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PURPOSE To describe four eyes of three patients with presumed acute fovealitis to expand the clinical variants of this recently described disorder. METHOD The patients underwent a comprehensive ophthalmic examination, including multimodal imaging and electrophysiological tests. RESULTS Three female patients aged from 18 to 24 years presented sudden central visual disturbances in one or both eyes. The visual acuity ranged from 20/25 to 20/70 in the affected eyes. All of them showed a subtle yellowish lesion in the foveola. Fundus autofluorescence and fluorescein angiography were unremarkable. Optical coherence tomography disclosed focal disarrangement of the outer retinal layers restricted to the fovea and hyperrefective lesions above the external limiting membrane. Multifocal electroretinography responses were attenuated. The electrooculogram response was normal. All patients recovered normal visual acuity and the outer retinal layers. CONCLUSION Attenuated multifocal electroretinography foveal response and normal electrooculogram are newly described clinical findings in patients with acute fovealitis.

Purpose To describe the clinical findings and outcome of an unusual case of subfoveal yellowish deposits associated with excessive doses of phosphodiesterase type 5 inhibitors intake. Methods Single retrospective case report. Results A 50-year-old South American man with a personal history of multiple orally administered phosphodiesterase type 5 inhibitors drugs to treat a chronic erectile dysfunction complained of dyschromatopsia, photophobia and a three-months history of blurred vision. The fundus examination revealed a symmetrical yellowish lesion in both eyes with a subfoveal hyperreflective signal on the spectral domain optical coherence tomography at the level of the ellipsoid zone. Dysregulation of the blue-yellow axis was noticed in the color vision test and the electroretinogram demonstrated a tiny decrease in the b-wave amplitude. The use of phosphodiesterase type 5 inhibitors was discontinued and the patient was examined three months later. Optical coherence tomography revealed complete disappearance of the deposit in both eyes and the associated symptoms resolved rapidly with discontinuation of the drug. Conclusions A bilateral ellipsoid zone subfoveal focal thickening may be related to a yellowish symmetrical spot on the fovea in patients with a history of phosphodiesterase type 5 inhibitors overdose intake. Functional and structural findings are reversible in a short-term period after discontinuing the oral treatment.

This study analyses whether prematurity, retinopathy of prematurity (ROP), and associated factors lead to altered foveal shape in adulthood and whether these alterations are associated with visual acuity.The Gutenberg Prematurity Eye Study is a German cohort study with a prospective ophthalmologic examination (participants aged 18-52 years) of individuals born preterm and full-term that were examined with spectral domain optical coherence tomography. Participants were grouped according to gestational age (GA) and postnatal ROP status. Multivariable linear regression analyses for foveolar retinal thickness, foveal hypoplasia, and posterior vitreous status were performed.A total of 755 eyes of 414 preterm and full-term individuals were included (aged 28.6 ± 8.6 years, 233 female individuals). Central foveal retinal thickness increased as GA decreased. The prevalence of foveal hypoplasia was 2% (control group), 9% (GA 33-36), 18% (GA 29-32), 48% (GA ≤28), 50% (ROP without treatment), and 82% of eyes (with ROP requiring treatment). In multivariable analyses, central foveal thickness was independently associated with GA and advanced stages of ROP requiring treatment while foveal hypoplasia was only associated with GA. Posterior vitreous was more frequently visible as partially detached in full-term than in preterm individuals. Lower distant-corrected visual acuity correlated with increased foveolar thickness (rho = 0.08; P = 0.03) and with foveal hypoplasia (rho = 0.15, P0.001).Our findings indicate that there are fetal origins affecting foveal shape, resulting in foveal hypoplasia potentially affecting the visual acuity in adulthood.

This study aimed to evaluate fixation stability and position changes after epiretinal membrane (ERM) surgery.This is a retrospective study that included 60 consecutive eyes with idiopathic ERM. All patients received comprehensive ophthalmic examinations preoperatively and 1, 4, and 10 months postoperatively. Main outcome measures included fixation stability and position, bivariate contour ellipse area, and preferred retinal locus.The number of patients with stable fixation and predominantly central fixation was increased significantly after ERM surgery (61.7 vs. 73.3%, P = 0.001; 41.7 vs. 71.7%, P = 0.037). The correlation analysis showed that the preoperative factors associated with improved postoperative fixation stability were a larger bivariate contour ellipse area value (P0.001), poorer visual acuity (P = 0.002), advanced stage (P = 0.002), thicker central fovea thickness (P = 0.015), and a longer preferred retinal locus-fovea distance (P = 0.025). As for the improved fixation location, the associated preoperative factors were age (P = 0.003), central fovea thickness (P = 0.044), and preferred retinal locus-fovea distance (P0.001).Our observations point to the changes of fixation parameters in patients after ERM surgery. We found that patients with preoperative unstable, eccentric fixation and poor BCVA can significantly benefit from the surgery. This result indicates that even in patients with severe macular damage, the ERM surgery still has great benefits in recovering visual function.

To analyze the relationship between Henle fiber (HF) integrity and visual acuity in highly myopic eyes with foveoschisis.Three hundred and fifty-eight highly myopic eyes were included in this study and divided into three groups according to the Triton optical coherence tomography results. Group 1 included 19 eyes with myopic foveoschisis where the inner and outer retina were connected by a columnar structure in the HF layer at the foveolar area. Group 2 included 17 eyes with myopic foveoschisis where the columnar structure was disrupted in the HF layer at the foveolar area. Group 3 included 322 eyes without myopic foveoschisis or other ocular disease. Clinical and optical coherence tomography findings were obtained and compared.Eyes with vitreomacular traction, arteriolar traction, and longer axial length were more likely to have myopic foveoschisis with intact columnar structure in the HF layer (odds ratio [OR], 12.84; P = 0.001; OR, 7.63; P = 0.04; OR, 2.03; P = 0.03) and with disrupted columnar structure in the HF layer (OR, 65.21; P = 0.001; OR, 6.60; P = 0.02; OR, 2.63; P = 0.01). Moreover, eyes with disrupted columnar structure in the HF layer presented a lower best-corrected visual acuity (P = 0.001), longer axial length (P = 0.001; P = 0.009), higher central foveolar thickness (P = 0.001; P = 0.02), and a higher prevalence of vitreomacular traction (P = 0.001; P = 0.04) than control or integrity HF eyes. Furthermore, worse best-corrected visual acuity was related to myopic foveoschisis with disrupted columnar structure in the HF layer (P0.01).The integrity of HF in patients with myopic foveoschisis appears to play an important role in visual acuity.

To evaluate the visual outcomes and the affect of timing of surgical repair of fovea-splitting rhegmatogenous retinal detachments.A retrospective, consecutive cohort from multiple surgeons at a single center. Fovea status (fovea-on, fovea-splitting, or fovea-off) was classified by preoperative optical coherence tomography. The primary outcome measure was the visual acuity at the last follow-up that was further correlated with the timing of surgical repair.One hundred and ninety-five eyes were included with 62 fovea-on, 65 fovea-splitting, and 68 fovea-off detachments. The mean preoperative logarithm of the minimum angle of resolution visual acuity for fovea-on, fovea-splitting, and fovea-off groups was 0.16 ± 0.21, 0.70 ± 0.56, and 1.67 ± 0.87, respectively (P =0.001). Mean postoperative logarithm of the minimum angle of resolution visual acuity for fovea-on, fovea-splitting, and fovea-off groups were 0.07 ± 0.13, 0.10 ± 0.15, and 0.20 ± 0.22, respectively (P =0.001). A statistically significant difference in mean postoperative logMAR visual acuity was found between fovea-off and fovea-on groups (P = 0.003) and between fovea-off and fovea-splitting groups (P = 0.013), however not between fovea-on and fovea-splitting groups (P = 0.827). Visual acuity improved when repair was performed earlier after presentation for fovea-on (R = 0.378, P = 0.002) and fovea-off groups (R = 0.277, P = 0.022), but not for the fovea-splitting group (R = 0.089, P = 0.481).We described the favorable visual outcomes of surgery for fovea-splitting rhegmatogenous retinal detachment and correlated these with the timing of surgical repair, which may help guide the management of this urgent, vision-threatening condition.

