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Purpose: The number of patients with acute acquired comitant esotropia (AACE) has been increasing in Japan. The purpose of this study was to investigate the changes in the number and characteristics of patients with AACE examined in our institution during a 12-year period. Methods: We retrospectively reviewed the medical records of patients with AACE aged < 30 years who suddenly developed diplopia or esotropia and were examined in Hyogo College of Medicine Hospital from January 2008 to December 2021. We investigated the association of the yearly changes in the number of patients with the age category, refractive error category, AACE type, esotropia type, and use or nonuse of smartphones. Results: The total number of patients with AACE was 171, and this number significantly increased each year (Pearson correlation coefficient, 0.9450; p < 0.0001). Significant increases were found among students in junior high school and beyond, patients with myopia, patients with Bielschowsky type AACE, and patients with basic esotropia (p < 0.0001 for all). We compared two age groups, elementary school students and below versus junior high school students and above, and found that the rate of increase was significantly higher in the junior high school students and above (estimate, 1.951; p < 0.0001), and the non-myopia group and myopia group and found that the rate of increase was significantly higher in the myopia group (estimate, 1.891; p < 0.0001). Excessive use of smartphones was confirmed in 82 of 133 patients, and the rate of the increase in the number of patients with AACE was significantly greater among patients with than without excessive use of smartphones (estimate, 1.098; p = 0.0009). Conclusion: This study confirmed a significant increase in the number of patients with AACE in recent years. The excessive use of smartphones may be associated with the increase in AACE. [ABSTRACT FROM AUTHOR]

Introduction: Neovascular age-related macular degeneration (nAMD) is the world's leading cause of blindness in elderly people. While anti-vascular endothelial growth factor (VEGF) treatments are used as the first option for patients with nAMD, they are generally expensive and need repeated injections. This study aimed to evaluate the cost-effectiveness of anti-VEGF therapies, focusing on the newly launched ranibizumab biosimilar (RBZ BS) in patients with nAMD from a Japanese societal perspective. Methods: A Markov model was developed to simulate the lifetime transitions of a cohort of treatment-naïve patients with nAMD through health states that were based on the involvement of nAMD (single eye vs. both eyes), the treatment status of the patients, and decimal best-corrected visual acuity. The model compared RBZ BS with branded RBZ, aflibercept (AFL), and AFL as loading dose switched to RBZ BS in maintenance in the treat-and-extend (TAE) regimen (RBZ TAE, AFL TAE, and AFL to RBZ BS TAE, respectively), and with branded RBZ in the pro re nata (PRN) regimen, as well as best supportive care (BSC). All processes were validated by five clinical experts. Results: When TAE regimens were compared, RBZ BS was dominant (higher quality-adjusted life-years (QALYs) and lower total cost) to AFL TAE and AFL to RBZ TAE. The result was robust regardless of whether the clinical data were taken from the direct head-to-head clinical trial or from indirect treatment comparison. RBZ BS TAE was cost-saving compared to RBZ TAE. RBZ BS TAE was estimated to be dominant to BSC owing to a lower societal cost. Like TAE regimens, RBZ BS was cost-saving compared to RBZ PRN and was dominant to BSC in PRN regimens. Conclusion: This study suggests that RBZ BS is dominant to other anti-VEGF treatments in patients with nAMD in both TAE and PRN regimens and BSC from a Japanese societal perspective. [ABSTRACT FROM AUTHOR]

In the sentence beginning 'The top three influential parameters for RBZ BS TAE versus AFL TAE were..'. of the Deterministic Sensitivity Analysis section in Results, the text '(3) treatment frequency of AFL TAE in year 2 (lower to favorable for RBZ BS TAE)' should have read '(3) treatment frequency of AFL TAE in year 2 (higher to favorable for RBZ BS TAE)'. In the sentence beginning "Fourth, since the productivity loss of caregivers..". under the Discussion, the text 'with daily care related to the severity of visual impairment was available in existing studies and relied on expert opinions,..'. should have read 'with daily care related to the severity of visual impairment was not available in existing studies and relied on expert opinions,...'. [Extracted from the article]

