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Reflex Sympathetic Dystrophy (CRPS I)

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Definition:• Complex regional pain syndrome (CRPS) is a chronic progressive disease characterized by severe pain, swelling and changes in the skin. [1]

• It often affects an arm or a leg and may spread to another part of the body.

• Though treatment is often unsatisfactory, early multimodal therapy can cause dramatic improvement or remission of the syndrome in some patients.[1] Hunter, Mackin, Callahan. Rehabilitation of the Hand and Upper Extremity, 5th Edn, Ch 104, Pg:1695

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Classification:The International Association for the Study of Pain has proposed dividing CRPS into two types based on the presence of nerve lesion following the injury.

• Type I, formerly known as reflex sympathetic dystrophy (RSD), does not have demonstrable nerve lesions.

• Type II, formerly known as causalgia, has evidence of obvious nerve damage.

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Synonyms for CRPS:

• Algodystrophy• Mimocausalgia• Peripheral trophoneurosis• Posttraumatic sympathetic dystrophy• Reflex neurovascular dystrophy• Shoulder-hand syndrome• Sudeck’s atrophy• Sympathetic neurovascular dystrophy

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CRPS I: Reflex Sympathetic Dystrophy

• RSD is a somewhat generic term used to describe post traumatic pain accompanied by inappropriate autonomic activity and impaired extremity function.

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(International Association for the Study of Pain)

"CRPS Type I is a syndrome that usually develops after an initiating noxious event, is not limited to the distribution of a single peripheral nerve, and is apparently disproportioned to the inciting event. It is associated at some point with evidence of edema, changes in skin blood flow, abnormal sudomotor activity in the region of the pain, or allodynia or hyperalgesia"

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Susceptibility

• CRPS can strike at any age, but the mean age at diagnosis is 42.

• CRPS has been diagnosed in children as young as 2 years old.

•  It affects both men and women; however, CRPS is 3 times more frequent in females than males.

• Investigators estimate that 2-5 percent of those with peripheral nerve injury, and 13-70 percent of those with hemiplegia will suffer from CRPS.

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Symptoms:• The most common symptoms overall are burning and electrical sensations, described to be like "shooting pain". 

• muscle spasms• local swelling, abnormally increased sweating• changes in skin temperature (usually hot but sometimes cold) 

• Changes in color (bright red or a reddish violet)• softening and thinning of bones• joint tenderness or stiffness, • and/or restricted or painful movement.

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Stages of Progression:

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Stage 1

• Stage one is characterized by severe, burning pain at the site of the injury. 

• Muscle spasm• joint stiffness• restricted mobility• rapid hair and nail growth, and • vasospasm

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Stage 2

• Stage two is characterized by more intense pain. • Swelling spreads• hair growth diminishes• nails become cracked, brittle, grooved, and spotty 

• osteoporosis becomes severe and diffuse• joints thicken• muscles atrophy.

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Stage 3

• Stage three is characterized by irreversible changes in the skin and bones

• Pain-unyielding, may involve the entire limb• marked muscle atrophy• severely limited mobility of the affected area• flexor tendon contractures• marked bone softening and thinning

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Diagnosis:

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The International Association for the Study of Pain (IASP) lists the diagnostic criteria for RSD as follows:

1. The presence of an initiating noxious event or a cause of immobilization

2. Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia (an exaggerated sense of pain)

3. Evidence at some time of edema, changes in skin blood flow

4. The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.

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Thermography

• Use of infrared radiation to view or locate over heated parts of the limb.

• Not a reliable tool for diagnosis• Results affected by smoking, drinking coffee, recent physical activity, topical lotions and creams, etc.

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Yoichi Koike, Hirotaka Sano. Changes with time in skin temperature of the shoulders in healthy controls and a patient with shoulder-hand syndrome. Upsala Journal of Medical Sciences. 2010; 115: 260–265

 “A thermography is unable to capture temperature changes over time. In contrast, a Thermocron is an effective measuring device to monitor temperature changes over time.

Thermocron is a more effective way to detect shoulder skin temperature abnormalities in a patient with shoulder-hand syndrome.

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Sweat testing

• A powder that changes color when exposed to sweat can be applied to the limbs

• However, this method does not allow for quantification of sweating. 

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Radiography

• Patchy osteoporosis- detected through X-ray imagery- as early as two weeks after onset.

• A bone scan of the affected limb may detect these changes even sooner. 

• Bone densitometry can also be used to detect changes in bone mineral density. 

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Electrodiagnostic testing

• EMG should not be done for the diagnosis of CRPS I or II. 

• It is extremely painful for patients suffering from CRPS and may even be considered unethical and cruel. 

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Assessment:

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• Presenting signs and symptoms:– Pain, including spontaneous burning pain, allodynia, hyperalgia.

– Sensory hyperasthesia– Tissue abnormality, including vasomotor instability, edema, skin color changes, subcutaneous bone and joint changes.

