CRPS ImagesComplex Regional Pain Syndromes (CRPS)

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Definition of CRPS Type I

a syndromea syndrome initiating noxious eventinitiating noxious event not limited to the distribution of a single peripheral nervenot limited to the distribution of a single peripheral nerve disproportionate to the inciting eventdisproportionate to the inciting event associated with edema, vasomotor, sudomotor, associated with edema, vasomotor, sudomotor,

allodynia, and hyperalgesia in the region of painallodynia, and hyperalgesia in the region of pain

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Causes

TraumaTrauma

sprain, strain, dislocation, fracture, laceration, contusion, crush injury, surgery, manipulation, tight cast, occupational repetitive trauma

DiseaseDisease

intracerebral, intraspinal, nerve roots, ami, infection( joint, skin, periarticular), peripheral vascular

Idiopathic ( about 1/3rd of all the cases)Idiopathic ( about 1/3rd of all the cases)

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Epidemiology

Onset 9 – 85 years of ageOnset 9 – 85 years of age Median 42 yearsMedian 42 years Women 3x > menWomen 3x > men

Veldman PH, Reynen HM, Arntz IE: Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet 1993 Oct 23; 342(8878): 1012-6

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Modified from Blumberg, J. Auton. Nerv. Sys. 1983

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Pathophysiology

Sympathetically maintained painSympathetically maintained pain sympatholytic therapy abolishes pain and hyperalgesia sympatholytic blockade followed by administration of

adrenoceptor agonists, rekindles pain distal electrical stimulation of a freshly cut sympathetic nerve

induced pain in a patient with sympathetically maintained pain

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Pathophysiology(continued)

Ghostine et al - ephaptic transmissionGhostine et al - ephaptic transmission

erosion of nerve insulation -> abnormal internerve communication short circuiting between somatic afferents and sympathetic efferents

Bennett (NIH) - sprouting of damaged nervesBennett (NIH) - sprouting of damaged nerves

sensitive to norepinephrine will discharge upon exposure to norepinephrine sympathetic fibers as a source of norepinephrine produce norepinephrine receptors at damaged ends nociceptors in intact nerves fire more in response to norepinephrine

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Pathophysiology(continued)

Schwartzman et al. - autoimmune etiologySchwartzman et al. - autoimmune etiology

tissue injury -> nerve growth factor release -> activation of sympathetic neurons -> recruitment of neutrophils/monocytes -> complement activation -> interleukin 2

Roberts - sensitization of intraspinal wide dynamic range (WDR) neuronsRoberts - sensitization of intraspinal wide dynamic range (WDR) neurons

C fiber nociception A fiber mechanoreceptor sympathetic efferents C fiber blockade fails alleviation of SMP mechanoreceptor response to sympathetic activity

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Thalamus

WDR Neurons

Sympathetics

A Fiber Receptor

C Fiber Receptor

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Pathophysiology(continued)

Sympathetic postganglionic neuron/afferent neuron couplingSympathetic postganglionic neuron/afferent neuron coupling

direct noradrenergic coupling within traumatized nerve within dorsal root ganglion via microvascular bed indirect noradrenergic coupling ephaptic coupling

? Abnormal inflammatory response? Abnormal inflammatory response

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CLINICAL HISTORY

ANTECEDENT TRAUMAANTECEDENT TRAUMA WHEN WHERE TYPE SEVERITY NERVE INVOLVEMENT

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CLINICAL HISTORY (CONTINUED)

PAINPAIN BURNING, ACHING, THROBBING, STINGING, CONTINUOS WITH

EXACERBATIONS, “EXCRUTIATING”, “UNBEARABLE” SYMPATHETIC PAIN: CONSTANT, SPONTANEOUS, WORSE AT NIGHT,

WORSE WITH MOVEMENT, TACTILE AND THERMAL STIMULI IMMEDIATE OR DELAYED ONSET(WEEKS), GRADUAL INCREASE IN

INTENSITY PROPENSITY TO DIFFUSE, IPSILATERAL/CONTRALATERAL LIMB

INVOLVEMENT

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CLINICAL HISTORY (CONTINUED)

INITIAL DESCRIPTION OF PAIN ADEQUACY OF TREATMENT CHANGE IN CHARACTER/INTENSITY

IMMOBILIZATIONIMMOBILIZATION HOW LONG, TO WHAT EXTENT

HAS THE PRECIPITATING FACTOR RESOLVED? HAS THE PRECIPITATING FACTOR RESOLVED? VASOMOTOR CHANGES?VASOMOTOR CHANGES? SUDOMOTOR CHANGES?SUDOMOTOR CHANGES?

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CLINICAL HISTORY(CONTINUED)

TROPHIC CHANGES?TROPHIC CHANGES? PSYCHOLOGICAL COMPONENT?PSYCHOLOGICAL COMPONENT? LITIGATION?LITIGATION? PAST MEDICAL HISTORYPAST MEDICAL HISTORY

SYMPATHOLYTC MEDICATIONS FACTORS LIMITING PHYSICAL ACTIVITY NICOTINE, CAFFEINE

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PHYSICAL EXAMINATION

COMPLETE GENERAL EXAM COMPLETE GENERAL EXAM CARDIOPULMONARY VASCULAR NEUROLOGIC MUSCULOSKELETAL

GENERAL APPEARANCEGENERAL APPEARANCE AFFECT, MOODAFFECT, MOOD APPREHENSION, PROTECTIVE AND PAIN BEHAVIORSAPPREHENSION, PROTECTIVE AND PAIN BEHAVIORS

