A CASE OF UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS

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Abstract

Purpose:

To report a case of unilateral retinal pigment epithelium dysgenesis.

Methods:

An 8-year-old boy with a large grayish lesion and leopard-spot pattern lesion at its periphery in his left eye underwent fluorescein angiography, fundus autofluorescence imaging, optical coherence tomography, and electroretinography.

Results:

Fluorescein angiography showed an area of geographic hyperfluorescence with a peripheral pattern of dark spots. The area of retinal pigment epithelial atrophy showed hypofluorescence on fundus autofluorescence. Optical coherence tomography of the left eye showed attenuation of the inner segment–outer segment junction and choroidal thinning. Single-flash electroretinography and scotopic electroretinography showed normal results and did not differ between both the eyes. The 30-Hz flicker test and photopic electroretinography showed a decrease in amplitude in the left eye.

Conclusion:

Unilateral retinal pigment epithelium dysgenesis is very rare, and its prognosis is still unknown. Careful follow-up of the patient seems to be essential.

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