Natural Course, Clinical Profile, and Treatment Strategies for Cerebral Cavernous Malformations

Abstract

A large body of evidence has suggested that the natural biology for symptomatic cerebral cavernous malformations (CCMs) is dynamic. These lesions exhibit a temporal clustering epiphenomenon and usually manifest with multispectral clinical patterns, the most relevant being hemorrhagic and seizurogenic events. Most patients with cerebral cavernous malformations are asymptomatic, and the lesions are detected as incidentalomas. However, association with the CCM3 gene, Zabramski type I and II lesions, and brainstem location have the propensity to increase the bleeding events. The rebleeding risk is 20%/year per lesion, which supports the need for surgical strategies for brainstem cavernous malformations; paradoxically, almost 50% of these patients develop new deficits postoperatively. A navigation-aided approach through safe entry zones is therefore of paramount importance in mitigating the surgical risks. Radiosurgery is currently reserved for biologically aggressive lesions that are not amenable for excision. Similarly, antiepileptic drug treatment is advised following the index seizure event. Early resection of the astrogliotic tissue, barring tissues within the brainstem, following detailed seizure semiology may be beneficial to patients with mesiotemporal lesions and in patients with noncompliance and severe adverse reactions to antiepileptic drug treatment. The proper dichotomization of symptomatic and high-risk cohorts and implementation of stringent surgical strategies performed by experienced surgeons result in good surgical outcomes. The guidelines from the Angioma Alliance Scientific Advisory Board Clinical Experts Panel greatly facilitate in formulating the proper management algorithm.