PSU Vol 22, 2004

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PEDIATRIC SURGERY UPDATE

VOLUME 22, 2004

Volume 22 No 01 JANUARY 2004

Hashimoto Thyroiditis

Hashimoto thyroiditis (HT) is a chronic lymphocytic autoimmune thyroiditis seen with some frequency in adolescent females and children. M ost common cause of

of the thyroid gland in children in iodine-sufficient geographic regions. Thyroid cell damage in HT is caused by antithyroid antibody-dependent cell-mediated direc

in antigen-specific suppressor T lymphocytes. The gland shows lymphocyte infiltration with follicular cell hyperplasia. Thyroid antibodies are e levate d. Radionucl

uptake. Initially the child deve lops e levated thyroid hormones (T3 and T4) followed by symptomatic hypothyroidism. Following the hypothyroid phase there is fina

Indications for surgery in HT include: 1- firm enlargement of the gland causing tracheal compress ion with dyspnea, hoarseness or swallowing difficulties, 2- failur

therapy and development of symptomatic hyperthyroid goiter, and 3- deve lopment and enlargement of a solitary thyroid nodule. The incidence of malignancy in H

hyperplastic follicular cell nodule from a follicular neoplasm is very difficult using fine needle aspiration biopsy. Patient with malignant nodules in Hashimoto glan

papillary, females , low frequency of extrathyroidal invasion and nodal metas tasis with absent distal metastas is. It is believe d the lymphocytic infiltration of HT ca

reaction to control tumor growth and proliferation.

References:1- Okayasu I, Fujiwara M, Hara Y, Tanaka Y, Rose NR: Association of chronic lymphocytic thyroiditis and thyroid papillary carcinoma. A study of surgical cases among Japanese, and white and African

1995

2- Webb AJ, Brewster S, Newington D: Problems in diagnosis and management of goitre in childhood and adolescence. Br J Surg 83(11):1586-90, 19963- Nguyen GK, Ginsberg J, Crockford PM, Villanueva RR: Hash imoto's thyroiditis: cyto diagnos tic accuracy and p itfalls. Diagn Cyto patho l 16(6):531-6, 1997

4- Loh KC, Greenspan FS, Don g F, Miller TR, Yeo PP: Influence o f lymphocytic thy roiditis on t he pro gnos tic outcome of patient s with p apillary thyroid carcinoma. J Clin Endocrinol Metab 84(2):458-63, 1

5- Hopwood NJ, Kelch RP: Thyroid masses: approach to diagnosis and management in childhood and adolescence. Pediatr Rev 14(12):481-7, 1993

6- Lafranchi S: Thyroiditis an d acq uired hy pothy roidism. Pediatr Ann 21(1):29, 32-9, 1992

7- Strakosch CR: Thyroiditis. A us t N Z J Med 16(1):91-100, 1986

Bile Reflux Gastritis

Alkaline reflux gastritis develops in patients with previous operations that destroy the integrity of the pylorus as a true sphincter by removing (antrectomy), bypa

obliterating (pyloroplasty) the pylorus. It can be seen in children after repair of duodenal atresia. Symptoms of bile reflux gastritis consis t of e pigastric pain, bilio

gastrointestinal bleeding and weight loss . Eating increase s the discomfort. Endoscopy with biopsy in the presence of achloridia is diagnostic. Mainstay of treatme

consists of histamine 2-rece ptor blockers, aluminum-containing antacids (to absorb bile salts) and metoclopramide (improve gastric emptying). Me dical managem

months. The operation of choice is a Roux-en-y diversion. If the original operation was vagotomy with pyloroplasty, the gastric antrum should be removed (to e li

phase of gastric secretion) and a Roux-en-y gastrojejunostomy constructed.

References:

1- Davidson ED, Hersh T: The s urgical treatment of bile reflux gastritis: a s tudy of 59 patients . Ann Surg 192(2):175-8, 1980

2- Cooperman A M: Pos toperat ive alkaline reflux gastritis. Surg Clin North Am 56(6):1445-59, 1976

3- Sorgi M, Keighley MR: Alkaline reflux gastritis: ass ess ment and therapy . Surg A nnu 14:153-79, 1982

4- Burden WR, Hodges RP, Hsu M, O'Leary JP: Alkaline reflux gastritis. Su rg Clin North Am 71(1):33-44, 1991

5- Ritchie WP Jr.: Alkaline reflux gastritis. Gastroenterol Clin North Am 23(2):281-94, 1994

Ovarian Teratoma

Two-third of all malignant tumors of the ovary in children are germ cell tumors. Overall, teratoma is the most common germ cell tumor. Ovarian teratomas contai

primitive germ cell layers in an ectopic location and seldom appear before the age of five years. Ovarian teratomas are classified as mature, immature and malig

ovarian teratomas in children are benign, cystic, mature tumors. Plain abdominal films may show calcifications. Degree of immaturity depends on cellular differenneuroepithelium. Immature teratoma can grow into large tumors presenting with ascites , peritoneal implants and liver metas tasis . Also, AFP and HCG levels can

ovarian teratoma is inversely proportional to the grade of immature elements present and stage of the disease. Mature teratomas are cured with surgical resectio

curative for most children and adolescents with resected ovarian immature teratoma of any grade, even when elevated levels of serum AFP or microscopic foci of

Chemotherapy should be reserved for cases with relapse. Upon resection surgeons must collect peritoneal fluid for cytology, examine peritoneal surface and liver

suspicious contralateral ovarian lesions, omentectomy and lymph node sampling of e nlarged retroperitoneal nodes.

