Targeted lipidomics uncovers oxylipin perturbations and potential circulation biomarkers in Bietti's crystalline dystrophy.

PURPOSE: Abnormalities in lipid metabolism have been proposed in Bietti's crystalline dystrophy (BCD). We aim to characterize the lipid profiles in a case-control study. METHODS: All participants were genetically confirmed by CYP4V2 gene sequencing and underwent chorioretinopathy evaluation by calculating the percentages of AF atrophy (PAFA). Fasting blood samples of BCD patients and controls were collected, and plasma was analyzed for routine lipid profiles. Targeted lipidomic evaluation includes long chain polyunsaturated fatty acids (LCPUFA) and associated eicosanoid metabolites. RESULTS: Routine lipids profiles showed elevated plasma levels of triglyceride (P = 0.043) and low-density lipoprotein cholesterol (P = 0.024) in BCD patients. Lipidomic analysis showed significantly decreased levels of ω-3 LCPUFA including docosahexaenoic acid (DHA, 22:6, P = 0.00068) and eicosapentaenoic acid (EPA, 20:5, P = 0.0016), as well as ω-6 LCPUFA arachidonic acid (ARA, 20:4, P < 0.0001) in BCD patients. Eicosanoid metabolites, either derived from ω-3 and/ or ω-6 LCPUFAs via cyclooxygenase (COX) or lipoxygenase (LOX) pathways, including 5-HEPE, 12-HEPE, 13-HDHA, 15-HETE, 12-HETE, 5-HETE, 6k-PGF1a, PGE2, PGJ2, and TXB2, exhibited significant differences (P < 0.0001) between BCD patients and controls. Genotypes of CYP4V2, specifically the biallelic null mutations, were observed to correlate with more remarkably reduced levels of oxylipins, involving major LOX pathway metabolites including 5-HETE, 5-HEPE, 12-HEPE and LTB4. CONCLUSIONS: BCD patients demonstrated significant decreases in plasma levels of ω-3 and ω-6 LCPUFA (DHA, EPA, and ARA), as well as their downstream metabolites via the COX and LOX pathways, suggesting that these might be implicated in BCD pathogenesis and could serve as biomarkers and therapeutic targets of the disease. KEY MESSAGES: What is known BCD is a vision-threatening hereditary disease the causative gene of which is CYP4V2. Abnormalities in lipid metabolism have been proposed and demonstrated previously in BCD studies. The detailed pathogenesis remains unclear and controversial. What is new We observed prominent lipidomic alterations in the circulation when compared with age, gender, and bodymass index (BMI)-matched healthy controls. BCD patients demonstrated significant decreases in plasma levels of ω-3 and ω-6 LCPUFA (DHA, EPA, and ARA). Remarkable changes were observed in the downstream metabolites of the LCPUFA via the COX and LOX pathways. Genotypes of CYP4V2, specifically the biallelic null mutations, were observed to correlate with more remarkably reduced levels of oxylipins, involving major LOX pathway metabolites.

The multimodal imaging features and outcomes of multifocal choroiditis/punctate inner choroidopathy lesion with multiple evanescent white dot syndrome-like features: a retrospective study.

BACKGROUND: Multiple evanescent white dot syndrome (MEWDS)-like features is a rare condition triggered by a macular disease or iatrogenic injury, exhibiting MEWDS changes in the fundus. This study aims to describe the multimodal imaging features and outcomes of multifocal choroiditis/punctate inner choroidopathy (MFC/PIC) lesions with MEWDS-like features. METHODS: Six cases were studied retrospectively. All cases were given regional and oral corticosteroids. RESULTS: All cases showed an isolated juxtafoveal yellowish-white MFC/PIC lesion with disruption of RPE-Bruch's membrane-choriocapillaris complex (RPE-BM-CC), subretinal hyperreflective materials and choroidal thickening on optical coherence tomography. Two weeks after presentation, the grayish-white dots disappeared spontaneously and the corticosteroids were given. After four weeks, the ellipsoid zone (EZ) around the lesion and hyper-autofluorescence resolved. After 13 weeks, five cases showed shrinkage of the juxtafoveal lesion and restoration of foveal EZ. After six months, the juxtafoveal lesion became pigmented. Only one case developed type 2 choroidal neovascularization. CONCLUSIONS: The clinical course of MEWDS-like manifestations is still evanescent in our cases. The yellowish-white juxtafoveal MFC/PIC lesions with disruption of RPE-BM-CC and choroidal thickening showed a well-controlled prognosis after corticosteroid treatment.

