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. 2018 Jun 18;11(6):986-990.
doi: 10.18240/ijo.2018.06.15. eCollection 2018.

Clinical observations of vitreoretinal surgery for four different phenotypes of X-linked congenital retinoschisis

Chen Zhao  1 , Qi Zhang  1 , Hai-Ying Jin  1 , Pei-Quan Zhao  1
Affiliations

Clinical observations of vitreoretinal surgery for four different phenotypes of X-linked congenital retinoschisis

Chen Zhao et al. Int J Ophthalmol. .

Abstract

Aim: To evaluate the outcomes of vitreoretinal surgery for four different phenotypes of X-linked retinoschisis (XLRS).

Methods: This study included thirty-one eyes of 25 patients who developed XLRS with severe complications. Among the 31 eyes, there were 7 eyes with vitreous hemorrhage, 8 eyes with retinal detachment and vitreous hemorrhage, and 16 eyes with rhegmatogenous retinal detachment. All of the patients underwent standard three-port pars plana vitrectomy. All of the eyes were divided into 4 groups before surgery according to a modified classification scheme, with the first three groups as follows: group A, with foveal cystic schisis; group B with macular lamellar schisis; and group C with foveolamellar changes. Peripheral schisis was not present in these 3 groups; however, group D was a complex group with both macular and peripheral changes. One year after surgery, we analyzed the best corrected visual acuity and postoperative anatomical and functional outcomes of these 4 groups.

Results: There were 7 eyes in group A (22.6%), 1 eye in group B (3.2%), 15 eyes in group C (48.4%) and 8 eyes in group D (25.8%). Postoperative anatomical and functional outcomes were satisfactory at the last visit, while the mean visual acuity was increased to 0.27±0.11, with a significant difference (t=-1.049, P=0.000) compared with the results before surgery (0.1±0.08). Visual acuity was improved in 23 eyes (74.2%). Complications were found in three eyes: two eyes with proliferative vitreoretinopathy and traction retinal detachment 10 and 12mo after surgery, respectively; and one eye with vitreous hemorrhage 15mo after surgery. These eyes were in groups C and D. The retinas remained attached in these 3 eyes after reoperation.

Conclusion: We should pay greater attention to XLRS with foveolamellar changes because it is the most changeable phenotype. Once complications occur, vitreoretinal surgery can significantly improve visual acuity and restore the anatomic structure of the retina.

Keywords: X-linked retinoschisis; complications; vitreoretinal surgery.

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Figures

Figure 1
Figure 1. Fundus photograph of a patient in group A showing foveal schisis with a spoke-wheel pattern.
Figure 2
Figure 2. Topcon Dri-OCT image of a patient in group D showing foveal cystic retinoschisis in two different retinal layers.
Figure 3
Figure 3. Fundus photograph of an XLRS patient in group D. Vascular changes are remarkable, appearing as extensive vascular sheathing.
Figure 4
Figure 4. It was necessary to apply dam-type endolaser to the peripheral retinoschisis areas (one case in group D).
See this image and copyright information in PMC

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