jurnal crps

7/25/2019 JURNAL CRPS

1/4

omplex

regional

pain

syndrome

ANN

E APRILE

CRNA, MSN

Royal

Oak,

Michigan

Complex

regional

pain

syndrome

(CRPS)

is a

progressive,

chronic

llness

that

is

enigmatic

because

the

mechanisms

or

its

pathogenesis

have

yet

to

be

determined.

Syndromes

synonymous

with

CRPS

are

reflex

sympathetic

dystrophy,

reflex

neurovascular

dystrophy,

causalgia,

algoneurodystrophy,

ympathetically

maintained

pain,

clenched

ist

syndrome,

and

Sudek s

syndrome.

The diagnosis

of CRPS

is

categorized

into three

stages:

acute,

dystrophic,

and

atrophic.

CRPS

is

most often

precipitated

by

peripheral

rauma

(crushing

njuries,

lacerations,

ractures,

sprains,

burns,

or

surgery)

to

soft

tissueor

nerve

complexes.

The

pathogenesis

or

CRPS has

been

speculated

as

being either

a

disease

process

of the

peripheral

nerves,

a disease

process

ofperipheral

oft

tissue,

or a

disease

process

of

the spinal

cord.

Patients

suffering

from

CRPS

may be

limited

in their

ability

to

function

in

a

self-directed,

independent

ashion.

longitudinal

tudy

of

CRPS

on

1,348 patients

revealed

that

96

of the

study

subjects

still

suffer

some

pain

and

disability

regardless

of

the

duration

of the

disease

or course

of

treatment.

Although

the primary

etiology

for

CRPS

is not

clearly

understood,

key

progress

has

been

made

in

terms

of

establishing

apsychological

as

well

as

therapeutic

reatment

plan

once

the

diagnosis

has

been

made.

Key words:

Causalgia,

complex

regional

pain

syndrome

(Type

I

and Type

II ,

neuropathic

pain,

reflex

sympathetic

dystrophy,

sympathetically

maintained

pain.

Introduction

Complex regional

pain

syndrome

(CRPS)

is a

pro-

gressive,

chronic

illness

that

is

enigmatic

because

the mechanisms

for

pathogenesis

are

yet

to be

de-

termined.

CRPS is

the most

recent acronym

for

a

syndrome

that

has been

historically

most

often

referred

to as

reflex

sympathetic

dystrophy

(RSD).

CRPS has

been

clinically

described

for

27 years;

however,

diagnosis is elusive

due

to

the inconsis-

tency of

presenting

clinical

manifestations

and dis-

cordant

labeling

largely

dependent

on the

field

of

specialization

to which

the

patient

was

referred.

Syndromes

synonymous

with CRPS

are

RSD,

re-

flex

neurovascular

dystrophy,

causalgia,

algoneu-

rodystrophy,

sympathetically

maintained

pain,

clenched fist

syndrome,

and

Sudek s

syndrome.1-3

A descriptive

term meaning

a complex

disor-

der

or

group

of

disorders

that may

develop

as a

consequence

of trauma

affecting

the

limbs,

with or

without

obvious

nerve

lesion.

CRPS

may

develop

after

visceral

diseases

and

central

nervous system

lesions or,

rarely,

without

any

antecedent

event.

It

consists

of

pain

and

related

sensory

abnormalities,

abnormal

blood

flow

and sweating,

abnormalities

in

the motor

system,

and changes

in the

structure

of

both

superficial

and deep tissues

trophi

changes).

It

is not

necessary

that all

components

December

1997/ Vol.

65/No.


2/4

are present.

It is

agreed

that the

name CRPS

is

used in a descriptive

sense and

not

to

imply spe-

cific underlying

mechanisms. It is based

upon

the

patient's history,

presenting symptoms,

and find-

ings

at

the

time

of diagnosis.

34

Pathogenesis

and diagnosis

Complex

regional pain syndrome

was

initially

described

in clinical

literature

during

the Civil

War era (1860s)

when

the

condition

was

diagnosed

in soldiers suffering

from gunshot wounds.

In

1864,

three

U.S. Army

surgeons

described

a

group of 11

soldiers

suffering

from similar

gunshot

wounds

in-

volving

peripheral nerves.

The

associated

symp-

toms described

for this group

included hyperalge-

sia,

allodynia, skin

temperature changes,

trophic

changes, skin

mottling,

and

sweating,

and

were cat-

egorized

as causalgia.

