Complex regional pain syndrome underdiagnosed

,; ,; ,

Applied Evidence

Complex regional pain syndrome underdiagnosed

J Fam Pract. 2005 June;54(6):524-532

By

Anna Quisel, MD

James M Gill, MD, MPH

Peter Witherell, MD

CRPS type 1 is an under-recognized problem in limbs recovering from fracture or immobilized post-stroke.

PDF Download

References

1. Allen G, Galer BS, Schwartz L. Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain 1999;80:539-544.

2. Birklein F, Kunzel W, Sieweke N. Despite clinical similarities there are significant differences between acute limb trauma and complex regional pain syndrome I (CRPS I). Pain 2001;93:165-171.

3. Schurmann M, Gradl G, Andress HJ, Furst H, Schildberg FW. Assessment of peripheral sympathetic nervous function for diagnosing early posttraumatic complex regional pain syndrome type I. Pain 1999;80:149-159.

4. Stanton-Hicks M, Janig W, Hassenbusch S, Haddox JD, Boas R, Wilson P. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995;63:127-133.

5. Lynch ME. Psychological aspects of reflex sympathetic dystrophy: a review of the adult and paediatric literature. Pain 1992;49:337-347.

6. Field J, Atkins R. Algodystrophy is an early complication of Colles’ fracture: What are the implications. J Hand Surg Br 1997;22B(2):178-182.

7. Reinders MF, Geertzen JH, Dijkstra PU. Complex regional pain syndrome type I: use of the International Association for the Study of Pain diagnostic criteria defined in 1994. Clin J Pain 2002;18:207-215.

8. Atkins R, Duckworth T, Kanis JA. Algodystrophy following Colles’ fracture. J Hand Surg Br 1989;14:161-164.

9. Baron R, Fields HL, Janig W, Kitt C, Levine JD. National Institutes of Health Workshop: reflex sympathetic dystrophy/complex regional pain syndromes— state-of-the-science. Anesth Analg 2002;95:1812-1816.

10. van de Beek WJ, Schwartzman RJ, van Nes SI, Delhaas EM, van Hilten JJ. Diagnostic criteria used in studies of reflex sympathetic dystrophy. Neurology 2002;58:522-526.

11. Bruehl S, Harden RN, Galer BS, et al. External validation of IASP diagnostic criteria for Complex Regional Pain Syndrome and proposed research diagnostic criteria. International Association for the Study of Pain. Pain 1999;81:147-154.

12. Galer BS, Bruehl S, Harden RN. IASP diagnostic criteria for complex regional pain syndrome: a preliminary empirical validation study. Clin J Pain 1998;14:48-54.

13. Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet 1993;342:1012-1016.

14. Oerlemans HM, Oostendorp RA, de Boo T, Perez RS, Goris RJ. Signs and symptoms in complex regional pain syndrome type I/reflex sympathetic dystrophy: judgment of the physician versus objective measurement. Clin J Pain 1999;15:224-232.

15. van de Vusse AC, Stomp-van den Berg SG, de Vet HC, Weber WE. Interobserver reliability of diagnosis in patients with complex regional pain syndrome. Eur J Pain 2003;7:259-265.

16. Perez RS, Burm PE, Zuurmond WW, et al. Interrater reliability of diagnosing complex regional pain syndrome type I. Acta Anaesthesiologica Scandinavica 2002;46:447-450.

17. Harden RN, Bruehl S, Galer BS, et al. Complex regional pain syndrome: are the IASP diagnostic criteria valid and sufficiently comprehensive? Pain 1999;83:211-219.

18. Wasner G, Schattschneider J, Baron R. Skin temperature side differences—a diagnostic tool for CRPS? Pain 2002;98:19-26.

19. Sandroni P, Low PA, Ferrer T, Opfer-Gehrking TL, Willner CL, Wilson PR. Complex regional pain syndrome I (CRPS I): prospective study and laboratory evaluation. Clin J Pain 1998;14:282-289.

20. Kingery WS. A critical review of controlled clinical trials for peripheral neuropathic pain and complex regional pain syndromes. Pain 1997;73:123-139.

21. Perez RS, Kwakkel G, Zuurmond WW, de Lange JJ. Treatment of reflex sympathetic dystrophy (CRPS type 1). a research synthesis of 21 randomized clinical trials. J Pain Symptom Manage 2001;21:511-526.

22. Cepeda MS, Lau J, Carr DB. Defining the therapeutic role of local anesthetic sympathetic blockade in complex regional pain syndrome: a narrative and systematic review. Clin J Pain 2002;18:216-233.

