Recurrent perforation complicating intestinal neurofibromatosis.

A patient with diffuse intestinal neurofibromatosis who presented with recurrent small bowel perforation is described. Such recurrent perforation has not been reported previously. Management at first perforation consisted of laparotomy, and excision of the perforated nodular lesion, with removal of the gall-bladder and appendix. The diagnosis of von Recklinghausen's disease was confirmed by skin biopsy. Management of the second perforation was conservative, with administration of intravenous antibiotics, fluid replacement therapy, and nasogastric suction. The third perforation was treated surgically, with resection of the small bowel, leaving approximately 50 cm of small bowel. Such an approach represented a compromise between cure of the neurofibromatosis and leaving sufficient small bowel to allow satisfactory alimentation.