Long-term Outcomes of Surgeries for Retinal Detachment Secondary to Parasitic or Viral Infectious Retinitis.

Purpose: This study sought to compare the long-term outcomes of surgeries for retinal detachment (RD) secondary to viral or parasitic infectious retinitis. Methods: 47 eyes that received pars plana vitrectomy with or without scleral buckling due to RD secondary to polymerase chain reaction-proven viral (Cytomegalovirus, Varicella zoster virus, and Herpes zoster virus) or parasitic (toxoplasma and toxocara) retinitis from October 1st, 2006 to June 30th, 2023 in a single medical center were retrospectively enrolled. Results: Mean follow-up period was 59.03 ± 55.24 months in viral retinitis and 34.80 ± 33.78 months in parasitic retinitis after primary reattachment surgery. During follow-up, 9 eyes (24.3%) with viral retinitis and 5 eyes (50.0%) with parasitic retinitis developed retinal redetachment. Visual acuity success at final follow-up was achieved in 19 eyes (51.4%) with viral retinitis and 6 eyes (60.0%) with parasitic retinitis (p = 0.64). The incidence of retinal redetachment during the first postoperative year was significantly higher in parasitic retinitis compared with viral retinitis (crude incidence 0.21 vs 0.85 in viral and parasitic retinitis, respectively; p = 0.02). Hazard ratio analysis adjusted for age and sex showed 4.58-fold (95% confidence interval 1.22-17.27, p = 0.03) increased risk of retinal redetachment in parasitic retinitis compared with viral retinitis during the first postoperative year. Tamponade with silicone oil and preoperative diagnostic vitrectomy were associated with significantly decreased risk of retinal redetachment in patients with parasitic retinitis. Conclusions: Compared with RD secondary to viral retinitis, RD secondary to parasitic retinitis showed higher incidence of retinal redetachment during the first postoperative year. Tamponade with silicone oil and preoperative diagnostic vitrectomy were associated with significantly decreased risk of retinal redetachment in patients with parasitic retinitis.

Next-Generation Sequencing of Vitreoretinal Lymphoma by Vitreous Liquid Biopsy: Diagnostic Potential and Genotype/Phenotype Correlation.

Purpose: To determine the diagnostic potential of next-generation sequencing (NGS) in vitreous samples, analyze genotype-phenotype characteristics, and compare NGS of matched vitreous and brain samples in patients with associated central nervous system lymphoma (CNSL). Methods: A total of 32 patients suspected of vitreoretinal lymphoma (VRL) who underwent diagnostic vitrectomy and NGS were included in this retrospective observational case-series. Fresh vitreous specimens from diagnostic vitrectomy of VRL-suspected patients underwent NGS using a custom panel targeting 747 candidate genes for lymphoma. They also underwent malignancy cytology, interleukin (IL)-10/IL-6, immunoglobulin heavy chain (IGH)/immunoglobulin kappa light chain (IGK) monoclonality testing. MYD88 L265P mutation was examined from anterior chamber tap samples. The diagnosis of VRL was made based on typical clinical characteristics for VRL, as well as malignant cytology, IGH/IGK clonality, or IL-10/IL-6 > 1. Sensitivity and specificity of NGS were compared with conventional diagnostic tests. Brain tissues suspected of lymphoma were collected by stereotactic biopsy and underwent NGS. Genetic variations detected in NGS of vitreous and brain tissue specimens were compared. Results: The sensitivity values for cytology, IL-10/IL-6 > 1, clonality assays for IGH and IGK, MYD88 L265P detection in anterior chamber tap samples, and vitreous NGS were 0.23, 0.83, 0.68, 0.79, 0.67, and 0.85, with specificity values of 1.00, 0.83, 0.50, 0.25, 0.83, and 0.83, respectively. The sensitivity (0.85) of vitreous NGS was the highest compared to other conventional diagnostic tests for VRL. The most common mutations were MYD88 (91%), CDKN2A (36%), PIM1 (32%), IGLL5 (27%), and ETV6 (23%). Although several gene alterations demonstrated heterogeneity between the brain and eyes, some common mutational profiles were observed in matched vitreous and brain samples. Conclusions: Overall, NGS of the vitreous demonstrated high sensitivity among conventional diagnostic tests. VRL and CNSL appeared to have both shared and distinct genetic variations, which may suggest site-specific variations from a common origin.

