Abstract
Two cases of von Recklinghausen's disease developing hemorrhagic shock by intratumorous bleeding were successfully treated by arterial embolization followed by surgical resection. Case 1 involved a 38-year-old woman who had been having a subcutaneous tumor with the size of infant's head in the left hip for several years. The patient was transferred to th hospital because of abrupt pain and enlargement of the tumor (27×18×15cm) leading to hemorrhagic shock. Case 2 involved a 53-year-old man who was emergently admitted to the hospital because of an enlarging huge hematoma (30×37×35cm) in the right shoulder to back. The systolic pressure was 40mmHg and the patient was in a shock state. Both cases had no epidode of trauma before admission. Emergency arterial embolization was performed in both cases to achieve the sufficient hemostsis and prevent the progression of hemorrhagic shock. Thereafter, one and two weeks later, elective resection of the neurofibroma with hematoma was successfully performed in both cases.
It has been reported that acute hemorrhage is a rare complication for elephantiasis neurofibroma, but easily develops deep shock and becomes lethal. And the emergent operation is unavoidable of massive amount of intraoperative bleeding. We emphasize that combined treatment of preoperative arterial embolization with surgical resection should be recommended to treat this fatal complication of von Reclinghausen's disease.
