TY - JOUR
T1 - Prevalence of esophageal atresia among 18 international birth defects surveillance programs
AU - Nassar, Natasha
AU - Leoncini, Emanuele
AU - Amar, Emmanuelle
AU - Arteaga-Vázquez, Jazmín
AU - Bakker, Marian K.
AU - Bower, Carol
AU - Canfield, Mark A.
AU - Castilla, Eduardo E.
AU - Cocchi, Guido
AU - Correa, Adolfo
AU - Csáky-Szunyogh, Melinda
AU - Feldkamp, Marcia L.
AU - Khoshnood, Babak
AU - Landau, Danielle
AU - Lelong, Nathalie
AU - López-Camelo, Jorge S.
AU - Lowry, R. Brian
AU - Mcdonnell, Robert
AU - Merlob, Paul
AU - Métneki, Julia
AU - Morgan, Margery
AU - Mutchinick, Osvaldo M.
AU - Palmer, Miland N.
AU - Rissmann, Anke
AU - Siffel, Csaba
AU - Sìpek, Antonin
AU - Szabova, Elena
AU - Tucker, David
AU - Mastroiacovo, Pierpaolo
PY - 2012/11/1
Y1 - 2012/11/1
N2 - 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.
AB - 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.
KW - Congenital anomalies
KW - Epidemiology
KW - Esophageal atresia
KW - Prevalence
UR - http://www.scopus.com/inward/record.url?scp=84869388600&partnerID=8YFLogxK
U2 - 10.1002/bdra.23067
DO - 10.1002/bdra.23067
M3 - Article
C2 - 22945024
AN - SCOPUS:84869388600
SN - 1542-0752
VL - 94
SP - 893
EP - 899
JO - Birth Defects Research Part A - Clinical and Molecular Teratology
JF - Birth Defects Research Part A - Clinical and Molecular Teratology
IS - 11
ER -