Purpose: To evaluate real-world evidence for intraocular pressure (IOP) elevation after subtenon triamcinolone acetonide injection (STTA) in 1252 Japanese patients (1406 eyes) in the Japan Clinical REtina STudy group (J-CREST). Methods: This was a multicentre retrospective study of the medical records of 1252 patients (676 men (758 eyes); mean age: 63.8 ± 12.9 years) who received STTA in participating centres between April 2013 and July 2017. Results: IOP elevation was observed in 206 eyes (14.7%) and IOP increase ≥ 6 mmHg was found in 328 eyes (23.3%). In total, 106 eyes (7.5%) needed medication and two eyes (0.14%) needed surgical procedures. Younger age, higher baseline IOP, and steroid dose were risk factors associated with IOP elevation. Risk factors associated with IOP increase ≥ 6 mmHg were younger age, lower baseline IOP, steroid dose, and higher incidences of diabetic macular oedema (DME) and uveitis. In contrast, with steroid dose fixed at 20 mg, a lower incidence of DME was a risk factor for increased IOP, suggesting that STTA had dose-dependent effects on IOP increase, especially in patients with DME. Conclusion: Our real-world evidence from a large sample of Japanese patients who received STTA showed that the incidence of IOP elevation after STTA was 14.7%, and was associated with younger age, higher baseline IOP, and steroid dose. Thus, IOP should be monitored, especially in patients with younger age, higher baseline IOP, and higher incidences of DME and uveitis. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate real‐world evidence for young Japanese patients with rhegmatogenous retinal detachment (RRD) by retrospectively examining surgical procedures and clinical outcomes in the Japan Clinical Retina Study group. Methods: This was a multicentre retrospective study of the medical records of 562 young patients (384 males and 178 females; mean age: 33.0 ± 11.8 years) who had been diagnosed with RRD and who underwent surgical procedures in participating centres during the period between April 2013 and August 2016. Results: The selected surgeries were scleral buckling (SB) for 295 eyes (49.7%) and pars plana vitrectomy (PPV) for 262 eyes (44.1%). Between the two surgical procedures, there was no significant difference in the primary anatomical reattachment rate (PARR, SB = 92.2%, PPV = 93.9%); improvements in vision were noted in both groups. The incidences of proliferative vitreoretinopathy and cataract formation within 1 year of PPV were 2.3% (p = 0.0047) and 6.5% (p = 0.0005), whereas they were 0% and 1.0% in the SB group, respectively. Conclusion: Scleral buckling (SB) and PPV were chosen with almost equal frequency for young patients with RRD. Clinical outcomes for SB and PPV exhibited a similar PARR. The incidence of cataract formation after PPV may constitute an important limitation of the procedure. [ABSTRACT FROM AUTHOR]

Aims/Introduction: To determine the current clinical preferences of anti‐vascular endothelial growth factor (VEGF) treatment protocols for diabetic macular edema (DME) in Japan. Materials and Methods: This was a descriptive cross‐sectional study. Answers to a questionnaire consisting of 16 questions were obtained from 176 of 278 (63.3%) surveyed ophthalmologists. Results: The results showed that 81.2% preferred intravitreal injections of anti‐VEGF antibodies as the first‐line therapy. The most important indicators for beginning anti‐VEGF therapy were: the best‐corrected visual acuity in 44.3% and the retinal thickness in 30.7%. In the loading phase, 53.4% preferred a single injection, and in the maintenance phase, 75.0% preferred the pro re nata regimen. Financial limitation (85.8%) was reported as the most important difficulty in the treatment. For combination therapy with anti‐VEGF treatment, panretinal photocoagulation, focal photocoagulations and a sub‐Tenon steroid injection were preferred. The contraindications for anti‐VEGF therapy were: prior cerebral infarction (72.7%). Regarding the use of both approved anti‐VEGF agents in Japan, ranibizumab and aflibercept, 39.8% doctors used them appropriately. Conclusions: Our results present the current clinical preferences of anti‐VEGF treatment for DME in Japan. The best‐corrected visual acuity and the retinal thickness are important indicators to institute this therapy. The majority of the ophthalmologists use anti‐VEGF treatment as first‐line therapy and prefer the 1 + pro re nata regimen. We show the current clinical preferences of anti‐VEGF)treatments for DME in Japan. [ABSTRACT FROM AUTHOR]

In the original publication, incorrect Supplementary Material has been published. The online version of the original article can be found at https://doi.org/10.1007/s10384-023-00985-w. [Extracted from the article]

Purpose: To determine the baseline characteristics of patients with central retinal vein occlusion (CRVO) that were significantly associated with the best-corrected visual acuity (BCVA) at the initial examination. Methods: This was a retrospective multicenter study using the medical records registered in 17 ophthalmological institutions in Japan. Patients with untreated CRVO (≥20-years-of-age) who were initially examined between January 2013 and December 2017 were studied. The patients' baseline factors that were significantly associated with the BCVA at the initial examination were determined by univariate and multivariate linear regression analyses. Results: Data from 517 eyes of 517 patients were analyzed. Univariate analyses showed that an older age (r = 0.194, p < 0.001) and the right eye (r = −0.103, p < 0.019) were significantly associated with poorer BCVA at the initial visit. Multivariate analyses also showed that an older age (β = 0.191, p < 0.001) and the right eye (β = −0.089, p = 0.041) were significantly associated with poorer BCVA at the initial visit. Conclusions: The results indicate that an older age, a known strong factor, and the right eye were significantly associated with poorer BCVA at the initial visit to the hospital. These results suggest that functional and/or anatomical differences between the right and left eyes may be involved in these results. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to assess driving capabilities in patients with exudative age-related macular degeneration (AMD) causing unilateral blindness or paracentral scotoma without vision deterioration. Methods: Of the 275 patients with AMD who responded to a questionnaire regarding car driving at Osaka University Hospital, we excluded 78 patients who answered that they had never driven. Finally, 197 patients were included (50 with bilateral and 142 with unilateral AMD). We investigated the relationship between the questionnaire findings and best-corrected visual acuity (BCVA). Results: The mean age was 74.8 ± 6.9 years, and the mean BCVA in the right and left eyes were 0.48 and 0.47, respectively. A negative correlation was observed between the proportion of patients who stopped driving due to AMD and the vision in the worse eye (p < 0.0001); however, 66% of participants were still driving. Regardless of the BCVA, 84% of them wished to continue driving. Concerning perceived dangerous situations, all patients reported an oversight of people or signals and night driving; further, patients with unilateral and bilateral vision deterioration reported vision narrowness and difficulty with discerning signal colours, respectively. Conclusion: Despite the associated danger, patients with AMD continued driving. Close attention should be paid to the driving activities among patients with AMD, even if they have passed the relevant driving tests. [ABSTRACT FROM AUTHOR]

