focal choroidal excavation: cause or effect?may case reports2019 697 case reports case 1 a...
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696 Indian Journal of Ophthalmology Volume67Issue5
understandthattheirnamesandinitialswillnotbepublishedanddue effortswill bemade to conceal their identity, butanonymitycannotbeguaranteed.
Financial support and sponsorshipNil.
Conflicts of interestTherearenoconflictsofinterest.
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Department of Vitreo‑Retina, Giridhar Eye Institute, Ponneth Temple Road,Kadavanthra,Cochin,Kerala,India
Correspondence to:Dr.MaheshGopalakrishnan,Giridhar EyeInstitute,PonnethTempleRoad,Kadavanthra,Cochin,Kerala‑682020,India.E‑mail:[email protected]
Manuscriptreceived: 31.08.18;Revisionaccepted: 05.01.19
Cite this article as: Shah RC, Gopalakrishnan M, Goyal A, Anantharaman G, Sethia A. Focal choroidal excavation: Cause or effect? Indian J Ophthalmol 2019;67:696-8.
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Focal choroidal excavation: Cause or effect?
Raj C Shah, Mahesh Gopalakrishnan, Anubhav Goyal, Giridhar Anantharaman,
Abhishek Sethia
We report the imaging characteristics of focal choroidalexcavation (FCE) and associated choroidal neovascularmembrane(CNVM)andinterprettheprobableetiopathogenesisof FCE through findings in four patients detected byspectral‑domain optical coherence tomography (SD‑OCT).FCE was found as an acquired entity in two of our casessubsequenttotreatmentofCNVM,whereas inthetwoothercasesFCEwaspre‑existing. Furthermore, associationofFCEwith pachychoroid spectrum is reaffirmed through this caseseries.
Key words: Choroidal neovascularmembrane, focal choroidalexcavation,pachychoroid
Focalchoroidalexcavation(FCE)isaconcavityinthechoroidofunknownetiology,occurringwithoutanyadjacentscleralabnormalityorectasia,andcharacterizedbygoodvisualacuityandshowsminimalchangesovertime.Itwasfirstdescribedwith time‑domainoptical coherence tomography (OCT)byJampol et al.in2006.[1]
FCE is believed bymost authors to be a congenitalcondition, though its etiopathogenesis is yetunclear. FCEis detected primarily as anOCT finding. It is frequentlyassociatedwith pachychoroiddiseases, including centralserous chorioretinopathy (CSCR), choroidal neovascularmembrane (CNV) andpolypoidal choroidal vasculopathy(PCV).AlthoughFCEisclassicallythoughttobecongenital,acquiredFCEformspossiblyexist.[2]
Toourknowledge, theknown reports of acquiredFCEfollowingCNVMwasbyLeeet al.,[2]whereasmallchoroidalexcavation developed during CNV scarring changes inage‑relatedmaculardegeneration,whereas another singlecasereportbyHashimotoet al.[3]wassecondarytomultifocalchoroiditis.
Here,wereportfourcasesofFCEassociatedwithCNVM,including twoof acquired FCEpost treatment ofCNVM,onewithpre‑existingFCE,whichlaterincreasedinsizeovertimeaftertreatmentofCNVM,andonewheretheFCElesionremainedstableoverthefollow‑upperiod.
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May2019 697Case Reports
Case ReportsCase 1A17‑year‑old femalepresentedwithmetamorphopsia andblurred vision in right eye (OD) since 1week.Her bestcorrectedvisualacuity(BCVA)was6/9;N8inODand6/6;N6in left eye (OS). Clinical examination revealed findingssuggestiveofchoroidalneovascularmembrane(CNVM).Herspectral‑domain optical coherence tomography (SD‑OCT)showedanactiveType2CNVMassociatedwithaFCE,whichwasconfirmedbyfundusfluoresceinangiography(FFA).ShewastreatedwithmultipleintravitrealantiVEGFinjections.Atherfollow‑upvisitstherewassignificantCNVMresolution,butFCEwasprogressivelyincreasingindepth,asmeasuredfromahorizontallinedrawnatthelevelofRPEtotheinnermarginofFCE, from121µmto256µm over the period of 2years, possiblydue to the contractionof theunderlyingscar[Fig.1].
Case 2A21‑year‑oldmalepresentedwithdistortionofvisioninOSnotedaccidently since8dayswithBCVAof6/6;N6 inbotheyes. Fundus examination revealed an extrafoveal grayishmembranewith overlying hemorrhages. Enhanceddepthimaging showedpachychoroidwith thickness of 443µm.SD‑OCT revealedCNVMassociatedwith FCE.AntiVEGFmonotherapywasgivenwithcompleteresolutionofCNVM,buttheFCEremainedstableinsizeandconfigurationduringsubsequentfollow‑ups.FinalBCVAwasmaintainingto6/6;N6inboththeeyes[Fig.2].
