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Hemorrhage from cerebral cavernous malformations: The role of associated developmental venous anomalies
Bixia Chen1, Annika Herten2, Dino Saban2
1From the Department of Neurosurgery (B.C., A.H., D.S., S.R., Y.Z., R.J., K.H.W., U.S., P.D.), Institute of Diagnostic and Interventional Radiology and Neuroradiology (A.R.), Institute for Medical Informatics, Biometry and Epidemiology (B.S.), and Department of Neurology (C.K.), University Hospital Essen, University of Duisburg-Essen, Essen, Germany. bixia.chen@uk-essen.de.
Insights
Developmental venous anomalies (DVAs) are associated with a lower risk of intracranial hemorrhage (ICH) in patients with cerebral cavernous malformations (CCMs). However, DVAs do not independently alter the long-term risk of hemorrhage in CCM patients.
Area of Science:
- Neurology
- Neurosurgery
- Radiology
Background:
- Cerebral cavernous malformations (CCMs) are vascular malformations that can lead to intracranial hemorrhage (ICH).
- Developmental venous anomalies (DVAs) are often found incidentally alongside CCMs.
- The role of DVAs in the clinical presentation and prognosis of CCMs remains incompletely understood.
Purpose of the Study:
- To investigate the influence of associated DVAs on the occurrence of ICH in patients diagnosed with CCMs.
- To assess the impact of DVAs on the risk of future hemorrhage events in CCM patients.
Main Methods:
- Analysis of a registry of 731 patients with CCMs treated between 2003 and 2018.
- Multivariate logistic regression to evaluate predictors of ICH presentation.
- Kaplan-Meier and Cox regression analyses to determine cumulative 5-year hemorrhage risk and identify prognostic factors.
Main Results:
- A negative correlation was found between the presence of DVA and ICH presentation (OR 0.635).
- Brainstem localization was positively correlated with ICH presentation (OR 6.277).
- Initial ICH presentation and brainstem localization were independent predictors of recurrent hemorrhage, while DVA presence did not add independent prognostic value.
Conclusions:
- Associated DVAs may reduce the likelihood of initial ICH presentation in patients with CCMs.
- The long-term risk of hemorrhage in CCM patients with associated DVAs is comparable to those without DVAs when not treated.
- Brainstem location is a significant risk factor for both initial presentation and recurrent hemorrhage in CCM patients.
Objective:
To determine the role of associated developmental venous anomalies (DVAs) in intracranial hemorrhage (ICH) caused by cerebral cavernous malformations (CCMs).
Methods:
We analyzed patient registry data of 1,219 patients with cavernous malformations treated in our institution between 2003 and 2018. Patients with spinal and familial CCM and patients without complete MRI data were excluded. The impact of various variables on ICH as a mode of presentation was assessed with multivariate binary logistic regression analysis. Kaplan Meier/Cox regression analysis was performed to analyze cumulative 5-year-risk for (re)hemorrhage and to identify baseline predictors of this outcome.
Results:
Seven hundred thirty-one patients with CCM were included. Multivariate logistic regression confirmed a statistically significant negative correlation with DVA (odds ratio [OR] 0.635 [95% confidence interval (CI) 0.459-0.878]) and positive correlation with brainstem localization (OR 6.277 [95% CI 4.287-9.191]) with ICH as the mode of presentation. Among 731 patients, 76 experienced (re)hemorrhage during 2,338 person-years of follow-up. Overall cumulative 5-year risk was 24.1% (95% CI 21.1%-27.5%). Cox regression analysis revealed initial presentation with ICH (hazard ratio [HR] 8.0 [95% CI 3.549-18.122]) and brainstem localization (HR 2.9 [95% CI 1.756-4.765]) as independent baseline predictors of (re)hemorrhage. Presence of DVA added no independent prognostic information (HR 1.1 [95% CI 0.717-1.885]).
Conclusion:
Patients with CCM with associated DVA are at lower risk to present with ICH. During untreated 5-year follow-up, they showed equal (re)hemorrhage risk compared to patients with CCM without DVA.
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