Splenogonadal fusion: a case report.

Splenogonodal fusion is a rare congenital abnormality. We present the case of a continuous splenogonadal fusion diagnosed in the course of a laparoscopy for left non-palpable testis in a 1-year-old boy. This case illustrates that even though testicular salvage is the recommended approach, various anatomical features might preclude conservation.

European biliary atresia registries: summary of a symposium.

Biliary atresia (BA) is a rare but potentially devastating disease. The European Biliary Atresia Registry (EBAR) was set up to improve data collection and to develop a pan-national and interdisciplinary strategy to improve clinical outcomes. From 2001 to 2005, 100 centers from 22 countries registered with EBAR via its website (www.biliary-atresia.com). In June 2006, the first meeting was held to evaluate results and launch further initiatives. During a 5-year period, 60 centers from 19 European countries and Israel sent completed registration forms for a total of 514 BA patients. Assuming the estimated incidence of BA in Europe is 1:18,000 live births, 35% of the expected 1488 patients from all EBAR participating countries were captured, suggesting that reporting arrangements need improvement. At the meeting, the cumulative evaluation of 928 BA patients including patients from other registries with variable follow-up revealed an overall survival of 78% (range from 41% to 92%), of whom 342 patients (37%) have had liver transplants. Survival with native liver ranged from 14% to 75%. There was a marked variance in reported management and outcome by country (e.g., referral patterns, timing of surgery, centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support for a pan-European registry with coordination of clinical standards, further participation of parent support groups, and implementation of online data entry and multidisciplinary clinical and basic research projects.

[The medico-surgical department of uro-nephrology, dialysis and renal transplantation in the Children's Hospital of Brussels]. / Le département médico-chirurgical d'uro-néphrologie, dialyse et transplantation rénale à l'HUDERF.

The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, various sectors have been developed including: hemodialysis and peritoneal dialysis, kidney transplantation, urological and genital surgery, antenatal screening and rapid management of uronephropathies, treatment of voiding dysfunction and neurogenic bladder, management of tubular and glomerular diseases. The progress in genetics, medical imaging, obstetrics, neonatology and surgery has allowed us to take care of our young patients within a multidisciplinary framework. The most original contributions of the department are related to the performance of combined liver-kidney transplantation in primary hyperoxaluria, to the determination of the natural history of several congenital anomalies of the kidney and urinary tract, to the assessment of the role of genetical mutations on tubular and glomerular diseases, to the usefulness of radioisotopic tracers in the measurement of renal function in infants, and to the study of experimental tolerization of

[Thoracoscopic surgery in children]. / Chirurgie thoracoscopique chez l'enfant.

Thoracoscopy offers numerous diagnostic and therapeutic options in pediatric surgery. This paper reviews some of the principal thoracoscpic procedures performed in children. Thoracoscopy for empyema, pectus excavatum, chylothorax, etc., will be discussed. Thoracoscpy has been postulated to offer similar results as conventional surgery with lower morbidity and reduced hospital stay. These assumptions still await systematic studies , however.

Gunshot injuries of the extrahepatic biliary ducts.

OBJECTIVE: To assess our management of gunshot injuries of the extrahepatic biliary ducts. DESIGN: Retrospective study. SETTING: Urban teaching hospital, South Africa. SUBJECTS: 17 patients who were found to have gunshot injuries of the extrahepatic bile ducts, January 1993-June 1998. INTERVENTIONS: Packing and damage control, staged repair, or definitive repair. MAIN OUTCOME MEASURES: Morbidity and mortality: Three of the 17 died intraoperatively or postoperatively from causes unrelated to the biliary injury. Of the 14 survivors, 3 were managed initially with packing and damage control, 7 by staged repair, and 4 by definitive biliary repair. Eleven patients had a Roux-en-Y biliary jejunostomy, of whom 1 developed a late biliary stricture related to a postoperative anastomotic leak. End-to-end anastomosis of the bile duct was done for 2 patients and they both developed late biliary strictures. Ligation of the injured duct and cholecystojejunostomy was done for I patient. CONCLUSION: Roux-en-Y biliary-jejunal anastomosis is appropriate in the treatment of gunshot injuries of the extrahepatic biliary ducts as there is always a degree of tissue loss and some debridement is required, making it difficult to do a tension-free anastomosis.

[Gastric pneumatosis as a sign of duodenal stenosis in a child with Down syndrome]. / Pneumatose gastrique révélatrice de sténose duodénale chez un nourrisson trisomique 21.

A case of gastric pneumatosis is described in a 6 month old girl with Down syndrome. Plain abdominal radiographs showed a radiolucent rim within the gastric wall and a dilated stomach. Ultrasonography showed diffuse echoic streaks of air in the gastric wall, pneumobilia and gastroduodenal dilatation. Upper GI series showed duodenal stenosis. Gastric pneumatosis (air within the gastric wall), is an unusual radiographic finding. It may be of mechanical or inflammatory origin or, in some rare cases, remain unexplained.

Surgical repair of coarctation of the aorta in infants under one year of age. Long-term results in 146 patients comparing subclavian flap angioplasty and modified end-to-end anastomosis.

Between July 1976 and February 1991, 146 consecutive infants underwent surgical repair of coarctation of the aorta. Age at operation varied from 2 days to 11 months (median 1 month). Ninety-two (63%) were less than 2 months. Isolated coarctation was present in 65 patients (group 1), associated ventricular septal defect in 49 patients (group 2) and complex anomalies in 32 patients (group 3). The majority (65%) were in a critical condition and 45 patients (31%) were artificially ventilated. Subclavian flap angioplasty was performed in 39 patients and resection and end to end anastomosis in 107 patients. Neither hospital mortality was significantly different between subclavian flap angioplasty (15%) and end-to-end anastomosis (18%) nor was the postoperative hypertension. Actuarial survival at 10 years were 100% for group 1, 94% for group 2, and 62% for group 3. Seventeen patients had recurrent coarctation. No significant difference was found in terms of types of repair or age at operation. As no major advantage in terms of mortality and morbidity to either technique was found, we recommend resection and end-to-end anastomosis. This technique not only relieves the obstruction whatever the level is but also eliminates the ductal tissue, preserves the subclavian artery and avoids the use of prosthetic material.