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ADVANCESIN Ophthalmology andOptometry Editor-in-Chief MyronYanoff,MD
ChairEmeritus,DepartmentofOphthalmology,DrexelUniversity, AdjunctProfessor,DepartmentofOphthalmology,Universityof Pennsylvania,Philadelphia,Pennsylvania
Editor:MeganAshdown
DevelopmentalEditor:JessicaCanaberal
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ADVANCESIN OphthalmologyandOptometry Editor-in-Chief
MYRONYANOFF,MD,ChairEmeritus,DepartmentofOphthalmology,DrexelUniversity,AdjunctProfessor,DepartmentofOphthalmology,UniversityofPennsylvania,Philadelphia,Pennsylvania
SectionEditors
BHAVNACHAWLA,MD –OphthalmicPathology&OcularOncology
RPCenterforOphthalmicSciences,AllIndiaInstituteofMedicalSciences,New Delhi,India
DAVIDA.CRANDALL,MD –Cataract&RefractiveSurgery
GlaucomaFellowshipDirector,HenryFordHealthSystem,Detroit,Michigan UniversityofUtah,SaltLakeCity,WayneStateUniversity,SaltLakeCity,Utah
GABRIELAMABELESPINOZA,MD –Oculoplastics
ClinicalProfessor,DepartmentofOphthalmologyOphthalmicPlasticand ReconstructiveSurgeryAssistantDeanofEducationalProgramming,Graduate MedicalEducationSaintLouisUniversitySchoolofMedicine
PAULB.FREEMAN,OD,FAAO,FCOVD –Optometry
Diplomate,LowVision,Pittsburgh,Pennsylvania,ClinicalProfessor,Rosenberg SchoolofOptometry,UniversityoftheIncarnate,Word,SanAntonio,Texas
RUSTUMKARANJIA,MD,PhD,FRCSC,DABO –Neuro-ophthalmology
DepartmentofOphthalmology,DavidGeffenSchoolofMedicineatUCLA, LosAngeles,California;DohenyEyeInstitute,LosAngeles,California,Doheny EyeInstitute,LosAngeles,California,DepartmentofOphthalmology,University ofOttawa,Ottawa,Ontario,Canada,OttawaHospitalResearchInstitute,The OttawaHospital,Ottawa,Ontario,Canada
RONNIM.LIEBERMAN,MD –VitreoretinalDisease
AssistantProfessorofOphthalmology,IcahnSchoolofMedicineatMt.Sinai, QueensHospitalCenter,Jamaica,NewYork
ANN-MARIELOBO,MD –Uveitis
Co-Director,UveitisService,AssociateProfessor,Departmentof OphthalmologyandVisualSciences,IllinoisEyeandEarInfirmary, UniversityofIllinoisatChicago,Chicago,Ilinois
STEPHENE.ORLIN,MD –CorneaandExternalDiseases
AssociateProfessor,ScheieEyeInstitute,UniversityofPennsylvania,Perelman SchoolofMedicine,Philadelphia,Pennsylvania
JOSEPHM.ORTIZ,MD,FRCOphth –Glaucoma
ConsultantinOphthalmology,SacredHeartHospital,Allentown, Pennsylvania;ConsultantinOphthalmology,AbingtonMemorialHospital, Abington,Pennsylvania;FormerlyAssistantProfessorofOphthalmology, HahnemannUniversityHospital,DrexelUniversitySchoolofMedicine, Philadelphia,Pennsylvania
LEONARDJ.PRESS,OD,FAAO,FCOVD –Optometry
PressConsulting,P.C,Lakewood,NewJersey;AdjunctProfessor,Southern CollegeofOptometry,Memphis,Tennessee
APARNARAMASUBRAMANIAN,MD –PediatricOphthalmology
DirectorofRetinoblasmaandOcularOncology,PhoenixChildren’sHospital, Phoenix,Arizona
ADVANCESIN OphthalmologyAndOptometry CONTRIBUTORS REDDINAHMAD,BMBS,iBSC,FoundationYear2Doctor,BradfordTeaching HospitalsNHSFoundationTrust,Bradford,WestYorkshire,UnitedKingdom
MARIBAKANDA,MD,ResidentPhysician,DepartmentofOphthalmology,Northwell HealthEyeInstitute,GreatNeck,NewYork,USA
TOMASANDERSEN,MD,MPH,ScheieEyeInstitute,PerelmanSchoolofMedicine, UniversityofPennsylvania,Philadelphia,Pennsylvania,USA
SHAHZANANJUM,MSc,OcularPathologyServices,Dr.RajendraPrasadCentrefor OphthalmicSciences,AllIndiaInstituteofMedicalSciences,NewDelhi,Delhi, India
RAMESHAYYALA,MD,DepartmentofOphthalmology,UniversityofSouthFlorida CollegeofMedicine,Tampa,Florida,USA
NOORANBADEEB,MBBS,DepartmentofOphthalmology,SchoolofMedicine, UniversityofJeddah,Jeddah,SaudiArabia
ALIREZABARADARAN-RAFII,MD,DepartmentofOphthalmology,Universityof SouthFloridaCollegeofMedicine,Tampa,Florida,USA
SUZANNAHBELL,MBChB,DTMH,MSc,QueenAlexandraHospital,Portsmouth, UnitedKingdom
RUPAKBHUYAN,MD,DepartmentofOphthalmology,IcahnSchoolofMedicineat MountSinaiHospital,NewYork,NewYork,USA
KASTURIBHATTACHARJEE,MS,FRCSEd,Head,DepartmentofOphthalmicPlastic andReconstructiveSurgeryandOculofacialAesthetics,SriSankaradeva Nethralaya,Guwahati,Assam,India
ELENAZ.BIFFI,OD,MS,FAAO,AssociateProfessorofOptometry,NewEngland CollegeofOptometry,Boston,Massachusetts,USA
MARIEI.BODACK,OD,FAAO,Diplomate,BinocularVision,Perceptionand PediatricsOptometry;Professor,DepartmentofPediatricPrimaryCare,Southern CollegeofOptometry,Memphis,Tennessee,USA
