A Common Finding in Foveal-Sparing Extensive Macular Atrophy With Pseudodrusen Implicates Basal Laminar Deposits

Abstract

Purpose: To characterize structural and clinical alterations preceding the diffuse macular atrophy in extensive macular atrophy with pseudodrusen (EMAP) and their evolution toward atrophic changes.Methods: A retrospective chart review was performed of patients with early-onset reticular pseudodrusen (i.e., pre-EMAP) younger than 55 years and EMAP with foveal sparing. Patients were included if they had complete medical records and multimodal imaging.Results: A total of 12 patients were reviewed, of whom 4 of 12 patients (7 eyes) presented a pre-EMAP stage, characterized by the presence of pseudodrusen-like deposits without atrophic changes, while the remaining 8 of 12 patients (10 eyes) exhibited EMAP with foveal sparing (60.1 ± 6.4 years). Subretinal deposits of various stages tended to fade, leaving subretinal pigment epithelium accumulation of hyperreflective material with a physical separation between the retinal pigment epithelium-basal lamina and the Bruch membrane, along with the persistence of hyperreflective material after retinal pigment epithelium loss. These findings preceded atrophy development in a pre-EMAP stage and the EMAP stage with foveal sparing.Conclusion: Our findings presented distinct multimodal imaging features in eyes with reticular pseudodrusen depicting a peculiar phenotype of rapidly progressing atrophy in midlife. The disease spectrum may include other forms of geographic atrophy allied by thickened basal laminar deposits.

Natural Course and Classification of Extensive Macular Atrophy With Pseudodrusen-Like Appearance

“…Indeed, knowledge regarding the early retinal and functional alterations occurring in the initial stage of EMAP is currently limited in the literature and deserves further in-depth studies. 48 In conclusion, our study prospectively investigated the structural and spatial progression of RPE atrophy in EMAP patients. Based on our extensive analyses, we propose the first disease classification.…”

“…Indeed, knowledge regarding the early retinal and functional alterations occurring in the initial stage of EMAP is currently limited in the literature and deserves further in-depth studies. 48…”

Subretinal Lipid Globules an Early Biomarker of Macular Neovascularization in Eyes With Intermediate Age-Related Macular Degeneration

“…As recently demonstrated, this finding may result from a non-neovascular splitting of the RPE/Bruch membrane complex representing thick basal laminar deposits on OCT B-scans. 27,28 Despite a higher specificity compared with the DLS, the diagnostic performance of SLGs as a biomarker for NE-MNV appears inferior to the DLS, as confirmed by Youden's index. Moreover, SLGs and DLS cannot be considered interchangeably, as they are more likely complementary predictors in NE-MNV.…”

Identifying geographic atrophy

“…Reticular pseudodrusen (RPD), also referred to as subretinal drusenoid deposits (SDDs), are differentiated from conventional drusen in that they are located above the RPE [23 ▪▪ ,25]. They are often difficult to visualize on clinical examination but can be more apparent with the use of fundus autofluorescence (FAF), near-infrared reflectance (NIR), and OCT [26 ▪ ].…”

“…A rare and aggressive variant of atrophic AMD referred to as extensive macular atrophy with pseudodrusen (EMAP) has been described as early-onset (<60 years), bilateral, and severe macular atrophy associated with numerous deposits resembling reticular pseudodrusen which extend towards the midperiphery and are accompanied by peripheral pavingstone degeneration [25,29]. The differentiating factor between EMAP as compared to the reticular pseudodrusen found in AMD is that patients with EMAP do not exhibit the same cardiovascular risk factors which are found in AMD patients [25]. It is suggested that RPD/SDDs should be included in updated AMD classification systems, risk calculators, and clinical trials.…”