Anatomy The Bochdalek defect is most common (posterolateral). The Morgagni defect (anteriom edial) is most often a late presentation. Hernia of intra- abdominal viscera into chest \200\224commonly small and large intestine, also stomach, liver, spleen and occasionally ki dney also occurs, 80 \200\22390% left sided. Pulmonary hypertension is secondary to pulmona ry hypoplasia. Echocardiography aids diagnosis and can monitor treatment. Timing of surgery and adjuncts Delayed surgery now standard practice. It is not advisable t o proceed to repair until ventilatory parameters have improved. Newer treatments include in haled nitric oxide, surfactant, extracorporeal membrane oxy- genation and sildenafil. Gentl e ventilation with permissive hypercarbia is recommended. This includes high frequency osci llation. Position Supine. Incision Ipsilateral transverse upper quadrant or subcostal (1cm below margin). Procedure (cid:142)(cid:3) Open peritoneum and retract upper edge of wound to reveal anteri or rim of diaphragm. (cid:142)(cid:3) Gently deliver herniated contents from chest into wound. Carry out soft re traction of liver and gut to expose size of defect. Look into hemithorax to determine if sa c present. If sac is present, excise it. Iden- tify the posterior rim and mobilize it. (cid:142)(cid:3) Insert interrupted horizontal mattress sutures with 2/0 or 3/0 non- absorbable. Place each suture individually and tie after all in place. If no posterior rim and defect large, a patch (PTFE, Silicone) may be used. Cut patch to appropriate size and s uture in place with 2/0 or 3/0 non- absorbable sutures. Sutures may be inserted around ribs posteriorly. Chest drains are used uncommonly. (cid:142)(cid:3) Close the wound in layers with 3/0 absorbable and subcuticular 4/0 or 5/0 absorbable to skin. CONGENITAL DIAPHRAGMATIC HERNIA (CDH)1 595 Mortality Mortality of live born infants around 40%, is usually due to intractable pulmonar y hypertension. Some centers report improved survival with multidisciplinary directed appro ach. Complications (cid:142)(cid:3) Recurrence of hernia (cid:142)(cid:3) Chronic lung disease ( cid:142)(cid:3) Intestinal obstruction \200\224due to adhesions or volvulus (universal mesentery) (cid:142)(cid:3) Gastro-oesophageal reflux. Diaphragmatic eventeration The diaphragm is present but muscle atrophied. Presentation is s imilar to CDH although it is often less severe. Radiograph shows pronounced smooth dome of elevated diaphragm and ultrasound scanning shows paradoxical movement of diaphragm (upwards on inspiration). Treatment is by plication of diaphragm through abdominal incision or thor acotomy. Left lung Right lung Bowel loops Heart Diaphragm defect Liver Fig. 14.6 Diaphragmatic hernia CHAPTER 14 Pediatric surgery 596 Spina bifida There is defective neural tube formation with exposed neural tissue at the are a of failed posterior vertebral arch development often in the lumbosacral spine. It is ofte n prenatally detected by raised maternal alphafetoprotein levels and anomalous ultrasound s can. Associated con- ditions are hydrocephalus (cognitive dysfunction), neurogenic bladder and bowel (incontinence) and lower limb paresis/paralysis and sensory losses. The back lesi on may be \200\230closed \200\231 (meningocele) or \200\230open \200\231, leaking CSF (my elomeningocele). Open lesions expose the newborn to risks of further neurological damage an d ascending CNS infection and warrant early operation to mobilize and close the meningeal m embranes, fascia and skin over the exposed central nerve cord. Preoperative Antibiotic prophylaxis. Position Prone with support to upper chest and pelvis to avoid ventilatory prob- lems relat ed to abdominal pressure. Procedure (cid:142)(cid:3) Outline the exposed neural plaque and meninges by a skin incisio n. (cid:142)(cid:3) Tabularise the nerve cord, if possible, with very fine (5/0, 6/0) absor b- able continuous sutures to minimize CSF leak. Close the dura similarly with 5/0 or 6/0 a bsorbable. Close the fascial layer in a vertical line achieved by longitudinal incision in the erector spinae fascia which is reflected medially to the midline over the bifid spines. (cid:142)(cid:3) Close the skin with 3/0 absorbable in either vertical or transverse line w ith wide undermining to release tension \200\224occasionally rotation or lateral release fl aps are needed for a wide defect. Insert a suction drain, useful if extensive undermining o f skin. Complications (cid:142)(cid:3) Wound infection (cid:142)(cid:3) Skin necrosis/dehiscence du e to excessive tension (cid:142)(cid:3) Ascending CNS infection and meningitis (cid:142)(ci d:3) More rapid progression of hydrocephalus related to cessation of CSF leak. This page intentionally left blank CHAPTER 14 Pediatric surgery 598 Release of tongue tie A tight frenulum of the tongue can restrict movement to extent of int er- ference with feeding or speech \200\224rarely the case if tip protrudes beyond lower te eth. Anxiety about possible effects is a common reason for referral. Reassurance alone is o ften sufficient. Procedure Usually under general anesthesia but local possible as procedure takes seconds. ( cid:142)(cid:3) Tongue tip grasped with forceps and drawn upwards and forwards to expose ti ght border of frenulum \200\224divided with scissors to release tongue. Two leaves of frenu lum divided further posteriorly for greater advance and the frenular artery in midline avoi ded. (cid:142)(cid:3) This procedure is usually bloodless but significant bleeding requires suture (4/0 absorbable) to artery at base of frenulum. Complications (cid:142)(cid:3) Reactionary bleeding \200\224may demand re-exploration and s uture of artery (cid:142)(cid:3) Inadequate release and residual restriction. This page intentionally left blank CHAPTER 14 Pediatric surgery 600 Ranula Anatomy and presentation This is a smooth, bluish, cystic swelling arising in floor of mouth beneath tongue from sublingual gland. It may cause problem with feeding, inter- fe rence with speech development and cosmetic concern but there are rarely symptoms of pain/di scomfort or infection. Preoperative Nasal endotracheal intubation improves the surgeon \200\231s access to oral ca vity. Jaw opened with dental gag and hypopharynx/posterior airway packed to avoid potential aspiration. Procedure (cid:142)(cid:3) Holding suture in tongue tip may help control and access. (cid:1 42)(cid:3) Excise small, discrete, well-defined cysts completely with repair of mucosa with interrupted absorbable 4/0. (cid:142)(cid:3) Make no attempt to excise larger cysts as risk of damage to lingual nerve and submandibular duct. (cid:142)(cid:3) Excise a segment of anterior cyst wall with scissor dissection or elec- tr ocautery to open and drain cyst. Outer (mucosal) and inner (duct epithelial) linings of cys t then sutured together (with continuous absorbable 4/0) to control bleeding, prevent re-ad herence and produce a stoma in cyst wall (marsupialisation). Cyst then rapidly shrinks down and sublingual saliva drains via stoma. Complications (cid:142)(cid:3) Infection if oral hygiene poor and foreign material lodges i n cyst (cid:142)(cid:3) Bleeding from resected cyst wall (cid:142)(cid:3) Recurrence if mar supialisation inadequate. RANULA1 601 Support suture Tongue Ranula Marsupialised wall Fig. 14.7 Marsupialisation of ranula CHAPTER 14 Pediatric surgery 602 Preauricular skin tag and sinus Skin tag/appendages and sinuses arise in the pre-auricular position ante- rior to the tragus or on the helix as remnants of the first branchial arch a nd cleft. Skin tags are a cosmetic issue. Sinuses may discharge or be- come inflamed and ch ronically infected. Procedure: excision of skin appendage (cid:142)(cid:3) Make an elliptical skin incision in a vertical orientation around the base of the tag judging the dimensions required to produc e a flat skin sur- face without \200\230dog-ears \200\231 on closure. If present, dissect a central cartilage rudiment down to its origin and core it out. (cid:142)(cid:3) Close skin with interrupted 5/0 absorbable or Nylon sutures. Procedure: excision of sinus (cid:142)(cid:3) Remove a non-infected sinus by an elliptical incision around its orifice and dissection around the sinus to its base. The sinus tract ma y be defined using a fine lacrimal duct probe. It may extend as deeply as the temporo-mandi bular joint. (cid:142)(cid:3) Close the wound with 5/0 absorbable or Nylon sutures. An infected sinus ma y ramify in subcutaneous tissues and produce a complex abscess with overlying erythematous skin. (cid:142)(cid:3) Carry out wider exploration and excision by raising skin flaps using a Y incision with upper limbs anterior and posterior to the lower pole of the ear and the lower limb just behind the jaw line. Expose the parotid gland \200\224take care to preserve facial nerve and branches. Complications (cid:142)(cid:3) Residual/recurrent infection following incomplete sinus exci sion (cid:142)(cid:3) Facial nerve branch damage (cid:142)(cid:3) Temporo-mandibular joint discomfort and stiffness. This page intentionally left blank CHAPTER 14 Pediatric surgery 604 Branchial cyst/sinus This arises from a remnant of the second branchial arch/cleft. The cys t presents as a smooth swelling around midpoint of the line of anterior border of sternocle idomastoid muscle; the sinus may extend from tonsil- lar fossa of pharynx to skin surface. Differentiation from other cervical masses may need US or CT scanning. Usually asymptomatic but viscid mucus may discharge from a small skin punctum. Infection leads to ten- derness/ abscess formation and incision and drainage followed by delayed formal excision. Other arch remnants are very rare. First arch occasionally results in cyst/fistula at angle of jaw and around the external auditory meatus. Third and