JEFFREYBROWN,MD,DepartmentofOphthalmology,IcahnSchoolofMedicineat MountSinaiHospital,NewYork,NewYork,USA
MEHARCHANDSHARMA,MD,DepartmentofPathology,AllIndiaInstituteof MedicalSciences,NewDelhi,Delhi,India
EMILYCHANG,MD,Resident,DepartmentofOphthalmology,KelloggEyeCenter, UniversityofMichigan,AnnArbor,Michigan,USA
BHAVNACHAWLA,MD,ProfessorofOphthalmology,OcularOncologyService,RP CentreforOphthalmicSciences,AllIndiaInstituteofMedicalSciences,New Delhi,India
BENSONS.CHEN,MBChB,MSc,FRACP,DepartmentofClinicalNeurosciences, UniversityofCambridge,Cambridge,UnitedKingdom;CambridgeEyeUnit, Addenbrooke’sHospital,CUH-NHS,Cambridge,UnitedKingdom;Gonvilleand CaiusCollege,UniversityofCambridge,Cambridge,UnitedKingdom
RAOV.CHUNDURY,MD,TruhlsenEyeInstitute,UniversityofNebraskaMedical Center,Omaha,Nebraska,USA
CAROLINEW.CHUNG,MD,ResidentPhysician,ScheieEyeInstitute,Philadelphia, Pennsylvania,USA
Y.GRACECHUNG,BS,EmoryUniversitySchoolofMedicine,Atlanta,Georgia,USA
DEVINCOHEN,MD,ScheieEyeInstitute,PerelmanSchoolofMedicine,Universityof Pennsylvania,Philadelphia,Pennsylvania,USA
STUARTCOUPLAND,PhD,DepartmentofOphthalmology,FacultyofMedicine, UniversityofOttawa,OttawaHospitalResearchInstitute,TheOttawaHospital, Ottawa,Ontario,Canada
KEVINJ.EVERETT,MD,DirectorofRefractiveSurgery,HenryFordHospital DepartmentofOphthalmology,Detroit,Michigan,USA;HenryFordOptimEyes SuperVisionCenter-SterlingHeights,SterlingHeights,Michigan,USA
SHAILESHGAIKWAD,MD,FNAMS,CCST,Professor,DepartmentofNeuroimaging andInterventionalNeuroradiology,AllIndiaInstituteofMedicalSciences,New Delhi,India
SAMUELGELNICK,MD,NorthwellHealthEyeInstitute,GreatNeck,NewYork,USA
ROBINGINSBURG,MD,DirectoroftheVitreoretinalService,AssistantProfessor, DepartmentofOphthalmology,IcahnSchoolofMedicineatMountSinai Hospital,DepartmentofOphthalmology,NewYorkEyeandEarInfirmaryat MountSinai,NewYork,NewYork,USA
CHLOEGOTTLIEB,MD,DepartmentofOphthalmology,FacultyofMedicine, UniversityofOttawa,OttawaHospitalResearchInstitute,TheOttawaHospital, Ottawa,Ontario,Canada
ANGELAGUPTA,MD,PhD,ResidentPhysician,ScheieEyeInstitute,Philadelphia, Pennsylvania,USA
MARIAMS.HAMID,MD,ChiefOphthalmologyResident,HenryFordHospital DepartmentofOphthalmology,Detroit,Michigan,USA
GEORGEJIAO,MD,ResidentPhysician,DepartmentofOphthalmology,Northwell HealthEyeInstitute,GreatNeck,NewYork,USA
MANLIJIN,MD,ChiefOphthalmologyResident,HenryFordHospitalDepartmentof Ophthalmology,Detroit,Michigan,USA
PRIYAKALYAM,MD,OculoFacialPlasticandReconstructiveSurgeon,LegacyEyelids &CosmeticSurgery,Plano,Texas,USA
PUSHPINDERKANDA,MD,PhD,DepartmentofOphthalmology,FacultyofMedicine, UniversityofOttawa,Ottawa,Ontario,Canada
JOSEPHEDWARDKANEJr,OD,FAAO,AttendingOptometrist,VeteransAffairs BostonHealthcareSystem
LYNCAKANTUNGANE,COMT,DepartmentofOphthalmology,FacultyofMedicine, UniversityofOttawa,Ottawa,Ontario,Canada
RUSTUMKARANJIA,MD,PhD,DepartmentofOphthalmology,FacultyofMedicine, UniversityofOttawa,OttawaHospitalResearchInstitute,TheOttawaHospital, Ottawa,Ontario,Canada;DohenyEyeInstitute,DohenyEyeCentersUCLA, DepartmentofOphthalmology,DavidGeffenSchoolofMedicineatUCLA,Los Angeles,California,USA
JAMIEA.KEEN,MD,DepartmentofOphthalmologyandVisualSciences,University ofIowa,IowaCity,Iowa,USA
SERENALI,BA,DepartmentofClinicalNeurosciences,UniversityofCambridge, Cambridge,UnitedKingdom;GonvilleandCaiusCollege,Universityof Cambridge,Cambridge,UnitedKingdom
RONNIM.LIEBERMAN,MD,AssistantProfessor,DepartmentofOphthalmology, MountSinaiMedicalCenter,IchanSchoolofMedicine,MountSinaiHealth Systems,NewYork,NewYork,USA
JACOBLIECHTY,MD,DepartmentofOphthalmology,UniversityofSouthFlorida CollegeofMedicine,Tampa,Florida,USA
TIANYULIU,MD,OphthalmologyResident,ScheieEyeInstitute,Universityof Pennsylvania,Philadelphia,Pennsylvania,USA
STEPHANIEM.LLOP,MD,BascomPalmerEyeInstitute,UniversityofMiamiMiller SchoolofMedicine,Miami,Florida,USA
SAMANTHAL.MAREK,MD,OphthalmologyResident,ScheieEyeInstitute, UniversityofPennsylvania,Philadelphia,Pennsylvania,USA
KERRIMCINNIS-SMITH,BA,MayoClinicAlixSchoolofMedicine,Scottsdale, Arizona,USA
ADITIMEHTA,MD,Fellow,DepartmentofOphthalmicPlasticandReconstructive SurgeryandOculofacialAesthetics,SriSankaradevaNethralaya,Guwahati, Assam,India
