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Microvascular occlusions caused by sickle cell disease (SCD) can affect all ocular and orbital structures. Sickle cell retinopathy (SCR) is the most common ophthalmic manifestation of SCD. Fortunately, most individuals with SCR are visually asymptomatic. Vision loss in SCD most commonly occurs as a consequence of proliferative sickle cell retinopathy (PSR), in which pathologic retinal neovascularization occurs. To prevent significant vision loss and blindness, which can occur from complications of PSR, regular retinopathy surveillance screening examinations and consistent follow-up with a retina specialist are recommended. Scatter laser photocoagulation is the current gold-standard treatment to prevent vision threatening progression of PSR. Patients with sickle cell disease should have regular checkups with their dental care provider. Patients should be educated on the importance of proper dental care, a healthy diet, and the need for early intervention if they suspect any dental problems or are having dental pain. If any dental procedures that involve surgery or sedation are planned, it is critical to consult with the hematologist before the procedure is started. Prophylactic antibiotics may have to be prescribed before invasive dental procedures, such as extractions or periodontal surgery but is best determined by discussions between the dental care provider and the hematologist. Osteonecrosis is a highly prevalent skeletal complication of sickle cell disease that affects all genotypes. Risk factors for osteonecrosis include older age, HbSS genotype with concomitant alpha-thalassemia trait, frequent vaso-occlusive episodes, history of acute chest syndrome, elevated body mass index, and low white blood cell counts. Osteonecrosis causes progressive joint damage and associates with chronic pain, frequent acute care visits, and overall poor health-related quality of life. Current consensus guidelines recommend analgesics, physical therapy, and early consideration of joint arthroplasty in sickle cell-related osteonecrosis, although surgery may be deferred until late adolescence after growth plates have fused.

To determine the relationship between macular microvascular abnormalities on optical coherence tomography angiography and silent cerebral infarctions (SCIs) on cerebral magnetic resonance imaging in sickle cell disease.Patients (age18 years old) from our previous pediatric sickle cell disease study cohort who had prior optical coherence tomography angiography and brain magnetic resonance imaging were identified. Brain magnetic resonance imaging images were compared with macular optical coherence tomography angiography scans to identify macular vascular density differences between patients with SCI and without SCI.Sixty-eight eyes from 34 patients who underwent optical coherence tomography angiography were evaluated, of whom 28 eyes from 14 patients met the inclusion criteria for this study. Eight patients (57%) with SCI and 6 patients (43%) without SCI were identified. The mean age (17 years in SCI and 16.3 years in non-SCI) was comparable between groups. There was no statistically significant difference in systemic complications. Deep capillary plexus vessel density was lower in the temporal quadrant in patients with SCI (49.3% vs. 53.7%, P = 0.014).Patients with SCI were found to have lower vessel density in the deep capillary plexus compared with those without SCI. This finding suggests that deep capillary plexus vessel density may have utility as an imaging biomarker to predict the presence of SCI.

To determine if vision impairment (VI) is associated with food insecurity among the United States (US) adults.This is a cross-sectional study of US adults ≥18 years below a threshold of 150% poverty from the National Health Interview Survey (NHIS), years 2011-2018. Outcome measures included food insecurity status, based on response to the NHIS adult (10-item) food insecurity tool, either as a binary (food secure or insecure) or ordinal (high, marginal, low, and very low) variable. VI was defined as self-reported trouble seeing, even when wearing glasses or contact lenses. Multivariable logistic regression analyses adjusted for potential confounders examined associations between VI and food insecurity.Participants (N = 62075) were majority female (57%), White (62%), and non-Hispanic (74%). Of them, 16% reported VI and 28% were food insecure. In fully adjusted logistic regression models, adults with VI had 216% higher odds (95% CI = 2.01-2.31) of being food insecure than adults without VI. Further, there was a dose-response relationship between VI and food insecurity noted in a multinomial model: VI predicted 159% higher risk of marginal food security (95% CI = 1.44-1.75), 197% higher risk of low food security (95% CI = 1.80-2.16), and 295% higher risk of very low food security (95% CI = 2.69-3.22), as compared to high food security.VI is associated with food insecurity, increasingly so among adults with highest levels of food insecurity in this national sample of low-income US adults. This data highlights the need for targeted interventions to address and reduce the burden of food insecurity among US adults with VI.

Purpose: This work evaluated the use and type of dietary supplements and home monitoring for nonneovascular age-related macular degeneration (AMD), as well as the prevalence of genetic testing among patients with AMD. Methods: A cross-sectional study was conducted of 129 participants older than 50 years who completed self-administered questionnaires regarding usage and type of dietary supplements and home monitoring, as well as the participants’ use of genetic testing for AMD. Results: Of 91 participants with AMD, 83 (91.2%) took vitamins, including 55 (60.4%) who used an Age-Related Eye Disease Study (AREDS) or AREDS2 formulation. Of 38 without AMD, 31 (81.6%) took vitamins (difference from participants with AMD = 9.6% [95% CI, 0%-23.2%]), including 2 on an AREDS formulation. Among 82 participants with AMD who were AREDS candidates (intermediate or advanced AMD in 1 or both eyes), 51 (62.2%; 95% CI, 51.7%-72.7%) took an AREDS or AREDS2 formulation, and 31 (37.8%) did not (5 were unsure). Additionally, 50 (61.0%; 95% CI, 50.4%-71.6%) AREDS candidates did some type of home monitoring. Only 1 (1.2%; 95% CI, 0%-3.6%) underwent genetic testing for AMD. Among 9 with AMD who were not AREDS candidates, 4 (44.4%) used an AREDS formulation, 4 (44.4%) did not, and 1 (11.1%) was unsure; only 1 (11.1%) of these 9 performed home monitoring. Conclusions: Despite similar results from past surveys and AREDS2 data supporting supplement use in 2013 and home monitoring in 2014, these findings suggest about one-third of AREDS candidates do not do so, providing further support for improving education regarding appropriate supplement and home monitoring usage. Genetic testing for AMD also appears infrequent.

