Cerebral Cavernous Malformations

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 0.4% to 0.5% of the population, with male and female patients equally affected and all ages represented. They constitute 8% to 15% of all vascular malformations. Patients commonly present with gross intracranial hemorrhage and focal neurologic deficits. Lesions are frequently multiple in the same patient, and 10% to 20% are associated with familial form. Several reports have documented a dynamic clinico-radiologic lesion behavior with de novo lesion genesis, intralesional and perilesional hemorrhage, and corresponding fluctuations in lesion size. Hemorrhagic risk and neurologic disability seem to be associated to multiple factors, including lesion location, age, sex, 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 consist of expectant follow-up in patients with asymptomatic or inaccessible lesions, excision of symptomatic and accessible lesions, and radiosurgery could be discussed for progressively symptomatic lesions at inoperable locations.