医学资料 眼科 Wills Manual Chapter 11

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.

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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

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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

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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

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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.

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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.

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•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