Clinical
Practice Guidelines - Third Edition
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Reflex Sympathetic Dystrophy
/ Complex Regional Pain Syndrome (RSD/CRPS)
Anthony F. Kirkpatrick,
M.D., Ph.D.
Editor
Department of Internal Medicine
University of South Florida
Tampa, Florida
International Research Foundation for RSD/CRPS
Last update: January 1, 2003
Preface
to the Third Edition
The first "International Update
on RSD/CRPS" was held at the University of South Florida
on February 1 and 2, 2002. Thanks to the organizational
efforts of Dr. Srinivasa Raja at John Hopkins University,
the symposium presented an outstanding faculty of international
experts on RSD/CRPS. The symposium formed a foundation for
writing the third edition of the Clinical Practice Guidelines.
The Clinical Practice Guidelines have become a standard
for managing RSD/CRPS. As with prior editions, my intention
has been to focus on providing health care providers and
patients unbiased information that will enable patients
to make informed choices about their treatment. As noted
in a recent issue of the JOURNAL OF THE AMERICAN MEDICAL
ASSOCIATION, the information had to be carefully selected
and organized. * One of the major reasons why physicians
do not follow clinical practice guidelines is that most
are not concise enough for both physicians and patients
to read, understand and follow. Guidelines need to be presented
in a concise manner saying in effect, "These measures are
recognized as essential, these others should be considered,
and this group has been found ineffective or contraindicated."
The most notable addition to third edition is a new section
titled "RSD in Children." During the course of preparing
this section, it was necessary to consult extensively with
experts in the field of pediatric pain management. These
persons included Dr. Sabine Kost-Byerly at Johns Hopkins
University, Dr. Robert Wilder at Mayo Clinic and Dr. Robert
Schwartzman at Allegheny University of Health Sciences.
Their efforts were a significant factor in completing the
third edition, for which I am most appreciative.
The Scientific Advisory Committee has reviewed and commented
on the third edition of the Guidelines. These persons were
chosen because of their acknowledged expertise worldwide
in the area of RSD/CRPS. The members of the Committee are
listed on the Foundation's web site. If you would like to
see a list of the Committee members, please click
here.
Last, but not least, I am grateful to the patients and health
providers throughout the world who took the time to provide
feedback on the second edition. The Foundation welcomes
feedback on the clinical practice guidelines at "Contact
Us".
Anthony F. Kirkpatrick, M.D., Ph.D.
Chair, Scientific Advisory Committee
Director of Research
January 1, 2003
* Veatch RM, Montgomery
AA, Dahlberg, K, Cabana MD, Rand CS, Powe NR, Wu AW, Abboud
PC, Wilson MH. Reasons physicians do not follow clinical
practice guidelines. JAMA 2000; 283: 1685-1686.
Contents:
Diagnosis
Treatment
Photo Gallery
Opioid Treatment Protocol
External Battery System
Vs Internal Battery System
How to Determine the Effectiveness
of Treatments
RSD In Children ---
NEW!
References
Glossary of Medical Terms
REFLEX SYMPATHETIC DYSTROPHY
SYNDROME
(RSD/CRPS)
Reflex Sympathetic Dystrophy Syndrome
(RSD) is also known as Complex Regional Pain Syndrome (CRPS).
RSD/CRPS is a multi-symptom, multi-system, syndrome usually
affecting one or more extremities, but may affect virtually
any part of the body. Although it was clearly described
125 years ago by Drs. Mitchell, Moorehouse and Keen, RSD/CRPS
remains poorly understood and is often unrecognized.
The best way to describe RSD/CRPS is in terms of an injury
to a nerve or soft tissue (e.g. broken bone) that does not
follow the normal healing path. The development of RSD/CRPS
does not appear to depend on the magnitude of the injury
(e.g. a sliver in the finger can trigger the disease). In
fact, the injury may be so slight that the patient may not
recall ever having received an injury. For reasons we do
not understand, the sympathetic nervous system seems to
assume an abnormal function after an injury. There is no
single laboratory test to diagnose RSD/CRPS. Therefore,
the physician must assess and document both subjective complaints
(medical history) and, if present, objective findings (physical
examination), in order to support the diagnosis. There is
a natural tendency to rush to the diagnosis of RSD/CRPS
with minimal objective findings because early diagnosis
is critical. If undiagnosed and untreated, RSD/CRPS can
spread to all extremities, making the rehabilitation process
a much more difficult one. 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.
