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MULTIMODAL FUNDUS IMAGING OF PSEUDOXANTHOMA ELASTICUM

Zweifel, Sandrine A MD*†; Imamura, Yutaka MD*†; Freund, K Bailey MD; Spaide, Richard F MD

doi: 10.1097/IAE.0b013e3181f056ce
Original Study
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Purpose: To investigate spectral domain optical coherence tomography and autofluorescence findings in eyes with pseudoxanthoma elasticum.

Methods: A retrospective analysis of visual acuity, spectral domain optical coherence tomography, and autofluorescence findings of consecutive patients with pseudoxanthoma elasticum was performed. The spectral domain optical coherence tomography was evaluated for retinal architecture, subretinal accumulations, and photoreceptor layer thickness. Autofluorescence and near-infrared reflectance images were reviewed for correlative findings.

Results: There were 21 patients (42 eyes) with pseudoxanthoma elasticum with a mean age of 56.1 ± 12.4 years. Subretinal fluid was found in 14 eyes, 7 of which had no signs of choroidal neovascularization. In six of the seven eyes with a history of choroidal neovascularization controlled with antivascular endothelial growth factor injections, there were areas of subretinal fluid that were not contiguous with the choroidal neovascularization and did not seem responsive to antivascular endothelial growth factor injections. Two types of formed material were observed in the subretinal space and outer retina. The first was hypoautofluorescent deposits above the retinal pigment epithelium resembling subretinal drusenoid deposits (reticular pseudodrusen). The second was yellow to brown hyperautofluorescent aggregates in the subretinal space and outer retina similar to those seen in pattern dystrophies and was found in 19 eyes. There was an apparent association between the presence of subretinal fluid and pattern dystrophy-like findings.

Conclusion: Subretinal fluid in patients with pseudoxanthoma elasticum is not always indicative of active leakage from underlying choroidal neovascularization and can be resistant to antivascular endothelial growth factor injections. This fluid is associated with pattern dystrophy-like findings and may indicate abnormal retinal pigment epithelial function.

Subretinal fluid is not always indicative of active leakage from underlying choroidal neovascularization and can be resistant to antivascular endothelial growth factor injections. This fluid is clinically more subtle compared with the exudation ordinarily seen in neovascular age-related macular degeneration, is associated with pattern dystrophy-like findings, and may indicate abnormal retinal pigment epithelial function.

From the *LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear & Throat Hospital, New York, New York; and †Vitreous-Retina-Macula Consultants of New York, New York, New York.

Supported in part by the Macula Foundation, Inc.; Dr. Y.I. was funded by grants from Koureisha Ganshikkan Kenkyu Zaidan, Mishima Saiichi-kinen Gankakenkyu Kokusaikouryu Kikin, and Takeda Kagaku Shinkou Zaidan.

R.F.S. receives royalty payments from Topcon America, Paramus, NJ. The other authors have no conflicts of interest to disclose.

Reprint requests: Richard F. Spaide, MD, 460 Park Avenue, 5th Floor, New York, NY 10022; e-mail: rickspaide@yahoo.com

© The Ophthalmic Communications Society, Inc.