PURPOSE To examine hypotony-associated foveal lesions (FovLs) using optical coherence tomography, and to assess the risk factors of visual deterioration after glaucoma filtering surgery. METHODS Parameters that may be associated with postsurgical deterioration of visual acuity were retrospectively studied in 44 eyes of 44 patients who experienced postsurgical intraocular hypotension ≤6 mmHg between 2015 and 2019. RESULTS Six eyes (14%) had FovLs, such as detachment of photoreceptors (5 eyes, 11%) and acquired vitelliform lesions (1 eye, 2%) at 3 months after trabeculectomy. Logistic regression analysis revealed that hypotony maculopathy (P = 0.0141 at 3 months) and FovLs (P = 0.0486 and 0.0296 at 3 and 12 months, respectively) were significant risk factors for Visual acuity loss after trabeculectomy. The FovLs were located just behind the Muller cell cone. Visual acuity at 3 and 12 months after surgery in patients with FovLs was significantly lower than in those without FovLs (P = 0.0013 and P = 0.006, respectively). Epiretinal membrane was more common in eyes with FovLs (5 of 6 eyes, 83%) than in eyes without FovLs (7 of 38 eyes, 18%; P = 0.0037). CONCLUSION Muller cell cone-associated FovLs lead to long-lasting visual acuity loss after filtering surgery.

PURPOSE To investigate the foveal movement in eyes with epiretinal membrane after vitrectomy and membrane peeling. METHODS A retrospective review of 85 eyes with epiretinal membrane treated with vitrectomy, membrane peeling, and internal limiting membrane removal. Using a self-designed computer program to compare the preoperative and postoperative images of optical coherence tomography to measure the amount of foveal movement. Analyze the relationships between foveal displacement, preoperative and postoperative best-corrected visual acuity, central foveal thickness, and the stage of epiretinal membrane. RESULTS Most of the fovea were nasally shifted. More movement happened in the first month and almost finished in the first year. The greater degree of foveal displacement was correlated with poorer initial visual acuity and thicker central foveal thickness. In considering with the ectopic inner foveal layer staging of epiretinal membrane by structural optical coherence tomography, the foveal realignment is greatest in Stage 4 (394.47 ± 171.44 µm), followed by Stage 1 (251.21 ± 135.40 µm), Stage 2 (235.70 ± 147.51 µm), and Stage 3 (219.86 ± 117.91 µm) at the postoperative 1 month. CONCLUSION Most eyes of epiretinal membrane had the foveal moved nasally after membrane peeling and internal limiting membrane peeling. The larger amount of foveal movement was correlated with poorer initial best-corrected visual acuity. Dystopia fovea may be another factor affecting visual acuity in addition to other biomarkers in optical coherence tomography.

PURPOSE To investigate whether the parafoveal capillary architecture predicts clinical course and visual outcomes after epiretinal membrane (ERM) surgery. METHODS A total of 71 eyes of 71 patients treated with vitrectomy for idiopathic ERM were enrolled. The parafoveal capillary displacement and fractal geometries were compared according to the stage of ERM. Correlations between the parafoveal capillary displacement in the superficial capillary plexus, the fractal dimension and lacunarity in the deep capillary plexus (DCP), foveal thickness, and visual outcomes were evaluated. RESULTS Compared with eyes with mild ERM, eyes with severe ERM exhibited higher parafoveal capillary displacement in the superficial capillary plexus, lower fractal dimension and higher lacunarity in the DCP, and greater foveal thickness (P < 0.05). The parafoveal capillary displacement in the superficial capillary plexus and fractal dimension and lacunarity in the DCP improved significantly, particularly at 1 month postoperatively (P < 0.05) and reached a plateau thereafter. The preoperative fractal dimension in the DCP showed a significant correlation with the best-corrected visual acuity at all follow-up time points (P < 0.05). CONCLUSION The parafoveal fractal dimension in the DCP was significantly correlated with the visual acuity before and after ERM surgery. The parafoveal fractal dimension may serve as a predictive marker for visual outcomes after ERM surgery.

PURPOSE To characterize the early retinal microvascular changes in young adults (age: 22.69 ± 3.50 years) with Type 1 diabetes mellitus without clinically detectable diabetic retinopathy using optical coherence tomography angiography and investigate the associated factors. METHODS A total of 36 participants with Type 1 diabetes mellitus (70 eyes) and 34 healthy controls (57 eyes) were retrospectively reviewed. The analyzed optical coherence tomography angiography indices included capillary vessel density, foveal avascular zone area/perimeter/acircularity index, and foveal vascular density, acquired in the 6 × 6-mm2 area centered on the fovea. The generalized estimation equations model was applied to compare the mean values and to study the associated factors. RESULTS In subjects with diabetes, statistically significant decreases were observed in parafoveal vessel density in both superficial and deep capillary plexuses, foveal avascular zone area/perimeter, and foveal vascular density when compared with controls (all P < 0.05). Higher glycated hemoglobin level was independently associated with the decrease of parafoveal vessel density as well as the increase of foveal avascular zone area/perimeter (all P < 0.05). Prepubescent onset of diabetes mellitus was also independently associated with the decrease of superficial parafoveal vessel density, foveal avascular zone area/perimeter, and foveal vascular density (P = 0.015, 0.011, 0.015, and 0.001, respectively). CONCLUSION In young adults with Type 1 diabetes mellitus lacking clinical signs of diabetic retinopathy, optical coherence tomography angiography revealed alterations in retinal microvasculature that were associated with glycated hemoglobin level and onset of diabetes mellitus related to puberty.

Purpose To investigate the dilated choroidal veins (DCVs) at or around myopic macular neovascularizations (MNVs) and to determine whether there is a hemodynamic relationship between them. Methods Fifty-eight eyes of 57 patients with myopic MNVs were examined. Dilated choroidal veins were defined as choroidal veins whose diameter was 2X larger than adjacent veins. Indocyanine green angiography and swept-source optical coherence tomography images were reviewed to detect DCVs that crossed the subfoveal area. The filling sequence of the DCVs and MNVs was determined. Results Patients' mean age was 71.4 ± 10.6 years. The mean axial length was 29.3 ± 1.8 mm. Dilated choroidal veins below or around the MNV were found in 17 eyes (29.3%). Emissaries of the short posterior ciliary arteries were seen at or around MNVs in 8 of the 17 eyes. In these eyes, the short posterior ciliary artery was filled first or almost simultaneously with the filling of the MNV, followed by a laminar filling of the DCVs. In one eye, afferent arterioles from the short posterior ciliary arteries and efferent venules connected to DCVs were seen. Conclusion Dilated choroidal veins are present below or around MNVs in about 30% of eyes with myopic MNVs. Our findings suggest that an MNV might be a vascular unit consisting of short posterior ciliary arteries, afferent arterioles, efferent venules, and DCVs.

PURPOSE To evaluate associations between choroidal thickness and features of polypoidal choroidal vasculopathy (PCV) lesions based on multimodal imaging. METHODS This cross-sectional analysis included treatment-naive PCV eyes from a prospectively recruited observational cohort. Associations between of subfoveal choroidal thickness (SFCT) and qualitative and quantitative morphologic features of PCV lesions on color fundus photographs, indocyanine green and fluorescein angiography, and spectral-domain optical coherence tomography were evaluated. RESULTS We included 100 eyes with indocyanine green angiography-proven PCV. Subfoveal choroidal thickness showed a bimodal distribution with peaks at 170 µm and 350 µm. There was a significant linear increase in the total lesion area (P-trend = 0.028) and the polypoidal lesion area (P-trend = 0.030 and P-continuous = 0.037) with increasing SFCT. Pairwise comparisons between quartiles showed that the total lesion area (4.20 ± 2.61 vs. 2.89 ± 1.43 mm2, P = 0.024) and the polypoidal lesion area (1.03 ± 1.01 vs. 0.59 ± 0.45 mm2, P = 0.042) are significantly larger in eyes in Q4 (SFCT ≥ 350 μm) than eyes in Q1 (SFCT ≤ 170 μm). Although there was no significant linear trend relating SFCT to best-corrected visual acuity, pairwise comparisons showed that eyes in Q4 (SFCT ≥ 350 μm) have significantly worse vision (0.85 ± 0.63 vs. 0.55 ± 0.27 logMAR, P = 0.030) than eyes in Q2 (SFCT 170-260 μm). CONCLUSION Total lesion areas and polypoidal lesion areas tend to be larger in eyes with increasing SFCT. Choroidal background may influence the phenotype or progression pattern of PCV.

PURPOSE To evaluate association of the baseline macular hole (MH) geometric features and longitudinal ellipsoid zone integrity with the visual acuity outcome after surgical repair. METHODS This was a post-hoc analysis of eyes in the DISCOVER study undergoing vitrectomy repair for MH. Anatomical and functional data were collected through one year postoperatively. An automated retinal layer segmentation platform was used for the assessment of outer retinal metrics and volumetric reconstruction of MH. Association of longitudinal ellipsoid zone features and baseline MH height, width, and volume with VA outcomes were investigated. RESULTS Eighty-four eyes with MH were included. The mean baseline VA was 20 of 114 and increased to 20 of 45 (P < 0.001) at postoperative Month 12 (N = 45). Successful MH closure was achieved in 98.8% of cases. Ellipsoid zone integrity metrics significantly improved from baseline (P = 0.002) and postoperative Month 1 (P < 0.001) to post-operative Month 12. Ellipsoid zone metrics independently correlated with VA at all follow-up visits (P < 0.05). Increased baseline MH width and volume negatively correlated with the VA at postoperative Month 12 (P < 0.001). Preoperative VA and EZ integrity on optical coherence tomography were predictors for postoperative VA. CONCLUSION Baseline MH volumetric parameters and EZ parameters were associated with VA outcomes after repair.