Subset of retinoblastoma tumours is associated with BRCA1/2 mutations.

BACKGROUND: We investigated the potential association between pathogenic BRCA1/2 variants and retinoblastoma pathogenicity. METHODS: In this single-centre, retrospective case series, we performed hereditary cancer panel tests using blood samples for patients with retinoblastoma diagnosed between March 2017 and October 2021. Bioinformatics prediction tools were then used to conduct in silico pathogenicity assessments for patients with BRCA1/2 family variants, in addition to the American College of Medical Genetics and Genomics (ACMG) variant classification. One patient with a germline BRCA1 variant was analysed with whole-genome sequencing (WGS), mutational signature analysis and methylation analysis for RB1 and BRCA using the patient's tumour and blood samples. RESULTS: Of 30 retinoblastoma patients who underwent panel sequencing, six (20%) were found to carry germline variants in the BRCA1/2 or BRIP1 genes. Among these six patients, two had pathogenic or likely pathogenic variants as per the ACMG variant classification. Additionally, three patients showed potential pathogenic BRCA1/2 family variants through further analysis with alternative bioinformatics prediction tools. In the WGS analysis of a tumour from a patient with a germline likely pathogenic BRCA1 variant in one allele, we observed the loss of one RB1 allele due to a large deletion. No somatic non-synonymous mutations or frameshift indels were detected in the RB1 locus of the remaining allele. This sample also showed BRCA1 gene promoter hypermethylation in the tumour, indicating additional epigenetic silencing. CONCLUSION: This study demonstrated that some retinoblastoma patients harboured germline BRCA1/2 family variants, which may be associated with the development of retinoblastoma along with RB1 mutations.

Genotypic Profile and Clinical Characteristics of CRX-Associated Retinopathy in Koreans.

This study aimed to investigate the clinical characteristics of Korean patients with retinal dystrophy associated with pathogenic variants of cone rod homeobox-containing gene (CRX). We retrospectively enrolled Korean patients with CRX-associated retinal dystrophy (CRX-RD) who visited two tertiary referral hospitals. Pathogenic variants were identified using targeted panel sequencing or whole-exome sequencing. We analyzed clinical features and phenotypic spectra according to genotype. Eleven patients with CRX-RD were included in this study. Six patients with cone-rod dystrophy (CORD), two with macular dystrophy (MD), two with Leber congenital amaurosis (LCA), and one with retinitis pigmentosa (RP) were included. One patient (9.1%) had autosomal recessive inheritance, and the other ten patients (90.9%) had autosomal dominant inheritance. Six patients (54.5%) were male, and the mean age of symptom onset was 27.0 ± 17.9 years. At the first presentation, the mean age was 39.4 ± 20.6 years, and best-corrected visual acuity (BCVA) (logMAR) was 0.76 ± 0.90 in the better eye. Negative electroretinography (ERG) was observed in seven (63.6%) patients. Nine pathogenic variants were identified, including two novel variants, c.101-1G>A and c.898T>C:p.(*300Glnext*118). Taken together with the variants reported in prior studies, all variants within the homeodomain are missense variants, whereas most variants downstream of the homeodomain are truncating variants (88%). The clinical features of pathogenic variants within the homeodomain are either CORD or MD with bull's eye maculopathy, whereas variants downstream of the homeodomain cause more diverse phenotypes, with CORD and MD in 36%, LCA in 40%, and RP in 24%. This is the first case series in Korea to investigate the CRX-RD genotype-phenotype correlation. Pathogenic variants downstream of the homeodomain of the CRX gene are present as RP, LCA, and CORD, whereas pathogenic variants within the homeodomain are mainly present as CORD or MD with bull's eye maculopathy. This trend was similar to previous genotype-phenotype analyses of CRX-RD. Further molecular biologic research on this correlation is required.

