The involvement of two or more systems and the severity of associated anomalies significantly influence mortality in esophageal atresia.

ABSTRACT

PURPOSE: The aim of this study was to examine the influence of associated anomalies in babies born with esophageal atresia (EA). METHODS: A retrospective review of the records of 41 consecutive cases of esophageal atresia managed over an 11-year period was undertaken. RESULTS: A higher incidence of associated anomalies was seen in those babies with lower birth weights. Although all five (100%) babies with EA who weighed less than 1,800 g had associated anomalies, those who weighed 1,800 to 2,500 g and more than 2,500 g were associated with 67% (10 of 15) and 43% (9 of 21) anomalies, respectively. The most common system in which anomalies occurred was the cardiovascular system (37%) followed by gastrointestinal (24%), musculoskeletal (17%), genitourinary (7%), chromosomal (5%), and others (12%). All 17 (41%) babies with no associated anomalies survived. Four of the 10 babies who had two or more systems involvement died, whereas only one of 31 babies with less than two systems involvement died; the difference between these two groups was highly significant (Fisher's Exact test, P = .009). The overall mortality rate was 12%. Three of the deaths were associated with severe anomalies that were incompatible with life such as bilateral renal agenesis, trisomy 18, and complex cardiac anomalies. CONCLUSION: The association of two or more system anomalies and the severity of associated anomalies influence mortality in esophageal atresia.