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Diagnosis and Treatment Diagnosis and Treatment Options for Complex Regional Options for Complex Regional Pain Syndrome Pain Syndrome (CRPS) (CRPS) Karsten Bartels, MD Srinivasa N. Raja, MD Division of Pain Medicine Johns Hopkins University School of Medicine Baltimore, Maryland

Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

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Page 1: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Diagnosis and Treatment Diagnosis and Treatment

Options for Complex Regional Options for Complex Regional

Pain SyndromePain Syndrome (CRPS)(CRPS)

Karsten Bartels, MD

Srinivasa N. Raja, MD

Division of Pain Medicine

Johns Hopkins University

School of Medicine

Baltimore, Maryland

Page 2: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

IntroductionIntroduction

�� CRPS (formerly known as Reflex Sympathetic CRPS (formerly known as Reflex Sympathetic

Dystrophy [RSD]) is a chronic disease, most Dystrophy [RSD]) is a chronic disease, most

often resulting from trauma, characterized by often resulting from trauma, characterized by

pain of varying intensitypain of varying intensity

�� Early diagnosis and appropriate treatment are Early diagnosis and appropriate treatment are

importantimportant

�� CRPS is underdiagnosed and undertreated by CRPS is underdiagnosed and undertreated by

the medical communitythe medical community

Page 3: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

What Is CRPS?What Is CRPS?

�� CRPS is a debilitating chronic pain syndrome CRPS is a debilitating chronic pain syndrome

characterized by varying degrees of:characterized by varying degrees of:

�� Pain and skin hypersensitivityPain and skin hypersensitivity

�� Vasomotor skin changesVasomotor skin changes

�� Sweat disturbance/ edemaSweat disturbance/ edema

�� Motor disturbancesMotor disturbances

�� TrophicTrophic changeschanges

�� CRPS often follows a musculoskeletal trauma, CRPS often follows a musculoskeletal trauma,

surgery, or immobilization.surgery, or immobilization.

BogdukBogduk N. N. CurrCurr OpinOpin AnesthesiolAnesthesiol. 2001;14(5):541. 2001;14(5):541--546.546.

Harden RN, Harden RN, BruehlBruehl SP. In: SP. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines.Milford, CT: RSDSA Press; 2006:1Milford, CT: RSDSA Press; 2006:1--11.11.

Page 4: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

ChallengesChallenges

�� Natural course and pathophysiology remain poorly Natural course and pathophysiology remain poorly

understoodunderstood11

�� Inflammation, Inflammation, vasodysregulationvasodysregulation, and axonal , and axonal

injury have recently been implicated in the injury have recently been implicated in the

pathogenesis of CRPSpathogenesis of CRPS22

�� Therapies remain controversial due to lack of Therapies remain controversial due to lack of

controlled trialscontrolled trials33

�� Diagnosis is made by exclusion of other causesDiagnosis is made by exclusion of other causes..

�� Associated with significant morbidity and loss of Associated with significant morbidity and loss of

quality of lifequality of life44

1.1. JJäänignig W. In: Harden, Baron, W. In: Harden, Baron, JJäänignig. . Complex Regional Pain Syndrome. Complex Regional Pain Syndrome. 2001:32001:3--15.15.

2.2. OaklanderOaklander AL. AL. Pain. Pain. 2009;139;2392009;139;239--240.240.

3.3. BogdukBogduk N. N. CurrCurr OpinOpin AnaesthesiolAnaesthesiol.. 2001;14:5412001;14:541--546.546.

4.4. Raja SN et al. Raja SN et al. Anesthesiology.Anesthesiology. 2002;96:12542002;96:1254--1260.1260.

Page 5: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Linda LangLinda LangRSDSA Board member and coRSDSA Board member and co--author of author of Living with RSDSLiving with RSDS

““Consider, too, that in publicizing Consider, too, that in publicizing

RSD, we generally focus on the RSD, we generally focus on the

pain, not the disabilities that come pain, not the disabilities that come

with itwith it––the legs and hands that no the legs and hands that no

longer work, the bones that longer work, the bones that

become osteoporotic , the joints become osteoporotic , the joints

that become locked, the muscles that become locked, the muscles

that become spasticthat become spastic…….There is .There is

an awful lot we leave outan awful lot we leave out––how a how a

productive member of society can become too productive member of society can become too

disabled to work or take care of her children. We disabled to work or take care of her children. We

dondon’’t discuss the tremendous personal lossest discuss the tremendous personal losses––

families, friends, jobs that RSD wreaks.families, friends, jobs that RSD wreaks.””

