In Vivo Visualization of Variable Photoreceptor Alteration in a Case of Peripapillary Congenital Hypertrophy of the Retinal Pigment Epithelium Using Spectralis ® High Magnification Module

Purpose: To analyze using the high magnification module (HMM) a case of peripapillary congenital hypertrophy of retinal pigment epithelium (CHRPE) and to correlate the findings to multimodal imaging and swept-source optical coherence tomography angiography (SS-OCTA, PLEX® Elite 9000, Carl Zeiss Meditec, Dublin, California, USA) imaging.

Observations: A 57-year-old Caucasian woman presenting a peripapillary CHRPE of the left eye (LE) was examined using HMM and SS-OCTA, in addition to multimodal imaging. SS-OCTA disclosed the gradual changes, with four distinguishable zones: Zone 1 with complete outer retinal and retinal pigment epithelium (RPE) atrophy, Zones 2 and 3, corresponding to incomplete outer retinal (and RPE) atrophy presenting increased flow deficits, and normal choriocapillaris outside the lesion (Zone 4). High Magnification Module (HMM, Spectralis ®, Heidelberg Engineering) showed small polygonal hyperreflective outlines over the pigmented parts of the lesion (Zone 2), and partly over the narrow halo surrounding the lesion (Zone 3), with an absence of these outlines over the lacunae (Zone 1).

Conclusions And Importance: HMM is a non-invasive imaging modality, allowing the in vivo visualization of a mosaic pattern, corresponding to the hyperreflective polygonal outlines, or absence thereof, in different zones of CHRPE.