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  1. CRPS Images Complex Regional Pain Syndromes (CRPS)

  2. 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

  3. 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)

  4. 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

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

  6. 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

  7. 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 • 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

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

  9. Thalamus Sympathetics WDR Neurons A Fiber Receptor C Fiber Receptor

  10. Pathophysiology(continued) • Sympathetic 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

  11. CLINICAL HISTORY • ANTECEDENT TRAUMA • WHEN • WHERE • TYPE • SEVERITY • NERVE INVOLVEMENT

  12. 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

  13. CLINICAL HISTORY (CONTINUED) • 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?

  14. CLINICAL HISTORY(CONTINUED) • TROPHIC CHANGES? • PSYCHOLOGICAL COMPONENT? • LITIGATION? • PAST MEDICAL HISTORY • SYMPATHOLYTC MEDICATIONS • FACTORS LIMITING PHYSICAL ACTIVITY • NICOTINE, CAFFEINE

  15. PHYSICALEXAMINATION • COMPLETE GENERAL EXAM • CARDIOPULMONARY • VASCULAR • NEUROLOGIC • MUSCULOSKELETAL • GENERAL APPEARANCE • AFFECT, MOOD • APPREHENSION, PROTECTIVE AND PAIN BEHAVIORS

  16. PHYSICALEXAMINATION • AFFECTED LIMB • SYMMETRICAL VISUAL INSPECTION • PALPATION • MOTOR/SENSORY EXAM

  17. 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

  18. 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

  19. PhysicalExamination: Palpation • Affected Limb • allodynia • hyperesthesia • hyperalgesia • warmth • coolness • sweaty • coarse skin

  20. Physical Examination: Motor & Sensory Exam • Affected Limb • 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

  21. 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

  22. 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

  23. Diagnostic Tests • Psychological • Illness Behavior Questionnaire • general hypochondria • illness conviction • psychological/somatic perception • emotional inhibition • dysphoria • rejection • irritability • Depression and Anxiety Tests

  24. Treatment • Overview • Prevention • Early Diagnosis • Physical Therapeutics • Pharmacological Therapeutics • Psychological Therapy • Prevention of Late Complications • Outcome Measurement

  25. 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

  26. Treatment: Early Diagnosis • improved outcome • high degree of suspicion • early treatment

  27. Treatment: Physical Therapeutics • elevation • compression • heat/cold • tens/ultrasound • stretching/AROM/PROM • stress loading • exercise(active/passive)

  28. Treatment: Pharmacological Therapeutics • Components of Pain • inflammatory • neuropathic • sympathetic • central nervous system

  29. 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

  30. 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

  31. Treatment: Pharmacological Therapeutics • Calcitonin • ? mechanism of action in CRPS I • moderate efficacy in some studies

  32. 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

  33. Treatment: Prevention of Late Complications • muscle atrophy/weakness • osteoporosis • contractures • pain

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

  35. 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.

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

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

  38. 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.

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

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