Purpose: We investigated potential risk factors for visual prognosis in Japanese patients with exogenous endophthalmitis., Methods: In this retrospective observational multicenter cohort study, risk factors for legal blindness at 12 weeks after treatment initiation were evaluated based on patient characteristics, initial BCVA, causative events, pathogens, ocular symptoms, duration from symptom onset to initial treatment, and selected treatments., Results: Overall, 23.1% of eyes developed legal blindness. The six risk factors for legal blindness were presence of eye pain, pathogen identification, poor BCVA at the initial visit, longer duration from symptom onset to initial treatment, type of causative event, and type of causative pathogen. Regarding the type of causative pathogen, coagulase-negative staphylococci was associated with a better visual impairment outcome., Conclusion: Exogenous endophthalmitis remains a severe ocular infection; however, it can be managed with rapid treatment, as well as other advances in medical knowledge and technology.

Purpose: To evaluate 2-year efficacy, durability, and safety of faricimab in the TENAYA Japan subgroup and pooled global TENAYA/LUCERNE cohort of patients with neovascular age-related macular degeneration (nAMD)., Methods: Subgroup analysis of TENAYA/LUCERNE (NCT03823287/NCT03823300): phase III, multicentre, randomised, active comparator-controlled, double-masked, non-inferiority trials. Treatment-naïve patients aged ≥ 50 years with nAMD were randomised (1:1) to intravitreal faricimab (6.0 mg up to every 16 weeks [Q16W] after 4 initial Q4W doses) or aflibercept (2.0 mg Q8W after 3 initial Q4W doses). Outcomes were assessed through year 2 for the TENAYA Japan subgroup (N = 133) and global pooled TENAYA/LUCERNE cohort (N = 1329)., Results: Vision and anatomic improvements achieved with faricimab at year 1 were maintained over 2 years and were generally comparable between the TENAYA Japan subgroup and pooled TENAYA/LUCERNE cohort. Adjusted mean best-corrected visual acuity (BCVA) change from baseline at year 2 for the TENAYA Japan subgroup and global pooled TENAYA/LUCERNE cohort was +7.1 (3.7-10.5) and +4.4 (3.2-5.5) letters in the faricimab arm, respectively, and +5.2 (1.9-8.6) and +4.3 (3.1-5.4) letters in the aflibercept arm, respectively. At week 112, the proportion of faricimab-treated patients on Q16W dosing was 61.0% and 63.1% in the TENAYA Japan subgroup and pooled TENAYA/LUCERNE cohort. Faricimab was well tolerated through year 2., Conclusion: Year 2 TENAYA Japan subgroup findings for faricimab were generally consistent with the pooled global TENAYA/LUCERNE results in patients with nAMD. Vision and anatomical benefits with faricimab were similar to those with aflibercept but with fewer injections., (© 2024. The Author(s).)

We investigated the impact of drainage retinotomy on the outcome of pars plana vitrectomy for repair of rhegmatogenous retinal detachment (RRD). This study was a retrospective observational multicenter study. All patients were registered with the Japan-Retinal Detachment Registry. We analyzed 1887 eyes with RRD that had undergone vitrectomy and were observed for 6 months between February 2016 and March 2017. We compared the baseline characteristics and postoperative outcomes between eyes with and without drainage retinectomy. We then performed propensity score matching using preoperative findings as covariates to adjust for relevant confounders. Of 3446 eyes, 1887 met the inclusion criteria. Among them, 559 eyes underwent vitrectomy with drainage retinotomy, and 1328 eyes underwent vitrectomy without drainage retinotomy. After propensity score matching, each group comprised 544 eyes. There was no significant difference between the two groups in BCVA at 6 months after vitrectomy (0.181 vs. 0.166, P = 0.23), the primary anatomical success rate (6.3% vs. 4.4%, P = 0.22), or the rate of secondary surgery for ERM within 6 months (1.5% vs. 1.3%, P = 1.0). Drainage retinectomy does not increase the risk of decreased postoperative BCVA, surgical failure, or secondary surgery for ERM within six months outcomes., (© 2024. The Author(s).)

