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Original Study


Iwahashi, Chiharu MD*; Matsushita, Itsuka MD; Kuniyoshi, Kazuki MD*; Kondo, Hiroyuki MD; Kusaka, Shunji MD*

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Retina 43(1):p 64-71, January 2023. | DOI: 10.1097/IAE.0000000000003633
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Congenital X-linked retinoschisis (CXLRS) is caused by mutations in the retinoschisin 1 gene on the distal short arm of the X chromosome (Xp22.1–p22.3), which encodes protein retinoschisin.1 Retinoschisin functions as a cell adhesion protein that maintains the synaptic structure of the retina.2 Congenital X-linked retinoschisis is characterised by foveal retinoschisis, which occurs in nearly all patients, whereas peripheral schisis is present in approximately half of patients with CXLRS.3 Congenital X-linked retinoschisis occurs almost exclusively in male patients. Female carriers generally do not display fundus changes, although there are rare reports of heterozygotes with clinical signs.4 The disease presents in most affected male patients in the first decade of life, especially at school age, and it is associated with impaired vision and strabismus.5 In most patients, the disease displays no or minimal progression.6–8 However, complications occur in approximately 5% of all affected male patients, most commonly arising in the first decade of life.9 Bullous retinoschisis is more frequently reported in patients as young as 3 months, which can lead to nystagmus or strabismus because of the large shadow in front of the macula.10,11

The vitreoretinal complications of CXLRS include retinal detachment (RD), vitreous hemorrhage, hemorrhage within a large schisis cavity, neovascular glaucoma, and macular dragging.12 Surgical intervention is required for eyes with nonclearing vitreous hemorrhage and/or intraschisis cavity hemorrhage, bullous schisis cavity hanging over or threatening the macula, and RD. Vitrectomy for such pathological conditions was reported previously in small case series.12–19 Although gas or silicone oil (SO) tamponade is necessary to achieve retinal reattachment in the treatment of rhegmatogenous RD, the optimal surgical procedure and the need for gas or SO tamponade during the treatment of vitreous hemorrhage and bullous schisis cavity hanging over or threatening the macula remain controversial. Assuming that intraschisis cavity hemorrhage and bullous schisis cavity hanging over or threatening the macula are caused by progressive enlargement of the schisis cavity, inner wall retinectomy (IWR) and internal limiting membrane peeling around the retinoschisis area were previously recommended.12,15,18 However, these previous reports were small case series from a single institute, and the appropriate surgical intervention for bullous schisis cavity hanging over or threatening the macula in patients with CXLRS remains unclear.

This study investigated the surgical outcome of vitrectomy for bullous schisis cavity hanging over or threatening the macula in patients with CXLRS and evaluated surgical techniques, including IWR, and the use of intraocular tamponade.


Patients and Examinations

The medical records of consecutive patients with CXLRS who underwent vitrectomy at Osaka University Hospital (Suita, Japan), Kindai University (Osakasayama, Japan), or the University of Occupational and Environmental Health (Kitakyushu, Japan) between August 2009 and June 2017 completed at least 3 years of postoperative follow-up were retrospectively reviewed. The indication of vitrectomy included bullous schisis cavity with macular obscuration or schisis border involving the macula. The diagnosis of CXLRS was made clinically, i.e., the presence of macular schisis with or without peripheral retinal schisis and the presence of negative b-wave on electroretinogram (ERG). In addition, the diagnosis was confirmed using genetic screening for retinoschisin 1 gene. The study protocols were approved by the Institutional Review Boards of Kindai University Hospital (#28-264) and the University of Occupational and Environmental Health (#UOEHCRB20-148). This study adhered to the tenets of the Declaration of Helsinki.

Patients underwent ophthalmic examinations before and after surgery including best-corrected visual acuity assessments, fundus photography, visual field testing using Goldmann kinetic perimetry (Goldmann kinetic perimetry), and full-field ERG. In the initial examination, fundus photographs were taken using a RetCam three digital fundus camera (Natus, San Carlo, CA), and full-field ERGs were obtained according to the guidelines of the International Society for Clinical Electrophysiology of Vision including dark-adapted 0.01 ERG (stimulation, 0.01 cd·s·m−2), dark-adapted 10.0 ERG (stimulation, 10.0 cd·s·m−2), light-adapted (LA) 3.0 ERG (stimulation, 3.0 cd·s·m−2; background, 30 cd·m−2), and LA 30-Hz flicker (stimulation, 3.0 cd·s·m−2; background, 30 cd·m−2).20 Electroretinogram was performed using a contact lens electrode with a built-in white light-emitting diode system (LE-4000, Tomey Co, Nagoya, Japan).

