Spinal arteriovenous fistulae in patients with hereditary hemorrhagic telangiectasia: A case report and systematic review of the literature

Abstract

Background: Although rare, spinal arteriovenous malformations (AVMs) are thought to be more prevalent in the hereditary Hereditary Hemorrhagic Telangiectasia (HHT) population.

Methods: We report a case of a spinal AVM in a 37-year-old female with HHT treated with endovascular embolization. In addition, we report findings from a systematic review of the literature on the clinical characteristics, angioarchitecture, and clinical outcomes of HHT patients with spinal AVMs.

Results: The patient is a 37 year-old female with definite HHT who presented with a one-year history of progressive gait difficulty. The spinal fistula was incidentally detected on chest computed tomography (CT). Spinal angiography demonstrated a large perimedullary arteriovenous fistula was supplied by a posterolateral spinal artery. The fistula was treated with detachable coils. The patient made a complete neurological recovery. Our systematic review yielded 25 additional cases of spinal AVMs in HHT patients. All fistulae were perimedullary (100.0%). Treatments were described in 24 of the 26 lesions. Endovascular-only treatment was performed in 16 cases (66.6%) and surgical-only treatment was performed in five cases (20.8%). Complete or near-complete occlusion rates were 86.7% (13/15) for endovascular treated cases, 100.0% (4/4) for surgery and 66.6% (2/3) for combined treatments. Overall, 80.0% of patients (16/20) reported improvement in function following treatment, 100.0% (5/5) in the surgery group and 84.6% (11/13) reported improvement in the endovascular group.

Conclusions: Spinal fistulae in HHT patients are usually type IV perimedullary fistulae. Both endovascular and surgical treatments appeared to be effective in treating these lesions. However, it is clear that endovascular therapy has become the preferred treatment modality.

Keywords: Hereditary hemorrhagic telangiectasia; spinal fistula.

Figure 1.

(a) Right ICA angiogram demonstrates a nidal-type cerebral arteriovenous malformation (AVM) in the high temporal lobe with a feeding artery from an MCA branch without evidence of a feeding aneurysm or substantial dilatation (white arrow). The nidus is small and compact (black arrow). The draining vein demonstrated no ectasia or stenosis (wide black arrow). (b) Left ICA angiogram demonstrates a “micro-AVM” or capillary vascular malformation in the left frontal lobe. The feeding artery was not dilated and there was a small draining vein that filled late.

Figure 2.

(a) Flat panel CT image during injection of the left T9 intercostal artery demonstrates a large radiculomeningeal artery entering the left T9 neural foramen (arrow). This is also seen in panel (e) on DSA (black arrow). The site of the fistula was at T6 (white arrow on panel (b) and black arrow on panel (f)). Dilated transmedullary venous anastomoses at the level of T12 are seen on panel (c) (black arrow) and (g) (black arrow). Venous drainage was not only along the perimedullary venous system, but also through the left L2 radicular vein into the IVC ((d) and (h)). The 3D reconstruction of the fistula is demonstrated in panel (i).

Figure 3.

(a) Pre-embolization angiogram again demonstrated the feeding artery/posterolateral spinal artery arising off of T9 with the fistulous connection at the site of the ectatic vein (black arrow). The artery of Adamkiewicz is also fed by the left T9 intercostal artery but is not supplying the fistula (white arrows). (b) The microcatheter tip was placed in the distal aspect of the feeding artery during coil embolization (black arrow). Coil embolization of the fistulous connection and distal feeding artery was performed with complete angiographic occlusion.