Natural course of adult-onset foveomacular vitelliform dystrophy: a spectral-domain optical coherence tomography analysis

Abstract

Purpose: To describe the natural course of adult-onset foveomacular vitelliform dystrophy using spectral-domain optical coherence tomography (SD-OCT).

Design: Retrospective study.

Methods: We reviewed the charts of all consecutive patients with adult-onset foveomacular vitelliform dystrophy who underwent SD-OCT at baseline and at least 12 months later (last visit). Main outcome measures were changes of clinical and SD-OCT features over time.

Results: Forty-six eyes (31 patients, 15 male and 16 female; mean age 74.6 ± 8.2 years) were included. Follow-up was 16.2 ± 6 (range, 12-30) months. Visual acuity (VA) reduced from 0.32 ± 0.22 logMAR at baseline to 0.39 ± 0.28 logMAR at last visit (P=.03). The stage of the disease was vitelliform in 28 eyes (60.8%), pseudohypopyon in 7 eyes (15.2%), vitelliruptive in 11 eyes (23.9%) at baseline; vitelliform in 23 eyes (50%), pseudohypopyon in 5 eyes (10.9%), vitelliruptive in 13 eyes (28.2%), and atrophic in 5 eyes (10.9%) at last visit. Stabilization of the disease stage, inner segment/outer segment (IS/OS) interface status, and lesion reflectivity on SD-OCT determined no VA changes (P>.05), while their worsening determined a reduction of VA (P=.03). In eyes that presented a progression of the disease stage, mean central macular thickness, maximal thickness of the lesion, and maximal width of the lesion showed a significant change (from 404.1 ± 107.6 μm to 246.1 ± 74.0 μm, P = .004; from 277.0 ± 80.8 μm to 105.3 ± 92.3 μm, P=.001; from 2324.2 ± 1250.3 μm to 1751.0 ± 858.3 μm, P = .04, respectively).

Conclusions: In adult-onset foveomacular vitelliform dystrophy, progression of the lesion stage (partial/complete resorption of the material) is generally accompanied by IS/OS interface disruption/loss and visual impairment.