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Jung, Jesse J. MD*,†,‡; Khan, Samira MD§; Mrejen, Sarah MD†,¶; Gallego-Pinazo, Roberto MD, PhD*,†,**; Cunningham, Emmett T. Jr MD, PhD, MPH††,‡‡,§§; Freund, K. Bailey MD*,†,¶,‡; Jampol, Lee M. MD§; Yannuzzi, Lawrence A. MD*,†,¶,‡

doi: 10.1097/IAE.0000000000000079
Original Study

Purpose: To report thirteen cases of idiopathic multifocal choroiditis with discrete chorioretinal lesions who were found to have zonal, multizonal, or diffuse outer retinal or chorioretinal atrophy.

Methods: A retrospective observational case series using multimodal imaging including high-definition optical coherence tomography, fundus autofluorescence imaging, and fluorescein and indocyanine green angiography.

Results: Twenty-one eyes in 13 patients with idiopathic multifocal choroiditis were found to have zonal, multizonal, or diffuse outer retinal or chorioretinal atrophy visualized using multimodal imaging. Thirteen eyes presented with diffuse disease, six eyes with multizonal, and two with zonal atrophy. Patterns of atrophy included zones surrounding the optic nerve, multiple geographic zones in the mid and far periphery, and a diffuse peripheral pattern with relative sparing of the central macula until later in the course of disease. Eleven of the 13 patients were treated with topical, periocular, or systemic corticosteroids, and 1 patient was also treated with systemic immunomodulatory treatment. The atrophic changes progressed over an average of 8 years of follow-up in 10 eyes despite therapy.

Conclusion: Idiopathic multifocal choroiditis can present with an uncommon pattern of zonal, multizonal, or diffuse outer retinal or chorioretinal atrophy as part of its clinical spectrum. The severity, extent, and progression of these atrophic changes are best appreciated using multimodal diagnostic imaging.

Idiopathic multifocal choroiditis may be associated with zonal, multizonal, or diffuse outer retinal or chorioretinal atrophy. These patients present with a unique form of zonal atrophy within the clinical spectrum of idiopathic multifocal choroiditis that is best evaluated with multimodal imaging.

*Department of Ophthalmology, New York University School of Medicine, New York, New York;

Vitreous Retina Macula Consultants of New York, New York, New York;

Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University College of Physicians and Surgeons, New York, New York;

§Department of Ophthalmology, Feinberg School of Medicine of Northwestern University, Chicago, Illinois;

LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, New York;

**Department of Ophthalmology, University and Polytechnic Hospital La Fe, Valencia, Spain;

††Department of Ophthalmology California Pacific Medical Center, San Francisco, California;

‡‡Department of Ophthalmology, Stanford University School of Medicine, Stanford, California; and

§§West Coast Retina Medical Group, San Francisco, California.

Reprint requests: Lawrence A. Yannuzzi, MD, Vitreous Retina Macula Consultants of New York, 460 Park Avenue, Fifth Floor, New York, NY 10022; e-mail:

Supported by the Macula Foundation, Inc; an unrestricted grant from Research to Prevent Blindness, Inc, NYC (Northwestern University); and a grant from Mary Dempsey and Kevin Hitzeman.

None of the authors have any conflicting interests to disclose.

Lee M. Jampol and Lawrence A. Yannuzzi are co-senior authors.

© 2014 by Ophthalmic Communications Society, Inc.