Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy

Introduction. Gyrate atrophy is a rare genetical metabolic disorder affecting vision. Here, we report a 9-year-old boy with gyrate atrophy associated with bilateral macular edema at the time of diagnosis and the effect of long term metabolic control on macular edema. Case Presentation. A 9-year-old...

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Main Authors: Sibel Doguizi, Mehmet Ali Sekeroglu, Mustafa Alpaslan Anayol, Pelin Yilmazbas
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Ophthalmological Medicine
Online Access:http://dx.doi.org/10.1155/2015/137270
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author Sibel Doguizi
Mehmet Ali Sekeroglu
Mustafa Alpaslan Anayol
Pelin Yilmazbas
author_facet Sibel Doguizi
Mehmet Ali Sekeroglu
Mustafa Alpaslan Anayol
Pelin Yilmazbas
author_sort Sibel Doguizi
collection DOAJ
description Introduction. Gyrate atrophy is a rare genetical metabolic disorder affecting vision. Here, we report a 9-year-old boy with gyrate atrophy associated with bilateral macular edema at the time of diagnosis and the effect of long term metabolic control on macular edema. Case Presentation. A 9-year-old boy presented with a complaint of low visual acuity (best corrected visual acuity: 20/80 in both eyes, refractive error: −12.00 D). Dilated fundus examination revealed multiple bilateral, sharply defined, and scalloped chorioretinal atrophy areas in the midperipheral and peripheral zone. Spectral-domain optical coherence tomography revealed bilateral cystoid macular edema in both eyes. Serum ornithine level was high (622 μmol/L). An arginine-restricted diet reduced serum ornithine level (55 μmol/L). However, visual findings including macular edema remained unchanged in 2 years of follow-up. Conclusion. Arginine-restricted diet did not improve macular edema in our patient with gyrate atrophy. A more comprehensive understanding of the underlying factors for macular edema will lead to the development of effective therapies.
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issn 2090-6722
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publishDate 2015-01-01
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series Case Reports in Ophthalmological Medicine
spelling doaj-art-28466401a5ad4b91831ee7f64b47325f2025-02-03T05:51:56ZengWileyCase Reports in Ophthalmological Medicine2090-67222090-67302015-01-01201510.1155/2015/137270137270Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate AtrophySibel Doguizi0Mehmet Ali Sekeroglu1Mustafa Alpaslan Anayol2Pelin Yilmazbas3Ulucanlar Eye Training and Research Hospital, Department of Ophthalmology, 06240 Ankara, TurkeyUlucanlar Eye Training and Research Hospital, Department of Ophthalmology, 06240 Ankara, TurkeyUlucanlar Eye Training and Research Hospital, Department of Ophthalmology, 06240 Ankara, TurkeyUlucanlar Eye Training and Research Hospital, Department of Ophthalmology, 06240 Ankara, TurkeyIntroduction. Gyrate atrophy is a rare genetical metabolic disorder affecting vision. Here, we report a 9-year-old boy with gyrate atrophy associated with bilateral macular edema at the time of diagnosis and the effect of long term metabolic control on macular edema. Case Presentation. A 9-year-old boy presented with a complaint of low visual acuity (best corrected visual acuity: 20/80 in both eyes, refractive error: −12.00 D). Dilated fundus examination revealed multiple bilateral, sharply defined, and scalloped chorioretinal atrophy areas in the midperipheral and peripheral zone. Spectral-domain optical coherence tomography revealed bilateral cystoid macular edema in both eyes. Serum ornithine level was high (622 μmol/L). An arginine-restricted diet reduced serum ornithine level (55 μmol/L). However, visual findings including macular edema remained unchanged in 2 years of follow-up. Conclusion. Arginine-restricted diet did not improve macular edema in our patient with gyrate atrophy. A more comprehensive understanding of the underlying factors for macular edema will lead to the development of effective therapies.http://dx.doi.org/10.1155/2015/137270
spellingShingle Sibel Doguizi
Mehmet Ali Sekeroglu
Mustafa Alpaslan Anayol
Pelin Yilmazbas
Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy
Case Reports in Ophthalmological Medicine
title Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy
title_full Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy
title_fullStr Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy
title_full_unstemmed Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy
title_short Arginine-Restricted Therapy Resistant Bilateral Macular Edema Associated with Gyrate Atrophy
title_sort arginine restricted therapy resistant bilateral macular edema associated with gyrate atrophy
url http://dx.doi.org/10.1155/2015/137270
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AT mehmetalisekeroglu argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy
AT mustafaalpaslananayol argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy
AT pelinyilmazbas argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy