Bilateral vitrectomy in patients with proliferative diabetic retinopathy-characteristics and surgical outcomes.

PURPOSE: Comparing characteristics and outcomes of patients with bilateral proliferative diabetic retinopathy (PDR) undergoing concurrent and sequential vitrectomy. METHODS: Patients having bilateral vitrectomy were classified into concurrent (requiring bilateral surgery simultaneously) and sequential (indicating vitrectomy in one eye later) groups. Clinical characteristics and outcomes were compared, and correlation between the first and second-operated eyes was analyzed. RESULTS: One hundred eight and 126 eyes were in the concurrent and sequential groups, respectively. The sequential group was older (50 vs. 45 years, P = 0.017), had less retinal detachment (54 vs. 77%, P < 0.001), and better visual outcomes (0.79 vs. 1.30, P = 0.021), especially the second-operated eyes. The concurrent group had weaker correlations of disease severity (phi coefficient: 0.36 vs. 0.61) and post-operative visual acuity (r: 0.12 vs. 0.34) between the first- and second-operated eyes than the sequential group. Prior intravitreal injection of anti-vascular endothelial growth factor (odds ratio [OR] 0.37, 95% confidence interval [CI] 0.15-0.86, P = 0.025) predicted better outcomes, while post-operative neovascular glaucoma predicted worse outcomes (OR 6.5, 95% CI 1.7-27.9, P = 0.008). CONCLUSIONS: PDR patients requiring surgery concurrently were younger and had more severe diseases and worse outcomes. However, poor outcomes in the first eye did not predict similar outcomes in the second eye.

Lamellar macular hole in highly myopic eyes and insights into its development, evolution, and treatment: a mini-review.

Lamellar macular holes (LMHs) are a manifestation of myopic tractional maculopathy (MTM). Owing to the complex and multidirectional traction force in the elongated eyeball, the clinical features, development, evolution, and treatment algorithms of LMH in highly myopic eyes may differ from those of idiopathic LMH or MTM in general. This review aimed to specifically explore the LMHs in highly myopic eyes. Several developmental processes of LMH and their association with macular retinoschisis have been demonstrated, with the tractional component identified in all processes. Epiretinal proliferation was more prevalent and more extensive in LMHs in highly myopic eyes than in idiopathic LMHs. LMHs in highly myopic eyes may remain stable or progress to foveal detachment and full-thickness macular hole with or without retinal detachment. The predictive factors associated with disease progression were summarized to facilitate monitoring and guide surgical intervention. The treatment of LMHs in highly myopic eyes was based on an algorithm for treating myopic tractional maculopathy, including gas tamponade, pars plana vitrectomy, macular buckling, and a combination of vitrectomy and macular buckling. New internal limiting membrane (ILM) manipulation techniques such as fovea-sparing ILM peeling or fovea-sparing ILM peeling combined with ILM flap insertion could reduce the risk of developing iatrogenic full-thickness macular holes postoperatively. Further research should focus on the treatment of LMH in highly myopic eyes.

BROAD VITREOMACULAR ATTACHMENT-INDUCED MACULAR HOLE: Structural Changes and Surgical Outcome.

PURPOSE: This study aims to describe the structural changes and surgical outcomes of full-thickness macular holes (FTMHs) induced by vitreomacular traction with broad vitreomacular attachment (VMA). METHODS: A retrospective analysis of idiopathic FTMHs from October 2010 to May 2022 was conducted. Patients with FTMHs and no retinal detachment (RD) induced by broad VMA (the study group) were compared with a control group consisting of typical FTMHs with focal VMA. RESULTS: Thirty-one eyes had broad VMA-associated FTMH, among which seven eyes (22%) were with concurrent RD. Among the total of 24 cases without RD, 8 (33%) exhibited schisis. The incidence of lamellar holes associated epithelial proliferation and multiple membrane traction was significantly higher in the study group. One hundred percent FTMH closure was observed postoperatively in both groups. Although the postoperative visual acuity was not significantly different, the study group showed a greater ellipsoid zone disruption length. CONCLUSION: Broad VMA-induced FTMHs are characterized by diverse macular structural changes, including schisis and macular detachment. These FTMHs are associated with a higher incidence of lamellar holes associated epithelial proliferation and multiple membrane traction. Surgical outcomes for FTMHs induced by broad VMA are similar to those induced by typical focal VMA, with both groups demonstrating a 100% hole-closure rate.

Genetics in neovascular age-related macular degeneration susceptibility and treatment response to anti-VEGF intravitreal injection: A case series study.

BACKGROUND: To identify genotypes associated with neovascular age-related macular degeneration (nAMD) and investigate the associations between genotype variations and anti-vascular endothelial growth factor (VEGF) treatment response. METHODS: This observational, retrospective, case series study enrolled patients diagnosed with nAMD who received anti-VEGF treatment in National Taiwan University Hospital with at least one-year follow-up between 2012 and 2020. A genome-wide association study (GWAS) was conducted on enrolled patients and controls. Correlations between the genotypes identified from GWAS and the treatment response of functional/anatomical biomarkers, including visual acuity (VA), presence of intraretinal or subretinal fluid (SRF), serous or fibrovascular pigmented epithelium detachment (PED), and disruption of the ellipsoid zone (EZ), were analysed. RESULTS: In total, 182 patients with nAMD and 1748 controls were enrolled. GWAS revealed 16 single nucleotide polymorphisms (SNPs) as risk loci for nAMD, including seven loci in CFH and ARMS2/HTRA1 and nine novel loci, including rs117517872 and rs79835234(COPB2-DT), rs7525578(RAP1A), rs2123738(LOC105376755), rs1374879(CNTN3), rs3812692(SAR1A), rs117501587(PRKCA), rs9965945(CNDP1), and rs189769231(MATK). Our study revealed rs800292(CFH), rs11200638(HTRA1), and rs2123738(LOC105376755) correlated with poor treatment response in VA (P = 0.005), SRF (P = 0.044), and fibrovascular PED (P = 0.007), respectively. Rs9965945(CNDP1) was correlated with poor response in disruption of EZ (P = 0.046) and serous PED (P = 0.049). CONCLUSIONS: Among the 16 SNPs found in the GWAS, four loci-CFH, ARMS2/HTRA1, and two novel loci-were correlated with the susceptibility of nAMD and anatomical/functional responses after anti-VEGF treatment.

Association between non-steroidal anti-inflammatory drug use and development of age-related macular degeneration-A 10-year retrospective cohort study.

PURPOSE: To analyze the associations between development of age-related macular degeneration (AMD) and regular use of aspirin or non-aspirin non-steroidal anti-inflammatory drugs (NA-NSAIDs). METHODS: We retrospectively recruited individuals who received ≥28-day prescriptions of aspirin or NA-NSAIDs exclusively between 2008 and 2017 in one tertiary center as regular users. Non-regular users were free from regular use of any anti-inflammatory drugs and were matched to regular users in terms of age, sex, and visit date at a ratio of 1-4:1. The aspirin cohort included 36,771 regular users and 110,808 matched non-regular users, while the NA-NSAID cohort included 59,569 regular users and 179,732 matched non-regular users. Stratified multivariate Cox regression analyses with adjustment for systemic confounding factors were performed for the development of AMD and neovascular AMD. RESULTS: In the aspirin cohort, the adjusted hazard ratios of aspirin use for AMD in the whole cohort, individuals without cardiovascular diseases (CVDs), and those with CVDs were 0.664, 0.618, and 0.702, respectively (P < 0.0001 for all), while those of aspirin use for neovascular AMD were 0.486, 0.313, and 0.584 (P < 0.05 for all), respectively. In the NA-NSAID cohort, regular use of NA-NSAIDs was associated with a decreased risk of AMD (hazard ratio = 0.823, P < 0.0001) and neovascular AMD (hazard ratio = 0.720, P = 0.040) only in people without arthritis. CONCLUSIONS: Regular use of aspirin or NA-NSAIDs had protective effects on AMD and neovascular AMD. The effect of aspirin was observed in all patients, while the effect of NA-NSAIDs was observed only in people without arthritis.

