MANAGEMENT OF A LARGE ARTERIOVENOUS-FISTULA IN THE FACE - A CASE OF NEUROFIBROMATOSIS TYPE-1

Neurofibromatosis type 1 (von Recklinghausen's disease or peripheral n eurofibromatosis) is of particular interest to the plastic surgeon, as it affects the skin. The majority of affected patients require operat ive procedures for cosmetic and functional reasons, Rarely, vascular l esions such as stenosis, rupture of an aneurysm, and fistula formation in neurofibromatosis type 1 can create some difficulties during opera tion without any symptoms before surgery, We report the management of a large occipitojugular fistula, This life-threatening fistula was loc ated on the right side of the face of a 28-year-old patient who had be en operated six times for the same neurofibromatous mass without any o perative complications, During the operation for partial excision of t he neurofibromatous mass, life-threatening, uncontrollable hemorrhage began and the operation was ended without excision, Postoperative angi ography revealed an arteriovenous fistula between the occipital artery and jugular vein, and also total occlusion/agenesis in the postclinoi d cisternal segment of the internal carotid artery, The fistula was ob literated with coil and histoacryl lipiodol mixture. After this proced ure partial excision was performed without abnormal bleeding.