MIXED PATHOPHYSIOLOGIES OF LAMELLAR MACULAR HOLES AND RELATED DISEASES: A Multimodal Optical Coherence Tomography-Based Study.

PURPOSE: To investigate the characteristics of mixed pathophysiologies in lamellar macular holes (LMHs) and related diseases using multimodal optical coherence tomography. METHODS: Overall, 126 eyes diagnosed with LMH, epiretinal membrane foveoschisis, or macular pseudohole using the horizontal B-scan image according to the definition proposed by Hubschman et al in 2020 were analyzed using multimodal optical coherence tomography imaging including horizontal and vertical 5-line B-scan, radial scan, and macular three-dimensional volume scan images. If at least two diagnostic criteria for LMH, epiretinal membrane foveoschisis, or macular pseudohole were satisfied in these scans, the patient was diagnosed as having a "mixed type." Retinal traction force was quantitatively evaluated by measuring the maximum depth of the retinal folds using en-face images. RESULTS: Mixed types constituted 34.1% of the cases. The LMH-related mixed group demonstrated intermediate characteristics between the epiretinal membrane foveoschisis/macular pseudohole and true LMH groups in terms of retinal traction and LMH-specific features and had a significant positive correlation between the maximum depth of the retinal folds and mean M-CHARTS scores (P = 0.034). CONCLUSION: A thorough optical coherence tomography analysis is necessary to accurately diagnose LMH and related diseases. A significant positive correlation was observed between the maximum depth of the retinal folds and the degree of metamorphopsia in the LMH-related mixed group.

Stellate nonhereditary idiopathic foveomacular retinoschisis and an approach to the differential diagnosis of macular star.

PURPOSE OF REVIEW: This review aims to introduce stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) and its differential diagnosis. We summarize findings from case reports and series published in the last few years on the clinical and imaging findings in SNIFR. RECENT FINDINGS: SNIFR presents as either a unilateral or bilateral macular star on fundus examination without clinical or imaging evidence of exudation or frank vitreomacular traction. optical coherence tomography (OCT) imaging shows schisis cavities in the Henle fibre and outer plexiform layers that correspond to the stellate en face findings. Visual acuity is usually minimally affected, and the presence of significant vision loss should prompt high clinical suspicion for alternate diagnoses. SUMMARY: SNIFR is a recently characterized clinical entity that serves as an important addition to the differential diagnosis of a macular star. It is a diagnosis of exclusion and should be distinguished from other causes of macular star such as neuroretinitis, vitreomacular traction, ocular manifestations of malignant hypertension, congenital juvenile X-linked macular schisis, myopic maculopathy, optic pit maculopathy, nicotinic acid maculopathy or taxane maculopathy among others.

MACULAR MICROVASCULATURE IN X-LINKED RETINOSCHISIS: Optical Coherence Tomography and Optical Coherence Tomography Angiography Study.

PURPOSE: The study aimed to evaluate the macular microvasculature of X-linked retinoschisis (XLRS) and identify correlations between vascular changes, structural changes, and functional outcome. METHODS: Genetically confirmed XLRS patients and heathy control subjects underwent complete ophthalmic examination, dilated funduscopic examination, optical coherence tomography, and optical coherence tomography angiography. Schisis distribution, outer plexiform layer discontinuation, photoreceptor layer thickness, and photoreceptor outer segment length were reviewed using optical coherence tomography. Vascular flow density and foveal thickness at foveal and parafoveal area were measured using optical coherence tomography angiography. RESULTS: A total of 17 eyes of 9 XLRS patients and 22 eyes of 11 control subjects were examined from July 2018 to August 2020. Flow density in the deep capillary plexus at foveal and parafoveal area decreased in XLRS patients compared with control subjects (P = 0.014 and 0.001, respectively), whereas foveal avascular zone area and perimeter remarkably increased (P = 0.015 and 0.001, respectively). Although outer and total retinal layers were significantly thicker in XLRS, inner retinal layer was thinner with reduced photoreceptor layer thickness and shortened photoreceptor outer segment length (P < 0.001 and P < 0.001, respectively). Foveal flow loss in deep capillary plexus, foveal avascular zone enlargement, thinner inner retina and photoreceptor layer thickness, and shortened photoreceptor outer segment length correlated with best-corrected visual acuity. CONCLUSION: X-linked retinoschisis eyes exhibit decreased flow density in the deep capillary plexus and variable foveal avascular zone with enlarged perimeter. Structural deterioration of the photoreceptor best reflects the degenerative changes, whereas microvascular alteration shows considerable correlation with functional outcome in XLRS.