There are no studies showing that RSD/CRPS affects the patient's
life span. The potential exists for long-term financial
consequences. At an advanced state of the illness, patients
may have significant psychosocial and psychiatric problems,
they may have dependency on narcotics and may be completely
incapacitated by the disease. The treatment of patients
with advanced RSD is a challenging and time-consuming task.
Diagnosis
If one can demonstrate major nerve
damage associated with the development of RSD/CRPS symptoms,
the condition is called complex regional pain syndrome (CRPS)
type II or causalgia. Generally, causalgia provides more
objective evidence of disease due to neurological changes
(numbness and weakness).
The terms complex regional pain syndrome (CRPS) type I and
type II have been used since 1995, when the International
Association for the Study of Pain (IASP) felt the respective
names reflex sympathetic dystrophy and causalgia were inadequate
to represent the full spectrum of signs and symptoms. 1-8
The term "Complex" was added to convey the reality
that RSD and causalgia express varied signs and symptoms.
Many publications, particularly older ones, still use the
names RSD and causalgia. To facilitate communication and
understanding the designation RSD/CRPS is generally used
throughout these practice guidelines. The principles applicable
to the diagnosis and management of RSD are similar to those
principles applicable to the diagnosis and management of
causalgia.
To make the early diagnosis of RSD/CRPS, the practitioner
must recognize that some features/manifestations of RSD/CRPS
are more characteristic of the syndrome than others, and
that the clinical diagnosis is established by piecing each
bit of the puzzle together until a clear picture of the
disorder emerges. Often the physician needs to rule out
other potentially life-threatening disorders that may have
clinical features similar to RSD/CRPS, e.g. a blood clot
in a leg vein or a breast tumor spreading to lymph glands
can cause a swollen, painful extremity. Indeed, RSD/CRPS
may be a component part of another disease, (e.g. a herniated
disc of the spine, carpal tunnel syndrome of the hand, heart
attack). Thus, treating RSD/CRPS will often be directed
to treating clinical features rather than a well defined
disease. When RSD/CRPS spreads the diagnosis can be more
complicated. For example, if it spreads to the opposite
limb, it may be more difficult to establish a diagnosis
because there is no normal side (control) to compare for
objective findings. On the other hand, the spreading of
RSD/CRPS symptoms may actually facilitate the diagnosis
of RSD/CRPS because spreading symptoms is a characteristic
of the disorder. (See below).
Importance
of Objective Findings
Many patients who develop RSD/CRPS
as the result of an injury do so in the context of legal
liability. Some patients can be expected to defend their
rights in courts of law. It is not uncommon for the defendant
to accuse the patient of faking their condition, especially
if there are no objective findings for RSD/CRPS documented
on the medical record. Therefore, the evaluating physician
must assess more than just subjective complaints (medical
history). The physician must aggressively
seek and document objective findings. For example, about
80% of RSD/CRPS cases have differences in temperature in
opposite sides that may be either colder or warmer. These
temperature changes may be associated with changes in skin
color. Furthermore, the temperature differences are not
static. The skin temperature can undergo dynamic changes
in a relatively short period of time (within minutes) depending
critically on room temperature, local temperature of the
skin and emotional stress. In some cases, the differences
in temperatures may fluctuate spontaneously even without
any apparent provocation. 4
Thus, the objective finding of differences in temperature
and color of the skin can be missed by the physician if
only a single physical examination is made. A useful and
relatively inexpensive instrument to have available at the
time of the physical examination is a portable infrared
thermometer to measure differences in skin temperature.
Changes in skin temperature and color are only two examples
of several objective findings that should be sought in the
patients with RSD/CRPS.
Making
the Diagnosis of RSD/CRPS
The diagnosis of 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
- 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).
- 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. 9
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.
- 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.
- 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 phenomena 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.7
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 seemly 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. 10
Unfortunately, physicians sometimes fail to follow these
diagnostic guidelines and carelessly report a "psychogenic"
disorder. 11
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.
- 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.
Maleki et. al. recently described three patterns of
spreading symptoms in RSD/CRPS: 12
- A "continuity type"
of spread where the symptoms spread upward from
the initial site, e.g. from the hand to the shoulder.
- A "mirror-image type"
where the spread was to the opposite limb.
- 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.
- 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 (technecium 99m)
in bones after intravenous injection.
- 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
- Onset of severe, pain limited
to the site of injury
- Increased sensitivity of skin
to touch and light pressure (hyperasthesia).
- Localized swelling
- Muscle cramps
- Stiffness and limited mobility
- 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.
- Increased sweating (hyperhydrosis).
- In mild cases this stage lasts
a few weeks, then subsides spontaneously or responds
rapidly to treatment.
