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

Sténoses post-traumatiques du grêle chez l’enfant : à propos de 2 cas

Post-traumatic small-bowel obstructions are rare and late complications following blunt abdominal trauma. Timely diagnosis is frequently impeded because of nonspecific associated symptoms, and furthermore, the degree of urgency is frequently underestimated due to the accidental cause. During the last 6 years (2005–2011), we have observed only 2 cases (4 and 10 years old) with post-traumatic small-bowel obstruction. On admission the patients had a contusion on the abdominal wall, duodenal hematoma and a Chance fracture of the lumbar spine on the computed tomography (CT) scan. Although early clinical improvement was noted, progression of the disorder was observed for the following 15 days like an occlusive syndrome (abdominal distension, nausea, emesis). Abdominal ultrasound and computed tomography scan revealed small-bowel obstruction. At laparotomy, jejunal stenosis was found associated with mesenteric tears, which was resected with end-to-end anastomosis. Six to 8 months of follow-up ascertained the therapeutic efficacy of the procedure. Post-traumatic small-bowel obstructions are rare; the clinical presentation is unspecific and appears with a time lag following the trauma. Echography and computed tomography scan can establish both the diagnosis and therapeutic choice.IIntroductionLes sténoses post-traumatiques du grêle sont une des complications tardives et rares des traumatismes fermés de l’abdomen. Leur diagnostic est difficile et peut être retardé compte tenu de leur faible fréquence et de leur mode de révélation peu spécifique.ObservationsNous rapportons 2 observations de garçons de 4 et 10 ans admis pour un traumatisme abdominal fermé à haute énergie entre 2005 et 2011. À leur admission, ils présentaient une contusion pariétale péri-ombilicale, des hématomes profonds (duodénal, caecal) et, pour l’un, une lésion type Chance en L2-3 à la tomodensitométrie (TDM). Malgré une amélioration clinique initiale, il persistait un état subocclusif (nausées et vomissements lors de la réalimentation) au-delà du 15e j. L’échographie et la TDM permettaient d’objectiver une occlusion grêlique. L’exploration chirurgicale mettait en évidence dans les 2 cas, plusieurs sténoses jéjunales associées à une dilacération du mésentère, imposant à chaque fois une résection-anastomose termino-terminale.CommentairesLes sténoses post-traumatiques du grêle sont rares et leur symptomatologie non spécifique apparaît avec un délai variable par rapport au traumatisme abdominal. La surveillance clinique, ainsi que l’échographie et la TDM permettent d’évoquer le diagnostic et de conduire à un traitement chirurgical efficace

Le mégauretère primitif de type obstructif : stratégies thérapeutiques à propos de 30 cas

Objectif: Présenter l’histoire naturelle des mégauretères primitifs de type obstructif, réévaluer notre attitude thérapeutique face aux différentes étiologies et situations du patient. Patients et méthodes: Nous avons revu rétrospectivement 30 enfants de 0 à 15 ans entre Janvier 1996 et février 2008, suivis et traités dans le service pour mégauretère primitif de type obstructif : 16 enfants ont bénéficiés d’un diagnostic anténatal (groupe A) contre 14 enfants de diagnostic post-natal (groupe B). Ces patients ont été explorés par une échographie de l’appareil urinaire, une cystographie rétrograde et mictionnelle, une scintigraphie et une Imagerie par résonance magnétique. Résultats: l’étude a porté sur 30 enfants dont 25 garçons. L’age moyen du groupe A était de 32semaines et 3 jours d’aménorrhée contre 4 ans dans le groupe B. Le mégauretère était à gauche dans 48% des cas, 30% à droite et bilatéral dans 22%. Le mégauretère était obstructif non refluant dans 87% des cas, et obstructif refluant dans 13%. Le délai de suivi de nos patients de groupe A était de 4,2 ± 1,4 ans et le groupe B : 3,6 ± 1,2 ans. La dilatation urétérale rétrovésicale a régressé spontanément dans 50% des cas au cours des 2 premières années de vie. Une correction chirurgicale a été nécessaire chez 63% de nos patients. Conclusion: cette étude a permis de montrer l’intérêt du diagnostic précoce et de la surveillance clinique et radiologique, proposée en 1ère intention, dans la prise en charge du mégauretère et pouvant entraîner la résolution spontanée du mégauretère