Cerebral Cavernous Malformations

Excerpt

Cerebral cavernous malformations (CCMs) are abnormally large collections of "low flow" vascular channels without brain parenchyma intervening between the sinusoidal vessels (see Images. Pons Cavernoma, Head CT, Cavernous Malformation). McCormick (1966) recognized CCMs as a class of cerebral vascular malformations, which include arteriovenous malformations, developmental venous anomalies (DVA), and capillary telangiectasia. Due to recurrent microhemorrhages and thrombosis, they are typically surrounded by hemosiderin deposits and gliosis. These lesions have slow flow and low pressure, causing the average rupture risk to be much lower than some other vascular malformations, eg, arteriovenous malformations. Cavernomas are often found incidentally but can also present during the evaluation of headaches, seizures, focal neurologic deficits, or symptomatic hemorrhage.

Clinically, CCMs are highly variable in both symptomatic presentation and natural history. Adding to the confusion, CCM is referred to by various terms in the medical literature, including cavernomas, cavernous angiomas, and cavernous hemangiomas, although CCM is the preferred nomenclature (see Image. Cerebral Cavernous Hemangioma). CCMs range in size from punctate to several centimeters in diameter and may occur anywhere in the central nervous system, with up to 20% of them located in the brainstem. CCM may be diagnosed in both young children and adults and may develop de novo or even regress spontaneously during a patient's lifetime. A thorough understanding of this entity's natural history is paramount to avoid unnecessary and potentially morbid interventions. Given the heterogeneity of this condition, the ontogenesis, diagnosis, and management strategies for CCMs are subjects of ongoing debate among neuroscientists, and treatment paradigms continue to evolve.