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INTRODUCTION
Complex regional pain syndrome (CRPS) is adisorder of the extremities.
A diagnosis of CRPS requires the presence of regional pain and sensory changes followinga noxious event.
The pain is more severe than expected fromthe injury which caused it.
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ETIOLOGY The pathogenesis of CRPS is unclear.
It is thought to involve the formation of areflex arc after an inciting event.
The reflex arc follows the routes of the
sympathetic nervous system.
It is modulated by cortical centers whichproduce peripheral vascular changes.
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Introduction
CRPS frequently begins following: An injury Surgery
A vascular event such as a myocardial infarction orstroke
It is characterized by: Pain Swelling/edema Limited range of motion Vasomotor instability Skin changes/abnormal skin color Patchy bone demineralization Temperature changes Atrophy
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Introduction
CRPS is also known as: Reflex Sympathetic Dystrophy (RSD) Algodystrophy
Causalgia Sudecks atrophy Transient osteoporosis Acute atrophy of bone Shoulder-hand syndrome
A consensus development conference in 1999grouped these disorders under the singleheading of complex regional pain syndrome
(CRPS). -Harden RN, Bruehl et al Pain, 1999
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Introduction
Two types of CRPS have beenrecognized:
Type I: No definable nerve lesion is present. Represents about 90% of clinical cases.
Type II: A definable nerve lesion is present. Formerly termed causalgia .
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Etiology
There is decreased, rather than increased,sympathetic outflow to the affected limb.
Autonomic features are thought to be dueto catecholamine hypersensitivity : Cyanosis Mottling
Increased sweating Abnormal growth of hair Diffuse swelling Coldness
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Etiology
A proposed mechanism for the persistent pain &allodynia is the release of inflammatory mediators
and pain producing peptides by peripheral nerves,including: Substance P (Pleger, et al., Ann Neurol 2005) Neuropeptide Y
Calcitonin gene related peptide IL-6, IL-8, IL-1beta Tumor necrosis factor alpha (Munnikes, et.al., Mediators
Inflamm 2005)
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Inciting events
Trauma Stroke & CNS injuries (3%)Crush injuries Peripheral nerve diseaseFractures (25%) Postherpetic neuralgiaSoft tissue injuries (40%) Cervical spine pathologySprains Myocardial infarction (12%)Immobilization in a cast Pleuropulmonary disease
Surgery (CTS, Mortons neuroma) S.C injectionChemical & electrical burns Drugs ( barbiturates ).Malignancies (ovarian carcinoma) Emotional stressPregnancy -Allen G, Galer BS, et. al Pain, 1999
- OBrien SJ, Gibney MA, et al Am J Sports Med 2004
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Predisposing Factors
Diabetes MellitusHypertriglyceridemia
HyperthyroidismHyperparathyroidismAlcohol Abuse
Tobacco useMultiple sclerosisNeurovegetative dystonia
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INCIDENCE
Age: 40-60 yrs. with a mean age of 50 yrs
*In children:No preceding neurologic or traumatic eventLL>UL
Osteoporosis is rareBone scan: variable, decreased uptakeif positive
Prognosis is favorable
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Incidence
Sex: Equal in men & womenDepends on incidence of inciting event
*In women : Greater sensitivity to painfulstimuli
Greater coping skillsWorse > 50 yrsSeeks early intervention
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How soon after precipitating event will a
patient develop CRPS?
- Usually begins days to weeks after the
inciting event.- In about 80% of cases it occurs within
3 months of a traumatic episode, but
- In many cases, CRPS begins over 6months later.
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DIAGNOSTIC CRITERIA (IASP):
CRPS 11) Presence of noxious event/nerve injury2) Spontaneous, prolonged pain out of proportion,
not limited to a single peripheral nerve area.3) Edema or skin blood flow abnormality in area of
pain since event.4) Other diagnoses excluded
- Harden, et.al., Sept 2007
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DIAGNOSTIC CRITERIA (IASP):
CRPS 21) Presence of continuing pain, allodynia or
hyperlagesia after a nerve injury.2) Edema or skin blood flow abnormality inarea of pain since event.
3) Other diagnoses excluded.
A demonstrable lesion must be present.
- Harden, et.al., Sept 2007
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CLINICAL MANIFESTATIONS
CRPS may occur in either the upper orlower extremities.
Involvement of both upper and lowerextremities in the same patient is unusual.It may be recurrent.
Three clinical stages can occur during thecourse of illness.
- Sheon, Robert P., 2007
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Stage 1
Typically last 3-6 mo. after development of CRPS
Characterized by:Pain in the extremity (limited to site of injury) or in shoulder Movement is painful & tendency for immobilizationLocalized swelling in the extremity
Skin changes of extremity (red warm sweaty, then bluecold dry)
Burning or throbbing painRadiographs: usually normal but may show patchy
demineralization of the involved bones
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Stage 2 (Dystrophic Stage)Persists an additional 3-12 months.
Characterized by: Pain continues, more severe & more diffuseSwelling spreads & changes to brawny hard
edema
Beginning of atrophy of SC tissue & intrinsic ms.Hair is coarse, nails grow faster.Unilateral cold extremity
Progression of osteoporosis
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Stage 3 (Atrophic Stage)
Classically described beginning 9-18 mos after CRPS starts, most severe stage
Characterized by:Pain remains constant or diminishesLimitation of movementShoulder-hand syndrome
Contractures of digitsWaxy trophic skin changesBrittle ridged nailsRadiographs: severe bone demineralization.
