, if the child survives.  Encephalocoele (Fig. 16.34): It 1s also known as cranium  bifidum. It 1s a neural tube defect characterised by sac-like    protrusion of brain and meninges through an opening in the  skull.    Midline swellings: From above downwards  |. Ludwig’s angina  2. Enlarged submental lymph nodes   Present due to adherence of the nerve roots to the skin    Trophic ulcers, bladder and bowel incontinence, locomotor prob-  lems are present    ¢ This is an inflammatory oedema of the floor of the mouth.  It spreads to the submandibular region and submental  region.   Tense, tender, brawny, oedematous swelling in the  submental region with putrid halitosis is characteristic of  this condition.   1. Tuberculosis: Matted submental nodes, firm in  consistency, with enlarged upper deep cervical lymph nodes,  with or without evening rise of temperature are suggestive  of tuberculosis.   . Non-Hodgkin’s lymphoma can present with submental  nodes along with other lymph nodes in the horizontal group  of nodes such as submandibular, upper deep cervical, pre-  auricular, post-auricular and occipital lymph nodes (extemal  Waldeyer’s ring). Nodes are firm or rubbery, discrete  without matting.   . Secondaries in the submental lymph nodes can arise from  carcinoma of the tip of the tongue, floor of the mouth,  central portion of the lower lip. The nodes are hard in  consistency and sometimes, fixed.   e [tis atype of sequestration dermoid cyst which occurs due  to sequestration of the surface-ectoderm at the site of fusion  of the two mandibular arches. Hence, such a cyst occurs in  the midline, in the floor of the mouth (Key Box 16.13).    ¢ When they arise from 2nd branchial cleft, they are found  lateral to the midline. Hence, lateral variety.    ¢ The cyst is lined by squamous epithelium and contains hair  follicles, sebaceous glands and sweat glands. It does not  contain hair.    1. Young children or patients between the age of 1@ and 20  years present with painless swelling in the floor of mouth.    Differential diagnosis  e Ranula: Transillumination is positive  e Thyroglossal cyst: Moves with deglutition    2. Swelling is soft and cystic. Fluctuation test is positive.  Bidigital palpation gives a better idea about fluctuation  with one finger over the swelling in the oral cavity and the  other finger in the submental region.    3. Transillumination test is negative as it contains thick,  cheesy, sebaceous material.    1. Ranula: When a sublingual dermoid cyst is in the mid-  line in the floor of the mouth and above the mylohyoid  muscle, ranula is considered as differential diagnosis.  However, ranula is bluish in colour, brilliantly trans-  illuminant.    2. Thyroglossal cyst should be considered as differential  diagnosis when the sublingual dermoid cyst is below the  mylohyoid muscle. Thyroglossal cyst moves up with  deglutition whereas a sublingual dermoid cyst does not.    ¢ Through intraoral approach, excision can be done for both  types of sublingual dermoid cyst.    ¢ Accumulation of inflammatory fluid in the subhyoid bursa  results in a swelling and 1s described as subhyoid bursitis.    e The bursa is located below the hyoid bone and in front of  thyrohyoid membrane.    ¢ The swelling is in front of the neck, in the midline below  the hyoid bone (Fig. 16.43).    ¢ The swelling is oval in the transverse direction.  ¢ It moves up with deglutition.    Fig. 16.38: Incompletely treated thyroglossal  cyst. Recurred, about to rupture and form  thyroglossal fistula   2008: MS Examination  short case, KMC, Manipal  (Courtesy: Professor BH  Ananda Rao, Department  of Surgery)   Fig. 16.41: Cold abscess in the suprasternal space of burns: One  of the common sites of cold abscess    Fig. 16.36: Submental swelling of  30 years duration (Courtesy: Prof. KK  Rajan, Calicut Medical College)   Fig. 16.40: Pretracheal lymph nodes and  upper neck nodes: Case of non-Hodgkin’s  lymphoma    Fig. 16.42: Aneurysm of innominate artery. Atherosclerosis is the  cause. Before incising a cold abscess in this location, make sure  that it is not an aneurysm    1. Thyroglossal cyst is a vertically placed oval swelling,  whereas subhyoid bursitis is transversely placed oval  swelling (Fig. 16.44).    ¢ Thyroglossal cyst moves on protrusion of the tongue  outside (subhyoid bursitis does not).    ¢ Itarises from thyrog/ossal tract/  duct which extends from  foramen caecum at the base of  the tongue to the isthmus of  the thyroid gland. Hence, the  thyro-glossal cyst can develop  anywhere along this duct. For  anomalies are shown in Fig.  16.45.    ¢ Itis lined by pseudostrati-fied,  ciliated, columnar orsquamous epithelium which produces  desquamated epithelial cells or mucus at times.   . At the level of thyroid cartilage: 2nd common site  . Suprahyoid: Double chin appearance    ¢ Even though congenital, thyroglossal cyst appears around  the age of 15-30 years.   ¢ They are more common in females who present with  painless, midline swelling. However, in the region of thyroid  cartilage, the swelling is slightly deviated to the left side.    The cyst is soft, cystic, fluctuant, transillumination—  negative swelling (very rarely, it can give rise to  transillumination). It can be firm if the tension within the  cyst 1s high.    Mobility: Thyroglossal cysts exhibit 3 types of mobility  which are characteristic of this condition:    — Moves with protrusion of the tongue (Fig. 16.47 and  Key Box 16.14): Hold the thyroglossal cyst with the  finger and thumb and ask the patient to protrude the  tongue outside. The movement of the cyst upwards is  described as a tug because of its attachment with the  hyoid bone.    Before excision of cyst, a thyroid scan 1s mandatory since  it may be the only functioning thyroid tissue.    Sistrunk operation: Excision of the cyst along with the  entire thyroglossal tract which may include part of the  hyoid bone, is the recommended treatment. The intimate  relationship of hyoid bone can be explained by its  development from 2nd and 3rd branchial arches.    . Recurrent infection: The wall of the thyroglossal cyst  sometimes contains lymphoid tissue which can get  infected, resulting in an abscess. If it ruptures or 1s incised  it results in thyroglossal fistula (Key Box 16.15).    . Rarely, a papillary carcinoma can occur in the thyroglossal  cyst (see page 313).   Fig. 16.47: Thyroglossal cyst: It moves upwards with protrusion  of the tongue    e The cyst is attached to the hyoid bone. Hence, it gives a  classical TUG.   e Base of the tongue to rule out lingual thyroid and lymph  nodes.    Do not forget to feel base of the tongue for lingual  thyroid/ectopic thyroid. Whenever a patient has one  congenital anomaly, examine thoroughly for ‘more’  associated anomalies.   Infected thyroglossal cyst rupturing into the skin.  Inadequately drained infected thyroglossal cyst.  Incompletely excised thyroglossal cyst.    Previous history of swelling in front of the neck, which is  now painful, red and ruptured resulting in discharging  pus. Once the pus ts drained, the opening closes. However,  after an interval of time, the ‘pain and discharge’ reappear.    RECURRENT ABSCESS: RUPTURE  FISTULA OR SINUS  e Thyroglossal fistula  e Osteomyelitis  e Stitch abscess  e Pilonidal sinus  e Median mental sinus  e Cold abscess  e Umbilical sinus    ¢ When there is no infection, the fistula discharges only  mucus and the surrounding skin 1s normal. Infected fistulae  are tender, discharging pus and the skin 1s red hot.    ¢ Majority of the patients presenting are young in the age  group of 10 to 20 years.    ¢ A fistulous opening in the centre of neck which Is covered  by a hood of skin can occur due to increased growth of the  neck, when compared to that of fistula. This is described as  semilunar sign or hood sign.    ¢ Surgical excision should include the fistula with removal  of the entire tract up to the foramen caecum. Otherwise,  recurrence will occur.    ¢ The central portion of the hyoid bone is removed due to  close proximity of the fistula.    ¢ However, thyroglossal cyst is common. Lingual thyroid  and ectopic thyroid tissue are uncommon swellings.    e Central portion of the hyoid bone and lingual muscle are  removed.    e Removal is facilitated by pressing the posterior 1/3rd of  the tongue.    THYROGLOSSAL DUCT ANOMALIES  e Lingual thyroid  e Levator glandulae thyroidae  e Ectopic thyroid tissue  e Thyroglossal cyst    ¢ They have to be kept in mind as a differential diagnosis of  the swellings in the midline of the neck.    Almost all the diseases of the thyroid gland result in  enlargement of the isthmus. However, a solitary nodule and  cysts can occur in relation to isthmus. The swelling moves  with deglutition. However, it does not move on protrusion of  the tongue.    These lymph nodes produce nodular swelling in the midline.  One or two discrete nodes are palpable. They can enlarge due  to the following conditions:    2. Papillary carcinoma of thyroid: The nodes are firm  without matting, with or without evidence of thyroid  nodule.    3. Carcinoma of the larynx: The nodes are hard in consis-  tency.    4. In India, tuberculosis should be considered as a possible  diagnosis when other diseases are ruled out.    SWELLINGS IN THE SUPRASTERNAL Anterior triangle  SPACE OF BURNS |. Submental triangle   2. Sequestration dermoid cyst is a  midline, soft, cystic, fluctuant  swelling.   3. Gumma produces a firm swelling  with evidence of syphilis else-  where in the body.    4. Thymic swellings, an aneurysm  of innominate or subclavian artery  are the other causes (Fig. 16.49).   A lady of 65 years presented with swelling in the suprasternal - This triangle is covered by deep fascia.  space (Fig. 16.92). Candidate gave the diagnosis of lymph  node swelling—probably cold abscess. He failed. [t had  expansile pulsations. It was a case of aneurysm of innominate  artery. M.S. exam case 2008, JNMC, Belgaum (Courtesy: Space.  Professor Ashok Godhi, HOD of Surgery)    ¢ The floor is formed by mylohyoid muscle which arise:  from mylohyoid line of the mandible, thus closing the    . Ludwig’s angina—not uncommon  Before we discuss the swellings in the lateral side of the neck,    it is essential to know the various triangles in the neck. These  are discussed below.    Each side of the neck is a quadrilateral space subdivided by  sternocleidomastoid into anterior triangle and posterior triangle.  They are further subdivided as given below (Fig. 16.50).   movement with deglutition. It turned out to be an ectopic thyroid   swelling (Courtesy: Prof Sampath Kumar, Dept. of Surgery, KMC,  Fig. 16.50: Triangles of the neck Manipal)    They form a nodular swelling which is deep to deep fascia.  They are palpable only in the neck (not intraorally). The nodes  can get enlarged due to the following conditions:    1. Acute lymphadenitis: Very often, poor oral hygiene or a  caries tooth produces painful, tender, soft enlargement of  these lymph nodes. Extraction of the tooth or with  improvement of oral hygiene, lymph nodes regress.    2. Chronic tuberculous lymphadenitis can affect these nodes  along with upper deep cervical nodes. The nodes are firm  and matted.    3. Secondaries in the submandibular lymph nodes arise from  carcinoma of the cheek, tongue, palate. The nodes are hard  with or without fixity.    4. Non-Hodgkin’s lymphoma can involve submandibular  lymph nodes along with horizontal group of nodes in the  neck. The nodes are firm or rubbery in consistency.    The various causes of submandibular salivary gland enlarge-  ment have been discussed under the salivary gland chapter.  The common causes are chronic sialadenitis with or without  a stone, tumours of the salivary gland or enlargement due to  autoimmune diseases. They form irregular or nodular  swelling. The diagnosis ts confirmed by bidigital palpation  of the gland. Enlarged submandibular gland is bidigitally  palpable because the deep lobe is deep to mylohyoid  muscle.    The carotid triangle has following boundaries (Fig. 16.52):  Laterally by sternomastoid muscle, superomedially by  digastric muscle and stylohyoid muscle and inferomedially  by omohyoid muscle.    Some important swellings in  this triangle are as follows:  . Branchial cyst  2. Lymph node swelling (cold  abscess)  3. Aneurysm of carotid artery.    e¢ Branchial cyst arises from vestigeal remnants of 2nd  branchial arch.   ¢ The cyst is lined by squamous epithelium and contains  desquamated epithelial cells which slowly forms a tooth  paste-like material.    ¢ Even though congenital, majority of patients are young  between the age group 15 and 25 years.   ¢ The swelling ts typically located in the anterior triangle  of the neck partly under cover of the upper 1/3rd of  anterior border of sternomastoid. This can be explained  because of the development of sternomastoid muscle  from the myotome in the ridge of second branchial arch  (Fig. 16.53),   Fig. 16.53: Branchial cyst—typical location—partly covered by  sternocleidomastoid muscle. Differential diagnosis for this swelling  will be cold abscess   ¢ The swelling has smooth surface and round borders. It 1s  soft, cystic, fluctuant and transillumination-negative'.  The consistency is that of a rubber bag half filled with  water. The swelling is very often firm due to thick  inspissated content. In such situations, itis very difficult to  elicit fluctuation. The mobility of the swelling is also  restricted because of its adherence to the sternomastoid  muscle.    ° No other lesion is found in the neck (lymph nodes).   Fig. 16.54: Swelling mimicked branchial cyst. However, it was <  subcutaneous swelling with lobularity—it turned out to be lipome  (Courtesy: Dr P Rajan, Calicut Medical College, Calicut, Kerala)    ¢ Excision of the cyst along with its entire epithelial lining  with a curved incision centred over the swelling. One must  ensure that epithelial lining should be removed completely  or else recurrence will occur.    Since the wall is rich in lymphatic tissue, it can undergo  secondary infection with pain and swelling. Hence, the  swelling has to be excised (Fig. 16.54).    cyst. However, a few swellings have to be considered as Classical location    culosis of jugulodigastric nodes. Presence of multiple lymph  nodes in the neck with or without fever gives clue to the  diagnosis.    compressible swelling. However, anterior triangle is not a  common site for lymphangioma.    . Lipoma can also occur in the neck, though it is an    Fig. 16.55: Branchial cyst—one example of diagnosing a case  using clinical methods. It is important that each clinical method  elicited carefully, gives a clue to the diagnosis    'Branchial and thyroglossal cysts rarely give rise to transillumination if contents are clear and unless the cyst is small, it is difficult to elicit  fluctuation. These cysts can be firm or hard swellings also.    ¢ External opening is situated at the junction of middle  1/3rd and lower 1|/3rd of stemomastoid (Fig. 16.56).    ¢ The tract from the skin passes through the fork of common  carotid artery deep to the accessory and hypoglossal  nerve and opens inthe anterior aspect of posterior pillars  of tonsils (Fig. 16.56). The tract is lined by ciliated  Squamous epithelium and discharges a mucopurulent  discharge. Sometimes, the upper end 1s blind resulting in a  sinus.    e The patient may complain ofa dimple, discharging mucus  and the dimple becomes more obvious when the patient is  asked to swallow.    ¢ Can be unilateral or bilateral, equally common in males  and females.    e It is also called lateral fistula of the neck. (Thyroglossal  fistula is called median fistula of the neck.)   e Stylopharyngeous muscle  Pierces superior constrictor muscle and opens on posterior  pillar of the fauces behind tonsil.    ¢ Fistulogram can be done by injecting methylene blue into  the external opening and defining the tract (Fig. 16.57).  This 1s followed by exploration of the tract. At surgery, it  should be carefully dissected up to the internal opening  and then excised. May have to be done by two different  incisions: Upper incision at upper border of thyroid cartilage  and lower incision encircling fistula and dissecting upwards.    ¢ In India, this 1s the commonest cystic swelling in the carotid  triangle. The cold abscess occurs as a result of caseation  necrosis of the lymph nodes. This forms a soft, cystic,  fluctuant swelling with negative transillumination. Presence  of other lymph nodes in the neck or sinuses in the neck  gives the clue to the diagnosis.    ¢ Loss of appetite, weakness and fever with chills may be  other features.    ¢ Atherosclerosis is the most common cause of aneurysm.  This weakens the vessel walls uniformly and produces  fusiform dilatation of the blood vessel. Hypertension 1s  another factor which adds to the aneurysm.    ¢ Abdominal aorta is the commonest site for aneurysms  followed by popliteal artery.    Types  A.Fusiform: Atherosclerosis, hypertension (Fig. 16.58A).  B. Saccular: Due to injury (Fig. 16.58B).    C. False: In this condition there 1s a sac lined by cellular tissue  which communicates with the artery through an opening in  its wall (Fig. 16.58C).    Figs 16.59A to F: Different methods of operation for aneurysm. (  (C) Excision and side-to-side graft, (D) Excision and bypass nn a (E and F) Matas ae aay   ANEURYSM: CAUSES  Congenital: Berry aneurysm in the circle of Willis  Traumatic  Degenerative: Atherosclerosis  Rare causes:  Syphilis: Endarteritis obliterans  Mycotic: Infective emboli  Subacute bacterial endocarditis  Marfan’s syndrome  Polyarteritis    Elderly patients are commonly affected.  Evidence of atherosclerosis in the form of thick walled  vessel 1s present.    negative swelling with expansile pulsation (when the  fingers are kept over the aneurysm fhey are not only elevated  but they are separated).    Angiography to confirm the diagnosis followed by repair  of aneurysm with graft—PTFE graft (polytetrafluoro-  ethylene graft, Fig. 16.59).    This 1s a benign tumour arising from chemoreceptors in  the carotid body (Key Box 16.23). They are situated in the  tunica adventitia at the bifurcation of common carotid artery  (Fig. 16.60).   Chronic hypoxia can lead to carotid body hyperplasia.  Hence, there is a higher incidence of chemodectoma in  people living at higher altitudes.    Middle-aged or elderly patients are affected (Sth decade).  The patient gives long history of painless, slow-growing  swelling for many years.   Typical location: In the upper part of the anterior triangle  of the neck, at the level of the hyoid bone, beneath the  anterior edge of the stemomastoid muscle (Fig. 16.61).  Surface 1s smooth or lobulated, borders are round, and is  an oval, vertically placed swelling. Consistency 1s firm to  hard. Hence, called classical potato tumour.    ¢ Horner’s syndrome and unilateral vocal cord paralysis  can occur due to involvement of the nerves.   e Pressure on the tumour gives rise to syncopal attack due to  decrease in the pulse rate (carotid body syndrome)   ¢ Carotid artery is stretched over the swelling and so,  transmitted pulsations are felt (Fig. 16.62).   ¢ Intra-oral examination shows prolapse of ipsilateral tonsil,  unless it grows in parapharyngeal space.   ¢ Carotid angiography (Fig. 16.63A) should be done if there  are neurological symptoms such as syncopal attack. It may  demonstrate separation of the carotid bifurcation.   Fig. 16.62: Carotid body tumour in a 45-year-old lady—duration  5 years (Courtesy: Dr MR Srivatsa, Prof and Head, Prof Bagali  Babasaheb, Prof Bharathi, Dr Srikar Pai, Department of Surgery,  MS Ramaiah Medical College and Hospital, Bangalore)   Figs 16.63A and B: Carotid angiography—observe separation  of internal and external carotid artery by the tumour (A) and vascular  blush (B)   e Experience of a general surgeon with this tumour is very,  very rare.    ¢ Very rarely, it can turn into a malignant carotid body tumour  with lymph nodal metastasis.   ¢ Injury to the sternomastoid during birth causes rupture of  few fibres and haematoma. Later, healing occurs with  fibrosis, resulting ina swelling inthe middle of sternomastoid  muscle.   ¢ The other possible theory is that this is a congenital  anomaly—short sternomastoid muscle.    e This is seen in infants or children. Firm to hard, 1—2 cm  swelling in the middle of the sternomastoid muscle.   ¢ Tender and mobile sideways. Medial and lateral borders  are distinct but superior and inferior borders are continuous  with the muscle.   ¢ Division of lower attachment of sternomastoid from clavicle  and sternum with or without removal of lump 1s the surgical  treatment.    ¢ It occurs due to herniation of the laryngeal mucosa  (Fig. 16.64, external laryngocoele)   ¢ When it enlarges within the larynx, it may displace vocal  cord, produce hoarseness and is called internal laryngo-  coele.    ¢ Glass blowers, musicians, wind instruments and trumpet  players are commonly affected.  ¢ Chronic cough may be one of the predisposing factors.    ¢ Smooth, oval, boggy swelling which moves upwards on  swallowing, in relation to thyrohyoid membrane (subhyoid  position).   ¢ Swelling becomes prominent when the patient is asked to  cough or blow (Valsalva manoeuvre).   e Treatment: Ligation of its neck and division of the whole  Sac.    Othercystic swellings such as branchial cyst and lymphangioma  should be ruled out.    Secondary infection results in laryngopyocoele. The opening  in the thyrohyoid membrane may be blocked by mucopus in  such cases.    ¢ Herniation or protrusion of  mucosa of the pharyngeal wall  through Killian’s dehiscence.    ° Killian’s dehiscence is a  potential area of weakness in  between the two parts of the  inferior constrictor muscle  (Fig. 16.65): (A) Upper oblique  fibres (thyropharyngeus) and  (B) Lower horizontal fibres  (cricopharyngeus).    Due to increase in the intra-  pharyngeal pressure, mucous  membrane bulges in between parts of inferior constrictor  muscles due to neuromuscular imbalance. Hence, it is a pulsion  diverticulum.    Pulsion diverticulum deviates to one side mostly to the left  because of the rigid vertebral column in the midline posteriorly.   e Initially foreign body sensation is present in the throat.  Later, gurgling sound regurgitation of food on turning to  one side, sense of suffocation, cough or dysphagia is  present.    Pharyngeal pouch is a swelling behind sternocleido-  mastoid below the level of thyroid cartilage—soft  swelling which can be emptied.   ¢ Barium swallow followed by excision of the pouch  ¢ Cricopharyngeal myotomy may also be done.    e Anteriorly : Sternomastoid (posterior border)  SCHWANNOMA OF THE VAGUS NERVE (Fig. 16.66) °° Laterally  : Trapezius (anterior border)  ; = iia Cee e Above : Mastoid process  * This condition produces swelling in the carotid triangle in | gaigw 1 @levicia    Most of the swellings have been discussed under appropriate  chapters. Haemangioma, metastasis in the cervical lymph nodes  and Pancoast’s tumour have been discussed below.    Definition  This is a swelling due to congenital malformation of blood  vessels. It is an example of Hamartoma.   Fig. 16.66: Schwannoma of the vagus nerve (Courtesy: |. Involuting haemangioma Strawberry naevus    The posterior triangle (Key Box 16.26) is an interesting area.) portwine stain Poriinestain  as far as swellings are concerned. It is the commonest area of  metastasis in lymph nodes from occult primary. Lymphangiomas,  haemangiomas, cold abscess, lymphomas commonly occur  here. Interesting cases of cervical rib, Pancoast’s tumour,  aneurysms also occur here. ¢ Arteriovenous fistula   ¢ Combined (superficial + deep) Strawberry naevus  Capillary haemangioma  Cavernous haemangioma   |. Metastasis in the lymph nodes Lymphangioma Subclavian artery aneurysm  2. Tuberculosis Haemangioma Vertebral artery aneurysm  3. Lymphoma Cold abscess    They consist of dilated capillaries and proliferation of  endothelial cells. Hence, it commonly occurs in the skin (Figs  16.67 and 16.68). They can be of the following types:    1. Salmon patch ts a bluish patch over the forehead, in the  midline, present at birth and disappears by | year of age.  Hence, no treatment 1s required.    2. Port-wine stain is an extensive intradermal haemangioma.  This is bluish purple in colour, commonly affects the face  or other parts of the skin, is present at birth and usually  progresses and does not regress (Key Box 16.28).  ¢ Jt 1s a noninvoluting capillary haemangioma (dilatation  due to defective maturation of cutaneous innervations  during embryogenesis).   ¢ Pulsed dye laser using light with specific wavelength of  585 or 595 nanometres is one of the best treatments  available. This process is called as ‘photothermolysis’.    Flat, patchy lesion on the face in the area of fifth cranial  nerve that does not fade is port-wine stain.    CAPILLARY HAEMANGIOMA  e Skin and soft tissue involvement  e Salmon patch: Midline—forenead  e Port-wine stain: Head and neck  e Strawberry angioma: Compressible  e Wait and watch policy is the best.    e Port-wine colour even though to start with it is red in colour   e Occurs usually in the face, can also occur on the shoulder  and trunk    e Worrying because it becomes more keratotic and nodular  as age advances    3. Strawberry angiomas produce swelling which protrud:  from the skin surface. The child is normal at birth. After :  month, a bright red swelling appears over the head anc  neck region, which exhibits sign of compressibility. Thi  lesion consists of immature vascular tissue. Even thoug]  the lesion grows initially, by 5—7 years of age, swelling  regresses and colour fades. Hence, no specific treatmen  is necessary. The treatment is indicated only when the  swelling persists. 70% resolve by 7 years of age.    This occurs in place where venous space is abundant, e.g. lip.  cheek, tongue, and posterior triangle of the neck (Fig. 16.66  and Key Box 16.29).    ¢ History of a swelling in the neck of long duration. History  of bleeding is present when it occurs in the oral cavity.   ¢ The swelling is warm and bluish in colour but not pulsatile.   ¢ Compressibility is present. This sign is also called ‘sign  of emptying’ or ‘sign of refilling’. When the swelling ts  compressed between the fingers, blood diffuses under the  vascular spaces and when pressure is released, it slowly  fills up. Compressibility is a diagnostic sign of  haemangioma.    1. Lymphangioma is brilliantly transilluminant. If a  lymphangioma is infected or has been treated with  preliminary injections, it may not show transillumination.   Fig. 16.67: Capillary haemangioma (Courtesy: Prof Pramod  Kumar, HOD of Plastic Surgery, KMC, Manipal)   Gluteal region is one of the sites of haemangioma, AV fistula,  aneurysm and neurilemmoma. A patient presented with this  swelling which was firm with a few cystic areas. It was excised  in toto. Remember to check whether it is a low pressure  (venous) or a high pressure arterial lesion before excising a  haemangioma. Massive bleeding can occur if it is a high  pressure lesion.   1. Injection is the first line of treatment of cavernous  haemangioma. It makes the swelling fibrotic, less vascular  and small. Thus, excision can be done at a later date (Key  Boxes 16.30 and 16.31).   2. Excision of haemangioma in the oral cavity is more difficult  than in the neck.   3. It is better to have a control of external carotid artery in the  neck, while excising haemangioma in the oral cavity. If  necessary, external carotid artery should be ligated in order  to control the bleeding.   5. Previous embolisation into the feeding artery decreases  the size of the haemangioma (therapeutic embolisation).    ¢ Boiling water, hypertonic saline or sodium tetradecyl sulphate  (STD solution) can be used.    SWELLINGS: TREATED WITH SCLEROSANTS &  e Haemangioma  e Haemorrhoids  e Prolapse rectum  e Oesophageal varices  e Varicose veins    6. Large haemangiomas in the oral cavity should be excised  only after preliminary sclerotherapy and taking all the  precautions mentioned above.    ¢ AV fistula can be congenital or acquired (Figs 16.73, 16.79  and 16.80).    ¢ Since high pressure blood from an artery flows into the  vein, the veins get dilated, tortuous and elongated. This  arterialisation of the vein results in secondary varicose  veins.    1. Naevus flammeus, osteohypertrophy of extremities, AV fistula and  varicose veins    Fig. 16.68: Capillaryhaemangiomaofthe Fig. 16.69: Cavernous haemangioma ofthe Fig. 16.70: Local gigantism due t  nose cheek haemangioma involving gluteal region   It was initially diagnosed as post-auricular dermoid cyst Causing macroglossia. She also had birth and growing since 3 years.   Note: Importance of complete clinical examination haemangioma of the cheek and skin The patient had bleeding due to  trivial trauma. Local rise of tempera-  ture and machinery murmur was  present   Fig. 16.74: AV malformation being excised Fig. 16.75: Sclerosants were injected initially to reduce vascularity.  This was followed by wide excision   Fig. 16.83: After therapeautic embolisation it has regressed by  over 70%. Patient is waiting for another course of embolisation.  (Courtesy: Dr Umesh Bhat, Surgeon, Kundapur and Dr Subhaschandra,  Interventional cardiologist, Manipal Hospital, Bangalore)   ¢ Increased pulse rate, increased cardiac output, increased  pulse pressure result due to increased venous pressure and  arteriovenous shunt.    Functional effect  ¢ Soft, cystic, fluctuant, transillumination—negative,  pulsatile swelling.  ¢ A continuous bruit/murmur is characteristic (Fig. 16.73)  ¢ Nicoladoni’s sign or Branham’s sign  — On compressing the feeding artery, the venous return to  the heart diminishes, resulting in fall in pulse rate and  pulse pressure.  — On compressing feeding artery, pulsation or continuous  murmur may also disappear and swelling will diminish  In size.  e If the AV fistula is big, a high output cardiac failure can  occur.  ¢ The affected part is swollen (because of high pressure)  than—local gigantism (Figs 16.70 and 16.72). Thus,  overgrowth of the limb or toe can occur.  e Distal to the AV fistula, there are ischaemic ulcers, due to  comparative reduction in the blood supply.   This lady visited many general practitioners for a swelling  near the coccyx region (adjacent to natal cleft). The diagnosis  which was made in this case was subcutaneous neurofibroma,  pilonidal sinus abscess, boil and haemangioma. Careful  examinations revealed pulsations. History dates back to 20  years duration. It was a case of congenital AV fistula. Wide  excision was done. She has been asymptomatic for 2 years  now. [Courtesy. Dr B Hartimat, Asst. Prof of Surgery, K MC,    A 30-year-old patient who had an injury to the dorsum of  hand, had pain and swelling in dorsum of hand for 60 days  duration. Many had missed the diagnosis. However, swelling  had local rise of temperature, pulsations and reducibility (Fig.   ¢ Angiography with DSA pictures (digital subtraction  angiography) are essential before treating these patients  (Fig. 16.78B).    ¢ Therapeutic embolisation is the treatment of choice for  arteriovenous fistula, in congenital cases (Figs 16.82 and  16.83).   ¢ Acquired lesion need to be observed or treated by quadruple  ligation (Fig. 16.84).   ¢ The arteries and the veins are dilated and tortuous and are  compared to pulsating bag of worms.    |. Posterior cervical  lymph nodes pri-  marily involved—  route of infection  from adenoids or  other lymph nodes in  the anterior triangle.   ¢ Clinically it presents as pain in the back, cold abscess  and neurological presentation.   A. Anterior rupture: It ruptures deep to prevertebral layer  of deep cervical fascia. From here, it can take the  following routes:  ¢ Upper cervical region: Presents as deep seated   abscess in the posterior wall of the pharynx in the  midline.   e Laterally pus passes deep to prevertebral fascia  behind carotid sheath in the posterior triangle  (Fig. 16.86).    Central part of the lower lip, floor of the mouth, tip   Fig. 16.86: Schwannoma in the posterior triangle. Soft tissue  sarcoma is another diagnosis. Undergraduate clinics at JNMC,  Belgaum, Karnataka (Courtesy: Dr Aditya Patil, Prof. Ashok Godhi,  HOD of Surgery)    SECONDARIES IN THE LYMPH NODES OR  LYMPH NODE METASTASIS IN HEAD AND NECK    Very often, the patients present to the surgeon with lymph  node swelling in the neck with or without any complaints. If  there is an obvious lesion in the oral cavity, the diagnosis 1s  easy. On the other hand, difficulty arises in locating the  primary malignancy, which ts hidden or occult. It is important  to know the anatomical location of the lymph nodes in the  neck and drainage area, so that drainage areas can be  investigated.    e They can be divided into horizontal (Table 16.7) and vertical  groups of lymph nodes. Interestingly, very often circular  group of nodes are enlarged in non-Hodgkin’s lymphoma  on both sides.    4. Preauricular Outer surface of pinna, side of scalp  5. Postauricular    They can be classified into central and lateral group of lymph  nodes. Most of the central group of lymph nodes are in relation  to laryngeal cartilages or the trachea. The lateral lymph nodes  are the most popular lymph nodes called deep cervical nodes.  Majority of them are in close relationship with internal jugular  vein. Jugulo-omohyoid and jugulodigastric lymph nodes  belong to this category.    The details of the vertical group of lymph nodes are given  in Table 16.8.    Level I : Lymph nodes in the submental triangle anc  submandibular triangle.   ¢ When block dissection is being done, you will find the  lymph nodes being mentioned tn the form of levels.  Factors which accelerate local and regional spread of    5. Middle group of lymph nodes Thyroid, supraglottis  6. Lower anterior (jugulo-omohyoid)    Example: In papillary carcinoma thyroid, level III, level  IV, level V and level VI lymph nodes are removed (see  next page).    Majority of patients are elderly males (> 50 years), present  with painless swelling in the neck of a few months duration.    . The symptoms with which a patient presents to the hospital    gives the clue to the site of origin of the primary. Few   ¢ Difficulty in swallowing: Carcinoma posterior 1/3  tongue, oropharyngeal carcinoma or carcinoma  oesophagus   ¢ Obvious growth in oral cavity: Carcinoma cheek,  alveolus, tongue, etc.    Lymph nodal metastasis appears as a hard, nodular or  irregular mass in the anatomical location of the lymph  nodes.   Early cases may have some mobility. However, in majority  of cases nodes get fixed and they attain a huge size. Very  often, what appears as one lymph node, 1s a complex mass  of multiple lymph nodes.   On sternomastoid contraction test or chin test, these nodal  swellings become less prominent.   The primary malignancy may be evident in the anterior  third of tongue, cheek, alveolus, etc.   Posterior one-third of the tongue should be palpated with  gloved finger (Key Box 16.36).   Pain in the side of the head due to involvement of 5th cranial  nerve    Posterior 1/3rd of the tongue, oropharynx  Nasopharynx, sinuses  Upper oesophagus, bronchus, thyroid    or may cause paralysis of nerves. Upper anterior deep  cervical lymph nodes can cause hypoglossal nerve  paralysis (Key Box 16.37) where in the tongue points  towards the side of lesion. When there is no evidence of  the primary lesion clinically, the situation is described  as occult primary with secondaries in the neck.   Pain in the distribution of trigeminal nerve (face) suggests  nasopharyngeal malignancy infiltrating skull base (foramen  lacerum).    Any elderly patient presenting to the hospital with firm to hard  lymph node in the neck of short duration with or without fixity.  He has no signs and symptoms of inflammation such as fever  or pain to begin with.   Nodes in the upper half (Level I and II) can be due to  primary in the oral cavity, tongue, oropharynx, larynx.  Nodes in the lower half (Level II] and IV) can be due to  primary in the thyroid, tongue.   Nodes in the supraclavicular region (Level V): Carcinoma  in the GIT, genitourinary tract, lungs and nasopharynx.  Nodes in the pretracheal, suprasternal region (Level VI):  Papillary carcinoma thyroid.    CLINICAL EXAMINATION IN A CASE OF  LYMPH NODES IN THE NECK    'More details about nasopharngeal carcinoma are given in page 291 in the chapter on oral cavity.    Fig. 16.88: Level | and Il nodes. Carcinoma Fig. 16.89: Level II and Ill Fig. 16.90: Level Ill, IV and V nodes  floor of the mouth nodes: Carcinoma alveolus Carcinoma posterior 1/3rd of tongue  Ne a  \) ee "  Fig. 16.91: Level V nodes: Post- Fig. 16.92: Predominantly Level Il, Ill and Fig. 16.93: Predominantly Level Il, III anc  Cricoid carcinoma V nodes: From nasopharyngeal carcinoma V nodes: From nasopharyngeal carcinome  _—_——. > | = ——— r “ F anil   ¢ Crile in 1906, first described a standardised dissection of  cervical nodes by applying anatomical and oncological  principles including removal of surrounding fibrofatty  tissue from various compartments of the neck.    ¢ Thus with better understanding of tumour biology, natural  history of disease and availability of adjuvant radiotherapy,  radical neck dissection is not frequently done but its  modifications are done.    Types (see next page)  I. Classical radical neck dissection (Crile’s operation)  Removal of level I to V nodes + IJV, sternomastoid + spinal    ¢ Upper incision extends from mastoid process to hyoid  bone up to the point of chin across intermediate tendon  of digastric muscle.    ¢ Gives a very good exposure and vascularity of flaps are  good. No corners and hence, no necrosis.    2. Crile’s incision  ¢ Upper incision is similar to MacFee. The other incision  is oblique along the length of sternocleidomastoid  inclining more in the posterior triangle (Fig. 16.98).    ¢ When sternocleidomastoid has to be removed as a part  of neck dissection or to clear the lymph nodes which  are badly stuck to jugular vein or to accommodate  PMMC flap, Crile’s is a better incision (Fig. 16.99).   ¢ Here all the cervical lymph nodes from Level | to Level  VI are removed along with nonlymphatic structures  such as sternocleidomastoid muscle, internal jugular  vein, accessory nerve (XI), submandibular salivary gland  and cervical sympathetic plexus. A few examples wherein  radical neck dissection is done are carcinoma tongue,  oropharyngeal carcinoma, etc.    ¢ Classically done for squamous cell carcinoma of upper  aerodigestive tract with clinically positive neck dissection.    ¢ Preserve three structures: Spinal accessory, sterno-  cleidomastoid and internal jugular vein.   ¢ Done for metastatic well differentiated carcinoma  thyroid.  In this all the lymph nodes from Level I to Level V are  removed but nonlymphatic structures are preserved.   ¢ Dissection is from lower border of mandible to clavicle  and from anterior border of trapezius to midline.    ¢ Here any of the lymphatic compartments is preserved  (which should have been removed as part of classic  RND). A few examples are given below   a. Supraomohyoid dissection (Fig. 16.102): Removal of  nodes in Level I, II and III is done for carcinoma  oropharynx, carcinoma cheek. Usually done for  carcinoma floor, lateral tongue, etc.   b. Lateral neck dissection: Level II, III, IV are removed  as In carcinoma larynx and cervical oesophagus.    c. Posterolateral neck dissection: Level II to V are  removed as in cutaneous malignancy of posterior scalp  and neck. Can also be done for thyroid malignancies.    RND, hemimandibulectomy with radical glossectomy.  It is a very radical and aggressive surgery done for  carcinoma tongue.   ¢ 80% of metastatic neck masses are from primary sites above  clavicle.   e Secondaries in the lungs with cannonball appearance as  in cases of malignant melanoma of head and neck.   ¢ Bronchogenic carcinoma can be suspected by an irregular  dense shadow in the peripheral lung fields.   e Large mediastinal node mass may be seen, with or  without tracheal shift.    . Biopsy from clinically obvious lesion (tongue, cheek, etc.).  . Triple endoscopy includes    e Direct and indirect laryngoscopy  ¢ Oesophagoscopy  ¢ Bronchoscopy and biopsy of the suspicious area.    . X-ray base of the skull may show destruction of the bone    . CT scan of the sinuses, or nasopharyngeal area, or skull    . FNAC of the lymph nodes can give a diagnosis 1s more    than 90% of the cases, avoid incision biopsy as it will  result in tumour recurrence and wound necrosis.    blind biopsy is taken from posterior wall of the fossa of  Rosenmiiller and of the pyriform fossa on the same  side.    lymph node metastasis, radiotherapy is the preferred line  of treatment. However, see Chapter 17 for management of  oral malignancy.   e If the lymph node or nodes are mobile, radical block  dissection 1s done. If histopathologic report detects poorly  differentiated carcinoma, extracapsular spread, or nodes  are large (> 3 cm), radiotherapy is given.   ¢ If there are large, bulky, bilateral secondaries or fixed  secondaries, radiotherapy 1s given to the neck masses. If  the nodes do not completely regress or the tumour  becomes bigger in size after 6—8 weeks of radiotherapy,  radical neck dissection would be considered.    About 30-40% of treated patients with occult primary with  metastatic nodes die with no evidence of the primary later.  In about 30% of patients, the primary will manifest within  ]—2 years time.   About 10% of patients are cured but primary 1s not  detected.    ¢ Pancoast’s tumour or superior sulcus tumour is a  bronchogenic carcinoma arising from the apex of lung.   ¢ Typically, the patient is an elderly male around 70 years,  chronic smoker who presents with cough, weight loss,  dyspnoea and chest pain.   ¢ As the tumour grows, it compresses the lower roots of  brachial plexus C8 and T1 and results in tingling, pain  and paraesthesia in the distribution of ulnar nerve.   ¢ The tumour ts felt in the lower part of the posterior triangle.  It is hard in consistency, fixed, irregular and sometimes  tender. The lower border of the mass cannot be appreciated.   4. Horner’s syndrome due to paralysis of cervical  sympathetic chain. The preganglionic sympathetic fibres  of the head and neck are given from the Ist and sometimes  the 2nd thoracic segments of the spinal cord. These nerve  fibres synapse with the cells in the three cervical  sympathetic ganglia. They give rise to postganglionic fibres  to the head and neck region.   Thus, anywhere along this pathway, disruption, damage  or infiltration of the nerve roots results in Horner’s syndrome.  The causes of Horner’s syndrome are depicted in Key Box  16.39.    ¢ Pseudoptosis: Drooping of upper eyelid (Fig. 16.104).  ¢ Enophthalmos: Regression of the eyeball  ¢ Nasal vasodilatation: Nasal congestion    Investigations  1. Chest X-ray: May demonstrate a dense mass or collapse  of the lobe, etc.    2. CT scan may demonstrate infiltration of the tumour intc  ribs or vertebra.    4. Flexible bronchoscopy: Tissue biopsy or sputum sample  can be collected.    5. FNAC of the tumour gives the diagnosis in majority of  cases.  Treatment    e Simple flow charts which help in diagnosing many short  cases in the examination have been included.    . Following are true for ranula except: 6. Most important stimulus for carotid body tumour is:  A. It 1s a swelling in the floor of the mouth A. Hypoxia B. Hyperbaric oxygen  B. It is a retention cyst C. Hypothermia D. Hypercarbia  C. [tis transilluminant I  D. Plunging ranula produces one more swelling in the 7. Tuberculous cervical spine can give rise to cold abscess  submental region in following locations except:  A. Posterior wall of the pharynx in the midline  . Which one of the following does not give rise to cross B. Behind the carotid sheath  fluctuation: C. Front of the carotid sheath  A. Iliopsoas abscess D. Along the anterior primary division of the cervical  B. Compound palmar ganglion spinal nerves  C. Sebaceous cyst  D. Hydrocoele en bisac 8. Pancoast’ tumour has following features except:  A. It is a superior sulcus tumour  . Following are true for thyroglossal fistula except: B. It can given rise to Homer’s syndrome  A. Always congenital C. It can erode first rib  B. It is lined by columnar epithelium D. It is ususually resectable  C. Semilunar sign is seen in adults  D. Surgery done for this is called Sistrunk's operation 9. sagt of Horner’s syndrome are following  except:  . Following are derived /arises from 2nd branchial arch A. Muiosis B. Anhydrosis  except: C. Pseudoptosis D. Exophthalmos  A. Sternomastoid muscle  B. Branchial cyst 10. Following organs drain to posterior triangle lymph  C. Facial muscles nodes  D. Anterior belly of the digastric A. Adenoids B. Thyroid  C. Retropharynx D. Tonsil  . Which one of the following swelling does not contain  cholesterol crystals? 11. Content of sebaceous cyst includes  A. Branchial cyst A. Desquamated epithelial debris  B. Sebaceous cyst B. Keratin  C. Dental cyst C, Seeum  D. Hydrocoele D. Pus  ANSWERS  + D 2cC 3 A 4D 5 B 6A 7C 8D 9D 10D    Oral cavity is bounded by the lips anteriorly, the cheek on  each side, tonsils posteriorly, superiorly by the palate and  inferiorly by the floor of the mouth (Key Box 17.1). It is lined  by squamous epithelium. Oral cavity is a common site of  malignancy because it is insulted by various agents such as  alcohol, smoking, tobacco chewing. Oral cancer is the  commonest malignant neoplasm in the head and neck.  Hence, it is discussed first in this chapter.    Abuse of tobacco and alcohol are the most common  preventable risk factors for development of head and neck  cancers (Key Box 17.2). Those who smoke 2 packs/day and  drink 4 units of alcohol/day have an odds ratio of 35 for  development of carcinoma. Tobacco quid is very dangerous  and highly carcinogenic.    An interaction between redox-active metals in saliva and  low reactive free radicals in cigarette smoke occur. Net  result is that saliva loses its antioxidant capacity and instead  becomes a potent pro-oxidant milieu.    Field cancerisation is a concept based on prolonged  exposure of oral and pharyngeal mucosa to carcinogens.  15 to 20% of the survivors of one cancer of head and neck  develop another primary head and neck cancer.    e e e . ( s e e e e e e e e e e    'Betel nut chewed in combination with lime and cured tobacco is called ‘quid’. It is commonly placed in gingivolabial sulcus. It is highly  carcinogenic. This type of carcinoma 1s very common in India called Indian oral cancer.  “Reverse smoking: Smoking a cheroot with burning end inside mouth. The risk of hard palate carcinoma 1s 47 times more in these patients.   *« Cheek : 20-25%  « Floor : 10-15%  ¢ Gums :10%  Definitions    ¢ Hyperkeratosis refers to increase in keratin layers. It  occurs due to constant irritation. Once the cause is  removed, it is reversible. It is a microscopic diagnosis.    For example, Smokers’ hyperkeratosis of the palate and  lips. Once the aetiological agent 1s withdrawn, the lesion  returns back to normal.   ¢ Leukoplakia appears clinically as a white patch in the  mouth and cannot be scraped off. It is irreversible and not  attributable to any known disease. It is important to biopsy  leukoplakic portion to rule out malignancy.   1. Leukoplakia  The causes for leukoplakia are as follows:  ¢ Smoking results in hyperkeratosis. Nicotine in the form  of cigarettes, chewed tobacco!, powdered snuff produces  premalignant changes in the oral cavity.  ¢ Spices  ¢ Spirits have synergistic action with smoking   Fig. 17.1: Leukoplakia of the tongue—  biopsy was positive for malignancy. He was  treated with radiotherapy   Syphilis causes endarteritis obliterans and results in  chronic superficial glossitis of the tongue which 1s a  precancerous condition (rare these days).  Susceptibility’ of a person    ¢ Betel nut, and slaked lime with betel leaf and tobacco  (pan) 1s eaten and usually kept inside the cheek for many  hours. Over the years, it brings about chronic irritation  of mucosa of the cheek and causes leukoplakia. Tobacco  contains multiple carcinogens including aromatic  hydrocarbons.    I. Keratosis appears as a milky blush on the surface.  II. Acanthosis refers to elongation of rete pegs. This appears  as a smooth, white, dry patch.   [1]. Dyskeratosis means the formation of keratin cell layer  in the deeper aspect of epidermis, before they reach the  Surface.   IV. Speckled leukoplakia appears as multiple, small white  patches on an erythematous base. /t has the highest rate  of malignant transformation (Figs 17.1 and 17.3).    Cy.  CHRONIC HYPERPLASTIC CANDIDIASIS (Fig. 17.2)  Commissures of the mouth commonly affected  Albicans Candida invasion  No response to drugs, then surgery/laser treatment  Dense plaques of leukoplakia  Immunodeficiency can precipitate this condition  Dangerous because of malignant potential  Antifungal treatment—Topical application may help  Remember as CANDIDA    (Courtesy: Dr Keerthilatha Pai, Head, Oral Medicine, College of Dental Sciences, Manipal)    'An 18-year-old girl, nonsmoker, nonalcoholic, with good oral hygiene had carcinoma tongue. What was the cause?    About 10% of leukoplakia patients develop oral cancer.  Hence, superficial excision of the lesion followed by skin  grafting should be done.   Even though leukoplakia is irreversible, Isotretinoin (1 3-  cis-retinoic acid) can reverse some cases of leukoplakia  and possibly reduce the development of squamous cell  carcinoma.    of malignancy around 15% (17 times more malignant than  leukoplakia). It 1s irregular in outline and may be nodular.    ¢ This is supposedly due to use of pan masala, arecanut  with or without alcohol.   Initially it produces ulceration of mucosa of the cheek.  These ulcers heal resulting in a dense submucous  fibrosis, which appear clinically firm to hard. Itcan affect  the tongue also. It is a progressive disease entirely  confined to Asian population.    Sideropenic dysphagia (Plummer-Vinson and Paterson-  Kelly syndrome). Iron deficiency occurs in the absence of  anaemia in these patients. Common in Scandinavian  women. Iron supplements reduce epithelial atrophy.    Papilloma of the tongue or cheek  Discoid lupus erythematosis  Dyskeratosis congenita    Syphilitic glossitis: Tertiary syphilis produces chronic  superficial glossitis which can lead to carcinoma of the  tongue. However, it is rare these days.    Human papilloma virus is a epitheliotropic virus. Its  oncoproteins suppress tumour suppressor gene. It can give  rise to tonsillar carcinoma and oropharyngeal carcinoma.   Most common premalignant lesion is leukoplakia.  Multiple anatomic sites can be involved simultaneously  (synchronous).    Generous biopsy and MRI (if necessary) are the  investigations of choice.   Surgery, radiotherapy and chemotherapy are used singly  or in combination in appropriate cases.    Distant metastasis is more common with nasopharyngeal  carcinoma than with any other head and neck cancer.    Common symptoms and common sites of oral  cancer (Ca = Carcinoma)   Trismus Extension of cancer into pterygoid muscles  Dysphagia Base of tongue, hypopharynx, oesophagus    1. Cure of the patient: Cure of the cancer, if possible, with    wide excision of the tumour which includes removal of the  tumour with 2 cm of the normal tissues, with or without  bone.    curative radiotherapy (RT). Even when nodes are not  palpable, following guidelines are given (Key Boxes 17.4  and 17.5).    Carcinoma lateral tongue, floor of mouth and mandibular  alveolus commonly cause occult metastasis.    Hence selective neck dissection of levels |, Il and Ill are  indicated in continuity with tumour excision in these cases.    N1  : Selective supra-omohyoid neck dissection  {N2a :Modified radical or radical neck dissection followed by  N2b} postoperative radiotherapy    N2c_ :Bilateral radical neck dissection—preserve at least  one Internal Jugular Vein (IJV) + postoperative  radiotherapy    *« 74: Any cancer invading adjacent structures such as  cartilage, cortical bone, deep (extrinsic) muscles of the  tongue, skin or soft tissue of the neck.   T4a: Moderately advanced local disease  T4b: Very advanced local disease (skull base, pterygoid  plate, internal carotid artery, masticator space)   * NI: Single positive ipsilateral node less than or equal  to 3 cm in greatest dimension   * N2a: Single positive ipsilateral node more than 3 cm  but less than or equal to 6 cm    Stage I T1, NO, MO  Stage II T2, NO, MO  Stage III T3, NO, MO, TI-3, N1, MO  Stage IV T4, NO, MO , T, N2—-3, MO    are managed by combination of surgery with postoperative  RT. Usually, surgery is the principal therapeutic  modality of treatment followed by postoperative  radiotherapy. The treatment depends upon general  condition of the patient, risks of anaesthesia, adequate  intensive care management, etc. Chemotherapy also has  been tried before or after surgery. However, response rate  has improved but it has not affected the survival.    The most important benefit of chemotherapy has been in  the treatment of laryngeal and nasopharyngeal carcinomas.    Cisplatin is clearly the most effective drug. Other drugs  such as carboplatin, 5-fluorouracil (5-FU), bleomycin,  gemcitabine, etc. are also used.    Induction chemotherapy: In advanced cases, chemo-  therapy is given before surgery or RT. In more than 80%  of cases, tumour regression can occur.    Concurrent chemoradiotherapy (CCRT): [timproves both  local and regional controls, specially in those patients with  high risk cancers, e.g. locally advanced cancers of the oral  cavity, larynx, oropharynx. Drug used 1n CCRT can be high  dose cisplatin—100 mg/m’ IV for 3 cycles every 21 days  concomitantly with RT (for other dosage, kindly refer to  oncology manual). Side effects include severe mucositis,  xerostomia. Gastrostomy may be necessary for feeding.    Irradiation of the oral cancers achieves a cure in about  80-90% of patients. It preserves anatomical part and also  preserves the function.   The dose of RT: 6500-7500 cGy units is required to  eradicate squamous cell carcinoma of head and neck. It is  usually given in the daily dose of 180-200 cGy units.   RT: ADVANTAGES  Easy, safe with minimal mortality  Preservation of an organ  Function of the part is preserved  Cure rate is around 80 to 90%  First line in early cases.    RT: DISADVANTAGES  Long stay in the hospital (can also be taken as an out patient)  Tumour Cure cannot be assesed by pathology  Soft tissue fibrosis resulting in ankylostomia  Adverse effects on skin, loss of hair, mucositis of oral cavity,  xerostomia, etc.   In advanced lesions,  surgery is found to be superior  to radiotherapy as a  first line of treatment    Dose: 60 Gy to lymph  nodes + primary  and  50 Gy lower risk  | nodal stations in the neck    Drugs  * Cisplatin—100 mg/m’ on  days 1, 22 and 43 of 7-week  course of 70 Gy of radiation  ¢ Ceruximab, an EGFR*  antibody 200-400 mg/m’ weekly  8 weeks    Fig. 17.5: Management of locally advanced nonmetastatic  disease (III to IV B)   ¢ Radiotherapy is given in T! and T2 lesions as the first line  of treatment and postoperatively in T3 and T4 lesions after  surgery.    ¢ Surgery is done in all stages of oral cancers. It may be in  the form of wide excision or wide excision with removal  of the bone (composite resection). In advanced stages it  may be palliative surgery such as excision of a fungating,  ulcerating, bleeding mass. Surgery is also done for the  lymph nodes in the torm of radical neck dissection (RND),  or modified RND.   ¢ The pectoralis major myocutaneous flap (PMMC flap):  It is the most widely used flap for the reconstruction of  oral cancers.    e The specimen is available for histopathological examination  for cancer clearance.  e 80-90% cure is possible.  SURGERY: DISADVANTAGES  e Loss of an organ—total glossectomy  e Functional and cosmetic disability  e Significant morbidity  e Mortality: 8-10%.   Carcinoma of the buccal mucosa is very common in India due  to the habit of keeping tobacco quid in the cheek pouch  (gingivoalveolar sulcus).    A nonhealing ulcer, with slough in the centre of the lesion  An exophytic growth, or a proliferative growth—verrucous  carcinoma.    structures such as tongue, mandible, floor of the mouth and  skin. Skin infiltration results in orocutaneous fistula.    A nonhealing ulcer or cauliflower like growth. Verrucous  carcinoma is an exophytic growth.   Edges are everted (Fig. 17.6) with induration at the base  as well as at the edge. Induration clinically presents as a  hard feeling. Pathologically, it is due to fibrosis, caused by  malignancy (carcinomatous fibrosis). It 1s a diagnostic  feature of squamous cell carcinoma. Possibly, it 1s a host  reaction indicating good immunity. Due to fibrosis, some  lymphatics get obliterated. This delays spread of the disease,  thereby improving the prognosis.   Ulcer bleeds on touch. Due to secondary infection most  of the oral cancers are tender to touch (Fig. 17.9).    PECULIARITIES OF VERRUCOUS CARCINOMA  Very slow-growing  Growth is exophytic (than infiltrative)  Rarely spreads by lymphatics  It is a well-differentiated carcinoma  Surgery is the treatment of choice.    Fixity to the underlying structures such as mandible may  be present.   Evidence of leukoplakia may be present in the oral cavity.  Trismus is due to involvement of pterygoid muscles and  masseter. This occurs when carcinoma buccal mucosa  extends into the retromolar trigone. Trismus can also be  due to soft tissue fibrosis caused by radiation. Once  perineural lymphatics areinvolved, spread can occur in  infratemporal fossa resulting in trismus (Key Box 17.9  and Fig. 17.10).    Difficulty in opening the mouth is called trismus. Normal mouth  opening ranges from 35 to 45 mm.  Grades:    Grade | : Mouth opening is between 2.5 and 4 cm  Grade || : Between 1 and 2.5 cm  Grade Ill: Less than 1 cm    . Mandible fractures  . Pterygoid muscle infiltration by growth in the retromolar    . Acute inflammatory lesions in the oral cavity  . Tetanus and tetany  . Radiation fibrosis of soft tissues/muscles of mastication    ¢ Bidigital palpation of mandible is done by examining with  index finger on the outer aspect of the mandible and the  thumb on the under surface of the mandible. This test should    be done on the opposite side first. Only then, the thickening  of the mandible can be appreciated. The mandibular canal ts close to occlusive surface, in    edentulous elderly patients due to decrease in the vertical  height of horizontal ramus thus facilitating easy spread  (Figs 17.12 and 17.13) oforal cancer to mandible.    3. Blood spread: It is very rare and it occurs late.    ¢ Gingival cancers (Fig. 17.11)  — Early cases present as mucosal change in leukoplakia  — Loosening of tooth may be a presenting feature.  — Can present as bleeding and pain Investigations  ~ Bone involvement occurs early 1. Wedge biopsy from the edge of the ulcer is taken because  of the following reasons:  ¢ Tumour cells are concentrated more in the growing edge  ¢ Comparison with the normal tissues is possible.    e e e % @ e * e * @ ® 6 e e a ? S 5] a e * e ° e * 2 e a e ®   eee Se Ne 3. Chest X-ray to detect inhalation pneumonia.  CARCINOMA BUCCAL MUCOSA AND MANDIBLE 4. FNAC of the lymph node.   e Direct infiltration by the tumour 5. Magnetic resonance imaging (MRI)   ¢ Orthopantomogram or spiral CT can be used for imaging dictating the extent of resection especially in patients    e Loss of central part of mandible results in pouting of lower  lip and continuous drooling of saliva. It is called as Andy  Gump deformity’.    with restricted mouth opening.  ¢ Itis the investigation of choice to look for involvement  of skull base, brachial plexus, spinal nerve roots and  lymph nodes.  2. Lymphatic spread: Submandibular nodes and upper deep © MRI has no radiation hazards.  cervical nodes get enlarged (Levels | and II). In 50% of  the cases, lymph node enlargement is due to infection and  remaining 50%, it is due to metastasis. Metastatic deposits __It can be classified into early disease and advanced disease  are hard in consistency, indurated and with or without (Figs 17.9 to 17.11).    'Andy Gump was one of the characters in a popular comic strip, ‘The Gumps’ created by Sidney Smith in 1917. The character’s face seems to  end at the upper lip due to ‘absence of mandible’ and is chinless. A statue of this comic character 1s on display at Lake Geneva Museum    1. A small superficial ulcer (T1, T2) 1s treated by wide  excision followed by split skin graft (SSG).    2. Aninfiltrative lesion is treated with wide excision followed  by a flap reconstruction. Usually, PMMC (pectoralis major  myocutaneous flap) is used.    PM MC flap: This is the most widely used flap now for head  and neck reconstruction. The flap is raised along with muscle  and an island of skin based on pectoral branch of  thoracoacromial artery. It 1s tunnelled under the skin of  chest wall and neck and brought to the area of the defect. It  has been described as the ‘workhorse’ for head and neck  reconstruction.    ¢ Radial artery based flap is the workhorse of microvas-  cular reconstruction.   As mentioned earlier, early lesions can be managed with  radiotherapy (RT). The advantage of RT 1s that it cannot  only cure the disease but also preserve the organ and its  function.   Indications for radiotherapy  1. Patient not willing for surgery  2. Patient not fit for surgery    1. External radiotherapy: Large total dose of 6000—8000  cGy units are given at the rate of 200 cGy units/day.    2. Interstitial radiotherapy ts indicated in infiltrative small  lesions. Caesium 137 or iridium wires are placed within  the tumour. Advantage of this method is minimal tissue  reaction.    Surgery: T3 and T4 lesions require surgery as the main  modality of the treatment followed by postoperative radio-  therapy. Most of the lesions require full thickness resection  leaving behind large defects. Such defects can be repaired using  myocutaneous flap.    When the primary lesion is removed en bloc with  mandible and cervical lymph nodes, it is called composite  resection.    1. Carcinoma buccal mucosa fixed to the mandible: Wide  excision of the growth along with segmental resection of  the mandible or hemimandibulectomy is done depending  upon the infiltration of the tumour. Very often, whole  thickness of the cheek is lost which is reconstructed by  using PMMC flap (see next page).   Fig. 17.17: Gross lymphoedema of left side  of the face with enlargement of sub-  mandibular lymph nodes   of either inner or outer table of the mandible or excising    Fig. 17.15: Neglected carcinoma buccal  mucosa infiltrating floor of the mouth,  commissure and lower lip    Fig. 17.16: Advanced carcinoma  buccal mucosa with involvement of halt  of lower lip    2. Orocutaneous fistula is treated by wide excision which  refers to removal of the entire thickness of the cheek along  with the growth.    Reconstruction is done by using PMMC flap. Radiotherapy  should not be given as it results in persistence of fistula.    3. Carcinoma of the buccal mucosa with lymph nodes:  Along with the primary, submandibular nodes and upper  deep cervical nodes (Levels I, II and III) are removed,  along with submandibular salivary gland. This ts called  supraomohyoid block dissection. If surgery has been used  to treat the primary, the lymph nodes also should be treated  by surgery in the form of neck dissection.    Both primary lesion and lymph nodes should be treated  by radiotherapy and reassessment done after 3-4 weeks.  If residual glands persist or if the glands become mobile,  neck dissection can be done at a later date. Fixity to internal  jugular vein or sternocleidomastoid muscle are not contra-  indications for radical block dissection. Those structures  can be removed. However, when the lymph nodes are fixed  to the carotid artery, radiotherapy ts preferred.    ¢ It is advocated in T3 and T4 lesions irrespective of nodal 1. Orocutaneous fistula  status. This amounts to minimal supraomohyoid neck )  dissection withremoval of Levels I, II and III lymph nodes.  It has shown survival benefits.   ¢ It is also indicated in carcinoma mandibular alveolus or  buccal mucosa extending into the floor of mouth. 4. Cancer cachexia.    . Trismus—It can be due to direct infiltration of pterygoid  muscles or masseter or soft tissue fibrosis following RT.   Fig. 17.20: Amount of tissue to be removed Fig. 17.21: Skin paddle outlined prior to Fig. 17.22: Skin paddle mobilised  (wide excision) is marked. Horizontal incision elevation  iS given in the neck for block dissection    (Courtesy. Prof. Satadru Ray, Head of the Department, Surgical Oncology, KMC, Manipal)    Fig. 17.30: Completely mobilised PMMC flap ready for reconstruction. Fig. 17.31: Healing after 10 days  Main vessels in the paddle are pectoral branches of acromiothoracic  artery. Mandible reconstruction is not mandatory    . Nonhealing ulcer, commonly on lateral border of tongue  in 60% of cases, with slough (Fig. 17.32)    Pain in the tongue 1s due to involvement of lingual nerve.  In such cases, pain from the tongue can be referred to the  ear and lower temporal region. '   Ankyloglossia is restricted mobility of the tongue. It is due  to infiltration of the floor of the mouth or mandible, or due  to an advanced lesion (Figs 17.33 and 17.34).  Disarticulation—difficulty in talking is due to inability of  the tongue to move freely.   Dysphagia is a common presentation from carcinoma of  posterior |/3rd (in 20% cases). An elderly gentleman sitting    in the outpatient department spitting blood-stained saliva  is suggestive of carcinoma posterior 1/3rd of the tongue  . (Figs 17.35 and 17.36).   Fig. 17.36: Carcinoma tongue with absolute  dysphagia, fixed lymph nodes in the neck and    ' Auriculotemporal nerve and lingual nerve are posterior branches of mandibular division of trigeminal] nerve.   ¢ Bilateral massiveenlargement of lower deep cervical nodes  in an elderly patient is suggestive of carcinoma of posterior  1/3rd. The patient may not be aware of growth at all.    Tongue cancers tend to be more rapid in their onset than  other cancers in the oral cavity. Compared with other  cancers within the oral cavity, tongue cancers have  greater potential of lymph node metastasis.    ¢ Inspection and palpation of the growth or the ulcer should  be described in the same manner as that of carcinoma  cheek. Typically, the ulcer bleeds on touch with central  slough. The edge, base and surrounding area are indurated.  Carcinoma of the tongue and carcinoma of the penis are  two places in the body wherein induration can be much  more extensive than the primary growth or an ulcer. In  some cases, induration may be the only finding. Everted  edge 1s commonly seen (Figs 17.37 to 17.39).  e Digital palpation of posterior 1/3rd of tongue should be  done with a glove.  * Test for mobility of the tongue.  — Forward protrusion—genioglossus. This is the muscle  commonly involved.  — Backward movement—styloglossus  — Elevation—palatoglossus  — Depression—hyoglossus  All these muscles are supplied by hypoglossal nerve  except palatoglossus which 1s supplied by glosso-  pharyngeal nerve.  ¢ Bidigital palpation of the mandible should be done which  may show thickening.    1. Apical vessels drain the tip of the tongue into submental  lymph nodes, bilaterally.    2. Lateral vessels drain into submandibular lymph nodes,  from here to the lower deep cervical lymph nodes and  jugulo-omohyoid nodes—level II].    Basal vessels drain the posterior 1/3rd of the tongue. There  is criss-crossing of the lymphatics on both sides. Hence,  they drain into bilateral lower deep cervical lymph nodes.  (Some lymphatics on their way to the nodes, pass through  the mandible which explains the frequent involvement of  mandible in carcinoma tongue. This is a debatable issue now.)  ¢ In 50% of cases, the lymph node enlargement is due to  secondary infection. Such nodes are tender and firm and  respond to antibiotics. In remaining cases, they are hard  and fixed and hence, significant.    Posterior 1/3 tongue has very less cornification but has  abundant lymphatics which explains massive nodes  (Key Box 17.12).    Wedge biopsy from edge of the ulcer can be taken under  local anaesthesia. In cases of proliferative growth, punch  biopsy is recommended. In cases of growth arising from  posterior 1/3rd of the tongue, biopsy can be taken under  general anaesthesia. It also provides an opportunity to  examine in detail the posterior spread of the disease into  tonsils, pharynx, etc.    Orthopantomogram: X-ray of the mandible can  demonstrate an irregular defect due to invasion, erosion  or pathological fracture.    CARCINOMA POSTERIOR 1/3rd  lt presents with dysphagia or with a change in voice.  Easily missed in a clinical examination  Biopsy should be done under general anaesthesia to avoid  aspiration and to assess the spread posteriorly.  Palpation will give the diagnosis—induration  It is one of the occult primaries for lymph node secondaries  in the neck.  Criss-crossing of the lymphatics explain bilateral lymph  nodes in the neck.  Blood spread is more common.  Prognosis is bad because well-differentiated carcinoma in  this location is rare.    3. Chest X-ray is taken to rule out aspiration or inhalation  pneumonia.    4. Routine investigations such as complete blood picture,  fasting and postprandial sugar estimation to rule out  diabetes and electrocardiography to assess cardiovascular  function should be done.    Carcinoma of the tongue is managed similar to a cancer in the  oral cavity. However, to preserve the function of the tongue,  widespread disease in the posterior one-third tumours, general  health of patient (elderly with bad bronchopneumonia) may  decide the treatment in favour of radiotherapy. However,  results of surgery or radiotherapy for early carcinoma of tongue  are equivalent.    f. Carcinoma ia situ: This type is uncommon in our country.  Wide excision with 1 cm margin and a depth of | cm is  sufficient. Reconstruction of the tongue is not necessary.    2. Partial glossectomy is indicated when the lesion is less  than 2 cm (TI) and confined to the lateral border of the  tongue. About 1/3rd of the anterior 2/3rd of the tongue 1s  removed. The wide excision should include at least 2 cm  of tissue away from the palpable indurated edge of the  tumour (Figs 17.41 and 17.43).    3. Hemiglossectomy refers to removal of around 50% of the  tongue. This is indicated in a radio-residual tumour, radio-  recurrent tumour or where radiotherapy facilities are not  available (Fig. 17.42). Reconstruction of the tongue can  be done by nasolabial flap and division of pedical at later  date. Radial forearm free flap can also be used.    4. Total glossectomy: Indications are similar to those  mentioned above. However, very extensive growth  involving the entire tongue are given radiotherapy initially,  to reduce the size of the tumour. Surgery can then be  undertaken. Total glossectomy carries significant mortality  and morbidity.    Fig.17.43: Partial glossectomy done with the help of laser—  excellent tool for haemostasis (Courtesy: Dr Balakrishnan, Prof of  ENT and Head and Neck, KMC, Manipal)    5. Commando’s operation: This is indicated when  carcinoma of tongue ts fixed to the mandible with  infiltration of the floor of the mouth. Hemiglossectomy  with hemimandibulectomy, removal of the floor of the  mouth and radical neck dissection is described as  Commando’s operation (Key Box 17.13).    ¢ However, in a few selected cases, removal of the  haemimandible is not necessary. Growth which is  close to the margin of the mandible without infiltration  (confirmed by X-ray) needs to be treated by marginal  mandibulectomy. Carcinoma of the tongue with  involvement of only a small portion of mandible can  be managed by segmental excision. Advantage of this  method ts that it is not only cosmetic but also preserves  the function of the tongue by preserving genio-  glossus. Hence, the tongue may not fall backwards after  surgery.   e The fat, fascia, lymphatics from midline to the anterior border  of trapezius, from mandible to clavicle below.   e The lymph nodes—submental, submandibular, upper and  lower deep cervical nodes, posterior group of nodes (Levels  I-V).   e Submandibular salivary gland, sternomastoid, one side  internal jugular vein (IJV) are sacrificed   e Lower pole of parotid is removed to facilitate lymph node  clearance.    * Incidence of carcinoma of the lip is about 10 to 12%. It is  common in the western, elderly, white people, specially  those exposed to sunlight. The actinic rays produces actinic   TREATMENT OF LYMPH NODES (Fig. 17.44) cheilitis—inflammation of the lip, especially lower lip,    secondaries in the neck. However, its main indication is a  large primary tumour with neck nodes. In such situations   both the primary and secondary can be managed with KEY BCX 17.14 \  radiotherapy alone which carries minimal morbidity and Ng  meatallite: COUNTRYMAN’S LIP  = ae Sunlight  ¢ Ifthe general condition of the patient is good and the lymph fi  nodes are hard and mobile, hemiglossectomy with excision Actinic rays  of the floor of the mouth with radical dissection of the neck ip  is done (Commando’s operation). Cheilitis  ¢ If radical neck dissection has to be done on both sides, hha  the IJV should be preserved at least on one side to J  prevent cerebral oedema. In such cases, radiotherapy Cracks    Please note: Details about radical neck dissection is given in  Chapter 16, page 267.    3. Uncontrolled haemorrhage from growth: [n such cases  ligation of external carotid artery above superior thyroid  branch should be done (Fig. 17.45). If ligature is applied  below the origin of superior thyroid artery, it results in  eddy currents and thrombus at bifurcation of common _ Fig. 17.46: Advanced carcinoma lip—a proliferative lesion, treated  carotid artery. with radiation   ¢ Leukoplakia is also responsible for squamous cell  carcinoma. Smoking, spirits and spices are the common  precipitating factors.   ¢ Genetic factors also may play a role. Blacks are less  susceptible. On the other hand, increased incidence of  carcinoma lip has been found in Caucasians.   * Khaini chewers are more susceptible for carcinoma of  the lip (Ahaini is a mixture of tobacco and lime).   * Edge is everted and indurated. Induration of the edge  and the base 1s characteristic (Fig. 17.47).   * The entire upper lip and lateral portions of the lower lip  drain into upper deep cervical nodes. Central portion of  the lower lip drains to submental nodes and submandibular  nodes. Like elsewhere in the oral cavity, in 50% of the  cases, nodes are enlarged due to secondary infections. In  remaining 50% of the cases, they are enlarged due to    Fig. 17.48: Carcinoma lip with infiltration  of buccal mucosa. Requires lip and cheek  reconstruction   Fig. 17.49: A bleeding exophytic lesion.  Better managed by surgery than radio-  therapy    metastasis. Such nodes are hard, with or without fixity.  Blood spread is uncommon.   DIFFERENTIAL DIAGNOSIS OF CARCINOMA -  e Keratoacanthoma  e Ectopic salivary gland tumour  e Pyogenic granuloma  e Leukoplakia.    In a classical case of carcinoma of the lip with everted edges  and induration, there is no differential diagnosis. However,  following are a few conditions to be remembered:  1. Keratoacanthoma  ¢ It is a cutaneous tumour arising from hair follicles on  the lips. It is common in white, western, males between  50 and 70 years of age.  ¢ Sunlight (actinic rays), chemical carcinogen, viral factors  may be responsible for this lesion.  ¢ The central portion of the nodule may ulcerate. The  lesion may progress for 6 weeks and may resolve  spontaneously within 4-6 months.  2. Ectopic salivary gland tumour  ¢ The lip is one of the common sites of malignant salivary  gland tumours. This presents with submucous nodules  that grow slowly and ulcerate and may mimic squamous  cell carcinoma (Fig.17.51).  ¢ They are also indurated lesions.  ¢ However, the characteristic everted edge may not be  seen.  ¢ These are adenocarcinomas which are treated by surgery.  3. Pyogenic granuloma (Fig. 17.52)  ¢ Recurrent infections or trauma produces a polypoidal  mass with significant bleeding.  ¢ It is rich in granulation tissue and resembles a polyp.  ¢ It1s devoid of epithelium  ¢ Histologically, it is a capillary haemangioma  ¢ Absence of induration gives the diagnosis.    Fig. 17.51: This lesion was diagnosed as carcinoma lip. However,  it did not have everted edges. It was indurated. Biopsy reported  as ectopic salivary gland tumour. On careful questioning, patient  says it Started as a swelling not as an ulcer   Fig. 17.52: Pyogenic granuloma due to caries tooth (Courtesy:  Dr Keerthilatha Pai, HOD, Department of Oral Medicine, College  of Dental Sciences, Manipal)    4. Leukoplakia  A slow developing leukoplakia presents as whitish nodule  or an ulcer. However, biopsy confirms the diagnosis.  Treatment    Surgery and radiotherapy are the two modalities available for  the treatment of carcinoma of the lip.    I. Surgery  ¢ T] and T2 lesions can be excised followed by direct suturing  without much functional problems. This 1s described as “V”’  excision which includes removal of growth with | cm healthy  margin. Care should be taken to excise full thickness of the lip.  ¢ When removal of more than 1/3rd of the lip is required,  flap reconstruction may be necessary. The primary goal in  lip reconstruction surgery 1s oral competence.  Examples  1. Abbe flap: Based on upper labial artery—a pedicled  flap 1s rotated down and sutured to the defect at the  lower lip (Fig. 17.55).  2. Estlander’s flap: Wedge-shaped flap is used to  reconstruct carcinoma of lower lip, when it involves  the angle (Fig. 17.54).   * Larger tumours: T3 and T4 lesions are irradiated first. If  the tumour persists after radiotherapy, excision of the entire  lip may be necessary followed by PMMC flap  reconstruction.   ¢ Significant lymph nodes can be removed along with the  primary tumour—supraomohyoid block dissection.   * Its indicated tn all stages of carcinoma of the lip. Radio-  therapy produces tumour necrosisresulting in a slow healing  rate. Treatment lasts for several weeks and it delays the  wound healing. Elderly patients who are not fit for surgery  and carcinoma lip with fixed nodes are treated by irradiation.    ¢ There are various methods available to reconstruct the lip  * Upto 1/3rd of the lip can be sacrificed with direct closure  * Details are given in page 289 and summary (Table 17.2).    Please note: Students are advised to refer plastic surgery books  for more details. Knowledge of some of these flaps will help  you in getting more marks in the examinations.    Wide excision with removal of anterior mandible  Wide excision with removal of underlying   Total lip reconstruction by using forearm flap  Primary closure or secondary healing  Reconstruction by radial forearm flap  Reconstruction by using rectus abdominus flap  Radial artery forearm flap   Fibula flap or iliac crest graft—deep circumflex iliac  artery (DCIA) is used    Primary reconstruction with fibula flap for edentulous  mandible and DCIA for dentate mandible.   Fig. 17.53: Karapandzic flap—used for defect up Fig. 17.54: Estlander flap. Used Fig. 17.55: Abbe’s flap. Used for up to  to 2/3rd of lip size. Good functional result, good for lateral lip defects up to 1/3rd_ = 1/8rd lip defect. Can be modified for  colour and texture match size. Good functional result both upper and lower lip. Produces   Figs 17.56 to 17.58: Wide excision of lip followed by reconstruction using nasolabial flap (Courtesy: Dr Biswas, Dr JK Jha, Dr SP  Nayak, Chittaranjan National Cancer Institute (CNCI), Kolkata)   ¢ Antral carcinoma can be divided by a line joining medial  canthus of eye to the angle of mandible. This line is called  as Ohngren’s line    ¢ Poor prognosis in suprastructure because of proximity to  skull base and pterygoid region   It is rare in western countries but common in Asia. The workers  in furniture industries, chromic and nickel industries are more  prone for the development of carcinoma maxillary antrum.    bulge of the hard palate. This results in pain in the teeth = 1    2. When medial wall is involved, nasal obstruction and  epiphora occurs due to obstruction of the lacrimal duct.  Bleeding from the nose can also occur, if there is ulceration.    3. If anterolateral wall is involved, asymmetry of the face  results in pain in the cheek. Anaesthesia over the skin of  the cheek including upper lip occurs due to involvement  of infraorbital nerve, a branch of maxillary division of the    Posterior extension of the growth is difficult to assess  clinically. When it involves the pterygoid muscles, it  results in trismus. Paraesthesia over the cheek, gums, lower  lip, postnasal discharge are the other features of these  tumours. They carry poor prognosis because of late  presentation.    Computed tomography (CT scan) can define a lesion,  its extent, bony destruction, posterior extension, etc. Hence,  it is the first investigation of choice.   CARCINOMA MAXILLARY ANTRUM  Majority are squamous Cell carcinoma  Most important method of spread is contiguous spread    Early presentation is rare because maxillary antrum serves  no important function    Tissue diagnosis is by biopsy of the mass protruding through  nasal cavity/oral cavity or transnasal needle through medial  wall of the maxilla.    CT scan for bone involvement and MRI for soft tissue  involvement are investigations of choice    ¢ Unilateral nasal obstruction (He is asked to breathe,  closing the nostrils one by one)    ¢ Asymmetry of the face  ¢ Change in the level and sharpness of inferior orbital margin, proptosis    — Posterior extension into infratemporal region and then into  temporal region.    followed by curettage to reduce the tumour bulk and to  drain necrotic contents outside.    Radiotherapy is the main modality of treatment in  carcinoma maxillary antrum. Curative rate is around 70%  in early cases. In advanced cases, radiotherapy is given  first. This reduces the tumour bulk so that an unresectable  lesion becomes resectable and maxillectomy can be done.  Surgery can be done in the form of total maxillectomy  when the growth involves entire maxilla or it is of high  grade followed by postoperative radiotherapy.   Tumours of the lower half of the antrum are treated by  partial maxillectomy. It includes removal of the entire  hard palate, alveolus and medial wall of the antrum up to  and including middle turbinate.   Indications and contraindications for surgery and  radiotherapy are in similar lines as discussed earlier in this  chapter.   1. Retrosphenoidal syndrome from involvement of  cranial nerves II through VI manifests as unilateral  ophthalmoplegia, trigeminal neuralgia, ptosis, etc.    2. Retroparotid syndrome: Occurs due to compression  of cranial nerve IX through XII and causes various  symptoms depending on nerve involvement including  Homer’s syndrome.    Differential diagnosis of ulcer in the tongue  (Fig. 17.60 and Key Boxes 17.17 and 17.18)    Small, multiple, very painful ulcers, can occur at any  age group. More common in females at the time of  menstruation. These are called as minor aphthous  ulcers.    When they are larger, deeper and painful, they are called  major aphthous ulcers.    They are due to viral infection. These ulcers are super-  ficial ulcers with erythematous margin.    They subside within a few days. Temporary relief can  be obtained by applying salicylate gel.    Vitamin B complex is usually given for the satisfaction  of the patients.    e These ulcers occur due to broken tooth, sharp tooth, 1ll-  fitting dentures, prosthesis, etc. They are very painful  ulcers.   e Such ulcers are common on the lateral margin and they  heal when the tooth 1s removed. This is an example for  traumatic ulcer. It should not be confused with  carcinomatous ulcer which commonly occurs on the  lateral margin.   3. Tubercular ulcer of tongue  ¢ Tuberculosis affects tip of the tongue. These ulcers are  very painful with enlargement of regional nodes.  ¢ It occurs in patients with fulminating pulmonary  tuberculosis.  ¢ Ulcers have undermined edges. These ulcers are  sometimes multiple with serous discharge.  4. Gummatous ulcer  ¢ Gumma Is a complication of tertiary syphilis resulting  in a firm swelling in the midline in the anterior 2/3rd of  the tongue. Induration is absent. Ulcer 1s nontender.  Severe endarteritis obliterans results in the necrosis of  gumma giving rise to gummatous ulcer. It has punched  out edges and wash leather slough on the floor. Other  sites of gumma include testis, palate, clavicle and liver.  These ulcers are rare these days.  5. Systemic diseases  e Pemphigus  e Systemic lupus erythematosus (SLE)  e Lichen planus  6. Post-pertussis ulcer  e It occurs in children due to repeated coughing. Typical  location of the ulcer on the under surface of the tongue  and on the frenulum clinches the diagnosis.  7. Carcinomatous ulcer  ¢ Lateral margin  ¢ Nonhealing ulcer  e Everted edge  e Edge and base are indurated  ¢ Bleeds on touch  e Fixity  e Significant lymph nodes in the neck.    Diffuse painless enlargement of the tongue is described as  macroglossia. It is a rare condition and can occur due to various  causes.    1. Lymphangioma: In this condition, the tongue diffusely  enlarges. Sometimes, it is a localised swelling. It may be  associated with lymphangiomas elsewhere in the body such  as cheek mucosa, lips, etc. The tongue becomes larger,  indurated and gives rise to severe discomfort to the patients.  Due to repeated trauma, the surface becomes ulcerated. It  is treated by injecting sclerosants such as ethanolamine  oleate, hypertonic saline. Partial excision may be necessary  in cases of large lymphangioma.    2. Haemangioma: Cavernous haemangiomas occur in the  tongue, lips, etc. Itis present since birth but manifests during  childhood. It presents with soft, cystic, fluctuant swelling  and at times, pulsatile. Trauma due to teeth or food results    in bleeding. Haemangioma of the tongue is treated on the  same lines as lymphangioma. It is much more difficult to  excise it, especially a large haemangioma. Preoperative  angiography and ligation of lingual artery on both sides 1s  necessary.    3. Neurofibroma: It may be associated with von  Recklinghausen’s disease. Itis treated by hemiglossectomy.    4. Muscular macroglossia: This condition, though rare, is  seen in cretins. The tongue is thickened and cannot be held  in place. Hence, it protrudes outside. It 1s treated by partial  excision.    I. Primary syphilis: Primary chancre that occurs in the tongue  is highly contagious. It affects the tip of the tongue. It  produces a painful ulcer with large significant enlargement  of regional lymph nodes.    2. Secondary syphilis produces a white patch in the tongue,  lips and on the anterior pillars of fauces. In the tongue,  these are multiple which coalesce to form snail track  ulcers. The ulcers heal with fine tissue paper scar. Insome  cases syphilitic organisms produce a flat, hypertrophied  epithelium which is described as condyloma. This ts called  as Hutchinson’s wart.    3. Tertiary syphilis: It produces gumma. Syphilis also  produces chronic superficial glossitis, which is  characterised by bald tongue with loss of papilla and  fissured tongue. It is a precancerous condition.    Definition: Odontomes are the cysts, malformations arising  from epithelial or mesothelial elements of tooth resulting in  swelling of the jaw. As a developmental anomaly, few  epithelial cells proliferate, persisting as epithelial debris of  Mallasez. Three important odontomes have been given in  Table 17.4.    This arises from a normally erupted, chronically infected,  pulpless caries tooth. The caries tooth produces a low grade,  chronic inflammation which stimulates epithelial debris to  proliferate. Later this brings about degeneration of epithelial  and mesothelial cells resulting in a cyst within the maxilla.    ¢ It presents as a slow-growing swelling in the maxillary  region resulting in deformity of the face.    Excision of cyst with its epithelial lining through intraoral  approach. After excision of the epithelium the cyst wall should  be curetted, followed by soft tissue ‘push-in’ to obliterate dead  space.   e [It occurs in relation to unerupted, permanent, molar tooth,  most commonly the upper or lower third molar tooth.   ¢ This unerupted tooth constantly irritates the cells, produces  degeneration of the cells resulting in a dentigerous cyst.  The cyst 1s lined by squamous epithelium surrounded by  connective tissue. Within the cyst, the tooth lies obliquely  or sometimes is embedded in the wall of the cyst. As it  grows further, the cyst displaces the tooth to which it 1s  attached. Thus, the tooth is displaced deeper and deeper  and prevented from eruption.    ¢ Expansion of mandible—since the inner table of the  mandible is strong, the expansion mainly occurs In the outer    aspect of mandible. The bone gets thinned out resulting in  egg-shell crackling.   ¢ Itis a benign tumour, very slow-growing and behaves like  a basal cell carcinoma. Inadequate treatment results in local  recurrence and later metastasis, Hence, even though the  tumour 1s benign, it has to be treated like malignant tumour.   ¢ Tibia is the 2nd common site. It can be explained by  inclusion of abnormal embryonic epithelium.   ¢ Pituitary 1s another common site where adamantinoma can  occur. Both pituitary stalk and enamel arise from oral  epithelium.    ¢ Patients in 4th or Sth decade are commonly affected.  ¢ This is a slow growing jaw tumour in the region of angle of  mandible and horizontal ramus of the mandible.   ¢ As the tumour grows, it undergoes cystic degeneration  resulting in multiple cystic spaces. Hence, it 1s called  multilocular cystic disease.   ¢ As it grows it causes expansion of the outer table of the    ¢ Even though it is benign, simple curettage or enucleation  may result in recurrence and chances of recurrent  adamantinoma turning into malignancy are high. Hence,  wide excision with | cm of healthy normal tissue should  be removed. It may amount to segmental excision of the  mandible or hemimandibulectomy (Key Box 17.19 and  Table 17.4).    e Subperiosteal excision should not be done as it may result  in recurrence    e Incomplete excision results in recurrence and metastasis  to the lung    e Hence, even though it is a benign tumour it is treated by  wide excision or hemimandibulectomy.    It is a benign tumour which occurs due to haemorrhage with!  the bone marrow.   ¢ It affects antral part of maxilla or mandible causing;  enlargement of the jaw.   * Stroma is vascular consisting of thin-walled blood vessels  scanty collagen, connective tissue cells.   * Microscopic features mimic giant cell epulis or brow  tumour of hyperparathyroidism.   * Unlike an adamantinoma, this tumour affects females ir  the age group of 10-25 years.   * Painless enlargement of the jaw is the presenting feature  X-ray demonstrates radiolucent artery.   * Calcitonin 0.5 mg (100 units) daily subcutaneous injection  over a period of one year has been recommended as a first  line of treatment. It has shown resolution of the tumour.   ¢ Males between the age of 25 and 40 years are commonly  affected.   * As the tumour enlarges, both tables of the lower jaw are  thinned out.   * X-ray may show a large, radiolucent cyst with pseudo-  trabeculation.   * However, recurrence can occur and can turn into  malignancy like that of adamantinoma.   4. Palpation Smooth, thin bone-eggshell crackling | Smooth, expansion of the outer table Expansion of mandible in the 3rd molar  of mandible region, cystic areas   5S. X-ray Radiolucent unilocular cyst Radiolucent unilocular cyst with Large radiolucent area with fine honey-   Wide excision even though benign  Local spread, recurrence, late spread to  the lung are the features    ¢ Very often patients present with swelling on the gum which  is painless.    « It manifests as a mass of granulation tissue around the  teeth on the gums. It is a soft to firm, fleshy mass and  bleeds on touch.    * [tis the commonest form. A simple fibroma arising from  periodontal membrane, presents on the gum. It may  undergo sarcomatous change. It 1s a firm polypoidal mass,  slowly growing and nontender.   * It 1s an osteoclastoma arising in the jaw. It presents as  hyperaemic vascular, oedematous, soft to firm gums with  indurated underlying mass due to expansion of the bone.  It may ulcerate and result in haemorrhage. X-ray shows  bone destruction with ridging of walls (pseudo-  trabeculation).    Carcinomatous epulis  « This is an epithelioma arising from mucous membrane of  the alveolar margin.    ¢ Typically, it presents as a nonhealing, painless ulcer. It  slowly infiltrates the bone.    * Treated by wide excision which includes removal of  segment of the bone.    This is a sinus 1n the midline just beneath the mentum.    It 1s produced by an apical abscess of lower incisors which  penetrate buccal cortical plate below the origin of mentalis  muscle. This muscle takes origin from labial surface of alveolar  process just above the labial sulcus. Hence, pus discharges  through a sinus in the centre of chin.   « Patients present with recurrent swelling in the submental  region which bursts open spontaneously discharging at  times mucus and seropurulent fluid.   * Repeated history of swelling, discharge and healing are  common presentations.    We had a patient with discharging sinus in the region of  mentum for 1% years who had seen many practitioners  including a surgeon who curetted the lesion twice. However,  the lesion reappeared soon. This case is a classical example  of what the mind does not know, eyes cannot see.    * Diagnosis is established by examination of the oral cavity.  which reveals evidence of caries tooth.    It 1s an acute ulceromembranous stomatitis or acute ulcerative  gingivitis and stomatitis. The disease is caused by Vincent’s  organisms—Borrelia vincentii, an anaerobic spirochaete and  fusiformis. These are gram-negative rods which are the normal  pathogens of oral cavity.    * The disease starts in the intergingival defects as a deep  penetrating ulcer which results ina spontaneous gingival  haemorrhage. There is a thick membrane covering the  ulcer.   * Common in children and young adults between 20 and 40  years of age.   * Itpresents with very painful gums with fever, malaise and  toxaemia.   « Difficulty in swallowing, painful swallowing (odynophagia),  foetor oris, features of toxaemia and high grade fever are  characteristic of this condition.    * Since they are anaerobic organisms, metronidazole  400 mg thrice/day for 7—10 days, should be given.    * Cleft lip results from abnormal development of the mediar  nasal and maxillary process.   * Cleft palate results from a failure of fusion of the twc  palatine processes.    I. Central: It is very rare and occurs due to failure of fusior  of two median nasal processes.   II. Lateral: It is the commonest variety wherein there 1s a  cleft between the frenulum and the lateral part of the upper  lip. This is due to imperfect fusion of maxillary process  with median nasal process. The lateral variety can be  unilateral or bilateral.    I11.Complete or incomplete: In cases of complete variety, cleft  lip extends to the floor of the nose. In cases of incomplete  variety, the cleft does not extend up to the nostril.    IV. Simple or compound: Compound refers to cleft lip  associated with a cleft in the alveolus.    1. In 80% of the cases, cleft lip is unilateral and in about  60% of the cases it is associated with cleft palate.   Figs 17.70 and 17.71: Cleft lip (Courtesy: Dr CG Narasimhan,  Senior Consultant Surgeon, Mysore, Karnataka)    3. Maldevelopment or malalignment of the teeth in relation  to the cleft is common.    1. Presence of cleft lip does not interfere much with suck-  ing. However, there may be some difficulty in bottle  feeding.    ¢ Palate is developed around 6—8 weeks of intrauterine life  from 3 components. The premaxilla is developed from the  median nasal process and maxillary process contributes one  palatine process on each side.    ¢ The line of fusion of these processes is in the form of a  letter Y.    I. Complete: Failure of fusion of palatine processes and  premaxilla results in complete cleft palate. In such  situations, the nasal cavity and mouth are interconnected.  When premaxilla 1s not fused with both palatine processes,  it hangs down from the septum of nose. Thus, complete  cleft can be of two types as shown below in the diagram.    II. Incomplete: When the fusion of three components of  palate takes place, it starts from uvula and then backwards.  Thus, various types of incomplete fusion results.  ¢ Bifid uvula.  ¢ The whole length of soft palate 1s bifid.  ¢ The whole length of soft palate and the posterior part of   hard palate are involved. On the other hand, anterior  part of palate 1s normally developed. In about 25% of  cases, cleft palate alone and in 50% of cases, both cleft  palate and cleft lip are encountered.    1. Presence of cleft palate interferes with swallowing to some  extent.   2. They are unable to pronounce the consonant sounds such  as B, D, K, P, T.   3. Teeth: Upper lateral incisors may be small or even absent.  The maxilla tends to be smaller. Teeth are crowded.    4. Nose: Oral organisms contaminate the upper respiratory  mucous membrane through cleft palate.   5. Hearing: Even with repair, acute and chronic otitis media  and hearing problems can occur.    ¢ A multidisciplinary approach involving plastic surgery,  orthodontics, speech pathology, ENT department,  prosthodontics and paediatrics department is needed to  rehabilitate the cleft palate cases. This approach to the  problem results in aesthetically acceptable end result  without much functional deficiencies.   ¢ Feeding advice: Cleft palate babies are unable to suck  mothers’ milk because intraoral negative pressure cannot  be created due to communication between oral and nasal  cavity. Thus, expressed mother’s milk is given by spoon  with head end of baby elevated by 45 degrees. Swallowed  air during feeding 1s released frequently by burping.    Timing: Majority ofsurgeons follow “RULE OF 10” asa guide  for timing of lip and anterior palate repair. At the time of repair,  haemoglobin should be more than 10 g%, age approximately  10 weeks, weight more than 10 lb (4.54 kg) and total leukocyte  count less than 10,000/cu mm (1.e. no infection).    ¢ For unilateral cleft lip repair most commonly used methods  are Millard rotation advancement flap and Tennison-Randall  triangular flap method.    ¢ Bilateral cleft lip can be repaired in single stage or in two  stages at the interval of 3-6 months. For two stage repair,  any one of the methods described for unilateral cleft lip  can be used. For bilateral repair in one stage, Veau III  method is simple and gives satisfactory results. Other single  stage methods which give good results are Millard’s single  stage procedure and Black procedure.    *« Markings are made according to the method selected (e.g.  Millard’s repair, Fig. 17.73A and Tennison-Randall repair,  Fig. 17.73B).    « Adrenaline-saline solution (1:200,000) is injected in the  lip and labial sulcus for haemostasis.    ¢ Lip repair is done in three layers—mucosa, muscle and  skin. For better aesthetic result, Cupid’s bow should  become horizontal, white line continuity should be  repaired and there should be no vermilion notching.   Timing: Early repair results in retarded maxillary growth due  to surgical trauma to growth centre and periosteum. Delay in  repair results in speech defect. Best balanced result 1s achieved  by repairing between one and a half years.   ¢ Palate repair: Palate is repaired by palatal shelves.  Mucoperiosteal flaps raised from various methods are  available for palate repair.    ¢ Two mucoperiosteal flaps are elevated, one from either  side of palatal shelves. Then, nasal layers are mobilised.    ¢ [In V-Y push back palatoplasty, palatal lengthening 1s  achieved by V-Y plasty. Hook of hamulus can be fractured  to relieve tension on suture line by relaxing the tensor palati  muscle.    ¢ These tumours can also occur anywhere in the mouth or  pharynx   ¢ It is a slow-growing and painless tumour. At this stage,  it may feel firm or hard without ulceration of mucous  membrane.    ¢ Slowly it ulcerates. Thus, it can have the shape of a  ‘verrucous’ carcinoma. Such lesions are ulcerated, hard,  and painful with irregular margins.    ¢ Neglected cases can invade base of skull and spread to  lymph nodes.    e« Wide excision with or without reconstruction is the  treatment of choice.    ¢ They occur due to obstruction of the duct of many mucous  secreting glands which cover the inner surface of the lips  and whole of inside of mouth (Fig. 17.75).    ¢ Painless, slow growing swelling on the inner side of lip or  cheek.   ¢ Transillumination is positive but difficult to demonstrate  as the cysts are small.    A case of malignant melanoma of the oral cavity  (see the clinical notes below)   This 48-year-old man (Figs 17.76 to 17.78) presented with  an innocent looking, painless submandibular lymph node  enlargement. It was firm and nontender. Initially, it was  thought to be due to dental caries. Examination of the floor  of the mouth revealed an interesting melanomatous lesion.  Diagnosis was malignant melanoma with metastasis in nodes.  This is just to remind that melanoma can also occur in the  oral cavity (mucosa and firm skin).    ¢ Pyogenic granuloma: It is red in colour, soft and bleeds.  It may be associated with trauma or persistent infection.   ¢ Once mucous membrane is incised, swelling can be  dissected all around separating it from orbicularis oris/  buccinator muscle and it is removed.    e All the topics have been updated  e New photographs and key boxes have been added  e Reconstruction of the oral cavity has been dealt in detail  including lip  _° PMMC flap has been discussed in more detail.    . Which one of the following has highest chance of  malignancy?  A. Acanthosis  C. Leukoplakia    . Following malignant lesions in the posterior third of  the tongue can occur except:    . The investigation of choice in oral cancer to find out  the skull base involvement is:    . Which one of the following condition has high  incidence of distant spread?   Which is the most effective chemotherapeutic drug fo  head and neck cancers?   A. Central defects of lip—lower lip  B. Central defects of lip—upper lip  C. Lateral defects of more than 50%  D. Lateral defects up to 30%   Which one of the following swelling does not contain  cholesterol crystals?    ANSWERS  il gD 2 € 3B 4D 5D 6C “ib 8 D ey) 10 D  11 C 12 A 13 D 14 A 15 D 16D 17 A 18 D 19 A 20 D  21D 22 1B 23 A 24 A 25 b    There are three pairs of salivary glands—parotid,  submandibular and sublingual. In addition to these, there are  many (450) minorsalivary glands located in the cheek, mucosa,  lips, palate and base of the tongue. Parotid, the “big brother of  3”, suffers mainly from three diseases—infection, enlargement  and tumour. Submandibular salivary gland suffers from mainly  two diseases—sialoadenitis and tumours. Othersalivary glands  are of minor importance. However, it should be remembered  that the commonest tumour of minor salivary glands is  malignancy.    Parotid gland is present on the lateral aspect of the face, divided  by the facial nerve into superficial lobe and deep lobe.  Superficial lobe overlies the masseter and the mandible. Deep  lobe is wedged between the mastoid process and the styloid  process, ramus of the mandible and medial pterygoid muscle.    The superficial lobe also receives a duct from the accessory  lobe which is in the region of zygomatic arch/zygomatic  process. The duct of parotid, Stensen’s duct, 2-3 mm in  diameter, receives tributaries from superficial, deep and  accessory lobes, passes through the buccinator muscle and  opens in the mucosa of the cheek opposite the upper 2nd  molar tooth. Parotid gland is covered by a true capsule which  is a condensation of fibrous stroma of the gland and a false    capsule formed by parotid fascia, a part of the deep cervical  fascia.    After emerging from stylomastoid foramen, it hooks around  the condyle of mandible, enters the substance of the parotid  and divides into 2 major branches, zygomaticotemporal and  cervicofacial. Facial nerve along with retromandibular vein  (which is formed by the union of superficial temporal vein  and maxillary vein, formed from branches of pterygoid plexus  of veins) are present in this plane. This plane is called the  fasciovenous plane of Patey (Figs 18.1 and 18.2). The facial  nerve then gives rise to 5 branches which are interconnected    Temporal —->  frontalis  . Zygomatic  upper oat Frontalis and upper half of orbicularis  oculi  lower > Lower half of orbicularis oculi and muscles  below the orbit  . Buccal = Buccinator, orbicularis oris and a few    fibres of elevators of the lower lip  Muscles of the lower lip    like the foot of a goose, called Pes anserinus. Branches of  facial nerve in the face and the muscles supplied by these  nerves are given in Table 18.1.    Acute inflammation of the parotid can occur due to bacterial  or nonbacterial causes. It can be unilateral or bilateral  (Key Box 18.1). Three important causes and their treatment  are given below:    Mumps parotitis: Mumps! is an acute generalised viral  disease with painful enlargement of salivary glands, chiefly  the parotids. The virus belongs to Paramyxoviridae family  and only one serotype is known. The disease spreads from  a human reservoir by direct contact, airborne droplets  or fomites contaminated by saliva and possibly by urine  (Fig. 18.3).    Incubation period is 10-24 days. Fever, headache and  muscular pain are usually found. Both parotids are enlarged  with pain and temperature.    CAUSES OF ACUTE PAROTITIS  . Viral : Mumps—Commonest  Coxsackie A and B  Parainfluenzae 1 and 3  Echovirus  Lymphocytic choriomeningitis  . Bacterial Usually ascending infection  Staphylococcus aureus  . Recurrent Recurrent, mistaken for mumps  parotitis of Resolves at puberty  childhood  . Specific Mycobacterial, cat-scratch  infections disease, syphilis, toxoplasmosis  . Allergic Food and drugs    If symptomatic: Maintenance of good oral hygiene and  hydration is useful. Antibiotics may be given to prevent  secondary infection. One episode of infection confers  lifelong immunity.    of parotid produces serious illness with marked  engorgement of parotid. Typically, it produces parotid  abscess. Diabetes, malignancy, malnutrition increase the  risk. Decreased salivary secretion is an important  predisposing factor.    . Reduction in salivary juice: It can occur due to various    factors mentioned in the box. Postoperative parotitis can  be prevented by good mouth care and good oral hygiene.  Due to poor oral hygiene, ascending infection occurs from  the oral cavity resulting in parotitis (Key Box 18.2).    ¢ A patient who Is recovering in the postoperative period may  complain of pain and swelling in the parotid region.  Presence of severe pain with a very sick, toxic look and  high grade fever, chills and rigors indicates parotid abscess.  Diffuse brawny swelling is characteristic.   ¢ The swelling is due to inflammation of parotid and since it  is enclosed by parotid fascia, the swelling takes the shape  of parotid gland. However, it is not common for a parotid  abscess to raise the ear lobule. For the reason mentioned  above, fluctuation is a late feature. If the abscess is not  drained, it is likely to rupture into the external auditory  canal (Key Box 18.3).   ¢ The opening of the parotid duct may be inflamed and on  gentle compression of the parotid gland, pus can be seen  coming out of the parotid duct.   SWELLINGS WHEREIN ONE SHOULD NOT  WAIT FOR FLUCTUATION  e Parotid abscess  e Breast abscess  e Ischiorectal abscess  e Pulp space infection  e Any deep seated abscess   ¢ Maintaining good hydration of the patient in the post-  operative period.   ¢ Improvement in the oral hygiene—mouth washes with  potassium permanganate (KMnQ,) solution.   ¢ Appropriate antibiotics against staphylococci, such as  cloxacillin, is administered in the dose of 500 mg, 6th  hourly along with metronidazole 400 mg, 8th hourly to  treat anaerobic infections.   ¢ It takes about 3-5 days for the inflammation to settle  down.    ¢ Under general anaesthesia, an adequate vertical incision  is made in front of the tragus of the ear up to deep fascia.    Open the deep fascia in two or three places and drair  with blunt haemostat so as to avoid damage to facia  nerve. This is described as Blair’s method of drainage  of parotid abscess. A drainage tube has to be kept whick  can be removed after 3-4 days (Key Box 18.4).    ¢ Children between ages of 3 and 6 years are commonly  affected.   ¢ Aetiology is unknown, may be due to sialectasis (dilatation  of branches of salivary duct)   ¢ Recurrent pain and swelling of one or both parotids is  common.   ¢ A short course of antibiotics has to be given to cover  Streptococcus viridans.   Fig. 18.4: Superficial parotidectomy for recurrent parotitis. Please  remember chronic parotitis in children is pathognomonic of  HIV infection   ¢ Submandibular salivary gland is located in the sub-  mandibular triangle. It lies partly below and partly above  the mandible.   ¢ Itisinveryclose contact with the belly of the digastric muscle.  At surgery, once the deep fascia is opened, the intermediate  tendon of digastric is located and when it is retracted  downwards, mobilisation of the gland becomes easy.    infection. Despite control of acute symptoms with  antibiotics, the gland becomes chronically inflamed.   ¢ Calculi (80% of them occur in the submandibular salivary  gland) commonly occur in the duct and also within the  gland and produce recurrent sialoadenitis. Calculi are more  common in the submandibular salivary gland than in the  parotid gland because of the following reasons:   2. Calcium and phosphate content in the secretion is  high. Hence, 80% of them are radiopaque and are  detected by plain X-ray (Fig. 18.6).   3. Nondependent drainage of the secretions. Gland is  in the neck and the duct opens into the oral cavity.    ¢ Submandibular salivary gland is divided into a superficial  and a deep part by the mylohyoid muscle which forms the  oral diaphragm. During excision of the gland, a few fibres  of mylohyoid are also removed. When submandibular  salivary gland enlarges, it is bidigitally palpable because ,  the deep portion is deep to mylohyoid and it is in the '  floor of the mouth.   and deeply grooves the gland. It is ligated at this place  first during excision of the gland. After grooving the gland, I  it ascends laterally and curls around the lower border of  mandible to enter the face. It is ligated at this place also.    ¢ Salivary colic: It is a severe pricking type of pain which  is exaggerated at the time of meals. Salivary secretions are  induced by a meal or lemon (Lemon juice test). Asa result  of blockage due to a stone, the tension within the gland    CHRONIC SUBMANDIBULAR SIALOADENITIS increases, resulting in pain.  ¢ Lingual colic: If a calculus is situated within the sub-  ¢ Obstruction is the most important cause of submandibular mandibular duct where it is hooked by lingual nerve, the  Sialoadenitis. Trauma to the floor of the mouth is another  cause.  ¢ Obstruction can be due to stone, disease, stricture Of the  [qRRgRRRDRI IANNIS NGNII NON NINO =nO=RO = Rs= sine nsislsine insite ineas ‘  duct, or fibrosis of the papilla (calculus — most common). eB eal Ne  ¢ The causative organism is Staphylococcus. SUBMANDIBULAR SALIVARY GLAND  ENLARGEMENT  SIALOADENITIS DUE TO CALCULI ¢ Location—submandibular region    ¢ The disease starts with acute bacterial sialoadenitis which  occurs secondary to obstruction. The submandibular  gland has a poor capacity for recovery following    pain can radiate to the tongue as a result of irritation to the  lingual nerve.   Enlargement of salivary gland during meals is the  characteristic feature of salivary calculus. Classically  submandibular salivary gland swelling 1s located in the  submandibular region. It is firm in consistency witha lobular  surface. It 1s tender and both lobes are enlarged. It is  bidigitally palpable (Submandibular lymph node is  palpable only in the neck) both inside the oral cavity and  in the neck. The swelling reduces in size once the stimul!  are withdrawn (after meals).   The stone may be palpable within the gland (in the neck),  within the duct (intraorally), or sometimes it may be seen  at the orifice of the submandibular duct on the side of lingual  frenulum.   It is not uncommon to get a severe septic sialoadenitis with  gross swelling of the gland and inflammatory oedema  almost like Ludwig’s angina (Fig. 18.7).    An oblique lateral or posterior oblique occlusal radiography  may demonstrate a stone.  1. Stone in the submandibular duct: This can be  removed by incising the mucosa over the floor of the  mouth, after stabilising the stone. Removal of the stone   Protect 2 superficial nerves  Protect 2 deep nerves  Ligate facial artery 2 times  Divide 2 muscles    Incision is 2 cm medial to mandible, 2 cm anterior to angle of  the mandible    is followed by gush of old dirty contents of the  submandibular gland (Fig. 18.8).   2. Chronic sialoadenitis: This requires excision of  submandibular salivary gland. Three steps of dissection  of the gland include incision, mobilisation and excision  (Manipal Rule of 2—Table 18.2).   Incision: It should be a skin crease incision over the lower  pole of the gland, the posterior limit of the incision should  be at least 2 cm away from the angle of the mandible, to  avoid damage to the cervical branch of facial nerve. The  incision is deepened till the deep fascia is opened.  Mobilisation of the gland: Division of the facial artery  twice, once in a deeper plane on the posterolateral aspect  and another at the superolateral aspect close to the lower  border of the mandible is an important step which permits  mobilisation of the gland. Separation of the gland from  fibres of mylohyoid muscle by dividing small arteries  completes the mobilisation (Fig. 18.9).   Excision of the gland: It is done by ligating and dividing  submandibular duct.    even to hypoglossal nerve. Seroma and infection are the  other complications.   Cervical and marginal mandibular branches of facial nerve  Lingual and hypoglossal nerve   First at deeper plane and then at superficial plane  Superficial—platysma; Deep—fibres of mylohyoid  Superficial and deep    ¢ Transection of the nerve to mylohyoid muscle produces  anaesthesia of the submental skin.    A. Adenoma  1. Pleomorphic adenoma  2. Monomorphic adenomas  ¢ Adenolymphoma (Warthin’s tumour)  ¢ Oxyphilic adenoma (oncocytoma)  e Other types  B. Mucoepidermoid tumours  C. Acinic cell tumour  D. Carcinoma  ]. Carcinoma in pleomorphic adenoma  2. Adenoid cystic carcinoma  3. Undifferentiated carcinoma  4. Adenocarcinoma  5. Epidermoid carcinoma  6. Acinic cell tumour  7. Mucoepidermoid carcinoma  8. Malignant mixed tumour.    Salivary gland tumours are not uncommon. There are dozens  of histological types of salivary gland tumours. However,  pleomorphic adenoma and adenolymphoma are the common  benign types. Carcinoma arising in pleomorphic adenoma,  mucoepidermoid tumours and adenoid cystic carcinoma are  important malignant tumours.    307  Incidence: 80% of salivary gland tumours are found in the  parotid gland. Out of these, 80% are benign, of which 80%  are pleomorphic adenomas.   In the submandibular salivary gland, 50% are benign and  50% are malignant.   In the minor salivary glands, 90% are malignant. Thus,  the incidence of malignancy increases from major to minor  salivary glands.    ¢ Epithelial cells proliferate in strands, or may be arranged  in the form of acini or cords.   ¢ There are also myoepithelial cells which proliferate in  sheets. They are called spindle-shaped cells.   ¢ The tumour produces mucoid material, which displaces and  separates the cells resembling cartilage in histological  section.    Because of the presence of epithelial cells, myoepithelial  cells, mucoid material, pseudocartilage and lymphoid  tissue, the tumour ts called pleomorphic adenoma.    ¢ As the tumour grows, it compresses the normal parotid  tissue and the branches of the tumour penetrate the thin  capsule and enter the substance of the parotid. Simple  enucleation will result in a recurrence. Hence, superficial  parotidectomy has to be done.    1. Middle-aged women, around 40 years, are commonly  affected (Fig. 18.10) (female, fifth decade and fullness near  ear lobule).    2. Typically, a history of a very slow growing swelling (for  a few years) is usually present.   3. The swelling 1s painless. Any painless swelling near  the ear is best assumed to be parotid gland neoplasm  unless proved otherwise (Fig. 18.11).    1. Parotid swelling has the following classical features:  ¢ It presents as a swelling in front, below and behind  ear.  ¢ Raises ear lobule.  ¢ Retromandibular groove is obliterated.  2. Itis rubbery or firm. Soft areas indicate necrosis. In long-  standing cases, it can be hard. Surface can be nodular or    CLINICAL EXAMINATION OF PAROTID mero Oy”  e Swelling firm, nodular  e Facial nerve involvement  (80-90% cases of malignancy)  e Fixity to mandible and masseter  e Deep cervical nodes  e Opening of parotid duct  e Shift of tonsil and pillar of the fauces  e Other salivary glands (both sides).   sometimes bosselated. Skin ts stretched and shiny. Howeve  being a benign tumour, it 1s neither adherent to the skin ne  to the masseter (Figs 18.12, 18.13 and 18.17).   3. After a few years, pleomorphic adenoma may show feature  of transformation into malignancy (carcinoma exple«  morphic adenoma).    ¢ Approximately 10% of the parotid tumours are behind th  facial nerve in the deep lobe.   e This is appreciated by intraoral examination wherein th  tumour presents with a parapharyngeal mass whic  displaces the tonsil or soft palate medially.    (Courtesy: Dr Sreejayan, Prof of Surgery, Calicut Medical College, Calicut, Kerala)   Fig. 18.15: Large sebaceous cyst in the preparotid region. It had  sebaceous punctum    KEY BOX 18.  DIFFERENTIAL DIAGNOSIS OF SWELLING  IN THE PAROTID REGION  1. When it involves the preparotid region and angle of  mandible  e Preparotid lymph node enlargement: It may be enlarged  due to tuberculosis, metastasis or in non-Hodgkin’s  lymphoma. Sebaceous cyst in pre-parotid region  (Fig. 18.15)  e Preauricular dermoid cyst (young patients)  e Mesenchymal tumours such as lipoma, neurofibroma  e Haemangioma, lymphangioma (Fig 18.16)  2. When it involves lower pole of parotid  e Upper jugular chain lymph node enlargement—  metastatic or tubercular  e Branchial cleft cyst  e Epithelial inclusion cyst    Slow growing parotid tumours should not be subjected to  biopsy for 2 reasons:    Fig. 18.16: Parotid cyst in a young boy—lIt was fluctuant and  transillumination-negative. It was a haemangioma    ¢ Injury to the facial nerve  ¢ Seeding of tumour cells in the subcutaneous plane which  causes recurrence in about 40-50% of cases.    I. Fine needle aspiration cytology (FNAC) is done to  confirm the diagnosis and rule out malignancy.    2. CT scan is done when the tumour ts arising from the deep  lobe. It helps to define the extraglandular spread, the  extent of parapharyngeal disease, cervical lymph nodes and  bony infiltration.    3. FNAC of the lymph nodes that are palpable in the neck in  cases of malignancy of the parotid gland.    4. X-ray of the bones (mandible and mastoid process) to look  for bony resorption, 1f malignancy 1s suspected.    5. MRI is a better investigation. However, it is expensive—  CT Scan and MRI lack specificity for differentiating  between benign and malignant lesions.   1. Suspected bone destruction at skull base  2. Suspected involvement of mandible   ¢ It 1s the standard surgery done for benign pleomorphic  adenoma. It means removal of the entire lobe containing  the tumour which ts superficial to the facial nerve. Facial  nerve should always be preserved. Enucleation should  never be done as it causes recurrence and can injure facial  nerve. It is difficult to remove a recurrent tumour.    ¢ Trace the posterior belly of digastric up to the  mastoid process. Facial nerve is in between the muscle  and tympanic plate.   3. Developing a plane: Facial nerve and retromandibular  vein divide the parotid gland into superficial and deep  lobes. Benign tumours do not invade this faciovenous  plane of Patey.   4. Gentle handling, good suction and perfect haemostasis  help in clear recognition of the nerve.   5. In toto removal—The tumour along with the lobe to  avoid spillage (which is one of the causes of recurrence).    Fig. 18.18: Conservative superficial parotidectomy: Facial  avoid haematoma, wound infection, etc.    nerve divides the gland into superficial and deep lobes. The entire  superficial lobe is removed preserving the facial nerve    KEY BOX 18.8 WHAT SHOULD BE DONE IN  a PAROTID SURGERY  CONSERVATIVE SUPERFICIAL e Wide exposure by an adequate skin flap  PAROTIDECTOMY ¢ Always identify facial nerve (best way to avoid injury).   ¢ Preserve the facial nerve, even in malignant tumours e Always try to preserve facial nerve even in malignancies  untess grossly involved. unless it is directly infiltrated.   ¢ Avoid rupture of the gland (Fig. 18.19) e If facial nerve is excised, try to reconstruct immediately by    [. Adequate exposure by an incision which starts in front of Total parotidectomy with the excision of facial nerve when  tragus of ear, vertically descends downwards, curvesround nerve is involved.  the ear lobule up to the mastoid process and is carried    along with parotid gland and facial nerve  2. Recognising the facial nerve at surgery (Fig. 18.19) e Skin  - Facial nerve lies 1 cm inferomedial to the bony ~ {nfratemporal fossa  cartilagenous junction of external auditory canal) . \jandible  (Conley’s pointer). - TM joint   Postoperative radiotherapy  Fig. 18.19: Superficial parotidectomy specimen ¢ T3/T4 cancer: Adenoid cystic carcinoma recurrent tumours   ¢ Adenolymphoma is not a lymphoma. It 1s a misnomer (vide  infra).   ¢ It is a benign parotid tumour and next common to  pleomorphic adenoma. It constitutes about 10% of parotid  tumours.   ¢ Origin of adenolymphoma: During development some  parotid tissues get included within lymph nodes (preparotid)  which are present within the parotid sheath.    Histology  ¢ It is composed of double layered eosinophilic epithelium.  The inner cells are columnar.    ¢ Presence of lymphatic tissue in the stroma, andlymph follicles  is characteristic of adenolymphoma (hence the name).   ¢ It has smooth surface, round border with soft, cystic  consistency (Fig. 18.20).   ¢ Classically, situated at the lower pole of parotid elevating  the ear lobule. Sometimes it may be multicentric.   ¢ This tumour affects only parotid gland (very, very rarely  other glands may be affected).    ¢ It has got a well-defined capsule. Hence, enucleation used  to be done earlier but not now. Superficial parotidectomy  is the treatment of choice.   ¢ As the name itself suggests, it consists of sheets of  epidermoid cells and cystic spaces lined by mucus secreting  cells (Fig. 18.21).   ¢ In childhood, it is the commonest parotid tumour. They  are benign, slow-growing but hard in consistency.  (Adenolymphoma and mixed tumours are firm but  mucoepidermoid tumour is hard). Parotid is the commonest  site. In cases of minor salivary glands, palate is the  commonest site.   ¢ Mucoepidermoid tumours can infiltrate local tissues, lymph  nodes or skin. Hence, a few consider that mucoepidermoid  tumours are always carcinomatous.   ¢ Well-differentiated tumours behave like benign tumours,  intermediate ones are aggressive and undifferentiated  tumours metastasise early. Mucoepidermoid carcinoma is the  most common malignant epithelial neoplasm of salivary gland.   ¢ The low grade tumours are composed of predominantly  mucus secreting cells. High grade tumours have pre-  dominantly epidermoid cells.   ¢ Benign tumours need excision and malignant tumours need  radical parotidectomy. Radiation is required in the post-  operative period.    ¢ These are the uncommon parotid tumours. Commonly occur  in women.   ¢ The cells resemble those of serous acini and this tumour  also has properties of invasion such as mucoepidermoid  tumour. It tends to be soft and sometimes cystic.    ¢ Also called oncocytoma. It occurs exclusively in the parotid  gland. It is a solid tumour and occurs 1n the sixth decade of  life.    - Itisahighly malignant tumour consisting of cords of dark (Figs 18.22 to 18.29 and Table 18.4)  staining cells with cystic spaces containing mucin. It also To find out the exact type of malignant tumour is of interest  consists of myoepithelial cells and duct epithelium. to pathologists. Clinically, one can suspect malignancy when  a salivary tumour has one of the following features:  ¢ Rapidly growing neoplasm  ¢ Change in consistency (the tumour tends to be hard)  ¢ Fixity to underlying muscle such as masseter as in parotid  e These tumours have a high incidence of distant metastasis tumours  but in general they display indolent growth. Skip lesions. _Fixity to mandible as in parotid or submandibular tumour  are common as it spreads along the nerve tissue, which . [nyolvement of facial nerve as in 80% of cases of    ¢ Eventhough slow-growing, it spreads along the perineural  tissue, may invade periosteum or medullary bone at a  distance. This bone resorption results in bony tenderness.    leads to treatment failure. malignant parotid tumours.  ¢ Local infiltration, lymphatic and blood spread, and local ° Resorption of adjacent bone such as mastoid, tenderness  recurrence are important features. as in adenoid cystic carcinoma.   ¢ They are treated by radical sialadenectomy with radical  block dissection of the neck. Radiotherapy is used as a   ¢ Early cases are treated by radical parotidectomy with block palliative treatment.  dissection of the neck. However, many cases present late  to the hospital. Thus, palliative radiotherapy is given to  reduce pain and to arrest progress of the disease.    ¢ It is hard and fixed and can produce anaesthesia of the  skin overlying the tumour.    e Most common type of cancer arising in the parotid glands    e Most common site of squamous cell carcinoma is sub-  mandibular salivary gland    e Lymph nodes Selective neck dissection—supraomohyoid neck Selective neck dissection (lateral) levels II, Il, IV  dissection and V lymph nodes    It occurs after surgery for parotid tumours, surgery in the  region of temporomandibular joint or due to injury to the  parotid gland. Injury to the auriculotemporal nerve can occur  at a site where it turns around the neck of the mandible. The  injury manifests ata later date, e.g. 2-3 months (Fig. 18.36).  Because of the injury, postganglionic parasympathetic  fibres from otic ganglion unite with sympathetic fibres  of superior cervical ganglion which supplies the vessels  and sweat glands over the skin overlying parotid region  (Key Box 18.12).    Ds.  PARTS SUPPLIED BY  AURICULOTEMPORAL NERVE  Auricular part:  - External acoustic meatus  - Tympanic membrane surface  - Skin of auricle above external acoustic meatus  e Temporal part: Hairy skin of the temple.    As a result of this, whenever the act of chewing o1  mastication is started,  there is increased  sweating and hyper-  aesthesia in the region  supplied by auriculo-    neous branch of  mandibular division ,  of trigeminal nerve).  Hence, it is called )  auriculotemporal  syndrome.  Diagnosis: Starch iodine test—paint the affected area with  iodine and allowed it to dry before applying the dry  starch.    The starch turns blue on exposure to iodine in the presence  of sweat.    Principle is to provide a barrier between the skin and parotid  bed by using temporalis fascial flap or sternomastoid  muscle flap.   3. Latest treatment includes injection of botulinum toxin into  the affected skin (see page 441 also).    It 1s the diffuse infiltration of salivary and lacrimal glands  with lymphocytes resulting in enlargement of glands and slow  destruction of acini. Thus, clinical features include dry eyes  (keratoconjunctivitis sicca) and dry mouth (xerostomia). These  along with a third component rheumatoid arthritis, form the  triad of Sjégren’s syndrome (primary).  ¢ 30% of patients with systemic lupus erythematosus and all  patients with primary biliary cirrhosis develop Sjégren’s  syndrome. This is termed secondary Sjégren’s syndrome.  ¢ Other features: This disease is 10 times more common in  females and presents with painful enlargement of the glands.  ¢ Complications  1. Lymphomatous transformation (high in primary).  2. Oral candidiasis.    Due to autoimmune mechanism, symmetrical enlargement of  all salivary glands and lacrimal gland enlargement occur. Dry  mouth and narrow palpebral fissures are diagnostic of this  condition.    5. Granulomatous sialoadenitis  These are rare, painless swellings. Following are the causes:  ¢ Tuberculosis    It is an uncommon condition which commonly occurs after  surgery on the parotid gland (Key Box 18.13).   e Any surgery on the parotid gland—superticial parotidectomy,  drainage of abscess, surgery for carcinoma cheek, facio-  maxillary trauma are the causes.   e Exploration and excision of fistula and ligation of duct is  required.    ¢ Even though they are called minor, numberwise they are  major (many), about 450 in number.   ¢ They can present as mucus retention cyst (common in lip)  or as malignant tumour.   e Since they are submucosal, they start as a submucous nodule  (very important point in the history) to differentiate from  carcinoma buccal mucosa/lip, etc.   ¢ As they grow, they ulcerate. Ulceration is a feature of  malignancy.   ¢ Treatment of benign cyst/tumour is by simple excision and  malignant tumour 1s by wide excision.    1. Early immediate nerve repair, 1n case of injury to the nerve.  2. Late nerve crossing by suturing peripheral branches of  facial nerve to one of the following nerves:  ¢ Hypoglossal nerve, spinal accessory nerve  ¢ Phrenic nerve.  3. Surgery to achieve movement in long standing facial palsy  (usually after | year).    Suspension of lips, cheek and angle of mouth to zygomatic  bone or temporal fascia using fascia lata, palmaris longus  tendon or other alloplastic materials.   Lateral tarsorrhaphy (canthoplasty) to prevent exposure  keratitis due to widened palpebral fissure.    Muscle transfer with carefully preserved muscle nerve and  vessel, e.g. temporalis muscle transfer, masseter muscle  transfer.   Cross face nerve transplantation using sural nerve. Using  microscope, sural nerve is sutured to the two or three  relatively insignificant branches of facial nerve (selected  by intraoperative electric stimulation) on normal side. Other  end of the sural nerve is sutured to distal end of the divided  facial nerve on paralysed side.    Preoperative assessment of motor and sensory system  Microsurgical tools should be available   Tension free repair should be done. If tension free repair is  not possible, then nerve graft can be used   Ends prepared with microscissors. First suture to be loose  to ease the alignment of other sutures.   Avoid postural manoeuvre to avoid tension. Nerve can be  mobilised 1—2 cm proximally and distally for tension free  suturing but not more than that.    To place a polyglycolic acid tube to bridge the gap rather  than to perform nerve grafting.   — Nerve regeneration and proximal and distal axonal  matching result from a combination of neurotropism and  contact guidance.   — The graft should be oriented in a reverse fashion frorr  its native position so that the regenerating fibres are not    diverted from the distal neurorrhaphy site and distal  stump. Misalignment should be avoided.    If the nerve gap 1s small, fascicular matching can be done   — Posterior interosseous nerve, medial antebrachial cutaneous  nerve for small digital nerves, sural nerve for nerve gaps  involving larger nerves, greater auricular nerve.    Drawbacks of nerve graft that become the merits of nerve  transfer:    ¢ Timely re-innervation may not be possible if the target  muscle 1s too far from the regenerating site.    A nerve transfer converts a high level nerve injury to a low  level nerve injury by recruiting expendable nerve fascicles  from the donor nerve to innervate critical nerves close to  their target end organs.    Donor nerves are preferentially selected according to their  proximity to the target site    e Granulomatous sialoadenitis, peripheral nerve repair  and transfers have been added.    . The investigation of choice in deep lobe tumours of  parotid gland is:  A.CT scan   B. Location of facial nerve in relation to posterior belly  of diagstric    Which benign tumour of parotid produces Hot Spot  in Technetium scan?    Following are true for precancerous lesions of lip/ oral  cavity except:    Which one of the following swelling does not contai  cholesterol crystals?    ANSWERS  1 4G 2C 3 C 4 D s D 6 C 7A 8 D 2, 16.4  11. D 12 6 13 B 14 A 15 D 16 D 17 A 18 D 19 A 20 D  21 D 22 B 23 A 24 D 25 D    Thyroid gland 1s an endocrinal gland present in the neck  secreting T3 and T4hormones. It is richly vascular and highly  functional. Effects of hormonal changes affect every part of  the body such as central nervous system, cardiovascular  system, gastrointestinal system and reproductive system. It 1s  also the site of various diseases—a simple enlargement,  toxicity and malignant transformation.   * It develops from median down growth (midline  diverticulum) of a column of cells from the pharyngeal  floor between first and second pharyngeal pouches.   * By 6weeks of intrauterine life, the central column, which  becomes the thyroglossal duct, gets reabsorbed.   * Pyramidal lobe is formed by a portion of the duct.   * Calcitonin producing parafollicular or C cells originate  from the fourth branchial pouch.    pretracheal fascia which 1s a part of deep cervical fascia.  It has a right and left lobe joined by the isthmus in front    of 2nd, 3rd and 4th tracheal rings. It weighs about  20-25 g. A projection from the isthmus usually on the left  side 1s called pyramidal lobe. It 1s attached to the hyoid  bone by a fibrous band or muscle fibres called levator  glandulae thyroideae.    * Suspensory ligament of Berry: This pair of strong  condensed connective tissue binds the gland firmly to each  side of cricoid cartilage and upper tracheal rings.    Pretracheal fascia, which is part of deep cervical fascia  splits to invest the gland. These structures (ligament of  Berry and pretracheal fascia) are responsible for thyroid  gland moving with deglutition.    Superior thyroid artery, a branch of the external carotid  artery, enters the upper pole of the gland, divides into  anterior and posterior branches and anastomoses with  ascending branch of inferior thyroid artery. Since the upper  pole 1s narrow, ligation 1s easy.   Inferior thyroid artery is a branch of thyrocervical trunk’  and enters the posterior aspect of the gland. It supplies the  gland by dividing into 4 to 5 branches which enter the  gland at various levels (not truly lower pole).   Inferior thyroid artery used to be ligated well away from  the gland to avoid damage to RLN. However, ligation of  these arteries on both sides will cause permanent hypopara-  thyroidism. Hence, the current practice is to identify and  ligate the branches of inferior thyroid artery (3—4) separately.  Thyroidea ima artery 1s a branch of either brachiocephalic  trunk or direct branch of arch of aorta and enters the lower  part of the isthmus in about 2 to 3% of the cases.    Superior thyroid vein’? drains the upper pole and enters  the internal jugular vein. The vein follows the artery.    Middle thyroid vein is single, short and wide and drains  into internal jugular vein.   Inferior thyroid veins form a plexus which drain intc  innominate vein. They do not accompany the artery.  Kocher’s vein is rarely found (vein in between middle  and inferior thyroid vein).    Superior laryngeal nerve: The vagus nerve gives rise to  superior laryngeal nerve, which separates from it at skull  base and divides into two branches. The larger internal  laryngeal nerve Is sensory to the supraglottic larynx. The  smallerexternallaryngeal nerve runs close to the superior  thyroid vessels and supplies cricothyroid.  ¢ This nerve is away from the vessels near the upper pole.  Hence, during thyroidectomy, the upper pedicle should  be ligated as close to the thyroid as possible.    Recurrent laryngeal nerve (RLN) is a branch of vagus,  hooks around ligamentum arteriosum on the left and  subclavian artery on the right and runs in the  tracheoesophageal groove near the posteromedial surface.  Close to the gland, the nerve lies in between (anterior or  posterior) branches of inferior thyroid artery (Figs 19.3  and 19.4 in the Riddle’s triangle).    ¢ On the right side, itis 1 cm within the tracheoesophageal  groove (Key Boxes 19.1A and B).    KEY BOX 19.1A  RECURRENT LARYNGEAL  NERVE AT SURGERY  Lack of colour—white in colour  e Lack of elasticity  Lack of pulsation  e Longitudinal vein on the surface  e Longitudinal course  e Location—Riddle’s  triangle—t is between  inferior thyroid artery  superiorly, carotid  artery laterally and  trachea medially. From  here RLN runs up-  wards to enter larynx  at greater cornu of  thyroid cartilage    'Branches of thyrocervical trunk can be remembered as SIT—suprascapular, inferior thyroid and transverse cervical artery.  *Superior thyroid artery and vein are like newly married couple, they go together, hand in hand.  3Middle thyroid vein is single, a bachelor and inferior thyroid artery and vein are a divorced couple.    * Subcapsular lymphatic plexus drains into pretracheal nodes  (delphic nodes means uncertain) and prelaryngeal nodes  which ultimately drain into lower deep cervical nodes and  Superior mediastinal nodes (Fig. 19.5).  thyroid artery ¢ The chief lymph nodes are middle and lower deep cervical  lymph nodes (Levels III and IV).  * Supraclavicular nodes and nodes in the posterior triangle  can also be involved in malignancies of the thyroid gland,  especially papillary carcinoma thyroid.   ¢ In the centre, colloid is present which is secreted from  epithelial cells in response to calcitonin.    Fig. 19.38: Course of recurrent laryngeal nerve—gland is  mobilised. The branches of inferior thyroid are ligated (not the main  trunk)    NERVE ANOMALIES  e The nerve traverses through the gland in about 5-8% of  cases.  e The nerve may be very closely adherent to the posteromedial  aspect of the gland.  e Nerve notseen—may be far away in the tracheo-oesophageal  groove.   1 in 1,000 cases. The nerve has a horizontal course. Fig. 19.6: Diffuse colloid goitre—Acini lined by cuboidal follicular  ¢ In 25% of the cases, it is within the ligament of Berry. cells with luminal colloid (Courtesy: Dr Laxmi Rao, HOD Pathology,  = KMC, Manipal)    Tri-ilodothyronine (T3) and thyroxine (T4) are the hor-  mones secreted by the thyroid gland. Dietary requirement of  iodine per day is 100-200 micrograms or 0.1 mg. Sources  of iodine are milk, dairy products and sea food including  fish.    Steps involved in the synthesis of these hormones  1. Iodide trapping from the blood into the thyrocyte is the  first step in the formation of T3 and T4.    2. Oxidation of iodide to inorganic iodine: This step needs  the enzyme peroxidase.    ¢ Drugs which block this stage (thioamides) are  sulfonamide, PAS (para-amino-salicylic acid),  carbimazole and propylthiouracil.    ¢ Coupling of two molecules of DIT results in T4 and one  molecule of DIT and MIT results in T3.    ¢ The hormones combine with globulin to form a colloid-  thyroglobulin. They are stored in the thyroid gland and  released as required by process of hydrolysis.    Serum T3 and T4 estimation is most commonly performed.  Other tests are not commonly done and some of them are  obsolete (Table 19.1).    ¢ Most (80%) T3 is produced by deiodination of T4 in the  liver, muscle, kidney and anterior pituitary.    ¢ T3 is 3 to 4 times more potent than T4.    ¢ The half-life of T3 1s approximately 24 hours, whereas  half-life of T4 is about 7 days.   ¢ Free T3 (3 to 9 pmol/L) is most useful in confirming the  diagnosis of early hyperthyroidism, especially in  pregnancy where in levels of free T4 and free T3 rise  before total T4 and T3.    ¢ In euthyroid state, T4 is the predominant hormone  produced by the thyroid.    ¢ Total T4 levels reflect output from the thyroid gland.  Both T3 and T4 increase cell metabolism, normal growth,  facilitate normal mental development and increase local  effects of catecholamines.    0.3-5 IU/ml of plasma. Table 19.1 shows the levels of T3,  T4 and TSH in a few common conditions.    ¢ It is produced by thyroid tissue only. Hence, the levels  should be low after total thyroidectomy.    ¢ The most important use of this test 1s to monitor patients  after total thyroidectomy for well-differentiated  carcinoma.    ¢ Itis not normally released into circulation in large amount  but increases suddenly in thyroiditis, Graves’ disease or  toxic multinodular goitre (MNG).    patients with Hashimoto’s thyroiditis and 80% of patients  with Graves’ disease have antibodies which are called  as ‘LATS’ (long acting thyroid stimulator). The detection  of these antibodies help in the diagnosis of such cases  and also to suspect these diseases before clinical mani-  festation.    i Low dose radiation (30 mrad) Shorter 8-10 hours Well differentiated carcinoma for  bony metastasis.   99m Tc (technetium) ~— Lowest radiation (5 mCi) Shorter half-life Sensitive for nodal metastasis  6-8 hours Lingual thyroid. Fig. 19.7  _ Nomenclature of certain thyroid diseases  ¢ Ectopic thyroid ¢ Thyroid tissue along the line of descent  Diagnosis by isotope scan or CT scan Example: Floor of the mouth, submental region, mediastinum  ¢ Lingual thyroid ¢ Swelling in the region of foramen caecum in tongue  Diagnosis by isotope scan or CT scan  ¢ Dyshormonogenesis ¢ Autosomal recessive condition with deficiency of peroxidase or dehalogenase (enzymes)  It is familial.  ¢ Pendred’s syndrome ¢ Dyshormonogenesis with congenital deafness  ¢ Struma ovarii ¢ Malignant ovarian teratoma containing thyroid tissue  ¢ Jod Basedow’s disease (German word) e Excessive iodine given for hyperplastic goitre resulting in hyperthyroidism   1. Swelling: Long duration of thyroid swelling indicates congestive cardiac failure can cause dyspnoea and  orthopnoea.   e Short duration with rapid growth indicates malignancy  such as anaplastic carcinoma. Majority of thyroid  swellings do not produce pain.    ¢ Long-standing MNG compresses on the tracheal  cartilages and produces pressure atrophy of tracheal  cartilages. This is called tracheomalacia.    4. Dysphagia is relatively uncommon because oesophagus  is a posterior structure and it is a fibromuscular tube.    2. Rate of growth: Usually slow-growing in benign disease. 5. Hoarseness of voice indicates malignancy. It always   3. Dyspnoea: Difficulty in breathing in a patient with goitre ¢ Tremors of the hand   ¢ Prominent eyes are observed by other persons. Double  vision, oedema of the conjunctiva can be the presen-  ting complaints in late cases.   B. Cardiovascular symptoms (CVS) are predominantly seen  In secondary thyrotoxicosis. Even though both forms of  thyrotoxicosis produce palpitations, it is a significant  complaint in multinodular goitre with thyrotoxicosis  (secondary thyrotoxicosis). Precordial chest pain and  dyspnoea on exertion are late manifestations of secondary  thyrotoxicosis (Summary—Fig. 19.8)   SWELLINGS WHICH MOVE UPWARDS  WITH DEGLUTITION  Thyroid swellings  Subhyoid bursitis  Pretracheal and prelaryngeal lymph nodes  Thyroglossal cyst  Laryngocoele    ligament of Berry. These are pairs of ligaments attached  above to cricoid cartilage. During deglutition, the cricoid  cartilage moves upwards and with it, the thyroid gland  (give the patient a glass of water and check for movement  with deglutition).   ¢ If there is restriction of movement, it can be due to  — Malignancy with fixity to the trachea  — Retrosternal goitre    Movement on protrusion of the tongue suggests  thyroglossal cyst. This test should be done when there ts a  nodule or a cyst in the region of isthmus of the thyroid  gland. This test has no relevance in cases of MNG or other  thyroid swellings.    1. The location of the swelling is in front of the neck, extend-  ing from one sternomastoid to the other sternomastoid,  vertically from suprasternal notch to the thyroid cartilage.   ¢ Thyroid is enclosed by pretracheal fascia which ts  condensed to form a ligament posteromedially called    rise of temperature is a feature of toxic goitres. Very large  nodular surface is described as bosselated surface (Figs 19.9  and 19.10).    ¢ Soft: Graves’ disease, colloid goitre.  ¢ Firm: Adenoma, multinodular goitre.  ¢ Hard: Carcinoma, calcification in the MNG.    . Intrinsic mobility of the gland is very much restricted in    Figs 19.9 and 19.10: Endemic goitre of 35 years duration turned into multinodular goitre. She presented with 3 months history of rapid  increase in the size of the swelling. She underwent total thyroidectomy. Final histopathology report was follicular carcinoma thyroid.  Observe large nodular surface—bosselations. (Courtesy: Dr Chidananda KV, Prof and Head of the Department and  Dr Gopinath Pai, Prof of Surgery, KVG Medical College, Sullia, Dakshina Kannada, Karnataka. MBBS exam case 2007)    . Sternomastoid contraction test is done when only one  lobe is enlarged. In this situation the examiner keeps the  hand on the side of the chin, opposite the side of the lesion  and asks the patient to push his hand against resistance. If  the gland becomes less prominent (as with thyroid  swellings), it indicates the swelling is deep to the  sternomastoid muscle.  . Chin test (neck fixation test) 1s classically done in multi-  nodular goitre, wherein both lobes are enlarged. The patient  is asked to bend the chin downwards against resistance.  This produces contraction of both sternomastoids and strap  muscles and the gland becomes less prominent (Fig. 19.11).  . Special tests or methods of examination of thyroid gland:  a. Crile’s method is indicated when there is a doubtful  nodule. Keep the thumb over the suspected area of the  nodule and ask the patient to swallow. The nodularity is  appreciated better with this test (Fig. 19.12).  b. Lahey’s method of examination of thyroid can be done    goitres causing tracheomalacia and carcinoma with  infiltration into trachea may give rise to stridor.    from front as well as behind. In order to palpate the right —g___pgsition of trachea: In cases of solitary nodule confined to  lobe, push the gland to the right side and feel the nodules one lobe, trachea is deviated to the opposite side. However,  in the posteromedial aspect of the gland. The lobe in cases of multinodular goitres, trachea need not be deviated  becomes more prominent and thus nodules are because of symmetrical enlargement of both lobes.  appreciated better.    with head extended. Thyroid gland becomes more  prominent and thus, palpation becomes better.   d. Kocher’s test: If gentle compression on lateral lobes  produces stridor, it is described as positive. This is due  to scabbard! trachea. Long-standing multinodular    mastoid process to sternoclavicular joint. Then draw a  horizontal line from upper border of thyroid cartilage. The  point where these two lines meet is the site of bifurcation  of common carotid artery. This artery should be palpated  just below this point.    'Scabbard is a sheath for holding a sword or other large blade (it is narrow and curved).    ¢ In large multinodular goitres, the artery may be pushed  laterally. Hence, pulsations are felt in the posterior  triangle. Carcinoma of the thyroid engulfs the carotid  sheath. Consequently, pulsations may be absent. Absent  carotid artery pulsation is called Berry sign positive.  Since the lumen is not narrowed, superficial temporal  artery pulsations are felt normally.   PALPATION TESTS  e Local rise of temperature, size, shape, surface, borders  e Consistency, movement with deglutition  e Intrinsic mobility test  e Sternomastoid contraction test and chin test  e Position of trachea  e Palpation of lymph nodes  e Pulsations of common carotid artery  e Special tests  e Evidence of toxicity    Percussion over the sternum gives a resonant note in normal  cases. In retrosternal goitres, it gives a dull note.    ¢ It should be done in the upper pole because of following  reasons: Superior thyroid artery is a direct branch of external  carotid artery. It is more superficial than inferior thyroid artery.  ¢ Presence of thrill and bruit are the features of toxic goitre.    This includes CNS and eye signs, as in Graves’ disease,  examination of skeletal system to rule out metastasis as in  carcinoma of the thyroid, and examination of cardiovascular  system in cases of toxic goitre. These have been dealt with in  detail in the corresponding topics. Deep tendon reflexes also  have to be elicited—there 1s a slow relaxation phase in  hypothyroidism.   It is based on its anatomical location and features. It should  be noted that the neural tumours arising from vagus nerve can  present in the same location but it will not move with  deglutition (Key Box 19.6).    Diffuse enlargement of the thyroid gland 1s described as goitre  (it is derived from the Latin word, Guttur = the throat).    ¢ Benign adenoma (follicular adenoma)  ¢ Malignant tumours: They are further classified as follows:    A. PRIMARY  ¢ Well-differentiated carcinoma  — Papillary carcinoma, follicular carcinoma.  ¢ Poorly differentiated carcinoma  — Anaplastic carcinoma  ¢ Arising from parafollicular cells  — Medullary carcinoma  ¢ Arising from lymphatic tissue  — Non-Hodgkin’s lymphoma.   * Malignant melanoma, renal cell carcinoma, breast  carcinoma produce secondaries in the thyroid, due to  blood spread.    Multinodular goitres, malignant goitres, puberty goitres  are common causes of goitre. Bacterial thyroiditis is  rare. Riedel’s thyroiditis is very rare.    A multinodular goitre 1s the end-stage result of diffuse  hyperplastic goitre. Excessive metabolic demands cause an  excessive enlargement of the thyroid. Therefore, it is  common in women.   Metabolic demands increase during puberty. A goitre  appearing during that period is called puberty goitre. A  goitre can develop during pregnancy and is called pregnancy  goitre. Both of them are physiological but may eventually  develop into multinodular goitre (MNG).    Multinodular goitre occurs due to continuous stimulation by  TSH secreted from the anterior pituitary.    It is seen in girls at puberty or during pregnancy when the  metabolic demands are high and the production of T3, T4  are comparatively normal. Due to feedback mechanism,  TSH levels increase, which stimulate thyroid gland and  causes diffuse hypertrophy and hyperplasia.   This is also called physiological goitre and can be treated  by giving tablet thyroxine (T4) 0.2 mg/day to suppress TSH.  Goitre may disappear if treatment 1s given in the stage of  diffuse hypertrophy.    Common in hilly/mountainous or low-lying areas because  of decreased iodide content of water. This causes iodine  deficiency goitre mediated by the same feedback  mechanism.    If the iodine deficiency status continues for a long time, it  results 1n accumulation of colloid material in the gland and  causes colloid goitre.    All these three types of goitre if left untreated will change  to multinodular goitre (Fig. 19.16).    ¢ Stage I: Stage of diffuse hypertrophy and hyperplasia  of thyroid.    ¢ Stage II: Due to fluctuating levels of TSH because of  pregnancy, lactation, menstruation, etc. Some areas in  thyroid are overstimulated and are converted to active  follicles.    ¢ Stage III: The active follicle ultimately undergoes  necrosis and many such necrosed follicles join to forma  nodule. Many such nodules form a multinodular goitre.  Nodules contain necrosed tissue, i.e. inactive tissue. The  intemodular tissue is active.    such as PAS and sulfonamides, cause goitre by preventing  oxidation of 1odide to iodine. Excess iodides inhibit organic  binding of iodine and produce goitre.    Common in females. Female : male ratio is 10 : 1. Seen in  the age group of 20—40 years.    ¢ Long duration of swelling in front of the neck, dyspnoea  due to tracheomalacia and dysphagia are the presenting  features. The gland is nodular, firm in consistency and both  the lobes are enlarged. Hard areas may suggest calcification  and soft areas, necrosis.   e Sudden increase in size with pain is mainly due to  haemorrhage in a nodule.    The most common site of a nodule ts at the junction of  isthmus with one lobe.   3. In 10-20% of cases, patients can develop secondary  thyrotoxicosis with CVS involvement. Toxic multinodular  goitre is also called Plummer’s disease.    Grade | < 90/min—mild  Grade || 90 to 100/min—moderate  Grade IIl> 110/min—severe    1. Complete blood picture (CBP), routine urine examination  and fasting and postprandial blood sugar to rule out diabetes  mellitus.    2. X-ray of the neck: Anteroposterior and lateral view.  ¢ To look for compression of trachea—to check feasibility  of intubation during anaesthesia (Fig. 19.17).  ¢ To rule out retrosternal extension—soft tissue shadow  seen.    3. Flexible laryngoscopy is done to see vocal cord mobility  (this has replaced indirect laryngoscopy).    4. Ultrasonography: High frequency ultrasound ts a very  useful investigation specially in cases of solitary nodule.  Even in multinodular goitres, ultrasound guided FNAC  can be done. It can also detect clinically impalpable  lymph nodes in the neck (suggest malignancy) (Key  Box 19.7).    e Ultrasound examination Is inexpensive, easily done and has  more advantages than disadvantages. Thus, it is often the  first investigation in thyroid swellings.    5. Fine needle aspiration cytology (FNAC): It can be done  in suspected hard nodule of multinodular goitre. It is a  simple and useful investigation which can detect    e Ithas become the investigation of choice (frontline) in thyroid  diseases.    malignancy. Since the treatment of MNG is often total  thyroidectomy and an ultrasonogram can also help to rule  out malignancy, FNAC is done only in suspicious cases  of MNG.    6. CT scan is done when you suspect retrosternal extension,  doubtful resectability or large lymph nodes in the neck.  They may also have intrathoracic lymph nodes (Fig. 19.55).    Prevention is mainly important in endemic area and is done  by supplementing iodine (Key Box 19.9).    e flodine deficiency goitre: Use iodised salt, sea food, milk,  egg, etc.   1. A 22-year-old lady was referred to us for a nodule in the  right side of neck in the thyroid region that was moving with  deglutition. She also had epigastric pain since 6 months.  Clinical diagnosis of solitary thyroidnodule with hyperacidity    was made. An ultrasound examination of the neck was  requested and the report was a surprise. She was having a  parathyroid adenoma with calcific pancreatitis and  nephrocalcinosis. You know the diagnosis now—-A case of  hyperparathyroidism.    2. We sent a case of MNG to thyroid scan. The scan detected  jugular lymphadenopathy on both sides. The diagnosis  changed from MNG (benign) to malignancy (papillary  carcinoma because of lymph nodes). Lymph nodes were  not palpable on clinical examination in this patient.    3. A 21-year-old lady had a multinodular goitre with the entire  gland replaced by nodules. Case was posted for a total/near  total thyroidectomy. A senior experienced Professor asked  one question, ‘what is the duration of this swelling?’ It was  10 years. As per his advice, FNAC was done. FNAC report  was lymphocytic thyroiditis. Surgery was cancelled. She was  put on T-Eltroxin 0.1 mg, 2 tablets /day. She is on follow up  now. Gland size is reduced to 30% in the last 2 years.    ¢ Subtotal thyroidectomy: In this operation, parts of right  and left lobes and entire isthmus are removed in flush with  tracheal surface leaving behind a little tissue in the  tracheoesophageal groove to protect recurrent laryngeal  nerve and parathyroid gland (Fig. 19.18).    * Some surgeons treat these patients with 0.2 mg of thyroxine  to suppress the TSH stimulation in the postoperative  period, for a period of 2—S years.    * Total thyroidectomy is the choice today provided  complications such as recurrent laryngeal nerve, paralysis  and hypocalcaemia due to removal of parathyroid glands  can be avoided. Thus, it 1s desirable to do a total  thyroidectomy if experience of the surgeon is good and  in a high volume centre (Fig. 19.19).    ¢ Total lobectomy on one side, ]  subtotal on other side is  indicated if one entire lobe is  involved    Very often, it is a multinodular goitre that develops in the neck  and is slowly pulled down behind the sternum due to following  reasons.    I. Primary: It arises from ectopic thyroid tissue in the  mediastinum. It also derives blood supply from media-  stinum. It is rare (1%)    ¢ Substernal: The most common type where the lower  border of the gland is behind the sternum.   ¢ Intrathoracic: No thyroid is seen in the neck, diagnosed  by radio-iodine scan.   ¢. Plunging goitre: When patient is asked to cough,  intrathoracic pressure increases. As the thyroid plunges  out, the lower border of gland 1s clearly seen in the neck.    ¢ Itcan be suspected when the lower border of the swelling  is not seen.   * Most of the patients have difficulty in breathing or even  stridor.   ¢ Engorgement of neck veins and superficial veins. These  become more prominent when the hands are raised above  the head, and the arms touch the ears—Pemberton’s sign  (Fig. 19.20).   Fig. 19.20: Observe the engorged veins due to retrosternal goitre.  Such veins are also seen when mediastinal nodes are enlarged  as in papillary carcinoma thyroid    « They are similar to MNG. However, isotope scan is very  useful in the diagnosis of intrathoracic goitres.  ¢ CT scan to localise and to know the size and extent    * It can be easily explored through the neck incision and  removed.    RETROSTERNAL GOITRE es  e Very often, it is an MNG with the lower border unseen  e Rarely from ectopic thyroid tissue  e Severe breathlessness even though small  e Drugs should not be given if it is toxic  e Pressure effects diagnosed by Pemberton’s test  e Surgical excision is the treatment  e No radioiodine therapy    It is a complex disorder which occurs due to increased levels  of thyroid hormones (hyperthyroidism) and manifests  clinically with various signs and symptoms involving many  body systems. Following are the causes of thyrotoxicosis:    3. Solitary toxic nodule: Autonomous nodule which 1s not  under the influence of TSH but occurs due to hypertrophy  and hyperplasia of gland (tertiary thyrotoxicosis).  4. Other causes of thyrotoxicosis  ¢ Thyrotoxicosis factitia: False thyrotoxicosis occurs due  to overdosage of thyroxine, given for puberty goitre.   ¢ Jod-Basedow's thyrotoxicosis: Jod means iodine in the  German language, Basedow means toxic goitre. lodine-  induced thyrotoxicosis (iodine given for hyperplastic  endemic goitres).    332  ¢ Drugs: Amiodarone is an iodine containing preparation  given as antiarrhythmic drug.  Please note: In the clinicalexamination, primary thyrotoxicosis  and secondary thyrotoxicosis are commonly kept as long cases.  Hence, these two have been discussed below in detail and also  see Table 19.5.    The exact aetiological factors responsible for the disease are not  clear. Following are considered as possible aetiological factors  (Key Boxes 19.11 and 19.12):   1. Autoimmune disorder is the first possible cause due to  the demonstration of auto-antibodies in the circulation.  Example: TSH receptor antibodies. It can also be  associated with other autoimmune disorders like vitiligo.   3. Thyroid stimulating immunoglobulins (TSI) and long-  acting thyroid stimulator (LATS) are responsible for  pathological changes in the thyroid gland in Graves’  disease. They stimulate thyrocytes to grow and synthesise  excess thyroid hormones.   5. Female sex, emotions, stress, young age also have been  considered as other factors responsible for the disease.    * As aresult of continuous stimulation, acinar hypertrophy  and hyperplasia take place. The acinar cells which are  normally flat, become tall columnar. The normal colloid   1. An 18-year-old girl visited many doctors for her complaint  of loss of weight. She was investigated for tuberculosis  (common disease in India), malignancy, etc. She was given  unnecessary tonics. After nearly 6 months, when eye-signs  started developing, it was proved to be Graves’ disease.   2. A bank clerk s only complaint was that he could not sign  the cheque because of excessive sweating. Thyroid gland  was not palpable. His pulse rate was very high,  investigations revealed that it was a case of primary  thyrotoxicosis. On careful questioning, he admitted that  he was a “nervous character”.   3. A 24-year-old lady was being asked by her friends every  day why her eyes were prominent. Her only complaint was  prominent eyes. On careful questioning, she admitted  having anxiety, tension, excitability.    disappears and the cells are empty. However, rich  vascularity is seen. Thus, small follicles with hyperplastic  columnar epithelium is characteristic.    * Primary thyrotoxicosis is 8 times more common in females  than in males, especially in the age group of 15—25 years.   « Very often young women present with unexplained loss  of weight in spite of good appetite. Diarrhoea occurs due  to increased smooth muscle activity of small intestines.  Intolerance to heat, preference to cold, fine tremors,  excitability, hyperkinetic movements, excessive sweating  are the other features. Free steroid hormone levels  decrease Graves’ disease. This results in decreased  effective oestrogen at the cell level which in turn causes  oligomenorrhoea.    Fig. 19.21: She was 42-year-old with a 2-month history  of thyrotoxicosis that developed after viral infection.  (Courtesy: Dr Sreejayan, Prof. of Surgery, Calicut  Medical College, Calicut, Kerala)    Tremors of the tongue when the tongue is within the oral  cavity and tremors of the outstretched hands are  characteristics. A piece of paper may be placed on the  fingers in doubtful cases for demonstrating the tremors of  the hand. Extensor surface of the hand is used because  extensors are weak when compared to flexors.    Pulse rate is always raised and rapid indicating tachycardia.  Depending upon the pulse rate, thyrotoxicosis can be  classified as follows: mild—90-—100/minute, moderate—  100—110/minute, severe—more than 110/minute.    Palpitation and extrasystoles can also be found in primary  thyrotoxicosis even though other cardiac features such as  fibrillation and cardiac failure are rare.    Prominent eyeballs and retraction of the eyelid result in  thyrotoxic exophthalmos. This is due to retrobulbar de-  position of inflammatory cells and round cells with venous  congestion resulting in oedema (Figs 19.21 to 19.23).    Levator palpebrae superioris muscle is innervated by  oculomotor nerve which also carries sympathetic fibres  derived from cavernous plexus for the smooth muscle part  of the levator. Contraction of this muscle produces lid  spasm.    This is aided by spasm of Miiller's muscle, a sympathetic  muscle which lies adjoining the levator palpebrae superior   Fig. 19.22: He was 31-year-old with  loss of weight of 12 kg in 3 months.  There was diffuse enlargement of the  gland with exophthalmos    Fig. 19.23: She was 48-year-old lady  who had multinodular goitre of 10  years duration. She also had lid-lag  sign (rare in toxic MNG)    muscle. This is responsible for keeping the eyeball  forwards. All these factors together produce a classical  stare.   ¢ Naffziger’s method: Stand behind the patient and look  at the supraciliary arch, by tilting the patient's head  backwards. In normal cases, eyeball is not seen. In cases  of exophthalmos, eyeball is protruded outside and hence  it Is seen.    to muscle paresis as a part of thyrotoxic ophthalmoplegia.  Diplopia is due to weakness of extraocular muscles  (inferior oblique-elevators).    palpebral fissure is due to spasm of sympathetic fibres in  the levator palpebrae superioris.    the patient is asked to look upwards. This occurs due to  increase in the field of vision due to exophthalmos.    . von Graefe’s sign (Lid lag sign): When the patient is    asked to look up and down, upper eyelid cannot cope up  with the speed of movement of the finger because of the  lid spasm. Hence, the lid lags behind.    8. Kocher’s sign: When an attempt is made to lift the eyes   ¢ This occurs in untreated cases of Graves’ disease. lh EASE = opsomerve: dalnane Aperblin dite yaa  ¢ If the disease continues, infrequent blinking secondary —  to exophthalmos results in constant exposure of the Important causes of exophthalmos  cornea to the atmosphere. This results in keratitis, 1. Thyrotoxicosis  comeal ulcer, conjunctivitis, chemosis and may even lead 2. Primary CNS tumours: Meningioma, optic nerve glioma,  to blindness. This is called malignant exophthalmos. haemangioma, lymphoma, etc.  3. Metastatic tumours: Neuroblastoma, central carcinoma.  Tilo lo ee ar wn ae 4. Vascular: Cavernous sinus thrombosis—aneurysm of  KEY BOX 19.14 _— ophthalmic artery  THYROTOXIC EXOPHTHALMOS Causes of pulsating exophthalmos  e Proptosis and lid retraction result in exophthalmos 1. Cavernous sinus thrombosis  e Sclera is visible beyond limbus 2. Carotid—cavernous sinus, A-V fistula  e Naffziger’s method to examine 3. Orbital vascular tumour  e Staring look 4. Ophthalmic artery aneurysm    e e e ee. h6°8 e ee e 6° Ce e e he e e t e e Co e e e e 2    4 = a =1 0) Geb: a) é .  _— 3. Orbital decompression may be necessary in late cases  INTERESTING 6 Ps OF 4. Guanethidine eyedrops are useful to decrease lid spasm  GRAVES’ OPHTHALMOPATHY and lid retraction.  e Prominent eyes 5. Head end elevation and disease control.  e Periorbital oedema 6. Dark spectacles, 7% methylcellulose eye drops.  e Papilloedema  ¢ Proptosis V. Thyrotoxic myopathy  e Palpebral fissure widening * Mild weakness of proximal limb muscles, ocular and  e Progression to blindness frontalis muscles is not uncommon. On careful questioning,  “man fre ss ae eee patient may admit difficulty in climbing steps.  Se a BP le Se dct Se ie, Se * Weakness of extraocular muscles results in double vision  KEY BOX 19.16 _— (diplopia).  GRADING OF THYROID EYE DISEASES + Features suggestive of myaesthenia gravis, periodic  Grade O No signs or symptoms paralysis can be found.  Grade 1 Only signs, no symptoms ¢ Myopathy improves with treatment.  Grade 2 Both signs and symptoms  Grade 3 Proptosis VI. Thyrotoxic dermopathy (Key Box 19.17)  Grade 4 Extraocular muscle involvement ¢ Popularly called as pretibial myxoedema—is seen in  Grade 5 Corneal involvement thyrotoxicosis patients treated with surgery or antithyroid  Grade 6 Loss of vision with optic nerve atrophy  Rea coae- 2 A ees bea  = Symptoms of thyrotoxicosis  Symptoms of Symptoms of increased In females In children  hyperthyroidism adrenergic stimulation  Heat intolerance Palpitations Amenorrhoea Rapid growth    drugs. It is always associated with exophthalmos. It is seen  in 1—2% of patients.   ¢ Bilateral symmetrical deposition of myxomatous tissue  (glycosaminoglycans) mainly in the pretibial region, may  also affect the foot and ankle, sometimes the entire leg  below knee. Skin is dry and coarse (thickening of skin by  mucin—like deposits). Swelling is due to the obliteration  of initial lymphatics by mucin (see page 111).   ¢ Pretibial myxoedema (misnomer) is nonpitting in nature  and may be associated with clubbing of fingers and toes  called thyroid acropachy (Key Boxes 19.18 and 19.19).    Thus 4 important features are seen only in primary thyro-  toxicosis, not in secondary thyrotoxicosis (Key Box 19.20).    At the end of clinical examination, commonly asked  question 1s, how will you differentiate primary thyrotoxicosis  from secondary thyrotoxicosis (Table 19.5).    Routine isotope scanning has been abandoned in toxic  goitres except when toxicity is associated with nodularity.    « Routine investigations such as complete blood picture,  fasting and post-prandial blood sugar estimation, flexible  laryngoscopy are done.   * Serum T3, T4 and TSH are measured. T3 or T4 levels are  high and TSH levels are low. The normal level of T3 is  ].3—3.5 nmol/L and normal level of T4 1s 55—150 nmol/L.   e Sleeping pulse rate is counted after the patient is sedated  with 30 mg of phenobarbitone. In a case of toxic goitre,  the pulse rate remains high even during sleep because of  increased metabolism. This is a simple bedside    1. Age 20-40 years 35—50 years  2. Symptoms and signs Appear simultaneously, duration is short Long duration of a swelling and short duration of signs  3. Skin over thyroid Warm Not warm  4. Consistency Soft or firm Firm or hard  5. Surface Smooth Nodular  6. Auscultation Bruit is common Bruit uncommon  7. Eye signs Commonly found Rarely found (lid lag)  8. Predominant symptoms CNS CVS  9. Pretibial myxoedema Seen in I—2% patients Never seen  10. Proximal myopathy Seen in 5% patients Never seen  11. Malignant exophthalmos Can be seen Never seen    investigation in cases of toxic goitre. In anxiety states,  pulse rate may be high in the waking hours and it comes  back to normal during sleep.   * Thyroid stimulating antibodies are elevated (TSH-RAbs)  Measurement of IgG immunoglobulins (TSH-RAbs) ts not  essential to make the diagnosis of thyrotoxicosis.    Aim of treatment  I. To restore patients to euthyroid state  II. To reduce the functioning thyroid mass to a very critical  level (about 6—8 g of thyroid tissue)  III. To minimise complications   ¢ Other drugs such as potassium perchlorate are given in  the dose of 200 to 400 mg daily. Propylthiouracil 1n the  dose of 200 mg three times a day can also be given in  patients who develop neutropaenia due to carbimazole.   ¢ Propranolol inhibits peripheral conversion of T4 to T3.  This results in rapid control of tachycardia and surgery  can be scheduled in a few days (within one week). It should  be continued for one week after surgery because it does  not interfere with synthesis of hormones.   e Antithyroid drugs will not cure the disease. In selected  patients (30-40%) remission is possible with regular intake  of drugs. They may be continued for a maximum period of  2 years. If toxicity persists or recurs on stopping drugs,  surgery is recommended. However, majority of the patients  ultimately require Surgery or radioiodine.    ¢ Ifasmall dose of T3 (20 ug up to 4 times/day) or T4  (0.1 mg/day) is given along with antithyroid drugs, there  is less incidence of development of hypothyroidism and  increase in the size of goitre.    1. Subtotal thyroidectomy: In this procedure, the amount of  thyroid gland removed is more than toxic multinodular  goitre. However, being a toxic goitre, very little gland  should be left behind so as to avoid persistent toxicity in  the postoperative period. This quantity is difficult to  measure. Hence, some surgeons advocate leaving thyroid  tissue as small as the tip of the little finger, on both sides.  By convention it 1s said that a total of about 6—8 g of the  thyroid tissue should be left on both sides of the tracheo-  esophageal groove.    2. Total thyroidectomy can be offered to young patients with  a small-sized gland. It controls the toxicity very faster.  Hypothyroidism occurs but it 1s easy to treat.    3. Radioiodine therapy: This is a suitable alternative to  surgery In cases of primary thyrotoxicosis In patients above  the age of 30 (Key Box 19.21).    Good preoperative preparation of the patient, good anaesthetic  and surgical techniques, and good postoperative care will  reduce the complications of surgery.    Thus, antithyroid drugs, subtotal thyroidectomy and radio-  iodine therapy are the three different modalities available for  the treatment of primary thyrotoxicosis. The indications, merits  and demerits of each treatment are given in Fig. 19.24.    * Patients with severe cardiac damage entirely or partly due  to hyperthyroidism are middle-aged or elderly with  secondary thyrotoxicosis and the hyperthyroidism 1s not very  severe. These patients develop atrial fibrillation and cardiac  failure, if left untreated. In elderly patients, when the  Operative risk 1s unacceptable, radioiodine ts given.  Treatment with antithyroid drugs is started 48 hours later  and continued until radioiodine has had its effect (6 weeks).   * Ifthe cardiac symptoms are controlled well and anaesthesia  risk is acceptable, subtotal thyroidectomy is done.  However, the gland that is left behind should be equal to  the distal phalanx of the thumb of the patient.    ¢ Carbimazole: It blocks oxidation of  iodide to iodine and coupling reactions  thus reducing T3 and T4 levels. It is  metabolised to methimazole after  ingestion   ¢ Lugol's iodine is given to reduce  vascularity of the gland before surgery    10 mg, 6th hourly and maintenance dose of  10 mg two to three times a day    10-12 drops (minimum three times a day for  10 days before surgery)   It should be given 3-8th hourly interval  Dangerous agranulocytosis can manifest as  sore throat   Can precipitate bronchial asthma. T3 and T4  levels are not decreased   1. Patient not willing for surgery  2. Postponement of surgery —  3. Recurrence after surgery |    1. In good hands, permane  cure to the patient  | 2. Cure rate is high    _ 1. Long duration of treatment | . 1. Associated with mortality an  1-2 years | / morbidity  2. Agranulocytosis, fever, skin | 2. Side-effects of thyroidectom\  rash 3. Recurrence   2. Permanent hypothyroidism in 10-15%  patients. ws  _ 3. Exacerbation of cardiac arrhythmias.   Fig. 19.24: Indications, merits and demerits of different modalities of treatments for primary thyrotoxicosis   ¢ This is an indication to do I'+! scan or preferably IV 99mTc  scan. Hot nodule takes up isotope (also in carcinoma). Rest  of the gland does not.    1. Thyrotoxicosts in children: \nitially antithyroid drugs are  given for 10-15 years followed by surgery.  ¢ Radioiodine is absolutely contraindicated as there is a    e Today there is ‘no restriction of age and gender’. (However,  this is preferred in children only after completion of growth  and in adults only after family is complete)   e Conception must be avoided for a period of 4 months after  radioiodine therapy    due to hyperdynamic circulation in thyrotoxicosis (Described by J. Lerman and  J.H Means of Massachusetts General Hospital)    4. Recurrent thyrotoxicosis Antithyroid drugs  after surgery  . Toxic goitre in pregnancy    ¢ Propranolol controls the disease very well.  ¢ Radioiodine therapy is the treatment of choice.    3. Hyperthyroidism in pregnancy  ¢ Invariably it is Graves’ disease.  ¢ In the first trimester, surgery and radioiodine are  contraindicated. Carbimazole and propylthiouracil cross  the placenta.  ¢ Surgery, if necessary, can be done in 2nd trimester.    4, Apathetic thyrotoxicosis  ¢ Thyrotoxicosis in elderly wherein pulse rate 1s low, they  appear to be hypothyroid rather than hyperthyroid.  Thyroid gland is rarely palpable.  ¢ Lethargy and behaviour changes.    ¢ The benign tumours of the thyroid gland are not  uncommon. They present asa solitary nodule, thus causing  a worry to the clinician. Adenomas are of follicular type.   ¢ However, FNAC cannot distinguish between a follicular  adenoma and follicular carcinoma. Hence, a frozen section  has to be done.    « Thyroid is the only endocrine gland wherein malignant  tumours are easily accessible to clinical examination.   * Thyroid is the only endocrine gland wherein malignant  tumours occur in children, young age, middle age, old age,  and in both sexes.   * Thyroid is the only endocrine gland wherein malignant  tumours spread by all possible routes—local, lymphatic  and blood spread.    * Thyroid is the only endocrine gland wherein malignant  tumours are usually nonfunctional.   Malignant tumours of the thyroid are common. They are  interesting tumours, having good prognosis if diagnosed early.  Papillary and follicular carcinoma are well differentiated,  medullary carcinoma are poorly differentiated.    1. Irradiation to the neck during childhood: In olden days  radiotherapy was given for benign conditions such as  acne in teenagers or enlarged tonsils or thymus gland.  Those children had increased risk of papillary carcinoma  thyroid. These indications are obsolete now. However,  accidental radiation to the neck or radiation given to   3. Papillary cancer of thyroid occurs more often in patients  with Cowden’s syndrome, Gardner’s syndrome or Camey’s  syndrome.   4. Associated mutations: Chromosomal translocation  involving RET proto-oncogene (tyrosine kinase)  chromosome |0q!1.    Neck radiation increases risk not only for carcinoma  thyroid but also for parotid gland tumours and  hyperparathyrotdism.    * It 1s made up of colloid-filled follicles with papillary  projections. In some cases, calcific lesions are found which  are called psammoma bodies. These are diagnostic of  papillary carcinoma of thyroid. Characteristic pale, empty,  nuclei are present in a few cases which are described as    'These were the teachings or sayings of Late Prof Sharath Chandra, FRCS, Professor of Surgery, Madras Medical College, Chennai (Conveyed to  me by Late Prof. Subbu, MMC, Chennai, when for the first and last time, | sat with Prof. Subbu as a co-examiner at Govt. Medical College,  Coimbatore, 1998).    Orphan Annie eye nuclei. Thyroid gland has very rich  intrathyroidal lymphatic plexus. Papillary carcinoma can  be unifocal or multifocal (Figs 19.25 and 19.26).   * Papillary microcarcinoma: They measure | cm or less  in diameter. Distant metastasis is extremely rare. Hence a  simple hemithyroidectomy is the treatment of choice.   ¢ Follicular variant of papillary cancer: This is a mixed  lesion with a predominance of follicles over papillae. These  are treated by total thyroidectomy. It is called Lindsay  tumour.   ¢ Tall cell papillary cancer: This is an aggressive and  rapidly growing tumour. It occurs in elderly patients and  should be treated by total thyroidectomy.   * Very often, the lymph nodes in the lower deep cervical  region are involved and thyroid may or may not be palpable.   Fig. 19.26: PCT showing papillae with fibrovascular cores lined  by follicular cells with Orphan Annie nucleus (Courtesy: Dr Laxmi  Rao, Head of the Department, Pathology, KMC, Manipal)    PATHOLOGY OF PAPILLARY  CARCINOMA MICROSCOPY  ¢ Calcification  e Cystic changes/necrosis  ¢ Cuboidal pale cell with grooving  ¢ Crowded nuclei  e Cytoplasmic inclusions—intranuclear  e Cartoon character—Orphan Annie  ¢ Calcified deposits—Psammoma bodies  Observe 7 Cs  Types—Woolner classification  e Minimal/Occult/Microcarcinoma: Tumour of 1 cm or less in  size with no invasion/no lymph node metastasis  e Intrathyroidal: Confined to thyroid gland  e Extrathyroidal: Invasion of adjacent structures.   When thyroid gland is not palpable, it is called occult  (hidden). However, papillary carcinoma less than 1.5 cm in  diameter 1s also called ‘occult’ (Key Box 19.24).   « A few patients present late to the hospital with fixed  nodes in the neck, and fixed thyroid to the trachea with or  without recurrent laryngeal nerve paralysis (Figs 19.27 to  19,32).    ‘It should be noted that papillary carcinoma can be  offered as a clinical diagnosis only in the presence of  significant lymph nodes in the neck along with a thyroid  swelling.’   4. They need not be hard, are often cystic and firm in  consistency   5. At operation, they are bluish in colour because of rupture of  the papillae   6. Presence of lymph node metastasis does not affect the  prognosis   7. Mostly central neck nodes are cleared. Dissection of  posterior triangle and suprahyoid dissection is not  necessary.   There are many prognostic criteria that have been used tn cases  of well-differentiated carcinoma. Various scoring systems are  available for well-differentiated carcinomas. Some examples  are given in the next page.    Fig. 19.27: Papillary carcinoma thyroid  presenting as solitary nodule. FNAC gave  the correctdiagnosis. Total thyroidectomy  was done in this patient   Fig. 19.30: Papillary carcinoma thyroid ina  75-year-old lady—it behaves aggressively  in elderly patients. Total thyroidectomy was    AMES scoring  A: Age less than 40 years—better prognosis  M: Distant metastasis—poor prognosis  E: Extent of tumour extracapsular spread—poor prognosis  S: Size less than 4 cm, good prognosis    AGES scoring  A: Age less than 40 years better prognosis  G: Grade of the tumour—high grade—poor prognosis  E: Extracapsular spread—poor prognosis  S: Size less than 4 cm, good prognosis    If a patient undergoes hemithyroidectomy for suspicious  adenoma and histopathology reported is papillary carcinoma,    Fig. 19.28: This lady underwent total  thyroidectomy for MNG. It was reported  as PCT. After one year she presented  with a solitary lymph node   Fig. 19.29: This lady underwent  hemithyroidectomy two years back ano  Ccamewith recurrence in the opposite lobe.  Completion thyroidectomy was done   Fig. 19.32: Same patient as in Fig. 19.31—  underwent total thyroidectomy with functional  block dissection of the neck. See also the  displacement of carotids    dilemma exists as to how to proceed. Straightaway, one can  advice the patient for completion (total) thyroidectomy.  According to another school of thought, if the patient 1s in  low risk category with intrathyroidal malignancy and other  factors favouring good prognosis, then ‘wait and watch’ policy  can be undertaken.    It can be discussed under three headings. Treatment of the  primary, treatment of the secondaries in the lymph nodes and  suppression of TSH.   A. Total thyroidectomy is the treatment of choice. It means  removal of the entire thyroid gland. It has the following  advantages (Key Box 19.25 and Fig. 19.33).    After total thyroidectomy, thyroxine ts not given for a period  of 4 weeks so that thyroid remnants can be ablated with  radioiodine—till TSH are levels are above 30 MIU/L    B. Lobectomy (hemithyroidectomy) means removal of one  lobe and entire isthmus. Those who favour lobectomy have  following arguments (Fig. 19.34):   1. Total thyroidectomy has high complication rates such  as RLN paralysis, permanent hypothyroidism,  permanent hypoparathyroidism, etc.   2. Recurrence 1n opposite lobe 1s only 5% which means in  95% of the cases, removal of the opposite lobe 1s  unnecessary.   3. Even if it occurs later, since it 1s not dissected at the  time of initial surgery, the lobe can be removed easily.   4. Tumour multicentricity has little prognostic signi-  ficance. Thus tn a few selected cases, lobectomy can be  done.   * Mostly central neck nodes are cleared. If nodes are  enlarged in the anterior triangle, they are dissected and  removed en b/oc along with fat and fascia. This 1s called  functional block dissection (Berry picking means  removal of enlarged lymph nodes only. It 1s no longer  followed).    * Structures such as internal jugular vein, sternomastoid  muscle, accessory nerve are not removed because  lymph nodes are slow growing and they rarely spread/  outside the capsule of the lymph node. However, in  exceptional cases of papillary carcinoma with infiltration  to these structures, there should not be hesitation to remove  these structures.   * Lateral aberrant thyroid: Initially believed to be thyroid  tissue. It is actually metastasis into levels 3 and 4 lymph  nodes from papillary carcinoma thyroid.   ¢ Failure of suppression of TSH to a level < 0.1 mU/litre  suggest inadequate dose of thyroxine or noncompliance.    When to give tri-iodothyronine (T3) in the follow-up period  of well-differentiated thyrotd cancers?    * Patients who require regular radio-iodine for scanning and  ablation should be given T3 because it acts quickly and it  can be stopped and restarted quickly. On the other hand,  T4 has to be stopped for almost 30 days prior to scanning  and ablation rendering patients severely hypothyroid for  4 weeks. (Dose of T3 1s 40-60 ug/day. It is very costly;  not freely available).   "4 10) Ge Ls : Neg  SUMMARY OF PAPILLARY CARCINOMA THYROID    e Most common histological type of thyroid cancer (60 to 65%)   e Most often it presents as solitary nodule and with or without  lymph node metastasis   e Most commonly done procedure for papillary carcinoma  thyroid is total thyroidectomy with or without functional neck  dissection   Fig. 19.35: This patient had undergone  hemithyroidectomy in a peripheral hospital  15 years back. He did not receive any  medication in these years. He presented  with ulcerated nodules on the right side of  the neck (lymph nodal mass). He success-  fully underwent completion thyroidectomy  with functional neck dissection on the right  side. Also observe sebaceous cyst on the  right side of the chest wall (Courtesy: Dr  Annappa Kudva, Prof of Surgery, KMC,  Manipal)   Fig. 19. 36: This 24-year-old lady presented  with swelling in the submandibular region.  FNAC revealed salivary tissue. Hence,  submandibular sialoadenitis was diagnosed.  At surgery, the salivary gland was separate  from this mass which was excised. Histo-  pathology reported as papillary carcinoma  arising from thyroglossal duct cyst in ectopic  area. She is on L-thyroxine 0.3 mg/day for last  18 months. As of now she has no compli-  cations. (Courtesy: Dr Padmanabha Bhat, Prof  of Surgery, KMC, Manipal)    Fig. 19.38: Observe bluish colour lymph nodes at surgery and  specimen of lymph nodes    Total thyroidectomy with central neck dissection  (no prophylactic lateral neck dissection)    Fig. 19.37: Functional neck dissectior  is done for papillary carcinoma thyroid  Routinecentral compartment dissectior  is not required. Figure shows vagus  nerve, carotid artery and internal jugula  vein. Sternocleidomastoid is retracted  All these structures are preserved.  Hence, the name functional neck dis-  section (Courtesy: Dr Gabriel  Rodrigues, Prof of Surgery, Dr Suresh  BP, Asst Professor, Dept. of Surgery,  KMC, Manipal)   T2 : Limited to thyroid > 2 cm but < 4 cm  T3 : Limited to thyroid > 4 cm    ¢ Follicular carcinoma usually arises in a multinodular goitre,  especially in cases of endemic goitre. It should be suspected  when MNG starts growing rapidly.   e Itcan present as solitary nodule also (Figs 19.40 and 19.41).   follicular (differentiated), medullary, and anaplastic (undifferen- ¢ Medullary carcinoma 59-10%  tiated) carcinoma e Others 10%    Papillary and follicular thyroid cancer (age < 45 years):  Stage T N M   Stage T N M  IVA T4a Any N MO  IVB T4b Any N MO  IVC Any T Any N M1   Stage T N M  | T1 NO Mo  lI Te, 13 NO MO  Il T1-T3 Nia MO  IVA T4a NO MoO  T4a Nia Mo |  Ly aoe He Fig. 19.41: A 35-year-old female with solitary nodule. FNAC  T3 Nib MO revealed papillary carcinoma  T4a Nib Mo  T4a NO, Nib MO Pathology  T1-T4a Nib Mo Depending upon the property of invasion it is classified into:  IVB T4b Any N MO * Noninvasive which means minimal invasion.  IVC Any T Any N M1 * Invasive refers to angio-invasion and capsular invasion,   thyroid. The tumour cells line the blood vessels and get  dislodged into the systemic circulation producing  secondaries in the bones. Microscopically, most of the  tumours are well-encapsulated (Fig. 19.42).    It can present as a solitary nodule. The diagnosis is  considered only after an ultrasound scan reveals some  features of malignancy such as microcalcification. Peak  age group Is around 40 years (Fig. 19.43).   In case of long-standing multinodular goitres, if the goitre  is rapidly growing, hard or has restricted mobility, follicular  carcinoma can be considered.   Metastasis in the flat bones: The only clinical situation  wherein a follicular carcinoma can be considered as the  diagnosis 1s when a patient with a thyroid swelling presents  with metastasis in the bone in the form of bony swelling  or pathological fractures. Commonly, secondaries develop  in the flat bones such as skull, ribs, sternum, vertebral   Fig. 19.42: Follicular carcinomathyroid showing well differentiated  follicles invading the capsule and capsular veins. Please remember  follicular carcinoma cannot be diagnosed by FNAC but by  histopathology only (Courtesy: Prof Laxmi Rao, HOD, Pathology,  KMC, Manipal)   Fig. 19.43: Secondary deposit in the sternum in a patient who  underwent near-total thyroidectomy for follicular carcinoma 5 years  back   Fig. 19.44: Chest X-ray show-  ing osteolytic lesion in the left  clavicle    longer time. When bony swelling is obvious and thyroid is  not palpable clinically, it is called occult primary site.   ¢ Underlying bony erosion may be present (Figs 19.44 to  19.52 and clinical notes).   A patient with a diagnosis of “lipoma of the scalp” was  posted for excision in the prone position. As the chief surgeon  scrubbed and was about to paint the part, he could see the  pulsatile nature of the swelling, which he had missed in the  outpatient department. Fortunately, it was not excised. A  needle was introduced and frank blood was aspirated.  Surgery was cancelled. X-ray skull showed osteolytic lesion.  She had a small thyroid nodule.   Ultrasound scan is done (Figs 19.53 and 19.54) to  demonstrate nature of the nodule, whether solid or cystic  and to guide FNAC.   FNAC of the nodule. It should be remembered that FNAC  cannot differentiate a follicular adenoma from follicular  carcinoma. Hence, 1f FNAC reports as follicular cells,  overtreat the patient by total thyroidectomy. Some follow  with frozen section and proceed. This is not favoured by  many. If it is reported as follicular carcinoma, no further  Surgery 1s required. If it is reported as benign, patient  requires to be treated for hypothyroidism. Open biopsy  is not taken in operable cases (Key Box 19.28).   Plain X-ray of the involved bone can reveal osteolytic  lesions (Fig. 19.48 and Key Box 19.29),    * When primary is not found, bone biopsy is required to  find out the site of the primary.    © Thyroid |. Treatment of the primary  e Parotid * Situation I: When a patient has enlarged thyroid gland  ¢ Testis and scalp swelling, total thyroidectomy is the treatment of    choice. Secondaries do not take up the radioisotope ('°'1)  in the presence of primary tumour. Hence, lobectomy or  hemithyroidectomy should not be done.  ¢ Situation II; A patient undergoes subtotal thyroidectomy  for MNG and final report is follicular carcinoma thyroid.  In such cases, it is better to do completion thyroidectomy  within 7 days or after 4 weeks.  If done, within 7 days, or after 4 weeks it is relatively easy  to do. Between 7 days and 4 weeks, the inflammatory  process would have produced dense fibrosis, chances of  injuring RLN, parathyroids are high.  Situation III: A patient with solitary nodule—high  suspicion of malignancy after sonography and FNAC  reported as follicular cells, it is better to go ahead with  total thyroidectomy.   A 38-year-old lady underwent total thyroidectomy for a  solitary nodule, with FNAC revealing follicular carcinoma.   A fter3 years, she came with pain on the right side of sternum.  Careful examination revealed a tender, warm, bony swelling .  arising from sternoclavicular joint. The diagnosis was  follicular carcinoma of the thyroid. No wonder it is said   Fig. 19.48: Total thyroidectomy in progress. This is the best option  for most of Indian patients who present with fairly big lesions. The  need to take a small dose of thyroid hormone lifelong is a  disadvantage but if the report is ‘malignancy’, it is the treatment    duration with swelling in the scalp and sternum of 2 months duration. Bony from follicular carcinoma thyroid. See dilated veins  swellings were painful—it was a case of follicular carcinoma of thyroid with in the neck. No other differential diagnosis in such  multiple bony metastasis: Lady with three swellings patients    Fig. 19.52: This lady, who had undergone hemithyroidectomy for Fig. 19.53: Ultrasound revealing recurrence (nodules) in both  follicular carcinoma thyroid 3 years back, presented with lobes of the thyroid gland (Courtesy: Dr Chandrakanth Shetty,  recurrence. Total thyroidectomy could be done Prof of Radiodiagnosis, KMC, Manipal)   Fig. 19.54: Colour Doppler showing the relationship of the gland _— Fig. 19.55: CT neck and chest showing the large mass displacing  to major vessels of the neck (Courtesy: Dr Chandrakanth Shetty, — the carotid artery, internal jugular vein thrombosis and mediastinal  Professor of Radiodiagnosis, KMC, Manipal) lymph nodes. Lymph nodes were cleared after sternotomy    After total thyroidectomy, a whole body bone scan is done to  look for metastasis in the bone. A single secondary can be  treated by oral radioiodine therapy, followed if necessary by  external radiotherapy depending upon the response of the  tumour. Multiple secondaries, if present, are treated by oral  radioiodine therapy.    ¢ In the postoperative period, patients should receive  thyroxine 0.3 mg/day to suppress TSH and to supplement  thyroxine.  Prognosis: 15% mortality in 10 years.    e¢ Hiirthle cell carcinoma is a variant of follicular  carcinoma (Key Box 19.30). It is more aggressive than  follicular carcinoma.   e These tumours are defined by the presence of more than  75% of follicular cells having oncocytic features.   ¢ Even if Hiirthle cell adenoma is well encapsulated, it is  potentially malignant.   ¢ It does not take up '*'I. Hence, it is less likely to respond  to '?'J ablation.   ¢ Higher chance of spread to lymph nodes compared to  follicular thyroid carcinoma.    ¢ Total thyroidectomy is the treatment of choice. In many  cases of Hiirthle cell carcinoma, lymph nodes are enlarged.  Hence, modified radical neck dissection is done (MRND).  ¢ TSH suppression and follow-up are regularly required.    PEARLS OF WISDOM  All Hirthle cell neoplasms are almost malignant and  all adenomas are almost follicular  In all suspicious solitary nodules of follicular cell origin,  it is better to overtreat them by total thyroidectomy than  relying upon the frozen section, to avoid second surgery.    Follow-up of patients with papillary and follicular  carcinoma thyroid—differentiated thyroid cancer    ¢ Serum thyroglobulin (Tg): Thyroid is the only organ  which produces thyroglobulin. Levels greater than 1 to  2 mg/ml in patients receiving replacement thyroxine    therapy indicates presence of metastasis. Hence, assess the  serum Tg response to injected recombinant human TSH,  every year. In thyrotoxicosis and thyroiditis, thyroglobulin  levels may be increased (Key Box 19.31).   e tis produced by follicular cells of the thyroid. Hence elevated  in well-differentiated carcinoma. Not in medullary  carcinoma thyroid.   e Anti-thyroglobulin antibodies (ATA) TgAb are found in  Hashimoto’s thyroiditis and in Graves’ disease   ¢ Majority of the patients present with rapidly-growing  thyroid swelling of short duration. The surface is irregular  and consistency is hard.   * Early infiltration of the trachea results in stridor  (scabbard trachea).   ¢ Early fixity is characteristic. Thus, the resectability rate  is almost nil (Figs 19.56 and 19.57).    It is established by FNAC, other investigation being CT scan  (Fig. 19.58).   Fig. 19.57: A 40-year-old male with 3 cm hard, fixed nodule with  recurrent laryngeal nerve paralysis. FNAC—anaplastic carcinoma    Fig. 19.58: Highly pleomorphic malignant cells. (Courtesy: Pre  Laxmi Rao, HOD, Department of Pathology, KMC, Manipal)    * Due to the gross local infiltration into the vital structure  in the neck such as common carotid artery and trachee  the resectability rate is low.   ¢ However, very rarely a surgeon will get an opportunity t  excise isthmus so as to relieve compression of the trachea   ¢ [In many cases, death occurs within 6 to 8 months.   ¢ These tumours arise from parafollicular ‘C’ cells which are  derived from ultimobranchial bodies and not from thyroic  follicle.  e These tumours present in two different ways.  1. Sporadic is common, seen in about 80-90% of cases.    2. Familial variety present as a part of multiple endocrine  neoplasia (MEN).    * When it 1s associated with mucocutaneous neuromas  involving lips, tongue, eyelids, it 1s called Sipple syndrome,  with an occasional marfanoid habitus (MEN type IIb)  (Fig. 19.59).   * These tumours are not TSH-dependent and do not take up  radioactive iodine (Key Box 19.33).   e It produces hormones such as calcitonin and carcino-  embryonic antigen.   e Tumour is not TSH-dependent and hence does not take up  radioactive iodine.   e Radical neck dissection for lymph node metastasis should  be done.    e Prophylactic total thyroidectomy for infants with genetic trait  (RET gene mutations).  e Cannot predict prognosis (variable).    1. Total thyroidectomy with radical neck dissection  ¢ Before proceeding with surgery, look for an associated  phaeochromocytoma.    2. The lymph nodes are treated by radical block dissection  because they are fast-growing, when compared to papillary  carcinoma.    If there are multiple secondaries in the bone, oral '*'I has  no role because this tumour does not arise from thyroid  cells. Only palliative radiotherapy can be given.    A thyroid swelling which ts rapidly growing.  2. Thyroid swelling with lower deep cervical lymph nodes    and lymph nodes in the posterior triangle (papillary  carcinoma thyroid) involved.    4. Thyroidswelling with arapidly growing, vascular, pulsatile  swelling, commonly in the skull (follicular carcinoma).   Thyroid swelling with Berry sign positive (anaplastic  carcinoma of the thyroid)   Kocher’s test positive may be an indication of infiltration  into trachea.   Summary of the malignant tumours of the thyroid gland is  shown in Table 19.8.    Definition: Solitary nodule (SN) is a clinical term denoting  presence of a single palpable nodule in the thyroid gland (No  other part of thyroid gland is palpable) (Fig. 19.62A).   The tumour can present as rapidly-growing, large thyroid  swelling (primary lymphoma).   Sometimes, it can appear as a part of generalised lymphoma  (non-Hodgkin’s variety).   FNAC may give the diagnosis—Tru cut biopsy is ideal.  It is interesting to note that lymphomas of the thyroid  respond very well to chemotherapy and radiotherapy (Figs   Figs 19.61A and B: Lymphoma of the thyroid gland with  mediastinal lymph nodes and superior vena caval obstruction.  Watch dilated veins and also three cutaneous nodules    multiple nodules. However, very often a solitary nodule  on clinical examination, may tum out to be a multinodular  goitre at exploration. The solitary nodule has a higher  incidence of malignancy when compared to MNG    diffuse toxic goitres produce uniform enlargement of the  thyroid gland. Multinodular goitre (MNG) presents as    Figs 19.62A and B: (A) Solitary nodule. Rest of the gland is normal  (B) Solitary nodule. Rest of the gland is enlarged—mostly it is    e [tis acommon surgical problem encountered by general  surgeons. By definition, one can call it a solitary nodule  when only the nodule is clinically palpable but rest of  the gland is not palpable.   ¢ However, when a nodule 1s palpable and the opposite lobe  or any other part of the thyroid is palpable, it is a  ‘dominant’ nodule. It may probably be a case of  multinodular goitre (Fig. 19.62B).   ¢ It is the most common surgical disease of the thyroid  gland (Figs 19.63 to 19.65).   ¢ 15-30% patients of SN have nodule on the other side.   ¢ Ultrasonography followed by FNAC is the first line of  investigations.   ¢ Aim is to identify a solitary nodule which has high risk,  e.g. male, solid, solitary nodule (Key Box 19.35).    HIGH RISK SOLITARY NODULE  e Male patients with ‘solid’ nodule  e Rapid growing nodule of short duration  e Nodule which has restricted mobility  e Nodule with jugular lymphadenopathy on ultrasound  e Nodule which is hard in consistency  e Nodule with hoarseness of voice   Fig. 19.63: A 35-year-old female with Fig. 19.64: A 4  solitary nodule. FNAC revealed papillary   1. In 50% of the cases, a clinically palpable solitary nodule is  a part of multinodular goitre    About 2 to 3% of solitary nodules are associated with  hyperthyroidism. Chances of malignancy in a  hyperthyroid nodule is very low.   ¢ T3, T4 and TSH: Free thyroxine—Better indicator of  thyroid status than total T4. T3 is useful when T4 level is  border line (T3 toxicosis). TSH is needed when there is no  clinical manifestation of hyper (or) hypothyroidism.    Fig. 19.65: A 45-year-old male presented with  solitary nodule. Hormone levels were elevated. He  underwent hemithyroidectomy—solitary toxic nodule   c. STG level goes above 100 ug/L suggests pulmonary (or  skeletal metastasis)   e. Serum calcitonin—useful for screening medullary  carcinoma when a patient has history of thyroid cancer  and presents with a solitary nodule    phaeochromocytoma (MEN II syndrome) because of  association with medullary carcinoma.  Ultrasonography (USG): It is noninvasive, cheap and it  has become the investigation of choice in solitary nodule  (Key Box. 19.36).   Sonolucent cyst with projections of echogenic solid nubbins.  Rich in vascularity   Ultrasonography guided FNAC of gland and lymph node  can be done.   ¢ Hot nodule: The gland does not take up isotope but the  nodule takes it up, which is a feature of autonomous  solitary toxic nodule. Here, the normal thyroid tissue  is suppressed. Localisation of over activity in a nodule  with suppression of remainder of the gland 1s  characteristic of solitary toxic nodule. In toxic MNG,  multiple areas show overactivity.   ¢ Cold nodule is a nodule which does not take up isotope.  It should be remembered that only 10% of the cold  nodules are malignant. Keeping this in mind, surgeons  do not investigate MNG with isotope scan. The assess-  ment of malignancy 1s done clinically (Key Box 19.38).    7. T3, T4: In toxic goitre, T4 level should be assessed. T3 is  useful where T4 level is borderline (T3 toxicosis)    2. Carcinoma  3. Thyroiditis  4. Fine needle aspiration cytology (FNAC) (Key Box 19.37) 4. Thyroid cyst    e Class | : Paucicellular aspirate rich in colloid  e Class Il: Paucicellular smear rich in follicular cells.  e Class Ill: Frankly malignant (papillary and medullary   5. CT Scan: Expensive investigation involving exposure to  ionising radiation is not done routinely. Specific indications wat    It 1s largely classified into two groups: One, where FNAC  results are available and the other, where they are not available.    The final decision about treatment of solitary nodule depends  upon the clinical findings, sonography findings and FNA_  cytology results.   papillary carcinoma thyroid (PCT). It was a histological  surprise. How to proceed now?   ¢ At our hospital ultrasound neck was done again which  could not reveal any abnormality. She underwent  completion thyroidectomy (of the opposite lobe) after 4  weeks (re-exploration is done within 7 days or after 4 weeks  once tissue oedema subsides completely). Thus, the patient  underwent total thyroidectomy. HPR of opposite lobe did  not show papillary carcinoma. No lymph node dissection  was done. The patient was not given T4 for 6 weeks.   - After 6 weeks, 3/1 scan was done giving | mCi, residual  thyroid tissue was found in neck. It was ablated with 100  mCi of ‘3/1.   ¢ Even though the aim of surgery is to remove the entire  thyroid gland (total thyroidectomy), some parts of gland  might have been left behind by the surgeon, specially in  the region of ligament of Berry. Hence, a routine post-  operative radionuclide scan is advisable.   ¢ After ablation, she was put on T4 0.3 mg/day to be taken  in the morning on an empty stomach.   ¢ She came after 6 months with pain in the neck for which  ultrasound was done. It revealed suspicious residual  thyroid tissue.    ¢ Serum thyroglobulin was estimated. It was < 0.2 (almost  undetectable limits; Normal 0.2—55). So, the suspicious  lesion could have been some fibrous tissue due to surgery.  As thyroglobulin was normal, we did not investigate her  further.   ¢ Now it is 3 years since completion of thyroidectomy and  the patient has no disease but is on T4 0.3 mg/day.    ¢ Serum TSH, calcium, thyroglobulin, ultrasound, Thyro-  globulin antibody and if necessary, nuclear scan of neck.    I have heard in a few surgical meetings, the following  statement made by a surgeon, ‘I havedone total thyroidectomy,  how can residual thyroid tissue be present in the neck?’  Remember, it is always possible that some thyroid tissue is  left behind especially in the region of ligament of Berry,  tracheoesophageal groove and posteromedially.    dissection  ¢ Adenoma : Lobectomy  (hemithyroidectomy)  ¢ Toxic nodule in young : Lobectomy   ¢ Simple cyst : If it resolves after aspiration,  wait and watch. If it recurs,  do a lobectomy   ¢ If the lesion 1s cystic and the patient 1s a female, it is less  likely to be malignant and can be observed.   ¢ If the lesion 1s solid and the patient is a male, it needs  to be explored (Key Boxes 19.39, 19.40 and Fig. 19.68).   Most common surgical disease of thyroid gland is solitary  nodule  Most of the thyroid diseases begin as solitary nodule.  Most common investigation of choice for solitary nodule is  ultrasonography.  Most common cause of a solitary nodule is multinodular  goitre.  Most common cause for solid solitary nodule in a male is  malignancy.  Most commonly used investigation of choice for tissue  diagnosis is FNAC.   Male solitary solid nodule should never be left alone for  observation   Suppression therapy should never be attempted for a solitary  nodule without a definitive diagnosis.  Radio-ablation/suppression therapy should never be given  to a nodule causing compression on the trachea.    FNAC should not generally be done to a nodule, ff it is a  post-irradiated gland or there is familial thyroid cancer.    As the name suggests these are incidentally detected during  head and neck ultrasonography done for some other  problems.   In such situations, the management ts similar to that of a  solitary nodule.   Any lesion more than 1.5 cm and lesion which is solid  should be subjected to sonography-guided FNA.  Depending on the results of the FNA, treatment is  recommended.   If it is < 1.5 cm, just follow up with annual sonogram.    