1. Whatever happened to RSD?
Andrew Muir

2. 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)
1864 Mitchell described causalgia after treating casualties from American civil war. Pain often accompanied by including various sensory disturbances;
temperature and sweating changes; glossy and other disturbances of the skin, subcutaneous tissues, muscles and joints; paralysis; and involuntary movements.
1940 Evans described the term RSD. Evans envisaged that prolonged bombardment of pain impulses set up a "vicious circle of reflexes" in the spinal cord that generated efferent activity in the sympathetic system leading to spasm in the peripheral blood vessels. As a consequence there was leakage of fluid from the capillaries which eventually caused dystrophic changes in peripheral tissues.
The French surgeon Leriche had already noted that the limbs of patients with causalgia showed features that he thought reminiscent of vascular insufficiency. Because patients with ischaemic limbs were often treated by sympathectomy, Leriche argued by analogy that causalgia was due to an "irritation of the sympathetic" and might be alleviated by sympathectomy. These notions were later extended to RSD, although few noted that Leriche was later to retract his hypothesis.

3. 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.

4. 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.

5. 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.

6. 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.

7. 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.

8. Pathophysiology:
Sympathetic nervous system elaboration of noradrenalin can activate mast cells, inviting a immuno-inflammatory aspect to this.

9. Na+ Ca++ Ca++ Mao et al, Pain, 1995 AMPA-R NMDA-R mGluR G Mg++ IP3SP
Glu
AMPA-R
NMDA-R
mGluR G
Mg++
Na+
IP3
Ca++
Ca++
L-arg
PKC
activation
Gene
expression
Nos NO

10. Practical Clinical Features:
Pain
Allodynia
Temperature change
Colour change
Sweating
Dystrophy
Motor change
Non dermatomal
Should be marked
Uncommon
Non-specific

11. 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?

12. 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)

13. 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 !

14. 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: (now more recent)

15. 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

16. Frequency of Treatments

17. Pharmacotherapy

18. Timing of treatment
97% believed better outcome if referred within 3 months of onset

19. 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

20. 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

21. What do we know? IVRB guanethidine is ineffective,
bretyllium works (single trial)
Ketanserin effective
Ketorolac effective (1 paper)

22. 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

23. 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

24. 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

25. Case 1: History Investigations Referred to pain clinic
x-ray, bone scan, ultrasound
inflammatory markers
Referred to pain clinic

26. 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

27. 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

28. 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

29. 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

30. Case 2: History Pain increased No progress with hand therapy
burning, painful on light touch
extending up arm
No progress with hand therapy
Referred to pain clinic for SGBs

31. Case 2 : Examination Wearing glove
Holding arm up close to chest difficulty swinging arm/initiating movement
decrease grip strength
Hand cold blue sweaty, swollen

32. 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

33. 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

34. Case 2 : Management
further interventional Mx by pain specialist number 3
guanethidine blocks
Spinal cord stimulation
Unsuccessful

35. 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

36. Case 2 : Management
ASSESSED AS “NOT READY” for CBT based Pain Management Program

37. 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

38. 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

39. 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

40. 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

41. 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

42. Case 3 : Management Diagnosis? Management ??????????Nerve injury
?????????CRPS
??Conversion disorder
Management
Full assessment (multi-disc)
Counselling/ Reassurance
No medications, general gym program

43. 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

44. 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

45. 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

46. 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

47. 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)

48. 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

49. 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

50. 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

51. 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

52. 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

53. 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

54. 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