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Chapter11
Retina
11.1PosteriorVitreousDetachment
PosteriorVitreousDetachment
Symptoms
Floaters(ucobwebs,““bugs,"or"spots”thatchangepositionwith
eyemovement),blurredvision,flashesoflight,whicharemorecommon
indimilluminationandaretemporallylocated.
Signs
Critical.Oneormorediscretelightgraytoblackvitreousopacities,
oneoftenintheshapeofaring("Weissring")orbrokenring,suspended
overtheopticdisc.SeeFigure11.1.1.
Other.Retinalbreak,retinaldetachment,orvitreoushemorrhagemay
occurwithorwithoutaposteriorvitreousdetachment(PVD),withsimilar
symptoms.Peripheralretinalanddiscmarginhemorrhages,pigmentedcells
intheanteriorvitreous[releasedretinalpigmentepithelium(RPE)cells
or“tobaccodust”].
Figure11.1.1.PosteriorvitreousdetachmentwithoutWeissring.
Note
Approximately8%to10%ofallpatientswithacutesymptomaticPVDhave
aretinalbreak.Thepresenceofpigmentedcellsintheanteriorvitreous
orvitreoushemorrhageinassociationwithanacutePVDindicatesahigh
probabilityofacoexistingretinalbreak(>70%).See11.2,RetinalBreak.
DifferentialDiagnosis
?Vitritis:ItmaybedifficulttodistinguishPVDwithanterior
vitreouspigmentedcellsfrominflammatorycells.Invitritis,
vitreouscellsmaybefoundinboththeposteriorandanterior
vitreous,theconditionmaybebilateral,andthecellsarenot
typicallypigmented.See12.3,PosteriorUveitis.
?Migraine:Multicoloredphotopsiasinazig-zagpatternthat
obstructvision,lastapproximately20minutes.Aheadachemayor
maynotfollow.Normalfundusexamination.See10.27,Migraine.
Work-Up
?History:Distinguishretinalphotopsiasfromthevisualdistortion
ofmigraine,whichmaybeaccompaniedbynewfloaters.Durationof
thesymptoms?Riskfactorsforretinalbreak(previousintraocular
surgery,highmyopia,familyhistoryofretinaltearsand/or
detachments,darkcurtaininvision)?
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?Completeocularexamination,includingexaminationoftheanterior
vitreousforpigmentedcellsandadilatedretinalexaminationwith
indirectophthalmoscopyandscleraldepressiontoruleouta
retinalbreakanddetachment.
?VisualizethePVDattheslitlampwitha60-or90-diopterlens
byidentifyingagray-to-blackstrandsuspendedinthevitreous.
Ifnotvisible,havethepatientlookup,down,andthenstraight
tofloatthePVDintoview.
?Ifavitreoushemorrhageobscuresvisualizationoftheretina,
u11rasonography(US)isindicatedtoidentifythePVDandruleout
aretinaldetachment(RD),tumor,orhemorrhagicmacular
degeneration.Occasionally,theflapofatearcanbeidentified.
See11.13,VitreousHemorrhage.
Treatment
NotreatmentisindicatedforPVD.Ifanacuteretinalbreakisfound,
see11.2,RetinalBreak.
Note
Aretinalbreaksurroundedbypigmentisoldandusuallydoesnotrequire
treatment.
Follow-Up
?ThepatientshouldbegivenalistofRDsymptoms(anincreasein
floatersorflashinglights,ortheappearanceofapersistent
curtainorshadowanywhereinthefieldofvision)andtoldtoreturn
immediatelyifthesesymptomsdevelop.
?Ifnoretinalbreakorhemorrhageisfound,thepatientshouldbe
scheduledforrepeatedexaminationwithscleraldepressionin2to
4weeks,2to3months,and6monthsafterthesymptomsfirst
develop.
?Ifnoretinalbreakisfound,butmildvitreoushemorrhageor
peripheralpunctateretinalhemorrhagesarepresent,repeated
examinationsareperformed1to2weeks,4weeks,3months,and6
monthsaftertheevent.
