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Multimodal Chorioretinal Imaging in Erdheim-Chester Disease

Authors
  • Sacconi, Riccardo1
  • Campochiaro, Corrado2
  • Rabiolo, Alessandro1
  • Marchese, Alessandro1
  • Tomelleri, Alessandro2
  • Tomasso, Livia1
  • Cicinelli, Maria Vittoria1
  • Querques, Lea1
  • Bandello, Francesco1
  • Dagna, Lorenzo2
  • Querques, Giuseppe1
  • 1 Department of Ophthalmology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Milan , (Italy)
  • 2 Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan , (Italy)
Type
Published Article
Journal
Clinical Ophthalmology (Auckland, N.Z.)
Publisher
Dove
Publication Date
Feb 28, 2020
Volume
14
Pages
581–588
Identifiers
DOI: 10.2147/OPTH.S224672
PMID: 32184546
PMCID: PMC7054052
Source
PubMed Central
Keywords
License
Green

Abstract

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.

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