Beneficial Effects of Fibroblast Growth Factor-1 on Retinal Pigment Epithelial Cells Exposed to High Glucose-Induced Damage: Alleviation of Oxidative Stress, Endoplasmic Reticulum Stress, and Enhancement of Autophagy.

Diabetic retinopathy (DR) severely affects vision in individuals with diabetes. High glucose (HG) induces oxidative stress in retinal cells, a key contributor to DR development. Previous studies suggest that fibroblast growth factor-1 (FGF-1) can mitigate hyperglycemia and protect tissues from HG-induced damage. However, the specific effects and mechanisms of FGF-1 on DR remain unclear. In our study, FGF-1-pretreated adult retinal pigment epithelial (ARPE)-19 cells were employed to investigate. Results indicate that FGF-1 significantly attenuated HG-induced oxidative stress, including reactive oxygen species, DNA damage, protein carbonyl content, and lipid peroxidation. FGF-1 also modulated the expression of oxidative and antioxidative enzymes. Mechanistic investigations showed that HG induced high endoplasmic reticulum (ER) stress and upregulated specific proteins associated with apoptosis. FGF-1 effectively alleviated ER stress, reduced apoptosis, and restored autophagy through the adenosine monophosphate-activated protein kinase/mammalian target of the rapamycin signaling pathway. We observed that the changes induced by HG were dose-dependently reversed by FGF-1. Higher concentrations of FGF-1 (5 and 10 ng/mL) exhibited increased effectiveness in mitigating HG-induced damage, reaching statistical significance (p < 0.05). In conclusion, our study underscores the promising potential of FGF-1 as a safeguard against DR. FGF-1 emerges as a formidable intervention, attenuating oxidative stress, ER stress, and apoptosis, while concurrently promoting autophagy. This multifaceted impact positions FGF-1 as a compelling candidate for alleviating retinal cell damage in the complex pathogenesis of DR.

Refining vitrectomy for proliferative diabetic retinopathy.

Pars plana vitrectomy (PPV) is the main treatment modality for patients with severe diabetic retinopathy. With the development of systems for microincision, wide-angle viewing, digitally assisted visualization, and intraoperative optical coherence tomography, contemporary PPV for diabetic retinopathy has been performed on a wider range of indications than previously considered. In this article, we reviewed, in conjunction with our collective experiences with Asian patients, the applications of new technologies for PPV in eyes with diabetic retinopathy and highlighted several important procedures and entities not generally reiterated in the literature, in order for vitreoretinal surgeons to optimize their approaches when facing the challenges imposed by the complications in diabetic eyes.

CLINICAL CHARACTERISTICS AND PROGNOSTIC FACTORS AFFECTING CLINICAL OUTCOMES IN CYTOMEGALOVIRUS RETINITIS WITH OR WITHOUT HIV INFECTION.

PURPOSE: To explore the clinical features and outcomes of cytomegalovirus retinitis (CMVR) in patients with HIV and non-HIV. METHODS: This retrospective cohort study included all patients with CMVR in National Taiwan University Hospital from 2013 to 2018. Demographic data, clinical characteristics, CMVR recurrence, and overall survival were compared between the HIV and non-HIV groups. Generalized estimating equation models were implemented to analyze the risk factors of poor visual prognosis. The Kaplan-Meier survival analysis was performed to investigate recurrence and survival. RESULTS: A total of 66 patients (95 eyes) with CMVR were enrolled, with no significant differences between the HIV (41 patients; 61 eyes) and non-HIV (25 patients; 34 eyes) groups in initial/final visual acuity, lesion area, or viral loads. Poor visual outcome was associated with poor initial visual acuity, retinal detachment, and a higher plasma cytomegalovirus titer. The HIV group had significantly longer survival rate ( P = 0.033) and lower recurrence rate ( P = 0.01) than the non-HIV group, and it also presented with better prognosis in recurrence-free survival analysis ( P = 0.01). CONCLUSION: Patients with CMVR without HIV had higher mortality and recurrence rates than the HIV group. Risk factors of poor visual outcome included poor initial visual acuity, retinal detachment, and a high plasma cytomegalovirus titer.

