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PEDIATRIC SURGERY UPDATE 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 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, re 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 dis respiratory distress and decompress proximal esophageal pouch (Reploge tube). Evaluate for associated conditions such as VACTERL association. Early surgical repair ( extrapleural) is undertaken for those babies with adequate arterial blood gases, adequate weight (>1200 gm) and no significant associated anomalies. Delayed repair (gast other patients. Repair consists of muscle-sparing thoracotomy, closure of TEF and primary anastomosis. Esophagogram is done 7-10 days after repair. Most important pre birth weight, severity of pulmonary dysfuntion, and presence of major congenital cardiac disease. Complications after surgery: anastomotic leak, stricture, gastroesophage tracheomalacia and recurrent TEF. Increase survival is associated with improvements in perioperative care, meticulous surgical technique and aggressive treatment of ass
References 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 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

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 ben 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 diaphoresis, anxiety, dizziness, seizures and even coma. Aggressive management is mandatory to avoid permanent sequelae. Whipple's triad: symptoms of hypoglycemia w 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 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 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 tum 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% gla cure (multiple microadenomatosis).
References 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 fa dominant syndrome. Mucocutaneous melanin deposits include small blacks or brown dark spots around the lips, buccal mucosa, fingers and toes usually identified during in large, pedunculated with high malignant potential (48%) later in adult life (cancer development is 18 times greater than general population). Clinical presentation may inclu abdominal colicky pain, gastrointestinal bleeding, obstruction, rectal prolapse, anemia and intussusception. Most polyps affect the jejunum and are multiple, although they 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 intervent intraoperative endoscopy with polypectomy may prevent the development of short bowel syndrome.
References 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 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

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

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, level 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 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 suppor expertise, adequate instrumentation and technical experience surgeons are managing HD as a single procedure early in life with identical results to the traditional approac 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 o the laparoscopic) primary PT procedure has shortened the hospital stay, decreased morbidity (that associated with a colostomy) and produce earlier intestinal continuity. T 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: perf
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megacolon, severe enterocolitis, questionable pathology, unavailable frozen section and in the premature infant.
References 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 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

Typhlitis (also known as neutropenic enterocolitis) refer to a necrotizing inflammatory process seen in myelosuppressed patients with malignancy (prevalence = 5-9%) wh chemotherapy-induced intestinal wall damage affecting primarily the ileo-cecal region and ascending colon. Typhlitis is most frequent in patients treated for acute leukemi intensity of chemotherapeutic regimens may account for a marked increase in the incidence of typhlitis over the past five years. It sometime mimics appendicitis character RLQ abdominal pain, tenderness, nausea, diarrhea and lower GI bleeding. Chemotherapy causes agranulocytosis, intestinal stasis and ischemia with resultant secondary b 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 i 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 suc TPN and selective use of antibiotics. Clinical deterioration, failure to improve promptly, persistent peritoneal findings and evidence of pneumatosis are indications for surg colectomy with diverting ileostomy is usually the most appropriate procedure.
References 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. accomplished with initial ultrasound-guided percutaneous drainage followed by single session ethanol injection of the cyst cavity. The concentration of ethanol should be be 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 sign 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 p elevation, and hemorrhage into the cyst have been reported. Other series have reported a 75% disappearance rate with minimal morbidity and mortality recommending th for all patients with symptomatic hepatic cysts. Other sclerosants used are tetracycline and doxycycline.

References 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 a 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 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. 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 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 wa a few series have retrospectively compared results between both approaches (open and laparoscopic). Major advantage of the laparoscopic technique is in wound cosmesi 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, curve and operating time. The most dreaded complication is duodenal perforation during the procedure since this changes the morbidity and hospital stay. Difficulties in de 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 re 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 assoc of tumor delivery, more gastric manipulation (atony) and a higher infection rate.
References 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 gu 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 sin 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 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. M 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-loo 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 proximal diversion, but the higher the enterostomy the greater the problems of fluid losses, electrolyte imbalances, skin excoriation, sepsis and malnutrition. Closure of th
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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.

References 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, geni retroperitoneum, mediastinum, heart, gastrointestinal tract and perianal region). Primary breast location is extremely rare. World review of 26 cases found four of these pa 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 p 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 ex 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 th

References 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: Primar 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

VACTERL

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 anom vertebrae), A = anorectal malformation (imperforate anus), C = congenital cardiac defects (VSD, ASD, Tetralogy of Fallot), TE = trachea-esophageal anomalies (esophage 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. Oth problems are a single umbilical artery, duodenal atresia, Meckel's diverticulum. Cases are preferentially males, with higher perinatal mortality rates, higher frequency of pregnancies and lower mean birth weights. Most patients have normal brain function. VACTERL is generally described whenever an infant born with esophageal atresia h 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 mo 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 com nonlimb anomalies. Growth retardation can be seen during the first three years of life. Despite multiple operative procedures infants with VACTERL association may lead normal life.

