Chapter 90

Upper Extremity Arterial Disease

Heron E. Rodriguez

Key Points

1 Presentation includes arm claudication, distal embolism, finger necrosis, and vasomotor changes

described as Raynaud phenomenon.

2 Given the multiple potential etiologies of upper extremity ischemia, a thorough occupational and

social history is of crucial importance.

3 Unilateral hand ischemia points toward an isolated anatomic lesion whereas bilateral hand ischemia

is more often related to systemic diseases.

4 A variety of noninvasive tests are available for the initial investigation of arm ischemia and detailed,

bilateral arterial imaging is mandatory for treatment planning.

5 Although endovascular revascularization is an excellent option for many causes of upper extremity

ischemia, open surgical revascularization should be used for some of the diseases causing arm

ischemia (i.e., thoracic outlet syndrome, hypothenar hammer syndrome, etc.)

INTRODUCTION

1 Compared to arterial occlusive lesions in the lower extremity, occlusive disease of the arteries of the

upper extremity is often better tolerated and severe symptoms of ischemia are less frequent in the arms

than in the legs. This not only can be attributed to the presence of an extensive collateral network, but

also to the very different physiologic requirements of the upper extremities when compared to the legs.

Another striking difference between arterial disease of the arms compared to that of the legs resides in

its etiology. Whereas in the lower extremities, atherosclerosis is responsible for the overwhelming

majority of cases of critical limb ischemia, in addition to atherosclerosis, a variety of anatomic,

occupational, and iatrogenic etiologies are responsible for occlusive disease of the arms.

PRESENTATION

Patients suffering from arterial insufficiency affecting the upper extremities can present with a variety

of symptoms. Arm claudication (pain during exercise) is rare and often points to a lesion in the proximal

vasculature (innominate, subclavian, or axillary arteries). More commonly, patients present with skin

changes affecting the hand or fingers. Splinter hemorrhages of the bed of the nail, livedo reticularis,

cyanosis of segments of the fingers, and gangrene are signs of embolism or ischemia affecting the

arteries of the hand (Fig. 90-1).1 The term Raynaud phenomenon indicates an episodic series of skin

changes affecting the fingers or the hand that are triggered by changes in temperature and sometimes

even emotions. The classic description includes the sequential appearance of pallor, then cyanosis, then

erythema. These correspond to initial vasospasm (causing pallor), desaturation of hemoglobin (causing

cyanosis), and finally compensatory vasodilation, also known as reactive hyperemia (causing erythema).

Raynaud phenomenon (also called secondary Raynaud syndrome) can be the manifestation of occlusive

disease or, more commonly, associated with autoimmune conditions (scleroderma, Sjögren syndrome,

rheumatoid arthritis, lupus).1 In contrast, the term Raynaud disease (or primary Raynaud syndrome) is

used to describe a condition of unknown etiology that occurs in patients without occlusive lesions or

autoimmune disease and who experience vasomotor symptoms in both arms, and sometimes even the

legs, for longer than 2 years.2

ETIOLOGY

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2 A myriad of conditions can cause upper extremity occlusive disease (Table 90-1). Atherosclerosis

typically affects the origins of the supra-aortic trunks. Ostial lesions at the origin of the subclavian

artery are most common. The innominate artery and distal arteries can also be affected. Involvement of

multiple segments of the upper extremity circulation is possible and is often asymptomatic even in the

presence of total occlusion. Patients with renal failure typically have normal proximal vessels with

severe disease in the blood vessels of the forearm and hand (Fig. 90-2). In this patient population, hand

ischemia tends to occur at a younger age and is frequently associated with calciphylaxis.3

Inflammatory conditions like Takayasu arteritis, giant cell arteritis or temporal arteritis can also

affect the proximal portion of the great vessels.4 Takayasu arteritis is a nonspecific inflammatory

disease that has a predilection for young women. This so-called “pulseless disease” typically occurs in

women in the second or third decades of life who present with symptoms of ischemia in the arms or the

cerebrovascular territory. An initial acute inflammatory phase of the disease leads to a late chronic

phase where long segments of stenosis appear in the vessels (Fig. 90-3). These lesions have a low

embolic potential but typically result in ischemic symptoms due to low flow. In contrast, giant cell

arteritis involves the more distal segments of the subclavian arteries and affects older people. The

typical pattern of disease is that of areas of normal luminal diameter alternated with dilated or stenotic

lesions. The most common symptoms are headaches and visual changes related to involvement of the

cerebral vascular arteries. Buerger disease is an obliterative disease that typically affects medium and

small caliber arteries. The sine qua non for Buerger disease is tobacco use in a young patient. It affects

the lower extremities more commonly than the upper limbs and occurs more frequently in males than

females.

