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Complex Regional Pain Syndrome

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Other Names

  • Reflex dystrophy syndrome
  • Causalgia
  • Reflex Sympathetic Dystrophy (RSD)
  • Algodystrophy
  • Complex Regional Pain Syndrome (CRPS)

Background

  • This page refers to Complex Regional Pain Syndrome (CRPS), an umbrella term describing excess and prolonged pain and inflammation that follows an injury to the extremities

History

  • Very controversial disease
  • Ambroise Paré, the father of modern surgery, was the first to describe a disorder that could resemble current CRPS [1]
    • Successfully treated a severe and persistent pain syndrome in French King Charles IX of Valois after a limb phlebotomy
  • First written description was made by Denmark, a British surgeon that used to work at the Royal Navy Hospital in Hampshire in 1812 [1]

Epidemiology

  • Incidence between 5.5 - 26.2 cases per 100,000 people per year from two major studies [2]
    • Variation likely present due to use of different diagnostic criteria
    • Budapest Criteria have only been accepted and validated for the past decade (see below)

Pathophysiology

  • General
    • May be secondary to trauma, stroke, or heart attack
    • Pain is usually out of proportion (soft stimuli like touch will be painful)
    • Occurs acutely in ~7% of patients who have limb fractures or limb surgeries [3]
    • Many cases resolve within the first year [3]
    • May have genetic and psychological contributions
    • Diagnosis of exclusion

Definition

  • CRPS-I (previously known as RSD)
    • Historically diagnosed when there was uncertainty about the exact nerve injured [4]
    • Nerve injuries in CRPS I are thought to be more subtle
  • CRPS-II (previously known as causalgia) was diagnosed [4]
    • Labeled once the culprit nerve was identified
    • Nerve injuries in CRPS II are more debilitating and cause weakness and muscle shrinkage
  • Clinically, these are indistinguishable and follow a regional distribution instead of a dermatomal distribution
  • Can also be classified as “warm” CRPS where inflammatory characteristics are dominant or “cold” CRPS where autonomic characteristics are dominant [3]

Etiology

  • Most cases (>90%) are caused by improper function of the peripheral C-fiber nerve (unmyelinated afferent nerve fibers found in the somatic sensory system) [4]
  • It is unclear why certain people develop CRPS, while others who have had similar trauma do not
  • Can be caused by
    • Fractures
      • In large multicenter prospective study found that 48.5% of patients developed CRPS following a single fracture of the ankle, wrist, scaphoid, or the fifth metatarsal [5]
    • Surgery
    • Sprains and strains
    • Burns
    • Lacerations
    • Limb immobilization (from casting)
    • Penetrating injury
  • Other considerations
    • Fractures:
    • Hypotheses that CRPS is related to hormonal changes in the postmenopausal period remains unproven (need citation)
    • Anecdotal reports that long-term administration of phynotoin and isonizaixd drugs increases the risk of RSD [6]

Risk Factors

  • Demographic
    • Female gender (up to twice as common) [6]
  • Iatrogenic
    • Inadequate anesthesia during fracture reductions [6]

Differential Diagnosis

Clinical Features

  • History
    • Continuous burning or throbbing pain usually in the extremities
    • Disproportionate hyperalgesia
    • Allodynia
    • Swelling
    • Skin changes including erythema, mottling, and "shiny" appearance to skin
    • Loss of function
  • Physical Exam
  • Special Tests

Evaluation

  • No specific test available to diagnose CRPS
  • Diagnosis is made through a thorough history, physical examination, and review of symptoms

Diagnostic Criteria

  • Budapest criteria is associated with improved diagnostic consistency between clinicians compared to IASA criteria [7]
  • Requires presence of both signs of symptoms of CRPS to make the diagnosis
  • The following criteria must be met:
    • Continuing disproportionate pain to any inciting event
    • No other diagnosis can better explain signs and symptoms
    • At least one symptom in all four of the following categories:
      • Sensory: reports of hyperesthesia and/or allodynia
      • Vasomotor: temperature asymmetric and/or skin color changes/asymmetry
      • Sudomotor/edema: edema and/or sweating changes/asymmetry
      • Motor/trophic: decreased range of motion and/or motor dysfunction and/or trophic changes (hair, nail skin)
    • At least one sign at the time of evaluation in two or more of the following categories:
      • Sensory: evidence of hyperalgesia and/or allodynia
      • Vasomotor: evidence of temperature asymmetry (> 1 °C) and/or skin color changes/asymmetry
      • Sudomotor/edema: evidence of edema and/or sweating changes/asymmetry
      • Motor/trophic: evidence of decreased range of motion and/or motor dysfunction and/or trophic changes

