Complex Regional Pain Syndrome

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Summary

The description of complex regional pain syndromes (CRPS) dates back to the mid-19th century when Mitchell first described this condition in 1864. Mitchell, a civil war surgeon, coined the term causalgia, meaning burning pain.(1) The most striking feature of this condition is that the degree of pain is disproportional to the extent of injury. The onset of CRPS typically follows an injury. Injuries may be minor, such as a sprain or fracture, or may be significant, such as an amputation.(2)

Symptoms

  • Burning pain made worse by light touch
  • Sharp, shooting pain
  • Temperature changes in the skin
  • Changes in the texture (turgor) and color of the skin
  • Changes in hair and nails
  • Loss of motion of the affected extremity
  • Inability to bear weight on a leg or grip with a hand

Complex regional pain syndrome symptoms vary dramatically in their onset, intensity, duration, and response to care.(3) The characteristic symptom is pain that is disproportional to the injury resulting in functional disability and psychological distress.(4)  One of the difficulties in treating CRPS is making an initial diagnosis due to the complex nature of this group of conditions. Once diagnosed, aggressive and prompt treatment is important to arrest the progression of this disabling disease.

Description

Complex regional pain syndromes (CRPS) are described by a number of names:

Algodystrophy
Peripheral trophoneurosis
Post-traumatic pain syndrome
Reflex sympathetic dystrophy syndrome (RSD/RSDS)
Shoulder-hand syndrome
Sudeck's atrophy
Sympathalgia
Sympathetically independent pain
Sympathetically maintained pain
Sympathetic overdrive syndrome

Due to confusion arising from the many names for this set of symptoms, The International Association for the Study of Pain (IASP) developed nomenclature to more accurately describe chronic pain.(5) IASP coined the term complex regional pain syndrome (CRPS) and broke CRPS into two categories:

CRPS 1 - Consists of pain, sensory abnormalities, abnormal sweating and blood flow, abnormal motor system function and trophic changes (thickening of the skin and nails, coarse thin hair growth) and atrophy of the superficial and deep tissues (skin, muscle, bone.) CRPS 1 may or may not present with an injury. Injuries are benign, such as a sprain or contusion with no identifiable injury to the nerve.

CRPS 2 - Same as CRPS 1 but presents with an identifiable nerve injury. Symptoms include burning pain made worse by light touch, temperature changes or motion of the limb. These findings are most common in the foot or hand following partial injury to the nerve. The affected area appears cool, reddish, and clammy. The superficial and deep tissue structures may also begin trophic changes.

CRPS may be unilateral or bilateral effecting a portion or entire extremity. Progression from one extremity to another, say arm-to-leg, is uncommon.

Causes and contributing factors

CRPS is often associated with chronic depression that is present prior to the onset of injury.(6) Females are 4 times more likely to develop CRPS. The average age of onset is 47 years of age.(7)  White, middle-aged females are predominantly at risk.(8)

Differential Diagnosis

The differential diagnosis of CRPS includes:

Arthritis
Cellulitis
Charcot arthropathy
Dermatitis
Edema
Fracture
Lupus
Peripheral neuropathy
Septic joint
Tarsal tunnel syndrome

Treatment

Treatment of CRPS 1 and 2 consists of many different measures, but there is general agreement that the success of treatment depends upon early implementation of treatment.(9,10) Treatment may include:

Medications

Narcotics for pain suppression
Anti-inflammatory, non-narcotic control of inflammatory pain
Antidepressants; maintenance of normal sleep cycles, anxiety control
Calcium channel blockers; increased blood flow to extremities
Anticonvulsant regulation of normal sleep cycle, control of pain
Morphine pump implanted

Pain blocks

Peripheral nerve blocks of the affected area
IV regional blocks of the affected extremity
Lumbar sympathetic blocks- given by an anesthesiologist

Physical therapy

Range of motion, strengthening exercises, continuous passive motion (CPM)
Whirlpool, ultrasound, heat treatment
TENS, nerve stimulation

Additional methods of treatment include:

Steroid injections
Lumbar sympathectomy
Dorsal column stimulation
Neurectomy - surgical excision of the nerve
Amputation - surgical removal of the affected extremity

The prognosis for patients with CRPS varies greatly and depends upon the severity of symptoms, when treatment is initiated, and the type(s) of treatment employed. Studies have shown that the overall success rate of the treatment of CRPS has been 50%. In a study performed by Anderson and Fallat, they found that 3.5 years following the onset of traumatic injury resulting in CRPS, 12 of 13 patients still had pain considered to be moderate to considerable. (8)

Treatment of CRPS requires a team approach to treat not only pain but also the numerous problems associated with chronic pain. These problems include:

Depression
Disruption of normal sleep cycles
Inability to walk or bear weight
Inability to work
Disruption of relationships with spouse or offspring

Resources that may be helpful in addition to pain management include psychological counseling, physical therapy, and occupational therapy.

When to contact your doctor

CRPS needs to be treated early and aggressively.  All cases of CRPS should be evaluated by your podiatrist, orthopedist or family doctor.

References

1. Mitchell SW.  Injuries of Nerves and their consequences, JB Lippincott, New York, 1872.
2. Bruehl S. An update on the pathophysiology of complex regional pain syndrome.  Anaesthesiology 113:713-725, 2010.
3. Wasner G, Schattschneider J, Baron R.  Skin temperature differences - a diagnostic tool for CRPS?  Pain 98: 19-26, 2002.
4. Geertzen JH, Dijkstra PU, Groothoff JW, ten Duis HJ, Eisma WH.  Reflex sympathetic dystrophy of the upper extremity - a 5.5-year follow-up.  Part 1.  Impairments and perceived disability.  Acta Orthop Scand Suppl 279:12-18, 1998.
5. Merskey H, Bogduk N.  Classification of chronic pain: descriptions of chronic pain syndromes and definitions of pain terms, ed 2, IASP Press, Seattle, 1994.
6. Sandroni P Berud-Larson LM, McClelland RL, Low PA.  Complex regional pain syndrome type 1: incidence and prevalence in Olmstead County, a population-based study.  Pain 103:199-207, 2003.
7. Hemler DE, McAuley R, Belandres PV.  Common clinical presentations among active duty personnel with traumatically induced reflex sympathetic dystrophy. Milit Med. 153:493-495, 1988.
8. Harris J, Fallat L, Schwartz S.  Characteristic trends of lower extremity chronic regional pain syndrome. J Foot Ankle Surg 43:296-301, 2004.
9. Kemler MA, de Vet HC, Health-related quality of life in chronic refractory reflex sympathetic dystrophy (complex regional pain syndrome Type 1). J Pain Sympt Manag 20:68-76, 2000.
10. Burton AW, Bruehl S, Harden RN, Current diagnosis and therapy of complex regional pain syndrome: refining diagnostic criteria and therapeutic options.  Expert Rev Neurother  5:643-651, 2005.

Author(s) and date

Dr. Jeffrey OsterThis article was written by Myfootshop.com medical advisor Jeffrey A. Oster, DPM.

Competing Interests - None

Cite this article as: Oster, Jeffrey.  Chronic Regional Pain Syndromes.  https://myfootshop.com/article/complex-regional-pain-syndrome

Most recent article update:  December 10, 2020

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