Introduction: The aim of this study was to determine symptom-level risk factors for retinal tear/retinal detachment (RT/RD) in our patients presenting with symptoms of posterior vitreous detachment (PVD). Methods: We conducted a prospective cohort study of patients presenting to outpatient ophthalmology clinics at a single academic institution with complaint(s) of flashes, floaters, and/or subjective field loss (SFL). Patients received a standardized questionnaire regarding past ocular history and symptom characteristics including number, duration, and timing of flashes and floaters, prior to dilated ocular examination. Final diagnosis was categorized as RT/RD, PVD, ocular migraine, vitreous syneresis, or “other.” Simple and multivariate logistic regressions were used to identify symptoms predictive of various pathologies. Results: We recruited 237 patients (age 20–93 years) from March 2018 to March 2019. The most common diagnosis was PVD (141, 59.5%), followed by vitreous syneresis (38, 16.0%) and RT/RD (34, 14.3%). Of those with RT/RD, 16 (47.1%) had retinal tear and 15 (44.1%) had RD. Significant differences in demographic and examination-based factors were observed between these groups. Symptom-based predictive factors for RT/RD were the presence of subjective visual reduction (SVR; OR 2.77, p = 0.03) or SFL (OR 2.47, p = 0.04), and the absence of either floaters (OR 4.26, p = 0.04) or flashes (OR 2.95, p = 0.009). The number, duration, and timing of flashes and floaters did not predict the presence of RT/RD in our cohort. Within the RT/RD group, patients with RT were more likely to report floaters (100% vs. 66.7%, p = 0.018) and less likely to report SFL (0% vs. 86.7%, p < 0.001) compared to those with RD. Conclusion: While well-known demographic and exam-based risk factors for RT/RD exist in patients with PVD symptoms, the relative importance of symptom characteristics is less clear. We found that the presence of SVR and SFL, as well as the absence of either flashes or floaters, predicts RT/RD in patients with PVD symptoms. However, the number, duration, and timing of flashes and floaters may be less relevant in the triage of these patients.

Retinopathy of prematurity (ROP) represents a leading cause of childhood blindness. The purpose of our study was to evaluate incidence, trends in cost and length of hospital stay, and risk factors for ROP using a publicly available population-based dataset, the National Inpatient Sample.This cross-sectional study analyzed data from 2009 to 2018 using the National Inpatient Sample.Premature neonates (n = 717 277) who met the screening criteria for ROP with gestational age of ≤ 30 weeks or birthweight (BW) of ≤ 1500 g were identified.Database analysis.Incidence, demographics, risk factors for ROP development, trends in cost, and length of stay were evaluated.In total, incidence of ROP increased from 11% in 2009 to 15% in 2018 (P0.001). Multivariate logistic regression model of ROP development showed its associations with female sex (odds ratio [OR], 1.13; 95% confidence interval [CI], 1.10-1.17), Hispanic (OR, 1.10; 95% CI, 1.03-1.18), and Black (OR, 0.91; 95% CI 0.86-0.96) ethnicity. Neonates with lower BWs, particularly those in the 500- to 999-g subgroup (OR, 2.64; 95% CI, 2.44-2.85) and younger gestational ages, particularly those born between 25 and 28 weeks gestational age (OR, 2.41; 95% CI, 2.25-2.58), had increased risk of developing ROP. Comorbidities associated with the development of ROP were perinatal jaundice (OR, 1.84; 95% CI, 1.74-1.94), patent ducts arteriosus (OR, 1.67; 95% CI, 1.60-1.75), intraventricular hemorrhage (OR, 1.41; 95% CI, 1.35-1.48), perinatal infection (OR, 1.84; 95% CI, 1.74-1.94), and respiratory distress syndrome (OR, 1.05; 95% CI, 1.01-1.10).Retinopathy of prematurity develops in about 1 of 10 premature infants and incidence has been shown to be increasing. Significant risk factors were female sex, Hispanic ethnicity, lower BW, younger gestational age, and systemic comorbidities, including perinatal jaundice, patent ductus arteriosus, intraventricular hemorrhage, perinatal sepsis, and respiratory distress syndrome.Proprietary or commercial disclosure may be found after the references.

PurposeTo evaluate longitudinal quantitative ischaemic and vasculature parameters, including ischaemic index, vessel area, length and geodesic distance in sickle cell retinopathy (SCR) on ultra-widefield fluorescein angiography (UWFA).MethodsOptimal UWFA images from two longitudinal timepoints of 74 eyes from 45 patients with SCR were aligned and a common region of interest was determined. A deep-learning augmented ischaemia and vascular segmentation platform was used for feature extraction. Geodesic distance maps demonstrating the shortest distance within the vascular masks from the centre of the optic disc were created. Ischaemic index, vessel area, vessel length and geodesic distance were measured. Paired t-test and linear mixed effect model analysis were performed.ResultsOverall, 25 (44 eyes) patients with HbSS, 14 (19 eyes) with HbSC, 6 (11 eyes) with HbSthal and other genotypes were included. Mean age was 40.1±11.0 years. Mean time interval between two UWFA studies was 23.0±15.1 months (range: 3–71.3). Mean panretinal ischaemic index increased from 10.0±7.2% to 10.9±7.3% (pConclusionLongitudinal ischaemic index and retinal vascular parameter measurements demonstrate statistically significant progression in SCR. The clinical significance of these relatively small magnitude changes remains unclear but may provide insights into the progression of retinal ischaemia in SCR.

Background and objectives With the resumption of elective ophthalmic surgery during COVID-19, revised protocols were mandated to protect both staff and patients from transmission while increasing case numbers. We discuss a widely generalizable and in-depth protocol intended to safely allow the restart of elective procedures in 2 dedicated ophthalmic ambulatory surgical centers (ASCs) using monitored anesthesia care. Methods A single-center review of protocols and practices designed to limit COVID-19 transmission. Results All patients were tested within 72 hours prior to the procedure with a COVID-19 nasal swab to assess for active disease. A distance of 6 ft between each stretcher and the staff within the ASC was maintained when possible. Preoperative anesthetizing and dilating eye drops were administered from multiuse bottles without contact with surfaces. Surgical cases were restarted at a reduced capacity of a maximum of 7 per day to distance patient arrivals. Removal of waiting room chairs and the creation of new break areas allowed for social distancing. Conclusion As recommendations change on the basis of an increased understanding of the COVID-19 virus, ophthalmologists and ASC staff need to tailor protocols and workflows to limit transmission of virus with resumption of ocular surgery.

