Complex Regional Pain

Syndrome

A.Sridhar, MPT

1864 - Colonel Weir Mitchell, MD

“severely painful dystrophic syndrome following ballistic

injuries” in Civil War soldiers: Causalgia

History

Paul Sudeck

Suggested that the signs and symptoms of RSD may be caused by an exaggerated inflammatory response to injury or operation of an extremity.

Sudeck’s Atrophy: Bone

loss associated with RSD

Rene Leriche

Sympathetic

nervous system

dysfunction as a

cause of pain.

Therapeutic surgical

sympathectomy

John Bonica

The syndrome much as we

know it today

Promoted the term RSD

Described 3 stages

CRPS History

CRPS (complex regional pain syndrome) initially considered in early 1800s by Claude Bernard et al.

During the Civil War it was seen that soldiers who suffered from low-velocity, high-mass missile injuries developed a neuropathic pain that was termed "causalgia" by Silas Weir-Mitchell.

In 1940s the term of reflex sympathetic dystrophy came into use relating the belief of an abnormal efferent reflex from the sympathetic nervous system to bodily injury.

Since that time much study and frustration has come from this relatively rare condition of which the pathophysiology is still not fully understood.

Basics

Often seen after injury to a limb or related to

some inciting event.

The patient complains of and can manifest

skin color/ temperature/ appearance changes

in the affected limb.

Pain often excruciating – burning, tingling,

electric-like, etc. are often symptoms that

patients feel. The pain is often out of

proportion to stimulus or the event.

Physical Appearance

Diagnostic Criteria

IASP (International Assoc for the Study of Pain) diagnostic criteria include 4 subjective and/ or objective findings:

1. The presence of an initiating event or a cause of immobilization – peripheral injury or central (stroke, etc)*. (Injury)

2. Continuing pain, allodynia, or hyperalgesia in which the pain is disproportiate to inciting event. (Sensory)

3. Evidence of edema, changes in skin blood flow, or abnormal sudomotor activity in region of pain. (Vasomotor)

4. Diagnosis is excluded by the existence of other conditions that would otherwise account for the degree of pain/ dysfunction.

One symptom from each category (except #1 as 5% of pts lack known event) and at least one sign from 2 categories must be evident to diagnose CRPS, at least by research criteria.

*Not always present or identifiable.

CRPS More Widely Diagnosed

Quickly becoming more recognized, and

possibly overly diagnosed for several

reasons:

The standardization of diagnosis.

The "discovery" by personal injury lawyers

who use it as a tool for settlements.

New treatments and research for chronic

pain.

Patient self-research and the internet.

Diagnostic Si/Sx

The most common pain finding is a "burning" and "stinging” sensations that occur spontaneously. This is seen in as many as 87% of cases.

69% of patients report hyperesthesia with light touch (such as clothing laying on the skin or even draughts of blowing wind).

Vasomotor dysfunction is manifested by asymmetrical edema in affected limb, skin color and temperature changes, abnormal sweating (either hyperhidrosis or anhidrosis), and skin/ nail changes.

Patients may also complain of muscle jerking, myoclonus, or rigidity in affected limb including contractures of hands and feet.

RSD RENAMED

Reflex Sympathetic Dystrophy renamed as

Complex Regional Pain Syndrome in 1995

CRPS type 1 is RSD

CRPS type 2 is Causalgia (nerve lesion)

Current evidence suggests CRPS 1 is minute

nerve injury in C fibers (Oaklander AL MD

PhD et al Pain 2006)

CRPS II

The majority of patients seen are diagnosed with

CRPS type I – or reflex sympathetic dystrophy

(RSD). This is the less painful, debilitating of the two.

CRPS II (causalgia) is related to a known injury to a

specific major nerve with neuropathic pain frequently

following along the distribution of that nerve alone,

though not always.

While the diagnostic criteria of the IASP may apply,

these patients may also demonstrate hypoesthesia in

the nerve distribution associated with electrical shocks

and extreme allodynia. This is rarer than CRPS I and

the patients are often more debilitated.

IASP Nomenclature

CRPS I = Reflex Sympathetic Dystrophy

CRPS II = Causalgia

The only difference between the two is the inciting event: minor trauma (I) versus major peripheral nerve injury (II).

���Algodystrophy, Sudeck’s atrophy, sympathetically maintained pain, shoulder/hand syndrome, transient osteoporosis, and acute atrophy of bone.

Clinical Presentation

Precipitating event:

CRPS I

Minor trauma, contusion, sprain or strain

Fracture (especially colles fx)

Post surgical

Immobilization

Less frequently: CVA, spinal cord injury

CRPS II

Documented peripheral nerve injury and concordant

focal deficits (but the signs and symptoms of CRPS are

not limited to the same distribution as the affected nerve.)

Clinical Presentation

Time Course

Three stages:

Stage 1 (acute)

Stage 2 (dystrophic)

Stage 3 (atrophic)

CRPS Stage 1 (Acute) Immediately after injury--3 months MOST LIKELY TO BE REVERSED AND CURED

SKIN: Red, warm, swollen, dry, inflamed. Later color may

change to mottled and colder with marked hyperhydrosis. Changes back and forth especially with painful use.

DISTRIBUTION: Pain is not compatible with a single peripheral nerve, trunk, or root lesion.

SYMPATHETIC:

VASOMOTOR: Disturbances occur with variable intensity, producing altered color and temperature. Hyperemic Mottled

SUDOMOTOR: Dry Hyperhydrosis

MOTOR: Decreased ROM, weakness

X-RAYS: Normal

BONE SCAN: Increased uptake

Stage 1 (Acute)

Stage 1 (Acute)

QuickTime™ and a decompressor

are needed to see this picture.

CRPS Stage 2 (Dystrophic)

Pain remains SEVERE. Same characteristics

as Stage 1.

CRPS Stage 2 (Dystrophic)

6 weeks--1 year SKIN: Cool, moist, tight/shiny, swelling,

coarse/sparse hair, brittle nails, discolored, edema

SYMPATHETIC: VASOMOTOR: Mottled/cyanotic

SUDOMOTOR: Hyperhydrosis

MOTOR: Weakness, decreased ROM

BONE SCAN: No longer helpful.

Stage 2 (Dystrophic)

Stage 2 (Dystrophic)

QuickTime™ and a decompressor

are needed to see this picture.

Stage 2 (Dystrophic)

CRPS Stage 3 (atrophic)

6 months--Forever? Pain is somewhat decreased (but still debilitating)

less at rest, worse with passive motion

Changes are irreversible, poor outcomes, permanent disability

SKIN: Atrophy, “waxy”, very thin, ulcerations, brittle nails

SYMPATHETIC: VASOMOTOR: Cold, intermittently cyanotic/mottled

MOTOR: Decreased ROM, weakness, muscle & tendon atrophy, contractures, dystonia, tremor. Nonfunctional limb.

X-RAYS: Diffuse patchy osteoporosis (Sudeck’s Atrophy)

Atrophic Stage 3

Severe Mottling

Atrophic Stage 3

Contractures

Skin Ulceration

Migratory/progressive

CRPS I Diagnostic Criteria - IASP

1. The presence of an initiating noxious event or a cause of immobilization.

2. Continuing pain, allodynia or hyperalgesia with which the pain is disproportionate to the inciting event.

3. Evidence at some time of edema, changes in skin blood flow or abnormal sudomotor activity in the painful region.

4. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. note: Criteria 2,3 and 4 are necessary for a

diagnosis of complex regional pain syndrome. International Association for the Study of Pain: Diagnostic Criteria for Complex Regional Pain

Syndrome with 1997 ICD Codes

Merskey H, Bodguk N, eds. Classification of chronic pain, descriptions of chronic pain syndromes and definitions of pain terms. Id ed. Seattle: IASP Press, 1994:40-3.

CRPS II (Causalgia) - IASP

1. The presence of continuing pain, allodynia or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve.

2. Evidence at some time of edema, changes in skin blood flow or abnormal sudomotor activity in the region of the pain.

3. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction.

note: All three criteria must be satisfied. International Association for the Study of Pain: Diagnostic Criteria for Complex Regional

Pain Syndrome with 1997 ICD Codes

Merskey H, Bodguk N, eds. Classification of chronic pain, descriptions of chronic pain syndromes and definitions of pain terms. Id ed. Seattle: IASP Press, 1994:40-3.

