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. Most common cause of asymptomatic enlargement of the thyroid gland in children in iodine-sufficient geographic regions. Thyroid cell damage in HT is caused by antithyroid antibody-dependent cell-mediated direct toxicity linked to deficiency in antigen-specific suppressor T lymphocytes. The gland shows lymphocyte infiltration with follicular cell hyperplasia. Thyroid antibodies are elevated. Radionuclear scans show absent uptake. Initially the child develops elevated thyroid hormones (T3 and T4) followed by symptomatic hypothyroidism. Following the hypothyroid phase there is final recovery in most patients. Indications for surgery in HT include: 1- firm enlargement of the gland causing tracheal compression with dyspnea, hoarseness or swallowing difficulties, 2- failure to respond to suppressive therapy and development of symptomatic hyperthyroid goiter, and 3- development and enlargement of a solitary thyroid nodule. The incidence of malignancy in HT is low. Differentiating a hyperplastic follicular cell nodule from a follicular neoplasm is very difficult using fine needle aspiration biopsy. Patient with malignant nodules in Hashimoto glands are most commonly papillary, females, low frequency of extrathyroidal invasion and nodal metastasis with absent distal metastasis. It is believed the lymphocytic infiltration of HT causes a form of immune reaction to control tumor growth and proliferation.

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 Americans. Cancer 76(11):2312-8, 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, 1996
3- Nguyen GK, Ginsberg J, Crockford PM, Villanueva RR: Hashimoto's thyroiditis: cytodiagnostic accuracy and pitfalls. Diagn Cytopathol 16(6):531-6, 1997
4- Loh KC, Greenspan FS, Dong F, Miller TR, Yeo PP: Influence of lymphocytic thyroiditis on the prognostic outcome of patients with papillary thyroid carcinoma. J Clin Endocrinol Metab 84(2):458-63, 1999
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 and acquired hypothyroidism. Pediatr Ann 21(1):29, 32-9, 1992
7- Strakosch CR: Thyroiditis. Aust 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), bypassing (gastrojejunostomy) or obliterating (pyloroplasty) the pylorus. It can be seen in children after repair of duodenal atresia. Symptoms of bile reflux gastritis consist of epigastric pain, bilious vomiting, anemia, gastrointestinal bleeding and weight loss. Eating increases the discomfort.  Endoscopy with biopsy in the presence of achloridia is diagnostic. Mainstay of treatment for bile reflux gastritis consists of histamine 2-receptor blockers, aluminum-containing antacids (to absorb bile salts) and metoclopramide (improve gastric emptying). Medical management should be tried for many 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 eliminate cephalic and humoral phase of gastric secretion) and a Roux-en-y gastrojejunostomy constructed.

1- Davidson ED, Hersh T: The surgical treatment of bile reflux gastritis: a study of 59 patients. Ann Surg 192(2):175-8, 1980
2- Cooperman AM: Postoperative alkaline reflux gastritis. Surg Clin North Am 56(6):1445-59, 1976
3- Sorgi M, Keighley MR: Alkaline reflux gastritis: assessment and therapy. Surg Annu 14:153-79, 1982
4- Burden WR, Hodges RP, Hsu M, O'Leary JP: Alkaline reflux gastritis. Surg 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 contain tissue from the three 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 malignant. The vast majority of ovarian teratomas in children are benign, cystic, mature tumors. Plain abdominal films may show calcifications. Degree of immaturity depends on cellular differentiation and foci of neuroepithelium. Immature teratoma can grow into large tumors presenting with ascites, peritoneal implants and liver metastasis. Also, AFP and HCG levels can be elevated. Survival in ovarian teratoma is inversely proportional to the grade of immature elements present and stage of the disease. Mature teratomas are cured with surgical resection only. Surgery is also 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 yolk sac tumor are present. Chemotherapy should be reserved for cases with relapse. Upon resection surgeons must collect peritoneal fluid for cytology, examine peritoneal surface and liver, perform wedge biopsy of suspicious contralateral ovarian lesions, omentectomy and lymph node sampling of enlarged retroperitoneal nodes.

