COMPLEX REGIONAL PAIN SYNDROME; POST SURGERY AND

TRAUMA
Dr Ashok Jadon, MD, DNB, MNAMS, FIPP
Chief Consultant and HOD
Department of Anaesthesia & Pain Relief Service
Tata Motors Hospital, Jamshedpur-831003
jadona@rediffmail.com

Introduction:
Complex regional pain syndrome (CRPS) is a debilitating, painful condition in a limb,
associated with sensory, motor, autonomic and trophic (skin and bone) abnormalities. CRPS
usually occurs after injury however, it may occur without injury and, severity of trauma has no
relationship with occurrence of CRPS.
Definition: CRPS is a variety of chronic neuropathic painful conditions usually following injury
that, exceeds both in magnitude and duration of the expected healing. According to IASP the
term CRPS has specific relevance for usage and each word describe an aspect of the disease:
a.
b.
c.
d.

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

Alternative terms for CRPS: CRPS also known by many names although they are now only of
historical significance.
(a) Reflex Sympathetic Dystrophy (promoted by John Bonica; the founder of international
association for study of pain (IASP).
(b) Causalgia
(c) Reflex neurovascular dystrophy (RND)
(d) Amplified musculoskeletal pain syndrome(AMPS)
(e) Algoneurodystrophy
Pathogenesis: CRPS is a multifactorial disorder clinically manifesting with:

(a)
(b)
(c)
(d)

Neurogenic inflammation
Nociceptive sensation (causing extreme sensitivity or allodynia)
Vasomotor dysfunction
Maladaptive neuroplasticity generated by an aberrant response to tissue injury.

Some theories suggest that CRPS is associated with dysregulation of the CNS & Autonomic
nervous system resulting in multiple functional loss, impairment and disability.
Types of CRPS: There are 3 variants of CRPS:
1. TYPE I:-formerly known as RSD, Sudecks Atrophy, RND or Algoneurodystrophy. It is a
syndrome that develops after an initiating noxious event that may or may not be
associated with period of immobilization.Demonstrable nerve lesions are not found.
2. TYPE II: - Formerly known as Causalgia.There is evidence of obvious nerve damage
(partial or complete) in almost all cases.
3. CRPS-NOS* (not otherwise specified): partially meets CRPS criteria; not better
explained by any other condition.
*This subtype was added to capture any patients previously diagnosed with CRPS who now did
not meet criteria.
Symptoms/clinical presentation
Patient usually complains of spontaneous pain variedly described as burning, shooting or
stabbing, aching, and throbbing. Moving or touching the limb is often intolerable.Patient may
also experience muscle spasms, local swelling, and sensitivity to things such as water, touch and
vibrations (Figure-1).

Figure-1: A case of CRPS left hand with severe

allodynia, hand is covered to prevent pain due
to touch of air

On examination changes in skin temperature (usually hot but sometimes cold) along with joint
tenderness or stiffness and or restricted /painful movements are noticeable. The motor changes
include weakness, distal tremors, dystonia and myoclonus. Radiologically softening and thinning
of bones (osteoporosis).
Over a period of time loss of function; muscle and limb atrophy become evident with trophic
changes like reduced and abnormal nail growth, thin and glossy skin associated with reduced
elasticity and ulceration. Over prolonged period of immobility contractures & fibrosis develop in

the nearby joints. Due to long standing and progressive nature of disease patients often have
associated psychological and psychiatric disturbances.
Evolution of CRPS can be divided into three stages.
Stage 1: Acute : Begin after injury for up to 3 months characterized by severe burning pain at
the site of injury.There is associated muscle spasm, joint stiffness and restricted mobility. This
stage lasts a few weeks. It can subside spontaneously or respond rapidly to treatment.
Stage 2: Subacute or Dystrophic: It lasts from 6weeks to 1 year. The pain intensity increases
Swelling spreads, hair growth diminishes, nails become cracked, brittle,grooved and spotty
(mainly due to vasospasm),osteoporosis becomes severe.
Stage 3: Atrophic: After 6months to may be forever. It is characterized by irreversible changes
in the skin and bones. There is marked muscle atrophy and flexion contractures. The limb has
woody feel on examination. The nails become brittle. Occasionally, the limb is displaced from
its normal position. Severe osteoporosis with washed out appearance of bone is seen in
radiographs.
Diagnosis: The diagnosis of CRPS is mainly clinical and rests on identification of specific
clinical features with typical developmental history and evolution of symptomatic disease. Few
groups and investigators have proposed criteria for objectively diagnosing CRPS and are
presented below.
Budapest diagnostic criteria: Results corroborate the validity of the Budapest Criteria and
suggest they improve upon existing IASP diagnostic criteria for CRPS.

