Complex Regional Pain Syndrome (CRPS)

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Original Editors - Yves Hubar

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Search Strategy

Literature was found on pubmed and the vub v-spaces system.

Definition/Description

Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. It is a disease that may develop after a limb trauma. This appears mostly in 1 or more limbs, usually in the arms or legs. We can say a CRPS is a regional posttraumatic neuropathic pain problem. Neuropathic pain disorders are a disproportionate consequence of painful trauma or nerve lesion.

CRPS is subdivided into type I and type II CRPS.

In the literature, there are a lot of names used to describe this syndrome such as ‘‘Reflex Sympathetic Dystrophy’’, ‘‘causalgia’’, ‘‘algodystrophy’’, ‘‘Sudeck’s atrophy’’, ‘‘neurodystrophy’’, and ‘‘post-traumatic dystrophy’’. To standardize the nomenclature, the name ‘complex regional pain syndrome’ was adopted in 1995 by the ‘International Association for the Study of Pain’ (IASP).

Clinically Relevant Anatomy

CRPS can take place in any body part, but the wrist is most frequently affected after fractures.

An important aspect of the disease is the occurance of vascular disturbances. Mostly affected are primary small vessels, causing an impact on microcirculation, skin temperature and clinical appearance of the limb.
A paper described the changes in microcirculation as an increase in the number of capillaries, endothelial swelling and changes in the vessel luminal wall.
According to a review, the acute stage features inhibited sympathetic vasoconstriction and exaggerated neurogenic inflammation, whereas the cold stage features vasoconstriction and endothelial disfunction or vascular hyperreactivity while neurogenic inflammation is less severe.

Epidemiology /Etiology

CRPS is found to result:
- After traumatic injury (65%)

• 1-2% of all fractures result in CRPS
• Largest risk of CRPS for fractures of the wrist

- After surgical intervention (19%)
- Infection (4%)
- Prior inflammation (2%)
- No clear cause (10%)

A review stated that women are predominantly affected, by a factor of 3,5 and a genetic predisposition has also been theorized.
The disease affects all ages, though most cases are between 50 and 70 years old, and it is generally believed to occur mainly in caucasian and Japanese people.

Characteristics/Clinical Presentation

The following symptoms have been found in literature:
- Autonomic and trophic disorders:

• Distal Edema in 80% of the patients
• Skin temperature changes at the affected body part in 80% of the patients, initially warmer and in 40% of patients gradually cools down until colder in comparison to the rest of the body as the disease progresses. Another review mentioned that 30% of the patients start off from the primarily cold stage.3
• In 40% of the patients skin at the affected body part starts showing redness, but becomes pale or livid in later stages
• In 55% altered sweating takes place, with hyperhydrosis being more common than hypohydrosis.
• Hair and nail growth possibly increase in early stages
• Atrophy of skin and muscles in later stages, as well as contractures may severely restrict movement

- Sensory disturbances (90%) typically in a glove or stocking-like distribution

• Spontaneous pain occurs in 75%, usually burning dragging or stinging

• 68% felt in deep structures
• 32% felt in skin
• In 77% pain shows fluctuating intensity, lesser proportion shows shooting pain
• Pain can be increased by orthostasis, anxiety, exercise or temperature changes.
• In many cases, pain is more pronounced at night

• Sensory gain (Mechanical hyperalgesia, allodynia, ...) or sensory loss (hypaesthesia, hypalgesia, …) may be present.

- Motor dysfunction

• Motor weakness
• Severe impairment of complex movements
• Impairment of range of motion, initially by concomitant edema, later by contractures and fibroses
• Neglect like symptoms have been found in some patiënts, described as the body part in question feeling foreign.
• Enhanced physiological tremor in around 50%
• Myoclonus or dystonia, especially in type II CRPS
  

Differential Diagnosis

The differential diagnostic consists of:

• Rheumatic conditions
• Inflammatory conditions (infections following bone surgery, neuritides)
• Thromboembolic conditions
• Compartment syndrome
  
• Peripheral neuropathy (mainly for type II CRPS)

Diagnostic Procedures

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Outcome Measures

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Examination

No golden standard has been developed yet, but included here are the Budapest criteria.

The following must be met
- Continuing pain, which is disproportionate to any inciting event
- Must report at least one symptom in three of the four following categories:

• Sensory: reports of hypaesthesia and/or allodynia
• Vasomotor: reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
• Sudomotor/edema: reports of edema and/or sweating changes and/or sweating asymmetry
  
• Motor/trophic: reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

- Must display at least one sign at time of evaluation in two or
more of the following categories:

• Sensory: evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement)
• Vasomotor: evidence of temperature asymmetry (>1°C) and/or skin color changes and/or asymmetry
• Sudomotor/edema: evidence of edema and/or sweating changes and/or sweating asymmetry
• Motor/trophic: evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

- There is no other diagnosis that better explains the signs and symptoms

Medical Management

Concerning pharmacogenic treatment:

- Pathophysiologically oriented pharmacogenic treatment include application of glucocorticoids, tnf-alpha antibodies, free radical scavengers and sympathic blockade.
- Symptomatically oriented pharmacogens include opioids, gabapentin, NSAIDs and baclofen.
- To inhibit osteoclastic activity calcitonin, bisphosphonates and mannitol and vasodilating drugs may be given.

Definite reports on the efficacy of sympathectomy are currently lacking and there is a risk of developing post sympathectomy pain syndrome.

A review has been found, describing the positive effects of Spinal Cord Stimulation and several theories regarding its effectiveness.

Physical Therapy Management

The following interventions were found in literature:

• Lymphatic drainage to facilitate regression of edema
• Mirror Therapy
• Graded motor learning
• TENS (Unless patient cannot tolerate the therapy due to allodynia or hyperalgesia)

A paper on movement disorders in CRPS stated that splints or plaster casts are often ineffective and might even worsen dystonic postures related to CRPS.

Key Research

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Resources

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Clinical Bottom Line

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Recent Related Research (from Pubmed)

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References

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