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This study aimed to investigate the heritability of genetic influence on macular choroidal vascularity index (CVI). This was a cross-sectional healthy twin study. We included 373 Korean adults with healthy eyes from 79 families. CVI, Total choroidal area (TCA) and luminal area (LA) were measured using horizontal scan of spectral-domain optical coherence tomography with enhanced depth imaging. Characteristics of the participants were investigated (age, gender, drinking habits, smoking status, axial length, hypertension, and diabetes). The correlations of these characteristics with CVI were assessed using univariate and subsequently multivariate regression models. Participants who were older and had a higher intraocular pressure, higher diastolic blood pressure, and lower pulse pressure showed associations with lower CVI (p < 0.001, p = 0.014, p = 0.005, and p = 0.015, respectively). The covariate-adjusted heritability (±standard error) of the CVI was 0.716 ± 0.091, and the heritabilities of the TCA and LA were 0.691 ± 0.089 and 0.634 ± 0.100, respectively. The CVI, TCA, and LA are highly heritable.

This study aimed to investigate the change of choroidal venous overload in Vogt‒Koyanagi‒Harada (VKH) disease. Clinical records of 52 patients with VKH disease (52 eyes) and 24 control subjects (24 eyes) who underwent multimodal imaging, including fluorescein angiography (FA) and indocyanine green angiography (ICGA), were retrospectively reviewed. Imaging data were assessed for signs associated with choroidal venous overload, e.g., choroidal perfusion delay, choroidal vascular hyperpermeability, dilated choroidal veins, and intervortex venous anastomosis (IVA). Dual FA and ICGA scoring for active posterior segment inflammation was performed. Clinical and imaging features associated with choroidal venous overload were compared between early- and late-stage VKH disease. Choroidal perfusion delay, choroidal vascular hyperpermeability, dilated choroidal veins, and IVA were more prevalent in eyes with VKH disease (69.2%, 67.3%, 61.5%, and 65.4%, respectively) than in control eyes (25.0%, 20.8%, 25.0%, and 37.5%, respectively) (p < 0.05). All eyes with IVA in the early-stage of VKH disease had got other 3 signs. All choroidal venous overload signs were more prevalent in patients with early-stage (20 eyes) than in those with late-stage VKH disease (32 eyes) (p < 0.05). The number of choroidal venous overload signs were inversely related to disease duration (p < 0.001) and proportionally related to the total ICGA score (p < 0.001). IVA was significantly associated with the total ICGA score in logistic regression (p = 0.014). Choroidal venous overload occurs early in VKH disease. Angiographic signs of choroidal venous overload may be useful markers to assess the status of VKH disease.

This study aimed to investigate the longitudinal change in the reticular pseudodrusen (RPD) area in the fundus and its association with late age-related macular degeneration (AMD). 91 RPD eyes (55 patients; age 67.9 ± 7.3 years) with > 5 years’ follow-up (6.8 ± 0.9 years) from a single medical center were enrolled. Ultrawide-field photography images were analyzed using the concentric rings method, and the RPD area was semi-quantitatively classified according to the affected segment number into central, intermediate, and extensive types. Correlations of longitudinal changes in the RPD area and late AMD risk were investigated. RPD area increased significantly during the follow-up (p .048), RPD area at first visit (r = − 0.222; p = 0.035), and the decrease rate of subfoveal choroidal thickness (SFCT) (r = 0.217; p = 0.039). Many central (18/49, 36.7%) and intermediate (15/23, 65.2%) types switched to the more advanced type during the follow-up. Macular neovascularization and geographic atrophy developed in 12.3% and 18.7% of patients by 7 years. Late AMD incidence was significantly higher in eyes with large than in those with small RPD areas (p = 0.002). Larger RPD area at baseline, faster increase in RPD area, thinner SFCT, rapid decrease in SFCT, and the presence of late AMD on fellow eye were associated with late AMD. All RPD areas progressively increase over time. The regular assessment of RPD area may help to predict late AMD risk in RPD eyes.

To evaluate the degree of genetic influence on macular choroidal volume.A cross-sectional twin and family study.In total, 353 Korean adults with healthy eyes from 78 households with 2 or more family members were included in the study. Macular choroidal volume was measured using spectral-domain optical coherence tomography with enhanced depth imaging at 9 macular subfields defined by the ETDRS. Demographics and clinical characteristics were investigated, including age, sex, axial length, hypertension, diabetes, drinking habits, and smoking status. The associations of these factors with macular choroidal volume were assessed using univariate and subsequent multivariate regression analyses while accounting for family structure. The heritability estimates of macular choroidal volume in total and at each of the 9 macular subfields were calculated after adjusting the covariates.Patients who were younger, male, and had a shorter axial length showed associations with greater choroidal volume (P.001 for all 3 independent variables). The covariates-adjusted heritability (±standard error) of the total macular choroidal volume was 0.76 ± 0.06, and the heritabilities of choroidal volume at each subfield ranged from 0.55 ± 0.09 (inner temporal subfield) to 0.77 ± 0.08 (inner superior subfield).The macular choroidal volume is highly heritable.

