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.

Preventive antireflux surgery in patients with congenital diaphragmatic hernia.

BACKGROUND: Gastroesophageal reflux (GER) is common in children with congenital diaphragmatic hernia (CDH), and the optimal management in these patients is not well established. The aim of this study was to assess whether a preventive fundoplication performed during the first surgery will improve GER and nutritional morbidity in patients with a left CDH. METHODS: Thus we studied one group of 17 patients treated with a one-step antireflux procedure (group A) and a group of 19 patients with only initial diaphragmatic repair (group B) between January 1994 and December 2004. The patients were classified in three stages, mild intermediate and severe form, based on anatomic criteria (liver position and patch requirement). Most of the patients with the severe form of disease were in group A. Clinical charts were compared between the two groups with a mean follow-up of 3 years. RESULTS: For patients with intra-abdominal liver and direct diaphragmatic closure, the duration of hospitalization was significantly longer in group A than in group B (p = 0.08). The incidence of GER was significantly lower in group A (17.6%) than in group B (52.6%) at 1-year follow-up (p < 0.5). In the last group, 30% required a fundoplication before 6 months. Seven patients (19%) required intermittent nutritional supplementation via nasogastric tube feeding; for these patients there was no difference between the two groups. A gastrostomy was performed in only one patient. Twelve percent of patients had growth retardation during most of their first year, again with no difference between the two groups. CONCLUSIONS: Based on the published data, recommendations can be made regarding the efficacy of adding an antireflux surgery procedure to left CDH repair in only patients with intrathoracic liver and/or patch requirement.