title: Prevalence of esophageal atresia among 18 international birth defects surveillance programs.
abstract: 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. Birth Defects Research (Part A), 2012.
authors.affiliation: Population Perinatal Health Research, Kolling Institute of Medical Research, University of Sydney, Australia.
authors.forename: Natasha
authors.initials: N
authors.lastname: Nassar
authors.forename: Emanuele
authors.initials: E
authors.lastname: Leoncini
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Amar
authors.forename: Jazmín
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authors.lastname: Arteaga-Vázquez
authors.forename: Marian K
authors.initials: MK
authors.lastname: Bakker
authors.forename: Carol
authors.initials: C
authors.lastname: Bower
authors.forename: Mark A
authors.initials: MA
authors.lastname: Canfield
authors.forename: Eduardo E
authors.initials: EE
authors.lastname: Castilla
authors.forename: Guido
authors.initials: G
authors.lastname: Cocchi
authors.forename: Adolfo
authors.initials: A
authors.lastname: Correa
authors.forename: Melinda
authors.initials: M
authors.lastname: Csáky-Szunyogh
authors.forename: Marcia L
authors.initials: ML
authors.lastname: Feldkamp
authors.forename: Babak
authors.initials: B
authors.lastname: Khoshnood
authors.forename: Danielle
authors.initials: D
authors.lastname: Landau
authors.forename: Nathalie
authors.initials: N
authors.lastname: Lelong
authors.forename: Jorge S
authors.initials: JS
authors.lastname: López-Camelo
authors.forename: R Brian
authors.initials: RB
authors.lastname: Lowry
authors.forename: Robert
authors.initials: R
authors.lastname: McDonnell
authors.forename: Paul
authors.initials: P
authors.lastname: Merlob
authors.forename: Julia
authors.initials: J
authors.lastname: Métneki
authors.forename: Margery
authors.initials: M
authors.lastname: Morgan
authors.forename: Osvaldo M
authors.initials: OM
authors.lastname: Mutchinick
authors.forename: Miland N
authors.initials: MN
authors.lastname: Palmer
authors.forename: Anke
authors.initials: A
authors.lastname: Rissmann
authors.forename: Csaba
authors.initials: C
authors.lastname: Siffel
authors.forename: Antonin
authors.initials: A
authors.lastname: Sìpek
authors.forename: Elena
authors.initials: E
authors.lastname: Szabova
authors.forename: David
authors.initials: D
authors.lastname: Tucker
authors.forename: Pierpaolo
authors.initials: P
authors.lastname: Mastroiacovo
mesh_terms: D004933:Esophageal Atresia / Q000453:epidemiology* / Q000208:ethnology; D005006:Ethnicity; D005260:Female; D006801:Humans; D007223:Infant; D007391:International Cooperation; D050498:Live Birth / Q000453:epidemiology / Q000208:ethnology; D008297:Male; D011159:Population Surveillance; D011247:Pregnancy; D015995:Prevalence; D012042:Registries; D050497:Stillbirth / Q000453:epidemiology / Q000208:ethnology; D014138:Tracheoesophageal Fistula / Q000453:epidemiology* / Q000208:ethnology