. burns, skin disease, trauma). Congenital diffuse alopecia A rare autosomal recessive condition. Hair is present in the ne wborn pe- riod, but total hair loss occurs over the next few months and does not regrow. Ma y be associated with other anomalies. Systemic disease Hair loss can be s to hypothyroidism , diabetes mellitus, severe systemic disease, iron or zinc de \201 ciency, chemotherapy. Management of hair absence or loss \200 include general health, recent illnesses, drug history, family History: history of alopecia, age of onset. Examination: Investigations: Treat any underlyi ng condition. Wigs may be helpful. Topical steroids may be helpful for alopecia areata. \200 \200 \200 \200 pattern of hair loss; scalp and general examination. hair M,C&S; Wood \200\231s light (tine a capitus); scalp biopsy. 22_McClure_22.indd 830 11/14/2012 3:29:35 PM HAIR DISORDERS 831 Excessive hair Hypertrichosis De \201 ned as hair growth in areas not normally hairy in ei ther sex. Causes \200 \200 \200 Racial. Familial. Certain rare syndromes (e.g. Cornelia De Lange syndrome, mucopolysacchari dosis). Drugs (e.g. diazoxide, ciclosporin, minoxidil). Anorexia nervosa. Protein-energy ma lnutrition. Persistence of fetal lanugo hair at birth. \200 \200 \200 Localized hypertrichosis may be associated with pigmented naevi, spina bi \201 da occulta, in \202 ammatory skin diseases, or topical steroids. Treatment If required remove or treat underlying cause if possible; hair removal using depi latory creams or waxing. Hirsutism Male pattern of hair growth in females. Causes \200 \200 \200 Racial. Familial. Androgen excess (adrenal hypoplasia or tumor, Cushing \200\231s disease, polycystic ovary syndrome). Turner \200\231s syndrome. Drugs (e.g. anticonvulsants, progest erones, anabolic steroids). \200 Investigate If there is any suggestion that not racial or familial (e.g. virilization evide nt). \200 \200 \200 \200 \200 \200 FBC. Plasma free testosterone. Plasma 17-OH progesterone. Serum cortisol. Urine steroid pro \201 le. Skull X-ray to detect possible pituitary tumor. Treatment Treat any underlying disease; reassure if racial or familial; hair removal using depilatory creams or waxing. Hair diseases All diseases are rare and include: \200 \200 wiry wool hair (b p.976); a rare autosomal dominant condition that causes brittle Menkes kinky hair disease: Monilethrix: hair that fails to grow and breaks at 1 \200\2232cm ; Pili torti: \200\230 \202 ickers \200\231 under direct light; Wooly hair syndrome: Cauc asians. hair repeatedly twists over 180*, leading to brittle hair that wiry wooly Afro-Caribbean-like hair in 22_McClure_22.indd 831 11/14/2012 3:29:35 PM CHAPTER 22 Dermatology 832 Nail disorders Paronychia Acute paronychia Common, particularly in newborns. It presents as acute in \202 ammation and tenderness of nail folds and surrounding skin. Treatment Topical antiseptics, and, if severe, oral antibiotic, e.g. cephalexin. Chronic paronychia Associated with nail dystrophy. It is usually caused by chronic wetness (e.g. thumb sucking, resulting in infection with mixed bacteria and Candida). Treatment Keep nail dry; topical nystatin and antiseptics. Tinea unguium (onychomycosis) Se e b p.828. Nail biting Common habit. Permanent nail damage may occur if nail matrix dam- aged. Treatment Gentle dissuasion! Proprietary topical nail solutions that impart a very unpleasa nt taste may be effective. Ingrowing toe-nails Most commonly involves the hallux. A spicule of nail grows into lateral nail fold leading to pain, bacterial paronychia, and granulation tissue. Treatment \200 \200 \200 \200 Local antiseptic. Careful trimming of nail spicule. Education on correct toe-nail cutting. Silver nitrate cauterization of granulation tissue or radical surgery is required when seve re. Subungual hemorrhage Caused by trauma leading to hemorrhage under nail. Perforation of nail with hot needle is curative and relieves pain immediately. 22_McClure_22.indd 832 11/14/2012 3:29:35 PM NAIL DISORDERS 833 Nail abnormalities secondary to generalized disease \200 Congenital abnormal nails (usually atrophic) may be due to rare inherited conditions, e.g. ectodermal dysplasia. s to chronic pulmonary suppuration, e.g. cystic \201 brosis, Clubbi ng: \201 brosing alveolitis, bacterial endocarditis, cyanotic congenital heart disease, m alabsorptive states, IBD, hepatic cirrhosis. Onycholysis: trauma, eczema. Koilonychia: (Koi lonychia is normal in the \201 rst few months of life.) Nail pitting: Beau \200\231s line : Splinter hemorrhages: Yellow nail syndrome: the lungs). Nail \200\223patella syndrome: ra re autosomal dominant condition with small rudimentary patella, elbow deformities, reduced or longitudinal split nails. Rarely, chronic glomerulonephritis develops. premature separation of nail from nail bed due to psoriasis, spoon-shaped nails due to chronic iron de \201 ciency anemia. \200 \200 \200 occurs in psoriasis, eczema, alopecia areata. transverse groove in nail caused by severe sy stemic illness. due to bacterial endocarditis, trauma. due to defective lymphatic drainage (also affects 22_McClure_22.indd 833 11/14/2012 3:29:35 PM CHAPTER 22 Dermatology 834 Photosensitivity and light eruptions Photosensitivity Reactions to sunlight can be precipit ated by drugs (e.g. thiazide diuretics, nalidixic acid), soaps, perfumes, plant pollens, pl ant contact (e.g. giant hog- weed plant). Most common is a dermatitis-like reaction, but al so it may be erythematous or blistering. Porphyria Some forms are photosensitive, e.g. erythropoietic protoporphyria (skin burning, redness, swelling, serous crusting +/ \200\223 subsequent scarring). Treatment is that of t he underlying porphyria together with sun protec- tion (see also b p.819). Juvenile spring eruption Red papules and herpetiform vesicles or blisters develop, usually in spring, over light-exposed skin, particularly ear helices. Commoner in boys. Lesions heal without scarri ng. Topical steroids hasten healing. Polymorphic light eruption Itchy, erythematous, papular rash occurring in sun-exposed areas 6 \200\22348hr after exposure. Most commonly affects adolescent girls. Treatment is with h igh factor sun screen. Actinic prurigo An uncommon condition precipitated by sunlight. Irritant papules, exuda- ti on, and excoriation develop on both exposed and unexposed skin areas. Treatment is with sun protection. Generally resolves after several years. Xeroderma pigmentosum A rare autosomal recessive condition in which hypersensitivity to sun light causes marked erythema followed by dry skin, freckles, hyperpigmentation, atrophy, an d scarring. Solar keratosis and skin cancers eventually develop due to the decreased abilit y to repair DNA damaged by UV radiation. 22_McClure_22.indd 834 11/14/2012 3:29:35 PM PHOTOSENSITIVITY AND LIGHT ERUPTIONS 835 22_McClure_22.indd 835 11/14/2012 3:29:35 PM CHAPTER 22 Dermatology 836 Pigmentation disorders Hyperpigmented lesions Generalized hyperpigmentation (hypermelanosis ) Causes \200 \200 \200 \200 \200 \200 Racial. Sun. ACTH (e.g. hypoadrenalism). Chronic renal failure ( Malabsorption. Drug reacti on. i melanocyte-stimulating hormone (MSH)). Localized hyperpigmentation (hypermelanosis) Causes \200 \200 \200 \200 \200 Pigmented naevi (see bullet points in Freckles. Lentigines. Caf au lait Neuro \201 broma tosis (before puberty diameter, axillary freckles). Viral warts. Polyostotic \201 brous d ysplasia (McAS). Peutz \200\223Jegher \200\231s syndrome (perioral brown macules); post-in \202 ammatory skin disease or trauma. Pigmented naevi, following). macules. (cid:2)6 caf au lait macules >0.5cm \200 \200 Pigmented naevi \200 Melanocytic migration. May be brown, black, or pink, macular, papula r, hyperkeratotic or smooth, hairy or hairless. Almost universal, commonly on face, neck, o r back, appearing after birth throughout childhood, particularly at puberty. Treatment usua lly not required. Surgical removal appropriate for cosmetic reasons, recurrent trauma (e.g. from bra straps), or malignant change (rare in childhood). If congenital, can be extensive \200\224refer to dermatologist/plastic surgeon for treatment and follow-up. Halo naevus: a rea of depigmentation around mole due to production of autoimmune antibodies to melanocytes . Usually reassurance alone is needed, but if irregular depigmentation or irregular mole is present refer to a dermatologist. Mongolian common in dark-skinned races, particularly ove r sacrum, buttocks, back, and shoulders. Most fade spontaneously by age 10yrs. Spindle-cell shaped nodule. Treated by simple excision. Malignant sun exposure. Occurs in older childre n, those with giant congenital pigmented naevi, immunosuppressed, previous chemotherapy, al binism, xeroderma pigmentosum. Change in mole color, shape, size (unless in proportion to c hild \200\231s growth), ulceration, itch, or hemorrhage requires urgent specialist excision biopsy and histology. naevus (mole): developmental anomaly of melanocyte blue spot: macular blue-black lesion present at birth, naevus: benign melanocyte tumor. Red-brown dome- melanoma: rare in childhood. Risk increases with increased 22_McClure_22.indd 836 11/14/2012 3:29:35 PM PIGMENTATION DISORDERS 837 Hypopigmented lesions Generalized hypomelanosis Causes \200 \200 \200 \200 d ACTH and d MSH). Hypopituitarism ( Oculocutaneous albinism. Protein-energy malnutrition. Poorly controlled p henylketonuria (phenylalanine acts as a competitive inhibitor of tyrosinases). Oculocutaneous albinism An autosomal recessive disorder of melanin synthesis. It presents w ith hypopigmented skin, blonde hair, pink irises, photophobia, reduced visual acuity, nysta gmus. Treatment Restrict sunlight exposure, e.g. protective high-level sunscreen; ophthalmology r eferral. Localized hypomelanosis \200 common autoimmune disease (anti-melanocyte antibodies Vitiligo: present) resulting in sharp ly demarcated, often symmetrical white patches. Treatment Reassurance. If severe \200\224to pical steroids; cosmetics; sun protection; phototherapy. Lesions usually persist. Pityriasi s Pityriasis eczema with post-in \202 ammatory hypopigmentation. Hypopigmented 1 \200\2232 cm macules +/ \200\223 \201 ne scale on face or upper body. Treatment \200\224 topical hy drocortisone 1%, frequent moisturizing. Resolves in 2 \200\2233wks. Post-in \202 ammatory Tuberous that are more easily seen under Wood \200\231s light examination. \200 versicolor. See b p.828. alba: common in prepubertal children. Represents low grade \200 depigmentation. sclerosis \200\230ash leaf \200\231 macules: small oval hypopigmented macules 22_McClure_22.indd 837 11/14/2012 3:29:35 PM CHAPTER 22 Dermatology 838 Collagen and elastin disorders Collagen disorders Ehlers \200\223Danlos syndrome See b p.94 6. Comprises a group of several rare genetic (most autosomal dominant) disorders of collage n. In classical EDS the skin is soft, hyperextensible, easily bruised, and heals poorly wit h thin, atrophic \200\230cigarette paper \200\231 scars. Hypermobile EDS is characterized by soft skin with hypermobility of large and small joints. There is no speci \201 c treatm ent. Striae (stretch marks) Result from linear growth exceeding the capacity of new collagen pro - duction (e.g. pubertal growth spurt, with glucocorticoids). Linear reddish- purple marks develop. Most commonly occur on lower back and outer thighs. There is no treatment, but the marks slowly fade. Keloid An excessive \201 brous tissue response to skin trauma. The cause is un- known, bu t often familial and more common in Afro-Caribbean children. Skin trauma results in well-de marcated raised, smooth, scar that extends beyond original injury. Treatment Repeated intralesional triamcinolone injections are helpful if given early in kel oid development. Radiotherapy also may be helpful if given early or before surgery. Osteogenesis imperfecta See b p.762. Group of several rare genetic diseases, mostly autosom al recessive, in which there is inadequate or defective collagen production. Presentation Frequent skeletal fractures and multiple deformities; thin skin; defective tee th; hypermobile joints; and blue sclera. Treatment There is no speci \201 c treatment. Supportive therapy includes use of wheel- ch airs, orthoses, and analgesics for fractures, etc. Severe forms are lethal in infancy. Less severe forms lead to short stature, multiple or recurrent fractures, and deformities. Elastin disorders Cutis laxa A rare congenital disorder of defective elastin that presents with loose skin folds and easily stretched skin that only slowly returns to original positi on. It is associated with later hernia, large vessel rupture, and em- physema. 22_McClure_22.indd 838 11/14/2012 3:29:36 PM CONNECTIVE TISSUE DISORDERS 839 Connective tissue disorders See b pp.784 \200\223787, 789. Skin manifestations of connectiv e tissue disor- ders include the following: Systemic lupus erythematosus Widespread or \200\230butter \202 y rash \200\231 facial ery thema, scalp alopecia, chronic dis- coid patches, light sensitivity. Dermatomyositis Violaceous erythema +/ \200\223 oedema of face (especially eyelids), upper chest, elbows, knees, knuckles, around nails. Rash may become scaly. Morphoea In this idiopathic disorder there is localized sclerosis of the skin. Usually an e nlarging large oval plaque of violaceous hue develops which then gradu- ally becomes indura ted, smooth, and shiny. Usually resolves spontane- ously. Treat severe facial or restrictiv e linear morphoea with pulsed IV methylprednisolone and oral methotrexate. Systemic sclerosis This condition manifests as Raynaud \200\231s phenomenon; \201 nger ti p ulceration; skin of the face and hands becoming progressively indurated and \200\230boun d down \200\231 to underlying tissues; restricted facial movements; beaked nose; mouth puck ering; skin atrophy; telangiectasia; pigmentation; calcinosis. Cutaneous polyarteritis nodosa Tender nodules (usually lower legs) surrounded by livedo ret icularis. Nodules may ulcerate or become necrotic. Lichen sclerosus et atrophicus In this idiopathic chronic in \202 ammatory skin disorder l ocalized distinct atrophic changes with associated pallor usually affect genital and perian al regions, almost always in females (the male variety is balanitis xerotica obliterans, wh ich causes phimosis). Pruritus, blistering, or erythema may occur. Treat with emollients, o r potent topical steroids if severe. 