RATE OF AND TIME TO COMPLETE RETINAL VASCULARIZATION IN PREMATURE INFANTS AND ASSOCIATED FACTORS.

PURPOSE: To evaluate the rate of and time to complete vascularization in premature infants and to explore associated factors. METHODS: A monocentric, retrospective cohort study including 541 premature infants who underwent screening for retinopathy of prematurity (ROP) between July 2016 and June 2019. Patients underwent regular dilated fundus examinations with indirect ophthalmoscopy until complete vascularization. The worse eye of each patient was included for analyses. The proportion of infants with complete retinal vascularization at the last visit and the time to full vascularization was analyzed. RESULTS: Among all infants (average gestational age 31.29 ± 3.12 weeks), 490 (90.57%) had complete records of retinal vascularization outcomes, of whom 439 (89.59%) achieved complete vascularization. The average postmenstrual age for complete vascularization was 45.39 ± 11.04 weeks, and 95.22% achieved completion before 64 weeks of postmenstrual age. Retinopathy of prematurity developed in 118 (22.56%) infants; 33 (6.10%) received antivascular endothelial growth factor treatment. For all infants screened for ROP, lower birth weight, presence of ROP, and antivascular endothelial growth factor therapy predicted delayed complete vascularization; for infants diagnosed with ROP, only lower birth weight predicted delayed complete vascularization. Subgroup analysis showed significant differences between patients without ROP, with untreated ROP, and with treated ROP in time to complete vascularization and its rate (99.7%, 66.2%, and 16.7%, respectively). CONCLUSION: Lower birth weight predicted delayed complete vascularization. Antivascular endothelial growth factor therapy and the presence of ROP, including ROP severity, may also affect time to complete vascularization. These findings should help improve the understanding and management of persistent avascular retina in preterm infants.

Multimodel Imaging Evidence of Traction Component in Lamellar Macular Hole with Epiretinal Proliferation.

INTRODUCTION: The aim of this study was to investigate the association of epiretinal traction in idiopathic lamellar macular hole (LMH) with or without lamellar hole-associated epiretinal proliferation (LHEP). METHODS: A retrospective consecutive case series included 108 eyes diagnosed with LMH in a single tertiary referral center. Epiretinal traction was determined by the presence of epiretinal membrane (ERM), attached posterior hyaloid, or vascular traction with multimodal imaging studies and intraoperative findings in those received surgical interventions. RESULTS: The 53 LMHs with LHEP had similar age, refraction, initial, and final visual acuity to the 55 LMHs without LHEP. Both groups exhibited high incidences of vascular traction (with and without LHEP: 92% and 84%, p = 0.36, respectively) and ERM and/or attached posterior hyaloid (both 100%, p = 1.00). The vision improved 10.5 and 14 ETDRS letters (p = 0.60) in the 30 eyes with and 19 eyes without LHEP that underwent vitrectomy. Vascular tractions released postoperatively in 88% and 100% of LMHs with and without LHEP, respectively (p = 0.27). The LMH, ERM foveoschisis, and mixed subtypes exhibited epiretinal traction in 100% of cases in all subtypes (p = 1.00). CONCLUSION: Our findings indicated that epiretinal traction, evaluated by multimodal imaging, is the norm rather than the exception in LMHs showing LHEP. The presence of tractional forces should be taken into consideration when treatment was planned in LMHs.

Dilated choroidal vascular channels in pachychoroid neovasculopathy.

