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## Complete Summary

#### GUIDELINE TITLE

Reflex sympathetic dystrophy/complex regional pain syndrome clinical
practice guidelines - third edition.

#### BIBLIOGRAPHIC SOURCE(S)

International Research Foundation for RSD/CRPS. Reflex sympathetic
dystrophy/complex regional pain syndrome. 3rd ed. Tampa (FL):
International Research Foundation for RSD/CRPS; 2003 Jan 1. 48 p. \[66
references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

### \*\* REGULATORY ALERT \*\*

#### FDA WARNING/REGULATORY ALERT

**Note from the National Guideline Clearinghouse**: This guideline
references a drug(s) for which important revised regulatory and/or
warning information has been released.

-   [December 12, 2007,
    Carbamazepine](http://www.fda.gov/medwatch/safety/2007/safety07.htm#carbamazepine):
    The U.S. Food and Drug Administration (FDA) has provided
    recommendations for screening that should be performed on specific
    patient populations before starting treatment with carbamazepine.

-   [May 2, 2007, Antidepressant
    drugs](http://www.fda.gov/medwatch/safety/2007/safety07.htm#Antidepressant):
    Update to the existing black box warning on the prescribing
    information on all antidepressant medications to include warnings
    about the increased risks of suicidal thinking and behavior in young
    adults ages 18 to 24 years old during the first one to two months of
    treatment.

### COMPLETE SUMMARY CONTENT

 \*\* REGULATORY ALERT \*\*\
 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Reflex sympathetic dystrophy syndrome, also known as:

-   Complex regional pain syndrome (CRPS)

-   Causalgia (minor or major)

-   Sudeck´s atrophy

-   Post traumatic dystrophy (minor or major)

-   Shoulder hand syndrome

-   Reflex neurovascular dystrophy

To facilitate communication and understanding of the full spectrum of
signs and symptoms, the designation reflex sympathetic dystrophy
syndrome/complex regional pain syndrome (RSD/CRPS) is generally used
throughout these practice guidelines.

#### GUIDELINE CATEGORY

Diagnosis\
Management\
Treatment

#### CLINICAL SPECIALTY

Anesthesiology\
Internal Medicine\
Neurological Surgery\
Neurology\
Physical Medicine and Rehabilitation

#### INTENDED USERS

Advanced Practice Nurses\
Health Care Providers\
Health Plans\
Managed Care Organizations\
Occupational Therapists\
Patients\
Physical Therapists\
Physician Assistants\
Physicians\
Psychologists/Non-physician Behavioral Health Clinicians

#### GUIDELINE OBJECTIVE(S)

To provide concise, unbiased information that enables health care
providers and patients to make informed choices about the treatment of
reflex sympathetic dystrophy syndrome/complex regional pain syndrome
(RSD/CRPS)

#### TARGET POPULATION

Individuals with signs or symptoms suggestive of RSD/CRPS

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis**

1.  Assessment of clinical features

    -   Pain

    -   Skin changes

    -   Swelling

    -   Movement disorder

    -   Spreading symptoms

    -   Bone changes

    -   Duration

2.  Laboratory diagnostic aids

    -   Thermogram

    -   Bone scans

    -   Sympathetic blocks

    -   X-rays, electromyography (EMG), nerve conduction studies,
        computed axial tomography (CAT), and magnetic resonance imaging
        (MRI) studies

**Management/Treatment**

1.  Patient education

2.  Written treatment protocols

3.  Psychosocial modalities

    -   Pain-coping techniques

    -   Relaxation techniques

    -   Biofeedback

    -   Self-hypnosis

4.  Sequential drug trials/medication for chronic pain control

    -   Nonsteroidal anti-inflammatory agents (e.g., aspirin, ibuprofen,
        naproxen, indomethacin, etc.)

    -   Agents acting on the central nervous system by an atypical
        mechanism (e.g., tramadol)

    -   Anti-depressants (e.g., amitriptyline, doxepin, nortriptyline,
        trazodone, etc.)

    -   Oral lidocaine (mexilitine - somewhat experimental)

    -   Anti-convulsants (e.g., carbamazepine, gabapentin)

    -   Oral opioids (e.g., narcotics with names such as Darvon,
        Vicodin, Loratab, Percocet, morphine, codeine, etc)

    -   Clonidine patch

    -   Klonopin (clonazepam)

    -   Baclofen

    -   Capsaicin cream

5.  Physical and occupational therapy

    -   Weight-bearing versus non-weight-bearing exercises

    -   Hydrotherapy

    -   Pressure massage

    -   Moist heat applications

    -   Use of a transcutaneous electronic nerve stimulation (TENS) unit

    -   Pool therapy

6.  Sympathetic blocks

    -   Upper extremity, stellate ganglia block (SGB)

    -   Lower extremity, lumbar sympathetic block (LSB)

    -   Local injection of muscle trigger-points

    -   Phentolamine test

    -   Epidural block

    -   Intravenous tourniquet technique

7.  Sympathectomy

    -   Traditional approach

    -   Laparoscopic approach

8.  Placebo effect for assessing treatment effectiveness

9.  Spinal cord stimulation

10. Morphine pump

    -   Implantation of a morphine pump for the spinal infusion of
        morphine

    -   Implantation of a morphine pump for the spinal infusion of
        baclofen

#### MAJOR OUTCOMES CONSIDERED

-   Physical functioning

-   Pain

-   Health-related quality of life

-   Sensitivity and specificity of diagnostic tests

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

Not stated

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Subjective Review

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

Not applicable

#### METHODS USED TO ANALYZE THE EVIDENCE

Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not stated

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Not stated

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Not applicable

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

A Scientific Advisory Committee reviewed and commented on the third
edition of the guidelines. The committee members represented the fields
of neurology, anesthesiology, and psychiatry. They were chosen because
of their acknowledged expertise worldwide in the area of reflex
sympathetic dystrophy/complex regional pain syndrome (RSD/CRPS).

