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Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis

Gyrate atrophy of choroid and retina is an autosomal recessive condition characterized by peripheral multiple sharp areas of chorioretinal atrophy which become confluent with age. Macula and central vision is typically involved late in the disease. Macular involvements such as cystoid macular edema,...

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Autores principales: Tripathy, Koushik, Chawla, Rohan, Sharma, Yog Raj, Gogia, Varun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4932791/
https://www.ncbi.nlm.nih.gov/pubmed/27433038
http://dx.doi.org/10.4103/0974-620X.184529
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author Tripathy, Koushik
Chawla, Rohan
Sharma, Yog Raj
Gogia, Varun
author_facet Tripathy, Koushik
Chawla, Rohan
Sharma, Yog Raj
Gogia, Varun
author_sort Tripathy, Koushik
collection PubMed
description Gyrate atrophy of choroid and retina is an autosomal recessive condition characterized by peripheral multiple sharp areas of chorioretinal atrophy which become confluent with age. Macula and central vision is typically involved late in the disease. Macular involvements such as cystoid macular edema, epimacular membrane, and choroidal neovascularization have been reported in gyrate atrophy. In this report, we present a family with diminished central vision presenting within 8 years of age. All of three siblings had typical peripheral chorioretinal atrophic lesions of gyrate atrophy and hyperornithinemia. On spectral domain optical coherence tomography, two of elder siblings showed macular edema. Hyporeflective spaces appeared to extend from outer nuclear layer to the inner nuclear layer level separated by multiple linear bridging elements in both eyes. Ultrawide field fluorescein angiogram (UWFI) even in late phase did not show any leak at macula suggesting foveoschisis. Foveoschisis in gyrate atrophy has not been reported before.
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spelling pubmed-49327912016-07-18 Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis Tripathy, Koushik Chawla, Rohan Sharma, Yog Raj Gogia, Varun Oman J Ophthalmol Case Report Gyrate atrophy of choroid and retina is an autosomal recessive condition characterized by peripheral multiple sharp areas of chorioretinal atrophy which become confluent with age. Macula and central vision is typically involved late in the disease. Macular involvements such as cystoid macular edema, epimacular membrane, and choroidal neovascularization have been reported in gyrate atrophy. In this report, we present a family with diminished central vision presenting within 8 years of age. All of three siblings had typical peripheral chorioretinal atrophic lesions of gyrate atrophy and hyperornithinemia. On spectral domain optical coherence tomography, two of elder siblings showed macular edema. Hyporeflective spaces appeared to extend from outer nuclear layer to the inner nuclear layer level separated by multiple linear bridging elements in both eyes. Ultrawide field fluorescein angiogram (UWFI) even in late phase did not show any leak at macula suggesting foveoschisis. Foveoschisis in gyrate atrophy has not been reported before. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC4932791/ /pubmed/27433038 http://dx.doi.org/10.4103/0974-620X.184529 Text en Copyright: © Oman Journal of Ophthalmology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution NonCommercial ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Tripathy, Koushik
Chawla, Rohan
Sharma, Yog Raj
Gogia, Varun
Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
title Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
title_full Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
title_fullStr Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
title_full_unstemmed Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
title_short Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
title_sort ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4932791/
https://www.ncbi.nlm.nih.gov/pubmed/27433038
http://dx.doi.org/10.4103/0974-620X.184529
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