Purpose: Neovascular age-related macular degeneration (nAMD) is classified into typical AMD (tAMD), polypoidal choroidal vasculopathy (PCV), and retinal angiomatous proliferation (RAP). This study investigated clinical features of the 3 subtypes and visual outcome associated with treatment regimens in a large cohort of patients with nAMD in a clinical setting., Design: Retrospective multicenter cohort study., Participants: Five hundred patients with treatment-naive nAMD (268 tAMD, 200 PCV, and 32 RAP) initiated with anti-VEGF agents and followed for 1 year., Methods: Medical records were reviewed to extract demographic data, best-corrected visual acuity at baseline and 1 year after treatment initiation, spectral-domain OCT findings, baseline fellow eye condition, systemic factors, treatment strategies, and number of intravitreal injections in the first year., Main Outcome Measures: Primary outcome measures were anti-VEGF treatment strategy (ranibizumab or aflibercept, anti-VEGF regimen, concomitant photodynamic therapy, drug switch), best-corrected visual acuity at 1 year, and factors associated with visual acuity., Results: Patients with RAP were significantly older, were more commonly women, and had more macular lesions in fellow eye than patients with tAMD and PCV. Smoking history and diabetes prevalence were not different among the 3 subtypes. Frequencies of subretinal fluid were higher and intraretinal fluid were lower in tAMD and PCV than in RAP, whereas serous pigment epithelial detachment and subretinal hemorrhage were higher in PCV than in tAMD and RAP. Choice of anti-VEGF agents and treatment regimens did not differ among 3 subtypes. The aflibercept-to-ranibizumab ratio was approximately 7:3. The mean number of injections in 1 year was 5.3 ± 2.4 in nAMD overall, which was significantly less in pro re nata (PRN) than in treat and extend (TAE) regardless of the anti-VEGF agent. Best-corrected visual acuity improved in all 3 subtypes, although it was not significant in patients with RAP., Conclusions: This clinical study demonstrates that treatment regimens were similar in 3 subtypes and aflibercept was used in 70% of all patients. Approximately 5 injections were given in the first year regardless of the anti-VEGF agent, which was significantly less in PRN regimen than in TAE. Visual acuity improvement was observed after 1-year anti-VEGF therapy in all 3 subtypes, but was not significant in RAP., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023. Published by Elsevier Inc.)

Purpose: To evaluate the 1-year efficacy, durability, and safety of faricimab versus aflibercept in patients with neovascular age-related macular degeneration (nAMD) enrolled in the Japan subgroup of the TENAYA trial., Study Design: TENAYA (NCT03823287) was a global, phase 3, multicenter, randomized, active comparator-controlled, double-masked, noninferiority, parallel-group, 112-week trial. After completion of global enrollment, additional patients were enrolled in the Japan extension of TENAYA., Methods: Treatment-naïve patients aged ≥ 50 years with nAMD were randomized (1:1) to intravitreal faricimab 6 mg up to every 16 weeks (Q16W) after 4 initial Q4W doses based on disease activity at weeks 20 and 24 or aflibercept 2 mg Q8W after 3 initial Q4W doses. Primary endpoint was mean change in best-corrected visual acuity (BCVA) from baseline averaged over weeks 40, 44, and 48. Anatomical/durability outcomes were assessed., Results: Overall, 133 patients were included in the TENAYA Japan subgroup analysis (faricimab, n = 66; aflibercept, n = 67). The adjusted mean (95% confidence interval) BCVA changes were + 7.1 (4.6‒9.7) and + 7.7 (5.2‒10.1) letters in the faricimab and aflibercept treatment groups, respectively. At week 48, 66.1%, 22.6%, and 11.3% of patients in the faricimab group were on Q16W, Q12W, Q8W and dosing intervals, respectively. Ocular adverse event rates were similar between treatment groups (faricimab, n = 14 [21.2%] versus aflibercept, n = 17 [25.4%])., Conclusion: The TENAYA Japan subgroup analysis showed that faricimab up to Q16W had sustained efficacy with an acceptable safety profile. These findings are consistent with the global TENAYA and LUCERNE findings., (© 2023. Japanese Ophthalmological Society.)

We describe the clinical characteristics of treatment-naïve polypoidal choroidal vasculopathy (PCV) in three tertiary clinic settings in 2 cities (Chicago in the USA and Nishinomiya in Japan). This cohort study was a retrospective, multicenter, consecutive case series. A total of 126 patients with treatment-naïve PCV-46 in Chicago and 80 in Nishinomiya-were identified. The proportion of PCV in patients with neovascular age-related macular degeneration was lower in Chicago (10.8% vs. 36.9%). Patients in Chicago had a significantly higher prevalence of soft drusen (50.0% vs 25.0%, p = 0.006) and intra-retinal cyst (37.0% vs 15.0%, p = 0.008), and a significantly lower prevalence of pachyvessels (41.3% vs 62.5%, p = 0.03). At baseline, presenting vision for patients in Chicago was worse than in Nishinomiya (mean log MAR: 0.609 vs. 0.312, p < 0.001). Ninety-five eyes were followed for more than one year. The Nishinomiya group received a higher rate of combination therapy (61.0%) compared to the Chicago group (5.3%). Vision and central foveal thickness at month 12 were significantly improved from baseline in both Chicago (p = 0.009 and p = 0.01) and Nishinomiya groups (both p < 0.001). Our study highlights interesting differences in the proportion of PCV, clinical findings and treatment responses of PCV, that need to be further evaluated in larger, epidemiologic cohorts., (© 2023. The Author(s).)