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

**Making the Diagnosis of RSD/CRPS**:

The diagnosis of reflex sympathetic dystrophy/complex regional pain
syndrome (RSD/CRPS) can be made in the following context. A history of
trauma to the affected area associated with pain that is
disproportionate to the inciting event plus one or more of the
following:

-   Abnormal function of the sympathetic nervous system

-   Swelling

-   Movement disorder

-   Changes in tissue growth (dystrophy and atrophy)

Thus patients do not have to meet all of the clinical manifestations
listed above to make the diagnosis of RSD/CRPS. The new CRPS
classification system acknowledges this fact by stating that some
patients with RSD/CRPS may have a third type of CRPS by categorizing it
as \"otherwise not specified.\" There seems to be a small group of
patients whose pain following trauma resolves over time, leaving the
patient with a movement disorder. The pain and symptoms of RSD/CRPS may
exceed both the magnitude and duration of symptoms expected from the
normal healing process expected from the inciting event. Similarly, the
RSD/CRPS diagnosis is precluded by the existence of known pathology that
can be explained by the observed symptoms and degree of pain. There are
\"grades\" of this syndrome described in the literature with symptoms
ranging from minor to severe.

**Clinical Features of RSD/CRPS**:

1.  **Pain** -- The hallmark of RSD/CRPS is pain and mobility problems
    out of proportion to those expected from the initial injury. The
    first and primary complaint occurring in one or more extremities is
    described as severe, constant, burning and/or deep aching pain. All
    tactile stimulation of the skin (e.g. wearing clothing, a light
    breeze) may be perceived as painful (allodynia). Repetitive tactile
    stimulation (e.g. tapping on the skin) may cause increasing pain
    with each tap and when the repetitive stimulation stops, there may
    be a prolonged after-sensation of pain (hyperpathia). There may be
    diffuse tenderness or point-tender spots in the muscles of the
    affected region due to small muscle spasms called muscle trigger
    points (myofascial pain syndrome). There may be spontaneous sharp
    jabs of pain in the affected region that seem to come from nowhere
    (paroxysmal dysesthesias and lancinating pains).

2.  **Skin changes** - Skin may appear shiny (dystrophy-atrophy), dry or
    scaly. Hair may initially grow coarse and then thin. Nails in the
    affected extremity may be more brittle, grow faster and then slower.
    Faster growing nails is almost proof that the patient has RSD/CRPS.
    RSD/CRPS is associated with a variety of skin disorders including
    rashes, ulcers and pustules. Although extremely rare, some patients
    have required amputation of an extremity due to life-threatening
    reoccurring infections of the skin. Abnormal sympathetic (vasomotor
    changes) activity may be associated with skin that is either warm or
    cold to touch. The patient may perceive sensations of warmth or
    coolness in the affected limb without even touching it (vasomotor
    changes). The skin may show increased sweating (sudomotor changes)
    or increased chilling of the skin with goose flesh (pilomotor
    changes). Changes in skin color can range from a white mottled
    appearance to a red or blue appearance. Changes in skin color (and
    pain) can be triggered by changes in the room temperature,
    especially cold environments. However, many of these changes occur
    without any apparent provocation. Patients describe their disease as
    though it had a mind of its own.

3.  **Swelling** - Pitting or hard (brawny) edema is usually diffuse and
    localized to the painful and tender region. If the edema is sharply
    demarcated on the surface of the skin along a line, it is almost
    proof that the patient has RSD/CRPS. However, some patients will
    show a sharply demarcated edema because they tie a band around the
    extremity for comfort. Therefore, one has to be certain that the
    sharply demarcated edema is not due to a previously wrapped bandage
    around the extremity.

4.  **Movement Disorder** - Patients with RSD/CRPS have difficulty
    moving because they hurt when they move. In addition, there seems to
    be a direct inhibitory effect of RSD/CRPS on muscle contraction.
    Patients describe difficulty in initiating movement, as though they
    have \"stiff\" joints. This phenomenon of stiffness is most
    noticeable to some patients after a sympathetic nerve block when the
    stiffness may disappear. Decreased mobilization of extremities can
    lead to wasting of muscles (disuse atrophy). Some patients have
    little pain due to RSD/CRPS but instead they have a great deal of
    stiffness and difficulty initiating movement. Tremors and
    involuntary severe jerking of extremities may be present.
    Psychological stress may exacerbate these symptoms. Sudden onset of
    muscle cramps (spasms) can be severe and completely incapacitating.
    Some patients describe a slow \"drawing up of muscles\" in the
    extremity due to increased muscle tone leaving the hand-fingers or
    foot-toes in a fixed position (dystonia).

