Prevalence of intracranial aneurysms in Marfan syndrome

Objective: Aneurysmal pathology of the aorta is well-defined in the Marfan syndrome (MFS) population. Owing in part to the rarity of pathologies, the prevalence of intracranial aneurysms (IA) in MFS is poorly defined. There is debate as to whether or not there is an association between the two. The aim of this study was to evaluate the prevalence of IA in a population of patients with MFS who underwent intracranial imaging. Methods: This was a single-center retrospective review of patients with MFS. Between 1995 and 2021, 983 patients were reviewed. We identified 198 patients with MFS who had intracranial imaging. Imaging consisted of CTA and/or MRA, and was read by an attending radiologist. Details of the aneurysm, patient demographics, and aortic characteristics were collected. Results: The prevalence of IA was 7.1% (14/198). Age of patients with IA (55.0 +/- 15.1 years) was not significantly different than those without IA (52.6 +/- 16.0 years) (P = .58). The most common location of IA was the internal carotid artery. The mean diameter of the IA was 7 +/- 5.8 mm. No ruptures of the internal carotid artery were identified. One patient (0.5%) underwent intervention for the IA. There were no significant differences found in aortic characteristic including dimensions, history of dissection, or aneurysm. Conclusions: In a large, single-center experience over 20 years, we identified patients with confirmed MFS who underwent intracranial imaging. The prevalence of IA in our experience was 7.1%. There were no patient or aortic characteristics found to be significantly associated with IA; however, this finding may be due to the small number of aneurysms. Although this number is higher than the historically reported prevalence in the general population, a collection of experiences from multiple institutions will likely be required to truly define the risk of IA in MFS and to determine whether screening is warranted.

Automated summary

This study evaluated the prevalence of intracranial aneurysms (IA) in patients with Marfan syndrome (MFS) who underwent intracranial imaging, and found a prevalence rate of 7.1%. There were no significant associations between age, aortic characteristics, and the occurrence of IA, but this finding may be limited by the small number of aneurysms.