[Fibromuscular dysplasia of the brachial artery, an unusual cause of upper extremities ischemia. A case report and review of the literature]

Abstract

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic sistemic disease of unknown etiology, primarily affecting muscular arteries of intermediate size. It has been most commonly observed in the renal, carotid, and intracerebral arteries, although it has been reported in other arterial beds. However, being an uncommon disease in general, the manifestation of FMD in the upper extremities is exceedingly rare. The authors report the case of a 69 years old female admitted with ischemia of the right hand, secondary to fibromuscular dysplasia of the midbrachial artery. The patient presented to vascular surgery clinic with a 4 month history of numbness, pain, and coolness of her right hand, with a small necrotic lesion on her right index finger. Peripheral pulses were barely palpable, and doppler-derived brachial and radial systolic pressures suggested midbrachial artery stenosis. Arteriography showed a normal arch and normal innominate, subclavian, and axillary arteries. The midbrachial artery was markedly abnormal and had alternating areas of stenosis and aneurysm formation - "string-of-beads" appearance. The patient underwent surgical excision of the abnormal right brachial artery, and reconstruction was accomplished with a reversed saphenous vein graft. Distal pulses were restored postoperatively. Pathologic examination confirmed the diagnosis of fibromuscular dysplasia. A review of the literature on the topic was made.