> Patients with such seemingly bizarre movements might be inaccurately
> diagnosed with a psychogenic movement disorder. Additionally, the fact
> that quite extreme behavioral changes often follow rather trivial
> injuries in patients with RSD/CRPS, this observation might contribute
> to the perception that the patient suffers from a psychogenic disorder
> as well.
>
> Specific diagnostic criteria have been established for the diagnosis
> of psychogenic movement disorders in cases involving RSD/CRPS.
> Unfortunately, physicians sometimes fail to follow these diagnostic
> guidelines and carelessly report a \"psychogenic\" disorder. This
> misdiagnosis can be devastating to the patient and can lead to
> delaying urgent medical care.
>
> A clinical test sometimes used to demonstrate that a patient is faking
> muscle weakness is called \"give away weakness\". This test is NOT a
> reliable indicator of a psychogenic movement disorder. Patients with
> RSD/CRPS will give away when a force is applied to their extremity
> because of pain. Also, because patients with RSD/CRPS have difficulty
> sustaining muscle contraction, they will give away as well.

5.  **Spreading Symptoms** - Initially, RSD/CRPS symptoms are generally
    localized to the site of injury. As time progresses, the pain and
    symptoms tend to become more diffuse. Typically, the disorder starts
    in an extremity. However, the pain may occur in the trunk or side of
    the face. On the other hand, the disorder may start in the distal
    extremity and spread to the trunk and face. At this stage of the
    disorder, an entire quadrant of the body may be involved.
    Researchers have described three patterns of spreading symptoms in
    RSD/CRPS:

    a.  A \"continuity type\" of spread where the symptoms spread upward
        from the initial site, e.g. from the hand to the shoulder.

    b.  A \"mirror-image type\" where the spread was to the opposite
        limb.

    c.  An \"independent type\" where symptoms spread to a separate,
        distant region of the body. This type of spread may be
        spontaneous or related to a second trauma.

6.  **Bone changes** -- X-rays may show wasting of bone (patchy
    osteoporosis) or a bone scan may show increased or decreased uptake
    of a certain radioactive substance (technetium 99m) in bones after
    intravenous injection.

7.  **Duration of RSD/CRPS** -- The duration of RSD/CRPS varies, in mild
    cases it may last for weeks followed by remission; in many cases the
    pain continues for years and in some cases, indefinitely. Some
    patients experience periods of remission and exacerbation. Periods
    of remission may last for weeks, months, or years.

**Stages of RSD/CRPS**:

The staging of RSD/CRPS is a concept that is dying. The course of the
disease seems to be so unpredictable between various patients that
staging is not helpful in the treatment of RSD/CRPS. Not all of the
clinical features listed below for the various stages of RSD/CRPS may be
present. The speed of progression varies greatly in different
individuals. Stage I and II symptoms begin to appear within a year. Some
patients do not progress to Stage III. Furthermore, some of the early
symptoms (Stage I and II) may fade as the disease progresses to Stage
III. The following stages are presented in these guidelines merely for
historical significance.

**Stage I**

1.  Onset of severe, pain limited to the site of injury

2.  Increased sensitivity of skin to touch and light pressure
    (hyperesthesia)

3.  Localized swelling

4.  Muscle cramps

5.  Stiffness and limited mobility

6.  At onset, skin is usually warm, red and dry and then it may change
    to a blue (cyanotic) in appearance and become cold and sweaty

7.  Increased sweating (hyperhydrosis)

8.  In mild cases, this stage lasts a few weeks, then subsides
    spontaneously or responds rapidly to treatment

**Stage II**

1.  Pain becomes even more severe and more diffuse

2.  Swelling tends to spread and it may change from a soft to hard
    (brawny) type

3.  Hair may become coarse then scant, nails may grow faster then grow
    slower and become brittle, cracked and heavily grooved

4.  Spotty wasting of bone (osteoporosis) occurs early but may become
    severe and diffuse

5.  Muscle wasting begins

**Stage III**

1.  Marked wasting of tissue (atrophic) eventually become irreversible

2.  For many patients the pain becomes intractable and may involve the
    entire limb

3.  A small percentage of patients have developed generalized RSD
    affecting the entire body

**Laboratory Diagnostic Aids**:

There is no laboratory test that can stand alone as proof of RSD/CRPS.
However, there are a couple of tests (thermogram and bone scan) which
can be useful in providing evidence for RSD/CRPS.

**Treatment**:

The single most important modality for treating the patient with
RSD/CRPS is education. The informed consent process should be the focus
of education. The physician defines the potential benefits, risks,
alternatives (and costs). From the start, the therapeutic goals must be
defined and accepted by the patient:

-   Educate about therapeutic goals

-   Encourage normal Use of the limb (physical therapy)

-   Minimize pain

-   Determine the contribution of the sympathetic nervous system to the
    patient\'s pain

The cornerstone in the treatment of RSD/CRPS is normal use of the
affected part as much as possible. Therefore, all modalities of therapy
(drugs, nerve blocks, transcutaneous electronic nerve stimulation
\[TENS\], physical therapy, etc.) are employed to facilitate movement of
the affected region of the body. Although physical therapy is an
important treatment modality, significant misuse and overuse of this
modality may occur. Often the physical therapist will treat the patient
with RSD/CRPS the same as a stroke or a nerve plexus injury, (which will
fail due to extreme pain and possible injury with passive manipulation).
The primary goal of the physical therapist should be to teach the
patient how to use their affected body part through activities of daily
living. Swimming pool exercises are very helpful, especially for
RSD/CRPS of the lower extremity where weight-bearing can be problematic.
The goal of physical therapy should be to create independence from the
health care system in the shortest period. Learning that \"to hurt is
not to harm\" is difficult, but it is essential to avoid re-injury.
Learning the non-protective nature of pain due to RSD/CRPS takes time.