CRISPR/Cas9 mediated specific ablation of vegfa in retinal pigment epithelium efficiently regresses choroidal neovascularization.

The CRISPR/Cas9 system easily edits target genes in various organisms and is used to treat human diseases. In most therapeutic CRISPR studies, ubiquitously expressed promoters, such as CMV, CAG, and EF1α, are used; however, gene editing is sometimes necessary only in specific cell types relevant to the disease. Therefore, we aimed to develop a retinal pigment epithelium (RPE)-specific CRISPR/Cas9 system. We developed a CRISPR/Cas9 system that operates only in retinal pigment epithelium (RPE) by expressing Cas9 under the RPE-specific vitelliform macular dystrophy 2 promoter (pVMD2). This RPE-specific CRISPR/pVMD2-Cas9 system was tested in human retinal organoid and mouse model. We confirmed that this system works specifically in the RPE of human retinal organoids and mouse retina. In addition, the RPE-specific Vegfa ablation using the novel CRISPR-pVMD2-Cas9 system caused regression of choroidal neovascularization (CNV) without unwanted knock-out in the neural retina in laser-induced CNV mice, which is a widely used animal model of neovascular age-related macular degeneration. RPE-specific Vegfa knock-out (KO) and ubiquitous Vegfa KO were comparable in the efficient regression of CNV. The promoter substituted, cell type-specific CRISPR/Cas9 systems can be used in specific 'target cell' therapy, which edits genes while reducing unwanted off- 'target cell' effects.

Association between polycystic ovary syndrome and non-infectious uveitis.

Polycystic ovary syndrome (PCOS) is a common endocrine disease in young women. It has been reported that increased proinflammatory cytokines can induce systemic inflammation. However, the association between PCOS and uveitis remains elusive. In this study, we investigate the possible association between PCOS and uveitis using Korean National Health Insurance Service-National Sample Cohort. The incidence of non-infectious uveitis was compared between patients with and without PCOS before and after propensity score matching. Hazard ratios were determined using univariate and multivariate Cox regression models. Of 558,302 female participants, 2039 had PCOS and 8122 had non-infectious uveitis. The incidence of non-infectious uveitis was 35.1 per 10,000 person-years in the PCOS patients compared to 16.6 in non-patients (P < .001). This tendency remained after 1:3 propensity score matching. The hazard ratio of PCOS using a multivariate Cox regression model was 2.79 (95% CI, 1.92-4.05; P < .001) and 2.87 (95% CI, 1.77-4.67; P < .001) before and after matching, respectively. Our results suggests that PCOS is associated with non-infectious uveitis, particularly in women of reproductive age. This may be due to hormonal changes and proinflammatory factors. Future investigations should examine the clinical features and underlying mechanisms.

Refractive Errors, Retinal Findings, and Genotype of Tuberous Sclerosis Complex: A Retrospective Cohort Study.

PURPOSE: To examine the refractive errors, retinal manifestations, and genotype in tuberous sclerosis complex (TSC) patients in a Korean population. MATERIALS AND METHODS: A total of 98 patients with TSC were enrolled in Severance Hospital for a retrospective cohort study. The number of retinal astrocytic hamartoma and retinal achromic patch within a patient, as well as the size, bilaterality, and morphological type were studied. In addition, the refractive status of patients and the comorbidity of intellectual disability and epilepsy were also examined. RESULTS: Retinal astrocytic hamartoma was found in 37 patients, and bilateral invasion was observed in 20 patients (54%). TSC1 mutation was associated with myopia (p=0.01), while TSC2 mutation was associated with emmetropia (p=0.01). Retinal astrocytic hamartoma was categorized into three morphological types and examined as follows: type I (87%), type II (35%), and type III (14%). Single invasion of retinal astrocytic hamartoma was identified in 32% of the patients, and multiple invasions in 68%. The TSC1/TSC2 detection rate was 91% (41/45). Among them, TSC1 variant was detected in 23 patients (54%), whereas TSC2 variant was detected in 18 patients (40%). The results showed that TSC2 mutations are correlated with a higher rate of retinal astrocytic hamartoma involvement (all p<0.05), and multiple and bilateral involvement of retinal hamartomas (all p<0.05). However, the size of retinal astrocytic hamartomas, comorbidity of epilepsy, or intellectual disability did not show correlation with the genetic variant. CONCLUSION: TSC1 variant patients were more myopic, while TSC2 variant patients showed association with more extensive involvement of retinal astrocytic hamartoma.

