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Complex Regional Pain Syndrome
Umesh D. Oza, MD
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KEY FACTS

  • Terminology

    • Imaging

      TERMINOLOGY

      • Abbreviations

        • Complex regional pain syndrome (CRPS)
      • Definitions

        • Painful disorder of extremities
          • Usually due to injury, surgery, vascular event
          • Usually unilateral; bilateral in ~ 25%
        • Once thought to be mediated by sympathetic nervous system (not proven)
        • Current thought: Likely neurological disorder affecting vascular system, pain receptors
          • CRPS type 1
            • No detectable nerve lesion
            • Reflex sympathetic dystrophy
          • CRPS type 2
            • Detectable nerve lesion
            • Causalgia
        • Staging
          • Stage 1: Extremity pain characterized by aching, throbbing, burning, cold/touch intolerance, swelling
            • Early, hyperemic stage
            • Pain, sensory abnormalities predominate
          • Stage 2: Muscle wasting, ↑ soft tissue edema, brawny skin, ↑ pain, vasomotor abnormalities
            • Dystrophic stage
            • 3-6 months after onset of stage 1
            • May last weeks, months
          • Stage 3: ↓ range of motion, digit/joint contracture, waxy skin, brittle, ridged nails, vasomotor abnormalities, ↓ pain
            • Atrophic stage
            • Permanent
            • 6 months to 1 year duration of symptoms

      IMAGING

      • General Features

        • Nuclear Medicine Findings

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. AlSharif A et al: Is there a correlation between symptoms and bone scintigraphic findings in patients with complex regional pain syndrome? Ann Nucl Med. 26(8):665-9, 2012
                                    2. Cappello ZJ et al: Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. J Hand Surg Am. 37(2):288-96, 2012
                                    3. Hsu W et al: Radionuclide imaging in the diagnosis and management of orthopaedic disease. J Am Acad Orthop Surg. 20(3):151-9, 2012
                                    4. Moon JY et al: Analysis of patterns of three-phase bone scintigraphy for patients with complex regional pain syndrome diagnosed using the proposed research criteria (the 'Budapest Criteria'). Br J Anaesth. 108(4):655-61, 2012
                                    5. Ringer R et al: Concordance of qualitative bone scintigraphy results with presence of clinical complex regional pain syndrome 1: meta-analysis of test accuracy studies. Eur J Pain. 16(10):1347-56, 2012
                                    6. Wüppenhorst N et al: Sensitivity and specificity of 3-phase bone scintigraphy in the diagnosis of complex regional pain syndrome of the upper extremity. Clin J Pain. 26(3):182-9, 2010
                                    7. Park SA et al: Patterns of three-phase bone scintigraphy according to the time course of complex regional pain syndrome type I after a stroke or traumatic brain injury. Clin Nucl Med. 34(11):773-6, 2009
                                    8. Park SG et al: Quantitative evaluation of very acute stage of complex regional pain syndrome after stroke using three-phase bone scintigraphy. Nucl Med Commun. 28(10):766-70, 2007
                                    9. Harden RN et al: Diagnosis of complex regional pain syndrome: signs, symptoms, and new empirically derived diagnostic criteria. Clin J Pain. 22(5):415-9, 2006
                                    10. Lee E et al: Role of radionuclide imaging in the orthopedic patient. Orthop Clin North Am. 37(3):485-501, viii, 2006
                                    11. Shehab D et al: Impact of three-phase bone scintigraphy on the diagnosis and treatment of complex regional pain syndrome type I or reflex sympathetic dystrophy. Med Princ Pract. 15(1):46-51, 2006
                                    12. Wilder RT: Management of pediatric patients with complex regional pain syndrome. Clin J Pain. 22(5):443-8, 2006
                                    13. Intenzo CM et al: The role of nuclear medicine in the evaluation of complex regional pain syndrome type I. Clin Nucl Med. 30(6):400-7, 2005
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Complex regional pain syndrome (CRPS)
                                        • Definitions

