LARGE VESSEL DISEASE OF THE UPPER EXTREMITY

Large vessel occlusive disease of the upper extremity encompasses several well-recognized disease entities. The most frequently encountered disease underlying upper extremity ischemia is atherosclerosis. Other entities one is likely to encounter are thoracic outlet syndrome, autoimmune vasculitis, thromboembolism, and vascular damage caused by trauma, sports injuries, and radiation therapy.

Patients with atherosclerotic occlusive disease typically present with upper extremity claudication or steal phenomenon. The most common locations for upper extremity large vessel involvement include the brachiocephalic and subclavian arteries. However, atherosclerosis can also cause small vessel obstruction by atheromatous embolization or thromboembolism. A particularly helpful clinical clue in establishing the correct cause is the age of the patient. Atherosclerotic disease of the upper extremity tends to affect older adult patients, whereas nonatherosclerotic upper extremity arterial occlusive disease affects younger patients.¹

The upper extremity is a frequent site for autoimmune vasculitis. Vasculitis is usually defined as a sterile inflammation of the vessel wall. In contrast to patients suffering from atherosclerosis, patients with vasculitis are often younger and have elevated erythrocyte sedimentation rates, antineutrophilic cytoplasmic antibodies (ANCA), and antiendothelial cell antibodies.² In addition to the local symptoms caused by vessel narrowing, many patients also suffer from concomitant constitutional symptoms such as fever, malaise, myalgia, and arthralgia. The upper extremity is most often affected by Takayasu disease and giant cell arteritis (GCA). These are histologically similar diseases but Takayasu disease tends to affect any of the aortic arch vessels as well as the arch itself, and sometimes the pulmonary and coronary arteries, whereas GCA tends to affect the subclavian arteries in a typical symmetrical pattern.³ A specific clinical constellation associated with GCA is polymyalgia rheumatica. In polymyalgia rheumatica, the vasculitis is located more distally in the cervicocranial arteries. Up to 50% of patients suffer from intermittent claudication of the jaw, tongue, or throat, and up to 33% of patients present with visual complaints such as amaurosis fugax or even blindness.⁴

The group of neurovascular disorders caused by extrinsic compression of the subclavian and axillary arteries and veins as well as the brachial plexus is called cervicoaxillary compression syndrome. The more popular name for this disorder is the thoracic outlet syndrome (TOS). Symptoms are caused by compression at the interscalene triangle, costoclavicular space, or retropectoralis minor space.⁵ Compression can be caused by a variety of causes such as a fibrous band, supernumerary cervical ribs, or excessive callus formation subsequent to a clavicular fracture. A large majority of cases (70% to 90%) is caused by involvement of the brachial plexus. In less than one third of cases, arteries and veins are involved.

Blunt and penetrating trauma to the chest and upper extremity may compromise the vascular supply as well. Other causes of injury to the upper extremity vessels are radiation therapy for malignancies of the breast, head, and neck and repetitive injury in athletes such as baseball pitchers, volleyball players, butterfly swimmers, and weight lifters.

Prevalence and Epidemiology

The incidence and prevalence of upper extremity atherosclerotic disease are not known. However, it is important to realize that the prevalence of atherosclerotic lesions in the upper extremity in absolute terms is much lower compared with the aortoiliac arteries and lower extremities. The reasons for this relative paucity of symptomatic atherosclerosis in upper extremity arteries remain unknown at present. Possible explanations include better collateral circulation, reduced muscle mass, and less vigorous use of the upper extremity compared with the lower extremity.

The estimated incidence of Takayasu arteritis is 2.6 cases per million in the United States, with higher incidences reported in young women and patients of Asian origin. Women in their second and third decades of life account for the vast majority of patients because they tend to be affected about eight to nine times more often than men. Conversely, GCA tends to affect older patients, peaking in the age group between 70 and 80 years. The estimated incidence of GCA is 2 to 20/million, with women being affected about two to four times more often than men.⁶

The incidence of thoracic outlet syndrome (TOS) is not well established. Although it is generally accepted that TOS is caused by compression of brachial plexus elements or subclavian vessels in their passage from the cervical area toward the axilla and proximal upper arm, there is much disagreement among clinicians regarding its diagnostic criteria and optimal treatment. Because there is no objective confirmatory test for TOS, the true prevalence of the disease remains elusive. Reported prevalence ranges from 3 to 80 cases/1000 population.⁷ TOS is mostly considered a diagnosis of exclusion.

