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Whatever happened to RSD?. Andrew Muir. History . 1872 Mitchell described a syndrome of causalgia: Limbs of American Civil War soldiers who sustained nerve injuries Burning pain, hyperaesthesia, trophic changes with glossy skin

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history
History
  • 1872 Mitchell described a syndrome of causalgia:
    • Limbs of American Civil War soldiers who sustained nerve injuries
    • Burning pain, hyperaesthesia, trophic changes with glossy skin
    • The nomenclature relates to the Greek ‘kausis’ burning and ‘algos’ pain after a nerve injury
  • 1901 Sudeck (bone changes after injury)
  • 1940 Reflex Sympathetic Dystrophy (RSD)
crps nomenclature
CRPS: Nomenclature

The nomenclature of CRPS Types I, II was adopted after a Consensus Conference in 1993

    • Standardised terminology
    • Avoid unsustainable pathophysiological implications
    • Take up has been patchy but increasing: 11% of articles between 1995 and 1999 used it but 3.5% 1995 & 27.5% in 1999
  • Type II refers to major nerve injury, Type I to the rest.
crps diagnostic criteria
CRPS: Diagnostic Criteria
  • A. Presence of an initiating noxious event or cause of immobilisation.
  • B. Continuing pain, allodynia or hyperalgesia with which the pain is disproportionate to any inciting event.
  • C. Evidence at some time of oedema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain.
  • D. This diagnosis is precluded by the existence of conditions that would otherwise account for the degree of pain or dysfunction.
crps diagnostic criteria5
CRPS: Diagnostic Criteria
  • One group found that the criteria did not discriminate between CRPS I and Diabetic Peripheral neuropathy and positive predictive value between 40 and 60%.
  • Criteria used in a check list can improve PPV to 0.91, sensitivity to 0.71 and specificity to 0.95
  • Baron suggests current presence of 3 symptoms and 2 signs.
pathophysiology
Pathophysiology:
  • It can be shown that cooling the body with affected limb isothermic causes pain associated with sympathetic tone.
  • Controversial pharmacological challenge of Raja etc
  • Some studies have demonstrated an overall decrease in sympathetic nervous system activity explaining the
    • Acute ‘hot’, hypercirculation phase
    • Chronic ‘denervation supersensitivity’ phase with the cold blue limb.
pathophysiology7
Pathophysiology:
  • Most of the following have been demonstrated in animal models of nerve damage.
  • Peripheral changes
    • Expression of adrenoceptors on a subset of C-fibres, OR
    • Noradrenaline mediated release of prostanoids
  • Central changes
    • ‘wind up’
    • Autonomic/somatic crosstalk & sprouting after nerve injury.
pathophysiology8
Pathophysiology:
  • Sympathetic nervous system elaboration of noradrenalin can activate mast cells, inviting a immuno-inflammatory aspect to this.
slide9

Glu

Glu

SP

Glu

Mao et al, Pain, 1995

NMDA-R

AMPA-R

mGluR

G

Mg++

Na+

IP3

Ca++

Ca++

L-arg

Gene

expression

PKC

activation

Nos

NO

practical clinical features
Pain

Allodynia

Temperature change

Colour change

Sweating

Dystrophy

Motor change

Non dermatomal

Should be marked

Should be marked

Uncommon

Non-specific

Practical Clinical Features:
practical clinical features11
Practical Clinical Features:

A continuum from:

Icy cold, immobile, dripping with sweat, profound allodynia

TO

Hey! The X-ray looks OK … so how come it still hurts?

practical clinical features12
Practical Clinical Features:
  • There exist a number of potential differential diagnoses, the most common and important one is DISUSE secondary to persistent pain, (where the clinical signs are likely to be less marked).
  • Unrecognized local pathology(sprain, #, sepsis, cellulitis, allergy)
  • Vascular insufficiency (Raynaud’s disease, thromboangiitis obliterans, thrombosis)
practical clinical features13
Practical Clinical Features:
  • In all cases, the aims of treatment must be considered through the same process as any other patient with chronic pain.
  • RESTORATION OF FUNCTION !
treatment algorithms
Treatment algorithms
  • Guideline published in 1998
    • Functional restoration
    • Physical and psychological methods
    • To move through to another modality if no response in defined period
      • Consensus report Complex Regional Pain Syndrome:
      • Guidelines for therapy Stanton Hicks et al Clin J of Pain 14: 155-66 1998 (now more recent)
response to algorithm
Response to Algorithm
  • 100 experienced pain specialists
  • Referral
    • 32% orthopaedic specialist
    • 12% neurologist, 12% GPs
    • 9% self referred, 9% anaesthetist
    • 8%neurosurgeon, 8%physiotherapist
    • 6% lawyer/ case manager
    • 4% podiatrist
timing of treatment
Timing of treatment
  • 97% believed better outcome if referred within 3 months of onset
evidence based guidelines
Evidence based guidelines
  • Don’t really exist
  • Cochrane data base of RCTs
  • Critical analysis of 22 RCTs
    • Poor methodology
    • Only looking at one modality
    • Difficult to compare
    • Calcitonin deceases pain of CRPS
      • Perez et al Journ of Pain and Sympt Mgt 21, No6, June 2001
what do we know
What do we know?
  • Oral corticosteroids are effective (2 papers, 1 RCT)
  • Bisphosphanates:
    • Alendronate improved bone density with a trend to decrease in pain and swelling
    • Clodronate improved pain substantially
  • Spinal cord stimulation – moderate improvement
  • Some support for:
    • DMSO cream
    • Epidural clonidine
    • Intravenous bretyllium, ketanserin
what do we know21
What do we know?
  • IVRB
    • guanethidine is ineffective,
    • bretyllium works (single trial)
    • Ketanserin effective
    • Ketorolac effective (1 paper)
a reasonable approach
A Reasonable Approach:
  • Physiotherapy – (rest or mobilisation)
  • Adequate analgesia
  • Early pulse of corticosteroids
  • Early referral to Pain Clinic for:

