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Author Notes:

Corresponding author and reprint requests to: Steven Yeh, MD, Emory Eye Center, 1365 B Clifton Road, Suite B2400, Atlanta, GA 30322, Phone: (404) 778-5073, Fax: (404) 778-4530, steven.yeh@emory.edu

Conformity of Information: The Emory University School of Medicine Institutional Review Board approved this study and all work pertaining to this project maintained HIPAA compliance.

Access to Data: The corresponding author, Dr. Steven Yeh, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

The authors have no financial or proprietary interest in any product mentioned herein.

Subjects:

Research Funding:

Supported in part by a grant to Emory University Eye Center from the Research to Prevent Blindness, Inc.

Multimodality Diagnostic Imaging in Unilateral Acute Idiopathic Maculopathy

Tools:

Journal Title:

Archives of ophthalmology (Chicago, Ill. : 1929)

Volume:

Volume 130, Number 1

Publisher:

, Pages 50-56

Type of Work:

Article | Post-print: After Peer Review

Abstract:

Objective To describe the clinical features and imaging characteristics in unilateral acute idiopathic maculopathy (UAIM). Methods This is a retrospective review of four patients diagnosed with UAIM. Clinical characteristics (age, symptoms, Snellen visual acuity (VA), and funduscopic features) and images from spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein (FA), and indocyanine green (ICG) angiography were analyzed. Results The median age at presentation was 31 years (range 27–52 years). The median interval between symptom onset and presentation was four weeks (range 1–20 weeks). Associated systemic findings included a viral prodrome (50%), orchitis (50%), hand-foot-mouth disease (25%), and positive Coxsackie virus titers (50%). The median presenting VA was 20/400 (range 20/70–1/400), which improved to 20/30 (range 20/20–20/60) at final follow-up. The median follow-up time was 6 weeks (range 0–8 weeks). Early in the disease course, the central macula developed irregular, circular areas of white-grey discoloration. Following recovery, the macula had a stippled retinal pigment epithelium characterized by rarefaction and hyperplasia. FA demonstrated irregular early hyperfluorescence and late subretinal hyperfluorescence. SD-OCT showed a partially reversible disruption of the outer photoreceptor layer. FAF initially revealed stippled autofluorescence that eventually became more hypoautofluorescent. ICG showed “moth-eaten” appearing choroidal vasculature, suggestive of choroidal inflammation. Conclusions The imaging characteristics highlight the structural changes during the active and resolution phases of UAIM. The visual recovery correlates with structural changes and suggests that the pathogenesis involves inflammation of the inner choroid, retinal pigment epithelium, and outer photoreceptor complex that is partially reversible.

Copyright information:

© 2012, American Medical Association

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