陨灶贼允韵责澡贼澡葬造皂燥造熏灾燥造援 3熏晕燥援 1袁 Mar.18, 圆园10 www.IJO.cn 栽藻造押8629原愿圆圆源缘员苑圆 8629-83085628 耘皂葬蚤造押陨允韵援圆园园园岳员远猿援糟燥皂 窑CaseReport窑 Vitrectomyofrhegmatogenousretinaldetachmentin morningglorysyndrome

1 DepartmentofOphthalmology,theAffiliatedHospitalofNorth SichuanMedicalCollege,Nanchong637000,SichuanProvince, China 2 DepartmentofOphthalmology,theFirstPeople's Hospital AffiliatedtoShanghaiJiaotongUniversity，Shanghai200080,China Correspondenceto: XiaoliYang.DepartmentofOphthalmology, theAffiliated HospitalofNorthSichuan Medical College, Nanchong637000,SichuanProvince,[email protected] Received:2010-01-18Accepted:2010-02-19

Abstract · Wereportacaseofretinaldetachmentinmorningglory Figure1 Fundusphotographyoftherighteye Thelarge syndrome(MGS).Wethinkthatidentificationoftheretinal excavateddiscanomalywithretinalvesselsradiatingfromits break,removalofthetractionforcebyvitrectomy,theuseof periphery(whitering),yellowishmaculaandcompletelyretinal long-actinggasasendotamponade,allcontributedtothe detachment successfultreatmentofthedisease.Thepathogenic mechanismsofthediseaseandthefunctionofB-scantothe diagnosisareincluded. · KEYWORDS:morningglorysyndrome;retinaldetachment; DOI:10.3980/j.issn.2222-3959.2010.01.21

YangXL,ZhangX.Vitrectomyofrhegmatogenousretinaldetachment inmorningglorysyndrome. 2010;3(1):89-91