Repeated temporary sympathectomies

Epidural clonidine

Bisphosphanates

  • Long term management of chronic pain
case study 1 history
Case study 1: History
  • Mrs C
  • Italian woman 70 years old
    • History: 3mths ago gardening
    • Stick pierced palm R hand
    • Hot, swollen, dry, painful
    • Treated antibiotics, sling
    • deteriorated
case 1 history
Case 1: History
  • Referred to orthopaedic hand surgeon
    • ? Hysterical, ?CRPS type 1
    • unable to move arm, fingers
    • unable to hold knife and fork
    • unable to do washing, cooking
case 1 history25
Case 1: History
  • Investigations
    • x-ray, bone scan, ultrasound
    • inflammatory markers
  • Referred to pain clinic
case 1 examination
Case 1: Examination
  • Pain on light touch,
  • Increased reaction to pain in most of arm viz palm, classic tender points
  • Motor neglect.
  • All upper limb movements impaired
  • tissue swelling
  • temperature cooler than other limb
  • colour change
case 1 management
Case 1: Management
  • Management: Initial
    • TCA, oxycontin, physiotherapy
    • cease sling,
    • start hanging washing on clothes line
  • Series of 3 stellate ganglion blocks
    • Good response for some days with lasting improvement(SMP)
    • Combined with physiotherapy:
    • EMLA cream to palm, trigger point injections extensor origin
case 1 management28
Case 1: Management
  • Outcome good.
    • Swelling gone,
    • Movements substantially improved
    • Function: returned to most activities
    • Residual thickening of palmar flexion tendon middle finger
    • Swelling substantially reduced
    • Pain Medications ceased
case 2 history
Case 2: History
  • Mr U
    • Turkish man aged 48
    • Injured at work end 1999
    • conveyor belt fault results in open injury to R hand
    • laceration palmar branch of digital nerve
    • repair of digital nerve
case 2 history30
Case 2: History
  • Pain increased
    • burning, painful on light touch
    • extending up arm
  • No progress with hand therapy
  • Referred to pain clinic for SGBs
case 2 examination
Case 2 : Examination
  • Wearing glove
  • Holding arm up close to chestdifficulty swinging arm/initiating movement
  • decrease grip strength
  • Hand cold blue sweaty, swollen
case 2 management
Case 2 : Management
  • Diagnosis of CRPS type 2
  • Trial of oral medications
    • neuorpathic agents, SR opioids, TCAs
  • Trial of stellate ganglion blocks/ activation
    • temporary improvement (SMP)
    • poor compliance
  • Multi-disciplinary pain assessment
case 2 management33
Case 2 : Management
  • Not suitable for pain management
  • seeking cure
  • unresolved anger/ litigation
  • Referred for in-patient rehabilitation program (Plan: Cx epidural/ phys ther)
  • Unsuccessful
case 2 management34
Case 2 : Management
  • further interventional Mx by pain specialist number 3
    • guanethidine blocks
    • Spinal cord stimulation
  • Unsuccessful
case 2 management35
Case 2 : Management
  • Further deterioration
    • now back and leg pain, using stick
    • not working/ low function at home
    • depressed
    • arm wasted, sweaty hand, no movement
    • heavily involved with litigation,
    • still focussed on cure and blame
    • seeking multiple medical opinions
case 2 management36
Case 2 : Management
  • ASSESSED AS “NOT READY” for CBT based Pain Management Program
case 3 history
Case 3 : History
  • Mr M.R.
    • Aged 24, Australian born
    • Had a venipuncture from R cubital fossa (lateral aspect) November 2000
    • Felt pain shoot up to shoulder/ felt faint
    • 36hrs later woke up with clawed R hand
    • Has not been able to open hand since
    • Has not worked since
case 3 history38
Case 3 : History
  • Referred by GP for pain management
    • 2 overdoses
    • Had been working at previous job for 3 days prior to Venipunture
    • No real indication for VP
    • did not attend a doctor prior to VP
    • Litigation in progress against pathology firm
case 3 history39
Case 3 : History
  • Now living with grandparents who are “looking after him”
  • Has initiated referral to multiple specialists
  • No reports available
  • Difficulty contacting referring GP
  • Using self prescribed splints at night
case 3 examination
Case 3 : Examination
  • Presentation
    • agitated
    • conflicting history with Mother