Specific Changes in OCT Imaging Associated with a Novel Mutation of the RS1 Gene in X-Linked Retinoschisis. / Spezifische OCT-Veränderungen bei einer neuen Mutation im RS1-Gen bei X-chromosomal-rezessiver Retinoschisis.

X-linked retinoschisis (XLRS) is a rare vitreoretinal dystrophy caused by molecular genetic changes in the RS1 gene. It usually manifests itself at a young age with symmetrical splitting within different layers of the retina and leads to a significant reduction in visual acuity. Correct diagnosis at older ages is difficult due to nonspecific changes in OCT scans. We report the morphological changes in OCT scans at different stages of life in a family with XLRS and a novel mutation in the RS1 gene. Our 78-year-old index patient presented with visual disturbances that he had experienced since his childhood. After a detailed anamnesis, complete clinical examination and measurement with SD-OCT, we performed germline genetic testing using whole blood DNA on the index patient, his clinically unaffected daughter and her clinically affected son. The OCT examination of the index patient showed nonspecific atrophic macular changes on both sides. A fundoscopy of the 8-year-old grandson showed the typical macular star pattern. The OCT scan showed the typical retinoschisis of the macula. The genetic analysis revealed the previously undescribed pathogenic variant c.487T>G; p.Trp163Gly in the RS1 gene in all 3 patients. The typical fundus image and OCT pattern, which are absent in the 78-year-old patient, are also present in childhood with the novel RS1 mutation. Our case shows that even with nonspecific changes in the OCT scans, a detailed family history can provide important information on X-linked recessive inheritance and thus for an appropriate molecular genetic diagnosis, so that rare retinal diseases can be diagnosed even at an advanced age.

Importance of Paravascular Vitreal Adhesions for Development of Myopic Macular Retinoschisis Detected by Ultra-Widefield OCT.

PURPOSE: To examine most postequatorial retina in eyes with myopic macular retinoschisis (MRS) by ultra-widefield (UWF) OCT and to determine whether paravascular vitreal adhesions play a role in the development of MRS. DESIGN: Retrospective single-center observational case series. PARTICIPANTS: One hundred fifty highly myopic participants who were older than 50 years with and without an MRS were studied. High myopia was defined as an eye with an axial length of more than 26.5 mm. METHODS: All participants underwent UWF OCT imaging with a scan width of 23 mm and a depth of 5 mm using a prototype swept-source OCT device. The vitreoretinal adhesions to the foveal retina and retinal vessels and paravascular abnormalities, including paravascular retinal cysts, paravascular retinoschisis, and paravascular lamellar holes, were analyzed in the UWF OCT images. The findings in eyes with an MRS were compared with those in eyes without an MRS. MAIN OUTCOME MEASURES: The relationships between MRS and vitreal adhesions to the retinal vessels or to the fovea were determined. RESULTS: An MRS was found in 49 of the 150 eyes (33%). Vitreal adhesions to the retinal vessels were found more frequently in eyes with an MRS than in eyes without an MRS (63% vs. 44%; P = 0.04). In contrast, the number of eyes with adhesions to the fovea in eyes with an MRS was not significantly different from that in eyes without an MRS (57% vs. 59%). Paravascular lesions, for example, retinal cysts, retinoschisis, and lamellar holes, were more common in eyes with an MRS than in eyes without an MRS (71% vs. 36%, 61% vs. 17 %, and 20% vs. 8% [P < 0.001, P < 0.001, and P = 0.03], respectively). Multivariate analysis showed that the presence of paravascular vitreal adhesions was a significant predictor for MRS development (odds ratio, 2.56; P = 0.02). CONCLUSIONS: Paravascular vitreal adhesions may be related to the development of the different types of paravascular lesions including retinal cysts and retinoschisis, and play a more important role in the development of an MRS than vitreal adhesions to the fovea.

