To describe the epidemiology of small intestinal atresia (SIA) in Atla
nta, Georgia, from 1968 through 1989, we used the Metropolitan Atlanta
Congenital Defects Program, an active, population-based surveillance
system for birth defects diagnosed during the first year of life. We i
dentified 176 infants with SIA, a prevalence of 2.8 per 10,000 livebir
ths. Among black infants, the prevalence was 3.7 per 10,000 livebirths
, significantly higher than the prevalence of 2.4 per 10,000 among whi
te infants [relative risk (RR) = 1.6, 95% confidence interval (CI) = 1
.1,2.1]. Nine infants were each one member of a unique pair of twins.
The prevalence among twin infants was 7.3 per 10,000, significantly hi
gher than the prevalence of 2.8 per 10,000 among singletons (RR = 2.7,
95% CI = 1.4,5.2). Forty-nine percent of the infants had duodenal atr
esia, 36% had jejunal atresia, and 14% had ileal atresia. Two infants
(1%) had atresia at an unspecified site in the small intestine. We gro
uped the infants by anatomic location of SIA into four categories: iso
lated SIA (53%), SIA with multiple unrelated defects (21%), sequences
(16%), and syndromes (10%). We then compared the isolated and multiple
unrelated defects groups by gender, race, maternal age, birth weight
and one-year mortality for each location of SIA. Among black infants t
he prevalence of isolated jejunal atresia was 1.4 per 10,000, signific
antly higher than the prevalence of 0.2 per 10,000 among white infants
(RR = 6.3, 95% CI = 2.9, 13.5). The increased prevalence of these def
ects among twins was a particularly interesting finding. (C) 1993 Wile
y-Liss, Inc.