Jan-Karl Burkhardt1, Michael M Safaee1, Aaron J Clark1, Michael T Lawton2. 1. Department of Neurological Surgery, University of California, 505 Parnassus Avenue, M779, San Francisco, CA, 94143-0112, USA. 2. Department of Neurological Surgery, University of California, 505 Parnassus Avenue, M779, San Francisco, CA, 94143-0112, USA. Michael.Lawton@ucsf.edu.
Abstract
BACKGROUND: Sacral epidural arteriovenous fistulas (eAVFs) are rare and often misdiagnosed because of the incongruence between the thoracic level of clinical deficits and the sacral location of the offending pathology. Failure to diagnose this lesion delays treatment, resulting in prolonged venous hypertension in the cord, progressive neurological deterioration, and decreased chances of recovery. METHODS: A single-institution case series and the published literature were reviewed. RESULTS: Three patients had sacral eAVFs are located in the ventral epidural space with outflow connections to radicular veins that arterialized spinal cord veins, all presenting with thoracic myelopathy, venous engorgement, and delayed diagnosis. All eAVFs were occluded completely with radiographic and clinical improvement. CONCLUSIONS: Sacral eAVF pathophysiology, namely venous hypertension and compromised spinal cord circulation, is exactly the same as dural AVFs, as is their treatment: the interruption of outflow by occlusion of the draining vein, which effectively eliminates venous hypertension, without occlusion of the actual fistula itself. Epidural exposure of sacral eAVFs is not necessary, whereas complete intradural occlusion of their radicular drainage is. Draining radicular veins intermingle with the nerve roots and their occasional multiplicity makes them more difficult to identify intraoperatively.
BACKGROUND: Sacral epidural arteriovenous fistulas (eAVFs) are rare and often misdiagnosed because of the incongruence between the thoracic level of clinical deficits and the sacral location of the offending pathology. Failure to diagnose this lesion delays treatment, resulting in prolonged venous hypertension in the cord, progressive neurological deterioration, and decreased chances of recovery. METHODS: A single-institution case series and the published literature were reviewed. RESULTS: Three patients had sacral eAVFs are located in the ventral epidural space with outflow connections to radicular veins that arterialized spinal cord veins, all presenting with thoracic myelopathy, venous engorgement, and delayed diagnosis. All eAVFs were occluded completely with radiographic and clinical improvement. CONCLUSIONS: Sacral eAVF pathophysiology, namely venous hypertension and compromised spinal cord circulation, is exactly the same as dural AVFs, as is their treatment: the interruption of outflow by occlusion of the draining vein, which effectively eliminates venous hypertension, without occlusion of the actual fistula itself. Epidural exposure of sacral eAVFs is not necessary, whereas complete intradural occlusion of their radicular drainage is. Draining radicular veins intermingle with the nerve roots and their occasional multiplicity makes them more difficult to identify intraoperatively.