Figure 90-1. Manifestations of upper extremity ischemia. Splinter hemorraghes in the nails arising from a proximal embolic source

(A). Gangrene affecting the tip of the third finger (B). Advanced, severe gangrene in the radial artery distribution secondary to

complications related to an arterial line (C).

Ergotamine-induced vasospasm can create ischemia of the hands and the feet. Historically, several

epidemics occurred in Europe during the Middle Ages caused by the ingestion of rye contaminated by

alkaloid-producing fungi. After a miraculous cure that was believed to have occurred thanks to the relics

of Saint Anthony the Great, the Hospital Brothers of St. Anthony’s order was founded and the term “St.

Anthony’s Fire” became popular. Today, most cases of ergotism are due to antimigraine drugs or

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recreational substance abuse.5

Connective tissue disorders are a common cause of upper extremity ischemia. Lesions typically affect

the distal vasculature and are more common in scleroderma, rheumatoid arthritis, CREST syndrome,

mixed connective tissue disorder, and lupus. Raynaud phenomenon is often the manifestation in early

stages whereas digital gangrene occurs in late stages of the disease.

Peet et al. first coined the term thoracic outlet syndrome (TOS) in 1956 to encompass a group of signs

and symptoms related to compression of the brachial plexus, subclavian artery, or subclavian vein as

they pass from the thorax into the axilla. Only in 1% of all cases of TOS, the artery, is the structure

compressed. Although TOS is often asymptomatic, patients may present with a range of signs and

symptoms, from fatigue or pain in the affected arm upon exertion and Raynaud phenomenon to critical

ischemia of the upper extremity because of emboli arising from the chronically injured artery or from a

mural thrombus in a poststenotic aneurysm sac. The artery is compressed as it passes through the thorax

into the axilla through the narrow gap formed by the clavicle superiorly, the first rib inferiorly and the

subclaveous muscle anteriorly and superiorly, as well as the scalene muscles posteriorly (Fig. 90-4).

Bony anomalies, such as cervical rib or an abnormal first thoracic rib, are sometimes present.

Aneurysms can also form because of repetitive trauma in branches of the subclavian artery. These

aneurysms can be the source of emboli that manifest as ischemic lesions in the hands.6 Pectoralis minor

syndrome is a distinct condition caused by compression of the subclavian artery by the pectoralis minor

as it travels cephalad to insert in the coracoid process (Fig. 90-4).

Ischemia caused by abnormalities in the axillary, brachial, radial, and ulnar arteries is often the result

of iatrogenic etiologies. One of the most common causes of ischemia seen in our practice is due to

hemodialysis access. Hand ischemia due to steal phenomenon is common and ischemia as a result of

intra-arterial catheters also occurs frequently.

Occupational injuries to the arteries of the hand result in thenar or hypothenar hammer syndrome.

The ulnar artery is vulnerable to injury at the hypothenar eminence, just distal to the wrist where the

ulnar artery passes through Guyon canal bound by the pisiform and hamate bones. Here, the ulnar

artery is cushioned only by skin, subcutaneous tissue, and the thin palmaris brevis muscle. Repetitive

use of the palm of the hand as a “hammer” compresses the unprotected ulnar artery against the nearby

hook of the hammate bone. Both aneurysm formation and occlusive disease can occur (Fig. 90-5). 7

HISTORY AND PHYSICAL EXAMINATION

3 Given the frequent association of upper extremity ischemia with systemic and occupational disorders,

a thorough medical and social history is mandatory. Interrogation should include inquiring about

tobacco and drug use. Antimigraine medications, HIV antivirals, and most commonly recreational drugs,

like cocaine and other alkaloids, can cause ergotism. Occupational history is of particular importance

when evaluating upper extremity vascular issues. TOS occurs in athletes who are required to perform

repetitive overhead shoulder motion (swimmers, baseball pitchers, weight lifters, etc.). Repetitive

trauma to the palmar eminences (thenar and hypothenar) is typically observed in manual laborers

(machinists, carpenters, and construction workers) but has also been described in athletes, musicians,

and even computer workers who use their hands repetitively causing injury to the palms.

ETIOLOGY

Table 90-1 Conditions and Risks for Upper Extremity Ischemia

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Figure 90-2. Selective left-hand angiogram in a patient with chronic renal insufficiency. There is severe, diffuse disease affecting a

heavily calcified ulnar artery (arrow). In addition, there is lack of contrast opacification in several of the digital arteries.