Page 6: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Terminology: RSD vs. CRPSTerminology: RSD vs. CRPS

� Traditional term = RSD

� More comprehensive term = CRPS

� Complex regional pain syndrome (CRPS)

� Includes disorders not related to sympathetic nervous system dysfunction

Galer BS, et al. In: Loeser, ed. Bonica’s Management of Pain. 2001: 388-411.

Harden RN, Harden RN, BruehlBruehl SP. In: SP. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines.Milford, CT: RSDSA Press; 2006:1Milford, CT: RSDSA Press; 2006:1--1111

Page 7: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Terminology: RSD vs. CRPSTerminology: RSD vs. CRPS

� Types

� CRPS 1 = RSD

� CRPS 2 = Causalgia (involves major nerve injury)

� CRPS-NOS (Not Otherwise Specified) = partially meets CRPS criteria; not better explained by other condition

Galer BS, et al. In: Loeser, ed. Bonica’s Management of Pain. 2001: 388-411.

Harden RN, Harden RN, BruehlBruehl SP. In: SP. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines.Milford, CT: RSDSA Press; 2006:1Milford, CT: RSDSA Press; 2006:1--1111

Page 8: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

EpidemiologyEpidemiology

�� Incidence 26.2 per 100,000 person yearsIncidence 26.2 per 100,000 person years11

�� Age: common in younger adultsAge: common in younger adults

�� Mean 41.8 years Mean 41.8 years

�� Mean age at time of injury 37.7 yearsMean age at time of injury 37.7 years

�� Mean duration of symptoms before pain center Mean duration of symptoms before pain center

evaluation = 30 monthsevaluation = 30 months

�� 3.4 times more frequent in females than males3.4 times more frequent in females than males11

�� Usually involves a single limb in the early Usually involves a single limb in the early

stagestage22

1.1. De De MosMos M. M. Pain.Pain. 2007;129;122007;129;12--20. 20.

2.2. GalerGaler BS, et al. In: BS, et al. In: LoeserLoeser. Ed. . Ed. BonicaBonica’’ss Management ofManagement of Pain.Pain. 2001;3882001;388--411.411.

Page 9: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

CRPS EpidemiologyCRPS Epidemiology

�� Prospective epidemiology links CRPS with Prospective epidemiology links CRPS with

nerve injury, asthma, migraine, osteoporosis, nerve injury, asthma, migraine, osteoporosis,

NOT with NOT with somatizationsomatization or psychiatric diseaseor psychiatric disease

�� There may be as many 50,000 new cases a There may be as many 50,000 new cases a

yearyear1,21,2

1. de 1. de MosMos M, de M, de BruijnBruijn AG, AG, HuygenHuygen FJ, et al. FJ, et al. PainPain. 2007 May;129(1. 2007 May;129(1--2):122):12--20.20.

2. de 2. de MosMos M, M, HuygenHuygen FJ, FJ, DielemanDieleman JP, et al. JP, et al. PainPain. 2008 Oct 15;139(2):458. 2008 Oct 15;139(2):458--66.66.

