Surgery for Reflex Sympathetic Dystrophy (Complex Regional Pain Syndrome Type 1) 

Updated: May 08, 2018
Author: Satishchandra Kale, MD, MBBS, MBA, MCh(Orth), FRCS(Edin), FRCS(Tr&Orth); Chief Editor: Vinod K Panchbhavi, MD, FACS 

Overview

Background

Reflex sympathetic dystrophy (RSD) is a condition that is often described under various synonyms that point to its incompletely understood etiology. In 1864, Weir Mitchell coined the term causalgia to designate severe pain following nerve injury. In 1900, Sudeck described regional demineralization accompanying posttraumatic pain. In 1923, Leriche described vasomotor disequilibrium. In 1947, Evans introduced the term reflex sympathetic dystrophy.

In 1993, the International Association for the Study of Pain renamed algodystrophy complex regional pain syndrome (CRPS; also known as chronic regional pain syndrome). RSD is type 1 CRPS. It can be considered an excessive sympathetic reaction of joints and periarticular soft tissues to any insult, traumatic or unknown.[1] This is quite different from causalgia (type 2 CRPS), in which the etiology is a partial nerve injury.

RSD is characterized by pain, regional edema, joint stiffness, muscular atrophy, vasomotor disturbances (including temperature changes), trophic skin changes, and regional skeletal demineralization seen on radiographs. These changes are aggravated by activity and extend over a larger area than the primary injury or surgery, including the area distal to this focus.[2, 3]

Because pathognomonic criteria are lacking for RSD, a taxonomic system based on clear definitions and objective quantification is desirable. Therefore, the current terminology of CRPS is increasingly being used as an umbrella to replace the myriad empirical descriptions used previously. No apparent relation exists between the degree of initial trauma and the severity of RSD, but RSD generally is more frequent following minor trauma or operations.

Pathophysiology

RSD usually follows minor trauma or surgery. It also has been associated with various clinical conditions (eg, diabetes, parkinsonism). It begins with spontaneous pain associated with vasomotor and sudomotor disturbances.

Bonica described the progress of severe cases of RSD in three stages, as follows[4] :

  • The first (acute) stage is marked by pain, swelling, and warmth; neurologic changes, such as hyperesthesia (glove and stocking distribution), incoordination, tremor, muscle spasms, and paresis, may be seen
  • The second (dystrophic) stage is characterized by cold skin with trophic changes
  • The final (atrophic) stage is manifested by muscle wasting and joint contractures; symptoms usually are disproportionate to the cause and reflect disturbance of autonomic, sensory, and motor function

Etiology

Iatrogenic causes of RSD following surgery, such as carpal tunnel decompression or Dupuytren release, can be diagnosed easily.[5]  No clear etiology (including trauma) can be identified in 25-35% of cases. A detailed history can be useful to pinpoint uncommon causes of RSD.

Potential causes of RSD include the following:

  • Trauma (~60-65% of cases), minor (eg, Colles fracture, fracture of the metacarpals) rather than major in a significant number of cases [6]
  • Minor surgery (eg, release of carpal tunnel for median nerve compression, release of Dupuytren contracture [see the image below])
  • Treatment with antituberculous drugs or phenobarbital
  • Atypical causes such as pregnancy or postpartum causes, diabetes mellitus, malignant tumors, parkinsonism or other central nervous system (CNS) disorders [7]
Reflex sympathetic dystrophy following surgery for Reflex sympathetic dystrophy following surgery for Dupuytren contracture.

Possible contributory causes include the following:

  • Persistent mechanical irritation of peripheral nerves
  • Incomplete regeneration of the peripheral nerves
  • Abnormal neurotransmitter activity [8]
  • Nutritional deprivation secondary to abnormal arteriovenous shunting
  • Central pain imprinting
  • Genetic or familial predisposition (suggested but not proven) [9]

Statistically significant associations include the following:

  • Cigarette smoking has been associated with RSD
  • Patients frequently show high scores on  schizophrenia or  depression scales; investigating and treating the so-called diathesis is worthwhile
  • Incidence of RSD is high in wartime casualties

Epidemiology

United States and international statistics

According to various researchers, the incidence of RSD may be 2-17% following minor trauma or surgery. If causalgia is included in the broad definition, the incidence can be as high as 32-35%. In the past, many subtle forms of RSD were missed, but with increased awareness of the condition, actual incidence may be much higher than initially thought.

