the challenges of early diagnosis - muskuloskeletal · the challenges of early diagnosis ... n.b...

DrSueGreenhalghConsultantPhysiotherapistRoyalBoltonNHSFoundationTrustSusan.greenhalgh@boltonft.nhs.uk

TheChallengesofEarlyDiagnosis

VulnerableAnatomy;Asurgicalemergency

CaudaEquinafirstdescribedin1600CEprovidesinnervationtolowerlimbs,sphincter,sensoryinnervationtosaddleandparasympatheticinnervationtobladderanddistalbowel.

•  no Schwann cell cover •  hypovascularity

CaudaEquinaSyndrome(CES)

Syndromenotdescribeduntil1934byMixterandBarrIn2017weareStillimprovingourapproachtoCES

Anorthopaedic

surgicalemergency

CaudaEquinaSyndrome;Asurgicalemergency

¡  Rareanddisabling

‘LifeChanging’

1/5patientswillhavepooroutcome;¡  on-goingtreatmentforsexualdysfunction

¡  selfcatheterisation

¡  colostomy

¡  psycho-social

ClinicalDiagnosis

¡  Until recently no broadly accepted definitive diagnostic criteria; 17 different definitions of CES recorded (Fraser et al, 2009)

¡  Initial signs and symptoms are often subtle and vague, varying in intensity and evolution (Bin et al, 2009)

BritishAssociationofSpinalSurgeons(BASS)definitioninStandardsofCare,(Germonetal,2015)

Apatientpresentingwithacute(de-novoorasanexacerbationofpre-existingsymptoms)backpainand/orlegpainWITHasuggestionofadisturbanceoftheirbladderorbowelfunctionand/orsaddlesensorydisturbanceshouldbesuspectedofhavingordevelopingacaudaequinasyndrome.MostofthesepatientswillnothavecriticalcompressionHowever,intheabsenceofreliablypredictivesymptomsandsigns,thereshouldbealowthresholdforinvestigationwithanEMERGENCYMRIscan.Thereasonsfornotrequestingascanshouldbeclearlydocumented.Subjectivehistorykeytoearlydiagnosis

CaudaEquinaSyndromeGroups(Todd&Dickson,2016)

CESSsuspected

Bilateralradicularpain(progressingunilateral)

CESIincomplete

Urinarydifficultiesofneurogenicorigin,alteredurinarysensation,lossofdesiretovoid,poorurinarystream,needtostraintomicturate

CESRreten,on

Painlessurinaryretentionandoverflowincontinence

CESCcomplete

ObjectivelossofCEfunction,absentperinealsensation,patulousanus,paralysedinsensatebladderandbowel

‘TheprobabilityofaCESpatientdeteriorating,withwhatspeedandtowhatlevelisnotpredictable

TheDiagnosticChallenge

Significantly more patients are referred on for further investigation compared with those having a radiologically confirmed diagnosis of CES Bladder and bowel dysfunction, saddle anaesthesia and sexual dysfunction are all multifactorial in their causes e.g. Comorbidities, medication, pain (Woodsetal,2015)

CESMasqueraders;EpiduralCompressionSyndrome

(Stopleretal,2016)

¡  Compressionofspinalcord,conusmedullarisorcaudaequina

¡  Thoraciclesionscanmasqueradeascaudaequinasyndrome

¡ Masqueradersincludethoracicdisc,thoracicmeningioma,ligamentossificationandthoraciccordtumour

¡  Rare;diseasevresources

AbdominalAorticAneurysm(Engambaetal,2017)

¡ Middle-agedandelderlypatientswithlowerlimbpainand/orneuropathy,inthecontextofahistoryofabdominalaorticaneurysm(AAA).

¡  Haematomacausingmasseffectonneuralstructures

Bladder&Bowel,SexualDysfunctionRedHerrings

¡  Opioid Salts; constipation (e.g. Tramadol, Codeine) ¡  Anticonvulsants; urinary incontinence (e.g. Gabapentin, Pregabalin) ¡  Antidepressants; retention, sexual dysfunction (e.g. Amitriptyline, Nortriptyline)

PharmacologycausesofSexualDysfunction

Class Drug

Hypnotics Benzodiazepines

Antihypertensive Beta blockers

Antidepressants Tricyclic antidepressants; Selective serotonin reuptake inhibitors e.g fluoxetine; Monoamine oxidase inhibitors; Viloxazine and L-tryptophan; Nefazodone; Vanlafaxine; Reboxetine; Mirtazepine; Trazodone; Duloxetine

Diuretics Bendroflurazide,

Anti-epileptics

Carbamazepine; Phenytoin; Sodium valproate

Antipsychotics Thioridazine; aliphatic phenothiazines e.g chlorpromazine, sulprides atypical antipsychotic risperidone