Page 10: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Surgery and TraumaSurgery and TraumaCommon inciting factorsCommon inciting factors

�� PostPost--surgerysurgery

�� 20% of CRPS patients seen in pain clinics 20% of CRPS patients seen in pain clinics

have treatment history of prior surgery, have treatment history of prior surgery,

mostly orthopedic in the involved body mostly orthopedic in the involved body

region region

�� Smaller prospective studies suggest 11% Smaller prospective studies suggest 11%

to 18% of patients following fracture or to 18% of patients following fracture or

total knee total knee arthroplastyarthroplasty may develop may develop

CRPSCRPS--II

Page 11: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

CRPS: A webCRPS: A web--based surveybased survey

Surgery29.3%

Fracture15.1%

Sprain11.1%

CrushInjury10.4%

Others34.1%(Contusion, Stroke, Dislocation, MVA, Electrical Injury,Injection)

Inciting event Factors associated with increased pain

Factors

Physical stress

Emotional stress

Hot weather

Cold weather

Lying down

Moving the affected area

Working

Perc

en

t o

f su

bje

cts

0

20

40

60

80

100

Sharma A, Sharma A, AgarwalAgarwal S, S, BroatchBroatch J, Raja SN. J, Raja SN. RegReg AnesthAnesth Pain MedPain Med. 2009;34:110. 2009;34:110--115.115.

Page 12: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Spread of SymptomsSpread of Symptoms

�� More than 77% of respondents report spreading More than 77% of respondents report spreading

of symptoms to a region other than the site of of symptoms to a region other than the site of

the original diseasethe original disease11

�� The exact frequency of spread of CRPSThe exact frequency of spread of CRPS--I is not I is not

available in published literatureavailable in published literature22

�� A pattern called A pattern called ‘‘Independent SpreadIndependent Spread’’ is is

estimated to occur in 6.4% of CRPSestimated to occur in 6.4% of CRPS--I patientsI patients22

�� Other investigators agree that spread is not Other investigators agree that spread is not

uncommonuncommon

1. 1. Sharma A, Sharma A, AgarwalAgarwal S, S, BroatchBroatch J, Raja SN. J, Raja SN. RegReg AnesthAnesth Pain MedPain Med. 2009;34:110. 2009;34:110--115.115.

2. 2. Maleki J, LeBel AA, Bennett GJ, Schwartzman RJ. Maleki J, LeBel AA, Bennett GJ, Schwartzman RJ. PainPain. 2000 Dec 1;88(3):259. 2000 Dec 1;88(3):259--266.266.

Page 13: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Remission Remission

� Remission of CRPS is difficult to predict based on demographic and clinical characteristics.

� The correlation between remission and improvement with sympathetic blocks suggests CRPS patients with sympathetically-maintained pain are more likely to achieve remission.

� Remission is often transient, with most experiencing recurrence of symptoms.

Lesley M, et al. Remission and Recurrence of Complex Regional PaLesley M, et al. Remission and Recurrence of Complex Regional Pain Syndrome: Analysis in Syndrome: Analysis

of a Webof a Web--based Survey. Abstract.based Survey. Abstract.

http://www.rsds.org/3/research/Lesley_Mazloomdoost_Agarwal.html http://www.rsds.org/3/research/Lesley_Mazloomdoost_Agarwal.html

Page 14: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Quality of Life IssuesQuality of Life Issues11

�� Employment Employment

�� 60% rated themselves as disabled60% rated themselves as disabled

�� Only 16% were employed fullOnly 16% were employed full--timetime

�� SleepSleep

�� 94% said that their pain affects sleep94% said that their pain affects sleep

1.1. AgarwalAgarwal S, S, BroatchBroatch J, Raja SN. J, Raja SN. AnesthesiologyAnesthesiology. 2005;103:A902.. 2005;103:A902.

Page 15: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Clinical FeaturesClinical Features

CRPS describes an array of painful conditions that CRPS describes an array of painful conditions that are characterized by a continuing regional pain are characterized by a continuing regional pain that is seemingly disproportionate in time or that is seemingly disproportionate in time or degree to the usual course of any know trauma or degree to the usual course of any know trauma or other lesion. other lesion.

The pain is regional and usually has a distal The pain is regional and usually has a distal predominance of abnormal sensory, motor, predominance of abnormal sensory, motor, sudomotorsudomotor, vasomotor, and/or , vasomotor, and/or trophictrophic findings. findings.

The presence of a group of symptoms has been The presence of a group of symptoms has been tested for optimal sensitivity and specificity. The tested for optimal sensitivity and specificity. The syndrome shows variable progression over time. syndrome shows variable progression over time.