[A case of microvascular anomalies in myopic retinoschisis].

It is a case found during routine reexamination one year after implantable Collamer lens (ICL) implantation. The patient had no complaints. The naked eye visual acuity of the left eye was 1.0, and abnormal blood vessels were seen in the supranasal retina. After fluorescein fundus angiography and sweep source OCTA, it was finally diagnosed as retinoschisis with microvascular anomalies of the left eye. This case suggests that the fundus of patients with high myopia without complaint should also be examined in detail and comprehensively. In addition to paying attention to peripheral retinopathy, the posterior pole and middle peripheral retina should be carefully examined, especially the areas that cannot be covered by conventional OCT.(Chin J Ophthalmol, 2021, 57: 944-945).

[Macular choroidal volume in patients with highly myopic foveoschisis and its clinical value].

Objective: To study the macular choroidal volume (MCV) of patients with highly myopic foveoschisis and its clinical value. Methods: In this cross-sectional study, 39 outpatients (39 eyes) with highly myopic foveoschisis were included from January 2016 to December 2020 in Peking University People's Hospital, including 18 males and 21 females. Their age was (59.3±6.7) years old. Thirty-nine highly myopic patients (39 eyes) with no macular complications were enrolled as control group. The age, gender, and refractive error were matched between two groups. Medical history information and eye examination information including refractive error, axial length and best corrected visual acuity were recorded. All patients had undergone high-resolution enhanced depth imaging optical coherence tomography to measure the retinal and choroidal thickness of multiple parts of the macular zone. According to the image characteristics, myopic foveoschisis patients were divided into the inner and outer myopic foveoschisis subgroups, and the MCV characteristics were analyzed. The independent sample t test, Pearson correlation analysis and linear regression analysis were used. Results: The subfoveal choroidal thickness was (74.9±59.3) and (155.6±47.1) µm, and MCV was (2.3±0.8) and (5.3±1.0) mm3 in the foveoschisis group and the control group, respectively. The differences were statistically significant (t=-6.649, -15.229; P<0.01). Although no correlation was found between subfoveal choroidal thickness and central foveal thickness in both groups (r=0.103, 0.214; P>0.05), MCV was negatively correlated with macular retinal volume (MRV) in the foveoschisis group (y=-2.90x+18.48; r2=0.47, P= 0.01). In the control group, there was a positive correlation between MCV and MRV (y=0.74x+2.02; r2=0.64, P=0.01). The best corrected visual acuity was positively associated with MCV in patients with foveoschisis (r=0.677, P<0.05). The MCV of inner (19 eyes) and outer (15 eyes) foveoschisis subgroups was (2.80±0.81) and (1.92±0.27) mm3, and the difference was statistically significant (t=4.610, P<0.05). Conclusions: The MCV significantly decreased in patients with highly myopic foveoschisis. The smaller the MCV, the scarcer the blood supply of the outer retina, the more serious the foveoschisis, and the larger the MRV. (Chin J Ophthalmol, 2021, 57:419-425).

Clinical findings and RS1 genotype in 90 Chinese families with X-linked retinoschisis.

