Prominent clinical, structural, and vascular features of adult-onset foveomacular vitelliform dystrophy: a comparative study with subgroup analysis.

CLINICAL RELEVANCE: Adult-onset foveomacular vitelliform dystrophy (AFVD) is a rare, slowly progressive disorder that shares features with other macular diseases. A thorough analysis of its structural and vascular characteristics is increasingly important for understanding its aetiology and distinguishing it from others. BACKGROUND: Structural retinal changes of AFVD are well documented; the relationship between vascular alterations and disease mechanisms remains unclear. This study aimed to compare the clinical, structural, and vascular characteristics of AFVD patients and healthy controls. METHODS: This cross-sectional study included 48 patients with AFVD and 40 healthy controls. Participants underwent assessment of demographic and functional characteristics with structural and vascular density measurements, using optical coherence tomography (OCT) and OCT- angiography. Data were compared between the AFVD and control groups and among AFVD subgroups. RESULTS: The mean best-corrected visual acuity of AFVD patients was 0.29 ± 0.18 logMAR, and there was no significant difference among the subgroups. The mean central macular thickness in the AFVD group was higher than in the controls (282 ± 55 vs 251 ± 27 µm; p = 0.003). The pseudohypopyon subgroup had the highest mean central thickness (303 ± 27 µm) and lesion base measurements (1212 ± 375 µm). There was a positive correlation between age and lesion height and lesion base among AFVD patients, while there was a negative correlation between visual acuity and choroidal thickness (r =  -0.468 and r =  -0.600). Vessel density of the superficial parafovea and fovea, the deep parafovea, the outer-retinal fovea, and all choriocapillaris regions, as well as some FAZ parameters measured by OCT-A, in eyes with AFVD, were significantly lower than those in the controls. CONCLUSION: AFVD is characterised by reduced visual acuity, age-related lesion enlargement, and decreased macular and choriocapillaris vessel density. Advanced longitudinal multimodal imaging may contribute to understanding the disease aetiopathogenesis.