Utilizing support vector machines to foster sustainable development and innovation in the clean energy sector via green finance.

As the global demand for clean energy continues to grow, the sustainable development of clean energy projects has become an important topic of research. in order to optimize the performance and sustainability of clean energy projects, this work explores the environmental and economic benefits of the clean energy industry. through the use of Support Vector Machine (SVM) Multi-factor models and a bi-level multi-objective approach, this work conducts comprehensive assessment and optimization. with wind power base a as a case study, the work describes the material consumption of wind turbines, transportation energy consumption and carbon dioxide (CO2) emissions, and infrastructure material consumption through descriptive statistics. Moreover, this work analyzes the characteristics of different wind turbine models in depth. On one hand, the SVM multi-factor model is used to predict and assess the profitability of Wind Power Base A. On the other hand, a bi-level multi-objective approach is applied to optimize the number of units, internal rate of return within the project, and annual average equivalent utilization hours of the Wind Power Base A. The research results indicate that in March, the WilderHill New Energy Global Innovation Index (NEX) was 0.91053, while the predicted value of the SVM multi-factor model was 0.98596. The predicted value is slightly higher than the actual value, demonstrating the model's good grasp of future returns. The cumulative rate of return of Wind Power Base A is 18.83%, with an annualized return of 9.47%, exceeding the market performance by 1.68%. Under the optimization of the bi-level multi-objective approach, the number of units at Wind Power Base A decreases from the original 7004 to 5860, with total purchase and transportation costs remaining basically unchanged. The internal rate of return of the project increases from 8% to 9.3%, and the annual equivalent utilization hours increase to 2044 h, comprehensively improving the investment return and utilization efficiency of the wind power base. Through optimization, significant improvements are achieved in terAs the global demand for clean energy continues to grow, the sustainable development of clean energy projects has become an important topic of research. In order to optimize the performance and sustainability of clean energy projects, this work explores the environmental and economic benefits of the clean energy industry. Through the use of Support Vector Machine (SVM) multi-factor models and a bi-level multi-objective approach, this work conducts comprehensive assessment and optimization. With Wind Power Base A as a case study, the work describes the material consumption of wind turbines, transportation energy consumption and carbon dioxide (CO2) emissions, and infrastructure material consumption through descriptive statistics. Moreover, this work analyzes the characteristics of different wind turbine models in depth. On one hand, the SVM multi-factor model is used to predict and assess the profitability of Wind Power Base A. On the other hand, a bi-level multi-objective approach is applied to optimize the number of units, internal rate of return within the project, and annual average equivalent utilization hours of the Wind Power Base A. The research results indicate that in March, the WilderHill New Energy Global Innovation Index (NEX) was 0.91053, while the predicted value of the SVM multi-factor model was 0.98596. The predicted value is slightly higher than the actual value, demonstrating the model's good grasp of future returns. The cumulative rate of return of Wind Power Base A is 18.83%, with an annualized return of 9.47%, exceeding the market performance by 1.68%. Under the optimization of the bi-level multi-objective approach, the number of units at Wind Power Base A decreases from the original 7004 to 5860, with total purchase and transportation costs remaining basically unchanged. The internal rate of return of the project increases from 8% to 9.3%, and the annual equivalent utilization hours increase to 2044 h, comprehensively improving the investment return and utilization efficiency of the wind power base. Through optimization, significant improvements are achieved in terms of the number of units, internal rate of return within the project, and annual average equivalent utilization hours at Wind Power Base A. The number of units decreases to 5860, with total purchase and transportation costs remaining basically unchanged, the internal rate of return increases to 9.3%, and annual equivalent utilization hours increase to 2044 h. Energy consumption and CO2 emissions are significantly reduced, with energy consumption decreasing by 0.68 × 109 kgce and CO2 emissions decreasing by 1.29 × 109 kg. The optimization effects are mainly concentrated in the production and installation stages, with emission reductions achieved through the recycling and disposal of materials consumed in the early stages. In terms of investment benefits, environmental benefits are enhanced, with a 13.93% reduction in CO2 emissions. Moreover, there is improved energy efficiency, with the energy input-output ratio increasing from 7.73 to 9.31. This indicates that the Wind Power Base A project has significant environmental and energy efficiency advantages in the clean energy industry. This work innovatively provides a comprehensive assessment and optimization scheme for clean energy projects and predicts the profitability of Wind Power Base A using SVM multi-factor models. Besides, this work optimizes key parameters of the project using a bi-level multi-objective approach, thus comprehensively improving the investment return and utilization efficiency of the wind power base. This work provides innovative methods and strong data support for the development of the clean energy industry, which is of great significance for promoting sustainable development under the backdrop of green finance.

