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Rutherford’s 143: Fibromuscular Dysplasia

Rutherford’s 143: Fibromuscular Dysplasia

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Drawing from Rutherford’s Vascular Surgery textbook, Fibromuscular Dysplasia (FMD) is a non-atherosclerotic, non-inflammatory condition causing arterial wall thickening due to cellular overgrowth. Its cause is unknown (idiopathic). Thought to be rarer initially, evidence suggests it might be more common than previously believed, affecting women over 90% of the time, typically aged 20-60. While it can affect almost any artery, it commonly targets medium-sized vessels, notably the renal arteries (leading to renovascular hypertension, especially in young people) and extracranial carotid and vertebral arteries (linked to headaches, pulsatile tinnitus, TIAs, and strokes). A genetic component is suggested, with a familial tendency in up to 10% of cases, often showing autosomal dominant inheritance with incomplete penetrance. Hormonal influences and smoking are also implicated.

FMD is primarily classified by angiography into multifocal (“string of beads,” most common) and focal (single narrowing) types. Diagnosis involves clinical suspicion, ruling out conditions like vasculitis (FMD lacks typical inflammatory markers), and imaging. While Doppler ultrasound, CTA, and MRA are used, invasive angiography remains the gold standard for precise mapping, especially for complex lesions or branches, and allows pressure gradient measurement.

Treatment depends on the affected artery and symptom severity. Medical management includes standard anti-hypertensives and often antiplatelet agents like aspirin due to clot risk. For symptomatic renal artery stenosis, percutaneous transluminal renal angioplasty (PTRA) is often the first choice, yielding good blood pressure outcomes. Balloon angioplasty is usually sufficient; stents are typically avoided unless complications occur. Surgical revascularisation is reserved for complex cases, PTRA failures, or large aneurysms. For carotid FMD, asymptomatic patients are medically managed, while symptomatic cases often warrant intervention, typically angioplasty (sometimes with stenting or via open techniques like TCAR). FMD is associated with increased risk of aneurysms (including intracranial), which also require management.

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