PurposeTo evaluate genetic influence on macular ganglion cell inner plexiform layer (GCIPL) thickness.MethodsMacular GCIPL thickness was measured with optical coherence tomography in nine macular subfields defined by the E TDRS. Intraclass correlation coefficients (ICC) of GCIPL thickness by different types of family relationships were estimated to assess intrafamilial resemblance. Then, heritability of GCIPL thickness was estimated.ResultsThree hundred and sixty-one Korean adults from 89 families with normal healthy eyes were included. GCIPL thickness was highest in inner subfields and lowest in fovea. Monozygotic twin pairs showed significantly higher ICCs of GCIPL thickness in all subfields compared to those in parent–offspring pairs and sibling pairs. GCIPL thickness was highly heritable in the centre (0.71) and outer subfields but moderate to highly heritable in inner subfields. Heritability of GCIPL thickness in outer subfields was 0.69, 0.67, 0.72 and 0.68 for superior, inferior, temporal and nasal fields, respectively. Heritability of GCIPL thickness in inner subfields was 0.55, 0.56, 0.75 and 050 for superior, inferior, temporal and nasal subfields, respectively.ConclusionMacular GCIPL thickness is significantly influenced by genetic factors. It varies according to subfields with moderate to high heritability in all subfields.

We aimed to evaluate the association between obesity and the incidence of retinal vein occlusion (RVO) with and without diabetes mellitus (DM).This is a retrospective cohort study using Korean National Health Insurance System data. The participants were 23,061,531 adults older than 20 years who received a health examination at least once between 2009 and 2012, and all patients were observed for RVO development until 2015. We used a multivariate adjusted Cox regression analysis to evaluate the association between RVO and body mass index (BMI) with and without DM. The analysis were evaluated via a hazard ratio (HR) and 95% confidence interval (CI). The age-, sex-, and multivariable-adjusted HRs for RVO were stratified by BMI. This population-based study revealed evidence that obesity has a different effect on the incidence of RVO in the presence and absence of DM.In people with DM, a lower BMI was associated with an increased risk of RVO, and a higher BMI was associated with a lower risk for RVO. In people without DM, the correlation was reversed: a lower BMI was associated with a lower risk for RVO and vice versa.

The purpose of the study is to evaluate the heritability of inner retinal layer (IRL) and outer retinal layer (ORL) thicknesses in the healthy Korean population. This was a cross-sectional, twin and family study. We included 374 Korean adults with healthy eyes from 89 families. IRL thickness (from the internal limiting membrane to the external limiting membrane) and ORL thickness (from the external limiting membrane to the outer border of the retinal pigment epithelium layer) were measured in the nine macular subfields as defined by the Early Treatment of Diabetic Retinopathy Study using optical coherence tomography. The heritability on IRL and ORL thicknesses were investigated using a variance decomposition model. The heritability of IRL thickness was 0.87, 0.58, 0.85, 0.89, and 0.74 for the central, inner superior, inner inferior, inner temporal, inner nasal subfields, respectively; and 0.62, 0.83, 0.62, and 0.60 for the outer superior, outer inferior, outer temporal, outer nasal subfields, respectively. The heritability of ORL thickness was 0.56, 0.75, 0.66, 0.72, and 0.56 for the central, inner superior, inner inferior, inner temporal, inner nasal subfields, respectively; and 0.64, 0.63, 0.73, 0.54 for the outer superior, outer inferior, outer temporal, and outer nasal subfields, respectively. The heritability estimates of IRL thickness and ORL thickness ranged from moderate to high. The IRL thickness at the central, inner temporal, and inner inferior subfields had particularly high heritability.

This study aimed to describe the clinical characteristics of age-related macular degeneration (AMD) eyes with both cuticular drusen (CD) and reticular pseudodrusen (RPD). Clinical records of patients diagnosed with CD or RPD with multimodal imaging was reviewed for patients diagnosed with both CD and RPD. The distribution patterns of CD (macular and diffuse type) and RPD (localized, intermediate, and diffuse type), presence of soft drusen, large drusen (> 200 µm), variant subretinal drusenoid deposits, and macular complications were investigated. Of the 220 eyes of 110 patients diagnosed with CD and 926 eyes of 463 patients diagnosed with RPD, 13 eyes of seven patients met the diagnostic criteria for both CD and RPD. The mean age at initial presentation was 71.4 ± 8.8 years and six patients were female. The mean subfoveal choroidal thickness was 143.8 ± 25.1 µm. The distribution of CD was of the macular type in all eyes. Distribution of RPD was localized in 11 eyes (84.6%) and intermediate in two eyes (15.4%). Soft drusen, large drusen, and variant subretinal drusenoid deposits were present in 13 (100%), 12 (92.3%) and, seven (53.8%) eyes, respectively. Macular neovascularization was observed in two eyes (15.4%). CD and RPD can coexist in eyes with AMD. Multimodal imaging should be used for AMD eyes with features suggestive of CD and RPD, considering the high likelihood of developing late AMD.