fourth arch remnants are at the level of thyroid. Position Supine with neck extended using a shoulder roll \200\224head rotated to con- trala teral side. Slight head up tilt decreases venous engorgement (too much tilt risks air embol us). Preoperative management IV antibiotics at induction if evidence of infection. Sinus/fistula is best defined by inserting a fine lacrimal probe and anchoring with skin suture for trac tion. Dye may be instilled (methylene blue) but any spill- age/leakage stains normal tissue s and obscures dissection. Incision Neck skin crease incision over swelling or skin ellipse around sinus orifice. Procedure (cid:142)(cid:3) Divide the platysma and superficial cervical fascia in the line of incision. Carry out dissection between branches of carotid artery avoiding vagus, hypoglossal and glo ssopharyngeal nerves. If there is close prox- imity to parotid, carry out careful separatio n. (cid:142)(cid:3) The tract/sinus may extend to pharyngeal wall and some times a sec- ond (higher) parallel incision is needed to follow dissection superiorly. It is important t o excise deep components completely to avoid recur- rence. (cid:142)(cid:3) Close the fascia with interrupted absorbable 3/0 sutures and subcuticu- la r absorbable 4/0 to skin. Insert a suction drain if dead space remains. BRANCHIAL CYST/SINUS1 605 Complications (cid:142)(cid:3) Local oedema/infection (cid:142)(cid:3) Recurrence due to in complete excision (cid:142)(cid:3) Nerve or vascular damage Cyst Sinus Fig. 14.8 Excision of branchial cyst/sinus CHAPTER 14 Pediatric surgery 606 Thyroglossal cyst and fistula Definition and anatomy The cyst is a firm, rounded, midline, anterior neck swelling in line of descent of thyroid gland \200\224usually above hyoid bone . It can occur lower in neck, on left of midline, or within the tongue. If in doubt about d iagno- sis, carry out thyroid scan (ultrasound or isotope). It moves up and down with tongue protrusion/retraction. Infection in cyst may lead to skin fistula and discharge of \200\230glairy \200\231 mucus. Position Supine with head midline and neck extended by roll beneath shoulders. Preparation Check with anesthetist that digital access to the airway is possible. A larynge al mask is not appropriate (distorts surgical anatomy). Give IV antibiotics at induction if infection suspected. Incision Transversely (collar) over hyoid incorporating skin ellipse if adherent to cyst. Procedure (Sistrunk \200\231s procedure) (cid:142)(cid:3) Dissect down and round central hy oid partly dividing strap muscle insertion (sternohyoid). Excise central hyoid (bone-cutter ) with attached cyst. Remove central segment of hyoglossus muscle including thyroglossal tr act to base of tongue (foramen caecum). Assistant \200\231s finger in floor of mouth pushes forward base of tongue to improve definition and access. (cid:142)(cid:3) Closure is helped by neutralizing neck extension. Obliterate dead space with interrupted absorbable 3/0. If there is residual dead space or infection suspect ed, leave thin rubber/Silastic tissue or suction drain. Close skin with subcuticular contin uous 4/0 monofilament sutures and tie-over dressing or subcuticular continuous absorbable 4 /0 sutures. Postoperative Overnight stay to monitor airway. Complications (cid:142)(cid:3) Swelling and potential upper airway obstruction if bleeding/ haematoma (cid:142)(cid:3) Wound infection/abscess if excision incomplete or cyst/tract inf ected (cid:142)(cid:3) Incomplete excision with recurrence (5 \200\22320 % depending on whe ther tract or cyst alone excised) THYROGLOSSAL CYST AND FISTULA1 607 Thyroglossal tract (to foramen caecum) Thyroglossal cyst (overlying hyoid) Thyroid cartilage Fig. 14.9 Approach for thyroglossal cyst and fistula CHAPTER 14 Pediatric surgery 608 Lymphangioma/cystic hygroma of neck Cystic hygroma may develop on either side of neck (post erior triangle) in relation to defective thoracic and right lymphatic duct drainage into th e venous system. It can reach a large size detectable on antenatal scans. Lymphangiomas ram ify extensively between and through normal neck structures as multiloculated, cystic, diffu se masses. They may lead to respiratory distress and feeding problems and can extend into f loor of mouth and base of skull. Occasionally a rapid size increase due to infec- tion or b leeding. US or MRI scan delineates the relation to anatomical structures. They can be manag ed conservatively or by sclerosant injec- tion but cosmetic problems or compression/obstruc tion may demand surgery. Position Neck extended over roll and rotated away from side of lesion. Procedure (cid:142)(cid:3) Make a transverse (collar) incision in skin crease. Divide the s uperficial fascia and platysma and expose the multiloculated cystic lesion. (cid:142)(cid:3) Carry out exploration with regard to displaced position of facial and hypo glossal nerves and with care to