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PediatricCataracts:OverviewAmericanAcademyofOphthalmology

ClinicalEducation / PediatricOphthalmologyEducationCenter / BrowseTopics

NOV11,2015

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ByM.EdwardWilson,MD
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Inchildren,cataractcausesmorevisualdisabilitythananyotherformoftreatableblindness.
Childrenwithuntreated,visuallysignificantcataractsfacealifetimeofblindnessattremendous
qualityoflifeandsocioeconomiccoststothechild,thefamily,andthesociety.Morethan200,000
childrenareblindfromunoperatedcataract,fromcomplicationsofcataractsurgery,orfromocular
anomaliesassociatedwithcataracts.1Manymorechildrensufferfrompartialcataractsthatmay
slowlyprogressovertime,increasingthevisualdifficultiesasthechildgrows.Thecumulativerisk
ofcataractduringthegrowingyearsisashighas1per1000.2
Themanagementofcataractsinchildhoodistediousandoftendifficult,requiringmanyvisitsover
manyyears.Successrequiresadedicatedteameffortthatofteninvolvesparents,primarycare
pediatricians,surgeons,anesthesiologists,technicians,orthoptists,lowvisionrehabilitation
specialists,andcommunityhealthworkers.

f ()
Cataractsinchildrencanbeclassifiedusinganumberofmethodsincludingageofonset,etiology,
andmorphology.

f
/f
Whilethepresenceoflensopacitiesatbirthindicatesacongenitalonset,thediagnosisand
recognitionofalensopacityatalateragedoesnotexcludeacongenitalonset.Itiscriticalto
provideadetaileddescriptionofthetypeoflensopacitiesbeforethecataractisextractedandinthe
operativenotesothetypecanbedeterminedandanylaterstudycorrelatinggeneticetiologyor
associatedsystemicdiseasecanbedonemoreaccurately.Somemorphologicalcategoriesof
cataractssuchasanteriorpolar,centralfetalnuclear,andposteriorpolarclearlyindicatea
congenitalonset,whileotherssuchascorticalorlamellarmaybeassociatedeitherwithalater
onsetorbecongenitalinnature.

q/Jv
Thiscategorycanbeconfusing.Strictlyspeaking,anacquiredcataractisonefromanexternal
cause,asopposedtooneinwhichthecauseisgeneticallydetermined,suchasamutationinone
ofthecrystallinegenes.However,somewoulduseacquiredtoindicateanonsetafterinfancy,
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whichdoesnotnecessarilyindicateanongeneticcause.Juvenilecataractsarebydefinitionthose
withanonsetinchildhood,afterinfancy,irrespectiveofunderlyingetiology.

Approximately50%ofchildhoodcataractsarecausedbymutationsingenesthatcodeforproteins
involvedinlensstructureorclarity.Table1listsgenesinwhichmutationscancausecataracts.3
Whilemanyofthesegenesaredominantlyinherited,othersareautosomalrecessiveorXlinked.
Drawingapedigreeandrecognizingsomecataractandocularphenotypesthatareassociatedwith
specificmutationswillhelpdeterminetheprobablemodeofinheritanceandthepossibleunderlying
syndrome.Recentadvancesingenetictesting,includingnextgenerationsequencing,allowthe
determinationoftheprecisegeneticcauseofisolatedcongenitalcataractsin75%ofindividual
familiesand63%ofthosewithsyndromiccongenitalcataracts.4Mutationsincrystallinsaccountfor
50%ofisolated(noassociatedsystemicabnormalities)cataracts,whilemutationsinthegap
junctionproteinconnexinsaccountfor25%ofcasesandmutationsingenesforheatshock
transcriptionfactor4,aquaporin0,andbeadedfilamentstructuralprotein2accountforthe
remaining25%.
Metabolicdisorderscancausecataracts,whichmayhaveparticularmorphologiesthatpointtothe
underlyingcause.Nextgenerationsequencingofgenesassociatedwithsyndromicormetabolic
cataractscanprovideaprecisediagnosisifthesystemicfindingsdonotallowrecognitionofthe
metabolicorsystemicillness.Table1summarizesfindingsinsomeofthemaindiseases
associatedwithacquiredsyndromiccataracts.
Traumaremainsamajorcauseofacquiredcataractsinchildren.Traumaticcataractsaremore
commoninboysandcanbetheresultofpenetratingorbluntinjuriestotheeye.Onehastobe
carefulinrulinginoroutthepresenceofanintraocularorintraorbitalforeignbody,hencethe
importanceofadetailedphysicalexaminationandofimagingstudiessuchasultrasonographyand
computedtomography.Magneticresonanceimaging(MRI)studiesarecontraindicatediftheforeign
bodyissuspectedtobemetallic.
Table1.Commoncausesofcongenitalorearlyacquiredcataracts3
Disease

Location

Gene

Phenotype

OMIMnumber

AUTOSOMALDOMINANT
Hyperferritinemiacataractsyndrome

19q13.33

FTL

Congenitalnuclearcataractand
elevatedserumferritin

600886

604307

CRYBB2

Dustyopacityofthefetalnucleus
withfrequentinvolvementofthe
zonularlens

1p36

Unknown

Centralandzonularcataract

115665

Zonularwithsuturalopacities

17q11.2

CRYBA1

Zonularcataractswithsutural
opacities

600881

Posteriorpolar1(CTPP1)

1p36.13

EPHA2

Opacitylocatedatbackoflens

116600

Posteriorpolar2(CTPP2)

11q23.1

CRYAB

Singlewelldefinedplaquein
posteriorpoleoflensbilateral

613763

2q33.3

CRYGC

22q11.23
Volkmanntypecongenitalcataract

Coppocklikecataracts

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Posteriorpolar3(CTPP3)

20q11.22

CHMP4B

Progressive,discshaped,posterior
605387
subcapsularopacity

Posteriorpole4(CTPP4)

10q24.32

PITX3

Singlewelldefinedplaquein
posteriorpoleoflens

Unknown

Matreflexofposteriorcapsulethat
progressesintowelldemarcated
610634
discinposteriorpole,forming
opaqueplaque

GJA8

Lenticularopacitieslocatedinthe
fetalnucleuswithscattered,fine,
diffusecorticalopacitiesand
incompletecortical'riders'

116200

601885

Posteriorpole5(CTPP5)

Zonularpulverulent1(CZP1)