References:

1- Piver MS, Patt on T: Ova rian cancer in children. Semin Surg Onco l 2(3):163-9, 1986

2- Lazar EL, Stolar CJ: Evaluation and management of pediatric solid ovarian t umors. Semin Pediatr Surg 7(1):29-34, 1998

3- Kobayas hi RH, Moore TC: Ovarian t eratomas in early ch ildhoo d. J Ped iatr Surg 13(4):419-22, 1978

4- Chaung JH, Chen L: Ovarian terato ma with gliomatos is periton ei. J Pediatr Su rg 27(5):662-4, 1992

5- Brown MF, Hebra A, McGeehin K, Ross AJ 3rd: Ovarian masses in children: a review of 91 cases of malignant and benign masses. J Pediatr Surg 28(7):930-3, 1993

6- Cass DL, Hawkins E, Brandt ML, Chintagumpala M, Bloss RS, Milewicz AL, Minifee PK, Wesson DE, Nuchtern JG: Surgery for ovarian masses in infants, children, and adolescents: 102

consecutive patients treated in a 15-year period. J Pediatr Surg 36(5):693-9, 20017- Marina NM, Cus hing B, Giller R, Cohen L, Lauer SJ, A blin A, W eetman R, Cullen J, Rogers P, Vinocu r C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP: Complete su rgica

children with immature t eratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Inte rgroup Study. J Clin Onco l 17(7):2137-43, 19998- Cushing B, Giller R, Ablin A, Cohen L, Cullen J, Hawkins E, Heifetz SA, Krailo M, Lauer SJ, Marina N, Rao PV, Rescorla F, Vinocur CD, Weetman RM, Cast leberry RP: Surgical resect ion alone is effecti

teratoma in children and adolescents: a report of the pediatric oncology group and the children's cancer group. Am J Obstet Gynecol 181(2):353-8, 1999

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Volume 22 No 02 FEBRUARY 2004

Forme Fruste Choledochal Cyst

In 1985, a new variant of choledochal cyst known as forme fruste was described in the pediatric literature. Forme fruste choledochal cyst (FFCC) is characterized

extrahepatic bile duct which does not grows with time. The normal diameter of the common bile duct in children ranges between two and 6 mm. FFCC is associate

mm and below 10 mm. Most patients with FFCC have a long common channel, in which the common bile duct-pancreatic duct junction is away from the duodenal p

obstruction of the terminal common bile duct. FFCC is associate d with feve r, jaundice, abdominal pain, recurrent pancreatitis and altered liver function tes ts. Hist

demonstrates thickened fibrous connective tissue, absent muscular layer with flattened, ulcerated and dysplastic mucosa. Diagnosis is established with ultrasoun

can help delineate the anatomy and presence of a long common pancreaticobiliary channel in FFCC. Management consists of cyst excision and Roux-en-y hepatic

small size the anastomosis is technically difficult and should be performed carefully to avoid stricture and postoperative cholangitis. To maintain ductal anastomos

that diseased ductal tissue not be incorporated in the anastomosis, the circumstance most likely responsible for the high incidence of anastomotic stricture in choloperations.

References:

1- Lilly JR, Stellin GP, Karrer FM: Forme fruste choledochal cyst. J Pediatr Surg 20(4):449-51, 1985

2- Okada A : Forme fruste ch oledocha l cyst . J Pediatr Surg 21(4):383, 1986

3- Okada A, Oguchi Y, Kamata S, et al: Common channel syndrome - diagnosis with endoscopic retrograde cholangio-pancreatography and surgical treatment. Surgery 93: 634-642, 1983

4- Ando H, Ito T, Nagaya M, Watanabe Y, Seo T, Kaneko K: Pancreaticobiliary Maljunction without choledochal cysts in Infants and Children: Clinical features and surgical therapy. J Pediatr Surg 30(12

5- Thomas S, Sen S, Zachariah N, Chacko J, Thomas G: Choledochal cyst sans cyst--experience with six "forme fruste" cases. Pediatr Surg Int 18(4):247-51, 2002

6- Shimotakahara A, Yamataka A, Kobayashi H, Okada Y, Yanai T, Lane GJ, Miyano T: Forme fruste choledochal cyst: long-term follow-up with special reference to surgical technique. J Pediatr Surg 38(1

