Outcomes of Stereotactic Radiosurgery for Brain Arteriovenous Malformations in Patients With Hereditary Hemorrhagic Telangiectasia: A Multicenter Retrospective Study.
retrospective_cohort · Level III
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- Also identified by DOI 10.1227/neu.0000000000004003.
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Abstract
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. 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. 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. 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.