Analysis of 309 cases of esophageal atresia for associated congenital malformations.

Three hundred nine cases of esophageal atresia were retrospectively investigated. One hundred and sixty-two (52.4%) had associated congenital malformations. The total number of malformations was 357. The eight groups of malformations in decreasing order of frequency were as follows; cardiac, 72 cases (23.3%); musculoskeletal, 55 cases (17.8%); anointestinal, 50 cases (16.2%); genitourinary, 45 cases (14.6%); head and neck, 31 cases (10.0%); mediastinal, 26 cases (8.4%); chromosomal, 17 cases (5.5%); pulmonary, 6 cases (1.9%). Of the 162 cases with associated malformations, 83 (51.2%) had a single group of malformations, 41 (25.3%) had two groups of malformations, and 38 (23.5%) had three or more groups of malformations. More than 50% of pulmonary (83.3%), head and neck (77.4%), chromosomal (58.8%), and mediastinal malformations (53.8%) were found in association with cardiac malformations. The frequency of associated malformations in esophageal atresia cases was found to be increased significantly during the investigated interval. Early gestational age and lower birthweights were significantly correlated with higher rates of malformations. The survival rates of patients with esophageal atresia was also found to be significantly increasing over time. The mean survival rates over 32 years for groups with and without malformations were 38.3% and 70.7%, respectively, and were statistically significant. The mean survival rates of the same groups in the last 5 years were found to have changed dramatically, rising to 62.9 in the group with malformations and to 100% in the group without malformations; again, the difference is significant.