22_McClure_22.indd 839 11/14/2012 3:29:36 PM CHAPTER 22 Dermatology 840 Miscellaneous skin conditions Ichthyoses Inherited group of disorders with underlying abnor mal keratinization. The most common variants include: \200 \200 \200 \200 \200 ichthyosis vulgaris; x-linked recessive ichthyosis; lamellar ichthyosis (LI); non-bullous c ongenital ichthyosiform erythroderma (CIE); bullous congenital ichthyosiform erythroderma. Some types present as a \200\230collodion \200\231 baby (LI and CIE most commonly). There may also be eye ectropion and lip eclabium. Otherwise, presents in the \201 rst few month s of life with dry scaly skin +/ \200\223 erythema. Investigation Skin biopsy and histology. Treatment Avoid soap and detergents, use bath oils, apply regular urea-containing emollient s, or mild keratolytics (e.g. 1% salicylic acid in aqueous cream). If severe, oral retinoid s are justi \201 ed. Prognosis Most forms improve with age (except X-linked ichthyosis). Dermatitis herpetiformis \200 s to IgA antibody directed A rare, chronic autoimmune disease against dermoepidermal junctional antigen. Occurs in coe liac disease. Affects ages 6 \200\22312yrs. Presents as an initial itchy rash of knees, elb ows, buttocks, perineum that evolves into blisters. \200 Treatment Gluten-free diet; oral dapsone. Prognosis is good. Epidermolysis bullosa A group of genetically distinct disorders in which the epidermis sepa rates from dermis. Often presents at birth with sloughing of skin (9 mucous membranes) foll owing minor skin trauma; blister or bulla formation; exhibits positive Nikolsky \200\231s s ign. The level of epidermal/dermal cleavage differs between disorders with the more severe form resulting in scar- ring, \201 nger pseudowebbing, oesophageal strictures, and limb c ontractions. Nails, hair, teeth may also be affected. Skin biopsy for immuno \202 uorescen t mapping determines precise diagnosis. Treatment Supportive (e.g. minimal handling, skilled nursing on silk sheets, foam padding, IV \202 uid/protein/electrolyte replacement as needed, antibiotics for super \201 cial i nfection, nutritional support, topical paraf \201 n and non- adherent bandaging of blister ing areas). Referral to specialized unit is recommended. Prognosis Variable and depends on exact disorder. Generally, autosomal recessive forms are more severe, result in scarring, and present at birth. Severe 22_McClure_22.indd 840 11/14/2012 3:29:36 PM MISCELLANEOUS SKIN CONDITIONS 841 forms are frequently lethal in newborn period. Prognosis improves with skilled input. \200\230Adult \200\231 seborrhoeic eczema Affects post-pubertal child. Caused by yeast ove rgrowth, e.g. Malassezia ovale. Presents with erythema with overlying scaling affecting sca lp (dan- druff), eyebrows, nasolabial folds, cheeks, and joint \202 exures. Treat with a mild topical steroid/antifungal. Zinc de \201 ciency Causes Dietary de \201 ciency (e.g. breastfed very preterm infants) a nd acrodermatitis enteropathica (rare autosomal recessive defect in zinc absorption). Presentation Infants develop demarcated areas of erythema, scaling, and pustules around the mouth, ears, \201 ngers, and toes, anogenital regions; diarrhea; FTT. Investigation Low plasma zinc levels. Treatment Oral zinc supplements restore health. Ectodermal dysplasia There are many forms, the commonest is hypohidrotic ectodermal dyspla- sia (X-linked recessive). Presentation Sparse sweat glands, dry skin, sparse hair, thin eyebrows, characteristic faci es (prominent frontal ridges in chin, saddle nose, sunken cheeks, thick lips, large ears), and defective peg-shaped teeth. Patients are prone to hyperthermia and heat stroke due to r educed/absent sweating. Treatment Supportive. Avoid hyperthermia and treat appropriately if it occurs with rehydrat ion and salt replacement. Use dental prosthetics. Incontinentia pigmenti In this rare X-linked dominant ectodermal dysplasia girls present in the neonatal period with blistering lesions (cropping circumferentially on the trunk and i n a linear distribution on the limbs) that within weeks turn into warty plaques and nodules that resolve to leave streaky hyperpigmenta- tion. Ultimately, the lesions regress by late childhood to leave atrophic, streaky areas of hypopigmentation (often most noticeable on t he back of the calves). Associated with dental, eye, musculoskeletal, and neurological abno rmalities. No speci \201 c treatment available. Dermatitis artefacta Caused by self-in \202 icted skin lesions. Usually affects adolescent girls. Lesions are very variable, but are usually bizarre and sudden in appearance. A help - ful clue is that the patient is often inappropriately unconcerned. Occlusive dressing lea ds to rapid healing. Sympathetic listening is most likely to be helpful. Consider underlyin g abuse. Psychiatric input may be helpful. 