Posterior ischaemic optic neuropathy

19 Pages·2004·0.77 MB·English

Eye(2004)18,1188–1206
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Posterior ischaemic SSHayreh
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optic neuropathy:
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R clinical features,
I
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pathogenesis, and
G
E
management
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P
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T
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M
Abstract nonarteritic PIONproduced significant
O
L improvementofvisualacuityaswellasvisual
Purpose To investigateandpresent a
O
fields,butnotsoinarteriticorsurgicalPION.
G comprehensiveaccountoftheclinicalfeatures,
However,some spontaneousvisual
IC pathogenesis,andmanagement ofposterior
improvementalso occurred insome untreated
A ischaemicoptic neuropathy (PION).
L nonarteritic PIONcases.
Methods Thisretrospectivestudyisbasedon
S Conclusions PIONisadistinctclinicalentity
Y 53consecutive eyesof42patients withPION
but should bediagnosedonlyafter exclusion
M seeninmyclinicsince1973,whofulfilledthe
P ofallothercausesofvisualloss.Inallpatients
inclusion criteria.They weresystematically
O olderthan50,GCAmustberuledout.Thereis
S evaluated,treated,andfollowed byme.All
IU patients hadinitiallydetailed ophthalmic usually markedvisualloss, withcentralfield
M evaluation ofthe anterior andposterior defectbeingthe most common. Thestudy
suggests thathigh-dose steroid therapyin
segments,includingvisual field with
nonarteritic PION, soonafter theonset of
Departmentof Goldmannperimeterandfluorescein fundus
visualloss, resulted insignificant visual
OphthalmologyandVisual angiography.All patients aged50years and
Sciences,Collegeof improvementcomparedtotheuntreatedcases,
olderwere also investigatedfor giant cell
Medicine,Universityof but not inarteritic andsurgicalPION.
arteritis(GCA). Everyattemptwas madeto
Iowa,IowaCity,IA,USA Eye (2004)18, 1188–1206.
ruleoutothercausesofvisualloss.Follow-up
doi:10.1038/sj.eye.6701562
Correspondence:SSHayreh evaluationwassimilartotheinitialevaluation
Departmentof exceptangiography. Aetiologically, PIONcan
Keywords: giant cellarteritis; ischaemic optic
OphthalmologyandVisual bedivided intothree types:arteritic dueto
Sciences neuropathy;posterior ischaemic optic
GCA,nonarteritic not dueto GCA,and
UniversityHospitals& neuropathy;radical neck dissection; spinal
surgicalfollowinga surgicalprocedure.
Clinics surgery
200HawkinsDrive Steroidtherapywas givento onlythose
IowaCity nonarteritic PIONpatients whooptedto try
IA52242-1091,USA that,but was givento allarteriticPION
Tel: þ13193562947 Introduction
patients.
Fax: þ13193537996
Results PIONwasnonarteriticin28patients In mystudieson theblood supplyofthe optic
E-mail:sohan-hayreh@
uiowa.edu (35eyes),arteriticin 12(14 eyes),andsurgical nerve,it wasfound that, unlike the rest ofthe
inthree (four eyes).Visualacuity varied optic nerve,the anterior part ofthe optic nerve
Received:4September between20/20andnolightperceptionFitwas (opticnervehead)hasitsowndistinctsourceof
2003 countfingers orlessin19of35eyeswith bloodsupply,thatis,posteriorciliary arteries
Accepted:4September
nonarteritic PION,four of14in arteritic, and (PCAs),which do notsupplythe restofthe
2003
allfourwithsurgicalPION.Themostcommon optic nerve.1,2Fromclinical andexperimental
SupportedinpartbyGrants visualfield defectwas centralvisualloss, studies, Ifound in1974thatischaemic optic