neck, it is not surprising to see many such cases of  incidentaloma detected by ultrasound.    Bacterial infection of thyroid is very rare because of its  rich blood supply.    Thyroiditis is broadly classified into granulomatous  autoimmune and Riedel's thyroiditis (Table 19.9).    It is also called subacute thyroiditis or de Quervain's disease  This occurs due to viral infection. It usually follows sore  throat (mumps virus has been incriminated in a few cases)  Patients present with fever, body ache and painfu  enlargement of thyroid gland. The gland is enlarged, tende1  to touch, soft to firm and a few symptoms of hyperthyrot-  dism occur initially.    Majority of the patients respond to conservative treatment in  the form of analgesics and a short course of prednisolone.  There are no permanent sequelae of this condition.    Hashimoto's thyroiditis is the main component of thyroiditis.  Autoimmune aetiology is characterised by extensive  lymphocytic infiltration resulting in destruction of thyroid  follicles with variable degree of fibrosis.   Females in perimenopausal group (40—50 years) are  commonly affected. Initially, symptoms of mild  hyperthyroidism (hashitoxicosis) may be present. Later,  extensive intrathyroidal fibrosis results in permanent  hypothyroidism.   The thyroid follicles are destroyed by significant fibrosis.  The deep eosinophilic-staining thyroid follicular cell,  Askanazy cell, is characteristic of Hashimoto’s thyroiditis.   The gland can be firm to hard and sometimes rubbery in  consistency, smooth or irregular and can involve a lobe or  the entire gland.   In many cases, thyroid antibodies are raised, suggesting  an autoimmune disorder.    Thyroxine 0.2 mg/day is given as a supplementary dose.  If there is compression on the trachea, isthmusectomy 1s  done to relieve compression.   If the goitre 1s big and causing discomfort, subtotal  thyroidectomy can also be done.    This is a very, very rare cause of a goitre which 1s supposed  to be a collagen disorder.   It can be associated with mediastinal fibrosis,  retroperitoneal fibrosis and sclerosing cholangitis.   In this condition, there is intrathyroidal fibrosis but  extrathyroidal fibrosis is more.   Involvement of trachea, oesophagus, internal jugular vein,  carotid artery, etc. result in dysphagia and dyspnoea.   As aresult of fibrosis, all the thyroid follicles are replaced  by fibrous tissue.   By the time patients present to the hospital, it is an  advanced stage and excision is very difficult.   In selected difficult cases, isthmusectomy can be tried to  relieve compression on the trachea. Three types of  thyroiditis are compared in Table 19.9.    Reactionary haemorrhage 1s more dangerous and occurs  within 6—8 hours after surgery. This is due to slipping of  ligature because of straining, coughing, hypertension,  éfc.    e It 1s a tension haematoma which develops deep to deep  fascia, compressing the larynx. Re-exploration of neck  under GA, control of bleeding points and evacuation of  haematoma should be done immediately.    ¢ Without evacuating haematoma, an attempt to intubate  the patient may result in cardiac arrest (as such patient  will be struggling).    Respiratory obstruction can be due to tension haematoma  resulting in compression of the larynx, collapse, tracheal  cartilage softening (tracheomalacia).    ¢ Endotracheal intubation and a short course of steroid  therapy 1s necessary.  Laryngeal nerve paralysis (Tables 19.10 and 19.11)    A.Unilateral recurrent laryngeal nerve (RLN) palsy  produces a whispering voice. The opposite vocal cord  compensates. There will not be problems of aspiration  or airway obstruction (Fig. 19.69A).    B.Bilateral recurrent laryngeal nerve palsy: It is also  known as bilateral abductor paralysis. Both the vocal  cords come to be in median or paramedian position, the  airway 1S inadequate causing dyspnoea and stridor but  the voice 1s good (Fig. 19.69B).   Figs 19.69A and B: Vocal cord position in unilateral and bilateral  recurrent laryngeal nerve paralysis    tei) ahs) Various positions of vocal cord in laryngeal nerve paralysis  Position Inference  1. Median Seen in phonation  2. Paramedian Bilateral  RLN palsy  3. Intermediate Bilateral, combined paralysis of RLN and  (cadaveric) superior laryngeal nerve  4. Full abduction Forceful inspiration    Treatment: Tracheostomy is required as an emergency  procedure. Following tracheostomy, the patient is  followed up on an outpatient basis for a period of 8—9  months. This period is required for any spontaneous  recovery. If no recovery occurs after this period, it  requires a permanent solution. The choice is between a  permanent tracheostomy with a speaking valve or a  surgical procedure to lateralise the cord which can be  done by endoscopic method. The former relieves stridor  and preserves good voice but has the disadvantage of a  tracheostomy hole in the neck. The latter relieves airway  obstruction but at the expense of good voice. However,  there is no tracheostomy hole in the neck.    It means to move and fix the arytenoid, vocal process  and vocal cord into an abducted position.   Endoscopic lateralisation can be done by laser cordotomy  KTP-532 nm laser is used    It is a branch of vagus-gives a ‘motor’ branch—external  laryngeal nerve. It supplies cricothyroid which is  adductor of the cord. Paralysis causes weak and husky  voice and inability to raise the pitch of voice (This 1s of  particular importance in singers).    I. Unilateral: This results in paralysis of all muscles of  larynx on one side. Vocal cord will be in cadaveric  position. The healthy cord is unable to approximate the  paralysed cord, thus causing glottic incompetence. This  results in hoarseness of voice and aspiration of liquids  through glottis. Cough is ineffective due to air wastage.    II. Bilateral: This is an uncommon condition. This results  in paralysis of all the intrinsic muscles of the larynx.  Both vocal cords assume cadaveric position. There is  also a total anaesthesia of larynx.    It is better to identify RLN in all cases of thyroidectomy—  specially total thyroidectomy and lobectomy.    4. Permanent hypothyroidism can develop slowly after  thyroid surgery especially after subtotal thyroidectomy for  Graves’ disease. It takes 2—3 years for manifestation of  hypothyroidism to become apparent.    5. Permanent hypoparathyroidism is managed with  calcium tablets or with 1, 25-dihydroxy cholecalciferol.    « Thyrotoxic storm occurs in patients with primary  thyrotoxicosis who are improperly treated or prepared  for surgery. At surgery, due to handling of the gland,  sudden release of thyroxine into the systemic circulation  results in thyrotoxic crisis (Key Box 19.41).   Hyperpyrexia—Above 105°F, severe sweating, gross  dehydration, hypovolaemic shock and tachycardia are  the diagnostic features.   It is treated by following measures: Fig. 19.71: Four centimetre lingual thyroid in the classical location    there is evidence of local erythema, tendemess and if the  patient has fever. Clinical features   ¢ Swelling in the tongue in the classical location, firm in  consistency, can be irregular. Impairment of speech,  haemorrhage, dysphagia, airway obstruction may be the  presenting features.   * Majority of patients have hypothyroidism (70%) and 10%  are cretins.    9. Stitch granuloma: May occur with/without sinus  formation and is seen after the use of nonabsorbable suture  material. Absorbable ligatures and sutures (VICRYL) can  be used throughout thyroid surgery except for skin closure  where silk is still appropriate.    LINGUALTHYROID a Thyroid scan-ultrasound of neck is done to find out whether  thyroid tissue is present or not (often it 1s absent).   ¢ Occasionally, a patient presents with a small swelling in  the middle of the tongue at the junction of anterior 2/3 and  posterior 1/3 of the tongue.    ¢ Tc (technetium) scan will detect ectopic thyroid tissue  (Fig. 19.72).    found in the region of foramen caecum on the tongue. : :  | | nis 1. Ectopic salivary gland tumour. Tongue is the commonest  * Foramen caecum represents the junction of epithelial floor site. To begin with mucosa is normal starts as a submucosal  of the mouth with proximal portion of thyroglossal duct. swelling.    ¢ Even though lingual thyroid ts rare, it can give rise to 2. Gumma: Rare nowadays. It is a midline, indurated, firm,  significant complications (Fig. 19.71). smooth swelling, painless.    3. Lymphangioma and haemangioma: These are present  since birth, more diffuse, fluctuant cystic swellings.  Haemangioma is compressible and lymphangioma is  transilluminant.    Lingual thyroid may be the only thyroid tissue present  in a patient. Hence, a thyroid scan ts done to confirm  the presence of normal thyroid tissue.    ¢ Small dose of thyroxine may decrease the size of the  swelling (similar to a puberty goitre) as a treatment of  hypothyroidism or to suppress TSH.    ¢ It occurs due to failure of median thyroid anlage to descend  normally. Hence thyroid tissue can be found anywhere  from the tongue to mediastinum (Key Box 19.42 and  Figs 19.73 and 19.74).   e Thyroglossal ectopic thyroid in the upper part of the neck   e Adjacent to aortic arch, aortopulmonary window, within upper  pericardium, etc.   Fig. 19.74: Ectopic thyroid  tissue in the submandibular  region turned out to be  papillary carcinoma thyroid    Ectopic thyroid tissue can have the diseases similar t  normal thyroid tissue, such as solitary nodule, multi  nodular goitre, malignancies, etc.   Diagnosis is by ultrasound, CT scan. FNAC to confirn  the diagnosis.   Most common surgical disease of the thyroid gland is  solitary thyroid nodule.    Most common site of thyroid nodule is at the junction of  isthmus and lobe.    Most commonly done surgery for well-differentiated  Carcinoma thyroid is total thyroidectomy.    Most common thyroid cancer following exposure to external  radiation is papillary carcinoma thyroid.    Most common noninvasive investigation in the evaluation  of solitary nodule is USG.    Most diagnostic feature of papillary carcinoma thyroid is  presence of psammoma bodies    Superior thyroid artery is a branch of:  A. Common carotid artery   B. It can be produced by peripheral conversion of T4  C. It is more quickly acting    Which one of the following is the treatment of choice  for toxic goitre in a child?    Which is the most effective drug for thyrotoxic storm?  A. Steroids    Which one of these operative steps is not done routinely  in thyroidectomy?    Few landmarks for identification of recurrent  laryngeal nerve include following except:   A. Parallel to trachea-oesophageal groove on the left side  B. Below the inferior thyroid artery on the right side   B. Paralysis of this nerve leads to loss of tension in the  vocal cord    Following are the important steps of surgery for well-  differentiated thyroid cancers of more than  2 cm in size except:    18. 21. Following is an indication for core needle biopsy o  radioiodine scan: thyroid swellings:  A. If T; is given, only one week of stopping of hormone A. Lymphoma  is enough B. Papillary carcinoma thyroid  B. Otherwise minimum 4 weeks stoppage of T4 Is C. Follicular carcinoma thyroid  required D. Medullary carcinoma thyroid  C. Alternative to thyroxine withdrawal is to administer ,  synthetic TSH over a 48 hours po92e Following are pathological features of papillar:  D. TSH administration is not at all effective carcinoma except:  A. Microcarcinoma  19. Following are true about thyroglobulin except: B. Orphan Annie-eyed nucleat  A. Normal levels are not detectable C. Tall cell cancer  B. Increasing levels indicate metastasis in papillary D. Hiirthle cell cancer  asuapeninalins nite 23. Following are true for medullary carcinoma thyroic  C. Increasing thyroglobulin prompts surgeon to get except:  ultrasound of the neck A. It arises from parafollicular cells  D. Useful in medullary carcinoma thyroid B. Cells are like carcinoid tumour  ; ; : C. Does not spread by lymphatics  20. co are true for medullary carcinoma thyroid Di Tumotisare vet Sli dependent  pn eae 24. Follicular carcinoma thyroid is diagnosed by:  A. Calcitonin is the tumour marker pang: Aintic-aved ie;  ite . Orphan Annie-eyed nuclei  B. It arises from parafollicular cells B. Askanazy cell  C. It has amyloid stroma C. Angioinvasion and capsular invasion  D. It can be treated by lobectomy D. Amyloid stroma  7 ANSWERS  1C 2D 3 ¢ 4A 5 b 6D 7D 3 ID 9D 10 €  me iwc 13D 14D 15D 16B 17 A 18 D 19D 20 D  21 A 22 D 23 C 24C    These are the endocrinal glands situated in the neck and  secrete the hormone parathormone (PTH). Their secretion  is not dependent on pituitary gland.    They are 4 in number; 2 on the right and 2 on the left. They  are pinkish in children, and yellow to brown in adults.    Superior parathyroids are derived from endoderm of 4th  branchial arch and thus, they develop along with the  thyroid gland.    Superior parathyroids are found in relation to inferior  thyroid artery in the middle of posterior aspect of thyroid  gland. They are situated in the fat above inferior thyroid  artery, close to cricothyroid artery and cricothyroid  articulation. They are constant in position (Fig. 20.1).    They are smaller (20-40 mg). They are dorsal to recurrent  laryngeal nerve (RLN).    Inferior parathyroids develop from endoderm of 3rd  branchial arch (with thymus) and are not constant in  position. They may be seen 1n the lower pole, within fascial  sheath of thyroid gland, low down in the neck (rarely in  the mediastinum), outside the fascial sheath or even within  thyroid gland. They are large (30 to 50 mg). They are  ventral to RLN (Fig. 20.2).    Inferior parathyroid gland is  usually found within 2 cm  radius of the lower pole    Inferior thyroid artery supplies both the parathyroids in about  95% of cases by a leash of vessels. Ligature of both inferior  thyroid arteries may not result in hypoparathyroidism  because there is adequate collateral circulation and few  branches of superior thyroid artery also supply 20% of upper  glands.    Principal cells (chief cells) are the cells which secrete PTH  (parathormone). Water clear cells are found in hyperplastic  and neoplastic glands (rich in glycogen).    It is a peptide containing 84 amino acids. It is secreted in  response to increase in calcium or decrease in magnesium.  Calcitonin has ‘NO ROLE’ in human beings.    DiGeorge syndrome: Congenital absence of the parathyroid  glands and thymus. Neonatal hypoparathyroidism along with  absence of thymus-dependent lymphoid system.    1. Resorption and mobilisation of calcium from the bone  (Key Box 20.1).   2. Increased reabsorption of calcium from kidney prompting  excretion of phosphate.   3. Enhances absorption of calcium from the gut.  ¢ Thus, PTH increases serum calcium level.  ¢ Normal serum calcium level 9—11 mg/dl (total).  ¢ In normal persons, the PTH is balanced by calcitonin,   e 50% of calcium is in the ionised form, which can be  measured   e 50% of calcium is bound to albumin. Hence, total calcium  levels will be low when albumin levels are low   e Sources of calcium: Milk, yoghurt and cheese, green peas,  beans, oranges. Daily requirement: 1000-1300 mg/day   e Calcium is an important ion for blood coagulation, cellular  activity, bone density and neuromuscular activity    Tetany is a condition wherein there is hyperexcitability of  peripheral nerves.    1. Hypoparathyroidism: It results from surgical removal o  parathyroids. Subtotal thyroidectomy and near-tota  thyroidectomy are the most common causes of tetany  Hypoparathyroidism may be ‘familial’ variety or neonata  variety. It may be temporary after thyroidectomy o  permanent if all the 4 parathyroids are removed or deprivec  of their blood supply. The incidence is around 1—2%.    3. Low calcium levels can occur due to dietary factors or poo!  absorption from the gut or acute pancreatitis, chronic rena.  failure, etc.    4. Osteomalacia and rickets due to deficiency of vitamin D.  5. Hypokalaemic alkalosis of pyloric stenosis.    1. Tingling and numbness of the fingers, toes, lips (circum  oral paraesthesia) and occasionally, with circumoral pallor.    3. In severe hypocalcaemia, there may be carpopedal spasm.  Metacarpophalangeal (MP) joints are flexed, inter-  phalangeal joints are extended and the thumb is adducted.  This is called thumb in palm deformity (obstetrician’s  hand). Inthe foot, extension in the ankle joints and flexion  of the toes is seen.    4. Stridor is a dangerous complication of severe tetany due  to spasm of muscles of respiration.    ¢ Tapping the facial nerve at the angle of jaw. This produces  twitching of eyelids, corner of the mouth, etc. It 1s called  Chvostek’s sign. It indicates facial nerve hyperexcitability.    ¢ Trousseau’s sign: When a blood pressure cuff applied  to the arm is inflated above the systolic pressure (200  mm of Hg), the hand and feet go into spasm  (obstetrician’s hand, carpopedal spasm).    6. Spasm of intraocular muscles results in blurring of vision.  7. Convulsions, even though rare, can occur in infants.    Diagnosis  This is established by estimating serum calcium level which  is usually < 7 mg%.    1. Oral calcium such as calcium lactate, calcium gluconate  may relieve mild symptoms.   2. In acute cases, injection calcium gluconate 10% (10 ml)  should be given s/owly intravenously over 10 minutes to  avoid cardiac arrhythmias.   3. If any precipitating cause 1s detected, it needs to be  corrected.    Hyperparathyroidism is an uncommon disease and occurs due  to an increased activity of parathyroids and manifests as  hypercalcaemia.    ¢ Primary hyperparathyroidism: It refers to hyperactivity  of parathyroids due to an adenoma or primary hyperplasia  of parathyroid glands.    ¢ Secondary hyperparathyroidism: It occurs due to  persistently low levels of calcium as in chronic renal failure  and malabsorption, which results in decreased levels of  calcium and increased levels of hyperphosphataemia.    ¢ Tertiary hyperparathyroidism: This ts seen in patients  who undergo dialysis and transplantation for chronic renal  failure. After a few years, autonomy develops and the  secondary hyperparathyroidism changes into tertiary  hyperparathyroidism. They can also have problems similar  to primary hyperparathyroidism.    1. Familial/genetic: Can be a part of MEN syndrome - I and  MEN IA or familial PHPT with jaw tumour syndrome.  Both occur due to genetic mutation.    Primary HPT ts the earliest and most common  manifestation of MEN | and develops in 80 to 100% of  these patients by 40 years of age.    PATHOLOGY  e Adenoma—reddish brown, only 1 gland is enlarged  e Chief cell hyperplasia—reddish brown, > 1 gland is enlarged  e Water cell hyperplasia—chocolate brown, > 1 gland is  enlarged.  e Multiple gland enlargement is a feature of familial disease.    3. Lithium 1s associated with parathyroid hyperplasia and  primary HPT. Interestingly there is no hypercalciuria and  bone mineral density is not affected.   ¢ Renal leak of calcium and declining renal function with  age may also be causative factors for primary HPT.    ¢ Single chief cell adenoma is the most common cause (80%).  It can be due to diffuse hyperplasia involving all 4 glands  (5 to 10% of cases).   e¢ Very rarely, it can be due to carcinoma arising in the  parathyroid glands (1%).   e Adenoma can be a part of multiple endocrine neoplasia  (MEN) syndrome.   e Age: 30-60 years, the most common age group Is 5th  decade—middle aged   ¢ The most common presentation is asymptomatic  hypercalcaemia 1n about 50% of the patients and renal  stones in 25% of the patients. The clinical features are as  follows:   ¢ Due to increasing levels of PTH, extensive skeletal  decalcification occurs. This results in bony pains,  pathological fractures due to brittle bones, subperiosteal  erosions, cysts in the phalanges, mandible, skull, etc. They  are called pseudotumours. The changes are similar to that  seen in osteitis fibrosa cystica (von Recklinghausen’s  disease). Thus, osteopaenia, osteoporosis and osteitis fibrosa  cystica are found in 15% of PHPT.   ¢ Increased calcium levels result from increased calcium  absorption from the kidneys. Hence, patients are prone to  develop renal stone and nephrocalcinosis (calcification  of kidney), hypertension.   e Calcium also increases the tone of the vessels which adds  to the hypertension. Primary hyperparathyroidism 1s the cause  of stones in 1-3% of all patients with kidney stones and in  10% of those who have recurrence of stones.   ¢ Increased incidence of gallstones has been reported as a When a patient develops multiple urinary calculi,  consequence of increase in secretion of calcium tn the bile- reduced bone density, high serum calcium, symptomatic  calcium bilirubinate stone. hypercalciuria and pathological fractures, suspect   * Hypercalcaemia can result in depression, fatigue, anxiety,  psychosis and even coma. mee |   - These patients, more often women, mostly middle age,  MESRM=T2321  having bony pains, backaches and behavioural abnormalities CAUSES OF HYPERCALCAEMIA  are thought to have a psychiatric illness. They are referred    2. Albumin is the main calcium binding protein in the  plasma. Hence, it should also be measured.   3. Serum PTH level which is estimated by immunoassay 1s  the diagnostic investigation. It is called tumour marker  for hyperparathyroidism. Estimation of PTH 1s difficult,  costly and needs sophisticated setup.   6. X-ray of the hand may reveal decalcification cysts in the  phalanges, telescoping of finger tips, etc. X-ray of the  skull may reveal subperiosteal erosions, hazy outline of  the skull and pepper pot appearance (Key Box 20.6).   Seen in radial aspect of middle  phalanges of 2nd and 3rd  fingers, bone cysts and tufting of  the distal phalanges    1. High frequency ultrasound of the neck can be very  accurate in the hands of an experienced sonologist. It can  also detect renal disease, pancreatic disease, etc. It cannot  scan behind sternum and cannot pick up lesions less than  0.5 cm. Specially useful in detecting intrathyroidal para-  thyroid adenoma (sensitivity is about 75%).  2. Thallium-technetium isotope scan: First the thyroid is  outlined with °?™Tc and then isotope 7°! TICI (thallium  chloride) is administered. This is taken up by both the  thyroid and parathyroid. By computer subtraction of the  two and enlargement of images, the parathyroid appears  as a hot spot.  3. Technetium-99m 7?" Tc)—labelled sestamibi (MIBI)  scanning: MIBI (methyl-isobutyl-isonitrile radionuclide—  sestamibi is concentrated in tissues rich 1n mitochondria  ¢ Heart  ¢ Salivary glands  ¢ Thyroid glands  ¢ Parathyroid glands  This test has proved to be superior to thallium and  technetium subtraction scanning.  ¢ Sestamibi is a protein labelled with technetium 99m that  localises diseased gland—most widely used and is an  accurate modality.   ¢ It is very sensitive to identify adenomas (90%) than  hyperplasia.    Fig. 20.6: Severe tetany following  removal of the parathyroid adenoma  requiring calcium infusion    (Courtesy: Prof L Ramachandra, Dr S Prasad, Dr Chethan, Department of Surgery, KMC, Manipal)    366  4. SPECT: Single Photon Emission Computed Tomography  when used with sestamibi is useful in evaluation of ectopic  parathyroid adenomas—deep in the neck or mediastinum.    CT scan, PET scan, MRI are not indicated prior to first  time surgery.   . Decreased creatinine clearance by 30%  . Reduced cortical bone density   4. Methylene blue is not recommended because of the risk  of toxic encephalopathy.    B. Surgery: The surgery of the parathyroid glands needs  patience, skill and expertise. The neck is explored with a  collar neck incision (3-4 cm) caudal to cricoid cartilage.  Parathyroid gland, when it is enlarged can be dark-brown  or chocolate brown colour. Occasionally, the surgeon is  lucky to encounter a single adenoma usually located on  the posterior surface of the thyroid gland, when it arises  from superior parathyroid (Key Box 20.7). Very often,  identification of the parathyroid may be difficult because  they may be intrathyroidal or within the mediastinum (Key  Box 20.8).   ¢ Frozen section of parathyroid glands is essential to confirm  whether it is an adenoma or hyperplasia because treatment  depends upon the pathology of the gland (Key Box 20.9).    |. Single adenoma: Excision of the gland. However, one  other normal parathyroid gland is also removed for  histopathological study.    Single adenoma is more common cause of PHPT in  about 80% patients. Multiple adenomas are more  common in older patients.    2. Diffuse hyperplasia: 3'4 or 3% parathyroids are removed  and small pieces are autotransplanted into the forearm  muscle tissue. In case there is hyperactivity of this   e Ligate middle thyroid vein. Then, retract thyroid gland  medially and anteriorly.    kev eq 208 <  WHEN YOU CANNOT IDENTIFY AT SURGERY  e Divide thyrothymic ligament.  e Search in tracheoesophageal groove.   e Serum calcium levels greater than 14 mg/dl  e Significantly elevated PTH levels    parathyroid tissue, surgical exploration becomes easy. At  the same time, if this functions normally, patient will not  develop hypoparathyroidism (13-14 pieces of 1 mm each  in brachioradialis)   3. Carcinoma: All four glands should be removed along with  ipsilateral thyroid lobe with modified radical neck  dissection in the presence of lymph node metastasis (Key  Box 20.9).    e Estimation of calcium should be done in the postoperative  period to assess the functioning of the parathyroid tissue.  Very often, after surgery for adenoma, there is a sudden  drop in the levels of calcium because of absorption of the  calcium by the bones. This is described as ‘hungry bone  syndrome’. This is seen in patients who have  generalised bone disease. In one of our patients, calcium  levels dropped down to 4 mg with severe tetany. It took  7—10 days for it to return to the normal levels. She required  24 h constant infusion of calcium.    ¢ Absorption of calcium can be enhanced by oral  administration of 1, 25-dihydroxycholecalciferol, which  is the most active metabolite of vitamin D. Vitamin D  stimulates the absorption of calcium and phosphate.    ¢ This is possible today because of localisation by  ultrasound/sestamibi combined with SPECT    ¢ Can be done under general anaesthesia or regional—  cervical block by using xylocaine (1%) with adrenaline    3. Totally endoscopic parathyroidectomy by lateral  approach by Henry, et al. It is the choice for adenoma deeper  to thyroid gland. Not for bilateral cases. 10 mm endoscope  is required. Require CO, insufflation.    e Most widely used and accurate investigation for localising  parathyroid gland is Sestamibi scan.    e Most commonly done surgery for PHPT is excision of  adenoma.   A 20-year-old boy attended dental outpatient department  fora loose tooth. He was diagnosed to have a loose tooth  and a cyst in the lower jaw as confirmed by X-ray.  Patient also had genu valgus deformity with backache  (Fig. 20.5). An ortho opinion was sought for.  Investigations revealed high calcium levels. An  ultrasound neck revealed a parathyroid adenoma of 6  cm. At exploration a single adenoma was found arising  from right inferior parathyroid and was excised.  Incidentally, X-ray abdomen also revealed bilateral  nephrocalcinosis. This case illustration has been given  to highlight the various presentations of hyper-  parathyroidism. High degree of suspicion is necessary  for a clinician to consider the possibility of hyper-  parathyroidism.    * It presents with severe abdominal pain and effortless  vomiting. Dehydration, oliguria, renal failure follow  soon.    * Sudden increase in PTH levels occur due to spontaneous  bleeding in a parathyroid tumour or rupture of a cystic  parathyroid tumour. Severe dehydration also precipitates  a CTISIS.    2. Disseminated carcinoma with bony metastasis (usually  carcinoma of the breast).    2. The bisphosphonates—disodium pamidronate slow  15—60 mg single IV infusion or over 2-4 days. Maximum  dose 1s about 90 mg. This drug stops mobilisation of  calcium from the bone   e Mithramycin rapidly controlled the hypercalcemia of  malignant disease in every patient. This control was  temporary, and intermittent administration of the antibiotic  was required. Hypercalcaemia frequently responds to  hydration and moderate doses of corticoids.   ¢ Gallium nitrate therapy is highly effective and superior to  maximally approved doses of calcitonin for acute control  of cancer-related hypercalcaemia.   Fig. 20.10: Ultrasonography of the neck reveals heterogeneous Fig. 20.11: Ultrasonography of the abdomen shows gall stone  nodule predominantly echogenic with posterior acoustic shadow   Fig. 20.12: Sestamibi scan shows bright spot—parathyroid Fig. 20.13: Upper pole of thyroid gland is mobilised and  adenoma. He also had hypercalcaemia (Key Box 20.10) 2 cm superior parathyroid adenoma is removed    Weight of a normal gland is 4 g.  Yellowish in colour  Surrounded by perinephric pad of fat.    Middle suprarenal arteries arise from abdominal aorta.  Inferior suprarenal arteries arise from renal artery.    Suprarenal arteries branch before entering the gland so  that 50 to 60 arteries penetrate the capsule.    Venous drainage is through a single large suprarenal  vein. On the right side, it is short and drains into 1VC. On  the left side, it is longer and joined by inferior phrenic  vein and empties into left renal vein.    One has to clearly identify and ligate suprarenal vein  especially on the right side because of its short course.  Avulsion of right suprarenal vein can be catastrophic.   Hormone  Cells of zona glomerulosa ¢ Aldosterone  ¢ Cortisol  reticularis (corticosteroids)  ¢ Adrenaline  (epinephrine)  ¢ Noradrenaline  (norepinephrine)  ¢ Dopamine   It is a mass of nervous tissue associated with sympathetic  system.    Triad of PHA is hypertension, hypokalaemia and hyper-  secretion of aldosterone (Key Box 20.11).   PHA can be due to a single adrenocortical adenoma  wherein it is called Conn’s syndrome.   It can also be due to bilateral micronodular hyperplasia  Clinical features are headache, hypertension, hypokalaemia  and weakness, commonly in women.   The diagnosis 1s established by measuring potassium level  and aldosterone to plasma renin activity ratio.   CT scan is the most important test to locate the lesion  (usually it is 1-2 cm).    ALDOSTERONE  Angiotensin |! increases aldosterone secretion.  Hyperkalaemia is another potent stimulator of  aldosterone.  It functions mainly to increase sodium reabsorption and  potassium and hydrogen ion excretion.  It acts at distal convoluted tubules.    ¢ Refers to various clinical manifestations resulting from  increased secretion of corticosteroids.   ¢ When it is due to pituitary adenoma secreting large amount  of ACTH—t is called Cushing’s disease.   ¢ Ectopic ACTH producing tumours causing Cushing’s  syndrome are:  Small cell lung cancers  Foregut carcinoid  Medullary carcinoma thyroid  Neuroendocrine pancreatic tumour.   They are mainly due to increased corticosteroids, aldosterone  and androgens. The net result is typical Cushingoid facies —  with facial plethora, buffalo hump and moon face.   CLINICAL FEATURES  e Central obesity  e Unusual site obesity: Supraclavicular space, posterior neck  e Secondary osteoporosis: Buffalo humps  e Hypertension, Hyperglycaemia, Hypokalaemia, Hirsutism.  e Irregularity in menstruation  e Neurological: Depression, mania  e Gain in weight  e Skin changes: Abdominal striae, ecchymosis, acne,  plethora due to thinning of subcutaneous tissues   2. Serum ACTH levels: If increased, itis from pituitary source  or ectopic ACTH.   3. CT chest and abdomen to assess not only adrenals but also  to detect ectopic sites.   1. Medical: Ketoconazole or metyrapone can reduce steroid  synthesis. Thus, symptoms can be controlled. They are also  indicated if surgery is not possible.    3. Unilateral adenoma: Adrenalectomy  4. Bilateral tumours: Bilateral adrenalectomy  Postoperatively cortisol should be given.    Stereotactic radiosurgery, which uses CT guidance to  deliver high doses of radiotherapy to the tumour (photon  or gamma knife) is being increasingly used to treat  pituitary adenomas.   ¢ Age: Bimodal with a first peak in childhood and second  between fourth and fifth decades.   ¢ Diagnosis of malignancy is (> 6 cm) by size of the tumour,  presence of necrosis or haemorrhage, capsular or vascular  Invasion.   ¢ Open radical tumour resection (R,)—en bloc removal of  tumour lymph nodes and involved organs is the treatment  of choice.    Definition: Loss of function of adrenal cortex— 1s also called  Addison’s disease.  e Diseases associated with Addison’s disease include    2. Tuberculosis  3. Metastases  4. Haemorrhage  5. Amyloidosis, Wilson’s disease  6. HIV infection  Types  1. Acute adrenal insufficiency  * Shock  * Fever    ¢ Abdominal pain, vomiting confused for acute abdomen  Waterhouse-Friderichson syndrome. It is a bilateral  adrenal infarction associated with meningococcal sepsis.   * ACTH tests: No rise in cortisol levels following  administration of ACTH.   ¢ Chronic cases: Oral hydrocortisone (10 mg/m? body  surface area) and fludrocortisone (0.1 mg)   1. Ganglioneuroma: It 1s a benign neuronal tumour,  commonly arising from retroperitoneal lumbar sympathetic  trunk. FNAC, ultrasound followed by surgical excision is  the management.   ¢ Since it originates from the neural crest, it may be found  from orbit to pelvis where sympathetic nervous tissue 1s  found.   e It is the most common solid tumour of infancy and  childhood.   ¢ As the name suggests, the tumour occurs due to malignant  proliferation of the neuroblasts or failure of maturation  of primitive sympathetic nerve cells, the neuroblasts (Key  Box 20.13).   ¢ Characterised by presence of immature cells derived from  neuroectoderm of sympathetic nervous system.   NEUROBLASTOMA  e Most common solid tumour in infancy and childhood.  e Adrenal gland is the most common site.  e Mass abdomen and metastasis are common presenting  features.  e Surgical excision is the best treatment.  e Younger the child, better the prognosis.  ¢ Highest incidence of spontaneous remission.   ¢ Microscopically—uniform round cells containing  hyperchromatic speckled nucleus, Homer-Wright  rosettes with central fibrillar core.    * 50% of children present to the hospital under the age of  one year and 80-90% are less than 3 years of age.   ¢ An abdominal mass is the most common presenting  feature. The mass has all the features of a renal mass but  location 1s slightly higher. It is firm to hard, nodular and  fixed.   * The child is sick with weight loss, fever, abdominal  distension, anaemia, etc.   * Functional tumours produce diarrhoea and hypokalaemia  due to release of vasoactive intestinal polypeptide (VIP),  sweating and flushing due to release of catecholamines.   * Proptosis and periorbital swellings are due to bony  metastasis and subcutaneous nodules are quite common  (Key Box 20.15).    * Posterior mediastinal neuroblastomas can produce cord  compression and even paraplegia due to protrusion within  the spinal canal (dumbbell tumours).    * Vanillyl mandelic acid (VMA) and homovanillic acid  (HVA) are the byproducts of catecholamines passed in the  urine. 24-hour urinary excretion of catecholamines and  these two metabolites will be very high.    * Plain X-ray abdomen shows fine stippled calcification.  X-ray chest 1s done to rule out cannon-ball secondaries.    * Abdominal ultrasound and computed tomography (CT)  can define the mass, nature and extent of the tumour and  can detect hepatic metastasis.    EE a  TYPES OF NEUROBLASTOMA  1. Pepper type: Right-sided tumours with secondaries in the  liver.    e Magnetic resonance imaging (MRI) is better than CT  scan in detecting the mass as well as bony metastasis. CT/  MRI guided core biopsy can be done.    ¢ Bone marrow aspiration is positive in around 60-70%  of cases.    e¢ Intermediate and high risk cases can be managed by  chemotherapy followed by surgery.   ¢ However, many children present with metastasis and,  chemotherapy and radiotherapy are given first to control  the disease followed by surgical excision. Autologous  bone marrow transplantation has improved the outlook  for patients with advanced disease.    Definition: These are catecholamine producing tumours  arising from adrenal medulla and sympathetic ganglia which  are derived from chromaffin cells (Key Box 20.16).   e As the name suggests, phaeo (dusty) chromo (chromaffin  cells)—phaeochromocytoma is a neoplasm arising from  chromaffin cells.   e When phaeochromocytoma occurs in extra-adrenal sites,  it is called paraganglioma.   « Extra-adrenal sites include organ of ZuckerkandI (the most  common site), urinary bladder, renal hilum, chest, neck,  etc. These are the sites of paraganglionic system.   * Jn about 5% of cases, the tumour can be a component of  multiple endocrine neoplasia (MEN) Type Ia or Type IIb.   ¢ Other syndrome associated with Phaeochromocytoma is  von Hippel-Lindau syndrome (Key Box 20.17).   e The clinical manifestations are due to release of adrenaline  and noradrenaline. When the level of noradrenaline is high,  symptoms are severe.    ¢ Itisasoft, highly vascular tumourconsisting of large sympa-  thetic ganglionic cells. Most of the cells are differentiated.    PHAEOCHROMOCYTOMA  10% Bilateral  10% Malignant :  10% Extra-adrenal  10% Multiple been challenged  10% Familial  10% Children   Neurofibromatosis (NF) type |  Acoustic neuroma  Meningioma, glioma, haemangioblastoma (von Hippel-  Lindau (VHL) syndrome  Astrocytoma  MEN type Il—hereditary phaeochromocytoma  Familial paraganglioma syndrome along with carotid body  tumours    Microscopic features are polygonal or spheroida)  chromaffin cells within a vascularised fibrous stroma.   Presence of high number of K1-67 positive cells, vascular  invasion or breached capsule and PASS—phaeochromo-  cytoma of the adrenal gland scale score suggests metastasis.   The paroxysmal attack may last for a few minutes to a few   — Classical triad consisting of headache, diaphoresis and  palpitations is seen in only a few patients.    Urinary levels of free catecholamines, vanilly! mandelic  acid (VMA) in excess of 7 mg/24 hours and meta-adrena-  line 1-3 mg/24 hours are suggestive of phaeochromocytoma.   ¢ It can pick up lesions of less than | cm in size.   ¢ Iodine labelled MIBG (radionuclide) scan is found to be  very specific for phaeochromocytoma. This radionuclide  scan locates only abnormal adrenal tissue and is more  useful in detecting ectopic sites of phaeochromocytoma  (Figs 20.14).    Surgical removal is the treatment'. However, a good pre-  operative preparation 1s essential before doing surgery. It  includes control of blood pressure and tachycardia, by @  and B-blockers (Key Box 20.19).   Contraction of vascular bed which occurs due to  catecholamines results in hypovolaemia which must be  corrected with IV fluids.   First, alpha-adrenergic blockers such as phenoxy-  benzamine Is given to control hypertension and to permit  re-expansion of intravascular volume. Only after the  complete alpha-adrenergic blockade, beta-adrenergic  blockade may be added.    Phenoxybenzamine: 20-60 mg/day for 3-4 weeks before  surgery to control hypertension—increased to 10 mg/day—  total of 100-160 mg.   Propranolol: 20-60 mg/day for 5—7 days before surgery to  control tachycardia and arrhythmias.   . CVP and intra-arterial monitoring is a must, as they may    Sodium nitroprusside must be kept ready to treat  hypertension during surgery as it 1s a direct arterial  vasodilator (0.5 to 10 »g/kg/minute).   Thorough search in the abdomen for other sites, due to  the possibility of multiple tumours.    'Surgery for phaeochromocytoma is like going to a war. Without proper preparation, going to war is a sure failure.    ¢ Gentle handling ¢ Indication to do adrenal gland biopsy is to rule out/diagnose  e Haemodynamic monitoring—CVP, arterial monitoring metastasis  e Avoid rupture of the tumour to prevent recurrence ¢ Absolute contraindication to do adrenal biopsy in  e Laparoscopic adrenalectomy is very popular now phaeochromocytoma  e¢ Any nonfunctioning adrenal incidentaloma should be  resected    ¢ Postoperative hypotension can be a serious problem  which needs to be treated with large volume of plasma  expanders, blood transfusions, corticosteroids and  vasopressors.    Debulking should be done even if metastasis is present, so  as to decrease tumour burden and to control catecholamine  excess.    ¢ Detected by ultrasound or CT scan (1 to 5% of patients   have migraine. Preoperative blood pressure showed mild  elevations which were thought to be due to anxiety. During  tonsillectomy, there was a rise in BP, which was controlled  well. However, in the postoperative period, there was severe  tachycardia, hypertension, arrhythmias and hypovolaemia.  Within 8 hours of surgery, the patient died even as the  diagnosis of phaeochromocytoma was being considered.    New key boxes and pearls of wisdom have been added.  Disorders of adrenal cortex including Conn’s syndrome  and Cushing’s syndrome have been added.    . Superior parathyroids derive blood supply from:  A. Common carotid artery    . Superior parathyroids are found:  A. Above inferior thyroid artery  B. Below inferior thyroid artery  C. Above superior thyroid artery  D. Below superior thyroid artery    . The best investigation of choice to localise parathyroid  gland is:    . Which one of the following is not the site of inferior  parathyroid gland:   B. It is not the common cause of hyperparathyroidism  C. Total parathyroidectomy is the treatment of choice  D. Thymectomy need not be done    Which one of the following is the treatment of choice  for acute hypercalcaemic crisis?    Which is the first drug to used in the preoperative  preparation of a patient with phaeochromocytoma?  A. Steroids    Which one of these operative steps is done first in  excision of phaeochromocytoma?   C. It 1s dangerous to infuse large volumes of fluid after  ligation of adrenal vein    The majority of the sporadic primary hyperpara-  thyroidism is due to:  A. Hyperplasia  C. Carcinoma    18. Superior parathyroid gland can be found by:  A. Ligating the middle thyroid vein and dividing the lobe  B. Ligating inferior thyroid artery and mobilising the  gland  C. Ligating superior thyroid pedicle and mobilising the  gland anteriorly  D. Ligating inferior thyroid veins and diving isthmus    e At Sth or 6th week of intrauterine life, 2 ventral bands of  thickened ectoderm called mammary ridge/milk line or line  of Schultz appear. The line extends from axilla to the groin.  Thus, accessory nipples can appear along the milk line trom  axilla to groin (Fig. 21.1).   ¢ The nipple is everted at the site of original position.   e Oestrogens cause enlargement of mammary glands at  puberty and progesterone stimulates development of  secondary alveoli.   can be unilateral or bilateral. Fig. 21.1: Milk line with possible positions of accessory nipples    II. Poland’s syndrome  ¢ Amazia  ¢ Absence of sternal portion of pectoralis major  ¢ Occurs commonly in males  III. Supernumerary nipples: Accessory nipples, polythelia  (Fig. 21.2)  IV. Athelia: Absence of nipple  V. Polymastia: Accessory breasts occur along the milk  line—axilla (most common), groin, thigh or buttock  (Figs 21.3 and 21.4).  VI. Micromastia: Due to congenital defects of ovary, lack of  hormonal stimulation occurs which results in small breast.    Breast, a modified sweat gland occupies the pectoral region  from the 2nd to the 6th rib vertically, and from the lateral  border of sternum to the midaxillary line, horizontally. It 1s  hemispherical, and lies in the superficial fascial planes.   It is composed of fatty tissue and does the function of  secreting milk. The axillary tail of Spence is the part of  the breast which is in the axilla and is deeper to the  deep fascia, whereas the entire breast 1s a subcutaneous  Structure.    Nipple and areola complex: The nipple is located in the 4th  intercostal space, in the midclavicular line. It 1s the erectile  structure of the breast, and is directed forwards and laterally  for the convenience of feeding the child. Areola has  modified sweat glands and sebaceous glands. These enlarge  during pregnancy and are called glands of Montgomery.  Both nipple and areola are pigmented due to melanin  deposition which increases during pregnancy. Hair is absent  in the areola of women (present in males).    lie ligaments of Cooper which are cone-shaped fibrous  bands. Their apex 1s attached to overlying skin, and base to  the fascia over pectoralis major. Puckering of the skin is  due to infiltration of the ligaments of Cooper.    . Lobule is the chief functional and structural unit of breast.    Many lobules join to form a lobe. There are 15—20 lobes  and each lobe is drained by a lactiferous duct. They are  15—20 in number arranged radially, lined by myoepithelial  cells, which converge into the nipple. Diameter of a  lactiferous duct 1s 2-4 mm.    Lymphatic drainage  They can be divided into lymph nodes and lymphatics  (Fig. 2th),    Anterior or pectoral: They are under the pectoralis major  which forms anterior fold of axilla    . Central group: These lymph nodes are present in the centre   ¢ Anterior and central group of nodes are commonly  involved in carcinoma breast.    very high in the axilla. They are difficult to feel clinically.    5. Posterior: They are also called subscapular group of lymph  nodes. They are felt along the posterior fold of the axilla.  These five groups together form the axillary group of lymph  nodes.   6. Internal mammary lymph nodes: Also called parasternal  nodes. They lie along internal mammary vessels. They are  located in the 2nd, 3rd and 4th space.   7. Supraclavicular lymph nodes: Spread to supraclavicular  lymph nodes indicate advanced stage of the disease. It  indicates poor prognosis.    1. Superficial lymphatics: Drain skin over the breast except  nipple and areola. Superficial lymphatics of one breast  communicate with the contralateral breast across midline.   2. Deep lymphatics: Drain parenchyma of the breast. They  also drain nipple and areola.    ¢ The first lymph nodes draining the tumour bearing area is  called sentinel node.   ¢ 75% of the lymph from the breast drains into axillary nodes.   ¢ Most of the lymphatics eventually drains into central to  apical and then to supraclavicular lymph nodes.   e Internal mammary nodes receive lymphatics not only from  inner quadrant but also from outer quadrant.   ¢ Lymphatics from inner quadrant of the breast penetrate  rectus sheath and thus spread into coelomic cavity. It results  in ascites, rectovesical deposits and Krukenberg tumours.   ¢ Krukenberg tumours are bilateral bulky ovarian metastasis  in premenopausal women. During ovulation, raw surface  develops over the ovary into which malignant cells drop  and develop into large tumours (transcoelomic spread).    1. Lateral thoracic artery gives many branches which  penetrate through the pectoralis major and supply the breast.   3. Pectoral branches of thoraco-acromial artery supply  upper part of the breast.   4. Lateral branches of posterior intercostal arteries  Veins: Breast is drained by perforating branches of internal    *These swellings may give rise to fluctuation in a ‘small’ part of  the swelling. Truly, they are not cystic swellings.   on  mammary veins, tributaries of axillary veins and perforating  branches of posterior intercostal veins.  Venous return follows the arteries but drain into large  veins that also receive blood from vertebrae and thoracic  cage. E.g. posterior intercostal veins joining paravertebral  plexus of veins (Batson’s venous plexus). This explains  the occurrence of metastasis in the vertebrae and pelvic  bones from carcinoma of the breast.   e Prolactin: Lactogenesis in late pregnancy and postpartum  period. It also upregulates hormone receptors and stimulates  epithelial development.    1. Inflammatory: Acute bacterial mastitis with abscess*  2. Neoplastic:  a. Benign: Phylloides tumour*  b. Malignant: Intracystic carcinoma*  3. Non-neoplastic cyst  a. Fibroadenosis—cyclical mastalgia  b. Simple cysts of the breast  c. Cyst of Bloodgood—blue domed cyst  4. Retention cyst of the breast: Galactocoele  §. Other rare causes of cysts of the breast  a. Tuberculous mastitis with cold abscess*  b. Lymphatic cyst of the breast (congenital)  c. Hydatid cyst of the breast  d. Haematoma of the breast    It is most commonly encountered during lactation. Hence,  it is called lactational mastitis.   Fig. 21.7: Large breast abscess presentedlate Fig. 21.8: Extensive necrosis of skin due to severe  to the hospital. Managed by incision and mastitis (Courtesy: Dr C.G. Narasimhan, Surgeon,  drainage (I and D), not aspiration   Staphylococcus produces many enzymes/toxins such as  catalase, coagulase, hyaluronidase which result in an  abscess. It also inhibits phagocytosis because of type ‘A’  protein on its surface. Staphylococcus aureus, which enters  through the nipple, proliferates intraductally and produces  clotting of the milk. Within the clot the organisms multiply,  which results in a cellulitic stage of the breast (mastitis)  and in untreated cases, it may give rise to a breast abscess.  Initially, only one lobule and duct get affected. Later other  lobules get infected, giving rise to an intramammary  abscess.    It occurs in patients with duct ectasia and periductal mastitis.  When such an abscess ruptures, it results in a mammary  duct fistula. It classically drains at the junction between  the areola and breast skin. Anaerobic bacteria are the cause  in majority of cases.    Methicillin resistant Staphylococcus aureus (MRSA) or  community acquired MRSA or CA-MRSA can cause breast  abscess in patients who have no traditional risk factors  Hospitals, nursing homes (patients with open wounds) are  the risk areas   Co-amoxyclav 1000 mg 2 times/day—7-10 days  Erythromycin 500 mg 3 times/day—7-10 days  Vancomycin 1.5 g vial 12th hourly x 7-10 days   Suspect MRSA infection when abscesses recur, abscess  persists and is nonlactational.   Fig. 21.9: Current thinking is  that repeated aspiration of the  breast abscess can also be an  alternative treatment to incision  and drainage    exudate. Breast is swollen, tense, tender and warm to touch.  These are the signs of cellulitic stage.   Once breast abscess develops, there is high grade fever  with chills and rigors and a soft, cystic fluctuant swelling  can be felt in the breast. In untreated cases, abscess may  rupture through the skin resulting in necrosis of the skin of  the breast, ulceration and discharge (Figs 21.7 and 21.8)  In deep-seated abscess, it is difficult to elicit fluctuation  and often fluctuation is a late sign. Hence, if throbbing  pain and fever with chills and rigors are present,  immediate drainage is mandatory. If not done, significant  amount of breast tissue will be destroyed.   ¢ Anti-inflammatory drugs such as ibuprofen 400 mg,  three times a day.   BREAST ABSCESS =f  Common organism—Staphylococcus aureus  Retracted nipple is one of the causes  Commonly seen during lactational period  Very painful condition  Do not wait for fluctuation  Guided aspiration should be done (Fig. 21.9)  Ultrasound can be done  Cloxacillin is the drug of choice  Nonlactational abscess—Metronidazole is drug of choice   e Repeated aspiration with antibiotic cover with or without  ultrasound guidance can be offered (Fig. 21.9)    ¢ For nonlactational breast abscess, add metronidazole  400 mg, 3 times a day for 5—7 days.    e The abscess should be drained—incision and drainage  (I and D) under antibiotic cover.   ¢ Ifthe abscess is situated in any quadrant of breast, other  than lower quadrant, it is drained by radial incision.   ¢ Abscess in lower quadrant ts drained by inframammary  incision placed in the inferior aspect of breast (refer to  breast abscess drainage under operative surgery section).   ¢ When both the breasts have an abscess, the breasts should  be emptied and the milk that is expressed can be boiled  and given to the child.    Fig. 21.11: Breast abscess drainage. Observe corrugated  drainage tube. Mostly it is replaced by tube drains today   An 18-year-old girl was admitted in a medical ward with the  diagnosis of pyrexia of unknown origin (PUOQO). All  investigations were normal except a high total WBC count.  After about a week, the duty nurse noticed that the patients  dress was stained with pus. It was a large breast abscess with  necrosis of the overlying skin. It was drained later. The girl  was so shy that she did not even complain of pain or swelling  of the breast. These types of cases are not uncommon in our  country.   e¢ When an abscess occurs in the breast and antibiotics are  given, without draining the abscess, the abscess cavity  may become fibrous and it results in firm to hard lump  in the breast. It gives rise to vague ill health of the patient.   RETROMAMMARY ABSCESS  It is collection of pus behind the pectoralis major (Fig. 21.12).   4. Empyema necessitans: Empyema of lung, if left  untreated, tracks out and the pus collects in the subcu-  taneous plane posteriorly and retromammary region  anteriorly, thereby forming retromammary abscess. There  may be a tense, tender and cystic lump palpable which  can be confused with breast abscess.    ¢ Chest X-ray to rule out pulmonary tuberculosis.  ¢ It is treated by draining the abscess by means of sub-  mammary (Galliard-Thomas) incision.   ¢ In chronic cases, retraction of the nipple can occur which  is partial or slit-like.   ¢ It can also be due to an infected sebaceous cyst.    OTHER TYPES OF BREAST ABSCESSES ¢ Presents as a lump, which can be hard and mimic   ¢ Clinical features may include multiple tender abscesses  sinuses and matted nodes in the axilla (need not always be  present).   ¢ Few types: Nodular, sclerosing, disseminated type, TB  mastitis obliterans and miliary form.   ¢ FNAC, biopsy will help in the diagnosis—caseating  granuloma with Langhan type of giant cells.   ¢ Aspiration of pus, lumpectomy, excision/even mastectomy  may be required along with concurrent antitubercular  treatment (Fig. 21.13).   Fig. 21.13: Tuberculous mastitis (Courtesy: Dr Maruthu Pandyan,  Govt Medical College, Madurai)   ¢ They are fibroepithelial tumours composed of an epithelial  and cellular stromal component.   ¢ Earlier known as cystosarcoma phylloides and mistakenly  labelled as a giant intracanalicular fibroadenoma. They are  tumours of the breast, with a predilection to attain massive  size and recur locally after lumpectomy (Figs 21.14 to  21.18).   More than 60% younger women  Depends upon mitotic activity, cellularity  and infiltration at the edge of the tumour   Red, dilated veins over surface, warm to touch  Bosselated surface (big nodules), a few cystic areas.    ¢ As the tumour grows very fast, 1t undergoes necrosis in  various places resulting in cystic areas within the breast.  It discharges serous fluid. Hence, the name serocystic  disease of Brodie. However, it rarely feels cystic.    ¢ Histologically, the tumour cells have a branching pattern,  penetrating the cystic cavity (phy/lus means leaf-like  pattern). Fibrous stromal proliferation is a feature.   Ultrasound will help to detect the size of the tumour, solid  and cystic areas    Small: Wide excision (1—2 cm margin). Lumpectomy  Should not be done as it can cause recurrence.   Fig. 21.14: Phylloides tumour of the left breast Fig. 21.15: Phylloides tumour of the left Fig. 21.16: Malignant phylloides  tumour (recurrent)   Fig. 21.17: Phylloides tumour of the left breast at  surgery (Courtesy: Dr Hartimath B, Associate  Professor, KMC, Manipal)   Fig. 21.18: Phylloides tumour with dilated  veins (Courtesy: Dr Sreejayan, Professor  of Surgery, Calicut Medical College, Kerala)    Rapidly growing carcinoma of the breast can undergo cystic  degeneration resulting in a cyst, called intracystic carcinoma  of the breast. Aspirationreveals haemorrhagic fluid, containing  malignant cells and which refills after aspiration. Other features  of carcinoma of the breast may be present.   The concept of Aberrations of Normal Development and  Involution (ANDI) of the breast was first published by LE  Hughes et al of Cardiff Breast Clinic in 1987. The ANDI  classification is based on pathogenesis, and recognises that a  spectrum exists from normal, through mild abnormality, to  disease. This has resulted in a radical change in attitude to the  understanding and management of breast disorders. Changes  previously regarded as disease are so common that they must be  regarded as lying within the spectrum of normality (Fig. 21.19).   This concept is of value in dispelling the supposed  association between the benign conditions and cancer. Most  patients with lumps and mastalgia are concerned that they may    Aberration  (Benign breast disorder)  Conditions considered too common an occurrence and  too mild a state to warrant    be harbouring cancer (the result of a successful information  campaign!!), and once a definite opinion of the benign nature  of the lump is conveyed and the patient 1s informed that no  further treatment 1s required, most would be reassured.   Having noted the above, a word of caution is due here—It  is preferable to overtreat a benign breast disease rather than  miss or delay treatment of an early carcinoma of the breast  (Fig. 21.19). So following the clinical dictum “In a patient  who is above the age of 40, with a recently detected lump in  the breast, 1t should be considered to be carcinoma breast until  proved otherwise” may prove to be a wrong answer in many  but the correct management in most.   From the above, it follows that, in case of doubt (where  there 1s a high risk patient or doubtful signs of malignancy) it  would be prudent on the part of the treating surgeon to rule    Fig. 21.19: Benign breast disorders and diseases (Courtesy: Dr Stanley Mathew, Professor of Surgery, KMC, Manipal)    'Note: Fibroadenoma is an AND (Aberration of Normal Development) of a lobule.  : Cyst (macrocyst) is an ANI (Aberration of Normal Involution) of a lobule.    Aberration of the Normal Development and Involution (ANDI) of the breast    35_55 Macrocysts  (35-55 years) ing microcysts, apocrine Sclerosing lesions Cystic diseases  changes, fibrosis, adenosis) adenosis X-ray abnormalities    Ductal involution Duct dilatation  (including periductal round  cell infiltrates), dilate with    Terms to be avoided: Fibroadenosis—replace with mastalgia and nodularity.  Cystosarcoma phylloides—replace with phylloides tumour.   Instructions to the students: Carefully go through Tables 21.1, 21.2 and Fig. 21.1    If it is not possible to conclusively rule out the possibility It can be classified as given in Table 21.3.    of malignancy, then the patient would be best advised a  lumpectomy.    * If painful nodularity is present for more than one week, it  is significant (more than normal discomfort).    fibroadenosis (now termed mastalgia with nodularity).  ANDI includes several aberrations and disorders (Table  21.1). The following table includes all the aberrations,  disorders and disease entities originally included under the  ANDI classification as proposed by LE Hughes, et al.    Also called mammary dysplasia, fibrocystic disease,  Schimmelbusch disease, hormonal mastopathy, or fibro-  adenosis.   ¢ Cysts, apocrine metaplasia  ¢ Duct ectasia .  ¢ Calcification .   Pain can be due to periductal  mastitis or costochondritis  (Tietz’s disease)    Analgesics may be beneficial  Injection with local anaes-  thetic on a trigger point    The term ANDI should not be confined to imply fibro-  adenosis (now termed mastalgia and nodularity). In fact  fibroadenoma 1s an AND of a lobule and cyst (macrocyst) is  an ANI of a lobule.   * [tis an aberration of physiological changes that occur in  the breast from menarche till menopause. It 1s an ANDI  (aberration in normal development and involution).   ¢ Women around the age of 40 are the usual sufferers.    I. Fibrosis results in increased connective tissue growth. Fat  and elastic tissue become less, and chronic inflammatory  cells such as plasma cells, can be present.    2. Cyst formation: Fibrosis compresses the ductules, which  is responsible for cyst formation. Hence, it 1s a retention  cyst. The cyst contains dark mucoid material and it may  discharge serous fluid or green coloured fluid through the  nipple. Hence, it is called fibrocystic disease of the breast.   Fig. 21.20: Submammary incision is given to  excise a large lump in the breast    Cyst may be single or multiple confined to one lobe or  many lobes. These are microcysts.    3. Adenosis: Proliferation of the acini and gland ts an  important feature of fibroadenosis.    4. Epitheliosis: Fibroadenosis is not a precancerous condition  but if the degree of epitheliosis is more, it can be considered  as premalignant condition. Epithelial hyperplasia mainly  occurs in the acini.    5. Papillomatosis and apocrine metaplasia of the epithelium  lining cystic spaces are the other features. These changes  are not considered premalignant.    ¢ Females around the age of 30-40 are the victims—spinsters,  married childless women and women who have not suckled  their babies are the usual sufferers.    e Severe pain in the breast in the premenstrual period and  during menstruation. Itis called cyclical mastalgia. Upper  outer quadrant, bilaterally is affected.   ¢ Clinical examination of the breast reveals a coarse, nodular,  tender, lumpiness which is better felt with the finger and  the thumb. Often, there are multiple, irregular, firm,  nodularities palpable bilaterally, especially in the upper  outer quadrants. Nipple discharge which Is serous or green  coloured may occur.    ¢ Evening primrose oil in adequate doses for 3 to 4 months  are beneficial in a few patients. These patients have  abnormal fatty acid profiles and decreased levels of  metabolites of linolenic acid. Treatment with primrose oil  improves essential fatty acids because it is rich in  polyunsaturated essential fatty acids (oleic acid and linoleic  acid). Costly but still worth trying as first line of treatment,  specially useful in women above the age of 40 (Key Box  at OY.    First choice of treatment of cyclical mastalgia is evening  primrose oil (EPO)   Bromocriptine which decreases the prolactin levels, 1.25  mg, twice a day, may reduce the pain and may be increased  to 2.5 mg twice a day. It 1s useful for cyclical mastalgia.    Danazol, it is a gonadotrophin releasing hormone inhibitor.  200-400 mg/day, thrice a day 1s given. It acts by reducing  FSH and LH levels.   Tamoxifen 10 mg, twice a day 1s a better alternative to  danazol.   Treatment may have to be continued for some months  (Table 21.4).    Danazol is the best drug in cases of noncyclical mastalgia  but it is teratogenic. Hence, the patient should take  precautions against pregnancy.    Indications for surgery in fibroadenosis:  1. FNAC suggests epitheliosis  2. A very painful lump  3. A hard lump about which the patient is worried and anxious  ¢ Excision of the lump. Surgery for fibroadenosis ends  up in removing some breast tissue and the lump. As it  has no capsule of its own, it is a messy surgery unlike  fibroadenoma surgery (Figs 21.20 to 21.23).  ¢ Istherearole for subcutaneous mastectomy? In patients  with family history of breast carcinoma, if they have   ~ ™/ al  Fig. 21.23: A circumareolar incision is given to excise the lump    lumps in the breast with severe degree of epitheliosis, it  may be worthwhile doing subcutaneous mastectomy.    It is a benign tumour In which the epithelial cells are arranged  in a fibrous stroma. It is an AND (Aberration of Normal  Development) of a single lobule.    1. Pericanalicular type in which fibrosis is more  2. Intracanalicular type in which fibrosis ts less.    e Small fibroadenomas (1 cm in size or less) are consi-  dered normal.    Clinical features  ¢ Peak age of incidence 1s at 20 years.  e Patients present with painless lump in the breast.    ¢ It is smooth, round bordered, firm to hard 1n consistency,  and freely mobile within the breast.   ¢ Due to its free movement within breast tissue, it is known  as breast mouse.    ¢ However, when fibroadenoma occurs in elderly patients, it  may not have characteristic features because of fibrosis.   A paediatrician s mother, 52-year-old, underwent surgery for  fibroadenosis. The pathology report was fibroadenosis with  severe epithelial dysplasia. She came for follow up after  3 months. There were no freshchanges in the breast. However,  after 9 months of surgery she presented with a hard lump  with significant nodes in the axilla. She underwent Patey  mastectomy. The case illustrates, epitheliosis is undoubtedly  premalignant. Should the lady have been advised to undergo  subcutaneous mastectomy after the first surgery?    ¢ Excision of the lump  1. Inintracanalicular type (Fig. 21.23), the lump 1s deeper  and peripheral. It 1s removed by submammary incision.  2. In pericanalicular type (Figs 21.24 and 21.25), the  lump 1s superficial. It is removed by periareolar incision    ¢ Fibroadenoma and malignancy: Patients with simple  fibroadenoma and no family history of breast cancer have  no risk of cancer. Complex fibroadenomas which show  cysts, sclerosing adenosis, calcification have increased risk  (3 to 4 times) of cancer.  Fibroadenoma and fibroadenosis are compared in Table  a    ¢ Actual cause 1s not known. Mild low-grade infection  (anaerobic bacteria) has been considered as one of the  factors.   ¢ Due to dilatation, the contents tend to undergo stasis. The  epithelial debris, and serous fluid collectively form a thick  paste-like material rich in lipid.   ¢ There is intense periductal inflammation with lympho-  cytes and plasma cells (Fig. 21.26). Hence, it is called  plasma cell mastitis (Key Box 21.10).   e After some time, because of fibrosis, a lump can be felt,  which can be confused for carcinoma of breast.   Fig. 21.26: Excised specimen. Observe the fibrosis due to  periductal inflammation   OF PERIDUCTAL MASTITIS  Periductal infiltration of plasma cells, lymphocytes  Excision of all major ducts (Hadfield’s operation)  Retraction of nipple (partial)  Infection by anaerobes/irritation by smoking  Dilatation of breast /ducts  Untreated—Abscess/fistula/lump  Creamy/paste-like discharge  Treated by co-amoxiclav and metronidazole  Remember as PERIDUCT   Fig. 21.27: Periductal mastitis at surgery. It was feeling hard  (Courtesy: Dr Geetha, Associate Professor of Surgery, KMC,  Manipal)    Often patients present with features of mastitis  (nonlactating) with pain, nipple retraction, lump in the  breast.   Some of them are treated with antituberculous treatment  with FNAC, trucut or even biopsy findings of granuloma  but they are nontuberculous.   When tuberculosis, sarcoidosis, diabetes and Wegener’s  granulomatosis are excluded, the diagnosis of idiopathic  granulomatous mastitis 1s made.    ¢ Symptomatic treatment to decrease pain and fever  ¢ Steroids—30-40 mg prednisolone for 3 to 6 months period.  ¢ Refractory cases need to be treated with surgery.    ¢ They occur due to excessive secretion of thin fluid within  the lobules which enlarge to produce a cyst. Clinically, they  present as firm swelling in the breast. Itis an ANDI of a  single lobule (firm because it 1s tense).    ¢ One-third of patients may have more than one cyst in the  breast.   e If you suspect cyst, the investigation of choice is ultra-  sonography.   e Increased incidence of benign breast lumps including cysts  have been reported with prolonged use of hormone  replacement therapy.    Excision of cyst in the following situations:  1. Recurrence after 2 aspirations.    It is a solitary, subareolar retention cyst filled with milk. It  occurs and dates back to the lactational period.   It occurs due to inadequate drainage of the milk added by  epithelial debris which block the lactiferous duct. Once the  duct 1s blocked, proximally, the milky fluid accumulates  resulting in a huge enlargement of breast.   It is treated by repeated aspirations. Duct patency can be  restored by using 4-O nylon.    It is a common problem encountered in the outpatient  department. It can be physiological such as lactational and  or pathological from various causes.   The clinical evaluation of the case of nipple discharge  consists of following history examination.    2. Unilateral or bilateral: Duct papilloma 1s unilateral;  fibrocystic disease and hyperprolactinaemia are bilateral.   3. Single or multiple ducts: Duct carcinoma/duct papilloma  involves single duct, but fibrocystic disease affects multiple  ducts.   4. Spontaneous discharge is typical of duct papilloma. Blood  stained discharge on pressing the mass may be seen in  carcinoma breast.   5. Related to menstruation: This is seen in fibrocystic disease  and patients who are taking oestrogen replacement therapy.    ¢ Mammogram and ultrasound to detect any mass, any cystic  lesions. Very often they do not help in the diagnosis.    ¢ Stimulation during sexual activity.  ¢ Extremes of reproductive age (puberty and menopause)    ¢ If the cause is identified, such as drugs mentioned above,  stop them.   ¢ If any other reason is detected, then treat the cause.    ¢ It is a benign lesion of the breast, usually single and  unilateral, rarely multiple.   ¢ Middle-aged women are affected and present with bleeding  per nipple (Fig. 21.32).   ¢ The tumour Is situated in one of the larger lactiferous ducts.   ¢ It presents as a small swelling just beneath the areola,  palpation of which results in discharge of blood.   ¢ Since, it 1s a premalignant condition, it is treated by  microdochectomy.    ¢ Microdochectomy: The opening of the lactiferous duct  discharging blood is identified. It 1s probed with lacrimal  probe or a straight needle. A small triangular piece of skin,  along with the needle, and a wedge of breast tissue to a  depth of about 4—5 cm is removed (Figs 21.33 and 21.34).   ¢ Hadfield’s operation: Cone excision of the lactiferous  ducts is done when duct of origin of nipple bleeding 1s  uncertain. A circumareolar incision confined to one-third  of the circumference is given and deepened. The flap ts  better not raised to avoid necrosis of areola. A core or cone  of breast tissue from nipple to pectoral fascia or about 5 cm  length is removed. Most of the ductal papilloma will be  located within 5 cm from the nipple.   ¢ Itis usually bilateral and feels like fatty tissue, lobular, soft  to firm. This is how it is differentiated from lymph nodes  in the axilla.    T: Trauma either by a direct blow or by a seat belt or trivial  contraction of pectoralis major. Sometimes no history  of trauma.   R: Retraction of nipple, palpable hard lump, tethering of  skin mimic carcinoma   ¢ Idiopathic 1s the most common wherein no cause can be  identified. However, there are many other causes of  gynaecomastia which can be summarised as “MASTIA”.   ¢ In idiopathic variety, gynaecomastia is bilateral. In other  cases, it may be unilateral (Fig. 21.35).   Lumpectomy or mastectomy with preservation of nipple and  areola (subcutaneous mastectomy).    e Rarely, gynaecomastia can predispose to male breast  carcinoma (Fig. 21.36).   Fig. 21.35: Unilateral gynaecomastia—Often no cause is found:  It is tender (Courtesy: Dr Surya Prakash Saxena, Chief Surgeon,  District Hospital, Durg, Chhattisgarh)    Fig. 21.36: Gynaecomastia  (Courtesy: Dr Satish  Deshmukh and Dr Murtaza  Akhthar, NKP Salve  Institute of Medical  Sciences, Nagpur)    Carcinoma of the breast 1s the major killer of middle-aged  women In western countries. It 1s the second leading cause of  cancer death. However, mortality from breast cancer is  declining due to better screening and adjuvant treatment.    However innocent looking the breast lump may be, it  can be malignant unless proved otherwise.    1. Women: Women are 100 times more likely to have breast  cancer as compared to men.  2. Age: Carcinoma breast is very rare below 20 years of age.   3. Race: Highest in whites, rare in Japanese and Taiwanese  population  4. Breast cancer and hereditary factors:  a. BRCA I and BRCA II genes have been found in long  arm of chromosome 17 and 13 respectively in women  with a family history of carcinoma of the breast. BRCA  1 and BRCA 2 are the genes associated with increased  risk. BRCA I and II mutations are more common in  Ashkenazi Jews. They are more prone for ovarian cancer  also. Hence all patients with BRCA I and BRCA Il  mutations should consider a prophylactic bilateral  oophorectomy after child bearing is completed, with  bilateral mastectomy.  b. Cowden’s disease (multiple hamartoma syndrome)  ¢ Associated with reduced tumour suppressor gene PTEN  ¢ 30-50% of patients will develop breast cancer by 50  years of age.   ¢ The lesions found in this syndrome are multiple facial  trichilemmoma (pathognomonic), oral papilloma,  bilateral breast cancer, haemangiomas, lipomas, thyroid  tumours, etc.   c. Ataxiatelangiectasia: It is associated with haeman-  glioma and carcinoma breast.    A 35-year-old lady came to the hospital with fracture of the    right femur. An intern, during history taking, elicited history  of breast lump of 8 months duration. The patient reluctantly  allowed the doctor to examine her breast as the lump was  painless. She was found to have carcinoma breast with  pathological fracture of femur.    d. Li-Fraumeni syndrome is a rare disease with familial  breast cancer and is associated with inherited mutation  of tumour suppressor P53 gene.   ¢ 90% of carriers will develop breast cancer by the age of  50.   ¢ They also can have other tumours in childhood such as  soft tissue sarcoma, osteosarcoma, leukaemia.    Breast cancer with BRCA I tends to be ER negative (ER-)  Breast cancer with BRCA 2 tends to be ER positive (ER+)    5. History of breast cancer: Risk of developing second  breastcancer 1s about 0.5 to 0.7% in women with previous  invasive breast cancers.    Breast cancer is 3 to 4 times more likely to develop in  women with a first degree relative who had breast cancer.  This risk is further increased if they had premenopausal  and bilateral cancer.    Women with ductal carcinoma in situ (DCIS) are at an  increased risk of developing ipsilateral and contralateral  breast cancers (4.1% after 5 years).    6. Benign breast disease: In general proliferative breast  lesions are more vulnerable for malignancy. Nonpro-  liferative lesions such as cysts and duct ectasia do not  increase the risk of breast cancer (Key Box 21.12).    ¢ Alcohol > 2 units/day  ¢ Hormone replacement therapy  ¢ Proliferative benign breast disease (LCIS and ADH)   Radial scar and sclerosing adenosis: Both are microscopic  findings. They can be felt as hard lesions. Radial scars are  less than 1 cm. Doubtful role in malignancy or no increased  risk.    obese women and Is due to increased synthesis of oestrogen  (oestradiol) in the body fat. As a result of aromatisation  of androgens in adipose tissue, circulatory oestrogen  levels are increased. Alcohol intake is associated with a  1.5 fold increased risk of breast cancer. Vitamin C may  have protective value. Increased intake of saturated fats  and reduced intake of phyto-oestrogens increases risk.   a. Longer the cumulative period of menstruation more the  risk (early menarche and late menopause)    d.Hormone replacement therapy (HRT) _ in  postmenopausal women increases incidence of breast  cancer particularly when a combination of oestrogen and  progesterone 1s used.    ¢ Oral contraceptive pills are thought not to increase  breast cancer.    received mantle radiation for Hodgkin’s disease have  increased risk (19%) by the age of 50.    white, western women. It is rare in Japan and Taiwan.  Genetic predisposition exists in a few cases, especially in  bilateral breast carcinoma.    Majority of the tumours arise in the ductal epithelium  (90%) which 1s called the ductal carcinoma and about 10%  arise within lobular epithelium (lobular cancer).    It is the most common form seen in about 60-75% of  patients. In this variety, there is increased fibrotic reaction  and less cellular reaction. It presents as a hard lump. Hence  the name, scirrhous carcinoma. It produces grating sound  when it is cut. Cut surfaces are concave. The chalky white  necrosis and calcification may be visible occasionally. This    is the type of lesion which produces retraction of the nipple,  infiltration of the skin and fixity to the chest wall.  Malignant cells are round to polygonal with dark nuclei.  Perivascular and perineural space infiltration 1s common.  Atrophic scirrhous 1s an infiltrating duct carcinoma seen in  elderly patients when there 1s atrophy of the breast.    It is seen in around 15% of cases. It tends to occur in well-  formed breasts in the reproductive age group and it feels  more soft than hard. In addition to undifferentiated cells,  occasionally well differentiated gland formation ts present.    —  Redness, pain and enlargement appear so suddenly that it  is diagnosed as breast abscess. Hence the name, mastitis  carcinomatosa. It is differentiated from breast abscess by  absence of fever and presence of gross peau d ‘orange due  to blockage of subdermal lymphatics.  Blunder biopsy (incision) should not be done thinking that  it is an abscess (Fig. 21.41).  This variety has the worst prognosis.  High angiogenic and angioinvasive capability. Most of  them are ER negative.    Hence, the name, medullary adenocarcinoma. Presence of  lymphatic infiltration 1s thought to represent a good host  response, thus indicating a good prognosis.    e It is a misnomer. It 1s not a disease of the nipple but an    It constitutes less than 1% of all cases of carcinoma breast.  Dermal lymphatic invasion 1s characteristic.  Predominantly seen during pregnancy and lactation.  Malignancy grows so rapidly that it invades more than half  of the breast tissue. It comes under locally advanced breast  cancer (LABC).    intraductal carcinoma involving excretory ducts that  infiltrates nipple and areola early.   In advanced cases, entire nipple 1s destroyed (ulcerated).  Lump appears much later than changes in the nipple.   ¢ Microscopically, large hyperchromatic cells with clear  cytoplasm or clear halo are seen.   e It is due to intracellular accumulation of mucopoly-  saccharides. These cells are called Paget’s cells.    ¢ It1is diagnosed because of production of mucin, intracellu-  larly and extracellularly.    e Carcinoma in situ: This term is used when cancer cells  do not invade through basement membrane. Examples:  Lobular carcinoma in situ (LCIS) and ductal carcinoma in  situ (DCIS) (Table 21.7).    e Invasive carcinoma: This term 1s used when cancer cells  invade through basement membrane. A few examples are  Paget’s disease of the nipple, adenocarcinoma (scirrhous,  simplex, medullary carcinoma, etc).    Mutlticentricity refers to occurrence of a second in situ  breast cancer outside the breast quadrant of primary in  situ. carcinoma.    | Table 21.7 Comparison of lobular carcinoma in situ (LCIS) with ductal carcinoma in situ (DCIS)    Multifocality refers to occurrence of a second in situ breas  cancer within same breast quadrant as the primary in sift  cancer (Figs 21.43 and 21.44)    Upper and outer quadrant is commonly involved (60-65%  because breast tissue is more there.   Figs 21.43 and 21.44: Multicentric and multifocal—this is the  reason behind getting a mammogram before doing a breast  conservative surgery    Depending upon specific histologic features, they are further  classified into subtypes which are given below. However, it 1s  Interesting to note that 80% of invasive breast cancers are  described as invasive, ductal carcinoma of ‘no special type’  (NST).    I. Paget’s disease of the nipple  II. Invasive ductal carcinoma: 85% (Fig. 21.47)  a. Adenocarcinomas [scirrhous, simple, NST (80%)]  b. Medullary carcinoma 4%  c. Mucinous (colloid) carcinoma 2%  . Papillary carcinoma 2%  e. Tubular carcinoma 2%  III. Invasive lobular carcinoma 10%  IV. Rare types: Adenoid cystic, squamous cell, apocrine.    Fig. 21.45: Comedocarcinoma in situ—it is a DCIS—dilated  terminal duct lobular units (TDLU) lined by malignant epithelial  cells with luminal necrosis. No invasion into stroma. Slowly, the  lumen gets occluded, can present as cyst and later calcification   Fig. 21.46: Normal vs carcinoma in situ: Here you can see normal  breast lobule (left) and in situ carcinoma (right) (Courtesy: Both  Figs 21.45 and 21.46 by Professor Laxmi Rao, Head, Dept. of  Pathology, KMC, Manipal)    ¢ It can be ductal—ductal carcinoma in situ (DCIS)  ¢ Lobular—lobular carcinoma in situ (LCIS)   This is a type of cancer without infiltrating epithelial  basement membrane. No lymph nodes will be enlarged. Hence,  no role for axillary block dissection. 5-years disease free  survival for DCIS 1s calculated by using modified Van Nuys  prognostic index for ductal carcinoma in situ (DCIS).   Fig. 21.47: Infiltrative ductal carcinoma: Infiltrating islands of  malignant cells forming glands and trabeculae surrounded by  desmoplastic stroma (Courtesy: Dr Laxmi Rao, HOD, Pathology)    5-year disease free survival for VNPI 4 to 6 = 100%.    Lobular carcinoma is more dangerous because it is  multifocal and bilateral.    ¢ Lobular carcinoma refers to a lesion developing from the  acini or terminal ductules of the lobule.   ¢ More in situ carcinomas are diagnosed because of  mammography and most of the breast conserving surgery  1s done in this group of patients.    ¢ Comedos means cast or plug. It is a peripheral carcinoma  wherein the tumour cells block the ductules by forming a  case or plug producing a small cystic lesion. It is an example  for intracystic carcinoma.    CLINICAL FEATURES OF CARCINOMA BREAST ¢ Centrally retracted nipple: Recent retraction indicate    ¢ Lump in the breast is the most common presentation. It is malignancy. It is due to fibrosis caused by extension 0  the breast tissue in the upper and outer quadrant which is growth along the lactiferous duct. Remote retraction 1  most frequently involved (65%). Typically it is hard and either idiopathic or due to recurrent mild infectio1  irregular but it can also be firm. Fixation to the skin, (mastitis).    wall occurs late ( Figs 21.48 and 21.49). PEARLS OF WISDOM    ¢ Bleeding per nipple is an uncommon symptom of carcinoma Circumferential retraction is due to carcinoma. Slit-  of the breast. It involves multiple ducts and is unilateral. like retraction is due to duct ectasia or periductal mastitis.   e About 5% of patients present with bony metastasis give . .  rise to bony pains, 1.e. pathological fractures, paraplegia, Causes of retraction of the nipple (Figs 21.50 to 21.59  quadriplegia, etc. ¢ Carcinoma of the breast    ¢ Chronic mastitis  ¢ Congenital  ¢ Chronic disease—tuberculosis  ¢ Level of the nipples: The nipple may be elevated and  retracted (Fig. 21.50) because of fibrosis induced by  the growth. One should carefully watch for the level of  nipple to detect an early case of carcinoma. Destruction  of the nipple is a feature of Paget’s disease of the  nipple.  2. Areola  e Presence of peau d’ orange indicates the tumour  infiltrating the areola. It has been compared to orange  —, skin because of following reasons:  Fig. 21.48: Scirrhous carcinoma presenting as hard lump with ~The areola becomes thick because of lymphatic  peau dorange obstruction giving rise to lymphoedema.  — Fixation of hair follicles and sweat glands to the  underlying malignancy.   3. Skin over the breast  ¢ Puckering or dimpling of skin is due to thin fibrous  bands which are embedded tn the subcutaneous fat and  are attached to the skin and pectoral fascia called  ligaments of Cooper which are infiltrated by the  malignancy. Multiple nodules indicate advanced disease.    5. Oedema of the arm is due to lymphatic blockage caused  by lymph nodes in the axilla.    1. Local rise of temperature and tenderness are usually not  found in cases of carcinoma of the breast. However, rapidly   Fig. 21.59: Cancer en  Cuirasse (Courtesy: Dr  Sreejayan, Prof. of Surgery,  Calicut Medical College,  Calicut, Kerala)    Fig. 21.58: Paget's disease of the nipple (Courtesy: Dr Sreejayan, é  Prof. of Surgery, Calicut Medical College, Calicut, Kerala)    2. Describe the lump: The lump is the commonest  presentation of carcinoma of the breast. The upper and outer  quadrant is the commonest site of carcinoma of the breast  because of more breast tissue in that quadrant. Typically,  the lump is hard and irregular. However, very often  carcinoma breast can present as a firm lump. In mastitis  carcinomatosa the lump can be soft due to tumour necrosis.    3. Intrinsic mobility may be present but it moves with the  breast tissue (fibroadenoma moves independent of breast  tissue).    ¢ Lift the skin. If it is not possible, it indicates that the  tumour 1s fixed to skin.    ¢ Pectoralis major contraction test: Ask the patient to keep  the hands on the flanks and press against the hip. If the  lump cannot be moved after contraction, it indicates  fixity to pectoralis major.    ¢ Fixity to the chest wall is assessed by 2 methods:    1. A tumour which is fixed to the chest wall will not be  mobile when pectoralis major is relaxed.    ¢ Serratus anterior contraction test by pressing the hand  against the wall. The test has to be done when the tumour  is situated in the outer and inferior quadrant.    * There are 5 groups of nodes in the axilla which are  described under lymphatic drainage of the breast. However,  very often, central group of nodes and pectoral nodes are  enlarged. It is very difficult to feel the apical group of  nodes.   * Ifthe axillary nodes are hard, with or without fixity, they  are significant.   ¢ Soft to firm nodes need not be malignant but can be due to  secondary infection because of fungating, ulcerating  growth.    2. Abdominal examination for secondaries in the liver which  present as nodular liver, ascites and Krukenberg’s tumour  bilateral bulky ovarian metastasis.    5. Bony tendermess should be looked for in the spine, lon:  bones, skull, etc.    As the tumour grows in size, it infiltrates the skin causin;  ulceration, fungation, bleeding, foul smelling discharge  Later, it involves pectoral muscles, chest wall, etc.   ¢ Medial quadrant tumours may involve the interna  mammary glands in the upper three or four intercosta  spaces, close to the stemum.   ¢ Lymphatics from inner medial quadrant of the breast  penetrate the rectus sheath and join the intraperitonea  lymphatics, thus producing ascites, Krukenberg’s tumours  (in premenopausal patients, ovary is vascular and fertile).  rectovesical deposits, secondaries 1n the liver.    ¢ Secondaries in flat bones are common (vertebral column.  femur, ribs, scalp, etc).   ¢ Secondaries in brain results in headache, vomiting and  blurring of vision.   ¢ Malignant pleural effusion is the common cause of death  in carcinoma of breast.   2. Increased ALP levels in the blood suggests bone metastasis  or liver metastasis   3. Mammography (Figs 21.60 to 21.63)  ¢ This has become the first diagnostic investigation  ¢ Diagnostic accuracy 1s about 90-95%.  ¢ It should always be combined with a clinical examination  ¢ Mammography is done when there is a doubt about the    This is a classification system that is currently used in  clinical radiology practice now.    Procedure  ¢ A selenium coated X-ray plate is used directly in contact  with the breast and the breast 1s exposed to low voltage  and high amperage X-ray.  e 2 views: (a) Mediolateral and (b) craniocaudal  Indication  ¢ Coarsely nodular breast  ¢ Fibroadenosis  ¢ Woman, aged 40 years with family history of breast cancer   microinvasion 0.1 cm orless in greatest dimension  0.1-0.5 cm in greatest dimension    extension into chest wall including intercostal  muscles, ribs, serratus anterior (not pectoralis major)  extension into skin—peau d’ orange, ulceration,  cancer en cuirasse/satellite lesions (no puckering/  dimpling)   metastasis to ipsilateral axillary lymph node  fixed/matted to one another or other structures  ipsilateral internal mammary nodes in the absence  of clinically evident axillary lymph node    metastasis to ipsilateral infraclavicular lymph node  ipsilateral internal mammary and axillary lymph  node    Stage 0  Stage |  Stage lla  Stage IIb  Stage Illa  Stage IIIb  Stage Illc  Stage IV   pN1b internal mammary nodes (not clinically apparent),  microscopic disease in sentinel lymph nodes   pNic metastasis in 1-3 axillary lymph nodes and in  internal mammary nodes (pN1a+pN1b)   pN3a > 10 lymph nodes metastasis or metastasis to  infraclavicular lymph nodes   e What is given here is staging after an axillary block  dissection. Hence, it is called pathological staging. If you  know this, you may score higher marks.    Disadvantages of mammography  ¢ False positive cases are around 5%. Hence, mammo-  graphic positive cases should undergo FNAC to confirm  the diagnosis.  ¢ If a lesion is detected by mammography, but is  impalpable, the following special techniques will help.    In young women, muicrocalcifications are the only  mammographic abnormalities suggesting malignancy.    lete. The X-ray may have beencloudy, making it difficult to read  the images. This can happen, for example, if the patient moved  at the precise moment the picture was taken.    : This score means that mammogram is negative (that is, no    : This score also means that mammogram is normal, with no    apparent cancer, but that other findings (Such as cysts) are  described in the report.    Screening: Asymptomatic women of more than 40 years.  Diagnostic: Women with pain in the breast, mass,  discharge, family history of breast cancer   2 views—Mediolateral oblique view is for outer quadrant  and axilla. Craniocaudal view is for medial quadrants.  Radiation dose is 0.1 centigray (cGy)—4 times that of chest  X-ray dose but no side effects.    3: A score of 3 means that mammogram is probably normal Benign lesions are round, punctate, popcorn like, etc.  but that there is an approximately 2 percent chance of Highly suspicious—pleomorphic, heterogeneous  cancer. Hence, follow-up with a repeat mammogram in six — Solid mass with irregular edges, spiculation  months. And if there is a family or personal history of breast — Long tentacles—tentaculation  cancer, the radiologist may opt to do more tests at this stage Sie SPAR SEG Wa CTOs Ran eR iGhD GuICIREGNon  rather than wait. ;   4: This score means that the findings on the mammogram are — Distortion of architectural pattern of the breast.  suspicious and that there is an approximately 20 to 35 — Asymmetrical thickening of breast tissues.  percent chance that a breast cancer is present. Core biopsy  is a must in such cases for tissue sample.   5: This score means that the mammogram results are highly 100% accurate in the diagnosis of breast cancer (see under  suspicious, with a 95 percent chance of breast cancer. image guided biopsy).    45% of breast cancers can be seen on mammography  before they are palpable.    A. Double dye technique of injecting contrast medium and 4. FNAC (Fine Needle Aspiration Cytology) is quick, safe and   ¢ False negative 15% (infiltrating lobular carcinoma,  tubular carcinomas may mimic fibroadenoma)    preoperative assessment of hormone receptors can be  done. False negative (25%) and no false positive (0%).   6. Incisional biopsy: Under local anaesthesia, a small  Incision 1s made overthe lump anda biopsy is taken, which  is sent for frozen section. If frozen section report 1s  malignant, an appropriate mastectomy Is done. If the frozen  section 1s negative, a lumpectomy 1s done and if report  comes later as malignant, a mastectomy is done.    a. Ultrasound guided biopsy: Microcalcifications are seen  infrequently but if found, they can be biopsied using  ultrasound as a guide. Success rate of positive histo-  pathology is high (Fig. 21.64)   b. Wire localisation (Figs 21.65 to 21.67)  ¢ Indication: Localising nonpalpable breast lesions.   e Procedure: Mammogram is done and the suspicious  area 1s localised (microcalcification). A hooked wire  enclosed within a needle is placed within the breast  under guidance. The needle is removed. Patient is  shifted to OT. In the OT incision and dissection follows  the wire till ‘Hook’ is reached and tissue 1s excised  and sent for histopathology.   d.Mammotome: It is a hand held vacuum-assisted biopsy  instrument which can be used under image (ultrasound  or stereotactic) guidance. It gives accurate diagnosis of  nonpalpable mammographic abnormalities.   8. Chest X-ray: Rule out pulmonary secondaries, effusion,  or mediastinal widening (2 types) (Fgis 21.68 and 21.69)  ¢ Cannon ball type (haematogenous)—presents late  ¢ Lymphangiectatic type (lymphatic spread))—presents   9. Abdominal ultrasonography is done to rule out secon-  daries in the liver, ascites, rectouterine deposits.  ¢ Incidence of liver metastasis in Ca breast 1s 6%. Routine   USG abdomen its indicated only if hepatomegaly is  present or any abnormality in LFT or in stage III or IV.   Fig. 21.64: Ultrasound detecting a Figs 21.65 to 21.67: Mammography directed wire localisation (Courtesy: Dr Sampath    lump in the breast and a wire is being Kumar, Professor of Surgery, Dr. Chandrakant Shetty, Professor of Radiology and Imaging,  introduced within Dr Poornima, Resident, Dept of Surgery, KMC, Manipal)    Intracellular steroid hormone receptor proteins, primarily  ER (oestrogen receptors) and PR (progesterone receptors)  have shown to be indicators of prognosis and a guide to  hormone and endocrine therapy. About 50 to 85% of  invasive breast cancers contain measurable amounts of ER.  The concentration of ER increases with age with both  having their highest levels in postmenopausal patients.    Normal value of ER is < 10 fmol/mg proteins. It is consi-  dered positive > 10 fmol/mg proteins. Upper levels as high  as 1000 fmol/mg proteins may be there.    The presence of ER implies that normal cellular mechanism  for processing oestrogen has been maintained despite the  malignant change. Patients with ER positive tumours  have longer disease-free survival after primary treatment,  Superior overall survival and longer survival after recur-  rence compared with patients with ER-negative tumours.    Before starting hormonal therapy steroid hormone receptors  have to be checked and measured. Depending upon the  levels, whether positive or negative, the hormonal therapy  is given (details below).   All patients may benefit from tamoxifen except  premenopausal ER/PR negative patients. Tamoxifen 1s  started only after chemotherapy is completed. It should be  given for 5 years.    HER-2/neu receptor: It is a membrane tyrosine kinase  receptor and a marker of cellular proliferation, expressed  in up to 50% of cases. It is usually associated with ER  negativity and high grade tumour, poor prognosis but there  is a better response to adriamycin (Key Box 21.15).    Measured by immunohistochemistry  Test for gene amplification—fluorescent in situ hybridisation  (FISH) is gold standard.  25% of all breast cancers overexpress HER-2  It is a transmembrane tyrosine kinase receptor  High grade tumours usually express this  ER negative—poor prognosis  Responds to ‘TRASTUZUMAB’  Thus ‘FISH’ is done only when HER-2/neu is 2+    Detailed histopathological examination and followed by  immunohistochemistry (IHC) and DNA microarrays fortr  an important part of treatment or the breast cancer. The  new marker for cellular proliferation has been identifiec  and named Ki 67 (Key Box 21.16).   A cut off value of < 14% is generally used to denote low and  high values    Investigational Algorithm in a 40-year-old female with 5 cm lump in the outer upper quadrant   IHC-ER, PR, HER-2/neu,  SBR grade, Ki67 index  DNA _ microarrays j    D. DNA microarrays: They compare DNA from normal cells  to breast cancer cells. They are very expensive.    Examples:  1. Oncotype Dx—it is 21 gene assay  2. Mammaprint—/70 gene assay    Based on these, treatment is divided into specific groups  referred to as ‘luminals’—this is the latest treatment and is  given in page 422.    ¢ Tumour, node, metastasis (TNM), oestrogen receptor (ER)  ¢ Progesterone receptor (PgR)    Tumour grade: Depending upon three factors such as nuclear  pleomorphism, tubule formation and mitotic rate, tumours are  given scores and graded. Grade III tumours means they are  poorly difterentiated. Grading system is popularly called “SBR’  system—Scarff Bloom Richardson—Elston-Ellis modified  ‘SBR’ grading system.   Score |— > 75% cells in tubule form  Score 2— > 10—75% cells in tubule form  Score 3—< 10% cells in tubule form   Well-differentiated: Grades 3,4, 5  Moderately differentiated: Grades 6, 7  Poorly differentiated: Grades 8—9    13. Role of MRI!  * Particularly useful in detecting malignancy when  mammographically subtle or occult (lobular carcinoma).  ¢ It can differentiate scar tissue from cancer. Hence can  detect local recurrence after surgery.  ¢ MRI is better than mammogram in assessing the response  of the tumour to neoadjuvant chemotherapy.    It is also better investigation in dense breasts and in  pregnancy.  ¢ Also for imaging of the breast with implants.    When axillary nodes are positive for adenocarcinoma,  but the primary is unknown, MRI is the tdeal  investigation to detect an impalpable breast lump.    14. Bone marrow aspiration in cases of unexplained  cytopaenia or leukoerythroblastic blood smear.    Thus ‘TRIPLE ASSESSMENT? is the most important  method for the diagnosis of carcinoma. It includes:  e History and clinical examination  ¢ Imaging: Below 40 years—Ultrasound;   Above 40 years—Mammogram  e fine needle aspiration cytology.  Students are hereby instructed to follow the investigation  which are available and routinely done in your hospital  from the clinical point of view and select the investigation  based on staging of the disease.    ¢ Halsted did the first radical mastectomy for carcinoma  breast in 1878. This was the gold standard operation for  carcinoma breast for nearly 100 years. Carcinoma breast 1s  considered to be a systemic disease to begin with according  to Fischer’s theory if we consider the biology of tumour  growth.    'From practical point of view, the clinician should select the best investigation in each patient and not all the investigations given here.    10' <0.5 cm No independent blood supply, less aggressive  10? @.S.em Has its own blood supply  10? lcm Has its own blood supply    Hence tumours with 0.5—1 cm or more may exhibit  haematogenous spread owing to their own blood supply.  Therefore, it is logical to treat it as a systemic disease and  apply some mode of systemic therapy for tumours > | cm.    Also, the other modalities of treatment such as radiotherapy,  chemotherapy, hormonal therapy are playing a major role  together with surgery. Hence, the current approach is to do  minimal local surgery and aggressive approach towards  management of lymph nodes or metastasis.    a. Early breast cancer—Stages I and IIA  b. Locally advanced—Stages IIB and III  c. Advanced/metastatic—Stage IV    Surgical management is thus based on the stage of the  disease. There 1s no confusion regarding management of either  early breast cancer or metastatic breast cancer (MBC).  Controversy exists in the management of LABC (IIT) which  has both operable and inoperable diseases which is given  in page 411.    ¢ Locoregional control of primary disease is by surgery  in the form of modified radical mastectomy (MRM)  or breast conservative surgery (BCS).   ¢ Systemic control of disease is by hormonal therapy or  chemotherapy.    1. Wide local excision (lumpectomy) is indicated in tumours  less than 4 cm in size and with well-differentiated histology.  It includes removal of the tumour with a rim of at least  1 cm of normal breast tissue. If the nodes are palpable and  enlarged, this is combined with axillary block dissection,  using separate incision. Currently, this procedure has  become more popular. It is also called Breast Conservative  Surgery (Key Box 21.17). Contraindications for BCS are  given in Key Box 21.18.   e Should not do undermining of the flaps as we do in MRM   e Direct approximation of the skin with no attempt to close  the fatty tissue.    CONTRAINDICATIONS FOR  LUMPECTOMY (BCS)  e Multicentric disease (rule out by mammogram)  e Pregnancy  e Central quadrant tumour (relative)  e Prior radiotherapy to the breast  e Prior chest irradiation  e Collagen vascular diseases  e Recurrence after BCS  e Skin fixation, involvement of nipple, chest wall   Mostly done and removal of pectoralis minor  helps the axillary dissection    5. Ideally specimen mammogram and frozen section is done widely practised surgery. It 1s also called modified radical  to look for clearance mastectomy. In this, the entire breast including nipple and  6. Cavity should not be closed or obliterated by sutures as areola (simple mastectomy) are removed with, pectoralis  small seromas get absorbed minor, followed by axillary block dissection. A complete    posteriorly. : '  8. Metal clips or coloured beads can be applied. ¢ Level : Extends from axillary tail to the lateral  9. Drain should not be kept border of pectoralis minor.  10. Anterior and posterior margins are less important if a full ¢ Level II : Extends from lateral border of pectoralis  thickness of breast tissue is removed. minor to medial border of pectoralis minor.   4. QUART therapy by Veronesi: It includes Quadran-  tectomy (the entire segment of the breast containing tumour  is removed), Axillary block dissection and Radiotherapy  to the breast or axilla.  ¢ However, quadrantectomy, by removing large amount of   breast tissue, gives rise to poorer cosmetic results. It seems  unnecessary to remove entire segment of the breast, when  in reality breast cannot be strictly divided into quadrants  (unlike hepatectomy). Hence, it is not very popular.   ¢ Function of the shoulder is better, and it gives a stronger  and more useful arm.   ¢ Entire breast including nipple and areola, skin overlying  the tumour along with fat, fascia and lymphatics.   ¢ Axillary block dissection, including complete clearance  of axillary fat and up to Level III nodes clearance.   ¢ Sternocostal portion ofpectoralis major, entire pectoralis  minor, few fibres and aponeurosis of internal oblique,  serratus anterior, latissimus dorsi and subscapularis.   Fig. 21.72: Local wide excision is done—if the skin is involved, it  is also removed, undermining of the flaps is not required    Fig. 21.73: Local wide excision is in progress. 1 cm of normal * Lymphoedema of arm  breast tissue of the cancerous lesion is all that is necesary ¢ High morbidity rate    Fig 21.78: Same patient as shown in Fig. 21.77. Axillary block  dissection is complete   Fig. 21.75: Patey mastectomy specimen: In this operation, entire  breast including axillary tail with all the axillary group of lympr  nodes and pectoralis minor are removed   Fig. 21.77: You can see the axillary vein and thoracodorsal nerve  as the axillary dissection is in progress   Fig 21.79: Initially local wide excision was planned in this patient.  Mammogram revealed multicentricity. Hence, MRM was done    Please note: In our country, modified radical mastectomy 1s  more frequently done. However, an early carcinoma is being  treated more and more by breast-conserving surgery nowadays.  Radical mastectomy is obsolete now.    ¢ In early breast cancer with no clinically apparent nodes  and if the disease is not multicentric, then sentinel lymph  node (SLN) biopsy can be considered. Otherwise, axillary  dissection is recommended. Following surgery adjuvant  treatment should be instituted in all tumours > 1 cm.  Presence of metastatic disease within axillary lymph nodes  remains the best single marker for prognosis.   ¢ What does it mean? If no node is detected in this procedure  in the axilla, axillary block dissection 1s not required. This  will be of great significance in early breast carcinomas  wherein lymph nodes arenot clinically palpable nor detected  by investigations such as ultrasound/CT scan of the axilla.   ¢ Indication: Early breast cancer with (T1 or T2 No) clinically  node negative axilla.    Peroperative is better and followed more commonly. Patent  blue (Isosulfan vital blue dye 2.5-7.5 ml) or ?”"Tc radioisotope  labelled albumin is used.   ¢ Hand held gamma camera. The node is biopsied and sent  for frozen section biopsy or imprint cytology.  Where exactly to detect the sentinel lymph node (SLN)?   ¢ A 24cm incision 1s made in between the pectoralis major  and lattissimus dorsi, after 5—7 minutes after the injection.    ¢ Ifsentinel lymph node (SLN) ts negative for malignant cells,  no axillary block dissection is required.    1. Seroma/lymph collection: (30 to 50%). In spite of  adequate drainage of the chest wall and axilla, drainage  occurs for about 5-10 days. Just patiently reassure the  patient.   2. Secondary infection: \t manifests as redness, discharge,  fever, etc. Appropriate antibiotics are necessary.   3. Flap necrosis: True mastectomy requires elevation of  both upper and lower flaps (almost skin thin). Thus it  predisposes to flap necrosis. This requires debridement,  antibiotics, suturing and rarely skin grafting also.   4. Haemorrhage: Not common. If infection is severe  especially in the axillary tissue, it is possible that the  blood vessels may get eroded and bleeding can occur.  This usually stops with pressure but may require ligation  of bleeders in the operating theatre.   5. Pain and numbness in the axilla, medial side of arm. It  is usually due to irritation of intercostobrachial nerve.  Generally subsides by a few days of time. They require  simple analgesics.   6. Shoulder dysfunction can occur especially when pectoral  muscles are injured or retracted resulting in haematoma  or when pectoralis muscles are removed. It improves  over a period of time. Incidence is about 8—10%.   7. Injury/thrombosis of axillary veins. It manifests as severe  pain in the hand and swelling. Treated by low molecular  weight heparin.   8. Injury to axillary vein, needs to be repaired by 5 or 6—0  prolene sutures.   9. Winging scapula is due to injury to long thoracic nerve  of Bell. The good anatomical knowledge is essential to  prevent this complication.    ¢ Following either MRM or BCS, the residual disease is  controlled by administering radiotherapy in a dose of 50  gray in 25 fractions.   ¢ In the absence of nodal involvement axilla may be spared  radiation.   ¢ If4or more nodes are involved, then the field of radiation  should include supraclavicular and internal mammary  chains.    POSTOPERATIVE RADIOTHERAPY  e Tumour margin is positive  e Pectoralis major is involved  e Inner quadrant tumour  e High grade tumours  e Axillary clearance not satisfactory  e Breast conservative surgery    e Tumour size more than 5 cm, 4 or more nodes are positive    1. It refers to use of radiation sources in or close to the    2. Breast is one of the organs which can be treated with    Hormonal therapy is administered to patient in whom the  tumour expressed steroid hormone receptors, i.e. ER/  PR.Those who are negative for ER/PR, will not benefit from    ¢ Raloxifene is an oral selective oestrogen receptor modulator  (SERM) that has oestrogenic actions on bone and anti-  oestrogen actions on the uterus and breast. It is used in the  prevention of osteoporosis in postmenopausal women.  Studies have proved that no specific advantage of raloxifene  in the adjuvant treatment of breast cancer rather than  established drugs such as tamoxifen.    2nd line—Aromatase inhibitors. They prevent synthesi  of endogenous oestrogens/steroids by blocking th  aromatase enzyme which converts androstenedione te  oestradiol in the adrenals. They are: Ist generation—  aminoglutethimide and 2nd generation—anastrozole  letrozole, exemestane given to postmenopausal patients.    Letrozole is costlier than tamoxifen, reduces oestroger  levels by 98%, and thus slows down oestrogen sensitive  breast cancers. It is given in a dose of 2.5 mg per day. Side  effects include vaginal bleeding, vaginal dryness, nigh  sweats, hot flushes, osteoporosis, etc. (Key Box 21.20).    4th line—androgens such as fluoxymesterone in the dose  of 30 mg daily 1s another drug (last 2 drugs are rarely given)    Should be considered in all cases of early breast cancel  irrespective of menopausal status, hormone receptor status  and nodal status.    Thus in premenopausal women—it can be used to  decrease production of oestrogen by ovary. However,  tamoxifen is better    In postmenopausal woman—oestrogen is produced in  peripheral tissues such as fat, liver and muscle    Hence, drug of choice in postmenopausal patients is an  aromatase inhibitor    Anastrazole, letrozole, exemextane are the drugs  Cardiac problems, osteoporosis are side-effects.   = Ist line agents: Cyclophosphamide, adriamycin,  5-fluorouracil (FAC). Because of cardiotoxicity of  adriamycin, epirubicin is preferred as in FEC regimen.  The preferred regime is anthracyclines (adriamycin) which  have a better response rate. Either CAF or FEC every 21  days x 6 cycles or at every 21 days x 4 cycles or just  adriamycin with cyclophosphamide (AC). However, the  CMF (cyclophosphamide, mitomycin, fluorouracil)  regimen Still continues to be used widely due to economical  reasons.    Trastuzumab: A monoclonal antibody against tyrosine kinase  receptor (HER-2 receptor) 1s administered in patients with  HER-2 +ve patients since it has been shown to improve disease  free survival (DFS) by 50%, when it is combined with taxane-  based chemotherapy. It potentiates effects of chemotherapy.    Treatment of early carcinoma of the breast is summarised in  Figs 21.82.    Many patients with breast carcinoma come to the hospital with  gross skin involvement, pectoral muscles involvement or chest  wall involvement. Even though the cure rates are low, with  proper planning, good control of the disease, good palliation in  majority of patients and ‘cure’ 1nasmall groupcan be achieved.  They do not have metastasis. Hence, grouped under locally  advanced breast carcinoma (T3 and T4). With neoadjuvant  chemotherapy (NACT), survival rates have improved (Figs  21.83 and 21.84).    e Any tumour more than 5 cm with or without skin and chest  wall involvement are included under LABC.   ¢ Under TNM, any stage IIB and stage III can be included  under LABC. Isolated supraclavicular metastasis 1s also  included in the stage III/LABC category.    A. Operable LOBC (large operable breast cancer) (IIB and  III A): Ulceration, limited skin oedema, fixation to the  pectoralis muscles and bulky axillary nodes are grave signs  but resection can be done.    Fig. 21.83: Carcinoma of the breast—See the puckering of the Fig. 21.86: LABC—tumour was fixed to skin with peau d’orange  skin, nipple retraction—Core biopsy or incision biopsy also can _ tumour was 8 cm in size (Courtesy: Dr Basavaraj Patil, Associate  be taken here. This case is not ideal for resection. Hence, Professor, Dept of Surgery, KMC, Manipal)   Fig. 21.84: Large lump displacing the nipple areola complex  downwards—core biopsy for receptor studies. MRM may not give  negative margin. Hence, this is an ideal case for neoadjuvant  chemotherapy followed by surgery (T4 N1 MO)   Fig. 21.87: LABC (Courtesy: Dr Satish Deshmukh and  Dr Murtaza Akhthar, NKP Salve Institute of Medical Sciences,  Nagpur)    B. Inoperable (IIIB): Extensive breast skin oedema, inter-  costal nodes, arm oedema, or inflammatory breast cancer.   ¢ Core biopsy tissue is also evaluated for ER, PR, HER  2/neu status, P53 overexpression, etc.   ¢ Biological markers important because if positive, NACT  1s Offered in the first place. Those who respond to NACT,  are subjected to mastectomy or BCS.   Fig. 21.85: LABC—Tumour was fixed to chest wall with fungating Sa  ulcer and oedema arm. (Courtesy: Dr Ankur Sharma, Assistant This modality is used for treated locally advanced breast cancer  Professor, Dept. of Surgery, KMC, Manipal) (LABC) wherein due to the advanced nature of the disease in    the breast and/or axilla, the surgeon 1s unable to operate upon  the patient initially. The exact TNM stage for defining LABC  remains controversial (II B versus II] A). However, any breast  cancer in which the operating surgeon feels that he will be  unable to completely resect the tumour in the breast and/or  axilla can be treated with neoadjuvant chemotherapies.   If the growth becomes impalpable clinically after finishing  ALL rounds of chemotherapy, it is called a Complete Clinical  Response (cCR). However, if the resected specimen shows  viable microscopic disease in a patient with cCR, it is termed  Partial Pathological Response (pPR). If no microscopic  growth is seen in resected specimen, then it is termed  Complete Pathological Response (cPR).   whether or not the growth has completely resolved, e.g.  4 cycles of AC followed by 4 cycles of paclitaxel  followed by surgery.   chemotherapy before surgery need not receive any more  chemotherapy in the adjuvant setting.   neoadjuvant chemotherapy before surgery should finish  their remaining cycles after surgery.   neoadjuvant setting till all courses of chemotherapies  are over and should be continued in the adjuvant setting  for a total duration of one year.  2. Neoadjuvant hormonal therapy  ¢ Postmenopausal patients who are not fit to receive systemic  cytotoxic therapies should undergo a trial of this modality.  ¢ A prior assessment of the ER/PR status should be done to  make sure that the tumour is hormone responsive. This  modality does not work in hormone negative tumours.  ¢ Tamoxifen/letrozole/anastrozole all can be used.    e Trucut or open biopsy is done to confirm the diagnosis  followed by identification of receptor status.   e Neoadjuvant chemotherapy is given first which will shrink  the tumour.   e Surgical resection is carried out to achieve a clear margin  (mastectomy or breast conservation surgery).   e f the tumour is fungating and the surgeon feels adequate  cancer clear margin can be achieved, toilet mastectomy  can be done first followed by chemoradiotherapy.    e Duration of the treatment should be up to the achievement  of maximal response.   e Patients not responding to the above mentioned treatment  may be given a trial of this modality.   e Neoadjuvant therapy apart from making inoperable  tumours operable also act as prognostic marker as in  patients who achieve cPR have a far better outcome of  the disease that those who do not.   ¢ Evenin LABC, if the response to treatment is favourable  breast conservation is possible, even in T4 tumours.   e ER/PR positive has the option of NAHT (Neoadjuvant  hormonal therapy).   e HER-2/neu positive responds to Trastuzumab with  taxanes, and response ts better (treatment is costly)    e Anthracycline (doxorubicin, epirubicin) + cyclopho-  sphamide (AC/EC) with or without addition of 5  fluorouracil is found to be superior to CMF regimen.   negative case is given below:  Doxorubicin 60 mg/m*—IV, and Cyclophosphamide 600  mg/m? IV day 1, cycled every 21 days for 4 cycles followed  by Paclitaxel 175 mg/m? by 3 hour IV injection day/cycled  every 21 days for 4 cycles.   ¢ Increased rates of wound dehiscience, wound infection,  seromas—when associated with preoperative radiotherapy    ¢ Breast cancer is a systemic disease at the onset. Even at  early stages, patient may harbour micrometastasis at some  sites, which may remain dormant for years together or may  get treated with adjuvant therapy. Metastatic breast cancer  (MBC) may be seen in almost all organs but some organ  involvement is seen more frequently than others. Soft tissue  recurrence, bone, liver and lung metastasis 1s seen more  commonly. Metastasis to brain, pleura and pericardium,  altogether less common, may have devastating  consequences and require specific treatment.   ¢ Aim of the treatment is to give palliation in the form of  treatment of symptoms.   ¢ As these patients have advanced disease/disseminated  disease, chemotherapy is given first followed by excision  of lump/simple mastectomy to get rid of fungating tumour.  However, if the lump disappears totally, there is no  indication for excision of the lump or mastectomy.    ¢ Following chemotherapy, tamoxifen is given for a period  of five years. Repeated courses of second and third line  chemotherapeutic drugs may have to be given.    ¢ The treatment of individual organ involvement or system  involvement is given below.    Patients with oestrogen receptor +ve status are more likely to  have a bony recurrence, whereas ER —ve tumours tend to  recur in liver and brain. After diagnosis of MBC, patients live  an average of 16~—30 months.    In general, hormone treatment is better tolerated as it is  associated with fewer side effects. Hence, it is preferred in  patients who have steroid receptor positive status and/or  asymptomatic visceral metastasis.    ¢ Patients who have overexpression of HER-2 receptor, may  also receive trastuzumab in addition to the above.    ¢ Once effusion is confirmed by aspiration and cytology, an  intercostal drain is left in place for a few days. Once the  drainage is nil or completely dry, talc insufflation is done,  to achieve pleurodesis. Talc is the most effective agent,  followed by tetracycline or bleomycin, etc. In selected cases,  effective pleurodesis has given an asymptomatic period up  to 1—2 years.    ¢ Patients present with features of raised intracranial pres-  sure such as headache, nausea, vomiting and papilloedema.    ¢ Eventually 60—70% of patients with carcinoma breast  develop bone metastasis. Bone metastasis produces  intractable pain, pathological fractures, quadriplegia and  paraplegia, etc. (Figs 21.88 and 21.89 and Key Box 21.22).   ¢ Localised bone lesions are treated by palliative radiotherapy  or decompression as in quadriplegia, etc.   ¢ Bisphosphonates, e.g. oral clodronates have been found  to arrest progression of bone disease, given at the dose of  1,600 mg/day (Key Box 21.23).   ¢ Cancer cachexia.  Management of metastatic breast cancer is summarised in  Fig, 21.90.    e Important drugs in addition to radiotherapy to treat bone  metastasis in malignancies.   e |V—pamidronate or zoledronic acid 4 mg IV monthly for 10  courses are used   Fig. 21.89: Whole body bone scan showing disseminated bony  metastasis—*Y"Tc-labelled phosphate compounds are agents of  choice for detection of osseous metastasis    See Fig. 21.90 for flow chart of management of metastatic  breast cancer    areolar lymphatic obstruction and fixation of hair follicle  to the underlying malignancy.   ¢ It is a complication of radical mastectomy when all the  nodes in the axilla are removed, especially when  postoperative radiotherapy is given in the axilla.   ¢ It occurs due to destruction of all lymphatics and lymph  nodes.   ¢ This occurs in inoperable carcinoma of breast with fixed  nodes in the axilla.   e In this, the entire chest wall is studded with cancerous  nodules which are hard and fixed to the skin and to the  chest wall. The condition has been compared to an  armour used by the soldier. It indicates advanced  carcinoma of breast. Palliative treatment 1s given in the  form of radiotherapy or chemotherapy and analgesics  to relieve the pain.   Fig. 21.94: Extensive lymphoedema and lymphangitis due to car-  cinoma axillary tail of the breast    : —_" , : = |  Fig. 21.92: Postmastectomy lymphoedema (Courtesy: Dr  Sreejayan, Professor of Surgery, Calicut Medical College, Calicut)    ¢ The ideal candidate for breast reconstruction is a patient  who has undergone modified radical mastectomy.    COMPONENTS OF BREAST RECONSTRUCTION  e Chest wall reconstruction: This can be achieved by using    * Improves self confidence latissimus dorsi musculocutaneous flap or contralateral  ¢ Better social life transversus abdominis muscle (TRAM) flap.  * Decreases concern about cancer ¢ Creation of a mould: A silicone gel implant is inserted under  ¢ Better sexual life pectoralis major muscle (subpectoral pocket).  * Feel “whole again” e Reconstruction of nipple and areola: This can be achieved  Timing: Immediate or delayed by skin taken from inner thigh, labia minora or from opposite  ¢ Immediate reconstruction: Should be done if no breast (nipple sharing). However, this reconstruction is done  contraindications. It has proven psychological benefits and 4—6 weeks later, once the implant settles down.  patient satisfaction, it is cost effective, it does not delay ¢ Symmetry with the opposite breast: It is achieved by doing  adjuvant treatment and recurrence detection. reduction mammoplasty of the other breast.    1. Pedicled flaps e Anterolateral thigh flaps  e Latissimus dorsi myocutaneous flap (with implant) 3. Silicon compound gel/saline  ¢ TRAM flap ¢ Expandable    Fig. 21.98: TRAM flap is raised—flap design: Zone I-IV Fig. 21.99: TRAM flap is shown with its blood supply    (Courtesy: Dr Bhaskar KG, Senior Consultant, Plastic Surgeon, Medical Trust Hospital, Cochin, Kerala (next page photos also)    Transversely oriented rectus abdominis myocutaneous flap  popularly known as TRAM flap is the most common re-  constructive option in postmastectomy breast recon-  struction. This flap can be pedicled based on the superior  pedicle or based on inferior pedicle. It can be used for breast  reconstruction as a free flap using microvascular technique.   Superior epigastric artery is the artery supplying the flap.  If it is used as a pedicled flap for breast reconstruction, the  contralateral side is used for reconstruction.    If TRAM flap is taken with inferior pedicle using  microvascular technique, deep inferior epigastric artery forms  the main vascular basis of the flap.    Indications: Breast or skin reconstruction, post mastectomy  or wide excision of large chest wall   2. Very large breast in patient who does not desire reduction  3. Planned postoperative radiation therapy   Vasculature/Blood supply: Thoracodorsal pedicle  Procedure: Skin island of about 5—6 cm wide, outlined  transversely. Divide the paraspinal origins of the muscle  preserving the pedicle. Rotated on its humeral insertion  towards the anterior chest wall beneath a bridge of thoracic  skin below the axilla. Breast implant can be placed in a  submuscular pocket.    Advantages:  1. Proximity to the breast  2. Reliable/robust circulation (rich blood supply)    Disadvantages:  1. Large scar on the back.  2. Cannot be used if there is an injury to thoracodorsal pedicle.    Constitutes less than 1% of all cancer in men. Minimum age  is around 65 years   Male breast cancers tend to be more advanced at the time of  presentation because of less subcutaneous fat. More than 90%  cases are infiltrating duct carcinoma, about 10% are ductal  carcinoma in situ (DCIS).   Fig. 21.109: Male breast carcinoma in pre-existing gynaecomastia.  You can see destruction of the nipple and infiltration into the skin:  Commorly it is infiltrating ductal carcinoma    ¢ Lump in the breast which is hard and irregular.  ¢ Nipple retraction   ¢ 80% of these cases are ER positive, hence role for tamoxifen   ¢ Goserelin 3.6 mg/28 days for 2 years—it is a LHRH agonist.  It causes reversible chemical castration.    ¢ BRCA 1: 65 to 85% (long arm of chromosome 17)  ¢ BRCA 2: 40-85% (long arm of chromosome 13)    Fig. 21.110: Male breast carcinoma—tends to ulcerate and infil-  trate chest wall early (Courtesy: Dr Surya Prakash Saxena, Dis-  trict Surgeon, Durg, Chhattisgarh)    ¢ BRCA mutations: Highest incidence in Ashkenazi Jews  ¢ High risk of ovarian cancer    Aim  ¢ To decrease the risk of developing invasive cancer  ¢ Also to decrease the risk of dying.    2 specific genes—BRCA 1 and BRCA 2 are responsible for  5% of all breast cancers.    ¢ BRCA | associated cancers are invasive, high grade, ER,  PR and HER-2/neu negative. Hence, more aggressive and  carry poor prognosis.    Indications (Pennisi, 1986)  1. Biopsy proved  e Noninvasive intraductal carcinoma in situ  ¢ A single focus of lobular carcinoma in situ  e Florid cystic disease  2. A mammogram that is suspicious of moderate to severe  mammary dysplasia    3. Persistant breast nodules that do not vary with menstrual  cycles and are of concern to the patient and physician  4. Familial or hereditary breast carcinoma.   ¢ Skin flap along with even thickness of subcutaneous fat  which is retained under the skin, is raised (helps in  retaining vascularity of the flap). Haemostasis is  obtained. Immediate reconstruction or reconstruction at  a later date can be done.   ¢ Remove entire breast including axillary tail. Flaps  should be thin. Axillary block dissection is not necessary.    Mondor’s disease is spontaneous thrombophlebitis of the  superficial veins of the breast and anterior chest wall. The  common causes of thrombophlebitis such as injury or infection  are not found in these cases.   |. Indurated subcutaneous thrombophlebitic cord about 3 mm  diameter of varying length is situated in the subcutaneous  plane with an attachment to the skin. Consistency is like  that of vas deferens.   2. When the skin over the breast is stretched by raising the  arm, a narrow, shallow subcutaneous groove along the side  of the cord becomes apparent.    ¢ Restricted arm movements (otherwise it is very painful).  Spontaneous recovery is expected within a few days.   ¢ Clinically it presents as rapidly growing lump in the breast  with infiltration of the skin producing ulceration.   ¢ On careful observation, the edges are not everted and the  lesion is very vascular.   ¢ It is difficult to make a clinical diagnosis of angiosarcoma  of the breast (Fig. 21.111). However, with careful examina-  tion it is possible to suspect because of increased vascularity  of the tumour and the features mentioned above.   ¢ Confirmation of the diagnosis is by FNAC or by biopsy  from the lump. Chest X-ray and CT scan of the chest are  other helpful investigations.   ¢ Implant used is outer silicone shell filled with silicon gel,  saline or a combiantion of both   A. Subpectoral: More often this is the side. Even if a cancer  develops in this breast, mammography can detect early  lesion. After mastectomy—if recurrence develops, easy to  detect if it is subpectoral   B. Subcutaneous: Not many advantages: Easy to keep and  easy to remove. It interferes with detection of malignancy.  Easy to rupture.    1. Squamous cell (epidermoid) carcinoma  ¢ Rare cancer from metaplasia within duct system and  devoid of distinctive clinical/radiological characteristics.  ¢ Regional metastasis occurs in 25% of patients  ¢ Distant metastasis is rare  2. Adenoid cystic carcinoma  ¢ Rare, less than 0.1%.  ¢ Well circumscribed and usually 1-3 mm in diameter.  ¢ Axillary lymph node metastasis are rare  ¢ Death usually occurs from pulmonary metastasis.  3. Apocrine carcinoma  ¢ Wellcircumscribedcancers have rounded vesicular nuclei  and prominent nucleoll.  ¢ Usually aggressive.   ¢ Axilla is addressed only if palpable lymph nodes are  present   a. Women < 39 years—bilateral, associated with Burkitt’s  lymphoma. Axillary dissection is done for staging and  clearance of the disease. Recurrence is treated with CT/  RT    A 59-year-old post-menopausal lady complains of lump in the  right breastof 3 months duration. Lump was hard in consistency.  Sonomammogram revealed ill-defined hypoechoic lesion with  spiculations seen in the superolateral quadrant of the breast.  FNAC was suggestive of malignancy. Whole body bone scan  after 3 hours IV inj. of 20 mCi of Tc-99 m MDP showed few  osteoblastic lesions—so degenerative changes?  Treatment: She underwent local wide excision followed by  axillary block dissection. Final report was tubular carcinoma—  30% arising in a background of proliferative breast disease  (70%). All margins were free. Lymph nodes were free from  metastasis  Immunohistochemistry: Tumour cells are ER positive PR  positive Quick score 2. Her-2/neu 1+ Ki 67: 12%—Hormone  responsive  Next plan: External beam radiotherapy of 60 Gy in 30 fractions  over 6 weeks, including boost to the lumpectomy side.  ¢ She also received tablet letrozole 2.5 mg HS and 6 monthly   Based on DNA microarrays, breast cancer can be divided  into specific groups referred to as luminals depending on which  the choice of the treatment can be given. However, since these  tests can be very expensive, IHC results (ER/PR/HER/Ki 67)  can be used as surrogate markers to define various categories  of breast cancer.    HER-2 negative  Ki 67 ‘low’ (i.e. <14%)  Recurrence risk ‘low’ based on multi-gene-expressior  assay (Oncotype DX) 1.e. < 25 score  2. Luminal B  ‘Luminal B-like (HER-2 negative)’  ER positive  HER-2 negative and at least one of:  Ki 67 ‘high’  PR ‘negative or low’ 1.e. score less than or equal to 19%  Recurrence risk ‘high’ based on multi-gene-expression  assay , 1.e. > 25 score  ‘Luminal B-like (HER-2 positive)’  ER positive  HER-2 over-expressed or amplified  Any Ki 67  Any PR  3. Non-luminal ( Erb B2 over-expression)  HER-2 over-expressed or amplified  ER and PR absent  4. Basal-like-triple negative (ductal)  ER and PR absent  HER-2 negative.    1. Anthracycline based chemotherapies (e.g. doxorubicin)  should be preferred over the first generation chemotherapy  regimens like CMF.    2. Taxols should be added sequentially to anthracycline based  agents especially when the tumour is HER-2 positive and/  or more than 4 lymph nodes are positive or triple negative  disease.   3. 6-8 cycles should be the number of cycles given to the  patient.    One example would be 4 cycles of adriamycin (doxorubicin)  + cyclophosphamide given 3 weekly followed by 4 cycles  of single agent paclitaxel given 3 weekly.    4. Duration of trastuzumab should be | year. It should not be  given along with anthracycline based chemotherapy but can  be given along with taxols.    5. Dose dense chemotherapies are the ones where the overall  duration of chemotherapy cycles is reduced, e.g. instead of  conventional three weekly gap between two cycles, the  gap is reduced to 2 weeks. These are typically used in  aggressive breast cancers like triple negative breast cancers.    1. Tamoxifen can be used in both pre- and post-menopausal  ladies, the total treatment duration is now recommended  to be 10 years (earlier it was 5 years), especially when  the chances of recurrence is high.   Luminal B (HER-2 positive) Adjuvant chemotherapy + anti HER-2  (trastuzumab) + hormonal therapy    2. Aromatase inhibitors are typically used in post-meno-  pausal ladies and should not be used in pre-menopausal  females unless it is given along with ovarian suppression  agents like Goserelin.    3. Other agents like Fulvestrant should only be used in the  second line setting.    e SERMs: Selective estrogen receptor modulators  (agonistic in some tissues while antagonist in others)    ¢ Example: Tamoxifen is agonist on bone and uterus but  antagonist in breast. Other example: Raloxifen    1. Trastuzumab—monoclonal antibody against HER-2/neu  receptor on the breast cancer cell surface.    6. Denosumab—a RANK ligand inhibitor used for palliation  of bone metastasis.   1. High recurrence score on oncotype DX, 1.e. > 25  2. High score on MammaPrint   4. Involvement of 4 or more axillary lymph nodes.  5. Age < 35 years    Trastuzumab should be given in any tumour size greater  than 5 millimeters   Most effective drug used to treat cyclical mastalgia is  evening primrose oil.   Most breast cancers arise in ductal epithelium (90%)  Most breast cancers occur in upper outer quadrant (60%)  Most common presentation of carcinoma breast is with a  lump   Most common type of carcinoma breast is scirrhous  carcinoma (60 to 70%).   Most common reconstructive option in postmastectomy  breast reconstruction is TRAM flap.   e The understanding of pathology and treatment of breast  abscess has been updated.   e Prophylactic mastectomy, sentinal node biopsy, flap  breast reconstruction, has been edited   A. It is the best modality for women with breast implants   D.It can distinguish scar from recurrence 1n women who  have undergone breast conservative surgery   C. Extension of the growth along lactiferous duct causes  retraction of the nipple   B. Majority of the cases are due to anaerobic infection  C. Repeated aspiration is recommended treatment    . Smoking is associated with which of the following  breast disease?  A. Tuberculosis  C. Duct ectasia    The widely used first investigation of choice in a lady  of 25 years with lump breast is:    . Which one of the following is not the treatment for  mastalgia?    . Which one of the following is the treatment of choice    for early breast cancer in a 30-year-old lady who is ¢  months pregnant?    . Which is the drug used in a patient to prevent breast    . Which one of this operative step is not done in   A. Excision of tumour plus a rim of | cm of normal breast  tissue   C. Excision with or without radiotherapy is the treatment  of choice   C. Excision with or without radiotherapy is the treatment  of choice    Following are true for sentinel node biopsy in  carcinoma breast except:    ideal  ANSWERS  1 G 2¢ 3 D 4 6B ee, 6 D  11 B 12 C 12s G 14 D 15 A 16 B  21 D 22 D 23 D 24 D 25. B    Following is an indication for radiotherapy to chest  wall in carcinoma breast except:   D.It is not recommended in patients who have family  history of carcinoma breast but unlikely carriers of  breast cancer gene    Which one of the following is not true in male breast  cancer?   36. Blunt Abdominal Trauma, War and Blast Injuries and Triage  37. Abdominal Mass    It is a jagged line where the oesophageal mucosa changes  to gastric mucosa.   Collar of Helvitius: It is the site at which the circular  muscles of oesophagus turns to oblique muscles of the  stomach at the incisura.   It runs in the posterior mediastinum as a continuation of  pharynx. 2 cm of this tube lies below the diaphragm.    Fig. 22.1: Division of oesophagus into 4 parts based on distance from incisor teeth    e It has an inner circular layer and an outer longitudinal layer.  ¢ Upper 1/3rd has striated muscle fibres.  ¢ Lower 2/3rd has smooth muscle fibres.    Clinically significant motility disorders affect lower  two-thirds (smooth muscle) of oesophagus.    e The entire oesophagus is lined by squamous epithelium  except last 3 cm which ts lined by columnar cells. The  columnar cells are similar to gastric mucosa but oxyntic  and peptic cells are absent.    e Upper oesophagus drains into the left and right supra-  clavicular nodes.   e Middle oesophagus drains into the tracheobronchial nodes  and paraoesophageal nodes.   ¢ Lower oesophagus drains into lymph nodes along the lesser  curvature of stomach and then into coeliac nodes. Involve-  ment of coeliac nodes indicate inoperability.    The parasympathetic nerve supply is by vagus nerve through  extrinsic and intrinsic plexuses. The intrinsic plexus has no  Meissner’s network and Auerbach’s plexus 1s present only in  the lower two-thirds.   Fig. 22.2: You can see  the multilayered structure.  Endosonography detects |  all these layers   e Extensive lymphatic plexus in the submucosa which  explains the easy spread   e Like in the colon they are classified as perioesophageal,  paraoesophageal and lateral oesophageal lymph nodes   e Thus throughout the entire length of oesophagus various  groups of lymph nodes are enlarged such as deep cervical,  mediastinal, subcranial, hilar, diaphragmatic, para and peri-  oesophageal, gastric, coeliac, etc.   e Longitudinal lymphatics are 6 times more than transverse.  Hence, distant spread occurs fast—satellite nodules occur  proximal and distal to the growth. This is the reason why  10 cm margin must be given in resection of the oesophagus   e One of the advantages of transhiatal resection over Ivor  Lewis operation (see page 449)   ¢ Cervical oesophagus: Mainly from branches of the inferior  thyroid artery.   ¢ Upper thoracic oesophagus: Mainly from branches of the  inferior thyroid artery and less consistently from anterior  oesophagotracheal branch from aorta.    ¢ Lower oesophagus: Small branches of the left gastric artery.  Rich internal arterial anastomosis is present in the    Surgical anatomy Significance Surgical points of interest  1. Upper oesophageal sphincter Dense cricopharyngeus muscle Zenker's diverticulum through Killian triangle  2. Lower oesophageal sphincter High pressure zone at gastro-oesophageal Weakness causes reflux oesophagitis  junction  3. Oesophageal mucosa Toughest coat of oesophagus Important in oesophageal anastomosis  4. No serosa, no mesentery Requires mobilisation Leak rate is high, easy spread of carcinoma  5. Helicoidal muscle Helps in peristalsis but it recoils due to Hence, once resection takes place—specimen  elasticity ‘shortens’ or retracts—anastomosis may become  difficult especially in abdominal anastomosis  6. Segmental arterial supply Extensive mobilisation can be done without Hence, transhiatal esophagectomy (THE) is done  compromising blood supply with ease  7. Lower end of oesophagus—veins Rich intercommunicating veins between portal Oesophageal varices occur here  and systemic veins  8. Azygos vein crossing oesophagus Can get injured and bleed Resection of midoesophageal tumours  in thorax  9. Mucosal and submucosal lymphatics Oesophageal tumours extend over a long Need for total oesophagectomy and cervical  out number capillaries distance within oesophageal wall anastomosis + lymph node clearance    Veins accompany corresponding arteries. Importantly, thoracic  oesophagus drains into azygos and hemiazygos veins.  e Table 22.2    The main function of the oesophagus is to transfer food from pharyngeal constrictors    the mouth to the stomach. Voluntary contraction of the  oropharynx pushes food into the upper oesophagus through a Pressure rises to 60 mmHg |  relaxed cricopharyngeal sphincter (Key Box 22.2). Then, due :  to primary and secondary peristalsis, the food bolus is  transferred to the stomach.   e Food stimulates mechanoreceptors  e Soft palate is pulled upwards so that food will not enter and vagal co-ordination takes place   epiglottis so that food will not enter trachea ‘Opening of lower oesophageal sphincter (relaxation)  ¢ Cricopharyngeal sphincter opens es  e Muscular wall of the pharynx then contracts from above   downwards. Fig. 22.6: Mechanism of swallowing—2 phases  ¢ Cricopharyngeal sphincter is closed at rest. It helps in long with a resting pressure of 20-25 mmHg. Hence, it is  preventing regurgitation of oesophageal contents into the known as high pressure zone (HPZ).  respiratory passage. ¢ This HPZ prevents reflux of gastric contents into the  ¢ Lower oesophageal sphincter (LOS): It is a physiological oesophagus. Cigarette smoking affects this zone and so,    sphincter at the gastro-oesophageal junction. It is 3-4 cm smokers have a high incidence of reflux oesophagitis.   ¢ Loss of competence of LOS leads to gastro-oesophageal  reflux disease (GORD). The competence of LOS can be  affected by obesity, smoking, excessive eating, etc. Sliding  hernia is associated with GORD.   ¢ Asaresult of reflux of gastric acid, extensive inflammation  of the lower oesophagus occurs which results in various forms  of oesophagitis.   2. Chronic: It is associated with hiatus hernia or following  oesophagojejunostomy.   3. Ineffective oesophageal pump affecting clearance of food  into stomach. This is influenced by following factors:  ¢ Gravity  ¢ Oesophageal motility  ¢ Salivation—Saliva neutralises the minute amount of acid   that is left following a peristaltic wave. When salivary  flow is decreased (following radiotherapy), the reflux can  get exaggerated.   4. Increased gastric pressure: Can occur due to pyloric  stenosis and diabetic gastroparesis. Following vagotomy,  loss of receptive relaxation occurs resulting in increased  intragastric pressure.    ¢ Acid refluxes into the lower oesophagus and produces  diffuse inflammation with multiple ulcers.    ¢ Due to vagal hyperactivity, inflammation and ulcers develop  which produce severe longitudinal muscle spasm.  Consequently, the cardia is drawn up into the thorax, leading  to an increase in the oesophago-cardiac angle. This increases  the reflux. Later, fibrosis causes shortening of the  oesophagus.    Most common presentation of GORD is history of heart burn  and regurgitation. Heartburn is confined to epigastrium and  retrosternal areas, does not radiate to the back. Clinical  presentation of the GORD can be classified as follows: Atypical  symptoms can be so many and can thus confuse the picture.    Oesophageal : Dysphagia, regurgitation, heartburn  Gastric : Early satiety, belching, bloating, nausea  Pulmonary : Asthma, wheezing, aspiration, cough,    dyspnoea, bronchitis, hoarseness of voice  due to damage to vocal cords, etc.    What is globus? Sensation of a substernal lump (globus)  When this occurs during fasting, it is termed ‘globus hystericus’.  It is a neurotic symptom in patients with emotional instability.  ¢ Retrosternal pain: It is burning in nature and becomes   position. The pain is described as heart burn and can be  confused for angina pectoris. \t is relieved on taking  antacids.   * Occult blood in stools and streaks of blood in the vomitus  are common.   ¢ Dysphagia: Transient difficulty in swallowing results from  spasm due to inflammation of the lower end of oesophagus.    Indications: When symptoms are certain but endoscopy is  normal, atypical symptoms   It involves the transnasal placement of a pH measuring  electrode in the lower oesophagus. The pH electrode  monitors the changes in intra-oesophageal pH over 24 h  A 24h pH profile is thus obtained that provides information  on frequency, duration and pattern of reflux    Barium swallow! in the Trendelenburg’s position (head  down position) can demonstrate the reverse flow of barium  into the lower end of the oesophagus (from the stomach).  Oesophagoscopy may reveal red, angry looking mucosa  in the lower end of the oesophagus.   Oesophageal manometry to detect motility disorders.  24-hour pH monitoring is the gold standard.    Manometry is essential. To place the pH probe 5 cm above  upper border of LOS for ambulatory pH monitoring.    e Areflux episode is defined as a pH drop to below pH 4  e Thus identification, type and duration of reflux is noted    Medical management  Treatment of uncomplicated GORD can be discussed under  following headings.   Grade I: Small, circular, nonconfluent erosions  Grade II: Linear erosions lined with granulation  tissue that bleeds on touch    Antacids with alginate—antireflux floating alginate.  Proton pump inhibitors: Pantoprazole 40 mg,  esomeprazole till 20 mg may have to be given for one  or two months or full symptoms are controlled. These  are antisecretory drugs.   Prokinetics: Itopride 50 mg can be given 2-3 times a  day for 8 weeks on empty stomach. Prokinetics enhance  motility. Cisapride and mosapride are not favoured  because they can cause cardiac arrhythmias.   Sucralfate colloidal bismuth---cytoprotective agent.  It is a sucrose sulfate—aluminium complex which binds  to the mucosa. Thus it protects mucosa of GI tract  against hydrochloric acid.    ' Barium swallow: Thick paste to see the oesophagus. Barium meal: A thin paste to see the stomach. Both are radio-opaque.    IV: Endotherapy  ¢ Endoscopic plication/suturing  e Enteryx injection  ¢ Plexiglass microspheres (PMMA): Through an  endoscopic needle, microspheres suspended in gelatin  are injected. Gelatin is absorbed and spheres cause  tissue bulking.    e Alcohol to be minimised  e Lose weight  ¢ Coffee/tea to be minimised    To summarise, quit smoking, decrease alcohol intake, avoid  overweight and start walking. Do not drink coke and do not eat  chocolates, take mucosa protective agents (Key Box 22.5).  ]. Intractable pain  2. Complications such as haemorrhage or stricture  ¢ The results of antireflux surgery are good with a small  mortality rate (0.1 to 0.5%). However, careful selection  of patients depending upon their symptoms and lifestyle  are important factors.    1. Nissen’s total fundoplication (Figs 22.7A and B): The  aim is to restore 2-4 cm of intra-abdominal oesophagus by  reducing the hernia, followed by repair of the hiatus.  ¢ In this operation, fundus of the stomach is mobilised by  dividing short gastric arteries.    ¢ Fundus is brought behind the oesophagus and wrapped in  front of oesophagus (Fig. 22.8). It is a loose wrap (Floppy  Nissen’s).   ¢ Diaphragmatic defect is repaired by using nonabsorbable  sutures such as nylon or silk. This is the operation which  serves all aims.    ¢ 360° gastric fundoplication should be no longer than 2 cm.   ¢ Vagus should not be damaged because it may result in  failure of sphincter to relax.   ¢ Lengthen the oesophagus with a Collis gastroplasty (in  cases of short oesophagus).   ¢ Patients with normal peristaltic contractions do well with  360° Nissen’s fundoplication—for others, two-thirds partial  fundoplication may be the procedure of choice.    ¢ Too tight a plication may result in dysphagia or gas bloat  syndrome wherein belching ts prevented.   2. Partial fundoplication (Toupet) solves the above problem  wherein fundus is sutured around the back of oesophagus  (Fig. 22.9) or Dorr’s, where fundus is sutured anterior to  the oesophagus.   e There are 3 layers of sutures to be placed in this operation  via a thoracotomy (Key Box 22.6).   e First row: Four interrupted silk mattress sutures are placed  between the anterior surface of the oesophagus and adjacent  fundus of the stomach so as to wrap the stomach around  the anterior two-thirds of the oesophagus.   ¢ Second row: Sutures are placed between oesophago-gastric  junction and under surface of the diaphragm to maintain  the junction below the diaphragm.   ¢ Third row: Posterior crural sutures are placed to tighten  the opening.   ADVANTAGES OF BELSEY MARK IV  e Reduces size of the ring  e Creates a valve at the oesophagogastric junction in order  to prevent reflux  e Recurrence is low   e Inthis, the long intra-abdominal segment of the oesophagus  is firmly fixed below the diaphragm by anchoring the gastro-  oesophageal junction to the crura just above the median  arcuate ligament. This is described as posterior  gastropexy.   5. Recurrent GORD: Success of revision surgery is much  less than primary surgery. Partial gastrectomy with Roux-  en-y reconstruction is the final surgery for GORD. It diverts  bile and pancreatic juice and reduces acid secretion.    Definition  Abnormal protrusion of abdominal viscus through the  oesophageal hiatus into the chest.    1. Sliding hernia or oesophagogastric hernia: It is the  commonest type of hiatus hernia, accounting for about 80%  of cases (Fig. 22.11). It may be associated with GORD.    1. Symptoms due to reflux: Regurgitation and heart burn are  the two most common symptoms.   2. Symptoms due to complications: They are dysphagia,  odynophagia, haematemesis and melaena.    1. Presence of 2 cm of intra-abdominal oesophagus.  2. The angle of His: The oesophago-cardiac angle of about  45° has valvular effect.  . Mucosal folds at the oesophago-cardiac junction.  4. Positive intra-abdominal pressure which closes the  abdominal oesophagus.   5. Lower oesophageal sphincter (LOS): It is a functional  sphincter which increases the pressure during coughing,  straining, etc.    1. Deposition of fatty tissue in the hiatus leads to weakening  of the hiatus in obese individuals.   2. Advancing age resulting in muscular degeneration can  predispose to hernia.   3. Raised intra-abdominal pressure due to lower abdominal  tumours, pregnancy, etc.   4. Saint’s triad: Gall stones, diverticulosis and hiatus hernia  can occur together in a patient.    ¢ Sliding hernia produces symptoms like reflux oesophagitis.  ¢ More common in women, especially if obese.    ¢ Oesophagoscopy reveals varying degree of inflammation.  During oesophagoscopy, when the patient is asked to strain  (Valsalva’s manoeuvre), the sphincter 1s seen to be more  patulous and herniation of gastric mucosal folds can be seen.  Reflux of the gastric acid is the most valuable sign.    In all cases of GORD, conservative treatment has to be tried  first. The results of surgery are appreciated only when the  symptoms are significant and conservative treatment fails.    ¢ Diet control with increased intake of proteins anc  decreased consumption of fat and sugar.  ¢ Decreased alcohol and tobacco consumption.    In this condition, cardio-oesophageal junction 1s normal.  Greater curvature of the stomach ascends into a preformed  sac in the mediastinum (Fig. 22.12). Thus, there are no features  of reflux oesophagitis but the sac containing stomach in the  thorax causes compression of the heart and lung.   ¢ Feeling of fullness after a meal or dysphagia due to large sac   ¢ Respiratory tract infection and hiccough due to irritation of  phrenic nerve.    Barium meal shows the sac in the thorax containing stomach.  Sometimes, it can be upside down.    'Causes can be remembered as FATTY (F: fat, A: age, T: tumour, T: triad (Saint’s triad), Y: yielding hiatus.)    Reduction of the sac and repair of the hiatus by using gastroplasty by using stomach (short oesophagus).    rolling hernia. oesophagus or when it shows intestinal metaplasia, it is  described as Barrett’s oesophagus (Figs 22.14 to 22.17).  Complications of GORD ¢ Ulcer in the Barrett’s CLO is called Barrett’s ulcer.    develop in the lower end of the oesophagus. Early diagnosis junction upwards, which further increases the reflux resulting    ¢ Surgery is indicated in refractory cases of dilatation, in  the form of gastroplasty.    1. Gastric type with chief and parietal cells.  . Intestinal type with goblet cells is a marker of intestinal  metaplasia. This mucosa is smooth (unlike gastric folds).  3. Junctional type: It has mucus glands and resembles  gastric cardia.   Sliding  Clinical types  GE junction is — Rolling 1. Long segment: Metaplastic changes involving more than  in the thorax 30cm.  - 2. Short segment: Metaplastic changes involving less than  Diaphragm < ere  Incidence of malignancy  40 times more prone to carcinoma of the lower and middle  oesophagus as compared to the general population.  Types of dysplasia  Fig. 22.13: Mixed hernia Low grade: Negligible risk of carcinoma   RISK FACTORS FOR CARCINOMA  « CLO>8cm  e Smoking  e Reflux due to previous gastric surgery  e High-grade dysplasia—indications for oesophagectomy   ¢ It is important to screen these patients regularly with  repeat endoscopies and multiple biopsies to find  dysplasia (Key Box 22.9).    1. Corrosive: Lye, acid, alkali Burns, stricture  2. Infective: Candida, Herpesvirus Chronic illness, AIDS  3. Radiation: 20-40 Gy units to Ulceration and stricture    are summarised in Table 22.4. Basic treatment is similar t  that of reflux oesophagitis and avoid the causative agents.    he MOTILITY DISORDERS OF THE  CARCINOMA IN BARRETT’S OESOPHAGUS PHARYNX AND OESOPHAGUS    ¢ Asymptomatic, symptomatic Barrett’s oesophagus responds  well to laparoscopic antireflux surgery. Antireflux surgery  also prevents progression to high-grade dysplasia or  adenocarcinoma.    Antireflux surgery may protect mucosa against  development of dysplasia in patients with Barrett’s  oesophagus.    2. Oesophageal stricture: Usually located in the middle  or upper oesophagus.  * Peptic stricture occurs in the distal oesophagus.    This 1s also a condition which occurs due to several factors  such as corrosives, drugs, chemoradiation, AIDS, etc. They    It is a precancerous condition 1n which there Is a severe spasrr  of circular muscle fibres at the cricopharyngeal sphincter leve  or pharyngo-oesophageal junction, and it 1s associated wit  development of postcricoid web.   ¢ Asa result of the spasm and web, dysplasia occurs and  leads to features of anaemia later. The proximal mucosa 1s  constantly irritated due to stasis. It undergoes hypertrophy,  hyperkeratosis and desquamation. This, over a period of  years, predisposes to carcinoma oesophagus (carcinoma  oropharynx).   e Features of anaemia—pallor, stomatitis, ulcerations, bald  tongue (without papillae), koilonychia, splenomegaly and  microcytic hypochromic anaemia.   ¢ As aresult of obstruction, the fluid tends to spill over into  the larynx giving rise to recurrent aspiration, respiratory  tract infection, cyanosis or choking.   PLUMMER-VINSON SYNDROME  e Women  e Dysphagia, anaemia  e Cricopharyngeal spasm  e Dilatation  e tron supplements  e Precancerous condition   ¢ Improving anaemia: Iron tablets or blood transfusion and  correction of nutritional deficiencies.   e It is also called cardiospasm because of severe spasm of  the circular muscle fibres of the lower end of the  oesophagus. The contracted segment does not relax during  the act of swallowing (achalasia = failure of relaxation). As  a result of this, there is dilatation, tortuosity and hypertrophy  of the oesophagus above.    1. Idiopathic: This occurs due to absence or degeneration of  Auerbach’s plexus throughout the body of the oesophagus  leading to improper integration of the parasympathetic  impulse. This is called primary achalasia.   3. Stress and emotional factors and vitamin deficiencies are  also associated with this disease.    e Classical achalasia: Loss of ganglion cells and neural fibrosis  occurs.   ¢ Vigorous achalasia: No loss of ganglion cells but ganglionitis  Occurs.    ¢ Women around 30-40 years of age are commonly affected.  The ratio of affected females to males 1s 3:2.   — Solids, by forming a bolus and aided by gravity, as they  touch the contracted segment, may partially open up the  sphincter. Thus, there is no dysphagia for solids.  Dysphagia for liquids is an important feature and it results  in regurgitation (oesophageal pseudovomiting). The  regurgitant material contains foul-smelling oesophageal  contents. Malnourishment, ill health and weight loss  follow soon. Dysphagia, regurgitation and weight loss  form the triad of achalasia cardia.    Inhalation of amyl nitrite leads to sphincter relaxation  in achalasia but not in pseudoachalasia.   ¢ In chronic cases, 1t may be sigmoid-shaped (Figs 22.18  and 22.19).    ° Mediastinal mass (pseudotumour)* produced by dilated  oesophagus can be seen (Fig. 22.20).    stagnant food and fluid due to stasis which splashes out  with each heart beat and with each respiratory movement.   ¢ Also done to evaluate oesophagitis, stricture or a tumour  at cardia.    | Trypanosoma cruzi causes megaoesophagus, megaduodenum, megacolon and megaureter. Can we call it a ‘mega’organism?    * Aortic aneurysm, cold abscess (paravertebral), scoliosis are the other pseudotumours in the chest X-ray.   Fig. 22.18: Barium swallow Fig. 22.19: Barium swallow  showing dilated oesophagus showing sigmoid oesophagus    5. Oesophageal manometry: Following features are  characteristic of achalasia cardia:  ¢ Hypertensive lower oesophageal sphincter (LOS): It does  not relax on swallowing.  ¢ Aperistalsis in the body of oesophagus  ¢ Increased resting pressure in the oesophagus    6. Ultrasound: It may detect subepithelial tumour infiltration  in secondary achalasia due to a distal carcinoma.    You suspect of achalasia by clinical features  You support achalasia by Barium swallow  You confirm achalasia by manometry   More importantly, the obstruction at the lower end o  oesophagus must be relieved.   ¢ With a left thoracoabdominal incision, the oesophagus  and the stomach are completely mobilised.   ¢ A 7 to 10 cm long incision is made through the lower  end of the oesophagus and carried over to the stomach.  The muscles are cut till the mucosa bulges out. The  myotomy should extend proximally up to the aortic  arch and distally up to the stomach to I to 2 cm below  the junction. Success rate 1s around 90%. 3 to 5% of the  patients develop reflux oesophagitis which needs to be  treated conservatively.    balloon 1s positioned under fluoroscopic control within LOS.  It should be rapidly inflated to a pressure of 300 mmHg for  15 seconds. Success rate 1s around 70%. 20% chances of  reflux are present. Oesophageal perforation can also occur.  Recurrences are common (Figs 22.22 and 22.23).    the lower oesophageal sphincter (LOS) endoscopically.  Injection acts by interfering with cholinergic excitatory neural  activity at LOS and blocking acetylcholine release from nerve  terminals. It is temporary and repeat injections are necessary.    through endoscope, a submucosal injection of a mixture of  indigo rouge, epinephrine, and saline is done. A 2 cm long    incision is made and submucosal tunnel is created. Then  circular muscle bundle is cut slowly, beginning from 7-8  cm above GE junction to 2—3 cm below GE junction.    * Due to prolonged stasis and chronic irritation, it can  predispose to carcinoma of the mid and lower oesophagus  (due to metaplasia). Hence, it is a precancerous condition.    ¢ Squamous cell carcinoma is the most common type  identified in a patient with achalasia.    An entity of unknown aetiology, presents with dysphagia and  chest pain.    Aetiology: Exact cause is not known. Stress may be one of  the factors (Fig. 22.23).    Symptoms and signs  Discomfort in the chest (chest pain) or spasmodic pain,  dysphagia, weight loss.   1. Barium swallow: It is typically described as corkscrew  oesophagus—segmental spasms, area of narrowing and  irregular uncoordinated peristalsis.   2. Manometry: It is the diagnostic test. Findings are normal  lower oesophageal sphincter, alteration of oesophageal  peristalsis and contractions.   3. Ambulatory 24-hour pH monitoring: This is an  important test to rule out GORD.   ¢ Chest pain is the most common symptom followed by  dysphagia.   ¢ Majority of the patients have normal propagation of  peristaltic waves. However, in the distal oesophagus,  peristaltic waves have very high amplitude (>180 mmHg)  and duration (> 6 seconds).   ¢ Dilatation, myotomy, proton pump inhibitors, calcium  channel blockers are used with limited success.   ¢ Majority of oesophageal cancers are squamous cell  carcinoma, worldwide. However, in most Western countries,  more than 70% are adenocarcinomas. This shift is mainly  due to smoking, alcohol and Barrett’s oesophagus. Some  improvement has taken place now because of early diagnosis,  detection and multimodality treatment.   ° Oecsophageal cancers have poor prognosis because of the  following reasons:   — More lymph vessels are present in the submucosa than  blood capillaries and hence, spread is fast.   — Lymph flow tn submucosal plexus runs in a longitudinal  direction. Hence, primary tumour can extend for a  considerable length both superiorly and inferiorly.    ¢ Survival is improving  e There is improvement in detection and staging.  ¢ Detected more frequently in younger patients.    AETIOPATHOGENESIS  I. Precancerous/predisposing conditions  1. GORD with Barrett’s oesophagus (see page 432)    Malignancy in Barrett’s oesophagus is curable if detected  at an early stage. Hence, endoscopic surveillance is  recommended.    2. Plummer-Vinson syndrome with squamous metaplasia  (see page 438)—upper oesophagus.  . Achalasia cardia (see page 439)—lower oesophagus.  . Corrosive strictures (see page 451).  5. Familial keratosis palmaris or plantaris (tylosis): It is a  condition inherited as an autosomal! dominant trait. It has  increased incidence of oesophageal cancer.    1. Tobacco, heavy smoking and abuse of alcohol increases  incidence of both squamous cell carcinoma and  adenocarcinoma by almost 20-25%.   2. Human papilloma virus (HPV) infection increases  Squamous cell carcinoma. This oncogenic virus, has  been associated with cervical and oropharyngeal cancers  also.   3. Dietary carcinogens and squamous cell carcinoma  A. Elevated nitrates in drinking water.   B. Food (pickles, com) containing fungi—Geotrichum  candidum as in endemic areas of China.  C. Baked bread  4. Factors for squamous cell carcinoma  A. Hovels-Evans syndrome or tylosis—40 to 60% of  the patient develop cancers. Also called familial  keratosis plantaris.  Tylosis: (focal nonepidermolytic palmoplantar  keratoderma) 1s a rare disease inherited in an autosomal  dominant manner that 1s characterised by hyper-  keratosis of the palms and soles and oesophageal  papillomas. Patients with this condition exhibit  abnormal maturation of squamous cells. They also  cause inflammation within the oesophagus and are at  extremely high risk of developing oesophageal cancer.  B. Lye strictures up to 30%: Squamous cell carci-  nomas may arise in lye strictures, often developing  40 to 50 years after caustic injury. The majority of  these cancers are located in the middle third of the  oesophagus.    5. Factors for adenocarcinoma  A. Barrett’s oesophagus  B. Obesity: Increased body mass index ts a risk factor  for adenocarcinoma of the oesophagus, and  individuals with the highest body mass index have  up to a seven-fold greater risk of oesophageal cancer  than those with a low body mass index. Probably,  the linkage between obesity and gastro-oesophageal  reflux disease is presumed to be a chief factor.  C. Reflux oesophagitis  6.Low socioeconomic status as defined by income,  education, or occupation Is associated with increased risk  for oesophageal squamous cell carcinoma and,  adenocarcinoma to a smaller extent.  7. Helicobacter pylori infection: Infection with  Helicobacter pylori, and particularly with cagA+ strains,  IS Inversely associated with the risk of adenocarcinoma  of the oesophagus. Mechanism ts not clear. In fact, it  should not have been a factor responsible because  H. pylori infection can cause chronic atrophic gastritis  leading to decreased acid production, which negates the  effects of chronic reflux, including the potential for  development of Barrett’s oesophagus.    ¢ 50%—middle 1/3rd of oesophagus  ¢ 33%—lower 1/3rd of oesophagus  ¢ 17%—upper 1/3rd of oesophagus.   3. Infiltrative variety or annular stenosing variety with a  5 years survival of 10%, gives rise to early dysphagia due  to circumferential and longitudinal spread.   ¢ Dysphagia which ts progressive and mainly for solids (it  takes 18 months for dysphagia to develop). It means 60%  of the circumference of the lumen ts involved by the  growth. By the time, the patient presents with dysphagia,  the disease is fairly advanced. Hence, it has a poor  prognosis. Dysphagia is a late symptom because smooth  muscle of oesophageal wall can dilate with ease due to  lack of serosal layer on the oesophagus.   ¢ Regurgitation of the food contents. Haematemesis is not  very common. Vomitus may contain streaks of blood and  melaena Is rare.    ¢ To start with, it is a mucosal ulceration which spreads to  the submucosa. Later, it causes fibrosis and the lumen gets  narrowed. The spread occurs transversely and  longitudinally. Once 1t spreads to all the layers, the structures  in the vicinity are involved (Key Box 22.11).   ¢ When the trachea is involved, tracheo-oesophageal fistula  develops from carcinoma upper 1/3rd of oesophagus.   ¢ Oecesophagoaortic fistula results in massive bleeding (one  of the causes of death).    FACTORS RESPONSIBLE FOR EARLY  SPREAD AND AGGRESSIVE BEHAVIOUR  OF CARCINOMA OESOPHAGUS  Lack of serosal layer  Proximity of vital structures  Extensive mediastinal lymphatic drainage  Late presentation    Studies have showed that patients having metastasis to 5 or  fewer lymph nodes have a better outcome.    It results in secondaries 1n the liver, which clinically appear as  nodular enlarged liver. Later, ascites and rectovesical deposits  occur. Palpable left supraclavicular nodes indicate advanced  disease. This sign is described as Troisier’s sign.   2. Liver function test (LFT) ts affected, if secondaries in liver  occur (increased ALP).   3. Ultrasound is done to rule out liver secondaries, lymph  nodes in the porta hepatis, coeliac nodes, etc.   5. Oesophagoscopy to visualise the growth and to take biopsy  (Figs 22.28 to 22.33 and Key Box 22.12).    (indigocarmine) 1s done to identify dysplastic epithelium.  , 7. Bronchoscopy to rule out involvement of bronchus, as  If found, some advocate endoscopic mucosal resection    8. CT scan (Figs 22.34 and 22.35) of the chest to detect  local infiltration. It is a very useful investigation before  contemplating for total oesophagectomy. It is useful to    Dysplasia in Barrett’s mucosa ts a prognostic sign of  impending malignant degeneration.    assess involvement of vital structures such as bronchus,  airway, pericardium, aorta, etc. Obliteration of fat planes  gives the clue    . Endoscopic ultrasound to know the depth of wall  involvement, to detect mediastinal lymph nodes, etc  (Fig. 22.36 and Key Box 22.13).   To determine the depth of spread of a malignant tumour  through the oesophageal wall   Can also detect small lymph nodes which are less than  5 mm (which cannot be detected by CT scan also)   If ‘endosono’ detects more than 5 lymph nodes, it is not a  case for Curative resection.    tomography scanning: PET scan 1s based on the principle  that metabolically active tumour cells can be visible once  radiopharmaceutical agent | 8F-fluorodeoxyglucose   e More accurate in determining nodal status than non-  invasive techniques and helpful in evaluating the extent  of local invasion and metastatic disease.    e Thoracoscopy is accurate in 93% and laparoscopy in 94%  of patients in identifying metastatic disease.   e 6 cases of unsuspected coeliac nodal disease were  identified in 19 patients despite preoperative CT and EUS.   LAPAROSCOPIC ULTRASOUND (LUS)  e LUS is also a staging modality and may provide improved  accuracy in T and N staging.  e LUS may also be more accurate for staging coeliac nodes  because EUS probe is more distant and does not provide  direct inspection.   e For T1 lesions, conservative oesophageal resection such  as vagal-sparing, transhiatal or minimally invasive  oesophagectomy can be opted.   e For localised intramucosal tumours of limited extent, EMR  or ESD are an acceptable alternative.   e There is almost no role for chemoradiotherapy for treatment  of T1 lesions.   e Surgical or endoscopic resection alone carries a good long-  term survival, as high as 88% in some series.    cae 1. T1 lesion (intramucosal) 18% LNI  (FDG) is given intravenously. These lymph nodes, if 2. T1 lesion (submucosal) 55% LNI  malignant are clearly visible thus differentiating them 3. T2 lesion 60% LNI  from nonmalignant enlargement. 4. T3 lesion 80% LNI    Applying the same principles, PET can be used to know  the ‘response’ after radiotherapy or chemotherapy.    investigations, it 1s important to decide one can resect  this or not? Is 1t worthwhile resecting? Can patient  tolerate the procedure? Even in the absence of systemic  spread, some features will suggest surgical cure is  unlikely (Key Boxes 22.14 to 22.16).    ¢ General health of the patient, co-morbid conditions, fitness  for anaesthesia—all these factors decide the choice of therapy.   T4 Involvement of recurrent laryngeal nerve, phrenic nerve,  sympathetic chain, azygos vein; malignant effusion  (adjacent structures)   Mi Upper thoracic oesophageal carcinoma with spread to  other nonregional nodes or distant spread. Middle thoracic  oesophageal carcinoma with spread to neck nodes/  coeliac nodes or other nonregional nodes. Lower thoracic  oesophageal carcinoma with spread to other nonregional  nodes or distant spread.   Fig. 22.35: Contrast CT lateral picture showing total occlusion of Stage Ill - T3N1MO, T4 and N1NO    Fig. 22.36: Endosonography showing oesophagus and stomach  layers (Courtesy: Prof Ganesh Pai, HOD of Medical  Gastroenterology, KMC, Manipal)    I. A few considerations before treatment of carcinoma—since  they often present at an advanced stage, 5-year survival is very  low in the majority of cases. The following factors are  considered for palliative treatment:    3. Contiguous organ Trachea, aorta, pericardium  invasion (CT scan):  Peritoneal spread    Squamous cell carcinoma 1s the most common type  identified in patient with achalasia cardia.    In vast majority of cases, treatment of carcinoma  oesophagus 1s aimed at to relieve dysphagia by palliative  resection/palliative radiotherapy/metallic stenting.    Tumours that have not penetrated through oesophageal  wall and have not metastasised to regional lymph nodes  are potentially curable.    Decision of surgery/radiotherapy also depends upon  location of the tumour, histology, site of the tumour, staging  and cardiopulmonary reserve. Nutritional status has to be  improved to raise albumin levels from 3 to 3.4 g/dl to  decrease complications such as anastomotic leak.    Cervical oesophageal cancers (Fig. 22.37) are almost  always squamous cell carcinoma. Usually they are  postcricoid in a female patient. It is very unusual for this  malignancy to involve intrathoracic nodes. Upper thoracic  is also squamous cell carcinoma. Involvement of thoracic  nodes, early spread to trachea, bronchus and great vessels  is common.    Surgery followed by radiotherapy is the treatment of choice.  Local recurrence is high and they succumb to local disease.    If adjacent structure is not involved, total oesophagectomy  with or without laryngectomy can be done. Preoperative  neoadjuvant chemotherapy can also be given first (2-3  cycles followed by 3-5 Gy of radiation therapy).    This is followed by total laryngectomy with bilateral neck  dissection and reconstruction using gastric pull-up.    Tumours in the upper thoracic oesophagus are removed by  posterolateral thoracotomy, excision of the tumour and  lymph nodes followed by gastric pull-up requiring  laparotomy and pharyngogastric anastomosis in the neck.  This is the three-stage oesophagectomy.    They are usually adenocarcinomas. These tumours have a  tendency to spread for long distances submucosally and the  lymphatic flow is in the longitudinal direction.    In this operation, abdomen is opened first, stomach 1s  mobilised and the wound is closed. The patient is put in left  lateral position, and right thoracotomy is done through 6th  intercostal space. The growth is removed and oesophago-  gastric anastomosis 1s done inside the thorax, above the  level of aortic arch. Hence, it 1s described as a two-stage  [vor-Lewis approach (Figs 22.38 to 22.40).    However, a few surgeons do not advocate this surgery  because of following reasons:   1. Higher incidence of pulmonary complications.  2. Higher morbidity associated with anastomotic leak.  3. Higher incidence of oesophagitis.  4. Higher chances of local recurrence due to inability to   Hence, the alternate procedure (McKeown) ts a better choice.  McKeown’s three stage en bloc oesophagectomy  En bloc resection is the best treatment with removal of all  lymph nodes (abdominal and thoracic). Hence, three  incisions are required (1) abdominal, (2) right posterolateral  thoracotomy through the fifth space and (3) left cervical  incision. Local recurrence at the anastomosis is not a    Three incisions: Consequently, mobility is restricted and  postoperative pain is more. Hence, morbidity and mortality are  higher   Under vision all lymph nodes in the thorax, neck and the abdomen  can be removed   When lymph nodes involved are less than 8 in number, survival 1s  better than THE.    problem because of wide clearance (by obtaining a 10 cm  margin of normal oesophagus above the tumour).  Gastrointestinal continuity is obtained by gastric tube  anastomosis to cervical oesophagus.   En bloc resection is the best treatment with removal of all  lymph nodes (abdominal and thoracic).   Figure 22.41 shows various types of oesophageal surgery done  for carcinoma oesophagus.   Orringer’s transhiatal oesophagectomy—upper midline incision and  incision in the left side of the neck along the sternomastoid muscle and  anastomosis at cervical oesophagus to stomach.   Alternate choice  Two incisions: Abdominal and cervical. Hence, mobility is better  and postoperative pain is less  Pulmonary complications are less as chest is not opened. Hence,  mortality and morbidity is less than en bloc resection  Some mediastinal lymph nodes get cleared if proper dissection is  done through hiatus  Surgery takes much shorter period of time    1. Intubation: The choice of the intubation material is self  expanding metal stent (SEM). Two types are available—  covered and noncovered. The stent is deployed by endoscopy  under fluoroscopic guidance. It is collapsed during insertion  and once confirmed radiologically by position, it is expanded.  Minimal dilatation of the oesophagus (8 mm) is required to  place the stent (Figs 22.48 to 22.51). On the other hand, the  plastic tubes which were used earlier, required more than 8 mm  dilatation and there was always a risk of perforation. If the  patient is expected to survive beyond 3 months, SEM stent  can be considered. Complications include aspiration, feeling  of tightness in the chest, bleeding, food blockage, etc.   2. Laser therapy: Endoscopic laser treatment is used to core  the tumour and widen the lumen to relieve dysphagia. Principle  is thermal destruction of the tumour. The procedure has to be  repeated. Laser used is Nd: YAG laser or diode laser. Infiltrative  lesions are not suitable for laser treatment but exophytic lesions  are ideal—less than 6 cm.    3. Photodynamic therapy: It is used in early carcinomas—  mucosal lesions wherein patients are not willing for surgical  procedures including endoscopic mucosal resection. In this  procedure, a photosensitiser (porfimer sodium) Is given. It will  be taken by dysplastic cells and malignant cells.   4. Radiotherapy, brachytherapy: It 1s intraluminal radiation  (RT) with short penetration distance 1500 cGy radiation is  given. No systemic side-effects. Palliative external beam  radiation to doses of 50 to 60 Gy 1s successful in 50 to 70% of  patients.  §. Intratumour injection of absolute alcohol under endoscopic  control: Used as an adjunct to stenting to deal with tumour  overgrowth.    Carcinoma lower  end of oesopha-  gus—without open-  ing the thorax by  mobilisation from  above and below,  the oesophagus with  the growth can be  removed.   Figs 22.45 and 22.46: Opened specimen showing extensive ulceration Fig. 22.47: Transhiatal (Orringer) total oesophagectomy    and nodularity—minimum 10 cm clearance for oesophageal cancer is (Courtesy: Dr Sachidanand, Associate Professor,  important. Hence, THE is better than Ivor-Lewis operation to get this Karnataka Institute of Medical Sciences, Hubli,  length (Courtesy: Dr Basavananda Hartimath, Associate Professor, Karnataka)    Comments: Transhiatal oesophagectomy is a very good alternative (almost as good as en bloc) to lower oesophageal and GE  junction tumours. No specific attempt is made to remove lymph nodes. Patients tolerate the procedure very well. No mediastinal  complications related to leak are seen because of anastomosis in the neck. It gives a reasonably good relief from dysphagia and  survival. It is not blind oesophagectomy. With good retraction of the hiatus, mobilisation can be done under vision. It is not the type of  operation that influences survival but rather the stage of the disease at the time of the operation is performed.    6. Argon plasma coagulation  7. BICAP: BICAP tumour probes (Circon-ACMI, Stamford,  CT), which provide direct application of electrical current, are  limited to treatment of tumours that are circumferential. Bipolar  electrocoagulation tumour probe was employed for palliation  of obstructing circumferential oesophageal cancer.  8. Surgical palliation  * Resection: In fact transhiatal oesophagectomy 1s  considered as palliative treatment  ¢ Bypass: Palliative bypass: Oesophagus 1s not resected—  gastric tube to cervical oesophagus—mortality and  morbidity is high. To be considered last, that too only in  expert hands. Otherwise, consider feeding jeyunostomy.    Metallic self-expandable stents are currently the choice  of tubes to relieve dysphagia and to achieve palliation.    ¢ Complications such as bronchopleural fistula, aspiration  pneumonia, haematemesis due to erosion of aorta,  perforation of the growth and mediastinitis.    In spite of endoscopy and other investigations, the treatment  of carcinoma oesophagus is palliative in more than 95% of  cases, especially in our country. By the time the first symptom  of dysphagia appears, it 1s too advanced. Also, the location of  the tumour, adjacent structures in the mediastinum, widespread  lymphatic drainage alters the overall prognosis to a very low  percent of survival (Table 22.7).    ¢ Majority of oesophageal cancers are advanced at the time  of diagnosis.   ¢ 5 years survival rates after curative resections are around 10%.   ¢ Curative resections are major surgeries and should be  undertaken by an experienced surgeon.   ¢ Curative resections can be attempted at all levels of  carcinoma oesophagus (upper, middle and lower) provided  vital structures are not involved (assessment by CT scan  and endosonogram).   e Palliation: As majority of the patients with carcinoma  oesophagus have dysphagia, all the methods of palliation  are aimed at relieving dysphagia. Endoscopy followed by  dilatation using guide wire 1s a simple procedure with rate  of complications such as perforation around 2—3%.  However, the results are only for a few weeks. Self-  expandable metal stents are regularly used nowadays as a  palliation. They have two layers of superalloy monofilament  wire with a layer of silicon between them. This prevents  the tumour from growing within the stent.   ¢ Endoscopic laser therapy relieves obstruction and  bleeding. It can be carried out as an outpatient treatment  but needs multiple sittings.   ¢ Radiotherapy alone has been tried for squamous cell  carcinoma of the oesophagus—dose is 6,000 cG units. No  survival advantage is seen with this method.   ¢ Combination chemotherapy using cisplatin and  5-fluorouracil with radiotherapy has been tried in patients  who are not fit for surgery. Paclitaxel achieves high response  rate In metastatic oesophageal cancer and it also acts as a  radiation sensitiser.   ¢ Endoscopic intraluminal brachytherapy is used in cases  of recurrent tumour growing inside the lumen and causing  obstruction.    The entire treatment of carcinoma of oesophagus ts  aimed at ‘Cure’ in minority of cases and relief of  dysphagia in almost all cases.    These caustics are taken accidentally or in attempted suicide.  The agents are sodium hydroxide, sulphuric acid, household  bleach.   . Chronic reflux due to GORD, results in Schatzki ring—a  stricture at lower oesophagus at Squamocolumnar junction.  It is a circular ring consisting of fibrous tissue and cellular  infiltrates.   ¢ The squamous epithelium of oesophagus is relatively  resistant to acids (also due to rapidity of flow of fluids).   ¢ The corrosive injuries may involve oropharynx, larynx,  oesophagus, stomach and sometimes intestines also.   ¢ Alkali ingestion almost always leads to significant  destruction of oesophagus    e Superficial: Erythema, oedema, blisters, etc. Re-  epithelialisation of mucosa is complete by 6th week.   e Deep: Circumferential ulcers, produces scarring and  contractures. Major injury of the stomach produces gastric  outlet obstruction.    ¢ What should not be done: Stomach wash or induced  vomiting, as it will aggravate oesophageal injury or it  may result in aspiration. No role for corticosteroids in  cases of deep injuries or acid injuries.   ¢ What should be done: Verification of the aetiologic  agent and accurate assessment of depth and extent of  injury.    |. Examination of the oral cavity, chest and abdomen.  2. Auscultation of the lungs  3. Examination of upper airway   2. CT scan can reveal any perforation in the thorax or in  the abdomen.   Can be divided into acute and chronic cases. In acute cases,  treatment depends upon the severity of the injury or burn  Acute cases: Any surgical intervention should be done only  inselected referral centres. Kindly follow the instructions given  below.   7. First degree burns: Observation for 48 hours. If he can  swallow saliva, clear fluids can be started. Repeat  endoscopies after a month, 3 or 6 months later to rule out  stricture.   8. Second degree burns: Intravenous fluids, nil per oral,  acid suppression, relieve airway obstruction, parenteral  nutrition   9. Cervical oesophagostomy, staple the GE junction, feeding  jejunostomy. 6 weeks later, gastric pull up and oesophago-  gastric anastomosis, in cases of perforation.   dilatation. This is ideally done after 6 weeks (the time for re   e |ndicated in all cases of symptomatic oesophageal stricture.  Peptic strictures, corrosive strictures, anastomotic strictures  following oesophagogastric anastomosis in the neck after  ‘THE’ or following oesophagogastrectomy for lower  oesophageal cancer.   e Flexible oesophagoscopy is done and a guide wire is  passed. The scope is withdrawn. Solid dilators of increasing  diameter are passed over the guidewire.   e Astricture should be dilated to at least 16 mm in diameter  to restore normal swallowing.   e Beware of a GE junction stricture. It may be malignant.  Once it is dilated, a growth may be seen. Take a biopsy.   ¢ Oesophageal perforation is a serious, acute emergency  carrying a very high mortality rate. Fortunately it is not  common. There are many causes and are totally different  from gastric or duodenal perforations.    oesophagus and pyloric antrum of stomach. Oesophageal!  stricture was dilated. However, antral stricture could not be  dilated. Hence, he underwent vagotomy and gastrojejunostomy    of oesophagus. The scope could not be negotiated through the  oesophagus. He underwent dilatation. At least 16 mm diameter  of the oesophagus is the requirement for normal swallowing    1. Instrumentation: Endoscopy, dilatation of strictures,  injection sclerotherapy and laser treatment are the common  causes.    ¢ Severe pain in the neck, chest or abdomen, stiffness of the  neck depending upon the site of perforation.   ¢ Haematemesis, dysphagia, dyspnoea, hypotension,  tachycardia, shock and pleural effusion are the other  features.   ¢ Mackler’s triad: Chest pain, vomiting and subcutaneous  emphysema. It is present only in 10 to 15% of patients.    ¢ The investigation of choice is plain chest X-ray which can  demonstrate pneumomediastinum, subcutaneous  emphysema and mediastinal air fluid levels. When 1n doubt,  CT chest is diagnostic.   ¢ Early surgery should be done within 24 hours. Closure of  perforation and external drainage is the treatment.   ¢ Conservative treatment has risk of continuing leak,  sepsis and mediastinitis.   a case of grand admiral who was a glutton and practising   ¢ Excessive eating as in bulimia/excessive alcohol has been  thought to be responsible.    Site of perforation  Posterolateral wall of the lower third of the oesophagus.  Investigations   It is the investigation of choice to  detect the exact site of perforation    ¢ Cervical diverticulum (Fig. 22.55)  is the commonest type of diverticulum  which can present with regurgitation  of meals, aspiration of food contents  into the lungs or recurrent respiratory  tract infections. It is treated by  excision of the sac and repair of the  defect along with cricopharyngeal  myotomy—posterior midline.    Protrusion of oesophageal mucosa between cricopharyngeus muscle inferiorly  and thyropharyngeus muscle superiorly    It is due to some kind of motility disturbance, wherein protrusion occurs in  the lower end   I. Causes from outside (extraluminal) oesophagus  ¢ Thyroid swellings  ¢ Cardiomegaly  ¢ Aortic aneurysm  ¢ Mediastinal nodes: Tuberculosis, lymphoma or secondaries  ¢ Rolling hiatus hernia  If. Causes in the wall of oesophagus (luminal)  1. Oesophageal stricture  ¢ Corrosive acid poisoning  ¢ Tuberculous stricture    1. Acute dysphagia: Common in children. Foreign bodies  are common causes. Acute dysphagia with pain suggests  tonsillitis or pharyngitis.    In the absence of usual causes mentioned above, acute  dysphagia in an old man may be a manifestation of  vertebrobasilar insufficiency.    2. Chronic dysphagia: Stricture, achalasia, Plummer-Vinson  syndrome and carcinomas produce chronic dysphagia. The  increasing difficulty to swallow, first to solids and later to  liquids 1s typical of carcinoma oesophagus (in achalasia  cardia, it is the reverse).    3. Age and sex: Achalasia and Plummer-Vinson’s syndrome  is common in females, carcinoma in men and foreign body  in children.   4. Change in voice or even hoarseness with dysphagia  suggests advanced laryngeal carcinoma.    Dysphagia means difficulty in swallowing. Odynophagia  means painful swallowing as in quinsy, retropharyngeal  abscess, tonsillitis, etc.    1. Vascular slings and pulmonary artery slings: Occur due  to developmental anomalies of great vessels causing  dysphagia. More common 1s aortic arch anomaly wherein  the right subclavian artery arising from the descending aorta  travels behind oesophagus creating incomplete vascular ring  to complete the course. Pulmonary artery sling is due to  left pulmonary artery arising from right pulmonary artery  causing anterior compression of oesophagus.   2. Oesophageal ring: Schatzki’s ring: It is located at the  Squamo-columnar junction with oesophageal squamous  epithelium above and columnar epithelium below. There  is a concentric symmetrical narrowing with restricted  distensibility of lower oesophagus. It presents as dysphagia  and episodic aphagia. Diagnosis 1s by barium oesophago-  graphy. It is treated by repeated bougie dilatation. The cause  is obscure but there 1s strong association with reflux disease.   (median arcuate ligament)  Anterior at the level of 10th  thoracic vertebra | inch to the left side    Many rings are incidental findings in radiology. They do  not have any symptoms.   3. Webs: Oesophageal webs are uncommon causes of  dysphagia. They are treated by oesophageal dilatation.  See clinical notes.   A 60-year-old agriculturist was referred to the  department of ENT for dysphagia of 3—4 days duration.  A Registrar who saw the case did rigid oesophagoscopy  under GA. The findings were normal. Later in the evening  he was called to see this patient who had rigid abdomen.  A general surgeon was consulted. He suspected a  perforation. An X-ray abdomen (erect) however, was  normal. A senior faculty surgeon was consulted who  examined the case properly and gave a correct diagnosis.  It was a case of tetanus with mild trismus. The patient  had injured his left thumb a few days back. The  anaesthesiologist acknowledged later that there was some  difficulty in opening the patients mouth during  endotracheal intuabtion.    Occasionally, patients undergo gastroscopy for dysphagia  which will be normal only to realise later that what he is  having is a stroke!!!.    ¢ The diaphragm is a dome-shaped thin sheet of muscle which  separates the thoracic cavity from the abdominal cavity.   ¢ Itis derived from the innermost layer of the muscles of the  body. Hence, it arises in continuity with transversus  abdominis fibres from within the costal margins.    Oesophagus, vagus nerves, oesophageal branches of left  gastric artery, veins and lymphatics    The muscles of the dome on its abdominal surface 1s supplied  by phrenic nerve (C4). The crura are supplied by the lower  intercostal nerves.    Lower five intercostal arteries, subcostal arteries, and right  and left phrenic arteries supply diaphragm.    ¢ Maldevelopment of the septum transversum results in  diaphragmatic hernia. The causes of diaphragmatic hernia  are of two types: Congenital and acquired. The acquired  diaphragmatic hernias are very often (almost always) due  to road traffic accidents or due to stab injuries of the  abdomen. They are discussed in Chapter 36.   ¢ Majority of congenital hernias present shortly after birth.  However, a few cases present late to the hospital, and most  of such children are treated for recurrent respiratory tract  infection elsewhere, without a proper diagnosis.   ¢ 80% of congenital diaphragmatic hernias occur on the left  side.    1. Hernia through the foramen of Bochdalek: This is the most  common diaphragmatic hernia in children. These hernias  occur through the persistence of the pleuroperitoneal canals.  Respiratory distress, shift of mediastinum and scaphoid  abdomen clinch the diagnosis (Fig. 22.58).    2. Hernia through the foramen of Morgagni (parasternal  hernia): These hernias present in late childhood or in adult  life with features of partial (subacute) intestinal obstruction,  pain in the right hypochondrium or tightness in the chest  (Fig. 22.59). This is because majority of them contain  transverse colon.   Fig. 22.58: Bochdalek hernia showing intestine above the liver  as soon as laparotomy is done. The defect was two fingers (5 cm)  in diameter. Nonabsorbable sutures were used to close the defect  (Courtesy: Dr Balakrishna Shetty, Surgeon, Chinmaya Hospital,  Kundapur)    Fig. 22.59: Morgagni hernia—CT scan showing intestine  (colon within the thorax) (Courtesy: Dr Srinivas Pai, Dr Nitin Nagpal,  Dr Rohith Jain, Dept of Surgery, KMC, Manipal)    3. Herniation through the central tendon:  ¢ This can affect the right or the left apex of the cupola or  the central portion. The contents may be fundus of the  stomach on the left side and a portion of the liver on the  right side.    ¢ These cases are very often diagnosed accidentally by  routine chest radiograph.    4. Eventration: Due to paralysis or atrophy of the muscle  fibres, one or both hemidiaphragms are weak and they are  elevated in position. Truly speaking, this is not a hernia,  but signs and symptoms are almost like other types of hernia.   e Chest radiograph: Gas and fluid level is within the chest.   ¢ The thin rim of the diaphragm, as seen in a plain radiograph,  is broken or shows a defect.    ¢ The repair of the defect is done by using nonabsorbable  sutures.   ¢ Eventration of the diaphragm is repaired by plicating the  redundant diaphragm.    In majority of the cases (85%), lower end of the oesophagus  communicates with the trachea and upper end is blind  (Fig. 22.60).    ASSOCIATED ANOMALIES  : Vertebral defects  : Anorectal malformation  : Cardiac defect (PDA / VSD)  E : Tracheo-oesophageal fistula  : Radial hypoplasia and renal agenesis    ¢ Continuous pouring of saliva from the mouth in the newborn  baby is a diagnostic feature.   ¢ In difficult cases, where there is a long atretic segment,  colonic or gastric transposition is required.    Pneumonia, reflux, dysphagia, anastomotic leak.  MISCELLANEOUS  See the following two Key Boxes 22.20 and 22.21.   ¢ Investigation of choice to know the depth of invasion is  endosonography.    B) Lower end is blind and upper end is connected to trachea,   Mostcommon among the oesophageal diverticula is Zenker’s  diverticulum.  Most common benign oesophageal tumour is leiomyoma or  stromal tumour (GIST).  Most common upper GI disorder of the Western world is  gastro-oesophageal reflux disease.  Most common histological type of carcinoma oesophagus in  the world is squamous cell carcinoma.  Most common histological type of carcinoma oesophagus  among whites is adenocarcinoma.  Most commonly adenocarcinoma affects lower oesophagus.  Most common presenting feature of Barrett’s oesophagus is  dysphagia.  Most common site of gastrointestinal involvement in  scleroderma is smooth muscle of oesophagus.  Most common cause of oesophageal perforation is  instrumentation.  Most serious but rare type of perforation is Boerhaave  syndrome—barotrauma, seen in gluttons.    e Mechanism of swallowing and surgical anatomy  correlated to surgery has been discussed.    e Endoscopic resection: !t is attempted as a cure for  Carcinoma confined to mucosa. It is guided by  endosonography. Re-epithelialisation is complete by  3 weeks.    B. They are classified as perioesophageal, paraoeso-  phageal and lateral oesophageal lymph nodes    Factors for development of reflux disease of  oesophagus include following except:   Gold standard for the treatment of reflux disease is:  A. Barium meal    Which of the following conditions predisposes to  volvulus of the stomach?    C. Can be used after chemotherapy to see the response of  the tumour   A. Thoracotomy is a must for completion of the procedure  B. Anastomosis ts in the neck   A. Balloon dilatation is ideal for patients above the age of  45 years    A. The vertical split occurs in the lower end of oesophagus  in majority of cases    The oesophagus continues from the oesophagogastric (OG)  junction as a muscular tube—the stomach.    Part of the stomach which projects upwards and lies in contact  with the left dome of diaphragm. It is usually full of gas.    ¢ To identify the side (right or left) of the body in a plain  X-ray abdomen.   ¢ During mobilisation of the fundus as in splenectomy or other  upper gastric surgery, short gastricarteries need to be divided.  If ligatures are too close to the stomach near the fundus,  gastric fistula may occur due to necrosis of the stomach.    Extends from fundus to /mcisura angularis. It has a lesser  curvature and a greater curvature.    ¢ Ability to have a large meal is due to receptive relaxation  of the body of the stomach.   Duodenal anatomy and obstruction  Gastric surgery for morbid obesity  What is new?/Recent advances    ¢ Classical gastrojejunostomy (GJ), anterior or posterior,  involves using body of the stomach.    Pyloric antrum  It extends from incisura till pylorus. Pylorus is thicker than    e¢ Pyloric antrum is a common site for gastritis, ulcer and  carcinoma.    e It 1s in close contact with the head of pancreas. During  gastrectomy, extreme care has to be taken to mobilise the  antrum to avoid bleeding in the pancreatic head region.   It lies in contact with transverse colon and gastrocolic omentum  (Fig. 23.1 and Key Box 23.1). This has to be divided from  transverse colon during gastrectomy which is done for  carcinoma or ulcer.   e [tis suspended between the lesser curvature of the stomach  and the proximal 0.5 inch (2 cm) of the first part of the  duodenum inferiorly and the porta hepatis and the fissure  of the ligamentum venosum superiorly.   2. Hepatoduodenal: Located within the lesser omentum are  the hepatic triad, branches of the anterior vagus nerve,  some lymph nodes, and the right and left gastric arteries.    ¢ The left and right vagus nerves descend parallel to the  oesophagus and form oesophageal vagal plexus between the  level of the tracheal bifurcation and level of the diaphragm.  ¢ From this plexus, two vagal trunks, anterior and posterior  form and pass through the oesophageal hiatus of the    GREATER OMENTUM IS A FRIEND AND &  SOMETIMES ENEMY OF THE PATIENT  AND THE SURGEON    The greater omentum is the policeman of the peritoneal  cavity. It exhibits protective action in inflammatory  processes and perforations.   Omentoplasty (omental wrapping of anastomosis) may  decrease the occurrence of anastomotic leakage when  oesophagogastric anastomosis is performed for cancer of  oesophagus or as in closure of duodenal perforation.   It has many important applications and can be used in  colorectal surgery, reconstruction of the irradiated pharyngeal  wound and packing of bleeding liver wounds.   lt was used to treat peripheral vascular disease such as  TAO wherein direct arterial surgery was not possible.   It becomes an enemy when involved with metastasis or  directly by malignant neoplastic process from ovary or  colorectal cancer. Omentum is one of the common sites of  involvement in tuberculosis also. Omentum itself can act like  a band and can cause intestinal obstruction.    diaphragm (mnemonic LARP: Left trunk—anterior  gastric wall; right trunk—posterior gastric wall).    1. Left gastric artery is a direct branch of coeliac trunk. It    ascends up to oesophageal hiatus and turns to the right along  the lesser curvature of stomach. It branches and anastomoses  with branches of right gastric artery and supplies anterior  and posterior wall of the stomach. There is true anastomosis  between branches of left gastric artery and branches from  other arteries.    2. Right gastric artery is a branch of hepatic artery which  comes from coeliac trunk. [t also supplies lesser curvature  and body of stomach, along with left gastric artery.   3. Left gastroepiploic artery arises from splenic artery and  supplies greater curvature of stomach and anastomoses with  right gastroepiploic artery.   4. Right gastroepiploic artery is a branch of gastroduodenal  artery, which 1s a branch of hepatic artery.   5. Short gastric arteries are the branches of splenic artery.  They supply the fundus of the stomach. They are also called  vasa braevia.   e Right and left gastric veins drain into the portal vein directly.   ¢ Because of extensive anastomoses of blood vessels,  (extramural and intramural collateral vessels) stomach can  survive with right gastric and right gastroepiploic arteries  only. Thus stomach can be used to replace the entire  oesophagus after oesophagectomy—-gastric pull up.   ¢ The order of ligation of blood vessels in gastrectomy Is as  follows: Left gastroepiploic, right gastroepiploic, right gastric  (then stomach is divided) and lastly, left gastric artery.   ¢ Gastroduodenal artery, a branch of hepatic artery runs behind  first part of duodenum and divides into right gastroepiploic  artery and superior pancreaticoduodenal artery.   e It is this artery which bleeds when a posterior duodenal  ulcer erodes into It.    Lymphatic drainage (Fig. 23.3)  It is an important pathway for spread of carcinoma of the  stomach. The spread occurs both by emboli and permeation.   Inferior gastric subpyloric group  Fig. 23.3: Lymphatic drainage of the stomach    2. Subpyloric nodes/gastroepiploic nodes (right) drain the  greater curvature of stomach and pyloric antrum.   3. Left gastroepiploic nodes (splenic nodes) drain the upper  portion of stomach, mainly the fundus (carcinoma of fundus).   4. Left gastric (superior gastric) nodes drain the lesser curvature  and body of the stomach (anterior and posterior wall).   5. Coeliac nodes receive lymph from the entire foregut  (including stomach) and drain directly into the cisterna chyli  and the thoracic duct. Later, mediastinal nodes and left  supraclavicular nodes (Virchow nodes) are involved.    The lymphatics of the stomach are formed from two networks:  Intrinsic and extrinsic.   ¢ Upward invasive spread of gastric cancer to the oesophagus  is common but downward mucosal or submucosal invasion  of the duodenum is not common.    ¢ Hence, gastrectomy with distal oesophagectomy is done  for carcinoma of the proximal stomach.   ¢ They drain to perigastric lymph nodes, which in turn drain  to the lymph nodes located at the coeliac axis.   3. The pancreaticolienal group of nodes drains lymph high  on the greater curvature into the left gastroepiploic and  splenic nodes.   4. Theinferior gastric and subpyloric group of nodes drains  lymph along the right gastroepiploic vascular pedicle.   ¢ All four zones of lymph nodes drain into the coeliac group  and then into the thoracic duct.   CAUSES OF HYPERGASTRINAEMIA wd  Ulcerogenic causes Nonulcerogenic causes  e Antral G cell hyperplasia e Antisecretory agents (PPIs)  or hyperfunction  e Retained excluded antrum e Atrophic gastritis  e Zollinger-Ellison syndrome e Pernicious anaemia  e Gastric outlet obstruction ° Acid-reducing procedure  (vagotomy)  e Short-gut syndrome e Helicobacter pylori infection  ¢ Chronic renal failure    Gastric acid secretion by the parietal cells is regulated by  three local stimull:   These three stimuli account for basal and stimulated gastric  acid secretion.   Acetylcholine is the principal neurotransmitter  modulating acid secretion and is released from the vagus  and parasympathetic ganglion cells.   Vagal fibres innervate not only parietal cells but also G cells  and enterochromaffin-like (ECL) cells to modulate release  of their peptides.   Gastrin has hormonal effects on the parietal cell and  stimulates histamine release (Fig. 23.4).   Histamine has paracrine-like effects on the parietal cell.  Its release from ECL cells plays a central role in the  regulation of acid secretion by the parietal cell. Ingestion  of a meal stimulates vagal fibres to release acetylcholine  (cephalic phase). Binding of acetylcholine to parietal cell  and G cell results in the release of histamine, hydrochloric  acid and gastrin, respectively. Acetylcholine also inhibits  somatostatin release by inhibiting ECL.   Following a meal, G cells are also stimulated to release  gastrin, which interacts with receptors located on ECL cells  and parietal cells to cause the release of histamine and  hydrochloric acid (gastric phase). Release of somatostatin  from D cells decreases histamine release and gastrin release  from ECL cells and G cells, respectively. In addition,  somatostatin inhibits parietal cell acid secretion (not shown).  The principal stimulus for activation of D cells is antral  luminal acidification (not shown).   Release of somatostatin from antral D cells is stimulated  in the presence of intraluminal acid to a pH of 3 or less.  After its release, somatostatin inhibits gastrin release  through paracrine effects and also modifies histamine  release from ECL cells.    In some patients with peptic ulcer disease (PUD), this  negative feedback response is defective.    Consequently, the precise state of acid secretion by the  parietal cell is dependent on the overall influence of the  positive and negative stimull.    Gastric acid plays a critical role in the digestion of a meal.  It is required to convert pepsinogen into pepsin, which is  necessary for hydrolysis of proteins into polypeptides.  Gastric acid also elicits the release of secretin from the  duodenum, which results in pancreatic bicarbonate secretion.  In addition, gastric acid functions to limit colonisation of the  upper GI tract with bacteria.    There are numerous ways to assess acid secretion in the  stomach.   Aspiration of gastric contents through a nasogastric tube  is probably the most accurate.   The study requires complete emptying of gastric contents  followed by instillation and recovery of 50 ml of saline.    Drug Mechanism  Metoclopramide Dopamine antagonist  Domperidone Dopamine antagonist  Erythromycin Motilin agonist  Bethanechol Cholinergic agonist  Neostigmine Cholinergic agonist    The stomach is then aspirated every 5 minutes for | hou  and the aspirates pooled in 15 minute aliquots.   At the end of | hour, the stomach ts stimulated to secrete  acid by intravenous (IV) administration of a secretogogue  such as Histamine (2 g/kg) or Pentagastrin (6 g/kg).  Aspiration of the stomach continues with four 15-minute  collections obtained over a |-hour period.   The volume of collections is measured, and each aliquot is  titrated to determine the amount of hydrogen ions present.  The rate of secretion is expressed as the number of milli-  equivalents produced per hour during the basal (unstimulated)  state and during maximal and peak acid output (Key Box 23.4).  Maximal acid output (MAO) is obtained by averaging the  output of the two final 15 minute periods. MAO Is in the  range of 10 to 15 mEq/hour.   Peak acid output is the highest rate of secretion obtained  duringa 15-minute period following secretogogue stimulation.  Basal acid output (BAO) is generally about 2 to 3 mEq/hour.    It is now established that 90% of duodenal ulcers and 75%  of gastric ulcers are associated with H. py/ori infection.  Warren and Marshall were the first to identify and isolate  the organism, originally referred to as Campylobacter pyloridis.  The organism 1s a spiral or helical gram-negative rod with  4 to 6 flagella and resides in gastric-type epithelium within  or beneath the mucus layer, which protects it from both  acid and antibiotics.   Its shape and flagella aid its movement through the mucus  layer, and it produces a variety of enzymes that help it adapt  to a hostile environment.   Most notably, it is one of the most potent producers of  urease among any bacteria yet described.   This enzyme is capable of splitting urea into ammonia  and bicarbonate, creating an alkaline microenvironment  in the setting of an acidic gastric milieu, which facilitates  establishing a diagnosis of this organism by various  laboratory tests.   The organism is microaerophilic, and the optimal tempera-  ture for isolation is 35 to 37°C, with growth occurring after  2 to 5 days.    Interestingly, H. py/ori can only live in gastric epithelium  because only gastric epithelium expresses specific  adherence receptors in vivo that can be recognised by the  organism. Thus, it can also be found in heterotopic gastric  mucosa in proximal oesophagus and Barrett’s  oesophagus, in gastric metaplasia in the duodenum and  Meckel's diverticulum, and in heterotopic gastric mucosa  in the rectum.   The mechanisms responsible for H. pylori-induced GI  injury is not very clear but three potential mechanisms have  been proposed:   3. Increased gastrin levels with a resultant increase in acid  secretion.    Phospholipases that damage epithelial cells and mucus cells.  Platelet-activating factor, which is known to cause mucosal    Most H. py/ori strains express—vacuolating cytotoxin.  The VacA exotoxin causes the release of cytochrome c from  mitochondria and induces apoptosis.   H. pylori infection is accompanied by an abnormal  T-cell response which may be partially responsible for the  long-term persistence of H. pylori infection.   Enzyme produced is urease, which splits urea. Resulting  bicarbonate creates alkaline environment. Another enzyme  mucinase degrades mucus.    H. pylori infection can be diagnosed by both noninvasive and  invasive means.   They are urea breath test, serology, and detection of  antigen in stool samples.   The urea breath test is based on production of urease by H.  pylori in the gastric mucosa.   '4C_labelled urea is ingested and '*C-labelled CO, is  produced and excreted in the breath.   This test has a sensitivity and specificity of greater than  90% and indicates ongoing infection.   The urea breath test is useful for initial diagnosis of infection  and for follow-up after eradication therapy.   The sensitivity of this test varies from 80 to 100% and   Culture of H. py/ori is not routine and is usually reserved    for recurrent infection and for antibiotic sensitivity testing  when second-line therapy has failed.    To be effective, antimicrobial drugs must be combined with  gastric acid secretion inhibitors or bismuth salts.   Cure rates of 80-85% are achieved using combination  therapy, usually proton pump inhibitors, ranitidine, or  bismuth citrate with two antibiotics.   The most common antibiotics used are clarithromycin,  amoxicillin, and metronidazole or tinidazole.  Metronidazole has been a mainstay of H. py/ori treatment.  Eradication therapy with a proton pump inhibitor,  metronidazole, and amoxicillin decreases the prevalence  of metronidazole-resistant 1. py/ori strains.    