Purpose: To describe novel microperimetry and imaging findings in two patients affected by extensive macular atrophy with pseudodrusen-like appearance without signs of retinal pigment epithelium atrophy.
Methods: Case series. Both patients underwent mesopic and dark-adapted two-color scotopic microperimetry, followed by multimodal imaging assessment including ultra-widefield photography, fundus autofluorescence, high-resolution optical coherence tomography, optical coherence tomography angiography, and high-magnification module.
Results: Albeit normal visual acuity, both patients had a significant reduction of retinal sensitivity-especially under scotopic cyan conditions. One patient had macular pigment abnormalities, whereas the combination of blue and near-infrared autofluorescence modalities highlighted different patterns of pseudodrusen-like lesions.Of notice, high-resolution optical coherence tomography revealed a marked separation between the retinal pigment epithelium and Bruch membrane, containing a hyperreflective material with two different reflectivities. Optical coherence tomography angiography excluded the presence of macular neovascularization and documented several choriocapillaris flow voids. High-magnification module images showed severe alteration of photoreceptors' mosaic in the perifovea.
Conclusion: Our comprehensive assessment of two stage 1 extensive macular atrophy with pseudodrusen-like appearance patients revealed a predominant damage of perifoveal rods over areas of retinal pigment epithelium-Bruch membrane separation. These findings underscore the importance of basal laminar deposits in the initial stages of extensive macular atrophy with pseudodrusen-like appearance, contributing to a deeper understanding of its underlying mechanisms.