UNRAVELED FRINGE-LIKE MARGINS AND BIPHASIC AUTOFLUORESCENCE OF UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS

摘要： To illustrate the features of unilateral retinal pigment epithelium dysgenesis (URPED) in an African-American male patient. 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 mm from the foveola. The dark portion of the lesion was grey-black and demonstrated homogeneous hypoauto?uorescence, particularly at the site of grey-white peripheral fringe of ?brous metaplasia. By contrast, there was an additional, subtle lacey arrangement of normal-appearing RPE traversing over the entire lesion demonstrating isoauto?uorescence. On ?uorescein angiography, the lesion was generally hypo?uorescent, particularly in the dark portion of the lesion, but the peripheral fringe of ?brous metaplasia displayed angiographic hyper?uorescent staining, and the subtle lacey normal RPE showed iso?uorescence. 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. In this case, URPED demonstrated biphasic auto?uorescence implying RPE dysfunction in the hypoauto?uorescent area and partial RPE function in the lacey isoauto?uorescent region.