EY-1151andRR-59from aloneorin combination withother types of neuropathy involvingthe optic nerve headisa
theNationalInstitutesof visualfield defects. Initially,opticdisc and distinct clinical entity,dueto interferencewith
Health,Bethesda,Maryland,
fundus showed noabnormality but thedisc the PCAcirculation,andI namedit anterior
andinpartbyunrestricted
usuallydeveloped pallor inabout6–8weeks. ischaemic optic neuropathy (AION).3An
grantfromResearchto
PreventBlindness,Inc.,New Aggressivetreatmentwithhigh-dosesystemic accompanying editorial, commenting onmy
York. steroidduringthe veryearly stagesof paper,stated: ‘TheabbreviationAIONadopted
Posteriorischaemicopticneuropathy
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byHayrehalmostofnecessitypresupposestheexistence Inclusion criteria
ofaposteriorcounterpart‘PION’whichmight,perhaps,
beappliedtothepost-traumaticopticatrophyassociated IstressedinmyoriginalpaperonPION:‘Therearesome
with damagetothe optic nerve withinits bonycanal.’4 diagnoseswhich should not bemade until other
Later,in1978,havingseenmorepatientswithischaemic possibilities have been carefully ruled out.I thinkPION
opticneuropathy,Iclassifiedischaemicopticneuropathy isoneofthese.’7Iadheredtothisruleverystrictlyinthe
into twotypes:AION, involving theanterior part ofthe presentstudy.Therefore,Imadeallpossibleattemptsto
optic nerve,and posterior ischaemic optic neuropathy excludefromthis studypatients whose diagnosiswas
(PION) involvingthe restofthe optic nerve.5,6In 1981, evenslightly indoubt, evenif theywere referredto me
Isubmitted apaperdescribing PIONindetail tothe withadiagnosisofPION.Myinclusioncriteriawere:(1)
Archives ofOphthalmologyfor publication; it was suddenonsetofvisualdeterioration;(2)presenceofoptic
rejected with the remarkthat‘thereis noreasonto nerve relatedvisual fielddefect(s);(3) initially, normal
believethat anysuch clinical entityexists’. It was opticdiscandrestofthefundusonophthalmoscopyand
published elsewhere.7 Innumerable reports have since fluorescein angiography;and(4) exclusionof anyother
establishedPION asa clinical entity,but allofthem ocular,optic nerve, or neurologic disorders,including
except twoareanecdotalcase reports,basedon oneor compressive, inflammatory,orother mechanisms,as
twopatientsonly.Theexceptionsare:(1)byIsayamaetal8 causeofvisual loss. Forpatients with GCA,Iused the
in14PIONpatients and(2) bySaddaet al9 in72PION criterion ofpositivetemporal artery biopsyforGCA, in
patients. allbutthreewhereforvariouslogisticreasonsIcouldnot
Thus,basedonitsbloodsupply,theopticnervecanbe getatemporalarterybiopsy;thesethreepatientshadall
dividedintotwodistinctregions:(a)theopticnervehead theclassicalclinicalfindingsofGCA,includinganorexia,
almostentirelysuppliedbythePCAcirculation1,2and(b) weight loss,anaemia, malaise,tender temporalarteries,
the rest ofthe opticnerve (ieposterior part ofthe optic scalp tenderness, headache,neck pain andjaw
nerve) supplied from multiple other sources2,10–12(see claudication, highly elevatederythrocytesedimentation
below). Pathogenetically, aswellas clinically,acute rate(ESR), and/or C-reactive protein (CRP)and
ischaemiaofthe optic nerve resultsintwo verydistinct thrombocytosis,sothattherewasnodoubt oftheir
typesofischaemicopticneuropathy:(a)AIONinvolving diagnosisofGCA.
theopticnervehead,and(b)PIONinvolvingasegment PIONwasnonarteriticin28(35eyes)patients,arteritic
ofthe posterior part ofthe optic nerve. in12(14eyes)patients,andsurgicalinthree(foureyes)
Aetiologically, PIONcan beclassified into three patients.