1. Continuing pain, which is disproportionate to any inciting event.

2. Must report at least one symptom in three (clinical diagnostic criteria) or four(Research diagnostic
criteria) of the following categories:
a. Sensory: hyperesthesia or allodynia
b. Vasomotor: temperature asymmetry and /or skin colour changes and/ or skin colour
asymmetry
c. Sudomotor or oedema: oedema and/or sweating changes, or sweating asymmetry
d. Motor or trophic: decreased range of motion and/or motor dysfunction (weakness, tremor,
or dystonia), and/or trophic changes (hair, nails, or skin)
3. Must display at least one sign at time of diagnosis in two or more of the following categories:
Sensory: hyperalgesia (to pinprick) and/or allodynia (to light touch, deep somatic pressure, or
joint movement)
a. Vasomotor: temperature asymmetry and/or skin colour changes and/or asymmetry
b. Sudomotor or oedema: oedema and/or sweating changes and/or sweating asymmetry
c. Motor or trophic: decreased range of motion and/ or motor dysfunction (weakness, tremor, or
dystonia) and/or trophic changes (hair, nails, or skin)
4. No other diagnosis better explains the signs and symptoms
The diagnosis of CRPS is by exclusion and it should be differentiated with diseases of similar
clinical sign and symptoms
Investigations and diagnostic tests:

(1) SYMPATHETIC BLOCKADE:

a. Stellate Ganglion Block(upper extremity)
b. Lumbar Sympathetic Block (lower extremity)
(2) Regional intravenous blockade with guanethidine
(3) OTHER DIAGNOSTIC TESTS:
a. Thermography:
b. Sweat testing: Abnormal sweating can be detected by several tests. A powder that changes
colour when exposed to sweat can be applied to the limbs; however, this method does not
allow for quantification of sweating.
c. Quantitative Sensory Testing and Autonomic Testing: Quantitative sensory testing
demonstrates increase in warm perception thresholds and decrease of cold pain thresholds in
patients with CRPS types I and II.
d. Radiography: Patchy osteoporosis due to disuse of the affected extremity may be present. It
is not sensitive or specific for CRPS.
e. Three phase bone scintigraphy: The best timing for this study is in the Sub acute (up to 1
year) phase of the condition. It is suggestive of CRPS but it is not diagnostic.
f. ELECTRODIAGNOSTIC TESTING: Electromyography (EMG) and Nerve Conduction
Studies (NCS) are important tests in CRPS in detecting nerve injury. CRPS is a "diagnosis
of exclusion", which requires that there be no other diagnosis that can explain the patient's
symptoms.
Differential Diagnosis of Complex Regional Pain Syndrome:
a. Inflammation
a. Erysipelas
b. Seronegative arthritis
c. Rheumatologic diseases
b. Vascular diseases
a. Thrombosis
b. Acrocyanosis
c. Atherosclerosis
d. Raynauds disease
c. Neuropathic Pain
a. Peripheral (poly)neuropathy
b. Plexopathy
c. Nerve entrapment
d. Radiculopathy
e. Post herpetic neuralgia
f. Deafferentation pain after CVA
d. Myofascial pain
a. Overuse
b. Disuse
c. Tennis elbow
d. Repetitive strain injury

e. Fibromyalgia
e. Psychiatric problems
a. Somatoform pain disorders
b. Mnchhausen syndrome
TREATMENT:
(1) Physical and occupational therapy: In the acute stage physical therapy is the most
important factor in reversing the syndrome. In sub-acute and chronic stages it works to improve
pain & function and help prevent joint stiffness and contractures. Goal directed therapy is
especially useful where the patient initiates treatment at whatever point and with any therapy
and then gradually increase activity each week. Specific modalities used are transcutaneous
electrical nerve stimulation, progressive weight bearing, tactile desensitization, massage, and
contrast bath therapy.
(2) Drugs: Innumerable drugs have been tried in CRPS successfully and many are still being
investigated. Multiple drugs have been used and include antidepressants, anti-inflammatory
drugs, bisphosphonates, vasodilators, GABA analogues such as gabapentin and pregabalin,
alpha- or beta-adrenergic-blocking compounds, and the opioids.

(3) Mirror box therapy: Mirror box therapy confuses patient to assume that his affected limb is
actually functional and may reinforce the effect of other therapies. It uses a mirror box, to
create a reflection of the normal limb such that the patient thinks they are looking at the
affected limb.
(4) Tactile discrimination training: Originally developed for phantom limb pain. In CRPS,
tactile training increases tactile acuity and reduces pain and recovery is also associated with
normalization of cortical reorganization.
(5)Intravenous regional sympathetic blockade: Intravenous regional blocks with guanethidine
did not prove superior to placebo and associated with frequent side effects. One study each with
ketanserin and bretylium showed potentially beneficial effects.
(6) Local anesthetic sympathetic blockade:
Patients of CRPS with severe pain, allodynia, that do not respond to medication and physical
therapy, a diagnostic block of the stellate ganglion or the lumbar sympathetic block can be
performed. A local anaesthetic solution can be injected by placing a needle tip in the proximity of
sympathetic structures such as the stellate ganglion (Figure-2) or lumbar sympathetic chain
(LASB) (Figure-3) under fluoroscopic or computed tomographic guidance.For decades these
techniques were considered a gold standard in the treatment of patients with CRPS. For patients
suffering from CRPS refractory to conventional treatment and sympathetic blocks, brachial
plexus block or continuous epidural infusion analgesia coupled with exercise therapy may be
tried.
(7) Spinal cord stimulation: Spinal cord stimulation (SCS) is the newest therapeutic modality
acceptance and being used for CRPS. It may also be surgically implanted to reduce the pain by

directly stimulating the spinal cord. The mechanisms are unclear but may restore normal GABA
levels in the dorsal horn and affect the release of adenosine, thus reducing neuropathic pain.
(8) Neuraxial techniques: These are complicated and considered last resort measures to treat
CRPS. Epidural local anaesthetics, clonidine, or opioids have been studied in the management of
CRPS. Intrathecal medications (morphine, bupivacaine, clonidine or baclofen) delivered via an
implanted pump has shown positive results.

Figure-2: CRPS of Left Hand treated with Stellate

Ganglion Block

Figure-3: CRPS of Left foot treated with Lumbar

Sympathetic Block

(9) Sympathetic denervation: Patients who show good response to initial sympathetic blocks
will logically respond to radiofrequency denervation, chemical destruction of the sympathetic
innervation, or surgical sympathectomy.
(10) Intramuscular Botox injections: Intramuscular botulinum injections have been shown
recently to benefit patients with CRPS symptoms localized to one extremity. These injections
may reduce the muscle spasm associated with CRPS and likely also decrease the inflammation
associated with CRPS. The main advantages of this treatment are that it is relatively cheap, safe,
and easy to administer. The major disadvantage is that it may need to be repeated after a few
months.
(11) Neuropathic pain medication: They are the essential ancillaries for any other treatment
being done for CRPS. The medications commonly used are tricyclic antidepressants, gabapentin
and pregabalin, centrally acting medicationssuch as tramadol, opioids, and clonidine.
(12) Adjunctive treatment: Of unknown utility but commonly prescribed as a comprehensive
curative program at dedicated centresare EEG Biofeedback, various forms of psychotherapy,
relaxation techniques, and hypnosis that probably facilitate maintain compliance of patient than
actually having any curative effect.
(13) Miscellaneous and newer therapeutic options: Graded imagery, repetitive transcranial
magnetic simulation, and hyperbaric oxygen, intravenous infusion of iloprost and
immunoglobulin, sub-anaesthetic infusion of ketamine are newer options. Interestingly it has
been found the incidence of CRPS is significantly reduced with use of axillary block or
intravenous regional anaesthesiawith clonidine (and lidocaine). Microneurography is being used
to discover the unique mechanism that causes the spontaneous pain of CRPS and may help in
identifying new pathways that can be potential targets in future.
(14) Amputation: Not very well accepted among the fraternity but this remains the ultimate
option for intractable symptoms of a debilitating limb affected by CRPS. It should be borne in
mind that mere absence of limb created by amputation may still fail to eradicate the symptoms if
central neurocircuitary get established. So, late amputations may not be beneficial at all.
Treatments to be avoided: Deep brain stimulation is ineffective. High dose opioid should be
avoided due to possible opioid induced hyperalgesia, addiction, diversion risk (the transfer of a
prescription drug from a lawful to unlawful channel of distribution or use) and over-dosage.
Prognosis: Good progress can be made in treating CRPS if treatment is begun early, ideally
within three months of the first symptoms. If treatment is delayed, however, the disorder can
quickly spread to the entire limb, and changes in bone, nerve, and muscle may become
irreversible. The prognosis is not always good. Those who have cold CRPS, in which impaired
thermoregulatory blood flow to the limb leaves it colder than it counterpart, have a worse
prognosis than those with warm CRPS, in which excessive vasodilatation in the affected limb
leaves it warmer than its counterpart. Left untreated the limb can experience muscle atrophy, loss
of use, and functionally redundant requiring amputation. CRPS will not "burn itself out", but if
treated early, it is likely to go into remission. Timely pain relief and interventional pain
procedures, as well as psychological support, are important. Until a mechanism is discovered and
a specific treatment for the syndrome is developed, an interdisciplinary approach, including

pharmacologic and interventional pain management in a step wise fashion, will likely remain as
the best route to follow.