22_McClure_22.indd 841 11/14/2012 3:29:36 PM CHAPTER 22 Dermatology 842 Lichen planus Cause is unknown. Itchy, \202 at-topped violaceous papules develop, usual- ly over \202 exor aspects of wrist and trunk. Papules tend to coalesce into hypertrophic plaque. The nails (pits or ridges) and mouth (white lacy net- work) are also often involved . Treatment Topical steroids. Lesions may recur for several years. Mastocytosis In this developmental, abnormal collection of skin mast cells, single or multi ple macular or nodular lesions urticate when rubbed (Darier \200\231s sign). Hyperpigmentat ion develops after several months. There may be systemic involvement. Treatment Antihistamines. Lesions and pigmentation resolve. 22_McClure_22.indd 842 11/14/2012 3:29:36 PM 843 Chapter 23 Pediatric surgery Symptoms and signs that should cause concern 844 Congenital abnormalities: upper airway 846 Congenital abnormalities: tracheo-oesophageal 848 Congenital abnormalities: esophagus 850 Congenital abnormalities: lung 851 Congenital abnormalities: chest 852 Idiopathic hypertrop hic pyloric stenosis 854 Ingested foreign bodies 856 Bezoars 857 Midgut malrotation and vol vulus 858 Intussusception 860 Duodenal atresia 862 Small bowel atresias 862 Meconium ileus 862 Acute appendicitis 864 Mesenteric adenitis 865 Meckel \200\231s diverticulum 865 Gastro schisis 866 Exomphalos (omphalocele) 866 Inguinal hernias 868 Hydroceles 868 Hirschsprung \200\231s disease 870 Rectal prolapse 871 Anorectal malformations 872 Umbilical anomalies 8 74 Testicular torsion 876 Orchitis and epididymitis 877 Testicular trauma 877 Undescended t estes (cryptorchidism) 878 Retractile testes 879 Hypospadias 879 Phimosis and paraphimosis 880 Balanitis/balanoposthitis 880 Priapism 880 Penile trauma 881 Imperforate hymen 881 Labi al adhesions in infants 881 Miscellaneous conditions 882 Perioperative care 884 Consent for surgery 886 Post-operative care: \202 uids 888 Post-operative care: analgesia 890 Post-o perative care: drains and wounds 891 23_McClure_23.indd 843 11/14/2012 3:30:18 PM CHAPTER 23 Pediatric surgery 844 Symptoms and signs that should cause concern Neonates and infants As a pediatrician you wil l be involved with the surgical care of newborn babies, infants, and older children. It is important that you recognize im- portant symptoms and signs that indicate a surgical emerge ncy. Neonatal intestinal obstruction \200 \200 vomiting: the cardinal sign of an intestinal obstruction. assessment: check vital signs and commence resuscitation. Bile-stained Emergency Pass a 10F NGT if the baby is vomiting. X-ray: taken. all children with bile-stained vomiting should have an AXR Radiology \200 \200 Dilated bowel loops on the AXR suggest an intestinal obstruction. Look for free air to indi cate a perforation. In the supine \201 lm this will outline the falciform ligament (umbil ical vein). Be aware of the radiological appearance of a midgut volvulus (see b p.859: prom pt diagnosis is essential if the bowel is to be salvaged. Clinical assessment \200 \200 Anus: Meconium: passage of meconium in a baby with abdominal distension could mean Hirschsp rung \200\231s disease (HSD). Rectal examination: do not perform a rectal examination, inse rt a suppository, or perform a rectal washout without seeking advice \201 rst because som e surgeons use lower GI contrast studies for diagnosis and this may obscure the signs of HS D. make sure the baby has an anus, especially females. most babies pass meconium within 24hr of birth. Delayed Oesophageal atresia (OA) \200 The combination of OA. See also b p.848. Pass a 10F NGT bef ore feeding the baby. Babies with OA and a tracheo-oesophageal \201 stula (TOF) who are v entilated represent a surgical emergency because air escapes down the \201 stula causing gastric distension that cannot be relieved. These babies are at risk of gastric perforation . polyhydramnios and a mucousy baby is suspicious of \200 Congenital diaphragmatic hernia (CDH) Most CDHs are now diagnosed antenatally. Delivery sho uld be arranged in a neonatal surgical center. At delivery secure IV access so that the bab y can be sedated, paralyzed, and then intubated. See also b p.852. \200 Avoid ventilating the baby with a bag and mask because this distends the stomach. If the di agnosis is not made prenatally, suspect CDH in a baby with respiratory distress and apparen t dextrocardia. 