Management of autoimmune retinopathy treated with intravitreal dexamethasone implant.

PURPOSE: The purpose of this study is to evaluate clinical outcomes of autoimmune retinopathy (AIR) in the patients treated with intravitreal dexamethasone implant (IDI). METHOD: Twenty-one eyes of 11 AIR patients treated with at least 1 injection of IDI were retrospectively reviewed. Clinical outcomes before and after treatment, including best corrected visual acuity (BCVA), optic coherence tomography (OCT), fundus autofluorescence (FAF), full-field electroretinography (ff-ERG), and visual field (VF) at last visit within 6 and/or 12 months, were recorded. RESULTS: Among all the patients, 3 had cancer-associated retinopathy (CAR) and 8 had non-paraneoplastic-AIR (npAIR) with mean followed up of 8.52 ± 3.03 months (range 4-12 months). All patients achieved improved or stable BCVA within 6 and/or 12 months after the treatment. Cystoid macular edema (CME) in 2 eyes and significant retinal inflammation in 4 eyes were markedly resolved after single injection. Central retinal thickness (CFT) in all eyes without CME, ellipsoid zone (EZ) on OCT in 71.4% of eyes, ERG response in 55% of eyes, and VF in 50% of eyes were stable or improved within 6 months after treatment. At last visit within 12 months, both BCVA and CFT remained stable in the eyes treated with either single or repeated IDI; however, progression of EZ loss and damage of ERG response occurred in some patients with single IDI. CONCLUSION: Clinical outcomes, including BCVA and parameters of OCT, ERG, and VF, were stable or improved after IDI in a majority of AIR patients. Local treatment of AIR with IDI was a good option to initiate the management or an alternative for the patients' refractory to the systemic therapy but with limited side effect.

CHARACTERIZATION OF MACULAR NEOVASCULARIZATION IN BIETTI CRYSTALLINE DYSTROPHY USING MULTIMODAL IMAGING MODALITIES.

PURPOSE: To characterize the clinical features of macular neovascularization (MNV) secondary to Bietti crystalline dystrophy. METHODS: The imaging data of 157 eyes in 79 patients with Bietti crystalline dystrophy were retrospectively reviewed. 12 individuals (19 eyes) were found to have MNVs. Multimodal retinal imaging was used to evaluate the features of MNVs and the primary chorioretinopathy. RESULTS: The MNV lesions were shown as typical type 2 MNVs with subretinal hyperreflective material (SHRM), and usually detected along the borders of the retinal pigment epithelium/choriocapillaris dropout. The active MNVs were noted in earlier stages of Bietti crystalline dystrophy, while the activity was observed to be reduced in advanced cases. On spectral domain optical coherence tomography, the outer retinal structures were demonstrated to be partially preserved above the SHRMs compared with the extensive atrophy contiguously. Fibrotic scaring of the MNVs was commonly observed and arteriolarization was usually shown within the scars. CONCLUSION: MNV was demonstrated to be a common complication secondary to Bietti crystalline dystrophy. The lesions were typical type 2 MNV of varied activities possibly associated with the degrees of the primary degeneration. Choriocapillaris hypoperfusion may participate in MNV development.

Efficacy and safety of first-line combination therapy versus monotherapy for vitreoretinal lymphoma: a systematic review and meta-analysis.

