OPEN ACCESS TEXTBOOK OF GENERAL SURGERY

MINOR SURGICAL CONDITIONS OF CHILDHOOD AJW MILLAR RA BROWN

STERNOMASTOID `TUMOUR' Management (FIBRO- MATOSIS COLLI) AND A combination of active and postural TORTICOLLIS physiotherapy is successful in the vast majority of cases. Active: the child's Clinical evidence head is taken gently through a full This may present in three different range of movement (chin to shoulder, ways depending upon the age of the ear to shoulder on each side and patient. extension/flexion.) Postural: · In the neonate 2 - 3 weeks old a positioning such that the child is firm swelling is noticed in the neck encouraged to turn the head towards which is localized to the the affected side e.g. cot position, car sternomastoid muscle. Usually seat, etc. Facial asymmetry cannot be detected because of torticollis - expected to correct itself after the age `wry neck'. Occasionally presents of 5 years. In those cases which with facial and cranial asymmetry. present late or where no progress is · In the older infant there may be no obtained, the muscle is surgically history of a swelling but the divided. In the older child the sternomastoid muscle is now firm investing deep cervical fascia on that and foreshortened. `Wry neck' side is also divided. Less than 5% of · The older child may present with all cases diagnosed in infancy require torticollis. The face is rotated away surgery. It is important to continue from the affected side and the with physiotherapy after surgery. head tilted towards the affected side. This is due to the shortening THYROGLOSSAL CYST AND and fibrosis in the muscle with FISTULA facial and cranial asymmetry are usually apparent. A common problem. Most appear before 5 years of age. Pathology The cause is unknown. However, it Clinical evidence: The cyst usually may well be present in utero thus lies in the midline below the hyoid causing obstetric problems. There is a bone. It is a discreet, firm cystic 20% incidence of abnormal structure. It may become inflamed presentation. Histological changes and subsequent discharge or drainage are the same at all ages; a typical will lead to a persistent fistula. The endomycial fibrosis around individual diagnosis is suggested when, with the muscle fibres which then undergo mouth opened thus fixing the platysma atrophy and fibrous replacement. The and mandible, the tongue is protruded effect of this is to shorten the and the cyst moves upwards. sternomastoid muscle, which over a period of time results in facial Embryology: At the 4th week the asymmetry and plagiocephaly. tongue develops from several growth buds in the floor of the primitive Other causes of torticollis should be pharynx. Just caudal to this is the excluded: localized pathology in the foramen caecum from which the neck which may be inflammatory or thyroid diverticulum develops. During neoplastic causing tilting of the head, the 7th week this diverticulum ophthalmologic conditions such as descends to the front of the neck and squints and nystagmus, and skeletal remains connected to the foramen abnormalities of the cervical spine. caecum by the thyroglossal duct. The

