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Macular retinoschisis and macular retinal detachment without hole in highly myopic eyes

Objective　To evaluate the clinical features of macular retinoschisis (MRS) and macular retinal detachment without hole (MRDH) in highly myopic eyes. Methods　The clinical data of 19 patients (24 eyes) with MRS and MRDH from 186 patients (349 eyes) with high myopia were retrospectively analyzed. All of the patients had undergone the examinations of subjective refraction, binocular indirect ophthalmoscope, slit lamp microscope combined with Goldmann threemirror contact lens, fundus images, A/Bscan ultrasonography, and optical coherence tomography (OCT). Results　In 349 eyes, 24 (6.9％) had MRS and (or) MRDH at the posterior pole. The results of ocualr fundus examinations showed that all of the 24 eyes (100%) had posterior scleral staphyloma (PS), 2 (8.3%) had vitreomacular traction (VMT), 2 (8.3%) had macular local superficial retinal detachment, and 1 (4.2%) had fullthickness macular hole. The results of Bscan ultrasonography also indicated PS in all 24 eyes (100%), macular local superficial retinal detachment in 7 (29.2%) with a bowlike configuration formed by the detached retina and the coneshaped roof of PS, and VMT in 2 (8.3%). The results of OCT revealed macular outerlayer retinoschisis (ORS) in 22 eyes (91.7%) in which 8 (36.4%) also had macular innerlayer retinoschisis (IRS); MRDH in 5 eyes (20.8%) in which ORS was found in 3 (60.0%) and simplex MRDH in 2 (40.0%) including 1 with VMT; VMT in 13 eyes (54.2%); cystoid macular edema (CME) in 3 eyes (12.5%); and lamellar macular hole in 4 eyes (16.7%). Conclusions　MRS and MRDH are common complications in highly myopic eyes with posterior scleral staphyloma.OCT is more sensitive and accurate in detecting MRS and MRDH than routine ophthalmoscopic examination and B-scan ultrasonography.

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

ObjectiveTo 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. ResultsThere 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). ConclusionsIn 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.

Long-term efficacy of pars plana vitrectomy with fovea-sparing internal limiting peeling for macular foveoschisis in pathologic myopic

ObjectiveTo observe the long-term clinical effect of pars plana vitrectomy combined with fovea-sparing internal limiting peeling in the treatment of macular foveoschisis in pathologic myopic.MethodsA prospective case series study. Fifteen patients (15 eyes) with pathological myopic macular foveoschisis who received treatment in Eye Hospital of Wenzhou Medical University from December 2015 to December 2016 were enrolled. There were 4 males (4 eyes) and 11 females (11eyes), with an average age of 55.33±8.34 years. All patients underwent BCVA, diopter, spectral domain OCT and axial length measurement. The mean logMAR BCVA was 0.95±0.64. The mean central fovea thickness (CFT) was 576.00±185.32 μm. All patients underwent vitrectomy combined with fovea-sparing internal limiting peeling. After gas-liquid exchange, 12% C3F8 was filled and followed up at 1, 3, 6 and 12 months after surgery. Follow-up time was more than 12 months. The structural changes of BCVA and macular area were observed.ResultsThe foveal internal limiting membranes was successfully preserved in all eyes using the techinique. At the final follow-up, the CFT was 258.60±175.22 μm and the BCVA was 0.46±0.43, which were significantly improved compared with preoperative measurements (t=4.90, 5.20; P＜0.001). Macular foveoschisis was resovled in 13 eyes. BCVA increased in 14 eyes. Internal limiting membranes proliferation and contraction occurred in 5 eyes and full-thickness macular hole occurred in 1 eye.ConclusionsPars plana vitrectomy with fovea-sparing internal limiting peeling is effective in the treatment of myopic macular retinoschisis. It can improve BCVA and CFT.