Purpose To evaluate topical dorzolamide hydrochloride-timolol maleate as an adjunct therapy to intravitreous anti-vascular endothelial growth factor injections in eyes with retinal vein occlusion resistant to treatment. Methods Retinal vein occlusion patients with a history of persistent macular edema, despite fixed-interval intravitreous anti-vascular endothelial growth factor injections, were enrolled between April 4, 2016, and June 4, 2017. On enrollment, patients were instructed to administer one drop of topical dorzolamide-timolol twice daily for the duration of the study. They were maintained on the same anti-vascular endothelial growth factor drug and same interval between injections as preenrollment for the subsequent two visits. Primary outcome measures were change in central foveal thickness, central subfield thickness, and thickest macular cut at the final study visit. Results Eight patients (8 eyes) were eligible for enrollment and completed the study. There was a significant decrease in central foveal thickness (P = 0.02), central subfield thickness (P = 0.03), and thickest macular cut (P = 0.01) between the enrollment visit and the final visit. There was a decrease in mean (±SD) logarithm of the minimal angle of resolution from 0.52 (±0.35) (20/66, Snellen equivalent) at the enrollment visit to 0.41 (±0.35) (20/51, Snellen equivalent) at the final visit (P = 0.08). Conclusion Topical dorzolamide-timolol may have a beneficial anatomical and functional effect in eyes with macular edema secondary to retinal vein occlusion resistant to treatment.

Prcis Recognizing the shortcomings of poor consistency of the fovea-disc relationship at different time points and comparing PanoMaps may well facilitate the understanding of the spatial relationship between parapapillary and macular areas in glaucoma. Purpose The aims of this study are to analyze the spatial distribution of the frequently damaged areas in early glaucoma, compare the patterns between nonprogressors and progressors using the combined wide-field parapapillary and macular deviation maps (PanoMap), and to evaluate the consistency of the fovea-disc relationship in PanoMap. The fovea-disc distance (FDD) and fovea-disc axis (FDA) were compared at baseline and at the last follow-up. Materials and methods In total, 118 patients with early glaucoma and a minimum follow-up period of 3 years were included in this retrospective observational study. The pattern of structural changes was evaluated by averaging the PanoMaps of the enrolled patients at baseline and last follow-up. Longitudinal comparison of the FDD and FDA was performed at baseline and the last follow-up. Patients were divided into nonprogressor (n=44) and progressor (n=74), and the patterns of glaucoma progression in PanoMaps were compared between them. Results At baseline, the glaucomatous damage was found more frequently in the macular compared with the parapapillary area. The spatial distribution of frequently damaged areas was similar between the nonprogressor and progressor. At the last follow-up, compared with the baseline structural change on the PanoMap, the progressive structural changes extended toward the fovea at both the parapapillary and macular areas in the progressor. The FDD and FDA were significantly different between the baseline and the last follow-up. Conclusions The PanoMaps showed a clear spatial distribution of early glaucomatous changes, indicating that the damaged area was frequently observed in the macular area. As the consistency of the fovea-disc relationship in PanoMaps was not excellent, this aspect should be considered when interpreting the PanoMap.

PURPOSE To measure the vascular density (VD) of the retinal capillary plexuses by optical coherence tomography angiography (OCTA) after surgery for an idiopathic macular hole. METHODS Retrospective, observational case series.Sixteen eyes of 16 patients with an idiopathic macular hole underwent vitrectomy with internal limiting membrane peeling. The VDs of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were determined by OCTA, and the retinal sensitivity (RS) and the inner retinal thickness (IRT) were measured before, and at 3, 6, and 12 months after the surgery. The VD, RS, and IRT were measured at the four parafoveal quadrants. RESULTS The mean age was 68.9 years. The VDs of the SCP and DCP were significantly correlated with the RS and IRT at 12 months postoperatively (all P < 0.001 for both SCP and DCP). The VDs of the SCP and DCP were higher, the RS more sensitive, and the IRT thicker in the nasal than the temporal quadrant at 12 months (P < 0.001

Purpose To describe a case of stellate nonhereditary idiopathic foveomacular retinoschisis in a middle-aged woman and to depict the classic retinal fluorangiography (FA) findings, structural characteristics using macular spectral-domain optical coherence tomography angiographic data of vascular and perfusion density using optical coherence tomography angiography (OCT-A), and standardized multifocal electroretinography (mfERG) findings. Methods This is a case report of a 53-year-old ophthalmologist who was incidentally diagnosed with unilateral idiopathic foveomacular retinoschisis. Stellate nonhereditary idiopathic foveomacular retinoschisis is defined as a foveal elevation without alternative explanation for retinoschisis. FA, spectral-domain optical coherence tomography, optical coherence tomography angiography, and multifocal electroretinography were used as tools to obtain an integral multimodal diagnosis of this entity. Results Clinical examination and multimodal imaging were able to detect unilateral idiopathic retinoschisis, revealing a stellate pattern of retinal concentric cysts with minimal changes in vascular and perfusion density metrics and confirming the absence of bridging vessels. There were consistent FA findings, with almost unaltered foveal changes. Multifocal electroretinography depicted a subtle reduction in dark-adapted a-wave and b-wave amplitudes. Conclusion Improvements and innovations in technology for ophthalmic diagnosis have revolutionized our capacity for diagnostic decision-making. Spectral-domain optical coherence tomography and optical coherence tomography angiography are useful tools for diagnosis and follow-up assessment. This fortuitous case gives a window on the importance of a routine specialized ophthalmic examination and how multimodal imaging can depict important and specific findings not evident from a clinical point of view. The subtle but important changes observed in optical coherence tomography angiography and multifocal electroretinography will help better define this clinical entity.

PURPOSE To assess the status of the cone photoreceptors using adaptive optics flood illumination ophthalmoscopy in patients with birdshot chorioretinopathy and to study their relationship with optical coherence tomography (OCT) angiography. METHODS Seventeen patients with birdshot chorioretinopathy were studied using standard automated perimetry, color vision testing, fluorescein angiography, indocyanine green angiography, spectral domain OCT, enhanced depth imaging OCT, and adaptive optics flood illumination ophthalmoscopy (rtx1, Imagine Eyes, Orsay, France) in a 90 × 90-μm area at 1.5° temporal of the fovea and OCT angiography (Spectralis OCT2, Heidelberg Engineering, Heidelberg, Germany). For adaptive optics flood illumination ophthalmoscopy, spectral domain OCT, and OCT angiography, a control group of 12 healthy patients (12 eyes) matched for age, sex, and refractive error was included. RESULTS After excluding low-quality adaptive optics flood illumination ophthalmoscopy images, 12 eyes (12 patients, 7 women, mean age 53.5 ± 10.8 years) were analyzed. Best-corrected visual acuity was 0.01 ± 0.03 LogMAR (20/25 Snellen equivalent), and foveal threshold at standard automated perimetry was 35.2 ± 3.0. Cone density at 1.5° of the fovea in the birdshot chorioretinopathy group (11,435.25 ± 4,342.9 cells/mm) was significantly smaller than in the control group (24,594.04 ± 4,764.3 cells/mm, P < 0.001). In the same area, birdshot chorioretinopathy eyes showed disruption of the ellipsoid/interdigitation zone and vascular abnormalities, such as capillary loops (58.3%), increased superficial intercapillary spaces (75%), increased deep intercapillary spaces (58.3%), capillary dilations (58.3%), telangiectatic vessels (66.6%), and absence of choriocapillary flow (58%). Cone density at 1.5° was associated with duration of the disease (Spearman's rho -0.8, P = 0.01), although it was not associated with OCT angiography abnormalities in the same area. CONCLUSION Birdshot chorioretinopathy can result in a reduction in cone density and development of macular vascular abnormalities even in the presence of preserved visual function.