Harden RN, et al. Harden RN, et al. Pain MedPain Med. 2007;8:326. 2007;8:326--331.331.

Page 16: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Diagnostic CriteriaDiagnostic Criteria

�� Continuing pain, which is disproportionate to Continuing pain, which is disproportionate to any inciting eventany inciting event

�� There is no other diagnosis that better There is no other diagnosis that better explains the signs and symptomsexplains the signs and symptoms

Harden RN, et al. Harden RN, et al. Pain MedPain Med. 2007;8:326. 2007;8:326--331.331.

Page 17: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Diagnostic CriteriaDiagnostic CriteriaSymptoms and SignsSymptoms and Signs

Report three of the four symptoms following and display at the time of evaluation at least two or more of the signs:

1. Sensory

� Allodynia: pain from a stimulus that does not normally provoke pain or

� Hyperesthesia: increased sensitivity to a sensory stimulation

2.Vasomotor

� Temperature asymmetry (2°C) and/or skin color changes and/or skin color asymmetry

Harden RN, et al. Harden RN, et al. Pain MedPain Med. 2007;8:326. 2007;8:326--331.; 331.; KrumovaKrumova EK, et al. EK, et al. Pain.Pain. 2008;140:82008;140:8--22.22.

Page 18: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Diagnostic CriteriaDiagnostic CriteriaSymptoms and SignsSymptoms and Signs

3. Sudomotor/Edema

� Edema and/or sweating changes

4. Motor/Trophic

� Decreased range of motion and/or motor dysfunction and/or trophic changes (hair, nail, skin)

Harden RN, et al. Harden RN, et al. Pain MedPain Med. 2007;8:326. 2007;8:326--331.; 331.; KrumovaKrumova EK, et al. EK, et al. Pain.Pain. 2008;140:82008;140:8--22.22.

Page 19: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Swelling/EdemaSwelling/Edema

Page 20: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Vasomotor ChangesVasomotor Changes

Page 21: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Abnormal SweatingAbnormal Sweating

Page 22: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Motor Disturbance: Motor Disturbance: DystoniaDystonia

Page 23: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Differential DiagnosesDifferential Diagnoses

� Diabetic and other small-fiber peripheral neuropathies

� Entrapment neuropathies

� Thoracic outlet syndrome

� Discogenic disease

� Deep vein thrombosis

� Cellulitis

� Vascular insufficiency

� Lymphedema

� Erythromelalgia

Raja SN, et al. Raja SN, et al. Anesthesiology.Anesthesiology. 2002;96:12542002;96:1254--1260.1260.

Page 24: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Psychological AspectsPsychological Aspects

� Pain can cause symptoms of psychologicdistress, including

� Anxiety

� Depression

� Posttraumatic Stress Syndrome

� Fear

� Anger

Raja SN, et al. Raja SN, et al. Anesthesiology.Anesthesiology. 2002;96:12542002;96:1254--1260.1260.

Page 25: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Treatment Guidelines Treatment Guidelines

Stanton-Hicks M, et al. Pain Practice. 2002;2:1-16.

Harden RN, Swan M, Costa BR, et al. In: Complex Regional Pain Syndrome: Treatment Guidelines. Milford CT: RSDSA Press;2006:12-24.

1. Physiotherapy

2. Pain management

3. Psychological therapy

Sequential progression through functional restoration pathway

Page 26: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Treatment Guidelines Treatment Guidelines

Physical therapy (PT), Occupational therapy (OT), and Rehabilitation therapy (RT) are crucial to patient’s progression

� Assessment of patient’s motivation

� Goal setting

� Adequate analgesia, encouragement, and education

Stanton-Hicks M, et al. Pain Practice. 2002;2:1-16.

Harden RN, Swan M., Costa BR, et al. In: Complex Regional Pain Syndrome: Treatment Guidelines. Milford CT: RSDSA Press;2006:12-24.

