Classification of Spinal Vascular Malformations

Abstract

SUMMARY -Spinal vascular malformations are rare diseases with a wide variety of neurological presentations. Their classification depends on the differentiation of shunting versus non-shunting lesions, the latter being the spinal cord cavernomas. In the shunting lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural vascular shunts from the pial vascular malformations: while those shunts that are fed by radiculomeningeal arteries (i.e. the counterparts of meningeal arteries in the brain) constitute the dural arteriovenous fistulas, the shunts that are fed by arteries that would normally supply the spinal cord (i.e. the radiculomedullary and radiculopial arteries) are the pial cord arteriovenous malformations (whose cranial counterparts are the brain AVMs). Depending on the type of transition between artery and vein the latter pial AVMs can be further subdivided into glomerular (plexiforme or nidus-type) AVMs with a network of intervening vessels inbetween the artery and vein and the fistulous pial AVMs. The last step in the classification then describes whether the type of fistula has a high or a low shunting volume which will differentiate the "Macro-" from the "Micro-"fistulae. The proposed classification is therefore based on a stepwise analysis of the shunt including its arterial anatomy, its nidus-architecture and its flow-volume evaluation. The major advantage of this approach is that it leads to a subclassification with direct implications on the choice of treatment, thereby constituting a simple and practical approach to evaluate these rare diseases.

Remote Intramedullary Hemorrhage in the Thoracic Spinal Cord Secondary to a Perimedullary Arteriovenous Fistula of the Distal End of Conus Medullaris Mimicking Filum Terminale Arteriovenous Fistula: A Case Report

“…[9][10][11] Conus PMAVFs usually manifest by progressive myelopathy or acute nonhemorrhagic paraplegia. 12 Our case initially presented with progressive paresthesia of the lower extremities secondary to venous congestion and subsequently developed sudden severe pain in the left lower leg from intramedullary hemorrhage. Conus PMAVF in our case was supplied by single feeder from the ASA.…”

Remote intramedullary hemorrhage in the thoracic spinal cord secondary to a perimedullary arteriovenous fistula of the distal end of conus medullaris mimicking filum terminale arteriovenous fistula

“…Conus PMAVFs usually manifest by progressive myelopathy or acute nonhemorrhagic paraplegia. [ 12 ] Our case initially presented with progressive paresthesia of the lower extremities secondary to venous congestion and subsequently developed sudden severe pain in the left lower leg from intramedullary hemorrhage. Conus PMAVF in our case was supplied by a single feeder from the ASA.…”

Progressive Medullary Congestion Resulting in Life-Threatening Condition Caused by Lower Cervical Dural Arteriovenous Fistula Successfully Treated with Endovascular Treatment: A Case Report and Literature Review

“…SDAVFs can be treated by surgery, endovascular treatment, or both depend on institutions preference [2,3]. The goal of treatment is to obliterate the fistula, including the proximal portion of the draining radicular vein [1,32].…”

“…The fistulas usually locate intradural dorsal aspect close to the root sleeves. The patients typically manifest with gradual progression of myelopathy induced by chronic venous hypertension [1][2][3]. Intramedullary hemorrhage secondary to SDAVFs is extremely rare, and usually causes by venous varix [4].…”