?Ifnoretinalbreakisfoundbutsignificantvitreoushemorrhage
oranteriorpigmentedvitreouscellsarepresent,repeat
examinationshouldbeperformedthenextdaybyaretinaspecialist
becauseofthehighlikelihoodofaretinalbreak.
11.2RetinalBreak
RetinalBreak
Symptoms
Acuteretinalbreak:Flashesoflight,floaters("cobwebs”or“spots”
thatchangepositionwitheyemovement),andsometimesblurredvision.
CanbeidenticaltosymptomsassociatedwithPVD.
Chronicretinalbreaksoratrophicretinalholes:Usuallyasymptomatic.
Signs
(SeeFigure11.2.1.)
Critical.Afull-thicknessretinaldefect,usuallyseenintheperiphery.
Other.Acuteretinalbreak:Pigmentedcellsintheanteriorvitreous,
vitreoushemorrhage,PVD,retinalflap,subretinalfluid,oranoperculum
(afree-floatingpieceofretina)suspendedinthevitreouscavityabove
theretinalhole.
Chronicretinalbreak:Asurroundingringofpigmentation,ademarcation
linebetweenattachedanddetachedretina,andsigns(butnosymptoms)
ofanacuteretinalbreak.
PredisposingConditions
Latticedegeneration,highmyopia,aphakia,pseudophakia,age-related
retinoschisis,vitreoretinaltufts,meridionalfolds,historyof
previousretinalbreakordetachmentinthefelloweye,trauma.
DifferentialDiagnosis
?Meridionalfold:Smallradialfoldofretinaperpendiculartothe
oraserrataandoverlyinganoraltooth;mayhavesmallretinalhole
atthebase.
?Meridionalcomplex:Meridionalfoldthatextendstoaciliary
process.
?Vitreoretinaltuft:Focalareaofvitreoustractioncausing
elevationoftheretina.
P.276
Figure11.2.1.Giantretinaltear.
?Pavingstonedegeneration.
?Latticedegeneration.
Work-Up
Completeocularexaminationwithaslit-lampandindirectophthalmoscopy
ofbotheyeswithscleraldepression.Scleraldepressionisdeferreduntil
2to4weeksafteratraumatichyphemaormicrohyphema.
Treatment
Ingeneral,lasertherapyorcryotherapyisrequiredwithin24to72hours
foracuteretinalbreaks,andonlyrarelyforchronicbreaks.Eachcase
mustbeindividualized;however,wefollowthesegeneralguidelines:
?Treatmentrecommended
o-Acutesymptomaticbreak(e.g.,ahorseshoeoroperculated
tear).
o一Acutetraumaticbreak(includingadialysis).
?Treatmenttobeconsidered
o一Asymptomaticretinalbreakthatislarge(e.g.,>1.5mm)
orabovethehorizontalmeridianorboth,particularlyif
thereisnoPVD.
o一Asymptomaticretinalbreakinanaphakicorpseudophakic
eye,aneyeinwhichtheinvolvedorthecontralateraleye
hashadanRD,orinahighlymyopiceye.
Follow-Up
?Patientswithpredisposingconditionsorretinalbreaksthatdonot
requiretreatmentarefollowedevery6to12months.
?Patientstreatedforaretinalbreakarereexaminedin1week,1
month,3months,andthenevery6to12months.
?RDsymptoms(anincreaseinfloatersorflashinglightsorthe
appearanceofacurtain,shadow,orbubbleanywhereinthefield
ofvision)areexplainedandpatientsaretoldtoreturnimmediately
ifthesesymptomsdevelop.
11.3RetinalDetachment
Therearethreedistincttypesofretinaldetachment(RD).Allthreeforms
showanelevationoftheretina.
RhegmatogenousRetinalDetachment
Symptoms
Flashesoflight,floaters,acurtainorshadowmovingoverthefieldof
vision,peripheralorcentralvisualloss,orboth.
Signs
(SeeFigures11.3.1,11.3.2,and11.3.3.)
Critical.ElevationoftheretinafromtheRPEbyfluidinthesubretinal
spaceduetoanaccompanyingfull-thicknessretinalbreakorbreaks.See
11.2,RetinalBreak.
