Prevalence of esophageal atresia among 18 international birth defects surveillance programs

Natasha Nassar, Emanuele Leoncini, Emmanuelle Amar, Jazmín Arteaga-Vázquez, Marian K. Bakker, Carol Bower, Mark A. Canfield, Eduardo E. Castilla, Guido Cocchi, Adolfo Correa, Melinda Csáky-Szunyogh, Marcia L. Feldkamp, Babak Khoshnood, Danielle Landau, Nathalie Lelong, Jorge S. López-Camelo, R. Brian Lowry, Robert Mcdonnell, Paul Merlob, Julia MétnekiMargery Morgan, Osvaldo M. Mutchinick, Miland N. Palmer, Anke Rissmann, Csaba Siffel, Antonin Sìpek, Elena Szabova, David Tucker, Pierpaolo Mastroiacovo

Research output: Contribution to journalArticlepeer-review

117 Scopus citations


BACKGROUND: The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS: The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS: Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35-2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS: On average, EA affected 1 in 4099 births (95% CI, 1 in 3954-4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference.

Original languageEnglish
Pages (from-to)893-899
Number of pages7
JournalBirth Defects Research Part A - Clinical and Molecular Teratology
Issue number11
StatePublished - 1 Nov 2012
Externally publishedYes


  • Congenital anomalies
  • Epidemiology
  • Esophageal atresia
  • Prevalence

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Embryology
  • Developmental Biology


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