branchial arches are developing at the anterior fontanelle. Dermoids over the same time, the ventral 2nd and 3rd eyebrow (external angular dermoid) arch cartilages forming the hyoid bone. very uncommonly have a deeper There is thus an intimate relationship extension. Those on the scalp also between the thyroglossal duct and the only rarely have a deep extension but hyoid bone. The cyst may be found may be mistaken for other pathology, anywhere from the thyroid to the base e.g. haemangiomas, hamartomas and of the tongue, the cyst arising from the lipomas. thyroglossal duct. Although the cyst is usually connected by a single Treatment: Excision. Pre-op x-ray primary duct structure there may be and ultrasound to exclude deep several small accessory ducts running extension. Dermal sinuses and parallel particularly above the hyoid posterior cysts nearly always have a bone. The duct is lined with stratified deep extension into the CNS, and squamous or ciliated columnar require pre-op CT scan. epithelium with associated mucus secretion. There may be an incidence BRANCHIAL FISTULAE, CYSTS of ectopic thyroid tissue, usually AND CARTILAGINOUS REMNANTS dysplastic, in relation to the duct or cyst. These occur in the head and neck and result from embryonic structures that The treatment is to remove the cyst or have failed to completely disappear sinus and the thyroglossal duct in its after their temporary foetal presence in entirety up to the base of the tongue. early embryonic life. To understand This will include the mid-third of the these lesions the embryology of the hyoid bone (Sistrunk's operation). If head and neck should be studied. only the cyst is removed morbidity is (See Chapter: The Anatomy of the significant with persistence of Abnormal, Part II. A synopsis of secretion local recurrence (30%) and Surgical Anatomy - Lee McGregor, DJ sinus formation. Recurrence is also du Plessis; or The Developing Human, attributed to failure of removal of Clinically Orientated Embryology - KL inferior extension of the tract; or in the Moore.). supra-hyoid area multiple fistulous tracts Sinuses or fistulae related to . second arch. These are the EPIDERMOID / DERMOID CYST commonest branchial remnants. Fistulae discharging mucus are These cystic structures occur in lines present from birth and are evident of fusion. Ectodermal elements along the anterior border of the become buried beneath the skin sternomastoid. There is a 15% surface leading to the formation of incidence of bilaterality. They may cysts. Sebaceous glands, hair follicles also occur in families. The lining of the and connective tissue may be present tract on the inner two-thirds is in the cyst wall giving rise to the typical columnar epithelium and the outer sebaceous material. There may be a one-third is squamous epithelium. The deeper penetration through underlying course of this tract can be predicted bone giving rise to a `dumb-bell' because it arises from the cervical configuration. These inclusion cysts sinus where the second branchial arch are differentiated from the so-called overgrows the third and fourth. The dermoids that occur in ovaries and track is related above to the IXth testes which are in fact teratomas. nerve, the stylopharyngeus muscle extending into the tonsillar fossa, and Common sites: Epidermoid cysts - more inferiorly it traverses the fork of midline supraclavicular area of the the carotid bifurcation, the XIIth nerve, neck and on the scalp over the internal jugular vein and goes beneath

the posterior belly of digastric and HAEMANGIOMAS / VASCULAR stylohyoid to finally open on the skin MALFOR-MATIONS along the lower part of the anterior medial border of the sternomastoid. Although grouped under a single term, haemangiomas encompass a wide Sinuses or fistulae related to the range of pathology. Histological first arch are uncommon and classification is based on the size of classically present inferiorly as an the vascular structure in the lesion: opening on the skin below the ramus capillary, mixed, cavernous, A-V of the mandible, with a tract extending fistulae. Clinically they can be divided upwards to the junction of the bony into two groups. Haemangiomas and and cartilaginous part of the external malformations. auditory canal. The track is intimately related to the facial nerve, the Haemangiomas usually occur in branches of which may have a females, are visible at birth in only variable relationship to the sinus tract. ±40%, but subsequently undergo tremendous endothelial proliferation Pre-auricular sinuses are probably with rapid increase in size. Histology ectodermal inclusions related to the is mainly capillary. This group can be development of the auricle, which expected to subsequently involute. forms from 6 cartilaginous tubercles. The commonest of this variety is Cartilaginous remnants usually arise termed a strawberry haemangioma along the anterior border of the (capillary histology). Treatment is sternomastoid and extend into the thus expectant and involution should fascia of the muscle. These also be complete by about 5 years of age. commonly have a family history and In those areas where the lesion is are bilateral. Surgery is only advised if constantly traumatized, causing symptomatic. infection and bleeding, e.g. the scalp and perineal area, or where its site Branchial cysts tend to present in affects vital functions e.g. over the eye later childhood or early adult life and where complete closure may rapidly are again related to the anterior border result in amblyopia, excision may be of sternomastoid, usually lying deep to attempted. the muscle. They are typically described as feeling like a "half-filled The stork bite or neonatal staining hot-water bottle" with a thick wall and may appear shortly after birth and is contain a yellow creamy substance classically related either to the nape of with cholesterol crystals. They are the neck, over the forehead or sacrum. usually unilateral. Branchial cysts It can be expected to spontaneously related to the first arch may occur fade over several months. within the parotid gland - often misdiagnosed as a pleomorphic The second group can be termed adenoma. malformations in that the lesions are present to their total extent at birth and will not fade but will grow at the same Treatment of all these is careful rate as the child. These show normal excision, being aware of the related endothelial mitotic activity. The port structures. Surgery of branchial wine stain consists of a malformation fistula is usually conducted before the located within the dermis - third year of life as the neck is shorter histologically it is a capillary vascular and the whole fistulous track can be malformation. These lesions removed through a single incision. In correspond to the skin distribution of the older child two separate "step various sensory nerve branches, e.g. ladder" incisions may become on the face the branches of the necessary. trigeminal nerve. Those more deeply