Worldwide, no regions or population groups have been demonstrated to have a predilection for RSD.

Age-, sex-, and race-related demographics

Most patients with RSD are aged 30-55 years; the mean age is 45 years. With increasing awareness, RSD is being diagnosed in children more often; however, no studies exist pointing to a particular age distribution.

RSD is more common in women than in men; the male-to-female ratio is approximately 3:7. The ratio of upper-extremity to lower-extremity involvement is approximately 2:1. Even in children, girls are affected more frequently than boys, but peculiarly, the lower extremity is involved more frequently than the upper extremity.[10]

No particular race has a predilection for RSD.

Prognosis

Mortality associated with RSD is negligible, though morbidity is extremely high. About 80% of patients with RSD diagnosed within 1 year of injury improve significantly. However, 50% of patients with untreated symptoms lasting more than 1 year have profound residual impairment.

Despite good results following intravenous sympathetic blockade and intensive mobilization techniques, weakness of the extremity resulting from RSD is seen in almost 50-65% of patients, even 18-24 months after the initial diagnosis. Full range of movement accompanying the above aggressive therapies is seen in 60-74% of patients. Prolonged morbidity is observed in about 50% of patients with psychiatric diathesis, workers' compensation claims, and lawsuits.[11]

Patient Education

Emphasize to all patients the importance of early and supervised mobilization of the affected part. Give patients a list of plaster instructions and a list of realistic goals to be achieved within a specified time interval. Any lag in achieving these objectives due to pain, swelling, or other causes should inspire concern leading to early diagnosis and institution of treatment.

 

Presentation

History

Patients with reflex sympathetic dystrophy (RSD; also referred to as complex regional pain syndrome [CRPS] type 1) have a history of trauma, minor rather than major (eg, Colles fracture), in about 50-65% of cases.[12] The condition may also follow a surgical procedure (see the image below).

Reflex sympathetic dystrophy following surgery for Reflex sympathetic dystrophy following surgery for Dupuytren contracture.

Physical Examination

Symptoms and signs of RSD include the following:

  • Pain, described as burning, throbbing, shooting, or aching
  • Hyperalgesia
  • Allodynia (perception of pain with normally innocuous stimuli, characteristic of sympathetically mediated pain [SMP])
  • Hyperpathia

Trophic changes (occurring within 10 days of onset of RSD in 30% of the extremities affected) include the following:

  • Stiffness and edema
  • Atrophy of hair, nails, and/or skin

Changes in autonomic function include the following:

  • Abnormal sweating, either excess or anhydrosis
  • Heat and cold insensitivity
  • Redness or bluish discoloration of the extremities
 

DDx

Diagnostic Considerations

Other problems to be considered include the following:

 

Workup

Imaging Studies

Radiography

Soft-tissue swelling and regional osteopenia may be present in patients with reflex sympathetic dystrophy (RSD; also referred to as complex regional pain syndrome [CRPS] type 1). Regional osteopenia is evident on plain films in 80% of extremities (see the image below).

Radiograph of affected extremity, depicting region Radiograph of affected extremity, depicting regional osteopenia contrasted with normal radiographic appearance of the opposite extremity.

The following five radiographic patterns were described by Genant et al[13] :

  • Irregular resorption of trabecular bone giving patchy appearance
  • Subperiosteal bony resorption
  • Intracortical bone resorption
  • Endosteal bone resorption
  • Surface erosions in subchondral and juxtacortical bone

Bone scanning

Three-phase technetium-99m scanning is commonly used. Scan findings are considered positive if flow is asymmetric in phases 1, 2, and/or 3. Bone scans do not correlate with symptoms or provide prognostic information.

Other Tests

Other tests to be considered include the following:

  • Diagnostic sympathetic blockade - Pain relief following sympatholytic intervention (eg, IV phentolamine administration) is indicative of RSD; a positive result to a phentolamine block test usually indicates a good prognosis with significant relief following administration of IV sympatholytic drugs
  • Thermography - Sweating is analyzed by using the resting sweat output (RSO) or the quantitative sudomotor axon reflex test (QSART)

Experimental investigations include the following:

  • Total digital blood flow using digital temperature measurements and laser Doppler flowmetry
  • Vital capillaroscopy - A technique using Doppler flowmetry to gauge anatomic vascular mapping and capillary blood flow in the affected extremity; in such an extremity, enlarged, dilated, distorted, and irregularly spaced capillary loops are depicted, and in addition, nail-fold capillaries may be absent in patients with underlying connective-tissue disease

Questionnaires used for subjective complaints of pain include the following:

  • Visual analogue score (VAS)
  • McGill pain questionnaire

Histologic Findings

Because RSD is a condition that seldom is treated surgically, histopathologic descriptions are rare.

