Recent Developments in the Understanding and Management of Fibromuscular Dysplasia

O’Connor, Sarah; Gornik, Heather L.

doi:10.1161/jaha.114.001259

Cited by 23 publications

(22 citation statements)

References 56 publications

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“…8 Of the three types of FMD (intimal, medial, and adventitial), medial is the most common representing approximately 90% of fibromuscular disease. 5,[9][10][11][12] The medial type of FMD is associated with the diagnostic "string-of-beads" sign noted by imaging techniques. 6,11,[13][14][15] Cerebral aneurysms appear to be more prevalent in patients with FMD, as are aneurysms of mostly any medium-sized artery.…”

Section: Discussionmentioning

confidence: 99%

“…In middle-aged females, FMD should be considered in the presence of a carotid bruit, arterial dissection, or an aneurysm of the aorta or intracranial vessel. 3,5,11,12 F I G U R E 3 Volume-rendered computed tomography angiography images of the great vessels in a slightly rotated coronal view. The LICA (horizontal arrow) and right internal carotid artery (oblique arrow) reveal a "string-ofbeads" pattern in both distal vessels.…”

Section: Pre S Entati On and Phys I C Al E X Aminationmentioning

confidence: 99%

“…6 A large autopsy study found the prevalence of carotid FMD to be 0.02% (4 of 20 244 autopsies). 5,[9][10][11][12] The medial type of FMD is associated with the diagnostic "string-of-beads" sign noted by imaging techniques. 3 Tobacco abuse seems to be associated with FMD to some extent.…”

Section: Introductionmentioning

confidence: 99%

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Carotid artery fibromuscular dysplasia: Ultrasound and CT imaging

Kerut

Sheahan

2019

Echocardiography

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A 54-year-old Caucasian female was referred by her primary care physician for evaluation of a right carotid bruit heard on routine physical examination. The patient was asymptomatic with no neurological or cardiovascular-related symptoms. There was no history of hypertension, diabetes mellitus, or tobacco abuse. The patient's only medication was simvastatin 20 mg/day for a diagnosis of hypercholesterolemia. Family history was pertinent for a sister diagnosed as developing hypertension of unknown etiology as a teenager.Physical examination was unremarkable except for a long systolic high pitched right carotid bruit heard best high in the neck. Carotid duplex ultrasound revealed intimal left carotid disease with normal velocities and laminar color Doppler flow within the left internal carotid artery (ICA). Evaluation of the right carotid artery revealed intimal disease with an increase in velocity (140 cm/s peak systolic velocity) in the distal right ICA (Figure 1). Color flow Doppler turbulence was also noted in the distal right ICA (Figure 2). Both vertebral arteries demonstrated normal Doppler velocities.Because of the quality and location of the right carotid bruit, and an increase in Doppler velocity and turbulence in the high right ICA by carotid duplex scanning, computed tomography angiography (CTA) was performed of the great vessels. A "string-of-beads" sign was noted in both distal ICAs, diagnostic of fibromuscular dysplasia (FMD) (Figures 3-4). CTA screening of the abdomen and pelvis revealed no evidence of FMD elsewhere. | D ISCUSS I ONFibromuscular dysplasia is a noninflammatory, nonatherosclerotic disease of unknown etiology. 1,2 It affects mostly women between 20 to 60 years old and may occur in nearly any artery. 3 Most often it affects the renal arteries with an incidence of about 80% of all An asymptomatic 54-year-old Caucasian female was found to have a right carotid bruit located high in the cervical region. Carotid duplex ultrasound revealed carotid intimal disease with increased Doppler flow in the mid-distal right internal carotid artery (RICA) with color Doppler turbulence in the distal RICA. Computed tomography angiography (CTA) revealed a typical "string-of-beads" sign in both distal internal carotid artery (ICAs), diagnostic of fibromuscular dysplasia (FMD). While often notdiagnostic, carotid duplex ultrasound may yield clues as to diagnosis of FMD with elevated Doppler velocity in the distal carotid, turbulent distal carotid flow, and possibly a "string-of-beads" sign. An "S-curve" distal ICA is associated with FMD and may warrant further evaluation. Magnetic resonance angiography and CTA appear to be better for diagnosis of carotid FMD. The typical "string-of-beads" may be readily identified. While invasive angiography is considered the "gold standard" for diagnosis of carotid FMD, it is infrequently required. Generally, invasive angiography is performed in symptomatic patients in planning for interventional therapy. K E Y W O R D Sbruit, CT, FMD, ultrasound

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Section: Discussionmentioning

confidence: 99%

Section: Pre S Entati On and Phys I C Al E X Aminationmentioning

confidence: 99%

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Carotid artery fibromuscular dysplasia: Ultrasound and CT imaging

Kerut

Sheahan

2019

Echocardiography

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“…In order to attribute aneurysm or dissection to FMD, evidence of FMD lesions in another vascular bed is required. Diagnosis of FMD requires exclusion of VA spasm, median arcuate ligament syndrome (triad of postprandial abdominal pain, weight loss and often an abdominal bruit due to compression of the coeliac trunk and eventually the SMA by this ligament), trauma, atherosclerosis (but patients may have both), inflammatory arterial diseases (large artery vasculitis, ie, Takayasu arteritis, giant‐cell arteritis), segmental arterial mediolysis, arterial diseases of monogenic origin (ie, vascular type of Ehlers‐Danlos syndrome, neurofibromatosis type 1, …) …”

Section: Screening and Diagnosismentioning

confidence: 99%

“…After diagnosis is established, surveillance imaging of affected vascular beds has to be tailored for the individual FMD patient, depending on severity, location and nature (stenosis, aneurysm, dissection) of FMD lesions. 41 Nowadays, there is no optimal monitoring protocol in case of medical treatment alone, or post revascularization in VA FMD. If no revascularization procedure has been performed, clinical FU, at least annually, and imaging studies to evaluate progression of disease are advisable.…”

Section: Of or After Revascularizationmentioning

confidence: 99%

Visceral Fibromuscular Dysplasia: From asymptomatic disorder to emergency

Tussenbroek

Devos

Debing

et al. 2018

Eur J Clin Investigation

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Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. The clinical picture is very diverse, and diagnosis is based on CT-, MR- or conventional catheter-based angiography. Involvement of VAs generally occurs among patients with multi-vessel FMD. Therefore, screening for VA FMD is advised especially in renal artery (RA) FMD and in case of aneurysms and/or dissections. Treatment depends on the clinical picture. However, the level of evidence is low, and much of the common practice is extrapolated from visceral atherosclerotic disease.

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