23_McClure_23.indd 844 11/14/2012 3:30:18 PM SYMPTOMS AND SIGNS THAT SHOULD CAUSE CONCERN 845 Intussusception in infants Suspect intussusception in any infant with gastroenteritis who i s not get- ting better, is unusually miserable, vomits bile or has blood in the stool (see also b p.858. The classical presentation is an infant with: \200 \200 \200 intermittent colicky abdominal pain; episodic drawing up of the knees; passes \200\230red currant jelly \200\231 stool (late sign). Assessment \200 Resuscitation: restore the circulation. Con \201 rm diagnosis by US. Do not consider radio logical reduction unless you have a surgeon and anesthetist who are able to operate on the child in the event of perforation or failure. these patients often require large volumes of \202 uid to \200 Incarcerated inguinal hernia An irreducible swelling in the groin in a baby who is ill and vomiting is probably an incarcerated inguinal hernia. See also b p.868. \200 \200 \200 Resuscitate a NGT. Pass AXR may clarify the diagnosis by showing an intestinal obstruction and a gas shadow in the bowel trapped in the hernia. Transfer to a pediatric surgeon; do no t wait overnight. the baby. Older children Acute appendicitis Be wary of children with abdominal pain who are taking an tibiotics for a presumed sore throat or UTI. The diagnosis may be appendicitis, but the his tory will be atypical and the abdominal signs are dif \201 cult to decipher or absent (see also b p.864). \200 \200 Observe: Urine: urinalysis is abnormal in 30% of children with acute appendicitis. Resist t he temptation to assume the diagnosis is a UTI unless the urine culture is positive. US: Pe lvic appendicitis: signs will be minimal or absent. Exclude a pelvic abscess with US before assuming the diagnosis is gastroenteritis. admit the child for observation. \200 request an US scan if there is clinical doubt. presentation is with diarrhea and the abdominal Acute scrotal pain Any boy with acute scrotal pain has a testicular torsion until proven ot h- erwise (see also b p.876). \200 \200 Refer all these children to a surgeon. The medicolegal consequences of missing a torsion ar e substantial. 23_McClure_23.indd 845 11/14/2012 3:30:18 PM CHAPTER 23 Pediatric surgery 846 Congenital abnormalities: upper airway Choanal atresia (CA) Congenital obstruction of the p osterior choana of the nose may be unilateral or bilateral. Babies are obligate nose breath ers and bilateral obstruction presents with asphyxia during feeding and sleep. Unilateral o bstruction may pass unnoticed. CA may be a presenting feature of the CHARGE association, wh ich is: C \200 oloboma; H \200 A \200 R \200 etardation of growth and development; G \200 enitourinary abnormalities; E \200 ar abnormalities and hearing loss. Diagnosis \200 NGT: \200 CT scan will determine whether the obstruction is membranous or bony. eart defects; tresia of the choanae; the diagnosis is excluded by passage of a tube down each nostril. Treatment \200 Emergency treatment comprises an oropharyngeal airway and an orogastric tube for feeding. S urgery (which is performed through a transnasal approach) restores the patency of the choan ae. Laryngeal atresia A rare condition that is invariably fatal at birth. The condition is rela tively easy to detect using antenatal US because the fetal lungs appear bright and large. T he large airways can also be visualized because they are dis- tended with fetal lung \202 uid. The condition is not amenable to correction (due to lung hypoplasia) and termination of pregnancy should be offered. Cleft lip and palate See also b p.191. Approximately 1 baby per 1000 is born with a cleft l ip and palate. This may occur sporadically or there may be a family history. A cleft lip is immediately apparent. An isolated cleft palate may not be noticed immediately, but will pr esent with feeding dif \201 culties, particularly nasal regurgitation of milk. A cleft pal ate will interfere with breastfeeding as it precludes generation of suction. Bottle-feeding may also be dif \201 cult unless a squeezable bottle, rather than a rigid bottle, is used . Management \200 Lip repair: \200 Palate repair: \200 Follow-up: hearing. at around 3mths of age. at around 6mths of age. long-term because of problems with speech, dentistry, and 23_McClure_23.indd 846 11/14/2012 3:30:18 PM CONGENITAL ABNORMALITIES: UPPER AIRWAY 847 Pierre \200\223Robin sequence The Pierre \200\223Robin sequence (Fig. 23.1) is characterize d by three features: \200 \200 \200 micrognathia; glossoptosis; cleft palate. Management \200 The large tongue has a tendency to obstruct the airway causing apnoea, particularly during sleep. Prone positioning may help, allowing the tongue to fall forward, but occasionally tr acheostomy is necessary. Endotracheal intubation is often dif \201 cult. Tube feeding may be necessary. The palate is generally repaired between 9 and 18mths of age. The airway prob lems invariably improve with growth. \200 \200 \200 Cleft palate Large tongue occludes airway (glossoptosis) Micrognathia Fig. 23.1 Pierre \200\223Robin sequence. 