Other.Anteriorvitreouspigmentedcells,vitreoushemorrhage,PVD,
usuallylowerIOPintheaffectedeye,nonshiftingclearsubretinalfluid,
sometimesfixedretinalfolds.The
P.277
detachedretinaisoftencorrugatedandpartiallyopaqueinappearance.
AmildRAPDmaybepresent.
Figure11.3.1.Rhegamatogenousretinaldetachment.
Note
Achronicrhegmatogenousretinaldetachment(RRD)oftenshowsapigmented
demarcationlineattheposteriorextentoftheRD,intraretinalcysts,
fixedfolds,orwhitedotsunderneaththeretina(subretinalprecipitates)
oracombinationofthese.Itshouldbedifferentiatedfromretinoschisis,
whichproducesanabsolutevisualfielddefect.
Figure11.3.2.Retinaldetachmentwithretinalbreakinlattice
degeneration.
Figure11.3.3.B-scanUSofretinaldetachment.
Etiology
Aretinalbreakallowsfluidtomovethroughtheholeandseparatethe
overlyingretinafromtheRPE.
Work-Up
?Indirectophthalmoscopywithscleraldepression.Slit-lamp
examinationwithcontactlensmayhelpinfindingsmallbreaks.
?B-scanUSmaybehelpfulifmediaopacitiesarepresent.
ExudativeRetinalDetachment
Symptoms
Minimaltoseverevisuallossoravisualfielddefect;visualchanges
mayvarywithchangesinheadposition.
Signs
Critical.Serouselevationoftheretinawithshiftingsubretinalfluid.
Theareaofdetachedretinachangeswhenthepatientchangesposition:
Whilesitting,thesubretinalfluidaccumulatesinferiorly,detachingthe
inferiorretina;whileinthesupineposition,thefluidaccumulatesin
theposteriorpole,detachingthemacula.Thereisnoretinalbreak;fluid
accumulationis
P.278
duetobreakdownofthenormalinnerorouterblood-retinalbarrier.The
detachmentdoesnotextendtotheoraserrata.
Other.Thedetachedretinaissmoothandmaybecomequitebullous.Amild
RAPDmaybepresent.
Etiology
?Neoplastic:Choroidalmalignantmelanoma(MM),metastasis,
choroidalhemangioma,multiplemyeloma,capillaryretinal
hemangioma,etc.
?Inflammatorydisease:Vogt-Koyanagi-Harada(VKH)syndrome,
posteriorscleritis,sympatheticophthalmia,otherchronic
inflammatoryprocesses.
?Congenitalabnormalities:Opticpit,morning-glorysyndrome,and
choroidalcoloboma(althoughtheseusuallyhavearetinalbreak).
?Vascular:Coatsdisease,malignanthypertension,andpreeclampsia.
?Nanophthalmos:Smalleyeswithasmallcorneaandashallowanterior
chamberbutalargelensandathicksclera.
?Idiopathiccentralserouschorioretinopathy(CSCR):Maybeseen
withbullousRDfrommultiple,largeRPEdetachments.See11.15,
CentralSerousChorioretinopathy.
?Uvealeffusionsyndrome:Bilateraldetachmentsoftheperipheral
choroid,ciliarybody,andretina;leopard-spotRPEchanges(when
retinaisreattached);cellsinthevitreous;dilatedepiscleral
vessels.
Work-Up
?Intravenousfluoresceinangiography(IVFA)mayshowsourceof
subretinalfluid.
?B-scanUSmayhelpdelineatetheunderlyingcause.
TractionalRetinalDetachment
Symptoms
Visuallossorvisualfielddefect;maybeasymptomatic.
Signs
Critical.Thedetachedretinaappearsconcavewithasmoothsurface;
cellularandvitreousmembranesexertingtractionontheretinaare
present;retinalstriaeextendingfromtheseareasmayalsobeseen.
DetachmentmaybecomeaconvexRRDifatractionalretinalteardevelops.
Other.Theretinaisimmobile,andthedetachmentrarelyextendstothe
oraserrata.AmildRAPDmaybepresent.