Gallbladder Polyps

A polypoid lesion identified in the gallbladder of a child is a very rare event. It represents an elevated lesion of the mucosal surface of the gallbladder which in moconcern. Fortunately, most polypoid lesions identified in gallbladders are benign (90%). Histologically they are either adenomatous, hyperplastic, gastric heteroto

The prevalence of such polyps is greater among males and obese children. Ultrasonography is the image method of choice in diagnosing gallbladders polyps in ch

seen as pedunculated or sessile e chogenic les ions attached to the gallbladder wall protruding toward the lumen and fixed in changed of posture. Gallbladder polyp

acalculous cholecystitis. Lesions smaller than 10 mm do not progress to malignancy or development of stones, and none produces symptoms or complications of b

management of gallbladder polyps is indicated when the s ize of the polypoid lesion is above 10 mm in diameter, when associated with gallstones and when the chil

symptoms. Treatment consists of laparoscopic cholecys tectomy. Asymptomatic small polyps (< 10 mm) should be maintained under ultrasonographic surveillance.

References:

1- Yang HL, Sun YG, Wang Z: Polypoid lesions of the gallbladder: diagnos is and indications for su rgery. Br J Surg 79(3):227-9, 1992

2- Barzilai M, Lerner A: Gallbladder polyps in children: a rare condition. Pediatr Radiol 27(1):54-6, 1997

3- Stringel G, Beneck D, Bostwick HE: Polypoid lesions of the gallbladder in children. JSLS 1(3):247-9, 1997

4- Csendes A, Burgos AM, Csendes P, Smok G, Rojas J: Late follow-up of po lypoid lesions of the gallbladder smaller than 10 mm. Ann Surg 234(5):657-60, 2001

5- Kikiros C, Arunacha lam P, Lam MH: Adenomatous hype rplastic po lyp of th e gall bladder a ss ociated with cholelithiasis in a child. Pediatr Surg Int 19(1-2):118-9, 2003

6- Stringer MD, Ceylan H, W ard K, W yatt JI: Gallbladder Polyps in Children - Classification an d Management. J Pediatr Su rg 38(11): 1680-1684, 2003

Gastrocutaneous Fistula

Gastrocutaneous fistula (GCF) is most commonly identified after removing long standing gastrostomy tubes in children. Other times is the result of gastrojejunal

After removing a temporary gastrostomy tube most stomas close between three and six weeks after removal. Persistence of stomach leakage through the gastros

the surrounding skin and causes nutritional depletion. GCF does not close spontaneously when the stoma has been used for a long period of time, when there is di

gastric emptying), foreign body reaction (s ilk), epithelization of the tract (multiple granulomas formation), or associated chronic granulomatous disease (Crohn). S

when constructing surgical gastrostomies. When the tube is in place for more than nine months before removal the incidence of GCF can be as high as 45%. Initi

include H2-antagonist therapy and silver nitrate cauterization. If this does not work permanent management of GCF consists of surgical closure.

References:

1- Kobak GE, McClenathan DT, Schurman SJ: Complications of removing percutaneous endoscopic gastrostomy tubes in children. J Pediatr Gastroenterol Nutr 30(4):404-7, 2000

2- Gordon JM, Langer JC: Gastrocutaneous fistula in children after removal of gastrostomy tube: incidence and predictive factors. J Pediatr Surg 34(9):1345-6, 1999 3- Aronian JM, Redo SF: Gastrocutaneous fistula after tube gastros tomy. Incidence in infants and

children. N Y State J Med 74(13):2364-6, 19744- Davies BW, Watson AR, Coleman JE, Rance CH: Do gastrostomies close spontaneously? A review of the fate of gastrostomies following successful renal transplantation in children. Pediatr Surg Int

Volume 22 No 03 MARCH 2004

Umbilical Granuloma

Persiste nt umbilical swelling and discharge during the neonatal period is of serious concern to both parents and physicians. Among umbilical swelling, the umbilica

most commonly seen condition in the pediatric practice. The normal granuloma, a common inflammatory reaction to the resolving umbilical stump of a newborn sh

3rd week of life after proper hygiene. Persistent beyond this time will need some type of therapy. Umbilical granuloma is managed with 75% Sylver nitrate stick

not innocuous and when apply liberally can cause a minor burn of the periumbilical skin area of the baby. Caution must be observed while applying Sylver nitrate,

exudate to prevent periumbilical spillage, and discuss ion with parent that burns may occur but apparently are not serious. Whenever Sylver nitrate therapy fails a

contains urine or fecal material, the physician should suspect that the child has either a patent urachus or omphalomesenteric duct remnant as both conditions resgranuloma seen in general practice. Ultrasound studies of the periumbilical area looking for a cyst, masses or fixed bowel loops can help determine the presence

Management of the persiste nt umbilical granuloma is surgical with double ligature, cauterization of the base or formal umbilical exploration.