Prostate medications Finasteride (BPH); Anti androgens e.g. cyproterone acetate, flutamide (Prostate Cancer); Gonadotrophin releasing hormone analogues e.g goserelin, leuprorelin (Prostate Cancer)

Anti-parkinsonian drugs

L-dopa

Recreational drugs Psychstimulants, Amphetamine, Ecstacy, Crystal methamphetamine, Alcohol, Anabolic steroids, cannabis, Opiates (Heroin, Methadone, Buprenorphine), Poppers, Tobacco

Pharmacologicalagentsassociatedwithurinaryretention

Class Drugs

Antiarrhythmics Disopyramide, procainamide, quinidine

Anticholinergics Atropine (Atreza), belladonna alkaloids, dicyclomine (Bentyl), flavoxate (Urispas), glycopyrrolate (Robinul), hyoscyamine (Levsin), oxybutynin (Ditropan), propantheline (Pro-Banthine), scopolamine (Transderm Scop)

Antidepressants Amitriptyline, amoxapine, doxepin, imipramine, maprotiline, nortriptyline

Antihistamines (selected) Brompheniramine, chlorpheniramine, cyproheptadine, diphenhydramine, hydroxyzine

Antihypertensives Hydralazine, nifedipine

Antiparkinsonian agents Amantadine, Benztropine, bromocriptine, levodopa, trihexyphenidyl

Antipsychotics Chlorpromazine, fluphenazine, haloperidol, prochlorperazine, thioridazine

Hormonal agents Estrogen, progesterone, testosterone

Muscle relaxants Baclofen, cyclobenzaprine, diazepam

Sympathomimetics (alpha-adrenergic agents)

Ephedrine, phenylephrine (Neo-Synephrine); phenylpropanolamine. pseudoephedrine (Sudafed)

Sympathomimetics (beta-adrenergic agents)

Isoproterenol (Isuprel); metaproterenol (Alupent); terbutaline (Brethine)

Miscellaneous Amphetamines, carbamazepine, dopamine, mercurial diuretics, nonsteroidal anti-inflammatory drugs

1. Selius, B. and Subedi, R. (2008). Urinary Retention in Adults: Diagnosis and

Management. American Family Physician, 77 (5), 643-650.

CausesofUrinaryRetention

Cause Men Women BothObstructive Benign prostatic hyperplasia,

meatal stenosis, paraphimosis, penile constricting bands, phimosis, prostate cancer

Organ prolapse (cystocele, rectocele, uterine prolapse); pelvis mass (gynaecological malignancy, uterine fibroid, ovarian cyst); retroverted impacted gravid uterus

Aneurysmal dilation; bladder calculi; bladder neoplasm; faecal impaction; gastrointestinal or retroperitoneal malignancy/mass; urethral strictures, foreign bodies, stones, edema

Infectious or inflammatory

Prostatic abscess, prostatis Acute vulvovaginitis; vaginal lichen planus; vaginal lichen sclerosis; vaginal pemphigus

Bilharziasis; cystitis;echinococcosis; Guillain-Barre syndrome; herpes simplex virus; Lyme disease; periurethral abscess; transverse myelitis; tubercular cystitis; urethritis ; varicella zoster virus

Other Penile trauma, fracture, laceration

Postpartum complication; urethral sphincter dysfunction (Fowler’s syndrome)

Disruption of posterior urethra and bladder neck in pelvic trauma; postoperative complication; psychogenic

1. Selius, B. and Subedi, R. (2008). Urinary Retention in Adults: Diagnosis and

Management. American Family Physician, 77 (5), 643-650.

NeurologicalCausesofUrinaryRetention

Lesion type Causes

Autonomic or peripheral nervous system

Autonomic neuropathy; diabetes mellitus; Guillian-Barre Syndrome, herpes zoster virus; Lyme disease; pernicious anaemia; poliomyelitis; radical pelvis surgery; spinal cord trauma; tabes dorsalis

Brain Cerebrovascular disease; concussion; multiple sclerosis; neoplasm or tumour; normal pressure hydrocephalus; Parkinson’s disease, Shy-Drager Syndrome

Spinal cord Dysraphic lesions; invertebral disc disease; meningomyelocele; multiple sclerosis; spina bifida occulta; spinal cord hematoma or abscess; spinal cord trauma; spinal stenosis; spinovascular disease; transverse myelitis tumours or masses of conus medullaris or cauda equina

1. Selius, B. and Subedi, R. (2008). Urinary Retention in Adults: Diagnosis and

Management. American Family Physician, 77 (5), 643-650.