avoid vascular damage (thyroid vessels). Meticulous time-co nsuming exploration may allow safe removal of entire swelling but excision often limited or partial and restricted to debulking large hygromas. Insert a tissue vacuum drain to preven t lymph accumulation. (cid:142)(cid:3) Close the wound with 3/0 absorbable to platysma and fascia and 4/0 subcuticular absorbable or monofilament Nylon. Complications (cid:142)(cid:3) Haematoma and infection related to extensive dead space (cid :142)(cid:3) Cervical branch of facial nerve damage leading to droop of angle of mouth (cid:142)(cid:3) Hypoglossal nerve damage leading to asymmetric tongue movement and speech impairment (cid:142)(cid:3) Residual cosmetic problem due to irregular, puckered scar. This page intentionally left blank CHAPTER 14 Pediatric surgery 610 Dermoid cysts Dermoid cysts are subcutaneous inclusions of dermal tissue, usually at fusion lines, which lead to development of small, firm cysts containing sebaceous secretions. Unl ike epidermoid (sebaceous) cysts, there is no communication with the surface (punctum) and neither discharge nor infection occurs. Cysts are mobile with respect to both the skin and the deeper tissues. Typical sites are the outer end of eyebrow (external angular dermoid), midline of nose, suprasternal notch. Occasionally deeper (dumb-bell) extension occurs into cranial fossa or an encephalo- cele is associated (ethmoid). Diagnosis If there is any doubt about deep extension, CT scan is needed. Incision Make the incision in skin tension (Langer \200\231s) lines and, for an external an gular dermoid, in plane of hair shafts in eyebrow. Procedure (cid:142)(cid:3) Deepen the incision to reveal cyst capsule. Carry out sharp diss ection in plane around capsule to allow complete mobilization of cyst. Take care to avoid c apsule breach which a) loses definition of cyst, b) risks leaving capsule tissue from which cyst may regenerate, and c) releases potentially irritant sebaceous material. Fine blood v essels may need cautery but this is often a \200\230bloodless \200\231 procedure. (cid:142)(cid:3) Close the dead space closed with 3/0 or 4/0 absorbable suture and close skin with subcutaneous continuous 4/0 or 5/0 absorbable or with Nylon/Prolene continu ous 4/0 tie-over suture. Apply pressure dressing. Complications (cid:142)(cid:3) Haematoma/bleeding if dead space is left (cid:142)(cid:3) In fection (cid:142)(cid:3) Recurrence if any residual cyst capsule (cid:142)(cid:3) Risk of s erious intracranial sequelae (abscess) if deep extension left (cid:142)(cid:3) Unsatisfacto ry eyebrow scar unless incision carefully sited in hair- bearing area. DERMOID CYSTS1 611 External angle Bridge of nose Tip of nose Submental Suprasternal Fig. 14.10 Sites of dermoid cysts CHAPTER 14 Pediatric surgery 612 Pyloric stenosis Presentation and diagnosis Classically presents in males (4:1) at about si x weeks age with projectile, non-bilious vomiting, visible gastric peristalsis, weight loss , and hypochloraemic alkalosis. Family history may be of value. Differential diag- noses in clude gastro-oesophageal reflux and overfeeding although neither of these should cause alka losis. Confirmation: a) palpation of \200\230tumor \200\231, b) visualization on ultrasound or barium meal. Preparation Preoperative correction of dehydration and metabolic derangement (low serum sod ium, potassium, chloride, and hydrogen ion) with 0.45% sodium chloride/5% dextrose take 24 \200\22348 hours. Monitor resuscitation with U&Es and capillary gasses. NG tube passed and stomach aspirated \200\224saline lavage if evidence of stasis or bleeding. (and potassium). Correction may Position of patient Supine with support under upper lumbar spine. Incision/approach (cid:142)(cid:3) Right upper-quadrant, transverse incision (traditional) at level of liver margin. Anterior and posterior rectus sheaths divided transversely \200 \224 muscle either divided or retracted laterally. (cid:142)(cid:3) Umbilical approach through a curved skin incision in the upper umbilical c rease and vertical midline linea alba split; leaves less conspicuous scar but access is sli ghtly more restricted. (cid:142)(cid:3) Laparoscopy. Procedure (cid:142)(cid:3) Deliver the pylorus through the wound by traction on the greater curve of the stomach. Stabilize the \200\230tumor \200\231 between thumb and fore- finger of the operator. (cid:142)(cid:3) Make a longitudinal incision of serosa in avascular area of mid-anterior w all from the pyloroduodenal boundary (vein of Mayo) on to antral wall proximally. (cid:142)(cid:3) Split all thickened circular muscle fibers transversely with blunt forceps to reveal bulging external surface of mucosa. (cid:142)(cid:3) Test the mucosal integrity by squeezing gastric secretions or air through pylorus. Manage any suspicion of mucosal breach (perforation) by stitching tag of o mentum to pyloromyotomy site, maintaining