Etiology
Fibrocellularbandsinthevitreous(e.g.,resultingfromproliferative
diabeticretinopathy,sicklecellretinopathy,retinopathyof
prematurity,familialexudativevitreoretinopathy(FEVR),toxocariasis,
trauma,previousgiantretinaltear)contractanddetachtheretina.
Work-Up
?Indirectophthalmoscopywithscleraldepression.Slit-lamp
examinationwithcontactlensmayhelpinfindingsmallbreaks.
?B-scanUSmaybehelpfulifmediaopacitiesarepresent.
DifferentialDiagnosisForAllThreeTypesofRetinalDetachment
?Acquired/age-relateddegenerativeretinoschisis:Commonly
bilateral,usuallyinferotemporal,nopigmentedcellsor
hemorrhagearepresentinthevitreous,theretinalvesselsinthe
innerretinallayersareoftensheathedperipherally,white
“snowflakes”areoftenseenontheinnerretinallayers.See11.4,
Retinoschisis.
?X-linkedretinoschisis:Petaloidfovealchangesarepresentover
90%ofthetime.Dehiscencesoccurinthenervefiberlayer50%of
thetime.See11.4,Retinoschisis.
?Choroidaldetachment:Orange-brown,moresolidinappearancethan
anRD,oftenextends360degrees.Hypotonyisusuallypresent.See
11.27,ChoroidalEffusion/Detachment.
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Treatment
?PatientswithanacuteRRDthatthreatensthefoveashouldbeplaced
onbedrestuntilsurgicalrepairisperformedurgently.Surgical
optionsincludelaserphotocoagulation,cryotherapy,pneumatic
retinopexy,vitrectomy,andscleralbuckle.
?AllRRDsthatdonotthreatenfixationoraremacula-off,or
tractionalretinaldetachments(TRDs)thatinvolvethemaculaare
repairedpreferablywithinafewdays.Visualoutcomesfor
macula-offdetachmentsdonotchangeifsurgeryisperformedwithin
10daysoftheonset.
?ChronicRDsaretreatedwithin1week.
?Forexudativeretinaldetachment,successfultreatmentofthe
underlyingconditionoftenleadstoresolutionofthedetachment.
Follow-Up
PatientstreatedforRDarereexaminedat1day,1week,2weeks,1month,
2to3months,thenevery6to12months.
11.4Retinoschisis
Retinoschisis,asplittingoftheretina,occursinX_1inked(juvenile)
andage-relateddegenerativeforms.
X-Linked(Juvenile)Retinoschisis
Symptoms
Decreasedvisionduetovitreoushemorrhage(25%)andmacularchanges,
orasymptomatic.Theconditioniscongenital,butmaynotbedetectedat
birthifanexaminationisnotperformed.Afamilyhistorymayormaynot
beelicited(X-linkedrecessive).
Signs
(SeeFigure11.4.1.)
Critical.Fovealschisisseenasstellatemaculopathy:Cystoidfoveal
changeswithretinalfoldsthatradiatefromthecenterofthefoveal
configuration(petaloidpattern).Unlikethecystsofcystoidmacular
edema(CME),theydonotstainorleakonintravenousfluorescein
angiography(IVFA),butcanbeseenwithindocyaninegreen(ICG).The
macularappearancechangesinadulthood.
Other.Separationofthenervefiberlayerfromtheouterretinallayers
intheretinalperiphery(bilaterallyintheinferotemporalquadrant,
mostcommonly)withthedevelopmentofnervefiberlayerbreaks;this
peripheralretinoschisisoccursin50%ofpatients.However,schisismay
occurbetweenanytworetinallayers.RD,vitreoushemorrhage,and
pigmentarychangesalsomayoccur.Pigmenteddemarcationlinesmaybeseen
eventhoughtheretinaisnotdetachedatthetime,unlikeacquired
age-relateddegenerativeretinoschisis.
DifferentialDiagnosis
?Age-relateddegenerativeretinoschisis(seethefollowing).
?RRD:Usuallyunilateralandacquiredandassociatedwitharetinal
tear.Pigmentinthe
P.280
anteriorvitreousisseen.See11.3,RetinalDetachment.
Figure11.4.1.Retinoschisis.
Work-Up
?Familyhistory.