Purpose: To evaluate the clinical characteristics, treatment trends, and visual prognosis of submacular hemorrhage (SMH) secondary to neovascular age-related macular degeneration (nAMD) and retinal arterial macroaneurysm (RAM)., Methods: This retrospective study enrolled 187 Japanese patients with SMH at 10 institutions from 2015 to 2018. Medical records including SMH etiology, best-corrected visual acuity (BCVA), fundus photographs, optical coherence tomography images, and selected treatments were analyzed., Results: Major causes of SMH were typical nAMD (tnAMD) (18%), polypoidal choroidal vasculopathy (PCV) (50%) and RAM (29%). Age, male/female ratio, baseline BCVA, central retinal thickness, and involved retinal layers were significantly different between etiologies (all P<0.0001). Treatment with anti-vascular endothelial growth factor drugs with and without intravitreal gas injection was chosen for half of eyes in the tnAMD and PCV groups, whereas vitrectomy was performed in 83.7% of eyes with RAM. The final BCVA improved significantly from baseline in the PCV and RAM groups (P = 0.0009, P<0.0001) and final BCVA was significantly better in the PCV group at a level similar to the other groups (P = 0.0007, P = 0.0008). BCVA improvement from baseline was significantly greater in the RAM group compared with the tnAMD (P = 0.0152) and PCV (P = 0.017) groups. Multivariate analysis revealed better final BCVA was significantly associated with younger age (P = 0.0054), better baseline BCVA (P = 0.0021), RAM subtype (P = 0.0446), and no tnAMD (P = 0.001)., Conclusions: The characteristics of, and treatment strategy for, SMH were different between the underlying diseases. Anti-vascular endothelial growth factor treatment with or without expansile gas was mainly chosen for SMH in tnAMD and PCV, whereas vitrectomy with gas was the most common treatment for RAM, and the higher rate for vitrectomy might result in the greater BCVA improvement in the RAM group than in the other groups. Final BCVA was better in PCV, RAM, and tnAMD, in that order, because patients with PCV were younger and had better baseline BCVA., Competing Interests: The authors have declared that no competing interests exist.

Purpose: To evaluate the progression of early age-related macular degeneration to neovascular age-related macular degeneration (nAMD), and identify the abnormal fundus autofluorescence (FAF) patterns and markers of choroidal neovascularization (CNV) in fellow eyes of patients with unilateral nAMD., Methods: Sixty-six patients with unilateral nAMD who developed abnormal FAF in the fellow eyes were enrolled in this multicenter, prospective, observational study, and followed-up for 5 years. FAF images on Heidelberg Retina Angiogram Digital Angiography System (HRA) or HRA2 were classified into eight patterns based on the International Fundus Autofluorescence Classification Group system. The patients in which the fellow eyes progressed to advanced nAMD, including those who did not develop nAMD, were assessed based on the following factors: baseline FAF patterns, age, sex, visual acuity, drusen, retinal pigmentation, baseline retinal sensitivity, family history, smoking, supplement intake, hypertension, body mass index, and hematological parameters., Results: Of the 66 patients, 20 dropped out of the study. Of the remaining 46 patients, 14 (30.42%, male: 9, female: 5) progressed to nAMD during the 5-year follow-up. The most common (50% eyes) FAF pattern in the fellow eyes was the patchy pattern. According to the univariate analysis, CNV development was significantly associated with age, supplement intake, and low-density lipoprotein levels (p<0.05). Multivariable analysis revealed that patients who showed non-compliance with the supplement intake were more likely to develop nAMD (p<0.05). No significant association was found between the patchy pattern and CNV development (p = 0.86)., Conclusion: The fellow eyes (with abnormal FAF) of patients with unilateral nAMD may progress from early to advanced nAMD. However, no FAF pattern was found that predicted progression in nAMD., Competing Interests: Santen Pharmaceutical Co., Ltd. provided support in the form of honoraria for speaking and/or organizing at meetings [TY, RM, MS, AS, TS, Yuji Oshima, Yuichiro Ogura, TI, FG]. Mayumi Nakamura and Erika Kimura are employees of Santen Pharmaceutical Co., Ltd. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Purpose: To investigate the effects of cataract surgery in Japanese patients with neovascular age-related macular degeneration (nAMD)., Methods: The primary endpoint in this prospective, observational study was patient satisfaction at 6 months after cataract surgery in patients with nAMD. Secondary endpoints comprised changes in best-corrected visual acuity (BCVA), M-chart score, central retinal thickness (CRT), AMD status, and number of AMD treatments. All examinations were performed before surgery, and at 1, 3, and 6 months postoperatively., Results: Fifty patients (52 eyes) were included in this study (32 men; mean age, 76.1 ± 7.1 years). Thirty-nine patients (75.0%) reported satisfaction with cataract surgery. BCVA significantly improved at all postoperative timepoints (all p < 0.0001), whereas differences in M-chart scores were not statistically significant. The number of eyes with BCVA ≤ 0.3 logarithm of the minimum angle of resolution (logMAR) increased from 21 to 38; however, CRT did not change. The number of AMD treatments did not change during follow-up. All questionnaire scores showed postoperative improvement. Univariate and multivariate analyses revealed that final BCVA ≤ 0.3 logMAR was significantly associated with patient satisfaction., Conclusion: Cataract surgery significantly improved vision in Japanese patients with nAMD, without affecting AMD status. Patients were satisfied with cataract surgery, especially with respect to improvement of distance vision.

