Cerebral cavernous malformations (CCMs) are vascular malformations formed by abnormal collections of dilated capillaries within the brain. Their weak walls make them prone to rupturing, resulting in intracerebral hemorrhage. CCMs have no sex predilection and typically present in adulthood. Patients often present with signs of intracranial hemorrhage including headache, seizure, or focal neurologic symptoms such as weakness, vision changes, or aphasia. The hemorrhages are usually small, but location can be critical. Hemorrhages can be recurrent. Nearly 25% of patients with CCMs remain asymptomatic. CCMs often occur sporadically and are typically solitary. Familial CCMs represent approximately 20% of all cases and are caused by genetic mutations in genes CCM1 (KRIT1), CCM2, and CCM3 (PDCD10), inherited in an autosomal dominant fashion and typically presenting with multiple CCMs. Most CCMs occur supratentorially, although lesions can occur within the posterior fossa and spinal cord. CCMs can also occur as a consequence of prior radiation therapy to the brain.
ICD10CM: Q28.3 – Other malformations of cerebral vessels
SNOMEDCT: 444869007 – Cavernous hemangioma of brain