51. Intracranial Aneurysms in Loeys-Dietz Syndrome: A Multicenter Propensity-Matched Analysis.
- Author
-
Perez-Vega C, Domingo RA, Tripathi S, Ramos-Fresnedo A, Martínez Santos JL, Rahme RJ, Freeman WD, Sandhu SS, Miller DA, Bendok BR, Brinjikji W, Quinones-Hinojosa A, Meyer FB, Tawk RG, and Fox WC
- Subjects
- Diagnostic Imaging, Humans, Intracranial Aneurysm diagnostic imaging, Intracranial Aneurysm epidemiology, Loeys-Dietz Syndrome complications, Loeys-Dietz Syndrome diagnosis, Loeys-Dietz Syndrome epidemiology
- Abstract
Background: Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by a classic triad of hypertelorism, bifid uvula and/or cleft palate, and generalized arterial tortuosity. There are limited data on the prevalence and rupture risk of intracranial aneurysms (IAs) in the setting of LDS, with no established guidelines., Objective: To analyze the prevalence and rupture risk of IA in LDS., Methods: Electronic medical records of patients with a confirmed diagnosis of LDS and available cerebrovascular imaging were reviewed. Patients were divided into 2 groups based on the presence of IA. Unmatched and propensity-matched analyses were used to identify potential risk factors for aneurysm formation., Results: Records of 1111 patients were screened yielding a total of 60 patients with a diagnosis of LDS. Eighteen (30%) patients had IA, 4 (22.2%) of whom had multiple aneurysms for a total of 24 IAs. Twenty-three (95.8%) aneurysms were located in the anterior circulation; none of them were ruptured. On unmatched analysis, age ( P = .015), smoking history ( P = .034), hypertension ( P = .035), and number of extracranial aneurysms ( P < .001) were significantly higher in patients with IA. After matching for age, sex, race, stroke history, family history, and extracranial aneurysms, smoking history ( P = .009) remained significant., Conclusion: Patients with LDS have an increased risk of IAs, especially with a history of smoking. The prevalence rate of IAs in our series was 30%. Screening imaging should be considered at diagnosis, and patients should be encouraged to abstain from smoking. Further studies are needed to elucidate the risk of IA rupture and treatment considerations in this unique population., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)
- Published
- 2022
- Full Text
- View/download PDF