Spinal Vascular Malformations Overview¶
Classification¶
Spinal vascular malformations are classified by their angioarchitecture and location:
Type I — Dural Arteriovenous Fistula (dAVF) The most common spinal vascular malformation (60–80%). An abnormal arterial-venous connection within the dural sleeve of a nerve root, causing venous congestion of the cord. Typically affects older men.
Type II — Intramedullary AVM (Glomus type) A true AVM nidus within the cord parenchyma. Compact nidus with high flow. Most common in young adults.
Type III — Intramedullary AVM (Juvenile type) A large, diffuse AVM involving the cord, often with extramedullary and even extraspinal components. Rare and aggressive.
Type IV — Perimedullary (Intradural Extramedullary) AVF A direct arterial-venous fistula on the surface of the cord (pial surface), without a nidus. Three subtypes based on size and flow.
Cavernous Malformation Low-flow lesion within the cord parenchyma, not visible on angiography.
Imaging Approach¶
MRI¶
- T2-weighted — Flow voids on the cord surface (dilated perimedullary veins) are the hallmark of dAVFs and high-flow AVMs
- Cord edema — Diffuse T2 hyperintensity within the cord from venous congestion (dAVF) or ischemia
- Cord expansion — Variable
- Contrast — Enhancing serpiginous vessels on the cord surface; cord enhancement from congestion/ischemia
Spinal Angiography (Gold Standard)¶
- Identifies the feeding artery, fistula point, draining veins, and nidus
- Essential for treatment planning (endovascular embolization vs surgical disconnection)
MR Angiography¶
- Non-invasive screening; can localize the fistula level to guide catheter angiography
Clinical Pearl
Serpiginous flow voids on the dorsal cord surface on T2-weighted MRI in a patient with progressive myelopathy should immediately raise suspicion for a spinal dural AVF. This is a treatable cause of progressive paraplegia — early diagnosis and treatment can prevent irreversible cord damage.
Key Points¶
- Dural AVF (Type I) is the most common spinal vascular malformation
- Flow voids on the cord surface on T2 MRI are the hallmark
- Spinal angiography is the gold standard for diagnosis and treatment planning
- Early diagnosis is critical — venous congestion myelopathy from dAVF is reversible if treated early
- Classification is based on angioarchitecture: dural AVF, intramedullary AVM, perimedullary AVF, cavernous malformation
References¶
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Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg. 2002;96(2 Suppl):145-156. https://pubmed.ncbi.nlm.nih.gov/12450276/
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Kim LJ, Spetzler RF. Classification and surgical management of spinal arteriovenous lesions: arteriovenous fistulae and arteriovenous malformations. Neurosurgery. 2006;59(5 Suppl 3):S195-201; discussion S3-13. https://pubmed.ncbi.nlm.nih.gov/17053603/
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McCarty J, Chung C, Samant R, et al. Vascular pathologic conditions in and around the spinal cord. RadioGraphics. 2024;44(9):e240055. https://pubmed.ncbi.nlm.nih.gov/39207926/
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Kona MP, Buch K, Singh J, Rohatgi S. Spinal vascular shunts: a patterned approach. AJNR Am J Neuroradiol. 2021;42(12):2110-2118. https://pubmed.ncbi.nlm.nih.gov/34649916/
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Alkhaibary A, Alharbi A, Alnefaie N, et al. Spinal dural arteriovenous fistula: a comprehensive review of the history, classification systems, management, and prognosis. Chin Neurosurg J. 2024;10:2. https://pmc.ncbi.nlm.nih.gov/articles/PMC10775532/
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Eddleman CS, Jeong H, Cashen TA, et al. Advanced noninvasive imaging of spinal vascular malformations. Neurosurg Focus. 2009;26(1):E9. https://pmc.ncbi.nlm.nih.gov/articles/PMC2843400/
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Spinal vascular malformations. Radiopaedia.org. https://radiopaedia.org/articles/spinal-vascular-malformations