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Magella M Neveu,1,2 Srikanta Kumar Padhy,3 Srishti Ramamurthy,4 Brijesh Takkar,4 Subhadra Jalali,4 Deepika CP,4 Tapas Ranjan Padhi,3 Anthony G Robson1,2 1Department Electrophysiology, Moorfields Eye Hospital, London, EC1V 2PD, UK; 2Institute of Ophthalmology, University College London, London, UK; 3Anant Bajaj Retina Institute, LV Prasad Eye Institute, Bhubaneswar, India; 4Anant Bajaj Retina Institute, LV Prasad Eye Institute, Hyderabad, IndiaCorrespondence: Anthony G Robson, Department of Electrophysiology, Moorfields Eye Hospital, 162 City Road, London, EC1V 2PD, UK, Tel +44 20 7566 2554, Email anthony.robson3@nhs.netAbstract: Albinism describes a heterogeneous group of genetically determined disorders characterized by disrupted synthesis of melanin and a range of developmental ocular abnormalities. The main ocular features common to both oculocutaneous albinism (OCA), and ocular albinism (OA) include reduced visual acuity, refractive errors, foveal hypoplasia, congenital nystagmus, iris and fundus hypopigmentation and visual pathway misrouting, but clinical signs vary and there is phenotypic overlap with other pathologies. This study reviews the prevalence, genetics and ocular manifestations of OCA and OA, including abnormal development of the optic chiasm. The role of visual electrophysiology in the detection of chiasmal dysfunction and visual pathway misrouting is emphasized, highlighting how age-associated changes in visual evoked potential (VEP) test results must be considered to enable accurate diagnosis, and illustrated further by the inclusion of novel VEP data in genetically confirmed cases. Differential diagnosis is considered in the context of suspected retinal and other disorders, including rare syndromes that may masquerade as albinism.Keywords: albinism, visual electrophysiology, misrouting, foveal hypoplasia, VEP

Srikanta Kumar Padhy,1 Brijesh Takkar,2,3 Raja Narayanan,2 Pradeep Venkatesh,4 Subhadra Jalali2,5 1Vitreoretina and Uveitis Services, L V Prasad Eye Institute, Mithu Tulsi Chanrai Campus, Bhubaneswar, India; 2Srimati Kanuri Santhamma Center for Vitreoretinal Diseases, Kallam Anji Reddy Campus, L.V. Prasad Eye Institute, Hyderabad, India; 3Center of Excellence for Rare Eye Diseases, Kallam Anji Reddy Campus, L V Prasad Eye Institute, Hyderabad, India; 4Dr RP Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India; 5Jasti V. Ramanamma Childrens’ Eye Care Centre, Kallam Anji Reddy Campus, L V Prasad Eye Institute, Hyderabad, IndiaCorrespondence: Brijesh TakkarSrimati Kanuri Santhamma Center for Vitreoretinal Diseases, Kallam Anji Reddy Campus, L.V. Prasad Eye Institute, L V Prasad Marg, Banjara Hills, Hyderabad 500034, Telangana, IndiaTel +91-9868092215Fax +91-40-23548271Email britak.aiims@gmail.comAbstract: Gene therapy has now evolved as the upcoming modality for management of many disorders, both inheritable and non-inheritable. Knowledge of genetics pertaining to a disease has therefore become paramount for physicians across most specialities. Inheritable retinal dystrophies (IRDs) are notorious for progressive and relentless vision loss, frequently culminating in complete blindness in both eyes. Leber’s congenital amaurosis (LCA) is a typical example of an IRD that manifests very early in childhood. Research in gene therapy has led to the development and approval of voretigene neparvovec (VN) for use in patients of LCA with a deficient biallelic RPE65 gene. The procedure involves delivery of a recombinant virus vector that carries the RPE65 gene in the subretinal space. This comprehensive review reports the evidence thus far in support of gene therapy for LCA. We explore and compare the various gene targets including but not limited to RPE65, and discuss the choice of vector and method for ocular delivery. The review details the evolution of gene therapy with VN in a phased manner, concluding with the challenges that lie ahead for its translation for use in communities that differ much both genetically and economically.Keywords: gene therapy, voretigene neparvovec, Luxturna, Leber’s congenital amaurosis, retinal dystrophy

This report presents a case of a 22-year-old woman with bilateral giant choroidal caverns (CCs) in rod-cone dystrophy. Her best-corrected visual acuity was 20/80 in the right eye and 20/50 in the left eye. Fundus examination showed optic nerve pallor, narrowed blood vessels, and diffuse pigmentary changes. Fundus autofluorescence showed a central area of reduced autofluorescence surrounded by stippled autofluorescence. An electroretinogram showed decreased amplitude of both scotopic and photopic waves, consistent with a diagnosis of rod-cone dystrophy (retinitis pigmentosa). Optical coherence tomography revealed large CC and foveal atrophy in each eye. A giant CC alongside rod-cone dystrophy is a distinctive feature. [ Ophthalmic Surg Lasers Imaging Retina 2025;56:XX-XX.] .

