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下肢纤维肌发育不良:临床表现、诊断检测及处理方法

Lower Extremity Fibromuscular Dysplasia: Clinical Manifestations, Diagnostic Testing, and Approach to Management.

作者信息

Brinza Ellen, Lenihan Victoria, Durga Sridevi, O'Connor Sarah, Yesenko Sandra L, Kim Esther S H, Gornik Heather L

机构信息

1 Section of Vascular Medicine, Miller Family Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA.

2 Department of Cardiovascular Medicine, Borgess Medical Center, Kalamazoo, MI, USA.

出版信息

Angiology. 2017 Sep;68(8):722-727. doi: 10.1177/0003319716682121. Epub 2016 Dec 22.

DOI:10.1177/0003319716682121
PMID:28006960
Abstract

Fibromuscular dysplasia (FMD), a disease well described in the renal and cerebrovascular circulations, also manifests in the lower extremity (LE) arteries. This study reports on the clinical presentation, imaging findings, and treatment of patients with LE FMD seen at a single center. Over a 7-year span, 100 of 449 patients with FMD had imaging of the LE arteries, of which 62 were found to have LE FMD (13.8% of the entire FMD cohort including patients with and without LE imaging). The majority of patients were women (96.8%), with an average age of 52 ± 11.3 years at the time of diagnosis. All patients had FMD present in another vascular bed, most commonly in the renal (80.6%) and extracranial carotid arteries (79.0%). Most patients had multifocal FMD (95.2%) and bilateral LE disease (69.4%), with the external (87.1%), common (19.4%), and internal (11.3%) iliac arteries most commonly affected. Presenting symptoms of LE involvement included claudication (22.6%), atypical leg symptoms (14.5%), and dissection (6.5%), but most patients were asymptomatic (71.0%). Nearly all patients were managed conservatively (98.4%) and only 1 patient required intervention.

摘要

纤维肌发育不良(FMD)是一种在肾循环和脑血管循环中已有充分描述的疾病,也可出现在下肢(LE)动脉。本研究报告了在单一中心就诊的下肢FMD患者的临床表现、影像学表现及治疗情况。在7年时间里,449例FMD患者中有100例接受了下肢动脉成像检查,其中62例被发现患有下肢FMD(在整个FMD队列中占13.8%,包括有和没有下肢成像的患者)。大多数患者为女性(96.8%),诊断时的平均年龄为52±11.3岁。所有患者在其他血管床也存在FMD,最常见于肾动脉(80.6%)和颅外颈动脉(79.0%)。大多数患者有多灶性FMD(95.2%)和双侧下肢疾病(69.4%),最常受累的是髂外动脉(87.1%)、髂总动脉(19.4%)和髂内动脉(11.3%)。下肢受累的主要症状包括间歇性跛行(22.6%)、非典型腿部症状(14.5%)和动脉夹层(6.5%),但大多数患者无症状(71.0%)。几乎所有患者都采用保守治疗(98.4%),只有1例患者需要干预。

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引用本文的文献

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A rare case report of bilateral common and internal iliac arterial fibromuscular dysplasia: Coexisted dissection, aneurysm, and stenosis.双侧髂总动脉和髂内动脉纤维肌性发育异常的罕见病例报告:合并夹层、动脉瘤和狭窄。
Medicine (Baltimore). 2017 Dec;96(50):e8896. doi: 10.1097/MD.0000000000008896.