INTRODUCTION orningglorysyndrome(MGS)isacongenital Figure2Fundusphotographyoflefteye(normal) M anomaly,inwhichtheopticnerveheadisenlarged andexcavated,itisnamedforsimilarityinappearanceto washighmyopic(-20.0diopters)andhisbest-correctvision thetrumpetshapedmorninggloryflower [1].Theincidence wasCF/10cmintherighteye.Slit-lampexaminationand oftheanomalyisunknown,butmostauthorsagreethatis rare.Usuallyunilateral,itischaracterizedbyanexcavated intraocularpressurewerenormal.Thefundusshoweda nerve-headdefectwithacentraltuftofwhitefibroglial retinaldetachmentadjacenttothemorningglorydisk tissue,withstraightretinalvesselsradiatingfromtheedgeof anomaly.Therewasatinyslitlikebreaknearthemarginof theanomaly.Retinaldetachmenthasbeenreportedin excavationatthetemporalside.Themacularofthepatient 26-38%ofpatientswithMGS[2].However,thepathogenesis looksyellowish(Figure1).Thelefteyewasunremarkable ofretinaldetachmentinthesecaseshasbeencontroversial. (Figure2).Thepatientdidnothavehearingproblemsand We reportacasewithretinaldetachmentthatwas systemicexaminationwasunremarkable.Thepatientwas successfullytreated. diagnosedwiththemorningglorysyndromewithretinal CaseReport detachmentand amblyopiainrighteye.Thepatient A35-year-oldmalepatientpresentedwithahistoryof6 underwentaparsplanavitrectomywithremovalofthe daysphotopsiaandvisualfielddefectofhisrighteye.A posteriorhyaloid,fluid-airexchange,laserendophotoco- completeocularexaminationwasperformed.Thepatient agulationtothetemporalmarginoftheexcavateddiskand 89 VitrectomyofrhegmatogenousretinaldetachmentinMGS injection20% perfluoropropane. Subretinalfluidwas evacuatedthroughthetinyslitlikebreak.Post-operatively, thepatientwaskeptonaface-downpositionfor3weeks. Theretinawasreattachmentandthebestcorrectedvision was0.08duringthefollow-upfor4months. DISCUSSION Morningglorysyndromeisararecongenitalanomalywith theopticnerve.Manyocularabnormalitieshavebeen reported.Theseincludestrabismus,,, lenscoloboma,eyelidhemangioma,,microph- thalmos, Duane'sretractionsyndromeandretinal Figure3B-scanultrasonogramoftherighteye Thevitreous [3-4] detachment .Retinaldetachmentisthemostcommon cavityextendedtotheposteriorpoleandopticpapilla,projectedto ocularcomplicationandisthoughttobedifficulttorepair thebasalpartofmuscleconesandthustheposteriorpartof becauseretinalbreakswerenotdetectedinmostcases. vitreouscavitylookedlikean upside-down bottleneck;The Severalpathogenicmechanismshavebeenproposedto echogenicbandofretinaldetachmentcouldalsobeseen explainthehighriskofdevelopingretinaldetachmentin patientwithMGS:(1)Continuoustractionexertedbythe detachmentcouldalsobe seen(Figure3).B-scan graduallyincreasingaxialretro-displacementoftheoptic ultrasonography,inparticularly,isconsideredtobereliable nerve [4,5].(2)Abnormalcommunicationbetweenthe imageologicalmethodfortheaccuratediagnosisofMGS sub-arachnoidspaceoftheopticnerveandthesubretinal complicatedwithretinaldetachment. spacemayoccurallowingcentralserousfluidaccumulation RetinaldetachmentrelatedtoMGSwasthoughttode sub-retinally [2,6].(3)Liquefiedvitreousenteringthe difficulttorepair,someofthepatientrequiredmultiple intraretinalspaceattheedgeoftheopticnerve.[4].(4) operationstoachieveretinalreattachmentandhadapoor Leakageoffluidfrombloodvesselswithintheanomalous visualoutcome.Collandcolleaguesreportedapatientof opticdiscorfromtheperipapillarychoroidmaybe retinal detachmentinMGS,therewasapigmented responsibleforthesub-retinalfluid.Asmallretinalholein membranecoveringtheopticdiscandaslit-likeretinal tissuelyingintheopticdiscanomalymayprovideafluid breakwasobservedwithinthecup.Unfortunately,the pathwaybetweenthevitreouscavityandsub-retinalspacein siliconeoilleakedintothesub-retinalspacethroughthe someeyeswithMGS.Ourpatientwashighmyopicandhas retinalopening.Theyemphasizedtheimportanceoftraction aslitlikebreakintheopticdiscanomaly,sothesmallhole membraneremovaltopreventasub-retinalmigrationof wouldallowtheliquefiedvitreoustoenterthesubretinal siliconeoil [2].Inourpatient,theretinawassuccessfully spaceandcausearhegmatogenousdetachment.Themacula reattachedafterasingleoperationandthevisionimproved. mayalsobeabnormalinMSGpatients.Yamanaand Wethinkthatthesuccessfuloutcomemaybeattributedto colleaguescorrelatedalargerelevatedperipapillarywhite thefollowingfactors.(1)Identificationoftheretinalbreak. ringwithanabnormalmaculalackingareflexandwiththe Thelackofcontrastbetweenthewhitescleralbackground retinaltissueappearingyellowish.Ourpatient'sappearance andtheanomalousdiscmaymakeidentificationdifficult. ofmaculawassimilartotheirpatients(Figure1).Thecolor Horeported that Optical coherencetomographywas ofthemaculawasthoughttobeduetoyellowpigmentsin beneficialinthedetectionofsubtleslit-likebreaksatthe theganglioncells[7]. marginofexcavationinretinaldetachmentinMGSandalso FortheaccuratediagnosisofMGS,exceptforthespecial providedagoodguidanceinconfirmingtheclosureofthe appearanceoffundus,wecancheckA/B-scan retinalbreak [8].(2)Removalofepipapillaryfibroglialtissue ultrasonographyandFFAofMGSpatients.Theseexamines anditstractionforces.(3)Useofgas,ratherthansilicone couldshowtheimageologicalfeaturesofMGSfrom oil,asendotamponade.Thelowerinterfacialtensionisnot differentaspects,whichhelpsclinicianstodifferentiateit adequatetopreventbubblesofsiliconeoilfrompassing fromotherdiseasessuchasopticdisccoloboma.For throughtheretinalbreakandthebuoyantforceofsilicone example,thespecialimageologicalfeaturesofB-scan oilisinsufficienttoflattentheperipapillaryretina.The ultrasonogram:thevitreouscavityextendedtotheposterior long-actinggaswithhighsurfacetensionispreferredfor poleandopticpapillaprojectedtothebasalpartofmuscle retinalretachment. cones,thustheposteriorpartofvitreouscavitylookedlike ThevisionisusuallypoorinpatientswithMGS.Thevision anupside-downbottleneck.Theechogenicbandofretinal lossmaybeduetothepresenceofretinalabnormalitiesin 90 陨灶贼允韵责澡贼澡葬造皂燥造熏灾燥造援 3熏晕燥援 1袁 Mar.18, 圆园10 www.IJO.cn 栽藻造押8629原愿圆圆源缘员苑圆 8629-83085628 耘皂葬蚤造押陨允韵援圆园园园岳员远猿援糟燥皂 themacularoramblyopiasecondarytoanisometropiaor 1995;233(7):441-443 .ManydoctorsthinkthatMSGisnon-progressive 3Hope-RossM,JohnstonSS.Themorningglorysyndromeassociatedwith sphenoethmoidalencephalocele. 1990;2(2):147-153 anddoesnotrequiretreatment.ButLoudotandcolleagues 4DebneyS.VingrysAJ.Casereport:themorningglorysyndrome. reportedaclinicalobservationofa2.5-year-oldgirl, 1990;73(1):31-35 referredforthediagnosisofMGSinthelefteyewithsevere 5HaikBG,GreensteinSH,SmithME,AbramsonDH,EllsworthRM.Retinal ,butafter1yeartreatmentwithfunctional detachmentinthemorningglorydiscanomaly. 1984;91 渊12冤: amblyopiatherapy,visualacuityimprovedfrom1/10to7/10[9]. 1638-1647 SoinviewofMSGwithahighriskfordevelopingretinal 6ManschotWA.Morningglorysyndrome:ahistopathologicalstudy. 1990;74(1):56-58 detachmentandassociationwithsomeocularabnormalities, 7YamanaT,NishimuraM,UedaK,ChijiwaT.Macularinvolvementinmorning accuratediagnosis,monitoringandamblyopiatherapyare glorysyndrome. 1983;27渊1冤:201-209 essentialforchildrenwithMorningglorysyndrome. 8HoTC,TsaiPC,ChenMS,LinLL.Opticalcoherencetomographyinthe REFERENCES detectionofretinalbreakandmanagementofretinaldetachmentinmorningglory 1KindlerP.Morningglorysyndrome:unusualcongenitalopticdiskanomaly. syndrome. 2006;84(2):225-227 1970;69(3):376-384 9LoudotC,FogliariniC,BaetemanC,ManciniJ,GirardN,DenisD. 2CollGE,ChangS,FlynnTE,BrownGC.Communicationbetweenthesubretinal Rehabilitationoffunctionalamblyopiainmorningglorysyndrome. spaceandthevitreouscavityintheMorningglorysyndrome. 2007;30(10):998-1001

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