Concise guidance to good practice series

Complex regional pain

syndrome
Setting higher standards
December 2011
596 Royal College of Physicians, 2011. All rights reserved.
CONCISE GUIDANCE Clinical Medicine 2011, Vol 11, No 6: 596600
ABSTRACT Complex regional pain syndrome (CRPS) is a
debilitating, painful condition in a limb associated with sensory,
motor, autonomic, skin and bone abnormalities. Pain is typically
the leading symptom, but is often associated with limb
dysfunction and psychological distress. Prompt diagnosis and
early treatment is required to avoid secondary physical problems
related to disuse of the affected limb and the psychological
consequences of living with undiagnosed chronic pain. UK
guidelines have recently been developed for diagnosis and
management in the context of primary and secondary care.
1
The
purpose of this concise guideline is to draw attention to these
guidelines. Information in this article has been extracted from the
main document and adapted to inform the management of CRPS
as it presents to physicians in the course of their daily practice.
KEY WORDS: clinical guidelines, complex regional pain syndrome
Background
Complex regional pain syndrome (CRPS) is a debilitating con-
dition, characterised by pain in a limb, in association with sen-
sory, vasomotor, sudomotor, motor and dystrophic changes. It
commonly arises after injury to that limb. Pain is typically the
leading symptom of CRPS, but is often associated with limb dys-
function and psychological distress. Patients frequently report
neglect-like symptoms or a feeling that the limb is alien.
2
CRPS can be divided into two types based on the absence (type 1,
much more common) or presence (type 2) of a lesion to a major
nerve. The subtype of CRPS has no consequences for the general
approach to management, but the cause of nerve damage in CRPS 2
should always be clarified urgently in acute cases (see guideline 1.2).
The diagnosis of CRPS cannot be made on imaging or labora-
tory tests. The condition is diagnosed on the basis of clinical cri-
teria
3
shown in Box 1. Differential diagnoses are listed in Box 2.
Aetiology and course
The cause of CRPS is currently unknown.

Precipitating factors include injury and surgery. However,

there is no relationship to the severity of trauma while in
some cases there is no precipitating trauma at all (9%).
4
The
development of CRPS does not mean that surgery was
suboptimal.
5

Transient features of CRPS are much more common than

most clinicians realise occurring in up to 25% of minor
limb injuries.
6

Approximately 15% of sufferers will have unrelenting pain

and physical impairment >5 years after CRPS onset,
although more patients will have a lesser degree of ongoing
pain and dysfunction
7
impacting on their ability to work
and function normally.

There is no medical cure for CRPS.

It is also now clear that CRPS is not significantly associated with a
history of pain-preceding psychological problems, or with somati-
sation or malingering. Patients still report feeling stigmatised by
health professionals who do not believe that their condition is real.
However, patients may require psychological intervention and
support to deal with the particularly distressing nature of CRPS
Lynne Turner-Stokes, Herbert Dunhill Chair of rehabilitation, King's
College London School of Medicine and director, Regional Rehabilitation
Unit, Northwick Park Hospital; Andreas Goebel, consultant and senior
lecturer in pain medicine, Walton Centre NHS Foundation Trust and
Liverpool University
On behalf of the guideline development group (GDG). Please refer
to supplementary electronic documentation for full list of GDG
members. The full guideline will be available from the Royal College
of Physicians website (www.rcplondon.ac.uk) in 2012.
Series editors: Lynne Turner-Stokes and Bernard Higgens
Complex regional pain syndrome in adults: concise guidance
Lynne Turner-Stokes and Andreas Goebel on behalf of the guideline development group
8ox 1. 8udapest d|agnost|c cr|ter|a. Adapted from reference 3.
All of Lhe followlng sLaLemenLs musL be meL:
- 1he paLlenL has conLlnulng paln whlch ls dlsproporLlonaLe Lo any
lnclLlng evenL
- 1he paLlenL has aL leasL one slgn ln Lwo or more of Lhe caLegorles
below
- 1he paLlenL reporLs aL leasL one sympLom ln Lhree or more of Lhe
caLegorles below
- no oLher dlagnosls can beLLer explaln Lhe slgns and sympLoms.
Category S|gn]symptom
1 'Sensory' Allodynla (paln Lo llghL Louch and/or
LemperaLure sensaLlon and/or
ueep somaLlc pressure and/or [olnL
movemenL) and/or
Pyperalgesla (Lo plnprlck).
2 'vasomoLor' 1emperaLure asymmeLry and/or
Skln colour changes and/or
Skln colour asymmeLry.
3 'SudomoLor/oedema' Cedema and/or
SweaLlng changes
and/or SweaLlng asymmeLry.
4 'MoLor/Lrophlc' uecreased range of moLlon and/or
MoLor uysfuncLlon (weakness, Lremor,
dysLonla) and/or
1rophlc changes (halr/nall/skln).
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Complex regional pain syndrome in adults: concise guidance
Royal College of Physicians, 2011. All rights reserved. 597
symptoms. Psychosocial risk factors that may predict chronicity
8
are shown in Box 3.
Treatment approach