situated in the dermis tend to have a recurrent pulmonary infections; and in darker colour and in time become the gastro-intestinal tract, with hyperkeratotic with the development bleeding. often of troublesome eczema. The salmon patch is fainter in colour and Complications of Haemangiomas blanches on pressure but is not A. Local: Disfigurement, infection, elevated above the skin. pain, ulceration, bleeding, recurrent trauma, regional increase The cavernous haemangioma can in growth, e.g. limb varicosities, also be regarded as a malformation pressure effects, airway and appears as an isolated, puffy, compromise. spongy, soft swelling with a blue B. Systemic: Cardiac failure, colour. These may also occur deep to consumption coagulopathy. the subcutaneous tissue and may extend into muscle. They consist of Summary of treatment mainly large venous spaces. 1. Observation. Involution tends not to occur. 2. Await involution in those cases of the proliferative group or where A combination of both groups occurs there is a proliferative component. where there is some degree of 3. High dose short course steroids in capillary proliferation but usually the those larger proliferative lesions major element is cavernous. These with complications (4-8 mg/kg/day malformations may be more extensive Prednisone). with large fields of greatly increased 4. Interferon may assist involution. purpura and arterio-venous shunting. 5. Intermittent compression if Some involution may be expected to feasible. occur. The local increased blood 6. Surgical excision or local steroid supply may lead to regional gigantism, (triamcinolone) injection for those varicose veins, and systemic small malformations causing local complications such as cardiac failure complications. and consumption coagulopathy, with 7. Embolisation, in some instances. most commonly a thrombocytopaenia. Difficult because often multiple feeding vessels. Congenital arteriovenous fistulae 8. For extensive localized lesions are multiple between small arteries causing life-threatening and veins. Rarely evident at birth, but complications - surgical excision become apparent in childhood, with hypothermia and cardiac enlarging at irregular rates with standstill or bypass. elevation of skin temperature, red 9. Radiotherapy is contra-indicated. colour if superficial and progressive 10. Pulsed flash lamp laser therapy of enlargement of the involved area. port wine stains has had good Arteriography reveals multiple AV results. Treatment of capillary shunts. These tend to be refractory to haemangioma with laser has been treatment. Embolization of feeding less rewarding but worth arteries may help temporarily and attempting in young infants with amputation is a last resort. prominent lesions.

Haemangiomas in Internal Organs Where consumption coagulopathy and Haemangiomas may occur anywhere thrombo-cytopaenia is a primary in the body including the gastro- problem, low dose aspirin and intestinal tract and other abdominal persantin has success when used in viscera, e.g. the liver, leading to some cases. massive hepatomegaly, portal hypertension and heart failure; in the lung resulting in haemoptysis or