Purpose Patients with myopia are at increased risk for the development of glaucoma. The inability to correct for axial length on spectral-domain (SD) OCT translates into lower signal strength and scan reliability in patients with high axial myopia. We evaluated the effectiveness of a contact lens to increase the signal strength and to assess optic nerve dimensions and nerve fiber layer (NFL) thickness using SD OCT in patients with glaucoma or who are glaucoma suspects with high axial myopia. Design Single-center, prospective, interventional study. Participants Patients with axial lengths of more than 25.5 mm with a diagnosis of glaucoma or glaucoma suspect. Methods The optic nerve cube 200×200 scan using the Cirrus SD OCT 400 (Carl Zeiss Meditec, Inc., Dublin, CA) was carried out first without the use of a contact lens and then repeated with placement of the contact lens to correct for the spherical equivalent of the refractive error. Main Outcome Measures The primary outcome measure was the change in the average NFL thickness before and after use of the contact lens. Secondary outcome measures included the changes in cup volume, disc area, and rim area on OCT. Results Twelve patients were recruited (20 eyes); the average axial length was 27.06 mm, and the average signal strength interval increased by 1.73 (P = 0.001). With the use of a contact lens, the average NFL thickness was significantly thicker. None of the changes in the secondary outcome measures were significant: rim area, cup volume, or disc area. Conclusions Based on our data, the use of a contact lens statistically improved the signal strength and average NFL thickness of the SD OCT scan. The ability to capture the perimeter of the optic disc accurately can be limited in the setting of peripapillary atrophy, which was present in all but 2 participants. Future studies with a larger number of participants and a wider range of axial myopia to discern if contact lens correction has a greater effect on the highest axial lengths are needed.

To compare the severity of macular vascular changes in children with sickle cell disease (SCD) vs age- and race-matched controls.Cross-sectional study.Children (18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes, and their age- and race-matched controls, were recruited between January 2017 and December 2018. All subjects underwent optical coherence tomography angiography (OCTA) scans centered on the fovea and temporal macula. Retinal thickness, superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) size were measured and compared between HbSS and HbS variant vs controls.Thirty-four HbSS, 34 HbS variant (Goldberg staging 0-3 for SCD eyes), and 24 control eyes (total 48 children, aged 5-17 years) were included. Total VD (3-mm ETDRS circle) was lower in HbS variant eyes than in controls for both the SCP (42.9% vs 47.7%, P = .02) and DCP (47.4% vs 52.6%, P = .01). In HbSS eyes, VD was lower in the DCP (47.7%, P = .008) but not in the SCP (45.5%, P = .5), compared to controls. A higher proportion of HbSS (n = 18, 55%) than HbS variant eyes (n = 9, 26%) had pathologic areas of retinal thinning associated with SCP and DCP flow loss (P = .03). However, retinal thickness measurements and FAZ size did not differ between either HbSS or HbS variant group vs controls.Children with SCD have similar retinal thickness but less dense vasculature on OCTA compared to age and race-matched controls, suggesting that microvascular insult may precede structural thinning.

To characterize incidence rates and identify risk factors for admission and mortality in patients with endogenous endophthalmitis (EE) in the United States (US).Patients with EE were identified using the Nationwide Emergency Department (NEDS) Database from 2006 to 2017 in this cross-sectional study. Subjects were required to have diagnoses of both endophthalmitis and septicaemia using contemporary International Classification of Diseases diagnosis codes. Incidence rates, mortality rates and demographics were evaluated. Risk factors for admission and mortality were identified using weighted logistic regression analysis.A total of 6400 patients with EE were identified. Incidence increased from 0.10 (95% confidence interval [CI]: 0.07-0.12) per 100,000 in the US population in 2006 to 0.25 (95% CI: 0.21-0.30) in 2017 (p 0.05). Most were female (55.4%), insured with Medicare (53.5%), were in the first income quartile earnings (29.3%) [bottom 25% income bracket], lived in the South (40.5%), and presented to metropolitan teaching hospitals (66.6%). Mortality increased from 8.6% (95% CI: 3.8-18.3%) in 2006 to 13.8% (95% CI: 9.7-19.2%) in 2017 (p = 0.94). Factors predicting admission included older age (odds ratio [OR] 32.59; [95% CI 2.95-359.78]) and intravenous drug use (OR 14.90 [95% CI: 1.67-133.16]). Factors associated with increased mortality included: human immunodeficiency virus infection/immune deficiencies (OR 2.58 [95% CI: 1.26-5.28]), heart failure (OR 2.12 [95% CI: 1.47-3.05]), and hepatic infections/cirrhosis (OR 1.89 [95% CI: 1.28-2.79]). Pneumonia and renal/urinary tract infections (UTI) were associated with both increased hospital admission [(pneumonia OR 9.64 (95% CI: 1.25-74.35, p = 0.030), renal/UTI OR 4.09 (95% CI: 1.77-9.48)] and mortality [(pneumonia OR 1.64 (95% CI: 1.17-2.29, p = 0.030), renal/UTI OR 1.87 (95% CI: 1.18-2.97)]. Patients with diabetes mellitus (DM) had decreased odds ratio for mortality (OR 0.49 [95% CI: 0.33-0.73]).EE has increased in incidence throughout US. The two systemic factors that conferred both an increase in mortality and admission were pneumonia, and renal/UTI. Additional exploration of the potential protective association of DM with decreased mortality in this context is needed.

The proportions of individuals who are underrepresented in medicine (URiM) in ophthalmology lag behind their proportions within the US population. Diversity trends have been studied in other specialties, but literature examining trends in ophthalmology is lacking. In this study, we investigate trends in the racial and ethnic demographics of ophthalmology residents and residency applicants.Trend study.Demographic data of all residency programs across all specialties were extracted from Accreditation Council for Graduate Medical Education yearly reports from 2011 to 2019. Data from 2016 to 2019 on ophthalmology match applicants were analyzed using the 2020 Ophthalmology Residency Match Summary Report and the 2020 Association of University Professors of Ophthalmology Residency GenderEthnicity Summary. Linear trends were examined by the Cochran-Armitage test.From 2011 to 2019, the raw percentage of URiM ophthalmology residents increased from 4.7% (66/1419) to 5.8% (85/1473) (P.001) whereas that of surgical specialties decreased from 9.9% (2164/21,967) to 9.1% (2370/26,082) (P.001). The percentage of URiM ophthalmology match applicants increased from 5.9% (43/726) of all applicants to 11.8% (87/741) from 2016 to 2019 (P.001), and the percentage of URiMs matching into ophthalmology increased from 4.9% (23/467) of all participants to 10.8% (52/484) from 2016 to 2019 (P.001).Although there has been a positive trend in the percentage of URiMs applying and matching into ophthalmology, a lack of racial and ethnic diversity relative to other specialties persists. Strategies are needed to increase diversity in the field.

