Abstract
A 13-year-old boy with Cornelia de Lange syndrome was admitted with the chief complaints of nausea, diarrhea and fever. He had undergone fundoplication and gastrostomy for gastroesophageal reflux. After admission, his abdominal distension got worse and he underwent computed tomography, which showed pneumoperitoneum, mediastinal emphysema and subcutaneous emphysema. Surgical exploration disclosed no apparent causative findings such as any perforated portion. Afterwards he underwent an operation twice because of residual abscess and adhesional ileus. When he developed ileus, a decompression tube was inserted, which showed intestinal malrotation. We believe that the idiopathic pneumoperitoneum in our case seems to have been induced by intermittent volvulus of the small intestine. We report herein on a case in which we encountered difficulty in its treatment.