14q22q23

1q21.2

610623

Zonularpulverulent3(CZP3)

13q12.11

GJA3

Centralpulverulentopacity
surroundedbysnowflakelike
opacitiesinanteriorandposterior
corticalregionsofthelens

Anteriorpolarcataract1

14q24qter

Unknown

Smallopacitiesonanteriorsurface
115650
oflens

Anteriorpolarcataract2

17q13

Unknown

Smallopacitiesonanteriorsurface
601202
oflens

Ceruleantype1(CCA1)

17q24

Unknown

Peripheralblueandwhiteopacities
115660
inconcentriccircles

Ceruleantype2(CCA2)

22q11.23

CRYBB2

Numerousperipheralblueflakes
andoccasionalspokelikecentral
opacities

601547

Ceruleantype3(CCA3)

2q33.3

CRYGD

Progressivebluedotopacities

608983

Crystallineaculeiformcataract

2q33.3

CRYGD

Needlelikecrystalsprojectingin
differentdirections,throughorclose 115700
totheaxialregionofthelens

Nonnuclearpolymorphiccongenital
cataract

2q33.3

CRYGD

Opacitiesbetweenthefetalnucleus
601286
andthecortexofthelens

Suturalcataractwithpunctateand
ceruleanopacities

22q11.23

CRYBB2

Dense,whiteopacificationaround
theanteriorandposteriorYsutures,
ovalpunctateandcerulean
607133
opacitiesofvarioussizesarranged
inlamellarform

Myotonicdystrophy1(DM1)

19q13.32

DMPK

Myotonia,musculardystrophy,
cataracts,hypogonadism,frontal
balding,andECGchanges

Polymorphicandlamellarcataracts

12q13.3

MIP

Cataract,autosomaldominant,multiple
3q22.1
types1

BFSP2

160900

Lamellar,sutural,polarandcortical 604219
opacities

Nuclearandsuturalopacities.

611597

AUTOSOMALRECESSIVE

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604168

Congenitalcataracts,facial
dysmorphism,andneuropathy
(CCFDN)

18q23

CTDP1

Congenitalcataracts,facial
dysmorphism,neuropathy,delayed
psychomotordevelopment,skeletal

anomalies,microcorneaand
hypogonadism

MarinescoSjgrensyndrome

Congenitalcataracts,cerebellar
ataxia,muscleweakness,delayed
psychomotordevelopment,short
248800
stature,hypergonadotrophic
hypogonadism,andskeletal
deformities

5q31.2

SIL1

2q21.3

RAB3GAP1

1q41

RAB3GAP2

Warburgmicrosyndrome3

10p12.1

RAB18

Martsolfsyndrome

1q41

RAB3GAP2

Mentalretardation,hypogonadism,
212720
microcephaly

GJA1

Brachycephaly,hypotrichosis,
microphthalmia,beakednose,skin
234100
atrophy,dentalanomalies,short
stature

RECQL4

Skinatrophy,telangiectasia,hyper
andhypopigmentation,congenital
skeletalabnormalities,premature 268400
aging,increasedriskofmalignant
disease

Warburgmicrosyndrome1

Warburgmicrosyndrome2

HallermannStreiffsyndrome(Francois
6q22.31
dyscephalicsyndrome)

RothmundThomsonsyndrome

8q24.3

600118
Microcephaly,microphthalmia,
microcornea,opticatrophy,cortical
dysplasia,inparticularcorpus
callosumhypoplasia,severe
614225
mentalretardation,spasticdiplegia,
andhypogonadism
614222

SmithLemliOpitzsyndrome

11q13.4

DHCR7

Microcephaly,mentalretardation,
hypotonia,,polydactyly,cleft
palate

Congenitalnuclearcataracts2

22q11.23

CRYBB3

Nuclearcataractwithcorticalriders 609741

270400

XLINKED
Norriedisease

NanceHoransyndrome

Xp11.3

Xp22.13

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NDP

Earlychildhoodblindness,mental
310600
disorder,sensorineuraldeafness

NHS

Maleshavedensenuclear
cataracts,microcornea,dental
abnormalities,anddevelopmental
302350
delay.Carrierfemaleshave
posteriorYsuturalcataractswith
smallcorneas
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XuLT,TraboulsiEI.Geneticsofcongenitalcataracts.In:WilsonME,TrivediRH,editors.Pediatric
CataractSurgery:Lippincott,WaltersKluwer2014.p.18.

UveitisCataractsdevelopinpatientswithuveitisasaresultofthechronicocularinflammationor
secondarytothechronicuseofsteroids.Surgeryforsuchcataractscanbecomplicatedbysevere
postoperativeinflammation,hencetheneedforabsenceofpreoperativeinflammationintheanterior
segmentoftheeyeandthepre,intra,andpostoperativeuseofvariouscombinationsoftopical,
subconjunctival,intracameral,andsometimessystemicsteroids.Manypatientswillhavea
pupillarymembranethatcoversthelensandattachestotheiris,makingsurgerymoredifficult.
Suchmembranescanbepeeledoffoftheanteriorlenscapsuleatthetimeofsurgerytofacilitate
lensremoval.Theuseofanintraocularlens(IOL)islefttothediscretionoftheindividualsurgeon.
Juvenileidiopathicarthritis:Oneofthemorecommoncausesofanterioruveitisin
children.Theuseofsystemicantimetabolitesinrecentyearshasledtobettercontrolof
uveitisinsuchpatientsandtoareductionintheincidenceofcataracts.
Othertypesofuveitiscanalsocausecataractseitherbecauseoftheinflammationorasa
complicationofsteroiduse.
IntraoculartumorsItisveryuncommonforcataractstodevelopasaconsequenceof
intraoculartumors.Thelensischaracteristicallyclearinpatientswithuntreatedretinoblastoma.
Treatmentsofthetumorsuchasradiotherapymayleadtothedevelopmentofcataracts,inwhich
casetimingofcataractremovalhastobeverycarefullyconsideredandsurgeryonlyperformed
whenalltumorintheeyehasbeeneradicated.Patientswithradiationcataractscanhave
significantocularsurfacedrynessandwillnottoleratecontactlenses,hencetheneedfor
intraocularlens(IOL)implantation.
ChronicretinaldetachmentThesecataractsareseeninthesettingofinjuriesorinassociation
withSticklersyndrome.Ifthelensistotallyopaque,preoperativeultrasonographyshouldbe
performedtoruleoutachronicretinaldetachment.Thepresenceofanafferentpupillarydefectisa
poorprognosticsign.
Maternalinfection(rubella)Thistypeofcataracthasnotbeenseenincountrieswhererubella
hasbeeneradicated,butcontinuestooccurinsomepartsoftheworld.