BACKGROUND: To elucidate the pattern of the choroidal vasculature in exudative pachychoroid neovasculopathy (PNV) and its correlation with the clinical course and treatment outcomes. METHODS: The retrospective study included consecutive patients in National Taiwan University Hospital between 2014 and 2020 who fulfilled the criteria for exudative PNV defined as active type 1 macular neovascularization (MNV) on optical coherence tomography angiography (OCTA) and with leakage on fluorescein angiography (FA) or indocyanine green angiography (ICGA) associated with pachychoroid features. The corrected distance visual acuity (CDVA), FA, ICGA, and OCT images obtained by Optovue (Optovue Inc, Freemont CA, USA) spectral domain OCT were evaluated at baseline and various time points during the 12-month treatment period. The correlations between the choroidal vascular patterns, specifically those with or without dilated choroidal vascular channels (DCVC) revealed by ICGA, and baseline characteristics and treatment outcomes were evaluated using multiple regression models. RESULTS: The study enrolled 34 eyes of 31 patients. The average age was 59.0 ± 9.3 years, and 20 participants were men. ICGA revealed DCVCs in 21 eyes, while the remaining 13 eyes did not have DCVCs. At baseline, DCVC group was older (p = 0.03) and had a longer duration of visual symptoms (p = 0.02), with a higher vessel density (defined as the percentage of the measured area occupied by flow area) of MNV (p = 0.04), higher proportion of ellipsoid zone disruption (p = 0.01), and poorer CDVA (p = 0.03). After the 12-month treatment period, the frequency of requirement of anti-VEGF injections (p < 0.01) was higher, and the risk for CDVA <20/40 was higher (adjusted OR: 5.29, 95% CI: 1.24-22.48, p = 0.02) in eyes with DCVCs. CONCLUSIONS: For PNV, eyes with DCVCs were associated with higher vessel density of macular neovascularization and poorer CDVA at baseline, and had poorer visual and anatomical outcomes although more anti-VEGF injections were given.

Characteristics and outcomes of full-thickness macular holes created during vitrectomy for proliferative diabetic retinopathy.

PURPOSE: To investigate the clinical features, management, and prognosis of full-thickness macular holes (FTMHs) inadvertently created during vitrectomy for eyes with proliferative diabetic retinopathy (PDR) and fibrovascular proliferation (FVP). METHODS: Eyes with PDR and FVP that had intraoperatively created FTMHs were retrospectively collected as the study group, and age- and sex-matched subjects with PDR and FVP who did not have intraoperative FTMHs were selected as the control group. Fundus abnormalities, optical coherence tomography (OCT) features, and anatomical and functional outcomes were compared between the two groups. RESULTS: Eleven eyes of 11 patients (5 male and 6 female) were identified as the study group. Follow-up duration was 36.8 ± 47.2 months. FTMHs were managed by ILM peeling or the inverted ILM flap technique. Anatomical success and MH closure were achieved in 100% of eyes in the study group. In comparison to the control group, the study group had a higher proportion of condensed prefoveal tissue (63.6% vs. 22.7%, p = 0.028), and a higher ratio of silicone oil tamponade (63.6% vs. 18.2%, p = 0.014), whereas there were no differences in preoperative and final BCVA, and the severity, activity, and locations of FVP between the two groups. CONCLUSION: Condensed prefoveal tissue was a risk factor of FTMHs created during operation for eyes with PDR and FVP. The ILM peeling or the inverted ILM flap technique may be beneficial for the treatment with favorable anatomical and functional outcomes.

Combined fovea-sparing internal limiting membrane peeling with internal limiting membrane flap technique for progressive myopic traction maculopathy.

BACKGROUND: To present the anatomical and functional outcomes of combined fovea-sparing internal limiting membrane peeling (FSIP) with internal limiting membrane flap (ILMF) for myopic traction maculopathy (MTM). METHODS: This is a retrospective, observational study. Included were 66 eyes of 62 patients who underwent vitrectomy with combined FSIP and ILMF (or modified ILMF) for MTM with a minimal follow-up of 6 months. Thirty-one eyes were treated with FSIP, and 35 with modified ILMF. RESULTS: The post-operative best-corrected visual acuity (BCVA) improved from 20/148 to 20/87 in the FSIP group (p < 0.001), and from 20/121 to 20/66 in the modified ILMF group (p < 0.001). The post-operative macular thickness (MT) reduced after FSIP (from 739.58 to 223.81 µm, p < 0.001) and modified ILMF (from 706.43 to 236.59 µm, p < .001). The degree of the improvement of BCVA and MT in both groups was insignificant. The incidence of a post-operative macular hole (MH) was 9.7% (3/31 eyes) with FSIP and 0% (0/35 eyes) with modified ILMF. All patients with a MH had foveoschisis in association with a lamellar hole (LMH) pre-operatively. After controlling the surgical technique, pre- and post-operative MT, follow-up duration, myopic atrophy maculopathy, and FD, the surgical technique showed significant effects on post-operative MH in all cases (p = 0.022) and in those with pre-operative LMH (p = 0.029). CONCLUSION: Our pilot study showed both methods result in significant anatomical and functional improvement. The combined FSIP with ILMF method may prevent post-operative macular hole development in cases with MTM and LMH.