Purpose To determine whether ultra-widefield (UWF) retinal imaging changes the staging or management of sickle cell retinopathy compared with clinical examination. Methods Prospective, observational study including patients with sickle cell disease. All patients underwent dilated fundus examination by a fellowship-trained retina specialist, as well as UWF fundus photography (FF) and fluorescein angiography (FA). Sickle retinopathy stage and treatment recommendation per eye were determined after clinical examination, UWF-FF, and UWF-FA, respectively, and differences in retinopathy stage and treatment recommendation were compared. Results A total of 70 eyes from 35 patients (17 women, 48.6%), mean age 30.4 years, were included. Sickle genotypes included 26 patients with sickle SS (74.3%), 7 SC (20.0%), and 2 β(+)thalassemia (5.7%). Based on examination, most eyes (42/70; 60.0%) had no visible retinopathy. Based on UWF-FF, about half of the eyes were found to be Goldberg Stage 2 or above (36/70; 51.4%). Based on UWF-FA, nearly all eyes were Goldberg Stage 2 or above (63/70; 90%). However, clinical examination reliably detected neovascularization, and in no case did the addition of UWF imaging change management relative to examination alone. Conclusion Ultra-widefield imaging detects a higher stage of sickle cell retinopathy compared with clinical examination alone, but these differences may not be clinically significant.

Therapies targeting VEGF have proven only modestly effective for the treatment of proliferative sickle cell retinopathy (PSR), the leading cause of blindness in patients with sickle cell disease. Here, we shift our attention upstream from the genes that promote retinal neovascularization (NV) to the transcription factors that regulate their expression. We demonstrated increased expression of HIF-1α and HIF-2α in the ischemic inner retina of PSR eyes. Although both HIFs participated in promoting VEGF expression by hypoxic retinal Müller cells, HIF-1 alone was sufficient to promote retinal NV in mice, suggesting that therapies targeting only HIF-2 would not be adequate to prevent PSR. Nonetheless, administration of a HIF-2–specific inhibitor currently in clinical trials (PT2385) inhibited NV in the oxygen-induced retinopathy (OIR) mouse model. To unravel these discordant observations, we examined the expression of HIFs in OIR mice and demonstrated rapid but transient accumulation of HIF-1α but delayed and sustained accumulation of HIF-2α; simultaneous expression of HIF-1α and HIF-2α was not observed. Staggered HIF expression was corroborated in hypoxic adult mouse retinal explants but not in human retinal organoids, suggesting that this phenomenon may be unique to mice. Using pharmacological inhibition or an in vivo nanoparticle-mediated RNAi approach, we demonstrated that inhibiting either HIF was effective for preventing NV in OIR mice. Collectively, these results explain why inhibition of either HIF-1α or HIF-2α is equally effective for preventing retinal NV in mice but suggest that therapies targeting both HIFs will be necessary to prevent NV in patients with PSR.

Diabetic retinopathy is a progressive disease and primary retinal vascular complication of diabetes mellitus, the third leading cause of blindness in the United States. Racial and ethnic minorities are more likely to suffer from diabetic retinopathy and diabetic macular edema, and typically undergo less screening. Lack of screening is due to a variety of factors, such as patient, provider, and institutional barriers. Due to the disparity in screening, minorities often present with more advanced stages of diabetic eye disease. As the minority population increases, the burden of treatment for these patients will also increase. It is imperative to understand the barriers and social determinants of health limiting visual outcomes in minority populations. Diabetic retinopathy and its complications are often preventable if detected and treated early. Advances in screening technology and intravitreal anti-VEGF injections have changed the landscape in preventing vision loss in diabetic patients.

Purpose: The purpose of this study is to determine the sensitivity and specificity of detecting age-related macular degeneration (AMD) using portable optical coherence tomography (OCT) operated by nonexpert photographers on undilated patients. Methods: In this case-control study, 92 individuals were recruited from the glaucoma and retina clinics at the Wilmer Eye Institute (Johns Hopkins University, Baltimore, MD). Using the portable iVue (Optovue, Inc, Fremont, CA) spectral-domain OCT (SD-OCT), 2 nonexpert photographers acquired retina map scans on undilated eyes of all participants. In total, 33 AMD eyes and 105 control eyes were evaluated and graded by ophthalmologists masked to the diagnoses. Results: Detection of AMD on the portable OCT by ophthalmologists exhibited sensitivities of 0.91 and 0.94 and specificities of 0.88 and 0.89, for graders 1 and 2, respectively. A strong intergrader agreement was observed (κ = 0.87). Conclusions: Nonexpert photographers can use a portable OCT on undilated eyes to acquire images for the detection of AMD. These findings present the potential utility of implementing a portable OCT in community screenings for earlier detection and treatment of disease.

Purpose To determine the feasibility and accuracy of non-mydriatic ultra-widefield (UWF) fundus photographs taken in a hematology clinic setting for screening of sickle cell retinopathy (SCR) Design Prospective cohort study Methods Setting: Single-site study at the Johns Hopkins Sickle Cell Center for Adults and the Wilmer Eye Institute Study Population: Ninety eyes of 46 consecutive adult participants with sickle cell disease (SCD) Observation Procedure: Bilateral non-mydriatic fundus photos taken by clinic personnel during the participants’ routine hematology appointment were graded by two masked retina specialists at the Wilmer Eye Institute for the presence of non-proliferative SCR (NPSR) and proliferative sickle retinopathy (PSR). A third retina specialist adjudicated in cases of grader disagreement. All participants underwent the standard dilated fundus exam (DFE) within two years of acquisition of UWF photos. Main Outcome Measure: Sensitivity and specificity of non-mydriatic UWF images for the detection of NPSR and PSR Results PSR was noted in 19 of 90 eyes that underwent DFE and 9 of 67 gradable UWF images. Inter-rater agreement between the two graders was moderate with κ = 0.65 (0.43-0.87) for PSR. For gradable UWF photos, the sensitivity and specificity of detecting SCR using the non-mydriatic photos compared to the DFE were 85.2% and 62.5% for NPSR, respectively, and 69.2% and 100% for detection of PSR, respectively. One participant required ophthalmic therapy in both eyes for active sea-fan neovascularization. Conclusions UWF imaging shows utility in screening for SCR and may help identify patients with PSR who require a DFE and who may benefit from treatment.

