Fibromuscular dysplasia

David S Liebeskind MD (Dr. Liebeskind of the University of California, Los Angeles, received consultation fees from Stryker and Medtronic.)
Steven R Levine MD, editor. (Dr. Levine of the SUNY Health Science Center at Brooklyn has received honorariums from Genentech for service on a scientific advisory committee and a research grant from Genentech as a principal investigator.)
Originally released February 28, 1995; last updated January 16, 2017; expires January 16, 2020

This article includes discussion of fibromuscular dysplasia, angiodysplasia, arterial dysplasia, fibromuscular hyperplasia, FMD, and medial hyperplasia. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Fibromuscular dysplasia is a nonatherosclerotic vasculopathy affecting various circulatory beds, including the cerebral circulation. Cerebrovascular lesions may include arterial dissection and aneurysms. Neurologic manifestations typically result from ischemic events associated with these lesions. Management considerations are highlighted in this overview of cerebrovascular fibromuscular dysplasia.

Key points

 

• Although the histologic correlates of fibromuscular dysplasia have been delineated, diagnosis is typically established based on angiographic appearance.

 

• Associated intracranial aneurysms may occur with this disorder.

 

Stroke is a relatively rare complication of fibromuscular dysplasia.

Historical note and terminology

Fibromuscular dysplasia, also known as fibromuscular hyperplasia, medial hyperplasia, or arterial dysplasia, is a relatively uncommon multifocal arterial disease of unknown cause, characterized by nonatherosclerotic abnormalities involving the smooth muscle, fibrous and elastic tissue, of small- to medium-sized arterial walls (Slovut and Olin 2004). The first clinical and pathological description was by Leadbetter and Burkland in 1938 (Leadbetter and Burkland 1938). They reported a 5-year-old boy with renal hypertension who underwent a unilateral nephrectomy. The affected renal artery was noted to harbor "an intraarterial mass of smooth muscle." For many years it was assumed that fibromuscular dysplasia damaged only renal vessels. However, in 1964, Palubinskas and Ripley published a case of fibromuscular dysplasia involving the celiac artery in a 36-year-old woman; this was the first indication that the disease may be generalized (Palubinskas and Ripley 1964). Subsequent reports followed that described involvement of the visceral arteries, iliac artery, femoral artery, axillary artery, subclavian artery, coronary artery, aorta, and cephalic arteries (Hill and Antonius 1965; Osborn and Anderson 1977; Luscher et al 1987). Involvement of the internal carotid artery was radiologically and histologically proven in 1965 (Connett and Lansche 1965). Cephalic vessels are involved in 25% to 30% of reported cases of fibromuscular dysplasia (Begelman and Olin 2000). The cephalic vessels are the second most common location, after renal arteries. Fibromuscular dysplasia predominantly involves the cervical and distal internal carotid artery (the location of the disease in 90% of cases). However, similar vascular abnormalities are observed in the middle cerebral arteries, the anterior cerebral arteries, the posterior cerebral arteries, the vertebral arteries, and the basilar artery (Frens et al 1974; Osborn and Anderson 1977; Hegedus and Nemeth 1984; Vles et al 1990; Arunodaya et al 1997; Demirkaya et al 2001; Hatayama et al 2001; van de Nes et al 2007; Ribeiro et al 2009). Moyamoya syndrome has also been described in association with fibromuscular dysplasia (Ou et al 2006). Vertebral artery involvement is less common and typically coexists with carotid involvement. Although cephalic fibromuscular dysplasia is often an incidental finding on autopsy or angiography, it could be complicated by cerebral infarction secondary to thromboemboli arising from irregularities of the arterial wall, spontaneous arterial dissection, formation of a carotid artery cavernous sinus fistula, or development of an aneurysm (Hill and Antonius 1965; Osborn and Anderson 1977; So et al 1981; Bellot et al 1985). Sethi and colleagues have described a characteristic “S-curve” in the mid-distal carotid that is suggestive of the underlying disorder (Sethi et al 2014; Haussen et al 2016). Multiple vessels may be involved with dissecting pathology in such cases (Hassan et al 2013). Subarachnoid hemorrhage may result from saccular aneurysmal rupture or extension of a dissecting aneurysm.

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