1- Piver MS, Patton T: Ovarian cancer in children. Semin Surg Oncol 2(3):163-9, 1986
2- Lazar EL, Stolar CJ: Evaluation and management of pediatric solid ovarian tumors. Semin Pediatr Surg 7(1):29-34, 1998
3- Kobayashi RH, Moore TC: Ovarian teratomas in early childhood. J Pediatr Surg 13(4):419-22, 1978
4- Chaung JH, Chen L: Ovarian teratoma with gliomatosis peritonei. J Pediatr Surg 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, 2001
7- Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP: Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study. J Clin Oncol 17(7):2137-43, 1999
8- 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, Castleberry RP: Surgical resection alone is effective treatment for ovarian immature 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

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 by minimal dilatation of the 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 associated with a diameter above six 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 papilla, with partial obstruction of the terminal common bile duct. FFCC is associated with fever, jaundice, abdominal pain, recurrent pancreatitis and altered liver function tests. Histologically FFCC demonstrates thickened fibrous connective tissue, absent muscular layer with flattened, ulcerated and dysplastic mucosa. Diagnosis is established with ultrasound. ERCP or better yet MRCP can help delineate the anatomy and presence of a long common pancreaticobiliary channel in FFCC. Management consists of cyst excision and Roux-en-y hepaticojejunostomy. Due to the small size the anastomosis is technically difficult and should be performed carefully to avoid stricture and postoperative cholangitis. To maintain ductal anastomosis patency it is imperative that diseased ductal tissue not be incorporated in the anastomosis, the circumstance most likely responsible for the high incidence of anastomotic stricture in choledochal cyst past drainage operations.

1- Lilly JR, Stellin GP, Karrer FM: Forme fruste choledochal cyst. J Pediatr Surg 20(4):449-51, 1985
2- Okada A: Forme fruste choledochal 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): 1658-1662, 1995
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(12):1833-6, 2003

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 most instances causes parental concern. Fortunately, most polypoid lesions identified in gallbladders are benign (90%). Histologically they are either adenomatous, hyperplastic, gastric heterotopia or cholesterol polyps. The prevalence of such polyps is greater among males and obese children. Ultrasonography is the image method of choice in diagnosing gallbladders polyps in children and adults. They are seen as pedunculated or sessile echogenic lesions attached to the gallbladder wall protruding toward the lumen and fixed in changed of posture. Gallbladder polyps can be associated with acalculous cholecystitis. Lesions smaller than 10 mm do not progress to malignancy or development of stones, and none produces symptoms or complications of biliary disease. Surgical management of gallbladder polyps is indicated when the size of the polypoid lesion is above 10 mm in diameter, when associated with gallstones and when the child has consistent biliary symptoms. Treatment consists of laparoscopic cholecystectomy. Asymptomatic small polyps (< 10 mm) should be maintained under ultrasonographic surveillance.

1- Yang HL, Sun YG, Wang Z: Polypoid lesions of the gallbladder: diagnosis and indications for surgery. 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 polypoid lesions of the gallbladder smaller than 10 mm. Ann Surg 234(5):657-60, 2001
5- Kikiros C, Arunachalam P, Lam MH: Adenomatous hyperplastic polyp of the gall bladder associated with cholelithiasis in a child. Pediatr Surg Int 19(1-2):118-9, 2003
6- Stringer MD, Ceylan H, Ward K, Wyatt JI: Gallbladder Polyps in Children - Classification and Management. J Pediatr Surg 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 tubes and Crohn's disease. After removing a temporary gastrostomy tube most stomas close between three and six weeks after removal. Persistence of stomach leakage through the gastrostoma is a nuisance, erodes 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 distal obstruction (delayed gastric emptying), foreign body reaction (silk), epithelization of the tract (multiple granulomas formation), or associated chronic granulomatous disease (Crohn). Silk suture should be avoided 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%. Initial non-surgical therapy should include H2-antagonist therapy and silver nitrate cauterization. If this does not work permanent management of GCF consists of surgical closure.

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 gastrostomy. Incidence in infants and
children.  N Y State J Med 74(13):2364-6, 1974
4- 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 17(4):326-8, 2001

Volume 22 No 03 MARCH 2004

Umbilical Granuloma

Persistent umbilical swelling and discharge during the neonatal period is of serious concern to both parents and physicians. Among umbilical swelling, the umbilical granuloma is one of the most commonly seen condition in the pediatric practice. The normal granuloma, a common inflammatory reaction to the resolving umbilical stump of a newborn should disappear by the 2nd to 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 application. Sylver nitrate is 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, careful drying the umbilical exudate to prevent periumbilical spillage, and discussion with parent that burns may occur but apparently are not serious. Whenever Sylver nitrate therapy fails and discharge persists, or contains urine or fecal material, the physician should suspect that the child has either a patent urachus or omphalomesenteric duct remnant as both conditions resemble the common umbilical granuloma seen in general practice. Ultrasound studies of the periumbilical area looking for a cyst, masses or fixed bowel loops can help determine the presence of such congenital remnants. Management of the persistent umbilical granuloma is surgical with double ligature, cauterization of the base or formal umbilical exploration.