BILATERAL MACULAR CHOROIDAL ABNORMALITIES WITH DRUSENOID DEPOSITS IN PATIENTS WITH UNILATERAL PERIPHERAL EXUDATIVE HEMORRHAGIC CHORIORETINOPATHY.

PURPOSE: To investigate bilateral macular features on optical coherence tomography in patients with unilateral peripheral exudative hemorrhagic chorioretinopathy (PEHCR). METHODS: In this cross-sectional study, optical coherence tomography features of affected eyes (PEHCR group, n = 30) and unaffected contralateral eyes (contralateral group, n = 30) were investigated. Age-matched and sex-matched patients with polypoidal choroidal vasculopathy (PCV group, n = 51) and healthy controls (normal group, n = 50) were included to compare choroidal thickness, measured at six points apart from the fovea, with the PEHCR group. RESULTS: Subretinal drusenoid deposits were the most common feature in the PEHCR (20%) and contralateral (23%) groups, followed by soft drusen. Although the macular choroid was comparably thin in both the PEHCR and contralateral groups, pachyvessels were also observed. The choroids of the PEHCR group were significantly thinner than those of the normal group at the subfovea and 1-mm temporal to the fovea and considerably thinner than those of the polypoidal choroidal vasculopathy group from 3-mm nasal to 3-mm temporal to the fovea. CONCLUSION: In patients with unilateral PEHCR, bilateral choroidal thinning and drusenoid deposit accumulation were noted in the macula. The pathophysiology of PEHCR may be a rare peripheral complication of age-related macular degeneration with pathologic choroid.

Predicting Visual Outcomes in Choroidal Osteoma Treated with Anti-vascular Endothelial Growth Factor.

PURPOSE: To determine potential factors associated with the long-term visual prognoses in patients with choroidal osteoma undergoing anti-vascular endothelial growth factor (VEGF) treatment. DESIGN: Retrospective case series. METHODS: Patients diagnosed at tertiary high-volume hospitals between January 2000 and December 2020 were evaluated. The primary outcome measure was visual acuity at 5-year follow-up. The secondary outcome measures included factors associated with favorable vision, defined as loss of <1 line and >20/200 vision. RESULTS: Of 38 eyes from 36 patients (22 female, 61%; mean age 38 years) with choroidal osteoma, 23 eyes (61%) received anti-VEGF treatment (bevacizumab 1.25mg/0.05 cc, monthly or treat-and-extend) and 65% completed the 5 years of follow-up. All treated eyes had associated chorioretinal comorbidities (subretinal fluid 91%; choroidal neovascularization 74%; subretinal hemorrhage 30%). Although there was significant vision loss by 5 years (P = .002), 12 eyes (44%) had favorable outcomes. Only tumor thickness was significantly associated with unfavorable visual outcomes (OR 917.1, 95% CI 1.0-5687.7; P = .049). The optimal cut-off point predictive of visual outcomes was 1.4 mm, and tumor thickness ≥ 1.4 mm was associated with unfavorable vision (OR 27.0, 95% CI 2.0-368.4; P = .013). CONCLUSIONS: Among patients with choroidal osteoma undergoing anti-VEGF therapy, a particular patient subset presented with divergent outcomes with very poor vision. Tumor thickness appeared to be the differentiating factor and is thus a potential prognostic indicator for long-term visual prognoses.

