Reflex Sympathetic Dystrophy (CRPS)

Author: Daniel Hatch

Topic updated on 06/07/13 6:56am

Introduction

Sustained sympathetic activity in a perpetuated reflex arc characterized by pain out of proportion to physical exam findings
- also known as complex regional pain syndrome (CRPS)
- known as causalgia when associated with defined nerve
Pathophysiology
- trauma from an exagerrated response to injury
  - most common reason for a poor outcome following a crush injury to the foot
- surgery
- prolonged immobilization
- possible malingering
Prevention
- Vitamin C 500 mg daily is an effective prophylactic agent in distal radius fractures treated with conservative management
- avoid tight dressings and prolonged immobilization
Prognosis
- responds poorly to conservative and surgical treatments

Classification

Lankford and Evans Stages of RSD
Stage	Onset	Exam	Imaging
Acute	0-3 months	Pain, swelling, warmth, redness, decreased ROM, hyperhidrosis	Normal x-rays, positive three-phase bone scan
Subacute	3 to 12 mos	Worse pain, cyanosis, dry skin, stiffness, skin atrophy	Osteopenia on x-ray
Chronic	> 12 months	Dimished pain, fibrosis, glossy skin, joint contractures	Extreme osteopenia on x-ray

Presentation

Cardinal signs
- exaggerated pain
- swelling
- stiffness
- skin discoloration
Physical exam
- vasomotor disturbance
- trophic skin changes
- hyperhidrosis
- "flamingo gait" if the knee is involved

Imaging

Radiographs
- patella osteopenia if the knee is involved
Three-phase bone scan
- RSD shows positive phase III that does not correlate with positive Phase I and Phase II
- phase background
  - phase I (2 minutes)
    - shows an extremity arteriogram
  - phase II (5-10 minutes)
    - shows cellulitis and synovial inflammation
  - phase III (2-3 hours)
    - shows bone images
  - phase IV (24 hours)
    - can differentiate osteomyelitis from adjacent cellulitis
Thermography
- questionable utility

Studies

Diagnosis
- diagnosis is clinical, but can be confirmed by pain relief with sympathetic block
- early diagnosis is critical for a successful outcome

Treatment

Nonoperative
- physical therapy and pharmocologic treatment
  - indications
    - indicated as first line of treatment
  - modalities
    - gentle physiotherapy
    - tactile discrimination training
    - graded motor imagery
    - medications
      - NSAIDs
      - alpha blocking agents (phenoxybenzamine)
      - antidepressants
      - anticonvulsants
      - calcium channel blockers
      - GABA agonists
- nerve stimulation
  - indications
    - symptoms present mainly in the distribution of one major peripheral nerve
  - programmable stimulators placed on affected nerves
- chemical sympathectomy
  - indications
    - acts as another option when physical therapy and less aggressive nonoperative management fails
Operative
- surgical sympathectomy
  - indications
    - failed nonoperative management, including chemical block

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Qbank (8 Questions)

TAG

(OBQ10.253) Level 1 evidence has shown vitamin C reduces the incidence of reflex sympathetic dystrophy (RSD) or complex regional pain syndrome type I (CRPS) in patients with which of the following? Topic

Review Topic

1. Tarsal tunnel syndrome
2. Distal radius fractures
3. Carpal tunnel syndrome
4. Cervical radiculopathy from herniated nucleus pulposis
5. Ankle fractures

PREFERRED RESPONSE ▶

DISCUSSION: Two different prospective, double-blind studies performed by the same institution have shown that vitamin C administration is associated with a lower risk of RSD(i.e CRPS) after wrist fractures. Vitamin C is thought to reduce lipid peroxidation, scavenge free hydroxyl radicals, protect the capillary endothelium, and inhibit vascular permeability.

The first trial by Zollinger was published in Lancet and included 115 adults with 119 fractures treated with conservative management. They found that RSD/CRPS occurred in four (7%) wrists in the vitamin C group (500mg daily for 50 days) and 14 (22%) in the placebo group.

The second trial by Zollinger published in JBJS included 317 adult patients sustaining 328 distal radius fractures treated conservatively. They had allocated treatment groups to 200mg, 500mg, or 1500mg vitamin C dosages for 50 days. RSD/CRPS occurrence was 4.2% in the 200mg group, 1.8% in the 500mg group, and 1.7% in the 1500mg group and thus the 500mg dosage for 50 days was recommended at the conclusion of the study. Patients making early cast-related complaints to their provider had a higher incidence of developing RSD/CRPS.