STAGE II
- Pain becomes even more severe
and more diffuse
- Swelling tends to spread and
it may change from a soft to hard (brawny) type
- Hair may become coarse then scant,
nails may grow faster then grow slower and become
brittle, cracked and heavily grooved
- Spotty wasting of bone (osteoporosis)
occurs early but may become severe and diffuse
- Muscle wasting begins
STAGE III
- Marked wasting of tissue (atrophic)
eventually become irreversible.
- For many patients the pain becomes
intractable and may involve the entire limb.
- A small percentage of patients
have developed generalized RSD affecting the entire
body. 6
Photo
Gallery
Dr. Robert J. Schwartzman, whose
name is synonymous with RSD/CRPS, has contributed a photo
gallery to the Clinical Practice Guidelines. His photos
illustrate some of the objective findings that may be observed
in patients with RSD/CRPS. It
should be emphasized that patients with RSD/CRPS may not
present with these objective findings, especially during
the early stages of the disease.
Dr. Schwartzman is Professor and Chairman of the Department
of Neurology at Hahnemann School of Medicine in Philadelphia,
PA, USA. He is a member the Scientific Advisory Committee.
Through his numerous publications, lectures, and research
efforts, he shares his clinical experience by making others
aware of the effects of RSD/CRPS. Dr. Schwartzman has received
several honors and awards, including the Mayo Clinic Neurology
Teaching Award and the Dean's Special Award for Excellence
in Teaching from Hahnemann University of Medicine in both
1998 and 1999.
Pictures
1 - 4
Pictures 5 - 8
Pictures 9 - 12
Pictures 13 -16
Some other names
given to this syndrome:
- Causalgia (Minor or Major)
- Sudeck's Atrophy
- Post Traumatic Dystrophy (Minor
or Major)
- Shoulder Hand Syndrome
- Reflex Neurovascular Dystrophy
Incidence
of RSD/CRPS:
- The exact prevalence of RSD/CRPS
is unknown; however, data from several studies suggest
it is more frequent than commonly believed.
- Both sexes are affected, but
the incidence of the syndrome is higher in women,
especially in the pediatric population.
Etiology:
- A number of precipitating factors
have been associated with RSD/CRPS including:
- Trauma (often minor) ranks
as the leading provocative event
- Ischemic heart disease and
myocardial infarction
- Cervical spine or spinal cord
disorders
- Cerebral lesions
- Infections
- Surgery
- Repetitive motion disorder
or cumulative trauma, causing conditions such as
carpal tunnel.
However, in some patients a definite
precipitating event can not be identified.
Cause of
RSD/CRPS
Current research suggest that the
mechanism by which an injury triggers RSD/CRPS is unclear.
Figure 1 is intended to give the reader a simplified view
of how an injury might lead to the symptoms of RSD/CRPS.
Figure 1
Activation of the sympathetic nervous
system following an injury is part of a fright-flight response
to an emergency situation. This response is very important
for survival. For example, firing of sympathetic nerves
causes blood vessels in the skin to contract, forcing blood
deep into muscle and enabling the victim to use his muscle
to get up after an acute injury and escape from further
danger. Also the decreased supply of blood to the skin reduces
blood loss through superficial injuries that may occur on
the surface of the body. Ordinarily, the sympathetic nervous
system shuts down within minutes to hours after an injury.
For reasons we do not understand, individuals who go on
to develop RSD/CRPS, the sympathetic nervous system appears
to assume an abnormal function. Theoretically, this sympathetic
activity at the site of injury could cause an inflammatory
response causing the blood vessels to spasm leading to more
swelling and pain. (See B, C, and D in Figure 1) The events
could lead to more pain which triggers another response,
establishing a vicious cycle of pain.
For a video
animation depicting how an injury might trigger RSD/CRPS:
CLICK HERE
Failure to mobilize the affected
region of the body might be a critical factor in prolonging
the recovery from RSD/CRPS. However, disuse of muscle does
not appear to be responsible for the genesis of RSD/CRPS.
For example, investigators in Sweden have reported highly
unusual data that makes it difficult to explain the genesis
of RSD/CRPS simply on the basis of disuse. They carried
out a pathological analysis of peripheral nerve and muscle
taken from amputated legs of eight patients with RSD/CRPS.
13 In
all patients, amputation was performed because the painful
(hyperpathic) limb was useless or subject to recurrent infections.
Skeletal muscle specimens were abnormal in all cases, but
myelinated nerve fibers were normal, and in half the patients
there was a loss of unmyelinated fibers. These findings
suggest a microangiopathy in the affected limbs. Thus, patients
with RSD/CRPS have tissues that are truly abnormal, and
abnormal in ways that cannot be readily explained by disuse
and/or psychological factors.
