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Cerebrovascular malformations are a diverse group of congenital or acquired vascular anomalies of the brain. They differ by vessel type (arterial, venous, capillary), flow dynamics (high vs low), and clinical behavior (risk of hemorrhage, seizure, or neurologic deficits).

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Classification

International Society for the Study of Vascular Anomalies (ISSVA) 2018 classification: http://www.issva.org/content.aspx?page_id=22&club_id=298433&module_id=152904

![Division of vascular anomalies with a focus on the sub-classifications of vascular malformations. Arteriovenous malformations are categorized in the group of simple malformations, according to ISSVA’s 2018 criteria. Note that the main topics covered in this systematic review are shown in green.

Castillo-Rangel C, Marín G, Hernandez-Contreras KA, Zarate-Calderon C, Vichi-Ramirez MM, Cortez-Saldias W, Rodriguez-Florido MA, Riley-Moguel ÁE, Pichardo O, Torres-Pineda O, et al. Atlas of Nervous System Vascular Malformations: A Systematic Review. Life. 2022; 12(8):1199. https://doi.org/10.3390/life12081199](attachment:6bcb577d-6b34-4908-b9fa-f6126fc075d9:life-12-01199-g001.png)

Division of vascular anomalies with a focus on the sub-classifications of vascular malformations. Arteriovenous malformations are categorized in the group of simple malformations, according to ISSVA’s 2018 criteria. Note that the main topics covered in this systematic review are shown in green.

Castillo-Rangel C, Marín G, Hernandez-Contreras KA, Zarate-Calderon C, Vichi-Ramirez MM, Cortez-Saldias W, Rodriguez-Florido MA, Riley-Moguel ÁE, Pichardo O, Torres-Pineda O, et al. Atlas of Nervous System Vascular Malformations: A Systematic Review. Life. 2022; 12(8):1199. https://doi.org/10.3390/life12081199

Pathophysiology

Cellular mechanisms of cerebrovascular malformations: https://doi.org/10.1038/nn.2947

![(a) In the healthy brain, a feeder artery (red) ramifies into a branched network of capillaries that are drained by a vein (blue). (b) CCMs are low-flow lesions characterized by dysplastic capillaries forming cavernous sinusoids. (c) AVMs are fast-flow lesions wherein feeder arterioles shunt directly to veins without intervening capillaries. Candidate disease-associated molecules that are upregulated (+) or downregulated (−) are shown. EC, endothelial cell.

Storkebaum, E., Quaegebeur, A., Vikkula, M. et al. Cerebrovascular disorders: molecular insights and therapeutic opportunities. Nat Neurosci 14, 1390–1397 (2011). https://doi.org/10.1038/nn.2947](attachment:8af1b31f-14f0-4094-8d19-deae1817777a:41593_2011_Article_BFnn2947_Fig1_HTML.webp)

(a) In the healthy brain, a feeder artery (red) ramifies into a branched network of capillaries that are drained by a vein (blue). (b) CCMs are low-flow lesions characterized by dysplastic capillaries forming cavernous sinusoids. (c) AVMs are fast-flow lesions wherein feeder arterioles shunt directly to veins without intervening capillaries. Candidate disease-associated molecules that are upregulated (+) or downregulated (−) are shown. EC, endothelial cell.

Storkebaum, E., Quaegebeur, A., Vikkula, M. et al. Cerebrovascular disorders: molecular insights and therapeutic opportunities. Nat Neurosci 14, 1390–1397 (2011). https://doi.org/10.1038/nn.2947

Clinical Presentations

Radiology

https://doi.org/10.1007/s00247-014-3248-x

Management