1- Campbell J, Beasley SW, McMullin N, Hutson JM: Clinical diagnosis of umbilical swellings and discharges in children. Med J Aust 145(9): 450-3, 1986
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 discharging umbilicus. Pediatr Radiol 26(5): 362-4, 1996
4- Nagar H: Umbilical granuloma: a new approach to an old problem. Pediatr Surg Int 17(7): 513-4, 2001
5- Lotan G, Klin B, Efrati Y: Double-ligature: a treatment for pedunculated umbilical granulomas in children. Am Fam Physician 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 stroma and size of the cysts can 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 the most common form of 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 ipsilateral ureter. Ultrasound is diagnostic of MCDK. Renal scan studies (DMSA) will not concentrate the contrast material. Retrograde studies will show an atretic ureter. The differential diagnosis consists of cystic mesoblastic nephroma which will show some function on excretory urography or nuclear studies different from MCDK. MCDK does not have a premalignant potential. The incidence of short term complications of MCDK is very low. Regional pain caused by the expanding kidney mass is probably the most absolute indication for nephrectomy in MCDK. Relative indications consist of reversible hypertension, symptomatic urinary tract infection and increasing kidney size. Almost 20% of these lesions will regress within the first three years of life of the child.

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 AD, Watts FB Jr, Chang CH: Follow-up studies of multicystic dysplastic 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, Kennedy WA, Shortliffe LD: Serial followup of the contralateral renal size in children with multicystic dysplastic 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% of children with idiopathic constipation respond to medical treatment (bulk diet, laxatives and enemas). Less than 10% develops intractable constipation. Intractable constipation, not associated to Hirschsprung's disease, neuromuscular disease or repaired anorectal malformations, that fails to respond to aggressive medical management is one of the most difficult conditions to manage in children. Children have duration of symptoms for a period beyond five years. Intractable constipation produces progressive fecal retention, fecal incontinence, distension of the rectum and sigmoid colon with loss of rectal sensory and motor function. Encopresis ensues when fecal soiling results from the retained fecal material. Idiopathic constipation is associated with a thickened internal anal sphincter. Colonic manometry helps differentiate causes of intractable constipation in childhood showing the length of the abnormal colonic involved segment. Surgical management for intractable constipation can consist of internal myectomy, placement of cecostomy or left-colon tubes for antegrade enema cleansing, or resection of the disease colonic segment when there is severe stasis and luminal dilatation. Outcomes have thrown mixed results.

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, 1997
3- 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 Pediatr Gastroenterol Nutr 33(5): 588-91, 2001
4- Dey R, Ferguson C, Kenny 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, Atkinson JB: Left-colon antegrade continence enema (LACE) procedure for fecal incontinence. J Pediatr Surg 38(12): 1778-80, 2003
6- Youssef NN, Pensabene L, Barksdale E, Di Lorenzo C: Is there a role for surgery beyond colonic aganglionosis and anorectal malformations in children with intractable constipation? J Pediatr Surg  39 (1): 73-77, 2004

Volume 22 No 04 APRIL 2004

Spigelian Hernias

A spigelian hernia is a rare protrusion of peritoneal sac that occurs through the transversus aponeurosis between the semicircular and lateral border of the rectus sheath below the level of the umbilicus. Spigelian hernias are more common in adults than children. The hernia appears as an intermittent mass in the lower abdominal quadrant and flank seen when the child exerts an increase abdominal pressure. Bowel or omentum can be identified within the hernia content Some children manifests intermittent abdominal pain. Spigelian hernias have been associated with cryptorchidism and neuroblastoma. Diagnosis depends on finding an unusual mass on the anterior abdominal wall and palpation of the rim of the hernia defect upon reduction of the mass. Spontaneous closure has not been reported. Management of spigelian hernias is straightforward: surgical repair when diagnosed to avoid incarceration and strangulation. As in most hernia repairs the defect should be marked prior to anesthesia since it will not be palpable during abdominal wall relaxation. The internal oblique fascia along with the transversalis fascia is closed in an overlapping manner followed by the external oblique fascia preferably with interrupted nonabsorbable sutures. Recurrence of the defect after surgery is extremely rare.

