Prevalence and descriptive epidemiology of esophageal atresia in the Russian Federation

Background This study examined the prevalence of esophageal atresia (EA) and the relationship between EA and demographic factors in the Russian Federation. Methods Data were obtained from a population-based congenital malformations registry across 14 years (2000–2013) in 24 regions of the Russian Federation and included cases of EA among live births and stillbirths. RESULTS The total number of births was 6,478,706. There were 1317 cases of isolated EA, resulting in a rate of 2.03 (95% confidence interval [CI], 1.92–2.15) per 10,000 births or 1 case per 4926 births. There were differences in the prevalence of EA among regional registries of the Russian Federation. The prevalence of EA during the study period was stable. 57.3% of all cases were cases of EA with tracheo-esophageal fistula (compared with 42.7% of cases without fistula). The male/female sex ratio was 1.3. The relative risk of EA was higher for live births with birth weight less than 3000 g (relative risk [RR] = 2.58 (95% CI, 2.36–2.82), for older maternal age (RR = 1.47 (95% CI, 1.24–1.75), for males (RR = 1.09; 95% CI, 1.03–1.17), and for the first gravidity (RR = 1.17; 95% CI, 1.09–1.25). CONCLUSION In this study, the prevalence of EA across different regions of the Russian Federation was analyzed. The prevalence of EA in the period under study remained stable, and the relative risk of EA was associated with maternal age, birth weight and gravidity. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc.