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Abstract
A 38-year-old female presented to us with a history of fluctuating blood pressure.
On physical examination, her blood pressure in the right arm was 180/110 mmHg. Physical
examination revealed no other abnormalities. Her laboratory parameters were within
normal limits. Renal artery duplex ultrasound was suggestive of significant stenosis
in the left renal artery.
Subsequent renal angiography demonstrated a classical "string of beads" appearance
of both the renal arteries (saccular dilatations and constrictions) that spared the
ostium; this was suggestive of fibromuscular dysplasia. The left renal artery also
exhibited short-segment web-like stenosis (70% to 80%) in the mid-segment with a significant
gradient across it (Fig. 1). Angioplasty of the stenotic segment was performed using
an angioplasty balloon at a pressure of 10 atmospheres with good results (Fig. 2).
After angioplasty, the patient's blood pressure normalized and she was discharged
without any antihypertensive medications. At the 6-month follow-up, her blood pressure
remained normal and she required no antihypertensive medications.
Fibromuscular dysplasia is a noninflammatory, nonatherosclerotic vascular disease
that commonly involves the renal and internal carotid arteries; however, it can involve
any arterial bed. Renal fibromuscular dysplasia usually affects females from 15 to
50 years of age and accounts for around 10% of cases of renal artery stenosis. Renal
fibromuscular dysplasia is classified based on the predominant arterial layer involved:
intima, media, or adventitia. Involvement of the media is further subdivided into
medial, perimedial, and hyperplastic medial fibromuscular dysplasia. The three most
common and classically described subtypes include medial (70% of cases), perimedial
(15% to 25%), and intimal fibrodysplasias (1% to 2%). The classical angiographic "string
of beads" appearance is seen in medial and perimedial fibromuscular dysplasias. It
is characterized by alternating stenoses and aneurysms and frequently involves the
bilateral renal arteries.
The most accurate method for diagnosis of fibromuscular dysplasia remains catheter-based
angiography.
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