    • Pain not a major complaint
    • Both hands cool sweaty
    • Holding R hand in tight claw
    • Resistance to opening
case 3 management
Case 3 : Management
  • No wasting in arm in general
    • Increased forearm muscle bulk
    • Possibly some wasting dorsum of hand
  • No difference in temperature, swelling, sweating
  • No allodynia
  • No motor akinesia of arm in general
  • Normal movements of shoulder and upper arm. Cannot move fingers
case 3 management42
Case 3 : Management
  • Diagnosis?
    • ??????????Nerve injury
    • ?????????CRPS
    • ??Conversion disorder
  • Management
    • Full assessment (multi-disc)
    • Counselling/ Reassurance
    • No medications, general gym program
case 3 management43
Case 3 : Management
  • Participating in competitive manner in Gym program
  • Enjoys being videoed
  • Has taken up a correspondence course (sports psychology)
  • Will have an EUA
  • Unable to get any reports
case 4 history
Case 4 : History
  • MRS B
    • 58 year old woman (Australian born)
    • Working as nurse in aged care
  • MCA 1997: injured shoulder and ankle(soft tissue)
  • Recovered, RTW
    • Persistent swollen R leg
    • Intermittent shoulder stiffness
case 4 history45
Case 4 : History
  • 1998 R leg gave way, fell
    • fractured ankle POP/ int fixn
    • pain and spasm swelling persistent problem when in POP
    • prolonged rehabilitation 2X 3 mths IP
    • persisting pain, swelling, spasm
    • 2 further operations
    • No progress, Referred to pain clinic
case 4 examination
Case 4 : Examination
  • Pleasant co-operative woman
    • Wearing rigid ankle brace/ using wheelchair
    • leg swollen, cool compared to L side
    • intense allodynia, skin dry, discoloured
    • multiple tender points over entire leg, back shoulder
    • out of brace grossly abnormal gait and devel of spasm on light touch/ movet
case 4 management
Case 4 : Management
  • Management initial
    • Oxycontin/ gabapentin: Good analgesia
    • No improvement in function/spasm
    • Lumbar sympathetic block
    • Excellent block with no change in symptoms (SIP)
case 4 management48
Case 4 : Management
  • Case conference Rehab/ Physio
  • in-patient admission: epidural opiate/ clonidine/ Local Anaesthetic
  • Allodynia/ spasm disappeared
  • gait re-training, gym program
  • ceased all analgesics
  • returned to normal activities
  • no splint/ no wheelchair
  • skin/ temp/ swelling abated
case 4 management49
Case 4 : Management
  • 12 months later
    • noted recurrence of spasm and pain
    • skin changes/ allodynia
    • trial hydrotherapy/ gym
    • finding this difficult,
    • further deterioration
    • requested epidural treatment
    • underwent multi-disc assessment
case 4 management50
Case 4 : Management
  • Cure focussed, not interested in CBT Program
  • Admitted for epidural
    • Similar response to previous
  • Pt anxious that found walking difficult.
    • Had persistent muscle cramp
  • Referral to IP rehab (Not accepted by TAC)
  • OP physio attempted: poor progress
case 4 management51
Case 4 : Management
  • became increasingly frustrated by TAC
  • Frustrated that not cured
  • Told that time to accept as chronic problem
  • Reacted to this
  • Now overall improvement, walking/ holidaying in USA
role of primary care physician
Role of Primary Care Physician
  • (1) DIAGNOSIS early
  • (2) Early Use of adequate analgesia to promote normal activity/ posture
    • active physio/ not passive/ gentle reactivation.
    • if physio cannot progress 1st step is increase in time based analgesia
  • (3) Early referral to Multi disciplin PU
    • urgent, not to go on long waiting list
be aware
Be Aware
  • Some pain specialists unimodal approach
    • diagnostician eg phentolamine infusion/ guanethidine block/ no response/ discharge
    • interventionist: blocks/ more blocks/ spinal cord stimulation/ no rehab/ psych
    • rehab/ no intervention/ pain relief
    • psych/ no intervention/ rehab
be aware54
Be Aware
  • Adequate education/ counselling
    • patients ill informed/ self help groups/ Internet: progressive disease
    • explanation of the importance of return to normal function
    • avoid surgery if possible/ only if appropriate and covered by analgesia
    • Role of cognitions/ depression/ litigation as mediating factors