Cerebral Cavernous Malformations, Developmental Venous Anomaly, and Its Coexistence: A Review

Eur Neurol. 2020;83(4):360-368. doi: 10.1159/000508748. Epub 2020 Jul 30.


Background: Cerebral cavernous malformations (CCMs) are intracranial vascular malformations that can exist as a single lesion or mixed vascular lesions. The most common mixed form is the coexistence of CCM with an associated developmental venous anomaly (DVA). In this paper, we aim to give a comprehensive review of CCM, DVA, and their coexistence as mixed lesions. A PubMed search using the keywords "Cerebral cavernous malformations, Developmental venous anomaly, Mixed Cerebral cavernous malformations with Developmental venous anomaly" was done. All studies in the English language in the past 10 years were analyzed descriptively for this review.

Summary: The search yielded 1,249 results for "Cerebral cavernous malformations," 271 results for "Developmental venous anomaly," and 5 results for "Mixed Cerebral cavernous malformations with Developmental venous anomaly." DVA is the most common intracranial vascular malformation, followed by CCM. CCM can have a wide array of clinical presentations like hemorrhage, seizures, or focal neurological deficits or can also be an incidental finding on brain imaging. DVAs are benign lesions by nature; however, venous infarction can occur in a few patients due to acute thrombosis. Mixed CCM with DVA has a higher risk of hemorrhage. CCMs are angiographically occult lesion, and cerebral digital subtraction angiography is the gold standard for the diagnosis of DVA. Mixed lesions, on the other hand, are best diagnosed with magnetic resonance imaging, which has also been effective in detecting specific abnormalities. Asymptomatic lesions are treated through a conservative approach, while clinically symptomatic lesions need surgical management.

Conclusion: Individual CCM or DVA lesions have a benign course; however, when they coexist in the same individual, the hemorrhagic risk is increased, which prompts for rapid diagnosis and treatment.

Keywords: Cerebral cavernous malformations; Developmental venous abnormality; Mixed cerebral vascular malformations.

Publication types

  • Review

MeSH terms

  • Adult
  • Brain Neoplasms / complications*
  • Brain Neoplasms / pathology*
  • Cerebral Veins / abnormalities*
  • Female
  • Hemangioma, Cavernous, Central Nervous System / complications*
  • Hemangioma, Cavernous, Central Nervous System / pathology*
  • Humans
  • Male