VOLUME 11, 1998

Volume 11 No 01 JULY 1998

Esophagel Atresia and Tracheo-Esophageal Fistula

Esophageal atresia (EA) with distal tracheo-esophageal fistula (TEF) is the most common congenital anomaly of the esophagus, followed by EA without TEF also known as pure esophageal atresia and pure TEF. Incidence is one in every 2500 live births. Polyhydramnios is most commonly seen in pure EA. EA causes excessive salivation, choking, coughing, regurgitation with first feed and inability to pass a feeding tube into the stomach. Contrast studies are rarely needed and of potential disaster (aspiration). Correct dehydration, acid-base disturbances, respiratory distress and decompress proximal esophageal pouch (Reploge tube). Evaluate for associated conditions such as VACTERL association. Early surgical repair (transpleural or extrapleural) is undertaken for those babies with adequate arterial blood gases, adequate weight (>1200 gm) and no significant associated anomalies. Delayed repair (gastrostomy first) for all other patients. Repair consists of muscle-sparing thoracotomy, closure of TEF and primary anastomosis. Esophagogram is done 7-10 days after repair. Most important predictors of outcome: birth weight, severity of pulmonary dysfuntion, and presence of major congenital cardiac disease. Complications after surgery: anastomotic leak, stricture, gastroesophageal reflux, tracheomalacia and recurrent TEF. Increase survival is associated with improvements in perioperative care, meticulous surgical technique and aggressive treatment of associated anomalies.

1- Okada A,Usui N, Inoue M, Kawahara H, Kubota A, Imura K, Kamata S: Esophageal atresia in Osaka: a review of 39 years' experience. J Pediatr Surg 32(11):1570-4, 1997
2- Tsai JY, Berkery L, Wesson DE, Redo SF, Spigland NA: Esophageal atresia and tracheoesophageal fistula: surgical experience over two decades. Ann Thorac Surg 64(3):778-83; discussion 783-4, 1997
3- Engum SA, Grosfeld JL, West KW, Rescorla FJ, Scherer LR 3rd: Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades. Arch Surg 130(5):502-8; discussion 508-9, 1995
4- Spitz L, Kiely EM, Morecroft JA, Drake DP: Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg 29(6):723-5, 1994
5- Spitz L: Esophageal atresia and tracheoesophageal fistula in children. Curr Opin Pediatr 5(3):347-52, 1993
6- Poenaru D, Laberge JM, Neilson IR, Guttman FM: A new prognostic classification for esophageal atresia. Surgery 113(4):426-32, 1993


Insulinoma, a rare islet beta-cell adenoma, is the most common pancreatic endocrine tumor in children.  The vast majority are sporadic in nature (90%) consisting of a benign solitary small intra-pancreatic nodule. The rest of the time insulinomas are associated to familial syndromes (MEN type 1) with multiple localization within the gland. Insulinomas cause hypoglycemia, diaphoresis, anxiety, dizziness, seizures and even coma. Aggressive management is mandatory to avoid permanent sequelae. Whipple's triad: symptoms of hypoglycemia while fasting, serum glucose level less than 40 mg% and resolution of the symptoms after sugar administration suggest the diagnosis. Measurement of insulin level while fasting produced by a plasma insulin to glucose ratio greater than 1.0 is diagnostic. Preop localization of the tumor is generally difficult to achieve (CT-Scan, selective arteriography and MRI) and believed to be neither indicated nor cost-effective. At surgery the tumor is pink, firm, discrete and well-encapsulated being amenable to enucleation. Intra-operative US localization or palpation of the tumor is essential to resect the tumor or perform a distal pancreatectomy. Once the tumor is eradicated, the child is cured and the prognosis is excellent. Familial syndromes may need 95% gland resection for cure (multiple microadenomatosis).

1- Huai JC, Zhang W, Niu HO, Su ZX, McNamara JJ, Machi J: Localization and surgical treatment of pancreatic insulinomas guided by intraoperative ultrasound. Am J Surg 175(1):18-21, 1998
2- Doski JJ, Robertson FM, Cheu HW: Endocrine Tumors, In Andrassy ‘Pediatric Surgical Oncology', WB Saunders Co, 2nd ed, 1998, pag. 380-381
3- van Heerden JA, Grant CS, Czako PF, Service FJ, Charboneau JW: Occult functioning insulinomas: which localizing studies are indicated? Surgery 112(6):1010-4; discussion 1014-5, 1992
4- Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM, Shandling B: A 20-year review of pediatric pancreatic tumors. J Pediatr Surg 27(10):1315-7, 1992
5- Grosfeld JL, Vane DW, Rescorla FJ, McGuire W, West KW: Pancreatic tumors in childhood: analysis of 13 cases. J Pediatr Surg 25(10):1057-62, 1990
6- Zuppinger K: Disorders of the endocrine pancreas. Prog Pediatr Surg 16:51-61, 1983

Peutz-Jeguers Syndrome

Peutz-Jeghers represent the association of benign hamartomatous polyps of the gastrointestinal tract with abnormal pigmentation of the mouth and skin transmitted as a familial autosomal dominant syndrome. Mucocutaneous melanin deposits include small blacks or brown dark spots around the lips, buccal mucosa, fingers and toes usually identified during infancy. Polyps are large, pedunculated with high malignant potential (48%) later in adult life (cancer development is 18 times greater than general population). Clinical presentation may include recurrent abdominal colicky pain, gastrointestinal bleeding, obstruction, rectal prolapse, anemia and intussusception. Most polyps affect the jejunum and are multiple, although they are also found in the stomach, small bowel, and colon. Management is dictated by symptoms. A cautious approach is advised to preserve as much bowel length as possible. If surgical intervention is necessary, intraoperative endoscopy with polypectomy may prevent the development of short bowel syndrome.