23_McClure_23.indd 847 11/14/2012 3:30:18 PM CHAPTER 23 Pediatric surgery 848 Congenital abnormalities: tracheo-oesophageal Tracheo-oesophageal \201 stula TOF is usual ly associated with OA. However, an isolated TOF will pre- sent with: \200 \200 \200 choking or coughing during feeding; abdominal distension; recurrent LRTI. Although symptoms are present from birth the diagnosis is frequently not made until later i n childhood. The investigations of choice are a tube injec- tion of X-ray contrast into the esophagus and bronchoscopy. Treatment is surgical division of the TOF through a neck incis ion. Oesophageal atresia and tracheo-oesophageal \201 stula See also b p.844. The incidence of OA and TOF (Fig. 23.2) is 1/3500 live births. \200 \200 75% babies with OA will have a TOF. 10% will have isolated OA, which is usually associated with a long gap or defect. Rare isolated TOF. Rare OA with both upper and lower pouch TOFs. \200 Maternal polyhydramnios is common, although antenatal diagnosis is rare. Babies present at birth with: \200 \200 \200 excess mucus or \200\230mucousy \200\231; choking and cyanosis on feeding; associated malf ormations in 50%, usually the VACTERL association (Box 23.1). Box 23.1 VACTERL association \200 \200 \200 \200 \200 \200 Vertebral anomalies (fused vertebrae, hemivertebrae) Anorectal anomalies (imperforate anus) Cardiac anomalies (all types) TOF Renal abnormalities (all types) Limb abnormalities (radi al ray anomalies, e.g. hypoplastic thumbs) Diagnosis Con \201 rmed or excluded by: \200 Passage of a 10F NGT. \200 CXR: a \201 stula between the trachea and the distal esophagus (TOF). the tube stops in the upper thorax. Air in the stomach indicates Acute management \200 \200 The baby should be kept warm and disturbed as little as possible. The upper oesophageal pou ch should be aspirated regularly by oropharyngeal suction or a Replogle tube. Standard IV \202 uids started. 23_McClure_23.indd 848 11/14/2012 3:30:19 PM CONGENITAL ABNORMALITIES: TRACHEO-OESOPHAGEAL 849 Pre-operative antibiotics are not required unless there is evidence of aspiration pneumonia . Babies who require mechanical ventilation must be referred urgently for surgery because g as will escape down the TOF and produce progressive gastric distension, which impairs venti lation further, ultimately leading to gastric perforation. Surgery \200 Disconnection of the TOF and anastomosis of upper and lower esophagus through a right thora cotomy. Long gap OA may require a feeding gastrostomy and a cervical oesophagostomy in the neonatal period followed by oesophageal replacement during infancy. Some specialist centers now perform the Foker operation, where prolonged internal \200\230stretch \200\231 makes the remnant of the upper and lower esophagus \200\230grow \200\231! High-risk babies may h ave a staged procedure \200\224the TOF is ligated and then the OA repaired a few days later . Complications include anastomotic leak, anastomotic stricture, gastro- oesophageal, and r ecurrent \201 stula. Follow-up \200 Respiratory morbidity in the early years after OA/TOF repair is relatively high, particular ly in the winter months. Consider admitting these children during respiratory infections. O bstruction of the esophagus by food boluses is common in toddlers and young children after OA repair. Usually, it is caused by meat that has not been chewed. Refer for urgent oesopha goscopy. OA + TOF Isolated OA OA + double TOF Isolated H-TOF Fig. 23.2 Types of OA/TOF. 23_McClure_23.indd 849 11/14/2012 3:30:19 PM CHAPTER 23 Pediatric surgery 850 Congenital abnormalities: esophagus Oesophageal stricture Oesophageal strictures in childre n may be congenital (5%) or acquired (95%). Strictures may be acquired as a result of re \202 ux oesophagitis, caustic ingestion, or following repair of OA. Congenital oesophageal strictures most commonly affect the middle and distal third of the esophagus and rarely cau se symptoms in the neonatal period. They may be due to: \200 \200 \200 membranous diaphragm; segmental submucosal \201 brosis; presence of ectopic tracheobronch ial rests. Presentation Strictures present with: \200 \200 \200 regurgitation of undigested food; bolus obstruction; failure to thrive. Diagnosis \200 \200 Barium swallow. Oesophagoscopy. Treatment. \200 \200 Peptic strictures are an absolute indication for anti-re \202 ux surgery. Congenital stric tures may respond to dilatation but resection or oesophageal replacement is often necessary . Caustic ingestion \200 \200 \200 \200 Acute Endoscopy: Feeding Chronic children require oesophageal replacement. phase: resuscitate and support with IV \202 uids and antibiotics. to con \201 rm the severity of the burn. gastrostomy: in severe strictures. phase: serial oesophageal dilatation is performed, but m any 23_McClure_23.indd 850 11/14/2012 3:30:19 PM CONGENITAL ABNORMALITIES: LUNG 851 Congenital abnormalities: lung Congenital cystic adenomatoid malformation (CAM) CAM is a co ngenital malformation of the lung bud characterized by dys- plasia of respiratory epitheliu m. The majority of CAMs are now diagnosed prenatally by US and high-risk cases will be asso ciated with hydrops. Symptomatic CAMs should be resected. \200 Large CAMs present at birth with respiratory distress that is managed along conventional li nes. Small CAMs will be asymptomatic at birth, but may present in early