types:(1) arteriticPION duetogiant cellarteritis Attheinitialvisit,allpatientshadadetailedsystemic
(GCA),(2) nonarteritic PIONduetocausesother than andophthalmichistory,includingspecificquestioning,in
GCA,and(3)surgicalPIONattributable toa surgical detail,ofpatientsaged50yearsandolderfortheocular
procedure.9,13 andsystemic signsand symptomsofGCA.All patients
The objectiveofthe presentstudyisto presenta hadophthalmic evaluation byme;this included Snellen
comprehensive account ofthe clinical features, visual acuity andvisual fieldtesting with a Goldmann
pathogenesisand managementofPIONbased ona perimeter usingI2e, I4e, andV4eisopters (unlessthe
retrospective studyof53consecutive PIONeyesof43 visual losswas soseverethatthe fields could notbe
patients, systematicallyevaluated, treated,and plotted), andmost ofthem alsohad anAmslergrid
followed duringthe past 30yearsinmyOcular evaluation ofthe sizeofthe centralscotoma. All had
Vascular Clinic at the University ofIowa Hospitals external andslit-lamp examinationofthe anterior
andClinics. segment, lensandvitreous,intraocular pressure
measurement,ophthalmoscopicexaminationand,almost
invariably, fluorescein fundus angiography.Allpatients
aged50yearsandolderor suspected tohave GCAalso
Materials andmethods
hadESR (Westergren)and, from1985,CRP to ruleout
Thisretrospectivestudyisbasedon53consecutiveeyes GCA;ifGCAwassuspected,theywerepromptlystarted
of42Caucasianpatients andone Blackpatientwith on high-dosesteroid therapy,similar tothe steroid
PIONseenintheOcularVascularClinicattheUniversity therapyregimendescribed indetail elsewhere.14–16
ofIowa Hospitals andClinics since1973, whowere Temporalartery biopsy wascarried outas soon as
systematically evaluated,treated,and followedby me convenientto confirmthe diagnosis. To ruleout other
personally, except forthreepatients,investigated causesofvisual loss,includinginflammatory,
similarlybyDrRandyKardon,myneuro-ophthalmology compressive, infiltrative orother forms ofoptic
colleague. neuropathy (including Leber’s optic neuropathy),
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appropriateinvestigations werecarried out, including mildinFigures2dand3c,moderateinFigures2aand3b,
orbital echographic,magnetic resonanceimaging, and/ marked inFigures1c,d, 2b, 3aand 4a,b,andseverein
orcomputertomography,andneurologicevaluation.All Figure1a,b.
nonarteritic patients had asystemic evaluation. Ateach
follow-up visit, allpatients hadthe sameevaluation as
on the initialvisit except forfluoresceinfundus Statistical analyses
angiography.
The linear mixedmodelanalysis for repeatedmeasures
wasused tocompareLogMAR visual acuityandvisual
Steroidtherapy field grade(0–4Fsee above)changes innonarteritic
PIONeyes thathadsteroid treatmentandthose not
Arteritic PION To prevent further visual loss,all
treated.Thefactorsinthemodelweretreatment(steroid
patients weretreatedwith a steroid therapyregimen
or none), time (baselineandfinal follow-up), andthe
similar tothatI haveused inmyother studies inGCA
treatment–timeinteractioneffect.Specificmeancontrasts
patients; it isdescribed elsewhere.14–16
thatweretested included (1)between-treatmentgroup
Nonarteritic PION Since thereisno known
comparison ofmeans atbaseline andat last follow-up,
treatmentforthisandnoinformationwasavailableasto
and(2)testofmeanchangefrombaselinetofinalfollow-
whethersteroidtherapyisbeneficialtothesepatientsor
up withineach treatmentgroup. The P-valuesfor these
not,allthesepatientsweregiventheoptiontotrysteroid
twosets ofcomparisons were adjustedusing
therapyiftheywishedafterafullexplanationofthepros
Bonferroni’smethod. ABonferroniadjusted
andconsofthetherapy.Of28patientswith nonarteritic
P-valueo0.05 wasconsidered statistically significant.
