Recurrent near-fatal haemorrhage in von Recklinghausen's disease

Abstract

Von Recklinghausen's disease (congenital neurofibromatosis) is rarely associated with spontaneous haemorrhage. When such bleeding occurs, it is usually from the gastrointestinal tract and due to mucosal hypervascular plexiform neurofibromata. The upper small bowel is most commonly involved. Very rarely intracoelomic haemorrhage occurs and is usually fatal. We report the case of a patient with von Recklinghausen's disease who suffered three spontaneous, near-fatal haemorrhages (two retroperitoneal and one intrathoracic) following a routine hysterectomy. The patient underwent immediate surgery on each occasion and has survived.