The relationship of retinal morphological features and visual acuity of X-linked juvenile retinoschisis_Chinese Journal of Ocular Fundus Diseases

Authors：

ZhaoChen , ZhangQi , PengJie , 虞思伊 , 蔡璇 ,  赵培泉

Department of Ophthalmology, Xinhua Hospital, Shanghai Jiaotong University, Shanghai 20092, China;

Corresponding author：

赵培泉, Email: zhaopeiquan@126.com

Keywords：

Retinoschisis/congenital; Tomography, optical coherence

DOI：

10.3760/cma.j.issn.1005-1015.2014.06.009

Video：

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Objective To observe the optical coherence tomography (OCT) features of X-linked juvenile retinoschisis (XLRS) and its relationship and visual acuity. Methods Twenty-three XLRS patients (38 eyes) were enrolled in this study.All patients underwent best corrected visual acuity (BCVA) and frequency-domain optical coherence tomography (SD-OCT) examination. The international standard visual acuity chart was used for BCVA test and the results were then converted into logarithm of the minimum angle of resolution (logMAR) visual acuity. The mean logMAR BCVA of all patients was 0.41±0.28. Topcon 3D-OCT 1000 and Topcon DRI-OCT were applied to detect the retinal layers of retinoschisis. Based on the OCT features, retinoschisis was classified into 3 types, including splitting between outer nuclear layer (ONL) and outer plexiform layer (OPL), inner nuclear layer morphology (INL) splitting, splitting between ganglion cell layer (GCL) and nerve fiber layer (NFL). Based on the OCT features of fovea, there were photoreceptor inner segment (IS)/outer segment (OS) presence group and IS/OS absence group. Results There were 17 eyes (44.7%) with splitting between ONL/OPL, 38 eyes (100%) with INL splitting, 13 eyes (34.2%) with splitting between GCL/NFL. INL splitting can coexist with splitting in other retinal layers. There were 25 eyes (65.8%) with splitting in 2 or more retinal layers. The mean logMAR BCVA were 0.32±0.17 and 0.44±0.22 respectively in the IS/OS absence group and presence group, the difference was statistically significant (t=6.531, P=0.008).The mean fovea thickness (FT) were(517.10±96.92)and(523.08±72.84) μm respectively in the IS/OS absence group and presence group, the difference was not statistically significant (t=2.282, P=0.061). There was no correlation between logMAR BCVA and FT (r=0.200, P=0.929). Conclusions In patients with XLRS, the foveomacular schisis involved in multiple retinal layers and most frequently in the INL. The defect of IS/OS is the important reason of the low visual acuity.

Citation： ZhaoChen, ZhangQi, PengJie. The relationship of retinal morphological features and visual acuity of X-linked juvenile retinoschisis. Chinese Journal of Ocular Fundus Diseases, 2014, 30(6): 571-573. doi: 10.3760/cma.j.issn.1005-1015.2014.06.009 Copy

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14.	Apushkin MA, Fishman GA, Janowicz MJ, et al.Correlation of optical coherence tomography findings with visual acuity and macular lesions in patients with X-linked retinoschisis[J]. Ophthalmology, 2005, 112:495-501.
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1. Haas J. Ueber das zusammenvorkommen von veränderungen der retina und chorioidea[J]. Arch Augenheikd, 1898, 37:343-348.
2. Kjellstr m S, Vijayasarathy C, Ponjavic V, et al. Long-term 12 year follow-up of X-linked congenital retinoschisis[J]. Ophthalmic Genet, 2010, 31:114-125.
3. Takano M, Kishi S. Foveal retinoschisis and retinal detachmentin severely myopic eyes with posterior staphyloma[J]. Am J Ophthalmol, 1999, 128:472-476.
4. Keane P, Bhatti R, Brubaker J, et al. Comparison of clinically relevant findings from high-speed fourier-domain and conventional time-domain optical coherence tomography[J].Am J Ophthalmol, 2009, 148:242-248.
5. Gregori N, Berrocal A, Gregori G, et al. Macular spectraldomain optical coherence tomography in patients with X-linked retinoschisis[J]. Br J Ophthalmol, 2009, 93:373-378.
6. Edwards AO, Robertson JE. Hereditary vitreoretinal degenerations[M]//Ogden TE, Hinton DR. Retina. St Louis:Mosby, 2001:482-498.
7. 聂爱光.遗传性黄斑变性[M]//聂爱光.现代黄斑疾病诊断治疗学.北京:北京医科大学, 中国协和医科大学联合出版社, 1996:155-168.
8. Ooto S, Hangai M, Sakamoto A, et al. Three-dimensional profile of macular retinal thickness in normal Japanese eyes[J]. Invest Ophthalmol Vis Sci, 2010, 51:465-473.
9. Lesch B, Szabó V, Kánya M, et al. Clinical and genetic findingsin Hungarian patients with X-linked juvenile retinoschisis[J]. Mol Vis, 2008, 14:2321-2332.
10. 刘杏.光学相干断层成像学[M].广州:广东科技出版社, 2006:246-250.
11. Genead MA, Pasadhika S, Fishman GA.Retinal nerve fiber layer thickness analysis in X 1inked retineschisis using fourier-domain OCT[J].Eye, 2009, 23:1019-1027.
12. Lee JJ, Kim JH, Kim SY, et al.Infantile vitreous hemorrhage as the initial presentation of X-linked juvenile retinoschisis[J].Korean J Ophthalmol, 2009, 23:118-120.
13. Urrets-Zavalia JA, Venturino JP, Mercado J, et al. Macular and extramacular optical coherence tomography findings in X-linked retinoschisis[J]. Ophthalmic Surg Lasers Imaging, 2007, 38:417-422.
14. Apushkin MA, Fishman GA, Janowicz MJ, et al.Correlation of optical coherence tomography findings with visual acuity and macular lesions in patients with X-linked retinoschisis[J]. Ophthalmology, 2005, 112:495-501.
15. Sayanagi K, Ikuno Y, Soga K, et al. Photoreceptor inner and outer segment defects in myopic foveoschisis[J]. Am J Ophthalmol, 2008, 145:902-908.

Chinese Journal of Ocular Fundus Diseases

The relationship of retinal morphological features and visual acuity of X-linked juvenile retinoschisis

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