LYMPHANGIOMA / CYSTIC tongue with gross enlargement. HYGROMA Again staged excision is the treatment, remembering that there will be a Cystic Hygroma period of prolonged post-operative This is multilocular cystic malformation oedema in the remaining tissue which of the lymphatic system. Incidence may compromise the airway for some 1:12000, 60% present at birth, 90% time. detected before the 2nd year. During development of the lymphatic system, NEUROFIBROMA isolated cell buds fail to achieve venous connections. These continue This is commonly an expression of to produce lymph and this results in Von Recklinghausen's disease, a fluid filled endothelial cysts. These dominant inherited genetic disorder of may enlarge and infiltrate surrounding variable penetrance. Isolated tumours tissue. Many have a solid component may occur in the skin, subcutaneous with bleeding and infection. tissue or in relation to spinal nerves. They are soft, grey, fleshy and slightly Lymphangioma / Mixed mesodermal mucoid. The subcutaneous lesions lie mal-formation in the dermis, producing an elevated There is an ill defined gradation of nodule with compression of the malformation from pure cystic epidermis and stretching of the hygroma to those with an increase in overlying skin. The deep aspect has angiomatous element - so called an ill-defined edge. Where multiple lymphangioma. 75% occur in the nerve twigs are involved, this is called lateral neck and they are twice as a plexiform neurofibroma. There is a common on the left side. Extension definite incidence of malignancy in the into the axilla or mediastinum is lesion itself and an association with common. Secondary infection, which neural crest tumours. In addition in produces massive swelling, often those related to spinal nerves there prolonged due to poor lymphatic may be intraspinal extension with drainage, is common and if this is in spinal cord compression. the neck, it may lead to suffocation. Neurofibromatosis is suggested when the following are present: Treatment is early excision (1) Five or more café au lait macules remembering that the lesion is benign >0.5 cm in diameter. and therefore attempting to avoid (2) Cutaneous neurofibromas. surrounding major structures, (3) Other neural crest tumours, e.g. particularly the facial nerve. The use phaeochromocytoma. of sclerosant injection, Bleomycin in an (4) Leisch nodules in the iris. oil emulsion or OK432 an anti- (5) A relative with neurofibromatosis endothelial cell antibody is plus 2 of the above. occasionally successful but aspiration, drainage or radiotherapy are contra- Diagnosis can be made by skin indicated. In those cases where the biopsy, looking for anastomosing lymph channels are macromelanosomes. cystic spaces (small, almost honeycombed) and where infiltration FIBROLIPOMA surrounds vital structures - there being a predilection to tongue, cheek, This is a common hamartomatous pharynx and retroperitoneum - the lesion, which usually presents as a operations should be staged. The subcutaneous swelling with a poorly commonest form is involvement of the defined edge. Treatment is excision tongue with extension into the floor of biopsy. There is a 15% incidence of the mouth. This often results in local recurrence. Beware of those multiple cystic vesicles over the occurring over the sacrum - usually

associated with a meningocoele that implies excessive development of the may extend into the spinal cord. male breast and usually occurs in adolescent boys. There is often a THE BREAST tender, uncomfortable granular disc under the nipple, with variable soft Neonatal mastitis tissue enlargement. This is believed The majority of infants, both male and to be a result of altered testosterone: female, develop enlargement of one or oestrogen ratio, which originates from both breasts in the first weeks of life. the testis. The disorder is benign and Swelling may be tender and is often self-limiting. accompanied by secretion of a few Carcinoma extremely uncommon in drops of milky material, `witch's milk'. children. This is thought to be due to maternal gonadotrophic stimulation of foetal THE UMBILICUS prolactin and/or oestrogen. Withdrawal of this stimulation at birth Umbilical hernia allows the swelling and secretion to Twenty percent in the newborn. The subside. No treatment is required. defect is round and most close Any attempt to express the breasts spontaneously. The skin may look thin may prolong secretion with increased though it never ruptures. milk production and greatly Complications are rare. If the predisposes to infection. Infection diameter is less than 1 cm at 1 year, itself may be devastating with the defect can be expected to involvement and destruction of the spontaneously close; if the defect is breast bud and chest wall. greater than 2 cm at 4 years, it is Overwhelming septicaemia may lead unlikely to close and an operation is to death. Abscess formation requires usually attempted after 5 years of age. formal drainage and systemic Irreducibility may be caused by antibiotic therapy against the inspissated sand in the small bowel organisms most involved - due to pica. Most reduce Staphylococcus and Streptococcus spontaneously with bed rest and sedation, and are then electively Premature thelarche repaired. A condition of bilateral breast development usually occurring before Para-umbilical hernia/Supra- puberty with no other signs of sexual umbilical hernia maturation nor evidence of oestrogen This is differentiated from umbilical effect on vaginal cells. Although both hernia by the defect being elliptical breasts are involved one may develop and in the linea alba adjacent to the before the other. There is breast umbilicus. These will not close and enlargement but the nipple shows little require surgical repair. Irreducibility is or no change. The condition is also more common. believed to be the result of increased sensitivity to circulating gonadotrophin Umbilical sepsis. and oestrogen. Treatment is Infection in the neonatal period is not expectant with reassurance. Do not uncommon and usually presents with biopsy. This must be distinguished erythema, oedema and swelling of the from precocious puberty where there umbilicus with extending oedema into is early generalized maturation with the abdominal wall. Infection here evidence of secondary sex should never be taken lightly as rapid characteristics and is usually the result progression to septicaemia or of ovarian, or more rarely, intracranial necrotising fasciitis may also occur. or adrenal tumours. Infection may spread via superficial lymphatics to the inguinal lymph Gynaecomastia or pubertal mastitis nodes. Portal vein thrombosis can