Case 3A 48‑year‑old female presented to uswith blurring anddistortionofvisioninOSsince2weeks.HerBCVAwas6/6;N6inODand6/24;N10inOSatpresentation.FundusexaminationshowedanextrafovealCNVM.BasedonmultimodalimagingincludingSD‑OCTandFFA,patientwasdiagnosedtohave
Figure 4: (a) Color fundus photograph showing orangish mass (choroidal osteoma) with hemorrhages. (b) FFA showing staining of osteoma and blocked fluorescence. (c) Enhanced depth imaging of the osteoma. (d) B-scan image showing highly reflective choroidal mass. (e) SD-OCT showing an active CNVM. (f) Development of subfoveal FCE (arrow) during the treatment course
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Figure 3: (a) Color fundus photograph showing grayish membrane surrounded by hemorrhage. (b) Fundus fluorescein angiography showing hyperfluorescence. (c) SD-OCT at presentation showing active CNVM features of subretinal fluid and hyper-reflectivity without FCE. (d, e, f) Serial SD-OCT images showing development of acquired FCE during the treatment course
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Figure 2: (a) Color fundus photograph showing subretinal grayish membrane with hemorrhage suggestive of active CNVM. (b, c) FFA early and late phase showing staining of occult CNVM. (d) Enhanced depth imaging showing thick choroid (443 µm). (e, f) SD-OCT images show CNVM at the site of excavation with the size of FCE (269 µm) remaining almost constant during follow‑up
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eFigure 1: (a) Color fundus photograph showing serous macular detachment with grayish membrane and overlying hemorrhage. (b, c) Fundus fluorescein angiography early and late phase showing classic CNVM leak. (d) Enhanced depth imaging showing thick choroid (480 µm). (e, f) SD-OCT of FCE with CNVM at presentation and posttreatment with antiVEGF therapy
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698 Indian Journal of Ophthalmology Volume67Issue5
type‑2CNVMwithsubfovealchoroidalthicknessof505µm.ShewasalsotreatedwithantiVEGFmonotherapyon prorenata(PRN)basisandhadmultiplerecurrencesover5years.Interestingly,shedevelopedFCEatthesiteofresolvedCNVMscar, during 23months of treatment,whichprogressivelyincreasedfrom85µmto154µmindepthoverthesubsequentfollow‑ups.WereportthisasacaseofacquiredFCEsecondarytoCNVM[Fig.3].
Case 4A46‑year‑oldmalepatientpresentedtouswithblurringofvisionOSsince2months.HisBCVAwas6/6,N6inODand6/12,N8inOSatpresentation.FundusexaminationofOSshowed an irregular elevated, yellowish‑white choroidallesionwithwelldefinedgeographic borders involving theinferiormaculawithareasofsubretinalhemorrhageatandabovethefoveasuggestiveassociatedchoroidalneovascularmembrane. SD‑OCT showed a dense hyper‑reflectivechoroidalmasswithsignificantwideningof thechoroidallayers.B‑scanultrasonographyrevealedhigh‑spikeechoesthrough the lesion suggesting choroidal osteoma. FFA inthat area showed staining conforming to the diagnosisof choroidal osteomawith a secondary CNVM. Patientunderwent treatmentwithmultiple antiVEGF injectionsand standard fluence photodynamic therapy (PDT). Thepatient developed an acquired FCE after 17months oftreatment[Fig.4].
DiscussionSeveral authors have postulated that FCE lesions arecongenitalchoroidalabnormalitiesrelatedtodevelopmentaldefectsorsomeothertypeoffocalstructuraldefectwithinthechoroid.[1,2,4,5]
Ellabbanet al.[4] and Hashimoto et al.[3] suggested,theOCTfindingofan“unusualhyper‑reflectivetissue”beneathsomeFCElesionscouldrepresentscarringofchoroidalconnectivetissue fromaprevious inflammatoryprocess.Presumably,scarcontractioncoulddrawthechoroidandRPEtowardthescleraproducingFCE.Ourcasessupporttheabovealternativeetiopathogenesis leading to anacquired subtypeofFCE inaddition to theknowncongenital formofFCEcomplicatedsecondarilybyCNVM/CSCR.
TheoccurrenceofFCE in eyeswith increased choroidalthicknesshasbeennotedbyseveralinvestigators.[6,7]Ourcasesalsohad increased choroidal thicknesswithpachyvessel/sadjacent to the excavation, reaffirming the fact that FCE isassociatedwiththepachychoroidspectrum.
ConclusionToconclude,FCEisanOCT‑baseddiagnosis.FCEisknowntobeassociatedwithpachychoroidspectrumofdiseases,withknownassociationsbeingCSCRandCNVM.OriginofFCEcanbeoftwotypes:congenitalandacquired.Thesignificanceoftheacquiredvarietyremainsunknownuntilmoresuchcasesarereported.Periodicmonitoringofsuchcasesisofimportancetodetectandtreatanyoftheknownassociations.
Declaration of patient consentTheauthors certify that theyhaveobtainedall appropriatepatient consent forms. In the form thepatient(s) has/havegivenhis/her/theirconsentforhis/her/theirimagesandotherclinicalinformationtobereportedinthejournal.Thepatientsunderstandthattheirnamesandinitialswillnotbepublishedanddue effortswill bemade to conceal their identity, butanonymitycannotbeguaranteed.
Financial support and sponsorshipNil.
Conflicts of interestTherearenoconflictsofinterest.
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