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POSTERIOR STAPHYLOMAS IN EYES WITH RETINITIS PIGMENTOSA WITHOUT HIGH MYOPIA

Xu, Xian, MD*,†; Fang, Yuxin, MD*; Yokoi, Tae, MD*; Shinohara, Kosei, MD*; Hirakata, Akito, MD; Iwata, Takeshi, PhD§; Tsunoda, Kazushige, MD, PhD; Jonas, Jost B., MD**; Ohno-Matsui, Kyoko, MD, PhD*

doi: 10.1097/IAE.0000000000002180
Original Study: PDF Only

Purpose: To describe features of posterior staphylomas in nonhighly myopic eyes with retinitis pigmentosa (RP).

Methods: The retrospective observational case series study included patients with RP and an axial length of <26.5 mm and searched for eyes with posterior staphylomas. All study participants underwent fundus photography and optical coherence tomography.

Results: The study identified 13 eyes of 7 patients with a narrow macular staphyloma. Mean age was 40.9 ± 17.9 years (range 9–62 years) and mean axial length was 24.90 ± 0.69 mm. The staphyloma edges corresponded to the margin between the retinal atrophic area in the fundus midperiphery and the relatively unaffected fundus center. On vertically orientated optical coherence tomography images, the staphyloma edges showed a slight inward protrusion of the sclera and a ring-like localized choroidal thinning with choroidal rethickening in direction toward the fovea and toward the periphery of the fundus. The upper and lower staphyloma edges did not differ in steepness. The thickness of the subfoveal choroid (138.6 m ± 50.1 µm) was thinner than the normal range after adjusting for age and axial length in all eyes. Two eyes with advanced RP in the macula showed a subfoveal choroidal thickness of 95 µm and 88 µm.

Conclusion: Narrow macular staphylomas can occur in nonhighly myopic eyes with RP and, in contrast to staphylomas in highly myopic eyes, show a less marked thinning of the subfoveal choroid. The occurrence of posterior staphylomas in nonhighly myopic eyes with RP may provide hints to unravel the etiology of posterior staphyloma formation.

This was a retrospective observational case series study. Narrow macular staphylomas can occur in nonhighly myopic eyes with retinitis pigmentosa and, in contrast to staphylomas in highly myopic eyes, show a less marked thinning of the subfoveal choroid. It may provide hints to unravel the etiology of posterior staphyloma formation.

*Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, Tokyo, Japan;

Department of Ophthalmology, Shanghai General Hospital, Shanghai Jiaotong University, Shanghai, China;

Department of Ophthalmology, Kyorin Eye Center, Kyorin University School of Medicine, Tokyo, Japan;

§Division of Molecular and Cellular Biology, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan;

Department of Ophthalmology, National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan; and

**Department of Ophthalmology, Medical Faculty Mannheim of the Ruprecht-Karls-University of Heidelberg, Heidelberg, Germany.

Reprint requests: Kyoko Ohno-Matsui, MD, PhD, Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan; e-mail: k.ohno.oph@tmd.ac.jp

Supported by grants from the Japanese society for promotion of science, Tokyo, Japan (number; 15h04993, 15k15629).

J. B. Jonas: Consultant for Mundipharma Co (Cambridge, United Kingdom); patent holder with Biocompatibles UK Ltd (Franham, Surrey, UK) (Title: Treatment of eye diseases using encapsulated cells encoding and secreting neuroprotective factor and/or antiangiogenic factor; Patent number: 20120263794), and patent application with University of Heidelberg (Heidelberg, Germany) (Title: Agents for use in the therapeutic or prophylactic treatment of myopia or hyperopia; Europa¨ische Patentanmeldung 15 000 771.4). The remaining authors have no financial/conflicting interests to disclose.

All authors attest that they meet the current ICMJE criteria for authorship.

© 2018 by Ophthalmic Communications Society, Inc.