Data were collected from the medical charts included sex, diagnosis at the referring hospital, mutations in retinoschisin 1 gene, ocular features, indication for vitrectomy, age at vitrectomy, surgical procedure, postoperative complications during follow-up, and best-corrected visual acuity at the final follow-up.

Surgical Procedure of Vitrectomy

All surgeries were performed by two surgeons (S.K. and H.K.). The operating surgeon selected the detailed surgical approach for each patient because there was no predefined protocol in this retrospective study. All eyes were subjected to small-gauge (23-gauge, 25-gauge, or [more recently] 27-gauge) vitrectomy. The surgical procedures were as follows. After conjunctival peritomy, sclerotomies were made 1 mm to 3 mm from the limbus depending on the patient's age. For the fundus view, the wide-angle viewing system Resight (Carl Zeiss Meditec AG, Jena, Germany) was used during surgery. We adopted previously reported techniques.15,18 In brief, separation of the posterior vitreous was initiated from the disc area and then extended peripherally. However, it was sometimes difficult at the area in which the schisis was bullous. In eyes subjected to IWR, diathermy was applied to the major vessels in the inner wall, followed by dissection using a vitreous cutter. In some cases in which the schisis was near the macula, photocoagulation was applied to the boundary. At the end of the surgery, 10%–20% short-acting gas (sulphur hexafluoride) or SO was used for intraocular tamponade. The lens was preserved as much as possible unless lensectomy was necessary to treat complex retinal conditions. As for the indication of IWR, the inner wall retina in the early cases of this case series was preserved based on the previous report.21 However, adhesion between the inner and outer walls of the retina was not achieved even with similar techniques. We decided to perform IWR at the initial surgery for subsequent cases. All parents or guardians were well informed about the efficacy and possible complications of vitrectomy, and written informed consent was obtained from each patient's parents or guardians.

Genetic Examinations

Genomic DNA was extracted from peripheral blood using DNA extraction kits or manual extraction with ethanol. Polymerase chain reaction followed by Sanger sequencing was performed for six coding exons of retinoschisin 1 gene as described previously.22

Visual Acuity Measurements

Cycloplegic refraction of the patients was tested after instillation of cyclopentolate 1.0%, atropine 1.0%, or atropine 0.5% eye drops, and automatic refraction was tested using the auto refractometer (NIDEK ARK-530A; Nidek, Gamagori, Japan or Topcon KR-8100; Topcon Corporation, Tokyo, Japan) or the Righton Retinomax three Refract Keratometer (Right Group, Tokyo, Japan). Visual acuity was assessed considering the age of the infant using suitable refractive correction with Teller Acuity Cards (Stereo Optical, Inc, Chicago, IL), Morizane, single-symbol optotypes (Handaya Co, Ltd, Tokyo, Japan), or Landolt ring.

Statistical Analysis

Statistical analyses were performed using JMP version 14.0 for Windows (SAS Institute, Cary, NC). Visual acuity was converted to the logarithm of the minimum angle of resolution for data analysis. Categorical variables were compared using the Fisher exact test. P < 0.05 indicated statistical significance.


Nine consecutive male patients (12 eyes) who underwent follow-up for ≥3 years after surgery were analyzed. The patients' profiles and surgical outcomes are presented in Table 1. Genetic examinations were performed in all patients. The genetic changes were previously reported.22 The ERG revealed typical negative results in all patients. The follow-up periods ranged from 3.0 to 10.3 years (median: 6.0 years). Age at vitrectomy ranged from 4 months to 8.6 years (median: 14 months). The ocular findings of the youngest patient (Case 1) are presented in Figure 1. The referral diagnoses were RD in five patients, suspected retinal schisis in three patients, and subretinal hemorrhage in one patient.

