Widefield Fundus Autofluorescence in Gyrate Atrophy
A 16-year-old myopic girl presented with a complaint of diminished vision, especially at night. Current spectacle visual acuity (VA) was 20/60 in the right eye (OD) and 20/50 in the left eye (OS). Lensometry showed –8.25 OD and –8.75 OS. Refraction improved VA to 20/25 in both eyes with correction of –9.00 OD and –9.50 OS. Fundus exam showed well-demarcated, scalloped areas of outer retinal and inner choroidal atrophy in the retinal periphery that were hypoautofluorescent. Ornithine plasma levels were more than 10 times above the normal range (hyperornithinemia). A diagnosis of gyrate atrophy due to a deficiency of ornithine aminotransferase was made, and she was placed on a low arginine diet.
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