REFERENCES:

1. Zollinger PE, Tuinebreijer WE, Breederveld RS, Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? A randomized, controlled, multicenter dose-response study. J Bone Joint Surg Am. 2007 Jul;89(7):1424-31 PMID:17606778 (Link to Abstract)

2. Zollinger PE, Tuinebreijer WE, Kreis RW, Breederveld RS. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomised trial. Lancet. 1999 Dec 11;354(9195):2025-8. PMID:10636366 (Link to Abstract)

Question Authors:

Michael Hughes MD,

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(OBQ07.202) Which of the following modalities has been shown to have a positive effect when treating early stages of complex regional pain syndrome? Topic

Review Topic

1. Casting of the involved extremity
2. Plyometric exercises
3. Ultrasound therapy
4. Acupuncture
5. Gentle physiotherapy

PREFERRED RESPONSE ▶

DISCUSSION: Complex regional pain syndrome type I (reflex sympathetic dystrophy) is defined as a disease that develops after an initial noxious or painful event which causes the development of pain and dysfunction out of proportion to the event. It sometimes cannot be linked to a specific physiologic process. Hyperesthesias, edema, and/or blood flow changes are prevalent. Type II (synonym for causalgia) has a known identifiable nerve injury. Hypotheses include increased sympathetic tone causes feedback loop, activation of nociceptors to neurons in spinal cord, continued ischemia, re-activation of pain receptors, and possibly unregulated sensitivity of alpha adrenergic receptors.

For treatment, early gentle physiotherapy is recommended for this condition. Aggressive passive range of motion is contraindicated in the early phases because it will provoke pain and inflammation. The primary goal of therapy is to decrease pain and prevent stiffness. Contrast baths can help desensitize and improve blood flow, and TENS (transcutaneous electrical nerve stimulator) has been shown to have a positive outcome on CRPS type II only (those with identifiable nerve lesions). Illustration A shows the basic pathology of this condition.

Illustrations: A

REFERENCES:

1. Trumble TE (ed): Hand Surgery Udate 3: Hand, Elbow, & Shoulder. Rosemont, IL, American Society for Surgery of the Hand, 2003, pp 335-351

Question Authors:

Ben Taylor MD, John Badylak MD, Dave Marcu MD,

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(OBQ06.29) A 52-year-old woman falls stepping off the escalator and sustains the wrist fracture shown in Figures A and B. Post-reduction radiographs demonstrate 20 degrees of residual dorsal angulation. The decision is made to proceed with open reduction internal fixation with a volar plate. Which of the following adjuvant interventions has been shown to improve outcomes? Topic

Review Topic

FIGURES: A

1. Application of a bone stimulator within one week following surgery
2. Supplemental percutaneous pin fixation that is removed 4 weeks following surgery
3. Immobilization of the wrist in an extension splint or cast for 3 weeks following surgery
4. Administration of oral vitamin C beginning the first day after surgery
5. Use of an axillary regional block during the surgery

PREFERRED RESPONSE ▶

DISCUSSION: The radiographs demonstrate a distal radius fracture. Given the residual angulation, open reduction internal fixation is necessary. Supplemental vitamin C has been shown in 2 separate level 1 studies to reduce the incidence of reflex sympathetic dystrophy.

Cazanueve et al evaluated 195 patients who underwent open reduction internal fixation of a distal radius fracture. The first 100 did not receive vitamin C postoperatively. The next 95 patients received oral vitamin C for 45 days beginning the first day after surgery. The patients who received vitamin C had 5 times less incidence (2% vs. 10%) of reflex sympathetic dystrophy.

REFERENCES:

1. Cazeneuve JF, Leborgne JM, Kermad K, Hassan Y. [Vitamin C and prevention ofreflex sympathetic dystrophy following surgical management of distal radiusfractures]. Acta Orthop Belg. 2002 Dec;68(5):481-4. PMID:12584978 (Link to Abstract)

Question Authors:

John Badylak MD, Ben Taylor MD,

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(OBQ05.139) All of the following are clinical features of complex regional pain syndrome (reflex sympathetic dystrophy) of the lower extremity EXCEPT: Topic

Review Topic

1. Swelling
2. Cool and shiny skin
3. Allodynia
4. Crepitus
5. Hyperpathia

PREFERRED RESPONSE ▶

DISCUSSION: Complex regional pain syndrome is a complex clinical problem for the orthopaedic surgeon. In the acute stage (<3 months), burning or aching pain that cannot be controlled by narcotics is the major feature. Swelling, cool and shiny skin, allodynia and hyperpathia are also clinical features. Crepitus, however, is not a clinical feature.