PION, 14opted totry the steroid therapy,andit was
The observed prevalenceofsevenmajor systemic
started the dayofthe initial visitto theclinic or soon
diseasesin nonarteritic PIONpatients waseach
after.Theinitialdosewasusually80mgoralPrednisone
comparedwith those expected inthe age-matched
daily,except for threewhoweregivena single
controlpopulation from estimatesreportedfor US
intravenous mega-doseofcorticosteroids initiallyFthis
Caucasianpopulation for1990–1992 bythe National
wascompletelyrandom,withoutanyparticularreasonto
Centerfor Health Statistics.17 Statistical significance was
do so.Usually,80mgPrednisonewasgiven for2 weeks
assessed usingexact binomialprobabilities.
andthengradually taperedoff,with the wholesteroid
therapy lastingforabout 2–21months.
2
SurgicalPION Two ofthethreehadintravenous
Results
mega-dose steroid therapyfor 2 dayson the daythe
visual loss wasdiscovered,followed byrapidtapering Demographic characteristics
within afewdays.
This retrospective studyhad43patients (53 eyes):28
(35 eyes) withnonarteritic PION,12(14 eyes)with
Visual statusevaluation arteriticPIONandthree(foureyes)withsurgicalPION.
Nonarteritic PIONwasseenin17womenand11 men
The visual outcome wasevaluated ina maskedmanner
(nineright,12left,andsevenbotheyes),arteriticPIONin
withoutknowledgeofthetypeofPION,treatmentgiven,
10womenandtwomen(threeright,sevenleft,andtwo
orother factors influencingit. The methods used for
botheyes),andsurgicalPIONin2womenandoneman
evaluationofvisualacuityandvisualfieldswereexactly
(oneright, oneleft, andone both eyes).Agerange was
the sameasdescribed indetail elsewhere.14In addition,
20–90(median 61.5,interquartile 52–70) yearsin
visual field losswasoriginally gradedsubjectively into
nonarteritic PION, 62–83(median 73.4,interquartile 71–
arbitrary gradesfrom 0 (normal)to 4 (severe loss)in
79)yearsinarteriticPION,and50–82(median77.3)years
stepsof0.5;the findings werethencondensed for
insurgicalPION.Ofthe12patientswitharteriticPION,
descriptivepurposesintomild(grades0.5–1.0),moderate
threehadoccult GCA18 with no systemic symptomsor
(1.5–2.0),marked(2.5–3.0),andsevere(3.5–4.0)loss.The
signs ofGCA.
grade wasjudged byqualitatively assessingon
computing clinically the amountofisopteric visual field
loss andfactoring inthe functional disability produced
Follow-up
bythatdefect;forexample,inferiorand/orcentralvisual
fielddefect,producingfarmorefunctionaldisability,was This wasevaluatedseparately forthe threetypes of
assignedamuchhighergradethanacorrespondingloss PION.
inthe upper fieldorelsewhere.These gradesarebest Nonarteritic PION Ofthe35eyes, threeeyeswere
described by thefollowing figures: normalinFigure2c, followed less than2 weeksafterthe onset ofPION.
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The follow-upfor the remaining32eyesafteronset Table2 VisualfielddefectsinarteriticandnonarteriticPION
variedbetween 1.3and214(median 16.2)months. eyes
Arteritic PION The follow-upfor allthe 14eyeswith
Typeofvisualfielddefect Nonarteritic Arteritic
arteriticPIONafteronsetvariedfrom1.3to55.6(median PION PION
11.4)months.
Totaleyesa 32 13
SurgicalPIONThefoureyeswerefollowedfor4.6,9.3,
Superioraltitudinaldefect 3 0
9.3,and56.6months after the onset ofPION.
Inferioraltitudinaldefect 0 1
Centralscotomaalone 6 3
Superiornasalparacentral 1 2
Visualsymptoms scotoma
Inferiornasalparacentral 1 0
Apartfromthe symptom ofsuddenvisual loss of
scotoma
variable degree, fiveeyeshadalsoexperienced Centralscotomawithother 9 2
amaurosisfugaxbeforethat(threeofthemwitharteritic fielddefects
PION). Centrocecalscotomaalone 5 0
Markedgeneralized 1 2
constrictiononlywithno
centralscotoma
Visualacuity
Nasalperipheralloss 1 0
Table1 summarizes theinitial visual acuity inthethree Temporalperipheralloss 2 0
Onlyperipheralislandfield 5 2
types ofPION.
remaining
Inferiornasaldefect 4 2
Superiortemporaldefect 1 1
Visualfields
Superiornasaldefect 2 0
Peripheralconstrictionwith 3 0
Table2 summarizes thevarious typesof visual field
centraldefect
defects seeninarteritic andnonarteritic PIONon initial
examination.Centralvisualloss,aloneorincombination aVisualacuitynolightperceptionortoopoortorecordvisualfieldsin
threenonarteriticPIONeyesandonearteriticPIONeye.
with other types ofvisual fielddefects, wasthe most
common visual fielddefect seeninthe presentstudyin
PION(Figures1,2a,band3).Forexample,ineyeswhere
nasalperiphery intwo; inthe twoarteritic PIONeyesit
thevisualfieldscouldbeplotted,27ofthe32eyes(84%)
waslocatedintheinferiorperipheryinoneandinferior
with nonarteritic PIONandnineof13eyes(69%) with
temporalperiphery inone.
arteritic PIONhada centralfield defect.However,in
Asdiscussedabove,theseverityofthevisualfieldloss
contrast tothis,a marked generalizedperipheral
wasgraded into mild,moderate, marked,andsevere.
constriction, with onlya smallnormal centralresidual
Figure5gives the information innonarteritic PION. In
field,waspresentinoneeyewithnonarteriticPIONand
arteriticPION,thevisualfieldcouldnotbeplottedinone
twoeyeswith arteritic PION(Figure4).Whenonlya
eyewith light perception visiononlyand inthe
peripheral island visual fieldwaspresent,in thefive
remaining13eyesit wasmildin five,moderate inone,
nonarteriticPIONeyes,itwasinthetemporalperiphery
markedinfour,andsevereinthree.Amongthefoureyes
intwo, inferiortemporal periphery inoneand inferior
withsurgicalPION,intheonlyseeingeyeitwassevere.
Table1 InitialvisualacuityindifferenttypesofPION Opticdisc changes
Visualacuity Nonarteritic Arteritic Surgical The optic discwasinitially normal inalleyes.The disc
PION PION PION
developed pallorofvariabledegreeafter a variable
20/20–20/25 6 4 0 lengthoftime inallexcept seveneyes(five with
20/30–20/40 1 2 0 nonarteritic PIONandtwo arteriticPION)with a final
20/50–20/70 3 1 0 visualacuityof20/20or20/25andnocentralvisualfield
20/80 1 0 0
defect except intwowith a tinyparacentral scotoma.
20/200–20/400 5 3 0
Thiswouldindicatethatthesesevennervessufferedlittle
Countingfingers 14 2 1
Handmotion 2 0 0 or nopermanent ischaemic optic nerve damage.In the
Lightperception 2 1 0 eyesthatdeveloped optic discpallor,it wasusually not
Nolightperception 1 1 3 possibleto determine the precise timeof itsonset
becauselogisticallyitwasnotpossibletoexamineevery
Totaleyes 35 14 4
patienteveryweekorso.Thetimeintervalbetweenthese
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Figure1 Fourvisualfieldsshowingvaryingsizesanddensitiesofcentralscotomawithnormalperipheralvisualfieldsinnonarteritic
PION.Righteyefieldsof(a)a74-year-oldwomanand(b)an87-year-oldwoman;and(c)and(d)fieldsintheleftandrighteyes,
respectively,ofa52-year-oldman.Noteinallfigures:scotomawithI4eisthesolidblackareaandwithV4eisthedottedarea.
patients’visitstotheclinicwasusually1monthoreven generalized pallor, it wasusually more markedinthe
longerforvariousgeographicandclimaticreasons.(Our temporal partFthis agreeswith centralscotoma being
TertiaryCareUniversityofIowaHospitaldrawspatients themostcommonvisualfielddefectinPION.Therewas
fromanareawitharadiusofabout300–400miles.)Also, no evident difference inthe opticdisc pallor amongthe
sincethe development ofpallor isa gradual andsubtle differenttypesofPION.Inthepresentseries,intwoeyes
process,it is notalways possibletopinpoint itsexact with nonarteritic PION, the discofthe eyewith PION
onset.As farasIcould judge,itwasusually 6–8weeks, developedanincreaseincupsizecomparedtothefellow
although sometimes it wasas shortas only3–4weeks, normal eye,inaddition totemporal pallor.