1- Tovar JA, Eizaguirre I, Albert A, Jimenez J: Peutz-Jeghers syndrome in children: report of two cases and review of the literature. J Pediatr Surg 18(1):1-6, 1983
2- Giardiello FM, Welsh SB, Hamilton SR, Offerhaus GJ, Gittelsohn AM, Booker SV, Krush AJ, Yardley JH, Luk GD: Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med 11;316(24):1511-4, 1987
3- Foley TR, McGarrity TJ, Abt AB: Peutz-Jeghers syndrome: a clinicopathologic survey of the "Harrisburg family" with a 49-year follow-up. Gastroenterology 95(6):1535-40, 1988
4-  Buck JL, Harned RK, Lichtenstein JE, Sobin LH: Peutz-Jeghers syndrome. Radiographics 12(2):365-78, 1992
5- Evans M: Peutz-Jeghers syndrome. Can J Surg 38(3):209, 1995 

Volume 11 No 02 AUGUST 1998

Hirschsprung's Disease: Colostomy?

Hirschsprung's disease (HD) or the absence of ganglion cells in the distal bowel has traditionally been managed as a three-stage procedure: diagnostic rectal biopsy, leveling colostomy where ganglion cells are present followed by a pull-through (PT) procedure later in life (six months to one year). Historic arguments considered in avoiding a primary neonatal PT were: increased mortality, the limited pelvic size, fragility of neonatal bowel, risk of pelvic nerve damage and injury to muscular sphincters. With the advent of better intensive care support, pathological expertise, adequate instrumentation and technical experience surgeons are managing HD as a single procedure early in life with identical results to the traditional approach. This entails early diagnosis with imaging and suction rectal biopsy, the use of rectal irrigation washout for decompression, and a PT procedure during the same hospitalization. Overall, the open (and recently the laparoscopic) primary PT procedure has shortened the hospital stay, decreased morbidity (that associated with a colostomy) and produce earlier intestinal continuity. This can be accomplished during the first week of life when the weight of the infant is above the four kilograms. Colostomy in the setting of HD will then be needed for cases with: perforation, toxic megacolon, severe enterocolitis, questionable pathology, unavailable frozen section and in the premature infant.

1- Teitelbaum DH, Coran AG: Primary Pull-Through in the Newborn. Sem Pediatr Surg 7(2): 103-107, 1998
1- Hackam DJ, Pearl RH, Superina RA: Single-stage repair of Hirschsprung's disease: a comparison of 109 patients over 5 years. J Pediatr Surg 32(7):1028-31, 1997
2- Pierro A, Spitz L, Drake D, Kiely EM, Fasoli L: Staged pull-through for rectosigmoid Hirschsprung's disease is not safer than primary pull-through. J Pediatr Surg 32(3):505-9, 1997
3- Wilcox DT, Bianchi A, Bowen J, Bruce J: One-stage neonatal pull-through to treat Hirschsprung's disease. J Pediatr Surg 32(2):243-5, 1997
4- Nour S, Stringer MD, Beck J: Colostomy complications in infants and children. Ann R Coll Surg Engl 78(6):526-30, 1996
5- Langer JC, Lau GY, Ternberg JL, Skinner MA, Foglia RP, Srinathan SK, Winthrop AL, Fitzgerald PG: One-stage versus two-stage Soave pull-through for Hirschsprung's disease in the first year of life. J Pediatr Surg 31(1):33-6, 1996
6- Cilley RE, Coran AG, Hirschl RB, Statter MB: Definitive treatment of Hirschsprung's disease in the newborn with a one-stage procedure. Surgery 115(5):551-6, 1994
7- Rescorla FJ, Grosfeld JL, West KW, Engles D, Morrison AM: Hirschsprung's disease. Evaluation of mortality and long-term function in 260 cases. Arch Surg 127(8):934-41, 1992


Typhlitis  (also known as neutropenic enterocolitis) refer to a necrotizing inflammatory process seen in myelosuppressed patients with malignancy (prevalence = 5-9%) who have chemotherapy-induced intestinal wall damage affecting primarily the ileo-cecal region and ascending colon. Typhlitis is most frequent in patients treated for acute leukemias. Increased intensity of chemotherapeutic regimens may account for a marked increase in the incidence of typhlitis over the past five years. It sometime mimics appendicitis characterized with fever, RLQ abdominal pain, tenderness, nausea, diarrhea and lower GI bleeding. Chemotherapy causes agranulocytosis, intestinal stasis and ischemia with resultant secondary bacteria bowel wall invasion. Typhlitis begin five to 7 days after neutropenia is established. Physical findings are those of abdominal distension and diffuse tenderness. The KUB might show ileus, pneumatosis intestinalis or frank perforation. The CT-Scan demonstrates thickening of the cecal wall, with or without pneumatosis. Most cases can be effectively managed with NG suction, bowel rest, TPN and selective use of antibiotics. Clinical deterioration, failure to improve promptly, persistent peritoneal findings and evidence of pneumatosis are indications for surgery. Right partial colectomy with diverting ileostomy is usually the most appropriate procedure.

