Visual Outcomes and Clinical Features of Extensive Macular Atrophy with Pseudodrusen (EMAP).

Abstract

Purpose: To describe extensive macular atrophy with pseudodrusen (EMAP) using multimodal imaging and analyze factors associated with visual loss.

Methods: This observational case seriesretrospectively reviewed the clinical and multimodal imaging (color fundus photography [CFP], fluorescein angiography, fundus autofluorescence (FAF), and optical coherence tomography [OCT]) of patients between January and December 2023. The EMAP diagnosis was based on early-onset macular atrophy, pseudodrusen, and bilateral disease. Univariate and multivariate Tobit regression models identified and ranked imaging findings and risk factors (P<0.05) for visual loss.

Results: Seventy-four patients (52.7% women) were diagnosed with EMAP; 86.5% had a history of rheumatic fever (RF), and nearly 17% had heart valve disease. Mean age at onset was 58.7 years (range, 43 -73 years), and the mean best-corrected visual acuity (VA) was 20/60 (range, 20/800-20/20). CFP or FAF identified pseudodrusen in 91.7% and 93.0% of eyes, respectively, and OCT did so in all evaluable patients. FAF revealed a hyperautofluorescent edge around the hypoautofluorescent central lesion in 86.6% of eyes. OCT showed a diffuse pseudodrusen pattern in 88.9%, outer retinal loss/thinning in 80.8% and macular neovascularization in 9.4% of eyes. Subfoveal choroidal thickness (P=0.004), outer retinal loss/thinning (P<0.01), and preserved fovea (P<0.01) were associated significantly with VA. Visual loss was associated with younger age at diagnosis (P=0.04) and co-existence of RF and heart valve disease (P=0.006).

Conclusions: Early diagnosis and treatment of RF complications may be related to EMAP progression and outcomes. Longitudinal studies should characterize the natural history of EMAP.