HOLLYK.MILLER,DO,PhoenixChildren’sHospital,Phoenix,Arizona,USA;Mayo Clinic,Scottsdale,Arizona,USA
MARIYAMOOSAJEE,MBBS,BSc,PhD,FRCOphth,Professor,MoorfieldsEye HospitalNHSFoundationTrust,UniversityCollegeLondonInstituteof Ophthalmology,TheFrancisCrickInstitute,London,UnitedKingdom
JENNIFERB.NADELMANN,MD,ResidentPhysician,ScheieEyeInstitute, Philadelphia,Pennsylvania,USA
MEHDINAJAFI,MD,PhD,VitreoretinalSurgeon,BostonVision,BethIsrael DeaconessMedicalCenter,Boston,Massachusetts,USA
BRIANJ.NGUYEN,MD,OphthalmologyResident,ScheieEyeInstitute,Universityof Pennsylvania,Philadelphia,Pennsylvania,USA
JACQUELYNO’BANION,MD,MSc,EmoryEyeCenter,Atlanta,Georgia,USA; DepartmentofOphthalmology,EmoryUniversitySchoolofMedicine
STEPHENE.ORLIN,MD,AssociateProfessorofOphthalmology,ChiefofCornea Service,ScheieEyeInstitute,PerelmanSchoolofMedicine,Universityof Pennsylvania,Philadelphia,Pennsylvania,USA
CHRISTIEM.PERSON,OD,FAAO,GradyHealthSystem,EyeClinic,Atlanta, Georgia,USA
SUSANA.PRIMO,OD,MPH,FAAO,EmoryEyeCenter,Atlanta,Georgia,USA; DepartmentofOphthalmology,EmoryUniversitySchoolofMedicine
VIVIANL.QIN,MD,ScheieEyeInstitute,PerelmanSchoolofMedicine,Universityof Pennsylvania,Philadelphia,Pennsylvania,USA
APARNARAMASUBRAMANIAN,MD,PhoenixChildren’sHospital,Phoenix, Arizona,USA
ERICKRIVERA-GRANA,MD,AssistantProfessorofClinicalOphthalmology, DepartmentofOphthalmology,UniversityofPuertoRicoSchoolofMedicine, SanJuan,PuertoRico,USA
CHRISTINERYU,MD,TruhlsenEyeInstitute,UniversityofNebraskaMedicalCenter, Omaha,Nebraska,USA
SEEMASEN,MD,OcularPathologyServices,Dr.RajendraPrasadCentrefor OphthalmicSciences,AllIndiaInstituteofMedicalSciences,NewDelhi,Delhi, India
RAHULA.SHARMA,MD,MPH,FRCSC,DepartmentofOphthalmologyandVision Sciences,UniversityofToronto,Toronto,Ontario,Canada;PrismEyeInstitute, Oakville,Ontario,Canada
ERINM.SHRIVER,MD,DepartmentofOphthalmologyandVisualSciences, UniversityofIowa,IowaCity,Iowa,USA
NAVNEETSIDHU,MD,SeniorResident,Ophthalmology,RPCentreforOphthalmic Sciences,AllIndiaInstituteofMedicalSciences,NewDelhi,India
MINHTRINH,MD,NorthwellHealthEyeInstitute,GreatNeck,NewYork,USA
SRAVANTHIVEGUNTA,MD,PediatricOphthalmologyandAdultStrabismusFellow, DepartmentofOphthalmology,IndianaUniversitySchoolofMedicine, Indianapolis,Indiana,USA;AssistantProfessor,DepartmentofOphthalmology andVisualSciences,UniversityofUtahSchoolofMedicine,SaltLakeCity,Utah, USA
VATSALYAVENKATRAMAN,MD,Fellow,DepartmentofOphthalmicPlasticand ReconstructiveSurgeryandOculofacialAesthetics,SriSankaradevaNethralaya, Guwahati,Assam,India
LAURAWINDSOR,OD,FAAO,LowVisionCentersofIndiana,DivisionoftheEye AssociatesGroup,LLC,Indianapolis,Indiana,USA
RICHARDWINDSOR,OD,FAAO,DPNAP,LowVisionCentersofIndiana,Division oftheEyeAssociatesGroup,LLC,Indianapolis,Indiana,USA
BENJAMINYOUNG,OD,FAAO,AssistantProfessorofOptometry,NewEngland CollegeofOptometry,Boston,Massachusetts,USA
CAROLINEY.YU,MD,DepartmentofOphthalmologyandVisualSciences,University ofIowa,IowaCity,Iowa,USA
AMYZHANG,MD,AssistantClinicalProfessor,DepartmentofOphthalmology, KelloggEyeCenter,UniversityofMichigan,AnnArbor,Michigan,USA
ADVANCESIN OphthalmologyAndOptometry LookingBackwardandForward
Optometry
BiopticDrivingintheUnitedStates
LauraWindsorandRichardWindsor
TheconceptofbiopticdrivingbeganintheUnitedStates afterthedevelopmentofapracticalbiopticsystemby WilliamFeinbloom,OD,PhD.Abioptictelescopeisa telescopemountedinoratthetopofthespectaclelenses, allowingthewearertoviewunderneathit,throughthe carrierlenses,andtointermittentlytiptheirheaddownto quicklyviewthroughthetelescope.Thebioptictelescope isusedforspottingsigns,trafficlights,andotherobjects inthedistancewhendriving.Mildtomoderatelyvisually impairedpatientshavebeenusingbiopticsformorethan 50yearstodrive.Moststateshaveregulationsonbioptic driving,buttherulesvarygreatly.Propercandidate selection,fittingofthebioptic,andtrainingarecrucialfor thedriver’ssuccessandsafetyontheroad.Patients shouldbeconsideredonacase-by-casebasistodetermine theireligibilitytodrive.Morenaturalisticresearch,thatis, intherealworld,isneededtofurthervalidatethesafety ofbiopticdriving,thetrainingneeded,andtolookat statevisionregulationsfordriving.
