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Pier Luigi Grenga, Stefano Lupo, Marco Marenco, Alessandro Meduri, Serena Fragiotta, Enzo M. Vingolo; Morphological features in Adult Onset Foveomacular Vitelliform Dystrophy (AOFVD). Invest. Ophthalmol. Vis. Sci. 2011;52(14):1048.
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to describe morphological features in adult onset foveomacular vitelliform dystrophy (AOVFD) using spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA) and fundus autofluorescence (FAF).
in this prospective observational case series complete ophthalmologic examination including SD-OCT, FA and FAF (Heidelberg Engineering, Heidelberg, Germany) was performed in two patients (4 eyes) with AOVFD. The diagnosis of the disease was confirmed by normal or subnormal electrooculogram (EOG).
SD-OCT showed the vitelliform material as a highly reflective dome-shaped lesion located between the photoreceptor layer and RPE in 3 eyes, one of that with hyper reflective clumps within the outer nuclear layers. The fourth eye showed the atrophic stage of AOVFD with atrophy of outer plexiform and outer nuclear layers and IS/OS interface and hyper reflective clumps in the remaining outer layers. FAF showed hyper autofluorescence in the 3 eyes with vitelliform lesion, hypoautofluorescence in the atrophic stage. FA showed central hypofluorescence surrounded by hyperfluorescence ring in the 3 eyes with vitelliform lesion; hyperfluorescent spots due to window defect in the atrophic one.
our cases confirm hypothesis from previous study that early changes involve the layer between RPE and the IS/OS interface, and the evolution of the disease is to an atrophic stage with severe reduction of BCVA. SD-OCT associated with FA and EOG is mandatory for the diagnosis of AOVFD.
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