1- Bensard DD, Haase GM: Special Considerations for the Neurologically and Immunologically Impaired Child. Sem Pediatr Surg 6(2): 92-99, 1998
2- Ojala AE, Lanning FP, Lanning BM: Abdominal ultrasound findings during and after treatment of childhood acute lymphoblastic leukemia. Med Pediatr Oncol 29(4):266-71, 1997
3- Sloas MM, Flynn PM, Kaste SC; Patrick CC: Typhlitis in children with cancer: a 30-year experience. Clin Infect Dis 17(3):484-90, 1993
4- Katz JA, Wagner ML, Gresik MV, Mahoney DH Jr, Fernbach DJ: Typhlitis. An 18-year experience and postmortem review. Cancer 65(4):1041-7, 1990

Hepatic Cysts: Sclerotherapy

Benign congenital symptomatic (non-neoplastic) simple hepatic cysts not amenable to surgical therapy can be alternatively managed with ethyl alcohol (ethanol) sclerosis. This can be accomplished with initial ultrasound-guided percutaneous drainage followed by single session ethanol injection of the cyst cavity. The concentration of ethanol should be between 80-95%, at a dose of 10-25% of the cyst volume (never more than 100 cc) applied through the catheter for a short period. During injection and for the next hours monitoring of vital signs, alcohol blood levels, liver function tests and level of consciousness will be needed since some of this alcohol might be absorbed into the blood stream. Minor complications of transient pain, temperature elevation, and hemorrhage into the cyst have been reported. Other series have reported a 75% disappearance rate with minimal morbidity and mortality recommending this as initial therapy for all patients with symptomatic hepatic cysts. Other sclerosants used are tetracycline and doxycycline.

1- Larssen TB, Horn A, Rokke O, Sondenaa K, Jensen DK,Viste A: Single-session alcohol sclerotherapy in benign symptomatic hepatic cysts. Acta Radiol 38(6):993-7, 1997
2- Tikkakoski T, Kairaluoma MI, Siniluoto T,Karttunen A, Merikanto J, Paivansalo M, Leinonen S, Makela JT: Treatment of symptomatic congenital hepatic cysts with single-session percutaneous drainage and ethanol sclerosis: technique and outcome. J Vasc Interv Radiol 7(2):235-9, 1996
3- Montorsi M, Filice C, Mosca F, Rovati V, De Simone M,Rostai R, Bona S, Fumagalli U, Torzilli G: Percutaneous alcohol sclerotherapy of simple hepatic cysts. Results from a multicentre survey in Italy. HPB Surg 8(2):89-94, 1994
4- vanSonnenberg E, Cooperberg PL, O'Laoide R, Casola G,Mathieson JR, D'Agostino HB, Wroblicka JT: Symptomatic hepatic cysts: percutaneous drainage and sclerosis. Radiology 190(2):387-92, 1994
5- Simonetti G, Orlacchio A, Meloni GB, Sergiacomi GL, Profili S: Percutaneous treatment of hepatic cysts by aspiration and sclerotherapy. Cardiovasc Intervent Radiol 16(2):81-4, 1993
6- Kairaluoma MI, Siniluoto T, Kiviniemi H, Paivansalo M, St~ahlberg M, Leinonen A: Percutaneous aspiration and alcohol sclerotherapy for symptomatic hepatic cysts. An alternative to surgical intervention. Ann Surg 210(2):208-15, 1989
7- Valette PJ, Paliard P, Partensky C, Chataing L: [Treatment of hepatic polycystosis by intracystic injection of alcohol]. Gastroenterol Clin Biol 1987 Dec;11(12):898-900
8- Bean WJ, Rodan BA: Hepatic cysts: treatment with alcohol. AJR Am J Roentgenol 144(2):237-41, 1985

Vol 11 No 03 SEPTEMBER 1998

Laparoscopic Pyloromyotomy

Infantile pyloric stenosis (PS), the most common abdominal surgical condition in infancy, has been traditionally managed with an open myotomy from antrum to short of the pyloro-duodenal border (Fredet-Ramstedt's) since early century with excellent results. In 1991 the suggestion that the procedure could benefit from the video-endosurgical point of view was posed. Since then a few series have retrospectively compared results between both approaches (open and laparoscopic). Major advantage of the laparoscopic technique is in wound cosmesis, a theoretical reduction in the incidence of adhesions and a reduced  postoperative wound infection rate. Lap technique is more expensive given the fact on the need of video equipment, training learning curve and operating time. The most dreaded complication is duodenal perforation during the procedure since this changes the morbidity and hospital stay. Difficulties in detecting this complication represent a serious limitation of the lap approach emphasizing the need to inject air through a nasogastric tube to check for leaks. Mucosal perforation is a reason for conversion to the open technique. The number of days spent in the hospital is similar with both techniques. Circumbilical open incisions have similar cosmetic results, but can be associated with problems of tumor delivery, more gastric manipulation (atony) and a higher infection rate.

