Clinical Management of Cerebral Cavernous Malformations: Consensus Recommendations


This report addresses 5 key topics for cerebral cavernous malformations: epidemiology and natural history, genetic testing and counseling, diagnostic criteria and radiology standards, neurosurgical considerations, and neurologic considerations.  Search terms were derived for each key topic by the Angioma Alliance Scientific Advisory Board Clinical Experts Panel from questions from the lay community, 23 consensus management recommendations were made, 11 being level B and 12 being level C.  There were no recommendations made with level A/randomized controlled trial evidence.  8 of these recommendations were class I, 10 were class II, and 5 were class III.  These include recommendations on screening for family history and obtaining genetic testing in high risk patients, for imaging studies to characterize and follow lesions, for anti-epileptic therapy for associated seizures, and for surgical vs radiosurgical vs conservative therapy based on location, symptoms, and risk factors.  Important knowledge gaps and controversies were identified for further research.  Some unknowns regarding CCMs include relative risk of pregnancy, anti-platelet therapy, or thrombolytic therapy on hemorrhage, whether or not associated developmental venous anomalies should also be resected in operative cases, and efficacy and optimal dosage data for radiosurgery.

Stephen Grupke, MD
Lexington, KY