Purpose: Leber's hereditary optic neuropathy (LHON) is a mitochondrial neuropathy that causes acute vision loss. Idebenone, a short-chain ubiquinone analog that preserves mitochondrial function is thought to suppress disease progression in early-onset LHON patients. We investigated the effects of idebenone in Japanese LHON patients., Study Design: Prospective, interventional, non-comparative study in patients with definite LHON diagnosis, under trial registration number UMIN000017939., Methods: Fifty-seven patients received 900 mg/day idebenone for 24 weeks. We measured baseline best-corrected visual acuity, visual fields, critical fusion frequency and retinal ganglion cell layer complex thickness; we assessed efficacy at 24 and 48 weeks, and safety throughout., Results: Patients were predominantly male (91.2%) and most had an mt.11778G>A mutation (94.7%). All patients tolerated idebenone therapy well. Data from the 51 mt.11778 patients were compared with their baseline data. At 48 weeks, significant improvement in best-corrected visual acuity was observed in 17 patients (33.3%). Furthermore, 25.5% of patients showed improvements in visual fields and 33.3% in critical fusion frequency. However, retinal ganglion cell layer complex thickness was significantly reduced. Among patients who started idebenone >1 year after disease onset, visual improvement was found in 12 (38.7%). Among patients who developed LHON before 19 years of age, visual improvement was found in 11 (42.3%)., Conclusion: Idebenone's potential and favorable safety profile were confirmed in Japanese LHON patients. However, this study had no placebo group; therefore, we need to undertake a prospective intervention study to further investigate the therapeutic effects of Idebenone in Japanese LHON patients.

Purpose: To assess the 5-year change in abnormal fundus autofluorescence (FAF) patterns and retinal sensitivity in the fellow eye of Japanese patients with unilateral exudative age-related macular degeneration (AMD)., Methods: Patients with unilateral exudative AMD who developed abnormal FAF in the fellow eyes were enrolled. FAF imaging and microperimetry were performed at baseline and follow-ups. FAF findings were classified into 8 patterns based on the International Fundus Autofluorescence Classification Group to assess retinal sensitivity. Forty-five points covering the central 12 degrees on microperimetry were superimposed onto the FAF images. Each point was classified depending on the distance from the abnormal FAF. "Close" was defined as the portion within 1 degree from the border of any abnormal FAF, and "Distant" was defined as the portion over 1 degree from the border of abnormal FAF. To investigate the association between the retinal sensitivity and distance from the abnormal FAF, hierarchical linear mixed-effect models were used with the distance, time and time squared from baseline (months), and angle (degrees) as fixed effects. Differences among patients, eyes, and test point locations were considered successively nested random effects., Results: We studied 66 fellow eyes with abnormal FAF. Twenty-seven eyes were followed-up during the 5 years. In the 13 of 27 eyes (48%), the abnormal FAF patterns had changed during the 5 years. We found retinal sensitivity was associated significantly with the distance from the abnormal FAF ("Distant": p<0.001, time2 from baseline: p<0.001, angle: p<0.001). The mean retinal sensitivity of the "Close" tended to deteriorate after the third year and eventually showed the similar sensitivity as the portion within the abnormal FAF., Conclusion: FAF patterns can change about half during the 5 years and the retinal sensitivity near abnormal FAF tends to deteriorate after the third year., Competing Interests: Santen Pharmaceutical Co., Ltd. provided support in the form of honoraria for speaking and/or organizing at meetings [TY, RM, MS, AS, TS, Yuji Oshima, TI, Yuichiro Ogura, TI, FG]. Mayumi Nakamura, Kiyoshi Matsuno, Noriyuki Kuno, and Erika Kimura are (were) employees of Santen Pharmaceutical Co., Ltd. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Purpose: To evaluate vision-related quality of life (QoL) in wet age-related macular degeneration (wAMD) patients receiving intravitreal aflibercept (IVT-AFL)., Study Design: Prospective, observational Japanese postmarketing surveillance study., Methods: All decisions were made by the treating physician. QoL was assessed using the 25-item National Eye Institute-Visual Functioning Questionnaire (NEI-VFQ-25) composite score administered at baseline, 6 months, and 12 months (primary assessment). Secondary assessments included NEI-VFQ-25 subscale scores, resource use, and best-corrected visual acuity (BCVA; logarithm of the minimum angle of resolution [logMAR])., Results: In total, 576 patients (baseline), 555 patients (6 months), and 446 patients (12 months) were included. The mean (SD) number of IVT-AFL injections was 3.5 (1.2) at 6 months and 4.6 (2.2) at 12 months. The mean (SD) improvement from baseline in the NEI-VFQ-25 composite score was 3.1 (11.1) at 6 months and 2.7 (12.3) at 12 months (P < .0001). For the NEI-VFQ-25 subscale scores, the mean change was ≥ 4 (minimally important difference) for general vision, near vision, and mental health at 6 months, and for general vision and mental health at 12 months (all P < .0001). A significant improvement from baseline was found in mean BCVA (logMAR) at 6 months (-0.1) and 12 months (-0.1) (P < .0001). The mean change from baseline in the NEI-VFQ-25 scores was greatest in patients with improved BCVA (gain of ≤ -0.3 logMAR units or ≥ 15 letters) after treatment., Conclusion: IVT-AFL was associated with significant improvements in QoL and visual acuity in Japanese patients with wAMD in a real-world setting.

