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RT Journal Article
SR Electronic
T1 Prevalence and Characteristics of Intracranial Aneurysms in Hereditary Hemorrhagic Telangiectasia
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
DO 10.3174/ajnr.A8058
A1 Cheng, How-Chung
A1 Faughnan, Marie E.
A1 terBrugge, Karel G.
A1 Liu, Hon-Man
A1 Krings, Timo
A1 the Brain Vascular Malformation Consortium Hereditary Hemorrhagic Telangiectasia Investigator Group
YR 2023
UL http://www.ajnr.org/content/early/2023/11/30/ajnr.A8058.abstract
AB BACKGROUND AND PURPOSE: The association between hereditary hemorrhagic telangiectasia and intracranial aneurysms remains controversial. This study evaluated the prevalence and characteristics of intracranial aneurysms in patients with hereditary hemorrhagic telangiectasia with brain vascular malformations.MATERIALS AND METHODS: Between 2007 and 2021, patients enrolled in the Brain Vascular Malformation Consortium with definite hereditary hemorrhagic telangiectasia, the presence of brain vascular malformations, and available angiographic studies of the brain were retrospectively reviewed. Angiographic features of intracranial aneurysms and their relationship to coexisting brain vascular malformations were analyzed. We also examined the association between baseline clinical features and the presence of intracranial aneurysms.RESULTS: One hundred eighty patients were included. A total of 14 intracranial aneurysms were found in 9 (5%) patients, and 4 intracranial aneurysms were considered flow-related aneurysms. Patients with intracranial aneurysms were significantly older than patients without intracranial aneurysms (mean, 48.1 [SD, 18.2] years versus 33.5 [SD, 21.0] years; P = .042). If we excluded flow-related intracranial aneurysms, the prevalence of intracranial aneurysms was 3.3%. All intracranial aneurysms were in the anterior circulation, were unruptured, and had an average maximal diameter of 3.9 (SD, 1.5) mm. No intracranial aneurysms were found in pediatric patients with hereditary hemorrhagic telangiectasia. No statistically significant correlation was observed among other baseline demographics, hereditary hemorrhagic telangiectasia features, and the presence of intracranial aneurysms.CONCLUSIONS: The prevalence of intracranial aneurysms in this large cohort study is comparable with that in the general population and might be increased slightly due to hemodynamic factors associated with shunting brain vascular malformations.HHThereditary hemorrhagic telangiectasiaIAintracranial aneurysmVMvascular malformation