Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia

Abstract

IMPORTANCE The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain.OBJECTIVE To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. DESIGN, SETTING, AND PARTICIPANTSThis cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers. Registry enrollment began in 2008, and data were abstracted in September 2015. Patients younger than 18 years at the time of FMD diagnosis were excluded. Imaging reports of all patients with reported internal carotid, vertebral, or suspected intracranial artery aneurysms were reviewed. Only saccular or broad-based aneurysms 2 mm or larger in greatest dimension were included. Extradural aneurysms in the internal carotid artery were included; fusiform aneurysms, infundibulae, and vascular segments with uncertainty were excluded. MAIN OUTCOMES AND MEASURESPercentage of women with FMD with intracranial imaging who had an intracranial aneurysm. RESULTSOf 1112 female patients in the registry, 669 (60.2%) had undergone intracranial imaging at the time of enrollment (mean [SD] age at enrollment, 55.6 [10.9] years). Of the 669 patients included in the analysis, 86 (12.9%; 95% CI, 10.3%-15.9%) had at least 1 intracranial aneurysm. Of these 86 patients, 25 (53.8%) had more than 1 intracranial aneurysm. Intracranial aneurysms 5 mm or larger occurred in 32 of 74 patients (43.2%), and 24 of 128 intracranial aneurysms (18.8%) were in the posterior communicating or posterior arteries. The presence of intracranial aneurysm did not vary with location of extracranial FMD involvement. A history of smoking was significantly associated with intracranial aneurysm: 42 of 78 patients with intracranial aneurysm (53.8%) had a smoking history vs 163 of 564 patients without intracranial aneurysm (28.9%; P < .001). CONCLUSIONS AND RELEVANCEThe prevalence of intracranial aneurysm in women diagnosed with FMD is significantly higher than reported in the general population. Although the clinical benefit of screening for intracranial aneurysm in patients with FMD has yet to be proven, these data lend support to the recommendation that all patients with FMD undergo intracranial imaging if not already performed.

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

“…Registry [5]. The most common locations of dissection were the cerebrovascular and renal arteries [5,22]. Due to this high rate of aneurysm and dissection, it is recommended that every patient with FMD undergo one-time cross-sectional imaging from head-to-pelvis with CTA or MRA [5,9,[23][24][25][26].…”

Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia

“…The clinical presentation varies, depending on the location of the arterial lesions. 3 The most common manifestation is renovascular hypertension secondary to involvement of the renal artery. The FMD of the cervical artery, which mainly affects the ICA, is rarely symptomatic.…”

“…1,2 Fibromuscular dysplasia can occur in any artery, although it is more prevalent in the renal and cervical arteries. 3 We report a case of FMD presenting with subarachnoid hemorrhage (SAH) due to left vertebral artery (lfVA) dissection, treated successfully by angioplasty.…”

Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia

“…By including the extracranial carotid and vertebral artery, the prevalence of aneurysms rose to 12.3% and the prevalence of dissection along the carotid or vertebral artery was 21.7% [3]. A more recent report from the US FMD registry found that 12.9% of women with FMD who had vascular imaging had an intracranial aneurysm [4]. Even after adjusting for the preponderance of women among people with FMD and people with aneurysm, the prevalence of aneurysms among FMD patients exceeds the population average, which is between 0.4 and 6.0%, higher in prospective angiographic studies and lower in retrospective autopsy studies [11].…”

“…The medial type, the most common histopathology, is characterized on angiography by alternating fibrous constrictions and aneurysmal dilations, commonly referred to as “string of beads” [1]. Of relevance to neurosurgeons and neurointerventionalists, the prevalence of cervical dissection and intracranial aneurysms is increased in patients with FMD [2,3,4]. As more such lesions are incidentally discovered, the presence of FMD can serve as discouragement to elective treatment of cerebral aneurysms [5].…”