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
The Reflex Sympathetic Dystrophy Association of America (RSDSA) statistic show the average patient visits 4.8 physicians before being correctly diagnosed with RSD/CRPS.
Because many physicians are unaware of the clinical signs of RSD/CRPS, patients are frequently told their symptoms are phycosomatic.
Dr. Robert J. Schwartzman states that early diagnosis and treatment (within 6 months of onset) is vital for any hope of remissions.

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
RSD reflects the prevailing belief that conditions [characterized by pain associated with sensitivity to touchy, together with color, temperature and trophic changes] involved abnormal reflex activity in the sympathetic nervous system.
Causalgia is reserved for those conditions caused by an injury to a major peripheral nerve. 1
1 Bogduk N. Current Opinions in Anesthesiology. 2000;14:
Bogduk N. Current Opinions in Anesthesiology. 2000;14:

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
Lack of consensus and confusion re RSD and causalgia have retarded clinical research
Complex regional pain syndromes are difficult problems in treatment and diagnosis.
1 Bogduk N. Current Opinions in Anesthesiology. 2000;14:
2.Raja SN and Grabow TS. Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy). Anesthesiology :
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:
3. Raja SN et al. Anesthesiology. 2002;96:

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:

6. Name Change to CRPS
Goals: standardized, reliable diagnostic criteria and decision rules
Allow generalization
Make appropriate treatment selection
Identify reproducible research samples
1 Galer et al
Term RSD changed to CRPS I because the lack of consensus and the confusion with regard to RSD and causalgia diagnostic criteria have retarded clinical research.
Little agreement existed among those from different medial fields and parts of the worked as to the diagnositic criteria and appropriate therapies for RSD and causalgia.
Medical folklore exists re RSD and causalgia based on anecdotal personal physician experiences that hove not been proven scientifically or are inconsistent with more extensive worldwide clinical experience
Many patients do not demonstrate the classically described dystrophic signs (atrophy and changes in skin, nails, and hair).
Bonica’s Management of Pain. 3rd edition, Ed. John D. Loesser, a chapter by Galer BS, Schwartz L, Allen R. Complex Regional Pain Syndromes—type I: Reflex Sympathetic Dystrophy, and Type II: Causalgia.P 389
Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001:

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

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
Notes:
A US study from a chronic pain clinical reported only 77% of patients had a known event that resulted in the development of CRPS (strain or sprain, post surgical, contusion or crash injury)1
CRPS type II has the same clinical features as CRPS type I except for the present of clinical signs and history consistent with a nerve injury. 2
1. Bonica’s Management of Pain. 3rd edition, Ed. John D. Loesser, a chapter by Galer BS, Schwartz L, Allen R. Complex Regional Pain Syndromes—type I: Reflex Sympathetic Dystrophy, and Type II: Causalgia.P 389
2.Raja SC and Grabow TS. Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy). Anesthesiology :
Stanton-Hicks M et al. Pain. 1995;63: Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001;

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

10. Checklist for the Diagnosis of RSD: 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:

11. 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 < 1ْ C)
Limited range of movement
Motor neglect
Bogduk N. Current Opinions in Anesthesiology. 2000;14:

12. Revised Diagnostic Criteria 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: Harden et al. Pain. 1999;83: .

13. Swelling and Color Changes

14. Abnormal Sweating in RSD

15. 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:

16. Psychological Aspects
Pain can cause symptoms of psychologic distress including
Anxiety
Depression
Fear
Anger
Notes:
Most patients with RSD/CRPS suffer some form of psychologic distress. The symptoms listed above are deemed to be the result of the pain associated with RSD/CRPS, not the cause of it. The signs and symptoms of RSD/CRPS may get worse because of illness behaviors, such as disuse and immobilization, or by maladaptive coping skills.
Raja SC and Grabow TS. Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy). Anesthesiology :
Raja SN et al. Anesthesiology. 2002;96:

17. Treatment Goals Multidisciplinary Rehabilitation Pain management
Psychological treatment
Multidisciplinary
Physiotherapy
Medical
Psychological
Notes:
No scientifically established treatments exist for RSD/CRPS. A few treatments have some controlled trial data suggesting efficacy, no treatment cures RSD/CRPS.
Many treatments for RSD/CRPS have been reported or recommended, but the majority are descriptive and anecdotal, not based on the results of controlled clinical trials.
Galer et al. pg 400
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

18. 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.

19. 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:

20. Pharmacalogic Pain Management
Most drugs used for neuropathic pain are used to treat RSD/CRPS
IV bretylium
IV ketanserin
IV phentolamine
IV lidocaine
Intranasal calcitonin
IV alendronate (bisphosphonate)
Topic dimethyl sulfoxide
Topical clonidine
Notes:
Few placebo-controlled trials have shown therapeutic efficacy of analgesics for patients with CRPS regardless of route of injection or drug delivery technique.
Raja SN et al. Anesthesiology. 2002;96:
Kingery WS. Pain.1997;73:

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
Notes
Nerve blocks can reduce pain and aid physiotherapy and functional rehabilitation.
Historically, interventions that interrupt sympathetic nervous system or adrenergic receptor function have been used to treat RSD/CRPS. However, there is little evidence-based information regarding timing, number, necessity, or appropriateness of nerve blocks for RSD/CRPS
Raja SC and Grabow TS. Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy). Anesthesiology :
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.

22. More Invasive Therapies
Neuroaugmentation
Spinal cord stimulation
Intrathecal drug delivery
Notes:
Neuroaugmentation includes neurostimulation.
Spinal cord stimulation has produced analgesic results
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
Notes:
The sympathectomy is very controversial but may be used in patients with SMP who respond to sympathetic blockade
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.
Bandyk DF et al. J Vasc Surg. 2002;35:

24. Other Therapies Behavioral modification Psychiatric consultation
Complimentary and Alternative therapies
Acupuncture
Few placebo-controlled trials have shown therapeutic efficacy of analgesics for patients with CRPS regardless of route of injection or drug delivery technique.
Raja SN et al. Anesthesiology. 2002; 96:

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:

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:
Bogduk N. Complex regional pain syndrome. Current Opinions in Anesthesiology. 2000;14:
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:
Galer BS, Schwartz L, Allen RJ. In: Loeser, ed. Bonica’s Management of Pain. 2001:
Harden RN, Bruehl SP, Galer BS, et al. Complex regional pain syndrome: are the IASP diagnostic criteria valid and sufficiently comprehensive? Pain. 1999;83:

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:
Raja SN , Grabow TS. Complex regional pain syndrome I (Reflex Sympathetic Dystrophy) Anesthesiology. 2002;96:
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:
Stanton-Hicks M, Baron R, Boas R, et al. Complex Regional Pain Syndrome: guidelines for therapy. Clin J Pain. 1998;14: