The Correct answer is E
Fibromuscular dysplasia (FMD) typically occurs in young persons, particularly women <35 years of age. The abrupt onset of hypertension in a young patient with a lateralizing abdominal bruit suggests the diagnosis. For patients with suspected renal artery fibromuscular dysplasia, CT angiography (CTA) is the initial imaging modality of choice. CT angiography is a noninvasive imaging method that has high accuracy in confirming the diagnosis of fibromuscular dysplasia, which most commonly affects the renal and carotid arteries. Renal artery stenosis due to FMD leads to hypertension, as seen here. Imaging typically shows a so-called “string of beads” appearance of the renal arteries due to segmental hyperplasia of the tunica media. Other imaging methods that can be used to diagnose FMD are duplex ultrasonography and magnetic resonance angiography.
Patients with fibromuscular dysplasia are at risk for aneurysm formation and/or dissection, most commonly of the renal, carotid, and intracranial arteries. Patients with fibromuscular dysplasia should undergo at least a one-time assessment for intracranial aneurysm with brain CTA or MRA.
⚠Choice A is not correct:
Echocardiography with Doppler is used e.g., to confirm coarctation of the aorta. Although coarctation of the aorta can lead to hypertension, other symptoms are also typically present, such as hypotension and claudication of the lower extremities, or a continuous murmur below the left clavicula and between the shoulder blades. An abdominal bruit, however, would not be expected. Echocardiography has no role in diagnosing fibromuscular dysplasia.
⚠Choice B is not correct:
Serum PTH levels are used in the diagnostic evaluation of hyperparathyroidism. Primary hyperparathyroidism can lead to hypertension due to progressive calcification of the arteries and heart muscle. However, this condition is typically associated with other findings, such as nephrolithiasis, constipation, gastric ulcers, psychological symptoms (e.g., depression, fatigue), and/or bone, muscle, and joint pain. In addition, primary hyperparathyroidism would not explain this patient's abdominal bruit.
⚠Choice C is not correct:
A high-dose dexamethasone suppression test is used to confirm Cushing syndrome. While Cushing syndrome is a common cause of secondary hypertension, it is usually accompanied by other symptoms, such as obesity, hirsutism, and easily bruisable skin with stretch marks. In addition, Cushing syndrome would not explain this patient's abdominal bruit.
⚠Choice D is not correct:
Oral sodium loading test is used to confirm primary hyperaldosteronism. Although primary hyperaldosteronism is a common cause of secondary hypertension in young people, symptoms of hypokalemia, such as fatigue or muscle weakness, are also typically present. In addition, primary hyperaldosteronism would not explain this patient's abdominal bruit.
Summarized Points:
In a young woman (<35 years of age) with a hypertensive emergency (blood pressure of 185/125 mm Hg, papilledema, severe headache) and an epigastric or flank bruit on examination, fibromuscular dysplasia (FMD) is the most likely diagnosis. The best initial test for renal FMD is duplex ultrasonography and/or CT angiography. The common finding is “string of beads” sign which is from segmental hyperplasia of the media and beaded stenosis.