Table 1. - Profiles and Surgical Outcomes of Vitrectomy for Bullous Schisis Cavity Hanging Over or Threatening the Macula in Patients With Congenital X-Linked Retinoschisis
Case No. Eye Nucleotide Change (NM_000330.3) Age at Vitrectomy (months) Surgical Procedure Postoperative Complications 1st Additional Surgery 2nd Additional Surgery Follow-Up (months) Age at Last F/U (years) SE at Last F/U Decimal (Snellen) VA at Last F/U
1 R c.544C>T 4 LSV, SO Cataract PPV, IWR, P+I, SO removal 72 6 0 0.6 (20/33)
L c.544C>T 4 LSV, SO Cataract PPV, IWR, P+I, SO removal 72 6 −2.75 0.3 (20/66)
2 R c.35T>C 5 LSV, IWR 46 4 0 0.2 (20/100)
L c.35T>C 5 LSV, IWR 46 4 0 0.05 (20/400)
3 R c.589C>T 7 LSV, IWR, gas PVR, RRD PPV, lensectomy, SO Encircling, SO removal, gas 48 4 NA HM
4 R c.544C>T 13 LSV, IWR, gas 64 6 0 0.3 (20/66)
5 L c.305G>A 15 LSV, gas Cataract, macular pucker
PPV, IWR, ILMP, P+I, gas PPV, ahmed 95 9 1 0.1 (20/200)
6 L c.38T>C 37 PPV, P+I, IWR, SO SO removal 123 13 5.5 0.03 (20/666)
R c.38T>C 41 LSV, IWR, SO SO removal 120 13 0 0.15 (20/133)
7 R c.326G>C 33 LSV, IWR, gas 100 11 5 0.6 (20/33)
8 L c.304C>T 54 LSV, IWR, gas 37 7 2 0.1 (20/200)
9 L exon2-3del 103 LSV, SO Unresolved retinoschisis LSV, SO removal, IWR, gas 84 15 4 0.3 (20/66)
Ahmed, Ahmed glaucoma valve implantation; F/U, follow-up; HM, hand motion; ILMP, internal limiting membrane peeling; L, left eye; LSV, lens-sparing vitrectomy; P + I, phacoemulsification with an intraocular lens implantation; PPV, pars plana vitrectomy; R, right eye; SE, spherical equivalent; VA, visual acuity

Fig. 1.:
Fundus photographs and postoperative OCT images in the left eye of the youngest patient (Case 1). The age at vitrectomy was 4 months. Fundus photographs taken preoperatively (A) and 6 years after the initial vitrectomy (B). OCT image 6 years after the initial vitrectomy revealing defects of the inner wall of the retina (range of double-headed arrow) (C).

Surgical procedures during the initial vitrectomy included lens-sparing vitrectomy with (seven eyes) or without (four eyes) IWR, and vitrectomy with IWR and cataract surgery was performed for one eye. Five eyes achieved a clear central visual axis after the initial vitrectomy, and seven eyes required an additional vitrectomy.

The postoperative complications after initial vitrectomy included cataract and unresolved retinoschisis in two eyes (Case1R and 1L), proliferative vitreoretinopathy (PVR) (Case 3), macular pucker and cataract in one eye (Case 5), and unresolved retinoschisis in one eye (Case 9, Figure 2). A total RD with PVR developed in Case 3 after a retinal tear formation which occurred temporal to the macula 3 months after the initial vitrectomy, probably because of the traction by membranes developed on the surrounding retina. The retina was reattached after two additional surgeries, including lensectomy, membrane peeling, encircling, and SO tamponade.

Fig. 2.:
Fundus photographs and OCT images in the left eye of Case 9. A and D. Fundus photograph and OCT images after SO tamponade. Note the out-of-focus image of the inferior retinal surface and OCT image indicating unresolved retinoschisis (*) that nearly extents to the macula. C. Intraoperative images revealing IWR with radial incisions at the cauterized edge of the inner retina. B and E. Fundus photograph and OCT images after SO removal and IWR.

A second surgery was performed to treat cataract and macular pucker in case 5. This case developed glaucoma and underwent Ahmed glaucoma valve implantation 7 years after the second surgery. Consequently, five eyes required one surgery, five eyes required two surgeries, and two eyes required three surgeries. Postoperative complications by surgical procedure are presented in Table 2. All eyes maintained a clear central visual axis at the last examination.

Table 2. - Postoperative Complications by Surgical Procedures
Postoperative Complications Needed Reoperation Total P
(+) (−)
Inner wall retinectomy (+) 1 7 8 0.01*
(−) 4 0 4
SO tamponade (+) 3 2 5 0.56*
(−) 2 5 7
*Fisher exact test.

Inner wall retinectomy was performed in eight of 12 eyes during the initial vitrectomy. Of these eight eyes, seven eyes achieved a clear central visual axis without additional treatment, whereas the remaining eye required repeated vitrectomy to treat postoperative PVR. All four eyes treated with lens-sparing vitrectomy without IWR required repeat vitrectomy. Of these eyes, three developed unresolved retinoschisis and one developed macular pucker. As a result, all four eyes underwent IWR in the second surgery. Overall, one of eight eyes (12.5%) treated with IWR and all four eyes (100%) that were not treated with IWR in the initial vitrectomy required subsequent retinal surgeries for postoperative complications (P = 0.01). Thus, IWR seemed to be associated with fewer complications after surgery.

