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PREVALENCE OF ESOPHAGEAL ATRESIA AMONG 18 INTERNATIONAL BIRTH DEFECTS SURVEILLANCE PROGRAMS
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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.
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