For patients who are significantly impaired in their ability to mobilize
their extremity, it is urgent to offer the patient the opportunity to
determine the contribution of their sympathetic nervous system to their
pain. This is accomplished by a sympathetic nerve block to the affected
extremity (Figure 2, \"Treatment Protocol\" in the original guideline).
Future therapeutic options for the patient will depend on whether their
pain is determined to be sympathetically maintained pain (SMP) or
sympathetically independent pain (SIP). Published reports suggest that
the best response to sympathetic blocks will occur if the blocks are
given as soon as possible during the course of the disease.

The \"LET\'S TRY THIS NOW\" approach is to be deplored because it
indicates that the physician has not defined a strategy to achieve
specific therapeutic goals in the shortest period of time. It also adds
to the confusion, frustration, anxiety and depression of the patient,
which may intensify the patient\'s pain and adversely affect the
doctor-patient relationship.

1.  **Establish a written treatment protocol**:

> Initiate the safest, simplest, and most cost-effective therapies
> first. (Refer to Figure 2 in the original guideline for details). If
> the patient fails to progress in mobilizing the extremity, it is
> essential to offer the patient a series of 3 sympathetic blocks
> immediately. The purpose of the sympathetic blocks is three-fold: to
> treat, to diagnose if the pain is sympathetically maintained and to
> provide prognostic information. The sympathetic block provides a
> prognostic indicator if sympathectomy or other treatment modalities
> would be the next appropriate step. Sympathetic blocks are discussed
> in detail below.
>
> After the physician has completed a defined course of treatment (e.g.,
> a series of 3-6 sympathetic blocks), it would be helpful to prepare an
> update report that would document the patient\'s response to the
> course of treatment. The report should reflect a basis for further
> treatment and it should address future rehabilitation needs. Sharing a
> copy of the update report with the patient will help ensure that all
> parties are kept informed. Sharing the report with the patient helps
> keep the patient and physician focused on achieving appropriate
> therapeutic goals. An update report should address five areas of care:

1.  Procedures (e.g., nerve blocks)

2.  Medications

3.  Physical/occupational therapy

4.  Psychosocial issues

5.  New laboratory tests or consults

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2.  **Psychosocial modalities must be considered in all patients with
    RSD/CRPS**:

> Psychiatric illness or personality disorder does not cause RSD/CRPS
> but it is likely that personality contributes to the disease. Patients
> with severe, advanced stage RSD/CRPS usually undergo a psychosocial
> evaluation during the series of sympathetic blocks or prior to
> offering the patient more invasive treatments. In some cases, a formal
> psychosocial evaluation should be initiated much earlier in the course
> of treatment. For example, children with RSD/CRPS may require a
> thorough evaluation to determine the family support structure and the
> coping mechanisms needed by the family for optimal rehabilitation of
> the child.
>
> The psychosocial evaluation should always be done by an expert in
> chronic pain and should always include an assessment of pain coping
> skills and drug abuse potential. Stress is a known cause of
> exacerbation of this disease, making emergency treatment more
> necessary. A lot of memorials sent to fund RSD/CRPS research are the
> result of suicides! ***The potential for committing suicide needs to
> be assessed.*** The patient may need to participate in a formal pain
> management program as an outpatient or an inpatient. Chronic pain
> patients referred for a psychosocial evaluation tend to be defensive.
> An MMPI or other psychological test can help identify the psychosocial
> problems. Patients must be properly motivated to improve their coping
> skills; otherwise, application of these psychosocial modalities is a
> waste of time. Relaxation techniques (e.g. breathing exercises) as
> well as biofeedback and self-hypnosis may be appropriate treatment
> modalities for some patients.

3.  **Sequential Drug Trials**:

> Try to initiate sequential trials for each modality of therapy. The
> application of multiple therapies at the same time, a shotgun
> approach, makes it almost impossible to evaluate and optimize an
> individual therapy for safety and efficacy. Patients must be advised
> that the optimal dose for medications varies greatly among patients.
> Therefore, it is usually necessary to gradually increase the dose of
> their medication to the point of significant toxicity in order to
> determine optimal dose. The dose is then reduced to the next lower
> level. Thus it is important for the patient to become familiar with
> all of the potential side effects of a medication before trying it.
> Sequential trials with many different drugs may be required to
> determine the best medication for the patient.
>
> Medications are generally prescribed according to the following
> characteristics of the pain:

-   Constant pain

-   Pain causing sleep problems

-   Inflammatory pain or pain due to recent tissue injury

-   Spontaneous jabs (paroxysmal dysesthesias and lancinating pain)

-   Sympathetically maintained pain (SMP)

-   Muscle cramps

> **Medications Used to Treat Chronic Pain**:
>
> \"Off-labeling\" prescribing means that a government (e.g., the U.S.
> Food and Drug Administration \[FDA\]) approved the medication for one
> purpose but it is used by physicians for another purpose. For example,
> aspirin is a pain medication but it can also be used to decrease the
> risk of a heart attack by inhibiting the aggregation of platelets.
> Off-label prescribing is a common practice in treating various chronic
> pain problems. Some of these drugs have been proven to be effective in
> decreasing pain due to nerve injury (neuropathic pain) in
> well-controlled clinical trials. Since RSD/CRPS is believed to be
> caused by nerve injury (neuropathic pain), these drugs are used to
> treat this condition as well. The patient should consider weaning
> themselves from these various medications periodically with the
> treating physician\'s knowledge to determine for themselves that the
> medication is actually helping to alleviate their symptoms. Some
> medications need to be weaned slowly (e.g. narcotics, baclofen) to
> minimize withdrawal symptoms.
>
> **Medications Commonly Used to Treat RSD/CRPS Based on the Type of
> Pain Include**:
>
> *[For constant pain associated with inflammation]{.underline}*:

-   Nonsteroidal anti-inflammatory agents (e.g., aspirin, ibuprofen,
    naproxen, indomethacin, etc.)