1- Alain JL, Terrier G, Lansade A, Grousseau D, Longis B, Moulies D: [Pyloric stenosis in infants. New surgical approaches]. Ann Pediatr 38(9):630-2, 1991
2- Alain JL, Terrier G, Grousseau D: Extramucosal pyloromyotomy by laparoscopy. Surg Endosc 5(4):174-5, 1991
3- Najmaldin A, Tan HL: Early experience with laparoscopic pyloromyotomy for infantile hypertrophic pyloric stenosis. J Pediatr Surg 30(1):37-8, 1995
4- Scorpio RJ; Hutson JM; Tan HL: Pyloromyotomy: comparison between laparoscopic and open surgical techniques. J Laparoendosc Surg 5(2):81-4, 1995
5- Greason KL, Lo Sasso B, Downey EC, Thompson WR: Laparoscopic pyloromyotomy for infantile hypertrophic pyloric stenosis: report of 11 cases [see comments]. J Pediatr Surg 30(11):1571-4, 1995
6- Ford WD, Holland AJ, Crameri JA: The learning curve for laparoscopic pyloromyotomy. J Pediatr Surg 32(4):552-4, 1997
7- Bufo AJ, Lobe TE, Schropp KP, Cyr N, Shah R; Merry C: Laparoscopic pyloromyotomy: a safer technique. Pediatr Surg Int 13(4):240-2, 1998
8- Tan KC, Bianchi A: Circumbilical incision for pyloromyotomy. Br J Surg 73: 399, 1968
9- Sitsen E, van der Zee DC, Bax NMA: Is laparoscopic pyloromyotomy superior to open surgery? Surg Endosc 12(6):813-5, 1998

Gastroschisis with Intestinal Atresia

Gastroschisis is  a congenital evisceration of part of the abdominal content through an anterior abdominal wall defect found to the right of the umbilicus. The protruding gut is foreshortened, matted, thickened and covered with a peel. In a few babies (4 to 23%) an intestinal atresia (IA) further complicates the pathology. IA complicating gastroschisis may be single or multiple and may involve the small  or large bowel. The IA might be the result of pressure on the bowel from the edge of the defect (pinching effect) or an intrauterine vascular accident. Rarely, the orifice may be extremely narrow leading to gangrene or complete midgut atresia. In either case the morbidity and mortality of the child is duplicated with the presence of an IA. Management remains controversial. Alternatives depend on the type of closure of the abdominal defect and the severity of the affected bowel. With primary fascial closure and good-looking bowel primary anastomosis is justified. Placement of a silo calls for delayed resection performing a second look operation at a  later stage to save intestinal length. Angry looking dilated bowel prompts for proximal diversion, but the higher the enterostomy the greater the problems of fluid losses, electrolyte imbalances, skin excoriation, sepsis and malnutrition. Closure of the defect and resection with anastomosis two to four weeks later brings good results. Success or failure is related to the length of remaining bowel more than the specific method used.

1- Gornall P: Management of intestinal atresia complicating gastroschisis. J Pediatr Surg 24(6):522-4, 1989
2- Shah R, Woolley MM: Gastroschisis and intestinal atresia. J Pediatr Surg 26(7):788-90, 1991
3- Bhatia AM; Crino JP; Musemeche CA: Gastroschisis complicated by midgut atresia and closure of the defect in utero. J Pediatr Surg 31(9):1288-9, 1996
4- Cusick E, Spicer RD, Beck JM: Small-bowel continuity: a crucial factor in determining survival in gastroschisis. Pediatr Surg Int 12(1):34-7, 1997
5- van Hoorn WA, Hazebroek FW, Molenaar JC: Gastroschisis associated with atresia--a plea for delay in resection. Z Kinderchir 40(6):368-70, 1985
6- Hoehner JC, Ein SH, Kim PCW: Management of gastroschisis with Concomitant Jejuno-Ileal Atresia. J Pediatr Surg 33(6): 885-888, 1998

Breast Rhabdomyosarcoma

Breast rhabdomyosarcomas are more commonly metastatic with primary tumors originating in many possible locations (head, neck, orbit, trunk, extremities, buttock, genitourinary system, retroperitoneum, mediastinum, heart, gastrointestinal tract and perianal region). Primary breast location is extremely rare. World review of 26 cases found four of these patients to be younger than age sixteen. There are several reports of breast rhabdo in which a breast mass is the sole presentation of an occult primary tumor. The tumor commonly shows as a palpable, rounded, movable mass with no skin involvement but rapid increase in size. Management must be governed by the principles used for rhabdomyosarcoma that include wide local excision of the primary lesion and multiagent chemotherapy. Few studies report a familial distribution of certain cancers conforming to the Li-Fraumeni syndrome related to a genetic defect on the p53 gene locus.

