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Prevalence of Intracranial Aneurysm in Women with Fibromuscular Dysplasia a Report from the US Registry for Fibromuscular Dysplasia

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Prevalence of Intracranial Aneurysm in Women with Fibromuscular Dysplasia a Report from the US Registry for Fibromuscular Dysplasia Research JAMA Neurology | Original Investigation Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia A Report From the US Registry for Fibromuscular Dysplasia Henry D. Lather, BS; Heather L. Gornik, MD, MHS; Jeffrey W. Olin, DO; Xiaokui Gu, MA; Steven T. Heidt, BA; Esther S. H. Kim, MD, MPH; Daniella Kadian-Dodov, MD; Aditya Sharma, MBBS; Bruce Gray, DO; Michael R. Jaff, DO; Yung-Wei Chi, DO; Pamela Mace, RN; Eva Kline-Rogers, MS, RN, NP; James B. Froehlich, MD, MPH 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 PARTICIPANTS This 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 MEASURES Percentage of women with FMD with intracranial imaging who had an intracranial aneurysm. RESULTS Of 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 RELEVANCE The 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. Author Affiliations: Author affiliations are listed at the end of this article. Corresponding Author: James B. Froehlich, MD, MPH, Cardiovascular Center, University of Michigan Medical School, 1500 E Medical JAMA Neurol. 2017;74(9):1081-1087. doi:10.1001/jamaneurol.2017.1333 Center Dr, SPC 5853, Ann Arbor, MI Published online July 17, 2017. Corrected on January 2, 2018. 48109 ([email protected]). (Reprinted) 1081 © 2017 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Research Original Investigation Intracranial Aneurysm in Women With Fibromuscular Dysplasia ibromuscular dysplasia (FMD) is an uncommon, non- inflammatory, nonatherosclerotic disease of the me- Key Points dium and large arteries.1,2 Its cause is not known; how- F Question What is the prevalence of intracranial aneurysm in 3 ever, it is far more common in women. It can occur in any women with fibromuscular dysplasia? arterial bed, although it is most commonly found in the renal Findings In this cross-sectional registry study that included 669 and cervical arteries.3 The clinical presentation of FMD var- women with a diagnosis of fibromuscular dysplasia and intracranial ies, depending on the location of the arterial lesions. Often FMD imaging, the prevalence of intracranial aneurysm was 12.9%. is asymptomatic and therefore frequently found inciden- tally. In addition, FMD is associated with a substantial risk of Meaning The prevalence of intracranial aneurysm in women with fibromuscular dysplasia is high enough to warrant consideration of aneurysm formation and rupture, as well as arterial dissec- screening patients with fibromuscular dysplasia for intracranial tion and occlusion.3,4 aneurysm with noninvasive imaging. Previously published data suggest an increased risk of in- tracranial aneurysm (IA) in patients with carotid artery (CA) and vertebral artery (VA) FMD.2 Intracranial aneurysms may confirmed by vascular imaging and currently enrolling at 14 rupture, leading to subarachnoid hemorrhage (SAH) and sig- participating centers. Registry enrollment began in 2008, and nificant morbidity and mortality. The mortality among pa- data were abstracted in September 2015. Registry details and tients who experience SAH is approximately 50%,5 with 12% standardization of data collection have been described dying immediately.6 The most commonly identified cause of previously.3 Written informed consent was obtained from all SAH is IA rupture (85%).7 It is not known whether the natural participants, and all sites had institutional review board ap- history of IA in patients with FMD is similar to that in patients proval (Institutional Review Boards of the University of Michi- without FMD. Although outcomes in patients with SAH are gan Medical School, Cleveland Clinic Foundation Institu- poor,6 sizable IAs identified before rupture can be repaired or tional Review Board, Program for the Protection of Human excluded with endovascular or surgical techniques.8 How- Subjects at Icahn School of Medicine at Mount Sinai, Univer- ever, the risk of treatment must be balanced with the risk of sity of Virginia Institutional Review Board for Health Sci- rupture9; therefore, the clinical benefit of screening for IA, even ences Research, Greenville Health System Institutional Re- in populations with known increased risk, is uncertain.10,11 view Board, Partners Institutional Review Boards, and Previous estimates of IA prevalence among patients with University of California, Davis, Institutional Review Board). All CA and/or VA FMD come from neurosurgery and radiology case data were deidentified. series, which suffer from selection bias in patient inclusion.12 Patients enrolled from all sites in the US Registry for Fibro- Previous studies12 estimated the prevalence of IA to be 21% to muscular Dysplasia who had undergone intracranial imaging at 51%. The most recent estimate was published in a 1998 meta- or before enrollment, as documented on the initial registry data analysis and case series by Cloft and colleagues12 in which IA form, were included. For the purposes of this report, intracra- prevalence was calculated with and without IA symptoms (eg, nial imaging was defined as catheter-based angiography, com- SAH) among patients with CA and/or VA FMD who underwent puted tomographic angiography, or magnetic resonance angi- cerebral angiography.In most patients with IA in that series, SAH ography (MRA). Patients were also included in this study if they was the indication for angiography; FMD was often inciden- had a known repaired IA. Patients younger than 18 years at the tally found. Among asymptomatic patients with FMD, there was time of FMD diagnosis were excluded because of concern that a mean (SD) prevalence of IA of 7.3% (2.2%; 38 of 517 patients). FMD and IAs in children may be different from those in adults.8,13 In the 2014 American Heart Association (AHA) Scientific Because male sex may affect the pathophysiologic mechanism Statement on FMD, the writing committee identified the deter- of FMD14 and aneurysms,15 male patients were also excluded. mination of the prevalence of IA among patients with FMD as In addition, male patients constituted only 5.7% of individuals a top research priority.2 Although there have been several esti- in the registry. Although positive family history of IA or SAH in- mates of IA prevalence among patients with FMD in the CAs creases the likelihood of IA by 3.4-fold,15 this information was and/or VAs, to our knowledge, there are no published reports not available in the database. of the prevalence of IA among patients with FMD in the renal Intracranial aneurysm was defined as a saccular or broad- arteries. Because of the life-threatening consequences of rup- based aneurysm greater than or equal to 2 mm in greatest di- tured IAs and the possible association with FMD, the writing mension occurring at or above the level of the skull base. Ex- committee recommended IA screening for all patients with FMD tradural (but intracranial) aneurysms in the petrous segment found in any arterial bed. The current study uses the largest da- of the internal CA (C2 in the Bouthillier classification16) and tabase of patients with FMD, the US Registry for Fibromuscu- above were included. To ensure that the definition of IA was met, lar Dysplasia, to estimate the prevalence of IA and characterize all available imaging reports from each patient with a CA aneu- IAs in FMD. rysm, VA aneurysm, and/or IA were reviewed and assessed using these criteria. If patients had multiple imaging reports, the larg- est recorded dimension of each IA was used. Any registry site Methods that was unable to provide deidentified imaging reports to the coordinating center was excluded from the analysis. The US Registry for Fibromuscular Dysplasia is an observa- To address the question of whether IA is more prevalent in tional disease-based registry of patients with diagnosis of FMD patients with cervical FMD vs noncervical FMD, we analyzed 1082 JAMA Neurology September 2017 Volume 74, Number 9 (Reprinted) jamaneurology.com © 2017 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Intracranial Aneurysm in Women With Fibromuscular Dysplasia Original Investigation Research Table 1.
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