Fibromuscular dysplasia of the brachial artery: A case report and review of the literature

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1 CASE REPORTS Fibromuscular dysplasia of the brachial artery: A case report and review of the literature William W. Lin, BA, Gregory S. McGee, MD, Bruce K. Patterson, MD, James S. T. Yao, MD, PhD, and William H. Pearce, MD, Chicago, Ill. Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily medium-sized and small arteries. Fibromuscular dysplasia is characterized by medial fbrosis with or without smooth muscle cell hyperplasia and may produce luminal impingement with severe turbulence. Secondary aneurysmal deformity with or without thrombosis may also contribute to the obstruction. Fibromuscular dysplasia most commonly involves the renal and carotid arteries, with upper-extremity disease rarely reported. This case report describes a patient with digital embolization from brachial artery fibromuscular dysplasia. Angiography demonstrated significant narrowing and irregularity with a characteristic "string-of-beads" appearance of the right midbrachial artery. The abnormal segment was resected and reconstructed with a reversed saphenous vein graft. Histologic studies revealed disruption of the internal elastic lamina and disorientation of the hyperplastic medial smooth muscle cells characteristic of fibromuscular dysplasia. (J VASC SURG 1992;16:66-70.) Upper extremity ischemia is an uncommon clinical entity representing less than 4% of all vascular surgical procedures.* Unlike the lower extremity, a great diversity of diseases may produce ischemic symptoms in the upper extremity, including fibromuscular dysplasia (FMD). Although FMD usually affects the renal and carotid arteries, other small and medium-sized arteries including the brachial artery may be involved. 2,s To date there are four reported cases of brachial artery FMD in the world literature. 46 The pertinent aspects of this fifth reported case of brachial artery FMD are reviewed, with emphasis placed on early detection with noninvasive examination and complete upper-extremity angiography in patients with unilateral ischemic symptoms. CASE REPORT A 74-year-old white woman was seen with pain, bluish discoloration, and a nonhealing ulcer of the distal right- From the Department of Surgery, Division of Vascular Surgery, and the Department of Pathology, Northwestern University Medical School, Chicago. Supported in part by the Alyce F. Salerno Foundation. Reprint requests: William H. Pearce, MD, 251 E. Chicago Ave., Suite 626, Chicago, IL /4/ third finger. In addition, the patient described increased cold sensitivity of the right hand with color changes consistent with Raynaud's phenomena. Her left hand was free of symptoms. She had no known medical illness and denied any history of trauma, arthalgias, malaise, fevers, or weight loss. She had smoked one pack of cigarrettes a day for 20 years. Her laboratory findings included a normal renal and hepatic profile, a normal platelet count and prothrombin/partial thromboplastin times, and a normal erythrocyte sedimentation rate. Neither anemia nor leukocytosis were present, and her antinuclear antibody test result was negative. The pertinent physical findings were limited to the upper extremities. No supraclavicular masses or bruits were observed, and the axillary, brachial, and radial pulses were strong and symmetric. Equal brachial artery pressures were present, and provocative arm maneuvers failed to alter the arterial examination. Cyanosis of the distal phalanx of the right third finger was present with tuft ulceration. Noninvasive examination with segmental pressures and waveforms revealed mild right ulnar artery disease with occlusive disease of right third digit, and cold stimulation testing was abnormal in the right hand. Duplex scan of the right subclavian, axillary, and brachial arteries revealed turbulence in the brachial artery with an irregularappearing intimal surface. No intraluminal thrombus was seen. Complete bilateral upper extremity arteriography revealed normal subclavian, axillary, ulnar, radial, and