Complex regional

pain

syndrome

is most

often

precipitated

by

peripheral

trauma (crushing inju-

ries, lacerations,

fractures, sprains,

burns, or sur-

gery)

to

soft

tissue or

nerve complexes.

There

are

five major components

associated with the diagno-

sis of

CRPS.

These include

pain, edema, dysregula-

tion

of

autonomic function,

alterations in

motor

function, and

dystrophic changes. The

three-phase

technetium

bone scan

is

helpful in confirming

the

diagnosis

of CRPS for patients who fail

to meet the

clinical criteria for

diagnosis.

5

-

8

The

pathogenesis

for

CRPS

has

been specu-

lated

as

being

either

a

disease process

of

the pe-

ripheral

nerves,

a

disease

process

of peripheral soft

tissue, or a

disease

process

of the spinal cord. Pe-

ripheral

nerve involvement has

been

postulated

to

represent

a

cycle of peripheral

nociceptive stimu-

lation

leading

to reflexive spinal cord emission of

efferent

sympathetic

stimulation, whereas tissue

in-

jury has been speculated to produce artificial

syn-

apses between peripheral nerve

fibers

allowing

for

efferent transmission to sensory

afferent fibers.

The

role of

oxygen

free

radicals

has also

been

investi-

gated

in

relation

to disease

pathology

associated

with CRPS.

79

linical

course

The diagnosis

of

CRPS

represents

a

major

cause of disability

for

one

in

five CRPS patients.

The precipitating injury as

it relates to frequency

of

presentation is

stratified accordingly: fractures

(1-2 ),

postperipheral

nerve

injury (2-5 ),

myo-

cardial

infarction

(shoulder-hand

syndrome)

(5 ),

hypothermic

insult

(trench

foot) (5 ),

and

5 for

peripheral

revascularization of extremities.

0

Complex regional

pain syndrome is

catego-

rized

into three definable

yet

overlapping

stages:

acute,

dystrophic, and atrophic.

The first stage

(roughly

1 to 3

months) is characterized

by severe,

localized,

burning

pain,

focal

edema, muscle

spasm,

rapid

growth

of hair and

nails, stiffness or

restricted mobility,

hyperesthesia,

and

vasospasm

affecting

skin color and temperature.

The second

stage

(approximately

3

to

6

months) involves

exac-

erbation and diffusion of pain and edema,

dimin-

ished

hair

growth,

brittle nails,

deossification

of

bone, joint thickening,

and atrophy of

the muscles.

The third

stage

implies irreversible

trophic

changes, intractable pain

involving the entire

limb,

flexor tendon

contractions,

marked atrophy

of the

muscles,

severe

limitations

in

joint

and

limb mo-

bility,

and

marked

bone

deossification.

1112

ausalgia

The

diagnosis of causalgia (a

widely used

synonym for CRPS Type

II)

is

considered as

the

most dramatic form

of

this

illness. Affliction of

this severity

is

in

the majority

of

cases

linked

with

an incomplete injury

to the brachial plexus,

sci-

atic,

or median nerve.

The arms or thighs are

the

site

of insult

in

80

of these

cases,

with rapid

onset

of

pain

(usually within the first

month) following

initial

injury.

Some

authorities

differentiate

CRPS

Type I from

causalgia (CRPS Type II) based on

involvement

(or lack

of major nerve damage.

Patients

suffering from

CRPS experience pro-

found

emotional

distress and

behavioral changes.

These

patients experience pronounced

pseudo-

motor, vasomotor, and

trophic

alterations.

The

out-

come

for patients

symptomatic to

this

magnitude

is

favorable with

early intervention

utilizing sym-

pathetic blockade. Delay in providing timely treat-

ment may

lead to

osteoporosis and

atrophy

of

the

musculature

that becomes

refractory and

amena-

ble to

temporary

relief

only.

7

13

Exploration

of

symptoms

Pain

Pain

associated

with CRPS is classified

as

neuropathic,

may imply

diverse

causal

mecha-

nisms, and will

in

many cases manifest away

from

the original

site

of

injury.

Sympathetic

ctiv tion

of

primary afferent

chains represents a catchall

ra-

tionale

for the mode of

pain

transmission

attrib-

uted to CRPS.

4

It

is

speculated that interaction

between sympathetic

activity

and nociceptor sensi-

tivity

occurs

due to cross-over

transmission of sym-

pathetic

stimulation

to

pain

receptors.

