Management of a large arteriovenous fistula in the face: a case of neurofibromatosis type 1.

Neurofibromatosis type 1 (von Recklinghausen's disease or peripheral neurofibromatosis) is of particular interest to the plastic surgeon, as it affects the skin. The majority of affected patients require operative procedures for cosmetic and functional reasons. Rarely, vascular lesions such as stenosis, rupture of an aneurysm, and fistula formation in neurofibromatosis type 1 can create some difficulties during operation without any symptoms before surgery. We report the management of a large occipitojugular fistula. This life-threatening fistula was located on the right side of the face of a 28-year-old patient who had been operated six times for the same neurofibromatous mass without any operative complications. During the operation for partial excision of the neurofibromatous mass, life-threatening, uncontrollable hemorrhage began and the operation was ended without excision. Postoperative angiography revealed an arteriovenous fistula between the occipital artery and jugular vein, and also total occlusion/agenesis in the postclinoid cisternal segment of the internal carotid artery. The fistula was obliterated with coil and histoacryl lipiodol mixture. After this procedure partial excision was performed without abnormal bleeding.