Choroidal Neovascularization and Extensive Macular Atrophy With Pseudodrusen: a Retrospective Comparative Case Series

Purpose: Extensive macular atrophy with pseudodrusen (EMAP) is a bilateral retinal condition characterized by symmetric macular atrophy, typically affecting patients around age 45 years. Although rare, choroidal neovascularization (CNV) can occur with EMAP and remains poorly understood. Methods: A retrospective analysis of 87 patients with EMAP was conducted. Demographics, medical history, and imaging findings were evaluated. Optical coherence tomography (OCT) and OCT angiography (OCTA) were used to identify and classify CNV. Treatments included antivascular endothelial growth factor (anti-VEGF) agents. Results: Among 87 patients, 15 eyes of 10 individuals developed CNV. The mean age was 57 years. All patients had a history of childhood rheumatic fever and prolonged benzathine penicillin use (mean duration of use 13 years). Treatments included aflibercept (7 eyes), ranibizumab (1 eye), and 1 eye treated with combination therapy with aflibercept, ranibizumab, and faricimab (mean 8 injections/eye); 3 eyes had incomplete treatment information and 3 eyes did not receive treatment. OCT and OCTA revealed type 1 CNV in 46.7% of eyes and type 2 CNV in 46.7% of eyes. Conclusions: Early CNV diagnosis is crucial for timely treatment initiation, aiming to optimize anatomic and functional outcomes. While visual responses varied, our findings indicate that anti-VEGF therapy plays a significant role in stabilizing or improving vision.