Complex regional pain syndrome

COMPLEX
REGIONAL PAIN
SYNDROME
- D R . P O O N A M N . B A N T H I A 1 S T M P T H
12:59:06 PM Complex Regional Pain Syndrome 1

CONTENTS :
• Definition
• Types of CRPS
• Pathophysiology
• Predisposing factors / Causes
• Symptoms and signs
• Stages of CRPS
• Diagnosis
• Treatment
• Prognosis

SYNONYMS FOR CRPS:
• Algodystrophy
• Mimocausalgia
• Peripheral trophoneurosis
• Posttraumatic sympathetic dystrophy
• Reflex neurovascular dystrophy
• Shoulder-hand syndrome
• Sudeck’s atrophy
• Sympathetic neurovascular dystrophy

HISTORY :
A. First described by Weir Mitchell after the American Civil War in 1872
• When he encountered soldiers who were injured by gun-shot wounds
exhibiting “bizarre” symptoms and coined the term “Causalgia”
B. Early 20th century Peter Sudeck
• Described features of pain, swelling, atrophy etc. following minor injury to
limbs – hence this phenomenon came to be called “Sudeck’s atrophy”
C. A few years on – its association with the sympathetic nervous system was
recognized and hence the term “reflex sympathetic dystrophy” was
increasingly used

DEFINITION
• RSD is defined as a pain state maintained by sympathetic efferent activation, by
circulating catecholamines, or by neurochemical action
CRPS
TYPE I
(RSD)
TYPE II
(CAUSALGIA)

TYPE I
• The presence of an initiating noxious
event, or a cause for immobilization
• Continuing pain and allodynia which is
disproportionate to any inciting event
• Evidence at some time of oedema,
changes in skin blood flow, abnormal
sudomotor activity in the region of pain
• The diagnosis is excluded by the existence
of other conditions that can account for the
degree of pain and dysfunction
• Criteria 2-4 must be satisfied
TYPE II
• Continuing pain, allodynia, or
hyperalgesia after nerve injury, not
necessarily limited to the distribution of
the injured nerve
• Evidence at some time of oedema,
changes in skin blood flow, abnormal
sudomotor activity in the region of pain
• The diagnosis is excluded by the
existence of other conditions that can
account for the degree of pain and
dysfunction
• All 3 Criteria must be satisfied

• Also called as CRPS I (RSD)
• Circumferential in distribution
• Good response to sympathetic blocks
• No response to nerve blocks
• Both thermal and mechanical allodynia
• Also called as CRPS II (CAUSALGIA)
• Follows discrete nerve distribution
• Variable response to sympathetic
blocks
• Responds well to nerve blocks
• Mechanical allodynia

Michael stanton-hicks. Complex regional pain
syndrome. Anesthesiology clin N am 21 (2003) 733–
744.
• The distinction between CRPS I and II is the evidence of a definable nerve lesion. The
signs and symptoms for both conditions, however, are clinically indistinguishable.

CAUSES :

PATHOPHYSIOLOGY :
• Normally, sympathetic stimulation secondary to injury results in
vasoconstriction, which decreases blood loss and swelling.
• Sympathetic tone then decreases after injury, allowing increased blood flow.
• In the abnormal situation (RSD), inappropriate continuation of sympathetic
activity results in edema, with capillary collapse, and ischemia, causing
continued pain (positive feedback loop).

SUSCEPTIBILITY :
• CRPS can strike at any age, but the mean age at diagnosis is 42.
• It has been diagnosed in children as young as 2 years old.
• It affects both men and women; however, CRPS is 3 times more frequent in
females than males.
• Investigators estimate that 2-5 percent of those with peripheral nerve
injury, and 13-70 percent of those with hemiplegia will suffer from CRPS.

PREDISPOSING
FACTORS :• The most common history given by the RSD patient is a trivial or minor initial injury
accompanied by disproportionate pain persisting long after healing.
• Compression neuropathy at any level can be complicated by RSD
• There is no way to predict who will get CRPS
• Very often, a minor trauma will trigger CRPS
• Post-operatively, if there is a painful limb, the more likely diagnoses are nerve injury
or compression, due to surgery or to the use of a tourniquet, infection, or an occult
fracture
• Immediate post-op pain is not CRPS I
• A tight cast may trigger CRPS I
• Type I : Colles fracture (lack of nerve involvement)
• Type II : Any Nerve trauma
median or tibial nerve

SYMPTOMS AND SIGNS :
• The predominant symptom is PAIN out of proportion to the initial injury.
• Primary signs :
Severe hyperalgesia: poorly localized, progressing to a diffuse
distribution that does not fit a dermatomal
distribution.
Edema : spreads proximally, changes from soft to hard (brawny)
edema with time.
Stiffness : rapid onset following swelling if swelling is not treated
early; fibrous ankylosis of joint with time