PURPOSE To evaluate the subfoveal choroidal thickness (SFCT) and vascular architecture in the fellow eyes of patients with circumscribed choroidal hemangioma (CCH). METHODS In this retrospective observational study, patients were selected from outpatient ophthalmology clinics at the Memorial Sloan Kettering Cancer Center and Vitreous Retina Macula Consultants of New York. Subfoveal choroidal thickness was measured using enhanced depth imaging spectral domain optical coherence tomography from the outer portion of Bruch membrane to the choroidal-scleral interface. Choroidal vascular architecture was qualitatively examined. The main outcome measure was SFCT in fellow eyes of patients with CCH, which was compared with an age- and gender-matched control group. RESULTS Thirty-one fellow eyes (15 right eyes and 16 left eyes) of patients with CCH (23 males and 8 females) were examined. The fellow eye had a mean SFCT of 361.2 ± 99.9 μm compared with 252.0 ± 77.6 μm in the control group (P < 0.0001). Vascular architecture was disorganized in 13 (42%) fellow eyes and 1 (3%) control eye (P < 0.0001), with no apparent gradient of vessel sizes or discrete choroidal layers. The normal association between older age and a thinner choroid existed in control eyes but not in fellow eyes. Hemangioma thickness measured by ultrasound and the presence of subfoveal fluid in the CCH eye did not correlate with the fellow-eye SFCT. CONCLUSION In patients with CCH, fellow eyes had thicker SFCT when compared with age- and gender-matched control eyes. Choroidal architecture was often irregular, without segmented vascular layers. These findings suggest that inherent choroidal changes may exist in patients with CCH.

PRECIS The foveal avascular zone (FAZ) in optical coherence tomography angiography is significantly correlated with retinal inner layer thickness, Humphrey field analyzer (HFA) 10-2 sensitivity threshold, and mean deviation (MD) value in open-angle glaucoma patients. PURPOSE The purpose of this study was to measure the FAZ area using optical coherence tomography angiography and investigate its relationship with retinal inner layer thickness and visual field defects in eyes with open-angle glaucoma. PARTICIPANTS AND METHODS A total of 52 eyes with open-angle glaucoma from 52 patients. FAZ area was measured using optical coherence tomography angiography in angio-macula mode. Thicknesses of retinal nerve fiber layer (RNFL), ganglion cell layer plus inner plexiform layer (GCL+IPL), and ganglion cell complex (GCC) were determined using a 7 mm×7 mm macular (V) map via 3D-OCT. Correlations of FAZ area with sensitivity threshold measurements, foveal threshold (FT), and MD value in the HFA10-2 visual field were analyzed for each inner retinal layer. RESULTS FAZ area was 0.26±0.07 mm; overall mean thicknesses were 19.14±6.55 μm (RNFL), 57.34±5.93 µm (GCL+IPL), and 75.87±10.96 μm (GCC); mean FT was 35.23±3.15 dB, and sensitivity threshold was 20.81±7.22 dB in the HFA10-2 visual field. FAZ area was significantly correlated with the thickness of individual retinal layers in the entire field (RNFL, P

To determine whether the size of the foveal avascular zone (FAZ) is significantly correlated with the best-corrected visual acuity (BCVA) and to examine the relationship between the size and microstructural changes of the photoreceptors in eyes with a branch retinal vein occlusion.The medical records of 69 eyes of patients (mean age, 64.6 ± 11.7 years) with a branch retinal vein occlusion were reviewed after the resolution of macular edema. All the patients underwent optical coherence tomography angiography for measurement of the FAZ area and spectral domain optical coherence tomography for determination of microstructural changes of the photoreceptors at the fovea.The superficial and deep FAZ areas in eyes with a branch retinal vein occlusion were 0.39 ± 0.36 mm and 0.63 ± 0.18 mm, respectively, and both were significantly larger than those observed in the fellow eyes (both, P0.001). The superficial FAZ area correlated with the posttreatment BCVA (r = 0.285, P = 0.027) but not with any parameters regarding the microstructures of the photoreceptors. Multivariate linear regression analysis showed that the pretreatment BCVA (β = 0.519, P0.001) and integrity of the external limiting membrane (β = -0.373, P = 0.001) were independent factors that significantly correlated with the posttreatment BCVA.There was no significant correlation between the FAZ area and microstructural parameters. However, the integrity of the external limiting membrane was significantly correlated with the posttreatment BCVA in eyes with a branch retinal vein occlusion.

Significance Risk stratification plays a large role in cardiovascular disease (CVD) management. Spectral-domain optical coherence tomography (SD-OCT) allows for noninvasive analysis of the ophthalmic vasculature, with potential for systemic disease surveillance. Subfoveal choroidal thickness (SFCT) may serve as a marker for CVD risk and play a role in risk stratification. Purpose This study aimed to explore the association of major CVD risk factors CVD on SFCT measured from SD-OCT images in a general population of U.S. veterans. Methods One hundred fifty veterans were prospectively recruited at the Jamaica Plain VA Hospital (Boston, MA). A total of 143 participants were included in the final analysis. The SFCT was manually measured from Spectralis SD-OCT macular scans, and medical chart review was analyzed for CVD data. The SFCT measurements were correlated with CVD risk factors and CVD clinical makers while controlling for age and refractive error. Mean differences in SFCT between those with and without CVD risk factors were analyzed. Results In multivariate analysis, diabetes diagnosis was independently associated with thinner subfoveal choroid (P = .001) and hypertension and hyperlipidemia with thicker subfoveal choroid (P = .006 and P = .05). After adjusting for age and refractive error, veterans with diabetes had thinner choroids than those without (P = .02), and veterans with hypertension and hyperlipidemia had thicker choroids than those without, although these differences did not reach statistical significance (P = .07 and P = .1). Conclusions Comorbid risk factors for CVD are independently associated with optical coherence tomography-derived measurements of subfoveal choroidal thickness in a vasculopathic population of U.S. veterans, and there are detectable differences in subfoveal choroidal thickness between groups with and without CVD risk factors. Larger studies with adequate controls and longitudinal design are necessary to assess the clinical role of SFCT measurements in CVD risk analysis.

To assess the morphological changes of cone photoreceptors in eyes with autosomal recessive bestrophinopathy.Both eyes of five patients with autosomal recessive bestrophinopathyunderwent spectral domain optical coherence tomography and adaptive optics fundus imaging. The cone photoreceptor densities were measured at intervals of 100 μm between 500 μm nasal and temporal eccentricities from the foveal center.The median age of the patients was 30 years (range, 23-45 years), and the best-corrected visual acuity ranged from 20/20 to 20/80. Adaptive optics fundus images showed reduced cone photoreceptor densities corresponding to the damages of the photoreceptor layer in the spectral domain optical coherence tomography images in four patients with relatively good best-corrected visual acuity. The cone photoreceptor densities at the center of the fovea were less than one-third of the normal cone densities (range 11,600-30,400 cells/mm). Cone photoreceptor mosaics were visible over the lesions with serous retinal detachment and retinal edema, although they were partially hyporeflective.There is a significant cone photoreceptor loss in the macular region of patients with autosomal recessive bestrophinopathy, although they had relatively good visual acuity. Monitoring cone photoreceptors by adaptive optics fundus imaging should provide accurate assessments of the disease status and indications for future therapeutic interventions.

Tuberous sclerosis complex (TSC) is an autosomal dominant multisystemic disorder caused by mutations in either TSC1 or TSC2 genes and is characterized by hamartomas in multiple organs. The most frequent and best-known ocular manifestation in TSC is the retinal hamartoma. Less frequent ocular manifestations include punched out areas of retinal depigmentation, eyelid angiofibromas, uveal colobomas, papilledema, and sector iris depigmentation. In this article, we report 2 patients carrying known pathogenic variants in the TSC2 gene who exhibited an atypical, unilateral, iris coloboma associated with localized areas of retinal dysembryogenesis.

Muller cells seem to be important in maintaining foveal morphology through connections between their foot processes and the internal limiting membrane (ILM). Internal limiting membrane peeling causes Muller cell trauma. We hypothesized that leaving a rim of unpeeled ILM around idiopathic macular holes undergoing vitrectomy surgery would improve postoperative foveal morphology and vision.Prospective pilot study of fovea-sparing ILM peeling in a consecutive cohort of patients with macular holes over a 12-month period. Spectral-domain optical coherence tomography and Early Treatment Diabetic Retinopathy Study letters best-corrected visual acuity were assessed preoperatively and postoperatively, and foveal morphology and metamorphopsia postoperatively. The foveal sparing group was compared with a second consecutive cohort who received standard ILM peeling (control group).Thirty-four eyes of 34 patients were included in each group. Groups showed no significant preoperative differences. 34/34 holes were successfully closed with surgery in the foveal sparing group and 32/34 in the control group. The foveal sparing group showed better postoperative best-corrected visual acuity (67.7 vs. 63.8, P = 0.003) and best-corrected visual acuity improvement (25.1 vs. 20.2, P = 0.03). The foveal sparing group demonstrated thicker minimum foveal thickness (211 vs. 173 μm, P = 0.002) and less steep foveal depression (158 vs. 149, P = 0.002).Preserving nonpeeled ILM around macular holes resulted in a high closure rate, improved foveal morphology, and better postoperative best-corrected visual acuity. An appropriately powered randomized controlled study is warranted.

