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Risk factors for worsening morphology and visual acuity in eyes with adult-onset foveomacular vitelliform dystrophy.

PURPOSE: To explore clinical risk factors and optical coherence tomography (OCT) features associated with worse visual acuity (VA), progression of disease, choroidal neovascularization (CNV), and atrophy in eyes with adult-onset foveomacular vitelliform dystrophy (AOFVD).

DESIGN: Single-center, retrospective, observational cohort study PARTICIPANTS: Patients seen at Duke Eye Center between January 2012 and May 2023 with a diagnosis of AOFVD confirmed via OCT and fundus autofluorescence.

METHODS: Baseline and final visit images from eyes with AOFVD were examined. Disease stage was assigned, and presence of atrophy or CNV was determined. Clinical and OCT features associated with progression to atrophy and CNV were determined using t-tests and chi-square analysis. Correlation with lower VA was determined using linear regression.

OUTCOME MEASURES: Association of clinical characteristics and OCT features with worse VA, progression of disease, CNV, and atrophy as determined by independent t-tests, chi-square analysis, and linear regression (p<0.05).

RESULTS: 101 eyes (63 patients) met inclusion criteria for this study, with mean follow-up duration of 48 months (SD 31 months). 51% of eyes progressed beyond baseline staging during follow-up; among baseline stage 1 eyes, incidence of atrophy was 0.068/person-year; incidence of CNV was 0.022/person-year. Risk factors for worse final VA were baseline presence of vitreomacular traction (VMT) (p=0.006), ellipsoid zone attenuation (p=0.024), increased lesion height and width (p<0.001). Predictors of progression include diabetes mellitus (p=0.012), statin use (p=0.031), presence of hyperreflective foci (p=0.012), and increased lesion width and volume (p=0.034, p=0.040). Predictors of atrophy include the baseline presence of VMT (p=0.018), decreased choroidal thickness (p=0.027), and greater maximal height, width, and volume of the lesion (p=0.031, p=0.020, p=0.009, respectively). Lower baseline VA (p=0.031) and increased lesion volume (p=0.042) were associated with CNV.

CONCLUSIONS: Clinical and OCT imaging features at baseline may prove useful in stratifying patient risk for progression, atrophy, CNV, and worse VA. Features such as statin use, diabetes, baseline VA, and laterality should be accounted for. OCT features such as lesion size, VMT, ellipsoid zone attenuation, choroidal thickness and hyperreflective foci may impart greater risk of poor outcomes. Future prospective analysis accounting for the time to development of atrophy and CNV is needed.

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