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. McCormick (1966) recognized CCMs as one of the four classes of cerebral vascular malformations which include arteriovenous malformations (AVM), developmental venous anomalies (DVA), and capillary telangiectasia. Clinically, CCMs are highly variable in both symptomatic presentation and natural history. Adding to the confusion, CCM has assumed a variety of names in the medical literature including cavernomas, cavernous angiomas, and cavernous hemangiomas, though CCM is the preferred nomenclature. CCMs range in size from punctate to several centimeters in diameter and may occur anywhere in the central nervous system with up to 20% 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 the natural history of this entity is of paramount importance to avoid unnecessary and potentially morbid interventions. Given the heterogeneity of this condition, the ontogenesis, diagnosis, management strategies for CCMs are subjects of ongoing debate among neuroscientists and treatment paradigms continue to evolve.