Cerebral Cavernous Malformations: Which Ones Should We Worry About?

Summary and Comment |
December 15, 2015

Cerebral Cavernous Malformations: Which Ones Should We Worry About?

  1. Seemant Chaturvedi, MD

Brainstem location and presentation with intracerebral hemorrhage or focal deficits were associated with increased risk in a pooled analysis.

  1. Seemant Chaturvedi, MD

Cerebral cavernous malformations (CCMs) are the second most common incidental vascular lesion detected on brain magnetic resonance imaging. CCMs are heterogeneous in prognosis: Some cause repeated cerebral hemorrhages, whereas others are asymptomatic for decades.

To assess the predictors of brain hemorrhage associated with CCMs, researchers combined data from several independent longitudinal studies totaling 1620 patients (mean age at CCM diagnosis, 45; 54% women). The primary endpoint was estimated 5-year risk for a symptomatic intracerebral hemorrhage (ICH).

More than one CCM was present in 17% of patients. Half of the CCMs were lobar, and 35% were in the brainstem. Of patients presenting with a seizure, 86% had a lobar CCM. Among patients presenting with ICH, 62% had a brainstem CCM, as did 55% of those with focal neurological deficits (FNDs).

Presentation with either ICH or FND was associated with increased risk for ICH during follow-up (unadjusted hazard ratio, 5.6; 95% confidence interval, 3.2–9.7), as was brainstem versus other locations (unadjusted HR, 4.4; 95% CI, 2.3–8.6). The 5-year estimated ICH risks during untreated follow-up were 3.8% for nonbrainstem CCM presenting without ICH or FND, 8.0% for brainstem CCM presenting without ICH or FND, 18.4% for nonbrainstem CCM with initial ICH or FND, and 30.8% for brainstem ICH presenting with ICH or FND.

Comment

These authors provide a useful guide to help clinicians in stratifying the risk for future brain hemorrhage according to location and clinical presentation of a CCM. For some patients, such as those without brainstem location and without hemorrhage at presentation, the risk for stroke is <1% per year; these patients should likely receive conservative treatment. However, other lesions are more ominous and should be evaluated for definitive treatment such as surgical excision. Future studies with patient-centered outcomes would also be of interest.

Editor Disclosures at Time of Publication

  • Disclosures for Seemant Chaturvedi, MD at time of publication Consultant / Advisory board Abbott Vascular; Boehringer Ingelheim; Genentech Grant / Research support Pfizer Editorial boards Neurology; Stroke; Journal of Stroke and Cerebrovascular Diseases Leadership positions in professional societies American Academy of Neurology (Vice Chair of Vascular Neurology Section)

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