New mutation site c.361C > T of RS1 gene in X-linked retinoschisis

ObjectiveTo study the characteristics of the genotype and phenotypic in a family with X-linked retinoschisis (XLRS) due to RS1 mutation. MethodsA retrospective clinical study. An XLRS family of 4 generations of 26 people were included in the study. Among them, 8 participants were males and 7 participants were females. Routine ophthalmologic examination was performed on 3 patients in the family including the proband and 12 patients with normal phenotype. Optical coherence tomography was performed in 2 of the 3 patients. Peripheral venous blood was extracted from all participants, whole-genome DNA was extracted, and potential pathogenic genes were screened by Panel sequencing. Conservative analysis, pathogenicity analysis and protein structure prediction were carried out by software tools. The pathogenicity of gene mutations was analyzed according to the American Society of Medical Genetics and Genomics (ACMG) guidelines. ResultsThe proband was 3 years old. Optical coherence tomography (OCT) examination showed that the retinal core layer in the macular area of both eyes had a cystic change, which was segmented by vertical or oblique bridging tissue. The proband's uncle was 32 years old. OCT examination showed atrophy in the macular area of the left eye. The macular area of the right eye was cystoid, segmented by vertical or oblique bridging tissue. No abnormality was found in the fundus examination of the proband's parents and 10 members of his family. Panel sequencing showed that c.361C>T/ p.Q121X hemizygous mutation was found in the fifth exon of RS1 gene in the proband (Ⅳ3) and 2 patients (Ⅱ1, Ⅲ8). The mother was a heterozygous mutation carrier of the gene, while the father had no mutation. The mutant gene causes premature termination of RS1, a truncated protein encoding 224 amino acids to 120 amino acids. Of the 10 patients with normal fundus examination, 6 participants were normal. The mutation was carried by four people, which were women. Homology analysis of the protein sequence showed that the mutant site was highly conserved in 12 mammals. Three-dimensional structural analysis of RS1 protein showed that the c-terminal amino acid sequence of the mutant protein was more than 50% missing. Analysis of ACMG guidelines indicated that the mutation was pathogenic. ConclusionThe RS1 mutation site c.361C>T/p.Q121X is a new mutation site of XLRS.

Anti-vascular endothelial growth factor treatment in X-linked retinoschisis with vitreous hemorrhage

ObjectiveTo review the outcome of intravitreous anti-vascular endothelial growth factor (VEGF) treatment in patients with X-linked retinoschisis (XLRS) complicated with vitreous hemorrhage (VH). MethodsA retrospective clinical study. From March 1, 2016 to April 1, 2022, 18 patients (19 eyes) diagnosed with XLRS complicated with vitreous hemorrhage in Beijing Tongren Hospital, Capital Medical University of Eye Center were included. All the patients were male, with a median age of 7.05±3.8 years. Best corrected visual acuity (BCVA) and wide-angle fundus photography were performed in all the patients. BCVA was carried out using international standard visual acuity chart, and converted into logarithm of minimum resolution angle (logMAR) in statistics analysis. According to whether the patients received intravitreal injection of ranibizumab (IVR), the patients were divided into injection group and observation group, with 11 eyes in 10 cases and 8 eyes in 8 cases, respectively. In the injection group, 0.025 ml of 10 mg/ml ranibizumab (including 0.25 mg of ranibizumab) was injected into the vitreous cavity of the affected eye. Follow-up time after treatment was 24.82±20.77 months. The VH absorption time, visual acuity changes and complications were observed in the injection group after treatment. Paired sample t test was used to compare BCVA before and after VH and IVR treatment. Independent sample t test was used to compare the VH absorption time between the injection group and the observation group. ResultsLogMAR BCVA before and after VH were 0.73±0.32 and 1.80±0.77, respectively. BCVA decreased significantly after VH (t=－3.620, P=0.006). LogMAR BCVA after VH and IVR were 1.87±0.55 and 0.62±0.29, respectively. BCVA was significantly improved after IVR treatment (t=6.684, P＜0.001). BCVA records were available in 5 eyes before and after IVR, and the BCVA values after VH and IVR were 0.58±0.31 and 0.48±0.20, respectively, with no statistically significant difference (t=1.000, P=0.374). BCVA increased in 1 eye and remained unchanged in 4 eyes after treatment. BCVA records were available in 5 eyes before VH and after VH absorption in the 8 eyes of the observation group. LogMAR BCVA before VH and after VH absorption were 0.88±0.28 and 0.90±0.26, respectively, with no significant difference (t=－1.000, P=0.374). After VH absorption, BCVA remained unchanged in 4 eyes and decreased in 1 eye. The absorption time of VH in the injection group and the observation group were 1.80±1.06 and 7.25±5.04 months, respectively. The absorption time of VH was significantly shorter in the injection group than in the observation group, the difference was statistically significant (t=－3.005, P=0.018). Multivariate linear regression analysis showed that IVR treatment was significantly correlated with VH absorption time (B=－6.66, 95% confidence interval －10.93-－2.39, t=－3.40, P=0.005). In the injection group, VH recurrence occurred in 1 eye after IVR treatment. Vitrectomy (PPV) was performed in one eye. In the 8 eyes of the observation group, VH recurrence occurred in 2 eyes, subsequent PPV in 1 eye. The rate of VH recurrence and PPV was lower in the injection group, however, the difference was not statistically significant(P=0.576, 1.000). In terms of complications, minor subconjunctival hemorrhage occurred in 2 eyes and minor corneal epithelial injury occurred in 1 eye in the injection group, and all recovered spontaneously within a short time. In the injection group, 9 eyes had wide-angle fundus photography before and after IVR treatment. There was no significant change in the range of peripheral retinoschisis after treatment. No obvious proliferative vitreoretinopathy, infectious endophthalmitis, retinal detachment, macular hole, complicated cataract, secondary glaucoma or other serious complications were found in all the treated eyes, and there were no systemic complications. ConclusionIntravitreous anti-VEGF treatment may accelerate the absorption of vitreous hemorrhage in patients with XLRS. No impact is found regarding to the peripheral retinoschisis.

