Fibromuscular dysplasia what the radiologist shou

Authors: L Varennes F Tahon A Kastler S Grand F Thony J P Baguet O Detante E Touzé A Krainik

Publish Date: 2015/04/30

Volume: 6, Issue: 3, Pages: 295-307

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Abstract

Fibromuscular dysplasia FMD is an idiopathic segmentary noninflammatory and nonatherosclerotic disease that can affect all layers of both small and mediumcalibre arteries The prevalence of FMD is estimated between 4 and 6  in the renal arteries and between 03 and 3  in the cervicoencephalic 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 stringofbeads 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 managementFibromuscular dysplasia FMD is an idiopathic segmentary noninflammatory and nonatherosclerotic disease that can affect all layers of both small and mediumcalibre arteries Although not known the FMD prevalence in the general population is considered to be low by several authors 1 The cause and pathophysiology of FMD are still unknown 2 3 Clinical manifestations of FMD are primarily dependent on the vessels that are involved FMD most frequently affects the renal carotid and vertebral arteries but can be found in all territories 3 It is a potentially serious disease especially in the cervicoencephalic location where it can lead to severe stenosis hypoperfusion aneurysm subarachnoid haemorrhage dissection or arterial occlusion 3 4 5FMD is often not considered in the differential diagnosis because radiological aspects can be difficult to diagnose and the symptoms reported are not specific Therefore the mean delay from symptoms to diagnosis has been reported to be up to 41 years 6Radiologists play an important role in the diagnosis of FMD and a good knowledge of FMD’s presentations should help to reduce the delay between the first symptoms and final diagnosis The objective of this review is therefore to raise radiologists’ awareness of FMD’s epidemiology pathophysiology clinical presentation typical and atypical radiological aspects and possible complications

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