Quantitative analysis of branching neovascular networks in polypoidal choroidal vasculopathy by optical coherence tomography angiography after photodynamic therapy and anti-vascular endothelial growth factor combination therapy.

PURPOSE: To study serial changes in branching neovascular networks (BNN) by using optical coherence tomography angiography (OCTA) in patients with polypoidal choroidal vasculopathy (PCV) who underwent combined photodynamic therapy (PDT) and anti-vascular endothelial growth factor (anti-VEGF) therapy. METHODS: In this retrospective study of 30 PCV patients who underwent combined therapy, OCTA images obtained at baseline and 1, 3, and 6 months after treatment were collected. The vessel area, vessel percentage area, average vessel length, and presence of polypoidal lesions on OCTA images as well as best-corrected visual acuity (BCVA), central retinal thickness (CRT), and central choroidal thickness (CCT) were recorded at each time point. RESULTS: The BNN- and polypoidal lesion-detection rates on baseline OCTA images were 100% and 71%, respectively. The vessel area decreased during the first 3 months, and increased 6 months post-treatment, showing significant differences from baseline (p = 0.031). The vessel percentage area also reduced 1 and 3 months post-treatment (p = 0.025) and increased 6 months post-treatment. Continuous polypoidal lesion regression was observed from 1 to 3 and 6 months post-treatment (p = 0.031, p = 0.004, p = 0.002, respectively, in comparison with baseline). Patients with a decreasing vessel area over 6 months showed greater choroidal thickness than those with increasing vessel area (p = 0.004). CONCLUSIONS: The BNN showed initial regression but were enlarged at 6 months after therapy. Patients showing continuous BNN regression showed a thicker choroid at baseline. This difference should be considered during treatment for PCV, and OCTA could be used for follow-up evaluations of PCV patients.

HYPERREFLECTIVE FOCI AS IMPORTANT PROGNOSTIC INDICATORS OF PROGRESSION OF RETINITIS PIGMENTOSA.

PURPOSE: To investigate the presence and clinical relevance of hyperreflective foci (HRFs) in retinitis pigmentosa. METHODS: Seventy seven retinitis pigmentosa cases were retrospectively reviewed. The 10-mm wide cross-line macular scans in optical coherence tomography were acquired. Hyperreflective foci were classified according to the location in optical coherence tomography: outer layers within the macula (HRF-outer-central), macular border beyond the central 3 mm (HRF-outer-perifoveal), and choroid (HRF-choroidal). The visual acuity at baseline, at 12 months, and other fundus characteristics were collected. RESULTS: The mean logMAR best-corrected visual acuity decreased from 0.59 ± 0.66 (20/78 in Snellen) to 0.74 ± 0.81 (20/106 in Snellen) in 1 year. Sixty-six (42.9%), 105 (68.2%), and 98 (63.6%) eyes were classified to HRF-outer-central, HRF-outer-perifoveal, and HRF-choroidal group, respectively. Hyperreflective foci were positively correlated with poorer vision, central macular thinning, and ellipsoid zone disruption (all P < 0.001). Worse vision was associated with older age, macular involvement, and the coexistence of two or three HRF groups (P = 0.014, 0.047, 0.019, <0.001, respectively). Hyperreflective foci developed more frequently in patients with thick choroid than in those with thin choroid. The coexistence of three HRF groups was correlated with quicker visual deterioration (P = 0.034). CONCLUSION: Hyperreflective foci are common in retinitis pigmentosa and can be a negative prognostic indicator of macular thickness and visual preservation. Thick choroid was associated with all groups of HRFs, especially HRF-choroidal.