23. Demangeat JL, Constantinesco A, Brunot B, Foucher G, Farcot JM. Three-phase bone scanning in reflex sympathetic dystrophy of the hand. J Nucl Med 1988;29:26-32.

24. Lee GW, Weeks PM. The role of bone scintigraphy in diagnosing reflex sympathetic dystrophy [comment]. J Hand Surg Amer 1995;20:458-463.

25. Schiepers C, Bormans I, De Roo M. Three-phase bone scan and dynamic vascular scintigraphy in algoneurodystrophy of the upper extremity. Acta Orthop Belg 1998;64:322-327.

26. Todorovic-Tirnanic M, Obradovic V, Han R, et al. Diagnostic approach to reflex sympathetic dystrophy after fracture: radiography or bone scintigraphy? Eur J Nuclear Med 1995;22:1187-1193.

27. Zyluk A. The usefulness of quantitative evaluation of three-phase scintigraphy in the diagnosis of posttraumatic reflex sympathetic dystrophy. J Hand Surg 1999;24:16-21.

28. Chapurlat RD, Duboeuf FP, Liens D, Meunier PJ. Dual energy x-ray absorptiometry in patients with low limb reflex sympathetic dystrophy syndrome. J Rheumatol 1996;23:1557-1559.

29. Murray CS, Cohen A, Perkins T, Davidson JE, Sills JA. Morbidity in reflex sympathetic dystrophy. Arch Dis Child 2000;82:231-233.

30. Wesdock KA, Stanton RP, Singsen BH. Reflex sympathetic dystrophy in children. A physical therapy approach. Arthritis Care Res 1991;4:32-38.

31. Sandroni P, Benrud-Larson LM, McClelland RL, Low PA. Complex regional pain syndrome type I: incidence and prevalence in Olmsted county, a population-based study. Pain 2003;103:199-207.

32. Hove LM. Nerve entrapment and reflex sympathetic dystrophy after fractures of the distal radius. Scan J. Plast Resconstr Surg Hand Surg 1995;29:53-58.

33. Schurmann M, Gradl G, Zaspel J, Kayser M, Lohr P, Andress HJ. Peripheral sympathetic function as a predictor of complex regional pain syndrome type I (CRPS I) in patients with radial fracture. Auton Neurosci 2000;86:127-134.

34. Bickerstaff DR, Kanis JA. Algodystrophy: an underrecognized complication of minor trauma. Br J Rheumatol 1994;33:240-248.

35. Sarangi PP, Ward AJ, Smith EJ, Staddon GE, Atkins RM. Algodystrophy and osteoporosis after tibial fractures. J Bone Joint Surg Br 1993;75:450-452.

36. Greyson ND, Tepperman PS. Three-phase bone studies in hemiplegia with reflex sympathetic dystrophy and the effect of disuse. J Nucl Med 1984;25:423-429.

37. Wang YL, Tsau JC, Huang MH, Lee BH, Li CH. Reflex sympathetic dystrophy syndrome in stroke patients with hemiplegia-three phase bone scintigraphy and clinical characteristics. Kaohsiung J Med Sci 1998;14:40-47.

38. Petchkrua W, Weiss DJ, Patel RR. Reassessment of the incidence of complex regional pain syndrome type 1 following stroke. Neurorehabil Neural Repair 2000;14:59-63.

39. Geertzen JH, Dijkstra PU, van Sonderen EL, Groothoff JW, ten Duis HJ, Eisma WH. Relationship between impairments, disability and handicap in reflex sympathetic dystrophy patients: a long-term follow up study. Clin Rehabil 1998;12:402-412.

40. van de Beek WJ, Roep BO, van der Slik AR, Giphart MJ, van Hilten BJ. Susceptibility loci for complex regional pain syndrome. Pain 2003;103:93-97.

41. Commentary on RSD focus article Bandolier 2002. Available at: www.jr2.ox.ac.uk/bandolier/booth/ painpag/wisdom/RSD.html. Accessed on May 17, 2005.

42. Alvarez-Lario B, Aretxabala-Alcibar I, Alegre-Lopez J, Alonso-Valdivielso JL. Acceptance of the different denominations for reflex sympathetic dystrophy. Ann Rheum Dis 2001;60:77-79.