References:

1- Campbell J, Beasley SW , McMu llin N, Hutso n JM: Clinical diagnos is of u mbilical swellings and d ischarges in children. Med J Aus t 145(9): 450-3, 1986

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2- Chamberlain JM, Gorman RL, Young GM: Silver nitrate burns following treatment for umbilical granuloma. Pediatr Emerg Care 8(1): 29-30, 1992

3- Boothroyd AE, Cudmore RE: Ultrasound of the discharg ing umbilicus . Pediatr Radiol 26(5): 362-4, 1996

4- Nagar H: Umbilical granu loma: a new appro ach t o an old prob lem. Pediatr Su rg Int 17(7): 513-4, 2001

5- Lotan G, Klin B, Efrati Y: Double-ligature: a t reatment fo r ped uncu lated u mbilical granulomas in children. A m Fam Phys ician 65(10): 2067-8, 2002

Multicystic Dysplastic Kidneys

Multicystic dysplastic kidneys (MCDK) is a severe form of dysplasia without any regular lobar development or normal calyceal drainage system. The kidney stro

vary. The bigger the cysts the less stroma. Most cases are unilateral; left side affected more often. Bilateral disease is usually incompatible with life. MCDK is t

renal cystic disease and most common entity responsible for an abdominal mass in infants. Most MCDK are associated with atresia of part or all of the ipsilatera

diagnostic of MCDK. Renal scan studies (DMSA) will not concentrate the contrast material. Retrograde studies will show an atretic ureter. The differential diag

mesoblastic nephroma which will show some function on excretory urography or nuclear s tudies different from MCDK. MCDK does not have a premalignant pot

term complications of MCDK is very low. Regional pain caused by the expanding kidney mass is probably the most absolute indication for nephrectomy in MCD

consist of reversible hypertension, symptomatic urinary tract infection and increasing kidney size. Almost 20% of these lesions will regress within the first three

References:

1- Hartman GE, Smolik LM, Shochat SJ: The dilemma of the multicystic dysplastic kidney. Am J Dis Child 140(9): 925-8, 1986

2- Vinocur L, Slovis TL, Perlmutter A D, Watts FB Jr, Chang CH: Follow-up st udies of multicystic dy splas tic kidneys. Radiology 167(2): 311-5, 1988

3- Webb NJ, Lewis MA, Bruce J, Gough DC, Ladusans EJ, Thomson AP, Postlethwaite RJ: Unilateral multicystic dysplastic kidney: the case for nephrectomy. Arch Dis Child 76(1): 31-4, 1997

4- Perez LM, Naidu SI, Joseph DB: Outcome and cost analysis of operative versus nonoperative management of neonatal multicystic dysplastic kidneys. J Urol 160(3 Pt 2): 1207-11, 1998

5- Abidari JM, Park KH, Kenned y W A, Sho rtliffe LD: Serial followup of th e co ntralateral renal s ize in ch ildren with multicys tic dys plastic kidney . J Urol 168(4 Pt 2): 1821-5, 2002

Intractable Constipation

Constipation is a common abdominal symptom in childhood. In the majority of cases no cause is identified and the condition is labeled as idiopathic. More than 90

constipation respond to medical treatment (bulk diet, laxatives and enemas). Less than 10% develops intractable constipation. Intractable constipation, not assoc

disease, neuromuscular disease or repaired anorectal malformations, that fails to respond to aggressive medical management is one of the most difficult conditio

Children have duration of symptoms for a period beyond five years. Intractable constipation produces progressive fecal retention, fecal incontinence, distension o

colon with loss of rectal sensory and motor function. Encopresis ensues when fecal soiling results from the retained fecal material. Idiopathic constipation is assoc

internal anal sphincter. Colonic manometry helps differentiate causes of intractable constipation in childhood showing the length of the abnormal colonic involved

management for intractable constipation can consist of internal myectomy, placement of cecostomy or left-colon tubes for antegrade enema cleansing, or resectio

segment when there is severe stasis and luminal dilatation. Outcomes have thrown mixed results.

References:

1- Pemberton JH, Rath DM, Ilstrup DM: Evaluation and surgical treatment of severe chronic constipation. Ann Surg 214(4): 403-11, 1991

2- Hosie GP, Spitz L: Idiopathic constipation in childhood is associated with thickening of the

internal anal sphincter. J Pediatr Surg 32(7): 1041-1043, 19973- Villarreal J, Sood M, Zangen T, Flores A, Michel R, Reddy N, Di Lorenzo C, Hyman PE: Colonic diversion for intractable constipation in children: colonic manometry helps guide clinical decisions. J Pe

91, 2001

4- Dey R, Fergus on C, Kenn y SE, Shankar KR, Coldicutt P, Baillie CT, Lamont GL,

Lloyd DA, Losty PD, Turnock RR: After the honeymoon--medium-term outcome of antegrade continence enema procedure. J Pediatr Surg 38(1): 65-8, 2003

5- Churchill BM, De Ugarte DA, At kinson JB: Left-colon antegrade continence enema (LACE) procedure for feca l incontinence. J Pediatr Surg 38(12): 1778-80, 20036- Yous sef NN, Pensabene L, Barksdale E, Di Lorenzo C: Is there a role for surg ery beyo nd colonic agan glionosis and ano rectal malformations in children with intractab le const ipation? J Pediatr Surg 39