The efficacy of vitrectomy combined with internal limiting membrane peeling to treat high myopia macular hole with macular retinoschisis and it affecting factors

ObjectiveTo observe the clinical effects of pars plana vitrectomy (PPV) combined with internal limiting membrane peeling (ILMP) for macular hole (MH) and macular retinoschisis in high myopic eyes, and to analyze factors affecting the MH closure rate.MethodsThis is a retrospective case study. 21 high myopic patients (22 eyes) with MH and macular retinoschisis were enrolled in this study. All eyes were examined for best corrected visual acuity (BCVA), intraocular pressure, slit lamp microscope, indirect ophthalmoscope, A and/or B-scan ultrasound, optical coherence tomography and visual electrophysiological examination. The BCVA was ranged from finger counting to 0.2. The axial length (AL) was ranged from 26.00 to 31.00 mm, with an average of 27.47 mm. Among 22 eyes, AL was between 26.00 mm to 27.00 mm in 9 eyes, 27.10 mm to 28.00 mm in 5 eyes, 28.10 mm to 29.00 mm in 3 eyes, 29.10 mm to 30.00 mm in 3 eyes, and longer than 30.00 mm in 2 eyes. The diameter of MH was ranged from 227 µm to 597 µm and with an average of 432 µm. Among them, the minimum diameter was between 200 µm to 400 µm in 4 eyes, 401 µm to 450 µm in 13 eyes, 451 µm to 500 µm in 3 eyes, 501 µm to 600 µm in 2 eyes. All the eyes were treated with PPV combined with ILMP surgery. The average follow-up time was 17 months after surgery. The efficacy was determined at the final follow up, including the MH closure, the state of macular retinoschisis and the BCVA. MH closure rate with different MH diameters and different AL were compared and analyzed.ResultsDuring the final follow-up, MH were fully closed in 17 eyes (77.3%), bridge-closed in 4 eyes (18.2%) and not closed in 1 eye (4.5%). Retinoschisis was resolved in 19 eyes (86.4%), partially resolved in 2 eyes (9.1%) and not changed in 1 eye (4.4%). MH with smaller diameter had higher MH closure rate (χ2=12.036, P=0.032). MH with longer AL had lower MH closure rate (χ2=16.095, P=0.003).The final BCVA was ranged from finger counting to 0.25. Among 22 eyes, BCVA or metamorphopsia were improved in 9 eyes (40.9%), stable in 8 eyes (36.4%). BCVA was reduced and metamorphopsia was more severe in 5 eyes (22.7%).ConclusionsPPV combined with ILMP is a safe and effective surgical treatment for MH (with minimum diameter ≤600 μm) and macular retinoschisis in high myopic eyes. After surgery, MH was closed and retinoschisis was resolved in most patients. The major factors affect the MH closure were the minimum diameter of MH and AL.

Classification of macular retinoschisis in pathological myopic eyes by optical coherence tomography and its clinical applications

Pathological myopic macular retinoschisis can be classified into 4 types based on optical coherence tomography (OCT) images: outer layer retinoschisis, outer + middle layer retinoschisis, outer + inner layer retinoschisis and multilayer retinoschisis. Currently vitrectomy is the major option to treat this condition as it can remove the posterior vitreous cortex completely and peel the internal limiting membrane (ILM) around the posterior vessels arch. Vitrectomy benefits the visual function significantly for outer layer retinoschisis with foveal detachment, but has no or very little effects on multilayer retinoschisis. The appropriate starting site for removal of posterior cortex and ILM should be the site without inner layer retinoschisis. The knowledge and understanding of the OCT classification of pathological myopic macular retinoschisis is important for us to chose correct operation methods and determine the prognosis after treatment.