Practice recommendations

Complex regional pain syndrome (CRPS) type 1 may be diagnosed by history and physical exam with no further testing (B). Several different diagnostic criteria have undergone validity testing: the 1993 IASP criteria, Bruehl’s criteria, and Veldman’s criteria; there is no compelling reason to recommend 1 set of criteria over the others (C).
Some cases of CRPS type 1 may be preventable. Some cases of CRPS type 1 in post-stroke upper extremity hemiplegia (also known as shoulder-hand syndrome) may be prevented by early inpatient rehabilitation (C) and avoidance of shoulder trauma to the affected arm (B). Some cases of post-fracture CRPS type 1 may be prevented with 500 mg vitamin C daily started upon diagnosis of fracture and continued through healing (B).

Do you have a patient recovering from a limb fracture who is complaining of pain and tenderness long after most patients with a similar injury would be symptom free? The problem may be an under-recognized one—complex regional pain syndrome (CRPS) type 1, also known as reflex sympathetic dystrophy. The problem is also encountered in immobilized limbs of post-stroke patients.

Persons with persistent post-traumatic pain eventually diagnosed with CRPS type 1 often undergo unnecessary testing resulting in inappropriate or delayed treatment.¹

Signs and symptoms typical of CRPS type 1 can also occur transiently with a normally recovering immobilized limb,^2,3 so diagnosis of CRPS type 1 is based on increasing severity and duration of signs and symptoms (level of evidence [LOE]: 3; consensus guidelines)⁴:

pain
hyperalgesia/allodynia (pain or exaggerated response resulting from a normally painless or only slightly painful stimulus)
joint stiffness
swelling
autonomic abnormalities (often sweating and temperature differences compared with the unaffected limb).

Diagnosis: Watch recovery course over first 9 weeks

Clinicians face a number of challenges in diagnosing CRPS type 1. No psychological or personality traits appear to predispose to CRPS type 1 (LOE: 2, lower-quality literature review).⁵ Fracture types and severity of injury among persons who develop CRPS type 1 are not significantly different from persons who recover normally (LOE: 2, case control studies).^6,7 The key is to remain alert to deviation from the normal course of recovery.

Studies have shown that 9 weeks post injury, persons with persistent pain, tenderness, swelling, joint stiffness (fingers and wrist), and sweating or temperature changes in the injured limb may have CRPS type 1 (LOE: 2, case series and case control studies).^6,8 In a prospective case series (n=109), no new cases of CRPS type 1 developed beyond 9 weeks (LOE: 2, case series).⁸

Diagnostic criteria: No consensus

No one test identifies all persons with CRPS type 1. There is no objective gold standard for diagnosis.⁹ Instead, researchers and clinicians must rely on clinically derived diagnostic criteria. Unfortunately, despite the development of diagnostic criteria by the IASP in 1994 (TABLE 1),⁴ experts have not reached consensus on the best method of diagnosis, and several different sets of diagnostic criteria are used.^7,10

Initial IASP criteria.Of these, the 1994 IASP consensus-based diagnostic criteria appear to be most widely used in the litera- ture. These criteria were intended as a starting point, requiring validation through future clinical research.^4,11 In further studies using controls with neuropathic conditions, IASP criteria have demonstrated low specificity (TABLE 2 ).^11,12

CRPS underdiagnosed

The diagnosis of CRPS type I is often missed,1,29,30so it is likely that the diagnosis rate per population of 0.02% reported in a recent population based study is an underestimate of the actual prevalence.³¹ After distal radial fracture, rates of CRPS type I have varied widely in reports, from 0.9% ³² to 15% ³³ to 28%.³⁴ After tibial shaft fracture, Sarangi et al ³⁵ reported that 30% of persons developed CRPS type I.

In cases of post-stroke hemiplegia, CRPS type I has been reported in the paralyzed arm at rates between 25% ³⁶ and 40%.³⁷ However, in a more recent study among stroke patients in the US who underwent early inpatient rehabilitation, Petchkrua et al reported a lower incidence of about 2%.³⁸ Impairment can be severe among persons with persistent CRPS type 1. A prospective study revealed that activities of daily living were significantly impaired in 62% of persons with chronic CRPS type 1.³⁹

Criteria refinements.Derived from 1 of these studies, Bruehl’s criteria were subsequently developed to improve the IASP criteria (TABLE 1).¹¹Several other sets of diagnostic criteria exist, but only Veldman’s criteria ( TABLE 1),¹³ which have been adopted as the standard in the Netherlands, have undergone further study.¹⁴ Studies of Bruehl’s and IASP criteria have measured specificity and sensitivity, and along with Veldman’s criteria, interobserver reliability (TABLE 2 ).^11,12,14,15 However, these numbers must be interpreted with care due to the absence of an objective and independent gold standard.