Prompt diagnosis and early treatment is required to avoid

secondary physical problems associated with disuse of the
affected limb and the psychological consequences of living
with undiagnosed chronic pain.
10

Early referral to physiotherapy and encouraging gentle

movement as early as possible, may potentially prevent pro-
gression of symptoms.
11

Except in mild cases, patients with CRPS are generally best

managed in specialist pain management or rehabilitation
programmes.

An integrated interdisciplinary treatment approach is

required, including the four pillars of intervention (see
Box 4).
This guidance
UK guidelines have recently been developed for diagnosis and
management in the context of primary and secondary care.
1
Information in this concise guidance document has been
extracted from the main document and adapted to inform the
management of CRPS as it presents to physicians in the course
of their daily practice.
The guidelines were drawn up in accordance with the princi-
ples recommended by the AGREE Collaboration.
12
The
methodology table is available online, and a full description of
the methodology and systematic literature review may be found
in the main guideline document.
11
The original recommenda-
tions were developed on the basis of panel consensus and expert
opinion with reference to the existing literature. Where possible,
recommendations were informed by evidence from the reviews
of RCTs. However, in the absence of RCT-based evidence to
inform specific guidance with respect to diagnosis and manage-
ment, the majority of recommendations in this document are
based on the expert opinion of service users (E1) and profes-
sionals (E2) (according to the classification used by the National
Service Framework for Long Term Conditions).
13
Implications for implementation
The implications for implementation primarily relate to
training requirements for clinical staff to recognise CRPS and
make the appropriate referrals for specialist management.
Currently, access to specialist pain management and rehabili-
tation for CRPS is patchy across the UK. For the subgroup of
patients with complex needs, there is an additional requirement
for closer liaison between these services to share their expertise
and resources, as well as ongoing facilities (including self-help
and support groups) to assist patients to manage their own
symptoms and optimise their level of physical psychological and
social function.
References
1 Goebel A, Barker CH, Turner-Stokes L et al. Complex regional pain
syndrome in adults: UK guidelines for diagnosis, referral and manage-
ment in primary and secondary care. London: Royal College of
Physicians (in press).
2 Frettlh J, Hppe M, Maier C. Severity and specificity of neglect-like
symptoms in patients with complex regional pain syndrome (CRPS)
compared to chronic limb pain of other origins. Pain 2006;124:
1849.
8ox 3. Ident|f|ed psychosoc|a| r|sk factors (ye||ow f|ags) wh|ch may
pred|ct chron|c|ty. AdapLed from reference 9.
e||ow f|ags
- laLrogenlc facLors, le prevlous negaLlve experlences wlLh healLh
professlonals.
- Lxcesslve lllness behavlour.
- oor coplng sLraLegles, eg ongolng 'guardlng ' of Lhe llmb desplLe
educaLlon.
- lnvolvemenL ln llLlgaLlon ls affecLlng wllllngness Lo progress ln
LreaLmenL.
- Cveruse of appllances.
- ulsLress.
