A 66-year-old man with von Recklinghausen's disease was admitted to our emergency room because of sudden left facial pain, swelling on the left side of the neck and dyspnea.
Close examinations revealed two extracranial internal carotid artery aneurysms and we diagnosed the rupturing of these aneurysms. Half a day after his admission, sudden massive epistaxis occurred, and an emergency operation was performed. During the operation, on blocking a root of the left internal carotid artery, the epistaxis was completely stopped. To maintain brain blood flow, a superficial temporal artery-middle cerebral artery (STA-MCA) bypass, external carotid artery-middle cerebral artery bypass with his left radial artery graft (ECA-RA-MCA bypass) and ligation of the intracranial internal carotid artery were performed. The postoperative course was uneventful, but eight days after the operation, the patient underwent cardiopulmonary arrest.
Sometimes von Recklinghausen's disease is associated with vascular lesions. Though it is rare, it may cause life-threatening massive hemorrhaging.