> *[For constant pain not caused by inflammation]{.underline}*:

-   Agents acting on the central nervous system by an atypical mechanism
    (e.g., tramadol)

> *[For constant pain or spontaneous (paroxysmal) jabs and sleep
> disturbances]{.underline}*:

-   Anti-depressants (e.g. amitriptyline, doxepin, nortriptyline,
    trazodone, etc.)

-   Oral lidocaine (mexilitine - some what experimental)

> *[For spontaneous (paroxysmal) jabs]{.underline}*:

-   Anti-convulsants (e.g., carbamazepine, gabapentin may relieve
    constant pain as well)

> *[For widespread, severe RSD/CRPS pain, refractory to less aggressive
> therapies]{.underline}*:
>
> **Oral opioid**. The use of opioids (e.g. narcotics with names such as
> Darvon, Vicodin, Loratab, Percocet, morphine, codeine, etc) to treat
> RSD/CRPS is debated and there are potential hazards. Therefore, in
> order to ensure appropriate informed consent, it is recommended that
> the patient sign a doctor-patient \"contract\" (Refer to the \"Opioid
> Treatment Protocol\" in the original guideline for details).
>
> Patients may require immediate and adequate pain relief. In some cases
> it may take time to transpire from the time of the patient\'s first
> visit to the time of adequate treatment. In all probability, the pain
> and degenerative cycle would progress. Since the abuse potential is
> minimal when narcotics are used for severe pain, practitioners should
> not withhold narcotic treatment, if the patient demonstrates pain
> relief with this medication.
>
> *[For the treatment of sympathetically maintained pain
> (SMP)]{.underline}*:

-   Clonidine Patch. Studies suggest that clonidine may decrease pain in
    RSD/CRPS by inhibiting the sympathetic nervous system. A treatment
    protocol for using the Clonidine Patch to treat RSD/CRPS can be
    found in the journal *Regional Anesthesia*.

> *[For muscle cramps (spasms and dystonia) which can be very difficult
> to treat]{.underline}*:

-   Klonopin (clonazepam)

-   Baclofen

> *[For localized pain related to nerve injury]{.underline}*:

-   Capsaicin cream. (This medication is applied to the skin and behaves
    like hot peppers. The effectiveness of capsaicin cream in the
    treatment of RSD/CRPS has not been determined).

1.  **Physical and Occupational Therapy**:

> Patients need to be educated on how to use their affected body part
> through activities of daily living. For example, for lower extremity
> RSD/CRPS, patients may need to be taught weight bearing versus
> non-weight bearing exercises. Hydrotherapy is usually medically
> necessary for muscle (myofascial) pain and spasms. Application of
> pressure (massage) and/or moist heat applications can sometimes
> relieve severe muscle cramps. The physical therapist can also teach
> the patient how to use a TENS unit (a noninvasive electrical device
> that stimulates the surface of the skin). Pool therapy can be very
> effective for improving mobility

2.  **Sympathetic Blocks**:

> There are three reasons to consider sympathetic blockade to facilitate
> the management of RSD/CRPS. First, the sympathetic block may provide a
> permanent cure or partial remission of RSD/CRPS. Second, by
> selectively blocking the sympathetic nervous system the patient (and
> physician) will gain further diagnostic information about what is
> causing the pain. The sympathetic block helps determine what portion
> of the patient\'s pain is being caused by malfunction of their
> sympathetic nervous system. Third, the patient\'s response to a
> sympathetic block provides prognostic information about the potential
> merits of other treatments.
>
> The maximum sustained benefit from a series of sympathetic blocks is
> usually apparent after a series of 3 to 6 blocks. Even if the original
> site is unresponsive to sympathetic blockade, future exacerbation of
> RSD/CRPS symptoms at the same site or at a distant site may be
> responsive to 1 to 3 sympathetic blocks. THE GOAL IS ALWAYS TO TREAT
> BUT DON´T OVER TREAT. (Refer to the treatment section of the original
> guideline for additional details and rationale for sympathetic
> blockade.)

3.  **Sympathectomy**:

> If there is a significant decrease in pain following the sympathetic
> block, the patient is said to have sympathetically maintained pain
> (SMP). If there is not a significant decrease in pain, the patient has
> sympathetically independent pain (SIP). Only patients with SMP should
> be considered for a sympathectomy. Patients are advised to expect no
> more relief of their pain from a permanent block, i.e. sympathectomy,
> than they received from either a SGB or a LSB. Thus the patient must
> really pay attention to the magnitude of pain relief and improvement
> in function following each sympathetic block. Sympathectomy is a
> relatively invasive procedure with potential complications and should
> be pursued by the patient only if they are certain about the temporary
> therapeutic benefits that they received from a series of SGBs or LSBs.
>
> Recently, laparoscopic sympathectomy has been developed for
> sympathectomy of the upper extremity. This technique requires the
> placement of three small holes temporarily in the side of the chest
> wall while the patient is under general anesthesia. For the lower
> extremity, the patient has the choice of dissolving (destroying) the
> sympathetic nerves with phenol injected through a needle while the
> patient is awake (percutaneous phenol sympathetic neurolysis) or a
> surgical sympathectomy under general anesthesia. Other techniques for
> sympathectomy have also been used. The patient must be informed of the
> pros and cons of each approach.
>
> Post-sympathectomy pain (neuralgia) is a potential complication of all
> types of sympathectomy. Post-sympathectomy pain is typically proximal
> to the original pain (e.g. proximal means that the pain may appear for
> the first time in the groin or buttock region for sympathectomy of the
> lower extremity and pain in the chest wall region for sympathectomy of
> the upper extremity). Patients may think that their RSD/CRPS has
> spread to a new region after sympathectomy because the pain feels
> similar to their original RSD/CRPS pain. The post-sympathectomy pain
> usually resolves on its own or with 1-3 sympathetic blocks. Thus for
> some patients, sympathectomy may be a two-step procedure; destruction
> of sympathetic nerves followed by a sympathetic block.
>
> Published data suggests that sympathectomy in properly selected
> RSD/CRPS patients may provide one of the most effective treatments for
> RSD/CRPS. The selection criteria for sympathectomy are critical in
> achieving long-term success.