Hogan et al provide a review of the evaluation and treatment of complex regional pain syndrome. They argue "despite many divergent and often conflicting theories, the cause of the severe pain, alterations in regional blood flow, and edema is unknown. Interventions that have proved successful for treating similar conditions in the arm and hand frequently do not relieve pain similarly in the lower extremity. Common treatment regimens target individual components of this symptom complex, namely, sympathetic or afferent nerve hyperactivity, vasomotor instability, or regional osteoporosis. Despite widespread use of some of these treatments, few controlled clinical trials quantify their effectiveness."

Illustrations A and B shows characteristic features of complex regional pain syndrome of the lower extremity.

Illustrations: A

REFERENCES:

1. Hogan CJ, Hurwitz SR. Treatment of complex regional pain syndrome of the lower extremity. J Am Acad Orthop Surg. 2002 Jul-Aug;10(4):281-9. Review. PMID: 15089077 (Link to Abstract)

Question Authors:

Derek Moore, John Badylak MD, Ben Taylor MD,

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(OBQ05.230) A 58-year-old female complains of continued pain and swelling 6 months following total knee arthroplasty. She describes a burning pain that radiates from the knee down the anterior compartment of the leg. The pain arises sporadically and is associated with swelling, sweating, and a purplish hue of the leg. Knee radiographs are provided in Figures A and B. Which of the following is the best management? Topic

Review Topic

FIGURES: A

1. Lumbar spine MRI to evaluate for radiculopathy of the L3 nerve root
2. Alpha-adrenergic blockers, physical therapy, tactile discrimination training, and graded motor imagery
3. Surgical exploration of the knee
4. Surgical debridement, pulsatile irrigation, tissue sampling for culture/biopsy, and polyethylene exchange
5. Magnetic resonance arthrogram (MRA) with intra-articular contrast and diagnostic steroid injection

PREFERRED RESPONSE ▶

DISCUSSION: The clinical scenario and radiographs are consistent with a patient who is experiencing complex regional pain syndrome following total knee arthroplasty. Treatment is multi-modal including GABA agonists, alpha-blockers, beta-blockers, physical therapy, occupational therapy, graded motor imagery, tactile discrimination treatments, sypathectomy, local anesthetics, and even spinal cord stimulators. Mont et al reported limited success in 27 patients who had surgical exploration of radiographically normal knees following TKA with unexplained pain. Outcomes were especially poor in patients who had acheived adequate range of motion and continued to have pain prior to surgical exploration. Patients with decreased range of motion who acheived improvement in motion postoperatively also demonstrated great relief of pain.

REFERENCES:

1. Mont MA, Serna FK, Krackow KA, Hungerford DS. Exploration of radiographicallynormal total knee replacements for unexplained pain. Clin Orthop Relat Res. 1996Oct;(331):216-20. PMID:8895641 (Link to Abstract)

Question Authors:

John Badylak MD,

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(OBQ05.269) A 38-year-old patient presents 6 months after intramedullary nailing of a distal third tibia fracture with symptoms consistent with complex regional pain syndrome. During the early stage of the disease he was treated with intermittent splinting, elevation and massage, contrast baths, and transcutaneous electrical nerve stimulation. Despite these modalities, he continues to have severe and debilitating systems. Which of the following treatment options is indicated as a second line of treatment? Topic

Review Topic

1. Long leg cast immobilization for 3 months
2. Walking boot with non weight bearing for three months
3. Exchange nailing to stimulate healing response to the limb
4. Epidural spinal cord stimulator
5. Surgical sympathectomy of the affected limb

PREFERRED RESPONSE ▶

DISCUSSION: Complex regional pain syndrome is a chronic progressive disease of unknown etiology characterized by pain, swelling and skin changes. If nonoperative modalities fail, a surgical sympathectomy of the affected limb is indicated.