Importance Adherence to screening for vision-threatening proliferative sickle cell retinopathy is limited among patients with sickle cell hemoglobinopathy despite guidelines recommending dilated fundus examinations beginning in childhood. An automated algorithm for detecting sea fan neovascularization from ultra-widefield color fundus photographs could expand access to rapid retinal evaluations to identify patients at risk of vision loss from proliferative sickle cell retinopathy. Objective To develop a deep learning system for detecting sea fan neovascularization from ultra-widefield color fundus photographs from patients with sickle cell hemoglobinopathy. Design, Setting, and Participants In a cross-sectional study conducted at a single-institution, tertiary academic referral center, deidentified, retrospectively collected, ultra-widefield color fundus photographs from 190 adults with sickle cell hemoglobinopathy were independently graded by 2 masked retinal specialists for presence or absence of sea fan neovascularization. A third masked retinal specialist regraded images with discordant or indeterminate grades. Consensus retinal specialist reference standard grades were used to train a convolutional neural network to classify images for presence or absence of sea fan neovascularization. Participants included nondiabetic adults with sickle cell hemoglobinopathy receiving care from a Wilmer Eye Institute retinal specialist; the patients had received no previous laser or surgical treatment for sickle cell retinopathy and underwent imaging with ultra-widefield color fundus photographs between January 1, 2012, and January 30, 2019. Interventions Deidentified ultra-widefield color fundus photographs were retrospectively collected. Main Outcomes and Measures Sensitivity, specificity, and area under the receiver operating characteristic curve of the convolutional neural network for sea fan detection. Results A total of 1182 images from 190 patients were included. Of the 190 patients, 101 were women (53.2%), and the mean (SD) age at baseline was 36.2 (12.3) years; 119 patients (62.6%) had hemoglobin SS disease and 46 (24.2%) had hemoglobin SC disease. One hundred seventy-nine patients (94.2%) were of Black or African descent. Images with sea fan neovascularization were obtained in 57 patients (30.0%). The convolutional neural network had an area under the curve of 0.988 (95% CI, 0.969-0.999), with sensitivity of 97.4% (95% CI, 86.5%-99.9%) and specificity of 97.0% (95% CI, 93.5%-98.9%) for detecting sea fan neovascularization from ultra-widefield color fundus photographs. Conclusions and Relevance This study reports an automated system with high sensitivity and specificity for detecting sea fan neovascularization from ultra-widefield color fundus photographs from patients with sickle cell hemoglobinopathy, with potential applications for improving screening for vision-threatening proliferative sickle cell retinopathy.

A questionnaire regarding sickle cell retinopathy evaluation and management distributed to retina specialists revealed variable practice patterns and a need for clinical diagnostic and treatment guidelines.

Objectives To demonstrate the feasibility of a deep learning-based vascular segmentation tool for UWFA and evaluate its ability to automatically identify quality-optimized phase-specific images. Methods Cumulative retinal vessel areas (RVA) were extracted from all available UWFA frames. Cubic splines were fitted for serial vascular assessment throughout the angiographic phases of eyes with diabetic retinopathy (DR), sickle cell retinopathy (SCR), or normal retinal vasculature. The image with maximum RVA was selected as the optimum early phase. A late phase frame was selected at a minimum of 4 min that most closely mirrored the RVA from the selected early image. Trained image analysts evaluated the selected pairs. Results A total of 13,980 UWFA sequences from 462 sessions were used to evaluate the performance and 1578 UWFA sequences from 66 sessions were used to create cubic splines. Maximum RVA was detected at a mean of 41 ± 15, 47 ± 27, 38 ± 8 s for DR, SCR, and normals respectively. In 85.2% of the sessions, appropriate images for both phases were successfully identified. The individual success rate was 90.7% for early and 94.6% for late frames. Conclusions Retinal vascular characteristics are highly phased and field-of-view sensitive. Vascular parameters extracted by deep learning algorithms can be used for quality assessment of angiographic images and quality optimized phase selection. Clinical applications of a deep learning-based vascular segmentation and phase selection system might significantly improve the speed, consistency, and objectivity of UWFA evaluation.

Purpose To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). Design Retrospective case series. Participants Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated vision loss attributed to AMN (n = 2) or PAMM (n = 2). Methods Clinical characteristics including best-corrected visual acuity and multimodal imaging features from fundus photography, fluorescein angiography, OCT, and OCTA were analyzed. Main Outcome Measures Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined. Results In 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 patient, the lesion was located in the foveal avascular zone, and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. Conclusions To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.

PurposeDescribe the demographics of new patients with uveitis presenting to an urban, academic centre and affiliated, suburban satellite clinics to assess if changes in infrastructure were needed for clinical care. Secondarily, examine the frequency of infectious uveitides.MethodsA retrospective chart review of single academic centre of 436 consecutive, new patients with uveitis (686 eyes) and 3 affiliated, satellite clinics seen by 8 uveitis specialists from 1 July 2013 to 31 March 2017. Demographics recorded included patient age, race, associated systemic disease, uveitis chronicity, and anatomic location. The main outcome measure was comparing frequencies of patient demographics, immunosuppressive agent use, and infectious uveitis between locations.Results366 patients (587 eyes) were evaluated at the academic clinic and 70 (99 eyes) at the satellite locations. Anterior uveitis was the most common anatomic location; more acute, unilateral cases were seen at satellites (p=0.007; p=0.002, respectively). A larger percentage of posterior and panuveitis cases presented to the academic centre (pConclusionsAnterior uveitis comprised the majority of cases in both clinics. Non-corticosteroid immunosuppressive agents were used more frequently at the academic clinic, reflecting more cases of chronic posterior and panuveitis. Compounded intravitreal injections, specialised ophthalmic imaging studies and high-risk medication monitoring can be centralised in the academic clinic. Infectious uveitis cases were seen at both locations, with an increase in syphilis diagnoses at the academic centre.

This chapter details the clinical features of pediatric sickle cell retinopathy that a clinician may observe on dilated examination, optical coherence tomography (OCT), OCT angiography (OCTA), fluoresceine angiography (FA), and fundus photography. Current recommendations for the surveillance, prevention, and treatment of sickle cell retinopathy are given. These recommendations, based on expert consensus and peer-reviewed scientific literature, may help health care providers to meet the needs of their pediatric patients with sickle cell disease. This review is particularly applicable to eye care professionals and hematologists.

