Fibromuscular dysplasia: what the radiologist should know: a pictorial review

Abstract

Fibromuscular dysplasia (FMD) is an idiopathic, segmentary, non-inflammatory and non-atherosclerotic disease that can affect all layers of both small- and medium-calibre arteries. The prevalence of FMD is estimated between 4 and 6 % in the renal arteries and between 0.3 and 3 % in the cervico-encephalic arteries. FMD most frequently affects the renal, carotid and vertebral arteries, but it can theoretically affect any artery. Radiologists play an important role in the diagnosis of FMD, and good knowledge of FMD’s signs will certainly help reduce the delay between the first symptoms and diagnosis. The common string-of-beads aspect is well known, but less common presentations also have to be considered. These less common imaging findings include vascular loops, fusiform vascular ectasia, arterial dissection, aneurysm and subarachnoid haemorrhage. These radiologic presentations should be known by radiologists in order to diagnose possible FMD, particularly when present in young females or when associated with personal or familial hypertension, to reduce the delay between the onset of the first symptom and the final diagnosis. The patients have to be referred to specialised FMD centres for dedicated management.Teaching Points• Fibromuscular dysplasia is not a rare disease.• Radiologists should recognise less common presentations to orient specific management.• Vascular loops, fusiform vascular ectasia and a “string-of-beads” aspect are typical presentations.• Arterial dissection, aneurysm and subarachnoid haemorrhage are less typical radiologic presentations.

Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association

“…Magnetic resonance angiography has lower spatial resolution than computed tomography angiography for FMD diagnosis, 12,272 but newer magnetic resonance angiography can take advantage of volume rendering and strong magnets (3.0 T) that have increased the sensitivity for detection of aneurysms, despite the potential for false-negative and false-positive results. 273 Benefits of magnetic resonance angiography include the absence of radiation, highly sensitive imaging of the brain parenchyma, and no requirement for contrast agent.…”

Revisiting Fibromuscular Dysplasia

“…However, the gold standard remains catheter-based angiography. 23 In equivocal cases, intravascular ultrasound and pressure measurements can help to assess the hemodynamic significance of a stenosis and the anatomic success after percutaneous intervention. 1 The treatment of patients with renal FMD may include medical therapy with surveillance, endovascular therapy (angioplasty without stenting), or surgery.…”

Carotid artery fibromuscular dysplasia: Ultrasound and CT imaging

“…1,2 It affects mostly women between 20 to 60 years old and may occur in nearly any artery. 1,2 It affects mostly women between 20 to 60 years old and may occur in nearly any artery.…”

“…2,11,17 Therefore, both magnetic resonance angiography (MRA) and CTA appear to be more sensitive for the detection of carotid FMD. Whereas carotid atherosclerotic disease is usually located F I G U R E 1 Carotid duplex ultrasonography with pulsed wave Doppler interrogation of the mid-distal right internal carotid artery (arrow).…”