Purpose To investigate association between the development of retinal vein occlusion (RVO) and blood high-density lipoprotein cholesterol (HDL-C). Design A retrospective, nationwide, population-based cohort study. Methods This study was set in the Republic of Korea and included 23,149,403 people ≥20 years of age who underwent the Korean National Health Screening Program examination between January 2009 and December 2012. Among them, the RVO group was composed of patients with an initial diagnosis of RVO made between 2009 and 2015 (n = 117,639). The earliest claim with an RVO diagnostic code was considered as the incident time. The predictive value of HDL-C level for RVO was analyzed using hazard ratios. The primary outcome measure was the incident cases of RVO. Results Subjects with RVO were generally older; had high body mass index, waist circumference, fasting blood glucose, blood pressure, total and low-density lipoprotein cholesterol, and triglyceride values, and low glomerular filtration rate and HDL-C values; and were more likely to experience diabetes mellitus and hypertension compared with the non-RVO group. The fully adjusted hazard ratio of RVO was 1.12 (95% confidence interval 1.10-1.14) in the lowest quartile of HDL-C versus in the highest quartile. The association between the development of RVO and HDL-C was higher those with a younger age, male sex, current smoking habit, diabetes mellitus, and hypercholesterolemia. In addition, we observed a significant synergistic effect of low HDL-C level with obesity and hypertension. Conclusion This is the first nationwide population-based epidemiologic study evaluating the association between HDL-C level and the risk of RVO development. A significant association between low HDL-C and RVO development was found.

Cuticular drusen show some similarities to and differences from soft drusen in age-related macular degeneration (AMD) and might thus be a unique AMD subtype. Previous studies on cuticular drusen were performed mainly in white ethnic groups, but AMD shows ethnic differences. We investigated clinical manifestations of cuticular drusen in Korean patients to evaluate possible ethnic differences. Clinical records of Korean patients with cuticular drusen were retrospectively reviewed. Fundus distribution pattern, imaging features, and presence of large drusen, drusenoid pigment epithelial detachment (PED), and macular complications, including geographic atrophy (GA), choroidal neovascularization (CNV), and acquired vitelliform lesion (AVL), were assessed via multimodal imaging in 162 eyes with cuticular drusen (n = 81 patients; 67 females; mean age: 66.6 ± 9.1 years). Diffuse distribution was found in 61.7% and peripapillary involvement in 75.3% of eyes. Large drusen, drusenoid PED, GA, CNV, and AVL were observed in 59.3%, 26.5%, 18.5%, 3.7%, and 1.2% of eyes, respectively. The macular complication prevalence was similar between patients ≤ 60 and those > 60 years old. In Korean patients, cuticular drusen were less frequently associated with macular complications than in white patients, and the proportion of macular complications differed significantly, with AVL representing an uncommon complication.

PURPOSE To evaluate changes in the peripapillary retinal nerve fiber layer (RNFL) thicknesses using spectral-domain optical coherence tomography (SD-OCT) in hydroxychloroquine (HCQ) users. METHODS The medical records of HCQ users were retrospectively reviewed. In these HCQ users, an automated perimetry, fundus autofluorescence photography, and SD-OCT with peripapillary RNFL thickness measurements were performed. The peripapillary RNFL thicknesses were compared between the HCQ users and the control groups. The relationships between the RNFL thicknesses and the duration or cumulative dosage of HCQ use were analyzed. RESULTS This study included 77 HCQ users and 20 normal controls. The mean duration of HCQ usage was 63.6 ± 38.4 months, and the cumulative dose of HCQ was 528.1 ± 3.44 g. Six patients developed HCQ retinopathy. Global and six sectoral RNFL thicknesses of the HCQ users did not significantly decrease compared to those of the normal controls. No significant correlation was found between the RNFL thickness and the duration of use or cumulative dose. The eyes of those with HCQ retinopathy had temporal peripapillary RNFL thicknesses significantly greater than that of normal controls. CONCLUSIONS The peripapillary RNFL thicknesses did not change in the HCQ users and did not correlate with the duration of HCQ use or cumulative doses of HCQ. RNFL thickness is not a useful biomarker for the early detection of HCQ retinal toxicity.

Background/aimsTo investigate the prevalence of abnormal central and peripheral ultra-wide-field (UWF) angiography findings, and their association with clinical features in acute Vogt-Koyanagi-Harada (VKH) disease.MethodsThis retrospective, observational study included 26 eyes of 13 treatment-naïve patients with acute VKH disease who underwent UWF fluorescein angiography (FA). Sixteen eyes of eight patients also underwent UWF indocyanine green angiography (ICGA). A circle simulating the central 75° field was used to divide the acquired image into the central fundus area (CFA) and peripheral fundus area (PFA), in which the presence of six previously reported abnormal angiographic findings were analysed. Correlations between abnormal angiography findings in FA and clinical features were also investigated.ResultsAll eyes demonstrated more than one abnormal angiographic finding in both the CFA and PFA. UWF FA revealed three abnormal findings in the CFA versus the PFA: focal leakage (92.3% vs 76.9%); pooling with a dark rim (84.6% vs 53.8%); and retinal vascular leakage (0% vs 46.2%). UWF ICGA revealed three abnormal findings in the CFA versus the PFA: hypofluorescent dark dots (100% vs 100%); diffusely leaking fuzzy choroidal vessels (93.8% vs 75.0%); and late hypofluorescent patches (81.3% vs 31.3%). Pooling with a dark rim and retinal vascular leakage in the PFA were significantly associated with low initial visual acuity (p=0.03) and subfoveal choroidal thickness change ratio (p=0.04), respectively.ConclusionAbnormal UWF angiography findings were frequently detected in the CFA and PFA. Such findings may be useful in evaluation and monitoring of VKH disease.

