Abstract
Purpose: Gyrate atrophy (GA) of the fundus is a rare, progressive metabolic disease secondary to the deficiency of the pyridoxal phosphate-dependent enzyme, ornithine aminotransferase. GA may lead to cystoid macular edema (CME) resulting from chronic inflammation. We aimed to report a child case with CME secondary to gyrate atrophy. Methods: Herein we presented a GA case treated with posterior sub-Tenon triamcinolone acetonide injection. Results: Optical coherence tomography examination revealed the disappearance of the macular edema that is a vision-threatening complication in GA. Conclusion: The present case showed that the posterior sub-Tenon injection of long acting steroids might be a promising treatment in CME secondary to GA.
Highlights
Gyrate atrophy (GA) of the fundus is a rare, progressive metabolic disorder secondary to the deficiency of the pyridoxal phosphate-dependent enzyme, ornithine aminotransferase (OAT) [1]
We aimed to present a child with cystoid macular edema (CME) secondary to gyrate atrophy treated with injection of subTenon triamcinolone acetonide (TA)
To the best of our knowledge, this is the first case report of CME secondary to GA treated with sub-Tenon TA
Summary
Gyrate atrophy (GA) of the fundus is a rare, progressive metabolic disorder secondary to the deficiency of the pyridoxal phosphate-dependent enzyme, ornithine aminotransferase (OAT) [1]. Deficiency in OAT leads to 10 to 20-fold higher plasma ornithine levels than those of controls [2]. The sharply demarcated, round shaped chorioretinal atrophy with pigmented margins in the peripheral retina is the pathognomonic finding of GA. The patients with GA complain with visual deterioration and loss of night vision due to retinal atrophy, cataract formation, or cystoid macular edema (CME). Imaging provides more information about macular edema in GA [3]. We aimed to present a child with CME secondary to gyrate atrophy treated with injection of subTenon triamcinolone acetonide (TA)
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