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Journal of Glaucoma May 2024Peripapillary retinoschisis may bias optical coherence tomography's monitoring of glaucoma progression. Its impact on glaucoma still remains uncertain. Only two out of...
PRCIS
Peripapillary retinoschisis may bias optical coherence tomography's monitoring of glaucoma progression. Its impact on glaucoma still remains uncertain. Only two out of the ten included studies illustrated a correlation between peripapillary retinoschisis and glaucoma progression.
PURPOSE
The frequent use of optical coherence tomography increased the detection of peripapillary retinoschisis, which poses challenges in the follow-up of glaucoma patients. This systematic review aims to summarize the literature regarding peripapillary retinoschisis in glaucoma, exploring its prevalence, impact on disease, and clinical management implications.
METHODS
We searched PubMed, Embase, Web of Science and Scopus with tailored search queries for each platform. All studies had to report peripapillary retinoschisis in glaucoma patients. Exclusion criteria included studies with less than 10 eyes, studies focusing on schisis outside the disc area, with concomitant retinal or optic nerve lesions, with animals, reviews, studies written in non-English language and congress abstracts.
RESULTS
Ten studies were included, of which 7 were case-control, one was a cohort study and two were case series.Six studies showed that peripapillary retinoschisis often overlapped pre-existing retinal nerve fiber layer defects. One study reported that the de novo development of peripapillary retinoschisis was more frequent in eyes with glaucoma progression than in eyes without progression.Visual field findings were inconsistent, with just one study (out of six) showing that glaucoma patients with peripapillary retinoschisis experienced faster visual field deterioration than those without it. Overall, solely two studies (out of seven) associated peripapillary retinoschisis with faster glaucoma progression.
CONCLUSIONS
Peripapillary retinoschisis biases optical coherence tomography analysis in glaucoma. Caution is needed against overestimation of retinal nerve fiber layer thickness when peripapillary retinoschisis develops, and misinterpretation of its resolution as rapid progression. Peripapillary retinoschisis' exact impact on glaucoma progression remains unclear.
PubMed: 38771637
DOI: 10.1097/IJG.0000000000002437 -
Indian Journal of Ophthalmology Dec 2021To present a selected case series of advanced glaucoma-associated peripapillary and macular retinoschisis and response to various treatment strategies with a...
PURPOSE
To present a selected case series of advanced glaucoma-associated peripapillary and macular retinoschisis and response to various treatment strategies with a comprehensive literature review.
METHODS
Retrospective observational case series. Retrospective review of five selected cases of advanced glaucoma with peripapillary and macular retinoschisis.
RESULTS
All five patients had advanced glaucomatous damage with macular and peripapillary retinoschisis, three (patients 2, 3, and 5) had a neurosensory detachment of the macula. Increased intraocular pressure was managed with maximal antiglaucoma medications and G6 micropulse diode laser treatment in the first patient, transscleral diode laser in the second patient, mitomycin-C augmented trabeculectomy in the third patient, maximal antiglaucoma medications alone in the fourth patient, pars plana vitrectomy followed by trabeculectomy in the fifth patient.
CONCLUSION
We speculate that peripapillary and macular retinoschisis may indicate a vision-threatening sequelae of advanced glaucoma. The probable inciting factor for this vision-threatening pathology being elevated intraocular pressure, fluctuations in intraocular pressure, and chronic glaucoma with advanced cupping. We emphasize that meticulous examination of the macula in patients with advanced glaucoma is mandatory. It is imperative to do OCT macula in patients with advanced glaucoma to diagnose this distinct entity at an earlier stage and preserve the existing visual potential.
Topics: Antiglaucoma Agents; Glaucoma; Humans; Intraocular Pressure; Retinoschisis; Retrospective Studies; Tomography, Optical Coherence
PubMed: 34826994
DOI: 10.4103/ijo.IJO_668_21 -
Current Opinion in Ophthalmology May 2022This review aims to introduce stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) and its differential diagnosis. We summarize findings from case... (Review)
Review
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.
