Complex Regional Pain Complex Regional Pain Syndrome Syndrome

&& Physiotherapy Physiotherapy

Dr. D. N. Bid [PT]Dr. D. N. Bid [PT]

Sarvajanik College of Physiotherapy,Sarvajanik College of Physiotherapy,

Rampura, Surat.Rampura, Surat.

HistoricalHistorical Perspective Perspective

• During the Civil War, Silas Weir Mitchell observed a chronic pain syndrome in soldiers who suffered traumatic nerve injuries

• Their symptoms included constant burning pain and significant trophic changes

• He described this syndrome using the term causalgia (from the Greek kausis – “burning” and algos – “pain”)

• Half a century later, a French surgeon

named Rene Leriche implicated the sympathetic nervous system in causalgic pain.

• He treated these patients with surgical sympathectomy.

• In the 1950’s, John Bonica (founder of the IASP) introduced the phrase reflex sympathetic dystrophy after noticing the efficacy of temporary blockade of the sympathetic nervous system in these patients.

• There have since been many confusing terms used to describe the condition:

• Acute atrophy of the bone• Algodystrophy• Algoneurodystrophy• Postinfarctional sclerodactyly• Post-traumatic algodystrophy• Post-traumatic dystrophy• Post-traumatic sympathetic dystrophy• Pseudodystrophy• Reflex neurovascular dystrophy• Shoulder hand syndrome• Sudeck’s dystrophy• Sympathalgia etc.

• In 1993, the International Association for the study of pain [IASP] introduced the term Complex regional pain syndrome to describe all pain states that previously would have been diagnosed as RSD or causalgia-like syndromes.

CRPS• Complex: Varied and dynamic clinical

presentation

• Regional: Non-dermatomal distribution of symptoms

• Pain: Out of proportion to the inciting events

• Syndrome: Constellation of symptoms and signs

• The term “sympathetic” was avoided in the revised definition because its contribution is not constant across patients

• CRPS pain may be “sympathetically maintained pain” (SMP) or “sympathetically independent pain” (SIP)

• CRPS can be separated into two types based on the presence or absence of a nerve injury

• CRPS type I: A syndrome that develops after an initiating noxious event that may or may not be associated with a period of immobilization (without a nerve injury)

• CRPS type II: Differs from CRPS type I by the presence of a known injury to a nerve or nerves

EpidemiologyEpidemiology• Prevalence: 20.57/100,000

• Female : Male ratio: 3-4:1

• 80-85% have experienced preceding trauma (fractures, surgery)

• 10% have experienced minor trauma

• 5-10% occur spontaneously

• There is no correlation between the severity of trauma and the degree of CRPS symptoms.

• No psychological factor or personality structure predisposing for CRPS has been identified, however, studies have demonstrated that up to 80% of CRPS patients had experienced ‘stressful life events’ close to the time of diagnosis.

Clinical presentationClinical presentation• Characteristic triad of symptoms comprising

autonomic, sensory, and motor disturbances.

• Triad can differ amongst individuals.

• Symptoms will generally change over time in a given individual

• Distal edema – 80%• Skin temperature changes – 80%

• The affected area is initially warm, but over the course of the disease the skin temp decreases

• Skin color changes• Initially red, becomes pale in chronic disease

• Altered sweating• Increased sweating more common

• Nail and hair changes• Increased growth in early disease

• A 29-year-old woman with reflex sympathetic dystrophy in the right foot demonstrates discoloration of the skin and marked allodynia.

• This photo shows the same patient as in the above image, following a right lumbar sympathetic block. Marked increase in the temperature of the right foot is noted, with more than 50% pain relief.

• A 68-year-old woman with complex regional pain syndrome type II (causalgia).

• A 36-year-old woman with right arm reflex sympathetic dystrophy and dystonic posture (movement disorder).

• Spontaneous pain:• Often described as burning, aching, throbbing,

shooting, or deep pressure pain

• Hyperpathia:

• Hyperalgesia:

• Allodynia:

• Motor changes:• Weakness, distal tremors, dystonia, myoclonus• Not clear whether these are part of the clinical

presentation of the disease or a result of protection/disuse of the painful limb

• Bony changes:• Osteoporosis – periarticular distribution

• Joint stiffness

• Patients often have associated psychological and psychiatric disturbances

PathophysiologyPathophysiology

• Three main hypotheses

• Facilitated neurogenic inflammation

• Autonomic dysfunction

• Neuroplastic changes within the CNS

DiagnosisDiagnosis• Based exclusively on the characteristic clinical

features of the condition• IASP diagnostic criteria for Complex Regional

Pain Syndrome:• Presence of an initiating noxious event or cause of

immobilization• Continuing pain, allodynia, or hyperalgesia, with pain

disproportionate to any inciting event• Evidence at some time of edema, changes in skin blood flow,

or abnormal sudomotor activity in the region of pain• Diagnosis is excluded by the existence of conditions that

would otherwise account for the degree of pain and dysfunction

• A modified diagnostic criteria was proposed by the IASP in 2007 to increase specificity (the ‘Budapest criteria’):