Page 27: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Overall Evidence Gaps Overall Evidence Gaps Based upon literatureBased upon literature

�� Inadequate evidence of efficacy; lack of Inadequate evidence of efficacy; lack of

multiple high quality multiple high quality RCTsRCTs

�� Small sample sizesSmall sample sizes

�� FollowFollow--up periods variableup periods variable

�� Few studies on QOL and function, not used Few studies on QOL and function, not used

as primary outcomeas primary outcome

�� Few studies compare interventional Few studies compare interventional

therapies to other treatments or placebotherapies to other treatments or placebo

Page 28: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Functional Restoration: Functional Restoration:

General StepsGeneral StepsReactivation, contrast baths, desensitization, and Reactivation, contrast baths, desensitization, and

exposure therapyexposure therapy

Flexibility, mobilization, edema control, isometric Flexibility, mobilization, edema control, isometric

strengthening, and correction of postural strengthening, and correction of postural

abnormalitiesabnormalities

Stress loading, isotonic strengthening, range of Stress loading, isotonic strengthening, range of

motion, postural normalization, and aerobic motion, postural normalization, and aerobic

conditioningconditioning

Ergonomics, movement therapy, normalization of use, Ergonomics, movement therapy, normalization of use,

and vocational and functional rehabilitationand vocational and functional rehabilitation

StantonStanton--Hicks M et al. Hicks M et al. ClinClin J Pain.J Pain. 1998;14:1551998;14:155--166.; Harden RN, et al. In: 166.; Harden RN, et al. In: Complex Complex Regional Pain Syndrome: Treatment Guidelines.Regional Pain Syndrome: Treatment Guidelines. Milford, CT: RSDSA Press;2006:12Milford, CT: RSDSA Press;2006:12--24.24.

Page 29: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Therapeutic Options for Therapeutic Options for

Pain ManagementPain Management

� Pharmacologic therapy

� Intravenous ketamine

� Intrathecal baclofen

� Sympathetic neural blockade

� Psychological intervention

� Complementary therapy

� Mirror therapy

� Spinal cord stimulation

Page 30: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Mirror Therapy for CRPSMirror Therapy for CRPS

�� Exercise of cardinal Exercise of cardinal

movements of the affected movements of the affected

limb while viewing an image limb while viewing an image

of their unaffected limb in a of their unaffected limb in a

mirror for 30 minutes daily mirror for 30 minutes daily

has been shown to improve has been shown to improve

pain, motor function, and pain, motor function, and

edemaedema11

�� Sensory discrimination training while looking Sensory discrimination training while looking

toward the affected body part but seeing the toward the affected body part but seeing the

opposite part of the body in the mirror also opposite part of the body in the mirror also

decreased pain and increased tactile acuitydecreased pain and increased tactile acuity22

CacchioCacchio C et al. C et al. N Eng J Med. N Eng J Med. 2009;361(6);6342009;361(6);634--636.; Moseley GL, et al. 636.; Moseley GL, et al. Pain.Pain.2009;144;3142009;144;314--319.319.

Page 31: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

EvidenceEvidence--based Practice based Practice

RecommendationsRecommendations

QuiselQuisel A, Gill JM, A, Gill JM, WitherellWitherell P. P. J J FamFam PractPract. 2005 Jul;54(7):599. 2005 Jul;54(7):599--603.603.

�� Treatments for CRPSTreatments for CRPS--I supported by evidence I supported by evidence

of efficacy and little likelihood for harm are:of efficacy and little likelihood for harm are:

�� Topical DMSO creamTopical DMSO cream

�� IV IV bisphosphonatesbisphosphonates

�� Limited courses of oral corticosteroidsLimited courses of oral corticosteroids

�� Despite some contradictory evidence, physical Despite some contradictory evidence, physical

therapy and therapy and calcitonincalcitonin (intranasal or (intranasal or

intramuscular) are likely to benefit patients intramuscular) are likely to benefit patients

with CRPSwith CRPS--II

Page 32: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Pharmacotherapy GuidePharmacotherapy Guide

Symptoms/ Signs Treatment(s)

MildMild--toto--moderate painmoderate pain Simple analgesics, sympathetic Simple analgesics, sympathetic

nerve blocks nerve blocks

Severe/intractable painSevere/intractable pain Opioids, nerve blocks, IV ketamine Opioids, nerve blocks, IV ketamine

