TheSaveSightKeratoconusRegistry:Trackingtreatmentoutcomesinpatients

withKeratoconusProfessorStephanieWatson

SaveSightInstituteBondiJunction

KERATOCONUS

• Affects1in2000,progressivelyreducesvision

• Onsetinchildhood/youngadult

• Life-longvisualdisability

• Evidencelackingontreatments

• asidefromcornealtransplantation(

• ACGRthankyou!

2007 2013 2015

RIGHTEYE

CORNEALCROSS-LINKING?

• Cornealstroma

• Collagenlayersconnectedtogetherbybridges‘x-links’

• Topicalriboflavin(VitaminB2)andUVlight(370nm)• Cornea300%strongerandmorestable

Wollensak,Spoerl andCataractRefractSurg 2003;29:1780–1785

MECHANISMSOFACTION

• Biological• Stimulationofkeratocyte apoptosisandrepopulation

• Mechanical• Increaseincrosslinkedcollagenfibre diameter• Increasedresistancetodegradation

• Chemical• Directelectrontransferorreactiveoxygenspecies

Mazzotta et al Eye, 2014;28:1179

• Riboflavin0.1%in20%Dextran

• q3to5minfor30min

• UVirradiation:3mW/cm2 30min

• Riboflavinq4to5min

Spoerl E,etlaCornea2007;26:385–389

HOWISITITDONE?

PROTOCOLVARIATIONS

• Treatthinnercorneas

• Avoidepithelialdebridement

• ‘Flash-linking’/AXL

• Combinedrefractivesurgery

• Iontophoresis

KERATOCONUS

• Progression• stabilizesby30’s

• Over3years

• 14to40%ofadults

• Children• moresevereKC

• morelikelyprogress

Godefrooij.Cornea2016;35:954–958

Ectasiaprogression

• consistentchangeinatleast2

• magnitudeofthechange• abovethenormalnoiseofthetestingsystem:

• Steepeningoftheanteriorcornealsurface

• Steepeningoftheposteriorcornealsurface

• Thinningand/oranincreaseintherateofcornealthickness

changefromtheperipherytothinnestpoint

Gomes etal,Cornea2015;34:359–369)

Ectasiaprogression

• Changesconsistentovertimeandabovethe

normalvariability(ie,noise)ofthemeasurement

system(thiswillvarybysystem).

• ChangeinuncorrectedvisualacuityandBSCVAis

notrequiredtodocumentprogression

Howaccurateistopography?

Eyes(KCgrade) Kmax (D)95%interobserver limits

Normal 0.78AllKC 1.51,2.30EarlyKC(1-2) -0.90to1.01AdvancedKC(>2) -3.71to3.86

EarlyKCK1,K2,orKmax >1Dlikelytobesignificant

Flynn,Sharma,Bunce,Wilkins.Br JOphthalmol 2016;100:1183–1187McAlinden etal.InvestOphthalmol VisSci.2011;52:7731–7737Hashemi K etal Ophthalmology 2015;122:211–12.Epstein RLetal JRefractSurg 2012;28:890–4.

Topographicprogression

• Sameoperator

• Sameequipment

• Repeatunreliablemeasurements

• Adjustparametersdependingthedegreeofectasia

Contraindications

• Pachy <400microns• Herpetickeratitis• Concurrentocularinfection• Severescarring• Neurotrophia• Historypoorcornealwoundhealing• Severedryeye• Pregnancy• Autoimmunedisorders

YOUR PATIENTS?

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

Don Peggy Sally

• Prospective,randomized,controlledclinicaltrial

• n=179

• Cornealectasiaafterpreviousrefractivesurgery

• StandardCXLvsriboflavinepitheliumintact

Outcomes

• EyeswithsteeperKmax >55.0D

• morelikelytohave>2Dormoreofflattening

Correcteddistancevision

RegistriesvsClinicalTrials

• Realworldtreatment• Realworldpatients

• Co-morbidities

• Largepatientnumbers

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

AimToestablisharegistryofoutcomesoftreatmentofoculardiseasestodeterminewhetherthesetreatmentsworkaswellintherealworld astheydidinclinicaltrials

MethodEfficientWeb-basedsoftwaretoolQualityassurancemeasuresSophisticatedstatisticalback-endTracksoutcomesthatpatientscareabout(vision,numberofinjections)

Save Sight Registries!

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

Design Goals• Basicanonymised patientdatamanagementsystem

• Compatiblewithdataentryinroutinepractice• Baselinevisitentry:30sec• Followupvisitentry:15sec

• Generategraphs“onthefly"ofvision,treatmentandrelevantaudit-specificclinicalmarkers

• Selfaudittool

• OptoutconsentinAustralia– patientgivenabrochure

• Patientdataremainsunidentifiable

• Doctor’sdataisidentifiableONLYbythetreatingDoctor

• Thecopieddataisownedbythecollaborationbutmaybewithdrawnatanytimewithoutnoticeorreasonbythecontributingpractitioner

Consent, Anonymity and Ownership

Tracking treatment outcomes in nAMD

FRB!MacularDegeneration

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

Save Sight Registries

FCB:KERATOCONUS• Nationalregistryto

• Collecthighqualitydataoutcomes

• Determineclinicaleffectivenessandsafetyofemergingtherapiesandsurgicaltechniques

• Patientsinreallifeclinicalsettings

• BasedonthenationalandinternationalsuccessoftheFRB!ProjectdevelopedbyProfMarkGilles

FCB!Keratoconus

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

Quick comparisonofclinicaloutcomesagainstpeers

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

Patienttreatmentoutcomesbydoctorpracticereport:• informativereport• selfaudittool• anonymouslygeneratedwithinthesystemuponrequest

• evaluatingeffectivenessoftreatment

GeneratingDoctor’sActivityReport

BENCHMARKING

FCB!Keratoconus

Patientjourney

KORQ

• Benefittopatients

oftreatments

Orthopaediccentre

UniversityofRochester

MedicalCentre

USA

INTHECLINIC

• Orthoptic review• Vision• Topography• Dataentry

• KORQcompletedonIpad• Clinicianconsult

• Finalise data• Review• Discuss

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

RANZCOApprovedCPDAuditTheProjectisaRANZCOapprovedCPDAuditactivity.Participatingfellowsare

potentiallyabletoclaim30pointsinclinicalexpertiselevel2.