• Continuing pain that is disproportionate to any inciting event• Must report at least one symptom in three of the four following

categories: – Sensory: hyperalgesia, allodynia– Vasomotor: temp. asymmetry, skin color changes or asymmetry– Sudomotor/Edema: edema, sweating changes or asymmetry– Motor/Trophic: decreased range of motion, weakness, tremor, dystonia,

trophic changes

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

– Sensory: hyperalgesia, allodynia– Vasomotor: temp. asymmetry, skin color changes or asymmetry– Sudomotor/Edema: edema, sweating changes or asymmetry– Motor/Trophic: decreased range of motion, weakness, tremor, dystonia,

trophic changes

• No other diagnosis better explains the signs and symptoms

• Differential diagnosis:• Unrecognized local pathology (fracture, sprain)• Traumatic vasospasm • Cellulitis • Lymphedema • Raynaud’s disease • Thromboangiitis obliterans • Erythromelalgia • DVT • Also, nerve entrapment syndromes, occupational

overuse syndromes, and diabetic neuropathy

Diagnostic testsDiagnostic tests

• Three-phase bone scintigraphy:• Significant uptake in the metacarpophalangeal or

metacarpal bones appears to have high sensitivity and specificity for CRPS

• The best timing for this study is in the subacute (up to 1 year) phase of the condition

• Plain radiographs, x-ray bone densitometry, and magnetic resonance imaging have not been shown to be sensitive or specific for CRPS

• Diagnostic tests used to assess for a sympathetically maintained component:

• Sympathetic ganglia blockade

• Regional intravenous blockade with Guanethidine

• Phentolamine infusion test

TreatmentTreatment

ManagementManagement of CRPS of CRPS• No scientifically validated cure exists

• Therapy is directed at managing the signs and symptoms of the disease

• A multidisciplinary approach utilizing pharmacotherapy, physical therapy and psychological therapy is most appropriate

Pharmacologic therapyPharmacologic therapy

• Drugs demonstrated to be effective for CRPS based on randomized controlled trials, and their proposed mechanism of action:

– Prednisone (oral): anti-inflammatory, neuronal membrane stabilizer– Vitamin C (oral): antioxidant– Alendronate (IV): osteoclast inhibitor– Bretylium (IV): Autonomic ganglia blocker– Ketansarin (IV): serotonin and alpha receptor antagonist– Phentolamine (IV): alpha-1 receptor antagonist– Lidocaine (IV): sodium channel blocker– DMSO (topical): free radical scavenger– Calcitonin (intranasal): osteoclast inhibitor– Clonidine (epidural): alpha-2 receptor agonist– Baclofen (intrathecal): GABA-B receptor agonist

Physical/Occupational therapyPhysical/Occupational therapy

• Early physical therapy is essential to avoid atrophy and contractures of the affected limb.

• PT/OT have been shown to reduce pain and motor impairment, and improve function and coordination ability of the limb.

• Requires that the patient take an active role in their care

• Treatment objectives for CRPS are to:– minimize pain– minimize edema, – normalize sensation, – promote normal positioning, – decrease muscle guarding and– increase independence in all areas—mobility, work,

leisure and activities of daily living (ADL).

• Active weight bearing exercises are emphasized

PainPain

• Transcutaneous electrical nerve stimulation (TENS) may help some patients.

• This treatment, using electrodes placed on the painful area, causes a tingling sensation, which may reduce the pain.

Edema Edema

• Edema is managed using specialized garments (Jobst® garments, Isotoner® gloves, Coban™) and manual mobilization techniques.

• Stress loading and AROM activities are also fundamental in managing edema. Elevation of the extremity can be effective; however it can sometimes become part of a cycle of guarding and disuse.

Desensitization Desensitization • Desensitization techniques are implemented to assist

with normalizing sensation to the affected area. This consists of progressive stimulation with very soft material to more textured fabricsor materials.

• Stimulation can be graded from light touch to deep pressure and from consistent to intermittent with each material.

• Contrast baths that gradually broaden the temperature difference between the two can work toward tolerance of heat or cold.

PosturePosture

• Posture is an important component to consider in treating CRPS.

• Proper posture and alignment can minimize protective guarding of the extremity, promote balanced use of muscles and facilitate improved functional use of the affected extremity.

Stress LoadingStress Loading• Stress Loading consists of two principles: scrubbing and

carrying.

• A stress loading program promotes active movement and compression of the affected joints for a minimum of 3-5 consecutive minutes, three or more times each day.

• Though stress loading may initially produce an increase in pain or swelling of the extremity, after several days a decrease in symptoms will begin to be evident.

• Use of the affected extremity in daily tasks is encouraged throughout rehabilitation to inhibit muscle guarding and disuse atrophy.

ScrubbingScrubbing

• Scrubbing consists of moving the affected extremity in a back/forth motion while weight bearing through the extremity.

• The patient scrubs against a hard surface, keeping the bristles of the brush in constant contact with the surface, while maintaining constant pressure on the brush. The amount of weight placed through the affected extremity and the duration of the activity are gradually increased

• Scrubbing is performed with the patient in quadruped for upper extremity involvement and in elevated sitting or standing for lower extremity involvement.