ObjectivesTo examine interocular asymmetry of foveal avascular zone (FAZ) and parafoveal capillary density metrics in sickle cell retinopathy (SCR) using optical coherence tomography angiography (OCT-A).MethodsThis cross-sectional, retrospective study evaluated SCR patients and unaffected controls who underwent 3x3mm macular OCT-A imaging using a spectral domain-OCT system. FAZ (area, perimeter, and acircularity index) and parafoveal capillary density metrics were computed for both eyes of each participant. In unaffected controls, interocular difference in FAZ and parafoveal capillary density metrics were evaluated using Bland-Altman plots. SCR patients with interocular difference outside the upper 97.5% and lower 2.5% limits of agreement from controls were defined as having interocular asymmetry. Area under receiver operating characteristic curve (AROC) was also performed to determine the ability of the absolute interocular difference to differentiate between subjects with SCR-including non-proliferative SCR (NP-SCR) and proliferative SCR (P-SCR)-and unaffected controls.ResultsThirty-one patients with SCR (21 NP-SCR and 10 P-SCR) and 14 race-matched and age-matched controls were included for analysis. Interocular asymmetry was seen for all FAZ and parafoveal capillary density metrics in NP-SCR and P-SCR subjects. SCR subjects showed greater disease severity in the left-eye for FAZ and parafoveal capillary density metrics.ConclusionsNP-SCR and P-SCR patients demonstrated quantifiable interocular asymmetry in FAZ and parafoveal capillary density metrics compared to unaffected subjects, with left-eye predominance in disease severity.

Purpose Retinal hemorrhages are observed frequently in patients with leukemia. However, little is known about the impact and natural history of these hemorrhages. The purpose of this study was to describe leukemic retinal hemorrhages using multimodal imaging and to monitor their evolution longitudinally. Design Retrospective case series. Participants A total of 11 eyes of 7 symptomatic leukemic patients with posterior segment hemorrhages. Methods Single-center study performed at the Johns Hopkins Hospital. Symptomatic leukemic patients with posterior segment hemorrhages underwent serial dilated fundus examinations. The hemorrhages were documented longitudinally with color fundus photographs and spectral-domain (SD) OCT. Main Outcomes Measures Microanatomic locations of leukemic retinal hemorrhages and their impact on vision and evolution over time. Results A total of 7 patients (71.4% men; 57.1% white, 28.6% black, and 14.3% Hispanic) were included, with 11 eyes showing posterior segment hemorrhages. The median age at presentation was 49.8 years. All patients had intraretinal hemorrhages; these involved the vitreous and sub–internal limiting membrane (ILM) space in 1 and 3 patients, respectively. The median total follow-up duration was 4.0 months. At the final follow-up visits, 4 of 6 patients showed complete resolution of hemorrhages on examination and color fundus photographs. The final SD-OCT images of all patients did not show any retinal thinning, disruption of the ellipsoid zone, disorganization of the retinal layers, intraretinal fluid, or subretinal fluid. Conclusions Symptomatic leukemic retinal hemorrhages are associated with anemia and thrombocytopenia. These hemorrhages, including visually significant central sub-ILM hemorrhages, tend to be self-limiting and resolve within a few months with treatment of the underlying disease.

Purpose: To report outcomes of intravitreal bevacizumab therapy for proliferative sickle retinopathy (PSR). Methods: A retrospective, interventional case series. Five eyes of 5 patients with PSR were managed with intravitreal bevacizumab therapy over a 13-year period at a single institution. Results: Four patients had sickle cell-hemoglobin SC disease and 1 had sickle cell-beta thalassemia disease. Four of the patients treated with intravitreal bevacizumab injection were treated for recurrent vitreous hemorrhage and 1 was treated for new peripheral sea fan neovascularization. In those patients treated for vitreous hemorrhage, there was improvement in visual acuity as early as 2 weeks after treatment. Only 2 of the patients had documented recurrent vitreous hemorrhage during the period of follow-up after the initial injection. In 1 patient, the vitreous hemorrhage did not recur until 13 months after the injection. All patients showed an anatomic response to intravitreal bevacizumab therapy with partial regression of the peripheral sea fan neovascularization. All patients tolerated the injections without any complications. Conclusions: Intravitreal bevacizumab injections appear to be well tolerated and may be an effective treatment of PSR. Regression of peripheral sea fan neovascularization and decreased duration of vitreous hemorrhage may be observed. Large-scale randomized controlled trials are needed to further clarify the role of bevacizumab in PSR.

To determine changes in quality of life measures when choroidal neovascularization (CNV) developed in the second eye of patients with initially unilateral neovascular age-related macular degeneration (AMD). We analyzed responses to the 39-item National Eye Institute Visual Function Questionnaire (NEI-VFQ), 36-item Short Form Health Survey (SF-36), and Hospital Anxiety and Depression Scale (HADS) at baseline, and prior to and following second eye CNV diagnosis in 92 participants enrolled in two Submacular Surgery Trials. Paired t-tests for sample sizes over 30 and Wilcoxon signed-rank tests for sample sizes

Background/aimsTo assess foveal avascular zone (FAZ) morphology and parafoveal capillary perfusion in patients with various stages of sickle cell retinopathy (SCR) using optical coherence tomography angiography (OCT-A).MethodsThis is a multi-institutional retrospective study of patients with various stages of SCR compared with healthy controls. Parafoveal OCT-A images obtained using a commercial spectral domain-OCT system were reviewed. Foveal-centred 3×3 mm full vascular slab OCT-As were used for image processing and data analysis. FAZ area, perimeter, and acircularity index were determined on the OCT-A image after manual delineation of the FAZ border. Quadrant-based parafoveal capillary density and per cent area deviating from normal distribution were also measured.ResultsFifty-two patients with SCR (33 non-proliferative and 19 proliferative) and 20 age and race-matched healthy controls were included. One randomly selected eye per study participant was analysed. FAZ perimeter and acircularity index were significantly greater in SCR eyes when compared with the controls. While parafoveal capillary density was significantly lower, per cent area deviated from normal distribution was significantly higher in SCR eyes than that of the control. However, no statistically significant difference between the two SCR stages was observed. In quadrant-based analysis, the temporal quadrant showed greater parafoveal capillary dropout due to SCR, with the most profound effect in patients with proliferative SCR.ConclusionsAbnormal FAZ morphology and altered parafoveal capillary perfusion were found in patients with SCR. Our customised OCT-A image analysis method uniquely highlights significant quantitative alterations in perfusion density mapping in a qualitative display, with minimal obscuration of OCT-A image detail.