Pathologic findings from osteonecrotic femoral head specimens have been studied extensively on the basis of intramedullary pressures (IMP) and intraosseous phlebography.

The gross appearance is as follows:

  • Spongy bone
  • Easily collapsible trabeculae
  • Medullary necrosis
  • Trabecular necrosis

The microscopic appearance is as follows:

  • Areas of vascular stasis and fibrosis
  • Lipoblastomatosis
  • Thickened arteriolar walls
  • Preserved articular cartilage and synovium
  • Thickening and retraction of the joint capsule limiting movements of the joints
  • No tendon involvement

Staging

The triphasic course of vasomotor instability has been used to stage RSD, as follows:

  • Phase 1 - The limb is swollen, hot, pink, and dry
  • Phase 2 - The limb is swollen, cool, blue, and damp with sweat
  • Phase 3 - The edema and vasomotor irritability have settled, resulting in a largely contracted extremity
 

Treatment

Approach Considerations

The goals of treatment of reflex sympathetic dystrophy (RSD; also referred to as complex regional pain syndrome [CRPS] type 1) are as follows[14, 15, 16, 12] :

  • Reduction of edema
  • Reduction of reflex muscular contractures
  • Reduction of articular stiffening
  • Pain blockage with mobilization under sympathetic blockade [17]

Management approaches include the following:

  • Prophylactic vitamin supplementation
  • Pharmacologic therapy [18]
  • Physical therapy [19]
  • Surgery

Pharmacologic Therapy

There is evidence that prophylactic oral administration of vitamin C may reduce the incidence of CRPS after wrist fractures. A daily dose of 500 mg for 50 days is recommended.[20]

Pharmacologic agents used are classified as follows:

  • Analgesics - Drugs with a long half-life are preferred (eg, codeine)
  • Antidepressants - These drugs modulate sympathetic activity and provide analgesia (eg, amitriptyline)
  • Anticonvulsants (eg, phenytoin)
  • Membrane-stabilizing agents (eg, lidocaine, tocainide)
  • Adrenergic compounds - Phentolamine (relief with intravenous [IV] phentolamine is pathognomic of sympathetically maintained pain [SMP], and patients with a positive response to IV phentolamine are likely to respond to other forms of sympatholytic interventions); phenoxybenzamine (nonselective adrenergic agent [21] ); clonidine (alpha 2-adrenergic agonist)
  • Calcium channel blockers - These reduce sympathetic tone by blocking calcium release following stimulation of adrenergic receptors (eg, nifedipine, amlodipine)
  • Corticosteroids - Membrane-stabilizing action (prednisolone 60 mg rapidly tapered over 5-10 days is commonly used)

Various routes of administration for pharmacologic interventions include the following:

  • IV regional infusions - Guanethidine sulfate; phentolamine; bretylium tosylate; reserpine; cortisone sulfate
  • Epidural injections (eg, clonidine) may provide relief in selected patients
  • Intra-articular injections of steroid preparations (eg, triamcinolone)
  • Intra-arterial injections (eg, reserpine, guanethidine)
  • Continuous autonomic blockade using local anesthetic agents for scalene/axillary/brachial/stellate ganglion blocks

In using sympathetic blockade, the presence of allodynia and hypoesthesia are negative predictors for success. The use of sympathetic blocks as treatment for RSD (CRPS 1) should be considered carefully between potential success and possible adverse effects. The procedure is as likely to cause a transient increase in pain as a decrease in pain. Patients should be warned about this symptomatology.[22]

Physical and Other Therapies

The following physical therapies may be useful[23, 19] :

  • Active and passive range-of-motion exercises
  • Transcutaneous electrical nerve stimulation (TENS)
  • Desensitization techniques
  • Sensory reeducation of the extremity
  • Mirror visual feedback in the treatment of complex regional pain syndrome [24]

Newer interventions include the following:

  • Biofeedback
  • Acupressure
  • Acupuncture

Surgical Therapy

Surgical/ablative therapies include the following[17] :

  • Chemical sympathectomy
  • Surgical sympathectomy
  • Implantable electrical stimulators
  • Bilateral anterior cingulotomy

Activity

Intensive physical therapy following a successful sympathetic blockade is key to faster rehabilitation in patients with RSD.