BACKGROUND: Vitreoretinal lymphoma (VRL) is usually treated with a combination of intraocular methotrexate (ioMTX), high-dose intravenous methotrexate (HD-MTX), or local radiotherapy (RT) as the first options. The effectiveness and safety of monotherapy like bruton's tyrosine kinase inhibitors (BTKi) for PVRL remain uncertain. METHODS: A systematic review and meta-analysis of clinical trial data and conference abstracts in VRL patients treated with first-line combination therapy or monotherapy were conducted through a search of PubMed, Embase, and Scopus databases until December 2022. A total of 24 studies comprising 517 patients were included, and survival data were extracted from 279 patients due to inconsistent units across studies. RESULTS: The combined treatment group used ioMTX + chemotherapy (in 4 studies), RT + chemotherapy (in 2 studies), ioMTX/HD-MTX based regimen (in 2 studies), ioMTX + RT + chemotherapy (in 2 studies), ioMTX + lenalidomide/BTKi (in 2 studies) and combination of multiple therapies (in 7 studies). The monotherapy group was mainly treated with oral monotherapies such as BTKi. The combination therapy had a higher overall response rate (ORR) and complete response rate (CRR) than monotherapy (ORR: 96% vs. 72%, CRR: 92% vs. 63%). Combination therapy also resulted in a longer median progression-free survival (28.8 months vs. 13 months, p = 0.012). However, the combination therapy group had more severe side effects (grade 3/4 toxicity) than the monotherapy group (45% vs. 8%). CONCLUSION: The study showed combination therapy had better OR and CR rates, longer survival, and more toxicity than monotherapy. While BTK inhibitors were well-tolerated, long-term effectiveness needs confirmation from prospective studies. In addition, given the small number of studies of monotherapy for VRL, more studies are needed to validate its effects. TRIAL REGISTRATION: CRD42023400305.

Spectral-domain optical coherence tomography biomarkers in vitreoretinal lymphoma.

BACKGROUND: Although early detection is critical, diagnosing vitreoretinal lymphoma (VRL) remains difficult. We sought to assess the potential diagnostic value of spectral-domain optical coherence tomography (SD-OCT) in VRL. METHODS: We reviewed the clinical records and pre-treatment SD-OCT images of biopsy-confirmed VRL and uveitis patients, with primary involvement of the sub-retinal pigment epithelium (RPE) and the outer retina, including acute syphilitic posterior placoid chorioretinitis (ASPPC), chronic stage sympathetic ophthalmitis (SO), and idiopathic multifocal choroiditis (MFC). RESULTS: We included 45 eyes of 45 VRL patients and 40 eyes of 40 uveitis patients (17 ASPPC eyes, eight chronic SO eyes, and 15 MFC eyes). On SD-OCT, lymphoma cell infiltration was observed in various retinal layers, most commonly in the sub-RPE (80%) and sub-retinal space (62%). Highly sensitive features for VRL as compared to uveitis included vitreous cells (93%), focal hyper-reflective sub-retinal infiltration (51%), and diffuse RPE elevations (56%). The features strongly specific for VRL included preretinal deposits (92.5%), intra-retinal infiltration (except the incomplete vertical hyper-reflective type, 100%), banded hyper-reflective sub-retinal infiltration (90%), and confluent RPE detachments (100%). We identified an approach to VRL diagnosis based on these SD-OCT findings: (1) two highly sensitive features plus one strongly specific feature; or (2) one highly sensitive feature plus two strongly specific features, demonstrated a sensitivity of 80% and specificity of 95% for VRL. CONCLUSIONS: The SD-OCT may enable the detection of detailed lymphoma infiltration characteristics and provide significant supplemental value for VRL diagnosis, particularly when combining highly sensitive and specific VRL-associated SD-OCT features.

[Identification of a NONO gene variant in a child with congenital heart disease and global developmental delay].

OBJECTIVE: To explore the genetic basis for a child with congenital heart disease (CHD) and global developmental delay (GDD). METHODS: A child who was hospitalized at the Department of Cardiac Surgery of Fujian Children's Hospital on April 27, 2022 was selected as the study subject. Clinical data of the child was collected. Umbilical cord blood sample of the child and peripheral blood samples of his parents were collected and subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a 3-year-and-3-month-old boy, had manifested cardiac abnormalities and developmental delay. WES revealed that he had harbored a nonsense variant of c.457C>T (p.Arg153*) in the NONO gene. Sanger sequencing showed that neither of his parents has carried the same variant. The variant has been recorded by the OMIM, ClinVar and HGMD databases, but not in the normal population databases of 1000 Genomes, dbSNP and gnomAD. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), it was rated as a pathogenic variant. CONCLUSION: The c.457C>T (p.Arg153*) variant of the NONO gene probably underlay the CHD and GDD in this child. Above finding has expanded the phenotypic spectrum of the NONO gene and provided a reference for the clinical diagnosis and genetic counseling for this family.

Diffusion kurtosis imaging as an imaging biomarker for predicting prognosis in chronic kidney disease patients.

