CRPS by Dr Ashok Jadon, MD, FIPP

8/10/2019 CRPS by Dr Ashok Jadon, MD, FIPP

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COMPLEX REGIONAL PAIN SYNDROME; POST SURGERY AND

TRAUMA

Dr Ashok Jadon, MD, DNB, MNAMS, FIPP

Ch!" Cons#$%an% and &OD

D!'ar%(!n% o" Ana!s%h!sa ) Pan R!$!" S!r*+!

Ta%a Mo%ors &os'%a$, Ja(sh!d'#r-./00.

1adona2r!d""(a$3+o(

In%rod#+%on4

Complex regional pain syndrome (CRPS) is a debilitating, painful condition in a limb,

associated with sensory, motor, autonomic and trophic (skin and bone) abnormalities. CRPSusually occurs after inury howe!er, it may occur without inury and, se!erity of trauma has no

relationship with occurrence of CRPS.

D!"n%on4CRPS is a !ariety of chronic neuropathic painful conditions usually following inury

that, exceeds both in magnitude and duration of the expected healing. "ccording to #"SP theterm CRPS has specific rele!ance for usage and each word describe an aspect of the disease$

a. C%&P'*!aried and dynamic clinical presentation

b. R+#%"'*non*dermatomal distribution of symptoms

c. P"#*out of proportion to the inciting e!entsd. S-R%&*constellation of symptoms and signs

A$%!rna%*! %!r(s "or CRPS4 CRPS also known by many names although they are now only of

historical significance.

(a) Reflex Sympathetic ystrophy / (promoted by 0ohn 1onica2 the founder of international

association for study of pain (#"SP).(b) Causalgia

(c) Reflex neuro!ascular dystrophy (R)

(d) "mplified musculoskeletal pain syndrome("&PS)(e) "lgoneurodystrophy

Pa%ho5!n!ss4CRPS is a multifactorial disorder clinically manifesting with$


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(a) eurogenic inflammation

(b) ocicepti!e sensation (causing extreme sensiti!ity or allodynia)

(c) 3asomotor dysfunction(d) &aladapti!e neuroplasticity generated by an aberrant response to tissue inury.

Some theories suggest that CRPS is associated with dysregulation of the CS 4 "utonomicner!ous system resulting in multiple functional loss, impairment and disability.

T6'!s o" CRPS4 5here are 6 !ariants of CRPS$

7. 5-P #$*formerly known as RS, Sudeck8s "trophy, R or "lgoneurodystrophy. #t is a

syndrome that de!elops after an initiating noxious e!ent that may or may not beassociated with period of immobili9ation.emonstrable ner!e lesions are not found.

:. 5-P ##$ * ;ormerly known as Causalgia.5here is e!idence of ob!ious ner!e damage

(partial or complete) in almost all cases.6. CRPS*%S< (not otherwise specified)$ partially meets CRPS criteria2 not better

explained by any other condition.


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the nearby oints. ue to long standing and progressi!e nature of disease patients often ha!e

associated psychological and psychiatric disturbances.

!olution of CRPS can be di!ided into three stages.

S%a5! /4 A+#%!$ 1egin after inury for up to 6 months characteri9ed by se!ere burning pain at

the site of inury.5here is associated muscle spasm, oint stiffness and restricted mobility. 5his

stage lasts a few weeks. #t can subside spontaneously or respond rapidly to treatment.S%a5! 84 S#9a+#%! or D6s%ro'h+$ #t lasts from >weeks to 7 year. 5he pain intensity increases

Swelling spreads, hair growth diminishes, nails become cracked, brittle,groo!ed and spotty

(mainly due to !asospasm),osteoporosis becomes se!ere.

S%a5! .4 A%ro'h+$ "fter >months to may be fore!er. #t is characteri9ed by irre!ersible changes

in the skin and bones. 5here is marked muscle atrophy and flexion contractures. 5he limb has

?woody8 feel on examination. 5he nails become brittle. %ccasionally, the limb is displaced from

its normal position. Se!ere osteoporosis with washed out appearance of bone is seen inradiographs.

