Prevalence and Characteristics of Intracranial Aneurysms in Hereditary Hemorrhagic Telangiectasia.

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.

Transvenous retrograde pressure cooker technique for embolization of an ethmoidal dural arteriovenous fistula.

SummaryEthmoidal dural arteriovenous fistulas (DAVFs) are rare lesions, accounting for approximately 10% of intracranial DAVFs.1 2 As ethmoidal DAVFs commonly demonstrate cortical venous drainage, treatment is always warranted.2-6 Endovascular transvenous embolization has been increasingly reported as an effective and safe treatment for ethmoidal DAVFs, and since occlusion of the central retinal artery and resulting blindness are not a concern, it has an advantage over transarterial embolization.3-6 To ensure curative embolization, we adopted the transvenous retrograde pressure cooker technique (RPCT), creating a plug with n-butyl cyanoacrylate (NBCA) in the draining vein to allow a more comprehensive and efficient injection of Onyx (Medtronic, MN) while avoiding excessive reflux.7 8 In this technical video (video 1), we report the first case using the transvenous RPCT for successful Onyx embolization of an ethmoidal DAVF, with emphasis on the technical nuances of the RPCT and important tips to avoid periprocedural complications.neurintsurg;jnis-2023-020393v1/V1F1V1Video 1 Video demonstrating Onyx embolization of an ethmoidal dural arteriovenous fistula using the transvenous retrograde pressure cooker technique.