Neurofibromatosis type 1 with subarachnoid haemorrhage from the left vertebral arteriovenous fistula: case presentation and literature review

Abstract

We present the case of a 47-year-old woman with neurofibromatosis type 1 (NF1) with subarachnoid haemorrhage (SAH) from the left vertebral arteriovenous fistula, along with a review of previous cases. Our patient had a family history of NF1 and presented to the emergency department with a sudden-onset severe headache and neck pain. CT scan showed SAH. CT angiography revealed a left vertebral arteriovenous fistula and an epidural haematoma. She underwent direct surgery and was discharged without neurologic deficits. To our knowledge, this is the first case of SAH caused by perimedullary drainage of a vertebral arteriovenous fistula associated with NF1. In a literature search, we identified 40 cases of vertebral arteriovenous fistula associated with NF1. The majority of vertebral arteriovenous fistulas occurred on the left side and in women. Patients with vertebral arteriovenous fistula typically experience neck pain, radiculopathy, radiculomyelopathy and bruits.

Keywords: coma and raised intracranial pressure; dermatology; general practice / family medicine; headache (including migraines).

Figure 1

Subcutaneous neurofibroma and diffuse café-au-lait spot on the trunk.

Figure 2

Subarachnoid haemorrhage in the ambient cistern and the prepontine cistern.

Figure 3

(A) Left vertebral angiogram demonstrated the left vertebral arteriovenous fistula fed by the branches of the left vertebral artery, which had not been completely occluded after coiling. (B) Left carotid angiogram revealed the feeder from left internal carotid artery. (C) Right vertebral angiogram showed the feeder from the right vertebral artery.