ObjectiveTo observe the correlation between posterior myopic retinoschisis(MRS) and posterior scleral staphyma (PS) in pathological myopia (PM), and to preliminarily explore the influencing factors of MRS.MethodsA retrospective case series study. From November 2016 to November 2019, 38 patients with PM with MRS diagnosed in Henan Eye Hospital & Henan Eye Institute from were included in the study. There were 10 males and 28 females; 13 patients were binocular and 25 patients were monocular. The average age was (49±13) years old. BCVA, retinoscopy optometry, frequency domain OCT, three-dimensional magnetic resonance imaging (3D-MRI) examination and axial length (AL) measurement were performed. According to the frequency domain OCT inspection results, MRS was divided into inner splitting, outer splitting and mixed splitting; based on the 3D-MRI scan results, PS was divided into broad macula, narrow macula,discoid, nasal, subdisc and other types. The correlation between MRS and PS was tested by χ2 test or Fisher exact test.ResultsAmong 60 eyes, 58 eyes (96.77%) of MRS combined with PS. Among them, the wide macula, narrow macula, discoid, nasal, subdisc, and other types were 30 (51.72%), 19 (32.75%), 1 (1.72%), 2 (3.48%), 2 (3.48%) and 4 (6.85%) eyes; inner split, outer split, and mixed split were 10 (17.24%), 24 (41.38%), 24 (41.38%) eyes. Of the 19 eyes with narrow macular PS, MRS involved the fovea in 16 eyes; of the 39 eyes with PS of other forms, MRS involved the fovea in 22 eyes. There was a statistically significant difference between the narrow macular type and other types involving foveal eyes (P=0.044). The correlation between MRS involving the fovea and narrow macular PS was moderate (Cramer's V=0.275). The ages of patients with inner split, outer split, and mixed split were 44±12, 56±10, and 44±13 years, respectively. Patients with inner splitting were younger than those with outer splitting, and those with outer splitting were older than those with inner splitting and mixed splitting. The differences were statistically significant (P=0.010, 0.010, 0.060).ConclusionPM with MRS mostly occur in PS-affected eyes, and mainly macular PS (wide macula, narrow macula).
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.
We report 19 cases(38eyes)of congenital retinoschisis,whose genetic characteristics conform to x-linked recessive heredity.Maculare lesions were found in all cases and 42.1%(16/38)of involved eyes had peripheral retinschisis.In addition to the typical manifestations of multiple cystic appearance of the central vascular veil,we discovered some infrequent sighs,i.e.displaced macula,peripheral globular retinoschisis,solitary vasculare elevation,and retinoschisis area surrounded by the retinal vasculature. （Chin J Ocul Fundus Dis,1993,9:232-233）
Objective To observe and analyze the imaging features of pathologic myopic paravascular abnormalities (PVA) and macular retinoschisis (MRS) and their relationship with clinical features. MethodsA retrospective case series study. A total of 371 eyes of 224 patients with pathological myopia with PVA and/or MRS diagnosed by examination in Department of Ophthalmology of the First Affiliated Hospital of Zhengzhou University from September 2021 to December 2023 were included in the study. There were 74 eyes in 48 males and 297 eyes in 176 females. Age were 54 (49, 61) years; equivalent spherical lens (SE) was ?13.375 (?18.00, ?10.00) D. Axial length (AL) was 29.84 (28.27, 31.24) mm. According to the features of ultra-wide-angle sweep source optical coherence tomography, PVA morphology was divided into blood vessels and paravascular microfolds, paravascular retinal cysts, paravascular retinal splits and paravascular lamellar macular holes. MRS was divided into inner layer, outer layer and mixed layer according to splitting level, and grouped accordingly. The presence of dome-shaped macula (DSM), internal and external lamellar macular hole, and full lamellar macular hole (FTMH) were recorded. According to whether PVA combined with MRS or not, the affected eyes were divided into PVA group and PVA combined with MRS group. According to whether MRS involved the fovea, the affected eyes were divided into two groups: MRS not involved the fovea group and MRS involved the fovea group. Mann-Whitney U test was used to compare age, SE and AL among different groups. Qualitative data were compared by χ2 test. ResultsIn 371 eyes, there were 120 eyes in the simple PVA group; in the MRS group, there were 251 eyes, of which 