IV blocks with rigorous mobilization techniques must be implemented early in the treatment process for RSD.

Active and passive mobilization and heat and cold modalities all have been used with good effect in RSD, acting via the gate theory. The gate theory suggests that a finite amount of information can be received at the spinal cord or at the cortical level. The gate is the dorsal horn of the spinal cord. Therefore, painful stimuli, if displaced or modified by less noxious stimuli, cannot be processed through the gate.

Prevention

Most cases of RSD in orthopedic practice can be prevented through early detection and early treatment. In most cases, a possible cause can be identified, such as overly tight bandages or plasters, a limb improperly splinted, or neglect of active movement of the part. The treating physician should be aware of and alert for compression syndromes of the underlying nerve or for swelling and pain resulting from a displaced bone fragment. The mainstay of treatment is mobilization of the affected part.

 

Medication

Medication Summary

In patients with reflex sympathetic dystrophy, the goals of pharmacotherapy are to reduce morbidity and prevent complications.

Sympatholytic drugs

Class Summary

Suppress sympathetic nerve function / noradrenaline inhibitor.

Reserpine (Serpalan)

Inhibits vesicular uptake of noradrenaline and thus stops excitation of sympathetic nervous system; inhibits beta-hydroxylation of dopamine to noradrenaline.

Lewis et al reported good results in 90% of patients at 18 months, with 93% of patients experiencing pain relief.

Alpha-adrenergic blocking agents

Class Summary

May exert effect by causing blockade of postganglionic synapses.

Phenoxybenzamine (Dibenzyline)

May have effect through long-lasting noncompetitive alpha-adrenergic blockade of the postganglionic synapses in smooth muscle.

Phentolamine (Regitine)

Alpha-1 and alpha-2 adrenergic blocking agent that blocks circulating epinephrine and norepinephrine action.

Alpha-adrenergic agonists

Class Summary

May act to decrease muscle tone.

Clonidine (Catapres)

Stimulates alpha2-adrenoreceptors in brain stem, activating an inhibitory neuron, which in turn results in reduced sympathetic outflow.

Guanethidine (Ismelin)

Acts on postganglionic fibers at the presynaptic level, releasing noradrenaline and inhibiting reuptake and rerelease. Fiscat et al reported good results in 63% of cases; Bensigner et al reported good results in 58.6% of cases.

For administration, IV access is gained as close as possible to the involved part. A BP cuff is tied well above the site of pain and inflated above the systolic pressure; a second BP cuff is tied below the first cuff as in a Bier block. Pressure is maintained for 20 min, the limb manipulated, and pressure is reduced while alternating between the 2 cuffs.

Six blocks are performed on alternate days with rigorous rehabilitation; repeated blocks even if the first block fails.

Calcium channel blockers

Class Summary

Inhibit calcium ions from entering slow channels; select voltage-sensitive areas, or vascular smooth muscle.

Nifedipine (Adalat, Procardia)

May have a relaxant effect on certain muscles. Inhibits transmembrane influx of calcium ions into smooth muscle, which, in turn, inhibits contraction of the muscle fibers.

Amlodipine (Norvasc)

May have a relaxant effect on certain muscles. Inhibits transmembrane influx of calcium ions into smooth muscle, which, in turn, inhibits contraction of the muscle fibers.

Corticosteroids

Class Summary

Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisolone (Articulose-50, Delta-Cortef, Pediapred)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.

Anesthetics

Class Summary

Local anesthetics stabilize the neuronal membrane and prevent the initiation and transmission of nerve impulses.

Lidocaine (Anestacon, Dermaflex, Lidoderm, Zilactin-L)

Decreases permeability to sodium ions in neuronal membranes. This results in the inhibition of depolarization, blocking the transmission of nerve impulses.

Tricyclic antidepressants

Class Summary

A complex group of drugs that have central and peripheral anticholinergic effects and sedative effects. They have central effects on pain transmission. They block the active reuptake of norepinephrine and serotonin.

Amitriptyline (Elavil)

Analgesic for certain chronic and neuropathic pain.

Analgesics

Class Summary

Pain control is essential to quality patient care. Analgesics ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who experience pain.

Codeine

Binds to opiate receptors in CNS, causing inhibition of ascending pain pathways, altering perception and response to pain.