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. Thermogram
- A thermogram is a noninvasive means of measuring heat
emission from the body surface using a special infrared
video camera. It is one of the most widely used tests
in suspected cases of RSD/CRPS. As noted, detecting an
abnormal change in skin temperature in RSD/CRPS depends
on many factors. A normal thermogram does not necessarily
mean the patient does not have RSD/CRPS. An abnormal thermogram
may be helpful when there are minimal objective findings
for RSD/CRPS documented in the medical record. Furthermore,
certain patterns of abnormal heat emission from the body
(e.g. circumferential versus dermatomal changes) are more
indicative of the existence of RSD/CRPS than others. The
thermogram should be performed at a reputable medical
facility. The quality of the test may vary among providers.
Three phase radionuclide bone
scanning – the role of the 3 phase bone scan
in the diagnosis of RSD/CRPS has been debated and is controversial.
Sympathetic blocks
- See below under "sympathetic blocks".
X-rays, EMG, Nerve Conduction
Studies, CAT scan and MRI studies – All of
these tests may be normal in RSD/CRPS. These studies may
help to identify other possible causes of pain; for example,
RSD/CRPS plus a carpal tunnel syndrome.
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,
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 reinjury.
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). 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 effect the
doctor-patient relationship.
Figure
2
1. Establish
a written treatment protocol.
Figure
2 illustrates a typical treatment protocol that was designed
to rehabilitate the patient in the shortest possible time.
Initiate the safest, simplest, and most cost-effective therapies
first. 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:
- Procedures (e.g. nerve blocks)
- Medications
- Physical/occupational therapy
- Psychosocial issues
- New laboratory tests or consults
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. 14,15
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.
For a free 4-minute
video on the emotional consequences of RSD/CRPS in children:
CLICK HERE
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:
Nonsteroidal anti-inflammatory
agents (e.g. aspirin, ibuprofen, naproxen, indomethacin,
etc).
For constant pain
not caused by inflammation:
Agents acting on the central
nervous system by an atypical mechanism (e.g. tramadol)
For constant pain
or spontaneous (paroxysmal) jabs and sleep disturbances;
Anti-depressants (e.g.
amitriptyline, doxepin, nortriptyline, trazodone, etc)
1,6
Oral lidocaine (mexilitine - some what experimental)
For spontaneous
(paroxysmal) jabs
Anti-convulsants (e.g.
carbamazepine, gabapentin may relieve constant pain
as well) 17-19
For widespread,
severe RSD/CRPS pain, refractory to less aggressive therapies
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." A typical doctor-patient contract
can be found by clicking on the link below. 20
CLICK
HERE FOR OPIOID TREATMENT PROTOCOL
Patients may require immediate and
adequate pain relief. In some cases it may take time to
transpire from the time of the patients 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)
Clonidine Patch. Studies
suggest that clonidine may decrease pain in RSD/CRPS
by inhibiting the sympathetic nervous system. 21,22
A treatment protocol for using the Clonidine Patch to
treat RSD/CRPS can be found in the journal Regional
Anesthesia. 23
For muscle cramps
(spasms and dystonia) which can be very difficult to treat.
Klonopin (clonazepam)
Baclofen
For localized pain
related to nerve injury.
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). 24
4. 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.
5. 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. 25-27
There is evidence that there might be a role for sympathetic
blocks in preventing RSD/CRPS. A retrospective study demonstrated
that the prophylactic use of sympathetic blocks in patients
with a history of RSD/CRPS decreased the occurrence rate
of the disease from 72% to 10% after re-operation on the
affected extremity. 28
If sympathetic blocks are not properly performed and evaluated,
time and money will be wasted, and diagnostic-prognostic
information will be lost. A good sympathetic block should
increase the temperature of the extremity without producing
increased numbness or weakness. The sensation of warmth
tells the patient that they have had a sympathetic block.
If the block causes numbness or weakness, more than just
the sympathetic nerves were blocked and the patient will
get an overestimation of the amount of their pain that
is contributed by their sympathetic nervous system; hence,
the diagnostic and prognostic value of the nerve block
would be lost. The amount of pain relief and improvement
in range of motion and in exercise tolerance should be
noted by the patient and recorded by the physician. This
information about the patient's response to sympathetic
blockade will serve as a prognostic indicator for rehabilitation
following the series of sympathetic blocks and it will
help the patient decide if a permanent block (destruction
of the nerve by sympathectomy) would be appropriate. Also,
the information will aid in directing future medications
in a more rational manner. Some patients will experience
a "booster effect" with each sympathetic block,
i.e. each successive sympathetic block in the series provides
greater and greater pain relief and improvement in exercise
tolerance. The maximum sustained benefit from a series
of sympathetic blocks is usually apparent after a series
of 3-6 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 1-3 sympathetic blocks. THE GOAL IS ALWAYS
TO TREAT BUT DON'T OVER TREAT.