208 eyes were in the PVA combined with MRS group. There were 33, 27, 3, 14, 12, 56, 28, and 7 eyes with or without retinal detachment, choroidal neovascularization, DSM, preretinal membrane, and simple superficial detachment of neuroepithelium in inner lamina, outer lamina, and FTMH, respectively. In 328 eyes with PVA, blood vessels and paravascular microfolds, paravascular retinal cysts, paravascular retinal splits and paravascular lamellar macular holes were 151, 236, 202 and 72 eyes, respectively. There were 142 eyes with single lesion (43.29%, 142/328). There were 186 eyes with 2 or more lesions (56.71%, 186/328). There were 34 eyes in the inner MRS group, 92 eyes in the outer MRS group and 125 eyes in the mixed MRS group, respectively. It involved 155 eyes in the fovea group; 96 eyes in the fovea group were not involved. There were significant differences in the number of PVA eyes between the outer MRS group, the inner MRS group and the mixed MRS group (χ2=30.614, 28.379; P＜0.001). Compared with PVA group, PVA group combined with MRS group was more likely to have two or more PVA lesions, the difference was statistically significant (χ2=30.535, P＜0.001). Compared with the inner MRS group and mixed MRS group, the age of the simple PVA group was younger, and the age of the outer MRS group was older, and the differences were statistically significant (P＜0.05). Compared with the inner MRS group and the outer MRS group, the simple PVA group had short AL, less myopia and better optimal corrected visual acuity (BCVA), while the mixed MRS group had long AL, more myopia and worse BCVA, and the differences were statistically significant (P＜0.05). Compared with the PVA group and the inner MRS group, the fovea was more involved in the outer MRS group and the mixed MRS group, and the difference was statistically significant (χ2=3.906, 10.836; P＜0.05). Those with MRS involved in fovea were older, AL was longer, myopia was more severe, and BCVA was worse (P＜0.001). DSM-associated splits were less likely to involve macula (P＜0.001). ConclusionPVA and MRS exhibit a variety of presentations, and their imaging features correlate with clinical features.
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.
ObjectiveTo observe the clinical efficacy of digital 3D heads-up display viewing system (3D viewing system) and intraoperative OCT (iOCT) in vitrectomy for myopic foveoschisis (MF).MethodsA retrospective, consecutive case series. From October 2018 to May 2019, Nineteen eyes of 19 consecutive patients with MF diagnosed in Xiamen Eye Center of Xiamen University who underwent vitrectomy were included in this study. There were 7 males and 12 females, with the mean age of 54.47±11.38 years. The average axial length was 30.40±2.30 mm, the mean logMAR BCVA was 0.56±0.31, the mean central foveal thickness (CFT) was 317.80±151.9.32 μm, the mean max retinal thickness (maxRT) was 556.7±143.7 μm. All the surgeries performed combined with 3D viewing system with iOCT. The standard 25G pars planar vitrectomy were performed with removing the posterior vitreous and indocyanine green (ICG) staining of internal limiting membrane (ILM) and air-fluid exchange. Thirteen of 19 eyes underwent fovea-sparing ILM peeling and the other 6 eyes not. The average follow-up was 4.2±1.4 months. All the patients were on regular follow-up to document the changes on BCVA, anatomical changes in macula, CFT and maxRT. Paired t test was used to compare BCVA, CFT and maxRT before and after surgery.ResultsThe fine images of macula were clearly shown on the 3D viewing system in all eyes. The electronic green filter enhanced the contrast sensitivity of ICG stained images. Clear images of macula were captured by iOCT in all eyes. The average surgical time was 35.5±8.2 min. On the last follow-up, 16 of 19 eyes with MF resolved. The mean CFT was 178.5±103.5 μm, the maxRT was 341.8±83.8.16 μm, and the mean logMAR BCVA was 0.35±0.22. The differences of CFT, maxRT and logMAR BCVA before and after surgery were statistically significant (t=4.181, 7.154, 5.129; P＜0.001). Minimal invisible full thickness macular hole were detected in 2 eyes by iOCT and repaired with auto serum or ILM flap covering. There was no complication associated with the 3D viewing system.Conclusions3D viewing system provides improved contrast and crystal clear macular image stain with ICG in pathological myopia. iOCT can detect the minimal invisible full thickness macular hole during surgery. Both may contribute to improved MF closure rate and BCVA.
