Updated: 5/8/2019

Complex Regional Pain Syndrome (CRPS)

Topic
Review Topic
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0
Questions
11
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Evidence
14
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https://upload.orthobullets.com/topic/6095/images/rsd foot.jpg
https://upload.orthobullets.com/topic/6095/images/rsd (swelling).jpg
Introduction
  • Overview
    • complex regional pain syndrome is defined as sustained sympathetic activity in a perpetuated reflex arc characterized by pain out of proportion to physical exam findings
      • formerly known as reflex sympathetic dystrophy (RSD); many different names in past (remember "hand shoulder syndrome" as you will see this coexisting pathology)
      • known as causalgia when associated with specific nerve
  • Epidemiology
    • incidence
      • US incidence is 5.5 per 100,000 person-years
    • demographics
      • females more commonly affected (4:1)
      • incidence increases with age up until 70 years old
    • location
      • 60% occurs in the upper extremities
      • 40% occurs in the lower extremities
    • risk factors
      • trauma with an exagerrated response to injury
        • most common reason for a poor outcome following a crush injury to the foot 
      • surgery
      • prolonged immobilization
      • anxiety or depression
      • use of ACE inhibitors at the time of trauma
      • history of migraines or asthma
      • smoking
  • Pathophysiology
    • aberrant inflammatory response
    • vasomotor dysfunction
    • maladaptive neuroplasticity
  • Genetics
    • thought to have a genetic predisposition
      • siblings of young-onset CRPS patients have an increased risk of developing CRPS
      • associated with polymorphisms in TNF-alpha and ACE genes
  • Prevention
    • vitamin C 500mg daily x 50 days in distal radius fractures treated conservatively    
      • 200mg daily x 50 days if impaired renal function
      • vitamin C also has been shown to decrease the incidence of CRPS (type I) following foot and ankle surgery
    • avoid tight dressings and prolonged immobilization
  • Prognosis
    • typically responds poorly to conservative and surgical treatments
    • better prognosis if upper extremity, warm CRPS, children
Classification
 
Lankford and Evans Stages of RSD
Stage
Onset
Exam
Imaging
Acute
0-3 months
Burning pain, redness, swelling, warmth, hyperhidrosis, hyperesthesia, cold intolerance, joint stiffness
Normal x-rays, positive three-phase bone scan
Subacute (dystrophic)
3-12 months
Worsening pain, cyanosis, dry skin, stiffness, skin atrophy Subchondral osteopenia on x-ray
Chronic (atrophic)
> 12 months
Diminished pain, glossy skin, fibrosis, joint contractures, loss of hair and nails
Extreme osteopenia on x-ray
  •  International Association for the Study of Pain Classification
    • type I 
      • CRPS without demonstrable nerve damage
      • most common
      • results from trauma, casting, or tight dressings
    • type II
      • CRPS with evidence of identifiable nerve damage
      • minimal positive response with sympathetic blocks
Presentation
  • Cardinal signs 
    • exaggerated pain
    • swelling
    • stiffness
    • skin discoloration
  • Physical exam
    • vasomotor disturbance
    • trophic skin changes
    • hyperhidrosis
    • "flamingo gait" if the knee is involved
    • equinovarus defomity if the ankle is involved
Imaging
  • Radiographs
    • findings
      • osteopenia
        • affects the patella if the knee is involved 
      • soft tissue swelling
      • subperiosteal bone resportion
      • preservation of joint spaces
  • Three-phase bone scan
    • indications
      • can help to rule out CRPS type I (has high negative predictive value)
    • phases
      • 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
    • findings
      • increased uptake in all phases
        • phase III is most sensitive
  • Thermography
    • used to quantify temperature differences between the limbs
    • questionable utility
  • EMG/NCV
    • may demonstrate slowing in known nerve distribution (e.g. slowing of median nerve conduction for CRPS type II in the forearm)
Studies
  • Diagnosis
    • usually clinical but can be confirmed by pain relief with sympathetic block
    • early diagnosis is critical for a successful outcome
    • Budapest diagnostic criteria
      • 1. Continuing pain that is disproportionate to any inciting event
      • 2. Must report at least one symptom in three (clinical diagnostic criteria) or four (research diagnostic criteria) of the following categories:
        • sensory: hyperesthesia or allodynia
        • vasomotor: temperature asymmetry, skin colour changes, or skin colour asymmetry
        • sudomotor/edema: edema, sweating changes, or sweating asymmetry
        • motor/trophic: decreased range of motion, motor dysfunction (weakness, tremor, or dystonia), or trophic changes (hair, nails, or skin)
      • 3. Must display at least one sign at time of diagnosis in two or more of the following categories:
        • sensory: hyperalgesia (to pinprick) or allodynia (to light touch, deep somatic pressure, or joint movement)
        • vasomotor: temperature asymmetry, skin colour changes or asymmetry
        • sudomotor/edema: oedema, sweating changes, or sweating asymmetry
        • motor/trophic: decreased range of motion, or motor dysfunction (weakness, tremor, or dystonia), or trophic changes (hair, nails, or skin)
      • 4. No other diagnosis better explains the signs and symptoms
Differential
  • Soft tissue infection
  • Malingering
  • Psychiatric disease
  • Neuropathic pain
  • Chronic pain
  • Raynaud disease
  • Thoracic outlet syndrome
  • Arterial insufficiency
  • Erythromelalgia
Treatment
  • Nonoperative  
    • physical therapy and pharmacologic treatment 
      • indications
        •  first line of treatment
    • nerve stimulation
      • indications
        • symptoms present mainly in the distribution of one major peripheral nerve
    • nerve blockade
      • indications
        • failed initial nonoperative treatment
    • 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 sympathectomy)
    • surgical decompression
      • indications
        • CRPS type II with known nerve involvement (e.g. carpal tunnel release if median nerve involved)
      • best success for CRPS is if you can find an associated nerve problem and treat it
Techniques
  • Physical therapy and pharmacologic treatment
    • modalities
      • gentle physiotherapy 
      • tactile discrimination training
      • graded motor imagery
        • sequential process consisting of laterality reconstruction, motor imagery, and mirror therapy
    • medications
      • NSAIDs
      • alpha blockers (phenoxybenzamine, prazosin)
      • beta blockers (propranolol)
      • anti-depressants
      • anti-convulsants
      • calcium channel blockers
      • GABA agonists (gabapentin)
      • bisphosphonates
      • anti-arrhythmics
      • corticosteroids
      • calcitonin
  • Nerve stimulation
    • programmable stimulators placed on affected nerves
    • types
      • transcutaneous electrical stimulation (TENS)
      • peripheral nerve stimulation
      • spinal cord stimulation
  • Nerve blockade
    • types
      • sympathetic
        • stellate ganglion (for upper extremity)
        • lumbar spinal (for lower extremity)
      • peripheral nerve
      • neuraxial/epidural
    • agents
      • anesthetics (lidocaine or bupivicaine +/- epinephrine)
      • sympatholytics (bretylium, guanethidine)
  • Chemical sympathectomy
    • types
      • stellate ganglion (for upper extremity)
      • lumbar spinal (for lower extremity)
    • agents
      • phenol
      • alcohol
  • Surgical sympathectomy
    • ideal for patients who have had a response to sympathetic nerve blockade
    • methods
      • excision
      • electrocautery
 