The substance used for intraocular tamponade in the initial vitrectomy was SO and short-acting gas in five eyes each. Silicone oil was used in a possible retinal break in Case 6L. Silicone oil was used to achieve adhesion between the inner and outer walls of the retina in the other four eyes. Two eyes subjected to lens-sparing vitrectomy without intraocular tamponade did not require any additional surgery (Figure 3). Among five eyes treated with gas tamponade, two were treated by repeated vitrectomy for postoperative PVR (one eye) or macular pucker (one eye). Among five eyes in which SO tamponade was provided, two were subjected to SO removal without any additional procedures and three eyes received SO removal with additional procedures, i.e., lensectomy and intraocular lens implantation, SO removal, and IWR. Inner wall retinectomy was deemed in these eyes necessary because no adhesion of the inner and outer walls and the presence of a relatively large amount of emulsified SO droplets between them were observed after lensectomy. Overall, three of five (60.0%) eyes that received SO tamponade during the initial vitrectomy and two of seven (28.6%) eyes that did not receive SO tamponade during the initial vitrectomy required subsequent retinal surgeries for postoperative complications (P = 0.56).

Fig. 3.:
Preoperative and postoperative fundus photographs in the right eye of Case 2. A. Large schisis hanged over the macula, and optic disc was observed preoperatively. B. Postoperative fundus photographs 4 years after successful lens-sparing vitrectomy and IWR without tamponade.

The best-corrected visual acuity using Landolt ring at the final follow-up ranged from hand motion to 0.22 logMAR (20/33 in Snellen) (median, 0.76 logMAR, 20/115 in Snellen) in patients aged 3 years to 15 years. Excluding one eye with hand motion and three eyes that underwent lensectomy with intraocular lens implantation, the refractive errors in spherical equivalent ranged from 0 to +5.5 diopters. Four eyes were hyperopic, and four eyes were emmetropic. Visual field was tested using Goldmann kinetic perimetry in four eyes from three patients (Cases 6–8). The results revealed nasal visual field defects consistent with the extent of retinal schisis in all eyes. In addition, central scotoma was observed in two eyes with extensive retinal schisis.


This study investigated the surgical outcomes of vitrectomy for bullous schisis cavity hanging over or threatening the macula in patients with CXLRS and evaluated surgical techniques including IWR and the use of intraocular tamponade. The results demonstrated that vitrectomy and IWR are effective for restoring a clear visual axis with a lower likelihood of additional procedures in eyes with CXLRS. In addition, SO tamponade was not advantageous for the treatment of bullous schisis cavity hanging over or threatening the macula without RD.

The range of clinical findings in CXLRS is broad, and diagnosis may be challenging. The exudative form may mimic Coats disease, familial exudative vitreoretinopathy, or uveitis-related detachments, and the nonexudative form may be similar to traumatic or nontraumatic paediatric RRD.23 In particular, the distinction between CXLRS and vision-threatening RRD is essential for patient management because most eyes with CXLRS do not require surgery, whereas RRD requires surgical intervention in most cases. In fact, we experienced one case, which was not included in this study because there was no indication for vitrectomy, in which scleral buckling surgery had been performed elsewhere under a diagnosis of RRD. If confusing, examination under anesthesia including ERG is recommended. Genetic testing can lead to a definitive diagnosis if possible.

In the histopathological study of eyes with CXLRS, prominent immunolabelling of retinoschisin is observed at the extracellular surfaces of the inner segments of rod and cone photoreceptors, most bipolar cells, and the two plexiform layers.24 Corresponding to the localization of retinoschisin, optical coherence tomography revealed schisis of the retina in various retinal layers from the retinal nerve fiber layer to the outer nuclear layer, most prominently occurring in the inner nuclear layer.25 Because it is difficult to achieve the complete posterior vitreous detachment of eyes with CXLRS, Wu et al26 recommended plasmin-assisted vitreoretinal surgery to facilitate complete removal of the vitreous from the retina. However, the procedure for producing the enzyme plasmin is complex, and the intraocular use of plasmin is not approved by the Institutional Review Boards of our institutes. The area of retinoschisis corresponds to the absolute scotoma on the visual field15; therefore, removing the inner wall of retinoschisis itself should not have any detrimental effect on visual function. Based on this idea, IWR was proposed and has been proven to be of benefit by Trese and Ferrone.15,18 In the early cases of this series, the inner wall was preserved based on the report that adhesion between the inner and outer walls of the retina was achieved after vitrectomy by aspirating schisis cavity fluid using a 42-gauge cannula, SO tamponade, and no dissection of inner wall retina.21 In our cases, however, adhesion of the inner and outer walls of the retina was not achieved using the similar techniques; therefore, we needed to perform IWR during reoperation for all four eyes that did not undergo IWR at the initial surgery. Therefore, we believe that preserving the inner wall retina during the surgery offers no advantage for eyes with bullous schisis cavity hanging over or threatening the macula in patients with CXLRS.