2 Volume 16 Number 1 July 1992 Fibromuscular dysplasia of the brachial artery 67 interosseous arteries; however, in the midportion of the right brachial artery there was an irregular lesion with a "string-of-beads" appearance (Fig. 1). Magnified views of the right hand demonstrated occlusion of the proper digital arteries of the third finger. Treatment consisted of brachial artery resection and saphenous vein graft interposition. Gross examination of the resected specimen revealed an irregular intima with small areas of adherent thrombus. There were thickened fibromuscular ridges with intervening areas of vessel wall thinning distorting the vessel lumen. Histologically, there was circumferential intimal fibrosis with disruption of the internal elastic lamina (Fig. 2). In addition, there were abundant and disorganized medial smooth muscle cells lacking normal polarity (Fig. 3). The patient had an uneventful postoperative course and was discharged on the fourth postoperative day. DISCUSSION Fibromuscular dysplasia is a nonatherosclerotic and noninflammatory vascular disease of unknown cause that affects primarily medium-sized and small arteries. In general, FMD occurs in white women with onset of disease in the fourth and fifth decades. 7,8 After the first description of FMD by Leadbetter and Burkland 9 in 1938, FMD was thought to be confined to the renal and carotid arteries. However, subsequent reports have documented FMD in many other sites. In 1982 Metinger et al.10 reviewed the literature and classified 1100 cases of FMD by location. He found the renal arteries involved in 58% of cases, the carotid or vertebral arteries in 32%, the intracerebral vessels in 3.2%, and the iliofemoral arteries in 2.5% of cases. Fibromuscular dysplasia of the brachial artery was not reported. The first case of brachial artery FMD was reported by Kessler s later in 1982, and three cases have since been reported. 4,6 This report represents the fifth reported case of brachial artery FMD. As seen in Table I, there is only a slight female predominance in patients with brachial artery involvement (three women/two men), with a wide age distribution. Furthermore, it is also unusual for FMD to begin in the seventh decade. For these reasons the preoperative diagnosis was a long segmental ulcerative atherosclerotic plaque. Based on the predominant site of dysplasia in the arterial wall (intima, media, or adventitia), a pathologic classification of FMD was proposed by Harrison and McCormack ~1 in 1971 and modified by Stanley et al.12 in Three predominant types of FMD were identified: intimal fibroplasia (hyperplasia), medial FMD, and periadventitial fibroplasia. Medial FMD accounts for 70% to 95% of all fibromuscular vascular lesions and is divided into Fig. 1. Angiogram of the right midbrachial artery shows the string-of-beads appearance characteristic of FMD. three subtypes: medial fibroplasia, perimedial fibroplasia, and medial hyperplasia. Medial fibroplasia is known by its angiographic string-of-beads appearance, produced by alternating stenoses and aneurysreal dilatations. Perimedial fibroplasia also gives rise to the string-of-beads appearance yet with fewer and smaller aneurysms than in medial fibroplastic lesions. Medial hyperplasia results in smooth tubular stenoses without aneurysm formation. Intimal fibroplasia represents only 2% to 5% of cases of FMD and is angiographically identical to medial hyperplasia. Periadventitial fibroplasia is the least common type, constituting less than 2% of all cases of FMD. Histologically, all medial fibromuscular lesions are characterized by increases in either the medial cellular content or the medial fibrous connective tissues. Medial fibroplasia, the most common subtype, results from medial fibrosis combined with a loss of medial smooth muscle cells. Intimal fibroplastic lesions are described as having circumferential intimal fibrosis, a clearly identifiable internal elastic lamina, and normal medial and adventitial architecture. Of the five reported cases of brachial artery FMD, three were intimal fibroplasia. Periadventitial fibroplasia, the rarest type of FMD, is characterized by adventitial fibroplasia with collagen surrounding

3 68 Lin et al. Journal of VASCULAR SURGERY Fig. 2. Microscopic section of the affected arterial segment shows a loss of elastic fiber and disruption of the internal elastic lamina. (Elastin stain. Original magnification 100.)!i!!i!i~!!!i~i!!!!~i~!!i?: ~ i/~ f~i ~ ~!~! i~ Fig. 3. Microscopic section of the affected arterial segment shows muscle fiber disarray in the media. (Hematoxylin and eosin stain. Original magnification 40.) the adventitia and extending into the adjacent tissues. In the case reported the fibromuscular lesion appeared to have both intimal and medial components, including intimal fbrosis with disruption of the internal elastic membrane (Fig. 2), as well as medial fibroplasia with fibromuscular ridges and smooth muscle cell disarray (Figs. 3 and 4). The cause of FMD is unknown, and several hypotheses have been proposed. These include (1) a traumatic cause xs suggesting that recurrent microtrauma initiates excess production of collagen by smooth muscle cells, (2) a hormonal hypothesis ~4 explaining FMD's predominantly female distribution (smooth muscle cells increase production of collagen after exposure to estrogen), (3) a genetic hypothesis is supporting FMD's inheritance as an autosomaldominant trait, and finally (4) an ischemic vascular wall hypothesis 16 proposing that functional occlusion of the vasa vasorum induces excess production of collagen by the smooth muscle cells. It is most likely that FMD is a disease whose cause encompasses several of the hypotheses.

