Visual outcomes and clinical features of Extensive Macular Atrophy with Pseudodrusen (EMAP)

Abstract

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

Methods: This observational case series retrospectively reviewed the clinical and multimodal imaging (color fundus photography, fluorescein angiography, fundus autofluorescence, and optical coherence tomography) of patients between January and December 2023. The extensive macular atrophy with pseudodrusen 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 extensive macular atrophy with pseudodrusen; 86.5% had a history of rheumatic fever, 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 was 20/60 (range, 20/800-20/20). Color fundus photography or fundus autofluorescence identified pseudodrusen in 91.7% and 93.0% of eyes, respectively, and optical coherence tomography did so in all evaluable patients. Fundus autofluorescence revealed a hyperautofluorescent edge around the hypoautofluorescent central lesion in 86.6% of eyes. Optical coherence tomography 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 visual acuity. Visual loss was associated with younger age at diagnosis ( P = 0.04) and coexistence of rheumatic fever and heart valve disease ( P = 0.006).

Conclusion: Early diagnosis and treatment of rheumatic fever complications may be related to extensive macular atrophy with pseudodrusen progression and outcomes. Longitudinal studies should characterize the natural history of extensive macular atrophy with pseudodrusen.