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Itay Chowers, Liran Tiosano, Michelle Grunin, Shira Hagbi-Levi, Eyal Banin, Edward Averbukh; Long Term Follow Up of Sporadic Adult-Onset Foveomacular Vitelliform Dystrophy. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):3845. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
Adult-Onset Foveomacular Vitelliform Dystrophy (AFVD) is a slowly progressing macular degeneration. The aim of this study was to evaluate the long-term course of sporadic AFVD.
Retrospective cohort study from a single retina clinic including a consecutive group of 95 eyes of 51 patients (25 male and 26 female) diagnosed with AFVD was performed. Demographics and clinical characteristics were recorded, and SD-OCT features were analyzed.
The mean age (±SD) was 73.8±10.7 years (range 51-93 years). Mean (±SD) follow up period was 30.4±16.3 months (range 15-44 months). During 36 months, the LogMAR best corrected visual acuity (BCVA) (±SD) deteriorated from 0.27±0.35 to 0.38±0.35 (n=53 eyes; p=0.02). At baseline, 51 of the 95 eyes (53.7%) manifested the vitelliform stage while only 10 eyes of 53 eyes(18.9%) which were followed for at least three years remained at this stage at 36 months (p=0.02). LogMAR BCVA at presentation was 0.19±0.18 for eyes with an intact ellipsoid zone on SD-OCT, 0.38±0.4 for eyes with a disrupted line, and 0.55±0.42 for eyes in which the ellipsoid zone is absent (p=0.012). Ellipsoid zone alterations also progressed during follow-up and showed correlation with BCVA (Pearson correlation coefficient = 0.7, p=0.03). Drusen were observed in a quarter of cases and were not associated with outcome.<br />
This long term follow-up study suggests that sporadic AFVD is a slowly progressing disorder associated with approximately one ETDRS line loss every 3 years. While age of onset for sporadic AFVD is higher than described for monogenic AFVD, the course of visual loss and lesion progression are similar.
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