多次玻璃体腔C3F8填充治疗先天性视盘小凹合并视网膜劈裂症一例

Objective quantitative assessment of visual quality with a double-pass instrument for myopic foveoschisis patients

ObjectiveTo objectively quantitative assess the visual quality in patients with myopic foveoschisis (MF) using a double-pass optical quality analysis system (OQASⅡ). MethodsSixty-two subjects participated in this cross-sectional, observational study, who were divided into three groups based on the pathologic conditions including myopic foveoschisis group (MFG), myopic control group (MCG) and normal control group (NCG). Measurements with OQASⅡwere performed for the modulation transfer function cut off frequency (MTF cut-off), the Strehl ratio (SR) and the objective scatter index (OSI). Visual data were analyzed using ANOVA and Pearson's correlation accompanied by logMAR BCVA and axial length (AL). ResultsThe mean values for MTF cut-off, SR and OSI of MFG, MCG and NCG were 18.18±4.81, 0.13±0.03, 3.50±0.44; 22.87±2.66, 0.14±0.02, 2.42±0.29; 33.68±4.70, 0.23±0.02, 1.68±0.20 respectively, and statistical difference were proved except SR between MFG and MCG, or BCVA between MCG and NCG (P < 0.05). LogMAR BCVA and AL have negative correlations to MTF cut-off (r=-0.928, -0.658; P < 0.05) and SR (r=-0.577, -0.893; P < 0.05) with high coefficients in MFG. Log MAR BCVA has negative correlations to MTF cut-off and SR (r=-0.659, -0.806; P < 0.05) in MCG. Log MAR BCVA has negative correlations to MTF cut-off and SR (r=-0.606, -0.602; P < 0.05) and positively correlated to OSI (r=0.561, P < 0.05) in NCG. ConclusionsThe mean value of BCVA, MTF cut-off, SR, OSI of myopic foveoschisis patients were lower than those myopic patients without foveoschisis and normal people. there exists a significant negative correlation between Log MAR BCVA, AL to MTF cut-off and SR. Compared with myopic and normal subjects, myopic foveoschisis have lower BCVA, MTF cut-off, SR but higher OSI.

Comparison of the efficacy of vitrectomy combined with complete internal limiting membrane peeling and fovea-sparing internal limiting membrane peeling for high myopia macular foveoschisis

ObjectiveTo observe and compare the outcomes of vitrectomy (PPV) combined with complete internal limiting membrane (ILM) peeling and fovea-sparing ILM peeling for the high myopic foveoschisis (MF).MethodsA retrospective case study was performed. From June 2016 to June 2018, 31 eyes of 31 patients with high myopic MF diagnosed in Department of Ophthalmology of Central Theater Command General Hospital were included in the study. There were 9 males and 22 females, who were monocular. The mean age was 57.55±9.45 years. All patients underwent BCVA, diopter, spectral domain OCT and axial length measurement. Snellen visual acuity chart was used for BCVA examination, and which was converted into logMAR visual acuity. According to the surgical method, patients were divided into PPV combined with ILM complete removal group (group A) and PPV combined with retained fovea ILM group (group B), which were 16 patients (16 eyes) and 15 patients (15 eyes ), respectively. The mean logMAR BCVA was 1.03±0.33 in group A and 1.11±0.35 in group B. The mean CFT was 596.51±196.69 μm in group A and 578.33±200.18 μm. There were no statistically significant differences in age (t=0.649, P=0.527), AL (t=－0.639, P=0.533), logMAR BCVA (t=－0.368, P=0.718), CFT (t=0.228, P=0.823) and MF type (P=0.576) between the two groups. The mean follow-up time after operation was 18.65±5.15 months. At 7 d, 1, 3, 6 and 12 months after surgery, and at the last follow-up, the same equipment and methods for relevant examinations. The changes of BCVA, CFT, macular hole and other complications were compared between the two groups. Comparison of BCVA and CFT between the two groups before and after operation was performed by paired t test. The count data were compared by using Fisher exact probability method.ResultsAt the last follow-up, MF was completely restored in 27 eyes (87.1%) of 31 eyes, partially restored in 4 eyes (12.9%). The mean logMAR BCVA of group A and Group B was 0.67±0.24 and 0.64±0.21, respectively. The average CFT was 126.25±36.61 μm and 134.27±25.29 μm, respectively. Compared with pre-operation, BCVA was obviously improved in both groups (t=6.630, 9.260; P=0.000, 0.000), CFT was significantly decreased in both groups (t=10.206, 8.799; P=0.000, 0.000). There were no statistically significant differences in logMAR BCVA and CFT between the two groups (t=0.156, －0.924; P=0.878, 0.371). In group A, full-thickness macular hole occurred in 1 eye (6.3%), while no macular hole occurred in group B. There was no significant difference in macular hole incidence between two groups (χ2=0.969, P=0.516). No intraocular hemorrhage, endophthalmitis and other complications occurred during the follow-up period.ConclusionsPPV combined with ILM peeling or fovea-sparing ILM peeling is effective in the treatment of high myopic MF. Both may contribute to improved MF closure rate and BCVA.