Chapter 127

 

 

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.

 

 

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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

 

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SURGICAL MANAGEMENT

 

The surgical management of vasospastic and vaso-occlusive diseases should proceed in a systematic fashion.

 

Indications for operative management are progressive symptoms (eg, Raynaud syndrome, ulcers, pain, cold intolerance) despite optimal medical management and with angiographically defined occlusion of one or both inflow arteries (ie, radial, ulnar).

 

Indications for a digital sympathectomy are progressive symptoms of Raynaud syndrome or ulcerations refractory to medical management with no evidence of major occlusion of the radial or ulnar arteries and with good visualization of three common digital arteries in the palm.

 

Cold challenges are very painful for patients with scleroderma and systemic lupus erythematosus and are used on a case-by-case basis.

 

The patient should be educated on the outcomes of the various procedures and realize the limitations of each one.

 

Newer techniques such as fat grafting may improve symptoms in certain patients.2

 

Preoperative Planning

 

The preoperative history and physical examination are reviewed.

 

 

The site of operative intervention is determined primarily by the preoperative imaging studies (eg, angiogram). If vascular grafting is indicated, the donor vessels are identified and marked.

Positioning

 

The patient is placed in the supine position on the operating room table with the extremity on an appropriately padded hand table.

 

An upper arm tourniquet is placed because a bloodless field is essential.

 

If a vein graft is anticipated, another extremity (usually a leg) is prepped and a proximal tourniquet is applied.

 

Approach

 

Usually, the surgeon must access proximal arterial inflow vessels when treating either vasospastic or vaso-

occlusive disorders of the hand.

 

The brachial artery in the upper arm is approached via an incision on the medial aspect of the arm.

 

The distal brachial artery and proximal radial and ulnar arteries are approached through a lazy S incision in the antecubital fossa.

 

 

Care is taken to avoid making a straight line incision across the antecubital fossa.

 

The radial and ulnar arteries in the forearm are approached through a longitudinal incision over the specific vessel.

 

The palmar arches are accessed via Bruner incisions extending proximally from the proximal phalanges, using natural creases in the palm where possible, or through an inverted J-shaped incision in the palm.

 

The digital arteries are approached through Bruner incisions on the palmar aspect of the finger or through midlateral incisions on the digit.

 

TECHNIQUES

• Flatt Digital Sympathectomy

   

  Flatt digital sympathectomy is used for patients with vasospastic disorders such as Raynaud phenomenon.6

   

  Proximal or cervical sympathectomy has largely fallen out of favor due to the high recurrence rates.

   

  Peripheral sympathectomy has gained popularity since Pick21 identified sympathetic nerve fibers innervating the arteries from the wrist to the fingers.

   

  Sympathectomy is performed at the level of the digital arteries.

   

  Make Bruner incisions in the distal palm and expose the digital arteries.

   

   

   

  TECH FIG 1 • A,B. View through the operating microscope before (A) and after (B) removal of the adventitia from a common digital artery. (continued)

   

   

  Disrupt all connections between the digital nerves and digital arteries.

   

  Strip the adventitia from the digital arteries over a distance of 0.5 to 2.0 cm using the operating microscope (TECH FIG 1A,B).

   

  Stripping must be performed very carefully to avoid damaging the digital arteries themselves.

   

  In cases of more widespread vasospasm, when more radical digital sympathectomy is desired, many surgeons recommend to strip the adventitia from the distal radial and ulnar arteries, the superficial palmar arch, and the common digital arteries in the palm11,12,19 (TECH FIG 1C,D).

   

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  TECH FIG 1 • (continued) C,D. Radical or extensive digital sympathectomy before (C) and after (D) stripping the adventitia from the distal ulnar artery, superficial palmar arch, and common digital arteries to the index-middle, middle-ring, and ring-small finger web spaces.

• Leriche Sympathectomy

   

  If adequate collateral flow is present, consider excision of a segment of thrombosed or occluded artery.15

   

  The concept is to reduce the sympathetic discharge from the diseased artery that is producing vasospasm in the more distal vessels.

   

  Resection is also used to treat a thrombosed or occluded ulnar artery in hypothenar hammer syndrome.

• Microsurgical Revascularization

   

   

  Reconstruction of a thrombosed or occluded artery is considered if: A discrete segment of artery can be resected and bypassed.

   

  Adequate arterial inflow and patent distal arteries with adequate distal “runoff” are present to take

  advantage of the restored blood flow.

   

  Resect the arterial segment and measure the defect.

   

  Reverse vein grafts (eg, cephalic, saphenous) or arterial grafts (eg, deep inferior epigastric artery, lateral circumflex artery, thoracodorsal artery) are harvested in the standard fashion.

   

  Draw an axial line down the length of the vessel to be harvested while in situ.

   

  The line helps prevent inadvertent “twisting” of the graft during the anastomoses.

   

  Perform standard microsurgical anastomoses using 9-0 or 10-0 nylon sutures and the operating microscope, between the distal radial or ulnar arteries and the deep or superficial palmar arches, respectively, or directly to one or more common digital arteries (TECH FIG 2).

   

  An end-to-side anastomosis of the graft to the inflow artery is preferable to maximize any remaining

  circulation to the hand, but end-to-end anastomoses are technically easier.

   

  The distal anastomosis is usually end to end to the superficial or deep palmar arches or end to side to the common digital arteries.

   

  After the anastomoses are completed, the tourniquet is deflated, and vascular inflow through the other artery is occluded by manual compression for a few minutes to maximize flow across the anastomoses.