Peripheral

neural trauma

results in demyelinization of local

nerve

endings

allowing

for

depolarization of noci-

ceptor fibers

through

efferent sympathetic

stimula-

tion.

3

Edema

Persistent

local edema

is a

major

component of CRPS

during

stages and

II. This

process is thought

to

be mediated by

cutaneous

Journal

of the

American Association of

Nurse Anesthetists


3/4

nerve stimulation

resulting

in the release

of the

neuropeptide

substance P

(SP). Neuropeptide SP

plays

a

dual

role

by

increasing

capillary

perme-

ability and promoting the release of histamine

from

mast cells. Subsequent vascular smooth mus-

cle relaxation results in localized

vasodilatation,

increased

blood

flow, and

extravasation

of plasma

to

the

interstitium.

6

Movement Dyskinesis associated with CRPS

may present as muscle spasm,

intention

or

pos-

tural

tremor, weakness, hyper-reflexive movement,

or the inability to initiate

movement. Motor

symp-

toms may precede the onset of

pain, may appear

contralaterally, and

are always associated with the

later two

stages of CRPS. Clinical evidence

sug-

gests

that

there

is:

1. No relationship between

tremors and

any

specific

symptom

of CRPS.

2 Frequency of tremors may be reduced by

weight

loading.

3

The tremor is postural and presents

with

voluntary

movement.

4 The tremor may be relieved by

sympathetic

blockage

or

remission

of CRPS.

6

Thermal responseand circulatory manifestations

Alterations

regarding autonomic and

peripheral

control

of

cutaneous blood flow are evident

in

cases

involving

CRPS. Vasoconstriction plays

a

role

in

thermoregulation

and is mediated by the

hypo-

thalamus. This process may be inhibited when

muscle receptors

(which fall under

medullary

con-

trol) are stimulated.

There

are indications that

fol-

lowing

local neural

insult,

skin receptors

may

fall

under

medullary

control.

It is suggested that

dur-

ing

the

second and

third

stages

of

CRPS,

observed

decreases in

skin

blood flow

may

be due to in-

creased microvascular sensitivity

to

circulating

cat-

echolamines.

Affected

extremities

exhibit de-

creased autonomic control of thermal

and

arterial

regulation

and

may initially

present with

skin that

is

warm,

red, and dry,

that during

later

stages

be-

comes

cool,

pale,

and

cyanotic.

4

,6,1

5,1

6

re tment

Early diagnosis

and

treatment

for

CRPS is

es-

sential for

establishing

a

cure.

The primary

treat-

ment for injuries

known to

be associated with

CRPS is

mobilization of

the affected

limb

to

rees-

tablish

normal function

and

active

range

of mo-

tion. This

may

be

accomplished

with adjunct phar-

macologic support

utilizing

anti-inflammatory and

analgesic agents.

In

latter

stages, physical

therapy

modalities,

such as passive

range of

motion, should

take

into consideration

the possibility of restimu-

lating or triggering the

symptoms

of CRPS.

4

*

Anesthetic

blockade

Local

anesthetic

block-

ade

(such

as cervical, lumbar

sympathetic blocks,

stellate

ganglion blocks, or

celiac

plexus blocks)

may prove

diagnostically useful

for differential

diagnosis

in

cases

where the presenting

symptoms

are masked or partially

evident.

Neural blockade

can discriminate

the

source

of pain whether

origi-

nating

from

the

central nervous

system or

the pe-

riphery.

This approach

may

help

to

confirm

or

deny the presence

of

nociceptive

stimulation

as

the

precursor to

pain, as well

as

diagram

the

spe-

cific

pain pathway. This

process

of mapping

the

pain

pathway

may provide

prognostic insight

into

potential

outcomes

using neurolytic

or surgical

de-

nervation

techniques.

13

Specific

information

that

will assist in deter-

mining treatment efficacy and/or disease

pathol-

ogy is

when and if pain

is

relieved.

Cessation

of

pain utilizing placebo

may

indicate psychogenic

origin. Pain relief

in

complement

with sympathetic

blockade may

substantiate

a

positive diagnosis

for

CRPS and

demonstrate

a need for

further

trials

using sympathetic

blockade.

Pain

relief

associated

with sensory blockade

may

indicate

a

source

that is

of

somatic

nociceptive

origin.

Pain

persisting de-

spite high spinal blockade implies that this

popu-

lation may

not

benefit from

continued

diagnostic

evaluation using

nerve block therapy.

A signifi-

cant subset within this group experience

psychotic

reactions

in

association

with

the

latter

modality.