Purpose: X-linked retinoschisis (XLRS) is an early-onset retinal degenerative disorder caused by mutations in the RS1 gene. The objective of this study was to describe the clinical and genetic findings in 90 unrelated Chinese patients with XLRS. Methods: All patients underwent clinical examination, including best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, fundus examination, and spectral domain-optical coherence tomography (SD-OCT). A combination of molecular screening methods, including Sanger-DNA sequencing of RS1 and targeted next-generation sequencing (TES), were used to detect mutations. In silico programs were used to analyze the pathogenicity of all the variants. Long-range PCR with subsequent DNA sequencing was employed to find the breakpoints of large deletions. Results: The 90 probands (mean age 17.29±12.94 years; 3-52 years) showed a variety of clinical phenotypes, and their average best correct visual acuity was 0.81±0.48 (logarithm of the minimal angle of resolution, 0-3). Of the 175 eyes analyzed, 140 (80%) had macular retinoschisis, 84 (48%) had peripheral retinoschisis, 28 (16%) had macular atrophy, and five (3%) had a normal macular structure. We identified 68 mutations in this cohort of patients, including 15 novel mutations. Most mutations (65%) were missense; the remaining null mutations included nonsense, splicing effect, frameshift indel, and large genomic DNA deletions. The 62 patients with missense mutations seemed to have relatively milder visual defects than the 28 patients with null mutations. Conclusions: Patients with RS1 mutations present profound phenotypic variability and show no clear genotype-phenotype correlations. Patients with null mutations tend to have more severe XLRS-related visual defects.

High myopic patients with and without foveoschisis: morphological and functional characteristics.

PURPOSE: Myopic foveoschisis (MF) is characterized by the splitting of the retinal layers in the fovea of patients with high myopia (HM). MF may progress into foveal detachment or macular hole formation with consequent loss of central vision. The aim of this study is to investigate morphological and functional changes of the macular region in myopic subjects with and without foveoschisis. DESIGN: Observational, cross-sectional, comparative study. METHODS: Forty-eight patients with HM and 24 healthy controls were evaluated by spectral domain-optical coherence tomography (SD-OCT), multifocal electroretinography (mfERG) and microperimetry (MP-1) tests to assess macular thickness, functionality and sensitivity values, respectively. The results of the diagnostic examinations were compared between three groups: HM patients with MF (N = 24), HM patients without MF (N = 24) and control group (CG) (N = 24). All statistical analyses were performed with STATA 14.0 (Collage Station, Texas, USA). One-way analysis of variance (ANOVA) followed by Tukey's post hoc test was used to analyze differences between groups unless specified; p values < 0.05 were considered as statistically significant. Gender distribution was compared by the Chi square test. RESULTS: The statistical analysis with one-way ANOVA followed by Tukey's post hoc test showed a significant increase in macular thickness in HM patients with MF when compared to both HM patients without MF and CG. Morphological changes were associated with functional impairment as demonstrated by the significant decrease in amplitude of the P1 wave and MP-1 sensitivity (p < 0.05), according to the anatomical landmarks. CONCLUSIONS: This study showed that the morphological changes observed in the central retina of HM patients with MF are associated with functional alterations. High-tech diagnostic tests such as SD-OCT, mfERG and MP-1 could be useful for management in complications of MF.

HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD.

BACKGROUND/PURPOSE: Using handheld spectral domain optical coherence tomography (SDOCT) imaging to investigate in vivo microanatomic retinal changes and their progression over time in young children with juvenile X-linked retinoschisis (XLRS). METHODS: This retrospective analysis was of handheld SD OCT images obtained under a prospective research protocol in children who had established XLRS diagnosis based on genetic testing or clinical history. Three OCT graders performed standardized qualitative and quantitative assessment of retinal volume scans, which were divided into foveal, parafoveal, and extrafoveal regions. Visual acuity data were obtained when possible. RESULTS: Spectral domain OCT images were available of both eyes in 8 pediatric patients with ages 7 months to 10 years. The schisis cavities involved inner nuclear layer in over 90% (15/16) of eyes in all 3 regions. Retinal nerve fiber and ganglion cell layer involvement was present only in the extrafoveal region in 63% (10/16) eyes and outer nuclear and plexiform layer in few others. In 7 children followed over 2 months to 15 months, the location of schisis remained consistent. Central foveal thickness decreased from the baseline to final available visit in 4/6 eyes. Ellipsoid zone disruption seemed to accompany lower visual acuity in 1/4 eyes. CONCLUSION: Early in life, the SD OCT findings in XLRS demonstrate differences in schisis location in fovea-parafoveal versus extrafoveal region, possible association between poor visual acuity and degree of ellipsoid zone disruption and decrease in central foveal thickness over time in this group. Furthermore, they illustrates that the pattern of XLRS in adults is already present in very young children, and unlike in older children and adults, those presenting with earlier disease may have a more aggressive course. Further studies in this early age group may provide more insights into treatment and prevention of progressive visual impairment in children with XLRS.