Purpose To evaluate long-term visual and anatomic outcomes in patients with retinal vein occlusion (RVO) treated with anti–vascular endothelial growth factor (VEGF) agents. Design Prospective, interventional case series. Participants Patients with central RVO (CRVO) or branch RVO (BRVO). Methods Number of anti-VEGF injections and improvement from baseline best-corrected visual acuity (BCVA) and central subfield thickness (CST) were prospectively recorded in 40 eyes of 39 CRVO patients and 50 eyes of 47 BRVO patients. Results Mean follow-up was 58 months for BRVO and 78 months for CRVO. Within 6 months of last follow-up, 58% of BRVO patients and 75% of CRVO patients required anti-VEGF injections to control edema. Analysis of the course of each patient over time showed that for BRVO patients, BCVA letter score increased by a mean of 24, from baseline of 52 (20/100) to peak of 76 (20/32), and subsequently decreased by 13, to 63 (20/50), at final visit; and for CRVO patients, BCVA letter score increased by a mean of 26, from baseline of 48 (20/100) to peak of 74 (20/32), and subsequently decreased by 18, to 56 (20/80), at last follow-up. Loss from peak BCVA occurred primarily owing to persistent/recurrent edema and related foveal damage. Conclusions Patients with RVO showed large improvements in BCVA after initiation of anti-VEGF injections, but in many patients some visual gains were lost over time owing to bouts of recurrent edema. Sustained suppression of VEGF may help to provide optimal outcomes in RVO and reduce treatment burden.

IMPORTANCE: Determination of retinal thinning rates may help to identify patients who are at risk of progression of sickle cell retinopathy. OBJECTIVE: To assess the rates of macular thinning in adults with and without sickle cell retinopathy using spectral-domain optical coherence tomography (OCT) and to identify ocular and systemic risk factors associated with retinal thinning. DESIGN, SETTING, AND PARTICIPANTS: This longitudinal prospective case-control study enrolled adult participants from a university-based retina subspecialty clinic between February 11, 2009, and July 3, 2019. The study was designed in autumn 2008 and conducted from February 2, 2009, to July 3, 2020. Participants with sickle cell retinopathy (sickle cell group) were matched by age and race with participants without sickle cell retinopathy (control group). Participants received annual spectral-domain OCT and clinical examinations. Those with at least 1 year of follow-up by July 3, 2020, were included in the analysis. Data were analyzed from February 2, 2009, to July 3, 2020. MAIN OUTCOMES AND MEASURES: The primary outcome was comparison of spectral-domain OCT measurements from early-treatment diabetic retinopathy study subfield rates of retinal thinning between eyes with and without sickle cell retinopathy and between different sickle cell hemoglobin subtypes. The secondary outcome was identification of ocular and systemic risk factors associated with rates of retinal thinning. RESULTS: Among 370 adults (711 eyes) enrolled in the study, 310 participants (606 eyes) had sickle cell retinopathy, and 60 participants (105 eyes) did not. Of those, 175 of 310 participants (56.5%; 344 of 606 eyes [56.8%]; mean [SD] age, 37.8 [12.8] years; 126 women [72.0%]) in the sickle cell group and 31 of 60 participants (51.7%; 46 of 105 eyes [43.8%]; mean [SD] age, 59 [15.4] years; 22 women [71.0%]) in the control group had at least 1 year of clinical and spectral-domain OCT follow-up data from baseline. The mean (SD) follow-up was 53.7 (32.6) months for the sickle cell group and 54.6 (34.9) months for the control group. Rates of macular thinning in the sickle cell group were significantly higher than those in the control group for the inner nasal (difference, −1.18 μm per year; 95% CI, −1.71 to −0.65 μm per year), inner superior (difference, −1.03 μm per year; 95% CI, −1.78 to −0.29 μm per year), inner temporal (difference, −0.61 μm per year; 95% CI, −1.16 to −0.07 μm per year), and outer nasal (difference, −0.41 μm per year; 95% CI, −0.80 to −0.03 μm per year) quadrants. Patients with sickle cell hemoglobin SC and sickle cell hemoglobin β-thalassemia subtypes had higher rates of retinal thinning than those with the sickle cell hemoglobin SS subtype. Risk factors associated with greater rates of retinal thinning included participant age, stage of retinopathy, previous stroke, and presence of hypertension, acute chest syndrome, or diabetes. Hydroxyurea therapy was associated with decreased rates of retinal thinning and may be a protective factor. CONCLUSIONS AND RELEVANCE: In this study, rates of retinal thinning were higher among participants with sickle cell retinopathy compared with those without sickle cell retinopathy, and thinning rates increased with participant age and stage of retinopathy. These findings suggest that identifying anatomic worsening of sickle cell maculopathy through spectral-domain OCT may be a useful parameter to evaluate the progression of sickle cell retinopathy.

Sickle cell disease (SCD), the most common inherited blood disorder, is characterized by defective oxygen transport. Every part of the eye can be affected by microvascular occlusions from SCD; however, the major cause of vision loss is proliferative sickle cell retinopathy (PSR). Although individuals with the HbSS genotype of SCD manifest more systemic morbidity and those with the HbSC genotype have a milder clinical course, those with HbSC have an increased risk of developing PSR and resultant vision loss. Sickle cell retinopathy has a variable phenotype, even among individuals with the same genotype. Most patients with SCD maintain good vision because the associated retinopathy occurs in the retinal periphery, and any associated "sea fan" neovascularization has a high tendency to autoinfarct and regress. Vision loss from PSR is largely preventable via regular retinal examinations and treatment as indicated. Novel retinal imaging techniques such as wide-field fluorescein angiography, spectral domain optical coherence tomography, and optical coherence tomography angiography can identify evidence of retinal microvascular occlusions in most patients with SCD. Further study is necessary to discover which individuals are at highest risk for vision loss, which of these retinal imaging modalities is clinically important, and which systemic treatments may decrease risk of vision loss from sickle cell retinopathy.

