Presentation
Arm pain for many years. Now with feet paresthesia and progressive ataxia.
Patient Data
Cervical spine MRI demonstrates an undulating appearance of the ventral cervical cord related to prominent vessels in the canal, but no abnormal cord signal.
MR angiogram of the spine confirms an abnormally dilated epidural and perimedullary plexus centered in the cervical spine.
Left vertebral artery injection demonstrates early filling of the left epidural plexus at the level of the left V2/V3 vertebral artery junction.
Case Discussion
Spinal epidural arteriovenous fistulas (AVFs) are exceedingly rare and only a few case reports exist in the literature. The mechanisms driving development and progression of spinal epidural AVFs remain unclear, but there have been associations with previous surgeries, trauma, and neurofibromatosis.
Spinal epidural AVFs compromise the outflow of the epidural venous plexus, resulting in reflux to the intradural veins and eventually to the perimedullary veins. However, unlike classical spinal dural AVFs, the epidural plexus does not directly connect with the perimedullary plexus. Epidural AVFs are found in the ventral epidural space and drain into the ventral epidural venous pouch. Thus, the clinical course of spinal dural AVFs tends to be slower than that of classical spinal dural AVFs, where the radiculomedullary vein drains directly into the perimedullary plexus. Patients with spinal epidural AVFs tend to present later, with more benign symptoms such as radiculopathy. However, once the perimedullary plexus becomes engorged, the clinical presentation becomes identical to classical spinal dural AVFs, with congestive myelopathy.
As with classical spinal dural AVFs, the goal in the treatment of epidural AVFs is to occlude fistulous communication by either surgery or endovascular embolization. Most epidural AVFs can be successfully treated with transarterial embolization.
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