PURPOSE:: To describe retinal pigment epithelium (RPE) aperture and to generate hypotheses about pathogenesis of this previously unreported finding in the evolution of avascular pigment epithelium detachment (PED) secondary to age-related macular degeneration. METHODS:: Medical records and multimodal imaging results from 10 patients with RPE apertures were reviewed between January 2009 and December 2014 by 2 institutions. Main outcome measures were analysis of RPE aperture imaging characteristics, including aperture areas and PED diameters, and their temporal course. Lesions preceding RPE aperture development were also evaluated. RESULTS:: Eleven RPE apertures were identified in 10 eyes of 10 patients (1 male, 9 females; mean age 73.1 ± 6.7 years) and included for analysis. The RPE apertures appeared as round discontinuities either at the apex or at the base of avascular PED. No rippling or retraction of the RPE was found at the sites of aperture. The RPE apertures enlarged homogeneously (mean round area of hypoautofluorescence significantly increased from 0.18 ± 0.13 to 0.93 ± 1.2; P = 0.005), and PED flattened (PED maximal height on spectral domain optical coherence significantly decreased from 445.2 ± 259 to 206.4 ± 218; P = 0.04) after a mean of 38.6 ± 16.3 months. Analysis of lesions preceding RPE apertures revealed areas of focal hyperautofluorescence at the site of development, in some cases appearing as drusenoid material connected with the base of avascular PED. CONCLUSION:: The RPE aperture represents a previously unreported possible evolution of avascular PED, which should be distinguished by typical RPE tears. Analysis of lesions preceding RPE apertures suggests focal atrophic progression of drusenoid material in its pathogenesis.
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