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Questions (11)
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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. Aspiration is negative for infection. Which of the following is the best management? Review Topic

QID: 1116
FIGURES:
1

Lumbar spine MRI to evaluate for radiculopathy of the L3 nerve root

2%

(19/847)

2

Alpha-adrenergic blockers, physical therapy, tactile discrimination training, and graded motor imagery

92%

(783/847)

3

Surgical exploration of the knee

1%

(8/847)

4

Surgical debridement, pulsatile irrigation, tissue sampling for culture/biopsy, and polyethylene exchange

3%

(22/847)

5

Magnetic resonance arthrogram (MRA) with intra-articular contrast and diagnostic steroid injection

1%

(11/847)

ML 1

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PREFERRED RESPONSE 2

(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? Review Topic

QID: 3356
1

Tarsal tunnel syndrome

0%

(9/2930)

2

Distal radius fractures

91%

(2676/2930)

3

Carpal tunnel syndrome

4%

(110/2930)

4

Cervical radiculopathy from herniated nucleus pulposis

1%

(35/2930)

5

Ankle fractures

3%

(87/2930)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(OBQ13.176) Vitamin C has been shown to decrease the likelihood of which of the following complications following surgery on the foot and ankle in non-diabetic patients? Review Topic

QID: 4811
1

Nonunion

1%

(38/3875)

2

Complex Regional Pain Syndrome, type II

15%

(581/3875)

3

Malunion

0%

(14/3875)

4

Complex Regional Pain Syndrome, type I

79%

(3059/3875)

5

Wound infection

4%

(160/3875)

ML 2

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PREFERRED RESPONSE 4

(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: Review Topic

QID: 104
FIGURES:
1

Workers compensation injury

2%

(7/414)

2

Development of reflex sympathetic dystrophy (complex regional pain syndrome)

1%

(6/414)

3

Delayed soft-tissue coverage in mangled extremities

10%

(40/414)

4

Immediate skeletal stabilization

85%

(352/414)

5

Ongoing litigation

2%

(9/414)

ML 1

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PREFERRED RESPONSE 4

(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 studies in the literature to support an improvement in outcomes? Review Topic

QID: 140
FIGURES:
1

Application of a bone stimulator within one week following surgery

3%

(44/1695)

2

Supplemental percutaneous pin fixation that is removed 4 weeks following surgery

3%

(52/1695)

3

Immobilization of the wrist in an extension splint or cast for 3 weeks following surgery

6%

(106/1695)

4

Administration of oral vitamin C beginning the first day after surgery

85%

(1433/1695)

5

Use of an axillary regional block during the surgery

3%

(54/1695)

ML 1

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PREFERRED RESPONSE 4

(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 symptoms. Which of the following treatment options is indicated as a second line of treatment? Review Topic

QID: 1155
1

Long leg cast immobilization for 3 months

3%

(18/651)

2

Walking boot with non weight bearing for three months

4%

(29/651)

3

Exchange nailing to stimulate healing response to the limb

5%

(32/651)

4

Epidural spinal cord stimulator

19%

(123/651)

5

Surgical sympathectomy of the affected limb

68%

(442/651)

ML 3

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PREFERRED RESPONSE 5

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

QID: 863
1

Casting of the involved extremity

18%

(315/1710)

2

Plyometric exercises

4%

(63/1710)

3

Ultrasound therapy

4%

(75/1710)

4

Acupuncture

4%

(67/1710)

5

Gentle physiotherapy

69%

(1184/1710)

ML 3

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PREFERRED RESPONSE 5

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

QID: 1025
1

Swelling

1%

(22/2173)

2

Cool and shiny skin

2%

(41/2173)

3

Allodynia

1%

(20/2173)

4

Crepitus

95%

(2058/2173)

5

Hyperpathia

1%

(24/2173)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

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

QID: 1296
1

patella baja

2%

(6/385)

2

patella alta

5%

(18/385)

3

patella osteopenia

43%

(165/385)

4

generalized osteopenia

50%

(192/385)

5

supracondylar stress fracture

1%

(2/385)

ML 4

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PREFERRED RESPONSE 3
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