TOQUALIFYFORCPDPOINTS

Institutingchangesinyour

practice

Auditingatleast85%ofpatientstreatedinyourpracticewithAMD/DME/KC

Reflectingonresults

generatedfromyourowndata

Attendingmeetings

Sharingorcomparingfindingswith

peers

Generatingdoctor's

activityreports

Publications accepted in following journalsOphthalmologyRetinaBJO IVOS

Global Implementation

Recruitment Update

Eyes 2298

Patients 1228

AllVisitsTotal 11349

Sites 37

X link 1062

SaveSightRegistries

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

RECRUITMENTUPDATE

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

ADVERSEEVENTS

Haze MicrobialkeratiitisPersistentepitheilaldefect ScarringSterileinfiltrates Stromaloedema

FCB!Keratoconus

COMPLICATIONS

• Loss≥ 2SnellenlinesVA 2.9%(95%CI,0.6%-8.5%).• Riskfactors

• Ageolderthan35yearsand

• Preop CDVA> 20/25

• Continuedprogression 8%- 11%• Riskfactors

• HighpreKmax Ê 54D

• Centralcone

• Pachy Ê 450microns

Koller etal JCataract Refract Surg 2009;35:1358–1362

HAZE

• Common

• Decreasesinfirstyear

• Extendsto60%depth(300microns)

• PRKhazeissubepithelial

Koller etal JCataract Refract Surg 2009;35:1358–1362

SCARRING• Centralstromalscarsin2.8%

• Infectionacquiredpostop

Koller etal JCataract Refract Surg 2009;35:1358–1362

BENEFITSFORYOURDOCTOR

• Individualise patienttreatment

• Patientdatagraphed

• Practicelevelsummaries

• Contributetodevelopmentofnationaltrends

• Compareowndatatobenchmarks

FCB!Keratoconus

REGISTRYINPRACTISE

• Individualise patienttreatment

• Patientdatagraphed

• Practicelevelsummaries

• Compareowndatatobenchmarks

• Contributetodevelopmentofnationaltrends

FCB!Keratoconus

BENEFITSTOYOU

• Trackyourtreatmentjourney

• Contributetobestpractise

• Impactsofkeratoconus

• Datareferral

• Yourdatacanbereferredtoanotherpractitioner

FCB!Keratoconus

Keratoconusinayoungadult

Year 2007 2013 2015

Unaided 6/6 6/18Glasses +0.25/-3.50at65º

6/7.5+0.25/-3.50at70º6/5

Year 2007 2013 2015

Unaided 6/36 6/18Glasses - /-1.25 at120º

6/12-1.50/-1.00at150º6/30

-2.50/-3.50at135º6/12

LEFTEYE

RIGHTEYE

2007 2013 2015

RIGHTEYE

2007 2013 2015

LEFTEYE

• StableKCN• VAsatisfactoryinglasses

• Glassesalone• +/- CLsorCXL

• ProgressiveKCN• VAsatisfactoryinglasses

• CXL• Glasses+/- CL

Gomes etal,Cornea2015;34:359–369

Keratoconusinyoungadult

Keratoconusinayoungchild

• Progressioncanberapid

• Highqualityevidencelacking

• Caseseriesshowprogressioncanbestopped

• Risks:progression

• Over5years:25%mayprogress

• Infectionandscarring

Wisse RP,Godefrooij DA,Soeters N.Reply.Cornea.2016;35(11):e36.

Keratoconusinayoungchild

• Mildcases• VA6/6

• Fewtopographicsigns

• Followup:1- 3months

• AdvancedKCN• ConsiderCXL

• AsymmetricKCN• Monitorgoodeye

• Casebycasedecision• Risksvsbenefits

Keratoconusinanadult

• 55yearold• c/oreducedvision• Pohx:

• Glassessinceteenager

• Usetorubeyesbuthasstopped

• RVA6/12 LVA6/12

-1.50/-1.25at84º +0.25/-4.50at109º

RIGHTEYE

LEFTEYE

Keratoconusinolderadult

• StableKCN• VAsatisfactoryinglasses

• GlassesorCL

• ProgressiveKCN• VAsatisfactoryinglasses

• CXL

Gomes etal,Cornea2015;34:359–369

• CurrentlyonlyRxabletohaltprogressiveectasia

• Evolvingmethods

• Newindications

• Monitoroutcomes

CXL

©2010– 2015SaveSightInstitute,TheUniversityofSydney.

CIB: DanielBarthelmesProjectManager: AmparoHerrera-BondCoordinators: AmandaDinhBiostatisticians: VuongPhuc

RichardWalton

ENQUIRIES– amparo.herrerabond@sydney.edu.au

Prof Mark GillesAProf Mark DaniellDr Elsie ChanDr Daniel BarthelmesDr Martina BoschDr John Males Dr Yves Kerdraon

Dr Nigel MorletDr John Males Dr Yves KerdraonDr Richard MillsDr Andrew ApelDr Peter Beckingsale

• ORIA• KeratoconusAustralia

• Claffy Foundation