Abstract 18: Defining Clinical Presentation, Imaging Findings, and Management of Intracranial Fibromuscular Dysplasia

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Introduction: Intracranial (IC) artery involvement in fibromuscular dysplasia (FMD) may manifest as stenosis, dissection, or cerebral aneurysm. We sought to identify clinical, imaging, and treatment characteristics of FMD patients with IC manifestations.

Methods: Analysis of prospectively collected data from a single center of the US FMD patient registry with additional medical record and imaging review. Patients were consented for enrollment if FMD was confirmed by imaging in at least one vascular territory.

Results: From 2/2/2009-7/10/2015, 474 FMD patients were enrolled in the registry. Of the 421 patients with IC arterial imaging, 52 (12.4%) had IC FMD, with catheter angiography available in 29 (55.8%) patients. Median age of initial FMD symptoms was 47 years (IQR 38 - 55) and 51 patients were female. Concomitant extracranial (EC) carotid FMD was present in 92.3%, vertebral FMD in 48.9%, and renal FMD in 50.0%. Among 52 patients with IC FMD, 41 (78.8%) had IC aneurysm (9.7% of entire cohort with IC imaging), 7 (13.5%) had IC dissection, 6 (11.5%) had focal IC stenosis, and 2 (3.8%) had multifocal IC FMD. Fifteen patients with IC FMD (28.8%) were symptomatic (9 TIA/ischemic stroke, 2 hemorrhagic stroke, 4 isolated cranial neuropathy). Among patients with IC FMD, 22 (42.3%) required intervention. Treatment included EC-IC carotid artery bypass in 2 patients, aneurysm coiling in 13, aneurysm clipping in 9, and IC stenosis stenting in 1 patient. At the time of last follow-up, 47 patients (90.4%) were treated with an anti- platelet agent.

Conclusion: In a single center cohort, 12.4% patients with FMD had IC involvement. Arterial aneurysm was the most common manifestation followed by dissection and stenosis. Aneurysm was present in 9.7% of patients who underwent IC imaging, highlighting the importance of comprehensive vascular assessment of patients with FMD.

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