The possible disadvantages of IWR include bleeding from the cutting edge of the inner wall retina may occur intraoperatively or postoperatively. Furthermore, it may induce inflammation, leading to postoperative proliferative changes. Finally, IWR may damage the outer wall through accidental aspiration by the vitreous cutter, which may cause RD. However, here, these complications were not a problem, except for minor bleeding during surgery.

To date, few studies have described the detailed surgical procedure of vitrectomy for bullous schisis cavity hanging over or threatening the macula in patients with CXLRS and the selection of the tamponade substance. Silicone oil was first described as an intraocular tamponade for RD in 1962 by Cibis and colleagues.27 The indications for SO tamponade include complex RD attributable to PVR, giant retinal tears, traumatic RD, and some cases of tractional RD.28 There are several advantages of using SO over gas as a tamponade agent. Unlike gas, SO remains in the eye until it is surgically removed, and thus, many surgeons prefer it for all types of RD in children, owing to the difficulties of postoperative positioning and intraocular pressure monitoring.29 Based on the report suggesting that adhesion between the inner and outer walls of the retina was achieved after vitrectomy using SO tamponade,21 we selected SO for intraocular tamponade in the early cases of this case series. However, adhesion between the inner and outer walls of the retina was not achieved. In addition, we experienced difficulty in removing the emulsified SO droplets between the inner and outer walls of the retina. The possible disadvantages of SO tamponade include its inability to provide adhesion between the inner and outer walls, lack of effect on visual function, need for reoperation for SO removal, and the challenge of removing the emulsified SO that may migrate between the inner and outer walls. Further investigation with a larger number of cases is required to clarify the role of SO tamponade in vitrectomy for CXLRS without RD.

The youngest patient who underwent vitrectomy for CXLRS reported to date was aged 6 months, as described by Trese et al.18 Two infants (four eyes) aged 4 and 5 months, respectively, treated in this study are the youngest among all reported patients.10 Although some of the peripheral ballooning retinoschisis was described to regress gradually during the first several years of life, we considered early vitrectomy for eyes with obscuration of the macula results in better anatomic and functional results and decided to perform surgical intervention. Hinds et al30 reported the clinical features of nine patients with bullous CXLRS, including one surgically treated patient, and the average visual acuity was reported to be logarithm of the minimum angle of resolution 1.1 (20/252 in Snellen), which was worse than that of our patients. Favorable surgical and functional outcomes were achieved in most of our patients; therefore, vitrectomy seems effective for restoring a clear visual axis in eyes and maintaining vision for bullous schisis cavity hanging over or threatening the macula in patients with CXLRS.

Nonetheless, a vitrectomy for eyes with CXLRS may have a risk of developing RDs afterward. We experienced one case (Case 3) of postoperative PVR in which a spontaneous retinal tear developed 3 months postoperatively. The retinal tear was presumably located in the posterior edge of the peripheral retinoschisis. The border area may have the risk of developing retinal break, and careful attention should be paid intraoperatively and postoperatively. Long-term, meticulous follow-up is advisable after vitrectomy for eyes with CXLRS.

This retrospective case series had several limitations. Because of the relatively small number of patients, it was difficult to perform adequate statistical analysis. The high variability in the duration of follow-up and lack of uniformity in the severity of the disease could have biased the reported outcomes. Despite these limitations, our study demonstrated the efficacy of IWR and provided useful information regarding surgical procedures for the treatment of CXLRS without RD.

In summary, vitrectomy is effective for restoring a clear visual axis for bullous schisis cavity hanging over or threatening the macula in patients with CXLRS. IWR, but not SO tamponade, seemed to be associated with better surgical results. Further studies are needed to elucidate long-term functional outcomes.


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congenital X-linked retinoschisis; vitrectomy; inner wall retinectomy; silicone oil

Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.