Surgical Treatment of Vasospastic and Vaso-occlusive Diseases of the Hand

Scott L. Hansen

Neil F. Jones

DEFINITION

Vasospastic and vaso-occlusive diseases of the hands include a wide range of disorders that cause decreased or limited blood flow to the digits, resulting in chronic ulcerations and potentially loss of digits.
Vasospastic disorders result from constriction of the microvasculature, resulting in decreased blood flow.
- The most common vasospastic disorder is Raynaud syndrome.
  - Raynaud syndrome may also have an obstructive component.
Vaso-occlusive disorders produce disruption of blood flow due to a reduction in cross-sectional area of the vessel lumen.

ANATOMY

The right common carotid artery and right subclavian artery originate from the brachiocephalic trunk, whereas the left subclavian artery branches directly from the aorta.
The subclavian artery becomes the axillary artery at the distal edge of the first rib and ends at the distal edge of the teres major tendon.
The brachial artery is a continuation of the axillary artery, beginning at the distal margin of the teres major.
The hand is supplied by the radial and ulnar arteries, which originate from the brachial artery at the level of the antecubital fossa.
The radial artery becomes the deep palmar arch; the ulnar artery becomes the superficial palmar arch (FIG 1).
The superficial palmar arch is usually the major arterial inflow to the fingers on the ulnar aspect of the hand, whereas the deep palmar arch supplies blood to the digits on the radial aspect of the hand.
- The superficial palmar arch lies more distal in the palm than the deep palmar arch.
In about 80% of patients, the deep and superficial palmar arches are in continuity, a configuration described as a complete palmar arch.⁴
In a very small percentage patients, a persistent median artery also can contribute blood supply to the hand.
Sympathetic nerves exit the spinal cord along with the ventral roots of the second and third thoracic nerves, passing via the brachial plexus into the forearm and hand.
- The sympathetic nerve fibers innervate the blood vessel walls, contributing to control of tone of the vascular smooth muscle.

PATHOGENESIS

Raynaud syndrome, a vasospastic disorder, is characterized by significant structural narrowing of the arterial lumen due to intimal hyperplasia. Vasospasm can occur from increased sympathetic tone in response to temperature, vibratory stimuli, and sometimes emotional stress, causing further ischemia and the clinical manifestation of color changes.
Vasospasm can also be associated with pheochromocytoma, carcinoid syndrome, and cryoglobulinemia.
Emboli can shower from a cardiac source (eg, chronic atrial fibrillation) or from microemboli in ulcerated, atherosclerotic plaques, either spontaneously or from iatrogenic cannulation of vessels during vascular procedures.
Thrombosis may occur spontaneously from atherosclerotic disease or from repetitive blunt trauma to the vessels, as in hypothenar hammer syndrome.
Low-flow states can occur in sepsis, malignant disease, hypercoagulable states (eg, polycythemia, lupus anticoagulant antibody), and after intra-arterial drug injections.
- Low-flow states predispose end organs to global thrombosis.
Focal stenosis and segmental occlusion of vessels may result from intimal proliferation secondary to connective tissue disorders, atherosclerosis, and renal vascular disease.

FIG 1 • Vascular anatomy of the hand.
Vasospastic disorders may result from increased sympathetic tone.
Vaso-occlusive disorders result in ischemia distal to the site of occlusion.

NATURAL HISTORY

Clinical manifestations of vasospastic disorders range from episodic digital vasospasm and pain to severe hand and digit ischemia, progressing to gangrene.
The classic triphasic attack in Raynaud syndrome consists of sudden onset of digital pallor or blanching after cold exposure or emotional stress, followed by a period of cyanosis and then redness with rewarming, resulting in the classic white-blue-red sequence of color changes.¹
- The typical Raynaud attack lasts for 15 to 45 minutes.
Vaso-occlusive disorders follow a more predictable clinical course in that they usually result from fixed lesions that are progressive.
Cold intolerance and vasomotor color changes in the hand develop, forcing patients to seek treatment.

PATIENT HISTORY AND PHYSICAL FINDINGS

A complete history and physical examination must be done on each patient, focusing on evidence of connective tissue or cardiovascular disease.
- Does the patient describe paresthesias, pallor, cold intolerance, pain, and digit ulceration?
The entire upper extremity is examined for range of motion, skin color and turgor, capillary refill, radial and ulnar pulses, temperature, and presence of ulcerations.
The distal fingertips and nails of each finger are examined closely.
The radial and ulnar pulses are palpated and examined by Doppler probe if necessary.
The palmar arch is assessed with the Doppler probe as well as the radial and ulnar digital arteries to each finger.
Allen test is performed.
- After the patient makes a tight fist to exsanguinate the blood form the hand, the radial and ulnar arteries are occluded at the level of the wrist.
- The arterial flow is then reestablished to the hand by sequentially releasing the radial and ulnar arteries while capillary refill is assessed simultaneously.
- This test evaluates the patency of arterial inflow to the hand through the radial and ulnar arteries.
- The same technique can be applied to each digit after compressing or milking the blood in the digit from distal to proximal.
Any pulsatile masses, chronic skin changes, or superficial vascular lesions are evaluated.

IMAGING AND OTHER DIAGNOSTIC STUDIES

Posteroanterior (PA), lateral, and oblique radiographs to evaluate bone architecture and the presence of any calcification in the radial and ulnar arteries, palmar arches, or digital arteries
Doppler examination of the arteries from the wrist to the digits
Echocardiogram to evaluate potential sources of emboli
Digital photoplethysmography, which measures digital volume changes over time, can be used to differentiate vasospastic from vaso-occlusive disease.
Segmental arterial pressure measurements
Nielsen digital hypothermic challenge test¹⁸
Ultrasonography for vascular tumors¹⁰
Angiography: remains the gold standard to evaluate blood flow to the hand
Magnetic resonance (MR) angiography⁵
Laboratory tests: complete blood cell count (CBC) with platelet count, coagulation studies, markers for collagen vascular diseases

DIFFERENTIAL DIAGNOSIS

Raynaud disease
Hypothenar hammer syndrome
Malignancy
Trauma
Buerger disease (thromboangiitis obliterans): an inflammatory occlusive disease of the small and medium-sized vessels of the limbs
Arteritis: a group of disorders characterized by acute or chronic inflammation in the walls of small, medium, and large arteries. Patients with these conditions often present with concurrent fever, malaise, weight loss, cutaneous lesions, and arthralgias.
Diabetes
Peripheral vascular disease, atherosclerosis
Thoracic outlet syndrome
Connective tissue disorders (eg, scleroderma, systemic lupus erythematosus, rheumatoid arthritis)
Illicit drug use
Vascular tumors
Pseudoaneurysm
Iatrogenic injury

NONOPERATIVE MANAGEMENT

Pharmacologic therapy is the mainstay of treatment of vasospastic disorders of the hand.
Avoidance of smoking and exposure to cold temperatures may control vasospastic episodes.⁹
Biofeedback
- Patients are trained to control certain bodily processes that occur involuntarily.
- Electrodes are attached to the skin of the patient and physiologic responses monitored.
- The biofeedback therapist then leads the patient through exercises that bring about desired physical changes.
Occlusive dressings may be helpful both to protect areas from recurrent trauma and to promote healing of lesions.
Calcium channel blockers (eg, nifedipine)
Pentoxifylline decreases blood viscosity and may result in relaxing vascular smooth muscle.
Prostacyclins²⁶
Nitrates
Local anesthetic blockade
Botulinum toxin A¹⁷,²⁵
Thrombolytic therapy