Short-Term Outcomes of the First in Vivo Gene Therapy for RPE65-Mediated Retinitis Pigmentosa.

Here, we report early treatment outcomes of gene therapy for early onset retinitis pigmentosa (RP) (Leber congenital amaurosis) associated with biallelic RPE65 mutation in a 30-year-old female patient. Initially, her visual acuity (VA) was 20/200, and her visual field (VF) was severely constricted to the center in the left eye. Her electroretinography showed nearly extinct signals. Full-field stimulus threshold test (FST) revealed diminished dark-adapted light sensitivity. Voretigene neparvovec-rzyl (VN) is the first in vivo viral gene therapy agent to be approved. At 3 months after subretinal injection of VN in the left eye, VA, VF, and FST showed sustained improvement. She did not exhibit any signs of adverse effects from the treatment. Gene therapy for RP proved to be an effective and safe treatment in an advanced case of RPE65-associatied early onset RP.

Clinical Characteristics of recurrent intraocular lens dislocation after scleral-fixated sutured intraocular lens and long-term outcomes of intraocular lens re-fixation.

PURPOSE: This study aimed to investigate the clinical characteristics of patients with recurrent intraocular lens (IOL) dislocation after scleral-fixated sutured IOL implantation and evaluate the long-term outcomes of scleral re-fixation of IOL. METHODS: The medical records of patients who underwent surgery for IOL dislocation between January 2011 and January 2021 were reviewed. The study included 164 patients (male: 131, female: 33) (176 eyes). Patient demographics, preoperative, intraoperative and postoperative data, and the ocular and systemic conditions associated with IOL re-dislocation were analyzed. RESULTS: The study included 176 consecutive cases of scleral-fixated sutured IOL. Twenty-six eyes (14.8%) showed re-dislocation of IOL after the initial IOL scleral fixation and underwent reoperation (mean 75.5 ± 62.5 months after the first surgery); three (11.5%) of them required a third surgery. Younger adults (aged less than 40 years), and patients who underwent IOL scleral fixation in complicated cataract surgery or aphakic state had a higher risk of re-dislocation. Diabetes mellitus (DM) was the only statistically significantly higher risk factor in the re-dislocated group (p = 0.041). The complication rate with scleral re-fixation was higher than that in the non-re-dislocated group. No statistically significant differences were observed, except for vitreous hemorrhage (p = 0.024). CONCLUSIONS: Caution should be exercised when performing sutured scleral fixation of IOL in younger patients, cases of complicated cataract surgery and aphakia, and patients with DM to prevent IOL re-dislocation. Scleral-fixated sutured IOL in eyes with recurrent IOL dislocation seems to be a safe and effective procedure with a relatively low complication rate.

Association of Keratin 8 Level in Aqueous Humor With Outcomes of Intravitreal Ranibizumab Treatment for Neovascular Age-Related Macular Degeneration.

Purpose: To investigate keratin 8 (KRT8) level in the aqueous humor (AH) of patients with neovascular age-related macular degeneration (nAMD) and elucidate its association with intravitreal ranibizumab (IVR) treatment outcomes. Methods: This prospective study involved 58 eyes of treatment-naïve nAMD patients treated with three IVR doses monthly and whose AH samples were collected at baseline and two months after the initial treatment. KRT8 level was determined using the enzyme-linked immunosorbent assay and compared with that of the control group, which comprised patients who underwent cataract surgery during the same period. The nAMD-affected eyes were classified into responder (dry) and poor responder (persistent fluid) groups, according to optical coherence tomography (OCT) findings at month three. Additionally, associations between the KRT8 level and IVR treatment outcomes were analyzed. Results: The baseline KRT8 level was significantly higher in the AMD group than in the control group. In the AMD group, responders demonstrated significant differences between the KRT8 level at the baseline and month two, whereas poor responders exhibited no significant change. Regression analysis revealed that a higher KRT8 level at month two was significantly associated with persistent fluid upon OCT at months three and six. Conclusions: Monitoring aqueous KRT8 level may facilitate early determination of the therapeutic effects of IVR in nAMD patients and reflect the conditions of retinal pigment epithelium during the disease course. Translational Relevance: Monitoring aqueous KRT8 may aid early determination of therapeutic effects of IVR in neovascular AMD patients and reflect the health conditions of retinal pigment epithelium.

