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Symptomatic upper extremity arterial occlusive disease is uncommon because of the abundant collateral network and the infrequency of atherosclerosis in the upper extremity. Patients who present with upper extremity ischemia range from young adults with nonatherosclerotic causes to elderly patients with atherosclerosis.
What is it?
Arterial occlusive disease of the upper extremity may represent either local or systemic disease. The pattern of arterial disease varies according to etiology. Diseases that affect the brachiocephalic vessels include atherosclerosis, arteritis, congenital anomalies, trauma, and fibromuscular dysplasia. In the United States, atherosclerosis is the most common cause of subclavian artery stenosis. Outside of the United States, Takayasu arteritis is more common. The axillary and brachial arteries are common sites of injury. One third of peripheral emboli lodge in the upper extremity, producing acute arterial occlusion. Radiation therapy of the chest or breast may induce subclavian artery disease.
Causes
- Large-vessel occlusion (eg, subclavian, brachial, forearm arteries)
- Atherosclerosis
- Trauma (eg, thoracic outlet syndrome, penetrating, blunt, iatrogenic)
- Arteritis (eg, Takayasu arteritis, giant cell)
- Irradiation
- Embolic (eg, cardiac or thoracic outlet in origin, including bacterial endocarditis, microemboli from ascending aorta, paradoxical emboli)
- Fibromuscular dysplasia
- Digital artery occlusion
- Connective-tissue disease - Scleroderma; chondrocalcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia (CREST); and mixed connective-tissue disease
- Buerger disease
- Hypersensitivity angitis
- Hematologic - Hypercoagulable states, hyperviscosity, malignancy
- Traumatic - Occupational (eg, hypothenar hammer syndrome, vibratory tools), iatrogenic, recreational (baseball palmar artery injuries)
- Infection - Infection from injection of drugs, infection from arterial procedures
- Flow phenomenon - Vascular steal related to dialysis access graft or fistula placement
Signs
- The patient’s history may include the following :
- Arm fatigue upon exercise (ie, subclavian artery occlusion)
- Vertebrobasilar insufficiency (ie, subclavian steal)
- Rest pain that involves hand and digits
- Digital gangrene
- Raynaud syndrome (eg, color changes—white, blue, red or white, red, blue)
- Smoking history
- Occupational and recreational history (eg, baseball pitcher, tennis player, handballer, carpenter)
- Drug ergots (peripheral vasoconstrictors used in the treatment of shock [eg, dopamine, adrenaline])
- The results of physical examination include the following:
- Fever (if an associated vasculitis is present)
- Unequal arm pressures (>20 mm Hg difference)
- Supraclavicular or infraclavicular bruit
- Adson maneuver (loss of radial pulse upon abduction and external rotation of the upper extremity)
- Supraclavicular pulsatile mass (associated with a subclavian aneurysm or cervical rib)
- Palpation of pulses (axillary, brachial, radial, ulnar)
- Digital gangrene
- Color and capillary refill of the digits
- A positive Allen test result : An abnormal result on the Allen test demonstrates an incomplete palmar arch. In this test, the ulnar and radial arteries are occluded with the fist clenched. The hand is then opened, releasing one of the arterial occlusions (radial or ulnar); prompt capillary refill should result. The same maneuver should then be performed with the release of the other artery. If the palmar arch is not intact, the release of the affected artery produces a sluggish capillary refill. Alternatively, a Doppler stethoscope is used to map these collateral flow patterns in the hand by manually occluding, one at a time, the radial and ulnar arteries.
Symptoms
- Arm fatigue : Carotid-subclavian bypass, percutaneous transluminal angioplasty (PTLA), and stent
- Vertebrobasilar insufficiency : Carotid subclavian bypass and possible vertebral artery transposition to carotid artery
- Subclavian aneurysm and thoracic outlet injuries with distal embolization : Resection of subclavian artery aneurysm and venous bypass and rib resection with thoracic outlet
- Acute arterial occlusion : Embolectomy for embolus and repair for trauma (blunt or penetrating)
- Chronic arterial occlusion with pain at rest, ulcer, or gangrene : Bypass using the autogenous vein for distal segments and prosthetic material for larger proximal segments, amputation (digital or forearm), and sympathectomy (controversial)
Tests
Imaging Studies
- Complete arteriography of both upper extremities is necessary to establish the diagnosis and plan an effective treatment.
- The arteries to the upper extremity must be clearly visualized, beginning with the arch and extending to the digits. Magnification produces detailed studies of the hand .
- Intra-arterial vasodilation often provides a detailed anatomy of the hand.
- The arm should be placed in the abducted externally rotated position to determine arterial occlusion produced by thoracic outlet structures .
- Chest radiography and cervical spine views reveal a cervical rib or abnormality of the first rib in patients with thoracic outlet syndrome. Alternatively, CT imaging with 3-dimensional reconstruction can be used.
- Transesophageal echocardiography (TEE) is performed in patients with a peripheral embolus suspected of originating from a cardiac source. TEE can be used to assess plaque in the ascending aorta as a source of the emboli or determine the presence of a right-to-left shunt through which paradoxical emboli might travel.
- Hand radiographs reveal calcinosis and tuft resorption.
Diagnostic Procedures
- Noninvasive laboratory studies (see Image 5) include bilateral upper extremity arm, forearm, and digital blood pressures.
- Doppler arterial waveforms are taken at the subclavian, axillary, brachial, ulnar, radial, and palmar arch. A triphasic waveform denotes normal arterial blood flow.
- Duplex scanning with Doppler spectral analysis and B-mode ultrasound scan provides a detailed anatomy of the subclavian, axillary, and brachial arteries.
- Photoplethysmography (PPG) is used to monitor arterial blood flow to the fingers during the Adson maneuver and provides objective evidence of arterial occlusion.
- The cold stimulation test is painful and rarely needed. A baseline temperature is recorded with a small digital thermistor. The hand is immersed in ice water for 20 seconds. The time to return to baseline temperature is normally 15 minutes. In patients with vasospastic disease, the recovery time is prolonged.
Treatment
Medical Therapy
- Long-term warfarin anticoagulation is recommended in patients with peripheral emboli from a cardiac source. An international normalized ratio (INR) of 2-3 is recommended.
- For emboli off the ascending aorta, aspirin or clopidogrel may be used. In rare cases, low-dose aspirin has been used with warfarin.
- Nifedipine (10 mg PO tid) is used in patients with vasospastic disease of the hand. If this is not tolerated, prazosin at low dose may be tried. A third-line drug with some effectiveness is hydralazine.
- Lifestyle changes are essential. Warm gloves must be worn, and the skin must be protected from drying and fissuring. Cold avoidance may require moving to a warm climate and avoidance of significantly chilled or air-conditioned environments. Avoidance of vibration trauma from work or hobbies may be necessary.
- In patients with Takayasu arteritis or giant cell arteritis, prednisone is the first-line agent. Immunosuppression with methotrexate or Cyclophosphamide (Cytoxan) may be necessary.
- Risk-factor modification and aspirin are essential for the treatment of atherosclerotic occlusion. Smoking cessation is mandatory, particularly in patients with Buerger syndrome. Total cholesterol levels should be reduced to below 200 mg/dL, and the low-density lipoproteins (LDL) levels should be 100 mg/dL or less.
Surgical Therapy
- Vein or prosthetic bypass
- Percutaneous balloon angioplasty and stenting
- Resection of aneurysm
- Removal of first rib or cervical rib
- Cervical sympathectomy
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