PURPOSE To investigate the anatomical and refractive outcomes in patients with Type 1 retinopathy of prematurity in Zone I. METHODS The medical records of 101 eyes of 51 consecutive infants with Type 1 retinopathy of prematurity in Zone I were analyzed. Infants were treated by conventional laser photocoagulation (Group I), combined intravitreal bevacizumab injection and Zone I sparing laser (Group II), or intravitreal bevacizumab with deferred laser treatment (Group III). The proportion of unfavorable anatomical outcomes including retinal fold, disc dragging, retrolental tissue obscuring the view of the posterior pole, retinal detachment, and early refractive errors were compared among the three groups. RESULTS The mean gestational age at birth and the birth weight of all 51 infants were 24.3 ± 1.1 weeks and 646 ± 143 g, respectively. In Group I, an unfavorable anatomical outcome was observed in 10 of 44 eyes (22.7%). In contrast, in Groups II and III, all eyes showed favorable anatomical outcomes without reactivation or retreatment. The refractive error was less myopic in Group III than in Groups I and II (spherical equivalent of -4.62 ± 4.00 D in Group I, -5.53 ± 2.21 D in Group II, and -1.40 ± 2.19 D in Group III; P < 0.001). CONCLUSION In Type 1 retinopathy of prematurity in Zone I, intravitreal bevacizumab with concomitant or deferred laser therapy yielded a better anatomical outcome than conventional laser therapy alone. Moreover, intravitreal bevacizumab with deferred laser treatment resulted in less myopic refractive error.

This study aimed to evaluate the frequency and clinical characteristics of hydroxychloroquine (HCQ) retinopathy in Korean patients with rheumatologic diseases. We retrospectively reviewed medical records of 310 patients taking HCQ. Ophthalmic examinations included spectral-domain optical coherence tomography (SD-OCT), automated visual field test, and fundus autofluorescence. The severity of retinopathy was categorized as early, moderate, or severe, and the location was categorized as parafoveal, pericentral, or mixed pattern. Among 310 patients, 9 patients (2.9%) were diagnosed as HCQ retinopathy. Among the patients with HCQ use ≥ 5 years (n = 174), the frequency was 5.2%. Only 1 (11.1%) of the 9 patients was symptomatic. The mean daily dose per kilogram of real body weight of the 9 patients was 5.6 mg, and only 3 had used 6.5 mg or more. Four of the 9 patients had severe HCQ retinopathy. Six of the 9 patients showed pericentral or mixed pattern of retinal damage. Consequently, the frequency of HCQ retinopathy in Korean patients was not low, especially when administered at a high cumulative dose and for a long duration. Screening of HCQ retinopathy by the recommended guidelines that include SD-OCT seems useful and should be done to detect retinal damage earlier in patients with chronic exposure to HCQ., Graphical Abstract

The purpose of the study is to propose choroidal spatial distribution indexes (CSDIs) to represent choroidal topographic features, establish a normative database for CSDIs, and identify factors associated with CSDIs in healthy eyes. Retrospective data analysis of 363 healthy eyes from a single-center, prospective, cross-sectional, non-interventional study. Subjects were evaluated using spectral domain OCT with enhanced depth imaging. Choroidal volume and average thickness were measured with OCT in nine macular subfields defined by the Early Treatment Diabetic Retinopathy Study. Vertical CSDI was defined as the natural logarithm of superior choroidal volume divided by inferior choroidal volume. Horizontal CSDI was defined as the natural logarithm of temporal choroidal volume divided by nasal choroidal volume. The overall distributions of vertical and horizontal CSDIs was analyzed. Linear regression analyses were used to identify ocular and systemic factors associated with vertical and horizontal CSDIs. The average vertical CSDI was 0.062 ± 0.206, and average horizontal CSDI was 0.138 ± 0.226. Both vertical and horizontal CSDIs followed normal distribution. Increasing age was significantly associated with greater vertical CSDI (choroidal volume distribution tilted toward the superior region), and longer axial length and thinner subfoveal choroidal thickness were significantly associated with greater horizontal CSDI (choroidal volume distribution tilted toward the temporal region).