1- Howarth CB, Cases JN, Pratt C: Breast Metastasis in Children with Rhabdomyosarcoma Cancer 46:2520-2524, 1980
2- Reale D, Guanino M, Sgroi G, Castelli F, et al: Primary Embryonal Rhabdomyosarcoma of the Breast: Description of a Case. Pathologica  86(1): 98-101, 1994
3- Rogers DA, Lobe TE, Raro BW, Fleming ID, et al: Breast Malignancy in Children. J Pediatr Surg  29(1): 48-51, 1994
4- Grosfeld, JL, Weber TR, Weetman RM, Baehner RL: Rhabdomyosarcoma in Childhood: Analysis of Survival in 98 Cases. J Pediatr Surg 18: 141, 1983 5- Hays DM, Donaldson SS, Shimada H, et al: Primary and metastatic rhabdomyosarcoma in the breast: neoplasms of adolescent females, a report from the Intergroup Rhabdomyosarcoma Study. Med Pediatr Oncol 29(3):181-9, 1997
6- Birch JM, Hartley AL, Blair V, Kelsey AM, Harris M, et al: Cancer in the Families of Children with Soft Tissue Sarcomas. Cancer 66(10): 2239-2248, 1990
7- Herrera LJ, Lugo-Vicente HL: Primary Embryonal Rhabdomyosarcoma of the Breast In An Adolescent Female: A Case Report (In press J Pediatr Surg).

Vol 11 No 04 OCTOBER 1998


The VACTERL association was described by Quan and Smith in 1973 as a group of congenital anomalies with a nonrandom tendency for concurrence. V = vertebral anomalies (hemi- and bifid vertebrae), A = anorectal malformation (imperforate anus), C = congenital cardiac defects (VSD, ASD, Tetralogy of Fallot), TE = trachea-esophageal anomalies (esophageal atresia), R = renal-urinary defects (absent kidney, hypospadia) and L= limb defects (radial dysplasia). The mesodermic defect has been traced to the third week of intrauterine life. Other associated problems are a single umbilical artery, duodenal atresia, Meckel's diverticulum. Cases are preferentially males, with higher perinatal mortality rates, higher frequency of fetal loss in previous pregnancies and lower mean birth weights. Most patients have normal brain function. VACTERL is generally described whenever an infant born with esophageal atresia has two or more of this associated defects. Those associated with vertebral, ribs or sternal anomalies may harbor a high lying upper esophageal pouch. Heart failure is the major cause of mortality. Infants with VACTERL that included both renal anomalies and anorectal atresia is more likely to have genital defects. Preaxial but not other limb defects are associated with any combination of the four nonlimb anomalies. Growth retardation can be seen during the first three years of life. Despite multiple operative procedures infants with VACTERL association may lead a reasonably normal life.

1- Rittler M, Paz JE, Castilla EE: VATERL: an epidemiologic analysis of risk factors. Am J Med Genet 73(2):162-9, 1997
2- Ein SH, Shandling B, Wesson D, Filler RM: Esophageal atresia with distal tracheoesophageal fistula: associated  anomalies and prognosis in the 1980s. J Pediatr Surg 24(10):1055-9, 1989
3- Touloukian RJ, Keller MS: High proximal pouch esophageal atresia with vertebral, rib, and sternal anomalies: an additional component to the VATER association. J Pediatr Surg 23(1 Pt 2):76-9, 1988
4- Fournier JL, Jacquemin J, Farriaux J, Lequien P, Walbaum R, Toursel F,Ponte C, Debeugny P, Fontaine G: [V.A.T.E.R. association and its limits]. J Genet Hum 27(4):265-88, 1979
5- Barnes JC, Smith WL: The VATER Association. Radiology 126(2):445-9, 1978
6- Quan L, Smith DW: The VATER association. Vertebral defects, Anal atresia, T-E fistula with esophageal atresia, Radial and Renal dysplasia: a spectrum of associated defects. J Pediatr 82(1):104-7, 1973

Vaginal Rhabdomyosarcoma

Vaginal rhabdomyosarcoma presents during the first two years of life with vaginal bleeding, discharge and prolapse associated with a vaginal mass. The diagnosis is made by vaginoscopy and biopsy of the lesion. Most cases are of the embryonal histologic subtype (botryoid). The lesion usually arises from the anterior vaginal wall around the embryonic vesico-vaginal septum (urogenital sinus). This means that structure such as bladder, prostate and lower vagina might be involved with tumor extension. Initial work-up must include biopsy, cystoscopy, vaginoscopy and pelvic/chest CT-Scan. Since the tumor is very chemosensitive most patients are managed with primary combination chemotherapy (pulse VAC plus adriamycin and cisplatinum). Repeated biopsy and chemotherapy without resection may be adequate for many patients. Radiotherapy and surgical resection are reserved for cases with residual disease. Sequelae of irradiation (colorectal, vaginal, urethral, and ureteral stenosis) can be seen in these children. Tumors with diameters of five cm or more have a less favorable outcome compared with smaller tumors. Although late relapses do occur, they can usually be salvaged by a combination of chemotherapy, radiotherapy and surgery.