Volume 22 No 04 APRIL 2004

Spigelian Hernias

A spigelian hernia is a rare protrusion of peritoneal sac that occurs through the transve rsus aponeurosis between the semicircular and lateral border of the rectus

umbilicus. Spigelian hernias are more common in adults than children. The hernia appears as an intermittent mass in the lower abdominal quadrant and flank see

increase abdominal pressure. Bowel or omentum can be identified within the hernia content Some children manifests intermittent abdominal pain. Spigelian hernia

cryptorchidism and neuroblastoma. Diagnosis depends on finding an unusual mass on the anterior abdominal wall and palpation of the rim of the hernia defect upo

Spontaneous closure has not been reported. Management of spigelian hernias is straightforward: surgical repair when diagnosed to avoid incarceration and stran

repairs the defect should be marked prior to anes thesia since it will not be palpable during abdominal wall relaxation. The internal oblique fascia along with the tr

an overlapping manner followed by the e xternal oblique fascia preferably with interrupted nonabsorbable sutures. Recurrence of the defect after surgery is extre

References:

1- Jarvis PA , Seltzer MH: Pediatric Spigelian hernia: a cas e repo rt. J Ped iatr Surg 12(4):609-10, 1977

2- Graivier L, Brons ther B, Feins NR, Mestel A L: Pediatric lateral ven tral (sp igelian) he rnias. Sou th M ed J 81(3):325-6, 1988

3- Komura J, Yano H, Uchida M, Shima I: Pediatric spigelian he rnia: reports of three cases. Su rg Today 24(12):1081-4, 1994

4- Walton JM, Bass JA: Spigelian hernias in infants: report of two cases. Can J Surg 38(1):95-7, 1995

5- Al-Salem AH: Congenital sp igelian h ernia and cryptorch idism: cause or coincidence? Pediatr Surg In t 16(5-6):433-6, 2000

6- White JJ: Concomitant Spigelian and inguinal hernias in a neo nate. J Pediatr Surg 37(4):659-60, 2002

7- Losanoff JE, Richman BW, Jo nes JW: Spigelian hernia in a child: case repo rt and review of the literature. Hernia 6(4):191-3. Epub 2002 Sep 07, 2002

Pericardial Cysts

Pericardial cysts are benign large, single, spheroids congenital collections of serous fluid originating in the mediastinum. Histologically they are made of mesothel

the native pericardium, the appearance if these intrathoracic masses in simple chest films are that of cardiomegaly. Pericardial cysts account for 7% of all medias

Most pericardial cysts are asymptomatic, located in the right cardiophrenic angle and dete cted incidentally during routine ches t films. Symptoms and serious com

cough, respiratory distress, chest pain and cardiac tamponade can occur the result of an expanding lesion on vital adjacent structures . The diagnosis of pericardia

using ultrasonography. Once suspected the diagnosis is established by noninvasive studies such as echocardiography and CT scans. Bronchogenic cysts have s i

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Management of pericardial cysts is surgical excision whenever possible. The objective of removal of the lesion is elimination of the tumorous mass, relieve of sy

histological examination. Approach can be open or video-assisted thoracoscopic surgery. Prognosis is excellent in most cases.

References:

1- Bini RM, Nath PH, Ceballos R, Bargeron LM Jr, Kirklin JK: Pericardial cyst diagnos ed by two-dimensional echoca rdiography and co mputed to mography in a n ewborn. Pediatr Cardiol 8(1):47-50, 1987

2- Abad C, Rey A, Feijoo J, Gonzalez G, Martin-Suarez J: Pericardial cyst. Surg ical resection in two symptomatic cases . J Cardiovasc Surg (To rino) 37(2):199-202, 1996

3- Bava GL, Magliani L, Bertoli D, Gorrieri PF, Rimini A, Zaccagnini G, Bertolini A: Complicated pericardial cyst: atypical anatomy and clinical course. Clin Cardiol 21(11):862-4, 1998

4- Eto A, Arima T, Nagash ima A: Pericardial cyst in a child treated with video-as sist ed th oracos copic s urgery. Eur J Ped iatr 159(12):889-91, 2000

5- Noyes BE, Weber T, Vogler C: Pericardial cys ts in ch ildren: surgical or conservative ap proach? J Pediatr Surg 38(8):1263-5, 2003

Total Urogenital Mobilization

Total urogenital mobilization (TUM) was initially described by Pea in 1997 to technically ease the surgical management of persistent cloaca. Specifically cloacas

than three centimeters in length managed using the posterior sagittal approach during separation of the vagina from the urinary tract. TUM, as the word implies,

and mobilization of the entire urogenital sinus as a single unit anteriorly, posteriorly and laterally until enough length is achieved to connect the vaginal edges to t

technical approach reduces operating time and improves final cosmetic appearance. Furthermore, TUM can reduce the incidence of postoperative complications s

fistulas, vaginal stricture and acquired vaginal atresia. Following this initial report the te chnique has been expanded to include case s of congenital adrenal hyperp

(Prader Classification II, III and IV), female bladder exstrophy/epispadia and penile agenesis. When the urogenital sinus is not associated with a cloacal deformit

performed perineally. These cases might need a posterior perineal skin flap to widen the vaginal introitus. The technique can be combined

with reduction clitoroplasty for the surgical management of girls with masculinized external genitalia. With adequate urogenital circumferential mobilization urinar

preserved.