PURPOSE: To investigate the Stargardt disease phenotype associated with an unusually common and “extremely hypomorphic” ABCA4 variant, p.N1868I. METHODS: The charts of 27 patients with p.N1868I on one allele and a severe/deleterious mutation on the other allele were reviewed. Subjective age of onset, best-corrected visual acuity, and stage of disease were recorded for all 27 patients, 18 of whom had multiple visits. When available, fundus photography, spectral domain optical coherence tomography, fundus autofluorescence, full-field electroretinograms, Goldmann visual fields, and fluorescein angiography were included. Five families with multiple affected members were analyzed. RESULTS: The median age at symptom onset was 41.5 years, and 3 p.N1868I patients had not developed visual symptoms as of the most recent eye examination. Median best-corrected visual acuity in the better-seeing eye at baseline was 20/25(−2), and the median duration from symptom onset to legal blindness was 25 years. The five families described in this study demonstrated clinically significant intrafamilial variability, and affected family members who did not share the p.N1868I variant had relatively more severe phenotypes. CONCLUSION: This study demonstrates the consistency of foveal sparing, the variation in age at onset, the intrafamilial variability, and the prognosis with regard to visual acuity in p.N1868I-associated Stargardt disease.

SIGNIFICANCE Contour interaction describes an impairment of visual acuity produced by nearby flanking features, which exerts a significant impact in many clinical tests of visual acuity. Our results indicate that the magnitude of interaction depends either on the flanker contrast energy (i.e., the product of flanker contrast and width) or the flanker contrast alone, depending on the contrast energy of the flankers. PURPOSE The discrimination of acuity targets is impaired by the presence of nearby flanking contours, a phenomenon known as contour interaction. METHODS In this study, we measured percent correct identification for threshold size, high-contrast Sloan letters at the fovea and at 5° in the inferior visual field for different combinations of flanking-bar width, and Weber contrast corresponding to specific fixed values of contrast energy (width × contrast, in %-min arc). RESULTS For flanking bars with low-contrast energy, contour interaction exhibited no systematic dependence on the flanking-bar width. However, when the flanking bars had higher contrast energy, narrower high-contrast bars produced significantly greater contour interaction than wider bars of lower contrast. CONCLUSIONS The results are consistent with the interpretation that contour interaction depends primarily on the contrast energy of flanking contours when their contrast energy is low. As the contrast energy of the flanking contours increases, the magnitude of contour interaction depends on the flanker contrast. For high-contrast flanking contours, the magnitude of contour interaction saturates when the width of the flanking contours is approximately 20% of letter size.

PReCIS:: Superior oblique palsy (SOP) eyes show thinner inferotemporal retinal nerve fiber layer (RNFL) without adjusting for foveal position. There was a strong correlation between the degree of torsion and the difference in the RNFL thickness before and after adjusting foveal location. Purpose The impact of foveal position on RNFL thickness has been shown. In this study, we evaluate RNFL thickness profiles according to the disc-foveal angle in SOP and control eyes. Materials and methods In 44 eyes of 22 patients with unilateral congenital SOP and 42 eyes of 42 normal controls, the position of the fovea relative to the optic disc was calculated by optical coherence tomography using FoDi (fovea-to-disc) technology. After measuring RNFL thickness with FoDi alignment technology, each optical coherence tomography image was reevaluated with FoDi turned off, and the measurements were repeated to determine RNFL values according to the disc-foveal angle. Results The average disc-foveal angle was -10.85±6.60 degrees and -10.71±6.63 degrees in the affected and fellow eyes of SOP patients; these values were significantly greater than control subjects (-5.88±4.09 degrees). There was no significant difference in RNFL sector values between SOP and control eyes with FoDi. From all RNFL sectors, the measured inferotemporal thickness was less in SOP eyes without FoDi than in SOP eyes with FoDi (129.7±20.5 µm vs. 144.6±17.8 µm, respectively, P=0.001). Differences of the RNFL thicknesses with and without FoDi in SOP eyes in the superotemporal and inferotemporal sectors were 5.40±13.42 and 14.84±15.00, respectively, which were significantly more than the same changes in control eyes with amount of 0.30±6.57 and 8.52±10.4 (P=0.02 for both sectors). Conclusions In SOP eyes with large amounts of torsion, a correction for the disc-foveal angle is necessary for accurate determination of the RNFL thickness profile.

PURPOSE To assess foveal and parafoveal vasculature at the superficial capillary plexus, deep capillary plexus, and choriocapillaris of patients with X-linked retinoschisis by means of optical coherence tomography angiography. METHODS Six patients with X-linked retinoschisis (12 eyes) and seven healthy controls (14 eyes) were recruited and underwent complete ophthalmologic examination, including best-corrected visual acuity, dilated fundoscopy, and 3 × 3-mm optical coherence tomography angiography macular scans (DRI OCT Triton; Topcon Corp). After segmentation and quality review, optical coherence tomography angiography slabs were imported into ImageJ 1.50 (NIH; Bethesda) and digitally binarized. Quantification of vessel density was performed after foveal avascular zone area measurement and exclusion. Patients were additionally divided into "responders" and "nonresponders" to dorzolamide therapy. RESULTS Foveal avascular zone area resulted markedly enlarged at the deep capillary plexus (P < 0.001), particularly in nonresponders. Moreover, patients disclosed a significant deep capillary plexus rarefaction, when compared with controls (P: 0.04); however, a subanalysis revealed that this damage was limited to the fovea (P: 0.006). Finally, the enlargement of foveal avascular zone area positively correlated with a decline in best-corrected visual acuity (P: 0.01). CONCLUSION Prominent foveal vascular impairment is detectable in the deep capillary plexus of patients with X-linked retinoschisis. Our results correlate with functional outcomes, suggesting a possible vascular role in X-linked retinoschisis clinical manifestations.

PURPOSE To evaluate, in eyes with radiation maculopathy, the effect of 2-month-interval anti-vascular endothelial growth factor therapy on best-corrected visual acuity and foveal avascular zone (FAZ) enlargement using optical coherence tomography angiography. METHODS Consecutive treatment-naive patients with radiation maculopathy after proton beam irradiation for choroidal melanoma were retrospectively included. Clinical and optical coherence tomography angiography data at baseline and the 6-month visit were recorded. Two independent observers measured FAZ area manually on 3 × 3-mm optical coherence tomography angiography images of the superficial capillary plexus and deep capillary plexus. Patients were encouraged to follow strictly a 2-month-interval intravitreal anti-vascular endothelial growth factor treatment by either bevacizumab or ranibizumab. Findings were analyzed based on the adherence to the treatment scheme. RESULTS According to the adherence to the bimonthly anti-vascular endothelial growth factor treatment protocol, patients were categorized into 3 groups: treatment protocol (n = 19, strict adherence), variable intervals (n = 11, intervals other than 2 months), and no treatment (n = 11). The estimated radiation dose to the foveola in each group was 49 ± 16, 46 ± 17, and 46 ± 18 cobalt gray equivalent, respectively (P = 0.85). For the entire cohort, best-corrected visual acuity loss (P < 0.02) and FAZ enlargement (P < 0.0001) were observed over 6 months. Best-corrected visual acuity loss was significantly less pronounced in the treatment-protocol group than in the variable-interval and no-treatment groups (P = 0.007 and P = 0.004). The FAZ enlargement was lower in the treatment-protocol group compared with the variable-interval group for both superficial capillary plexus (P = 0.029) and deep capillary plexus (P = 0.03), and to the no-treatment group for the deep capillary plexus only (P = 0.016). CONCLUSION Decrease in best-corrected visual acuity and FAZ enlargement on optical coherence tomography angiography occurred over 6 months in eyes with radiation maculopathy and were significantly reduced under 2-month-interval anti-vascular endothelial growth factor therapy.

To investigate the surgical results and morphologic characteristics of macular hole (MH) and macular hole retinal detachment (MHRD) associated with extreme myopia.We retrospectively reviewed consecutive cases with axial length ≥28 mm who were treated with pars plana vitrectomy for MH or MHRD. The choroidal and scleral thickness at the fovea, presence of dome-shaped macula, and the height of posterior staphyloma 3 mm from the fovea were measured from postoperative optical coherence tomography images.Significant improvement in visual acuity was obtained postoperatively in both MH (16 eyes; 15 patients) and MHRD (19 eyes; 18 patients) groups (P0.05). Final MH closure rate was not significantly different between the groups (MH: 15/16, MHRD: 14/19, P = 0.19). Axial length was not significantly different between the groups (MH: 30.5 ± 1.5 mm, MHRD: 29.6 ± 1.3 mm, P = 0.098). Eyes with MH had significantly greater choroidal thickness (MH: 61.9 ± 66.0 μm, MHRD: 24.1 ± 19.8 μm, P = 0.045), greater scleral thickness (MH: 294 ± 77 μm, MHRD: 232 ± 89 μm, P = 0.008), higher frequency of dome-shaped macula (MH: 6/16, MHRD: 1/19, P = 0.032), and lower staphyloma height (MH: 190 ± 113 μm, MHRD: 401 ± 156 μm, P0.001).Surgical outcomes were generally favorable. The pathogenetic differences between the two conditions may be attributable to differences with respect to eye morphology.