The first line of treatment is physical therapy including intermittent splinting, elevation and massage, contrast baths, and transcutaneous electrical nerve stimulation. Aggressive passive range-of-motion exercises should be avoided. If nonoperative modalites fail and symptoms remain severe, a surgical sympathectomy of the affected limb is indicated. Keys to successful treatment include early clinical suspicion and treatment. Late CRPS is highly refractory to treatment and often results in permanent disability.

Two forms of Complex regional pain syndrome exist: 1) Reflex sympathetic dystrophy - which does not demonstrate nerve lesions, and 2) Causalgia - which is associated with damage to peripheral nerves. Diagnostic criteria include:
Major criteria: intense and prolonged pain, swelling, stiffness, and discoloration (vasomotor disturbances).
Minor criteria: trophic changes, osseous demineralization, temperature changes, and palmar fibromatosis.

Tran et al present their systemic review of 41 RCTs of the research regarding treatment of CRPS. Their data suggest that only bisphosphonates offer clear medicinal benefits in the treatment of CRPS. Evidence regarding a beneficial effect of lumbar sympathetic blocks, gabapentin, and physical therapy is lacking. As such, these authors advocate for further study thru well-designed RCTs to better evaluate appropriate and effective treatment strategies.

REFERENCES:

1. Trumble TE (ed): Hand Surgery Update 3: Hand, Elbow, & Shoulder. American Society for Surgery of the Hand, Rosemont, IL. 2003, pp 335-351

2. Tran de QH, Duong S, Bertini P, Finlayson RJ. Treatment of complex regional pain syndrome: a review of the evidence. Can J Anaesth. 2010 Feb;57(2):149-66. PMID:20054678 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Patrick McCulloch MD, Sanj Kakar MD,

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(OBQ04.43) A 34-year-old laborer has her left foot crushed in a piece of farming equipment as shown in Figure A. All of the following are reasons for a poor outcome following a crush injury to the foot EXCEPT: Topic

Review Topic

FIGURES: A

1. Workers compensation injury
2. Development of reflex sympathetic dystrophy (complex regional pain syndrome)
3. Delayed soft-tissue coverage in mangled extremities
4. Immediate skeletal stabilization
5. Ongoing litigation

PREFERRED RESPONSE ▶

DISCUSSION: This patient has a mangled extremity. Rigid skeletal stabilization is recommended to enhance soft-tissue healing.

Level 4 evidence from Myerson et al found that delayed soft-tissue coverage in mangled extremities correlated with poor outcome. Poor results also occurred if treatment was not immediately initiated (immediate debridement shown in Illustration A), if patients subsequently had neuritis or reflex sympathetic dystrophy, or if patients were involved in ongoing workers' compensation and litigation. Neuroischemia following substantial soft-tissue injury likely plays a role in the development of chronic pain after crush injuries to the foot, either through direct trauma to the peripheral nerves or by intraneural or extraneural fibrosis. This trauma to the nerve may cause chronic neuritis, which then triggers a sympathetically mediated reflex sympathetic dystrophy (complex regional pain syndrome).

Illustrations: A

REFERENCES:

1. Myerson M, McGarvey WC. Crush injuries and compartment syndromes, in Myerson M (ed): Current Therapy in Foot and Ankle Surgery. 1993 pp 264-273

2. Myerson MS, McGarvey WC, Henderson MR, Hakim J. Morbidity after crush injuries to the foot. J Orthop Trauma. 1994 Aug;8(4):343-9. PMID: 7965298 (Link to Abstract)

Question Authors:

Michael Hughes MD, Dave Marcu MD, John Badylak MD,

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(OBQ04.191) What is the most common radiographic finding in reflex sympathetic dystrophy (RSD) or complex regional pain syndrome of the knee? Topic

Review Topic

1. patella baja
2. patella alta
3. patella osteopenia
4. generalized osteopenia
5. supracondylar stress fracture

PREFERRED RESPONSE ▶

DISCUSSION: Reflex Sympathetic Dystrophy (RSD) of the knee is different than that of the upper extremity. Pain out of proportion to the initial injury is the hallmark symptom. Other features include vasomotor disturbances, delayed functional recovery and various associated trophic changes. The JAAOS article by Cooper et al states that patellar osteopenia "is the most common radiographic finding". However, they go on to state that the most reliable diagnostic test is symptom relief after sympathetic blockade. The JBJS article by Cooper et al treated 14 patients with RSD of the knee with epidural blocks for 4 days. Eleven patients had complete resolution of their symptoms, and pain that was out of proportion to the severity of the injury was the most consistent finding. Katz et al reviewed 36 patients with RSD primarily affecting the knee. They found that injuries or operation about the patellofemoral joint triggered its onset in 64% of patients.

