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Graefe's Archive for Clinical and Experimental Ophthalmology

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01-03-2013 | Retinal Disorders

Multimodal morphological and functional characterization of Malattia Leventinese

Authors: Giuseppe Querques, Benjamin Guigui, Nicolas Leveziel, Lea Querques, Francesco Bandello, Eric H. Souied

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 3/2013

Abstract

Background

To analyze the morphological and functional characteristics of malattia leventinese.

Methods

This was a chart review of patients with Malattia Leventinese. All patients underwent a complete ophthalmologic examination, including best-corrected visual acuity (BCVA), fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). Microperimetry and Preferential Hyperacuity Perimeter (PHP) were performed in a subset of patients.

Results

Twelve eyes of six patients were included. BCVA ranged from 20/25 to 20/200. The largest drusen were round, not radially distributed, localized in the perimacular area and around the optic disc. The smallest drusen were not round, radially distributed, mostly localized temporally to the macula. FAF revealed an intense autofluorescence of large drusen. On both FA and ICGA, large round drusen turned to hyperfluorescent in the late phase, while small radial drusen progressively decreased their fluorescence. OCT showed the large round drusen as focal or diffuse deposition of hyperreflective material between the RPE and Bruch membrane within the macula, determining focal dome-shaped or diffuse RPE elevation respectively, and the small radial drusen, which ranged from irregular slight thickening of the RPE/Bruch membrane complex to sawtooth RPE elevation. In three patients (six eyes) that underwent microperimetry and PHP, there was a good correspondence between macular sensitivity and PHP score. Functional impairment correlated topographically to sub-RPE deposition of drusenoid material.

Conclusions

In this series, large round drusen of Malattia Leventinese appeared similar to drusen in age-related macular degeneration, while small radial drusen of Malattia Leventinese shared similarities with early-onset cuticular drusen.

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Title: Multimodal morphological and functional characterization of Malattia Leventinese
Authors: Giuseppe Querques
Benjamin Guigui
Nicolas Leveziel
Lea Querques
Francesco Bandello
Eric H. Souied
Publication date: 01-03-2013
Publisher: Springer-Verlag
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 3/2013
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI: https://doi.org/10.1007/s00417-012-2106-5

At a glance: The STEP trials

Springer Medicine

Multimodal morphological and functional characterization of Malattia Leventinese

Abstract

Background

Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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