Natural Course of Adult-Onset Foveomacular Vitelliform Dystrophy: A Spectral-Domain Optical Coherence Tomography Analysis

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 mu m to 246.1 +/- 74.0 mu m, P = .004; from 277.0 +/- 80.8 mu m to 105.3 +/- 92.3 mu m, P = .001; from 2324.2 +/- 1250.3 mu m to 1751.0 +/- 858.3 mu m, 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. (Am J Ophthalmol 2011;152:304-313. (C) 2011 by Elsevier Inc. All rights reserved.)