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ACUTE ZONAL OCCULT OUTER RETINOPATHY: Structural and Functional Analysis Across the Transition Zone Between Healthy and Diseased Retina

Duncker, Tobias, MD*; Lee, Winston, MA*; Jiang, Fan, EdM*; Ramachandran, Rithambara, BA; Hood, Donald, C., PhD*,†; Tsang, Stephen, H., MD, PhD*; Sparrow, Janet, R., PhD*; Greenstein, Vivienne, C., PhD*

doi: 10.1097/IAE.0000000000001513
Original Study
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Purpose: To assess structure and function across the transition zone (TZ) between relatively healthy and diseased retina in acute zonal occult outer retinopathy.

Methods: Six patients (6 eyes; age 22–71 years) with acute zonal occult outer retinopathy were studied. Spectral-domain optical coherence tomography, fundus autofluorescence, near-infrared reflectance, color fundus photography, and fundus perimetry were performed and images were registered to each other. The retinal layers of the spectral-domain optical coherence tomography scans were segmented and the thicknesses of two outer retinal layers, that is, the total receptor and outer segment plus layers, and the retinal nerve fiber layer were measured.

Results: All eyes showed a TZ on multimodal imaging. On spectral-domain optical coherence tomography, the TZ was in the nasal retina at varying distances from the fovea. For all eyes, it was associated with loss of the ellipsoid zone band, significant thinning of the two outer retinal layers, and in three eyes with thickening of the retinal nerve fiber layer. On fundus autofluorescence, all eyes had a clearly demarcated peripapillary area of abnormal fundus autofluorescence delimited by a border of high autofluorescence; the latter was associated with loss of the ellipsoid zone band and with a change from relatively normal to markedly decreased or nonrecordable visual sensitivity on fundus perimetry.

Conclusion: The results of multimodal imaging clarified the TZ in acute zonal occult outer retinopathy. The TZ was outlined by a distinct high autofluorescence border that correlated with loss of the ellipsoid zone band on spectral-domain optical coherence tomography. However, in fundus areas that seemed healthy on fundus autofluorescence, thinning of the outer retinal layers and thickening of the retinal nerve fiber layer were observed near the TZ. The TZ was also characterized by a decrease in visual sensitivity.

Multimodal imaging was used to clarify the structure and function of the transition zone between relatively healthy and diseased retina in acute zonal occult outer retinopathy. The transition zone was associated with loss of the ellipsoid zone band outlined by a hyperautofluorescent border, thinning of the outer retinal layers and markedly decreased visual sensitivity.

Departments of *Ophthalmology, and

Psychology, Columbia University, New York, New York.

Reprint requests: Vivienne C. Greenstein, PhD, Department of Ophthalmology, Columbia University, 160 Fort Washington Avenue, Room 508, New York, NY 10032; e-mail: vcg17@columbia.edu

Supported in part by grants from the National Eye Institute/National Institutes of Health EY024091, EY021163, and EY09076; Foundation Fighting Blindness; and a grant from Research to Prevent Blindness to the Department of Ophthalmology, Columbia University.

None of the authors has any conflicting interests to disclose.

W. Lee and T. Duncker contributed equally to the work presented here and should therefore be regarded as equivalent authors.

© 2018 by Ophthalmic Communications Society, Inc.