Cornelia de Lange syndrome associated with cecal volvulus: report of a case

Cornelia de Lange syndrome is known to be occasionally associated with gast rointestinal malformation. However, the occurrence of cecal volvulus in suc h anomalies: is very rare. We report a 15-y-old Japanese boy with Cornelia de Lange syndrome associated with a cecal volvulus secondary to non-fixatio n of the cecum and ascending colon. The patient was admitted to our hospita l because of abdominal pain and bilious vomiting. At emergent laparotomy, c ecal volvulus with non-fixation of the ascending colon and cecum was diagno sed. Release of the cecal volvulus and the fixation of the cecum and ascend ing colon to the right retroperitonium were performed. The postoperative co urse was uneventful, except for sepsis caused by aspiration pneumonia soon after the operation. Conclusion: Clinicians treating patients with this syndrome should be aware that such patients may be at risk for developing severe gastrointestinal a nomalies, including cecal volvulus.