OBJECTIVE: To assess central and peripheral retinal and choroidal diseases using ultra-widefield (UWF) fundus imaging in combination with navigated central and peripheral cross-sectional and three-dimensional (3D) swept source optical coherence tomography (SS-OCT) scans.
METHODS: Retrospective study involving 332 consecutive patients, with a nearly equal distribution of males and females. The mean age of patients was 52 years (range 18-92 years). Average refractive error was -3.80 D (range +7.75 to -20.75 D).
RESULTS: The observations in this study demonstrate the efficacy of peripheral navigated SS-OCT in assessing various ocular conditions. The technology provides high-quality images of the peripheral vitreous, vitreoretinal interface, retina, and choroid, enabling visualization of vitreous floaters and opacities, retinal holes and tears, pigmented lesions, and peripheral retinal degenerations. 3D OCT scans enhance the visualization of these abnormalities and improve diagnostic and therapeutic decisions.
CONCLUSIONS: Navigated central and peripheral cross-sectional and 3D SS-OCT scans offer significant complementary benefits in the assessment and management of retinal diseases. Their addition to UWF imaging provides a comprehensive view of central and peripheral ocular structures, aiding in early detection, precise anatomical measurements, and objective monitoring of disease progression. In addition, this technology serves as a valuable tool for patient education, a teaching tool for trainees, and documentation for medico-legal purposes.

As globalization progresses, cases of sickle cell disease (SCD) are now being seen even in Japan, where SCD did not originally exist. SCD causes not only anemia but also peripheral blood flow obstruction, which can lead to systemic complications. This report represents a case of sickle cell retinopathy (SCR) in Japan discovered with the onset of retinal artery occlusion (RAO). The patient, a 20-year-old African-Japanese male, was being monitored for SCD at the Nagoya University Hospital, Pediatrics Department, Nagoya, Japan. Following a chest pain episode, he reported a loss of vision in his right eye and was referred to the ophthalmology department. Examination showed reduced visual acuity in the right eye 20/40 compared to the left 20/20. A Goldman visual field test indicated central vision loss in the right eye, and fundoscopic examination revealed yellow-white lesions centered on the macula and peripheral salmon-patch-like lesions in the right eye, with peripheral black sunburst-like lesions in the left eye. Optical coherence tomography (OCT) of the right eye showed inner retinal edema within the macula, suggesting an SCR accompanied by branch RAO. Six months later, he complained of further vision loss in his right eye. Examination and OCT revealed sub-inner limiting membrane hemorrhage in the right eye, suggesting worsening of the SCR. SCD is exceedingly rare among native Japanese but is likely to be encountered more frequently as globalization progresses. Even in countries where SCD has traditionally been rare, attention must be paid to the occurrence of severe SCR when managing SCD.

Oral ingestion of fluorescein can be done in ambulatory pediatric clinics. We show that oral ultra-widefield fluorescein angiography is a non-invasive approach to rapidly diagnose and manage a diverse set of pediatric retinal vascular diseases.

BACKGROUND: Sickle cell disease (SCD) is the commonest inherited blood disorder leading to complications occurring due to vaso-occlusion including sight-threatening retinopathy. Retinopathy can be managed if diagnosed early and vision loss can be prevented. Since, very less data are available from India, hence, this study was conducted in children (7-18 years) with SCD to diagnose retinopathy by using ocular coherence tomography (OCT) in subclinical stages.
METHODS: This cross sectional single-center study was performed in 7-18 years age group children with SCD without any visual symptoms. Enrolled participants underwent complete ophthalmological examination including macula and optic disc thickness measurements using Cirrus HD-OCT and results were analyzed.
RESULTS: Among 55 participants, none had visual impairment. Significant fundoscopy finding (nonproliferative sickle cell retinopathy/NPSR) was found in three patients (5.4%), thinning of central macula in four patients (7.27%), inner macula thinning in eight patients (14.5%), outer macula thinning in one patient (1.81%), retinal nerve fiber layer thinning in five patients (9%), ganglion cell layer to inner plexiform layer thinning in eight patients (14.54%). Overall NPSR was found in 5.4% patients detected with fundoscopy, whereas retinal layer thinning was found in 14 patients (25.4%) using OCT.
CONCLUSIONS: Despite of the significant prevalence of SCR, it is still underdiagnosed complication, leading to thinning of the retina from early ages; thus, its early diagnosis by regular screening using newer diagnostic methods can prevent progression to sight-threatening complications and provide better quality of life for these patients.

