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Updated: Mar 27, 2026
Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Outcomes of Stereotactic Radiosurgery for Brain Arteriovenous Malformations in Patients With Hereditary Hemorrhagic
Takenori Akiyama1, Yumiko Okada2, Jun Kawagishi3
1Department of Neurosurgery, Keio University, School of Medicine, Tokyo, Japan.
Background And Objectives:
Brain arteriovenous malformations (BAVMs) in hereditary hemorrhagic telangiectasia (HHT) differ genetically and structurally from sporadic BAVMs. However, the efficacy and safety of stereotactic radiosurgery (SRS) for HHT-BAVMs remain unclear. This study aimed to evaluate the outcomes of SRS for HHT-BAVMs in the largest cohort to date.
Methods:
We retrospectively analyzed 31 patients with definite HHT (Curaçao criteria 3 and 4 or genetically confirmed) who underwent SRS for 63 BAVM nidi at 12 Gamma Knife centers from 2003 to 2021. Clinical and treatment characteristics were collected, and outcomes were evaluated. Kaplan-Meier analysis was used to estimate cumulative obliteration rates. Cox proportional hazards models identified factors associated with nidus obliteration.
Results:
The median age was 28 years; the median follow-up was 74 months (range, 6-230 months). Most lesions (95%) were Spetzler-Martin grade I or II. The median nidus size was 9.0 mm. The median target volume was 0.40 mL (range, 0.02-13.8 mL), and the median marginal dose was 20.0 Gy (range, 14.9-24.0 Gy). Obliteration was confirmed in 46 nidi (27 by digital subtraction angiography). The 5- and 7-year cumulative obliteration rates were 61.9% and 81.2%, respectively. The median time to obliteration was 40 months. Multivariate analysis showed that smaller nidus size (P = .008) and higher marginal dose (P = .004) were independent predictors of obliteration. No symptomatic hemorrhages from BAVMs occurred during follow-up. Radiation-induced changes were observed in 23.8% of nidi; only 6.5% of patients experienced transient symptoms. Functional outcomes remained stable in 93.5% of patients, with 87.1% maintaining modified Rankin Scale 0 to 1 at the last follow-up. One patient died from non-AVM-related hemorrhage, and 2 patients developed de novo AVMs during follow-up.
Conclusion:
SRS for HHT-BAVMs achieved high obliteration rates with minimal morbidity. Given the low rate of hemorrhage and favorable functional outcomes, SRS could be considered a safe and effective treatment option for this unique patient population.