- AnxleLy/depresslon.
- Lack of wllllngness Lo seL goals.
- asslve ln LreaLmenL sesslons.
- lnapproprlaLe bellefs desplLe educaLlon.
- negaLlve famlly lnfluences.
8ox 2. D|fferent|a| d|agnoses for comp|ex reg|ona| pa|n syndrome
(CkS).
More common cond|t|ons:
- bony or sofL Llssue ln[ury (lncludlng sLress fracLure, llgamenL
damage, lnsLablllLy)
- neuropaLhlc paln (eg due Lo perlpheral nerve damage or cenLral
nervous sysLem/splnal leslon)
- arLhrlLls/arLhrosls
- lnfecLlon (bony, [olnL, sofL Llssue, skln)
- comparLmenL syndrome
- arLerlal lnsufflclency (usually due Lo aLherosclerosls ln Lhe elderly,
Lrauma or LhromangllLls obllLerans (8urger's dlsease))
- 8aynaud's dlsease
- lymphaLlc or venous obsLrucLlon
- Lhoraclc ouLleL syndrome (due Lo elLher nerve compresslon or
vascular compresslon).
kare cond|t|ons:
- Cardner-ulamond syndrome*
- eryLhromelalgla (may lnclude all llmbs)
- self-harm/mallngerlng.
*lsycboqeolc potpoto (CotJoet-ulomooJ syoJtome, ootoetytbtocyte
seosltlzotloo, polofol btolsloq syoJtome) ls o tote ooJ pootly
ooJetstooJ cllolcol pteseototloo of ooexploloeJ polofol eccbymotlc
lesloos, mostly oo tbe exttemltles ooJ/ot tbe foce.
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Lynne Turner-Stokes and Andreas Goebel
598 Royal College of Physicians, 2011. All rights reserved.
3 Harden RN, Bruehl S, Stanton-Hicks M et al. Proposed new diagnostic
criteria for complex regional pain syndrome. Pain Med 2007;8:32631.
4 Veldman PJHM, Reynen HM, Arntz IE et al. Signs and symptoms of
reflex sympathetic dystrophy: prospective study of 829 patients. Lancet
1993;342:10126.
5 Atkins RM. Principles of complex regional pain syndrome. In: Bucholz W,
Heckman JD, Court-Brown CN et al (eds), Rockwood and Greens fractures
in adults, 7th edn. London: Lippincott Williams & Wilkins, 2010:60215.
6 Atkins RM, Duckworth T, Kanis JA. Features of algodystrophy after
Colles fracture. J Bone Joint Surg 1990;72:10510.
7 Schasfoort FC, Bussmann JB, Stam HJ. Impairments and activity
limitations in subjects with chronic upper-limb complex regional
pain syndrome type I. Arch Phys Med Rehabil 2004;85:55766.
8 Geertzen J, Van Wilgen C. Chronic pain in rehabilitation medicine.
Disabil Rehabil 2006;28:3637.
9 Main CJ, Williams, ACC. Clinical review. ABC of psychological medi-
cine: musculoskeletal pain. BMJ 2002;325:5347.
10 Harden RN, Swan M, King A et al. Treatment of complex regional
pain syndrome: functional restoration. Clin J Pain 2006;22:
4204.
11 Oerlemans HM, Oostendorp RAB, de Boo T et al. Pain and reduced
mobility in complex regional pain syndrome I: outcome of a prospec-
tive randomised controlled clinical trial of adjuvant physical therapy
versus occupational therapy. Pain 1999;83:7783.
12 Guideline development in Europe: an international comparison.
J Technol Assess Health Care 2000;16:103646.
8ox 4. Iour p|||ars of treatment for comp|ex reg|ona| pa|n syndrome (CkS) - an |ntegrated |nterd|sc|p||nary approach.
1 at|ent |nformat|on and educat|on
aLlenLs should be provlded wlLh approprlaLe educaLlon abouL C8S Lo supporL self-managemenL:
- a sample paLlenL lnformaLlon sheeL ls avallable (see full guldellnes).
aLlenLs should be:
- reassured LhaL physlcal and occupaLlonal Lherapy are safe and approprlaLe
- engaged ln Lhe process of goal-seLLlng and revlew.
2 a|n re||ef (med|cat|on and procedures)