References:

1- Pea A : Total uro genital mobilization--an eas ier way to repair cloacas . J Pediatr Surg 32(2):263-7, 1997

2- Ludwikowski B, Oesch Hayward I, Gonzalez R: Total uro genital s inus mobilization: expanded applications . BJU Int 83(7):820-2, 1999

3- Kropp BP, Cheng EY: Total urogenital complex mobilization in female patients with exstrophy. J Urol 164(3 Pt 2):1035-9, 2000

4- Jenak R, Ludwikowski B, Gonzalez R: Total urogen ital sinus mobilization: a modified perineal ap proach for feminizing genitop lasty and urogen ital sinus repair. J Urol 165(6 Pt 2):2347-9, 2001

5- Hamza AF, Soliman HA, Abdel Hay SA, Kabesh AA, Elbehery MM: Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia. J Pediatr Surg 36(11):1656

6- Hamza AF, Soliman HA, Hay SA, Kabesh AA, Soliman SM, El Behery MM: Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia. Saudi Med J 24(5

Volume 22 No 05 MAY 2004

Pyomyositis

Pyomyositis is a purulent infection of skele tal muscle commonly seen in children who live in tropical countries. For pyomyositis to develop initial muscle injury foll

coexist. The initial traumatic event causes a localized cutaneous infection which is the source of the bacteremia that seeds the injured muscle tissue. Pyomyositis

especially those who participate in strenuous physical activity. Peak incidence occurs between two and five years of age in children. Associated conditions (60%)

disease and HIV. Clinically, pyomyositis is accompanied by abscess formation in the suppurative phase (fluctuance) but may be without a focal fluid collection in t(pain, fever, cellulitis, indurated muscle). Most common sites of abscess formation are the quadriceps and gluteal muscles. Organisms more commonly associated

Staphylococcal Aureus which affects 90% of cases and streptococcus species. MRI is the most accurate means of diagnosing a lesion within muscle determining l

les ion. Initial management consists of systemic antibiotics. Surgical drainage and debridement are of paramount importance in the management of pyomyositis. A

aspiration or drainage should be cultured for aerobic and anaerobic bacteria. In immunocompromised patients progression to the s epticemic stage is associated w

mortality.

References:

1- Meehan J, Grose C, Soper RT, Kimura K: Pyomyositis in an ad olescen t female athlete. J Ped iatr Surg 30(1):127-8, 1995

2- Brook I: Pyomyos itis in children, caus ed b y anaerobic bact eria. J Pediatr Surg 31(3):394-6, 1996

3- Akman I, Ostrov B, Varma BK, Keenan G: Pyomyos itis: report o f three patients and review of th e literature. Clin Pediatr (Phila) 35(8):397-401, 1996

4- Ameh EA: Pyomyositis in ch ildren: analysis o f 31 cases . Ann Trop Paed iatr 19(3):263-5, 1999

5- Bibbo C, Patel DV, Mackes sy RP, Lin SS, Barricella RL: Pyomyos itis of the leg with ea rly neuro logic compromise. Pediatr Emerg Care 16(5):352-4, 2000

6- Flier S, Dolgin SE, Saphir RL, Shlasko E, Midulla P: A cas e con firming the progres sive s tages of pyo myositis. J Ped iatr Surg 38(10):1551-3, 2003

Congenital Tracheal Stenosis

Congenital tracheal stenosis (CTS) is a rare condition seen immediately after birth or in early infancy that is uniformly life-threatening. Infants with CTS prese nts

distress , recurrent pulmonary infections or failure to thrive. Inflammation of the mucosa or mucous accumulation can eas ily obstruct the already ste notic airway.

or long segment of circular cartilaginous ring. Diagnosis is established using bronchoscopy, MRI (assessment of vascular structures and relation to the stenosis),

delineation of the airway). Each individual malformation is studied using rigid tracheo-broncho- esophagoscopy. Associated cardiac defe cts should rule out with ec

of CTS is surgical. Selection of the type of treatment depends on the patient's clinical status and the anatomic pattern of the stenosis. Resection of a short stenos

possible with a length that does not exceed half of the trachea. For longer stenosis the most useful technique consists of enlargement tracheoplasty with cartilagin

the more recent and slide-tracheoplasty. The latter technique is preferable because it preserves native tracheal tissue with fewer postop complications. For CTS o

other associated thoracic malformations, such as a pulmonary sling which may compromise the results of the surgical correction of the tracheal stenosis. Best pro

simultaneous correction of the respiratory and cardiovascular malformation. Overall survival of these children is 75%.

