2. Historical Perspective
• During the Civil War, Silas Weir Mitchell
observed a chronic pain syndrome in
soldiers who suffered traumatic nerve
injuries
• Their symptoms included constant burning
pain and significant trophic changes

3. • He described this syndrome using the
term causalgia (from the Greek kausis – “burning” and
algos – “pain”)

4. • Half a century later, a French
surgeon
named Rene Leriche
implicated the sympathetic
nervous system in causalgic
pain.
• He treated these patients with
surgical sympathectomy.

5. • In the 1950’s, John Bonica (founder of the
IASP) introduced the phrase reflex
sympathetic dystrophy after noticing the
efficacy of temporary blockade of the
sympathetic nervous system in these
patients.

6. • There have since been many confusing terms
used to describe the condition:
•
•
•
•
•
•
•
•
•
•
•
•
Acute atrophy of the bone
Algodystrophy
Algoneurodystrophy
Postinfarctional sclerodactyly
Post-traumatic algodystrophy
Post-traumatic dystrophy
Post-traumatic sympathetic dystrophy
Pseudodystrophy
Reflex neurovascular dystrophy
Shoulder hand syndrome
Sudeck’s dystrophy
Sympathalgia etc.

7. • In 1993, the International Association for the
study of pain [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.

8. CRPS
• 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

9. • 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)

10. Sympathetically
maintained pain
Sympathetically
independent pain
Nociceptor input
maintained by
interaction
btw Primary afferents
and sympathetic
efferents through
alpha adrenergic
mechanism
Maintaining nociceptor
input comes from other
causes such as
peripheral nerve
stimulation
Eg: Neuroma

11. • 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 (without
a nerve injury)
• CRPS type II: Differs from CRPS type I by the
presence of a known injury to a nerve or nerves

12. Epidemiology
• Prevalence: 20.57/100,000
• Female : Male ratio: 3-4:1
• 80-85% have experienced preceding trauma
(fractures, surgery)
• 10% have experienced minor trauma
• 5-10% occur spontaneously

13. P
athophysiology

15. Clinical presentation
• Characteristic triad of symptoms comprising
autonomic, sensory, and motor disturbances.
• Triad can differ amongst individuals.
• Symptoms will generally change over time in a
given individual

16. • Distal edema – 80%
• Skin temperature changes – 80%
• The affected area is initially warm, but over the course of the
disease the skin temp decreases
• Skin color changes
• Initially red, becomes pale in chronic disease
• Altered sweating
• Increased sweating more common
• Nail and hair changes
• Increased growth in early disease

17. • Spontaneous pain:
• Often described as burning, aching, throbbing,
shooting, or deep pressure pain
•
•
•
•
Hyperpathia:
Hyperalgesia:
Allodynia:
Motor changes:
• Weakness, distal tremors, dystonia, myoclonus
• Not clear whether these are part of the clinical
presentation of the disease or a result of
protection/disuse of the painful limb

18. • Bony changes:
• Osteoporosis – periarticular distribution
• Joint stiffness

19. • Patients often have associated
psychological and psychiatric
disturbances

20. SPREADING SYMPTOMS
1.
•
A "continuity type" of spread where the symptoms
spread upward from the initial site, e.g. from the hand
to the shoulder.
•
A "mirror-image type" where the spread was to the
opposite limb.
•
An "independent type" where symptoms spread to a
separate, distant region of the body. This type of
spread may be spontaneous or related to a second
trauma.
•
*Total body RSD

21. Stages of CRP
S
Based on severity and chronology

24. Diagnosis
• Based exclusively on the characteristic clinical
features of the condition
• IASP diagnostic criteria for Complex Regional Pain
Syndrome:
• Presence of an initiating noxious event or cause of immobilization
• Continuing pain, allodynia, or hyperalgesia, with pain
disproportionate to any inciting event
• Evidence at some time of edema, changes in skin blood flow, or
abnormal sudomotor activity in the region of pain
• Diagnosis is excluded by the existence of conditions that would
otherwise account for the degree of pain and dysfunction

25. • A modified diagnostic criteria was proposed by the IASP
in 2007 to increase specificity (the ‘Budapest criteria’):
• Continuing pain that is disproportionate to any inciting event
• Must report at least one symptom in three of the four following
categories:
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–
–
–
Sensory: hyperalgesia, allodynia
Vasomotor: temp. asymmetry, skin color changes or asymmetry
Sudomotor/Edema: edema, sweating changes or asymmetry
Motor/Trophic: decreased range of motion, weakness, tremor, dystonia,
trophic changes
• Must display at least one sign at the time of evaluation in two or
more of the following categories:
–
–
–
–
Sensory: hyperalgesia, allodynia
Vasomotor: temp. asymmetry, skin color changes or asymmetry
Sudomotor/Edema: edema, sweating changes or asymmetry
Motor/Trophic: decreased range of motion, weakness, tremor, dystonia,
trophic changes
• No other diagnosis better explains the signs and symptoms

26. • Differential diagnosis:
•
•
•
•
•
•
•
•
Unrecognized local pathology (fracture, sprain)
Traumatic vasospasm
Cellulitis
Lymphedema
Raynaud’s disease
Thromboangiitis obliterans
DVT
Also, nerve entrapment syndromes and diabetic
neuropathy