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DIFFERENTIAL DIAGNOSIS
Cervical nerve rootimpingement
Pancoasts syndrome VasculitisRheumatoid arthritisPeripheral neuropathy
Migratory osteolysis Venous thrombosis
Arteriovenous fistulaeProgressive systemicsclerosisDisuse atrophy
Angioedema
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Autonomic Testing
Includes: Resting sweat output (RSO) Resting skin temperature (RST) Quantitative sudomotor axon reflex test (QSART)These tests are typically performed byneurologists & physiatrists.
Only recommended in patients in whom thediagnosis is in doubt or where medicolegal issuesare involved.
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Autonomic Testing
Measurement of skin temperature, and sensorychanges can be performed and are less costly,but less sensitive.
Imaging studies may be helpful diagnosticallyincluding:
Bone scintigraphy Plain radiographs Magnetic resonance imaging
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Diagnostic Tests
Bone scanTemperature MeasurementThermographyBone ScintigraphyPlain Film RadiographyMRI
CT Scan
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Diagnostic TESTS
Bone Scan (three phase) positive early sensitive/specific
increased flow increased periarticular uptake 3rd phase > sensitive than
early phase
Temperature Measurement 2 F difference is significant
Thermography
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Bone Scintigraphy
Shows decreased perfusion of the affectedarea and increased uptake in the peripheraljoints of the extremity.
Has a higher sensitivity and specificity thanplain radiography, especially in stage 1.
Is of limited value when performed morethan six months after the onset ofsymptoms.
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Plain Film RadiographyMost useful during stage 3 & stage 2Patchy osteopeniaDemineralization
Less common findings include: Destruction of the joints and
adjacent bones Subluxation
Proliferative new boneformation Extensive degenerative
changes
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Magnetic Resonance Imaging
May show changes in all stages of disease.Especially useful for identifying stage 1 and
stage 3 disease.Is able to identify: Skin thinning/thickening Tissue enhancement Soft tissue edema Muscle atrophy
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MANAGEMENT
A multidisciplinary management approach ofCRPS has been recommended by a consensus ofexperts.
Treatment is more effective when initiated instage 1, as soon as the diagnosis is established,
and before radiographic changes appear.
Prognosis of CRPS type 1 is generally favorable.
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Psychologic Assessment andCounseling
Job and interpersonal relationships should beevaluated in all patients.
Stress-management techniques may reduce theeffect of stress on the autonomic & central nervoussystem.
Consulting a psychiatrist/clinical psychologist isrecommended if: CRPS has been present for more than two months duration. There is no response to treatment. There is a suspected comorbid psychologic or psychiatric
disorder.
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Physical and Occupational Therapy
PT/OT are effective in preventing CRPS.Stimulation of inhibitory neurons :
UltrasoundTENSMassageContrast baths (address vasomotor component)Wax bath (hand stiffness)
Early MobilizationRehab Med referral is recommended soon after the diagnosis is
established.PT should be performed twice daily at home for patients in allstages of CRPS & should begin before limitation of movementoccurs.If PT/OT is delayed to stage 3, impairment is not likely to improve.
- DArcy Y, Werdell J Therapy Insider, June 2008
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Physiatric Therapeutics
elevationcompressionheat/coldtens/ultrasoundstretching/AROM/PROMstress loadingexercise(active/passive)
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Pharmacologic Therapy
Only a few pharmacologic agents have beenstudied in well designed clinical trials to treatCRPS.Medications that appear to resolve painsignificantly better than placebo include agentsin the following classes: Anticonvulsants Bisphosphonates Oral glucocorticoids Nasal calcitonin
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Pharmacologic Therapy
Antidepressant medications, including tricyclicantidepressants, are often effective in reducingneuropathic pain, but have not been studied in CRPS.
Guidelines developed by a consensus of CRPSexperts suggest beginning treatment for pain with: A tricyclic antidepressant (eg, amitriptyline or nortriptyline) An anticonvulsant (eg, gabapentin) A nonsteroidal antiinflammatory drug And an opioid for those with severe pain
-Harden Norman Am J PMR 2005
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Response to Treatment
Patients with stage 1 disease should beseen on a weekly basis.
The treatment should be changed if theresponse is suboptimal.
Goals of therapy are: Decreased pain Improved mobility
Better function
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Response to Treatment
Patients in stage 1 who are not improvingdespite two weeks of oral treatment in
addition to PT, should be considered forinvasive forms of treatment.
Patients in stages 2 and 3 of CRPS are alsocandidates for invasive therapies, if painand function have not rapidly improved.
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Invasive Treatment
Invasive treatments include: Tender point injections
Nerve stimulation Epidural clonidine Regional sympathetic nerve block Ganglion blocks
Intravenous regional blocks Dorsal column spinal cord stimulation Sympathectomy
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Invasive Treatment
Patients receiving noninvasive therapy whoare not improving are offered increasinglyinvasive interventions, allowing 2 weeks forimprovement before moving on to the nexttreatment type.
In tertiary care centers, spinal cord stimulationis considered if the patient has not respondedwithin 12 to 16 weeks of the initiation oftherapy.
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Other Modalities
Intrathecal baclofen may relieve dystonia inpatients with CRPS.
A skilled hypnotherapist can be helpful forpatients with heightened arousal including fear,anxiety, excessive sweating, and weakness.
Hypnosis has allowed physical therapy toprogress in some patients with otherwiseintractable disease.
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Treatment of Relapse
Individual episodes of CRPS may last 6 to 9months.
These may be followed by spontaneousresolution or successful therapy.However, sequelae may persist andrecurrences can occur.
Cold or emotional trauma can cause anexacerbation several months after treatment.Small doses of TCAs and oral guanethidinehave been helpful in treating recurrences.
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PREVENTION
The best treatment of CRPS isprevention!
Early mobilization after stroke or MIis recommended to reduce the risk ofdeveloping CRPS.
- Littlejohn, G., Sept 2007