Inflammation, edemaInflammation, edema Steroids, Steroids, NSAIDsNSAIDs

Depression, anxiety, insomniaDepression, anxiety, insomnia Sedatives, antidepressants, Sedatives, antidepressants,

anxiolyticsanxiolytics

AllodyniaAllodynia, , hyperalgesiahyperalgesia Anticonvulsants, Anticonvulsants, lidocainelidocaine patchpatch

OsteopeniaOsteopenia, immobility, , immobility,

trophictrophic changeschangesCalcitoninCalcitonin, , bisphophonatesbisphophonates

Vasomotor disturbancesVasomotor disturbances Calcium channel blockers, Calcium channel blockers,

sympatholyticssympatholytics, and/or blocks, and/or blocks

Spasticity Spasticity BaclofenBaclofen

Harden RN. In: Harden RN. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: Milford, CT:

RSDSA Press;2006:25RSDSA Press;2006:25--36.; Schwartzman RJ. 36.; Schwartzman RJ. Pain.Pain. 2009;147:1072009;147:107--115.115.

Page 33: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Role of OpioidsRole of Opioids

�� Only one controlled trial evaluating controlledOnly one controlled trial evaluating controlled--

release morphine in CRPS; showed no difference release morphine in CRPS; showed no difference

(only 8(only 8--day trial)day trial)11

�� Methadone may have a special place because it Methadone may have a special place because it

blocks NMDA receptorblocks NMDA receptor

�� The use of opioid therapy should be linked to The use of opioid therapy should be linked to

increased participation in the functional increased participation in the functional

restoration process, as with all medications or restoration process, as with all medications or

interventionsinterventions22

1.1. HarkeHarke H, H, GretenkortGretenkort P, P, LadleifLadleif HU, HU, RahmanRahman S, S, HarkeHarke O. O. AnesthAnesth AnalgAnalg. 2001;92:488. 2001;92:488--495.495.

2.2. Harden RN. In: Harden RN. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: Milford, CT:

RSDSA Press; 2006:25RSDSA Press; 2006:25--36.36.

Page 34: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Pharmacologic Pain TherapyPharmacologic Pain Therapy

�� IV IV alendronatealendronate

((bisphosphonatebisphosphonate))

�� Topic Topic dimethyldimethyl

sulfoxidesulfoxide

�� Topical Topical clonidineclonidine

�� Topical ketamineTopical ketamine

�� IV IV bretyliumbretylium

�� IV IV ketanserinketanserin

�� IV IV phentolaminephentolamine

�� IV IV lidocainelidocaine

�� Intranasal Intranasal calcitonincalcitonin

�� Most medications used for neuropathic pain Most medications used for neuropathic pain are used to treat pain in CRPS, such as are used to treat pain in CRPS, such as antidepressants, anticonvulsants, and opioidsantidepressants, anticonvulsants, and opioids

�� Other medications:Other medications:

Schwartzman RJ, et al. Schwartzman RJ, et al. Pain Med.Pain Med. 2009;10:4012009;10:401--411.; Raja SN, et al. 411.; Raja SN, et al.

AnesthesiolAnesthesiol.. 2002;96:12542002;96:1254--1260.; 1260.; KingeryKingery WS. WS. Pain.Pain.1997;73:1231997;73:123--139.139.

Page 35: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Intravenous KetamineIntravenous Ketamine

High doseHigh dose

Anesthetic doses of Anesthetic doses of

ketamine have been ketamine have been

used successfully in used successfully in

treatment of CRPS. treatment of CRPS.

However, these However, these

findings have not been findings have not been

tested in randomized tested in randomized

trialstrials

Low dose Low dose

Intravenous ketamine at Intravenous ketamine at

low doses (25low doses (25--30 mg/hr 30 mg/hr

in 70 kg adult) resulted in 70 kg adult) resulted

in significant pain relief in significant pain relief

but no functional but no functional

improvementimprovement

SigtermansSigtermans MJ. MJ. Pain.Pain. 2009;145:3042009;145:304--11.11.

