Burkhardt Jan-Karl, Safaee Michael M, Clark Aaron J, Lawton Michael T
Department of Neurological Surgery, University of California, 505 Parnassus Avenue, M779, San Francisco, CA, 94143-0112, USA.
Acta Neurochir (Wien). 2017 Jun;159(6):1087-1092. doi: 10.1007/s00701-017-3170-8. Epub 2017 Apr 12.
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.
A single-institution case series and the published literature were reviewed.
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.
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.
骶部硬脊膜外动静脉瘘(eAVF)较为罕见,且常因临床缺损的胸段水平与病变所在的骶部位置不一致而被误诊。未能诊断出该病变会延误治疗,导致脊髓长期静脉高压、进行性神经功能恶化以及恢复机会降低。
回顾了单机构病例系列和已发表的文献。
3例骶部eAVF位于腹侧硬脊膜外间隙,与使脊髓静脉动脉化的神经根静脉存在流出道连接,所有患者均表现为胸段脊髓病、静脉充血及诊断延迟。所有eAVF均通过影像学检查完全闭塞且临床症状改善。
骶部eAVF的病理生理学,即静脉高压和脊髓循环受损,与硬脊膜动静脉瘘完全相同;其治疗方法也是如此:通过闭塞引流静脉来中断流出道,这可有效消除静脉高压,而无需闭塞实际的瘘口本身。骶部eAVF无需进行硬脊膜外暴露,而其神经根引流的完全硬脊膜内闭塞则是必要的。引流神经根静脉与神经根相互交织,且其数量偶尔较多,这使得术中更难识别。