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 • 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 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 • 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 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: • 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.
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.
Pathophysiology: • Sympathetic nervous system elaboration of noradrenalin can activate mast cells, inviting a immuno-inflammatory aspect to this.
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
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 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 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 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 • 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 • 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 • 97% believed better outcome if referred within 3 months of onset
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? • 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 know? • IVRB • guanethidine is ineffective, • bretyllium works (single trial) • Ketanserin effective • Ketorolac effective (1 paper)
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 • 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 • 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: History • Investigations • x-ray, bone scan, ultrasound • inflammatory markers • Referred to pain clinic
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 • 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: 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 • 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: 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 • Wearing glove • Holding arm up close to chestdifficulty swinging arm/initiating movement • decrease grip strength • Hand cold blue sweaty, swollen
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 : 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 : Management • further interventional Mx by pain specialist number 3 • guanethidine blocks • Spinal cord stimulation • Unsuccessful
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 : Management • ASSESSED AS “NOT READY” for CBT based Pain Management Program
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 : 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 : 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 • 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 • 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 : Management • Diagnosis? • ??????????Nerve injury • ?????????CRPS • ??Conversion disorder • Management • Full assessment (multi-disc) • Counselling/ Reassurance • No medications, general gym program
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 • 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 : 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 • 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 • Management initial • Oxycontin/ gabapentin: Good analgesia • No improvement in function/spasm • Lumbar sympathetic block • Excellent block with no change in symptoms (SIP)
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 : 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 : 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