Schwartzman RJ. Schwartzman RJ. PainPain. 2009;147:107. 2009;147:107--115115Kiefer RT. Kiefer RT. Pain MedPain Med. 2008;9:1173. 2008;9:1173--201.201.

Page 36: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Interventional Pain Therapy Interventional Pain Therapy

� Minimally Invasive Therapies

� Sympathetic/Somatic nerve blocks

� IV Regional nerve blocks

� More Invasive Therapies

� Epidural/Plexus Catheter Blocks

� Neurostimulation

� Intrathecal Drug Infusion

� Surgical Therapies

� Sympathectomy

� Motor Cortex Stimulation

Burton A. In: Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: RSDSA Press; 2006:512006:51--62.;62.; Velasco F. Pain. 2009;147:91-98.

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Minimally Invasive TherapiesMinimally Invasive Therapies

The choice of the nerve block should depend on The choice of the nerve block should depend on

the presence or absence of a demonstrable the presence or absence of a demonstrable

sympathetic component to the pain (SMP) in the sympathetic component to the pain (SMP) in the

patient.patient.

�� Patients with SMP may benefit from Patients with SMP may benefit from

sympathetic, IV regional, and somatic nerve sympathetic, IV regional, and somatic nerve

blocks.blocks.

�� For patients without SMP, a somatic block or For patients without SMP, a somatic block or

epidural infusion may be indicated to optimize epidural infusion may be indicated to optimize

analgesia for PT.analgesia for PT.

Burton A. In: Burton A. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: Milford, CT:

RSDSA Press; 2006:51RSDSA Press; 2006:51--62.; Stanton62.; Stanton--Hicks M, et al. Hicks M, et al. Pain PracticePain Practice. 2002;2:1. 2002;2:1--16.16.

Page 38: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

More InvasiveMore Invasive TherapiesTherapies

�� Spinal cord stimulation Spinal cord stimulation maymay result inresult in

�� Pain reductionPain reduction

�� Subjective improvement of health statusSubjective improvement of health status

�� Objective improvement of functional status Objective improvement of functional status

remains to be provenremains to be proven

Van Rijn MA, et al. Van Rijn MA, et al. Pain. Pain. 2009;143:412009;143:41--47.; Stanton47.; Stanton--Hicks M, et al. Hicks M, et al. Pain PracticePain Practice. . 2002;2:12002;2:1--16.; Burton A. In: 16.; Burton A. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines.Milford, CT: RSDSA Press; 2006:51Milford, CT: RSDSA Press; 2006:51--62.62...

Page 39: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

More InvasiveMore Invasive TherapiesTherapies

�� IntrathecalIntrathecal drug delivery drug delivery

�� Role of Role of intrathecalintrathecal opioids or combination opioids or combination

therapies have not been studied well and therapies have not been studied well and

should be used with cautionshould be used with caution

�� IntrathecalIntrathecal baclofenbaclofen might offer patients with might offer patients with

CRPS associated CRPS associated dystoniadystonia pain relief and pain relief and

increased quality of lifeincreased quality of life

Van Rijn MA, et al. Van Rijn MA, et al. Pain. Pain. 2009;143:412009;143:41--47.; Stanton47.; Stanton--Hicks M, et al. Hicks M, et al. Pain PracticePain Practice. . 2002;2:12002;2:1--16.; Burton A. In: 16.; Burton A. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines.Milford, CT: RSDSA Press; 2006:51Milford, CT: RSDSA Press; 2006:51--62.62...

Page 40: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

IntrathecalIntrathecal BaclofenBaclofen

�� DystoniaDystonia in CRPS that can in CRPS that can

not be treated by more not be treated by more

conservative measures conservative measures

can be alleviated through can be alleviated through

intrathecalintrathecal baclofenbaclofen

�� In patients with In patients with dystoniadystonia, ,

baclofenbaclofen possibly possibly

improves pain, disability improves pain, disability

and quality of lifeand quality of life

Van Van HiltenHilten BJ, et al. BJ, et al. N N EnglEngl J MedJ Med. 2000 Aug 31;343(9):625. 2000 Aug 31;343(9):625--630.; Van Rijn MA et al. 630.; Van Rijn MA et al.