RadiationExternalbeamradiationisavoidedinpatientswithretinoblastoma.Theeyeis
typicallyshieldedifradiationisgiventothebrainorotherpartsoftheheadandneck.
Systemicsteroidsareveryrarecausesofcataractsinchildren.Inhaledsteroidsforasthma
donotcausecataracts.Thetypicalsteroidinducedcataractisposteriorsubcapsular.
VitrectomyAlargepercentageofchildrenwhoundergovitrectomydevelopcataracts.
Thesearemostlyposteriorsubcapsular.
LaserforretinopathyofprematurityCataractscandevelopfromthermalinjurytothe
lenswhenaprominenttunicavasculosalentisispresent.

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Asmentionedabove,itisimportanttoutilizetheappropriateterminologytodescribepediatric
cataracts.Themorphologycangiveacluetotheunderlyingetiology(isolatedorassociatedwith
systemicdisease),andpossiblytothevisualprognosisfollowingsurgery.

ff/
Thisisnotanuncommontypeofcongenitalcataract.Therearenospecificcausesofdiffuseor
totalcataracts.

AnteriorpolarTheopacityisinthecapsuleitselfandcanprotrudeintotheanteriorchamberas
asmallmammillation.Theremaybeanunderlyingcircularlayerofcorticalopacityslightlylarger
thanthewhitepolaropacity.Whilethemajorityarestableanddonotinterferewithvision,somecan
progressandrequiresurgicalremoval.Theycanbedominantlyinherited,especiallyinbilateral
cases.Unilateralcasescanbeassociatedwithanisometropia(astigmatismorhyperopia),whichif
leftuntreatedcancauseamblyopia,evenifthecataractitselfisnotvisuallysignificant.

Figure1.Anteriorpolarcataract.

PyramidalTheseareusuallylargerthanpolarcataractsandmorelikelytoprogresstovisual
significance.Theyaredifficulttoremovewithavitrectomyinstrumentandmayrequireexcisionand
removalwithforcepsbeforetherestofthelensisaspirated.

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Figure2.Pyramidalcataract.

AnteriorlenticonusThisreferstoathinnedoutcentralanteriorcapsulewithorwithoutanterior
corticalopacities.AnteriorlenticonusissaidtobecharacteristicofAlportsyndrome.Spontaneous
ruptureofthelenscanoccur,resultinginahydratedtotalcataract.

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Figure3.Anteriorlenticonus(CourtesyofK.DavidEpley).

m
Inthistypeofcataract,theopacificationisofalamella(anovoidlayerofcortex)thatcanbe
visualizedbetweenadjacentclearlamellae.Thesearefrequentlyassociatedwithradialrider
opacities.Familiallamellarcataractsaremostlyautosomaldominantandaregenerallyassociated
withagoodvisualprognosisaftertheirremoval.Theycanbestableormaybeassociatedwith
progressiveopacificationofinterveningcortex,necessitatingremoval.

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Figure4.Lamellarcataracts(Top:CourtesyofK.DavidEpley,MD.Bottom:CourtesyofFarukH.
rge,MD).

F
Theseopacitiesoccupythecentralmostpartofthelens.Theycanbedotlikeorcanbequite
dense.Theygenerallymeasure23.5mmandcanbeassociatedwithmicrophthalmia.Theyare
saidtobeassociatedwithahigherincidenceofpostoperativeglaucomabecauseofassociated
microphthalmiaandtheneedforsurgeryearlyininfancy.

Figure5.Congenitalnuclearcataract.

P p
Inthistypeofcataract,theopacityisinthecapsuleitself.Itisnecessarytodifferentiateposterior
polarfromposteriorsubcapsularcataracts.Posteriorpolarcataractsaregeneticallydetermined
andsomehavebeenassociatedwithmutationsinPITX3.
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Figure6.Posteriorpolarcataract.

P b ()
Inthisgroupofconditions,thecentralandsometimesparacentralposteriorcapsuleisthinand
bulgesposteriorly.Thisusuallyoccursatthelocationwherethehyaloidsystemattachestothe
eye.Thedistortioncancausealocalizedareaofextrememyopicrefraction.Theremayormaynot
besubcapsularcorticalopacification.Interferencewithvisioncanbetheresultofopticaldistortion
orofcapsularopacification.Mostcasesareunilateral,althoughbilateralandfamilialcaseshave
beenreported.Surgeryisassociatedwithgoodvisualoutcomesinmostcases.Spontaneous
ruptureofthelenscanrarelyoccur,leadingtoabruptprogressiontototalcataract.

Figure7.Posteriorlentiglobus(lenticonus)cataract.(A)Earlycleardefectincentralposterior
capsuleand(B)earlyopacificationofcentraldefect.(C)Ultrasoundbiomicroscopyofadvanced
posteriorlenticonus.

P bp

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P bp
Thesecanbecongenitalbutaremorecommonlyacquiredasaresultofinjuryorsteroiduse.The
opacitiesarecorticalanddonotinvolvethecapsuleproper.

Figure8.Posteriorsubcapsularcataract.

P f v (PFV) (v v f
p pp pm v)
ThelensopacitiesinpatientswithPFVaregenerallycapsularandcanbeassociatedwith
shrinkage,thickening,andvascularizationofthecapsule.Theremaybeaposteriorplaqueoutside
orinvolvingthelenscapsulewithaclearlensthatnonethelessmustbetreatedasacataract.

Figure9.Persistentfetalvasculature.

m p f
Inchildren,traumaticanteriorlenscapsulerupturequicklyresultsinahydratedwhitecataract.
However,inchildren,lenscortexintheanteriorchambermaybewelltoleratedwithoutan
intraocularpressure(IOP)rise.Cataractsurgerycanoftenbedelayedforafewdaysorupto3or
4weekstoallowthetraumaticiritistosubsidebeforethecataractandIOLsurgery.

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Figure10.Traumaticdisruptionoflens(CourtesyofK.DavidEpley).

v -p
f v
Visionscreeningismandatorytodetectcataractsassoonaspossible.Latedetectionmayresultin
poorvisualoutcomes.Allnewbornsmusthaveredreflexscreening,ideallyfollowedbyanotherred
reflexexaminationatthe68weekneonatalcheckup.Redreflextestingisdonebyusingdirect
ophthalmoscopefromadistanceof12feetinadarkenedroom.Preschoolvisionscreening(at3
and5years)isoftendoneinthecommunity.Photoscreenersareusedinpreverbalandverbal
children.Thesemayhelpthepediatriciansavetimeinscreening.Theyworkbyacomputer
analyzingtheredreflexforinequalityincolor,intensity,orclarity.Newscreenersutilizingpolarized
laserlightaremoreaccurateatdetectingdecreasedvision.Thepresenceofanyopacities,an
absentredreflex,orleukocoriashouldpromptanurgentreferraltoapediatricophthalmologist.

v b pm

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v b pm
Adetailedhistoryistakenthatincludesaskingaboutthechildsdevelopmentalmilestones,and
abouthealthproblemsinthesiblingsandparents.Visualassessmentisconductedbyusingage
appropriatetesting.Whenthechildistwomonthsold,visionassessmentcanbedonewithforced
preferentiallookingtechniques(eg,Telleracuitycards,Cardiffcards),fixationandfollowing
evaluation,andassessingobjectiontoocclusionofeacheye.Thepresenceorabsenceof
nystagmusisnoted.Subjectivevisualtesting(HOTVmatching,LEAsymbols,ortumblingEs)is
doneassoonasthechildisabletoplayamatchinggameoridentifythesymbolsandletters.
Thesetestscanusuallybedoneatage3yearsandabove.
Biomicroscopy(standardorportableslitlampexamination)iscompleted.Severityandmorphology
ofthecataractandanyassociatedabnormalitiesofcorneaoranteriorsegmentaredocumented.
Examinationofsiblingsandparentsmightindicateinheritedcataracts.Intraocularpressureis
checkedifpossible.
Ifthereisaviewoftheretina,fullretinalexaminationdocumentingopticnerves,retina,andfoveais
performed.Ifthereisnoview,ultrasonography(Bscan)iscarriedout.Ifthereistrauma,thenchild
abusemustberuledout.Inunilateralcataracts,laboratorytestsarenotneeded.
Forbilateralcataracts,ifthereisfamilyhistoryofchildhoodcataracts,thechildhasnoother
medicalproblems,andtheparentshavelensopacities,thensystemicandlaboratoryevaluations
arenotneeded.Ifthereisnofamilyhistoryofcataracts,apediatricsystemicevaluationisrequired
becausethesecataractsmaybeassociatedwithsystemicormetabolicdisease.Laboratorytests
mayalsobeneeded.Theophthalmologistoftenworksinconjunctionwithapediatricianand/ora
clinicalgeneticistwhendirectingthelaboratoryworkup.Aurinetestforreducingsugars,TORCH
(toxoplasmosis,rubella,cytomegalovirus,varicella)screening,aVenerealDiseaseResearch
Laboratory(VDRL)testforsyphilis,andabloodtestforcalcium,phosphorus,glucose,and
galactokinaselevelscanbechecked.
Mostinheritedcataractsareautosomaldominant.RecessiveandXlinkedcataractsareless
common.Genetictestingisarapidlyevolvingfield.Mutationsthatcausecongenitalcataractshave
beendiscoveredinover100genes.Usingthelatestsequencingtests,itwillbepossibletocheck
allgenesinvolvedincongenitalcataractsfromonebloodsample.Thismightleadtoquickerand
cheaperpersonalizedtreatmentandcounselingbythegeneticist.
Ifcataractsarelessthan3mmindiameterorareofpartialdensity,theymaybeobservedor
treatedwithdilatingdrops.Anydensecentralopacityinthelensofthreeormoremminayoung
childissignificantandrequiressurgery.Inadditiontothesizeofcataract,blackeningofthe
retinoscopicreflexisthemostimportantfactordeterminingneedforasurgery.5Inanolderchild,
anyopacitycausingadecreaseinqualityoflifeshouldbeconsideredforsurgery.Atthesame
time,thelossofaccommodationthatoccurswhenachildslensisremovedshouldbetakeninto
accountwhenmakingasurgicaldecision.Withincreasingage,visualdemandsofthechild
increaseandtheassessmentofwhetherapartialcataractisvisuallysignificanthastobe
constantlyrevisited.
Biometryisdonetogetkeratometrymeasurements,preferablywithoutaspeculum.Axiallengthis
oftenmeasuredinchildrenbyAscanultrasound,withtheimmersionmethodbeingmoreaccurate
thanthecontactmethod.6,7Often,thesemeasurementsarenotpossibleinclinicandexamination
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underanesthesiaisrequired.Ifthechildisolderandcooperative,andthecataractisnotvery
dense,thenopticalbiometryisdone.
ForcalculationoftheIOL,thirdgenerationtheoreticalformulae(eg,SRK/T,HolladayI&II,HofferQ
I&II,andHaigis)canbeused.Targetrefractionmaybeaimedforinitialhypermetropia(highor
low)oremmetropia.SuggestedtargetrefractionsforagearegiveninTable2.5Otherfactorssuch
asamblyopia,felloweyeconditionorrefraction,assumedcompliance,andparentalrefractiveerror
shouldalsobetakenintoconsiderationwheninterpretingthetable:oneIOLpowerchoiceforevery
agedoesnotworkforeverysituation.
Table2.Ageatcataractsurgeryandresidualrefraction
recommendationsfortargetrefraction
Ageatcataractsurgery Residualrefraction(Diopters)
<6months