We investigated the rates of the use of steroids in Japanese central serous chorioretinopathy (CSC) cases and differences in the characteristics of CSC with and without steroids. A total of 538 eyes of 477 patients diagnosed with CSC, with 3 months or more of follow-up between April 2013 and June 2017 at 8 institutions. Patients with CSC with more than 3 months of follow-up were identified by OCT and fluorescein angiography at 8 institutions. Data collected included patient demographics, history of corticosteroid medication and smoking, spherical errors, findings of angiography, subfoveal choroidal thickness, and changes through the follow-up period. Differences in these findings were analyzed in cases with and without corticosteroid treatment. Among the 477 patients (344 men,133 women), 74 (15.5%) (39 men, 35 women) underwent current or prior steroid treatment. Cases with steroids were higher age (p = 0.0403) and showed no male prevalence, more bilateral involvement (p < 0.0001), and the affected eyes had multiple pigment epithelial detachment (p <0.0001), more fluorescein leakage sites (p < 0.0001), greater choroidal thickness (p = 0.0287) and a higher recurrence rate (p = 0.0412). Steroids can cause severer CSC through an effect on choroidal vessels and an impairment of retinal pigment epithelium., Competing Interests: The authors have declared that no competing interests exist.

Purpose: Abnormal fundus autofluorescence (FAF) potentially precedes onset of late age-related macular degeneration (AMD) in Caucasian patients. Many differences exist between Asian and Caucasian patients regarding AMD types and severity, gender, and genetic backgrounds. We investigated the characteristics of abnormal FAF and retinal sensitivity in the fellow eyes of Japanese patients with unilateral neovascular AMD., Methods: Sixty-six patients with unilateral neovascular AMD and abnormal FAF in the fellow eye were enrolled in this multicenter, prospective, observational study. The best-corrected visual acuity, fundus photographs, FAF images, and retinal sensitivity on microperimetry were measured periodically for 12 months. The FAF images were classified into eight patterns based on the International Fundus Autofluorescence Classification Group. The points measured by microperimetry were superimposed onto the FAF images and fundus photographs and classified as "within," "close," and "distant," based on the distance from the abnormal FAF and other findings. The relationship between the location of the baseline abnormal FAF and retinal sensitivity was investigated., Results: In Japanese patients, patchy (33.3%) and focally increased (30.3%) patterns predominated in the abnormal FAF. Intermediate-to-large drusen was associated predominantly with hyperfluorescence and hypofluorescence. Neovascular AMD developed within 1 year in six (9.1%) eyes, the mean baseline retinal sensitivity of which was 12.8 ± 4.7 dB, significantly (p<0.002) lower than the other eyes. In 44 of the other 60 eyes, microperimetry was measurable at baseline and month 12 and the mean retinal sensitivity improved significantly from 13.5 ± 4.4 to 13.9 ± 4.8 dB (p<0.001), possibly associated with lifestyle changes (e.g., smoking cessation, antioxidant and zinc supplementation). The mean retinal sensitivities of points within and close to the abnormal FAF were 9.9 and 11.7 dB, respectively, which were significantly lower than the 14.0 dB of the points distant from the abnormal FAF., Conclusion: In Japanese patients, patchy and focally increased patterns predominated in the abnormal FAF. The retinal sensitivity was lower close to/within the abnormal FAF. FAF and microperimetry are useful to assess macular function before development of neovascular AMD or geographic atrophy., Competing Interests: Santen Pharmaceutical Co., Ltd. provided support in the form of honoraria for speaking and/or organizing at meetings [TY, RM, MS, AS, TS, Yuji Oshima, TI, Yuichiro Ogura, TI, FG]. Mayumi Nakamura, Kiyoshi Matsuno, Noriyuki Kuno, and Erika Kimura are(were) employees of Santen Pharmaceutical Co., Ltd. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Background/aims: To evaluate efficacy and safety of intravitreal aflibercept (IVT-AFL) in Japanese patients with wet age-related macular degeneration (wAMD) from the VIEW 2 trial., Methods: In this double-masked study, patients were randomised to: 0.5 mg IVT-AFL every 4 weeks (0.5q4); 2 mg IVT-AFL every 4 weeks (2q4); 2 mg IVT-AFL every 8 weeks (2q8) after 3 monthly injections; or 0.5 mg ranibizumab every 4 weeks (Rq4). Main efficacy outcomes included vision maintenance and best-corrected visual acuity (BCVA) at week 52., Results: At week 52, all Japanese patients in the IVT-AFL groups (n=70) maintained vision, compared with 96% of Japanese patients (n=23/24) treated with ranibizumab. Japanese patients in all treatment groups showed improvement in BCVA after treatment. The Rq4, 2q4 and 2q8 groups experienced similar gains in BCVA from baseline. The 0.5q4 group had higher gains due to an unexpected drop in BCVA between screening and baseline. Central retinal thickness and mean area of choroidal neovascularisation decreased in all treatment groups with similar magnitude. Ocular treatment-emergent adverse events were balanced across treatment groups., Conclusions: IVT-AFL was effective and well tolerated in Japanese patients. Outcomes in this population were consistent with those in the overall VIEW 2 population., Trial Registration Number: NCT00637377., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Purpose: To describe the incidence and characteristics of neovascularization in fellow eyes of Japanese patients with unilateral retinal angiomatous proliferation (RAP)., Methods: We retrospectively studied patients with unilateral RAP in one center between 2003 and 2010. The minimal follow-up time was 2 years. The prevalence rates of soft drusen and reticular pseudodrusen in the fellow eyes at the first visit were examined in color fundus photographs and optical coherence tomography images. Stepwise analysis was performed to identify a correlation between the incidence of RAP in the fellow eyes and age, gender, follow-up time, soft drusen, and reticular pseudodrusen., Results: Twenty eyes were included in this study. The mean follow-up time was 49 months (range, 24-108 months). At the first visit, soft drusen was seen in 19 eyes (95%) and reticular pseudodrusen in 11 eyes (55%). Neovascular age-related macular degeneration developed in 10 eyes, including RAP in 9 eyes (45%) and polypoidal choroidal vasculopathy in 1 eye (5%). Stepwise analysis showed that reticular pseudodrusen and longer follow-up time were correlated significantly (P = 0.0384 and P = 0.0341, respectively) with the incidence of RAP., Conclusion: Bilateral RAP developed in almost half of the eyes initially diagnosed with unilateral RAP and the incidence increased with time. Reticular pseudodrusen is a risk factor for bilateral RAP.

