A Pigmented Fundus Lesion with Pseudohypopyon and Subretinal Fluid Resembling Unilateral Retinal Pigment Epithelium Dysgenesis (URPED).

Abstract

To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium (RPE). Case report. A 36-year-old female presented with photopsia in her left eye. Funduscopy revealed an 8 × 7 mm, dark brown lesion at the level of the RPE inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular RPE. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During three years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion. Multimodal imaging findings of a solitary pigmented RPE lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.