– Motor changes, including decreased ROM and weakness and movement disorders (tremor, dystonia and neglect)

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Hand & Orthopedic Rehabilitation services Pain profile

JOINT PAIN0= no pain1= mild pain with deep palpation2= severe pain with deep palpation3= severe pain with mild palpation4= hyperesthesia

PAIN QUALIFIERS Aching Burning Cramping Heaviness/fatigue Numbness Sharp/stabbing Stiffness Swelling Throbbing Tingling/pins and needles Weakness Other ______________

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• VAS; 10 cms line.• Body chart:

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Sensory evaluation

• Cutaneous Pressure SensationPathology Fingertip (mg) Thumb (mg) Palm (mg)

Normal 2.36-2.83 2.36-2.83 2.44-2.83

Diminished Light touch

3.22-3.61 3.22-3.61 3.22-3.84

Diminished Protective sensation

3.84-4.31 3.84-4.31 4.07-4.56

Loss of Protective sensation

4.56-6.65 4.56-6.65 4.74-6.65

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Differential Diagnosis:

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    Michael Stanton-Hicks. Complex regional pain syndrome. Anesthesiology Clin N Am 21 (2003) 733– 744.

    The distinction between CRPS I and II is the evidence of a definable nerve lesion. The signs and symptoms for both conditions, however, are clinically indistinguishable.

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MANAGEMENT

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Pharmacological interventions:

Physicians use a variety of drugs to treat RSD

• antidepressants• anti-inflammatory such as corticosteroids • COX-inhibitors such as piroxicam,• vasodilators• GABA analogs such gabapentin and pregabalin, • alpha- or beta-adrenergic-blocking compounds, and the entire pharmacy of opioids.

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Invasive techniques:

Local anaesthetic blocks/injections• Injection of lidocaine is often the first step in treatment. 

• Injections are repeated as needed. • Results- short lasting.

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• Neurostimulation (spinal cord stimulator) may also be surgically implanted to reduce the pain by directly stimulating the spinal cord. 

• These devices place electrodes either in the epidural space or directly over nerves located outside the central nervous system.

Spinal cord stimulators

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• Implantable drug pumps may also be used to deliver pain medication directly to the cerebrospinal fluid which allows powerful opioids to be used in a much smaller dose than when taken orally. 

Drug pump

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Sympathectomy

• Surgical, chemical, or radiofrequency sympathectomy — interruption of the affected portion of the sympathetic nervous system — can be used as a last resort

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Physiotherapy Management:

• Goal: ‘symptomatic treatment’

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Pain

• TENS• Thermal agents• Cryotherapy• Vibration• Splinting-dynamic• CPM

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Edema

• Elevation• Soft tissue manipulations• Active exercises• Compression- continuous

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Allodynia

• Vibration• Massage• Progressive weight bearing• Contrast bath• Desensitization

– Peripheral to central– Fine texture to coarse texture

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Dystonia and joint stiffness• Biofeedback and activities• Active exercises• Splinting

– Dynamic: pain and motion– Static: prevention, assist weak muscles– Static progressive: joint stiffness, tissue contracture

• CPM• Modalities

– Superficial heat with gentle stretch– Ultrasound with gentle stretch

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Vasomotor instability• Low-impact aerobic activities• Thermal biofeedback

VasoconstrictionThermal agentsMassageUltrasoundTENS(burst)Patient education-caffiene/nicotine    intake

VasodilationCryotherapyTENSNeural mobilizationPatient education- alcohol

Sayantika DharVolker et al. Interaction of Hyperalgesia and Sensory Loss in Complex

Regional Pain Syndrome Type I. July 2008, Volume 3, Issue 7, e2742. www.plosone.org.

They proposed three pathomechanisms of CRPS I, which follow a distinct time course: 

• Thermal hyperalgesia, observed in acute CRPS, indicates an ongoing aseptic peripheral inflammation. 

• Thermal hypoaesthesia, as detected in acute and chronic CRPS, signals a degeneration of A-delta and C-fibres, which further deteriorates in chronic CRPS. 

• Paradoxical Heat Sensation(PHS) in acute CRPS I indicates that both inflammation and degeneration are present, whilst in chronic CRPS I, the pathomechanism of degeneration dominates, signalled by the absence of PHS. 

Conclusion: Acute CRPS I :- Heat and Cold Pain thresholds reduced but Warm and Cold Detection thresholds were normalChronic CRPS I :- Thermal hyperalgesia declined. But Warm and Cold Detection thresholds deteriorated.

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Thacker, M., Gifford, L., 2002. A review of physiotherapy management of complex regional pain syndrome. Topical Issues in Pain, Vol. 3. CNS Press, Falmouth, pp. 119–141.

Use of electrotherapy may cause more symptoms in patients with mechanical allodynia through stimulation of large myelinated A fibers

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Chang-Zern Hong. Specific sequential Myofascial Trigger Point therapy in the treatment of a patient with Myofascial Pain syndrome with reflex Sympathetic dystrophy. ACO. Vol 9, No.1, March 2000.

MTrP for- wrist and finger extensors and anterior deltoid were commenced.

MTTrP can be used along with:o Intermittent cold (sprays or ice massage)o Stretching and post isometric relaxationo Deep pressure soft tissue massageo Thermotherapyo Trigger point injections with local anaesthetic solution or dry needling on

trigger points.

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