?Dilatedretinalexaminationwithscleraldepressiontoruleouta
retinalbreakordetachment.
?Opticalcoherencetomography(OCT)canhelpdeterminethelayerof
theschisis.
Treatment
?Notreatmentforstellatemaculopathy.
?Forunresolvedvitreoushemorrhage,considervitrectomy.
?SurgicalrepairofanRDshouldbeperformed.
?Superimposedamblyopiashouldalwaysbeconsideredinchildren
youngerthan9to11yearswhenoneeyeismoreseverelyaffected,
andatrialofpatchingshouldbeconsidered.See8.7,Amblyopia.
Follow-Up
Every6months;sooneriftreatingamblyopia.
Age-RelatedDegenerativeRetinoschisis
Symptoms
Usuallyasymptomatic,mayhavedecreasedvision.
Signs
Critical.Theschisiscavityisdome-shapedwithasmoothsurfaceandis
usuallylocatedtemporally,especiallyinferotemporal1y.Usually
bilateralandmayshowsheathingofretinalvesselsand“snowflakes”
or“frosting”(persistentMuellerfibers)ontheelevatedinnerwall
oftheschisiscavity.UnlikeX-1inkedjuvenileretinoschisis,splitting
usuallyoccursattheleveloftheouterplexiformlayer.Theareaof
schisisisnotmobile,andthereisnoassociatedRPEpigmentation.
Other.Prominentcystoiddegenerationneartheoraserrata,anabsolute
scotomacorrespondingtotheareaofschisis,hyperopiaiscommon,no
pigmentcellsorhemorrhageinthevitreous,andabsenceofademarcation
line.ARRDmayoccasionallydevelop.
DifferentialDiagnosis
?RRD:Surfaceiscorrugatedinappearanceandcanbeseentomove
morewitheyemovements.Along-standingRDmayresemble
retinoschisis,butintraretinalcysts,demarcationlinesbetween
attachedanddetachedretina,andwhiteretroretinaldotsmaybe
seen.Onlyrelativescotoma.See11.3,RetinalDetachment.
?X-linkedjuvenileretinoschisis(seeprevious).
Work-Up
?Slit-lampevaluationforanteriorchamberinflammationand
pigmentedanteriorvitreouscells;neithershouldbepresentin
isolatedretinoschisis.
?Dilatedretinalexaminationwithscleraldepressiontoruleouta
concomitantRDoranouterlayerretinalhole,whichmayleadto
anRD.
?Afunduscontactlensevaluationoftheretinaasneededtoaidin
recognizingouterlayerretinalbreaks.
?OCTcanhelpdeterminewhichlayeroftheretinaissplit.
Treatment
?SurgeryisindicatedwhenaclinicallysignificantRDdevelops.
?AsmallRDwalledoffbyademarcationlineisusuallynottreated.
Thismaytaketheformofpigmentationattheposteriorborderof
outerlayerbreaks.
Follow-Up
Every6months.RDsymptoms(anincreaseinfloatersorflashinglights
ortheappearanceofacurtainorshadowanywhereinthefieldofvision)
areexplainedtoallpatients,andpatientsaretoldtoreturnimmediately
ifthesesymptomsdevelop.
P.281
11.5Cotton-WoolSpot
Cotton-WoolSpot
Symptoms
Visualacuityusuallynormal.Oftenasymptomatic.
Signs
(SeeFigure11.5.1.)
Critical.Whiteninginthesuperficialretinalnervefiberlayer(NFL).
Note
Thepresenceofasinglecotton-woolspot(CWS)inapatientthatdoes
nothavediabetesmellitus,acutehypertension,oracentralretinalvein
occlusion(CRVO)/branchretinalveinocclusion(BRVO)meritsawork-up
foranunderlyingsystemiccondition.
DifferentialDiagnosis
?Retinalwhiteningsecondarytoneuroretinitis,suchasthatseen
intoxoplasmosis,HSV,VZV,andcytomegalovirus(CMV).These
entitiestypicallyhavevitritisandretinalhemorrhages
associatedwiththem.See12.5,Toxoplasmosis,and12.8,Acute
RetinalNecrosis.