Case Report: Glaucoma-associated Peripapillary Retinoschisis with Corresponding Lamina Cribrosa Defect.

SIGNIFICANCE: Peripapillary retinoschisis is associated with primary and secondary glaucoma. It is important that clinicians are familiar with the presentation and management of peripapillary retinoschisis to understand its effects on the patient's glaucoma and to avoid unnecessary referral when the macula is not involved. PURPOSE: We present a case of peripapillary retinoschisis found incidentally on routine optical coherence tomographic (OCT) surveillance of primary open-angle glaucoma. CASE REPORT: A 70-year-old man presented for his annual diabetic eye examination. Surveillance with OCT revealed a splitting of the inner peripapillary retina corresponding to a previously noted notch in the right optic nerve. Further imaging of the right eye using enhanced depth imaging OCT revealed a defect in the lamina cribrosa that may have contributed to the formation and persistence of peripapillary retinoschisis. Retinal nerve fiber layer analysis showed a 5-year history of progressive temporal and inferotemporal thickening in the right eye. The patient was managed conservatively with instruction on regular Amsler grid testing. CONCLUSIONS: As seen in this case, peripapillary retinoschisis typically alters retinal nerve fiber layer thickness on OCT and can be mistakenly attributed to glaucomatous change. Glaucoma-associated peripapillary retinoschisis is usually not vision threatening and can be managed conservatively; in rare cases of progression to macular involvement, patients should be referred to a retina specialist.

CAPILLARY NETWORK ALTERATIONS IN X-LINKED RETINOSCHISIS IMAGED ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To assess foveal and parafoveal vasculature at the superficial capillary plexus, deep capillary plexus, and choriocapillaris of patients with X-linked retinoschisis by means of optical coherence tomography angiography. METHODS: Six patients with X-linked retinoschisis (12 eyes) and seven healthy controls (14 eyes) were recruited and underwent complete ophthalmologic examination, including best-corrected visual acuity, dilated fundoscopy, and 3 × 3-mm optical coherence tomography angiography macular scans (DRI OCT Triton; Topcon Corp). After segmentation and quality review, optical coherence tomography angiography slabs were imported into ImageJ 1.50 (NIH; Bethesda) and digitally binarized. Quantification of vessel density was performed after foveal avascular zone area measurement and exclusion. Patients were additionally divided into "responders" and "nonresponders" to dorzolamide therapy. RESULTS: Foveal avascular zone area resulted markedly enlarged at the deep capillary plexus (P < 0.001), particularly in nonresponders. Moreover, patients disclosed a significant deep capillary plexus rarefaction, when compared with controls (P: 0.04); however, a subanalysis revealed that this damage was limited to the fovea (P: 0.006). Finally, the enlargement of foveal avascular zone area positively correlated with a decline in best-corrected visual acuity (P: 0.01). CONCLUSION: Prominent foveal vascular impairment is detectable in the deep capillary plexus of patients with X-linked retinoschisis. Our results correlate with functional outcomes, suggesting a possible vascular role in X-linked retinoschisis clinical manifestations.

OBSERVATION OF VITREOUS FEATURES USING ENHANCED VITREOUS IMAGING OPTICAL COHERENCE TOMOGRAPHY IN HIGHLY MYOPIC RETINOSCHISIS.