Background/aimCharacterising genetic effect on macular retinal nerve fibre layer (RNFL) is needed to obtain better understanding of various retinopathies and optic neuropathies. The purpose of this study was to evaluate genetic influence on macular RNFL thickness.MethodsThis is a cross-sectional, twin and family study. Three hundred and sixty-two Korean adults with healthy eyes were included in the study from 79 households with two or more family members. Macular RNFL thickness was measured with optical coherence tomography at nine macular subfields defined by the ETDRS. Intraclass correlation coefficients (ICCs) were estimated to assess intrafamilial resemblance of RNFL thickness by different types of family relationship. Heritability of RNFL thickness was evaluated using variance decomposition model.ResultsRNFL thickness increased from central subfield to outer subfields. Temporal quadrant RNFL was thinner compared with other quadrants. Monozygotic twin pairs showed the highest ICCs of RNFL thickness, although the ICC level varied across different subfields. Heritability of RNFL thickness was the highest at central subfield (0.81). RNFL thicknesses of outer subfields were moderately to highly heritable: 0.53, 0.71, 0.47 and 0.66 for superior, inferior, temporal and nasal fields, respectively. RNFL thicknesses at inner subfields showed the lowest heritability: 0.21, 0.24, 0.27 and 0.27 for superior, inferior, temporal and nasal subfields, respectively.ConclusionMacular RNFL thickness is significantly influenced by genetic factors. It varies largely by subfields with the highest heritability at the central subfield and a relatively lower heritability at inner subfields.

To investigate the error rate of automated choroidal segmentation and the effect of frame averaging on error rate.A horizontal B scan at the fovea was performed in patients having various retinochoroidal disorders using swept-source optical coherence tomography (OCT) with frame-averaging technique. Scanned images were classified into four morphological groups: normal from fellow eyes (NF ), normal from pathologic eyes (NP ), retinal abnormality (R) and retinochoroidal abnormality (RC) group. Choroidal segmentation was automatically performed using built-in software of a swept-source OCT device, and the error rate of choroidal segmentation was analysed.Qualified images for all four averaging types with different number of averaged frames were acquired in 89 eyes of 77 patients. Images of 12, 20, 24 and 33 eyes were classified as NF , NP , R and RC group, respectively. The choroidal segmentation error was detected in 1-2 images (8.3-16.7%) in the NF group, 3-6 images (15.0-30.0%) in the NP group, 4-8 images (16.7-33.3%) in the R group and 17-19 images (51.5-57.6%) in the RC group. The error rate was significantly higher in RC group than other groups (p 0.05). Increasing the number of frames for averaging showed no significant effect on the error rate in all groups (p 0.05).Automated choroidal segmentation showed a high error rate in images with choroidal abnormalities, and the averaging effect could not reduce the error rate significantly. Thus, further technological improvement is needed to increase the accuracy of the automated choroidal segmentation.

Purpose To investigate the impact of the metabolic syndrome (METS) on the incidence of retinal vein occlusion (RVO). Methods This is a retrospective cohort study using Korean National Health Insurance System data. 23,153,600 subjects without previous history of RVO underwent a National Health Screening Program examination between 2009 and 2012. They were monitored for RVO development (registration of diagnostic code for RVO) until 2015. Presence of METS was defined using the data from the National Health Screening Program examination according to the revised criteria of the National Cholesterol Education Program Adult Treatment Panel III. A multivariate adjusted Cox regression analysis was used to reveal hazard ratios and 95% confidence interval for RVO development in the presence of METS. Results The age of the subjects was 47.64 ± 13.51 years. In this cohort, 11,747,439 (50.7%) were male, 11,406,161 (49.3%) were female, and 6,398,071 subjects (27.6%) were diagnosed with METS. The overall incidence of RVO was 0.947 per 1000 person-years. The adjusted hazard ratio of RVO in the presence of METS was 1.458 (95% confidence interval, 1.440–1.475; P < 0.001) after adjusting for age, sex, smoking status, alcohol consumption, physical activity, and income. Among all of the criteria for METS diagnosis, elevated blood pressure was the greatest risk for RVO development (adjusted hazard ratio, 1.610; 95% confidence interval, 1.589–1.631; P < 0.001). Conclusions METS and each of diagnostic criteria was associated with an increased risk of RVO development. Elevated blood pressure seems to be especially important factors for RVO development. Translational Relevance Our results provide information about the link between METS and RVO.

Purpose To investigate the incidence of late age-related macular degeneration (AMD) over 3 years and risk factors for the development of late AMD in Korean patients having reticular pseudodrusen (RPD). Methods Clinical records of Korean RPD patients with no late AMD at first examination and completion of 3 years of regular follow-up were retrospectively reviewed. All patients underwent complete ocular examinations, including multimodal imaging. Reticular pseudodrusen were classified as a separate lesion different from other early AMD lesions, and RPD were not considered a sign of early AMD. Risk factors for the development of late AMD were assessed. Results One hundred and ninety-two RPD eyes of 104 patients were included in this study. Mean age of patients was 69.4 ± 8.9 years, and other early AMD lesions were accompanied in 152 eyes (79.2%) at baseline. During 3 years, late AMD occurred in 30 eyes (15.6%); geographic atrophy in 24 eyes (12.5%); and neovascular AMD in 6 eyes (3.1%). Eyes having early AMD at baseline revealed significantly higher incidence for late AMD than those eyes having no early AMD at baseline (18.4% vs. 5%, P = 0.048). Late AMD occurred in 5 eyes (38.5%) from 13 fellow RPD eyes of unilateral late AMD at baseline. In logistic regression analysis, thin choroidal thickness, diffuse distribution of RPD, and the presence of late AMD on fellow eye at baseline were significant risk factors for developing late AMD in RPD eyes. Conclusion Reticular pseudodrusen eyes revealed various progression rates to late AMD according to AMD status of both eyes. More frequent monitoring should be considered for patients with RPD at risk of progression to late AMD.