aln speclallsLs should be aware of Lhe evldence for efflcacy of paln lnLervenLlons ln Lhe conLexL of C8S (see full guldellne for sysLemaLlc revlew).
no lndlvldual drugs or paln lnLervenLlon procedures can be recommended aL Lhe currenL Llme due Lo lack of evldence (see full guldellne for
sysLemaLlc revlew).
1he followlng may be consldered:
- neuropaLhlc paln medlcaLlon dellvered accordlng Lo naLlonal lnsLlLuLe for PealLh and Cllnlcal Lxcellence guldellnes
14
- pamldronaLe (60 mg lnLravenous slngle dose) for sulLable paLlenLs wlLh C8S of 6 monLhs duraLlon as a one-off LreaLmenL
- splnal cord sLlmulaLor LreaLmenL for paLlenLs wlLh C8S who have noL responded Lo approprlaLe lnLegraLed managemenL.
3 hys|ca| and vocat|ona| rehab|||tat|on
hyslcal rehablllLaLlon should be dellvered by LheraplsLs compeLenL ln LreaLlng paLlenLs wlLh chronlc paln and/or C8S.
Lmphasls should be on resLoraLlon of normal funcLlon and acLlvlLles Lhrough acqulslLlon of self-managemenL skllls, wlLh paLlenLs and Lhelr
famllles acLlvely engaged ln goal seLLlng.
1he programme may lnclude elemenLs of chronlc paln managemenL lncludlng:
- general body re-condlLlonlng Lhrough graded exerclse, galL re-educaLlon, posLural conLrol
- resLoraLlon of normal acLlvlLles, lncludlng self-care, recreaLlonal physlcal exerclse and soclal and lelsure acLlvlLles
- paclng and relaxaLlon sLraLegles, Lo supporL self-managemenL of Lhe condlLlon
- vocaLlonal supporL.
lL may also lnclude speclallsed Lechnlques Lo address alLered percepLlon and awareness of Lhe llmb, for example:
- self-admlnlsLered desenslLlsaLlon wlLh LacLlle and Lhermal sLlmull
- funcLlonal movemenL Lo lmprove moLor conLrol and llmb poslLlon awareness
- graded moLor lmagery, mlrror vlsual feedback, menLal vlsuallsaLlon
- managemenL of C8S-relaLed dysLonla.
4 sycho|og|ca| |ntervent|on
sychologlcal lnLervenLlon ls based on lndlvlduallsed assessmenL, Lo ldenLlfy and proacLlvely manage any facLors whlch may perpeLuaLe paln
or dlsablllLy/dependency lncludlng:
- mood evaluaLlon - managemenL of anxleLy/depresslon
- lnLernal facLors, eg counLer-producLlve behavlour paLLerns
- any exLernal lnfluences or perverse lncenLlves.
lL usually follows prlnclples of cognlLlve behavloural Lherapy dellverlng:
- coplng skllls and poslLlve LhoughL paLLerns
- supporL for famlly/carers Lo manage Lhelr own needs and Lo malnLaln relaLlonshlps.
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Complex regional pain syndrome in adults: concise guidance
Royal College of Physicians, 2011. All rights reserved. 599
1 D|agnos|s and exc|us|on of remed|ab|e cond|t|ons Grade
1.1 Comp|ex reg|ona| pa|n syndrome (CkS) should be consldered ln Lhe presence of pers|stent or d|sproport|onate ||mb pa|n, L2
parLlcularly ln Lhe conLexL of Lrauma or surgery.
All cllnlclans should be:
- aware of Lhe condlLlon
- famlllar wlLh Lhe 8udapest cr|ter|a for dlagnosls (see 8ox 1).
1.2 Lxc|us|on of revers|b|e causes L2
Where d|sproport|onate pa|n pers|sts for |onger than expected:
1 dlfferenLlal dlagnoses should be consldered and lnvesLlgaLed as a maLLer of urgency, Lo exc|ude remed|ab|e cond|t|ons
(see 8ox 2)
2 where paln occurs ln Lhe dlsLrlbuLlon of a perlpheral nerve followlng a surglcal procedure, Lhe paLlenL should be referred
back Lo Lhe responslble surgeon for urgenL re-evaluaLlon and posslble surglcal exploraLlon.
