complex regional pain syndrome dr jason brooks consultant anaesthesia and pain medicine belfast...

Complex Regional Pain Syndrome

Dr Jason Brooks

Consultant Anaesthesia and Pain Medicine

Belfast Trust

Orthopaedic Update

June 2013

CRPS

• What is it?• Diagnosis• Treatment – general

principles• Pain Clinic treatment

Dr Brooks www.paindocni.co.uk

Key Messages

• Clinical Diagnosis of exclusion• Uncertain cause• No specific treatment• Rehabilitation key treatment• Other treatments aimed to facilitate

above

Dr Brooks www.paindocni.co.uk

Health-care services involved in the care of patients with CRPS.

Goebel A Rheumatology 2011

• In 1993, the IASP introduced the term Complex regional pain syndrome to describe all pain states that previously would have been diagnosed as RSD or causalgia-like syndromes

Posttraumatic dystrophyCausalgiaMinor causalagiaSudek atrophyShoulder-hand syndromeReflex sympathetic dystrophy

CRPS

Dr Brooks www.paindocni.co.uk

• Complex: Varied and dynamic clinical presentation

• Regional: Non-dermatomal distribution of symptoms

• Pain: Out of proportion to the inciting events

• Syndrome: Constellation of symptoms and signs

• The term “sympathetic” was avoided in the revised definition because its contribution is not constant across patients

• CRPS pain may be “sympathetically maintained pain” (SMP)

• or “sympathetically independent pain” (SIP)

Dr Brooks www.paindocni.co.uk

• CRPS can be separated into two types based on the presence or absence of a nerve injury

• CRPS type I: A syndrome that develops after an initiating noxious event that may or may not be associated with a period of immobilization

• CRPS type II: Differs from CRPS type I by the presence of a known injury to a nerve or nerves

• Incidence: 26/100,000 life years Hip OA = 88 per 100,000 person years

• Female:Male ratio: 3-4:1• 80-85% have experienced preceding trauma

(fractures, surgery)

How common is CRPS?

deMos et al 2007

? 1-2% following #7-35% following colles2-5% following nerve injuryVeldman et al 1993

Natural History

• Natural history uncertain• 30% consider resolved by 6yrs• 50% disease stable• 15% no improvement• Later improvement less common with time

Dr Brooks www.paindocni.co.uk

deMos etal 2009

Signs% Symptoms%

Burning pain 80

Hyperethesia 65

Temperature diff 55 78

Colour changes 66 85

Sweating 24 52

Oedma 56 21

Nail Changes 9 24

Hair changes 8 18

Weakness 20 75

Tremor 9 23

Dystonia 14 20

Reduced ROM 70 80

Hyperalgesia 63

Allodynia 74

Features - Harden 2001

Dr Brooks www.paindocni.co.uk

Early CRPS of the right hand; clearly visible signs include swelling, red colour and a shiny skin.

Goebel A Rheumatology 2011

Dr Brooks www.paindocni.co.uk

Budapest Diagnostic criteria

A) Continuing pain disproportionate to initiating event

B) At least 1 sign in 2 or more categories

C) The patient symptoms in 3 or more categories

D) No other diagnosis can better explain the signs and symptoms

Sign>2

Symptom>3

1) SensoryAllodynia(to light tough or temperature deep somatic pressure or hyperalgesia to pinprick

2) Vasomotor Temperature asymmetry and or skin colour changes and or skin colour asymmetry

Must be > 1C

3) Sudomotor/Vasomotor

Oedema and or sweating and or sweating asymmetry

4) Motor/Trophic Decreased range of motion and or motor dysfunction

FeaturesPAIN

Spontaneous

Disproportionate to initiating event

Allodynia / Hyperalgesia (variability in reported prevalence)

Dr Brooks www.paindocni.co.uk

WHAT IS PAIN ?

This artwork represents my daily struggle with constant pain.