occur with subsequent development of mucosa, leaving a pseudopolyp, portal hypertension. Common which presents as a shiny, red organisms are staphylococci, (Cherry tumour) nodule in the haemolytic streptococci and umbilicus with a mucoid discharge clostridium. present from birth. (ii) Patency of the whole tract leaves a An umbilical granuloma presents as fistula, which discharges bile- heaped up grey red granulations and stained small bowel material from is a pyogenic granuloma, which can be the umbilicus. treated with either ligation if there is a (iii) Enterocystoma. Where there is stalk, or daily applications of silver partial obliteration, a cyst may be nitrate with care taken to protect the present which usually lies beneath surrounding skin. Infection in an the umbilicus within the abdomen. obliterated umbilical artery may also This may become infected, present with intermittent purulent resulting in an abscess with discharge. intermittent discharge of pus.

Umbilical discharge Treatment: Exploration of the Discharge from the umbilicus may be umbilicus. A check for an purulent, watery, mucoid or faecal. intraperitoneal remnant, and excision Purulent discharge is most of the ectopic mucosal cyst. commonly related to localised infection of the umbilicus. An inflammatory MECKEL'S BAND response at the umbilicus is part of the A Meckel's band is where there has normal mechanism of shedding of the been obliteration of the omphalo- cord. Prolonged adherence of the mesenteric duct but a residual fibrous cord should make one suspicious of band remains, attaching the umbilicus either a generalised condition relating to the mid-ileum. This may be to the inflammatory response e.g. associated with an antimesenteric immune deficiency, or a localised diverticulum, Meckel's diverticulum, underlying defect at the umbilicus. which is the patent inner segment of the duct. This attachment leads to Watery discharge: This is usually twisting and knotting of the bowel due to a persistent communication around the band, resulting in small with the urachus and bladder and may bowel obstruction and strangulation. suggest lower urinary tract obstruction. Thus the urinary tract should be ANORECTAL PATHOLOGY investigated with ultrasound and cystogram prior to surgical excision of Fissure-in-ano the urachal remnant. A partly patent This, the commonest cause of a minor urachus may present as a midline sub- fresh rectal bleed occurring in infants umbilical mass or if infection has and toddlers, is due to passage of a occurred, as an abscess. This large stool, splitting the muco- requires initial drainage if infected and cutaneous lining of the anal canal. subsequent excision. Usually associated with pain and a streak of blood on the outside of the Mucus or faecal discharge: These stool. Most often occurs in the midline are due to abnormalities of the vitello- posteriorly. Many heal quickly. It is a intestinal duct. Embryonic common cause of rectal bleeding and communication between the midgut realisation that if not treated, the and the yolk sac leaves the yolk stalk. complication of fear of defaecation, This usually completely obliterates. stool retention and subsequent Anomalies of the above may be:- persistent constipation and faecal (i) Enterotoma. This is a soiling may occur. Treatment is to sequestrated nodule of alimentary treat the constipation with a stool

