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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Wiley
2015-01-01
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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. |
format | Article |
id | doaj-art-28466401a5ad4b91831ee7f64b47325f |
institution | Kabale University |
issn | 2090-6722 2090-6730 |
language | English |
publishDate | 2015-01-01 |
publisher | Wiley |
record_format | Article |
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 |
work_keys_str_mv | AT sibeldoguizi argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy AT mehmetalisekeroglu argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy AT mustafaalpaslananayol argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy AT pelinyilmazbas argininerestrictedtherapyresistantbilateralmacularedemaassociatedwithgyrateatrophy |