+6to+10

612months

+4to+6

13years

+4

34

+3

46

+2to+3

68

+1to+2

>8

+1to0

pfm
Adultcataractsurgeryisamajoremphasisofresidencytrainingprogramsinophthalmology.The
skillsneededtoperformadultcataractsurgeryarealsoimportantforperformingpediatriccataract
surgery,butadditionalskillsareneededforthepediatricsurgery.Pediatriccataractsurgeryshould
onlybeperformedbyophthalmicsurgeonswhoperformthemonaweeklyorbiweeklybasisso
thattheycanperformthemwithahighlevelofcompetency.8Forthisreason,mostlargegroup
practicesassignonlyonesurgeonintheirpracticetoperformthesesurgeries.Whenpossible,
childrenshouldbereferredtoregionalcenterswherelargenumbersofpediatriccataractsurgeries
areperformed.Afterthepostoperativeperiod,inmostcasesthesechildrencanthenbefollowedon
alongtermbasisbyalocaldoctorandonlyreferredbacktotheregionalcenterifproblemsarise.
Pediatricophthalmologistsinterestedinperformingpediatriccataractsurgeryshouldpursue
fellowshiptrainingataninstitutionwheretheywillbetrainedhowtoperformpediatriccataract
surgery.Aftercompletingtheirfellowship,theyshouldtakeinstructionalcoursesasneededto
incorporatenewtechniquesastheyarise.Whileadultcataractsurgeonsareusuallyskillfulat
performingintraocularsurgery,theyoftenhavenotbeentaughtthespecialtechniquesrequiredto
successfullyperformpediatriccataractsurgery.Iftheyareinterestedinperformingpediatric
cataractsurgery,theyshouldseekoutopportunitiestolearnitsbestpracticeseitherby
observationorbytakinginstructionalcourses.

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m p
Inthe1960s,HubelandWiesel9introducedtheconceptofalatentperiodandacriticalperiod
forvisualdevelopment.Duringthelatentperiod,visualdeprivationhasnolastingeffectonvisionin
thedeprivedeye.Afterthelatentperiod,thereisacriticalperiodduringwhichvisualdeprivation
resultsinirreversiblevisionlossinthedeprivedeye.Thecriticalperiodforachildwithacataract
extendstoage910years.

Theoptimalageforperformingcataractsurgeryinachildwithaunilateralcongenitalcataractis
generallyagreedtobe6weeksofage.BirchandStager10evaluatedtherelationshipbetweenthe
ageatcataractsurgeryandvisualoutcomesinnewbornswithadenseunilateralcongenital
cataract.Themodelthatbestfittheirdatawasbilinear,withnodifferencesinthevisualoutcomesif
thesurgerywasperformedbetweenbirthandage6weeks.However,afterage6weeks,there
wasalineardeclineinvisualoutcomesrelatedtotheageatcataractsurgery.Theirmodelwould
suggestthatthereisa6weeklatentperiodfordenseunilateralcataractsinhumans.Morerecently,
Hartmannetal11foundthattheageatcataractsurgerywasonlyweaklyassociatedwithvisual
acuity.Whilethemedianvisualacuitywasbetteramongpatientswhohadcataractsurgery
betweenages4and6weeks,theassociationbetweenageatcataractsurgeryandthevisual
outcomewaslessrobustthanthedatareportedbyBirchandStager.

B
Itisgenerallyagreedthatbilateralcongenitalcataractsshouldberemovedby8weeksofageto
achievethebestvisualoutcomes.Lambertandcoworkers 12notedthatdelayingcataractsurgery
to10weeksofageorlaterincreasedthelikelihoodofa20/100orworsevisualoutcome.Birchand
coworkers 13reportedabilinearrelationshipbetweentheageofsurgeryandthevisualoutcomein
infantswithdensebilateralcongenitalcataracts.Betweenbirthand14weeksofagetheynoted
progressivelyworsevisualoutcomestheolderachildwasatthetimeofcataractsurgery.
However,afterage14weeksuntil31weeks,thevisualoutcomewasindependentofthechilds
ageatthetimeofcataractsurgery.Sinceitisunclearifthereisalatentperiodinchildrenwith
densebilateralcongenitalcataracts,thetimingofcataractsurgeryinthesechildrenisoften
determinedbyothercomorbiditiesandtheincreasedriskofglaucomaassociatedwithveryearly
cataractsurgery.

/ f
m f pvb
Densecataractsthatblocktheredreflexbeforethepupilsaredilatedandareassociatedwith
abnormalvisualbehaviorshouldberemovedduringinfancy.Othersignssuggestiveofvisually
significantcataractsarestrabismusinachildwithaunilateralcataractornystagmusinachildwith
bilateralcataracts.Incompletecataractsdonotalwaysrequirecataractsurgery.Ifthechildhas
incompletecataractsandnormalvisualbehaviorandthefundicanbeclearlyviewedwithan
ophthalmoscope,cataractsurgeryshouldbedeferred.Generally,posteriorlenticularopacitiesare
morevisuallysignificantthananteriorlensopacities.Iftheincompletecataract(s)isunilateralor
asymmetrical,parttimepatchingtherapyofthenormal/bettereyemaybebeneficialtoimproveor
maintainvisioninthemostaffectedeye.

V f
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Generally,cataractsurgeryshouldnotbeperformedonchildrenwithbilateralcataractswhohave
bestcorrectedvisualacuityof20/40orbetter.However,thevisualthresholdforperforming
cataractsurgeryshouldbetailoredtotheneedsofthechild.Forinstance,ifachildhasvisual
acuityworsethan20/40,butisdoingwellinschoolanddoesnothaveanyvisualbehavioral
problems,cataractsurgerycanbedeferreduntillater.Visualbehaviorislesshelpfulinassessing
theneedforcataractsurgeryinchildrenwithaunilateralcataract.Generally,ifbestcorrected
visualacuitycannotbeimprovedto20/50orbetterwithamblyopiatherapy,cataractsurgeryshould
beconsidered.

V f p-p f mm
Theimprovementinvisualacuityassociatedwithcataractsurgerymustbeweighedagainstthe
lossofaccommodationassociatedwithremovingthecrystallinelens.Whilemultifocalor
accommodativeIOLsareavailableforadultsandmaymitigate,somewhat,thelossof
accommodationassociatedwithcataractsurgery,theyareinfrequentlyimplantedingrowing
childrenbecauseoftherefractivechangesthatoccurasanimmatureeyegrows.Parentsshould
betoldthatwhiletheirchildmayseemoreclearlyafterundergoingcataractsurgery,thechildwill
havetowearbifocalsinordertooptimizedistanceandnearvision.

fm /p
Therisksandbenefitsofcataractsurgeryshouldbeclearlyoutlinedtoparents.Itisoftenhelpfulto
showthemmodelsoftheeyeorillustrationstohelpthemunderstandwhatacataractisandhow
cataractsurgerywillbeperformed.Theimportanceofamblyopiatherapyandopticalcorrection
followingcataractsurgeryshouldbediscussedindetail.TheprosandconsofimplantinganIOLor
creatingaposteriorcapsulotomyshouldbediscussedwithparents.Itshouldalsobeexplainedthat
theUSFoodandDrugAdministration(FDA)hasnotapprovedtheimplantationofIOLsinchildren,
andtheiruseinchildrenisofflabel.