PURPOSE: To observe features of the posterior vitreous and vitreoretinal interface in highly myopic eyes with retinoschisis using enhanced vitreous imaging optical coherence tomography. METHODS: Comprehensive ophthalmologic examination and enhanced vitreous imaging optical coherence tomography were performed in 77 eyes of 63 patients with highly myopic retinoschisis. Two different modes of spectral domain optical coherence tomography were employed to estimate retinoschisis and the posterior vitreous features in optical coherence tomography images, respectively. The types and distribution of vitreoretinal interface abnormalities were also analyzed. RESULTS: Complete posterior vitreous detachment (PVD) was identified in 55 eyes (71.4%) with a Weiss ring. Residual cortex was found in 39 eyes (70.9%) with complete PVD. Vitreoretinal interface changes, including vitreoretinal adhesion and epiretinal membrane (ERM), most frequently appeared in the macular area (47.3%), followed by the inferior arched vessels region (34.5%). In partial PVD eyes, vitreoretinal traction, vitreoretinal adhesion, and epiretinal membrane tended to be observed in the inferior and superior arched vessels regions (54.5 and 40.9%, respectively). Among all types of vitreoretinal interface abnormalities, epiretinal membrane comprised the largest proportion (46.8%) despite the status of PVD. The presence of inner layers of retinoschisis connoted a relatively high possibility of vitreoretinal interface abnormalities occurring. CONCLUSION: Enhanced vitreous imaging optical coherence tomography reveals a high prevalence of vitreoretinal interface abnormalities in highly myopic eyes with retinoschisis. Vitreous cortex tends to remain on the macular area in eyes with complete PVD. Our findings may lead to better guidance for the surgical treatment of highly myopic retinoschisis.

Multimodal imaging in a pedigree of X-linked Retinoschisis with a novel RS1 variant.

BACKGROUND: To describe the clinical phenotype and genetic cause underlying the disease pathology in a pedigree (affected n = 9) with X-linked retinoschisis (XLRS1) due to a novel RS1 mutation and to assess suitability for novel therapies using multimodal imaging. METHODS: The Irish National Registry for Inherited Retinal Degenerations (Target 5000) is a program including clinical history and examination with multimodal retinal imaging, electrophysiology, visual field testing and genetic analysis. Nine affected patients were identified across 3 generations of an XLRS1 pedigree. DNA sequencing was performed for each patient, one carrier female and one unaffected relative. Pedigree mapping revealed a further 4 affected males. RESULTS: All affected patients had a history of reduced visual acuity and dyschromatopsia; however, the severity of phenotype varied widely between the nine affected subjects. The stage of disease was classified as previously described. Phenotypic severity was not linearly correlated with age. A novel RS1 (Xp22.2) mutation was detected (NM_000330: c.413C > A) resulting in a p.Thr138Asn substitution. Protein modelling demonstrated a change in higher order protein folding that is likely pathogenic. CONCLUSIONS: This family has a novel gene mutation in RS1 with clinical evidence of XLRS1. A proportion of the older generation has developed end-stage macular atrophy; however, the severity is variable. Confirmation of genotype in the affected grandsons of this pedigree in principle may enable them to avail of upcoming gene therapies, provided there is anatomical evidence (from multimodal imaging) of potentially reversible early stage disease.

Multimodal imaging of foveoschisis and macular pseudohole associated with gyrate atrophy: a family report.

BACKGROUND: To report the results of multimodal imaging of a biochemically confirmed case of a family with gyrate atrophy (GA) associated with foveoschisis and macular pseudohole. CASE PRESENTATION: Two sisters presented to us with progressive bilateral decreased vision. The 26-year old sister had a best corrected visual acuity (BCVA) of 20/32 in the right eye (RE) and 20/100 in the left eye (LE). Dilated fundus examination revealed multiple bilateral chorioretinal atrophy areas in the midperipheral and peripheral retina. Fluorescein angiography did not show any leak in the macular area. Swept-source optical coherence tomography (SS-OCT) showed increased central macular thickness in both eyes with foveoschisis. Optical coherence tomography angiography (OCTA) showed petaloid non-reflective areas and some perifoveal microvascular alterations similar to telangiectasias in the deep capillary complex. The 30-year-old sister had a BCVA of 20/20 in the RE and 20/32 in the LE. SS-OCT was normal in the RE and demonstrated a macular pseudohole with a fine epiretinal membrane in the LE. The persistent retinal tissue at the base of the pseudohole was disorganised. Blood tests showed hyperornithinemia in the 2 cases. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina and were treated with a pyridoxine supplement and an arginine-restricted diet. CONCLUSIONS: Foveoschisis and macular pseudohole may be associated in GA, increasing the risk of rapid vision loss. OCTA is an interesting imaging tool that can help to better understand the pathophysiological mechanism of these macular involvements in GA.