It is estimated that vascular injuries to the upper extremity represent approximately 30% to 50% of all peripheral vascular injuries. Usually, the brachial artery is involved and most injuries are caused by penetrating trauma.⁸ Blunt injuries such as motor vehicle accidents account for 6% to 10% of upper extremity vascular trauma and are often associated with musculoskeletal injuries and neural injuries.⁹ The functional impact of the trauma is often related to concomitant injury to peripheral nerves. The extent of the vascular compromise following radiation therapy is directly proportional to the amount of radiation given, but symptoms may present only decades after therapy.

Cause and Pathophysiology

Atherosclerosis is considered to be a chronic inflammatory disease of the large arteries.¹⁰ The disease starts at an early age and remains clinically silent for decades. For a detailed discussion of the pathophysiology of atherosclerosis, see Chapters 51 and 88. It is important to note that imaging of the vascular lumen alone may underestimate the burden of disease related to atherosclerosis.

Takayasu arteritis is a classic vasculitis of unknown cause involving the upper extremity arteries. Infection, in particular tuberculosis, has been implicated in the pathogenesis of Takayasu arteritis, but a definitive link between the two diseases remains elusive at present.³^,¹¹ The disease can be divided into two stages, with an acute period of large vessel vasculitis, followed by fibrosis and scarring. In the acute stage, the adventitial vessels of the arterial walls become inflamed as a result of unknown causes. This leads to a generalized, smooth, circumferential thickening of the affected segment, including the media. In the chronic stage, elastic tissue is replaced by fibrosis, with thickening of all three layers of the vessel wall, leading to irreversible segmental smooth luminal narrowing. Other vasculitides that affect the upper extremities are thought to be of similar pathogenesis, although the clinical manifestations may vary (see earlier).

Cervicoaxillary compression syndrome can be the result of a heterogeneous set of activities or trauma, as noted. The exact clinical symptoms depend on which anatomic structures are involved. Local vascular injury is often typified by intimal damage, with subsequent aneurysm formation. This, in turn, may lead to mural thrombus and potentially distal embolization.

Manifestations of Disease

Clinical Presentation

The most common presentations of chronic large vessel upper extremity occlusive disease are arm claudication, or steal phenomena. Clinical clues are helpful to elucidate the underlying disease process and a thorough medical, surgical, occupational and sports history should be obtained in every patient. Symptoms of upper extremity occlusive disease that suggest nonatherosclerotic causes are young age, long-standing fatigue and malaise, high erythrocyte sedimentation rate, and vigorous occupational or sports activities involving repetitive strain to the shoulder and hand, such as frequent baseball pitching, mountain biking, and using the hand to pound structures (e.g., carpenters). Patients with vasculitis typically report having had vague complaints for months or even years prior to consulting a physician. A possible explanation for the relative rarity of arm claudication symptoms is the reduced muscle mass, less vigorous use, and abundance of numerous and well-developed collateral pathways compared with the lower extremity.¹²

Patients with steal syndromes present with upper extremity weakness, dizziness, and sometimes angina. The most well-known steal syndrome is subclavian steal, or reversal of antegrade flow in the vertebral artery caused by the presence of ipsilateral significant subclavian artery stenosis or occlusion, proximal to the origin of the vertebral artery. Another well-known steal phenomenon can be seen in the coronary artery circulation after coronary artery bypass grafting using the internal thoracic artery. In the presence of a subclavian artery stenosis, flow may reverse in the internal thoracic artery to supply the upper extremity arterial bed instead of augmenting flow in the coronary arteries. It is important to realize that an angiographic steal phenomenon does not necessarily imply symptoms. In fact, only about one third of all patients with angiographically proven steal syndromes suffer from characteristic complaints.¹³^–¹⁵

Acute injuries to the upper extremity often involve vascular structures and should be managed according to the presumed cause. In many cases, conventional angiography is the diagnostic modality of first choice because it allows for immediate intervention. This is also the case with suspected arterial embolism, for which catheter-based therapies can be used to perform thrombosuction and delivery of clot-lysing agents.

