Natural history of cerebral cavernous malformations

Abstract

Cerebral cavernous malformations (CCM) are vascular abnormalities of the central nervous system with an incidence of 0.4-0.5% and an annual rate of hemorrhage ranging from 0.7% to 1%. Most lesions are located in the cerebral hemisphere but some occur in deeper locations such as the basal ganglia and pons. The most common symptoms during presentation are headache, seizures, and focal neurologic deficits. Surgery remains the most effective treatment modality for symptomatic CCM, while the management of incidental CCM remains controversial. Factors associated with increased risk of hemorrhage include being female and less than 40 years old. This finding, however, is not consistent in all natural history studies evaluated. During follow-up, the most important and consistent risk factor for rebleed was a prior hemorrhage. Here, we provide an indepth but concise review of the literature regarding the natural history of CCMs.

Keywords: brainstem; cavernous malformations; familial cavernoma; hemorrhage; microsurgery; natural history; pediatric; radiation therapy; rebleed; seizure.