Bismuth, 2 tablets four times daily + Metronidazole, 250 mg  three times daily + Tetracycline, 500 mg four times daily.    . Histologic test  is done by  staining with  silver, Giemsa,  etc.    When the patient has not received  treatment for H. pylori infection, test of    This test is to confirm cure of H. pylori  infection, but no sooner than 4 weeks after  completion of therapy    Presence of 7. pylori in biopsy material  taken during endoscopy establishes the    Sensitivity of 80 to 100%, specificity  of > 95%; Giemsa stain has sensitivity  of > 95% and specificity of 99%    Simple; rapid (once biopsy specimen  has been obtained); sensitivity of 80 to  95%, specificity of 95 to 100%    Allows determination of antibiotic  susceptibility. Sensitivity is 80% and  specificity is 100%    Does not confirm eradication, because  serologic positivity remains for  indefinite period after microbiologic  cure    False-negatives possible if testing is  done too soon after treatment for  H. pylori infection. Small radiation  exposure with '*C method, cost and  availability are disadvantages    Requires laboratory facilities and  experience; when haematoxylin-eosin  stain is nondiagnostic, other stains  have to be used    PPI twice daily + Amoxicillin, 1000 mg two times daily + 1. Chronic duodenal ulcer   Clarithromycin, 500 mg two times daily or metronidazole, 500 2. Gastric carcinoma   mg two times daily. 3. Gastro-oesophageal reflux disease  4. Barrett’s oesophagus    metronidazole, 250 mg three times daily + Tetracycline, 500 7. Oesophageal cancer  mg four times daily. 8. Idiopathic thrombocytopaenic purpura.   ¢ Fundus and body are affected ¢ Antrum is affected - Antrum is affected ¢ It affects entire stomach   ¢ Atrophy of parietal cell mass. ¢ Predisposes to peptic ulcer ¢ Usually does not give rise to ¢ It occurs due to defective  Hence, hypochlorhydria and disease peptic ulcer disease gastric mucosal barrier  decreased intrinsic factor    Type B gastritis and erosive gastritis are common.  Endoscopy and biopsy are the key investigations in all forms  of gastritis.   Acid peptic digestion of the alimentary mucosa resulting in  an ulcer 1s called peptic ulcer disease (PUD). The corrosive  effects of acid with proteolytic effect of pepsin are responsible  for PUD. Duodenum and stomach are the common sites  of peptic ulcer disease. Rarely, they can occur in the jejunum  and in Meckel’s diverticulum when it contains ectopic gastric  mucosa.    A. Chronic duodenal ulcer: Typically occurs in the first inch  of the first part of the duodenum.    B. Chronic gastric ulcer: Occurs in the lesser curvature  adjacent to acid secreting parietal cell mass.   Before we start discussion on peptic ulcer disease, we will  discuss nonulcerative dyspepsia (commonly mistaken as peptic  ulcer disease) and Zollinger-Ellison’s syndrome which is one  of the rare causes of peptic ulceration.    e Dyspepsia is characterised by symptoms related to upper  abdominal viscera. Symptoms may include abdominal pain,  bloating, indigestion, nausea and heart burn. Heart burn as  a single symptom suggests gastro-oesophageal reflux  disease and excludes the diagnosis of dyspepsia.    Nonulcerative dyspepsia is considered when no anatomic  or biochemical abnormality 1s discovered to explain the  patient’s symptoms.    After H. pylori infection, ingestion of nonsteroidal anti-  inflammatory drugs (NSAIDs) is the most common cause  of nonulcerative dyspepsia and PUD.    ¢ H. pylori infection is always associated with histologic  gastritis whichis absent in cases of nonulcerative dyspepsia.   ¢ Hence H. pylori eradication therapy is not recommended  in the treatment of nonulcerative dyspepsia.   ¢ For surgeons, the importance of nonulcerative dyspepsia  relates to its place in the differential diagnosis of epigastric  pain. There is no role for surgery in the treatment of this  disorder.   ¢ Some of these patients who have been diagnosed as  nonulcerative dyspepsia may have gall stones or  oesophageal reflux disease. Hence abdominal ultrasound  and oesophageal manometry 1s required to rule out these  diseases.   ¢ Ocsophagogastroduodenoscopy should be done in all the  patients with nonulcerative dyspepsia followed by biopsy  from stomach to rule out H. py/ori infection.One important  cause of peptic ulcer disease—Zollinger-Ellison syndrome  is being discussed first.    ¢ Zollinger-Ellison syndrome (ZES) 1s a clinical triad  consisting of gastric acid hypersecretion, severe PUD,  and non-islet cell tumours of the pancreas.   ¢ The tumours are known to produce gastrin and are referred  to as gastrinomas (Fig. 23.5).   e These tumours are usually localised to the head of the  pancreas, duodenal wall or regional lymph nodes.   ¢ About one-half of these gastric tumours are multiple and  two-thirds are malignant.    About one-fourth are associated with multiple endocrine  neoplasia syndrome (MEN 1). Other tumours in MEN | are  pituitary adenoma and parathyroid adenoma.    Pathophysiologic features of ZES that distinguish it from  duodenal ulcer are present and are all explained either by the  actions of gastrin to stimulate gastric acid secretion and  mucosal growth or by the action of other associated hormones  in the MEN | syndrome.    ¢ Abdominal pain and PUD are the hallmarks of the syndrome  and typically occur in more than 80% of patients.   ¢ About one-half of patients have diarrhoea secondary to  increased gastric acid secretion.   ¢ Weightlossand steatorrhoea alsooccur secondary to decreased  duodenal and jejunal pH and inactivation of lipase.    ¢ Demonstrates prominent gastric rugal folds, reflecting  the trophic effect of hypergastrinaemia on the gastric  fundus in addition to evidence of PUD.    ¢ Gastrinoma and ZES are always considered and ruled out  in patients who have:    1. Recurrent or intractable PUD despite eradication of  H. pylori and appropriate antisecretory therapy.    2. Multiple or atypically located ulcers.  3. PUD associated with significant diarrhoea.    4. PUD associated with symptoms of MEN | such as  hyperparathyroidism or kindred of MEN 1.    7. Patients undergoing elective surgical intervention for  PUD have the possibility of gastrinoma included in their  preoperative evaluation.    Most patients with gastrinoma have elevated fasting levels  (> 200 pg/ml), and values greater than 1000 pg/ml may be  diagnostic.    ¢ It is the most sensitive and specific provocative test for  gastrinoma and aids in the differentiation between  gastrinomas and other causes of ulcerogenic hyper-  gastrinaemia.   ¢ Secretin (2 g/kg) is administered intravenously and serum  gastrin samples are measured before and after secretin  administration at 5-minute interval for 30 minutes.   e An increase in serum gastrin of greater than 200 pg/ml above  basal levels 1s specific for gastrinoma.    ¢ Noninvasive methods include computed tomography (CT  scanning, MRI, endoscopic ultrasound, and '''In-octreotid  scintigraphy (somatostatin receptor imaging).    ¢ Invasive modalities include selective visceral angiography  percutaneous transhepatic portal venous sampling for gastrir  and selective arterial secretin stimulation test.    ¢ Inpatients with resectable gastrinomas, surgical resectior  is performed that includes tumour resection from the  duodenum, pancreas and regional lymph nodes.    ¢ Total gastrectomy is rarely indicated and is reserved fo!  patients who are noncompliant with acid suppressior  therapy or when the tumour cannot be localised.    Hyperacidity is the chief cause of duodenal ulcer. No acid, no  ulcer still holds good for CDU.    2. Nonsteroidal anti-inflammatory drugs: They are  responsible for gastric ulcer rather than duodenal ulcer by  altering mucosal defence.    3. Genetic causes: Family history of duodenal ulcer may be  present in a few cases which suggests a genetic cause.    e Patients with blood group ‘O’ are more prone, for the  development of CDU because of increased parietal cell  population.    4. Food habits: Spicy food, diet poor in vitamins, smoking  and alcohol, alone or in combination precipitate the  development of chronic duodenal ulcer.    ¢ Helicobacter pylori, a spirochaetal bacteria has been  demonstrated in the submucosa of the antrum and  duodenum, from the biopsies of the ulcer. It increases  PH levels by splitting urea and releasing ammonia.  Rise in pH results in proliferation of bacteria.    ¢ Zollinger-Ellison syndrome is a non-f cell tumour of the  pancreas with hypergastrinaemia.    ¢ Hyperparathyroidism causes increased levels of calcium  which stimulates the parietal cell mass resulting in  hyperacidity.    The only absolute requirements are acid and pepsin secretion  in combination with either infection with H. pylori or  ingestion of NSAIDs.    Although there is a strong correlation between parietal cell  number and MAO, only patients with duodenal ulcers have  an increase in mean parietal cell number, whereas gastric  ulcer patients do not.    In majority of the patients there is no hyperacidity. Many  patients have hypoacidity, or normoacidity.   Ulcer occurs due to a defective gastric mucosal barrier. This  barrier 1s a coat of thick mucus which 1s impermeable to  pepsin.   Prostaglandins normally present in the gastric mucosa do  not allow back diffusion of hydrogen ions from the lumen.  Nonsteroidal anti-inflammatory drugs inhibit the production  of prostaglandins causing a loss of protective activity.  This can also be damaged by smoking, spicy food, alcohol  and reflux of bile into the stomach.   SOME CONDITIONS THAT MAY S  PREDISPOSE TO GASTRIC ULCERATION  Age older than 40, sex (female to male ratio of 2 : 1).    Ingestion of barrier-breaking drugs such as aspirin or  NSAIDs—nonsteroidal anti-inflammatory drugs    Abnormalities in acid and pepsin secretion  Gastric stasis through delayed gastric emptying  Coexisting duodenal ulcer    Type 1: Primary gastric ulcer on the lesser curvature in the  antrum near the junction of oxyntic cells and central mucosa.    Type 2: Gastric ulcer with duodenal ulcer.  Type 3: Prepyloric or channel ulcer.    Type 4: High gastric ulcer (cardia, proximal stomach)  < 2 cm from oesophageal junction.   ¢ High chances of perforation and bleeding.  e Medical treatment heals majority of cases (80%) 1    Types 2, 3 gastric ulcer are to be treated like duodenal  ulcer because they are associated with hyperacidity.    . Oesophagogastroduodenoscopy (OGD): Ulcer appears as  a crater with/without slough or bleeding in their typical  locations. In gastric ulcers, routine biopsy is advised to rule  out malignancy. In duodenal ulcers, biopsy is done in    e Re-endoscopy biopsy is a must to look for healing and also  to rule out malignancy  e Gastrectomy including ulcer bed. Add vagotomy for type 2   3. Pain It is due to the acid irritating the ulcer (hunger pain). It is  relieved on taking food. After 1-2 hours of food, the pain  becomes severe. It 1s burning in nature with retrosternal  radiation (heart burn) and increased salivation (water brash)   4. Vomiting Never occurs in duodenal ulcer till the  patient develops pyloric stenosis   e Percutaneous endoscopic gastrostomy (PEG)  e Endoscopic mucosal resection for early carcinoma stomatch    ¢ In long-standing duodenal ulcers, there may be narrowing  of the pylorus, with stasis of food in the stomach  suggestive of pyloric stenosis.   ¢ Duodenal ulcer: Deformed duodenal cap. Trifoliate  deformity is seen when secondary duodenal diverticulum  occurs.   ¢ Gastric ulcer appears as a niche in the lesser curvature  due to ulcer crater and as a notch on the greater curvature  due to the spasm of stomach.   ¢ In general benign ulcers  are smoother, more regular,  with rounded edges and a  flat, smooth ulcer base. In  malignancy, the mass  protrudes into the lumen  or has folds surrounding  ulcer crater.    Aim Is to decrease the pain because of its severity (by reducing  acidity) and to prevent relapses (Key Box 23.9).   ¢ Ranitidine: 150 mg twice a day, for 6 weeks. 90-95% of  the healing occurs within 6 weeks. At the end of 6 weeks,  150 mg at bed time is given for a period of 3 months as  maintenance therapy. Re-endoscopy can be done in between  to assess the healing of the ulcer.   Fig. 23.13: Gastric outlet  obstruction. Barium meal can  detect dilated stomach   AIMS OF THE TREATMENT  e To relieve symptoms  e To heal the ulcer  e To prevent recurrence  e To prevent complications    ¢ Famotidine: 20 mg twice a day 1s as effective as ranitidine.   ¢ Roxatidine: Can be used along with food, bronchodilators  and antacids, unlike the other H, blockers. The dosage 1s  75 mg twice a day.  — The problem with H, blockers is that relapse occurs if   they are stopped. 80—90% healing rates occur after  6 weeks of therapy.   ¢ 95-99% of healing within 2-4 weeks. More rapid healing  than H, receptor blockers.   ¢ Patients who receive omeprazole for one month are put on  a maintenance dose of ranitidine for 3 months (150 mg HS  for 3 months).   e Other drugs such as esomeprazole: 40 mg/day, lansoprazole:  30 mg/day, pantoprazole: 40 mg/day are also used.   ¢ Pantoprazole in a dose of 40 mg/day for 4 weeks has  become the first line of treatment for chronic duodenal  ulcer.   ¢ Given in high doses (120 ml/day) they will neutralise the  acid (not practical). Small dose of antacids can be added to  H, blockers. This gives psychological benefit to the patient.   4. Diet should be bland. Spicy food, coffee, alcohol, and  smoking are discouraged. Discontinue aspirin or NSAIDs  and other gastric irritants.    Highly selective vagotomy (HSV): It is also called PCV  (parietal cell vagotomy) or PGV (proximal gastric  vagotomy) (Fig. 23.22).    In this operation, vagi are not divided at the trunks. Both  anterior and posterior vagus are identified, isolated and  preserved. Their branches which run along the lesser  curvature are isolated. They are anterior and posterior  greater gastric nerves of Latarjet. The branches of the nerves  of Latarjet supplying parietal cell mass are divided. Hence,  it is called parietal cell vagotomy. The terminal fibres of  the nerve of Latarjet which supply pylorus are preserved  (5-7 cm of ‘Crow foot’).    More physiological, with minimal disturbances.  No drainage procedure is required because pyloric functions  are preserved.    This 1s not the procedure for prepyloric ulcer as there is a  high recurrence rate.    ¢ This is the most popular and most commonly done  operation for peptic ulcer disease. However, it 1s  important to realise that indications for vagotomy and  GJ are becoming less and less today.    ¢ Transection of both vagal trunks at oesophageal hiatus  denervates acid producing fundus of stomach. It also  denervates the remainder of the viscera supplied by vagus  including liver, biliary tree, pancreas, intestines up to mid-  transverse colon.    Anterior and posterior trunks of the vagus are divided  just below the diaphragm followed by a drainage procedure  such as gastrojeyjunostomy (GJ). Vagus is secretomotor to    1. Intractable pain in spite of treatment with proton pump  inhibitor and not responding to anti H. pylori regime    stomach and after vagotomy the motility of the stomach  is lost and gastric stasis occurs. Hence, drainage  procedure is done.    ¢ Posterior GJ is preferred because it gives a dependent  drainage of the food contents by gravity. Classically, it is  described as “Posterior, Vertical, Retrocolic, Isoperistaltic,  No loop (short loop), No tension, GJ of Mayo (PVRING)”’.    ¢ Alternatively, pyloroplasty is preferred by a few surgeons  instead of GJ.   ¢ In Heinecke-Mickulicz pyloroplasty, pylorus is incised  longitudinally and sutured vertically. Thus, the pyloric ring  becomes incompetent and wide open. Bile reflux gastritis  and diarrhoea are major problems after pyloroplasty  (Fig. 23.24).   ¢ Finney pyloroplasty is another procedure wherein inverted  U-type of incision is given involving distal stomach and  duodenum and a gastroduodenal stoma is created. This type  of pyloroplasty is indicated when duodenum is dilated  (Fig. 23.25).    ¢ By removing the vagal stimuli and the antral gastrin, the  entire stimulus to acid secretion is lost. Hence, it carries  the least recurrence rate (1%) but carries 3-4%  mortality rate. Not done routinely.    ¢ Aim: Healing of the ulcer and relief of symptoms.  ¢ Frequent biopsy is done to rule out malignancy.    I. Medical line of treatment can be given for duodenal ulcer,  in the form of ranitidine or omeprazole. Cigarette smoking  should be stopped. Drugs such as NSAID and aspirin are to  be avoided. If an ulcer persists after 6 weeks, the aim is to  eradicate H. pylori provided malignancy is ruled out.    II. Surgery is indicated in case of gastric ulcer persisting in  spite of medical treatment.    ¢ Partial gastrectomy Is done including removal of the ulcer  followed by gastroduodenal anastomosis. It has the least  recurrence rate of less than 1% but mortality rate is around  1-2%.    Fig. 23.23: Posterior GJ (Mayo)' with vagotomy—motility of  the stomach is lost after vagotomy. Hence, drainage procedure (GJ  Pyloric in this picture) is required (vagus is secretomotor of the stomach)  crow foot  Fig. 23.22: Highly selective vagotomy—pyloric crow foot is  preserved. Hence, drainage procedure is not required   gastroduodenal artery can be Incision extended  easily done with this operation into the first part of  duodenum P  toma  Figs 23.24A to C: Steps of Heinecke-Mickulicz pyloroplasty Fig. 23.25: Finney pyloroplasty    ¢ It 1s indicated when the gastric ulcer 1s located on the  lesser curvature. Here the gastrectomy is done below the  ulcer and remnant of the stomach is anastomosed to a  jejunal loop (gastrojeyunal anastomosis). This is also  described as Polya gastrectomy.    3. HSV with excision of the ulcer can be done if the  experience of surgeon is good.    ¢ The type 4 gastric ulcer presents a difficult management  problem.   The surgical treatment depends on the ulcer size, distance  from the GE junction and the degree of surrounding  inflammation.   The most aggressive approach Is to perform a gastrectomy  that includes a small portion of the oesophageal wall and  the ulcer followed by a Roux-en-Y oesophago-gastro-  jejunostomy to restore intestinal continuity.   For type 4 gastric ulcers that are located 2 to 5 cm from the  gastroesophageal junction, a distal gastrectomy with a  vertical extension of the resection to include the lesser  curvature with the ulcer can be performed (1.e. Pauchet  procedure). After resection, bowel continuity is restored  with an end-to-end gastroduodenostomy.   The Csendes procedure may be useful in stable patients  or the Kelling-Madlener operation in unstable ones  (Fig. 23.29).   Some have even advocated leaving the ulcer in place or  locally excising it In conjunction with truncal vagotomy  and pyloroplasty.    More common in males. The ratio is 8-10 men to 1 woman  in India.   Anterior duodenal ulcer perforates and posterior  duodenal ulcer bleeds. An ulcer on the posterior wall of  the stomach can perforate into the lesser sac.   Usually, patients with a long history of peptic ulcer,  suddenly complain of feeling something that has given way  in their abdomen. It may be precipitated by excessive  smoking alcohol, drugs, etc. Rarely a ‘silent’ ulcer can also  perforate (especially those patients treated with cortisone).  Patients taking NSAIDs (often elderly) can present less  dramatically.   Two factors are associated with most perforated peptic  ulcers: Chronic use of NSAIDs and Helicobacter pylori  infection.   Patients with H. pylori infection and perforated peptic  ulcers tend to be younger, with a male preponderance  and more prolonged period of dyspepsia. However, NSAIDs  category of patients are elderly with equal proportion of  both sexes being affected.    Perforated peptic ulcers have a mortality rate of 5—10%.  Perforated gastric ulcers in the elderly have 20-30%  mortality rate.    Immediately after the perforation, gastric and duodenal  contents leak into the peritoneal cavity and produce severe  agonising pain in the right hypochondrium. It is mainly HCl  which produces pain.   There may be an episode of coffee-ground vomitus,  followed by melaena later.   The pulse rate increases. The patient 1s pale and anxious.  Blood pressure may be normal in the initial few hours.  Per abdomen, there is guarding and rigidity of the abdominal  wall.   Rebound tenderness is present all over the abdomen. This  sign 1s called Blumberg’s sign.   On percussion, liver dullness is obliterated because of  collection of free air (gas) under the right dome of  diaphragm. This stage is seen for about 2—4 hours from the  time of perforation.    ¢ The peritoneum reacts to the chemical irritants by secreting  peritoneal fluid. As a result of this, HCI and bile are diluted  by the peritoneal secretions (reaction of peritoneum to the  insult) resulting in an improvement of symptoms. Hence,  it is also called stage of delusion or stage of illusion. This  stage lasts for 3—6 hours. However, the signs are worse.   — Evidence of dehydration due to loss of fluid into  peritoneal cavity.    ¢ The peritoneal contents get contaminated with gram-  negative organisms resulting in bacterial peritonitis (the  organisms are from the intestine itself and not from the  peritoneum).   ¢ The patient becomes severely ill, dehydrated, toxic with  drawn in cheeks. The tongue is dry and coated but with  bright eyes (Hippocratic facies, Fig. 23.31).   ¢ Features of hypovolaemic and septicaemic shock such as  feeble thready pulse, cold peripheries, shallow respiration,  high grade fever and persistent hypotension are present.  Gross abdominal distension, guarding, rigidity, abdominal  tenderness all over suggest generalised peritonitis.   e Plain X-ray chest or abdomen in erect position shows  collection of free gas under the right dome of diaphragm,  in majority of cases. If patient is unable to stand, left lateral  decubitus films are taken (Figs 23.32 and 23.33).   ¢ When in doubt, request a CT scan with contrast which can  demonstrate pneumoperitoneum, fluid in the abdomen, site  of perforation and some surprises also.   Fig. 23.32: Plain X-ray abdomen erect showing collection of free  gas under the right dome of the diaphragm   Fig. 23.33: Plain X-ray abdomen lateral decubitus showing  collection of free gas under the abdominal wall    A Aspiration of stomach contents with Ryle’s tube to reduce  further contamination and to decrease biliary and pancreatic  juice.   C Charts: Temperature, pulse, BP, respiration, urinary output  (urinary bladder is catheterised using a Foley’s catheter).    'It resembles a strike by employees paralysing the work of a factory in response to an insult.  Students should remember that ABCDEF are the basic principles of treatment of any acute abdomen. In majority of cases of acute abdomen,   ¢ Cephalosporins can also be used depending upon the  severity of the shock.   E Exploratory laparotomy is done through a midline incision.  The perforation 1s identified and closed with interrupted  nonabsorbable silk sutures, which is strengthened by  placement of omentum (Fig. 23.34). Peritoneal toilet/wash  is given to avoid residual abscess. Abdomen 1s closed with  a drain which 1s removed after 3—5 days. If it is a large  gastric ulcer, it is better to do a gastrectomy, if condition of  the patient permits.    ¢ Vagotomy and GJ is not done at this stage as the general  condition of the patient is very poor and there Is peritoneal  sepsis.   ¢ Anendoscopy is done after 2 months. If the ulcer persists,  it is likely to be a chronic ulcer and an elective operation  such as vagotomy and GJ is done. Simple suturing cures  majority of acute ulcers.   F Fluids are given preoperatively to treat dehydration and  postoperatively for 3-4 days till the paralytic ileus settles  down (soft abdomen and bowel sounds present).   It can also be managed by laparoscopic closure of perforation  with peritoneal drainage. In fact, a thorough wash is really  possible with a laparoscope.   Perforation with abscess. Some patients present late to the  hospital with features of sealed perforation.    ¢ Haemorrhage from peptic ulcer can be chronic which causes  anaemia, or acute, resulting in massive haematemesis and  melaena.    ¢ It is the posterior duodenal ulcer which commonly  bleeds, because it erodes into the gastroduodenal artery  which runs posterior to the duodenum. A gastric ulcer on  the lesser curvature erodes into one of the branches of left  or right gastric artery.    1. Chronicity, results in destruction of the layers of the  stomach, exposing the vessel.   2. Sudden, severe acid peptic digestion brought about by  irritants such as alcohol, drugs, etc.    3. Atherosclerosis: Sclerotic artery does not contract,  resulting in massive haemorrhage.    Characteristics of individuals at an increased risk  of developing acute GI bleeding    Clinical features of bleeding peptic ulcer  1. Previous history of abdominal pain of peptic ulcer disease.  2. History of haematemesis or melaena (black tarry stools),  one or more attacks.  3. There may be features of haemorrhagic shock such as  feeble, thready pulse, hypotension, syncope.  e Oliguria, due to inadequate renal perfusion.  ¢ Brainstem hypoxia results in change in rate and depth  of respiration.    e There may not be any abdominal signs. However, due  to accumulation of blood in the intestines and stomach,  mild distension may be present. Perforation produces  abdominal signs and haemorrhage produces  systemic signs.    The following are some of the factors associated with  increased morbidity and mortality (Key Box 23.11).   e 2% ethanolamine, a sclerosant causes dehydration and  shrinkage of surrounding tissues.   la Spurting, bleeding ¢ This is the most popular method. The success rate 1s  ID NODSPOMUTD: active bleeding around 80-90%. It is a cheap and easy treatment.    As previously mentioned, endoscopy provides the  opportunity not only for diagnosis but also for therapy.    e Emergency upper OGD ts done to confirm the diagnosis. If  the source cannot be detected due to large clots or massive  bleeding, it can be repeated a few hours after a stomach  wash and blood transfusion.    of bleeding depending upon condition of the patient. 4. Bipolar electrocoagulation—failure rate is 50%  e Resuscitation is more important than an urgent endoscopy. - Surgical eradication of H. pylori prevents rebleeding.  e Since elderly patients cannot tolerate shock well, decision    2. Ryle’s tube is passed and cold saline stomach wash Is given  to produce vasoconstriction.  3. Cold antacids are given every 2nd hourly, about 10—20 ml.  4. IV ranitidine 50 mg, 8th hourly or IV pantoprazole 40 mg  1s given to reduce acidity.  ¢ Majority of cases respond to conservative line of  management within 48 hours.    2. Rebleeding in the hospital (rebleeding 1s more common in  gastric ulcer patients).   3. Bleeding requiring transfusion of more than 2000 ml blood  in 24 hours (6 units).    1. Laser coagulation  ¢ It can arrest the bleeding without direct tissue contact. KEY BOX 23.13  Nd:YAG laser has been used more commonly because it  can penetrate tissue more deeply compared to argon laser ENDOSCOPIC PROGNOSTIC FACTORS  which penetrates very superficial tissues. The success ° Visible level: 40-60% rebleeding ulcer > 2 cm   2. Sclerotherapy (Key Boxes 23.12 and 23.13)  ¢ Epinephrine (1:10,000) arrests bleeding by vaso-  constriction.    Surgery for bleeding duodenal ulcer  ¢ Laparotomy and anterior gastroduodenotomy.  ¢ Visualise the bleeding ulcer in the first part of duodenum    ¢ Under-running of the ulcer base by direct suture or 4  quadrant ligation of gastroduodenal artery by using  nonabsorbable sutures (Fig. 23.37).    ¢ Gastroduodenotomy incision is converted into a  pyloroplasty followed by vagotomy which completes  the treatment.    Under-running of the ulcer base. There are chances of  rebleeding with this method.    Partial gastrectomy is the best treatment provided general  condition of the patient is good (Figs 23.38 and 23.39).  Otherwise, local excision of the ulcer, vagotomy followed  by GJ or pyloroplasty can also be done.    Haemostatic methods currently employed include  thermotherapy (heater probe, multipolar or bipolar  electrocoagulation) as well as injection of ethanol or  epinephrine solutions.    When the bleeding 1s controlled, long-term medical therapy  includes antisecretory agents, usually in the form of a proton  pump inhibitor, in addition to testing for H. pylori with  treatment if positive.    If H. pylori is present, documentation of eradication should  be performed after therapy.   Haematemesis refers to vomiting of fresh red blood.  Melaenemesis refers to vomiting of dark altered blood.  However, both are included under upper GI tract bleeding.  Small bowel, even though is a midgut structure, some of its  lesions can produce haematemesis. Hence, they are also  included under this heading.    Acute erosive gastritis, chronic peptic ulcer and  oesophageal varices constitute almost 90% of the cases.    ¢ Fibreoptic endoscopy should be done on an emergency  basis within 6 to 36 hours of admission. It can diagnose  variceal bleeding, erosive gastritis, bleeding peptic ulcer,  carcinoma stomach, etc.    ¢ Only when endoscopy cannot yield any diagnosis, selective  coeliac angiography should be done which can detect  uncommon causes such as angiodysplasia of the stomach  or bleeding from a rolling hernia, etc. Barium study is done  to rule out intestinal causes in less urgent cases.    ¢ Isotope studies: Intravenous injection of "Tec  pertechnetate can demonstrate hypertrophic gastric like  mucosa in Meckel’s diverticulum.   ¢ CT angiogram is the gold standard investigation when  haemobilia or haemosuccus pancreatitis 1s suspected  (Figs 23.42 to 23.44).    Initial management Is to treat the shock in the line discussed  for peptic ulcer haemorrhage.    Fig. 23.41: Investigation algorithm of UGI bleeding (Courtesy: Dr Sibacis Bisoi, Asst Professor, KMC, Manipal)   Figs 23.42 and 23.43: CT hepatic angiogram demonstrating extravasation of Fig. 23.44: Massive haematemesis in this patient  dye into a pseudoaneurysm of a branch of middle hepatic artery and thrombus— of haemobilia  case of haemobilia    Fig. 23.46: Chronic duodenal ulcer with massive UGI bleeding.  Diagnostic use of nasogastric tube is to detect active bleeding   Moderate haemorrhage (SOO—1000 ml of blood)  Mild haemorrhage (less than 500 ml of blood)    1. Elderly patients with rebleed in the hospital  2. Rarity of blood groups  3. Spurting vessel in an endoscopy    ¢ Surgical management of individual case has been discussed  along with the concerned chapter. However, summary of  the treatment is discussed in Table 23.7.   Causes Conservative a  1. Acute erosive gastritis Yes  2. Chronic duodenal ulcer Yes  3. Chronic gastric ulcer Yes  4. Mallory-Weiss syndrome Yes  5. Cancer of the stomach No  6. Duodenal polyp Endoscopic snaring  7. Haemobilia Yes, therapeutic embolisation  8. Variceal bleeding Yes, sclerotherapy  9. Haemosuccus pancreatitis Yes, therapeutic embolisation  10. Dieulafoy lesion Yes, injection sclerotherapy    Thus, upper GI bleeding can occur due to various causes.  However, acute erosive gastritis, chronic peptic ulcer and  oesophageal varices are the three important causes of  bleeding. Endoscopy is the investigation for the diagnosis of  upper GI bleeding (Fig. 23.52). Today, most of the upper GI  bleeding is managed either in the form of injection  sclerotherapy, laser coagulation, or with powerful H, blockers  or proton pump inhibitors. In appropriate cases, surgery 1s  definitely indicated. Some common conditions which give  rise to haematemesis are discussed in the following pages.   Surgery, if endoscopic facility not available  Ligation of feeding vessel  Devascularisation    They are also called acute erosive gastritis/acute stress ulcers/  acute steroidal ulcers and Acute Gastric Mucosal Lesions  (AGML).   ¢ Following hypotension and shock, there is hypoperfusion,  which results in mucosal ischaemia and acute stress ulcers.  Example: Myocardial infarction, trauma, sepsis.    There is a diffuse mucosal damage and disruption of gastric  mucosal barrier. Reflux of bile also may be a precipitating  factor. This results in acute erosions when they are | to 2 mm  or acute ulcers when they are | to 2 cm in size, shallow and  well-demarcated. The entire stomach is involved by these  ulcers.   ¢ Acute abdominal pain due to acute erosions or perforation  of an acute ulcer.    e In ICU: Drop in blood pressure, nasogastric aspirate o  blood and/or melaena.    Diagnosis  Emergency endoscopy to confirm diagnosis and to rule ou  other causes of upper GI tract bleeding.    1. Admission in intensive care unit (ICU) (Key Box  2 1D):    2. Ryle’s tube to be introduced first and empty the  stomach. It helps in reducing vomiting and sometimes  aspiration of gastric contents into the lungs also. Ice  cold water or saline stomach wash (vasoconstriction)  reduces bleeding.    5. Injection ranitidine 50 mg IV, 8th hourly or pantoprazole  20 mg IV. Majority of cases respond to conservative line  of treatment.    7. Surgery: With modern investigations and their therapeutic  role in addition to good ICU care, availability of blood and  nursing care, almost all cases of erosive gastritis can be  managed without surgery. In spite of above treatment if the  condition deteriorates, a total gastrectomy or a subtotal  gastrectomy may be necessary (very, very rare) as a life-  saving measure.    e It occurs due to a tear in the gastric mucosa near the  oesophagogastric (OG) junction.   ¢ In Boerhaave syndrome, all layers are involved in a tear.   ¢ The patient is usually a middle-aged male who, after  consumption of alcohol, vomits the food contents first.  During the course of vomiting because of straining and   Fig. 23.53: Mallory-Weiss tear—with the endotherapy bleeding  stopped (Courtesy: Dr Filipe Alvares, Gastroenterologist, KMC,  Manipal)    retching, a tear develops near the oesophagocardiac  junction. Hence, the second vomitus contains blood.    ¢ Sometimes, the bleeding can be so massive to produce  hypotension and shock. In 90% of cases, the bleeding stops  spontaneously.    ¢ Endotherapy—injection adrenaline 1:10,000 dilution is  effective. If not, haemoclipping can be done.    e In this condition, an unusually large artery runs in the sub-  mucosa which lies 1n close contact with mucous membrane  (Fig. 23.54).    ¢ Inmore than 80% cases, bleeding  occurs within 6 to 8 cm from OG  junction, at the lesser curvature.    ¢ Endoscopy and endotherapy  should be tried and if necessary,  repeated also.   ¢ Sudden, massive, painless,  recurrent haematemesis with  hypotension is the presenting  feature.   ¢ Angiography to confirm the  bleeding lesion and gelfoam  embolisation must be tried first.   ¢ Failure to achieve control of the  bleeding—gastrotomy and  wide excision of the lesion  Should be done.    ¢ It is an uncommon cause of chronic GI bleeding resulting  In iron deficiency anaemia.    ¢ It is also called watermelon stomach because of the  presence of long red streaky area which are present in the  stomach resembling a watermelon (Key Box 23.17).    A small perforation of peptic ulcer which is sealed off by  omentum may result in a residual abscess in one of the  subphrenic spaces. It responds to conservative treatment.  Otherwise, percutaneous drainage can be done with  ultrasonographic guidance.   WATERMELON STOMACH  (GASTRIC ANTRAL VASCULAR ECTASIA)  e Women in their 50s are commonly affected    Earlier it was called pyloricstenosis. However, gastric outlet  obstruction is a better word. Chronic cicatrisation of a  duodenal ulcer or juxtapyloric ulcer results in narrowing of  pyloric antrum which ts described as pyloric stenosis. In India,  pyloric stenosis is more common in South Indian patients, who  usually present with a long history of duodenal ulcer and a recent  history of vomiting (Key Box 23.18).   PYLORIC STENOSIS  e Pyloric stenosis in CDU—Misnomer  e Stenosis is very often found in the first part of duodenum  e Incases of pyloric channel ulcer, true pyloric stenosis occurs    e Metabolic changes, such as paradoxical aciduria (see below)  are usually seen in patients with ulcer, not in carcinoma  because of relative achlorhydria in the latter.   ¢ Classical hunger pain of duodenal ulcer disappears. It may  be replaced by a dull aching pain because of gastric distension.  Colicky pain 1s due to hyperperistalsis of stomach.   ¢ Vomiting is profuse, projectile, persistent, foul-smelling  (because of stasis) and nonbilious.   ¢ There may be distension of upper abdomen with epigastric  fullness.    Signs  1. Visible gastric peristalsis (VGP): Stomach that you see  ¢ Itis a wave of contraction of the stomach which starts in  the left hypochondrium, runs across the umbilicus and  ends in the right hypochondrium. These contractions can  be felt—stomach that you feel. Presence of VGP is  diagnostic of pyloric stenosis (right to left peristalsis is  seen in left-sided obstructive colonic tumours). If VGP  is not seen, it can be made prominent by:  ¢ Asking the patient to drink at least 5|00—1000 ml of water  (It is difficult. Try and see!).  ¢ Stimulating the abdomen by flicking movement.  2. Succussion splash  ¢ Should be done on ‘fasting’ stomach. This test should be  done before asking the patient to drink water.  ¢ In pyloric stenosis there is always residual fluid in the  stomach, which gives a splashing sound that can be heard  with/without stethoscope—stomach that you hear.    Thus, the stomach which ts seen, felt and heard is  diagnostic of pyloric stenosis.    3. Auscultopercussion test/auscultoscraping test to find ou  the greater curvature of the stomach.  Procedure: Keep the “bell” of stethoscope in the centre o1  epigastrium (ask the patient to hold the bell of steth) and  percuss radially. Percussion over the stomach gives a dull  note because of presence of fluid. When the note changes,  it indicates the greater curvature of stomach. Mark it on the  abdomen (instead of percussion, scraping can be done with  finger nail). A line joining 3 or 4 such marks outlines the  greater curvature of stomach.    Saline load test: 700 ml of normal saline is infused into  stomach over a period of 3.5 minutes through a nasogastric  tube and is clamped. If a volume more than 350 ml can be  aspirated, it indicates obstruction.    Investigations  1. Barium meal X-ray  ¢ Hugely dilated stomach (large and low stomach).    ¢ Barium does not enter the duodenum. Barium mixed with  food residue can give rise to mosaic appearance. Delay in  evacuation (on repeat X-ray) of barium into the duodenum  is seen (Fig. 23.56).    2. Gastroscopy  The scope will not enter the duodenum. Stomach is full of  foul-smelling food residue.   ¢ Gastroscopy Is also done to rule out carcinoma of the stomach.   Fig. 23.56: Barium meal showing intrinsic, irregular, persistent fill-  ing defect involving pyloric antrum    decreases oedema of the stomach wall. Stomach wash  should be given for at least 3—5 days before surgery.   B: Blood is arranged for surgery. Blood may be required pre-  operatively to treat anaemia.   C: Charts: Adequate urine output is maintained by infusion of  intravenous fluids.   E:Exploratory laparotomy: Vagotomy followed by GJ is  done. Pyloroplasty should not be done because the  duodenum Is scarred, cicatrised, fibrosed and narrowed.   F: Fluids to correct electrolyte abnormalities. Pyloric stenosis  patients can develop “Hyponatraemic, Hypochloraemic,  Hypokalaemic alkalosis”, Ringer lactate 1s an ideal  supplement.  ¢ Postoperatively these patients recover very fast.    ¢ A long-standing lesser curve gastric ulcer causes shortening  of the lesser curvature due to fibrosis. Such stomach    resembles a tea-pot. As a result of this, the pylorus becomes  nondependent. Hence, stasis occurs.    ¢ When a saddle-shaped ulcer in the lesser curvature gets  cicatrised, it involves both surfaces of the stomach resulting  In conversion of stomach into two compartments.   ¢ Features of stasis such as fullness, distension and persistent  vomiting are present.   ¢ Itis treated by BillrothI partial gastrectomy with removal  of 2nd pouch.    Posterior gastric ulcer can penetrate into pancreas, resulting  in severe referred pain to the back resembling pancreatic  pathology. However, this type of pain 1s relieved on lying down.    ¢ Carcinoma stomach is more common (2 times) in men  compared to women.  ¢ Rare below 40. Average age is 63 years.   2. Duration of the Long duration of abdominal pain and recent Pain is usually absent, but vomiting is a  symptoms history of vomiting feature. Symptoms are of short duration    * The palpable mass is the deciding clinical factor (sign). Other differences cannot truly differentiate between the two conditions. Rarely,  carcinoma of the stomach is also seen in young patients at the age of 20. In congenital hypertrophic pyloric stenosis, the mass is palpable   CARCINOMA PROXIMAL STOMACH (CARDIA)  e Incidence is increasing. Obesity and high socioeconomic  group  e More aggressive  e Thin muscularis mucosa. Hence, submucosal invasion is    e Surgical resection involves oesophageal anastomosis which  is technically demanding. Leak rates are high    ¢ Incidence of proximal gastric carcinoma is increased—may  be due to obesity and tn rich socioeconomic status patients. Gastritis  ¢ Carcinoma distal stomach is more commonly associated  with 7. pylori. Fig. 23.60: Risk factors for carcinoma stomach    ¢ Overall 5-year survival after the diagnosis of gastric cancer The incidence Is increased in persons who consume red    = 10 to 20% meat, cabbage, spices, spirits, salt-fish (Key Box 23.20).   ¢ Smoked salmon fish was responsible for increased incidence  of carcinoma stomach in Japanese population. Probably, it ts  related to release of polycyclic hydrocarbons and aromatic  amino acids. Smoking, spicy food and alcohol consumed over    ¢ Those who undergo potentially curative resection (RO)  have a 5-year survival rate of 25-50%.    A 32-year-old male was admitted with loss of appetite of 3 emg eh  KEY BOX 23.20  months duration. Endoscopy revealed a growth in the body  of the stomach. At exploration, large para-aortic nodes FOOD PRODUCTS WHICH MAY BE  were present. Subtotal gastrectomy was done. He died after CARCINOGENIC  6 months due to extensive metastasis. No wonder, ¢ Smoked food  carcinoma of the stomach is called Captain of men of ° Spirits  death. * Smoking  e Salted food  ° Soil    WHO recommends increased consumption of fruits and  vegetables. Vitamin C, is an antioxidant which its a  protective agent. Ascorbic acid prevents conversion of  nitrates to nitrites.   a. Atrophic gastritis: This may be due to smoking, spicy food,  continuous ingestion of drugs, reflux of bile into the  Fig. 23.59: Site and incidence of gastric cancer stomach, etc.    b. Pernicious anaemia: Patients have increased risk (four to  six times) of development of carcinoma when compared to  general population.  ¢ It causes atrophic gastritis and precipitates carcinoma of   d. H. pylori infection results in atrophic gastritis, followed  by the intestinal type of gastric mucosa, metaplasia and then  dysplasia. Eventually it leads to intestinal type of gastric  cancer (Figs 23.61 and 23.62). H. pylori can also cause  proliferation of gastric cancer cells and decrease secretion  of vitamin C. Cytotoxin associated gene A (Cag A) is  associated with increased risk.    ¢ Also both type A and type B gastritis can predispose to  carcinoma stomach. Type A—proximal stomach, type  B—distal stomach.   ¢ Polyp more than 2 cm, pedunculated polyp can be removed —— :  by endoscopically. Higher chances of malignancy is seen  in sessile polyps.    with giant hypertrophy of gastric mucosal folds. It is a g- Gastric ulcer (benign): Incidence of malignancy is 2%  precancerous condition. (0.5 to 5%). Carcinoma arising in a gastric ulcer is called    h. Previous GJ or gastric resection predisposes to  development of carcinoma of the stomach after a period of  15-20 years. Such a carcinoma is described as stump  carcinoma. Pathogenesis is related to development of  atrophic gastritis, achlorhydria and duodenogastric bile  reflux.    ¢ Carcinoma stomach can run in families. However, only 10%  of patients give family history of carcinoma stomach.    ¢ Carcinoma stomach is more common in patients with blood  group A. These patients have different mucopolysaccharide  secretion in the stomach and greater susceptibility to  ingested carcinogens. They develop diffuse type of  carcinoma.    ¢ Several genetic alterations have been identified such as  ease activation of oncogenes, inactivation of tumour suppressor  6 times) for developing gastric canc genes p53 and p16, reduction or loss in the cell adhesion  us anaemia is an autoimmune gastritis of the molecule E-cadherin (met proto-oncogene).    Fig. 23.61: H. pylori and pathogenesis of duodenal ulcer and 1. Cauliflower like growth with friable tissue. This variety  carcinoma stomach can give rise to melaena or bleeding causing anaemia.   FAMILIAL GASTRIC CANCER o  e Only 10% of patients give family history of carcinoma  stomach   e Diagnosis is made when carcinoma is detected below the  age of 40 years   e E-cadherin mutation results in lifetime risk of gastric cancer  by 60-90%.   e Familial diffuse gastric cancer—(FGC) at least 3 relatives in 2  generations are affected, one of them before 50 years of age.    2. Infiltrative type of lesion (diffuse) with dense submucosal  fibrosis which converts the stomach into a small contracted,  functionless stomach—linitis plastica or leather bottle  stomach (Key Box 23.22). Mucosa may appear normal.    4. Ulcer cancer refers to carcinoma arising in a pre-existing  gastric ulcer. In this variety, complete destruction of the  muscle coat is present.   e The Lauren system separates gastric adenocarcinoma into  intestinal or diffuse types based on histology (Key Box  23.20)   ¢ Other histologic types include squamous cell carcinoma,  adenoacanthoma, carcinoid tumours, GI stromal tumours,  and lymphoma.    ¢ Early gastric cancer is defined as adenocarcinoma limited  to the mucosa and submucosa of the stomach, regardless of  lymph node status.   ¢ The entity is common in Japan, where gastric cancer is the  number one cause of cancer death, and where aggressive  surveillance programs have been established.   ¢ Approximately 10% of patients with early gastric cancer  will have lymph node metastasis.    EARLY GASTRIC CANCER  (JAPANESE CLASSIFICATION)  Type! Exophytic lesion extending into the gastric lumen  Type Il Superficial variant  ll AElevated lesions with a height no more than the  thickness of the adjacent mucosa  li B Flat lesions  li! C Depressed lesions with an eroded but not deeply  ulcerated appearance  Type Ill Excavated lesions that may extend into the muscularis  propria without invasion of this layer by actual cancer  cells    ¢ Approximately 70% of early gastric cancers are well  differentiated and 30% are poorly differentiated.    ¢ The overall cure rate with adequate gastric resection and  lymphadenectomy is 95%.    ¢ Insome Japanese centres, 50% of the gastric cancers treated  are early gastric cancers.    ¢ In the US, less than 20% of resected gastric adeno-  carcinomas are early gastric cancers.    Five-year survival in node negative early gastric cancer is  more than 95%. However, it falls to 70% if nodes are positive.  Hence, the suggestion is that node-positive cases should not  be included under early gastric cancer.   ¢ The Borrmann classification system was developed in  1926 and remains useful today for the description of  endoscopic findings (Fig. 23.63).   ¢ The Borrmann system divides gastric carcinoma into five  types depending on the lesion’s macroscopic appearance.    — Type 4 diffusely infiltrating lesions  — Type S lesions that do not fit into any of the other categories.    ¢ Linitis plastica is the term to describe type 4 carcinoma  when it involves the entire stomach (Key Box 23.24 and  Figs 23.64 to 23.66).    THE DIFFUSE FORM OF GASTRIC ~S  ADENOCARCINOMA—LINITIS PLASTICA  e Poorly differentiated    e Route of spread is generally by transmural extension and  through lymphatic invasion    degree of cellular differentiation, independent of  morphology, and ranged from | (well-differentiated) to 4  = (anaplastic).    e The prognosis is less favourable for patients with diffuse-  subtype histology.    Very often patients would have vague symptoms—  early satiety, flatulence, discomfort, pain in the upper  abdomen.   Early satiety 1s due to decreased distensibility of the stomach  (Fig. 23.67).   Silent: Growth is silent but manifests as secondaries in the  liver, ascites, Virchow’s node, rectovesical deposits,  (Blumer’s shelf), umbilical nodule (Sister Mary Joseph’s  nodule), left axillary nodes (Irish nodes), palpable ovarian  mass (Krukenberg tumour) (Figs 23.67 to 23.72).  Obstruction at pylorus (pyloric antrum) producing pyloric  obstruction with features of vomiting with/without blood.  Visible gastric peristalsis can also be present. Obstruction  at cardio-oesophageal junction produces dysphagia.  Lump inthe abdomen which ts hard and irregular. Clinically,  stomach mass is differentiated from liver mass by features  mentioned below.   Dyspepsia in a man over the age of 40: Carcinoma stomach  should be ruled out. Early gastric cancer presents as dyspepsia.    Nonmetastatic conditions such as thrombophlebitis  (Trousseau’s sign) and deep venous thrombosis can occur due  to change in thrombotic and haemostatic mechanisms.    ANAEMIA IN CARCINOMA 8  This is one of the common presentations. Often patients get  investigated for anaemia by the physician only to discover  Carcinoma stomach.  Achlorhydria results in poor conversion of ferrous to ferric  which causes anaemia.  15% of patients may also develop haematemesis as in  ulcerative lesions or proliferative lesions. GI blood loss also  accounts for anaemia.  Early satiety, loss of appetite and poor intake also contribute  to anaemia (minor role).  40% of carcinoma stomach patients have anaemia.   Fig. 23.67: Carcinoma stomach large ulcerative lesion with  involvement of serosa    . Stomach moves with respiration.  . Upper border of the stomach mass can be made out.  . Anatomical location of the mass: Right hypochondrium in    a pyloric mass, epigastrium and left hypochondrium in a  mass arising from body of the stomach.    1. Penetration of gastric serosa: This is the most important  prognostic indicator. When serosa 1s NOT penetrated, 50%  survive for 5 years after resection. When serosa 1s  penetrated, this figure drops to 20%.    ¢ Once serosa is involved, adjacent organs such as liver,  pancreas, spleen, omentum, transverse colon get involved.  2. Lymphatic spread: 420 lymph nodes have been identified  ¢ Lymph node involvement is a poor prognostic indicator.   ¢ Zone 1: In the gastrocolic omentum along the right  gastroepiploic vessels. This drains the pyloric antrum and  lower half of greater curvature.   Tis Carcinoma in situ. Intraepithelial tumour without  invasion of the lamina propria    Tla Tumour invades lamina propria or muscularis mucosa  T1b Tumour invades submucosa   T3 Tumour penetrates subserosal connective tissue  without invasion of visceral peritoneum or adjacent  structures    adjacent structures  T4a Tumour invades serosa (visceral peritoneum)  T4b Tumour invades adjacent structures    NX Regional lymph nodes (S) cannot be assessed  NO No regional lymph node metastasis   N3 Metastasis in 7 or more regional lymph nodes  N3a Metastasis in 7 to 15 regional lymph nodes    N3b Metastasis in 16 or more regional lymph nodes  Distant metastasis (M)   ¢ Zone 2: It lies in the gastrocolic omentum and gastrosplenic    ligament along the left gastroepiploic vessels. This drains  upper half of the greater curvature.    two-thirds of the stomach. From here, lymph drains into  perioesophageal lymph nodes.    ¢ Zone 4: It is from distal portion of the lesser curve and    pylorus along hepatic artery and right gastric artery into  para-aortic nodes.    3. Blood spread: Most common sites are liver and lungs. It    Investigations Laparoscopy is an ideal investigation. Almost 20 to 30Y  of so-called operable cases become inoperable. Laparosopi    1. Complete blood picture: 20% of early gastric cancer : : ,  peritoneal lavage for cytology is best test.    anaemia. Preoperative blood transfusion may be necessary. 7- Barium meal may show intrinsic, persistent, irregular, filling  defect. Double contrast air-barium study is used for mas:    screening in Japan to detect early cases (Figs 23.77 to 23.79)  ¢ Barium meal study is useful in cases of linitis plasticz    3. Flexible oesophagogastroduodenoscopy (Figs 23.73 to wherein mucosa may appear to be normal in early cases  2 ©)    2. Routine examination, fasting and postprandial sugars, ECG,  renal function for fitness before surgery.    ¢ Today use of barium has become almost nil with the  availability of endoscopy.    8. CEA: Carcinoembryonic antigen is elevated in about  60—70% patients. It indicates extensive spread of the disease.    e Itis an adenocarcinoma of the stomach. There are basically  two types of gastric carcinomas as per Lauren’s classification.   ¢ Diffuse is more common in young, females and carries poor  prognosis. The leather-bottle stomach or linitis plastica 1s  poorly differentiated with anaplastic cells.    Fig. 23.73: Endoscopy showing Fig. 23.74: Endoscopy showing of intestinal metaplasia. It has better prognosis.  superficial ulceration ulceroproliferative growth    ¢ To know the extent of the lesion  ¢ To confirm the diagnosis  ¢ To take multiple biopsy—6 pieces  ¢ Also to aid brush cytology  4. Ultrasound and CT scan  ¢ To rule out secondaries in the liver.  ¢ To look for enlarged coeliac nodes.  ¢ Can detect ascites—guided fluid tap and cell cytology.  ¢ To detect Krukenberg tumour (pelvic CT).  ¢ Useful in detecting metastatic disease.  5. Endoscopic ultrasonography can differentiate early gastric  cancer from advanced tumours in 80% of patients. Overall  staging accuracy 1s about 75%.    Surgery is the main modality of the treatment. Adjuvant  chemotherapy has been found to be beneficial in a few  patients only.   Inoperable means there are no chances of cure but growth  may be resectable. Operable means cure is possible.   ¢ Rectovesical deposits, due to peritoneal seedings which  are felt during per rectal examination   ¢ Enlarged, fixed coeliac nodes, para-aortic nodes and left  supraclavicular nodes   Results from spread of tumour within the falciform ligament  90% of tumours are adenocarcinomas    Bypass procedure (GJ) to relieve vomiting in advanced cases  of pyloric obstruction.    ¢ There is no evidence of microscopic or gross residual  tumour.   ¢ Serosa is not involved (this means that curative resection 1s  not possible for T3/T4 tumours).    e Japanese Research Society for gastric cancer advocates very  aggressive resection including lymphadenectomy (Fig. 23.80).  Hence, more details of lymph node station are given in  Table 23.9. However, the rest of the cancer research groups  were not able to produce the same results as the Japanese.   ¢ D1 resection refers to the removal of primary group of nodes  such as nodes along the lesser and greater curvature, and  juxtapyloric nodes. This will become curative resection  when lymph nodes are clinically not enlarged (NO) but  they are removed (Fig. 23.81).   ¢ D2 resection refers to the removal of lymph nodes such as  left gastric, common hepatic, splenic, retropancreatic nodes,  etc. This will become curative resection when lymph  nodes are clinically enlarged (N1) (Key Box 23.26 and  Fig. 23.82).   ¢ D3 resection refers to the removal of lymph nodes such as  para-aortic, porta hepatis nodes, behind the head of the  pancreas, etc. (not done).    ¢ The extent of the gastrectomy is site-dependent and focuses  on complete removal of the gastric carcinoma with  preferably a6cm margin from the gross edge of the tumour.   Fig. 23.80: Perigastric nodes: (1) right pericardial; (2) left  pericardial; (3) lesser curvature; (4) greater curvature;  (5) suprapyloric; (6) infrapyloric   (number) Description Upper third Middle third Lower third  l Right paracardial : | 2  2 Left paracardial I 3 M  3 Lesser curvature | l ]  4sa Short gastric | 3 M  Asb Left gastroepiploic | | 3  4d Right gastroepiploic e l |  5 Suprapyloric 3 | |  6 Infrapyloric 3 | l  Zi Left gastric artery 2 2 2  8a Anterior common hepatic 2 2 2  8p Posterior common hepatic 5 3 3  9 Coeliac artery Z 2 2  10 Splenic hilum 2 a M  llp Proximal splenic 2 2 2  I1d Distal splenic Z 3 M  L2 Hepatoduodenal 3 vi 2  13 Retropancreatic M 3 3  14v Superior mesenteric vein M 3 2  l4a Superior mesenteric artery M M M  15 Middle colic M M M  16al Aortic hiatus 2 M M   Fig. 23.82: D2 resection of tumours of the proximal third of  stomach    Fig. 23.81: Extent of D1 resection of tumour in distal one-third of  stomach    ¢ Clearly, anatomic limitations influence this margin because  in antral lesions close to or involving the pylorus, only a »  limited portion of the duodenum can be removed.   ¢ In patients with a distal lesion, essentially a distal subtotal  gastrectomy 1s performed regardless of T stage.    For proximal gastric cancers (fundic), total gastrectomy  1S required.  For midbody or more extensive lesions, total gastrectomy  is required.   Gastric cancer has a great propensity to spread through the  rich plexus of lymphatics of the stomach to local or regional  lymph nodes. Hence, the need to remove stomach and lymph  nodes.  Gastric cancer, unlike breast cancer, remains for a long  time as a locoregional disease.  It is striking too, that when gastric cancer recurs it often does  so locoregionally rather than more wide-spread  dissemination.  It is very rare for gastric cancer to recur 5 years after  surgery, whereas for breast cancer, disseminated  micrometastases continue to take their toll some 10—20 years  after surgery.    For more distal lesions, a subtotal gastrectomy is the  preferred approach.    Extended organ resection is reserved for patients with  apparently node-negative T4 lesions, in which complete  resection requires resection of the invaded portions of the  diaphragm, pancreas, spleen, adrenal gland or colon.    Generally these are grouped into level NI (i.e. stations  3 to 6),    Level N2 (i.e. stations 1, 2, 7, 8 and 11) and  Level N3 (i.e. stations 9, 10 and 12) nodes.    The nodal stations defined as level NI, N2, and N3 vary  depending on the location of the tumour.    ¢ In general, N1 nodes are within 3 cm of the tumour.  ¢ N2 nodes are along the hepatic and splenic arteries.  ¢ N3 nodes are the most distant.    A tumour is considered resectable if confined to stomach  or NI or N2 nodes are involved   Nodes greater than 3 cm from tumour = N2 nodes  Iftumourand NI nodes (Group |) resected= DI! gastrectomy  Iftumour and N2 nodes (Group 2) resected = D2 gastrectomy  D3 resection is a D2 resection plus removal of para-aortic  lymph nodes.    1. Removing part or whole of the stomach.  2. The NI (groups 1-6) and N2 (groups 7—11) lymph nodes.    . The greater and lesser omentum.  . The spleen and tail of the pancreas for tumours of the    proximal stomach in order to remove groups 10 and 11  lymph nodes. Otherwise, they are preserved.    omental bursa.  The hepatoduodenal nodes in antral tumours.  The splenic artery nodes    The term R status was first described by Hermanek in 1994  and is used to describe the tumour status after resection  (Key Box 23.27).    RO describes a microscopically margin-negative resection,  in which no gross or microscopic tumour remains in the  tumour bed.   Ri indicates removal of all macroscopic disease but  microscopic margins are positive for tumour.    R2 indicates gross residual disease. Since the extent of  resection can influence survival, some authors include this  R designation to complement the TNM system.    Long-term survival can be expected only after a RO resection;  therefore, a significant effort should be made to avoid R1 or  R2 resections.   If no disease is identified in the lymph nodes, (NO)  nomenclature is used.    Endoscopy has revolutionised the diagnosis of carcinoma  stomach. Early diagnosis should be the aim since early  carcinoma stomach is curable.   Endosonogram detects various layers of the stomach which  are involved by the tumour.   Resection is the best line of treatment which can be curative  in a few patients.   Any radical surgery will involve wide excision of the tumour  with lymph nodes and omentum.   Because of added morbidity and a very small survival  advantage of D2 resections over D1 resections, many still  consider D1 resection as adequate.   As far aS we are concerned, an understanding of these  various terminologies and their concepts may enable you  to score better marks in theory papers.   A simple summary of surgical procedures (what everyone  should know) has been given here.    1. Right paracardiac 5. Suprapyloric  3. Lesser curvature _ 6. Infrapyloric  4. Greater curvature 7. Left gastric    1. Carcinoma of pyloric antrum and distal body of  the stomach    Radical subtotal gastrectomy which includes the removal of  60—70% of the stomach, greater omentum along with enlarged   lymph nodes (N1) followed by gastrojejunal anastomosis is  the treatment of choice (Figs 23.92 and 23.93).   Figs 23.92 and 23.93: Radical subtotal gastrectomy with Billroth II  anastomosis   Oesophagogastrectomy (Figs 23.94 and 23.95): Removal of  the entire stomach, lower end of oesophagus, with regional  lymph nodes, followed by oesophagojejunal anastomosis.    1. Carcinoma pyloric antrum (inoperable): Palliative anterior  GJ is done to relieve vomiting, by anastomosing a jejunal  loop to the stomach in the prepyloric region. If posterior  GJ is done, the growth may involve the GJ stoma early  resulting in stomal obstruction. With anterior GJ, entero-  enterostomy can be added to prevent bilious vomiting.   2. Palliative gastrectomy to get rid of ulcerated, necrotic or  bleeding lesion.    ¢ This is indicated in early gastric cancer confined to mucosa.  The cancer should be less than 2 cm and there should not  be node enlargement.   e Stomach is suitable for endoluminal surgery because it can  be distended and contents are sterile.   4. Radical refers to removal of lymph nodes, fat, fascia greater  and lesser omentum.   5. When level 1 to 6 (N1) nodes are removed along with  stomach, it is D1 gastrectomy   6. When level 7-11 (N2) lymph nodes are also removed along  with 1-6 nodes and stomach, it is called D2 gastrectomy.   7. Early gastric cancer without lymph nodes can be treated  with endoscopic mucosal resection or endoluminal gastric  resection.    ¢ Now it is understood that gastric cancers partially responc  to chemotherapy—in about 30% of cases given at advancec  stage (results are better than cancer colon). Injection 5-FL  (fluorouracil) 500 mg IV daily for five days, every 28 days.  It can be given by IV infusion or IV bolus over 15 minutes.   ¢ Mechanism of action: It is an antimetabolite and acts by  interfering with DNA synthesis. Side-effects are  myelosuppression, mucositis, excessive lacrimation, nausea.  vomiting, etc.   ¢ Combination of 5-FU with adriamycin, mitomycin and  cisplatin has also been tried. However, toxicity is more with  these drugs. FAM (5-fluorouracil, adriamycin and  mitomycin C) and ECF (epirubicin, cisplatin and 5-FU) are  popular agents.   A. RO resection (free margins): Any tumour greater than T2  including selected T2 cases should be considered for  adjuvant therapy that includes | cycle of 5-FU+/—  leucovorin OR capecitabine chemotherapy ——~>  followed by 5-FU+/— leucovorin OR capecitabine based  chemoradiation ———~ 3-4 more cycles of 5-FU+/—  leucovorin OR capecitabine chemotherapy.   B. R1| (microscopic margin positive) and R2 (grossly  positive margins) regardless of the TMN stage should  receive chemoradiation followed by further cycles of  adjuvant chemotherapy.    ¢ Chemotherapy alone may also be used as a neoadjuvant  modality.    Metastatic gastric cancer:  ¢ Trastuzumab (Herceptin) can be used in metastatic or  locally advanced gastric adenocarcinomas which are  overexpressing HER-2 neu _ receptors (recent  recommendation)  ¢ External beam radiotherapy can also be used in metastatic  cases as a part of palliation, especially when there is tumour  2,  A 37-year-old female was admitted with vomiting, early  satiety and abdominal pain of 20 days duration. Clinical  examination revealed anaemia. No mass was palpable. UGI  scopy revealed an ulcer in the lesser curvature and biopsy  was reported as chronic gastritis—to rule out H. pylori  infection. Ultrasound showed diffuse wall thickening (8-10  mm) in the anterior and posterior walls of the stomach.  Barium follow revealed hour glass contracture probably  secondary to chronic gastric ulcer. At surgery, wide stomach  was thickened. Total gastrectomy (D-1) was done. Specimen  when opened, revealed extensive infiltrative cancerous tissue.  Final report was diffuse infiltrative adenocarcinoma. In a  doubtful ulcer, investigate thoroughly and if necessary, do  a laparoscopy/laparotomy.   10 fractions over 2 weeks. Fig. 23.98: Alarge GIST arising from the fundus of the stomach  presenting as a nodular mass in the left hypochondrium resected  successfully along with spleen   ¢ In gastrointestinal tract, stomach is the commonest site  of GIST.   ¢ Bleeding is the commonest presentation. It occurs due to  ulceration of mucosa which on endoscopy gives  appearance of cervix (Fig. 23.97).   e Larger tumours can present with dysphagia, weight loss,  anaemia and palpable mass (Fig. 23.98).   ¢ CT scan is a useful investigation (Figs 23.99 and 23.100). .    Capsule Imatinib 100 mg 4 capsules—to a total of 400 mg per  day is given till toxicity appears or metastasis or residual  tumour regresses (investigated by CT scan). This 1s a promising  new drug.   ¢ They are B-cell derived from mucosa associated lymphoid  tissue (MALT)—MALTOMA.   ¢ Endoscopic features are not specific but diffuse thickening  with or without ulcerations may be seen.    Low-grade lymphoma associated with H. pylori infection  may regress and totally disappear after eradication  treatment for H. pylori.    Can be classified as complications of vagotomy,  complications of GJ and complications of gastrectomy.    . Stasis of food in the stomach resulting in nausea, loss of  appetite, distension of upper abdomen, foul eructation, etc.   . Vagotomy produces hypoacidity which allows bacterial  proliferation. Nitrates are reduced to nitrites which are  carcinogenic. Such a malignancy which develops at GJ site  is called stump carcinoma.   e Vagotomy results in removal of parasympathetic influence  on the function of foregut and midgut.    It 1s due to oedema as in gastroduodenal anastomosis or  nondependent drainage as in GJ. Sometimes, fat in the  transverse mesocolon undergoes necrosis resulting in  obstruction to the loops. Stomal obstruction also develops  if there is narrowing of the lumen. Treatment is conservative.  Surgery may also be required later, after confirming  obstruction by gastrografin studies.    It develops if efferent and afferent loops are not sutured  properly. It can appear at any time after surgery.    ¢ Previous history of abdominal surgery (surgery done  for peptic ulcer)    Barium meal X-ray shows filling defect in the stomach  (Fig. 23.106). Sometimes, following a barium meal,  intussusception 1s reduced (Fig. 23.107).    Fig. 23.106: Barium showing filling defect, in a patient who had  undergone GJ   Fig. 23.108: Gangrene of the intestines in a patient who had    Reduction of the intussusception and suturing of intestinal  loops properly. If the loops are gangrenous, resection may    Fig. 23.109: Barium enema —barium entering into the stomach from  colon (barium meal is not the investigation for gastrojejunocolic fistula)    It is a complication of GJ done for peptic ulcer, especially    After a few years of GJ, a recurrent ulcer can develop at the   ¢ Foul eructation and foul vomiting due to colonic  contents entering the stomach which is loaded with  faecal matter and foul contents.   e Intense diarrhoea due to severe jejunitis brought about  by colonic bacteria entering the jeyunum.   ¢ Rapid deterioration in health—loss of weight, loss of  appetite, dehydration and emaciation.    Diarrhoea is not due to food entering the colon.  Contents of the colon enter the stomach and then  jejunum resulting in jejunitis causing diarrhoea.   ¢ Confirm by barium enema—barium entering the  stomach (because of high pressure in the colon). Barium  meal study should not be done (Fig. 23.109).   ¢ Preoperative preparation is necessary in the form of  blood transfusion, stomach wash, nutritional  supplementation and correction of dehydration.   ¢ Resection of portions of stomach, intestine and colon  followed by end to end anastomosis.    It refers to carcinoma developing in the stomach after some  surgery on the stomach. Classically it happens after a  gastrojejunostomy (GJ) Billroth II or after a pyloroplasty.  It develops after 10 to 20 years of surgery.   Reflux of bile, changes in the acidity due to vagotomy are  a few factors that precipitate stump carcinoma. Metaplasia  is due to enterogastric reflux and bacterial reduction of  nitrates.   Clinical features include sudden loss of appetite, loss of  weight with or without mass abdomen.   Treated by resection. However, many cases are advanced  and they are inoperable.   Instead of the short loop—jejunum, ileum is anastomosed  to the stomach.   There will be severe uncontrolled diarrhoea, loss of weight  and emaciation within a short period.   Barium meal with fluoroscopy should be done which  shows rapid flow of barium from stomach into the ileum.  Laparotomy—undoing of gastroileostomy and fresh  gastrojejunostomy should be done.   the stomach, coming from colon (see  Fig. 23.110: Specimen of triple resection the triangular folds of colon)   This syndrome complex results due to rapid emptying of  stomach contents to the distal intestines resulting in various  physiological changes such as vasomotor symptoms,  hypoglycaemia, etc. They are of two types. Their comparison  is given in Table 23.10.    Alkaline reflux gastritis which develops after GJ should  be differentiated from afferent loop syndrome (see Table  23.11 on the next page).    Early Late  I. Onset Increased emptying immediately after meals One to two hours afterwards.  2. Causes Rapid passage of food of high osmolarity from Initial hyperglycaemia causes increased release of  stomach into small intestine causes hypovolaemia of insulin which is followed by hypoglycaemia.  J ROUEH Lying down Glucose  4.  Aggravating factors More food, glucose, fullness Exercise  5. Symptoms Epigastric distension, sweating, diarrhoea Tremors, fainting attacks (release of adrenaline)  6. Treatment Small quantity, dry food. Over a period of time, Dietary adjustment of food   1) <5 Comparison of chronic afferent limb syndrome and alkaline reflux gastritis    Limb obstruction—long loop  Avoid a long loop at surgery  Convert Billroth IT to Billroth I  Roux-en-Y jejunostomy    ¢ Diarrhoea is due to vagotomy causing intestinal hurry or  due to dumping.    It is the leakage of duodenal contents to the exterior. It  commonly occurs after surgery.    e After a partial gastrectomy/total gastrectomy, where the  closure of duodenum was difficult.   ¢ After closure of perforated duodenal ulcer, which gives way  once again.   e If there is a distal obstruction, it increases tension in  duodenal loop and may result in fistula.    ¢ Signs and symptoms develop usually after 4 to 5 days when  oral fluids are commenced. These stimulate outpouring of  biliary and pancreatic juices.    e Severe upper abdominal  pain and guarding, rigidity,  hypotension and shock-like  features of biliary peritonitis  develop if there 1s no  drainage tube.    ¢ If drainage tube is kept in  the first surgery, bile flows  to the exterior. In suchcases,  signs of peritonitis are  usually not present. How-  ever, severe electrolyte  imbalance can occur (Fig. 23.117).    ¢ Surgical—if the fistula persists, laparotomy and closure  of the fistula can be done by repairing with nonabsorbable  sutures.    ¢ Excoriation of abdominal skin can be prevented by zinc  oxide application.  ¢ Fluid and electrolyte imbalance.    It can be true anastomotic ulcer (gastrojejunal, gastroduodenal  or jejunal ulcer), or a gastric ulcer in the remnant, or recurrent  ulcer following highly selective vagotomy (HSV).   ¢ 3% after Billroth II gastrectomy  ¢ 5 to 8% after vagotomy and GJ  ¢ 40% after gastrojeyunostomy    Fig. 23.118: Various causes of recurrent ulcer (see the text for  numbers)   e Severe persistent pain, ‘boring’ type, which gets worse  within a few minutes of taking food. The pain is felt on the  left side of the abdomen, near the umbilical region and it  passes downwards.   ¢ The pain is felt in the lower left chest following antecolic  anastomosis. It 1s not relieved by antacids or milk unlike  peptic ulcer. Bleeding may manifest as haematemesis,  melaena or anaemia. Perforation can occur, resulting in  peritonitis.    Diagnosis  ¢ Gastroscopy gives the correct diagnosis.  ¢ Hypercalcaemia and hypergastrinaemia should be ruled out.    ¢ Conservative treatment with H, receptor blockers is nearly  always effective but relapse occurs if they are stopped.  Smoking should be stopped.    These tests are not routinely done nowadays due to the  availability of endoscopy facilities. However, in rare cases of  recurrent peptic ulcer disease or as in Zollinger-Ellison  syndrome, these tests are done. Hence, these are discussed in  the last pages.    Pentagastrin stimulates parietal cell mass resulting in  outpouring of gastric acid.    1. GJ alone =D  2. Vagotomy + GJ (incomplete vagotomy) ZS,  3. Vagotomy + GJ (complete vagotomy) >  4. Billroth partial gastrectomy ry   ¢ 4 samples of stomach secretion are collected for one hour,  once every 15 minutes.    ¢ Its values are very high in duodenal ulcer patients.  Vagotomy and antrectomy may be the treatment of choice.    ¢ In patients with gastrinoma, the basal acid output is  unusually high and there may be a little response to  pentagastrin stimulation.    ¢ If vagotomy is complete, there is no change in acid output  during insulin test—Hollander’s test.    ¢ Toa fasting patient, 0.2 units /10 kg body weight of insulin  is given subcutaneously.    ¢ Blood sugar is estimated at 15-minute intervals and it is  maintained between 30 and 40 mg% after two hours.    Acid output is measured for one hour. If there is no change in  acid output, vagotomy is complete. If there is a rise in  concentration of 20 mmol per litre above the basal level in the  first hour, it suggests incomplete vagotomy.    ¢ To diagnose Zollinger-Ellison syndrome, where very high  values of acid are seen.    The secretions of the stomach in the resting period or  interdigestive period for 12 hours in the night are measured.    'Many deaths have occurred due to this test in the past.   Introduce a Ryle’s tube and aspirate the stomach contents fo  12 hours from 9 PM to 9 AM. The volume and HCI in thi:  gastric juice are measured.    ¢ In normal patients, the total amount of gastric secretion 1:  around 400 ml. Above this, it suggests vagal hyperactivity    ¢ In Zollinger-Ellison syndrome the levels may be as high a:  one litre.    ¢ Free HCI in normal patients is 10-20 mEq/L, in duodena  ulcer 60-80 mEq/L, in gastric ulcer 10-20 mEq/L, and ir  Zollinger-Ellison syndrome, it may be around 100-—30(  mEq/L.    1. Can occur after any operation, particularly splenectomy and  pelvic procedures.    2. It can occur following fracture femur, application of plaster  of Paris, etc. Malnutrition, excessive distension of the  stomach due to ventilation, aerophagia are other  precipitating factors.    There is a sudden loss of sympathetic tone resulting in  massive dilatation of stomach. Improper Ryle’s tube aspiration  and permitting intake of oral fluids too early before paralytic  ileus settles down are additional factors.    ¢ Hiccoughs—due to irritation of under surface of the  diaphragm, by the hugely distended stomach.    ¢ Abdominal pain, vomiting, distension. Vomiting contains  foul smelling dirty fluid and blood and is effortless.    ¢ Features of shock. In untreated cases, can lead to  cardiovascular collapse.    ¢ Effortless vomiting of litres of dark watery fluid is  characteristic of this condition.   e Introduce a Ryle’s tube and aspirate the stomach. It is  the life-saving use of Ryle’s tube (Fig. 23.119).    saline. Both crystalloids and colloids may be necessary to  treat the shock and electrolyte abnormalities.   Fig. 23.119: Plain X-ray showing acute dilatation of the stomach.  Ryle’s tube insertion is a life-saving simple procedure in this  condition    Reduce the volvulus by dividing gastrocolic omentum  Fix the greater curvature of the stomach to the duodeno-  jejunal flexure or perform a GJ without stoma.    Bezoars are collections of nondigestible materials, usually  of vegetable origin (phytobezoar) but also of hair  (trichobezoar).   Trichobezoars are concentrations of hair, generally found  in long-haired girls or women who often deny eating their  own hair (trichophagy) (Figs 23.120 and 23.121).    Pulmonary: In debilitated patients, aspiration may result in  aspiration pneumonitis (Mendelson syndrome).  It carries significant mortality.    It is arare condition in which stomach rotates in a horizontal  (organoaxial) and vertical (mesentericoaxial) direction  resulting in an acute abdomen.   In general, initially the colon moves upwards and later  greater curvature of the stomach.   There is associated eventration of the diaphragm which also  precipitates this condition (in children, congenital).   In adults, diaphragmatic defects are more commonly  traumatic or paraoesophageal herniation.    . Sudden, constant, severe upper abdominal pain  . Recurrent retching with production of little vomitus  . Inability to pass a nasogastric tube.    Plain X-ray abdomen/chest: Gas filled viscus  Barium meal can demonstrate twisted stomach  Upper GI scopy.   Fig. 23.120: Trichobezoar: Appreciate the shape of stomach and  first part of the duodenum in bezoars (Courtesy: Dr Rajiv Shetty,  Prof & Head, Dept of Surgery, Bangalore Medical College and  Medical Superintendent, Bowring Hospital, Karnataka)   Fig. 23.121: Trichobezoar (Courtesy: Dr YV Krishna Rao, Prof of  Surgery, Mamata Medical College and Hospital, Khammam, Andhra  Pradesh)   ¢ Phytobezoars are most commonly found in patients who  have undergone surgery of the stomach and have impaired  gastric emptying.    ¢ The symptom of gastric bezoars include early satiety, weight  loss, nausea, pain and vomiting due to gastric outlet  obstruction with occasional gastric perforation and small  bowel obstruction.   ¢ A large mass may be palpable on physical examination  and the diagnosis 1s confirmed by a barium examination or  endoscopy.    — Papain found in Adolph’s Meat Tenderizer (AMT) 1s  given in a dose of one teaspoon in 150 to 300 ml] water  several times daily.    Trichobezoars tend to form a cast of the stomach, with  strands of hair having been observed as far distally as  the transverse colon.    e Inthis condition there is hypertrophy involving the pyloric  antral circular muscle fibres. Duodenum is normal  (Fig, 23.123).    ¢ The lumen is so much narrowed as to give rise to pyloric  obstruction.   ¢ Prenatal incoordination of muscles, lack of nitric oxide  relaxation of smooth muscles may be some factors  responsible for IHPS.   ¢ Incidence: 3—5/1000 births. First born male child 1s affectec  very often. The child is normal at birth and the symptoms  appear around 6-8 weeks.   ¢ First symptom is vomiting. It is projectile, forcible.  does not contain bile. A visible gastric peristalsis (VGP)  can be seen especially when the mother feeds the child  (Fig. 23.122).    ¢ When muscle thickness is more than 7-15 mm size and « Laparotomy is done and an incision is made through the  pyloric canal length is 20—25 mm, it 1s diagnostic. serosa. It then cuts through the circular muscle fibres till all  the muscle fibres are divided and the mucosa bulges out.   ¢ Avoid injury to the mucosa. If mucosa Is opened, it is sutured    ¢ Ramstedt’s pyloromyotomy is the surgical treatment  (Figs 23.124 to 23.127). CHRONIC DUODENAL ILEUS    ¢ It is also called superior mesenteric artery (SMA) syndrome,  Wilkie’s disease or arteriomesenteric compression  (Figs 23.128 and 23.129). The third part of the duodenum 1s  compressed between vertebral column and superior  mesenteric vessels. Acute loss of fat and immobilisation with  cast precipitates this disease. Hence, it is also called cast  syndrome. Normal angle between superior mesenteric artery  and aorta is 38—56°. Due to loss of fat or due to abnormal  origin of the SMA, when the angle reduces to 6—25°, SMA  syndrome occur (for risk factor—see Key Box 23.29).    Fig. 23.127: Ramstedt’s pyloromyotomy being done (Courtesy: ABCDEFGH of SMA syndrome  Prof Vijaykumar, HOD of Paediatric Surgery, KMC, Manipal) -—==—    In this condition, duodenum is dilated up to the 3rd part with  obstruction to the 3rd or 4th part of duodenum (Fig. 23.128).    Abdominal pain is not severe as in intestinal obstruction  but on careful questioning history of true nature of  spasmodic pain is obtained.    Pyloric stenosis: In this condition, vomitus does not contain  bile. Barium meal X-ray shows distended stomach.  Duodenum is not seen.    There are many causes of duodenal obstruction in children  In adults important causes are given in Table 23.13.  Ist part: Postbulbar ulcer with stenosis.  2nd part: 2 causes:  1. Annular pancreas in adults  2. Carcinoma head of pancreas  3rd part: 3 causes:  1. Carcinoma head of pancreas  2. Arteriomesenteric compression  3. Tuberculosis/metastatic nodes causing compression  of the 3rd part of duodenum. Most common cause  of duodenal obstruction in adults is carcinoma  pancreas.  4th part: Carcinoma pancreas (Fig. 23.131)    2. Annular pancreas: Rarely, it can present in adults with  obstruction to the second part of the duodenum. Vomitus  may contain bile. Barium meal X-ray shows dilatation  of the first part and also the second part of the duodenum.    ¢ It is done for morbid obesity.  3. Tuberculosis of lymph nodes can compress the 3rd part . Morbid obesity is defined as being 100% over the ideal    of the duodenum and can mimic Wilkie’s disease. weight for height or having body mass index (weight in kg/    Parts Development Length Relationships Blood supply Surgical importance  Ist part ¢ Foregut 2 inches ¢ Gall bladder ¢ Superior pancreaticoduodenal _* Perforation of Ist part  ¢ Liver ¢ Supraduodenal artery of (causes peritonitis)  ¢ Bile duct Wilkie ¢ Injury to duodenum in  ¢ Portal vein laparoscopic cholecystectomy  2nd part  »¢ Foregut above 3 inches ~~ * Right lobe of liver ¢Perforation of 2nd part manifests  ampulla of Vater transverse colon anter- late because it 1s retroperitoneal  ¢ Midgut below iorly and kidney, renal ¢ Injury to duodenum tn right  ampulla of Vater vessels posteriorly hemicolectomy  3rd part ¢ Midgut 4 inches ¢ Retroperitoneal ¢ Inferior pancreaticoduodenal «Careful separation of mesenteric  fixed artery vessels from duodenum during  ¢ Anteriorly crossed by pancreaticoduodenectomy  superior mesenteric  vessels  ¢ Posteriorly related to  inferior vena cava  4th part e Midgut | inch e Anteriorly by ¢ Inferior pancreaticoduodenal 4th part continues as duodenojejunal    'Nutcracker syndrome is the entrapment of right renal vein between abdominal aorta and the SMA   ¢ Often these patients are young with diabetes and renal  problems. Obesity related comorbidities such as diabetes  mellitus, hypertension, coronary artery disease, osteo-  arthritis, sleep apnoea, etc., will improve after surgery.    1. Lap band procedure: It is one of the commonly performed  bariatric procedure. In this procedure an inflatable cuff 1s  placed laparoscopically just below oesophagogastric  junction. It is adjustable. The balloon can be filled to 15 cc  with saline. This can be increased or decreased also. Band  slippage and stricture are problems. Band can also be  removed. Advantage of banding is high safety and easy  reversibility.    2. Laparoscopic gastric bypass 1s the procedure of choice  with good results (Fig. 23.132).    ¢ 30 cc stomach pouch is fashioned using endostaplers. WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES   ¢ Duodenojejunal junction is identified by ligament of Treitz ° All the topics have been updated. 2  and jejunum is divided 100 cm distally. ° New photographs and key boxes have been added.   e Helicobacter pylori infections and carcinoma stomach  have been discussed in detail.   * Distal jejunojejunostomy is made at a distance of 150 cm. e Bezoars, surgery for morbid obesity have been added.   e Stump carcinoma, SMA syndrome have been explained  in more detail.    ¢ There is significant improvement in diabetes mellitus and  hypertension, high cholesterol after weight loss surgery. A  gastric pouch 1s created by using circular and linear staples.   C. Right gastroepiploic artery runs along greater  curvature of the stomach   . It can erode branches of left gastric artery and result  in massive bleeding    . Most important cause of duodenal stump leakage after  Billroth II gastrectomy is:    . About truncal vagotomy which one of the following is  not correct?    More accurate investigation for the diagnosis of the  perforated duodenal ulcer is:    Following are true with Dieulafoy's lesion except:  A. It 1s an arteriovenous malformation    H. pylori is an important factor for carcinoma stomach  in which region?   Which one of the following is not a risk factor for  proximal carcinoma stomach?   Which one of the following is not a feature of early  gastric cancer?    Following are features of gastrointestinal stromal  tumours (GISTs) of the stomach except:  A. Stomach is the most common site of GIST    B. More than 5 cm size tumour is considered to have  malignant potential    C. Wedge excision is the treatment of choice for small  lesions    Which one of the following features is false for  gastrointestinal stromal tumour (GIST)?    ANSWERS  1D 2 A 3A 4D os D 6C 0G 8 D 3 10 B  11 C 12 D 13 C 14D te GC 16A 17 B 18 D to 20 D    Liver is located in the right hypochondrium extending into  epigastrium and left hypochondrium.    It weighs about 1,500 g—three quarters of it is the right  lobe and rest is the left lobe (Figs 24.1 and 24.2).    Right triangular ligament fixes the right lobe to the  undersurface of the right dome of diaphragm. When this  ligament is divided, right lobe can be mobilised and can be  turned to left as during right hepatic lobectomy or to control  bleeding from liver trauma.    diaphragm in a similar fashion. For a complete vagotomy,  when this ligament 1s divided, left lobe can be easily  retracted—vagotomy becomes easy. This ligament also  needs to be divided to get access to retrohepatic inferior  vena cava.    . Falciform ligament: It is the remnant of umbilical vein. It    runs from umbilicus to the liver dividing the liver into right  and left lobes, passing into the interlobar fissure. Division  of superior leaves of falciform ligament helps in exposing  suprahepatic inferior vena cava (IVC).    Right branch of hepatic artery supplies entire right lobe and  left branch supplies the left lobe.    Ligation of hepatic artery may not cause necrosis of liver  in normal individuals. However, it can cause liver failure  in cirrhotic patients (it was done in olden days to control  secondaries in the liver), and in obstructive jaundice  patients.    Major venous drainage is through 3 hepatic veins: right  middle and left. They join the inferior vena cava (IVC)  immediately below the diaphragm. Right hepatic vein  has a small extrahepatic course. On the other hand,  middle and left hepatic veins usually join within liver  parenchyma.    ¢ This 1s also called porta hepatis. It has the most vital  structures within it—hepatic artery, bile duct anteriorly and  portal vein posteriorly.   ¢ Within the hilum, these structures divide into right and left  branches. Left bile duct has a longer extrahepatic course  of approximately 2 cm. In CBD strictures, this can be used  for hepaticojejunostomy.    The functional unit of the liver is the hepatic lobule. Each  hepatic lobule consist of sheets of liver cells, separated by  hepatic sinusoids, venous channels whichdrainblood to central  vein, a tributary of the hepatic vein from portal tracts that  contain branches of the hepatic vein and portal vein.    I. Infection through the portal vein  ¢ Acute appendicitis  ¢ Acute diverticulitis (sigmoid)  ¢ Acute amoebic colitis  ¢ Acute bacillary dysentery  ¢ Ulcerative colitis    ¢ Liver has an excellent capacity for regeneration after Jy Infection through the common bile duct (CBD)  partial resection. ¢ Stricture of the CBD  ¢ A factor released from pancreas is supposed to be ¢ Periampullary carcinoma resulting in stasis of the bile,  responsible for this. precipitating infection (cholangitis)  Physiology of the liver  Functioning of the liver Significance   ¢ Principal site of synthesis of all proteins except globulins,  which are produced in reticuloendothelial systems   Hepatitis due to drugs such as rifampicin and other antituberculous drugs  Severely impaired in FHF    Majority of the infective bacteria are derived from gastro-  intestinal tract (Key Box 24.1)   E.coli is the most common facultative organism  Bacteroides fragilis is the most common anaerobe  Candidal infection is increasing due to chemotherapy,  especially for leukaemic patients.    Alcoholic males and debilitated men suffer more, probably  because of poor immunity.    Acute abscesses are usually multiple, chronic are single.  Tender hepatomegaly, low grade or high grade pyrexia with  abdominal discomfort are the main features.   Stool routine examination: Amoebic cysts, culture and  sensitivity for typhoid bacilli.   When in doubt, CT scan can be done (Figs 24.4 and 24.5),  followed by FNAC which draws frank pus. Pus is sent for  Gram’s stain, culture and sensitivity. CT also helps in the  diagnosis of associated conditions such as diverticulitis  of the colon.   Fig. 24.4: CT scan showing hypodense lesion in the right lobe of  liver Suggestive of pyogenic liver abscess   Fig. 24.5: CT scan showing multiple liver abscesses proved to be  due to amoebiasis    e Further investigations are directed towards the associated  conditions. Examples:  — Chest X-ray: Air under the diaphragm (perforation of  hollow viscus) or diagnosis of empyema thoracis.    1. Conservative: Multiple small abscesses may respond to  antibiotics. However, they have to be given for 4 to 6 weeks.   2. Percutaneous drainage (Key Box 24.2)  Method  ¢ Ultrasound or CT-guided aspiration and drainage by using   3. Open (surgical) method (Key Box 24.3)  Laparotomy is required mainly to treat the primary causes,  e.g. appendicectomy, drainage of appendicular abscess. If  liver shows a significant abscess, it is drained. Alternately,  a pigtail catheter is introduced into the abscess cavity and  brought outside through a separate opening. It helps to drain  for a longer period of time.   PERCUTANEOUS DRAINAGE—INDICATIONS s  e Superficial abscesses  e Abscess with no intra-abdominal pathology  e Abscess of unknown aetiology   OPEN (SURGICAL) METHOD—INDICATIONS Y  e Abscess with intra-abdominal pathology  e Ascites  e Deep-seated abscess  e Multiple abscesses    It is also called Tropical abscess (dysenteric abscess). It is the  commonest extraintestinal manifestation of amoebiasis.    Aetiopathogenesis  e This disease is caused by Entamoeba histolytica.  ¢ It is almost always a complication of amoebic dysentery.  This can occur in the acute stage or in the chronic carrier  stage.  — Infection from the caecum (typhlitis) spreads through  the tributary of superior mesenteric vein.  — From sigmoid colon, through the tributary of inferior  mesenteric vein (Fig. 24.6).    The right branch of the portal vein is in direct line with the  portal vein. Hence, by streamline phenomenon organisms  reach the right lobe more often than the left lobe. The right  lobe is also much bigger than the left lobe.   In the right lobe, it is the posterosuperior surface which  gets involved because it is extraperitoneal (bare area of  liver). It has no peritoneal covering.   After reaching the liver, the organism causes destruction of  hepatocytes by releasing powerful cytolytic enzymes  resulting in liquefaction necrosis. It also causes aseptic  thrombosis of blood vessels resulting in necrosis of liver  tissue.   At the same time, some RBCs! are also broken down. This  causes anchovy sauce pus, which is chocolate brown in  colour, and is a mixture of broken downRBCs, hepatocytes,  etc.   Green pus Is referred to pus mixed with bile, which is seen  in a few patients.   In majority of the cases, pus is sterile. Secondary infection  occurs in about 20 to 30% of the cases.   Amoebae are rarely present in the pus but are present in  the wall of the abscess cavity. The wall contains monocytes,  plasma cells, lymphocytes and fibroblasts. Abscesses are  multiple which fuse to form a single large abscess cavity in  about 70% of the cases. Due to perthepatitis, abscess gets  fixed to the diaphragm resulting in immobility of the  diaphragm. Liver abscess in the left lobe gets adhered to  anterior abdominal wall.   It is interesting to note that amoebic infection of gall bladder  and bile does not occur because of deleterious effect of  bile on amoebae.    Male alcoholics are commonly affected, in the age group  of 20-40 years. It 1s eight times more common in men.    ' Absence of WBC is an important feature. Hence, regeneration of abscess cavity is complete without scarring.   Fig. 24.7: Intercostal bulge and tenderness are important features  of amoebic liver abscess of the liver—these signs are not seen in  acute cholecystitis   ¢. Severe pain in the right hypochondrium is due to the  enlarged liver. This stage is called stage of Amoebic  Hepatitis. If USG is done, it may not demonstrate any  abscesses but there may be many microabscesses. At this  Stage, there is low grade fever, weakness, anorexia, etc.   ¢ High grade fever with chills and rigors develop if the stage  proceeds to pyogenic liver abscess due to secondary  bacterial infection of amoebic abscess.   ¢ Thoracic symptoms such as nonproductive cough, pleurisy  and right shoulder pain are common.   e Anaemia, emaciation, toxic look and an earthy complexion  is present.   ¢ Jaundice may be present if abscesses are multiple, due to  compression of biliary radicles. However, it is rare (15%).  It is of cholestatic variety.   ¢ Liver is enlarged in the right hypochondrium, tender and  soft (liver enlarges in upward direction) (Key Box 24.4).   VERY TENDER LIVER J  e Amoebic liver abscess  e Hepatoma  e Congestive cardiac failure    ¢ Intercostal tenderness differentiates it from acute  cholecystitis. Intercostal oedema can also be present  (Figs 24.7 and 24.8A).    1. Total WBC count may be increased if there is secondary  infection.   2. Stool examination for ova and cysts of Entamoeba  histolytica may be positive in 25% of cases.   3. Serologic testing: The indirect haemagglutination test 1s  positive in 90-95% of patients with an amoebic abscess.   4. Screening chest: When the patient is asked to take a deep  breath, right side of the diaphragm does not move due to  inflammatory (perihepatitis) adhesions between liver and  diaphragm. This is called homolateral immobility of the  diaphragm. A small pleural effusion may also be present.   6. Abdominal USG: It is the investigation of choice.  ¢ To locate site of abscess and then to confirm diagnosis.  ¢ Ultrasound guided needle aspiration can also be done   7. CT scan can demonstrate an abscess cavity as a low density  zone surrounded by peripheral hypodense zone due to  inflammatory reaction.    ¢ It is indicated in amoebic hepatitis. Tab. Metronidazole  400-800 mg, 3 times a day is given for 14 days.    ¢ If the condition does not improve, injection Emetine  1 mg/kg body weight to a total of 60 mg/day deep IM for a  maximum of 6 days Is given.   ¢ ECG should be recorded before, during and after the therapy.   ¢ Absolute bed rest during treatment (because of these  complications, it is not used nowadays).   ¢ Adequate hydration, rest, analgesics to relieve the pain.  Improvement can be seen within one to two days in the   ¢ Before it is aspirated, bleeding profile (BT, CT, PT) should  be normal and injection vitamin K 10 mg, IM should be  given for at least 3 days.   e US-guided aspiration is also the treatment of choice where  metronidazole is contraindicated, e.g. \st trimester of  pregnancy.   Typically it is anchovy sauce pus. Aspiration is followed  by insertion of pig tail catheter.    Before removal of the catheter do a repeat ultrasound to  check for residual pus.   PIGTAIL CATHETER  e They are the catheter used to drain clear fluids from cavities  such as pleural fluid, ascites, bile, etc.  Different sizes are available 8 to 12 French  Radio-opaque polyurethane catheter  Length — 20 or 30 cm  Large oval holes in pigtail for maximum drainage  Thus, maximum efficiency is when ‘transudate’ pleural  effusion is drained, than empyema, haemothorax or  anchovy sauce pus.    The clinical response to the aspiration may be observed as  subsiding of fever (Figs 24.8B to 24.11).   2. Incomplete aspiration results in leakage of pus and bile into  peritoneal cavity which may produce peritonitis. Hence,  prophylactic antibiotics need to be given before and after  the procedure along with metronidazole therapy.    1. Failure of US-guided needle aspiration.  2. Ruptured amoebic liver abscess with amoebic peritonitis.    ¢ Laparotomy is done first. Abscess cavity is identified.  Contents are evacuated, a thorough peritoneal wash 1s given  and a self-retaining Malecot’s catheter (any tube drain)  is introduced into the abscess cavity, brought outside and  connected to a bag.   * With the advent of metronidazole, Amoebiasis Cutis is  rarely seen. Hence, catheter can be safely placed inside  the cavity and brought out. Malecot’s catheter is now being  replaced by other tube drains.   ¢ Postoperatively for 3-5 days, necrotic liver tissue, anchovy  sauce pus and blood drain outside.   ¢ Once the drainage becomes minimal, Malecot’s catheter is  pulled out.  ¢ Same procedure can be done by laparoscopic method.    ¢ Amoebic peritonitis, resulting in acute abdomen with  shock. It has to be treated like any peritonitis—laparotomy,  drainage of pus and drain the abscess cavity to outside  (possibility of amoebiasis cutis is still present but rare).   ¢ Amoebic pericardial effusion occurs due to rupture of left  liver lobe abscess into pericardial space.    The disease is caused by Echinococcus granulosus, transmitted  by dogs which are the chief mediators (host) and man is the  intermediate host (Fig. 24.12). After swallowing the ova, they  penetrate gastric mucosa!, reach retroperitoneal structures,  penetrate portal vein directly and then enter into liver. Having  reached liver, the organisms grow and develop their own  protective layer and form hydatid cyst.    1. The adventitia (pseudocyst)*: This is the fibrous layer  derived from the liver tissue. It 1s the reaction of liver to the  parasite. It 1s adherent to the liver and cannot be separated—  pericyst.    'Reason why Hydatid ova do not produce lesions in the intestine.  “It is the protective coat given by the host to the ghost.    2. The ectocyst (laminated membrane): It 1s white anc  elastic and is produced by endocyst. It 1s this layer whick  gets peeled off at surgery.    3. The endocyst: This is called germinal epithelium and it is    the innermost part of hydatid cyst. It secretes hydatid fluid  inside and ectocyst outside. Within the hydatid fluid, the  ‘brood capsules’ develop within which the scolices of  Echinococcus granulosus develop.    ¢ It can be silent—without any symptoms throughout life,  accidentally discovered on routine examination.   ¢ Liver is enlarged, has a smooth surface, round borders and  is nontender.   ¢ Typical hydatid thrill can be present on rare occasions.  Hydatid thrill is demonstrated by 3-finger method. Keep 3  fingers over the liver, percuss over the middle finger and  get the impulse by other 2 fingers (fluid thrill).   e Patient may present as an emergency with severe abdominal  pain following minor trauma.   ¢ May present as an emergency with features of anaphylatic  shock without any obvious cause.    calcified cyst; unlikely to contain viable  protoscolices  2. Plain X-ray abdomen may demonstrate speckled  calcification.    3. CT scan may be necessary in selected cases (Figs 24.14  and 24.15). The cyst which 1s superficial and has reached  surface, should be operated upon.   4. ERCP if there is obstructive jaundice—in such cases, a  wide sphincterotomy should be given so as to allow free  drainage of the hydatid contents into the duodenum.   5. Casoni’s intradermal test: Sensitivity and specificity of  this test 1s low and hence, no longer used.    symptomatic, or if the size 1s more than 5 cm, they may  be treated.    II. Medical treatment (Key Box 24.6)  ¢ Albendazole 8 mg/kg or 400 mg BD 1s given for 21 days  followed by drug holiday for 2 weeks.    There are different types of surgeries for hydatid disease of  the liver which have been summarised below. Indications are  given in Key Box 24.7.   INDICATIONS FOR MEDICAL TREATMENT y  e Disseminated hydatid cyst  e Inaccessible for surgery (deep seated, multiple, recurrent)  e Patient unfit for surgery  e Contamination of peritoneal cavity at surgery   INDICATIONS FOR SURGERY y  e Symptomatic cyst  e Asymptomatic patient with cyst > 5 cm, noncalcified  e Infected cyst (Figs 24.20 and 24.21)    |. Laparotomy and isolation of the cyst from peritoneal  cavity by packs dipped in hypochlorite solution.    2. Aspirate the contents and inject scolicidal agents such as  savlon or hypertonic saline.    3. Incise the cyst, peel off the laminated membrane by using  sponge-holding forceps (Figs 24.16 to 24.21).    4. An attempt to remove adventitial layer may result in  bleeding. It need not be removed.    5. At surgery, all laminated membrane, endocyst has to be  removed. Perfect haemostasis must be achieved. Any bile  leak to be sutured. Postoperative significant bile leak 1s  managed by ERCP stenting.   2. Avoid spillage into the peritoneal cavity to avoid peritoneal  hydatid.   3. Injection hydrocortisone 100 mg IV before, during and  after surgery to avoid anaphylactic shock.    They have been summarised in Key Box 24.9. However,  excision of the cyst leaving the adventitial layer is the most  commonly performed surgery (Figs 24.19 to 24.21).    IV. Percutaneous drainage  ¢ It is done after taking all necessary precautions/equipment  with all emergency drugs and with CT/US guidance.    (Courtesy: Dr Geetha R, Dr Ankur Sharma, Dr Rajesh Nair, Department of Surgery, KMC, Manipal)    Fig. 24.19: Laminated membrane removed at surgery Figs 24.20 and 24.21: Laminated membrane and daughter cysts are being    e Capitonnage: If omentum is not available for closing the  cavity, redundant cyst wall can be infolded into the cyst  cavity and sutured.    e Hepatic resection in good centres when there are proper  indications.    1. Rupture of the cyst into the peritoneal cavity can occur  due to trauma (rare) and may result in anaphylactic shock.  It should be treated accordingly with injection hydro-  cortisone and laparotomy.   as a part of PAIR treatment ted fluid was turbid, open  drainage was done in this  patient    ¢ Rupture can result in implantation of active cysts within  peritoneal cavity and they grow within. Rupture through  diaphragm can cause empyema. It can also rupture into  biliary radicles and can cause obstructive jaundice.   2. Jaundice due to cysts within biliary tree or due to a large  cyst compressing biliary ducts. ERCP followed by  endoscopic removal of hydatid cysts from the common bile  duct followed by sphincterotomy should be done.   4. Calcification of the cyst wall in long-standing cases  (Fig. 24.24).   This parasitic infestation is uncommon in our country but it 1s    ¢ When the cyst 1s thin walled, unilocular and regular it 1s  usually a simple cyst (Figs 24.25 to 24.27).   ¢ Clinically, if they are big, liver will be palpable with smooth  surface and round borders. It is nontender.   Fig. 24.25: Simple cyst at surgery. Observe the thin wall with slight  bluish colour    ¢ Ultrasound guided aspiration, laparoscopic de-roofing and open  surgical de-roofing are the methods of treating the cyst.   ¢ Cysts do not produce liver cell failure (unlike polycystic  kidneys).   ¢ Cyst haemorrhage may be the presenting feature with right  hypochondriac pain mimicking cholecystitis.   ¢ They are more frequently diagnosed now with frequent use  of ultrasound. Majority of them are asymptomatic and do  not require specific treatment.   ¢ Ifit is due to OCPs, it can regress after discontinuing OCPs.  Otherwise, adenomas need to be resected    Their removal should be attempted only by an experienced  surgeon. Hepatic adenoma and focal nodular hyperplasia  are compared in Table 24.2.   Most of them are solitary, subcapsular, occur in right lobe  of liver   It may be associated with cavernous haemangioma at some  other sites such as head and neck region. Clinically, it is  difficult to diagnose as it presents as hepatomegaly.  Kasabach-Merritt syndrome: Thrombocytopaenia and  consumptive coagulopathy.   Sometimes it may cause cardiovascular failure due to  sequestration of blood.   Ultrasound/CT scan can diagnose their location, number  (single or multiple) or presence of any other complications  associated with that (thrombosis, infection). MRI is a better  investigation.   Fig. 24.29: Haemangioma arising from the inferior surface of the  liver, is exposed at surgery    |. Trans-arterial embolisation (TAE):  e Indicated in large haemangiomas which are unresectable.  ¢ Large haemangiomas on the inferior surface of the liver  are ideally suitable for the treatment (Figs 24.29 to 24.32).  ¢ Embolic materials  (A) Temporary: Gel foam  (B) Permanent: Steel coils, polyvinyl alcohol, isobutyl  cyanoacrylate.  ¢ Polyvinyl alcohol particles 300-500 um in size are used often.  2. They can be resected or can be enucleated.  Caution: Before putting a needle into the space occupying  lesion in the liver, make sure it is NOT a haemangioma.    e Hepatoma, liver cysts, liver abscess  Other benign tumours are hepatic adenomas and focal  nodular hyperplasia.   ¢ Before discussion on HCC, one has to have a knowledge  about segmental anatomy of the liver, which forms the basis  of hepatic resection.   e Even though the details about hepatic resection may not be  required for undergraduate students, it is desirable to know  about the segmental anatomy to have a better understanding  about hepatic resection.    ¢ The liver is divided into two lobes by the main portal fissure,  which 1s also called Cantlie’s line. This line extends from  the gall bladder fossa to the left side of IVC and 1s inclined at   Figs 24.30 and 24.31: As it had a very narrow strip of the liver tissue,    an angle of 75° to the horizontal plane. The main portal  fissure is a constant feature. Each lobe is equal in size.   ¢ The right portal fissure divides the right lobe into an  anteromedial and posterolateral sector. The right hepatic  vein courses along this fissure. This fissure is inclined at an  angle of 40° with the horizontal.   ¢ The left portal fissure divides the left lobe into an anterior  and posterior sector, and it is in this fissure that the left  hepatic vein courses.   e The liver is further divided into segments, which represents  the smallest anatomic unit of the organ. In the right lobe,  each of the two sectors is divided into two segments: The  anteromedial sector and posterolateral sector. The antero-  medial sector 1s divided into segment V anteriorly and  segment VIII posteriorly, while the posterolateral sector is  divided into segment V1 anteriorly and segment VII posteriorly.   ¢ Inthe left lobe, the anterior sector 1s divided into segment IV  (or quadrate lobe) and the segment III. The posterior sector  is comprised of only one segment, segment IT. The Spigelian  lobe or segment I is considered as an autonomous segment.  It is also labelled caudate lobe.   e Each segment has its own identifiable portal vein, hepatic  arterial supply, bile duct and hepatic veins. All these  segments can be removed individually. However, segment I  receives tributaries from both the right and left branches of  portal vein and hepatic artery. However, hepatic venous  drainage is independent and may terminate directly into  IVC (Fig. 24.33).    ¢ Hepatocellular carcinoma is uncommon in United States  but more in Asians. It arises from hepatocytes.   ¢ When it arises from biliary epithelial cells, it is intrahepatic  cholangiocarcinoma.   8 segments, based on portal vein blood  supply and hepatic venous outflow as |  designated by Couinaud.    Segment |is also known as the caudate  ee lobe, segments II and Ill comprise the  lateral segment of the left lobe,  segment IV is the medial segment of  the left lobe.   Pathology: It is a highly vascular tumour and fatal if left untreated.  Few types and histological features are given below    athology: Histology types  e It is a highly vascular tumour with  indistinct margin.  Hanging type: It is attached to the  liver by a small stalk—easily  resected.  e Pushing type: A tumour pushing  the blood vessels. It is also    1. Hepatitis B virus (HBV): High titres of hepatitis B surface  antigen (HBsAg) was found in 50 to 60% of patients with  HCC. Its incidence 1s higher in chronic carriers and it is  increased if patients consume alcohol, aflatoxin and tobacco.   2. Hepatitis C virus (HCV): HCV infection induces cirrhosis  and increases the incidence of development of HCC.   3. Cirrhosis: It is a definite premalignant condition. A palpable  hard liver in a cirrhotic patient suggests development of  HCC.   4. Aflatoxin consumption (a fungus found in infected butter  yellow, bread) is responsible for increased incidence of HCC  as in Mozambique.   S. It 1s 4 times more common in Asians and two times in  Hispanics. Less common in Caucasians.   6. Oral contraceptive pills are known to cause adenoma and  there is a rare chance of adenoma tuming into HCC.   7. Other risk factors: Heavy alcohol consumption, cigarette  smoking, diabetes mellitus, haemochromatosis are the other  factors responsible for HCC. Wilson’s disease is rarely  associated with increased risk of HCC.   8. Hereditary tyrosinemia is a metabolic disorder characterised  by deficiency of enzyme fumaryl acetoacetate resulting in,  accumulation of toxic tyrosine metabolites—can progress  to HCC.   9. Other causes: Type I Glycogen storage disease and familial  polyposis coli Alpha-1 Antitrypsin deficiency, Budd-Chiar1  syndrome    ¢ Age group: Highest incidence is found after 50 years of  age. It is rarely seen in children also.   e infiltrative type: Difficult to resect  because of infiltration into surround-  ing structures.    Thus almost 80% of the cases are due to underlying  chronic hepatitis B and C virus infection.   A slow growing mass in the right hypochondrium. It is the  enlarged liver with an irregular surface and ts hard in  consistency. Evidence of cirrhosis may be present.   It can be a rapidly growing neoplasm, highly vascular and  clinically palpable as a soft to firm pulsatile mass with or  without a bruit.   Jaundice 1s not common unless the tumour arises in a  cirrhotic liver in hepatocellular failure.   Low grade pyrexia is common. It is due to tumour necrosis.  Liver being an important organ of metabolism, hypo-  glycaemia is found in 10% of the patients.   Triad of abdominal pain, weight loss and abdominal mass  is the most common clinical presentation.    Basic structure is trabecular  pattern with acidophilic  cytoplasm and large nuclei    hepatis) are involved first. Later, mediastinal nodes and left  supraclavicular nodes (Virchow’s node) are involved.    by dislodgement of tumour emboli into inferior vena cava  because of spread through the hepatic veins. Vertebral  involvement follows soon.   Liver function tests may show hepatocellular failure in  the form of high bilirubin, low albumin and high globulin  levels.    . Chest X-ray: CT chest pulmonary metastasis  . Abdominal USG: Findings    ¢ Diffuse distortion of hepatic parenchyma and a well-  circumscribed hyperechogenic mass suggests HCC.   ¢ Mosaic pattern of the tumour with thin halo and lateral  shadows, nodule in nodule pattern with separating  fibrous septa and posterior echo enhancement.    hypodense mass with internal mosaic architecture in the  early phase and hypodense lesion with enhanced fibrous  capsule in late phase. Guided FNAC can be done to confirm  the diagnosis. A small fear of tumour embolisation via portal  venous radicles exists and is a complication. Enhancement  in arterial phase and washout in delayed portovenous phase  is diagnostic of HCC (Figs 24.34 and 24.35).   Deeply eosinophilic hepatocytes, fibrous  stroma is arranged in the form of thin  hyalinised bands in layers. Hence the name, fibrolamellar  More   Fig. 24.34: CT scan showing a large hypodense mass lesion in  the left lobe   Fig. 24.35: CT scan showing a large hepatoma displacing the  portal vein branches    'Triple phase—Precontrast, early vascular or arterial, portal phase and delayed phase—multislice spiral CT is the gold standard investigation for  liver tumours. If clinical, radiological and biochemical features strongly suggest HCC, there is no need to prove histopathological diagnosis by  FNAC.    e It is a foetal antigen which disappears after birth. Hence,  it is not measurable in normal persons.   ¢ Levels above 400 ng/ml in a cirrhotic patients with hyper-  vascular mass larger than 2 cm in diameter is diagnostic.   ¢ o-fetoprotein levels indicate size of the tumour, invasion  and even recurrence.   ¢ In patients who are not operated, levels reflect response  to treatment (Key Box 24.11).    Selective angiography: It is done only if hepatic resection  is planned. It can demonstrate a highly vascular tumour  (tumour blush due to neovascularisation) or involvement  of portal vein thrombus in the IVC. Hence, venous phase  must be recorded. It can define arterial supply also.    e Itis done only in unresectable cases or metastatic disease  before starting other treatment such as chemotherapy, etc.   e With normal INR, fine needle aspiration can be done in  selected cases (clinical trials) with minimal risk.   Hydatid cyst of the liver—smooth surface, round borders.  Refer TNM staging of liver cancer    DIFFERENTIAL DIAGNOSIS J  Fibrolamellar carcinoma  Secondaries in the liver  Haemangioma  Focal nodular hyperplasia  Polycystic liver   T2 Solitary tumour with vascular invasion/multiple tumours  equal to or less than 5cm   T3 Multiple tumours more than 5 cm/tumour invading major  branch of portal or hepatic veins   T4 Tumour with direct invasion of adjacent organs other than  gall bladder and visceral peritoneum    ¢ Points 1] point each 2 points each 3 points each    1. Resection is the best possible treatment for hepatocellular  carcinoma. Up to 3 segments of the liver can be resected.  Rest of the liver will be enough to maintain life provided  remaining liver is healthy/noncirrhotic (Tables 24.4 and 24.5).    ¢ Functional liver remnant (FLR) should be > 20% of  standardised total liver volume after resection.    e¢ The remnant liver should have vascular inflow, venous  outflow, biliary drainage.  Two contiguous liver segments must be left behind  (Figs 24.36 to 24.41).    . Systemic chemotherapy using intravenous doxorubicin has  been tried with some success in the postoperative period.  Intra-arterial 5-FU has also been tried.    introducing gel foam into the branches of hepatic artery  produces some amount of tumour necrosis. If this arterial  embolisation is combined with chemotherapeutic agents   . Resection of entire right lobe and the medial segment of left lobe  . Removal of segments I] and III   . Removal of a small portion of liver, entirely within a single  segment or transferring segmental plane    Figs 24.36 and 24.37: Liver resection for adenoma (Courtesy: Dr Prasad Babu TLVD, Dr Ramcharan Thyagarajan, Dr Srikanth  Gadiyaram, Prof Sadiq S Sikora, Department of GI Surgery, Manipal Hospital, Bengaluru, Karnataka)    such as doxorubicin, results are better because the tumour  has prolonged exposure to the drug. This is called trans  arterial chemoembolisation. This method 1s followed only  as a palliative procedure.   Fig. 24.38: Left lobectomy for cholangio-  carcinoma (Courtesy: Dr Ramesh Rajan,  Associate Professor, Department of  Surgical Gastroenterology, Trivandrum  Medical College, Kerala)   Fig. 24.41: Liver resection for Caroli’s disease. (Courtesy: Dr  Nagaraj Palankar, Assistant Professor, Kasturba Hospital,  Manipal)   ¢ When radioactive agents are used it is called transarterial  radio embolisation (TARE)    Lipiodol is derived from poppy seed oil. It is an iodinated  ester. It is highly concentrated ester. It is highly  concentrated by hepatocytes. The contact time between  tumour cells and chemotherapeutic agents is prolonged  and it leads to cancer cell death.   Figs 24.39 and 24.40: Liver resection for adenoma (Courtesy: Dr Prasad Babu TLVD, Dr  Ramcharan Thyagarajan, Dr Srikanth Gadiyaram, Prof Sadig S Sikora, Department of GI  Surgery, Manipal Hospital, Bengaluru, Karnataka)    4. Percutaneous ethanol ablation (PEA): It is cheaper and  is a palliative treatment. Tumour less than 3 cm and nodules  not more than 3 are candidates. It reduces the tumour size  and decreases pain (Fig. 24.42).  ¢ Tumour necrosis occurs as a result of cellular dehydration  denaturation of proteins and occlusion of small tumour  vessels.   ¢ It requires 4—6 sessions under ultrasound guidance with  2-10 ml alcohol injection.   ¢ Injections are given on an outpatient basis. Easy to give  because of the soft consistency of the tumour.    ¢ It is now being tried for inoperable tumours or patients  who are not ideal candidates for surgery.  Indications: Tumour deep in parenchyma, away from  hilum and less than 3 cm.    ¢ Electrical energy (500 kHz) is delivered through a 18 G  needle inserted under US or CT guidance through the  skin into the tumour. In the following few months, the  tumour is destroyed and the cells are killed.   ¢ The route of insertion of this needle can be percutaneous,  laparoscopic or by open route.   ¢ There should not be any evidence of extrahepatic disease  preoperatively or intraoperatively.   Mode of actions: Cytodestruction of the tumour. In one  sitting 6—7 cm of necrosis zone can be achieved for a  5 cm tumour.   ¢ In most cases a tumour can be adequately treated in one  session. The procedure can also be repeated. It is done  under general anaesthesia.   ¢ It is better to avoid RFA when the tumour ts in the hilar  plate wherein portal vein and the hepatic arterial branches  enter the liver. Damage to these structures and bile duct  can occur.   6. Injection octreotide, an analogue of somatostatin, has been  used in advanced cases. It has shown promising results in  decreasing the size of the tumour. It also reduces the pain  and discomfort. This is combined with chemotherapy such  as gemcitabine and carboplatin. However, the treatment is  costly and results are temporary.   7. Soraferib is the drug of choice in advanced HCC and with  good liver function.    It should be the first choice in HCC, provided there 1s no extra-  hepatic disease or vascular invasion. After getting a negative  margin, adequate functioning liver tissue should be present.   ¢ A transverse abdominal incision in the right upper quadrant  with a vertical extension to xyphoid process gives excellent  exposure to the liver.    ¢ The key point is division of ligaments which are attached  to it or which support the liver and suspend the liver.   ¢ The falciform ligament is divided and followed along  anterior surface of the liver.   ¢ The left triangular ligament is divided by gentle pull on the  left lobe of liver towards right side.   ¢ The right triangular ligament is divided between diaphragm  and right lobe of the liver by traction and countertraction.   ¢ Small multiple veins from the inferior surface of the liver  join inferior vena cava. These should be divided.    ¢ Cholecystectomy is done by ligating cystic artery and cystic  duct. The CBD is then exposed at the free edge of lesser  omentum. A sling is applied around CBD to tsolate it and  gently traction is applied.   ¢ By dissection of common hepatic artery, its right and left  branches are dissected out.   e Slings are applied to these arteries, portal vein is exposed.  This facilitates freeing of portal vein by dividing lymphatic  tissues joining portal vein.   ¢ The congenital anomalies such as aberrant right hepatic artery  and accessory left hepatic artery also should be looked for.   ¢ CBD, CHD, left and right hepatic ducts are exposed at the  hilum.    ¢ This requires ligation of artery or branch by 4/0 prolene,  transfixation of the bile duct by 4/0 PDS and transfixation  of portal vein with 4/0 prolene.   ¢ Once the demarcation appears on the liver, parenchyma 1s  divided by using diathermy.   ¢ Cavitron ultrasonic surgical aspirator (CUSA) is the  commonly used dissector for dividing liver parenchyma.   ¢ Vessels and bile duct branches which are exposed are  clipped or diathermised.    ¢ Since each segment (see segmental anatomy) carries its own  arterial supply, venous drainage and bile drainage, resection  can be done with | cm margin.    It is a common feature of most of the  malignancies. Severe pallor may indicate  carcinoma stomach or carcinoma colon.    Dull-aching and continuous pain in the right hypochondrium  of short duration (3—6 months)   Loss of appetite, weakness, asthenia, malaise  Jaundice of a few days’ duration, is mild, usually  nonprogressive and is not associated with pruritus  (hepatocellular variety)   H/o previous surgery such as mastectomy  Amputation of toes or leg or wide excision of mole    Example 1: Secondaries in the liver from carcinoma  stomach. A distinct stomach mass may be felt (Figs 24.43  and 24.44).    Example 2: Secondaries in the liver from periampullary  carcinoma. A palpable gall bladder gives clue to the diagnosis  (Fig. 24.45).    Example 3: Secondaries in the liver from carcinoma colon  or rectum. H/o bleeding per rectum with or without mass.  Diagnosis is made by per rectal examination (Fig. 24.46).    Example 4: Secondaries in the liver from seminoma testis.  Many times, deep seated seminoma arising from  undescended testis cannot be felt per abdomen.    Example 5: Secondaries in the liver from malignant  melanoma of foot or leg. Evidence of amputation with  enlarged inguinal nodes 1s usually present (Fig. 24.47).    Enlarged liver stretches the parietal capsule which is  responsible for dull-aching pain   Due to destruction of an important metabolic organ  By the time jaundice appears, it is late and enough  liver tissue 1s damaged   Primary may be in the colon or rectum  Probably primary is carcinoma of the breast  Malignant melanoma is the likely cause   Fig. 24.44: Classical secondary in the liver with umbilication from  Carcinoma colon. However, it is impossible to appreciate it clinically   When there are massive secondaries in the liver and if you  suspect melanoma—look for    They depend upon the type of primary which could be detected  in a clinical examination. However, when the primary could  not be found out, following investigations may have to be done.  1. Upper gastrointestinal scopy and biopsy: To detect a  growth in the oesophagus or stomach.  2. Sigmoidoscopy/colonoscopy: To rule out carcinoma rectum  and colon (melanoma of the anal canal).  3. Abdominal ultrasound  ¢ It can diagnose secondaries in the liver which can be  hypo- or hyperechoeic and multiple with normal hepatic  parenchyma in between.  ¢ Itcan detect acarcinomatous growth in the periampullary  region.  ¢ Itcan detect enlarged lymph nodes—portal, coeliac, para-  aortic, etc.  ¢ It can detect deep seated testicular tumour arising from  the testis.  In spite of these investigations, if the primary cannot be  detected, other investigations may have to be done such as  bronchoscopy for bronchogenic carcinoma, acid phospha-  tase for carcinoma prostate or computed tomography (CT  scan), etc.   A 38-year-old lady came to the hospital with pain in the right  hypochondrium. On examination, firm to hard nodular liver  was palpable up to umbilicus. Before investigations, the  treating doctor told the husband and relatives that she has  secondaries in liver and she may not live for 6 months. 5 years  later, she came to the hospital. What she was having was  Polycystic Disease of Liver!!— (Table 24.7)    High quality CT scan triple phase with IV and oral contrast  medium provides actual assessment of the liver disease  (decision of hepatectomy) and other pathology in the  abdomen.    graphy is used to stage the disease, to detect extrahepatic  disease.    if primary cannot be detected. Lymphoma, carcinoid  tumours and metastatic neuroendocrinal tumours have  better prognosis.    In majority of the cases, treatment is only palliative aiming at  relieving symptoms. The following are a few examples:   In cases of colonic cancer and carcinoid tumour, the primary  can be resected because these primaries are slow growing  and carry good prognosis. If metastasis is to one lobe of  liver, hepatectomy can also be done.    'It is important to prove the diagnosis by FNAC or laparoscopic biopsy because if it is neuroendocrinal in origin or is a carcinoid, patient can live    Hepatoma Secondaries in the liver Hydatid cyst  1. Not uncommon Common Uncommon  2. One lobe is enlarged Both lobes are enlarged One lobe is enlarged  3. Consistency is hard or firm Hard Firm  4. Surface irregular Nodular Smooth  5. Short duration Short duration Long duration  6. Tender Nontender Nontender  7. General condition poor Poor Good  8. Vascularity—thrill and bruit may be present Absent Absent  9. H/o alcohol intake—features of cirrhosis Symptoms of primary such as loss of appetite, H/o contact with dog may  may be present vomiting, backache or another mass may be present be present  ll. Chemotherapy 5. More hepatic resections are done for colorectal metastasis  ¢ When the primary Is detected to be in gastrointestinal tract, than hepatocellular carcinoma.  injection 5-FU can be given. The dosage is 500 mg IV for5 6. Liver is the most common site of cancer recurrence after a  days/28 days for five cycles. complete resection.    tract, depending on the nature of the malignancy, appropriate  chemotherapy 1s given (they are discussed in appropriate  chapters).    ¢ A catheter 1s placed in the hepatic artery and substances  such as blood clot or gel foam are injected. They produce  thrombosis of hepatic artery resulting in livernecrosis. Thus,  liver regresses in size leading to decrease in pain.   ¢ Gel foam is cheap and easy to manipulate. The effect is  temporary.   4. When metastasis appear in the liver from lung cancer, e Synchronous metastasis—primary and metastasis  mostly bones, brain, adrenals would have been involved detected at the same time, e.g. carcinoma stomach  by metastasis, but it is not true/for colorectal cancer. Often, | * Metachronous metastasis—metastasis appearing much  liver metastasis for colorectal is the only visceral site of later than treatment of primary, e.g. melanoma of the    ¢ When portal venous pressure 1s more than 15 mmHg or  20 cm of saline, it is called portal hypertension.   ¢ Despite high prevalence of varices in patients with cirrhosis,  bleeding occurs in only 1/3rd of patients.   ¢ 10-30% of upper GI bleed are due to rupture of varices due  to cirrhosis.   ¢ Up to 30% of initial bleeds are fatal and as many as 70% of  survivors have recurrent bleeds after the first variceal bleed.    ¢ Splenic vein after receiving inferior mesenteric vein, joins  the superior mesenteric vein behind the neck of pancreas  and forms the portal vein. It runs in the free edge of the  lesser omentum covered by common bile duct.   ¢ [tis 6—8 cm in length in its extrahepatic course and divides  at the porta hepatis into right and left branches. Rarely, two  branches are given on the right side.   ¢ Portal vein carries 75% of the blood supply to the liver.  Left gastric vein enters the portal vein on its anteromedial   aspect just cephaloid to the margin of the pancreas. In 25% of   PORTAL VEIN S  Portal vein is 6-8 cm in length  Occurs by confluence of superior mesenteric vein and splenic  vein  Runs posterior to common bile duct    Two venous communications between coronary and azygos  veins give rise to varices    Approximately, it supplies 75% of total blood flow to the liver  Ligament related to portal vein is hepatoduodenal  Valveless structure    Increased portal pressure results in liver damage and bleeding  Normal pressure is 3-5 mmHg  Remember as PORTAL VEIN    |. Extrahepatic portal hypertension (prehepatic)  1. Splenic vein thrombosis  2. Portal vein thrombosis    It is secondary to umbilical vein sepsis in neonates  (omphalitis). In adults, thrombosed portal vein 1s surrounded  by a leash of vessels ‘cavernoma’. Portal vein thrombosis    can also occur due to carcinoma of the pancreas in elderly  patients.    Extrahepatic portal hypertension is seen in 20—25% of cases  and 1s common in female children. Liver function 1s normal  in these cases.    1. Cirrhosis of the liver  The regenerating nodules compress the portal venous  radicles within the liver. Liver function is very poor.    Common in Egypt. Schistosoma mansoni and S. japonicum  lay eggs within branches of portal vein and cause hepatic  fibrosis resulting in portal hypertension.    Ill. Posthepatic portal hypertension  These are medical causes:  1. Tricuspid incompetence  2. Constrictive pericarditis  3. Budd-Chiari syndrome    As aresult of obstruction to portal vein, in an attempt to reduce  portal pressure, the normally present insignificant collaterals  open up!. They become significant and result in development  of portosystemic anastomosis.    ¢ Even though, portosystemic shunts develop in many places,  their important locations and clinical significance have been  outlined in Table 24.8 and Fig. 24.49.    'When the main highway is blocked, small insignificant roads become significant, is it not?    1. Lower end of oesophagus Branches of left gastric vein  and short gastric vein    2. Falciform ligament of liver  (paraumbilical vein)  3. Lower end of rectum    Anterior abdominal wall veins Caput medusae”  Inferior and middle haemorrhoidal  vein   ¢ The lower end of the oesophagus 1s one of the important  areas giving rise to oesophageal varices. These varices  rupture resulting in massive haematemesis.   ¢ This 1s exactly the reason why every attempt at newer  modality of treatment, medical or surgical, is aimed at  controlling oesophageal varices.    Clinical features (Table 24.9)  Bleeding gastroesophageal varices are one of the common  causes of death among cirrhotics patients.   1. Physical factors: Elastic properties of vessels, intravariceal  and intraluminal pressure, and variceal wall tension are  major factors.   ¢ HVPG = (wedged/occluded hepatic venous pressure)—  (free hepatic venous pressure). Normal gradient is  5 mmHg. HVPG > 12 mmHgindicates portal hypertension.    ¢ Anaemia is usually present due to bleeding.  ¢ Peripheral smear to rule out hypersplenism—pancyto-  paenia in the presence of normal bone marrow.    2. Liver function tests  ¢ Serum proteins: Total protein, albumin are low and  albumin : globulin ratio is reversed in cirrhosis of the  liver.    Extrahepatic Intrahepatic  1. Age 10-25 years > 40 years  2. H/o alcohol Absent Strongly positive  3. H/o jaundice Absent Present  4. Sex More in females More in males  5. Splenomegaly Moderate to big Mild to moderate  6. Ascites Absent Present  7. Liver cell failure Absent Present  8. Encephalopathy Nota feature It is common   ¢ Enzymes SGOT (serum glutamic oxaloacetic trans-  aminase) and SGPT (serum glutamic pyruvic acid  transaminase) are increased in hepatocellular failure.   ¢ Prothrombin time, bleeding time and clotting time must  be checked before a liver biopsy is done.   ¢ Liver biopsy is done provided bleeding profile is normal.  However, injection vitamin K 10 mg, IM ts given for 3  to 5 days.    3. SreST( AEC aan e NT ASSDASE DY (OGD)  ¢ To confirm oesophageal varices which appear as a bluish  red longitudinal column in the lower end of the  oesophagus. When they extend to the stomach, they are  called gastric varices (Figs 24.50 to 24.52).   A fine lumbar puncture needle is passed through the 9th  space in the left midaxillary line into the spleen and a  radiopaque dye injected into the splenic pulp which fills up  the entire portal system.    ENDOSCOPIC GRADING OF VARICES BASED S  ON VARIX HEIGHT AT 2 CM FROM OG JUNCTION   ¢ To know the anatomical configuration of portal system,  so that a proper shunt operation can be done.   ¢ To assess the diameter of splenic vein. When it is more  than 1 cm, it is an indication for splenorenal shunt (lieno-  renal shunt).   ¢ If the needle is connected to a manometer, splenic pulp    5. Ultrasonography  To assess the nature of the liver (cirrhosis), to know the  portal vein, portal cavernoma, splenic vein diameter, etc. It  has become a valuable investigation today.    ¢ Variceal bleeding can occur even without any specific cause.  The bleeding can be very minor in the form of occult blood  in the stools to massive bleeding. Following hypothesis has  been postulated for massive bleeding from varices.  Eruptive theory: Due to increased intravariceal pressure.    ¢ Reducing portal pressure by at least 20% or HVPG < |  mmHg is associated with significant protection against  bleeding.    ¢ The dose of B-blocker is titrated to maintain heart rate  around 55 beats/min or reducing by 25% from baseline  rate.    Endoscopic band ligation of varices is better than  sclerotherapy. It 1s very effective and associated with less  side-effects.   Indicated in patients who do not tolerate B-blockers and  varices which are large and are associated with red wheal  markings.   The different forms of treatment and their success depends  upon various criteria. The most popular of these is the  Modified Child’s criteria (Table 24.10). Serum albumin,  bilirubin, ascites, encephalopathy and prothrombin time are  included under this criteria.    Modified Child’s criteria Grade A = 5-6 points,  Grade B = 7-9 points, Grade C = above 10 points   . Albumin g/L 35 28-35 < 28  2. Bilirubin mol/L 15-30 30-45 > 45  3. Ascites Absent Easily Not    - Bleeding manifests as haematemesis or malaena. ||, Treatment of massive bleeding from varices    ¢ Admission, hospitalisation, preferably in an intensive care  unit, elevation of foot end of the bed to increase venous  return.   ¢ Intravenous cannulation or a venous cut down and  replacement of fluid till blood is ready.    This 1s aimed at reducing portal pressure and consequently  intravariceal pressure.    Bowel wash Is given to decrease blood from gut, to  decrease ammonia and uric acid levels so as to prevent  encephalopathy.   Oral neomycin | g, 6th hourly as intestinal antiseptic.  Oral lactulose 30 ml, 8th hourly is given. It also helps in  decreasing encephalopathy.   Injection Vasopressin, 20 units in 200 ml of saline 1s  given intravenously over a period of 20 minutes. This  dose avoids coronary and peripheral vasoconstriction. By  causing powerful splanchnic vasoconstriction, it  decreases the blood flow to the varices and reduces  bleeding in about 50% patients.    Injection Somatostatin 250 ug IV bolus followed by  an infusion of 50 ug/h. It is very effective and has no  side-effects. However, it 1s costly.    Injection Octreotide and Terlipressin are preferred  because of their long half-life. They act by producing  splanchnic vasoconstriction. Thus, they decrease the  portal pressure. Once started, they have to be continued  for 2—5 days. Once bleeding stops, the varices are treated  with endotherapy to prevent rebleeding.    Metoclopramide 20 mg IV arrests the bleeding by  constricting gastro-oesophageal sphincter.    Today, variceal banding has become the gold standard for  oesophageal varices because of their efficiency in  controlling the bleeding and less chances of complication.  In 10% of the cases, rebleeding can occur.    2% ethanolamine oleate or sodium tetradecyl sulphate  (STD) is used, 3—5 ml into each varix (intravariceal) or  0.5 ml into the side of varices (paravariceal)   Bleeding can be controlled in about 80—90% of patients.  If the bleeding continues, repeat injections can be given.    If the bleeding continues in spite of the above measures,  balloon tamponade ts done.    Gastric varices cannot be managed by sclerotherapy.  Multiple oesophageal ulcers can develop at the injection  site.   . It can cause mediastinitis, left pleural effusion.  .Chances of rebleeding are high. Hence, repeated   e Complications such as perforation or stricture and massive  bleeding can occur    ¢ Balloon tamponade must be used as a rescue procedure  and to bridge more definitive therapy in case of  uncontrolled haemorrhage.   ¢ Sengstaken’s tube or its modification, the Minnesota tube  is used. It acts directly by internal tamponade. It 1s passed  through the nose. Gastric balloon is distended with up to  250-300 ml of air and oesophageal balloon with about  50-60 ml of air or to get a pressure of 20-30 mmHg. The  tube cannot be kept in place for more than 12 to 24 hours  as it can cause pressure necrosis. The method is reliable  only in experienced hands.   ¢ Consists of vascular placement of expandable metal stent  across the tract created between hepatic vein and major  branch of portal system.   ¢ TIPSS helps a small group of patients (5 to 10%) who  have refractory bleeding.   ¢ Incidence of encephalopathy is similar to surgical shunts.  (See Figs 24.59 for management of acute oesophageal  variceal bleeding.)    Devascularisation procedures are aimed at reducing the  pressure in the varices by disrupting the blood supply and  Shunt procedures are aimed at reducing portal pressure.    1. Oesophageal transection  ¢ It is done through left thoracoabdominal route called  ‘Milnes-Walker operation’.  ¢ During the transection, varices are disconnected, bleeders  are ligated or under-run, followed by oesophagogastric  anastomosis.  2. Gastric transection of Tanner  In this operation the division is made in the stomach through  abdominal route.  3. Sugiura and Futugawa operation  It is an extensive devascularisation procedure.  Aim  To interrupt the intramural and submucosal veins.  Procedure (Figs 24.60 and 24.61)  In this operation, the following procedures are done:   e Devascularisation of the greater curvature of the stomach and  devascularisation of lesser curvature of the stomach as in highly  selective vagotomy.   ¢ Oesophagogastric transection and suturing by using EEA (End  to End Anastomosis) staplers. It is a major surgery. Mortality  is around 10-15%. It is the most effective devascularisation.    1. Continuing variceal bleeding in spite of sclerotherapy wherein  the general condition of the patient is good (Child’s A,  Child’s B).   Fig. 24.60: Sugiura and Futugawa operation (splenectomy with  devascularisation). Ideally done through thoracoabdominal Fig. 24.61: Usage of EEAstaplers for devascularisation procedure  incision (Courtesy: Prof U Santhosh Pai, KMC, Manipal)    2. As an elective procedure in patients who had bleeding in  the past.   3. The selection of shunt is based on the availability of a good  vein. Jaundice, ascites and low albumin are contrain-  dications for shunt surgery.   ¢ Portal vein is divided from the liver and the end 1s  anastomosed to the side of inferior vena cava (IVC).   ¢ This controls the bleeding in 90-95% of patients but  chances of encephalopathy is 30% because of sudden  deprivation of liver blood supply.  Indications: Cirrhosis of liver due to schistosomiasis,  provided liver function is reasonably good.   2. Side to side portocaval shunt: Side of portal vein is  anastomosed to side of inferior vena cava (Fig. 24.63).  Advantage: Incidence of encephalopathy is 10%.  Disadvantage: Control of bleeding 1s about 50-70%.    In this operation, spleen is removed. Then, proximal end of  the splenic vein is sutured to the side of renal vein in the  retroperitoneum. This 1s preferred in children.    * Extrahepatic portal hypertension with portal vein  thrombosis, provided the splenic vein is dilated more than  I cm.    ¢ It can also be done in cirrhosis of liver. Chances of  encephalopathy is almost minimal after this procedure.    In this operation, distal end of splenic vein 1s divided  and sutured to the side of renal vein in the retroperito-  neum. Postoperatively, ascites can occur due to extensive  retroperitoneal dissection which damages lymphatics.    Indications  I. Splenic vein is not dilated or thrombosed.  2. Portal vein is thrombosed    ¢ The inferior vena cava is divided and its proximal  end is sutured to the side of superior mesenteric vein  (SMV).    ¢ Otherwise, a dacron graft can be placed between IVC and  SMV. This is described as mesentericocaval H-graft or jump  graft.    Shunt procedures have to be undertaken only by an  experienced surgical gastroenterologist in an institution.  These patients have to be carefully monitored in the  postoperative period for possible blockage of shunt.    ¢ Ascites in portal hypertension is a slow, insidious  accumulation of free peritoneal fluid, an occurrence in  advanced liver disease (Key Box 24.21).   ¢ When there is a doubt regarding the diagnosis, laparoscopy  is advisable which not only detects and confirms cirrhosis  but also rules out other intra-abdominal pathologies  (malignancy, tuberculosis, etc.).    1. Diuretics  K* sparing diuretics such as spironolactone or frusemide  can be given. However, hyponatraemia and hypokalaemia  should be monitored.    2. Restriction of salt intake: 20 mg/day 1s advised.  3. Peritoneovenous shunt: Le Veen shunt    ¢ Le Veen shunt is done by using a silastic tube to insert  into ascites. It 1s then tunnelled subcutaneously to the  neck and inserted under vision into internal jugular veins.   CAUSES OF ASCITES IN  PORTAL HYPERTENSION  ¢ Reduction in plasma albumin  ¢ Central hypovolaemia  ¢ Retention of salt and water by kidneys  ¢ Portal hypertension    circulation with each respiration because of the presence  of a one-way valve.    1. All cases of cirrhosis should undergo upper gastro-  intestinal endoscopy to rule out varices.    2. Almost all cases of varices should receive propranolol  prophylaxis. Propranolol prophylaxis reduces the risk of  bleeding in varices by at least 10%.    3. Acutely bleeding oesophageal varices should be first  treated with octreotide and endoscopic treatment.    4. Endoscopic sclerotherapy, and variceal banding should  be tried in all patients with variceal bleeding. Banding is  safer than sclerotherapy.    5. One should consider TIPSS if available, when  pharmacotherapy and endoscopic therapy fail.    6. One should consider Sengstaken-Blakemore tube if  endoscopic facilities and TIPSS are not available although  it is associated with a high rate of recurrent bleeding.    7. Inexperienced surgeon should consider oesophageal  transection to tide over bleeding varices resistant to  octreotide and endoscopic treatment.    9. Surgical shunts should be considered for Child’s A  and B category.  10. In Child’s C category, one should consider TIPSS  followed by liver transplant.    ¢ Due to increased portal pressure and collaterals, vascular  dilatation and ectasia occur in the stomach.   ¢ Endoscopically they appear pink speckled with red mosaic  like pattern in the gastric mucosa.   ¢ They are responsible for about 3.5% of cases of upper Gl  bleeds and 15% in portal hypertension cases.   e They can present as acute bleeding with needs to be  managed conservatively.   ¢ Propranolol is used to reduce portal pressure which in turn  reduces bleeding.   ¢ It refers to bile duct changes due to portal hypertension.   ¢ Portal cavernosa, ischaemic injury to the bile duct are the  causes. Infection is also one of the causes.   ¢ Changes in the bile duct include strictures and dilatation of  both extra and intrahepatic bile ducts secondary to varices  encircling CBD and gall bladder wall. Choledocholithiasis  is common.   ¢ Can present with bile duct obstruction, cholangitis and  bleeding into the CBD.    1. Congenital: It is a potentially curable condition where in a  web exists in the suprahepatic portion of inferior vena cava.    1. Acute form is dangerous, resulting in rapid enlargement  of liver, severe abdominal pain, vomiting and hypotension.  2. Chronic form resembles cirrhosis  ¢ Hepatomegaly—firm, can be irregular, dilated veins over  the abdominal wall. Bilateral pedal oedema which can  be irritating, signs of liver cell failure are seen.  Haematemesis occurs later.    In congenital cases, if web can be proved by venacavography,  it can be excised by transatrial meatotomy. However, in general,  prognosis 1s very poor. Peritoneovenous shunt has to be  considered as an alternate method to drain the ascitic fluid  into one of the veins in the neck (internal jugular vein).    ¢ Long-term prophylaxis is 100 ug s/c tds for 15 days.   3. Bowel dysfunction—AIDS-related diarrhoea, short bowel  syndrome, dumping syndrome, chemotherapy-induced  diarrhoea.   6. Advanced malignancies: Hepatocellular carcinoma,  metastatic breast cancer, colonic cancer, pancreatic cancer.   e¢ Acute fulminant hepatic failure (FHF): Causes are para-  cetamol overdosage, acute hepatitis B infection, Wilson’s  disease, drugs.    Types  1. Living donor transplant: Easily available, no waiting  period but potential risk to donor (normal person) Is present.  2. Cadaveric: Preserving organ by hypothermia (Key Box 24.23)  e Waiting period  ¢ No risk to donor  ¢ To be done within 12 hours, ideally within 6 hours.    ¢ Partial hepatectomy: Usually right lobe (60% of total liver) LFT, USG abdomen, selective arteriography, upper GI  ¢ Split liver transplant: Divide into two functioning units and endoscopy.   Definition WHAT IS NEW IN THIS CHAPTER? / RECENT ADVANCES be  Bleeding from liver into the biliary tree. There is usually a   communication between a blood vessel and the bile duct ¢ All the topics have been updated    . Which of the following statements is false about hepatic  encephalopathy?    . Which of the following is not the cause for over-  production of bilirubin?    . The following are indications for percutaneous method  for drainage of pyogenic liver abscess except:    . Adifferentiating feature of amoebic liver abscess from  acute cholecystitis is:    . The drug of choice for the treatment of amoebic liver  abscess is:    A 65-year-old patient presents with the following:  Serum bilirubin: 4 mg, serum albumin: 1.8 mg,  INR: 2. He has gross ascites and is drowsy. What Child-  Pugh Class does he belong to?    Which of the following is the commonest benign  tumour of the liver?    For purposes of hepatic resection, the liver can be  divided into number of segments.   The following conditions are associated with increased  risk of hepatocellular carcinoma except:  A. Hepatitis B carrier state    Up to _ segments of the liver can be resected  safely without the danger of hepatic failure provided  rest of the liver is normal.    Which of the following is true criteria for secondaries  in the liver?    Commonest gastrointestinal tract primary in a nodular  liver secondaries is from:   A. 20 units is diluted in 200 ml of saline and given over  20 min    22. Portal hypertension is defined as a portal venous A. Carcinoma stomach  pressure exceeding mmHg. B. Carcinoma pancreas  Aa 3 B. 10 C. Portal hypertension  C. 15 D. 20 D. Hepatocellular carcinoma  ANSWERS  1D 2C 3A 4D 5 bp 6A 7A 8C 9D 10 A  i B 12 C 13 C 14 A ae 8) 16 A 17 C 18 B 19 D 20 D  21A 22 C 23 £ 24D 25 A 26 C    One may wonder why gall bladder and pancreas are  discussed together!! Yes, biliary tract (gall bladder is an  important part of that) ends after joining with pancreatic  duct by opening into 2nd part of duodenum.   Gall stones, when they block/pass through ampulla of Vater,  produce acute pancreatitis.   An important surgical condition—surgical jaundice or  obstructive jaundice can be discussed better only if you  know gall bladder and pancreas.   Any surgery involving sphincter of Oddi in the form of  sphincteroplasty/or removal (as in Whipple’s pancreati-  coduodenectomy), the gall bladder does not function and  hence it needs to be removed.   Embryologically, liver, biliary tree, ventral pancreas, gall  bladder—all developed from a diverticulum on the ventral  aspect of the foregut of the embryo.   And gall bladder will be palpable in cases of periampullary/  carcinoma head of the pancreas.    Gall bladder is a pear or globular shaped organ present in the  right hypochondrium on the inferior surface of the liver,  situated in the gall bladder fossa. It is about 8—12 cm long.   Chronic pancreatitis  Cholangiocarcinoma  Congenital biliary atresia  Carcinoma of gall bladder  Carcinoma of pancreas  Endocrine tumours    Fundus: It is the dilated portion of the gal Ibladder adherent  to undersurface of liver from which it can be separated  easily.   Neck: The narrow angulated distal portion of the neck is  called Hartmann’s pouch—common site where stones  occur and tend to stay for a long time (also called  infundibulum of gall bladder) (Fig. 25.1).   Gall bladder drains into the common bile duct (CBD)  through cystic duct, which is 3 cm long. It 1s lined by  cuboidal epithelium. There are prominent mucosal folds  within the cystic duct due to the presence of prominent    circular muscle fibres underneath. Its lumen is usually 1-3  mm in diameter. Contraction of gall bladder produces a  functional valve called valve of Heister which prevents the  migration of stone into the CBD. The wall of cystic duct is  surrounded by a sphincter structure called sphincter of  Lutkens. A spiral fold keeps cystic duct open for drainage  of bile.    ¢ Lateral: Cystic duct and gall bladder  ¢ Medial: Common hepatic duct  ¢ Above: Inferior surface of right lobe of the liver.   It is an important landmark in the identification of cystic  duct, and cystic artery during cholecystostomy so as to avoid  damage to extrahepatic biliary tree.    ¢ Right hepatic artery and its branch, the cystic artery  ¢ Cystic lymph node of Lund.    Cystic artery, a branch of right hepatic artery arises behind  the common bile duct. Soon, it branches out over the surface  of gall bladder. Cystic artery is an end artery (Fig. 25.2).  Multiple small veins from the surface of gall bladder join the  liver surface. There is also a cystic vein, from the neck of gall  bladder draining into portal vein directly. This explains early  spread of gall bladder malignancy to the liver.    1. Subserosal and submucosal lymph nodes drain into cystic  lymph node of Lund and from here they drain into nodes  in the hilum of liver and coeliac nodes.   2. Subserosal lymphatic vessels of gall bladder are also  connected to subcapsular lymph channels of liver, which    55  accounts for frequent spread of carcinoma gall bladder to  the liver.    ¢ Common hepatic duct (CHD) is formed by the union of  right and left hepatic ducts. It is 3 cm long, receives cystic  duct and continues as common bile duct (CBD).   ¢ Common bile duct is about 8 cm long. It has four parts:  Supraduodenal, retroduodenal, infraduodenal and  intraduodenal. Along with pancreatic duct, it forms ampulla  of Vater. Controlled by sphincter of Oddi, it ends by an  opening into the second part of duodenum (Fig. 25.3).    Fig. 25.3: Surgical anatomy of the bile ducts. Note how close  gall bladder is to the duodenum (chances of injury in laparoscopic  cholecystectomy)    ¢ Reservoir for bile: Bile excreted by the liver is stored 1n  the gall bladder as total of about 500 to 1000 ml per day. At  fasting, the tone of sphincter of Oddi 1s high. Food contents  in the duodenum stimulates release of cholecystokinin,  which causes gall bladder to contract.    ¢ Concentration: Bile is 98% water. Due to active absorption  of water, sodium chloride and bicarbonate, bile gets  concentrated 5—10 times. Thus, a relative increase in bile  salts, bile pigments, cholesterol and calcium occurs.    ¢ Mucus secretion: [t secretes about 20 ml/mucus per day.  Obstruction to the cystic duct causes mucocoele of the gall  bladder.    ¢ Fatty food stimulation releases cholecystokinin, which  stimulates gall bladder to contract and at the same time,  sphincter of Oddi to relax.   ¢ It also has inorganic ions (more than plasma) and hence,  severe electrolyte imbalance is seen in biliary fistula.   ¢ Cholesterol, synthesised in the liver, gives rise to bile  acids——cholic and chenodeoxycholic acids. They are meta-  bolised in the colon to deoxycholic acid and lithocholic  acids.   e¢ Main function of bile acids in the bile is to maintain  cholesterol in solution.   A. Floating gall bladder: Results due to long mesentery. It is  more vulnerable to torsion—a rare cause of recurrent upper  abdominal pain. Such a gall bladder can be easily removed.   B. Phrygian cap: Cap which was worn by people of Phrygia  (ancient Asian country, Mongolia). It is an anomaly  connected with the fundus of the gall bladder.   C. Double gall bladder: The second one is always intrahepatic  (rare).   D. Absence of cystic duct: Cholecystectomy becomes  difficult. There are high chances of injury to the common  bile duct.   E. Low insertion of cystic duct: Cystic duct opens into the  common bile duct near the ampulla. This anomaly should    be kept in mind when operating on cases of obstructiv  jaundice.  F. An accessory or aberrant cholecystohepatic duct 1  present in about 10% of the patients. It may be the cause o  significant bile leakage after cholecystectomy. It is th  segmental duct that joins biliary system outside the live  instead of within it.  G. Diverticulum of gall bladder  H. Cystic duct joining right hepatic duct  I. Anomalies of blood supply  1. Very, very tortuous hepatic artery: Caterpillar turn o  Moynihan’s hump. It runs in front of the origin of cysti:  duct.    ¢ Cholesterol is produced from the liver which gives rise tc  bileacids. Cholesterol is insoluble and it must be transportec  within the bile salt micelles and phospholipid (lecithin  vesicles. Normal ratio of bile acids: cholesterol is 25:]  (Fig. 25.5).   ¢ This ratio is necessary to maintain the cholesterol in liquic  form by forrning micelles. When the ratio drops down tc  13:1 (which is called critical ratio), cholesterol crystals will  nucleate and stones will form.    ¢ Obesity, high calorie diet and medications which increase  cholesterol secretion can result in stone formation.   Fig. 25.4: Congenital anomalies of the gall bladder (see text A to 1)    ¢ Itis the most common cause responsible for a gall stone in  80% of patients. Sources of infection are tonsils, tooth,  bowel, etc.   ¢ Organisms such as E. coli, Proteus, anaerobic organisms,  Streptococci, etc. reach the gall bladder wall through the  blood stream and form a focus/nidus around which  cholesterol and bile salts get precipitated.   ¢ Over a period of many years, this results in a mixed stone.  They are usually multiple and occur in infected bile.    ¢ Pregnancy, oestrogens, following vagotomy and prolonged  total parenteral nutrition are associated with bile stasis.    CAUSES OF DECREASED BILE ACID POOL s  . Cirrhosis of liver—pigment stones   ¢ Bilirubin production ts increased because of increased  break down of RBCs. Since the production 1s more, they  cannot conjugate with glucuronic acid, which 1s produced  at normal levels.   ¢ Such unconjugated bilirubin combines with calcium and  is excreted in the biliary tree resulting in calcium bilirubinate  stones (pigment stones) not only in the gall bladder but  also in the entire ductal system.    Gall stones (can occur along with two other conditions  mentioned below)    In Oriental countries, Clonorchis sinensis (Chinese liver  fluke) infestations can cause stone tn the billary tree.  Ascaris lumbricoides in the biliary tree may produce stones  In our country (India).    It is produced in congenital cystic fibrosis. Gall stones occur  in these children due to impairment of bile flow.    Risk factors for gall stone disease  (Key Box 25.2 and Fig. 25.6)   ¢ It is single, solitary, occurs in aseptic bile. Sometimes  they can be multiple. Precipitation of cholesterol gives  rise to stone.    Clinical presentation of these patients vary from dyspepsia tc  severe forms such as pancreatitis and perforation of the  gall bladder (Key Box 25.3). Classified as in the gall bladder  in the CBD and in the intestines.    e Such stones can be silent for many years. They are  radiolucent.  e Pigment can also get precipitated along with cholesterol.   ¢ Composed of calcium bilirubinate, calcium palmitate and  calcium stearate + cholesterol.   ¢ Occur due to bile stasis caused by foreign bodies, endo-  prosthesis, Clonorchis sinensis and Ascaris lumbricoides.    ¢ They constitute about 80% of gall stones.  ¢ They contain alternating layers of cholesterol and COMPLICATIONS IN THE GALL BLADDER    infective organisms. e This is usually a single, silent, cholesterol stone which 1s  ¢ They are multiple, small, faceted by mutual pressure. symptomless.  ¢ It 1s accidentally discovered, may be by an ultrasound or  plain X-ray abdomen (since calcium content is low in a  ¢ Th found in 5 to 10% of patient 4 ‘sible 4  ae TAL NONE le er eo a eet cholesterol stone, it is very rarely visible in a plain X-ray).    ¢ Bacteria also have a major role to play in the formation discomfort in the abdomen, heartburn and belching, she  of pigment stones. Patients with pigment stones have probably has gall stones. These patients benefit from  more sepsis than patients with cholesterol stones. cholecystectomy.   Fig. 25.13: Multiple 2 mmstones. This ladyhad 25.14: Gallbladder Figs 25.15 and 25.16: Gall stones: Multiple stones are  3 attacks of gall stone pancreatitis was opened to responsible for obstructive jaundice  extract stones    e It usually occurs at night wherein a stone tends to block the  cystic duct or neck of gall bladder in the supine position.   ¢ [tis a severe colicky upper abdominal pain felt in the right  hypochondrium, may shoot to the back or between shoulder  blades. The pain is continuous and lasts for a few hours.  Pain may radiate to chest also.  — The pain is due to spasm of gall bladder  — Itis associated with vomiting due to reflex pylorospasm,   ¢ Pain may last for a few minutes to a few hours.   qs l= 10) Oto}  DIFFERENTIAL DIAGNOSIS OF GALL  STONE COLIC  e Chronic duodenal ulcer  e Reflux oesophagitis (some cause can present as precordial  chest pain)  e Pancreatitis  e Myocardial infarction    Definition: Acute bacterial inflammation of the gall bladder  with or without stone.    1. Calculous: Obstructive cholecystitis. It is the commonest  variety. Calculi cause bile stasis.   2. Acalculous: Nonobstructive cholecystitis. It is not  uncommon and is seen in patients who are recovering from  major illness (Key Box 25.5).    1. Majority of the cases of calculous cholecystitis are due  to organisms such as E. coli, Streptococci, Salmonella,  Klebsiella, etc.    e n life-threatening situations with severe sepsis due to gall  stones, percutaneous cholecystostomy seems to be a very  good alternative to save the life of the patient.   e Indicated in acalculous cholecystitis in which the patient's  condition is serious with sepsis with co-morbid conditions.   e Using ultrasound or CT guidance, a pigtail catheter can be  inserted into the gall bladder, ideally transperitoneally.   e 3-4 days later, when sepsis improves, laparoscopic/open  cholecystectomy can be done.    2. Typhoid fever can also cause Typhoid Cholecystitis aroun:  2nd week of infection (Key Box 25.6).    e Acute cholecystitis can occur in the 2nd week of typhoid  fever.   e Bacilli can be present in the bile for a long time   e Local tenderness, on the right side of abdomen, guarding  can occur.    ¢« Acute calculous cholecystitis appears to be caused by  obstruction to bile flow from gall bladder by stone or  oedema formed as a result of local mucosal erosion and  inflammation caused by stone. Once mucosa is eroded,  tissue planes are exposed to bile salts. Toxic bile salts  destroy cells by their detergent action leading to necrosis  and perforation of gall bladder.    ¢ At the same time, bacterial infection adds to the morbidity    of acute cholecystitis. Positive bile cultures are found in  70% of cases of acute calculous cholecystitis.    1. Inflammation: Entire gall bladder is inflamed, swollen and  is friable. When the inflammatory exudate surrounding the  gall bladder collects under the diaphragm, it results in pain  radiating to the right shoulder (C3, 4) due to phrenic nerve    Fig. 25.17: Diagrammatic representation of the complications of gall stones. They are discussed in the subsequent pages    irritation. It may undergocomplete resolution with antibiotic  therapy but such recurrent attacks are common at a later  date (Fig. 25.17).   2. Perforation: Extensive ulcerations of gall bladder may  result in perforation with biliary peritonitis and carries a  very high mortality rate. Perforation can occur when the  stone is impacted in the Hartmann’s pouch.   3. Obstruction to the neck of gall bladder results in muco-  coele or pyocoele (empyema). Empyema of the gall bladder  can occur in diabetic patients and 1s associated with high  grade fever, chills, rigors and even septicaemia.   4. Gangrene of gall bladder can occur if the blood vessels get  thrombosed. All these features are more in an obstructive  variety.  ¢ If there is clostridial infection as can occur in diabetics   because of extensive gas production in biliary tree and  associated toxicity, perforation is likely even without a  Stone.    e A fatty, fertile, female is the typical victim who presents  with severe upper abdominal pain (Figs 25.18 and 25.19).  The pain is colicky in nature and more prolonged because  of inflammation. Severe nausea and vomiting are present.  In the initial phase, there is low grade fever, except in  clostridial infection where there is high grade fever.   ¢ You may be surprised to find a young boy, a girl or even a  child with gall stones—suspect haemolytic anaemias.    Signs  |. Murphy’s sign  ¢ Keep the fingers in the right hypochondrium and ask the  patient to take a deep inspiration.    ¢ At the height of inspiration there is a sudden catch in the  inspiration.   ¢ It 1s due to inflamed gall bladder coming in contact with  the abdominal wall under the fingers and producing pain.  This is called Murphy’s sign positive. It is a diagnostic  sign of acute cholecystitis (Fig. 25.20).    2. Boas’ sign: An area of hyperaesthesia between 9th and 11th  ribs posteriorly on the right side is a feature.  3. Upper abdominal guarding, rigidity.    4. Vague mass consisting of inflamed gall bladder, omentum,  inflammatory exudate can be felt at times. Hence, even if a  perforation occurs, generalised peritonitis is uncommon.    Perforated peptic ulcer: Severe sudden pain, severe  tenderness in the right hypochondrium, guarding and  rigidity caused by perforated peptic ulcer, can mimic acute  cholecystitis. Obliteration of liver dullness, coffee ground  vomitus, generalised guarding and rigidity clinches the  diagnosis of perforated duodenal ulcer.    tenderness in the right hypochondrium and epigastrium  mimic cholecystitis. One should remember that pain of  pancreatitis is more severe and classically radiates to back.    is in the subhepatic position. Once inflammatory fluid  spreads in the general peritoneal cavity, there will be more  difficulty in diagnosing clinically.    Lobar pneumonia (basal): Can cause right hypochondriac  guarding and rigidity. It is a referred pain.   ¢ Gall stones can be demonstrated in 10% of the patients  as radio-opaque shadows in the right hypochondrium.  In lateral view, the stone is seen in front of vertebral bodies.   ¢ To rule out other causes such as perforated peptic ulcer  (air under diaphragm).   ¢ Rarely, it may show calcified gall bladder (porcelain gall  bladder).   e It can demonstrate inflamed, thickened organ, in cases  of acalculous cholecystitis.   ¢ Ultrasound can also measure gall bladder function by  using ultrasonic dimensions of the gall bladder.    ¢ HIDA is hepatic iminodiacetic acid.  ¢ 9™To labelled HIDA agent is excreted in the biliary tree,  within one hour following IV administration.   Fig. 25.24: HIDA scan showing nonvisualisation of gall bladder—  a case of acute cholecystitis (CT is more specific)    e In acute cholecystitis, even though the dye 1s excreted in  the biliary tree, it does not enter the gall bladder due to  oedema of the cystic duct. Hence, nonvisualisation of  gall bladder 1s diagnostic of acute cholecystitis.   ¢ Its importance lies in the diagnosis of acalculous  cholecystitis and when diagnosis 1s in doubt.   ¢ It not only diagnoses gall stones, but also detects other  complications such as perforation, stones 1n the CBD,  etc.    |. Conservative treatment is followed in majority of  the cases (60 to 70%)    2. Aspiration with Ryle’s tube: Aspiration of HCI decreases  the stimulus to the secretion of bile. Spasm of gall bladder  may come down.   3. Antispasmodics: Injection morphine 8-10 mg IM as  analgesic along with injection atropine 0.6 mg to relieve  spasm of sphincter of Oddi.   4. Antibiotics: Broad spectrum antibiotics are given against  gram +ve, gram —ve and anaerobic organisms. Cefazolin,  cefuroxime or amikacin are the drugs of choice. The patient  is kept nil orally for 2—3 days and during this time IV fluids  are given.   After 2—3 days, pain comes down, signs (tenderness)  disappear and abdomen becomes soft. Ryle’s tube is removed,  clear oral fluid is given for 2—3 days followed by soft diet.  After 6 weeks, the patient 1s advised to undergo elective  cholecystectomy. Reason for conservative treatment 1s 1n  majority of cases, inflammation will settle down.    e Patients in the first group need two admissions, cost 1s  increased and return to work is also delayed.   * Hence if a surgeon is experienced and the set up 1s good, one  can proceed to early cholecystectomy from 2nd day to 7th day.   e It has been proved that even though gall bladder 1s inflamed,  complications are no way more than elective cholecystec-  tomy inthe hands of experienced surgeon while performing  laparoscopic cholecystectomy.   ¢ Thus, if a firm preoperative diagnosis is established and  some of the comorbid conditions are corrected (diabetes,  hypertension, etc.), surgery can be done safely. This is called  early cholecystectomy.    * About 10% of cases of acute cholecystitis require  emergency cholecystostomy.   e In these patients, high grade fever, sepsis, shock, high  leukocyte count are the deciding factors.   ¢ Acalculous cholecystitis and perforated gall bladder with  peritonitis are definitely strong indications for emergency  cholecystectomy.   ¢ Contributing factors for death are diabetes, age above 60  years, cardiovascular or pulmonary disease.    PROPHYLACTIC CHOLECYSTECTOMY  e Diabetic patients  e Congenital haemolytic anaemia  e Patients undergoing bariatric surgery    ¢ Recurrent attacks of cholecystitis will convert the gall  bladder into a fibrosed, nonfunctioning, contracted,  shrunken, small gall bladder. Gall bladder wall is grossly  thickened. Stones are invariably present. Such patients  present with classical fatty food intolerance. Murphy’s sign  1S positive.   ¢ They are diagnosed by ultrasound which reveals a small,  contracted gall bladder. Otherwise, oral cholecystography  (OCG) can be done to know the function of the gall bladder.    cholesterol crystals in the mucosa/submucosa. Yellov  specks of cholesterol crystals are seen when gall bladde  is opened (xanthogranulomatous) (Key Box 25.9 anc  Figs 25.26 and 25.27).    thickening and hyperplasia of the gall bladder. All layers  of gall bladder are thickened.    . Diverticulosis of gall bladder  . Gall bladder wall with fistula   Uncommon group of conditions affecting gall bladder in which  there are chronic inflammatory changes and hyperplasia of  all tissue elements.    XANTHOGRANULOMATOUS s  CHOLECYSTITIS  e It is a pathological diagnosis  e Gall bladder is chronically thickened, irregular with extension  of yellow xanthogranulomatous inflammation to adjacent  organs.    e tis thought to be due to bile penetrating gall bladder wall    e It occurs when there is a stone blocking the cystic duct and   As aresult of obstruction, all the bile within the gall bladder  is absorbed and is replaced by the mucus secreted from  gall bladder epithelium.    ¢ Clinically, it results in a soft, fluctuant, globular mass in   Patients present with high grade fever with chills and rigors,  toxicity, high leukocyte count.   Perforation can cause local abscess, if there are adhesions  due to previous inflammation.   Perforation into the general peritoneal cavity is rare but  produces diffuse biliary peritonitis which has a high  mortality rate.   Urgent laparotomy, aggressive resuscitation, good antibiotic  cover may help in reducing mortality. At laparotomy,  removal of gall bladder is difficult. Hence, drainage of the  pus and cholecystostomy with removal of gall stones can  be done (Figs 25.31 A and B).    Long-standing gall stones can bring about squamous  metaplasia of gall bladder epithelium and can cause carcinoma  of gall bladder. However, the incidence is very low. Hence,  routine cholecystectomy is not advised for silent gall stones.    Type I: Compression of CBD without lumen narrowing.  Type II: Compression of CBD with lumen narrowing.   Type IV: Stone ulcerating into CBD resulting in cholecysto-  choledochal fistula.    ERCP (Fig. 25.34) followed by open cholecystectomy wa:  the most popular method till recently, now replaced by  laparoscopic cholecystectomy.    It has become the most popular choice today. More than 95%  of gall bladders can be removed through a laparoscope. Some  principles and procedure of laparoscopic cholecystectomy are  discussed below (Figs 25.35 and 25.36).  Conversion to open when:  ¢ Very badly contracted, fibrosed gall bladder.  ¢ Very difficult gall bladder—Calot’s triangle anatomy is  not defined well. Dissection may cause injury to the bile  ducts and can cause stricture. Partial cholecystectomy  is a Safe alternative (Fig. 25.37).  Procedure    ¢ 1 cm incision 1s made below the umbilicus, through which  a pneumoperitoneum Is maintained by CO, insufflation.   Fig. 25.37: Partial cholecystectomy could be done in this patient  and all stones were cleared—asymptomatic since 3 years. It is a  safe alternative to complete cholecystectomy. It is not a failure    Following this, a laparoscope is introduced and a camera  is attached. Three small, 1 cm incisions are made in  epigastrium and 1/2 cm in the right hypochondrium. These  are used for suction, instrumentation, cauterisation,  dissection, retraction purpose—dissection port.   Cystic duct and cystic artery are clipped and gall bladder is  removed using gall bladder-holding forceps and is brought  outside through the epigastric port (Figs 25.38 to 25.40).  Bleeding from liver is controlled using lasers/cautery.  The procedure is done under general anaesthesia. It may take  1—3 hours depending upon the experience of the surgeon.    . Hospital stay is 1-2 days, recovery is very fast.  . Pain is minimal. Hence, mobilisation of the patient is much    better and easy.  It gives an acceptable and better cosmetic result.   ¢ Patients with functioning gall bladder proven by OCG or  scintigraphy.    Drawbacks  ¢ Recurrence of stones once treatment is stopped  ¢ Life-long maintenance is needed.    ¢ After dissolution of the stones, lithotripsy or extracorporeal  shock wave lithotripsy should be done.   ¢ Mechanism of action: They inhibit HMG-CoA, a rate  limiting step in the synthesis of cholesterol, thereby  increasing the bile salt pool. UDCA also acts by decreasing  cholesterol absorption in GIT.    MTBE—methyl terbuty! ether is the drug which is given  through a catheter placed in gall bladder percutaneously.    Drawbacks: Explosive and toxic if it enters bile duct or  duodenum.   ¢ The patient must have patent cystic duct (proven by OCG/  scintigraphy).    Thus we have completed gall stones and their complications  in the gall bladder. Now we will study the complication of  gall stones in the common bile duct, mainly obstructive  jaundice.   e Jaundice that occurs due to obstruction to the outflow of  bile is called obstructive jaundice.   ¢ Since these cases have to be managed by surgical  intervention, it is also called surgical jaundice.   ¢ However, haemolytic jaundice cases are not obstructive but  a few are managed by splenectomy (surgical).   ¢ Before we start a detailed discussion of obstructive jaundice,  we will study anatomy of the pancreas. This is an important  long case in the university examination. For a better  understanding of obstructive jaundice, students are  requested to understand various causes of obstructive  jaundice which are given in the subsequent pages.    The pancreas is both endocrine and exocrine organ situated  retroperitoneally behind the stomach. It is a soft and fleshy  gland (pancreas—all flesh), extending from the duodenum on  the right side to the spleen on the left side, the entire length  being 6 inches (Fig. 25.41). It weighs approximately 80 g.    ¢ The head lies within the C-loop of duodenum. The uncinate  process projects from the left inferior portion of the head  over which course the superior mesenteric vessels. There  are 5—6 small thin veins connecting this portion of the head  with superior mesenteric veins. These veins have to be  carefully divided during pancreaticoduodenectomy.   Fig. 25.41: Blood supply and parts of pancreas: (1) Uncinate  process, (2) Superior pancreaticoduodenal, (3) Coeliac artery,  (4) Splenic artery, (5) Inferior pancreaticoduodenal, (6) Head of  pancreas, (7) Body of pancreas, (8) Tail of pancreas and (9) Spleen    Superior mesenteric vein continues above as portal vein  after joining the splenic vein. During pancreatico-  duodenectomy for periampullary carcinoma, infiltration into  the portal vein should be ruled out before any major  structure is divided. This 1s done by inserting a finger  between the portal vein and head of pancreas, both from  above and below.    ¢ Body and tail: The head and neck continue as body which  is placed transversely. It slopes upwards across the aorta  and ends as tail of the pancreas, which is enclosed within  lienorenal ligament along with splenic vessels. A large  cystadenoma arising from the tail of the pancreas can move  with respiration because of its contact with the spleen.    e Splenic artery is the chief artery supplying the neck, body  and the tail. Arteria pancreatica magna refers to one large  branch of splenic artery.    e Superior and inferior pancreaticoduodenal arteries supply  not only head of pancreas but also the adjacent duodenum.  Thus, during any surgery which involves excision of the  head, the C-loop of the duodenum is also removed. Thus,  pancreaticoduodenectomy becomes a major surgery.   ¢ The head ts drained by superior pancreaticoduodenal vein  which drains into portal vein and inferior pancreatico-  duodenal vein drains into superior mesenteric vein.    ¢ The main pancreatic duct (duct of Wirsung), a tubular  structure drains entire pancreas from tail to the head. It joins  the common bile duct and forms ampulla of Vater. This  ampulla opens on the duodenal papilla (a nipple-like  elevation) inthe 2nd part of the duodenum. Normal diameter  of pancreatic duct is 2—3 mm. When it is dilated more than  6—8 mm, as in chronic pancreatitis, longitudinal pancreatico-  jejunostomy can be done.   ¢ Accessory pancreatic duct of Santorini drains the uncinate  process and lower portion of the head and opens into the  duodenum 2 cm above the opening of the main duct. The  two ducts communicate with each other at many sites.   ¢ The main pancreatic duct is lined by columnar epithelium  which becomes cuboidal in the ductules.    ¢ Bicarbonate rich, protein rich, alkaline fluid  ¢ 2.5 litres/day  ¢ Rich in proteins 15 g of protein/day  ¢ Thus acidic chyme in the duodenum ts alkalinised by  pancreatic juice  ¢ Protein is secreted by acinar cells, fluid and electrolytes by  ductal cells  ¢ Secretions are stimulated by 3 phases  a. Cephalic phase is mediated by acetylcholine—10% of  secretion  b. Gastric phase—mediated by gastrin and vagus—15%  c. Intestinal phase—75% release mediated by release of  secretion from duodenal acidification and stimulated  release of bile into 2nd part of duodenum following entry  of fat and proteins.    Choledocholithiasis and carcinoma head of pancreas  periampullary region are two important causes of obstructive  jaundice. They constitute more than 90% of cases of  obstructive jaundice treated surgically. Presence of jaundice,  high-coloured urine, clay-coloured stools and itching gives  the clue to the diagnosis. Palpable liver, palpable mass  (cholangiocarcinoma, carcinoma head of pancreas) and  palpable gall bladder (periampullary carcinoma) supports the  diagnosis. Early diagnosis by imaging, endoscopy, ERCP,  MRCP is possible. Since causes are many, they are treated   accordingly. Relief of pain, relief of jaundice with or without  resection (in malignancies) should be done early to prevent  complications such as cholangitis, liver abscesses, septicaemia,  renal failure and even death.    1. Carcinoma head of pancreas  2. Chronic pancreatitis  3. Lymph nodes at the porta hepatis obstructing the billary tree.    Differences between stone in the CBD and periampullary carcinoma (Ca)/Ca head of pancreas    First study to differentiate choledocholithiasis from carcinoma  periampullary/head of pancreas (Table 25.1).   Pain It is due to a stone blocking the CBD  resulting in spasm of CBD. It is severe  colicky pain like gall stone colic.   Fever As a result of obstruction, multiplication  of organisms results in fever.   Jaundice Occurs due to obstruction. Once inflammation  subsides all these 3 symptoms are relieved  partly but they occur after sometime. Hence,  intermittent pain, intermittent fever,  intermittent jaundice are classical of a  stone in CBD—Charcot’s triad.   Stools Since the obstruction 1s never complete,  clay-coloured stools are not commonly seen.    Loss of appetite No  Loss of weight No  5. Signs  Jaundice Deep yellow  Anaemia Absent  6. Per abdomen Liver can be enlarged due to back  pressure. It is smooth, with round  border, firm in consistency.  7. Gall bladder As a rule, gall bladder is not palpable  8. Metastasis No    50—70 years  Equally common in both sexes  Short duration (1-3 months)    There may be some discomfort in abdomen but colicky pain is   not a feature. Pain is relatively rare in carcinoma head of the  pancreas.   When obstruction becomes severe, there 1s bile stasis. Cholangitis,  fever with chills and rigors can occur.   As a result of slow developing obstruction in periampullary region,  jaundice is persistent, progressive, painless, pruritic.   In 5% of cases, growth may ulcerate into the duodenum. It can cause  melaena and jaundice may temporarily subside.    Clay-coloured stools are common and when mixed with blood, it 1s  called silvery stools or aluminium paint stools.   Liver can be enlarged due to back pressure. If it is nodular, with sharp  border, hard in consistency, it is due to secondaries in the liver.    Gall bladder is palpable in 70-75% cases.  Left supraclavicular node, ascites, etc. may be seen.    ¢ In a jaundiced patient, if the gall bladder is palpably  enlarged, it is not due to stones (Key Box 25.10). In case  of stones, previous inflammation would have made gall  bladder fibrotic and hence, will not be palpable.   ¢ From clinical point of view, 90% of cases of obstructive  jaundice are due to stones, periampullary carcinoma or  carcinoma of the head of pancreas.    Fig. 25.44: Enlarged palpable gall bladder in cases of  periampullary carcinoma   e Tensely cystic, feels firm, tender and sometimes located in  the right hypochondrium    1. Double impaction: One stone in the CBD and one stone in  the cystic duct.   3. Primary oriental cholangiohepatitis causing stones in the  CBD (gall bladder is normal in these cases).    I. Primary stones: These stones are formed in the CBD or  within intrahepatic ducts. They are multiple, pigment stones  or often mixed stones (Key Box 25.11). Various causes are:   II. Secondary stones: These stones originate from gall  bladder and stay in CBD—usually supraduodenal portion—  then get enlarged to attain large size over a period of time.   e Almost all primary CBD stones are pigment type (brown  stones).   e Associated with biliary stricture, papillary stenosis or  sphincter of Oddi dysfunction.    Less important causes are pseudocyst of pancreas, chronic  pancreatitis stenosing sphincter of Oddi, biliary parasitic  infections, etc.   Post ERCP, if stent cannot be passed as in obstructive  jaundice cases.    Biliary colic, jaundice and chills and rigors are called  Charcot’s triad.    Leukocytosis, high bilirubin levels and alkaline phosphatase  levels are diagnostic tests.    Ultrasound, CT scans, ERCP are indicated to confirm/rule  out the various causes.    Emergency endoscopic sphincterotomy, extraction of stones  in choledocholithiasis, endoscopic stoning in cases of  stenosis or stricture.    3. BT, CT, PT are altered in case of obstructive jaundice.    ¢ These are the enzymes which bring hydrolysis o  phosphate esters in alkaline medium.    ¢ Sources of alkaline phosphatase include liver, biliar:  tree, bone, intestine, kidney.    It is the product of epithelial cells of cholangioles—T levels  are due to increased enzyme production.   Intrahepatic cholestasis, cholangitis, extrahepatic obstruction  are the chief factors causing elevation.    Focal lesions in the liver—single hepatic metastasis or liver  abscess or a tumour can cause increased levels without  jaundice.    In cholangitis, bilirubin may be normal but alkaline  phosphatase may be very high.    reliable and quick investigation for obstructive jaundice.  Dilated biliary radicles, both intrahepatic and extrahepatic  can be demonstrated (First clue in obstructive jaundice).    ¢ Mass lesion in the head region can be seen in cases of  chronic pancreatitis or carcinoma head of the pancreas  causing obstructive jaundice.   ¢ Ultrasound can detect multiple secondaries in the liver,  thus, favouring the diagnosis of malignancy. Endosono-  gram can detect lymph nodes also.    . CECT (contrast enhanced CT) scan: A head mass of even    2-3 cm in size and portal vein infiltration can be  demonstrated by CT scan. Obliteration of fat plane between  the mass and superior mesenteric vessels can be demons-  trated by CT scan which decides the operability of  periampullary carcinoma or carcinoma head of the pancreas  (Figs 25.45 to 25.48).    ¢ CT scan cannot differentiate head mass of carcinoma  from chronic pancreatitis (PET scan may differentiate).    ¢ CT scan can also detect coeliac nodes, presence  of which is a contraindication for radical resections  (Key Box 25.13).   Fig. 25.45: CT scan showing hugely distended gall bladder and _Fig. 25.46: Large 6 cm mass in the head of pancreas—patient  common bile duct—periampullary carcinoma. Growth was _ not only had obstructive jaundice but also duodenal obstruction.  resectable. He underwent Whipple’s operation He underwent triple bypass   Fig. 25.47: 6 cm mass abutting mesenteric vessels andinvolving Fig. 25.48: Large head mass with obstructive jaundice with  root of the mesentery. Such masses are usually nonresectable intrahepatic dilatation. When in doubt, diagnostic laparoscopy will  help—to operate or palliate nonsurgically    eg ¢ In case of obstructive jaundice due to stones, smooth  COMPLICATIONS OF PERCUTANEOUS bulge can be seen in the second part of the duodenum.  CT-GUIDED BIOPSY OF HEAD MASS In carcinoma, ulcerated lesion can be seen.  eath 9.Barium meal follow through to see the C-loop of  nzymatic autodigestion duodenum (Fig. 25.50).  bscess ¢ In periampullary carcinoma, there may be distortion of  raumatic fistula the medial border of the duodenum giving rise to  aemorrhage Inverted 3 sign.  Remember as ¢ In carcinoma head of the pancreas there may be widening    8. Endoscopy is useful to diagnose a periampullary carcinoma graphy) (Figs 25.51 to 25.53)  which may be seen as an ulcerative lesion in the second ¢ With the help of a side viewing endoscope, ampulla of  part of the duodenum. Biopsy can also be taken which Vater 1s cannulated and a radio-opaque dye is injected.   Fig. 25.49: Endoscopy showing growth in the periampullary region.  These lesions are usually resectable   Fig. 25.51: ERCP showing stones in the CBD, stones are extracted  and stent has been placed in the common bile duct   Fig. 25.50: Barium meal showing widening of C-loop of duodenum.  It is not done nowadays   ¢ Stones appear as filling defects in the CBD or in the  common hepatic duct (CHD) (Figs 25.51 to 25.53), which  may be mobile (change position if patient is moved).   ¢ A periampullary carcinoma gives rise to an irregular  filling defect or there may be total cut off in the flow of  dye.   If stones in the CBD are diagnosed, they can be treated in    Sphincterotomy (incision of sphincter of Oddi) can be  done to facilitate extrusion of small stones.    In patients with cholangitis with obstructive jaundice,  stenting of common bile duct can be done to relieve  obstruction. Stent removal is necessary at a later date.    In selected patients with biliary strictures, stent is placed  after ERCP to relieve obstructive jaundice (sometimes  permanent in malignancies).    In selected patients with chronic pancreatitis, pancreatic  duct can be stented to relieve pain.    ERCP and stenting ts NOT indicated in operable cases  of carcinoma periampullary/head of pancreas unless  cholangitis is present.   Severe infection of biliary tree (cholangitis) and acute  pancreatitis can occur in 1-2% of the patients. Hence,  prophylactic antibiotics are given before the procedure.    missed stones in the CBD. It can also detect pancreatic  head mass, lymph nodes. Endosonoguided FNAC can also  be done (Fig. 25.54).    . Percutaneous transhepatic cholangiography (PTC):  Using an ultrasound image-intensifier, a dilated biliary  radicle is identified within the liver and a fine needle (Chiba  needle!) is introduced into it. The stylet is then removed  and a radio-opaque dye injected. Chiba needle is 15 cm  long and 0.7 mm in diameter (Figs 25.55A and B, Key  Box 25.14).    Fig. 25.54: Endosonography showing pancreatic tumour with coelic  lymph nodes (Courtesy: Dr Ganesh Pai, HOD, Medical Gastroen-  terology, KMC, Manipal)    BT, CT, PT should be normal. Otherwise, vitamin K  Injection 10 mg is given IV or SC for 3 days.   If there is a bleeding tendency, this procedure should  not be done.   Biliary leak can be significant producing abdominal pain  and guarding. Hence, PTC should be done just prior to  the surgery.    Figs 25.55A and B: (A)  Diagrammatic representation  of percutaneous transhepatic  cholangiography (PTC);  (B) Dye had failed to fill-up  the bifurcation of the common  hepatic duct suggesting the  obstruction    1. In the diagnosis of high strictures, PTC is better than ERCP.  2. It can also be used in ERCP failure cases.  3. In the diagnosis of Klatskin’s tumour, PTC is extremely    useful. It can also delineate the dilated proximal duct, which  helps in planning for a biliary-enteric anastomosis.   4. Catheter can be kept in the bile ducts to provide external  drainage as in strictures or in inoperable malignancies with  obstructive jaundice (Fig. 25.55C).    ¢ It is the investigation of choice in cases of obstructive  jaundice or of high strictures and cholangiocarcinomas.    ¢ It is noninvasive and delineates the bile ducts very well  so that a biliary bypass can also be planned.   ¢ It can complement the staging by CT, MRI, etc. It  can improve prediction of resectability to about 98%  accuracy.   ¢ Specially excellentto detect peritoneal metastasis (which  cannot be picked by other tests) which 1s the sign of  inoperability.    15. CA 19-9: Often head mass can be due to carcinoma/    chronic pancreatitis. Biopsy 1s not mandatory. Clinical  suspicion of a head mass may be treated with Whipple’s  pancreaticoduodenectomy. However, gross elevation of  CA 19-9 (carbohydrate antigen), will suggest carcinoma.  Figure 25.58 shows investigations and treatment of  obstructive jaundice.    1. Correction of fluid and electrolyte status and adequate  hydration before surgery for 2—3 days 1s essential, especially  when patients have vomiting or have developed sepsis.   2. Injection dopamine 2 wg/kg/min can be given to improve  the urinary output (diuretic dose).   3. Injection vitamin K, 10 mg, subcutaneously or intra-  venously for 3 days 1s given to correct the prothrombin  time. If prothrombin time is not corrected with this treat-  ment, fresh frozen plasma should be given.   4. Broad spectrum antibiotics are given before, during and  after surgery.   - ¢ IHBR: Intrahepatic biliary radicles  CT scan for operability * HJ: Hepaticojejunostomy  — ¢ Ca: Carcinoma  ¢ ERCP: Endoscopic retrograde cholangio-  pancreatography  ¢ CT to look for liver metastasis, lymph nodes, superior  | mesentric artery, portal vein or hepatic artery infiltration  Whipple's and extension into hepatic parenchyma  pancreaticoduodenectomy ¢ PTBD: Percutaneous transhepatic biliary drainage    Fig. 25.58: Investigations and treatment of obstructive jaundice (Courtesy: Dr Ankur Sharma, Asst. Professor, Dept. of Surgery,  KMC, Manipal)    ¢ Cholecystectomy is done first. This is followed by  introduction of a cannula into the cystic duct and a radio-  Opaque dye Is injected.   ¢ This is called OTC (On Table Cholangiography). If the  dye goes freely into the duodenum without filling defect, it  is anormal OTC. If there are filling defects in the CBD, it  is explored.    INDICATIONS FOR EXPLORATION OF CBD es  . History of Charcot's triad  . Ultrasound proven stones in CBD  . CBD dilated more than 1 cm in size  . OTC shows filling defect  . Palpable stones in the CBD (open method)   the CBD. Depending upon the merit of the case and experience   of the surgeon any one of the methods can be selected.   |. Supraduodenal choledocholithotomy: Supraduodenal  CBD 1s explored through an incision over the anterior wall  and the stones are removed. Operating choledochoscope Is  passed into the common hepatic duct and its branches and  the stones, if present, are removed. Closure of the CBD 1s  done after inserting ‘T-tube’. After 8-10 days, a T-tube  cholangiography is done and if the dye goes freely into  duodenum and no filling defect is seen in the CBD, the T-  tube 1s removed by gentle traction. By 10—12 days, the  track is well formed. Hence, even if minor leak occurs,  bile flows outside without causing peritonitis (Key Box  25.15, Figs 25.59A and B).   Fig. 25.59B: T-tube cholangiogram showing residual (retained)  stone in the lower CBD—it was removed through endoscopic  papillotomy and basketing    2. Cholecystectomy + choledocholithotomy + choledochoduo-  denostomy: It can be done when CBD 1s dilated more than  1.5 cm in diameter and stoma should be at least 2—3 cm in  size (Key Box 25.16 and Fig. 25.60).   INDICATIONS  e Recurrent stones in the CBD  e Multiple intrahepatic stones (Caroli’s disease)  e Stricture of the lower CBD   ¢ There is no worry even if there are retained stones in the  CBD.   ¢ It is a permanent solution for stenosis, stricture or  multiple intrahepatic stones.   Fig. 25.61: Stone was removed through endoscopic papillotomy  followed by basketing. There is a small risk of perforation and  pancreatitis in these patients (Courtesy: Dr Ganesh Pai, Prof and  HOD of Medical Gastroenterology, KMC, Manipal)    Symptom % of patients  e Asymptomatic — 40%  e Cholangitis — 20%  e Jaundice — 20%  e Biliary colic — 15%  e Pancreatitis — 5%    Treatment of periampullary carcinoma  I. Surgical treatment (Figs 25.62 to 25.69)   ¢ In this operation, the growth along with ‘C’ loop of  duodenum up to DJ flexure, proximal jeyunum, head of  the pancreas up to the neck are removed and partial  gastrectomy is done. This is followed by:    Preoperative tissue diagnosis is NOT necessary with  appropriate clinical and imaging findings, specially in  head masses.    ¢ This is a major operation and carries 5—10% mortality  due to pancreatic leakage or biliary leakage.  ¢ Whipple’s operation is indicated in cases of mobile  growth with no metastasis and where the general  condition of the patients is reasonably good.  2. Pylorus-preserving pancreaticoduodenectomy (PPPD)  In this operation, pylorus is preserved. Thus, gastric motility  is not disturbed (Fig. 25.64).   Fig. 25.65B: Whipple’s specimen (Courtesy: Dr  Satyanarayana N, Associate Professor, Yenepoya  Medical College, Mangalore)    During resectional surgery for pancreatic head and uncinate  3 process tumours (Whipple’s pancreaticoduodenectomy), after   an | a preliminary search for metastases, a tunnel (the so-called  | > tunnel of love) needs to be developed behind the neck of the  if f 3 pancreas and anterior to the underlying visceral vessels before  concluding that the lesion can be resected.    3. Triple bypass: Cholecystojejunostomy + enteroentero-  stomy + gastrojejunostomy  e This isa palliative surgery in which distended gall bladder  is anastomosed to a long loop of jeyunum (40 cm) to  relieve jaundice. To prevent food particles entering into  the gall bladder, enteroenterostomy is done (Fig. 25.61).  ¢ Most of the patients develop duodenal obstruction caused  by the growth in the postoperative period. Hence, a  palliative GJ 1s done at the same time.  ¢ Inthe absence of duodenal obstruction, 1f surgeon thinks  that patient may live longer, beyond 6 months, GJ is indica-  ted (for a possible duodenal obstruction occurring later).    Very elderly patients (age criteria not clear) who are not fit  candidates for surgery and patients who have metastasis can  be treated by palliative stenting. However, results of a surgical  bypass 1S superior to stenting. Also, the stent needs to be  changed frequently.    ¢ 80% of strictures occur following surgery on the biliary  tree. They are called postoperative strictures. 20% are due  to inflammatory pathology.    ¢ Difficult cholecystectomy: When the gall bladder is  fibrosed, densely stuck to the right hepatic duct or to the  common bile duct or as in early cholecystectomy due to  oedema around Calot’s triangle, injury can occur to the right  hepatic duct or to the CBD or CHD resulting in stricture.   Type II: Middle common hepatic duct, stump < 2 cm  Type III: Hilar—confluence of right and left duct intact  Type IV: Right and left ducts separated   e Parasites like Ascaris lumbricoides in the biliary tree or  Asiatic cholangiohepatitis produced by Chinese liver fluke  infestation (Clonorchis sinensis) (page 591).    Difficult cholecystectomy, dangerous cholecystectomy  and faulty dissection are the important factors for  postoperative bile duct strictures.    ¢ Dangerous cholecystectomy: Sudden bleeding from  cystic artery can occur due to traction on the gall bladder  or due to lack of gentleness in ligating cystic artery.  Sudden application of an artery forceps to control the  bleeding may injure CBD. In such situations, packing  the area, good suction and visualisation of the bleeding  artery and ligation should be done. If the bleeding  continues, the hepatic artery can be compressed between  the finger and the thumb in the lesser omentum through Fig. 25.72: ERCP showing complete transection of bile duct—  the foramen of Winslow. This is called Hogarth-Pringle type IV injury   Dissection at fault: Ignorance of anomalies such as short  cystic duct or too much traction on the gall bladder  distorts CBD and predisposes to injury (Figs 25.72 and  Teles dE   It is the duty of the surgeon to show his assistants the Y  junction which is formed by the cystic duct, common  hepatic duct above and common bile duct below before  dividing any structures in this area.    ¢ History of cholecystectomy in the past with or without  profuse discharge of bile 1n the postoperative period.   ¢ ERCP or PTC may demonstrate a stricture in the CBD or  CHD with proximal dilatation.    ¢ If it is due to laparoscopic clipping without transection of  the CBD, it is better to re-explore and remove the clips and  a T-tube or an endoscopic stent can be placed in the CBD  (Fig. 25.74).   e Late cases can be managed by choledochojejunostomy or  hepaticojejunostomy by anastomosing a loop of jejunum | ee  to the dilated portion above the stricture. However, the Fig. 25.74: Same patient as in Fig. 25.73. You can see the stent  general condition of the patient should be improved before __ in the common hepatic duct. It was removed after 6 months  surgery (Figs 25.75 to 25.79).   Figs 25.78 and 25.79: PTBD catheter was passed from above  across Strictured hepaticojejunostomy and dilatations were done  in high strictures    Prevention ts better than treatment. Try to prevent bile  duct injuries during laparoscopic cholecystectomy.    It is characterised by development of multiple strictures and  dilatation of CBD with features of fibrous thickening of CBD.    ¢ Primary  No cause is found. However, it can be associated with  following conditions (Key Box 25.18).    Fig. 25.77: Hepaticojejunostomy was done in another patient with  [t is due to stones or injuries.    MRCP is a noninvasive investigation which can demon- . 7  strate multiple strictures and dilatation. Pig. Gatem: “Saickaaing, Col angh ly  ¢ ERCP is the investigation of choice which can demonstrate 7  the strictures in the CBD and dilatation which ts described  as having a beaded appearance. However, the risk of  suppurative cholangitis is present. ¢ It is a congenital cyst occurring in the CBD due to partia,  or complete weakness of the wall of the CBD.  ¢ Majority of cases manifest by |-2 years of age.    Stenting is the choice although stents may have to be replaced Pes want .  or changed if blockage occurs or if infection sets in. Classification (Fig. 25.81 and Key Box 25.19)    ¢ Age: Majority of cases manifest in children within 1-2 years  of age. It can also present in adults. Stricture    ¢ More common in females 4:1.  ¢ Abdominal distension can be due to a large cyst. The cyst Fig. 25.83: Choledochal cyst—MR cholangiogram showing gross    can be palpated per abdomen in the right hypochondrium. dilatation of the common bile duct and common hepatic duct  ¢ Slow progressive jaundice, recurrent attacks with abdominal   2. MRC: It will define the relation between lower end of the  bile duct and pancreatic duct to know basic anatomy  (Figs 25.82 and 25.83).   3. CT is also useful to know intrahepatic and extrahepatic  dilatation.   4. ERCP may be done but it will not give any more informa-  tion than MRC.   ¢ Type IV: They are difficult to treat. Due to recurrent  cholangitis, if total excision is not possible due to  adhesions between the cyst and portal vein, posterior  wall of the cyst can be left behind, after removal  of mucosa. This ts described as Lilly’s technique.  Type V will be described later.    well-recognised complication and it carries poor prognosis.  Hence, excision of the cyst and reconstruction is the  treatment of choice (Figs 25.84 to 25.87).   Fig. 25.85: Choledochal cyst at surgery. It was excised completely  and hepaticojejunostomy was done (Courtesy: Dr Ramesh Rajan,  Associate Professor, Surgical Gastroenterology, Trivandrum  Medical College, Kerala)   4. Carcinoma in the cyst (25-30% of cases). It 1s a  cholangiocarcinoma common in types I and V.    ¢ It is a hereditary condition wherein there is dilatation o  intrahepatic ducts with stenotic segments in between.   ¢ It is also an example of type V choledochal cyst.  Two types:  1. Simple: Presents later with abdominal pain and sepsis.  2. Periportal: It occurs in childhood, presents as recurren   ¢ Diagnosed by ultrasound and CT scan. MRI and ERCP are  other investigations (Fig. 25.88).   Diffuse inflammatory process of pancreas involving head,  body and the tail resulting in permanent structural and  functional damage to the pancreas.   Fig. 25.89: Left lobectomy with extraction of stone—Caroli’s disease  (Courtesy: Dr Nagaraj Palankar, Consultant, Manipal Hospital,  Bangalore)    1. Alcohol: High alcohol consumption is the most frequent  cause. Alcohol stimulates pancreatic secretion rich in  protein. This forms plugs in the pancreatic duct and results  in stasis of secretion and stone formation. Alcohol also  causes spasm of sphincter of Oddi.   2. Idiopathic: It is common in Kerala, and was thought to be  due to consumption of tapioca (Fig. 25.90). It 1s also called  Kerala pancreatitis or tropical pancreatitis. It is now  thought to be due to malnutrition. It is also called  fibrocalculous pancreatic diabetes.   3. Hereditary pancreatitis: It is a genetic disorder transmitted  as a Mendelian-dominant trait.   4. Cystic fibrosis: Generalised dysfunction of exocrine glands  cause secretions to precipitate in the lumen.   5. Hyperparathyroidism favours precipitation of calcium  intraductally. It can also activate pancreatic enzymes.   6. Autoimmune pancreatitis: Diffuse enlargement of  pancreas and narrowing of pancreatic duct is seen. Auto-  antibodies may be present. IgG4 1s increased.    There is destruction of pancreas by ductal sclerosis, ductal  strictures, glandular fibrosis and calcification, both intraductal  and parenchymal (Fig. 25.91 and Key Box 25.21).   CHRONIC PANCREATITIS  IDIOPATHIC TYPE  e Common in warm climates (Kerala)  e Common in young age  e High incidence of diabetes  e High incidence of stones in the duct  e Increased chances of parenchymal calcification  e Increased chances of pancreatic cancer    ¢ Malabsorption occurs due to damage to exocrine glands  resulting in steatorrhoea—10 to 15 stools per day, bulky,  frothy, rich in fat, foul-smelling. Malabsorption indicates  late disease and results in weight loss. Creatorrhoea refers  to excessive loss of protein (Figs 25.92 and 25.93).    ¢ Obstructive jaundice can occur due to oedema of the head  of pancreas. Later, fibrous constriction of CBD due to  fibrotic indurated mass in the head region can cause jaundice  by compressing the CBD.    ¢ Pain abdomen—upper abdominal pain radiating to the  back in the region of L, and L, due to retroperitoneal  inflammation (Key Box 25.22). Pain may be severe,  sometimes radiating to both right and left sides. The pain   PAIN IN CHRONIC PANCREATITIS  e Parenchymal hypertension  e Perineural inflammation  e Ductal hypertension  e Pseudocyst formation  e Stenosis of bile duct    Figs 25.92 and 25.93: This 21-year-old girl was weighing 32 kg  in 2006 when she underwent longitudinal pancreatico-  jejunostomy (LPJ) for failed medical treatment including stents.  After 3 years, she has put on 22 kg weight   Exploratory laparotomy—many cases are diagnosed a  laparotomy where irregularity and hardness involving the  entire pancreas are seen. Exploration is done for evaluatior  of obstructive jaundice or for chronic abdominal pain.  Diabetes—incidence of diabetes is about 10—20%. It shoulc  be suspected in diabetic patients with pain abdomen.    Obstructive jaundice due to a mass lesion in the head region  Carcinoma of pancreas    ¢ Painrelief by analgesics, epidural analgesia, or splanchnic  nerve block. Slow release opioid skin patches are useful.   ¢ Supplement pancreatic enzyme—diet should be low  in fat and vitamin D supplements should be given.   Fig. 25.97: CT shows dilated pancreatic duct of 10 mm in size  with stones. ERCP is not required in such patients. This patient  underwent LPJ    Fig. 25.96: CT scan showing chronic pancreatitis with head mass.  This case should be treated with head-coring operation    ¢ Pancreatic enzymes not only help in treating exocrine  insufficiency but they also denature CCK releasing  peptide, thereby diminishing the release of CCK. Thus,  pain 1s decreased. Six capsules of pancreatin are given.    1. Chronic pancreatitis involving tail of pancreas  ¢ Distal pancreatectomy with removal of spleen.  2. Diffuse chronic pancreatitis with dilated (large duct)  pancreatic duct  ¢ Duct is laid open widely, strictures are cut open, stones  are removed and it 1s anastomosed to a loop of jeyunum—  longitudinal pancreaticojejunostomy—Puestow’s  operation (Roux-en-Y jejunal segment). The duct should  be at least 8 mm in diameter. Sutures hold very nicely  because of fibrosis of pancreas. Pain relief is obtained in  about 80% of the cases. Pancreatic fistula is a  complication of this surgery. Majority of the fistulae close  spontaneously.  ¢ This is a bypass procedure which preserve endocrine and  exocrine functions. Fig. 25.99: Pancreaticojejunal anastomosis is being done   Fig. 25.104: Specimen of the portion of the head which was  excised—Frey’s procedure (Courtesy: Dr Girish MS, MCh, Surgical Fig. 25.105: Specimen of the portion of the head along with    Fig. 25.106: Head-coring gives rise to bleeding which can be  controlled with cautery (Key Box 25.23). Pain relief is the main  aim of surgery of chronic pancreatitis and anew carcinoma  developing. Both these problems are dealt with in this operation    3. Chronic pancreatitis with a head mass  In this situation, doubt arises whether it is malignancy or  not. Even trucut biopsy and frozen section are not foolproof.   e It is located in the crucial triangle (see Fig. 25.102) which  has to be excised.    Hence, pancreaticoduodenectomy is advised, provided  experience of the surgeon 1s good and the mortality rate is  less than 5%.    ¢ If malignancy 1s ruled out, a bypass procedure is the  treatment of choice.  — Choledochojejunostomy is the ideal treatment.  — Pancreaticoduodenal resection (Whipple) can also be  done (as mentioned above).    Here also, resection of the head mass or gastrojeyunostomy  is the treatment of choice.    Patients having following diseases have increased risk of  developing cholangiocarcinoma (Key Box 25.24).   Elderly males > 60 years are commonly affected.  