CECAL VOLVULUS IN THE CORNELIA-DE-LANGE-SYNDROME

Cornelia de Lange syndrome is a congenital malformation characterized by severe growth failure, mental retardation, and multiple physical an omalies. A variety of gastrointestinal anomalies have been described, including malrotation, colonic duplication, and nonfixation of the col on. Two patients with Cornelia de Lange syndrome presented to our inst itution with acute distal bowel obstruction. In both cases, emergency laparotomy showed cecal volvulus with necrosis of the terminal ileum, cecum, and ascending colon, secondary to nonfixation of the colon. Res ection and an end-ileostomy were performed and later successfully reve rsed in both patients. Intestinal obstruction is a known cause of deat h in these children, and nonfixation of the colon has been identified during autopsy. Parents of children with Cornelia de Lange syndrome sh ould be counseled as to the possibility bf bowel obstruction resulting from cecal volvulus. This awareness may lead to earlier identificatio n and treatment of this potentially lethal gastrointestinal tract anom aly. Copyright (C) 1994 by W.B. Saunders Company