BACKGROUND: Renal fibrosis is the strongest prognostic predictor of end-stage renal disease (ESRD) in chronic kidney disease (CKD). Diffusion kurtosis imaging (DKI) is a promising method of magnetic resonance imaging successfully used to assess renal fibrosis in immunoglobulin A nephropathy. This study aimed to be the first to evaluate the long-term prognostic value of DKI in CKD patients. METHODS: Forty-two patients with CKD were prospectively enrolled, and underwent DKI on a clinical 3T MR scanner. We excluded patients with comorbidities that could affect the volume or the components of the kidney. DKI parameters, including mean Kurtosis (K), mean diffusivity and apparent diffusion coefficient (ADC) of kidney cortex were obtained by region-of-interest measurement. We followed up these patients for a median of 43 months and investigated the correlations between each DKI parameter and overall renal prognosis. RESULTS: Both K and ADC values were correlated well with the estimated glomerular filtration rate (eGFR) on recruitment and the eGFR of the last visit in follow-up (P ˂ 0.001). K and ADC values were also well associated with the eGFR slopes in CKD patients, both with the first-last time point slope (P = 0.011 and P ˂ 0.001, respectively) and with the regression slope (P = 0.010 and P ˂ 0.001, respectively). Cox proportional hazard regression indicated that lower eGFR and ADC values independently predicted eGFR loss of ˃30% and ESRD. The receiver operating characteristic analysis showed that K and ADC values were predictable for renal prognosis, and ADC displayed better capabilities for both ESRD [area under the curve (AUC) 0.936, sensitivity 92.31%, specificity 82.76%] and the composite endpoint (eGFR loss ˃30% or ESRD) (AUC 0.881, sensitivity 66.67%, specificity 96.3%). CONCLUSIONS: Renal ADC values obtained from DKI showed significant predictive value for the prognosis of CKD patients, which could be a promising noninvasive technique in follow-up.

BODIPY Photocatalyzed Beckmann Rearrangement and Hydrolysis of Oximes under Visible Light.

A novel, efficient, and mild protocol for rearrangement of oximes to amides or hydrolyzing to ketone/aldehyde using a simple BODIPY dye as a photocatalyst and air as an oxidant via propagation reaction under visible-light irradiation is reported. The triplet excited state of BODIPY played a significant role in the catalytic process. It was found that the various substituted ketoximes, both with electron-withdrawing and electron-donating substituents, afforded the corresponding products with moderate to excellent yields, and the catalytic efficiency was up to 0.01 mol %.

LRP5 BIALLELIC MUTATIONS CAUSE A HIGHER INCIDENCE OF SEVERE PHENOTYPE COMPARED WITH LRP5 MONOALLELIC MUTATION.

PURPOSE: To analyze the clinical features of LRP5 gene mutation-related familial exudative vitreoretinopathy and explore the potential phenotype-genotype correlation on LRP5 gene. METHODS: Eighty-seven familial exudative vitreoretinopathy (FEVR) families with LRP5 mutations were selected from 722 FEVR patients, which were divided into 2 groups, including 22 autosomal-recessive FEVR (ar-FEVR) families and 65 autosomal-dominant FEVR (ad-FEVR) families. Clinical and genetic data were retrospectively analyzed. The potential phenotype-genotype correlation was explored from the mutation type and inheritance pattern. RESULTS: No significant difference between the LRP5 null mutation subgroup and the LRP5 missense mutation subgroup was observed in the proportion of FEVR stage and the ratio of ocular involvement. Instead, a significant difference between the LRP5 ar-FEVR subgroup and the LRP5 ad-FEVR subgroup was observed in the proportion of FEVR stage and the ratio of binocularly severe phenotype. The probands with LRP5 gene recessive mutation showed a higher incidence of severe phenotype. Moreover, the ratio of binocularly severe patients in ar-FEVR was nearly 3.5 times higher than that in ad-FEVR. CONCLUSION: The severity of phenotype was more likely to be related to the synergistic effect of the variants.

CLINICAL CHARACTERISTICS OF STREAKY MULTIFOCAL CHOROIDITIS: A Subtype of Multifocal Choroiditis.