References:1- Lang FJ, Hurni M, Monnier P: Long-segment congenital tracheal stenosis: treatment by slide-tracheoplasty. J Pediatr Surg 34(8):1216-22, 1999

2- Matute JA, Romero R, Garcia-Casillas MA, de Agustin JC, Marhuenda C, Berchi FJ, Vazquez J: Surgical approach to funnel-shaped congenital tracheal stenosis. J Pediatr Surg 36(2):320-3, 2001 3- Grillo HC, Wright CD, Vlahakes GJ, MacGillivray TE: Management of congenital tracheal stenosis by means of slide tracheoplasty or resection and reconstruction, with long-term follow-up of growth

Cardiovasc Surg 123(1):145-52, 2002

4- Backer CL, Mavroudis C, Holinger LD: Repair of congen ital tracheal st enos is. Semin Thorac Cardiovas c Surg Ped iatr Card Surg An nu 5:173-86, 2002

5- Rutter MJ, Cotto n RT, Azizkhan RG, Manning PB: Slide trach eoplast y for t he management of complete trach eal rings. J Pediatr Surg 38(6):928-34, 2003

6- Anton-Pacheco JL, Cano I, Garcia A, Martinez A, Cuadros J, Berchi FJ: Patterns of management of congenital tracheal stenosis. J Pediatr Surg 38(10):1452-8, 2003

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7- Koopman JP, Bogers AJ, Witsenburg M, Lequin MH, Tibboel D, Hoeve LJ: Slide tracheoplasty for congenital tracheal stenosis. J Pediatr Surg 39(1):19-23, 2004

Aplasia Cutis Congenita

Congenital absence of skin, better known as Aplasia Cutis Congenita (ACC) is a rare birth malformation characterized by partial or full-thickness absence exte nd

tissues in a localized manner. Sites involve in ACC include most commonly the vertex of the scalp region (85%) followed by truncal and extremity skin areas. In t

include the dura with brain exposure. Life threatening hemorrhage from the sagittal sinus or sepsis may occur if closure is delayed. Most affected children with A

is not clear. A few are associated with other malformations such as omphalocele, absence of distal limbs and cleft deformities. The majority of these lesions are si

centimeters in diameter. Management of ACC is conservative for small lesions with excision and primary closure. A larger lesion might need split-thickness skin

technique for closure.

References:

1- Vinocur CD, Weintrau b W H, Wilensky RJ, Coran AG, Dingman RO: Surgical management of aplasia cu tis congen ita. Arch Surg 111(10):1160-4, 1976

2- Sargent LA: Aplas ia cutis congen ita of the sca lp. J Pediatr Surg 25(12):1211-3, 1990

3- Ross DA, Laurie SW, Coombs CJ, Mut imer KL: Aplasia cutis congen ita: failed co nservative trea tment. Plast Reco nst r Surg 95(1):124-9, 1995

4- Casanova D, Amar E, Bardot J, Magalon G: Aplasia cutis congenita. Report on 5 family cases involving the scalp. Eur J Pediatr Surg 11(4):280-4, 20015- Verhelle NAC, Heymans O, Deleuze JP, Fabre G, Vranckx JJ, Van den hof B: Abd ominal Aplasia Cutis Congenita: Case Report an d Review of t he Literature. J Pediatr Sug 39(2): 237-239, 2004

Volume 22 No 06 JUNE 2004

Foveolar Hyperplasia

Idiopathic focal foveolar hyperplasia (FH) is a rare cause of gastric outlet obstruction in infants. These non-neoplastic polyps are usually found in adults. Children

early in life with persiste nt postprandial vomiting and failure to thrive, signs which are undistinguishable from hypertrophied pyloric ste nosis. The characteristic h

hyperplasia consists of enlarged, tortuous and dilated gastric pits (fove olas), producing a redundant mucosa that causes partial obstruction of the antro-pyloric are

eosinophilic inflammatory reaction suggesting an allergic component. Some reports have suggested cows' milk protein allergy as a key factor. In general, the etio

reports have found that foveolar hyperplasia develops after prostaglandin E infusion, an effect which is dose related, and resolves with cessation of the drug. Ultr

canal will demonstrate a filling defect, polypoidal, redundant lesion with central echogenic folds without muscular wall thickening. UGIS shows a longitudinal fillin

reveals polypoidal mucosal hypertrophy originating from antrum and extending into the duodenal cap. Biopsy e stablishes the diagnosis. Management of sympto

hyperplasia consists of surgical excision of the involved redundant mucosa with pyloroplasty or pyloromyotomy.