Introduction1 HistoryofbiopticsanddrivingintheUnitedStates2 Alegalpathwayforbiopticdriving3 Controversiesandresearchinbiopticdriving4 Wearingthebiopticonlytoobtainlicensure4
Ringscotomaofthetelescope5
Biopticdrivingsafety5
BiopticsintheUnitedStates6
Visualacuities7
Visualfields8
Bioptictraining8
Nighttimedriving8
Bioptictelescoperequirements8
Ocularconditionsandabilitytodrive8
Otherfactorsinpatientselectionforbiopticdriving14
Visualprocessingskillsandcognitiveabilities14
Motorskillsandfunctioning15
Ageandmaturity15
Medicalandmedicationhistory15
Emotional/mentalstatus15
Familysupport15
Designingthebiopticsystem16
Powerandpositionofthetelescope16
Fieldofviewthroughthetelescope17
Adaptabilityofthebiopticforlightandglareissues17
Trainingthebiopticdriver18
Trainingtousethebioptic19
Trainingwiththecertifieddriverrehabilitationspecialist19
Afterbiopticlicensure20
Vehiclemodificationsforbiopticdrivers21
Vehicleselection21
Globalpositioningsystems21
Speedometer21
Windshieldanddashglare21
TheIndianaprogramforvisuallyimpaireddrivers22
Part1:waivereddrivers22
Part2:biopticdrivers22
Thefacesofbiopticdriving23
Dr.DennisKelleher:firstlegallylicensedbiopticdriverinthe UnitedStates26
Futureresearchinbiopticdriving26
Summary27
Clinicscarepoints27 Disclosure27
TheUseofB-ScanUltrasoundinPrimaryEyeCare
ElenaZ.Biffi,BenjaminYoung,JosephEdwardKaneJr.,and MehdiNajafi
B-scanultrasoundhasbeenapartofaneyecareprovider’s armamentariumofophthalmicimagingtechniquesdating backtoatleastthe1950s.Evenwiththeadventofnewer imagingdevices,B-scanultrasonographyservesanimportant adjunctroleinassessmentofvariousophthalmicconditions. Relativelybenignocularconditionsaswellaspotentiallylife-
threateningpathologiesmaybeidentifiedandassessedusing thisprocedure.Athoroughunderstandingoftheprinciples andpracticalapplicationsof B-scanultrasonographyis essentialtomaximizeuseofthistechniqueinpatientmanagement.
Introduction31
Indicationsandcontraindications32 PrinciplesofB-scanultrasoundexamination33 B-scandescription33
Probepositionsandimageinterpretation34
Clinicalexaminationprinciples36 B-scanapplicationsinclinicalcare36
Vitreousdiseases36 Retinaldiseases39 Choroidaldiseases42
NonsurgicalTreatmentofStrabismus MarieI.Bodack
Nonsurgicaltreatmentoptionsforesotropiaandexotropia maybeindicatedforsomepatientstoimproveoutcomes presurgeryorpostsurgeryorinlieuofsurgery.The idealoutcomeisforapatienttohavebinocularityand excellentalignment.Currentnonsurgicaltreatment optionsforpatientswithesotropiaandexotropiainclude lenses,prisms,occlusion,andvisiontherapy.
Introduction51
Pediatric UpdateonPediatricOpticNeuritis SravanthiVegunta
Pediatricopticneuritis(ON)isaconditionwithabroad differentialincludingdemyelinatingsyndromes, astrocytopathies,andinfectiousprocesses.Many retrospectivestudieshavebeenpublishedonthe similaritiesanddifferencesbetweenpediatricON secondarytoclinicallyisolatedsyndrome,multiple sclerosis,myelinoligodendrocyteglycoprotein,and neuro-myelitisopticaspectrumdisorder.The presentations,visualoutcomes,andlaboratorytesting, andimagingfindingshavesignificantoverlapbut importantdifferences.Thisarticledetailshowto distinguishbetweenvariouscausesofautoimmuneON toprovidetheappropriateacuteandchronictherapiesto patients.Gapsinourcurrentknowledgeandsuggestions forfuturestudiesarediscussed.