PAIN :
• Pain is constant, but varies in intensity
• Pain is circumferential, around entire foot or arm. The pain is not in a peripheral nerve
distribution (RSD)
• The pain will be in peripheral nerve distribution i.e. in affected nerve area
• Pressure on the ulnar, radial, median, tibial, sural, superficial and deep peroneal
nerves will be no more or less painful than pressure any where else on the limb
(RSD).
• Pain, OUT OF PROPORTION to the inciting cause.
• Occurring in one or more extremities is described as severe, constant, burning and/or
deep aching pain
• All tactile stimulation of the skin (e.g. wearing clothing, a light breeze) may be
perceived as painful (allodynia).
• Paroxysmal dysaesthesias and lancinating pains

• Osteopenia.
• Sudomotor or vasomotor changes (mottling, discoloration).
• Temperature changes (cold sensitivity).
• Trophic changes.
• Palmar fibrosis.
• Hyperhydrosis (sweating).
• Skin changes.
• Loss of skin creases.
• Loss of hair.
• Decreased moisture (late).
• Dystonic posture of affected limb
Secondary Signs :

Associated findings :
• Tremor.
• Weakness, muscle wasting (atrophy).
• Dystonia.

IASP REVISED CRITERIA –
“BUDAPEST CRITERIA”

3 PATTERNS OF 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

STAGES OF CRPS :
1. Stage I (Traumatic Phase)
a. Onset: at the time of initial injury or within several weeks
b. Increase in edema
i. Soft and localized
ii. Spreads to periarticular tissue, resulting in increasing stiffness
c. Hyperhidrosis
d. May see fine tremor (3 to 6 Hz)
e. Skin
i. Cool, pale, cyanotic, mottled
ii. May show erythema and warmth secondary to an increase in superficial blood flow
f. Increased hair and nail growth
g. Pain
i. Increased by movement and weight-bearing
ii. Voluntary disuse to minimize discomfort
iii. Increases in severity over time
iv. Diffuse
h. Osteopenia on x-rays within 4 to 8 weeks after onset of pain
i. Duration: 3 to 6 months

2. Stage II (Dystrophic Phase)
a. Onset: 3 to 6 months after onset of pain
b. Pain becomes more diffuse, increases in severity
c. Edema changes from soft to brawny edema (creases disappear)
d. Warmth followed by cyanosis
e. Trophic changes
i. Hair loss
ii. Nails brittle, cracked, grooved
iii. Glossy skin
iv. Decreased moisture
f. Joint range of motion (ROM) decreased, increased stiffness
g. Muscle weakness or atrophy
h. Less responsive to sympathetic blockade
i. Radiographs: osteopenia
j. Duration: 3 to 6 months

3. Stage III (Atrophic Phase)
a. Onset: 6 to 12 months after injury
b. Pain
i. Intractable; although it may decrease
ii. Proximal spread
iii. Worsens with motion
c. Joints
i. Decreased ROM or lack of functional motion
ii. Rigid (fibrous ankylosis)
iii. Flexor tendon contraction
iv. Joint subluxation
v. Potentially ankylosed
d. Skin
i. Marked trophic changes owing to decrease blood flow
ii. Cool, pale, dry, glossy
iii. Subcutaneous fat atrophy (fingers narrow)
e. Muscle atrophy
f. Radiographs
i. Diffuse
ii. Disuse osteoporosis or osteopenia
g. Duration: years or permanent12:59:06 PM Complex Regional Pain Syndrome 25

DIAGNOSIS

THREE PHASE BONE
SCANNING:
• The most reliable imaging modality for the diagnosis of RSD is three-phase bone
scanning.
• Periarticular or diffuse mottled demineralization is evident earlier on three-phase
bone scanning than on conventional radiographs.
Phase I: angiogram
Phase II: blood pool
Phase III: delayed 3 to 4 hours after injection
• For a scan to be considered diagnostic of RSD, the delayed phase must show
diffusely increased activity in the involved joints with periarticular accentuation in
the delayed phase

• Reflex sympathetic dystrophy of the foot. Increased
tracer uptake diffusely involving the lowermost right leg,
ankle, tarsals, metatarsals, and phalanges. Uptake is
less distally than proximally, but all areas are involved.

RADIOGRAPHY
Findings-
• Severely patchy Osteopenia particularly
in the peri-articular region (after 2-4
weeks of symptoms)
• Soft tissue swelling, with eventual soft
tissue atrophy
• Subperiosteal bone absorbtion
• Preservation of Joint spaces
• 1/3rd pts has normal X ray picture

THERMOGRAPHY
• Use of infrared radiation to view or locate
over heated parts of the limb.
• Not a reliable tool for diagnosis
• Results affected by smoking, drinking
coffee, recent physical activity, topical
lotions and creams, etc.
The left leg is so cold that it
doesn’t show
CRPS I

ELECTRODIAGNOSTIC
TESTING• EMG should not be done for the diagnosis of CRPS I or II.
• It is extremely painful for patients suffering from CRPS and may even be
considered unethical and cruel.

MANAGEMENT

4 PILLARS OF MANAGEMENT

TREATMENT :
• Initial treatment should focus on the initiating cause of pain if possible.
• The goal of treatment is interruption of the continuous feedback loop. A
multidisciplinary approach is essential.
• Early diagnosis is the best treatment.
• Delayed diagnosis may result in prolonged rehabilitation and the physical
changes secondary to established RSD.
• A result may be RSD that is more refractory to treatment.
• During the acute postinjury period, treatment may include NSAIDs, analgesics,
corticosteroid therapy (pulsed), and physical or occupational therapy

LIST OF TREATMENTS :
• Medical Mx
• Physiotherapy
• Blocks
• Surgical Mx
• Amputation : not typically recommened

MEDICAL MX
• Antidepressants
• Anti-inflammatory such as corticosteroids
• COX-inhibitors such as piroxicam,
• Vasodilators
• GABA analogs such gabapentin and pregabalin,
• Alpha- or beta-adrenergic-blocking compounds, and the entire pharmacy of opioids.

BLOCKS
• Local anaesthetic blocks/injections
• Injection of lidocaine is often the first step in treatment.
• Injections are repeated as needed.
• Results- short lasting
• Stellate ganglion block
• Lumbar block
• The use of sympathetic blockade in combination with physiotherapy leads to an
improvement in 80% of patients
• If sympathetic blocks provided 100% relief, but did not last, do a surgical
sympathectomy.

DRUG PUMP
• Implantable drug pumps may also be used to deliver pain
medication directly to the cerebrospinal fluid which allows
powerful opioids to be used in a much smaller dose than
when taken orally.

SURGICAL MX
SPINAL CORD STIMULATORS :
• Neurostimulation (spinal cord stimulator) may also be
surgically implanted to reduce the pain by directly
stimulating the spinal cord.
• These devices place electrodes either in the epidural
space or directly over nerves located outside the central
nervous system.

SYMPATHECTOMY :
• Surgical, chemical, or radiofrequency
sympathectomy — interruption of the affected
portion of the sympathetic nervous system —
can be used as a last resort

PHYSIOTHERAPY TREATMENT :
INDIVIDUALIZE !
No protocol works for all patients with CRPS. It is dependent on current
pain level, symptoms, and tolerance

PHYSIOTHERAPY TX
:
• The primary role of physiotherapy in the early stages of RSD is to help decrease
pain and prevent the development of stiffness.
• Therapy alone may be successful in mild cases.
• It is important to use early aggressive physical therapy and encourage motion.
• Orner and Thomas (1971) reported that 20% of patients with causalgia were treated
successfully with therapy that included elevation, traction, splinting, and
conditioning.
Goal: ‘symptomatic treatment’

PAIN
• TENS : can also be used in childrens
• Thermal agents & Cryotherapy : Heat or ice packs may be used but avoid extreme
temperatures.
• Splinting :
• Used to prevent contractures.
• Dynamic splints may be necessary in stages II/III disease for
stiffness.
• CPM

EDEMA
• Elevation of extremity.
• Place the hand in a position of function.
• A dependent extremity may have increased edema.
• Soft tissue manipulations
• Active exercise
• Massage.
• Compression garments.

ALLODYNIA
• Massage
• Progressive weight bearing
• Contrast bath
• Desensitization
– Peripheral to central
– Fine texture to coarse texture

DYSTONIA AND JOINT
STIFFNESS• Biofeedback and activities
• Active exercises :
-Active and active-assisted motion (not passive motion) are
essential.
-Minimize painful passive motion.
-Mobilize other areas of the extremities to prevent other joints
from stiffening and becoming painful
• Stretching
• Tendon gliding and PNF can also be given
• Splinting :
– Dynamic: pain and motion Used to prevent contractures.
It may be necessary in stages II/III disease for stiffness.
– Static: prevention, assist weak muscles
– Static progressive: joint stiffness, tissue contracture

• CPM
• Modalities
– Superficial heat with gentle stretch
– Ultrasound with gentle stretch
• Motor re-education and strengthening as motion permits.
• Mirror therapy
• "Stress-loading" program of Watson and Carlson (1987)
• Traction plus compressive exercises.
• Improved pain scores.
• Ninety-five percent improved motion, 84% returned to work, and 88%
decreased pain.
• Home exercise program is important.
• Inappropriate use of physical or occupational therapy may worsen symptoms

Thacker, M., Gifford, L., 2002. A review of physiotherapy
management of complex regional pain syndrome. Topical
issues in pain, vol. 3. CNS press, falmouth, pp. 119 141.
• Use of electrotherapy may cause more symptoms in patients with mechanical allodynia
through stimulation of large myelinated A fibers

PSYCHOLOGICAL THERAPY
• Patient education and support
• Goal setting
• Relaxation techniques
• Pacing techniques
• Coping skills
• Facilitating self-management of condition

PROGNOSIS :
• Completely individual
• The sooner treatment begins, the quicker improvements are noted
• Its best if the diagnosis is made and treatment begun in less than 6 months.
• The longer treatment is delayed, the more likely it is to require long-term
treatment.
• The prognosis is generally better in children than adults.

WORLD
ORANGE
DAY

The 6 blind wise men examining an elephant, in order to describe it to
their king. The king can visualize an elephant, only by integrating all the
descriptions.

Complex regional pain syndrome

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