References:

1- Katz ME, Blocker SH, McAlister WH: Focal foveo lar hyperplas ia presen ting as an an tral-pyloric mass in a youn g infant. Ped iatr Radiol 15(2):136-7, 1985

2- McAlister WH, Katz ME, Perlman JM, Tack ED: Sonography of focal foveolar hyperplasia causing gastric obstruction in an infant. Pediatr Radiol 18(1):79-81, 1988

3- Mercado-Deane MG, Burton EM, Brawley AV, Hatley R: Prostaglandin-induced foveolar hyperplasia simulating pyloric stenosis in an infant with cyanotic heart disease. Pediatr Radiol 24(1):45-6, 1994

4- Holland A J, Freeman JK, Le Quesne GW, Khon g TY: Idiopath ic focal foveolar hy perplasia in infant s. Ped iatr Surg Int 12(7):497-500, 1997

5- Master V, Davidson G, Morris L, Martin J, Kennedy D, Byard R, Freeman J: Focal foveolar cell hyperplasia presenting as recurrent emesis in a young infant. J Pediatr Gastroenterol Nutr 26(2):222-5, 19

6- Morinville V, Bernard C, Forget S: Foveolar hyperplasia secondary to cow's milk protein hypersensitivity presenting with clinical features of pyloric stenosis. J Pediatr Surg 39(1):E29-31, 2004

Congenital Extremity Gangrene

Being born with arterial or venous occlusion of a distal extremity and gangrene is a rare event of obscure etiology in newborns. Arterial thrombosis , emboli, trau

sepsis, dehydration, coagulopathies , venous occlusion from direct pressure, constrictive bands, compression by the encircling umbilical cord, and venipuncture are

should be considered in the differential diagnosis of congenital gangrenous extremity. Unfortunately in most cases the etiology cannot be established. When gang

surgical amputation, autoamputation, or some loss of function is usual. Management is in general supportive, allowing the ischemic area to demarcate and slough.

and splinting to avoid contracture are helpful in the rehabilitative phase. In very rare occasions early aggressive systemic thrombolytic therapy (urokinase) follow

debridement and ultimate skin cove rage through cultured epithelial autografts have been reported with good limb salvage results. Peripheral Ischaemic insults pr

of a wider spectrum of disorders, both prenatal and perinatal, attributable to occlusive vascular disruption.

References:

1- Hensinger RN: Gangrene of the newborn. A case report. J Bone Joint Surg Am 57(1):121-3, 19752- Nazer H, Abu Rajab A, Qaryou ti S, Tarawneh M, Hamzeh Y, Arda H, Mus tafa M: Neonata l limb gangrene and ren al vein thrombos is. Case repo rt with review of literature. Eur J Pediatr 146(4):429-31, 1

3- Turnpenn y PD, Stahl S, Bowers D, Bingham P: Peripheral isch aemia and gan grene p resen ting at birth. Eur J Pediatr 151(8):550-4, 1992

4- Ricciardelli E, Morgan RF, Lin KY: In utero brachial artery th rombosis: limb s alvage with po stn atal urokinase infusion. A nn Plas t Surg 34(1):81-3, 1995

5- Carr MM, al-Qattan M, Clarke HM: Extremity gangrene in utero. J Hand Surg [Br]. 21(5):652-5, 1996

Parotid Hemangioma

Parotid hemangioma (or hemangioendothelioma) is by far the most common tumor of the parotid gland seen in infants and children. Initially the infant presents wi

cheek during the first weeks of life. The swelling is generally confined to the superficial lobe of the parotid gland, but it can involve the masseter muscle. With cap

of the skin and subcutaneous tissue the diagnosis is easier to establish. MRI is the investigation of choice because of picture quality, definition of soft tissues and

radiation. MRI allows a definite diagnosis to be made without any invasive procedure being required. When in doubt a fine-needle biopsy will establish the histolo

with Doppler imaging (lobular internal structure, fine echogenic internal septations, mildly lobulated contour and extremely high vascularity), and labeled red ce ll

area of intense activity) can also sustain the diagnosis of parotid hemangioma. Management is conservative since most lesions involute spontaneously. During in

calcification can occur. Medical management (intralesional injection of steroids, systemic steroids or interferon) is given when the tumor is large, deforming, ulcerstructures with functional consequences. The overall response rate is very high.

References:

1- Tress erra L, Martinez-Mora J, Boix-Ochoa J: Haemangiomas o f the parotid gland in children. J Maxillofac Su rg 5(4):238-41, 1977

2- George CD, Ng YY, Hall-Craggs MA, Jones BM: Parotid haemangioma in infants: MR imaging at 1.5T. Pediatr Radiol 21(7):483-5, 1991

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3- Huchzermeyer P, Birchall MA, Kend all B, Bailey CM: Parotid haemangiomas in childhood: a case for MRI. J Laryngol Otol 108(10):892-5, 1994

4- Roebuck DJ, Ahuja A T: Hemangioend othelioma of the parotid g land in infants : son ograph y and correlative MR imaging. AJNR Am J Neuroradiol 21(1):219-23, 2000

5- Espos ito C, Zupi A , Califano L: Surgical therapy o f parot id hemangiomas. Ped iatr Surg In t 17(5-6):335-7, 20006- Greene A K, Rogers GF, Mulliken JB: Management o f parotid hemangioma in 100 children. Plas t Recons tr Surg 113(1):53-60, 2004

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