Complex regional pain syndrome
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Complex regional pain syndrome (CRPS), also known as Sudeck atrophy, is a condition that can affect the extremities in a wide clinical spectrum. No one imaging study is sensitive or specific to rule in or rule out the syndrome.
Two forms of complex regional pain syndrome have been described 8:
type 1: no underlying single nerve lesion (formerly known as reflex sympathetic dystrophy) and considered the commonest type 21
type 2: underlying nerve lesion identified (formerly known as causalgia)
Patients often have both types present.
Complex regional pain syndrome is principally a clinical diagnosis seen more commonly in females than males with a mean age of presentation of 50 to 70 years 12.
Patients present after an initiating event (see causes below) with symptoms of more than 6 months duration such as edema, changes in skin blood flow, abnormal motor activity, allodynia, hyperalgesia or hyperesthesia 11, 21. Symptoms are often out of proportion to the initiating event and not limited to a single peripheral nerve 8.
trauma: often minor
unknown in many cases
Complex regional pain syndrome occurs in hands and feet distal to the injury.
severe patchy osteopenia, particularly in the periarticular region
soft tissue swelling, with eventual soft tissue atrophy
preservation of joint space
It is important to differentiate this from disuse osteopenia since the clinician could initiate aggressive physical therapy for the latter.
soft tissue edema and enhancement
muscle atrophy in later stages
presence of complex regional pain syndrome can be evaluated with a 3-phase bone scan:
the classical presentation is increased bisphosphonate tracer uptake in the affected limb on all three phases (seen less than 50% of the time, but has the highest diagnostic accuracy)
diffusely increased juxta-articular activity around all joints of the affected hand or foot on delayed images is the most sensitive indicator (infection and arthritis are potential false-positive findings)
Treatment and prognosis
In most cases of complex regional pain syndrome, a multidisciplinary approach is required whereby a combination of various treatments may be employed, such as physical therapy, systemic or regional medications, sympathectomy or spinal cord stimulation, and psychotherapy.
Interventional radiology can offer pain relief by peripheral nerve block procedures.
History and etymology
Paul Hermann Martin Sudeck (1866-1945) 17 was a German surgeon who described this condition in 1900 16,18,19. He was erroneously said to be an early radiologist in an article published in 1994, but this is probably because his paper in 1900 was the first to describe the characteristic osteopenia seen on radiographs in this condition 16,20.
- 1. Kozin F, Soin JS, Ryan LM et-al. Bone scintigraphy in the reflex sympathetic dystrophy syndrome. Radiology. 1981;138 (2): 437-43. Radiology (citation) - Pubmed citation
- 2. Hayes CW, Conway WF, Daniel WW. MR imaging of bone marrow edema pattern: transient osteoporosis, transient bone marrow edema syndrome, or osteonecrosis. Radiographics. 1993;13 (5): 1001-11. Radiographics (citation) - Pubmed citation
- 3. Helms CA, O'brien ET, Katzberg RW. Segmental reflex sympathetic dystrophy syndrome. Radiology. 1980;135 (1): 67-8. Radiology (citation) - Pubmed citation
- 4. Koch E, Hofer HO, Sialer G et-al. Failure of MR imaging to detect reflex sympathetic dystrophy of the extremities. AJR Am J Roentgenol. 1991;156 (1): 113-5. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Guiberteau MJ. Essentials of Nuclear Medicine Imaging: Expert Consult - Online and Print, 6e. Saunders. ISBN:1455701041. Read it at Google Books - Find it at Amazon
- 6. Manaster B, Petersilge CA, Roberts CC et-al. Diagnostic Imaging. Lippincott Williams & Wilkins. (2010) ISBN:193188479X. Read it at Google Books - Find it at Amazon
- 7. Schweitzer ME, Mandel S, Schwartzman RJ et-al. Reflex sympathetic dystrophy revisited: MR imaging findings before and after infusion of contrast material. Radiology. 1995;195 (1): 211-4. doi:10.1148/radiology.195.1.7892471 - Pubmed citation
- 8. Perez RS, Zollinger PE, Dijkstra PU et-al. Evidence based guidelines for complex regional pain syndrome type 1. BMC Neurol. 2010;10 (1): 20. doi:10.1186/1471-2377-10-20 - Free text at pubmed - Pubmed citation
- 9. Lia Angela Moulopoulos, Vassilis Koutoulidis. Bone Marrow MRI. (2014) ISBN: 9788847053168
- 10. Marsland, Daniel & Konyves, Arpad & Cooper, R. & Suvarna, Kim. (2008). Type I complex regional pain syndrome: MRI may be misleading. Injury Extra. 39. 102-105. doi:10.1016/j.injury.2007.09.012
- 11. Crane M, Crane. Complex regional pain syndrome. (2019) JAAPA : official journal of the American Academy of Physician Assistants. doi:10.1097/01.JAA.0000578792.77298.8a - Pubmed
- 12. Rand S, Rand BS, Rand KS, Rand. Complex Regional Pain Syndrome: Current Diagnostic and Treatment Considerations. (2019) Current sports medicine reports. doi:10.1249/JSR.0000000000000633 - Pubmed
- 13. Neumeister M, Neumeister RM, Neumeister. Complex Regional Pain Syndrome. (2020) Clinics in plastic surgery. doi:10.1016/j.cps.2019.12.009 - Pubmed
- 14. Janis P. O'Malley, Harvey A. Ziessman. Nuclear Medicine and Molecular Imaging: The Requisites E-Book. (2020) ISBN: 9780323550741
- 15. Dey S, Guthmiller K, Varacallo M. Complex Regional Pain Syndrome. 2021. - Pubmed
- 16. Sudeck PHM. Ueber die acute entzundliche Knochenatophie. Arch Klin Chir 1900; 62: 147-56.
- 17. Thomas Lathrop Stedman. Stedman's Medical Eponyms. (2005) ISBN: 9780781754439 - Google Books
- 18. Howat D. Paul Sudeck--His Contribution to Anaesthesia. Anaesthesia. 1989;44(10):847-50. doi:10.1111/j.1365-2044.1989.tb09107.x - Pubmed
- 19. Reme H. [Paul Sudeck's Work and the Present State of the Teachings on Sudeck's Syndrome]. Bruns Beitr Klin Chir. 1955;191(2):228-43. - Pubmed
- 20. Cooke E. Reflex Sympathetic Dystrophy. Vascular Medicine Review. 1994;vmr-5(4):319-30. doi:10.1177/1358863x9400500405
- 21. Kim Y. Diagnosis of Complex Regional Pain Syndrome. ACN. 2022;24(2):35-45. doi:10.14253/acn.2022.24.2.35