PREDICTED PROTEIN STRUCTURE VARIATIONS INDICATE THE CLINICAL PRESENTATION OF CYP4V2-RELATED BIETTI CRYSTALLINE DYSTROPHY.

PURPOSE: To investigate the relationship between different CYP4V2 disease-causing variants and disease severity in Bietti crystalline dystrophy (BCD). METHODS: Twenty-one subjects from 19 unrelated families with a clinical diagnosis of BCD were enrolled. A novel severity prediction score for BCD based on the predicted molecular impact of CYP4V2 variants was applied for grouping and subsequent analyses. The more severe variants led to less CYP4V2 protein function preservation and a higher severity prediction score. RESULTS: All subjects harbored two alleles of CYP4V2 disease-causing variants, of which c.802-8_810del17insGC was the most prevalent (14/21, 66.67%) and c.1507G>C was novel. According to the severity score, the subjects were categorized into severe, moderate, and mild groups with different preservation of central vision (mean logMAR visual acuity 0.95 ± 0.82, 0.89 ± 1.22, and 0.56 ± 0.64, respectively). The patients with a lower severity score had slower disease progression. CONCLUSION: This is the first cohort study of BCD in Taiwan, and we established a novel BCD severity index based on the molecular impact of different CYP4V2 variants. More severe impairment of CYP4V2 protein led to a more severe disease course with earlier progression. Our results could be helpful in identifying a therapeutic window for patients with BCD.

Formation and evolution of idiopathic lamellar macular hole-a pilot study.

BACKGROUND: The developmental pathways and subsequent evolutional processes of idiopathic lamellar macular hole (LMH) were studied with spectrum domain optical coherence tomography (SD-OCT). METHODS: Twenty-seven eyes of 26 patients of idiopathic LMH with pre-LMH SD-OCT available were retrospectively reviewed. Relevant OCT parameters and best-corrected visual acuity (BCVA) were collected and analyzed. RESULTS: Four types of developmental pathways of idiopathic LMH were noted. Type 1 (5 cases), involved disruption of a foveal cyst from vitreomacular traction. Type 2 (10 cases), demonstrated rupture of parafoveal cysts or schisis mainly from epiretinal membrane (ERM). In type 3 pathway (5 cases), a central intraretinal cyst formed under tight ERM with subsequent cyst roof dehiscence. Type 4 (7 cases), showed gradual loss of foveal tissue without cystic lesions from ERM traction. There was no statistically significant change in BCVA during LMH formations or subsequent evolutional processes in any types of the developmental pathways. Three cases developed epiretinal proliferation (EP) during evolution, which showed tendency of decrease in BCVA. Among the three cases, one later developed the degenerative configuration. CONCLUSIONS: In summary, four types of tractional developmental pathways of idiopathic LMH were identified. BCVA was relatively stable during LMH formation and follow-up. Deterioration of visual acuity were found in cases that developed EP during evolution. Transformation into degenerative configuration might be possible after LMH formation.

Intravitreal gas injection for early persistent macular hole after primary pars plana vitrectomy.