1- Andrassy RJ, Hays DM, Raney RB, Wiener ES, Lawrence W, Lobe TE, Corpon CA, Smith M, Maurer HM: Conservative Surgical Management of Vaginal and Vulvar Pediatric Rhabdomyosarcoma: A Report From the Intergroup Rhabdomyosarcoma Study III. J Pediatr Surg 30(7): 1034-1037, 1995
2- Heij HA, Vos A, de Kraker J, Voute PA: Urogenital rhabdomyosarcoma in children: is a conservative surgical approach justified? J Urol 150(1):165-8, 1993
3- Flamant F, Gerbaulet A, Nihoul-Fekete C, Valteau-Couanet D, Chassagne D, Lemerle J: Long-term sequelae of conservative treatment by surgery, brachytherapy, and chemotherapy for vulval and vaginal rhabdomyosarcoma in children. J Clin Oncol 8(11):1847-53, 1990
4- Hays DM, Shimada H, Raney RB Jr, Tefft M, Newton W, Crist WM, Lawrence W Jr, Ragab A, Beltangady M, Maurer HM: Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents. Cancer 61(9):1893-903, 1988

Cystic Neuroblastoma

Suprarenal masses in the newborn period include a differential of adrenal hemorrhage, simple cyst, abscess, neuroblastoma, renal duplication, hydronephrosis, renal cyst, nephroblastoma, pancreatic cyst, hepatic cyst, choledochal cyst or duplication cyst. Hemorrhagic pseudocyst (adrenal hemorrhage) accounts for more than 80% of all adrenal cystic lesions. Cystic neuroblastoma (NB) is a rare form of neuroblastoma. Development of cysts may be related to a prominent microcytic arrangement of tumor nests. US characteristics of NB are usually of a complex or echogenic mass with a thick complex wall. Solid tissue within the mass suggests the possibility of a tumor. Serial US exam and urinary collection of VMA and HVA is warranted. In situ NB can be an incidental finding in a small percentage of autopsies done for other reason. Therapeutic decisions are dictated by symptoms, size and potential complications. Given the good prognosis of cystic NB in the newborn period it seems feasible to avoid prompt surgery after birth for patients with an adrenal cyst without any evidence of metastasis, reserving indications for surgery until they fail to resolve after several weeks of follow-up.

1- Atkinson GO Jr, Zaatari GS, Lorenzo RL, Gay BB Jr, Garvin AJ: Cystic neuroblastoma in infants: radiographic and pathologic features. AJR Am J Roentgenol 146(1):113-7, 1986
2- Croitoru DP, Sinsky AB, Laberge JM: Cystic Neuroblastoma. J Pediatr Surg 27(10): 1320-1321, 1992
3- Weber T, Sotelo-Avila C, Gale C: Cystic Neuroblastoma in a Newborn. J Pediatr Surg 28(12): 1603-1604, 1993
4- Iuchtman M, Abudi Z, Yurman S, Koren I, Kessler FB: Giant Adrenal Cyst in the Newborn: Perinatal Diagnosis and Management. Eur J Pediatr Surg 4(2): 122-124, 1994
5- Yamagiwa I, Obata K, Saito H: Prenatally detected cystic neuroblastoma. Pediatr Surg Int 13: 215-217, 1998

Vol 11 No 05 NOVEMBER 1998

Deep Venous Thrombosis in CVL

Deep venous thrombosis (DVT) is probably the second most common complication of central venous line (CVL) placement followed very closely by sepsis. Catheters are thrombogenic because they are foreign, damage vessel wall, disrupt blood flow and contain damaging infused substances. Thrombotic complications related to catheters include occlusion (fibrin sleeve) and DVT. Suspicion of DVT in CVL should arise when there is no blood return, increase pressure are needed for infusion, repeat urokinase instillation to remove blockage is needed and the life span is shortened in the face of a recent catheter changed. Symptoms include swelling, pain and discoloration of the face or limb. Diagnosis of DVT can be made using non-invasive techniques (doppler ultrasound) or more accurately a venogram. High risk patients for DVT are: young age, long-term users (home parenteral nutrition and chemotherapy), cystic fibrosis and ventriculo-atrial shunts. Potential effective prophylactic anticoagulant therapy may prevent both short-term and long-term problems. Most DVT occurs in the upper venous system. Complications associated to DVT are: loss of venous access, pulmonary embolism, chylothorax, superior vena cava syndrome, postphlebitis syndrome and death. Flushing the blocked catheter with heparin is insufficient. Randomized trials in adults have shown a reduced incidence of DVT in patients receiving low dose warfarin that did not prolong the INR. Once DVT is diagnosed therapeutic options include heparin therapy followed by oral anticoagulant therapy, and in some patients thrombolytic therapy (streptokinase) followed by anticoagulant therapy (heparin, warfarin). Catheter removal is controversial.

1- Andrew M, Marzinotto V, Pencharz P, Zlotkin S, Burrows P, Ingram J, Adams M, Filler R: J Pediatrics 126: 358-363, 1995
2- Bern MM, Lokich JJ, Wallach SR, et al: Very Low doses of warfarin can prevent thrombosis in central venous catheters: a randomized prospective trial. Ann Intern Med 112: 423-428, 1990
3- David M, Andrew M: Venous thromboembolic complications in children. J Pediatrics 123: 337-346, 1993
4- Moore RA, McNicholas KW, Naidech H, Flicker S, Gallagher JD: Clinically silent venous thrombosis following internal and external jugular central venous cannulation in pediatric cardiac patients. Anesthesiology 62: 640-643, 1985
5- Beck C,Dubois J, Grignon A, Lacroix J, David M: Incidence and risk factors of catheter-related deep vein thrombosis in a pediatric intensive care unit: a prospective study. J Pediatr 133(2):237-41, 1998
6- Kenney BD, David M, Bensoussan AL: Anticoagulation without catheter removal in children with catheter- related central vein thrombosis. J Pediatr Surg 31(6):816-8, 1996


The three most common abdominal wall defect in newborns are umbilical hernia, gastroschisis and omphalocele. Omphalocele is a milder form of primary abdominoschisis since during the embryonic folding process the outgrowth at the umbilical ring is insufficient (shortage in apoptotic cell death). Bowel and/or viscera remains in the umbilical cord causing a large abdominal wall defect. Defect may have liver, spleen, stomach, and bowel in the sac while the abdominal cavity remains underdeveloped in size. The sac is composed of chorium, Wharton's jelly and peritoneum. The defect is centrally localized and measures 4-10 cm in diameter. A small defect of less than 2 cm with bowel inside is referred as a hernia of the umbilical cord. There is a high incidence (30-60%)of associated anomalies in patients with omphalocele. Epigastric localized omphalocele are associated with sternal and intracardiac defects (i.e., Pentalogy of Cantrell), and hypogastric omphalocele have a high association with genito-urinary defects (i.e., Cloacal Exstrophy). All have malrotation. Cardiac, neurogenic, genitourinary, skeletal and chromosomal changes and syndromes are the cornerstones of mortality. Antenatal diagnosis may affect management by  stimulating search for associated anomalies and changing the site, mode or timing of delivery. Cesarean section is warranted in large omphaloceles to avoid liver damage and dystocia. After initial stabilization management requires consideration of the size of defect, prematurity and associated anomalies. Primary closure with correction of the malrotation should be attempted whenever possible. If this is not possible, then a plastic mesh/silastic chimney is fashioned around the defect to cover the intestinal contents and the contents slowly reduced over 5-14 days. Antibiotics and nutritional support are mandatory. Manage control centers around sepsis, respiratory status, liver and bowel dysfunction from increased intraabdominal pressure.

1- Langer JC: Gastroschisis and omphalocele. Semin Pediatr Surg 5(2):124-8, 1996
2- Molenaar JC,Tibboel D: Gastroschisis and omphalocele. World J Surg 17(3):337-41, 1993
3- Krasna IH: Is early fascial closure necessary for omphalocele and gastroschisis? J Pediatr Surg. 30(1): 23-28, 1995
4-  Grosfeld JL, Dawes L, Weber TR: Congenital abdominal wall defects: current management and survival. Surg Clin North Am 61(5):1037-49, 1981
5- Moore TC: Gastroschisis and omphalocele: clinical differences. Surgery  82(5): 561-568, 1977.
6- Fernández MS, et al: Cantrell's pentalogy. Report of four cases and their management. Pediatr Surg Int. 12(5/6): 428-431, 1997
7- Molenaar JC: Cloacal Exstrophy. Semin Pediatr Surg. 5(2): 133-135, 1996

Trans-Anal Pull-Through Video

We recently created a VIDEO of a single stage trans-anal endorectal pull-through (Soave-Boley) procedure without abdominal incision (incisionless) for Hirschsprung's Disease performed in a four kilogram newborn girl.
To purchase a copy of the video e-mail Humberto Lugo-Vicente at or send check/money order ($40 US dollars) to:
Humberto Lugo-Vicente, MD
P.O. Box 10426
Caparra Heights Station
San Juan, Puerto Rico, USA 00922.
Allow 2-4 weeks delivery.

Vol 11 No 6 DECEMBER 1998

Profile Surgery Section AAP

Communication through electronic networks is becoming the most useful resource of Internet by health care workers. To establish the demographic and professional profile as well as  the Internet service provider of physician members of the Surgery Section (SS) of the American Academy of Pediatrics (AAP) a survey questionnaire that included variables of age, gender, years of experience, type of practice and Internet service provider was mailed. Two-hundred and five responses of 588 (35%) were received and analyzed. Mean age of the group was 52 years with 88% males and 12% female (7.5:1). With an average of eighteen years of practice 185 members (90%) have access to Internet and 188 (92%) use it mainly for e-mailing from either home (25%), hospital/office setting (30%) or both (42%). Members are willing to receive section news and correspondence by electronic means 23%, print-mail 34% or both 44%. Overall type of practice is private 21%, University 54% or combined 23%. No access to Internet portrays an older member (57 yrs; p = 0.02) sharing solo (private) practice (p = 0.006). Two-third of Internet service providers is university-based (-edu) or hospital organizations (-org) within a younger age group (48 yrs; p = 0.000001). E-mailing is becoming the preferred method of communication among a substantial number (one-third) of pediatric surgeons' members of the Section of Surgery of the AAP. Net accessibility through University or Children Hospital servers account for the high number of young members in this practice setting.