Competing Interests: Disclosure: The authors have disclosed no potential conflicts of interest, financial or otherwise.

Purpose: To evaluate the safety and efficacy of fibrin glue in preventing early recurrence of vitreous hemorrhage following surgery for proliferative diabetic retinopathy (PDR)., Design: Single-masked randomized controlled clinical trial., Subjects: Consecutive patients with vitreous hemorrhage due to PDR undergoing primary vitreoretinal surgery were screened. After completing all vitreoretinal maneuvers including endocautery to bleeders, infusion pressure was gradually reduced to 5 mm Hg. Eyes with persistent bleeders at the macula and/or the optic nerve head not amenable to endocautery were included. Eyes with tractional retinal detachment more than 5-disc-diameter areas and/or retinal breaks were excluded., Methods: Twenty eyes were randomized into 2 groups. Controls were treated with conventional surgery, whereas cases were additionally treated with fibrin glue application over the posterior bleeding sites., Main Outcome Measures: Vitreous haze due to early recurrent vitreous hemorrhage was graded at weeks 1 and 4 as the main efficacy outcome measure. Absence of macular membranes at 3 months was the main safety outcome measure., Results: Mean age, frequency of preoperative anti-vascular endothelial growth factor injection or retinal photocoagulation, antiplatelet usage, and mean intraoperative blood pressures were similar between the 2 groups. Mean surgery duration (54.6 vs 51.6 minutes) and frequency of fibrous proliferation >2-disc-diameter areas (7 each) was similar. Fine dissection with forceps and/or scissors was required in more cases than controls (6 vs 3). Macular membranes were noted in 1 participant in each group. Mean grade of vitreous haze was significantly higher in controls than cases at week 1 (2.4 vs 0.6, P = .027) and week 4 (2 vs 0, P = .029). Cases had significantly better odds of having optically clear vitreous cavity at both week 1 (odds ratio [OR] 8.167, CI 1.02-64.03; P = .047) and week 4 (OR 33, CI 1.43-760.67; P = .029). Repeat retinal intervention for vitreous hemorrhage within 3 months was required in 5 of 10 controls, and in none of the cases., Conclusion: Our findings indicate fibrin glue application under direct visualization to be safe and efficacious in preventing recurrent vitreous hemorrhage for PDR in the early postoperative period., (Copyright © 2025 Elsevier Inc. All rights reserved.)

Purpose: To explore the relationship between gut microbiome, gut mycobiome, and intraocular (aqueous humor) microbiome dysbiosis in people with type 2 diabetes (T2DM) and diabetic retinopathy (DR)., Design: Multiple case-control studies., Methods: We evaluated three groups of people: healthy controls (HC), people with T2DM without retinopathy, and those with DR. The study samples included fecal matter (30-50 g) and aqueous humor (0.05-0.1 mL). After amplicon sequencing, we analyzed microbiome profiles (V3-V4 region of bacterial 16S rRNA gene) and mycobiome (ITS2 region of fungal rRNA gene). The main outcome measures were relative abundance, α and β diversity, and dysbiotic bacteria and fungi, analyzed based on the inferred functions of the taxa., Results: We recruited 82 people for gut microbiome (30 HC, 24 DM, and 28 DR); 75 people for gut mycobiome (30 HC, 21 DM, and 24 DR); and 12 people for aqueous humor microbiome (4 each HC, DM, and DR) studies. Generally, there was an increased abundance of pro-inflammatory and pathogenic microorganisms and a decreased abundance of anti-inflammatory and probiotic microorganisms. The differences were higher between HC and DM/DR than between DM and DR. In aqueous humor, there was a wider separation in microbiome profiles of people with DR than their gut microbiome., Conclusion: The gut and aqueous humor microbiota of people with diabetes and DR may differ from those without diabetes. Given these unique observations in individuals living in one region of India, further research involving people from different regions is required to identify indices for possible regional or global use., (Copyright © 2024 Indian Journal of Ophthalmology.)

