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Multimodal Chorioretinal Imaging in Erdheim-Chester Disease

Authors Sacconi R, Campochiaro C, Rabiolo A, Marchese A, Tomelleri A, Tomasso L, Cicinelli MV, Querques L, Bandello F, Dagna L, Querques G

Received 25 July 2019

Accepted for publication 19 September 2019

Published 28 February 2020 Volume 2020:14 Pages 581—588


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Scott Fraser

Riccardo Sacconi,1,* Corrado Campochiaro,2,* Alessandro Rabiolo,1,* Alessandro Marchese,1 Alessandro Tomelleri,2 Livia Tomasso,1 Maria Vittoria Cicinelli,1 Lea Querques,1 Francesco Bandello,1 Lorenzo Dagna,2,* Giuseppe Querques1,*

1Department of Ophthalmology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Milan, Italy; 2Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy

*These authors contributed equally to this work

Correspondence: Giuseppe Querques
Department of Ophthalmology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, Milan 20132, Italy
Tel +39 0226432648
Fax +39 0226433643

Purpose: To analyze the subclinical intraocular involvement using multimodal imaging approach in patients affected by Erdheim-Chester disease (ECD) without ocular symptoms.
Patients and Methods: In this prospective cross-sectional study, 18 eyes of 9 consecutive patients with ECD were enrolled. Each patient underwent comprehensive ocular examination and extensive multimodal chorioretinal imaging.
Results: None of the patients presented any evidence of chorioretinal localization of disease using multimodal imaging. One patient exhibited a choroidal nevus complicated by active polypoidal choroidal neovascularization. Subretinal hyperreflective material was seen in three eyes, mainly resembling acquired vitelliform lesion. One patient had an isolated intraretinal hemorrhage. Most patients exhibited peripheral vascular abnormalities (ie, microaneurysms, peripheral vascular leakage). Fundus autofluorescence showed faint hyperautofluorescence in eleven eyes.
Conclusion: Intraocular involvement is an extremely rare event of an extremely rare disease. In patients affected by ECD without ocular symptoms, advance multimodal imaging examinations did not show signs of subclinical chorioretinal involvement related to the disease.

Keywords: Erdheim-Chester disease, histiocytosis, fluorescein angiography, indocyanine green angiography, optical coherence tomography

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