Intracranial Aneurysms in Patients with Marfan Syndrome: A Multicenter Propensity-Matched Analysis

Abstract

We sought to identify risk factors for intracranial aneurysms (IAs) in Marfan syndrome (MFS) patients and to describe their characteristics. Patients with confirmed MFS and vessel-dedicated brain imaging from January 1, 1980-December 1, 2020 were categorized according to the presence (IA) or absence (NIA) of IAs. Unmatched logistic regression analysis and propensity score matching were used for comparison. We included 159 patients, of whom 18 (11.3%) patients had radiographic diagnosis of IAs. Three patients (16.7%) had multiple lesions for a total of 24 IAs. One patient (5.5%) had de novo IA formation. Four patients (22.2%) underwent treatment: Two (11.1%) had open surgical clipping for ruptured aneurysms, and two (11.1%) patients had endovascular embolization for growth. In the unmatched analysis, current tobacco smoker status (odds ratio [OR]: 4.20; confidence interval [CI]: 1.11-15.6; P= 0.027) and history of coronary artery disease (CAD) (OR: 5.79; CI 1.76-20.2; P= 0.004) increased the odds for IA. Propensity score matching yielded 18 IA and 18 NIA patients matched for age, gender, race, priorstroke, and family history of aneurysms. History of CAD (IA= 11 [61.1%] vs. NIA= 4 (22.2%), P= 0.043) and current smoker status (IA= 6 [33.3%] vs. NIA= 0 (0%), P < 0.01) weresignificantly higher in the IA cohort. Body mass index (P= 0.622), diabetes (P= 0.180), hypertension (P= 0.732), prior stroke (P= 1.00), family history (P= 0.732), alcohol (P= 0.314), recreational drugs (P= 1.00), and other aneurysms (P= 0.585) were not statistically significant. Prevalence of IAs in our series of MFS patients was 11.3%, and de novo formation was 5.5%. MFS patients with a history of CAD and current smoker status had an increased risk of IA. Neurovascular radiographic screening should be considered in all patients with MFS, particularly in patients who smoke or have a history of heart disease.