Modified classification of spinal cord vascular lesions

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The literature on spinal vascular malformations contains a great deal of confusing terminology. Some of the nomenclature is inconsistent with the lesions described. Based on the experience of the senior author (R.F.S.) in the treatment of more than 130 spinal cord vascular lesions and based on a thorough review of the relevant literature, the authors propose a modified classification system for spinal cord vascular lesions.

Lesions are divided into three primary or broad categories: neoplasms, aneurysms, and arteriovenous lesions. Neoplastic vascular lesions include hemangioblastomas and cavernous malformations, both of which occur sporadically and familially. The second category consists of spinal aneurysms, which are rare. The third category, spinal cord arteriovenous lesions, is divided into arteriovenous fistulas and arteriovenous malformations (AVMs). Arteriovenous fistulas are subdivided into those that are extradural and those that are intradural, with intradural lesions categorized as either dorsal or ventral. Arteriovenous malformations are subdivided into extradural-intradural and intradural malformations. Intradural lesions are further divided into intramedullary, intramedullary-extramedullary, and conus medullaris, a new category of AVM.

This modified classification system for vascular lesions of the spinal cord, based on pathophysiology, neuroimaging features, intraoperative observations, and neuroanatomy, offers several advantages. First, it includes all surgical vascular lesions that affect the spinal cord. Second, it guides treatment by classifying lesions based on location and pathophysiology. Finally, it eliminates the confusion produced by the multitude of unrelated nomenclatural terms found in the literature.

Article Information

Address reprint requests to: Robert F. Spetzler, M.D., Neuroscience Publications, Barrow Neurological Institute, 350 West Thomas Road, Phoenix, Arizona 85013–4496. email: neuropub@chw.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Upper: Anteroposterior angiogram demonstrating subarachnoid hemorrhage associated with a dissecting aneurysm on the artery of Adamkiewicz. Lower: Intraoperative photograph. The lesion was wrapped with muslin gauze and has been asymptomatic more than 5 years. From Vishteh AG, Brown AP, Spetzler RF: Aneurysm of the intradural artery Adamkiewicz treated with muslin wrapping: technical case report. Neurosurgery 40:207–209, 1997. With permission from Lippincott-Williams and Wilkins.

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    Left: Artist's rendering of an extradural AVF. Right: Right anteroposterior vertebral artery angiogram revealing a large serpiginous fistula in the epidural space compressing the spinal cord in a 47-year-old woman who developed progressive quadriparesis.

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    Upper Left and Center: Artist's rendering of an intradural dorsal AVF. Upper Right: Artist's rendering of an intradural dorsal fistula that has its recruited blood supply from several levels. Lower Inset: Selective angiogram obtained in a 41-year-old woman who developed progressive paraparesis, demonstrating a fistula between the radiculomedullary artery and the coronal venous plexus. The fistula was coagulated and transected.

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    Upper Left: Artist's rendering of an intradural ventral AVF. Upper Right: Anteroposterior spinal angiogram obtained in a 16-year-old woman with progressive headache and back pain, revealing a small AVF (Type A) located on the anterior surface of the spinal cord. From Carter LP, Spetzler RF: Spinal arteriovenous malformations. Surgical treatment, in Carter LP, Spetzler RF, Hamilton MG (eds): Neurovascular Surgery. New York: McGraw-Hill, 1995, pp 1197–1212. With permission from McGraw-Hill. Lower Left: Sagittal T2-weighted MR image of the cervical spine obtained in a 34-year-old woman with progressive left upper-extremity pain, revealing serpiginous flow voids ventral and lateral to the spinal cord. The lesion was exposed by a cervical corpectomy and durotomy. The AVF was coagulated and sharply transected, completely relieving the patient's preoperative pain. From Spetzler RF, Koos WT (eds): Color Atlas of Microneurosurgery, ed 2. Volume III: Cerebral Revascularization, Extracranial Vascular Disease, and Intraspinal Pathology. Stuttgart: Georg Thieme Verlag, 1999, p 413. With permission from Georg Thieme Verlag Medical, Stuttgart. Lower Right: Axial T1-weighted MR images obtained in a 10-year-old boy with severe progressive thoracolumbar pain, demonstrating a large, circular flow void in the anterior spinal canal that is indenting the cord. Selective angiography demonstrated a large ventral AVF (Type C). The lesion was occluded using an endovascular approach. With permission from Barrow Neurological Institute.

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    Intraoperative photograph demonstrating the intradural location of the fistula (F), feeding artery (A), and associated arterialized veins (V). The nerve root (N) is seen coursing below the fistula.

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    Left: Artist's rendering of an extradural—intradural AVM. Right: Coronal T1-weighted MR image obtained in an 8-year-old girl who developed severe paraparesis, revealing involvement of the spinal cord, vertebral column, and extraspinal soft tissue.

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    Left: Artist's rendering of a compact intramedullary AVM. Right: Anteroposterior right vertebral artery angiogram obtained in a 32-year-old woman who developed acute onset of upper- and lower-extremity weakness, demonstrating multiple fistulas feeding an intramedullary malformation and associated aneurysm. The malformation and associated aneurysm were exposed and resected by a laminectomy and dorsal myelotomy. Postoperative right lateral vertebral angiography (not shown) demonstrated the patency of the ASA and no residual malformation.

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    Artist's rendering of a diffuse intramedullary AVM.

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    Upper: Artist's rendering of a conus AVM. Selective angiography (not shown) demonstrated a T-10 fistula in a 36-year-old Hispanic man with progressive paraparesis and lower-extremity pain. The fistula fed into the coronal venous system. Lower: Intraoperative photograph showing exposure of the conus and cauda equina achieved through a multilevel lumbar laminoplasty. The lesion consisted of multiple fistulas feeding the malformation located on the posterior surface of the conus. Preoperative spinal angiography (not shown) showed only one fistula fed from above. Intraoperatively, three additional fistulas, originating from vessels coursing along the cauda equina nerve roots and being fed from below, were identified. The lesion was resected. The patient's neurological condition remained stable, and her lower-extremity pain resolved.

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