1- Jarvis PA, Seltzer MH: Pediatric Spigelian hernia: a case report. J Pediatr Surg 12(4):609-10, 1977
2- Graivier L, Bronsther B, Feins NR, Mestel AL: Pediatric lateral ventral (spigelian) hernias. South Med J 81(3):325-6, 1988
3- Komura J, Yano H, Uchida M, Shima I: Pediatric spigelian hernia: reports of three cases. Surg 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 spigelian hernia and cryptorchidism: cause or coincidence? Pediatr Surg Int 16(5-6):433-6, 2000
6- White JJ: Concomitant Spigelian and inguinal hernias in a neonate. J Pediatr Surg 37(4):659-60, 2002
7- Losanoff JE, Richman BW, Jones JW: Spigelian hernia in a child: case report 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 mesothelial cells. Since they adhere to the native pericardium, the appearance if these intrathoracic masses in simple chest films are that of cardiomegaly. Pericardial cysts account for 7% of all mediastinal masses in children. Most pericardial cysts are asymptomatic, located in the right cardiophrenic angle and detected incidentally during routine chest films. Symptoms and serious complications such as dyspnea, cough, respiratory distress, chest pain and cardiac tamponade can occur the result of an expanding lesion on vital adjacent structures. The diagnosis of pericardial cysts can be done prenatally using  ultrasonography. Once suspected the diagnosis is established by noninvasive studies such as echocardiography and CT scans. Bronchogenic cysts have similar appearance in CT scans. Management of pericardial cysts is surgical excision whenever possible. The objective of removal of the lesion is elimination of the tumorous mass, relieve of symptoms and allowance of histological examination. Approach can be open or video-assisted thoracoscopic surgery. Prognosis is excellent in most cases.

1- Bini RM, Nath PH, Ceballos R, Bargeron LM Jr, Kirklin JK: Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn. Pediatr Cardiol  8(1):47-50, 1987
2- Abad C, Rey A, Feijoo J, Gonzalez G, Martin-Suarez J: Pericardial cyst. Surgical resection in two symptomatic cases. J Cardiovasc Surg (Torino) 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, Nagashima A: Pericardial cyst in a child treated with video-assisted thoracoscopic surgery. Eur J Pediatr 159(12):889-91, 2000
5- Noyes BE, Weber T, Vogler C: Pericardial cysts in children: surgical or conservative approach?  J Pediatr Surg 38(8):1263-5, 2003

Total Urogenital Mobilization

Total urogenital mobilization (TUM) was initially described by Peña in 1997 to technically ease the surgical management of persistent cloaca. Specifically cloacas with common channels less 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, consists of total dissection 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 the perineum. This innovative technical approach reduces operating time and improves final cosmetic appearance. Furthermore, TUM can reduce the incidence of postoperative complications such as urethrovaginal fistulas, vaginal stricture and acquired vaginal atresia. Following this initial report the technique has been expanded to include cases of congenital adrenal hyperplasia with urogenital sinus (Prader Classification II, III and IV), female bladder exstrophy/epispadia and penile agenesis. When the urogenital sinus is not associated with a cloacal deformity, the procedure can be 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 urinary continence can be preserved.

1- Peña A: Total urogenital mobilization--an easier way to repair cloacas. J Pediatr Surg 32(2):263-7, 1997
2- Ludwikowski B, Oesch Hayward I, Gonzalez R: Total urogenital sinus 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 urogenital sinus mobilization: a modified perineal approach for feminizing  genitoplasty and urogenital 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):1656-8, 2001
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 Suppl):S47, 2003