4

3

7

Pharmacologic

The

benefits

associated

with

pharmacologic

intervention

for chronic

pain

should

include reduction

of

the patient's

percep-

tion of

pain, normalization

of

sleep patterns,

and

relief from

anxiety or depression.

Pharmacologic

intervention

may

include the

use of

tricyclics, anti-

depressants (inhibition

of presynaptic

uptake of

seratonin

and or

epinephrine),

antipsychotics

(blockade of dopamine

receptors),

hydroxyl

radi-

cal scavengers,

or anticonvulsants (multiple

sites

of

action).4,18

Psychological

Chronic

pain

invokes

an

emo-

tional

vocational

psychological

and

functional

loss

on the

individual.

Treatment

for

chronic

pain

requires

assessment

of

the

physical s

well

s

the

psychological

determinants

and ramifications that

chronic

pain

represents to

the

patient.

4 7

Research

on

patients suffering

from CRPS has

demonstrated

a significant

relationship

between

major stressful

events in

life (professional

and/or

personal loss)

and the

onset of

symptoms. The re-

lationship

between

the

autonomic

reflex and

emo-

tional status is

raised when

comparing psychologi-

cal

profiles of patients

suffering

from

CRPS. A

large

segment of

this patient

population

demon-

strates

emotional

lability, depression, manic

behav-

ior,

insecurity,

or

undue

anxiety.

9

December

1997/ Vol

65/No

6


4/4

Behavioral analysis

should be recognized as

an adjunct

modality

in the treatment

plan

for

pa-

tients who

have chronic pain when behavioral fac-

tors are believed

to cause pain. (It is

rarely effective

in cases of acute tissue damage.) This endeavor

serves to elicit and identify which factors increase

and/or

reinforce pain

behavior.

The

patient s per-

ception

of

pain

may

be

enabled

or

exacerbated

based

on

reinforcing

behaviors mirrored to the

pa-

tient

by the individuals around him.

Conditions

where

the

severity of pain seems

grossly disproportionate

to the assessed

tissue dam-

age

may indicate a need for detailed

inspection of

the relationship between specific daily

activities

(postural

changes, times

of

onset,

and threshold/

medication

administration)

and the level of pain

associated with them.

For example, in cases where

pain manifests

during

daytime activities while

abating at night,

nociceptive pain (usually

incom-

patible

with

this type

of

pattern)

may

be

ruled

out

and

environmental factors

(as opposed

to the

tis-

sue

injury)

may

be

indicated.

1

0,

13

* Other techniques. A

transcutaneous nerve

stimulator

is recommended for

the

treatment of

chronic

pain

associated

with peripheral

nerve in-

juries,

myofascial syndromes,

and stump

pain.

This

device is a battery powered, patient

operated

electrical stimulator

that emits a

low intensity tin-

gling or vibrating

sensation.

Theoretically, it is

believed

to

supplant

pain stimulus

by delivering

nonpain messages

to

the

spinal

cord or higher

neu-

ral

centers.

13

Numerous

types

of

neurosurgical approaches

to pain

relief for

CRPS patients,

spanning

from

peripheral spinal nerves

to invasive

cranial proce-

dures, have

been attempted

with

marginal

success.

Techniques such as dorsal

rhizotomy and percuta-

neous cordotomy produce

effective,

yet short-term

pain

management

and therefore

are

rarely

under-

taken.

Neural

ablation

proves

to be ineffective

over

time

and

should

be

considered

only for

patients

with a short

life expectancy,

such

as in the

case of

terminal

cancer.

1

3

Thermocoagulation

of the

gasserion ganglion,

cryo-neural techniques, deep

brain

stimulation,

and alcohol

injection into

the pituitary

gland

are

recently

developed techniques

that have specific

applications not

consistent

with

the treatment

of

chronic pain

associated

with

CRPS.

on lusion

Patients

suffering from CRPS

may be limited

in their ability to function in

a

self-directed, inde-

pendent

fashion.

A

longitudinal study

of

CRPS

on

1,348 patients

was

conducted by Greipp

and

6

Thomas,

and

revealed

that

96 of the study sub-

jects

still

suffer pain and

disability regardless

of

the duration

of the disease.

2

Diagnosis of CRPS

is

based

purely on

the physical examination and the

personal

history

of

the

patient,

regardless

of

whether or not symptomatic

relief can be demon-

strated using regional anesthesia, pharmacologic,

or behavioral techniques.

