Descriptive epidemiology of small intestinal atresia, Atlanta, Georgia.

To describe the epidemiology of small intestinal atresia (SIA) in Atlanta, 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 identified 176 infants with SIA, a prevalence of 2.8 per 10,000 livebirths. Among black infants, the prevalence was 3.7 per 10,000 livebirths, significantly higher than the prevalence of 2.4 per 10,000 among white 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 higher 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 atresia, 36% had jejunal atresia, and 14% had ileal atresia. Two infants (1%) had atresia at an unspecified site in the small intestine. We grouped the infants by anatomic location of SIA into four categories: isolated 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 the prevalence of isolated jejunal atresia was 1.4 per 10,000, significantly 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 defects among twins was a particularly interesting finding.