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Complex Regional Pain Syndrome

  • Also known as
    • RSD Reflex Sympathetic Dystrophy
    • Causalgia
Complex Regional Pain Syndrome
CRPS I = RSD CRPS II = Causalgia
Initiating noxious event or cause for immobilisation Initiating injury to nerve
Continuing pain or hyperalgesia disproportionate to initiating event Continuing pain, hyperalgesia or allodynia following nerve injury but not necessarily limited to distribution of the nerve
Evidence of oedema, cutaneous blood flow changes or abnormal sudomotor activity Evidence of oedema, cutaneous blood flow changes or abnormal sudomotor activity
Excluded by diagnosis that would account for degree of pain and dysfunction Excluded by diagnosis that would account for degree of pain and dysfunction

Types

  • Type 1: RSD
    • Sympathetically mediated pain syndrome
    • Excessive or exaggerated response of extremity to injury, surgery or disease
    • Manifested by
      • Intense or unduly prolonged pain
      • Vasomotor disturbances
      • Trophic changes
      • Delayed functional recovery
  • Type 2: Causalgia
    • Constant burning pain following injury to nerve plexus or peripheral nerve
  • Sudeck's Atrophy
    • Acute atrophy of bone associated with local injury
    • Associated with pain, swelling & loss of function
    • Pain in extremity following injury
    • Associated with marked spotty osteoporosis

Aetiology

  • Usually preceded by injury
    • more common after trivial injury rather than major
  • can be either sympathetically mediated or sympathetically independent
  • exact pathophysiology is unknown
  • may involve all motor, sensory, sympathetic and parasympathetic fibres
  • pathological changes are thought to occur in the spinal cord where abnormal connections form between motor / sensory / autonomic pathways
  • Most common Colles fracture
    • 25 %
    • 60 % with tight cast
  • Seen with crush injury
  • May be trivial & forgotten
  • May occur with Coronary Artery Disease
    • Like Frozen Shoulder
  • Shoulder-Hand Syndrome
    • Associated with partial or complete nerve injury
    • 10% cord or head injury

Pathology

  • Three factors
    1. Injury - Often trivial
    2. Diathesis
      • Some personalities predisposed
      • Anxious & hypersensitive
    3. Disturbance of centrally mediated autonomic regulation
  • Basis is excessive Sympathetic Efferent Activity
  • Numerous Theories
    1. Feedback Theory
      • Cycle of 3 factors
        • Chronic irritation of Peripheral Nerves 2° -> Trauma or soft tissue damage
        • Abnormal state of activity in interneurones
        • Continued stimulation of sympathetic & motor efferents
    2. Gate Control Theory
      • Disorder of inhibitory fine tuning
      • Cells in Dorsal Horn that modulate afferent transmission
      • Small stimulatory "C" & large inhibitory "A" fibres
      • Cortical feedback
      • Selective activation of stimulated fibres opens gate
    3. Peripheral X Stimulation Theory
      • Peripheral Nerve trauma leads to formation of synapse between sensory afferent & motor efferents
      • Allows for direct cross stimulation & cycle formation

Clinical Features

  • Mean duration of symptoms 32/12
  • Cardinal features
  • Burning pain out of proportion to injury
    • Swelling
    • Stiffness
    • Vasomotor discoloration
  • Autonomic
    • oedema, vascular, sudomotor
  • Sensory
    • allodynia
    • pain from non noxious stimuli to skin
  • Motor = spasm
  • UL commoner than LL

Stages

Stages of CRPS
  Stage 1 Stage 2 Stage 3
  Acute Dystrophic Atrophic
Time 0-3/12 3-6/12 > 6/12
Symptoms
  • Continued localised pain
  • Aggravated by stress
  • Proximal spread of pain
  • Intractable pain
Signs
  • Skin changes
    • Swollen & warm
  • sensory - allodynia
  • autonomic - wet with excess swelling
  • motor - joints have decreased ROM
  • Skin changes
    • Cool & dry
    • Mottled & dusky
    • Atrophic with shininess & decreased hair
  • Oedema of limb
  • Atrophy of skin, muscles & bone
  • Flexion contractures
Investigations
  • XR: normal
  • Bone scan +ve with Periarticular accentuation on delayed scan
    • Highly specific
    • Not predictive
XR: Early osteoporosis
  • XR:
    • Narrow joint space
    • Diffuse osteoporosis
 

Prognosis

  • sympathetically mediated CRPS has better prognosis than sympathetically independent CRPS
  • Mean duration of symptoms 32m

Management

  • Early = best results
  • Late = poor outcome
  • functional use of entire limb through supervised physiotherapy
  • diagnostic or therapeutic neural block
  • includes stellate ganglion; brachial plexus & IV guanethidine block
  • anti-neuropathic pain medication
  • Physical - stress loading
    • Early active ROM
    • Aggressive splinting
    • Avoid contractures
    • Deep friction massage
    • May enhance "A" fibres
    • TENS - May stimulate "A" fibres
    • Avoid - Passive ROM
      • Temperature extremes
  • Sympathetic Interruption
    • Regional Sympathetic Blockade
    • Almost always effective
    • If not effective consider another cause
    • Effect usually temperature
    • Multiple procedures usually required
    • If > 4 required, consider surgical sympathectomy

Stellate Ganglion Block

  • Technique
    • 0.25% Marcaine
    • Anterior paratracheal approach
      • At C6 Level ~ Cricoid cartilage
  • Success manifested by
    • Profound Horner's
    • Rapid onset of pain relief
    • Cool, dry hand
    • Followed by gentle physiotherapy

Intravenous block

  • Technique
    • Bier's block
    • Infusion of Guanethedine or Reserpine
  • Basis
    1. Guanethedine is false transmitte
      • Taken up by sympathetic nerve endings & displaces Noradrenaline
    2. Reserpine depletes sympathetic nerve ending stores of Noradrenaline by decreased storage vesicle reuptake

Surgical Sympathectomy

  • Indicated if partial /temperature relief from 4 blocks

Corticosteroids

  • Short high doses used
  • No controlled trials
  • MOA unclear
  • Significant Side Effects

Acupuncture

  • Effective in 90%
  • ? "Closes the gate"

Pharmacologic

  • Amitriptyline
  • Nifedipine - Peripheral vasodilation
  • Gabapentin
  • Oxycodone
  • Therapeutic Trial

    Amitriptylline

    Up to 50mg nocte

    Inc every 5 days

    Gabapentin

    Up to 600mg tds

    Slow introduction

    Mexiletine

    Up to 100mg tds

    Slow introduction

    Oxycodone

    5mg prn

    Convert to SR

Dorsal column stimulator

Intraspinal opioid

Amputation

  • Phantom Limb Pain
    • 50-75% incidence
    • early onset
    • diminishes with time
    • distally located
    • lower > upper
    • Mechanisms
      • peripheral
        • neuroma
        • spontaneous activity of peripheral nerve
      • spinal
        • disinhibition of dorsal horn neurons
        • expansion of receptive fields
      • supraspinal
        • cortical reorganisation
    • Treatment
      • Peri-operative regional anaesthesia
      • Anti-neuropathic pain medications
      • Revision of stump neuroma
      • Treatment of stump infection or pressure areas
      • Revision of prosthesis
Webpage Last Modified: 14 October, 2011