Development of macular retinoschisis long after the onset of retinal arterial occlusion (RAO): a retrospective study.

BACKGROUND: To describe a retrospective study of macular retinoschisis that developed long after the onset of retinal artery occlusion (RAO) using optical coherence tomography (OCT). METHODS: We describe changes in macular findings and visual acuity (VA) of 29 patients (21 males and 8 females, mean age: 66.1 ± 16.9 years) with RAO (18 branch RAOs [BRAOs] and 11 central RAOs [CRAOs] who visited Osaka Medical College Hospital over an 8-year period based on a medical chart review. RESULTS: The mean VA (logMAR) increased from 1.06 ± 1.08 (CRAO: 2.04 ± 0.99; BRAO: 0.37 ± 0.40) at the first visit to 0.71 ± 0.87 (CRAO: 1.46 ± 0.86; BRAO: 0.18 ± 0.30) at the final visit. Macular OCT revealed swelling or hyper-reflectivity of the inner retina in the early phase of RAO and retinal thinning in the late phase. Among the 29 patients, two patients (a patient with BRAO and a patient with CRAO) developed macular retinoschisis about 1 year after RAO onset. The VA of the patient with BRAO was 20/300 at the first visit, and it improved to 20/25 two days after onset following eye massage and anterior chamber paracentesis. However, his VA worsened, declining from 20/25 to 20/50, and retinoschisis occurred 13 months after RAO onset. The patient with CRAO showed macular changes including small cystoids at the first follow-up visit more than 3 weeks after onset and developed retinoschisis 11 months after the first visit. In addition, two patients with BRAO and one patient with CRAO developed macular changes including small cystoids 3 weeks after onset, with the BRAO complicated by retinal vein occlusion. In the CRAO patient, the cystoid macular edema was resolved 1 month after the first visit. CONCLUSIONS: Macular retinoschisis is unusual, but a possible complication of RAO that can develop long after the onset of the occlusion, potentially resulting in renewed VA deterioration.

Bilateral retinoschisis in a dog: A veterinary clinical application for optical coherence tomography.

A 11-year-old neutered male Labrador retriever-cross dog was presented to the University of Missouri-Columbia Veterinary Ophthalmology Service for subtle visual deficits. Indirect ophthalmoscopy revealed a smooth, bullous elevation in the superior-temporal retina OU. Optical coherence tomography (OCT) performed OU showed inner retinal separation consistent with retinoschisis. Electroretinography (ERG) revealed markedly reduced b-wave amplitudes in the more severely affected eye (OD) compared with the less severely affected eye (OS). The most notable reductions were in the rod response and 30-Hz flicker b-waves OD which were approximately 50% of the corresponding amplitudes OS. Implicit times, particularly the a-wave implicit times, were noticeably longer OD compared with OS. Lesions remained unchanged over 4 months at which time the dog was humanely euthanized for reasons unrelated to the ocular disease. Significant light microscopic ocular findings were bilateral superior temporal peripheral retinoschisis. The separation of the retinal tissue was similar between eyes and effectively divided the outer plexiform layer. In addition, thinning of the surrounding retinal layers was present. To the authors' knowledge, this is the first case of canine retinoschisis diagnosed with OCT, evaluated with electroretinography, and confirmed with light microscopic examination. History, clinical, and diagnostic findings, with the absence of disease progression over time, are analogous with cases of acquired senile retinoschisis in humans.

A Case of Acquired Peripheral Retinoschisis with Retinal Detachment in Young Adult.