Introduction71 Significance72 Overviewofpediatricopticneuritis72 Multiplesclerosis-associatedopticneuritis76 Myelinoligodendrocyteglycoprotein-associateddiseaseopticneuritis76 Neuromyelitisopticaspectrumdisorderopticneuritis80 Othercausesofopticneuritis83
Relevanceandfutureavenuestoconsider84
Summary84
Acknowledgments85
GeneticsofCongenitalCataract ReddinAhmad,SuzannahBell,andMariyaMoosajee
Upto40,000childrenarebornwithcataractsannually worldwide.IntheUK,childrenpresentingwithbilateral cataractmostcommonlyhaveageneticbasis.Mutations ingenesinvolvedinlens-specificproteinsorregulation ofeyedevelopmentcancauseisolatedcataract,those associatedwithmorecomplexoculardefectsand/or syndromicfeatures.Earlysurgeryandfrequentfollowuparevitaltoavoidamblyopiaandoptimizevisual potential.Routinegenetictestingisimportanttoallow accuratediagnosisandpersonalizedmanagementof patientsincludingtailoredgeneticcounseling. Multidisciplinarycareisvital,includingclinicalgenetic
andophthalmicteams,pediatricians,school,andlocal pediatricvisualsupportservices.
Introduction89
Causeandclassification90
Significance91
Nonsyndromiccongenitalcataract91
Crystallins91
Cytoskeletalstructuralproteins95
Membraneproteins95
Transcriptionfactors98
Syndromiccongenitalcataract110
Downsyndrome(Trisomy21)110
Patausyndrome(Trisomy13)110
Lowesyndrome110
Norriedisease110
Nance-Horansyndrome111
Myotonicdystrophy1111
Neurofibromatosistype2111
Zellwegerspectrumdisorder111
Rothmund-Thompsonsyndrome112
Cockaynesyndrome112
Cerebrotendinousxanthomatosis112
Galactosemia112
Acquiredcauses112
Congenitalinfections112
Trauma113
Othercommoncausesofcataractinchildren113
Uveiticcataractandsteroid-relatedcataract113
Radiationcataract113
Relevance113
Managementofchildhoodcataract113
Genetictesting114
Multidisciplinarycare114
Summary114
Clinicscarepoints115
Disclosure115
PediatricGraft-Versus-HostDisease KerriMcInnis-Smith,HollyK.Miller,and AparnaRamasubramanian
Evenwiththesupportofevidence-basedmedicine available,theproperdiagnosisandtreatmentofgraftversus-hostdisease(GVHD)anditsophthalmologic manifestations,especiallyinthepediatricpopulation,is quitenuanced.Thewidearrayofdiseasepresentations, theexistenceofconfoundingfactorssuchasunderlying diseaseandconcurrenttreatment,andthechallengeof
childrenaccuratelyconveyingtheirsymptomscanfurther contributetothedifficultyineffectivelymanagingyoung patientswithchronicGVHD.Cliniciansmustconsider theexpertrecommendationsformanagementwhile choosingdiagnosticapproachesandtherapeuticregimens accordingtotheindividualpatient.
Background119
Hematopoieticstemcelltransplant120
PathophysiologyofsystemicGVHD121
IncidenceofChronicGVHD121
DiagnosisandClassificationofSystemicGVHD122
GradingofSystemicDiseaseSeverity124
RiskFactorsforSystemicGVHD125
Prognosis125
OcularGVHD125
Mainstaysandrecentdevelopmentsintherapy130
Summary135 Clinicscarepoints135 Disclosure135
OphthalmicPathology&OcularOncology FluorescenceInSituHybridizationinOcularOncology
SeemaSen,ShahzanAnjum,andMeharChandSharma
Fluorescenceinsituhybridizationisausefultooltovisualize andmapthegeneticmaterialinanindividual’scells, includingspecificgenesorportionsofgenes.This techniquehasdiagnosticandprognosticimplicationsfora varietyofocularmalignanttumorsbecausegenetic mutationsdetectedbyFISHprovideusefulinformationfor bettermanagementincludingtargetedtherapy. Chromosomalabnormalitiesincludingt(14;18) translocationinlymphoma,monosomyofchromosome3 inuvealmelanoma,andt(6;9)translocationinadenoid cysticcarcinomaoflacrimalglandaresomeofthecommon genetichallmarksofthesemalignancies.
Introduction139
Significanceoffluorescenceinsituhybridization140 GeneralPrincipleofFluorescenceInSituHybridization141 DetailedFluorescenceInSituHybridizationProtocolforTissue Sections141
OcularLymphomas142 UvealMelanomas143 AdenoidCysticCarcinoma144 Presentrelevanceandfutureoffluorescenceinsituhybridization145 Clinicscarepoints146
Intra-arterialChemotherapyforRetinoblastoma:AnUpdate BhavnaChawla,NavneetSidhu,andShaileshGaikwad
Retinoblastomaisthemostcommonprimaryintraocular tumorinchildren.Italsoisoneofthemostsuccessfully treatedtumorsusingvarioustreatmentstrategies. Conventionalglobesalvagetreatmentsincludetheuseof focaltherapiesincombinationwithsystemic chemotherapy.Intra-arterialchemotherapy(IAC)isa comparativelynewertreatmentmodalityusedprimarily forglobesalvageinrefractoryretinoblastoma.It involvesthedirectadministrationofchemotherapy drugsintotheophthalmicarterytoachieveahighlocal intraocularconcentration.IAChasbeenfoundto successfullytreatretinoblastoma,andafewnewer modificationsarebeingtried.Ourarticlediscussesthe significanceofIAC,techniqueemployed,drugsused, andneweradvancesinitsadministration.