Purpose The Diabetic Macular Edema Treated with Ozurdex (DMEO) Trial measured aqueous pro-permeability factors (PPFs) in diabetic macular edema (DME) patients before and after injection of dexamethasone implant or vascular endothelial growth factor (VEGF)-neutralizing protein and correlated changes in levels with changes in excess foveal thickness (EFT) to identify potential PPFs contributing to DME. Design Prospective, randomized crossover clinical trial. Methods Twenty DME patients randomized to dexamethasone implant or VEGF-neutralizing protein had aqueous taps and spectral-domain optical coherence tomography (SDOCT) at baseline and every 4 weeks for 28 weeks. Aqueous levels of 55 vasoactive proteins were measured with protein array. Crossover at week 16 provided changes in protein levels after each intervention in all 20 patients. Results After dexamethasone implant there was significant correlation between changes in levels of 13 vasoactive proteins with changes in EFT, including 3 known PPFs: angiopoietin-2 (r = 0.40, P = .001), hepatocyte growth factor (HGF; r = 0.31, P = .02), and endocrine gland-VEGF (EG-VEGF, r = 0.43, P Conclusions Correlation of edema reduction with reduction in the PPFs angiopoietin-2, HGF, and EG-VEGF provides potential insight into the multifactorial molecular mechanism by which dexamethasone implants reduce edema and suggest that additional study is needed to investigate the contributions of these 3 factors to chronic DME.

To identify associations between severity of sickle cell retinopathy (SCR) and other clinical, laboratory, or treatment factors relevant to sickle cell disease (SCD).Retrospective cohort study.We investigated clinical, laboratory, and demographic associations with the severity of SCR in 296 patients seen at both our SCD specialty clinic and our retina clinic. Multivariate multinomial logistic regression was used to estimate the association between each clinical variable and severity of SCR.Multivariate analysis showed that in patients with sickle cell anemia (SCA) genotypes, older age (95% confidence interval [CI], 1.04-1.15; P.001) and male sex (95% CI, 0.13-0.87; P = .02) were associated with proliferative sickle cell retinopathy (PSR). In patients with genotypic variants, visual symptoms (95% CI, 1.36-21.62; P = .02) were associated with PSR. Laser photocoagulation and vitrectomy surgery, the standard interventions for PSR, were associated with older age (95% CI, 1.05-1.13; P.001), visual symptoms (95% CI, 1.48-7.40; P = .004), higher hemoglobin level (95% CI, 1.14-1.65; P = .001), and no chronic transfusion (95% CI, 0.16-1.09; P = .08) across the whole cohort.These findings may inform clinicians of the symptoms, systemic findings, and disease-modifying therapies most frequently associated with SCR in SCD patients. Visual symptoms such as blurred vision or floaters were associated with progression of SCR and may be criteria for referral for retinal examination. Chronic transfusion therapy may be protective against the need for retinal laser photocoagulation or vitrectomy. Prospective studies are necessary to further explore risk factors for SCR and to identify which individuals with SCD are at risk for incident or progression of retinopathy.

Importance While symptomatic vitreomacular interface abnormalities (VIAs) are common, assessment of vision preference values and treatment preferences of these may guide treatment recommendations by physicians and influence third-party payers. Objective To determine preference values that individuals with VIA assign to their visual state and preferences of potential treatments. Design, Setting, and Participants In this cross-sectional one-time questionnaire study conducted between December 2015 and January 2017, 213 patients from tertiary care referral centers in Thailand, the United Kingdom, and the United States were studied. Patients with symptomatic VIA diagnosed within 1 year of data collection, visual acuity less than 20/20 OU, and symptoms ascribed to VIAs were included. Data were analyzed from January 2017 to November 2017. Main Outcomes and Measures The primary end points were overall mean preference value that individuals with VIA assigned to their visual state and patients’ preferences for potential treatments. Preference values were graded on a scale from 0 to 1, with 0 indicating death and 1 indicating perfect health with perfect vision. Results Of the 213 included patients, 139 (65.3%) were women, and the mean (SD) age was 65.6 (7.7) years. Diagnoses included epiretinal membrane (n = 100 [46.9%]), macular hole (n = 99 [46.5%]), and vitreomacular traction (n = 14 [6.6%]). The mean (SD) vision preference value was 0.76 (0.15), without differences identified among the 3 VIA types. More participants were enthusiastic about vitrectomy (150 [71.1%]) compared with intravitreal injection (120 [56.9%]) (difference, 14.2%; 95% CI, 5.16-23.3; P = .002). Adjusted analyses showed enthusiasm for vitrectomy was associated with fellow eye visual acuity (odds ratio, 10.99; 95% CI, 2.01-59.97; P = .006) and better-seeing eye visual acuity (odds ratio, 0.03; 95% CI, 0.001-0.66; P = .03). Overall enthusiasm for treatment was associated with fellow eye visual acuity (odds ratio, 7.22; 95% CI, 1.29-40.40; P = .02). Overall, most participants (171 [81.0%]) were enthusiastic about surgery, injection, or both. Conclusions and Relevance Study participants reported similar preference values among 3 types of VIAs. The data suggest that most patients with these conditions would be enthusiastic about undergoing vitrectomy or an injection to treat it, likely because of the condition’s effect on visual functioning, although there may be a slight preference for vitrectomy at this time.

Purpose This pilot study was conducted to assess optical coherence tomography (OCT) as a screening tool for glaucoma when used by nonexpert personnel. Methods This prospective case-control study included 54 patients with open-angle glaucoma and 54 age-matched comparison individuals. Optovue iVue SD-OCT imaging was performed by nonprofessional photographers on undilated patients. The sensitivity, specificity, negative predictive value, and positive predictive value of iVue scan parameters for detecting open-angle glaucoma were evaluated. Results The iVue cup to disc vertical ratio had a sensitivity of 0.96 [95% confidence interval (CI), 0.90-1.00] at 90% specificity and was strongly correlated with both the Cirrus HD-OCT cup to disc vertical ratio (Pearson coefficient=0.84) and the cup to disc ratio observed on dilated clinical examination by faculty ophthalmologists (Pearson coefficient=0.80). The retinal nerve fiber layer (RNFL) parameters performed robustly, but the ganglion cell complex parameters showed limited diagnostic value. The inferior quadrant thickness was among the best performing RNFL parameters, with a sensitivity of 0.87 (95% CI, 0.78-0.96) and a specificity of 0.88 (95% CI, 0.80-0.97) using the iVue normative database thresholds for abnormality. Conclusions OCT imaging may be performed by nonprofessional photographers on undilated patients, and quantitative parameters derived from the resultant images, particularly the vertical cup to disc ratio and the RNFL inferior quadrant thickness, demonstrate sensitivities and specificities that may be adequately robust for glaucoma screening in the community setting.