Purpose We investigate the measurable range of subfoveal choroidal thickness (SFCT) with conventional spectral domain optical coherence tomography (SD-OCT) in normal healthy eyes. Methods All subjects underwent an SD-OCT horizontal line scan centered at the fovea with standard and enhanced depth imaging (EDI) techniques. Two independent observers manually measured SFCT on standard and EDI-OCT images, if two choroidal borders were identified. The rate of successful measurement with standard OCT was evaluated. Inter- and intraclass correlation coefficients and Bland-Altman plots were used for analysis. Results OCT images of 724 normal healthy eyes from 374 subjects were evaluated. Although the inner choroidal border was identified on all OCT images, the outer choroidal border was identified on 378 of 724 (52.2%) conventional OCT images. Mean SFCT of all study eyes measured by EDI-OCT images was 292.6 ± 94.0. The number of successful measurements with conventional OCT was 100%, 87.5%, 48.1%, 33.0%, and 0%, in the SFCT ranges of 360 μm, respectively. The accumulated rate of successful measurement with conventional OCT was 100%, 96.4%, and 82.2% in the SFCT ranges of ≤240, ≤280, and ≤320 μm, respectively. Two protocols showed good inter-correlation of SFCT, when SFCT were measurable in both scans. Conclusions SFCT can be measured by conventional OCT in eyes with thin choroid, and the measured value shows high agreement with those measured by EDI-OCT. In such eyes, conventional OCT can be used potentially to evaluate the retina and choroid. Translational Relevance In eyes with a thin choroid, conventional OCT can be used potentially to evaluate the retina and choroid with high agreement with EDI-OCT.

Reticular pseudodrusen (RPD) could be present not only in the posterior pole but extramacular area also as a confluent morphological pattern. Thus RPD can be classified by the fundus distribution for the assessment of visual prognosis. The electrophysiological function in eyes with reticular pseudodrusen (RPD), showing various fundus distribution were evaluated using full-field electroretinogram (ERG). Retinal distribution of RPD was divided into three types (localized, intermediate, and diffuse) according to the extent of involvement of retinal areas by fundus photograph montages. RPD were present with the diffuse type in 21 eyes (25.6%), with the intermediate type in 30 eyes (36.6%), and with the localized type in 31 eyes (37.8%). The average age was 74.76 ± 4.52 (range, 65-81) years in the diffuse type, 72.47 ± 9.13 (range, 55-91) years in the intermediate type, and 70.26 ± 7.77 (range, 61-89) years in the localized type. The mean amplitudes of the scotopic rod response, scotopic maximal combined response, oscillatory potentials (OP), photopic cone response, and 30Hz cone flicker response were more decreased in the diffuse, intermediate, and localized types in order, except for the photopic cone a-wave response. The diffuse type showed reduced amplitudes of ERG responses than the normal control group under all testing conditions except for the photopic a-wave response, and differences were statistically significant with the age restriction and adjustment methods (Bonferroni-corrected P < 0.05). The mean implicit times of ERG responses were significantly delayed in the diffuse type in the photopic b-wave. (Bonferroni-corrected P < 0.05). Extensive retinal involvement of RPD correlates with severely reduced electrophysiological retinal function. This acquired form of decreased electrophysiological function should be regarded as different from those of hereditary retinal degeneration.

Eyes with polypoidal choroidal vasculopathy (PCV) were recently reported to have various choroidal thickness, and choroidal thickness might be associated with visual outcome in the treatment of many retinal disorders. The range of subfoveal choroidal thickness (SFCT), clinical features, and 3-month treatment response among three groups having different range of SFCT were investigated in PCV eyes. In 78 treatment-naïve eyes with PCV, SFCT was measured using optical coherence tomography. Eyes were classified into thin, medium, and thick groups, using mean and one standard deviation of SFCT. Clinical features and imaging findings were compared among the three groups. Some eyes were treated with three consecutive monthly injection of anti-vascular endothelial growth factor (VEGF) as an initial treatment. They were also classified into three thickness groups, and the short-term post-treatment improvement in visual acuity and central retinal thickness were compared among groups. The mean SFCT was 271.9 ± 135.6 μm. Twelve, 53, and 13 eyes were classified into thin (407.5 μm) groups, respectively. The thin group showed older age, lower visual acuity, and a higher prevalence of fundus tessellation than the other two groups (P

Purpose To compare the risk factors and rate of progression of zone I versus zone II type 1 retinopathy of prematurity (ROP). Methods The medical records of consecutive preterm infants with bilateral type 1 ROP in zone I and age-matched control infants with type 1 ROP in zone II were retrospectively analyzed. Fundus findings at each screening examination and systemic parameters were compared between groups. Univariate and conditional multivariate regression analyses were employed to identify variables significantly associated with zone I ROP. Results A total of 30 cases and 30 controls were included. The mean gestational age of included infants was 24.6 weeks in both groups, and the mean birth weights were 685 g in the zone I group and 667 g in the zone II group. The postmenstrual age (PMA) at the time of initial ROP detection did not differ between groups, but the PMA at the time of type 1 ROP detection was significantly earlier in the zone I group (mean, 34.9 vs 37.6 weeks). Conditional multiple logistic regression revealed that mechanical ventilation for 30 days or more was significantly associated with the type 1 ROP in zone I compared with zone II (OR, 3.5; 95% CI, 1.2-10.0). Conclusions Zone I ROP exhibited rapid progression, necessitating close monitoring and prompt treatment. Compromised pulmonary function with associated mechanical ventilation in early life may restrict retinal vascular growth and increase the likelihood of zone I type 1 ROP.

