Surgical Outcome of 21 Patients with Congenital Upper Eyelid Coloboma

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Surgical Outcome of 21 Patients with Congenital Upper Eyelid Coloboma 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ClinicalResearch窑 Surgical outcome of 21 patients with congenital uppereyelidcoloboma DepartmentofOphthalmology,LiaquatUniversityEyeHospital, ocularstructuresareincompletelyformedduetofailureof Hyderabad,Pakistan theembryonicopticfissuretofuse [1].Theresultantgap, Correspondence to: ArshadAliLodhi.Departmentof notchorholemayinvolvedifferentstructures,includingthe Ophthalmology,LiaquatUniversityEyeHospital, Hyderabad, eyelid,iris,lens,ciliarybody,choroids,opticnerveor [email protected];[email protected] retina,causingmildtoseverevisionloss.Thefrontonasal Received:2009-12-11 Accepted:2010-01-05 andmaxillaryprocessesofanembryoareformedatthe4th weekoflifeandcontinuetheirstructuraldifferentiationuntil Abstract 8to9weeks.Anyformofdisturbancethatoccursduring · AIM:Toevaluatethesurgicaloutcomeofcongenitalupper thisembryologicalperiod(4to9weeks)canaffectthe eyelidcolobomarepair. formationofnormaleyelid [2].Althoughtheexactcauseof · METHODS:Allpatientsunderwentcompleteophthalmic congenitaleyelidcolobomaremainsuncertain,several theorieslikeitisaformoffacialcleft [3],andintrauterine andgeneralexaminationbeforegoingtosurgery,andthen examinationunderanesthesiawasperformedtoassessthe factorssuchasamnioticband,inflammation,decreased siteandsizeofeyeliddefect,conjunctivalinvolvement.The placentalcirculation,mechanicalinfluences,orabnormal statusofcorneaandocularmotilitywithforcedductiontest vascularsystemhavebeensuggestedtobefactorsinvolved wasalsobeingnoted.Thesurgicalprocedurewasperformed ineyelidcoloboma[3,4]. accordingtothesizeofdefect. Adefectintheeyelidmarginwasfirstdescribedin1585by [5] · RESULTS:Outof21casesofcongenitaluppereyelid JacquesGuillemeau .Varioussystemsforclassificationof [6-8] [6] coloboma,18occurredinisolationwithuppereyelidmedial eyelidcolobomashavebeendescribed .Jackson defect,13werebilateraland5wereunilateral.Otherswere reclassifiedthelidanomaliesin1988ascryptophthalmos associatedwithGoldenharsyndromeandCHARGEsyndrome and eyelidcolobomas.Eyelid colobomasaremost withbilateralupperlidmedialdefects.Allpatientswere commonlytriangularinshapewiththebaseattheeyelid presentedforsurgicalcorrectionsduringageof2.5-4.0years margin.Itisusuallylocatedonthemedialhalfoftheupper exceptonethatpresentedat25yearsofage.Cosmetically eyelidorlateralshalfofthelowereyelidandcanvaryin surgicalresultswereacceptable,exceptonethatwasalready sizeformasmallindentationoftheeyelidtonearabsence presentedwithopaquecorneal. oftheentireeyelid.Eyelidcolobomaisrareandcommonly · CONCLUSION:Inthisstudy,overallsurgicalresultswere occursasanisolatedfinding.However,theyhavebeen satisfactoryexceptonethatwaspresentedlatewith reportedtooccuraspartofamultisystemsyndrome.These compromisedcornea. include Goldenhar [9,10].Teacher-Collins [11],Delleman [12] [13] ·KEYWORDS:coloboma;upperlidcongenital;surgicaloutcome syndromes andfrontonasaldysplasia .Otherless [14] DOI:10.3980/j.issn.2222-3959.2010.01.16 commonassociationincludeFraser andnasopalpebral lipomacolobomasyndromes [15],andanewun-named [16] LodhiAA,JunejoSA,KhanzadaMA,SahafIA,SiddiqueZK.Surgical syndromedescribedbyBalei consistingofupper outcomeof21patientswithcongenitaluppereyelidcoloboma. eyelidcoloboma,hypertolorism,hypospadiasandhearing 2010;3(1):69-72 loss. Theeyelidcolobomausuallypresentswithexposure INTRODUCTION keratopathyduetopoorBell'sphenomenon.Soitrequires hewordcolobomaisGreek(Koloboma)meaning immediateattention [2],otherwisethecorneainevitably T mutilatedorcurtailedandreferstoacongenital ulceratesandtheeyebecomesblind [17,18].Managementof malformationthatoccursinabout1:10000births,inwhich thesecasesinvolvesprovidingcornealprotection,surgical 69 Surgicaloutcomeofcongenitaluppereyelidcolobomarepair Figure1Directclosureofbilateralupperlidmedialtwothirdsfullthicknesscoloboma Figure2Cutlerbeardprocedure Figure3Cutlerbeardprocedure,CHARGEsyndrome Figure4Directclosureofleftupperlidlessthanmedialonefourthfullthicknesscoloboma Figure5Directclosureofbilateralupperlidmedialonefourthfullthicknesscoloboma Figure6Bilateralupperlidmedialtwothirdsfullthicknesscoloboma repairoftheeyeliddefectandanyassociatedocularand SurgicalProcedure Allsurgerieswereperformedbya systemicanomalies,andmonitoringvisualdevelopment[2]. singlesurgeon.Thedefectsonefourth'soruptoonethird's MATERIALSANDMETHODS theeyelidwererepairedbydirectclosure(Figure1).The Subjects Twenty-onepatientsforthisstudywereselected edgesofthedefectswererefreshedwithsharpincisionsand fromoutpatientdepartmentofOphthalmology,Liaquat preciseanastomosiswasperformedbybringinglidmargins UniversityofMedical&HealthSciencesEyeHospital, togetherandbyusingtwolayerapproximationsofthetarsus HyderabadfromOctober2008toJune2009.Onlypatients andskin.Thenlateralcanthotomyorcantholysiswascarried ofagerangedfromoneyearto25yearswithuppereyelid outtominimizethehorizontaltensionofthelid.Thedefect colobomawereincludedinthestudy.Patientsolderthan25 morethanonethird'soruptohalf'stheeyelidwererepaired yearsofageandwithlowereyelidcolobomawereexcluded withCutlerBeardprocedure(Figures2,3).Inthis fromthestudy.Allpatientsunderwentcompleteophthalmic procedureafullthicknesslowereyelidflapwasadvanced andgeneralmedicalexaminationbeforegoingtosurgery intothedefectofuppereyelidbypassingitbehindthe andalldatawererecordedonaprintedprofroma.Atthe remaininglowereyelidmargin. timeofinitialvisitallmotherswereinterviewedregarding RESULTS thetermofdelivery,theirexposuretodrugsandinfections Inthisstudy,16patientsweremaleand5werefemale. diseases duringpregnancyandtheheritanceofthe Therewasnohistoryofimmaturebirthofanycase.Ageof coloboma.Thenallpatientswerereferredtopediatric patientsrangedfrom2.5to4.0years(median3years),only specialistforgeneralmedicalexaminationtoexcludethe onepatienthadlatepresentationthatwas25yearsofage systemicdisease.Duringgeneralophthalmicexamination (Figure4).Outof21cases13presentedwithbilateralupper positionofeyeball,Bell'sphenomenonandanyfacial lidmedialdefectasisolatedcoloboma (Figures5,6).Five deformity werealsonoted.Allpatientsthenwent casespresentedwithleftupperlidmedialdefectasisolated examinationunderanaesthesia(EUA)toassessthesiteand coloboma.Threecasespresentedwithsystemicassociation. sizeoftheeyeliddefect,conjunctivalinvolvement,statusof Outofthreeonepatienthadtheproblemofexposure