Objective

To determine the role of associated developmental venous malformations (DVA) in intracranial hemorrhage (ICH) caused by cerebral cavernous malformations (CCM).

Methods

We analyzed patient registry data of 1219 patients with cavernous malformations treated in our institution between 2003 and 2018. Patients with spinal and familial CCM as well as patients without complete MRI data were excluded. Impact of various variables on ICH as mode of presentation was assessed using multivariate binary logistic regression analysis. Kaplan Meier/Cox regression analysis was performed to analyze cumulative 5-year-risk for (re-) hemorrhage and identify baseline predictors of this outcome.

Results

Seven-hundred-thirty-one CCM patients were included. Multivariate logistic regression confirmed statistically significant negative correlation with DVA (Odds ratio (OR) 0.635 (0.459 – 0.878)) and positive correlation with brainstem localization (OR 6.277 (95% CI 4.287 – 9.191)) with ICH as mode of presentation. Among 731 patients, 76 experienced (re-) hemorrhage during 2338 person-years of follow-up. Overall cumulative 5-year risk was 24.1% (21.1%-27.5% 95% CI). Cox regression analysis revealed initial presentation with ICH (hazard ratio (HR) 8.0 (3.549 – 18.122 95% CI)) and brainstem localization (HR 2.9 (1.756 – 4.765 95% CI)) as independent baseline predictors of (re)-hemorrhage. Presence of DVA added no independent prognostic information (HR 1.1 (0.717 – 1.885 95% CI)).

Conclusions

CCM with associated DVAs are at lower risk to present with ICH. During untreated 5-year follow-up they showed equal (re-)hemorrhage risk compared to CCM without DVA.