mm q b f

Theoptionofperformingimmediatesequentialbilateralcataractsurgeryshouldbediscussedwith
theparentsofinfants,particularlyiftherearecomorbiditiesthatincreasetheriskofgeneral
anesthesia.Theyshouldbeinformedoftherisksandbenefitsassociatedwithimmediatesequential
bilateralcataractsurgery,includingthebenefitofadministeringonlyonegeneralanesthetic,butthe
increasedriskofbilateralendophthalmitis.14Itshouldalsobeexplainedthatprecautionswillbe
takentoreducetheriskofendophthalmitis,includingusingdifferenttraysofinstrumentsforeach
eye,disposablecannulas,redrapingbetweeneyes,andusingdifferentlotsofirrigatingsolution
andmedicationsforeacheye.

mm
Generalanesthesiaisrequiredtoperformpediatriccataractsurgery.Theanestheticagentsshould
beadministeredonlyunderthedirectsupervisionofananesthesiologistwithspecialexperienceor
specialtraininginpediatricanesthesia.Veryyoungchildren,especiallywhenbornprematurely,will
oftenneedtobehospitalizedovernightaftercataractsurgerybecauseoftheirincreasedriskof
experiencingapneaafterundergoinggeneralanesthesia.Cataractsurgerycanbeperformedasan
outpatientprocedureinolderchildren.

pv q

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pv q
Preoperativepreparationistypicallydoneusingpovidoneiodine.Theuseofintracameralantibiotics
ineithertheirrigatingsolutionorinjectedpostoperativelyhasbeenextensivelytestedinadults,and
whilenotwidelypracticedamongpediatriccataractsurgeons,trendsforecastmoreacceptancein
thecomingyears.
Surgicalincisionsareusuallydoneanteriorlythroughclearcorneaorusingascleraltunnel.Ifno
IOListobeplaced,aminorityofsurgeonswilloptforaposteriorparsplana/plicataapproach.
Continuouscurvilinearcapsulorhexiswithorwithoutcapsularstainingisthegoldstandard
capsulotomy,butvitrectorhexisalsoworkswellandiscommonlyusedinthefirstfewyearsofage
whenthecapsuleisveryelastic.Theanteriorchamberismaintainedwitheitheraseparatenon
heldinfusioncannula(ananteriorchambermaintainer)orwithmatchedhandheldbimanual
irrigationandaspirationhandpieces.Pupildilationisenhancedwithnonpreservedepinephrineor
phenylephrine/ketorolac(recentlyFDAapprovedforadults)addedtotheinfusionbottle.
Thelenscontentsareaspiratedcompletely(Figure11).Phacoemulsificationultrasoundenergyis
neverneededwithpediatriccataracts.Hydrodissectionisnotnecessary,butcanbeusedatthe
surgeonsdiscretion.However,thelargenumberofpediatriclensopacitiesassociatedwith
posteriorcapsulepathologymustbenoted.Hydrodissectioniscontraindicatedinposteriorpolar
cataracts.

Figure11.Anirrigation/aspirationhandpieceremovingalamellarcataract(CourtesyofFarukH
Orge).
AposteriorchamberIOLinsertedintothecapsularbagisalwayspreferred,butciliarysulcus
placementofafoldableacrylicorsinglepiecerigidIOLcanbedone.Incasesofnocapsular
support,posteriorchamberIOLscanbesewninplacehowever,placementofiris(claw)fixated
lensesisbecomingmorepopular.
InchildrentooyoungtotolerateaYAGlaserposteriorcapsulotomyintheoffice,aprimaryposterior
capsulotomyatthetimeofinitialcataractsurgeryisrecommended.Thiscanbedoneeitherbefore
orafteranIOLisplacedandcanbedoneanteriorlythroughthecornealtunnelorposteriorly
throughtheparsplana.Allbutthesmallestwatertightincisionsshouldbeclosedinchildren,usually
withasyntheticabsorbable100suture.

Ppv m

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Ppv m
b
Afterpediatriccataractsurgery,eithermoxifloxacinortobramycin,thetwomostwidelyused
antibioticeyedrops,canbeused.Theeyedropsareinstilledfourtimesperdayforaweek.There
isnoneedtoprescribesystemicantibiotics.

Prednisoloneeyedropsarethemainstayoftreatmenttocontrolsevereinflammation,whichis
generallyinevitable.Insomecasesofveryseverepostoperativeinflammation,steroideyedrops
mustbeinstilledasfrequentlyasonanhourlybasis.Otherwise,theroutinedosagerangeis48
timesperday.Somesurgeonsadvocatesupplementingthetopicalsteroidwithoralprednisolone
dosedat1mg/kg/dayforthefirstweektohelpreduceinflammation.

p m
Homatropineoratropineeyedropsaresometimesusedpostoperativelyascycloplegics.The
possiblesideeffectsofatropinemustbediscussedwiththepatientsparents.

F-p
Pediatriccataractcasesarenormallyexaminedonthefirstpostoperativeday.Thenextfollowup
dependsontheamountofinflammationbutismostoftenat1weekaftersurgery.Oncebotheyes
areoperatedon,periodicexaminationsarerequiredtodeterminerefraction,IOP,andretinal
evaluation.Glassesorcontactlensesareprescribedasearlyaspossible,preferablywithinthefirst
weekforaphakiccorrectionandwithin4weeksforresidualrefractiveerrorinpseudophakic
children.

