Clinical course of patients with conservatively managed cerebral cavernous malformations

Abel Clemens Adriaan Sandmann; Marinus Abraham Kempeneers; René van den Berg; Dagmar Verbaan; William Peter Vandertop; Jonathan M Coutinho

doi:10.1177/23969873241246868

Clinical course of patients with conservatively managed cerebral cavernous malformations

Eur Stroke J. 2024 Apr 16:23969873241246868. doi: 10.1177/23969873241246868. Online ahead of print.

Authors

Abel Clemens Adriaan Sandmann¹, Marinus Abraham Kempeneers², René van den Berg³, Dagmar Verbaan², William Peter Vandertop², Jonathan M Coutinho¹

Affiliations

¹ Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
² Department of Neurosurgery, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
³ Department of Radiology and Nuclear Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

PMID: 38624046
DOI: 10.1177/23969873241246868

Abstract

Introduction: There is uncertainty whether patients with a cerebral cavernous malformation (CCM) should undergo conservative or surgical treatment, resulting in practice variation among hospitals. Our objective was to report clinical outcomes of patients with primarily conservatively managed CCMs.

Patients and methods: This single-center cohort study included consecutive adult CCM patients, diagnosed in 2000-2023, who underwent conservative management as primary treatment strategy. Data were extracted from medical records, and we systematically conducted telephone and questionnaire follow-up. Functional status was assessed on the modified Rankin Scale (mRS).

Results: Of 345 patients, we included 265 patients with a CCM (median age 46 years; 45% male). At baseline, 131 (49%) patients presented with symptomatic hemorrhage (SH), and 134 (51%) with other symptoms or asymptomatically. During 58 months (IQR 35-94) median follow-up, 51 (19%) patients experienced a SH, 33 (12%) a seizure, and 13 (5%) focal neurological deficits. Fourteen (5%) patients underwent intervention (surgery n = 11, radiosurgery n = 4). Presentation with SH was associated with higher annual bleeding rates (6.0% vs 1.5%, p < 0.001), and higher cumulative 5-/10-year bleeding risks (31%/41% vs 7%, p < 0.001). Brainstem CCM was associated with higher cumulative 5-/10-year bleeding risks (27%/38% vs 17%/21%, p = 0.038). Nineteen (7%) patients died; two (0.8%) directly attributable to CCM. Of 246 surviving patients, 205 (83%) completed the questionnaire. At follow-up, 172/224 (77%) patients were functionally independent (mRS score ⩽2).

Discussion and conclusion: The majority of conservatively managed CCM patients remained free of a SH during follow-up. Few patients required intervention, and death attributable to the CCM was rare. These data may help patient counseling and treatment decisions.

Keywords: Cavernous malformation; cavernoma; cavernous angioma; cavernous hemangioma; conservative management; natural history; surgery; symptomatic hemorrhage; treatment.