NeurologicalCausesofUrinaryRetention

Lesion type Causes

Autonomic or peripheral nervous system

Autonomic neuropathy; diabetes mellitus; Guillian-Barre Syndrome, herpes zoster virus; Lyme disease; pernicious anaemia; poliomyelitis; radical pelvis surgery; spinal cord trauma; tabes dorsalis

Brain Cerebrovascular disease; concussion; multiple sclerosis; neoplasm or tumour; normal pressure hydrocephalus; Parkinson’s disease, Shy-Drager Syndrome

Spinal cord Dysraphic lesions; invertebral disc disease; meningomyelocele; multiple sclerosis; spina bifida occulta; spinal cord hematoma or abscess; spinal cord trauma; spinal stenosis; spinovascular disease; transverse myelitis tumours or masses of conus medullaris or cauda equina

1. Selius, B. and Subedi, R. (2008). Urinary Retention in Adults: Diagnosis and

Management. American Family Physician, 77 (5), 643-650.

NEVERASSUME

ResultsofEmergencylumbarMRI-Thenwhat?(Germonetal,2015)

▪  Caudaequinacompressionconfirmed;urgentsurgicalreferral

▪  Caudaequinacompressionexcludedbutapotentialstructuralexplanationofpainidentified;referraltotheappropriatesurgicalservice.

▪  Non-compressivepathologymaybeidentified(forexample,demyelination);referraltotheappropriateservice.

▪  Noexplanationofthepatient’ssymptoms;probablyappropriatetoreferbacktotheGP

……orMRIhigher?….orcouldtherebealesionintheabdomenorpelviccavity

SurgicalIntervention

¡  “AllCESpatientsshouldhaveemergencyimagingandtreatmentassoonaspracticallypossibletomaximisegoodoutcomes”(Todd,2015)

§  “Nothingistobegainedbydelayingsurgeryandpotentiallymuchtobelost”BritishAssociationofSpinalSurgeonsstandardsofcareforcaudaequinasyndrome(2015)

Surgery

¡  Aimofsurgeryistopreservefunctionpresentatthetimeofsurgery.

¡  Thereisscopeforimprovement,butsmallriskofdeterioratione.g.paralysis,lossofbladderandbowelcontrol,impotence/sexualdysfunction.

¡  Bladderfunctionattimeofsurgeryimportant

WorkingwithPatientsasPartners

¡  ‘thenumberone‘spinal’patientsafetyissue’….averagecompensationfollowingdelayeddiagnosis-£336,000intheUnitedKingdom(Fairbank,2014)

¡  WhatisthepatientexperienceofonsetofCESsymptoms?

Copyright

Whatcanwelearnfromourpatients?

DermatomesS3,4,5

©Copyright2016BoltonNHSFoundationTrust.Nottobecopiedwithoutpermissionofthecopyrightowner,allrightsreserved.

Copyright

©Copyright2016BoltonNHSFoundationTrust.Nottobecopiedwithoutpermissionofthecopyrightowner,allrightsreserved.

DermatomesS3,4,5

AQualitativeInvestigationintoPatientsExperienceofCaudaEquinaSyndromeGreenhalghS,TrumanC,WebsterV,SelfeJ(2015)PhysiotherapyResearchFoundation(PRF)Grant

AimToidentifyhowCESsymptomsmaybeeffectivelysharedbetweenpatientsandclinicianObjectivesDrawinguponpatientexperienceofsignsandsymptomsassociatedwithCESincludingchangesinbladder,bowelandsexualfunction§  whatsymptomspatientsactuallysuffer§  patientsownreasoningofthesesymptoms§  thepatientexperienceofdivulgingthis

information

7themesemerged

§  CatastrophicPain

§  ImpactonLife

§  CommonSymptoms/VaryingChronology

§  Senseofchange/Seriousness

§  ContactwithHealthProfessionals

§  CarersExperience

§  Suggestionstoaidearlydiagnosis

CatastrophicPain

CatastrophicPain

¡  ‘….ThewomanwhowasdoingtheMRIsaidohgosh.Iwasallscreamingandhyperventilatingandshesaidareyouok,areyouclaustrophobic?IsaidI’minbloodyagony-Strong

pain,paininwholepelvis,realagony’

¡  ‘Idon’tthinkhisquestionsweren’tclear,Ithinkthatitwasimpossibletoconcentrateonanythingotherthanpain

management’.

CommonSymptoms/VaryingChronology

‘……Itwaslikeyoucouldnottellwhereyourfeetwereinspace’‘Iwassortoflosingcontrol...mylegsweren’tworkingproperlyliketheyweremadeofrubber.’‘itwasasifIhadbeenridingahorseforaweekorsomethingandobviouslythatwastodowiththesaddlenumbness.‘Thefirstthingtogowasmybladderfunction’

Senseofchange/Seriousness

‘Ihadnocomprehensionthatthiscouldhavepermanentlyaffectedmymobilityandmylife…throughallofthisandthroughallthepain,andthroughallthepeoplethat;theambulances,theGPI’dseenatnight,itwasonlywhenthe

Consultantsaidtomejustbeforethesurgeryyou’rewithinthefortyeighthourwindowsoyourprospectsarequitegood.Ididn’tappreciatetherewasanythingbutalltheyhadtodo

wastakethispainaway’

N.BImportanceofsafetynettingthoseatrisk

ContactwithHealthProfessionals

Usuallyalreadyunderhealthprofessionalscare

Theyreallydoneedtolistentoyouandtheyneedtolistentoyourindividualcircumstances.

