Complex Regional Pain Syndrome

  • Also known as
    • RSD Reflex Sympathetic Dystrophy
    • Causalgia
CRPS I = RSDCRPS II = Causalgia
Initiating noxious event or cause for immobilisationInitiating injury to nerve
Continuing pain or hyperalgesia disproportionate to initiating eventContinuing 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 activityEvidence of oedema, cutaneous blood flow changes or abnormal sudomotor activity
Excluded by diagnosis that would account for degree of pain and dysfunctionExcluded by diagnosis that would account for degree of pain and dysfunction
Complex Regional Pain Syndrome

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

 Stage 1Stage 2Stage 3
 AcuteDystrophicAtrophic
Time0-3/123-6/12> 6/12
SymptomsContinued localised pain
Aggravated by stress
Proximal spread of painIntractable pain
SignsSkin 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
InvestigationsXR: normal
Bone scan +ve with Periarticular accentuation on delayed scan
Highly specific
Not predictive
XR:
Early osteoporosis
XR:
Narrow joint space
Diffuse osteoporosis
Stages of CRPS

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

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