Cerebral cavernous malformations: Viewpoint—surgery

Abstract

Cerebral cavernous malformations are one of the more common subtypes of vascular malformations and represent an important cause of hemorrhagic stroke, particularly in young adults and children. Understanding the etiology, confirming the diagnosis, and initiating appropriate treatment are essential, as this form of stroke accounts for a 2–3-fold greater risk of death or dependency as compared with stroke from ischemic origins. This updated review details the epidemiology, clinical presentation, natural history, diagnostic criteria, and treatment options, focused mainly on when and how to intervene, as this is typically considered a surgical disease. The outcomes data from the most up-to-date clinical series are described, which confirm overall excellent clinical results with microsurgical resection with a low complication rate. This chapter also concisely details the current knowledge regarding the molecular pathogenesis, genetic basis, and how the aberrant molecular signaling pathways interact to express the clinical phenotype. It further outlines clinical algorithms and special techniques that can be employed in the even rarer instances when these vascular lesions are located in the deepest parts of the brain parenchyma. For the neurologist, neurosurgeon, or basic scientist, this chapter offers an introduction into the relevant issues for patients with this disease and provides an extensive reference list for further reading.​