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

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.

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

“…According to the recent registries, FMD involving the iliac arteries is present in 7%-62% of patients [9,11,12,14]. Most patients with lower extremity involvement are asymptomatic and may be diagnosed by femoral bruit noted on physical examination or incidentally on imaging studies.…”

“…Surgery is reserved for complicated cases, progressive aneurysm growth following dissection or for treating complications of endovascular treatment. However, most patients are asymptomatic and can be managed conservatively including antiplatelet therapy [14].…”

“…Aneurysms, dissections, and arterial tortuosity are also frequent manifestations of the disease, but these lesions can only be attributed to FMD in the presence of a typical string-ofbeads or focal stenosis, as these lesions have been described in other vascular diseases as well [3,[6][7][8]. Renal and cerebrovascular arteries are most frequently involved, but all other mediumsized arteries may be affected [3,[9][10][11][12][13][14]. Involvement of >1 vascular bed, i.e.…”

A rare case report of bilateral common and internal iliac arterial fibromuscular dysplasia

“…A total of 104 cases on iliac arterial FMD were reported (Table 1 ). [ 3 – 16 ] The mean age at diagnosis was 52.2 ± 12.6 years, and woman accounted for 86.5%. In terms of clinic presentation related to iliac arteries, 54 (51.9%) patients were asymptomatic, while 43 (41.3%) patients presented intermittent claudication or leg pain (an atypical symptom of claudication).…”

“…[ 2 ] However, iliac arterial FMD was limited reported in case reports or series, and the demographic, clinical, and imaging features of iliac arterial FMD have not been precisely described. [ 3 – 16 ] We herein report the first man case of bilateral common and internal iliac arterial FMD in China, with renal artery involvement discovered by computed resonance angiography (CTA) and contaminant stenosis, aneurysm, and dissection, and then perform a literature review on iliac arterial FMD.…”