childhood with pulm onary sepsis. Sequestration Pulmonary sequestrations are segments of lung parenchyma with an anomalous bl ood supply from the aorta. There may be an abnormal bron- chial connection with the foregut or tracheobronchial tree. The majority are detected prenatally and the management is resec tion. \200 Large sequestrations will present at birth with respiratory distress or with heart failure from high \202 ow through the feeding vessel. Sequestrations may present in infancy or ch ildhood with pulmonary sepsis. Congenital lobar emphysema (CLE) CLE is an unusual lung bud anomaly characterized by massiv e air trapping in the emphysematous lobe. This compresses the surrounding normal lung and m ay result in mediastinal shift. \200 CLE presenting with progressive respiratory distress within the \201 rst few weeks or mon ths of life nearly always require lobectomy. In the acute phase positive pressure ventilati on may produce rapid worsening of the emphysema. Lobar emphysema identi \201 ed on CXR in the absence of symptoms will need close follow-up, and surgery may be avoided. 23_McClure_23.indd 851 11/14/2012 3:30:19 PM CHAPTER 23 Pediatric surgery 852 Congenital abnormalities: chest Congenital diaphragmatic hernia See b p.844. The incidence of CDH is 1/2400. The main problem is not the diaphragmatic hernia, but rather the associat ed pulmonary hypoplasia that is often severe and determines prognosis. The most common type of diaphragmatic defect is posterolateral (Bochdalek) and left-sided, occur- ring in 90% ( see Figs 23.3 and 23.4). \200 screening: most CDHs are identi \201 ed on antenatal US, but the Antenatal prognosis for these fetuses is poor (i.e. 20% survive). Birth: if the diagnosis i s not made antenatally and the baby presents at birth, clinical \201 ndings may include r espiratory distress, scaphoid abdomen, and apparent dextrocardia. The prognosis for surviva l is 760%. Coincidental: including most anterior (Morgagni) defects. The prognosis is excel lent. 710% of CDHs are discovered during early childhood, Neonatal management \200 Initial management consists of sedation, paralysis, endotracheal intubation, and mechanical ventilation with 100% O2. NGT placement and avoid bag \200\223mask \200\223valve ventilati on. If oxygenation is good and pulmonary hypoplasia is not severe, repair of the diaphragma tic defect is undertaken after a few days either by primary suture or insertion of a prosth etic patch. \200 Hiatus hernia (HIH) HIH refers to herniation of the stomach into the chest through the oe- sophageal hiatus in the diaphragm. The lower oesophageal sphincter also moves and becomes i ncompetent. Most children with HIH present with gastro-oesophageal re \202 ux (GOR). Two t ypes of hiatus hernia are recog- nized (Fig. 23.5): \200 \200 sliding (common); rolling or paraoesophageal (rare). Management \200 \200 \200 Diagnosis is made radiologically by barium meal. Treatment comprises management of the GOR, initially medically. Surgery is reserved for children who fail to respond to medication, c omplicated re \202 ux (e.g. peptic strictures), and paraoesophageal hernias (because of th e risk of incarceration and infarction of the herniated stomach). Surgery involves repair o f the hiatus hernia and a fundoplication to prevent GOR. 23_McClure_23.indd 852 11/14/2012 3:30:19 PM CONGENITAL ABNORMALITIES: CHEST 853 Anterior (Morgagni) hernia Posterolateral (Bochdalek) hernia Fig. 23.3 Types of diaphragmatic hernia. Apparent dextrocardia because mediastinum displaced into right thorax Signs of respiratory distress : tachypnoea cyanosis grunting Bowel sounds may be audible in chest (uncommon) Scaphoid abdomen because bowel in chest Fig. 23.4 Clinical features of CDH. Sliding hiatus hernia (common) Rolling hiatus hernia (rare) Fig. 23.5 Types of hiatus hernia. 23_McClure_23.indd 853 11/14/2012 3:30:20 PM CHAPTER 23 Pediatric surgery 854 Idiopathic hypertrophic pyloric stenosis The incidence of idiopathic hypertrophic pyloric s tenosis (IHPS) is 73/1000 live births. Boys are affected more frequently than girls and IHP S is more common in Whites than in dark-skinned races. The pylorus enlarges as a result of hypertrophy of the circular muscle to produce the typical \200\230tumor \200\231. The caus e remains unknown. Familial occurrence is well docu- mented, particularly in girls. Clinica l features (Fig. 23.6) \200 Vomiting: vomitus is always non-bilious, but it may contain altered blood ( \200\230coffee ground \200\231) or fresh blood from oesophagitis. Vomiting occurs within an hour of feedin g and the baby is immediately hungry. Vomiting may not be projectile in babies who present early ( \201 rst or second week); they are often misdiagnosed as suffering from GOR. Const ipation Dehydration IHPS is rare beyond 12wks of age. projectile, starting in the third or fourth week of life. The \200 \200 