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.
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.