softener and a mild laxative, having unknown. It is important to attended to any predisposing dietary differentiate these from other intestinal factors. A local anaesthetic cream polyps. Inflammatory polyps are may be used for a short period. The unusual but may be associated with rectum must be emptied of hard stool schistosomiasis and ulcerative colitis - (disposable enema). In resistant previously described as pseudopolyps. cases or where a large faecaloma is present, under GA the faecaloma is Hamartomatous polyps removed and the anus forcibly dilated Peutz-Jegher's syndrome: This is a to temporarily paralyze the sphincters, syndrome of inherited polyposis. The thus preventing sphincter spasm (pain polyps are usually multiple +). This allows normal bowel function hamartomatous polyps varying in size to resume. Partial internal from 0.5 to 2 cm diameter anywhere in sphincterotomy is rarely necessary. the gastro-intestinal tract, with the Nitric oxide paste applied locally has majority limited to the small bowel. some benefit There is associated external evidence with hyper-pigmentation of the Rectal Polyps mucosa. Freckles may also occur on These may be single, multiple or the face, hands, feet, digits, and hard diffuse. The commonest is the palate. Inheritance is autosomal juvenile polyp, which is a dominant with high penetrance and pedunculated, round, friable, partly variable expressivity. The sex ratio is cystic lesion composed of regular equal and there is no racial rectal colonic glands set in a fibrous predilection. Symptoms are usually stroma with mucus retention. The due to chronic blood loss and surface is ulcerated, and is covered by anaemia, or bouts of abdominal pain a layer of granulation tissue. This is with incipient or established probably an acquired inflammatory intussusception. Gastro-intestinal lesion although commonly termed a malignancy is rare.(incidence is hamartoma. Presentation is almost approximately 2-3%.) There is a universally with fresh rectal bleeding higher malignancy rate in those polyps and in some cases with prolapse of occurring in the stomach, duodenum the polyp through the anus. Auto- and rectum. In females there is a amputation is recorded in about 10% predisposition to ovarian tumours. of cases. Juvenile polyps have no Treatment is tailored to the number, potential for cancer and treatment is size, location and presentation of the with local excision, >90% being within polyps and can be defined as reach of a sigmoidoscope. Rarely the conservative surgery. polyps may be diffuse and these children usually present young, with Adenomatous polyps symptoms of bleeding, anaemia, Familial polyposis coli: Autosomal prolapse, episodes of intussusception dominant inheritance with varying and malnutrition. Treatment is to degrees of penetrance. These polyps identify the extent of the disease with are adenomas. The condition is barium enema, sigmoidoscopy and inevitably pre-cancerous but rarely colonoscopy, and if extensive, presents in the paediatric age group. resection of the diseased colon should The diagnosis is made by histological be done early before significant examination of a resected polyp. The complications develop. Rarely in treatment is total colectomy, ileo-rectal diffuse juvenile polyposis, a gradation anastomosis and subsequent to adenoma may be seen on histology. fulguration of rectal polyps with regular follow up. Lymphoid polyps are not uncommon and are produced by submucosal Rectal Prolapse lymphoid hyperplasia. The cause is This may be partial-mucosal or full

thickness. Most are partial and due to under GA is indicated. There is a faulty bowel habit training - easily tendency to recurrence. treatable. Predisposing factors of full thickness prolapse are poor stool SWALLOWED FOREIGN BODIES habit, malnutrition with chronic diarrhoea, parasitic infestation, These are very common and rarely whooping cough, cystic fibrosis, present with complications. The neurological defect, e.g. spina bifida, length of the object is more important or anatomical defects, e.g. ectopia than it's sharpness. As a general rule vesicae. if the object has reached the stomach it should be passed uneventfully. Management Occasionally long objects lodge in the Exclude an underlying cause. Buccal duodenum, terminal ileum, or rectum. smear and sweat test; spine x-ray; stool for parasites. If presenting Treatment: Following a history of acutely, conservative treatment with ingestion the position of the FB must reduction of the prolapse and be established by AP and lateral x-ray strapping of the buttocks, thus allowing of neck, chest and abdomen. Those in the anal sphincter to regain tone, may the oesophagus should be removed be sufficient. Where rectal prolapse immediately and others can be recurs after strapping, treatment is watched expectantly. Surgical with sclerosant injections (phenol in treatment is only indicated where almond oil). If chronic and persistent symptoms and signs appear. An a circum-anal suture (Thiersch) or exception is ingestion of alkaline more formal reconstructive operation button batteries (calculator, watch, may be indicated. It is important to hearing aid) as these contain differentiate this from an concentrated sodium/potassium intussusception prolapsing through the hydroxide and also poisonous metals. anus. The prolapsed rectal mucosa is Charged batteries may cause a pale red colour with an inverted coagulative necrosis of adjacent beehive shape whereas the tissue. Thus if lodged in the intussusception is usually grossly oesophagus, a very careful congested, dark plum coloured with a endoscopic assessment must be curved sausage shape. There should carried out after removal. Fatalities always be a sulcus on the edge of the due to oesophageal perforation have intussusception and should this be been recorded. Most button batteries easily reduced, diagnosis must be swallowed pass through the gastro- confirmed sigmoidoscopically or with intestinal tract uneventfully. However contrast enema. Haemorrhoids rarely an acid environment (stomach) occur in children. The other encourages erosion of the plastic differential diagnosis is that of grommet seal, leading to disintegration prolapse of a rectal polyp. of the battery and leakage of its contents. Thus antacids and aperients FUSED LABIA should be given. If the battery remains in the stomach for more than 12 hours Labial adhesion is often referred by it should be removed. This can easily the family physician as an `absent be done by endoscopy. vagina'. It is common and is the result of adhesion of the labia minora. This PAROTID SWELLINGS may cover the introitus sufficiently to Diffuse allow urine into the vagina and may be cribriform in nature. Application of an Inflammation oestrogen cream usually results in 1. Mumps Pus cannot be spontaneous separation after ± 10 expressed from the duct, serum days. If this fails, digital separation amylase increased, neutropoenic,

bilateral 2. Acute suppurative parotitis; Treatment Painful, red, indurated gland Antibiotics for the acute attack associated with duct orifice where indicated. Prevention of stenosis, caused by injury from further attacks with improved oral developing teeth, dehydration from hygiene and gland massage. diarrhoea and vomiting, cystic Spontaneous remission expected fibrosis and AIDS. at age of approx. 13 years. 3. Acute suppurative lymphadenitis in Complications are usually due to an intraparotid lymph node ill-advised surgery. 4. Recurrent parotitis of childhood; Common. Male:female ratio 3:2 Lumps within parotid with maximum incidence after 4 1. Lymphadenopathy years. Rare in Blacks. Due to - pyogenic 80% immaturity of basement membrane - TB of acini; not congenital sialectasis - lymphoma as previously thought. Chemical 2. Mesodermal malformations parotitis due to liberation of parotid - lymphangioma juice into parenchyma which may - haemangioma become secondarily infected. - neurofibroma - fibrolipoma Symptoms 3. Cysts - first branchial arch Recurrent acute episodes of pain and cyst swelling in the parotid, made worse by 4 Neoplasms eating. Fairly sudden onset. Lasts 3-7 5. Rarer lesions : Duct days with frequency varying 3-6 stenosis monthly. There may be constitutional : Stone signs of fever and malaise. Examination: Lymphangioma These may be The whole gland is enlarged and intracapsular or may be primarily in tender. adjacent tissues in the cervico-facial No erythema of overlying skin. region, usually unilateral. If small, Flocculent pus expressible from treatment is conservative, if large and the duct orifice. unsightly careful excision is advised The orifice is often red and after the age of 5 years with oedematous; 60% one side preservation of the facial nerve. involved. Most begin with

unilateral attacks, the other side Neoplasms of the parotid: These becoming involved months or are rare - 5% of all parotid tumours years later. Simultaneous attacks occur in childhood. There is on both sides are rare. commonly a lymph node overlying the

parotid gland and there is also Investigations significant lymphoid tissue within the Pus culture -strep. viridans, gland. staphylococcus, pneumococcus.

Sialogram: punctate sialectasis, Presentation probably caused by the technique Age - approximately 5 years. of sialography on the basis of Long history. underlying duct epithelial and Painless, smooth, localized basement membrane abnormality and usually superficial swelling. with increased fragility and easy rupture. Investigation Sialography - to define

its position within the gland.

Distortion of ducts by tumour careful frequent may suggest malignancy. clinical examinations.

Management 2. Congenital (Giant) pigmented Excision biopsy with naevus preservation of the facial nerve. Pathology: Site: Usually in distribution of (a) Benign: Pleomorphic adenoma. dermatomes. Warthin's tumour Appearance: Uneven (adenolymphoma). Vascular or pigmentation, with 95% lymphatic hamartomas. containing hair. (b) Malignant: 1o: Muco-epidermoid Pathology: Intradermal or carcinoma compound naevus, arising from duct cells. penetrating lower 2/3 of Lymphoma/Rhabdomyos dermis. arcoma. Significant pre- disposition to 2o: Metastases, e.g. malignancy (2-16%). retino- Treatment: Total excision blastoma, as early as technically neuroblastoma. possible. Staged

