Rare giant traumatic cervical arteriovenous fistula in neurofibromatosis type 1 patient

Abstract

Arteriovenous fistulas can rarely occur in patients with neurofibromatosis type 1. These lesions typically result from traumatic insult to the dysplastic parent artery. The damaged artery forms abnormal connections with nearby paraspinal and epidural venous structures. Surgical treatment of these lesions can be extremely challenging given the proximity to the spinal cord and the ability of the fistula to recruit vessels from adjacent vascular structures. A 29-year-old woman with neurofibromatosis type 1 and a motor vehicle collision 2 years earlier presented with gait difficulty, lower extremity spasticity and neck and arm pain. Her investigation revealed a giant cervical vertebral arteriovenous fistula. The fistula was successfully treated in multiple stages using all endovascular techniques including detachable coils, stents and glue embolisation. Reduction in flow and improvement in symptoms are reasonable goals in this specific rare subgroup of complex cervical arteriovenous fistulae.

Figure 1

Right vertebral injection, AP view (A) and lateral view (B), shows major afferent flow to fistula.

Figure 2

Right external carotid injection, lateral view (A), and right thyrocervical trunk, AP view (B), provides feeders to the fistula.

Figure 3

Right vertebral artery injection, AP view (A), and left vertebral artery injection, AP view (B), shows proximal and distal coil occlusion of the right vertebral artery.

Figure 4

Right subclavian artery injection shows recruitment of vessels from the costocervical trunk.

Figure 5

Aortic arch injection shows two covered stents in the right subclavian artery.

Figure 6

External jugular venogram, AP view, shows ectatic venous aneurysm.

Figure 7

Pre- (A) and post (B) embolisation images, AP views, showing coil mass in venous aneurysm.

Figure 8

Left vertebral artery injection, AP view, shows no new vessel recruitment.