Volume 22 No 05 MAY 2004


Pyomyositis is a purulent infection of skeletal muscle commonly seen in children who live in tropical countries. For pyomyositis to develop initial muscle injury followed by bacteremia must coexist. The initial traumatic event causes a localized cutaneous infection which is the source of the bacteremia that seeds the injured muscle tissue. Pyomyositis is more common in males, especially those who participate in strenuous physical activity. Peak incidence occurs between two and five years of age in children. Associated conditions (60%) includes diabetes, liver disease and HIV. Clinically, pyomyositis is accompanied by abscess formation in the suppurative phase (fluctuance) but may be without a focal fluid collection in the presuppurative phase (pain, fever, cellulitis, indurated muscle). Most common sites of abscess formation are the quadriceps and gluteal muscles. Organisms more commonly associated with pyomyositis are Staphylococcal Aureus which affects 90% of cases and streptococcus species. MRI is the most accurate means of diagnosing a lesion within muscle determining location and extension of the lesion. Initial management consists of systemic antibiotics. Surgical drainage and debridement are of paramount importance in the management of pyomyositis. All specimens obtained by aspiration or drainage should be cultured for aerobic and anaerobic bacteria. In immunocompromised patients progression to the septicemic stage is associated with high morbidity and mortality.

1- Meehan J, Grose C, Soper RT, Kimura K: Pyomyositis in an adolescent female athlete. J Pediatr Surg 30(1):127-8, 1995
2- Brook I: Pyomyositis in children, caused by anaerobic bacteria. J Pediatr Surg 31(3):394-6, 1996
3- Akman I, Ostrov B, Varma BK, Keenan G: Pyomyositis: report of three patients and review of the literature. Clin Pediatr (Phila) 35(8):397-401, 1996
4- Ameh EA: Pyomyositis in children: analysis of 31 cases. Ann Trop Paediatr 19(3):263-5, 1999
5- Bibbo C, Patel DV, Mackessy RP, Lin SS, Barricella RL: Pyomyositis of the leg with early neurologic compromise. Pediatr Emerg Care 16(5):352-4, 2000
6- Flier S, Dolgin SE, Saphir RL, Shlasko E, Midulla P: A case confirming the progressive stages of pyomyositis. J Pediatr 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 presents with stridor, respiratory distress, recurrent pulmonary infections or failure to thrive. Inflammation of the mucosa or mucous accumulation can easily obstruct the already stenotic airway. The stenosis includes a short or long segment of circular cartilaginous ring. Diagnosis is established using bronchoscopy, MRI (assessment of vascular structures and relation to the stenosis), or CT-scan (good anatomic delineation of the airway). Each individual malformation is studied using rigid tracheo-broncho- esophagoscopy. Associated cardiac defects should rule out with echocardiogram. Management 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 stenosis with anastomosis can be possible with a length that does not exceed half of the trachea. For longer stenosis the most useful technique consists of enlargement tracheoplasty with cartilaginous or a pericardial graft or the more recent and slide-tracheoplasty. The latter technique is preferable because it preserves native tracheal tissue with fewer postop complications. For CTS one should always look for other associated thoracic malformations, such as a pulmonary sling which may compromise the results of the surgical correction of the tracheal stenosis. Best prognosis is obtained with simultaneous correction of the respiratory and cardiovascular malformation. Overall survival of these children is 75%.

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 after slide tracheoplasty. J Thorac Cardiovasc Surg 123(1):145-52, 2002
4- Backer CL, Mavroudis C, Holinger LD: Repair of congenital tracheal stenosis. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5:173-86, 2002
5- Rutter MJ, Cotton RT, Azizkhan RG, Manning PB: Slide tracheoplasty for the management of complete tracheal 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
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 extending through the underlying 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 the scalp the lesion can 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 ACC are normal. Pathogenesis 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 single and less than two centimeters in diameter. Management of ACC is conservative for small lesions with excision and primary closure. A larger lesion might need split-thickness skin grafting or tissue expansion technique for closure.

1- Vinocur CD, Weintraub WH, Wilensky RJ, Coran AG, Dingman RO: Surgical management of aplasia cutis congenita. Arch Surg 111(10):1160-4, 1976
2- Sargent LA: Aplasia cutis congenita of the scalp. J Pediatr Surg 25(12):1211-3, 1990
3- Ross DA, Laurie SW, Coombs CJ, Mutimer KL: Aplasia cutis congenita: failed conservative treatment. Plast Reconstr 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, 2001
5- Verhelle NAC, Heymans O, Deleuze JP, Fabre G, Vranckx JJ, Van den hof B: Abdominal Aplasia Cutis Congenita: Case Report and Review of the 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 affected with FH presents early in life with persistent postprandial vomiting and failure to thrive, signs which are undistinguishable from hypertrophied pyloric stenosis. The characteristic histology of faveolar hyperplasia consists of enlarged, tortuous and dilated gastric pits (foveolas), producing a redundant mucosa that causes partial obstruction of the antro-pyloric area. Associated is submucosal eosinophilic inflammatory reaction suggesting an allergic component. Some reports have suggested cows' milk protein allergy as a key factor. In general, the etiology of FH is unknown. A few reports have found that foveolar hyperplasia develops after prostaglandin E infusion, an effect which is dose related, and resolves with cessation of the drug. Ultrasound of the antro-pyloric canal will demonstrate a filling defect, polypoidal, redundant lesion with central echogenic folds without muscular wall thickening. UGIS shows a longitudinal filling defect. Upper endoscopy reveals polypoidal mucosal hypertrophy originating from antrum and extending into the duodenal cap. Biopsy establishes the diagnosis. Management  of symptomatic idiopathic focal foveolar hyperplasia consists of surgical excision of the involved redundant mucosa with pyloroplasty or pyloromyotomy.