Complications, treatments, and visual prognosis of choroidal osteomas.

PURPOSE: This study aims to report complications, treatments, and visual prognosis of choroidal osteoma. METHODS: We retrospectively reviewed electronic medical records and multimodal images of 41 patients with choroidal osteoma. RESULTS: Visually significant complications included choroidal neovascularization (CNV) in 21 (47.7%) eyes and subretinal fluid (SRF) without CNV in 14 (31.8%) eyes. The most common treatment was intravitreal anti-vascular endothelial growth factor (VEGF) injection: 13 (61.9%) eyes with CNV received an average of 6.3 injections, and 6 (42.9%) eyes with SRF but without CNV received 1.8 injections. As the first-line treatment, intravitreal anti-VEGF injection induced complete or partial remission in 93.4% of eyes with CNV and 57.1% of eyes with SRF. The probability of legally low vision estimated at 3 and at 5 years was 29.1% and 34.2%, respectively. The presence of CNV and outer retinal tubulation (ORT) was independent risk factors for vision loss (adjusted odds ratio, 8.08 and 6.94, respectively). CONCLUSIONS: The development of CNV and ORT was strong risk factors for visual impairment. Due to the frequent recurrence of complications and poor visual prognosis, regular check-ups and appropriate treatment choices are warranted.

Comparison of long-term surgical outcomes for scleral-fixated versus retropupillary iris-claw intraocular lens.

BACKGROUND: To compare the clinical features and long-term treatment outcomes of patients undergoing secondary intraocular lens (IOL) surgery with scleral-fixated (SFIOL) versus retropupillary iris-claw IOL (RPICIOL). METHODS: Consecutive patients treated between June 2014 and December 2019 at two tertiary centres were retrospectively reviewed. The primary outcome was the best-corrected visual acuity (BCVA). Secondary outcomes included factors associated with significant corneal endothelial injury and postoperative redislocation. RESULTS: This study included 395 eyes undergoing surgery with SFIOL (237 eyes, 60%) or RPICIOL (158 eyes, 40%), with no differences in baseline BCVA (p = 0.76) or endothelial cell density (ECD) (p = 0.39). Compared with the SFIOL group, the RPICIOL group had faster visual recovery (postoperative month 1, 20/37 vs. 20/46, p = 0.005) sustained to over 36 months (p = 0.034), favourable absolute prediction errors (within 0.5D, 67.3% vs. 54.9%, p = 0.027), and shorter operation times (52.7 vs. 60.9 min, p = 0.015). There was no difference in ECD after 12 months (P = 0.282). Over a mean follow-up duration of 33 months (152 cases >3 years, 38.5%), the SFIOL group experienced more cases of tilted/decentred IOLs (14 vs. 1, p = 0.006), suture exposure (7 vs. 0, p = 0.045), and redislocation (17.7% vs. 10.1%, p < 0.001). Multivariable regression showed that a final BCVA below 20/40 was associated with SFIOL (P = 0.007), older age (p = 0.001), intraoperative complications (p = 0.002), past history of vitrectomy/glaucoma surgery or uveitis (p = 0.046), and surgically induced astigmatism >1D (p = 0.029). CONCLUSIONS: RPICIOL appears to be a safe and effective surgical option for secondary IOL surgery over a long-term follow-up, comparing favourably against conventional SFIOL.

Pachydrusen, choroidal vascular hyperpermeability, and punctate hyperfluorescent spots.