1.3. at|ent |nformat|on
- Ceneral posL-fracLure/operaLlon pat|ent |nformat|on |eaf|ets should lnclude advlce Lo observe and reporL CkS warn|ng s|gns. L1]2
- Management shou|d |nc|ude reassurance LhaL:
o C8S ls a recognlsed condlLlon alLhough lLs causes are poorly undersLood
o lLs developmenL should noL be consldered evldence of subopLlmal surglcal managemenL
o sympLoms are LranslenL ln Lhe ma[orlLy of cases, and usually respond well Lo desenslLlsaLlon (see 2.3), movemenL
and resumpLlon of normal acLlvlLles.
2 Lar|y management of CkS - ana|ges|a and exerc|se
2.1 Medlcal managemenL should lnclude: L2
- regu|ar rev|ew, wlLh advlce abouL Lhe use of slmple analgeslcs, eg paraceLamol, co-codamol (parLlcularly for Lrauma-relaLed
paln)
- non-stero|da| ant|-|nf|ammatory drugs (ln Lhe presence of bony or sofL Llssue Lrauma, and ln Lhe absence of conLralndlcaLlons).
lf slmple medlcaLlon does noL reduce paln Lo a mlld level afLer 3-4 weeks, conslder uslng med|cat|on for neuropath|c pa|n accordlng
Lo Lhe naLlonal lnsLlLuLe for PealLh and Cllnlcal Lxcellence guldellnes for neuropaLhlc paln.
14
Larller use may be approplaLe.
2.2 1he followlng LreaLmenLs are not genera||y recommended and should be used wlLh cauLlon by approprlaLely experlenced cllnlclans: L2
- op|ate ana|ges|a - excepL wlLh speclflc advlce from a speclallsL paln cllnlc
- |ntravenous reg|ona| sympathet|c b|ocks - Lhese have no proven efflcacy ln prospecLlvely randomlsed sLudles
- amputat|on - Lhls does noL provlde paln rellef ln C8S buL may be necessary for llfe LhreaLenlng sympLoms, such as lnfecLlon.
1he paLlenL should be warned LhaL Lhe C8S may recur ln Lhe sLump and Lhe paln may be worsened
- surgery on a CkS-affected ||mb - surgery represenLs a palnful sLlmulus and may worsen C8S. lL should be avolded where
posslble. Where surgery ls unavoldable, lL should be carrled ouL by a surglcal Leam experlenced ln Lhls area and an anesLheLlsL
wlLh experlence ln Lhe managemenL of paLlenLs wlLh chronlc paln.
2.3 Ana|ges|a shou|d not be g|ven |n |so|at|on, buL should be prescrlbed wlLh Lhe alm of supporLlng an exerc|se]therapy programme. k8]L2
lf tbete ls ooy Joobt oboot tbe sofety of movemeot, tbe oJvlce of oo ottbopoeJlc sotqeoo ot tbeomotoloqlst sboolJ be sooqbt.
2.4 unless conLralndlcaLed, phys|otherapy and]or occupat|ona| therapy should be lnlLlaLed |mmed|ate|y when CkS |s suspected. k8]L2
- Larly physlcal Lherapy should lnclude:
o encouragemenL Lo look aL Lhe affecLed llmb
o gent|e movement and desens|t|sat|on (eg genLle sLroklng wlLh LexLured fabrlcs)
o early lncorporaLlon lnLo funct|ona| act|v|ty and welghL-bearlng when LoleraLed.
- Sllngs, spllnLs and oLher |mmob|||s|ng dev|ces shou|d be avo|ded wherever posslble
- CrLhoLlc devlces (such as lnsoles) can supporL welghL bearlng and funcLlon, buL should only be provlded under a physloLheraplsL's
supervlslon.
3 Mon|tor|ng and esca|at|ng referra|
3.1 aLlenL should be kepL under regu|ar mon|tor|ng Lo: L2
- assess Lhe effecLlveness of LreaLmenL and paln conLrol
- assess and monlLor mood, ln parLlcular for slgns of developlng dlsLress and/or evldence of depresslon/anxleLy.
1he gu|de||nes
CMJ1106-Dec_CG.qxd 11/23/11 5:33 PM Page 599
Lynne Turner-Stokes and Andreas Goebel
600 Royal College of Physicians, 2011. All rights reserved.
13 Turner-Stokes L, Harding R, Sergeant J et al. Generating the evidence
