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The dark-adapted human electroretinogram (ERG) response to a standard bright flash includes a negative-going a-wave followed by a positive-going b-wave that crosses the baseline. An electronegative waveform (or negative ERG) results when the b-wave is selectively reduced such that the ERG fails to cross the baseline following the a-wave. In the context of a normally sized a-wave, it indicates a site of retinal dysfunction occurring after phototransduction (commonly at the photoreceptor to bipolar cell synapse). This is an important finding. In genetic disease, the pattern of ERG abnormality can point to variants in a small group of genes (frequently those associated with congenital stationary night blindness and X-linked retinoschisis, but negative ERGs can also be seen in other conditions including syndromic disease). In acquired disease, there are numerous causes, but specific features may point to melanoma-associated retinopathy (MAR). In some cases, the visual symptoms precede the diagnosis of the melanoma and so the ERG findings can initiate investigations facilitating early detection and treatment. Negative ERGs can occur in other paraneoplastic conditions, and in a range of other diseases. This review will outline the physiological basis for the negative ERG, report prevalences in the literature from different cohorts, discuss the range of causes, displaying examples of a number of ERG phenotypes, highlight features of a clinical approach to patients, and briefly discuss further insights relating to current flows shaping the a-wave trough and from single-cell transcriptome analysis.摘要: 负性视网膜电图: 遗传及获得性疾病的病因、诊断方法和生理解析摘要暗适应视网膜电图 (ERG) 对标准闪光反应包括一个负向a波和一个穿过基线的正向b波。当b波被选择性地降低, 以致ERG不能穿过a波之后的基线时, 就会产生负性波 (或负性ERG) 。在正常大小的a波背景下, 它意味着视网膜功能障碍发生在光转导之后 (通常在光感受器到双极细胞的突触部位) 。这是一个重要发现。在遗传性疾病中, ERG的负性异常改变可以指向一小群基因的变异 (通常与先天性静止性夜盲和X连锁视网膜劈裂有关, 但在其他综合征性的疾病中也可以看到负性ERG) 。在获得性疾病中, 有许多原因可以引起负性ERG, 但具体特征可能与黑色素瘤相关视网膜病变有关 (MAR) 。在一些病例中, 视觉症状的发现早于黑色素瘤的诊断, 因此ERG的发现可以尽快启动诊断和治疗。负性ERGs可在其他副肿瘤性疾病和其他一系列疾病中出现。本文将概述负性ERG的生理学基础、报告不同队列文献中的患病率、讨论病因的种类范围、展示一些典型ERG的表型病例以及突出临床治疗方法的特点, 并简要讨论了有关形成a波谷的电流和单细胞转录组分析的更多见解。.

To compare the visual and anatomical outcomes of two different treatment strategies (non-internal limiting membrane (ILM) peeling and fovea-sparing ILM peeling) for retinoschisis with foveal detachment (FD) in highly myopic eyes. A retrospective cohort study. Ninety-five eyes from 92 highly myopic patients with retinoschisis with FD were divided into two groups, including 44 eyes from 43 patients who received 23-gauge, 3-port vitrectomy without ILM peeling (group A) and 51 eyes from 49 patients who received vitrectomy with fovea-sparing ILM peeling (group B). All eyes also underwent cataract surgery. There were no significant differences between the two groups in terms of sex, age, diopters, axial length (AL), or central foveal thickness (CFT) before surgery (P > 0.05). One month after surgery, foveoschisis and FD were resolved in 74.47% of the eyes in group B and in only 12.50% of those in group A. Six months after surgery, foveoschisis and FD were resolved in 96.08% of the eyes in group B and in only 72.73% of those in group A (P

OBJECTIVES: The objective of this study is to evaluate the progression and new onset of macular retinoschisis (MRS) in the patients treated with intravitreal Conbercept injections for myopic choroidal neovascularization (mCNV). METHODS: Post-hoc analysis of 160 mCNV patients included in SHINY study was performed to evaluate the impact of Conbercept injection on MRS in patients with mCNV undergoing intravitreal Conbercept injections. The patients were 3:1 randomized to the study group (three loading dose and thereafter pro re nata [PRN]) and the control group (3 months’ sham injection, then one Conbercept injection at month 4 and thereafter PRN). MRS was assessed with optical coherence tomography by masked graders. RESULTS: At baseline, 28 of 122 eyes in study group and 10 of 38 eyes in control group had MRS. At month 3, two patients showed MRS progression and one patient had new onset MRS in study group. No MRS progression nor new onset MRS was found in the control group. At final visit, the cumulative incidence of MRS was 1.3% (2/160). Both Spearman’s correlation and multiple logistic regression demonstrated no association between the progression and new onset of MRS and intravitreal injection frequency (correlation coefficient = 0.017, P = 0.851 and odds ratio = 0.996, P = 0.982). In addition, baseline vitreoretinal adhesion was the most likely potential risk factor resulting in MRS progression (odds ratio = 4.566, P = 0.027). Furthermore, MRS progression was more likely to take place in outer retinal layers. CONCLUSIONS: The progression and new onset of MRS was not associated with the frequency of intravitreal Conbercept injections.

