Título

TOPICAL NONSTEROIDAL ANTIINFLAMMATORY DRUGS FOR THE TREATMENT OF CYSTOID MACULAR EDEMA IN GYRATE ATROPHY

Introdução / Purpose

Gyrate atrophy is a rare genetic and progressive disorder affecting vision, due to ornithine aminotransferase (OAT) enzyme deficiency. This report describes a child with gyrate atrophy associated with bilateral cystoid macular edema (CME) at the time of diagnosis, confirmed by coherence tomography angiography (OCTA), which improved CME with topical non-steroid anti-inflammatory drug (NSAID).

Material e Método / Methods

A 6year-old girl complains of low visual acuity (best corrected VA was 20/60 in both eyes, refractive error: – 4.5D). Anterior segment was normal bilaterally. Dilated fundus examination revealed multiple bilateral, sharply defined, and scalloped chorioretinal atrophy areas in the midperipheral and peripheral zone. Spectral-domain optical coherence tomography (SD OCT) revealed bilateral CME in both eye and OCTA showed no sign of neovascularization. Serum ornithine level (990

Resultados / Results

The mechanism of the CME is still unclear. OCT examination is critical for diagnosing CME, even with a clinically normal macula. OCTA can help monitoring vascular changes in GA patients with a non-invasive imaging technique. It was seen that NSAIDs are effective to control CME, especially with the advantage of being a non-invasive treatment.

Discussão e Conclusões / Discussion

In this way, continued follow-up of these patients may provide further insights into the development of new and improved methods of treatment.

Palavras Chave

gyrate atrophy;cystoid macular edema;non-steroid anti-inflammatory drug;coherence tomography angiography