[Digestive hemorrhage in a patient with multiple neurofibromatosis]

Neurofibromatosis is an autosomal dominant trait with variable expressivity clinically defined by the coexistence of multiple cafè au lait spots, subcutaneous neurofibromas and Lisch nodules. Hemorrhage from intestinal neurofibromas or related tumors may be life-threatening because often inaccessible and therefore difficult to locate. The Authors report a case of gastrointestinal hemorrhage in a 68 year old patient with neurofibromatosis who had an episode of melena from duodenal ulcer endoscopically detected 5 years earlier. Endoscopy failed to discover the site of bleeding; this was successfully demonstrated by angiography: superior mesenteric arteriography disclosed 32 hypervascular masses supplied by a digiunal branch and by the oleo-colic artery. Because of continuing hemorrhage the patient was submitted to surgery. The exploration demonstrated ulcerated neurofibromatous neoplasms at the level of the digiunum and terminal ileum, with a Meckel's diverticulum and gallbladder stones. Intestinal resection, right emicolectomy, Meckel's diverticulum resection and colecystectomy were performed. Review of the literature demonstrates angiography is the most reliable imaging modality for detecting such tumors.