Obstructive jaundice is the presenting feature.   Tender hepatomegaly is present due to congestion and may  be due to cholangitis.   Gall bladder is not palpable in cases of cholangio-  carcinoma unlike in periampullary carcinomas.  Cholangiocarcinoma at the bifurcation of the hepatic duct  is called Klatskin’s tumour.    ¢ Abdominal ultrasound, CT scan, ERCP will help in loca-  lising the site of obstruction (Figs 25.108 and 25.109).  ERCP and stenting the CBD ts indicated in inoperable  cases. However, MRI 1s better.    ¢ Treatment 1s difficult because most of the lesions are  high up in the hilar region infiltrating the liver, portal  vein, etc. Resection and hepaticojejunostomy or  endoscopic stent placement are the treatment modalities  available.    A disease of unknown aetiology, though rare, 1s fatal. Vira  aetiology and defective embryogenesis have been blamed fo  the development of biliary atresia.    Types (Fig. 25.110)  ¢ Type I: Common bile duct is involved  ¢ Type II: Common hepatic duct is involved    Clinical features  ¢ It presents as jaundice at birth or in the neonatal period.    ¢ Due to absence of bile in the gut, meconium ts not bile-  stained. Hence, stools are pale.    ¢ Enlargement of the spleen may follow if there is develop-  ment of portal hypertension.    * Surgical drainage of bile is the only available treatment  provided patent bile duct or radicle is seen. Anastomosis  of Roux-en-Y loop of jejunum to the dilated bile duct or  sometimes excision of bile duct tissue up to the liver capsule  should be done followed by Roux-en-Y anastomosis. This  1s called Kasai’s portoenterostomy.    Incidence: It 1s common in North Eastern India. Occurs in  elderly patients in their sixties/seventies.   80-90% of gall bladder cancers are associated with gall  stones. Calcification of gall bladder is associated with  carcinoma gall bladder.   High incidence of gall bladder and biliary cancer is noted  In people who work in rubber industries.   Adulterated mustard oil for cooking 1s found to precipitate  carcinoma gall bladder.    85% of cases. It is usually adenocarcinoma. Undifferen-  tiated carcinoma and squamous cell carcinoma may also  occur. The tumour is most commonly nodular, infiltrates  the entire gall bladder and adjacent tissues.   3 types of adenocarcinomas have been identified: nonpap1-  Ilary adenocarcinoma, papillary adenocarcinoma and  mucinous adenocarcinoma. Majority are nonpapillary  adenocarcinoma.    Significant weight loss, jaundice and mass in the right upper  quadrant are commonpresentations. Clinically, it is palpable  as a hard irregular mass.   A few cases can present as chronic cholecystitis with a mass  or acute cholecystitis. Ultrasonography may diagnose as  stones and they are subjected to cholecystectomy. During  surgery or postoperatively, the diagnosis gets confirmed.   Fig. 25.111: You can see extensive carcinomatous polypoid  lesions inside the gall bladder    Fig. 25.112: Advanced case of carcinoma gall bladder with  ascites and liver infiltration   Ultrasonography and endosonography are very useful  investigations. Ultrasonography-guided FNAC can be done  for histological diagnosis in suspected cases of gall bladder  mass.   CT scan is useful for staging—lymph nodes metastasis in  the liver (Fig. 25.112).   ERCP if there is obstructive jaundice to localise the exact  site and nature of obstruction.   Diagnostic laparoscopy. If peritoneal metastasis 1s present,  it is not worth resecting.   MRCP can be done. It visualises bile duct better than CT  scan.   If gall bladder cancer is found at cholecystectomy and if  mucosa alone is involved, then cholecystectomy 1s  sufficient.    Biologically very aggressive cancer (unlike basal cell cancer,  carcinoma colon, etc.)   Spreads by blood, neural, intraperitoneal routes  Intraductal extension into CBD causes obstructive jaundice  Infiltration into stomach, colon, duodenum and liver can  occur because of its location   M1 — distant spread ts present  Stage I: T] NO MO (up to muscle) Simple cholecystectomy  Stage II]: T2 NO MO (up to serosa) Extended cholecystectomy  Stage III: T3 NO — beyond serosa, liver < 2 cm, | adjacent  organ 1/2/3 N]—hepatoduodenal ligament Different procedures   ¢ Chemotherapy also has been tried. 5-FU, mitomycin C,  doxorubicin are the drugs used.   Prognosis  In general, five-year survival is very poor (Key Box 25.26).  Aggressive surgery and complete clearance give best result  (Fig. 25.113).   Thus, we have discussed various causes of obstructive  jaundice. Students should be able to differentiate between all    these different causes and to offer an appropriate diagnosis 1n  the examination. Each of these short topics can be asked Fig. 25.114: Ascariasis of the biliary duct. (Courtesy: Dr C.G.    as short notes in the theory paper. Understanding of all these Narasimhan, Surgeon, Mysore, Karnataka)    ¢ 70% of the cases occur in the head of the pancreas including  periampullary region.   ¢ Itis 4th leading cause of death due to cancer in males, after  lung, colon, prostate.    1. Chronic tropical pancreatitis and hereditary pancreatitis  are associated with pancreatic cancer—such malignancies  can be multifocal.   2. Haemochromatosis produces extensive calcification of  pancreas. It is also a precancerous condition.   3. Diabetes: Diabetic patients are 10 times more vulnerable  to develop carcinoma of pancreas.    ¢ Alcohol and smoking: It is related to tobacco specific  nitrosamines (including smokeless tobacco).   ¢ Westernisation of diet: Fatty food, rich in animal  proteins can cause pancreatic cancer.    ¢ Periampullary refers to carcinoma arising from ampulla of  Vater, the duodenal mucosa or the lower end of the common  bile duct.   ¢ Microscopically, the types are:  1. Mucus secreting carcinoma of ductal origin.  2. Nonmucus secreting carcinoma of acinar origin.  3. Anaplastic carcinomas are poorly differentiated and tend    4. Cystadenocarcinomas are rare, slow-growing and tend  to attain a large size.    e Periampullary and carcinoma head of pancreas present as  obstructive jaundice (discussed already). Discussion here  is On carcinoma body and tail of pancreas.   ¢ Microscopically, the tumour shows malignant cells with a  variable degree of differentiation, mitotic figures, etc. Not  only it invades vessels and lymphatics, but also perineural  spread occurs fast whichexplains backache (Key Box 25.27).   e Being retroperitoneal in location, manifests late, spreads  fast and carries poor prognosis.   e Severe pain radiating to the back in the region of LI] and  L2. It is due to infiltration of retroperitoneal nerve plexuses  or pancreatic duct obstruction.   ¢ Pain is so severe, often patient requests for narcotic  analgesics.   e¢ Jaundice cannot occur in carcinoma body and tail of  pancreas unless there are secondaries in liver or lymph  nodes at porta hepatis.    — Migrating thrombophlebitis of the legs can occur in  visceral malignancies particularly from carcinoma of  pancreas, rarely carcinoma stomach, colon, etc.    — It is supposedly due to sluggish blood flow resulting in  thrombus formation.    Sudden development of diabetes mellitus ts an early  manifestation in 25% of patients.    Signs  ¢ Anaemia may be present as in any other malignancy.    ¢ Jaundice is not a feature. Left supraclavicular node may  be palpable.    ¢ Per abdomen findings (Table 25.4): Majority of these cases  are advanced, fixed and are felt as a mass in the upper  abdomen.    e It 1s situated on the left side involving left hypochon-  drium, umbilical region and epigastrium.   Pain radiating to the back is the presenting feature  Haematemesis is not a feature   The mass does not move with respiration  Mobility is not a feature    1. Carcinoma stomach infiltrating the pancreas  ¢ Such mass may not be mobile. It does not move with  respiration because it is fixed to pancreas.  ¢ These patients will have vomiting first followed by  backache at a later date.  2. Carcinoma transverse colon  ¢ Produces constipation and bleeding per rectum. Vertica  mobility may be present.  ¢ Right to left peristalsis may be present.  3. Para-aortic lymph node mass may be due to:  ¢ Intra-abdominal malignancies, lymphoma, testicular  tumour, etc.    I.Investigations for periampullary carcinoma and  carcinoma of head of pancreas (discussed before).  I]. Investigations for carcinoma of body and tail  1. USG: First investigation of choice  ¢ Rules out calculous obstruction  ¢ Can detect a mass as small as 2 cm  ¢ Intraoperative ultrasound can be used to take a biopsy.  2. Contrast-enhanced CT scan (Figs 25.115 and 25.116)  ¢ Retroperitoneal invasion  e Lymph node enlargement  ¢ Invasion of hepatic artery, superior mesenteric artery,  ascites, liver metastasis—unresectable.  ¢ Infiltration of portal vein which makes it inoperable  ¢ In more than 70% of cases pancreatic duct is dilated and  in more than 60% of cases bile duct is dilated.  ¢ When both these ducts are dilated, it is most likely  pancreatic cancer.  ¢ CT-guided aspiration biopsy is indicated for lesions to  start neoadjuvant chemotherapy/RT (infiltrative lesions).    Vomiting, dyspepsia Is present. Pain is not a feature  Haematemesis is a common feature   Fig. 25.116: Large mass in the head and body of pancreas    3. ERCP is indicated in the absence of a mass. Findings can    which are elaborated by malignancies. /t is a tumour  marker of pancreas to monitor carcinoma pancreas.  Increased in 75% of patients with carcinoma pancreas and  10% of the patients in benign disease of pancreas, liver  and bile ducts. Hence, it 1s not a diagnostic investigation.  However, very high values suggest malignancy even when  histology is inconclusive.    .If the tumour is very small and diagnosed very early, they    are ideally treated with total pancreatectomy with  removal of involved lymph nodes.    due to fixity to portal vein or due to metastasis. Hence,  there 1s no role for curative surgery. For the confirmation  of diagnosis, transabdominal USG-guided fine needle  aspiration cytology can be done. Surgery 1s not indicated  in such cases; only palliative treatment can be offered.  Prognosis is very poor.  ¢ Palliative radiotherapy: 4000-6000 cGy units can be  given. Response rate is 5—10%. It reduces size of the  tumour and some pain relief is obtained (Table 25.5).  ¢ Palliative treatment is an important aspect of carcinoma  pancreas even though such patients succumb to the disease  within 3 to 6 months. Palliation 1s mainly to relieve pain.    ¢ Coeliac plexus nerve block (pain is due to invasion  of retroperitoneal nerve trunks)    ¢ Choledochojejunostomy, cholecystojejunostomy  (if cystic duct enters CBD well above the   These tumours can be confused with pseudocyst of the  pancreas. Hence, they should be differentiated at surgery  (Figs 25.117 to 25.120). Otherwise, gross blunders can take  place (Table 25.6).    account for 30% of all CPNs. They can be observed if  asymptomatic.   Fig. 25.118: Ultrasound showing cystic mass in the body of  pancreas    These tumours are members of APUDOMA arising from  APUD (Amine Precursor Uptake Decarboxylation) cells. They  are neuroectodermal in origin. Accordingly, following tumours  can occur (Table 25.8).   ¢ Familial: Occur with other adenomas as in multiple  endocrine neoplasia syndrome—MEN TYPE I: Pituitary,  parathyroid, pancreatic adenoma.   Majority of the insulinomas occur in the tail and body of  pancreas and majority of them are benign, one-third are  malignant and one-third are multiple.   It can be a single adenoma, can be due to diffuse hyperplasia  or due to carcinoma.   Clinical features are that of hypoglycaemia  (Key Box 25.29 for other causes of hypoglycaemia).   In early stages, 1t can mimic a duodenal ulcer. Due to  hypoglycaemia, hunger pain develops and a tendency to  ask for food 1s present.   As it becomes severe, giddiness, dizziness, syncopal attacks,  blurring of vision and diplopia can occur.    PERSISTENT HYPOGLYCAEMIA—CAUSES sg  Insulinoma  Hepatoma  Hepatocellular damage  Hypopituitarism  Addison's disease  Large mesenchymal tumour    1. Attack of hypoglycaemia in morning hours, in the fasting  state.   3. Blood sugar in the fasting state is less than 45 mg% during  the attack.    The author remembers a case of recurrent epilepsy getting  admitted to the department of neurology. He was found to have  hypoglycaemia and was diagnosed as insulinoma only during  the 4th admission. A 3 cm benign insulinoma was enucleated  from the pancreas. The patient is asymptomatic till date.    1. Serum insulin levels done by immunoassay method are  found to be very high. Proinsulin levels and C-peptide level  are also very high.   2. Persistent hypoglycaemia—blood sugar level of less  than 50 mg/d! relieved by glucose is suggestive of  insulinoma.   3. USG and CT scan of abdomen can demonstrate the tumour  if itis more than 2 cm (intraoperative ultrasound ts a useful  test).   4. Selective angiography will demonstrate tumour blush as  majority of them are very vascular.   ¢ Resection of the tumour and if necessary distal pancrea-  tectomy can also be done in selected patients.   ¢ For inoperable islet cell carcinoma, streptozocin 1s the best  chemotherapeutic agents.   e Mostly present as bizarre behaviour confusing type due to  hypoglycaemia.    e The most important test to localise the tumour before surgery  is CT/MRI.   e The most important test to localise at surgery is intra-  operative ultrasound.   e Mostly they can be enucleated when occur in the head.    Two types are recognised  ¢ Type! (rare): These have antral G cell hyperplasia, where  gastrin is stored. Hypergastrinaemia with chronic pepti  ulceration 1s a feature. Pancreas is normal.  ¢ Type II: May be due to an ulcerogenic, non £-islet cel  tumour or sometimes diffuse hyperplasia of the islet cells  Tumour secretes gastrin and 1s usually found tn the tail o  the pancreas. 50% of them are malignant.  ¢ Intractable peptic ulceration, hypergastrinaemia wit  massive acid hypersecretion of up to 500 ml/hr can occul  e Diarrhoea, steatorrhoea and hypokalaemia occur due ti  acid irritating the small bowel activity (Key Box 25.30)   KEY BOX 25.30 _—  GASTRINOMA SHOULD BE SUSPECTED  IN FOLLOWING SITUATIONS   1. Unusual ulcer: Peptic ulcer not responding to intensive  medical treatment.   4. Unusual sites: Ulcers present in the 2nd part of duodenum,  an ulcer just distal to the ligament of Treitz.    5. Unusual age: Sudden development of an ulcer in a young  boy or in a very elderly patient.    Site  It commonly occurs in the gastrinoma triangle also called  Passaro’s triangle. Following three points form Passaro’s triangle.    1. Junction of the cystic duct with the common bile duct  (Fig. 25.121).   Fig. 25.121: Passaro’s triangle of gastrinoma  (Courtesy: Dr. Vidushi, Undergraduate student, KMC, Manipal)   1. Endoscopy will show prominent mucosal folds and large  amount of acid in the stomach.   2. Serum gastrin is increased above normal levels (normal  value < 150 ng/dl).   1. Type I: Partial gastrectomy to remove G cells bearing area.   2. Type II: If the tumour is small, removal of the tumour can  be done (enucleation).   3. If the tumour is large, omeprazole 20 mg twice a day or  somatostatin derivatives have been used to control acidity.  Total gastrectomy is the last resort, if gastrinoma is not  found.    It arises from alpha cells producing glucagon. Around 90%  are in the body and tail.   Clinically, it presents with extensive necrolytic migratory  erythematous rashes. Rashes occur due to low amino acid  levels due to neoglucogenesis brought about by glucagon.  Rashes involve legs and perineum.   Mild diabetes, weight loss, diarrhoea are the other features.  Glucagon levels are increased in serum diagnostic.  Localisation is by elective arteriography and CT scan  Treatment is enucleation    they occur, they can present with varying clinical manifesta-  tions which confuse a clinician. A high index of suspicion 1s  necessary. They may be benign but can be fatal if left untreated.    Definition: It is defined as acute nonbacterial inflammatory  condition caused by activation, interstitial liberation and auto-  digestion of pancreas presenting as acute abdominal pain.   Acute pancreatitis drinks like a fish (produces dehydration).  Acute pancreatitis eats like a wolf (pancreatic necrosis).  Acute pancreatitis burrows like a rodent (produces fistula).  Acute pancreatitis kills like a leopard (life-threatening).   ¢ In both these conditions there is always permanent  damage to the pancreas.   Alcohol abuse (40 to 50%): It 1s the major cause of  acute pancreatitis in our country and is seen in about  50% of the cases. Alcohol stimulates pancreatic secretions  rich in protein, forms protein plugs and results in  obstruction to the pancreatic duct. Alcohol stimulates  trypsinogen. It causes spasm of sphincter of Oddi. It also  has direct toxic effect on the pancreas. Tobacco smoking  contributes to its effects.   Biliary tract disease: Stone in the biliary tree (gall stone  pancreatitis) is the major cause of acute pancreatitis in  the Western world (40%). In our country, it may be  responsible for pancreatitis in about 20-30% of patients.    Collagen vascular disorders: Autoimmune disease such  as polyarteritis nodosa can be a causative factor in acute  pancreatitis.    Drugs: Corticosteroids, tetracycline, oestrogens, azathio-  prine, valproic acid and diuretics can cause pancreatitis.    Endoscopic procedures: Sphincterotomy, cannulation of  CBD or pancreatic duct, or basketing of stones from CBD  can precipitate acute pancreatitis by duct disruption and  enzyme extravasation.    Hyperparathyroidism causing hypercalcaemia may  stimulate pancreatic juices and can cause pancreatitis. I  also facilitates precipitation of calcium in the ducts.    Hyperlipidaemia (> 1000 mg/dl of triglycerides) can alsc  cause pancreatitis. Dietary control can cure pancreatitis.    Hypothermia and hypotension can cause ischaemia to  the pancreas resulting in acute pancreatitis.    . Ductal: Around 20%. Some of them are due to pancreatic    divisum seen during ERCP. The opening of the minor  papilla is inadequate for drainage of pancreatic juice.    It follows operations on CBD-open or laparoscopic. More  common after T-tube insertions (now it is less).    Cardiac surgery. Here risk factors are perioperative  administration of calcium, postoperative hypotension, pre-  operative renal failure.    14. Idiopathic: It is seen in about 15% of cases. Even though,  classified as idiopathic (no cause 1s found), they are found  to have sludge/gall stones undetected by ultrasound  examination (Key Box 25.31).    QUICK REFERENCE TO COMMON  CAUSES OF PANCREATITIS  ¢ Pancreatitis in teens—suspect hereditary or APBDJ'  e Pancreatitis in women—suspect gall stones  e Pancreatitis in males—alcoholic pancreatitis  e Pancreatitis with fleeting joint pains—autoimmune  pancreatitis.  e Pancreatitis with bony lesions/cysts—hyperparathyroidism  e Pancreatitis with fever—viral    ¢ Autodigestion is the final common pathway leading to  pancreatitis, a few theories have been explained as possible  factors for autogenesis.   1. Obstruction/secretion: Recurrent attacks of pancreatitis  as in chronic cases can be due to multiple strictures, stasis,  resulting in autodigestion.   2. Common channel theory: In about 90% of cases, bile duct  and pancreatic duct converge into ampulla of Vater.  However, only in 10% of cases they have common channel.    duodenum through action of enterokinase. Hence, it 1s  postulated that duodenal reflux is one of the causes of acute  pancreatitis.    Acute pancreatitis 1s an autodigestion following activation  of trypsinogen. This 1s brought about by various agents  mentioned above.   It may also be due to reflux of bile into the pancreas.    . Lipase: Lipase splits the fat into fatty acids and glycerol.    Fatty acids combine with calcium to form calcium soap.  This 1s represented as fat necrosis seen in the omentum,  subsynovial pockets of knee joint, etc. This also explains  hypocalcaemia and tetany seen in acute pancreatitis.    . Elastase: It digests the elastic fibres of the blood vessels    the inflammatory mediators. They produce profound  hypotension, shock and collapse, due to loss of fluid in the  retroperitoneum (III space loss).    (myocardial depressant factor) which depresses ventricular  contraction resulting in cardiac failure. Ultimate result is  the development of multi-organ failure.   Fig. 25.127: Abdominal distension is due to paralytic ileus in the  early stages. It can also be due to pancreatic ascites    Severe upper abdominal epigastric pain radiating to the back  increases over a period of hours—uillimitable agony is a  characteristic feature. It is partially relieved on stooping  and bending forwards (Mohammedan Prayer sign). A meal  or alcohol triggers the pain.   Haematemesis and melaena can occur due to necrosis of  duodenum. It is a poor prognostic sign.   Faint jaundice due to oedema of the head of the pancreas.  Features of shock—feeble pulse, tachycardia, hypotension,  cold extremities.    — Abdominal distension due to either accumulation of  blood or fluid in the peritoneal cavity or due to paralytic  ileus.   — Bluish ecchymotic discolouration seen around umbilicus  (first described for ruptured ectopic pregnancy).   ¢ Both these signs are due to peri-pancreatic and  retroperitoneal haemorrhage and seepage of blood along  fascial planes, into the anterior abdominal wall and spread  through falciform ligament.    Investigations  1. Haemogram (CBP): Hb% may be low due to haemorrhagic    2. Blood for urea, creatinine to rule out renal failure  3. Serum amylase (widely used test) (Key Box 25.32).    ¢ Normal levels are 40-80 Somogyi units  ¢ Values around 400 are suggestive and values more than  1000 Somogyi units are diagnostic of acute pancreatitis.  ¢ [t is increased in the first 24-48 hours and returns to  normal within 3—4 days (Key Box 25.32).  ¢ Persistent high level of amylase in acute pancreatitis  indicates:  — Unresolving inflammation  — Recurrent attacks of pancreatitis  — Complications—pseudocyst, pancreatic abscess  — Serum lipase levels—more specific but difficult to  measure. Lipase is only secreted by pancreas.    INCREASED AMYLASE LEVELS ARE SEEN _  Acute pancreatitis and its complications  Parotitis  Afferent loop obstruction  Spasm of sphincter of Oddi  Biliary peritonitis—duodenal injuries  Mesenteric infarction  Ruptured ectopic gestation    Lipase levels tend to be higher in alcoholic pancreatitis  and amylase levels are higher in gall stone pancreatitis.    . Total proteins are usually low, especially albumin.  . Plain X-ray abdomen (erect position) (Fig. 25.130)    3—5 days in patients who fail to respond to conservative  treatment. If CT scan demonstrates infected necrosis, an  urgent CI-guided FNAC is done and Gram stain is sent.  If Gram stain is positive, it has to be treated urgently  (Key Box 25.33, Figs 25.131 and 25.132).    Early aggressive fluid replacement is the key in the  management of acute pancreatitis.   Fig. 25.131: Contrast enhanced CT (CECT) scan is the gold Key Box 25.34.   standard investigation in cases of acute pancreatitis. Useful after  ° Those of you who find it difficult can remember at least  7 days of acute pancreatitis. This photograph shows extensive few fundamentals of managing acute pancreatitis. They are  necrosis. CT scan also helps in detecting pseudocysts, abscesses, given in the form of simple headlines: ABCDEF    presence of gall stones or any other unsuspected pathology in a: Aspiration with Ryle’s tube, to give rest to the pancreas  the abdomen. Modern thinking is to wait for 4—6 weeks for (may be for one or two days)   WBC count > 16 x 10°/L WBC count > 15 x 10?/L  Blood glucose > 10 mmol/L Blood glucose > 10 mmol/L  LDH > 700 units/L Serum urea > 16 mmol/L  AST > 250 Sigma Frankel unit/unit PaO, < 60 mm Hg  WITHIN 48 HOURS   Increase in blood urea nitrogen levels > 5 mg% Serum calcium < 2 mmol/L  Drop in haematocrit > 10% Serum albumin < 32 g/L  Arterial oxygen saturation (PaO, < 60 mmHg) LDH > 600 units/L    Admission Acute ward with monitoring of vitals Intensive care unit Intensive care unit  IV fluids/ Early correction of hypotension, IV fluids and inotropic* support may May require inotropes and  hypotension hypovolaemia—crystalloids be required vasopressors for a long period    Antibiotics No antibiotics Early antibiotic prophylaxis is required Broad spectrum antibiotics—imipenem,  ciprofloxacin, metronidazole, etc.   Oral/nutrition Oral fluids, soft diet by 34 days, once If pain is still present even after 4 days, Enteral feeding/nasojejunal feeding.   pain and ileus settle down nasojejunal feeding to be done If calories are not sufficient, total    By 10—15 days, may resolve completely Ideal time to operate is after 4 weeks  or may develop into pseudocyst or when necrosis is demarcated well  infected necrosis which require surgery    Majority of patients will not require  surgery. If gall stones are impacted  at sphincter of Oddi, endoscopic  basketing and laparoscopic  cholecystectomy should be done    * Dopamine 2-5 ug/kg/min can help in renal perfusion. 5—10 ug/kg/min acts as an inotrope and 10-20 ug/kg/min as a vasopressor.    D: Drugs: Prophylactic antibiotics—used in cases of severe  pancreatitis for prevention of local or general complication.  IV cefuroxime or imipenem or ciprofloxacin with  metronidazole are given. Low molecular weight dextran  (lomodex) 500 ml can be used to increase renal perfusion.  Alternately, dopamine 2 ug/kg/min can be given IV which  helps in renal perfusion (in case of oliguria).    by insertion of tube drain. Lavage has shown some benefit.  In cases of infected necrosis, necrosectomy is done.    e The wound can be left open as laparotomy or with mesh  or with zip.  F: Fluid should be given early. Rapid infusion of 3-4 litres of  Ringer lactate is used to treat hypovolaemic shock. Plasma  or albumin may also be given.   e Pancreatic abscess IDENTIFICATION OF SEVERE PANCREATITIS  e Diagnosis is in doubt—perforated viscus cannot be ruled out. ¢ Age ; > 70 years  e Complications such as massive bleeding not responding to ° Obesity - BMI > 30 kg/m?    E: Exploratory laparotomy, only when diagnosisisindoubt, ,  when patient is not improving or when there is a  complication of pancreatitis such as pancreatic abscess,  fistula or necrosis. With the advent of wonderful imaging  techniques, surgical intervention Is rarely being done in the  initial phase of acute pancreatitis. In early cases, pancreas  should not be handled. Peritoneal lavage is done followed    In cases of infected necrosis proved by CT-guided FNAC  and Gram stain, necrosectomy should be done.    ¢ Ideally done 4-6 weeks later when sepsis is still present.   ¢ All fluid and tissue should be sent for aerobic and anaerobic  culture.   ¢ Blunt dissection rather than sharp dissection is done to  minimise bleeding.   Fig. 25.133: Acute necrotising pancreatitis. Patient had high  temperature. Nasojejunal tube feeding is being done    Fig. 25.135: Acute necrotising pancreatitis with massive upper  GI bleeding. Patient had haematemesis of one litre of blood with  clots   ¢ Perfect haemostasis should be achieved using manua  compression, sutures and ligatures.   ¢ Today all these cases are done through minimally invasive  methods.   Fig. 25.134: Acute necrotising pancreatitis with massive upper  GI bleeding. Patient had high temperature, angiogram revealed  pseudoaneurysm. He underwent therapeutic embolisation  followed by necrosectomy   Fig. 25.136: You can see necrosectomy being done. It was done  through lesser sac. Significant blood loss was present. Fingers  are used to separate necrosis and it is removed with a spoon   Fig. 25.139: After necrosectomy, it is better to perform zip  laparotomy so that it can be opened any number of times for  peritoneal toilet (Courtesy: Dr BH Ananda Rao, Prof and Head,  Department of Surgery, Dr Siddhartha Bhandary and  Dr Prashanth Shetty, Associate Professors, KMC, Manipal)    saline and tubes or sump drains are put so as to be able to irrigate If gall stone is obstructing the ampulla of Vater and causing  in the postoperative period pancreatitis, endoscopic sphincterotomy may be done within  days. It has shown some benefit to the patients. After 2-3  weeks, once the jaundice subsides, cholecystectomy is  recommended.   ACUTE PANCREATITIS es  e Lipase is a more reliable test because it is more pancreas-  specific and remains elevated for long after onset of symptom.  e However, amylase is widely used  e It is not necessary to demonstrate CBD stone to prove the  diagnosis of gall stone pancreatitis. Often stone has passed  into duodenum.    amount of fluid in the third space—peritoneal cavity,  pleural cavity and extravascular space, shock occurs. Fluid  replacement with blood or albumin should be done at  appropriate time to treat the shock.    Laparoscopy may be necessary not only for the diagnos!  but also as a therapeutic means for removal of necroti  pancreas.    ¢ Electrolyte abnormalities should be corrected.  2. Respiratory insufficiency: Factors responsible for this are  given In Key Box 25.37.    : Ae |  ¢ It 1s seen in about 20% of the patients.  RESPIRATORY FAILURE 3   e Lecithin present in the pulmonary surfactant is altered due  to lecithinase resulting in defective capillary alveolar  exchange. °    This complication is encountered after 2nd week following  an attack of acute pancreatitis.    4. Pseudoaneurysm resulting in massive upper gastro-  intestinal or lower gastrointestinal bleeding. Bleeding intc  the pancreatic duct is called haemosuccus pancreaticus.    This condition occurs due to enzymatic digestion of the  blood vessels in the vicinity of pancreas. Thus splenic artery.  gastroduodenal artery, etc. are commonly involved.    ¢ Jt has very high mortality. Prompt angiography followed  Measurement of arterial blood gas values and administration by embolisation is the treatment.  of oxygen 1s enough in the initial stages. In late stages,  pulmonary insufficiency needs to be treated with ventilatory    Otherwise, laparotomy, ligation of pseudoaneurysm with  or without intracystic ligation of the bleeders 1s the treatment  (Figs 25.141 to 25.143).    3. Hypocalcaemia needs to be treated with calcium IV. It is  due to hypoalbuminaemia and due to calcium soap.   4. Pleural effusion is treated by pleural tap (ultrasound-  guided), if it 1s symptomatic.    ¢ It develops after 3—4 weeks of pancreatitis. Secondary  infection in a pseudocyst results in pancreatic abscess. It  usually points out on the left flank.   Fig. 25.143: As a cause of massive upper gastrointestinal  bleeding (Courtesy: Dr Annappa Kudwa, Professor of Surgery,  KMC, Manipal)    Definition  Collection of amylase-rich fluid in the lesser sac, due to  pancreatic pathology. Fluid collection in the first 4 weeks is  an acute fluid collection. After 4 weeks, it becomes an acute  pseudocyst.    ¢ Fluid is enclosed by a wall of fibrous granulation tissue  and is called a pseudocyst.    1. Following an attack of acute pancreatitis, it usually appears  after 4 weeks, as upper abdominal swelling.    2. Blunt injury of abdomen causing a ductal disruption  wherein the pancreatic duct in the region of body 1s crushed  against vertebral body results in a pseudocyst.    3. Some cause of chronic pancreatitis may be associated with  pseudocyst.    Locations of pseudocyst  1. Between stomach and transverse colon  2. Between stomach and liver    Type I. Acute postnecrotic pseudocyst that occurs after an  episode of acute pancreatitis and 1s associated with normal  duct anatomy and rarely communicate with pancreatic duct.   Type II. Post necrotic pseudocyst that occur after an episodic  of acute on chronic pancreatitis and have a diseased but not  strictured pancreatic duct and there is after—communication  between the duct and pseudocyst.   Type III (also called retention cyst) occur in chronic  pancreatitis, uniformly associated with a duct stricture and a  communication between duct and pseudocyst.    1. Tensely cystic mass in the epigastrium, umbilical region or  in left hypochondrium. Tensely cystic mass feels firm on  palpation. Classically upper border of the mass is not felt.    2. Does not move with respiration because it is retroperitoneal  in location.    3. It may have transverse mobility  4. It does not fall forwards    5. Percussion: It gives a resonant note because of stomach  or intestine anterior to it.    8. Depending upon the tension within the cyst, it can be tender  or nontender.    1. USG/CT: It can detect size, location of cyst and wall  thickness. Confirm that there is no neoplasm of the pancreas  before doing cystogastrostomy (Fig. 25.144).   3. ERCP may demonstrate communication of the cyst with  the duct (routinely not done).   ¢ Majority of the pseudocysts following acute pancreatitis  resolve spontaneously within 3—4 weeks.   ¢ Hence, regular ultrasound examination is done to observe  the pseudocyst.    Increase in size of cyst, severe pain, no response to conser-   ¢ Timing: Surgery is done after 6 weeks because that 1s the  time required for the wall to become fibrous.    Fig. 25.146: Posterior gastric wall is sutured to anterior cyst wal  after creating a stoma of 6 cm    Figs 25.149A to D: Steps of cystogastrostomy (Courtesy: Miss Vidushi, MBBS student, KMC, Manipal)    ¢ Size of the cyst should be at least 6 cm. cystogastrostomy stoma is about 6 cm. This procedure  ¢ Procedure: Anterior gastrostomy is done and an incision can also be done by endoscopic method.  in the posterior wall of stomach opens into the cyst cavity. 2. Distal pancreatectomy: Cyst confined to the tail of the  The contents are drained, opening 1s enlarged and cut end pancreas Is treated with removal of the tail and the cyst.  of stomach in posterior wall is sutured to cut edge of cyst 3. Cystojejunostomy: By using Roux-en-Y loop can be done,  wall. After one week, the cyst collapses. For reasons not for large cysts by suturing jejunal loop to the cyst in the  known, the food does not enter the cyst cavity. Size of most dependent area (Figs 25.150 to 25.152).   Fig. 25.150: CT scan showing large pseudocyst with multiple Fig. 25.151: Roux-en-Y cystojejunostomy at surgery, inset shows  pockets. Cystojejunostomy is the ideal treatment the opened cyst wall which is thick   Fig. 25.153: Endoscopic cystogastrostomy—ideal to drain acute  pseudocysts. Inset—after draining a nasobiliary catheter can be  left in the cyst cavity (Courtesy: Dr Filipe Alvares, Medical Gastro-  enterologist, KMC, Manipal)    This 1s easy, less invasive and ideal in draining acute  pseudocysts. This procedure can also be repeated. However,  chances of introducing infection are present. Endoscopic  method of draining the pseudocysts ts also being done for  chronic cysts (Fig. 25.153).    e Pancreas 1s formed by fusion of ventral and dorsal buds.  This will occur by 6 weeks. Malrotation of ventral bud  causes annular pancreas.   It is arare anomaly which occurs due to persistence of a portion  of the ventral pancreatic anlage which fails to rotate. As a  result of this, second part of the duodenum Is surrounded by a  thin rim of pancreatic tissue. Hence the name, annular pancreas  (Key Box 25.38).    ¢ Neonatal type: It manifests early in life. It produces  symptoms of acute intestinal obstruction with vomiting and  inability to take food.   ¢ Adult type: It manifests after the age of 20. Vomiting is  bile-stained. Due to stasis in the pyloric antrum, features  of duodenal ulcer may be present.    e Plain X-ray abdomen: Double bubble appearance occurs  due to dilated stomach and dilated proximal duodenum.   ¢ Barium meal: X-ray can demonstrate obstruction to second  part of duodenum.    ¢ Duodenoduodenostomy is the treatment of choice. Other-  wise, duodenojejunostomy can be done.    [t is tempting to divide the thin pancreatic rim. This  Should not be done as it willresultin a pancreatic fistula.   ¢ Occasionally, at laparotomy for unrelated conditions, a  soft to firm irregular tissue or nodule, is found on the  intestinal surface.   e Itmay be found in the submucosa of the stomach, duodenum,  small intestines, Meckel’s diverticulum or in the hilum of  the spleen.   2. In the intestine: It can cause intussusception. Sometimes,  it may be the source of gastrointestinal bleeding.    The viscid mucin which is produced results in obstruction of  the ducts and ductules. Stasis of pancreatic secretions, alveolar  rupture due to increased pressure take place later. As a result  of alveolar rupture, pancreatic enzymes leak outside resulting  In pancreatitis.    |. At birth: Meconium ileus or meconium peritonitis 1s an  important manifestation of cystic fibrosis of pancreas.  2. In infants  ¢ Recurrent respiratory tract infection in the form of  bronchiolitis and bronchiectasis results in cough with  expectoration and dyspnoea.  ¢ Emaciation, steatorrhoea. Stools are pale and sticky.  3. Older children  Steatorrhoea, gross emaciation and wasting are the features.  Due to poor nutritional status, cirrhosis of the liver with  portal hypertension can be a feature.  4. In adult  Lucky survivors will suffer from gross wasting, diabetes,  bronchiectasis, cirrhosis of the liver, sialoadenitis and  choroiditis.    ¢ Normal contents of the electrolytes in the sweat: Na’ —70  mmol/L, C/'—60 mmol/L, K'— 20 mmol/L.    e In these patients, sodium excretion may be three to four  times more than normal.    Treatment  Only symptomatic  I. Nutritional support  ¢ Fat intake should be low, protein should be increased.  ¢ Pancreatic enzyme preparations (5 to 10 g) are given  two to three times per day, to supplement pancreatic  enzymes.  II. Control infection  Respiratory tract infection is treated with antibiotics,  bronchodilators, mucolytic agents, etc.  III. Role of surgery  Indicated in meconium ileus to relieve intestinal  obstruction.    e Pancreatic divisum is the most common congenital abnor-  mality of the pancreas. It is found in 5—10% of patients.   e It 1s caused by failure of fusion of the dorsal and ventral  portions of the developing pancreas. In the majority of  patients, this anomaly is of no clinical importance.   ¢ In a certain subset of patients, however, estimated to be  approximately 5—20%, pancreatic divisum 1s a clinically  important cause of abdominal pain, acute recurrent  pancreatitis or chronic pancreatitis.   ¢ The frequency of pancreatic divisum in patients with  pancreatitis in recent studies is approximately 10%  (Key Box 25.40).    e It is one of the uncommon causes of acute pancreatitis.   e In 10% of patients. Majority of pancreas drains through duct  of Santorini through lesser papilla.    The cause of pancreatitis in this common anomaly is hypo-  thesised to be caused by minor papillary insufficiency caused  by papillary fibrosis and subsequent stenosis.   ¢ MRCP 1s anoninvasive and accurate method of establishing  the diagnosis. However, anegative MRCP does not exclude  the diagnosis of divisum as the concordance with ERCP  ranges from 50% to more than 70%.   ¢ The accuracy of the MRCP may be improved by  administration of secretin.    ¢ In patients with minimal symptoms, treatment with  pancreatic enzymes may reduce pancreatic secretion and  hence, pain. Pain management referral may be necessary.   ¢ Patients with acute pancreatitis are estimated to benefit from  either surgical minor sphincteroplasty or endoscopic minor  papillotomy.    A few anomalies of pancreatic duct are given below  (Figs 25.155A to E)    |. External fistulae occur due to operative injury to the  pancreas or due to a pancreatic anastomotic leak.  ¢ Injury to the tail of pancreas during splenectomy or  adrenalectomy.   e Injury to the head and body during radical gastrec-  tomy.   ¢ Pancreaticojeyunostomy for chronic pancreatitis or  following Whipple’s operation can also give rise to fistula  in the postoperative period.   2. Internal fistulae: It can occur following a blunt injury  abdomen wherein the neck of the pancreas is crushed  against lumbar spine resulting in injury. Internal fistulae  can communicate with pleural space resulting in a  pancreaticopleural fistula.    e Inmany cases, the patients present with a discharge of straw-  coloured fluid from the drain site in the postoperative  period.   ¢ Internal fistula can manifest in a totally unexpected manner,  sometimes, as a case of pleural effusion.    ¢ Amylase levels in the pleural fluid, peritoneal fluid and in  the discharge will be high.    ¢ Abdominal ultrasound 1s done to rule out a pseudocyst of  the pancreas.   ¢ ERCP: It can demonstrate leakage of the dye from the  pancreatic duct into the surrounding area or along the  fistulous tract and proximal obstruction, if any.   ¢ CT fistulogram can define the exact site of communication  to pancreas. It can define the length of the fistula also.   ¢ CT angiogram is indicated in cases of bleeding associated  with pancreatic fistula.    ¢ In majority of the cases following surgery, the fistulous  discharge stops within one to three weeks of time. During  this period, the skin is protected by application of zinc oxide  cream. Electrolytes have to be checked frequently.   ¢ Injection octreotide in the dose of 50-100 mg, 8th hourly  will help in decreasing fistula output by more than  80-90%.    ¢ If the fistula persists in spite of conservative treatment,  fistulectomy with removal of involved part of the pancreas  and body or tail has to be done.   ¢ In very difficult cases (plastered abdomen) or in fistulas on  the anterior abdominal side, fistulogastrostomy can also  be done.   2. Massive bleeding: It occurs due to digestion of elastin  fibres of blood vessels resulting in pseudoaneurysms.  Morbidity is high.   3. Ramification which means branching pattern of the fistula  renders the surgery difficult.   A patient was admitted to the hospital with acute breathless-  ness. He was found to have left-sided pleural effusion  (massive). He had blunt injury abdomen 2 months back  during which time laparotomy and closure of a proximal  jejunal perforation had been done. There was no obvious  sign of pancreatic injury. Aspiration of pleural fluid revealed  high amylase. It was a case of pancreaticopleural fistula.   ¢ In long-standing cases of obstruction to the CBD, the bile  in the CBD gets absorbed and 1s replaced by mucus secreted  from the CBD.    ¢ It indicates a long-standing obstruction.  ¢ It has to be relieved as an urgent procedure.  ¢ White bile is seen in:   ¢ It is a protein-rich, noninfected fluid with protein levels  greater than 25 g/L.    ¢ Disruption of ductal system of pancreas followed by spread  of enzyme-rich fluid both anteriorly and posteriorly results  in pancreatic ascites.    e Serum amylase, may be elevated due to reabsorption across  parietal membrane.   ¢ Ultrasound/CT: It can detect fluid, pancreatic duct and guide  aspiration.    ¢ Repeated tapping—follow with albumin infusion. Most  of the cases are associated with hypoalbuminaemia  (Figs 25.158 and 25.159).   ¢ Surgical: If there is no response by 2—3 weeks, resection  (for tail lesion) or drainage for body lesion is indicated.    e This is emerging as a fine treatment for diabetes and chronic  pancreatitis after pancreatectomy.   ¢ Many patients have undergone islet cell transplantation with  minimal complications (see Chapter 49).    e Treatment of acute pancreatitis has been given in more  detail with more illustrations.   ¢ Islet cell transplantation, percutaneous cholecystostomy  and pancreatic divisum have been added.    Which of the following statements is false for  pseudocyst of pancreas?   B. Cystogastrostomy is usually the treatment of choice  C. Better to operate after 2 weeks    . Which of the following is not the cause for gall stone    Gall bladder carcinoma has following features except:  A. It is squamous cell carcinoma    Most ideal and quick method for the diagnosis of gall  stones is by:   B. If it perforates, gas under diaphragm is detected by  percussion    Cystic duct joins supraduodenal CBD in what  percentage of the cases?    Which one of the following is true for choledochal cyst?  A. Acquired dilatation    Which one of the feature is true in gall stone ileus?   C. Obstruction and features of perforation of gall bladder  are found   D. Cholecystectomy and extraction of the stones by  enterotomy is done   A. It can be seen in septic shock due to hypotension   C. Salmonella typhi can also give rise to acute acalculous  cholecystitis   C. Gall stone disease does not predispose to carcinoma  gall bladder   75% of islet cells are beta-cells producing insulin  80-90% pancreatic tissue is exocrine pancreatic tissue  5% of islet cells are A cells producing glucagon    To avoid spillage of cells after FNAC of the pancreatic  head mass which is the best investigation?   C. MRCP is not an ideal investigation for diagnosis  D. Endoscopic sphincterotomy has a definitive role   A. It is more prevalent in children with Down syndrome  B. It surrounds 2nd part of duodenum    The use of ultrasound in the diagnosis of acute  pancreatitis is mainly to:    Which one of the following is not routinely recom-  mended in severe pancreatitis?    The best surgical treatment for chronic pancreatitis  with dilated duct is:    ANSWERS  i € 2D 3 A 4D 5 B 6 D 7 A 8 B 9 C 10 A  11 D 2 BD 13 D 14 D 15 B To, D 17 D 18 D 19 D 20 D  2t G 22 A 25 VD 24 B 25 C 26 C 27 B 28 D 29 C 30 D    Diseases of the spleen and causes of enlargement of the spleen  are a great concern for physicians. Dozens of differential  diagnosis of splenomegaly are being taught in medicine.  However, a surgeon’s role in the treatment of an enlarged  spleen is minimal. However, it is he who does this ‘magic’ of  removing the spleen within minutes and saves the life of a  patient with a ruptured spleen. A surgeon plays a major role  by treating hypersplenism with splenectomy. In this chapter,  only topics related to surgeon’s interest are dealt with. Other  topics are mentioned briefly.    The spleen is an anatomically small organ, hidden underneath  the 9th to 11th ribs (dull note on percussion) measuring | x 3  x 5 inches and weighing about 7 oz (80—300 gm). It lies in  intimate contact with the undersurface of the diaphragm. This  explains why a splenic abscess can rupture through the  diaphragm causing empyema. When blood collects due to  splenic injury, it irritates the diaphragm causing referred pain  to the shoulder tip (Kehr’s sign).    The anterior border is notched and is in contact with the  stomach. It 1s enclosed by the gastrocolic omentum. The  surgical importance of this is that when traction is applied on  the stomach during vagotomy, a splenic capsular tear can occur,  resulting in bleeding. The two leaves of gastrocolic omentum    pass backwards 1n front of the kidney forming the lienorenal  ligament (Fig. 26.2). This ligament is responsible for some  part of the posterior fixation of the spleen. When this ligament  is divided, the spleen can be brought to the surface of the  wound during splenectomy.   The inferior part of the hilum of the spleen is (just) in  contact with the tail of the pancreas. Often, during an  emergency splenectomy, the tail of pancreas can get injured,  resulting in a pancreatic fistula (Figs 26.2 and 26.3)   ¢ Red pulp is a honeycombed vascular space which is made  up of cords of reticular cells and sinuses.   ¢ White pulp consists of lymphatic tissue and lymphoid  follicles containing lymphocytes, plasma cells and macro-  phages.    ¢ Arterial supply: Splenic artery is a branch of coeliac artery.  At the hilum of the spleen, it divides into 4—S trabecular  arteries and then branches into central arteries. Central  arteries pass through white pulp and give radial branches  to periphery including distal red pulp. Anatomical  knowledge of these branches helps when performing partial  splenectomy for ruptured spleen. (The blood flow rate  through spleen 1s about 300 ml/min.)   Fig. 26.1: Location of the spleen in relation to the fundus of the  stomach and the diaphragm    Fig. 26.3: Peritoneal ligaments attached to the spleen and com-  mon sites of accessory spleen    ¢ Venous drainage: Four to five branches of the vein joir  and form the splenic vein. This forms the portal vein by  joining with superior mesenteric vein behind the 2nd part  of the duodenum.   The lymph drains into hilar nodes, then into retropancreatic  nodes and finally into coeliac nodes. Since it has intimate  contact with the fundus of the stomach and tail of the pancreas,  spleen also used to be removed (nowadays not done) along  with the stomach and tail of the pancreas during radical  gastrectomy and distal pancreatectomy.    1. Destruction: Removal of abnormal aged, damaged red  blood cells by a process is called culling. Culling, the Greek  word implies sorting a collection of two groups. One that  will be kept and the other that will be rejected. For example,  spherocytes, in hereditary, spherocytes are broken down in  spleen. This happens in red pulp.   2. Production: Production of lymphocytes, monocytes and  plasma cells. Lymphopoiesis continues throughout life.  Lymphocytes take part in immune response of the body—  largest.   3. Antibody production: Spleen synthesises antibody in its  white pulp. It removes antibody-coated bacteria and anti-  body-coated blood cells via blood and lymph node  circulation.   4. Immune response: White pulp is composed of malpighian  corpuscles. They are composed of B lymphocytes and  T lymphocytes—which are in periarteriolar lymphoid  sheaths (PALS). Thus white pulp plays a major role in active  immune response through humoral and all mediated  pathways.   5. Reservoir of platelets: It 1s a storehouse of platelets, which  are released into circulation in an emergency situation.  Thus when spleen is enlarged, 80% of the platelets are  sequestrated resulting in thrombocytopenia.    6. Immune function: It is the major site for synthesis of  tuftsin, a peptide that stimulates phagocytic activity of  leucocytes. Opsonins are antibodies produced by spleen  against bacteria and fung.   7. Removal: Heinz bodies, Howell-Jolly bodies, etc. are  removed from RBCs. These cells are non-deformable intra-  cellular substances.    8. Erythrocyte production: During foetal development.  White and red blood cells production occur till Sth month  of gestation. In adults in case of myelofibrosis erythopoiesis  can occur.   — They can be found near the hilum of spleen, tail of  pancreas, splenic ligaments or in the mesocolon.   — In diseases of the spleen (haemolytic anaemia,  thrombocytopaenic purpura), all these accessory spleens  have to be removed along with spleen.   3. Polysplenia is rare. It occurs due to failure of splenic fusion.   4. Wandering spleen is due to loose ligaments. It is more  vulnerable for torsion.   5. Hamartoma: Rare, small masses of | cm in diameter. It  may in lymphoid tissue also (Hamartoma = misfire).   — Injury to the left side of chest, left lower rib fractures,  due to fall from a tree, road traffic accidents can be  associated with splenic rupture.   — Retroperitoneal haemorrhage, fracture spine and renal  injuries may be associated with splenic injury.   e Penetrating injuries to the abdomen may cause rupture  of spleen.   e Spontaneous rupture of the spleen is seen in malaria and  infectious mononucleosis, rarely in sarcoidosis, haemolytic  anaemia and leukaemia.   e latrogenic: Splenic capsule may be torn during surgical  procedures such as vagotomy or gastrectomy due to traction  on the stomach.    Most of these injuries are diagnosed by CT scan today (CT   1. Subcapsular haematoma: A capsular tear or parenchymal  tear results in subcapsular haematoma.   2. Clean (incised wounds): This can happen in sharp injuries,  staal injuries (not common) or operative injuries.   3. Lacerated wounds: Often they are multiple as in blunt  injuries abdomen.   4. Hilar injuries with pedicle avulsion are the most serious  injuries. Need urgent laparotomy and ligation of bleeders  and splenectomy. Otherwise, death will occur.   5. Splenic injury with other organs. Tail of pancreas,  diaphragm, left kidney, left colon.    Grade I: Nonexpanding subcapsular haematoma  <10% surface area  Nonbleeding capsular laceration with  < 2 cm deep parenchymal involvement.   Bleeding subcapsular. Haematoma or  subcapsular haematoma >50% surface area  Intraparenchymal haematoma >2 cm  Parenchymal laceration >3 cm deep  Ruptured intraparenchymal haematoma with  active bleeding.   Laceration involving segmental or renal  vessel > 25% spleen volume devascularised  Completely shattered or avulsed spleen.  Hilar laceration which devascularises entire   1. Tearing of splenic vessels: Avulsion of splenic pedicle can  result in severe haemorrhage and shock. Death can occur  within a few minutes. Even in the best of the situations,  death cannot be prevented often.   2. A slow developing haemorrhagic shock followed by  recovery. Examination reveals evidence of intraperitoneal  bleeding. This can occur due to a capsular tear or injury to  the splenic parenchyma. Its clinical features are as follows:  ¢ Anaemia—pallor  ¢ Pulse—tachycardia, more than 100/min  ¢ BP—low/hypotension  ¢ Cold, clammy extremities  ¢ Abdominal distension  ¢ Paralytic ileus develops slowly  ¢ Guarding and rigidity  ¢ Kehr’s sign 1s positive. Irritation of undersurface of the   diaphragm by the blood causes irritation of phrenic nerve  (C3, 4). Thus, this pain is referred to shoulder region  (supraclavicular nerve C4).    Kehr’s sign can be elicited by elevation of the foot end  of bed for about 10 minutes.    ¢ Ballance’s sign: Blood in the vicinity of spleen is fresh blood  which is coagulated and blood in the periphery is not  coagulated. Hence, there will not be shifting dullness on the  left side of the abdomen but can be present on the right side.   ¢ Saegesser’s splenic point of tenderness: An area of  tenderness on the left side between sternomastoid and  scalenus medius (rare sign).    3. Initial features of haemorrhagic shock, recovery and  sudden haemorrhagic shock after a few hours to a few  days. It is due to the following reasons:  ¢ Greater omentum seals off a tear which gets reopened after    In polytrauma, if a hypotensive patient responds to  intravenous fluids, tt is a hypovolaemic shock. Head  injury ts rarely a cause of hypovolaemic shock.    pancreatic ascites, pancreatic fistula or pseudocyst of pancreas.  Splenic artery pseudoaneurysm.  Splenic arteriovenous fistula.    Hb™ estimation, PCV (packed cell volume) estimation  (however, in appropriate cases it should be repeated at  frequent intervals to detect continuing haemorrhage).    (23 gauge) demonstrates blood either fresh or old. However,  diagnostic peritoneal lavage (DPL) is more reliable (vide    haematoma and can rule out other injuries. It is the most  important investigation in suspected cases of splenic injury.    . CT scan is more reliable. It should be used in cases of    doubtful diagnosis and stable patients.' It also rules out  hollow viscus perforation, pancreatic injuries, etc (Fig. 26.7).    'Deaths have been reported in CT scan room, in unstable patients with haemoperitoneum.    Diagnostic peritoneal lavage (DPL)  ¢ It is indicated in blunt injury abdomen where there are  equivocal signs or doubtful signs of peritonitis.    A | cm incision 1s made in the subumbilical region under  local anaesthesia (LA) after emptying the bladder. The  peritoneum 1s opened and a 12 or 14 Fr peritoneal dialysis  catheter is introduced into the peritoneal cavity.   The skin is closed and 1000 ml normal saline 1s allowed to  flow over 30 minutes.   The patient is turned to the right and left sides and fluid is  allowed to flow out.    DPL is positive when  1. Fresh blood of more than 20 ml isremoved immediately  after inserting dialysis catheter and it does not clot.    2. RBC count 1,00,000/mm? or WBC > 500/mm?.  3. Amylase level of over 175 units/dl.  4. Bile, food or any other fresh contents.  5. Gram stain is positive in the contents of lavage.  e Diagnostic accuracy 1s around 95%. DPL is useful to rule  out intestinal injury in addition to intraperitoneal bleeding.    DPL is more sensitive for mesenteric injury than CT  scan. However in early diagnosis of hollow viscus  injuries, DPL is superior to CT scan.    5. Plain X-ray abdomen! erect (should not be done in an   e It may detect free gas under the dome of diaphragm.    'Please do not make an unstable patient stand for the sake of X-ray. He may collapse due to hypotension.    When there 1s active bleeding with hypotension due to large  lacerations, emergency splenectomy is the treatment of  choice since it 1s quick, easy to perform and can be life-  saving. The splenic artery is ligated first at the upper border  of pancreas followed by splenic vein. In desperate  situations, the spleen 1s mobilised by incising the lienorenal  ligament, a large arterial clamp 1s applied at the splenic  hilum and splenectomy is done (Figs 26.10 and 26.11).    ¢ It can be done because the splenic artery gives an upper  polar branch and a lower polar branch which again  divides into 2 branches. Hence, when one of the branches  of splenic artery is ligated, the bleeding stops and a partial  splenectomy can be done comfortably.   After splenectomy, the spleen can be cut into multiple  pieces and can be implanted within the greater omentum.  The spleen survives because of neovascularisation and  functions like a spleen in the production of antibodies.  This can be done in children.    ¢ Itisa method of preservation of spleen when the general  condition of the patient is reasonably good.   ¢ In this, a small tear is sutured by using chromic catgut  and can be wrapped using greater omentum.    ¢ It is safe in selected adult and paediatric patients with  isolated splenic injuries.   4. Facilities for emergency resuscitation, ultrasonography, CT  scan should be available.    monitoring of haemoglobin, total WBC counts are done.  Abdominal girth and blood pressure are monitored. If  there is any doubt of hypotension, exploration is done.    2. Vascular injury (2-3%): Slippage of ligature (splenic .  artery), hilar dissection, capsular tear during retraction. - aa ac peoumese  Needs to be identified and sutured or coagulated or (d) anes ann  clamped III. Sometimes indicated   3. Injury to bowel: Colon, stomach. These can be avoided (a) Splenic ay ——  with careful dissection techniques. During ligation of short (b) Autoimmune haemolytic disease  gastric arteries too close to the stomach side, injury to the (c) Elliptocytosis haemolysis  stomach can occur. It is repaired in two layers. Colonic (d) Nonspherocytic congenital haemolytic anaemia  injury can occur during dissection to seperate the lower (e) Hodgkin’s disease with anaemia  pole. It is also repaired in the similar manner. (f) Thrombotic thrombocytopenic purpura   : (g) Idiopathic myelofibrosis  adhesions are present between spleen and diaphragm due  a ; ; (h) Splenic artery aneurysm  to perisplenitis or recurrent infarctions. Simple suturing . :  is enough if recognised. (1) Wiskott-Aldrich syndrome (Key Box 26.3)   5. Splenosis: This refers to multiple small implants of (j) Gaucher’s disease  splenic tissue on the peritoneal surface following traumatic  rupture of spleen. They can give rise to adhesions.   6. Pulmonary: These arecommon complications. Generally i  get unnoticed. It can range from minor cough to atelec- WISKOTT-ALDRICH SYNDROME SF  tasis to pneumonia and pleural effusion. Basal atelectasis  ° X-linked disorder predominantly presents as features of  is one of the common causes of postoperative fever. thrombocytopaenia such as epistaxis, bloody diarrhoea,   9. Subphrenic abscess: Not common. If It Occurs, ¢ The best treatment is human leukocytic antigen (HLA)  percutaneous drainage and antibiotics (Fig. 26.14). matched sibling bone marrow transplantation.    Pancreatic injury (0Q-6%): Treated by drain placement  and measuring drain amylase. Usually it is a minor  discharge, generally settles over a few days.    Sometimes it can be dangerous. They can present as  abdominal pain and vomiting which is confused for  incision pain, gastritis, etc. Ultrasound examination 1s a  must. One of our patients had portal vein thrombosis    I. Always indicated  (a) Primary splenic tumour  (b) Hereditary spherocytosis  II. Usually indicated  (a) Primary hypersplenism  (b) Chronic ITP    ¢ The spleen is probably responsible for sequestration of  platelets and for production of antibodies.    Type 1: Acute—children: Follows an acute infection and  resolves spontaneously in about two months.    ¢ Condition is common in females, F :M :: 3: 1.   ¢ Purpuric patches (ecchymosis refers to skin discolouration  due to extravasation of blood) are found on the buttocks  and petechial haemorrhages (spots) are found on the limbs.  These are dependent areas having high intravascular  pressure (Figs 26.15 to 26.17).   ¢ All types of haemorrhages are common and can be mild or  moderate (Key Box 26.5).   COMMON TYPES OF HAEMORRHAGES  e Epistaxis  e Menorrhagia  e Haematemesis  e Bleeding gums  e Haemarthrosis  e Haematuria    ¢ Intracranial haemorrhage is found in 1—2% of patients but  it may be the cause of death.    ¢ In 25% of cases, the spleen is just palpable (small size).    ¢ Hess’s tourniquet test: More than 20 petechiae in a circle  of 3 cm diameter in cubital fossa suggests purpura.    PEARLS OF WISDOM  If the spleen is big, it is not ITP.    ¢ Bleeding time is prolonged in purpura. Clotting and  prothrombin times are normal.   ¢ Bone marrow biopsy: Precursor of platelets (mega-  karyocytes) are increased.    ¢ Short course of corticosteroids is beneficial. Tablet pred-  nisolone in the dose of 10 mg/day over a period of  6 weeks is given.    In adults  Splenectomy is indicated in the following situations:  ¢ When ITP has presented for more than 6—9 months.    ¢ When ITP has relapsed in spite of steroids, the patient is  given a trial of prednisolone, 1 mg/kg/day. Platelet count  rises within 7 days of starting steroids after which splenec-  tomy can be done (Key Box 26.6).    ¢ Also indicated if a patient has two relapses on steroid  therapy or if the platelet count is less.    e Can be done even when platelets are as low as 10,000  cells/mm?,    e Ligate splenic artery first at the upper border of pancreas  after opening lesser sac.    e Splenectomy is not difficult. Accessory spleens  (15-30%) if present, should be removed.    e Platelet transfusion may be required postoperatively, if there  is bleeding.    70-80% of patients respond permanently and do not require  any further treatment. Counts rise above 1,00,000/mm? in  7 days. Even if counts are not raised, recurrent bleeding rarely  occurs.    This type of anaemia results from an increased red cell  destruction which occurs in the spleen. The life-span of red  blood cells is also shortened. Hence, irrespective of the cause  of haemolytic anaemia, these cases can benefit from  splenectomy. Anaemia, Jaundice and splenomegaly are the  triad of haemolytic anaemia.    This hereditary disorder is transmitted by either parent as  Mendelian autosomal dominant. The disease is characterised  by a defect in cell wall protein of the red cell, namely  spectrin, which increases reflux of sodium into the cell. This  causes the biconcave red cell to swell and become spherical.  Hence, the name spherocytosis.    1. The spherocyte 1s already weak. Adding on to this, there 1s  a greater loss of membrane phospholipid which results in a  delicate fragile spherocyte.   2. Because of the altered shape, oxygen and energy  requirements of RBCs increase which cannot be met by  the spleen. In the spleen, they are destroyed and release  excessive haemoglobin which is converted into bilirubin.  ¢ The amount of bilirubin produced is increased and most   of it is unconjugated. It gets attached to albumin as It Is  lipid soluble and therefore, cannot be excreted in the  urine. Hence, this type of jaundice is also known as  acholuric jaundice.    Most of this bilirubin is excreted in the biliary tree  resulting in pigment stones in the biltary tree.    ¢ Commonly seen in young children but can manifest in adults  also.   e History of recurrent attacks of jaundice can be elicited from  childhood.   e Jaundice is mild, never deep, not associated with itching or  bradycardia.   ¢ Pallor is an important feature due to destruction of the red  cells.   e Acute haemolytic crisis: It is precipitated by an acute  infection or stress. Abdominal pain, nausea, vomiting,  pyrexia, pallor are the features. The condition can be  confused for an abdominal emergency. In severe cases,  anaemia, thrombocytopenia, leukopaenia can occur.   e Spleen is moderately enlarged. The liver is also palpable  in a few cases.   ¢ In adults, gall stone colic and obstructive jaundice due to  CBD stone can also complicate the disease process.  Incidence of gall stone disease 1s around 50%. It is common  after the age of 10.    Young, pale, jaundiced, fatigued child with gall stones—  Think of hereditary spherocytosis.   2. Reticulocyte count is increased (15-25%): These are  immature red cells which are discharged by the bone  marrow due to loss of red cells.   ¢ Ideally, it should be done from the age of 6 to 10 years  Why after 6 years? The incidence of post-splenectomy  sepsis 1s more when splenectomy is done within 6 years 0;  age. Why before 10 years? After 10 years of age, the  incidence of gall stones increases.    Splenectomy helps in the following ways (Figs 26.19 to 26.22)  1. By decreasing erythrocyte destruction, anaemia improves.  thus avoiding blood transfusions.  2. Erythrocytes achieve normal lifespan. Hence, jaundice also  disappears.  3. Leg ulcers heal quickly.  ¢ If gall stones are also found in these cases, laparoscopic  splenectomy and cholecystectomy can be done in the  same sitting.    4. Fragility test  ¢ Normal red cell haemolysis occurs in 0.47% saline  solution. Here, it occurs at 0.6% or even in weaker  solution.   Fig. 26.19: Case of hereditary spherocytosis with faint jaundice  in a 22-year-old girl    Due to an autoimmune reaction, the red cell surface is damaged  and it gets destroyed in the splenic red pulp. Antibodies develop  against red cell antigens. In 50% of cases, the cause is not  known. The following are the probable causes:    ¢e More common in females after 50 years of age.  ¢ Splenomegaly, moderate in size  ¢ Gall stones are found in around 25% of the cases.   ¢ Spherocytes may be present because of damage to the red  cell surface.   ¢ Corticosteroids: A short course of prednisolone  60 mg/day 1s given.   ¢ Splenectomy is done when steroid response Is not  satisfactory. 80% of patients respond to splenectomy.    It 1s also called Cooley’s anaemia, Mediterranean anaemia,  Heinz body haemolytic anaemia.    e Itisa hereditary disorder, transmitted as a dominant trait.  It is characterised by a defect in haemoglobin peptide chain  synthesis. Depending on type of peptide chain involvement,  it is classified into a, B and y. However, [-thalassaemia is  more common. There 1s a decrease in haemoglobin and red  cells will be destroyed prematurely due to intracellular  precipitates (Heinz bodies).   ¢ When it 1s a heterozygous disorder, it is called beta-  thalassaemia minor and when homozygous, it 1s called beta-  thalassaemia major (Cooley’s anaemia). The abnormal  gene is inherited from either or both the parents.   ¢ Splenomegaly is mild to moderate in size. Sometimes, it 1s  massive resulting 1n abdominal discomfort or may cause  abdominal distension.   ¢ To compensate for anaemia, bone marrow hyperplasia  occurs resulting in bossing of skull and prominent malar   bones. Thalassaemia major usually manifests in the first  year of life.   1. Haemosiderosis (deposition of iron in tissues) of pancreas  results in chronic pancreatitis and diabetes.    ¢ Peripheral smear demonstrates microcytic hypochromic  anaemia. Red cells are small, thin and are resistant to  osmotic lysis.   ¢ Haemoglobin electrophoresis reveals reduction or absent  levels of haemoglobin A (HbA). There is a compensatory  increase in foetal haemoglobin (HbF).   ¢ Surgical: Splenectomy is indicated in a few cases who  require multiple transfusions and patients with gross  splenomegaly.    This is a hereditary haemolytic disorder in which haemoglobin A  is replaced by haemoglobin S (HbS). This results in crescent-  shaped erythrocytes. This HbS molecule crystallises if there  is hypoxia, strenuous work or due to dehydration. As a result  of this, red cells are distorted and elongated. Therefore, blood  viscosity 1S Increased, causing obstruction in the splenic  circulation (Key Box 26.7).    ¢ Splenic microinfarcts  ¢ Splenomegaly or autosplenectomy due to repeated infarcts  (Fig. 26.23).    e The disease 1s very common in Africans. Most of the  patients in India have high HbF levels which protect HbS.  Hence, symptoms are not seen in the first few weeks.    FUNCTIONAL ABNORMALITIES IN  SICKLE CELL ANAEMIA  e Spleen sometimes acts as a large reservoir for red cells  and the sickle cells are destroyed.  e Antibody production is decreased.  e Spleen’s ability to filter Streptococcus pneumoniae is  reduced.   Fig. 26.23: Sickle cell anaemia—Nugget spleen (3 cm size). An  example for autosplenectomy    ¢ Abdominal pain ts due to recurrent infarcts or due to gall  stones in a few patients.  e Skin ulcers occur due to anaemia or hypoxia.    Microinfarction of carpal and tarsal bones resulting in  bony pains is described as hand-foot syndrome.    ¢ Splenectomy has a doubtful value. It 1s indicated only when  excessive numbers of RBCs are sequestrated causing  anaemia (Table 26.1 for comparison).    ¢ Infarct of cerebrum resulting in hemiplegia.  ¢ Mesenteric infarction resulting in gangrene of the bowel.  ¢ Pulmonary infarction resulting in chest pain.   It is defined as pancytopaenia in the presence of normal or  hypercellular bone marrow. Following are the causes of  hypersplenism:    ¢ It usually involves red pulp expansion. It is to be  remembered that in cirrhosis of the liver, 15% of patients  develop hypersplenism. Anaemia and thrombocytopaenia  are mild. Rarely requires splenectomy (a few causes are  given below):   1. Tropical splenomegaly due to malaria, kala-azar or  schistosomiasis, etc. Since these diseases are endemic  In tropical countries, it is called tropical splenomegaly.  Gross enlargement of the spleen and hypersplenism are  indications for splenectomy in such cases.   2. Myeloproliferative disorders: Splenectomy reduces  need for transfusion and may relieve abdominal  discomfort.   3. Portal hypertension: Maximum benefit is in segmental  portal hypertension with oesophagogastric varices  (Fig. 26.24).    ¢ Parasitic cyst is rare. Typically, it is caused by echino-  coccal disease (hydatid disease).   ¢ Traumatic cysts are due to a haematoma giving rise to  liquefaction. These are false cysts of the spleen.   ¢ Congenital cysts can be due to a dermoid cyst, haeman-  gioma or lymphangioma.   ¢ Among neoplasms, lymphoma is the commonest cause  of enlargement of spleen. Spleen used to be removed as  a part of a staging laparotomy. Now, it 1s very rarely  removed. Patients with large spleen of chronic myeloid  leukaemia, Gaucher’s disease and hairy cell leukaemia  (details later) will also benefit from splenectomy.   ¢ Haemangioma is the most common benign tumour of  the spleen. It may turn into haemangiosarcoma.   ¢ Spleen is removed in oesophagogastrectomy or upper  carcinoma stomach—only when it is directly infiltrated.  Sometimes it happens in carcinoma tail of pancreas/  splenic carcinoma.   Fig. 26.24: Splenectomy done for left-sided portal hypertension  due to splenic vein thrombosis    (Figs 26.28 to 26.31)  ¢ Splenic abscess can occur due to infected septic embol!  from otitis, typhoid fever or due to thrombosis of splenic  vein causing infarction followed later by infection   Fig. 26.25: Simple cyst of the spleen resulting in compression of  the spleen—appearance like that of a globe   Fig. 26.29: Same patient as in Fig. 26.28 with multiple abscesses   ¢ Felty’s syndrome refers to splenomegaly, neutropenia =F PS,  with rheumatoid arthritis. After splenectomy, leg ulcers  heal quickly and neutropenia also improves. The patient  may respond to steroids better and the incidence of  recurrent infection becomes less (Fig. 26.32).    Remember as Fig. 26.32: Felty’s syndrome. The patient had received steroids  for one year. See the Cushingoid facies    Figs 26.33 to 26.35: A 28-year-old man presented with pain abdomen. CT scan showed splenic artery aneurysm with haematoma and  thrombus suggestive of ruptured splenic artery aneurysm. He also had splenomegaly and features of portal hypertension. He was  alcoholic. He might have had pancreatitis and developed splenic artery aneurysm (Courtesy: Prof Sampath Kumar, Dr Basavaraj Patil,  Dr Dinesh, Dr Pawan Kumar Addala, Department of Surgery, KMC, Manipal)    It is an uncommon condition. More common in women, and  some of them occur during pregnancy.    ¢ Congenital: Young patients. These patients may present for  the first time during pregnancy with enlargement or rupture.    ¢ Acute pancreatitis: Inflammatory process may give rise  to pseudoaneurysm specially because of pancreatic necrosis.    ¢ Operative: Ligation of splenic artery at the upper border  of pancreas after opening lesser sac followed by  splenectomy.   ¢ The cells on a blood smear have an irregular outline due to  the presence of filament-like cytoplasmic projections.  Hence, the name hairy cells.   ¢ HCL is a clonal proliferation of abnormal B cells (very  rarely T cells).   Fig. 26.36: Patient had pyrexia of unknown origin with  pancytopaenia. Spleen was enlarged. Splenectomy was done and  the biopsy report was non-Hodgkin's lymphoma    ¢ Bloodcounts are low. White  cell count may be raised  with circulating hairy cells.   ¢ Bone marrow has increased  cellularity with char-  acteristic infiltration by  hairy cells.    ¢ Drug: 2-Chloradenosine  acetate (2-CDA) has been  beneficial. It can induce  remission.   ¢ Splenectomy is indicated  in cases of diagnostic  difficulties and in very  large spleens (Fig. 26.37).   Fig. 26.37: Splenectomy was  done for large spleen causing  discomfort, reported as hairy cell  leukaemia    3. Sickle cell disease  4. Gaucher's disease  5. Hodgkin's lymphoma  6. Hairy cell ieukaemia    Overwhelming postsplenectomy infection (OPSI):  Incidence of meningococcal and pneumococcal infections is  more in children after splenectomy. Hence, pneumococcal  vaccine must be administered in the postoperative period.  Following splenectomy, especially children are vulnerable  for opportunistic infections such as Pneumococcus,  H. influenzae and Meningococcus. Hence prophylaxis is  necessary.   OPSI can also be a major problem in adults who undergo  splenectomy for different conditions.   ¢ All children who undergo splenectomy before 5 years of  age, should receive daily dose of penicillin until 10 years.   ¢ Haemophilus influenzae type B vaccination for all  irrespective of ageand pneumococcal vaccine to be given  (repeated once in 5 years).   ¢ Meningococcal protection for only those who travel to  high-risk areas.    Vaccination should be given at least 2 weeks before  elective splenectomy.    ¢ Vaccines should be given as early as possible. However  protection is not always guaranteed.   ¢ Daily dose of oral prophylaxis with penicillin  erythromycin or amoxycillin to all children till 10 years  who undergo splenectomy before the age of 5.   Lymphoma is the commonest cause of enlargement of  spleen (in India, malaria is the cause).   It is the most common solid organ injured in blunt abdo-  minal trauma.   Most significant benefit of doing a splenectomy for hyper-  splenism is from segmental left-sided portal hypertension.  Most commonly performed investigation for suspected  splenic trauma is abdominal ultrasound.   Most common malignancy affecting spleen is lymphoma  Most common complication of splenectomy is pulmonary  complication   Most common primary tumour of spleen—Haemangioma  Most common primary malignant tumour of the spleen—  haemangiosarcoma   Most common indication for splenectomy—splenic trauma  Most common indication for splenectomy in elective  setting—ITP.    1. In splenic injury, Kehr's sign refers to: 6. The following is true about purpura:  A. Absence of shifting dullness on the left side of abdomen A. Bleeding time 1s prolonged  B. Tenderness on the left side between sternocleido- B. Clotting time 1s prolonged  mastoid and scalenus medius muscle C. Prothrombin time is prolonged  C. Tender left iliac fossa D. Activated partial thromboplastin time is prolonged  D. Shoulder pain due to irritation of the under-surtace of —§ 7, Which of the following vaccination has to be given after  the diaphragm splenectomy to prevent overwhelming post-  2. Spontaneous rupture of the spleen is seen in the splenectomy infection?  following except: A. Measles vaccine  A. Idiopathic thrombocytopenic purpura B. Pneumococcal vaccine  B. Malaria C. Hepatitis vaccine  C. Infectious mononucleosis D. Varicella vaccine  D. Leukaemia 8. The following is true about splenectomy in idiopathic  3. Diagnostic peritoneal lavage is said to be positive in thrombocytopaenic purpura (ITP):  all of the following except: A. Is required in all cases of ITP  A. Fresh blood > 20 ml ts aspirated after inserting dialysis B. Must be done when platelet count is > 100,000 cells/  catheter cu mm  B. Gram stain is positive in the contents of the lavage C. Accessory spleens, if present must be left behind  C. RBC count > 100,000 cells/cu mm D. Two-thirds of patients will be cured by surgery  D. Amylase level of 75 units/dl 9. The following is true about splenectomy for hereditary  4. The following is not true about nonoperative treatment spherocytosis:  in splenic trauma except: A. Splenectomy should be done as a last resort  A. Hemodynamically stable patient. B. Splenectomy must be done either before 6 years or  B. Hollow viscus injury after ten years of age  C. Close monitoring 1s available C. Accessory spleen, if present must be left behind  D. Hospitalisation is required D. Jaundice disappears after splenectomy  5. Big spleen is not a feature of: 10. The most common benign tumour of the spleen is:  A. Malaria A. Haemangioma  B. Idiopathic thrombocytopaenic purpura B. Lymphoma  C. Portal hypertension C. Tuberculoma  D. Leukaemia D. Lipoma  ANSWERS  1D 2A S D 4B 5B 6A 7B 8 D 9D 10 A    Peritoneal cavity is the largest cavity in the body  accommodating various viscera. [tis divided into greater and  lesser sac (omental bursa) which communicate through the  foramen of Winslow or epiploic foramen. The peritoneum  lining inner side of the parietes is called parietal peritoneum.  It is very sensitive and is innervated by both somatic and  visceral afferent nerves. This explains the sharp, localised,  cutting pain of peritonitis. Diaphragm and central part of the  peritoneum is supplied by phrenic nerve (C4) and partly by  intercostal nerves. Rest of the peritoneum is supplied by  intercostal nerves and lumbar nerves.   Lesser omentum: It is also called hepatoduodenal ligament.  It extends from the duodenum to the liver. This has two layers  and within these layers are the common bile duct, hepatic artery  and hepatic portal vein.    The peritoneum is lined by a single layer of flattened cells  and a thin layer of fibroelastic tissue. It is parietal peritoneum.  A large peritoneal defect heals within a few hours because of  these mesenchymal cells (flattened polyhedral cells—  mesothelium). Applying this principle, some surgeons do not  close the peritoneal layer after laparotomy. When parietal  peritoneum 1s reflected into viscera, it 1s called visceral  peritoneum.    e Retroperitoneum  e Retroperitoneal cyst, abscess, tumour  e What is new?/Recent advances    It covers viscera and is supplied by autonomic nervous  system. Hence, it is not sensitive. Thus, gastrojeyjunostomy  can be done under local anaesthesia but distension and traction  to the bowel causes pain. During herniorrhaphy under spinal  anaesthesia, handling of bowel or traction on the bowel can  produce uncomfortable upper abdominal pain.    Peritoneal surface is a semipermeable membrane with an area  comparable to that of cutaneous body surface. Nearly | m? of  the total 1.7 m* area participates in fluid exchange with  extracellular fluid space atthe rate of 500 ml or more per hour.   It normally contains less than 50 ml fluid. When it is insulted  by infection, a large amount of fluid can collect in this space  giving rise to severe fluid and electrolyte imbalance. This ts  described as I/II space loss, e.g. peritonitis, pancreatitis.  Peritoneal fluid helps in smooth gliding of intestines.  Absorption of fluid and secretion of fluid are some important  functions of peritoneum (Key Box 27.1).    NORMAL PERITONEAL eae  e Specific gravity below 1016  e Protein concentration less than 3 gm/dL  e White blood cell count less than 3000/uL  e Complement mediated bacterial activity  Lack of fibrinogen related clot formation    This takes place through capillaries and lymphatics present in  between the two layers of peritoneum. This principle is applied  in dialysis. The direction of circulation is towards  subdiaphragmatic lymphatics.    It secretes prostaglandins, interferons and free radicals which  help in some protection against peritonitis.    This term refers to peritonitis from an intra-abdominal source  and is the most common form of peritonitis. The following  are the causes for secondary peritonitis (Fig. 27.1):    1. Perforation of a hollow viscus  ¢ Perforated duodenal ulcer, gastric ulcer  ¢ Perforated enteric ulcer, tubercular ulcer  ¢ Perforated Meckel’s diverticulum  ¢ Perforated colonic ulcer    2. Direct spread: Post-inflammatory  ¢ Acute cholecystitis-gangrenous  ¢ Acute appendicitis  ¢ Gangrene of the intestine  e Acute necrotising pancreatitis    3. Penetratinginjuries to the abdomen, where the organisms  gain entry from outside.    4. Postoperative peritonitis is due to the introduction of  infection during surgery which might be due to:  ¢ Postoperative leaks  ¢ Foreign body (mop) in the abdomen    5. Parturition peritonitis: It refers to peritonitis after  pregnancy and delivery.    6. Blunt injuries to the abdomen  ¢ Fluid which is spilled into the peritoneal cavity (example:  Blood and bile can travel along paracolic gutter and  manifest as pain in the right iliac fossa, causing guarding   and rigidity. This has been called Valentino syndrome  (see later in page 637).    Due to any one of the reasons mentioned above, infection sets  in and the causative organisms multiply in the peritoneal cavity.    ¢ Gram-negative organisms: Escherichia coli (E. coli),  Proteus, Klebsiella. They are present in the small and large  bowel. They are the commonest organisms producing  peritonitis (Key Box 27.2).   ¢ Enterococci: Streptococcus faecalis needs bile to grow. It  is present in the urinary tract, genital tract and also in the  intestines. However, both aerobic and anaerobic  streptococci are the second most common organisms  producing peritonitis. They are the chief organisms in  puerperal sepsis.    ¢ Bacteroides: They are anaerobic organisms, present mainly  in the lower intestine.    ¢ Bacteria from outside alimentary canal: Gonococcl,  pneumococci, tubercular organisms, etc.    ¢ These organisms proliferate in the peritoneal cavity  resulting in peritonitis. As a result of this, there 1s secretion  of a large amount of fluid into the peritoneal cavity resulting  in 3rd space loss which leads to severe hypovolaemic  shock. This fluid is rich in proteins, bacteria and toxins.  Due to powerful endotoxins released by gram-negative  bacteria, endotoxic shock or septic shock (refer to shock),  ensues.   ¢ The fluid is rich in fibrinogen which forms fibrin and helps  in localisation of infection (Fig. 27.3).   e Peritoneum loses its shiny surface, becomes reddish and  oedematous and is covered with thick fibrinous exudate.   ¢ Omentum: It is a fatty apron with rich blood supply. A  mobile double-layered peritoneal fold acts like a policeman  to seal the area of infection or perforation. Examples:  Perforated duodenal ulcer, acute appendicitis, acute  diverticulitis, etc. Probably it also serves to supply collateral  blood supply to the ischaemic viscera. It also has  immunological functions such as supply of phagocytes  which destroy unopsonised bacteria.   e lleal perforation, appendicitis—aerobic bacteria in 30%  patients, anaerobic in 10% patients. Predominant aerobic  bacteria include gram-negative E.coli, Streptococci,  Proteus and Klebsiella.   ¢ Colonic-rectum: Faecal spillage produces a load of 10'°  or more—gram-negative and anaerobic bacteria per gram  of stools.    Perforated duodenal ulcer  Perforated gastric ulcer  Perforated appendicitis  Perforated enteric ulcers  Perforated tubercular ulcers  Gangrene intestine   Fig. 27.3: Fibrin plaques: Early cases of peritonitis with fibrin  Fig. 27.2: Pathophysiology of peritonitis plaques all over the peritoneal cavity    A. Local peritonitis: If a perforation is small and if it is sealed  off immediately by omentum, it will give rise to local  peritonitis. Examples: Small gastric ulcer perforation or  diverticular perforation, gall bladder perforation.  Anatomical factors also play a role in local peritonitis.  Examples: Retrocaecal appendicitis with perforation. It 1s  behind the caecum and in retroperitonaeum. Signs are  confined to right iliac fossa only. In posterior gastric  perforations or acute pancreatitis, signs are limited to upper  abdomen. Pelvic peritonitis is another example—occur  following septic abortions or salpingo-oophoritis.   B. Generalised peritonitis: If the contents of the viscus leak  into the peritoneal cavity with force, as 1t occurs 1n intestinal  perforation or due to perforation of a free lying organ—  example: Meckel’s diverticular perforation. Virulence of  bacteria is more as tn colonic perforations with generalised  peritonitis. Duodenal ulcer perforation can manifest as  severe pain in the right iliac fossa mimicking appendicitis.   =  Many have been operated as appendicitis. Reason being a  broad right paracolic gutter and the contents travel down  into right iliac fossa. This has been referred to as Valentino  syndrome (Key Box 27.3).    e Rudolph Valentino was an Italian actor who lived in early  20th century.   e On August 15, 1926, he was admitted with the diagnosis  of appendicitis and gastric ulcers, with peritonitis.    e It is also called Valentino syndrome because in any  inflammation of the upper abdominal viscera, contents can  travel down the right paracolic gutter into the right iliac fossa  resulting in pain and tenderness mimicking acute  appendicitis.    ¢ Clean perforation: Upper Gl-Gastric juice remain sterile  for 6-8 hours. Hence, in early stages, there will be mild  chemical peritonitis and early treatment Is give good results.   ¢ Distal gut perforation and infected bile peritonitis: Very  dangerous and severe, causing sepsis and septic shock early.    It depends upon whether it is localised peritonitis or  generalised. In cases of retrocaecal appendicitis, the abdominal  signs may be minimal but guarding and rigidity of the back  muscles is characteristic. Features of generalised peritonitis  are as follows:   Fig. 27.5: Peritonitis due to ileal perforation consequent to tuber-  culosis. Usually it is an ulcerative variety   : Fig. 27.13: Faecal peritonitis and faecal fistula due to anasto-  Fig. 27.12: Sigmoid diverticular perforation causing peritonitis. | motic leak following right hemicolectomy. Ischaemia and tension  Sigmoid colon is the most common site of acquired diverticulli are the two common causes for the leak    ¢ Severe abdominal pain which is cutting in nature, becomes  worse on movement of the abdominal wall. Hence, the  patient lies still on the bed.   ¢ The pulse rate is increased. An increase in the pulse rate  may be an early indication of peritonitis, in cases of  gangrene of the bowel or peritonitis following perforation  of bowel.   ¢ High-grade fever with chills and rigors indicates a  septicaemic process.   ¢ Rebound tenderness (Blumberg’s sign): Abdomen is  pressed for a few seconds. The patient experiences pain.  Sudden release of pressure causes severe pain. It is due to  sudden movement of the sensitive parietal peritoneum  (Fig, 27,19).   ¢ Bowel sounds are absent. Distension of the abdomen occurs  within a few hours due to accumulation of fluid and paralytic  ileus.    1. Complete blood picture shows high total count with  predominant neutrophil count.    2. Blood examination for sugar is done to rule out diabetes  mellitus. Empyema gall bladder with or without perforation  can present as septic shock. Often, they are diabetic.   Fig. 27.22: Lateral decubitus X-ray showing free gas in the peri-  toneal cavity    4. Abdominal USG to detect fluid in the abdomen.  Following are different fluids which may give clue to the    ¢ Bile—green coloured—duodenum, stomach, gall bladder  perforation  ¢ Faeculent—dark green coloured thick aspirate with faecal  odour—ileal perforations, postoperative anastomotic leaks  e Serous—exudative—early acute pancreatitis, tuberculous  peritonitis  ¢ Haemorrhagic—haemorrhagic pancreatitis  ¢ Food particles—hollow viscus perforation  Thus ultrasound has so many advantages even though it  may not point at the specific site. However, probe tenderness  with fluid in the right iliac fossa may suggest acute  appendicular perforation. Very thick contents such as anchovy  sauce from ruptured amoebic liver abscess cannot be aspirated.  However, ultrasound will give clue about the liver abscess.    5. Abdominal tap  ¢ Aspiration of blood indicates haemoperitoneum or  gangrene of the bowel.  ¢ Aspiration of bile indicates biliary peritonitis due to  perforation of duodenal ulcer, gall bladder or intestine.    ¢ Aspiration of frank pus indicates peritonitis due to  gram-negative bacteria. Foul-smelling pus is due to  anaerobic bacteria producing free fatty acids and their  esters. Always send the fluid for culture sensitivity  GPig: 27.26):    6. Contrast-enhanced CT scan  ¢ When the signs and symptoms are equivocal, CT is the  ideal investigation.  ¢ CT can diagnose hollow viscus perforation, especially  when there 1s no gas under the diaphragm.  ¢ CT can detect ischaemic changes due to gangrene of the  bowel-gas in the bowel wall (Figs 27.27 and 27.28)    ¢ CT can diagnose unsuspected and unexpected lesions in  the abdomen including diverticular perforations, internal  herniation and gangrene, acute pancreatitis, etc.   ¢ Adequate hydration and normal renal function (as  indicated by normal creatinine values) are important  before a contrast-enhanced CT scan.    Fig. 27.28: CT showing hypodense lesion in the left iliac fossa—  a case of sigmoid diverticular perforation    7. Diagnostic laparoscopy can be used in suspected cases of  peritonitis (Key Box 27.6).   2. Bowel care and blood: Purgatives should not be given as  it may result in perforation. Blood is arranged for surgery.   3. Charts: Temperature, pulse rate, respiratory rate, intake-  output charts are maintained.   4. Drugs are given against gram-positive, gram-negative and  anaerobic organisms (Key Boxes 27.7 and 27.8).    e 2nd or 3rd generation cephalosporins should be started  as early as possible   e Once culture and sensitivity reports are available (after  surgery), antibiotics can be changed   5. Exploratory laparotomy and appropriate surgery is done  followed by thorough peritoneal toilet/wash with normal  saline.    6. Fluids—IV fluids are given before, during and after surgery.  Central venous cannulation and measurement of central  venous pressure (CVP) is indicated in unstable patients to  guide fluid therapy. If not possible, an emergency cut down   (venesection)—cephalic or basilic vein, is done followed  by fluid infusion. Preoperatively the aim is to maintain at  least 30 ml/hr of urine output.    e Restore intravascular volume. Crystalloids: Ringer lactate  or isotonic saline stay in the intravascular space for a short  period, larger volumes required  Colloids: Longer duration of action, smaller volumes are  sufficient and can be used in cardiac patients   e Restore oxygenation by face mask or mechanical  ventilation, as necessary    2. As soon as the peritoneal cavity is opened, purulent fluid  comes out. The fluid 1s collected and sent for culture and  sensitivity. Greenish fluid indicates a hollow viscus  perforation. All the fluid is drained, the source of peritonitis  is identified and appropriate surgical procedure is done.  Examples are  ¢ Appendicectomy for appendicitis.  ¢ Closure of perforation for perforated peptic ulcer.  ¢ Closure or resection for ileal perforation.  ¢ Resection of the bowel for gangrene.   Control of sepsis: This is the most important step of  treatment of peritonitis. Removal of septic focus is a  primary aim—examples: Appendicectomy, perforation  closures (dudodenal ulcer) or resection (intestinal or colonic  perforation) or cholecystostomy in difficult perforated gall  bladder diseases. However, all the septic foci in the abdomen  have to be removed—necrotic material, pus pockets and  food particles. Thorough irrigation with warm saline cleans  up subhepatic spaces, pelvic spaces and interloop  collections. Primary anastomosis In presence of sepsis may   Fig. 27.30: A late case of duodenal ulcer perforation who presented  with peritonitis with sympathetic pleural effusion. Laparotomy,  closure of perforation and feeding jejunostomy were done. Drains  in the subhepatic space and right pleural cavity were placed. Patient  had stormy postoperative period with biliary fistula, which was  closed after 6 weeks    result in leak and postoperative peritonitis. It is better to do  colostomy or ileostomy in such cases. Incision can be  partially closed leaving the skin open—sutures can be tied  after 2 days in the ward. In a few cases, laparostomy is  done if you suspect abdominal compartment syndrome.   3. [t is better to use nonabsorbable suture material such as  silk to do an intestinal anastomosis or for closure of  perforation. In the presence of infection, absorbable sutures  such as catgut get absorbed very fast.   4. A thorough peritoneal wash/lavage ts given by using warm  saline (up to 3—5 litres) to avoid intraperitoneal abscesses.  Antiseptic agent such as betadine solution should be avoided  because they can cause adhesions (Key Box 27.10).   5. Peritoneal cavity 1s drained to the exterior by using tube  drains. These are kept in the subhepatic space and in the  pelvic cavity.   e Aminoglycoside lavage may cause respiratory depression  due to neuromuscular blocking action of these drugs. Mops  must be used to dry the peritoneal cavity. If fluid is left over,  it may dilute the opsonins and thus decrease phagocytosis.   Fig. 27.33: Principles of management of peritonitis—identifying  the source of sepsis and elimination is the key to Success    7. Tension sutures are put depending upon the severity of  the peritonitis to prevent burst abdomen.    8. Laparostomy (vide infra): This method of exposing the  peritoneal cavity can be done in selected cases, if you  suspect abdominal compartment syndrome.    This refers to leaving peritoneal cavity exposed to outside  without approximation of the anterior abdominal wall. Some  situations arise, especially in emergency cases, where this 1s  required. Hence, it 1s important to know how to deal with this  situation.    1. Open laparostomy: Abdominal fascia and peritoneum are  not sutured.  Advantages: Abdominal compartment syndrome can be  prevented. Details are given later.  Disadvantages: Significant fluid loss and secondary  infection.    2. Closed laparostomy or mesh laparostomy: Here the  fascial layer is closed by using marlex mesh or prolene  mesh or even a zip to protect exposed viscera.  Advantages: One can minimise infection.  Disadvantages: Abdominal compartment syndrome and  perforation of bowel can occur.    When a second look procedure is contemplated, e.g. acute  pancreatitis, mesenteric ischaemia.    1. Too sick a patient to tolerate the surgical procedure.  2. Sealed perforation  3. Localised peritonitis—may resolve with treatment.    The phrase “abdominal compartment syndrome” was coined  in 1984 when Irving Kron, described the measurement of intra-  abdominal pressure as a means of developing criteria for  abdominal decompression to improve organ function.   ¢ Abdominal compartment syndrome (ACS) 1s defined as a  sustained increase in IAP more than 20 mmHg [with or  without an abdominal perfusion pressure (APP) < 60  mmHg)] that 1s associated with new organ dysfunction/  failure (Fig. 27.34).    ¢ Intra-abdominal pressure (IAP) 1s the pressure concealed  within the abdominal cavity. Normal IAP is 0-5 mmHg  showing phasic variation with respiration.    ¢ Intra-abdominal pressure is measured to detect abdominal  compartment syndrome and to decide on the requirment of  a decompression so as to improve organ function.    ¢ The adverse physiological effects of intra-abdominal  hypertension (IAH) affect almost every organ system  resulting in ACS. The major systems affected, 1n decreasing  frequency of incidence and morbidity are: Pulmonary,  cardiovascular, renal, splanchnic, central nervous system.  Thus the end result can be:    Intractable hypoxia, hypercarbia, ARDS  ~ Cardiac insufficiency and cardiac arrest  Oliguria, anuria, acute renal failure    Grading of IAP (Burch)  Grade I: IAP 12-15 mmHg  Grade II : [AP 16-20 mmHg  Grade III : [AP 21-25 mmHg  Grade IV : [AP > 25 mmHg    A. Diminished abdominal wall compliance  |. Acuterespiratory failure with elevated intrathoracic pressure.    2. Abdominal surgery with primary fascial or tight closure.  Example: Reduction of massive hernia (Figs 27.35 and 27.36).    3. Major trauma/burns  4. Prone positioning, head of bed >30°  5. High BMI, central obesity   Fig. 27.35: This boy had abdominal compartment syndrome Fig, 27.37: Close monitoring of patient with vital signs and blood  after reduction of intestinal contents from left thoracic cavity and — transtusion in addition to antibiotics play a major role in the    we could not close the abdomen. It was covered with a thin plastic management of septic shock  sheet—you can see the intestine. It took two months for granula-  tion tissue to cover the defect. Eventually, he recovered with an  incisional hernia. Bogota bag or VAC are the other alternative meth-  ods of closure   Fig. 27.36: Loss of abdominal layers following reduction of a mas-  sive ventral hernia    A young boy of 18 years, who suddenly had breathlessness  was found to have diaphragmatic hernia. After reduction  and closure, patient developed ACS. Reopening of abdomen  was done and peritoneum was not closed but a ‘cover’  was given by using ‘urosac’ bag which was split open.  Wound was allowed to heal by granulation tissue.   ¢ Bogota bag: A Bogota bag is a sterile plastic bag used for  closure of abdominal wounds.! It is generally a sterilised,  3 litre genitourinary irrigation bag that 1s sutured to the skin  or fascia of the anterior abdominal wall. The Bogota bag  acts as a hermetic barrier that avoids evisceration and loss  of fluids. Another advantage to the Bogota bag, is that the  abdominal contents can be visually inspected which 1s  particularly useful in cases of ischaemic bowel. Thus useful  in resections following mesenteric ischaemia. In our country  we can use urosac bag (which can be split open) or even a  thin plastic sheet can be used like a Bogota bag (Fig. 27.35).   ¢ Vacuum assisted closure (VAC): It has been extensively  used in the management of leg ulcers specially diabetic  ulcers. It has been used 1n a few cases of severe pancreatitis.  Here it 1s called open abdomen negative pressure therapy  system. It removes debris, inflammatory exudates.    No doubt, drainage of the septic focus is the most  important step.    1. Severe hypovolaemic shock giving rise to renal failure. It  can be prevented by adequate hydration of the patients and  careful usage of antibiotics such as gentamicin.   2. Septic shock, multiorgan failure and death occur in late  cases of peritonitis.    'Bogota bag’s use was first described by Oswaldo Borraez in Bogota,  Colombia.    This refers to accumulation of pus in the rectovesical pouch  or pouch of Douglas (rectouterine pouch).    ¢ Any peritonitis, commonly following perforation due to  acute appendicitis or following salpingo-oophoritis. The  rectovesical pouch 1s the most dependent part in the body.  Hence, the septic emboli accumulated in peritoneal space  give rise to pelvic abscess.    ¢ Perforated duodenal ulcer, perforated ileal ulcers on the  other common causes.    ¢ History of discharge of mucus per rectum for the first  time in a patient who is recovering from peritonitis  suggests pelvic abscess. It occurs due to irritation of the  rectum. Increased frequency of micturition occurs due to  Irritation of bladder.    A 36-year-old lady underwent vaginal hysterectomy for  dysfunctional uterine bleeding. To control the bleeders,  several gauze pieces were used without a proper count.  After 2 weeks, purulent discharge per vagina, fever, ill  health was reported. Ultrasound done showed a pelvic  abscess. CT scan done in our hospital showed foreign body  with air trap suggesting gauze pieces. Her abdomen was  explored, abscess drained and the gauze pieces were  removed.   ¢ Confirmed by per-rectal examination. A tender boggy  swelling is felt in the anterior wall of rectum. Ultrasound  can define an abscess and can detect the size of the  abscess.    ¢ CT scan is very useful in defining pelvic abscess, its extent  and to detect presence of a foreign body (Fig. 27.39).    Fig. 27.39: CT scan showing gauze pieces—she had persistent  foul-smelling vaginal discharge    ¢ Under general anaesthesia, a proctoscope !s introduced and  a nick is made in the anterior wall of the rectum to open  into the abscess cavity. Pus 1s drained with a sinus forceps  through the rectum. There 1s no peritoneal contamination.  The cavity collapses after a few days. Postoperatively, the  patient is given broad spectrum antibiotics.    ¢ As aresult of peritonitis, residual abscess can collect in the  intraperitoneal cavity. Pus that collects under the diaphragm  is described as subphrenic abscess. Subphrenic abscess is  the commonest intra-abdominal abscess.   ¢ Gastrointestinal perforations, postoperative leaks,  penetrations, traumas, puerperal sepsis, are the common  causes of subphrenic abscess.    There are 5 subphrenic spaces between the diaphragm and  the liver bounded by various peritoneal folds. Four are    intraperitoneal and one is extraperitoneal. The spaces, boun-  daries and the common causes of pus in these spaces are  described in Table 27.1 and Figs 27.41 to 27.43.   1. Right subhepatic space  2. Right subphrenic space  Duodenum 3. Left subhepatic space    Fig. 27.43: Cross-section showing subphrenic spaces: (1) Right  anterior intraperitoneal space, (2) right posterior intraperitoneal  space, (3) left anterior intraperitoneal space, (4) left posterior in-  traperitoneal space (lesser sac)   ¢ The causative organisms of peritonitis are Escherichia coli,  Enterococci, Klebsiella, Enterobacter, Proteus, Bacteroides,  etc.    It lies between right lobe of the liver and diaphragm  Posteriorly—anterior layer of the coronary ligament and right triangular  ligament. On the left side is the falciform ligament    2. Left paracolic gutter is narrow and colophrenic ligament is  present on the left side.   3. Majority of diseases affect right side (perforation, liver  abscess, appendicitis, gall bladder disorders, etc.)  (Fig. 27.44).    ¢ A patient who is recovering from peritonitis complains of  fever with sweating. Initially, fever is low grade, continuous.  Later, there is high-grade fever with chills and rigors.   ¢ Deterioration of health occurs very fast with wasting and  anorexia.   ¢ Shoulder pain is due to irritation of undersurface of the  diaphragm by the pus (sensory fibres of the phrenic nerve  are irritated—C3, 4).    Fig. 27.44: Common causes of subphrenic abscess A postoperative patient who has pyrexia, prolonged ileus,    ¢ The high incidence of subdiaphragmatic abscess is due to subphrenic abscess.    of following reasons: ¢ Tenderness is present in the epigastrium on deep palpa-  1. Upward movement of diaphragm during expiration. tion.  2. Decreased intra-abdominal pressure * Common causes of postoperative fever are absent, e.g.    Subphrenic abscess is common on the right side because  of the following reasons:    1. Right paracolic gutter is wide and deep and colophrenic Harold-Riutcard    2. Plain X-ray abdomen (erect)—may show gas and fluid  level under the diaphragm (Figs 27.45 and 27.46)  3. Fluorescent radiography may reveal absence of  movement of right side of diaphragm on inspiration.  4. Ultrasonography confirms the site of abscess, number of  abscesses, loculations, etc.  * Abscess is characterised by hypoechogenic cavity  surrounded by sharp distinct echogenic wall. It can be  therapeutic to insert catheter for drainage.   Fig. 27.47: CT scan showing left subphrenic collection followinc  acute pancreatitis—a pigtail catheter is inserted (also see Fig. 27.48)    5. CT scan demonstrates well defined, low density mass.  the rim of which 1s enhanced after intravenous injection of  contrast medium. The mass tends to be round because  of centripetal expansion—highly sensitivity > 95% (Fig.  27.47).    Fig. 27.45: Plain X-ray—gas and fluid level under diaphragm: PA Gallium binds to proteins lactoferrin and transferrin  view and lateral view which are present in high concentration in an abscess.    Today with the availability of sophisticated imaging facilities,  percutaneous drainage has become the choice of therapy rather  than surgery. Both have been described in next column.    I. Percutaneous drainage can be done with the help of  ultrasound or CT scan, provided the abscess cavity is  unilocular, and the track 1s safe.   1. Pigtail catheter (using Seldinger’s technique): It is a small  tube used to drain bile, urine, pancreatic fluid or abscess.  Fig. 27.46: Plain X-ray—gas and fluid level under diaphragm: PA    Fig. 27.48: Pigtail catheter drainage of left subphrenic abscess  following gastric ulcer perforation    3. Sump catheter: It has a double lumen which permits  irrigation as well as drainage and allows a good suction  (Key Box 27.11).    More than 90% of subphrenic abscesses are managed  by percutaneous drainage successfully.   INDICATIONS FOR REMOVAL OF CATHETER Ys  e Drainage less than 10 mi/day  e No fever, no pain  e WBC counts return to normal   ¢ However, lesser sac abscess and abscesses connected with  the bowel, discharging pus or bile, are drained by intra-  peritoneal route.    AS) od O37) i A ed SO) oe od RO RES  POSTOPERATIVE PERITONITIS    Introduction: This is not an uncommon problem encountered  in the surgical wards. Very often patient who undergoes an  intestinal or a biliary surgery and a few days later vague  symptoms and signs develop. Hence it 1s difficult to diagnose  if one takes a casual approach. It has high mortality. Hence  requires early detection and demands early effective solution.   It should be suspected following surgery on intestines or  biliary tract, when a patient who is recovering from paralytic  ileus starts deteriorating or when paralytic ileus does not return  back to normal.    Etiology (Figs 27.49 to 27.52)  ¢ Leakage from anastomotic line  e latrogenic visceral trauma    ¢ Presence of fever is attributed to other sources of infection  such as urinary tract infection, thrombophlebitis, etc.   ¢ Presence of pain and tenderness is attributed to recent  laparotomy scar.   ¢ Tachypnoea, hypotension are attributed to pre-existing  medical conditions such as COPD, cardiac failure.   ¢ Administration of antibiotics would have reduced the  severity of peritonitis (masking effect) only to manifest as  septicaemia some time later.   e Surgical drains e Faecal matter  e Suture materials e Necrotic tissue  e Surgical clips e Talcum powder  e Implants    Fig. 27.50: Postoperative peritonitis due to anastomotic leak with  faecal peritonitis    ¢ Deterioration after 3—5 days of operation (the time when  anastomotic dehiscence takes place).   ¢ Free drainage of bile and faecal matter or pus from the  drain site or the main wound.    In 1973, when ultrasound facilities were not available, a  35-year-old male who had undergone appendicectomy for  a gangrenous appendix, was found to have high spiking  fever on the 3rd postoperative day. All possible causes of  postoperative fever including malaria were ruled out. On  the 10th postoperative day, the patient developed a purulent  discharge of 100 ml through the lower part of the main  wound following which he had a spontaneous and dramatic  recovery.    e Leak or abscess cavity is confirmed by abdominal ultra-  sound.    e Exploration (preparation similar to a routine laparotomy)  e Resection or resuturing, ileostomy, colostomy    e Once abscess is drained or leakage is prevented, recovery  is wonderful.   Fig. 27.53: Pus pouring out as soon as peritoneum is opened.  First step is to send a pus for culture sensitivity   Fig. 27.54: Postoperative peritonitis following due to leak following  right hemicolectomy for carcinoma caecum. There were 2 sites of  leak: 1. lleocolic anastomotic site, 2. proximal jejunum. Both were  exteriorized. You can see the wound left open with loose skin  sutures—they will be tied after 3—4 days. This is to prevent wound  infection (almost inevitable)   ¢ Anergy: Anergy (immunologic tolerance) refers to the  failure to mount a full immune response against a target.   ¢ Leakage of bile into the peritoneal cavity results 1n biliary  peritonitis.   ¢ It will be more obvious and can be detected early if a  drainage tube has been kept.    ¢ In majority of the cases, the local signs are confined to one  quadrant of the abdomen in the form of guarding and  rigidity.   ¢ There may be excoriation of the skin due to drainage of  bile to the exterior (Fig. 27.57)   ¢ However, when the anastomosis gives way, generalised  peritonitis can occur.   Fig. 27.55: Post-cholecystectomy bile leak due to injury to the  common bile duct. Majority of such leaks can be managed  conservatively. If the leak continues, ERCP and treat the cause  accordingly    Fig. 27.56: Biliary peritonitis following duodenal ulcer perforation.  It requires urgent laparotomy. Closure of the ulcer perforation,  peritoneal lavage followed by drainage tube and closure of the  incision    1. Most of the biliary fistulae heal within 2-3 weeks with  conservative line of treatment.    2. If it does not heal, re-exploration and resuturing or resection  has to be done.    3. Feeding jeyunostomy is a very useful procedure in all cases  of reperforation of sutured duodenal ulcers or difficult  duodenal ulcer closures (Fig. 27.58).    It should be remembered that feeding jejunostomy Is  temporary and Is get till it is assured that there is no leak from    the operated site. If there is no peritonitis, patient has passed  flatus and stools, jejunostomy tube is removed.   Feeding jejunostomy should be done carefully with proper  placement of catheter within the small bowel, fixing it firmly  both inside and outside the abdominal wall and fixing to the  bowel wall.    ¢ Primary variety is more common. Girls of 3—6 years of age  are usually affected. Infection spreads from female genital  tract through vagina.   ¢ In boys, blood spread can occur following upper respiratory  tract infection.   ¢ Bloody diarrhoea and frequency of micturition are  indicative of pelvic peritoneal inflammation.    Diagnosis  Aspiration of peritoneal fluid demonstrates high WBC count—  30,000/mm?. More than 90% are polymorphs.    Laparotomy and drainage of pus (odourless and sticky initially  and creamy or purulent in the later stages), to be followed by  appropriate antibiotics.    ¢ Infants and children less than 4 years are commonly  affected.    ¢ This occurs after delivery if proper aseptic precautions are  not taken. The incidence has come down tn the recent years.  Attempted abortions by using instruments which are not  sterile results in peritonitis. Most of the time, peritonitis 1s  confined to pelvis with paralytic ileus, mucous diarrhoea  and offensive lochia (Key Box 27.14). Late cases develop  generalised peritonitis, intra-abdominal abscess, intestinal  obstruction and infertility. Table 27.2 gives summary of  various types of peritonitis.   Tuberculous 20-40 years Pulmonary tuberculosis  Perforation peritonitis 20-40 years Hollow viscus   ABORTION PERITONITIS  e Instrumentation  e Puerperal sepsis  e Offensive lochia  e Pelvic peritonitis  e Infertility    As the name suggests, in this condition, there is no demon-  strable intra-abdominal disease responsible for peritonitis.    1. In infants: It is more common tn female children. Spread  is by haematogenous route. The causative organisms are  Streptococcus pneumoniae. It may follow respiratory tract  or urinary tract infection (Key Box 27.15).    2. In adults: Male alcoholic patients are commonly affected  followed by patients with chronic liver disease. Causative  organisms are E. coli, S. faecalis, etc.    ¢ Portal hypertension increases permeability of gut wall,  thus increasing bacterial migration. These bacteria which  colonise in the small bowel reach systemic circulation  because of shunting of blood around liver sinusoids.  Portal lymph also gets contaminated giving rise to  increased ascitic fluid.    Odourless, sticky, turbid pus with high-grade fever  Cloudy and fibrin flakes +    Clinical features  ¢ Dull aching pain in the abdomen with low-grade fever.    ¢ Rebound tenderness is present, bowel sounds are absent  or sluggish—abdominal distension    ¢ Septic shock is a late feature with a high mortality rate.    ¢ Laparoscopy may help to rule out intra-abdominal  emergencies such as perforations, etc.    ¢ Ultrasound can detect nature of the liver and amount of  fluid in the abdomen.    Treatment  ¢ Conservative treatment is followed, provided secondary  bacterial peritonitis is ruled out.    ¢ Broad-spectrum antibiotics such as aminoglycosides with  3rd generation cephalosporins is the ideal choice.  Metronidazole 1s always added.    ¢ If laparotomy is done, peritoneal wash or toilet is given.    ¢ Presents as abdominal pain, tenderness, pyrexia and  increased total WBC count.    ¢ Primary: Mesothelioma is the tumour to be remembered  It is more common from the pleura. In the abdomen, pelvi:  peritoneum is the common site.    e In this condition, peritoneal cavity is filled with mucoic  substance (jelly-like) brownish or yellowish.   Fig. 27.59: Rupture of mucocoele of the appendix (Courtesy:  Dr Stanley Mathew, FRCS, Professor, Department of Surgery,  KMC, Manipal)    ¢ The patients can present with slow, painless and progressive  abdominal distension. No shifting dullness.    ¢ Aggressive surgery is the main mode of treatment. Masses  of tumour tissue (jelly) should be resected or scooped out.  Thus debulking, bilateral oophorectomy, appendicectomy,  omentectomy are done.   ¢ Combination chemotherapy by using cisplatin and/or  intraperitoneal alkylating agents have been used.   Operation: Surgery is the main treatment  Mucinous cyst of appendix/ovary is the cause  Extraperitoneal spread never happens   ¢ This name is applied to an advanced stage of intra-  abdominal malignancy involving the entire peritoneal  cavity.    COMMON CAUSES  Carcinoma of the:  e Stomach, colon, pancreas  e Breast, ovary    ¢ Multiple firm to hard nodules on the visceral and parietal  peritoneum.   e Entire omentum will be studded with hard nodules. It is  called omentum cake (Fig. 27.60).   ¢ Low protein ascites is more vulnerable for risk of  developing peritonitis. Incidence of peritonitis in  malignant ascites cases is low because of increased  immunoglobulin levels in malignant ascites and  increased opsonic activity.    ¢ The most common differential diagnosis is tuberculosis  (peritoneal). These nodules are firm and greyish.   ¢ Acute pancreatitis with fat necrosis: This is due to calcium  soap. They are yellow and soft.    2. Tamoxifen is useful for ascites due to carcinoma of the  breast.    ¢ Talc, gauze, starch, etc. are causative factors.  ¢ It occurs many weeks after surgery.    ¢ Low-grade fever, weight loss, distension, crampy  abdominal pain are the features.    ¢ Laparoscopy is the key investigation, can visualise  granuloma and biopsy can be taken. Also, fluid can be  aspirated and sent for histopathology. High concentrations  of lymphocytes are present in both.    ¢ Intravenous prednisolone followed by oral prednisolone  for 2-3 weeks is given.   ¢ Cleaning the gloves by wiping before handling bowel,  prevents many cases of granulomatous peritonitis.   ¢ In the initial surgery all the foreign bodies including ova,  cysts of parasites, ascariasis, ingested food particles have  to be removed.   Tuberculous peritonitis: Here omentum is involved. It  becomes nodular because it 1s studded with tubercles.  Classically seen in children who are brought with abdominal  distension. On palpation, omentum Is felt as granular mass  in the upper abdomen which moves with respiration.  Laparoscopy, biopsy and antitubercular treatment is given  in Fig. 27261.    commonly result 1n metastasis and they give rise to omental  cake (Fig. 27.62).    . Tumour (cyst): Omentum 1s the site of omental cyst which  is a lymphatic cyst (Fig. 27.63). It 1s a slow-growing,  painless swelling in the upper abdomen. On examination,  the patient 1s a child or an adult, with a smooth, firm mass  In epigastrium which moves with respiration. Excision of  the lymphatic cyst 1s the treatment. Massive cyst can be  confused for ascites.   Fig. 27.61: Rolled Fig. 27.62: Omental Fig. 27.63: Omental  up omentum in cake in metastasis cyst (lymphatic)  tuberculosis    4. Torsion: It is a rare surgical emergency wherein torsion of  omentum occurs due to old adhesions or it is primary due  to lengthy mobile omentum. It produces symptoms/signs  similar to that of appendicitis. Laparotomy and excision of  gangrenous omentum have to be done.    5. Non-Hodgkin’s lymphoma can affect ometum and can give  rise to granularity (Figs 27.64 and 27.65).    ¢ Anatomy: Given in page 666  ¢ Mesenteric tear has been discussed in page 667  ¢ Mesenteric lymphadenitis has been discussed in page 672    ¢ It means increase in the mesenteric fat and it is a finding in  the multi-detector CT scan.   ¢ A few pathological conditions which may give rise to  this entity are: Acute pancreatitis, retroperitoneal  haemorrhage, malignancies.   ¢ Weber-Christian disease: Lipodystrophy and mesenteric  lipogranuloma are the features. In this condition, it should  be called mesenteric panniculitis. Findings include  inflammation of the mesentery, fibrosis, shortening and  ischaemia. It 1s difficult to treat.    ¢ These are congenital cysts, enterogenous or chylolymphatic.  It manifests in young children or during adolescence.  Typically, the cyst 1s located in the umbilical region which  moves at right angles to the direction of mesentery  (Fig. 27.66).   Fig. 27.66: Diagrammatic representation of mesenteric cyst and  its movements at right angles to the direction of mesentery   A. Chylolymphatic cyst is a lymphatic cyst arising from  mesentery of ileum. It is a thin-walled cyst with clear fluid  or chyle. It has a separate blood supply. Hence, enucleation  is the treatment without sacrificing the bowel.    B. Enterogenous cyst is a duplication cyst from the intestine  or due to diverticulum of the mesenteric border of the  intestine. It 1s thick walled and contains mucus. This cyst  1s treated by excision of cyst with bowel segment because  both share the same blood supply.   3. It is dull surrounded by  a zone of resonance |  and traversed by band    Complications  ¢ Torsion of the cyst resulting in acute abdominal pain.  ¢ Rupture of the cyst due to trauma  ¢ Haemorrhage into the cyst    Retroperitoneal space: The retroperitoneal space lies between  the peritoneum and the posterior parietal wall of the abdominal  cavity and extends from the diaphragm to the pelvic floor.  Superiorly—12th thoracic vertebra and lateral lumbocostal  arch.   Anteriorly—posterior parietal peritoneum  Posteriorly—tfascia overlying the quadratus lumborum and  psoas major muscles.   3. Digestive: Oesophagus (thoracic part), rectum (part of  middle third and lower third 1s extraperitoneal).   4. The head, neck, and body of the pancreas, the duodenum,  except for the proximal first segment, ascending and  descending portions of the colon.   1 Abdominal aorta  | 2 Inferior venacava  Right and left kidneys with ureters  Duodenum on the right side    Body and tail of pancreas across the  posterior abdominal wall towards the  left kidney   * This is one of a group of fibromatosis (other being =. One day I was urgently called to assist the urosurgeon who  Dupuytren’s contracture and Peyronie’s disease).    e¢ Other types of fibrosis such as mediastinal fibrosis,  sclerosing cholangitis may also be associated features. :   e It can present with lower back pain, renal failure,  hypertension, deep vein thrombosis and other obstructive  features.  See Key Box 27.20 and clinical notes.   A 76-year-old lady was admitted under urology for the  diagnosis of renal failure. An ultrasound abdomen showed  bilateral hydronephrosis. She was diagnosed to have  idiopathic retroperitoneal fibrosis and posted for  ureterolysis. To my surprise, when I explored, the patient  had carcinoma colon in three places (synchronous),  carcinoma caecum infiltrating right ureter, carcinoma  sigmoid colon infiltrating left ureter and carcinoma  transverse colon. She underwent total colectomy with  ileorectal anastomosis. Ureteric reimplantations were done  into bladder. She lived for 3 years and then succumbed due  to liver metastasis.   ¢ Very often, it is a painless, smooth, firm enlargement.  e May have minor degree of mobility.    Carcinoid  Remember as IDIOPATHIC ¢ Histology: These cystic structures may be lined by    ¢ Gross: These cysts are not connected to the kidney or  adrenal; usually filled with clear or straw-coloured fluid.    ¢ They can be unilocular or multilocular.  ¢ CT scan is required to differentiate it from hydronephrosis.  ¢ Excision 1s the treatment.    Diagnosis  ¢ Ultrasound or CT scan-guided aspiration  ¢ Culture of the pus and antibiotic sensitivity.   1. Primary psoas abscess: It is caused by haematogenous  spread of Staphylococcus aureus. Source may be occult—  tonsils, middle ear, etc. More common in children and  young adults. Poor nutrition, may be a contributing factor—  It is monomicrobial.   2. Secondary psoas abscess: Secondary to intestinal  perforation, e.g. Crohn’s disease. It is polymicrobial. Other  causes are given below.    Fever, flank pain, flexion of the hip joint are triad of psoas  abscess. Pain on extension confirms the diagnosis.    ¢ CT scan is the diagnostic test. Gas bubbles are diagnostic  of an abscess.   ¢ Treatment include percutaneous catheter drainage,  treatment of the source of infection with antibiotics.   ¢ If necrotic tissue does not drain well or if patient is not  improving, open drainage should be done.   3. Tuberculous spine: Lower thoracic (T10) and upper lumbar  Spine are commonly affected (Fig. 27.72).    ¢ Pain in the back (localised to lesion) or referred pain if  there is a collapse.   Fig. 27.72: Various sites of tubercular cold abscess arising from  tuberculosis of the spine    e Pus enters psoas sheath and tracks downwards and causes  mass in the iliac fossa.   ¢ Spine X-ray—AP and lateral view. The earliest sign is a  decrease in the intervertebral space.    Definition  ¢ The term retroperitoneal tumour (RPT) is usually confined  to primary tumours arising in other tissues in this region,    e.g. muscle, fat, lymph nodes, nerves (Tables 27.3 to 27.5).  (Jean Lobstein, French pathologist and surgeon in ]1829—  coined the term retroperitoneal tumour.)   ¢ Other tissues refer to—tumours of retroperitoneal organs  such as kidneys, ureters, pancreas and adrenals are  conventionally not included in retroperitoneal tumours.    Fatty tissue origin Lipoma/liposarcoma  Smooth muscle origin Leiomyoma/leiomyosarcoma  Connective tissue origin Fibroma/fibrosarcoma   Age: Most occur between the sixth and seventh decade of  life.   Because of their location, these lesions usually demonstrate  indolent growth and present as relatively large lesions.  Their proximity to vital structures (especially vascular)  makes resection difficult.    I.  2. History of radiation: Accidental or given for lymphoma.  as    Idiopathic: The actual cause is not known  Exposure to vinyl chloride/thorium dioxide    Abdominal mass (80%): Slow growing painless mass 1s  the most common presentation. Typically confined to one  side rather than centre.   Nausea, vomiting and weight loss in about 20 to 30% of  patients.   Compressive symptoms: Abdominal pain, constipation,  recent haemorrhoids, haematochezia can occur. Back pain  and sciatica (30%) are common and are confused for spine  pathology.    Unilateral lower extremity oedema and pressure symptom  including secondary varicosities are common.  Acute urine retention, dysuria and increased frequency cal  occur due to compression on urinary bladder    Please note: When you suspect anyone of these as differential  diagnosis a CT-guided biopsy may be required which alters  the management.    Preoperative planning to assess the extent of the disease is  essential because successful surgery is defined by complete  excision of the mass with adequate margins of normal tissue.  1. CBP  2. LFT: Increase alkaline phosphatase may indicate  secondaries.  . RFT: Compressive uropathy with high urea and creatinine.  . Tumour markers: AFP, beta-HCG—germ cell tumour.  5. LDH in lymphoma/GCT: LDH levels indicate tumour  burden and growth rate.  6. CT Scan (Key Box 27.22)  ¢ Delineate the anatomic limits of the lesion.  ¢ Vascular involvement—vena cava, aorta, renal vessels.  ¢ Assess the integrity and function of adjacent organs—  renal function is one of the important advantage of CT  scan.  ¢ Visceral metastases +/-. If present, it is inoperable but still  worth trying a resection after giving neoadjuvant therapy.  ¢ Para-aortic, iliac, mesenteric lymphadenopathy.  ¢ Axial skeleton and renal involvement (Key Box 27.22).    CT SCAN: TYPICAL FINDINGS  e Lipoma: Homogenous fatty density  e Malignant fibrous histiocytoma: Calcifications  e Neurofibroma: Homogenous low density  e Teratoma: Mixed components  e Paraganglioma: Para-aortic location  e Neuroblastoma: Calcified tumour, usually in children  e Leiomyosarcoma: Large areas of necrosis  e Liposarcoma: Heterogenous fatty density  e Hemangiopericytoma: Hypervascularity    7. CT-guided core biopsy  ¢ Reserved for cases in which a diagnosis will change  therapy, such as the need for neoadjuvant chemotherapy  for  1. GIST—imatinib mesylate  2. Germ cell tumours  3. Lymphomas  8. Laparoscopic biopsy/retroperitoneoscopy  ¢ Equivocal history and diagnostic dilemma  ¢ Unusual appearance of the mass  ¢ Unresectable tumour  e Distant metastasis    Treatment: Surgery 1s the main modality and the most  effective modality of the treatment. It can be curative if R-0  resection is achieved. Chemotherapy, radiotherapy are  complementary to surgery. A few cases are also managed by  neoadjuvant therapy.    Principles: Extirpative surgery is the principal and most  effective form of therapy for primary retroperitoneal tumours.  Tumour histology, tumour size, or patient age not significant  factors in survival in multiple-variable analysis. Therefore  carefully planned and skillfully executed surgical therapy is  critical for any chance at long-term success.    High local recurrence rate and eventual mortality from this  disease has prompted the exploration of adjuvant therapeutic  modalities. Postoperative radiation increased toxicity to  surrounding structures.   2. Generous incision should be given to get into good access  to the tumour   3. Intra-abdominal adhesions should be released to separate  the tumour. Should use of sharp, curved Mayo scissors  for meticulous dissection.   4. Should never do enterotomy (accidental) because an  enterotomy and subsequent fistula formation can cause  major morbidity.   6. Should preserve important organs such as kidney, ureter  colon, etc.   7. Should resect the organs if found infiltrated and if R-O  resection is possible.   8. Should do a centripetal dissection. It allows one to  dissect those areas that are amenable to dissection.   9. Should use surgical clips, should be placed to mark the  periphery of surgical field and other relevant structures to  help guide potential future radiation therapy.    ¢ MAID regimen: Mesna, adriamycin (doxorubicin),  ifosfamide, and dacarbazine has been successful in  neoadjuvant programmes for sarcomas of the extremities  compared with historical controls. Yet less data are available  with regard to other soft tissue sites. Neoadjuvant therapy  for retroperitoneal sarcoma.    ¢ The viscera are often displaced by the tumour volume and  a lack of surgical adhesions further reduces dose to the  bowel.    These are pathological variants. It 1s difficult to consider them  as a diagnosis on clinical grounds. However, more common  ones have to be considered first such as liposarcoma. Patients  with von Recklinghausen’s disease may have neural tumours.  A few differential diagnosis are given below.   4. Dedifferentiated ]iposarcoma—the rate of metastasis  depends on the degree of tumour differentiation, with  nearly 90% of poorly differentiated tumours meta-  stasizing.    ¢ Pathology: The key feature of a liposarcoma 1s the lipoblast,  which 1s essentially an immature fat cell. LIPOBLAST  have multiple fat vacuole which compress the nucleus,  creating a scalloped appearance.   ¢ Site of origin (soft tissue, vascular or superficial), although  many of the soft tissue lesions are believed to originate  from smaller blood vessels.   ¢ Immunohistochemistry stain for smooth muscle myosin,  vimentin, and actin and less often for desmin. (Leiomyomas  stain positive for desmin, which separates them from their  malignant counterpart.)   ¢ Storiform-pleomorphic (40-60%) and myxoid type (25%)  are subtypes. Other types being giant cell type and  inflammatory.    ¢ These are the tumours arising from totipotential cells. Thus,  they can have ectoderm, mesoderm or endoderm elements.   ¢ Malignant mature cystic teratomas (0.2 to 2% of cases) have  the potential to metastasise to sites such as the  retroperitoneal lymph nodes and lung parenchyma.    ¢ Majority has mutations in NF2 gene.  ¢ Majority are sporadic tumours.    ¢ A minority (10%) are associated with syndromes such as  neurofibromatosis type 2, schwannomatosis and multiple  meningiomas.   ¢ Hallmark of schwannoma 1s alternating areas of cellular  (Antoni A) and hypocellular (Anton! B) areas.   ¢ Retroperitoneal tumours are larger and often show  degenerative changes such as cystic change, haemorrhage  and calcifications.    e All topics have been updated with flowcharts and  coloured pictures.    e Retroperitoneal mass/sarcoma and abdominal  compartment syndrome are discussed in more detail.   e Misty mesentery valentino syndrome and mesenteric  cyst have been added.   1. One need not close peritoneal layer after laparotomy  because:  A. The peritoneum can get stuck to the bowel  B. Flattened mesothelial cells heal within a few hours  C. The peritoneum tears when closure 1s attempted  D. It is very painful postoperatively    2. Peritoneum can be used for dialysis because:  A. It is close to kidney  B. It 1s faster than haemodialysis  C. Capillaries and lymphatics between two layers of  peritoneum help in absorption and exudation  D. It covers entire abdomen    3. Which of the following is an example of primary  peritonitis?  A. Tuberculous peritonitis  B. Perforation peritonitis  C. Postoperative peritonitis  D. Parturition peritonitis    4. Which of the following organisms are most commonly  involved in secondary peritonitis?  A. Enterococci  B. Streptococc!  C. Staphylococc!  D. Pneumococc!    . Abdominal tap is done in peritonitis for all of the    History of discharge per rectum for the first time in a  patient who is recovering from peritonitis suggests:  A. Anal prolapse B. Pelvic abscess   D. Right paracolic gutter is large and colophrenic ligament  is absent on the right side    Nonoperative treatment for peritonitis may be followed  in the following except:    percutaneous drainage of subphrenic abscess:  A. Pigtail catheter B. Trocar catheter  C. Sump catheter D. Foley’s catheter    Presence of sunken eyes, pale and pinched face, dry  cracked tongue, cold perspiration and cyanosis are all  typical features of:  A. Hippocratic facies  C. Marshall hall facies    Intra-abdominal pressure exceeds _emH,O A. Common in women  in abdominal compartment syndrome. B. Ovary is the main source  A.15 B. 25 C. Surgery cannot cure the disease  C..85 D. 45 D. Chemotherapy is also used  ANSWERS  1B 2C 3 A 4A & © 6 |D 7A 8 B 9 3D 10 B  Th 12 C 13 D 14 D 15 A 14 'C 17 A 18 D 19 D 20 B   Abdominal tuberculosis  Tuberculous peritonitis  Glandular tuberculosis  Intestinal tuberculosis  Inflammatory bowel diseases  lleostomy    Truly speaking small intestines extends from pylorus to ileo-  caecal junction. However, for all practical purposes it 1s  discussed as starting from duodenojejunal! flexure till caecum.  Small intestines play an important role not only in the  transfer of food contents distally but in the digestion,  absorption and secretion of the contents. Being the central  portion of the GI tract with long length, many diseases affect  the intestine. Surgically important topics have been covered  in this chapter.   3/5 ileum. It 1s about 6 metres in length.  ¢ Small intestine starts at duodenojejunal flexure just to the   In cases of intestinal obstruction, trace up to ileocaecal  junction. If caecum is distended, it is a case of large bowel  obstruction. If caecum is collapsed, it 1s a case of small bowel  obstruction.   e Jejunum resides in the left side of the peritoneal cavity and  ileum on the right side.    Jejunum lleum  e Length 2/5 3/5  e Diameter Wider (2-4 cm) Less (2-3 cm)  e Wall Thick and double Thin  (mucous membrane  can be felt)  e Colour Deep red Pale pink  e Peyer’s Very, very less More  patch  e Blood Long and few Short and  supply vasa rectae (1 or 2) numerous (5 or 6)  e Mesentery Transparent, less fat More fat    ¢ Superior mesenteric artery is the artery of the midgut  which supplies the entire midgut (entire small intestine).  Jejunal arteries are end-arteries.   ¢ Mesenteric border of the intestine gets more blood supply  when compared to anti-mesenteric border. Hence in cases  of diminished blood supply, anti-mesenteric border become  ischaemic first.   A. Mesenteric cyst moves at right angles to the direction  of the mesentery.   B. Mesenteric lymph nodes can be clinically palpable as  a nodular or a smooth mass.   C. Horizontal tear in the mesentery causes more gangrene  of the bowel than vertical tear (Fig. 28.2).    Basic unit of small bowel mucosa is the villus, which is a  finger-like projection. Each villus is covered with tall  columnar epithelium.   Goblet cells, Paneth cells and endocrine cells are seen in  the crypts.    (enterochromaffin cells) have cytoplasmic granules which  secrete 5-hydroxytryptamine, neurotensin, glucagon and  motilin. Importantly, mucosal cell mediated immunity 1s  brought by mucosal T lymphocytes.    Motility: Two types of muscle contractions occur—one  which does not propagate—it exposes the food contents to  the absorptive surface for a longer time by causing  segmentation thus better absorption of the food. Another  type is peristaltic which propagates the food contents.  Control of peristalsis is done by myenteric plexus. Time  taken by the solid food contents to reach from mouth to  colon is about 4 hours (Key Box 28.2).   Absorption and digestion: Except calcium and iron almost  everything is absorbed in the small intestines. To give a  few examples: out of 6—10 litres of water, almost 80% water  is absorbed in the small intestine and only 10-20% 1s  discharged into colon. Thus in cases of terminal ileal  obstruction, about 8—10 litres of fluid accumulate resulting  in gross distension of the abdomen and dehydration.  Carbohydrates and fat are mainly absorbed in duodenum  and proximal jejunum. Proteins require pancreatic enzymes.  Hence, they are broken down in the jejunum into amino  acids and peptides. Conjugated bile acids are absorbed in  the terminal ileum wherein enterohepatic circulation takes  place and again they are secreted in the bile. Thus in ileal  resections or diseases like Crohn’s disease, more amount  of bile acids enter colon resulting in diarrhoea due to  increased secretion of water and electrolytes.    FUNCTIONS OF THE SMALL INTESTINE  Digestion and absorption  Synthesis of lipoproteins  Secretion of regulatory peptides  — Secretin  — Cholecystokinin  — Somatostatin  — VIP  Immune function: Production of immunoglobulins (IgA). The  B cells and T cells help in phagocytosis and secretion of  cytokines.   ¢ Abdominal tuberculosis which includes intestinal,  mesenteric and peritoneal tuberculosis. We should realise  that all three types are very much connected to each other  in one way or other and hence are discussed together.   ¢ Inflammatory bowel disease includes ulcerative colitis and  Crohn’s, both are important health problems in the Western  world. Other inflammatory conditions include enteric fever  and its manifestations, intestinal amoebiasis, HIV associated  enteropathy and radiation-induced enteropathy.   ¢ Small intestinal obstruction, Meckel’s diverticulum,  superior mesenteric vascular occlusion and necrotising  enterocolitis have been discussed in Chapter 30 on intestinal  obstruction.    Abdominal tuberculosis 1s a common extrapulmonary mani-  festation of tuberculosis. Incidence in the West has increased  due to immigrant population and increased incidence of HIV  infections. In India, extrapulmonary TB Is also showing re-  emergence due to incomplete treatment and occurrence of  multidrug-resistant strains. Hence, it is a major health problem.   ¢ The term abdominal tuberculosis includes tuberculous  infection of gastrointestinal tract, mesenteric lymph nodes,  peritoneum, omentum and solid organs related to  gastrointestinal tract such as liver and spleen.    1. Intestinal tuberculosis 1s caused by Mycobacterium tuber-  culosis from swallowed sputum (pulmonary tuberculosis)  or milk (milk-borne infection—Mycobacterium bovis).  From intestinal tuberculosis, mesenteric nodes get involved  and later, the peritoneum can get involved.   2. Blood spread: Infection from pulmonary tuberculosis can  spread through blood during bacteraemic phase.   VARIOUS FORMS OF ABDOMINAL TB  |. Peritoneal tuberculosis: Acute, chronic  A. Chronic forms   1. Ascitic type (wet)  e Generalised  e Localised  2. Fibrous type (dry)  e Adhesive, plastic  e Miliary nodule type  B. Tuberculosis of peritoneal folds  1. Mesenteric adenitis  2. Mesenteric cysts/abscesses  3. Bowel adhesion  l!. Gastrointestinal  1. Ulcerative  2. Hyperplastic  3. Sclerotic/plastic  lll. TB of solid viscera  1. Liver  2. Spleen    4. Genitourinary tuberculosis: From here, cephalad spread  occurs and thus, peritoneum gets affected.   5. From bile: Granuloma in liver. Bacilli are excreted in bile.  Pathology and pathogenesis (Fig. 28.3).    It is important to realise that there are so many investigations  for abdominal tuberculosis. Although it is essential to have  a theoretical knowledge about all these investigation, all  of them need not be done during clinical management.   * To give an example: If chest X-ray and sputum AFB are  positive, one should start ATT that need not do costly  investigations such as CT scan or even diagnostic  laparoscopy, etc. Investigations done by the clinician should  be complementary to each other.   1. Complete blood picture (CBP) which include Hb%,  TC, DC and ESR. Haemoglobin may be low indicating  anaemia. Anaemia has to be corrected before surgery.   2. ESR will help in equivocal cases. High values and the  clinical situation may force the clinician to start anti-  tuberculous treatment in selected patients. However, with  treatment, if ESR comes down and patient 1s  symptomatically better with weight gain, settling fever,  improving appetite, 1t suggests tubercular pathology.   3. Sputum AFB (acid fast bacilli): Demonstrated by Zeihl-  Neelsen method. Many patients with abdominal  tuberculosis will not have pulmonary tuberculosis.  However, if sputum 1s present, it must be tested for AFB   4. Chest X-ray may suggest tuberculosis in the form of  cavity, calcification, etc.    Mantoux test is nonspecific but a strong ulcerated  Mantoux test result suggests tuberculosis.  . Ultrasound, being a noninvasive investigation 1s an  imaging of choice.  ¢ Ascites can be demonstrated and the aspirated fluid  is sent for analysis  ¢ Focal ascites between loops of bowel—Club  sandwich sign may be seen  ¢ Enlargement of mesenteric lymph nodes (common)  and retroperitoneal nodes (uncommon) can be  detected  ¢ Dilated loops and sometimes peritoneal tubercles are  seen as echo-poor shadows  ¢ Thickening of omentum, mesentery, peritoneum can  be found out. (However ultrasound is not the best  investigation to detect these findings).  ¢ Pseudokidney sign: Pulled caecum identified in the  right hypochondrium  ¢ Hepatosplenomegaly may be present  . Ascitic fluid analysis:  ¢ Ultrasound guided fluid is aspirated and about  20--40 ml is sent for analysis (Key Box 28.4).  . CECT: Contrast enhanced CT scan of the abdomen:  ¢ CT scan is objective. All the findings which can be  detected by ultrasound can be confirmed by CT scan.  Addition of the contrast 1s definitely more superior  in detecting strictures, dilatations, perforations, and  more importantly loculated ascites and intra-abdominal   e Bacterial isolation and culture is possible in 20-45% of  patients.    collections. CT guided biopsy can be done. If dis-  tension or matting of loops and adhesions are present,  it is not safe.    9. Barium studies: These are not done routinely. If  diagnosis is possible by the various investigations  mentioned above, there is no necessity to do them. In  fact it can harm the patient by precipitating obstruction  and barium peritonitis if there 1s a perforation.   e Narrowing of terminal ileum (Fleischner’s sign),  Fibrotic terminal ileum opening into the contracted  caecum (Stierlin’s sign)   ¢ Barium enema: Pulled up caecum, normal acute  ileocaecal angle becomes obtuse or sometimes  straightening of the ileocaecal angle.   10. Endoscopy:  ¢ Upper gastroduodenoscopy may detect tubercles in the  stomach or duodenum — rare  ¢ Push enteroscopy: Ulcers in the proximal jejunum can  be detected and biopsy can be taken—chances of  perforation are high.  ¢ Colonoscopy can detect nodular lesions, ulcerations in    the colon—caecum and terminal ileum (last 10 cm of Fig. 28.4: Various sites from where laparoscopic biopsy can be  ileum should be entered and biopsy should be taken). taken in suspected case of abdominal tuberculosis. Biopsy from  11. Laparoscopy: This is a diagnostic investigation as it peritoneal surface and omentum is safe. Otherwise lymph node   gives the tissue diagnosis (Figs 28.4 and 28.5). One can biopsy can be done  also evaluate all possible viscera, peritoneum, omentum  and pelvic organs. Biopsy is possible under direct  vision. Findings may include the following (Key Box 28.5).   ¢ Intestines: Matting of the loops of bowel, adhesions,  bands, strictures, dilatations, perforations of bowel    12. Polymerase chain reaction (PCR):  ¢ It 1s a technique used in medical and biological research    Laparoscopy can also be therapeutic, if a stricture is ne  identified, the diseased loop is isolated, brought out and .  resection and anastomosis/stricturoplasty done.    ¢ One can do functional analysis of genes; useful in the  diagnosis of hereditary diseases;   ¢ Helps in detection and diagnosis of infectious diseases  such as tuberculosis   1. Routine in all cases: CBP including ESR, chest X-ray and  sputum for AFB. Lymph node biopsy—If lymph nodes are  palpable (peripheral—cervical, axillary preferable —    inguinal last choice). Advantage being lymph node biopsy  can be done under local anaesthesia. If it is positive, all  other invasive and costly investigations can be avoided (see  clinical notes below). Imaging: Ultrasound examination  followed by guided ascitic fluid analysis has become a  routine imaging test. It may not be diagnostic but since it  has more advantages than any side-effects, it can be done.    barium studies, colonoscopy. If any one of these gives a  diagnosis, treatment is started. In the presence of obstruc-  tion, avoid barium studies, enteroscopy and colonoscopy.  The decision 1s made on clinical grounds to proceed with  laparotomy.    is the investigation of choice—often done as a last choice  when all investigations are equivocal or have failed to give  a diagnosis.    ¢ In most of the cases, tuberculous peritonitis results from  reactivation of latent primary peritoneal focus.    ¢ [tis common in children and young adults. The child is  brought to the hospital with abdominal distension.   ¢ Omentum can be felt as arolled up transverse mass, which  is nodular due to extensive fibrosis. Abdomen has a  doughy feel with fluid giving rise to shifting dullness.   ¢ Details are given in medicine textbooks. However, 4 drug  regimen for 2 months followed by 2 drug regimen for  4 months is recommended as a first line of treatment.   ¢ First line of drugs include INH, rifampicin, ethambutol and  pyrazinamide given for 2 months. This is followed by  rifampicin and INH for 4 months. Refractory cases are  treated by kanamycin, ofloxacin, ciprofloxacin, amikacin,  etc. see clinical notes.    ¢ Umbilical hernia or congenital hydrocoele appears in  children due to increased intra-abdominal pressure.   ¢ In this variety, ascitic fluid is present in one quadrant of  the abdomen which is sealed off by matted intestinal  coils surrounded by omentum. It gives rise to localised  swelling. These patients have no shifting dullness.   ¢ Differential diagnosis: Other cystic swellings in the  abdomen such as pseudocyst of the pancreas, mesenteric  cyst, retroperitoneal cyst.    e e e > ,  SE a.  CAUSES OF EXUDATIVE  ASCITES—INCREASE IN PROTEIN    It can be of two types: Acute and chronic. Basically, it produces  the following pathological changes:   An 18-year-old girl presented with colicky abdominal pain,  low grade fever, gross emaciation, etc. Barium meal follow  through demonstrated strictures of the small bowel.  Laparotomy was done. There were dense adhesions.  Minimal dissection resulted in two openings in the terminal  ileum. Peritoneum was studded with tubercles from which  biopsy was taken. Abdomen was closed with a drainage  tube. Postoperatively, the patient was given antituberculous  treatment. A fter three months, the patient came to the  outpatient department and had put on 14 kg weight. She  was asymptomatic. This case history illustrates the  presence of tuberculosis as a major cause of illness in India.  It also highlights the importance of antituberculous  treatment and recovery.    ¢ In this variety, there is no ascites but there 1s extensive  fibrosis which results in dense adhesions between the  coils of intestines. Intestines are matted, distended and  not able to empty properly due to adhesions and bands.  It is associated with strictures.   ¢ This gives rise to blind loop with steatorrhoea and  emaciation.   ¢ Usually, it presents with intestinal obstruction at a later  date due to fibrous band which needs to be divided to  relieve the obstruction. In some occasions, it is not possible  to enter the peritoneal cavity, due to dense adhesions.   ¢ It is not uncommon to create openings in the bowel at  laparotomy and end with a helpless situation wherein  one will not be able to close the perforation. The net  result is fistula formation.   ¢ Seen in females as a complication of genitourinary  tuberculosis (tuberculous salpingitis).   ¢ It presents with acute peritonitis at laparotomy, the  peritoneal cavity is seen studded with tubercles, cold  abscesses and pus.   ¢ Laparotomy, drainage of pus, followed by antitubercu-  lous treatment is the choice of therapy.   ¢ It carries poor prognosis because of complications such  as toxaemia and faecal fistula formation.   ¢ Tuberculous peritonitis can be associated with infections  of pleural space and pericardial space (effusion). It is  called polyserositis syndrome.   1. As acalcified lesion (Fig. 28.14) along the line of mesentery,  which extends from L2 vertebra, at the left of vertebral  column to the right sacroiliac joint. In 50% of cases, there  is no active infection but in the remaining, there is infection.  If the symptoms are that of tuberculosis, antituberculous  treatment should be given. The shadow caused by lymph  nodes are round to oval, mottled and may be regular or   e Pain in the right iliac fossa, vomiting, fever, rigidity can  be present.   ¢ On palpation, tender mass of swollen lymph nodes can  be felt in the right iliac fossa.    ¢ Laparotomy, appendicectomy and biopsy of the lymph  node is the procedure of choice.    as failure to thrive. Fever, loss of weight, loss of appetite,  emaciation and pallor are present. Abdomen Is protuberant.  On deep palpation, nodes can be felt in the right iliac fossa.  These nodes have to be differentiated from nodes that  enlarge due to lymphoma.    ¢ This is due to caseation of mesenteric lymph nodes  confined within two leaves of mesentery.   ¢ Due to adhesions, intestines can get kinked or twisted  causing intestinal obstruction.   2. Presence of alkaline media favours the growth of the  organisms.   ¢ Abdominal pain: It 1s the most common symptom. It can  be a dull, vague pain or colicky pain (stricture) which  increases after taking food or relieved by vomiting.   ¢ Diarrhoea: Watery, small quantity, abnormally foul  smelling. It may alternate with constipation.   ¢ Nonspecific symptoms such as flatulence, noisy sounds  in the abdomen (borborygmi) are not uncommon.   ¢ Weight loss is very common. Anorexia, tiredness, pallor  may be the presenting features.   ¢ Rolled up omentum, mass in the right iliac fossa or lumbar  region, loculated ascites, etc. are other features (Fig. 28.21).    1. No evidence of intestinal obstruction: Antituberculous  treatment.  2. With obstruction (stricture) (Figs 28.22 and 28.23)   A. Solitary stricture: It is best treated by stricturoplasty  by incising the stricture longitudinally and suturing it  transversely (Fig. 28.28).   B. Multiple strictures at long intervals: Stricturoplasty 1s  the ideal treatment.   C. Multiple strictures within a short segment. Resection  is the ideal treatment (Fig. 28.30).    Stricture within 10 cm from the ileocaecal junction ts  best resected.    1. Aetiology Secondary to pulmonary TB. Occurs  due to swallowing of TB bacilli  (Mycobacterium TB)    ileum with/without involvement of  lymph nodes. Ulcers are transverse.  Serosa 1s reddened and oedematous.   6. Clinical features Symptoms of pulmonary TB, blood  and mucus in stool resulting in gross  emaciation and cachexia. Diarrhoea is  also a feature.   ¢ Chronic: Healing of ulcer results  in stricture of terminal ileum and  subacute intestinal obstruction.   It is low grade, chronic continuous inflammation  involving ileocaecal region resulting in cicatrising  granuloma in right iliac fossa (mass in right   Abdominal pain and diarrhoea may be the initial  symptoms for a long time and later fever, weight loss  and subacute intestinal obstruction occur.    Nodular, mobile, firm mass in the right iliac  fossa which later produces subacute  intestinal obstruction.    Barium enema can demonstrate (1) contracted caecum,  (11) pulled up caecum (subhepatic), (111) luminal  obstruction, and (iv) obtuse ileocaecal angle.  Negative    in apatient who underwent appendectomy to ascites  for pain in the right iliac fossa. The diag-   Fig. 28.27: Colonoscopy showing nodular leison in the ascending  Fig. 28.26: Plain X-ray showing dilated air fluid levels colon. Biopsy proved tuberculosis    3. Surgical treatment of hyperplastic tuberculosis: Limited  resection 1s the treatment of choice. It includes removal of  terminal 8—10 cm of the diseased ileum, caecum with  appendix, diseased portion of the ascending colon, followed  by ileocolic anastomosis. All these cases have to be given  antituberculous treatment for a period of 9-12 months.  Nutritional supplementation to improve albumin and  haemoglobin levels and if necessary, blood transfusion  before and after surgery help in smooth recovery in the  postoperative period (Fig. 28.29).   Fig. 28.29: Limited colectomy followed by ileocolic anastomo-  sis. (Courtesy: Miss Vidushi Kapil, MBBS student, KMC, Manipal)   2. Perforation: It is not uncommon. Carries 6—8% mortality  rate specially in late cases with peritonitis.   3. Malnutrition: Diarrhoea, loose stools, blind loop  syndromes contribute for malnutrition    . Faecal fistula is usually due to operated cases of intestinal    tuberculosis by resection and anastomosis. In such cases,  anastomotic dehiscence may result in faecal fistula. The  faecal fistula is not due to a direct internal involvement of  viscera unlike in Crohn’s disease.    tuberculosis is not common. Most of such cases have  fulminant pulmonary tuberculosis. This happens tn  untreated cases and immunocompromised cases.    These are the diseases involving small and large bowel, of  unknown aetiology, characterised by multiple ulcerations in  the bowel, clinically manifesting as blood and mucus in stools.  Ulcerative colitis and Crohn’s disease (regional enteritis) are  included under this heading. However, both these diseases are  uncommon tn India. Hence, amoebic dysentery, intestinal TB,  enteric fever with intestinal manifestations can also be included  in this. Both diseases can present has acute abdomen with  intestinal obstruction and complications such as perforation  toxaemia, etc. Also both are premalignant conditions for  carcinoma colon.    1. Autoimmune factor: Even though exact mechanism of  ulcerative colitis 1s not clear, there are some factors which  may point out to autoimmune reaction. They are:    ¢ Presence of cytotoxic T lymphocytes against colonic  epithelial cells in the lamina propria of the bowel.    ¢ Whatever be the immune mechanisms, activation of  inflammatory mediators such as cytokines, growth  factors and arachidonic acids takes place and they are  responsible for the disease.    2. Dysfunctional immunoregulation in the intestinal wall  results in inappropriate production of cytokines. This  creates an imbalance between various interleukins resulting  in inflammatory changes.    3. Psychosomatic and personality factors: Ulcerative colitis  is more common in western women. Emotional stress,  family stress, stress from divorce are the contributing  factors.    ¢ Westernisation of the diet which 1s rich in red meat has  been blamed. Vegetarian diet is supposed to protect the  colonic mucosa.    ¢ Allergy to milk protein is responsible for ulcerative  colitis in a few patients.    §. Defective mucin production and a defective mucosal  immunological reaction is considered as a chief factor  responsible for ulcerative colitis.    Appendicectomy and smoking have been protective  factors for development of ulcerative colitis.    6. Genetic: 15% of patients have first degree relatives with  ulcerative colitis.    ¢ The disease always starts in the rectum and spreads in a  backward manner, thus involving the entire colon in  majority of cases. In 5% of cases, terminal ileum can also  be involved—back wash ileitis.   e As the disease progresses, inflammation spreads into the  submucosa of the colon. Attempt at healing may produce  pseudopolyp. There are areas of epithelial hypertrophy in  between the ulcers, resembling polyp. Healing with fibrosis  results in anarrow, contracted colon, called pipe stem colon.   e Microscopy: Pus (abscess) in the crypts and pus cells  (inflammatory cells) in the lamina propria are typical of  ulcerative colitis.   Age: Common age of presentation is 3rd decade followed  by 4th and 2nd decades.   The disease is characterised by passage of 15-20 stools  per day and contains blood and mucus. Sometimes, it may  be watery diarrhoea (Key Box 28.8). As the rectum loses  elasticity and lumen collapses, tenesmus occurs.   Relapses and remissions are common and are related to  emotional disturbances.    PATHOLOGY OF ULCERATIVE COLITIS  Pinpoint ulcers  Pseudopolyposis  Pipe stem colon  Pus cells    e Mild colitis: Refers to < 4 stools/day without systemic signs  and symptoms.    e Moderate colitis: Refers to < 6 stools/day without systemic  toxicity.   e Severe colitis: Refers to > 6 stools/day with systemic  toxicity—fever over 37.5°C, tachycardia more than 90/min,  hypoalbuminaemia less than 3 g/dl, weight loss more than  3 kg   ¢ Acute fulminating attack is associated with high grade fever,  bloody dysentery, distension and tenderness all over the  abdomen with profound weakness. Hypokalaemia, acidosis,  anaemia and shock are the other features.    1. Proctitis: Inabout 20-25% of the patients, the disease involves  only rectum. In such patients, stools are semisolid because  of absorption of water by normal colon. Also, the intensity  of the disease is not severe and risk of cancer is 2—5%.   2. Left-sided colitis: It is found in 15% of patients. It presents  as severe recurrent attacks of diarrhoea with blood in stools,  without systemic toxicity (Fig. 28.31A).   3. Total proctocolitis: It is seen in about 25% of the patients.  Severe bloody diarrhoea and hypoproteinaemia are its   1. Toxic megacolon: It is an abdominal emergency encoun-  tered with fulminating colitis. Severe abdominal pain and   tendemess, toxaemia, high fever, tachycardia and leucocytosis © Barium enema  are the features. Plain X-ray abdomen which shows colon ¢ Opiates, antidiarrhoeal drugs, anticholinergic agents  with a diameter more than 6 cm gives the diagnosis. It © Not known    requires emergency treatment by laparotomy and colec-  tomy. Supportive treatment and intravenous corticosteroids  are necessary (Key Boxes 28.9 and 28.10, Figs 28.32 and  28.33).    TOXIC MEGACOLON—CAUSES  e Ulcerative colitis, salmonella colitis  ¢ Pseudomembranous colitis, amoebic colitis   Fig. 28.31A: Sharp demar- Fig. 28.31B: Fully mobilised  cation at the splenic flexure colon. Observe loss of haustra-  may be due to an incomplete _ tions. Total proctocolectomy with  marginal artery ending at ileal pouch to anal anastomosis  point of demarcation. Case of was done—total proctocolitis Fig. 28.33: Total colectomy for toxic megacolon specimen showing  left-sided colitis pseudopolyposis    . Massive haemorrhage per rectum is uncommon. It is  treated by blood transfusion.  . Perforation is treated as peritonitis with resection of colon.  Mortality rate is around 25-50%. Steroids may mask the  symptoms. In an emergency situation, saving life is more  important. Hence, resection followed by ileostomy should  be done.  . Carcinoma of the colon (Key Box 28.11)  ¢ Overall incidence 1s 3% when the disease has been  present for 15 years.   e At the end of 25 years, the incidence may be around  20%.   ¢ Hence, routine sigmoidoscopy and biopsy have to be  done when the disease is present for more than 10 years  and if it shows epithelial dysplasia, it should be  considered as premalignant.   ¢ Incidence is more in total proctocolitis and when the  disease has started in the early age group.   sclerosing cholangitis is also found in many cases. Fatty  acid infiltration 1s seen in 40% of cases. It is reversible  after control of disease.   ¢ Incidence of bile duct cancer is high in these patients.    Peripheral arthritis and ankylosing spondylitis are the  two most common extraintestinal manifestations.    Crohn’s disease should be ruled out first. The differences  between the two inflammatory bowel diseases have been  discussed at the end of the chapter. In general, diarrhoea   AND COLORECTAL CANCER (CRC)  e CRC is more aggressive  e Multicentric and synchronous  e UC patients with primary sclerosing cholangitis (PSC) have   e More advanced stage at the time of presentation  e Risk increases with duration of the disease    e Malignancy develops on a background of dysplasia [dysplasia  associated lesion or mass (DAL-M)]    and bleeding are more common with ulcerative colitis than  Crohn’s disease.   Dysenteries: Bacillary dysenteries, Shigellosis, Salmo-  nellosis, Amoebiasis and other dysenteries have to be kept  in mind specially in developing countries including  tuberculosis which has been already discussed.  Diverticular disease of the colon: However, when  complications including bleeding occur, diverticular disease  of the colon and in cases of perforations, malignant  perforation must be considered as a differential diagnosis.   ¢ Most common cause of infective colitis in UK is  Campylobacterium.    ¢ Can demonstrate inflammatory changes in the mucosa.  ¢ Mucus, pus and blood are visible.  ¢ Multiple ulcers are visible with bleeding.    be done in acute cases) (Fig. 28.34)  ¢ Contracted colon/pipe stem colon  e Absence of haustrations and mucosal irregularity  ¢ Pseudopolyposis appears as stippled appearance   Figs 28.35 and 28.36: Colonoscopy—pinpoint ulcers with bleeding (Courtesy: Dr Filipe Alvares, Medical Gastroenterologist,  KMC, Manipal)   ¢ To find out the extent of involvement of colon  ¢ To follow up patients who are on treatment    . C-reactive protein: Its levels are very high in case of acute   ¢ Blood transfusions to correct anaemia and TPN for  hypoproteinaemia (Fig. 28.37).    Fig. 28.37: Total parenteral nutrition (TPN) before and after surgery  for ulcerative colitis plays an important role in the recovery of the    ¢ Salazopyrines are given in the dose of 2 g/day. Mode of  action: When given orally, it gets split into 5-amino-  salicylic acid and sulphapyridine in the colon. This  suppresses activity of prostaglandins El and E2 and thus  reduces inflammation. They are used mainly to induce  remission.    ¢ Corticosteroids: Less severe cases not responding to  salazopyrines are given a trial of oral prednisolone 60  mg/day. They decrease the frequency of stools. The dose  is tapered off over 3--4 weeks.    e Prednisolone retention enema: 20 mg 1n 200 ml saline in  intractable diarrhoea. It avoids systemic toxicity.  Prednisolone 20-40 mg/day can also be given orally for  3--4 weeks.    ¢ Role of cyclosporine: Those patients who do not respond  to corticosteroids, can be given IV cyclosporin 4 mg/kg/  day. It can induce remission.   Active disease in spite of medical line of management  Severe disease not responding to medical treatment  Dysplasia on biopsy    patient, as these patients are grossly emaciated and hypo- for surgery    3. Immunomodulators e Azathioprine 6-mercaptopurines ¢ Act at DNA level and inhibit  ¢ Cyclosporine used in refractory cases T-lymphocyte functions  4. Monoclonal antibody —  Antitumour necrosis factor-alpha-(TNF @) e Antitumour necrosis  Infliximab  5. Anti-inflammatory (salicylic acid) ¢ Olsalazine (Dipentum) ¢ Directly targeted/released into the    1. Total proctocolectomy followed by permanent ileostomy  (ileoanal anastomosis should not be done because of  incontinence). Ileostomy 1s connected to ileostomy bag.  Adhesive obstruction and chronic perineal sinus are late  complications. This is the procedure with least complica-  tions (Figs 28.38 and 28.39).   e This can be done as one- or two- stage procedure.   ¢ A pouch its created by anastomosing the loops of ileum. A  J shaped pouch is the most popular followed by W pouch  (Figs 28.40 to 28.43).   ¢ The pouch is anastomosed to the dentate line (junction of  upper and lower anal canal) by using stapler or by hand  sutures.   ¢ Protective ileostomy is done and it can be closed after two  months.    The length of the pouch  is generally 15 cm and  Capacity around 300 ml  when distended. Note  that it is a two-layered  anastomosis to create  the pouch. The same  can be achieved by  linear stapling device.   Fig. 28.42: J pouch (Courtesy: Dr Prasad Babu TLVD Ramcharan  Thyagarajan, Srikanth Gadiyaram, Prof Sadiq S Sikora,  Department of Surgical Gastroenterology, Manipal Hospital,  Fig. 28.38: Total colectomy Fig. 28.39: lleostomy Bengaluru)    Wound was sutured  after 48 hours  because wound  infection is a  common  complication after  this surgery    Fig. 28.43: Total proctocolectomy with a pouch and protective  ileostomy was done here    2. Continence is preserved and patient is able to pass the stools  via naturalis.   3. At the same time, all the diseased mucosa has been  removed. Thus, the risk of cancer is negligible.  ¢ Pouchitis 1s a complication.  ¢ See Table 28.3 also.    In general, emergency colitis is required in about 25%  patients with severe attacks. Perforation has a mortality rate  of up to 40% because of faecal contents and toxicity.  Incidence of carcinoma colon is about 10—15%. Longer the  duration of the disease, higher is the incidence.    High protein, carbohydrates, whole grains, and good fats.  Meat, fish, poultry, and dairy products breads and cereals;  fruits and vegetables may be consumed.   For vegetarians: Dairy products and plant proteins — such  as soya bean products may be consumed.   To avoid: Caffeine-Coffee, dried fruits and nuts, alcohol,  meat, spicy food, oily food, soda, etc.   ileum or end of the ileum is brought to the exterior (surface   1. Ileostomy bag has to be fitted well to the body surface.  It needs to be adjusted often   4. Avoid gas forming diet. Patients will learn slowly what  to take and what not to take   5. Long-term complications include—gall stones, kidney  stones, adhesions and intestinal obstruction.   Fig. 28.46: 18-year-old girl  was diagnosed with acute  appendicitis at exploration,  tuberculosis of the ileum  made and limited ileo-  colectomy was done fol-  lowing which ileum was  brought outside as tempo-  rary ileostomy    Fig. 28.47: Loop ileostomy is done following resection of perfo-  rated enteric ulcer. Distal ileum was friable and oedematous.  Hence it is brought out. This is a safer option    It was called regional ileitis because the disease was first  reported in the terminal ileum. However, today it 1s called  regional enteritis because the disease can occur in jejunum,  ileum, colon, oesophagus, etc. Involvement of ileum 1s more  common, followed by colon.    Definition  ¢ Crohn’s disease is a chronic transmural inflammatory  disease of the gastrointestinal tract of unknown aetiology.    1. Infectious agents: Mycobacterium paratuberculosis and  measles virus have been proposed as potential causes of  Crohn’s disease. However, it should be noted that  antimicrobial therapy has not been effective in eradicating  Crohn’s (unlike ATT in TB). Also, no immunological  reaction has been found.   2. Immunologic factors: Similar to UC. Focal ischaemia due  to autoimmune reaction has also been considered.   3. Genetic factors: Single strongest risk factor for  development of Crohn’s is a relative with Crohn’s disease.   4. Smoking: It increases the risk of Crohn’s disease three-  fold unlike its protective effect against ulcerative colitis.    e Disease starts in terminal ileum as ulcerations of intestine  in about 60% of cases.   ¢ There is extensive inflammatory oedema and mucosal ulcers  are present. Fibrotic thickening of the intestine results in  hose pipe rigidity of the intestine.   ¢ There are skip areas which are characteristic of Crohn’s  disease (segments of intestine are normal in between).   ¢ Mesenteric nodes are enlarged. They can be calcified but  do not show any caseation.   PATHOLOGY OF CROHN’S DISEASE  Transmural inflammation  Reticulation cobblestone  Aphthoid ulcers  Noncaseating cicatrising granuloma  Skip lesions  Mesenteric lymph nodes enlarged  Ulcers are in ileum, jejunum, colon, etc.  Rigidity—Hose pipe  Adhesions occur soon  Linear ulcers  Remember as TRANSMURAL   ¢ Once inflammation spreads to the serosa, adhesions develop  between bowel loops or other structures. Abscesses occur in  the mesentery which rupture resulting in internal fistula    The disease is often insidious, slowly progressive with a  protracted course and commonly affects young adults in the  second or third decade of life.   Intermittent colicky lower abdominal pain, diarrhoea and  weight loss are common. Depending upon symptoms, it can  be classified as follows: (Table 28.4)   1. Stage of ileocolitis: Clinically, 1t presents as pain abdomen  and mucus in stools. It is seen 1n younger patients. It may  be associated with fever. Presence of pain and tenderness  in the right iliac fossa mimics appendicitis. If there is a  mass, it may be confused for an appendicular mass.   : ; . patient who underwent loops, area of perforation,  pat Stage of subacute intestinal obstruction occurs due tO appendicectomy, only to dilated loops of bowel are seen  stricture of the terminal ileum. Strictures can be multiple. _ present later with features of in this picture  They are not reversible. Crohn's disease  ale 28.4 Crohn's disease zat . rt |  Colitis Obstruction Mass Nutrition Anorectal disease  ab  Diarrhoea Cramps, distension Right iliac fossa Emaciation Fissure, abscess  Water Vomiting Gurgling, tender, Dehydration Fistula in ane    enterocutaneous. Following are the examples of fistulae  encountered in Crohn’s disease—ileovesical, ileocolic,  ileoileal, ileovaginal, etc.    . Perianal disease in the form of multiple ulcers in the    anorectal region, perianal abscesses, multiple fistulae in ano  are much more common than in ulcerative colitis. Repeated  infection of anal crypt due to diarrhoea is common.  Extraintestinal complications are similar to ulcerative  colitis.    Anal fissure ts the most common anal problem in Crohn’s  disease. Noncaseating granuloma is most common in  anorectal disease.    . Appendicitis: Acute pain in the right iliac fossa can be    confused for acute appendicitis. For benefit of the doubt,  the patient can undergo laparotomy. Laparoscopy and  appendicectomy will be better   ¢ Cobblestone reticulation because of multipleulcers with  islands of normal mucosa in between.   ¢ String sign of Kantor is demonstrated in terminal ileum  due to narrowing of the lumen.   ¢ Multiple strictures and dilated segments in between can  be demonstrated.    inflamed mucosa, which 1s granular with aphthoid ulcers,  which are discrete (Fig. 28.52).    . Fistulography to localise the internal fistula.  . CT scan: It is done to detect thickening of bowel and   A low residue, high caloric diet such as high protein diet  Some patients may require total parenteral nutrition—some  at home called home parenteral nutrition   ¢ Steroids are the mainstay of treatment. They are effective  in inducing remission in 70 to 80% of cases.   ¢ Steroids are most effective in treating small intestinal  disease. They are anti-inflammatory. They do control  diarrhoea, induce remissions. Prednisolone is used for  short term treatment. Long-term use can give rise to  toxicity such as immunosuppression, bone loss, delayed  wound healing, etc.   ¢ Even though salazopyrines and corticosteroids have been  beneficial in Crohn’s disease, salazopyrines do not induce  remissions. They are used in acute ileocolitis. Steroids  can be used for anorectal disease.   ¢ They are effective in treatment of colonic disease. The  main concern is bone marrow toxicity. 6-mercapto-  purines are known to produce pancreatitis.   ¢ Most recent and promising drug 1s infliximab—a mono-  clonal antibody to tumour necrosis factor (TNFa). It  mainly helps in closure of fistula. It is given  intravenously and is used for intestinal and perianal  disease.    but it may have to be done in cases of obstruction, perfora-   4. If the fistulae are present, they are disconnected from  the bowel and excised.    Fig. 28.53: Resected specimen of small intestine. The patient  had intestinal obstruction    Comparison of intestinal tuberculosis, ulcerative colitis and  Crohn’s disease is given in Table 28.5.    In spite of various treatment, there is no cure for the disease.  About 10-20% patients come with relapses and recurrent  symptoms   But for repeated treatment including surgical procedures,  survival is still good as compared to general population    Both small bowel and large bowel in 50-60% patients  Duodenum, stomach, oesophagus can also be involved.  Inflammatory cells and mediators of the inflammation such  as cytokines, interleukins, tumour necrosis factors produce  the inflammatory changes resulting in granuloma  Sometimes, very difficult to differentiate clinically and  pathologically between tuberculosis and Crohn’s disease.  Anus 1s involved in Crohn’s colitis, not in ulcerative colitis.    During the third week of enteric fever, Salmonella typhi,  paratyphi (enteric bacilli) multiply in Peyer’s patches and can  give rise to following problems:    5. Clinical  Bleeding Uncommon Uncommon  Diarrhoea Uncommon Very common  Fever Common Common  Mass Very common Common  Anal disease Rare Very common  Toxic megacolon Never Rare  Fistulae Uncommon Very common  Stricture Common Common  6. Malignancy No Can predispose (low)    Continuous, pseudopolyposis.  Pinpoint ulcers. Crypt  abscesses. Superficial ulcers.  Mucosa and submucosa  involved    1. Haemorrhage ts seen in about 5—10% of cases due to  ulceration of Peyer’s patches. It can be occult, obvious or  rarely massive bleeding. It is managed conservatively in  majority of cases.  2. Perforation of terminal ileum: An oval, vertical  perforation results 1n peritonitis. Enteric perforation need  not give rise to all signs of peritonitis. Guarding and rigidity  can be minimal because of poor, immunocompromised  nature of the diseases and due to Zenker’s degeneration  of abdominal wall muscles. It is a single perforation in about  85% of the cases. It 1s situated in the antimesenteric border  of the terminal tleum. Typically, it occurs in the third week  of enteric fever. Bradycardia, dehydration, toxicity are the  other features.  ¢ Hyperplasia of reticuloendothelial system including  lymph nodes, liver and spleen is characteristic of typhoid  fever (Key Box 28.15).   ¢ Diagnosis of perforation is based clinically on the acute  abdominal pain, bleeding per rectum with/without  guarding and rigidity. High grade fever, toxicity and  bradycardia are other features that help in the diagnosis.   ¢ Plain X-ray abdomen may not reveal gas because of  small sealed off perforation.   ¢ The most useful investigation is CT scan which can  reveal not only pneumoperitoneum but also pericolic  collection, which can be missed by ultrasound (see the  clinical notes below).   ¢ Emergency laparotomy, resection of bowel and end-to-end  anastomosis or closure of the perforation by using non-  absorbable sutures. Abdomen is closed witha tube drain kept   A patient with partially treated enteric fever presented on  the 10th day with acute abdominal pain and fever. Lower  abdominal guarding and rigidity were present. Plain  X-ray abdomen did not reveal gas under the diaphragm.  Ultrasonography did not reveal pericolic collection. A CT  scan of the abdomen was done. It revealed pneumo-  peritoneum and pericolic collection. Immediate laparotomy  and closure of the perforation was done and peritoneal  cavity was drained. The patient recovered dramatically.  When in doubt, request a CT scan.    in the right iliac fossa. Wound infection is common in  such cases. Multiple fistulae are also common (Figs 28.55  to 28.58)   ¢ Small bowel exteriorisation: This can be considered in  cases after resection when both ends of the intestine are  friable. This is avery safe option. After 1-4 weeks, relaparo-  tomy and anastomosis of resected ends is done.    3. Paralytic ileus due to toxic dilatation of intestine results  in distension of abdomen. It 1s managed by drip and suction.    . Thrombosis of the common iliac vein occurs probably due  to sluggish blood flow.   Fig. 28.57: Both ends brought outside — small bowel exteriorisation  (Courtesy: Dr Vidushi, MBBS student, KMC, Manipal)    produced in the early septicaemic phase of enteric fever. 1. Amoebic typhlitis: Inflammation of the caecum by amoeba  Liver, spleen, bone and bowel are commonly affected. is described as amoebic typhlitis. It produces pain in the  Metastatic abscesses are common. right iliac fossa and it can be confused for appendicitis.    attack 1s associated with gripping pain abdomen with blood  and mucus in stool, an urgency to pass stools. High grade  fever and tenesmus are the other features. Chronic dysentery  is more common with 2-4 foul-smelling stools per day and   ¢ Immunocompromised status 3. Amoeboma: Chronic, low grade, persistent inflammation  * Decreased pulse rate—Faget sign of the caecum produces granulomatous hyperplasia of the  You can remember as TYPHOID caecum, with thickening of the pericaecal tissue (producing    ¢ This disease is caused by Entamoeba histolytica and  transmitted mainly by contaminated drinking water.    ¢ After the cysts are swallowed, they are broken down in the  intestine by trypsin into trophozoites which produce  inflammation of the colon. Trophozoites swallow red  blood cells and multiply by mitosis. They enter into  crypts of Lieberkuhn. They produce multiple submucous  loculi which later result in multiple ulcers. These ulcers  are flask-shaped (bottle neck) ulcers with healthy  intervening mucosa.    also occur in rectosigmoid junction. This 1s, however,  uncommon nowadays because of effective treatment with  metronidazole.   ¢ Clinically, this manifests as mass in the right iliac fossa  causing dull aching pain, vague ill health, tender palpable  caecum with guarding. It can be confused for ileocaecal  tuberculosis or carcinoma caecum.   4. Amoebic perforation of caecum or sigmoid can occur  resulting in pericolic abscess. Peritonitis needs emergency  surgery.   5. Massive bleeding per rectum is rare. It occurs due to  separation of the slough.    1. Metronidazole 400—600 mg, 3 times a day for 10 days. It  acts on amoebae present in the lumen and tissue.   2. Diiodohydroxyquin 650 mg, 3 times a day for 20 days 1s  another alternative.    3. Diloxanide furoate is ideal for chronic cases in the dose of  500 mg, 3 times a day for 10 days. It acts on luminal  amoebae. It is the drug of choice in chronic cyst passers.    ¢ Supportive treatment in the form of hospitalisation,  correction of dehydration, antispasmodics and bedrest  is also advised. Stool culture must be done before and  after treatment with antiamoebic drugs.    ¢ These are the changes which occur in the intestines after  they are exposed to radiation.   ¢ Incidence is 5% after 4500 cGy units to 30% after 6000  cGy units.   ¢ Chief factor 1s radiation induced damage to the blood vessel  supplying the vessel wall.   ¢ Chronic intestinal ischaemia occurs resulting in stricture  and intestinal obstruction. Some cases are due to fibrosis,  dense pelvic adhesions, bowel getting entrapped into the  fibrosis, fistula formation and pelvic abscess.   e When there is no obstruction, conservative line of treatment  to be followed.    ¢ If there is obstruction, laparotomy with resection or bypass  or any other treatment depending upon the findings.   ¢ Difficult to prevent these changes. However, an absorbable  mesh can be placed over the small intestines after pelvic  surgery when radiation is contemplated.   ¢ Malignant tumours such as adenocarcinoma, gastro-  intestinal stromal tumours (GIST), carcinoid and lymphoma  can occur in the small intestine.   Fig. 28.62: This patient presented to hospital with abdominal pain.  The Registrar who examined the case reported that there was a  mass abdomen. However, when he presented the case next day,  there was no mass. The patient had undergone laparotomy  10 years back for similar complaints. ASsegment of bowel had been  resected earlier. This photograph gives the clue to the diagnosis—  pigmentation of lips and oral mucosa. The mass (appearing and  disappearing) was due to intussusception (Courtesy: Prof Sreevatsa,  HOD, Prof Bharathi, Prof Bagli, Dr Srikar Pai, Department of  Surgery, MS Ramaiah Medical College, Bengaluru, CME 2008)    We had 3 interesting cases of Peutz-Jegher syndrome. The  first case was of a boy of 14 years, who presented with    The second case was a 50-year-old lady, who presented  with duodenal carcinoma. Endoscopy revealed polyps in  the stomach and duodenum. Specimen of pancreatico-  duodenectomy revealed it as a case of Peutz-Jegher  syndrome. This lady did not have pigmentation of the oral  mucosa.   The third case was of a 35-year-old male who has been  coming to our hospital with intermittent bleeding and  anaemia. Endoscopy revealed multiple polyps in the  stomach and duodenum. Small bowel enema demonstrated  multiple polyps in the small bowel. Even proctoscopy  showed multiple polyps in the rectum. He is being managed  conservatively.    2. Melanosis of mucosa of lip, cheek, interdigital space and  even perianal skin (Fig. 28.62 and Table 28.6).    3. Multiple polyps in the small bowel and large bowel mainly  in the jejunum. They are hamartomatous polyps.  ¢ It 1s an autosomal dominant disease.   ¢ As a cause of bleeding per rectum, results in chronic  anaemia.   ¢ Female patients have increased chances of breast and  cervical cancer (see clinical notes)    Fig. 28.63: CECT scan showing jejunal growth with narrowing of  the lumen of the jejunum   ¢ Resection of that portion of bowel containing polyp, in cases  of bleeding or intussusception.    ¢ Incidence is 40% of small bowel tumours. Overall, small  bowel tumours are rare. Reasons have been given in Key    Figs 28.64A and B: Push enteroscopy showing ulcerative lesion  in the proximal jejunum    Duodenum 1s the commonest site of adenocarcinoma. If It  arises from first part and second part, it may require  Whipple’s pancreaticoduodenectomy. From the third part,  early cases of adenocarcinoma can be resected without  removal of pancreas.   Some familial diseases predispose to adenocarcinoma. They  are familial polyposis coli, Adenomas, Crohn’s disease, etc.    Clinical features include vague features like nausea, poor  appetite, crampy abdominal pain, bleeding and intestinal  obstruction.   Push enteroscopy has the advantage of visualisation of  the growth and to take biopsy (Figs 28.64A and B).   Other option is capsule endoscopy. It is time consuming  and biopsy ts not possible   Fig. 28.66: Resection of the growth done with 6 cm margin with  lymph nodes    Fig. 28.67: This patient presented with abdominal pain, melaena  and loss of weight. CT scan of the abdomen revealed mass in the  jejunum. It was resected (inset). Mucosal surface showed ulceration.  Histopathology report was adenocarcinoma    ¢ Resection with at least 7 cm margin with lymphadenectomy  is the treatment of choice (Figs 28.65 to 28.67).  ¢ Hardly any role for radiotherapy and chemotherapy    e Earlier called as leiomyoma and leiomyosarcoma, they  occur in jeyunum or ileum. These are uncommon  mesenchymal tumours (Figs 28.68 to 28.70).    e They arise from interstitial cells of Cajal which are  pacemaker cells that regulate motility and peristalsis.   Fig. 28.68: GIST involving terminal ileum, caecum and ascending  colon (Courtesy: Dr Stanley Mathew, FRCS, Professor, KMC,  Manipal)    Fig. 28.69: GIST involving jejunum—resected specimen showing  mucosal ulceration (Courtesy: Dr Padmanabha Bhat, Dept. of  Surgery, KMC, Manipal and Prof HD Shenoy, Father Muller’s  Hospital, Mangalore)    ¢ Appendix: 65%. It is the most common site. It occu!  more commonly in females. When the tumour occurs 1  the appendix, it is usually benign, hard and occurs tn disté  one-third of appendix (Key Box 28.17).   ¢ Terminal ileum: 30%. Most of them are malignant.  When the tumour occurs in the ileum it is usually malig  nant and produces multiple bulky secondaries in the livei  even when primary Is very small. Fibrosis of the mesenter  results in kinking of bowel causing periodic abdominal pair   e Most common neoplasm of the appendix  e Mostly found in distal one-third of the appendix  e Mostly tumour is yellow staining for tmmunohistochemistry  e Mostly benign—very rarely metastasise   | ¢ Mostly appendicectomy is sufficient. Rarely, right hemico-  Fig. 28.70: GIST from small intestine (Courtesy: Dr Ramesh Rajan, lectomy may be required.  MS, MCh, Surgical Gastroenterologist, Trivandrum, Kerala)    ¢ They express CD117/CD34 antigen. CD117 is known as  C-kit receptor.    e Diagnosis of malignancy is by mitotic figures: < 10 mitoses/  high power field (HPF) suggests a low grade malignancy. Secondaries in the liver  ¢ > 10 mitoses/HPF suggests high grade malignancy.    ¢ Increased incidence of C-kit has also been found in patients  with neurofibroma.    It arises from argentaffin/chromaffin cells which are present  in the crypts of the villi of intestine. These cells are called as    Kulchitsky cells. These cells stain with ammonical silver salt peat caren  solution to black colour. Hence, they are called as  argentaffinoma or chromaffinoma. They secrete 5-HT (sero-  tonin or 5-hydroxytryptamine). They can be single or multiple  and can be associated with adenocarcinoma. Fig. 28.71: Carcinoid syndrome  Comparison of carcinoid tumours at various sites  Foregut Midgut Hindgut  Site Duodenum, stomach, bronchus, pancreas, etc. Jejunum, ileum, right colon Rectum  Incidence Rare Common Uncommon  Hormones No serotonin 5-HT, prostaglandins, insulin, ACTH Do not secrete    Bronchial carcinoid produces bronchial obstruction and produces carcinoid syndrome without secondaries in liver.    ¢ The hormones produced by the tumour 5-HT (serotonin)  are not metabolised because of the secondaries. So, they  are absorbed into the circulation and produce various  symptoms. This is called as carcinoid syndrome  (Fig. 28.71).   3. Symptoms are red-blue cyanosis of skin, flushing attack  and asthmatic attacks, intestinal hyperperistalsis causing  diarrhoea, pulmonary and tricuspid stenosis with CCF.   ¢ 24-hour urine for 5-hydroxyindole acetic acid. Normal  levels 2—9 mg/24 hours. Very high values are found in this  condition.   ¢ Ultrasound and CT scan are other investigations which will  help in detecting mass lesion or liver metastasis.    1. Resection of the tumour with a wide margin along with  lymph nodes. Otherwise debulking should be done.   2. Bromocriptine 2.5 mg twice a day can be given to reduce  the symptoms. Other agents which can be used are  methysergide and diphenoxylate hydrochloride.   3. Secondaries in the liver are treated by intra-arterial (hepatic  artery) streptozocin. Localised liver metastasis can be  treated with resection.   4. Therapeutic embolisation of hepatic artery by using gel  foam, etc. willreduce the size of the liver thereby decreasing  discomfort to the patient.   5. Injection octreotide 100 ug IV is the drug of choice 1n cases  of carcinoid crisis (Severe bronchospasm).    . Dose is: 100 micrograms subcutaneously tds in patients  with mild/moderate, non-life-threatening carcinoid  syndrome (Key Box 28.18).    Primary: Arising from lymphoid tissue  Secondary: Part of systemic lymphoma. Ileum is the most  common site of lymphoma (Key Box 28.19).    They may be associated with multiple endocrine neoplasia  (MEN ) type 1 and 2    About 15% of carcinoid patients may have carcinoma  elsewhere, example: carcinoma stomach, carcinoma lung or  Carcinoma colon.   Appendix is the most common site. Often appendicectomy  is enough for malignant carcinoid tumour.    Secondaries can be multiple and big, but slow growing like  primary. Arterial embolisation has been tried in many cases.  Since they are slow growing and prognosis is good-  aggressive treatment in the form of surgery, octreotide, liver  resections and rarely liver transplantation has been done   e Bleeding, obstruction, perforation and weight loss  2. Primary lymphoma with coeliac disease    'Alpha chain disease is a disorder characterised by the secretion  of a defective a-heavy chain. Patients present with steatorrhoea,  often progressive and fatal.    ¢ Short gut syndrome occurs due to massive resection of  the bowel resulting in loss of length of the bowel, loss of  absorptive area of the bowel and loss of valves. Superior  mesenteric artery being an end artery, thrombosis at its  origin is invariably fatal.    ¢ Midgut volvulus of neonates 1s congenital due to arrested  rotation resulting in floating caecum and mobile intestine.  We had an interesting case of midgut volvulus in an  18-year-old boy consequent to a laparotomy done for  perforated duodenal ulcer. While replacing the coils of  bowel within the abdomen, the mesentery was probably  twisted resulting in massive gangrene. This boy now has  about 100 cm of the small bowel and surviving on “baby  food” for the last 5 years.    ¢ Necrotising enteritis (enteritis necroticans) is a  complication of infection of small bowel by Clostridium  perfringens. It usually occurs after a heavy feast where pork  1s consumed. There is extensive suppuration of mucosal  and submucosal layer of jejunum (also ileum). Serosa may  show multiple dark bluish patches. Massive resection 1s  done for a necrotic, perforated, unhealthy bowel which  results in short gut syndrome.    patients who receive radiotherapy to the abdominal and  pelvic regions, e.g. carcinoma cervix. Arrest of cell division    resulting in mucosal thinning, ulceration followed b:  oedema and later, fibrosis are characteristics of thi:  condition. Endarteritis and vasculitis also add to thes:  changes resulting in stricture, perforations, abscess, mal  absorption and multiple resection, etc.    ¢ Infants tolerate extensive resections better than adults.  Patients with less than 100 cm of the small bowel will  develop severe nutritional deficiencies and may require  parenteral nutrition.    e e s e » e e € e e e e e e e e e e e e e e e e e e e e    EFFECTS OF SHORT GUT  e Severe malabsorption  e Gall stones  e Fatty infiltration of liver  e Urinary stones  e Gastric hyperacidity    . Malabsorption of fat and fat soluble vitamins: Can  occur after ileal resections due to interruption of  enterohepatic circulation of bile salts. These bile salts  enter the colon and are converted into secondary bile  salts. These bile salts block absorption of water and  electrolytes.    2.Gastric hypersecretion: Due to delayed clearance of  gastrin, as In proximal jejunal resections, there is  Increased gastric secretion of acid resulting in  hyperacidity.   3. Liver disease: Fatty infiltration of the liver and mild  hyperbilirubinaemia is a feature in massive resections  and jejyunoileal bypass. Acute fulminant hepatic failure  can also occur.   4. Gall stone formation: There is increased incidence of  cholesterol stones as a result of reduced bile salt pool,  after ileal resection and jejunoileal bypass.   e All types of urinary stones are common due to low levels  of calcium excretion in the urine and high levels of  oxalate.   ¢ Water and salt depletion, and loss of K” causes  hyponatraemia and hypokalaemia.    ¢ As aresult of loss of significant bowel, dilatation of the  remaining intestine and villous enlargement takes place.  This is brought about by a humoral agent, enteroglucagon.  In children, length of the bowel 1s increased. Also, the  number of cells in the villiis increased (work hypertrophy).  There is also evidence to suggest gradual slowing of the  transit time.    ¢ Treatment of short gut patients is difficult. It needs a special  set up of dieticians who plan “proper food” for these patients  in consultation with treating surgeons. It is a gradual process  of feeding the patient beginning with parenteral nutrition  and progressing to normal, low fat diet after a few months.  In the initial 2-3 months following massive resection, total  parenteral nutrition including supplementation of fluid and    electrolytes is the ideal treatment. Sips of plain water or  oral hypotonic solutions can be allowed.   After 2-3 months, when adaptation of bowel takes place,  enteral feeding 1s started gradually with baby food, fat-free,  fibre-free, protein rich, liquid diet. Essential fatty acids  should be supplied. Diarrhoea is a common problem and is  treated with loperamide tablets.   Enteral feeding can contain low fatty diet in addition to the  other nutrients mentioned above.    They occur in the mesenteric border unlike Meckel’s  which occur in the antemesenteric border.    Intestinal fistulae are abnormal communications between  two portions of the intestine, between the intestine and some  other hollow viscus, or between the intestine and the skin  of the abdominal wall.   When it involves skin and intestines, it is called  enterocutaneous fistula.   Despite significant advances in their management, intestinal  fistulae remain a major clinical problem, with an overall  mortality rate of 15 to 25%.    latrogenic (70%)—postoperative: Injury to intestines  unnoticed at the time of surgery or injury recognised at the  time of surgery, sutured but has given way are prime causes  of enterocutaneous fistula. Anastomotic leak, partial or  complete, is another important cause of entero-  cutaneous fistula. Meticulous surgical techniques, gentle  handling of the bowel, usage of the proper suture  material (Vicryl and silk), ensuring adequate blood  supply to the intestines which have to be anastomosed will  largely prevent anastomotic leak (Fig. 28.73).    Stump blow out: Duodenal blow out: Typically happens  4—5 days following surgery (see page 506).   Fig. 28.73: Small intestinal fistulae due to anastomotic disruption  and wound dehiscence    ¢ Inadequate resection of the diseased segment: In such  situations, anastomosis may be in unhealthy bowel.    ¢ Instrumentation: Oesophageal perforations are dangerous  ones and can occur even with flexible scopies.    ¢ Spontaneous (30%): Crohn’s disease, diverticular disease  of the colon, appendicitis are also known to give rise to  fistulae.   ¢ Foreign body: Swabs, tips of the suction tubes (detached),  retained sponges or instruments.    A 60-year-old man was operated for appendicitis. On the  fifth postoperative day, he developed faecal discharge from  the wound. Conservative management was done for 7 days  with total parenteral nutrition and other measures. Fistula  persisted. CT scan was done on the 14th day. It revealed a  growth in the hepatic flexure almost encircling the lumen.  Colonoscopy and biopsy proved adenocarcinoma colon.  He underwent re-exploration and right hemicolectomy was  done. The fistula stopped and he was discharged from the  hospital after 10 days. This case highlights the fact that in  the presence of a distal obstruction, fistula will not close.  More importantly, what the patient had was not  appendicitis but subacute intestinal obstruction ! The pain  was due to colic.   Lateral duodenal fistula does not heal because large  volume of contents are poured into the second part of  duodenum and there 1s no rest to the part.   When the defect is more than I cm, and the track is more  than 2 cm, fistula does not close (see clinical notes).    Delay in recovery from paralytic ileus or a common phrase  used—when the patient is not doing well are early  indications of a leak or a breakdown of anastomosis. Once  the fistula establishes, diagnosis 1s easy because the drain  will start draining intestinal contents including food  particles (if oral intake 1s started). Intra-abdominal  collections, abscess, fever, sepsis are other features of leak.  Recognising the cause is important because it can dictate  treatment. To give an example: If any foreign body is left  in the abdomen, exploration and removal may be  urgently required.   If a specific disease 1s suspected such as tuberculosis, anti-  tuberculous treatment may cure a fistula.    Nil by mouth, total bowel rest, introduction of a nasogastric  tube (Ryle’s tube) with continuous drainage decreases  fistula output.   Proton pump inhibitors decrease gastric secretions.  Protection of skin is by liberal zinc oxide application.  Common fluid and electrolyte problems seen in patients  with GI fistula include dehydration, hyponatraemia,  hypokalaemia and metabolic acidosis. They have to be  corrected.   Drainage of collections by CT or ultrasound-guided  aspiration or pig tail catheter insertions.    Nutrition has been discussed in detail in the chapter on fluid-  electrolyte balance and nutrition. A few points worth    mentioning are that more distal the fistula, less is the  requirement of total parenteral nutrition (TPN). TPN 1s  safe today. One may have to wait for 4—6 weeks with TPN  for the final repair of complicated fistula such as lateral  duodenal fistula or ileal fistula.   Placement of nasogastric, nasojejunal tubes, per-  cutaneous gastrostomy or jejunostomy for nutrition 1s  an important step which can be done with the help of  radiology and imaging department.   Blood culture helps in giving appropriate antibiotics  Chest X-ray to rule out static pneumonia or ARDS, pleural  effusions (source of sepsis also).    USG: Drainage of collections by catheters. If drainage  persists, suspect fistulous communication.   Is the bowel completely disrupted or is it a lateral fistula with  the bowel in continuity?    Contrast CT abdomen: To detect collections, disconti-  nuity, diseased segments, distal obstruction foreign body.  Extremely useful investigation in intestinal fistula.  Colonoscopy: Barium enema are other investigations used  In specific situations such as colonic fistulae.    Drainage or aspiration of pus urgently when there is sepsis  before doing an elective repair.   Feeding jejunostomy as in high fistulae, e.g. duodenal or  oesophageal fistula.  Diversion/exclusion/colostomy/bypass are various other  treatments depending upon the location of fistula (Fig. 28.74).  Definitive treatment: It includes restoration of continuity-  resection and anastomosis, internal diversion or exteriori-  sation of bowel as in suspicious viability (mesenteric  ischaemia or enteric perforations—triable bowel, known  for leak and releak).    Fig. 28.76: Extensive excoriation of the skin in a faecal fistula.  Zinc oxide has been applied to the abdominal wall   ¢ Suction catheter placed in situ: Low pressure suction  60-80 mmHg.  Generally, dressings need to be changed every 4th  hourly. Pouch/reservoir system should be employed.    ¢ Depending upon the size of the defect, immediate or delayed  closure can be achieved with tension-releasing incisions  on the external oblique or laparostomy and secondary  suturing, skin grafting or by using prosthetic mesh such as  Marlex or Prolene.    Small intestinal diverticula are far less common than colonic  diverticula. Multiple sac-like mucosal herniations occur    ¢ Pain abdomen, signs of peritonitis such as tenderness  Diverticula are more common in duodenum than jejunum or guarding or localised abscess.   o e e e 9 < . .  They occur in the mesenteric border unlike Meckel’s abscess. Later, at laparotomy it can turn out to be diverticul  which occurs in the antimesenteric border. (Figs 28.78 and 28.79).    e Diet low in fibre and rich in fat and some visceral myopathy    e Most common extraintestinal manifestations of ulcerative  colitis are peripheral arthritis and ankylosing spondylitis.    e Most common site of gastrointestinal perforation in enteric  fever is terminal ileum.    the cause of anaemia due to occult blood loss eo aa  e Most common site of malignant carcinoids is terminal ileum.   e All topics have been updated with flowcharts and  coloured pictures.    e Small intestinal divericula, intestinal fistulae and tumours  have been discussed in more detail.    Tenderness in the Sir Philip Manson-Bahr's point is a  feature of:    One of the drugs used in the treatment of carcinoid  syndrome is:    The most recent and promising drug used in the  treatment of Crohn's disease is:    Most common anal problem in Crohn's disease is:  A. Anal fistula B. Haemorrhoids  C. Anal fissure D. Perianal abscess   A. Crohn’s disease B. Tuberculous enteritis  C. Typhoid enteritis D. Ulcerative colitis    ANSWERS  1A 2A 3 A 4C Si © 6C 7D 8A 2B 10 A  1.8 12 D 13 B 14 C 15 D 16 A ae = 18 C 19 A 20 A    Large intestine extends from ileocaecal valve to anus. It  has five segments: right colon, left colon, transverse colon,  sigmoid colon, rectum and anal canal (Fig. 29.1).    Interestingly, alternating portions of the colon are mobile  and fixed. Ascending colon and descending colon are fixed  but caecum, transverse colon and sigmoid colon are mobile.  Mobile structures can undergo twisting (volvulus).    Layers of colon: Mucosa, submucosa, muscularis propria  andserosa. Innercircular and outerlongitudinal musclelayer  constitute muscularis propria. Inspite of 4 layers, wall of the  colon 1s thin. Hence it distends much more in obstruction.    Caecum continues as ascending colon.  Covered by peritoneum in front and on both sides.  In 25% of patients, it has a mesentery.    Right paracolic gutter 1s deep on the lateral aspect of  ascending colon—space for para-colic abscess in cases of  perforation peritonitis.   It is suspended by transverse mesocolon, loops down and  is adherent to the posterior wall of omental bursa.  Diseases   Splenic flexure is deeply situated. Therefore, malignancy  in this area can be easily missed.    Outer longitudinal muscle is arranged in the form of three  strips called taenia coli. All three join at the rectosigmoid  junction and form a complete longitudinal layer of the  rectum. These three taenia coli converge at the base of  the appendix. This is an important method to localise  appendix. Inner circular muscle coalesces distally to form  internal anal sphincter.   1. Mesocolic: Transverse and sigmoidcolon are attached by this.  2. Omental: To which omental appendices attach.    Since taenia are Shorter than intestine, the colon becomes  sacculated between taenia forming haustra.    1. Superior mesenteric artery, a branch of abdominal aorta  arises at the level of first lumbar vertebra (LI). It supplies  the entire small bowel and the right colon up to proximal  2/3rds of transverse colon. Branches of superior mesenteric  artery (SMA) supplying colon are:   A. Middle colic artery supplies ascending colon, hepatic  flexure and the transverse colon mainly. It divides into  right and left branches.   2. Hepatic flexure is broad 2. Splenic flexure is acute and narrow  3. Hepatic flexure is not deep 3. Splenic flexure is deep   4. Paracolic space is broad 4. Left paracolic space is narrow   C. Ileocolic artery supplies the terminal ileum and  ascending colon. It divides into anterior and posterior  caecal branches and supplies caecum and appendix  through appendicular artery.   2. Inferior mesenteric artery (IMA), a branch of abdominal  aorta arising at the level of L3 supplies the left colon up to  mucocutaneous junction at the lower end of anal canal  (Hilton’s line). Its branches are (Fig. 29.3):   A. Left colic artery which anastomoses with branches of  middle colic artery. It divides into upper and lower  branches supplying the descending colon.   ¢ The anastomotic branches form marginal artery of  Drummond, whichis relatively narrow inthe region of sple-  nic flexure (another reason for development of ischaemia).   ¢ Arc of Riolan is the anastomotic arcade formed between  branches of IMA and SMA.    ¢ Follows the corresponding artery and empty into superior  and inferior mesenteric veins, ultimately draining into the  portal vein (Fig. 29.4).   ¢ Thus, any inflammatory conditions of the colorectal/  surgical procedures if they infected, infection can easily  spread into portal vein and result in portal pyaemia.    Inferior vena cava  Right common iliac vein  Right internal iliac vein   N1: Epicolic, paracolic nodes are the first to get involved.  N2: Nodes at the origin of ileocolic and middle colic  arteries—intermediate nodes.   N3: Nodes at the origin of superior and inferior mesenteric  arteries. They are involved in approximately 50% of the   Colon has 4 types of motility. Propulsive, retropulsive, mass  peristalsis and gastrocolic reflux. Thus, contents travel  aborally. Retropulsive activity is more in the right colon, these  allowing the contents to ‘churn’ more and more. Mass  contractions are found more in left colon—specially after  meals.   ¢ Itdepends upon several factors such as food habits, genetic,  social customs.   ¢ Generally a patient is said to have constipation if he passes  less than 2 stools per week.   ¢ In addition to the low fibre diet, emotional feelings and  many rectal diseases also cause constipation. Example—  prolapsed rectum, solitary rectal ulcer syndrome. Colonic  disease such as megacolon—Hirschsprung disease is an  important cause of constipation in children.  Increasing constipation in elderly patient suggest carcinoma  in left colon. Needs to be evaluated by colonoscopy.   Fig. 29.5: Lymphatic drainage (see text for description of N1, N2  and N3)    These are called principal nodes. Recycling of various nutrients takes place in the colon.    - Sympathetic (inhibitory) arise from T10-T12 and LI-L3 Butyrate is the main product of bacterial fermentation. It 1s  ¢ Parasympathetic (stimulation) required mainly as a fuel for colonic epithelium.    Anaerobic bacteria: They constitute more than 99%. The  most common pathogen is bacteroides fragilis (10!° /g of  faeces). Other organisms are clostridia, cocci, etc.  Aerobic bacteria: Escherichia coli is the most common  organism about 10’/g of faeces. Other organisms are  Klebsiella, Proteus and Enterobacter.   Normal function: Bacteria degrade bile pigments thus  resulting in brown coloured stools. They alsohelp in colonic  motility and absorption. Fatty acids produced by bacteria  supply nutrition to colonic epithelium. Bacteria also supply  vitamin K to the host.    Prebiotics are non-digestible food ingredients that stimulate  the growth and/or activity of bacteria in the digestive system  in ways claimed to be beneficial to health. Traditional  dietary sources of prebiotics include soybeans, inulin  sources (such as Jerusalem artichoke, Jicama, soya and  chicory root), raw oats, unrefined wheat, unrefined barley,  and yacon.   Probiotic is defined as a “live microbial feed supplement  which beneficially affects the host animal by improving its  intestinal microbial balance”. They are non-degradable  oligosaccharides. They stimulate the growth of beneficial  intestinal bacteria.   