Neurofibromatosis with Extracranial Vertebral Arteriovenous Fistulae

Abstract

A 38-year-old female was hospitalized complaining of left cervical pain. On physical examination, bruits were audible in the painful region. Plain X-rays of the cervical spine showed widening of intervertebral foramina and scalloping of the vertebral bodies at C2-C5. Left vertebral angiography disclosed aneurysmal dilatation and arteriovenous fistulae at the C3 and C4-C5 levels of the artery. The right vertebral artery, bilateral occipital arteries, left thyrocervical trunk, and costocervical trunk were associated with the fistulae as collateral pathways to the left vertebral artery. In a sequential procedure, the left vertebral artery was clipped just proximal to the posterior inferior cerebellar artery, the lower fistula was occluded by a detachable balloon, and muscle was packed to the abnormal vessel close to the upper fistula. After treatment the bruits diminished and the radiculomyelopathy abated. Arteriovenous fistulae should arouse suspicion of neurofibromatosis. Auscultation of the neck and angiography are recommended for patients with neurofibromatosis presenting with radiculomyelopathy. Intravascular surgery with a detachable balloon is considered very effective for extracranial vertebral arteriovenous fistulae.