Introduction151 History152 Intra-arterialchemotherapyterminology153 Procedure153
Intra-arterialchemotherapydrugs154 Significanceofintra-arterialchemotherapy155 Complicationsofintra-arterialchemotherapy156 Local156 Systemic156 What’snewinintra-arterialchemotherapy?157 TreatmentApproaches157 Drugs157 Patientprofile157 Summary157 Clinicscarepoints158 Disclosure159
AdvancesinPediatricPeriocularVascularNeoplasms KasturiBhattacharjee,AditiMehta,andVatsalyaVenkatraman
Pediatricperiocularvascularneoplasmsoriginatefrom transformedendothelialcellsshowingincreased proliferation.Commonestoftheseisinfantile hemangioma,whichpresentsinthefirstyearoflife.The lesionexhibitsinitialrapidgrowthfollowedby spontaneousregression.Locallyinfiltrativeand malignanttumors,althoughrare,mayexhibitmetastatic
potential.Neoplasmsneedtobedifferentiatedfrom congenitalvascularmalformations,whichare noninvoluting,exhibitgrowthcommensuratelywithage, anddonotdemonstrateendothelialproliferation.Timely treatmentofperiocularlesionsobscuringthevisualaxis helpspreventamblyopia.Systemicinvolvementor presenceoflargelesions(>5cm)warrantsassessmentof cardiacfunctionasthehigh-flowlesionsmayproduce high-outputcardiacfailure.Masseffectofextensive perioral/nasal/subglotticlesionsmaycompromisethe airway.Amultidisciplinaryapproachisimportantfor optimizingtreatmentoutcomes.Propranololhasgained FDAapprovalin2017asthefirst-linetherapyfor infantilehemangioma.Incontrast,malformationsare difficulttotreat,andtherapymostlyissymptomatic.
Introduction161
Significance162
Vasculartumors162 Vascularmalformations165 Secondaryorbitalinvolvement169 Presentrelevanceandfutureavenuestoconsider170 Summary173 Clinicscarepoints174 Disclosure174
Cataract&RefractiveSurgery FemtosecondLaser-AssistedCataractSurgery EmilyChangandAmyZhang
Sincefemtosecondlaser-assistedcataractsurgery(FLACS) firstbecamecommerciallyavailableapproximatelya decadeago,ithascontinuedtoprovokemuchinterestas wellascontroversy.Asfemtosecondlasertechnology continuestoevolve,FLACShasshowntobeasafe alternativetotheconventionalphacoemulsification surgery.However,thesuccessfuladoptionofanew technologyrequiresbothcostefficiencyandsignificant improvementoverconventionaltechniques.Thisarticle summarizestheutilizationoffemtosecondlaser technologyincertainstepsofcataractsurgeryand reviewsitssafetyandefficacyineachstep.
Introduction177
Femtosecondlasertechnology178 Clearcornealincision178 Anteriorcapsulectomy179
Nuclearfragmentation181
Astigmatismmanagement183
Summary183
Clinicscarepoints184 Disclosure184
AdvancedTechnologyIntraocularLenses MariamS.Hamid,ManLiJin,andKevinJ.Everett
Recentlydevelopedadvancedtechnologyintraocular lenses(IOLs)aimtoprovidepatientswiththeabilityto seein-focusimagesatmultipledistancestomimicthe naturalaccommodativefunctionoftheeye.Multifocal IOLsusediffractive,refractive,orhybridtechnologiesto allowimagesfrommultiplefocalpointstobe simultaneouslyfocusedontheretina.Thisinadvertently causesphoticsideeffectsincludingglareorhaloesand reducedcontrastsensitivity,andtherefore,careful patientselectionandconsentareindicated.Extended depth-of-focusIOLsuseasingle,elongatedfocalpointto reducephoticsideeffects,thoughoftenattheexpenseof nearvisioncomparedwithmultifocalIOLs.Thisarticle willreviewrecentdevelopmentsinadvancedtechnology IOLssuchasmultifocal,accommodative,andextended depth-of-focusIOLsanddiscusstheirvisualoutcomes andsideeffects.
Introduction187 Significance188 AccommodativeLenses188 MultifocalLenses189
OpticalSideEffectsinMultifocalIntraocularLenses191 Extended-VisionIntraocularLens192 MonofocalIntraocularLenseswithIncreasedDepthofFocus193 LightAdjustableIntraocularLenses193 PhakicIntraocularLenses194 Discussion,futureconsiderations,andsummary194 Clinicscarepoints197
Videocontentaccompaniesthisarticleathttp://www. advancesinophthalmology.com
Macularholesarefovealneurosensorydisruptionsthat canimpairvisionandcausesymptomssuchas metamorphopsiaandscotomas.Stages1Aand1B shouldbeobserved.Stage2canbetreatedwithmultiple modalitiesincludingtopicaldehydrationagents, pneumaticvitreolysis,andsurgery.Surgeryremainsthe mostusedtreatment,becauseitcanpreventadditional visionlossfromprogressiontostages3and4.Surgeryis alsotherecommendedtreatmentofstages3and4. Invertedinternallimitingmembraneflaps,human amnioticmembraneplugs,mesenchymalstemcell injections,andautologousretinaltransplantsarenewer adjunctivetreatmentsthatofferencouragingresults.
Introduction201 Significance203 Nonsurgicaltreatment203 Surgery204 Presentrelevanceandfutureavenues211 Topicalagents211 Ocriplasmin212 Pneumaticvitreolysis212 Internallimitingmembranestaining213 Glialplugafterinternallimitingmembranemanipulation213 Intraoperativeopticalcoherencetomography213 Autologousmesenchymalstemcelltransplantation213 Autologousretinaltransplantation214 Summary214
Clinicalcarepoints216 Disclosure216 Supplementarydata216
PeripheralRetinalDegenerationsandTreatmentOptions GeorgeJiao,MaribAkanda,andRonniM.Lieberman
Therearenumerouspathologiesthatcanbefoundinthe peripheralretina,includingdegenerativedisease. Involvementofthevitreoretinalinterfaceandcertain intraretinaldegenerationscanleadtoretinaltearsor detachments.Thestandardofcarefordiagnosingand monitoringtheseperipheralretinaldegenerationsis dilatedfundusexamination,butcurrenttechnologies mayaiduswhenthediagnosisisunclear.Prophylactic treatmentsmaybewarrantedinmanycasesof predisposinglesions.Definitetreatmentsforretinaltears areimportantinpreventingretinaldetachments.