Probiotics are dietary supplements which contain live  cultures of bacteria and yeast that are beneficial to colonic  and host function. The common species used as probiotics  are Lactobacillus and Bifidobacterium. Probiotics stimulate  immune function, exhibit anti-inflammatory property and  suppress pathogenic organism.   Clinical application: When a person takes antibiotics, both  the harmful bacteria and the beneficial bacteria are killed.  Bacterial change in flora alters carbohydrate metabolism  with decreased short-chain fatty acid absorption and result  in osmotic diarrhoea. In a similar fashion, antibiotic therapy  causes increase in the growth of Clostridium difficile. Thus,  probiotics have been recommended in antibiotic induced  diarrhoea. Also, they have been used to treat diarrhoeas in  ulcerative colitis, in pouchitis (inflammation of the pouch  after total proctocolectomy for ulcerative colitis) and in  necrotising colitis in children.    Benign tumours (Fig. 29.6) are usually referred to as polyp,  which means elevated from the surface. They are as follows:   They are premalignant and the risk of malignancy is greater  with increase in the size of the adenoma.   Adenoma less than | cm—risk of malignancy is 1%;  1-2 cm is 10%; > 2 cm 1s 30%.    Most of the neoplastic polyps occur in elderly patients  (> 50 years).   Adenoma larger than 5 mm in diameter carry risk of  malignant potential.   More the polyps, more chances of synchronous carcinoma  Flat adenomas also carry malignant potential.    Symptoms and signs of polyps: Bleeding per rectum is the  most common symptom. Fresh bleeding 1s seen in rectal  polyps. Typically it is painless. It 1s intermittent. If it 1s  associated with change of bowel habits means probably a  malignant change. These changes include mucus discharge,  tenesmus, sometimes constipation. In children, polyp may  project outside the anus. In such cases, it has to be  distinguished from prolapsed rectum.    Colonoscopy and polypectomy 1s the standard treatment.  If specimen shows invasive carcinoma, radical surgery  needs should be done.    Although most neoplastic polyps do not evolve to cancer,  most colorectal cancers originate as a polyp.    This can occur in the colon as in Peutz-Jeghers syndrome.  Risk of malignancy 1s very limited. Symptomatic polyps  need to be treated.   Juvenile polyps are usually single and occur in children.  They give rise to bleeding and are easily resected. They do  not have malignant potential.   Fig. 29.8: Thousands of polyps. One of them is 2 cm and is  pedunculated    FAP is a genetic disorder inherited as a Mendelian  dominant. The gene APC (adenomatous polyposis coll) is  located on the short arm of chromosome 5. Prevalence: |  in 10,000. It is clinically defined by the presence of more  than 100 colorectal adenomas (Figs 29.8 and 29.9).   It is transmitted from both sexes. The incidence is same in  either sex.   When it is associated with desmoid tumour, craniofacial  osteoma, epidermoid cysts, congenital hypertrophy of  retinal pigment epithelium, it is described as Gardner’s  syndrome (Key Box 29.3).   When familial polyposis coli is associated with central  nervous system tumour and glioblastoma, it 1s called  Turcot’s syndrome.   50% of them have benign gastric polyps and 90% of them  have duodenal polyps.    Runs in families; other members of the family are affected.  Manifests at the age of 20 in the form of blood and mucus  in the stool, loose stools, etc. It produces crampy lower  abdominal pain.   Anaemia, weight loss and protein malnutrition occur slowly.  Mean age of development of carcinoma is 39 years.    Treatment  * NSAID: Sulindac 300 mg, twice a day and aspirin   325 mg once a day have been found to decrease the size of  polyps.   Other mesodermal tumours—desmoid tumours, osteoma,  epidermoid cysts can be present (Gardner’s syndrome).   Sigmoidoscopy from age of 15 at intervals is the  investigation of choice.    ¢ Patients with FAP who are above the age of 30 have high  chances of having a carcinoma in the colon. Hence, even  when there 1s no malignancy, surgery 1s advisable.    ¢ Many patients do not like ileostomy. Hence, a subtotal  colectomy with ileorectal anastomosis can be done. This 1s  done provided that rectum is examined frequently and  endoscopic snaring of the polyps is done regularly,  especially in a young patient.   ¢ Restorative proctocolectomy with ileoanal anastomosis  by using a pouch ts another alternative. However, it is a  major surgical procedure and should be undertaken only  by an experienced surgeon.    [f there are no adenomas by the age of 30 years, FAP is  unlikely.    Also called hyperplastic nodules. They are of viral aetiology.  They do not have malignant potential.   Figs 29.10 and 29.11: Familial adenomatous polyposis with two  malignancies—lower rectum and hepatic flexure. This patient was  being treated for chronic diarrhoea for 7-8 years with various  medications. ‘He underwent colonoscopy for the first time in our  hospital.’Total proctocolectomy specimen (Courtesy: Dr Challa  Srinivas Rao, Professor, Dept of Surgery, and Dr Ravi, Konaseema  Institute of Medical Sciences (KIMS), Amalapuram—Andhra  Pradesh)   ¢ Lynch’s syndrome II: Cancer family syndrome—they  have extracolonic cancers such as endometrial cancer,  Ovarian cancer, transitional cell cancer, etc.   ¢ Synchronous carcinoma means more than one cancer  at the time of diagnosis. Metachronous carcinoma  means appearance of second carcinoma after 6 months  can occur here (Fig. 29.12).   1. At least 3 members in a family should have colorectal  cancer—two of whom are first degree relations.   3. At least one relative should have colorectal cancer by less  than 50 years of age.    Anoproctoscopy: One can examine up to 10-12 cm of anal  canal and rectum. Rubber band ligation (for piles) and  polypectomy can be done with this instrument.   Flexible sigmoidoscopy: The scope measures about 60 cm  in length. One can easily reach up to splenic flexure. Bowel  wash or an enema 1s given before the procedure. No sedation  is required (Figs 29.13 and 29.14).   be taken. Polyps are visible after 15 years and certainly by the   e Itis the investigation of choice in most of the large intestinal  lesions.   ¢ Biopsy of caecum/ileum in suspected cases of cancer.  ¢ Ileocaecal tuberculosis—to take biopsy   It is the second most common cancer and cancer related death  cases in the western world next only to lung cancer. The  incidence increases with age. Multiple synchronous lesions   (more than | malignancy at the time of diagnosis) is found in  about 5% of the patients. Colon is also one of the sites of  metachronous cancer (new malignancy appearing after 6  months of curative surgery). More than 95% are adeno-  carcinoma and surgery remains the most effective treatment.  Survival has improved because of early diagnosis and  multimodality of the treatment.   Over a period of years the understanding of development  of carcinoma has changed and more and more molecular  biology of colonic cancer is being discussed. The Fearon  Vogelstein adenoma-carcinoma multistep model of  colorectal neoplasia represents one of the best-known models  of carcinogenesis (Key Box 29.4).    APC, K-RAS, DCC and p53 are oncogenes that  convert normal cell to cancerous cell   ¢ Terminology: Before we start the discussion on carcinoma  colon, we shall study a few terminologies used in carcinoma  colon. They are synchronous carcinoma, metachronous  carcinoma, familial colorectal carcinoma.    ¢ Moertel’s definition: Synchronous cancers as those occurring  within 6 months of the first primary cancer, or two or more  histologically distinct simultaneously detected malignancies  or more than one malignancy at the time of initial diagnosis.  This will happen especially in cases of colon and upper  aerodigestive tract wherein the stimulus or aetiological factor  for malignancy affects different parts of the organ.   ¢ Colon, head and neck, oesophagus are the site of  synchronous carcinomas.   ¢ Incases ofcarcinomacolon with specific aetiological factors  such as familial polyposis coli, ulcerative colitis, hereditary  non-polyposis cancer, often carcinoma is synchronous.   ¢ Thus, it is important to do a complete colonoscopy when a  patient comes with colonic carcinoma because he/she may  be having another synchronous carcinoma elsewhere. See  the case report in the next page.    Plain X-ray abdomen showed intestinal obstruction.  Exploratory laparotomy was done. A 3 cmconstricting growth  was identified at rectosigmoid junction and high anterior  resection and anastomosis was done. On the 4th postoperative  day, the patient was allowed liquid diet. Distension increased.  For another 3 days, distension went on increasing. Plain  X-ray abdomen revealed obstruction with more gas than before.  The patient was having colicky abdominal pain. Exploratory  laparotomy was done. Findings at 2nd laparotomy—  anastomosis was intact. Transverse colon was hugely dilated.  Careful palpation of splenic flexure revealed one more growth.  A resection and anastomosis was done again. Patient was  discharged after 10 days. The first surgeon agreed that after  finding out the rectosigmoid growth, he did not look for any  other lesions (mistake). This was obviously a case of  synchronous carcinoma.    Metachronous cancer was defined as those cancer occurr-  ing more than 6 months following resection of one  malignancy.   Family history of hereditary, nonpolyposis colorectal cancer  (HNPCC or Lynch syndrome), an autosomal dominant  disease, also can present with both synchronous or  metachronous colorectal cancers.   The associated genetic defect lies at the mismatchrepair genes,  responsible for the correction of DNA bases mismatch.  The coexistence of adenomatous polyps 1s also considered  a risk factor for the development of metachronous lesions.  Thus, after treating one carcinoma, example—carcinoma  sigmoid, annual colonoscopy is recommended. If polyps  are detected, patients have to be informed about the polyps  and their potential of malignancy.    All these have a carrier gene and thus, run in families.  Malignancies occur in young age group. Often they are  synchronous. Metachronous lesions are not uncommon.    Certain criteria have been laid upon for the diagnosis o:  these conditions which have been discussed already.    Genetic instability 1s the chief factor responsible. The  instability can be at chromosomal level called chromosomal  instability or at DNA level called microsatellite instability—  MSI. As a result of this after the cell division by duplication.  mismatched genes develop. These genes cannot be repaired.  This predisposes to mutation, results in a cancer gene.    Familial polyposis coli accounts for about 1% of colorectal  cancers. However, incidence of malignancy is 100%.  Gardner’s and Turcot’s syndrome are the variants of FPC.    HNPCC accounts for about 5 to 10% of colorectal cancers.  They also have extracolonic cancers such as endometrial,  ovarian and urinary bladder cancers.    Other familial syndromes are Cronkhite Canada  syndrome. Itis more common in females. Multiple polyps  develop in stomach, duodenum and in the colon. Diarrhoea  is the clinical presentation. Other features include  pigmentation, alopecia, loss of weight and cachexia.  Chances of developing malignancy is about 15%.    Polyps: Environmental and genetic factors favour the  development of colonic polyps and their transformation  into malignancy. The incidence of malignancy is increased  when the polyp is more than 1 cm, polyps are multiple or  flat (Table 29.2).    NPCC 80% chances  Icerative colitis 10-20% after 20 years  rohn’s colitis 5% after 10-20 years    size  Malignant potential Features/syndrome  1-10% Hypokalaemia, diarrhoea  Negligible Peutz-Jeghers syndrome  100% Gardner’s and Turcot’s syndrome  Nil Asymptomatic    A. Ulcerative colitis is a definite precancerous condition.  Presence of dysplasia diagnosed by colonoscopic biopsy  is an indication for colectomy.   B. Crohn’s involving colon also has a mildly increased risk  of developing carcinoma when compared to ulcerative  colitis.   C.Schistosomal colitis: The risk of colorectal cancer 1s  increased in patients with long-standing schistosomal  colitis. Long-standing cases are associated with mild to  severe grades of colonic epithelial dysplasia. Thus ulcers  or pseudo-polyps can occur. These dysplastic changes are  considered as premalignant.   D. Radiation exposure: Usually it is mucin secreting  adenocarcinoma with poor prognosis.    1. SAD factors: It 1s sad to know that SAD factors are  responsible for carcinoma colon. They are S—Smoking,  A—Alcohol, D—Dietary factors. Diet rich in red meat has  high animal fat. This alters intestinal bacteria, which convert  primary bile acids into secondary bile acids. This is the  beginning of formation of carcinogenic polycyclic  aromatic compounds. After cholecystectomy, there 1s  increase in free bile acid concentration, thus increasing the  risk of colonic cancer. Thus increased roughage is associated  with increased transit time which in turn reduces exposure  of mucosa to carcinogens.    ¢ Increase incidence 1s found in western countries wherein  diet rich in animal fat is consumed in large quantity.  Incidence is more after the age of 50 years. Obesity, poor  exercise and smoking are the contributing factors.    ¢ Women with breast cancer have increased incidence of  colonic cancer.    Calcium salts are protective. They form insoluble bile  salt complexes, thus reducing the concentration of bile  acids in the colon.   A. Annular stricture: Common in left colon (splenic  flexure, pelvic colon).   B. Tubular stricture: Common in left colon and at the  rectosigmoid junction.   Figs 29.17A to C: (A) Carcinoma caecum, (B) Carcinoma cae-  cum with secondaries in the liver, (C) Carcinoma left colon with  intestinal obstruction   Fig. 29.18: Large secondary in the liver. Look at the umbilication  (central necrosis)   ¢ Occasionally, growth at pelvirectal junction can be felt  on rectal examination.   ¢ However, on left-sided constrictive lesions, growth is  not often felt. It is the hard faecal matter and lymph nodes  which are felt as a mass.    to liver (mucoid adenocarcinoma), ascites, etc. Distant  metastasis is not common (Fig. 29.18).    may be due to blood loss ora proliferative growth secreting  toxins causing suppression of bone marow. Asthaenia and  anorexia are the other features.    pain is due to chronic obstruction as in left-sided growths  (napkin ring stricture).    increase in the dose of laxatives followed by attacks of  diarrhoea can be due to carcinoma colon. Diarrhoea is due  to hard faecal balls, irritating the colonic mucosa resulting  in increased secretion of mucus produced by proximal  colon.    lesions (Fig. 29.19). On the left side, diameter of the colon  is narrow, contents are solid and growth 1s constrictive.  Lower abdominal distension, right to left peristalsis are the    'Late Padmashree Dr TMA Pai, was the founder of Kasturba Medical  College which he started in 1953. He was also a banker, educationist.    e Can present as acute appendicitis when the lumen of  appendix is obstructed (in elderly patients).    e |t is a cause of intussusception (Secondary)  e Can present with flexion of hip due to infiltration of iliopsoas.    ¢ Early cases: It can be easily missed—such as change in e |Itcan present as abscess in the lateral abdominal wall.  bowel habits like diarrhoea, vague ill health, weakness (due  to anaemia), intermittent bleeding per rectum, often PEARLS OF WISDOM  attributed to piles or some other cause. Mass Is usually not   ¢ Late cases: It can present with obstruction (rectosigmoid Eee Berton’  junction growth), perforation, intussusception (right-sided  tumours), mass abdomen, secondaries in the liver, left Spread  supraclavicular nodes (Troisier’s sign), etc. 1. Local: For a long time, the lesion is confined to mucosa  See Key Boxes 29.7 to 29.9. and submucosa. They grow in annular fashion and later  Carcinoma right colon and carcinoma left colon  Carcinoma right colon Carcinoma left colon  ¢ Presentation Unexplained weakness, anaemia Change in bowel habits  ¢ Bleeding Occult blood in stools Gross blood in stools  ¢ Abdominal discomfort Right side and dyspeptic symptoms also Constipation and obstruction  ¢ Incidence More common in women More common in men  ¢ Frequency About 10-20% About 60—70% (including rectum)  ¢ Pathology Ulcerative/proliferative lesion Strictures (rectosigmoid)  ¢ Investigation Colonoscopy Flexible sigmoidoscopy  ¢ Complication Obstruction—less common Obstruction, perforation, pericolic    longitudinally. Once serosa is involved, spread occurs  rapidly into neighbouring structures such as ureter, bladder,  uterus, etc. The involvement of these structures is not a  contraindication for surgery (TNM staging).    . Lymphatic spread (see page 702)  . Blood spread: It occurs late, resulting in secondaries in    the lungs, liver, etc. Cannon ball in lung nodule in the liver.    Because of the drainage into the portal system, colonic  cancers spread to the liver first. On the other hand, rectal  cancers spread to the lungs because of drainage into  inferior vena cava.    There are many classifications and staging for carcinoma  colon/rectum. They are not important. A few important ones  have been given here.    ¢ Stage A: Invasion of but not breaching the muscularis  propria.   e Stage B: Breaching the muscularis propria but not  involving the lymph nodes.   ¢ Stage C: Lymph nodes are involved  Few authors describe a stage D for metastatic disease.  Since it has not been described by Duke, it is called  modified Dukes’ staging.    A few clinical photograph, staging pictures and operative  pictures have been given in next page.    Mla _ Spread confined to one organ or site—liver/lung/ovary/  nonregional nodes  Mlb Spread to more than one organ or site/peritoneum   I1]IB—T3-4a N1-lc MO; T2-3 N2a MO; T1-2 N2b MO  IC—T4a N2a MO0;T3-4a N2b MO;T4b NI-2 MO  IVA—Any T any N Mla   Fig. 29.22: A 34-year-old lady presented to the hospital with mass in the right iliac fossa with slight flexion of the right hip. Mass was  hard and irregular. She also had anaemia Fig. 29.23: Exploration of the mass (caecum). It was mobile Fig. 29.24: Right hemicolec-  tomy specimen—a case of carcinoma caecum   Fig. 29.28: Intestinal obstruction due to rectosigmoid stricture—  a common complication. Tumour rarely goes beyond 2 cm from  the edge of the tumour unless there is concomitant spread to  lymph nodes   Fig. 29.27: Rectosigmoid stricture. Patient underwent high ante-  rior resection. 5 cm proximal margin is enough for radical cure    Complications (Fig. 29.30)  1. Intestinal obstruction  2. Pericolic abscess: Pain is present in the tumour site and  may radiate to back, leg or hip as in caecal perforations. It  is due to irritation of the psoas muscles or due to irritation  of femoral nerve.  ¢ Diagnosis is confirmed by  ultrasound/CT scan.  ¢ Percutaneous aspiration,  followed by elective resec-  tion is the best treatment.  3. Faecal fistula (Fig. 29.31)  ¢ Pericolic abscess when it  is incised or drained to the  exterior may result in  faecal fistula if there is  malignancy.   4. Internal fistula: Colovesical, colocolic, coloenteric are not  uncommon complications of malignancies. They are  managed by resection. However, preoperative assessment  of fistulae by investigations should be done.    Involvement of local structures is not a contraindication  for radical resection.    3. Double contrast barium enema may show irregular filling  defect—intrinsic, persistent. It may also show an apple core  deformity (Figs 29.33 and 29.34 and Key Box 29.10).    e e ® ? @ e e e e e e e e e e e e e e e e e e e e e e e    . Stool: Occult blood positive nodes 3. CT is also done to know  3. Liver function tests, renal function 2. CT scan local and distant spread  tests and blood sugar estimation ~ ¢ Objective, more precise about mass, 4. PET scan: In follow-up of cases |   Figs 29.35A and B: Colonoscopy shows growth and biopsy is  being taken (Courtesy: Dr Filipe Alvares, Consultant, Medical  Gastroenterologist, KMC, Manipal)    4. Flexible sigmoidoscopy: 60 cm of colon can be visualised.  It is an outpatient procedure. It 1s indicated in rectal  bleeding. An enema is given before the procedure.   5. Colonoscopy is done to take a biopsy from growth and  also to rule out synchronous malignancy as seen in 5% of  the cases (more than one malignancy at the time of  diagnosis). If biopsy cannot be taken, as in obstruction,  brush cytology can be taken (Fig. 29.35).   ¢ Virtual colonoscopy can pick up polyp of 6 mm size also  but biopsy cannot be taken.   6. Ultrasound: It is the baseline investigation to be done first.   ¢ It can detect hydronephrosis, liver metastasis, ascites, para-  aortic nodes.   ¢ Definitely it cannot pick up early mucosal or lesions which  have penetrated up to muscularis mucosa or serosa.   ¢ Other than a biopsy, it has all the advantages and it is the  investigation of choice after colonoscopy.   ¢ Other associated diseases specially in elderly such as aortic  aneurysm, gall stones, hiatus hernia, etc.   ¢ 90% and 95% sensitivity and specificity in detecting liver  lesions greater than 1 cm.   ¢ It is a foetal glycoprotein, not present in normal human  beings (minute quantities). It 1s present in the cell  membranes of many tissues including colorectal cancer.   ¢ It has a prognostic rather than a diagnostic value. After  treatment of the primary, CEA level should come back to  normal. If it is increased, it suggests either recurrent tumour  or secondaries in the liver.    e Produced by colorectal epithelium but cleared by Kupffer  cells of liver. Its half-life is prolonged (normal—10 days) in  cholestasis and hepatocellular dysfunction.    e Significant increase in the levels is also found in pancreatic  carcinoma, gastric carcinoma, lung carcinoma, breast  carcinoma.    e Increased CEA in the follow-up period of colorectal cancer  suggests metastasis. PET scan may help in these patients  when CT/US are normal.    e If preoperative CEA is increased in node-negative colonic  cancer, chemotherapy is recommended.   ¢ Routine use of PET—positron emission tomographic  scanning in the primary management of colorectal cancer  is not recommended.   ¢ It is useful in the follow-up cases wherein CEA levels are  increasing and the actual cause for the rise 1s being  evaluated.   ¢ Chest X-ray: Colonic carcinoma spreads more often to  lungs than carcinoma of stomach giving rise to cannon ball  secondaries.    ¢ Spread: If it is limited to mucosa and there are no nodes,  5-year survival 1s 90-100%.   ¢ Obstruction and perforation: Poor prognosis due to  dissemination of malignant cells.    Blood transfusion increases number of suppressor  T-lymphocytes thus causing tmmunosuppression. Hence,  follow bloodless surgery or autologous blood transfusion.    1. Mechanical bowel preparation is necessary to reduce the  bacterial load in the colon. This reduces incidence of  anastomotic leakage.  ¢ Whole gut irrigation by oral polyethylene glycol is   found to be superior than enemas. It is the method of  choice today. It 1s mixed with 2 litres of water and Is  given 12 hours before surgery.    2. Antibiotics: Oral antibiotics neomycin/metronidazole or  neomycin/erythromycin are given in the afternoon and  evening before surgery. They act locally and are given  mainly to decrease wound infection.    Intravenous antibiotics such as ciprofloxacin and metro-  nidazole or 3rd generation cephalosporins such as ceftria-  xone 1s given one hour before the surgery. The target  bacteria are EF. coli and Bacillus fragilis.    3. Improvement of general condition by correcting albumin  levels and if necessary, TPN will definitely decrease the  incidence of anastomotic leakage.    4. A fat-free diet, low residue diet is prescribed two to three  days before surgery.    5S. Prophylactic fractionated heparin enoxaparin or  dalteparin is given subcutaneously, to prevent deep vein  thrombosis. Otherwise subcutaneous heparin is given.    Colonic surgery is a very common problem encountered in  the practice or institution. Every surgeon should be aware of  basic principles of colonic surgery and technique of  anastomosis. I have grouped them together under the heading  of ten commandments so as to understand better.    Over a period of years, radical resections for carcinoma of  colon have become less radical pertaining to the extent of  bowel resection. For example: One need not remove terminal  30 cm of ileum for carcinoma caecum today. Only 6—8 cm of  ileum removal is sufficient in a right hemicolectomy.    2. Should give an adequate incision.  3. Should explore the peritoneal cavity for metastasis.    7. Should ensure the cut ends bleed well before anastomosis.  8. Should ensure there is no tension at the suture line.    1. Carcinoma right colon including caecum: If it is  operable, the treatment is right radical hemicolectomy.  Structures removed in this operation are (Fig. 29.36 and  Key Box 29.12).   ¢ Fat, fascia, lymphatics and lymph nodes like tleocolic  nodes, pericolic nodes, nodes at the original of SMA.   ¢ If the growth is fixed to posterior abdominal wall,  common iliac vessels, palliative ileotransverse  anastomosis is done (ileotransverse colostomy).    2. Carcinoma transverse colon—‘V’ resection. The area  supplied by middle colic artery is removed followed by  end to end anastomosis. The patient may need removal of  entire transverse colon depending upon lesion.  ¢ When lesion is at hepatic flexure or in the transverse    3. Carcinoma left colon—left radical hemicolectomy.  ¢ Left half of the transverse colon and descending colon  are removed followed by anastomosis of transverse colon  to sigmoid colon—This 1s the area supplied by left colic  artery (Fig. 29.37).    STRUCTURES THAT CAN GET INJURED  DURING RIGHT HEMICOLECTOMY  ¢ Duodenum  e Ureter  ¢ Gonadal vessels    'Removal of a foot of ileum is not necessary unless it has a doubtful   Fig. 29.37: Left radical hemicolectomy including removal of the  spleen as the growth was infiltrating the spleen—routine removal  of spleen should not be done    4. Carcinoma sigmoid colon—radical sigmoid colectomy  followed by anastomosis of descending colon to the rectum  (colorectal anastomosis). Or in a few cases, left hemico-  lectomy may have to be done.    5. Left-sided colonic tumours with intestinal obstruction—  an emergency temporary transverse colostomy is done to  divert the faecal matter and to relieve intestinal obstruction  (for more details on colostomy see page 785). Resection 1s  not done because many patients are elderly with comorbid  illness such as diabetes, hypertension, cardiac illness.  ¢ General condition of the patient 1s poor with gross  abdominal distension and dehydration.   ¢ Left colon is loaded with faecal matter. Hence, high  chances of anastomotic leakage and faecal peritonitis are  present.   ¢ After 2 weeks, laparotomy is done once again. The  primary tumour is resected and end-to-end anastomosis  done.   ¢ This is followed by closure of the colostomy 8 weeks  later—three-stage operation.   ¢ Single-stage resection can also be done provided,  thorough colonic irrigation through appendicular stump  (after appendicectomy), is given and it should be  successful in cleaning the entire colon. This is the concept  of on-table irrigation and lavage.    In cases of left-sided colonic obstructions—classical example  being carcinoma rectosigmoid with obstruction.   ¢ Resection is done first. Clamps are applied to both ends.   ¢ Appendicectomy ts done and purse string suture applied  but not tied.   ¢ Through the appendicular stump lumen, a 30 Fr Foley  catheter is passed into colon.    ¢ The position of the catheter 1s checked to be lying safely in  the caecum and its balloon is inflated. A purse string suture,  previously applied to the appendix base is tied.   e Saline is irrigated at the rate of 50 to 100 ml per hour speed.  Proximal clamp is opened into a container (kidney tray).   ¢ It takes about an hour or so for the whole gut irrigation.    It avoids a stoma, decreases stay in the hospital and thus less  expensive.    Once anastomosis is completed, Foley catheter 1s brought out  through an opening in the abdominal wall and connected to a  bag. It is sutured to the inside of the parietes by vicryl sutures  and kept open.    Tube is removed after 7-10 days provided there is no leak  from the suture line.   ¢ Nasogastric tube is not required unless operating on  obstructed colons. Evidence is that the sutures /staplers are  good enough to prevent the leaks.   ¢ Laparoscopy has the definite advantage of small incisions,  less pain, easy breathing and less respiratory complications.   ¢ Early mobilization, early feeds, early recovery and early  removal of catheters.   ¢ Few diagrammatic representation of the colectomies are  shown in the next page (Figs 29.39 to 29.44).    Figs 29.39A and B: Right hemicolectomy followed by end-to-end Figs 29.40A and B: Palliative ileotransverse anastomosis  anastomosis—ileocolic inoperable case   Figs 29.41A and B: Extended right hemicolectomy—growth in Figs 29.42A and B: Transverse colectomy followed by colocolic  the hepatic flexure anastomosis   Figs 29.43A and B: Left hemicolectomy for growth inthe splenic | Figs 29.44A and B: Resection of rectosigmoid growth followed  flexure by colorectal anastomosis    Figs 29.39 to 29.44: All figures are contributed by Ms Vidushi Kapil, MBBS student, KMC, Manipal    |. pT l-2NOMO do not require any adjuvant treatment, such  patients can be kept on follow-up with routine 3 monthly  CEA and annual CECT thorax/abdomen/pelvis.   2. p!3NOMO or node positive disease requires adjuvant  treatment in the form of concurrent chemoradiotherapy and  chemotherapy. 2 cylces of FOLFOX (5-FU + Leucovorin  + Oxaliplatin) ~Concurrent 5-FU/Leucovorin and radiation  — 2 more cycles of FOLFOX.   3. It is preferable to add Oxaliplatin in the chemotherapy  regimen if nodes are positive for metastatic disease. In older  population (> 65—70 years), it might of less benefit.   4. Oxaloplatins have been shown to down size liver metastasis.  Chief complications of Oxaliplatins is peripheral  neuropathy.    Adenocarcinoma colon does not respond well to radiation.  Routinely it ts not given. Surgery remains the gold standard    Terminal ileum to midtransverse colon  Terminal ileum to midtransverse colon  Terminal ileum to descending colon    for carcinoma colon. Soft tissue infiltration into psoas muscle  or abdominal wall or inoperable recurrent tumours are  indications for radiotherapy.    Patients with isolated liver/lung secondaries should also  undergo treatment with a radical approach as even in these  cases with resection of the primary and adequate liver/lung  resection, a good disease control can be achieved.   1. A typical course of neo-adjuvant therapy comprises  concurrent 5-FU/Capecitabine and radiation in cases of  large lesions abutting the abdominal wall or down into the  pelvis. A dose of 45-50 Gy is used to treat the pelvis  including the growth and the draining lymphatic regions  followed by 5 Gy boost to the tumour itself.   2. Following neo-adjuvant therapy, patient should be re-  evaluated using CT/MRI for possibility of resection.   3. Surgery is usually considered after 6—8 weeks following  neo-adjuvant therapy as the maximal response to the  treatment may take up to 2 months.   4. Further adjuvant treatment 1s to be given following surgery  depending upon the histopathological report.    ¢ CT scan and PET scan are done to evaluate local/systemic  disease. Provided there 1s no systemic spread, liver secon-  daries have to be treated aggressively. Pattern of recurrence  in colonic carcinomas Is more commonly distant, that is they  tend to recur more commonly at distant sites such as liver,  and lungs. As aresult, systemic treatment is more necessary.   ¢ Liver directed therapies such as hepatic arterial chemo-  therapy infusion/embolisation, radiofrequency-ablation,  radiotherapy should be used in treatment of isolated liver  metastasis.   =k a =10) Gee! Neg :  INDICATION FOR RESECTION OF  LIVER METASTASIS  e Solitary metastasis or metastasis confined to one lobe.  e <3 metastasis in both lobes  e Absence of extrahepatic disease    1. Bevacizumab—anti-VEGF (vascular endothelial growth  factor) monoclonal antibody. It has anti-angiogenesis  property thereby controlling the tumour growth.    Most of the colonic cases are curable 1f diagnosed and treated  early. Also metachronous lesion can occur in the rest of the  colon. Hence, certain tests are necessary during follow-up.    e Recurrence or metastasis is suspected during follow-up by  abnormal values of investigation.   ¢ Recurrent tumour should be resected en bloc—it may  amount to a more radical procedure including resection of  duodenum, liver, kidney (Figs 29.45 and 29.46)    e e e e e e e e e e e e e ® e e e e e e e e e e e e e e e   2. Colonoscopy 6 months after surgery, Ss  later once in a year    1. Folic acid: It 1s an important vitamin with many functions.  In the absence of folic acid, hypomethylation can occur.  As aresult of this overexpression of proto-oncogenes such  as K-ras and c-Myc can occur. Deficiency of folic acid  causes imbalances in the nucleotide pool leading to DNA  break and mutation. Thus folic acid supplementation should  be given in adenoma specially when baseline levels of folic  acid 1s low.    2. Dietary fibres: Fibres decrease the transit time, they dilut  the carcinogens and are used to present development o  cancer. Cellulose, hemicelluloses and pectin are a few  examples. Fibres also produce short chain in fatty acid:  causing fermentation by faecal flora. Thus colonic pF  becomes more acidic which 1n turn inhibits carcinogenesis    3. Aspirin, calcium, and sulindac also have been used tc  prevent cancer developing in an adenoma.    ¢ Large bowel 1s the 4th most common site for cancer after  lung, stomach and breast    ¢ More common in North America, North Europe and  Australia. Lowest rates in Africa, India.    ¢ 75% of CRC develop in people with no known risk factors  apart from older age.    Figs 29.45 and 29.46: A colorectal cancer patient who was  operated 3 years back for carcinoma caecum presented with  recurrence in the abdominal wall. He underwent excision of the  tumour. He did not have any other site of metastasis. Postoperative  radiotherapy was given    ¢ It 1s guaiac test which will detect elevated level of blood  in stool. It requires 2 samples from each of 3 consecutive  stools which are smeared onto cards.   ¢ False positive: Vegetables, fruits, red meat, aspirin or  any other bleeding lesion proximal to colon screening,  FOBT has shown to decrease mortality by 20 to 30%.    ¢ Reduces incidence and mortality of distal CRC by around  60%.    ¢ Should be done if there is distal adenoma (chances of  proximal adenoma are high).   ¢ 70% of alladvanced colorectal neoplasia will be detected  with this strategy.    ¢ [tis an alternative but not yet become popular because  of time, cost and preparation.    It is an acquired condition, in which colonic mucosa herniates  through the circular muscle fibres at weak points, where blood  vessels penetrate the colonic wall. Since it is acquired it lacks  the muscle coat. They are thin, more prone for infections and  perforation. Hence, they are termed pseudodiverticull.    ¢ The disease 1s common in western population wherein diet  is very poor in fibres because of refining of sugar and flour.  Nonstarch polysaccharides (NSP) or low dietary fibres  are the chief factor for diverticulosis of colon (Key Box  29.16).    ¢ Itrequires very high pressure for propulsion of faecal matter  and this ts believed to cause characteristic thickening of  muscles and herniation of mucosa (Figs 29.47 and 29.48).   ¢ In Africans and Indians, the disease is rare because of high  fibre content of the diet.   ¢ The disease starts after the age of 40. Any stress or emotional  disorders may add to the constipation already caused by  dietary factors and result in diverticular formation.   ¢ 90% of them affect sigmoid colon. Rectum is spared in  majority. Rarely, it affects right colon.    ¢ Diverticulae project between antimesenteric and mesenteric  borders with taenia but they never penetrate taenia  (Fig. 29.49).   ¢ There is muscle hypertrophy, which project into the lumen  as obstructive folds. The mucosa is essentially normal.  Slowly, luminal diameter 1s narrowed.   ¢ Inflammation occurs in the pericolonic tissue with or  without abscess formation.   ¢ Thickening (neither hypertrophy nor hyperplasia) of the  circular muscle layer.   ° Collagen—connective-tissue diseases such as Ehlers-  Danlos syndrome, Marfan’s syndrome, and autosomal-  dominant polycystic kidney disease result in structural  changes in the bowel wall, leading to decreased resistance  of the wall to intraluminal pressures and thus allowing  protrusion of diverticula.    1. Diverticulosis: It refers to presence of diverticulosis  without much symptoms. But on careful questioning,  patients do have lower abdominal distention, heaviness,  flatulence, etc. Vague abdominal pain 1s also felt in the left  iliac fossa.  2. Diverticulitis: Left-sided lower abdominal pain, moderate  to severe is associated with passage of loose stools. The  pain Is partially relieved on passing flatus.  ¢ Bleeding per rectum can be the presenting feature,  sometimes it can be massive.   ¢ Low-grade fever, tenderness, rigidity and even mass may  be present in the left iliac fossa (like left-sided appendi-  citis). The mass is thickened, inflamed, tender and  sigmoid. Such attacks result in abscess which rupture  into hollow organs and give rise to fistulae (Fig. 29.50).   Fig. 29.50: Perforated diverticula at surgery—resected specimen  (Courtesy: Dr Ramesh Rajan, Surgical Gastroenterologist,  Trivandrum Medical College, Kerala)    3. Internal fistulae: Colovesical fistulae give rise to  pneumaturia (flatus in the urine) and rarely faeces in the  urine (Key Box 29.17).   I]. Walled off 3 pelvic abscess  III. Generalised purulent peritonitis  IV. Generalised faecal peritonitis    Diverticulosis of the colon is one ~  of the common causes of massive    Hinchey stages | and I] may be treated by sigmoid  colectomy and primary anastomosis (a one-stage operation).   Hinchey stages II] and IV are treated by sigmoid colectomy  followed by end-colostomy and Hartmann pouch.    The most common fistula in acute diverticulitis is  colovesical followed by colovaginal fistula.    1. Sigmoidoscopy: Mucosa may be normal or may show  erythematous and oedematous changes. Ulcers are absent.  Opening of diverticulae can be seen.  2. Barium enema: Contraindicated in acute cases.  ¢ It may show saw-tooth appearance due to muscle  hypertrophy.   ¢ Champagne glass sign: Partial filling of diverticula by  barium with stercolith inside the diverticula.    3. Colonoscopy to confirm the findings and to rule out  carcinoma colon (Figs 29.53 and 29.54).  PEARLS OF WISDOM    4. Ultrasound and CT scan are the investigations of choice in    1. Massive haemorrhage per rectum: Haemorrhage 1s due  to vessels in the base of diverticulae, more so in  atherosclerotic or hypertensive patients.   Fig. 29.55: CT scanshowing pericolic abscess on the left paracolic  space    inflammatory adhesions and abscess formation which  ruptures resulting in fistula. Thus, colovesical, colovaginal,  colointestinal fistula can occur.    Stage of diverticulosis or in those patients who have  recovered from one attack of diverticulitis.   ¢ High fibre diet, optimal amount of daily fibre is unknown.  ¢ 20 to 30 g per day is a widely recommended.   A. Hartmann’s procedure is the choice: Sigmoid colon  is resected, end-colostomy is done by using descending  colon followed by closure of rectal stump (Figs 29.57A  and B).  ¢ After 4-6 weeks, colorectal anastomosis is done.   B. However, if a perforation is small and the general  condition is good after the resection, the colon 1s irrigated  with 8—10 litres of saline till the contents are clear. This  is followed by colorectal anastomosis in the same sitting.    Abscess depends on the ability of the pericolic tissues to  localise the spread of the inflammatory process.  Intra-abdominal abscesses are formed by—anastomotic  leakage: 35%—diverticular disease: 23%.   Limited spread of the perforation forms an inflammatory  phlegmon, while further (but still localised) progression  creates an abscess.   Signs and symptoms: High grade fever with or without  leukocytosis despite adequate antibiotics, tender mass.  Treatment:    and conservative management alone. Often it is retrocolic.  May present as swelling in the loin.    choice for small, simple, well-defined collections. 100%  success in simple unilocular abscesses.   . Open drainage: In cases of multilocular collection,  abscesses associated with enteric fistulas, and abscesses  containing solid material. Drainage of pus, resection,  Hartmann’s operation is ideal choice. Closure of the  colostomy after 6—8 weeks is done.    4. Treatment of fistulae: As an elective procedure, with good  preparation, after confirming the site of fistula, resection  of the sigmoid colon with closure of fistula can be done.   TYPES OF FISTULA RELATED TO  DIVERTICULAR DISEASE  e Colovesical: 65%  e Colovaginal: 25%  e Colocutaneous  e Coloentero    Depending upon the nature of the disease or a surgical  procedure.    I. Primary or type I fistula: It develops as a result of an  underlying disease affecting gut. Example: Carcinoma  colon infiltrating urinary bladder resulting in colovesical  fistula or rupture through the skin resulting 1n pericolic  abscess, fistula.    II. Secondary or type II fistula: Occurs after injury to  otherwise normal gut. Examples: Left colonic/right colonic  injury in percutaneous nephrolithotomy (PCNL) for renal  stones or following anastomotic leak.   A. Lateral fistula: It occurs after a colocolic or ileocolic  anastomosis wherein a few sutures would have given way  in the immediate postoperative period.   B. End fistula: This means both ends of the intestines are  open following resection and anastomosis giving rise to  postoperative peritonitis.   ¢ Most common anaerobe is Bacteroides fragilis, with a  count of 10'%g of wet faeces. Clostridia, Lactobacillus are  other organisms.   ¢ E. coli is the predominant aerobic organism. Count is  | 0//g of faeces. Klebsiella, Proteus, Enterobacter are other  aerobes. Streptococcus faecalis is the principal  enterococcus.   ¢ Features of septic shock: Often patient may not complain  of abdominal pain but manifestations can be of renal failure,  tachycardia, tachypnoea and hypotension.   ¢ CECT can detect intraperitoneal collection, pneumo-  peritoneum, anastomotic leak. However, creatinine should  be normal before doing CT scan. Often patients are 1n sepsis  with renal failure.   ¢ Drainage of sepsis—laparotomy, resection and anastomosis,  ileostomy or colostomy may be necessary.   ¢ Lateral fistula may heal with total parenteral nutrition  provided there is no distal obstruction (TPN).   ¢ End fistula may require resuturing with or without proximal  diversion colostomy/ileostomy.°   Fig. 29.58: This patient underwent three surgical procedures for  treatment of fistula. Initial surgery was right hemicolectomy for  ileocaecal tuberculosis. Second surgery was resection and a  proximal diversion ileostomy because of the leak and finally after  two months, closure of ileostomy was done followed by  ileotransverse anastomosis   Fig. 29.60: A case of faecal fistula following ileal resection for  obstruction caused by a band. Following resection anastomosis  on the fifth postoperative day air mixed faecal matter started  coming out from the wound. CT scanrevealed small lateral fistula.  The patient responded to conservative treatment with TPN given  for fourteen days. Fistula subsided   4. Inflammatory bowel disease: Any ulcers, including amoebic,  may heal with fibrosis resulting in stricture.   7. Endometriomas: Ectopic endometriosis tissue responds to  cyclic hormonal stimulation causing inflammation and  fibrosis.    ¢ Single-stage resection and end-to-end anastomosis.  ¢ Treatment of the cause   C. Adenoma smaller than 15 mm in diameter do not carry  the risk of malignant potential    . Flexion of the hip can be present in:  A. Carcinoma splenic flexure    . Abscess in the lateral abdominal wall can be a feature  of:    . Mechanical bowel preparation is best given using:  A. Plenty of saline    . Which one of this is true in colonic surgery?  A. No touch technique of Turnbull   B. Right-sided lesions are treated by colostomy  C. Left-sided lesions are treated by resection   C. Survival is good if it is limited to mucosa and there are  no nodes   D. Detection of all advanced colorectal neoplasia is only  up to 20%    In which of the following malignancy anaemia is an  important method of presentation?    Which of the following diverticula is true diverticula?  A. Sigmoid diverticull    In hereditary nonpolyposis colorectal cancer, which  carcinoma is more often seen?   C. It should be done in all cases of carcinoma colon before  surgery   ANSWERS  1B 2D 3 B 4 D 5 D 6 A  11 D WZ D 13 A 14 B is ii6. BD    The most common aerobic organism present in a  sigmoid colonic faecal fistula is:    ¢ Intestinal obstruction is a challenging surgical emergency  encountered by general surgeons. This can affect any age  group starting from neonate to an old man. It can affect a  school going boy, working woman or a man during their  peak of life. Sometimes it can be fatal either due to delay  in the diagnosis, delay in the treatment or complications  related to surgery. Abdomen 1s a Pandora’s box. Sometimes,  it is difficult to pinpoint the cause of obstruction.   ¢ Adhesions and hernia are the two most common causes of  intestinal obstruction. Adhesions are more common than  hernias nowadays. Laparoscopic surgery has definitely  decreased incidence of adhesions. In Western countries  more than 50% cases of intestinal obstruction are due to  adhesions and only 10—15% are due to obstructed hernia  (Fig. 30.1). However, students should be able to diagnose  intestinal obstruction, resuscitate the patients and refer the  patient for further surgical treatment. With the availability  of sophisticated investigations such as CT scan, diagnosis  can be established in majority of cases before surgery.  However, in other cases, ‘exploratory laparotomy’ will  give the diagnosis.    intestinal obstruction. It 1s the most common surgical disorder  (emergency) of the intestines.    Few important facts about intestinal obstruction:  ¢ 80% occur in small bowel    ¢ 20% occur in large bowel  ¢ Majority (more than 80%) of small bowel obstructions are  benign in nature.  In the large bowel, more than 70% of colonic obstruction  is due to malignancy—others being inflammatory bowel  diseases, ileocaecal tuberculosis, volvulus, etc.   Fig. 30.1: Obstructed incisional hernia (Courtesy: Dr CG  Narasimhan, Senior Consultant Surgeon, Mysore, Karnataka)    e Mechanical obstruction  There is a physical barrier which prevents the abnormal  progress of intestinal contents.   e Paralytic ileus  There is no physical barrier but failure of peristalsis to  propel intestinal contents due to neurogenic causes.   e Strangulated obstruction  It refers to obstruction with impairment of blood supply to  the gut.   e Closed loop obstruction  In this condition, the intestine is occluded in two places.  More chances of gangrene and perforation are present,  e.g. volvulus.   C. Closed loop obstruction: It means both proximal and  distal ends are blocked. This occurs in carcinoma of  the right colon with constrictive lesions. If the ileocaecal  valve is competent and the obstruction ts total, the intra-  luminal pressure within the colon increases. As a result  of this, the caecum may perforate. Thus, closed loop  obstruction can be dangerous (Fig. 30.2). Another  example is sigmoid volvulus.   Fig. 30.2: Closed loop obstruction  in 40% of patients ileocaecal valve  is competent    Fig. 30.3: Dilated small  intestinal loops in a case of  ileal obstruction    A. Acute obstruction: Signs and symptoms appear very  early. Usually, it affects small bowel, obstructed hernia,  bands.    B. Chronic obstruction (e.g. carcinoma colon) affects  large bowel (colic comes first, distension later).  Diverticular disease also produces chronic obstruc-  tions (Fig. 30.3).   C. Acute on chronic obstruction develops in carcinoma  colon, wherein an acute obstruction suddenly results  due to the accumulation of faecal matter in the proximal  bowel (see Fig. 30.4 for various causes of intestinal  obstruction).    ¢ Asa result of obstruction, the proximal bowel undergoes  hyperperistalsis which 1s responsible for colicky pain  abdomen. The peristalsis may continue for a few days and  later the intestine may be paralysed and flaccid. After  3—4 hours, distal to the obstruction, all physiological  activities of the bowel are stopped. Intestine becomes  contracted, pale and does not exhibit peristalsis. After a  few hours, the proximal bowel gets dilated secondary to  obstruction.    ¢ Swallowed air (70%). Because of colic and anxiety, the  swallowed air is increased. Oxygen is absorbed and  nitrogen remains as it cannot be absorbed. This results  In distension.   ¢ Diffusion of air from the blood into bowel lumen  Increases carbon dioxide which diffuses very rapidly.   Normally, all this fluid is absorbed in the bowel. In cases  of intestinal obstruction, this fluid absorption is delayed. It  accumulates in the intestinal loop. Excretion of water and  electrolytes into the lumen ts also increased.    Activated neutrophils and macrophages accumulate within the  muscular layer of the bowel wall due to dilatation and  inflammation of the bowel wall. This damages the secretory  and motor processes by release of reactive proteolytic enzymes  and cytokines. Net result is increase in the local release of  nitric oxide, itself a potent inhibitor of smooth muscle tone.  It further aggravates the intestinal dilatation.    ¢ Bacterial colony count increases following obstruction  resulting in stasis. From less than 10° in jejunum and  from 10° in ileum, counts Increase.   ¢ Bacterial translocation can occur even in simple obstruc-  tion without strangulation. Thus, bacteria can enter into  lymph nodes and into systemic circulation. Abdominal  distension, hypovolaemia, renal failure and sepsis set in.  In addition to these changes, diaphragm gets elevated,  respiration 1s impaired which result in respiratory  complications such as atelectasis and basal pneumonia.    ¢ In doubtful cases of viability, if facilities are available, a  test called fluorescein test can be done. 1000 mg of  fluorescein is injected into peripheral vein and bowel is  inspected under Wood light. If loops are nonviable,  resection and anastomosis 1s done.    ¢ Interference with blood supply: As the tension within the  loops becomes more and more, venous congestion takes  place resulting in oedema of the bowel wall.   ¢ If the obstruction 1s not relieved, capillary rupture and  haemorrhage into bowel may ensue. In cases of volvulus  and intussusception, the arterial supply gets compro-  mised rapidly causing gangrene of bowel wall very early.  Bacterial proliferation takes place and endotoxins are  released.   ¢ Transmigration (translocation) of gram-negative  organisms, anaerobes and gram-positive organisms through  the gangrenous bowel results in peritonitis.   e The organisms release powerful endotoxins which are  absorbed from the peritoneal surface and cause gram-negative  shock or septic shock. It carries very high mortality rate (30%).   ¢ Loss of blood volume is an important feature of massive  gangrene.   FACTORS PREDISPOSING ISCHAEMIA  e Volvulus  e Mesenteric ischaemia  e Necrotising enterocolitis  e Intussusception  e Progressive distension  e Extrinsic compression by adhesions, bands, etc.    1. Pain abdomen: Central abdominal pain 1s a feature of small  intestinal obstruction and peripheral pain is a feature of  large intestinal obstruction. The pain is colicky in nature,  lasts for 5-10 minutes and is intermittent. On pressure, it  decreases.   2. Vomiting is due to reverse peristalsis. Vomitus consists of  stomach contents initially, then bile, followed by faeculent  matter. Faeculent is not faecal matter but terminal ileal  contents which undergo bacterial degradation and  fermentation resulting in the smell of faecal matter.  Vomiting of altered blood indicates haemorrhage and  gangrene. Frequent vomiting reflects jejunal obstruction  (Fig. 30.7 and Table 30.1).   CARDINAL FEATURES OF  INTESTINAL OBSTRUCTION  e Colicky abdominal pain  e Abdominal distension  e Vomiting  e Absolute constipation    distension as seen in ileal obstruction, peripheral abdominal  as in large bowel obstruction, or localised to one or two  quadrants as in sigmoid volvulus.    General signs of dehydration such as dry skin, dry tongue,  sunken eyes, feeble pulse, low urinary output are seen.  Dehydration occurs due to persistent vomiting and  sequestration of fluid and electrolytes. Hypokalaemia 1s an  important finding.    ¢ Step ladder peristalsis is seen in terminal ileal obstruction.  Right to left colonic peristalsis is seen in left-sided  colonic obstruction, large bowel obstruction.    ¢ On auscultation—loud, noisy intestinal sounds are heard.  They are called borborygmi.    ¢ Hernial orifices have to be examined, especially for a  femoral hernia in females.    High (jejunum) Distal (ileum) Low (colon)  « Vomiting Frequent, bilious Moderate bilious, faeculent Late vomiting, faeculent  « Distension No Moderate distension Marked distension  * Pain Intermittent, not crescendo type _ Intermittent, crescendo type, colicky Variable pain, not classical crescendo  * Constipation Not initially Not initially Initially present    Features of septic shock—fever, hypothermia, renal failure,  respiratory failure (Key Box 30.5).    Absent bowel sounds because rest of the bowel loops  undergo paralytic ileus.    Sudden symptoms—spasmodic pain (due to peristalsis)  and continuous pain suggest strangulation (Fig. 30.11).    Features of strangulation and perforation occur quickly in cases  of closed loop obstruction (Figs 30.8 to 30.11 and Key Box 30.6).    This occurs when the bowel is obstructed at both proximal  and distal points.    Proximal bowel is not distended as much in this condition  Gangrene and perforation can occur fast    Retrograde thrombosis of mesenteric vein, can result in  distension of the bowel.    A few examples of closed loop obstruction include sigmoid  volvulus, strangulated hernia, carcinoma right colon.    In small bowel obstruction, rectum is empty and Is often  ballooned out.    The small intestine 1s considered dilated if loops of bowel  measure more than 3 cm in diameter. Measurements for  the large bowel vary among different anatomic segments,  with a relative threshold of 9 cm in diameter for the  proximal colon and 5 cm for the sigmoid colon.    Complete blood picture: Low Hb% indicates underlying  malignancy. Increased total WBC count indicates infection  and sepsis (perforation).    intestinal obstruction and require correction preoperatively.  Strangulation may be associated with deranged potassium,  amylase or lactic dehydrogenase.   Plain X-ray abdomen in the erect position may show  multiple gas fluid levels. Gas levels appear earlier than fluid  level. Normally, two insignificant fluid levels can be  present, one in the terminal ileum and one in the first part  of the duodenum (Key Box 30.7). Supine films indicate  the distal limit of obstruction (Figs 30.12 to 30.16).    Enteroclysis is rarely performed in acute intestinal  obstruction but it has greater sensitivity in the detection  of partial small bowel obstruction (Fig. 30.17).    Jejunum is characterised by regularly placed mucosal folds  called valvulae conniventes (Fig. 30.14) placed opposite  to each other (Herring bone pattern). They are produced  by valves of Kerckring.    Large bowel is characterised by haustrations (Fig. 30.15):  Incomplete, large mucosal folds, not placed opposite to  each other.    Caecum has no haustrations. It appears as a round gas  shadow in the right iliac fossa.   Gas is absent in the small bowel as in mesenteric vascular  ischaemia.   Fig. 30.16: Plain X-ray abdomen supine—distended intestines.  Supine X-ray will indicate distal limit of obstruction    e Itis not the investigation of choice or it may not be required   e. Intussusception and can assess vascularity with the help  of duplex scan.   e lf bowel wall is thick and air is present (pneumatosis),  strangulation is likely.   e CT can detect mass lesions—carcinoma sigmoid, caecum  or ileocaecal mass (TB).   e CT has lowsensitivity in detecting low grade or partial small  bowel obstruction. Sensitivity increases in total obstruction.    Preoperative preparation includes correction of dehydration,  electrolytes and broad spectrum antibiotics. Principles in the  management of intestinal obstruction are as follows:    A. Aspiration with Ryle’s tube. This is the most important  step in the management of intestinal obstruction. It helps  in decreasing the distension and also prevents vomiting.  This will help in preventing respiratory complications, such  as aspiration following general anaesthesia.    C. Charts: Temperature, pulse, respiration and intake-output  chart. In cases of conservative management such as  obstruction due to adhesions, change in temperature and  increasing pulse rate suggests perforation or gangrene.  These cases have to be explored immediately.    E. Exploratory laparotomy is done and depending upon the  findings, obstruction is treated. A few examples are given  in Key Box 30.10 and Fig. 30.19.    F. Fluids should be given before, during and after surgery. It 2. Diagnosis of small bowel obstruction  forms the most important treatment of intestinal obstruction. at laparotomy  ¢ Caecum ts collapsed  ¢ Dilated loops of small intestine are present.  ¢ A stricture or a mass lesion may be obvious at laparotomy.    PRINCIPLES OF EXPLORATORY LAPAROTOMY 3. Diagnosis of simple obstruction  e Ideally done within 6—8 hours  e Long midline incision    ¢ A growth may be palpable and obvious in the transverse  colon or in the hidden colon, 1.e. splenic flexure.    ¢ Black, dark, foul-smelling bowel is seen as soon as laparo-  tomy 1s done.   No gangrene ¢ Precautions must be taken not to contaminate peritoneal    surgeons in treating an uncommon situation such as massive  ischaemia and gangrene of small bowel and colon (due to  mesenteric vascular occlusion), synchronous carcinoma and  ileosigmoid knotting, etc.    Ask the following questions to yourself and proceed. VIABLE BOWEL—FEATURES  1. What is the probable cause of obstruction? ¢ Normal peristalsis  2. Is it small bowel obstruction at laparotomy? ¢ Normal peritoneal sheen is present  3. Is it large bowel obstruction at laparotomy? ¢ Normal pulsations are visible or felt at the mesentery  4. Is it simple obstruction? e Normal pink colour is present  5. Is it strangulation?  6. Is it some kind of a surprise or a difficult case?  7. Can I manage conservatively?    ¢ A previous laparotomy scar may indicate that it could be  an adhesive obstruction (most common).   ¢ An obvious obstructed hernia (inguinal or femoral) can be  managed with inguinal approach.   ¢ An elderly man, hypertensive and atherosclerotic, with  features of blood 1n the stools and acute abdominal pain  may be having superior mesenteric ischaemia.   ¢ A constipated, elderly man in poor health, with acute or  chronic obstruction may be having carcinoma of the colon. Fig. 30.20: Viable and nonviable bowel    ¢ Surprises are well known tn intestinal obstruction. Congenital  bands, foreign bodies, internal herniation, lymphomatous  strictures are a few examples.    ¢ In these cases, a long intestinal tube called Miller-Abbott  tube can be passed to decompress intestines (Key Box 30.12).   1. Partial small bowel obstruction mostly due to adhesion:  Wait for 48 to 72 hours. They may show improvement. If not,  surgery is required.   2. Early postoperative obstruction: It rarely progresses to  strangulation. Hence, nonoperative management can be  extended to many days (3-7) provided there is no evidence  of peritonitis.   4. Carcinomatosis: Disseminated malignancy with obstruc-  tion. The aim is nonoperative treatment as nothing much  can be achieved with laparotomy.   ¢ In certain parts of India as mentioned above, it 1s one of  the common surgical emergencies in elderly population  (Key Box 30.13).    e 2/3 of the cases are sigmoid volvulus and 1/3 are caecal  volvulus.    e Itconsists about 10 to 15% of cases of intestinal obstruction  in India.    e More common in rural population  e It is not an uncommon event during pregnancy    1. Long mesentery of the pelvic colon  2. Narrow attachment at the base   5. Diverticulitis with a band, or adhesions  Sigmoid volvulus is a definite occurrence in mentally  disturbed patients, hypothyroidism, Parkinson’s disease,  multiple sclerosis, etc. probably due to severe constipation  due to medications.    1. Acute sigmoid volvulus (fulminant) presents as intestinal  obstruction. It starts usually after straining at stools.  Volvulus 1s usually in anticlockwise direction and after one  and a half turns, the entire loop becomes gangrenous.    Enormous distension of the abdomen takes place, which  gives a tympanitic note all over the abdomen. It 1s due to  diffusion of CO, (Fig. 30.22). Due to gross distension,    severe hypovolaemic shock develops within 6—8 hours of  volvulus. Gangrene sets in, which gives rise to features of  strangulation. A dilated loop can be seen and felt. Features  of peritonitis are seen within |I—2 days. Per rectal  examination shows rectum to be empty.    due to partial twisting and untwisting of the bowel. Elderly  patients present with recurrent lower abdominal pain on  the left side and distension of the abdomen which Is relieved  on passing large amount of flatus.    Plain X-ray abdomen erect shows a hugely dilated sigmoid  loop which is described as ‘bent inner tube sign’. The  dilated loop may be visible on the right side, centre and to  the left of abdomen, having two fluid levels, one on right  side and one on left side. This 1s also described as ‘omega  sign’ (Figs 30.23 and 30.24).    A successful passage of flatus tube or sigmoidoscope up to  25-30 cm results in release of a large amount of flatus and  fluid and obstruction 1s relieved. If obstruction is completely  relieved or if there is no gangrene and the general condition  of the patient improves, an elective resection 1s done after  7 days. Ifresistance 1s found while passing flatus tube, instill  barium for guidance.    Fig. 30.24: An 84-year-old  patient with sigmoid volvulus,  managed by sigmoidoscopic  decompression    condition of the patient is good. If the loop 1s gangrenous,  resection followed by end to end anastomosis is done,  after giving ‘on table’ lavage using saline washes till the  contents of the colon are clear. Sigmoid colon ts hugely  dilated (Figs 30.25 and 30.26).    proximal bowel ts loaded with faecal matter, resection of  the sigmoid colon 1s done. Proximal descending colon 1s  brought out as an end colostomy and rectum ts closed  (Hartmann’s procedure). After 6 weeks, colorectal  anastomosis 1s done.    sigmoid loop and fix the sigmoid to the posterior abdominal  wall (sigmoidopexy). If the mesentery 1s long, it can be  made short by plication.    general condition of the patient is poor as in elderly patients,  in severely dehydrated patient with impending septicaemia.  In such cases, the gangrenous loop is brought outside and  resected, with a proximal colostomy and a distal mucous  fistula (Fig. 30.27).    ¢ Inaplain X-ray abdomen, caecum produces round shado'  in the centre of the abdomen.    If loop is gangrenous, do not derotate—clamp the  mesentery first, then detorsion can be done to avoid  reperfusion injury.   - It is a rare cause of intestinal obstruction (Figs 30.28 to ° Clockwise twist ° Anticlockwise    procedure which might require some degree of medial  visceral rotation or disruption of the fusion plane between  the caecum or ascending colon with the lateral peritoneum,  providing sufficient mobility allowing caecal volvulisation  to occur.    ¢ A constant feature of caecal bascule is the presence of a  ischaemia.    constricting band across the ascending colon. This may be  found at laparotomy.    Fig. 30.29: Erect X-ray showing distended  loop. Caecum was palpable in the left  hypochondrium    It is a congenital diverticulum which occurs due to persistent  intestinal end of vitellointestinal duct. Being congenital, it  has all the layers of the bowel. Hence, a true diverticulum.    A. Fibrous band results when entire duct is obliterated and  bowel can twist around the band, resulting in volvulus.    C.Meckel’s diverticulum with the band attached to the  umbilicus can give rise to intestinal obstruction.   Fig. 30.31: Distended caecum |  at surgery  (Courtesy: Dr Basavaraj Patil,    Dr Hartimath, Dr Rasheed, Fig. 30.32: Caecal volvulus with though it is connected to the terminal ileum, the opening is  Dr Nikil, Department of gangrene very small. The discharge is rarely faecal. Often, it is the mucus  Surgery, KMC, Manipal) secreted from the lining of the duct (omphaloenteric fistula).   e Caecum will be markedly distended and will be found in  the centre of the abdomen.    e Occasionally, it is associated with malrotation of the gut :,  e There will be a constricting band across the ascending KEY BOX ee ee  colon.    ¢ |t is also argued that caecal bascule can also be due to OTHER ASSOCIATED ANOMALIES  lack of fixation of large bowel. Resection is ideal even    Figs 30.33A to F: Anomalies of vitellointestinal duct (A to F see text for details)   e Itis present in 2% of the cases, 2 inches long, 2 feet away  from ileocaecal region in the antimesenteric border (ileal  duplication can occur in the mesenteric border). It 1s two  times more common in females.   ¢ In 12% of the patients, heterotopic gastric tissue is found  which can produce peptic ulceration. In a few other patients,  it can contain pancreatic and colonic tissue.   e Presentation: 2 years or below 2 years is the most common  age    ¢ Massive bleeding per rectum: In the form of melaena, it  is not uncommon. In many other patients, mild chronic  bleeding can result in anaemia.  ¢ Acute Meckel’s diverticulitis: Factors which precipitate   — Peptic ulceration due to ectopic gastric mucosa of the  diverticulum   — Ingested foreign material, e.g. stalk of vegetable, seeds,  fish or chicken bones   — Inflammation and ischaemia caused by torsion due to an  associated band is called mesodiverticular band    MECKEL’S DIVERTICULUM AND y  INTESTINAL OBSTRUCTION  e Intussusception  e Band  e Volvulus due to band  e Internal herniation beneath mesodiverticular band  e Diverticulitis with band  e Littre’s hernia    ¢ As a cause of intussusception: Here also, inflamec  heterotopic tissue can be found in the diverticulur  (2% cases).    Hernia of Littre is a hernial sac containing Meckel’s  diverticulum (Key Box 30.17).    1. No investigation can prove diagnosis of Meckel’s  diverticulum. Small bowel enema may demonstrate the  diverticulum if opening 1s wide (fluoroscopy is more ideal).  2. °°™Tc-labelled pertechnetate given IV, may localise the  heterotopic gastric mucosa in the Meckel’s diverticulum,  in about 90% of patients.  ¢ This radionuclide is taken up by mucin-secreting cells  and parietal cells and it is secreted immediately. Thus,  if’ Tc appears in the stomach as well as in other part  of bowel, it indicates functioning heterotopic tissue.   ¢ Even when bleeding is at a rate of 0.1 ml/minute, it can  detect Meckel’s diverticulum. Hence, it 1s superior to  angiography.    The pertechnetate anion ’"" Tc is selectively taken up by  gastric mucosal cells, thyroid, salivary glands and  choroid plexus.    1. Incidentally found Meckel’s during laparotomy for some  other causes can be left alone, provided it has a wide mouth.  However, a note of it must be made in the operation register  (Key Box 30.18).    2. Meckel’s diverticulum with bleeding, band, perforation and  narrow mouth is treated by removal of diverticulum with    tine (Courtesy: Dr Pramod K, Dr Deviprasad Shetty, Kasturba Hos-  pital, Manipal)    adjacent intestine because the gastric tissue may often line  the intestine also (Figs 30.35 and 30.36).    They are common causes of intestinal obstruction in the  western world, Chinese population of Malaysia, etc. In India,  adhesions and obstructed hernia are the two common causes   that occur after damage to the peritoneum. The early balance aoe Ties ee oe  between fibrin deposition and degradation seems to be the Itis unportant to realise that secondary infertility in women  critical factor in adhesion formation. They also cause and ectopic gestation can occurs due to adhesions.    without perforation, perforation peritonitis, intra-abdominal  abscess have higher incidence of adhesions. Surgery 1s the  commonest cause of peritoneal adhesions.    . latrogenic: It refers to talc, silk thread, foreign body (mop),    etc. used for surgery which can induce extensive adhesions  due to foreign body reaction (spilled gall stone also).   Infiltration of inflammatory cell,  _ release of cytokines, activation of ©  complement system and activation    Ischaemia and irritation of the intestines are the chief factors  responsible for adhesions (Key Box 30.19).    1. Fibrinous adhesions (bread and butter adhesions): They  are the causes of early postoperative obstruction, which  settles down within 3—5 days. Majority of them disappear  in due course of time.    2. Fibrous adhesions: If the infection is continuing or if  foreign body is present, the fibrinous material is converted  to fibrous band. They also occur at the site of ischaemia.  They will cause late intestinal obstruction (Figs 30.38 and  30.39).    3. Tuberculous adhesions are dense adhesions that result in  matting of intestinal coils. Separating them at laparotomy  is extremely difficult (Fig. 30.40).    A congenital band, omental band, a string band of bacterial  peritonitis or a fibrous band of tuberculosis ts responsible  for obstruction (Figs 30.41, 30.42 and Key Box 30.20).    ¢ Recurrent abdominal pain, vomiting and distension are the  typical features. Often the attacks are mild and self limiting.  But persisting symptoms require monitoring and treatment.   ¢ There may be peristalsis as in cases of terminal ileum.   ¢ Gangrene 1s not common in cases of adhesive obstruction  (Figs 30.42 and 30.43). However, these cases have to be  closely monitored for any changes in the type of pain or    new abdominal signs. Severe pain, tachycardia, tempera-  ture, tachypnoea and tenderness in the abdomen indicates    Plain X-ray abdomen and small bowel enema are very  useful investigations to prove the obstruction.   — Thickening and oedema of the bowel wall suggests  intestinal ischaemia. Presence of intramural air is a late  sign (gangrene)   — CT has a sensitivity of 90% and specificity of 88%   — Thus, CT and MRI are very helpful in patients with small  bowel obstruction.    In the form of nasogastric aspiration, IV replacement c  fluids and electrolytes to correct dehydration may b  successful in early postoperative obstruction. If it is nc  successful, reoperation is required. Generally 48—72 hour  is the waiting period in patients who present to the hospite  as late adhesive obstruction. Further delay may result 1:  perforation or gangrene of the bowel.    Record pulse rate, blood pressure, abdominal girth an  intake output. Increasing pulse rate, hypotension, increasin;  abdominal girth and oliguria 1n spite of adequate IV fluid:  will suggest gangrene. Such cases need to be explore  immediately.    Where fibrous bands are the cause, they need to be dividec  to relieve obstruction.    Laparoscopic adhesiolysis is more often being used and 11  is indicated in pelvic adhesion, selected cases of abdominal  adhesion, single band adhesion and obstruction with mild  distension.    barriers suchas expanded polytetrafluoroethylene (PTFE)  and membrane composed of hyaluronic acid and carboxy-  methyl] cellulose have been used.    are fixed in a suitable relation to one another (not very  successful) (Fig. 30.45).    Thorough peritoneal toilet in cases of peritonitis with saline  or dextran to drain pus, bile, blood clots.   e The bioresorbable membrane called seprafilm is currently  the most effective membrane barrier.   e At the completion of surgery, these films are placed at the  potential sites of adhesion formation—such as pelvis,  between the intestinal loops.   e Mechanism of action: Within the next 24 to 48 hours, the  seprafilm membrane hydrates to form a gel-like barrier. It  slowly resorbs within 7 days.   ADHESIVE INTESTINAL OBSTRUCTION  e It is the most common cause of intestinal obstruction (in   e One can wait for 3—4 days with careful monitoring before a  decision of laparotomy is undertaken (worsening situation).   e Repeated X-ray abdomen and CT scan will show changes  and progression of the obstruction.   e Most valuable clinical signs of ischaemia/gangrene are  tachycardia and tenderness.    Gall stone ileus 1s more common in women  and accounts for 14% of all presentations to hospital with    Fig. 30.47: Gall stones ileus (both these figures are contributed  by Ms Vidhushi, Kasturba Medical College, Manipal)    small bowel obstruction. The term gall stone ileus 1s a  misnomer, as the condition 1s a mechanical obstruction of the  gut and not a true ileus.    It should be suspected in a patient who has gall stones and  present with intestinal obstruction.    Gall stone reaches the terminal ileum by forming  ‘cholecystoduodenal fistula’ due to recurrent attacks of  cholecystitis (Figs 30.46 to 30.49).    Due to recurrent inflammation, adhesions develop between  gall bladder and duodenum (common) or gall bladder and  colon or stomach (rare). Large stones cause pressure necro-  sis, resulting in formation of a cholecystoduodenal fistula.    Terminal ileum 1s the narrowest portion of the gut wherein  gall stone gets impacted. Sometimes, the stone may ulcerate  from gall bladder into jejunum, colon, etc.    1. Plain X-ray abdomen (erect position) may demonstrate  multiple gas and fluid levels and stone in the gall bladdet  and also in the lower abdomen suggesting gall stone ileus.    3. Computed tomography (CT) scanning is the investiga-  tion of choice. It has preoperative diagnosis of gall stone  ileus with a sensitivity of 93%. It will also indicate any  inflammation of the gall bladder, fistula-air pockets in the  gall bladder, distal stone and proximal dilatation.    ¢ There is difference of opinion regarding what is the ideal  surgery to be done in case of gall stone obstruction. It 1s    ¢ Clinical features: They are suggestive of small intestinal  obstruction—abdominal pain is severe, vomiting and  distension. Step ladder peristalsis may be seen. History  suggestive of recurrent cholecystitis may be present.    ¢ Rigler’s triad: Pneumobilia, the presence of an aberrant  gall stone and enteric obstruction (Fig. 30.50).    *Bouveret syndrome: |t is a gastric outlet syndrome  secondary to stone lodged in proximal duodenum due to  cholecystoduodenal fistula. Proximal migration of the stone  will precipitate obstruction. Fig. 30.51: Enterolithotomy and removal of stone   'Cholecystoduodenal fistula, choledochoduodenostomy, sphincteroplasty, emphysematous cholecystitis are a few other conditions wherein air is  found within biliary tree.    because many patients are elderly, with co-morbidity and  they have intestinal obstruction (dehydration, bowel  oedema, bacterial proliferation, etc). Whatever itis, the first  step is always to do enterolithotomy by incising the ileum  and deliver the stone/stones. It may be possible to crush  the stone and pass it onto caecum, avoiding enterolithotomy.  Search the proximal intestine for any other stones. What  must be done next? Two types are described.    1.Single stage procedure: If general condition of the  patient 1s good, and gall bladder is gangrenous or  inflamed (more chances of perforation) enterolithotomy,  cholecystectomy followed by closure of the duodenal  fistula 1s done.    2. Two stage procedure: If general condition is not good  or with no active inflammation of the gall bladder,  enterolithotomy is the procedure of choice, followed 6  weeks later by cholecystectomy with closure of the  duodenal fistula (Fig. 30.51).   Invagination of one segment of intestine into another (usually  the proximal into distal) is called intussusception.    {tis the most common cause of intestinal obstruction in  infants aged 6 to 18 months.   1. Simple ileocolic 1s the most common type, followed by  ileoileal or colocolic.    3. Retrograde jejunogastric intussusception, a complication  of gastrojejunostomy (GJ) is a rare but interesting type of  intussusception (see page 503).    1. Intussuscipiens: It is the outer tube (distal bowel which  receives the intestine).    2. Intussusceptum: Proximal bowel (inner tube) which enters  the distal segment.    3. Apex is the part which advances further into the distal  bowel.    4. Neck, the narrowest portion of intussusception, is the  junction of entering layer with the mass.    1. Idiopathic intussusception: Actual cause is not known. It  is seen in infants. Possible factors (Key Box 30.24).  ¢ Dietary factor: Around the age of 6—9 months, weaning  of breast milk is done. Weaning causes alteration in the  bacterial flora in the GIT, causing enlargement of the  Peyer’s patches. These protrude into the terminal ileum  and may precipitate intussusception.   PREDISPOSING FACTORS S  e Recent viral infection (upper respiratory )  e Recent operation  e Henédch-Schénlein purpura  e Cystic fibrosis  e Coeliac disease  e Haemophilia    ADULT INTUSSUSCEPTION  e Meckel’s diverticulum  e Polyps, tumours, submucous lipoma  e Carcinoma colon (caecum, transverse colon)    e As the apex advances, it drags the mesentery containing  blood vessels which get obstructed at the neck resulting in  mucosal ulcers and haemorrhages. Marked lymphadeno-  pathy and hypertrophy of Peyer’s patch 1s found at operation.   e If the neck 1s too tight, gangrene sets in very early, as in  ileocolic intussusception.   ¢ All other features of strangulation, dehydration, distension  and septicaemic shock develop later.   Fig. 30.53: Intussusception Fig. 30.54: Carcinoid of the ileum with intussusception   Fig. 30.55: Carcinoid of the ileum causing intussusception— Fig. 30.56: Intussusception due to polyp  resected specimen    First born male infants between 6 and 9 months are  commonly affected. Boys : Girls — 3: 2.   One attack of red currant jelly stools is characteristic.  Bleeding is due to mucosal ulcer (venous infarction). Mucus  secretion 1s due to Irritation of intestines. This 1s followed  by absolute constipation. Red currant jelly stools are not  found in adult intussusception.   Vomiting 3-4 times, initially due to pylorospasm. Later,  due to obstruction.   In between the spasms, the child sleeps but gets up suddenly  with pain.    The mother is asked to feed the baby in sitting position and  examination of the baby’s abdomen is done with the left  hand, standing in front of the mother.   A contracting, hardening mass in and around the umbilical  region can be felt (sausage-shaped, Fig. 30.63).  Emptiness in the right iliac fossa (Dance’s sign—signe  de dance).   Rarely, intussusception can be seen outside the anus due  to long mesentery.    diagnostic of intussusception. This 1s also called ‘meniscus  sign’. If there is any suspicion of gangrene, this test should  not be done. In many cases, the diagnosis is established on  clinical grounds.    is introduced into the rectum and |~—2 litres of saline from ¢  height of 1-2 metres is allowed to run. Catheter 1s removec  and buttocks are pressed together. 50-70% of cases are  reduced by this method, 1:3 barium sulphate in warn  isotonic saline can also be used.    is pumped into the colon at a pressure of 60-80 mmHg.  Contraindications:  ¢ Peritonitis with shock   2. Child is symptom-free and comfortable  3. Small bowel loops are filled with contrast    choice nowadays. It can detect target sign and detect  mass (Doughnut sign) with Doppler. It can be used to  assess vascularity of the bowel also. Thus, barium enema  has become obsolete.    © CT scan: It is the most sensitive imaging modality inthe °* Intussusception is reduced by milking (squeezing) the colon    diagnosis of intussusception. Sausage-shaped mass, with  blood vessels within bowel lumen are typical findings (see  Fig. 30.59).    in opposite direction, which is facilitated by breaking the  adhesions at the neck using the little finger. Appendicec-  tomy is also done, as it avoids any future confusion as to    the reason for the abdominal scar. Fixing the caecum Is not  necessary because idiopathic intussusception rarely recurs.  If the loop is gangrenous, resection and ileocolic anasto-  mosis is done.    Recurrent intussusception is rare: If it occurs, terminal  ileum 1s sutured to the side of the ascending colon.    Figs 30.67 and 30.68: Adult intussusception due to jejunal  lipomatosis. This patient was 24-year-old male who presented  with intestinal obstruction. A mass was palpable in the umbilical  region at laparotomy. This mass was resected. Opened specimen  showed extensive segmental lipomatosis (Courtesy: Dr Gabriel  Rodrigues, Dr Mahesh Gopa Setty, Dr Lavanya K, KMC, Manipal)    ACUTE INTUSSUSCEPTION—MOST ree Mh  e Most common in children between 5 and 10 months of  age  e Most common cause of intestinal obstruction in children  e Most common cause is idiopathic (90%)    e Most incidence of adenovirus infection, common in  midsummer and midwinter    e Most common type of nonoperative reduction is by using  air and barium enema    Acute mesenteric ischaemia is an abrupt reduction in blood  flow to the intestinal circulation of sufficient magnitude to  compromise the metabolic requirements and potentially  threaten the viability of the affected organs (Key Box 30.28).   e It 1s due to atherosclerosis causing thrombosis of the  Superior mesenteric artery or due to emboli which originate  from atheromatous plaques or from the infarcted heart.   e Superior mesenteric vein can also get thrombosed due to  injury during pancreatectomy or thrombosis as a result of  oral contraceptive pills.    1. Acute mesenteric ischaemia (AMI): It is a Sudden occlusion  of artery or vein resulting in gangrene. It has a mortality  rate of 60 to 90%.   2. Chronic mesenteric ischaemia (CMI): It is associated with  stenosis of coeliac artery, Superior mesenteric artery or  inferior mesenteric artery.    The pathological effects of arterial occlusion and venous  occlusion are the same. Superior mesenteric artery supplies  the entire midgut starting from the duodenojejunal flexure  to right one-third of the transverse colon. It 1s an end-artery.  As a result of thrombosis, the entire small bowel and portion  of the large bowel becomes gangrenous (if there is a thrombus  at the origin of superior mesenteric artery).    1. Arterial emboli are the most frequent cause of AMI and  are responsible for approximately 40 to 50% of cases. Most  mesenteric emboli originate from a cardiac source. They  lodge in the superior mesenteric artery (SMA) because It  emerges from the aorta at an oblique angle. Few emboli  lodge in the origin of middle colic artery.    2. Acute mesenteric thrombosis accounts for 25 to 30% of  all ischaemic cases. Atherosclerotic disease 1s the cause of  thrombosis. It typically occurs at the origin of the superior  mesenteric artery. Gangrene 1s more extensive in cases of  thrombosis than embolism.    3. Nonocclusive mesenteric ischaemia (NOMI): Typically  happens in cases of hypotensive patients. Low cardiac  output, ICU patients on vasoconstrictors and inotropes  precipitate the problem. Splanchnic vasoconstriction occur  In response to hypovolemia. Vasoactive drugs, particularly  digoxin, have been implicated in the pathogenesis of NOMI.    4. Mesenteric venous thrombosis (MVT) Its the least  common cause of mesenteric ischaemia, representing up  to 10% of all patients with mesenteric ischaemia related to  primary clotting disorders. Thrombi usually originate in the  venous arcades and propagate (Key Box 30.29).    e Hypercoagulable states (e.g. polycythaemia vera, protein  C and S deficiencies).   e Women taking orai contraceptives are also at increased  risk of venous thrombosis.    1. Atherosclerosis: Hypotension, haemorrhage into atheroma  2. Hypercoagulable state  3. Fibromuscular dysplasia    e Itis better to have some idea about the splanchnic circula-  tion to understand the effects of ischaemia to the gut (Key  Box 30.30).   ¢ The splanchnic circulation receives approximately 25% of  the resting and 35% of the postprandial cardiac output.   ¢ 70% ofthe mesenteric blood flow is directed to the mucosal  and submucosal layers of the bowel. Hence early cases  present with bleeding due to mucosal ulcerations.   ¢ Blood flow is regulated by intrinsic (metabolic and  myogenic) and the extrinsic (neural and humoral) factors.   ¢ An imbalance between tissue oxygen supply and demand  will raise the concentration of local metabolites (e.g.  hydrogen, potassium, carbon dioxide, and adenosine),  resulting in vasodilation and hyperaemia.    Abdominal pain: Severe, poorly localised, unresponsive  to narcotics, out of proportion to the physical findings  (Fig. 30.70).    . Onset may be abrupt (embolism) or insidious (thrombosis)  . Gastrointestinal emptying: Vomiting, diarrhoea, with    Early: Soft, nontender, normal bowel sounds.  Late: Distension, tenderness, rebound tenderness, rigidity,  absent bowel sounds.    Sudden event, sudden bleeding, sudden collapse, severe  pain, silent abdomen, shock progressing to sepsis is  mesenteric ischaemia.    Total counts are raised, in about 75% of cases > 15,000  cells/mm?.    the bowel loops and intramural gas. Blunt plicae are seen.  This is called as thumb printing (Figs 30.71 and 30.72).    - Metabolic acidosis in > 50% of cases.  . Serum phosphate levels are raised within 3—4 hours    . D-dimer is elevated in all cases.  . CT with or without angiogram is the investigation of    choice. It can detect intramural air pockets in the bowel  wall, air within biliary radicle, perforation, etc. It can also  detect thrombus or narrowing of superior mesenteric artery  or superior mesenteric vein (Figs 30.73 and 30.74).    . Emergency angiography is the test of choice and can be    done within 6 hours of ischaemia. It can demonstrate arterial  thrombosis at the origin or distally in one of the branches.  (It can also be therapeutic.)   Fig. 30.70: Attitude of a patient with severe ischaemia due to  superior mesenteric vascular occlusion. In these cases, the pain  is disproportionate to the abdominal signs   Figs 30.71 and 30.72: Plain X-ray showing presence of air in the  bowel wall   Fig. 30.74: CT confirming the same. A case of superior mesen-  teric arterial thrombosis with massive gangrene (See Fig. 30.75)    1. Majority of the patients present late with massive gangrene.  Massive resection of the gangrenous bowel followed by  end to end anastomosis is done. These patients suffer from  short bowel syndrome (vide infra), if they survive.    2. If patients come within 4—6 hours of ischaemia, emergency  angiography followed by papaverine infusion (30 to 60  mg/h) into the superior mesenteric artery can be tried.  Otherwise, emergency laparotomy 1s done and the superior  mesenteric artery 1s explored. A Fogarty catheter is  introduced and embolectomy 1s done. These patients may  require a second look operation within 24—48 hours to  rule out gangrene developing later due to rethrombosis of  the artery.    c. Nitroglycerine  d. Nitroprusside  e. Prostaglandin E  f. Phenoxybenzamine  g. lsoproterenol  5. Sympathetic epidural block    ¢ Doppler (84%)  — Absent arterial flow on antimesenteric border of bowel.  — Absent mesenteric arterial flow    Majority of the cases present with massive gangrene. Even  after massive resection, they succumb to the sepsis and  multiorgan failure (Fig. 30.75).    See next page for a few causes of intestinal obstruction  (Figs 30.76 to 30.81).   Fig. 30.75: Massive gangrene of small intestines and right side  of the colon due to thrombosis at the origin of superior mesen-  teric artery    Fig. 30.78: Obstructed hernia Fig. 30.79: Nonspecific stricture Fig. 30.80: Peritoneal metastasis causing obstruction opened lesion  Fig. 30.81: Laparoscopy detected a lesion in the small intestine    ¢ Radiation stricture, ischaemic strictures and nonspecific  strictures are the other causes.   ¢ Small bowel enema or enteroscopy are very useful investi-  gations (Figs 30.82 to 30.85).   NEONATAL INTESTINAL OBSTRUCTION Types of Hirschsprung’s disease (Fig. 30.87)  1. Ultrashort segment: Anal canal and terminal rectum is   3. Long segment: Anal canal, rectum and part of colon  3. Arrested rotation with bands "awe lwedl.  4. Volvulus neonatorum 4. Total colonic: Anal canal, rectum and whole length of colon  5. Meconium ileus is involved.  6. Imperforate anus    It is also called congenital megacolon, aganglionic megacolon  or primary megacolon. It is one of the common causes of neo-  natal intestinal obstruction.    ¢ The disease always involves the anus and rectum wherein  parasympathetic ganglion cells are absent in the neural  plexus of the intestinal wall. The defect involves internal  sphincter.   above which the pelvic colon (sigmoid) is enormously  a : .    ¢ In between, there may be a transition zone (cone), which  contains a few parasympathetic ganglion cells.    ¢ Male children are commonly affected, when compared  to females.  ¢ Incidence: | in 4000 to 5000 live births.    ¢ The child presents with acute neonatal intestinal obstruc-  tion as manifested by failure to pass meconium or delay  — Transition in passing meconium with abdominal distension.  Zone Woon) ¢ Within 12—24 hours, all features of intestinal obstruction  can be found. If it is complicated by enterocolitis, it may  Terminally result in perforation and septicaemia. A severe diarrhoea  constricted segment with blood and mucus, abdominal distension and  vomiting can occur within a few hours, followed by  Fig. 30.86: Pathophysiology hypovolaemic shock.    ¢ Rectal examination reveals that the rectum is empty,  finger is gripped by anal sphincter and there Is no perianal  soiling (Fig. 30.88). On the other hand, in acquired mega-  colon, rectum 1s loaded with faecal matter, perianal soiling  is present and there 1s no sphincter activity (Fig. 30.89).   ¢ Chronic variety: Chronic constipation manifesting in  the first few weeks of life. The child may be brought  with abdominal distension. Stools are goat pellet-like.    ¢ Acquired megacolon: Usually manifests by one to two  years of age. Rectum 1s loaded with faecal matter.    1. Full thickness rectal wall biopsy under GA demonstrates  absence of parasympathetic ganglion cells and hypertrophic  nerve fibres in the nerve plexus. It should be taken above  the anorectal junction.    Today submucosal suction biopsy is more popular than  biopsy since it avoids haemorrhage, infection and  scarring.    2. Barium enema: 3.6% solution of barium is used, the  intermediate zone appears as a cone with proximal dilatation  and a distal narrow zone which is characteristic of  Hirschsprung’s disease.    I. Emergency cases  ¢ Right transverse loop colostomy: In most of the cases,  aganglionic segment 1s limited to rectosigmoid region.    ¢ A full thickness biopsy of the colostomy is sent for   ¢ Can be done usually between the age of 3 and 6 months  (8 to 10 kg of weight).   ¢ Resection of aganglionic bowel (anorectum) followed  by a pull-through procedure. Maintaining continence  is the main aim.    1. The rectum is transected above the peritoneal fold and 1s  closed.   2. The proximal ganglionic segment is pulled down behind  the rectum (retrorectal space created by using blunt  dissector).   3. An incision is made in the posterior wall of the anorectum  above the dentate line and is deepened through the entire  bowel wall.   4. The end of the proximal colon is sutured to the opening in  the posterior anal canal all around.   5. The adjacent walls of rectum (posterior wall) and colon  (anterior wall) are crushed by using a Kocher’s forceps  which falls off by itself by the 14th day.   6. The open end of the rectum (above) is closed end to side to  the colon.    ¢ Commonly, it affects the duodenum, followed by ileum and  jejunum (Fig. 30.90).    Duodenal atresia ts the most common cause of intestinal  obstruction in neonates.   Types 2: There is no separation of the two parts. However, a   Type 3: It is incomplete obstruction: There may be a web or   ¢ Anastomosis: A special anastomosis named after Kimura  is being described. It is a diamond shaped anastomosis  between upper pouch (duodenum which is opened  transversely and lower pouch (distal duodenum) which is  opened longitudinally.   ¢ Needless to mention here that general condition has to be  taken care of with supportive therapy including fluid and  electrolytes, total parenteral nutrition, nasogastric  decompression and adequate urinary output.   ¢ Duodenal atresia presents as vomiting with or without bile  minimal distension and visible gastric peristalsis.    Duodenal atresia: Duodenojejunostomy by anastomosing  dilated duodenum above the atresia to the jejunal loop.   ¢ Like duodenal atresia, it can be associated with maternal  hydramnios or malrotation of the gut.   ¢ Exact reasons for atresia are not known. However, vascular  variations in the mesentery such as V-shaped mesentery or  due to occlusion of vessels in the intrauterine life are the  possible factors.    ¢ Type I: Simple stenosis. Mesentery is normal. It is mucosal  atresia (Fig. 30.91).    ¢ Type II: Proximal and distal bowel are connected by a  fibrous band which has no lumen—atretic segment. Here  again, mesentery is normal (Fig. 30.92).   ¢ Type II}: Further divided into 2 types. IIIa refers to atresia  with V-shaped loss of mesentery in between, IIIb refer to  complete jejunal atresia with coiled ileum. It has been called  Christmas tree deformity (Figs 30.93A and B).    Please note: Apple-peel or Christmas tree deformity. Here,  obstruction is usually in the proximal jejunum, which is supplied  by the entire superior mesenteric artery (SMA). This results in  a gap in the mesentery. So, rest of the small intestine is coiled    e It 1s a congenital anomaly wherein the caecum and right  colon are found on the left side (Fig. 30.95).    ¢ Asa result of this, there are peritoneal bands which run  across from left to right and cause intestinal obstruction  (duodenal obstruction).    ¢ One such band is called transduodenal band of Ladd  which compresses the second part of duodenum and gives  rise to obstruction (features similar to duodenal atresia).    Laparotomy, division of band and fixation of the caecum in  the right iliac fossa.   It is a complication of arrested rotation with bands which pre-  disposes to midgut volvulus (small bowel). These unfortunate  infants undergo massive resection of the bowel if it is  gangrenous (Fig. 30.96). Such massive resections give rise to  short gut syndrome. Thus, the patient becomes a digestive  cripple. If the loop is not gangrenous, it 1s treated by  laparotomy, untwisting of bowel and division of bands.    e It is a neonatal manifestation of mucoviscidosis of the  pancreas wherein the mucus ts thick and viscid. This, along  with meconium, produces obstruction. There may be ileal  atresia, which might have precipitated meconium ileus  (Fig. 30.97). Majority of meconium ileus is coupled by  complications such as gangrene, perforation and  peritonitis. As a sequel to peritonitis, calcification and  adhesive meconium obstruction develop.   ¢ Infants present with abdominal distension, bilious vomiting  and failure to pass meconium.   e Plain X-ray shows distended bowel and mottling due to  calcification.    appearance tn the right lower quadrant due to viscid  meconium mixed with air.    ¢ Ultrasonography: Dilated loops of bowel filled with  echogenic material are highly suggestive of meconium ileus  rather than ileal atresia.    ¢ Ifperforation is ruled out, barium enema can be done which  shows microcolon.    It is indicated if there is no peritonitis, general condition of  the child is reasonably good or if there is partial obstruction.  Dilute gastrograftin is introduced into the colon as enema. It  fills up terminal ileum. It absorbs fluid from the interstitial  space into the lumen because it is hyperosmolar. Consequently,  the meconium becomes soft and it is rejected naturally.  Hypervolaemia 1s to be corrected during this.    gives rise to urinary bladder and posteriorly postallantoic  gut gives rise to rectum and upper 2 cm of anal canal.   ¢ Postallantoic gut fuses with proctodeum, thus giving rise  to anal canal. If there is a defective fusion of this, it results  in imperforate anus.    outside as a fistula and regular saline washes are given  to dilute the meconium. Mucous fistula needs to be closed  after a few weeks.    B. Santulli operation (Fig. 30.99)  ¢ In this operation, proximal ileum 1s brought out as  ileostomy. Distal ileum 1s anastomosed to proximal ileum  as end-to-side anastomosis.    Resection is the surgery of choice today as more cases  present with short segment obstruction. Long-term results  and complications are much less than nonresectional  procedures.    ¢ To start with, there is a common chamber called cloaca,  which ts later divided into 2 chambers, anteriorly allantois    Types of imperforate anus  I, Low anomalies: It refers to termination of the bowel below  the anorectal bundle.  1. Covered anus (Fig. 30.100A)  The anal orifice 1s covered by a tag of skin.  2. Membranous anus (Fig. 30.100B)  Covered with a thin membrane  3. Anterior ectopic anus (Fig. 30.100C)  Anus 1s situated anteriorly  4. Stenosed anus (Fig. 30.100D)  Anal orifice is microscopic  Il. High anomalies: They are supralevator—the bowel  terminates above the anorectal bundle.  |. Anorectal and agenesis, with fistula: Rectovesical,  rectovaginal, rectourethral fistulae (Fig. 30.101A).  2. Rectal atresia: Colon ends as a blind pouch below  (Fig. 30.101 B).  3. Cloaca: In this variety, bowel, urinary and genital tracts  open into a common chamber. This occurs in females  only.    12 hours after birth, the child 1s held upside down (12 hours  is the time for the gas shadow to reach the distal portion of the  gut). A metal coin is strapped to the site of anus and  X-ray is taken. If the gas shadow ts above the pubococcygeal    A B  Cc . )  Figs 30.100A to D: Low anorectal anomalies  line, itis a high anomaly. If the distance between the coin and  gas shadow is more than 2.5 cm, it is a high anomaly. If the    gas shadow is below the pubococcygeal line, it 1s a low  anomaly. Pubococcygeal line 1s called as Stephen’s line.    I. Low anomaly: Easy to treat, division of membrane or  skin followed by dilatation is all that is required with  some amount of plastic reconstruction (anoplasty 1s  necessary).   II. High anomaly: Repaired by 3-stage procedure:  ¢ Ist stage: Preliminary transverse colostomy to relieve  intestinal obstruction.  ¢ 2nd stage: When the child is 8-10 kg of weight, a “pull-  through” operation 1s done with division of fistula.  ¢ 3rd stage: After 2 months, colostomy is closed.    CAUSES OF INTESTINAL OBSTRUCTION AS PER AGE  Neonates: 0 to 7 days (Figs 30.102 to 30.109)    This complication can occur, particularly when a GJ or partial  gastrectomy is done.   ¢ They get impacted in the terminal ileum which is the  narrowest portion of the gut.    Squeeze the bolus into the caecum. Otherwise, enterotomy  and removal may be necessary.    |. These are rare causes of intestinal obstruction. Due to some  congenital defect in the mesentery, the floating, mobile  intestines can herniate (Key Box 30.33). The defect may  be in:  e Mesentery  ¢ Transverse mesocolon or  ¢ Broad ligament  ¢ Foramen of Winslow  Hence, whenever a surgical procedure is done for resection  of the bowel or GJ, etc. Once the anastomosis 1s completed,  the rent in the mesocolon as in GJ or rent in the small bowel  mesentery should be closed.   2. Herniation can also occur through one of the potential  spaces (fossae) in and around a viscus.    Fig. 30.109: Necrotising enterocolitis with patchy gangrene  (Courtesy: Professor Vijaykumar, Department of Paediatric Surgery, KMC, Manipal)    Duodenal fossa  ¢ Left paraduodenal fossa: Inferior mesenteric vein lies very  close to the free border here.    Colonic fossa  ¢ Superior ileocaecal fossa  ¢ Inferior ileocaecal fossa  ¢ Sigmoid fossa    These are the rare causes of intestinal obstruction to be  Kept in mind.    In this condition, there is a failure of transmission of para-  sympathetic impulses from one segment to the other. That  means, there 1s a failure of parasympathetic mechanism, which  results in paralysis of bowel. It gives rise to a large collection  of fluid and gas within the bowel resulting in distension.    1. Postoperative: Exposure of the intestines, handling of the  intestines, contamination with blood, foreign body, etc.  causes temporary suppression of parasympathetic activity  and results in paralytic ileus. This gets aggravated by  allowing oral fluids too early.    4. Hypokalaemia: K’ is the chief intracellular ion ofall cells  including muscles. Hypokalaemia causes generalised  muscle weakness including muscles of the bowel.    2. No colicky pain abdomen (in dynamic obstruction, colicky  pain is a feature). Dull pain occurs due to distension of the  abdomen.   3. Failure to pass flatus, effortless vomiting is also  characteristic of paralytic ileus.   4. On auscultation, tinkling sounds are heard due to shift of  fluid from one coil of bowel to the other (Key Box 30.34).   e Wherever a defect is present which is an anatomically  present recesses or congenital or following surgery, loop  of small intestine can herniate resulting in hernias.   e They are difficult to diagnose. The diagnosis is by exclusion   e Triggering factors: Inflammation in the vicinity with or  without an adhesion and band trigger herniation and  complications.   e Most of these cases present as acute abdomen with  features of intestinal obstruction.   e It should be remembered that an important vessel runs in  the close vicinity of these sites in most of the cases.   e Treatment is reduction, suturing the defect or resection  anastomosis if the intestine is gangrenous taking care not  to damage the vessel. Decompression without dividing the  constricting ring may be required.    1. Plain X-ray abdomen erect demonstrates distended loops  of bowel (Fig. 30.110).  2. Electrolyte study and correction of any abnormality.   ¢ The cause of paralytic ileus has to be treated first, e.g. if  there is hypokalaemia, supplement potassium. If there  is pus in the peritoneal cavity drain it.   ¢ Intravenous fluids, supplementation of ions, correction of  dehydration, oliguria, etc. Such treatment 1s continued for  3--4 days.   ¢ Ryle’s tube is removed when abdomen ts soft, bowel sounds  are heard and patient has passed flatus. Clear oral fluids  are started for 2—3 days followed by soft diet. Small bowel  activity returns within |2—18 hours, followed by colon which  starts functioning within 36--48 hours. However, gastric  functions may return ranging from 18 hours to 4 days.    Acute colonic pseudo-obstruction (ACPO) 1s also called  Ogilvie’s syndrome. It is massive colonic distension in the  absence of a mechanically obstructing lesion.    It occurs mainly due to malfunctioning of sacral para-  sympathetic nerves (S2—-S4). It results in atony of the  descending colon resulting 1n functional obstruction. It 1s  interesting to note that the junction of the dilated and  collapsed bowel is near the splenic flexure. This is the place  wherein parasympathetic supply by vagus ends and sacral  autonomic nervous system starts. An increased sympathetic  tone results in colonic dilatation due to inhibition of contraction  (Key Box 30.35).    ¢ Elderly bedridden patient with cardiac/lower respiratory  illness are the victims. Aerophagia and drugs which  decrease colonic mobility are precipitating factors.    ¢ Tachypnoea due to elevation of the diaphragm due to  distended colon is common.   ¢ Rectal examination reveals some faeces (in cases of  mechanical obstruction, rectum is empty).    ¢ Plain X-ray abdomen erect may or may not show one or  two air fluid levels. Distension is mainly colonic.   ¢ Prokinetic drugs such as cisapride or mosapride have been  tried in selected cases.   Ce ee ee ee ee H  iia  CAUSES OF COLONIC PSEUDO-OBSTRUCTION  1. Retroperitoneal irritation  e Blood  e Urine  e Fracture spine and pelvis  2. Drugs  e Levodopa  e Tricyclic antidepressants  3. Metabolic  e Uraemia  e Diabetes  e Myxoedema  ¢ Hypokalaemia    ¢ Rarely, even after colonoscopic decompression, caecal  tenderness continues. If distension persists, laparotomy  followed by tube caecostomy may have to be done.    e Firstly, strangulation should be ruled out by clinical and  radiological tests. Extended nonoperative therapy may  be advised, e.g. if one can wait for 48 hours tn a case of  intestinal obstruction. In these cases 4—6 days of waiting Is  sometimes worth, especially in a patient who has been  operated many times earlier.   ¢ During this extended period, careful monitoring 1s important  to look for any new symptoms/signs of strangulation.    ¢ As far as possible, resection should be avoided in Crohn’s  disease because the aim 1s to save as much as possible.   ¢ Strictureplasty should not be done in patients with intra-  abdominal abscesses or intestinal fistulae.    ¢ The commonest cause of intestinal obstruction in pregnancy  and puerperium is adhesive bands.    and that is why the primary cause is adhesive bands  (Fig. 30.111).  ¢ Inguinal hernia and intussusception are the other causes.    ¢ The consequences of intestinal obstruction in pregnancy  do not differ from those found in nonpregnant women  except that in the pregnant patient, a second entity, the  foetus, is also threatened.    ¢ Most of the intestinal obstructions tend to occur in the third  trimester.    ¢ Maternal mortality for intestinal obstruction in pregnancy  (10-33%) is higher than in nonpregnant patients.    ¢ In the first half of the pregnancy, nausea, vomiting and  episodes of constipation are quite common. Hence, they  are confused for hyperemesis gravidarum, acute duodenal  ulcer and gastritis.    ¢ In the second half of the pregnancy, symptoms can be  confused for toxaemia, constipation, Braxton Hicks  contractions, etc.    ¢ Ultrasound can help in the diagnosis by detecting dilated  intestinal loops, fluid in the peritoneal cavity and intus-  susception. It also helps to rule out ovarian torsion, gall  Stone disease, etc.    ¢ Premature labour can be prevented with tocolysis  (abolition of uterine contraction).    ¢ Abdominal surgery in the third trimester does not  induce labour.    ¢ Negative laparotomy carries a small risk of disturbing the  pregnancy.   ¢ Volvulus and intussusception are the two major causes of  small intestinal obstruction in pregnancy. Both can give  rise to gangrene. Hence an intervention is required before  gangrene sets in.    suspected of having a bowel obstruction should be no  different from those given to a nonpregnant one.    It is rare type of knotting between sigmoid and ileum carrying  significant mortality if not treated timely (Key Box 30.36).    ¢ This is also a rare hernia following bone graft removal from  the iliac crest for treatment of fractures.   e The ileum twists around the sigmoid colon resulting in  gangrene of ileum or sigmoid colon or both.   e Even though sigmoid is also twisted, features are similar  to small intestinal obstruction—not massive distension as  seen in sigmoid volvulus.   e Resection anastomosis of gangrenous segment followed  by anastomosis of the bowel (ileoileal/colic and colocolic).    ¢ Iliac crest is the common donor site of bone graft. If the  bone graft removed is large, the intestines can herniate  resulting in intestinal obstruction.   ¢ CT scan gives the diagnosis  Following is the case report of a patient who had undergone    Details about the causes of large bowel obstruction, patho-  physiology and the treatment is given in Chapter 29. However  a few important points have been given in ten commandments.    A 50-year-old man presented to the casualty with abdominal  pain, vomiting and distension. Features were suggestive of  intestinal obstruction. On examination, there was a buldge  in the region of right iliac fossa—more lateral. There was a  scar of bone graft incision. On questioning the patient he  says that he had fracture humerus. Two months back, nailing  and bone grafting had been done. Ultrasound revealed bowel  loops. CT scan showed a large bone graft defect with  herniation of intestines resulting in intestinal obstruction.  Emergency surgery, reduction of hernia contents and mesh  repair was done. Recovery was uneventful.   4. Should take into account, the general condition of the  patient before resection anastomosis.   5. Should consider right hemicolectomy or extended  hemicolectomy for right-sided growth which is operable.   6. Should consider single stage resection anastomosis also  for left-sided growth provided general condition of the  patient is good and on table lavage is given before the  anastomosis.   7. Should consider exteriorisation of gangrenous bowel in a  very sick patient.   8. Should consider a simple diversion colostomy in moribund  patients in rectosigmoid obstructions (it is a common  problem).    The incidence of malrotation is | in 500 infants.  The male to female ratio is 2: 1.    Malrotation with midgut volvulus may become rapidly life-  threatening. The previously healthy infant with bilious  vomiting is a characteristic presentation of malrotation.  Usually it presents with bilious vomiting, failure to thrive  and features of intestinal obstruction (Fig. 30.115).    In approximately 60% of patients, malrotation presents by  one month of age. Another 20-30% of patients present at  age 1-12 months. Thereafter, it can present at any age, and  is seen in adults and even the elderly.    A volvulus is a complete twisting of a loop of intestine  around its mesenteric attachment site. This can occur at  various locations of the GI tract, including stomach, small  Intestine, caecum, transverse colon, and sigmoid colon.  Midgut malrotation refers to twisting of the entire midgut  about the axis of the superior mesenteric artery (SMA).  Malrotation is any deviation from the normal 270°  counterclockwise rotation of the bowel that occurs during  embryogenesis (Figs 30.115 and 30.116).   At the fourth week of gestation, the gastrointestinal system  is a Straight tube centrally located in the abdomen. During  the ensuing 8 weeks, the midgut rotates and becomes fixed  to the posterior abdominal wall. Arrest of development at  any stage narrows the mesenteric base and impairs  fixation, leaving the bowel at high risk for volvulus.   Fig. 30.115: Midgut volvulus: (A) Clockwise rotation resulting in  superior mesenteric ischaemia shown in red line, (B) Anticlockwise  untwisting to be done at surgery is shown as green line    ¢ The resultant shortened mesenteric pedicle predisposes to  midgut volvulus, a clockwise rotation around the superior  mesenteric artery axis that can lead to bowel ischaemia.    Investigations  ¢ Conventional radiographs are neither sensitive nor specific  for malrotation.    ¢ On the upper GI series, it is crucial to locate the position of  the duodenojejunal junction (DJJ). The DJJ must be at least    over (but more reassuringly lateral to) the left vertebral  pedicle and at the same height as the duodenal bulb on 2  well-centred view. If the DJJ does not meet these twe  criteria, malrotation 1s diagnosed.    ¢ Signs of midgut volvulus include an abrupt termination.  or break of the contrast column and the corkscrew  (apple peel, or barber pole) sign.    superior mesenteric artery (SMA) and superior mesenteric  vein (SMV) relationship may be reversed.    ¢ Normally, the SMV its to the right of the SMA; with  malrotation, the SMV may occupy a position directly  anterior or to the left of the SMA.    ¢ The Ladd procedure remains the cornerstone of surgical  treatment for malrotation today.    ¢ A classic Ladd procedure is described as reduction of  volvulus (if present), division of mesenteric bands, place-  ment of small bowel on the right and large bowel on the  left of the abdomen, and appendicectomy.    ¢ A laparoscopic variation of the Ladd procedure has been  used in some centres, with the general advantage of  decreased adhesions and scarring, but good visualisation  of the entire bowel is necessary. Laparoscopy is a  particularly suitable investigative procedure for children  presenting with acute abdomen.   Most common cause of small intestinal obstruction is intra-  abdominal adhesion.   Most commonly performed, simple diagnostic investigation  in case of obstruction is plain X-ray abdomen in erect  position.   Most common abdominal symptom of intestinal obstruction  is colicky abdominal pain.   Most common cause of intestinal obstruction in infants  between 6 and 18 months is intussusception.    e Malrotation and special causes of intestinal obstruction  have been added.    e |liac crest graft hernia and ileosigmoid knotting have  been added.   e Ten commandments of acute large bowel obstruction  have been added.    . Which of the following is not the cause of gangrene in    . The following is true in a plain X-ray of abdomen in   . Caecum can appear as round shadow  . Ileum has valvulae conniventes    The most common cause of intestinal obstruction in  infants aged 6—18 months is:    A. Worms B. Bands patients with parkinsonism, multiple sclerosis,  C. Intussusception D. Adhesions hypothyroidism is:  A. Diarrhoea  Dance’s sign (signe de dance) is a feature of: B. Constipation  A. Worms B. Bands C. Drugs  C. Intussusception D. Adhesions D. Long mesentery of the colon    A. Ultrasound abdomen A. CT scan  B. Plain X-ray abdomen B. Pet scan  C. CT with or without angiogram C. MRI scan  D. MRI D. Technetium scan  The most common cause of intestinal obstruction in 21. Presence of intramural air is diagnostic of:  neonates is: A. Gall stone ileus  A. Bands B. Sigmoid perforation  B. Duodenal atresia C. Duodenal atresia  C. Imperforate anus D. Intestinal gangrene  D. Meconium ileus  22. Following complications can occur after gastro-  The most common congenital anomaly of small jejunostomy except:  intestine is: A. Dumping syndrome  A. Bands B. Intussusception  B. Duodenal atresia C. Volvulus  C. Stenosis D. Stomal ulcer  D. Meckel’s diverticulum  23. Following are the causes of adult intussusception  Features of paralytic ileus include the following except: except:  A. Gross abdominal distension A. Meckel’s diverticulum  B. Pain abdomen B. Submucous lipoma  C. Failure to pass flatus C. Carcinoma caecum  D. Tinkling sounds D. Hypertrophy of Peyer’s patches  Melanosis of lips and mucosa with intestinal 24 The most common anomaly associated with  obstruction should arouse the suspicion of: Hirschsprung’s disease is:  A. Gardner’s syndrome A. Down’s syndrome  B. Turcot’s syndrome B. Hypothyroidism  C. Peutz-Jeghers syndrome C. Meckel’s diverticulum  D. Down’s syndrome D. Anorectal atresia  Which is the factor precipitate sigmoid volvulus? 25. Following are causes of paralytic ileus except:  A. Short colon A. Anastomotic leak  B. Broad attachment at the base B. Retroperitoneal irritation  C. Empty colon C. Hyperkalaemia  D. Long mesentery of the colon D. Fracture spine  ANSWERS   1A 2A 3 B 4C 5 A 6 B 7D 8 A 9 A 10 C   1d 12 C 136C 14 B 15 D 16 B 17 C 18 A 1 B 20D    The rectum starts at the rectosigmoid junction, opposite the third  piece of sacrum. It descends in the sacral hollow, passes through  the pelvic floor, and ends in the anorectal junction, which is  about 4 cm away from the anal verge. Anorectal junction 1s  enclosed by puborectalis muscle posteriorly and in the lateral  aspects. The rectum is 12—15 cm in length (Key Box 31.1).    ¢ Upper one-third is completely covered by peritoneum  (> 11 cm from anal verge) (Figs 31.1 and 31.2)   ¢ Lower one-third (O—6 cm) has no extraperitoneal covering  but has two fascial condensation layers. Posteriorly, the  strong Waldeyer’s layer separates the rectum from lower  sacral pieces and coccyx. At surgery, stripping of this  fascia results in uncontrollable bleeding from sacral  plexus of veins, which is underneath the Waldeyer’s fascia.    ¢ Anteriorly, the weak Denonvillier’s fascia separates the  rectum from prostate and bladder. Stripping of this fascia  results in troublesome bleeding from prostatic venous  plexus.    ¢ Rectum is attached to side wall of pelvis by lateral  ligaments, which contain middle haemorrhoidal vessels.  These need ligation or coagulation during mobilisation of  lower rectum.    ¢ Valves of Houston: Despite the name rectum means  straight, it is never straight in adults. It has one convexity  on the left and two convexities on the right side. There are  3 valves of Houston (prominent mucosal folds), two on  the left and one on the right.    1. Superior haemorrhoidal artery 1s a branch of the superior  rectal artery which is the continuation of the inferior  mesenteric artery. It divides into right and left branches.  The right branch divides into anterior and posterior branches  which supply the rectum (Fig. 31.3).   3. Eventhough it is a part of the large intestine, taenia,  appendices epiploicae and sacculations are absent.   5. Rectal carcinoma has high recurrence because of lack of  serosal layer and close relation to other pelvic viscera.   6. Principal route of lymphatic drainage is upwards towards  para-aortic nodes.    e After investing the upper rectum, pelvic peritoneum is  reflected anteriorly in males urinary bladder—thus form  rectovesical pouch and in females it reflects onto uterus  and thus form rectouterine pouch.   e It is one of the sites of transcoelomic spread of malignant  cells.   e Maligant cells settle down in this most dependent part of  the abdominal cavity and grow.   e lt is diagnosed by per rectal or per vaginal examination   e Pus can be drained through rectum or through posterior  fornix.   2. Middle haemorrhoidal artery, a branch of internal iliac  artery, runs in the lateral ligament of the rectum.    3. Inferior haemorrhoidal artery, a branch of internal  pudendal artery supplies the lower rectum.   1. Upwards to drain into superior rectal veins. These join  inferior mesenteric veins, which in turn drain into the portal  system.    Fig. 31.1: Sagittal section through the male pelvis showing the  location of the rectum and some of its anterior and posterior  relations    2. Across to drain into middle rectal veins, which run in the  lateral ligament of the rectum along with middle rectal  artery. Hence, the lateral ligaments have to be ligated and  divided during resection of rectum.    These veins drain into internal iliac veins (systemic  circulation). Hence, rectum is a site of portosystemic  anastomosis.    ¢ Upper 1/3rd of rectum is completely enclosed by  peritoneum and the middle I/3rd of rectum is covered in  front and on the sides by peritoneum. From these areas,  lymphatic drainage always occurs in the upward direction,  first to (A) pararectal nodes of Gerota followed by superior  haemorrhoidal nodes, middle haemorrhoidal nodes and  nodes at the origin of inferior mesenteric artery.   ¢ From lower I/3rd of rectum, lymphatics spread in the  lateral direction and can involve (B) internal iliac nodes.    Lymph nodes are also present in the hollow of sacrum along    Sympathetic: The fibres come from hypogastric plexus,  which is located at the aortic bifurcation at the level of Ls  Injury to this can cause absence of erection or dry orgasm.  Fibres also come along with inferior mesenteric artery and  Superior rectal artery.    Parasympathetic: (S2, S3, S4) by means of nervi erigentes  from the hypogastric plexus and supply motor fibres to  detrusor. Pain and ability to distinguish flatus and faeces is  because of these fibres. Loss of mucosa of the rectum results    in the loss of these sensations. During division of lateral  ligaments or during anterior dissection of the bladder base,  Injury to nervi erigentes can occur.    ¢ External anal sphincter and puborectalis are innervated by  inferior rectal branches of internal pudendal nerve.    Similar to carcinoma colon. However, precancerous conditions  and risk factors are given as follows.    These are the common anal/perianal conditions which can be diagnosed by inspection (only), or palpation or per rectal examination/  proctoscopic examination    Flexible sigmoidoscope is 60 cm long  An enema is given before procedure    Growth, ulcers, bleeding diverticulae,  polyps, colitis can be diagnosed and  biopsy can be taken    Polyps in FAP, villous adenoma (see page 704)  Ulcerative colitis (see page 677)  Crohn’s disease (see page 683)    Smoking, Alcohol, Diet (see page 709)  Genetic  Colorectal family (see page 705, 706)    Annular variety 1s common at the rectosigmoid junction.  It presents with constipation and intestinal obstruction. It  takes about a year for the growth to completely encircle the  lumen of the gut (napkin ring deformity) (Fig. 31.10).    Fig. 31.11: Fibreoptic sigmoidoscopy showing growth in the  upper rectum—biopsy proved signet ring carcinoma    Diffuse variety is similar to linitis plastica. It develops from  ulcerative colitis. It has a poor prognosis.    Constipation requiring increasing doses of purgatives due  to annular growth at rectosigmoid junction. Always a sense  of incomplete evacuation and altered bowel habits.  Bleeding per rectum, frank blood or mixed with stools is  common. It is painless, never massive and 1s the earliest  symptom of carcinoma rectum. Very often, it is confused  for haemorrhoids.   Early morning spurious diarrhoea is due to accumulation  of mucus overnight in the ampulla of rectum (dilated middle  portion of rectum), which causes an urgency to pass stools  but results in passage of only mucus with minimal stools.  There is always a sense of incomplete defaecation.    Fig. 31.12: Signet ring carcinoma colon—relatively poor  prognosis (Courtesy: Dr Laxmi Rao, Head, Department of  Pathology, KMC, Manipal)   Early morning  spurious diarrhoea  Bloody slime  Sciatic-like pain  Abdominal distension  Loss of weight/  abdominal distension  Strangury    Rectal examination: In every patient with bleeding per  rectum, rectal examination has to be done. More than 90%  of cases of carcinoma rectum can be diagnosed by rectal  examination. Always feel for the ulcer or growth, nodularity,  induration, fixity to posterior sacrum, anterior bladder base  and laterally to lateral ligaments. Look for the blood stains  specially in ulcerative cases. It 1s also possible to feel the  lymph nodes in the mesorectum in cases of lower third  carcinomas.    the anterior wall of the rectum, accurate assessment of the  growth can be done with one finger in the rectum and the  other in the vagina. Large Krukenberg tumours if present  can also be felt by vaginal and rectal examinations.   Evidence of metastasis: Palpable nodular liver, para-aortic  lymph nodes, ascites and supraclavicular nodes (Troisiers    Rectal cancer presenting as a fistula in ano is the  equivalent to a perforated colonic cancer. It is a bad    Villous adenomas (benign) present as bleeding per rectum  with mass coming out sometimes of anal canal. They have  frond like appearance. They are very friable, bleeds on touch  easily and they are bulky. Biopsy is a must. If it is benign,  they can be removed through the rectum—submucosally.    ulcerative colitis and Crohn’s disease produce diarrhoea,  blood in the stools and multiple ulcers. They are not  indurated. Whatever it is, biopsy is a must before doing a  major surgical resection. Ulcers in ulcerative colitis are  typically described as pinpoint ulcers. In Crohn’s they are  fissure type or patchy with a cobblestone appearance.   A 22-year-old girl was treated with iron tablets for anaemia  due to occasional bleeding per rectum. She was treated with  metronidazole because she was passing mucus along with  the stools. She developed intestinal obstruction after 6  months during which time a surgeon was consulted. Rectal  examination revealed a large growth, fixed all around. She  died after 6 months because of advanced disease. The case  illustrates the importance of rectal examination and that  carcinoma of the rectum often occurs in young patients also.    without obstruction. An ulcer over the surface will mimic  carcinoma. Biopsy is mandatory because amoebomas are  completely curable with antiamoebic treatment.   4. Tuberculous proctitis: Usually patients have pulmonary  tuberculosis. Submucosal abscess rupture and result in  ulcers with undermined edges. Hypertrophic tuberculosis  with stricture can also occur. Biopsy 1s mandatory before  resection.   5. Endometrioma: It presents as constipation, bleeding per  rectum especially bleeding during menstruation. Typically  young females between the age of 20-40 years are affected.  It produces constricting lesion in the rectosigmoid junction.  Mucosa 1s intact as seen by sigmoidoscopy. Treatment is  biopsy followed by treatment of endometriosis.   ¢ Site: Commonly occurs 1n the anterior wall of lower rectum,  an area of mucosal change.   ¢ Mucosa: It is erythematous, heaped up and bleeds on touch.   « The cause, even though not clear is probably due to trauma  by anal digitation. Today, it 1s believed that it 1s due to  internal intussusception or anterior wall prolapse.   ¢ Clinical features are passage of blood and mucus in stools.  Mucosal prolapse may also be a feature.   e A biopsy must be done to rule out carcinoma rectum.   ¢ Treatment is conservative: Avoidance of constipation and  straining may treat the prolapse.   e Ittakes eighteen months for a growth to encircle the lumen  of rectum, as in annular strictures at the rectosigmoid  junction.   e¢ Anteriorly, it involves prostate, seminal vesicles and  bladder base in males, vagina and uterus in females.   ¢ Posteriorly, sacral plexus gets involved in late cases and  causes sciatica-like pain.    3. Blood spread  It results 1n secondaries in the liver, lungs, etc. They are  common in young patients with anaplastic variety and ir  colloid carcinoma.    4. Peritoneal spread  e Jt results in ascites, carcinomatous nodules over the  peritoneum, etc.    A Growth confined to the rectal wall  B Growth involving perirectal pad of fat and tissues. No nodes  are involved.  B1: Invading muscularis mucosa  B2: Invading to or through serosa  C Nodes are involved  C,: Local lymph nodes—pararectal  C,: Distal lymph nodes—along the course of blood  vessels  D Distant spread—liver, lungs, etc.   Fig. 31.14: The three cardinal stages of progression of the  neoplasm    ¢ Dukes’ A: 5-year survival 1s 90 to 100%  ¢ Dukes’ B: 5-year survival is 50 to 80%  ¢ Dukes’ C: 5-year survival is less than 50%    T1 tumour invasion through the muscularis mucosae, but  not into the muscularis propria   T3 tumour invasion through the muscularis propria, but not  through the serosa (on surfaces covered by peritoneum) or  mesorectal fascia    The prefix ‘p’ indicates that the staging is based on  histopathological analysis and ‘y’ that it is the stage after  neoadjuvant treatment, which may have resuited in  downstaging   Posterior sacral infiltration and anterior bladder base  infiltration — surgery can be very difficult and dangerous  (uncontrollable bleeding from sacral plexus of veins and  prostatic plexus of veins in males. Hence preoperative  chemoradiotherapy followed by surgery is done  Involvement of mesorectum carry poor prognosis hence the  circumferential resected margin is important (see page  780)    Investigations (Table 31.2)  1. Proctoscopy: It should be done in all cases of bleeding per    rectum. It is done as an out patient procedure. The left lateral   position can also be used. The growth appears as an ulcer   with everted edges. A biopsy ts taken to prove the diagnosis.    sigmoidoscopy fail to give a diagnosis due to spasm of the  colon. When carcinoma arises in multiple polyposis coli or  ulcerative colitis, barium enema is done to rule out  synchronous malignancies.    biopsy can be taken to prove the diagnosis.  Increased levels of carcinoembryonic antigen  indicates metastasis.    metastasis such as secondaries in the liver, ascites with para-  aortic nodes, etc (colloid carcinoma).   UT2 Penetration of the muscularis propria but not through  to the mesorectal fat    ¢ It helps to detect the lesion, detect metastasis in liver.   ¢ To know the fixation to adjacent structures (ureter, uterus,  bladder base, etc. hydronephrosis).   9. MRI: Both MRI and EUS are good for assessment for  T-staging, MRI has following advantages  ¢ It is better for T—3 and T—4 stage  ¢ High resolution MRI is better for assessment of circum-  ferential resected margin (CRM).  ¢ Itis also good for nodal staging   2. Palliative resection is worth doing even in the presence of  metastasis, when there is obstruction.   3. Even though surgical treatment 1s the main modality,  radiotherapy and chemotherapy are beneficial.   4. At surgery, ligation of vascular pedicle is done first to  prevent tumour embolisation.   5. Ligation of bowel, proximal and distal to the tumour helps  to prevent transluminal dissemination.   6. 40% ethanol is used as tumouricidal agent to prevent suture  line recurrence. Solutions such as dilute povidone iodine  have been used to irrigate rectal bed after APR to prevent  recurrence but without much success.   8. An attempt should be made to perform a Total Mesorectal  Excision (TME)—which improves quality of life (see Ten  commandments and Key Box 31.6).   9. Colonic pouch: The splenic flexure is mobilised first. A 6  cm limb of sigmoid or descending colon is folded and a  pouch is created. A colotomy is made at the apex of the  pouch. Linear cutter is used to staple the pouch on itself to  create a common lumen. A second fire of the stapler may  be necessary. This pouch now acts like a neorectum.  ¢ A double-stapled anastomosis as described or ahand-sewn   anastomosis is then performed. A diverting loop ileostomy  is used routinely for these ultra low anastomoses.    Laparoscopic anterior resection and total mesorectal excision  are well established procedures now. It is possible because of    1. Should do TME in all cases of mid and lower carcinoma  rectum  2. Should excise the entire mesorectum (contains fat, lymph  nodes and superior rectal blood vessels)  3. Should do the dissection with electrocautery or scissors  4. Should open the posterior plane between visceral and  parietal layers of endopelvic fascia—Holy plane of Heald  or avascular plane  5. Should exert good traction and counter traction to develop  the planes  6. Should excise the entire mesorectum circumferentially-  minimum of 5 cm of the CRM  Should be inside the pelvic plexus laterally  Should excise Denonvilliers fascia anteriorly  9. Should excise rectosacral ligament so as to reach the  pelvic floor  10. Should do proximal diversion ileostomy   3. Impotence, urinary incontinence and retrograde ejaculation  are lesser after TME.    advances in the laparoscopic instruments, high definition  cameras and improved technology.    ¢ A 30° camera allows a beautiful magnified view of the  entire dissecting field specially low down in the pelvis.  Thus, no part of the laparoscopic procedure 1s blind.   ¢ Laparoscopic surgery allows the surgeon to adopt the  principle of no touch technique. At the same time with  better visualisation of the rectum and mesorectum all  around, it permits a good dissection.   ¢ Jt is not uncommon in the open method that the specimen  gets torn due to traction. It has been proved that this tearing  is much less in laparoscopy.   ¢ Better preservation of the pelvic autonomic nerves, low  anastomotic leak and low mortality rates.   ¢ For laparoscopic low resections, one need not mobilise  splenic flexure but sigmoid can be used for anastomosis.   ¢ Wound infection, paralytic ileus are much less after  laparoscopic surgery than open surgery.    ¢ Port site recurrence: Local tissue trauma due to trocars,  tumour manipulation, tumour behaviour are the factors  responsible for port site recurrence. Tumour spillage, tumour  cell aerosolisation due to sudden loss of pneumoperitoneum,  tumour spillage during extraction and immunosuppression  during pneumoperitoneum are a few factors responsible for  port site recurrence. This can be minimised by using bag  for extraction, minimal handling of the tumour, to avoid  tearing the specimen.    Cancers arising in the distal 15 cm of the large bowel are  included under this heading. These cancers behave almost  like colonic cancers. The resection 1s the best treatment in  early stages for cure. However, anatomy of the rectum, with  its retroperitoneal location, narrow pelvis in males and  proximity to the urogenital organs, autonomic nerves, and anal  sphincters, makes surgical access and resection relatively  difficult. In advanced cases neo-adjuvant therapy—chemo-  radiotherapy is given to downstage the disease and then  resections are done.   The treatment of rectal cancer has changed significantly  over the past 20 years with the aim of cure with multimodality  treatment and preserving the sphincter. Broadly the various  surgery done for carcinoma rectum are as follows.   1. Anterior resection: Refers to removal of rectum and  sigmoid colon. Indicated in cases of carcinoma rectum  above peritoneal reflection: It can be low anterior  resection when colorectal anstomosis is done below the  peritoneal reflection or high anterior resection where the  anastomosis 1s above peritoneal reflection. In this procedure  rectum and sigmoid colon is removed along with  mesorectum which contain lymphatic channels. Sphincter  function is preserved. Sigmoid colon has to be removed    because in cases of inferior mesenteric artery ligation,  blood supply becomes inadequate and anastomosis may  leak. Colorectal—colo anal anastomosis is done with or  without pouch. Stapler anastomosis 1s the choice for low  resections. Bowel is clamped and transected just proximal  to this point (Fig 31.15 to 18). When the anastomosis is  very low (just above anorectal ring), a protective diversion  ileostomy 1s done which 1s closed after 10 weeks. Patient  will have increased frequency of stools, incontinence or  soilage. Diet restrictions may help these patients. Total  mesorectal excision should be the aim. It improves the  survival rates, decreases incidence of local recurrence.    is also called as Miles-Walker’s operation It 1s indicated  when the growth 1s too low involving the anal sphincters,  poorly differentiated cancers which are very low. The  patient is put in Lloyd Davis position (supine with  lithotomy). Two surgeons operate simultaneously, one  from the abdomen and one from the perineum. Abdomen  is opened first and the growth is mobilised from the sacrum  and from the urinary bladder. Pelvic dissection 1s carried   Figs 31.18A to D: (Ato C) Parts of the stapler, (D) shows as the  Stapler is tightened, upper and lower rectum come together and  the anvil fits in very well in the circular stapler    STRUCTURES REMOVED IN APR  e Growth with entire rectum and anal canal.  e Fascia propria with pararectal nodes.  e Two-thirds of the sigmoid colon and mesocolon with  lymphatics and lymph nodes.  e Muscles and peritoneum of pelvic floor.  e Wide area of perianal skin, with part of ischiorectal fossa.    by abdominal surgeon till levator ani muscles. At this stage,  anus 1s closed by a purse string by perineal surgeon.  Rectum and anal canal is mobilized from below. The entire  specimen of rectum with meso rectum and anal canal and  the nodes are removed. It is followed by Permanent End-  Colostomy by bringing the sigmoid colon outside in the  left iliac fossa (sphincter sacrificing surgery). Thus  complete excision of the rectum, anal canal, mesorectum,  and lymph nodes are done through abdomino- perineal    Fig. 31.25B: Proximal colon is mobilised up to the transverse Fig. 31.26: Low anterior resection specimen—Stapler  colon so that it can be easily brought down without tension anastomosis has been done—you can see the complete  doughnuts   & B C  Figs 31.27A to C: Schematic representations of resection of the rectum. (A) AP resection followed by colostomy, (B) High anterior  resection followed by anastomosis, (C) Lo w anterior resection with colostomy and closure of rectal stump—Hartmann’s procedure—  in large bowel obstruction. The colostomy is closed and colorectal anastomosis is done after 4 weeks   LOCAL EXCISION OF CARCINOMA RECTUM  e Mobile tumours less than 4 cm in diameter  e Less than 40% of rectal wall involvement  e Located within 6 cm of anal verge  e Lesion should be T1 or T2  e No vascular or lymphatic invasion  e No nodal involvement—preoperative MRI or EUS.    incision. Usually one drainage tube is inserted brought out  through perineal wound.    3. Local excision: If the tumor is confined to the submucosa  (uT1, NO), excision can be done by a transanal approach.  The explanation being these cases will have less than  8%, lymphatic metastasis. In our country majority of  patients do not fall in this category. It is indicated for mobile  early tumors that are less than 4 cm in diameter, that  involve less than 40% of the rectal wall circumference,  with good histology like well or moderately differentiated  histologically, and that are located within 6 cm of the anal  verge. and with no vascular or lymphatic invasion.    Recent change in APR: In the conventional APR dissection  is within the levator ani and levator ani is preserved. The  change is APR with removal of levator ani—it is called extra  levator APR. The resected specimen has a waist—tt is called  cylindrical APR.    Locally advanced growths present with severe pain, bleeding  and with subacute intestinal obstruction. Temporary loop  colostomy is done in the left iliac fossa by bringing the sigmoid  colon outside. Postoperatively, radiation and chemotherapy  are given.    This 1s indicated in old and  debilitated patients who may not  withstand APR. The rectum is  excised, the lower end of the  rectum 1s closed and a colostomy  is performed. When the growth 1s  slow-growing, this operation gives  good palliation.    suturing. It can be done by transanal, trans-sacral or trans  sphincteric approach.  ¢ Trans-sacral (Kraske’s) is for posterior rectal tumour.    ¢ Colonoscopic polypectomy: All adenomas and potentia  adenomas should be removed. Pedunculated polyps are  removed by snare. Small polyps can be removed by colc  or hot forceps and hot or cold snare. Bleeding can be  controlled by injection of epinephrine, electrocautery, clips  or endoloops.   ¢ Endoscopic mucosal resection: It is indicated for flat.  sessile lesions, not more than 2.5 cm in diameter. First the  lesion is elevated by injecting saline or hypertonic dextrose  with epinephrine. The lesion is elevated, cut and removed  or sucked and cut.   ¢ Endoscopic submucosal dissection: Technically more  difficult. Principle of the procedure is same. More chances  of perforation.   ¢ Endoscopic stents for decompression for malignant  obstruction: Large bore colorectal decompression tubes  are available. They can be passed with or without endoscope  with a guidewire. They are used to relieve obstruction so  that bowel preparation can be done and one stage treatment—  resection and anastomosis can be done. Self expandable  metal stents—SEMS has been used as palliation in cases  of large gut obstruction. Quality of life is slightly better  but no increase in survival rate of patients. Stent migration,  tumour ingrowth, overgrowth, perforation and bleeding are  the other complications.   ¢ Laser therapy: Like oesophageal carcinoma, lower rectal  carcinoma with obstruction can be treated as a palliation  by Nd:YAG laser. It cannot be used in acute cases. Compli-  cations are perforation, bleeding, fistula formation, etc.    1. pT 1-2NOMO do not require any adjuvant treatment, such  patients can be kept on follow-up with routine 3 monthly  CEA and annual CECT thorax/abdomen/pelvis.   2. pT3NOMO or node positive disease requires adjuvant  treatment in the form of concurrent chemoradiotherapy and  chemotherapy.   Example: 2 cycles of FOLFOX (5-FU + Leucovorin +  Oxaliplatin) — concurrent 5-FU/Leucovorin and radiation  — 2 more cycles of FOLFOX.   3. It is preferable to add Oxaliplatin in the chemotherapy  regimen if nodes were positive for metastatic disease.  Although in older population (> 65—70 years) it might be  of less benefit.   bed, pre-sacral nodes, internal iliac nodes. External iliac  nodes can also be included in T4 tumors.  Radiation dose 1s usually 45—50 Gy given over 5 days a  week for 5 consecutive weeks. Another 5—9 Gy boost to  the tumor bed ONLY can be considered especially with  adverse features like lymphatic emboli, close margins.    Rationale: In locally advanced cases where the surgeon feels  that complete resection may not be feasible or sphincter saving  will not be possible, neoadjuvant chemoradiation can be  attempted thus saving the patient from having a permanent  colostomy bag and also a better curative outcomes.    Cora  ADVANTAGES OF PREOPERATIVE  RADIOTHERAPY  ¢ Decreased tumour seeding at surgery  e Increased radiosensitivity due to more oxygenated cells  * Conversion of APR to LAR   ¢ Generally clinically T3--4 tumors which may or may not  be node positive are eligible candidates for neoadjuvant  chemotherapy.   1. A typical course of neoadjuvant therapy comprises  concurrent 5-FU/Capecitabine and radiation. A dose of  45-50 Gy is used to treat the pelvis including the growth  and the draining lymphatic regions followed by 5 Gy boost  to the tumor itself:   2. Following neoadjuvant therapy, patient should be re-evalua-  ted using CT/MRI for possibility of resection.   3. Surgery is usually considered after 6-8 weeks following  neoadjuvant therapy as the maximal response to the treat-  ment may take up to 2 months.   4. Further adjuvant treatment ts to be given following surgery  depending upon the histopathological report.    Rectal cancers are more radiosensitive and colonic  cancers are more chemosensitive.    ¢ Major cause 1s a positive margin on pelvic side wall. This  is the reason why preoperative chemoradiotherapy should  precede excision of T3 and T4 lesions with TME.   ¢ Chemoradiation, surgery, local palliative treatment, pelvic  exenteration (resection of rectum and bladder—    Opening of the colon to the exterior, either temporary or  permanent, for the drainage of faecal matter 1s called  colostomy.    A. In cases of acute left-sided colonic obstruction, proximal  half of right transverse colon is brought out through the  upper part of the right rectus abdominis muscle. Later,  radical resection of the left colon 1s done followed by  closure of the colostomy.   B. In cases of traumatic or congenital fistula affecting the left  colon, temporary colostomy 1s indicated (Fig. 31.36). The  loop of the colon which is brought outside 1s held in place  by a glass rod. This is passed through the transverse  mesocolon and held by rubber tubing. This rod is removed  after 10 days.   C. Colostomy/Ileostomy is done if a distal colorectal  anastomosis gives way (Figs 31.29 and 31.30).    Fig. 31.29: Sigmoid  colon is brought out and  a glass rod connected to  rubber tube is used to  hold it out    Transverse colostomy is bulky, contents are semiliquid  and difficult to manage—Try to avoid it.    1. Should mark the colostomy site in the standing and sitting  positions   2. Should be at least 3 cm away from the bony landmark  (anterior Superior iliac spine)   3. Should be at least 3 cm away from the midline incision.  Otherwise, colostomy will contaminate the incision site  (Fig. 31.31)    4. Should close the paracolostomy space within the abdomen  to prevent herniation of the small intestines    5. The colostomy should be matured after the midline incision  is completely closed and sterile dressings applied    Should be in flush with skin surface in the left iliac fossa  Should excise a disc of skin for a permanent colostomy  The stoma should be brought out through the rectus muscle  Should avoid bringing stoma outside through scar tissue    It is indicated after abdominoperineal resection where the  end of sigmoid colon is brought outside in the left iliac  fossa as permanent colostomy (Figs 31.31 and 31.32,  Key Box 31.10). The colostomy site should be 3 cm away  from anterior superior iliac spine so that colostomy bag  can be fitted properly (Figs 31.33 and 31.34).    In this the adjoining walls of the intestine are crushed. Both  ends of the loop are defunctioned. This type of colostomy  is not frequently done now. It was done earlier for sigmoid  volvulus, resection of colonic stricture, etc.    Congenital: In Hirschsprung’s disease and anorectal  anomalies, temporary colostomy is done first.  Carcinoma: Following APR, permanent end-sigmoid  colostomy is done.   Colonic fistulae: Fistulae due to diverticulitis, Crohn’s  disease or due to tuberculosis.    Distal bowel takes complete rest, regains normal size and  bacterial colonisation is reduced. It becomes empty and sterile  so that chances of leakage at a later operation 1s reduced.   Fig. 31.33: Disposable colostomy bags are available with or  without flange. Depending upon the size of the stoma, it can be  cut open so as to fit into the colostomy, Fig. 31.34: A colostomy  bag has been applied. It can be reused    Fig. 31.35: Paracolostomy hernia: Herniation of bowel from the  side of colostomy   Figs 31.36A and B: Colostomy gangrene. It is mandatory to  inspect the end of the colsotomy everyday in the postoperative  period to check for vascularity. If it is gangrenous and if it is re-  tracted it is better to open the patient again and refashion colos-  tomy   Fig. 31.37: Colostomy prolapse because sutures would have  given way where it was anchored to aponeurotic layer. The patient  also had violent postoperative cough    Fig. 31.38: Burst abdomen due to contamination of the wound  from colostomy—proper care should be taken to isolate  laparotomy site and colostomy till complete healing of laparotomy  wound takes place    Bleeding, necrosis, retraction, prolapse, parastomal hernia  and colostomy diarrhoea are some complications.  Colostomy obstruction, gangrene.    Colostomy should be done by an experienced surgeon  as patient has to live with the colostomy life long.    Protrusion of the mucous membrane or the entire rectum  outside the anal verge. This condition 1s common in children  and elderly patients.    Prolapse can be of two types: Partial prolapse and complete  prolapse.    In this variety, the protrusion is between 1.25 and 3.75 cm  outside the anal verge (Fig. 31.39).  ¢ It is usually a mucosal prolapse.    In infants, it is due to undeveloped sacral curve and in  children it can be secondary to habitual constipation.    . It can follow an attack of whooping cough or excessive    . It can follow an attack of diarrhoea resulting in loss of fat    . In adults it is common in females mostly due to Torn    Digital reposition: In infants, partial prolapse 1s temporary.  The mother is advised to push the prolapse inside after  lubricating with lignocaine jelly.    the rectum. It causes aseptic fibrosis. Thus, mucosa gets  tethered to the other layers.   It is defined as protrusion of the rectum for more than 3.7.  cm outside the anal verge. Very often, it 1s the entire rectun  which protrudes out on straining, sometimes along wit!  the peritoneal sac.    It is composed of the two levator ani and a puborectali:  muscle.   Levator ani originate from pelvic side walls anc  sacrospinous ligament. It suspends the rectum 1n a muscula  sling which ends when puborectalis angulates rectum.  Puborectalis muscle takes origin from posterior aspect o1  pubis, forms a sling around rectum and return to posterio!  pubis.   Contracted puborectalis is responsible for normal acute  anorectal angle and it is critical for maintaining  continence. Thus during coughing, sneezing, anorectal  angle becomes more acute, increasing continence.    Various supports of the rectum keeps the rectum in place.  Failure of one or more of these factors may precipitate rectal  prolapse (Fig. 31.42). They have been enumerated in the  following lines.    Pelvic floor: Weakness of pelvic floor can be due to birth  injuries or due to defective collagen maturation.    of pelvic fascia on each side of the rectum. Excessive  mobility of these ligaments may be the contributing factor  for prolapse rectum.    rectovesical pouch is often found in prolapse rectum. In  all cases of complete prolapse rectum, please look for the  deep rectovesical pouch and if it 1s present it should be  obliterated.    These are useful in patients who have complaints of prolapse  rectum, constipation, incontinence.   ¢ Squeeze pressure: [t is maximum voluntary contraction  pressure minus resting pressure. It 1s 40-80 mm above  resting pressure. It reflects function of external anal  sphincter.    Fig. 31.41: Pelvic floor anatomy—weakness of the pelvic flooris Fig. 31.42: Prolapsed rectum—diagrammatic representation,  an important cause of prolapse rectum sometimes confused for prolapsed haemorrhoids    2. Function of pudendal nerve and puborectalis nerve:  These are nerve conduction studies which can reflect   1. Common in elderly women who are multipara.  Probably, it is due to repeated birth injuries to the  perineum causing damage to the nerve fibres. As age  advances, muscles become weak. This, together with fatty  degeneration of the muscle, results in prolapse rectum.   2. Excessive straining causes weakness of the supports of  the rectum.    Further progression 3. Defective collagen maturation results in failure of rectal  support by levators and pelvic fascia.    Descending perineum syndrome. 4. Presence of deep rectovesical pouch and excessive  mobility of the rectum (mesorectum) predisposes to    Irreducible prolapse — Ischaemia_ prolapse of the rectum.  5. Many people believe that prolapse of the rectum starts as  an intussusception in the first stage, initiated by certain  factors such as diarrhoea, constipation and disorder of  the pelvic floor. The process starts with anterior wall of  Fig. 31.43: Pathogenesis of prolapse rectum rectum, where supporting tissues are weakest (Fig. 31.45).    Fig. 31.45: Prolapse of the rectum with uterus: Procidentia—  not an uncommon presentation specially in multipara women   e 3rd degree perineal tears—weaken the internal sphincter  and pelvic floor.    'In fact, the surgeon should see the prolapse of the rectum outside when  patient strains to make a clinical diagnosis.    ¢ Some degree of incontinence of faeces and flatus 1s always  present. It gives rise to urgency and perianal soiling.    Palpation of prolapse between finger and thumb reveals  double thickness of tissue, especially anteriorly because  of deep pouch of Douglas.    ¢ Large third degree haemorrhoids  ¢ Large polypoid tumour  ¢ Prolapse of sigmoid colon    Proctitis, ulceration and rarely bleeding  Gangrene of the rectum (Fig. 31.48)    Safe procedure to correct with minimal morbidity and  without mortality. They are classified as perineal  procedures and abdominal procedures.    Delorme’s procedure (reefing the rectal mucosa): In this,  the prolapse 1s completely everted, mucosa Is stripped and  muscle coat is plicated. Mucosal continuity 1s maintained  by suturing anal canal mucosa below to the rectal mucosa  above. This is an easy operation to do in elderly patients.  However, relapse rates are high and it does not correct the  defect.    of the prolapsed rectum with part of sigmoid is excised  followed by anastomosis of part of the sigmoid to the anal  canal from below. To improve continence, plication of  levator ani and puborectalis muscle is done. Urgency and  incontinence are the features because of removal of  rectum.    . Thiersch wiring: In this operation, a steel wire or a thick    silk suture is applied all around the anus after reducing the  prolapse. The knot is tightened around a finger. Patients  with poor surgical compliance benefit from this operation.    Fig. 31.48: Fixing the mesh to the rectum—mesh is sutured to  the sacrum posteriorly and sutured to the sides of the rectum    However, breakdown of the wire, perianal sepsis and anal  stenosis are the complications.    upwards and Is sutured to the sacrum posteriorly with the  help of a polyvinyl alcohol sponge kept behind the rectum.  The sponge is sutured posteriorly and laterally to the walls  of the rectum. Dense fibrotic reaction occurs resulting in  fixation of the rectum to the sponge.    rectosigmoid junction is sutured to the sacrum by using  Teflon sling, below the sacral promontory. One  complication of this operation is constipation due to  rectosigmoid angulation. Hence, sigmoidectomy has been  suggested along with this operation.    mesh can be kept behind the rectum. This is sutured behind,  to the sacrum and then to the posterior and lateral surfaces  of rectum. Laparoscopic method of fixing the mesh has  become popular. This is the procedure of choice today.  Constipation is one of the complications of mesh rectopexy.  Hence, some resect sigmoid with this procedure (Goldberg  operation) (Fig. 31.49).    Laparoscopic mesh rectopexy has become gold  standard—fast recovery, less pain, short hospital stay.    Mesh rectopexy with resection is ideal for patients with  constipation or patients with a redundant sigmoid colon.  High operative risk patients—Thiersch wiring—anal  encirclement.    in patients with incontinence. Here, perineal proctectomy  and posterior sphincter enhancement is done.    It is 3 cm long, starts as the continuation of rectum, passes  through pelvic diaphragm and ends at the anal verge (skin).    It is the continuation of circular muscle fibres of rectum  and ends 0.5 cm below pectinate line.   Internal sphincter with fibres of external sphincter and  puborectalis which maintain the anorectal angle, form the  anorectal bundle, and maintains continence.   Its fibres are transversely placed. Motor fibres come from  presacral plexus.   It is formed by striated muscle fibres intermingled with  longitudinal muscle fibres of the rectum which get attached  to the skin of perianal region.    branch of internal pudendal nerve and perianal branch o  the 4th sacral nerve (motor to levator ani also).  It is voluntary and gives temporary continence.    Anal canal is developed from fusion of post-allantoic gut  with proctodeum.   The junction of these is the dentate line or pectinate line  Anal valves of Ball are remnants of proctodeal membrane.  At the level of dentate line, the mucosa is folded in the  form of longitudinal columns—colummns of Morgagni.   In between the columns of Morgagni, 4-8 anal glands open  into the small anal sinuses.    Table 31.3 Comparison of anal canal above and below the dentate line    Definition  Dilated plexus of superior haemorrhoidal veins, in relation to  anal canal.    1. Standing posture: It has been told nicely that varicosity is  the penalty for verticality against gravity. It is also true for  haemorrhoids. It is true that animals do not develop  haemorrhoids.   Thus man’s upright posture and absence of valve in the  portal system with other factors precipitate development  of haemorrhoids   2. Haemorrhoidal veins and their branches are the thin veins  which pass through submucosa of the rectum. They get  compressed due to contractions caused by rectal muscula-  ture (the sphincters) during the act of defaecation.   3. Genetic/ familial factors: Absence of valves, or congenital  weakness of the vessel wall are few other factors  contributing for the haemorrhoids   4. Diet: Adiet deficient in fibres which prolongs the gut transit  results in constipation and small hard pellet like stools. The  hard stools compress veins and result in haemorrhoids.    1. Carcinoma of rectum, by blocking the veins, can produce  back pressure and can manifest as piles.   3. Pregnancy, due to compression on superior rectal veins or  due to progesterone which relaxes smooth muscle in the  wall of the veins, can cause haemorrhoids.    Current view: Latest theory is that haemorrhoids occur  due to caudal displacement of anal cushions. It 1s due to  recurrent trauma, shearing forces, loss of elasticity. Thus  normally the cushions retract after defaecation.    Classically situated in the 3, 7, 11 o’clock positions (Fig. 31.51)   ¢ Superior haemorrhoidal artery (vein) gives 2 branches on  right side and | branch on left side. Hence, piles are two  on right side and one on left side.    ¢ Painless bleeding—fresh bleeding occurs after defaecation  Splash in the pan. This causes chronic anaemia. Haemorr-  hoids which bleed are called Grade I haemorrhoids.   ¢ The capillaries of the lamina propria are only protected by  a single layer of epithelial cells. Hence, minor trauma  precipitates bleeding.    ¢ As the straining increases, the haemorrhoids partly prolapse  outside. After defaecation, it returns back (Grade IT) or can  be digitally replaced (Grade III haemorrhoids).   ¢ Permanently prolapsed pile outside (Grade IV haemorr-  hoids). The patient complains of pain or discomfort.   e Discharge of mucus and soiling of perianal skin—pruritus  by prolapse of haemorrhoidal cushions and mucosa  (Figs 31.52 to 31.54).    ¢ Per rectal examination is done mainly to rule out carcinoma  rectum or other causes of bleeding per rectum. Haemorr-  hoids cannot be felt by rectal examination unless they are  thrombosed or fibrosed.   ¢ Proctoscopy: As the obturator is removed, piles prolapse  into the lumen of proctoscope as cherry red masses.   Fig. 31.52: Grade Ill Fig. 31.53: Grade IV Fig. 31.54: External pile    ¢ Sigmoidoscopy and proctoscopy are done to rule out  Narasimhan, Senior Consultant Surgeon, Mysore, Karnataka)    1. It can cause chronic anaemia. Rarely, massive bleeding * Metronidazole 400 mg, 3 times a day for 5 days.  can occur because of portal hypertension.   2. Aprolapse outside presents withsevere pain in the perianal  region—piles gripped by internal sphincter results in  venous congestion and oedema followed by strangulation. 3.  Such patients are treated by: 4. Thrombosis and fibrosis    A. Nonoperative treatment: It is indicated in Grade I and  Grade II piles which are not causing significant bleeding  or discomfort (Key Box 31.13).   NONOPERATIVE TREATMENT  e Fibre supplementation  e Increased fluid intake  e Bulk purgative—laxatives—isapgul husk, etc.  e Reading in toilet to be discouraged (respond to call and  do not strain)  e Encourage to lose weight  Remember as FIBRE    B. Injection of sclerosant: 5% phenol in almond oil is injected  into submucosa above the dentate line. Hence, it is painless.  It produces aseptic thrombosis of pile mass and 1s indicated  in Grade I. The injection is perivascular.   Fig. 31.61: Ligatures are applied at the base of the haemorrhoids  and they are being cut with cautery   BAND LIGATION: WISDOM LINES D. Operative treatment: Haemorrhoidectomy. Open  ¢ Bands should be applied 1-2 cm above dentate line method, closed method, stapler haemorrhoidopexy.  to avoid pain.   ¢ Bands should not be applied in patients who are taking HAEMORRHOIDECTOMY (Key Box 31.15)  anticoagulants.   e Bands should not be applied for immunocompromised  patients without broad spectrum antibiotics to avoid life-  threatening sepsis.   e Should not band all the three pile masses at same time.  Quadrant by quadrant with a gap of 2 weeks is ideal.   e If severe pain, fever and urinary retention develops after Excistonal haemorrhoidectomy produces more dis-  band (sepsis), examine under general anaesthesia and comfort (pain) than stapler haemorrhoidopexy but lesser  remove band. recurrences).   Excision of the pile masses up to base is indicated in  Grade II and Grade III hemorrhoids. It can be done by  3 methods: Open, closed and with stapler (vide infra).    e Haemorrhoids occur due to downward prolapse of vascular  cushions into and beyond anal canal.   e Urgency and tenesmus following stapled haemor-  rhoidoscopy responds well to oral nifedipine.   e Metronidazole is the most important agent in reducing  pain after haemorrhoid surgery.   e Grade | and II can be injected: Injections should be  perivascular, submucosal and above the level of dentate  line.    e Grade IV require initial conservative treatment followed by  surgical procedure.    I. Open method: Milligan-Morgan ligature and excision  (Figs 31.60 to 31.62)   ¢ A tube drainage is provided so that the blood (oozing)  can escape outside.    Always leave a bridge of skin in between the excised pile  masses to prevent anal stenosis.    ¢ Basic steps are the same as above  ¢ Cut mucosa and skin edges are sutured with absorbable  catgut sutures.    1. Strong analgesics, in the form of injection pethidine or  morphine, are given to reduce the pain.  2. Antibiotics along with metronidazole are given to prevent  secondary infection.  . Bulk purgatives are given to avoid constipation.  4. Sitz bath twice a day is given by using warm saline or  KMn0Q,j solution.    They can be classified into early and late complications. Acut  retention of urine and haemorrhage are early complication  Anal stricture, anal stenosis, anal fissure and incontinence ar  the late complications. Few complications are described belov   1. Retention of urine is common in men due to severe pait  It can be managed by treating the pain and hot wate  fomentation in the suprapubic region. Catheterisation 1  done as a last resort.   2. Reactionary haemorrhage is more common. It is due to  loose ligature or some opened up bleedings. Generally stop  by pressure packing. Otherwise, under anaesthesia, ligat  or cauterise bleeding point   3. Secondary haemorrhage can occur due to infection. |]  manifests 6 to 8 days later. If the bleeding is significant  exploration in the operation theatre may be necessary. |  should be done under anaesthesia. With good illuminatio)  it is possible to identify the bleeding points and ligate them   5. Anal fissure, submucous abscess, and incontinence cai  occur after haemorrhoidectomy.   6. Wound infection: Minor degree of wound infection doe:  occur and can be treated with sitz bath, antibiotics anc  regular dressings.    ¢ A novel method for 3rd and 4th degree haemorrhoids was  introduced by Dr Antonio Longo in 1997.   ¢ After reduction of prolapsed piles, a prolene purse string  Suture is applied circumferentially, taking good mucosal  bites 3 cm above dentate line.   ¢ By maintaining traction in the tails of suture, the stapler is  fully closed and fired.   ¢ Thus a circular ring of mucosal tissue above the level of  dentate line 1s removed. Internal haemorrhoids are not  removed, external haemorrhoids are also not removed  (eventually they regress).   ¢ Thus, 2 rows of staples and 28 staples are present.   Fig. 31.65: Purse string suture is applied Figs 31.66 and 31.67: Maintain traction in the tails of purse string suture and  stapler is closed and fired    Pan US Courtesy: Dr BH Anand Rao, Dr Vinayak Shenoy K,  & uN 7 Dr Ramachandra L, Dr Hartimath B, Dr Prashanth Shetty,  1 Oe Dr Saritha Kanth (Consultants), and Dr Akshay Nadakarni   e Advantages: Lesser postoperative pain, earlier return to  normal activities, better satisfaction.   e Side effects: Mucoid discharge with tenesmus (5 patients),  recurrences in 3 patients.   ¢ No long term side-effects such as anal stenosis or chronic  pain as may happen with open haemorrhoidectomy.   ¢ Rare complications such as intra- and postoperative  bleeding. Occasional cases of postoperative fissures,  mucosal discharge, persistent tenesmus, infection and long-  term complications such as_ rectovaginal fistulae, polyps  at stapler line and recurrence.    In open haemorrhoidectomy: If wound ts like clover, it  is over. In stapler haemorrhoidectomy: [If doughnut is  complete, it ts time to celebrate.    ¢ Doppler principle is used to identify the feeding artery to  the haemorrhoid mass and it Is ligated   e After this, the needle is inserted into the lumen of the  proctoscope   ¢ The artery is ligated by figure of eight suture. Thus, main  artery supplying the haemorrhoid ts blocked   ¢ Procedure is simple out patient procedure, no pain, no  anaesthesia. No blood loss, early recovery   ¢ Safe in all types of patients including patients with serious  morbidity   It is bluish in colour because it is a thrombosed vein or venule   can be given over the swelling and clot can be evacuated.   e In other cases, it will resolve within 5 days after fibrosis/  Suppuration.   ¢ Mostly originate from the anal gland opening at the base  of the anal crypts. This is eryptoglandular theory of   ¢ Other source of anorectal sepsis is foreign body, trauma.  sexually transmitted diseases for lower level abscesses.  Crohn’s disease and carcinomarectum with perforation may  form pelvirectal abscess (supralevator).   ¢ Typically patients presents with high grade fever with chills  and rigors. On examination, a tender indurated swelling is  found in the perianal region or in the ischiorectal fossa.   ¢ Culture usually shows £. coli in about 70-80% of cases.    ¢ It occurs due to infection of anal glands in the perianal  region.   ¢ It may be due toa boil, anal gland infection or thrombosed  external pile.   ¢ It produces severe pain, throbbing in nature and on  examination a soft, tender, warm swelling is found.   ¢ Rectal examination reveals a tender, boggy, swelling  under the anal mucosa.    ¢ Collection of pus under the mucous membrane of rectum  or anal canal.   ¢ It can also be due to infection of injected haemorrhoids.  It can be drained using proctoscope.    ¢ Collection of pus in the ischiorectal fossa, which 1s lateral  to rectum and medial to pelvic wall.   ¢ Bounded above by levator ani and inferiorly by pad of  fat in the ischiorectal fossa.   ¢ Ischiorectal fat 1s poorly vascularised. Hence, it is more  vulnerable to infection.   ¢ Abscess occurs due to spread of perianal abscess or due  to blood-borne infection.    CAUSES OF ANORECTAL ABSCESS  e Infection  e Irritation (Crohn’s disease, ulcerative colitis)  e Immunity low (diabetes, AIDS)   ¢ Per rectal examination is painful and bogginess can be  appreciated on the side of the lesion.    Under anaesthesia, a cruciate incision  (Figs 31.70 and  31.71) 1s made and the 4 flaps are raised. All the pus 1s  evacuated and the wound is packed with iodine roller gauze  and left open. Edges of the skin are trimmed to leave an  opening so that drainage of pus occurs freely. It heals with  granulation tissue within 10—15 days. Appropriate antibiotics  are given for a period of 5 to 10 days.    It 1s a pelvic abscess, which 1s drained through the rectum.  The common causes are pelvic peritonitis, appendicitis, septic  abortions, etc. The details of the causes, clinical features and  the management are discussed in page 647.    ANORECTAL ABSCESS  e Common causes such as boil or infected sebaceous cyst  has to be ruled out first.    e Remember other causes such as infection following  haemorrhoidal injection or band ligation.   e Uncommon causes such as foreign body or penetrating  trauma also can give rise to anorectal abscess.   e Last but not the least, AIDS and diabetes have to be  ruled out in all cases of anorectal abscess.    Abnormal communication between anal canal and rectum with  exterior (perianal skin) is called as fistula in ano. Even though  multiple openings are seen in the perianal skin, the internal  opening is always single.    1. They occur due to persistent anal gland infection, which  results in anorectal abscesses, rupture inside as well as  outside resulting in a fistula. Once a fistula occurs, it persists  because of infection and absence of rest to the part. As there  are many anal glands, often, problem persists inspite of  initial treatment of one fistula.    tuberculosis have 1—2% chances of developing multiple anal  fistulae. Whenever a patient presents with multiple anal  fistulae, it 1s but natural to think of tubercular aetiology.  Such fistulae are not indurated and there is watery  discharge without pus.    fistulae in ano. This type of carcinoma has worst prognosis.  Rectal examination should be done in every patient with  anal fistula.    II. Park’s classification (Fig. 31.73)  1. Intersphincteric  2. Trans-sphincteric  3. Supralevator (internal opening is situated above the  anorectal bundle).    ¢ Goodsall’s rule: A fistula, with an external opening in the  anterior half of anus within 3.75 cm tends to be direct type  and in the posterior half, indirect type or curved and  sometimes horseshoe type. It may communicate with the  opposite side (Fig. 31.74).   ¢ External opening is found at the bottom of a depressed  area or with granulation tissue or it is seen discharging pus.   ¢ Internal opening may be felt on digital examination as  indurated area or sometimes can be seen with proctoscopy  or after sigmoidoscopy.   * The entire track may be palpable as indurated cord like  structure.   ¢ Endorectal ultrasonography and MRI seem to identify  internal openings and fistula. However, they can be  selectively used 1n deserving cases.    It is indicated in low fistula (internal opening below the  anorectal bundle). A probe is passed through the external  opening into the rectum and along the length of this tract the  fistula is laid open. It is done under anaesthesia. The wound is  left open and allowed to heal by granulation tissue developing  from the floor of the fistula (marsupialisation).  Intersphincteric and low trans-sphincteric fistulas of recent  origin are treated by fistulotomy and marsupialisation.    All chronic fistulae (low) are treated by fistulectomy by  excising the entire fibrous tissues and tract. Here also, the  wound is kept open. This can also be done for posterior semi  horseshoe and horseshoe fistula. Some incontinence can  occur.    It is indicated in high fistula in ano. The internal opening is  situated above the anorectal bundle. Hence, during  fistulectomy, there is a chance of injury to the anorectal bundle  and may cause incontinence. Temporary or permanent  colostomy may be necessary. If there is a cause, treat the cause.  Surgery of intersphincteric fistula and trans-sphincteric fistula  may result in incontinence.    Ksharsutra is an ayurvedic term. It is a medicated thread passed  through the entire tract and both ends are tied and tightened  once a week so that by 6 weeks it cuts through (Key Box  31.20).    1. Biological agents: The basic principle is to plug and seal  the tract. It allows the ingrowth of healthytissue. Thus initially  fibrin glue was used but results are not good in long term.  Porcine small intestinal mucosa or porcine dermal collagen  also has been used. Results are not satisfactory.    2. Video-assisted anal fistula treatment (VAAFT): A novel  sphincter-saving procedure for treating complex anal fistulas  (more details in page 1179).   e Aim is correct localisation of the internal fistula opening  under direct vision   e Astapler or cutaneous-mucosal flap to close the internal  opening after endoscopic treatment of the fistula.   e Fistuloscopy is done under irrigation and followed by an  operative phase of fulguration of the fistula tract   Fig. 31.79: Fistula in ano— Fig. 31.80: Fistula in ano, reinforcement with cyanoacrylate.    2. Should try to define the type of the fistula in relation to  SETON sphincter  e It is a Latin word. Seton means bristle—material such as 3. Should define low or high fistula  thread, wire, or gauze that is passed through subcutaneous 4. Should rule out special types of fistula  tissues. 5. Should conduct thorough examination again under  e Varieties of materials used as setons—plastic tubes, infant anaesthesia before surgical procedure  feeding tubes, proline suture material, medicated thread 6. Should do MRI in difficult, recurrent and complicated  used in Ayurvedic method—Ksharasutra. Ksharsutra is a fistula  sanskrit phrase in which Kshar refers to anything that is 7. Should do fistulotomy in all intersphincteric fistulae and  corrosive or caustic; while sutra means a thread. trans sphincteric involving 30% of the voluntary  e Loose setons for long-term palliation. Examples: Fistulae musculature  associated with Crohnn’s disease, complicated recurrent 8. Should do fistulectomy in low fistula—it will open up the  fistulae. infected cavity better even though wound will be bigger  e Cutting tight setons: Used in complicated high fistulae than a simple fistulotomy wound  wherein a fistulotomy may result in anal incontinence. So, 9. Should use setons in high fistula or complicated fistula  seton is tied, patient will tighten it everyday for a period of wherein a fistulotomy may result in recurrence or  8-12 weeks till it comes down. Once it comes down, seton incontinence or when staged procedures are planned  is removed. This will decrease the chances of incontinence. 10. Should explain to the patient about possibility of some  e Main advantage of seton is it eliminates sepsis by keeping degree of incontinence and take consent for colostomy  the track open. in high fistula  e Disadvantage is that patient will always feel a foreign body  sensation in the rectum and anal canal.  See Key Box 31.21 for recent advances in fistula surgery. Aetiopathogenesis (Fig. 31.81 and Key Box 31.22)    See Ten commandments for fistula in ano. ;  ¢ 90% of anal fissures occur In the posterior part of anal    canal and 10% anteriorly. It 1s initiated by hard stool causing  : = i | a crack. As a result of this, defaecation results in pain. Anal  fissure Is more common posteriorly in the midline because   of relative ischaemia.  Definition ¢ Due to pain, internal sphincter spasm takes place which  Longitudinal tear 1n the lower end of anal canal results in makes constipation worse resulting in a chronic fissure.  fissure in ano. It is the most painful condition affecting the °¢ Anterior fissures occur in elderly women secondary to  anal region. Commonly seen In young patients. repeated pregnancies. This is due to damaged pelvic floor    and lack of support to anal mucous membrane. Acute fissure  in females may occur after vaginal delivery.   Fissure away from midline should raise the possibility  of Crohn’s disease, sexually transmitted diseases, etc.    Severe pain during and after defaecation, burning in nature,  lasting for about 2 to | hour because of which defaecation  is postponed.   Stools are hard, pellet like and there is a drop of blood or  streaks of fresh blood.    Drop of blood ts due to anal fissure. Splash of blood is  due to haemorrhoids, bloody slime is due to carcinoma.    Sudden onset—example after vaginal delivery or following hard stools  Acute pain in the anal canal, severe burning after defaecation with  bleeding   Usually responds to conservative treatment—local application of  glyceryl trinitrate (GIN) 0.2% 3-4 times a day or diltiazem 2% twice  a day    Sentinel pile refers to tag of skin at the outer end of the  fissure.   In some cases, fissure may be associated with a small  perianal abscess resulting in worsening of pain.    1. When the buttocks are spread apart, a longitudinal tear and   . Per rectal examination can be done (with lignocaine jelly  application) and sphincter spasm can be appreciated.    Avoid constipation—encourage fibre diet, mild laxatives  and not to postpone defaecation.   Chronic pain in the anal canal, burning after defaecation  with bleeding—few exacerbations   Sphincter spasm, chronic canoe shaped ulcer in the lower  anal canal   Responds to conservative treatment but effect is temporary  surgery is the treatment of choice    e Drug releases NO (nitric oxide) at a cellular level  and mediates relaxation of internal sphincter   ¢ Oral nifedipine 20 mg, twice daily. Topical  nifedipine also helps    ¢ Glyceryl trinitrate (0.2%) topical application: Significant  headache and 50% recurrence are drawbacks. It reduces  spasm, increases vascular perfusion.    ¢ Purified botulinum toxin injection into internal spincter:  It inhibits presynaptic release of acetylcholine from  cholinergic nerve endings and cause temporary paresis of  striated muscle. Cost, perianal thrombosis and sepsis are  drawbacks. Injection produces prolonged but reversible  effects, thus avoiding permanent injury (Key Box 31.23).    ¢ Calcium channel blockers: Nifedipine, diltiazem oral and  topical applications (2%) also have been used.    1. Lateral anal sphincterotomy of Notaras (or dorsal) 1s  the best alternative procedure. Here internal sphincter is  divided away from fissure either in right or left lateral  positions. The procedure can be easily done by using a  bivalved speculum in the anal canal. This is the procedure  of choice. Sphincterotomy should be limited to the  length of fissure to avoid incontinence.    2. Fissurectomy and local advancement flap: This 1s  indicated in persistent, chronic, nonhealing fissure. After  excision of the fissure, the resulting defect in the anal canal  is closed by a small (rhomboid) advancement flap. This  should be considered not as a first line of treatment.  Recovery from this operation takes much more time than  other treatments for anal fissures.    Manometry of anal sphincter can be done before  procedure especially in women because 30-40%  of patients develop incontinence following lateral  sphincterotomy    relieves the spasm and the fissure heals. Rarely, in female  patients it may result in incontinence. It is not a  recommended treatment nowadays.    ¢ Pilonidal sinus means nest of hairs in Greek. Also called  Jeep-bottom because it was very common in jeep drivers.  ¢ More common in dark people than fair people.  ¢ [tis an acquired condition, commonly found in hairy males.  It is acquired due to following reasons  — Appears between the age of 20 and 30 years  — Hairy men are more affected  — The hair follicle is never demonstrated in the wall of  the pilonidal sinus but hair is the content of pilonidal sinus.  ¢ Hair accumulates due to vibration and friction causing  shedding of the hair. Thus, it accumulates in the gluteal  cleft and enters the opening of the sweat glands.  ¢ Pointed end of the dead hair is inside (blind end of the  sinus).    ¢ External opening of the sinus seen just above the anal verge  in the midline over the coccyx (Key Box 31.24).    Diagnosis  Osteomyelitis of the coccyx is the only differential diagnosis  for pilonidal sinus. Hence, X-ray of the coccyx should be taken.    ¢ Inject methylene blue to demonstrate branches of the sinus  followed by excision of the sinus. The patient 1s positioned  prone with buttocks elevated (Jack knife position).    After excision there are two methods to treat the wound—  Open and closed methods (Key Box 31.23).    Open method: The wound is left open after excision  followed by regular packing with iodine or eusol gauze  pieces (Key Box 31.25).    It may take 3-4 weeks for the healing of pilonidal sinus.  Regular sitz bath is also given.    Closed method: The wound is closed by ‘z’ plasty. This  method carries 10-20% chances of recurrence. Rhomboid  flap (Limberg flap) can be raised to close the defect also.    Karydakis procedure: Primary procedure is to remove all  the sinus tracts and their branches till sacral bone. In this  operation, semilateral incision is made around the sinuses  and flaps mobilised to excise all the sinuses and their  branches. Then tension free closure is done. Compared to  elliptical incision, this incision and closure has decreased  chances of skin necrosis.   Bascom’s technique: In this procedure, an incision 1s given  laterally, not in the midline. After raising the flaps, wide  excision of the infected sinuses and tracts is done followed  by closure of the midline openings. Lateral wound 1s left  open (in the conventional operation, midline wound ts left  open).    PILONIDAL SINUS  It is acquired condition—popularly called Jeep-bottom.  Common in hairy men  Multiple sinuses communicating with each other  They open to the exterior by multiple openings  The direction of the sinuses is cephaloid  Recurrent abscesses which rupture are common  Excision with or without marsupialisation, flap closure or z  plasty are the treatment options.  Inspite of the adequate surgical procedures, recurrence is  common.    injected into the opening. The excision area is marked also with  methylene blue   Figs 31.84A and B: (A) Specimen of excised tissue with sinus  track, and (B) shows tuft of hair which was present within the sinus   Fig. 31.85: Neglected, persistent, recurrent pilonidal sinus.  Fistula probe is introduced into one of the openings followed  later by demarcation of the fistula   ¢ In this region, totipotential cells persist for a longer period  compared to rest of the area. Hence, it 1s the site of teratomas.    ¢ 20% of the cases are stillborn babies. It is common in a  female child.   ¢ Presents as a swelling in the sacrococcygeal region pushing  the rectum anteriorly.   ¢ The surface of the swelling ulcerates. Many cystic areas  are present in the swelling.   ¢ The swelling ts fixed to the sacrum and coccyx from which  it is impossible to separate/isolate.   Fig. 31.86: Squamous cell carcinoma Fig. 31.87: Melanoma anal region _ Fig. 31.88: Melanoma resembled prolapsed piles but    They are not uncommon tumours which present with  bleeding per rectum, burning and itching in the anal region.  The diagnosis is obvious in many cases once buttocks are  separated or by digital examination.    predisposing factors. Local excision or APR (abdomino  perineal resection (APR) is the treatment with external RT  in appropriate cases (Fig. 31.86).    keratinising, squamous cell carcinoma. Treatment is similar  to squamous cell carcinoma.    e \t is squamous cell carcinoma in situ of the anus.   e |t is associated with human papilloma virus type 16 and  18 (HPV 16,18).    malignant melanoma of anal canal—bluish/blackish ulcer  in the anal canal. APR 1s potentially curable in early cases  of melanoma. If metastasis is present, the prognosis 1s poor.  So, only local excision is done so as to provide palliation  but colostomy is avoided (Figs 31.87 to 31.90).   4. Adenocarcinoma is rare. It can occur from the anal glands  In pre-existing anal fistula. APR with 5-FU and radiation  therapy is indicated.    Please note that Bowen’s disease, Paget’s disease or  verrucous carcinoma, squamous and basal cell  carcinoma can also occur in the skin of anal margin.    4. Lymphogranuloma inguinale: A sexually transmitted  disease affecting both male and female patients. Initially    pararectal lymph nodes are enlarged followed by  development of multiple rectal strictures.    5. Inflammatory bowel diseases: Both ulcerative colitis and  Crohn’s disease result in rectal strictures (5—10%).    6. Rare: Congenital, amoeboma, carcinoid, endometriosis,  tuberculosis, CMV colitis (Figs 31.91 and 31.92).    Increasing constipation is the characteristic feature of stricture  of the rectum. It may be associated with hard stools, bleeding  and pain in some cases. Per abdominal examination may reveal  loaded colon with scybalous masses. Rectal examination can  detect a stricture.    It is mandatory to rule out carcinoma rectum which ts  the most common cause of stricture.    1. Conservative treatment includes bulk purgatives, a  vegetable diet.  2. Regular dilatation may be necessary for the strictures  situated low in the rectum and anal canal.  . Intractable strictures need to be resected.  4. Treatment of the primary disease.   e Distension of rectum causes tonic contraction of external  sphincter. This 1s controlled by cerebrum and the centre 1s  in the lumbosacral region of the spinal cord.   ¢ Faeces in contact with anal canal stimulates the specialised  nerve endings. Nerve endings are also present in the  puborectalis.   Figs 31.91 and 31.92: Rectal stricture due to CMV colitis  (Courtesy: Dr Satyanarayana N, Dr Srinivas Pai, Dr Madhu, KMC,  Manipal)    808  ¢ High pressure in the anal canal (25—120 mmHg) and angle  between rectum and anal canal (80°) are the important    ¢ It marks the junction between the rectum and anal canal.  ¢ It 1s formed by puborectalis, highest part of internal  sphincter, longitudinal muscle and external part of sphincter.    1. Traumatic: Injury to the anorectum due to sharp penetrating  objects occurs due to accidents.   ¢ Damage to the internal and external sphincter can occur  due to Lord’s dilatation’, a procedure done for fissure in  ano. However, most of it is temporary.   3. Mass in the anorectum: Prolapse piles, prolapse rectum  and carcinoma rectum may produce temporary incontinence  which subsides after surgical procedures.   4. Neurological causes: In females, pudendal nerve  neuropathy which occurs due to chronic straining may  result in incontinence. Spinal injuries, spina bifida,  meningomyelocoele are associated with anal incontinence.   6. Childhood/congenital causes: Anorectal malformations,  Hirschsprung’s disease, spinal bifida, abnormal behaviour.   7. Miscellaneous: Old age (senility), general debility and  faecal impaction Parkinson’s disease, behavioral problem,  etc.    I. Temporary incontinence: Reassurance. Perineal exercises  to improve the tone of internal and external sphincter.    e e e e ¢ e | e e s ® e e e e e e e e e e e ® e . ® e e q  KEY BOX 31.27 Ng    1. Divided sphincter can be reunited, followed by  overlapping of the remaining muscles.    2. Intersphincteric repair of puborectalis sling and plicatior  of the external sphincter.    3.Gracilis muscle can be used to create a new ana!  sphincter by transposing it followed by electrical  stimulation using a pacemaker.    ¢ This condition is characterised by attacks of severe cramp-  like pain arising in the rectum.    ¢ Anxiety status, straining at stools or ejaculation are a few  precipitating factors.    ¢ The pain may be unbearable, may recur at irregular  intervals. It 1s possibly due to segmental cramp in the  pubococcygeus muscle. The pain usually lasts for a few  minutes and subsides (fleeting perianal pain).    Perianal and anal discharge: Anal fissure, fistula in ano,  prolapsed piles, polyps, genital warts are a few conditions  which render the anus moist.    2. Poor hygiene, lack of cleanliness, excessive sweating and  wearing tight and rough underclothing are common causes.    Sexually transmitted diseases such as herpes, anal warts  and HIV infection must be excluded.    ¢ Hygienic measures  ¢ Prednisolone topical cream 1% with antifungal agent  (miconazole nitrate 2%)    Pelvic floor dysfunction also referred to as nonrelaxing  puborectalis syndrome is called as ANISMUS. These  patients present with constipation. [tis a difficult problem  fo treat.    HIDRADENITIS SUPPURATICA  Definition  It is a chronic recurrent suppuration of apocrine glands in the    ¢ Occlusion of the gland ducts result in stasis, bacterial pro-  liferation, abscess, rupture. Common organisms are Sfaphy-  lococcus aureus and anaerobes (somewhat like breast  abscess).   ¢ Anogenital disease is more common in men hence  androgens may play a role in this condition.   ¢ Interestingly, it does not affect above the level of dentate  line nor sphincters.    All diseases resulting in multiple sinuses in and around  perineum are the differential diagnosis such as Crohn’s disease,  tuberculosis, lymphogranuloma venereum, pilonidal sinuses,  actinomycosis, etc.    When 1n doubt, rule out other causes mentioned above and  if necessary a good biopsy from the sinus tract and from  the edge of the sinus.   General measures such as weight reduction, antibiotics,  antiseptic medicated soaps, washing the part with warm  saline or water.   Surgery includes laying open all the openings or wide  excision with or without skin ( radical excision ) and direct  closure or skin grafting of flap reconstruction are the other  choices.    e Pelvic floor anatomy and more details about prolapse  rectum have been added.    e Haemorrhoidal artery ligation has been added.  e VAAFT has been added    . Which of the following are causes of anorectal fistulae  in males except:    . The ideal surgical treatment for growth at 8 cm from  the anal verge is:    . On-table lavage of the intestines for resection and  anastomosis can be done via:    . Local excision of malignant rectal tumour can be done  if:    Above the dentate line, lymphatic drainage goes to:  A. Para-aortic nodes    B. Superficial inguinal lymph nodes  C. Deep inguinal lymph nodes  D.Pudendal lymph nodes    Which one of the precaution to be taken while applying  band for haemorrhoids?  A. Bands are applied in grade | pile masses    B. Bands are applied in grade 4 haemorrhoids  C. Bands are applied below the dentate line    D. Bands should not be applied in patients who are taking  anticoagulants    of the following should not be done?  A. Biopsy of the track    In cases of pilonidal sinus:  A. Hair is demonstrated in the wall    7 D 8 D 9 C 10 D  i 6 te D 13D 20 C    Lower gastrointestinal (GI) bleeding refers to bleeding which  occurs beyond the ligament of Treitz. Bleeding per rectum  may be a manifestation of upper GI bleeding, the causes of  which have been discussed under haematemesis. In this chapter,  bleeding per rectum due to lower GI causes will be discussed.   Lower gastrointestinal (GI) haemorrhage accounts for 1%  of acute hospital admissions each year. Severe bleeding is  that which continues for 24 hours after hospital admission or  that which recurs 24 hours after resolution.    In all, so-called the lower GI bleeding—rule out  3 important causes of upper GI bleeding, namely—  oesophageal variceal bleeding due to portal hypertension,  peptic ulcers—mainly duodenal ulcer bleeds and acute  erosive gastritis-—ac ute gastric mucosal lesions (AGML).    ¢ Haematochezia: Bloody stools (LGIB or rapid UGIB)  ¢ Melaena: Black tarry stools from digested blood. Bleeding  is there for more than 8 hours.  ¢ Massive GI tract bleeding: The bleeding which requires  more than 3 units of blood transfusions tn 24 hours.  ¢ OBSCURE: Bleeding which persists or recurs after initial  evaluation has failed(with EGD and colonoscopy). Two types:  A. Obscure occult: Iron deficiency anaemia, faecal occult  blood positive, no visible bleeding. More than 80%  resolve with no treatment.    COMMON CAUSES OF LOWER Gi BLEEDING ;  Most originate in the colon or rectum—haemorrhoids, polyps,  carcinoma, inflammatory bowel diseases are common causes.  10% from upper intestinal tract   Small intestinal haemorrhage is usually due to arteriovenous  malformations (angiodysplasia), accounting for 70-80%.  Jejunal diverticula, Meckel’s diverticula, neoplasia, Crohn’s  disease, and aorto-enteric fistula following a previous aortic  graft are other causes of bleeding from small intestines.    Peutz-Jeghers syndrome,  Familial polyposis coli (FPC)  ¢ Meckel’s diverticulum  ¢ Hereditary haemorrhagic  telangiectasia (HHT)   e Melaena: Passage of black tarry stools (altered blood) due  to slow bleeding or more proximal source of bleed.   e Haematochezia: Passage of bright red stools with or without  clots.   Prolapse rectum  Fissure in ano  Fistula in ano (rare)  Injuries to the rectum    ¢ Young age group: Piles, tuberculosis, Crohn’s, dysentery  ¢ Middle and old age: Carcinoma, piles, prolapse,  diverticular disease.   ¢ Firm to hard mass in the right iliac fossa: Ileocaecal  tuberculosis.    ¢ Multiple small pinpoint ulcers: Ulcerative colitis  ¢ Large deep flask-shaped ulcer: Amoebic ulcers  ¢ Multiple small polyps: Hereditary polyposis coli.    ¢ Itis the gold standard investigation for lower GI bleeding.  Itcan detect 3 important diseases: Carcinoma, inflammatory  bowel diseases (IBDs) and diverticular diseases (Figs 32.5    to 32.19). It can also detect ischaemic colitis, polyps and  angiodysplasia. It needs to be repeated. In massive  bleeding it can really tax an expert colonoscopist also.   ¢ Colonoscopic adrenaline injections, snaring and  coagulation (Argon plasma coagulation) are therapeutic  advantages.    ¢ Irregular filling defect in the colon: Cancer colon  ¢ Contracted pipe-stem colon: Ulcerative colitis    ¢ Diverticulum in the terminal ileum is Meckel’s diverti-  culum. Multiple ulcers and stricture terminal ileum can  be due to tuberculous ulcer.   ¢ Barium studies have a little value in the presence of acute  haemorrhage. They can be used in intermittent or chronic  bleeding wherein endoscopy has failed to detect the cause.    They are indicated when the diagnosis of lower GI bleeding  cannot be made out. They are more useful where there 1s  active bleeding or obscure bleeding.    is extremely sensitive, can detect as little as 0.1 ml/min of   Less precise but less invasive with least complications. If  ”°M™Tc_tagged RBC scan is positive, then angiogram is used  to localise the bleeding site.    All three vessels-—coeliac, superior mesenteric and inferior  mesenteric arteries are used.   Thus, Meckel’s diverticulum, angiodysplasia, small bowel  tumours, vasculitis, etc. can be diagnosed.    It is an investigation wherein a small camera pill 1s swallowed  to study the entire GI tract, in particular, small intestines  This ‘camera pill’ that is swallowed is disposable   It weighs 4 grams and is 26 mm x 11 mmin size   Parts: Video camera, lens, colour camera chip, 6 light  emitting diodes. As it passes through the entire gastro-  intestinal tract, images are taken.   Capsule endoscopy is useful to detect (observe) small  intestinal bleeds that are missed by routine upper GI scopy  and colonoscopy. The procedure takes very long time for  detection of the lesions.    Fig. 32.22: Crohn’s disease Fig. 32.23: Carcinoma caecum Fig. 32.24: Carcinoma sigmoid colon  y  Fig. 32.25: Colonic polyps Fig. 32.26: Colonic polyposis Fig. 32.27: Intestinal tuberculosis    Fig. 32.28: Tuberculosis of the colon Fig. 32.29: Colonic diverticula Fig. 32.30: Duodenal ulcer    Figs 32.31 and 32.32: Enteroscopy done at laparotomy for suspected case of angiodysplasia of the jejunum—resected successfully  (Courtesy: For all the endoscopic pictures: Dr Filipe Alvares, Gastroenterologist, ex-KMC, Manipal)    Fig. 32.33: Schematic representation of approach to lower GI bleeding  (Courtesy: Dr Prasad S, Associate Professor, KMC, Manipal)    e Extended enteroscopy is the same as enteroscopy but may  take 6—8 hours till the scope travels distally with peristalsis.  Up to 70% small intestine can be visualised.    Conclusion  ¢ Thus it can detect polyps, inflammatory bowel disease  (Crohn’s disease), ulcers and tumours of the small intestine.    ¢ Capsule endoscopy is an excellent tool in the patient who PEARLS OF WISDOM    is haemodynamically stable but continues to bleed.  Reported success rates as high as 90% 1n identifying small  bowel pathology. It 1s usually well tolerated, although it 1s  contraindicated in patients with obstruction or a motility  disorder.    A tagged red blood cell scan can detect bleeds as low as  0.1 ml/fminute but does not provide anatomical details.  Angiogram can detect bleed at 0.5 ml/min. Embolisation  with metal coils can be done to correct bleed. Methylene  blue can be injected to stain target segment of intestine.    ¢ Massive lower GI bleeding 1s defined as haemorrhage distal  to the ligament of Treitz that requires more than 3 units of  blood in 24 hours (Fig. 32.33).    intestines. It needs lots of skill and expertise. It can detect  leiomyoma of jejunum, small intestinal diverticular bleed  causing chronic anaemia, etc.    Diagnosis  Diagnosis is established by colonoscopy, RBC tagged scan  and angiography in most of the cases (follow algorithm).    Initial aggressive resuscitation by fluids, blood transfusion  and treatment of shock.   Emergency colonoscopy and vascular malformations if  detected can be treated by argon plasma coagulation or by  cauterisation.   Therapeutic vasopressin infusion 0.2 units/minute via  angiographic catheter with or without embolisation will  stop or arrest the lower GI bleeds in more than 85% of  cases.   Empty small bowel. Then palpate for hidden lesions.  Intraoperative enteroscopy if no obvious lesion is found.  Endoscopic evaluation of transilluminated gut wall.   Rarely, blind right hemicolectomy/subtotal colectomy or  blind resection of proximal jejunum may be necessary  in obscure bleeding (keeping in mind angiodysplasia).    All the topics related to GI bleeding have been discussed in  the respective chapters. Example: Haemobilia (Key Box 32.4)  in page 547, haemosuccus pancreatitis in page 606, diverticular  disease in page 721, inflammatory bowel diseases in page 677.  Summary and important causes of the GI bleeding are given  here.    Haemorrhoids: These are the common causes. They  cause splash in the pan. It is painless, fresh bleeding. It  is one of the differential diagnoses for anaemia.  Diagnosis is by proctoscopy—as cherry red spongy  masses. Sigmoidoscopy 1s done to rule out proximal  carcinoma. Treated by haemorrhoidectomy.    2. Fissure in ano: A severe painful condition of the anal  canal, results in constipation, hard pellet like stools and  drop of blood. Treated by lateral sphincterotomy.    Rare cause of UGI or LGI bleeding  Triad of Sandblom: Melaena, biliary colic and obstructive  jaundice  External trauma  latrogenic — Transhepatic puncture (PTC, stenting)  — Surgery on biliary tree or pancreas  — After dilatation of biliary strictures, etc.  Endoscopy — Blood emerging from ampulla of Vater    in an elderly patient suggests it could be carcinoma  rectum/colon. Diagnosis is by colonoscopy and biopsy.  Treated by colectomy.    Diverticular disease of the sigmoid colon: Common in  Western patients, diet in poor fibre is mostly the cause.  The diverticuli are acquired herniation of the mucosa,  hence thin. Bleeding can be occult/intermittent or  massive. Diagnosis is by colonoscopy—to visualise the  bleeders, endotherapy can be done by injecting  adrenaline into the bleeding vessel. In emergency  situations, with massive lower GI bleeding, emergency  colectomy is required.    Inflammatory bowel diseases: Commonly ulcerative  colitis and less commonly Crohn’s disease produces  lower GI bleeding. Bleeding 1s intermittent with mucous  diarrhoea, weight loss and malnutrition. Often patients  are young. Diagnosis is by colonoscopy and biopsy.  Initial treatment 1s always conservative—salazopyrines,  steroids, etc. In massive bleeding to save the life—  emergency total colectomy with or without pouch may  be required. In Crohn’s disease, the aim is always to  conserve the segment of the intestine. Resection is  required only if massive bleeding is present. This is rare  in Crohn’s disease.    Angiodysplasia (Key Box 32.5): Vascular ectasia also  called angioma, haemangiomas and arteriovenous  malformations are collectively grouped under  angiodysplasia. Commonly right side colon, 1.e. caecum  and ascending colon are affected. In the small intestines,  jejunum is the most common site. Typically elderly  patients present with intermittent bleeding 1s cause of  anaemia. Usual causes of lower GI bleeding are ruled  out by colonoscopy and other investigations. Suspect  angiodysplasia. A few cases present with massive  bleeding—a difficult problem to treat. Repeat colono-  scopy, capsule endoscopy, angiography, on-table entero-  scopy are the taxing investigations—all may provide no  results-emergency colectomy or intestinal resection of  the suspicious segment may be required.   Caecum and right colon are common sites—Caecum is  the most common site   Small bowel (proximal) is the second common site  Small red mucosal lesions between 2 and 10 mn, flat or  raised lesions—dilated tortuous submucosal veins  Recurrent painless and self-limiting bleeding often  associated with aortic stenosis—Heyde’s syndrome  Colonoscopy is the investigation of choice   They can be treated endoscopically—coagulation with heat  probe, bipolar electrode or laser, etc. but recurrence or  failure Can occur   Surgery by resecting the segment is a definitive procedure  Angiography is rarely positive   These lesions are seen in acute renal failure, von  Willebrand’s disease, HHT    Ischaemic colitis: Elderly, hypertensive patients present  with diffuse abdominal pain, severe innature, with blood  in stools—it is often massive, sometimes moderate. On  examination tenderness may be present on the left side  of the colon. Plain X-ray abdomen supine will show  thumb printing sign due to mucosal oedema and  submucosal haemorrhage. CT scan-colonic wall  thickening with posterior fat shadowing. Colonoscopy  may reveal ulcers or a few changes in the splenic flexure  region. If conservative measures fail such as blood  transfusion, segmental colectomy may be required.    Dysentery: Various dysenteries such as amoebic,  bacillary, Shigella, HIV related—all produce ulcerations  in the colon resulting in blood and mucus in the stools.  Gripping pain, acute in nature with or without fever and  tenderness over the colon—in the right iliac fossa and  in the left iliac fossa are suggestive. Diagnosis is by stool  examination and colonoscopy. Treated with antiamoebic  drugs or antibiotics.    Radiation proctocolitis: Usually occurs with pelvic  radiotherapy, example—radiation given to treat carcinoma  cervix. Most common site 1s rectum. Tenesmus, mucus  and blood in stools common. Proctoscopy reveals  ulceration. Treated with stool softening agents, 5 ASA  (amino salicylic acid) topical or steroid enema.    Adenoma, polyps, familial polyposis coli: They are  common in the colon. All are precursors for carcinoma  colon. Often patients are young with lower gastro-  intestinal bleeding. Diagnosis its by colonoscopy and    Tubercular ulcers: They are never massive bleeders.  Patients are between 20 and 40 years old with blood and  mucus in the stools, loss of weight, crampy abdominal  pain, evening rise of temperature with or without  pulmonary tuberculosis. On examination mass may be  palpable if caecum is also involved (ileocaecal  tuberculosis). Visible step ladder peristalsis indicates  obstruction from a tubercular stricture or obstruction due  to mass. Colonoscopy with visualisation of the terminal  ileum and biopsy 1s the key to the diagnosis. Obstructed  cases can be treated with stricturoplasty in a single  stricture or resection in appropriate cases. Cases without  obstruction are treated with antitubercular treatment.  Crohn’s ulcers: Ileum is the commonest site—rest of  the bowel can also be affected. Transmural inflammation,  multiple ulcers, skip lesions are other features. Diagnosis  is by CT scan, push enteroscopy and biopsy. Treatment  is as for ulcerative colitis (for more details see page 685)  Enteric ulcers: High grade tever—enteric fever patient  who has bleeding after 15 days of fever may be having  enteric ulceration of the Peyer’s patches with bleeding.  In majority of the cases, bleeding is occult and usually  stops once the disease 1s treated, rarely exploration and  resection of the segment may be required in cases of  massive bleeding cases.   Meckel’s diverticulum: Children or young patients,  often bleeds are intermittent, maroon coloured with or  without abdominal pain. Peptic ulceration in the ectopic  mucosal site in the Meckel’s diverticulum causes  bleeding. Colonoscopy is normal. RBC tagged  technetium scan 1s the investigation of choice. It can  pick up bleeding as little as 0.1 ml/min of bleeding.  Exploration and excision of the Meckel’s diverticulum  is the treatment of choice.   Angiodysplasias: Small intestines are the most common  sites of angiodysplasia. They are the differential  diagnosis for obscure bleeds. Angiogram and small  bowel push enteroscopy, capsule endoscopy are the  investigations. Diagnosis is by exclusion.   Small bowel tumours: They are uncommon causes of  lower GI bleeding. But they have to be kept in mind  when the common causes described above are ruled out  one by one. Adenocarcinoma, lymphoma and stromal  tumours (GIST—gastrointestinal stromal tumours) are  the few examples. GIST can affect small intestine. The  mucosal ulcerations cause GI blood loss. Bleeding ts  not massive—can be intermittent and result in anaemia.  Palpable mass sometimes massive which ts bosselated,  anaemia and bleeding are the triad of GIST. CT scan is  the investigation of choice. Resection almost cures the    disease. Degree of malignancy is decided by the mitotic  figures in pathology. Imatinib 1s the drug used in  recurrent cases of GIST or GIST with metastasis. Patients  with liver metastasis will live beyond 5—10 years with  imatinib.    Ischaemic colitis is anon-inflammatory condition affecting  splenic flexure region resulting in ischaemia and lower GI  track bleeding   The splenic flexure region can have relative vascularity.  The exact point is called Griffith’s point (Fig. 32.34).   It is defined as the site of (a) communication of the ascend-  ing left colic artery with the marginal artery of Drummond,  and (b) anastomotic bridging between the right and left  terminal branches of the ascending left colic artery at the    Anastomosis at Griffith’s point is present in 48%, poor or  tenuous in 9%, and absent in 43%.   Thus, it is important that in cases of ligation of inferior  mesenteric artery, there 1s a possibility of ischaemia  developing in that region.    A 28-year-old male patient had an urgency to pass stools  early morning. He collapsed while passing stools, with a  massive bleeding. He was brought to the hospital in a state  of shock. He was resuscitated and blood transfusions were  given. All investigations were normal. He had another bout  of massive bleeding the next day, during which time, even  an angiography could not detect the cause. Urgent  laparotomy was done. A 4 cm small bowel tumour  (haemorrhagic) was excised from jejunum and histology  confirmed it as leiomyoma. Letomyoma is called bleeding  tumour of the small bowel. The case history highlights the    Lower GI tract bleeding refers to bleeding:  A. Below ligament of Treitz   C. It is one of the causes of obscure bleeding  D. Small bowel is the second common site    Which one of the following is the cause for massive  lower GI bleeding?   