REFERENCES:

1. Cooper DE, DeLee JC, Ramamurthy S. Reflex sympathetic dystrophy of the knee. Treatment using continuous epidural anesthesia. J Bone Joint Surg Am. 1989 Mar;71(3):365-9. PMID:2925709 (Link to Abstract)

2. Cooper DE, DeLee JC. Reflex Sympathetic Dystrophy of the Knee. J Am Acad Orthop Surg. 1994 Mar;2(2):79-86. PMID:10708995 (Link to Abstract)

3. Katz MM, Hungerford DS. Reflex sympathetic dystrophy affecting the knee. J Bone Joint Surg Br. 1987 Nov;69(5):797-803. PMID:3680346 (Link to Abstract)

Question Authors:

Paul D. Kim MD, Patrick McCulloch MD, John Badylak MD,

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Groups

ABOS II: Reflex Sympathetic Dystrophy (Complex Regional Pain Syndrome)

General - Oral Boards Review

7/29/2011

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Evidence & References Show References

Level of Evidence 1 (Randomized Control Trial)

Zollinger PE, Tuinebreijer WE, Breederveld RS, Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? A randomized, controlled, multicenter dose-response study. J Bone Joint Surg Am. 2007 Jul;89(7):1424-31 PMID:17606778 (Link to Abstract)
Zollinger PE, Tuinebreijer WE, Kreis RW, Breederveld RS. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomised trial. Lancet. 1999 Dec 11;354(9195):2025-8. PMID:10636366 (Link to Abstract)

Level of Evidence 2 (Prospective Cohort Study)

Cazeneuve JF, Leborgne JM, Kermad K, Hassan Y. [Vitamin C and prevention ofreflex sympathetic dystrophy following surgical management of distal radiusfractures]. Acta Orthop Belg. 2002 Dec;68(5):481-4. PMID:12584978 (Link to Abstract)

Level of Evidence 4 (Case Series)

Mont MA, Serna FK, Krackow KA, Hungerford DS. Exploration of radiographicallynormal total knee replacements for unexplained pain. Clin Orthop Relat Res. 1996Oct;(331):216-20. PMID:8895641 (Link to Abstract)
Myerson MS, McGarvey WC, Henderson MR, Hakim J. Morbidity after crush injuries to the foot. J Orthop Trauma. 1994 Aug;8(4):343-9. PMID: 7965298 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Tran de QH, Duong S, Bertini P, Finlayson RJ. Treatment of complex regional pain syndrome: a review of the evidence. Can J Anaesth. 2010 Feb;57(2):149-66. PMID:20054678 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Myerson M, McGarvey WC. Crush injuries and compartment syndromes, in Myerson M (ed): Current Therapy in Foot and Ankle Surgery. 1993 pp 264-273
Trumble TE (ed): Hand Surgery Udate 3: Hand, Elbow, & Shoulder. Rosemont, IL, American Society for Surgery of the Hand, 2003, pp 335-351
Trumble TE (ed): Hand Surgery Update 3: Hand, Elbow, & Shoulder. American Society for Surgery of the Hand, Rosemont, IL. 2003, pp 335-351

Undefined

Cooper DE, DeLee JC, Ramamurthy S. Reflex sympathetic dystrophy of the knee. Treatment using continuous epidural anesthesia. J Bone Joint Surg Am. 1989 Mar;71(3):365-9. PMID:2925709 (Link to Abstract)
Cooper DE, DeLee JC. Reflex Sympathetic Dystrophy of the Knee. J Am Acad Orthop Surg. 1994 Mar;2(2):79-86. PMID:10708995 (Link to Abstract)
Hogan CJ, Hurwitz SR. Treatment of complex regional pain syndrome of the lower extremity. J Am Acad Orthop Surg. 2002 Jul-Aug;10(4):281-9. Review. PMID: 15089077 (Link to Abstract)
Katz MM, Hungerford DS. Reflex sympathetic dystrophy affecting the knee. J Bone Joint Surg Br. 1987 Nov;69(5):797-803. PMID:3680346 (Link to Abstract)

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Reflex Sympathetic Dystrophy (CRPS)

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