Topics: Diagnosis, Differential; Fluorescein Angiography; Humans; Macular Degeneration; Retinoschisis; Tomography, Optical Coherence; Vision Disorders
PubMed: 35239516
DOI: 10.1097/ICU.0000000000000844 -
Global Health & Medicine Jun 2020Pathologic myopia is a major cause of low vision and blindness worldwide. Its social and economic burden has been demonstrated by epidemiological studies. There have... (Review)
Review
Pathologic myopia is a major cause of low vision and blindness worldwide. Its social and economic burden has been demonstrated by epidemiological studies. There have been recent advances in the classification system for myopic maculopathy that enables clinicians to describe different types of lesions, including tessellated fundus, diffuse/patchy chorioretinal atrophy, macular atrophy, lacquer cracks, choroidal neovascularization (CNV), and Fuchs' spot, in a standardized format. From a therapeutic point of view, anti-vascular endothelial growth factor therapy has been established as first-line choice for myopic CNV. For myopic retinoschisis and macular holes with/without retinal detachment, pars plana vitrectomy has been generally accepted as an efficient strategy. Studies are being conducted to determine how to avoid the development of a postoperative macular hole and to improve the quality of vision after surgery. In recent years, studies have revealed preventive measures that can be taken against myopia progression, including low-dose atropine eyedrops and contact lens wearing with peripheral myopic defocusing.
PubMed: 33330799
DOI: 10.35772/ghm.2020.01007 -
Graefe's Archive For Clinical and... Jun 2023The purpose of this study is to investigate the clinical and morphological characteristics of epiretinal membrane (ERM)-Foveoschisis.
PURPOSE
The purpose of this study is to investigate the clinical and morphological characteristics of epiretinal membrane (ERM)-Foveoschisis.
METHODS
Medical charts of 2088 patients diagnosed with idiopathic ERM were screened and eyes with ERM-Foveoschisis were included. All eyes underwent a complete ophthalmological examination including spectral domain optical coherence tomography (SD-OCT). OCT features and best corrected visual acuity (BCVA) were analysed. ERM-Foveoschisis was defined as open, closed, elevated or flat based on the OCT features. Ellipsoidal zone (EZ) abnormality, intraretinal cystoid spaces, central foveal thickness (CFT), posterior vitreous detachment (PVD) and lens status were assessed.
RESULTS
One hundred-sixty-six patients (175 eyes) (72% female, mean age 70.46 years) were included. Incidence of ERM-Foveoschisis was 6.7%. Open type was seen in 86.8% and had a significantly better mean BCVA than closed type (p = 0.01). No statistically significant difference of mean BCVA was noted between the elevated and flat types. Mean BCVA was significantly lower in eyes with EZ abnormality (p = 0.03) and eyes with intraretinal cystoid spaces (p = 0.02). Patients with 'closed' ERM-Foveoschisis showed a significant higher median CFT than 'open' ERM-Foveoschisis (respectively, 364 µm and 176 µm, p < 0.001). A total of 81.9% eyes had PVD.
CONCLUSION
We differentiated four morphological types of ERM-Foveoschisis based on the OCT examination. Closed ERM-Foveoschisis presented with a higher CFT and lower BCVA than the open type. ERM-Foveoschisis with cystoid intraretinal spaces presented with a lower BCVA. The impact of the morphological types of the ERM-Foveoschisis on the clinical course and for therapy decision requires further long-term studies.
Topics: Humans; Female; Aged; Male; Epiretinal Membrane; Visual Acuity; Macula Lutea; Retinoschisis; Vitreous Detachment; Tomography, Optical Coherence; Retrospective Studies; Vitrectomy
PubMed: 36576570
DOI: 10.1007/s00417-022-05940-y -
Frontiers in Medicine 2021To investigate the relationship between myopic tractional maculopathy (MTM) and myopic atrophy maculopathy (MAM). Two hundred and six eyes with definitive myopic...
