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Liran Tiosano, Edward Averbukh, Itay Chowers; Multimodal imaging evaluation of the pseudohypopyon stage in Adult-Onset Foveomacular Vitelliform Dystrophy. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2700.
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© 2017 Association for Research in Vision and Ophthalmology.
A pseudohypopyon stage (PHS) was described in Adult-Onset Foveomacular Vitelliform Dystrophy (AFVD). At this stage, optical coherence tomography (OCT) demonstrate a lesion that is composed of hyperreflective material that was described to settle in the inferior part of the lesion and a hyporeflective material at the superior part. We sought to characterize this stage of the disease.
Clinical and multimodal imaging database including 126 AFVD eyes (n= 68 patients) was evaluated to identify the PHS. All patients in the database were previously genotyped negative for mutations in genes previously associated with AFVD (PRPH2, BEST1, IMPG-1, and IMPG-2). Demographics, clinical characteristics, SD-OCT findings, color and fundus autofluorescence findings and microperimetry studies were retrospectively analyzed.
Seven eyes (6 patients; male/female=3/3) harboring PHS, follow-up was 27±7.2 months (range: 18-32). All eyes remained in the PHS during the follow-up. In SD-OCT, hyperreflective material was demonstrated between the retinal pigmented epithelium and the ellipsoid zone in the temporal aspect of the lesion while hyporeflective material was evident nasally in six eyes; one eye had hyporeflective material in the superior lesion portion (P=0.01 for vertical vs. lateral distribution of lesion material). Ellipsoid zone remained intact above the hyperreflective area in 6 eyes, and disrupted above the hyporeflective area in 6 eyes. Mean±SD lesion size (width and height) at baseline were 1518±498 and 212±60 micron, respectively, and 1404±364 and 172±52 micron at the last visit (P=NS). The mean best corrected visual acuity (BCVA) at presentation and at last follow-up was 0.20±0.25 LogMAR and 0.26±0.19 LogMAR, respectively (P=0.014). Microperimetry demonstrated better retinal sensitivity over the hyperreflective compared with the hyporeflective lesion area. FAF demonstrated intense hyperautofluorescence over the hyperreflective area.
PHS in AFVD is uncommon. It composes a relatively large lesion where the degenerative process is usually initiated at the nasal side, and does not respect gravity. This process is accompanied by altered retinal function manifested by decreased sensitivity and progressive visual loss at a mean the rate of less than one ETDRS line during 2 years.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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