A CASE OF ADULT BOCHDALEK HERNIA WITH CORNELIA DE LANGE SYNDROME

Abstract

A 24-year-old woman was admitted to the hospital because of abrupt onset of dyspnea, abdominal pain and vomiting. She had been diagnosed as having Cornelia de Lange syndrome in her early childhood. After admission Bochdalek hernia was diagnosed and she was operated on. At first, we approached via laparoscopy, but it was difficult to return the escaped organs, and we changed to laparotomy. After repositioning the escaped organs, the hernia gate was closed with mesh. The postoperative course was uneventful and she was discharged from the hospital 17 days after the operation.
Cornelia de Lange syndrome is a congenital malformation of unknown origin and the patients often die in their childhood. So far no cases of Bochdalek hernia with Cornelia de Lange syndrome in adults have been reported in Japan. We report this first case in Japan with some bibliographical comments.