OBJECTIVE: To evaluate the morphologic and functional outcomes of different optical coherence tomography (OCT) patterns of myopic foveoschisis after vitrectomy with Inner Limiting Membrane (ILM) peeling. METHODS: In this prospective non-randomised study, 62 consecutive eyes with Myopic Foveoschisis were categorised into three groups according to OCT pattern: retinoschisis type (Rt) Group (23/62), foveal detachment type (FDt) Group (20/62) and macular hole type (MHt) Group (19/62). All patients underwent 25-Gauge vitrectomy and ILM peeling. Air or gas tamponade was used. All patients were observed at month 1, 2, 4 and 6 after surgery. Main outcomes measures: surgical success as resolution of myopic foveoschisis, central retinal thickness (CRT), IS/OS junction recovery and best-corrected visual acuity (BCVA) measurement. Any complication was reported. RESULTS: In all eyes OCT showed a resolution of the retinoschisis, foveal detachment and macular hole pattern, respectively. CRT significantly decreased in all Groups (p

BACKGROUND: To analyze the optical coherence tomography (OCT) characteristics and visual outcome of vitrectomy in myopic foveoschisis (MF) patients and identify prognostic factors. METHODS: This study is a retrospective clinical cohort study in tertiary care hospital. Thirty-two eyes of 32 patients who underwent MF-related vitrectomy in were investigated retrospectively. Best-corrected visual acuity (BCVA) measured at 1 year post surgery and changes in central foveal thickness (CFT) and co-existing macular pathologies, such as foveal detachment (FD), lamellar holes, and macular holes were the main outcome measures. Prognostic factors were identified using multivariate linear regression analysis. RESULTS: Average BCVA (in logarithm of the minimum angle of resolution) and mean CFT had improved from 0.46 ± 0.06 to 0.37 ± 0.07 (P = 0.089) and from 485.72 ± 164.69 to 341.71 ± 109.70 (P

Background/objectives Myopic traction maculopathy of the retinoschisis type is a unique entity that has been frequently overlooked in refraction correction in daily practice in myopic eyes. The objective of this study was to describe the imaging characteristics of myopic traction maculopathy (MTM) seen on optical coherence tomography angiography (OCTA) and to detect biomarkers of the associated functional changes. Subjects/methods We performed OCTA on eyes with MTM and retinoschisis (RS group; n = 27) and highly myopic eyes without MTM (control group; n = 27). The RS group was further divided into a dome-shaped macula (DSM) group and a non-DSM group. The groups were compared for morphologic and perfusion characteristics. Results The RS and control groups had comparable spherical equivalence (p = 0.65), but the RS group had worse best-corrected visual acuity (p

PURPOSE: To investigate the long-term results of a modified technique for parafoveal multiple curvilinear internal limiting membrane (ILM) peeling to preserve the epi-foveal ILM in myopic foveoschisis surgery. METHODS: Thirty-two consecutive patients (36 eyes) were retrospectively reviewed. Patients were divided into two groups according to the extent of ILM peeled: the fovea-sparing ILM peeling group (FS) (18 eyes) and total ILM peeling group (TP) (18 eyes). Patients were followed up for at least 12 months. The main outcome measures were best-corrected visual acuity changes, evolution of macular schisis and the factors associated with the development of a full-thickness macular hole (FTMH). RESULTS: FTMH developed in 1 of 18 eyes (5.6%) in the FS group and 3 of 18 eyes (16.7%) in the TP group (P = 0.28). Long-term follow-up showed visual improvement was better in the FS group than in the TP group (0.94 vs. 0.58 logMAR). Macular schisis disappeared in 13 of 18 eyes (72.2%) in the FS group, but disappeared in 7 of 18 eyes (38.9%) in the TP group (P = 0.04). Logistic regression analysis showed that only the preoperative outer lamellar macular hole (P = 0.016) was a significant risk factor for development of postoperative FTMH. CONCLUSIONS: Fovea-sparing ILM peeling achieves a higher rate of macular schisis resolution over total peeling. A preoperative outer lamellar macular hole can be a risk factor for the development of a macular hole.

