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J Pediatr Surg. 2013 Aug;48(8):1664-9. doi: 10.1016/j.jpedsurg.2013.03.075.

Esophageal atresia: data from a national cohort.

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1
Reference Center for Congenital Oesophageal Anomalies, University Hospital Lille, France. rony.sfeir@chru-lille.fr

Abstract

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.

KEYWORDS:

Cohort study; Epidemiology; Esophageal atresia; Neonatal surgery; Population-based registry; Prenatal diagnosis; Rare disease

PMID:
23932604
DOI:
10.1016/j.jpedsurg.2013.03.075
[Indexed for MEDLINE]
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