EMG/NCs

  • Nerve conduction studies may be used to reveal some CRPS-associated nerve injury [4]

Ultrasound

  • Ultrasound may be used to identify underlying nerve injury

MRI

  • May reveal underlying nerve damage

Nuclear Medicine

  • Triple phase bone scan with contrast
    • Can show CRPS-associated excess bone resorption
    • May aid in localization

Classification

  • No current classification system exists

Management

Nonoperative

Operative

  • Indications
    • Unknown
  • Technique
    • Spinal cord stimulation

Rehab and Return to Play

Rehabilitation

  • Needs to be updated

Return to Play/ Work

  • Needs to be updated

Complications and Prognosis

Prognosis

  • 70% of patients improve within the first year [2]
    • Improvements were usually seen in the function of the extremity
    • Visible symptoms like edema and skin color changes usually improve
    • 25% of these patients fulfilled the Budapest Criteria and only 5% were without complaints
    • Patients that report higher levels of anxiety and pain-related fear at the beginning of therapy have worse long term outcomes after a year

Complications

  • Chronic pain
    • Some patients may progress to severe pain with sympathetic dysfunction that will require regional anesthetic blockade to participate in physical therapy [8]
  • Pleiotropic effects involving most organ systems [9]:
    • Cardiovascular: increased heart rate, decreased heart rate variability, and atypical chest pain
    • Respiratory: shortness of breath and dystonia of chest wall muscles
    • Musculoskeletal: weakness, muscle atrophy, bone lakes due to osteoclastic activation, and bone marrow edema
    • Endocrine: stress, impaired hypothalamo-pituitary adrenal axis leading to tertiary adrenal insufficiency
    • Dermatologic: Erythema, mottling, “ligature sign,” livedo reicularis, and cyanosis
    • Urologic: Urinary frequency, urgency, and incontinence
    • Gastrointestinal system: constipation, nausea, vomiting, intermittent diarrhea, dysphagia, and indigestion

See Also

References

  1. ↑ 1.0 1.1 Iolascon, G., de Sire, A., Moretti, A., & Gimigliano, F. (2015). Complex regional pain syndrome (CRPS) type I: historical perspective and critical issues. Clinical cases in mineral and bone metabolism : the official journal of the Italian Society of Osteoporosis, Mineral Metabolism, and Skeletal Diseases, 12(Suppl 1), 4–10. https://doi.org/10.11138/ccmbm/2015.12.3s.004
  2. ↑ 2.0 2.1 Bruehl, S. (2015). Complex regional pain syndrome. BMJ. https://doi.org/10.1136/bmj.h2730
  3. ↑ 3.0 3.1 3.2 Bruehl, S. (2015). Complex regional pain syndrome. BMJ. https://doi.org/10.1136/bmj.h2730
  4. ↑ 4.0 4.1 4.2 4.3 U.S. Department of Health and Human Services. (n.d.). Complex regional pain syndrome fact sheet. National Institute of Neurological Disorders and Stroke. Retrieved June 19, 2022, from https://www.ninds.nih.gov/health-information/patient-caregiver-education/fact-sheets/complex-regional-pain-syndrome-fact-sheet
  5. ↑ Dey S, Guthmiller KB, Varacallo M. Complex Regional Pain Syndrome. [Updated 2022 May 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430719/
  6. ↑ 6.0 6.1 6.2 ZYLUK, A. N. D. R. Z. E. J. (2004). Complex regional pain syndrome type I. Risk factors, prevention and risk of recurrence. Journal of Hand Surgery, 29(4), 334–337. https://doi.org/10.1016/j.jhsb.2004.01.003
  7. ↑ Goebel A, Bisla J, Carganillo R, et al. A randomised placebo-controlled Phase III multicentre trial: low-dose intravenous immunoglobulin treatment for long-standing complex regional pain syndrome (LIPS trial). Southampton (UK): NIHR Journals Library; 2017 Nov. (Efficacy and Mechanism Evaluation, No. 4.5.) Appendix 3, Research diagnostic criteria (the ‘Budapest Criteria’) for complex regional pain syndrome. Available from: https://www.ncbi.nlm.nih.gov/books/NBK464482/
  8. ↑ Rho, R. H., Brewer, R. P., Lamer, T. J., & Wilson, P. R. (2002). Complex regional pain syndrome. Mayo Clinic Proceedings, 77(2), 174–180. https://doi.org/10.4065/77.2.174
  9. ↑ Schwartzman, R. J. (2012). Systemic complications of complex regional pain syndrome. Neuroscience and Medicine, 03(03), 225–242. https://doi.org/10.4236/nm.2012.33027
Created by:
John Kiel on 14 June 2019 08:25:15
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Last edited:
29 June 2022 18:26:42
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