Imaging Indications and Algorithm

The noninvasive nature of Doppler ultrasonography (DUS), magnetic resonance imaging (MRI), and computed tomography (CT) has lowered the threshold for ordering imaging tests in patients with suspected upper extremity vascular involvement. Apart from the arguments in favor of safety and patient comfort, there is another major reason to use modern cross-sectional imaging techniques. DUS, CT, and MRI not only enable visualization of the arterial lumen but also of the arterial wall and surrounding bony and soft tissue structures. Because of this capability, DUS, MRI, and CT can identify lesions amenable to percutaneous transluminal angioplasty (PTA) and surgery, and can inform the surgeon about the best surgical approach.

When a patient presents with clinical symptoms suggestive of vascular involvement in the arteries of the upper arm, forearm, or hand, it is important to evaluate the entire upper extremity vascular tree from the aortic root to the digital arteries so as not to miss relevant lesions in the vascular tree. Suspected peripheral arterial or venous thrombosis is often evaluated with ultrasonography as the first-line imaging modality because it is a rapid, reliable, and less expensive imaging test, with high sensitivity and specificity. In cases of suspected arterial or venous lesions in the chest itself or in the parts of vessels that pass underneath the clavicle, CT and MRI are preferred, because the visualization of vascular structures in these locations is often poor with DUS. The choice of whether to use CT or MRI largely depends on local skill and preference. Both imaging modalities are capable of high-fidelity depiction of the vascular lumen and wall, as well as of the surrounding structures.

For diagnostic purposes, intra-arterial digital subtraction angiography (IA-DSA) has been relegated to a secondary role because the risks associated with the procedure outweigh the benefits for most patients. An exception is the evaluation of patients with suspected arterial thrombosis or embolization. These patients are vascular emergencies that should be treated without unnecessary delay. IA-DSA is the modality of first choice for this condition because it allows for an unequivocal diagnosis and concomitant treatment, if needed.

MEDIUM AND SMALL VESSEL DISEASE OF THE UPPER EXTREMITY

Atherosclerotic lesions in the forearm and hand arteries are rarely the cause of ischemic symptoms. Other diseases predominate in the list of differential diagnoses when distal upper extremity vascular disease is suspected.

The prototypical disease affecting the medium-sized arteries (and veins) of the upper limb is thromboangiitis obliterans, or Buerger disease (or Winiwarter-Buerger’s disease). Although the lower extremity is involved far more often, Buerger disease may affect the radial and ulnar arteries and palmar arch. There are numerous other diseases that affect the distal forearm, hand, and digital arteries of the upper extremity. A full review of all conditions associated with distal upper extremity arterial disease is beyond the scope of this chapter but can be found in the excellent review by Greenfield and colleagues.¹⁶ In many patients, arterial disease of the distal forearm and hand is not isolated but is associated with underlying systemic connective tissue disease such as scleroderma and CREST syndrome (calcinosis cutis, Raynaud syndrome, esophageal motility disorder, sclerodactyly, and telangiectasia) or rheumatologic disorders such as rheumatoid arthritis, mixed connective tissue disease, systemic lupus erythematosus, antiphospholipid syndrome, polymyositis, and dermatomyositis.¹⁷^–¹⁹

Occupational, recreational, and iatrogenic trauma are important sources of distal upper extremity ischemic symptoms. Repetitive pounding with the ulnar side of the hand or fist may result in aneurysm formation and digital artery occlusion. This condition is known as hypothenar hammer syndrome. A similar complex of symptoms can sometimes be encountered in patients with a history of long-standing pneumatic tool use or in mountain bikers, baseball catchers, volleyball players, or practitioners of karate. Arterial occlusion may also be a consequence of traumatic catheterization of the radial or ulnar arteries.