gastric tube drainage and delaying feeds for at l east 24 hours. (cid:142)(cid:3) Use continuous absorbable 3/0 to rectus sheath and subcuticular ab- sorbable 4/0 sutures to skin. (cid:142)(cid:3) Leave a NG tube in situ to decompress hypertrophied stomach. Feeds reintroduced over the next 24 hours. PYLORIC STENOSIS1 613 Complications Morbidity: (cid:142)(cid:3) Mucosal perforation (about 1%) (cid:142)(cid:3) W ound infection (10%) \200\224related to poor immunity and nutritional state (cid:142)(cid:3) Wound dehiscence (cid:142)(cid:3) Persisting gastro-oesophageal reflux (no t unusual). Treat with thick- ened feeds. Mortality rare (<0.5%) \200\224can follow undiagnosed perforation or milk aspiration. Follow-up Review at two months to assess weight gain and any persisting gastro- oesophageal reflux. Stomach Pyloromyotomy Fig. 14.11 Ramstedt \200\231s pyloromyotomy CHAPTER 14 Pediatric surgery 614 Intussusception Description, incidence, presentation Intussusception is an invagination or telescoping of the terminal ileum/ leo-caecal valve into caecum/ascending colon probably in duced by enlargement of Peyer \200\231s patches. Commoner in males (2:1), it peaks at 3 \200\22310 months, and presents with sudden onset of severe, colicky abdomi- nal pain and p assage of redcurrant mucousy stool. Between spasms of pain, when abdominal wall relaxes, a sausage-shaped mass may be felt in the right hypochondrium. Confirmation is made by plain X -ray, ultra- sound or contrast enema with air or barium. In about 75% cases, air (pneumatic) or contrast (hydrostatic) pressure reduces the intussus ception, sometimes at a second attempt after some hours. Signs of peritonitis, perforation or marked and established ob- struction suggest irreducibility and surgery is then the firs t option. Close liaison between surgeon and radiologist is important in agreeing on indi- c ations for enema reduction, for a prolonged attempt at reduction and for a repeated attempt . Intussusception in older children, recurrent intussusception and ileoileal intussusceptio n raise the possibility of a lead point (e.g. polyp, Meckel \200\231s) and demands surgical reduction. Preoperative preparation Good IV line and correction of fluid/electrolyte imbalance is esse ntial. Position Supine. Procedure (cid:142)(cid:3) Make a right, transverse, subumbilical, muscle-cutting incision. Note any free intraperitoneal fluid (serous, blood-stained, fecal- contaminated). Reduce intussu sception partly intraperitoneally but the mass usually brought out through the wound. Note the degree of ischaemia and integrity of serosal surface noted. Attempt reduction by a comb ination of distal squeezing pressure and gentle proximal taxis. Serosal splitting suggests imminent rupture, and may require resection. Similarly, an obvious mass suggesting a lead-p oint requires resection and anastomosis. Otherwise, continue reduction and, once completed, bowel oedema and discoloration usually rapidly improves. If serious doubt remains about bo wel viability, resection and anastomosis is saf- est option. If caecal area is entirely una ffected by oedema and inflam- mation carry out appendicectomy. This is sometimes to avoid a ny fu- ture confusion about presence of appendix. (cid:142)(cid:3) Close the wound with absorbable continuous 2/0 or 3/0 to anterior and posterior rectus sheaths, use absorbable interrupted 3/0 to subcu- taneous tissue and a bsorbable continuous 4/0 subcuticular. The laparoscopic approach has been used; most useful when complete- ness of hydrostatic/pne umatic reduction is in doubt and in complex cases (e.g. Henoch \200\223Schonlein purpura). Recurrence is about 4%; less after surgi- cal reduction. INTUSSUSCEPTION1 615 Ascending colon Terminal ileum Intussuscipiens Intussusceptum (anterior wall excised) Fig. 14.12 Intussusception of terminal ileum CHAPTER 14 Pediatric surgery 616 Meckel \200\231s diverticulum Incidence and anatomy This represents failure of involution o f the omphalomesenteric duct. Situated on the antimesenteric border of ileum, within 100cm of ileo- caecal junction. Length between 1 \200\2238cm. Characteristically supplied by dist al section of right vitelline artery (end-artery branch of the SMA). Incidence is reported from 0.3 to 4%. In asymptomatic patients, male:female ratio is 1.3:1 but complications comm oner in males (3.3:1). Approximately 25% lined by heterotopic gastric, pancreatic or duoden al mucosa, which increases risk of complications. Presentation (cid:142)(cid:3) Gastrointestinal bleeding (cid:142)(cid:3) Intestinal obstruc tion (intussusception/bands/volvulus) (cid:142)(cid:3) Perforation or inflammation. It can also be found incidentally during laparotomy/laparoscopy. Diagnosis 99m technetium-pertechnetate scan detects heterotopic gastric mucosa (not other d iverticula). Glucagon, pentagastrin, and H2-blockers are given to augment sensitivity. Rena lly excreted