Background: Acquired peripheral retinoschisis (RS) is usually found in middle age and older patients, and is occasionally accompanies by retinal detachment (RD). In this report, a case of acquired RS with RD in a young adult is documented. Case: A 27 years old man diagnosed with RD in the right eye at a periodical examination. Findings: RD was located at upper nasal part of fundus with the margin close to the macula. Dome shaped retinal protrusion was also seen at the periphery of nasal upper retina. Optical coherence tomography revealed no macular abnormality. Following a diagnosis of RD with atrophic retinal hole, scleral buckling and SF6 gas injection were performed, but the RD was not fully resolved. Additional B mode ultrasonography visualized peripheral RS at the dome shaped retinal protrusion. To avoid macular detachment, pars plana vitrectomy was performed. After executing a posterior vitreous detachment and subretinal fluid extraction, photocoagulation was performed around the area of the RS. Consequently, both the RD and RS were completely resolved. Conclusions: Acquired RS with RD may occur in young adults. This can be successfully resolved by surgery.

[Relation between ultrasonographic characteristics of pathologic myopia posterior staphyloma and retinoschisis].

Objective: To study the ultrasonographic characteristics of pathologic myopia posterior staphyloma and the relation with retinoschisis. Methods: Retrospective case series study. Eighty-seven eyes of 66 pathologic myopia patients with posterior staphyloma were included. Staphyloma morphology and anteroposterior axis of the eyeball were observed by B-scan ultrasonography. Optical coherence tomography was used to explore the retinoschisis. Results: Arc-shaped (10.35%), cone-shaped (22.99%), wedge-shaped (33.33%) and rectangle-shaped (33.33%) posterior staphylomas were found by B-scan ultrasonography. Posterior pole or macular retinoschisis was found by optical coherence tomography in 65 eyes (74.71%), 56 of which (86.15%) were observed to have a rough posterior ocular wall or membranoid attachment by ultrasonography. The anteroposterior axis of arc-shaped posterior staphyloma was shorter than that of staphylomas in the other shapes. Posterior staphyloma morphology was related to retinoschisis(r=0.385, 0.406. P<0.01). The retinoschisis was at the macula in 80.00% of cone-shaped posterior staphyloma, at the wedge-shaped corner in 75.00% of wedge-shaped posterior staphyloma and at the rectangle-shaped corner in 62.50% of rectangle-shaped posterior staphyloma. Conclusions: It is hard to discover retinoschisis of pathologic myopia posterior staphyloma by ophthalmoscopy. Morphologic characters of posterior staphyloma and conditions of posterior eyewall can be showed directly under ultrasonographic examination. B-scan ultrasonography may provide a diagnostic basis for pathologic myopia retinoschisis. (Chin J Ophthalmol, 2017, 53: 46-52).

Characterization of retinal structure and diagnosis of peripheral acquired retinoschisis using high-resolution ultrasound B-scan.

PURPOSE: The purpose of the study was to report the ability of high-resolution ultrasonography (USG) B-Scan in differentiating between acquired retinoschisis (RS) and retinal detachment (RD), and to compare the findings with spectral-domain optical coherence tomography (SD-OCT). METHODS: Patients with acquired peripheral RS and RD undergoing imaging with high-resolution B-scan USG and SD-OCT were included in the study. Descriptive analysis was performed on the images obtained by high-resolution B-scan USG to identify various retinal interfaces. The findings were correlated with those obtained using the SD-OCT images. RESULTS: Six eyes of five patients (two males) with RS and seven eyes of four patients (three males) with RD were included in the study. In all eyes of patients with RS, the outer retina demonstrated the presence of two hyper-reflective lines corresponding to the interfaces of the outer plexiform layer (OPL) and the retinal pigment epithelium (OPL). Eyes diagnosed with RD demonstrated two hyper-reflective lines in the detached portion, corresponding to the nerve fiber layer and OPL interfaces, whereas the attached portion demonstrated the presence of the third hyper-reflective interface, i.e., RPE. These findings correlated well with SD-OCT. CONCLUSIONS: Analysis of retinal interfaces on high-resolution USG B-scan may allow precise differentiation of acquired RS from RD by identification of various retinal layers. These findings correlate well with SD-OCT.