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. 2022 Feb 1;63(2):25.
doi: 10.1167/iovs.63.2.25.

Choroidal Caverns in Stargardt Disease

Dario Pasquale Mucciolo  1   2 , Dario Giorgio  1 , Myrta Lippera  1 , Valeria Dattilo  1 , Ilaria Passerini  3 , Elisabetta Pelo  3 , Andrea Sodi  1 , Gianni Virgili  1   4 , Fabrizio Giansanti  1 , Vittoria Murro  1
Affiliations

Choroidal Caverns in Stargardt Disease

Dario Pasquale Mucciolo et al. Invest Ophthalmol Vis Sci. .

Abstract

Purpose: To report choroidal caverns in patients affected by recessive Stargardt disease (STGD1) and to investigate its clinical features.

Methods: Retrospective analysis of STGD1 patients recruited at the Regional Reference Center for Hereditary Retinal Degenerations at the Eye Clinic in Florence from 2012 to 2017. Patients included in the study underwent a complete ophthalmic examination including best-corrected visual acuity, color fundus photography, fundus autofluorescence, optical coherence tomography (OCT) and OCT angiography.

Results: Eighty-six patients (172 eyes) were included in the study. Twenty-three eyes (13.3%) of 21 patients presented choroidal caverns. The total number of detected choroidal caverns was 63. Choroidal caverns were only present in patients with stage III and IV STGD. Interestingly, patients with choroidal caverns presented larger macular atrophy (20.53 ± 16.9 mm2 vs. 18.11 ± 20.39 mm2), worse visual acuity (1.03 ± 0.29 vs. 0.83 ± 0.26), and a thinner choroidal thickness (245.9 ± 88.7 vs. 266.0 ± 110.5 µm).

Conclusions: Choroidal caverns are present only in the advanced stage of STGD1, and a possible degenerative origin of the finding has been hypothesized.

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Conflict of interest statement

Disclosure: D.P. Mucciolo, None; D. Giorgio, None; M. Lippera, None; V. Dattilo, None; I. Passerini, None; E. Pelo, None; A. Sodi, None; G. Virgili, None; F. Giansanti, None; V. Murro, None

Figures

Figure.
Figure.
(A) Wide-field color fundus photograph of the left eye of a patient affected by STGD1. (B) OCT scan showing choroidal caverns (arrowhead). (C) Fundus autofluorescence imaging characterized by a wide central hypoautofluorescent area surrounded by a speckled FAF response. (D) OCT-A examination does not reveal any flow signal inside the choroidal cavern lesion.
See this image and copyright information in PMC

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