To investigate the relationship between myopic tractional maculopathy (MTM) and myopic atrophy maculopathy (MAM). Two hundred and six eyes with definitive myopic retinoschisis were assessed in the retrospective observational case series study and the atrophic and tractional features were further evaluated. Atrophic changes were analyzed according to the atrophic component in the ATN classification and the occurrence of gamma zones and delta zones. Tractional changes were evaluated based on different retinoschisis layers, the location and range of outer retinoschisis, retinal detachment, inner lamellar macular hole (ILMH), outer lamellar MH (OLMH), full-thickness MH (FTMH), and paravascular abnormalities. Of all the eyes, 29.6, 42.7, 19.4, and 8.3% presented MAM grades with A1, A2, A3, and A4, respectively. The three layers of retinoschisis and the entire macular retinoschisis had the highest incidences in A2 (38.6%; 54.5%). The numbers of retinoschisis layers and the grades of outer retinoschisis had a weak negative correlation with MAM ( = -0.138, = 0.048; = -0.139, = 0.047). All the eyes had gamma zones, and 82.52% of eyes also had delta zones. The incidence of retinal detachment and OLMH reached the peak in A2 and then decreased gradually. With MAM aggravation, the prevalence of ILMH decreased. Eyes with A1 and A2 were more likely to have OLMH, and those with A3 and A4 were more likely to have FTMH ( = 0.028; OR, 3.423; 95% CI, 1.144-10.236; = 0.004; OR, 7.752; 95% CI, 1.951-30.803). With the MAM grades growing, the types of paravascular abnormalities increased ( = 0.165, = 0.018). Diffuse chorioretinal atrophy was the dominant MAM grade in eyes with MTM. In the study, 72.3% of eyes with MTM presented with diffuse chorioretinal atrophy and a tessellated fundus. Over 80% of eyes with MTM had both gamma zones and delta zones. Diffuse chorioretinal atrophy might be a complicated stage for MTM with the highest rate of three layers of retinoschisis, the entire macular retinoschisis, RD, and OLMH. Atrophic progression might involve the development of MH. When MTM combines with well-defined atrophy, the occurrence of FTMH should be noted.
PubMed: 34490288
DOI: 10.3389/fmed.2021.679192 -
Investigative Ophthalmology & Visual... Aug 2022To evaluate spatial and temporal integration across the visual field in individuals with juvenile X-linked retinoschisis (XLRS).
PURPOSE
To evaluate spatial and temporal integration across the visual field in individuals with juvenile X-linked retinoschisis (XLRS).
METHODS
Nine subjects with XLRS and 10 visually normal individuals participated. Luminance thresholds were measured at 15 locations along the horizontal visual field meridian. Locations were grouped into four regions for analysis: foveal, parafoveal (2°), perifoveal (5°-10°), and peripheral (10°-60°). For spatial integration measurements, stimulus duration was 100 ms, and size ranged from 0.01 to 2.32 deg2 (Goldmann I-V). For temporal integration measurements, stimulus size was 0.15 deg2 (Goldmann III), and duration ranged from 12 to 800 ms. The effect of stimulus size and duration on the subjects' threshold was described using integration models.
RESULTS
Luminance thresholds for the XLRS group were more elevated for small targets (2.0×-12.6×) than for large targets (1.25×-3.2×) compared to controls for all locations. Likewise, thresholds for the XLRS group were more elevated for short durations (6.3×) than for long durations (4.0×) in the fovea and parafovea but were similarly elevated at all durations (2.0×-2.5×) in the perifovea and periphery. For both the size and duration experiments, thresholds measured in the fovea, parafovea, and perifovea of XLRS subjects were highly similar to those measured from the peripheral field of the controls.
CONCLUSIONS
Spatial and temporal integration characteristics of the XLRS fovea, parafovea, and perifovea are similar to those of the normal periphery. The results also indicate that scaling stimulus size equates thresholds for the XLRS and control subjects throughout the visual field, but scaling duration does not.
Topics: Fovea Centralis; Humans; Retinoschisis; Visual Fields
PubMed: 35984651
DOI: 10.1167/iovs.63.9.22 -
Ophthalmic Genetics Feb 2023X-linked juvenile retinoschisis (×LRS) is an X-linked vitreoretinal degenerative disease that consists of variable phenotypes ranging from severe early-onset defects to... (Review)
Review
BACKGROUND
X-linked juvenile retinoschisis (×LRS) is an X-linked vitreoretinal degenerative disease that consists of variable phenotypes ranging from severe early-onset defects to subtle abnormalities diagnosed in elderly patients. XLRS is caused by a loss of function of the protein Retinoschisin (RS1), which is essential to preserve retinal integrity and function of photoreceptor-bipolar synapse. The literature data so far mostly agree on the absence of a clear genotype-phenotype correlation in XLRS. We reviewed clinical and molecular characteristics of a cohort of Italian pediatric XLRS patients to assess the presence of a correlation between genotype and phenotype severity.
MATERIALS AND METHODS
We retrospectively examined clinical and genetic features of a cohort of 27 XLRS patients. In this study we included patients with a diagnosis of XLRS confirmed by fundus photography, spectral domain optical coherence tomography, and molecular analysis and with an onset of less than 10 years of age. We sorted RS1 variants according to their effect of RS1 structure and function in three separate groups.
RESULTS
According to previous studies, we did not observe a conclusive genotype-phenotype correlation in our cohort; nevertheless, we noticed that patients harboring RS1 variants leading to RS1-secreted mutants show a more homogeneous phenotype, with an overall good visual acuity, compared to the other two groups.