Figure 90-3. Aortogram in the right oblique projection of a patient with Takayasu arteritis. Long-segment right common carotid

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stenosis (small arrows) leading into a normal appearing carotid bifurcation. Segmental occlusion of the right subclavian artery

(block arrow) just distal to the origin of the right vertebral. There is distal reconstitution via a large collateral. There is also an

aneurysm (star) of a previous bypass in the left subclavian artery.

Figure 90-4. Compressive zones of the thoracic outlet. The subclavian structures can be compressed at the costoclavicular space,

the scalene triangle, or distally at the border of the pectoralis minor muscle.

The presence of atherosclerotic disease in the heart or other vascular beds should also be investigated.

History of connective tissue disorders or their symptoms (dysphagia, arthritis, telangiectasia, and other

skin abnormalities) cannot be obviated during the evaluation of upper extremity ischemia. Changes in

the appearance of the skin of the hands suggestive of Raynaud’s should be explicitly investigated.

Figure 90-5. Ulnar artery aneurysm resulting from repetitive trauma in a construction worker.

It is necessary to obtain clear documentation of any prior intervention in the arm (hemodialysis

access, biopsies, brachial or axillary access for cardiac catheterizations) or episodes of trauma

(clavicular or rib fractures, humeral, shoulder, or elbow injuries) (Fig. 90-6).

A complete physical examination needs to include a systemic evaluation looking for signs of

atherosclerotic, inflammatory, or autoimmune disease. A meticulous inspection, auscultation, and

palpation of both sides of the neck, periclavicular areas, shoulders, and the entire arms and hands is

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done in the resting, neutral position, and also while abducting the arm. A bruit or a thrill in the

periclavicular area would suggest the presence of stenosis or poststenotic dilation arising from TOS and

a prominent pulse should point to an aneurysm in the subclavian artery. Comparison between the two

upper extremities is of crucial importance since bilaterality often points to a systemic disorder. A careful

hand examination includes inspecting for splinter hemorrhages under the beds of the nails, skin

evidence of embolism or gangrene, changes in the color of the skin related to vasospasm and signs of

trauma in the hand. Careful palpation of the radial, ulnar, snuffbox, and even digital pulses is done.

Digital pressure is then applied to the ulnar and radial arteries in the wrist. After the patient opens and

closes his or her hand, pressure is removed from one of the wrist arteries and inspection of the color of

the hand and palpation of the distal pulses is repeated (Allen test). The test is normal if the entire hand

recovers its color and capillary refill. If a portion of the hand fails to normalize, suspicion arises that the

palmar vascular collateral circulation (palmar arch) is incomplete. Finally, blood pressure measurements

in both arms are obtained and compared (brachiobrachial index).

Figure 90-6. Old clavicular fracture treated with a metallic prosthesis. Notice the luminal abnormality in the subclavian artery as it

crosses the clavicle. This lesion was caused by periosteal scarring and caused distal embolism in the hand.

LABORATORY AND NONINVASIVE TESTING

4 A series of serologic tests is typically obtained when autoimmune disease is suspected (Table 90-2). A

variety of tests are available from the blood flow laboratory to aid in the management of upper

extremity occlusive disease.8 All patients should be subjected to segmental arterial pressure

measurement and waveform analysis, at the minimum. In the upper extremities, this test usually

includes photoplethysmography (PPG) of the digital vessels. The absence of triphasic flow and the

presence of “blunted” PPG tracing in the affected arteries and digits, respectively, helps to localize

obstructive lesions. If intermittent occlusion is suspected due to TOS, noninvasive testing can be

performed along with the use of provocative maneuvers (Fig. 90-7). In patients with symptoms

suggestive of vasomotor disorders (cold intolerance, color skin changes, etc.) the cold challenge test is

used. In our lab, we immerse the hands in iced water and measure the digital temperatures every 5

minutes. A normal response should normalize the digital temperature within 1°C of preimmersion

measurements. Patients with cold intolerance fail to restore digital temperature in 20 minutes. Several

variations of this test exist.9

DIAGNOSIS

Table 90-2 Lab Tests for Systemic Causes for Hand Ischemia

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Figure 90-7. Noninvasive blood flow study in a major league baseball pitcher showing loss of normal signals during proactive

maneuvers.

Figure 90-8. Focused magnetic resonance angiogram of the hands.

A more sophisticated version of the Adson test can also be obtained in most blood flow facilities by

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