Diagnosis and Treatment Diagnosis and Treatment Options of RSD/CRPSOptions of RSD/CRPS

Srinivasa N. Raja, MD

Director of Pain Research

Johns Hopkins University

School of Medicine

IntroductionIntroduction

RSD/CRPS is a chronic neurologic syndrome characterized by pain of varying intensity

Early diagnosis and appropriate treatment are essential to avoid disabling pain

RSD/CRPS is often under-diagnosed and under-treated by the medical community

What Is Reflex Sympathetic What Is Reflex Sympathetic Dystrophy Syndrome?Dystrophy Syndrome?

Reflex sympathetic dystrophy syndrome (RSD) is a debilitating neurologic syndrome characterized by

• Pain and hypersensitivity• Vasomotor skin changes• Functional impairment• Various degrees of trophic change

RSD generally follows a musculoskeletal trauma

Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

ChallengesChallenges

Natural course and pathophysiology remain elusive1

Diagnosis made by exclusion of other causes2

Therapies remain controversial3

Underdiagnosed and undertreated

Significant morbidity and loss of quality of life

1. Jänig W. In: Harden , Baron Janig, eds. Complex regional Pain Syndrome, Progress in Pain Research and Management. 2001: 3-15.

2. Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

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

Terminology: RSD vs CRPSTerminology: RSD vs CRPS

RSD = traditional term

Complex regional pain syndrome

(CRPS) = more comprehensive term• Includes disorders not related to sympathetic

nervous system dysfunction

CRPS I = RSD

CRPS II = causalgia (involves nerve injury)

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

Name Change to CRPSName Change to CRPS

Goals: standardized, reliable diagnostic criteria and decision rules

• Allow generalization • Make appropriate treatment selection • Identify reproducible research samples

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

EpidemiologyEpidemiology

Age – common in younger adults• Mean 41.8 years • Mean age at time of injury 37.7 years

Mean duration of symptoms before pain center evaluation = 30 months

2.3 to 3 times more frequent in females than males1

Usually involves a single limb in the early stage 2

1. Raja SN et al. Anesthesiology. 2002;96:1254-1260. 2. Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001, 388-411.

Clinical FeaturesClinical Features

Presence of an initiating noxious event or a cause of immobilization

Continuing pain• Allodynia: pain from a stimulus that

does not normally provoke pain• Hyperalgesia: excessive sensitivity to

pain

Pain disproportionate to any inciting event

Stanton-Hicks M et al. Pain. 1995;63:127-133. Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001; 388-411.

Clinical FeaturesClinical Features(cont’d)(cont’d)

History of edema, changes in skin blood flow, or abnormal sweating in the region of pain

Exclusion of medical conditions that would otherwise account for the degree of pain and dysfunction  

Stanton-Hicks M et al. Pain. 1995;63:127-133. Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001: 388-411.

Checklist for the Diagnosis Checklist for the Diagnosis of RSD: of RSD: HistoryHistory

• Burning pain• Skin, sensitivity to touch• Skin, sensitivity to cold• Abnormal swelling• Abnormal hair growth

• Abnormal nail growth• Abnormal sweating• Abnormal skin color

changes• Abnormal skin

temperature changes• Limited movement

Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

Checklist for the Diagnosis of RSD/CRPS: Examination

• Mechanical allodynia• Hyperalgia to single pinprick• Summation to multiple

pinprick• Cold allodynia• Abnormal swelling

• Abnormal hair growth• Abnormal skin color changes• Abnormal skin temperature (> or < 1 ْC)• Limited range of movement• Motor neglect

Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

Revised Diagnostic CriteriaRevised Diagnostic CriteriaClinical PresentationClinical Presentation

Pain and sensory changes disproportionate to the injury in magnitude or duration

Patients should have at least one symptom in each of these categories and one sign in 2 or more categories

Sensory (hyperesthesia = increased sensitivity to a sensory stimulation)

Vasomotor (temperature or skin abnormalities) Sudomotor (edema or sweating abnormalities) Motor (decreased range of movement, weakness,

tremor, or neglect)

1. Bruehl et al. Pain. 1999;81:147-154. 2. Harden et al. Pain. 1999;83:211-219.

.