base for the National Service Framework (NSF) for long term condi-
tions: a new research typology. Clin Med 2006;6:917.
14 National Institute for Health and Clinical Excellence. CG96
Neuropathic pain: the pharmacological management of neuropathic
pain in adults in non-specialist settings. London: NICE, 2010.
www.nice.org.uk/CG96
Address for correspondence: Dr A Goebel,
Pain Research Institute, Department of Translational Medicine,
Liverpool University, Liverpool L9 7AL.
Email: andreasgoebel@rocketmail.com
ln Lhe presence of pers|st|ng or worsen|ng pa|n:
- t|me|y onward referra| should be made Lo spec|a||st pa|n and]or rehab|||tat|on servlces (see below)
- escalaLlon should be proporLlonaLe Lo Lhe severlLy/lmpacL of paln sympLoms
- Lhe presence of cerLaln psychosoc|a| r|sk factors |dent|f|ed as 'ye||ow f|ags' (see 8ox 3) may predlcL chronlclLy
7
and should
prompt ear|y referra| Lo mulLldlsclpllnary servlces wlLh access Lo psychologlcal lnLervenLlons.
4 Spec|a||st pa|n management and rehab|||tat|on
4.1 aLlenLs wlLh moderaLe or severe perslsLlng C8S should have t|me|y access to spec|a||st pa|n c||n|cs able Lo offer an |ntegrated
mu|t|d|sc|p||nary approach to treatment (Lhe four plllars of lnLervenLlon, see 8ox 4 for deLalls).
4.2 SpeclallsL paln managemenL servlces and rehablllLaLlon Leams should work LogeLher Lo share the|r expert|se and resources for
paLlenLs wlLh severe complex presenLaLlons of C8S.
S at|ents w|th comp|ex d|sab||ng CkS
3.1 aLlenLs wlLh comp|ex d|sab||ng CkS should have access Lo speclallsL lnLerdlsclpllnary rehab|||tat|on programmes.
3.2 Speclallsed rehablllLaLlon programmes led/supporLed by a consulLanL ln rehablllLaLlon, may be requlred ln Lhe followlng
slLuaLlons:
- C8S presenLlng ln Lhe conLexL of:
o anoLher exlsLlng dlsabllng condlLlon (eg sLroke, severe mulLlple Lrauma)
o complex psychologlcal or psychlaLrlc co-morbldlLles - elLher pre-or posL-daLlng Lhe onseL of C8S
- severe physlcal dlsablllLy/dependency requlrlng LreaLmenL ln an lnpaLlenL seLLlng
- speclallsL faclllLles, equlpmenL or adapLaLlons are requlred, or need revlew
- unable Lo work - requlrlng speclallsL vocaLlonal rehablllLaLlon/supporL
o eg supporLed work schemes, employer llalson for [ob modlflcaLlon
o or supporL for work wlLhdrawal where approprlaLe.
- llLlgaLlon ls ongolng - requlrlng supporL Lo faclllLaLe an early concluslon.
6 Long-term ongo|ng support
6.1 eople wlLh C8S should have access Lo appropr|ate |nformat|on, adv|ce, educat|on and support Lo allow Lhem Lo undersLand
and manage Lhelr condlLlon opLlmally.
6.2 eople wlLh C8S should have access Lo a range of faclllLles Lo malnLaln Lhelr lndependence, and levels of acLlvlLy and socleLal
parLlclpaLlon, whlch may lnclude:
- se|f-he|p and peer support groups
- faclllLles for se|f-d|rected exerc|se (eg adapLed gym, swlmmlng/hydroLherapy pool)
- supporL for vocat|ona|, soc|a| and |e|sure acLlvlLles
- counse|||ng]psycho|og|ca| support where |nd|cated.
1hese servlces are ofLen approprlaLely run by volunLary organlsaLlons wlLh lnpuL from professlonals as requlred.
6.3 Iam|||es and carers of people wlLh C8S should have access Lo adv|ce, support and |nformat|on Lo manage Lhelr own needs
and Lo ma|nta|n re|at|onsh|ps.
1he gu|de||nes ()
kC]L2
L2
L2
L2
L1]2
L1]2
L1]2
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