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Complex Regional Pain Syndrome: Differential Diagnoses & Workup

Author: Steven J Parrillo, DO, FACOEP, FACEP, Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Medical Director, Disaster Medicine and Management Masters Program, Philadelphia University
Contributor Information and Disclosures

Updated: Oct 26, 2009

Differential Diagnoses

Deep Venous Thrombosis and Thrombophlebitis
Thoracic Outlet Syndrome

Other Problems to Be Considered

Improperly placed splints or casts
Primary neurologic problems, such as carpal tunnel syndrome
Pain and/or edema from fractures or sprains

Workup

Laboratory Studies

  • A single, reliable, sensitive, and specific diagnostic test for reflex sympathetic dystrophy syndrome (RSDS) is not available.
  • Quantitative sensory testing and quantitative sudomotor axon reflex test (QSART) may be performed to look for sensory and sweating abnormalities.19

Imaging Studies

  • A 3-phase bone scan and gadolinium magnetic resonance imaging (MRI) have been used to diagnose and stage the disease.
  • Standard radiograph findings are normal in as many as 30% of patients. However, they may show osteoporosis as soon as 3-5 weeks of onset.
  • Laser Doppler flow studies have been used to monitor background vasomotor control.
  • A cold pressor test performed in conjunction with thermographic imaging observes vasoconstrictor response.
  • Functional MRI (fMRI) has been used to demonstrate that allodynic stimulation produces objective findings.19

Procedures

  • Many authors believe that the best diagnostic approach involves use of differential neural blockade. In those with sympathetically mediated pain (as opposed to those whose pain is sympathetically independent), response to neural blockade may help guide medical therapy.
    • For cases involving an upper extremity, a stellate ganglion block may be diagnostic and therapeutic. However, failure to relieve pain does not eliminate the diagnosis.1,15
    • Differential blockade has been performed using Bier blocks with a variety of agents, including local anesthetics, bretylium, steroids, ketorolac, reserpine, and guanethidine and clonidine.15,20
    • The rationale for selective neural blockade is to interrupt stimulation to the sympathetic nervous system. Again, this is effective only in those whose pain is sympathetically dependent.15

More on Complex Regional Pain Syndrome

Overview: Complex Regional Pain Syndrome
Differential Diagnoses & Workup: Complex Regional Pain Syndrome
Treatment & Medication: Complex Regional Pain Syndrome
Follow-up: Complex Regional Pain Syndrome
References

References

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  2. Campbell JN, Meyer RA, Raja SN. Is nociceptor activation by alpha-1 adrenoreceptors the culprit in sympathetically mediated pain?. Am Pain Soc J. 1992;1:3-11.

  3. [Guideline] Reflex Sympathetic Dystrophy Syndrome Association (RSDSA). Complex regional pain syndrome: treatment guidelines. Jun 2006;[Full Text].

  4. Maihofner C, Handwerker HO, Neundorfer B, Birklein F. Mechanical hyperalgesia in complex regional pain syndrome: a role for TNF-alpha?. Neurology. Jul 26 2005;65(2):311-3. [Medline].

  5. Blaes F, Schmitz K, Tschernatsch M, et al. Autoimmune etiology of complex regional pain syndrome (M. Sudeck). Neurology. Nov 9 2004;63(9):1734-6. [Medline].

  6. Oaklander AL, Fields HL. Is reflex sympathetic dystrophy/complex regional pain syndrome type I a small-fiber neuropathy?. Ann Neurol. Jun 2009;65(6):629-38. [Medline].

  7. de Mos M, Huygen FJ, Stricker BH, Dieleman JP, Sturkenboom MC. The association between ACE inhibitors and the complex regional pain syndrome: Suggestions for a neuro-inflammatory pathogenesis of CRPS. Pain. Apr 2009;142(3):218-24. [Medline].

  8. Schwartzman RJ, Erwin KL, Alexander GM. The natural history of complex regional pain syndrome. Clin J Pain. May 2009;25(4):273-80.

  9. Stanton-Hicks M. Complex regional pain syndrome. Anesthesiol Clin North America. Dec 2003;21(4):733-44. [Medline].

  10. Tong HC, Nelson VS. Recurrent and migratory reflex sympathetic dystrophy in children. Pediatr Rehabil. Apr-Jun 2000;4(2):87-9. [Medline].

  11. Kachko L, Efrat R, Ben Ami S, Mukamel M, Katz J. Complex regional pain syndromes in children and adolescents. Pediatr Int. Aug 2008;50(4):523-7. [Medline].

  12. Merskey H, Bogduk N, eds. International Association for the Study of Pain. Classification of chronic pain: descriptions of chronic pain syndromes and definitions of pain terms. IASP Press; 1996.

  13. Lankford LL. Reflex sympathetic dystrophy. In: Hunter JM, et al, eds. Rehabilitation of the Hand. Mosby-Year Book; 1990:763-86.

  14. van Hilten JJ, van de Beek WJ, Vein AA, van Dijk JG, Middelkoop HA. Clinical aspects of multifocal or generalized tonic dystonia in reflex sympathetic dystrophy. Neurology. Jun 26 2001;56(12):1762-5. [Medline].