1- Lugo-Vicente HL:  Profile of Internet Users: Survey of the Surgical Section of the AAP (in press), 1999
2- Wulkan ML, et al: Pediatric surgeons on the Internet: a multi-institutional experience.  J Pediatr Surg. 32(4): 612-614, 1997
3- Lugo-Vicente HL: Role of Internet in Medicine. Bol Asoc Med P R. 89(4-6): 82-87, 1997

Annular Pancreas

Annular pancreas is the most common congenital malformation of the pancreas in association with duodenal atresia. Embryologically the ring formation (annulus) originates from the ventral pancreas primordium (Lecco's theory). The pancreatic duct of the annular tissue passed from the anterior portion to the lateral and posterior portion finally joining with the main pancreatic duct. Two types of annular pancreas are recognized: 1) Extramural - causing high gastrointestinal obstruction; vomiting is the most common presenting symptom, and 2) Intramural - producing duodenal ulceration. Presentation at birth is affected by the degree of duodenal obstruction and coexistent anomalies. Polyhydramnios usually accompanies complete high intestinal obstruction in annular pancreas. Associated anomalies can range from malrotation, intrinsic duodenal obstruction, Down syndrome and duodenal bands. ERCP is the most important procedure to find the characteristic features and establish the therapeutic strategy in cases of annular pancreas. Experience militates against any direct attack on the offending annulus.  Therefore, all children with this abnormality are generally treated with a bypass procedure, preferably a duodeno-duodenostomy. Long-term complications may include cholestatic jaundice, upper gastrointestinal motility disorder, failure to thrive, chronic diarrhea  and chronic relapsing pancreatitis due to an incomplete divisum anomaly.

1- Reinhart RD, Brown JJ, Foglia RP, Aliperti G: MR imaging of annular pancreas. Abdom Imaging 19(4):301-3, 1994
2- Synn AY, Mulvihill SJ, Fonkalsrud EW: Surgical disorders of the pancreas in infancy and childhood. Am J Surg 156(3 Pt 1):201-5, 1988
3-Tolia V, Rao R, Klein M.: Annular pancreas. J Pediatr. 131(1 Pt 1): 14-15, 1997
4- Komura J, Yano H, Tanaka Y; Tsuru T: Annular pancreas associated with pancreaticobiliary maljunction in an infant. Eur J Pediatr Surg 3(4):244-7, 1993
5- Suda K: Immunohistochemical and gross dissection studies of annular pancreas. Acta Pathol Jpn 40(7):505-8, 1990
6- Kiernan PD; ReMine SG; Kiernan PC; ReMine WH: Annular pancreas: Mayo Clinic experience from 1957 to 1976 with review of the literature. Arch Surg 115(1):46-50, 1980
7- Johnston DW: Annular pancreas: a new classification and clinical observations. Can J Surg 21(3):241-4, 1978
8- Merrill JR, Raffensperger JG: Pediatric annular pancreas: twenty years' experience. J Pediatr Surg 11(6):921-5, 1976

Intra-Abdominal Lymphangiomas

Lymphangiomas are benign, cystic, endothelial-lined tumors of congenital origin formed after failure of communication between lymphatic and venous vessels during fetal life. Within the abdomen they can be found in the mesentery, the retroperitoneum or rarely the bowel wall. Most common site is the small bowel mesentery (ileal predominates). Presenting early in life, abdominal pain is the most common symptom followed by vomiting, increased abdominal girth, mass, anemia, anorexia, weight loss and fever. US and CT-Scan will show the septated, multiloculated,  cystic nature of the mass and suggest the diagnosis. Due to the risk of hemorrhage, torsion, obstruction and infection (and most presents as an emergency), complete surgical excision with or without intestinal resection is the next logic step in management. Prognosis is favorable.

1- Steyaert H, Guitard J, Moscovici J, Juricic M, Vaysse P, Juskiewenski S: Abdominal cystic lymphangioma in children: benign lesions that can have a proliferative course. J Pediatr Surg 31(5):677-80, 1996
2- Pang LC: Acute abdominal conditions in mesenteric lymphangioma.  South Med J. 83(4): 467-470, 1990
3- Levine C: Primary disorders of the lymphatic vessels--a unified concept. J Pediatr Surg 24(3):233-40, 1989
4- Chung MA, Brandt ML, St-Vil D, Yazbeck S: Mesenteric Cysts in Children. J Pediatr Surg 26(1): 1306-1308, 1991
5- Kosir MA, Sonnino RE, Gauderer MWL: Pediatric Abdominal Lymphangiomas: A Plea for early recognition. J Pediatr Surg 26(11): 1309-1313, 1991

Journal Club