Although

the

primary

etiology for CRPS

is

not clearly understood,

key

progress

has

been in

made

in

terms of establishing

a

psychological

as well

as therapeutic treatment

plan once the diagnosis has been made.

REFERENCES

(1)

Swift

DW, Walker SE.

The clenched fist

syndrome. Arthritis

Rheum.

1994;38(1):57-60.

(2)

Greipp ME, Thomas AF. Reflex sympathetic dystrophy syndrome:

A

longitudinal

study.

Medical

Surgical Nursing.

1994;3(5):378-394.

(3)

Stanton-Hicks

M,

Janig W, Hassenbusch

S,

Haddox

JD,

Boad R,

Wilson P.

Reflex

sympathetic

dystrophy:

Changing

concepts

and

tax-

onomy.

Pain

1995;63:127-133.

(4)

Abram

SE,

Haddox

JD. Chronic pain

management. In: Barash

PG, Cullen BF,

Stoelting

RK, eds. ClinicalAnesthesia 2nd

ed.

Philadel-

phia: J.

B. Lippincott.

1992:1579-1607.

(5)

Weiss

AP.

Thermal

regulatory

testing

for

pain dysfunction

syn-

dromes. Lancet

1994;344:209-210.

(6) Schwartzman

RJ.

Reflex

sympathetic

sympathy and causalgia.

Neurol Clin.

1992;10(4):953-963.

(7) del

Carmen RS. Reflex

sympathetic dystrophy. Int

Anesthesiol Clin

1991;29:79-91.

(8) Galer

BS.

1994.

Reflex sympathetic

dystrophy

syndrome. Lancet

1994;344:691.

(9) Van Houdenholve

B, Vasquez G, Onghena

P,

et al. Etiopathogen-

esis

of

reflex

sympathetic dystrophy:

A

review of biopsychosocial

hypotheses.

Clini

Pain.

1992;8(4):300-306.

(10)

Veldman

HJM, Reyynen HM, Arntz IE, Goris

RJA. Signs and

symptoms

of

reflex

sympathetic

dystrophy: Prospective

study of 829

patients.

Lancet

1993;324:1012-1015.

(11)

Teasell

RW, Potter

P,

Moulin D. Reflex sympathetic dystrophy

involving three limbs.

A case study. Archives

of Physical and

Medical

Rehabilitation.1994;1008-1010.

(12)

Greipp

ME, Thomas

AF

Skin

lesions

occurring in

clients

with

reflex sympathetic dystrophy

syndrome. American

Association

of

Neuro

science

Nurses. 1994;26(6):342-346.

(13)

Murphy

TM.

Chronic pain. In: Miller

RD,

ed.

Anesthesia

3rd

ed.

New

York:

Churchill Livingstone.

1990:1927-1950.

(14) Gaeta

RR,

Brose

WG. Pain management. In: Morgan GE,

Mikhail

MS,

eds. Clinical Anesthesiology

East

Norwalk, Connecticut:

Appleton Lange. 1992:268-284.

(15)

Kurvers HAJM,

Jacobs

MJHM, Beuk

RJ, et al.

Reflex sympa-

thetic dystrophy: Evolution of microcirculatory disturbances in

time.

Pain.

1995;60:333-340.

(16) Low

PA,

Amadio PC,

Wilson

PR, McManis PG, Wilner CL. Lab-

oratory findings

in

reflex

sympathetic dystrophy:

A preliminary re -

port.

Clin

J Pain.1995;10(3):235-239.

(17) Nesathurai

S Harvey

DT,

Schatz

SW.

Gustatory

facial sweating

subsequent

to

upper thoracic sympathectomy.

rch Phys Med

Rehabil

1995;76:104-107.

(18)

Geertzen

JHB, de Bruijn H,

de

Bruijn-Kofman AT, Arendzen

JH. Reflex sympathetic

dystrophy: Early treatment and psychological

aspects. Arch Phys

Med

Rehabil.

1994;75:442-446.

AUTHOR

Ann E.

Aprile, CRNA,

MSN,

is staff nurse anesthetist and clinical

instructor

at William Beaumont Hospital,

in Royal Oak,

Michigan.

She

received

her MSN from the Oakland University-Beaumont

Gradu-

ate

Program

in

Nurse Anesthesia. Currently,

she

is

a

DNSc

student

at

Rush University Chicago

llinois

Journal

of the

American

Association of

NurseAnesthetists

jurnal crps

Documents