“IfIhadbeentoldnumbnessaroundbackpassageor

genitals…everyoneIsawwhowasmedicallytrainedcalleditsaddlenumbness”

Noclearsafetynetadvice

Suggestionstoaidearlydiagnosis

©Copyright2016BoltonNHSFoundationTrust.Nottobecopiedwithoutpermissionofthecopyrightowner,allrightsreserved.

Painiseasiertocommunicate!

Documentation

¡  The questions and the patient’s response should be clearly documented in the medical record

¡  Medico-legal viewpoint

¡  Assists practitioners in recognising changes e.g CESI to CESR

Patientliterature

¡  Cliniciansmusteducatepatientsandmakethemeffectiveself-advocates.(Strigenz,2014)

¡  Patientswithexistingriskfactorsmustbemadeawareof‘RedFlags’;needforpatienteducationofneworvaguesymptoms.(Mitchelleetal,2012)

¡  CESrequiresproperpatientinformation(Korseetal,2013);.

¡  Despitethisclearmessagemuchofthedocumentationdirectedtowardspatientsusesmedicalterminology‘Boweland/orbladderdysfunctionwithsaddleandperinealanaesthesia’(EgtonMedicalInformationSystems,2015)

GoodCommunicationiskey

SafetyNetting

LocalPathways

WillIknowwhattodoonFridayafternoon?

Prognosis

¡  Degreeofneurologicaldeficit,durationofcompression,speedofonset(Todd&Dickson,2016)

¡  ‘bringingtheindividualpatientandthesurgicalteamtogetherattheearliestpracticalopportunity’(Sonntag,2014)

Working with patients as partners we can make a difference

CaudaEquinaSyndrome;AsurgicalEmergency

AvailableMay2017;CSP

Acknowledgements

¡  Danishmusculoskeletalphysiotherapyassociation

¡  PhysiotherapyResearchFoundation(PRF)

¡  Patientswhofreelysharedtheirexperiencetoenableustolearn

¡  ProfessorJamesSelfe,ProfessorCaroleTrumanC,ProfessorValerieWebster

Thank you for listening

DrSueGreenhalghsusan.greenhalgh@boltonft.nhs.uk

References

Bin,M.Hong,W.Lian-shun,J.Wen,J.Guo-dong,S.Jian-gang,S.2009:Caudaequinasyndrome:Areviewofclinicalprogress.ChinMedJ122:1214-22 EngambaS.GaralevicieneD.Baldry,J2017:Containedrupturedabdominalaorticaneurysmpresentingascaudaequinasyndrome.BritishMedicalJournalFraserS,RobertsL,Murphy,E,2009:Caudaequinasyndrome;aliteraturereviewofitsdefinitionandclinicalpresentation.ArchPhysMedRehabil11;1964-8Germon,T.Ahuja,S,Casey,A,Rai,A.BritishAssociationofSpinalSurgeonsstandardsofcareforcaudaequinesyndrome2015.TheSpineJournalGreenhalghS,TrumanC,WebsterV,SelfeJ2015:AnInvestigationintothePatientExperienceofCaudaEquinaSyndrome(CES).PhysiotherapyPracticeandResearchGreenhalghS,TrumanC,WebsterV,SelfeJ.2016;Developmentofatoolkitforearlyidentificationofcaudaequinasyndrome.PrimaryHealthCareResearch&Development.SeliusB.andSubediR.2008;UrinaryretentioninAdults;DiagnosisandManagement.AmericanFamilyPhysician.77(5),643-650Stopler,K.HanlinE.AprilM.RitterJ.HunterC.SamseyK.Maddry,J2016:Thoracicspinalcordcompressionmasqueradingascaudaequinasyndrome.AmericanJournalofEmergencyMedicine.34756.e3-756.e5SmithS.2007:Drugsthatcausesexualdysfunction.Psychiatry.6(3),111-114ToddNVandDickenson,RA.2016;Standardsofcareincaudaequinasyndrome.BritishJournalofNeurosurgery.Vol30,no5,518-522WoodsE,GreenhalghS,SelfeJ.2015;CaudaEquinaSyndromeandthechallengeofdiagnosisforphysiotherapists:areviewPhysiotherapyPracticeandResearch