PURPOSE: To study UK practice patterns for the management of retinal detachment secondary to macular hole (MHRD) and macular retinoschisis (MRS) in pathological myopia (PM). To review the anatomical and visual outcomes of the surgically managed cases. METHODS: A prospective observational case series for the management of MHRD was undertaken in association with the British Ophthalmological Surveillance Unit (BOSU). The results were combined with retrospective data, collected by the COllaboration of British RetinAl Surgeons (COBRA), on the management of both MHRD and MRS in PM in the UK. A total of 20 cases of MHRD and 53 cases of MRS (27 surgical cases and 26 cases managed conservatively) are reported in this combined study. RESULTS: MHRD: Mean baseline best corrected visual acuity (BCVA) was 1.60 logMAR. All cases underwent pars plana vitrectomy (PPV). Mean post-operative BCVA was 1.49 logMAR (p = 0.674). The macular hole was closed in 5/20 (25%) cases, open/flat in 10/20 (50%) cases and open/elevated in 4/20 cases (20%). MRS: Mean baseline BCVA was 0.87 logMAR in the surgical group and 0.45 logMAR in the conservatively managed group (p = 0.002). All eyes that had surgical intervention underwent PPV. Mean post-operative BCVA was 0.68 logMAR (p = 0.183). Anatomical outcomes demonstrated a persistent MRS in 2/27 (7.4%) cases, partial resolution in 7/27 (25.9%) cases and complete resolution in 16/27 (59.2%) cases. CONCLUSIONS: PPV is the only surgical procedure performed for the management of MHRD and MRS amongst the study participants. Success rates and visual outcomes are limited for MHRD and consistent with the current literature for MRS.

PURPOSE: To describe the principle characteristics of combined hamartoma of the retina and retinal pigment epithelium (‘combined harmatoma’) on MultiColor(TM) imaging and evaluate its role as a diagnostic and management tool. METHODS: Retrospective, observational case series comprising three patients with unilateral, juxtapapillary combined harmatoma. Complete ophthalmic examination was performed. MultiColor(TM), including green reflectance (GR) and near infra-red reflectance (NIRR), optical coherence tomography (OCT) and autofluorescence (AF) imaging were obtained (Heidelberg Spectralis). RESULTS: On MultiColor(TM), ‘red shifting’, indicated partial pigmentation. GR clearly defined epiretinal gliosis, retinal dragging and striations. On NIRR, all lesions were hypo-reflectant with hyper-reflectance at their edges. OCT showed full-thickness retinal thickening and disorganisation, intra-retinal fluid in two cases, sectoral RPE atrophy with photoreceptor loss in one case, RPE thickening and foveal pigment migration in one case; epiretinal membrane associated with ‘mini peaks’ of the inner retina in all cases and vitreous traction causing retinoschisis in one case. All lesions were hypo-autofluorescent. CONCLUSIONS: MultiColor(TM) enables combined harmatomas to be further characterised and may represent a valuable diagnostic and management tool: MultiColor(TM) and NIR define tumour boundaries and macular involvement, which may be useful for assessing visual impact; GR highlights inner retinal distortion, which may aid surgical management decisions.

To review the literature on epidemiology, clinical features, diagnostic imaging, natural history, management, therapeutic approaches, and prognosis of myopic foveoschisis. A systematic Pubmed search was conducted using search terms: myopia, myopic, staphyloma, foveoschisis, and myopic foveoschisis. The evidence base for each section was organised and reviewed. Where possible an authors' interpretation or conclusion is provided for each section. The term myopic foveoschisis was first coined in 1999. It is associated with posterior staphyloma in high myopia, and is often asymptomatic initially but progresses slowly, leading to loss of central vision from foveal detachment or macular hole formation. Optical coherence tomography is used to diagnose the splitting of the neural retina into a thicker inner layer and a thinner outer layer, but compound variants of the splits have been identified. Vitrectomy with an internal limiting membrane peel and gas tamponade is the preferred approach for eyes with vision decline. There has been a surge of new information on myopic foveoschisis. Advances in optical coherence tomography will continually improve our understanding of the pathogenesis of retinal splitting, and the mechanisms that lead to macular damage and visual loss. Currently, there is a good level of consensus that surgical intervention should be considered when there is progressive visual decline from myopic foveoschisis.