Introduction219
Benignlesions220
Peripheralretinaldrusen220
Paving-stone(cobblestone)degeneration220
Retinalpigmentepitheliumhyperplasia220
Retinalpigmentepitheliumhypertrophy221
Peripheralcystoiddegeneration222
Snowflakedegeneration222
Parsplanacysts222
Pearldegeneration222
White-with-pressureandwhite-without-pressure222
Lesionspredisposingtoretinaldetachment223
Latticedegeneration223
Senileretinoschisis224
Vitreoretinaltufts226
Meridionalfolds226
Enclosedorabays226
Peripheralretinalexcavations226
Chorioretinalatrophy227
Dark-without-pressure227
Snailtrackdegeneration227
RetinalTearsandHoles228
Dialysis228 Horseshoetear228
Operculatedroundtear229
Atrophicroundhole230
Treatmentoptionsforlatticeperipheraldegeneration231 Summary232 Clinicscarepoints232 Disclosure233
UpdateonRetinalandOcularImaging SamuelGelnick,MinhTrinh,andRonniM.Lieberman
Retinalimaginghasexperiencedrapidgrowthoverthe pastdecades.Anincreaseinresolution,fieldofview, andaccessibilityhasledtoanincreaseintheuseof retinalimaginginclinicalpracticeacrossallspecialtiesof ophthalmology.Understandingtheadvantagesand disadvantagesoftheseretinalimagingmodalitiesis crucialtoobtainingtheappropriatestudiestodiagnose andmonitorretinalpathologies.Inthisarticle,we reviewcommonimagingmodalities,includingtheir evolutionandclinicalapplications.
Backgroundandintroduction237 Opticalcoherencetomography238 Fundusphotography242 Artificialintelligence–deeplearning246 Adaptiveoptics247
Teleophthalmology248
Typesofteleophthalmology249
Diabeticscreening249
Retinopathyofprematurity249
Futuredirections249
Clinicscarepoints250
Disclosure250
Glaucoma
30-GaugeNeedleinGlaucoma/AnteriorSegmentManagement
RameshAyyala,AlirezaBaradaran-Rafii,andJacobLiechty
Videocontentaccompaniesthisarticleathttp://www. advancesinophthalmology.com
Manyclinicalpresentationsandcomplicationsof glaucoma,whetheracuteorchronic,includingbleb fibrosis,encapsulation,andsoforthalongwithanterior segmentsurgeriescanbemanagedattheslitlampwith thehelpofa30gauge(G)needle.Thisarticlepresents theindicationsandtechniquesinvolvedintheuseof 30Gneedleattheslitlampinthemanagementofa myriadofophthalmologicpresentationsandcomplications.
Introduction253
Preparationofthepatient254
Follow-upafterslitlampprocedures254
Indicationsandproceduredescription254
Blebneedlingrevisionforscarred,fibrosedblebs254
Blebneedlingrevisionforencapsulating/encapsulatedblebs followingAhmedglaucomavalveimplantation256
Paracentesistodecreaseintraocularpressure257
Anteriorchamberreformation257
Suturingattheslitlamp258
Sutureremoval258
Injections258
Ahmedglaucomavalveremoval259
Unpluggingsclerotomy259
Cornealprocedures259
DSEAK-relatedissuesand30Gneedle259
Vitreousstrandrelease260
Astigmatickeratotomy260
Summary261
Disclosure261
Supplementarydata261
Neuro-ophthalmology ScreeningforPlaquenil PushpinderKanda,StuartCoupland,ChloeGottlieb, LyncaKantungane,andRustumKaranjia
Hydroxychloroquineuseneedstobecarefullymonitored forretinaltoxicitythatcanleadtosignificantvisual impairment.Thisreviewsummarizesthecurrent screeningguidelinesandimagingmodalities recommendedbytheAmericanAcademyof Ophthalmologyandhighlightsfutureresearchtrendsfor improvingtestsensitivity.Italsodiscussestheutilityof usingmfERGfordetectingearlyretinopathyand researchsupportingitsuseasaprimaryscreeningtool.
Introduction263 Plaquenilscreeningmodalities266 Automatedvisualfields267 Spectral-domainopticalcoherencetomography270 Fundusautofluorescence270 Multifocalelectroretinogram271 Currenttrendsandfutureresearch273 Summary275 Clinicscarepoints276 Disclosure276
UpdateonOpticNeuritisinAdults:MultipleSclerosis, NeuromyelitisOpticaSpectrumDisorder,andMyelin OligodendrocyteGlycoproteinAntibody–AssociatedDisease
SerenaLi,RahulA.Sharma,andBensonS.Chen
Thisreviewprovidesanupdateonthediagnosisand managementofinflammatoryopticneuritisinadults, focusingonpresentationsassociatedwithmultiple sclerosis,neuromyelitisopticaspectrumdisorder,and myelinoligodendrocyteglycoproteinantibody–associated disease.Clinicalcluesthatenableearlyrecognitionof thesedistinctdisordersarediscussed,includingpatient demographics,clinicalfeatures,andradiologicfindings. Theuseofcorticosteroids,plasmapheresis,andother acutemanagementtreatmentstrategiesforopticneuritis areappraised,followedbyanoverviewoflong-term management,specificallystrategiesfor immunosuppressionincludingwhentoinitiateandwith whichtherapies.Finally,prognosisandfutureavenues areconsidered.