while inothercases it wasover 8weeks. Whetherthis
depends upon the siteoflesioninthe optic nerve or
Rest of the ophthalmicevaluation
severity ishardtocomment. Inthe eyesofthe present
study,mild-to-moderatetemporalpalloroftheopticdisc External and slit-lampevaluation ofthe anterior
wasthe most common finding, andwhen a dischad segment, intraocular pressuremeasurements,and
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Figure 2 Visual fields of both eyes of a 74-year-old woman with nonarteritic PION, showing marked visual improvement with
high-dosesteroidtherapy.(a)and(b)arevisualfieldsofleftandrighteyes,respectively,2daysaftertheonsetofvisualloss,witha
visualacuityof20/300inbotheyes.Thepatientwasstartedon80mgPrednisonedailythatday.(c)Fieldoflefteye13daysafter(a).
(d)Fieldofrighteye34daysafter(b).Totaldurationofsteroidtherapywas21months.Finalvisualacuitywas20/20inthelefteyeand
2
20/40intherighteye.After6months’follow-upthevisualfieldsandacuityremainedstable.
ophthalmoscopyrevealednoabnormalityinanyofthese artery disease (stenosis and/or plaqueinnine),
eyes, except forage-relatedlenschanges. peripheral vasculardisease(one), vasospasticdiseases
(migraine infourandRaynaud’s diseaseinone),
gastrointestinal ulcer (four),smoking (in nineF34715
Systemic diseases associated with nonarteriticPION
packyears) andhypercholesterolaemia(11).Magnetic
Thefollowingsystemicdiseaseswereseenin28patients resonance imaging and/orcomputer tomography was
with nonarteritic PION: arterial hypertension performedin16patients to rule out anyothercause of
(13patients), diabetesmellitus (six), ischaemic heart visual loss.Temporalartery biopsywasperformedin
disease (seven), othercardiacdiseases(including eightto ruleout GCA.
valvulardisease,atrialfibrillation,patentforamenovale, Whenthe prevalence ofmajorsystemic diseasesin
congestive heartfailure,andechocardiographic nonarteritic PIONpatients ofthe presentstudywas
abnormalities, insix), arterial hypotension comparedwiththose expected inthe age-matched
(threeForthostatic inone), thyroid disease (three), control population inthe US Caucasianpopulation, it
rheumatologic diseases(threeFtwoofthem had showed a significantly higher prevalenceofarterial
systemiclupus),cerebrovascularaccidents(five),carotid hypertension (P¼0.022), ischaemic heart disease
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Figure3 Threevisualfieldsoflefteyeofan81-year-oldmanwithnonarteriticPION:(a)onthedayofthevisualloss,withvisual
acuityonlycountfingers.Patientwasstartedon80mgPrednisonedailythatday.(b)Visualfieldsthenextdaywithvisualacuity
20/30,and(c)15dayslaterwithvisualacuity20/25.Totaldurationofsteroidtherapywas2months.Onafollow-upof19monthsthe
visualfieldsandacuityremainedstable.
(P¼0.026),cerebrovasculardisease (P¼0.006),carotid ESR was90.5(range 13–130)mm/h,andmedian CRP
artery and peripheral vasculardisease (Po0.0001), 4.75 (range1.5–12.2)mg/dl.
diabetes mellitus (P¼0.014), migraine (P¼0.039), and
gastrointestinalulcers (P¼0.011)inthe nonarteritic
SurgicalPION
PION patients.
Inthepresentstudy,therewereonlythreepatientswith
surgicalPION. This isa newly emerging type ofPION,
with medicolegal implications.Therefore,Ifeelthata
ESR and CRP
summary oftheircase reports isinstructive.