excision required in THE PIGMENTED SKIN LESION large lesions. Numerous different pathologies, 3. Café au lait spots including moles and naevi which are Site: Anywhere, especially in the commonest neoplasms in humans. axilla. Histologic assessment essential if excision indicated. Appearance: Round, oval, flat, light brown 1. Naevocellular naevi pigmentation, found in 10-20% people Site Anywhere on cutaneous and Pathology: Pigment in basal layer mucocutaneous of epidermis. surfaces. 90% people with Appearances Range from flat neuro-fibromatosis through nodular to (von Recklinghausen’s papillomatous. disease) have these Pathology: According to location but larger and more of naevus cells, viz. numerous. Junctional -confined Highly suggestive if: to dermo- epidermal >5 years old: 6+ spots junction. more than 0.5 cm. Intradermal - dermis <5 years old: 5+ spots only. more than 0.5 cm. Compound-both Treatment: Nil for skin lesion per sites. se.

Treatment: Nil unless: Cosmetic 4. Malignant melanoma excision. Very rare in children, only ± 100 Potential malignant cases reported. change - see melanoma. Site: Anywhere. Danger For suspicious areas: Back, Arms, features - exclude by Neck, Scapula.

Appearance: Pigmented nodule Deeper cells of variegated colours less active than (red, white, blue) superficial within a single (opposite to nodule. melanoma) Danger signs: Treatment: Excision biopsy Irregularity/notchi if clinically ng of border. worrying. Ulceration and bleeding. THE ENLARGED LIMB Rapid growth. Inflammation. There are multiple aetiologies of congenitally enlarged limbs, many of Pain/tenderness/itching. which are poorly understood. If there Satellite lesions. is sudden onset of an enlarged limb during childhood, an underlying Pathology: Proliferation of tumour must be excluded. melanoma cells from dermo-epidermal Causes of enlarged limb include: junction into dermis. 1. Congenital hemihypertrophy: Stage and prognosis, Ipsilateral enlargement of body related to depth of causing mild asymmetry which penetration into may result in orthopaedic deeper layer. problems. (No treatment required.) Treatment: Wide surgical Increased risk of developing excision (>5 cm in Wilms’ tumour (1:32 cf adults) including the 1:14,000) - requires screening; deep fascia. may be associated with Expert histological adrenocortical / hepatic assessment and further tumours; and Beckwith therapeutic options. syndrome. 2. Vascular and mesenchymal 5. Spindle/epitheloid cell naevus malformations: (Juvenile melanoma of Spitz) Haemangioma or a Site: Head combination with A-V fistulae and neck most e.g. Klippel Trenaunay and commonly. Lymphangioma e.g. Osler Appearance: Solitary pink to Rendu Weber Syndrome. tan coloured Often associated with overlying nodule up to 1 vascular markings; may cause cm diameter. disfigurement or Pathology: Benign. Occurs haemodynamic complications primarily in (see Haemangioma). children and Therapeutic problem - if adolescents. indicated, requires expert Little investigations and treatment. pigmentation. 3. Neurofibromatosis: Spindle and epitheloid cells Neurofibromatous overgrowth lying in may cause local enlargement epidermal- due to nerve involvement, as dermal junction. well as overgrowth of fibrous

tissue, fatty tissue and bone. Indications for surgery- Mass/size. Grotesqueness, cosmetic. To exclude malignancy if rapidly growing or painful lesions. 4. Lymphoedema: Primary Congenital (Millroy's disease) especially dorsum of foot or hand. Praecox (puberty onset). Female more than male, usually unilateral. Secondary: Infection, e.g. TB. Tumour. Post-operation, post-radiation. Due to deficient, absent or damaged superficial lymphatic network in the presence of normal arterial and venous systems. Results in pooling of lymph, and secondary venous decompression, in the subcutaneous fat.

Treatment: Non-operative primarily viz. weight loss, elastic stockings, elevation at night - to rid the limbs of as much oedema as possible. Operation only if: Functional improvement needed, e.g. fitting of shoe. Recurrent lymphangitis (uncommon). Rarely for aesthetic reasons.

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