Da5noss45he diagnosis of CRPS is mainly clinical and rests on identification of specific

clinical features with typical de!elopmental history and e!olution of symptomatic disease. ;ew

groups and in!estigators ha!e proposed criteria for obecti!ely diagnosing CRPS and arepresented below.

B#da'!s% da5nos%+ +r%!ra4 Results corroborate the !alidity of the 1udapest Criteria andsuggest they impro!e upon existing #"SP diagnostic criteria for CRPS.

7. Continuing pain, which is disproportionate to any inciting e!ent.

:. &ust 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. 3asomotor$ temperature asymmetry and =or skin colour changes and= or skin colourasymmetry

c. Sudomotor or oedema$ oedema and=or sweating changes, or sweating asymmetry

d. &otor or trophic$ decreased range of motion and=or motor dysfunction (weakness, tremor,

or dystonia), and=or trophic changes (hair, nails, or skin)

6. &ust 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, oroint mo!ement)

a. 3asomotor$ temperature asymmetry and=or skin colour changes and=or asymmetry

b. Sudomotor or oedema$ oedema and=or sweating changes and=or sweating asymmetryc. &otor or trophic$ decreased range of motion and= or motor dysfunction (weakness, tremor, or

dystonia) and=or trophic changes (hair, nails, or skin)

@. o other diagnosis better explains the signs and symptoms

5he diagnosis of CRPS is by exclusion and it should be differentiated with diseases of similar

clinical sign and symptoms

In*!s%5a%ons and da5nos%+ %!s%s4


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:/ SYMPAT&ETIC BLOC


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e. ;ibromyalgiae Psychiatric problems

a. Somatoform pain disordersb. &Enchhausen syndrome

TREATMENT4

:/ Ph6s+a$ and o++#'a%ona$ %h!ra'64 #n the acute stage physical therapy is the mostimportant factor in re!ersing the syndrome. #n sub*acute and chronic stages it works to impro!e

pain 4 function and help pre!ent oint stiffness and contractures. +oal directed therapy is

especially useful where the patient initiates treatment at whate!er point and with any therapyand then gradually increase acti!ity each week. Specific modalities used are transcutaneous

electrical ner!e stimulation, progressi!e weight bearing, tactile desensiti9ation, massage, and

contrast bath therapy.

:8 Dr#5s4 #nnumerable drugs ha!e been tried in CRPS successfully and many are still being

in!estigated. &ultiple drugs ha!e been used and include antidepressants, anti*inflammatory

drugs, bisphosphonates, !asodilators, +"1" analogues such as gabapentin and pregabalin,

alpha* or beta*adrenergic*blocking compounds, and the opioids.

:. Mrror 9o> %h!ra'64 &irror box therapy confuses patient to assume that his affected limb is

actually functional and may reinforce the effect of other therapies. #t uses a mirror box, tocreate a reflection of the normal limb such that the patient thinks they are looking at the

affected limb.

:? Ta+%$! ds+r(na%on %rann54 %riginally de!eloped for phantom limb pain. #n CRPS,

tactile training increases tactile acuity and reduces painand reco!ery is also associated with

normali9ation of cortical reorgani9ation.

:@In%ra*!no#s r!5ona$ s6('a%h!%+ 9$o+kad!4 #ntra!enous regional blocks with guanethidine

did not pro!e superior to placebo and associated with freAuent side effects. %ne study each with

ketanserin and bretylium showed potentially beneficial effects.

: Lo+a$ an!s%h!%+ s6('a%h!%+ 9$o+kad!4

Patients of CRPS with se!ere 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. " local anaesthetic solution can be inected by placing a needle tip in the proximity of

sympathetic structures such as the stellate ganglion (;igure*:) or lumbar sympathetic chain

('"S1) (;igure*6) under fluoroscopic or computed tomographic guidance.;or decades thesetechniAues were considered a ?gold standard8 in the treatment of patients with CRPS. ;or patients

suffering from CRPS refractory to con!entional treatment and sympathetic blocks, brachialplexus block or continuous epidural infusion analgesia coupled with exercise therapy may be

tried.

:S'na$ +ord s%(#$a%on4Spinal cord stimulation (SCS) is the newest therapeutic modality

acceptance and being used for CRPS. #t may also be surgically implanted to reduce the pain by


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directly stimulating the spinal cord. 5he mechanisms are unclear but may restore normal +"1"

le!els in the dorsal horn and affect the release of adenosine, thus reducing neuropathic pain.