Mirror Therapy The brain wants congruence between motor intention,

peripheral sensory input and visual input. Mirror

therapy “restores” this relationship.

Treatment

Treatment usually consists of several objectives:

Functional restoration of affected limb - often should be considered first before other treatments

Sympathetic and/or motor blocks

Cognitive behavioral techniques

Psychotherapy

Pharmacotherapy

Occupational and physical therapy

Tx – Functional Restoration

Functional restoration involves steady progression from gentle movements to gentle, weight bearing movement. Results in more active load bearing with expected gradual desensitization and increased functionality of limb.

Examples include moving from silk stimulation to other cloths and textures, the scrub and carry technique, and contrast baths that widen the temperature range that the patient can tolerate.

If limitations occur then addition of blocks, pharmacotherapy, etc. can help increase the patient's tolerance and improvement.

Tx - Psychotherapy

Psychotherapy is critical to improvement in patients with CRPS. There is a high incidence of depression and anxiety noted in many CRPS patients. Unknown at this time if antecedent psychological factors prior to injury are common.

CRPS patients also develop a type of PTSD termed "kinesophobia" or fear of movement related to prior pain or initial injury. The patient develops "negative reinforcements" through fear of initial movements that caused the injury of prior movements that resulted in extreme pain in the past. Fear of movement often results in contractures and reduced functionality.

Cognitive behavioral therapy is the most beneficial psychotherapy to help patients with these concerns, though other interventions including family therapy are also beneficial.

Tx - Pharmacotherapy

Pharmacotherapy is often on a trial and error basis and

is very patient specific. Drugs are considered based

on neuropathic pain treatments and then used for

CRPS and have yet to be shown effective in RCTs.

- initial drug(s) to consider include gabapentin and

pregabalin (both used for neuropathic pain) and are

approved for these conditions

- TCAs often used for patients with sleep

disturbances, but are hindered by their numerous

side effects and drug interactions; not currently

approved for pain treatment

Tx - Pharmacotherapy

- SSRIs and SNRIs like fluoxetine and duloxetine,

resp, are often used with the latter being approved for

neuropathic pain conditions.

- Opioids should be avoided as much as possible as

their effectiveness is not well proved and

dependence/ addiction are serious concerns in CRPS

patients.

- Intrathecal baclofen, IV steroids, IVIG, and

anticonvulsant medications are all treatments that

have been considered and used with variable

success, though studies are still lacking.

Tx – Interventional Approaches

Considering the nature of the sympathetic involvement in the patient, blocking the sympathetic nervous system seems inherent towards improvement.

Cervical and lumbar sympathetic blocks are frequently performed as outpatient procedures with patients receiving a few weeks or months of relief. Often done to help with movement therapies.

Beir blocks with local anesthetics, guanethidine, or other neurolytic agents have been performed with varying success.

SCS, pump implantation, and thermocoagulation have also been used to treat patients with CRPS with variable success rates.

Despite the belief that blocking the sympathetic

nervous system will result in reduced pain,

many patients do not gain substantial benefit

from these procedures for any substantial

length of time and the procedures themselves

may actually exacerbate the patient's pain.

CRPS Prognosis

Overall the prognosis for patients with CRPS is

relatively low. Effective treatments are very patient

specific and patient satisfaction, mental state,

willingness to be involved in their treatment all

contribute to their pain reduction.

Many patients report extreme lack of satisfaction with

their pain control and are usually disabled.

More research is required into chronic pain and

developing better methods to treat chronic autonomic

dysfunctional pain.

References

Harden RN, Complex Regional Pain Syndrome, British Journal of Anesthesia, 2001, 87, pp. 99-106.

Schott GD, Complex? Regional? Pain? Syndrome? Practical Neurology. 2007, 7, pp. 145-157.

Forouzanfar T, et al. Treatment of Complex Regional Pain Syndrome Type I. European Journal of Pain. Vol 6, issue 2. Apr 2002. pp. 105-122.

Thank U