PURPOSE: To describe and analyze clinical characteristics of multifocal choroiditis with linear streaks (LSs). METHODS: Eight cases of multifocal choroiditis with LSs were retrospectively studied. Multimodal imaging was performed. Demographic data and spherical equivalent were collected. Axial length was measured. RESULTS: All cases are young myopic women with a mean age of 17.13 ± 3.64 years (range, 13-23 years), presenting with vision loss and distortion. Nine eyes with LSs were high myopia of -8.97 ± 2.69 D (range, -6.00 to 12.5 D; growing by 1.88 ± 0.61 D annually since wearing glasses), with mean axial length of 26.36 ± 1.71 mm. Vitreous cells were noted in seven eyes. LSs were located in the equator (eight eyes), around the optic disk (three eyes), and at the edge of the posterior pole (one eye). Angio-optical coherence tomography showed choroidal neovascularization in eight eyes, especially 2 to 3 choroidal neovascularizations in three eyes. The location of choroidal neovascularization were in subfovea (three eyes), parafovea (six eyes), and perifovea (two eyes). Swept source optical coherence tomography showed punched-out disruption of retinal pigment epithelium‒Bruch's membrane‒choriocapillaris complex at the LSs' sites. LSs showed fluorescence staining on late FA but hypofluorescence throughout all phases on ICGA. CONCLUSION: Multifocal choroiditis with LSs mostly occurs in young women with high myopia, especially occurring in eyes with rapid progression of myopia. LSs are mainly located in the midperiphery near the equator, being prone to concur with choroidal neovascularization. Based on our findings, we propose a new term called "streaky multifocal choroiditis" as a subtype of multifocal choroiditis.

Effects of intensive insulin therapy on the retinal microvasculature in patients with type 2 diabetes mellitus: a prospective observational study.

BACKGROUND: We examined the retinal microvascular changes and associated factors in type 2 diabetes mellitus (T2DM) before and after intensive insulin therapy. METHODS: This prospective observational study recruited patients with T2DM and divided them into intensive insulin therapy and oral hypoglycemic agent groups. All patients enrolled in this study had diabetes without retinopathy or non-proliferative diabetic retinopathy. Optical coherence tomography angiography (OCTA) was used in all patients before treatment and at 1, 3, and 6 months after treatment. Vessel density (VD) and thickness changes in the macular and optic disc areas were assessed. RESULTS: The study included 36 eyes in the intensive insulin therapy group and 36 in the oral hypoglycemic agent group. One month after treatment, VD in the deep capillary plexus (DCP) and peripapillary capillary VD (ppVD) were significantly decreased by intensification (P = 0.009, 0.000). At three months after treatment, decreases in VD induced by intensification were found in the superficial capillary plexus (SCP), DCP, foveal density in a 300-µm-wide region around the foveal avascular area (FD-300), and ppVD (P = 0.032, 0.000, 0.039, 0.000). Six months after treatment, decreases in VD by intensification were observed in the DCP and ppVD groups (P = 0.000, 0.000). Vessel density showed no significant change in the oral hypoglycemic agent group after treatment. The amount of DCP-VD reduction was correlated with macular thickening (r = 0.348, P = 0.038; r = 0.693, P = 0.000 and r = 0.417, P = 0.011, respectively) after intensive insulin therapy. CONCLUSIONS: Insulin-intensive treatment caused a transient reduction in vessel density in the macular and optic disc areas. DCP-VD and ppVD were more susceptible at an earlier stage. Retinal microvasculature monitoring using OCTA is vital for patients with type 2 diabetes receiving intensive insulin therapy.

Herpetic uveitis caused by herpes simplex virus after cataract surgery in a patient without prior viral keratitis or uveitis: a case report.

BACKGROUND: To report a case of herpetic uveitis caused by herpes simplex virus after cataract surgery in a patient without prior viral keratitis or uveitis. CASE PRESENTATION: A 70-year-old female was referred to our clinic with a 16-day history of acute blurry vision with painful redness in the right eye. She accepted cataract surgery for the right eye ten days before initial of ocular symptoms. There was significant inflammation in anterior chamber of the right eye. Retina exam showed moderate dense vitreous opacity but not necrotic or focal retinal lesion in the right eye. The aqueous humor collected from the right eye was positive for herpes simplex virus (HSV) DNA by PCR. The diagnosis of herpetic uveitis in the right eye was made due to clinical presentations and aqueous humor examination. CONCLUSION: Herpetic virus reactivation might occasionally occur after intraocular surgery in patients without prior ocular viral diseases, inducing atypical postoperative intraocular inflammation.

Re-vitrectomy for recurrent retinal detachment in post-vitrectomy eyes of rhegmatogenous retinal detachment.