To describe early and late morphological and functional changes in subjects receiving photodynamic therapy (PDT) for chronic central serous chorioretinopathy.Patients with chronic central serous chorioretinopathy were prospectively enrolled and received standard PDT. At the baseline examination, each subject underwent complete ophthalmological examination, including best-corrected visual acuity (BCVA) assessment, fluorescein angiography and indocyanine green angiography, spectral domain optical coherence tomography, and microperimetry. Spectral domain optical coherence tomography, microperimetry, and BCVA assessment were repeated in multiple sections over 7 days after PDT and at 1-, 3-, and 12-month intervals. Main outcome measures were: identification of early changes (1-week examination) in BCVA, retinal sensitivity, and spectral domain optical coherence tomography parameters and their influence on outcomes at the 1-year follow-up.Three main patterns of early response to PDT were identified during the 1-week examination. The neurosensory retinal detachment most frequently decreased rapidly (12/19 pts), with complete resolution in 50% of cases. An increase in neurosensory retinal detachment height was registered in 16% (3/19) of cases, whereas in 21% (4/19), a large fluctuation in neurosensory retinal detachment was encountered. Best-corrected visual acuity declined significantly in 5/12 patients in the first group and was stable or improved in the remaining cases. Overall, retinal sensitivity diminished in 16/19 subjects, with a mean worsening of 2.56 dB (P = 0.0002). At the 12-month examination, final mean BCVA improved by 14.4 letters (P = 0.001) and a similar progressive recovery in the retinal sensitivity was observed (+2.69 dB, P = 0.0039). The neurosensory retinal detachment completely resolved in 18/19 (95%) cases, with a parallel significant reduction in central foveal choroidal thickness (P0.0001).Three patterns of early response to standard PDT can be identified. Although an early and abrupt reduction in BCVA and retinal sensitivity after treatment is possible, this does not compromise a final improvement in visual functions.

To compare the 6-month efficacy of the intravitreal injection of conbercept or ranibizumab for patients with polypoidal choroidal vasculopathy (PCV).This is a retrospective case-control study involved 79 PCV eyes of 77 patients. The PCV eyes were treated with an intravitreal injection of either ranibizumab (n = 44) or conbercept (n = 35). Three monthly loading doses were injected and followed by retreatment as needed. The best-corrected visual acuity and angiographic characteristics were evaluated after 6 months.The mean logarithm of the minimum angle of resolution best-corrected visual acuity had improved from 0.86 (Snellen equivalent, 20/145) at baseline to 0.70 (Snellen equivalent, 20/100) at 6 months in the conbercept group (P0.001), and from 0.74 (Snellen equivalent, 20/110) at baseline to 0.63 (Snellen equivalent, 20/85) at 6 months in the ranibizumab group (P = 0.032), respectively. The central foveal thickness was decreased from 407 ± 146 μm to 230 ± 71 μm in the conbercept group (P0.001), and from 394 ± 93 μm to 208 ± 56 μm in the ranibizumab group (P0.001). Polyps were completely regressed and in 21 (47.7%) eyes in the conbercept group at 6 months, significant higher than in 10 (28.6%) eyes in the ranibizumab group (P = 0.029).Both conbercept and ranibizumab effectively increased the visual acuity and regressed the polyps of PCV eyes. No significant difference was found in the visual acuity improvement of the patients with PCV between the conbercept group and ranibizumab group at 6 months. However, conbercept was superior to ranibizumab monotherapy in the regression of polyps.

Purpose To demonstrate factors related to the presence of subfoveal detachment (SD) secondary to epiretinal membrane (ERM) and to characterize the predictors for the resolution of SD after membrane peeling. Methods A retrospective chart review was conducted for the patients who underwent pars plana vitrectomy for the idiopathic ERM peeling. Preoperative spectral domain optical coherence tomography characteristics of the eyes including central foveal thickness, foveal center point thickness, presence of intraretinal cyst, continuity of the membrane, area of the membrane within fovea (1 mm), and entire macula (6 mm) were evaluated to find the best predictors for the presence of SD. These predictors as well as perioperative parameters including use of internal tamponade and volume of SD were considered for time of resolution of SD. Results Of 158 included eyes, 20.2% eyes (32 eyes) had SD, and the presence of SD was significantly related to involvement of the membrane within the macula. After surgery, SD completely resolved in 90.6% of eyes (29 eyes) at a median of 2.97 months (range: 0.03-12.0 months). The area of the membrane within fovea was the only significant predictor for time to resolution of SD (hazard ratio = 1.20, 95% confidence interval = 1.100-1.324, P = 0.021). A small percent of eyes showed some further changes including fluctuation (6.2%) or persistence (3.1%) of fluid. Conclusion A larger extension of ERM over the macula is related to higher likelihood of the presence of SD. Time for resolution of subfoveal detachment does not seem to be affected by the preoperative and perioperative factors except the extent of membrane within 1,000 μm of the fovea.

PURPOSE To determine the preoperative factors influencing visual recovery after vitrectomy for myopic foveoschisis. METHODS Sixty-six eyes of 65 consecutive patients operated on for myopic foveoschisis were retrospectively included. All eyes underwent a preoperative ocular examination including best-corrected visual acuity (BCVA) and spectral domain optical coherence tomography with central foveal thickness measurement and foveal status classification: simple foveoschisis, foveal detachment, or macular hole. To study the impact of preoperative visual acuity, 4 visual acuity groups separated by quartile ranges were defined. Postoperative visits at 1, 3, or 12 months including BCVA measurement and optical coherence tomography were recorded. RESULTS Mean refraction was -15.90 diopters, mean axial length was 30.30 mm, mean central foveal thickness was 590 μm, and mean baseline logarithm of the maximum angle of resolution visual acuity was 0.68 (Snellen equivalent of 20/96). The final BCVA improved significantly from 3 months after surgery until the last follow-up visit; the mean logarithm of the maximum angle of resolution visual acuity at last follow-up was 0.43 (Snellen equivalent of 20/54). Mean central foveal thickness decreased significantly as soon as the first postoperative month (P < 0.0001). The preoperative BCVA was the only independent factor significantly correlated with the final BCVA as opposed to the foveal status (P < 0.0001). The mean BCVA and mean visual gain at the last follow-up visit were significantly different between the four visual acuity groups (P < 0.0001 and P = 0.017, respectively). CONCLUSION The main factor influencing the postoperative visual acuity is the preoperative visual acuity. Although the preoperative anatomical status seemed important in surgeon decision making, once normalized on visual acuity, it no longer influenced the postoperative visual acuity.

To characterize the stability or progression of different stages of hydroxychloroquine (HCQ) retinopathy up to 20 years after stopping the drug.We reviewed findings from 13 patients with initial HCQ retinopathy classified as early (patchy photoreceptor damage), moderate (ring of photoreceptor thinning or scotoma), or severe (retinal pigment epithelial [RPE] damage). Patients had been off HCQ for as many as 14 years at initial examination and were subsequently followed for 5 years to 8 years with repeated fundus autofluorescence and spectral domain optical coherence tomography.Early and moderate cases stabilized in fundus autofluorescence appearance, foveal thickness, ellipsoid zone line length, and visual acuity for up to 9 years after stopping HCQ. By contrast, severe cases demonstrated a continual loss of these parameters for up to 20 years off the drug. The presence of RPE damage at initial examination predicted progressive retinopathy over many years.The steady progression of severe HCQ retinopathy in eyes showing RPE damage after drug cessation suggests a metabolic insult that chronically destabilizes rather than destroys cellular function, with a clinical course resembling that of genetic dystrophies. Our findings stress the importance of early detection to minimize progression and visual loss.

