Spinal Epidural Arteriovenous Fistula with Perimedullary Venous Reflux: Clinical and Neuroradiologic Features of an Underestimated Vascular Disorder

Abstract

BACKGROUND AND PURPOSE: The purpose of this study was to discuss the clinical and radiologic characteristics of spinal epidural arteriovenous fistulas (SEAVF) and demonstrate their specific angiomorphology in a single-center series. MATERIALS AND METHODS: Thirteen consecutive patients were diagnosed with SEAVF at RWTH Aachen University Hospital between 2006 and 2018 and were included in this study. All patients had MR imaging and DSA before treatment; 10 of these 13 patients received contrast-enhanced MRA (CE-MRA). RESULTS: The mean patient age was 72 Ϯ 8 years. Paraparesis was present in 12 (92%) patients. Sphincter dysfunction and sensory symptoms were observed in 7 (54%) and 6 (46%) patients, respectively. The mean duration of symptoms was 6 Ϯ 8 months. Congestive myelopathy on MR imaging was present in all patients. Prominent arterialized perimedullary veins were demonstrated in only 3 cases. CE-MRA revealed arterialized perimedullary veins and an arterialized epidural pouch in 9/10 (90%) patients, mostly located ventrolaterally. DSA demonstrated a multisegmental extension of the arterialized ventrolateral epidural pouch in 6 (46%) cases. An intradural radicular drainage vein was localized distant from the original fistula point in 3 (23%) patients. CONCLUSIONS: Congestive myelopathy with an acute/subacute clinical course was the dominant finding in spinal epidural arteriovenous fistulas. CE-MRA is a powerful diagnostic tool for identifying arterialized perimedullary veins as well as an arterialized epidural pouch. While arterialized perimedullary veins frequently present with only mild enlargement and elongation in spinal epidural arteriovenous fistulas, the arterialized epidural pouch is frequently located ventrolaterally and may extend over several vertebral levels. DSA remains the criterion standard to precisely visualize a spinal epidural arteriovenous fistula and its intradural radicular drainage vein, which may be located distant from the fistulous point. ABBREVIATIONS: AV ϭ arteriovenous; CE-MRA ϭ contrast-enhanced MRA; SDAVF ϭ spinal dural arteriovenous fistula; SEAVF ϭ spinal epidural arteriovenous fistula

Arteriovenous malformations of the filum terminale: clinical characteristics, angioarchitecture, and management of a rare spinal vascular pathology

“…7 These presentations differ substantially from findings in classic spinal intradural AVM, which typically present with intranidal bleeding, a space-occupying effect with subsequent medullary compression. 8 It is noteworthy that an intradural bleeding (hemorrhage) has never been found in any of our own patients with FT AVM, nor has it been previously reported in the literature.…”

Onyx embolization of a spinal epidural hemorrhage caused by thoracic spinal epidural arteriovenous fistula: A case report and literature review

“…The standard treatment for SEAVF has not yet been defined, with reported options including surgical, endovascular, and medical treatments. 1 , 2 , 3 Spinal epidural arteriovenous fistulas (SEAVFs) are accepted causes of spinal infarction or epidural hemorrhage (SEDH). 4 , 5 …”

Spinal epidural arteriovenous fistula with improved sphincter impairment detected by intraoperative neurophysiological monitoring

“…[ 8 ] SEAVFs primarily drain into the epidural venous plexus, which can cause cord compression or congestive myelopathy if intradural reflux to perimedullary veins exists. [ 10 ] Microsurgical resection or endovascular embolization is chosen based on the clinical condition and vascular anatomy of the lesion, and neurophysiological monitoring under general anesthesia is recommended for functional safety and treatment decision-making in both procedures. [ 13 ] Intraoperative neurophysiological monitoring (IOM), which includes measurement of somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) and bulbocavernosus reflex (BCR) monitoring, is useful for the assessment of functional integrity during endovascular treatment for spinal cord lesions.…”