UNRAVELED FRINGE-LIKE MARGINS AND BIPHASIC AUTOFLUORESCENCE OF UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS. Issue Volume 15:Issues 6(2021) (November 2021)

• Record Type: Journal Article
• Authors: Florakis, Eleni; Ancona-Lezama, David; Shields, Carol L.
• Is Part Of: Retinal cases & brief reports. Volume 15:Issues 6(2021)
• Publication Date: 2021-11
• Subjects: retinal pigment epithelium -- dysgenesis -- autofluorescence -- optical coherence tomography -- race -- African-American; Retina -- Diseases -- Periodicals; Retina -- Periodicals; Retinal Diseases -- Periodicals; Retina -- Case Reports; Retinal Diseases -- Case Reports; 617.7
• DOI: 10.1097/ICB.0000000000000880
• Languages: English
• ISSNs: 1935-1089

Abstract

Abstract : Purpose: To illustrate the features of unilateral retinal pigment epithelium dysgenesis (URPED) in an African-American male patient. Methods: Case report. Results: A 47-year-old asymptomatic African-American man was referred for an atypical subretinal pigmented mass in the left eye. On examination, visual acuity was 20/20 in both eyes. The right eye was unremarkable. The left eye revealed a darkly pigmented grey-black lesion at the level of the RPE with irregular, unraveled fringe-like margins, consistent with URPED. The lesion measured 5 mm in basal dimension and was located 400 μ m from the foveola. The dark portion of the lesion was grey-black and demonstrated homogeneous hypoautofluorescence, particularly at the site of grey-white peripheral fringe of fibrous metaplasia. By contrast, there was an additional, subtle lacey arrangement of normal-appearing RPE traversing over the entire lesion demonstrating isoautofluorescence. On fluorescein angiography, the lesion was generally hypofluorescent, particularly in the dark portion of the lesion, but the peripheral fringe of fibrous metaplasia displayed angiographic hyperfluorescent staining, and the subtle lacey normal RPE showed isofluorescence. Optical coherence tomography demonstrated RPE hyperplasia and shallow RPE detachment interspersed with normal-appearing RPE and thinning of outer retina and preservation of the foveola and choroid. Conclusion: In this case, URPED demonstrated biphasic autofluorescence implying RPE dysfunction in the hypoautofluorescent area and partial RPE function in the lacey isoautofluorescent region. Abstract : Unilateral retinal pigment epithelium dysgenesis is a focal developmental abnormality of the retinal pigment epithelium, appearing as a dark, flat mass in the macula or paramacular region with irregular unraveled fringe-like margins. In this article, we illustrate lacey curvilinear preservation of autofluorescence in a darkly hypoautofluorescent lesion.