INTRACRANIAL CAVERNOUS MALFORMATIONS - LESION BEHAVIOR AND MANAGEMENT STRATEGIES

INTRACRANIAL CAVERNOUS MALFORMATIONS are vascular anomalies consisting of endothelium-lined caverns filled with blood at various stages of thrombosis and organization and separated by a collagenous stroma devoid of mature vessel wall elements. They occur in an estimated 0.45 to 0.9% of the population, with male and female patients equally affected and all ages represented. They commonly manifest as seizures, gross intracranial hemorrhage, and focal neurological deficits. Lesions are frequently multiple in the same patient, and 10 to 30% are associated with familial clustering. Several reports have documented a dynamic clinical-radiological lesion behavior with de novo lesion genesis, intralesional and perilesional hemorrhage, and corresponding fluctuations in lesion size. Hemorrhagic risk and neurological disability seem to be related to multiple factors, including lesion location, age, gender, state of reproductive cycle, and previous hemorrhage. Lesions may behave aggressively with repetitive hemorrhages and cumulative disability or may remain quiescent for many years. Management strategies include expectant follow-up in patients with asymptomatic or inaccessible lesions, excision of symptomatic and accessible lesions, and radiosurgery of progressively symptomatic lesions in inoperable locations. Relevant disease-specific outcome parameters are proposed to guide clinical decisions and future research. Prospective, stratified, hypothesis-driven studies using rigorous epidemiological methods must be undertaken to delineate patient and lesion factors influencing clinical aggressiveness. Biological studies are essential to uncover strategies to predict and modify lesion behavior.