ObjectiveTo evaluate the outcomes of laser photocoagulation of congenital X-linked retinoschisis (XLRS) at progressive stage. MethodsTwenty-seven cases (36 eyes) of XLRS sick kids were enrolled in this study. All patients were followed up for more than 1 year, retinoschisis has developed slowly but complications occurred during the follow-up. They are all boys from 3 to 12 years old; the average age was 6.47 years old. There were 18 unilateral cases, 9 bilateral cases. The affected eyes were randomly divided into treatment group and control group (n=18 eyes). The treatment group eyes received multi-wavelength krypton yellow laser photocoagulation around the retinoschisis, but no laser spots were laid in a optic-disk area surrounding the macular and optic disc. Children in the control group were followed up every six months without treatment. Both groups of children were followed up for 3 years. The best corrected visual acuity (BCVA, logMAR), complications (vitreous hemorrhage, retinal detachment) were measured at the last follow up. ResultsAt the last follow-up, the treatment group mean logMAR BCVA was 0.73±0.41, which is the same as pre-treatment BCVA (t=1.187, P=0.201). The control group mean logMAR BCVA 0.88 ±0.60, which is the same as pre-treatment BCVA (t=-2.093, P=0.033). The changes of the BCVA in these two groups was statistically different (t=-2.093, P=0.033). For the treated 18 eyes, visual acuity improved in four eyes (22.2%); not changed in 10 eyes (55.6%) and decreased in four eyes (22.2%). For the 18 eyes in the control group, visual acuity improved in three eyes (16.7%); not changed in four eyes (22.2%) and decreased in 11 eyes (61.1%). The vision reduction rate in treatment group was statistically less than the control group (χ2=5.600, P<0.01). There were 2 eyes (11.1%) and 7 eyes (38.9%) with serious complications in the treated and control eyes respectively. The complication rate treatment group was statistically less than the control group (χ2=3.710,P<0.05). ConclusionLaser photocoagulation can stabilize or improve vision of advanced XLRS patients, and prevent the occurrence of serious complications.
Objective To observe the efficacy 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.
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.
X-linked retinoschisis (XLRS) is a rare X-linked inherited retinal disorder, caused by mutations in retinoschisin 1 (RS1) gene. Three XLRS mice were established, providing ideal systems to study the mechanism and treatment methods for XLRS. RS1 gene mutations can induce abnormal secretion or adhesion function of RS1 protein. In the past year, phase I clinical trials for XLRS has begun in USA, using adeno associated virus (AAV, AAV8 or AAV2)-mediated gene delivery. With the rapid development of new generation of AAV vector that can transduce more retinal cells through intravitreous delivery, gene therapy for XLRS will have a brighter future.
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.
ObjectiveTo evaluate the efficacy of vitrectomy with internal limiting membrane peeling without intraocular tamponade in the treatment of myopic foveoschisis. MethodsTwenty-three eyes of 23 patients with myopic foveoschisis underwent vitreoretinal surgery were analyzed retrospectively. All the patients had undergone the examinations of best corrected visual acuity (BCVA), intraocular pressure, slit lamp microscope, direct ophthalmoscope, A or B ultrasonic scan and optical coherence tomography(OCT).The mean BCVA was 0.02-0.4, mean diopter was (-14.1±3.8) D, mean axial length was (28.8±1.5) mm, mean central fovea thickness (CFT) was (573.2±142.8) μm. A standard 3-port pars plana vitrectomy (25-gauge system) was performed in all patients. There was no tamponade at the end of the operation. The follow-up varied from 6 to 28 months. The visual acuity, CFT, retinal reattachment and the complications were observed. ResultsAt the latest follow up, there were 16 eyes (69.6%) were anatomically reattached, 4 eyes (17.4%) were partly anatomically reattached, 3 eyes (13.0%) were not reattached. Postoperative BCVA improved in 22 eyes (52.2%), unchanged in 9 eyes (39.1%), and decreased in 2 eyes (8.7%). No ocular complications such as macular hole, fundus hemorrhage, low or high intraocular pressure, endophthalmitis were found. ConclusionVitrectomy with internal limiting membrane peeling without gas tamponade can effectively treat myopic foveoschisis without ocular complications.
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.