BACKGROUND: Recurrent retinal detachment (Re-RD) usually affects the prognosis of surgery for rhegmatogenous retinal detachment (RRD). Previous clinical studies of Re-RD were not specific. This study aimed to analyze the clinical characteristics of Re-RD in post-vitrectomy eyes with RRD and surgical outcomes after revitrectomy without combining it with retinectomy or scleral buckling. METHODS: This is a retrospective case series analyzed the ocular characteristics of 20 recurrent and contralateral eyes, evaluated the significance of the associations between variables before reoperation and the final best-corrected visual acuity (BCVA), and calculated the outcome of revitrectomy. RESULTS: Patients with phakic eyes, those undergoing only one surgery, and those with more than one break had better final BCVA. The final BCVA was negatively correlated with the axial length and positively correlated with the preoperative BCVA. Among the 12 eyes with no break detected before surgery, 11 (92%) were found to have a small crevice-like break beside the pigment scar of a large number of original laser spots. The single-operation complete retinal reattachment rate was 75%, the complete retinal reattachment rate was 80%, and the final incomplete retinal reattachment rate was 90%. The BCVA improved from 1.2 ± 0.6LogMAR (0.06 ± 0.25) before surgery to 0.8 ± 0.7LogMAR (0.15 ± 0.2) at the last follow-up. The BCVA of 16 patients with complete retinal reattachment improved from 1.0 ± 0.5LogMAR (0.1 ± 0.3) to 0.6 ± 0.4LogMAR (0.25 ± 0.4). In the contralateral eyes, 15% already had vision-damaging disease, and the incidence of eyesight-threating lesions was 5.9% during follow-up. CONCLUSIONS: Revitrectomy without retinectomy or scleral buckling can effectively treat Re-RD in post-vitrectomy eyes. In Re-RD patients with no definite retinal break detected preoperatively, the retinal hole usually shows small crevice-like changes alongside a large number of original laser pigment scars.

Long-term clinical prognosis of 335 infant single-gene positive FEVR cases.

PURPOSE: To describe and analyze the clinical prognosis of infants diagnosed of familial exudative vitreoretinopathy (FEVR) with single gene mutation in long-term follow-up. METHODS: A retrospective case study was conducted on 355 FEVR infants with single positive gene. RESULT: Of the 335 single-gene positive infant FEVR cases (under 3 years old), 20% (n = 67) was diagnosed of strabismus at first visit. Staging of various genotypes was different (P < 0.001). Patients with NDP mutations presented the most severe clinical phenotypes and patients with ZNF408 mutations presented the mildest clinical phenotypes. Most infants underwent surgery under 1 year old (5th stage 75 of 108 [69.44%]). The axial length of different genotypes showed no significant difference (P = 0.2891). The 1st to 3rd stage cases were given intravitreal injection and/or retina photocoagulation with the last follow-up vision above 20/67. The 4th to 5th stage cases received the transcorneal vitrectomy with lensectomy or lens sparing vitrectomy (LSV), whose lens maintained transparent after LSV (11/14[78.58%]). After 2 to 10 years of follow-up, 37.96% (41/108) of post-surgery cases showed retinal funnel-like unfold and posterior pole unfold, 69.57% (16/ 23) of which received second surgery for closure of pupil with good prognosis. At the last follow-up, 20% (60/300) were with vision above 20/200. CONCLUSION: LRP5 gene mutation was the most common mutation in FEVR patients. The severity of the clinical phenotype varied with different gene mutations. The main surgical methods for cases at Stage 4-5 were transcorneal vitrectomy with lensectomy or LSV. The earlier FEVR occurred, the worse prognosis would be. Active surgical intervention and lens sparing were necessary for cases at Stage 4-5.

[The location diagnostic value of morphologic features of macular ganglion cell complex thickness in optic pathway-related neuro-ophthalmology].

The current study analyzed the morphologic features of macular ganglion cell complex (mGCC) thickness in optic pathway-related neuro-ophthalmic diseases by swept-source optical coherence tomography (SS-OCT). mGCC has the location diagnostic value similar to visual field, and the distribution of macular ganglion cells has accurate horizontal, vertical and quadrant characteristics. Specifically, prechiasmatic lesions showed atrophy and thinning of mGCC ring in single eye or mGCC atrophy in the upper and lower half of horizontal demarcation, chiasmatic lesions exhibited atrophy and thinning of mGCC in the half of bilateral nasal side demarcated by mid-vertical line, and retrochiasmatic lesions presented with atrophy of mGCC in the half of mid-vertical line demarcated or 1/4 quadrant. Therefore, the morphological characteristics of mGCC thickness are of value in the location diagnosis of optic path-related neuro-ophthalmic diseases.

Does ganciclovir exert retinal toxicity after multiple continuous intravitreal injections?