isotope in bladder can hide uptake in a Meckel \200\231s (particularly in smal l children). Preparation Ensure normovolaemia if presentation is bleeding. Position of patient Supine. Incision/approach Right, transverse, muscle-cutting, sub-umbilical for open operation. Lapa roscopic-assisted or laparoscopy approach also used. Procedure (cid:142)(cid:3) Identify and ligate vitelline artery and divide it. Excise Mecke l \200\231s with resection of ileal wedge/segment (2 \200\2235cm on either side). Bleeding may be from an ul cer of adjacent ileal mucosa. Close the ileum by end-to- end single-layer interrupted, inve rting anastomosis using non-traumatic needle with 4/0 or 5/0 absorbable. (cid:142)(cid:3) Resect any incidental diverticulum (i.e. no other significant pathology \200\224 bowel not compromised) \200\224lifetime risk of complications about 5%. If inciden tal diverticulum not excised, record clearly in the case-notes and inform parents and G.P. Morbidity Adhesive obstruction following surgery 5 \200\22310%. Long-term complications hig her in previously symptomatic patients. Mortality close to zero. This page intentionally left blank CHAPTER 14 Pediatric surgery 618 Acute appendicitis Classical symptoms: central abdominal pain, vomiting, anorexia, malaise, mild/moderate pyrexia and migration of pain to right iliac fossa. Classical signs: right i liac fossa tenderness, guarding, rebound tenderness and rigidity. Confusing presentations: dysuria, diarrhea \200\224due to unusual position of appendix, young age of child and compl icating/intercurrent illness. White cell count, ESR/CRP, urinalysis, plain abdominal x-ray, ultra- sound scan and CT scan may aid diagnosis. The discipline of \200\230active observation \200\231 limits both unnecessary removal of normal appendix and delayed operation with perforation and peritonitis. Preoperative Fluid resuscitation essential \200\224patient may need 0.9% sodium chloride bo lus followed by 0.45% NaCl, 5% dextrose and added potassium chlo- ride (10 mmol/500ml). Imm ediate preoperative intravenous cefotaxime and metronidazole is required. Position Supine. Incision Transverse incision in skin crease (Langer \200\231s lines) centered on McBurney \200\231s point (1/3 of distance along line from anterior superior iliac spine to umbilicus ). Incision can be extended either laterally into loin or medially if necessary to deal wit h difficult appendicitis or other pathology. Procedure (cid:142)(cid:3) Divide superficial fascia in the line of wound. Split external o blique muscle in line of fibers extending medially to anterior rectus sheath \200\224 small nick i n lateral sheath gives extra access. Split internal oblique and transversus abdominis in li ne of fibers to allow gridiron access. (cid:142)(cid:3) Insert retractors inserted and pick up peritoneum with forceps and open it \200\224take bacteriology swab and any pus aspirated. (cid:142)(cid:3) Identify lower pole of caecum and base of appendix by following taenia col i. Free appendix from surrounding tissue with finger dissection. Deliver the appendix and c aecum into wound. (cid:142)(cid:3) Remove appendix either by retrograde (starting at base) or antegradely depending on its accessibility and degree of freedom. Clamp mesoappendix, divid ed and ligated with absorbable 3/0 suture. Insert purse-string suture (3/0 absorbable) into caecum around base of appendix which is cross-clamped, crushed, ligated and amputated allo wing stump to be buried by tying purse-string suture. If problems arise at any stage in defining or mobilizing appendix, in con- trolling bleedin g or potential bleeding points or in identifying or excluding other pathology, improve acce ss by extending incision as above. If appendix has perforated, search for and remove any in traperitoneal faecolith. Peritoneal lavage with antibiotic containing, warm, saline solu- t ion is helpful in removing intraperitoneal pus and fibrin. Drain peritoneal ACUTE APPENDICITIS1 619 cavity or wound continued oozing or retained faecolith). (rarely needed unless significant abscess, Postoperative No oral intake, NG tube aspiration and continued intravenous mainte- nance fl uid for 24 hours or until gastrointestinal function recovers. Three doses of antibiotic ove r 24 hours \200\224extended to three or five days if perforation/peritonitis especially in young children (<5 years.) Complications (cid:142)(cid:3) 10% normal appendicectiomy rate: minimized by careful \200 \230active obser- vation \200\231 policy. (cid:142)(cid:3) Wound infection (abscess) 5 \200\2237% overall incidence: higher with perf o- rated appendix and free pus. (cid:142)(cid:3) Wound dehiscence: usually heals by secondary intention. (cid:142)(cid:3) I ntraperitoneal collection/abscess 1 \200\2233% incidence: right iliac fossa, intraloop, pelvic, subphrenic. Usually presents at 1 \200\2233 weeks post-op. (cid:142)(cid :3) Secondary adhesion