Objective To observe the the clinical characteristics of images of optic coherence tomography (OCT) in highly myopic eyes with retinoschisis. Methods The clincial data of 158 patients (158 eyes) with high myopia diagnosed by examinations of best corrected visual acuity and refraction, indirect stereoscopic ophthalmoloscopy, A/Bscan ultrasonography, and OCT, were retrospectively analyzed. The patients were divided into retinoschisis group and nonretinoschisis group according to the results of OCT (whether the patients had macular reinoschisis at the posterior pole). There were 53 patients (55 eyes, 34.8%) in the former group, and 101 patients (103 eyes, 65.2%) in the latter group. The age, sex, diopter, visual acuity, ocular axial length, and incidence of posterior scleral staphyloma, vitreous traction, and retinal detachment of the two groups were compared. Results B-scan ultrasonography showed posterior scleral staphloma in all of the 158 eyes. OCT indicated that in the 55 eyes in the retinnoschisis group, 15 （27.3%） had inner, 53 （96.4%）had outer, and 7 （12.7%）had middle retinoschisis. The inner and outer one could exist independently or in the same eye, while the middle one was always accompanied by the outer retinoschisis. Two or more types of schisis coexisted in 13 eyes （23.6%）, single outer retinoschisis was found in 40 eyes （72.7%）, and single inner retinoschisis was found in 2 eyes （3.6%）. There were 26 eyes（47.3%）were accompanied with retinal detachment, 13 eyes（23.6%） with macular hole, and 12 eyes （21.8%）with vitreous traction. In the 103 eyes in the nonretinoschisis group， 23 eyes （22.3%）had vitreous traction, 19 eyes （18.4%） had macular hole, and 21 eyes （20.4%）had retinal detachment. The differences of age, diopter and ocular axial length, sex, incidence of macular hole and vitreous traction between the two groups were not statistically significant (Pgt;005）. The visual acuity in retinoschisis group was much lower than that in the nonretinoschisis group （Plt;005）, and the difference of incidence of the retinal detachment between the two groups was significant (Plt;001). 〖WTHZ〗Conclusion 〖WTBZ〗Macular retinoschisis in eyes with high myopia can exist in inner or middle retina, but most of them locate at outer retina.The patients always have poor visual acuity and are often accompanied by other macular lesions such as retinal detachment.
ObjectiveTo observe the imaging features of extramacular retinoschisis (EMRS) and paravascular abnormalities (PVA) in myopic patients, and preliminary analyze the differences in age, best corrected visual acuity (BCVA), spherical equivalent (SE), axial length (AL), and subfoveal choroidal thickness (SFCT). MethodsA cross-sectional clinical study. A total of 60 myopia patients with EMRS who were admitted to Department of Ophthalmology of The First Affiliated Hospital of Zhengzhou University from January 2023 to June 2024 were included in the study. There were 18 male cases with 18 eyes and 42 female cases with 42 eyes. Age was (37.57±17.14) years; SE was (−10.76±4.66) D; AL was (28.36±1.87) mm. According to the characteristics of ultra-wide-angle optical coherence tomography images, PVA was divided into perivascular cysts (PC), perivascular microfolds (PM) and perivascular lamellar holes (PLH). According to the splitting level, EMRS can be divided into inner layer, middle layer and outer layer. According to SE, the affected eyes were divided into low myopia group, moderate myopia group and high myopia group. The occurrence of EMRS near optic disc, supratemporal, suprasal and subnasal, as well as the clinical characteristics of patients with EMRS at different locations, levels and forms of PVA were observed. Age, BCVA, SE, AL and SFCT of EMRS patients at different locations and levels were compared by independent sample t test. χ2 test or Fisher exact probability test were used to compare the categorical variables between groups. ResultsIn 60 eyes, EMRS were located in supratemporal, infratemporal, supranasal, subnasal, and paratopic discs in 36, 43, 15, 13, and 14 eyes, respectively. The EMRS in the inner and outer layers were 59 (98.3%, 59/60) and 35 (58.3%, 35/60) eyes, respectively. PVA was present in 47 eyes (78.3%, 47/60). Among them, PC, PM and PLH were 45, 39 and 18 eyes, respectively. The age of those with paratopic splitting was older than those without paratopic splitting (t=2.720). Those with temporal splitting had worse BCVA and longer AL than those without splitting (t=2.139, 2.119). Those with subnasal splitting had worse BCVA, higher myopia, longer AL and thinner SFCT than those without splitting. The differences were statistically significant (t=2.926, −2.640, 2.635, −3.938; P＜0.05). Compared with other types of EMRS, patients with inner EMRS had younger age (t=−2.383), better BCVA (t=−4.825), shorter AL (t=−4.767), lower myopia (t=4.791), and thicker SFCT (t=4.791); patients with full-layer EMRS were older (t=2.419), worse BCVA (t=3.656), longer AL (t=2.677), higher degree of myopia (t=−2.755), and thinner SFCT (t=−3.283), with statistical significance (P＜0.05). There was significant difference in SFCT among patients with or without PC (t=−2.396, P＜0.05). Compared with eyes without PM and PLH, eyes with PM had worse BCVA, longer AL, higher myopia, and thinner SFCT, and the differences were statistically significant (PM: t=2.514, 3.078, −2.811, −4.205; P＜0.05; PLH: t=2.514, 2.992, −2.949, −1.773; P＜0.05). ConclusionsEMRS primarily occurs in the temporal side, with the highest frequency in the inner layer. Patients with inner-layer EMRS are younger, have better BCVA, shorter AL, lower myopia, and thicker SFCT, whereas patients with full-layer EMRS exhibit the opposite characteristics.