InpatientswithnonarteriticPION,themedianESRwas
25(range1–89)mm/h,andCRP o0.5mg/dlinall 1. A55-year-oldwoman,witharterialhypertensionand
patients except 2 (1.1mginoneand 10.6mginanother ischaemic heart disease,hadlumbarfusion spinal
with myocardialinfarction,and congestiveheart surgeryinthepronepositionlastingforabout6h.At
failureFinboth the temporalartery biopsywas theendoftheprocedure,shehadbilateralgrossfacial
negative).In patients with arteritic PION, the median andorbital oedema. Duringsurgery shelost about
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Figure 4 Visual fields of (a) right and (b) left eyes of a 79-year-old woman with arteritic PION, showing remaining, markedly
constrictedcentralvisualfields,withcompletelossofperipheralfieldsinbotheyes.
Figure5 Twographsshowingchangefrominitialtofinalvisualfieldsin15eyesofpatientstreatedwithsteroidtherapy(leftgraph)
and15eyesofthosenottreated(rightgraph).Normal ¼ normalvisualfields.
400ml ofblood,and hersystolic bloodpressure was rapidtapering,without anybenefit. Theeyesdidnot
around90mmHganddiastolicaround60mmHg,and recover anyvision ona follow-up for9 monthsand
later,during recovery fromanaesthesia, diastolic developedbilateral optic atrophy.
bloodpressure wasaslowas 45mmHg.When she 2. A17-year-oldmandevelopedorbitalfloorfractureon
becamealert aftersurgery,shereportednolight theleftsidefollowingfootballinjury.Thelefteyehad
perception inboth eyes. Bothfundi were normal. normalvisualacuityonexaminationthatdayandalso
Magnetic resonance imagingofthe brainandorbits the nextdayafter recovery fromanaesthesia for
showed noabnormality. Shehadintravenous surgerytorepairthefracture.However,laterthatday
megadose steroid therapyfor 2days followed by hedevelopedmarkedorbitaloedema.Whenseennext
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1196
morning he hadno lightperception inthateye. The andthereforecould not haveshown anysignificant
optic discand funduswerenormal, and improvement;theywereexcludedfromdataanalysison
electroretinography wasnormal. Hehadintravenous visual acuity improvement. Ofthe remaining14eyes
megadose steroid therapyfor 2 days followedby withaninitialvisualacuityof20/40orworse,nineeyes
rapidtapering, without any benefit. The eyedidnot (64%) showed visual improvement. Among the16eyes
recoverany visionon afollow-up for 16months and ofuntreatedpatients,fourshowed visual acuity
developed optic atrophy. improvement(Figure6).Again,fourofthe16eyes,with
3. An 82-year-old womanhad cataract surgeryin her aninitial visual acuityof 20/20–20/25,wereexcluded
right eye with retrobulbaranaesthesia.At herfirst fromdataanalysisforvisualacuityimprovement.Ofthe
chancetoseewiththateyethenextday,shereported remaining12eyeswithavisualacuityof20/70orworse,
onlycountfingersvision.Thevisualfieldexamination four(33%) improved.
showed a large absolutecentral scotomawith a Oftheeyesthathadvisualacuityatbaseline20/40or
normal peripheral field.The optic disc andfundus worse,themeanvisualacuity(logMAR)atbaselinedid
wereperfectlynormal. At 5weeksafter surgery,the not differ significantly between thesteroidtreatedand
optic discdeveloped pallor.The visual acuityand untreatedeyes(P¼0.165),indicatingthatthetwogroups
fieldsdidnotchangeonfollow-upforalmost5years. hadsimilarvisualacuitytobeginwith.Thetreatedeyes
In hermedical history,shehad arterial hypertension, showed a significant improvement frombaseline acuity
hyperlipidaemia, mitral annularcalcification, (P¼0.031),withthemeanacuityatfinalfollow-upbeing
transient ischaemic attacks,cortical atrophyon significantly better thanthe untreated eyes(P¼0.023).
magnetic resonanceimaging,erythrocyte Thus, the findings ofthisstudy showedthat eyesof
sedimentation rate27mm/h,and C-reactiveprotein patientstreatedwithhigh-dosesystemicsteroidtherapy
0.1mg/dl. showedsignificantvisualacuityimprovementcompared
to untreated eyes.
