Permanent neurological morbidity after microsurgical resection of hereditary hemorrhagic telangiectasia-associated brain arteriovenous malformations: a systematic review and illustrative case

Abstract

Introduction The optimal management of brain arteriovenous malformations (AVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) remains controversial. Some authors recommend conservative management based on the presumed lower rupture risk compared to sporadic AVMs, others advocate surgical treatment. No systematic review has focused on permanent neurological morbidity following resection of HHT-associated AVMs, leaving a significant gap in the literature. Research question Do the unique genetic features and angio-architecture of HHT-associated AVMs result in a different profile of postoperative neurological morbidity compared to sporadic AVMs? Materials and methods A systematic search of PubMed, Embase, and Cochrane databases (1947–April 2026) was conducted per PRISMA 2020 guidelines; 18 publications reporting surgically treated HHT-associated brain AVMs were identified, 12 were included in the comparative analysis. Study quality was assessed using the Joanna Briggs Institute Critical Appraisal Checklist. Permanent morbidity rates were compared with unselected sporadic AVM series using Fisher's exact test with sensitivity analyses. Results Among 91 patients, pooled incidence of new permanent neurological deficits was 9.9% (95% CI: 4.6–18.0%). No significant difference was found compared to unselected sporadic AVM cohorts. One study contributed 78% of permanent deficit events; its exclusion reduced the rate to 2.9%. No postoperative mortality was reported. Discussion and conclusion No significant difference in permanent neurological morbidity was demonstrated between HHT-associated and sporadic AVM resections. However, the small sample size precludes definitive conclusions. These hypothesis-generating findings may aid in individualized risk assessment and shared decision-making. An illustrative case is presented to demonstrate the clinical context motivating this review.