Symptomatic Hemorrhage From Cerebral Cavernous Malformations: Evidence from a Cohort Study

Abstract

Patients with a cerebral cavernous malformation (CCM) can have intracranial hemorrhages ranging from insignificant and chronic microbleeds to life-threatening hemorrhage. Management decisions and patient counseling are based on a heterogeneous body of evidence. We sought to improve the 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. A retrospective cohort analysis between 1990 and 2018 was performed, including patients with a diagnosis of a CCM. The primary outcome was acute symptomatic hemorrhages. We identified 438 patients harboring 632 CCMs. Mean age at diagnosis was 50 years (standard deviation ±17 years). Median follow-up was 26 months (interquartile range, 7-72 hours). Multiple lesions were encountered in 64 patients (15%). An initial symptomatic presentation was observed in 64% of the patients. There were 438 supratentorial lesions (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 showed 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 (odds ratio, 1.81; 95% confidence interval, 1.17-2.81; P= 0.008). Infratentorial cavernous malformations are more likely to present with symptomatic hemorrhages at diagnosis or during follow-up when accounting for size differences between lesions.