References 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 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-v (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, cysto and pelvic/chest CT-Scan. Since the tumor is very chemosensitive most patients are managed with primary combination chemotherapy (pulse VAC plus adriamycin and cis biopsy and chemotherapy without resection may be adequate for many patients. Radiotherapy and surgical resection are reserved for cases with residual disease. Sequela (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 Although late relapses do occur, they can usually be salvaged by a combination of chemotherapy, radiotherapy and surgery.

References 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 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 rha 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 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, pancreatic cyst, hepatic cyst, choledochal cyst or duplication cyst. Hemorrhagic pseudocyst (adrenal hemorrhage) accounts for more than 80% of all adrenal cystic lesions 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 N 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 H
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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 complicat 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 metastasi indications for surgery until they fail to resolve after several weeks of follow-up.

References 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 t because they are foreign, damage vessel wall, disrupt blood flow and contain damaging infused substances. Thrombotic complications related to catheters include occlusion 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 n 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 (doppler ultrasound) or more accurately a venogram. High risk patients for DVT are: young age, long-term users (home parenteral nutrition and chemotherapy), cystic fibr atrial shunts. Potential effective prophylactic anticoagulant therapy may prevent both short-term and long-term problems. Most DVT occurs in the upper venous system. C associated to DVT are: loss of venous access, pulmonary embolism, chylothorax, superior vena cava syndrome, postphlebitis syndrome and death. Flushing the blocked ca 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 diagn options include heparin therapy followed by oral anticoagulant therapy, and in some patients thrombolytic therapy (streptokinase) followed by anticoagulant therapy (hepa Catheter removal is controversial.

References 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. Anesth 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

Omphalocele

The three most common abdominal wall defect in newborns are umbilical hernia, gastroschisis and omphalocele. Omphalocele is a milder form of primary abdominoschisis 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 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 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 c incidence (30-60%)of associated anomalies in patients with omphalocele. Epigastric localized omphalocele are associated with sternal and intracardiac defects (i.e., Pental hypogastric omphalocele have a high association with genito-urinary defects (i.e., Cloacal Exstrophy). All have malrotation. Cardiac, neurogenic, genitourinary, skeletal an changes and syndromes are the cornerstones of mortality. Antenatal diagnosis may affect management by stimulating search for associated anomalies and changing the s of delivery. Cesarean section is warranted in large omphaloceles to avoid liver damage and dystocia. After initial stabilization management requires consideration of the si prematurity and associated anomalies. Primary closure with correction of the malrotation should be attempted whenever possible. If this is not possible, then a plastic mes 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 co sepsis, respiratory status, liver and bowel dysfunction from increased intraabdominal pressure.

References 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- Fernndez 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 D a four kilogram newborn girl. To purchase a copy of the video e-mail Humberto Lugo-Vicente at titolugo@coqui.net 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
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PSU Vol 11, 1997

Communication through electronic networks is becoming the most useful resource of Internet by health care workers. To establish the demographic and professional profil Internet service provider of physician members of the Surgery Section (SS) of the American Academy of Pediatrics (AAP) a survey questionnaire that included variables o 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 grou 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 (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 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). T 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 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 ser high number of young members in this practice setting.

References 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) originate 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 duct. Two types of annular pancreas are recognized: 1) Extramural - causing high gastrointestinal obstruction; vomiting is the most common presenting symptom, and 2) In duodenal ulceration. Presentation at birth is affected by the degree of duodenal obstruction and coexistent anomalies. Polyhydramnios usually accompanies complete high obstruction in annular pancreas. Associated anomalies can range from malrotation, intrinsic duodenal obstruction, Down syndrome and duodenal bands. ERCP is the most to find the characteristic features and establish the therapeutic strategy in cases of annular pancreas. Experience militates against any direct attack on the offending annu children with this abnormality are generally treated with a bypass procedure, preferably a duodeno-duodenostomy. Long-term complications may include cholestatic jaundi gastrointestinal motility disorder, failure to thrive, chronic diarrhea and chronic relapsing pancreatitis due to an incomplete divisum anomaly.

References 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 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). Presentin 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 th 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 excision with or without intestinal resection is the next logic step in management. Prognosis is favorable.

References 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

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

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