?Myelinatednervefiberlayer:Developspostnatally.Usually
peripapillarybutmaybeinretinalareasremotefromthedisc.
Figure11.5.1.Cotton-woolspot.
Etiology
Thoughttobeanacuteobstructionofaprecapillaryretinalarteriole
causingblockageofaxoplasmicflowandbuildupofaxoplasmicdebrisin
NFL.
?Diabetesmellitus:Mostcommoncause.Oftenassociatedwith
microaneurysms,dot-blothemorrhages,andhardexudates.See
Section11.12,DiabeticRetinopathy.
?Chronicoracutehypertension(HTN):Mayseeretinalarteriolar
narrowingandflamehemorrhagesinchronicHTN.AcuteHTNmayhave
hardexudates,opticnerveswelling,exudativeretinaldetachment.
SeeSection11.10,HypertensiveRetinopathy.
?CRVOorBRVO:Unilateral,multiplehemorrhages,venousdilation,
andtortuosity.MultipleCWS,usually>6to10,seeninischemic
varietyofCRVO/BRVO.See11.8,CentralRetinalVeinOcclusion,and
11.9,BranchRetinalVeinOcclusion.
?Retinalemboli:Oftenfromcarotidarteriesorheartwithresulting
ischemiaandsubsequentCWSdistaltoarterialocclusion.Patients
requirecarotidDopplerexaminationandechocardiography.See
10.22,TransientVisualLoss.
?Collagenvasculardisease:Systemiclupuserythematosis(most
common),Wegenergranulomatosis,polyarteritisnodosa,or
scleroderma.
?Giantcellarteritis(GCA):Age>55years.Symptomsincludevision
loss,scalptenderness,jawclaudication,proximalmuscleaches,
etc.See10.17,ArteriticIschemicOpticNeuropathy.
?HIVretinopathy:Singleormultiplecotton-woolspotsinthe
posteriorpole.See12.10,NoninfectiousRetinal
Microvasculopathy/HIVRetinopathy.
?Otherinfections:Toxoplasmosis,orbitalmucormycosis,Lyme,
leptospirosis,RockyMountainspottedfever,onchocerciasis,
subacutebacterialendocarditis.
P.282
?Hypercoagulablestate:Lupusanticoagulant,homocystinuria,
proteinCandSdeficiency,antithrombinIIIdeficiency.
?Radiationretinopathy:Followsradiationtherapytotheeyeor
periocularstructureswhentheeyeisirradiatedinadvertently.May
occuranytimeafterradiation,butoccursmostcommonlywithina
fewyears.Maintainahighsuspicioneveninpatientsinwhomthe
eyewasreportedlyshielded.Usually,3,000cGyisnecessary,but
ithasbeennotedtooccurwith1,500cGy.Resemblesdiabetic
retinopathy.
?Interferontherapy.
?Purtscherandpseudo-Purtscherretinopathy:MultipleCWSsand/or
superficialhemorrhagesinaperipapillaryconfigurationin
patientswithahistoryofsevereheadtraumaorcrushinjuryto
thechestorlowerextremities.Typicallybilateralbutcanbe
unilateralandasymmetric.See3.19,PurtscherRetinopathy.
?Cancer:Metastaticcarcinoma,leukemia,lymphoma.
?Others:Migraine,hypotension,intravenous(i.v.)druguse,
papilledema,papillitis,severeanemia,sicklecell,acuteblood
loss.
Work-Up
?History:Diabetesorhypertension?Ocularorperiocularradiation
inpast?GCAsymptomsincludingscalptenderness,jawclaudication,
etc.?Symptomsofcollagenvasculardiseaseincludingjointpain,
rashes,etc.?HIVriskfactors?
?Completeocularexamination,includingdilatedretinalexamination
withaslitlampanda60-or90-diopterlensandindirect
ophthalmoscopy.Lookforconcurrenthemorrhages,vascular
occlusion,vasculitis,hardexudates.
?Checkafastingbloodsugar.
?Checkbloodpressure.
?ConsiderESR,CRP,andplateletsifGCAissuspected.
?ConsidercarotidDopplerexaminationandechocardiog
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