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Joo Yong Lee, Hyun Seung Yang, Jung Bok Lee, Young Hee Yoon, June-Gone Kim; Relationship with Vision and Characteristics of X-linked Juvenile Retinoschisis in Fourier Domain Optical Coherence Tomography. Invest. Ophthalmol. Vis. Sci. 2014;55(13):1142.
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To investigate the tomographic characteristics of the outer retina and choroid and their relationship with visual prognosis in X-linked juvenile retinoschisis (XLRS) patients with Fourier-Domain optical coherence tomography (FD-OCT).
We analyzed 20 consecutive eyes with XLRS using FD-OCT, retrospectively. The clinical and tomographic features of the outer retina,including the external limiting membrane (ELM), inner segment/outer segment (IS/OS) junction,cone cell outer segment tips (COST) line, photoreceptor outer segment (PROS) length, and choroid,were evaluated. To better analyze choroidal thickness and PROS length, 40 age-, sex-, and refraction-matched healthy eyes (1:2 matched linear mixed model) were randomly selected.
The most prevalent area of abnormality in the outer retina layer was the outer plexiform layer (OPL; 60%) and COST line (75%). On average, the subfoveal choroid and PROS lengths were~35 µm thicker and ~25µm thinner, respectively,in XLRS patients (P= .084 and P<.001, respectively).A dominant IS/OS junction,COST line defects, and PROS length were related to patient BCVA (P =.029, P<.001, and P<.001, respectively) in univariate analysis. Outer nuclear layer schisis and PROS length were the only factors related to BCVA in multivariate analysis (P =.029 and 0.020, respectively).
OPL and photoreceptor microstructure defects were frequently observed in XLRS patients. In addition, outer nuclear layer defects and shortened PROS length as well as photoreceptor microstructure defects may be closely related to poor vision in XLRS.
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