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