4.  **Placebo**:

> The placebo effect (decreased pain due to an inactive treatment such
> as a sugar pill) must be considered in the treatment of RSD/CRPS.
> Although the figure of 33% is commonly quoted in papers and textbooks
> as the percentage of people who will respond to a placebo, it is
> misleading because the \"percentage\" varies enormously (from close to
> 0 to 100%) depending on the exact circumstances. Physician and patient
> must have an understanding about the placebo effect, otherwise the
> patient is at risk of being over-treated. Recognition of placebo
> versus specific pain-relieving treatment may be difficult, but there
> are some distinguishing characteristics.

-   The greater the invasiveness of the procedure itself, the greater
    the placebo effect.

-   The greater the expectation for pain relief, the greater will be the
    placebo effect.

-   The placebo tends to be of less duration. For example, close
    monitoring of the patient\'s pain for hours and days after each
    sympathetic nerve block has shown that the pain-reducing effect of
    the saline (placebo) injection subsides within the first few hours,
    whereas that of the local anesthetic injection persists for several
    days.

-   The placebo tends to be less reproducible with each successive
    treatment.

> **Therefore, it may be of great potential therapeutic value to provide
> each patient with a series of multiple sympathetic blocks separated by
> brief intervals (e.g. one week) simply to determine whether such
> blocks are effective treatments.**
>
> The time-course of pain relief and improvement in function must be
> monitored closely by the patient. The actual local \"anesthetic\"
> effect of a sympathetic block lasts for only a few hours. But patients
> with SMP usually experience pain relief that far outlasts the duration
> of the local anesthetic effect. This type of extended relief of pain
> and improvement in mobility beyond the duration of the nerve block is
> believed to indicate an element of \"reflex\" activity or a \"vicious
> cycle\" in the affected region of the body, either from muscle spasm
> or from sympathetic over-activity.
>
> Intentional or not, some patients may not reliably report the effects
> of sympathetic blocks. As noted, a good sympathetic block provides a
> feeling of warmth that will act as a \"cue.\" Some patients respond to
> that change in sensation by anticipating the results or stating it as
> a genuinely perceived reduction in pain. Others may deceitfully report
> pain relief, since they believe that such a report is necessary for
> further treatment, attention, or other desired gain. Some patients may
> feel that some \"treatment\" is better than no treatment at all, even
> if the treatment is ineffective.

5.  **Other Types of \"Sympathetic Blocks\"**:

> A sympathetic blocker (alpha adrenergic antagonist), phentolamine,
> given I.V. has been advocated as a diagnostic test for SMP. However,
> false negative tests have been reported as high as 43%. Moreover, this
> approach is somewhat elaborate and requires considerable technician
> time and expense. The phentolamine test is a diagnostic procedure
> while a sympathetic block is a diagnostic, prognostic and therapeutic
> procedure. However, the phentolamine test may be valuable treatment
> option in the situation where a sympathetic block is not possible or
> when multiple extremities are involved.
>
> Epidural blocks are less specific for blocking the sympathetic nervous
> system and, therefore, they are not as useful for diagnostic and
> prognostic objectives. The infusion of local anesthetic through the
> epidural catheter may cause temporary weakness in the legs, making
> walking dangerous. Placement of long-term epidural catheters to treat
> RSD/CRPS still occurs in practice. Perhaps this is because
> anesthesiologists are more familiar with the epidural catheter
> technique than with the selective sympathetic block technique. The
> long-term epidural catheter approach is more expensive and patients
> are placed at more risk for certain rare life-threatening
> complications, e.g. infection (epidural abscess). Often a short (2-5
> days) hospitalization will be necessary to determine the clinically
> most appropriate dose of the epidural agent for constant infusion.
> Dislodgment of the epidural catheter is a relatively common problem.
> The use of a lumbar sympathetic catheter may provide a more specific
> sympathetic block than an epidural catheter, but the lumbar
> sympathetic catheter is more likely to become dislodged during
> exercise. There is a place for the use of epidural and lumbar
> sympathetic catheters in the treatment of RSD/CRPS but the physician
> should justify these techniques on a case by case basis.
>
> Another technique used to carry out a sympathetic block involves the
> intravenous injection of sympathetic blocking agents (e.g.
> guanethidine, bretylium and clonidine) into an extremity and limiting
> spread of the agent to the entire body by applying a tourniquet to the
> extremity. This method requires placing an IV in the painful extremity
> and may be technically extremely difficult due to severe swelling
> (edema) of the extremity. The patient may not be able to confirm that
> they actually received a sympathetic block because the \"cue\", a
> warming sensation in the extremity, may not be felt. Furthermore,
> there is no evidence that this technique is more effective than the
> usual sympathetic blocks for the diagnosis and treatment of RSD/CRPS.
> The IV tourniquet technique using a sympathetic blocking agent may be
> considered as an option for patients who must take blood thinners
> (anticoagulants) where a SGB or a LSB may cause major bleeding.