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PHYSICAL EXAMINATION

AFFECTED LIMBAFFECTED LIMB SYMMETRICAL VISUAL INSPECTION PALPATION MOTOR/SENSORY EXAM

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PHYSICAL EXAMINATION OF THE AFFECTED LIMB

VISUAL INSPECTIONVISUAL INSPECTION SWELLING DISCOLORATION

(ERYTHEMA, PALLOR, BLUISH MOTTLING, BRAWNY EDEMA)

HYPERHIDROSIS MUSCLE WASTING POSTURING JOINT ABNORMALITY EVIDENCE OF TRAUMA

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PHYSICAL EXAMINATION OF THE AFFECTED LIMB

skin thickening, wrinkling, flakingskin thinning, smoothing, tightening, shininghair coarsening, lengthening, increase in distributionnail thickening, ridging, weakening with accelerated

growth, growth asymmetryarthritic appearing joints

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Physical Examination: Palpation

Affected LimbAffected Limb allodynia hyperesthesia hyperalgesia warmth coolness sweaty coarse skin

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Physical Examination: Motor & Sensory Exam

Affected LimbAffected Limb weakness tremor fine motor movement decreased AROM/PROM allodynia hyperesthesia hyperalgesia

Unaffected AreasUnaffected Areas neck/shoulder stiffness trapezial spasm with shoulder elevation and loss of motion altered gait with subsequent hip and back pain

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Diagnostic Tests

SensorySensory Von Frey hairs, brush hairs, feather

Sudomotor Sudomotor ninhydrine sweat test, skin conductance response, cobalt blue

test SwellingSwelling

tape measure water displacement

Joint mobilityJoint mobility goniometer

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Diagnostic Tests

PsychologicalPsychological

External Motor Behavior (ADL, disability) Visual Analogue ScaleMcGill pain questionnaireMinnesota Multiphasic Personality Inventory (MMPI)

chronic pain profileorganic vs. nonorganic patient

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Diagnostic Tests

PsychologicalPsychological Illness Behavior Questionnaire

general hypochondriaillness convictionpsychological/somatic perceptionemotional inhibitiondysphoriarejectionirritability

Depression and Anxiety Tests

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Treatment

OverviewOverview Prevention Early Diagnosis Physical Therapeutics Pharmacological Therapeutics Psychological Therapy Prevention of Late Complications Outcome Measurement

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Treatment: Prevention

high risk patienthigh risk patient trauma cva nerve injury

early mobilizationearly mobilization AROM/PROM Braus

patents with stroke and hemiplegiaearly PT27% to 8% incidence of CRPS Type I

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Treatment: Early Diagnosis

improved outcomeimproved outcome high degree of suspicionhigh degree of suspicion early treatmentearly treatment

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Treatment: Physical Therapeutics

elevationelevation compressioncompression heat/coldheat/cold tens/ultrasoundtens/ultrasound stretching/AROM/PROMstretching/AROM/PROM stress loadingstress loading exercise(active/passive)exercise(active/passive)

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Treatment: Pharmacological Therapeutics

Components of PainComponents of Pain inflammatoryneuropathicsympatheticcentral nervous system

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Treatment: Pharmacological Therapeutics

Inflammatory Component Inflammatory Component NSAIDS

central effect of prostaglandinsIM/IV RB toradol - one study with good effect early phase intervention

Prednisone - early phase interventionefficacy comparable to sympatholytics 1 mg/kg (up to 100 mg/day), 2 week taper membrane stabilizing effectsbinding to lamina III and VII

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Treatment: Pharmacological Therapeutics

Neuropathic ComponentNeuropathic Component anticonconvulsants - disappointing tricyclics - paucity of trials gabapentin - at least one study: highly effective

CNS ComponentCNS Component opioids TCAs anticonvulsants NSAIDs, steroids

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Treatment: Pharmacological Therapeutics

CalcitoninCalcitonin ? mechanism of action in CRPS I moderate efficacy in some studies

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Treatment: Surgical Intervention

Chemical SympathectomyChemical Sympathectomy phenol, alcohol longer than sympathetic blockade pain recurs

Radiofrequency SympathectomyRadiofrequency Sympathectomy Endoscopic-guided SympathectomyEndoscopic-guided Sympathectomy Open Surgical SympathectomyOpen Surgical Sympathectomy Results: Results: 12-90% efficacy 12-90% efficacy 30% 30%

recurrencerecurrence Complications: Complications: sympathalgia in 7-44% of patientssympathalgia in 7-44% of patients

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Treatment: Prevention of Late Complications

muscle atrophy/weaknessmuscle atrophy/weakness osteoporosisosteoporosis contracturescontractures painpain

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

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

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

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

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Normal laser Doppler study of the upper extremities. Normal laser Doppler study of the upper extremities. When the patient performs inspiratory gasp repeatedly When the patient performs inspiratory gasp repeatedly during laser Doppler image acquisition, the transient during laser Doppler image acquisition, the transient capillary flow decreases are displayed easily and capillary flow decreases are displayed easily and dramatically (as dark bands) in the pseudocolor image. dramatically (as dark bands) in the pseudocolor image.

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Laser Doppler study of the upper extremities in a patient Laser Doppler study of the upper extremities in a patient with right hand reflex sympathetic dystrophy. with right hand reflex sympathetic dystrophy.

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Laser Doppler study of the lower extremities in a 25-Laser Doppler study of the lower extremities in a 25-year-old woman with reflex sympathetic dystrophy in the year-old woman with reflex sympathetic dystrophy in the right foot. right foot.