Sveinsson chorioretinal atrophy: the mildest changes are located in the photoreceptor outer segment/retinal pigment epithelium junction

2.50
Hdl Handle:
http://hdl.handle.net/2336/19492
Title:
Sveinsson chorioretinal atrophy: the mildest changes are located in the photoreceptor outer segment/retinal pigment epithelium junction
Authors:
Jonasson, Fridbert; Sander, Birgit; Eysteinsson, Thor; Jörgensen, Thomas; Klintworth, Gordon K
Citation:
Acta Ophthalmol Scand. 2007, 85(8):862-7
Issue Date:
1-Dec-2007
Abstract:
PURPOSE: To locate the mildest and/or earliest changes in the retina and/or choroid in Sveinsson chorioretinal atrophy (SCRA), using more advanced techniques than previous studies. METHODS: We used fundus photography, intravenous fluorescein angiography (IVFA) enhanced ocular coherence tomography (OCT) scans, microperimetry and multifocal electroretinography (mfERG) in an attempt to locate the mildest changes in SCRA. Eight patients with SCRA were examined. To improve the resolution of OCT scans, several consecutive recorded B-scans were retrieved for each location of interest. The scans were processed off-line with an averaging algorithm developed to maximally reduce laser speckle (noise). Static microperimetry was performed using the Rodenstock scanning laser ophthalmoscope (SLO). RESULTS: Biomicroscopy and fundus photographs disclosed an apparent thinning of the retinal pigment epithelium (RPE) in the areas minimally affected, where possible changes in the transparent sensory retina were not visible. In minimally affected areas a choriocapillaris filling defect was evident on IVFA, but some choroidal blood vessels remained open. High-resolution OCT scans in normal eyes showed three highly reflective outer layers, probably representing the junction of the inner and outer photoreceptor segments in the case of the innermost layer, the interdigitizing outer photoreceptors and RPE villi in the case of the middle layer, and the outer RPE in the case of the outermost layer. The middle layer was absent in the transition between affected and unaffected areas in all eyes with SCRA. In the more severely affected areas, the innermost layer was discontinuous and associated with increasing thinning of the outermost layer. Microperimetry of the transition areas sometimes showed clearly defined lesions that were non-responsive to stimuli. It also revealed elevated thresholds (10-15 dB) at the margins and normal thresholds in apparently unaffected areas. CONCLUSIONS: The mildest changes seen in SCRA on OCT are found at the outer photoreceptor/RPE junction; the changes in the outer RPE, choriocapillaris and inner photoreceptor segments may be secondary. Corresponding functional deficits are confirmed on microperimetry and mfERG.
Description:
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Additional Links:
http://www.blackwell-synergy.com/doi/abs/10.1111/j.1600-0420.2007.01002.x

Full metadata record

DC FieldValue Language
dc.contributor.authorJonasson, Fridbert-
dc.contributor.authorSander, Birgit-
dc.contributor.authorEysteinsson, Thor-
dc.contributor.authorJörgensen, Thomas-
dc.contributor.authorKlintworth, Gordon K-
dc.date.accessioned2008-03-03T11:10:23Z-
dc.date.available2008-03-03T11:10:23Z-
dc.date.issued2007-12-01-
dc.date.submitted2008-03-03-
dc.identifier.citationActa Ophthalmol Scand. 2007, 85(8):862-7en
dc.identifier.issn1600-0420-
dc.identifier.pmid17683515-
dc.identifier.doi10.1111/j.1600-0420.2007.01002.x-
dc.identifier.urihttp://hdl.handle.net/2336/19492-
dc.descriptionTo access publisher full text version of this article. Please click on the hyperlink in Additional Links fielden
dc.description.abstractPURPOSE: To locate the mildest and/or earliest changes in the retina and/or choroid in Sveinsson chorioretinal atrophy (SCRA), using more advanced techniques than previous studies. METHODS: We used fundus photography, intravenous fluorescein angiography (IVFA) enhanced ocular coherence tomography (OCT) scans, microperimetry and multifocal electroretinography (mfERG) in an attempt to locate the mildest changes in SCRA. Eight patients with SCRA were examined. To improve the resolution of OCT scans, several consecutive recorded B-scans were retrieved for each location of interest. The scans were processed off-line with an averaging algorithm developed to maximally reduce laser speckle (noise). Static microperimetry was performed using the Rodenstock scanning laser ophthalmoscope (SLO). RESULTS: Biomicroscopy and fundus photographs disclosed an apparent thinning of the retinal pigment epithelium (RPE) in the areas minimally affected, where possible changes in the transparent sensory retina were not visible. In minimally affected areas a choriocapillaris filling defect was evident on IVFA, but some choroidal blood vessels remained open. High-resolution OCT scans in normal eyes showed three highly reflective outer layers, probably representing the junction of the inner and outer photoreceptor segments in the case of the innermost layer, the interdigitizing outer photoreceptors and RPE villi in the case of the middle layer, and the outer RPE in the case of the outermost layer. The middle layer was absent in the transition between affected and unaffected areas in all eyes with SCRA. In the more severely affected areas, the innermost layer was discontinuous and associated with increasing thinning of the outermost layer. Microperimetry of the transition areas sometimes showed clearly defined lesions that were non-responsive to stimuli. It also revealed elevated thresholds (10-15 dB) at the margins and normal thresholds in apparently unaffected areas. CONCLUSIONS: The mildest changes seen in SCRA on OCT are found at the outer photoreceptor/RPE junction; the changes in the outer RPE, choriocapillaris and inner photoreceptor segments may be secondary. Corresponding functional deficits are confirmed on microperimetry and mfERG.en
dc.language.isoenen
dc.publisherMunksgaarden
dc.relation.urlhttp://www.blackwell-synergy.com/doi/abs/10.1111/j.1600-0420.2007.01002.xen
dc.subject.meshAdulten
dc.subject.meshAtrophyen
dc.subject.meshChoroid Diseasesen
dc.subject.meshElectroretinographyen
dc.subject.meshFemaleen
dc.subject.meshFluorescein Angiographyen
dc.subject.meshFundus Oculien
dc.subject.meshHumansen
dc.subject.meshLasersen
dc.subject.meshMaleen
dc.subject.meshMiddle Ageden
dc.subject.meshOphthalmoscopyen
dc.subject.meshPerimetryen
dc.subject.meshPigment Epithelium of Eyeen
dc.subject.meshRetinal Diseasesen
dc.subject.meshRod Outer Segmentsen
dc.subject.meshTomography, Optical Coherenceen
dc.titleSveinsson chorioretinal atrophy: the mildest changes are located in the photoreceptor outer segment/retinal pigment epithelium junctionen
dc.typeArticleen
dc.contributor.departmentDepartment of Ophthalmology, Faculty of Medicine, University of Iceland, Reykjavik, Iceland. fridbert@landspitali.isen
dc.identifier.journalActa ophthalmologica Scandinavicaen

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