OOKPinSJS 窑CaseReport窑 Osteo-odontokeratoprosthesisinStevens-Johnson syndrome:acasereport
1DepartmentofOphthalmology,FacultyofMedicine,Universityof graftsmaynothelpinsuchcasestoprovideusefulvision. Malaya,KualaLumpur,Malaysia Forsuchcases,osteo-odontokeratoprosthesis(OOKP) 2Cornealservicesdepartment,SingaporeNationalEyeCentre& whichwasfirstdescribedbyStrampelli [1] andlateron DepartmentofOphthalmology,YongLooLinSchoolofMedicine, modifiedbyFalcinelli [2] andHull [3] isauseful NationalUniversityofSingapore,Singapore methodforvisualrehabilitationbecauseofitshigher Correspondenceto: ReddySC.DepartmentofOphthalmology, retentionrateandbetterlong-termvisualoutcome.Thisisa UCSISchoolofMedicine,TerengganuCampus,BukitKhor,21600 complexartificialcornealsurgicalprocedure thatis Marang,Terengganu,[email protected] performedintwostagesforvisualrehabilitationincasesof Received:2011-01-11Accepted:2011-03-10 cornealblindnesswithdryocularsurfaceinwhichcorneal transplantationwithamnioticmembraneandlimbalgrafts Abstract havefailed.Instageone,anautologuscaninetoothis ·AIM:Toreportasuccessfulosteo-odontokeratoprosthesis removed,modifiedto receivean optical polymethyl (OOKP)procedureinacaseofendstageofcornealblindness methacrylatecylinder;andimplantedinasubmuscular duetoStevens-Johnsonsyndrome(SJS). pocketunderthelowereyelidfor2-4months.Theocular ·METHODS:Aninterventionalcasereport. surfaceisdenudedandreplacedwithafullthicknessbuccal mucosa.Instagetwo,thetoothcylindercomplex(osteo- · RESULTS:Wedescribea35-year-oldIndianwoman,a odontolamina)isretrievedandsurroundingexcesssoft knowncaseofSJSwithbilateraldryeyesandcorneal tissueisremoved;andthenimplantedintothecorneaafter blindness(failedcornealgraftwithvascularisedtotalcorneal reflectionofbuccalmucosalflap,cornealtrephination,total opacityintherighteyeandnon-healingcornealulcerinthe iridodialysis, cryoextractionofthelens andanterior lefteye).Visionwashandmovementonlyinbotheyes.The vitrectomy.Awindowismadeinthebuccalmucosacorr- cornealulcerhealedwithmedicaltreatmentresultingin espondingtotheopticalcylinder,throughwhichthepatient vascularisedtotalcornealopacitywithnoimprovementin recoversthevision [4].Inthisreport,wedescribeapatient vision.OOKPwasperformedintherighteyeandthevision withbilateraldryeyesandend-stagecornealblindness wasimprovedfromhandmovementto6/6.Thesamevision secondarytoSJSinwhomexcellentvisualrecoverywas wasmaintainedintherighteyeatthelastfollow-up5years aftersurgery. achievedfollowingOOKPsurgeryintherighteye. CASEREPORT CONCLUSION:OOKPprovidesgoodvisualrehabilitation · A35-year-oldIndianwoman,aknowncaseofSJSsince withlong-termanatomicallystableprosthesisinpatientswith 1985secondarytoampicillin,presentedwithbilateraldry end-stageofocularsurfacedisordersandcornealblindness eyesandcornealblindness.OneyearafterSJSaffliction,she secondarytoSJS. underwentpenetratingkeratoplastyinthemoreadversely · KEYWORDS:osteo-odontokeratoprosthesis; Stevens- affectedrighteyein1986inourhospital.However,there Johnsonsyndrome;cornealblindness wasnoimprovementofvisionintherighteyeduetograft DOI:10.3980/j.issn.2222-3959.2011.02.23 failure.Shewasusingartificialteareyedrops4timesdaily inbotheyes.Shewasabletododailyroutineworkwiththe ReddySC,TajunisahI,TanDT.Osteo-odontokeratoprosthesisin poorvisioninthelefteye.Becauseofvisualdisability,she Stevens-Johnsonsyndrome:acasereport. 2011;4 underwentcataractsurgerywithintraocularlensimplantin (2):212-215 thelefteyein1995inChennai,India.Postoperatively,the visioninthelefteyewasgoodformanyyears.Shewas INTRODUCTION referredonFebruary6,2004tooureyeclinicbyaprivate ornealtransplantationisnotsuccessfulinallcasesof ophthalmologistfornon-healingcornealulcerinthelefteye. C Stevens-Johnsonsyndrome(SJS)becauseofdryocular Righteye:visionwashandmovementwithgoodprojection surfaceandadditionalamnioticmembraneandstemcell oflightinallthequadrants.Failedcornealgraftwithtotal 212 陨灶贼允韵责澡贼澡葬造皂燥造熏灾燥造援 4熏晕燥援 2袁 Apr.18, 圆园11 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
Figure1 Ocularfindings A:Righteyeshowingvascularizedtotalcornealopacitywithafailedcornealgraft;B:Lefteyeshowing vascularizedtotalcornealopacityandlateraltarsorrphy
Figure2Stageoneoperation A:Opticalcylinderfittedintheautologuscaninetoothbeforeitssubmuscularimplantationunderthelower eyelid; B:Buccalmucosaocularsurfaceofrighteyeattheendofstageoneoperation vascularisedcornealopacitywithnodetailsvisiblebehind Afterdetailedevaluationofthepatient,OOKPwasadvised wasnoted.Intraocularpressurewasnormal.Lefteye:vision intherighteye.Detailedexaminationofeyeincluded washandmovementwithgoodprojectionoflightinallthe slit-lampbiomicroscopyevaluationofocularsurface,tear quadrants.Lateraltarsorrhphywaspresent.Circumcorneal film,fornicialstatus;cornealandanteriorchamberstatus; congestionwithcentralcornealulcer(4mm),hypopyonand ultrasoundbiomicroscopy,A-andB-scanultrasonography, vascularizationintheupperpartoflimbuswerenoted.There elecrophysiologicalassessmentofopticnerveandretinal wasthinningofcentralcorneawithimpending function.Oralexaminationincludedexaminationofthe descemetocoeleformation.Posteriorchamberintraocular buccalmucosaforpreexistingscarring,generaldentaland lenswasstable.Patientwasadmittedintoeyewardand periodontalstatus,andidentificationofthecanineand treatedwithgentamycinfortifiedeyedrops,atropineeye premolarteethwiththeaidofpanoramicorperipheral drops,timololeyedropsandcarboxymethylcellulosetears radiographs. eyedrops.Abandagesoftcontactlenswasalsoapplied.The Thepatientwasexplainedaboutthemultistageoperation culturefromthecornealscrapingsshowed andinformedconsentwastaken.TheOOKPsurgerywas organism,sensitivetoamikacinandcefoxitim. doneintwostages:stageoneoperationonOctober1,2004- Amikacineyedrops3%everyhourlyandsystemicamikacin anautologuscaninetoothwasremoved,modifiedtoreceive (300mgI.V.every8hoursfor1week)wereaddedtothe anopticalpolymethylmethacrylatecylinder,4.0mmanterior abovetreatment.Astheulcerstartedhealing,thefrequency diameterand5.24mmposteriordiameter(Figure2A);and ofamikacindropswastaperedoffslowly.Thecornealulcer implantedinasubmuscularpocketunderthelowereyelid healedcompletely,after12weeksofadmission,resultingin for2-4months.Theocularsurfacewasdenudedand vascularizedcornealopacitywithnoimprovementinvision. replacedwithafullthicknessbuccalmucosa(Figure2B). ShewasreferredtoSingaporeNationalEyeCentre(SNEC) foradviceregardingthepossibilityofkeratoprosthesis After3months,stagetwooperationonJanuary6,2005-the surgeryinrighteye.Patientwasexaminedinthecorneal toothcylindercomplex(osteo-odontolamina)wasretrieved serviceunitofSNEConAugust5,2004.Righteye:vision andsurroundingexcesssofttissuewasremoved(Figure3A); andanteriorsegmentfindingsweresameasbefore(Figure1A). andthenimplantedintothecorneaafterreflectionofbuccal Theintraocularpressurewas18mmHg.andultrasound mucosalflap,cornealtrephination, total iridodialysis, B-scanwasnormal.Lefteye:visionandanteriorsegment cryoextractionofthelensandanteriorvitrectomy.A findingswerethesameasbefore(Figure1B).The windowwasmadeinthebuccalmucosacorrespondingto intraocularpressure (IOP)was13mmHgandultrasound theopticalcylinder,throughwhichthepatientperceivesthe B-scanwasnormal. vision(Figure3B). 213 OOKPinSJS
Figure3Stagetwooperation A:Toothcylindercompleximplantrecoveredfromthecheekduringstagetwooperation; B:Final appearanceofrighteyeafterstagetwooperation
Table 1 Frequency of SJS cases as indication for OOKP in the published literature Total number Number of Author Year Country % of cases SJS cases [10] Marchi et al 1994 Italy 85 10 11.8 [2] Falcinelli et al 2005 Italy 181 4 2.2 [6] Hille et al 2006 Germany 25 1 25.0 [4] Tan et al 2008 Singapore 16 7 44.4 [7] Michael et al 2008 Spain 145 22 15.1 Liu et al [8] 2008 UK 36 16 44.4 [9] Fukuda et al 2008 Japan 4 3 75.0 [5] Iyer et al 2010 India 50 24 48.0
Thedetailsofstageoneoperationprocedure,stagetwo antiglaucomamedicines.Visionintherighteyewas6/9 operationprocedures,postoperativemanagementandfollow unaided,butimprovedto6/6with+0.25DS. Buccal upweredescribedbyTan [4].Onday1postoperatively, mucosawashealthy.Opticalcylinderwascenteredwell.Her theglobewasslightlyfirmondigitalpalpationandvision intraocularpressurebypalpationappearednormal(inthe wasgood.Shewasmaintainedontab.Acetazolamideand highteens)withcup-discratioof0.8;andtherewasno oralpotassium.ShewasfollowedupregularlyinSNECand deteriorationofthevisualfield.Herlefteyevisionremained in-betweenthevisitsinourhospitalalso.During6weeks handmovementonlywithtotalvasculaizedcornealopacity. visit,hervisionwasimprovedto6/9unaided.Fundus DISCUSSION examinationshowedcup-discratio0.7;visualfieldtesting OOKPisanartificialcornealsurgicalprocedurewhereinthe revealedsuperiornasalstepinHumphryandGoldmann opticalcylinderisfittedintothepatients'owntoothand perimetry.Therefore,oralglycerolwasaddedtodiamox. implantedintotheeyeforimprovementofvisioninhopeless Fivemonthsafterthesecondstageoperationhervisionwas bilateralcornealblindnesscaseswithgoodfunctioningretina noted6/6unaided.Patientwasextremelyhappyaboutvisual andopticnerve.TheOOKPsurgerycanofferanatomically recoveryintherighteye.Buccalmucosawashealthy. stablecornealprosthesisinthelongterm,aswellasaidin Opticalcylinderwaswellcentred.Pilocarpineingelform visualrehabilitationincaseswithbilateralocularsurface wasaddedtotheglaucomatreatment.Shewasthen diseaseandcornealblindnessbutstillhavingafunctioning followedup6monthly. retinaandopticnerve.Thecommonconditionsthatleadto Onreviewoneyearlater,visionwasmaintained6/6unaided end-stagecornealblindnessincludeSJS,severechemical inrighteye;eyeballwasstillslightlyfirm,butacceptable; burns,ocularcicatricialpemphigoid,endstagetrachoma, buccalmucosawashealthy;CDratiowas0.7andvisual multiplefailedgrafts,graft-versushostdisease,Sjogren fieldunchanged.Oralglycerolwasstopped;Xalacomand syndrome,Lyellesyndrome,bullouskeratopathysecondary Alphaganeyedrops,tetracyclineeyeointmentwereadded. toglaucoma,xerosis,cornealopacitysecondarytointerstitial Then,shewasfolloweduponceinayear.Duringthe keratitisandbullouskeratopathy,burnsfromflames,molten follow-upvisits,theglaucomamedicationwaschangedto metalsandexplosions,andmultiplegraftfailures [1-10] .The GuttLumigan,GuttAzopt,GuttPilocarpine2%andTab contraindicationsforOOKPincludelackoflightperception Diamox250mgbd.Shewasadvisedtoconsultoureyeclinic vision,phthisisbulbi,end-stageglaucomawithadvanced immediatelyiftherewasanyproblemintherighteye. opticnervedisease,advancedmaculardiseaseorirreversible Thelastfollow-upwasonApril20,2010(5yearsafterthe retinalpathology,andpatientswhohavesatisfactoryvision operation).Shewasfullystable.Sheisstillonthesame fortheirdailyactivities [11] . 214 陨灶贼允韵责澡贼澡葬造皂燥造熏灾燥造援 4熏晕燥援 2袁 Apr.18, 圆园11 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 CornealblindnesssecondarytoSJSisoneofthecommon organismisolatedfromcornealscrapingsinclinicalpractice. indications(2.2%to75.0%)inthepublishedreportson Before1992, wasdesignatedas OOKP (Table1).Itconstituted44.4%inSingaporestudy species .Olderliterature,fromthe 1950s, andlittlemore(48.0%)inIndianstudy. describedrapidlygrowingmycobacteriaas VariouscomplicationsreportedfollowingOOKPsurgery complex.Speciatingisolatesiscriticalforchoosingtherapy includemucousmembraneulceration,mucousmembrane becauseantibioticsensitivityvariesfromspeciestospecies overgrowthontotheopticalcylinder,odontallaminaexposure, [15].Treatmentofinfectionscausedbythispathogenrequires laminaresorption,tiltingofopticcylinder,extrusionofoptic prolongedmultidrugtherapy. ,resistantto cylinder,denovopostoperativeglaucoma,hypotony,sterile conventionalantituberculousagents,isusuallysensitiveto vitritis, endophthalmitis,retinal detachment,vitreous amikacin,azithromycin,clarithromycinandcefoxitin [16]. haemorrhage,choroidaldetachment,expulsivehaemorrhage, Inconclusion,OOKPprocedureoffersagoodvisual retroprostheticmembrane,shorteningoffornix [1-10] . rehabilitationwithlong-termsurvivaloftheprosthesisin TheOOKPhasasyntheticoptic(polymethylmethacrylate) patientswithSJSandcornealblindness. withabiologicalhaptic(patient'sowntooth).Itcan REFERENCES withstandaveryhostileocularsurfaceenvironmentlikedry 1StrampelliB.Keratoprosthesiswithosteodontaltissue. 1963; 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