The impact of visual impairment in the context of sickle cell disease is poorly understood. Despite the significant advancements over the past three decades in retinal imaging and in the understanding of molecular mechanisms that drive retinal neovascularization, there has been little improvement in the management of proliferative sickle cell retinopathy. This article is co-authored by a patient impacted by proliferative sickle cell retinopathy. She highlights her personal experience of sight loss from proliferative sickle cell retinopathy and the impact on her daily life and mental health. Subsequent to diagnosis and management of proliferative sickle cell retinopathy, she continues to live with irreversible sight loss and provides crucial insight from a patient\'s perspective into the broad lack of high-quality educational materials online and lack of understanding of the disease within the clinical community. This article aims to provide a strong narrative to emphasize the need for further qualitative and quantitative research in this area, to bring about the holistic step-change required to improve visual outcomes and eyecare for people with sickle cell disease.

UNASSIGNED: To report a case of proliferative retinopathy with tractional retinal detachment associated with beta thalassemia minor in a 27-year-old female.
UNASSIGNED: A young lady having beta thalassemia minor presented with decreased vision in both eyes, the effect being more severe in her right eye. The patient\'s other systemic history, including ophthalmic history, was unremarkable. The fundus examination revealed peripheral retinal ischemia in both eyes and tractional retinal detachment in the right eye.
UNASSIGNED: Beta thalassemia minor is not associated with striking retinal pathology, nevertheless proliferative retinopathy. However, in this case the patient developed tractional retinal detachment that required surgery. This indicates that proliferative changes may develop in patients with beta thalassemia, and routine fundus examinations could be recommended for these patients.

This study aims to evaluate the readability and quality of Internet-based health information on sickle cell retinopathy.
Retrospective cross-sectional website analysis.
To simulate a patient\'s online search, the terms \"sickle cell retinopathy\" and \"sickle cell disease in the eye\" were entered into the top 3 search engines (Google, Bing and Yahoo). The first 20 results of each search were retrieved and screened for analysis. The DISCERN questionnaire, the Journal of the American Medical Association (JAMA) standards, and the Health on the Net (HON) criteria were used to evaluate the quality of the information. The Flesch-Kincaid Grade Level (FKGL), the Flesch Reading Ease (FRES), and the Automated Readability Index (ARI) were used to assess the readability of each website.
Of 16 online sources, 12 (75%) scored moderately on the DISCERN tool. The mean DISCERN score was 40.91 (SD, 10.39; maximum possible, 80). None of the sites met all of the JAMA benchmarks, and only 3 (18.75%) of the websites had HONcode certification. All of the websites had scores above the target American Medical Association grade level of 6 on both the FKGL and ARI. The mean FRES was 57.76 (±4.61), below the recommended FRES of 80 to 90.
There is limited online information available on sickle cell retinopathy. Most included websites were fairly difficult to read and of substandard quality. The quality and readability of Internet-based, patient-focused information on sickle cell retinopathy needs to be improved.

Hemoglobin C (HbC) disease is an uncommon disease that is generally considered benign, causing only occasionally painless hematuria, osteomyelitis, and dental abnormalities. Ocular manifestations have rarely been described in these patients. Here we report a novel ophthalmological manifestation of the disease. A 20-year-old woman presented with progressive visual loss in her right eye due to tractional retinal detachment. The left eye was apparently normal, but wide-field fluorescence angiography showed mild peripheral ischemia with multiple vascular abnormalities. Vitrectomy was performed and the systemic workup revealed the presence of hemoglobinopathy C in heterozygous form. HbC disease can be sight-threating due to retinal proliferation, similar to sickle cell retinopathy. Patients affected with this disease should undergo regular surveillance. Ultra-wide angiography is a helpful examination to detect peripheral ischemia in the earlier stages.

