Abstract
Introduction: This study is aimed at describing a patient with unilateral retinal pigment epithelium dysgenesis (URPED) using multimodal retinal imaging combined with ocular microperimetry and multifocal electroretinogram (ERG) analysis. Case presentation: A 56-year-old healthy male was referred for a routine ophthalmologic control. His best corrected visual acuity was 20/20 and 20/25 in the right and left eye, respectively. Fundus examination of the left eye revealed a well-circumscribed, large yellowish-white lesion on the posterior pole that extended from the peripapillary region to the inferior temporal vascular arcade, sparing the fovea. This characteristic fundus picture led us to the diagnosis of URPED. Microperimetry demonstrated a progressive decrease of sensitivity from the normal retina toward the lesion borders, reaching a value of 0 dB at its center. Multifocal ERG displayed a reduction of central amplitudes in the involved eye. Discussion: Our findings indicate a varied degree of sensitivity at the site of the lesion. Despite good visual acuity, multifocal ERG revealed reduced macular function.