Purpose To assess changes in retinal nonperfusion (RNP) in patients with retinal vein occlusion (RVO) treated with ranibizumab (RBZ) Design Secondary outcome measure in randomized double-masked controlled clinical trial Subjects Thirty-nine patients with central RVO (CRVO) and 42 with branch RVO (BRVO) Methods Subjects were randomized to 0.5mg or 2.0mg RBZ every month for 6 months and then re-randomized to pro re nata (prn) groups RBZ+scatter photocoagulation (laser) or RBZ alone for an additional 30 months. Main Outcome Measures Comparison of percentage of patients with increased or decreased area of RNP in patients with RVO treated with 0.5mg versus 2.0mg RBZ, during monthly injections versus prn RBZ, and in patients treated with prn RBZ versus prn RBZ+laser. Results In RVO patients given monthly injections of 0.5mg or 2.0mg RBZ for 6 months there was no significant difference in the percentage who showed reduction or increase in area of RNP. However, regardless of dose, during the 6 month period of monthly injections, a higher percentage of patients showed a reduction in area of RNP and a lower percentage showed an increase in area of RNP compared to subsequent time periods of prn RBZ treatment. After the 6 month period of monthly injections, BRVO, but not CRVO patients randomized to prn RBZ+laser showed significantly less progression of RNP compared to patients treated with prn RBZ. Conclusions Regardless of dose of ranibizumab (0.5mg or 2.0mg), monthly injections promote improvement and reduce progression of RNP compared to prn injections. Addition of scatter photocoagulation to prn RBZ may reduce progression of RNP in patients with BRVO, but a statistically significant reduction was not seen in patients with CRVO.

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Ultra-widefield imaging detects a higher stage of sickle cell retinopathy compared with clinical examination alone, but these differences may not be clinically significant.

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IMPORTANCE Incontinentia pigmenti (IP) is a rare, X-linked dominant disease with potentially severe ocular complications that predominantly affect the peripheral retina. However, little is known about its effects on the macula. OBJECTIVE To describe the structural and vascular abnormalities observed in the maculas of patients with IP and to correlate these findings with peripheral pathologies. DESIGN, SETTING, AND PARTICIPANTS Prospective, cross-sectional study at Wilmer Eye Institute, Johns Hopkins University. Five participants with a clinical diagnosis of IP were included and underwent multimodal imaging with ultra-wide-field fluorescein angiography (FA), spectral-domain optical coherence tomography (OCT), and OCT angiography. MAIN OUTCOMES AND MEASURES The structural and vascular abnormalities observed on spectral-domain OCT and OCT angiography and their correlation with peripheral pathologies seen on ultra-wide-field FA. RESULTS A total of 9 eyes from 5 patients (median age, 20.5 years; range, 8.4-54.2 years) were included. Median Snellen visual acuity was 20/32 (range, 20/16 to 20/63). ultra-wide-field FA-identified retinal vascular abnormalities in all 7 eyes in which FA was obtained. These abnormalities included microaneurysms, areas of nonperfusion, and vascular anastomoses, most of which were peripheral to the standard view of 30°FA with peripheral sweeps. Structural abnormalities were observed in 6 eyes on spectral-domain OCT, including inner retinal thinning and irregularities in the outer plexiform layer. Optical coherence tomography angiography abnormalities were noted in all 9 eyes, including decreased vascular density, abnormal vascular loops, and flow loss in the superficial and deep plexuses, which corresponded to areas of retinal thinning on spectral-domain OCT. CONCLUSIONS AND RELEVANCE Although our study is limited by the small sample size, the findings suggest that multimodal imaging is useful for detecting structural and vascular abnormalities that may not be apparent on ophthalmoscopy in patients with IP. Macular pathologies, especially a decrease in vascular density on OCT angiography, are common. Further studies are needed to characterize further the association between macular and peripheral abnormalities in patients with IP.

BackgroundWide-field imaging is a newer retinal imaging technology, capturing up to 200 degrees of the retina in a single photograph. Individuals with sickle cell retinopathy commonly exhibit peripheral retinal ischemia. Patients with proliferative sickle cell retinopathy develop pathologic retinal neovascularization of the peripheral retina which may progress into sight-threatening sequelae of vitreous hemorrhage and/or retinal detachment. The purpose of this review is to provide an overview of current and future applications of wide-field retinal imaging for sickle cell retinopathy, and recommend indications for best use.Main bodyThere are several advantages to wide-field imaging in the clinical management of sickle cell disease patients. Retrospective and prospective studies support the success of wide-field imaging in detecting more sickle cell induced retinal microvascular abnormalities than traditional non-wide-field imaging. Clinicians can easily capture a greater extent of the retinal periphery in a patient’s clinical baseline imaging to follow the changes at an earlier point and determine the rate of progression over time. Wide-field imaging minimizes patient and photographer burden, necessitating less photos and technical skill to capture the peripheral retina. Minimizing the number of necessary images can be especially helpful for pediatric patients with sickle cell retinopathy. Wide-field imaging has already been successful in identifying new biomarkers and risk factors for the development of proliferative sickle cell retinopathy. While these advantages should be considered, clinicians need to perform a careful risk–benefit analysis before ordering this test. Although wide-field fluorescein angiography successfully detects additional pathologic abnormalities compared to traditional imaging, a recent research study suggests that peripheral changes differentially detected by wide-field imaging may not change clinical management for most sickle cell patients.ConclusionsWhile wide-field imaging may not carry a clinically significant direct benefit to all patients, it shows future promise in expanding our knowledge of sickle cell retinopathy. Clinicians may monitor peripheral retinal pathology such as retinal ischemia and retinal neovascularization over progressive time points, and use sequential wide-field retinal images to monitor response to treatment. Future applications for wide-field imaging may include providing data to facilitate machine learning, and potential use in tele-ophthalmology screening for proliferative sickle retinopathy.

Purpose: This article describes pathological retinal vascular changes on multimodal imaging in patients with chronic leukemia. Methods: A prospective, observational study was conducted. Patients with chronic leukemia and concurrent leukocytosis were recruited from a tertiary-referral, academic medical center. Eligible patients received complete ophthalmic examinations and multimodal retinal imaging: spectral-domain optical coherence tomography (SD-OCT), optical coherence tomography angiography (OCTA), and ultra-widefield fluorescein angiography (UWF FA). Results: Six patients (11 eyes) were consecutively enrolled. At presentation, mean age was 56.9 years, median visual acuity was Snellen 20/20, and median leukocyte count was 114.5 K/mm3 (upper limit of normal: 11.00). Three of 11 eyes had SD-OCT abnormalities, including focal retinal thinning and hyperreflective material at the level of the retinal pigment epithelium. Five of 11 eyes showed OCTA abnormalities, including focal vascular flow loss and a diffuse decrease in vascular density. Four of 11 eyes had peripheral retinal abnormalities on UWF FA, including microaneurysms and nonperfusion. Five of 11 eyes showed pathological vascular changes on retinal imaging, and of these 5 eyes, 3 had unremarkable retinal examinations. Conclusions: Retinal vascular pathologies are frequently seen in patients with chronic leukemia and concurrent leukocytosis, though these patients are rarely symptomatic. Multimodal imaging allows for more sensitive detection of these pathologies compared with clinical examination alone. On OCTA, pathological vascular flow loss is more evident in the superficial capillary plexus. There may be a correlation between pathologies seen in the central and peripheral retina, but this observation will need to be confirmed by a future study with a larger cohort.