   

  Restoration of arterial flow into the hand is assessed either by using a pencil Doppler probe or by performing an Acland “adventitial strip test” distal to the distal anastomosis.

   

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  TECH FIG 2 • A. Microsurgical revascularization for thrombosis or occlusive disease of the distal ulnar artery and superficial palmar arch, using an interposition vein graft from the ulnar artery to the common digital arteries. B. Microvascular revascularization for thrombosis or occlusive disease of the distal radial artery and deep palmar arch, using an interposition vein graft from the radial artery to the princeps pollicis artery.

• Embolectomy

   

  An acute embolus is treated by immediate heparinization to prevent propagation of the embolus more distally into the digits.

   

  Small Fogarty embolectomy catheters may be used selectively at the arm, elbow, forearm, and wrist levels, but use of embolectomy catheters in the hand and digits is difficult and can itself lead to vascular injury and further thrombosis.

   

  After identification of the segment involved by embolus, control the affected artery both proximal and distal to the embolus.

   

  Make a longitudinal arteriotomy proximally to access the vessel lumen.

   

  A side branch may be chosen if available.

   

  Insert the Fogarty catheter into the artery and pass it down the lumen beyond the area of occlusion; then

   

  inflate the balloon.

  Gently withdraw the catheter to retrieve any thrombus.

  Repeat catheterization until the lumen is completely cleared of the embolus, as demonstrated by improved back-bleeding from the distal vessel.

  Suture the arteriotomy and release arterial inflow.

  Assess the restoration of arterial flow into the hand either by using a pencil Doppler probe on the artery more distally or by performing an Acland adventitial strip test distal to the site of embolism.

• Arterialization of the Venous System

Choose a suitable vein on the dorsum of the hand, that is, one that will lie in a straight line following anastomosis to the radial or ulnar artery near the palmar wrist.20

Mobilize the vein and ligate the multiple side branches of the vein with small hemoclips to maximize flow to the fingers.

Perform valvulotomies in the vein to prevent valvular obstruction.

Ligate the vein proximally and perform an end-to-side microsurgical anastomosis between the vein and the radial or ulnar artery at the wrist.

After the anastomosis has been performed, assess arterial flow through the distal vein.

Any remaining obstruction due to a valve should be relieved by an open valvulotomy and excision of the valve leaflets, followed by microsurgical closure of the vein.

Postoperative monitoring is performed using a pencil Doppler probe over the distal arterialized vein to the fingers.

 

 

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PEARLS AND PITFALLS
	Indications ▪ History and physical examination should isolate the probable cause for ischemia. Preoperative studies should confirm the cause before surgical intervention.     Sympathectomy ▪ Strip the adventitia of the artery over a distance of 0.5-2.0 cm.     Microsurgical ▪ A discrete segment of thrombosed or occluded artery are identified to reconstruction be effective. Adequate arterial inflow and distal runoff is essential.     Embolectomy ▪ Identification of an embolus must be treated with heparinization propagation of the clot immediately to prevent propagation. Use of embolectomy catheters in the hand and digits should be done selectively and with caution.     Arterialization of the ▪ Generally used for unreconstructable vascular lesions.

 

	venous system ▪ Valvulotomies are performed to prevent vascular obstruction when flow is established. All venous side branches are ligated to maximize flow distally.

 

 

POSTOPERATIVE CARE

 

The hand is immobilized in a lightweight splint to protect the operative site, with care taken to avoid any pressure on the underlying anastomoses or vulnerable mobilized arteries.

 

The fingertips are assessed for color and capillary refill, temperature using a small temperature probes, or oxygen saturation using a pulse oximeter.

 

Microvascular reconstruction with interposition grafts can be monitored using a pencil Doppler probe.

 

Relative anticoagulation can be achieved using a continuous infusion of dextran 40 or low-dose aspirin.

OUTCOMES

Calcium channel blockers are moderately effective in patients with Raynaud phenomenon, with 35% reporting improvement in severity of their symptoms.23

Results of sympathectomy remain variable, although surgeons have reported improvements in pain, ulcer healing, cold intolerance, and quality of life.8,11,14,22,24

Long-term patency rates for vascular bypass grafting secondary to occlusive disease have been reported to range between 53% and 94%.3,11,13,16

Combining sympathectomy with arterial reconstruction may offer improved outcomes versus sympathectomy alone.7

 

 

COMPLICATIONS

Bleeding and hematoma Infection

Thrombosis of the interposition graft Progression of the underlying systemic disease

 

 

REFERENCES

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   disorders. Ann Vasc Surg 1992;6:418-424.

    

    

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20. Pederson WC, Woodward C, Hermansdorfer J. Arterialization of the venous system for the treatment of end-stage ischemia of the upper extremity. J Reconstr Microsurg 1996;12:414-417.

     

     

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23. Thompson AE, Shea B, Welch V, et al. Calcium channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum 2001;44:1841-1847.

     

     

24. Tomaino MM, Goitz RJ, Medsger TA. Surgery for ischemic pain and Raynaud's phenomenon in scleroderma: a description of treatment protocol and evaluation of results. Microsurgery 2001;21:75-79.

     

     

25. Van Beek AL, Lim PK, Gear AJ, et al. Management of vasospastic disorders with botulinum toxin A. Plast Reconstr Surg 2007;119: 217-226.

     

     

26. Wigley FM, Wise RA, Seibold JR, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med 1994;120:199-206.