Purpose: To assess intravitreal bevacizumab (IVB) for choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD) in Japanese patients., Methods: Forty-seven patients treated with IVB for newly diagnosed subfoveal or juxtafoveal CNV (predominantly classic CNV, 15 eyes; minimally classic CNV, 11 eyes; occult CNV, 21 eyes) secondary to AMD and followed for more than 12 months were reviewed retrospectively. IVB (1 mg) was injected via the pars plana. Additional IVB or photodynamic therapy was administered for either persistent or recurrent exudation. The main outcome measurements were best-corrected visual acuity (BCVA), number of injections, and treatment other than bevacizumab., Results: The mean baseline visual acuity (VA) was 0.38 [logarithm of the minimum angle of resolution (logMAR), 0.42] and final VA was 0.42 (logMAR, 0.38). During 12 months, bevacizumab was injected a mean of 3.4 times. Eight eyes received additional treatment. Up until 12 months, the mean BCVA with predominantly classic CNV increased, whereas the BCVA with minimally classic or occult with no classic CNV stabilized. The mean number of injections for predominantly classic CNV was 2.5, that for minimally classic CNV was 4.9, and that for occult with no classic CNV was 3.3., Conclusions: IVB was especially effective for predominantly classic CNV, but CNV with subretinal pigment epithelial lesions might recur or enlarge despite additional IVB.

Purpose: To compare the long-term visual and anatomic outcome of treatment with photodynamic therapy (PDT) or intravitreal bevacizumab (IVB; Avastin; Genentech Inc, South San Francisco, California, USA) for choroidal neovascularization attributable to pathologic myopia (mCNV)., Design: An open-label, interventional case series., Setting: Multi-institutional., Patients: Thirty-one eyes of Japanese women who received either PDT or IVB for mCNV. Inclusion criteria were age 50 years or older, greatest linear dimension (GLD) 1200 to 3000 microm, and baseline best-corrected visual acuity (BCVA) 20/200 to 20/40. INTERVENTION PROCEDURES: Patients received either PDT or IVB (1 mg/40 microL) throughout the study, with re-treatment when necessary., Main Outcome Measures: BCVA and visual gain/loss at 3, 6, 12, 18, and 24 months after the initial treatment., Results: Age, BCVA, location of CNV, refractive error, and symptom duration at baseline did not differ significantly between groups. BCVA was significantly improved at 3 to 12 months (P < .05); however, the significance was lost at 18 and 24 months in the IVB group. The PDT group showed no significant improvement within the first year, and vision slowly worsened after 12 months, becoming significantly worse at 18 and 24 months compared to baseline (P< .01). BCVA was significantly higher in the IVB group at 6 months (P< .05), and 12 months or further (P < .01). Visual gain was significantly greater in the IVB group at 6, 12, 18, and 24 months (P < .05 for 6, 18, and 24 months and P < .01 for 12 months)., Conclusions: These findings indicate that the effects of PDT and IVB have a different time course, and that IVB provides a significantly better BCVA than PDT for mCNV over the long-term., (Copyright 2010 Elsevier Inc. All rights reserved.)

Objective: To calculate age at onset curves of retinitis pigmentosa (RP) to resolve the difficulty in calculating the recurrence risk in a family. Retinitis pigmentosa is a common hereditary retinal disease that leads to blindness. It is a slow-onset disease, and family members of patients sometimes develop RP later., Methods: We studied 370 patients with typical RP. The age at onset was defined as when the patient's RP was diagnosed by an ophthalmologist. After confirmation that the age at onset came from normal distribution, we drew the age at onset curves., Results: The average age when patients were diagnosed with RP was 35.1 years, and the median age was 36.5. The onset ratio straightly increased with age until 65 years, so the onset ratio was relatively low at young ages., Conclusions: The age at onset curves are quite simple and useful tools that facilitate counseling at an RP clinic. Without them, the recurrence risk would be misleading.