• For upper extremity involvement, the patient holds a scrub brush with the affected hand. For lower extremity involvement, a long Velcro® strap can assist in fastening the brush to the bottom of the affected foot.

• Modifications can be made to enhance performance or compliance. For example, upper extremity scrubbing may be done standing at a table or counter. Persons with limited wrist extension may benefit from using a handled brush.

• The Dystrophile® can be used to gauge reliable performance. It is a device designed to facilitate consistent weight bearing and compliance during scrubbing by activating a light when the patient has reached the preset load.

CarryingCarrying

• Carrying or loading, is the second component in the stress loading protocol. Small objects are carried in the hand on the affected side, progressing to a handled bag loaded with increasingly heavier weight.

• Carrying should be performed throughout the day, whenever the patient is standing or walking.

• The lower extremity can be loaded in a variety of ways. Walking is an important loading technique if care is taken to ensure weight bearing through the affected leg during gait, especially when an assistive device is used.

• Increased weight bearing can be accomplished with verbal/ physical cueing or by having the patient carry a weighted object or bag on the affected side.

• Loading can also be facilitated by engaging the patient in activities that promote weight shifting and balance (i.e. ball toss) or by placing the non-affected foot onto a small footstool during static standing tasks.

Treatment ProgressionTreatment Progression

• Once the patient is actively engaged in an edema management and stress loading program, treatment can progress toward increasing functional use of the extremity.

• As the pain and edema decrease, the patient will be better able to tolerate and participate in AROM, coordination, dexterity and strengthening tasks. Proprioceptive neuromuscular facilitation (PNF) patterns are often well tolerated during treatment.

• The therapist can help the patient to gradually improve AROM and flexibility through gentle progression of active and active-resistive exercises or gait training.

• These should be done within the patient’s tolerance and avoided in insensate situations (as after a nerve block).

• Pacing and pain management techniques, such as appropriate rest breaks, alternating tasks, thermal or EMG biofeedback, diaphragmatic breathing and relaxation techniques can assist the patient in minimizing pain flares while participating in intensive rehabilitation.

TREATMENT SUMMARYTREATMENT SUMMARY• The overall role of the therapist during rehabilitation of

CRPS is to guide the patient through a program designed to minimize pain and edema while maximizing functional use of the extremity.

• As CRPS varies greatly in severity and duration, it is very important for the therapist to demonstrate enthusiasm, support and encouragement of the patient during the treatment process.

• The patient, in turn, must be actively involved in integration of treatment techniques into all daily activities to achieve optimal function of the affected extremity.

Psychological therapyPsychological therapy

• An integral part of the multidisciplinary treatment approach. Many patients with CRPS have a significant amount of psychological dysfunction, which is a reflection of the disease process itself as opposed to a cause thereof.

• Pain coping skills• Biofeedback• Relaxation training• Cognitive behavioral therapy• Mirror Box therapy

Mirror Box therapyMirror Box therapy

• MIRROR VISUAL FEEDBACK: • This treatment is still experimental, but has helped in some cases. • A mirror is placed so that it reflects the opposite, unaffected limb, so

that it looks as if the affected limb is normal. • When the opposite limb is moved the person sees the affected limb

move in the mirror. • The affected limb can then also be felt to move. (This is called

kinesthetic sensations). • If this is repeated many times it may lead to normal movement of

the affected limb and reduction in pain.

Invasive/Interventional therapyInvasive/Interventional therapy

• Sympathetic nerve blocks

Spinal cord stimulationSpinal cord stimulation

• RCTs have demonstrated a significant reduction in level of pain and improvement in functional status and quality of life in patients with SCS plus physical therapy compared to those undergoing physical therapy alone.

• Surgical/chemical sympathectomy• Surgical sympathectomy appears significantly more effective

than chemical sympathectomy

• Intrathecal baclofen pump

Algorithm Algorithm for the for the management management of CRPSof CRPS..

Resolution of this syndrome does not commonly occur, and the patient will need chronic pain management.

ConclusionConclusion• CRPS is a complicated chronic pain syndrome

with variable clinical presentation and complicated diagnostic criteria.

• Diagnosis is made on a clinical basis and treatment is best managed with a multidisciplinary approach including: – medications, – interventional procedures, – physical therapy and psychological therapy

• References:

• C Maihofner, F Seifert and K Markovic. Complex regional pain syndromes: new pathophysiological concepts and therapies. Eur J Neuro 2010, 17: 649-660

• Hsu. Practical management of complex regional pain syndrome. Am J Therap 2009, 16: 147-154

• QH Tran, S Duong, P Gertini, RJ Finlayson. Treatment of complex regional pain syndrome: a review of the evidence. Can J Anaesth. 2010, 57(2): 149-166

• M de Mos, MC Sturkenboom, FJ Huygen. Current understandings on complex regional pain syndrome. Pain Pract. 2009, 9(2): 86-89

• Benzon. Essentials of Pain Medicine and Regional Anesthesia. Chapters 46-47, 376-385

• Longnecker. Anesthesiology. Chapter 91, 2020-2041

Thanks for your attention….Thanks for your attention….