4 Volume 16 Number 1 July 1992 Fibromuscular dy~olasia of the brachial artery 69 Fig. 4. Microscopic section of the diseased arterial segment shows alternating thick and thin regions of the affected arterial wall. (Hematoxylin and eosin stain. Original magnification x 100.) Table I. Reported cases of brachial FMD Age (yr) Gender FMD type Symptom Tream~m 44 Female Intimal Digital pain Oral corticosteroid 42 Female Medial Hand petechiae Resection 22 Male Intimal Gangrenous fingers None 37 Male Intimal Gangrenous fingers None 74 Female Intimal/medial Digital cyanosis Resection The natural history of brachial artery FMD is difficult to discern from only five cases but appears to be related to the development of turbulent flow, with thrombus formation and distal embolization. As an initial test, duplex scanning is useful to detect a proximal source of emboli in patients whose upper extremity segmental pressures and waveforms suggest distal occlusion. Resection of the lesion before irreversible distal vessel occlusion occurs is mandatory to prevent limb-threatening ischemia and tissue loss. Although balloon angioplasty is widely used for the treatment of FMD of the renal arteries, the role of balloon angioplasty in lesions with an embolic potential is uncertain. Several studies have reported successful balloon angioplasty of FMD of the carotid artery and peripheral atherosclerotic lesions associated with distal emboli, lr,ls In one instance a transient ischemic attack occurred during balloon angioplasty of an FMD lesion of the internal carotid artery.19 These early results should not be generalized until further experience is gained. In conclusion, brachial artery FMD is a rare, nonsystemic disease resulting in a local flow disturbance. Recurrent distal embolization from the irregular luminal surface is the dominant clinical feature. Management by early detection with Duplex scanning combined with angiographic mapping of the distal runoff provides a margin of safety for successful resection and revascularization. REFERENCES 1. McCarthy WJ, Flinn WR, Yao JST, et al. Results of bypass grafting for upper limb ischemia. J VASC SURG 1986;3: Garrett HE, Hodosh S, DeBakey ME. Fibromuscular hyperplasia of the left axiuary artery. Arch Surg 1967;94: Wylie EJ, Binldey FM, Palubinskas AJ. Extrarenal fibromuscular hyperplasia. Am J Surg 1966;112: Olson LA, Faber DB, Lemar JV, Routman BN, Hoff GL. Fibromuscular hyperplasia of the brachial artery: failure of calcium antagonist therapy. Angiology 1984;35: Kessler M. Fibromnskulare Dysplasie der A brachialis. Radiologe 1982;22: Esfahani F, Rooholamini SA, Azadeh B,.~)aneshbod K. Arterial fibrodysplasia: a regional cause of peripheral occlusive vascular disease. Angiology 1989;40: Luscher TF, Keller HM, Imhof HG, et al. Fibromuscular hyperplasia: extension of the disease and therapeutic outcome:

5 70 Lin et al. Journal of VASCULAR SURGERY results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia. Nephron 1986;44(suppl): Luscher TF, Lie JT, Stanson AW, Houser OW, Hollier LH, Sheps SG. Arterial fibromuscular dysplasia. Mayo Clin Proc 1987;62: Leadbetter WF, Burkland CE. Hypertension in unilateral renal disease. J Urol 1938;39: Mettinger KL. Fibromuscular dysplasia and the brain II: current concepts of the disease. Stroke 1982;13: Harrison EG Jr, McCormack LJ. Pathologic classification of renal artery disease in renovascular hypertension. Mayo Clin Proc 1971;46: Stanley JC, Gewertz BL, Bove EL, Sottiurai V, Fry WJ. Arterial fibrodysplasia: histopathologic character and current etiologic concepts. Arch Surg 1975;110: Leung DYM, Glagov S, Mathews MG. Cyclic stretching stimulates synthesis of matrix components by arterial smooth muscle cells in vitro. Science 1976;191: Ross R, Klebanoff SJ. The smooth muscle cell I: in vivo synthesis of connective tissue proteins. J Cell Biol 1971;50: Rushton AR. The genetics of fibromuscular dysplasia. Arch Intern Med I980;140: Wissler RW. The arterial medial cell, smooth muscle, or multifunctional mesenchyme. Circulation 1967;36: Kumpe DA, Zwerdlinger S, Griffin DJ. Blue digit syndrome: treatment with percutaneous transluminal angioplasty. Radiology 1988;166: Tsai FY, Matovich V, Hieshima G, et al. Percutaneous transluminal angioplasty of the carotid artery. Am I Neuroradiol 1986;7: Smith DC, Smith LL, Hasso N. Fibromuscular dysplasia of the internal carotid artery treated by operative balloon angioplasty. Radiology 1985;155: Submitted June 14, 1991; accepted Sept. 9, 1991.