We report the diagnosis and treatment of patients with retinal detachment and/or retinoschisis associated with optic nerve coloboma or morning glory syndrome. A retrospective review of patients with optic nerve coloboma or morning glory syndrome with associated retinal detachment or retinoschisis was conducted. For five patients (six eyes), we report the clinical findings, spectral domain optical coherence tomography (OCT) imaging, intraoperative findings, and treatment outcomes. OCT scans demonstrate a bilaminar structure of maculopathy, consisting of inner schisis-like changes and outer layer retinal detachment. In most cases, a retinal break was demonstrated within the optic disc defect with three-dimensional OCT imaging. Glial tissue was sometimes observed within the anomalous defect. Vitrectomy and resection of the tractional tissue in these cases produced good anatomical and visual outcomes. Retinal detachment spontaneously resolved in cases where traction was not present. Traction may contribute to the pathogenesis of retinal detachment associated with colobomatous optic disc anomalies, either directly or by creating a secondary retinal break. OCT imaging assists with understanding the contributing factors to retinal detachment in individual cases of colobomatous optic disc anomalies and can thereby assist with determining the most effective approach to management.

To evaluate the morphological changes that occurred during the development of a full-thickness macular hole (FTMH) from macular retinoschisis (MRS) in highly myopic eyes by optical coherence tomography (OCT). The clinical characteristics and OCT images of five eyes of five patients with myopic MRS who developed FTMH during the follow-up period were evaluated. The natural evolution from MRS to FTMH was classified into two patterns by OCT findings. In FTMH formation pattern 1, a focal area of the external retinal layer was elevated and followed by the development of a small outer lamellar macular hole (OLMH) and retinal detachment (RD). The OLMH and RD were then enlarged horizontally and elevated vertically until the OLMH was attached to the overlying retinal layer. A FTMH finally developed when the roof of RD opened. In FTMH formation pattern 2, the opening of the roof of MRS or cystoid space caused an inner lamellar macular hole (ILMH). The MRS was then gradually resolved except the residual MRS beneath the ILMH, the ILMH would finally proceed into a FTMH as long as the remained external retinal layer beneath the ILMH continued splitting posteriorly until it reached retinal epithelium pigment. Our longitudinal study revealed two patterns of FTMH formation in highly myopic eyes with MRS. This finding might be useful in further understanding the pathogenesis of macular hole formation in high myopia.

To investigate the associated factors of foveoschisis and foveal detachment without macular hole in highly myopic eyes. A cross-sectional study of 124 eyes of 68 highly myopic patients was performed. The patients underwent complete ocular examination, fundus photography, and optical coherence tomography (OCT). Of the 124 eyes, 10 (8%, 10/124) had foveoschisis or foveal detachment without a macular hole on OCT examination. On the basis of univariate analysis, six variables were associated with the pathologic changes, including age over 40 years (P=0.018), spherical equivalent over 10 D (P=0.042), axial length over 31 mm (P=0.001), macular chorioretinal atrophy (P=0.00003), posterior staphyloma (P=0.0003), and vitreoretinal interface factors, including epiretinal membrane, posterior vitreoschisis, and vitreomacular traction (P=0.00002). In the multivariate analysis, three factors were independently associated with foveoschisis and foveal detachment without macular hole in high myopia: axial length, macular chorioretinal atrophy, and vitreoretinal interface factors. Foveoschisis and foveal detachment without macular hole are sight-threatening disorders in highly myopic eyes. Axial length, macular chorioretinal atrophy, and vitreoretinal interface factors were independently associated with these pathological conditions. Thus, both intraocular and outer ocular wall factors play important roles and merit further study.

Purpose To evaluate various published views regarding the management of complications of senile retinoschisis. Methods To analyze and compare various reports of studies of natural history and prognosis, and comparative risks of non-intervention vs prophylactic surgery. Results Although a number of recent techniques for treatment or prevention of complications of senile retinoschisis have been reported with generally satisfactory results, the numbers of cases have been small and no consensus can be said to have been achieved as to superiority of method. When compared to the extremely low likelihood of the natural occurrence of complications such as posterior progression to involve the macula, or of the development of progressive clinical retinal detachment, as well as the occurrence of risks of treatment, it appears preferable to postpone treatment until these complications actually make their appearance. Conclusions It is recommended that in cases of retinoschisis either without or with breaks, or retinoschisis with localized 'schisis-detachment', that prophylactic treatment be withheld except in a very few exceptional cases. In all cases of progressive, symptomatic rhegmatogenous retinal detachment however, prompt surgical treatment should be carried out.

Sir, We present the first case of bilateral myopic foveoschisis without subretinal fluid with spontaneous improvement in both eyes.

Foveal schisis with Mizuo phenomenon: Etio-pathogenesis of tapetal reflex in X-linked retinoschisis