Ileal atresia, malrotation and Hirschsprung's disease: A case report

Ileal atresia associated with malrotation is rare, but the additional diagnosis of Hirschsprung’s disease (HD) in the same patient has not been described in the literature. This case report presents a newborn with a bowel obstruction requiring surgery for ileal atresia and malrotation. The post-operative course was complicated by a distal bowel obstruction. Evaluation of the resected bowel from the original surgery was consistent with total colonic aganglionosis, and a diverting ileostomy was performed. Delay in diagnosis of HD is not uncommonwhen an initial gastrointestinal anomaly is diagnosed and should be suspected when bowel function fails to return after corrective surgery. Published by Elsevier Inc.Open access under CC BY-NC-ND license. Atresia of the intestinal tract associated with malrotation is rare, but the additional diagnosis of Hirschsprung’s disease (HD), or total colonic aganglionosis, in the same patient does not appear in the literature. Jejunoileal atresia (JIA) occurs in approximately 1 in 5000 live births and occurs equally in males and females [1e4]. In rare instances, JIA has been found to be associated with HD, malrotation and other disorders [1,5e7]. The coexistence of these conditions can complicate the evaluation of bowel obstruction, and the co-diagnosis of HD is often not confirmed appropriately at the time of laparotomy. This article presents an infant who had ileal atresia with malrotation further complicated by total colonic aganglionosis. We report a unique combination of gastrointestinal anomalies in the same patient that has not been previously reported.