CONCLUSIONS
Our data support the hypothesis that secretion profile of RS1 could influence the severity of the phenotype. More extensive and functional studies are needed to acquire notions in view of the opportunity of gene replacement therapy for XLRS patients.
Topics: Humans; Retinoschisis; Retrospective Studies; Electroretinography; Mutation; Phenotype; Genotype; Eye Proteins; Tomography, Optical Coherence
PubMed: 36377647
DOI: 10.1080/13816810.2022.2141790 -
Graefe's Archive For Clinical and... Feb 2022This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis.
PURPOSE
This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis.
METHODS
This retrospective study included patients diagnosed with pars planitis between 1998 and 2019 and followed for at least 6 months. Demographics, best-corrected visual acuity (BCVA), anterior segment and fundus examination findings, intraocular pressure (IOP) values at baseline and final examination, treatments used during the follow-up, surgeries and complications were noted from medical records of the patients. The percentage of patients given adalimumab (ADA), the reasons for treatment switch and response to ADA were investigated.
RESULTS
One hundred fifteen eyes of 59 patients were included in the study. Forty-seven percent of patients were female. The median age of the patients was 10 (4-44) years. The median follow-up time was 33 (6-252) months. The median BCVA at admission was 0.20 (0.00-2.00) logMAR. The most common complications were cystoid macular oedema, cataract, epiretinal membrane and inferior peripheral retinoschisis. Prophylactic laser photocoagulation for peripheral retinoschisis was the most common surgical intervention, followed by cataract surgery and pars plana vitrectomy. Approximately 80% of patients received immunosuppressive and corticosteroid therapy for initial treatment. ADA was initiated in 23 patients (38.9%) due to refractory uveitis and adverse effects to the corticosteroid and helped control intraocular inflammation and decrease the use of systemic steroids/immunosuppressives in 22 of 23 (95%) of patients who received ADA. The median BCVA at final examination increased to 0.00 (0.00-2.00) logMAR.
CONCLUSIONS
Pars planitis is a chronic, progressive and insidious disease with several ocular complications and requires early and aggressive treatment. ADA appeared to be effective especially in patients' refractory to conventional treatment.
Topics: Adalimumab; Adolescent; Adult; Child; Child, Preschool; Female; Humans; Male; Pars Planitis; Retrospective Studies; Visual Acuity; Vitrectomy; Young Adult
PubMed: 34568953
DOI: 10.1007/s00417-021-05398-4 -
The British Journal of Ophthalmology Mar 2023X-linked retinoschisis (XLRS), associated with , is the most common type of X-linked retinopathy in children. This study aimed to identify clinical and genetic features...
BACKGROUND/AIMS
X-linked retinoschisis (XLRS), associated with , is the most common type of X-linked retinopathy in children. This study aimed to identify clinical and genetic features of retinoschisis in 120 families with variants in China.
METHODS
variants were collected from our in-house exome data and were predicted by multiple-step bioinformatics analysis. Clinical data of 122 patients from 120 families with potential pathogenic variants were analysed and summarised, respectively.
RESULT
Totally, 79 hemizygous variants (53 missense, 25 truncation and 1 indel), were detected. All except one (78/79, 98.7%), including 22 novels, were classified as potential pathogenic and detected exclusively in 120 families with retinoschisis. Clinical data demonstrated an average age of presentation at 5 years (1 month-41 years). Macular changes were classified as macular schisis (87.5%), macular atrophy (10.7%), normal (0.9%) and unclassified (0.9%). Patients with macular atrophy had older age but similar visual acuity compared with macular schisis. Peripheral retinal changes included flat retinoschisis (52.4%), bullous retinoschisis (BRS) (10.7%) and normal-like (36.9%) patients. Spontaneous regression was observed in two patients with BRS on follow-up examination. Visual acuity in the peripheral retinoschisis group was worse than that without peripheral retinoschisis.
CONCLUSION
Almost all rare variants were potential pathogenic. All patients with pathogenic variants showed detectable characteristics in the macula and/or peripheral retina. Our data on variants and associated clinical phenotypes may be of value for clinical diagnosis and genetic test of retinoschisis.
Topics: Humans; Retinoschisis; Mutation; Retina; Macula Lutea; Atrophy; Eye Proteins; Electroretinography
PubMed: 34645606
DOI: 10.1136/bjophthalmol-2021-319668