Which one of these causes bleeding with septic shock?  A. Carcinoma colon   Which one of these causes painless and massive  bleeding per rectum?    Acute appendicitis 1s the most common emergency encoun-  tered by the general surgeons. Men have slightly increased  incidence of acute appendicitis compared to women. Incidence  is 11 per 10,000 persons/year. Appendicectomy is a simple  surgery, no doubt, but sometimes it can be very difficult and  disappointing—sometimes one may not be able to find the  appendix. Hence, appendicectomy should not be taken lightly.  The choice of surgery today 1s laparoscopic appendicectomy—  one advantage being one can look into all quadrants of the  abdomen—not to miss other causes such as perforated duode-  nal ulcer (see later Valentino appendix), etc.    ¢ 1736: Claudius Amyand removed inflamed appendix from  the hernia sac of a boy.    ¢ 1886: Reginald Fitz of Boston identified the appendix as  the primary cause of the right lower quadrant inflammation.  He coined the word appendicitis.    ¢ 1889: Charles McBurney suggested early laparotomy and  removal of the appendix. He also describe the McBurney  point of maximum tenderness.   ¢ Embryologically, the appendix and caecum develop as  outpouchings of the caudal limb of the midgut loop in the  sixth week of human development. By the fifth month,  the appendix elongates into its vermiform shape, hence  called vermiform appendix. At birth, the appendix is  located at the tip of the caecum but due to unequal  elongation of the lateral wall of the caecum, the adult  appendix typically originates from the posteromedial wall  of the caecum, caudal to the ileocecal valve. A few  anomalies are given below:    |. Duplication of the appendix is one anomaly which is  further divided into following ways   Type B: Single caecum and 2 separate appendices  Type C: Double caecum with each one having one  appendix (Figs 33.1 and 33.2)   2. Situs inversus: In this condition appendix is found on  the left side. Adds confusion in the diagnosis of acute  appendicitis   3. Subhepatic appendix: It happens in malrotation of the  gut. Patients with subhepatic appendicitis may complain  of pain in the right lower quadrant. A McBurney incision  is usually given only to find no appendix in that location.  Laparoscopy has the advantage of looking into all  quadrants of the abdomen.    6. Subhepatic appendix is associated with subhepatic  caecum. It occurs due to malrotation of the gut (this  position 1s not depicted in the figure)    e Mesoappendix is the continuation of mesentery of the ileum  above. It comes down carrying blood vessels in the meso-  appendix.   e Appendix has a serosa and a mucosa lined by columnar  epithelium (similar to intestinal mucosa) between which are  the circular and longitudinal muscle fibres.   ¢ Submucosa has rich lymphoid follicles (lamina propria).  The lymphatic tissue decreases as age advances. Hence,    Fig. 33.2: Appendicular duplication and gangrene in one of the incidence of appendicitis is less after the age of 30  moieties years    SURGICAL ANATOMY OF THE APPENDIX! indistinct semilunar fold of mucous membrane, known as   ¢ Itis 8-10 cm long, may vary from 3 to 30 cm in length.  - It is situated 2 cm posteromedial to ileocaecal junction, at | Blood supply of the appendix    the point of convergence of the three taeniae coll. ¢ Appendicular artery is a branch of ileocolic artery.  ¢ Itis the primary cause of lower abdominal pain on the right Accessory appendicular artery of Sheshachalam (a branch  side. of posterior caecal artery) is a branch of ileocolic artery,    which runs in the mesoappendix (Fig. 33.4).  ¢ Veins follow the artery and end in the superior mesenteric  vein, thus draining into portal vein. This is the reason for  2. Pelvic in 20% of cases (4 o'clock) development of pylephlebitis in cases of suppurative  3. Preileal and postileal (2 o’clock) appendicitis.    'Appendix secretes immunoglobulins particularly IgA. So it is not considered as a vestigial organ anymore. However, appendicectomy is not  associated with any immunological compromise.    Suppurative appendicitis can give rise to pylephlebitis  (inflammation of portal venous radicles).    Trace the taenia coli or trace ileal loops at laparotomy.  Taenia coli point to the base of the appendix. However,  surface marking of the appendix is done as follows: Draw  a line from anterior superior iliac spine to the umbilicus.  The junction of lateral 1/3rd and medial 2/3rds of this line  indicates the location of appendix. This 1s the point of  maximum tenderness in appendicitis. This is called  McBurney’s point (Fig. 33.5).    The lymphatic channels which are 4 to 6 in number drain  into itleocolic nodes, ileocaecal nodes and appendicular  nodes in mesoappendix.    vein via tleocolic vein and can result in portal pyaemia, a  very dangerous condition.    region — to be kept in mind in cases wherein appendix is  not found in the right iliac fossa.    It is one of most common surgical emergencies encountered  by general surgeons. Sometimes acute appendicitis can be  dangerous (Key Box 33.1).   Recurrent appendicitis: Recurrent attacks of acute  appendicitis—incidence is 15 to 25%.   Grumbling appendicitis: Low grade recurrent bouts of  colics, vomiting with frequent admission, self-limiting  cases.   Simple appendicitis: [f duration of symptoms is less than  48 hours or imaging does not show any abscess or  phlegmon.   Complicated appendicitis: Acute appendicitis with  perforation or large abscess/phlegmon.  Pseudoappendicitis: Acute ileitis mimics appendicitis  following Yersinia infection. It can also be due to Crohn’s  disease.    The appendix is a cul-de-sac (closed at one end) and can  be easily blocked   The lumen of the appendix is very narrow—1-3 mm in  diameter   Closed loop obstruction: Intraluminal pressure builds up  as the appendicular mucosa secretes fluid resulting in  mucosal ischaemia. Slowly bacterial overgrowth and  translocation occurs    Stump appendicitis: It is the inflammation and infection  of appendicular stump, if a big stump 1s left behind (post-  operative cases). It may require stump appendicectomy. It  is important to ligate and divide at the base of the appendix  to avoid this complication (more so in laparoscopic  appendicectomy).    ¢ Itis more common in white race than in coloured persons.  Young males are affected more often.   ¢ It may be related to Westernisation of food—a diet rich  in meat precipitates appendicitis and a diet rich in fibre  (cellulose) protects the person from appendicitis.    retrocaecal appendix in which case the blood supply is  diminished to the distal portion and may precipitate  appendicitis.    appendix due to faeco-liths, worms, ova, cysts of  Entamoeba causes obstructive appendicitis. It is seen only  In One-third cases.    coli, Enterococci, Proteus, Pseudomonas, Klebsiella and  anaerobes which produce diffuse inflammation of appendix  and cause appendicitis. This seems to be more common  cause than obstruction.    ¢ Symptoms are abrupt, vomiting is more, pain is more  and tenderness is more.    ¢ Appendix looks inflamed, with congested blood vessels.  Tip especially looks more inflamed. As the inflammation  is more severe, the outer aspect looks dull and purulent  exudates may be seen. Areas of blackening or green  colour indicates gangrene or necrosis with perforation.  In acute inflammation neutrophils are dominant and in  cases of gangrenous appendicitis, vascular thrombosis  is a feature. The important pathological events can be  summarised as follows—due to obstruction, the contents  get infected fast and the tension increases. The appendix  becomes a closed loop, which results in septic thrombosis  of vessels. Gangrene of appendix, perforation, peritonitis,  followed by a local abscess can occur (Fig. 33.6).    ¢ In children, greater omentum is very thin. Hence, it  cannot localise the infection. In adults, omentum ts like  a fatty apron which localises the infection.    ¢ In aged patients, because of atherosclerosis, gangrene  occurs very fast resulting in peritonitis. Obstruction is  caused by faecoliths, worms and bands which cause  tenting. Obstructed appendicitis is one of the examples  for closed loop obstruction. Other causes are volvulus,  carcinoma hepatic flexure, etc.   ¢ Common bacteria encountered in acute appendicitis are  Bacteroides fragilis, Escherichia coli, Clostridium  perfringens, Streptococcus faecalis, Pseudomonas  aeruginosa, etc.    The peak incidence is in the second and third decades. Very  uncommon before the age of two.   ¢ Ulceration of the appendix results in slow bacterial  invasion of lymphoid tissue.   e This is seen in two-thirds of the cases. Hence, more  common than obstructive theory   e The decrease in the incidence of enteric fever in the  Western world has decreased incidence of acute  appendicitis—a support for infective theory   e In many cases of appendicitis, the appendix is not dilated  (against obstructive theory)   ¢ Pain is severe, colicky type, initially felt in the umbilical  region and it is due to distension of appendix. This is a  visceral pain. After a few hours, the pain localises to the  right iliac fossa. It is a somatic pain which is due to  inflammation of parietal peritoneum. This is called shifting  pain of acute appendicitis (Fig. 33.7). This is called  migratory pain—wmost reliable symptom of acute  appendicitis.   ¢ Normal appendix is mobile. So, the site of maximum pain  and tenderness can vary.    ¢ Vomiting occurs once or twice due to reflex pylorospasm.  Itcontains stomach contents. However, it is never frequent  such as in intestinal obstruction.   ¢ Appendicitis is unlikely in patients with normal appetite.  Usually patients have anorexia.    ¢ Fever is of low grade (around 1 00°F) and indicates bacterial  inflammation.    Pain first, followed by vomiting and then by fever is called  Murphy ’s! triad of symptoms of acute appendicitis  (Murphy’s syndrome).    'Can you find out what is Murphy’s sign and Murphy’s punch test?    Haematuria is uncommon and it is due to inflammatior  of retrocaecal appendix which irritates the ureter in the  retroperitoneum.   Constipation is the usual feature, except in pre- and post-  ileal appendicitis, where they produce diarrhoea due tc  irritation of ileum.    Cough tenderness (Fig. 33.8) indicates inflammation of  parietal peritoneum. This is an important physical sign  which differentiates acute appendicitis from right-sided  ureteric colic.   Tenderness and rebound tenderness are present at  McBurney’s point. Rebound tenderness is called  Blumberg sign. It is due to inflammation of the parietal  peritoneum. This physical sign can be elicited in all cases  of peritonitis.   Guarding and rigidity are present in the right iliac fossa.  However, guarding and rigidity of back muscles (erector  spinae) indicates retrocaecal appendicitis.   Rovsing sign: Palpation of left iliac region of abdomen  produces pain in the right iliac region. It 1s because of  displacement of colonic gas and small bowel coils  impinging upon the inflamed appendix (Fig. 33.9).    VARIATIONS IN ACUTE APPENDICITIS  . Retrocaecal: Silent (no rigidity in the right iliac fossa)  . Pelvic: Causes diarrhoea  . Postileal: Causes diarrhoea—called missed appendix    . Subhepatic: Manifests as pain in the right iliac fossa, very  difficult to remove from gridiron incision    5. In pregnancy: The location of the pain is shifted higher  up and laterally    and pubic symphysis. It is due to irritation of lower  abdominal nerves.    6. Cope’s psoas test: Seen in retrocaecal appendicitis.  There will be irritation of psoas major which produces  flexion at the hip. If any attempt is made to extend the  hip, it produces pain.    8. Features of generalised peritonitis are seen only when  there 1s a rupture. Gangrene and perforation is more  common in elderly patients because of atherosclerosis. In  infants, omentum Is very thin without much of fat. Hence,  diffuse peritonitis occurs very fast.    10. Per vaginal examination: Presence of ovarian mass,  tenderness on movement of cervix, adnexal tendemess  may suggest obstetric pathology.    1. Total WBC count is almost always increased above 10,000  cells/mm’, in most of the patients (95%).  ¢ A very high white blood cell count (> 20,000/mm?)   2. Urine examination is mainly to rule out urinary tract  infection, haematuria and sometimes pyluria.   3. C-reactive protein is elevated in any inflammatory condition  such as appendicitis. Elevated in the first 12 hours of acute  inflammation very non-specific.   4, Plain X-ray abdomen erect is taken to rule out perforation  and intestinal obstruction. It may show dilated small bowel  loops in the right iliac fossa.    Presence of faecolith is highly suggestive of acute  appendicitis in plain X-ray.    5. Abdominal ultrasound to rule out other causes including  gynaecological causes. Ultrasound can demonstrate a non-  compressible, aperistaltic tubular organ with a thick wall.  It can be used to elicit probe tenderness (sensitivity of 85%,  specificity 90%).   ¢ Appendicolith, pericaecal fluid collection or inflammation  can be diagnosed—indirect features of acute appendicitis    6. CECT—Contrast Enhanced CT scan is the investiga-  tion of choice (sensitivity 90%, specificity 90%), specially  when diagnosis 1s not established or in unclassic cases. All  the findings mentioned in the ultrasound can also be defined  by CT scan (Fig. 33.11).   ¢ Helps to rule out carcinoma caecum, duodenal perforation,  acute pancreatitis, etc.   ¢ In children—better to avoid it for the fear of radiation  exposure and risk of cancer developing at a later date   ¢ Expensive, long time for the contrast to reach the site    To avoid negative appendicectomies, many scoring systems  have been developed considering signs, symptoms and  investigations. Most commonly used Alvarado scoring  system is given in Table 33.1.    Score less than 5 Not sure  Score 5-6 Compatible  Score 6—9 Probable  Score more than 9 Confirmed    ¢ Even though Alvarado scoring is highly suggestive of  appendicitis, it is only a simple and cost-effective scoring  system. This can be applied when sophisticated investiga-  tions such as ultrasonography and CT scan are not available.    appendicitis of & hours duration. On examination, she had  McBurney tenderness but a vague mass was palpable. It  is unusual for an appendicular mass to appear within &  hours following appendicitis. Ovarian pathology was  considered and gynaecological opinion was requested. It  was normal. CT scan was done. It revealed mucocoele of  the appendix (8 cm size). She underwent lower midline  laparotomy and it was removed.    CT scan gave a correct diagnosis and it guided the  treatment policy.    Surgical wisdom: Symptoms, signs (tenderness in  McBurney point) with increased total counts, often you dc  not need any imaging tests.    Innumerable conditions may mimic some signs of appendicitis.  A few important conditions have been considered here.    A. Enterocolitis is common in children. It presents with severe  diarrhoea with blood and mucus in the stools.   B. Meckel’s diverticulitis can present with abdominal pain,  vomiting, fever—signs and symptoms are similar to acute  appendicitis (difficult to differentiate clinically).   C. Worm ball is common in children in the developing  countries. However, features of intestinal obstruction will  be present.    A.Right-sided ureteric colic: Haematuria, severe pain from  loin to groin, absence of cough tenderness help in excluding  acute appendicitis.    B. Amoebic typhlitis 1s associated with diarrhoea, blood in  the stools and tenderness in left iliac fossa (Manson Barr’s  amoebic point of tenderness).    C. Torsion of undescended testis: Absence of testis in the  scrotum clinches the diagnosis.    E. Yersinia ileitis: Acute, self-limiting inflammation of appendicitis. However, it is common in elderly females.    the ileum caused by Yersinia pseudotuberculosis. D. Pain in the right iliac fossa and tenderness is due to dilatec  intestinal loop peristalsis as 1n ileocaecal tuberculosis o1  carcinoma caecum. Presence of an irregular, hard mass  suggest carcinoma caecum.    A. Ruptured ectopic gestation: Missed periods, features of  haemorrhagic shock (pallor), extreme tenderness on  movement of cervix during per vaginal examination  clinches the diagnosis.    In middle age (Fig. 33.14A to D) Chlamydia trachomatis and Neisseria gonorrhoeae  A.Acute pancreatitis: Inflammatory exudate collects and culture to be done.    gravitates in the right iliac fossa resulting 1n pain, guarding  and rigidity in the right iliac fossa. History of alcohol intake,  severe backache and tenderness 1n the epigastrium helps in  diagnosing acute pancreatitis.    right side of the abdomen due to similar causes mentioned  above.   Fig. 33.14A to D: In middle age (see text) Fig. 33.15A to D: In females (see text)    e Incidence of acute appendicitis is more common in AIDS  patients—4 fold than non-AIDS patients   Any female patient with right-sided lower abdominal pain  Should undergo a gynaecological examination to rule  out the causes mentioned above, before subjecting to  appendicectomy.   4. Preherpetic pain of 10th and 11th dorsal nerve is located ° 4 30-year-old lady was diagnosed to have acute  over the same area. Marked hyperaesthesia 1s present.  5. Purpura and bleeding disorders.    appendicitis with classical features—pain, fever,  vomiting and tenderness in the McBurney’ point. A  gynaecological examination revealed pelvic infection.  An infected copper T was removed which was the cause   KEY BOX 33.4 of abdominal pain.  CHILDREN AND ACUTE APPENDICITIS 7 © A 22-year-old man underwent appendicectomy for right-  * Appendicitis is rare under 2 years of age because lymphatic sided abdominal pain. At laparotomy, appendix was  lipsue me eee \gloned © inet tiinnay normal. However, it was removed. He continued to have  * SIONS. are. Ol VERY mien OMe. . a abdominal pain. An ultrasound of the abdomen revealed  ¢ Greater omentum is very thin. Perforation peritonitis is    e Hence, early Surgery is recommended.  e Open or laparoscopic method is followed. * A 36-year-old male who had previous history of   (viral), Yersinia ileitis and Meckel’s diverticulitis. tenderness and rebound tenderness in the right iliac  fossa. Operative surgery notes said that the appendix  was slightly inflamed and seropurulent fluid was present  in the right iliac fossa. A fter 2-3 days, greenish fluid  (bile) started draining out through the tube. The   PREGNANCY AND ACUTE APPENDICITIS condition of the patient deteriorated and on   ¢ Most common cause of abdominal pain and nonobstetric re-exploration this time, by midline incision revealed  emergency in pregnancy is acute appendicitis. perforated chronic duodenal ulcer!!   ° Symptoms of nausea and vomiting are confused for Complications of acute appendicitis  morning sickness.   e Treatment is by laparotomy and appendicectomy. 2. Appendicular mass (Figs 33.16 and 33.17)   * Foetal loss is 3% but with perforation, it 1s 30%. ¢ Following an attack of acute appendicitis, infection is    e j j iciti j °o . .  Maternal mortality rate in perforated appendicitis is 4%. sealed off by greater omentum, caecum, terminal ileum,   How could it have been avoided?  Serum amylase, lipase were not sent.    Surgical wisdom: If these  investigations were done before surgery,  they could have helped the surgeon.  Luckily, the patient recovered from this  unnecessary, avoidable surgery.    mass and was undergoing conservative management.  On the fourth day, she developed features of early septic  shock. As the patient was not improving, laparotomy  was done. It was a case of volvulus of the caecum.    etc. which results in a tender, soft to firm mass in the  right iliac fossa.   from sucha mass. An attempt to remove it may result in   — Drugs to cover all the organisms—gram-positive,  gram-negative and anaerobic organisms.   — Exploratory laparotomy should not be done. However,  when the condition of the patient is not improving,  there is a suspicion of an abscess (Fig. 33.17) and  when doubtful of the diagnosis, exploration 1s  indicated (see the clinical notes above).   Patient is kept nil orally for a few days. During this time,    He had not done a simple chest  X-ray or plain X-ray abdomen  erect preoperatively in this case    Surgical wisdom: Surgeon  had not done ultrasound and  gynaecological consultation  was not requested before  surgery.    By one week, the patient is back to normal. After 6-8  weeks, patient 1s advised elective appendicectomy.   Delay in seeking medical treatment is the main factor.  Other factors which precipitate perforation are diabetes  mellitus, AIDS, faecolith.   The pain usually localises to the right lower quadrant if the  perforation has been walled off by surrounding intra-  abdominal structures including the omentum.   Rigors and chills with fever of 102°F (38.9°C) or above.  Asa complication of perforation peritonitis, portal pyaemia   Mortality in these cases can be high. e Ultrasound/CT scan is done to assess the size and location  of abscess    . Appendicular abscess (Fig. 33.18): If the infection is not | ° ‘Abscess greater than 4-6 cm in size needs to be drained  controlled properly following an attack of appendicitis, an by guided percutaneous, aspiration or drainage through    ' rectum or vagina  abscess can occur in relation to the appendix. They are (A) g    oi appendicectomy open/laparoscopic at the same admission  (D) subcaecal abscesses. Clinically, it presents with high- e Those who improve require appendicectomy after 6 weeks  grade fever with chills and rigors and a tender boggy    swelling intherightiliac fossa or in theright lumbar region.  Pelvic abscess presents with diarrhoea. Diagnosis 1s by late  presentation to the hospital (3—4 days) and high-grade fever  with chills and rigors (Key Box 33.7).    A.Retrocaecal abscess is drained by extraperitoneal  approach. An incision of 5 to 6 cm is made in the right iliac Preoperative resuscitation  fossa and all muscles are divided. However, perito-neum  is not opened. It is swept medially and pus is drained  outside. Appendicectomy is done at alaterdate(Fig.33.19).    C. Pelvic abscess is drained via the rectum (see page 647)    D. Lumbar abscess (perinephric abscess) is drained through  a loin incision.    ¢ Emergency appendicectomy: Emergency appendicectomy  is offered when patient comes within 24 to 48 hours of  abdominal pain. It is very important to rule out or detect a  mass, especially if a decision is made to operate around  2nd or 3rd day. If a mass is palpable, it is better not to  operate at that time (please refer to operative surgery,  appendicectomy). A few important steps are given here  (Figs 33.20 to 33.24).   Fig. 33.18: Appendicular abscess (see text for A to D) converges onto the base of the appendix. Mesoappendix 1s   Fig. 33.22: Inflamed appendix at surgery (Courtesy: Dr Prasad, Fig. 33.23: Appendicular perforation with abscess—  S. Professor of Surgery, KMC, Manipal) appendicectomy could be done (Courtesy: Dr Annappa Kudva,  Professor of Surgery, KMC, Manipal)    Appendicectomy ; Child/Pregnant/Adults | Nonpregnant Give the choice to the  patient if he is willing   divided in between ligatures. A purse-string suture is applied  all around the appendix in the caecum. The appendix 1s  divided in between ligatures, the stump 1s invaginated and  the purse string is tightened. The abdomen 1s closed in layers.  (see Chapter on operative surgery)   ¢ Laparoscopic appendicectomy has become more  popular nowadays. Less postoperative pain, speedy  recovery. Benefit is maximum in obese, women and elderly    When you trace Taenta coli and you are not getting  appendix, it means you are holding and tracing Taenia  of the sigmoid colon. (It can be found on the right  side sometimes.)    1. The incision is small: Location is higher up—never hesitate  to close the incision and a midline incision is given and do  appendicectomy. An attempt to remove the appendix with  traction and limited exposure through McBurney incision  may result 1n faecal fistula.   2. Normal appendix is found: Remove the appendix. Other-  wise it may cause confusion to the next surgeon when this  patient presents with abdominal pain. However, look for  Meckel’s diverticulitis, intestinal obstruction, stricture, etc.   3. Gangrenous appendix involving base: Problem one  can face here is that the purse string can be applied but  invagination of the stump 1s not possible. Risk of faecal  fistula is also present. Appendicectomy, wash and a drain  1S kept.   4. Difficult to isolate the appendix which is gangrenous  but pus is present: Limited ileocecectomy can be  done.   5. The appendix cannot be found: First mobilise the caecum  and look for subcaecal or retrocaecal sites. Look also into  preileal or postileal sites. Then mobilise the ascending colon  also. Agenesis of the appendix is very rare.   6. Surprise findings of carcinoma caecum (Fig. 33.25): If  suspicion of a carcinoma is high, hemicolectomy should  be done. Otherwise take a biopsy—do appendicectomy.    It means removal of normal appendix at laparotomy for another  condition. Examples: Laparotomy and ileal resection for  stricture and anastomosis (can we do appendicectomy’”).  Ovarian cyst: Torsion (right) ovary is removed. Can we  add appendicectomy?  Since benefits of appendicitis/appendicectomy is more in  young patients, if patient is under 30 years, it may be justifiable    to do incidental appendicectomy provided it can be removed  through same incision, without much difficulty.   ¢ Chances of infection are high in this group of patients. The  result will be faecal fistula—difficult to treat.    What to do if normal appendix is found at surgery?  ¢ Normal white appendix is called Lily-white appendix.    ¢ Itcan occur after appendicectomy especially when gangrene  of the appendix extends to base of the caecum. It can also  occur if purse-string suture is not properly applied, injury  to the terminal ileum or caecum, etc. occurs.   Fig. 33.26: This patient had faecal fistula which healed after two  weeks of conservative management   ¢ Can present as acute appendicitis due to obstruction  caused by the tumour.    ¢ Cases which do not respond to conservative treatment are  managed by resection of the diseased portion of the caecum  or ascending colon.    The important causes for faecal fistula are carcinoma  caecum and ileocaecal tuberculosis in India and Crohn’s  disease in the West.   ¢ It is the most common neoplasm of the appendix, less  aggressive, majority are benign and cured with simple  appendicectomy (see Chapter 28 for more details).   ¢ Goblet cell carcinoid tumour—it 1s more aggressive,  requires right hemicolectomy if the tumour is more than  2 cm, has more than 2 mitosis per high power field and  lymphovascular invasion, adenocarcinoma of the  appendix.   Causes: It can be a simple retention cyst due to blockage by  foreign body or mucosal hyperplasia. It can also be due to a  mucinous adenocarcinoma (Fig. 33.27).    e The majority of epithelial tumours of the appendix are  mucin rich, thus results in gross distension.   ¢ Mucocoeles resulting from non-neoplastic occlusion  (simple retention cysts) rarely exceed 2 cm in diameter.   ¢ Mucinous neoplasms of the appendix are by far the most  common cause of mucocoeles.   ¢ Mucocoeles larger than 2 cm are more likely to represent  benign neoplasms.   e Itis impossible to differentiate clinically mucocoele of the  appendix and acute appendicitis when they present with  abdominal pain. If a mass is palpable, it can be confused  with appendicular mass.   Fig. 33.27: Mucocoele of the appendix (Courtesy: Dr Raghunath  Prabhu, KMC, Manipal)   Fig. 33.28: Large mucocoele removed along with caecum and  terminal ileum   Fig. 33.30: Appendicular tumour arising from the tip has been  removed—reported as carcinoid (Courtesy: Dr Raghunath  Prabhu, KMC, Manipal)    ¢ CT scan is the investigation of choice. The anatomic  relationship between the elongated cystic mass and the  caecum 1s usually more clear at CT scan than at ultrasound.   2. Rupture will result in pseudomyxoma peritonei (more  details are given in Chapter 27, Peritonitis).    Rule out adenocarcinoma of the base of the appendix  causing mucocoele.    ¢ Rudolf Valentino was an Italian actor acting in Hollywood  who was operated for right iliac fossa pain with features of  peritonitis in the early 20th century. Following a few days  of surgery, he died of sepsis. The actual disease was  perforated duodenal ulcer. This is a typical case scenario  that holds true even today. The contents gravitate down  along right paracolic gutter. The symptoms and signs mimic  appendicitis. CT scan is the investigation needed to rule  out other causes (see page 637).    A 32-year-old man presented to casualty with septic shock  after 5 days after appendicectomy. It was a difficult  appendicectomy. Gangrene of the appendix was almost  involving the base.   On examination he was having paralytic ileus and jaundice.  Abdomen was distended—-guarding was present, more in the  right iliac fossa. He was admitted to the hospital.    ° K: 3.2, Na: 129  ¢ TB: 19 mg/DI, DB: 16.6, ALP: 167  e AST: 189 units, ALT: 100 units    Plain X-ray chest revealed free gas under the diaphragm  (Fig. 33.32).    CT scan: Done after hydration—showed pneumoperitoneum  and liver cyst (incidental) and free fluid in the peritoneal cavity  (Fig. 333 34).    He was in sepsis. The reason was probably that the  appendicular base (stump) had given way.   ¢ One litre of frank purulent pus in the peritoneal cavity.   ¢ Gangrene of lateral wall of caecum with sloughing of caecal  wall.   ¢ Cystic lesion on anterior surface of right lobe of liver   ¢ Limited resection of ileocecal segment and end-to-end ileo-  ascending single layer anastamosis   e Anastomotic leak and enterocutaneous fistula was suspected  e Patient was passing flatus    Fig. 33.32: Free gas under the right dome of the diaphragm    Fig. 33.33: Free gas under the right dome of the diaphragm  pneumoperitoneum and liver cyst    This case report has been given here for the following  message    . Acute appendicitis can be dangerous  . Leak should be suspected if a patient who underwent    . High total count, increased bilirubin, oliguria suggest sepsis  . CT scan is the best investigation in such cases. When in    e All topics have been updated with flowcharts and  coloured pictures.    e Valentino appendix and neoplasms of the appendix have  been added.   Most common surgical emergency encountered by a general surgeon is acute appendicitis.    Most common nonobstetric surgical disease of the abdomen during pregnancy is acute appendicitis.    Most significant sign of acute appendicitis is rebound tenderness in the McBurney’s point.   Most prominent scoring system to diagnose acute appendicitis is Alvarado score.   Most common anaerobic bacteria in acute appendicitis is Bacteroides fragilis and aerobic bacteria is Escherichia coli.  Most common complication after appendicectomy is wound infection.    Most common age group for acute appendicitis is below 40 years.  Most common neoplasm of the appendix is carcinoid tumour.    Most of the carcinoids are less than 1 cm in size.   1. The most common position of the appendix is: 9. The most common cause of nonobstetric emergency  A. Subhepatic B. Subcaecal with abdominal pain in pregnancy is due to:  @ Reyéeateal i “Pelade A. Acute appendicitis B. Acute cholecystitis  c- Se C. Acute gastritis D. Acute hepatitis  2. The incidence of appendicitis is less after 30 years i Pe sce ;  b ; 10. Contraindications for incidental appendicectomy  ecause: ; ;  : x 4 aachut include all of the following except:  A. The appen i un lees Invo cue B® Crohaseobeaceittn  B. The lymphatic tissue in the ap penenx eens B Padiauen Weanwent. atthe cectin  C. Most people would have had their appendices removed C. Immunocompetent individuals  D. The vascularity reduces D. Previous vascular reconstruction in the abdomen  3. The name Sheshachalam is associated with which of 11. The following statement is TRUE about appendicular  the following arteries? abscess:  A. Accessory appendicular artery A. Abscess greater than 4-6 cm in size needs to be drained  B. Appendicular artery by laparotomy  C. Ileocolic artery B. Appendicectomy must be done along with laparotomy  ; for appendicular abscess  D. Posterior caecal artery a  as C. Can present with diarrhoea  4. Appendicular orifice is occasionally guarded by an D. Conservative management is advised till inflammation  indistinct semilunar fold of mucous membrane called:  . settles down.  A. Valve of Gerlach —_B. Valve of Heister 12. Most common aerobic bacteria involved in acute  C. Valve of Kerckring D. Valve of Houston appendicitis is:  5. The most common scoring system used for appendicitis A. Samonella typhi B. Streptococcus  is scoring system. C. Escherichia coli B. Clostridium perfringens  A. Child-Pugh B. Furtado 13. The following statement is FALSE about occurrence of  C. Murray Annes faecal fistula following appendicectomy:  A.F | fistul if th f dicitis 1  6. Palpation of left iliac region of abdomen produces pain i aon alae cinta etna  in the right ili ion i dicitis b f: ; —  Bee ies — ee B. Faecal fistula can occur if chronic diseases such as  A. Sympathetic reaction tuberculosis is present  B. Displacement of colonic gas and small bowel coils C. Faecal fistula can occur if purse string sutures are not  C. Sigmoid colon is also affected applied properly  D. Ileocolic reflex D. It 1s always due to actinomycosis  7. Cope's psoas test is positive in: 14. The most reliable symptom oF acute aREGAAICHIS is:  A. Retrocaecal appendicitis = ho . 3. mnpraiory as  est C. Right iliac fossa painD. Vomiting  B. Pelvic appendicitis eae  C. Prejleal dicit 15. Appendicular perforation is common because of the  pple Sache ee following reasons except:  D. Subcaecal appendicitis A. Appendix is a cul-de-sac  8. Rebound tenderness in acute appendicitis is called: B. It has blood supply with profuse collaterals   ANSWERS  C 2B 3 A 4 A a 6 B 7A 8 B 9 A 10 C  11 C 12 C 13 D 14 B 15 B    e Anatomy ¢ Sportsmen hernia e Interparietal hernia  ¢ Indirect hernia * Sliding hernia ¢ Spigelian hernia  e Direct hernia e Femoral hernia    ¢ Hernia is acommon condition affecting patients, especially  inguinal hernia in males and incisional hernia in females.  Even though several types of surgery have been described  for hernias, mesh hernioplasty remains the gold standard  treatment. Majority of the hernias require surgical treatment  leaving apart small asymptomatic direct hernias in elderly.  Today, laparoscopic hernia is becoming gold standard.  Obstructed hernia is an emergency and late cases carry  significant mortality. As far as students are concerned, hernia  is the most common case in the examination. Hence, detailed  clinical examination of hernia, complications, and various  types of hernias and their treatment have been described in  this chapter.   Abnormal protrusion of a viscus or a part of it through a weak  point in the body (opening) is known as ahernia. Inguinal hernia  occurs either through the deep inguinal ring (indirect hernia) or  through the posterior wall of the inguinal canal (direct hernia).    ¢ It is the ligamentous portion of the external oblique  aponeurosis which folds inwards and extends from anterior  Superior iliac spine to the pubic tubercle.    Rare types of femoral hernia  Complications e Umbilical hernia  Incisional hernia  Epigastric hernia   ¢ Some fibres of inguinal ligament pass posteriorly to attach  to superior pubic ramus lateral to the tubercle and forms  lacunar ligament.   ¢ The midpoint between the anterior superior iliac spine and  pubic symphysis is called midinguinal point.    ¢ Itis 4 cm in length extending from the deep inguinal ring  to the superficial inguinal ring.    Itis a ‘U’ shaped defect in the fascia transversalis which forms  the posterior wall of the inguinal canal. It lies 1.25 cm above  the midpoint of the inguinal ligament.    Superficial ring is a triangular defect in external oblique  aponeurosis. It is bounded by the lateral and medial crura  formed by the external oblique aponeurosis and the base of  the triangle is formed by the pubic crest.    Anterior: External oblique aponeurosis and a few fibres  of the conjoined muscle (especially of internal oblique)  laterally.    Inferior: Inguinal ligament and the lacunar ligament on  the medial side (Gimbernat’s ligament).    Posterior: Fascia transversalis and the conjoined tendon  medially. Thus, the inguinal canal is strong in the lateral  part anteriorly and the medial part posteriorly.    This weak area is the site of all groin hernias according to  Fruchaud.   It is the area between inguinal ligament anteriorly and  iliopubic tract posteriorly.    Hiopubic tract: It is the thickened inferior margin of the  transversalis fascia which appears as a fibrous band running  parallel and posterior (deep) to inguinal ligament. It inserts  into superior pubic ramus to form lacunar ligament.   CONTENTS OF THE SPERMATIC CORD  Vas deferens  Testicular artery  Artery to the vas  Cremasteric artery  Pampiniform plexus of veins  Lymphatics  Sympathetic nerves  Genital branch of genitofemoral nerve  Processus vaginalis   ILIOINGUINAL NERVE  The ilioinguinal nerve is a branch of the first lumbar nerve  (L1). It separates from the first lumbar nerve along with the  larger iliohypogastric nerve.  llioinguinal nerve does not pass through the deep inguinal  ring. It only travels through part of the inguinal canal.  After going through inguinal canal, it pierces the internal  oblique muscle, distributes nerve fibres to it, and then  accompanies the spermatic cord through the superficial  inguinal ring.  Divides into anterior scrotal nerve and anterior labial nerve.    Supplies the skin of the upper and medial part of the thigh,  scrotum and vulva.   Entrapment or injury to the ilioinguinal nerve is one of the  causes of postherniorrhaphy pain.   Hence, a few recommend division of ilioinguinal nerve during  hernia surgery.    Boundaries of myopectineal orifice of Fruchaud:  Superiorly: Arched fibres of internal oblique  Laterally: \ltopsoas muscle    Surgical importance of iliopubic tract: Recognition of  this is a part of laparoscopic repair (initial step)—-visualising  from within. This structure reinforces posterior wall and  floor of inguinal canal as it bridges structures traversing  subinguinal space.    It is bounded medially by lateral border of rectus abdominus  muscle, laterally by inferior epigastric artery and inferiorly  by inguinal ligament.   Direct hernias occur commonly through Hesselbach’s  triangle (medial), indirect hernia lateral to inferior epigastric  artery.    2. During straining or coughing, the conjoined tendon  contracts, and since it forms the anterior, superior and  posterior boundaries, it closes the inguinal canal—shutter  or sphincter-like effect.    3. Increased intra-abdominal pressure produces plugging effect  at the external ring. The deep ring 1s pulled upwards and  laterally because it is adherent to the posterior surface of  transversalis muscle. This occludes the ring and prevents  herniation—Ball valve effect.   I. The European hernia society classification:  ¢ Primary (P), Recurrent (R)  ¢ Lateral (L), Medial (M), Femoral (F)  ¢ Defect size assumed to be 1.5 cm  Thus primary direct hernia with 3 cm defect size is written  as PM2.  Il. Anatomical classification  1. Indirect hernia  2. Direct hernia  III. Nyhus classification    This classification is based primarily on the defect, which  helps in planning an appropriate repair.    ¢ Itis based on the defect in the posterior wall (direct hernia)  or defect in the internal ring (indirect).   ¢ Depending upon the defect, the suggested repair is given  below. However, basic principles are the same.    ‘Tal le 34.1 Gilbert's classification and suggested repair | a  Types Classification Repair  I, Snug internal ring Herniorrhaphy or hernioplasty  Preperitoneal indirect sac  Does not admit one finger  II. Moderately enlarged internal ring Herniorrhaphy or hernioplasty  Bubonocoele  Admits one finger  Ill. Large defect—2 or 3 finger-breadths internal ring. May be Preperitoneal mesh by slitting transversalis fascia  sliding hernia  IV. Large direct hernia with full blow out defect Mesh repair  Internal ring is normal  V. Direct hernia with punched out hole/defect in the transversalis fascia Plug the defect or purse-string closure of the  The internal ring is intact defect followed by mesh repair  VI. Pantaloon hernia Mesh repair  VU. Femoral hernia Femoral hernia repair   ¢ Indirect hernia occurs largely due to persistent processus  vaginalis sac. Manifestations of this can be seen in elderly  patients in whom an indirect hernia can be triggered by  some factors which increase intra-abdominal pressure.   ¢ Direct hernia occurs mainly due to weakness of transversalis  fascia in Hesselbach’s area. Increase in abdominal pressure  can occur due to chronic cough, constipation or difficulty  in passing urine, development of ascites (portal  hypertension, nephrotic syndrome), etc.   ¢ Collagen disorder: In prune-belly syndrome, collagen fibre  disorder causes development of not only hernias but also  intestinal hernias, bilateral hernias, etc. (Key Box 34.3).   ¢ Hernia occur due to inherited imbalance in the types of  collagen.    It is a herniation of abdominal contents through the deep ring  into the inguinal canal. Indirect hernia occurs due to  persistent processus vaginalis sac. It is the most common  type of hernia in the body. The preformed sac passes through  the deep ring, traverses the inguinal canal and may extend  into the scrotum through the external ring. As it comes into  the inguinal canal, it is invested by the following coverings:  1. External spermatic fascia derived from external oblique  aponeurosis.  2. Cremasteric fascia derived from internal oblique.  3. Internal spermatic fascia from fascia transversalis.    e The testis originates in the lumbar region—in the  retroperitoneum. Testis is guided or pulled down by a ligament  called as gubernaculum. As the testis is pulled down it also  pulls down the peritoneum along with it. This is processus  vaginalis sac (tube). This process is obliterated in the normal  situation. However, failure to close or obliterate is responsible  for development of hernia.   e New theory or recent advances: Hormonal cause has been  responsible for development of a hernia. Hepatocyte growth  factor and calcium gene related peptide influence closure of  the tube.    Hernial sac is part of the peritoneum which ts dragged into the  inguinal canal. The mouth of the sac 1s in the peritoneal cavity.  The neck is the narrowest portion (deep ring). The actual  hernial sac has a body and a fundus. Depending upon the  contents it can be named as follows: Omentum—omentocoele,  Intestine— enterocoele, Littre’s hernia—hernia containing  Meckel’s diverticulum. It may also contain ovary or appendix.  When part of the wall of the gut is involved, it is known as  Richter’s hernia.    1. Complete hernia (scrotal): When the sac is patent up to  the bottom of the scrotum, it is a complete scrotal hernia  (Fig. 34.5).   2. Funicular: The processus vaginalis sac is patent up to the  root ofscrotum, it is aincomplete indirect hernia (Figs 34.6  and 34.8).   3. Bubonocoele: Processus vaginalis sac is confined to the  inguinal region or the inguinal canal only. Such hernias are  seen in young patients (Fig. 34.7).   2. Two layers of superficial fascia: Fatty and membranous  (Camper’s and Scarpa’s fascia respectively).   3. External spermatic fascia, a continuation of the external  oblique aponeurosis.   4. Cremaster muscle and fascia, a continuation of the internal  oblique.    1. Weakness of fibres of transversus abdominis or congenital  absence of a few fibres is a major factor responsible for  direct hernia.    2. In elderly patients, it is precipitated by:  ¢ Chronic cough, chronic bronchitis  ¢ Pops on coughing (Fig. 34.9)  ¢ Difficulty in passing urine due to benign prostatic  hypertrophy (BPH).  ¢ Chronic constipation due to habitual constipation, or  malignancy of left colon.    5. Intemal spermatic fascia: Derived from the fascia transversalis. PEARLS OF WISDOM    6. Extraperitoneal fat As the direct hernia pushes through the posterior wall,  7. Peritoneum it is very unusual for it to descend into the scrotum.    It is always acquired (Fig. 34.9). It occurs through This 1s a type of direct hernia wherein the hernial sac appears  Hesselbach’s triangle, a weakness in the posterior wallofthe through a circular defect (congenital) in the conjoined tendon.  inguinal canal (transversalis fascia). Its boundaries are:  ¢ Medially: Lateral border of the rectus abdominis   History  Coverings of the direct inguinal hernia from outside + Swelling in the inguinal region which is gradually increasing  to inside in size.  1. Skin ¢ To start with, the swelling disappears on lying down and  2. Two layers of superficial fascia increases on straining, walking, etc. Later it cannot be   e Since the omentum ts attached to the stomach above and  supplied by T10, the pain is referred to the umbilical region.   ¢ H/o chronic cough, constipation, difficulty in passing urine  should be asked. If present, it may suggest the cause of  hernia.    Division of ilioinguinal nerve during appendicectomy may  cause denervation of fibres of the right transversus abdominis,  which forms ‘U’ shaped ring, resulting in weakness of the  abdominal wall.    Inspection (a model case of incomplete hernia) (Fig. 34.10  and 34.11)    It should be done in the standing position. Both sides should   e There is a swelling in the inguinal region extending to the  root of the scrotum measuring about 6 x 3 cm. Its surface  is smooth, borders are round and skin over the swelling  is normal and it is pyriform in shape.   ¢ Direct hernia pops out as soon as the patient stands and  often it is bilateral.   ¢ Swelling ts soft, and gurgles if it is an enterocoele.   e It may be firm or granular if it is an omentocoele.   2. Getting above the swelling! should be done in the standing  position.   EXPANSILE IMPULSE ON COUGH  e Hernia  e Meningocoele  ¢ Dermoid cyst with intracranial communication  e Laryngocoele  e Lymphatic cyst in children  e Empyema necessitatis    e At the root of scrotum, the spermatic cord 1s palpated  between the finger and the thumb. In cases of complete  indirect hernia, spermatic cord cannot be felt as a naked  structure because it 1s covered anterolaterally by the sac.  This 1s called as getting above the swelling not possible  (negative).    Getting above the swelling is a test to differentiate scrotal  swellings from inguinoscrotal swellings.   ¢ Omentocoele: Initially, reduction 1s easy but later, becomes  difficult (due to adhesions).   ¢ If itis difficult to reduce, ask the patient to reduce it.  Otherwise, flex and medially rotate the hip and try to reduce  it, a method called as taxis.   ¢ If in spite of this, the swelling  is not reduced, it 1s called as an  irreducible hernia.   4. External ring invagination test  (Fig. 34.12): At the root of the  scrotum, skin is gathered and  lifted up with the little finger. It  is then invaginated into the  external ring. As the external  ring is stretched in indirect  hernia, the finger goes obliquely  and laterally. In a direct hernia,  the finger goes backwards, and  the superior ramus of the pubic  bone can be felt as a bare bone.  On asking the patient to cough,    'This test has no meaning or usefulness in bubonocoele. It is a test to differentiate scrotal swelling from inguinoscrotal swelling and assumes    the impulse touches the pulp of the finger in direct hernia  and the tip in indirect hernia.    This test causes discomfort to the patient. It cannot be  done in female patients because the labial skin is thick  and not lax. Hence, it is not a relevant test. However, in  very early doubtful cases of indirect hernia an impulse  on cough may be appreciated at the deep ring in this  fest.    5. Internal ring occlusion test (deep ring occlusion test):  Reduce the swelling first (Fig. 34.13). | :  Locate the deep ring above the midpoint between anterior Fig. 34.14: Leg raising test  superior iliac spine and symphysis pubis. Occlude the deep  ring with the thumb and ask the patient to cough.   a. If impulse and the swelling are seen, it is a direct hernia  because it occurs in the Hesselbach’s triangle (medial to  deep ring).   b. If the swelling is not seen, it is an indirect hernia. Deep 7. Zieman’s method: Three finger method  ring occlusion test can be done with the patient in standing  and supine position.    Keep index finger at deep ring, middle finger on the  posterior wall above and lateral to the external ring and  Problems of deep ring occlusion test ring finger at femoral ring. Now ask the patient to cough.  Depending upon the type of hernia, impulse is felt. It is  not necessary to perform this test in incomplete or  complete indirect hernias.    a. If occlusion is not done properly, results may vary.  b. Pantaloon hernia (Romberg hernia, saddlebag hernia, dual  hernia). It is a direct hernia having indirect component.  8. Per abdomen: To rule out any mass (colonic).    9. Look for phimosis/stricture urethra: Young patients  having urinary complaints with hernia may be suffering  from stricture urethra. Lift the scrotum and feel for any  strictures in the bulbar urethra. Retract skin of prepuce  and rule out phimosis.    10. Per-rectal examination should be done in elderly patients  to rule out prostatic enlargement.    11. Examination of respiratory system is done to rule out  chronic bronchitis, tuberculosis, etc.  PEARLS OF WISDOM   * Weakness of oblique muscles is manifested by (hold the child on its back), hernia may become apparent.    Right side, indirect, incomplete, uncomplicated, reducible  omentocoele. Table 34.2 for comparison and Table 34.3 for  differences between hernia and hydrocoele.    Groin refers to the junction of lower abdomen with the thigh.  Hence, swellings in the inguinal region and upper thigh close  to the inguinal ligament are included under groin swellings.  1. Inguinal hernia (Fig. 34.15)   3. Vaginal hydrocoele: Fluctuation and transillumination tests  are uSually positive and getting above swelling is possible.  (Please note that in infantile hydrocoele and hydrocoele en  bisac, getting above swelling 1s not possible) (Fig. 34.17).   4. Retractile testis: It can present as a firm swelling in the  inguinal region. Scrotum 1s empty (Fig. 34.18).   5. Saphena varix: Patient can present with a swelling in the  thigh. Swelling is usually about 2.5 cm below the pubic  tubercle. A swelling that disappears on elevation of the  leg is characteristic of a swelling of venous origin  (Fig. 34.19).   6. Funiculitis: A funiculitis can occur with or without acute  epididymoorchitis. Severe pain 1n the inguinal region, tender    Weakness of posterior wall of inguinal canal  Chronic bronchitis, enlarged prostate    swelling, high grade fever with chills and rigors are  characteristic. Spermatic cord 1s thickened and swelling is  not reducible (Fig. 34.20).   7. Inguinal lymphadenitis: Pain and nodular swelling below  inguinal ligament 1s a feature. It is not reducible and some  source of infection in the lower limb 1s usually present  (Fig. 34.21).   8. Lipoma of the cord: It presents as a soft, lobulated but  irreducible swelling in the inguinal region (Figs 34.22 and  34.23).    1. Routine investigations such as complete blood picture  (CBP) and urine examination are done. In elderly patients,  chest X-ray, electrocardiography or even pulmonary  function tests may be necessary. Patients with urinary  complaints are evaluated for prostatic enlargement and  stricture urethra.   2. Ultrasound: Hernia 1s a clinical diagnosis. In the vast  majority of the cases, no investigations are required specific  to the diagnosis of hernia. However, in appropriate cases  imaging can be done.   ¢ In so-called occult cases wherein patient has groin pain but  clinically not evident. Ultrasound can detect a sac—however  it is operator dependant, ultrasound ts also useful in cases  of postoperative swelling in the groin to rule out haematoma/  seroma/recurrence.    Fig. 34.15: Inguinal hernia Fig. 34.16: Femoral hernia Fig. 34.17: Infantile hydrocoele Fig. 34.18: Retractile testis    PEARLS OF WISDOM ¢ Elderly patients with bilateral hernia mostly suffer from  benign prostatic hypertrophy. Prostatectomy should be  considered first followed by repair of hernia, in such cases.  Recent history of constipation and appearance of a hernia  should arouse the suspicion of carcinoma colon. Investigate  by colonoscopy/fibreoptic sigmoidoscopy before the  treatment of hernia.    Hernia ts a clinical diagnosis. If you are asked to give  one investigation to confirm hernia (may be in early  cases) it is ultrasound.    3. Computed tomography(CT) scan 1s ideal in cases of giant  hernias, or special types such as obturator hernia, perineal    hernia, ete. . stricture urethra. They should undergo proper treatment for  4. Magnetic SS ee (MRI): Ideal In sportsmen the stricture. Now it is rare to find a patient with hernia  who complain of groin pain, to detect hernia or to rule out having a stricture urethra.    ¢ A patient with chronic bronchitis and bronchial asthma  should be properly treated with bronchodilators, antibiotics,  mucolytic agents, etc. Cigarette smoking should be stopped.    Herniotomy, herniorrhaphy and hernioplasty are the  three “key” operations for inguinal hernia.    Excision of the sac alone is done in patients up to 14-16  years of age (children). Hernia occurs due to preformed  sac. Hence, no repair is necessary.    ¢ It can be of two types: Bassini’s and Shouldice. Hence irrespective of the type of hernia, mesh repair  ¢ What is done today is the modified Bassini repair—-(Read (Lichtenstein—next page) ts recommended today as first  also original Bassini operation). line of repair.    Repair of stretched deep ring by narrowing and laterally Reinforced the posterior wall of the inguinal canal with a single   selected cases at the end of the procedure. sutured the internal oblique muscle, transversus abdominis  ¢ If there is tension, an incision over the anterior rectus muscle and upper leaf of the transversalis fascia (triple layer)    He then reapproximated the external oblique aponeurosis over  the cord structures.   e In modified Bassini repairs, the transversalis fascia is not  opened. Cremasteric muscle is not excised. Only conjoined  tendon is sutured to the inguinal ligament.    ¢ Itis the most popular tensionless method wherein only local  tissues are used.   ¢ Transversalis fascia which forms the posterior wall, 1s  incised from the internal ring till pubic tubercles.   ¢ Thus, upper and lower flaps of transversalis fascia are  sutured in a double-breasting manner by using non-  absorbable sutures such as 34 gauge stainless steel wire,  polyamide or polypropylene. This is the first layer of  Shouldice repair.   Fig. 34.24: Herniotomy tendon is sutured to the inguinal ligament by using  nonabsorbable sutures.   ¢ The third layer is completed by suturing upper flap of  external oblique aponeurosis to the inguinal ligament.    e Eduardo Bassini incised the external oblique aponeurosis  through the external ring.   Indications for Bassini’s herniorrhaphy  ¢ Indirect hernia with good muscle tone.  ¢ Direct hernia with good muscle tone.   ligament  e First layer: Double breasting of the transversalis fascia.  e Second layer: Conjoined tendon is sutured to inguinal  Spermatic ligament.  cord e¢ Third layer: Upper half of external oblique aponeurosis is    Indirect or direct hernia with a good muscle tone. In such  cases, darning can be done.    Hernioplasty refers to strengthening the posterior wall of  inguinal canal. There are two types of hernioplasties which  are commonly practised—A. Mesh repair, B. Darning.    (Figs 34.26 and 34.27, Key Boxes 34.6 and 34.7) of inguinal  canal by a prolene mesh or Marlex mesh. The fibroblasts  and capillaries grow over the mesh, converting it into a thick  fibrous sheath and strengthening the posterior wall. The  mesh is fixed inferiorly to lacunar and inguinal ligaments,  medially to overlap rectus sheath and fixed to fascia over  the pubic bone. A few interrupted sutures are put to fix it to  the transversalis fascia. Laterally, an artificial deep ring  is created by crossing of both upper and lower leaves of  the mesh. To attain this, a slit is given on one side of mesh.  (Lacunar ligament is that portion of the inguinal ligament  which extends backwards and upwards to the pectineal line  and forms the medial margin of the femoral ring).    Biocompatibility means it should not do any harm, should  be chemically and physically inert.    the inguinal ligament without tension in a criss-cross manner  by using prolene suture material (handmade mesh). This 1s  preferred in direct and indirect hernias (Fig. 34.28)  described by Maloney.   LICHTENSTEIN REPAIR  Polyprophylene mesh is used (Fig. 34.27)  8 x 16 cm mesh is tailored to patient’s requirement.  Preparation of mesh: Corners can be cut so as to give a  round shape. A slit is given on the lateral border of the mesh  at the junction of lower one-third and upper two-thirds, to  allow spermatic cord to pass through. The two tails (slit-  ends) are overlapped.  Suturing: Medially, the mesh overlaps the pubic tubercle  and is sutured over the tissue of symphysis (avoid pubic  bone to prevent osteitis pubis). Laterally, the two tails are  placed beyond deep ring and sutured. Inferiorly, itis sutured  to inguinal and lacunar ligaments and superiorly to conjoined  tendon.   Fig. 34.27: Lichtenstein repair is the most popular type of open    Less shrinkage of mesh, more flexible, better tissue  integration, better comfort.    These are sterilised sheets of connective tissue derived from  human or animal dermis or porcine intestinal submucosa:    Like the mesh, they provide scaffold for connective tissue  to grow and collagen deposition.    Enzymatic reaction takes place in the host, later fibrous  tissue formation occur.    Advantages: Chronic inflammation and foreign body  reaction, stiffness and fibrosis, and mesh infection are  uncommon—usually do not occur.    They can be used in presence of infection.  They are very expensive    In this operation the spermatic cord 1s divided at the deep  ring and it is removed along with the testis, so that the deep  ring can be permanently closed, and hernia never recurs. It  is indicated in elderly patients with recurrent hernia and  poor abdominal muscle tone. Hamilton Bailey’s operation—  cord 1s divided but testis 1s retained.    Ideally indicated in bilateral direct hernia or recurrent herniz  wherein a broad mesh is kept in the preperitoneal space.  Note: Students should remember that the operation whick  you have seen in your hospital should be mentioned in the  clinical examination.    The Stoppa repair is a tension-free type of hernia repair.  It is performed by wrapping the lower part of the parietal  peritoneum with prosthetic mesh and placing it at a  preperitoneal level over Fruchauds myopectineal orifice.  This operation is also known as giant prosthetic  reinforcement of the visceral sac (GPRVS).    Simple high ligation of the sac combined with tightening  of the internal ring (done in children).    1. What is Dasarda technique? In this operation, a strip of  external oblique aponeurosis is prepared isolated but still  connected medially and laterally to the external oblique  muscle, and sutured to conjoined tendon and inguinal  ligament below.   2. What is hernia system? A two layered mesh is used—one  to place deep to transversalis fascia (with finger in the deep  ring, blind and blunt dissection is done to develop a deep  plane) and the other in front of the transversalis fascia.   3. What is mesh plug repairs? These are simple plugs of  mesh inserted into the deep ring. It 1s a simple procedure  but mesh migration and seroma within the mesh called as  Meshoma are common.    These are common complications after surgery. Often they  are mild and note so worrisome. However, some of the  complications can be serious which require immediate attention  and treatment.   ¢ Injury to the iliac vessels: The most serious but rare  complication is injuryto iliac vessels. It can happen in thin  patients when suturing of the inguinal ligament 1s done from    lateral to medial side. The sudden Jet of fresh red blood  indicates that the bite has been taken through the artery. It is  better to call the vascular surgeon, extend the incision, have  a proximal control, suture directly or do a resection and end  to end anastomosis. They have to be anticoagulated with low  molecular weight heparin followed by oral anticoagulants.  Injury to the urinary bladder: This can happen when  anatomy Is not clear as in few giant or scrotal hernias,  perineal hernias or distortion due to previous surgery.  Sudden finding of clear fluid with urinary smell means  bladder injury. Immediate repair with 2—0 vicryl followed  by urinary catheter placement for 3 weeks is the treatment.   Pain: Pain is common due to the incision in the skin and some  degree of retraction of structures such as inguinal ligament  downwards and conjoint tendon upwards. The pain can be  decreased by local anaesthetic infiltration, examples  bupivacaine 0.25% up to a maximum of 2 mg/kg body  weight.   Bleeding: Perfect haemostasis is the aim of all surgeries.  In spite of this, a few bleeders may open up, mostly venous  blood—may be pampiniform plexus veins or arterial blood  from inferior epigastric artery. The bleeding may stop with  compression bandage. Otherwise, exploration and ligation  of bleeders needs to be done in the operation theatre.  Urinary retention is common, more so in males: Pain,  spinal or epidural anaesthesia, sedatives, lack of privacy  are contributing factors. Provide analgesia, privacy and hot  fomentation to suprapubic region. If all of these safe,  catheterise bladder as a last step.   Abdominal distension: This is not common. It can happen  when large intestinal contents of the hernia sac are reduced  or handled as in scrotal hernias or sliding hernias. It is also  important to realise that omentum 1s attached to stomach  and colon above. One should see that bleeders from injured  arteries of the omentum should be ligated properly. Some  intraperitoneal blood may add to paralytic ileus.   Seroma is due to inflammatory response to mesh or suture  materials. It causes swelling and anxiety that it may be a  recurrence. When in doubt, get an ultrasound examination  first. Seroma needs to be aspirated. Seroma 1s more common  after laparoscopic hernia repairs.   Wound infection: Hernia isa clean surgery. Infection should  not occur. However, poor handling of the tissues, haematoma,  seroma and diabetes may precipitate wound infection. Open  the sutures, drain the pus and use appropriate antibiotics in  such cases. Persistent wound infection may prompt removal  of the mesh. A few cases of tuberculosis have been reported.  This is due to improper sterilisation of the mesh used.    . Late: Late complications are not all that common. One  complication which bothers a few patients is chronic pain  called inguinodynia.    e Inguinodynia: The chief factor is injury to the following nerves:   iliohypogastric, ilioinguinal and genital branch of genitofemoral  nerve. Injury can be in the form of entrapment of the nerves,  traction injury, cauterisation, transection, etc. These are more  common after mesh repair because of entrapment of the  nerves or perineural fibrosis and adhesions between mesh  and the nerves.  Clinical features include dull aching or dragging pain in the  groin, genitalia, Suprapubic region. Some may complain of  diminished sensation or even hyperaesthesia. Treatment  include reassurance, simple analgesics, nerve blocks with  anaesthetic agents and injection of steroids. Neurolysis by  inguinal exploration or neurectomy may be required in  appropriate cases.   e Testicular atropy: It is due to injury to the testicular artery  which is not noticed during surgery. A few days later, the patient  may complain of small testis. Examination reveals no  sensation in the testis. Orchidectomy may be required in such  cases.    Occurs due to adhesions formed between omentum, sac and  the contents. Irreducibility produces dull aching pain.    DIAGNOSIS OF IRREDUCIBLE HERNIA  e Hernia is tense  e Tender  e Irreducible  e No impulse on cough  e Recent increase in size of swelling    Irreducible hernia + obstruction to the lumen of the gut gives  rise to obstructed hernia. Clinically, it produces severe colicky  abdominal pain, abdominal distension, vomiting and step  ladder peristalsis.    Urgent division of the neck of the sac followed by  herniorrhaphy or hernioplasty.    ¢ Strangulation commonly occurs in femoral hernia, obturator  hernia and in indirect hernia.   e Initially the venous returm Is occluded, the part gets congested  and mucosal ulceration and haemorrhage occurs into the gut  wall. It also results in oedema due to capillary exudation.   e Gangrene appears at the ring of constriction first. Later it  develops in the antimesenteric border (Fig. 34.31).   ¢ Such a gangrenous segment contains decomposed blood in  which gram-negative organisms multiply. They produce  endotoxins resulting in endotoxic shock. If the gangrene  extends into the intra-abdominal segment of the bowel,  peritonitis can occur.   ¢ The deep ring and the external ring are the common  constricting agents.    ¢ Sudden, severe, prolonged pain with some features of shock  are indicative of strangulation.  Clinical examination of such hernia reveals (Fig. 34.32):  . Tense (hernial sac is tense—differentiates it from obstructed  hernia)  . Tender  . No impulse on cough  . Irreducible  . Recent increase in the size of the swelling  . General condition of the patient is poor:  ¢ Feeble pulse  ¢ Hypotension  ¢ Rebound tenderness  ¢ Guarding and rigidity, if infection has spread intra-  peritoneally.  e Absent bowel sounds  ¢ Toxic look    ¢ The patient is hospitalised. The foot end of the bed is raised  so that an irreducible hernia may reduce by gravity. How-  ever, if there 1s a suspicion of gangrene, this step 1s not  recommended.    ¢ Intravenous fluids are given to correct dehydration and t  prevent renal failure.    ¢ An attempt should be made to reduce the swelling whe:  there is no gangrene by the following measures:    Fig. 34.31: Gangrene appears at ring of constriction first and  then progresses    C. With the right hand, the sac is gently squeezed by  applying pressure over the scrotum. At the same time  with the left hand, the proximal portion of the sac is  guided into the inguinal canal. This procedure is  described as taxis. Taxis is contraindicated if there is  gangrene.    D. Complications of forced reduction include contusion  of intestinal wall, rupture of the sac at the neck and  reduction en masse, 1.e. the entire sac with the contents  are reduced into the abdominal cavity but the intestine  still remains strangulated.    The patient 1s prepared for surgery and blood is grouped  and cross-matched.    With broad spectrum antibiotic coverage, the hernia 1s  explored by an inguinoscrotal incision and hernial sac 1s  defined. At this stage, the constricting ring should not be  divided. First all the toxic fluid from the sac is aspirated.  The constriction is then divided using a grooved director or  hernia bistoury. While dividing the constricting ring, the  inferior epigastric vessels which are situated medially  may be damaged. Therefore, care has to be taken to protect  these vessels.   If the bowel is gangrenous, resection of gangrenous segment  and anastomosis is done. Closure of incision includes  placement of tube drain, which is brought out through a  Separate incision.   If viability of the bowel is doubtful, the intestinal loops are  covered with hot wet mops for a period of 5—10 minutes  and 100% oxygen is given to the patient (request the  anaesthetist). Return to pink colour, peristalsis of bowel  and pulsations in the mesentery indicate viability.   If the general condition of the patient permits, repair of the  hernia may also be done.   If evidence of peritonitis is present or if gangrene 1s  spreading within, laparotomy should be done.    It is an obstructed hernia due to obstruction caused by faecal  matter. It generally occurs in a sliding hernia    SURGERY FOR STRANGULATION  Generous inguinal incision, identify the sac  First aspirate toxic fluid  Divide constricting agent  Check for viability  Resection of gangrene  Repair of hernia  Broad spectrum antibiotics    It occurs when the contents of hernia get inflamed, e.g.  appendicitis in a hernial sac, Meckel’s diverticulitis in  hernial sac.   Thus, complications of hernia can be dangerous (Key Box  34.11). It may range from a simple obstruction to a life-  threatening strangulation. Hence, early diagnosis and early  treatment are necessary in all cases of indirect hernia. In a few  selected cases of direct hernia, wherein the defect is big, the  chances of strangulation are less. However, if there are no  medical contraindications, surgery has to be advised.   SUMMARY OF COMPLICATIONS OF HERNIA  e |rreducibility  e Obstructed hernia  e Strangulation  e |ncarcerated hernia  e Inflamed hernia    Following are a few examples of strangulations without  obstruction. They have diarrhoea and bleeding per rectum  rather than constipation (Key Box 34.12).    1. Improper excision of the sac: The sac should be ligated at  the level of deep ring (neck). This is called high ligation of  the sac. Very often, the sac is seen as soon as the inguinal  canal is opened. If it is ligated at the fundus or body (low  ligation), it invariably results in recurrence. A missed  indirect sac can be a cause of recurrence (Figs 34.33 and  34.34A and B).    2. Absorbable sutures such as catgut have lifespan of 2-3  weeks. If they are used for reconstruction, they invariably  result in recurrence.   3. Bleeding: At the end of the surgery, small bleeding points  should be coagulated by using diathermy or ligatures.  Haematoma formation predisposes to infection, which can  be the cause of recurrence.   4. Tension between suture lines can cause strangulation and  fibrosis of muscle fibres. Hence, care and gentleness is  important while suturing conjoined tendon to the inguinal  ligament.   2. Haematoma can get secondarily infected resulting in pus  formation. The sutures give way leading to recurrence.  Hence, if there is a significant haematoma, it should be  drained.   3. Infection: Even though hernia 1s a clean surgery, chances  of infection are present specially in diabetics, alcoholics  and immunocompromised patients. Prophylactic antibiotics  such as 2nd generation cephalosporin should be given. If  infection occurs, it should be treated accordingly.   4. Exertion: Too much exertion in the postoperative period,  in the form of lifting heavy weights or carrying heavy  weights on the shoulder, may weaken the suture line,  resulting in hernia.   Most of the recurrences occur within one year. Incidence  of recurrent hernia may vary from 2 to 8% even in experienced  hands. Ina case of recurrent hernia, it is difficult to say whether  it is a direct hernia or indirect hernia. From the management  point of view, it does not matter.    e If the sac is present due to incomplete excision at the  previous surgery, it should be completely excised up to the  level of deep ring, followed by hernioplasty.   ¢ Meshplasty is the surgical treatment for a recurrent hernia.  However, if mesh cannot be placed either due to infection  or due to nonavailability, prolene darning can be done.    ¢ Tuberculosis must be ruled out in cases of persisting  infection.   ¢ In all these cases, precipitating factors if any, should be  treated first.   ¢ The only way to totally prevent recurrence is by closure of  the deep ring. This can be done only after dividing the  spermatic cord (Kuntz procedure). It 1s indicated in elderly  patients who have multiple recurrences.    Definition: A giant inguinoscrotal hernia 1s defined as a hernia  that extends below the midpoint of the inner thigh in the  standing position.    ¢ Most patients would have had their hernia for several years.   ¢ Hence, more prone for complications such as incarceration,  intestinal obstruction and scrotal ulceration. The last  complication is due to pressure necrosis or due to friction  while walking or moving.   — Perfect haemostasis.  Omentectomy and rarely colectomy may be required (Fig.  34.36).  If bowel 1s not resected, mesh can be placed safely.  In cases of emergency and patient with co-morbid factors  including cardiac problems, take consent for orchidec-  tomy—the best option will be to divide the cord at the  level of deep ring and close the deep ring during orchidec-  tomy.    2. Dual hernia: It has two sacs, one direct and another indirect,  connected by an isthmus which ts behind the inferior epigastric  artery. [tis also known as saddlebag hernia, pantaloon hernia,  dual hernia or Romberg hernia.    Significance  ¢ Deep ring occlusion test: The inference of the test may  not be correct.  ¢ It 1s the cause of recurrence if one sac is not treated  properly.  3. Prevesical hernia: It is also called funicular direct hernia.  Itis a hernia containing portion of the bladder with prevesical  fat through the defect in the conjoined tendon on the medial  side. History of the swelling becoming less prominent after  micturition may be present. Due to narrow neck, it 1s prone  for strangulation.    4. Littre’s hernia (Fig. 34.37): It is referred to a hernia  containing Meckel’s diverticulum. When the diverticulum gets  infected, such hernias are called inflamed hernias. The cause    of infection may be precipitated by partial obstruction to th:  diverticulum by constricting agents.   e [tis a W hernia wherein the intra-abdominal bowel loo]  segment becomes gangrenous very early but in the scrotum  there are no signs of gangrene.   e The patient presents with obstructed hernia and at operation  the inguinoscrotal segment has no gangrene. The intra  abdominal segment must be examined and the gangrenou:  portion should be excised. It can also be called retrograde  strangulation. Clinically, there 1s tenderness above the  inguinal ligament.   ¢ When only part of circumference of bowel becomes  strangulated, it is called Richter’s hernia.   ¢ Even though the patient has features of intestinal  obstruction, there will be diarrhoea and often blood 1n stools.    ¢ Femoral hernia, obturator hernia are a few examples of  hernia which can sometimes present as Richter’s hernia.   ¢ It occurs as a result of the slipping of posterior peritoneum  along with the retroperitoneal viscus. As a result of which,  the caecum, on the right side and the sigmoid colon on the  left side, form the posterior wall of the sac.   ¢ If the caecum and appendix are the contents of the  hernial sac, it is not a sliding hernia.    ¢ However, a true hernial sac containing omentum or  intestines exists.   ¢ Weakness of the abdominal wall at the deep ring lateral to  inferior epigastric vessels is a contributing factor also.   ¢ Urinary bladder can also be the content of the hernial sac  (Fig. 34.40A).   It can be suspected when there Is a large hernia descending  down into the scrotum.    inguinal hernia. State Medical University, Belarus, USSR)  ¢ They are not completely reducible.  ¢ It can be direct hernia and indirect hernia also. Complications    These hernias can easily strangulate and since its wall  contents include the large intestine, the mortality and  morbidity increases.   ¢ Once the hernial sac is opened, the sac should not be  twisted.   ¢ A purse string suture 1s applied within to avoid injury to  caecum/sigmoid colon.   ¢ The sac is removed and the repair of the hernia done.   ¢ In elderly patients, orchidectomy is advised to give a  permanent cure for hernia.    Always irreducible  Always colon or urinary bladder forms the wall of the sac    Alwaystechnical difficulties are encountered by junior surgeons  while operating on these patients   ¢ This is common in men who play rugby or football wherein  injury due to the ball may occur.   ¢ On examination, there may be tenderness tn the inguinal  region.   ¢ Orthopaedic disorders must be ruled out first by MRI or  CT scan. They are soft tissue injury in the groin, pubic bone  diastasis, adductor spasm, etc.    Herniation of intra-abdominal contents through the femoral  canal is described as femoral hernia (Key Box 34.13). Women  are more often involved, as compared to men with the ratio  being 2:1, which is doubled in parous women. However, it  should be remembered that in women, inguinal hernias are  the most common type of hernia, followed by incisional  hernia. Femoral hernia is the third most common type of  hernia.    Commonly the hernia is unilateral, the right side being  affected more often than the left side. It is bilateral in about  15-20% of the patients.   ¢ The femoral canal extends from the femoral ring to the  saphenous ring. It is 1’2 inches below and lateral to the  pubic tubercle. It is the innermost compartment of femoral  sheath.   ¢ It is similar to a truncated cone which is narrow at the  femoral ring.   ¢ Femoral vein is in the middle compartment of the femoral  sheath and femoral artery is in the lateral compartment.   ¢ Femoral sheath: Fascia transversalis is continued  downwards behind the inguinal ligament as the anterior  layer of the femoral sheath. Fascia iliaca continues behind  the femoral vessels as the posterior layer of the femoral  sheath.    ¢ Anterior: |nguinal ligament  ¢ Posterior: Ligament of Cooper, iliopectineal ligament.  ¢ Medial: Lacunar ligament (Gimbernat’s ligament)    1. Pregnancy: As the gravid uterus compresses the external  iliac vein, the empty femoral sheath on the medial side  allows the femoral vein to expand within femoral sheath.  Thus, increased abdominal pressure due to repeated  pregnancies is one of the chief factors responsible for  femoral hernias. The maximum incidence 1s around 30—40  years of age.    2. Wide femoral canal: This is due to the narrow insertion of  iliopubic tract into the pectineal line of the pubis and may  be responsible for a few cases of femoral hernia.    ¢ As the hernia comes into the femoral canal, it is an  oblong swelling due to the rigid femoral canal. When it  comes out through the saphenous opening, it expands  and becomes retort shaped because Scarpa’s fascia is  attached to the deep fascia of thigh below the saphenous  opening.    ¢ Females between the age of 20 and 40 years are commonly  affected.   ¢ Gaur sign: Dilatation of superficial epigastric/circumflex  iliac veins due to compression.   ¢ Right side is more commonly affected because of the  dominant nature of right side of the body.   ¢ To start with, there is a small swelling below the inguinal  ligament, which goes unnoticed very often.    Expansile impulse is often not present due to the narrow  canal.   Typically, the swelling 1s below and lateral to the pubic  tubercle (inguinal hernia is above and medial to pubic  tubercle) (Fig. 34.42).    Incision is placed directly over the swelling in the thigh.  The sac is carefully dissected out without damaging the  femoral vein. The sac 1s ligated at the neck, excised and the  hernia 1s repaired—the inguinal ligament is sutured to  Cooper’s ligament (iliopectineal ligament) thus obliterating  the femoral ring. Nonabsorbable suture such as prolene or  ethilon 1s ideal. Low approach ts indicated 1n uncomplicated  hernia. It is very difficult to manage a gangrenous loop of  bowel with this approach.   Through an inguinal incision, the inguinal canal 1s opened.  The transversalis fascia 1s incised. Hernial sac is visualised.  This is followed by excision of the sac. The high approach  1s preferred when there is a strangulated femoral hernia.  This offers a very good view of the abnormal obturator  artery from above, if it 1s present.    SURGERY OF FEMORAL HERNIA  Should be done as early as possible, once the diagnosis is  made.  For elective repair, low (femoral) approach: Incision directly  over the swelling is ideal. Injury to abdominal obturator artery  can occur in this route (found on lateral side in 20% of cases)  Transinguinal (Lothiessen): Can use it when there is  gangrene. However, it may weaken inguinal canal.  Combined: High approach is the choice for strangulated  femoral hernias. Approximate inguinal ligament and pectineal  ligament.    ¢ Repair is done by suturing the conjoined tendon to iliopecti  neal line.    Inguinofemoral approach: A vertical incision 1s made ove  the swelling and extended above the inguinal ligament, anc  the sac can be dissected from both above and below (lool  for abnormal obturator artery—see below). This approac}  has the advantages of both operations mentioned above.    Normal obturator artery is a branch of the internal iliac artery  It gives a pubic branch which anastomoses with pubic branct  of the inferior epigastric artery. Occasionally, this anastomosis  is large and obturator artery then appears to be a branch of the  inferior epigastric. Usually it passes lateral to the femoral cana!  in contact with the femoral vein. Occasionally, the abnormal  artery may lie along the medial margin of the femoral ring.  i.e. along the free margin of the lacunar ligament. This artery  is in danger during surgery for obstructed femoral hernia.    1. As the femoral ring and the neck of the sac are narrow,  obstruction and strangulation are very common.    ¢ Commonly seen in femoral hernias and obturator hernias,  which have narrow necks.   ¢ This occurs when a portion of the circumference of  the bowel is caught within the hernial sac and which is  constricted by the narrow ring. Signs and symptoms of  intestinal obstruction are absent, even though it is an  obstructed hernia, because the lumen is not obstructed.   ¢ The hernia is tense, tender, irreducible and has no cough  impulse.   ¢ As the lumen is patent, there may be bloody diarrhoea  rather than constipation. Gangrene can occur soon.   FEMORAL HERNIA  e Rarely occurs in males (5—10%)  e Commonly associated with Richter’s hernia  e Fatty female with small swelling under a big belly usually  goes undetected.  e Dangerous because of early strangulation  e Cannot be controlled by a truss  e Surgical repair is a must    RARE TYPES OF FEMORAL HERNIA 5. Enlarged femoral lymph nodes are firm and round. They    can be enlarged in lower limb infections, abrasions, wounds  in the perineum and also in carcinoma penis (Fig. 34.47).  6. Psoas bursa: Osteoarthritis of the hip can produce  distension of psoas bursa, which disappears on flexing    the hip. Tuberculosis spine can present as iliopsoas abscess  2. Prevascular hernia (Fig. 34.48).   1. Lacunar hernia (Laugier’s hernia)  In this case, the hernia passes through a small defect in the  lacunar ligament.    In this case, the hernial sac 1s located behind the femoral  vessels and the inguinal ligament. It may be associated with  congenital dislocation of the hip (Narath’s hernia).   3. Pectineal hernia  In this case, the hernia passes between the pectineus muscle  and its fascia, behind the femoral vessels. It 1s also called  Cloquet’s hernia.    It is a hernia lateral to the femoral artery (Hesselbach’s  hernia).    In congenital dislocation of the hip, femoral hernia occurs  behind femoral vessels. Hence, femoral artery pulsations  will be very prominent.    1. Inguinal hernia: An inguinal hernia is above and medial  to pubic tubercle. The femoral hernia is below and lateral  to pubic tubercle (Fig. 34.43).   2. Saphena varix: It is the dilated, saccular, upper end of  long saphenous vein with varicosity. It disappears on lying  down because of gravity. Thrill may be felt on coughing  (Fig. 34.44).    4. Femoral artery aneurysm is rare. It presents as a pulsatile  swelling in the groin with a continuous murmur. Peripheral  pulses are often weak (Fig. 34.46).    7. Psoas abscess: It 1s an iliopsoas abscess due to tuberculosis  of spine. There are two swellings, one above and one below  the inguinal ligament. Cross fluctuation can be elicited  between these two swellings. Tenderness over the spine and  X-ray of the spine help in arriving at a diagnosis  (Fig. 34.49).    It can be discussed under three headings:  I. Umbilical hemia of newborm  I]. Umbilical hernia of infants and children  III. Umbilical hermia of adults.   ¢ Failure of midgut as a whole or part to return into coelomic  cavity during embryonic life results in exomphalos.    ¢ It is also associated with weakness of abdominal  musculature (few fibres may be absent). Two types have  been recognised.   ¢ Inthis condition, the umbilical cord is attached to the summit  of the sac.   ¢ It is treated by twisting the cord and ligating the sac. Care  should be taken to avoid damage to the intestine. For  example, nursing the child preoperatively in prone position  can damage intestines.    In this condition, the umbilical cord is attached to the inferio  aspect of the sac, containing intestines, abdominal structures  e.g. liver, bowel.    Many children are still-born.  This type of hernia is usually associated with absen  abdominal musculature.    The operation should be done before the rupture of the sac  as the morbidity increases greatly in the event of a rupture  of the sac.  During the operation, skin flaps are raised on both sides tc  cover the defect. A true repair is necessary and tis done at <  later date.   Fig. 34.52: Same child on the 21st postoperative day. The repair  is done by rotating flaps from gluteal region of the parasitic baby  (Courtesy: Prof Vidyadhar Kinhal, Late Prof RS Channagiri,  Dr Channanna, Department of Surgery, Vijayanagar Institute of  Medical Sciences, Bellary, Karnataka)    e It occurs as a complication of umbilical sepsis, which  weakens the umbilical scar.   ¢ It is a true umbilical hernia containing either omentum/  intestines.   ¢ The child is brought with the complaint of swelling in the  umbilical region whenever the child cries.   ¢ Most of the cases are symptomless. Parents are anxious  about the swelling.    ¢ Reassurance is the most important advice given to the  parents.    No treatment is required other than strapping the  abdominal wall by keeping a pad in front of umbilicus.  Majority of the hernias get corrected by 2 years of age  (90%).   If the hernia does not correct itself, repair is necessary to  close the defect in the linea alba.    It is not a true umbilical hernia but it is a para-umbilical  hernia in which the hernia occurs either above, below or to  the side of the umbilicus, through the linea alba.   The contents are the greater omentum, transverse colon or  small bowel. Due to adhesion, it is often irreducible.    Females in the 5th decade are commonly affected. Male:  female ratio is 1:5.   Repeated pregnancies also weaken the abdominal wall.  Ascites may precipitate hernia specially in cirrhotic patients.    Patient presents with a swelling in the umbilical region,  which increases on straining or coughing.   On asking the patient to cough, expansile impulse is present  (Fig. 34.54).   Dragging pain is usually due to omentum which is felt as  a firm or granular mass. If gurgling 1s present, it 1s indicative  of small intestines in the hernial sac.    After reducing the swelling, the defect can be made out in  the linea alba.   See Table 34.4 for comparison between umbilical hernia in  infants and adults.   . Obstruction presents with colicky abdominal pain and  vomiting. Distension follows soon. Untreated cases develop  strangulation. Very often, these patients present with  incarcerated hernia due to the presence of transverse colon  in the sac. They require urgent intervention, failing which  gangrene will set in.   . As the sac enlarges, due to its weight and gravity, it sags  down resulting in friction of the skin and this causes  intertrigo (Fig. 34.55).    2. Anatomical repair: Small defects can be closed with non-  absorbable sutures such as nylon or prolene (Fig. 34.56).   Fig. 34.55: Umbilical hernia in a cirrhotic patient with skin changes  and ulceration   Fig. 34.56: True congenital umbilical hernia in an adult can be  repaired by simple closure of the defect by nonabsorbable sutures    3. Most favoured surgery for umbilical hernia 1s mesh repair.  It is a tensionless repair. It can also be done by laparo-  scopic method which Is popular today.   A curvilinear incision is made below the umbilicus or a  double semilunar incision can be used. Umbilical cicatrix  is then removed (in small hernias it can be preserved).    MAYO’S UMBILICAL HERNIORRHAPHY  e Excision of umbilicus  e Reduction of contents and excision of the sac  ¢ Double breasting of the fibrous aponeurotic layer  e Haemostasis, suction and obliteration of dead space  e Additional lipectomy and umbilicoplasty    e The sac 1s dissected all around and the defect in the linea  alba is defined.    ¢ Contents of the sac can be reduced with or without opening  of the sac.    ¢ Defect or cut in the linea alba is extended on both sides  (laterally) and then upper and lower aponeurotic flaps are  sutured together by using double breasting technique.    It is also called ventral hernia or postoperative hernia. It 1s a  hernia that occurs through a weak scar. Very common in  females.    It consists of skin, muscles, aponeurosis, linea alba, sheaths,  ligaments, openings—rings, blood vessels and nerves.  Anatomically weak areas are the rings, junctions, empty spaces  and where blood vessels pierce abdominal wall.    ¢ Above the costal margin, only external oblique with  aponeurosis contributes for rectus sheath.   ¢ Between xiphisternum and umbilicus, external oblique is  in front. Internal oblique splits to enclose the rectus muscles.  The transverse abdominus ts behind the internal oblique.  All fuse to form linea alba in the midline. Hence, this is the  strong midline area.   ¢ Below semilunar line: All 3 aponeurosis are anterior to the  muscles and fuse in the midline to form linea alba.   ¢ Rectus sheath—posterior rectus sheath 1s absent below  semilunar line. Incisional hernia and spigelian hernia are  common below the umbilicus.    wall. Hence, during the closure of the midline incisions, it  is important to include good bites through linea alba.   ¢ Umbilicus—strong fibrous ring. Umbilical hernias are common  in children due to childhood umbilical infections, in obese  patients due to weak muscles and in multiparous woman due  to stretching of the muscles due to repeated pregnancies.    ¢ The anterolateral abdominal wall is made up mainly of  muscles. On either side of the midline there are four large  muscles. These are the external oblique, the internal oblique,  the transversus abdominis and the rectus abdominis. Two  small muscles, the cremaster and the pyramidalis are also  present. The external oblique, the internal oblique and the  transversus abdominis are large flat muscles placed in the  anterolateral part of the abdominal wall. Each of them ends  In an extensive aponeurosis that reaches the midline. Here  the aponeuroses of the right and left sides decussate to form  a median band called /inea alba. The rectus abdominis runs  vertically on either side of the linea alba. It is enclosed in a  sheath formed by the aponeuroses of the flat muscles named  above.    e Nerve supply—lower six thoracic and first lumbar nerves.  They enter the rectus sheath laterally. In cases of para-  median incisions, after opening the anterior rectus sheath,  rectus should be retracted laterally to define and incise the  posterior rectus sheath.   ¢ Blood vessels run within the rectus sheath. Rupture of  inferior epigastric artery is a known entity resulting in a  haematoma below the umbilicus (see page 871). Differential  diagnosis includes spigelian hernia.   ¢ Weak muscles—interstitial hernias—prune-belly syndrome:  A partial or complete lack of abdominal muscles. There  may be wrinkly folds of skin covering the abdomen. An  undescended testicles in males.    : a a * = . a a = . a . a = c = = a = * . . * = a . a   CAUSES OF INCISIONAL HERNIA s  e Infection uncontrolled  e Incision wrongly placed  e Improper suture material . m  * Increased intra-abdominal pressure Fig. 34.60: Incisional hernia following laparotomy for perforated  "duodenal ulcer. He had stormy postoperative period   1. Infection: Cases operated for peritonitis such as perforated  duodenal ulcer, gangrene of the intestines, etc. usually  develop incisional hernia. The drainage tubes which are  placed inside the peritoneal cavity help in reducing the  postoperative incisional hernias, by draining peritoneal  contents outside.   2. Anatomical site: The midline! is especially weak in the  lower abdomen because of absence of posterior rectus sheath  below the arcuate line or semilunar line.   4. Faulty technique of closure of the abdomen or faulty a , st ot 4  sutures are also responsible for incisional hernia. | ee yy    further weakens the incision. . Narasimhan, Senior Consultant Surgeon, Mysore, Karnataka)  6. Wrongly placed incisions wherein nerves of the abdominal    muscles are cut, precipitate incisional hernia. Lumbar  Incisions, lower midline incisions and large transverse  Incisions Often give rise to incisional hernias (Figs 34.59 to  34.63).   7. Incisions wherein nerves are cut, the chances of incisional  hernias are move. Examples: Subcostal incision for removal  of gall bladder.    Fig. 34.62: Incisional hernia to the side (Courtesy: Dr Manjunath  Shenoy, Professor of Surgery JSS Medical Collage, Mysore)    Fig. 34.59: Incisional hernia with decubitus ulcer due to obesity  and weak muscle tone    Serosanguinous discharge on the 4th postoperative day  through the main suture line is a signal of development of  partial or total wound dehiscence. Such cases later develop  an incisional hernia.   History of infection during the first surgery, postoperative  cough is usually present.   Scar is thin and evidence of secondary healing in the form  of irregular scar may be present.   After reduction of the contents, a defect can be palpated  through the scar. Defect depends upon number of stitches  that have given way.    Surgical treatment is necessary if the defect is narrow, if  there is discomfort to the patient or if there is a danger of  obstruction (Figs 34.64 to 34.66).   There are various operations for the treatment of incisional  hernia depending upon the size of the defect, anatomical  location of the incision and presence of precipitating  factors.    In this operation, all the anatomical layers such as peritoneum,  posterior rectus sheath, linea alba and the subcutaneous tissue  are identified. Closure is done layer by layer by using  nonabsorbable suture material.    As most of the incisional hernias are due to a large defect  in the main incision and the majority of it occurs in obese  women, repair using mesh has become the most popular  method. Mesh repair is considered as the best repair,   In this operation, the sac is opened, greater omentum is  excised, the contents are reduced followed by closure of  the peritoneum. A mesh is kept in place which is sutured all  around to the locally available tissues, without tension.  Prolene mesh or Marlex mesh is commonly used. In all  these repairs, tensionless, nonabsorbable suture repairs  are done. The mesh is placed in the peritoneal space and  covered by rectus muscles. Seroma is a common  complication (Fig. 34.66).   Previous incision is opened to full length and the scar is  excised.   It is opened, contents reduced, may have to excise omentum  Redundant sac is excised   Placement of the mesh: Onlay means subcutaneous. It is a  simple procedure. Mesh should overlap at least 5 cm all  around the defect. Otherwise, plane is created between  posterior rectus sheath and rectus muscle, mesh is placed  in that location and anterior rectus sheath is sutured. This is  called retromuscular sublay mesh repair. This may not be  possible below the arcuate line because there is no posterior  rectus sheath there.   ¢ Mesh which has absorbable surface will face the peritoneal  cavity and nonabsorbable surface will face abdominal wall   e Transfascial sutures are put to fix the mesh (Figs 34.67 to  34.70).    * CT scan is the best investigation to assess how much bowel  is outside abdominal wall. If more than 25% is present  outside, sudden reduction of the contents will result in  compartment syndrome and pulmonary complications.   6. Additional lipectomy, removal of colon, omentectomy  may have to be done.   7. Ramirez component separation technique: In this,  relaxing incisions are given in the external oblique  aponeurosis or rectus sheath so that they can be brought  together and stitched together because in these cases  muscles are widely separated (Fig. 34.64).    This is the procedure of choice today. A very good view of  intraperitoneal contents and hernial sac 1s available once the  telescope is introduced within the peritoneal cavity. All contents  are reduced. A broad mesh can be placed in intraperitoneal or  preperitoneal space depending upon Its nature. Mesh Is sutured  all around. Recovery is fast and recurrence is very less.    ¢ Itis also known as the Keel operation of Rodney-Maingot.  It can be recommended for small hernias with good locally  available tissues.  -_ | ¢ In this operation, the sac is dissected and is pushed back  Fig. 34.69: Laparoscopic mobilisation of the sac into the abdomen without opening the peritoneum.   ¢ Typically the swelling is situated in the upper abdomen  midway between xiphoid process and umbilicus. Often, it  contains only an extraperitoneal protrusion of fat (Fig. 34.72).   ¢ Dull aching pain is due to the fatty contents which are  partially strangulated. However, tenderness is an important  feature of epigastric hernia (Key Box 34.18).    ¢ It is also called fatty hernia of the linea alba.    ¢ This type of hernia occurs in the epigastrium through the  linea alba which extends between the xiphoid process and  umbilicus.    ¢ Sudden straining or heavy exercise results in the tearing of  a few fibres of linea alba and 1s responsible for precipitating  an epigastric hernia. Initially there is a small protrusion of  extraperitoneal pad of fat. Rarely, if it enlarges, it 1s due to  the dragging of the peritoneal sac. The opening is very  narrow. Hence, the hollow viscus cannot enter the sac.  Diastasis of rectus muscles which results in a wide linea Fig. 34.73: Epigastric hernia: On head-raising, it becomes more  alba can also precipitate an epigastric hernia. prominent (head-raising test)    vessel enters the pad of fat. If a hernial sac is present, it 1s  opened, the contents are reduced and the defect is closed using  nonabsorbable sutures (Fig. 34.74).   PECULIARITIES OF EPIGASTRIC HERNIA  ¢ Common in muscular men  e Hernial sac is uncommon  e Hollow viscus in the sac is rare  e Impulse on cough is rare  e Reducibility is rare  e Tenderness is an important feature    ¢ Basically, they are inguinal hernias. However, the processus  vaginalis sac instead of following the normal route into the  scrotum, traverses between various layers of the abdominal  wall (parietes) resulting in interstitial hernias.    ¢ Patients with Down’s syndrome and prune-belly syndrome  are commonly affected.    1. Preperitoneal: In this variety the hernial sac lies between  the transversalis fascia and peritoneum. It 1s seen in about  20% of patients. The sac is like a small diverticulum.    2. Interparietal: It 1s also called as intermuscular type. It is  the commonest variety wherein the sac passes between the  external oblique and internal oblique muscles. The swelling  caused by the hernial sac causes discomfort to the patient.  Sometimes, this can be a bilocular sac.    3. Extraparietal: It is also known as inguino-superficial  variety. In this variety the hernial sac passes exterior  (superficial) to the external oblique aponeurosis beneath  superficial fascia of the abdominal wall. It is commonly  associated with undescended testis or ectopic testis.   ¢ Majority of such cases present with features of intestinal  obstruction.   ¢ They are treated by identifying the sac, excision followed  by closure of the defect or repair by using nonabsorbable  sutures.    ¢ Itisan interstitial hernia which occurs through the spigelian  fascia. This is a thin strip of fascia which runs parallel to  the outer border of rectus sheath from the tip of the 9th  costal cartilage to the pubic tubercle.   ¢ Since it is very wide in the region of umbilicus/arcuate line,  spigelian hernias occur commonly at this level.   ¢ Spigelian fascia contributes a few fibres to form rectus  sheath.   ¢ It is a 6 cm horizontal transverse zone located within  umbilicus and 2 anterior superior iliac spine.    Repeated pregnancies, advancing age, obesity, muscular  degeneration, sudden strain due to coughing, weight lifting,  etc. give rise to spigelian hernias.    ¢ Seen in both sexes equally around 50 years of age.   ¢ A round, soft, reducible swelling situated just below and  lateral to the umbilicus—located typically at the junction  of the arcuate line and lateral border of rectus abdominis.  Sometimes, it is tender.   ¢ As the hernia enlarges, it insinuates between external and  internal oblique muscle. Hence, it is an example for  interparietal hernia (Figs 34.75 to 34.78).    1. An ultrasound can define the defect in the semilunar line.  2. X-ray abdomen, lateral view shows coils of bowel outside  the peritoneal cavity.    ' Prof Spigel, Professor of Anatomy and Surgery not only described spigelian fascia but also the caudate lobe of the liver.    Fig. 34.75: Obstructed spigelian hernia in a 70-year-old lady—  first presentation to the hospital    give rise to impulse on cough. It occurs suddenly and it  will be a tender swelling.    ¢ Pyogenic or pyaemic abscess can occur in the abdominal  wall, more so, in diabetic patients. Tenderness and high  temperature clinches the diagnosis.    Strangulation is common due to the rigid fascial ring  surrounding the hernial sac. Richter’s hernia also can occur  here.    An incision of about 5 to 6 cm 1s made over the swelling and  abdominal wall muscles are split or cut. The sac is excised   Fig. 34.78: Contents of the sac  (Courtesy: Prof MG Shenoy and Dr Prasad, KMC, Manipal, Figs 34.77 and 34.78)    after reducing the contents and the defect is repaired.  Recurrence occurs 1n about 5% of the patients.    Two types of lumbar hernia are well-recognised. They are as  follows:  1. Primary which occurs through an anatomical defect:  ¢ Through the inferior lumbar triangle of Petit. Its  boundaries are:  Inferiorly: Iliac crest  Laterally: External oblique  Medially: Latissimus dorsi  ¢ Through the superior lumbar triangle of Grynfeltt. Its  boundaries are (Fig. 34.79)  Above: 12th rib  Medially: Sacrospinalis  Laterally:; Internal oblique    2. Secondary to a renal operation done through a loin incision.  It is an example of a /wmbar incisional hernia, which occurs  due to either infection or weakness of loin muscles. The  operation done for tuberculosis of spine through a loin  incision, very often gives rise to a secondary lumbar hernia  (it is an incisional hernia) (Fig. 34.80).    1. Lipoma is common in the lumbar region (loin). It 1s soft,  lobular, and slips under the palpating fingers.    2. Cold abscess secondary to tuberculosis of the spine gives  rise to a nontender swelling in the paravertebral space.  Tenderness is present over the spine which gives a clue to  the diagnosis. Patients may have deformity of the spine in  the form of gibbus.    Small defects can be closed with simple sutures. Large defects  need to be closed with or without mesh.    ¢ This hernia occurs through the obturator canal which is  bounded above by the superior ramus of pubis and below  by the sharp edge of the obturator membrane.   ¢ As the hernia is covered by the pectineus muscle, it is often  overlooked.    ¢ In females, the obturator foram en is wider in the transverse  direction (it is triangular in shape in females and oval in  males).    ¢ The most common presentation is acute intestinal  obstruction with strangulation (80%). Recurrent attacks  of intestinal obstruction which get resolved spontaneously  is also common (Key Box 34.19).   ¢ This hernia causes more pain than any other type of hernia.  Pain often radiates along the obturator nerve and may even  be referred to the knee via its geniculate branch called  Howship-Romberg sign. The leg is usually kept in the  semiflexed position and movement of the limb gives rise to  pain. If the limb is flexed, abducted and rotated outwards  the hernia becomes prominent. Patients are usually over 60  years of age and women are more frequently affected than  men.   ¢ Due to strangulation and blood in the hernial sac, bruising  is seen below the medial edge of the inguinal ligament.   ¢ A few patients (20%) complain of palpable Aernial mass  in the groin.   ¢ Per vaginal examination can reveal a tender lump on the  lateral side of the vault.    The constricting agent in case of obstruction is the obturator  fascia, which needs to be divided. Nerves and vessels are  posterolateral to the hernial sac. Since majority of the cases  present with intestinal obstruction and strangulation, a lower  laparotomy is done. A grooved director is used to divide the    e The most common presentation is not a swelling but acute  intestinal obstruction   e Vaginal examination: Tender mass can be felt on the lateral  side   ¢ Closure of the obturator opening is done by stitching the broad  ligament over the opening or by using monofilament nylon.    These are very rare hernias which confuse many clinicians  and present in a different varieties. Hernia protrudes through  muscles and fascia of the perineal floor.    1. Anterolateral perineal hernia: This occurs in women and  presents as a swelling of the labium majus. Often, the patient  is examined by a gynaecologist and Bartholin’s cyst is  diagnosed (Figs 34.81 and 34.82).   Figs 34.81 and 34.82: A 40-year-old lady with large perineal  hernia was diagnosed to have a Bartholin’s cyst and was explored  by a gynaecologist. To his surprise, it had 3 feet of small intestine  and portions of large intestines. It started bleeding and he kept a  drain and referred the patient to our hospital    2. Posterolateral perineal hernia: This type of hernia passe:  through levator ani and enters ischiorectal fossa.   4. Postoperative hernia through perineal scar, e.g. afte!  abdominoperineal resection wherein rectum is removed.  ¢ It also used to occur following perineal prostatectomy.    They can present as asymptomatic swelling, pain, dysuria.  bowel obstruction or perineal ulceration and bleeding.   ¢ CT scan/MRI can clearly define the course of hernia, its  relationship with urinary bladder/ureter and its descent into  the pelvis.    It can be very difficult in large hernias. Often a combined  approach, both perineal and abdominal, may be necessary.  Mesh repair with adequate fascial and muscular perineal repair  is required (Fig. 34.83).   Fig. 34.83: Contents were reduced, sac was excised and a large  mesh could be placed in the defect and sutured all around.  Postoperatively she had urinary fistula. CT cystogram revealed  bladder injury. It healed after two weeks   New development in the repair of hernia such as hernia  system and plug repair are added    About deep inguinal ring, which one of the following  is true?   C. Pantaloon hernia can be on both sides of deep ring   D. It is closed at the end of hernia repair to prevent  recurrence   C. Only a part of circumference of the intestine 1s caught  in a hernial sac    Immediate structure anterior to direct hernial sac is:  A. External oblique aponeurosis    Which one of the following is the most important step  in preventing recurrence of hernia?   B. Closure of the ring is done by prolene suture material    In spigelian hernia swelling is seen:  A. Below and lateral to umbilicus    Following are true for obturator hernia except:  A. Hernia is covered by pectineus muscle   D 12 D 113A 14 D 15 OD 16 A 17 A 18 D 19 C 20 B  22 A 23 D 24 A 25 B    CLASSIFICATION OF UMBILICAL DISEASES 1. Adenoma: Raspberry tumour  2. Endometrioma  B. Malignant  |. Primary carcinoma  2. Secondary carcinoma from: stomach, colon, ovary,    rate . and breast  rm nuLOnIEAD sintis IV. Umbilical hernia  I]. Fistulae V. Umbilical calculus (umbolith)  A. Faecal  1. Patent vitellointestinal duct (Key Box 35.1) INFLAMMATION  2. Carcinoma transverse colon a  3. Tuberculous peritonitis A. Omphalitis  B. Urinary: Patent urachus ¢ Inflammation of the umbilical cord due to Staphylococcus  C. Biliary aureus and Streptococci occurs in the neonatal period 3-4  III. Neoplasms days after birth. The incidence is increased in hospital births.  A. Benign ¢ Rarely, gram-negative organisms and Clostridium tetani can    cause omphalitis! , If strict aseptic precautions are not taken.  If infection is not controlled, it can result in further   UMBILICAL CORD STRUCTURES 1. Abscess of the abdominal wall: Pus can be seen coming  IN FOETAL LIFE out of umbilicus. It may need drainage with antibiotic  ¢ Umbilical vein cover. Gentle squeezing will help, followed by antiseptic  e Right and left umbilical arteries dressings and systemic antibiotics.  e Urachus 2. Extensive ulceration of the abdominal wall similar to  IN EMBRYONIC LIFE Meleney’s ulcer, is a rare complication (subcutaneous  Vitellointestinal duct and structures mentioned above. synergistic gangrene) of omphalitis.    ! Application of cowdung to the umbilical cord is still prevalent in a few places in our country. In addition to causing omphalitis, it can also cause  neonatal tetanus.    3. Septicaemia can occur due to organisms entering the  umbilical vein and then into portal vein. This results in  pylephlebitis with jaundice, fever, chills and rigors.    6. Umbilical hernia can occur due to a weak scar produced  by sepsis.    Granuloma indicates persisting inflammation underneath. It  is a cause of great concern and worry to the patients. This can  be destroyed by application of copper sulphate or silver  nitrate solution.    Dermatitis more often occurs in adults wherein chronic  infection of the umbilicus sets in with foul smelling discharge.    Umbilicus is a low area compared to the surface of abdominal  wall. Hence, hairy men may shed their hair which accumulates  in the umbilicus and may result in pilonidal sinus. It may need  removal of sinus along with tuft of hair or rarely the umbilicus  itself.    1. Persistent vitellointestinal duct is an uncommon  congenital anomaly. Many a time the intestinal opening is  so small that only mucoid contents come out of umbilicus.  Rarely, if the opening is big, omphaloenteric faecal fistula  results.    2. Internal hollow viscus malignancies, especially carcinoma  of the transverse colon can erode through umbilicus  resulting in a faecal fistula.    3. Tuberculous peritonitis induces dense adhesions, strictures  and perforations. A perforation which 1s sealed off by coils  of matted bowel and omentum results in local abscess which  may perforate through a weak point, 1.e. umbilicus resulting  in a faecobiliary fistula. If a diagnosis can be proved by  wall biopsy of the sinus/fistula antituberculous treatment  can cure the disease. Laparotomy is extremely difficult in  such cases. One may end up creating more holes in the  bowel and 1s better avoided.    One innocent disease (V I DUCT), one prevalent disease  (TB) and one malignant disease (carcinoma bowel) are  the causes of umbilical faecal fistula.    ¢ The ventral urogenital sinus  which forms the urinary bladder  is continued cranially as urachus  which extends into the umbilical  cord—allantoic stalk. If this  portion persists, patent urachus  forms which connect umbilicus  with urinary bladder. If it 1s  fibrosed, as it occurs normally,  it 1s called median umbilical  ligament.   ¢ A patent urachus may manifest  as urinary discharge from  umbilicus. It manifests usually in childhood and early adult  life. In most cases, there will be some kind of obstruction  to the normal passage of urine. Entire urachus 1s excised  after correcting distal obstruction.    Rarely, perforation of gall bladder due to severe form of  cholecystitis may result in local abscess which may rupture  through umbilicus resulting in biliary fistula. Instances are  recorded wherein stones have come out of umbilicus.    1. Umbilical adenoma is a pedunculated swelling having  raspberry colour. Hence, the name Raspberry tumour.    ¢ Mucosa of the persistent duct prolapses through  umbilicus and produces this adenoma.    e It is moist with mucus and tends to bleed (columnar  epithelium rich in goblet cells).    Treatment  e If the tumour is pedunculated, a ligature is tied around  it and by a few days, the adenoma drops off.  ¢ If tumour reappears, excision of umbilicus is advised.  2. Endometrioma of umbilicus is rare but patients have a  typical history to tell, i.e. it bleeds during menstruation.    3. Malignant: Secondary carcinomatous nodule in and around  umbilicus reflects advanced malignancy commonly from  stomach and colon. Ovary, uterus, breast are other causes.    The nodule is tender, fixed and reddish 1n colour(Fig. 35.2).  Figures 35.3 to 35.7 show some interesting diseases of  umbilicus.    Umbilicus is one of the weak points in the body. Hence, it is  one of the sites of hernia. All the details of umbilical hernia  have been discussed in page 566.    ¢ It1s composed of desquamated epithelium which becomes  inspissated and gets collected in the umbilicus. With  secondary infection, there will be blood-stained discharge.  It 1s treated by controlling infection, debridement and if  necessary removal of umbilicus.    Abdominal wall is one of the sites of pyogenic abscess  especially in diabetic patients. It is apart of pyaemia. Localised    Fig. 35.8: Abdominal  wall abscess in a  diabetic patient (see  clinical notes)    hE  A 60-year-old diabetic, male patient  had a large pyaemic abscess on the  abdominal wall below and to the right  ofumbilicus. An incision and drainage  of the abscess was done. Within 2 hours  of surgery, the surgeon was called to  see this patient who had hypotension  and blood was pouring out of the  incision. Exploration of the wound  revealed large ‘clots’ and bleeding  from inferior epigastric artery, which  was ligated. Probably, while breaking  all the loculi of the abscess cavity, the  vessel was injured.    tenderness suggests an abscess. Diagnosis can be confirmed  by ultrasound and it is treated by incision and drainage.    ¢ Veins are seen in portal hypertension. In relation to  umbilicus, they are called as caput medusae. Direction of   Fig. 35.10: Veins on the  lateral abdominal wall  suggestive of inferior vena  caval obstruction    Fig. 35.9: Dilated tortuous veins  due to portal hypertension—  caput medusae    the veins are important which can be demonstrated by  emptying the vein and filling.   ¢ In cases of inferior vena caval obstruction, veins are seen  on the flank. These veins are called as inguino-axillary veins  (Fig. 35.10).    A soundly healed abdominal scar can withstand any amount  of intra-abdominal pressure. However, 1—2% of the abdominal  wounds (incisions) give way resulting in prolapse of intra-  abdominal contents outside.   This causes great concern, or anxiety to the patient, and  more so for relatives. It is said that the anxiety and worry  caused by the intestines prolapsing out is much more than  that is caused by emergency re-explorations for open cardiac  surgery. It 1s not possible to prevent wound dehiscence totally  because causative agents are multifactorial.    1. Surgery: It depends upon the type of surgery done. Surgery  done for grossly contaminated cases such as peritonitis,  biliary fistula or faecal fistula have a high incidence of  wound dehiscence (Fig. 35.11).   Fig. 35.11: Wound dehiscence in a case of APR (abdomino  perineal resection)—probably due to placement of colostom\  closer to the suture line    2. Sepsis: Uncontrolled infection (sepsis) can digest the suture  material used and will result in burst abdomen.   3. Suture material used: Absorbable sutures such as catgu  give rise to increased incidence of wound dehiscence thar  nonabsorbable sutures.   4. Surgeon-related factors: Meticulous dissection, haemo-  stasis, gentle handling of tissues, a good tensionless tight  closure, carefully judged incisions will have reducec  incidence of burst abdomen. Midline vertical incisions have  decreased chance of wound dehiscence than paramedian  Incision.   5. Sick patient: Patients with malignancy, jaundice, obesity,  anaemia, hypoproteinaemia, uraemia have poor wound  healing.   6. Straining: In the postoperative period, violent cough,  persistent vomiting, abdominal distension due to paralytic  Ileus predispose to burst abdomen.    ¢ Patients who are recovering reasonably well in the  postoperative period suddenly complain of pink or brownish  coloured serosanguinous discharge. It is the pathognomonic  sign of burst abdomen.   « It usually occurs on the 6th to 8th postoperative day.   ¢ If skin sutures are removed, omentum or small bowel coils  will be seen outside.    Interestingly, it is a painless, shockless disruption (with)  full of apprehension.   ¢ The bowel or the contents are covered with pads and  bandage.   2. Bowel is washed with saline and gently replaced into the   peritoneal cavity.  . Edges of the wound/incision are trimmed.  4. A single layer closure of the abdominal layer, by taking  suture bites through whole thickness of the abdominal wall  is done.  ¢ A few tension sutures (Figs 35.12 and 35.13) tied over a  rubber or a plastic tube are placed and are removed after  2 weeks.   ¢ Itshould be remembered that secondary wound healing  is better than primary wound healing and infection  rarely occurs.   * ££ . |  Figs 35.12 and 35.13: Wound dehiscence due to gangrene of    the intestine—laparotomy was done, resection followed by closure  of the abdomen with tension sutures    5. Closer of midline incision: In all cases of midline incision  it is the linea alba with or without anterior fascial layer is  approximated to the corresponding fascia on the other side.  ¢ “Fascia” usually refers to the anterior rectus fascia, the  fascia above the rectus muscles. This fascia holds the  abdomen together and is the most important layer of  closure.   ¢ The fascia can extend beyond the muscles and bind to  other fascia.   ¢ Suture length (SL) : width length (WL) ratio > 4  ¢ Small stitches at close intervals   e Disadvantages (theoretical): Wound security depends on  single strand of suture and limited number of knots.    ¢ In this condition, the two rectus abdominal muscles are  widely separated (not in the midline).   ¢ Repeated pregnancy in quick succession 1s the most impor-  tant cause. Chronic constipation or overstraining may be  another factor. Obviously women are commonly affected.   occurs due to tearing of one of the branches of inferior   2. Straining: Sudden straining such as violent cough or  vigorous exercise in a muscular man can cause haematoma.   3. Pregnancy: Rarely, the cause of haematoma can be  pregnancy, In late trimester. The exact cause 1s not known.   e A tender lump develops just below and to the side of  umbilicus at the level of arcuate line where posterior rectus  sheath is absent.   * If it persists or progresses or if there 1s a doubt about the  diagnosis, exploration and evacuation of haematoma should  be done and the bleeding vessels are ligated. The results  and recovery are excellent.    This dangerous complication is rare nowadays, thanks to the  good pre- and postoperative antibiotics.   ¢ Surgical operations which have increased risk of Meleney’s  gangrene include perforated appendix, biliary tract surgery,  colectomy, etc.   e The spread may occur within 3—5 days, with extensive  gangrene and sloughing of the skin of the abdominal wall  with purulent discharge.   ¢ Fever of moderate degree, an extremely tender abdominal  wall and purulent discharge.    1. At the stage of cellulitis: Broad spectrum antibiotics to  cover not only the organisms mentioned above but to cover  anaerobic organisms. Thus, a combination of benzy!]  penicillin, gentamicin and metronidazole ts used.   2. At the stage of gangrene: Emergency aggressive debride-  ment is the treatment. Dead skin and subcutaneous tissue  1s excised, pus drained and the slough 1s removed.    ¢ Superficial or deep. Deep fibromatoses, also called  Aggressive fibromatoses and desmoid tumours 1s an  uncapsulated fibroma which occurs in the abdominal wall.    ¢ It arises from muscles and aponeurotic layer of the  abdominal wall.    ¢ In children, most desmoid tumours are extra-abdominal  with a female predominance.   ¢ In young adults desmoid tumours almost always occur in  the abdominal wall (of women). Hormonal effects and  pregnancy are believed to influence the growth of this  tumour.   ¢ Some tumours express hormone receptors (oestrogen and  progesterone), and therefore tamoxifen and other hormonal  modulators are among the adjuvant therapies for this  tumour.    ¢ Childbirth, trauma or operative scars are the possible  aetiological factors.    ¢ Desmoid tumour is one of the components of Gardner’s  syndrome.    e It is benign but has a tendency to infiltrate the muscles.  Some fibromas exhibit dysplastic changes. The cut surface  is compared to an onion—whorled fibroma with spindle-  shaped cells.    ¢ Abdominal fibromatosis is far less prone to recurrences  than desmoid tumours in other sites.   e It usually occurs in the abdominal wall of women of  childbearing age during or after pregnancy.   ¢ Clinically the lesions present as deep-seated, firm,  nonencapsulated, slow-growing, locally invasive and  painless masses.    e It typically manifests as a slow-growing, progressive mass  that becomes more prominent on abdominal muscle  contraction. Mass is in the abdominal wall and is firm to  hard 1n consistency. °  ¢ Mesenteric fibromatosis, probably the commonest among °  the group, usually presents as a slow-growing mass that 8  involves small bowel mesentery or retroperitoneum. °  e The recurrence rate of mesenteric fibromatosis seems to :  be substantially higher in patients who have Gardner’s :  syndrome than in patients who do not have this syndrome.    e Simpleexcision results in recurrence. Hence, wide excision  with 2—3 cm of normal healthy margins 1s necessary with  reconstruction of the abdominal wall (Figs 35.14 and 35.15). °  ¢ Inspite of adequate surgery, 10-20% chances of recurrence .    Fig. 35.14: Wide excision of recurrent desmoid tumour in the  abdominal wall   Fig. 35.15: Specimen of desmoid tumour which is removed along  with normal tissue   ¢ Painful, palpable swelling, more symptomatic at the time  of menstruation are characteristic features.  — Perimenstrual cyclical bleeding can occur   e Otherwise, excision of the nodule has to be done (Fig. 35.17).    PECULIARITIES OF DESMOID TUMOUR 2  Uncapsulated fibroma  infiltrates muscles, even though benign  Does not change into sarcoma  It may be a part of Gardner’s syndrome  Simple excision results in recurrence  Wide excision is recommended    Desmoid tumour and fibromatosis have been updated.  A few coloured photographs have been added.    Blunt abdominal trauma (BAT) is one of the common  surgical emergencies encountered by general surgeons.  Increasing number of vehicles, high speed and poor  maintenance of the roads are the contributing factors. Blunt  injury abdomen with polytrauma is one of the commonest  causes of death in the younger population. Thus, it 1s important  for a house officer to recognise a polytrauma patient, to  diagnose and to suspect intra-abdominal injury, so that urgent  resuscitation and treatment can be offered to the patient at the  proper time, at the proper hospital and by a proper surgeon.  Major systems involved are given in Key Boxes 36.1 and 36.2.   Craniospinal and chest injuries are discussed in their  respective chapters. Pelvic and skeletal injury 1s beyond the  limits of this book. In this chapter, blunt injury of the abdomen  1s discussed.    1. Rail and road traffic accidents (most common)  2. Fall from a height and dashing against an object  3. Seat belt syndrome    ¢ Vehicular trauma is by far the leading cause of blunt  abdominal trauma.  ¢ Broadly BAT can be explained by three mechanisms:  1. Rapid deceleration: It causes differential movement  among adjacent structures. Shearing forces are created;   2. Crushing effect: Here solidviscera are crushed between  anterior abdominal wall and vertebral column or posterior  thoracic cage.   3. Sudden dramatic rise in the intra-abdominal pressure  due to external compression  ¢ Hollow viscus ruptures (in accordance with principles  of Boyle’s law).    Complete blood count, coagulation studies, grouping and  cross-matching. Fall in haemoglobin 1s an indication of on-  going haemorrhage—especially while managing a patient  with liver/splenic injury on conservative line of management.    It is indicated in BAT in following situations:  Multiple injuries and shock    Open: Infraumbilical skin incision and open peritoneum.  Semiopen: [nfraumbilical skin incision deepen up to linea  alba.   Fig. 36.1: Chest X-ray showing diaphragmatic hernia on the lef  side    Foley’s catheter to empty bladder  Ryle’s tube to empty stomach  X-ray pelvis to detect pelvic fracture.    10 ml of gross blood aspirate before infusion of lavage fluid.  More than 100,000 RBC/ml   Positive DPL means intraperitoneal injury is present. It  does not mean that the patient should be shifted to  operation theatre immediately.    7. CT scan (Fig. 36.3)  ¢ Gold standard for solid organ injuries  ¢ CT also can reveal other associated injuries such as  vertebral or pelvic fractures.  ¢ CT can also pick up diaphragmatic injury (CT chest).  e Jt can detect source of haemorrhage  ¢ CT is an excellent scan for pancreas, duodenum, etc.   Fig. 36.3: Diaphragmatic hernia—CT scan (Courtesy: Dr Yashdeep  Sharma, Associate Professor, KMC, Manipal)    8. Diagnostic laparoscopy: Done when CT scan is negative,  suspicion of diaphragmatic injury is present (Fig. 36.4).    Stable patients with solid organ injuries are managed  more often by nonsurgical methods with close  monitoring.    With the availability of FAST and CT scan, role of DPL is  now limited to unstable patients whose FAST results are  negative or inconclusive.    * Initial clinical assessment in an alert patient: Pain,  tenderness, gastrointestinal haemorrhage, hypovolaemia.  Clinical evaluation alone has an accuracy rate of only 65%  for detecting blood in peritoneal cavity.   ¢ Abdominal examination: Abrasions, contusions,  ecchymosis may indicate underlying visceral damage.    . Saree sign (Fig. 36.28): Across upper abdomen may be  associated with pancreatic/bowel injuries.    . Cullen’s sign: Ecchymosis in the umbilicus  . Grey Turner’s sign: Ecchymosis in the flanks  . Rib fractures on right side: Look for liver injury    . Rib fractures on the left side: Look for splenic injury   Repeat primary survey, secondary survey and repeat  laboratory/imaging studies (see Key Box 36.3 for wisdom lines  in blunt abdominal trauma).    . First priority in BAT is to assess and manage airway,    If a hypotensive patient responds to fast transfusion of  warm crystalloid solution, it means it is a hypovolaemic  shock. it rules out head injury.    Liver injury should be suspected when a patient with suspected  blunt injury abdomen is brought with the following features:   Fig. 36.5: Massive bleeding—challenging task (Courtesy: Dr Suni  Krishna, Assistant Professor, KMC, Manipal)    The most common presentation is features of intraperitonea  haemorrhage, which includes hypotension, thready pulse  abdominal distension as in Fig. 36.5. Peritoneal signs are  minimal as early bleeding does not produce much peritonea  irritation.   However, massive lacerations of the liver including stellate  fractures present with rapidly developing hypotension anc  shock, which are life-threatening.    Ultrasonography and more precisely CT scan should be  done in all patients who are haemodynamically stable with  or without support (Figs 36.6 to 36.9 and Key Box 36.4).  Also see Table 36.1    Right lower ribs fracture °  Injury marks on the lower chest or upper abdomen.    Patient with persistent hypotension or patient who had shock  following blunt injury abdomen.    A child can have liver injury without fracture of ribs because °  of elastic nature of the rib cage.    CT SCAN WITH IV CONTRAST  It can grade the liver injury  It can guide a conservative or operative treatment.  It also rules out other injuries    Grade | and Grade Il injuries can be managed by non-  operative treatment.    Free contrast in and around the liver is indicative of active  bleeding.    Subcapsular haematoma; < 10% surface area  Capsular tear, < 1 cm parenchymal depth  Subcapsular, 10-50% surface area; intraparenchymal extension < 10 cm   Subcapsular, > 50% surface area; expanding intraparenchymal haematoma  of >10 cm or expanding   Parenchymal disruption of 1-3 Couinaud’s segments within a single lobe  Parenchymal disruption > 3 Couinaud’s segments within a single lobe  Retrohepatic vena cava/central major hepatic veins    . Simple lacerations which are not bleeding at  laparotomy: A drain 1s kept in the liver bed, blood and  clots are sucked out and peritoneal wash 1s given.   . Simple laceration with bleeding: It is sutured by  interlocking horizontal mattress sutures by using special  liver suturing needle. If too much tension is applied while    suturing, cutting through can occur. Omentum can be used  as a Plug in between the laceration (Fig. 36.10). Absorbable  Sutures are used.    . Subcapsular haematoma: If present, should be evacuated.  . Deep laceration with bleeding: In such situations, wound    should be opened. Dead liver parenchyma is removed,  bleeding vessel at depth and biliary radicle are ligated. It is  described as tractotomy.    bleeding. Temporary control is obtained by compression  of portal vein and hepatic artery in gastrohepatic omentum  in front of foramen of Winslow (Pringle manoeuvre). If  bleeding stops, portal veins or branches of hepatic artery  are damaged. If bleeding continues, hepatic veins are the  source of bleeding. Visualisation of source of bleeding with  debridement of avascular liver tissue is done by finger  fracture method. Perihepatic packing can be used to  compress the liver as a temporary measure to buy time  for resuscitation, to explore rest of the abdomen or as a  definitive treatment when other measures fail. Pack is  usually removed after 24—48 hours (Fig. 36.11).   ¢ Nonanatomical resection may have to be done, in a few   < 1. Massive bleeding, hypovolaemia and cardiac arrest.  HAEMOSTATIC TECHNIQUES AT SURGERY | 2. Haematoma can get infected resulting in an abscess.    SUMMARY OF MANAGEMENT OF After 15 days, he came to our hospital with haematemesis.  LIVER INJURIES Initially it was diagnosed as erosive gastritis. Ultrasono-  © Laceration Suturing graphy revealed a pseudoaneurysm of one of the branches  e Expanding sub- of middle hepatic artery. CT angiography and embolisation  capsular haematoma Evacuation was tried but not successful. He had another bout of haema-  ° Deep laceration Suturing biliary radicles and temesis. He underwent exploratory laparotomy and ligation  portal radicles, packing, etc. of branches of middle hepatic artery. Bleeding stopped. Now  e Severe laceration Debridement since 6 months, no further attacks of haematemesis (Fig. 36.12).  e Stellate fracture Tractotomy; hepatic artery [t was a case of haemobilia (Fig. 36.13).  ligation  e Complex injuries Ligation of hepatic vein, portal    6. Complex liver injuries: These injuries involve hepatic  veins, retrohepatic vena cava or branches of portal vein  resulting in massive haemorrhage. This type of massive  injury can be managed by a large thoracoabdominal incision  or abdominosternal incision by doing sternotomy. Division  of the right triangular ligament helps in visualising bleeding  from hepatic veins.   * Schrock shunt: Failure of Pringle manoeuvre means  juxtahepatic and retrohepatic vena caval injuries. In such  cases, Heaney manoeuvre clamping both infra and  suprahepatic vena cava followed by atriocaval shunt or  venovenous bypass can be done.   Fig. 36.12: Laparotomy scar of the patient who had liver  laceration which was sutured. He presented with massive  haematemesis later—a case of haemobilia   Fig. 36.11: Perihepatic packing Fig. 36.13: Branch of middle hepatic artery ligated    ¢ The shearing injuries produce either disruption or laceration  of the bowel between fixed and mobile points, 1.e. at the  duodenojejunal flexure or at ileocaecal junction. These  are the most common sites of small bowel injuries.   e Injury to the small bowel can also occur due to crush injury  between spine and a steering wheel or handle bars, etc.   ¢ Bruising on the abdominal wall may suggest perforation  (Fig. 36.14).   e Mesentery and its vessels also get damaged and bleeding  can be sufficient to produce hypovolaemia and shock  (Fig. 36.15).   Fig. 36.14: Observe London's sign: Bruising (imprint abrasion) over  the abdominal wall signifying hollow viscus perforation   Fig. 36.15: Gangrene of the jejunal loop due to injury to  mesenteric blood vessels in the same patient in Fig. 36.14    perforation peritonitis with guarding and rigidity. Erect  abdominal X-ray shows gas under the diaphragm.    sealed off by coils of bowel and omentum. Most of these  patients present with abdominal pain. However, very often,  features of peritonitis are missed as a result of other  associated injuries such as fracture pelvis or  retroperitoneal haematoma. After 3--4 days, a localised  abscess may form and rupture into the peritoneal cavity,  resulting in peritonitis. This is aggravated by intake of  oral fluids which stimulate peristalsis. Repeated  examination is the most honoured, most fruitful  investigation in blunt injuries of the abdomen (see  clinical notes).    demonstrates free gas under the right dome of the diaphragm  in majority of cases. Four quadrant tap or diagnostic  peritoneal lavage is also useful.   When in doubt, CT scan of abdomen should be requested  to diagnose hollow viscus perforation and bleeding.    A 23-year-old male with fracture femur and pelvic fracture  was admitted to the hospital after 12 hours of injury. A general  surgeon was consulted to rule out an intra-abdominal injury.  Pulse rate was 100/min and on deep palpation, there was  tenderness in the right iliac fossa. Keeping in mind associated  pelvic injury, it was decided to treat him conservatively.  X-ray abdomen lefi lateral decubitus (Fig. 36.16) (erect film  could not be taken as patient could not stand) film did not  show free intraperitoneal air (gas). Ultrasound revealed a  retroperitoneal haematoma of 8 cm X 3 cm.   The patient was treated conservatively with Ryle s tube for  3 days. On the 4th day, oral fluids were started, as patient  passed stools once. On 7th day morning, patient had  tachypnoea. Pulse was 120/min, BP was 90/60 mm of Hg.   Previous 24 hours urine output was only 450 ml. Abdominal  examination revealed guarding and rigidity in the right iliac   fossa. It was decided to do alaparotomy. At laparotomy, there  was a small 2 cm perforation in the ileum with bilioma  surrounded by intestinal loops and gross contamination of  peritoneal cavity. The perforation was closed and the peri-   toneal cavity was drained. The patient made a good recovery  from septicaemia, thanks to early antibiotics and surgery.   Fig. 36.16: Lateral decubitus picture is extremely useful inpolyttauma = Fig. 36.17: Jejunal transection—6 cm away from DJ flexure. It is  cases when patient is unable to stand due to fractured limbs. However, better to resect and anastomose in this type of cases (Courtesy.  CT scan is the best investigation in blunt abdominal trauma Dr Saurabh Aggarwal, Associate Prof, KMC, Manipal)  because it not only detects pneumoperitoneum but also other injuries   Fig. 36.18: 72 hours old perforation close to ileocaecal junction. Fig. 36.19: Anastomotic leak following limited colectomy. lleostomy  Limited colectomy done done. It was closed after 8 weeks. You can see the wound infection    Fig. 36.22: Peritonitis following  ileal perforation. Golden time to  operate is within 6 hours of  perforation   ¢ Perforation: Single or multiple, have to be closed, after  trimming the edges by using nonabsorbable sutures such  as silk.   ¢ A lacerated or a macerated bowel has to be resected.   ¢ Bleeding mesenteric vessels have to be ligated, haematoma  must be evacuated and bowel should be inspected for  any ischaemia. Food particles and bile should be evacuated  (Figs 36.17 and 36.23).   ¢ A perforation of ileum close to the ileocaecal junction 1s  treated by ileocolectomy rather than simple closure for the  fear of enterocutaneous fistula, due to suture line leakage.   SMALL BOWEL INJURY  e Acute abdominal pain  e Guarding and rigidity  e Rebound tenderness  e Gas under diaphragm in plain X-ray abdomen erect  e Laparotomy and closure of perforation   ¢ Steering wheel injury can directly crush the transverse colon  and can cause perforation.   e Bruise or laceration of the colon can undergo ischaemic  necrosis and it can present after 5—7 days with signs of  peritonitis/sepsis (Figs 36.23 and 36.25).   ¢ Depending upon the contamination, contusion or laceration  and duration of injury, treatment can be resection and    Fig. 36.23: Colonic perforations. The patient presented  7 days later with abdominal distension and early sepsis—steering  wheel injury to the transverse colon    anastomosis within 6-8 hours of the injury or simple  suturing or diversion colostomy if gross contamination 1s  present.    ¢ Retroperitoneal duodenum is commonly injured.  e Steering wheel, belt or a blow in the epigastrium may injure  the duodenum as it 1s crushed against the spine.   ¢ Peritonitis features are not common as it Is the retro-  peritoneal duodenum (part II and III) that is injured.   ¢ Being retroperitoneal, these injuries manifest late with  abscess formation or fluid in lesser sac, etc.   e Raised serum amylase is one of the biochemical parameters  that should arouse a suspicion of pancreatic injuries along  with duodenal injury.   e When in doubt, about narrowing of lumen, duodeno-  jejunostomy may be indicated.    Because of anatomical close approximation of pancreas with  vertebral column, blunt injury abdomen in the epigastrium,  kicks or seat belt injuries crush the pancreas against the  vertebral column (Key Boxes 36.8 and 36.9).    Mill belt: Saree or churidar cloth may be caught in the belt of  a running conveyor belt and result in compression force in the  centre of the abdomen (Figs 36.27 to 36.31 and clinical notes  see next page).   ¢ Pancreatic injury alone is diagnosed when patient presents  with a pseudocyst of the pancreas 2—3 weeks following an  Injury.   ¢ Very often, laparotomy is done for haemorrhage or  perforation. In such situations, retroperitoneal bleeding,  collection of bile or collection of fluid in the lesser sac  arouses suspicion of pancreatic injuries.    1. Pseudocyst following blunt injury abdomen invariably  requires surgical drainage, e.g. cystogastrostomy because  of injury to pancreatic duct.   2. Injury to body and tail require subtotal pancreatectomy  with splenectomy.   3. Rarely, pancreaticoduodenectomy may be required tor  significant injury to the head of pancreas with injury to the  duodenum.    PANCREATIC INJURIES  e Anatomically hidden  Very often, injuries missed  Peritonitis features are not seen  Dangerous because of enzymatic activation  Can manifest as pleural effusion   The saree and churidar are traditional Indian dresses. One  end of the saree is tied around the waist and the other  draped freely along the shoulder. If care is not taken, the  free end often gets caught in the mill belt and causes  injuries around the waist (blunt abdominal trauma) due  to the drag created. The impression created on the  abdomen is by the saree and not the mill belt. The shawl  of the churidar (has two free ends) can get caught in a belt  or a wheel to cause injuries around the neck. The drag  may be sufficient to cause even strangulation. The author  remembers a case of scalp avulsion due to long hair of a  woman getting caught in a mill belt.    Fig. 36.27: Grinding mill having a conveyor belt (Courtesy: pp, Raghunath Prabhu, Associate Prof, KMC, Manipal)  Dr Rakesh Hegde, Associate Professor, Department of Surgery,    Fig. 36.28: Impression of the saree all around waist in a lady a    who was dragged by the conveyor belt when her saree got caught Fig. 36.31: Pancreas split into 2 parts   Types (Fig. 36.32)  I. Minor injuries: Subcapsular haematoma, minor  laceration and renal contusions.  II. Majorinjuries: Bleeding into renal pelvis from laceration  of medulla, corticomedullary rupture, hilar injury.    ¢ Haematuria is the most important (80-90%) sign of renal  injury. It may be mild, or sometimes can be massive  depending upon the extent of injury. It may be absent in  renal pedicle avulsion.   ¢ Retroperitoneal haematoma compressing on splanchnic  nerves (meteorism) results in paralytic ileus, which causes  abdominal distension.   ¢ Associated injuries such as fractures of the transverse  process of lumbar spine may be present.    1. Intravenous pyelography can demonstrate  ¢ Intrarenal extravasation  e Extrarenal extravasation (pararenal pseudohydrone-  phrosis due to extravasated blood and urine, slowly  occluding pelviureteric junction).  ¢ Function of injured kidney  ¢ Function of opposite kidney  2. Ultrasound and CT scan are other investigations which  are useful when there is an expanding haematoma  (Figs 36.33 and 36.34).   Fig. 36.32: Closed renal trauma: (1) Subcapsular haematoma,  (2) laceration, (3) avulsion of one of the poles and (4) avulsion of  renal pedicle   N | e  Fig. 36.33: Renal trauma: Right kidney is transected and upper  pole is displaced by a large haematoma. Left kidney is normal  (Courtesy: Dr Padmaraj Hedge, Prof and Head, Dept. of Urology,  KMC, Manipal)    1. Conservative: Minor injuries are managed conservatively  with close monitoring of vital signs such as pulse, blood  pressure, temperature and respiration, Hb% and PCV.  ¢ Sedation and analgesics are also given   Bleeding from vena cava and aorta can be fatal.  Haematomas which are not expanding should not be  disturbed.    The most frequent mechanisms causing pelvic fractures are  motor vehicle accidents, motorcycle accidents, falls and  accidents involving pedestrians. Associated injuries to  urethra in males should be ruled out first. Per rectal  examination should be done to evaluate the position of the  prostate. CT scan is done to assess pelvic fracture and also  to assess retroperitoneal haematoma (Figs 36.36 and 36.37).    Retroperitoneal bleeding can be arterial, venous, or osseous  in origin. Unstable pelvic fractures are generally associated  with increased blood loss. Posterior fractures with  involvement of the sacroiliac joint are frequently associated  with arterial bleeding, which can be controlled by  embolisation of the bleeding vessel, usually branches of  the internal iliac artery. Unstable fractures should be fixed  by external fixation. Expanding haematoma should be  explored. Control the bleeders, otherwise pack the pelvis.  Nonexpanding haematoma should not be explored. Wait  and watch.    « They are due to injury to  renat artery, renal vein or  renal injuries   B. Inframesocolic haematomas  are usually due to infrarenal  artery or vein, vena Caval    ¢ Iliac artery injury should be suspected  when there is absent groin pulse and  when haematoma is expanding    * Therapeutic embolisation of bleeder,  and external fixation of pelvic (es  ture are other methods to control    ¢ A patient should be admitted to the hospital and carefully  monitored if there 1s a slight doubt regarding blunt injury  abdomen.   ¢ Repeated examination, careful monitoring of pulse rate,  temperature and blood pressure, chest X-ray, estimation of  Hb%, frequently help in many cases of silent blunt injuries  (Fig. 36.38).   ¢ Most of the cases today are polytrauma cases, Hence, all  systems should be examined. Among all these, priority  should be given to life-threatening, salvageable injuries such  as extradural haematoma, haemothorax, splenic  injuries, liver injuries.   ¢ It is easier to make a diagnosis of fracture’ (revealed  injuries) which can be treated later. FRACTURE CAN  WAIT BUT NOT RUPTURE. Concealed injuries should  be carefully looked for.   ¢ Undoubtedly, diagnostic peritoneal lavage, ultrasound  (CT scan is the immediate noninvasive investigation) help  in diagnosis of more than 90% of cases of blunt injury  abdomen.   ¢ Adequate blood, appropriate antibiotics, aggressive resusci-  tation before surgery to treat hypovolaemia and shock are  the major factors which decide the outcome of surgery.   ¢ In a major accident involving many patients and limited  resources, quick decision should be taken regarding  triage—who can be saved, who cannot be saved (Figs 36.39  and 36.40).   Fig. 36.38: Acase of missed liver injury with haemoperitoneum.  Repeated examination revealed deteriorating general condition.  CT scan provided the diagnosis   Fig. 36.39: Pelvic fracture with ileal injury—frequent clinical  examination revealed guarding and rigidity (Courtesy: Dr Rajesh  Sisodia, Associate Prof, KMC, Manipal)   Fig. 36.40: Splenic haematoma—initially managed by  conservative method. However, patient was developing  hypotension—importance of careful monitoring of the patient in  intensive care unit    ¢ Bursting of bombs or shells rupture their casing and impart  high velocity to resulting fragments. These fragments cause  more devastating injuries than bullets. All explosives are  accompanied by a complex blast wave.   ¢ The two main components are: Blast pressure wave  (dynamic overpressure) with positive and negative phase  and mass movement of air (blast wind).   ¢ Positive phase of blast wave lasts few milliseconds (close  to the explosion it may be over 7000 kN/m?) (tympanic  membrane ruptures at 150 kN/m7’).    'Even a grandmother can diagnose fractures. However, it needs lot of experience and skills in diagnosing blunt injury abdomen.    e Like sound waves blast, pressure waves flow over and  around an obstruction and affect persons sheltering behind  a wall. The pressure affecting such a person 1s known as  incident pressure (pressure at 90° to direction of travel of  blast shock front).    ¢ Person standing in front of a wall facing an explosion is  subject to added effect of reflected pressure. Mass  movement of air displaces air at supersonic speed. This  disrupts environment, hurting debris and people. Blast wave  under the water travels at great speed and to greater distance.  Injuries tend to be complex and severe.    Structures injured by primary blast wave are ears, lungs,  heart and gastrointestinal system.    ¢ Most will have combination of blunt, blast and thermal  injuries. Deafness, lung contusion, capillary leakage and  haemorrhage into alveoli and ARDS precipitated by over  transfusion are the features. Perforation of the intestines  and penetration injuries to the eye are the other features.    e Management consists of resuscitation in a well-equipped  trauma unit, blood transfusions, intensive care monitoring,  antibiotics and appropriate surgical procedures.   Penetrating missile wounds, injuries from blast phenomena and  bums are typical features of modern conventional war. The most  common wounding agent in surviving casualties 1s a fragment  wound and nota bullet wound as many erroneously believe. The  aim in modern war is to incapacitate and not to kill. Hence, large  number ofsurviving casualties are a major financial and logistic  burden on a nation engaged In war.    Bullets fired from hand guns are propelled at low velocity, have  low available energy and result in low velocity transfer wounds  (100-500 J), whereas those from assault rifle have high velocity  and have high available energy (2000—3000 J) and they cause  high energy transfer wounds. Low energy transfer wounds  leave injury confined to wound tract. High energy transfer  wounds causes local laceration, crush injury and also cause  remote injury from wound tract due to temporary cavitation  phenomena.    e Entrance and exit wounds do not indicate considerable  damage that may have occurred to deeper structures.  e Resuscitate as per ATLS guidelines.    ¢ Under anaesthesia, excise skin around entry and exit wounds.  give liberal longitudinal incision through skin and deep fascia.  which allows proper visualisation of underlying structures.   e Injured tendons are trimmed and tied for easy identification  at future surgery.    ¢ Every penetrating and perforating missile wound of the  abdomen should be explored by laparotomy. A full midline  incision from xiphisternum to pubis 1s recommended and  it may be extended to thorax if necessary.   ¢ The rest of the treatment depends on the nature of injury.  Bleeding mesenteric vessels are ligated, injured small bowel  is repaired by suturing or by resection and anastomosis. In  colonic injuries, simple closure or closure with protective  colostomy 1s necessary depending upon the nature of the  colonic injury and contamination.   ¢ Liver, splenic, pancreatic and renal injuries have been  discussed in respective chapters.    Today all penetrating injuries of the abdomen need not be  explored by laparotomy. Good physical examination of the  patient, entry and exit point, and haemodynamic status of the  patient followed by investigations such as ultrasonogram and  CT scan will guide the decision for laparotomy.    ¢ Theterm triage is derived from the French word trier, meaning  to sort. This is a method of sorting out injured patients, during  mass casualties depending on the severity of injury. If you are  the first on the scene, first priority 1s to get expert help (call up  fire services, regional trauma centre, etc).   ¢ Triage 1s a skilled activity by a Trauma team, wherein there  is a leader (usually senior-most doctor/surgeon) and there  are many assistants. The leader sorts out patients (people)  depending upon their severity of injury.   ¢ Each of the patients can be colour coded (a coloured flag  is attached to them). Examples:   1. Immediate help is necessary—Red: Otherwise the  person will die ina few minutes if no treatment is offered,  e.g. obstructed airway, tension pneumothorax.   2. Urgent help is necessary— + These patients may  die in ]—2 hour if no treatment is given. Examples are  cases of massive bleeding and hypovolaemia.   4. Expectant—Blue: Very severe injury and unsalvageable.  An attempt to treat them, may delay treatment to other  patients who are salvageable.    The leaderassesses the patient—flags and moves forward  and it ts his assistant who carries out the necessary  resuscitation. The leader should not resuscitate any  patient. There are others waiting for his expert help.    e Disability and assessment of level of consciousness by  Glasgow Coma Scale.   ¢ Ifany regional trauma centre is well equipped and nearby  one has to transport all the injured patients to hospita.  (scoop and run), where expert help is available.  Meanwhile trauma centre can be alerted about the arriva!  of casualties. If expert help is far away, then one may  have to treat the patients at the accident site (stay and  play). Resuscitation 1s done as per ATLS guidelines.    ¢ During these exercises do not forget to take care of  your own Safety, in burning vehicles, burning or falling  buildings, etc.  PEARLS OF WISDOM    An initial quick evaluation of the patient for anaemia,  level of consciousness, if necessary volume replacement,  application of cervical (C) collar to the neck, etc. are done.    These six initial steps are included under primary survey and  resuscitation in ATLS (advanced trauma life support) are given  below.   ¢ Lift the jaw, introduce airway, good suction of throat,  intubation or even tracheostomy if necessary.    ¢ Rule out tension pneumothorax, multiple fractures of ribs  and haemothorax, surgical emphysema, etc.  ¢ All trauma patients should receive high-flow oxygen.   Fig. 36.41: Management of a polytrauma patient in an intensive  care unit—cervical collar has been applied and patient is being  ventilated    Tension pneumothorax is a clinical diagnosis. Do not  delay the treatment while waiting for chest X-ray.    Immediate and quick assessment of circulation status,  evidence of shock and evidence of internal or external  bleeding should be looked for.   Feeble thready pulse, hypotension indicates volume or  blood loss. Splenic, liver or mesenteric injuries can be  diagnosed by ultrasound. Bleeding into the pleural space  or pericardium needs further tests—CT scan or echo  cardiography, etc.    The immediate goal ts to arrest bleeding rather than  replacing the blood.   DISABILITY: AVPU SYSTEM  e Awake  e Open eyes to Voice  e Open eyes to Painful stimulus  e Unarousable    Disability  Glasgow Coma Score and assessment of pupils can help  immediately to assess the status of the patient.    * Quick examination of all orifices. Examples: Look for  bleeding from the ear, nose, oral cavity, rectum, vagina,  and urethra.   e Nasogastric tube, endotracheal tube, catheter, intravenous  line or central line are the immediate requirements of a  polytrauma patient.    e Liver and pancreatic injury have been discussed in more  detail.    Which one of the followings is a definite indication for  laparotomy in blunt injury abdomen?    . If air bubble like picture is found within the thorax    . How do you rule out a head injury with factors given    The following are true for conservative management!  of liver injury except:    A. Compression of left gastric artery to stop the bleeding  from giant gastric ulcer   B. Compression of hepatic artery to stop the bleeding  during liver resection   C. Compression of hepatic artery and portal vein in front  of foramen of Winslow    Perforation within 4 cm of the ileocaecal junction  following blunt injury is better treated by:    Which of the following is not a feature of retro-  peritoneal duodenal perforation?    The abdomen ts like Pandora’s box. However, a student who  is examining a case of abdomen 1s like an investigating CBI  officer. He has to collect information at every level of  examination, 1.e. history, past history, general examination and  abdominal examination. An attempt has been done here to  highlight the importance of history and clinical examination.  Ten points in the history, if taken and analysed properly may  give a definite clue in majority of cases. After getting this  clue, clinical examination of the mass may become easy.    e Abdomen is divided into nine regions (quadrants) by two  horizontal lines and two vertical lines.   ¢ Upper horizontal line or transpyloric line is midway  between xiphisternum and umbilicus.   ¢ Lower hoizontal line (transtubercular line) is the line  joining iliac crest tubercles of each side, about 5 cm behind  anterior superior iliac spine.   ¢ The vertical lines are drawn on either side through midpoint  between anterior superior iliac spine and symphysis pubis.  Following are the nine regions of the abdomen (Fig. 37.1).  1. Right hypochondrium    e The cystic mass in the abdomen  e Mass in the epigastrium   e Mass in the right hypochondrium  e What is new?/Recent advances    1. Abdominal pain: It is present in most of the cases of  abdominal mass. Abdominal pain can be of the following  types:   A. Dull aching pain: It suggests a solid organ enlargement.  It is a continuous pain felt in the anatomical location of  the swelling. Patients often describe it as a discomfort  rather than pain.    ¢ Liver enlargement: Pain in the right hypochondrium. It  occurs due to stretching of parietal capsule (Glisson’s)  (Fig. 37.2A)    ¢ Renal enlargement: Pain in the back and costal region or  costovertebral pain (Fig. 37.2B)    B. Colicky pain suggests hollow viscus obstruction.  This pain is due to hyperperistalsis. It is severe and  Intermittent (comes and goes). Each attack may last  for 5-10 minutes. The patient bends on himself, holds  the abdomen and puts pressure on the abdomen which  gives some kind of relief. Being visceral type of pain, it  is not very well localised. Following are a few examples:    ¢ Mass in the right iliac fossa (carcinoma caecum or  ileocaecal tuberculosis). Initially there may be a vague  discomfort. However, when partial obstruction occurs,  It results in a colicky abdominal pain which 1s centrally  located and sometimes unbearable.    ¢ Carcinoma pyloric antrum or pyloric stenosis produces  colicky upper abdominal pain with gastric peristalsis.  However, this type of pain is not an unbearable one.    C. Referred pain: Tuberculosis of spine is a common  problem in India. Often patients present with ilopsoas  abscess. Patients can complain of referred pain in the  lower abdomen (Fig. 37.3).    2. Sensation of fullness/early satiety  ¢ Carcinoma of the stomach and pyloric obstruction. Also  hepatoma or large pancreatic tumours can cause  extraluminal compression on the stomach resulting in  sensation of fullness in the abdomen.    ¢ Early satiety is due to loss of receptive relaxation of  stomach due to malignant infiltration of muscle layer.   ¢ Persistent, profuse, projectile and nonbilious vomiting  suggests pyloric stenosis. Chronic duodenal ulcer and  carcinoma stomach are the common causes of pyloric  obstruction (pain is absent/negligible).   ¢ Persistent, profuse, projectile, bilious vomiting—  intestinal obstruction. For example, ileocaecal  tuberculosis, stricture of the small bowel, adhesions (pain  1s severe and colicky).    4. Haematemesis  ¢ Epigastric mass suggests carcinoma stomach  ¢ Splenomegaly may be an indication of portal  hypertension.  5. Bleeding per rectum  ¢ Fresh blood with or without melaena----carcinoma rectum  ¢ Melaena—carcinoma stomach, portal hypertension    6. Loss of appetite and loss of weight  ¢ These are common symptoms of GI malignancies. Please  note that these two symptoms are seen not only in intra-  abdominal malignancies but also in many diseases such    as tuberculosis. However, it should be noted that one of  the earliest signs of carcinoma stomach is loss of  appetite. Severe weight loss is an early and important  feature of carcinoma body of the pancreas.   ¢ Significant weight loss refers to loss of 10 kg or more in  the last 6 months.    ¢ Fresh bleeding per rectum: Carcinoma rectum  ¢ Blood and mucus (bloody slime): Carcinoma rectum  ¢ Alternate constipation and diarrhoea: Carcinoma colon    ¢ Progressive, persistent, pruritic jaundice: Periampullary  carcinoma or carcinoma head of pancreas. However, in  periampullary carcinoma, fluctuation can occur if growth  ulcerates.    ¢ High-grade fever, with chills and rigors: Stone in  common bile duct   ¢ Low-grade fever: Hepatoma, renal cell carcinoma,  lymphoma. Fever is due to some pyrogens released into  circulation or due to tumour necrosis.   ¢ Ina tropical country like India, hepatomas with fever  are often diagnosed as amoebic liver abscess and  mistreated.    The patient is asked to breathe well with mouth open.  Students should spend a few minutes watching the abdomen  carefully.   e Presence of step ladder peristalsis indicates small bowel  obstruction, visible gastric peristalsis indicates pyloric  stenosis and right to left peristalsis indicates colonic  obstruction.   2. Restricted movement of any one region of the abdomen  indicates an inflammatory pathology.    3. Umbilical nodule (Sister Joseph’s) indicates intra-  abdominal malignancy (carcinoma of stomach, colon,  pancreas)    4. Details about the mass such as size, shape, surface, borders,  movement with respiration have to be mentioned 1f mass 1s  visible. If the details about the mass cannot be appreciated  or if mass 1s not clear on inspection, it is better to say  “there is fullness” rather than trying to manipulate the  details about the mass.    5. Inspection of male genitalia: If scrotum 1s empty, it could  be a case of undescended testis!~.    Methods of palpation  Following are the methods of palpation available to the  clinician and done depending upon the merits of the case:    1. Superficial palpation: Gentle superficial palpation of the  abdomen gains confidence of the patient. It can detect  superficial lesion of the abdominal wall suchas lipomatosis,  neurofibromas or fibromas, etc. It can also detect an area  of tenderness, so that clinician is careful while doing deeper  palpation. Superficial palpation is done with the flat of the  hand or fingers.   ¢ Patient should be well-relaxed, with flexion of the knee  for about 45 degrees.   ¢ The patient’s face should be turned to the opposite side  and he is asked to breathe comfortably with open mouth.   ¢ Deep palpation should be started from the quadrant  situated diagonally opposite to the site of pain.   ¢ Palpation should cover not only the 9 quadrants of the  abdomen, but also 2 more quadrants, i.e. the 2 renal  angles and 12th quadrant—external genitalia in  males.   ¢ Deep palpation is carried out with the palmar surface of  the fingers and some degree of angulation depending  upon the depth of palpation.    1. Movement with respiration: This test is done by placing  the fingers (hand) over the lower border of the swelling  and the patient is asked to take a deep breath. Movement  with respiration 1s positive when there 1s “up and down”  movements not anteroposterior movement. Any structure    'A case of mass abdomen diagnosed to be soft tissue sarcoma or lymphoma of the para-aortic node region by U/S proved to be a seminoma in an    undescended testis. Patient said that 6 months back his right testis was removed by a ‘groin’ incision.  “Inspection and palpation of external genitalia is important in males only, NOT TO BE DONE in females.    MOVEMENT WITH RESPIRATION  Liver, spleen and stomach masses move freely with  respiration  Gall bladder mass also moves freely with respiration  because of its proximity to liver  Mass arising from hepatic flexure and splenic flexure of  colon also have some mobility because it is in contact with  liver and spleen  Renal masses exhibit minor degree of movement with  respiration because of indirect attachment to diaphragm   e Liver, stomach, spleen, gall bladder move very well with  respiration.   e Splenic flexure growth, due to contact with the lower  pole of the spleen and hepatic flexure growth due to  contact with liver move with respiration.    . Finger insinuation test: This test has relevance in an upper   e Liver and spleen are under right and left costal margins  respectively. Hence, it is not possible to get the upper  margin or upper border of these organs. An attempt to  invaginate between the costal margin and these masses  is not possible. On the other hand, finger invagination  under the costal margin is possible in a stomach mass.   e An egg-shaped mass or globular mass suggests gall  bladder lesion (Fig. 37.4A).   ¢ A horseshoe shape may indicate a horseshoe kidney  (Fig. 37.4B) with pathology, e.g. hydronephrosis.    4. Consistency  ¢ Hardness is a feature of malignant lump. Thus, hepatoma,  carcinoma stomach, pancreatic carcinoma present as hard    lump. However, it should be remembered that often the  malignant lump Is firm and not hard.   e Temporary contraction of a stomach (visible gastric  peristalsis) should not be confused as a mass.    ¢ Upper border cannot be made out in liver, splenic and  renal swellings.   ¢ Lower border ts not appreciated in pelvic masses, e.g.  uterine fibroid, ovarian cyst (pelvic).    e An intra-abdominal mass can be mobile if it has loose  attachments or if it 1s not within the bony cage. Thus,  liver, spleen, uterine mass are not mobile because of their  location within bony cage.    ¢ Carcinoma pyloric antrum can exhibit movements in  different positions—left lateral, right lateral or even in  the sitting position.    A. Ovarian cyst is a freely mobile swelling which can be    B. Mesenteric cyst moves at right angles to the direction of mobility, a cystadenoma of the pancreas because of the   C. Pseudopancreatic cyst may have a minimal side-to-side 37.6E). Any big mass abutting the undersurface of the  mobility (Fig. 37.6C). diaphragm also moves with respiration.    D. Carcinoma transverse colon has vertical mobility unless .  it is advanced (Fig. 37.6D). down, it can be held back and can be pushed back to the    B. Nose blowing test or straining test  ¢ This test can be done by asking the patient to blow  through the nose with mouth closed. The lateral  abdominal muscles are more contracted with this test.  ¢ It should be remembered that a swelling or the mass  which moves with respiration is obviously an intra-  abdominal mass.    C. Knee-elbow test  ¢ This test differentiates an intraperitoneal mass from  retroperitoneal mass. It 1s more useful when there 1s  a mass in the centre of the abdomen—more so in  the upper abdomen. To give a few examples,  intraperitoneal cyst or intraperitoneal mass falls  forward. On the other hand, pancreatic mass or a  lymph node mass will not fall forward. The test has  significance only in ‘selected’ cases.  ¢ However, knee-elbow test helps to differentiate  expansile pulsation from transmitted pulsation.  ¢ Examples: A pseudocyst of pancreas will give  transmitted pulsations because it overlies aorta. In the  knee elbow position, pulsation disappears as it gets  separated from the aorta. On the other hand,  aneurysms exhibit expansile pulsations.  Intraperitoneal mass with pulsations over it 1s likely  to be hepatoma. Retroperitoneal mass with pulsations  over it 1S pancreatic mass.    1. Bimanual palpation: Grossly enlarged swellings may  be bimanually palpable such as liver, spleen, kidney.    2. Ballottability: ‘Ballot’ means to toss about. To ballot,  the swelling should be bimanually palpable and there  should be a gap or space between hands which are kept  anterior and posterior to the mass. Typically, renal  swellings are ballottable. This test is done when the  patient is in supine position, by keeping one hand  anteriorly in the lumbar region over the swelling and  the other hand posteriorly in the renal angle. A gentle  push is given from behind and the swelling touches the  hand which is placed anteriorly and it goes back.  Ballottability is because of perirenal pad of fat and  due to ‘pedicle’.    1. To demonstrate mild ascites, the patient is put in a knee-  elbow position and percussion is done around umbilicus.  It gives a dull note if minimal fluid is present (normally  area around the umbilicus is resonant).    ¢ Significant or moderate fluid in the abdomen is  demonstrated by percussion of the centre and flanks  of the abdomen in the lying down position and in the  left or right lateral position.    ¢ In the supine position, flanks give a dull note due tc  fluid. However, in the lateral position, fluid shifts  down and coils of bowel float up.   2. Liver dullness is elicited in the 5th intercostal space and  the dullness is continuous with the mass, if it is arising  from the liver.   3. Splenic dullness is elicited in the 9th intercostal space in  the left midaxillary line.   ¢ Retroperitoneal masses may give resonant note  because of intestines anterior to it. However, when  they attain large size, e.g. sarcomas, they push the  bowel to one side and hence, they are dull to percuss.   e Stomach mass may give impaired resonant note  because of solid growth and due to the presence of  air in the stomach.   ¢ Renal angle percussion: In cases of enlargement of  kidney, there will be a band of resonance anteriorly  due to the colon but posteriorly it gives a dull note.   ¢ Hydatid thrill: It isdemonstrated by placing 3 fingers  over the swelling and percussing the middle finger.  Due to the fluid in the cyst, the fluid thrill (after-thrill)  is felt by the other two fingers. This clinical sign is  rarely demonstrable.    Loud noisy sounds (borborygmi) with or without peristalsis  may indicate subacute obstruction. Such patients may be  having ileocaecal tuberculosis or carcinoma caecum. This  should be done at right iliac fossa to listen bowel sounds.    . Auscultation over the liver mass may reveal a bruit as ina    obstruction either due to carcinoma or due to chronic  duodenal ulcer.    assess lower border of the stomach or greater curvature of  the stomach.    Rectal examination  Should be done in a case of intra-abdominal mass.    1. It can detect a carcinoma or a growth in the rectum in a  case of secondaries in the liver.    Should be done to rule out carcinoma cervix or to detect lymph  nodes in the pouch of Douglas.    This should be done in cases of pelvic masses. One hand (left)  is placed over the mass in the hypogastrium and right index  finger or fingers inserted in the vagina or rectum in virgin  females and the left hand is pressed downwards and backwards  above the pubic symphysis. By this manoeuvre, details of the  pelvic mass, solid or cystic, uterine or ovarian, free or fixed  can be made out.    In cases of abdominal masses arising from lymph nodes, a  thorough search of the body should be done to rule out other  group of lymph nodes such as axillary, iliac, inguinal, neck  nodes (lymphoma).   internal jugular vein and subclavian vein on the left side  This explains the significance of enlargement o  Virchow’s node. In 20% of cases, thoracic duct 1s singh  and 10—15% of cases, it is double.    ¢ Significance of right supraclavicular node: Th:  lymphatics from the right mediastinal lymph trunk, anc  from the posterior right thoracic wall which form th  right upper lymph trunk drain into the commencemen  of the right brachiocephalic vein.    Systemic examination should include respiratory system anc  cardiovascular system. Evidence of tuberculosis of the ches  gives a clue about the mass in the abdomen, which may be ¢  tubercular mass.    I. Intestines  1. Appendicular mass  . Appendicular abscess  . [leocaecal tuberculosis  . Carcinoma caecum  . Amoeboma  . Intussusception  . Actinomycosis  II. Lymph nodes  1. Acute lymphadenitis  2. Lymphoma  3. Secondaries  III. Retroperitoneal structures  1. Sarcoma  2. Aneurysm  3. Iliopsoas abscess  4. Chondrosarcoma  IV. In females  1. Ovarian cyst  2. Fibroid  3. Tubo-ovarian mass    Differential) diagnosis of mass in the  right iliac fossa (Figs 37.8 and 37.9)    I. Parietal swelling: They are extra-abdominal. On head or  leg raising test, they become more prominent. They are  uncommon swellings.   A. Parietal wall abscess: It is a pyogenic abscess which  can occur in a haematoma, or a pyaemic abscess which  can occur as a part of pyaemia as in diabetic patients.  Such abscesses are very tender, with warm surface and  are associated with fever, chills and rigors.   B. Desmoid tumour: It is an unencapsulated fibroma  occurring in the abdominal wall.   ¢ Occurs in multiparous females. Repeated stretching of  abdominal layers (due to pregnancy) is supposed to  initiate formation of tumour.   ¢ It can also occur following abdominal wall injury  including laparotomy.   ¢ It has no capsule. Hence, it should be treated with wide  excision.   e After wide excision, the abdominal wall has to be  reconstructed by using mesh.    A. Arising from structures normally present in the  right iliac fossa    because an attempt to remove the appendix may  result in faecal fistula. 6-8 weeks later, an elective  appendicectomy can be done.   2. Appendicular abscess: It will be a very tender, firm,  fixed mass. Such patients will have fever with chills  and rigors.   3. lleocaecal tuberculosis (Fig. 37.11): Hyperplastic  variety of tuberculosis forms a chronic cicatrising  granulomatous reaction involving terminal ileum,  caecum and part of ascending colon resulting in a  mass in right iliac fossa. It is a chronic, nontender,  firm, nodular mass, may have mobility, situated  slightly (lumbar) on the higher side. Features of  tuberculosis are usually present. It is treated by  limited resection followed by ileocolic anastomosis.   ¢ Hard, irregular mass in right iliac fossa with fixity or  restricted mobility is a usual feature. Psoas spasm  indicates infiltration into psoas muscle. It 1s treated by  right radical hemicolectomy.    . Amoeboma: Can be acute or chronic. It follows an attack    of amoebic typhlitis (inflammation of the caecum).  Amoeboma Is tender and soft to firm. It is not common to  find amoebomas nowadays because of effective treatment  of amoebiasis with metronidazole, tinidazole, etc.    rise to a mass in the right iliac fossa which is tender and  soft to firm. When acute intussusception occurs in children,  it 1s described as idiopathic intussusception. Chronic  intussusception may disappear spontaneously.    7. Actinomycosis: This is a rare mass in the right iliac fossa    which usually develops 2—3 months after appendicectomy.  A woody hard, indurated tender mass with multiple sinuses  is characteristic of this condition. Sinuses discharge sulphur  granules which can trickle down. Unlike tuberculosis,  narrowing of lumen of the gut and lymphnode enlargement  does not occur.    Acute mesenteric lymphadenitis 1s common tn children.  It produces tender, nodular and firm mass tn right iliac  fossa. The child usually has fever. Acute lymphadenitis  can also involve external iliac nodes as in filariasis.  Lymphoma involving external iliac nodes, nodular, firm  to hard mass with involvement of other nodes, liver,  spleen, etc.   Secondaries in lymph nodes (external iliac) from  carcinoma ovary, cervix, etc. Nodes are hard and fixity  is a feature.   Huge, nodular, fixed lump involving lumbar, umbilical  and right iliac fossa. Recent increase in size draws the  attention of the patient.   Later, obstruction of inferior vena cava results in oedema  of legs.   Pressure on the ureter can give rise to hydronephrosis.  Liposarcoma 1s the commonest and may arise from pre-  existing lipoma.    Chemotherapy is also helpful, when it is not possible tc  remove the entire mass.   Debulking even if it is an advanced case is recom-  mended.    10. Aneurysm: Iliac artery aneurysm Is rare and occurs in  old-aged patients. It produces a soft, pulsatile swelling in  the right iliac fossa. Bruit or thrill is usually present.    It is the result of tuberculosis of thoracolumbar spine. It  should be suspected when a young patient complains  of pain in the back referred to abdominal wall.   Spine movements are limited.  Gibbus is present. | a  Initially, it forms paravertebral  abscess and later it gravitates down |  beneath the medial arcuate ligament  and forms psoas abscess. Psoas | \  abscess burrows into the thigh under | |  inguinal ligament and forms iliopsoas  abscess. |  Fluctuation is present on both sides |    13. Ovarian cyst: To start with, the cyst develops in the pelvis  and gives rise to discomfort in the lower abdomen. As the  cyst grows, it comes out of the pelvis and forms a mass in  right iliac fossa. It has smooth surface, round borders, is cystic,  freely mobile and can be pushed back into the pelvis.  Sometimes, the cyst can attain huge size. Such freely mobile  ovarian cysts have a long pedicle. Per vaginal examination  gives the clue to the diagnosis.    14. Fibroid of the uterus: It presents as a firm to hard nodular  mass in the suprapubic region and in the right iliac fossa.  15. Tubo-ovarian mass    1. Unascended kidney: It can be either in the pelvis or in the  iliac fossa. Such kidney is usually not very well-developed.  It presents as a lobular mass.   2. Normal mobile kidney: It can be felt in lumbar region,  iliac fossa and can be pushed back into the loin.   3. Undescended testis: It is palpable in right iliac fossa enly  when it 1s involved by Seminoma. It is an intra-abdominal  testis and is hard, irregular, fixed mass. Absent testis in the  scrotum clinches the diagnosis. Patient may have palpable  para-aortic nodes, supraclavicular nodes, etc.    a. Metastasis or secondaries is one of the common lymph  node masses in the abdomen. Mass can be due to para-  aortic nodes from testicular tumour, melanoma,  carcinoma of ovary, carcinoma of penis, carcinoma of  rectum, colon, stomach in late cases.  ¢ A para-aortic lymph node mass has the following    b. Lymphoma: The mass is enlarged para-aortic group of  lymph nodes. It has all features of nodes mentioned above.  Presence of lymph nodes tn the neck along with palpable  liver and spleen clinches the diagnosis.   ¢ Rapidly growing, enlarging mass in the abdomen of short  duration.   ¢ It is firm, hard, nodular, fixed, does not fall forward and  has intestinal coils anterior to It.   ¢ Large sarcomas can cause compression on inferior vena  cava or on the ureter. Therefore, pedal oedema and  hydronephrosis can occur.   ¢ Radical surgery should be attempted and it is the only  hope of cure. (Many cases may require debulking and  follow-up with radiotherapy and chemotherapy.)    ¢ Cystadenocarcinoma of pancreas can attain a huge size.  Otherwise, it 1s uncommon to get a large nodular pan-  creatic mass. However, carcinoma pancreas presenting  as a palpable nodular mass indicates nonresectability.  Presence of pulsations over the mass (transmitted)  clinches the diagnosis.    ¢ Severe backache, loss of weight, recent development of  diabetes suggest pancreatic pathology.    e Jaundice does not occur unless and until liver secondaries  develop.   e It may have vertical mobility and being intra-abdominal,  it falls forward.   ¢ Caecum may be distended. Right to left peristalsis may  be visible.    ¢ The mass can be rolled up omentum, with lymph nodes  and coils of intestines which are matted.   ¢ History of evening rise in temperature, loss of weight,  loss of appetite, emaciation and improper digestion gives  the clue to the diagnosis.    Intra-abdominal cystic swellings are interesting swellings.  They occur in young children, adults, middle-aged persons.  There are many cases of cystic swellings which have given a  surprise at laparotomy (notoriously so in females). In children  cysts have confused many competent paediatricians!! Being  intra-abdominal cysts, it is not possible to elicit fluctuation  and very often they are firm due to increased tension. The  details of important cystic swellings are given below.    Tensely cystic upper abdominal mass may feel firm and  tender, and does not move with respiration (see page 607   for details). Getting above the swelling 1s possible.  Transmitted pulsations of the aorta can be felt over the mass  which disappears on knee-elbow position. History of acute  pancreatitis or blunt injury abdomen gives the clue to the  diagnosis (Fig. 37.22).    This swelling is of long duration, 1s symptomless or with  dull pain in the upper abdomen. The cyst is spherical with  smooth surface, rounded borders and feels firm. Since it is  a mass arising from liver, it moves with respiration and  getting above the swelling 1s not possible. Classical hydatid  thrill, mentioned in the books, is rarely appreciated. Simple  cyst of the liver can also present as a cystic mass  (Fig. 37.24).    These are congenital cysts, enterogenous or chylolymphatic,  manifests in young children or during adolescence.  Typically, the cyst is located in the umbilical region which  moves at right angles to the direction of mesentery.   A.Chylolymphatic cyst is a lymphatic cyst arising from  mesentery of ileum. It 1s a thin-walled cyst with clear fluid  or chyle. It has a separate blood supply. Hence, enucleation  is the treatment without sacrificing the bowel.    B. Enterogenous cyst is a duplication cyst from the intestine  or due to diverticulum of the mesenteric border of the  intestine. It 1s thick walled and contains mucus. This cyst  is treated by excision of cyst with bowel segment because  both share the same blood supply.    Complications  ¢ Torsion of the cyst resulting in acute abdominal pain.  ¢ Rupture of the cyst due to trauma.  ¢ Haemorrhage into the cyst.   MESENTERIC CYST—TYPES  e Chylolymphatic cyst  e Enterogenous cyst  e Urogenital remnant  e Teratomatous dermoid cyst    Large hydronephrosis can attain a huge size without  producing any symptoms. Bulk of the swelling is confined  to one side of abdomen, with prominent bulge in the loin.  It is difficult to elicit fluctuation in a tensely cystic intra-  abdominal mass. Bimanual palpation and ballotability give  the clue to the diagnosis. One of the large cysts of polycystic  kidney can present as a large renal cyst.    It is a freely mobile, firm or soft mass in any quadrant of  the abdomen. Such ovarian cysts, once they come out of    pelvis will have free mobility. On pushing the mass  upwards there will be traction on the pedicle, which may  result in pain (Fig. 37.28). In any female patient who  presents with lower abdominal mass, ovarian mass has to  be considered first, and only then consider other  possibilities.    Retroperitoneal cyst is one of the commonest of lymphatic  cysts, which grows slowly to attain large size. Typically it  is painless, seen in young patients and Is tensely cystic.  The bowel loop may be felt over the mass (retroperitoneal  mass), or bowel loops may be pushed to the side.    This consists of ascitic fluid loculated by many loops of  intestine along with omentum. Loss of weight, fever,  anorexia, emaciation are the other features.    ¢ Majority of cases are due to atherosclerosis and most of  the aortic aneurysms are infrarenal. Hence, they present    with a swelling in the umbilical region or epigastric  region and are associated with backache (Key Box 37.5).    ¢ Often they contain clotted blood. Hence, they feel firm,  not compressible, fixed and tender.    ¢ Characteristic feature of an abdominal aortic aneurysm  is expansile pulsation. This can be appreciated by  palpating the swelling gently all around. In the knee-  elbow position, the pulsations do not disappear.  (Transmitted pulsations disappear in the knee-elbow  position.)   ¢ The femoral pulses may be normal unless there is  thrombosis or rupture of the aneurysm, giving rise to  features of acute ischaemia.    e Pressure effects such as venous oedema due to pressure  on the inferior vena cava or erosion of vertebrae may be  found.   ¢ Ultrasound to confirm the aneurysm and also to rule out  Suprarenal aneurysm.   ¢ It is treated by repair of aneurysm, by incising the    aneurysm and suturing a dacron graft end to end, inside  the aneurysmal sac.    ¢ Omental cyst: This is usually a lymphatic cyst which  occurs in children and can attain a huge size. Sudden  enlargement indicates haemorrhage. Excision Is easy.    ¢ Large mucocoele of the gall bladder can present as a  tense cystic, slightly tender mass in the upper abdomen.    One should not forget distended bladder in the lower  abdomen as a cause of cystic swelling.   Mass in the epigastrium is one of the common long cases kept  in the examination. Students should consider mass arising from  liver and stomach first. Other possibilities must be considered  later because common cases are common.   A female child, aged 6 years, was examined by a  paediatrician for generalised abdominal distension. All  the investigations were normal. The child was put on  antituberculous treatment, as the treating paediatrician  diagnosed this case as tuberculous ascites. Child was  brought back after 9 months with no improvement and  having abdominal pain since 2 days due to sudden increase  in the size of the swelling. A paediatric surgeon was  consulted, who palpated the abdomen and said she does  not have ascites but has a cyst and that the wall of the thin  cyst can be felt. At exploration, a large cyst arising from  the omentum and occupying all the 9 quadrants of the  abdomen, was excised. Histopathological report was  lymphatic cyst (Fig. 37.29).   ¢ Lipoma, neurofibroma or desmoid tumour arising from  the rectus sheath can present as a mass in the epigastrium.   ¢ Also note epigastric hernia occurs in this region. It is a  hernia, not a mass.    Any hard subcutaneous swelling in the abdominal wall  of recent origin can be a metastasis.    A. Hepatoma (Fig. 37.30): Liver is enlarged, hard, irregular, malignancies as a hard, nodular mass (Fig. 37.35).  and nontender. However, rapidly growing hepatomas are Classically it moves with respiration.  tender, firm and even a bruit is heard over the swelling. ¢ Rarely, omental cyst can present as a tensely cystic mass  Rapid deterioration of health in a cirrhotic patient is in the epigastrium.  usually due to the development of a hepatoma. Ill, RETROPERITONEAL MASS   B. Secondaries in the liver (Fig. 37.31): Usually both lobes A. Pseudopancreatic cyst (Fig. 37.36): It forms a tense  are enlarged, have nodular surface without a bruit. cystic mass, felt as firm mass in the epigastrium. Its  Jaundice is alate feature in secondaries of the liver. The upper border can be made out. It does not usually move  peels eee be obvious as a colonic mass, a stomach with respiration. It has smooth surface and round  mds SO (tee Oa i iamiont, etvei(see pages): borders. History of acute pancreatitis or blunt injury   C. Hydatid cyst (Fig. 37.32): It is a benign swelling. abdomen is usually present. Pulsations over the mass  History of contact with a dog is usually present. (transmitted) suggest that it is a mass close to the aorta.  Epigastric swelling is due to enlarged liver which is In such a case, it is a pseudocyst. Gurgle heard  smooth or irregular, nontender with rounded borders. anteriorly suggests distended stomach.  Classical hydatid fremitus and thrill are rarely elicited. B. Cystadenoma: Cystadenomas of pancreas are benign  General health of the patient is usually good. and can attain huge sizes. It can present as a mass in the   C. Carcinoma body of pancreas can present as a mass  in the lower part of epigastrium or upper umbilical  region. The mass is hard, irregular, fixed and does not  move with respiration. Presence of severe backache  and loss of weight are important features.    D. Simple cyst (Fig. 37.33): It is not a clinical diagnosis  but is mentioned here only for discussion. It 1s a serous  cyst. Single big cyst can also be a part of polycystic  disease of the liver.    * For all practical purposes, the only mass arising from D. Abdominal aortic aneurysm (AAA) (Fig. 37.37): An  the stomach in the epigastrium is carcinoma stomach. It elderly patient, usually a hypertensive presents with  is hard, irregular and moves with respiration. Usually features of abdominal pain, swelling or features of  the patient is a male with loss of appetite and weight. ischaemia of the lower limb. On examination, tender  Vomiting is a feature. If there is a growth in the pyloric swelling in the epigastrium with a characteristic  antrum, visible gastric peristalsis can be seen in the expansile pulsation is present. Knee-elbow test will help  epigastrium. (Students are hereby requested not to offer differentiate it from transmitted pulsations. Presence of  lymphoma of the stomach or GIST of the stomach as a bruit and weak or absent lower limb pulses (due to  a Clinical diagnosis unless asked for by the examiner, thrombus) also helps in establishing the diagnosis.  for a differential diagnosis.) E. Lymph node mass (Fig. 37.35)   Fig. 37.30: Hepatoma Fig. 37.31: Secondaries in the liver Fig. 37.32: Hydatid cyst Fig. 37.33: Simple cyst    I. Parietal: On head raising test, the lump becomes more  prominent.  A. Lipoma, neurofibroma: They can be part of multiple  lipomatosis or multiple neurofibromatosis. If pain and  pigmentation are present, it is neurofibroma.  B. Ahard nodule in the parietal wall can be due to  a. Secondary deposit in the skin/subcutaneous tissue  specially when skin is infiltrated and ulcerated. Common  primaries are malignant melanoma, bronchogenic  carcinoma, hepatoma.   c. Cold abscess: Spine tenderness with or without history  of tuberculosis gives the clue to the diagnosis.    . Intra-abdominal swellings: On head raising test, the lump  becomes less prominent.   ¢ Rare umbilication sign, evidence of primary, emaciated  patient, poor health, loss of appetite and weight are other  features.   ANATOMIC FEATURES OF LIVER MASS  e Location: Hypochondrium (right and left) and epigastrium  e Moves with respiration  e Finger—insinuation between costal margin and mass is not  possible  e No intrinsic mobility and dullness  e Dull note over the liver which will continue with the mass   TENDER LIVER MASS  e Hepatoma  e Amoebic liver abscess  e Suppurative pylephlebitis  e Congestive cardiac failure  e Infected hydatid cyst   Fig. 37.39: Differential diagnosis of mass in the right hypochondrium: (1) Smooth hepatomegaly in lymphoma or due to medical  Causes, (2) secondaries in the liver—hard and nodular, (3) hepatoma—irregular, hard or firm, (4) polycystic disease of the liver—firm  and nodular with round borders, (5) with splenomegaly—could be portal hypertension, (6) hepatomegaly, splenomegaly, para-aortic  lymph nodes and iliac nodes—Hodgkin’s lymphoma, (7) carcinoma ascending colon—hard irregular mass, (8) palpable gall bladder—  smooth, round borders, and (9) renal mass   due to haemorrhage in a cyst. ¢ Other features of liver cell failure such as gynaecomastia,    ¢ Smooth or nodular surface ¢ Liver 1s palpable, one or two finger-breadths, firm or  ¢ Round borders, nontender hard, smooth or irregular, nontender.  ¢ General condition of the patient is good ¢ Splenomegaly and lymphadenopathy will help in the    ¢ It 1s a tongue-shaped projection from the inferior border  of liver. It is on the right side, can be mistaken for gall  bladder.    Causes of gall bladder enlargement  ¢ Back pressure: Distal obstruction periampullary  carcinoma. Such gall bladder 1s firm, smooth and asso-  ciated with jaundice.  ¢ Carcinoma gall bladder: Hard, irregular, fixed  ¢ Acute cholecystitis: Tender, vague, well-defined mass.  ¢ Mucocoele: Nontender, palpable gall bladder without  jaundice  ¢ Empyema: Very tender, gall bladder mass  3. Colonic mass  A. Carcinoma hepatic flexure  ¢ Firm to hard irregular mass  ¢ Restricted mobility  ¢ Moves with respiration because of its contact with liver    B. Large ileocaecal tuberculosis with pulled up caecum  may also be palpable. Such masses may be bimanually  palpable but not ballotable.    4. Renal mass  ¢ Importantly renal mass 1s palpable mainly in the lumbar  region, loin and in the right hypochondrium.  ¢ Carcinoma kidney 1s hard and irregular  ¢ Upper border is usually not palpable—it is under cover  of the | 2th rib.  ¢ Hydronephrotic kidney will be firm and smooth    Offer renal mass as diagnosis. When asked for  differential diagnosis, mention suprarenal mass.    e Mass in the right hypochondrium has been added.  e New simple diagrams have been added.    . The most diagnostic sign of a renal mass is: 9. Following appendicectomy, after 2 months, if wood  A. Moves with respiration indurated mass develops in the right iliac fossa wit  B. Upper pole cannot be felt sinuses, what is the diagnosis?   . Which of the following masses does not move with C. It is a sausage-shaped mass  respiration? D.In the right iliac fossa caecum gurgles  A. Kidney  B. Hepatic flexure 11. Which of the following masses does not have cross:  C. Tail of the pancreas fluctuation?    Specific gravity: \t varies from 1.005 to 1.040 according to  the state of hydration of the patient. In chronic renal failure,  the concentrating ability of the kidneys are lost and the specific  gravity remains fixed at 1.010.    PH: The urinary pH normally ranges from 4.5 to 8. It varies  depending on serum pH. Urine pH influences the type of stone  formed, e.g. alkaline urine—infection by urea splitting  organisms, infection stones are formed and in acidic urine,  uric acid and cystine stones occur.    Proteinuria: \t 1s defined as excretion of protein > 150 mg/  day. Protein, especially albumin, appears in urine following  exercise, glomerulonephritis and nephrotic syndrome.    Sugar: It appears in urine in diabetes mellitus. Transient  postprandial glycosuria may be seen when serum glucose  levels exceed the renal threshold.    Blood: This is seen as presence of red blood cells (RBCs) in  urine. Presence of more than 3 RBCs/high power field is  considered significant. Haematuria, haemoglobinuria and  myoglobinuria give rise to red colour in urine.    The causes of haematuria may be glomerular or  nonglomerular. Glomerular causes are nephritis, analgesic  nephropathy, IgA nephropathy and connective tissue disorders.  Nonglomerular causes are stones or tumours involving the    urinary tract (e.g. renal cell carcinoma, transitional cell  carcinoma). More details are given in Chapter 43, page 1002.    Ketones: Are seen in urine in diabetic ketoacidosis, starvation  and pregnancy.    White blood cells (WBCs): Presence of WBCs in urine 1s called  pyuria (>5 cells/hpf). The causes are urinary tract infection,  stones, glomerulonephritis, foreign bodies, tuberculosis (sterile  pyuria) and malignancies (sterile pyuria).    Casts: Tamm-Horsfall protein is a mucoprotein from renal  tubular cells. It forms nucleus of all casts. It entraps RBC/  WBC/epithelial cells to form respective casts. Hyaline casts  are without cells and are normal.    Crystals: Different types of crystals are formed in acidic and  alkaline urine and act as precursors for stone formation.  Calcium phosphate and struvite crystals form in alkaline urine.  Calcium oxalate, uric acid and cystine crystals form in acidic  urine.    Urine cytology refers to identification of malignant cells under  the microscope. If positive, it indicates transitional cell  carcinoma of bladder. Cytology is not useful in the detection  of other types of carcinoma such as squamous cell or adeno-  carcinoma.   These are indicated in metabolic evaluation of stone disease  (identifies various abnormalities of electrolyte homeostasis)  and in the evaluation of recurrent pyelonephritis in children  and diabetic nephropathy (degree of proteinuria).    ¢ Prostatic surface antigen (PSA): A glycoprotein, it 1s  elevated in prostatic diseases. Its estimation is used for  screening of carcinoma prostate but is not specific for this  condition. Normal levels 0-4 ng/ml. Carcinoma should be  suspected if levels exceed 4 ng/ml (see page 977).  Testicular tumour markers   Lactate dehydrogenase (LDH): Also elevated in embryo-  nal carcinoma and seminoma. It indicates bulk of the disease  or tumour burden. It 1s helpful in diagnosis, staging, follow  up during/after treatment and prognostication.   Sex hormones such as follicular stimulating hormone  (FSH), testosterone and prolactin may also be measured.   e A fat free, low residue diet, dimol 2 tablets, 3 times daily  for 2—3 days prior to the X-ray is required.   e Stones appear as a white shadow (images are given In renal  stones, see page 936, 937)    Today renal imaging (Fig. 38.1) 1s the most important part of  investigating a case of urinary tract. There are many  investigations, important ones being given below:    INTRAVENOUS PYELOGRAPHY (IVP) AND  INTRAVENOUS UROGRAM (IVU) (Figs 38.1 to 38.5)    2. To detect any pathology of kidneys, ureters and bladder  3. To study any anatomical variations of the renal system.    ¢ A fat-free, nonresidual diet is given for 2 to 3 days prior to  the procedure to avoid intestinal gas shadows.    ¢ Dimol 2 tablets, 3 times daily for 2—3 days prior to the  procedure to expel the gas.    ¢ The patient should not take oral fluids 6 hours before the  procedure.    ¢ Radiological contrast dye: 45% sodium diatrizoate, 20-40 ml  is injected through median cubital vein.    |. Normal renal function is a prerequisite for IVU. Serum  creatinine Is reliable, not urea because of variations in urea  levels depending on hydration. Normal value of serum  creatinine 1s 0.5 to 2 mg%.    2. Plain X-ray kidney ureter bladder (KUB) region to look  for a renal stone—90% of the renal stones are radio-opaque  (only 10% of gall stones are radiopaque).    ¢ To distinguish between renal stones and gall stones on  plain abdominal radiograph, in case of doubt, take lateral  film, stones anterior to the vertebral column are gall  stones and posterior to it are renal stones.   3. If bronchospasm occurs, 1n addition to inhale broncho-  dilator, hydrocortisone 100 mg and an antihistaminic should  be administered IV.   4. In cases of urticaria and skin rashes, an antihistaminic must  be given.    Radiography  |. Early films taken after 2 or 5 minutes, demonstrate the  kidney outline (nephrogram).    2. 5 minutes later, pelvicalyceal system 1s visualised (Fig. 38.1).  3. 15-20 minutes later, ureter, bladder can be visualised (Fig. 38.2).    4. Post-voiding picture 1s taken to demonstrate any residual  contrast in the urinary bladder.   1. Idiosyncrasy to iodine: Test dose should be given before  hand.    3. Multiple myeloma: The dye precipitates myeloma proteins,  blocks the ureter and kidney, and causes anuria.    4. Hyperuricaemia: Uricacidcrystals deposit inthe renal tubules.  5. Sickle cell anaemia: Precipitates sickle cell crisis.  6. Dehydration.    |. Diagnosis of congenital abnormalities such as polycystic  kidney, horseshoe kidney, single kidney, duplication of  kidneys and ureters.    4. Diagnosis of renal, ureteric stones and bladder stones.  5. To diagnose renal tuberculosis, tumours.    In ureteral injuries, when delayed contrast images are not  possible because of haemodynamic instability, an intra-  Operative one-shot (2 mg/kg IV contrast material given  10 min before flat plate abdominal X-ray) 1V pyelogram (IVP)  is recommended in patients with hypotension or a history of  significant deceleration, despite absence of gross haematuria.   2. To selectively collect the urine sample trom renal pelvis,  e.g. renal tuberculosis.    ¢ Ureteric orifices are identified and cannulated by a flexible,  catheter which 1s introduced up to the pelvis of kidney and    the dye 1s injected. X-rays are taken at 5 minutes, 15 minutes  and 30 minutes.   3. Since the dye is injected directly into the pelvis, pelvi-    calyceal system can be identified better which helps in the  diagnosis of early transitional cell carcinoma of kidney.    |. It is an invasive procedure and hence, urinary tract infection  can occur. Prophylactic antibiotics are given before the  procedure.   2. Rarely chances of perforation of the bladder or perforation  of the ureters may occur.    The technique used now is digital subtraction angiography  (DSA). There are two methods in which the test can be  performed.   1. Retrograde arteriography using Seldinger technique.  Selective renal angiography can be done by using a catheter  over a guide wire passed into renal artery.   2. Translumbar aortography is done wherein aorta Is  punctured with a needle from behind, above the renal  arteries at the level of Ist lumbar vertebra.    Dose  For aortography 30 ml of contrast (hypaque) and for selective    1. To demonstrate pathological anatomy of the renal artery  when renal artery stenosis or aneurysm Is suspected.   COMPLICATIONS  1. Tubular necrosis of the kidney  2. Paraplegia due to spasm of spinal arteries  3. Haematoma  4. Thromboembolism    2. In renal cell carcinoma, tumour vascularity and extension  of the tumour into the renal vein can be diagnosed during  the venous phase.   3. Bleeding from the kidney due to trauma, post-percutaneous  nephrolithotomy (PCNL) bleeding or arteriovenous  malformation.   4. Therapeutic application:  ¢ Transluminal angioplasty can be done by inflating the   balloon in cases of renal artery stenosis.  ¢ Embolisation of bleeding vessels, aneurysms (Fig. 38.7).    In this procedure, the dye is injected into the urinary bladder  via an indwelling catheter and X-rays are taken when patient  passes urine.    1. In children, to demonstrate vesicoureteric reflux  2. Posterior urethral valve    A catheter is passed into the urinary bladder in a child and the  dye is injected. The catheter is removed and the child is  screened for vesicoureteric reflux during voiding of urine  (Fig. 38.8).    Filling phase, full bladder, voiding and postvoiding phase films  are taken.    Direct or indirect radionuclide cystography wherein isotopes  are used. It can pick up minute reflux also.    Due to the invasive nature of the procedure, urinary tract  infection can occur. Hence, prophylactic antibiotics should  be used.    In the diagnosis of urethral stricture, to know the length of  stricture, proximal dilatation or diverticulum, urethrography  is used (Fig. 38.9).    Barium and medium containing oil such as lipiodol should  not be used because if there 1s a urethral mucosal tear or breach,    ¢ This is anoninvasive investigation, using ultrasonic waves  (sound waves with frequency > 20,000 Hz). These waves  cannot be heard by the human ear but are reflected or  absorbed by tissues to various degrees which helps to  diagnose different conditions. Ultrasound can be used  through different approaches:    TRANSRECTAL ULTRASONOGRAPHY IN  CARCINOMA PROSTATE  e Disruption of the architecture of echoes  e Invasion of capsule  e Biopsy—ultrasonography guided    A. Fluid can be differentiated from solid tissue. Hence, cystic  swellings can be made out.    B. Stones can be diagnosed. Stones appear as hyperechoic  lesions and postacoustic shadowing.    C. In an enlarged kidney thickness of cortex, disruption of  the architecture of echoes can be made out as in  hydronephrosis.    D. Residual urine in the bladder can be found out, which  may be an indication of enlarged prostate. The volume of  the prostate can be measured.    Ultrasonography has become the investigation of choice  to diagnose foetal hydronephrosis due to various reasons. This  has got dual advantages. Firstly, the management of the disease  causing hydronephrosis can be planned at the early stage  thereby preventing damage to the kidney and secondly intra-  uterine interventions are also possible if the need arises.    ¢ CT can be done with or without contrast (Fig. 38.11).   ¢ CT without contrast (plain CT) is the investigation of choice  for evaluation of renal/ureteric colic.   ¢ CT scan can visualise most urinary tract calculi, even  radiolucent stones not seen on plain X-ray KUB or IVU.    ¢ Contrast CT gives information about the function also—  similar to IVU. However, unlike [VU, which gives  information only about the renal parenchyma and collecting  system, CT can provide valuable information about  perinephric events (e.g. urinoma, abscess, lymph nodes  compressing ureters causing hydronephrosis).   ¢ CT angiography is replacing conventional angiography for  diagnostic evaluation of renal vascular anatomy.   e It is more useful than arteriography to assess and display  the images of the body at selected levels.   ¢ Itis useful in the diagnosis of kidney tumour and its extent,  spread and infiltration.   Fig. 38.11: CT scan showing normal kidneys: It gives information  about function of kidney. Now more commonly used than IVU    ¢ To stage cancer of prostate, bladder, kidney, testicular  tumours and renal trauma (Figs 38.12 and 38.13).    Presently, CT with contrast is fast replacing IVU for evalua-  tion of urinary tract because of tts obvious advantages.    Gamma camera screening following injection of technetium  99m gives information about proximal tubular function. To  assess differential renal functions, diethylene triamine penta-  acetic acid ("Tc DTPA) or dimercapto-succinic acid (aT    DMSA) is used. It is filtered and secreted into the tubular  lumen. Two types are given below.    This scan is done to find out relative functions of both kidneys;  it also tells about the total GFR and what percentage of total  GFR 1s contributed by each kidney. Relative function of 4542%  is considered acceptable for each kidney. The main indication  for DTPA 1s long-term hydronephrosis. Examples are newborn  with antenatally diagnosed hydronephrosis, children with    posterior urethral valves, etc. This scan 1s also useful ir  assessing the improvement in relative function of the kidney  after surgery for above conditions. The yield of DTPA scan  can be improved by injecting IV lasix. This is known as  diuretic renography. This will unmask marginal pelviureteric  junction (PUJ) obstruction (Fig. 38.14).    It is used most commonly for cortical imaging. It shows details  of renal parenchyma. It 1s particularly useful when looking  for segmental abnormalities of kidney (e.g. renal scarring  secondary to conditions such as chronic pyelonephritis and  renal tumours—Fig. 38.15 and Key Box 38.3).   e Preparation: The external genitalia 1s cleaned with soap  solution or an antiseptic agent and 1% lignocaine jelly is  injected into urethra, to provide lubrication and anaesthesia.  This should be left in place for 10 minutes for its action.   2. Position and character of ureteric orifices—in tuberculosis  involving the urinary bladder, the ureteric orifices are shifted  upwards and gaping—golf hole ureter.    Fig. 38.14: DTPAscan showing sluggish clearance of contrast in the right kidney. Left kidney is normal in clearance and function    intravenously. Observe ureteric orifice through cystoscope.  Unilateral delay in appearance of dye suggests obstruction.  If there is bilateral delay, it indicates impaired renal function.    . To remove bladder stones  . For transurethral resection of bladder tumour (TURBT) in    1. Anterior urethroscopy is done in urethral stricture or in  cases of chronic urethritis. It can rule out strictures due to  granuloma.  2. Posterior urethroscopy: To visualise prostatic urethra and  verumontanum  e Verumontanum Is red in cystitis  e In chronic prostatitis, prostatic ducts may be seen  discharging pus   ¢ When lateral lobes of prostate gland are enlarged, they  project into the internal meatus and produce an inverted  'V' appearance.    MRI of the genitourinary system is useful in many situations.  It is more expensive compared to other investigations. A few  uses have been given below.    Uses of MR urography  ¢ For accurate evaluation of inferior vena cava (IVC)  thrombus in renal cell carcinoma (RCC).    e Extrinsic causes of ureteric obstruction causing  hydronephrosis (e.g. retroperitoneal fibrosis, pelvic  tumours)   | WHAT IS NEW IN THIS CHAPTER? / RECENT ADVANCES (i    e Urine examination and CT scan have been discussed  in more details.    A. Presence of more than 2 RBCs/high power field  B. Presence of more than 3 RBCs/high power field  C. Presence of more than 1 RBC/high power field  D. Presence of RBCs in the urine    . Which one of the following can be detected by urine    . When do you suspect carcinoma prostate? i.  