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Variable Clinical Spectrum of Fibromuscular Dysplasia of the Brachial Cleveland, Ohio Arteries. Ana Casanegra, MD1, Vikram Kashyap, MD2, Sandra Yesenko, BA, RVT1, Carmela Tan, MD3, Heather L. Gornik, MD, MHS1 1: Vascular Medicine Section, Department of Cardiovascular Medicine, 2: Department of Vascular Surgery, 3: Department of Anatomic Pathology. Cleveland Clinic, Cleveland, Ohio. Abstract Case 1 Case 2 Findings 62 year-old female 63 year-old female Figure 4: Brachial Background: Fibromuscular dysplasia (FMD) is an uncommon artery surgical vascular disorder most frequently manifest in the renal and carotid • Referred to FMD clinic for a second opinion. • Developed acute pain and paleness in her left arm from the elbow to pathology. arteries. Involvement of the upper extremity arteries has been • FMD was diagnosed 15 years before with a carotid ultrasound and the hand. Hematoxylin & Eosin reportedly rarely in the medical literature and is usually unilateral. We (Panel A) and Movat’s subsequent angiogram as workup for pulsatile tinnitus. • Patient was anticoagulated and transferred to our institution. stain (panel B) with identified two patients in a single center with bilateral brachial FMD. • She had known FMD involvement of internal carotid and renal elastic fibers in black. Case 1: 62 year-old woman with pulsatile tinnitus due to FMD of • Cardioembolic sources were ruled out, as well as hypercoagulable Arrowheads mark the bilateral internal carotid arteries. She also had renal artery FMD with arteries bilaterally. states. external elastic well-controlled hypertension on two agents. She was found to have a • HTN controlled with two antihypertensive medications. lamina. There is • Upper extremity angiogram demonstrated bilateral beaded diminished left brachial pulse with associated bruit. Duplex ultrasound marked fibrosis of the • No neurological symptoms. No upper extremity symptoms appearance of the brachial arteries, occlusion of the left brachial medial layer of the arms demonstrated turbulent flow with a beaded appearance and artery with distal reconstitution through collaterals (Figure 3 and 4). consistent with medial velocity shifts in bilateral brachial arteries. She had no upper extremity • On exam she had bilateral cervical bruits, diminished left brachial fibroplasia. symptoms. pulse and a bruit over the brachial artery. The rest of the vascular • As she continued to have rest pain and pre ulcerative lesions in the A B Case 2: 63 year-old female with left upper extremity ischemia, exam was unremarkable. fingers she underwent a left brachial- radial bypass with good clinical presented with pain from the elbow to the thumb and digital pallor. results. Surgical pathology confirmed the diagnosis (Figure 5). • A duplex of the upper extremities showed beaded appearance and Workup for cardiac source of emboli was negative. Arteriography revealed findings of FMD in bilateral brachial arteries and occlusion of velocity shifts in both brachial arteries (Fig 1,2) • Renal and carotid arteries had no evidence of FMD. She has a small basilar artery aneurysm (incidental finding) Discussion the left brachial artery with partial collateral reconstitution. She had no evidence of FMD in the renal or carotid arteries. CTA identified a small • The brachial arteries are uncommonly affected by FMD, with 19 basilar artery aneurysm. She was anticoagulated and underwent left brachial to radial artery bypass grafting for arm claudication, rest pain Findings Findings cases reported in the English literature. Twelve (63%) with bilateral involvement3. and paresthesias of the hand with good initial results. Histopathology was consistent with FMD. • Clinical presentations include asymptomatic incidental finding, Conclusion: Though uncommon, FMD may involve the brachial A B digital embolism, Raynaud’s phenomenon, paresthesias and dialysis arteries, generally in association with disease in other vascular beds. fistula dysfunction4,5. The presentation of brachial FMD is variable and can range from no symptoms to an ischemic limb. The evaluation of the patient with FMD • Some of the patients had other vascular beds affected by FMD at should include query for arm or hand symptoms and vascular the time of presentation. examination of the upper extremity • Treatment has been reported with antiplatelet agents, and arterial angioplasty or reconstruction in symptomatic patients4. A B Figure 1: Color power angiography image of the right (Panel A) and left Conclusion (Panel B) Brachial arteries. Note the beaded appearance of these vessels. • Though uncommon, FMD may involve the brachial arteries, with or without associated disease in other vascular beds. Introduction A B Figure 3: Arteriography of brachial arteries right (Panel A) and left • The presentation of brachial FMD is variable and can range from no (Panel B) with “string of beads” symptoms to an ischemic limb. • The evaluation of the patient with FMD should include query for arm • FMD is a non-inflammatory non-atherosclerotic disease that affects or hand symptoms and a thorough vascular examination of the small and medium size arteries1. upper extremity • Woman in their 40s are primarily affected. • Renal and carotid arteries are the most commonly involved vascular beds 2. References • Other vascular beds can be affected although less frequently 2. • They are few case reports of FMD involving the brachial arteries3. 1. Olin Curr Opin Cardiol. 2008:527. 2. Mettinger et al. Stroke 1982:53. 3. Kolluri et al. Angiology 2004:685. 4. Dorman et al Cardiovasc Figure 2: Pulsed-wave Doppler of the brachial arteries. Panel A: Right Intervent Radiol 1994: 95. 5. Margoles et al J Vasc Interv Radiol Figure 4: Brachial artery occlusion, with distal reconstitution through 2009:1087 Brachial artery, PSV 144 cm/s. Panel B: Left Brachial artery, PSV 105 collaterals. cm/s. Note the beaded appearance of both brachial arteries.
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