1- Katz ME, Blocker SH, McAlister WH: Focal foveolar hyperplasia presenting as an antral-pyloric mass in a young infant. Pediatr 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 AJ, Freeman JK, Le Quesne GW, Khong TY: Idiopathic focal foveolar hyperplasia in infants. Pediatr 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, 1998
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, trauma, congenital heart disease, sepsis, dehydration, coagulopathies, venous occlusion from direct pressure, constrictive bands, compression by the encircling umbilical cord, and venipuncture are all possible causes which should be considered in the differential diagnosis of congenital gangrenous extremity. Unfortunately in most cases the etiology cannot be established. When gangrene is established at birth surgical amputation, autoamputation, or some loss of function is usual. Management is in general supportive, allowing the ischemic area to demarcate and slough. Range-of-motion exercises and splinting to avoid contracture are helpful in the rehabilitative phase. In very rare occasions early aggressive systemic thrombolytic therapy (urokinase) followed by serial soft-tissue debridement and ultimate skin coverage through cultured epithelial autografts have been reported with good limb salvage results. Peripheral Ischaemic insults presenting at birth may be part of a wider spectrum of disorders, both prenatal and perinatal, attributable to occlusive vascular disruption.

1- Hensinger RN: Gangrene of the newborn. A case report. J Bone Joint Surg Am 57(1):121-3, 1975
2- Nazer H, Abu Rajab A, Qaryouti S, Tarawneh M, Hamzeh Y, Arda H, Mustafa M: Neonatal limb gangrene and renal vein thrombosis. Case report with review of literature. Eur J Pediatr  146(4):429-31, 1987
3- Turnpenny PD, Stahl S, Bowers D, Bingham P: Peripheral ischaemia and gangrene presenting at birth. Eur J Pediatr 151(8):550-4, 1992
4- Ricciardelli E, Morgan RF, Lin KY: In utero brachial artery thrombosis: limb salvage with postnatal urokinase infusion. Ann Plast 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 with non-tender swelling of the 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 capillary and bluish involvement 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 lack of exposure to ionizing 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 histologic nature of the mass. US with Doppler imaging (lobular internal structure, fine echogenic internal septations, mildly lobulated contour and extremely high vascularity), and labeled red cell scintigraphy (well-defined area of intense activity) can also sustain the diagnosis of parotid hemangioma. Management is conservative since most lesions involute spontaneously. During involution ulceration and calcification can occur. Medical management (intralesional injection of steroids, systemic steroids or interferon) is given when the tumor is large, deforming, ulcerated, or involves nearby structures with functional consequences. The overall response rate is very high.

1- Tresserra L, Martinez-Mora J, Boix-Ochoa J: Haemangiomas of the parotid gland in children. J Maxillofac Surg 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
3- Huchzermeyer P, Birchall MA, Kendall B, Bailey CM: Parotid haemangiomas in childhood: a case for MRI. J Laryngol Otol 108(10):892-5, 1994
4- Roebuck DJ, Ahuja AT: Hemangioendothelioma of the parotid gland in infants: sonography and correlative MR imaging. AJNR Am J Neuroradiol 21(1):219-23, 2000
5- Esposito C, Zupi A, Califano L: Surgical therapy of parotid hemangiomas. Pediatr Surg Int 17(5-6):335-7, 2000
6- Greene AK, Rogers GF, Mulliken JB: Management of parotid hemangioma in 100 children. Plast Reconstr Surg 113(1):53-60, 2004

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