LAUSR

OBJECTIVES Patients with a cerebral cavernous malformation (CCM) can suffer from intracranial hemorrhages ranging from insignificant and chronic microbleeds to life-threatening hemorrhage. Current management decisions and patient counseling are based on a heterogeneous body of evidence. We sought to improve current literature by providing our results based on the standardized definitions, and aimed to delineate differences in the symptomatic burden for CCMs, based on their anatomic location and presence of developmental venous anomalies. This evidence will aid in clinical decision-making and patient counseling. METHODS A retrospective cohort analysis between 1990 and 2018, including patients with a diagnosis of a CCM. The primary outcome was acute symptomatic hemorrhages. RESULTS We identified 438 patients harboring 632 CCMs. Mean (Standard Deviation) age at diagnosis was 50 (± 17) years. Median follow-up was 26 months [Interquartile Range: 7-72]. Multiple lesions were encountered in 64 patients (15%). An initial symptomatic presentation was observed in 64% of the patients. There were 438 supratentorial (69%), and 194 infratentorial lesions (31%). A symptomatic hemorrhage was observed in 25% of the supratentorial lesions and 29% of the infratentorial lesions (P<0.001). A linear mixed effects regression model demonstrated a significant difference in developing a symptomatic hemorrhage at diagnosis or follow-up between CCMs with an infratentorial location and those with a supratentorial location (OR 1.81; 95% CI 1.17-2.81; P=0.008). CONCLUSION Infratentorial cavernous malformations are more likely to present with symptomatic hemorrhages at diagnosis or during follow-up when accounting for size differences between lesions.