obstruction: requiring gut rest/nasogastric tube aspiration/IV fluids or laparotomy. (cid:142)(cid:3) Respiratory complications of basal atalectasis/pneumonia. Laparoscopic approach (cid:142)(cid:3) Insert an infraumbilical port, left and right iliac fossae ports. (cid:142)(cid:3) Change camera to LIF port following initial inspection. Gras p appendix and mesoappendix divided with coagulation diathermy. (cid:142)(cid:3) Ligate appendix with 2 loops proximally and one loop more distally. (cid:1 42)(cid:3) Divide appendix between distal and proximal loops and removed through umbilical port. (cid:142)(cid:3) Close umbilical wound with 2/0 absorbable to linea alba. 3/0 absorbable to fascia and subcuticular 4/0 absorbable. (cid:142)(cid:3) Close peritoneum (continuous), internal oblique (interrupted) 2/0 absorbab le: external oblique (continuous), superficial fascia (interrupted) 3/0 absorbable; 4/0 sub cuticular absorbable. Appendix abscess/mass If, at presentation, a defined and localized right iliac fossa append ix abscess/mass without generalized peritonitis or obstruction is found, attempted laparoto my and appendicectomy not advisable. Initial man- agement options are: (cid:142)(cid:3) Con servative \200\224IV fluids and antibiotics (cid:142)(cid:3) Ultrasound assisted aspiration of abscess and insertion of tube drain (cid:142)(cid:3) Incision and drainage of abscess. If these measures are successful, interval appendicectomy can be per- formed at 3 \200\2236 months. CHAPTER 14 Pediatric surgery 620 Malrotation and volvulus Embryology and incidence The developing fetal intestine herniates into a physiological exomphalos during which time it lengthens. By the 12th gestational wee k the bowel returns to the abdominal cavity and rotates 270 anti-clockwise around the axi s of the vitello-intestinal artery. Duodenum, ascending and descending colon become fixed. Failure of this rotation and fixation is termed \200\230malrotation \200\231. The implicat ions of this are a midgut, suspended on a narrow-based universal mesentery that is at risk of volvulus \200\224almost invariably clockwise (. Quoted incidence is as high as 1 in 500 live births but this includes infants with abdominal wall defects and congenital dia- phrag matic hernia. Presentation Bile vomiting in the neonatal period indicates intestinal obstruction and, of all possible causes, volvulus requires urgent intervention as the bowel is at risk of becom ing ischaemic. In peritonitis, frank haematemesis, or PR bleeding, laparotomy should not be delayed by other investigations. Plain X-ray can be abnormal, ultrasound may show abnormal relative position of superior mesenteric vessels but upper GI contrast study is diagnostic (DJ junction on right, corkscrewing of small bowel). Preoperative Warn parents of possibility of catastrophic bowel ischaemia if you suspect. Cr oss-match blood and correct any coagulopathy. Incision Supra-umbilical transverse. Procedure (cid:142)(cid:3) Deliver intestine completely and determine if volvulus present. If pre- sent, it is usually clockwise. If intestine is compromised, untwist and cover with warm saline packs to encourage reperfusion. (cid:142)(cid:3) Determine nature of malrotation. Ladd \200\231s bands may run across duode - num to attach to mobile, high lying caecum. Divide these bands follow- ing diathermy coag ulation. Mobilize duodenum so that it lies relatively straight on the right side of the spi ne. Attempt to broaden mesentery by opening one leaf of the mesentery. (cid:142)(cid:3) Return intestine to the peritoneum in the non-rotated position \200\224lar ge bowel on the left and small bowel right. The appendix does not need to be excised but, i f left in situ, the fact and position should be accurately documented and parents informed. (cid:142)(cid:3) If intestine found to be ischaemic with little recovery with warm packs, consider closing and performing a \200\230second-look \200\231 laparotomy after 48 hours. (cid:142)(cid:3) Layered closure with continuous 2/0 or 3/0 absorbable. Subcutaneous 4/0 absorbable. Subcuticular 4/0 or 5/0 absorbable. MALROTATION AND VOLVULUS1 621 Postoperative management NG tube on free drainage until signs of ileus resolve. Consider pa renteral nutrition may be required if this is prolonged. Complications Short gut syndrome if a large amount of small bowel resected. Early diagnosis and intervention essential to avoid this. Stomach Duodenum Ladd s bands Colon Small bowel Clockwise volvulus Fig. 14.13 Volvulus of small bowel CHAPTER 14 Pediatric surgery 622 Epigastric hernia This is herniation of extraperitoneal fat through a small defect in linea alba of upper midline abdominal wall. Commonly midway between xiphisternum and umbilicus b ut can be at any point. It presents either as a visible lump or due to discomfort caused by traction of tethered hernia on underlying peritoneum. Confusion at referral with the much