EXPANDED CLINICAL SPECTRUM OF MULTIPLE EVANESCENT WHITE DOT SYNDROME WITH MULTIMODAL IMAGING.

Retina

*Vitreous-Retina-Macula Consultants of New York and the LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Institute, New York, New York; †Department of Ophthalmology, Columbia University, New York, New York; ‡Department of Ophthalmology, University and Polytechnic Hospital La Fe, Valencia, Spain; §Faculdade de Medicina da Universidade de São Paulo (FMUSP), Sao Paulo, Brazil; ¶Department of Ophthalmology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois; **Department of Ophthalmology, Inselspital, University Hospital Bern, Bern, Switzerland; ††Department of Ophthalmology, Shanghai Jiao Tong University Affiliated First People's Hospital, Shanghai, China; ‡‡Quinze-Vingts Hospital, DHU ViewMaintain, Paris, France; §§Department of Ophthalmology, California Pacific Medical Center, San Francisco, California; ¶¶West Coast Retina Medical Group, San Francisco, California; ***Department of Ophthalmology, The Icahn School of Medicine at Mount Sinai, New York, New York; †††Department of Ophthalmology, Bascom Palmer Eye Institute, Miami, Florida; ‡‡‡Hospital Lariboisiere, University Paris, Paris, France; §§§Centre Ophtalmologique de l'Odeon, Paris, France; ¶¶¶Department of Ophthalmology, The New York Eye and Ear Infirmary, New York, New York; and ****Department of Ophthalmology, New York University, New York, New York.

Published: January 2016

Purpose: To evaluate and characterize multiple evanescent white dot syndrome abnormalities with modern multimodal imaging modalities.

Methods: This retrospective cohort study evaluated fundus photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, enhanced depth imaging optical coherence tomography, short-wavelength autofluorescence, and near-infrared autofluorescence.

Results: Thirty-four multiple evanescent white dot syndrome patients with mean age of 28.7 years were studied (range, 14-49 years). Twenty-six patients were women, and eight were men. Initial mean visual acuity was 0.41 logMAR. Final mean visual acuity was 0.03 logMAR. Fluorescein angiography shows a variable number of mid retinal early fluorescent dots distributed in a wreathlike pattern, which correlate to fundus photography, fundus autofluorescence, and indocyanine green angiography. Indocyanine green angiography imaging shows the dots and also hypofluorescent, deeper, and larger spots, which are occasionally confluent, demonstrating a large plaque of deep retinal hypofluorescence. Optical coherence tomography imaging shows multifocal debris centered at and around the ellipsoid layer, corresponding to the location of spots seen with photography, indocyanine green angiography, and fluorescein angiography. Protrusions of the hyperreflectant material from the ellipsoid layer toward the outer nuclear layer correspond to the location of dots seen with photography, indocyanine green angiography, and fluorescein angiography.

Conclusion: Multimodal imaging analysis of the retina in patients with multiple evanescent white dot syndrome shows additional features that may help in the diagnosis of the disease and in further understanding its etiology. Multiple evanescent white dot syndrome is predominantly a disease of the outer retina, centered at the ellipsoid zone, but also involving the interdigitation zone and the outer nuclear layer.

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http://dx.doi.org/10.1097/IAE.0000000000000685DOI Listing

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