PURPOSE To assess and compare early modifications in inner retinal layer thickness and optical coherence tomography angiography parameters in patients with diabetes mellitus (DM) Types 1 and 2 without clinical signs of diabetic retinopathy. METHODS Ninety eyes of 90 subjects (24 Type 1 DM, 36 Type 2 DM, and 30 healthy controls) were prospectively evaluated with spectral domain OCT, swept-source OCT angiography, and color fundus photography (on the same day). Retinal nerve fiber layer, ganglion cell layer (GCL+), and nerve fiber layer + GCL+ (GCL++) thickness were automatically determined by the instrument in the 1, 3, and 6 central mm. On OCT angiography, the following parameters were evaluated: area of foveal avascular zone, number of focally dilated endings of the capillaries (detected only on OCT angiography), presence of regular/irregular foveal avascular zone, capillary loss, and capillary network irregularities in the superficial capillary plexus (SCP) and deep capillary plexus (DCP). RESULTS Ganglion cell layer+ (P = 0.0099) and GCL++ (P = 0.0367) were significantly thicker in DM Type 1 versus DM Type 2 in 1 central mm, after adjustment for age and DM duration. The area of foveal avascular zone was significantly larger in DM Type 1 versus controls in both SCP and DCP and in DM Type 1 versus Type 2 only in DCP (P < 0.05 for all); the number of focally dilated endings of the capillaries was higher in DM Type 1 versus controls in both SCP and DCP (P < 0.01 for all); and in DM Type 2 versus controls only in DCP (P = 0.007). Perifoveal capillary loss in SCP and inner retinal layer thickness had the highest correlation in both DM types. CONCLUSION There are specific neural and microvascular modifications even before clinical signs of diabetic retinopathy in DM Types 1 and 2. Perifoveal capillary loss in the SCP is highly correlated with inner retinal layer. These data may help in characterization of patients at the preclinical stage of diabetic retinopathy.

PURPOSE To determine the size of the foveal avascular zone (FAZ) by optical coherence tomography angiography before and after idiopathic epiretinal membrane surgery. METHODS Thirteen consecutive patients (13 eyes) with unilateral epiretinal membrane were studied retrospectively. Optical coherence tomography angiography was used to measure the FAZ area within 3 mm × 3 mm scans of the superficial (superficial FAZ) and deep plexus layers (deep FAZ) before and 6 months after vitrectomy. The unaffected fellow eyes were used as controls. RESULTS The mean superficial and deep FAZ areas at 6 months after vitrectomy (0.080 ± 0.038 and 0.113 ± 0.045 mm, respectively) were significantly (P < 0.0001, P = 0.0035) larger than the corresponding mean preoperative FAZ areas (0.056 ± 0.030 and 0.082 ± 0.035 mm). However, the areas of FAZ expansion were small (0.024 ± 0.013 and 0.031 ± 0.031 mm). The mean postoperative superficial and deep FAZ areas were significantly (P < 0.0001, P < 0.0001) smaller than those of fellow eyes (0.295 ± 0.108 and 0.410 ± 0.142 mm). Multiple regression analysis showed that preoperative FAZ area had the highest correlation with postoperative FAZ area (P < 0.05). CONCLUSION This study showed horizontal contraction of the FAZ area in eyes with epiretinal membrane. Because preoperative FAZ area correlates with postoperative FAZ area, FAZ area may be a useful parameter for determining timing of surgery for epiretinal membrane.

Purpose To describe features characteristic of multiple evanescent white dot syndrome (MEWDS) using adaptive optics scanning laser ophthalmoscopy (AOSLO). Methods Six women (seven eyes) who presented with MEWDS between June 2014 and April 2017 underwent ophthalmologic examinations and multimodal imaging including infrared, AOSLO, and spectral domain optical coherence tomography. Results Bright hyperreflective lesions on AOSLO throughout the course of MEWDS could be correlated to the hyperreflective dots of foveal granularity on infrared imaging without apparent corresponding changes on spectral domain optical coherence tomography. During the acute phase of MEWDS, extrafoveal hyperreflective dots were also visible on AOSLO and infrared and were associated with accumulations of hyperreflective material above the retinal pigment epithelium on spectral domain optical coherence tomography. Conclusion Foveal granularity on conventional fundus imaging could be correlated with hyperreflective lesions visible on AOSLO. We hypothesize that these hyperreflective lesions, "Jampol dots," are the foveal corollaries of the same process associated with the classic "dot" lesions in MEWDS. Based on the intact photoreceptor mosaic on AOSLO, we surmise that this material is accumulating at the level of the retinal pigment epithelium.

To examine the characteristics of the foveal vascular structure of patients with retinopathy of prematurity (ROP) by optical coherence tomography angiography.Ten patients with a history of laser photocoagulation or cryopexy treatment for Stage 3 (Zone ≥ II) ROP and 10 normal subjects (controls) were included. Foveal avascular zone, vessel density, vessel length, and vascular diameter index were measured by optical coherence tomography angiography using the 3 × 3-mm Early Treatment Diabetic Retinopathy Study (ETDRS) sectors.The median foveal avascular zone values of the patients with ROP and controls were 0.103 mm and 0.260 mm, respectively (P = 0.0025). The medians of the vessel density, vessel length, and vascular diameter index of the patients with ROP were 0.218 mm/mm, 11.75 mm/mm, and 18.00 μm, respectively, in ETDRS Sector 1 and did not significantly differ from those of the controls (P = 0.940, 0.733, and 0.705, respectively). For the average of ETDRS Sectors 2 to 5, the medians of the vessel density, vessel length, and vascular diameter index for the patients with ROP were 0.347 mm/mm, 18.95 mm/mm, and 18.28 μm, respectively; vessel density and vessel length were significantly smaller than those of the controls (P = 0.002 and 0.003, respectively), but there was no significant difference in vascular diameter index (P = 0.286).Optical coherence tomography angiography-guided foveal avascular zone was significantly smaller in patients with ROP than in controls. Our results indicate that foveal vascular development may be altered in patients with a history of treatment-requiring ROP.

Supplemental Digital Content is Available in the Text. Infrared light two-photon excitation provides a new parameter for testing visual function, one that can be used in the detection and monitoring of ocular abnormality. Diabetic retinopathy significantly impairs retinal sensitivity to infrared light., Purpose: Human photoreceptors are sensitive to infrared light (IR). This sensitivity can be used as a novel indicator of retinal function. Diabetic retinopathy patients were assessed using in vivo two-photon excitation and compared their scotopic IR threshold with that of healthy patients. Methods: Sixty-two participants, 28 healthy and 34 with diabetic retinopathy, underwent a comprehensive eye examination, where visual acuity and contrast sensitivity were assessed. Infrared thresholds were measured in the fovea and parafovea following 30-minute dark adaptation. A two-photon excitation device was used with integrated pulsed laser light (1,045 nm) for sensitivity testing and scanning laser ophthalmoscopy for fundus imaging. Results: The mean Snellen visual acuity of diabetic patients (6/7.7) was worse than that of the healthy patients (6/5.5), which was significantly different (P < 0.001). Disease patients had decreased contrast sensitivity, especially at 6 and 18 cycles/degree. The mean retinal sensitivity to IR light in eyes with diabetic retinopathy (11.6 ± 2.0 dB) was significantly (P < 0.001) lower than that in normal eyes (15.5 ± 1.3 dB). Conclusion: Compared with healthy control subjects, the IR light sensitivity of diabetic patients was significantly impaired. Two-photon measurements can be used in the assessment of retinal disease, but further studies are needed to validate IR light stimulation in various stages of diabetic retinopathy.

PURPOSE To identify quantifiable markers of disease progression in patients with foveal-sparing atrophic late-onset retinal degeneration using fundus autofluorescence and spectral-domain optical coherence tomography imaging. METHODS Natural history study evaluating patients within a 3-year interval. Disease progression was assessed based on the area of retinal atrophy, macular topographic distribution of lesions, retinal and choroidal thickness and volume, and choroidal vascularity index. RESULTS Twenty-four eyes (12 individuals) were included for fundus autofluorescence, and 31 eyes (16 individuals) for spectral-domain optical coherence tomography studies. Measurements were symmetrical between eyes of the same patient. The area of atrophy significantly enlarged (P = 0.002), with a growth rate of 2.67 mm2/year (SD: 2.13; square rooted: 0.57 mm/year, SD = 0.34). Baseline area of atrophy and progression both correlated with age. Most atrophic lesions were found in the temporal macula and progressed nasally at follow-up. Central choroidal and retinal thicknesses and volume in late-onset retinal degeneration cases were significantly reduced compared with controls, but only central retinal thickness decreased significantly at follow-up. CONCLUSION This study identifies the area of atrophy and central retinal thickness, but not chorioretinal volume or choroidal thickness, as markers of short-term progression in late-onset retinal degeneration. These findings may be useful for disease monitoring and late-onset retinal degeneration interventional studies.