The only part of my body that does not hurt yet is still reaching out for help because I am not giving up. The artwork also glows in the dark representing the relentless nature of my pain 24/7

The Eradicator – Consumed by Chronic Pain

A Definition

“an unpleasant sensory and emotional experience associated with actual or potential tissue damage or described in terms of such damage”

International Association for the Study of Pain

A Definition

“an unpleasant sensory and emotional experience associated with actual or potential tissue damage or described in terms of such damage”

International Association for the Study of Pain

A Definition

“an unpleasant sensory and emotional experience associated with actual or potential tissue damage or described in terms of such damage”

International Association for the Study of Pain

A Definition

“an unpleasant sensory and emotional experience associated with actual or potential tissue damage or described in terms of such damage”

International Association for the Study of Pain

Pain is whatever the experiencing person says it is, existing whenever the experiencing person says it does

McCaffery

Disease Model Pain

Pain = tissue injury

Tissue damage = impairment = disability = incapacity work

Cure pain – disability will recover

Problem

Pain tissue injuryPain, disability and work incapacity not same thingDifferent people respond very differentlySocial issues profound influence

Biopsychosocial Model Pain

CultureSocial interactions

Sick role

Illness behaviourBeliefs, coping strategies

Emotionsdistress

NeurophysiologyPhysiologic dysfunction

(Tissue damage?)

SOCIAL

PSYCHO

BIO-

BIOLOGICAL

Psyc

Social

Pain Experience

Biopsychosocial Model Pain

Social

Psychological

Pain ExperienceBio

Biopsychosocial Model Pain

Sympathetically Maintained Pain

Proportion of CRPS symptoms improved with sympathetic blockade

If symp outflow to limb stimulus evoked pain in those who responded to block

Proposed coupling between sympathetic NS and afferent neurones

Peripheral couplingIndirect via vascular bedVia adrenal medulla

Vasomotor changes

Colour – red, cyanotic or pale

Typically temp in acute stages < 6mths

ß in chronic state

? Reliability of HISTORYOften difficult to examine /

variable

Thermography

Difference 0.6 Sens & Spec 67% (Bruehl 1996)

Difference 2 Sens 32 % spec 100% (Wasner 2002)

Diagnostic value increases if multiple sitesVery dynamic measures

Not a reliable clinical test

Sudomotor & Oedma

Increased or decreased sweat production

? Reliability of history? Clinical assessment

Sweat testing – research settingResting sweat output

Dr Brooks www.paindocni.co.uk

• Trophic– Advanced – atrophy skin/

nails– Demineralisation bone– 7% develop severe

changes / refractory

• Motor– Weakness, poor

coordination, tremor and myoclonus

– ? Related to disuse / neglect

Dr Brooks www.paindocni.co.uk

Other Investigations• QST

– Not specific– No additional diagnostic information

• Neurophysiological procedures

– CRPS - borderline delay NCV / distal motor latency• > 20% suggest underlying peripheral nerve lesion

– Useful to distinguish between CRPS &

– Is that important??

• Radiography– Demineralisation– ? Related to disuse– Considered non-specific & late– Not part of screening procedure

Three-phase bone scintigraphy

Dr Brooks www.paindocni.co.uk

Unilateral Uptake tracerHigh sensitivityLow Specificity

Not useful in the work –up of patients

Neither makes or excludes the diagnosis

Integrative conceptual model of CRPSCentral SensitisationDriver CRPSDynamic changes in spinal cord increasing transmission of pain signal

Inflammatory Process↑ inflammatory agentsNeurogenic inflammationSkin reddening / oedma

Cortical ReorganisationReduced sensory representation in homouculus altered.Improves with RxMirror Therapy / GMI

Autoimmune ConditionNovel conceptEvidence antineuronal Ab’sIVIG effective in reducing pain short term

Ischaemia reperfusion injurySome evidence for low oxygen tension in peripheral tissues

Goebel A Rheumatology 2011

Sympathetic Dysfunction

Management

The Four Pillars of Treatment in CRPS.