                                          • Painful disorder of extremities
                                            • Usually due to injury, surgery, vascular event
                                            • Usually unilateral; bilateral in ~ 25%
                                          • Once thought to be mediated by sympathetic nervous system (not proven)
                                          • Current thought: Likely neurological disorder affecting vascular system, pain receptors
                                            • CRPS type 1
                                              • No detectable nerve lesion
                                              • Reflex sympathetic dystrophy
                                            • CRPS type 2
                                              • Detectable nerve lesion
                                              • Causalgia
                                          • Staging
                                            • Stage 1: Extremity pain characterized by aching, throbbing, burning, cold/touch intolerance, swelling
                                              • Early, hyperemic stage
                                              • Pain, sensory abnormalities predominate
                                            • Stage 2: Muscle wasting, ↑ soft tissue edema, brawny skin, ↑ pain, vasomotor abnormalities
                                              • Dystrophic stage
                                              • 3-6 months after onset of stage 1
                                              • May last weeks, months
                                            • Stage 3: ↓ range of motion, digit/joint contracture, waxy skin, brittle, ridged nails, vasomotor abnormalities, ↓ pain
                                              • Atrophic stage
                                              • Permanent
                                              • 6 months to 1 year duration of symptoms

                                        IMAGING

                                        • General Features

                                          • Nuclear Medicine Findings

                                            • Radiographic Findings

                                              • CT Findings

                                                • MR Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Gross Pathologic & Surgical Features

                                                          • Microscopic Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. AlSharif A et al: Is there a correlation between symptoms and bone scintigraphic findings in patients with complex regional pain syndrome? Ann Nucl Med. 26(8):665-9, 2012
                                                                      2. Cappello ZJ et al: Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. J Hand Surg Am. 37(2):288-96, 2012
                                                                      3. Hsu W et al: Radionuclide imaging in the diagnosis and management of orthopaedic disease. J Am Acad Orthop Surg. 20(3):151-9, 2012
                                                                      4. Moon JY et al: Analysis of patterns of three-phase bone scintigraphy for patients with complex regional pain syndrome diagnosed using the proposed research criteria (the 'Budapest Criteria'). Br J Anaesth. 108(4):655-61, 2012
                                                                      5. Ringer R et al: Concordance of qualitative bone scintigraphy results with presence of clinical complex regional pain syndrome 1: meta-analysis of test accuracy studies. Eur J Pain. 16(10):1347-56, 2012
                                                                      6. Wüppenhorst N et al: Sensitivity and specificity of 3-phase bone scintigraphy in the diagnosis of complex regional pain syndrome of the upper extremity. Clin J Pain. 26(3):182-9, 2010
                                                                      7. Park SA et al: Patterns of three-phase bone scintigraphy according to the time course of complex regional pain syndrome type I after a stroke or traumatic brain injury. Clin Nucl Med. 34(11):773-6, 2009
                                                                      8. Park SG et al: Quantitative evaluation of very acute stage of complex regional pain syndrome after stroke using three-phase bone scintigraphy. Nucl Med Commun. 28(10):766-70, 2007
                                                                      9. Harden RN et al: Diagnosis of complex regional pain syndrome: signs, symptoms, and new empirically derived diagnostic criteria. Clin J Pain. 22(5):415-9, 2006
                                                                      10. Lee E et al: Role of radionuclide imaging in the orthopedic patient. Orthop Clin North Am. 37(3):485-501, viii, 2006
                                                                      11. Shehab D et al: Impact of three-phase bone scintigraphy on the diagnosis and treatment of complex regional pain syndrome type I or reflex sympathetic dystrophy. Med Princ Pract. 15(1):46-51, 2006
                                                                      12. Wilder RT: Management of pediatric patients with complex regional pain syndrome. Clin J Pain. 22(5):443-8, 2006
                                                                      13. Intenzo CM et al: The role of nuclear medicine in the evaluation of complex regional pain syndrome type I. Clin Nucl Med. 30(6):400-7, 2005