PURPOSE: To report the clinical presentations and outcome of early intravitreal injection (IVI) of octafluoropropane (C3F8) for persistent macular holes (MH) after primary pars plana vitrectomy with the internal limiting membrane (ILM) peeling technique. METHODS: Nineteen eyes of 18 patients with persistent MH after vitrectomy underwent intravitreal injection of C3F8 between 11 and 21 days after the initial surgery (intravitreal gas injection group). Another nine eyes with a persistent MH without additional IVI C3F8 were included (non-intravitreal gas injection group). Best-corrected visual acuity (BCVA), optical coherence tomography (OCT) features including size and configuration of MH, and time duration between the 2 surgeries were compared between the MH closure and open groups. The closure rate of persistent MHs was compared between the intravitreal gas injection group and non-intravitreal gas injection group. RESULTS: Twelve of 19 eyes (63%) achieved MH closure after 1 to 3 times IVI C3F8. The final BCVA after vitrectomy and IVI gas was significantly better in the MH closure group (P = .005). Nine of 12 patients (75%) in the MH closure group had a visual acuity improvement of more than 2 lines. Original MHs with smaller minimal diameter, higher macular hole index (MHI) and higher tractional hole index (THI); and persistent MHs with smaller minimal diameter, higher THI, and lower diameter hole index (DHI) showed higher MH closure rate. None of the persistent MHs closed in the non-intravitreal gas injection group (0/9 eyes). CONCLUSION: Early intravitreal injection of C3F8 can be a cost-effective first-line treatment for early persistent MHs after primary surgery, especially in eyes with favorable OCT features.

Optimal approaches and criteria to treat-and-extend regimen implementation for Neovascular age-related macular degeneration: experts consensus in Taiwan.

The management of neovascular age-related macular degeneration (nAMD) has taken a major stride forward with the advent of anti-VEGF agents. The treat-and-extend (T&E) approach is a refined management strategy, tailoring to the individual patient's disease course and treatment outcome. To provide guidance to implementing anti-VEGF T&E regimens for nAMD in resource-limited health care systems, an advisory board was held to discuss and generate expert consensus, based on local and international guidelines, current evidence, as well as local experience and reimbursement policies. In the experts' opinion, treatment of nAMD should aim to maximize and maintain visual acuity benefits while minimizing treatment burden. Based on current evidence, treatment could be initiated with 3 consecutive monthly injections. After the initial period, treatment interval may be extended by 2 or 4 weeks each time for the qualified patients (i.e. no BCVA loss ≥5 ETDRS letters and dry retina), and a maximum interval of 16 weeks is permitted. For patients meeting the shortening criteria (i.e. any increased fluid with BCVA loss ≥5 ETDRS letters, or presence of new macular hemorrhage or new neovascularization), the treatment interval should be reduced by 2 or 4 weeks each time, with a minimal interval of 4 weeks. Discontinuation of anti-VEGF may be considered for those who have received 2-3 consecutive injections spaced 16 weeks apart and present with stable disease. For these individuals, regular monitoring (e.g. 3-4 months) is recommended and monthly injections should be reinstated upon signs of disease recurrence.

Idiopathic Macular Hole without Vitreomacular Separation.

PURPOSE: This study aimed to investigate the clinical features and treatment outcomes of idiopathic full-thickness macular hole (FTMH) without vitreomacular separation (VMS). METHODS: Consecutive cases of idiopathic FTMH at one tertiary center from January 2013 to April 2020 were retrospectively recruited. They were separated into two groups according to the findings in optical coherence tomography (OCT): FTMH with VMS and FTMH without VMS. Ophthalmic examinations and OCT were performed pre- and postoperatively. The clinical findings were compared between the two groups. RESULTS: Of the total 124 cases, 15 (12.1%) were noted as FTMH without VMS with the presence of an attached posterior hyaloid (PH) at macula. The macular hole (MH) size was smaller (276.06 ± 170.10 µm) compared to those with VMS (492.83 ± 209.31 µm) (p < 0.001). The incidence of lamellar hole-associated epiretinal proliferation (LHEP) was much higher in this group (13/15, 86.7%) compared to FTMH with VMS (11/109, 10.1%) (p < 0.001). A higher rate of spontaneous closure of MH (13.3%) was also noted in FMTH without VMS (13.3% vs. 0.9% in FTMH with VMS, p = 0.040). After operation, the MH closure rate was 93.3%. The postoperative best-corrected visual acuity was not significantly different between the two groups (p = 0.098). CONCLUSIONS: A small percentage (12.1% in this series) of idiopathic FTMH had no VMS. The completely attached PH along with the high incidence of LHEP implied a tangential traction in FTMH without VMS. The MH size was usually small, and the postoperative outcomes were similar to those of conventional FTMH with VMS.