Variable Clinical Spectrum of Fibromuscular Dysplasia of the by MikeJenny


									                                                                               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)
 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
 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
                                                                                     (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
                                                                                     cm/s. Note the beaded appearance of both brachial arteries.

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