Swelling and Color ChangesSwelling and Color Changes

Abnormal Sweating in RSDAbnormal Sweating in RSD

Differential DiagnosesDifferential Diagnoses

• Diabetic and small-fiber peripheral neuropathies

• Entrapment neuropathies

• Thoracic outlet syndrome

• Discogenic disease

• Deep vein thrombosis

• Cellulitis • Vascular

insufficiency• Lymphedema• Erythromelalgia

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

Psychological AspectsPsychological Aspects

Pain can cause symptoms of psychologic distress including

• Anxiety• Depression• Fear• Anger

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

Treatment Treatment

Goals• Rehabilitation• Pain management• Psychological treatment

Multidisciplinary• Physiotherapy• Medical • Psychological

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

Rehabilitation: Clinical Rehabilitation: Clinical Pathway Pathway

Physiotherapy + pain management + psychological therapies = sequential progression through the rehabilitation pathway

PT + OT crucial to patient’s progression

•Therapist assesses patient’s motivation and helps set goals•Adequate analgesia, encouragement, and education of disease process

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

Rehabilitation: General Rehabilitation: General StepsSteps

Desensitization of the affected region

Mobilization, edema control, and isometric strengthening

Stress loading, isotonic strengthening, range of motion, postural normalization and aerobic conditioning

Vocational and functional rehabilitation

Stanton-Hicks M et al. Clin J Pain. 1998;14:155-166.

Pharmacalogic Pain Management

• IV alendronate (bisphosphonate)

• Topic dimethyl sulfoxide

• Topical clonidine

•IV bretylium•IV ketanserin•IV phentolamine•IV lidocaine•Intranasal calcitonin

Most drugs used for neuropathic pain are used to treat RSD/CRPS

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

Kingery WS. Pain.1997;73:123-139

Minimally Invasive Therapies

Sympathetic, IV regional, and somatic nerve blocks

Patients with a sympathetic component to their pain (SMP) should receive nerve blocks

For patients without SMP, a somatic block or epidural infusion may be indicated to optimize analgesia for PT

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

More InvasiveMore Invasive Therapies

Neuroaugmentation

Spinal cord stimulation

Intrathecal drug delivery

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

Surgical Therapies: SympathectomySympathectomy

• Controversial procedure• In carefully selected patients, may result

in reduction in pain severity and disability• Patients with SMP who respond to

selectivesympathetic blockade

• Radiofrequency and neurolytic techniques are alternatives to a surgical sympathectomy

Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.Bandyk DF et al. J Vasc Surg. 2002;35:269-277.

Other Therapies

• Behavioral modification • Psychiatric consultation• Complimentary and Alternative therapies

Acupuncture

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

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.

ConclusionsConclusions

RSD/CRPS is a chronic neurologic syndrome

Not all patients have the same set of symptoms

Early diagnosis and appropriate treatment is essential

Ideal treatment should be multidisciplinary

Bibliography

Bandyk DF, Johnson BL, Kirkpatrick AF, Novotney ML, Back MR, Schmacht DC. Surgical sympathectomy for reflex sympathetic dystrophy syndromes. J Vasc Surg. 2002;35:269-277.

Bogduk N. Complex regional pain syndrome. Current Opinions in Anesthesiology. 2000;14:541-546.

Bruehl SP, Harden RN, Galer BS, et al. External validation of IASP diagnostic criteria for complex regional pain syndrome and proposed research diagnostic criteria. Internal Association for the Study of Pain. Pain. 1999;81:147-154.

Galer BS, Schwartz L, Allen RJ. In: Loeser, ed. Bonica’s Management of Pain. 2001: 388-411.

Harden RN, Bruehl SP, Galer BS, et al. Complex regional pain syndrome: are the IASP diagnostic criteria valid and sufficiently comprehensive? Pain. 1999;83:211-219.

Bibliography (continued)

Jänig W. CRPS-I and CRPS-II: A strategic view, In: Harden , Baron Jänig, eds. Complex regional Pain Syndrome, Progress in Pain Research and Management. 2001: 3-15.

Kingery WS. Pain. A critical review of controlled clinical trials for peripheral neuropathic pain and complex regional pain syndromes. 1997;73:123-139.

Raja SN , Grabow TS. Complex regional pain syndrome I (Reflex Sympathetic Dystrophy) Anesthesiology. 2002;96:1254-1260.

Stanton-Hicks M, Burton AW, Bruehl SP, et al. An updated interdisciplinary clinical pathway for CRPS: Report of an expert panel. Pain Practice. 2002;2:1-16.

Stanton-Hicks M, Jänig W, Hassenbusch S, et al. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain. 1995;63:127-133

Stanton-Hicks M, Baron R, Boas R, et al. Complex Regional Pain Syndrome: guidelines for therapy. Clin J Pain. 1998;14:155-166.