OBJECTIVE: To longitudinally assess macular thickness and microvascular changes in children with sickle cell disease (SCD).
METHODS: A retrospective consecutive series.
METHODS: Children with SCD aged ≤ 18 years who had an ophthalmic examination at Boston Children\'s Hospital between January 1998 and August 2022.
METHODS: Qualitative and quantitative analyses of both OCT and OCT angiography (OCTA) images were performed.
METHODS: Total retinal thickness measured on macular OCT, superficial capillary plexus and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6- × 6-mm OCTA scans.
RESULTS: International Classification of Diseases, 10th Revision, code search identified 303 pediatric SCD patients who underwent ophthalmic examination during the study period. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit, respectively. Overall, temporal retinal thinning was noted qualitatively in 35.6% of SCD patients at presentation and 39.6% at final visit. Of those patients with macular thinning, 94.6% and 90.5% had peripheral sickle cell retinopathy (SCR) at presentation and final visit. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in the fovea and temporal parafovea compared with HbSC (P < 0.05). Eyes with peripheral SCR had a larger FAZ at presentation compared with eyes without peripheral SCR (P = 0.004), a lower DCP VD at final visit in the inferior temporal macula (P = 0.03), and a higher DCP VD at final visit in the superior nasal macula (P = 0.01). Eighty eyes of 40 patients had OCT, and 34 eyes of 20 patients had both OCT and OCTA images acquired at both initial and final visits. At final visit, retinal thickness decreased at the fovea, inferior perifovea, and temporal perifovea compared with presentation (P < 0.05). In parallel, VD DCP in the superonasal quadrant increased at final visit (P = 0.03).
CONCLUSIONS: Macular retinal thinning was progressive and observed in eyes with and without peripheral SCR. Over time, there was a compensatory increase in DCP VD in the nasal macula on OCTA.
BACKGROUND: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

OBJECTIVE: To report treatment methods and visual outcome of treating proliferative sickle cell retinopathy (PSCR).
METHODS: Retrospective interventional.
METHODS: Review of PSCR eyes treated between 2017 to 2022. Patient demographics, fundus findings at presentation, genotype, PSCR stage, treatment used, and visual outcome were assessed.
RESULTS: 108 eyes of 88 consecutive patients were studied. Male: Female 48:40. Mean age: 38.91 (SD:12.52) years. Genotype: sickle cell haemoglobin C (SC) 83 eyes (76.9%), sickle cell haemoglobin S (SS) 19 eyes (17.6%), and sickle cell trait (AS) 6 eyes (5.5%). PSCR stages: 3: 15 eyes (11.0%), 4: 74 eyes (67.0%), and 5: 19 eyes (22.0%). Treatment methods: Intravitreal Injection (IVI) of anti-vascular endothelial growth factor (VEGF) only (27 eyes,25%), scatter retinal laser photocoagulation (SRLP) only (7 eyes, 6.5%), Vitrectomy + SRLP (29 eyes, 26.9%), IVI + SRLP (25 eyes, 23.1%), and Vitrectomy + IVI + SRLP (20 eyes, 18.5%). The treatment used correlated with PSCR stage (p = 0.000). IVI only was mostly used to treat stage 4 (81.4%), and SRLP only was used for stages 3 (42.9%) and 5 (57.1%). IVI + SRLP treated eyes had the best pre- and post-treatment vision. Vitrectomy + SRLP treated eyes had the most improved vision. SRLP only had least visual improvement. Fundus findings correlated with visual outcome (p = 0.003); but stage of PSCR, genotype and treatment used had no correlation (P > 0.05).
CONCLUSIONS: Several options effectively treat PSCR. Visual outcome improved or remained same in 90.7% of treated eyes. Randomized controlled trials will determine the optimum treatment for each distinct presentation of PSCR. Treatment guidelines and a disease classification system of prognostic value are unmet needs.