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D. C. Ferrara, D. Calucci, R. Jorge, J. L. Orefice, R. A. Costa; Combined Fundus Imaging in Best's Disease: A Cross-Sectional Study. Invest. Ophthalmol. Vis. Sci. 2009;50(13):3286.
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To investigate morphological features in Best’s disease using combined fundus imaging evaluations.
The median age of patients and controls was 35 years (range, 4 to 61) and 36 years (range, 5 to 63), respectively. Fusiform thickening of the middle highly reflective layer (HRL) at the outer foveal retina, with no other apparent fundus findings, was observed both eyes of 2 children. In vitelliform stage (2 eyes), a dome-shaped lesion encompassing the outer (RPE/Bruch’s membrane complex) and middle HRLs with increased FAF was observed. In pseudo-hypopion stage (2 eyes), hyperreflective material was observed just externally to the inner HRL (OS/IS photoreceptors’ junction) and gravitationally displaced. In vitelliruptive stage (8 eyes), three new features were observed: absence of reflective signals from photoreceptors’ layer, focal disruptions of the ELM with subjacent deposition of hyperreflective material just above the outer HRL, and fusiform thickening of the outer HRL. The thickness of the ONL at the macula was decreased in all patients with clinically evident lesions.
Morphological features demonstrated on SOCT suggest that the initial event in Best’s disease may be the accumulation of material between photoreceptors and RPE. While it accumulates, the resultant dome-shaped lesion shows increased FAF and decrease in the thickness of the ONL becomes apparent. Progreesive enlargement is associated with the formation of a hyporeflective space within the lesion, and gravitational displacement of the autofluorescent material. At later stages, ultimate disappearance of the photoreceptor layer and focal disruption of the ELM were seen; at this point, two associated features may be observed, named choroidal neovascularization and RPE metaplasia.
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