Objective To observe the ef?cacy of pars plana vitrectomy with internal limiting membrane (ILM) peeling and gas tamponade in the treatment of myopic macular retinoschisis (MF). Methods This is a retrospective case study. A total of 35 MF patients (36 eyes) were enrolled in this study. There were 5 males (5 eyes) and 30 females (31 eyes), with an average age of (60.13±10.00) years. All patients were examined for best corrected visual acuity (BCVA), diopter, optical coherence tomography (OCT) and axial length. The patients were divided into a MF group (group A, 10 eyes), MF with foveal detachment group (group B, 12 eyes) and MF with lamellar macular hole group (group C, 14 eyes) according to the OCT characteristics. There was no difference of age, gender, spherical equivalent refraction and axial length among 3 groups (F=0.020, 0.624, 0.009, 0.195; P＞0.05). There were significant differences of the minimum resolution angle logarithm (logMAR) BCVA and central fovea thickness (CFT) (F=11.100, 41.790; P＜0.05). All patients underwent pars plana vitrectomy with ILM peeling and gas tamponade. The follow-up was more than one year. The BCVA and macular structure at the final follow-up were analyzed. The efficacy between 3 forms of MF was compared. Results At the final follow-up, the BCVA was 0.40±0.44 and CFT was (213.35±97.58) μm, which were significantly improved compared with preoperative measurements (t=5.984, 5.113; P＜0.001). MF was resolved in 33 eyes. In group A, B and C, the logMAR BCVA were 0.13±0.10, 0.73±0.33 and 0.38±0.52, respectively; CFT was (222.40±57.16), (212.50±150.45), (206.67±55.97) μm, respectively; MF was resolved in 10, 11 and 12 eyes, respectively; complete ellipsoid was observe in 8, 2 and 12 eyes. The logMAR BCVA (F=6.750, P=0.003) and the rate of complete ellipsoid (χ2=18.590, P＜0.001) in group B was lower than group A and C, the differences were significant. There was no difference of CFT (F=0.068, P=0.935) and the rate of MF resolving (χ2=1.558, P=0.459) among the three groups. One eye (1/14) in group C suffered from full layer macular hole. Conclusion Pars plana vitrectomy with ILM peeling and gas tamponade is effective in the treatment of myopic macular retinoschisis. The macular structures and BCVA are worst in eyes with foveal detachment.
Objective To evaluate the efficacy of vitrectomy with internal limiting membrane（ILM）peeling and perfluoropropane tamponade (C3F8) to treat macular retinoschisis in high myopic eyes.Methods 33 eyes of 31 consecutive high myopia patients with macular retinoschisis were selected randomly; all had posterior staphyloma without retinal detachment. The preoperative refractive errors ranged from －9.5D to －21.0 D with the mean of －(13.1plusmn;3.6) D. The preoperative axial lengths ranged form 26 mm to 32 mm with the mean of (28.3plusmn;2.1) mm. Conventional 20G vitrectomy was performed with ILM peeling and 10% C3F8 infusion, ILM was labeled by Triamcinolone (TA). The best corrected visual acuity (BCVA) and macular structural changes were observed before the surgery, and at 1, 2, 3, 4, 8 months after the surgery. Results Beginning from 1 month after surgery all patients had significant improvement of the macular retinoschisis and BCVA. The macular structure changed very slightly along with the time. The foveal thickness were (327.6plusmn;51.7),(165.2plusmn;22.6),(159.3plusmn;28.7),(167.7plusmn;17.1),(142.7plusmn;13.8) and (169.1plusmn;19.6) mu;m respectively before surgery and 1, 2, 3, 4, 8 months after surgery. The mean foveal thickness was reduced significantly at 18 months followup compared with the preoperational result (t=9.21，9.23，9.21，10.67，9.21; Plt;0.05). The foveal thickness had no significant change at each timepoint after surgery.From 4 months after surgery, recurrence of macular retionoschisis was found in 3 eyes (9.1%).Conclusion Vitrectomy with ILM peeling and C3F8 tamponade is useful to treat macular retinoschisis in high myopic eyes.
ObjectiveTo observe the imaging features of optical coherence tomography (OCT) in peripheral retinal abnormalities of high myopia (HM). MethodsA retrospective series of case studies were conducted. From March 2019 to March 2021, 38 cases (50 eyes) in high myopia with peripheral retinal abnormalities who were confirmed to Henan Eye Hospital were enrolled in the study. There were 21 eyes in 17 males and 29 eyes in 21 females, age was 39.58±15.29 years, diopter was (－9.10±2.44) D. All patients underwent wide-angle fundus photography and OCT examination. According to wide-angle fundus photography and OCT, HM with peripheral retinal abnormalities were classified into white-without-pressure, black-without-pressure, lattice degeneration, peripheral pigmented degeneration, retinoschisis and retinal holes. OCT imaging features of peripheral abnormalities in high myopia was observed. ResultsIn 50 eyes, 65 peripheral retinal abnormalities were observed by OCT. In 6 white-without-pressure, intense hyperreflectivity was shown at the level of the ellipsoid zone that abruptly transitions to relative hyporeflectivity at the dark border of the lesion. In 16 black-without-pressure, reflectivity of the ellipsoid zone decreased. In 10 sites of lattice degeneration, cystoid degeneration, local thinning, retinal tear at the posterior edge and boundary of the lesion was shown, whcih may be accompanied by local vitreous condensation and traction. In 4 peripheral pigmented degeneration, retinal interlayer hyperreflectivity was shown. In 12 retinoschisis, neuroepith-elial separation was connected by vertical bridge or columnar light bands, of which 3 were accompanied with localized retinal detachment and 2 with splitting-related retinal vascular abnormalities. In 17 retinal holes, full layer of neuroepithelium lost, that 12 zones were accompanied with retinal detachment with vitreous adhesion or traction. ConclusionOCT manifestations of peripheral retinal abnormalities in HM varies.
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.