PURPOSE: To investigate the relationship between pachydrusen and features of choroidal vascular hyperpermeability (CVH) and punctate hyperfluorescent spots (PHS) on serial imaging in patients with polypoidal choroidal vasculopathy (PCV) or pachychoroid neovasculopathy (PNV). METHODS: Patients diagnosed between January 2007 and June 2016 at 2 high-volume, tertiary hospitals were retrospectively reviewed with serial multimodal imaging assessment. The primary outcome was the association between drusen subtypes (hard/soft drusen, subretinal drusenoid droplets, or pachydrusen) with CVH and PHS, previously described in central serous chorioretinopathy. RESULTS: Among the 105 eyes (105 patients; mean age, 67.0 years), 87 (82.9%) were diagnosed with PCV and 18 (17.1%) with PNV. Pachydrusen was the most frequently identified subtype (54 eyes, 51.4%). CVH (72.2% vs 41.4%, P = 0.021) and PHS (72.2% vs 44.8%, P = 0.041) were observed with greater frequency in PNV eyes. Significant correlations were found between CVH and PHS (phi coefficient φ 0.30, P = 0.003), and PHS with pachydrusen (φ 0.20, P = 0.040). Over a mean follow-up of 74.8 months, new drusen co-localizing to PHS were noted in 22 (21.0%) eyes (φ 0.54, P < 0.001). CONCLUSION: We observed a trend of pachydrusen appearing in conjunction with PHS in PCV or PNV. Frequent localization of new drusen to these choroidal lesions was observed over long-term follow-up. PHS may be a form of late-staining "forme fruste" drusen, possibly associated with micro-ischemic changes to the choriocapillaris.

Clinical features and prognostic factors in 71 eyes over 20 years from patients with Coats' disease in Korea.

This retrospective study assesses the clinical features, treatment strategies, and long-term outcomes of patients with Coats' disease in Korea. Multimodal imaging and medical records of consecutive patients treated between July 2000 and April 2020 at two tertiary centers were evaluated based on onset age (adult vs. childhood [< 18 years]). Factors associated with final visual acuity (VA) and risk of treatment failure were assessed. A total of 71 eyes of 67 patients were included, with subgrouping by onset age showing 45% childhood and 55% adult cases. Overall, Stage 2 disease was most common at presentation (76%), though childhood cases had more Stage 3b (22% vs. 3%, P = 0.02) and greater clock hours of retinal telangiectasia (7 vs. 5, P = 0.005). First-line treatment included laser (25%), combined laser/anti-VEGF (23%), cryotherapy (20%), surgery (16%), and anti-VEGF only (9%). Cryotherapy was associated with a higher risk for secondary interventions (OR 11.8, P < 0.001), required in 56% overall. Despite a 3-line VA decrease in 34% overall, adult cases had superior final VA (P = 0.037). Multivariable regression showed that the number of anti-VEGF injections performed during the initial treatment period was associated with a 9.4 letter improvement in vision (P = 0.041). We observed a higher proportion of adult-onset Coats' disease than previously reported in other non-Asian populations. An aggressive treatment with the addition of anti-VEGF may yield the most favorable long-term visual outcomes.

Long-term surgical outcomes of primary retropupillary iris claw intraocular lens implantation for the treatment of intraocular lens dislocation.

