CRPS Images
Complex Regional Pain Syndromes (CRPS)
Definition of CRPS Type I
 a syndrome
 initiating noxious event
 not limited to the distribution of a single peripheral nerve
 disproportionate to the inciting event
 associated with edema, vasomotor, sudomotor,
allodynia, and hyperalgesia in the region of pain
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Causes
 Trauma
 sprain, strain, dislocation, fracture, laceration, contusion, crush injury,
surgery, manipulation, tight cast, occupational repetitive trauma
 Disease
 intracerebral, intraspinal, nerve roots, ami, infection( joint, skin,
periarticular), peripheral vascular
 Idiopathic ( about 1/3rd of all the cases)
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Epidemiology
 Onset 9 – 85 years of age
 Median 42 years
 Women 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 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 transmission
 erosion of nerve insulation -> abnormal internerve communication
 short circuiting between somatic afferents and sympathetic efferents
 Bennett (NIH) - sprouting of damaged nerves
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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 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) neurons
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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
Sympathetics
WDR Neurons
A Fiber Receptor
C Fiber Receptor
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Pathophysiology(continued)
 Sympathetic postganglionic neuron/afferent neuron coupling
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direct noradrenergic coupling
within traumatized nerve
within dorsal root ganglion
via microvascular bed
indirect noradrenergic coupling
ephaptic coupling
 ? Abnormal inflammatory response
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CLINICAL HISTORY
 ANTECEDENT TRAUMA
 WHEN
 WHERE
 TYPE
 SEVERITY
 NERVE INVOLVEMENT
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CLINICAL HISTORY (CONTINUED)
 PAIN
 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)
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 INITIAL DESCRIPTION OF PAIN
 ADEQUACY OF TREATMENT
 CHANGE IN CHARACTER/INTENSITY
IMMOBILIZATION
 HOW LONG, TO WHAT EXTENT
HAS THE PRECIPITATING FACTOR RESOLVED?
VASOMOTOR CHANGES?
SUDOMOTOR CHANGES?
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CLINICAL HISTORY(CONTINUED)
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TROPHIC CHANGES?
PSYCHOLOGICAL COMPONENT?
LITIGATION?
PAST MEDICAL HISTORY
 SYMPATHOLYTC MEDICATIONS
 FACTORS LIMITING PHYSICAL ACTIVITY
 NICOTINE, CAFFEINE
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PHYSICAL EXAMINATION
 COMPLETE GENERAL EXAM
 CARDIOPULMONARY
 VASCULAR
 NEUROLOGIC
 MUSCULOSKELETAL
 GENERAL APPEARANCE
 AFFECT, MOOD
 APPREHENSION, PROTECTIVE AND PAIN BEHAVIORS
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PHYSICAL EXAMINATION
 AFFECTED LIMB
 SYMMETRICAL VISUAL INSPECTION
 PALPATION
 MOTOR/SENSORY EXAM
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PHYSICAL EXAMINATION OF THE AFFECTED
LIMB
 VISUAL 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, flaking
skin thinning, smoothing, tightening, shining
hair coarsening, lengthening, increase in distribution
nail thickening, ridging, weakening with accelerated
growth, growth asymmetry
arthritic appearing joints
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Physical Examination: Palpation
 Affected Limb
 allodynia
 hyperesthesia
 hyperalgesia
 warmth
 coolness
 sweaty
 coarse skin
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Physical Examination: Motor & Sensory Exam
 Affected Limb
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weakness
tremor
fine motor movement
decreased AROM/PROM
allodynia
hyperesthesia
hyperalgesia
 Unaffected 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
 Sensory
 Von Frey hairs, brush hairs, feather
 Sudomotor
 ninhydrine sweat test, skin conductance response, cobalt blue
test
 Swelling
 tape measure
 water displacement
 Joint mobility
 goniometer
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Diagnostic Tests
 Psychological
External Motor Behavior (ADL, disability)
Visual Analogue Scale
McGill pain questionnaire
Minnesota Multiphasic Personality Inventory (MMPI)
chronic pain profile
organic vs. nonorganic patient
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Diagnostic Tests
 Psychological
 Illness Behavior Questionnaire
general hypochondria
illness conviction
psychological/somatic perception
emotional inhibition
dysphoria
rejection
irritability
 Depression and Anxiety Tests
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Treatment
 Overview
 Prevention
 Early Diagnosis
 Physical Therapeutics
 Pharmacological Therapeutics
 Psychological Therapy
 Prevention of Late Complications
 Outcome Measurement
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Treatment: Prevention
 high risk patient
 trauma
 cva
 nerve injury
 early mobilization
 AROM/PROM
 Braus
 patents with stroke and hemiplegia
 early PT
 27% to 8% incidence of CRPS Type I
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Treatment: Early Diagnosis
 improved outcome
 high degree of suspicion
 early treatment
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Treatment: Physical Therapeutics
 elevation
 compression
 heat/cold
 tens/ultrasound
 stretching/AROM/PROM
 stress loading
 exercise(active/passive)
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Treatment: Pharmacological Therapeutics
 Components of Pain
 inflammatory
 neuropathic
 sympathetic
 central nervous system
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Treatment: Pharmacological Therapeutics
 Inflammatory Component
 NSAIDS
central effect of prostaglandins
IM/IV RB toradol - one study with good effect
early phase intervention
 Prednisone -
early phase intervention
efficacy comparable to sympatholytics
1 mg/kg (up to 100 mg/day), 2 week taper
membrane stabilizing effects
binding to lamina III and VII
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Treatment: Pharmacological Therapeutics
 Neuropathic Component
 anticonconvulsants - disappointing
 tricyclics - paucity of trials
 gabapentin - at least one study: highly effective
 CNS Component
 opioids
 TCAs
 anticonvulsants
 NSAIDs, steroids
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Treatment: Pharmacological Therapeutics
 Calcitonin
 ? mechanism of action in CRPS I
 moderate efficacy in some studies
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Treatment: Surgical Intervention
 Chemical Sympathectomy
 phenol, alcohol
 longer than sympathetic blockade
 pain recurs
 Radiofrequency Sympathectomy
 Endoscopic-guided Sympathectomy
 Open Surgical Sympathectomy
 Results: 12-90% efficacy
30% recurrence
 Complications: sympathalgia in 7-44% of patients
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Treatment: Prevention of Late Complications
 muscle atrophy/weakness
 osteoporosis
 contractures
 pain
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 A 29-year-old woman with reflex sympathetic dystrophy
in the right foot demonstrates discoloration of the skin
and marked allodynia.
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 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.
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 A 68-year-old woman with complex regional pain
syndrome type II (causalgia).
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 A 36-year-old woman with right arm reflex sympathetic
dystrophy and dystonic posture (movement disorder).
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 Normal laser Doppler study of the upper extremities.
When the patient performs inspiratory gasp repeatedly
during laser Doppler image acquisition, the transient
capillary flow decreases are displayed easily and
dramatically (as dark bands) in the pseudocolor image.
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 Laser Doppler study of the upper extremities in a patient
with right hand reflex sympathetic dystrophy.
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 Laser Doppler study of the lower extremities in a 25year-old woman with reflex sympathetic dystrophy in the
right foot.
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