Fq
Typicalfollowupfrequencyisasfollows:postoperativeday1,week1,month1,month3,every3
monthsfor2years,andthereafterevery6monthsfor3years.

v
Itiscrucialtocheckvisualacuity,ocularalignment,IOP,refraction,andclarityofthevisualaxisat
everyvisit.Shouldtherebeanycomplicationdetectedinanyofthefollowupvisits,itshouldbe
tackledpromptly.

p b f
Sinceuncorrectedrefractiveerrorintheearlyyearscanleadtoamblyopia,attentiontoappropriate
refractivecorrectionaftercataractsurgeryiscrucialinordertoobtaingoodfinalvisualacuity.For
infantsandtoddlers,refractivecorrectionshouldresultingoodnearvision(myopicrefractionof

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approximately2diopters).However,correctionfordistancevisionandabifocalcorrectionfornear
viewingshouldbeofferedaftertheageof2or3years,orbyprekindergarten.Childrenwhousea
contactlensmayalsobenefitfromaspectacleovercorrectionafterage2or3years.

p
ForchildrenwhohaveIOLimplantation,someresidualrefractiveerroristypicalandspectacle
correctionmaybeneededfordistanceand/ornearviewing.Additionally,whenIOLimplantation
occursatanearlyage,thegrowingeyewillexperienceamyopicshift,sothatchangingrefraction
isexpectedwithresidualhyperopiaintheearlyyearsbutsomedegreeofmyopiaexpectedlater.
CorrectionofaphakiawithspectaclesmaybepreferredforinfantsandyoungchildreninwhomIOL
implantationisnotpossibleorispurposelydelayed.Aphakicspectaclesaregenerallywell
tolerated,particularlybychildrenwhoarebilaterallyaphakic.Unilateralaphakiacanalsobe
correctedwithspectacles,thoughthisislessdesirablebecauseofmarkedimagesizedisparity
(aniseikonia)andpotentialdisruptionofbinocularvision,ifpresent.


Contactlenscorrectionofaphakiaisoftenplannedforveryyounginfantsafterlensectomy,
typicallywitheitherasiliconeelastomerlens(extendedwear)orrigidgaspermeablelens(daily
wear).Oneadvantageofcontactlensweariseasyadjustmentinpowerfortherapidlychanging
refractionsencounteredinyoungchildren.Contactlenscorrectionofresidualrefractiveerroris
alsopossibleafterIOLimplantation,andissometimesrequestedbyadolescentpatients.

Ppv mp q
Postoperativecomplicationsafterpediatriccataractsurgeryareinverselyproportionaltotheageat
thetimeofsurgery.Associatedocularanomalies,surgicaltechnique,andfollowupdurationare
someoftheotherimportantvariablesinfluencingtheprevalenceandseverityofthepostoperative
complicationsaftercataractsurgeryinchildren.

V x pf
Iftheposteriorcapsuleisleftintactatthetimeofcataractsurgeryinchildren,posteriorcapsule
opacification(PCO)isinevitable.Theyoungerthechild,themoreacutewillbetheopacity.After
primaryposteriorcapsulectomyandvitrectomy,visualaxisopacification(VAO)israreinolder
childrenhowever,despiteposteriorcapsulectomyandvitrectomy,VAOiscommonlyobservedin
infants.VAOininfantsreceivingposteriorcapsulectomyandvitrectomytypicallyrequiressurgical
removalfrom3monthsto1yearaftertheoriginalsurgery,whilePCOinolderchildrenwhohadan
intactposteriorcapsuletypicallyrequiresNd:YAGlaserorsurgicalremovalofthePCO2yearsor
moreaftercataractsurgery.15

m
Secondaryglaucomaisthemostsightthreateningcomplicationofpediatriccataractsurgery.
Youngerageatthetimeofsurgeryisthemostcommonlyreportedriskfactor.Openangle
glaucomacandevelopmonthstomanyyearsafterthesurgery,andchildrenmustbefollowedfor
thisregularlyfortheirentirelife.

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fmm mp
Duetoincreasedtissuereactivity,inflammatorycomplications(eg,anteriorchambercellandflare,
celldepositsontheIOLoptic,posteriorsynechiae,etc.)aremorefrequentlyobservedinchildren.
Toxicanteriorsegmentsyndrome(TASS)isarareinflammatoryconditionusuallyobservedduring
theearlypostoperativeperiod.


Bacterialkeratitis,cornealopacityduetotightcontactlenses,andcornealvascularizationarethe
mostcommoncontactlensrelatedcomplications.

mp
ExcessivecapsularfibrosisandasymmetricIOLfixationarethemostcommoncausesleadingto
malpositionofanIOL.Itcanalsooccurbecauseoftraumaticzonularlossand/orinadequate
capsularsupport.TheIOLmayhavetoberepositionedorexplantedinsomecaseswhenthereis
significantdecentration/dislocation.

pm
Theincidenceofpostoperativeendophthalmitisinchildrenissimilartothatreportedinadult
surgery.CommonorganismsareStaphylococcusaureus,Staphylococcusepidermidis,and
Streptococcusviridans.Recentstudiesinadultshavereportedamarkeddecreasein
endophthalmitiswhenintracameralantibioticsareused.IntheUS,theabsenceofanophthalmic
preparationspecificforuseasanintracameralinjectionhasslowedadoptionofintracameral
antibioticsforfearoftoxicityfromdilutionerrorsduringmedicationpreparation.Studiesinadults
haveusedcefuroxime,vancomycin,andundilutedmoxifloxacin.16,17,18,19,20

m
Theincidenceofretinaldetachment(RD)followingpediatriccataractsurgeryappearstohave
decreasedmarkedlyassurgicaltechniqueshaveadvanced.However,becauseRDmaydevelop
manyyearsaftersurgery,aretinalexaminationisrecommendedaftercataractsurgeryatleast
yearly.ThisisespeciallyimportantforthoseeyesathigherriskforRDbyvirtueofalongaxial
lengthforage,persistentfetalvasculature,traumaticcataract,ectopialentis,Sticklersyndrome,
repeatedsurgeries,etc.

Mp f
Atendencytowardaxialelongationandamyopicshiftofrefractioniswellknown.Thisismore
concerningifthechildreceivesanIOL.Theyoungerthechildatthetimeofimplantation,thehigher
themyopicshift.Highmyopiainpseudophakiceyescanbetreatedusingspectaclesorcontact
lens.Alternatively,IOLexchange,piggybackIOLimplantation,orcornealrefractivesurgerymaybe
required.

mp

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Cornealedema,cornealdecompensation,irisprolapse,heterochromiairidis,suturerelated
complications,apostoperativeIOPspike,astigmatism,ptosis,orphthisisbulbiareother
complicationsreportedafterpediatriccataractsurgery.

bm
Strabismuscancoincidewithcongenitalcataractandismorecommonlyseeninunilateralcases
butnotrareinbilateralcataractcases,especiallywhennystagmusispresent.Esotropiaisthe
mostcommonformofstrabismusincongenitalcataract,althoughcycloverticalstrabismusmay
alsocontributetotheclinicalpicture.Inaminorityofpatients,exotropiaoftheinvolvedeyeisthe
presentingsignofcongenitalcataract.

