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Graefe's Archive for Clinical and Experimental Ophthalmology

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01-02-2016 | Retinal Disorders

Retinal structure in young patients aged 10 years or less with Best vitelliform macular dystrophy

Authors: Patrik Schatz, Dror Sharon, Sermed Al-Hamdani, Sten Andréasson, Michael Larsen

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 2/2016

Abstract

Purpose

Our aim was to analyze retinal structure in young patients with Best disease with reference to future gene therapy.

Methods

This was a retrospective observational spectral domain optical coherence tomography study of four patients aged 10 years or less with Best disease.

Results

Findings ranged from subtle thickening at the level of the retinal pigment epithelium-photoreceptor interdigitation line, to subretinal fluid and precipitate-like changes at the level of the photoreceptor outer segments, and further to choroidal neovascularization. The photoreceptor inner segment ellipsoid layer could be visualized seemingly undisturbed above the vitelliform lesions, except in the case of choroidal neovascularization.

Conclusions

Clinical variability is evident even among young patients aged 10 years or less with Best disease. The earliest structural alterations seem to occur at the level of the retinal pigment epithelium-photoreceptor interdigitation line. The photoreceptor inner segment seems to be unaffected unless choroidal neovascularization develops, which seems promising regarding future gene therapy.

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Title: Retinal structure in young patients aged 10 years or less with Best vitelliform macular dystrophy
Authors: Patrik Schatz
Dror Sharon
Sermed Al-Hamdani
Sten Andréasson
Michael Larsen
Publication date: 01-02-2016
Publisher: Springer Berlin Heidelberg
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 2/2016
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI: https://doi.org/10.1007/s00417-015-3025-z

At a glance: The STEP trials

Springer Medicine

Retinal structure in young patients aged 10 years or less with Best vitelliform macular dystrophy

Abstract

Purpose

Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

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