thecorneaandocularmotilitywithforcedductiontestswas keratopathy (Figure7)andpoorBell'sphenomenonwith alsonoted.Beforerepairingeyelidcoloboma,thehorizontal bilateralupperlidmedialdefect.Systemicassociationofone defectsweremeasuredbygentlypullingtheedgesofthe casewasGoldenharsyndromewithunilateralupperlid eyeliddefects.Thentheverticaldefectsweremeasuredfrom medialdefectandofanotherwasCHARGEsyndrome thelashlinetotheapexofthedefect,asonefourth's,one (Figure 8)withbilateralupperlidmedialdefect third's,half'sormorethenhalf'stheeyelidsize. symblepheronwasfoundintwocasesthathavesystemic 70 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 Table 1 Clinical data of patients & surgical procedure N Sex Age(yr) Occular features Systemic features Surgical procedure 1 M 3.0 ·Bilateral upper lid medial one ·Depigmented skin around the ·Direct closure of lid with third’s full thickness coloboma mouth canthotomy and cantholysis ·Poorly formed eye brows. ·Vertical Hairy growth ·Symplepherectomy ·Bilateral superior symblepheron ·Poor Bell’s phenomenon ·Bilateral corneal opacities 6 M 3.5 ·Bilateral upper lid medial one - ·Direct closure of lid forth’s full thickness coloboma 3 F 3.0 ·Bilateral upper lid medial one ·Direct closure of lid forth’s full thickness coloboma 1 F 2.5 ·Left upper lid less than medial ·Goldenhar Syndrome Left ·Direct closure of lid full thickness coloboma hemifacial atrophy. Left ·Repair of cleft lip & papate. cleft upper lip. and palate By general plastic surgery 4 M 4.0 ·Bilateral upper lid medial two ·Direct closure of lid with third’s full thickness coloboma cantholysis 1 M 3.0 ·Bilateral upper lid medial two ·CHARGE syndrome ·Culter Beared rocedure third’s full thickness coloboma Symplepheronectomy with sympblepheron 4 M 4.0 ·Left upper lid less than medial ·Direct closure of lid one forth’s full thickness coloboma 1 F 25 ·Left upper lid less than medial one ·Direct closure of lid forth’s full thickness coloboma Howevertheyhavebeenreportedtooccurasapartof multipleocularandsystemicanomalies [2,9-16,20].Inourseries onlytwopatientshavesystemicanomaliesofGoldenharand CHARGEsyndrome.Noneofpatientshadiriscoloboma, cataract,glaucoma,strabismus,retinalcolobomaoriris atrophy.Cornealprotectionandcosmesisarecommon indicationsforrepairingtheeyeliddefectsanditstreatment dependonthesizeofdefect [12].Thecornealtoleranceto exposureisexcellentinyoungchildrenbecausetheyhavea [2] Figure7Bilateralupperlidmedialonethirdfullthickness goodBell'sphenomenon ,hencecornealprotectioncan coloboma usuallybeachievedwithadditionoflubricants.Therefore,it mayoftenbesafetodelaytherepairingofthesmalldefect tilltheeyelidtissueavailableforreconstruction [14].Inour study,allpatientsreferredforrepairingtheliddefectsafter 2.5to4.0and25yearsofage.Howeverearlysurgical interventionmaybenecessaryinpatientswithlargedefect orpoorBell'sphenomenon[2,21]. Mostofthesurgeonsliketorepairwithdirectclosure [2], Tenzelprocedure [15] andCutlerBeard'sprocedure [16],the defectsofonefourth's,onethird'sanduptohalf'stheeyelid respectively.Inpresentstudymostofthepatientsof Figure8CHARGEsyndrome,bilateralupperlidmedialtwo thirdthicknesscoloboma colobomapretendedwithsizeofonefourthtoonethird
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