6.  **Spinal Cord Stimulation (SCS)**:

> Spinal cord stimulation (SCS) uses low intensity, electrical impulses
> to trigger selected nerve fibers along the spinal cord (dorsal
> columns), which are believed to stop pain messages from being
> transferred to the brain. SCS replaces the area of intense pain with a
> more pleasant tingling sensation called paresthesia. The tingling
> sensation will remain relatively constant and should not hurt. There
> is some experimental evidence that SCS may enhance the flow of blood
> to the affected extremity by blocking the sympathetic nervous system.
>
> A temporary trial, with a temporary electrode, should be performed
> first before implanting permanent electrode(s). Given that SCS is a
> relatively invasive, costly procedure and given that RSD/CRPS patients
> are often desperate and frustrated, a baseline psychosocial evaluation
> that addresses pain management issues should be considered. Although
> rare, spinal infection and paralysis are potential complications. The
> ability to insert the electrode through a small needle has reduced the
> risk of the procedure and has facilitated the trial with a temporary
> electrode.
>
> Treating RSD/CRPS with SCS poses unusual clinical and technical
> problems. RSD/CRPS tends to be an unpredictable disease from a
> technical standpoint. The need to focus SCS on the most painful region
> must be kept in mind, which is more difficult in RSD/CRPS, because the
> location of the worst pain may change. Furthermore, the pain from
> RSD/CRPS may spread to distant parts of the body, requiring multiple
> successive implanted stimulators to cover the largest possible area.
> Therefore, even when RSD/CRPS is limited to one extremity, it is wise
> to widen stimulation to zones to which the pain might spread.
>
> Because of the risks and high costs of spinal cord stimulation, the
> treatment is reserved for severely disabled patients. A recent
> well-controlled study shows that with careful selection of patients
> and successful test stimulation, SCS is safe, reduces pain, and
> improves the health-related quality of life in patients with severe
> RSD/CRPS.
>
> [The External Battery System Versus the Internal Battery System for
> Spinal Cord Stimulation]{.underline}
>
> In order to make an informed choice about SCS, the patient and
> physician should consider the pertinent differences between the
> internal and external battery systems (refer to the original guideline
> document for details on comparisons between internal and external
> battery systems for spinal cord stimulation).
>
> [Radiofrequency (RF) vs. Implantable Pulse Generator (IPG)
> Neurostimulation Systems]{.underline}
>
> An informed choice of SCS system by the patient and physician should
> be made after considering the patient´s current pain patterns and the
> possibility of progression of the RSD and the pertinent differences
> between RF and IPG systems (refer to the original guideline document
> for further discussion).

7.  **Morphine Pump**:

> It is well-recognized that a single injection of morphine into the
> spinal fluid (within the intrathecal space) produces a selective
> pain-blocking effect on the spinal cord. This selective effect on the
> spinal cord spares the patient from many of the serious side effects
> caused by morphine when it is given orally (e.g. sedation). Soon after
> this discovery, enthusiasm developed to implant permanent morphine
> pumps to treat non-cancer chronic pain, especially after Medicare
> began to approve this surgical procedure for reimbursement. The
> implantation of a morphine pump is a relatively invasive and expensive
> treatment modality. Despite almost 20 years of testing, no scientific
> evidence has emerged that long-term use of the morphine pump offers an
> advantage over oral morphine for treating various chronic pain
> syndromes, including RSD/CRPS. In fact, many patients with the
> implanted morphine pump take oral morphine at the same time. The same
> complications sometimes associated with oral morphine use are also
> found with the morphine pump, such as development of drug tolerance,
> nausea, constipation, weight gain, decreased sex appetite (libido),
> swollen legs (edema), and increased sweating. In addition, malfunction
> of the pump system (dislodgement of the catheter) can be a significant
> problem.
>
> A recent study suggests that with careful selection of patients, the
> implantation of a pump for the spinal infusion of baclofen may be a
> valuable means for treating a certain type of muscle cramp called
> dystonia in patients with severe RSD/CRPS.

8.  **How to Determine the Effectiveness of Treatments for RSD/CRPS**:

> Refer to the original guideline document for details on the natural
> history of RSD/CRPS, placebo or nonspecific effects of treatment, and
> specific effects of treatment.

9.  **RSD/CRPS in Children**:

> A 30-minute video on RSD/CRPS in children was peer-reviewed by an
> international panel of experts. (Refer to the original guideline
> document for further details on the video.)

#### CLINICAL ALGORITHM(S)

The original guideline document contains an algorithm for a treatment
protocol designed to rehabilitate patients with reflex sympathetic
dystrophy/complex regional pain syndrome (RSD/CRPS) in the shortest
possible time.

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of supporting evidence is not specifically stated for each
recommendation.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

-   Appropriate, timely diagnosis and treatment of reflex sympathetic
    dystrophy/complex regional pain syndrome (RSD/CRPS)

-   If diagnosed early, physicians can use mobilization of the affected
    extremity (physical therapy) and sympathetic nerve blocks to cure or
    mitigate the disease. If untreated, RSD/CRPS can become extremely
    expensive due to permanent deformities and chronic pain.