Mm f -x mbp
Deprivationamblyopiaisverycommoninchildrenwithunilateralcataract,especiallywhenthe
opacityiscongenitalorinfantile.Also,childrenwithbilateralcataractscandevelopunilateralor
bilateraldeprivationamblyopiawhenthecataractsareasymmetric,whentheyareremovedtoo
late,orwhentheaphakiaisnotproperlycorrected.Sensorynystagmuswillfurtherlimitvisual
outcome.Themanagementoftheamblyopiashouldstartassoonaspossible,sincecompliancein
smallinfantsisbetterthanin2to3yearoldchildren.Patchingofthesoundeyeisthemainstayof
treatment.However,atropinepenalizationcanbeanalternativeiftheamblyopiceyecantakeover
fixation.Thisisquiterarebecausetheaphakicorpseudophakiceyehaslostaccommodationand
forthatreasonisalwaysatadisadvantagetothesoundeye,whichcanaccommodateupto10
dioptersdependingonthechildsage.Inbilateralaphakiceyeswithcontactlenses,thecontact
lensofthedominanteyecanberemovedafewhoursorseveraldaysperweekasapenalization
strategy.Theyoungerthechild,thebettertheeffectofamblyopiatreatmentperhourofocclusion.

v b q f f
m
Incaseswhenthetreatmentofthecongenitalcataractislesssuccessful,lowvisionrehabilitation
hasanimportantroleinhowthepatientcancopewiththelimitedvisualcapacitiesineducationand
dailylife.Inmostcountries,visualrehabilitationandeducationforvisuallyimpairedandblind
patientsareorganizedeitherbythegovernment,variousnongovernmentalorganizations,orprivate
foundations.Themottoshouldbe:Usetheremainingvisualfunctionwithallothersensesto
achievetheoptimumqualityoflife.

F
Earlydetectionwillallowmoretimelytreatmentofpediatriccataractinthefuture.Visionscreening
programsandimprovededucationofprimaryhealthcareworkersandthepublicwillhelpwiththis
evolution.Surgicaltechniquescontinuetoimproveandwillallowchildhoodcataractremovalwith
lessandlesssurgicaltrauma.PlanningforIOLimplantationwillbecomeeasierasourknowledgeof
myopicshiftandaxialglobegrowthevolve.Ultimately,futureIOLtechnologicaladvanceswillbe
aimedatrestorationorpreservationofyouthfulaccommodationandtheabilitytoeasilycompensate
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fortheinevitablemyopicshift.Intracameralmedicationsspecificallyforophthalmicusearebeing
developedandthesewillimproveoutcomesforchildrenastheydecreasethereliancewenow
haveontheabilityofparentstoadministertopicalmedicationsaftersurgery.

f
1. GilbertC.Worldwidecausesofblindnessinchildren.In:WilsonME,SaundersRA,Trivedi
RH,eds.PediatricOphthalmology:CurrentThoughtandaPracticalGuide.Heidelberg,
Germany:Springer2009:4760.
2. HaargaardB,WohlfahrtJ,FledeliusHC,RosenbergT,MelbyeM.Incidenceandcumulative
riskofchildhoodcataractinacohortof2.6millionDanishchildren.InvestOphthalmolVis
Sci.200445(5):13161320.
3. XuLT,TraboulsiEI.Geneticsofcongenitalcataracts.In:WilsonME,TrivediRH,editors.
PediatricCataractSurgery:Techniques,ComplicationsandManagement.Philadelphia:
LippincottWilliams&Wilkins2014:18.
4. GillespieRL,O'SullivanJ,AshworthJ,BhaskarS,WilliamsS,BiswasS,etal.Personalized
diagnosisandmanagementofcongenitalcataractbynextgenerationsequencing.
Ophthalmology.2014121(11):21242137e12.
5. SerafinoM,TrivediRH,LevinAV,WilsonME,NucciP,LambertSR,etal.UseoftheDelphi
processinpaediatriccataractmanagement.BrJOphthalmol.2015.doi:
10.1136/bjophthalmol2015307287.[Epubaheadofprint].
6. TrivediRH,WilsonME.Predictionerrorafterpediatriccataractsurgerywithintraocularlens
implantation:ContactversusimmersionAscanbiometry.JCataractRefractSurg.
201137(3):501505.
7. TrivediRH,WilsonME.Axiallengthmeasurementsbycontactandimmersiontechniquesin
pediatriceyeswithcataract.Ophthalmology.2011118(3):498502.
8. BellCM,HatchWV,CernatG,UrbachDR.Surgeonvolumesandselectedpatientoutcomes
incataractsurgery:apopulationbasedanalysis.Ophthalmology.2007114(3):405410.
9. HubelDH,WieselTN.Theperiodofsusceptibilitytothephysiologicaleffectsofunilateraleye
closureinkittens.JPhysiol.1970206(2):419436.
10. BirchEE,StagerDR.Thecriticalperiodforsurgicaltreatmentofdensecongenitalunilateral
cataract.InvestOphthalmolVisSci.199637(8):15321538.
11. HartmannEE,LynnMJ,LambertSR,InfantAphakiaTreatmentStudyGroup.Baseline
characteristicsoftheinfantaphakiatreatmentstudypopulation:predictingrecognitionacuity
at4.5yearsofage.InvestOphthalmolVisSci.201456(1):388395.
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periodforthesurgicaltreatmentofchildrenwithdensebilateralcongenitalcataracts?J
AAPOS.200610(1):3036.
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surgicaltreatmentofdensecongenitalbilateralcataracts.JAAPOS.200813:6771.
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surgeryforinfantswithcongenitalcataracts:Visualoutcomes,adverseevents,and
economiccosts.ArchOphthalmol.2010128(8):10501054.
15. WilsonME,Jr.,TrivediRH,BuckleyEG,GranetDB,LambertSR,PlagerDA,etal.ASCRS
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200733(11):19661973.
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16. BragaMeleR,ChangDF,HendersonBA,MamalisN,TalleyRostovA,VasavadaA.
ASCRSClinicalCataractCommittee.Intracameralantibiotics:Safety,efficacy,and
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