Cervical AVM Associated with Neurofibromatosis Presenting Unilateral Pulsating Exophthalmos

Abstract

The authors reported a case of cervical arteriovenous malformation (AVM) associated with neurofibromatosis. There was only one previous report presented as a case of spontaneous extracranial vertebral AVM with neurofibromatosis. A 46-year-old female was admitted because of neurofibromatosis and unilateral pulsating exophthalmos due to bone defect at the great wing of the sphenoid bone. Neurological examination showed hearing difficulty in the right with tinnitus due to vascular bruit at neck, right pulsating exophthalmos with total extraocular muscle paresis, tetraparesis with hyperreflexia dominant in the left upper extremity, as well as total sensory weakness. Cerebral angiography revealed abnormal vascular shadows at the level of C2 to C6, chiefly fed by bilateral vertebral arteries. Metrizamide myelography showed an incomplete block at the level of C5. Direct attacks to the cervical angioma were tried both by anterior and posterior approaches. The angioma situated at the cervical intra and extra-dural spaces and was treated by feeder clipping and trapping of left vertebral artery. Histological diagnosis was AVM. Postoperative angiography revealed a complete disappearance of the AVM. Five months later the bone defect at the great wing of the sphenoid bone was repaired. Postoperatively, pulsating exophthalmos with hearing difficulty and tinnitus disappeared, while motor and sensory disturbances were unchanged.