-   Minimization of pain

-   Improved quality of life

#### POTENTIAL HARMS

**Oral Opioid Use**

There is potential for abuse and/or life-threatening overdoses with the
use of oral opioids.

**Sympathetic Block**

Complications from sympathetic blockade are extremely rare. However, it
is always possible for the local anesthetic to be inadvertently injected
into a blood vessel or into the spinal fluid. If this should happen, the
patient may temporarily become weak and lose consciousness.

**Sympathectomy**

Post-sympathectomy pain (neuralgia) is a potential complication of all
types of sympathectomy. The post-sympathectomy pain usually resolves on
its own or with 1 to 3 sympathetic blocks.

**Epidural Blocks**

The infusion of local anesthetic through the epidural catheter may cause
temporary weakness in the legs, making walking dangerous. Dislodgment of
the epidural catheter is a relatively common problem. The long-term
epidural catheter approach is more expensive and patients are placed at
more risk for certain rare life-threatening complications, such as an
infection (epidural abscess).

**Morphine Pump**

Complications sometimes associated with oral morphine use are also found
with the morphine pump, such as development of drug tolerance, nausea,
constipation, weight gain, decreased sex appetite (libido), swollen legs
(edema), and increased sweating. In addition, malfunction of the pump
system (dislodgment of the catheter) can be a significant problem.

**Spinal Cord Stimulation**

Although rare, spinal infection and paralysis are potential
complications.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Clinical Algorithm\
Resources

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Living with Illness

#### IOM DOMAIN

Effectiveness\
Patient-centeredness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

International Research Foundation for RSD/CRPS. Reflex sympathetic
dystrophy/complex regional pain syndrome. 3rd ed. Tampa (FL):
International Research Foundation for RSD/CRPS; 2003 Jan 1. 48 p. \[66
references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2003 Jan 1

#### GUIDELINE DEVELOPER(S)

International Research Foundation for RSD/CRPS - Private Nonprofit
Research Organization

#### SOURCE(S) OF FUNDING

International Research Foundation for RSD/CRPS

#### GUIDELINE COMMITTEE

Scientific Advisory Committee

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Primary Authors*: Anthony Kirkpatrick, M.D., Ph.D., *Chair*; Charles
Berde, M.D., Ph.D.; Timothy Lubenow, M.D.; Wen-hsien Wu, M.D., M.S.;
Robert Jay Schwartzman, M.D.; Srinivasa Raja, M.D.; Gustavo C. Roman,
M.D.; Sabine Kost-Byerly, M.D.; Adam L. Seidner, M.D., M.P.H; Peter
Wilson, M.D.; Dr-med. Ralph-Thomas Kiefer; Dr. Peter Rohr; Stephen M.
Butler, M.D.; Takashi Mashimo, M.D., D.M.Sc.; Edward Covington, M.D.

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: Available from the [International Research Foundation
for Reflex Sympathetic Dystrophy Syndrome/Complex Regional Pain Syndrome
(RSD/CRPS) Web
site](http://rsdfoundation.usf.edu/en/en_clinical_practice_guidelines.html).

Print copies: Available from the International Research Foundation for
RSD/CRPS, c/o Anthony F. Kirkpatrick, M.D., Ph.D., University of South
Florida Medical Clinics, 12901 Bruce B. Downs Blvd., MDC 59, Tampa, FL
33612-4799; Phone: (813) 907-2312; Email: <info@rsdfoundation.org>.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following educational videos are available:

-   Reflex Sympathetic Dystrophy in Children - Free 43-Minute Video.
    Available on VHS from the [International Research Foundation for
    RSD/CRPS Web
    site](http://rsdfoundation.usf.edu/en/en_children.html). An extended
    2-hour version is also available for purchase on DVD or VHS from the
    [International Research Foundation for RSD/CRPS Web
    site](http://rsdfoundation.usf.edu/en/video.html)

-   Use of Opioids (Narcotics) to Treat Reflex Sympathetic Dystrophy
    (RSD/CRPS) in Adults and Children - Free 2-Hour Video. Available on
    VHS from the [International Research Foundation for RSD/CRPS Web
    site](http://rsdfoundation.usf.edu/en/en_use_of_opioids.html).

#### PATIENT RESOURCES

None available

#### NGC STATUS

This summary was prepared by ECRI on January 8, 2004. The information
was verified by the guideline developer on January 29, 2004. This
summary was updated by ECRI on January 12, 2005 following the release of
a public health advisory from the U.S. Food and Drug Administration
regarding the use of some non-steroidal anti-inflammatory drug products.
This summary was updated on April 15, 2005 following the withdrawal of
Bextra (valdecoxib) from the market and the release of heightened
warnings for Celebrex (celecoxib) and other nonselective nonsteroidal
anti-inflammatory drugs (NSAIDs). This summary was updated by ECRI on
June 16, 2005, following the U.S. Food and Drug Administration advisory
on COX-2 selective and non-selective non-steroidal anti-inflammatory
drugs (NSAIDs). This summary was updated by ECRI Institute on November
6, 2007, following the U.S. Food and Drug Administration advisory on
Antidepressant drugs. This summary was updated by ECRI Institute on
January 10, 2008, following the U.S. Food and Drug Administration
advisory on Carbamazepine.

#### COPYRIGHT STATEMENT

This NGC summary is based on the original guideline, which is subject to
the guideline developer\'s copyright restrictions.

### DISCLAIMER

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