Segmental defect of the intestinal musculature associated with ileal atresia and biliary atresia.

A full-term baby boy with a segmental defect of the ileal musculature associated with terminal ileal and biliary atresia is presented. The newborn had a dilated loop of the ileum 30 cm proximal to the ileal atretic site. Pathological study results showed absence of the intestinal musculature with relatively intact mucosa. Foci of recent muscular necrosis were found in the lesion. One month later, relaparotomy was performed because of persistent jaundice and hepatic duct atresia was confirmed. Segmental defect of the intestinal musculature associated with ileal atresia and biliary atresia has not been reported in the literature. The authors emphasize that in the management of this unusual defect, one should pay attention to the multiple associated malformations.