Esophageal atresia: data from a national cohort

PURPOSE: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS: Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS: Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable

Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l’œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer

Antegrade percutaneous balloon dilation of ureteral strictures after failed pelviureteric or ureterovesical reimplantation in children

OBJECTIVES: To assess the morbidity and success rate of percutaneous treatment of the postoperative ureteric strictures in children. MATERIALS AND METHODS: Between January 1994 and December 2003, 12 children were treated by antegrade percutaneous balloon dilation for postoperative ureteric strictures. Stenosis occurred at the pelviureteric junction in 5 cases and ureterovesical junction in 7 cases. The 10 boys and 2 girls were between 3 months and 14 years old (mean, 5 years +/- 4.7 years). General anesthesia was used in 10 cases for nephrostomy catheter placement. Five ureteral stents were used additionally for nephrostomy drainage with a 6-F catheter. Both nephrostomy and ureteric stents were in place for 28.5 +/- 12 days, then removed after control antegrade pyelography. RESULTS: Dilation was technically successful in 9 of our patients. Two peroperative complications occurred. Postoperative results were evaluated by ultrasonography, intravenous urography, antegrade pyelography, and diethylene triamine pentaacetic acid renography that confirmed no obstacle in all 5 cases of pelviureteric stricture with a follow-up of 4 +/- 2.9 years and in 4 cases of ureterovesical junction with a follow-up of 4.7 +/- 2.8 years. Three unsuccessful results were reported: in 2 cases, the guide wire could not be advanced over the stenotic ureterovesical junction and in 1 case an early restenosis occurred that eventually required surgery. CONCLUSIONS: Although the main treatment of the postoperative ureteral strictures is surgical, the percutaneous antegrade balloon dilation seems to be an alternative to surgery with a low morbidity rate and short hospitalization period