Case: 231

Fibromuscular Dysplasia

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History: 62 yo male presents for "Renal Donor" evaluation.
Solution: The findings on this examination are: Renal cortical scarring bilaterally (L > R) and "beading" of the renal arteries (interestingly, R > L, although the degree of associated stenosis may be slightly worse on the left). This combination of findings is very suggestive of Fibromuscular Dysplasia (FMD). Note that if this is seen in the renal arteries, the patient has a significant likelihood of having carotid artery involvement (worse prognosis) and most people would recommend an evaluation of the carotid arteries for this patient. The majority of FMD affects the tunica media and has this appearance. Can also affect the intima and cause concentric, smooth narrowing, and other less common manifestations. Patients are often asymptomatic, but can present with renal hypertension or insufficiency (renal involvement), or headaches, bruits, or stroke (carotid/vertebral artery involvement). If asymptomatic, the patient may not be put on any medications other than potentially anticoagulation, but hypertension may need to be treated and if severe enough, angioplasty, or surgery may be needed.

CT Images

Questions

A reasonable next step would be:
  • Angioplasty
  • Corticosteroid therapy
  • Proceed to kidney donation
  • Evaluation of the carotid arteries
  • Chemotherapy
  • Evaluation of the lower extremity vasculature
This patient is likely to have a progressive course of disease with eventual renal failure.
False
What is the most likely diagnosis?
Fibromuscular dysplasia of the renal arteries.