Diagnosis and Treatment Options of RSD/CRPS

1. Diagnosis and Treatment Options of RSD/CRPS Srinivasa N. Raja, MD Director of Pain Research Johns Hopkins University School of Medicine

2. Introduction • 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

3. What Is Reflex Sympathetic 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.

4. Challenges • 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.

5. Terminology: 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.

6. Name 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.

7. Epidemiology 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 ofPain. 2001, 388-411.

8. Clinical 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.

9. Clinical Features(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.

10. 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 Checklist for the Diagnosis of RSD: History Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

11. 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 < 1ْ C) Limited range of movement Motor neglect Checklist for the Diagnosis of RSD/CRPS: Examination Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

12. Revised Diagnostic CriteriaClinical 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. .

13. Swelling and Color Changes

14. Abnormal Sweating in RSD

15. Diabetic and small-fiber peripheral neuropathies Entrapment neuropathies Thoracic outlet syndrome Discogenic disease Deep vein thrombosis Cellulitis Vascular insufficiency Lymphedema Erythromelalgia Differential Diagnoses Raja SN et al. Anesthesiology. 2002;96:1254-1260.

16. Psychological Aspects • Pain can cause symptoms of psychologic distress including • Anxiety • Depression • Fear • Anger Raja SN et al. Anesthesiology. 2002;96:1254-1260.

17. Treatment Goals • Rehabilitation • Pain management • Psychological treatment Multidisciplinary • Physiotherapy • Medical • Psychological Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

18. 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 Rehabilitation: Clinical Pathway Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

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

20. Most drugs used for neuropathic pain are used to treat RSD/CRPS Pharmacalogic Pain Management • IV alendronate (bisphosphonate) • Topic dimethyl sulfoxide • Topical clonidine • IV bretylium • IV ketanserin • IV phentolamine • IV lidocaine • Intranasal calcitonin Raja SN et al. Anesthesiology. 2002;96:1254-1260. Kingery WS. Pain.1997;73:123-139

21. 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.

22. More Invasive Therapies Neuroaugmentation Spinal cord stimulation Intrathecal drug delivery Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

23. Surgical Therapies: Sympathectomy • 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.

24. Other Therapies • Behavioral modification • Psychiatric consultation • Complimentary and Alternative therapies Acupuncture Raja SN et al. Anesthesiology. 2002; 96:1254-1260.

25. Prognosis • 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.

26. Conclusions • 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

27. 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.

28. 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.