Effectofsteroidtherapy onvisualfunction
The following criteriawereused for visual acuity
Visual acuitystable or worse innonarteritic PION
improvement.(a)Visualacuityimprovementmustbeat
Ofthe16eyesinthetreatedgroup,fiveremainedstable
least X2 lines ofSnellenchart; (b)Theremust be
(20/20–20/25intwo, 20/80 inone, handmotioninone
concomitant improvement in thecentral 51ofvisual
andno light perception inone),and twodeteriorated
fields, torule out apparentvisual acuityimprovement
(from20/40to 20/400,and20/60 to20/400).Ofthe 16
fromsimple eccentric fixation orlearning experience;
eyesinuntreatedgroup, nineremainedstable(20/
and(c) The visual improvementmust have been
25–20/25infour,andcount fingers infive),and two
maintained onfollow-up andnot be justfluctuation or
deteriorated (from20/200 tocount fingers).
transient.
InnonarteriticPIONThiswasevaluatedin32ofthe35
eyeswithnonarteriticPION,becausetheremainingthree
Time intervalbetween theonset ofvisual loss andstart of
eyesoftwopatientshadafollow-upoflessthan2weeks
steroidtherapy
andwereexcluded fromthis evaluation.Ofthe 32eyes,
This wasasfollows. (i)In nineeyeswith improvement:
16wereofpatients treatedwith high-dosesteroid
the therapywasstarted the sameday5, and2,7 and9
therapy (seeabovefor therapyregimen)andthe
dayslaterin2,1,and1,respectively.(ii)Intwoeyeswith
remaining16hadnotreatment.Visualimprovementwas
deterioration:itwasstarted27daysaftertheonsetwhen
evaluatedonfollow-upbythechangeinvisualacuity,as
the patient wasfirstseen. (iii)In fiveeyeswith stable
well ascentraland peripheral visual fields.
visualacuityof20/20,20/25,20/80,handmotionandno
light perception,it wasstarted 1, 27,7,5, and24days,
Visual acuity respectively, afteronset.
Figure6 givesthe initialpretreatmentand finalvisual
acuity inthe treatedgroup,and initialandfinal visual
acuity inthe untreated group. Time intervalbetween start ofsteroidtherapyand onsetof
visual improvement
Visual acuityimprovementinnonarteritic PION Itwasthedayafterthestartoftherapyinfour,andafter
In the treated groupof16eyes,nine showed 7,9,13, and16days,with no datainoneeye.In the four
improvementbyX2linesofSnellenchart(Figure6).Two untreatedeyeswith visual acuityimprovement,it was
ofthe16eyeshadaninitialvisualacuityof20/20–20/25, seen4,6,43, and53days after theonset ofPION.
Eye
Posteriorischaemicopticneuropathy
SSHayreh
1197
Figure6 Twographsshowingchangefrominitialtofinalvisualacuityin16eyesofpatientstreatedwithsteroidtherapy(upper
graph)and16eyesofthosenottreated(lowergraph).
Visualfields that, theeyesof thetreatedgroup showeda significant
improvementfrom baseline(Po0.001)andit was
Figure5shows the changesinthe visual fields ineyes significantly greater(P¼0.030)thaninthe untreated
with nonarteritic PION(both treatedand untreated eyes. Thedataalso indicated a bettermean visual
groups) during follow-up. Themean visual field grade fieldgrade at finalfollow-up for thetreatedeyes
at baselinedid notdiffersignificantly betweenthe comparedtothe untreated eyes(P¼0.064).Thus, the
steroidtreatedanduntreatedeyes(P¼1.000),indicating findings ofthisstudy showed thateyesofpatients
thatthe twogroups weresimilarto beginwith. The treatedwith high-dosesystemicsteroid therapyshowed
eyesofthe untreated groupdidnot show asignificant significant visual fieldimprovementcomparedto
improvementfrom baseline(P¼0.465). Incontrast to untreated eyes.
Eye