We aimed to investigate the efficacy and safety of primary retropupillary iris claw intraocular lens (R-IOL) implantation in patients with complete intraocular lens (IOL) dislocation. In this single-center retrospective case series, we reviewed the medical records of patients who underwent R-IOL implantation surgery with pars plana vitrectomy for the treatment of IOL dislocation between September 2014 and July 2019. The primary outcome was change in visual acuity (VA) up to 24 months postoperatively. The secondary outcomes included changes in intraocular pressure (IOP), refractive errors, and endothelial cell count (ECC) over the same period. Data of 103 eyes (98 patients) were analyzed. The mean uncorrected VA was significantly improved at one month postoperatively (- 0.69 logMAR, P < 0.001), compared to the preoperative value. IOP (- 2.3 mmHg, P = 0.008) and ECC (- 333.4 cells/mm2, P = 0.027) significantly decreased one month post-surgery and remained stable thereafter. Postoperative mean spherical equivalents were similar to the prediction error throughout the follow-up period. IOP elevation (n = 8, 7.8%), cystoid macular edema (n = 4, 3.9%), and dislocation of the R-IOL (n = 10, 9.7%) were managed successfully. Overall, primary R-IOL implantation with pars plana vitrectomy is effective and safe for correcting IOL dislocation due to various causes.

Clinical outcomes of combined pars plana vitrectomy and scleral fixation of the intraocular lens with a suspension bridge method in eyes with aphakia or insufficient capsular support.

PURPOSE: To describe a modified technique of scleral fixation for intraocular lens (IOL) implantation and report the clinical outcomes of combined pars plana vitrectomy and scleral IOL fixation using the suspension bridge method. METHODS: This retrospective case series included 57 eyes (56 patients) of aphakia or phakic and pseudophakic eyes with insufficient capsular support that underwent IOL implantation or dislocated IOL repositioning with scleral fixation using the 'suspension bridge' method by a single surgeon between 1 July 2010 and 1 March 2019. Preoperative status, changes in visual acuity, refractive outcomes as spherical equivalent and related complications were assessed with a minimum follow-up of 3 months. RESULTS: The mean follow-up period was 25.5 ± 25.4 months. Preoperative visual acuity (logarithm of the minimum angle of resolution) was 1.32 ± 0.68 (20/400 Snellen), and it significantly improved to 0.80 ± 0.53 (20/125), 0.59 ± 0.56 (20/80) and 0.24 ± 0.37 (20/35) at 1 week, 1 month and 3 months, respectively (p < 0.001). Postoperative complications included corneal wound dehiscence (n = 1), vitreous incarceration (n = 1), optic-iris capture (n = 6) and cystoid macular oedema (n = 1). The above-mentioned complications were successfully corrected with simple procedures. However, one case of IOL dislocation required reoperation. CONCLUSION: The modified technique of the suspension bridge method precludes the need for a scleral flap, with the advantage of easy adjustment of the IOL position. It is a simple and feasible technique with good surgical results and low complication rates.

Clinical Spectrum of Uveal Metastasis in Korean Patients Based on Primary Tumor Origin.

PURPOSE: To describe the clinical features and prognosis of patients with uveal metastasis in Korea. DESIGN: Retrospective, observational case series. PARTICIPANTS: Patients diagnosed at 2 tertiary high-volume centers between November 2005 and November 2019. METHODS: Evaluation of multimodal imaging and electronic medical records. MAIN OUTCOME MEASURES: The clinical features and outcomes were assessed based on the primary cancer site. RESULTS: A total of 134 uveal metastases (128 choroidal, 3 iris, and 3 ciliary body tumors) were diagnosed in 95 eyes of 80 patients. Mean age at diagnosis was 56 years (median, 55 years; range, 24-86 years), with a minor preponderance of women (61%). Tumors were bilateral in 15 patients (19%) and the primary origin was established in 49 patients (61%) before ocular detection. The primary tumor originated in the lung (48%), breast (24%), gastrointestinal tract (10%), liver (3%), pancreas (3%), kidney (1%), cervix (1%), and nasopharynx (1%), with some remaining unknown (10%). The overall 5-year survival rate was 21%. Kaplan-Meier analysis revealed that the worst survival was found in pancreatic cancers (mean survival, 5.9 months; P = 0.045), and the best survival was found in gastrointestinal tract cancers (mean survival, 44.5 months). CONCLUSIONS: The primary tumor origins in Korean patients with uveal metastases differed from those reported in primarily population-based studies of White patients, with a higher prevalence of lung and gastrointestinal tract cancers.