This document provides an overview of Complex Regional Pain Syndrome (CRPS). It defines CRPS as a multi-symptom syndrome affecting one or more extremities that is usually out of proportion to the inciting cause. CRPS involves pain, changes in skin temperature or color, swelling, or restricted joint movement. It discusses the stages of CRPS from initial pain and swelling to potential long-term tissue damage. The document also outlines medical and surgical treatment options.

1. Complex Regional
Pain Syndrome
Aaron J. Mascarenhas
080201022

2. Background
• Recognized in during the civil war.
• The syndromes appearing under this
include Sudeck’s atrophy, Sympathetic
dystrophy, algodystophy, shoulder-hand
syndrome, causalgia.

3. common?
• Pain, OUT OF PROPORTION to the inciting
cause.
• Vasomotor instability
• Trophic skin changes
• Regional osteoporosis
• Functional impairment
Consesus Expert panel recommended: CRPS

4. Definition?
“CRPS is a multi-symptom, multi-system,
syndrome usually affecting one or more
extremities, but may affect virtually any part of
the body.”
-International Research Foundation For RSD/CRPS.

5. Etiology
A number of precipitating factors have been
associated with RSD / CRPS including:
•Trauma (often minor)
•Ischemic heart disease and myocardial infarction
•Spinal cord disorders
•Cerebral lesions
•Infections
•Surgery
•However, in some patients a definite precipitating
event can not be identified

6. Pathophysiology
• Sympathetic pain results from tonic activity in myelinated
mechanoreceptor afferents. Input causes tonic firing in neurons
that are part of a nociceptive pathway.
• Injury to central or peripheral neural tissue.
• Elevated levels of soluble tumor necrosis factor receptor 1
(sTNF-R1) and enhanced tumor necrosis factor-alpha activity in
patients with polyneuropathy with allodynia
• Distal degeneration of small-diameter peripheral axons may
be responsible for the pain, vasomotor instability, edema,
osteopenia, and skin hypersensitivity of CRPS-1.
• Cortical changes, suggesting a possible role in pathophysiology

8. Clinical features
1.PAIN
a. occurring in one or more extremities is
described as severe, constant, burning and/or
deep aching pain
b.All tactile stimulation of the skin (e.g. wearing
clothing, a light breeze) may be perceived as
painful (allodynia).
c. Paroxysmal dysaesthesias and lancinating pains

9. 2. SKIN CHANGES
a. Skin may appear shiny (dystrophy-atrophy), dry or scaly
b. Hair may initially grow coarse and then thin. Nails in the
affected extremity may be more brittle, grow faster and
then slower.
c. Rashes, Ulcers and Pustules
d. Abnormal sympathetic (vasomotor changes) activity may be
associated with skin that is either warm or cold to touch.
e. Increased sweating (sudomotor changes) or increased
chilling of the skin with goose flesh (pilomotor changes)

12. 3.SWELLING
a.Localised, initially pitting and later Brawny
b.Edema may be sharply demarcated along
a line on the skin surface.*

14. 4.MOVEMENT DISORDER
a.May develop dystonia
b.Tremors and involuntary jerking of
extremities may be present.
c.Disuse atrophy sets in natural history.

15. 1.SPREADING SYMPTOMS
a. A "continuity type" of spread where the symptoms
spread upward from the initial site, e.g. from the hand to
the shoulder.
b. A "mirror-image type" where the spread was to the
opposite limb.
c. 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.
d. *Total body RSD

16. When to make a
diagnosis?
CRPS Type 1:
1. Initiating noxious event or a cause of immobilization
2. Continuing pain, allodynia, or hyperalgesia disproportionate to the inciting event
3. Evidence at some time of edema, changes in skin blood flow, or abnormal
sudomotor activity in the area of pain
4. The diagnosis is excluded by the existence of any condition that would
otherwise account for the degree of pain and dysfunction.
CRPS Type 2:
• Continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily
limited to the distribution of the injured nerve
1. Evidence at some time of edema, changes in skin blood flow, or abnormal
sudomotor activity in the region of pain
2. The diagnosis is excluded by the existence of any condition that would
otherwise account for the degree of pain and dysfunction.

17. Stage I
1.Onset of severe, pain limited to the site of injury
2.Hyperesthesia
3.Localized swelling
4.Muscle cramps
5.Stiffness and limited mobility
6.At onset, skin is usually warm, red and dry and then it
may change to a blue (cyanotic) and become cold and
sweaty.
7. Hyperhydrosis
8. Lasts a few weeks, then subsides spontaneously or
responds rapidly to treatment.

18. Stage II
1.Pain becomes even more severe and more diffuse
2.Swelling tends to spread and it may change from a soft to hard
(brawny) type
3.Hair may become coarse then scant, nails may grow faster
then grow slower and become brittle, cracked and heavily
grooved
4.Spotty wasting of bone (osteoporosis) occurs early but may
become severe and diffuse
5.Muscle wasting begins

19. Stage III
1.Marked wasting of tissue (atrophic)
eventually become irreversible.
2.For many patients the pain becomes
intractable and may involve the entire limb.
3.A small percentage of patients have
developed generalized RSD affecting the
entire body.

20. Medical Management
For constant pain associated with inflammation
Nonsteroidal anti-inflammatory agents (e.g. aspirin, ibuprofen, naproxen, indomethacin, etc).
For constant pain not caused by inflammation:
Agents acting on the central nervous system by an atypical mechanism (e.g. tramadol)
For constant pain or spontaneous (paroxysmal) jabs and sleep disturbances;
Anti-depressants (e.g. amitriptyline, doxepin, nortriptyline, trazodone, etc)
Oral lidocaine (mexilitine - some what experimental)
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
Oral opiods tried.
For the treatment of sympathetically maintained pain (SMP)
Clonidine Patch.
For muscle cramps (spasms and dystonia)
Klonopin (clonazepam)
Baclofen

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