Raynaud phenomenon is a common ancillary finding in patients suffering from upper extremity vascular disease and may be the presenting symptom. It is defined as a reversible spasm of the small and medium-sized arteries, resulting in a characteristic triphasic white-blue-red color response. First, cessation of digital artery flow produces well-demarcated finger pallor. This is followed by vasorelaxation and return of arterial flow and subsequent postcapillary venule constriction, resulting in desaturated blood and producing cyanosis. Finally, postischemic hyperemia replaces cyanosis with rubor.^16,^20,²¹ A distinction is made between primary Raynaud phenomenon (formerly Raynaud disease), if there is no underlying illness, and secondary Raynaud phenomenon (formerly Raynaud syndrome) if there is an associated disorder detected on assessment. In approximately one third of cases, Raynaud phenomenon is an isolated and benign condition not associated with underlying disease. In cases of inflammatory arteritis, Raynaud phenomenon is common.

Prevalence and Epidemiology

Atherosclerotic occlusive disease of the medium and small arteries of the upper extremity is uncommon and comprises a minority of patients presenting with symptoms of forearm and hand ischemia. The exact prevalence is unknown, because many patients may never come to medical attention.

The incidence of Buerger disease is estimated at 8 to 12.6/100,000.²² Patients with Buerger disease are almost always young (younger than 45 years), use large amounts of tobacco, and characteristically show segmental occlusions in the radial, ulnar, palmar, and digital arteries, with typical bridging corkscrew collaterals. The disease is increasingly seen in women, commensurate with the increase in the proportion of female smokers.

Small vessel disease of the upper extremity is relatively common, especially as manifested as Raynaud disease and in the presence of systemic connective tissue disease. For example, over 90% of patients with scleroderma exhibit Raynaud-like symptoms (see later).

Hypothenar hammer syndrome (HHS) is an infrequent condition and its true prevalence is not known. Marie and associates ²³ have found HHS to be the cause of symptoms in 47 of 4148 patients (1.1%) referred for evaluation of Raynaud phenomenon. Serious complications, defined as requiring surgical intervention, caused by iatrogenic injury of the upper extremity arteries are also relatively rare. Myers and coworkers²⁴ have reported 11 patients over 4 years, whereas Deguara and colleagues²⁵ have reported 6 patients over 20 years. The incidence of serious iatrogenic upper extremity injury depends largely on the case mix of patients seen in the hospital and on the types of procedures performed.

Raynaud’s complex of symptoms is very common. Various studies that have been conducted in the general population in several different countries found the prevalence to range from 3% to 6% up to 30%. In these studies, between 70% and 90% of all reported patients were women. In a large United States registry of 1137 patients presenting with Raynaud, 356 (31.3%) suffered from pure vasospasm with no associated disease, 391 patients (34.4%) had associated connective tissue disease, and 389 patients (34.3%) suffered from other underlying diseases.²⁶

Etiology and Pathophysiology

For a detailed discussion of the pathophysiology of atherosclerosis, see Chapters 51 and 88.

Although the cause of Buerger disease is not known, there is a strong association between the use of tobacco and Buerger disease. Most patients are heavy smokers, but Buerger disease has also been reported in users of smokeless tobacco such as chewing tobacco and snuff. Tobacco use plays a central role in the pathogenesis, initiation, and continuation of the disease and is an absolute requirement for diagnosis. Buerger disease is characterized pathologically by highly cellular thrombus with relative sparing of the blood vessel walls. Multinucleated giant cells can even be observed within the clot in Buerger disease.

Involvement of the small arteries of the upper extremity is frequently encountered in rheumatic diseases. Szekanecz and Koch ²⁷ have recently reviewed the vascular biology underlying this process. Vascular injury is caused primarily by activated neutrophils and inflammatory mediators released by these cells. Rheumatic diseases are associated with accelerated atherosclerosis and increased cardiovascular morbidity and mortality.

Repetitive occupational or recreational trauma may damage the ulnar artery when it enters the hand through Guyon’s canal. The type of arterial abnormality often depends on the nature of the damage to the vessel. Intimal damage favors thrombotic occlusion, whereas injury to the media favors palmar aneurysms.²⁸^,²⁹ Thrombosis and aneurysm are common features seen angiographically with HHS.³⁰

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