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Complex Regional Pain Syndrome
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Contents
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
- Fractures
- 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
- Cellulitis
- Fibromyalgia
- Vascular insufficiency
- Lymphedema
- Deep Vein Thrombosis
- Renaud's phenomenon
- Small or large fiber sensorimotor neuropathy
- Diabetic Neuropathy
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
- General
- Early detection and treatment are important
- Requires multidisciplinary clinical care
- Current RCTs show supports multiple treatment options
- Physical Therapy, Occupational Therapy
- Including graded motor imagery and mirror therapy
- Medications
- Bisphosphonates
- Calcitonin
- Subanesthetic IV Ketamine
- Free radical scavengers
- Oral corticosteroids
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
- ↑ 1.0 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
- ↑ 2.0 2.1 Bruehl, S. (2015). Complex regional pain syndrome. BMJ. https://doi.org/10.1136/bmj.h2730
- ↑ 3.0 3.1 3.2 Bruehl, S. (2015). Complex regional pain syndrome. BMJ. https://doi.org/10.1136/bmj.h2730
- ↑ 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
- ↑ 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/
- ↑ 6.0 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
- ↑ 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/
- ↑ 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
- ↑ 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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