Purpose To characterize a variant type of drusenoid deposit with different imaging features in comparison to reticular pseudodrusen. Methods Retrospective observational consecutive case series. Eyes showing atypical drusenoid lesions were sorted out from 257 eyes of 133 patients previously diagnosed as reticular pseudodrusen. Eyes were evaluated using color fundus photography, confocal scanning laser ophthalmoscopy, and spectral domain optical coherence tomography. Results A variant type of drusenoid deposits showing different imaging features from reticular pseudodrusen was found in 17 eyes of 12 patients (6.6%). The mean age of patients was 62.7 ± 11.6 years, and all patients were women. These deposits were observed as yellowish white, round to oval lesions on color photographs, located under the sensory retina and above the retinal pigment epithelium on spectral domain optical coherence tomography similar to reticular pseudodrusen. However, they were present in a smaller number as discrete lesions and showed increased autofluorescence. None of them were accompanied by late age-related macular degeneration. Conclusion Subretinal drusenoid deposits are not homogeneous and can be classified into two types according to the fundus autofluorescence. Multimodal imaging tests are needed for the differential diagnosis of subretinal drusenoid deposits.

As younger preterm infants are able to survive, more extremely preterm infants are at risk of developing retinopathy of prematurity (ROP). To investigate the incidence, progression and risk factors of ROP in extremely preterm infants in Korea, the medical records of infants born before 25 weeks gestation were retrospectively reviewed. The criteria for laser treatment agreed with type 1 ROP as defined by the Early Treatment for Retinopathy of Prematurity study. Of the 121 infants included in the analysis, 119 (98.4%) infants developed any stage ROP, including 78 infants (64.5%) with type 1 ROP. The mean postmenstrual age (PMA) at the onset of any ROP and type 1 ROP were 33.5 and 36.1 weeks, respectively. All but one infant developed type 1 ROP after 31 weeks PMA. Univariate analysis showed that duration of total parenteral nutrition and onset of any ROP (PMA) were associated with the development of type 1 ROP. In conclusion, this study shows high incidence of ROP in extremely preterm infants and suggests that, although current screening protocols are feasible for most preterm infants born before 25 weeks gestation, earlier screening before 31 weeks PMA may be necessary in infants with an unstable clinical course.

Background To evaluate the morphological features and prevalence of accompanying late age-related macular degeneration (AMD) according to the fundus distribution of reticular pseudodrusen (RPD). Methods According to the involved area in the fundus, the distribution of RPD was classified as localised, intermediate, or diffuse type. Morphology of RPD was classified as discrete, branching, or confluent pattern. The presence of late AMD was evaluated. Results 233 eyes of 121 patients with RPD were included. The distribution of RPD was localised, intermediate and diffuse type in 30.9%, 40.3% and 28.8% of eyes, respectively. The discrete, branching and confluent morphological patterns were found in 45.8%, 44.8% and 9.7% of the localised type, and in 0%, 13.8% and 86.2% of the intermediate type, respectively. In contrast, the diffuse type showed only the confluent morphological pattern. The prevalence of accompanying late AMD was 13.9%, 13.8% and 56.7% in the localised, intermediate and diffuse type, respectively, and it was significantly higher in the diffuse type (p Conclusion RPD with diffuse distribution showed a confluent morphological pattern and a high prevalence of late AMD. RPD can be classified by the fundus distribution for the assessment of visual prognosis.

Purpose The purpose of this study was to demonstrate a negative association between intraocular pressure (IOP) and age in 2 Asian populations. In addition, we evaluated genetic and nongenetic factors associated with IOP. Design Family-based cohort study. Participants Study subjects >10 years of age from one Korean ( The Healthy Twin ; n=1431) and 2 Mongolian populations ( The GENDISCAN ; n=859 and 806) with IOP values. Methods The IOP values were measured with a noncontact tonometer. Each participant received a standard health examination and received questionnaires, which include candidate risk factors on IOP. Mixed models were used to identify risk factors for IOP. Variance-component methods were applied to estimate the heritability of IOP. Main Outcome Measures The negative trend of IOP with aging and evaluation of impact of genetic and nongenetic components on IOP. Results The mean ages were 43.6, 34.1, and 36.3 years for the Korean, Orhontuul, and Dashbalbar populations, respectively. The mean IOPs were 14.4 mmHg (95% confidence interval [CI], 14.3–14.6) in the Koreans and 14.1 mmHg (95% CI, 13.9–14.3) and 12.6 mmHg (95% CI, 12.4–12.9) in the Orhontuul and Dashbalbar populations, respectively. In the 3 populations, the IOP decreased as age increased. We replicated an association of systolic blood pressure (SBP) with IOP. In addition, components of the metabolic syndrome (MS), such as plasma glucose, lipid level, and body mass index, showed positive associations with IOP, after adjusting for age and SBP. The IOP also had strong genetic contributions in all populations (heritability, 0.47–0.51). Conclusions Negative associations between age and IOP were observed in all 3 populations, which cannot be explained by the increasing prevalence of myopia in the younger generation. The different age trend in IOP may in part be responsible for differences in the prevalence of glaucoma subtypes. Our findings suggest that associations between IOP and MS components were independent of established risk factors such as SBP or age. In addition, the importance of inherited risks requires further genetic dissection of IOP determinants for biological understandings of underlying pathophysiology. Financial Disclosure(s) The authors have no proprietary or commercial interest in any materials discussed in this article.

