Jejunoileal atresia and associated malformations: Correlation with the timing of in utero insult

Purpose: Duodenal atresia is associated with a higher incidence of associat ed congenital malformations than jejunoileal atresia, supporting the hypoth esis that the duodenal obstruction occurs early in fetal life. In this stud y, the authors analyzed the incidence of major associated malformations in jejunal atresia (JA) and ileal atresia (IA) to determine if there is a posi tive correlation between the proximity of the intestinal atresia and the as sociation of other major anomalies. Methods: Records of all patients with jejunoileal atresias treated at the a uthors' institution between 1980 and 1997 were examined. Results: There were 83 patients with jejunoileal atresias, 38 with JA, and 45 with IA. Sixteen (42%) of the JA patients had an associated major congen ital malformation, whereas only 1 (2%) of the IA patients had an associated malformation. A single atresia was found in 18 (47%) of JA patients and 41 (91%) of IA patients. Twenty (53%) of the JA patients had either multiple or apple-peel atresia. Thirteen patients (16%) died, 11 with JA, and 2 with IA. Of the 11 patients with JA who died, 6 had multiple atresias, 4 had cy stic fibrosis, and 1 had small bowel volvulus. Conclusion: The higher incidence of associated major congenital extraintest inal malformations in JA compared with IA patients suggests that some cases of JA may arise from a malformative process. J Pediatr Surg 36:774-776. Co pyright (C) 2001 by W.B. Saunders Company.