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Case Reports in Ophthalmological Medicine
Volume 2015, Article ID 137270, 3 pages
http://dx.doi.org/10.1155/2015/137270
Case Report

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

Ulucanlar Eye Training and Research Hospital, Department of Ophthalmology, 06240 Ankara, Turkey

Received 24 October 2015; Accepted 23 November 2015

Academic Editor: Takaaki Hayashi

Copyright © 2015 Sibel Doguizi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.