Background/aims: To analyse the clinical manifestations and results of treatment for patients with retinoblastoma in Korea. Methods: The medical records of 70 children (92 eyes) diagnosed as having retinoblastoma and treated between 2000 and 2006 were retrospectively analysed. Data on gender, age at diagnosis, laterality, presenting sign, classification of tumour, treatment modality and prognosis were collected. Results: The most common presenting sign was leucocoria (80%). 31.4% developed bilateral retinoblastoma. Using the International Classification of Retinoblastoma, 7.5% were group A, 23.8% were group B, 6.3% were group C, 38.8% were group D, and 23.8% were group E. 26.1% of eyes were treated with chemoreduction and/or focal therapy, namely, they achieved globe preservation, and all other eyes were enucleated. The globe preservation was achieved in 100% of group A, 77.8% of group B, 66.7% of group C, and 26.7% of group D. Conclusions: In Korea, most children with retinoblastoma showed an advanced stage of tumour at the time of diagnosis and although they were treated with an updated therapeutic approach according to the newly introduced classification, the rate of globe preservation did not reach that of developed countries. Increased surveillance should be performed.

To assess the correlation between the features of optical coherence tomography (OCT) and fluorescein angiography in clinically significant diabetic macular edema.Retrospective observational case series.This study involved 145 eyes (91 patients) with clinically significant diabetic macular edema. The fluorescein angiography features were categorized into focal leakage type, diffuse leakage type, and diffuse cystoid leakage type. The OCT features were categorized into four types: type 1, thickening with homogeneous optical reflectivity; type 2, thickening with markedly decreased optical reflectivity in the outer retinal layer; type 3A, foveolar detachment without traction; and type 3B, foveolar detachment with apparent vitreofoveal traction. The correlation between fluorescein angiography types and OCT types were analyzed, and their associations with visual acuity, central foveal thickness. and stages of diabetic retinopathy were evaluated.The prevalence of OCT type 1 was higher in the focal leakage type (73.0%) and in the diffuse leakage type (58.9%) than in the diffuse cystoid leakage type (3.8%) of fluorescein angiography (P.0001). The prevalence of OCT type 2 and 3A was higher in the diffuse cystoid leakage type (57.7% and 34.6%, respectively) than in the focal leakage type (20.6% and 3.2%) or diffuse leakage type(28.6% and 10.7%; P.0001). The stages of retinopathy correlated with fluorescein angiography types (P =.016). Optical coherence tomography type 1 and the focal leakage type of fluorescein angiography showed the least foveal thickness and the best visual acuity (P.05).There was a significant correlation between the features of OCT and fluorescein angiography in clinically significant diabetic macular edema. The combined data from both OCT and fluorescein angiography may provide a clearer understanding of the anatomic and physiologic characteristics of clinically significant diabetic macular edema.

We evaluated macular ganglion cell-inner plexiform layer (GC-IPL) thickness using spectral-domain optical coherence tomography (SD-OCT) in patients with chronic exposure to hydroxychloroquine (HCQ).This study included 130 subjects, who were divided into three groups: Group 1A, 55 patients with HCQ use ≥5 years; Group 1B, 46 patients with HCQ use5 years; and Group 2, 29 normal controls. In all patients with exposure to HCQ, fundus examination, automated threshold perimetry, fundus autofluorescence photography, SD-OCT, and GC-IPL thickness measurement using the Cirrus HD-OCT ganglion cell analysis algorithm were performed. Average and minimum macular GC-IPL thickness were compared between subjects groups, and correlations between GC-IPL thickness and duration or total dose of HCQ use were analyzed.Among the 101 patients of Group 1, six patients who showed clinically evident HCQ retinopathy also showed markedly thin macular GC-IPL. In addition, weak but significant negative correlations were observed between the average and minimum GC-IPL thickness of Group 1 patients and cumulative dose of HCQ, even when analyzing without the six patients with HCQ retinopathy. However, when analyzing after exclusion of patients with high cumulative doses (1000 g), significant correlations were not observed.This study revealed that macular GC-IPL thickness did not show definite correlations with HCQ use. However, some patients, especially with HCQ retinopathy or high cumulative doses, showed thin GC-IPL.