Sympathetic blocks are usually performed by a pain specialist
trained in anesthesia. In experienced hands, these nerve
blocks can be performed with minimal discomfort to the
patient with or without IV sedation. 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. For safety reasons, sympathetic
blocks are always performed under conditions where the
vital signs (blood pressure and breathing) can be monitored
closely. Patients should not eat for 6 hours prior to
a sympathetic block. For further information about safety
in performing nerve blocks refer to the web site for the
Anesthesia Patient Safety Foundation: http://www.apsf.org/
A sympathetic block of the upper extremity is called a
stellate ganglia block (SGB). The SGB is performed by
inserting a small needle along side the windpipe (trachea).
Patients are informed that they may notice a temporary
change in the tone of their voice following the block
because some of the local anesthetic may partially numb
the vocal cords. They are also informed that they should
sip fluids and take small bites of food immediately after
the block. The numbness around the vocal cords temporarily
places the patient at a slight risk of coughing in response
to drinking and eating. The patient may also notice a
temporary drooping of their upper eye lid due to the SGB
(Horner's sign). A sympathetic block of the lower extremity
is called a lumbar sympathetic block (LSB). For patient
comfort and safety, LSBs should be performed with the
aid of a fluoroscope (X-rays). A video of an LSB being
performed can be found on the Web Site for the journal
"ANESTHESIOLOGY": 29
To
view a 3 minute video of a lumbar sympathetic nerve block
being performed.
CLICK
HERE
As noted previously in
the Guidelines, there may be point-tender spots in the
muscles of the affected region due to small muscle spasms
called muscle trigger points (myofascial pain syndrome).
The patient may obtain significant relief of the diffuse
pain due to RSD/CRPS from a sympathetic block but the
pain due to muscle trigger point(s) may persist. Local
injection of local anesthetic into the trigger point region
and/or application of physical therapy techniques after
a sympathetic block may be necessary to provide further
relief of pain.
6. 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. 30-32
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, laproscopic sympathectomy has been developed
for sympathectomy of the upper extremity.33
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. 4,34
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 data35
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.
7. 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.
4,36
- The greater the expectation for
pain relief, the greater will be the placebo effect.
4,36
- 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. 4
- The placebo tends to be less
reproducible with each successive treatment. 4,36
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.
36
8. 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. 4
The phentolamine test is a diagnostic procedure while a
sympathetic block is a diagnostic, prognostic and therapeutic
procedure. 37
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.5
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.38
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.
9. Spinal
Cord Stimulation (SCS):
Figure
3 illustrates another method of pain control that works
well for some patients with chronic intractable pain due
to RSD/CRPS. 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. 39-42
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. 43-45
FIGURE 3
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. 46,47
The
External Battery System
Versus the Internal Battery System for Spinal Cord Stimulation
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. In this section, the relative merits of
the internal and external battery systems for spinal cord
stimulation are discussed.
CLICK
HERE
10.
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). 36
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. 48-50
In addition, malfunction of the pump system (dislodgement
of the catheter) can be a significant problem. 50
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.
51
11.
How to Determine the Effectiveness
of Treatments for RSD/CRPS:
Dr.
Anthony F. Kirkpatrick, Chairman of the Scientific Advisory
Committee, contributed a section to the Clinical Practice
Guidelines that focuses on how patients can determine the
effectiveness of treatments for RSD/CRPS. This section is
based on Dr. Kirkpatrick’s keynote address at RSDSA's Third
National Conference, held October 15-16, 1999, in Atlantic
City, New Jersey.
CLICK
HERE
12.
RSD/CRPS in Children
A 30-minute video on RSD/CRPS in
children was peer-reviewed by an international panel of
experts at the "International Update on RSD/CRPS" held at
the University of South Florida, February 1-2, 2002. As
a result of the comments received by this panel, the video
was expanded to 43 minutes in order to cover a broader range
of issues. During the course of preparing the extended version
of the video, it was necessary to consult extensively with
experts in the field of pediatric pain management. These
persons included Dr. Sabine Kost-Byerly at Johns Hopkins
University, Dr. Robert Wilder at Mayo Clinic and Dr. Robert
Schwartzman at Allegheny University of Health Sciences.
To view the video for free:
CLICK
HERE |