  15. Schwartzman RJ. New treatments for reflex sympathetic dystrophy. N Engl J Med. Aug 31 2000;343(9):654-6. [Medline].

  16. Beerthuizen A, van 't Spijker A, Huygen FJ, Klein J, de Wit R. Is there an association between psychological factors and the Complex Regional Pain Syndrome type 1 (CRPS1) in adults? A systematic review. Pain. Sep 2009;145(1-2):52-9. [Medline].

  17. Peterlin BL, Rosso AL, Nair S, Young WB, Schwartzman RJ. Migraine may be a risk factor for the development of complex regional pain syndrome. Cephalalgia. Jul 9 2009;[Medline].

  18. Borsook D, Sava S. Pain: Do ACE inhibitors exacerbate complex regional pain syndrome?. Nat Rev Neurol. Jun 2009;5(6):306-8. [Medline].

  19. Maihofner C, Handwerker HO, Birklein F. Functional imaging of allodynia in complex regional pain syndrome. Neurology. Mar 14 2006;66(5):711-7. [Medline].

  20. Dadure C, Motais F, Ricard C, Raux O, Troncin R, Capdevila X. Continuous peripheral nerve blocks at home for treatment of recurrent complex regional pain syndrome I in children. Anesthesiology. Feb 2005;102(2):387-91. [Medline].

  21. Markman JD, Philip A. Interventional approaches to pain management. Anesthesiol Clin. Dec 2007;25(4):883-98, viii. [Medline].

  22. Kemler MA, Reulen JP, Barendse GA, van Kleef M, de Vet HC, van den Wildenberg FA. Impact of spinal cord stimulation on sensory characteristics in complex regional pain syndrome type I: a randomized trial. Anesthesiology. Jul 2001;95(1):72-80. [Medline].

  23. Oakley JC, Weiner RL. Spinal cord stimulation in complex regional pain syndrome: a prospective study of 19 patients at 2 centers. Neuromodulation. 1999;2:47-50.

  24. Kemler MA, Rijks CP, de Vet HC. Which patients with chronic reflex sympathetic dystrophy are most likely to benefit from physical therapy?. J Manipulative Physiol Ther. May 2001;24(4):272-8. [Medline].

  25. Cleary AG, Sills JA, Davidson JE, Cohen AM. Reflex sympathetic dystrophy. Rheumatology (Oxford). May 2001;40(5):590-1. [Medline].

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  27. Reuben SS, Rosenthal EA, Steinberg RB, Faruqi S, Kilaru PA. Surgery on the affected upper extremity of patients with a history of complex regional pain syndrome: the use of intravenous regional anesthesia with clonidine. J Clin Anesth. Nov 2004;16(7):517-22. [Medline].

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  30. Finch PM, Knudsen L, Drummond PD. Reduction of allodynia in patients with complex regional pain syndrome: A double-blind placebo-controlled trial of topical ketamine. Pain. Nov 2009;146(1-2):18-25. [Medline].

  31. Sigtermans MJ, van Hilten JJ, Bauer MC, Arbous MS, Marinus J, Sarton EY, et al. Ketamine produces effective and long-term pain relief in patients with Complex Regional Pain Syndrome Type 1. Pain. Oct 2009;145(3):304-11. [Medline].

  32. Collins S, Zuurmond WW, de Lange JJ, van Hilten BJ, Perez RS. Intravenous magnesium for complex regional pain syndrome type 1 (CRPS 1) patients: a pilot study. Pain Med. Jul-Aug 2009;10(5):930-40. [Medline].

  33. Hsu ES. Practical management of complex regional pain syndrome. Am J Ther. Mar-Apr 2009;16(2):147-54. [Medline].

  34. Karmarkar A, Lieberman I. Management of complex regional pain syndrome type II using lidoderm 5% patches. Br J Anaesth. Feb 2007;98(2):261-2. [Medline].

Further Reading

Keywords

complex regional pain syndrome symptoms, complex regional pain syndrome treatment, RSDSRSDreflex sympathetic dystrophy syndrome, causalgia, sympathetic maintained pain syndrome, complex regional pain syndrome, CRPS, CRPS I, CRPS II, peripheral nerve injury

Contributor Information and Disclosures

Author

Steven J Parrillo, DO, FACOEP, FACEP, Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Medical Director, Disaster Medicine and Management Masters Program, Philadelphia University
Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Medical Editor

Joseph A Salomone III, MD, EMS Medical Director, Kansas City, Missouri; Associate Professor and Staff Physician, Truman Medical Centers/UMKC School of Medicine
Joseph A Salomone III, MD is a member of the following medical societies: American Academy of Emergency Medicine, National Association of EMS Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

J Stephen Huff, MD, Associate Professor, Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia Health Sciences Center
J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Robert E O'Connor, MD, MPH, Professor and Chair, Department of Emergency Medicine, University of Virginia Health System
Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American College of Physician Executives, American Heart Association, American Medical Association, Medical Society of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.

 
 
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