:- N!#ra>a$ %!+hn#!s45hese are complicated and considered last resort measures to treat

CRPS. pidural local anaesthetics, clonidine, or opioids ha!e been studied in the management of

CRPS. #ntrathecal medications (morphine, bupi!acaine, clonidine or baclofen) deli!ered !ia animplanted pump has shown positi!e results.

Figure-!: CRPS of "eft foot treated with "u#bar

Figure-&: CRPS of "eft 'and treated with Stellate


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: S6('a%h!%+ d!n!r*a%on4 Patients who show good response to initial sympathetic blocks

will logically respond to radiofreAuency dener!ation, chemical destruction of the sympathetic

inner!ation, or surgical sympathectomy.

:/0 In%ra(#s+#$ar Bo%o> n1!+%ons4 #ntramuscular botulinum inections ha!e been shown

recently to benefit patients with CRPS symptoms locali9ed to one extremity. 5hese inectionsmay reduce the muscle spasm associated with CRPS and likely also decrease the inflammation

associated with CRPS. 5he main ad!antages of this treatment are that it is relati!ely cheap, safe,

and easy to administer. 5he maor disad!antage is that it may need to be repeated after a fewmonths.

:// N!#ro'a%h+ 'an (!d+a%on4 5hey are the essential ancillaries for any other treatment

being done for CRPS. 5he medications commonly used are tricyclic antidepressants, gabapentinand pregabalin, centrally acting medicationssuch as tramadol, opioids, and clonidine.

:/8 Ad1#n+%*! %r!a%(!n%4 %f unknown utility but commonly prescribed as a comprehensi!ecurati!e program at dedicated centresare + 1iofeedback, !arious forms of psychotherapy,

relaxation techniAues, and hypnosis that probably facilitate maintain compliance of patient thanactually ha!ing any curati!e effect.

:/. Ms+!$$an!o#s and n!=!r %h!ra'!#%+ o'%ons4 +raded imagery, repetiti!e transcranial

magnetic simulation, and hyperbaric oxygen, intra!enous infusion of iloprost andimmunoglobulin, sub*anaesthetic infusion of ketamine are newer options. #nterestingly it has

been found the incidence of CRPS is significantly reduced with use of axillary block or

intra!enous regional anaesthesiawith clonidine (and lidocaine). &icroneurography is being used

to disco!er the uniAue mechanism that causes the spontaneous pain of CRPS and may help inidentifying new pathways that can be potential targets in future.

:/? A('#%a%on4ot !ery well accepted among the fraternity but this remains the ultimate

option for intractable symptoms of a debilitating limb affected by CRPS. #t should be borne inmind 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.

Tr!a%(!n%s %o 9! a*od!d4 eep brain stimulation is ineffecti!e. Figh dose opioid should be

a!oided due to possible opioid induced hyperalgesia, addiction, di!ersion risk (the transfer of a

prescription drug from a lawful to unlawful channel of distribution or use) and o!er*dosage.

Pro5noss4 +ood progress can be made in treating CRPS if treatment is begun early, ideally

within three months of the first symptoms. #f treatment is delayed, howe!er, the disorder canAuickly spread to the entire limb, and changes in bone, ner!e, and muscle may become

irre!ersible. 5he prognosis is not always good. 5hose who ha!e cold CRPS, in which impairedthermoregulatory blood flow to the limb lea!es it colder than it counterpart, ha!e a worseprognosis than those with warm CRPS, in which excessi!e !asodilatation in the affected limb

lea!es it warmer than its counterpart. 'eft untreated the limb can experience muscle atrophy, loss

of use, and functionally redundant reAuiring amputation. CRPS will not burn itself out, but iftreated early, it is likely to go into remission. 5imely pain relief and inter!entional pain

procedures, as well as psychological support, are important. Gntil a mechanism is disco!ered and

a specific treatment for the syndrome is de!eloped, an interdisciplinary approach, including


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pharmacologic and inter!entional pain management in a step wise fashion, will likely remain as

the best route to follow.

CRPS by Dr Ashok Jadon, MD, FIPP

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