27. Diagnostic tests
• Three-phase bone scintigraphy:
• Significant uptake in the metacarpophalangeal or
metacarpal bones appears to have high sensitivity
and specificity for CRPS
• The best timing for this study is in the subacute (up
to 1 year) phase of the condition

28. • Plain radiographs, x-ray bone
densitometry, and magnetic resonance
imaging have not been shown to be
sensitive or specific for CRPS

29. • Diagnostic tests used to assess for a
sympathetically maintained component:
• Sympathetic ganglia blockade
• Regional intravenous blockade with Guanethidine
• Phentolamine infusion test

30. Management of CRPS
• No scientifically validated cure exists
• Therapy is directed at managing the signs
and symptoms of the disease
• A multidisciplinary approach utilizing
pharmacotherapy, physical therapy and
psychological therapy is most appropriate

31. Individualize!
No protocol works for all patients
with CRPS. It is dependent on
current pain level, symptoms, and
tolerance

32. Medical Management
•
For constant pain associated with inflammation
–
•
For constant pain not caused by inflammation:
–
•
Oral opiods tried.
For the treatment of sympathetically maintained pain (SMP)
–
•
Anti-depressants (e.g. amitriptyline, doxepin, nortriptyline, trazodone, etc)
For spontaneous (paroxysmal) jabs
Anti-convulsants (e.g. carbamazepine, gabapentin may relieve constant pain as well)
For widespread, severe RSD / CRPS pain, refractory to less aggressive
therapies
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•
Agents acting on the central nervous system by an atypical mechanism (e.g. tramadol)
For constant pain or spontaneous (paroxysmal) jabs and sleep disturbances;
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•
•
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Nonsteroidal anti-inflammatory agents (e.g. aspirin, ibuprofen, naproxen, indomethacin, etc).
Clonidine .
For muscle cramps (spasms and dystonia)
–
–
Klonopin (clonazepam)
Baclofen

33. Physical/Occupational therapy
• Early physical therapy is essential to avoid
atrophy and contractures of the affected limb.
• PT/OT have been shown to reduce pain and
motor impairment, and improve function and
coordination ability of the limb.
• Requires that the patient take an active role in
their care

34. Nonpharmacological Option
•
•
•
•
•
Pain
– Modalities (moist heat,
fluidotherapy, contrast baths)
Edema
– Elevation with AROM, manual
edema mobilization,
compression, massage
Sensation
– Desensitization
Range of Motion
– PROM, stretching, blocking,
tendon gliding, PNF patterns
Splinting
– Resting position
•
•
•
Stress Loading
– “load and carry”
Joint Protection
Patient Education
– “To Improve, Move”

35. Psychological therapy
• An integral part of the multidisciplinary treatment
approach. Many patients with CRPS have a significant
amount of psychological dysfunction, which is a
reflection of the disease process itself as opposed to a
cause thereof.
•
•
•
•
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Pain coping skills
Biofeedback
Relaxation training
Cognitive behavioral therapy
Mirror Box therapy

36. Mirror Box therapy
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MIRROR VISUAL FEEDBACK:
This treatment is still experimental, but has helped in some cases.
A mirror is placed so that it reflects the opposite, unaffected limb, so
that it looks as if the affected limb is normal.
When the opposite limb is moved the person sees the affected limb
move in the mirror.
The affected limb can then also be felt to move. (This is called
kinesthetic sensations).
If this is repeated many times it may lead to normal movement of
the affected limb and reduction in pain.

37. Interventions
1.
2.
3.
4.
Sympathetic Blockade
Regional Block (Guanethidine)
Spinal cord stimulation
Morphine pump

39. Case report :
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•
•
•
25-year-old female patient with persistent pain and sclerotic and
edematous skin lesions in the left hand.
One year prior to her first visit, she had accidentally sprained her left
wrist. The initial pain subsided quickly, but after a month, severe
burning pain developed on her left thenar eminence with marked
swelling of the distal forearm and the hand.
pain gradually intensified and spread to the entire left hand,
accompanying numbness, severe cold intolerance and muscle
weakness.
She underwent physical therapy for several months at an orthopedic
clinic with no effect.

40. EXAMINATION:
•Pale-coloured, oedematous and
indurated skin with a shiny surface
was observed over the patient’s left
dorsal hand and fingers .
•The lateral 3 digits showed mild
flexion contracture.
• The dorsal skin over the left fingers
could not be pinched. Hair loss and
hypohidrosis were also observed.
•. Neurological examinations revealed
hyperalgesia, paresthesia, dysesthesia
and muscle weakness in the left
forearm and the hand.

41. laboratory test
•
•
•
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Routine laboratory test results were
unremarkable.
Nerve conduction studies were
normal.
Radiographic examination showed
marked decalcification of her left
lateral 3 fingers .
Thermography revealed a 2.6oC
lower temperature in the left dorsal
hand than in the right.., CRPS I was
diagnosed.

42. TREATMENT
• Treatment with regular stellate ganglion blockade was
initiated.
• The therapy proved to be effective in relieving pain and
hyperalgesia and in ameliorating the range of motion,

43. Conclusion
• CRPS is a complicated chronic pain syndrome
with variable clinical presentation and
complicated diagnostic criteria.
• Diagnosis is made on a clinical basis and
treatment is best managed with a
multidisciplinary approach including:
– medications,
– interventional procedures,
– physical therapy and psychological therapy

44. Thanks for your attention….