Upper Extremity Occlusive Disease Workup

• In patients with Raynaud syndrome who may have systemic vasculitis or underlying connective-tissue disease, the following tests should be obtained:

  • Sedimentation rate - To detect systemic inflammation or vasculitis
  • Antinuclear antibodies (ANA) - To test for serology of systemic lupus erythematosus (SLE)
  • Rheumatoid factor (RF) - To test for serology of rheumatoid arthritis
• In selected patients, obtain a hypercoagulable workup. Molecular tests of hypercoagulability include the following:

  • Factor V Leiden mutation
  • Prothrombin 20210 gene mutation
  • Methyl tetrahydrofolate reductase (MTHFR) (homozygote)
  • Factor V (heterozygote)
  • Antithrombin III deficiency
  • Protein C deficiency
  • Protein S deficiency
  • Dysfibrinogenemia
  • Antiphospholipid antibodies
  • Hyperhomocysteinemia
  • Elevated lipoprotein (a)
  • Platelets
• CBC count, platelet count, and urinalysis
• Cryoglobulins, cold agglutinins, and serum protein electrophoresis in selected patients

• 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, as depicted in the images below. Brachial segment that demonstrates a high take-off of the radial artery from the mid brachial artery. Forearm vessels in a patient with distal embolization that demonstrate (1) radial artery, (2) interosseous artery, and (3) ulnar artery. (The ulnar artery demonstrates distal occlusion.) Distal ulnar artery occlusion and proximal radial artery occlusion with obliteration of the superficial palmar arch from distal embolization. Normal results on right upper extremity Doppler examination demonstrate triphasic waveform and wrist/brachial index of 0.63. Left upper extremity demonstrates axillary, brachial, and palmar artery disease.
  • 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, as depicted in the image below. Digital subtraction angiogram that demonstrates a normal subclavian axillary brachial segment with the arm at the patient's side.
• 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.

• Noninvasive laboratory studies, as depicted in the image below, include bilateral upper extremity arm, forearm, and digital blood pressures. An arteriogram of the aortic arch that demonstrates the (1) brachiocephalic vessel, (2) the right subclavian, (3) the right carotid, (4) the left carotid, and (5) the left subclavian. These are normal findings.
• 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.

In patients with clinical findings and angiography findings consistent with giant cell arteritis, obtaining a biopsy of the affected arteries is usually impossible without risking the destruction of collateral vessels around the occlusion. Because this disease can affect other beds, results from a temporal artery biopsy may be abnormal.