Goebel A Rheumatology 2011;rheumatology.ker202

DCRPSPain Mx oral/topical meds

Psychological Rx with focus on Education

InterventionalPain Mx

SNBIVRASomatic

Epidural/PlexusNeurostimIntrathecal

Surgical /

Rehab

ReactivationDesensitisation

IsometricFlexibilityOedma control

ROM, Stress LoadIsotonic Aerobic conditioning

Other

Psychological

Assess for axis 1Pain copingBiofeed/RelaxCBT

Freq or psycotherapy

Fai

lure

to P

rogr

ess Failure to P

rogress

Medication

Very little good data for CRPS

Initial - Codeine / Paracetamol / NSAIDS

Next Step – Antiepileptics / Antidepressants used in Neuropathic pain conditions

Anticonvulsants Pregabalin/ Gabapentin

Antidepressants Amitriptyline

Dr Brooks www.paindocni.co.uk

Opiates – Care with prescribing especially ↑ doses Not increase above equivalent 60 mg morphine per 24 hrs No short acting

Medication

Second / Third Line Therapies

Lidocaine patches

NMDA antagonist Ketamine

iv infusion 5 days

Topical capsaicin No evidence in trials but still used

Cannabinoids Nabilone

Other Medications

• Iv palmidronate• Early CRPS

• Vit C• Steroids

Dr Brooks www.paindocni.co.uk

Psychological interventionsAll pain conditions complex biopsychosocial

disorder

Pain Disuse/Emotional arousal

Several case reports / series reporting benefits

RCTs contain psychological therapy as part physical/medical therapy

Dr Brooks www.paindocni.co.uk

• In general:– Relaxation therapy– Coping skills– Behavioural intervention to address disuse– CBT– Active participant in therapy– Potentially as part of more formal Pain

Management Programme

Dr Brooks www.paindocni.co.uk

Physiotherapy / Rehabilitation

See Louis Talk!

Interventional therapies

• Stellate ganglion block• Lumbar sympathetic chain

• Intravenous Regional anaesthesia– Guanethidine– Bretyllium

Sympathetic Block

Dr Brooks www.paindocni.co.uk

Stellate Ganglion Block

• Upper Limb Chronic Regional Pain Syndrome Type I (CRPS I)

• Sympathetic supply to the ipsilateral arm / hemi-face

Lumbar Sympathetic Block

• sympathetic chains–overlying anterior portion of vertebral bodies

• posterior to aorta/IVC• anteromedial to kidneys/ureter

LSB Needle Insertion

Dr Brooks www.paindocni.co.uk

Intravenous Regional Sympathetic Block

• Depletion of norepinephrine in sympathetic nerve terminals

• guanethidine• bretylium

• IVRA–Guanethidine – essentially little evidence efficacy

BUT ALL THE STUDIES– entry criteria / all included

Dr Brooks www.paindocni.co.uk

Sympathetic nerve blocks

• Very little evidence benefit or evidence to base judgement

• Still used routinely

• ? role

Dr Brooks www.paindocni.co.uk

Titrate to responseAllow Physio therapy3 studies demonstrated improvements

Epidural Infusions

Epidural Infusions

Study No. Pts Infusion Results Complications

Cooper 89 14 Bupiv-opioid4 days

Improved pain/ROM 13/14

none

Konnig 95 29 Bupiv7 days

83% improved pain

Infection catheter site

Rauck 93 19 Clonidine3 days

Improved pain

InfectionsNauseaDizziness

Dr Brooks www.paindocni.co.uk

Brachial Plexus Catheter

Several Case reports (level 4 evidence)

1-3 weeksAllow physiotherapy

Even less evidence

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Spinal Cord StimulationMeta-analysis Grabow 2003

15 studies 1 RCT / 14 observational case series

Only 1 RCT – 50% response ( 50% relief) Kemler MA 2000

Suggest benefit still questions re efficacyUse if other Rx failed

NICE approved

Take Home Message

• Clinical Diagnosis• Uncertain cause• No specific treatment• Rehabilitation key treatment• Moderate/Severe CRPS needs

multidisciplinary team management!

Dr Brooks www.paindocni.co.uk

Dr Brooks www.paindocni.co.uk

NHS Website CRPS

Useful links www.paindocni.co.uk