Purpose To compare 12-month treatment outcomes of Type 3 neovascularization among its different stages as classified using an optical coherence tomography-based method. Methods This retrospective observational study included 40 patients (40 eyes) who were newly diagnosed with Type 3 neovascularization. The patients were initially administered 3 monthly anti-vascular endothelial growth factor injections. Repeat treatment was performed when recurrence of fluid was noted. Disease staging was classified using the optical coherence tomography-based method. The best-corrected visual acuity at diagnosis and at 12 months and degree of change in best-corrected visual acuity were compared among the different stages of the disease. In addition, incidence of progression in the disease stages was estimated. Results Among the 40 patients, 14 (35.0%) were classified as Stage 2 and 26 (65.0%) were classified as Stage 3. The best-corrected visual acuity values at diagnosis and at 12 months were 0.61 ± 0.31 (20/81 Snellen equivalents) and 0.46 ± 0.30 (20/57) in the Stage 2 group and 0.67 ± 0.42 (20/93) and 0.70 ± 0.49 (20/100) in the Stage 3 group, respectively. There was a significant difference in best-corrected visual acuity change between the two groups (P = 0.036). During the follow-up period, 3 retinal pigment epithelium tears and 2 submacular hemorrhages had developed in the Stage 3 group. Progression of the disease from Stage 2 to Stage 3 was noted in 2 patients (14.3%). Conclusion The visual outcome was worse in Stage 3 than in Stage 2, and adverse events that may lead to abrupt visual deterioration developed only in Stage 3. Further studies are needed to reveal whether anti-vascular endothelial growth factor therapy can suppress the progression of the disease stages.

PURPOSE To determine the age distribution of children with Coats disease and the impact of age at diagnosis on the visual prognosis. METHODS Consecutive Coats disease cases aged 18 years or younger at diagnosis were retrospectively included. Clinical and imaging parameters were analyzed by comparative, correlation, survival, univariate, and multivariate statistics. RESULTS Ninety-eight patients were included. At diagnosis, mean age was 5.4 years ± 4.3 years (1 month-18 years). Younger age at diagnosis was correlated with more severe disease stage (P < 0.0001, r = -0.52), which was confirmed by survival analysis (P < 0.0001). Comparative analysis was performed between patients younger and older than 4 years at diagnosis. Leukocoria or strabismus was more frequent at presentation in patients younger than 4 years (P < 0.0001). Areas of peripheral nonperfusion and peripheral telangiectasia were more extensive at presentation in younger than older patients (P = 0.0003 and P = 0.039). Foveal sparing at diagnosis was less frequent in younger than older patients (2% vs. 23%, P = 0.002). The incidence of structural complications or enucleation during follow-up (mean duration: 5.9 years ± 4.5 years) was higher, and last-recorded visual acuity was lower in younger than older patients (P = 0.001 and P = 0.0009). Final logarithm of the minimal angle of resolution visual acuity was negatively correlated with age at diagnosis (P = 0.001, Spearman r = -0.42). Multivariate analysis indicated that disease stage (P < 0.0001), but not age at diagnosis (P = 0.07), independently influenced the last-recorded visual acuity. CONCLUSION Onset of Coats disease in children of younger age is associated with more severe manifestations, more advanced stage, and worse visual outcome. Age, correlated with disease stage, should be considered a prognostic marker in Coats disease.

Axial myopia is associated with elongation of the posterior ocular segment. The authors measured posterior fundus landmarks and assessed their associations with axial length.Using fundus photographs, the authors measured the vertical distance between the temporal superior and temporal inferior arterial arcade (VDA) and the angle kappa between the temporal arterial arcades among other morphometric variables.The study included 456 eyes with a mean age of 61.2 ± 14.2 years (range: 13-88 years) and mean axial length of 29.4 ± 2.1 mm (range: 23.2-35.3 mm). Mean angle kappa was 91.3 ± 17.2° (range: 39-161°), and mean VDA was 7.93 ± 1.71 mm (range: 2.72-12.85 mm). In multivariate regression analysis, wider angle kappa was associated (regression coefficient r: 0.47) with shorter axial length (P = 0.002; beta: -0.17; B: -1.37; 95% confidence interval [CI]:-2.23 to -0.51), longer VDA (P0.001; beta: 0.27; B: 2.70; 95% CI: 1.85-3.54), shorter disk-foveola distance (P0.001; beta: -0.22; B: -4.76; 95% CI: -7.05 to -2.46), shorter vertical optic disk diameter (P = 0.002; beta: -0.14; B: -6.83; 95% CI: -11.1 to -2.56), lower number of any chorioretinal lesions (P = 0.007; beta: -0.13; B: -2.11; 95% CI: -3.63 to -0.58), and longer maximal vertical chorioretinal lesion diameter (P = 0.05; beta: 0.09; B: 0.92; 95% CI: -0.02 to 1.86). A longer VDA was associated (r: 0.31) with longer axial length (P0.001; beta: 0.22; B: 0.18; 95% CI: 0.10-0.25), wider angle kappa (P0.001; beta: 0.28; B: 0.03; 95% CI: 0.02-0.04) and higher number of chorioretinal lesions (P = 0.03; beta: 0.10; B: 0.16; 95% CI: 0.02-0.31). If eyes with chorioretinal lesions were excluded, the association between longer VDA and longer axial length was no longer statistically significant (P0.10).Axial elongation was correlated with decreasing angle kappa, caused by an elongation of the disk-foveola distance because of an enlargement of the gamma zone, whereas VDA remained constant. By contrast, horizontal length of macular Bruch membrane and vertical length of macular Bruch membrane were independent of axial elongation. Axial elongation did not lead to lengthening of Bruch membrane in the macular region in eyes without macular chorioretinal lesions.

PURPOSE To measure the foveal avascular zone (FAZ) area after internal limiting membrane (ILM) peeling and to determine the factors significantly correlated with the FAZ area. METHODS This was a retrospective, observational, and cross-sectional study. The affected and normal fellow eyes of 102 patients with unilateral macular diseases and 169 healthy subjects were studied. The patients underwent successful vitrectomy with internal limiting membrane peeling for an epiretinal membrane (n = 56) or a macular hole (n = 46). The superficial FAZ area and average foveal (within 1 mm) thickness were measured. The main outcome measures were the en face FAZ area measured in the optical coherence tomography angiographic images. RESULTS The FAZ area in the epiretinal membrane group (0.148 ± 0.094 mm) and in the macular hole group (0.255 ± 0.111 mm) were significantly smaller than that in the healthy control group (0.358 ± 0.118 mm; all, P < 0.0001). Multiple regression analysis showed that a thicker fovea was significantly correlated with a smaller FAZ area in the epiretinal membrane group (r = -0.799, P < 0.0001), macular hole group (r = -0.473, P = 0.0042), and control group (r = -0.612, P < 0.0001). CONCLUSION The FAZ area after internal limiting membrane peeling was smaller than that of the controls. A smaller FAZ area was correlated with a thicker fovea both in internal limiting membrane-peeled eyes and normal eyes.

Purpose To measure the foveal avascular zone (FAZ) areas and vessel densities of patients with diabetic retinopathy and to study their relationship with diabetic cystoid changes and retinal thickness. Methods Prospective case series of 51 eyes of 31 patients with diabetic retinopathy. The eyes were grouped based on the presence or absence of cystoid edema and evaluated using optical coherence tomography angiography. The FAZ areas and vessel density were compared. Results The FAZ area at the superficial capillary plexus level was equal between the eyes with and without cystoid edema. Vessel density did not differ as well. There was no correlation with retinal thickness. In eyes with cystoid changes, FAZ area changes at the deep capillary plexus level were difficult to interpret. Conclusion The FAZ area and vessel density at the superficial capillary plexus level are reproducible and independent of the presence of cystoid edema.

PURPOSE To study correlations in patients with retinal vein occlusion between the automatically quantified macular vascular densities in the superficial and deep capillary plexus (DCP) obtained using optical coherence tomography angiography (OCTA) and the data from conventional examination, particularly visual acuity and peripheral retinal nonperfusion assessed using fluorescein angiography (FA). METHODS Retrospective, observational study of patients with retinal vein occlusion who underwent a comprehensive ophthalmic examination including FA and OCTA using the AngioVue OCTA system version 2015.100.0.35 (OptovueRTVue XR 100; AVANTI, Inc, Fremont, CA). Vascular densities in the superficial capillary plexus and DCP, as well as the area of the foveal avascular zone, were measured using the AngioAnalytics software. RESULTS Our study of 65 eyes of 61 patients (33 men, mean age: 67 years) showed a significant correlation between peripheral nonperfusion on FA and (1) automatically quantified global vascular density in both plexus (P = 0.021 for the DCP) and (2) foveal avascular zone area (P = 0.037). We also found significant correlations between capillary dropouts in both plexus and peripheral nonperfusion (P < 0.001 for both) and between visual acuity and vascular densities (P = 0.002 for the global density in the DCP). Global density less than 46% in the DCP was associated to the presence of peripheral nonperfusion area on FA (P = 0.003) and to enlargement of the superficial foveal avascular zone (P = 0.002). CONCLUSION Our study demonstrated a significant correlation between automatically quantified macular vascular density on OCTA and peripheral nonperfusion on FA; OCTA could help identify high-risk retinal vein occlusion patients who may benefit from further evaluation using FA.