Pain. Pain. 2009;143:412009;143:41--47.47.

Fig 2. Van Rijn. Fig 2. Van Rijn. PainPain. 2009;143:41. 2009;143:41--47.47.

Page 41: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Spinal Cord StimulationSpinal Cord Stimulation

�� Spinal cord stimulation (SCS) has a modest, Spinal cord stimulation (SCS) has a modest,

timetime--limited effect on pain scores but no limited effect on pain scores but no

effect on healtheffect on health--related quality of liferelated quality of life

KemlerKemler MA. MA. N N EnglEngl J MedJ Med. 2006 Jun;354(22):2394. 2006 Jun;354(22):2394--2396.; 2396.; KemlerKemler MA. MA. J J NeurosurgNeurosurg..2008;108:2922008;108:292--298.298.

Page 42: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

Psychological InterventionPsychological Intervention

�� Patient/family educationPatient/family education

�� Pathophysiology/disuse issuesPathophysiology/disuse issues

�� PsychophysiologicPsychophysiologic interactionsinteractions

�� SelfSelf--management focusmanagement focus

�� Psychological evaluationPsychological evaluation

�� Cognitive, behavioral, and emotional Cognitive, behavioral, and emotional

responseresponse

�� ComorbiditiesComorbidities

�� Response of family and friendsResponse of family and friends

BruehlBruehl SP. In: SP. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: Milford, CT:

RSDSA Press; 2006:37RSDSA Press; 2006:37--50.50.

Page 43: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

� Psychological pain management evaluation

� Relaxation training with feedback

� Constructive self-talk

� Behavioral intervention

� Family intervention

� Constructive social support

Psychological InterventionPsychological Intervention

BruehlBruehl SP. In: SP. In: Complex Regional Pain Syndrome: Treatment Guidelines.Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: Milford, CT:

RSDSA Press; 2006:37RSDSA Press; 2006:37--50.50.

Page 44: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

PreventionPrevention

�� Vitamin CVitamin C

�� In doses of 0.5In doses of 0.5--1gm Vitamin C daily, it has 1gm Vitamin C daily, it has

been shown to decrease the incidence of been shown to decrease the incidence of

CRPS after foot and ankle surgeryCRPS after foot and ankle surgery11 as well as well

as following wrist fractureas following wrist fracture22

�� Vitamin C appears to be a simple and costVitamin C appears to be a simple and cost--

effective way of limiting the incidence of effective way of limiting the incidence of

CRPS and can be considered in atCRPS and can be considered in at--risk risk

patientspatients

1.1. BesseBesse JL. JL. Foot and Ankle SurgeryFoot and Ankle Surgery; 2009; 15: 179; 2009; 15: 179--82.82.

2.2. ZollingerZollinger PE. J Bone Joint PE. J Bone Joint SugerySugery Am; 2007; 89: 1424Am; 2007; 89: 1424--3131

Page 45: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

PrognosisPrognosis

� Difficult to predict

� Earlier intervention may be more likely to be successful

� Some patients experience reduced symptoms or apparently full recovery

� Some patients continue to experience significant disability

Raja SN, et al. Anesthesiology. 2002;96:1254-1260.

Page 46: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

ConclusionsConclusions

�� CRPS is a chronic pain syndromeCRPS is a chronic pain syndrome

�� Not all patients have the same set of symptomsNot all patients have the same set of symptoms

�� Early diagnosis and appropriate treatment may Early diagnosis and appropriate treatment may

be associated with faster recoverybe associated with faster recovery

�� Ideal treatment should be multidisciplinaryIdeal treatment should be multidisciplinary

�� Consider prevention for high risk surgeryConsider prevention for high risk surgery

Page 47: Diagnosis and Treatment Options for Complex … and Treatment Options for Complex Regional Pain Syndrome (CRPS) KarstenBartels, MD SrinivasaN. Raja, MD Division of Pain Medicine Johns

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