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

2. Introduction Reflex Sympathetic Dystrophy Syndrome (RSD), also known as Complex Regional Pain Syndrome (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 CRPS? CRPS is a debilitating neurologic syndrome characterized by Pain and hypersensitivity Vasomotor skin changes Functional impairment Various degrees of trophic change CRPS generally follows a musculoskeletal trauma Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

4. Challenges Natural course and pathophysiology remain elusive 1 Diagnosis made by exclusion of other causes 2 Therapies remain controversial 3 Under-diagnosed and under-treated 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 For 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 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 males 1 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.

8. Clinical Features of CRPS 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 (swelling), 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. Clinical Aspects of CRPS Psychological Inflammatory/ Trophic Inflammatory/ Trophic Motor Autonomic Sensory PAIN

11. Sensory Changes in CRPS Allodynia Hyperalgesia Hyperesthesia Increased sensitivity to a sensory stimulation Hyperpathia Abnormally exaggerated subjective response to painful stimuli

12. Edema Color change Temperature (cooler or warmer) Sweating (  or  ) Autonomic Signs in CRPS

13. Abnormal Sweating

14. Abnormal Swelling

15. Altered nail growth Altered hair growth Skin changes Altered nail growth Altered hair growth Skin changes Trophic Changes

16. Trophic Changes (cont’d) Skin changes

17. Psychological Changes Fear Anxiety Anger Suffering Depression Failure to Cope Fear Anxiety Anger Suffering Depression Failure to Cope Raja SN et al. Anesthesiology. 2002;96:1254-1260.

18. Clinical 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..

19. Checklist for Diagnosis: History 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.

20. Checklist for Diagnosis: 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 < 1ْ C) Limited range of movement Motor neglect Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.

21. Differential 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.

22. CRPS Spreads Patterns of spread Contiguous spread  Gradual and significant enlargement of the affected area Independent spread  CRPS appears in a distant, non-contiguous area Mirror-image spread  Symptoms appear on the opposite side in an area that closely matches size and location of original Maleki J et al. Pain. 2000;88:259-266.

23. A patient with both upper and lower extremity being affected with RSD/CRPS at different time points about 2 years apart. Spread of CRPS

24. Goal and Strategy for Treatment Raja SN et al. Anesthesiology. 2002;96:1254-1260.

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

26. Rehabilitation: Clinical 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.

27. Rehabilitation: General Steps 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.

28. Pharmacologic Pain Management No “gold standard” treatment for CRPS Effective therapy has included Tricyclic antidepressants IV and topical lidocaine IV ketamine Carbamazepine Topical aspirin Raja SN et al. Anesthesiology. 2002;96:1254-1260. Kingery WS. Pain.1997;73:123-139

29. Pharmacologic Pain Management (cont ’ d) IV alendronate (bisphosphonate) Topic dimethyl sulfoxide Topical clonidine IV bretylium IV ketanserin IV phentolamine 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

30. Minimally Invasive Intervention 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.

31. More Invasive Intervention Tunneled epidural catheters Neuroaugmentation Spinal cord stimulation Intrathecal drug delivery Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

32. Sympathectomy Controversial surgical procedure In carefully selected patients, may result in reduction in pain severity and disability Patients with SMP who respond to selective sympathetic blockade Alternatives Radiofrequency Neurolytic techniques Stanton-Hicks M et al. Pain Practice. 2002;2:1-16. Bandyk DF et al. J Vasc Surg. 2002;35:269-277.

33. Psychotherapy Essential part of rehabilitation process includes Cognitive behavioral therapy Coping skills Stress management Relaxation techniques Imagery Self-hypnosis Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

34. 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.

35. 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

36. 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. 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.

37. Bibliography (cont’d) Kingery WS. Pain. A critical review of controlled clinical trials for peripheral neuropathic pain and complex regional pain syndromes. 1997;73:123-139. Maleki J, LeBel AA, Bennett GJ, Shwartzman RJ. Patterns of spread in complex regional pain syndrome, type I (reflex sympathetic dystrophy). Pain. 2000;88:259-266. 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.

38. Reflex Sympathetic Dystrophy Syndrome Association Promotes public and professional awareness of RSD and educates patients, their families, friends, insurance and healthcare providers on the disabling pain it causes Encourages those with RSD/CRPS to offer each other emotional support within affiliate groups Raises funds for research PO Box 502 Milford, CT 06460 (877) 662-7737