VITELLIFORM LESIONS ASSOCIATED WITH ANGIOID STREAKS: Long-Term Follow-Up of a Rarely Described Phenotype

To describe the clinical and multimodal imaging features, and long-term outcomes, of acquired vitelliform lesions (AVLs) in angioid streaks (AS).

Retrospective case series including 14 patients (23 eyes) with AS-related AVLs. Clinical data, color fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (OCT), en face OCT, and OCT angiography were evaluated at baseline and final visits. Snellen visual acuity (VA), lesion dimensions, subfoveal choroidal thickness (SFCT), and outer retinal integrity were recorded.

AS were secondary to pseudoxanthoma elasticum in 64.3% and idiopathic in 35.7%. Baseline VA was 0.18 ± 0.17 LogMAR (20/30) and declined to 0.43 ± 0.33 LogMAR (20/50) over a mean follow-up of 77 months (p<0.001). AVLs were often foveal (78.3%), multifocal (82.6%), and peripapillary (73.9%), with OCT detecting subretinal hyperreflective material in all eyes. Both lesion width and SFCT decreased over time. Complete retinal pigment epithelium (RPE) and outer retinal atrophy increased from 17.4% to 69.6%, and exudative choroidal neovascularization developed in 26.1%.

AS-related AVLs represent a rare phenotype reflecting multifactorial pathogenesis involving Bruch's membrane alterations and RPE dysfunction. Over prolonged follow-up, lesion size decreased, yet progressive retinal atrophy led to significant vision loss. Further research is warranted to clarify disease progression and optimize treatment approaches.