BACKGROUND: The objective of this study is to report a case of acute retinal necrosis in which abnormalities in visual function did not correspond to retinal anatomical outcomes. CASE PRESENTATION: A 39-year-old female diagnosed with acute retinal necrosis underwent repeated (nine rounds) intravitreal ganciclovir injection (3 mg/0.1 ml) into the left eye, one injection every 2 weeks. During the therapy, the patient noticed her visual acuity declining gradually. The best corrected visual acuity in the left eye was 20/33. The visual field showed massive visual damage. There was no posterior necrotizing involvement, no macular edema or exudation, and only slight abnormity of the interdigitation zone in the fovea area was visible on OCT. Angio-OCT revealed normal capillary density of three retinal capillary and choriocapillaris layers. The visually evoked potential was normal. The photopic single-flash response showed a declined amplitude of a-wave and b-wave. The amplitudes of photopic 30 Hz flicker were decreased. Multifocal electroretinography revealed macular dysfunction. CONCLUSION: Ganciclovir-associated photoreceptor damage may induce abnormalities in retinal function in response to multiple continuous intravitreal ganciclovir injections at a relatively high dosage (3 mg/0.1 ml).

Partial regression of foveoschisis following vitamin B6 supplementary therapy for gyrate atrophy in a Chinese girl.

BACKGROUND: To report a case of genetically confirmed gyrate atrophy (GA) of choroid and retina, who showed partial regression of foveoschisis following vitamin B6 supplementary therapy. CASE PRESENTATION: A 6-year-old Chinese girl complained about night blindness and progressive decreased vision in both eyes. Her best corrected visual acuity (BCVA) was 20/63 OD and 20/100 OS. Fundus examination showed bilateral multiple, sharply demarcated, scallop-shaped chorioretinal atrophy areas in the midperipheral and peripheral of the fundus. Spectral domain optical coherence tomography (SD-OCT) showed increased central macular thickness (CMT) with multiple intraretinal cystic spaces in the both eyes. There was no leakage or staining in the macular area in late phase of fluorescein angiography (FA). Blood tests confirmed hyperornithinemia and genetic analysis revealed two heterozygous mutations on ornithine aminotransferase (OAT) gene. Based on clinical presentation and genetic test, the patient was diagnosed as GA of the choroid and retina and further treated with vitamin B6 supplementary for three weeks. Her serum ornithine levels did not change but CMT on SD-OCT declined with partial regression of intraretinal cystic spaces. Then, the patient discontinued the drug because of severe muscle pain, and foveoschisis increased to initial level a month later. CONCLUSIONS: Foveoschisis is a rare complication of GA. Vitamin B6 supplementation may alleviate foveoschisis, but its effort for reducing serum ornithine level might be limited. Potential drug adverse effects should be noted in pediatric patients.

Outcome of scleral buckling with or without gas tamponade for recurrent retinal detachment in post-vitrectomy eyes.

BACKGROUND: Treatment of recurrent retinal detachment (re-RD) following vitrectomy (post-gas/air tamponade and post-silicone oil removal) is challenging. Previously reported treatment is commonly revision pars plana vitrectomy (PPV) combined with tamponade, which is invasive and a burden both economically and emotionally when compared with scleral buckling (SB). The purpose of this study is to report anatomical and functional outcomes of SB with or without gas tamponade in eyes with recurrent retinal detachment (re-RD) that previously underwent PPV at least once. METHODS: We retrospectively reviewed the medical records of 14 patients (14 eyes) who underwent PPV at least once and were treated with SB after re-RD. Preoperative characteristics, intraoperative complications, and postoperative data were assessed. The final anatomical and functional outcomes were analyzed. RESULTS: The original PPV was performed for primary rhegmatogenous retinal detachment in 11 eyes, macular hole retinal detachment in 2 eyes, and myopic foveoschisis in 1 eye. Previously, 3 eyes underwent one PPV with gas tamponade, and the remaining 11 (79%) eyes underwent 2-5 operations. Seven eyes underwent the procedure with gas injection. At the last follow-up, 13 eyes achieved total retinal attachment and 1 eye had re-RD. The postoperative intraocular pressure was within the normal range, except in 1 eye (6 mmHg). The finest postoperative best-corrected visual acuity (BCVA) was 20/25. There was a significant improvement in BCVA from 20/160 ± 20/63 at baseline to 20/80 ± 20/50 at the last visit in the 13 successfully treated eyes (P = 0.025). CONCLUSIONS: SB can be effective for re-RD after PPV in specific cases.