larger, diffuse, midline swelling due to divarication of the rectus abdominis mus- cles, wh ich is not a true hernia and improves and eventually disappears with spontaneous approximat ion of the muscles. Preoperative Mark hernia site carefully as it may be impossible to palpate in the anaes- th etised relaxed patient. Position Supine. Incision Short transverse centered on hernia in midline of abdomen. Procedure (cid:142)(cid:3) Divide subcutaneous fat and fascia transversely to reveal slight ly differ- ent texture of extra peritoneal fat protruding through linea alba fascia. (cid:1 42)(cid:3) The defect often very small (2 \200\2233mm) \200\224enlarged by lateral incision s to permit reduction of fatty hernia. If the fat amputated, supplying vessels must be liga ted/cauterized. Defect closed with 3/0 interrupted non- absorbable sutures. (cid:142)(cid:3) Use interrupted 3/0 absorbable to fascia subcutaneous fat: continuous 4/0 absorbable subcuticular to skin. Complications (cid:142)(cid:3) Bruising or bleeding if haemostasis not secured at operation (cid:142)(cid:3) Residual discomfort if peritoneum tethered to closure of defect. This page intentionally left blank CHAPTER 14 Pediatric surgery 624 Umbilical hernia This is due to a residual small defect in fascial closure at the umbilical ring following cord separation in newborn. It is noted as bulge into umbilicus when infant cries or strains. It is very rarely symptomatic and often remains small. There is potentia l for spontaneous closure up to about 4 years of age especially if defect <1cm at 1 year of age. It is diagnosed by tip of examining finger palpating defect in umbilical ring. Preoperative Standard preparation for day case surgery. Position Supine. Local anesthetic peri-umbilical infiltration or rectus sheath block improv es post-op recovery \200\224rectus sheath block preserves operative field for the surgeon. Incision Subumbilical curved (semilunar) incision in skin fold. Symmetry best achieved by s mall central stab incision and lateral split by opening forceps. Procedure (cid:142)(cid:3) Carry out subcutaneous dissection cephalad on each side around umbilical ring and hernia to isolate. (cid:142)(cid:3) Divide the ring completely to open defect with tissue forceps and retract each lateral extremity of sac. Inspect it to ensure no vitello- intestinal, intraperitoneal connection at umbilicus. (cid:142)(cid:3) Close the defect with closely-spaced, interrupted 2/0 absorbable suture. T ack down the umbilicus to site of hernia repair with one 4/0 absorbable suture. Closure Use subcutaneous interrupted 4/0 absorbable to obliterate dead space and subcuticul ar continuous/interrupted 4/0 absorbable or adhe- sive strip skin closure. Pressure dressin g for 24 \200\22348 hours reduces risk of haematoma. Complications (cid:142)(cid:3) Bruising or haematoma related to residual wound dead space ( cid:142)(cid:3) Infection (umbilicus harbors bacteria) (cid:142)(cid:3) Postoperative abdom inal pain due to intraperitoneal air/blood irritation. This page intentionally left blank CHAPTER 14 Pediatric surgery 626 Inguinal herniotomy Incidence, anatomy, and presentation Commoner in boys (8:1) and on righ t side (3:1). Almost invariably indi- rect (through both rings of inguinal canal), through a patent processus vaginalis. Emerges above and lateral to pubic tubercle (cf. femoral hern ia). Fundus varies from external ring to lower scrotum. Distinguish from hydrocoele by inab ility to get above swelling and no transillumination, but in neonates latter is less useful . Often presents as an intermittent swelling with a cough impulse at inguinal canal. Sperma tic cord feels thickened. Indications In first months of life, chance of irreducibility leading to bowel strangula- t ion and ischaemia is highest \200\224surgery should not be delayed. After 6 months of age, herniotomy is elective unless irreducibility occurs. Par- ents should be warned to present urgently if hernia is red, tender or hard. Incision Skin crease incision (about 2cm) over inguinal canal. Procedure (cid:142)(cid:3) Divide subcutaneous tissue and Scarpa \200\231s fascia in line o f incision (super- ficial epigastric vessels retracted or divided). Expose external oblique fascia and rolled edge of inguinal ligament. Perform herniotomy either at external ring or by opening inguinal canal by splitting external oblique fibers (avoiding the ilioinguinal nerve beneath). (cid:142)(cid:3) Expose hernial sac by dividing external spermatic fascia (if outside inguinal canal), cremaster fibers and internal fascia in line of cord. Lift sac with the co rd into the wound and posterior aspect freed after reducing contents. Separate vas (postero medial) and vessels (poster- olateral) from hernial sac \200\224great care needed to avoid damage to these fine structures. Separate fragile, friable sac from vas and vessels with mi xture of sharp and blunt dissection.