Introduction280
EfficacyandSafetyofTocilizumabintheTreatmentof OcularManifestationsinGiantCellArteritis
NooranBadeebandRustumKaranjia VisionlossinGCAisanophthalmicemergencythat requirespromptmedicalintervention.Thegoalofthe treatmentistopreservetheremainingvisionand preventfurthersystemiccomplications.Corticosteroids arethefirst-linetreatmentforGCAbutareproneto causeiatrogenicsideeffects.Inthisreview,wediscuss theroleofotherimmunosuppressantmedications, especiallyTocilizumabincontrollingcasesofGCAwith ocularinvolvementandreviewtheliteratureonthe indication,guidelinesofadministration,andsafetyofTocilizumab.
Y.GraceChung,ChristieM.Person,JacquelynO’Banion,and SusanA.Primo
Thecoronavirusdisease2019pandemichas disproportionatelyaffectedracialandethnicminoritiesin theUnitedStates.Althoughrecentmeta-analyseshave identifiedtheprevalenceofocularmanifestationsin severeacuterespiratorysyndromecoronavirus2 infection,nostudieswiththesepotentialfindingshave beenimplementedinexaminingophthalmicdisparitiesin racialandethnicminorities.Itisadditionallyclearthat patientaccesstoeyecarefromcoronavirusdisease2019 hasbeendisproportionateinunderservedcommunities. Largepublichospitalsandurbanacademicmedical centersprovideauniqueopportunitytofurtherstudy oculardiseasepresentationandhealthdisparitiesfrom coronavirusdisease2019inthesepopulations.
Introduction312 Significance313 COVID-19disproportionateimpact313 COVID-19relatedhealthcaredisparitiesinophthalmology313 COVID-19relatedophthalmicmanifestations314 Relevanceandfutureavenues318
CorneaandExternalDiseases StepwiseApproachtotheDiagnosisandManagementof DryEyeandOcularSurfaceDisease
JenniferB.Nadelmann,AngelaGupta,CarolineW.Chung,and StephenE.Orlin
Dryeyedisease(DED)isamultifactorialdisordercaused byabnormalitiesofthetearfilmthatcausesdamagetothe ocularsurface.Screeningtoolsarehelpfulinmakingthe diagnosisandindeterminingdiseaseseverity.Itis importanttotakeacarefulhistoryandtoevaluate meibomianglandphysiology,tearfilmquality, meibomianglandstatus,andtearproduction.Clinicians shouldtakeastepwiseapproachintreatingDED. Treatmentsvaryforevaporativedryeye,aqueous deficiency,and/orinflammatoryDED.Forpatientswith symptomsthathavenotimprovedfollowing conservativemeasures,prescriptionmedications,blood products,proceduresordevicescanbeconsidered.
Introduction325
UpdateontheClassificationandManagementof CornealDystrophies VivianL.Qin,DevinCohen,TomasAndersen,and StephenE.Orlin
ThemostrecentIC3Dclassification(2015)categorizesthe cornealdystrophiesbasedongenetic,clinical,and histologiccharacteristics.Herewereviewthe classificationofthecornealdystrophiesasepithelialand subepithelial,epithelial-stromal TGFB1,stromal,and endothelial.Wediscussthevarietyofavailablemedical andsurgicaltreatmentmodalitiesbasedon symptomatologyandanatomicareaofthecornea affected.Weexploretherecentadvancementsinthe areaofcornealregenerationwithouttheuseofdonor grafts,includinginjectionofculturedendothelialcells andgenetherapy.
Significance/in-depthanalysis347
EpithelialandSubepithelialDystrophies347
SurgicalManagementoftheAphakicEye TianyuLiu,BrianJ.Nguyen,SamanthaL.Marek,and StephenE.Orlin
Currentapproachestothesurgicalcorrectionofaphakia includeanteriorchamberintraocularlenses(IOLs),irisfixatedIOLs,sulcusIOLs,andscleral-fixatedIOLswith
orwithoutsutures.Anteriorchamberandiris-fixatedIOLs aretechnicallyeasiertoplacebutareassociatedwithrisks includingcornealdecompensation,uveitis,andglaucoma. SulcusplacementofIOLscanachievephysiologic positioningofthelensbutrequiresadequatecapsular support.Overall,currentapproachestocorrectionof aphakiahavecomparableefficacyandsafetyoutcomes, andtheadvantagesanddisadvantagesofeachtechnique shouldbeconsideredinlightofpatientandsurgeon factorsinsurgicalplanning.
Introduction367 Significanceandin-depthanalysisofthetopic368 Epikeratophakia368
Anteriorchamberintraocularlenses368 Iris-fixatedintraocularlenses369 Sulcusplacementofintraocularlenses370 Suturedscleral-fixatedintraocularlenses372 Suturelessscleral-fixatedintraocularlenses374 Currentrelevanceandfutureavenues376 MinimizingsutureerosionwithscleralpocketsandZ-sutures376 ModificationsoftheYamanesuturelessfixationtechnique378 Correctionofpediatricaphakia378 Summary379 Clinicscarepoints380 Disclosure380
Oculoplastics Teprotumumab:AMajorAdvanceintheTreatmentof ThyroidEyeDiseaseManagement
CarolineY.Yu,JamieA.Keen,andErinM.Shriver
Thyroideyedisease(TED)isanuncommonorbital inflammatorydiseasethatcanleadtodisfigurementand permanentvisionloss.Traditionaltherapiessuchas glucocorticoidsandorbitalradiationprimarilywereused totreatinflammationduringtheactivephaseofthe disease.Teprotumumab,thefirstFoodandDrug Administration-approvedmedicationforTED,targets theinsulin-likegrowthfactor-1receptorandhasbeen showntodecreaseproptosis,diplopia,andclinical activityscore.Inthisreview,thepathophysiologyand treatmentlandscapeofTEDaresummarized,andthe mechanism,clinicaldata,andassociatedadverseevents ofteprotumumabaredescribedindetail.
Introduction383