A. If patient has urgency of micturition  B. Rectal examination reveal grade 2 enlargement of  prostate  C. If prostate specific antigen levels are more than  4 ng/ml  D. If patient has recurrent urinary tract infection 9.  . Spider leg deformity in intravenous pyelography is a  diagnostic sign of which disease?  A. Hydronephrosis B. Polycystic kidney  C. Horseshoe kidney D. Carcinoma kidney  . Which of the following is a contraindication for  intravenous pyelography (IVP)? 10.  A. Staghorn calcul  B. Tuberculosis of kidney  C. Horseshoe kidney  D. Multiple myeloma  ANSWERS  1B 2A 3 C 4B S iD 6    Can be done when the kidney is not visualised by IVP  Intravenous contrast need not be given  It is an invasive procedure    Investigation of choice to know the renal parenchymal/  cortical function or damage is:   e Surgical anatomy  e Polycystic kidneys  e Horseshoe kidney  e Renal stones    Kidneys are the retroperitoneal organs, two in number on  either side of vertebral column. Each kidney is bean or  reniform in shape.   Owing to the presence of liver, right kidney 1s 1—2 cm lower  than the left kidney, extending from L|!—L3 and the left  kidney extends from T12—L3    These relationships are very important for surgeons, as the  structures mentioned above may get injured during  operations on kidney (Table 39.1). Also they may get  directly involved by renal malignancies (local spread).    Both the kidneys and associated adrenal glands are surrounded  by varying degrees of perirenal or perinephric fat and these  are enclosed loosely together by the perirenal fascia, commonly  called Gerota’s fascia.    Anterior Below: Hepatic flexure of colon  (Fig. 39.1) Medial: 2nd part of duodenum  Medial Above: Adrenal, liver, IVC  Lateral Below: Ascending colon  Above: Liver Above: Spleen    Below: Splenic flexure, pancreas and splenic vessels, below the pancreas 1s jejunum,  above the pancreas is stomach and spleen    Perinephric fat with renal pedicle are responsible for  classical ballotability of renal swelling.   The anterior and posterior sheaths of Gerota’s fascia,  become fused on three sides around the kidney laterally,  medially and superiorly.    Same in both kidneys. Each kidney rests upon four muscles: Psoas, transversus abdominis, quadratus lumborum, diaphragm    Gerota’s fascia fuses with the diaphragm (as a result ow  which kidney moves with respiration). Students should  remember here that movement with respiration is a  characteristic feature of intraperitoneal organs and masses, and  many times this feature is used to support the diagnosis of  intraperitoneal organ. However, even though the kidney is a  retroperitoneal organ, it also moves with respiration.    Medially  Gerota’s fascia of one side crosses the midline and fuses with  Gerota’s fascia of the opposite side.    ¢ Gerota’s fascia forms an important anatomical barrier and  tends to confine pathological processes originating from  the kidney.    ¢ However, because of its deficiency inferiorly, a collection  within Gerota's fascia may track down and extend into  pelvis.    * Renal artery is an end artery and the entire renal  arterial system is composed of end-arteries (without  anastomosis and collateral circulation). Occlusion of  any branches of renal artery (within kidney known as  segmental arteries) results in infarction of area  supplied by it.    ¢ Incontrast, the renal parenchymal veins anastomose freely  with each other and even with perinephric veins.    ¢ This is an autosomal dominant disease transmitted through  chromosomes from anyone of the parents. Early onset con-  genital cystic kidneys are found to be autosomal recessive.   ¢ ADPKD: Autosomal Dominant Polycystic Kidney Disease  (Figs 39.2 and 39.3).   ¢ During development, some of the uriniferous tubules fail  to join with the collecting ducts. Such uriniferous tubules  develop into cysts. The important pathological features are  as follows:   ¢ When the cystruptures into the pelvis of the kidney, it results  in haematuria.    e As the disease progresses, cysts progress in size which lead  to pressure atrophy of the functional renal parenchyma  leading to renal failure.    Types (for recessive)  1. Foetal type: Autosomal recessive disease may cause intra-  uterine death due to obstructed labour.    2. Infantile type: Autosomal recessive, early renal failure with  death within 3-5 years of life.    3. Adult type: Autosomal dominant type. Presents in the 3rd  or 4th decade with symptoms (Fig. 39.3).    ¢ Even though congenital, it manifests at middle age around  40 years.   2. Plain X-ray KUB: Enlarged kidney can be seen because of  changes in the density between the perirenal pad of fat and  the kidney.    ¢ Hypertension (secondary) (75%) 1s due to renal ischaemia  which stimulates juxtaglomerular apparatus to secrete  renin. It may also be related to a separate genetic factor.    ¢ Bilateral renal mass: Both kidneys are enlarged, surface  (Figs 39.4 and 39.5).    is nodular or bosselated, firm to hard and some times cystic.  ¢ Features of renal failure: Thirst, vomiting, abdominal 4. IVU: The spider leg deformity of the calyces.  distension due to paralytic ileus, anuria, uraemic smell,    coated tongue, anaemia. Treatment  ¢ Infection, pyelonephritis ¢ Asymptomatic polycystic kidney does not require any  ¢ Acute pain: If there is haemorrhage into or infection of a treatment other than regular follow-up.  cyst. Colicky pain is due to blood clot in the ureter. ¢ Polycystic kidney with hypertension: Control of  hypertension with drugs. When hypertension becomes  Diagnosis uncontrollable, bilateral nephrectomy followed by renal    1. Serum urea and creatinine to rule out renal failure. Normal  creatinine levels: 0.8—1.6 mg%. Normal urea: 20 to 40  mg%.    A 45-year-old lady with mass abdomen was allotted as my  long case. She had undergone open cholecystectomy 3    ‘= ; a : months back (ultrasound was not available at that time).  eo Se | She had a vague renal mass in the right loin. I was not sure.  — _——— 3 When I recorded BP it was 200/110 mm of Hg. I carefully  > * i a ~ palpated the opposite side where I was able to palpate a  a a renal mass. The diagnosis became evident. It was polycystic    kidney. In fact one of the examiners was the surgeon who  had operated on this patient for open cholecystectomy.  During that surgery, he discovered enlarged kidneys. The  patient was asymptomatic.    ¢ Infected cyst or if pyelonephritis develops: Appropriate  antibiotics are given and if necessary, the cyst should be  aspirated using ultrasound guidance.    ¢ Polycystic disease with renal failure, emergency dialysis  followed by renal transplantation 1s the treatment of choice.  The related donor should be screened for polycystic trait.    ¢ During development, 2 mesonephric buds appear on the  side of the future vertebral column and grows into  metanephros. Mesonephric buds form ureter and  metanephros kidneys.    ¢ If fusion occurs at lower pole, it results in a classical  horseshoe kidney (Fig. 39.6).    ¢ Rarely, upper polar fusion can occur giving rise to reverse  horseshoe kidney.    ¢ Inferior mesenteric artery crosses the isthmus at the level  of L3—L4. Hence, horseshoe kidney cannot ascend. It 1s  felt lower down in the abdomen.   ¢ A palpable mass below and to the right and to the left of  umbilicus or umbilical region can be a horseshoe kidney.   ¢ Recurrent urinary tract infection (UTI) is common  because the ureters are angulated over the kidney isthmus.   e They are more prone for hydronephrosis due to the  angulation of ureters.   ¢ Rovsing’s sign: Hyperextension of the spine results in  abdominal pain, nausea or vomiting due to stretching of  the capsule.   2. IVU: Upper and middle calyx are directed laterally but  lower calyx is directed medially where there is fusion,  which 1s characteristic of horseshoe kidney.    ¢ Removal of the stone, or repair and reconstruction of the  hydronephrosis are done in the usual manner.    1. Infection: Organisms such as Proteus, Pseudomonas,  Klebsiella produce recurrent UTI. These organisms produce  urea, cause stasis of urine and precipitate stone formation.  Nucleus of the stone may harbour these bacteriae (Fig. 39.7).    2. Hot climates cause increase in concentration of solutes,  resulting in precipitation of calcium and formation of  calcium oxalate stones.   ¢ Diet rich in red meat, fish, eggs can give rise to aciduria.   ¢ Diet lacking in vitamin A causes desquamation of renal  epithelium which precipitates calcium, alters it and forms  stones.  Epitaxy: Growth of one type of stone over another type.   Fig. 39.7: Chronic pyelonephritis with calculi (Courtesy: Prof  Sasidharan, Head, Dept of Urology (2002-2008), KMC, Manipal)    ¢ Hyperparathyroidism increases serum calcium levels  resulting in hypercalcinosis and pelvic stones.   ¢ Gout increases uric acid levels and causes multiple uric  acid stones.    of calcium in the urine resulting in calcium oxalate stones  (they pass skeletons in urine).    hours) keeps the urinary pH low. When citric acid levels  decrease, it promotes precipitation of urinary calcium.  Citrate excretion 1s under hormonal control.    kidney, unascended kidneys are more vulnerable for  development of stones due to stasis.    small erosion or an ulcer develops at the tip of renal papilla  on which minute concretions or minor calcium particles  get deposited and give rise to stone formation.   ¢ Produces haematuria very early, resulting in deposition  of blood over the stone giving a dark colour to the stone.   ¢ Contains alternate layer of calcium and bacterial  vegetation. It is visualised in plain X-ray KUB.   ¢ Contain calcium oxalate which makes them opaque. Pure  uric acid stones are radiolucent.   ¢ Commonly occur in renal pelvis and tend to grow in  alkaline urine.   ¢ As it enlarges in the pelvis, it grows within the major  and minor calyces and slowly forms staghorn calculus.  This calculus produces recurrent urinary tract infection  and haematuria and slowly damages the renal  parenchyma (Fig. 39.9).    ¢ Cystinuria is an inbom error of metabolism which occur:  due to decreased resorption of cystine from the rena  tubules.   ¢ Increased excretion of cystine in urine results in cystine  calculus.    in the renal angle formed by the sacrospinalis and 12th rib.  Murphy’s kidney punch test demonstrates tenderness at  renal angle. The same pain may some times be felt anteriorly    in the costal margin. Hence, it 1s described as costovertebral  pain. Nausea and vomiting is due to intense sympathetic  stimulation caused by stretching of renal capsule mediated  by coeliac plexus.   ¢ Ureteric colic: When the stone is impacted in the  pelviureteric junction or anywhere in the ureter, it results  in severe colicky pain originating at the loin and radiating  to the groin, testicles, vulva and medial side of the thigh.  This may be associated with strangury. The referred pain is  due to irritation of the genitofemoral nerve.   ¢ Haematuria is common with renal stone because the  majority of stones are oxalate stones. The quantity of blood  lost is small but it 1s fresh blood (Key Box 39.2).   ¢ Recurrent UTI: Fever with chills and rigors, burning  micturition, pyuria may occur, along with increased  frequency of micturition.   ¢ Guarding and rigidity of the back and abdominal muscles  during severe attack of pain.    1. Calculous hydronephrosis occurs due to back pressure  producing renal enlargement. Stretching of the renal capsule  results in pain. In such cases, an associated palpable kidney  mass suggests hydronephrosis.   3. Renal failure: Bilateral staghorn stones may not be  symptomatic until they present with uraemia and renal  failure.   4. Squamous cell carcinoma: Long-standing stones increase  the risk of carcinoma.    1. Blood urea and creatinine to rule out renal failure.  2. Plain X-ray KUB (Figs 39.14 and 39.15)  ¢ To diagnose stones 90% of the renal stones are radio-  opaque.  ¢ Enlarged renal shadow can be seen.  3. USG  ¢ Presence of the stone can be diagnosis  ¢ Exact size and location of the stone can be evaluated.    1. Polycystic disease of kidney  2. Renal stone, ureteric stone  3. Renal tuberculosis   Fig. 39.14: Plain X-ray KUB showing large stone in the pelvis    ¢ To locate the stone exactly in relation to kidney and ureter  and to assess renal function. A nonradiopaque stone  can be seen as a filling defect. Hydronephrosis and hydro-  nephroureterosis can also be seen.   ¢ Now noncontrast CT scan and contrast CT scan are used  for more accurate detection of causes of abdominal colic.    The treatment of renal stones can be divided into nonoperative  treatment and operative treatment.    1. Conservative: Small stones less than 5 mm itn size pass  off with intake of copious amount of fluids and at times  forced diuresis. Intravenous hydration followed by  intravenous frusemide may help pass the stones  spontaneously.   2. Extracorporeal shock wave lithotripsy (ESWL): After  cystoscopy, a ureteric stent (Double J stent) is placed into  the ureter on the side of a large renal stone (Fig. 39.16).  Shock waves are generated (around 500—1 500 shock waves)  which blast the stone. The stones get crushed and most of  the stones will come out by the side of the stent (Key Box  39.3). Small stones can be removed without prior crushing.   ¢ Small pieces of fragmented calculi collect and obstruct in  the distal ureter.    ¢ Holmium-YAG laser (Ho-YAG) 1s the most effective with  good margin of safety.   ¢ Ho-YAG laser lithotripsy occurs primarily through a  photothermal mechanism that causes stone vaporisation.   ¢ They have the ability to fragment all types of stones  regardless of the composition.  ESWL 1s not effective in fragmenting cysteine stones    Percutaneous nephrolithotomy (PCNL): Retrograde  pyelography (RGP) is done when the stone 1s located in  the pelvis of the kidney. With a small | cm incision in the  loin, the PCN needle 1s passed into the pelvis of the kidney  and is confirmed by fluoroscopy. A guide wire 1s passed  through the needle into the pelvis of the kidney. The needle  is withdrawn, with the guide wire left within the pelvis.  Dilators are passed over the guide wire and a working sheath  is introduced into the pelvis. A nephroscope is passed into  the pelvis and if the stone is small, it can be taken out. If it  is big, it may have to be crushed using ultrasound probes  and the fragments are removed. Ultrasound or pneumatic  energy 1s used for fragmenting.   B. Nephrolithotomy: When there is intrarenal pelvis, the stone  has to be taken out through the kidney parenchyma.   C.Extended pyelolithotomy: By retracting the kidney  parenchyma laterally, the incision over the pelvis can be  extended over to the calyx and the stone can be extracted  from the calyx. Even a large staghorn calculus can thus be  removed.   D. Pyelonephrolithotomy: Stone is extracted through an  Incision in the pelvis as well as the renal parenchyma.   E. Partial nephrectomy: When the stone from a lowermost  calyx 1s impacted, a lower pole nephrectomy can be done.    A. Bilateral renal stones: Kidney with better function has to  be operated first. |—2 weeks later, the opposite side can be  operated.   B. If there is pyonephrosis with a severe degree of fever, pain  and tendemess, nephrostomy is done and a tube drain 1s    placed in the pelvis of the kidney for drainage of pus and  urine. Once the pus is cleared, fresh assessment of renal  function is done. If the kidney is nonfunctioning,  nephrectomy is done. If the kidney 1s functioning ESWL/  PCNL/open procedure is done. This is known as percuta-  neous nephrostomy (PCN).    Open procedures for the management of stone disease  have become obsolete and are found in old surgery text-  books.    Stones come down from pelvis of the kidney and may get  impacted at any site of anatomical narrowing of ureter, namely:   3. Crossing of the vas deferens or broad ligament.  4. Site of entry into the bladder wall    This may lead to hydroureteronephrosis, renal parenchymal  atrophy, infection and pyonephrosis.    ¢ Pain in the loin radiating to groin: Pain ts severe, colicky,  intolerable and lasts for a few hours. When stone descends  into lower ureter, pain radiates to the testicles, labia majora  and to the upper portion of thigh due to irritation of  genitofemoral nerve. Colic lasts for about 4-6 hours and  is relieved by antispasmodics, narcotics and NSAID.    ¢ Guarding and rigidity of the abdominal wall if present on  the right side, 1s confused with acute appendicitis.    1. Most of the ureteric stones pass via naturalis (urine). The  patient is asked to consume a lot of water and  antispasmodics.    2. Flushing therapy: About 2 L of IV fluid, with 20-40 mg  inj frusemide (Lasix). It can be repeated for a few days.    3. Stone in the upper ureter: ESWL 1s the ideal treatment.    Retrograde intrarenal surgery (RIRS) for ureteric, renal and  calyceal stones. Flexible ureteroscopy with laser fulguration  can also be done.    4. Middle ureteric stone: ESWL, ureteroscopy basketing or  open surgery (ureterolithotomy).    5. Lower ureteric stone: Ureteroscopic removal. With the  usage of ureteroscope passed through the urethra, direct  visualisation and manipulation of the stone even if it 1s  impacted can be done. A laser lithotripter or ultrasonic  lithotripter can be used to disintegrate the stone.    6. Vesicoureteric junction: Ureteroscopic removal or  endoscopic meatotomy of vesicoureteric junction. For a  stone impacted at ureterovesical junction, cystoscopy 1s  performed. Ureteric orifice is identified and a cut is given  at its mouth. Under fluoroscopic monitoring, the stone can  be manipulated and basketed out using a dormia basket or  other types of baskets available.    7. An impacted stone which is not amenable to ESWL,  fluoroscopic or ureteroscopic manipulation have to be  extracted by ureterolithotomy (open surgical method).    1. Metabolic work up of urine and blood for identifying  metabolic causes. Example: Hyperparathyroidism to be  ruled out by 24 hours urine analysis for calcium, phosphate  and uric acid levels.   3. Dietary adjustments: Red meat to be avoided (rich in uric  acid).   4. Drug treatment  ¢ Xyloric, sodium bicarbonate: Uric acid stones  * Potassium citrate: Calcium stones  ¢ Thiazides small dose: Calcium stones  ¢ D-penicillamine: Cystine stones  ¢ Protease inhibitors: Infection stones against E.coli    Definition  Aseptic dilatation of the whole or part of the pelvicalyceal    system of the kidney due to partial or intermittent interruption  to the outflow of urine.    A. Pelviureteric junction (PUJ) abnormality (PUJ dyskinesia  or achalasia of PUJ) is a congenital lesion where  hydronephrosis occurs due to failure of transmission of  neuromuscular impulses through the narrow PUJ. It can    also be bilateral. Male : Female ratio is 2: 1. More common  in males on the left side.    A.Carcinoma of the ureter or carcinoma of the bladder  involving the ureteric orifice.    B. Stricture of the ureter secondary to stone: After dislodge-  ment of the stone there can be inflammatory stricture of  the ureter.   1. Involvement of ureter by carcinoma cervix, rectum, bladder,  the retroperitoneal tumours, primary or secondary deposits  in lymph nodes.    ¢ Aberrant renal artery going to the lower pole of kidney can  cause obstruction to the ureter causing hydronephrosis.    Lower urinary tract obstruction below the level of bladder neck  will give rise to bilateral hydronephrosis (Ormond’s disease).  Any of the causes for unilateral hydronephrosis when present  bilaterally cause bilateral hydronephrosis (Fig. 39.17).    1. Stricture urethra is commonly due to gonococcal  urethritis. Iatrogenic strictures following instrumentation  of urethra or following a rupture urethra later is becoming  more common.   2. Bilateral aberrant vessels: Often a time, these may be the  branches of renal artery and vein which cross the ureters.    1. Benign prostatic hypertrophy (BPH)  ¢ Common cause in middle age  2. Contracture of bladder neck  3. Idiopathic retroperitoneal fibrosis (Ormond's disease).    Due to growing foetus and partly due to the hormone  progesterone.    The back pressure effect depends upon the type of the pelvis  (Figs 39.19 and 39.20).    1. In patients with intrarenal pelvis the kidney gets damaged  very early. As the time goes on, the urine gets diluted. All  the salts are absorbed and ts replaced by a watery type of  fluid having a specific gravity of 1010.   2. Patients with extrarenal pelvis have minimal damage to the  renal parenchyma for a long time.    3. Even if there is complete obstruction to the urinary flow in  a hydronephrotic kidney, some amount of urine Is secreted,  some of it 1s absorbed by the renal pelvis and the collecting   of the kidney. The urine is thought to enter the interstitial   ¢ If the disease progresses further, it leads to a  nonfunctional kidney.    ¢ If the disease is bilateral, it may give rise to uraemia.    Painless enlargement of the kidney. A renal mass 1s felt in  the loin with a smooth surface and firm in consistency  (tensely cystic).    . A dull-aching pain in the loin  . Previous history of calculus disease  . Hypertension and haematuria are rarely seen in hydro-   ¢ Following an attack of renal colic, ureteric obstruction  occurs due to stone which results in enlargement of  the pelvis of the kidney resulting in a palpable mass in the  loin. After a few hours, the mass disappears due to the  passage ofa large quantity of urine due to reflux polyuria  or due to slipping of the stone.    . The symptoms of primary cause may be evident in the    history, e.g. colicky radiating abdominal pain due to stones  and haematuria.    USG can detect enlarged kidneys and can detect the cause  for hydronephrosis in majority of cases.   CT scan Is the investigation of choice. It can assess anatomy  and function more accurately than IVU (Fig. 39.21).    ¢ They become flat and later convex/club shaped followed  by dilatation of pelvis and ureter depending upon the  level of obstruction.    ¢ In hydronephrosis with gross impairment of renal function,  the dye may not be visible for a few hours in the X-ray.  In such cases, large quantity of dye (100-200 ml) may  have to be used and the pictures may have to be taken  even after 24 hours. Such a situation is seen in PUJ  dysfunction.    (diethylene triamine penta-acetic acid) scan using a  Gamma camera. The above Gamma radiation emitter 1s  injected intravenously and it can be detected to have been  trapped in the urinary tract above the level of obstruction.  It does not get washed off even after giving frusemide  injection.    6. Retrograde pyelography (RGP)  ¢ When the IVP fails to demonstrate the kidneys, RGP 1s a  useful investigation. It can be done just prior to definitive  surgery for confirmation of the site of obstruction.    ¢ Less quantity of dye is required and better configuration  of calyces can be made out.  7. Blood urea, creatinine is estimated to rule out renal  failure    |. Hydronephrosis secondary to a cause  Treatment of the cause has to be done. Examples:  a. Stones: Pyelolithotomy, ureterolithotomy    c. Aberrant vessel: Transection of the ureter and anastomosis  in front of the vessel.    g. Benign prostatic hypertrophy (BPH): Transurethral  resection of the prostate (TURP).    h. Carcinoma of prostate: TURP + bilateral orchidectomy +  hormonal therapy.    2. If the cortical thickness 1s adequate (0.5 cm) by ultra-  sonography, even though it 1s a nonfunctioning kidney, a  preliminary nephrostomy to decompress the system has  to be done. Reassessment of the renal function 1s done  after a few days. If the renal function improves, definitive  surgery for hydronephrosis can be done. If it remains a  nonfunctioning kidney and the opposite kidney ts normal,  nephrectomy is done.    3. In B/L hydronephrosis, kidney which is functioning better  should be operated first.    Congenital hydronephrosis needs special mention here. With  increasing use of obstetric ultrasound, the incidence of  antenatally detected foetal hydronephrosis is on the rise. In  the present scenario, antenatal detection of foetal  hydronephrosis has become the most common mode of  presentation of congenital hydronephrosis.    Congenital hydronephrosis is defined as the antero-  posterior diameter of renal pelvis > 10 mm at > 20 weeks of  gestation. PUJ obstruction is the main cause (Fig. 39.23). These  foetuses undergo serial ultrasound monitoring during the rest  of pregnancy and based on the increase or decrease 1n the  pelvic diameter during this period, postnatal management can  be planned even before the child 1s born. Recently one study  has graded antenatal hydronephrosis due to PUJ on the basis  of pelvic diameter and proposed the management guidelines  (Dr Vikas Jain, Professor Sasidharan et al—KMC, Manipal).    I Mild 11-20 mm  II Moderate 21-35 mm  III Severe >35 mm    ¢ Grade I hydronephrosis can be managed conservatively  by serial monitoring of pelvic diameter by ultrasound and  renal functions by DTPA scan. This is known as  conservative or nonoperative management. These kidneys  Improve over a period of time.   ¢ Grade II hydronephrosis: Majority (almost 80-90%) can  be managed conservatively. However, close monitoring of    (A) Excision of the redundant pelvis along with PU4J,  (B) Completed ureteropelvic anastomosis    the patient 1s required to detect any deterioration in renal  function. Any deterioration in renal function 1s an indication  for surgical intervention. In this group, 10-20% patients  benefit from early surgery (patients with renal function of  involved kidney < 40%).   ¢ Grade III hydronephrosis: All these patients should be  operated early—Anderson-Hynes pyeloplasty to prevent  permanent damage to kidney.   3. New ureteropelvic anastomosis 1s done in such a way that  urine should drain by gravity.    ¢ This is the most popular type of dismembered pyeloplasty  (Figs 39.24A and B).    ¢ They are Foley’s Y-V plasty or flap pyeloplasty. The PUJ  is not transected. However, they are not very popular.    ¢ This 1s secondary to pulmonary tuberculosis/lymphatic  tuberculosis. The primary focus ts often difficult to identify  — Common in males, in the 20-40 age group.  — Infection is always haematogenous. Often one may not  find any active lesion in the lung or in the lymph nodes.  — Usually unilateral.    1. Tubercles develop and coalesce over the papilla which may  ulcerate—ulcerative form.    2. The tubercles may caseate and rupture over the renal papilla  and communicate with the pelvis. This variety is called as  ulcerocavernous form.    4. Tubercular hydronephrosis is very rare. It is due to  tubercular stricture of the pelviureteric junction.    5. The opening of one of the calyces may get fibrosed leading  to hydrocalyx which may distort rest of the calyces.    6. Cortical abscess ruptures into the perinephric space and  forms a tubercular perinephric abscess. This may even  point at the loin and rupture forming a sinus in the loin.    7. Tubercular pyonephrosis (caseous kidney, putty kidney,  cement kidney). When it gets calcified, it is called cement  kidney. The entire kidney is converted into a bag of pus  which is tubercular caseous material with or without  secondary infection. The complete ureteric stricture due to  tuberculosis cutting off the pelvicalyceal system may result  in autonephrectomy because of fibrosis.    9. As apart of miliary tuberculosis—multiple small tubercles  may be seen in the renal parenchyma.    1. Frequency is the earliest symptom of tuberculosis. It is due  to renal tubular inflammation and later due to tubercular  cystitis.    Sterile pyuria ts seen in tuberculosis, stones and in  carcinoma in situ.    3. Haematuria 1s not uncommon. Usually it is a small quantity  due to ulcerocavernous variety.    4. Evening rise of temperature.  5. Loss of appetite and loss of weight.    6. Evidence of pulmonary or lymph node tuberculosis (TB)  may be present.    ¢ Early morning sample of urine has to be examined which  gives the highest concentration of AFB, for 3 days.    Newer easy methods like polymerised chain reaction  (PCR) and radioisometric culture are also used.   Fig. 39.25: Ulcerative form Fig. 39.26: Ulcerocavernous form Fig. 39.27: Pseudocalculi   Fig. 39.28: Hydronephrosis Fig. 39.29: Tuberculous perinephric abscess Fig. 39.30: Tuberculous pyonephrosis   ¢ Due to extensive periureteric fibrosis, the ureter becomes  thickened, shortened and straight. The ureteric openings  are lifted upwards and they are gaping which means it  does not contract/does not close when bladder contracts.  Such contracted, elevated, permanently opened, lower  end of ureter is called as golf hole ureter (Fig. 39.34).   ¢ As aresult of this, with each act of contraction of bladder,  there is reflux of urine into the kidney causing damage,    . When the disease involves the urinary bladder, it results in    fibrosis. It becomes small and contracted with ineffective  function. Storage capacity is lost resulting in intractable  frequency, with a few drops of urine. There is bleeding.  Micturition 1s painful and ts called strangury. There 1s also  severe pain in the suprapubic region which Is referred to  the tip of the penis. Such a small nonfunctioning urinary  bladder is called as thimble bladder (Fig. 39.35).    Conservative line of management with antituberculous  treatment is successful, provided kidneys are functioning  as in early stages.    ¢ Indicated when there is stricture of calyces which results  in a hydrocalyx.   ¢ In this operation the stricture is divided so that the  drainage becomes better.    ¢ In this 10-15 cm of ileal loop is isolated based on the  blood vessels, the fibrosed bladder dome is excised,  intestine is split open and sutured to urinary bladder  (Pro: 39.37).   ¢ This 1s to increase the capacity of bladder so as to store  urine and reduce frequency.   Fig. 39.35: Genitourinary tuberculosis: Left kidney is normal.  Right kidney shows hydroureteronephrosis. The urinary bladder  is contracted—thimble bladder    ¢ This isa malignant tumour of the kidney occurring in children.   ¢ The tumour 1s composed of epithelial and mesothelial  elements. Thus, it may contain bone, cartilage, muscle, etc.  Hence, it is called nephroblastoma (immature embryonic  tissue).   ¢ The tumour arises in one of the poles, distorting the reniform  shape of the kidney. It 1s greyish white or pinkish white in  colour. At places, there may be areas of haemorrhage/necrosis.   ¢ Microscopic features include connective tissue elements  cartilage, spindle cells, smooth striated muscle cells and  epithelial elements.   ¢ The child is brought with abdominal distension, due to  hugely enlarged kidney which on palpation feels nodular.   ¢ Haematuria is a bad prognostic symptom. It is an  indication of rupture of tumour into the pelvis of kidney.  Most of such children die by 2 years of age.   ¢ Low grade fever can occur in rapidly growing tumour due  to tumour necrosis, which releases pyrogens.    1. Abdominal USG can detect a solid tumour in the kidney.  Ultrasound also rules out opposite kidney tumour.   2. CT scan to know extent of lesion and spread to the adjoining  structures (Fig. 39.38).   3. IVP is done to study distortion of calyces and to evaluate  the function of the opposite kidney.    Fig. 39.38: A 3-year-old  male child had presented  with left loin mass. CECT  abdomen and pelvis was  done which showed a  large tumour in the left  kidney. Left radical  nephrectomy was done  and the histopathology  was reported as Wilms’  tumour    |. Neuroblastoma arises from adrenals. This is more common  than nephroblastoma.   Fig. 39.39: CECT abdomen and pelvis showing a large right  heterodense adrenal tumour   6. Homovanillic acid (HVA) and Vanillylmandelic acid  (VMA) increase in neuroblastoma.   2. Lymphatic spread occurs to the hilar lymph nodes, para-  aortic lymph nodes, mediastinal and left supraclavicular  lymph nodes.   3. Haematogenous spread occurs to the lungs, liver, bones,  brain, etc. The tumour thrombus can extend to the renal  vein and inferior vena cava.   1. For tumours confined to renal capsule or perirenal soft  tissue not infiltrating the adjacent organs, radical nephrec-  tomy followed by chemotherapy with actinomycin D and  vincristine are given for 6 months.    2. For tumours which have gone beyond renal capsule and  perirenal soft tissue, local infiltration to adjacent tissue,  lymphatic metastasis, nephrectomy followed by local radio-  therapy and chemotherapy is given with actinomycin  D and vincristine for 15 months.   3. If the tumour is found to be unresectable by CT scan or  magnetic resonance imaging (MRI), preoperative FNAC  to confirm diagnosis is indicated followed by preoperative  radiotherapy (1000 cGy) or chemotherapy. Once the tumour  regresses in size, nephrectomy has to be done. Postoperative  chemotherapy is given with actinomycin D, vincristine and  doxorubicin.   4. Bilateral Wilms’ tumour: Radical nephrectomy on the side  of the larger tumour and partial nephrectomy on side of the  smaller tumour should be done. As much of renal tissue as  possible should be preserved after leaving a tumour free  margin. Postoperatively the patient has to be treated with  chemotherapy. If the surgery is not feasible, only  radiotherapy and chemotherapy has to be given. Growth  disturbances, cardiac and pulmonary toxicities are  complications of radiotherapy.    ¢ It is also called Hypernephroma or Grawitz tumour.  ¢ Commonly found in the age group 40-60.  ¢ Male : female ratio 2: |    ¢ Not known. Rarely familial RCC is known. Specific  oncogenes have been identified. Smokers are twice as often  affected than nonsmokers.   RENAL CELL CARCINOMA: AETIOLOGY  1. Chronic cystic disease  2. Chromosomal defect  3. Cadmium exposure  4. Cigarette smoking  5. Coffee drinking  6. Congenital disease—von Hippel-Lindau disease  Observe 6 Cs    ¢ Nearly all renal cancer in adults are adenocarcinoma  ¢ Cell of origin: Proximal renal tubular epithelium.    ¢ Starts in one of the poles, commonly in the upper pole  and usually ruptures outside the capsule because of whick  reniform shape of kidney is maintained (Wilms’ tumour  grows within the capsule. Hence, the kidney shape is lost  very early) (Fig. 39.40A).    ¢ A few haemorrhagic areas are common because the  tumour is very vascular (Fig. 39.40B).    ¢ Microscopy: Alternate clear cells and dark cells.  ° Various subtypes of RCC  1. Clear cell carcinoma  ¢ Most common type (70-80%)  ¢ It can be familial, associated with von Hippel-Lindau  syndrome or sporadic (95%).  2. Papillary—both familial and sporadic forms.   TUMOUR | BONY HAEMATOLOGICAL |  Pain | Fractures Anaemia |  Haematuria | Secondaries Polycythaemia    3. Chromophobe—these tumours exhibit multiple ¢ Other hormones produced by the tumour are  chromosome losses and extreme hypoploidy. parathormone, adrenocorticotrophic hormone, human  4. Renal medullary carcinoma chorionic gonadotropin, glucagon, prolactin.    Robson's staging of renal cell carcinoma  CLINICAL FEATURES (Fig. 39.41) ¢ Stage I: Tumour limited to kidney    I. Triad of renal cell carcinoma  gland, but does not extend beyond Gerota’s fascia.    Triad is seen in only 9%, but if present, strongly indicates  metastatic disease.  1. Pain: Dragging or intermittent clot colic due to blood clot  blocking the ureter. : Tx Primary tumour cannot be assessed  . Intermittent haematuria 79. ‘Neaureaine  3. Palpable mass: Hard, nodular, ballotable and bimanually T1 < 70cm, within capsule, in greatest dimension  palpable, loin mass moving with respiration. T2 >7cm, within capsule    3. Mild elevation of temperature is due to tumour necrosis  producing pyrogens. It can present as pyrexia of unknown  origin (PUO) and hence it is called Internist’s tumour.    1. Urine examination is done when the patient has haematuri<  to look for malignant cells.  2. Plain X-ray KUB region: Enlarged kidney can be seen.  3. IVP: Distortion of calyces, missing of calyces or loss of  architectural pattern of kidney.  4. USG:  ¢ Enlarged kidney  ¢ Locate tumour, site and extent  ¢ USG-guided FNAC can be done  ¢ Can detect thrombus in inferior vena cava (IVC)   : 5. Contrast enhanced CT scan is the investigation of choice  Fig. 39.42: Right RCC with left adrenal metastasis for staging (Key Box 39.7 and Figs 39.42 to 39.46).    Any renal mass which enhances after contrast on a CT  scan in an elderly person should be considered as RCC  until proved otherwise.    6. Renal angiography is done by retrograde transfemoral  approach. Features are as follows:  Fig. 39.43: Chest X-ray showing cannon ball ¢ Neovascularisation: Tumour blush inside the tumour.  ¢ Venous phase has to be observed to rule out tumour  extension in the vein.    7. MRI scan: MRI scan ts the investigation of choice to know  the extent of IVC thrombosis (better than CT).   8. Venacavogram: It is done to know extent of tumour tn the  IVC and presence of collateral circulation.   ¢ En bloc removal of entire Gerota's fascia with its  contents, 1.e. kidney, proximal ureter, adrenal gland.   ¢ Retroperitoneal lymph node dissection does not improve  the survival rate.   ¢ Routine removal of ipsilateral adrenal gland is  uncommon. Unless tumour invovles large portion of  upper pole of kidney or there 1s suggestion of adrenal  gland involvement on preoperative radiologic exams.    2. Radical nephrectomy with extraction of tumour thrombus  ¢ The tumour thrombus can extend along the renal vein  into IVC and even into the right atrium (Fig. 39.47).   Fig. 39.47: Renal cell carcinoma with tumour thrombus in the  renal vein, inferior vena cava    ¢ Infradiaphragmatic tumour thrombus can be removed  with proximal control over the vena cava. The supra-  diaphragmatic IVC thrombus requires cardiopulmonary  bypass (Figs 39.48 and 39.49),   ¢ In the absence of distant metastasis after removal of the  thrombus these patients survive for a long duration.    3. Nephron sparing surgery  It is done when tumour length is less than 5 cm, in bilateral  tumours and in cases of renal cell carcinoma in solitary  kidney.    4. Therapeutic embolisation  ¢ This can be used as a palliative measure in advanced  carcinoma to relieve symptoms. This can also be used  preoperatively to regress the size of the large tumour.    ¢ A catheter is placed in the renal artery and substances  such as gel foam, blood clot, crushed muscle are injected.   ¢ They block the lumen of the vessel and reduce size of  the tumour so that radical nephrectomy can be undertaken  later.    5. Radiotherapy  Not of much use. However, it is a good form of palliation  for secondaries in the lung, bone and brain.    6. Immunotherapy  Administration of interferon or interleukin-2 has been  found to improve the survival rate.   ¢ Moves with respiration because the fascia of Gerota  encloses the kidney and fuses above with the diaphragm.    ¢ Upper border is not palpable because it is under the |] 2th  rib.    In this condition, the entire kidney is converted into a sa  containing pus or purulent urine—almost always the rena  parenchyma Is destroyed totally.    1. Renal calculous disease is the most common cause 0  pyonephrosis.   2. Acute pyelonephritis is more common in children and 11  females. Inadequately treated cases may develop intc  pyonephrosis, especially when pyelonephritis is associatec  with urinary tract obstruction.   ¢ Large swelling with high grade fever with chills and rigors  suggest an imminent danger of septicaemia and calls for  an immediate drainage of the pus.    e Urine examination may be positive for coliforms and other  gram-negative organisms.   ¢ Plain X-ray KUB may reveal a stone or an enlarged renal  outline.    ¢ Intravenous urogram demonstrates poor function of the * X-ray spine: Scoliosis with concavity towards abscess  kidney on the diseased side. As a rule the opposite kidney ° Screening chest: Diaphragm is immobile and elevated ot   * Broad-spectrum antibiotics (parenteral) should be started ° Pus Is drained by an incision in the loin, breaking all the  immediately once the urine and blood ts sent for culture locull.    ¢ Ultrasound-guided aspiration of pus or a percutaneous  nephrostomy (better), and drainage of pus greatly improves  the general condition of the patient.   ¢ If any obstruction or causative agent such as a stone Is  found, it should be removed.   ¢ Nephrectomy should be considered if the kidney is non-  functioning with significant damage.    PERINEPHRIC ABSCESS e Most common site is upper pole of the kidney.   ¢ Most common presentation of renal cell carcinoma is with  It refers to the collection of pus in the perirenal area. mass abdomen.  Causes e Most common investigation of choice is contrast enhanced  ¢ Infection in a perirenal haematoma i  ¢ Pyonephrosis when it ruptures ¢ Most common method of spread is by haematogenous  ¢ Tubercular perinephric abscess route.  ¢ Pus from retrocaecal appendicitis can extend into loin, ¢ Most common intra-abdominal malignancy which spreads   perinephric area and may present as abscess. within vena cava and into atrium is renal cell carcinoma.    = e Most common cell of origin is proximal renal tubular  Clinical features epithelium.  ¢ High swinging temperature -  ¢ Rigidity, tenderness, fullness in the loin |  © QOedema in the loin WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES ‘e    ¢ Total count: Raised above 20,000 cells/mm?  ¢ Urine analysis: No organisms are usually found   1. Following is not the feature of adult polycystic kidney: 3. Which of the following is the feature of horseshoe  kidney?    B. Hypertension is seen in about 75% patients  C. It is autosomal recessive B. It does not cause angulation of the ureter causing  hydronephrosis    Which type of stone typically causes haematuria?  A. Oxalate stone B. Phosphate stone   Patient has renal stones and swelling in the neck, the  diagnosis can be:   Which is the investigation of choice in hydronephrosis?  A. Ultrasound B. Intravenous pyelography  C. CT scan D. Isotope renography   C. Clear cells and dark cells are present in microscopy  D. Tumour cells do not line the blood vessels  Polycythaemia in renal cell carcinoma is due to:   The diagnostic CT finding of a renal cell carcinoma in  an elderly patient is:   ¢ Urinary bladder is lined by transitional epithelium that  covers a connective tissue known as lamina propria.   ¢ However, due to changes in the epithelium caused by  chronic irritation (stone), one can get other malignancies  such as squamous cell carcinoma.   ¢ Bladder cancer can easily spread through lamina propria  into the muscle coat (detrusor muscle).    It is asmooth muscle, fibres of which are intermingled. Hence,  in cases of bladder neck obstruction, changes such as  trabeculations/sacculations due to hypertrophy of this muscle  is found.   It is a triangular area lying between the internal urethral orifice  and the orifices of the ureter. [tis the most sensitive part of  the bladder, irritation of which is mainly responsible for  frequency of micturition (Fig. 40.1).    ¢ Internal sphincter is the smooth muscle which surrounds  the bladder neck. It is innervated by a-adrenergic fibres  and prevents retrograde ejaculation.    e Distal urethral sphincter is a somatic striated muscle which  is supplied by S2—S4 fibres via pudendal nerves.    ¢ Posteriorly endopelvic fascia, which is continuous with  lateral ligaments of the rectum, need to be divided during  radical cystectomy.   ¢ Anteriorly puboprostatic ligaments also need to be divided  during radical cystectomy.    False ligaments: Urachus and obliterated hypogastric  arteries together withthe fold of peritoneum overlying these  structures are called as false ligaments (median and lateral  ligaments).    Superior and inferior vesical arteries are derived from  anterior trunk of internal iliac artery as the main source of  arterial blood supply. Minor blood supply comes from  obturator, inferior gluteal and in females from uterine and  vaginal arteries also.   Veins form plexus on lateral and inferior surfaces of the  bladder. Hence during suprapubic cystostomy, these  structures have to be avoided while entering into the bladder.  Vesical plexus is continuous with prostatic plexus of veins  In males which drain into the internal iliac vein.    Internal iliac nodes are the first level of lymph nodes.  Obturator and external iliac lymph nodes get involved later.    Parasympathetic comes from anterior divisions of sacral  nerves—S2,S3,S4 through inferior hypogastric plexus.  Following excision of rectum, disturbance of micturition  and sexual function can occur due to damage to the pelvic  plexus.    Primary: Stone which develops in sterile urine. They  develop in the absence of bladder pathology. These  also include renal stones which have migrated to the  bladder.    ¢ Secondary: Stone develops in the presence of infectior  and stasis due to obstruction to the urinary flow. They  develop secondary to bladder pathology.    1. Oxalate stone: Moderate size, uneven surface, mulberry  stone is dark brown or black because of incorporation 0;  blood pigment in It.    2. Uric acid stone: Round to oval, smooth, pale yellow, not  opaque to X-rays. They are primary stones.    4. Triple phosphate: These stones consist of ammonium.  magnesium and calcium phosphates. They occur in urine  infected with urea-splitting organisms. Sometimes, they  grow rapidly. The nucleus of the stone can be made up of  bacteria, desquamated epithelium or a foreign body. Dirty  white in colour.   e Pain at the end of micturition referred to tip of penis in  young boys suggests bladder stone. In school-going  children, pain is aggravated by jumping and jolting. Painis  decreased on lying down because stone falls away from  the trigone of the bladder. Typically, oxalate stones produce  pain. Painful ineffective micturition is described as  strangury.   ¢ Haematuria, is due to stone causing abrasions in the  bladder mucosa.   ¢ Acute retention of urine due to the calculus obstructing  the internal meatus.    Fig. 40.3: Bladder stone with copper-T (see clinical notes in page  970)    Radiography: In 90% of cases, the stone is visible.  However, it 1s important to look for stones in the entire  urinary tract.   A click can be heard when the stone comes into contact  with the instrument.    Ultrasound lithotripsy — very safe, but only for small stones.  Laser lithotripsy (Holmium laser)—can break most large  stones.    By introducing a cystoscopic lithotrite, stone is grasped  firmly and broken. Small fragments of stone are evacuated  by using evacuator.    1. Urethra: Obstruction such as stricture, enlarged prostate.  2. Bladder: Cystitis, contracted bladder, carcinoma.    Can be done when the stone Is too big, too hard to crush or  too soft.    Incidence is more in aniline dye workers. Products such  as benzidine and 3-naphthylamine are carcinogenic.  Cigarette smoking   Chronic irritation by stones, catheter, also can produce  carcinoma: 95% of the tumours originate in mucous  membrane.    Bilharziasis or schistosomiasis increases the chances oi  bladder cancer (squamous cell carcinoma).   a. The villi are stunted, swollen and resemble cauliflower.  They are slow growing.   Severe cystitis like symptoms occur in carcinomatous ulcer.  Later painful, blood-stained micturition can occur.  Strangury: Painful micturition with bleeding and  incomplete emptying of bladder.   Loin pains due to ureteric obstruction with hydronephrosis.  Suprapubic pain, groin pain, perineal pain are due to infiltra-  tion of nerves. This indicates advanced nature of the growth.    . IVP: Filling defect in bladder, dilatation of ureter can be    a bladder carcinoma (Figs 40.7 and 40.8). It can also detect  liver metastasis.    the spread of the disease (Figs 40.5, 40.6 and 40.9 to 40.11).  ¢ Specially it is useful to know the infiltration of the muscle,  perivesical tissue and also the prostate and pelvic wall.    the lesion and to take biopsy which 1s very much required  for further management of the case (Key Box 40.2).   Fig. 40.5: CECT abdomen and pelvis showing a large tumour ir  the left lateral wall of bladder with left hydroureteronephrosis   Fig. 40.6: CECT abdomen and pelvis showing a large tumour in Fig. 40.7: Ultrasound suggesting carcinoma urinary bladder  the right lateral wall of bladder with right hydroureteronephrosis   Fig. 40.8: Ultrasound suggesting carcinoma urinary bladder Fig. 40.9: Plain CT of the bladder showing filling defect in the  (another case) bladder    e Haematuria with normal IVP  e Lower urinary tract symptom  e Malignant cells in the urine cytology.   Fig. 40.11: Contrast CT of the bladder showing filling defects in  two places suggestive of carcinoma bladder    I. Carcinoma not involving muscle layer (Tis Ta, T1!)  a. Transurethral resection (TUR) of tumour (resected base  to be screened for tumour by microscopy).  b. Postoperative intravesical chemotherapy with Thiotepa/  Adriamycin/Mitomycin retained inside the bladder for  ] hour. Such 6—8 courses at weekly interval are given to    6. Bimanual palpation, rectoabdominally in males and  vaginoabdominally in females is done under general  anaesthesia. Thickening of bladder wall, mobility, fixity  and hardness can be made out.    1. Clinical staging: Jewett, Strong and Marshall system  ¢ Clinically the tumours are broadly classified into three  groups: Superficial or noninvasive, infiltrating or  Invasive and carcinoma in Situ.    reduce recurrence.  c. BCG or interferon immunotherapy given postoperatively  intravesically to prevent tumour recurrence.    T2—-T4 lesions: Radical cystectomy followed by  systemic chemotherapy (MVAC: Methotrexate,  Vinblastine, Adriamycin, Cisplatin) (vide infra).  Radical cystectomy: Removal of the bladder with  pericystic fat and the prostate and seminal vesicles, urethra  in men and bladder and pericystic fat, cervix, uterus,  anterior vaginal vault, urethra and ovaries In women. It 1s  a major surgery with 3 to 8% mortality rate.    Any T,N1, MO orany T, NO, M1—Systemic chemotherapy  (MVAC) followed by radiation therapy has to be given.    Small lesion involving muscle in the vault of bladder or  posterolateral wall of the bladder, partial cystectomy  (segmental resection) of that part of the bladder containing  the growth with a wide margin of 2—3 cm. This should be  followed by intravesical chemotherapy.    ¢ A rare congenital anomaly seen in 1: 50,000 births  ¢ Male: Female =4: |    ¢ This occurs due to failure of development of lower  abdominal wall and anterior wall of the urinary bladder.   ¢ Asa result, the posterior bladder wall 1s seen protruding  out below the umbilicus. Hence, it is exostrophy of the  bladder.    1. Complete: Pubic symphysis is not formed, complete  epispadias in male or bifid clitoris in female.   ¢ Posterior bladder wall is seen in the lower abdomen as  a pink to red mucosa, partially inflamed.   ¢ Pubic symphysis is widely separated. It has the advantage  in female patients in that it facilitates the delivery.   ¢ In female children—umbilicus is absent, external  genitalia are poorly developed, and clitoris is bifid.   Fig. 40.12: Carcinoma bladder in a case of ectopia vesicae.  (Courtesy: Dr PS Aralikatti , Associate Professor, BIMS, Belgaum,  Karnataka)    1. Renal failure due to recurrent UTI  2. Adenocarcinoma of bladder and in early age (Fig. 40.12)  3. Ammoniacal dermatitis of the skin.   e Posterior bladder wall is seen in lower abdomen  e Pubic bone is widely separated    1. Incomplete: Reconstruction of the anterior wall of the  bladder with reconstruction of the bladder sphincter.   2. Complete: Total cystectomy followed by urinary diversion  by implantation of ureters in the sigmoid colon  (ureterosigmoidostomy) followed by reconstruction ot  anterior abdominal wall if the patient has urinary  Incontinence.    Acute uncomplicated bacterial cystitis predominantly affects  women. By definition these are infections occurring in the  absence of any anatomic or functional abnormality of the  urinary tract. The ascending taecal—perineal—urethral route  is the primary source of infection. Men are somewhat protected  from ascending infection because of long urethra and  antibacterial properties of prostatic secretions.    80% of bladder infections in women are caused by E. coli  followed by other gram-negative organisms such as K/ebsiella  and Proteus species.   Irritative voiding symptoms (frequency, urgency, dysuria) are  the hallmarks of cystitis. Low backache and suprapubic pain  are other complaints. Fever and other constitutional symptoms  are usually present. Physical examination is frequently  unremarkable except for suprapubic tenderness.   * Urinary microscopy is the mainstay of diagnosis.  Diagnosis is strongly considered positive 1f microscopy  shows > 5 WBCs/high power field in females and 2-3  WBCs/high power field in males.   ¢ Urine culture not only confirms the diagnosis but also  identifies the causative organisms.    ¢ Other tests and imaging studies are not indicated in an  uncomplicated infection, unless patient presents with  recurrent episodes.    ¢ Antibiotic therapy based on the culture and sensitivity report  given for a period of 7—10 days, 1s curative.   ¢ Symptomatic treatment in the form of antipyretics, urinary  analgesics and antispasmodics may help.   ¢ Congenital (situated midline anterosuperiorly): Rare and  usually symptomless. They represent the unobliterated  vesical end of the urachus. May require excision if chronic  infection persists.    ¢ Acquired: They are pulsion diverticula and occur due to  bladder outflow obstruction.    ¢ Symptoms of recurrent urinary infection: Suprapubic  pain, frequency of micturition, fever with chills, etc.   ¢ Symptoms of pyelonephritis: Backache, fever and renal  angle tenderness, etc.   Fig. 40.13: Bladder diverticulum arising from the posterior wall.  Ureter is seen entering the diverticulum    Investigations  1. Cystoscopy: Full bladder distension 1s necessary to search  for diverticulum.  2. Intravenous urography: It can detect the site of  diverticulum. [tcan also detect hydronephrosis (Fig. 40.13).  3. Ultrasonogram  ¢ It can detect residual urine  ¢ Itcan detect diverticulum  ¢ It can detect associated stone   e Recurrent urinary infections  e Bladder stone can occur and it may give rise to haematuria  due to    Urinary fistulae are not an uncommon problem encountered  by surgeons. Broadly classified into congenital and acquired.  Congenital causes are a few of them (Key Box 40.5) which  have been discussed in more detail in their respective chapters.  More important ones are acquired fistulae which are given  below. Among these vesicovaginal fistula 1s discussed in more  detail.    e Ectopia vesicae  e Patent urachus  e Association with imperforate anus    Leakage due to necrosis of tissues manifests usually after  7 days.    ¢ Leakage of urine from vagina  ¢ Excoriation of the vulva   ¢ Digital vaginal examination may reveal thickening on the  anterior wall of vagina.   ¢ Swab test: Methylene blue is injected into the urethra and  if vaginal swab is coloured blue, it is vesicovaginal fistula.    ¢ Low fistula: Transvaginal repair  ¢ High fistula: Suprapubic approach and repair.    ¢ Tuberculosis causes caseation and may result in fistula in  the loin    ¢ [Initial symptoms are increased frequency and pain 1s  relieved by micturition and aggravated by overdistension  of bladder.   ¢ There is severe fibrosis of the urinary bladder due to  pancystitis, resulting ina small thimble bladder. (In India,  tuberculosis must be considered.)   ¢ Frequency of micturition and pain due to decreased bladder  capacity are the features. It causes sterile pyuria.   ¢ It is called as urinary bilharziasis.  The disease is caused by embryos (cercariae) of  schistosoma, which enter the body through penetration of  the skin and reach the bladder via the portal vein in a  retrograde manner. In the bladder, ova are released which  are excreted back into the fresh water via the urine. Fresh  water snail is the intermediate host.   ¢ Multiple pseudotubercles, nodules, granulomas, fibrosis are  the prominent pathological features.   ¢ Treated by long-term praziquantel and surgery may be  required (ileocystoplasty).    Patients with lower urinary tract cancers or severe functional  or anatomic abnormalities of the urinary bladder may require  urinary diversion.   The most commonly used method of urinary diversion 1s  by incorporating various intestinal segments into the urinary  tract. Virtually every segment of the intestinal tract has been  used.   1. Ileal conduit: 18—20 cm of ileum ts used as a conduit.   Ureters are directly implanted into it. The end of the ileal  conduit is brought through lateral aspect of rectus abdominis  muscle and stoma is made.  This simply acts as a conduit carrying urine from the renal  pelvis or ureter to the skin, where urine is collected in an  appliance attached to the skin surface. It is not a continent  mechanism.   2. Ureterosigmoidostomy is an example of continent urinary  reservoir, Wherein ureters are anastomosed into the sigmoid  colon.    The most worrisome complication of this procedure is  development of adenocarcinoma, at the site where ureters  are implanted.   ¢ Newer method of continent diversion—orthotopic  bladder substitution.  3. Nephrostomy: It 1s required for drainage and decom-  pression of the upper urinary tract and 1s indicated in  following situations:  ¢ Retrograde ureteral catheterisation 1s not advisable (e.g.  In sepsis secondary to ureteral obstruction).   ¢ Retrograde ureteral catheterisation 1s impossible (e.g.  complete ureteral obstruction by stone, tumour or  stricture).    4. Uretero-ureterostomy: It means anastomosis of ureter to  ureter following resection. It can be on the same side, if the  resection length of the ureter 1s 4 cm or less or to the opposite  side ureter, if the ureter excised is more than 4 cm in length.    Indications  a. Trauma to ureter  b. Ureteric involvement by neoplastic conditions, e.g.  colonic carcinoma, which requires resection of ureter.  ¢ Mainly indicated for upper and midureteral involve-  ment. The procedure of choice for lower ureteric  involvement ts reimplantation into the bladder.    1. Surgical (iatrogenic): Bladder can be injured mostly during  pelvic surgery, 1.e. excision of the rectum or during  gynaecological procedures, etc.   2. Trauma: Blunt injury abdomen due to road traffic  accidents.  ¢ Kick or blow on abdomen, with a full bladder  ¢ Penetrating injury (very, very rare).    When there 1s surgical trauma or trauma to a distended bladder  the rupture will be intraperitoneal (Fig. 40.14A).   ¢ Lower abdominal guarding and rigidity occur after a few  hours of injury.   ¢ Even though the patient has not passed urine for a few hours  there 1s no desire to micturate.    ¢ Trauma either penetrating or blunt injury with fracture of  pubis gives rise to this type of injury.   Difficult to distinguish clinically from an injury to the  membranous urethra.   2. IVP: Extravasation of the dye into the peritoneal cavity or  extraperitoneally.    1. Intraperitoneal rupture: Laparotomy, repair of the bladder  in two layers with vicryl. Drain the suprapubic space with  a tube drain. Anindwelling urethral catheter has to be placed  for 10 days to 2 weeks to keep the bladder decompressed.    2. Treatment of extraperitoneal rupture: Extraperitoneally  expose the bladder with a suprapubic midline incision and  repair of the bladder. Drainage, as mentioned above, has to  be carried out.   ¢ Male urethra ts divided into anterior urethra (bu/bopenile)  and posterior urethra (prostatomembranous urethra).   ¢ Male urethra functions as a conduit for urine and semen.  Anterior urethra is covered with erectile tissue of corpus  spongiosum. Anterior urethra penetrates the urogenital  diaphragm to enter the pelvic cavity as prostatomembranous  urethra.   ¢ Since its margins are attached to the perineal membrane it  is vulnerable to tear at this point in pelvic bone fracture.    ¢ Veins drain into Santorini’s plexus around the bladder neck  and prostate.   ¢ Prostatic urethra has 2 sphincters at each end. Internal  sphincter at the bladder neck is composed of smooth muscle  fibres. The external sphincter is a rhabdosphincter, about  2 cm long, surrounding the membranous urethra.   ¢ Normally, urinary continence is maintained by the external  sphincter. When the external sphincter is damaged  (e.g. trauma, postsurgical) the internal sphincter maintains  continence but to a lesser degree.   ¢ Continence ts not affected by ablation of internal sphincter  (e.g. post-transurethral resection of prostate (TURP) but it  results in retrograde ejaculation, 1.e. the semen goes back  into the bladder instead of exiting through the urethra.    ¢ The most common urethral injury  ¢ Urethra angulates 1n the perineum, where it gets injured.  ¢ Superficial extravasation of urine.   3. Distended bladder: Diagnosed by percussion over  suprapubic region which gives dull note.   ¢ Should be shifted to the operation theatre and with aseptic  precautions a catheter is passed gently, if it enters the  bladder, it is kept in place for 2 weeks and perineal  haematoma Is drained.   ¢ If catheter does not reach the bladder, an incision is made  in the perineum and the catheter is guided into the urethra.   ¢ If all the measures fail, emergency suprapubic  cystostomy/catheter is done to drain the urine and later  repair of urethra 1s undertaken.   ¢ There may be disruption of pelvic bones, fracture symphysis  pubis, with avulsion of the puboprostatic ligament leading  to floating prostate.    1. Complete transection results in floating bladder. In t  condition, urethra is completely transected at the apex  the prostate. As the puboprostatic ligament is avulsed, |  prostate falls back and migrates upwards. On rec  examination prostate is felt as though it 1s floating  Floating prostate (Vermooten’s sign).   3. Associated with injury to bladder: Extraperitoneal ruptu  of the bladder is seen here (intraperitoneal rupture of bladd  occurs in a distended bladder).    ¢ In cases of extraperitoneal rupture of the bladder, it wi  not be palpable due to extravasation of urine into perineun    |. X-ray pelvic bones may show a fracture or separation 0:  pubic symphysis.   2. Ascending urethrography (ASU) to confirm the rupture  (Figs 40.16 and 40.17).   3. Once the stricture develops, voiding cystourethrogram  (VCUG) is done to know the exact location of the stricture.    Treatment (Figs 40.18 and 40.19)  ¢ Urgent blood transfusion to treat shock    ¢ A bougie/sound is passed from above and another  similar sound 1s passed through the external meatus  (penis). When the two meet, aclick 1s appreciated. With  both sounds in contact, the sound from bladder ts  withdrawn slowly. The lower one 1s advanced at this  stage and 2nd sound appears in the bladder. A red rubber  catheter is tied to it and the sound 1s withdrawn through  external meatus.    ¢ To this red rubber catheter which is seen outside, a  Foley catheter is tied and is drawn into the bladder and is  kept in place for 15 days. This is called rail roading  technique.    . Extravasation of urine into scrotum, beneath superficial  fascia of the penis, beneath Scarpa's fascia   ¢ Within 48 hours of exposure to the venereal disease  gonorrhoea, there is involvement of periurethral glands.  They are concentrated more in the bulbar urethra. Hence  strictures are more in bulbar urethra.   ¢ |t causes periurethral fibrosis, resulting in multiple dense  strictures within | year of infection but may not cause  difficulty in micturition for 10-15 years (Key Box 40.7).    Gleet: White flakes due to desquamated urethral  epithelium in the early morning urine   Fig. 40.18: Algorithm for management of bulbar urethral rupture (Courtesy: Dr Vikas Jain, Asst Professor, Department of Surgery,  KMC, Manipal, 2007-2008)    : Rupture urethra a  | (may be associated with bladder rupture)    Fig. 40.19: Algorithm for management of posterior urethral rupture (Courtesy: Dr Vikas Jain, Asst Professor, Department of Surgery,  KMC, Manipal)   Fig. 40.20: ASU and VCUG showing | Fig. 40.21: ASU: Total cut off seen due Fig. 40.22: ASU with MCU showing  stricture urethra to stricture urethra Stricture    Previous history of exposure to gonorrhoea, history of  instrumentation or history of injury to urethra is usually  present.  ¢ Common in young age (20--40 years).  ¢ History of straining while passing urine.  e Suprapubic pain and swelling due to distended bladder.  e Stricture urethra may be felt in the perineum as a button    Usually cut at 12 0’ clock position.  1. Visual internal urethrotomy (VIU) by using urethrotome    2. Open method Is indicated in long strictures not responding  to less invasive procedures. They are grouped under  urethroplasty.    1. Acute retention of urine either following alcohol or due to  postponement of micturition.   3. Recurrent periurethral abscesses (multiple) which rupture  and open externally in the perineal skin. When sucha patient  is asked to pass urine, urine can be seen coming out of  multiple holes in the perineum (Watercan perineum).    In this condition, some portion of distal urethra is not  developed, as a result of which external meatus ts situated in  the under surface of penis. Usually this 1s associated with  chordee and hooded prepuce.    ¢ In this external meatus is situated few mm away from  normal site within the glans.    ¢ It occurs due to failure of development of urethra which  runs in the glans penis.   ¢ As a result of this, urethra opens at the corona glands-  junction of glans and shaft of penis.    ¢ Both these varieties do not give major problems  functionally. It can be left alone without treatment.    ¢ In this the external opening Is situated somewhere in the  under surface of the penis.   ¢ Urethral opening is seen in between two halves of the  scrotum and often it 1s split.    e In such cases it is difficult to differentiate the sex of the  child   e Miuicturition: Stream is good, but it wets the clothes in 3rd  and 4th varieties.   ¢ Chordee: Many of the cases are associated with bending of  penis.    |. One-stage urethroplasty  ¢ Chordee correction: Always confirm by inducing  artificial erection  ¢ Urethral tube formation by tubularising urethra  e Inner prepuceal island tube urethroplasty.  2. Two-stage urethroplasty  ¢ When the child is 6—12 months old, chordee is corrected  by straightening the penis. This is called orthoplasty.    968  ¢ When the child is 5—6 years old, reconstruction of the  urethra is done by using locally available skin either from   I. Acute retention of urine  A. In males  ¢ Benign prostatic hypertrophy (BPH): In elderly  patients over 50 years of age.  ¢ Stricture urethra: In young patients  ¢ Postoperative retention of urine: Operations like  haemorrhoidectomy, fistulectomy, etc. produces reflex  spasm of internal sphincter, which precipitates retention  of urine. Management of such cases 1s given in Key  Box 40.8.  B. In females  ¢ Hysteria  ¢ Retroverted gravid uterus  ¢ Urethral stenosis    Normal—30 ml  >50—abnormal/significant  >200 in BPH requires surgery  C. In children  ¢ Meatal stenosis due to meatal ulcer with scab (due to  scratching habit of child).  D. In general  ¢ Spinal anaesthesia  ¢ Spinal injuries  ¢ Blood clot in the bladder following prostatectomy  ¢ Bladder stone in school-going children: Pain referred to  tip of penis  ¢ Acute urethritis and acute prostatitis due to bacterial  infection  ¢ Faecal impaction in the rectum    Ee.  POSTOPERATIVE RETENTION OF URINE:  TREATMENT  . Hot water fomentation to the suprapubic region  . Provide privacy  . Run a tap nearby    . Make the patient to stand and pass urine  . Catheterisation should be done as the last resort    ¢ Contracture of the bladder neck  ¢ Urethral calculus  ¢ Drugs: Atropine, carbachol, bethanechol.    II. Chronic retention of urine (Key Box 40.9)  1. Benign prostatic hypertrophy  2. Bladder neck contracture  3. Stricture urethra   ¢ It is one of the common causes of vesicoureteric reflux  and hydronephrosis in infants.    ¢ Bladder wall is thickened due to obstruction and  hypertrophy. The urinary bladder is palpable, hard and felt  in the suprapubic region—cricket ball bladder.    e It is also one of the common causes of renal failure in  infancy and childhood.    ¢ Micturating cystourethrography: Dilated proximal  urethra is highly suggestive of posterior urethral valve.  It is a diagnostic investigation (Figs 40.24 to 40.27).    ¢ In very ill patients, initially vesicostomy is done to improve  renal function and stabilise the patient. Fulguration is done  after 1-2 weeks.   ¢ These patients should be monitored for renal failure by serial  creatinine measurements.    Fig. 40.27: Micturating cystourethrogram showing bilateral  grade V vesicoureteric reflux (VUR)    Vesicoureteric reflux is the retrograde flow of urine from the  bladder into the kidneys.    Normally urine flows from the kidneys into the bladder  and backward flow is prevented by a complex anatomy at the  vesicoureteric junction, most importantly a good length of  submucosal tunnel of ureter. Lack of adequate submucosal  tunnel leads to VUR.    The VUR can be of mild to severe grades (graded I-mild  to V-severe).    ¢ Child might be diagnosed to have hydronephrosis detected  antenatally, depending on the severity of reflux.    ¢ Recurrent UTI: Due to increased amount of residual urine.  When the child voids, some urine refluxes back into the  kidneys, which comes back into bladder after voiding  ceases. This will lead to a high post-void residue (PVR),  causing recurrent infections.    ¢ Pyelonephritis and scarring: Reflux of infected urine  causes pyelonephritis. It can be acute or chronic. Recurrent  episodes can lead to scarring of kidneys and decreased  function, proteinuria and hypertension. Severe scarring  bilaterally can lead to chronic renal failure (CRF) and end-  stage renal disease (ESRD).   ¢ Complete blood picture: Anaemia in CRF and leukocytosis  in acute pyelonephritis.   ¢ Renal function tests: Increased urea and creatinine in renal  failure.   ¢ Ultrasonography (USG): Renal size, parenchyma and  hydroureteronephrosis with PVR can be assessed.   ¢ Micturating cystourethrogram (MCU) or voiding  cystourethrogram (VCUG)—investigation of choice for  diagnosis. Procedure: Bladder 1s filled with contrast after  catheterisation and X-rays taken during filling and full  bladder to look for reflux. After full bladder, catheter is  removed, patient 1s asked to void and X-rays are taken in  the voiding phase.   ¢ DMSA scan—for extent of renal scarring and function.  Helps in choosing the management strategy (medical vs  surgical).   I. Conservative management: Low grade reflux (grades  I-III) can be managed conservatively. The aims of  conservative therapy include:    ¢ Regular or timed voiding and double voiding to keep PVR  as low as possible.    II. Endoscopic management: Injection of a bulking agent  to provide support to VUJ, helps in a few cases.    Iil. Surgical management: Ureteric reimplantation is done  for severe reflux. The principle of surgery is to lengthen  the submucosal tunnel. If one kidney is poorly  functioning, nephrectomy can be done.    in some cases, bilaterally scarred, nonfunctioning kid-  neys can lead to ESRD. These patients require renal transplan-  tation.    A 45-year-old multiparous female patient was diagnosed to  have bladder stone. She underwent vaginal examination  because she also had pain in her genitalia.She underwent  dilatation and curettage.On dilatation, tail of Cu-T with  thread was seen in the uterus. On trying to remove Cu-T; only  thread could be extracted. She underwent laparotomy. Uterus  was found plastered to urinary bladder. On opening the  bladder, a stone of 6 X 6 cm with Cu-T in pregnated in it was  present. The Cu-T had perforated anterior wall ofuterus and  posterior wall of the bladder. It was removed. (Courtesy: Prof  Rajiv Shetty, Professor Shivaswamy, Professor Durganna,    e All the topics have been updated  e Vesicoureteric reflux has been added  e New images are added   B. It is innervated by adrenergic fibres  C. It prevents retrograde ejaculation  D. It is supplied by pudendal nerve    risk factor for carcinoma urinary bladder?  A. Bilharziasis  C. Leishmaniasis  Following are true for carcinoma urinary bladder  except:   D. Adenocarcinoma of the bladder is due to bilharziasis  Following are true in ectopia vesicae except:    Which of the following is not the feature of intra-  peritoneal rupture of the urinary bladder?   Which is the narrowest portion of male urethra?  B. External meatus    Which of the following is not part of the triad of  rupture of bulbar urethra?    What is the first advice given to the patient in rupture  bulbar urethra?    Primitive buds given from urethra form the glandular tissue  and surrounding mesenchyme forms the fibromuscular  stroma. Developmentally, the prostate has 5 lobes: Anterior,  posterior, 2 lateral and | middle lobe.   Middle lobe (median lobe) is situated 1n between the two  ejaculatory ducts and the urethra. The enlargement of this  lobe in benign hypertrophy of the prostate is responsible  for the obstruction of urethra. This lobe enlarges upwards  into the bladder (Fig. 41.1).    In benign prostatic hypertrophy (BPH), the glands of the  inner adenomatous zone hypertrophy and lead to urinary  outflow obstruction. Carcinoma usually occurs in the  outer nonadenomatous zone.   Urethral glands open into the prostatic urethra. These  submucosal glands are responsible for BPH.   When the prostate enlarges, it compresses the outer zone  resulting in a false capsule.   The outermost zone is the zone of prostatic glands proper  which is responsible for carcinoma of prostate (Fig. 41.2).   Fig. 41.2: Prostate: (A) Outer carcinomatous zone, (B) Inner  adenomatous zone    ¢ Surrounding this, there is fascia of Denonvilliers which is  a part of pelvic peritoneum.   ¢ Between the anatomical capsule and pelvic peritoneum,  prostatic venous plexus 1s present which may give rise to  massive haemorrhage, if injured.    1. Hormonal theory  ¢ It has been compared to fibroadenosis in female patients.  ¢ Asthe age advances, the levels of androgens come down.  There 1s a corresponding increase in the oestrogen which  stimulates the prostatic gland and produces BPH.    2. Neoplastic theory  According to this theory there is proliferation of all the  elements of prostate: Fibrous, muscular and glandular  resulting in fibromyoadenoma.    1. Urethral changes  ¢ Urethra gets compressed, elongated and gets converted  into a narrow, longitudinal slit.  ¢ The effect is more with median lobe enlargement which  is due to enlargement of subcervical glands.  * Lateral lobes enlarge when there is involvement of  submucous glands.  2. Changes in the bladder (Fig. 41.3)  ¢ As a result of obstruction, the bladder musculature  undergoes hypertrophy. Very prominent thick bundles  of the muscle can be seen, which are called fasciculations  or trabeculations.   Fig. 41.3: Secondary changes in the urinary bladder due to BPH   ¢ In between the fasciculations, there are depressed areas  which are called sacculations.   e Since the sacculi are thin, as the pressure increases,  herniation occurs outside resulting in diverticull.   ¢ In the diverticuli, there is stasis of urine resulting in  secondary infection and stone formation.    3. Changes in the ureter and kidney (Key Box 41.1)  Bilateral hydronephrosis and bilateral hydroureter are  the end result of BPH, which may result in renal failure.    — Frequency: To start with, frequency is present during  the day time followed by day and night (5—10 times  during the night). It is due to ineffective emptying of the  bladder. It results 1n residual urine in the bladder  precipitating cystitis.    — Urgency: As the prostate enlarges there is vesical  introversion of sensitive mucous membrane of  prostatic urethra within the bladder. This causes the  internal sphincter to stretch and prevents contraction. This  results in a few drops of the urine trickling down the  posterior urethra resulting in an urgent desire to pass urine  (urgency).   ¢ Haematuria is rare  — It is due to congestion of prostatic venous plexuses  resulting in hyperaemia and haematuria.  ¢ BPH with acute retention of urine  — This occurs due to postponement of micturition,  following alcohol or drugs like mydriatics.  ¢ BPH with chronic retention of urine    — Many of the patients present with chronic retention of  urine, with painless enlargement of the urinary bladder.    Digital rectal examination: Enlarged lateral lobes can be  easily felt. Rectal mucosa ts free (In an enlarged prostate gland,  in case of carcinoma of prostate, the mucosa of the rectum  cannot be moved if it has infiltrated into the rectum.).    I. The prostatic lobes protrude minimally into the rectal  lumen by I—2 cm, the median sulcus 1s palpable.  II]. Prostatic lobes protrude > 2 cm but < 3 cm into the rectal  lumen and the median sulcus ts obliterated.  II]. 3-4 cm protrusion  IV. > 4 cm protrusion of lobes, most of the rectal lumen is  filled by the projecting prostatic lobes.    2. Uroflowmetry: The person is asked to void urine from his  full bladder into the flowmeter. The flow rate is assessed.  Peak flow rate  ¢ Normal peak flow rate: 20 ml/sec.  ¢ Doubtful peak obstruction: 10 to 15 ml/sec.  ¢ Definite peak obstruction: Less than 10 ml/sec.  ¢ Thus, the degree of bladder outlet obstruction (BOO)   3. Ultrasonogram: To assess the size and weight of prostate,  to assess the residual urine and to look for hydro-  ureteronephrosis, bladder wall changes.    TREATMENT  It can be classified into medical treatment and surgical  treatment.    If the patient has mere frequency of micturition and if the  residual urine 1s not much (< 150 ml), uroflowmetry shows  more than 15 ml/sec of urine flow and if there are no back    pressure effects on the kidney, the patient can be reassurec  advised to avoid heavy alcohol consumption which may lea  to prostatic congestion and acute retention of urine. To avo!  over distension of the bladder, he has to void the urine as an  when he feels the urinary sensation of micturition, and shoul  not postpone micturition.    a. Finasteride acetate 5 mg daily for 6 months. It is a 50  reductase inhibitor. It helps in prevention of hyperplasi:  of the prostate. It is given for large prostates.   b. a-adrenergic blockers: It is supposed to relax the interna  sphincter for better drainage of the bladder. They are give  when the prostate 1s less than 40 g (small) as measured b‘  ultrasound. Tamsulosin (most selective), terazosin, alfazocit  are a few drugs used today.   2. Chronic retention of urine with postvoid residual urine  more than 200 ml.   3. If frequency of micturition 1s so much that it disturbs the  normal lifestyle during daytime.   4. Complications such as haematuria due to congestion of  prostatic venous plexuses, hydroureteronephrosis, prostatic  diverticulosis, vesical calculus and recurrent infections.    1. Transurethral resection of the prostate (TURP)  ¢ This is the most popular method today.  ¢ A resectoscope Is passed through the urethra and under  vision with constant irrigation with water or glycine, the  prostate 1s resected into multiple pieces and removed.  ¢ Haemostasis is obtained with the help of a cautery.    2. Incontinence is rare because the chances of damage to  the internal sphincter are very low.    Disadvantages  1. TURP syndrome with water intoxication and electrolyte  imbalance, if water is used as irrigating fluid.    2. If there is BPH with diverticuli and stone, TURP has to  be followed by litholapaxy.    ¢ This method is now restricted to glands more than  100 g in weight and associated with calculus.    * Through an extraperitoneal approach the bladder is  opened, prostate is enucleated with finger, bleeding  1s controlled by inflating the Foley bulb with about 30-  50 ml of air and by ligatures.   ¢ Bladder is drained by a Malecot’s catheter which is wider  than Foley’s so that it can drain well if the bleeding occurs  in the bladder.   ¢ After about 7-10 days, a tract develops along the length  of Foley catheter which heals by granulation, fibrosis  and forms the future prostatic urethra.    Disadvantages  ¢ Blind resection  ¢ Chances of haemorrhage are more  ¢ Stricture of prostatic urethra  3. Retropubic prostatectomy  Done by extraperitoneal approach without opening the bladder,  pushing the bladder to one side and excision of the prostate.  4. Perineal prostatectomy  Not done nowadays  Newer treatments  ¢ Holmium: YAG laser.  ¢ Intraurethral stents—in men who are grossly unfit (ASA  Grade IV) for surgery.    ¢ Carcinoma of the prostate is common after the age of  65 years. The incidence increases with age.   ¢ In Western countries, it is the second most common type  of carcinoma in males after 65 years, first being  bronchogenic carcinoma.   ¢ Prostatectomy done for BPH does not give protection  against development of carcinoma of prostate because  during prostatectomy the outer zone is left undisturbed (not  resected).    1. Histological surprise  Prostatectomy done with diagnosis of BPH but histology  reveals carcinoma of the prostate.   2. Multiple bone pain, confused for rheumatism, is due to  multiple metastasis.   3. Rectal examination: Reveals a hard nodule on the anterior  wall of the rectum, obliteration of median sulcus. The rectal  mucosa cannot be moved over the prostate but it is not  ulcerated (fascia of Denonvilliers prevents the spread of  carcinoma prostate into the rectum).   4. Elderly man with bilateral sciatica with metastasis in the  thoracolumbar vertebrae.   5. Acute retention of urine occurs in 5—10% of cases of  carcinoma of prostate.    6. Difficulty in passing urine, painful micturition and  sometimes with haematuria is due to involvement of  prostatic urethra.   ¢ This is due to retrograde tumour embolisation which  occurs through prostatic venous plexus which  communicates through the emissary veins with the bone  (Batson’s paravertebral plexus of veins).  Peculiarities of secondary deposit from carcinoma of  prostate:   ¢ When spread occurs along seminal vesicle, external iliac  nodes are enlarged.   ¢ From this group of nodes, para-aortic nodes, mediastinal  nodes, followed by left supraclavicular nodes get  involved.    On the medial side it can involve the prostatic urethra  and give rise to retention of urine.  When it spreads upwards, the bladder can get involved   . T1a, T1b and T1c. These are incidentally found tumours in  a Clinically benign gland after histological examination of a  prostatectomy specimen. T1a is a tumour involving less than  5 per cent of the resected specimen; these tumours are  usually well or moderately well differentiated. T1b is a tumour  involving > 5 per cent of the resected specimen. Tic tumours  are impalpable tumours found following investigation of a  raised PSA.   . T2a disease presents as a Suspicious nodule on rectal  examination confined within the prostate capsule and  involving one lobe. T2b disease involves both lobes.   . T3 tumour extends through the capsule (T3a, uni- or bilateral  extension. T3b, seminal vesical extension).    . x-ray of bones (Figs 41.6 and 41.7)  Which are likely to be involved (already mentioned).    concentrated in the prostate which are responsible for  acidic pH in the prostatic urethra.    ¢ Normally they are drained in the urine so that they are    ¢ In carcinoma prostate because of the ductal blockage;  it gets absorbed into the blood and thus high levels are  reached, especially with metastasis.   ¢ Perrectal examination should not be done before drawing  the sample of blood.    Significance of acid phosphatase  Levels come down with the treatment of carcinoma of  prostate, especially when bone metastasis disappears.    4. Serum alkaline phosphatase  It is increased if there is extensive liver metastasis or bone  metastasis.    5. Prostate-specific antigen  ¢ Prostate-specific antigen (PSA) is a neutral protease,   [fit is more than 4 nmol/ml, carcinoma is to be suspected;   ¢ The highest concentration of PSA occurs in the lumen  of the prostatic acini and ducts (up to million times greater  than in systemic circulations). The prostatic luminal cells  are normally surrounded by basal cells, prostatic  basement membrane and prostatic stroma.   ¢ A number of diseases disrupt some of the barriers to  absorption resulting in elevation of serum PSA, notably  prostatic cancer, prostatic inflammation and  infarction. PSA is also transiently elevated (up to 24  hours) after ejaculation and cycling.   ¢ PSA measurement is the most efficient screening test for  prostate cancer and it increases further if the measurement  is combined with digital rectal examination (DRA).   ¢ PSA measurement is also vital in staging prostate cancer  and assessing response to treatment.    7. CT scan or MRI scan  These are done before proceeding on to radical surgery to  assess the extent of the tumour.   8. Bone scan  Is indicated in cases of carcinoma prostate especially who  have bony pains, raised alkaline phosphatase and also very  high PSA levels (> 20 ng/ml) (see clinical notes).   A 75-year-old gentleman was admitted to the hospital  because of giddiness and syncope. He was a diabetic.  Initially, it was thought to be due to hypoglycaemia.   Investigations revealed creatinine of 7.5 mg%. Diagnosis  of renal failure was made. Urgent ultrasound revealed  bilateral hydronephrosis due to large para-aortic nodes  compressing both ureters. He underwent emergency  stenting of both ureters. Rectal examination revealed a hard  prostate. PSA was 20 nmol/ml suggestive of dissemination.   CT scan done later showed involvement of pelvic bones,  thoracolumbar vertebrae and iliac bones. It was a case of  carcinoma prostate. Retrospective analysis revealed that  the patient was having backache since the last 6 months.    It can be classified under following headings—early  malignancy and late malignancy.   ¢ Radical prostatectomy is done for Tl and T2 and in  menwithlife expectancy of > 10 years. Metastasis should  be excluded by a negative bone scan, chest radiograph  and serum PSA < 20 nmol/mL. Radical prostatectomy  involves pelvic lymphadenectomy and removal of the  prostate, seminal vesicle including the distal urethral  sphincter followed by anastomosis of urethra to the  bladder neck.   B. Early prostatic malignancy with PSA is 20 nmol/ml or  more and the patient is already beyond 65 to 70 years of  age, surgery is not favoured. Radical radiotherapy is given.    It means T3 lesions, involvement of regional nodes or presence  of metastasis.    1. Androgen ablation in the form of bilateral orchidectomy  is done as the tumour is androgen-dependent followed by    Age < 65 years Age < 65 years  General condition good   Subcapsular orchidectomy used to be done earlier wherein  tunica albuginea was left behind. It is now obsolete  because it has been proved beyond doubt that some  testicular tissue can still be left behind. Hence, a low  bilateral orchidectomy is done.    B. Drugs—(LHRH agonists, anti-androgens)  1.Oral stilboestrol therapy, dose 20-25 mg/day;  phosphorylated diethylstilboestrol (Honvon) IV initially  later, orally. Within 48 hours of treatment, dysuria  improves, pain disappears, bone pains improve and  metastasis may disappear.   ¢ Gynaecomastia: Can be prevented by preoperative  radiation to the breast—800 rads.    2. Phosphorylated diethylstilboestrol is an excellent drug  which can be given orally and IV, and the drug is not  broken down until it reaches prostate. Hence, systemic  toxicity of stilboestro] does not occur. In the prostate it  1s broken down by acid phosphatase to release stilbestrol  locally.    A. For localised metastasis in bones or brain, radiotherapy  relieves the symptoms.    B. For generalised metastasis, hemibody irradiation is  excellent to improve the symptoms and the survival time.    C. If carcinoma of the prostate is diagnosed only by a biopsy  of the prostate removed during TURP, no surgery needs to  be done. Local radiotherapy to the prostatic bed 1s  sufficient.    D. If the diagnosis of carcinoma of prostate is made on rectal  findings, surgeons have no role to play because many a  time it is an advanced cancer and there is a danger of  bleeding from prostatic venous plexus. Hence, radiotherapy  is administered by using linear accelerator (cobalt).    A. Local radiotherapy to the enlarged para-aortic nodes and  to the bone.    Prostate cancers exhibit heterogenecity within tissue, so two  histological areas of prostate are each scored between | and 5.   The two scores are added to give an overall Gleason score  of between 2 and 10. The score correlates well with the  likelihood of spread and prognosis.    ¢6 or lower—often candidates for an active surveillance  program (previously referred to as watchful waiting therapy).   e 7—uSually associated with a critical decision-making step  (usually some form of definitive therapy).   ¢ However, in both types, in addition to the prostate, seminal  vesicles and posterior urethra are involved.   ¢ Often the diagnosis is delayed because of varying symptoms  which will be attributed to a different cause and they are  being wrongly treated.    ¢ If the treatment is not effectively given, infection persists  and later it 1s difficult to eradicate.    Aetiology  ¢ Causative organisms are Escherichia coli, Staphylococcus  aureus and Staphylococcus albus.    ¢ The infection is usually due to haematogenous spread trom  a distant focus or secondary to urinary tract infection.   ¢ The patient is ill with high grade fever, chills and rigors.   ¢ Pain all over the body, more so in the back.   e Perineal heaviness or pain, rectal irritation and urethral  discharge are also the features.   ¢ Rectal examination finding: Tender, boggy enlarged  prostate. Fluctuation indicates prostatic abscess (rare).   ¢ Antibiotics such as ciprofloxacin or norfloxacin should be  given for 2-3 weeks. Otherwise, recurrent attacks of  prostatitis can occur.   ¢ If abscess is suspected, diagnosis can be confirmed by  transrectal ultrasound, it can be drained by transurethral  unroofing of abscess cavity similar in technique to  transurethral resection of prostate (TURP).    ¢ Elderly men are affected and they complain of perineal  heaviness, perineal discomfort or pain on sexual intercourse.   Prostatic massage 1s given by bidigital method—1index finger  in the rectum and the thumb in the perineum to one side. Now  the patient 1s asked to void the urine. Presence of prostatic  threads or mucopus in the postprostatic massage urine 1s  diagnostic of chronic prostatitis.    e Do not injure prostatic venous plexus (between anatomical  capsule and pelvic peritoneum) during prostatectomy or  abdominoperineal resection (APR) done for carcinoma    e Prostatectomy for BPH does not give protection from  development of prostatic carcinoma.   e Androgen ablation is the chief form of treatment in advanced  prostatic Cancers.    A. Facsiculations B. Sacculations 11. Which of the following is not true for acit  C. Diverticult D. Carcinoma phosphatase?  3. Vesical introversion of the sensitive prostatic urethra A. It 1s responsible for acidic pH in the prostatic urethr  within urinary bladder causes: B. High values suggest carcinoma prostate  A. Frequency B. Urgency C. It is elevated not only in carcinoma prostate but als  C. Hesitancy D. Haematuria other conditions  4. Following are complications of benign prostatic D. Early morning urine sample is the best to measure thi.  hypertrophy except: 12. Following are true for prostate specific antigen except  A. Stones A. Released from columnar prostatic acinar epithelia  B. Renal failure cells  C. Recurrent urinary tract infection B. More than 4 nmol/ml suggest carcinoma prostate  D. Carcinoma urinary bladder C. Prostatitis also can increase in the levels  5. Which of the following drug is used to treat benign D. It does not help in assessing the response to treatmen  prostatic hypertrophy? 13. Which of the following is not done in cases ol  A. @ adrenergic blockers carcinoma prostate?  B. B adrenergic blockers A. Radical prostatectomy  C. Oral stilboestro! B. Radical radiotherapy  D. Oral prednisolone C. High orchidectomy  6. Following are true for role of ultrasound in benign D. Stilboestrol therapy  prostatic hypertrophy except: 14. What is the treatment to follow after prostatectomy  A. Can assess the size done for benign prostatic hyperplasia is reported as  B. Can assess the weight carcinoma prostate?  C. Can assess residual urine A. Orchidectomy B. Stilboestrol  D. Can assess the flow rate C. Bisphosphonates __D. Local radiotherapy  7. Following are true for carcinoma prostate in digital 15. Following are true for acute prostatitis except:  rectal examination except: A. Usually it 1s haematogenous infection  A. Hard nodule ts felt B. Instrumentation can also cause this  B. Median sulcus ts obliterated C. Usually it 1s mild self-limiting  C. Rectal mucosa cannot be moved D. Urine sample may contain ‘threads’  D. Ulcerated mucosa 16. Which of the following is true for posterior urethral  8. The reason for early bone spread from carcinoma valves?  prostate to bones is spread through: A. They are acquired  as Balser Z fiesls ; B. Bladder ts thin walled and more prone for rupture  B. Santorini plexus of veins . .  C. Waldeyer’s plexus of veins C. Renal failure is uncommon  D. Denonvillier’s plexus of veins D. They are symmetrical valves  ANSWERS  1B 2D a B 4D 5A 6D 7D 8A 9C 10 C  ge ©) 12 D 131. 14 D te: D 1668   ¢ It consists of two corpora cavernosa and one corpus  spongiosum (Fig. 42.1).   ¢ Corpora cavernosa are vascular spaces wherein arterioles  open directly. They are corkscrew shaped (helicine  arteries) which allow their elongation in erection.   ¢ Corpora spongiosum is perforated by urethra and is  continuous distally with the glans.   ¢ Each corpus is enclosed by tough fibrous membrane—the  tunica albuginea of the corpus.   ¢ The fused fibrous sheaths are attached to the under surface  of the symphysis pubis by a triangular sheet of fibrous tissue  called as suspensory ligament. It has to be divided during  total amputation of the penis.    ¢ The artery to the bulb supplies corpus spongiosum and the  glans.   ¢ Superficial dorsal vein drains into superficial external pudendal  veins. Deep dorsal vein enters prostatic venous plexus.    ¢ Entire penile urethra is lined with transitional epithelium  except dilated anterior part in the glans—fossa  navicularis—lined by stratified squamous epithelium.    2. Secondary to chronic balanoposthitis: Balanitis means  inflammation of glans penis and posthitis means  inflammation of the prepuce. Balanoposthitis 1s common  in diabetic patients.    4. Carcinoma of the penis can present as a recent phimosis.    2. In children, ballooning of the prepuce (second bladder) can  be seen, which 1s diagnostic of phimosis.    3. Because of the phimosis, they are more prone for  balanoposthitis because of inability to clean the glans.   In this condition, the retracted skin of the glans penis (prepuce)  cannot be pulled forwards. As a result of this, the retracted  skin acts like a tight constricting agent which compresses the  corona resulting in venous congestion. As venous congestion  increases, glans swells up resulting in paraphimosis.    1. During catheterisation, if the retracted prepuce is not pulled  forwards, it results in paraphimosis.    2. Gross swelling of retracted prepucial skin and oedema of  the distal glans penis.   2. Injection hyaluronidase 250 units in 10—15 ml of saline 1  Injected into the constriction ring (prepucial skin which 1  retracted) 5—10 minutes later, when oedema Is reduced, witl  gentle manipulation it is possible to reduce thi  paraphimosis.   3. Dorsal slit is given so that reduction can be done  Circumcision follows later.    1. Ulcers of glans  2. Gangrene of glans in later stages    1. Genital warts: Buschke—Lowenstein tumour is a giant  penile condyloma and resembles squamous cell carcinoma.  It is a cauliflower-like lesion, may have foci of malignancy.    2. Erythroplasia of Queyrat or Paget’s disease of penis: a  persistent red, raw lesion which Is a precancerous condition.    3. Leukoplakia: Persistent nonspecific patch in the glans or  in the prepucial skin. Interestingly, leukoplakic patches are  not white in the penis.   4. Bowen’s disease: This presents as a small eczematous  plaque. Carcinoma in sifu is a complication which develops  within this plaque.    Fig. 42.3: This patient presented with fungating inguinal lymph  nodes. Observe the penis—he has undergone partial amputation  of the penis 2 years back.    population who do not practise circumcision. Due to the  prepucial skin, smegma collects within, which is responsible  for chronic irritation giving rise to carcinoma of penis.    Fig. 42.4: This patient had a large foul smelling proliferative lesion  in the glans penis with fungating inguinal lymph nodes. Such cases  are advanced cases with poor prognosis    . Recent phimosis due to growth underneath the prepuce.  . Haematuria, pain while passing urine indicate locally    growth with everted edges and induration of the base and  edge. The induration is much more extensive than the lesion.  Hence, entire shaft has to be examined for evidence of  induration.    . Urethra is rarely involved in carcinoma of penis because it    1S protected by the tough Buck's fascia, which ts a part of  pelvic fascia. In large fungating lesions, it may be   2. Lymphatic spread: Inguinal nodes are enlarged. 30% cases  are due to infection. Nodes are firm and tender in infection.  Hard nodes suggest metastases. Later, internal iliac and para-  aortic nodes can also get enlarged. In advanced cases the  lymph nodes may show fungation as in Fig. 42.4.    Cause of death in carcinoma penis: Erosion of femoral vessels  by inguinal lymph nodes.    1. Tumour confined to the glans or prepuce  I]. Tumour involving the penile shaft or corpora cavernosa  III. Mobile regional nodal metastases, with stage | or II  IV. Tumour beyond penile shaft, fixed regional lymph node  or distant metastases.    |. Wedge biopsy from the edge of the growth, proves the  diagnosis of squamous cell carcinoma nodes.   3. Ultrasound and MRI are helpful in lesions invading corpora  cavernosa (soft tissue details). Ultrasound guided FNAC  of lymph nodes.   4. CT scan is useful in obese patients wherein clinical  examination of inguinal nodes is difficult    It can be divided into treatment of the primary and treatmer  of the secondaries.   2. Growth involving the glans: Partial amputation with a  least 2 cm margin from the palpable indurated edge of thi  tumour.    1. Partial amputation: After having a macroscopic tumour  free, 2 cm margin proximally, if there is adequate length o  penile shaft (minimum 2.5 cm) to carry out the sexua  function and for directing the urinary stream, a partia  amputation can be done (Figs 42.7 and 42.8).    2. Total penectomy with perineal urethrostomy, if adequate  shaft cannot be retained. This is a major operation, patient  has to be clearly instructed about the consequences anc  complications of perineal urethrostomy.    1. Ammoniacal dermatitis of scrotum. To preventthis, patient  has to lift the scrotum to pass urine.    2. Stricture of perineal urethra which can be dilated by  Hegar’s dilators.  Stage lil    ¢ Circumcision, partial amputation or total penectomy  followed by ilioinguinal block dissection.    Before discussing ilioinguinal block dissection, it 1s  necessary to know the concept of sentinel lymph node  biopsy (SLNB).   The concept of SLNB was first described by Cabana in  L977.    He demonstrated consistent drainage of the penile  lymphatics into a sentinel lymph node or groups of lymph  nodes, located superomedial to the junction of  saphenous and femoral veins in the area of superficial  epigastric vein. He postulated that this sentinel lymph  node is the first to get involved in the penile malignancy  and if this sentinel lymph node 1s negative for tumour,  metastasis to other inguinal lymph nodes will not occur.  Metastasis to this lymph node will indicate the need for  complete ilioinguinal block dissection.    Technique: A dye, isosulphan blue, is injected at the site  of primary tumour. After sometime, the inguinal dissection  is done to expose the sentinel lymph node area and the  lymph nodes, which take up the dye. These are removed  and sent for pathological examination. Based on the report,    if node Is positive for malignancy, acomplete inguinal block  dissection 1s indicated. In case of negative nodes, nothing  else is required and patient is kept under regular follow-  up.   Only 50% of patients presenting with palpable lymph nodes  actually have metastatic disease, the remainder have lymph  node enlargement secondary to inflammation. So,  subjecting all the patients with inguinal lymphadenopathy  to surgery is not recommended. Hence, a course of antibiotic  is given and wait for a period of 4—6 weeks, if the nodes  are still palpable, block dissection is carried out.    However this antibiotic policy can be followed in low grade  tumour such as in sifu carcinoma and T1 lesions. If the  lymph nodes regress, wait and watch. If the grade of the  tumour Is high, do not give antibiotics. FNAC 1s done and  treat accordingly.   Also, if the nodes are not palpable, and if primary tumour  is poorly differentiated, superficial lymph node dissection  is done. If the nodes are positive, then modified inguinal  block dissection (Catalona) can be done.   Algorithm (Fig. 42.9) to help in the management of these  patients 1s given below.    Fig. 42.9: Algorithm of management of inguinal lymph node secondaries from carcinoma penis    Four weeks of antibiotics are advised after treatment of  primary to bring down the infective complications. It does  not direct the future management of nodal disease   1. Topical ointments such as 5-fluorouracil or imiquimod  cream in Tis only   3. Mohs surgery: Layer by layer complete excision of penile  lesion in multiple sessions with microscopic examination  of the undersurface of each layer.   4. Limited excision: It can be done in selected patients who  have discrete tumours, with well differentiated carcinoma  and after doing an intraoperative frozen section   5. Laser ablation: It is performed in selected patients in  conjunction with frozen section biopsies. CO, laser has been  used widely but recurrence rates are higher — about  40-50% for T] tumours. Treatment with Nd:YAG laser has  least recurrence rates. Advantages of laser treatment is the  rates of resumption of sexual activity is high   6. Modified radical inguinal block dissection (Catalona):  To minimise complications of inguinal block dissection,  following modifications have been done.    Radiotherapy + chemotherapy (cisplatin, methotrexate anc  bleomycin are the drugs used commonly).    1. Young patients who want to have a sexual life  2. Patient refuses surgery  3. Fixed/ulcerated inguinal metastasis    1. Dose: 4000-6000 cGy which can include iliac and inguinal  nodes also.   2. Interstitial radiotherapy: Iridium wires/tantalum wires  are implanted within the glans.   Aetiology  e Past trauma has been considered as one of the factors.  ¢ Venereal diseases also have been blamed.    Clinical features  ¢ Hard plaques of fibrosis can be palpated along the length of    ¢ Asa result of hard plaques, erection is not proper, and  erected penis tends to bend towards the side of the plaque.    Medical: Steroids, vit E, tamoxifen (not good results)  colchicine therapy, intralesional verapamil    Watch and observe some cases. It may recur after a few years.  Surgical: Straightening of the penis is recommended if the  deformity is distressing.    1. Nesbitt’s operation: Straightening the penis by placing  nonabsorable suture in corpora cavernosum opposite the  plaque.    ¢ The important ones being carcinoma and sexually  transmitted diseases (Figs 42.12 to 42.14).    ¢ The incubation period is an important clue followed by the  nature of the ulcer for the diagnosis.    Single, round, painless ulcer-  coronal sulcus, frenulum, glans  are the sites; base is indurated    Painless vesicle, changes  into an ulcer with  exuberant granulation  tissue; highly contagious  ulcer; bleeds but painless    Multiple, painful ulcers,  difficulty in retracting prepuce  Vesicles and pustules on    Multiple nodes above and  below in the inguinal region  form sign of groove, later  give rise to bubo and sinuses    Inguinal region may be  involved, but inguinal  lymph nodes are not  involved unless secondary  infection supervenes    Recurrent balanoposthitis  common in diabetic patients  Neuralgic pain and itching  occurs before the onset of ulcer    Size: 4 x 3 x 2.5 cm found one in each scrotal sac.  Functional unit is a lobule: 250 lobules filled with  seminiferous tubules. It has following cells and their  functions are given below:   The seminiferous tubules converge to form a rete testis,  which is connected to the epididymis through 5-7 efferent  ductules.   The serous space in front and lateral surface of testis is  tunica vaginalis (Fig. 42.15).   Blood supply is by testicular artery—a branch of aorta.  The testis gets additional blood supply from artery of the  vas and cremasteric arteries. In case of inguinal surgeries,    Collection of excessive fluid in the tunica vaginalis sac (TV  sac).    if testicular artery is accidentally injured testicula  vascularity is maintained by these two arteries. Veins for  pampiniform plexus in the scrotum.   Lymphatics: Drain via para-aortic nodes lying along the  side of aorta at the level of origin of the testicular arteries  (L2) just above umbilicus.   It has following parts—head, body and tail. Head and body  are commonly involved in tuberculosis resulting 1n posterior  sinus formation.   The head receives vasa afferentia from the rete testis and 1s  firmly attached to the testis.   From the tail, the vas deferens (ductus deferens), a direct  continuation of the canal of the epididymis, passes up  medially.   Occurs due to patent processus vaginalis sac either completely  or partially.    The sac from the scrotum 1s patent up to the deep inguinal  ring.    3. True congenital hydrocoele (Fig. 42.18)  ¢ In this condition, the scrotal sac communicates with the  peritoneal cavity. It is seen in infants, may be secondary  to TB peritonitis. The scrotal swelling appears when the  child assumes an erect posture for a long time and it  may not reduce due to inverted ink bottle effect. Hence,  congenital hydrocoele is not reducible. It regresses in  size if the child assumes supine position while sleeping.    4. Encysted hydrocoele of the cord (Figs 42.19 and 42.23 to   ¢ In this condition the sac is obliterated above (inguinal  canal) and below (scrotum) but patent at the root of the  scrotum around spermatic cord.   e It presents as a soft, cystic, fluctuant, transilluminant  swelling separate from testis, well above the testis.   ¢ Diagnosis is established by traction test: The swelling  has got free mobility but when traction 1s applied to testis  gently, the swelling becomes fixed and it moves down  when testis 1s pulled down. This variety of hydrocoele ts  treated by excision of the sac.    In this condition, the scrotal sac communicates with another  sac underneath the anterior abdominal wall musculature.  Diagnosis 1s made by eliciting cross-fluctuation test.    6. Hydrocoele of canal of Nuck (Fig. 42.21)  It presents as a swelling in the inguinal region in female.   This 1s the most common type of hydrocoele which ts seen in  young adults, middle age and beyond. It is due to following  causes:    Soft, cystic, fluctuant, transillumination positive swelling  confined to the scrotum (Fig. 42.22).    1. Recurrent epididymo-orchitis due to filariasis  ¢ Fluid that accumulates is due to obstruction of lymphatics.  The fluid is milky white. Such hydrocoeles are called  chylocoeles and often do not exhibit transillumination.  (Key Box 42.2).    2. Tuberculous epididymo-orchitis (Figs 42.28 and 42.29)  ¢ Retrograde infection from the seminal vesicles.    ¢ Craggy epididymis refers to rough, hard, irregular  surface. This involves the epididymal head and causes  fibrosis. So, the epididymis feels craggy. Vas deferens  feels like beads, called as beaded vas. Secondary  hydrocoele occurs in 30% of the cases. Eventually it  forms cold abscess which ruptures and results in sinus  posteriorly, in the scrotum.    3. Testicular tumours: They can present with a swelling of  the scrotum, often diagnosed as hydrocoele. Any young  patient with a rapidly growing scrotal swelling could be a  testicular neoplasm. Fluid within the sac is haemorrhagic.    4. Pyocoele: Infected hydrocoele. Infection in a hydrocoele  1s rare because of the tunica vaginalis sac which 1s relatively  avascular. However, few cases may get infected resulting  in pyocoele. These patients present with fever, chills and  rigors.   5. Haematocoele: Trauma to the hydrocoele or spontaneous  bleeding into the sac.    Comparison of primary and secondary hydrocoele  is given in Table 42.2    Treatment of hydrocoele (Key Box 42.3 and also see  Chapter 53)    1. Lord's plication is indicated in small hydrocoeles. The sac  is opened and the cut edge of the sac is plicated to tunica  albuginea. (It is the reflected portion of the processus   Primary hydrocoele Secondary hydrocoele  1. Aetiology Defective absorption of fluid Excessive production of fluid  2. Examples Vaginal hydrocoele, infantile hydrocoele. Filarial hydrocoele, secondary to malignancy  of the testis.  3. Size Moderate, big Small  4. Palpation of the testis Difficult Easily palpable  5. Transillumination Positive in majority of the cases Usually negative  6. Consistency Tensely cystic Lax, cystic  7. Treatment Partial excision and eversion Treatment of the primary    vaginalis.) As a result, the sac gets crumpled up near the  testis. The testicular secretions get absorbed by  subcutaneous lymphatics and venous system.    2. Partial excision and eversion of the sac: Jaboulay's  operation. This is indicated in large hydrocoeles. The thick,  large, sac 1s excised and 1s sutured behind testis.    3. Aspiration is a temporary method and there is a chance of  introducing infection. It can be done only in high-risk  patients. This 1s a procedure to be condemned.   2. Pyocoele: Infected haematocoele Fig. 42.31: Spermatocoele Fig. 42.32: Epididymal cyst   5. Hernia of the hydrocoele sac  occurs when there 1s a small tear  in the sac resulting in accumula-  tion of fluid in the subcutaneous _‘ Fig. 42.30: Hernia of  planes (Fig. 40.30). hydrocoele sac   II. Retention cyst  1. Spermatocoele (Table 42.3 and Fig. 42.31)  2. Sebaceous cyst (skin of the scrotum) (Figs 42.36 and 42.37)    III. Congenital cyst  1. Epididymal cyst (Fig. 42.32)  2. Cyst of the hydatid of Morgagni   * Cold abscess with a sinus in the posterior aspect of ia  scrotum (Figs 42.33 to 42.35) Fig. 42.34: Sinus scrotum Fig. 42.35: Abscess scrotum    2 Site Behind and above the testis in the region of Behind the body of the testis  epididymal head  3. Loculi Multilocular Unilocular  4. Contents Crystal clear, watery Barley water-like  5. Transillumination Brilliant (Chinese lantern pattern) Poor transillumination—very often negative  6. Aspiration Results in recurrence as the cyst is multilocular May cure as the cyst is unilocular  7. Excision Excision may be necessary if the cyst is large May be excised if aspiration is not successful   Fig. 42.36: Multiple sebaceous Fig. 42.37: Strawberry scro-  cysts of scrotum (Courtesy: tum (Courtesy: Dr Umesh  Prof Abdul Majeed, MS FRCS, Bhat, General Surgeon,  Yenepoya Medical College, Kundapur, Karnataka)  Mangalore)    When the descent of testis 1s arrested somewhere in its normal  pathway to the scrotum, undescended testis occurs.    ¢ The testis develops in the retroperitoneum close to the  posterior abdominal wall from the genital tubercles.   ¢ By around 7th month, it reaches the deep inguinal ring, 8th  month—1inguinal canal and 9th month—superficial inguinal  pouch. In normal situations, the testis reaches the scrotum  at full term.   ¢ As it comes down, it is surrounded by processus vaginalis  sac. This sac gets completely obliterated in normal persons.  The persistence of the processes vaginalis sac 1s responsible  for development of a hernia and hydrocoele.    Muscular hypotonia: The descent of the testis depends  upon muscular contractions of the anterior abdominal wall.  Hence undescended testis is seen in children with poor  muscle tone, e.g. prune-belly syndrome and Down’s  syndrome    causes development (maturation) of testis and also helps in  descent of the testis. If there is deficiency of HCG,  imperfectly developed undescended testis appears.  Familial    Right side is more often involved. Bilateral undescended  testis 1s found in about 20% of cases.   Cryptorchidism: When both testes are impalpable as in  cases of abdominal testis and inguinal testis.    developed, the testis 1s palpable at the root of the scrotum  and can be brought down to the scrotum. Retractile testis is  harmless and spontaneously gets corrected within |—2 years  of age, without any treatment. The squatting position may  help in such cases, in diagnosing the condition as well as  its descent down to the scrotum.   Epididymo-orchitis will mimic an acute abdomen.  Sterility: Histological changes start at the age of 2 and by  the age of 12, irreversible damage occurs to the spermato-  genesis, due to atrophy of testis. Endocrine function will  remain normal.   Seminoma of testis and other testicular malignancies are  reported in greater frequency in undescended testis than in  normal testis. (Risk of seminoma in both testis, not only  in undescended testis.)   ¢ It can be done by open method or laparoscopic method.   ¢ 2 stage procedure for undescended testis  (Fowler-Stephens technique)  Stage | — tethering spermatic blood vessels are divided  Stage I]— testicle placed into scrotum after collateral  blood supply to testicle has developed.   ¢ Considering the psychological, functional and malignant  potentials, 6 months to | year is the ideal age to operate  in bilateral undescended testis and 4 years for unilateral  cases. The inguinal canal is explored, testis mobilised  by dividing the adhesions and brought down into the  scrotum and fixed by using nonabsorbable suture  material.   ¢ A dartos pouch can be formed and followed later by  narrowing of the root of scrotum.   2. Orchidectomy is done after the age of 14 because of  malignant potential.   3. Ombredanne’s procedure: Testis 1s brought down to the  opposite scrotum through scrotal septum and kept in dartos  pouch.   4. Silbar procedure: Testicular artery and vein can be divided  and reanastomosed using microvascular techniques.    ¢ Testis is present in an ectopic site (not the route through  which is descends).    ¢ Embryology: Testis reaches the scrotum by the scrotal  tail gubernaculum. However, if this is weak, the other scrotal  tail may pull it in a different direction, resulting in ectopic  testis (Lockwood’s theory).    ¢ Complication: It is more prone for injuries  ¢ Treatment: Orchidopexy in a new scrotal pouch    e Dilatation of testicular veins with/without cremasteric veins  which drain into testicular veins.   ¢ Testicular veins which drain the testis and epididymis, form  multiple veins in the scrotum which are called as  pampiniform plexus of veins. As they travel the inguinal  canal, they are reduced to 6—8 in number. At the level of  deep ring, they are 2 in number and in retroperitoneum, it  forms the single testicular vein.   ¢ On the right side, the testicular vein drains into inferior  vena cava (IVC) directly.   ¢ On the left side, it drains into the left renal vein at right  angles where there is a valve.   1. Varicocoele is common on the left side (because left testis  is at a lower level than the right). The flow of the blood  from the left side 1s into the renal vein where it makes an  angle of 90 degrees.   3. A recent varicocoele in an elderly patient suggests renal  cell carcinoma invading the renal veins.   ¢ In the standing position, the diseased side appears to be  more swollen than the other side. It feels like a bag of worms.  On asking the patient to cough, there 1s fluid thrill, due to  regurgitation of venous blood. On the side of varicocoele,  scrotum 1s at a lower level.   ¢ On asking the patient to lie down, it is reducible (disappears).   ¢ Testis may appear small and soft with diminished testicular  sensation.   ¢ Blow test: On blowing, fluid thrill may be felt, 1t may  increase in size (Valsalva’s manoeuvre).  An USG demonstrating veins 3.5 mm or more in diameter  with reversal of venous flow after Valsalva manoeuvre—  varicocoele.    I (small) — palpable only with Valsalva manoeuvre  II (moderate) — palpable without Valsalva manoeuvre  III (large) — visible through scrotal skin.   A. The venous congestion due to the varicocoele results in  increased temperature in the scrotum which 1s supposed  to affect spermatogenesis.   B. Reflux of the blood from the renal vein brings powerful  hormones secreted from adrenal glands like cortico-  steroids and adrenaline which may suppress spermato-  genesis.    2. Hydrocoele (most common) — due to ligation of lymphatic  vessels.  3. Recurrence.    1. Inguinal approach: Excision of pampiniform plexus 1n the  inguinal canal after ligating them. Testis still has a venous  drainage through cremasteric veins.    2. Retroperitoneal approach (Palomo's operation): In the  retroperitoneum, testicular vein Is single and 1s separate from  vas deferens. Hence, it 1s ligated up in the retroperitoneum.  This operation was once thought better than inguinal  approach since there is no danger of damaging the vas and  ligation of testicular vein 1s easy but recurrence rates are  high and hence not favoured nowadays.    e Inversion of testis is the commonest cause where testis  lies horizontally or upside down.   e Sudden contraction of spirally attached cremasteric muscle  leads to rotation of testis around the vertical axis during  straining at stools, lifting heavy weight, coitus.   e A long redundant spermatic cord allows twisting of the  testis on its Own axis.   e¢ Extravaginal torsion: It is diagnosed in newborns and is  caused by non-adherence of tunica vaginalis to the dartos layer.  As a result, spermatic cord and tunica vaginalis are rotated  as a unit.   ¢ Intravaginal torsion: It 1s usually diagnosed in boys of  12-18 years of age, but it can occur at any age.    ¢ Age: 10-25 years, sudden agonising pain in the groin and  lower abdomen and may be with vomiting.   ¢ Scrotum is empty and oedematous on the side of lesion.   ¢ Scrotal doppler to confirm the diagnosis  ]. In the first hour untwist the testis manually    2. If this is not successful urgent exploration of the scrotum  and undo the torsion and viable testis should be fixed to  the scrotum to prevent recurrence    3. Gangrenous testis should be removed  4. Opposite side testis should be fixed at an early date to    prevent torsion as it also has higher risk of undergoing  torsion.  PEARLS OF WISDOM    They constitute 1% of all malignant tumours in the males and  almost all are malignant (more than 99%).    Seminoma Nonseminomatous  germ cell tumours  e Classic (85%) e Teratoma  e Spermatocytic e Embryonal cell carcinoma  e Anaplastic e Choricocarcinoma  e Lymphocytic e Yolk sac tumour    B. Other classification  I. Seminoma is the most common germ cell tumour: 50%  incidence  Types  1. Spermatocytic type—good prognosis  2. Lymphocytic type  3. Anaplastic type  II. Teratoma  ¢ Incidence: 30% (subtype will be discussed later)    IV. Interstitial cell tumours  a. Leydig cell tumour  b. Sertoli cell tumours    ¢ | in 20 abdominal testis, 1 in 60 testis at the level of  deep ring and | 1n 80 inguinal testis are prone for testicular  tumours.   ¢ However, it should be noted that 25% of testicular cancers  in patients with crypto-orchidism occur in normal,  descended testis.    2. Klinefelter’s syndrome: These patients are prone for  development of seminoma testis. Other features of the  disease are testicular atrophy, absence of spermatogenesis,  gynaecomastia, etc.    3. Trauma to the testis is a coincidence. This may not  precipitate a testicular tumour but draws the attention of  the patient to It.    ¢ Seminoma arises from the seminiferous tubules. As the  tumour grows, it compresses the normal testicular tissue.  The cut surface is smooth, homogenous (Fig. 42.38).  Microscopy: Round to oval cells with prominent nucleus.  In few cases, lymphocytic infiltration can be found.    1. Typical: It is the commonest type of seminoma. Also  called as classic variety. Syncytiotrophoblastic type  produces high levels of B-HCG.    2. Spermatocytic type: {t occurs in elderly patients. It is slow  growing, rarely spreads. Hence has good prognosis.   3. Anaplastic: As the name suggests, this variety has higt  mitotic index/anaplasia, thus spreads fast and carries poo!  prognosis.    Teratoma arises from rete testis. The tumour contains  totipotential cells and so can have ectodermal, mesodermal  and endodermal elements (Fig. 42.39).    Fig. 42.39: Testicular tumour. Whole of testicular parenchyma is  replaced by tumour    1. Malignant teratoma differentiated: It is the least common  variety (1%). It is almost a benign tumour—Dermoid cyst.  Orchidectomy cures the disease.    2. Malignant teratoma intermediate: This is the most  common variety (30%) of teratoma containing malignant  and incompletely differentiated components.    3. Malignant teratoma anaplastic: Highly malignant tumour.  Secretes alfa fetoprotein (AFP). Cells are presumed to be  from yolk sac.    4. Malignant teratoma trophoblastic: This 1s an uncommon  tumour (1%). This secretes very high levels of B-Human  chorionic gonadotropins (B-HCG).   ¢ Testicular swelling: More often heaviness rather than  hypertrophy or if it is infiltrated with tumour but vas is  never involved. This is called sign of vas negative (sign    or lymphatic invasion (tumour may invade tunica albuginea  but not vaginalis)   T2: Tumour limited to testis and epididymis + vascular or  lymphatic invasion or invasion of tunica vaginalis   of vas positive in TB epididymo-orchitis where there is | 13: Tumour invades spermatic cord (with or without vascular    beading of vas). or lymphatic invasion)  T4: Tumour invades scrotum with or without vascular or    lymphatic invasion  Regional lymph nodes  . Gynaecomastia iS seen in about 10% of the patients. Nx: Lymph node status cannot be assessed    ¢ Secondary hydrocoele is not uncommon. Young adult NO: No lymph node metastasis  with small hydrocoele and enlargement of the testis | N1: Single or multiple < 2 cm in greatest dimension  should arise suspicion of testicular caner N2: Nodes 2-5 cm in greatest dimension   1. Hurricane variety is the most malignant tumour. The = Mx: Distant metastasis cannot be assessed  tumour grows rapidly with pulmonary metastasis (cannon MO: No distant metastasis  ball) and death in a few days. M1: Distant metastasis present   2.Mimicking acute epididymo-orchitis: This variety | Mla: Nonregional nodal or lung metastasis  presents as severe pain along with the swelling of the | _M1b: Nonpulmonary visceral metastasis (M2)    testis but does not respond to antibiotics. ‘Staging also considers serum tumour markers -S-LDH, HCG, AFP    1. Lymphatic spread SPREAD  ¢ Para-aortic node mass—distension of the abdomen * Seminoma spreads by lymphatic route. Along the testicular  (Fig. 42.40). vessels it spreads to para-aortic lymph node mass, through  ¢ Left supraclavicular node mass—swelling in the neck. thoracic duct, to mediastinal nodes and left supraclavicular  ¢ Iliac node mass—swelling of the leg. nodes. Spread does not occur to inguinal nodes unless  2. Blood spread: Extensive pulmonary secondaries occur scrotum ts incised.  from a malignant teratoma. ¢ Malignant teratomatous tumours spreads predominantly by  blood.    ¢ Stage I: Tumour confined to the testis only.  ¢ Stage ITA: Tumour and lymph nodes below the diaphragm—  size less than 2 cm.  Para ¢ Stage I[B: Tumour and lymph nodes below the diaphragm—  aortic nodes ;  size more than 2 cm.  ¢ Stage III]: Tumour and lymph nodes above the diaphragm.  ¢ Stage IV: Blood spread to lungs/liver/elsewhere.    1. No biopsy should be done through scrotal route because  if the scrotal skin is involved, the spread occurs to inguinal  lymph nodes opening up one more channel of  lymphatics. Even FNAC is not recommended.    Fig. 42.40: Seminoma testis with para-aortic nodes—clinical 2. Chest X-ray: To rule out cannon ball secondaries as in  examination of testis is very important in an upper abdominal mass teratoma.    3. Ultrasound of the testis: Seminoma appear as  hypoechoic lesion and nonseminomatous tumours as  inhomogenous.    4. Abdominal ultrasonography to see for enlarged lymph  nodes, secondaries in the liver, or to detect a tumour in an  undescended testis. However, CT scan 1s a better  investigation.    5. CT scan: Heterogenously enhancing testicular mass with  retroperitoneal lymph nodes and liver metastasis can be  made out (Fig. 42.41).    ¢ HCG as a hole (a- and B-HCG) may be increased in  testicular neoplasm, melanoma, lymphoma, etc. It can  also be raised in nonmalignant conditions such as  cirrhosis, peptic ulcer disease.    Fig. 42.41: Testicular tumour with para-aortic lymph nodes and  liver metastasis   B-HCG  e Never found in normal persons  e Secreted by syncytiotrophoblasts  e Choriocarcinoma: 100% cases,Seminoma: 10% of cases  e Embryonal carcinoma: 65% of cases  e Increased levels after orchidectomy indicate recurrence,  residual tumour.  e Tumour marker of teratocarcinoma.    ¢ It is the B-HCG which is more important in diagnosing  testicular neoplasms and also useful in the postoperative  period to know residual tumour or recurrent tumour.  ¢ The blood levels of B-HCG is 0 ng/ml.   9. Lactate dehydrogenase (LDH): Increased in nonsemino-  matous germ cell tumours. LDH levels indicate tumour  load. It is not specific for any tumour.    ¢ Radical inguinal orchidectomy is the treatment of choice  in all testicular tumours irrespective of the histological  type and stage.  When a patient presents with rapidly growing testicular  swelling and the neoplasm is doubtful, testis is explored  through an inguinal incision. It is delivered out and a soft  clamp is applied to the testicular vessels at the level of  deep ring while doing the procedure so that tumour  embolisation does not occur. Testis is split open, the  Suspicious area is biopsied and sent for frozen section. If  the frozen section is positive, the cord and testicular vessels  are divided at the level of deep ring and testis 1s removed.  This 1s called as high orchidectomy. If frozen section is  negative, the testis is sutured back and replaced in scrotum.  This kind of procedure done through inguinal route 1s called  as Chevasu's procedure.    a. Stage I-IIA (Low stage): Radiotherapy to retroperitoneum  (2500-3000 cGy) is the treatment of choice:    e It is recommended in all cases of teratoma including  stage |. Also indicated in residual disease in lymph nodes  in Seminomatous tumours also.   e All groups of lymph nodes draining the tumour such as  precaval, paracaval, interaortocaval, retroaortic, para-  aortic, common iliac nodes are removed.   e it also involves removal of gonadal vein and fibrofatty tissue  around the vein from its origin near the internal ring to its  insertion to renal vein on the left side and inferior vena  cava on the right side.   e The procedure is done on both sides. Haemorrhage, injury  to ureter and bowel are other complications.    e e e e e e e e e e e e e e e e e e e e e    ¢ 5-year survival rate is around 95%.  ¢ Relapse after radiotherapy is managed by chemotherapy.  b Seminoma stage IIB, III, IV  ¢ Radical orchidectomy + chemotherapy-PVB regimen-  cisplatin, vincristine, bleomycin.  ¢ BEP: Bleomycin, etoposide and cisplatin  c. Treatment of residual disease in lymph nodes  ¢ In stage IIB and III, if there is residual lymph nodal mass  3 cm in size even after chemotherapy, then, a retroperi-  toneal lymph node dissection (RPLND) has to be done.  ¢ Stage IIB and III: Survival for 5 years—75%  ¢ Stage IV: Poor survival.    a. Stage I, ITA (Low stage)  ¢ Radical orchidectomy and RPLND 5-year survival—85%    b. Stage IIB, IIl  Radical orchidectomy and chemotherapy (PVB), post  chemotherapy residual tumour in the retroperitoneum and  if the tumour markers levels regress—retroperitoneal lymph  node dissection should be done. 5-year survival rate is  around 60%.    ¢ 2 types are important clinically.  e Leydig cell tumour and Sertoli cell tumour.  ¢ They should be treated like teratoma.    ¢ They are feminising tumours: Gynaecomastia, loss of  libido, and aspermia are other features.    Even though Fournier's gangrene 1s called as idiopathic  gangrene, certain factors precipitate the scrotal gangrene.    Seminoma Teratoma  1. Cell of origin Seminiferous tubules in the mediastinum of the testis Totipotential cells in the rete testis  2. Incidence 35-40% 30%  3. Age group 30-40 years 20-30 years  4. Shape of testis Retained Not retained  5. Surface Smooth Irregular  6. Cut surface Smooth Variegated—nodules, cysts  7. Consistency Firm Firm or soft (cystic)  8. Spread Mainly lymphatic Predominantly blood  9. Tumour markers - HCG—nmalignant teratoma  10. Radiation response Excellent—melts like snow Less sensitive    occurs resulting in sloughing of the scrotal skin exposing  the testicles. In few cases, the gangrene can involve skin of  the penis, anterior abdominal wall, medial side of thigh,  perianal region. In such situations, it is described as perineal  phlegmon (Figs 42.42 to 42.44).    Broad spectrum antibiotics are started, once pus is sent for  culture and sensitivity, e.g.    . Gangrenous portion of the scrotum has to be excised, as    soon as possible which brings a dramatic reversal of general  condition of the patient from toxic to near normal.    . If the testicles are exposed, they can be implanted in the    thigh or once the inflammation subsides, skin grafting is  done to cover testicles.   Fig. 42.45: Healing with skin grafting  (Courtesy: Dr Ramachandra L, KMC, Manipal)    4. If penile skin is gangrenous, it is excised and covered with    split skin graft later. Surprisingly, results are better than  expected!! (Fig. 42.45)    It is a misnomer  ¢ It is the traumatic rupture of corpora cavernosum. It Is   Sudden blunt trauma or abrupt lateral bending of penis in  an erect state can break the markedly thinned and stiff tunica  albuginea.   Causes: Sexual activity, masturbation, gun shot wounds,  industrial accidents, mechanical trauma.   If surgical therapy 1s delayed due to urethral injury, initial  medical therapy consists of cold compresses, pressure dress-  ings, anti-inflammatory medications and suprapublic  cystostomy.    correct the defect in tunica albuginea and repair urethral  injury urethral catheter removed after 2 weeks.    Erectile dysfunction, abnormal curvature, painful erections,  urethrocutaneous fistula, corpora-urethral fistula and painful  nodules.    Priapism is a pathologic condition of a penile erection that  persists beyond or is unrelated to sexual stimulation.   It can occur in all age groups including the newborn but  peak incidence:    Low flow: Sickle cell trait/disease, chronic myeloid  leaukemia, total parenteral nutrition, medications (sildenafil,  cocaine), malignant penile infiltration, spinal cord injury,  spinal anaesthesia or general anaesthesia.   Corporal blood gas analysis can differentiate low flow and  high flow priapsim.   and a-adrenergic intracorporal injections (epinephrine/  phenylephrine/ephedrine) every 5 min till detumescence.  In severe cases—shunts (corporoglandular), corporo-  spongiosal or corporosaphenous) may be necessary. If  patient has sickle cell disease, IV bicarbonate and blood  transfusions required.   Most common cancer in young adults is testicular cancer  Most common solid cancer in young man is seminoma  Most common tumour is seminoma   e All the topics have been updated  e New photographs and key boxes have been added   C. Attached to symphysis pubis by suspensory ligament  D. Deep artery supplies corpus spongiosum alone   C. Inflammation of glands in the fossa navicularis  D. Inflammation of the urethral glands    5. Which of the following is rare feature of carcinoma 13. Which of the following test differentiate encystec  penis? hydrocoele from spermatocoele?  A. Foul smelling discharge A. Fluctuation  B. Recent phimosis B. Getting above the swelling  C. Urethral involvement C. Impulse on cough  D. Induration is extensive D. Traction test  6. Treatment of carcinoma penis confined to prepuce is: 14. What is the classical clinical feature of tuberculou:  A. Circumcision epididymo-orchitis?  B. Dorsal slit and excision A. Thickened vas  C. Partial amputation B. Atrophy of the testis  D. Radiotherapy C. Craggy epididymis  7. Following are true for partial amputation of penis D. Anterior sinus  except: 15. Chinese lantern type of transillumination is classica  A. Partial amputation 1s the treatment for growth confined of:  to glans penis A. Spermatocoele B. Epididymal cyst  B. At least 2 cm proximal shaft 1s necessary in partial C. Ranula D. Cystic hygroma  amputation 16. Which of the following is true in cases of retractile  C. Long ventral flap is required to cover testis?  D. Perineal urethrostomy may be required A. It is premalignant  8. Few important consideration sin total amputation of B. Testis 1s generally not palpable  the penis include following except: C. Scrotum is well developed  A. Suspensory ligaments have to be divided D. Testis cannot be brought down into scrotum  B. Perineal urethrostomy has to be done 17. In torsion testis following are true except:  C. Severe excoriation of the scrotum is a complication A. Scrotum is empty  D. Advantage being no stricture urethra after surgery B. Tender lump at external abdominal ring  9. Following are the features syphilitic chancre of the C. Elevation of scrotum increases pain  penis except: D. Upper abdominal pain and vomiting are features  A. Single ulcer 18. Which one of the following is more prone for testicular  B. Hard chancre tumour?  C. Firm painful lymph nodes in the axilla A. Gardner’s syndrome  D. Contagious B. Down’s syndrome  10. Testosterone is produced by? C. Klinefelter’s syndrome  A. Germ cells B. Sertoli cells D. Sipple syndrome  C. Leydig cells D. Clear cells 19. Human chorionic gonadotrophin elevation is  11. Testicular artery is a branch of: diagnostic OF .........-++.00  A. Aoita A. Choriocarcinoma  B. Common iliac B. Seminoma  C. Internal iliac C. Leydig cell tumour  D. Early morning urine sample is the best to measure this D. Sertoli cell tumour  12. Following are true for prostate specific antigen except: 20. Following are true for increased LDH levels in  A. Released from columnar prostatic acinar epithelial PeTLOMIO? .  éalils A. It suggests liver metastasis  B. More than 4 nmol/ml suggest carcinoma prostate B. It suggests germ cell tumours  C. Prostatitis also can increase in the levels C. It suggests malignant melanoma  D. It does not help in assessing the response to treatment D. It suggests tumour load  ANSWERS  1D 2A 3 6 4D ee 6A 7D 8 D 9C 10 C  11 A 12 D ts D 14C iS B 16C i 1) 16 C 19 A 20 B    1. Infection  ¢ Acute glomerulonephritis  ¢ Tuberculosis  2. Infarction  ¢ SBE with emboli causing renal infarction  ¢ Massive haemolysis with acute renal tubular necrosis  ¢ Mismatched blood transfusion  3. Injury  ¢ Stab/blunt injury  4. Tumours  ¢ Wilms’ tumour: Nephroblastoma  ¢ Hypernephroma: Renal cell carcinoma (RCC)  ¢ Transitional cell carcinoma (TCC)  5. Stones  6. Polycystic kidney   HAEMATURIA SP  Common causes  e Urolithiasis  e Tumours  Uncommon causes  e Tuberculosis  e Cystitis, bladder tumours  e Polycystic kidney   . Stone: Common in school-going children  . Benign prostatic hyperplasia (BPH)    1. Patients on anticoagulants  2. Sickle cell anaemia  3. Bleeding disorders    ¢ Enlarged kidney * Renal cell carcinoma  * Polycystic kidney  * Wilms’ tumour  * Stones    Differential diagnosis of haematuria is a major theory  question in an undergraduate examination. | have given  some ideas about how to evaluate these cases by  analysing history, physical examination and investiga-  tions. This gives a good exercise to the students.    Urine may vary in colour from red to stale brown or chocolate  coloured. May be associated with clots. The nature of clots  may give a clue to the source or site of bleeding. Serpiginous  or vermiform clots indicate bleeding from the kidneys.  Amorphous clots are suggestive of lower tract bleeding. Most  patients with gross haematuria have some pathology in the  kidneys or the genitourinary tract.    It can be initial, terminal or throughout the stream. Urethral  bleeding usually manifests as the first 10--15 ml of blood-stained  urine, which gradually clears. Terminal haematuria usually  indicates trigonal irritation (e.g. bladder stone). It 1s usually  associated with dysuria and strangury. Bleeding from the upper  urinary tract (kidneys and ureters) 1s seen throughout the stream  as the blood mixes with the urine stored in the bladder.    It is defined as the presence of more than 3 RBCs per high  power field on microscopic examination. It is usually detected  incidentally on urine dipstick testing and subsequent  microscopic examination.    Evaluation: Numerous investigative modalities are available  for evaluation of haematuria. The choice of the correct  modality depends on the clinical suspicion and important clues  on clinical examination.    Other organs affecting the urinary tract can also be  imaged (e.g. carcinoma cervix causing ureteric    Initial evaluation includes  Ultrasonography: It is noninvasive and less expensive  ¢ Diagnosis of upper or lower urinary tract lesions    a. Stones b. Tumours c. Hydronephrosis turia.  a_i,  Table 4° 3. 2 . Distinguishing between glomerular and urological causes of haematuria  Glomerular Nonglomerular  Colour (if macroscopic) Pale red, smoky brown or stale tea Red or pink   1. Following is the cause of haematuria in bacterial 4. Palpable renal masses in 40-year-old hypertensive    endocarditis: patient with one attack of haematuria is:  A. Renal necrosis B. Renal sepsis A. Hypernephroma  C. Renal infarction D. Coagulopathy B. Hydronephrosis  2. Haematuria in polycystic kidney is due to: C. Adenomyolipoma  A. Hypertension B. Renal infarction D. Polycystic kidney    3. Which of the following conditions rarely gives rise to  haematuria?   e Tracheobronchial injuries  Myocardial contusion  Surgical emphysema  Mediastinal emphysema  Mediastinal masses    In chest trauma, the mortality is very high unless promptly  recognised and properly treated. The margin of safety is very  slim, initial care dictates the final result. With varying degree  of severity, chest injuries occur in almost 80% of road traffic  accidents (Key Box 44.1).    The standard method of resuscitation in all cases of polytrauma  is as follows:    Aspiration of blood and secretions from oral cavity, pharynx  and trachea    COMMON CAUSES  Automobile accidents  Gunshot wounds  Stab injuries  Blast injuries  Crush injuries   e Off pump coronary artery bypass surgery  e Abdominal aortic aneurysms (AAA)    ¢ Intubation  ¢ Intercostal chest tube (ICT)  ¢ Closure of any open chest wounds  These two steps (A and B) help in re-aerating the lung   * All the above steps are taken by the trauma centre team  simultaneously and not one by one.    ¢ Time since the injury  ¢ Details of the injury from the bystander or the police    ¢ High-speed deceleration injury (aortic and cardiac rupture  to be ruled out).   ¢ In stab injuries, length of the knife and direction of stab.    ¢ The clothing should be removed carefully without moving  the patient.   ¢ Palpate for clinical evidence of fracture ribs, surgical  emphysema, any paradoxical movement of the ribs and  auscultate for air entry in both lungs.   ¢ Even if it is a trivial injury, the patient should be admitted  and observed for a minimum of 24 hours before discharge.   ¢ It is reasonable to do unilateral or bilateral closed tube  thoracostomy (ICT) (Fig. 44.1) on suspicion of  haemothorax or pneumothorax when the patient is in  respiratory distress.    SIMPLE RIB FRACTURE  Rib fracture can be single or multiple (Key Box 44.2).    ¢ Often regarded as a trivial injury but should be treated with  respect in elderly patients.   ¢ The common site Is at the costal angle or middle of the shaft.   e Patients will have pain on breathing, coughing and on  palpation.   e First rib fracture is a marker of severe trauma—injury to  brachial plexus, subclavian artery and vein   e Penetrating injury to left lower chest wall—injury to heart,  lung, diaphragm, stomach and spleen    e They are treated with analgesics, intercostal nerve block  and assurance.   ¢ When there are multiple rib fractures without any pneumo-  or haemothorax and no other organs are involved,  intercostal nerve block and small amount of narcotics are  required.   ¢ Chest X-ray to be repeated after 24 hour and at the time of  discharge, to rule out late onset pneumo- and haemothorax.   e Inform the patient of deep breathing and coughing using  the rib belt.   ¢ Epidural analgesia is becoming the standard of care for pain  management in patients with multiple rib fractures.   Here there are fracture of four or more ribs at two places,  anteriorly and posteriorly, so that certain segments of ribs will  have no attachment to the chest wall. These ribs become indrawn  due to intrathoracic negative pressure as the patient inhales and  is driven outwards on expiration producing instability. This 1s  called paradoxical respiration. It results in hypoventilation,  carbon dioxide retention and respiratory failure.    ¢ Fracture of the costochondral junction on both side of the  sternum    1. Anterior flail: Seagull-shaped prosthesis introduced to  stabilise the flail segments.   2. Posterior flail: No treatment 1s required as the scapula acts  as a Support to stabilise the flail segment.   3. Lateral flail: It is treated by chest wall stabilisation,  reduction of the respiratory dead space, management of  the pulmonary contusion and pain control. Epidural  analgesia is recommended for pain management. Intercostal  nerve blocks may also be used.   ¢ Recent method ts to intubate and stabilise the flail segments  with positive pressure ventilation, which has to be done for  at least one week. This 1s called internal pneumatic fixation.   ¢ Physiologic stabilisation with intubation and IPPV must  be initiated before hypoxia develops. IPPV produces  satisfactory ventilation and helps the fractured ribs to unite  in the position of inspiration, thereby reducing the deformity  and improving the pulmonary function.    ¢ Localised, severe, blunt or crush injury produces depression  of a portion of the chest.   ¢ Treatment is same as that for flail chest. Sometimes,  thoracotomy may be needed if there are internal injuries.    ¢ Deceleration injuries or crush trauma often produces  extensive parenchymal damage. Haemorrhage and  interstitial oedema result in obliteration of alveolar spaces  and consolidation of large areas of pulmonary tissue.   ¢ Contusion of the lung can be unilateral or bilateral. The  contusion can be in the form of a small area of damage  with oedema and extravasation of the blood, or it may be  widespread damage. Haemoptysis and excessive  tracheobronchial secretions give the clue to the diagnosis  (Key Box 44.3).    PULMONARY INJURIES  e Contusion  e Pneumothorax/hnaemothorax  e Laceration  e Chest wall injuries    ¢ Chest X-ray: Early patchy consolidation. It must bs  differentiated from adult respiratory distress syndrom:  (ARDS).    ¢ Pneumothorax is the most common cause of respiratory  insufficiency following chest trauma.   ¢ A repeat chest X-ray after 12-24 hours is essential to  confirm that it is not progressing.   Open chest wound will produce complete collapse of lung  and paradoxical shift of the mediastinum with each  respiration (mediastinal flutter) causing hypoventilation and  reduced cardiac output.   Treatment is by closure of the wound, intercostal tube  drainage (ICD) and surgery.    ¢ Injury to the lung results in continuous valvular air leak.  The accumulating air collapses the lung on the same side  and pushes the mediastinum to opposite side. As a result of    TENSION PNEUMOTHORAX: CLINICAL FEATURES  Tachypnoea  Tachycardia  Tympanic note on percussion  Total absence of breath sounds  Tracheal shift Observe 5 Ts   TENSION PNEUMOTHORAX  e Diagnosis immediately  e Do not wait for CXR  e Thoracocentesis in 2nd intercostal space, midclavicular  line.  e Then, follow with intercostal chest tube insertion.    this tension, the intrapleural pressure increases, till it is  above atmospheric pressure at the time of expiration. This  reduces the venous return to the heart as well as  compromises the ventilation.    treated urgently with needle thoracocentesis in the second  intercostal space in the midclavicular line to release the  tension. Thoracocentesis converts tension pneumothorax  to simple pneumothorax.   This should be followed by urgent ICD insertion and  connected to underwater seal.   This occurs due to herniation of dilated and obstructed stomach  into the mediastinum due to diaphragmatic tear resulting in  haemodynamic compromise. It should be treated by reducing  the hernial contents and repair of diaphragmatic tear.    ¢ May be missed in chest X-rays in the supine position.   ¢ Classical signs are reduced—chest expansion, dullness to  percussion and absent breath sounds on affected side.   ¢ Treated by intercostal chest tube insertion (Figs 44.5 and  44.6).   ¢ If bleeding continues or features of shock develop,  thoracotomy has to be considered.   ¢ The bleeding may be delayed or may recur after several  days.    ¢ Initial volume of blood loss is not as important as the  amount of ongoing bleeding.   ¢ Drainage is more than 1000 ml or 100 ml each hour for  4 hours.   ¢ If clotted haemothorax is suspected (opacity persisting on  chest X-ray even after ICD).    Failure to adequately drain a haemothorax initially results in  residual clotted haemothorax and empyema or late fibro-  thorax.    ¢ Second intercostal space—anteriorly midclavicular line  is ideal for pneumothorax and sixth space in the midaxillary  line for haemothorax.    ¢ Can be introduced from sixth intercostal space midaxillary  line for pneumothorax also but the chest tube should reach  the apex of the lung (Figs 44.7 and 44.8).    ¢ Infiltrate local anaesthetics up to the parietal pleura  e 2-3 cm incision parallel to the ribs—deepened, suture taken  ¢ Insert the chest tube with trocar into the pleural cavity    e Then the trocar is removed and the chest tube 1s connected  to underwater seal. The ICT is fixed    e Intercostal drainage can also be done by connecting to  2 bottles (Fig. 44.9)   . Should take an X-ray after the insertion of the tube   . Should be always lower than patient level in the supine  position. Otherwise, contents of the bottle will enter the  pleural space    ICT—REMOVAL  e Chest X-ray—lung fully expanded and drainage should be  less than 100 ml for two days and no air leak. Patient should  not be on a ventilator  e Clamp the tube for 24 hours  e Removal after 24 hours of clamping, provided the patient  is comfortable and lung remains fully expanded.    ¢ Minor laceration: Haemopneumothorax. Usually inter  costal chest tube 1s enough.  e Major laceration: Haemopneumothorax  1. Introduce intercostal chest tube  141. Continuous air leak or bleeding through ICT and lung  not expanding. Requires thoracotomy and repair 01}  resection of lobe.   ¢ Alveolar collapse due to shock as a result of oedema.  impaired perfusion, and reduction in alveolar space resulting    in respiratory failure is called shock lung.  ¢ Itisalso called Acute Respiratory Distress Syndrome (ARDS).    e Major chest trauma with multiple rib fractures and lung  contusion   ¢ Focal disorders of the circulation, primarily because of  sluggish microcirculation due to leukostasis, sludge, leading  to extensive hyaline thromboses   ¢ Diffuse alveolar damage, decreased production of surfactant  by type II pneumocytes leading to bilateral extensive fine  atelectasis   ¢ Net result is pulmonary consolidation, decrease in the lung  compliance, poor gas exchange leading to stiff lung.    ¢ Arterial blood gas analysis  ¢ Chest X-ray  ° CT scan   ¢ Low to moderate doses of steroids may help in early ARDS,  in patients requiring high doses of vasopressors to maintain  blood pressure.    Injuries occur as a result of trauma sustained due to crush  injuries. Common in cervical trachea.    ¢ Blunt trauma produces stereotype injury to main bronchus  of either side. Lobar bronchi are less commonly injured  (Table 44.1).   ¢ Lesion is often a circumferential laceration with complete  separation or a partial tear.   ¢ Bronchoplasty, injury to the lobar bronchus—resection of  the lobe of the lung.   ¢ Diagnosed by CECT of the abdomen or chest X-ray reveals  the Ryle’s tube in the chest or coils of bowel (gas shadow)  in the chest and elevated fundic air bubble (Figs 44.10 to  44.12). Diagnostic laparoscopy is also a good investigation  in cases of rupture diaphragm.    Fig. 44.10: Chest X-ray left haemothorax with elevated fundic  air bubble    X-ray chest may show pneumothorax or air in the sub.  cutaneous plane (Fig. 44.14).   ¢ Two ICT, one apical and the other, basal, may need to be  introduced if there is continuous air leak.    2. Extensive from the eyelids to the scrotum, quite alarming Fig. 44.14: Chest X-ray showing 10th rib fracture with surgical  In appearance. Indicates lung injury (Fig. 44.13). emphysema on the right side   SURGICAL EMPHYSEMA  Air in the subcutaneous tissue  Palpable crepitus  Sometimes, can be gross  Disfigurement is more than symptoms and signs  Rib stabbing the lung  Rupture of bronchus—mediastinal emphysema    e The emphysema usually subsides within a week. Multiple  incisions and expelling the air from the subcutaneous space  manually are not required but may be carried out for cosmetic  purposes or if patient has respiratory distress (Key Box 44.8).    They depend on type of weapon, length and direction of the  stab (Key Box 44.9).    ¢ Relatively low velocity revolver bullets may perforate one  or two lobes of the lung with a little damage and only  drainage of the pleural space 1s required.   * High velocity perforating wounds cause more damage to  the tissue, adjacent to the tract. If the damage is extensive,  lobectomy or pneumonectomy has to be considered.   ¢ Bomb fragments, because of irregular shape, commonly  carry with them pieces of ribs and are associated not only  with severe haemorrhage and air leak from the torn  pulmonary vessels and bronchus but also with haemorrhage  from irregular entry and exit wounds. Urgent thoracotomy  is necessary.    Emergency thoracotomy is a life-saving procedure in the  trauma centre. Thoracotomy for polytrauma has a poor  prognosis than for isolated thoracic injuries.   ¢ Clamping of descending thoracic aorta for exsanguinous  bleeding in the abdomen.   ¢ On auscultation, pericardial crunching sound:  synchronous with the heart beat, are heard.   ¢ If there is haemodynamic instability, bilateral intrapleura  ICT has to be introduced as a precaution against tensior  pneumothorax.   2. Aspiration of secretions: Tracheal aspiration, nasotracheal  suction, aspiration of oral cavity and pharynx.    3. Relieving pain: Oral narcotics, parenteral narcotics,  thoracic epidural analgesia, intercostal nerve block.    5. Treatment of pneumothorax or haemothorax: Insertion  of intercostal chest tube.    6. Treatment of shock: First of all, the causes of shock in  chest injuries has to be determined by thorough assessment  of the patient. Once the cause is found out, depending upon  the nature of the problem, the patient is treated 1n an  intensive care unit (Key Box 44.10).    7. FAST (focussed abdominal sonography in trauma)  ultrasound: Focussed assessment with ultrasonography for  trauma and rapid assessment for ruling out fluid collection  in the abdomen, chest and pericardium.    Collection of the pus in the pleural space 1s called empyema.    e [tis the end stage result of pleural effusion and infection. A  few examples are—following haemothorax, lung abscess,  pneumonia. In India, tuberculosis 1s an important cause of  empyema (Figs 44.15 and 44.16)   ¢ Rupture of subphrenic abscess, rupture of hydatid cyst,  rupture of amoebic liver abscess also can result in empyema.   e The classical events which follow empyema following  pneumonia are exudative phase with pleural effusion,  followed by thickening of fluid—fibropurulent stage and Fig. 44.16: Consolidation  when the lung is covered by thick cortex, it 1s called  organising phase.   ¢ Presence of thick pus with thick cortex of fibrin and  coagulum over the lung.    e Chest X-ray may show collapse of the lung, tracheal  deviation, evidence of pneumonia or tuberculosis.    (Courtesy: Figs 44.15 to 44.18 are contributed by Dr C.S. Rajan, Senior Consultant and Head, Dept of Thoracic Surgery, St. Martha’s    ¢ Video Assisted Thoracoscopic Surgery (VATS): With 3-4  ports, video assisted procedures have become very popular.  Minimal incision, less pain and recovery 1s fast. Drainage,  pleural biopsy, talc pleurodesis, debridement of empyema,  intercostal tube drainage (ICD) can all be done.   ¢ Decortication: More radical procedure involves  thoracotomy, debridement, excision of thick cortex or the  covering of the lung, so that lung will expand. This 1s done  by posterolateral thoracotomy.    Empyema necessitans: It is a type of empyema which  presents as a swelling in the subcutaneous plane with  communication to the pleural space/cavity. Itis a tense, tender,  fluctuant swelling with local rise of temperature. Intercostal  bulge is also seen. On asking the patient to cough, expansile  impulse is felt. Otherwise, management is similar to that of  empyema such as drainage, treating the cause, ATT in  tuberculosis and antibiotics in pyogenic infections.    Definition  It is a fistulous communication between the pleural space and  the lung.    e [It may occur when large airways are in communication  with the pleural space following a large pneumothorax.    ¢ There will be a large empty space left behind following  pneumonectomy. This space will be filled with air. Over a  period of time air is absorbed. The gaping of bronchial  stump occurs resulting 1n bronchopleural fistula.   ¢ Specific treatment includes control of infection, treat the  primary cause, suturing, etc.   The mediastinum is a broad central partition that separates  the two laterally placed pleural cavities. It extends from the  sternum to the bodies of the vertebrae; and from the superior  thoracic aperture to the diaphragm.   Contents: The thymus gland, pericardial sac, heart, trachea  and major arteries and veins. It also serves as a passageway  for structures such as the oesophagus, thoracic duct and various  components of the nervous system as they traverse the thorax  on their way to the abdomen.    ¢ A transverse plane extending from the sternal angle (the Injet of thorax ——-—-—_____  junction between manubrium and the body of the stemum)  to the intervertebral disc between vertebrae thoracic 4 and  5 spaces separates the mediastinum into the superior  mediastinum and inferior mediastinum.    ¢ Inferior mediastinum is further partitioned into the thoracic artery  anterior, middle, and posterior mediastinum by the Body of sternum  pericardial sac. Anterior mediastinum    ¢ Anterior mediastinum: It lies between the back of the — sternopericardial ligament  sternum and the anterior aspect of the great vessels and  pericardium. It contains the thymus, internal mammary  arteries, lymph nodes, connective tissue and fat.   ¢ Middle mediastinum: It extends from the pericardium Middle mediastinum ==  anteriorly to the ventral surface of the thoracic spine Posterior mediastinum _  posteriorly. It contains the pericardium, heart, great vessels, ~/ 112  airway and oesophagus.   the spinal ganglia, the sympathetic chain, lymphatic tissue, Differential diagnosis of mediastinal masses 1s shown in  and connective tissue. Table 44.2.   i = Differential diagnosis of mediastinal masses  Anterior mediastinum Middle mediastinum Posterior mediastinum  Thymus Bronchogenic cyst Neurogenic tumours  ¢ Thymoma ¢ Neurofibroma  ¢ Thymic cyst ¢ Neurilemmoma  ¢ Thymic hyperplasia ¢ Neurosarcoma  ¢ Thymic carcinoma ¢ Ganglioneuroma   ¢ Most common anterior mediastinal primary tumour; 20%  of adult mediastinal neoplasms.   ¢ Presentation between ages 30 and 50 (most patients are  > 40 years old).   ¢ 50% are asymptomatic but others have symptoms secondary  to compression: Chest pain, cough, dyspnoea, compression  of the superior vena cava resulting in head and neck venous  congestion, facial oedema.   ¢ Myasthenia gravis: Seen in 30-50% of thymoma patients;  others can have hypogammaglobulinemia (10%), endocrine  disorders, connective tissue disorders.   ¢ Contrast CT scan of the thorax is the investigation of choice  which will detect a mass lesion and its relationship to the  adjacent structures (Fig. 44.20).    ¢ Surgery is the main modality of the treatment—a complete  resection of the thymus. Median sternotomy is done to  remove the tumour.   Stage II: Macroscopic invasion into surrounding fatty tissue  or mediastinal pleura, or microscopic invasion into  capsule   Stage III: | Macroscopic invasion into neighbouring organ, 1.e. peri-  cardium, great vessels, or lung   PREOPERATIVE BIOPSY IS NOT NECESSARY  FOR THE RESECTION OF FOLLOWING TUMOURS    e The mediastinum is the most common location for  extragonadal germ cell tumours in adults.   ¢ They are classified as benign (teratomas, dermoid cysts) or  malignant (seminomas, nonseminomatous GCTs).   ¢ Cystic teratomas are dermoid cysts, less malignant  compared to solid teratomas which have more chances of  malignancy.   e Diagnosis is by tumour markers: Alfa fetoproteins (AFP)  and beta-hCG. AFP is normal in teratoma and “pure”  seminomas. Ninety percent of nonseminomatous germ cell  tumours have elevated AFP and/or beta-hCG.   TUMOURS WHICH ARE HIGHLY sapocensrve ©  1. Germ cell tumours  2. Seminoma  3. Lymphoma  4. Squamous cell carcinoma    3. Lymphomas  e The most common are nodular sclerosing Hodgkin’s  lymphoma and primary mediastinal B-cell lymphoma.    e May present with systemic symptoms such as fevers, weight  loss or night sweats but can also present with symptoms  such as chest pain, dyspnoea, wheezing, stridor, hoarseness,    dysphagia, or superior vena cava syndrome due to  compression of mediastinal structures.    Core biopsy is required for the diagnosis  CT scan will help in ruling out other diseases.    Mediastinal mass ratio (MMR) the ratio of the mass to  the chest diameter 1s calculated (see page 119 of Chapter 9)    Intrathoracic thyroid tissue—ectopic or substernal,  typically causes symptoms of shortness of breath or  dysphagia. Sometimes they produce dangerous airway  obstruction, deviation of the trachea or narrowing.   The intrathoracic mass is usually continuous with the  thyroid gland in the neck; only 2 percent of cases are  separate from the cervical thyroid and are truly  intrathoracic.   Majority can be managed with cervical incision—a few  may require sternotomy.   Possibility of collapse of trachea should be kept in mind  after thyroidectomy—one should be ready with re-  intubation/tracheostomy.    Lymphadenopathy 1s the most common lesion presenting  as a mass in the middle compartment of the mediastinum.  The most common causes include lymphoma, sarcoid and  metastatic lung cancer.   Mediastinoscopy 1s a very useful technique to biopsy  lymphadenopathy tn this region.    . Bronchogenic cysts  Bronchogenic cysts are the most common cystic lesion    Secondary to abnormal lung budding during development.  Bronchogenic cysts are more common in men, in the right  paratracheal region and tn the subcarinal location.    Often presents with substernal pain, cough, recurrent  infection symptoms or dyspnoea.   They are the third most common benign oesophageal  masses after lelomyomas and polyps, and are usually  asymptomatic.   Three criteria are required to establish their diagnosis:  (a) Oesophageal attachment   Seventy percent arise in the right cardiophrenic angle.  Symptoms can include shortness of breath, right heart  failure secondary to compression, infection and bleeding.    Bronchogenic and enteric cysts need surgical resection to  establish a definitive diagnosis and to decrease the risk of  infection or malignant degeneration.   Pericardial cysts can typically be observed if asymptomatic  but resection can be utilised if there are symptoms or 1f the  diagnosis is not completely established by imaging.  Simple drainage 1s generally not recommended because  these cysts typically recur without complete resection.    Neurogenic tumours represent more than 60 percent of  posterior mediastinal masses. These lesions are classified based  upon their neural cell of origin. 95% of posterior mediastinal  masses arise in the intercostal nerve rami or the sympathetic  chain region.   These are benign lesions that arise from the intercostal  nerve sheath and make up 90 percent of adult neurogenic  tumours.   Neurilemmomas or schwannomas constitute 75% of this  group of masses. These tumours are firm, encapsulated  masses consisting of Schwann cells.   Neurofibromas are nonencapsulated, soft, friable and are  associated with von Recklinghausen neurofibromatosis.  The surgery of choice for removal of these tumours 1s by  thorocoscopy or thoracotomy.   Postoperative complications include Homer’s syndrome,  partial sympathectomy, recurrent laryngeal nerve damage  and paraplegia.    Malignant nerve sheath tumours are spindle cell sarcomas  of the posterior mediastinum and include malignant  neurofibromas, malignant schwannomas and neurogenic  fibrosarcomas.   They affect men and women equally in the third to fifth  decade of life and are closely associated with neuro-  fibromatosis, with a 5% risk of sarcomatous degeneration.  Pain and nerve deficits are common.   Complete surgical resection is the optimal treatment but in  patients with unresectable tumours, adjuvant chemotherapy  and radiation are options.    Neuroblastomas and ganglioneuroblastomas are malignant  tumours that occur most commonly in children and originate  from the sympathetic ganglia.    ¢ Ganglioneuromas are benign lesions that arise from the  sympathetic ganglia, and are most common in young adults.  Lesions that arise from paraganglionic cells include  phaeochromocytomas and paragangliomas.   e Resection usually requires a combined approach with  neurosurgery and thoracic surgery.    e Pulmonary aspergilloma, is a mycetoma or fungus ball,  caused by fungus of Aspergillus species   e The most common place affected by aspergillomas is the  lung   ¢ Tuberculosis of the lungs and immunocompromised  conditions are risk factors.   e Diagnosis is by chest X-ray and CT scan (Fig. 44.21),  majority of the cases do not require any treatment   ¢ In symptomatic cases (rarely), removal of the lesion,  lobectomy may be required (Fig. 44.22).   (Courtesy: Figs 44.21 and 44.22, Dr  C.S. Rajan, Senior Consultant and  Head, Dept of Thoracic Surgery, St.  Martha’s Hospital, Bangalore)    Bronchogenic cancer is the most frequent cause of cancer  death in men and women and accounts for 14.5% of all cancer  diagnoses and 27.6% of all cancer deaths in the United States.  Cigarette smoking is unequivocally the most important risk  factor in the development of lung cancer. In India,  bronchogenic carcinoma has become number one cancer in  men overtaking other cancers. The diagnosis is often delayed.  Many patients present as metastasis in bones. Surgical  resection alone cannot be curative in many cases because of  late presentation of the cases. Prognosis is poor in spite of the  treatment.   ¢ Industrial carcinogens: Asbestos, arsenic, chromium or  nickel, organic chemicals, radon.   ¢ Radon is the second leading cause of lung cancer. Radon  is a natural radioactive gas released from the normal decay  of uranium in the soil.    1. Adenocarcinoma (ADA) of the lung is the most frequent  histologic type and accounts for approximately 45% of all  lung cancers. Women smokers have more incidence of  adenocarcinoma. Adenocarcinoma of the lung develops  from the mucus-producing cells of the bronchial epithelium.  Cells are cuboidal to columnar cells. Most of these tumours  (75%) are peripherally located. Adenocarcinoma of the lung  tends to metastasise earlier than squamous cell carcinoma  of the lung and more frequently to the central nervous  system (CNS)—cases can present as weakness of the limbs,  hemiparesis, etc.   Sometimes it can be a more indolent disease. It 1s well    approximately 30% of patients with lung cancer.  Approximately 75% of these tumours are centrally located  and tend to expand against the bronchus, causing extrinsic  compression. These tumours are prone to undergo central  necrosis and cavitation. Squamous cell carcinoma tends  to metastasise later than adenocarcinoma. Microscopically,  keratinisation, stratification and intercellular bridge  formation are exhibited. SCCA may be more readily  detected on sputum cytology than ADA.    approximately 10% of all lung tumours. These tumours tend  to occur peripherally and may metastasise relatively early.  Small cell lung cancer represents approximately 20% of    all lung cancers; approximately 80% are centrally located.  The disease ts characterised by an aggressive tendency to  metastasise. It often spreads early to mediastinal lymph  nodes and distant sites, especially bone marrow and brain.    . Small cell lung cancer appears to arise tn cells derived    from the embryologic neural crest. Microscopically, these  cells appear as sheets or clusters of small dense cells, with  dark nuclei and little cytoplasm. This oat-like appearance  under the microscope gives the term oat cell carcinoma to  this disease. These tumours are often advanced at  presentation, with an aggressive tendency to metastasise  both by lymphatics and by blood. Chemoradiotherapy ts  generally used for treatment. However, the 5-year survival  rate is only 5%.    Cough and haemoptysis: It 1s a nonspecific symptom in  many patients and diagnosis is delayed because it is often  thought to be a smoker’s cough.   Dyspnoea is due to pleural effusion or due to restrictive  pulmonary disease.   Bloody effusion, clubbing of the fingers, localised chest  pain are other features.   Hoarseness due to recurrent laryngeal nerve paralysis, back  ache due to metastasis in vertebrae, or neurological features  due to metastasis in brain are also presenting features in  many cases.   Myopathy similar to myasthenia gravis can occur in small  cell carcinoma.    In addition to chest X-ray, CT scan, sputum for malignant  cells, diagnosis can also be made with bronchoscopic biopsy  and CT-guided biopsy. Spread is common PET scan: 18F-  fluorodeoxyglucose positron emission tomography (FDG-  PET) is the investigation of choice to detect the distant  lesions (Figs 44.23 to 44.25).   Fig. 44.24: Sagittal section of the CT showing bronchogenic  carcinoma right lung   PET-CT of a patient who was diagnosed to have carcinoma lung on bronchoscopy.  PET CT shows a hilar mass with a nodule anteriorly on left side of pleura.  It also shows pneumonic patch on lower zone of left tung which is FDG avid   TX: Primary tumour cannot be assessed, or tumour proven  by the presence of malignant cells in sputum or bronchial  washings but not visualised by imaging or bronchoscopy   T1: Tumour < 3 cm in greatest dimension, surrounded by  lung or visceral pleura, without bronchoscopic evidence of  invasion, more proximal than the lobar bronchus (i.e. not in  the main bronchus)   ¢ T 1b tumour > 2 cm but < 3 cm in greatest dimension    T2 tumour > 3 cm but < 7 cm or tumour with any of the  following features:   ¢ Involves main bronchus 2 2 cm distal to the carina   ¢ Invades visceral pleura associated with atelectasis or  obstructive pneumonitis that extends to the hilar region but  does not involve the entire lung   ¢ T2a tumour > 3 cm but < 5 cm in greatest dimension   ¢ T2b tumour > 5 cm but < 7 cm in greatest dimension    T3 tumour > 7 cm or one that directly invades any of the  following: chest wall (including superior sulcus tumours),  diaphragm, phrenic nerve, mediastinal pleura, parietal  pericardium; or tumour in the main bronchus < 2 cm distal  to the carina but without involvement of the carina or  associated atelectasis/obstructive pneumonitis of the entire  lung or separate tumour nodule(s) in the same lobe   TAM STAG  Stage T N M  Occult cancer TX NO MO  0 Tis NO Mo  IA T1la/b NO Mo  IB T2a NO MO  IIA T2b NO MO  Tia/b; T2a_ Nt MO  IIB T2b N1 Mo  i138 NO MO  IIA AnyT1;T2 N2 Mo  T3 N1/N2 Mo  T4 NO/N1 MO  HIB T4 N2 Mo  Any T N2 MO  Any T N5 Mo  IV Any T Any N M1a/b    ¢ CT of the chest, abdomen; MRI brain: Mediastinal lymph  node masses, metastases to the adrenal glands, brain, lung  and bone are common.    T4 tumour of any size that invades any of the following:  Mediastinum, heart, great vessels, trachea, recurrent laryngeal  nerve, oesophagus, vertebral body, carina; or separate tumour  nodule(s) in a different ipsilateral lobe   N1: Metastasis in ipsilateral peribronchial and/or ipsilateral hilar  lymph nodes and intrapulmonary nodes including involvement  by direct extension   N3: Metastasis in contralateral mediastinal, contralateral hilar,  ipsilateral or contralateral scalene or supraclavicular lymph  node(s)   e Mia separate tumour nodule(s) in a contralateral lobe; tumour  with pleural nodules or malignant pleural (or pericardial) effusion  ¢ Mib: Distant metastasis    1. Stages I and II tumours are contained within the lung and  may be completely resected with surgery.    2. Resectable stages IIIA and IIIB tumours are locally  advanced tumours with metastasis to the ipsilateral  mediastinal (N2) lymph nodes (stage IITA) or involving  mediastinal structures (T4NOMO). These tumours, by their  advanced nature, may be mechanically removed with  surgery followed by systemic chemotherapy. Radiotherapy  can also be given to locally advanced disease.    3. Stage IV disease includes metastatic disease and Is not  typically treated by surgery, except for patients requiring  surgical palliation. Systemic therapies—chemotherapy for  metastatic disease are common.   ¢ It develops between 3rd and 8th week of foetal life.   ¢ lst operation for congenital heart disease was ligation of  PDA by Cross in 1938.   e With new advances in neonatal cardiopulmonary bypass  (CPB), outcomes have improved.   ¢ Foetal circulation differs from adult in that right and left  ventricles pump blood in parallel rather than in series to  receive increasingly oxygenated blood.   ¢ 3 important structures are ductus venosus, foramen  ovale and ductus arteriosus.   ¢ Pulmonary vasodilation and ductus arteriosus constricts  within 30 minutes of delivery (due to increased oxygen  levels).   ¢ Reversal of pulmonary and systemic pressure gradient.  Termination of blood flow from pulmonary artery into aorta.   ¢ IVC and right atrial pressure decrease and left right atrial  pressure increases (due to increased systemic vascular  resistance)   e Failure of septation (atrial septal defect—ASD, ventricular  septal defect—VSD, tetralogy of Fallot—TOF).   ¢ Mixing of systemic and pulmonary flow (similar to  TAPVC): Heart failure in infancy.   Child adopts squatting posture during hypoxic spells which  Increases systemic vascular resistance and venous return to  heart. This directs the blood into pulmonary circulation and  oxygenation improves. Chest X-ray shows boot-shaped heart.  Treatment—single or two stage surgery (Key Box 44.15).   COMPONENTS  1. VSD  2. Overriding aorta  3. Pulmonary stenosis  4. Right ventricular hypertrophy    2. Transposition of great vessels (TGA)  e Most common cause of cyanosis in newborn.    e Aorta arises from right ventricle and pulmonary artery from  left ventricle.    ¢ Rashkind percutaneous balloon atrial septoplasty.  ¢ Definitive repair—arterial switch (2 stage)  ¢ Mustard or Senning surgery.    ¢ Pulmonary venous drain disconnected from left atrium and  drains to systemic circulation, e.g. inferior vena cava (IVC)  or superior vena cava (SVC).   ¢ Itoccurs following reversal of a previous left to right shunt  as with ASD or VSD.    Definition  Persistence of the foetal ductus arteriosus in the postnatal  period.    ¢ Blood ejected by right ventricle flows exclusively through  the ductus to the lower extremity and placenta bypassing  the high resistance pulmonary circulation in the foetus.   Shunt occurs across the ductus both in systole and diastole  resulting in left ventricle overload and pulmonary plethora.  e Shunt depends on the size of the ductus and pulmonary    and systemic vascular resistance. Continuous murmur 1s  due to shunt during both systole and diastole.   ¢ It depends on the size of the ductus, pulmonary vascular  resistance, age at presentation and associated anomalies.  a. Small ductus: Asymptomatic  b. Infants: Congestive heart failure  c. Children: Dyspnoea on exertion, repeated respiratory   e Machinery or continuous murmur (Gibson’s murmur) in  the left second intercostal space, radiating to the left  infraclavicular area   e Presence of PDA is sufficient indication for surgery even  without symptoms.   e It can also be done by thoracoscopy and video-assisted  thoracoscopic surgery (VATS) and clipping of PDA.    Care during surgery  Left recurrent laryngeal nerve must be carefully preserved    Complications during surgery  ¢ Haemorrhage, left recurrent laryngeal nerve palsy  ¢ Chylothorax   Congenital narrowing of the descending thoracic aorta usually  occurring just distal to the left subclavian artery origin, adjacent  to the site of insertion of the ductus arteriosus.    ¢ (.2—0.6 per 1000 live births  ¢ 5-8% of all cases of congenital heart diseases.    Flow theory: Reduced flow in the aorta due to multiple  abnormalities in the heart during the foetal period.    Ductal sling theory: When there is isolated coarctation, this  theory is applicable. Abnormal extension of contractile ductal  tissue into the adjacent aorta which results in coarctation.    Depend on: Age and symptoms at presentation, location of  the coarctation, severity of the coarctation, associated  anomalies.    Signs  ¢ Systolic murmur heard over the precordium and  posteriorly between the scapula.  ¢ Feeble femoral pulses.  ¢ Enlarged collateral vessels are seen (Suzman’s sign) and  palpable between scapula and bruit can be heard here.  ¢ Systolic gradient between arm and leg blood pressure.    Classic ‘three’ sign: Formed by dilated left subclavian  artery, narrowing of the coarctation and the poststenotic  dilatation of aorta.   COMPLICATIONS  e Circle of Willis’ aneurysm rupture  e Aortic dissection  e Early coronary atherosclerosis  e Bacterial endocarditis  ¢ Congestive heart failure  e Spontaneous rupture of aorta    ¢ Lower limb pressure during cross-clamping of aorta, to  be kept above 45 mmHg    e Ifthe pressure 1s low, then create a shunt from the aortic  arch to the descending thoracic aorta with the help of a  cannula.   e Haemorrhage, RLN injury, phrenic nerve injury  ¢ Horner’s syndrome, chylothorax    ¢ Recoarctation, aneurysm and left arm ischaemia (when  subclavian flap aortoplasty is done).    Rare condition which results from congenital elongation of  the aortic arch which leads to redundancy and kinking of the  aorta and may appear similar to coarctation but there is no  obstruction to blood flow.    Defect in septum between left and right atrium leading to left  to right shunt.    ¢ Ostium secundum: Defect in floor of fossa ovalis.  Presents late in life.   ¢ Ostium primum: Endocardial cushion defect associated  with mitral valve defects (mitral regurgitation) and  trisomy 21. Presents earlier in life.    Open heart surgery with CPB and closure of defect with sutures  or with pericardial or synthetic patch.    ¢ Perimembranous defect (in membranous septum):  70-80%  ¢ Muscular defect (multiple): 10%  ¢ Atrioventricular defect: 5%  ¢ Subarterial defect: 5—10%  O/E: Pansystolic murmur  Treatment: Surgical repair    Often simply called bypass surgery, it is the most commonly  performed open heart surgery all over the world. The  incidence of coronary artery disease 1s rapidly rising in the  developing countries. A vein graft from the lower limb or an  arterial graft (internal mammary artery or the radial artery)  is used to bypass the obstructed coronary artery. The bypass  is done from the root of the aorta to the distal coronary artery.  ¢ The obstruction or the block is not touched.   RISK FACTORS  I. Nonmodifiable  e Age, sex, family history  ll. Modifiable, controlled or treated by medicines:  e Smoking, uncontrolled diabetes  e Uncontrolled hypertension  e Obesity, overweight, lack of physical exercise  e Individual response to stress  e High blood cholesterol or lipid abnormalities   1. Usually indicated in symptomatic or for prognostic reasons  (i.e. balance between expectant benefit and risk faced by  patient).  a. > 50% stenosis of left main stem artery  b. > 70% stenosis of proximal left anterior interventricular   c. Triple vessel disease  d. Poor ventricular function associated with CAD.   4. Surgery for complications of MI such as ventricular septal  rupture, etc.    With interventional cardiology progressing rapidly the  indications for surgery are getting blurred.    2. Grafts  ¢ Veins—lower limbs (long saphenous vein)  ¢ Upper limb (cephalic vein) rarely used  ¢ Arteries—radial (Key Box 44.20)  e Internal mammary artery both right and left  ¢ Gastroepiploic  ¢ Inferior epigastric  ¢ Inferior mesenteric   e Dense nonfenestrated intact internal elastic lamina that  inhibits cellular migration and subsequent initiation of  hyperplasia   e Thin medial layer with a few smooth muscle cells which  provide a little vasoreactivity    ¢ Cryopreserved human saphenous vein allograft  ¢ Processed bovine sacral artery  ¢ Polytetrafluoroethylene graft (Gore-Tex)   * Aorta cross-clamped and cold K* rich solution given in the  aortic root   ¢ Inserted percutaneously in common femoral artery, threade  into aorta till its tip lies in distal arch vessels.   ¢ Balloon is triggered by the ECG, deflating during ventricula  systole (decreasing afterload) and inflating during diastol  (increasing diastolic pressure and blood flow to coronaries)    ¢ Creation of channels | mm in size in the myocardium from  epicardium to endocardium   ¢ Allows perfusion of blood directly from the LV cavity to  the intramyocardium   ¢ Creation of intramyocardial channels, neoangiogenesis and  denervation are the factors which improve perfusion to the  ischaemic myocardium.   ¢ Patients with total arterial grafts may not require future  surgery.   e Arterial grafts are used in the shape of T or Y.   ¢ CPB standby, all vessels can be bypassed by proper position  of heart, quick recovery, early extubation   ¢ No CPB, less trauma, reduce risk of bleeding and kidney  failure, reduced hospital stay.   3. Myocardial protection during CPB (ventricular assist  devices). Ischaemic preconditioning pharmacologically  preoperatively.   4. Vascular intimal hyperplasia: Important cause of graft  occlusion. Gene-based therapies to prevent this before  grafting the veins.   5. Minimally invasive direct coronary artery bypass  (MIDCAB) surgery: Anterior submammary incision.  LIMA dissected down with the aid of a thoracoscope and  grafted to the LAD.    ¢ Aneurysms are defined as a focal dilatation of at least 50%  larger than the expected normal arterial diameter  ¢ For AAA—transverse diameter 3 cm or greater    Most common type of true aneurysm with high  propensity to rupture and it ts the 15th overall leading  cause of death.    Growth rates  < 5cm diameter—0.3 cm/year > 5 cm diameter—0.5 cm/year.   2. Undergoing evaluation for another abdominal pathology  3. Surgery for unrelated abdominal or pelvic operation.    1. Sudden-onset midabdominal or flank pain  2. Shock  3. Presence of a pulsatile abdominal mass (expansile pulsation).    3. If upper border is palpable, then the origin is probably  infrarenal.   5. Ischaemic injury to the spinal cord or lumbosacral plexus  6. Postoperative sexual dysfunction    Low-risk AAAS  ¢ Followed with serial size measurements. Reduce expansion  rate and rupture risk by conservative treatment such as:    1. Smoking cessation  2. Blood pressure control  3. Reduction of cholesterol    2. Retroperitoneal approach ts indicated in cases of hostil  abdomen, suprarenal aneurysm, horseshoe kidney  peritoneal dialysis, inflammatory aneurysm and in ascites    3. Minimal incision aortic surgery  12 to 15 cm incision, 9 cm proximal to the umbilicus.    Stent-graft is introduced into the aneurysm through thi  femoral arteries and fixed in place to the nonaneurysmal aorti  neck and iliac arteries with self-expanding or balloon  expandable stents (Palmaz stents), or with barbs, pins o  hooks.    3. Horseshoe kidney  a. Kidney usually fused anterior to the aorta  b. Left retroperitoneal approach    e The most common cause of respiratory insufficiency  following chest trauma is pneumothorax.   e Most common cyanotic congenital heart disease is tetralogy  of Fallot.   e Most common cause of cyanosis in newborn is transposition  of great vessels.   Back and abdominal pain, pallor, diaphoresis and syncope. If | * Most commonly performed open heart surgery all over the    e Most common type of true aneurysm with high propensity  to rupture is abdominal aortic aneurysm.    mass, he is transferred immediately to OT.  ; ; ! is renal failure.  ¢ If stable + questionable diagnosis, a CT and open surgical  repair, control the haemorrhage + resuscitation + aneurysm  repair. Early postoperative mortality rate is 45%.    ¢ Angina pectoris, perforated peptic ulcer  ¢ Acute pancreatitis, acute cholecystitis   All topics have been updated.  e Mediastinal tumours, bronchogenic carcinoma have  been included.    . Fracture of the following rib is a marker of severe   B. Drainage of more than 100 ml/hour for 4 hours  C. If clotted, haemothorax is suspected    . The intercostal drain can be removed in all of the    The most common cause of noncardiac complicatior  after abdominal aortic aneurysm repair is:    The most common cause of respiratory insufficiency  following chest trauma is:    ANSWERS  2 © 3 A 4 C 5 B 6 C 7D 8 D < ©: 10 B  A 12 D 13 A 14 C 6 B    Head injuries derive their importance because of the fact that  many patients who die or who are disabled belong to the  younger age groups. Head injuries account for 1% of all deaths,  one-fourth of deaths due to trauma and they are responsible  for half of all deaths from road traffic accidents. Majority of  the patients are young, adult males.   ¢ The term open head injury is used to denote a type of  injury in which there is a fracture of the skull associated  with tear of the dura and arachnoid, resulting in  cerebrospinal fluid leak either to the external environment  or into one of the potentially infective areas in the base  of the skull, e.g. CSF rhinorrhoea or otorrhoea.    ¢ A closed head injury is one where there is no such  leakage. The advantage of this classification is that it helps  the treating physician to recognise a group of patients who    are likely to develop an infective complication following  the head injury and he can initiate measures to prevent it.   « Blunt injuries, depending on the severity of impact can  result in an open or closed head injury. Missile injuries tend  to result in an open head injury most often.   ¢ The brain is protected by a bony box which has a vault  and base of the skull. The base of skull in contrast to the  vault is a rough terrain due to the various bony  prominences, ridges and foraminae.   ¢ This factor is important in causing extensive brain damage  to the brain in acceleration/deceleration type of injuries. In  addition to the linear acceleration/deceleration, rotational  acceleration is also capable of producing damage to the  brain as the brain swirls about inside the skull. Such  injuries result in maximal damage at interfaces between  structures of different densities such as grey matter-white  matter junctions.    The pathological changes due to trauma to the brain can be  classified into primary and secondary.    ¢ Diffuse neuronal damage is the most constant feature of  blunt injuries. Immediately after an injury no changes may  be seen but changes begin after 14 hours of injury and  maximum effects may last up to one week. Prolonged  unconsciousness may follow injuries which produce only  diffuse neuronal damage without any obvious macroscopic  changes. Shearing lesions of the nerve fibres account for  some severe injuries without any conspicuous changes to  naked eye examination of the brain. A few important  primary lesions are discussed below.   ¢ Cerebral concussion: Alteration in consciousness without  structural damage as a result of non-penetrating traumatic  brain injury. There might be loss of consciousness,  confusion and amnesia. These are the features. Widespread  degeneration of white matter occurs without much changes  in the nervous system cortex or brainstem. These patients  have spasticity in all four limbs after injury and when they  regain consciousness, they are found to be severely  demented (Key Box 45.2).   ¢ Contusion and lacerations are the obvious naked eye changes  seen after injuries and were thought to be the main injuries  before diffuse neuronal damage and shearing lesions were  described. Contusionsareseenon the summit of the gyri which  get injured against the bone. The overlying pia is torn and  the blood seeps into the subarachnoid space. A bleeding  cortical vessel may result in the formation of the acute  subdural haematoma or intracerebral haemorrhage.  Brain oedema which develops surrounding the contusion  and lacerations 1s the one that determines the outcome. Most  often contusions are seen at the tips of the frontal and  temporal lobes, under surface of frontal and temporal  lobes, over corpus callosum, superior and anterior surfaces  of cerebellum and anterior surface of brainstem.   CEREBRAL CONCUSSION  e Temporary physiological paralysis of the nervous system  e Loss of consciousness  e Post-traumatic amnesia  e Recovery may be complete  e Some can develop complications    Brain swelling: This is a vague term applied to increase ir  brain bulk due to both oedema and venous congestion. It 1:  aggravated by hypoxia or respiratory insufficiency which may  be due to associated lung injury or obstruction to uppe)  respiratory passages. Sometimes such a swelling can lead tc  severe brain compression which is difficult to relieve, since  there 1s no single mass lesion.    Malignant cerebral oedema has close to 100% mortality.  This ts more common tn children.    Intracranial haemorrhage: Extradural or subdural haemorr-  hages may develop as a clean cut secondary event, even though  bleeding may have started at the time of injury. These cause  compression of brain, secondary rise in intracranial pressure  and can cause death if not detected and treated early.    Infections: All open head injuries are liable to result in  Intracranial infection either as generalised meningitis or focal  infection such as subdural empyema or brain abscess,  osteomyelitis of skull. After closed head injuries, infection  of a subpericranial blood clot may result in Pott’s puffy  tumour. When infection supervenes on an already injured  brain, it may retard the recovery or may even lead to death.  Hence, 1t becomes mandatory to treat all infections vigorously.    e [tis instructive to consider the pathological findings in fatal  cases and to speculate the deaths which might have been  prevented. For example, earlier many deaths which had  occurred as a result of aggravation of brain swelling due  to hypoxia could have been prevented by ventilation and  anti-oedema measures. It should be emphasised that the  role of the treating physician is to anticipate and take   SECONDARY LESIONS  1. Brain swelling  e Oedema  e Venous congestion  e Hypoxia  2. Intracranial haemorrhage  e Extradural  e Subdural  3. Infections  A. Open head injury  e Generalised meningitis  e Subdural empyema  B. Closed head injury  e Pott’s puffy tumour    appropriate measures to prevent the patient from  succumbing to the secondary injuries (damages—seizures,  hypoxia). In extensive primary damage to the brain,  apart from supportive treatment, one may have to wait  and hope.   Extensive injury to vital areas such as diencephalon, or  patients with diffuse damage are not likely to survive.  These are patients who are unconscious from the time of  injury with bilateral, dilated, fixed pupils, flaccidity in all  4 limbs and autonomic disturbances.   Sometimes a head injury associated with extensive injuries  to chest, abdomen or the limbs by their sheer severity can  cause death.   Intracranial complications such as haematomas, brain  swelling, infection, and extracranial complications such as  chest injury/metabolic abnormalities, if recognised and  treated early, can go a long way in saving the life of the  patients.    ¢ Most of the head injuries are mild or minor and irrespective    of how they are managed, the patient recovers on his own.  All those who are unconscious, even if briefly, run the risk  of respiratory obstruction. Some of the so-called trivially  injured run the risk of developing an intracranial  haematoma. Hence, all head injuries must be taken  seriously. A complicated head injury is one where anyone  of the secondary pathological changes may occur and  threaten the life of the patient. Uncomplicated head injury  is one where no such events occur. However, it could be a  severe one where the unconsciousness 1s prolonged.  These haematomas could develop in any one of the planes  intracranially. Extradural (epidural), subdural, intracerebral  haematoma, or a haemorrhagic contusion.   The clinical presentation of these haematomas are due to  either increase in the intracranial pressure or due to signs  of cerebral compression. In the case of acute subdural  haematomaor intracerebral haematoma, the clinical picture  and the outcome of treatment is also dependent on  associated brain damage.    The clot collects between the dura and the inner table of  skull. A majority of them occur in the middle cranial fossa,  since injury to middle meningeal vessels (vein and artery)  is the commonest cause. However, about 20-25% of the  extradural haematomas can occur in the frontal, parietal  regions, at the vertex or in the posterior fossa. Injuries to  the dural venous sinuses or a large diploic venous channel  are the other causes for the formation of a haematoma.  Depending upon the source of bleeding, the haematoma  could collect rapidly (hyperacute type) or slowly over a    period of a few hours to a few days and present as a chronic  lesion. 60-80% of these patients have an associated fracture  of the skull bone and only a few of them may present with  classical symptoms with lucid interval. In the remaining  patients, the initial picture can vary from an unconscious  state to a fully conscious person with or without a history  of post-traumatic amnesia. With the widespread availability  and use of CT scan, the diagnosis has become much simpler  nowadays. However, a few clinical features are worth  mentioning.    ¢ This is one of the hallmarks for the diagnosis of intracranial  haematoma. The term ‘lucid interval’ is used when a patient  recovers from an initial period of unconscious state. Though  in earlier days this was said to be associated with intracranial  haematomas, it can occur in other conditions such as brain  oedema, multiple contusions. To assess the consciousness  level properly, instead of using vaguely defined terms such  as semiconscious, obtunded, etc. the ‘Glasgow Coma  Score’ is widely used, to avoid observer errors in the  observation of such patients.   ¢ Restlessness in a previously quiet patient indicates  increasing intracranial pressure, which again needs to  be investigated. At the earliest appearance of focal neuronal  deficit, the patient has to be taken up for exploratory burr  holes.   ¢ Progressive neurological deficit indicates cerebral  compression and the manifestation may depend on the area  of the brain affected.    These are to be considered as a late manifestation. It 1s due to  pressure by the herniating temporal lobe on the ipsilateral  third nerve at the tentorial hiatus. In the early stages due  to irritation of the nerve, there 1s constriction. Since it is a  transient phenomenon, the early constriction goes unnoticed  most of time. The patients are often detected in the next stage,   i.e. pupillary dilatation, caused by paralysis of  pupilloconstrictor fibres in the third nerve. However, if the  cerebral compression 1s unrelieved, this may go onto bilateral.  Pupillary dilatation 1s due to ischaemia of third nerve nucleus  at the midbrain which 1s caused by pressure on the posterior  cerebral artery. These series of pupillary changes have been  termed Hutchinsonian pupils. The dilated pupil has a definite  localising value in that if an exploratory burr hole has been  decided upon, it should be done on the side of the initially  dilated pupil.    Bradycardia, though said to be a definite sign, is a late and not  an early sign. Initially, there may be a rise in the pulse rate  (tachycardia) which may progress to bradycardia, when the  systolic blood pressure increases. At times there may be a rise  in the diastolic pressure also. These changes occur due to  changes in the cerebral blood flow as a consequence of  increased intracranial pressure. Respirations become deep  and slow rate (bradypnoea) and later patients may develop  Cheyne-Stokes ventilation due to brainstem ischaemia.    ¢ Local scalp swelling is seen 1n more than half of the cases.  Thus, examination of the head for any such swelling  becomes important.   ¢ Some of these patients may have a stiff neck either due to  increased intracranial pressure or due to associated injury to  neck muscles. Mild fever may, at times, occur and this  sometimes confuses the observer. In such a case, the patients  must be investigated with a definitive investigation like CT  scan. If much time 1s not available, one should not hesitate  to proceed to exploratory burr holes or a ‘trauma craniotomy  flap’ has to be employed to rule out a haematoma.   ¢ Though in adults, ‘shock’ is a rare complication of head  injury, in children with intracranial haematoma and  associated cephalhaematomas, due to volume depletion  ‘shock’ may be encountered. Even in adults, if there 1s a  large scalp injury which is not sutured immediately, shock  can occur.   ¢ Posterior fossa haematomas in any plane are dangerous  because of the lesser space available for the haematomas.  As a result of this, rapid brainstem compression can occur  which may prove fatal. The availability of CT scan has made  detection of these so-called ‘unusual haematomas’ more  frequent. In a suspected case, even if facilities are not  available, the treating physician should explore the  posterior fossa, if the clinical features suggest haematoma,    or 1f the skull X-rays show a fracture line extending across  the occipital bone towards the foramen magnum.    As has been pointed out earlier, the advent of CT scan of the  head has made the diagnosis easier and more specific.  However, it should be emphasised that in the absence of CT  scan, 1f adequate clinical features point out to the possibility  of an intracranial haematoma, the patient must be taken up  immediately for an exploratory surgery rather than wait and  allow him to develop irreversible brainstem damage. Since  60-80% of patients with an intracranial haematoma have a  skull bone fracture, irrespective of his consciousness level has  to be observed for at least 24-48 hours. Occasionally, one  may have to resort to old investigations such as angiography  not only to establish the haematoma but also to rule out  associated dural venous sinus injury.    1. Admission is indicated when:  a. Definite history of unconsciousness  b. Fracture temporal bone  c. Person who cannot be attended by the doctors  immediately, i.e. no medical facilities nearby.  d. Post-traumatic seizures—patient should be admitted.  2. Casualty reception  a. Airway  ¢ Mouth gag—to prevent tongue falling backwards.  ¢ Endotracheal intubation with positive pressure ventilation.  Hypoxia is an important cause of cerebral oedema which  worsens the level of consciousness.  b. General assessment of patient  ¢ Torule out abdominal injuries such as splenic rupture.  ¢ Haemothorax—may need an intercostal tube.  ¢ Long bone fractures  c. General assessment of the degree of shock by pulse,  blood pressure monitoring and treatment.  d. Neurological assessment by Glasgow Coma Scale    To pain  None  2. Best verbal response  Oriented  Confused  Inappropriate words  Incomprehensible sounds  None  3. Best motor response  Obeys commands  Localises the pain  Withdrawal to pain  Flexion to pain 3  Extension to pain 2 (severe damage  with increase of ICP)  None l  ¢ Total score is 15; minimum score 1s 3. Any patient who  has acoma score of 8 or less than 8 is said to be in coma.    Immediate surgery for removal of haematoma and relief of  cerebral compression is a must. Extradural haematoma, in  particular, is a neurosurgical emergency and patient survival  will depend upon the speed with which the compression 1s  relieved. It is not an exaggeration to state that even if  decompression has to be done with unsterile instruments, at  the bedside it may be worththe effort. In every neurosurgeon’s  career, at least one such situation might have occurred and a  live patient may justify the means employed. Once  consciousness 1s lost, pupils are dilated and decerebrate rigidity  and periodic breathing develop, it may be only a few minutes  that may be available to save the life of the patient and one  should not wait and waste time. In the case of extradural  haematoma the outcome is dependent on the size of the  haematoma and the stage in which the patient was taken up  for surgery. In the case of acute subdural and intracerebral  haematoma it depends on associated brain damage. If the  associated brain damage Is very severe, patients succumbs to  the brain damage (Key Box 45.5 and Fig. 45.2).    Impact damage 1s more as compared to epidural haematoma.  There is associated underlying brain injury. Symptoms are due  to compression of underlying brain with midline shift, in  addition to parenchymal brain injury.    e Extend the burr hole and control bleeding middle-  meningeal artery by bipolar diathermy   ¢ In the elderly, the distance between the dura and the brain  increases because of the shrinkage of the brain. Even a  minor trauma can tear the cortical veins resulting in  collection of blood   ¢ Bleeding ts never progressive and the blood in the subdural  space slowly compresses the brain causing features of raised  intracranial pressure (ICP).   ¢ Slowness, confusion—later alteration in the level of  consciousness may progress to unconsciousness.   ¢ Waxing and waning of the level of consciousness Is seen in  some patients. If such a history ts elicited, one should always  suspect chronic subdural haematoma.    Diagnosis  CT scan or, if feasible, MRI scan are the ideal investigations  (cerebral angiography had been used and 1s still being used in   some centres where access to the latest imaging facilities are  not available).    Burr hole and drainage of the haematoma usually under  local anaesthesia or occasionally under general anaesthesia  is Often the practised mode of treatment.   At times, the patient may need two or more burr holes to  ensure adequate evacuation.   If the brain fails to expand and obliterate the cavity,  especially in older people or in persons with a very thick  inner membrane, a large craniotomy and wide excision of  the subdural membrane has to be carried out to remove the  constricting effect.   Adequate bedrest and plenty of fluid administration are also  important postoperative measures.    Measures to reduce the raised ICP  Aim is to keep ICP 20 mmHg   Steroids in severe head injury are associated with increased  mortality and should not be used.    Fracture cribriform plate can result in CSF rhinorrhoea.  Fracture may extend to the orbit—subconjunctival  haemorrhage.   Optic nerve may be contused or fracture may involve the  optic foramen resulting in partial or total loss of vision.  Rarely, 3rd nerve palsy gives rise to dilated pupil.   CSF from the ear: Blood mixes with CSF and so, does not  clot.    Extravasation of blood in the suboccipital region causing  boggy swelling in the nape of the neck.    There should be a communication between the intradural  cavity (subarachnoid space) and the nose.    It indicates tear of the dura mainly in the basal region and a  fracture involving paranasal sinuses—frontal, ethmoidal or  sphenoidal.   There is always an injury to a small portion of the brain. It  (the portion of brain) plugs the tear, preventing the dura  from healing. Thus, the rhinorrhoea persists for many days.   1. Traumatic: It can be iatrogenic following surgery or  can be post-traumatic (62 to 80%).   2. Nontraumatic: It can be due to high pressure  (hydrocephalus) or congenital.   2. Reservoir sign: Gush of CSF in certain position of the  head.    Acetazolamide 250 mg three times a day  Lumbar drainage can be done  Prophylactic antibiotics    If the rhinorrhoea persists, repair of the dural defect alone,  (or) at times with a shunt procedure will be needed.    This is subperiosteal infection usually caused by  osteomyelitis of the underlying skull.    It is common in the frontal region and the frontal bone is  commonly involved.    Another common cause of infection of a subpericranial  haematoma following needle aspiration.   Pus collects in the subpericranial space and extradural plane,  which communicate with each other (dumb-bell type  abscess).   It causes a boggy swelling in the frontal region and  tenderness over the scalp.   e Severe headache, vomiting and blurring of vision should  clinch the diagnosis.   2. A burr hole and aspiration of pus can be done followed by  6-8 weeks of antibiotics.   3. In chronic cases, the wall of the abscess may have to be  removed. The associated osteomyelitic skull bone requires  a radical removal under cover of antibiotics.   This 1s a condition that occurs due to disturbances of CSF  flow and imbalance between CSF production and absorption  resulting in the accumulation of CSF and dilatation of  ventricles.    CSF is mainly produced by the choroid plexus of lateral  ventricles by an active autoregulated process.    CSF leaves the 4th ventricle by foramen of Luschka and  Magendie to circulate over the convexity where it is finally  absorbed over arachnoid granulations.   A. Overproduction: True overproduction is rare and occurs  in cases of choroid plexus papilloma.   B. Decreased absorption: Failure of CSF absorption is much  more common due to infection and haemorrhage. Other  causes being: Structural abnormality occurring in CSF  pathway—tumour, congenital malformation such as  aqueduct stenosis.   ¢ Inability to retain feeds, mental retardation, delayed  milestones, hypothalamic disturbances.    I]. Childhood/adult hydrocephalus  ¢ By this time fontanelles have closed  ¢ Features of increased intracranial pressure: Headache,  nausea, vomiting.  ¢ Irritability, indifference, apathy, drowsiness   DIFFERENTIAL DIAGNOSIS OF LARGE HEAD  1. Megalencephaly Intracranial pressure is normal  2. Chronic subdural Enlargement of parietal  haematoma region  3. Cerebral atrophy May cause ventricular  enlargement  4. Cerebral tumours   ¢ Bradycardia, systemic hypertension, altered respiratory rate  is due to distortion of brainstem. Untreated cases also  develop unilateral or bilateral abducens palsy or upward  gaze palsy.   1. To decrease CSF production by using pharmacological  agents:  ¢ Acetazolamide  ¢ Furosemide  ¢ Isosorbide  ¢ Glycerol   3. Diversion of CSF to another viscous for reabsorption by  means of various shunt procedures.    COMPLICATIONS OF SHUNTS  . Shunt obstruction  . Shunt infection  . Seizures  . Extracerebral fluid collection  . Subdural haematoma  . Spontaneous pneumocephalus  . Ascites    Eight percent of all primary cancers arises in the CNS. Ir  adults, it constitutes the sixth largest group of cancers and Ir  children the CNS is the commonest site for solid tumours.    Most adult brain tumours are supratentorial but 60% of  tumours in children are infratentorial and arise in the  posterior fossa.    1. Progressive neurological deficit: In up to 2/3rds of the  patients, a focal motor weakness is the most common  presentation. This deficit arises from the direct neuronal  damage or are due to compression on brain or cranial nerves  by the tumour. Sudden change may be due to haemorrhage  into a pre-existing tumour.    2. Headache: It occurs in over 50% of patients caused by  raised intracranial pressure (ICP). Only 10% patients have  the classic presentation of headache with early morning  worsening, associated with nausea and a temporary relief  by vomiting.   3. Generalised or focal seizures occur in 25%. Focal seizures  may help to localise the site of tumour. A high index of  Suspicion is required for patients past the second decade  with recent onset epilepsy.    4. Other presentations include visual disturbances, visual  field defects, mental changes, etc. depending on the site of  tumour.    1. CT scan: The best initial investigation. This shows where  the tumour alters the attenuation of the X-ray beam as it  passes through the brain. It also shows distortion of the  ventricular system or obliteration of the pattern of the sulci.    2. MRI: Often gives extra information and may eventually  supersede CT completely. It is the investigation of choice  for imaging the posterior fossa and the spine as it can ‘see  through’ bone.    3. X-rays are only of anecdotal interest picking up incidental  calcifications or erosion of parts of the skull. Newer imaging  modalities including SPECT and PET scans are increasingly  being used in higher centres.   ¢ This 1s the most common primary brain tumour arising  from the supporting glial cells and diffusely infiltrates brain  tissue early on.   ¢ Two grading systems are available: WHO and St Anne/  Mayo. Grade | and Grade 2 (low grade) tumours are slow  growing and compatible with good quality survival. Grades  3 and 4 are rapidly growing. Grade 4 tumours are also called  glioblastoma multiforme are highly radio and chemo-  resistant with median survival of only 12 months even with  optimal treatment. Even low grade astrocytomas may evolve  over time into secondary glioblastomas.   ¢ Treatment includes surgery to confirm the diagnosis and  achieve a macroscopic excision, followed by a high dose  (60 gy) of irradiation.    ¢ This type of glioma Is usually a slow-growing tumour and  over half arise in the frontal lobes.   ¢ There may be a history of epilepsy or even focal  neurological signs of many years’ duration.    ¢ These glial tumours arise from cells that line the ventricles  of the brain and the central canal of the spinal cord.   ¢ They are most common in the fourth ventricle in children  and young adults where they block CSF flow and often  present with hydrocephalus.    e Primitive neuroectodermal tumours (PNETs) are a group  of highly malignant tumours of which the cerebellar  PNET or medulloblastoma ts the archetype.   ¢ Commonest in children and young adults. It may originate  from primitive cell nests which have undergone malignant  transformation. Medulloblastoma 1s the most common brain  tumour in children.   ¢ The patient presents with truncal ataxia, headache, vomiting  and sometimes diplopia. All PNETs are prone to spread  within the CNS producing ‘sugarcoating metastasis’ which  are best seen on MRI of the spine.    ¢ Peripheral neurons get their myelin sheaths from Schwan  cells. Occasionally, these form slow growing benig1  tumours on cranial or spinal nerves.   ¢ The cranial nerve most affected is the vestibular divisio1  of the 8th cranial nerve and the tumour ts then usuall  called acoustic neuroma.    Even though it does not arise from the acoustic division  and is not a neuroma, it is still called an acoustic  neuroma.    ¢ It causes progressive deafness, hydrocephalus and ataxia  It is slow growing. Less often it may involve the trigemina  or the vagus nerve.   ¢ These tumours arise from the arachnoid layer of the  meninges and the arachnoid villi. They are commonest over  the falx and the convexity of the skull but rarely may also  arise from the skull base (especially from the sphenoid wing  and olfactory groove) or inside the lateral ventricle.    ¢ Commonest in middle-aged women and may occur at the  site of a previous radiation field. Most are benign and slowly  growing. Signs occur based on the site of tumour (Key Box  45.8).    ¢ They tend to provoke endosteal hypertrophy or exostosis  of the overlying skull and 1s still occasionally detected on  an incidental skull film or even by palpation of the skull.  Patient with neurofibromatosis type 2 often have multiple  meningiomas. Rarely, they are malignant.    e Convexity of skull 20%  e Parafalcine arc 20%  e Sphenoid ridge 10%  e Tuberculum sella 10%  e Olfactory groove 10%  e Ventricle 2-5%   ¢ Endocrine dysfunction such as galactorrhoea, primary/  secondary amenorrhoea, Cushing’s syndrome, acromegaly.   ¢ Pituitary apoplexy—tresults in sudden onset of headache,  visual loss, ophthalmoplegia and altered conscious level.  Caused by haemorrhagic infarction of a pituitary tumour.    They are pineal region tumours, pituitary adenomas,  craniopharyngiomas, choroid plexus tumours, etc.    One-fourth of all cancer patients have intracerebral metastases  at the time of death. Common primary sites include the  bronchus (50%), breast (15%), and melanoma (10%).    They can be divided into medical and surgical line of treatment.   ¢ Acutely raised intracranial pressure (ICP) 1s treated with  IV mannitol 0.5—1 g/kg body weight.   ¢ Hydrocephalus can be relieved using a CSF diversion  system (closed external ventricular drainage or a  ventriculoperitoneal shunt).   ¢ Seizures are treated with lorazepam and phenytoin  commonly and maintained on phenytoin.   ¢ Corticosteroids, especially dexamethasone 4 mg qid is  given to reduce symptoms ofraised ICP, which may make  surgery easier.    e This is the mainstay of treatment. The aim of surgery  is to obtain a complete tumour excision without  producing a neurological deficit.   ¢ This is not always possible due to the site of the tumour,  in which case compromises must be made and a  debulking procedure is done.   e Any residual tissue may be observed or treated with  adjuvant radiotherapy.   e Stereotactic guided surgery is now a well-established  concept in brain surgery allowing more targeted treatment  of the lesion with minimal surrounding damage.    e Intracranial tumours are relatively radioresistant, and  radiotherapy is primarily a palliative treatment.    IV. Chemotherapy  ¢ No definite benefit of chemotherapy for the treatment o}  brain tumours. Temozolomide is a promising new drug  for the treatment of brain tumours.    Surgery offers good prospects for the treatment of benign brain  tumours such as meningioma or pituitary adenoma, but  outcome of treatment of malignant tumours 1s still poor.   e Severe episodic lancinating facial pain occurring in the  distribution of V cranial nerve.   ¢ Vascular compression of the nerve near the root entry zone,  multiple sclerosis may be causative factors.    ¢ Carbamazepine or gabapentin  ¢ 75% will not respond to medical treatment.  ¢ Surgery: Refractory cases  1. Percutaneous glycerol injection, radiofrequency ablation,  thermocoagulation, balloon compression, local nerve  block.  2. Microvascular decompression via craniotomy  3. Stereotactic radiosurgery    ¢ Pupillary reaction to light, corneal reflexes, vestibular ocular  reflex, cough reflex, gag reflex, motor response to pain.    Fig. 45.6: Right temporo-parietal extra- Fig. 45.7: Right temporal extradural Fig. 45.8: Postoperative scan-no EDH. Posi   toma. Left frontal and temporal nonhaemorrhagic frontoparietal and anterior interhemispheric right frontal, left temporal contusion,    Contributed by Dr. Sunil Upadhyay — Asst. Prof., Dept. of Neuro Surgery, KMC, Manipal   [fan exploratory burr hole has been decided upon, it should  be done on the side of the initially dilated pupil.   If hypotension responds to volume replacement in trauma  centre in a comatose patient, the coma is most likely not  due to head injury.   Admit the patient if there is fracture temporal bone.  Hypoxia is an important cause of cerebral oedema which  worsens the level of consciousness.   Steroids in severe head injury are associated with increased  mortality and should not be used.   CSF rhinorrhoea indicates tear of the dura mainly in the  basal region and a fracture involving paranasal sinuses.  Glasgow Coma Score of less than 8 means patient is in  coma.   . Following is not the feature of Glasgow Coma Scale: 6. The cause of extradural haematoma is bleeding from:  A. Eyes opening A. Venous sinuses  B. Motor response B. Cavernous sinus bleeding  C. Verbal response C. Basal veins bleeding  D. Sensory response D. Middle meningeal vessels bleeding  . Closed head injury include following except: fe Ee terval te typ redilly seen Mae  ie A. Extradural haematoma  A. Head injury with fracture skull  rb ; B. Acute subdural haematoma  B. Head injury with black eye  ak: C. Chronic subdural haematoma  C. Head injury with facial nerve palsy ih Bantnetiacestena  Di SICA TUT YAW EEL Sit taro reed 8. Which nerve is paralysed after herniation of temporal  paraly p  . Which are the following is included under secondary lobe in extradural haematoma?  lesions following head injury? A. Occulomotor nerve  A. Diffuse neuronal damage B. Ophthalmic nerve  B. Contusions C. Trigeminal nerve  C. Lacerations D. Facial nerve  D. Swelling 9. Pupillary dilatation following head injury is due to  . Post-traumatic amnesia is a feature of: ischaemia of the third nerve caused by:  A. Raised intracranial tension A. Middle cerebral artery  B. Fall from a height B. Posterior cerebral artery  C. Fracture skull C. Inferior cerebral artery  D. Cerebral concussion D. Anterior cerebral artery  Which i§ an important cause of brain swelling 10. Following are definite indications for admission in a  deilawing head iniury? head injury patient except:  A tate chen A. Fracture skull  B aoe B. CSF rhinorrhoea  C. Rieter C. History of unconsciousness  D. Bleediine D. Scalp bleeding  ANSWERS  1D 2 iD 3 D 4D 5 |B 6D 7A 8B 9B 10 D    These radiological investigations have become an integral part  of the management of the patients. A clear understanding of  these will help the students to read the images accurately,  interpret well and score good marks.   This is the study of the gastrointestinal tract by instillation/  ingestion of barium suspension made up of/made from pure  barium sulphate.    It is the contrast study from the oral cavity up to the fundus of  the stomach.    ¢ One mouthful of contrast media is given and the act of  deglutition 1s observed fluoroscopically. After a mouthful  of barium, films are exposed to the region of interest.   Fig. 46.1: Barium swallow: Opacified thoracic oesophagus  showing an intraluminal mass with mucosal irregularity in its lower  third suggestive of malignancy    1. Malignant obstructions are seen as annular constrictions,  shouldering cranial and caudal to the lesion, mucosal  destruction, ulceration and fistulae formation.   2. Benign strictures are long segment narrowings with no  mucosal abnormalities.    3. Achalasia cardia 1s evident as ‘rat tail’ appearance of the  lower end of the oesophagus, with gross dilatation of the  oesophagus proximally and thin streaks of contrast entering  the stomach.   4. Scleroderma shows dilatation, atonicity, poor or absent  peristalsis and free gastro-oesophageal reflux.   This 1s the radiological study of oesophagus, stomach,  duodenum and proximal jejunum.    An undiluted barium suspension is given and deglutition is  seen under fluoroscopy. Once barium reaches the stomach,  the patient is rotated so as to coat the entire stomach and filming  is done. More barium is given to distend the stomach wall.  Filming is done as contrast enters the duodenum and opacifies  proximal jejunum.    1. Hiatus hernia is evident as presence of the stomach above  the oesophageal hiatus. In addition gastro-oesophageal   Fig. 46.2: Barium meal: Contrast opacified stomach demons-  trating a projection from lesser curvature due to a benign gastric  ulcer (niche)    reflux will be evident. Mucosal ulceration and stricture:  may be demonstrable in long-standing cases.   2. Gastric and duodenal ulcers appear as projections frorr  the normal contour with pooling of contrast. Benign ulcers  usually project out and have the mucosal folds radiating  up to the edge of the ulcer. Deformity of the stomach anc  duodenal cap are seen in chronic stages.   3. Bezoars of stomach are seen as radiolucent masses in the  stomach and the barium fills the crevices between the  particles forming a characteristic appearance.   4. Infantile hypertrophic pyloric stenosis: Thin streak of  barium 1s seen extending across pylorus—indentation of  barium-filled antrum is seen.    It 1s the radiographic examination of the GIT-—oesophagus,  stomach, small bowel and ileocaecal junction, by oral  administration of contrast media.   ¢ Symptoms of small bowel disease such as diarrhoea,  abdominal pain, weight loss, Crohn’s disease.    A small mouthful of barium ts given after which the stomach  is Studied and films are taken. 500—800 ml barium Is then given  to the patient and filming 1s done after 15-20 minutes to  demonstrate the jeyunum and the proximal ileum. Subsequently,  films of the ileum and 1leocaecal junction are taken.    1. Intestinal malignancies such as lymphomas are evident as  strictures which may be short or long segment with or  without proximal dilatation. The mucosa shows irregularity  and ulcerations. Fistulous communications may be evident.  Displacement of bowel loops 1n large extraluminal masses  may be seen.    2. Inflammatory strictures are evident as sites of narrowing  with mucosal abnormality and ulcerations, proximal  dilatation and mucosal fold thickening.    This is the radiographic study of the large bowel by  administration of contrast media through the rectum.    1. Toxic megacolon  2. Pseudomembranous colitis  3. Rectal biopsy done recently (procedure withheld for 7 days)    ¢ Bowel is prepared with low residue diet, purgation and  cleansing water enema. High density barium suspension is  allowed to flow up to the ileocaecal junction and reflux  into the terminal ileum. Single contrast filming is done. The  patient is asked to evacuate the barium and a post evacuation  film is taken. Once barium is evacuated properly, air  insufflation is carried out so as to distend colon up to the  ileocaecal junction.   ¢ Filming is done to demonstrate the double contrast of large  bowel with additional spots of hepatic, splenic flexures and  rectosigmoid junction in oblique positions so as to open up  these regions.    1. Ulcerative colitis  ¢ Loss of haustral pattern  ¢ Fine granularity of mucosa  ¢ Fine ulcerations resulting in spiking of colonic surface  ¢ Strictures  ¢ Pipe stem colon, increase in presacral space    3. Tuberculosis  Ileocaecal region is the commonest site. Deformed, elevated  caecum, stricture and ulceration involving ascending colon  and ileum.    4. Crohn’s disease  Multiple ulcerations, thickening and distortion of valvulae  conniventes, short or long strictures, cobblestone pattern  and separation of bowel loops are the features.    5. Malabsorption  Dilution of barium, segmentation of the column of barium  ‘Moulage sign’ (barium in a featureless tube) and jejuna  dilatation are the findings.    SMALL BOWEL ENEMA OR ENTEROCLYSIS  (Fig. 46.3) (Key Boxes 46.1 and 46.2)    This is the radiological study of the small bowel (from jejunurr  to the ileocaecal junction) by intubation of the jejunum anc  instillation of contrast media through the tube.    ¢ Bilbao Dotter tube is inserted with the guide wire through  one of the nostrils and advanced caudally with the  swallowing action till the tip reaches the stomach. The  tube is then advanced through the antrum of the stomach  to the pyloric canal. Then it is advanced under  fluoroscopic guidance to about 4—S cm distal to the Trietz  ligament (duodenojejunal junction).   INDICATIONS  e Mechanical obstruction  e GIT bleeding  e Tumours of small intestine  e Unexplained abdominal pain  e Diarrhoea   CONTRAINDICATIONS 5  e Complete obstruction  e Suspected perforation  e Massive dilatation of small bowel  e Duodenal obstruction  e Gastrojejunostomy    e 200 ml barium suspension ts injected at a rate of 75 ml/  min followed by 5% of methylcellulose at a rate of  100 ml/min. The head of the barium column ts followed  with intermittent fluoroscopy and films exposed  wherever necessary.   * Ileocaecal spot films are taken when the junction 1s  opacified and distended.    1. Normal small bowel shows a decrease in calibre from  jejunum to ileum and the change of prominent valvulae  conniventes to featureless ileum 1s evident.   2. Malignancies and lymphomas show evidence of strictures,  proximal dilatations and mucosal abnormality. Large  mesenteric nodal masses displace the bowel loops.   3. Strictures and ulceration of terminal ileum: Dilatation of  the segment proximal to the narrowed segment and conical  shrunken caecum are seen in 1leocaecal tuberculosis. In later  stages, ileal strictures, fistulae, etc. may be seen.    Definition  This 1s the study of blood vessels by injection of a contrast  medium containing iodine into the vessel.   1. Primary vascular diseases such as vaso-occlusive disease,  aneurysm, arteriovenous malformation (AVM).   3. Cardiovascular disease such as recent myocardial infarction,  overt congestive cardiac failure.   Fig. 46.4: Arch aortogram: Contrast in the arch of aorta  demonstrating the major vessels arising from it    ¢ Local anaesthesia at site of puncture 1s preferred except in  children or restless patients, wherein general anaesthesia  is preferred. Using a Seldinger needle the artery 1s  punctured.   e The catheter of appropriate dimension 1s placed into the  artery and negotiated into the desired vessel to be studied.  Contrast 1s injected and filming is done.    1. Aneurysms are seen as focal dilatations of vessel or  projecting from the main vessel through a neck.    2. Tumour vessels show abnormal branching pattern, vascular  encasement, displacement, arteriovenous shunting and  pooling of contrast in the lesion.   3. AVM shows evidence of a dilated feeding artery/abnormal  blush and early draining vein.   4. Vascular occlusions are seen as abrupt or gradual tapering  of vessel with collateral supply distally.   ¢ Thrombosis of arteries, catheter clot embolus, haematoma  at puncture site    This is the contrast study of the portal venous system by  percutaneous splenic puncture.    ¢ Demonstration of the anatomy of the portal system in  patients with portal hypertension prior to surgery  ¢ Check the patency of a portosystemic anastomosis    Splenoportography needle is introduced into the spleen in the  midaxillary line under local anaesthesia. After confirming the    placement of needle in the splenic pulp, injection of iodine  containing contrast media is done and films are taken to includ  the splenic vein, portal vein and the portal radicles in liver.    ¢ Contrast injected into the splenic pulp drains into the hilun  through the splenic radicles and then through the spleni  vein and portal vein into liver.   e Thromboses are seen as filling defects in the contras  opacified splenic and portal vein.   e In severe portal hypertension multiple collaterals are seer  as branching channels from the normal pathway.    This is the contrast opacification of the duct and the glandular  acini by cannulating the ducts of salivary glands.   ¢ Post-secretory films are taken 5 minutes after administration  of a sialogogue to demonstrate sialectasis.    1. Stones in the gland are identified as filling defects and those  in the ductal system result in proximal obstruction and  dilatation.   2. Glandular enlargement due to inflammationresult in pooling  of the contrast injected.   3. Tumours of the salivary gland result in irregular filling  defect with duct distortion and pooling of contrast medium.    e Intra-abdominal and pelvic masses where exact site of origin  and relation to adjacent structures can be evaluated.    e Vascularity of the normal organ and the abnormal tissue  can be evaluated and compared.   Fig. 46.7: Cranial CT: Hypodensity of the left (L) frontoparietal  cerebral parenchyma suggestive of a (L) middle cerebral artery  territory infarct    Structures imaged appear densely white to densely black  depending on the absorption of X-rays and the emerging  resultant X-rays which are detected. The composite picture  is actually a collection of Hounsfield numbers. Each  Hounsfield number being assigned a specific shade of grey,  thus producing a picture that might be easily understood.  Some of the common densities to be encountered in practice  are as follows (Hounsfield units = HU):   CSF 0 to +3 HU  White matter De, tor+32, HU  Grey matter +36 to +46 HU  Clotted blood +60 to +80 HU   In order to increase the contrast that may exist between the  structures in the body, intravenous contrast (iodine  containing) is administered. Certain tissues show  enhancement of their density and various pathologies also  show fairly characteristic contrast uptake patterns (Figs 46.8  to 46.11).   In abdominal scanning, oral contrast is administered to  the patient before the procedure, to enable the operator to  accurately separate the bowel loops from the other intra-  abdominal structures.   The advantages of CT over conventional radiology are that  it can visualise extremely small pathology, not evident on  conventional films, is cost-effective as multiple X-ray films  and procedures can be avoided. It is noninvasive and the  radiation levels applied to the patient are extremely low.    This imaging modality is based on the piezoelectric effect  which is the property of certain substances to convertelectrical   Figs 46.8 to 46.10: CT scan axial section through liver shows large mass in plain scan. Brightly enhancing in arterial phase of contrast  study. Finally wash-out of contrast in portovenous phase which is a classical feature of hepatocellular carcinoma in multiphase contrast   Fig. 46.11: CT scan of abdomen: Contrast-enhanced scan  demonstrating a hypodense lesion in right lobe of liver posteriorly  due to an abscess    energy to sound energy. These are the active portions of the  ultrasonic transducers. The commonly used substance in the  transducer is lead zirconate titanate (PZT).    ¢ Ultrasonic beam of high frequency gives excellent resolu-  tion images of only superficial structures. This 1s used for  study of musculoskeletal system, joints, thyroid, scrotum,  etc.    ¢ Images are dependent on the intensity of echoes received  back by the transducer.   Fig. 46.12: Ultrasound of liver and gall bladder demonstrating  an isoechoic mass lesion occupying the lumen of gall bladder  which was due to malignancy    Fig. 46.13: Ultrasound of liver shows hydatid cyst which is anechoic  with multiple daughter cysts    e Structures which reflect all the sound waves back are  depicted as bright echoes and termed hyperechoic.   ¢ Fluid filled structures which transmit all the sound waves.  do not reflect any echoes and are termed hypoechoic.   ¢ The reflection of sound waves in the form of echoes depends  on the density of the organ and the transmission of sound  through the same.   . Owing to the relatively small size of the apparatus, it 1s  fairly portable, and can thus be brought to the bedside of  the moribund patient.   5. It does not involve the use of ionising radiation, and can   therefore be safely used in a pregnant patient and can be    Certain atomic nuclei, which possess unpaired protons or  neutrons, have an inherent spin. The nucleus ts positively  charged and therefore creates a small magnetic field around  itself, when it spins. The human body contains 1n abundance  such spinning nuclei in the atoms of hydrogen which 1s found  in water and lipids (Figs 46.14 to 46.18).   Fig. 46.14: MRI: T1 and T2 weight axial and coronal scans of Fig. 46.15: Soft tissue sarcoma involving the muscle of thigh.  normal brain The lesion is hyperintense in appearance   Fig. 46.16: Carcinoma tongue. MRI image shows hyperintense  lesion is involving the posterior and base of the tongue on the left Fig. 46.17: Intersphincteric fistula — axial section of MRI pelvis  side. The other bright structures seen bilaterally are parotid glands showing hyperintense fistula (arrow)   Fig. 46.18: Axial section of pelvis shows wall thickening of the rectal wall (arrow)    When the tissues containing these nuclei are within a strong  magnetic field, the nuclei tend to align themselves along the  lines of the force. The spinning protons now tend to precess,  i.e. wobble about the axis of the main magnetic field. Now a  radiofrequency (RF) is applied, being of the same frequency  as the processing but at right angles to the main magnetic field.  This excites the protons at low energy states into higher energy  states. Thus, an absorption of energy takes place, which 1s  used, as the excited protons ‘relax’ back to their original energy  level when the radiofrequency is switched off.   The relaxation of protons back to equilibrium and lower  energy state 1s termed spin-lattice relaxation or longitudinal  relaxation. It is exponential and referred to by the time  constant Tl. When the RF pulse ts applied the protons process  together in synchronism or in phase with each other. During  relaxation, however, they go quickly out of phase due to small  variations in local magnetic fields. This loss of phase 1s termed  spin-spin relaxation or transverse relaxation. It is also an  exponential and referred to by the time constant T2. Depending  on the type of tissue under study, the T] and T2 relaxation  times will differ, thus giving rise to differences in the image.   Depending on the characteristics of the above four  parameters, the signal intensity of the image will vary, thus  deciding the appearance that any given tissue will finally cast.   2. It does not involve the use of ionising radiation. Hence, it  is safe in that respect.   2. Due to variety of protocol options during scanning, the final  image is highly operator-dependent and this requires expert  technical staff.    This is the contrast study of the veins of the limbs.   3. In case of suspected venous obstruction by tumour  4. To outline venous malformations.    ¢ Tourniquet is applied just above the ankle or elbow t  occlude the superficial venous system   This is the study of the spinal canal  and spinal cord by injection of  contrast medium into the thecal  subarachnoid space.    ¢ Recent lumbar puncture, since  CSF collected outside the thecal  space may be tapped.    Fig. 46.19: Thoracic  myelogram: Contrast in  the thecal sac showing a  total interruption in the flow  of contrast cranially due to  an intradural mass lesion    ¢ Lumbar puncture is most  preferred. The puncture is done  at L2—L3 level.   e¢ Filming of lumbar region is done and with adequate tilt  towards head end, thoracic and cervical regions are also  filmed.    ¢ Cisternal puncture is done when lumbar puncture is a failure.    ¢ Disc lesions are seen as impressions on contrast column  and cut off the nerve roots at a particular level.   ¢ Vertebral and paravertebral lesions result in displacement  of the contrast column and cut off at a particular level.    The role of radiology was limited as only a diagnostic art until  mid 1970s. However, now radiology has taken on an exciting  new aspect and has entered the field of interventional  radiology. Two main types of interventional procedures:  Vascular and nonvascular.    1. Angioplasty: This is performed by the use of intraluminal  balloon catheters and may be performed for almost any  diseased vessel in the body. The more commonly treated  vessels are the coronaries, renal arteries, peripheral limb  vessels, etc.    2. Embolisation: This procedure is performed either  preoperatively to reduce the vascularity of certain  tumours, or as a curative treatment for vascular  malformations, aneurysms, GI bleeding, etc. Temporary  embolisation may be achieved by using gel foam or  autologous clots and permanent embolisation by using  balloons, steel coils, ethanol, etc. Inferior vena cava (IVC)  umbrella placement, 1VC membranotomy are also done.    3. Intravascular ultrasound: The use of ultrasound inside  a blood vessel to visualise the interior of the vessel in  order to detect problems inside the blood vessel.    4. Stent placement: A tiny, expandable coil, called a stent,  is placed inside a blood vessel at the site of a blockage.  The stent is expanded to open up the blockage.    Self-expanding stents are compressed within a  catheter device and released by removing a  constraining sheath or membrane. The final diameter  of the stent is a function of the outward elastic load of  the stent and the inward recoil of the elastic wall.    * Balloon expandable stents are mounted on  angioplasty balloons in a compressed state and then  deployed by balloon inflation. These stents retain the  diameter imposed by angioplasty balloon unless  externally compressed.    5. Foreign body extraction: The use of a catheter inserted  into a blood vessel to retrieve a foreign body in the vessel.    6. Needle biopsy: A small needle is inserted into the  abnormal area in almost any part of the body, guided  by imaging techniques, to obtain a tissue biopsy. This  type of biopsy can provide a diagnosis without surgical  intervention.    7. Blood clot filters: A small filter is inserted into a blood  clot to catch and break up blood clots.    8. Injection of clot-lysing agents: Clot-lysing agents, such  as tissue plasminogen activator (tPA) are injected into  the body to dissolve blood clots, thereby increasing blood  flow to the heart or brain.    9. Catheter insertions: A catheter is inserted into large  veins for giving chemotherapy drugs, nutritional support,  and haemodialysis. A catheter may also be inserted prior  to bone marrow transplantation.    10. Cancer treatment: Administering cancer medications  directly to the tumour site.    1. Hepatobiliary: Percutaneous transhepatic biliary drainage  (PTBD) is widely accepted in cases of biliary obstruction,  along with percutaneous biliary calculus removal. Biliary  stent placement across a malignant lesion 1s widely being  done in inoperable cases as a palliative procedure.    2. Urinary: Percutaneous nephrostomy, percutaneous stenting  and percutaneous nephrolithotomy are being performed.    3. Guided biopsy: Fluoroscopy, ultrasound or CT-guided  biopsy of various lesions are now part of routine technique.    catheter drainage of abscesses, pseudocysts, ultrasound-  guided intrauterine foetal surgeries, etc.    PET scan 1s a medical imaging technique that combines  computed tomography (CT) and nuclear scanning. It is used  to determine the metabolic or biochemical activity in the  brain, heart and other organs by tracking the movement and  concentration of a radioactive tracer injected into the blood  stream.   A camera records the tracer’s signal as it travels through  the body and collects information about the organs. A  computer then converts the signals into 3D images of the   One of the main differences between PET scan and othe  imaging tests like CT or MRI is that the PET scan reveal:  the cellular level metabolic changes occurring in an orgat  and functional changes at cellular level. A PET scan ofte1  detects these changes very early, whereas CT or MRI detec  changes a little later as the disease begins to cause structura  changes in organs or tissues.   Positron emission tomography (PET-CT) constitutes majo  progress in management of cancer patients for the initia  diagnosis, staging and follow-up of various malignancies  PET-CT is also useful in the follow-up of patients following  chemotherapy or surgical resection of tumour, since, mos  of them have a confusing appearance at CT or MR imaging  due to postoperative changes or scar tissue (Figs 46.2(  to 46.25).   Vil > mney N Mere) me) \\(e\-e1e)=)7 from different angles, providing a sort of movie of the  ; colon’s interior without having to insert an endoscope into    ¢ Itis arecently developed technique that uses a CT scanner  and computer virtual reality software to look inside the body Advantages  without having to insert a long tube (conventional  colonoscopy) into the colon or without having to fill the  colon with liquid barium (barium enema).   ¢ More formally known as three-dimensional CT colonography, * Useful when a tumour ts large enough to block passage of  the virtual procedure allows radiologists to obtain 3D images scope (Figs 46.26 and 46.27).   Figs 46.26 and 46.27: Virtual colonoscopy shows opened up view to look for small mass or polyp   ¢ Small polyps are located more reliably by colonoscopy e PET scan, virtual colonoscopy have been upgraded  e Strictly a diagnostic procedure (unlike colonoscopy). e A few photographs have been added   1. Niche and a notch is diagnostic of: 6. Achalasia cardia shows the following findings on  A. Chronic duodenal ulcer barium swallow except:  B. Chronic gastric ulcer A. Shouldering effect cranial and caudal to the lesion    B. Rat tail appearance of lower end of the oesophagus  C. Gross dilatation of the proximal oesophagus  D. Thin streaks of contrast entering the stomach    2. Trifoliate/Clover deformity is diagnostic of:  A. Chronic duodenal ulcer  B. Chronic gastric ulcer  C. Carcinoma stomach  D. Stromal tumour    D. Papilloedema is an absolute contraindication for  myelography  9. Which of the following statements is false?  A. Ultrasound is based on the principle of piezoelectric    5. Which of the following is a contraindication for barium effect. the most common substance used in ultrasonic  study? | | transducers being lead zirconate titanate (PZT)  A. Dysphagia and odynophagia B. MRI uses tonising radiation, hence it 1s unsafe  B. Motility disorders of the GIT C. Patients with pacemakers and critically ill patients  C. Perforation of gastric mucosa cannot be scanned using MRI   D. It helps in early detection of changes in various  pathologies.    ANSWERS  1B 2A 2 4C ae 6A 7D 8A 9B 10 A  11 A 12 D 138 B 14 D 15 D    Radiation oncology is that discipline of human medicine  concerned withthe generation, conservation, and dissemination  of knowledge concerning the causes, prevention, and treatment  of cancer and other diseases involving special expertise in the  therapeutic applications of ionising radiation.    Leopold Freund was the first to report in 1897, the use of  lonising radiation to “cure” a large nevus pigmentosus on the  back of a young girl. With time, ionising radiation became more  precise; high-energy photons, electrons, protons, neutrons, and  carbon ions became available; and treatment planning and  delivery became more accurate and reproducible. Advances in  computer and electronic technology fostered the development  of more sophisticated treatment-planning and delivery  techniques, leading to the development and eventually broad  implementation of three-dimensional conformal radiation  therapy (3DCRT) and intensity-modulated radiation therapy  (IMRT), stereotactic body radiation therapy (SBRT), etc.   The term radiation applies to the emission and propagation  of energy through space and material medium. Radiation  travels with the speed of light in a vacuum, and interacts  with living or nonliving matter resulting in varying degrees  of energy transfer to the biological medium. This process of  deposition of energy within the cells is brought about by  ionisation (removal of an orbital electron) of atoms and    molecules. Ionising radiations are off very high frequency  (3 x 107! hertz) and short wavelength (10'? m) electro-  magnetic waves. lonisation can occur within the nuclear DNA  molecule of a cell (directly acting) or interaction with other  molecules, mainly water (H,O) to produce free radicals  (indirectly), which in tum can damage DNA and result in  cell death or mutagenesis.   By damaging DNA, radiation interferes with cell division  and can result in reproductive death of a cell. This process in  a malignant tumour could mean the loss of its ability for  uncontrolled cell division or proliferation. This process is  unselective; it occurs both in cells of normal tissues and in  those of tumours. Therapeutic usefulness of radiotherapy,  therefore, depends on the differential sensitivity of tissues  (normal vs tumour cell), on careful treatment planning and  dose prescription to minimise normal tissue damage and the  patient’s tolerance to radiation.   1. The acute effects of single doses of radiation can be  decreased with fractionation. The patient’s symptomatic  tolerance improves with fractional radiation.   2. Fractionation exploits the difference in recovery rate  between normal tissues and tumours. Effects on normal  tissues are less because of repair of sublethal damage  between dose fractions and normal cellular repopulation.   3. Radiation-induced redistribution of cells within the cell  cycle tends to sensitise the rapidly proliferating cells, which  is seen more In tumours.   4. Radiosensitivity of cells depend markedly on the phase of  the cell cycle at which they receive the radiation. Cells in  mitosis and G2 phase are the most sensitive and cells in  early Gl, and late S phases are the most resistant.   5. Reduction in the number of hypoxic cells is brought about  through cell kill and reoxygenation. Also blood vessels  compressed by a growing cancer are decompressed as the  cancer shrinks, permitting better oxygenation.   kev adxa7s  BASIS OF FRACTIONAL RADIOTHERAPY  e Repair  e Redistribution  e Reoxygenation  e Repopulation/recovery  e Radiosensitivity    The biologic effects of ionising radiations are greatly influenced  by the presence of normal oxygen concentration within the cells.  The absence or low oxygen content conveys a resistance to  radiation requiring about three times the dose to produce the  same biologic effects. Certain solid tumours and large tumours  are likely to contain 10-15% of hypoxic cells.   For routine practice the “Conventional or Standard” dose  fractionation schedule is followed. This consists of 180—200  cGy fraction per day, and 5 fractions per week over 4—6 weeks  (depending on the total dose). The choice of optimal dose/  time/fractionation schedules for various tumours should be  individualised according to the cell kinetic characteristics and  clinical observations.    Radiocurability refers to the eradication of tumour at the  primary or regional site and reflects the direct effect of  irradiation, which may or may not parallel the patient’s ultimate  outcome.    It is axiomatic in radiation therapy that higher doses of radiation  produce better tumour control, and numerous dose-response  curves (sigmoid in shape) in a variety of tumours have been  published.   For every increment of dose a certain fraction of cells will  be killed. Therefore the total number of surviving cells will be  proportional to the initial number present and the fraction killed  with each dose. Thus, it 1s apparent that various levels of  irradiation yield a different probability of tumour control,  depending on the extent of lesion or number of clonogenic  cells present.   Fig. 47.1: The graph showing tumour probability control  For subclinical disease (10°~* cells) in squamous cell  carcinoma of the upper respiratory tract or for adenocarcinoma  of the breast, doses of 4500-5000 cGy result in control of  disease in over 90% of patients.   For microscopic residual disease or cell aggregates greater  than 10°/10? are required for the pathologist to detect them.  Therefore, these volumes must receive higher doses of  radiation in the range of 6000-6500 cGy in 6—7 weeks for  epithelial tumours.   For clinically palpable tumours (Gross disease), doses  of 6000 cGy (for Tl) to 7500 cGy to 8000 cGy for T4 tumours)  are required (200 cGy/day/5 fractions weekly). This dose range  and probability of tumour control have been documented for  various tumours.    Radiotherapy is the therapeutic use of high-energy ionising  radiation in the treatment/management of malignant disease.   These are either electromagnetic waves, X-rays, gamma  rays or corpuscular (subatomic particles) electrons, protons,  neutrons, alpha particles, or heavy ion nuclei. Ionising radiation  penetrates tissues to different depths according to its type of  energy and physical nature. Radiotherapy treatment planning  is an Important part of the radiation oncologist’s work.    Gamma and beta rays from radioactive isotopes (cobalt 60,  caesium 137, indium 197) and X-rays and electrons from a  high energy X-ray machine (linear accelerator). Protons,  neutrons and heavy ion nuclei from cyclotrons.   X-rays and gamma rays are identical in properties but are  produced by different sources. lonising radiations can be  classified according to their density of ionisations per unit  length of the distance in the absorbing media as low and high  LET (linear energy transfer) radiation.    ¢ High LET radiation has a mass heavier than electrons.  Hence, they cause dense ionisation and are biologically  more effective (damaging) than low LET radiation. They  are also less dependent on repair of sublethal damage, cell  cycle phases and oxygen content of the cells for radio-  sensitivity.   ¢ High LET radiation is available in limited cancer centres  around the world, require expensive equipment to produce  and are undergoing clinical trials.    From sources at a distance (usually 80—100 cm) from the body  surface. This includes ©°Co Teletherapy Units and X-ray  sources, such as linear accelerators (Fig. 47.3).   . Equal absorption in bone and soft tissues  . Improved dose distribution within tissue.    Brachytherapy refers to use of radiation sources in or close tc  the tumour.   Use of sealed (closed containers) radioactive sources fo  radiation treatment from a short distance.    1. Intracavitary (within a cavity), e.g. uterine cavity, vagina  cavity, oesophageal and bronchial lumen.    2. Interstitial when radioactive needles and wires are insertec  into and around a tumour.    3. Surface moulds or plaques as radioactive surface  applicators, e.g. skin cancer, eye cancers.  With this mode of therapy a high dose can be deliverec  locally to the tumour with rapid dose fall off in the  surrounding normal tissue. In the past, brachytherapy wa:  carried out mostly with radium or radon sources. Currently  use of artificially produced radionuclides such as !2’Cs  (caesium 137) , '”Ir (iridium 192), '8Au (gold 198), anc  251 (iodine 125) is rapidly increasing.   3. Remote afterloading, high dose rate (HDR) machines, have  increased accuracy, improved dosimetry, reduced (short)  treatment time, treatment on outpatient basis and have  improved patient compliance.    Brachytherapy is used very often. Combined with external  beam treatment and rarely alone (early stage). The rationale  behind combining the two 1s to treat the primary site and regional  spread (very often subclinical disease) with extemal RT and to  deliver ahigher dose (boost) to the primary (gross disease) with  brachytherapy. Aim is not to exceed the normal tissue tolerance   and at the same time the tumour should receive adequate curative  dose. Brachytherapy can also be used as palliative therapy, e.g.  bronchial and oesophageal obstruction.    From unsealed radioactive sources (i.e. '?!1, 7*P, ®?Sr)  administered enterally, intracavitarily or intravenously, for  diagnostic (nuclear medicine) and therapeutic purposes, e.g.  carcinoma thyroid, bone tumours and thyrotoxicosis.    1. The Roentgen is a unit of exposure. It 1s a measure of  lonisations produced per unit volume of air by X-rays and  gamma rays and cannot be used for photon energies above  3 Mev.  ¢ The SI unit for exposure is Coulomb per kilogram   ¢ Absorbed dose is a measure of the biologically significant  effects produced by ionising radiation. Absorbed dose =  De/dm, De is the mean energy imparted by the ionising  radiation to material of mass dm. The old unit of dose 1s  rad and represents the absorption of 100 ergs of energy  per gram of absorbing material.   ¢ The SI unit of absorbed dose is gray (Gy) and is defined as   Thus the relationship between rad and gray is 1 Gy = 100   Most useful range for clinical use is 6 to 20 Mev.    1. Superficial tumours up to a depth of 5 cm  2. Local boost    tissue morbidity (acute) may be associated.  ¢ Late side-effects of adjacent normal tissues may be dose limiting.    ¢ Patient often needs high doses and long courses of treatment.    1. Characteristic sharp dose fall off beyond the tumour  2. Dose uniformity within the target volume   2. Chest wall irradiation for breast cancer  3. Boost dose to nodes and tumour bed    Like surgery and chemotherapy, radiation therapy (RT) has  definite indications and contraindications for its application.  It can be used alone to cure or, in combination with other  methods, as an adjuvant. Currently 50-60% of all patients with  cancer receive RT during the course of their illness. If properly  used, 50% of these patients could get cured. For the other  half, incurable by any current method, palliation of specific  symptoms and signs can improve quality of life (Table 47.1).  Before treating a patient with radiotherapy, the radiotherapist  must be satisfied that the working diagnosis Is correct,  pretreatment investigations and staging have to be complete.  Then the radiation oncologist must address two questions:    1. Is the treatment intent curative or palliative?  2. What 1s the best approach to achieve this goal?    The first question is vital, for there are important differences  between radical and palliative radiotherapy.   The second question recognises that cancer can be treated  by surgery, radiotherapy and drugs. In many instances,  radiotherapy is the best approach. In view of the increasing  complexity of curative cancer management for many tumours,  with different combinations of surgery, radiotherapy and  chemotherapy for different stages of the disease, require a co-  ordinated Multidisciplinary approach. The correct initial  choice gives the best prospect for cure or good palliation.    A.RT frequently may be the sole agent used with curative  intent for anatomically limited tumours of the retina, optic  nerve, brain (craniopharyngioma, medulloblastoma,   Treatment is intended to control symptoms to improve quality of life.  Minimum doses of RT to achieve maximum control with minimal  side-effects.    Patient prefers few hospital visits, so short courses of treatment used.    ependymoma), spinal cord (low-grade glioma), skin, oral  cavity, pharynx, larynx, oesophagus, uterine cervix, vagina,  prostate and reticuloendothelial system (Hodgkin’s disease,  stages I, II and III A).   B. When no other potentially curative treatment exists.  Some cancers remain localised for all or much of their  natural history. These cancers might also be unresectable  by virtue of their anatomical location or because of local  infiltration into surrounding normal/vital structures, which  would mean that surgery will severely affect physiological  function, e.g. locally advanced head and neck cancer,  cervical cancers stage IIb-III, medulloblastoma (alternative  to surgery for inaccessible and inoperable malignancies).   C. Where alternative therapy is considered more “toxic”.  Carcinoma of the larynx, anal canal, breast can all be  managed by ablative surgery and in each case the anatomy  and physiology of the respective organ is lost. Each of  these cancers can be managed by irradiation with  preservation of anatomy and function.    Preservation of organ and its function (larynx, breast, anal  canal, limbs, cervix, tongue, bladder)    RT is combined with surgery for advanced cancers of the head  and neck, cancers of the lung, uterus, breast, urinary bladder,  testis (seminoma), rectum, soft tissue sarcomas and primary  bone tumours.   ADJUVANT RT (SURG+RT+/-CT)  e Head and neck cancer locally advanced  e Brain tumours  e Breast cancer  e Rectal cancer  ¢« Soft tissue sarcoma  e Bone sarcoma  e Endometrial Ca  e Paediatric solid tumours (Wilms’, rhabdomyosarcoma,  neuroblastoma)    RT is an adjuvant to chemotherapy for some patients witl  lymphomas, lung cancers and cancer in children (rhabdomyo-  sarcoma, Wilms’ tumour, neuroblastoma).    In some clinical situations the combined benefits of surgery  RT and chemotherapy might be exploited. It has been mos  useful in the management of breast cancer, bone sarcoma anc  Wilms’ tumour.    In many situations radiation therapy alone is inadequate fo!  achieving maximum cure levels. This can be because the  number of tumour stem cells is too large, some or all of the  cancer cells are radioresistant, or tolerance of the contiguous  normal tissues is too low. The rationale for combining surgery  and radiation therapy is the differing mechanisms of the two  disciplines. Radiation therapy fails at the centre of the tumour  where the concentrations of the tumour cells is the largest and  the conditions may be hypoxic (less sensitive to RT).    Surgical resection fails because the tumour extends further  than the margins of excision, infesting contiguous tissues with  undetectable microscopic foci. Radiation therapy 1s efficient  in the sterilisation of these tumour cell numbers that are well  vascularised, and the surgical resection 1s efficient in removing  the gross necrotic tumour masses.    Disadvantages  |. Delay in surgical (primary) treatment  2. Delay in wound healing   AIM AND ADVANTAGES  1. Exact disease extent known so as to tailor the treatment  individually.  2. Operative margins are well-defined—gross or microscopic.  3. Less postoperative complications—wound healing intact.  4. Gl anastomoses and ileal conduits can be done in a non-  irradiated field.  5. Potential for unnecessary irradiation in some patients is  reduced   2. Decreased radiosensitivity of the tumour due to rich  oxygen in vascular tumour.   5. Volume of normal tissue to be irradiated is more. Usually  all tissue planes are potentially contaminated by surgery.    Clinical situations may indicate different sequences but  such combinations of surgery and radiation therapy improve  the local tumour control rate for many advanced cancers.  Combined therapy may also improve the cure rate at the same  time reducing the morbidity associated with more aggressive  single modality treatment.    In general, chemotherapy is used 1n an adjuvant way to control  subclinical disease elsewhere in the body or in an additive  way to enhance the local effects of the radiation to achieve  higher rate of local control. Many other agents will act in both  ways.    Agents of choice are those whose toxic effects are in organs  not included in the radiation target volume. An example ts the  combination of the cisplatin compounds with radiation therapy  for head and neck cancers. Here, the toxicity of chemotherapy  is primarily haematogenous and renal. The toxicity of RT 1s  on the oral mucosa.    Chemotherapy could be combined with RT in three main  ways  1. Neoadjuvant: 1—3 courses before definitive RT.    3. Concurrent: During a course of radiotherapy.  4. Combinations of the above.    Certain cancers have a high incidence of developing brait  (CNS) metastases even after their primary disease is controlled  since the CNS because of the blood-brain barrier can act as <  sanctuary site for relapse. Among such patients, it 1s possible  to reduce their local CNS relapse rate and improve surviva  by treating the CNS by prophylactic cranial RT + intratheca!  chemotherapy. The total dose needed 1s low (18-24 Gy) anc  has minimal side effects, e.g. acute lymphoblastic leukaemias  high grade lymphomas.    Objectives of palliative irradiation include:  e Relief of pain, usually from metastases to bone    e Relief of obstruction, such as tumours involving ureter,  oesophagus, bronchus, lymphatic and blood vessels.    e Promotion of healing of surface wounds by local tumour  control.    The incidence of systemic symptoms from radiotherapy is  variable. In broad terms, the larger the treatment yield, the  fraction size and the total given dose, the greater will be the  chance of the patient developing problems. The dose of  radiation that can be delivered is limited by acute reactions  and by late irreversible organ/tissue damage. Each organ has  a known tolerance which should not be exceeded.    However, in order to achieve a given level of tumour control  probability certain amount of normal tissue sequelae are  unavoidable.    During a course of radiotherapy mild to moderate grade  acute reactions occur frequently and can be usually conser-  vatively managed and might require a short break in the  treatment whereas chronic reactions are usually the dose  limiting complications. Severe grade reactions should be  avoided using proper time dose fractionation regimens,  accurate treatment planning and execution.    Some of the important acute reactions following RT, their  threshold doses and management are shown tn Table 47.2.    Skin Erythema 10-12  Skin Dry desquamation 40-50  Shin Moist desquamation 45-55  Mucous Mucositis 30-40  Hair Alopecia 30-40  Lung Pneumonitis with cough, 20  dyspnoea  Gl tract Nausea, vomiting Any dose  Gl tract Diarrhoea 30-40  Bladder Urinary frequency and dysuria 40-50  Bone Suppression especially of 10—20  marrow. white blood cells and platelets   Current research in radiation oncology 1s of such significance  that it promises anew standard of care for patients with cancer.  Recent advances in radiation therapy include efforts to improve  the effectiveness of radiation and to improve the quality of  life of treated patients. Innovations in radiobiology, imaging  technology, computer technology and treatment machine  technology has resulted in marked changes in the way  radiotherapy is practised at present. The newer methods aim  at increasing the accuracy of treatment, planning and dose  delivery using the highly sophisticated features offered by the  modem day equipment (Key Box 47.8).    1. 3-D conformal radiotherapy (three-dimensional treatment  planning and conformal dose delivery). In 3-D CRT, patient  immobilisation, image-guided treatment planning and  computer-controlled treatment delivery can create a  radiation dose distribution that conforms to the shape of  the tumour volume (Key Box 47.9). The tumour volume  containing the cancer and areas of potential cancer is much  more accurately outlined as are normal tissues to be avoided.  The target radiation dose can be increased when necessary  without increased toxicity to normal tissue. This is  accomplished using volumetric CT data 1n a 3-D treatment  planning computer.    2. IMRT: IMRT 1s an advanced form of 3-DCRT. It is one of  the technologically most advanced treatment methods  available in external beam radiation therapy. IMRT allows    Keep the affected area dry. Gentian violet may be helpful in drying  the affected area    Topical benzydamine hydrochloride mouth rinse or spray. Stop  smoking. Mucaine for oesophageal mucositis. Always exclude  candidiasis    Advise low fibre diet when starting treatment. Antidiarrhoeal  preparations may be required for symptomatic relief    Exclude urinary tract infection and consider antimuscarinic drugs  Check full blood count regularly   ADVANCES IN RADIATION THERAPY s  . 38D conformal radiotherapy (3-dimensional)  . IMRT (intensity modulated radiation therapy)  . SRS/SRT (stereotactic radiosurgery and radiotherapy)  . IGRT (image guided radiation therapy)  . SBRT (stereotactic body radiation therapy)  . Proton therapy  . Intravascular brachytherapy  . CyberKnife    very precise external beam radiotherapy treatments. Rather  than having a single large radiation beam pass through the  body, with IMRT the radiation 1s effectively broken up into  thousands of tiny pencil-thin radiation beams of varying   e Metastatic brain tumours, primary brain tumours (glioblas-  tomas, gliomas, etc)   e Head and neck cancer (larynx, tongue, sinus, base of skull,  mouth, etc)    intensity with millimetre accuracy. These beams enter the  body from many angles and intersect on the cancer. This  results in a high dosage to the tumour and a lower dose to  the surrounding healthy tissues.    Intensity modulation radiotherapy can allow us to treat  tumours to a higher dose, retreat cancers that have  previously been irradiated, and safely treat tumours that  are located very close to delicate organs like the eye, spinal  cord, or rectum. Simply put, this can translate into a higher  cancer control rate and a lower rate of side effects.    High-dose highly focused radiation therapy for small  (SRS = Single fraction, SRT = Multiple fractions) target  lesions (< 2--4 cm) can be accomplished by either gamma  knife (multiple, fixed, precisely aimed cobalt teletherapy  beams) or stereotactic radiation therapy (multiple rotational  arcs of photon beams from a linear accelerator). Both  techniques are similar in their use of standard energy photon  beams for treatment and rely on meticulous patient  immobilisation to deliver treatment to a precisely localised  target within a co-ordinate mapping system. These  techniques have been widely used and well described for  the treatment of intracranial neoplasms (meningiomas,  acoustic neuromas and metastatic tumours) and for the  ablation of arteriovenous malformations and ocular  melanomas. [Equipment used: 1. Gamma Knife (Multiple  ©°Co sources) or 2. X- knife (modified linear accelerator)]    Image guided radiation therapy (IGRT), is one of the most  cutting-edge innovations in cancer technology available.  Tumors can move, because of breathing and other movement  in the body. Real-time imaging of the treatment target and  normal organs during each treatment allows for  minimisation of reduction of irradiated volumes, as well  decreases the chance of missing a target, helping to limit  radiation exposure to healthy tissue and reduce common  radiation side effects.   In IGRT, the linear accelerators are equipped with imaging  technology that take pictures of the tumour immediately  before or even during the time radiation is delivered.  There are numerous types of imaging modalities that can  be incorporated into an IGRT system. Examples include:  ultrasound images, low-energy (kV) CT scan images, high-  energy (MV) CT scan images, etc.    Stereotactic body radiation therapy (SBRT) is a technique  that utilises precisely targeted radiation to a tumour while  minimising radiation to adjacent normal tissue (Fig. 47.4).  This targeting allows treatment of small or moderate sized    tumours in either a single or limited number of dose  fractions. SBRT has beenused in hepatocellular carcinoma,  lung cancer, prostate cancer, pancreatic cancer. As with any  form of radiation therapy, careful attention to matters of  patient selection and technical quality assurance is essential  for the effective and safe implementation of SBRT.   Proton beam: Proton radiation reduces the dose to normal  tissues by allowing for more precise dose delivery because  of the unique physical properties of heavy particles. Protons  penetrate tissue to a variable depth, depending upon their  energy, and then deposit that energy 1n the tissue in a sharp  peak, known as a Bragg peak. This rapid dose fall off at a  depth that can be controlled by the initial energy of the  protons allows for decreased radiation to adjoining normal  tissue by a factor of 2 to 3.   Protons are used for uveal melanoma (ocular tumours), skull  base and paraspinal tumors (chondrosarcoma and  chordoma), and unresectable sarcomas, pediatric neoplasms  (such as medulloblastoma) and prostate cancer.    angioplasty or restenosis occurs in 30 to 40 percent of  patients and results from neointimal proliferation and  constrictive remodelling of the angio-injured artery.  Coronary stenting has led to a 30 to 50 percent decrease in  the rate of restenosis primarily by preventing the constrictive  remodeling of the artery but at the cost of an increase in  neointimal proliferation. The system used for intra-arterial  beta-radiation therapy has Yttrium-90 beta ray emitting  source (half-life, 64 hours; maximal energy, 2.284 Mev), a  centering balloon and an automated delivery device. An  18-Gy dose not only prevents the renarrowing of the lumen  typically observed after successful balloon angioplasty but  actually induces luminal enlargement.    alternative to surgery for the precise treatment and effective  removal of cancerous tumours (Fig. 47.5) from the body.    The system has a miniaturised linear accelerator mounted  on a robotic arm with 6 different points of axis where it  can bend, turn, tilt or swivel with sub millimetre accuracy.    Destroys tumours with highly precise beams of radiation,  tumours virtually anywhere in the body, quickly, painlessly  and without downtime or hospital stay in one to five sessions  (hypofractionation).   Thus offers new hope to patients who have inoperable or  surgically complex tumours and for those who may be  looking for an alternative to more invasive surgery.   1. The following statements about radiation are correct  except:  A. Fractional radiation reduces the acute effects of single  doses of radiation on normal tissues  B. Cells in mitosis and G2 phase are most sensitive to  radiation  C. Oxygen concentration within cells ts directly  proportional to the radiation dose required to kill them  D. Standard dose of radiation 1s 180-200 cGy per day  and 5 per week over 4—6 cycles  2. Adose-response curve for radiation is of which shape?  A. Sigmoidal  B. Linear-rising  C. Bell-shaped  D. Irregular  3. The following are the principles of brachytherapy  except:  A. Itis a type of method of delivery of ionising radiation  from sources inside or close to the tumour  B. External RT 1s used to deliver a higher dose (boost) to  treat the primary site while brachytherapy treats the  regional spread  C. Itishighly localised, specific to a given tumour volume  D. It has a high dose rate, given as continuous RT in a  single course  4. 1 cGy is:  A. l rad B. 10 rad  C. 100 rad D. 1000 rad  5. The most commonly used chemotherapeutic agent with   A. Agent of choice will have minimum toxic effects on  the organs included in the radiation target volume   D. Intrathecal CT is a prophylactic measure to prevent  CNS metastasis in cancers like ALL or high grade  lymphomas   C. Deeply situated tumours at a depth of > 5 cm are treated  with this   A. Exact disease extent is known and treatment can be  individualised    Surgery has been practised for ages. However, the advent of  modern techniques of anaesthesia has allowed surgery to  develop by leaps and bounds. If there is a well-informed,  vigilant and safe anaesthesiologist taking care of the patient,  the surgeon 1s able to concentrate on the surgical procedure  unhindered. The anaesthetist of yore who was treated often as  a technician assistant of the surgeon has metamorphosed into  the present day anaesthesiologist, who is also a perioperative  physician. An anaesthesiologist’s understanding of anatomy,  physiology and pharmacology, and his expertise of  management of critical illness and pain have taken him into  new fields such as Intensive Care, Pain and Palliative Care.  Even then, the main realm of the anaesthesiologist remains  the operation theatre.   A good understanding of the physiology and pharmacology,  complemented by continuous and vigilant monitoring has made  the practice of anaesthesia safer. Safe practice of anaesthesia  comprises the following steps: a good rapport with the patient,  a thorough preoperative preparation and premedication,  monitoring and perioperative care.    Every patient 1s anxious when he comes into the operating  room. The causes of anxiety can be varied: fear of the disease  condition, surgery, anaesthetic and the anticipated pain. A good  rapport developed between the patient and the anaesthesiologist  can build up his confidence and help allay many of these fears.    Anaesthesia is associated with changes in the internal  homeostasis. Normally these are well tolerated by the different  systems. However, if the patient has a pre-existing  derangement, his capacity to withstand changes in his internal  milieu may be limited. It is thus very important to know the  preoperative condition of the patient.    A detailed history of the patient with symptoms pertaining to  the various systems must be elicited (Table 48.1).    A detailed physical examination is done and the relevant  history specially borne in mind. General physical examination  includes:    Vital signs: Blood pressure, heart rate, respiratory rate,  temperature (and oxygen saturation, in relevant cases).    PICCLE: Pallor, icterus, cyanosis, clubbing, lymph-  adenopathy, oedema and raised jugular venous pulse.    Spine: To rule out infection over the skin covering the spine,  tenderness, stiffness or fractures of spine, to check spaces.    A detailed examination of the various systems is then carried  out and relevant findings noted.    Dyspnoea, angina, syncope, palpitations, pedal oedema, previously diagnosed to have a cardiac    Cough, fever, breathlessness, chest pain, recent onset upper respiratory tract infection    Consciousness level, convulsions, orientation, ability to walk, speech, movement of all four limbs,    bowel and bladder habits, any paraesthesia or altered sensation in the limbs.    Whenever a person becomes unconscious, the tongue and  epiglottis fall back on to the pharynx and obstruct the airway.  Since the anaesthesiologist makes the patient unconscious  during a general anaesthetic, it is his duty to ensure that the  patient’s airway patency is maintained. Hence, assessment of  the airway becomes important before a patient is made  unconscious.    ¢ When a person opens his mouth, one should be able to  insinuate one finger in the temporomandibular joint.   ¢ There should be at least three finger breadths’ distance  between the chin and the thyroid cartilage (thyromental  distance) of the patient.    The patient is made to sit upright, open his mouth wide and  protrude his tongue. The structures visualised are classified as  given in Fig. 48.1. These classes roughly correlate with the  following grades of laryngoscopic views (Fig. 48.2). Difficult  airway may be anticipated in Mallampati Class III and IV and  the anaesthesiologist must be prepared to secure airway in such  patients.    Previous surgeries/ anaesthetics, any problems during the previous experience. H/o hospitalisation in the past,  H/o tuberculosis, asthma, diabetes, hypertension, epilepsy, H/o current medications, steroid intake in the last 6   Class III Class IV  Fig. 48.1: Mallampati classification of the airway    Fig. 48.2: Modified Cormack and Lehane grading of laryngo-  SCOpiC view    Restriction of neck movements, especially extension makes  endotracheal intubation more difficult.    There is no place for routine investigations to be ordered before  any surgery. Investigations that are required to assess the  baseline status of the patient and have a bearing on the  perioperative course must be done. Thus, they should be  tailored to the individual patient. The common investigations  ordered are as follows:    Electrocardiogram, blood sugar estimation, blood urea,  serum creatinine and electrolytes: Male patients > 40 years,  smokers and female patients above 50 years.    Chest X-ray is requested for if a major abdominal or thoracic  Surgery is planned, postoperative ventilation is expected or  cardiorespiratory disease 1s suspected.    Stress test, echocardiogram, pulmonary function test or a  blood gas analysis are obtained as necessary.    Blood grouping and cross-matching are requested if the  surgery may be associated with major blood loss.    The patient’s preoperative physical status can be classified into  five different categories. The American Society of  Anaesthesiologists classification of Physical Status isas follows:    ASA V : Moribund, not expected to survive without the  operation    A suffix E is added if the surgery is of emergent nature. A  sixth category called ASA VI 1s given to the patient who 1s  declared brain dead whose organs are being removed for donor  purposes.    The risks associated with anaesthesia in a patient with  concurrent diseases must be assessed. Algorithms by different  organisations are available to help decision-making in individual  patients: For example, American Heart Association  recommendations for evaluation of ischaemic heart disease in  a patient posted for noncardiac surgery. The goal of preoperative  assessment and preparation is thus to ensure that the patient 1s  optimised and 1s in the best possible condition prior to surgery  and anaesthesia.    The patient 1s explained about the planned anaesthetic, the  problems anticipated and the risks involved in his owr  language and a written and informed consent is obtained.    The patient is permitted to take solids and milk up to 6-8 hours  and clear fluids up to 3 hours prior to surgery. This may be  relaxed for neonates and infants where a four-hour fast for  breast milk is sufficient. Formula feeds and milk from any  other source are treated as solids. If the last food intake  contained a considerable amount of fat, gastric emptying time  may be delayed (Key Box 48.1).   FASTING INSTRUCTIONS  Solids and milk: 6 hours  Clear fluids: 3 hours  Formula feeds: 6 hours  Breast milk: 4 hours  Clear fluids: 2 hours    Premedication 1s the term given to the administration of certain  medications prior to anaesthesia. The drugs used for  premedication and their objectives are as follows:    {. To allay anxiety: Certain degree of anxiety 1s felt by most  patients before surgery. A good rapport developed between  the patient and the anaesthesiologist helps relieve this  anxiety. Any of the following medications may be used to  reduce anxiety.   Children: To enable easy separation from parents.  Midazolam 0.5 mg/kg (maximum 10 mg) mixed with 5 ml  of paracetamol syrup 1s given orally 15—20 minutes prior  to the procedure.    2. To relieve pain: If the patient has any painful condition  that could get aggravated on movement, a narcotic 1s often  added to reduce the pain during shifting from the ward, e.g.  fractures. Morphine (0.1 mg/kg), pethidine (0.5 mg/kg) or  tramadol (0.5—1 mg/kg) given IM are often used.    3. To dry secretions: An anticholinergic such as  glycopyrrolate (0.2 mg) is added if a fibreoptic intubation  1s planned so that oral secretions do not hinder vision. Local  anaesthetic agents produce better local anaesthesia of the  upper airway when the mucosa Is dry. It may be given  intravenously just prior to surgery in ENT surgeries and  oral surgeries.   4. To help anaesthesia induction: Premedication with a  narcotic provides analgesia and helps induce anaesthesia  more smoothly.    5. To blunt baroreceptor reflexes: A small dose of 8 blockers  or clonidine may be given in certain patients to blunt  baroreceptor reflexes during intubation.    6. To reduce gastric volume and acidity: Some patients are  at risk of regurgitation of gastric contents and aspiration.  They may be premedicated with a prokinetic such as  metoclopramide (10 mg) and a H, blocker such as  ranitidine (150 mg orally). Pantoprazole 40 mg may be  given instead of ranitidine. The consequences of aspiration  of gastric contents depends on its quantity and acidity.  Particulate matter, if aspirated can cause mechanical  blockage of the airways. Metoclopramide reduces residual  gastric content by hastening gastric emptying and ranitidine  reduces the acidity. Sometimes, a nonparticulate antacid  such as sodium citrate may be given to neutralise existing  gastric acid. Multiple factors during anaesthesia and surgery  increase the chances of nausea and vomiting. Since  anaesthesia involves blunting of airway reflexes, patients  are at risk of aspiration. This is why patients are kept fasting  before elective surgery. This may not be possible for patients  who are posted for emergency surgery. It 1s assumed that  these patients are *“full-stomach’ and appropriate precautions  are taken.    Anaesthesia can be classified into two main categories: General  anaesthesia and regional anaesthesia.    The patient is unconscious and there 1s a generalised and  reversible depression of the central nervous system.    This involves injection of local anaesthetic agents in close  proximity to the nerves or nerve bundles supplying the site of  surgery. Regional anaesthesia can be central neuraxial block  or peripheral nerve blocks.    When regional anaesthesia is induced by injecting the local  anaesthetic agents around the spinal cord, it is called central  neuraxial block.    When regional anaesthesia is induced by injecting the local  anaesthetic agents around nerve plexuses or individual nerves,  it is called peripheral nerve block.   Regional anaesthesia has a small failure rate and certair  Surgeries may outlast the duration of block provided by the  regional block, in which case general anaesthesia may need tc  be given. Thus even though the primary anaesthetic  contemplated 1s regional anaesthesia, patients must also be fit  for general anaesthesia.   e Hypnosis (sedation)  e Amnesia  e Analgesia and  ¢ Muscle relaxation.   General anaesthetic agents are of two main types:  Inhalational anaesthetic agents or intravenous anaesthetic  agents.    ¢ Volatile anaesthetics: The volatile anaesthetic agents need  a vaporiser to calibrate and deliver the vapour accurately in  measured doses, e.g. halothane    I. Agents of mainly historical interest  1. Ethyl] chloride  2. Chloroform  3. Trichloroethylene  4. Cyclopropane  5. Methoxyflurane  6. Enflurane    II. Agents in occasional use  1. Drethy) «ther  IIt. Agents in clinical use  1. Halothane  2. Isoflurane  3. Sevoflurane  4. Desflurane  5. Nitrous oxide   A comparison of clinical effects of common inhalational  anaesthetic agents in use is given in Table 48.2. Onlyimportant  effects have been mentioned for the benefit of students.    This was a very popular anaesthetic agent till recently but is  gradually being replaced by isoflurane. Reasons for dwindling  popularity are:    Type 1: It is mild, self-limiting and more common. It Is  associated with mild increases in liver enzymes but not  jaundice. It is caused by reductive metabolism of halothane.    Type II: On extremely rare occasions (widely quoted incidence  1 :35,000), the administration of halothane may be associated  with the production of hepatitis. This entity, known as halothane  hepatitis is largely a diagnosis of exclusion. It 1s fulminant with  a high mortality rate (up to 50%). It is associated with fever,  jaundice and grossly elevated liver enzymes. The toxic  metabolite, trifluoroacetic acid reacts with liver proteins and  triggers immune-mediated reaction tn genetically susceptible  individuals. Risk factors for development of halothane hepatitis  are female gender, obesity, prior history of postanaesthetic  jaundice, genetic susceptibility, repeated administration and  enzyme-inducing drugs such as phenobarbitone.    and isoflurane (0.2%). Thus, hepatic dysfunction, although  reported with sevoflurane and isoflurane, is extremely rare.    ¢ Isoflurane causes less cardiac depression. Hence, it is usec  in patients with cardiac disease.    e It causes less cerebral vasodilatation than halothane.  Hence, it is preferred during neurosurgery.    ¢ Isoflurane is now routinely used in all cases in many centres  because it produces less arrhythmias and myocardial  depression, is associated with only a remote risk of hepatitis  and 1s not expensive.   e It has a pungent smell and hence cannot be used for  inhalation induction.   e Itis anewer general anaesthetic agent and has a sweet smell.  ¢ Useful for inhalation induction, especially in children.  ¢ Induction and recovery are faster than with halothane.    ¢ It is also a useful agent for induction of anaesthesia in  patients with difficult airways.    ¢ It 1s another new volatile anaesthetic agent.  ¢ It causes vasodilatation and increase in heart rate.  ¢ Induction and recovery are very fast.    ¢ However, it is irritant to the respiratory tract and hence  is not suitable for inhalational induction.    e It also requires a specially constructed heated vaporiser  because of its high volatility.    Parameter Halothane Isoflurane Sevoflurane  Smell Pleasant Pungent Pleasant  Inhalation induction Suitable Not suitable Suitable   Fig. 48.3: Inhalation induction of anaesthesia in a child using  sevoflurane. Note how the child is reassured by the comforting  arm of the anaesthesiologist    e It is an anaesthetic gas which is compressed and supplied  as a liquid in cylinders.   e It provides analgesia but is insufficient to produce an  adequate depth of anaesthesia when used alone.   e¢ It enhances induction of anaesthesia with the volatile  anaesthetics and reduces their requirement.   e It does not produce significant depression of the  cardiovascular system.    ¢ It is used along with volatile anaesthetic agents as part of  balanced anaesthesia.   ¢ A combination of 50% nitrous oxide and 50% oxygen is  available as Entonox. It is used to provide labour analgesia.   e Itmay also be used to provide analgesia for small procedures  in dentistry.    ¢ Itcan diffuse into closed gas spaces such as pneumothorax,  obstructed intestines, sinuses and middle ear, and can cause  barotrauma. The volume of a cavity can increase 3 to 4  times within |—2 hours. Hence, nitrous oxide is best avoided  in patients in whom such expansion may be anticipated.   e Its use is associated with increased incidence of  postoperative nausea and vomiting.   ¢ In prolonged administrations, it can affect vitamin Bj  synthesis causing megaloblastic anaemia.   ¢ Teratogenicity: This has been observed in pregnant rats  exposed to nitrous oxide for prolonged periods but not  proved in human beings. Nitrous oxide is best avoided in  early pregnancy.   Intravenously administered anaesthetic agents are more popula  for induction of anaesthesia because it is more rapid and smoot  than that associated with inhalational agents. They can also b  used for maintenance of anaesthesia, sedation during regiona  anaesthesia, sedation in the ICU and treatment of statu  epilepticus.    They can be classified into rapidly-acting (acting withi  one arm-brain circulation time) and slow-acting (those tha  take longer than one arm-brain circulation time) (Key Bo?  48.2).   INTRAVENOUS ANAESTHETICS  e Rapidly-acting: Thiopentone  Propofol  Etomidate  ¢ Slower-acting: Ketamine  High dose opioids  Benzodiazepines    It is an ultra-short acting barbiturate, available as a yellowish  powder. It is used as a 2.5% solution (25 mg/ml). It is used in  a dose of 4-5 mg/kg intravenously.   ¢ Generalised depression of the CNS is observed within 15  to 20 sof IV injection of thiopentone. Loss of eyelash reflex  is used as an end-point.    ¢ It is a potent anticonvulsant.  * It is not an analgesic. To the contrary, it increases pain   ¢ It produces myocardial depression, peripheral vaso-  dilatation and hypotension especially when large doses are  administered rapidly.    ¢ Profound hypotension may occur, especially in a patient  with hypovolaemia or cardiac disease. It may induce  tachycardia.    ¢ It crosses placenta rapidly, although foetal blood  concentration 1s far less than that observed in the mother.    ¢ Irritant to veins and can cause thrombophlebitis. If it  extravasates, can cause tissue necrosis.    ¢ Intra-arterial injection: Usually inadvertent, can cause severe  arterial spasm and pain. This may be treated with  vasodilators and heparin.    This drug became commercially available in 1986. It 1s  comparable to thiopentone but is five times more expensive.  It is highly lipid soluble and is formulated in a white, aqueous  emulsion containing soy bean oil and egg phosphatide. It is  used in a dose of 2 to 2.5 mg/kg intravenously. The dose should  be reduced in the elderly and in haemodynamically unstable  patients.    e Propofol depresses the central nervous system within 20 to  40 s of injection. Loss of verbal contact is used as an end-  point.    e Recovery is rapid and there is a minimal "hang-over" effect  even in the immediate post-anaesthetic period.    ¢ The arterial pressure decreases more than with thiopentone.  This is due to peripheral vasodilatation. The degree of hypo-  tension can be reduced by slowing the rate of administration.  ¢ Heart rate increases slightly.    RS  e After induction, apnoea occurs commonly and for a longe!  duration than with thiopentone.  ¢ It causes ventilatory depression, particularly with opioids.  ¢ It has no effect on bronchial muscle tone.  GIT, uterus and placenta: Propofol has no effect on Gl  motility but causes mild transient decrease in hepatorena]  function.   ¢ As an infusion, can be used to provide total intravenous  anaesthesia   ¢ Ascontinuous infusion to sedate patients in the ICU, since  its half-life is very short.    ¢ Etomidate is a rapidly acting intravenous anaesthetic agent  with a short duration of action of 3—5 minutes.   ¢ It is a very cardiostable agent and is used to induce  anaesthesia in cardiac patients and in haemodynamically  unstable patients.   e However, continuous infusions are not advisable as it is  known to depress synthesis of cortisol by the adrenal gland  and impair response to ACTH.   1. Suppression of synthesis of cortisol with infusions.  2. Excitatory phenomena: Involuntary movements, cough  3. Pain on injection and venous thrombosis    Ketamine differs from other intravenous anaesthetic agents in  many respects and produces dissociative anaesthesia, rather  than generalised depression of the central nervous system. It  is used in a dose of |—2 mg/kg IV or 4—S mg/kg IM.   ¢ Itinduces anaesthesia within 30-60 s of intravenous injection  and lasts for |0—20 mins. It is effective within 3-4 mins of  intramuscular injection and lasts for 15—25 mins.    e It produces anaesthesia by dissociating the cerebral cortex  from the limbic system.    RS  ¢ Respiration is usually well-maintained with ketamine  although transient apnoea may occur occasionally.    GIT  Increased salivation can occur and can be prevented by using  anticholinergic agents.    It readily crosses the placental barrier and hence should be  given in lower doses in pregnant patients.    e Vivid and unpleasant hallucinations are known with keta-  mine and can be prevented by prior injections of  benzodiazepines.   When a nerve impulse arrives at the neuromuscular junction  through the motor neuron, acetylcholine (ACh) molecules are  liberated from the nerve endings into the junctional cleft.  Acetylcholine molecules act as neurotransmitters by interacting  with the nicotinic ACh receptors on the postjunctional muscle  membrane at the motor end plates. This induces the opening of  ionic channel of the receptor to allow ionic (sodium, calcium)  flux into the muscle. The sudden influx of sodium results in  depolarisation and muscle contraction.    The acetylcholine receptor is flower-shaped with five petal-  like structures. These five subunits are named (2), B(1), €(1)  and o(1). Each alpha subunit must be occupied by an  acetylcholine molecule to open the channel. This acetylcholine    receptor at the neuromuscular junction is nicotinic in nature  and thus different from those in the rest of the body.    These are drugs that interfere with the combination of acetyl-  choline molecules with their receptors. These block neuro-  muscular transmission and cause relaxation of the muscle  resulting in muscle paralysis.   Neuromuscular blockers are of two types: Depolarising and  nondepolarising muscle relaxants.    It is the only depolarising muscle relaxant in clinical use. It  has a molecular structure similar to acetyl choline. It combines  with the alpha subunit of the ACh receptor and produces muscle  contraction. However, unlike acetyl choline, it has a prolonged  action. Continued depolarisation of a muscle results in  accommodation blockade and the muscle relaxes.    Succinyl choline is the only muscle relaxant which has the  shortest time to onset of action (60 seconds) and shortest  duration of action (3—5 minutes). It 1s used:    ¢ IJtis useful in patients with difficult airway because it gives  very good relaxation to facilitate intubation but if intubation  fails, the patient is likely to resume spontaneous breathing  early and hypoxic brain injury may be avoided.    ¢ Muscle pains: The initial depolarisation that occurs due  to succinyl choline causes uncoordinated contraction  (fasciculations) of different groups of muscle fibres. This  can cause severe muscle pain postoperatively.    ¢ Bradycardia, especially ifasecond dose of succinyl choline  is given. It is easily avoided by pretreatment with atropine.    ¢ Hyperkalaemia in patients with renal failure, burns,  massive crush injury, etc.   * Prolonged action in patients deficient in pseudo:  cholinesterase (plasma cholinesterase). This occurs as <  genetic problem in a small number of patients but may be  an acquired problem as in severe liver disease.    ¢ Malignant hyperthermia is a disorder that is unique anc  life-threatening disorder precipitated by exposure tc  inhalation anaesthetics and succinyl choline ir  susceptible patients. In malignant hyperthermia, the  metabolic rate of muscle cells is increased tremendously  due to a defective ryanodine receptor which is necessary  for reuptake of calcium after a depolarisation.    ¢ Those with a family history of anaesthetic mishaps,  neuromuscular diseases such as muscular dystrophy may  be susceptible to this disorder and succiny]! choline is best  avoided in these patients. It is not always possible to identify  latent muscular dystrophies in infants and children.  Administration of succinyl choline in these patients can be  disastrous. Hence, the use of succinyl choline should be  avoided in children less than 2 years unless an indication  such as a full stomach exists. Even then, rocuronium may  be considered as an alternative.    Pancuronium, vecuronium, rocuronium, atracurium and  cisatracurium are drugs belonging to this group in clinical use.  The nondepolarising muscle relaxants combine with the ACh  receptors but do not have any intrinsic effect on the muscle. They  cause muscle paralysis by preventing the acetyl choline  molecules that are released from the nerve terminal from  combining with the acetyl choline receptors on the postsynaptic  membrane and producing their action (competitive inhibition).  Even if one alpha subunit is combined with a molecule of  nondepolarising muscle relaxant, the muscle cannot contract in  response to a nerve impulse and gets paralysed. The muscle  regains its power when the muscle relaxant gets metabolised.    The nondepolarising muscle relaxants can be classified  according to their duration of action as follows:  ¢ Short-acting (10-20 min): Mivacurium    1. To facilitate endotracheal intubation  2. To maintain paralysis during anaesthesia and in the ICU.    At the end of anaesthesia, the muscle relaxation produced by  the nondepolarising muscle relaxant is usually reversed. This  is to ensure good recovery of muscle power to maintain airway  and respiration.   Acetylcholine molecules are broken down by choline-  sterases. Anticholinesterases such as neostigmine block the  action of cholinesterase, thus allowingacetyl choline molecules  to accumulate in the neuromuscular junction. The block  produced by the nondepolarising muscle relaxants is  competitive. When acetyl choline molecules increase in  number due to the action of the anticholinesterase, and  nondepolarising muscle relaxant molecules decrease in number  due to metabolism, muscle power returns. Neostigmine is the  only anticholinesterase in clinical use. Its dose 1s 0.05 mg/kg  body weight. Neostigmine increases the amount of acetyl  choline not only at the neuromuscular junction but also in the  entire body. It cancausethe muscarinic effects of acetyl choline  such as bradycardia, bronchoconstriction, etc. Hence,  neostigmine 1s always combined with atropine (0.025 mg/kg)  or glycopyrrolate (0.01 mg/kg) to counter the muscarinic  effects.    ¢ Ability to lift head against gravity (sustained head-lift) for  at least five seconds.    The amount of neuromuscular blockade can be checked using  a peripheral nerve stimulator. The ulnar nerve is stimulated  and the response of the adductor pollicis muscle 1s checked. If  good muscle contractions are seen in response to the nerve  stimulation, the muscle power is said to have returned.  Similarly, posterior tibial nerve and facial nerve may also be  used to monitor neuromuscular junction.    The patient is allowed to breathe spontaneously and his  trachea extubated only when there 1s clinical evidence of com-  plete recovery from neuromuscular blockade. If there is inade-  quate recovery of muscle power, the patient may need to be  ventilated artificially till muscle power is normal.    General anaesthesia involves induction of unconsciousness  during which the patient’s ability to maintain his airway and  breathing are impaired. It thus becomes necessary that the  anaesthesiologist maintains and protects the patient’s airway.  In addition, not only anaesthesia but also oxygenation and  ventilation are maintained well only if the airway is patent  and secured.    Management of airway is one of the basic skills acquirec  by an anaesthesiologist. This 1s relatively easy in mos  individuals. Difficult airway 1s the term given when there 1:  difficulty with mask ventilation, endotracheal intubation, o  both.    When a person becomes unconscious, the tongue and the  epiglottis fall back and obstruct the airway. Since they are  attached to the mandible, lifting up of the mandible also lifts  these two structures and opens airway. This is usually done by  head-tilt and chin-lift method as during cardiopulmonary  resuscitation. Jaw thrust is more popular with anaesthesio-  logists. The patient may be ventilated using a ‘bag’ (either a  self-inflating bag or anaesthetic circuit) and a facemask  (Fig. 48.4). The mask is triangular in shape, the narrow portion  of which is placed at the bridge of the nose and the base placed  in the depression between the lower lip and chin. The mask is  held in place by an E-C technique (Fig. 48.5).    Endotracheal intubation is the most definitive way of  maintaining airway in patients who require muscle paralysis  and require intermittent positive pressure ventilation. It  involves introduction of a tube into the trachea for maintaining  the patency and protecting the airway as well as to ensure  adequate oxygenation and ventilation. Whenever general  anaesthesia is induced and needs to be maintained for long  periods, endotracheal intubation 1s done.   Fig. 48.4: Maintenance of airway and ventilation using bag  (anaesthetic circuit) and mask    Fig. 48.5: The E-C technique of holding a face mask to get airway  seal. The thumb and index fingers form a ‘C’ at the top of the  mask holding it firmly down on the face. The middle, and ring  fingers lift up the mandible as well as provide head tilt. The little  finger aids in providing jaw thrust. Note that the finger pressure  should be given on the bony part and not on the soft tissues under  the chin   ¢ To ensure delivery of adequate tidal volumes to the lungs.   ¢ When the upper airway integrity is lost as in extensive  maxillofacial injury with bilateral fractures of mandible and  maxillae.    * The handle contains batteries. The blade has a flange to  push the tongue towards the left side. This ensures more  room for visualisation of the glottis. A bulb nearer the tip  of the blade lights up when the handle and blade are at right  angles to each other and electrical contact is made.    ¢ There are several types of laryngoscopes to aid in different  situations.    — Macintosh type blade (Fig. 48.6) is curved and is  popular for use in adults.   Fig. 48.6: Macintosh laryngoscope. Note the curved blade.  Sizes 1, 2 and 3 are displayed   Fig. 48.7: McCoy laryngoscope. Note the retractable tip of the  laryngoscope blade    — The Miller blade is straight and is used in children  and in adults with difficult airway.    — Bullard laryngoscope for use in patients with cervical  spine immobility.   — Videolaryngoscopes have a small camera on the blade  a little proximal to the tip and provide a better view of  ‘difficult to see’ larynx.   — Short-handled laryngoscopes are available for use in  difficult airways, e.g. pregnant women, obese patients.    Fig. 48.8: Endotracheal tube. (A) Standard 15 mm connector (B)  Pilot balloon (C) Inflatable cuff (D) Beveled patient-end (E) Black  line to indicate optimum positioning of the tube. This line is usually  placed just above the glottis    It is a 'C’-shaped tube and is commonly made of polyvinyl  chloride (PVC). The machine end has a standard 15 mm  diameter connector. The patient end is bevelled and has an  opening on the side just proximal to the tip called the Murphy's  eye. This ensures patency of the tube even if the bevelled tip  is against the tracheal wall.   7 or 7.5 mm/D  Age/3 + 3.5 mm/D  Age/4 + 4.5 mm/D    Endotracheal tubes are available in different sizes. Their size  is indicated as the internal diameter in mm (Key Box 48.3).  However, the correct size will depend on the growth of the  child.    ¢ The distance of several points on the tube from the patient  end is marked in cm along the tube. The tube is fixed at 22  or 23 cm in adult men and at 20 or 21 cm in adult women.  ¢ In children, the following formula is used:  Agel2 + 12-cm.    The tube should be positioned such that its tip must lie above  the carina but well below the glottis.    A pillow (7-10 cm) under the patient's head enables mild  flexion at the cervical spine. The head is then extended at the  atlanto-occipital joint. This is called the intubating position or  “sniffing position”.    Endotracheal intubation can be done either orally or nasally  (Figs 48.9 and 48.10). It can be done either under direct vision  or indirectly using a fibreoptic scope. It may need to be done  blindly when visualisation of the glottis by direct means is not  possible and a fibreoptic scope is not available.   In such cases, if the regular antegrade technique (mouth or  nose to larynx) is not possible, retrograde intubation (larynx  to mouth) may be tried. In the retrograde technique, a guide  wire is passed from the cricothyroid membrane upward into  the mouth or nose and the endotracheal tube is guided over it.    The patient’s head is placed in the sniffing position. The mouth  is opened and the laryngoscope blade is introduced through  the right angle of mouth along the tongue into the pharynx.  Once the epiglottis is seen, the tip of the laryngoscope blade is  pressed into the vallecula. This lifts up the epiglottis to reveal  the glottis. The glottis is identified by the two pearly white  vocal cords. Once the cords are seen, the endotracheal tube is  inserted between them into the trachea.   It may also be done with the patient awake after administer-  ing local anaesthesia to the upper airway when a difficult  intubation is anticipated.    Many airway adjuncts are available for use when a difficult  airway 1S encountered, especially when it is unanticipated.  These include oropharyngeal airway, nasopharyngeal airway,  laryngeal mask airway and Combitube.”    Correct position of the endotracheal tube may be confirmed  by the following    e Itis available in various sizes. The correct size 1s chosen  such that when it is placed along the side of the patient’s  face, 1t should extend from the angle of mouth up to the  tragus. This 1s inserted along the tongue and reaches up to  the posterior pharyngeal wall.   ¢ Care should be taken not to push the tongue backwards  with the airway itself.   ¢ To avoid that, the airway is inserted with its concavity  towards the palate and then turned once 50% of it is inserted  and passed further.   ¢ Please note that an oropharyngeal airway is excellent in  patients who are completely unconscious. Patients who  are conscious do not tolerate it. In patients who are  semiconscious, it may initiate a gag reflex and induce  vomiting. In such patients, a nasopharyngeal airway is a  better choice.    ¢ Itis a soft tube made of latex or silicon. It 1s available in  various sizes. The correct size 1s the same size as an  endotracheal tube for that patient.   ¢ The correct length is chosen such that when it 1s placed  along the side of the patient’s face, it should extend from   Fig. 48.12: Insertion of oropharyngeal airway. Take care not to  push the tongue backwards    the nostril up to the tragus. This 1s inserted along the nose  and reaches up to the posterior pharyngeal wall.    e This is a supraglottic airway that has revolutionised airway  management during anaesthesia. It has a tube or a shaft  with a standard 15 mm connector at the proximal end to  connect to the anaesthetic circuit. The patient end has an  oval-shaped inflatable cuff which when inserted rests just  above the larynx and creates an airtight seal.   ¢ To maintain airway in patients with difficult airway where  endotracheal intubation is not possible and are at risk of  hypoxia   e It may also be used as a conduit for passage of endotracheal  tube, either blindly or with the use of fibreoptic broncho-  scope.    Endotracheal extubation is as important as intubation. A  patient’s trachea may be extubated when the following criteria  are met    Equipment for reintubation and personnel skilled in  intubation should be readily available. After a good  oropharyngeal suction to clear the secretions, the cuff is  deflated and the tube removed. Oxygen should be administered  by face mask and the patient monitored till he is stable and  ready to go to the ward.    The administration of anaesthesia is associated with changes  in the internal homeostasis of the patient, especially the cardiac  and respiratory systems. Constant monitoring of the various  body systems is necessary to ensure the well-being of the  patient and prompt recovery from anaesthesia at the end of   ¢ Monitoring used in anaesthesia may be classified into:  noninvasive and invasive monitoring. The extent of  monitoring depends on the patient's preoperative condition,  the extent of surgery, the type of anaesthesia and the facilities  available.   ¢ Most patients are noninvasively monitored. Invasive  monitoring becomes necessary when considerable  haemodynamic instability exists or 1s expected to occur  perioperatively.   ¢ Minimum monitoring standards (according to guidelines  from Indian Society of Anaesthesiologists) for a patient  undergoing any type of anaesthesia are:  ~ Noninvasive blood pressure (NIBP)  — Electrocardiogram (ECG)  — Pulse oximetry   ¢ Monitoring capnography although not mandatory in India,  is an important monitor in patients undergoing anaesthesia.  In addition, monitoring of body temperature, anaesthetic  gases and neuromuscular blockade is desirable.    Basic noninvasive monitoring includes clinical observation  of the patient and monitoring of the patient's haemodynamic  parameters. Adequate cardiac output is associated with good  urine output, warm and well-perfused peripheries and  good capillary refill. Heart rate may be monitored with a finger  on the pulse. Blood pressure may be measured using a standard  sphygmomanometer. More conveniently and accurately, heart  rate and rhythm are continuously monitored using the  electrocardiogram and blood pressure using automated  noninvasive blood pressure monitoring systems.   Baseline readings of the heart rate, blood pressure and  oxygen saturation prior to induction of anaesthesia are  recorded. After induction, the patients are continuously  monitored and a record of these is made at least every five  minutes thereafter. When a patient undergoes an extensive or  a long procedure associated with large fluid shifts, his urinary  bladder 1s catheterised so that hourly urine output can be  measured. A normal person produces at least 0.5—1 ml/kg/hour  of urine output.    The objectives of electrocardiographic monitoring (Fig. 48.16)  are to monitor heart rate and rhythm and to look for myocardial  ischaemia. Lead II 1s monitored commonly as it 1s best for  identifying arrhythmias. V5 detects left ventricular ischaemia.  Hence, both Lead II and V5 should be monitored continuously  for patients suspected to have ischaemic heart disease. Subtle  changes in the electrocardiogram on the monitor must be  confirmed by a 12 lead—ECG.    Most automated devices use an oscillotonometric technique  and as a result the most accurate pressure 1s the mean arterial  pressure. They tend to overestimate at low pressures and  underestimate at high pressures. They may also give erroneous  results in patients with atrial fibrillation or with other  arrhythmias. The cuff width (Fig. 48.17) 1s the most important  determinant of the accuracy of the pressure reading. The cuff  width should be 40% of the mid-circumference of limb (the  length should be twice the width). Cuffs which are too narrow  tend to overestimate BP while those which are too wide tend  to underestimate BP.    The pulse oximeter displays the oxygen saturation of  haemoglobin continuously, noninvasively and in real-time  (Figs 48.18 and 48.19). Its use has revolutionised the way  patients are monitored, both perioperatively and in the ICUs.    The pulse oximeter works on the principle of Beer-Lambert's  law. This states that the amount of light absorbed bv a solution  is directly proportional to the amount of solute in it. The pulse    oximeter consists of two light emitting diodes (LEDs), emittins  light at around 660 nm and 940 nm. Oxyhaemoglobin (OxyHb  absorbs more of light at 940 nm whereas reduced haemoglobi1  (Hb) absorbs more light at 660 nm. The amount of OxyHb i  tissues increases with each pulse wave.   Fig. 48.18: A portable pulse oximeter and capnograph. (A) The  Capnograph and adaptor, (B) Pulse oximeter probe, (C) Graphical  display window that can be programmed to show either a  plethysmograph (as seen in the photograph) or a capnogram   Fig. 48.19: The plethysmograph and oxygen saturation (SpO>)  obtained by a pulse oximeter    The differential absorption of light by OxyHb and reduced  Hb and the varying concentration of these two substances  with arterial pulsations are utilised in the construction of  the pulse oximeter.   The pulse oximeter has one LED on, then the other on,  then both off. This happens several times a second. The ratio  of absorption of these two wavelengths of light by OxyHb  and reduced Hb is related to the oxygen saturation using a  nomogram.   e If the saturation dips below 90% or lower, hypoxia 1s said  to be imminent. Saturation of 90% corresponds to PaO, of  60 mmHg.   e Hence, the inspired oxygen concentration is adjusted to  maintain a SpO, of > 95% during anaesthesia.    e Movement: The pulse oximeter may not be reliable when  there is movement of the part monitored.   e Vasoconstriction: It may fail to pick up a pulse with  profound vasoconstriction as in hypothermia or low cardiac  output.   e COHb: It may over-read in patients with significant  carboxyhaemoglobin concentrations.   e Meth-Hb: It may under-read in patients with significant  methaemoglobin concentrations.   The amount of carbon dioxide in the exhaled gases can be  measured using a capnograph. The capnograph (Fig. 48.20)  passes infrared light through the gases to be analysed. The  carbon dioxide in the gases absorbs the infrared light in  proportion to its concentration. This absorption is measured  and the carbon dioxide concentration is displayed according to  a nomogram. When the carbon dioxide (CO) concentration 1s  displayed against time, a capnogram is obtained.    ¢ The normal capnogram has four different phases as  illustrated in Fig. 48.20.    ¢ The CO, concentration at the end of expiration is called  end-tidal CO, concentration (normal range: 35-40 mmHg)  which represents the alveolar carbon dioxide concentration.  Arterial CO, concentration is 0-5 mmHg more than the  end-tidal CO, concentration.   Fig. 48.20: The normal capnogram: Phase I—dead space gases,  Phase Il—mixed dead space and alveolar gases, Phase II|—  alveolar gases, Phase !V—inspiration. The arrow is pointing  towards the end-tidal carbon dioxide concentration (ETCO>)    Carbon dioxide is excreted from the body only through one  organ in the body, i.e. lungs. A normal capnogram obtained  from the exhaled gases helps identify the correct position of  the endotracheal tube.    e If the capnograph registers zero levels of carbon dioxide,  the endotracheal tube is malpositioned as in oesophageal  intubation or accidental extubation. Mechanical  ventilator disconnection also results in zero ETCO,  levels.    ¢ The ETCO, levels may also reduce to near zero levels if  no carbon dioxide retums to the lungs from the tissues as in  profound hypotension and an impending cardiac arrest  may be suspected.    ¢ However, the ETCO, will register zero if the patient is  in cardiac arrest since there is no circulation and no carbon  dioxide is brought to the lungs. In such situations, a  capnograph cannot be used to identify the correct position  of endotracheal tube. The capnograph will register CO, only  if effective CPR is being given.    ¢ However, a capnograph is extremely useful in assessing  the effectiveness of CPR. If ETCO, is > 15 mmHg  during CPR, chances of successful resuscitation are  higher.    ¢ A capnograph is also useful to detect hypo or hyperventi-  lation, bronchospasm and recovery from neuromuscular  blockade.    ¢ When there is an increase in physiological dead space  ventilation (that part of ventilation that does not take part  in gas exchange), the arterial to ETCO, gradient increases.    ¢ In such situations, arterial blood gas analysis can be done to  determine the gradient and then its trend can be followed.    Direct arterial pressure monitoring is preferred whenever  haemodynamic instability exists or is expected to occur  perioperatively. In such situations, invasive measurement  enables beat to beat monitoring of arterial pressure. A cannula  is inserted into an artery and connected to a pressure transducer.  The monitor displays beat to beat blood pressure both  numerically as well as graphically.    ¢ Dorsalis pedis artery: When radial arteries are inaccessible  or they have already been used.    Figs 48.21A to C: Radial arterial cannulation: (A) Cannulation,  (B) Catheter securely taped in place and labelled ‘Arterial line—  Do Not Inject!’, (C) The arterial waveform and pressure    ¢ Femoral artery: If the peripheral circulation is sluggish,  blood pressures are too low and the radial arteries are not  felt, cannulation of brachial or femoral arteries may be  considered. However, these sites should be changed to more  peripherally placed catheters as early as possible to avoid  complications such as distal ischaemia.   ¢ The distal circulation must be assessed periodically by  capillary refill time, colour of the digit, nail bed, pulse  oximetry, etc.   ¢ Contractility: The upstroke of the arterial waveform 1s  steep if the heart has good contractility.   ¢ Preload: The systolic variation in arterial pressure > 10%  with respiration indicates hypovolaemia.    ¢ A 20-gauge cannula may be used for the radial, brachial o  dorsalis pedis arteries 1n adults.    ¢ Ischaemia distal to cannula: Normally collateral circulatior  1s adequate to maintain perfusion distal to the arteria  cannulation site. Occasionally ischaemia may occur wher  associated with low cardiac output, sepsis, shock, high-dose  vasopressors or vasculitis.   ¢ Exsanguination: In case of accidental disconnection, blooc  loss of up to 500 ml/min can occur (through 18 G cannula)    A catheter inserted into the central vein with Its tip positioned  in the superior vena cava enables measurement of the right  atrial pressure or central venous pressure. It also provides a  good venous access.   Normal values: 2—5 cmH,O in a spontaneously breathing  patient. 5-10 cmH,O in a mechanically ventilated patient.   1. To guide fluid therapy as in hypovolaemia, low cardiac  Output states, renal failure    3. To infuse potent drugs such as antibiotics, vasopressors,  chemotherapeutic agents    4. When peripheral venous access 1s not available  5. For PA catheterisation and cardiac output measurement.    ¢ Internal jugular vein (IJV): Right IJV is preferred over  left IJV because:  — RIJV 1s more tn line with the superior vena cava.  — Injury to thoracic duct is avoided.   ¢ Peripherally placed central venous catheters may also be  useful in measuring CVP if the tip of the catheter 1s  intrathoracic.   ¢ Femoral vein catheters may also be used provided the tip  of the catheter 1s close to the diaphragm in the inferior vena  cava. However, It 1s not preferred as it 1s close to the inguinal  region and maintaining asepsis 1s difficult. It also interferes  with the patient’s mobility.   FLUID CHALLENGE TEST  CVP (cmH,O) Amount of fluid given over 10 min  8   e If CVP rises by < 5 but > 2 cmH,O, stop fluids, wait for  10 minutes and then give a smaller volume    CVP may be used as a guide to right ventricular filling. The  5—2 rule is a useful way of assessing fluid responsiveness.  Baseline CVP is recorded and then fluid is infused (SO—200 ml  of colloid over 10 min) if hypovolaemia ts suspected.    ¢ The CVP displayed depends on correct positioning of the  tip of the catheter.   ¢ The position and calibration of the transducer are important.  The transducer must be placed at the level of the midaxillary  line (Supine patient) and the sternal angle (sitting patient)  and then zeroed to atmospheric pressure.    ¢ It can change with changes in right ventricular compliance  also. For example CVP does not reflect left ventricula:  filling in patients with pulmonary hypertension or in righ:  ventricular dysfunction. In such cases, dynamic changes ir  CVP are more useful than absolute values.    ¢ Catheter-related infection and sepsis  ¢ Disconnection, leading to bleeding or air embolism  ¢ Pleural or pericardial effusion due to misplaced catheter    These are long catheters placed in the PA through the central  veins, right atrium and right ventricle (Fig. 48.24). The tip of  this catheter ends in a small distal branch of the pulmonary  artery. When the balloon on Its distal tip is inflated, the pressure  distal to the catheter reflects the left atrial pressure which  may reflect left ventricular end-diastolic pressure and hence  the preload more accurately (Fig. 48.25). PA catheter insertion  may be associated with a number of complications and must  be used judiciously.    ¢ If a PA catheter 1s in place, 7—3 rule can be used to guide  fluid challenge similar to the 5~2 rule with a CVP catheter.   ¢ A rise in PCWP may be the earliest indicator of left  ventricular dysfunction. A new onset mitral regurgitation  can also be detected in the wedge pressure trace.   ¢ Some PA catheters can be used to pace the left ventricle   Fig. 48.25: Insertion of a PA catheter with the pressure trace  display as a guide. The corresponding position of the catheter in  the heart is shown diagrammatically below    Local anaesthetics are drugs when injected around the nerves  block impulse conduction distal to the site of injection and  produce analgesia and anaesthesia in that area.    Local anaesthetics consist of a hydrophilic tertiary amine group  linked to a lipophilic aromatic group. They are classified into  two main categories based on this linking group: the  aminoamides and the aminoesters.    A nerve impulse is transmitted by progressive opening of  sodium channels across the membrane and sudden influx of  sodium into the intracellular fluid. Local anaesthetics produce  sodium channel blockade. They block the fast sodium  channels and the sodium influx, thus blocking all impulse  transmission across the membrane.   Local anaesthetic exists in two forms: Ionised and  nonionised. The nonionised form 1s lipophilic and crosses the  phospholipid membrane more easily. The ionised form is  hydrophilic and blocks the channel in the open state and blocks  nerve transmission (use-dependent blockade). The drug blocks  the channel from the intracellular direction.    Higher the lipid solubility, higher is its ability to penetrate the  lipoprotein membrane and hence greater is its potency.    The pKa of a drug Is the pH at which the ionised and nonionise  portions of the drug are equal. The lower the pKa, lower 1s th  degree of ionisation for any given pH. The nonionised portio1  1S lipophilic and crosses the cell membrane more easil:  hastening the onset of nerve blockade. For example, lignocain  has a pKa of 7.9 and acts faster than bupivacaine with a pK:  of 8.1.   Acidosis decreases the proportion of the nonionised drug anc  reduces the amount of drug able to cross the membrane. Tha  1s why local anaesthetics do not work optimally when injectec  into an infected tissue (pH is acidic in these tissues).    Protein binding  The greater the degree of protein (membrane proteins) binding.  longer is the duration of action.    ¢ Lignocaine: Skin infiltration—0.5—1%  Minor nerve block—1%  Epidural—1 .5-—2%  Spinal anaesthesia—5% hyperbaric (heavy)  Two topical preparations—2% lignocaine jelly and 4%  lignocaine spray for the mucosal surfaces of the body.  Note: |% means each ml contains 10 mg of the drug, 2% to  20 mg/ml, and so on.    ¢ Ropivacaine: Available as 0.2% for providing postoperative  analgesia, labour analgesia and as 0.75% for spinal and  epidural anaesthesia and nerve blocks.   * Ropivacaine—S5 mg/kg, not to exceed 200 mg for minor  nerve blocks.   ¢ Lignocaine (preservative free) when used as an anti-  arrhythmic agent 1s given IV, in a dose of 1—2 mg/kg.    Local effects  Local anaesthetics block the sodium channels in the neuronal  membrane and thus the propagation of impulses across It.    These drugs can produce systemic effects when high plasma  levels of the drug are achieved. This may also be deliberate as  when lignocaine is used as an antiarrhythmic agent. It is    classified as Class Ia drug (sodium channel blockers) in the  Vaughan Williams classification of antiarrhythmic agents.   When high plasma concentrations of local anaesthetics are  achieved, either due to accidental intravenous injection of the  drug or due to intravascular absorption of large amount of drug  infiltrated in a region, systemic toxicity can occur.    Adrenaline containing preparations should not be used  for nerve blocks of fingers, toes and penis as it can cause  ischaemia.   If significant amount of local anaesthetics reach the tissues of  heartand brain, they exert the same membrane stabilising effect  as on peripheral nerve, resulting 1n progressive depression of  function. The toxicity of local anaesthetics 1s dose-dependent.  These drugs always produce central nervous system (CNS)  toxicity first. As the plasma level rises, cardiovascular toxicity  and collapse occur.    The plasma levels of lignocaine required to produce  cardiovascular collapse (CVS toxicity) 1s seven times that  required to produce convulsions (CNS toxicity). Bupivacaine  requires only three times the plasma level to produce cardiac  toxicity as for CNS toxicity. Thus, bupivacaine has a greater  potential for cardiotoxicity.    With all local anaesthetics, central nervous system  toxicity always comes first. This is followed by cardiac  toxicity.    Clinical features of local anaesthetic toxicity are related to  the plasma level of the local anaesthetic. The clinical effects  and their relation to plasma level of lignocaine are given in  Table 48.4.    9 The clinical effects and their relation to plasma level of lignocaine    The likelihood of toxicity of local anaesthetics depends on  several factors:  . Amount of drug injected  . Site of injection—vascularity  . Addition of vasoconstrictors  . Rapidity of injection  . Nature of drug given  . Presence of associated conditions such as low cardiac output  or renal failure.    Lignocaine is mostly used to produce peripheral neural  conduction blockade. It can be used in doses not exceeding  5 mg/kg body weight for plexus blocks or infiltration. When  combined with vasopressors such as adrenaline (1:200,000 =  5 ug/ml), the dose can be increased up to 7 mg/kg. The  intravenous dose of lignocaine 1s 1—1.5 mg/kg when used as  an antiarrhythmic. However, even a small dose such as 20 mg  in an adult when injected accidentally into the carotid artery  may be sufficient to produce convulsions.   Bupivacaine is used only for nerve blocks or infiltration. It  is not an antiarrhythmic drug. Bupivacaine is cardiotoxic and  care should be taken not to exceed the prescribed doses.  Circulatory collapse and cardiac arrest due to large doses of  bupivacaine can be very resistant to resuscitation. It can be  used in a dose of up to 2.5 mg/kg body weight.   Ropivacaine is a newer amide local anaesthetic agent which  is similar to bupivacaine but with less cardiotoxicity. A levo  isomer of bupivacaine, called levobupivacaine ts also less  cardiotoxic and has been put into clinical use recently.    Certain sites are very vascular as compared to others. A higher  plasma level of local anaesthetic 1s reached when the same  amount of local anaesthetic is used for multiple intercostal  nerve blocks as compared to brachial plexus block.    ¢ Aspirate to rule out presence of the needle tip in a vessel  before injecting the drug.    ¢ Avoid injecting large boluses at once. Small boluses, given  slowly to achieve the desired effect are safer.    The toxicity of local anaesthetics manifests as CNS depression  and convulsions. Maintenance of airway, breathing and  circulation must be a priority. These convulsions generally last  for a short period of time.    ¢ Patency of the airway must be maintained.  ¢ Oxygen by face mask.  ¢ Ventilation, if apnoea occurs.    ¢ Convulsions are treated with intravenous diazepam or  thiopentone in incremental doses.    ¢ Cardiovascular collapse with ephedrine, inotropes and  vasoconstrictors, and CPR as needed.    ¢ Injection of local anaesthetics around the spinal cord to  produce a reversible blockade of impulses that pass through  it, is called central neuraxial blockade.    ¢ When the local anaesthetic 1s injected into the cerebrospinal  fluid bathing the spinal cord, it is called spinal anaesthesia  (subarachnoid block).    ¢ When the local anaesthetics are injected into the epidural  space to block the nerves that emerge from the spinal cord,  it is called epidural anaesthesia.   The local anaesthetics spread from the site of injection by mixing  with the cerebrospinal fluid (CSF). They reach the nerve fibres  in the spinal cord and block transmission of impulses below the  highest level of spread. The concentration of the drug is higher  in the caudal regions and reduces with increasing distance  cranially. A lower concentration of thedrug 1s sufficient to block  smaller fibres (C and Ad) whereas the thick motor fibres (AQ)  require a larger concentration to get blocked. Thus, a  differential blockade is seen after a spinal anaesthetic and 1s  as follows:    ¢ Motor block up to a certain level (depends on the dose of  drug injected)    ¢ Sympathetic block about two to four segments above th  level of sensory block.   ¢ Hypotension: Due to the blockade of sympathetic nerve  below the level of spinal block, there 1s profound vaso  dilatation in the affected areas. A relative hypovolaemi:  occurs and hypotension is usually seen. The extent o  hypovolaemia depends on the preoperative volume status  level of block and ability to compensate for the vaso  dilatation. The vessels in the upper limbs constrict tc  compensate for vasodilatation in the lower limbs. This 1:  described as pink trousers, blue jacket phenomenon.   ¢ Bradycardia: Heart rate is maintained in low blocks, but ir  higher blocks (high thoracic), sympathetic nerve block of the  cardioacceleratornerves can occur causing unopposed actior  of the parasympathetic system and bradycardia.   ¢ When the level of spinal anaesthesia ascends, the intercostal  nerves are gradually blocked.    ¢ The diaphragm 1s not easily paralysed as the phrenic nerve  is a thick and strong nerve and arises from cervical nerve  roots (C3 Cy Cs).   ¢ In high spinals, the alveolar ventilation reduces and may  lead to hypoxia and hypercarbia.    Gastrointestinal system  ¢ Unopposed parasympathetic activity leads to constriction  of gut with increased peristaltic activity.    e Nausea, retching or vomiting may occur and may be the  symptom of impending hypotension. These symptoms  disappear when the hypotension 1s corrected. Occasionally,  it may need administration of an anticholinergic or an  antiemetic agent.    ¢ However, since the bowel 1s contracted and small and the  skeletal muscle relaxation produced 1s greater, the surgeons  find it easier to operate on such a gut.    A preprocedure check of the anaesthesia equipment,  resuscitation equipment and drugs 1s made. An intravenous  line 1s placed and monitoring commenced. The patient is then  positioned for spinal anaesthesia.    The spinal anaesthetic may be administered with the patient  in lateral or sitting position.   Lateral: The patient lies either in the left or right lateral  position. The back should be parallel to the edge of the  operating table and perpendicular to the ground. The legs  should be flexed at the hips as much as possible.   Sitting position: The patient sits on the table, with the back  bent forward. He is allowed to rest his arms on pillows. The  back is cleaned with spirit and betadine and draped. Under  aseptic precautions, the vertebral spines are identified in the  lumbar region. The highest point of the iliac crest corresponds   Fig. 48.26: Spinal anaesthesia in the lateral position, spinal  needle is in place and CSF drop is seen at the hub    to L3-4 space. The L2—3, L3—4, L4—5 intervertebral spaces  can also be used. A space higher than this is not used as the  spinal cord ends at LI in adults. This point is lower in children  and should be borne in mind in paediatric spinals.    The subarachnoid space may be approached either from the  midline or by a paramedian technique. A subcutaneous wheal  of local anaesthetic is raised in the chosen intervertebral space.    Midline approach: The lumbar puncture needle ts inserted in  the midline, midway between the spines and perpendicular to  the skin. The spinal needle passes through the following  structures to reach the subarachnoid space:    Paramedian approach: The needle is inserted a finger-breadth  lateral to the spine and advanced ina slightly cephalad direction  towards the midline. If the needle touches the lamina, it should  be redirected medially. The correct position of the needle ts  identified by obtaining a free flow of CSF. This approach helps  access the subarachnoid space in those patients whose  interspinous and supraspinous ligaments are calcified or  in patients unable to bend enough to open the interspaces  well.    The local anaesthetic is now injected into the CSF, taking  care not to displace the needle.    These may be classified into Minor and Major based on the  reversibility and seriousness of the complication.   This is treated with intravenous fluids to compensate for the  vasodilatation. If necessary, incremental doses of a  vasoconstrictor may also be used.   If the cardioaccelerator nerves (T1—T4) are blocked. This is  usually easily treated with an anticholinergic such as atropine  or glycopyrrolate. If profound, a small dose of adrenaline may  be required (very rare).   The incidence of PDPH depends on the size of the needle used,  number of punctures made, fluid status and ambulation. With  finer (25 and 26# needles) and good hydration of the patient,    PDPH is uncommon. This may be treated with rest, increased  fluid intake, plenty of coffee and NSAIDs. Rarely, an epidural  blood patch (vide infra) may be required.   If the level of spinal anaesthesia is high and all intercostal  muscles are paralysed, respiratory depression may occur.  However, diaphragm, the principal muscle of respiration ts  supplied by the thick phrenic nerve which does not get blocked  easily. Any respiratory depression seen during spinal  anaesthesia is more due to hypoperfusion of the respiratory  centre (due to hypotension). This can be treated with respiratory  Support as required and stabilisation of blood pressure.    Backache: This is not a problem of spinal anaesthesia per se  but may be due to faulty positioning during surgery.    In this type of central neuraxial blockade, local anaesthetic is  injected in the space around the dura (epidural space). The  local anaesthetic blocks the nerves as they emerge through the  intervertebral foramen. Some of it diffuses through the  meninges into the spinal cord and acts on the spinal cord.    A preprocedure check of the anaesthesia equipment,  resuscitation equipment and drugs 1s made. An intravenous  line is placed and monitoring of heart rate, electrocardiogram,  blood pressure and oxygen saturation is established and the  baseline noted.    The epidural puncture can be done with the patient in sitting  position or in the lateral decubitus position.    The patient's back is cleaned with an antiseptic and then draped.  Under aseptic precautions, epidural puncture is done using a  16 or 18# Tuohy needle. This needle has a blunt tip to reduce  the risk of dural puncture. The needle is inserted either in the  midline or by a paramedian approach. It passes through the  same tissues as in lumbar puncture except the subarachnoid  space. The needle is inserted along with its stylet and always  advanced slowly from skin onwards. Once the subcutaneous  tissue 1s entered, the stylet is removed. A 2 ml or 5 ml syringe  with a freely moving plunger and containing either air, saline  or both is then connected to the needle hub. A gentle attempt    at injection of this air or saline is made as the needle advance  through the tissues. The entry of the needle into the epidure  space 1s heralded when it penetrates the ligamentum flavur  and there ts a loss of resistance to injection of air or saline  This is taken as the end-point. An epidural catheter 1s passe  through this needle and advanced to about 3-4 cm into th  space. The needle is removed and the catheter taped and fixe:  to the back. A bacterial filter is attached to the injection pot  of the catheter (Figs 48.28A to C).    Figs 48.28A to C: Epidural anaesthesia: (A) Eliciting loss of  resistance to air, (B) Epidural catheter attached to bacterial filter,  (C) Epidural catheter taped to the back    The epidural needle or catheter may accidentally enter an  epidural vein or the subarachnoid space. To avoid injecting a  large dose of local anaesthetic into either of these spaces, a  test-dose containing a small amount of local anaesthetic (3 ml  of 2% = 60 mg lignocaine) and 15 tg of adrenaline 1s injected.  Any sensory or motor block following this dose would suggest  an accidental dural puncture resulting in spinal anaesthesia.  An accidental intravascular injection is identified by an  increase in the heart rate and blood pressure within a minute  of the injection. In either situation, the epidural catheter may  need to be withdrawn or replaced. If neither response 1s seen,  an epidural placement 1s assumed and the full dose of local  anaesthetic 1s injected in divided doses. The patient should be  continuously monitored till the block wears off.   The epidural needles are large and dural puncture results in a  larger leak of cerebrospinal fluid (CSF). This results in low  CSF pressures. Whenever the patient sits up or becomes  ambulatory, a drag occurs on the brain and the meninges due  to gravity and loss of CSF. This results in a typical postural  headache referred to the occipital region. The pain disappears  when he lies down supine. This 1s more common in obstetric  patients. It may occur up to 2 to 7 days after lumbar puncture  and may persist for up to 6 weeks.    Plenty of oral fluids may increase CSF production. Rest, plenty  of coffee and NSAIDs may also help. Rarely, an epidural blood  patch may be required.    Epidural blood patch: If the headache is very severe, an  epidural blood patch may be given. 10 to 15 ml of patient's  own blood is drawn under aseptic precautions. Simultaneously,  epidural puncture 1s made in the same space as the previous  epidural puncture. The freshly drawn blood is injected into  the epidural space which clots and seals the puncture hole.  This is nearly 100% effective in relieving the headache.   When a large dose of local anaesthetic 1s injected intrathecally  inadvertently, all spinal nerves are blocked, causing profound  hypotension, bradycardia and collapse. If the patient is  continuously monitored and treated promptly, this 1s completely  reversible.    Treatment  1. Volume infusion and vasopressors  . Endotracheal intubation and ventilation as necessary.    Can be done in the lumbar, sacral (caudal), thoracic or cervical regions.    Placement confirmed by using loss of resistance to injection of air, saline  or both    The local anaesthetic spreads both caudad and cephalad. Segmental block  can be achieved    Epidural catheters are routinely introduced and hence duration of anaesthesia  can be prolonged with repeated boluses or continuous infusion through  catheters    PDPH not seen, unless dura is inadvertently punctured  Inadvertent intrathecal or intravascular injection of large amount of drugs    possible, with consequent complications such as total spinal blockade and  local anaesthetic toxicity respectively.    This is a very popular technique in providing postoperative  analgesia in children. It involves injection of local anaesthetics  with or without opioids in the caudal epidural space.    The patient is positioned in lateral position with the knees  flexed and the back perpendicular to the ground. It can also be  given with the patient in prone position. The area over the  sacrum and the gluteal region is cleaned and draped.    Needles  A 22 or 23 G hypodermic needle or a scalp vein set 1s used to  administer the block.   The needle is inserted at the apex of the sacral hiatus at a 60°  angle to the skin. A distinct ‘pop’ or a ‘give way’ Is felt as the  needle punctures the sacrococcygeal membrane. The angle of  the needle is then changed to about 15° to 20° to the skin and  advanced a little further into the sacral epidural space. The  latter step 1s optional and has to be done with caution as the  dural sac may end relatively low in infants. After careful  aspiration to rule out blood or CSF, a small dose of local  anaesthetic 1s injected. There should be no resistance to  injection. A subcutaneous injection should also be ruled out.   0.25% bupivacaine in a dose of 0.5 ml/kg 1s sufficient for  perineal and low sacral procedures, | ml/kg for lumbosacral  procedures and 1.5 ml/kg for lower abdominal procedures.  However, a total volume of 20 ml and a total dose of 2.5 mg/  kg of bupivacaine may not be exceeded.    ¢ Postoperative pain relief in children for perineal and  lumbosacral procedures.   ¢ Itis also used to supplement general anaesthesia for perianal  procedures in adults.    ¢ Intrathecal injection is possible, especially in smaller infants  who have extension of dural sac down to S3.    Injection of local anaesthetics injected around the brachiéz  plexus produces analgesia and even surgical anaesthesia 1  the upper limb. The brachial plexus can be blocked by fou  different approaches: interscalene, supraclavicular, infra  clavicular or the axillary. Of these, the supraclavicular and th  axillary techniques are the most popular.    Supraclavicular brachial plexus block  Position  The patient is positioned in supine position with the head tume:    to the opposite side of the block. The area over the lower par  of the neck 1s cleaned and draped.    A 22 G hypodermic needle, a scalp vein set or an insulatec  needle with an internal nerve stimulator may be used tc  administer the block. Ultrasound guidance is becoming  increasingly popular to guide nerve blocks.    The needle is inserted at a point 1 to 1.5 cm above the midpoin  of the clavicle. The subclavian arterial pulsations are felt witt  the thumb of one hand and the needle advanced posterior to 11  with a medial and caudal direction. Paraesthesia must be sough  in the upper limb and the injection of local anaesthetic made  at the point of paraesthesia. Undue pain during injection  suggests intraneural injection. Careful aspiration for absence  of blood to rule out intravascular injection 1s mandatory before  injection of the local anaesthetic.    ¢ Intraoperative analgesia and postoperative pain relief in  adults and children.    ¢ Sole anaesthetic in adults for procedures on the upper limb.    The patient 1s positioned in supine position with the arm  abducted to a right angle and the elbow bent at right angles to  the arm. The area over the axilla is cleaned and draped.    A 22 G hypodermic needle, a scalp vein set or an insulated  needle with an internal nerve stimulator may be used.   ¢ The needle is inserted at a point just above the point of  maximum pulsations of the axillary artery but parallel to  the artery. This point should be as high in the axilla as  possible. Paraesthesia must be sought in the upper limb and  the injection of local anaesthetic made at the point of  paraesthesia. Undue pain during injection suggests  intraneural injection. Careful aspiration for absence of blood  to rule out intravascular injection is mandatory before  injection of the local anaesthetic.    ° A single injection of the local anaesthetic can be made at  this point. Alternately, half the total volume of injection  can be made at a point on the other side of the artery.    Either bupivacaine not exceeding a total dose of 2.5 mg/kg;  lignocaine plain 5 mg/kg or with adrenaline 7 mg/kg may be  used.   Intraoperative analgesia, anaesthesia and postoperative pain  relief in children and adults for procedures on the upper  limb.    This is a popular technique in providing intra- and post-  Operative analgesia in adults undergoing procedures on the  foot.    The patient is positioned supine. The foot is raised by an  assistant and the area around the ankle 1s cleaned and draped.    Needles  A 22 G hypodermic needle ts used to administer the block.    |. The posterior tibial nerve 1s blocked with 3—5 ml of local  anaesthetic at a point midway between the medial malleolus  and the heel, just behind the posterior tibial arterial  pulsations.    2. The sural nerve may be blocked at a point midway between  the lateral malleolus and the heel, just lateral to the Achilles’  tendon.    3. The deep peroneal nerve is blocked at a point midway  between the lateral and the medial! malleoli lateral to the  tendon of extensor hallucis longus and anterior tibial artery.    4. The saphenous nerve and the superficial peroneal nerves  are easily blocked by raising a subcutaneous wheal of local  anaesthetic between the malleol! anteriorly.    Lignocaine plain not exceeding 5 mg/kg or bupivacaine not  exceeding 2.5 mg/kg may be used.    One of the main anxieties of undergoing an operation ts the  pain that is anticipated along with it. An anaesthesiologist’s  responsibilities do not end with providing safe perioperative  care. He must also ensure that the pain associated with the  surgery 1s relieved adequately so that the surgery itself is not  so unpleasant an experience.   Pain is defined as an unpleasant sensory and emotional  experience associated with actual or potential tissue  damage, or described in terms of such damage. Pain can be  acute or chronic. The pain that is seen immediately after  surgery 1S acute postoperative pain and Is easier to treat. The  mechanism of perpetuation of chronic pain is less well  understood and is more difficult to treat.    Pain begins with injury to the tissue. Both the peripheral and  the central nervous systems are involved in the ultimate pain  perception (Fig. 48.29).    Four basic mechanisms are involved in the appreciation of  pain: transduction, transmission, modulation and perception.  Pain relief can be achieved by blocking each of these steps.    The transduction of noxious stimuli begins with peripheral  nociceptors. These nociceptors convert the stimulus  (transducer) of injury (heat, pressure, mechanoception) to pain  sensation. This is also assisted by the production of  prostaglandins and leukotrienes in response to the injury.  Nonsteroidal anti-inflammatory analgesics can be used to  reduce their production. Local application of local anaesthetics  also reduces transduction.    Signals from these nociceptors travel along myelinated Ao and  unmyelinated C fibres. Their axons synapse in the dorsal horn  of the spinal cord, where the neurons of laminae (of Rexed) I,  II and V are most involved in the perception of pain. These  fibres can be blocked at the nerve plexus level using local  anaesthetics.    Large fibre inputs from other sensory modalities and  descending pathways can modulate activity in the dorsal horn.  The extent of this modulation results in differing levels of pain  experienced by patients. This can be further modified using    The signals then travel along the spinothalamic tract of thi  spinal cord to the thalamus and the cortex which ultimatel  cause activity in the appropriate portion of the sensory corte?  and the limbic system. The response of the cerebral cortica  cells and the limbic cells can be modified using opioids anc  alpha-2 agonists.    Often, a combination of all these techniques is used tc  achieve good pain relief. Relief of pain helps the patient hea  faster and return to normal activities early. The complications  of inadequate pain relief such as respiratory infections  hypertension, myocardial infarction and increased catabolism  are avoided or minimised.    Pain is more difficult to measure as compared to othe:  parameters such as temperature or heart rate. The measurement  of pain is mainly dependent on the patient’s perception of 11  and may be done in any of the following ways:    1. Visual Analogue Scale (VAS): The pain is assessed along  a 100 mm scale where 0 corresponds to no pain at all and  100 to the worst imaginable pain. The patient scores his  pain at any point he finds appropriate. The pain relief is  assessed thereafter from this point. The VAS is the most  popular tool used to assess pain and its relief.    2. Category rating scale: The different ratings are no pain,  mild, moderate or severe.    3. Wong—Baker Faces pain rating scale: A scale with  different faces, a happy face at one endand a face in severe  pain and crying at the other with four other faces in between  may be used for children (Fig. 48.30).    4. Biological measures such as tachycardia, hypertension,  tachypnoea or inadequate breathing are relatively inaccurate  but useful estimates of pain.    The initial response to a noxious stimulus 1s brief and correlates  with the sharp, well-localised initial pain. The second phase of  the response is more prolonged and correlates with the dull,  diffuse pain experienced after the initial injury. This second  phase is associated with a growing region of hypersensitivity    1098  around the point where the noxious stimulus was initially  applied and is said to result in chronic pain. The response to  noxiousstimulican be modulated by theirrepeated application.  Peripheral nociceptors become more responsive with the  repeated application of noxious stimul!. Their sensitivity canbe    further enhanced by many tissue factors and inflammatory  mediators released in the course of tissue injury.    The process through which the neurons of the dorsal horn  of the spinal cord become sensitised by prior noxious stimull  is often referred to as “windup” or “central sensitisation.” Much  less is known about pain-induced sensitisation of the  supraspinal components of the CNS. This phenomenon leads  to chronic pain where the patient experiences pain, often,  excruciating, and long after the stimulus that initially  caused the painis removed. The relief of chronic pain 1s more  difficult. Various techniques used to treat chronic pain are as  follows:    The practice of anaesthesia has become very safe due to better  preoperative evaluation and preparation, careful choice of  patients, better monitoring, availability of safer drugs and safer  anaesthetic techniques. The incidence of complications has  come down drastically. However, complications can still occur.  The perioperative (pre-, intra-, and postoperative periods)  complications can be classified as follows:    ¢ Postoperative drowsiness  ¢ Postoperative nausea and vomiting  ¢ Neurologic complications of regional blockade.    Airway obstruction may occur during the induction of general  anaesthesia. When a person becomes unconscious, the tongue  and the epiglottis fall back and can obstruct the airway. The  patency of the airway 1s usually maintained fairly easily by  the anaesthesiologist by using chin lift or jaw thrust  manoeuvres. A definitive airway such as an endotracheal tube  may then be inserted into the trachea.    Occasionally, the chin lift or jaw thrust manoeuvres are  inadequate to maintain a patent airway as in patients with  abnormal airways. Insertion of an endotracheal tube may also  prove to be difficult in certain individuals. An oral or  nasopharyngeal airway may be inserted to overcome this  obstruction and maintenance of the airway for a short duration.  If there is difficulty in inserting the endotracheal tube due to  supraglottic causes and oral or nasopharyngeal airways are  not sufficient to relieve the obstruction, insertion ofa laryngeal  mask airway or a Combitube® may be attempted. If the problem  is at the glottis or subglottis and the airway is obstructed, an  emergency cricothyrotomy or a tracheostomy may be required.    A thorough assessment of the airway must be done  preoperatively and a management plan A formulated. Plan B  and Plan C also should be considered in the eventuality of  failure of Plan A. Generally loss of life occurs not because of  inability to intubate but due to an inability to oxygenate and  ventilate the patient. This situation, also called, ‘cannot  intubate—cannot ventilate’ (CVCI) is one of the most dreaded  situations faced by an anaesthesiologist.    Bronchospasm may occur in a patient under anaesthesia. The  possible causes are as follows:    + Irritable airways as in a known asthmatic, chronic  obstructive airways disease, 1.e. exacerbation of pre-existing  bronchospastic disease.    A patient is said to be in respiratory failure if he is unable to  maintain adequate oxygenation and ventilation, 1.e. arterial  blood gas tension of O, less than 60 mmHg (when patient is  breathing 60% O,) and CO, more than 50 mmHg. This could  be acute or acute exacerbation of chronic respiratory failure.   ¢ Lung parenchymal disorders such as pneumonia, atelectasis,  aspiration, pneumothorax, pulmonary embolism   ¢ [Inadequate respiratory excursion due to pain or thoracic  cage abnormalities such as kyphoscoliosis.   e Weakness of muscles, e.g. prolonged effect of muscle  relaxants, myasthenia gravis    ¢ Intermittent positive pressure ventilation and ventilatory  support till the patient improves.    Hypertension, hypotension and arrhythmias occur  perioperatively due to various reasons such as inadequate  preoperative treatment, surgical stress, inadequate anaesthesia,  metabolic or endocrine causes or even drug interactions. Brief  periods of haemodynamic instability, although common are  well-tolerated by healthy individuals. Continuous and  appropriate monitoring and prompt treatment should avoid  long-term complications.   Hypotension may be due to decreased preload, reduced  contractility or increased afterload to the left ventricle. If not  identified or treated in time, 1t may progress to shock and  cardiac arrest. Hypovolaemic shock is the commonest type of    shock encountered during surgery. However, cardiogenic shoc  due to perioperative myocardial infarction, anaphylactic shoc  due to allergic reaction to anaesthetics or neurogenic shoc  due to vasovagal attack, high spinals may also occur 1  susceptible individuals.    Perioperative myocardial infarction (Ml) is a compl  cation that occurs in susceptible individuals. It may be cause  due to extreme and sustained variations 1n haemodynamic  such as hypotension, hypertension or tachycardia. It may als  be caused due to thrombosis as the patient 1s in  hypercoagulable state due to stress of surgery. The highe:  incidence of perioperative MI is seen not on the day of surger  (when the patient is under the vigilant care of th  anaesthesiologist) but on the second or the third day when th  attention given to him ts less in terms of pain relief o  haemodynamic changes.    Rarely, a patient under GA might recall events that occurrec  during the procedure. This is termed awareness durin;  anaesthesia and is one of the most dreaded complications b:  both anaesthesiologist and the patient. This is more likely t«  occur if the patient’s haemodynamics are very unstable (a:  in postpartum haemorrhage, trauma) and the anaesthesiologis  fears further cardiac depression may occur with the use o  inhalation agents. The use of opioids and nitrous oxide may  provide analgesia but not the amnesiaand anaesthesia required  With increased awareness of this complication among  anaesthesiologists coupled with the use of benzodiazepine:  and modern anaesthetic agents which are more cardiostable  the incidence of this is reduced. A new monitor called BIS  index monitor provides some information of the conscious  state of the patient but is not widely available yet.    A patient may be slow to awaken after anaesthesia due to  persistent effect of the anaesthetic agents or opioids  administered during the anaesthesia. However, it may be due  to metabolic causes such as hypoxia, hypothermia, hypo- or  hypernatraemia, hypo- or hyperglycaemia. If all these causes  are ruled out, a neurological consultation is obtained to rule  out any space-occupying lesions in the central nervous system,  stroke or hypoxic encephalopathy.    PONV is a frequent complication of anaesthesia. It is common  in women, after laparoscopic surgeries, squint surgeries and  is associated with the use of nitrous oxide and opioids or even  early oral intake postoperatively. It may be treated with IV  metoclopramide (10 mg), ondansetron (4-8 mg) or  dexamethasone (4—8 mg) 1n an average adult.    Nerve injuries may arise as a complication of regional  blockade. Complications such as adhesive arachnoiditis, cauda  equina syndrome or paraplegia have been reported after spinal  and epidural anaesthesia but are extremely rare. The use of  tourniquet, if prolonged or if very high pressures are used,  can also cause nerve injuries.    Peripheral nerve injuries may occur perioperatively due to  improper positioning under regional or general anaesthesia.  The common peroneal nerve and the sciatic nerve can be  injured during lithotomy position. The ulnar and the radial  nerves may be affected in the arm or at the elbow due to  inadequate attention to positioning. Brachial plexus stretch  injury can occur if the arms are allowed to be abducted more  than ninety degrees. Injury to optic nerves or the retina may  occur in prone position due to compression of the eyeball.  Corneal injury may occur due to exposure In an unconscious  patient.    Renal failure, usually prerenal is associated with large fluid  shifts or major haemodynamic instability. Direct injury to the  kidneys (acute tubular necrosis) may occur if adequate attention  is not given to prerenal failure. Methoxyflurane, an inhalation  anaesthetic agent can cause renal failure but is no longer in  clinical use. Renal failure can also occur as part of hepatorenal  syndrome or after a mismatched blood transfusion.    A patient with compromised hepatic function may proceed to  hepatic failure perioperatively, e.g. cirrhosis of liver,  obstructive Jaundice. Hepatic failure may also occur due to  infective complications such as hepatitis or sepsis. Massive  hepatic necrosis has been reported after repeated use of  halothane. The incidence of this is very rare and must be a  diagnosis of exclusion.    Cardiac arrest is an inevitable and natural event in every one’s  life. However, this event may have been precipitated  prematurely due to some underlying but treatable cause. If  prompt intervention 1s made and the patient’s heart and  respiration are supported at this stage, precious time may be  gained to treat this precipitating cause. Many victims of cardiac  arrest may lead normal lives once this calamity ts tided over.  This is the basis of cardiopulmonary resuscitation.   CPR when given without the help of any equipment ts called  Basic CPR (also called basic life support—BLS). When  equipment and drugs are used in CPR, it 1s called Advanced  CPR (advanced cardiac life support—ACLS). CPR must be  given promptly and in the correct manner to be effective. While    basic CPR alone may not be sufficient in resuscitating a victim  of cardiac arrest, it is this intial response that keeps the patient  alive till advance help can be provided. Success of resuscitation  as well as health-related quality of life depends largely on the  promptness and quality of basic CPR. Hence, knowledge of  CPR is mandatory for all medical and paramedical personnel.   ¢ The best of CPR can deliver only one-third of the normal  cardiac output. Hence, it is important to aim for return of  spontaneous circulation as early as possible.    The patient stops breathing due to a primary respiratory cause.  The heart continues to beat till all the oxygen in the lungs ts  removed. The heart will stop once hypoxia occurs. This may  take 1-2 minutes to occur. If the respiration is assisted  promptly, it 1s possible to avert cardiac arrest. However, this  is possible only in a witnessed respiratory arrest.    The heart stops beating and there is no circulation. Oxygen  delivery to the tissues stops. Within 15 seconds of a cardiac  arrest, a person loses consciousness. The brain stops  functioning within 3 minutes. This is called survival time. If  resuscitation 1s not done in another five minutes, brain death  occurs. This is called revival time. Many factors influence  these survival and revival times. The important ‘take home’  message 1s that prompt and effective treatment is necessary  for successful resuscitation.    The sequence of steps of basic CPR has been changed in the  2010 guidelines of American Heart Association (AHA).  Approach to CPR 1s currently done in the following order: C,  A and B. C for cardiac compressions, A for airway and B for  breathing.    In cardiac arrest, cerebral circulation comes to a standstill and  the person becomes unconscious within 15—20 seconds. Thus,  a person in cardiac arrest will not be conscious. Shortly  thereafter, the victim of cardiac arrest will also stop breathing.  On shaking gently and calling out loudly, if there is no response,  assume unconsciousness. Turn the patient in to a supine    position. Take care to turn the head, neck and body together in  a trauma victim who may possibly have a cervical spine injury.  Observe for any sign of breathing such as chest movement. If  there is no breathing, it confirms that the patient needs  assistance.    The chances of resuscitation are lower with basic CPR and it  is important to get help (in the form of ambulance services for  advanced CPR) as early as possible. It is also difficult for a  single rescuer to administer CPR for long periods. The rescuer  may take up to a minute to call for help. Since the majority of  adult, nontraumatic cardiac arrests are due to ventricular  fibrillation or pulseless ventricular tachycardia (shockable  rhythms), a defibrillator, either manual or automated, must be  obtained as quickly as possible.    Check for pulse or other signs of circulation such as coughing,  movement in response to the rescue breaths. When circulation  is inadequate, the body tries to conserve circulation to the vital  organs as long as possible. Thus, the carotid artery pulsations  are the last to disappear when cardiac output gradually reduces.  Hence, it 1s important to feel the carotid pulse to identify cardiac  arrest (Fig 48.31). Infants and neonates have short necks and  it is difficult to feel their carotid arteries. In them, the brachial  artery may be used to identify cardiac arrest. If no pulse 1s  felt, the patient 1s in cardiac arrest.    Compressions should be given with the heel of the hand placed  on the lower half of the sternum. Care should be taken not to  compress on either side of the sternum as it may causerib fractures.    Place the heel of one hand over the lower half of the sternum  in the midline. Clasp this hand with the heel of the other hand,  keep the elbows straight and move forward so as to place the    shoulders directly above the sternum. The rescuer can avo!  fatigue during compressions by allowing his or her body weig!  to be transmitted to the chest to create the required depth «  compression rather than use muscle power. Thus, th  movement occurs at the rescuer’s hips and not at the elbow  Only one hand may be used for compression in children les  than 8 years of age.    Rate of compression: Provide 30 compressions at a rate of a  least 100/minute.  Depth of compression: 2 inches (5 cm) in an adult.   Chest compressions are the most important part of basic  CPR. Hence they should be initiated as soon as cardiac arrest  is recognised. ‘Push hard, push fast’ is what the AHA  recommends. There should be minimal interruption in chest  compressions for any other interventions such as defibrillation,  airway management or even calling for help. The patient should  be lying on a relatively hard surface. If not a hard board may  be placed underneath the patients chest so that the  compressions become effective.    When a person loses consciousness, the muscles of the tongue,  epiglottis and pharynx relax. The tongue and the epiglottis  fall back and obstruct the glottis. Unless the airway is opened,  it is not possible to check accurately whether respiration is  present or not and if present, whether it is adequate or  inadequate. The airway is opened using a “head-tilt and chin  lift method” (Fig 48.33).   The palm of one hand ts placed on the forehead of the patient  and the head tilted posteriorly to extend the head as much as  possible. The chin of the patient is lifted up manually with the  thumb and fingers of the other hand so that the lower incisors  of the victim override the upper incisors. Since the tongue and    the epiglottis are attached to the mandible, chin lift also lifts  the tongue and the epiglottis.    The rescuer opens his/her mouth wide, makes a mouth to mouth  seal and delivers a large breath into the victim so as to expand  the chest adequately. He/she takes a breath in from the  atmosphere and gives another breath to the victim. Take two  seconds to deliver each breath.    Provide cycles of 30 compressions and 2 breaths (5 cycles  should be complete in two minutes). Continue cycles of 30  compressions and two breaths until help arrives or if the patient  shows some sign of life such as breathing or movement.    If the second rescuer arrives (with an AED), he proceeds to  switch on the AED, attach the defibrillator pads to the bare  chest of the patient, placing one pad in the right infraclavicular  region and the other on the precordium. He allows the AED to  analyse the cardiac activity for a shockable rhythm. During  analysis, the rescuers should not touch the patient. If the AED  confirms the presence of a shockable rhythm, it automatically  charges the pads and requests the user to press the shock button  so that the shock can be delivered to the patient. The rescuer  ensures that nobody 1s touching the patient or the bed, including  himself, announces loudly and delivers the shock. The second  rescuer takes over chest compressions whereas the first rescuer  (who may be tired by then) would take care of airway and  breathing. The two rescuers give chest compressions and  ventilations at a ratio of 30 : 2 (Fig 48.34).   Rescuer fatigue can affect the quality of CPR. After five  cycles of CPR (2 min), the rescuer must switch roles to provide  compression and ventilation. The heart rhythm is analyse every  two min to see whether there 1s a return of spontaneous cardiac  activity. If the AED declares that there is no shockable rhythm,  carotid pulse should be felt. If a palpable pulse 1s present, stop  chest compressions and continue rescue breathing as necessary    (1 breath every 5 seconds). If breathing also returns and is  adequate, the patient may be allowed to breathe spontaneously.  The victim may be turned to a lateral position (recovery  position) and monitored. The victim must then be shifted to a  hospital for further evaluation and care.   BLS is usually continued till help arrives and advanced life  support measures can be attempted. However, if no help is at  hand, and if the rescuer is unable to continue due to fatigue or  if the victim is obviously dead, CPR may be stopped.    Advanced CPR involves using airway adjuncts, other  equipment and drugs for resuscitation.    Bag-mask ventilation (Fig 48.35), if effective in achieving  chest expansion 1s adequate in the initial phase of CPR. Airway  is best maintained with endotracheal intubation. However,  chest compressions should not be interrupted to facilitate  endotracheal intubation. If it can be achieved without  interrupting chest compressions, then one may proceed to  secure the airway with an endotracheal tube. The route of  choice for intubation is through the mouth because it is faster  and less traumatic.   In case endotracheal intubation is difficult either due to  difficult airway or inexperience in the technique, oxygenation  and ventilation can be achieved by the use of an oropharyngeal    or nasopharyngeal airway along with a self-inflating bag and  face mask. Usually this is adequate but occasionally a laryngeal  mask airway or a Combitube® along with a self-inflating bag  may also be used.    After the airway is secured, ventilation must be provided at a  rate of 8-10 breaths per minute using a self-inflating bag  attached to the endotracheal tube. Oxygen must be attached  to the bag to improve oxygenation. These breaths need not be  interposed between cycles of 30 compressions. Once the airway  is secured with an endotracheal tube, chest compressions are  given uninterrupted at a rate of at least 100/min while breaths  are given every 6-8 seconds. It 1s important not to  hyperventilate during CPR as it can produce constriction of  cerebral arteries, reduce cerebral blood flow and adversely  affect outcome.   A capnograph may be attached to the endotracheal tube to  monitor end-tidal carbon dioxide. A capnograph has three  uses in CPR: |) To confirm the placement of endotracheal  tube 2) To monitor the effectiveness of CPR. If the end-tidal  carbon dioxide concentration is >10 mm Hg, the cardiac  compressions can be deemed effective and 3) A sudden increase  in end-tidal carbon dioxide to normal or near normal levels  heralds a return of spontaneous circulation (ROSC). Thus,  when an endotracheal tube is in place, efforts must be made to  obtain and use a capnograph, whenever possible.    Continue cardiac compressions as in BLS. Obtain peripheral  intravenous access quickly.    Cardiac arrest rhythms are one of the following three types:  Ventricular fibrillation/Ventricular tachycardia (VF/VT),  pulseless electrical activity (PEA) and asystole. A monitor is  obtained as early as possible in CPR to diagnose these rhythms.    Most of the adult nontraumatic cardiac arrests are due t  ventricular fibrillation (VF)/ventricular tachycardia (VT). |  untreated, VF/VT will degenerate into asystole. The onl  treatment of ventricular fibrillation is electrical defibrillatior  The success rate of defibrillation reduces by 10% every passin.  minute. Hence, defibrillator must be obtained as early a  possible (Fig 48.36).    If the rhythm shows ventricular fibrillation (VF) o1  ventricular tachycardia (VT) (Figs 48.37 and 48.38), the  following algorithm must be followed:  ¢ Provide Basic CPR. Once VF/VT is confirmed, interrupt   CPR briefly to deliver a single shock of 360 J (monophasic)   for another two minutes (5 cycles of compressions and  ventilations) before reanalyzing the rhythm.   Fig. 48.37: ECG rhythm showing ventricular fibrillation. Note  the saw tooth shaped waves that are irregular and chaotic in  pattern   Fig. 48.38: ECG rhythm showing ventricular tachycardia which  is more regular and shows wide QRS complex with inverted  T waves    ¢ If VF/VT persists, deliver another single shock of 360 J  (monophasic) or 200 J (biphasic) and resume CPR.  Administer adrenaline | mg or vasopressin 40 IU (one  dose only). Reassess after two minutes.   ¢ If VF/VT persists, deliver another single shock of 360 J  (monophasic) or 200 J (biphasic) and resume CPR. An  antiarrhythmic drug such as Amiodarone 300 mg may be  given IV.   ¢ Thus, analysis of the rhythm is repeated every two min and  if a shockable rhythm is present, shock is given. Epinephrine  1 mg is repeated every 3-5 min. A second dose of  amiodarone 150 mg may be considered for persistent VF/  VT. Always remember to look for treatable causes (Key  Box 48.6) of arrest.    If the monitor showed no VF/VT, but a discernible rhythm,  sinus or nonsinus with the patient manifesting no pulse (no  carotid pulsations), it 1s termed pulseless electrical activity  (PEA). This means that electrical activity is present while  mechanical activity is absent.   While PEA may be caused by massive myocardial infarction  where the myocardial necrosis 1s so severe that ventricular  contractions are absent (electromechanical dissociation -  EMD), it may also be caused by the following factors which  may result in ineffective contractions while electrical activity  is still present (pseudo-EMD). In pseudo-EMD, the causes  are reversible. If the cause is identified and treated promptly,  the chances of successful resuscitation are high.  |. Commence Basic CPR  2. This must be followed by adrenaline, | mg IV, every 3-5   is achieved.  3. Look for a treatable cause (Key Box 48.6) and treat promptly.    1. Hypoxia 1. Tension pneumothorax  2. Hydrogen ions (acidosis) 2. Tamponade (Cardiac)  3. Hypovolaemia 3. Thrombosis (Pulmonary,    Coronary)  4. Hyper/nypokalaemia 4. Toxins  5. Hypo/hyperthermia 5. Trauma  Remember 5 Hs, 5 Ts    If the monitor shows a flat line, it is asystole. Whenever  asystole is seen on a monitor the following things should be  done (flat line protocol). Confirm that it 1s asystole and then  commence therapy.    ¢ Check whether all leads are connected correctly. Improper  connections may manifest as asystole.   ¢ Change the Lead and check whether it is asystole in Lead I,  II and III. Occasionally, fine VF may be seen as asystole in  one lead but on changing the lead, VF may be seen. If VF is  seen, defibrillation must be attempted. There is no role for  defibrillation in asystole. In fact, it could make it worse.   ¢ Administer adrenaline, | mg IV, every 3—5 minutes as  necessary till return of spontaneous circulation is achieved.  Look for a treatable cause and treat.   ¢ If there 1s no response to resuscitation in spite of all the  above efforts even after a reasonable period of time (30-60  min) consider abandoning resuscitation efforts.    This is the drug of choice in cardiac arrest. It is given in a dose  of 1 mg, diluted to 10 cc (1:10,000) intravenously every 3-5  minutes: This should be followed by at least 20 cc of ‘chaser’  solution (0.9% saline or Ringer lactate) and limb elevation for  15-20 s. A peripheral intravenous access is adequate but if a  central Jine is in situ, the drug may be injected in the central  line. If intravenous access is not available, an interosseous  access may be secured (anteromedial aspect of tibia or lower  end of femur may be used). Alternately, adrenaline may be  injected through the endotracheal tube but higher doses need  to be used (2—5 mg diluted to 10 cc).   Intracardiac administration of adrenaline is not advisable  for the following reasons:   c. Accidental injection into the myocardium can precipitate  resistant ventricular fibrillation   d. Since the right ventricle is anterior, intracardiac injection is  usually into this chamber from which it has to traverse the  pulmonary circulation to reach the coronary arteries.   This may well be achieved by an intravenous injection,  running it in a drip and avoiding the complications of  intracardiac injection. Intracardiac injection is advisable only  when done under vision as during open heart surgery or  thoracotomy.    This is a potent vasoconstrictor that can be given as a single  dose of 40 IU and as an alternative to the first or the second  dose of adrenaline in a victim of cardiac arrest. The  vasoconstriction produced raises the diastolic pressure and  hence, the coronary perfusion pressure during CPR.    If VF/pulseless VT persists in spite of the adrenaline/  vasopressin followed by a DC shock of 360 J, amiodarone    may be given in a dose of 300 mg intravenously followed by  150 mg after five min, as necessary. Other drugs that may be  used are: Lignocaine: |—2 mg/kg intravenously or magnesium  sulphate: [—2 g intravenously. Not more than two anti-  arrhythmic drugs may be used to treat an arrhythmia as all  antiarrhythmics are also proarrhythmics and may also produce  myocardial depression.    An alkalinising agent is used in the following situations:  ¢ When there is documented metabolic acidosis   ¢ The arrest period is prolonged beyond 10 minutes.  The dose is 1—2 mmol/kg intravenously.   The dose is [0 ml of 10% calcium chloride or gluconate.  Thus, both calcium and sodium bicarbonate are indicated when  there is hyperkalaemic cardiac arrest. Their routine use in  cardiac arrest 1s not indicated as they can cause more harm.    If return of spontaneous circulation is achieved, it 1s important  to ensure it is sustained. Cardiac compressions are stopped  when the pulses begin to be felt without the compressions.  Intravenous fluids and/or an infusion of an inotrope such as  adrenaline or dopamine may be required to sustain normal  blood presasure. A blood sample may be taken to quickly  analyse the cause of cardiac arrest such as acidosis,  disturbances in potassium levels, poor oxygenation, and for  cardiac enzymes. A 12-lead electrocardiogram may be obtained  to rule out ST-elevation MI. If present, the patient will require  cardiology intervention such as coronary revascularisation. The  patient will need further care in the intensive care unit where  respiratory and renal support can be provided. It 1s important  to preserve cerebral function by maintaining normal blood  pressures, normal blood gases and pH, and normal cerebral  perfusion pressures. If the patient does not regain  consciousness after CPR and ROSC, consider induced  hypothermia by infusing one litre of cold saline (4° C) witha  target body temperature of 32 to 34°C. This is done in an  attempt to reduce cerebral metabolism so as to better preserve  brain function while the patient recovers from the after-effects  of cardiac arrest and CPR.    Thus, cardiopulmonary resuscitation should be aimed at  recovery of all functions of the body including the brain and  is more aptly called cardiopulmonary cerebral resuscitation  (CPCR).    Often cardiac arrest follows a period characterized by sor  abnormal rhythms such as symptomatic bradycardia (heart rai  < 50/min or symptomatic tachycardia (heart rate > 150/min  The symptoms are usually chest pain, dizziness and syncop:  palpitations or generally feeling weak. If these are identifie  and treated promptly, cardiac arrest may be averted. Oxyge  must be administered. Attach a monitor and secure a  intravenous access.    Symptomatic bradycardia may be due to sinus bradycardia c  heart blocks. Atropine (boluses of 0.5 mg IV, repeated to   maximum of 3 mg) is the drug of choice followed by a  infusion of adrenaline (2—10 mg/min) or dopamine (2—10 mg  kg/min). If the bradycardia 1s unresponsive to atropine  dopamine or adrenaline, a transcutaneous or transvenou  pacemaker may be required to pace the heart. Always look fo  and treat the cause.    Cardiac arrest may also be preceded by symptomati:  tachycardia (heart rate > 150/min). The symptoms are due t  inadequate circulation or the tachycardia itself such as ches  pain, dizziness and syncope, palpitations or generally feelin;  weak.    Symptomatic tachycardia may be due to sinus tachycardia  This may be due to causes such as pain, fever, sepsis, fear  hypovolaemia, etc. Identify and treat the cause.    ¢ If the patient is having symptomatic tachycardia, which 1:  not sinus in origin, measure blood pressure. If it 1s low  (<90/60 mm Hg) and the patient is unstable, proceed tc  synchronised cardioversion (shock levels will depend or  the rhythm — supraventricular tachycardia and monomorphic  ventricular tachycardia — 100 J, atrial fibrillation—200 .  and polymorphic ventricular tachycardia — 360 J — all shock  strengths given for monophasic defibrillator).    ¢ Ifthe patient is stable and has supraventricular tachycardia.  the options are vagal manoeuvres (carotid sinus massage  or Valsalva manoeuvre), adenosine (6 mg rapid IV push  followed by 12 mg x 2 doses as necessary) or simply rate  control with beta blockers or calcium channel blockers.    ° If the patient is stable and has broad QRS complex  tachycardia, the options are adenosine (6 mg rapid 1 V push  followed by 12 mg x 2 doses as necessary) to rule out  Supraventricular tachycardia with aberrant conduction. If  no response or ventricular tachycardia is suspected,  administer 150 mg of amiodarone, diluted in 5% dextrose  and given IV over |0 min. If no response, can consider  repeating the dose.    45-year-old doctor had a cardiac arrest due to myocardial  infarction. He was given effective CPR promptly. In   addition since the aetiology was ischaemic heart disease, he  was taken to cardiac catheterisation laboratory where his  myocardium was revascularised (PTCA). He received CPR  all through this procedure. His heart had stopped for 45  minutes but started beating once revascularisation was  complete. He required intensive care for cardiorespiratory  support initially and for complications such as renal failure  later. He went on to make a full recovery after a few weeks.    His recovery was so complete that he could present his own   ¢ Although survival rates are low, it is still possible to save  lives with a timely and well-performed CPR.   ¢ CPR is only a symptomatic therapy. Treating the cause  of arrest is equally important.   ¢ Good postresuscitation care is essential to ensure  recovery of all body systems.   ¢ Itis possible to recover good cognitive function with good  CPR. So, it is better termed cardiopulmonary cerebral  resuscitation (CPCR).    Students should aim to be able to quickly identify various  life-threatening and nonlife-threatening arrhythmias and  act accordingly. Students are urged to refer medical  textbooks for features of various nonlife-threatening  arrhythmias. However, always treat the patient and not  the monitor!    The outcome of cardiopulmonary resuscitation depends  on the time of commencement as well as quality of CPR.  The author feels that certification in basic as well as  advanced CPR must be made mandatory for all doctors.    Delivery of good quality CPR cannot be learnt from  books. The students are urged to get their certification  from a recognised training facility.    e Current CPR guidelines have been given with  emphasis on cardiac compressions.   e Importance of identification and treatment of  precardiac arrest rhythms has been added.    1. With the mouth wide open and tongue protruded in a  patient in sitting position, if only soft and hard palate    the view is said to be Cormack and Lehane:  A. |    3. The following period is adequate fasting before  administration of anaesthesia after taking a glass of    (ASA PS) 6 would be appropriate to describe the  following patient    S. The following drug is not helpful in reducing the effects    The following anaesthetic can be given by nasal,  intramuscular and intravenous routes for induction of  anaesthesia:  A. Thiopentone  C. Etomidate    The following intravenous anaesthetic is useful in status  asthmaticus for its bronchodilatory effect:   ANSWERS  C 2 iC 8 Cc 4 D 5 D 6  B 12 B 13 A 14 B 15 A 16    The following anaesthetic is described as an  antanalgesic:  A. Thiopentone  C. Etomidate    The compression-ventilation ratio in Basic CPR in an  adult is:  A. 30:2  Ch rl   PRINCIPLES OF TRANSPLANTATION  ° TypeS _ * Recepient evaluation  e Pathophysiology e Procedures e Procedures  e Major histocompatibility RENAL TRANSPLANTATION ¢ Complications    For end stage renal or liver disease, transplantation 1s the best  answer as of today. Over 25,000 transplantations are performed  annually for different conditions and more than 100,000  patients are awaiting an organ for kidney, liver or other organs.  The acceptance of an organ depends often on the beliefs and  decisions related to the country, race and religion. In certain  countries, living donor transplantation, even though risky, 1s  the only realistic organ donation method, greatly limiting the  potential for transplantation of organs from cadaver. Surgical  advances have permitted successful transplantation of lung,  pancreas and intestine from living donors. Because of the  limited supply; use of living donors may be the only means to  achieve timely kidney or liver transplantation as it 1s happening  in India. First let us study the principles and pathophysiology  of organ transplant.   but is pressed into action by the presence of suitable stimulus   ¢ Humoral immunity—mediated by antibodies which are  synthesised by B-lymphocytes (B cells). They offer  protection against extracellular microbes.   ¢ Cell mediated immunity—mediated by T-lymphocytes  (T cells). T cells may be CD4 T cells which indirectly  facilitate killing of microbes by macrophages or CD8 T cells  which directly kill microbes. CD8 T cells are also called  cytotoxic T cells.   (HLA) complex. It consists of a cluster of genes located on   1. Class | MHC molecules—which display peptides  synthesised within the cytoplasm of the respective cell. Class  1 MHC molecules are present in all cells.    2. Class 2 MHC molecules—which display antigens synthesised  outside the cell. Class 2 MHC molecules are mainly expressed  in antigen processing cells (APCs) or dendritic cells.   1. Direct recognition—the T cells recognise the foreign class |  MHC because of immunologic cross reaction. Subsequently  cytotoxic T cells are activated which kill the graft cells.   2. Indirect recognition—the host APCs present the antigens  in the graft (transplant) in class 2 MHC. This activates  T cells which secrete cytokines, induce inflammation and  damage the graft.    ¢ ABO blood group antigens are expressed in all cells and  are not just by RBCs. ABO incompatibility results in  hyperacute rejection of graft. Hence, it 1s recommended to  ensure ABO compatibility.   ¢ Better HLA matching improves transplant outcomes. This  is particularly relevant in live donor kidney transplant.  However, HLA matching is not done in heart, lung and liver  transplants as the urgency of transplant requirement  overweighs the benefits of HLA matching. Also in these  situations other factors such as size of the graft assume more  practical significance.    Based on the timing and mediators of rejection, graft rejection  may be classified as follows:  |. Hyperacute rejection  ¢ Occurs within minutes to days following transplant  ¢ Mediated by preformed antibodies  ¢ Untreatable but preventable  ¢ Cross-matching prevents hyperacute rejection  ¢ Methods of cross-matching include—lymphocytotoxic  assay, flow cytometric technique, bead-based screening  assays, panel-reactive antibody assay.  2. Acute rejection  ¢ Occurs in weeks to months following transplant  ¢ Mediated by T cells to a great extent and B cells to a  lesser extent.  ¢ Accordingly they are termed T cell-mediated rejection  (TCMR) or antibody mediated rejection (ABMR).  ¢ Immune suppression helps to overcome acute rejection  3. Chronic rejection  ¢ Most common cause of long-term allograft loss  ¢ Occurs over months to years following transplant  ¢ Involves both T cells and B cells  ¢ Examples for chronic graft reyection—ainterstitial fibrosis,  tubular atrophy and chronic allograft nephropathy in renal  transplant, vanishing bile duct syndrome in liver  transplant and bronchiolitis obliterans in lung transplant.   Liver transplantation is a lifesaving procedure for patients wh  have chronic end-stage liver disease and acute liver failur  (ALF) when there are no alternative treatment options. In 1963  Dr Thomas Starzl performed the first three human live  transplantations at the University of Colorado but patien  suffered from biliary atresia, had coagulopathy and did no  survive surgery. Introduction to cyclosporine for immuno  suppression in solid organ transplantation revolutionised thi  liver transplantation surgery.    1. Fulminant hepatic failure: It is acute onset of liver failure  in the absence of pre-existing liver disease. Here coagulo  pathy sets in within 8 weeks of onset of jaundice. Cause:  are acetaminophen overdose and hepatitis B infection 11  Asia. If left untreated, patients most often succumb to com:  due to cerebral oedema.   2. Hepatitis C: Chronic hepatitis C 1s the commones  indication for liver transplant in the West. However, hepatiti:  C recurs even after liver transplantation because the viru:  persists in the extrahepatic tissues. Pre-transplant and post-  transplant treatment with interferon and ribavirin car  potentially improve the outcome in these patients.   9. Hepatocellular carcinoma: Theoretically liver transplant  should offer the best chance of cure in HCC but there 1s  always the risk of recurrence of HCC following transplant.  Many transplant centres follow “Milan criteria” to decide  the feasibility of liver transplant in HCC, which says that  liver transplant can be considered in HCC when—there 1s  a single nodule of < 5 cm or there are fewer than 3 nodules.  the largest of which 1s measuring < 3 cm. Those who follow  the “Milan criteria” believe that the recurrence of HCC ts  very low if the criteria are strictly adhered to.   3. Inability to abstain from alcohol when liver transplantation  is being contemplated for alcoholic liver disease.   of such donors. The ideal donor should be young and otherwise   ¢ Older donors: If liver from older donors are used for  hepatitis C recipients, there is an increased risk of  development of cirrhosis in the transplanted liver.   ¢ Donor after cardiac death: These are donors who do not  meet brain death criteria but become donors once life  support is taken off. Liver from such donors can be  considered for transplant with cut off time of 30 minutes.    Candidates needing liver transplant far outnumber the available  donors. Therefore candidates needing liver transplant are listed  in the transplant register in organ transplant programmes. The  order in which candidates are placed in the waiting list for  liver transplant is determined by MELD formula. MELD  formula incorporates three components, namely-—creatinine,  bilirubin and INR. These components provide an objective  assessment of severity of liver disease.  MELD score =    In the waiting list, candidates with higher MELD score come  first. MELD score 1s nowadays preferred to Child-Pugh’s score  for being more objective.    1. Conventional liver transplant: This is performed to  replace the diseased liver with a healthy liver from a  deceased donor.   2. Expanded criteria donor (ECD): A diseased donor over  the age of 60 with mild liver abnormalities. The term  ‘‘expanded” is used because an expansion of the donor pool  is considered to increase transplantation. With an ECD liver,  the waiting time may be shorter—not done in India.   3. Living donor liver transplantation (LDLT): A procedure  in which a healthy, living person donates a portion of his or  her liver to another person. Finding a living donor match  shortens waiting time, increases long-term transplant  success and gives the flexibility of scheduling the date of  surgery.   4. Split liver transplant: A deceased donor liver is split into  two functioning units, which are used for transplantation to  a child (left lobe) and an adult (right lobe).    5. Combined organ transplant: A person may receive more  than one organ during the same transplant procedure such  as liver and kidney or liver and heart. This may be  recommended for patients who are experiencing multiple  organ failure.    More often the donor in liver transplant is deceased. However,  with severe shortage of donors, live donor liver transplants  are becoming common. The donor liver transplant is placed in  its native position in the abdomen of recipient after removing  the diseased liver. Hence, the term “Orthotopic liver transplant”  is used. (This may be compared with kidney transplant where  the transplanted kidney is placed in the iliac fossa thereby  deriving the term heterotopic transplant.) Sometimes, the donor  liver is split and the left liver is used for a paediatric recipient  and the right liver is used for an adult recipient. Such a liver  transplant is termed “Split liver transplant”. A few operative  steps in the back table where the donor live is prepared for  transplantation) is given in Key Box 49.1.   2. Preparation of cuffs of the suprahepatic and infrahepatic vena  cava, Cleaning of the portal vein and artery, and inspection  of the bile duct.   3. Verification of secure ligatures on small retrohepatic caval,  portal vein and hepatic arterial branches.   4. Confirm the continuity and integrity of all major structures  that must be anastomosed to the companion recipient  structures.   Figs 49.1 and 49.2: Cirrhotic liver which was removed (Courtesy:  Dr Sachidananda N, hepatobiliary and liver transplant surgeon,  Prof of Surgery, Akash Institute of medical Sciences and  Research, Devanahalli, Bangalore)   ¢ Rule out: Diabetes, hypertension, hepatitis B infectior  hepatitis C infection, HIV.    Recipient contraindication to renal transplantation is if  the recipient is suffering from diseases such as heart  disease or malignancy (advanced), which compromises  his survival.    Hepatocyte transplantation: In chronic liver diseases due to  specific enzyme deficiencies like Crigler-Najjar syndrome,  hepatocytes or stem cell transplant seems logical. With only a  few cells needed to restore functioning of liver, this concept  makes sense as it avoids the morbidity and mortality of a major  surgical procedure.    Recipient is prepared by haemodialysis. It also enhances the  chances of success of a transplant.   6. No systemic infections such as hepatitis B, C, HIV infection.    Living related donor: Donor nephrectomy wit]  preservation of as much length ofthe artery, vein and ureter    perfusion fluid and removed along with vena cava and aorta  and packed in plastic bags surrounded by ice in an insulatec  box. It can be stored like this for 72 hours.    mmol/L) and high osmolarity (400 mOsm/kg). This is usec  during transplant anastomosis.    iliac fossa by anastomosing renal artery to internal iliac artery  and renal vein to external iliac vein. Ureter is implanted in the  bladder (Figs 49.3 to 49.5).    Figs 49.4 and 49.5: Renal artery and renal vein are ready to be  sutured to common iliac artery and vein during rena! transplantation    1. Immunosuppression: Cyclosporin A ts given alone or in  combination with low doses of azathioprine and steroids.    2. Fluid balance: Aim to maintain urinary output at the rate  of 20 ml/hour. Even an anephric patient needs 500 ml of  fluid/day to replace insensible loss. CVP is ideal for  management of fluids in the postoperative period.    3. Postoperative oliguria: Monitoring of renal function—  Serum creatinine should drop by 50% in 48 hours post-  transplant period.    a. Acute tubular necrosis (ATN): Minimal ATN is common  due to short ischaemic time. Hence, oliguria phase may be  a few hours.    b. Rejection: More likely on the Sth day. Twice weekly DTPA  (diethyl! triamine penta-acetic acid) isotope renography and  gamma camera scan of the kidney confirms perfusion of  the kidney when oliguria 1s present. Percutaneous needle  biopsy can confirm the rejection.    1. Acute rejection: Within 3 months, this responds to high  doses of intravenous steroids. 90 mg methylprednisolone  IV/day x 3 days.    2. Chronic rejection involves the vascular element. It does no  respond to steroids.    1. Haemorrhage manifests as oliguria, hypotension. Wound  should be re-ex plored and bleeding vessels should be ligated.   2. Renal artery thrombosis results in nonfunctioning of the  transplanted kidney. It can be diagnosed by ultrasonogram  and isotope studies. This is treated by nephrectomy.   3. Renal artery stenosis can develop later. It should be treated  by an angioplasty.   4. Lymphocoele can develop due to perivascular dissection  and increased flow of lymph. Such lymphocoele can lead  to ureteric obstruction. Small collection needs to be  observed, large ones need drainage.   5. Ureteric obstruction or urinary leakage due to disruption  of ureteroneocystostomy can be due to ischaemia or necrosis  of the distal ureter. During donor nephrocystostomy, care  should be taken not to remove too much of periureteric  tissues and avoid too much dissection in the renal hilum to  prevent ureteric ischaemia.   ¢ Small bowel is rich in lymphoid tissue and the large mucosal  surface area with major histocompatibility antigens. This  definitely evokes a fight between graft and host.   ¢ Small bowel is colonised by a multitude of bacteria and  other micro-organisms. This is one of the reasons for failure.    ¢ The indication for intestinal transplant is intestinal failure  which means inability to maintain protein-energy, fluid,  electrolyte, or micronutrient balance. Most commonly it 1s  due to the result of extensive small bowel resections  following mesenteric ischaemia or following necrotising  enterocolitis.   ¢ In children: Children with massive intestine resection,  children with severely diseased intestine and unacceptable  morbidity, microvillus inclusion disease or intestinal  epithelial dysplasia.    1. In individuals who have significant coexistent medical  conditions thathave no potential for improvement following  transplantation.   1. Isolated transplantation of the small intestine (with or  without the portion of the right colon with the ileocaecal  valve).   2. Combined transplantation of the intestine and liver as  separate grafts.   3. Multivisceral transplantation (MVT), which 1s the simulta-  neous transplantation of a composite graft including the  liver, stomach, duodenum, pancreas, and small intestine.    1. Isolated transplantation of the small intestine: A few  steps are given below  ¢ A small bowel transplant from a deceased donor  comprises the entire small bowel.  ¢ The superior mesenteric artery of the graft (with an aortic  patch) 1s anastomosed to the recipient aorta, and the  superior mesenteric vein is anastomosed to the inferior  vena cava or to the side of the portal vein.  ¢ The proximal end of the small bowel graft is anastomosed  to the recipient jeyunum or duodenum.  ¢ The distal end of the graft is anastomosed to the side of  the colon.  ¢ Protective loop ileostomy is done. It 1s an end-ileostomy.  ¢ A gastrostomy tube is used for aspiration rather than to  overcome delayed gastric emptying.  ¢ Feeding jeyunostomy tube is inserted.  2. Combined transplantation of the intestine and liver  ¢ Itis indicated when patients have cholestatic jaundice also  secondary to TPN and require combined liver and small  bowel transplantation.  ¢ When combined liver and small bowel transplantation is  carried out, the two grafts are transplanted en bloc.  ¢ The donor aorta is fashioned into a conduit including the  Superior mesenteric and coeliac arteries and anastomosed  to the recipient aorta.  ¢ The portal vein anastomosis is similar to that in liver  transplantation.  3. Multivisceral transplantation (MVT)  ¢ Multivisceral, also called ‘cluster’ transplants may be  necessary in the case of large desmoid tumours.    ¢ Induction of immunosuppression with monoclonal  (alemtuzumab, basiliximab, daclizumab) or polyclonal  (thymoglobulin) antibody given intraoperatively or  preoperatively.    ¢ Immediately following surgery, maintenance immuno  Suppression therapy is initiated by oral administration o  tacrolimus. Steroids are also included in the postoperativ:  Immunosuppressive regimen.    A. Rejection  a. Acute cellular rejection: Acute cellularrejyection usualh  occurs within the first year post-transplantation but cai  occur at any time. Clinically, 1t can present as diarrhoea  unexplained fever, abdominal pain and/or cramping.    b. Chronic rejection: Clinically, these patients may have  chronic diarrhoea despite adequate treatment. Patient:  have an obliterative arteriopathy.    B. Infection  1. Bacterial: Bacterial infections can manifest as intra:  abdominal infection, opportunistic infections, surgica  site infections, pneumonia. Pathogenic organisms are  Escherichia coli, Klebsiella, Enterobacter, Enterococci  and commonly, polymicrobial infections.    2. Viral: Cytomegalovirus (CMV) 1s a common pathoger  post-intestinal transplantation, which often affects the  allograft. Treatment with ganciclovir and/or CMV  immunoglobulin and rarely, reduction in immuno-  suppression are methods that are used to minimise graft  loss.    3. EBV (Epstein-Barr virus) also presents a unique  challenge to intestinal] transplant recipients because o1  the higher rates of post-transplantation lympho-  proliferative disorder (PTLD) when compared with othe  solid organ transplant recipients.    C. GVHD: Graft-versus-host disease (GVHD) occurs when  donor lymphoid cells begin to target recipient tissues, most  notably the epithelial cells in the skin and intestine. It is  relatively uncommon.    D. Other complications: Postoperative haemorrhage, biliary  (if liver is also used) thrombosis of vessels resulting 1n graft  loss, bowel anastomosis leak and wound infections.    ¢ Type | diabetes mellitus, also known as juvenile-onset or  insulin-dependent diabetes, is a chronic polygenic  autoimmune disorder that has a strong hereditary basis inthe  human leucocyte antigen system. It results from destruction  of pancreatic beta cells inthe islets of Langerhans. Beta cells  constitute 28-75% of pancreatic islets.   e Percutaneous islet cell transplantation is a minimally  invasive cellular replacement therapy. It avoids risk of  hypoglycaemia which is one of the major problems of  exogenous insulin.    ¢ Type | Diabetes mellitus (DM) with disease presence for  at least 5 years, absence of endogenous C-peptide secretion.   e Islet cell transplantation may be performed alone, in  combination with renal transplantation, or following kidney  transplantation. Example: Diabetic patients with imminent  or established end-stage renal disease.    e Age less than 18 years or greater than 70 years   ¢ Active infection (including hepatitis C, hepatitis B, HIV  and tuberculosis).    e After cold perfusion of the abdominal organs, the pancreas,  spleen and duodenum is removed en b/oc. The portal vein  is transected at the duodenal border and the common bile  duct is transected close to the pancreatic border.   e The organ is packed in a triple-barrier bag with cold  preservation solution and stored at 4°C for transportation.  The maximum cold ischaemia time 1s 12 hours.    e Obtaining islet cells: The initial step is infusion of ae  Main and accessory ducts are identified,  collagenase through the main pancreatic duct; the clamped, and divided    includes five or six stainless steel balls, which provide fe eenase to distend pai   and the cells are washed and placed in culture media for  ]2—72 hours in an incubator at 22°-37°C. The isolated islets 7  are then transplanted.   ¢ With ultrasound (US) and/or fluoroscopic guidance, a °* An islet equivalent refers to an islet measuring at least  branch of portal vein is punctured with a 20—22-gauge 150 um in diameter. In general, harvested islet cells are  needle via a percutaneous transhepatic approach. infused by using gravity flow or direct syringe injection.    e The percutaneous access Is dilated to accept a 5S—6-F catheter  or vascular sheath, which is advanced into the main portal Peri- and post-procedural imaging,  vein. DM management, and immunosuppression  ¢ Confirm the catheter placement using ultrasound. It includes a combination of a T cell-depleting agent and  ¢ Systemic anticoagulation with heparin should be initiated- interleukin-2 monoclonal antibody receptor blocker  5000 units is routinely administered. (daclizumab) for induction and maintenance with a    combination of a calcineurin inhibitor (tacrolimus) and a  mammalian target of rapamycin inhibitor (sirolimus).    e Bleeding—either intraperitoneal or liver subcapsular is the  most common procedure-related complication. Fortunately,  effective hepatic parenchymal tract embolisation signifi-  cantly reduces bleeding.   e Partial portal vein thrombosis is another complication. It is  because of activation of coagulation system by transplanted  cells.   e Other complications of islet cell transplantation include  transient liver enzyme elevation, abdominal pain, focal  hepatic steatosis and severe hypoglycaemia.    Antiproliferating agents: Azathioprine is the drug used. |  inhibits both humoral and cell mediated immunity. Bon  marrow suppression and toxic hepatitis are side effects.    T cell directed immunosuppressants: Cyclosporine, Tacro  limus and Sirolimus are a few drugs. Cyclosporine inhibit  formation of mature CD4 and CD8 T cells in the thymus. |  does not cause myelosuppression. Side effects are nephro  toxicity, hypertension, hyperkalaemia, hirsutism, etc. It 1s usec  in a dose of 4 mg/kg in 500 ml saline, I'V and later-oral therap:  withl2 mg/kg daily. After a few weeks tapered to 5 mg/kg  day.    Tacrolimus: Used tn liver transplant and in acute rejection o  the kidney. Effects are similar to cyclosporine. Side effect:  are also similar to cyclosporin but without hirsutism anc  gingival hypertrophy.    1. Which of the following is not a component of innate  immunity?  A. Phagocytic neutrophils  B. Natural killer cells  C. Complements  D. Mast cells    2. Which of the following is a cytotoxic T cell?  A. CD8 B. CD4  C. CD6 D. CD2    3. Which are the following are included under MELD  score in liver transplantation?  A. Creatinine, bilirubin, albumin  B. Creatinine, bilirubin, INR    4. Tacrolimus is a:  A. Calcineurin inhibitor  B. Steroids  C. Antiproliferative agents  D. Antibacterial agent    One of the sessions in the undergraduate and postgraduate  examinations Is on X-rays and images. More and more images  such as ultrasound, CT scan, MRI and PET scan, etc. have  been used. Hence | have added a few of them here. More details  have been given in the chapter on radiology. Several CT images  have been included in the book. Students are requested to study  those CT scans and chapter on radiology first and then read  this chapter. This 1s only an exercise for you to perform better  in the final examination.    |. PLAIN X-RAY ABDOMEN SHOWING COLLECTION  OF FREE GAS UNDER THE RIGHT DOME OF THE  DIAPHRAGM    Normally, fundic air bubble is present on the left side. Hence,  importance is given to the gas on the right side.  1. What are the causes of free gas under the right dome of  the diaphragm?  ¢ Perforation of hollow viscus. Examples:  — Duodenal ulcer, gastric ulcer  — Perforation of enteric ulcer  — Perforation of Meckel’s diverticulum  — Malignant ulcers——colonic, gastric  — Perforation of the tuberculous ulcer—ileum  ¢ Abdominal stab injury  ¢ Laparotomy  ¢ Tubal insufflation test done for tubal patency.   Fig. 50.1: Plain X-ray abdomen showing collection of free gas  under the right dome of the diaphragm    ¢ Ground glass appearance indicates significant fluid in the  peritoneal cavity.    3. How do you manage a case of perforated duodenal ulcer?    * With antibiotic coverage, Ryle’s tube aspiration and early  resuscitation with intravenous fluids, exploratory  laparotomy is done. The site of perforation is identified  which is in the first part of the duodenum. The perforation  is closed by using nonabsorbable sutures. Omentum can be  used to reinforce the suture line. This is called Roscoe  Graham operation. Corrugated red rubber drain or tube drain  is used to drain peritoneal cavity.    4. Will you do elective surgery such as GJ and vagotomy  or HSV at this stage?    e Since the general condition of the patient will be very poor  at this acute stage because of hypovolaemic and septic  shock, elective surgery is not done.    . How do you identify jejunum, ileum and colon in a plain    ¢ Resection and end-to-end anastomosis  5. Can any other surgical procedure be done?  ¢ Stricturoplasty (like pyloroplasty)   Fig. 50.2: Plain X-ray abdomen showing multiple gas and fluid  levels    ll. PLAIN X-RAY ABDOMEN SHOWING RADIO-OPAQUE  SHADOW IN THE RIGHT UPPER ABDOMEN   ¢ The location of the stone is at lower level when comparec  to gall stone.   ¢ The shape of the stone suggests that it 1s a stone in the  pelvis growing within calyces.   4. What type of X-ray is ideal to distinguish renal stone   5. What will be the findings in case of renal stones in a    ¢ Renal stones are found superimposed on vertebral bodies.  On the other hand, gall stones are found anterior to it.   Fig. 50.3: Plain X-ray abdomen showing radio-opaque shadow in  the right upper abdomen    lV. PLAIN X-RAY ABDOMEN SHOWING RADIO-  OPAQUE SHADOW IN THE REGION OF GALL  BLADDER    2. What percentage of gall stones are visible in a plain  X-ray?    Fig. 50.4: Plain X-ray abdomen showing radio-opaque shadow in  the region of gall bladder    4. What are the causes of radio-opaque shadow in the  abdomen?    V. X-RAY CHEST PA VIEW SHOWING MULTIPLE  ROUND SHADOW IN BOTH LUNG FIELDS   Fig. 50.5: X-ray chest PA view showing multiple round shadows in  both lung fields    VI. X-RAY CERVICAL VERTEBRAE WITH UPPEF  RIBS SHOWING BILATERAL CERVICAL RIBS    On the right side, it is complete variety and on the left side,  it is incomplete.    Extraperiosteal excision of cervical rib which means  removal oftherib along with the periosteum. Some surgeons  also do cervical sympathectomy to decrease vasomotor tone  of vessels.   Fig. 50.6: X-ray cervical vertebrae with upper ribs showing  bilateral cervical ribs    Vil. BARIUM SWALLOW SHOWING INTRINSIC,  IRREGULAR, AND PERSISTENT FILLING DEFECT IN  THE LOWER OESOPHAGUS    Fig. 50.7: Barium swallow showing intrinsic, irregular and persis-  tent filling defect in the lower oesophagus    Vill. BARIUM SWALLOW SHOWING EXTENSIVE AND  IRREGULAR FILLING DEFECT INVOLVING MIDDLE  ONE-THIRD OF OESOPHAGUS    Inoperable—to relieve dysphagia, metallic stents can be  P Pee defect involving middle one-third of oesophagus    . What are the premalignant conditions? 3. What will be the biopsy report?  Achalasia cardia ¢ Squamous cell carcinoma  Reflux oesophagitis 4. What other investigations are necessary in such case?  Corrosive stricture ¢ Bronchoscopy, CT scan of the chest and endosonography  Plummer-Vinson syndrome are important investigations.    5. Looking at this advanced lesion, what is probably the  best treatment for this patient?    ¢ Radiotherapy followed by dilatation of the oesophagus,  since chances of fibrosis and narrowing of the lumen  following radiotherapy are high.    IX. BARIUM MEAL SHOWING INTRINSIC,  IRREGULAR, AND PERSISTENT FILLING  DEFECT INVOLVING PYLORIC ANTRUM    Fig. 50.9: Barium meal showing intrinsic, irregular and persistent  Oesophagoscopy and biopsy filling defect involving pyloric antrum    4. What is the treatment, if it is operable?  ¢ Subtotal gastrectomy    ¢ Growth along with 60—70% of distal stomach, omentum,  enlarged regional nodes such as prepyloric, suprapyloric,  infrapyloric, left and right gastric nodes are removed,  followed by gastrojejunal anastomosis.    X. BARIUM MEAL X-RAY SHOWING ENORMOUS  DILATATION OF THE STOMACH AND FAILURE OF  BARIUM TO FILL INTO THE DISTAL INTESTINE   ¢ Gastric outlet obstruction due to chronic cicatrised duodenal  ulcer (pyloric stenosis 1s an old terminology).    ¢ With a preoperative stomach wash, adequate intravenous  fluids, total truncal vagotomy with GJ is the treatment of  choice.    ¢ After vagotomy, motility of the stomach ts lost and in pyloric  stenosis, there is already obstruction at the pyloric antrum.   Fig. 50.10: Barium meal X-ray showing enormous dilatation of  the stomach and failure of barium to fill into the distal intestine    Hence, gastrojejunostomy 1s the drainage procedure o  choice.  5. Why not pyloroplasty or highly selective vagotomy?    XI. BARIUM ENEMA SHOWING THE LEFT COLON.  TRANSVERSE COLON AND A PART OF ASCENDING  COLON   Fig. 50.11: Barium enema showing the left colon, transverse colon  and a part of ascending colon with pincer ending    XIl. BARIUM ENEMA SHOWING INTRINSIC,  IRREGULAR AND PERSISTENT FILLING DEFECT IN  THE ASCENDING COLON    ¢ Right radical hemicolectomy, if it is operable. Structures  removed in this operation include terminal 1leum (6—8 cm),    right transverse colon. If it is inoperable, part of ileum is  anastomosed to the transverse colon to prevent or relieve  intestinal obstruction (side to side). One need not remove  two feet of ileum.    ¢ Ileocaecal tuberculosis: In this condition:  |. Irregular filling defect is not seen.  2. Caecum 1s usually pulled up and then ileocaecal angle  becomes obtuse.   Fig. 50.12: Barium enema showing intrinsic, irregular and persis-  tent filling defect in the ascending colon    XIll. BARIUM ENEMA SHOWING LOSS OF  HAUSTRATIONS IN THE LEFT COLON, SMALL AND  MULTIPLE, REGULAR FILLING DEFECTS DUE TO  PSEUDOPOLYPOSIS   e An attempt at healing in between the ulcers produces  granulation tissue which have the appearance of polyps.  Hence, pseudopolyposis.   Fig. 50.13: Barium enema showing loss of haustrations in the left  colon, small and multiple, and regular filling defects due to  pseudopolyposis    4. What are the drugs used in the treatment of ulcerative  colitis?    5. What are the surgical treatments?  e Total colectomy with permanent ileostomy. OR    ¢ Total colectomy, creation of a pouch with anastomosis of  the pouch to the anal canal.    XIV. X-RAY LATERAL VIEW OF THE SKULL  SHOWING A LARGE SWELLING WITH EROSION IN  THE PERICRANIUM    1. What is the diagnosis?  ¢ Secondary deposit in the skull    e Erosion of the bone is seen in malignancy, not in benign  tumours.    3. If this patient is a female aged 40 years, what are the  causes?   Fig. 50.14: X-ray lateral view of the skull showing a large swelling  with erosion in the pericranium   ¢ Near total/total thyroidectomy followed by radio-1odine  therapy and external radiotherapy for metastasis 1n bones.    XV. ENDOSCOPIC RETROGRADE CHOLANGIO-  PANCREATOGRAPHY (ERCP) SHOWING THE  BILIARY AND PANCREATIC SYSTEMS    1. What is the diagnosis?  ¢ Chronic pancreatitis  2. Why do you say so?    Fig. 50.15: Endoscopic retrograde cholangiopancreatography  (ERCP) showing the biliary and pancreatic systems    5. If pancreatic duct is dilated more than 8 mm in a patient  with severe abdominal pain with chronic pancreatitis, what  is the treatment?    ¢ Longitudinal pancreaticojejunostomy—Puestow’s operation.  In this operation, pancreatic duct is laid open, strictures are  divided and the duct is anastomosed to jejunum.    XVI. T-TUBE CHOLANGIOGRAPHY SHOWING A  FILLING DEFECT IN THE LOWER END OF THE  COMMON BILE DUCT (CBD)    ¢ In case of distal obstruction by a residual stone, the bile  starts leaking from the suture line on the CBD and may  result in biliary peritonitis. In such situations, T-tube helps  in drainage of the bile.   5. How do you treat this patient in order to extract the  stone?   Fig. 50.16: T-tube cholangiography showing a filling defect in the  lower end of the common bile duct (CBD)    XVIl. SPLENOPORTOVENOGRAPHY (SPV)  SHOWING EXTENSIVE COLLATERALS IN THE  REGION OF SPLEEN   ¢ Splenic vein, portal vein and its branches within the liver  are visualised.   5. What is the first line of specific treatment for bleeding   ¢ Sclerotherapy can be perivariceal or intravariceal-injection  of 2% solution of sodium tetradecyl sulphate.   ¢ Sclerotherapy is given at multiple sites and in multiple  sittings.   Fig. 50.17: Splenoportovenography (SPV) showing extensive  collaterals in the region of spleen    XVIill. RETROGRADE ANGIOGRAPHY  SHOWING OCCLUSION OF FEMORAL  ARTERY ON THE LEFT SIDE   Fig. 50.18: Retrograde angiography showing occlusion of femoral  artery on the left side    ¢ By reducing the sympathetic tone of the lower limb,  arterioles and capillaries get dilated allowing cutaneous  ulcers to heal.    XIX. CONTRAST ENHANCED (CE) CT ABDOMEN  SHOWING MASS IN THE RIGHT ILIAC FOSSA    3. What is the name used to the picture in terms of number  of units?    ¢ Pregnancy is a contraindication. Iodine containing contrast  can give rise to nephropathy. Allergy to contrast can happen.  Hence, dehydration should be corrected. Serum creatinine  should be checked before contrast.   ¢ It is showing a hypodense lesion in the right iliac fossa   Fig. 50.19: A hypodense mass in the right iliac fossa involving  caecum   ¢ Anatomically it is a lesion occupying the right iliac fossa  involving caecum.   e It is a hypodense mass with solid and cystic areas. Cystic  areas represent tumour degeneration.    obliterated.  12. What is the importance of that?  ¢ Probably it 1s infiltrating the abdominal wall.  13. Why do you want to know this information? °    e At surgery, the involved portion of the abdominal wall has’ g    to be removed.  14. How do you confirm the diagnosis?  ¢ Colonoscopy and biopsy  15. What will be the report expected?  e In majority of the cases it is adenocarcinoma  16. What is the treatment if it is operable?  ¢ Right radical hemicolectomy  17. If it is inoperable, what is the treatment? °  e Palliative ileo-transverse anastomosis   PET-CT of a patient who was diagnosed to have carcinoma lung on bronchoscopy.  PET CT shows a hilar mass with a nodule anteriorly on left side of pleura.  It also shows pneumonic patch on tower zone of left tung which is FOG avid    Hilar mass with a nodule anteriorly on the left side of pleura    . If the report is adenocarcinoma lung, what is the next    To stage the disease by whole body bone scan, PET scan,  and CT scan.   1. Name this investigation. 7  ¢ PET-CT scan .  2. What is PET scan?    . What is the most commonly used positron emitting  radionuclide?  ¢ Fluoro-deoxyglucose (FDG)    Certain atomic nuclei, which possess unpaired protons or  neutrons, possess an inherent spin. The nucleus 1s positively  charged and therefore creates a small magnetic field around  itself, when it spins. The human body contains in abundance  such spinning nuclei in the atoms of hydrogen, which is  found in water and lipids.    What are the chief advantages of MRI over CT scan?  It is noninvasive and does not involve the use of ionising  radiation. Hence, it is safe.    5. What does this picture show?  ¢ A hyperintense mass occupying the thigh region.    Fig. 50.21: MRI of the thigh 9, What are common tumours in this location?  ¢ Malignant fibrous histiocytoma (MFH) and liposarcoma.    - The imaging time is long. Hence, movement of the patients 10. What is the treatment: |  may produce artefacts. ¢ Wide excision with 2-3 cm margin    Please note: X-rays discussed here are the common X-rays which are asked in the MBBS examination. However, modern investi-  gations such as mammogram, ultrasound, CT scan and MRI also may be asked. You are requested to read the chapters on  radiology and breast for more details about these investigations.   It is also called Spencer Well’s artery forceps. It has a  ratchet and two blades with uniform serrations. 
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