Purpose: To evaluate the clinical profile of retinal artery occlusion (RAO) and impact of presentation on visual outcomes., Design: A retrospective analysis of case files of 3070 patients with RAO was performed using electronic medical records., Methods: Demographic data were analyzed using descriptive statistics. The differential distribution of risk factors of RAO with age was studied. Time to presentation and treatment effects were assessed using multivariate regression. Interaction plots were drawn to assess the impact of risk factors on outcomes., Results: Central RAO was the most common type (n = 2443, 77.11%), followed by branch RAO (n = 500, 15.78%), while combined retinal vascular occlusion and cilio-RAO were rare. Most of the patients (71.40%) were male and had unilateral (96.81%) affliction. Almost half presented within the fifth (24.85%) and sixth (21.4%) decades of life. Hyperhomocysteinemia had a higher association (1.95, P = 0.0019) with younger patients (<40 years), while hypertension (3.64, P < 0.001), diabetes mellitus (DM; 4.18, P < 0.001), and coronary artery disease (CAD) (4.26, P = 0.002) were significantly commoner in older patients. CAD (5.1%) and cerebrovascular disease (0.6%) were detected after ocular presentation in some patients. Embolus, though detected rarely (1%), was associated (60%) with serious systemic disorders. Early presentation (<6 h) was associated with better visual outcomes (mean log of minimum angle of resolution 1.8 ± 1.3 vs. 2.1 ± 1.3, P = 0.032). Neovascular glaucoma (2.14% overall) was twice more common in DM (P < 0.0004) and led to further vision loss. Visual improvement occurred in 10% of patients over follow-up., Conclusion: RAO occurs slightly earlier compared to coronary and cerebrovascular disorders and can precede their detection. Associated risk factors vary with the age of presentation. Presentation within 6 h needs facilitation for better outcomes and management., (Copyright © 2024 Indian Journal of Ophthalmology.)

Purpose: To assess the clinical phenotypes and genetic mutations in patients with Leber congenital amaurosis (LCA) from a tertiary eye care center in India., Design: Retrospective observational study., Methods: The study includes patients with a clinical diagnosis of LCA who underwent genetic testing from January 2016 to December 2021. The clinical exome of the patients was analyzed by targeted next-generation sequencing. The genetic variants found were classified as per standard American College of Medical Genetics and Genomics (ACMG) criteria and ClinVar database., Results: There were 35 patients (19 females, 16 males) of LCA. Family history was positive in 29% (10/35) and a history of consanguinity was noted in 54% (19/35) of the patients. The mean presenting best-corrected visual acuity was 2.48 ± 0.59 logMAR. Retinal pigment epithelial abnormalities and macular involvement were seen in 83% (58/70) and 23% (16/70) of the eyes, respectively, at presentation. The most common causative genes for LCA in our cohort were: GUCY2D (20%, 7/35), CRB1 (14%, 5/35), RPE65 (11%, 4/35), RPGRIP1 (11%, 4/35), and LCA5 (9%, 3/35). Autosomal recessive inheritance was seen in 94% (33/35). Macular involvement at presentation was seen in CRB1 (3/5), NMNAT1 (2/2), and one each of RPE65, LCA5, and RDH12 patients. The genetic testing cost was reduced from 23,800 INR to 15,000 INR per test in the study duration., Conclusions: Genetic screening of LCA cases identified various genotypes, with GUCY2D being the most common. Increased awareness and reduced costs of genetic testing would benefit both patients and caregivers. With promising clinical trial outcomes, genotyping is crucial for better patient selection and treatment., (Copyright © 2024 Indian Journal of Ophthalmology.)

Introduction: Bardet-Biedl Syndrome (BBS) is a ciliopathy causing developmental defects and progressive retinal dystrophy, whereas choroidal coloboma is a developmental defect causing structural deficiency in the posterior retina. Both are rarely reported together., Methods: Here, we describe the phenotype and genotype of three unrelated patients with co-occurrence of Bardet-Biedl Syndrome and chorioretinal coloboma and review the pertinent literature., Results: We describe three unrelated patients, with variable clinical features of Bardet Biedl syndrome. None had family history of BBS or coloboma. Each carried biallelic variants in BBS1 , BBS9 and TTC8 gene, respectively. Two had unilateral chorioretinal coloboma, while one had bilateral chorioretinal coloboma., Discussion: Although there may be other explanatory factors yet to be revealed, our data suggests that chorioretinal coloboma may be associated with BBS. The Hedgehog (Hh) signaling pathway, an intercellular communicator for development of the eye, is dependent on the primary cilia and plays a crucial role in the closure of the optic fissure. Both disorders therefore involve disruption of primary cilia function which may explain their association.

Objective: To profile a cohort of gyrate atrophy patients classified by widefield retinal imaging and correlate the structural, biochemical, and functional characteristics., Design: Retrospective observational cohort study., Participants: Sixty-five patients (129 eyes) with gyrate atrophy., Methods: Data of participants with a diagnosis of gyrate atrophy were retrieved from their electronic medical records (January 2015 to December 2023). Retinal involvement was classified into 3 zones using widefield retinal images. Zone 3 had atrophic patches in the area anterior to the equator; zone 2 had involvement limited to the arcades but posterior to the equator; zone 1 had involvement within the vascular arcades and/or peripapillary region, with or without any other zone involvement. Macular assessment was performed using swept-source OCT (n = 104). Flash electroretinogram (ERG) was performed in 40 eyes. Serum ornithine levels (n = 35) were measured, and genetic analysis was conducted (n = 18)., Main Outcome Measures: Demography, patient profile, zone of retina involved, macular features, and serum ornithine levels., Results: The average age at presentation was 26.4 (range, 5-67) years; the majority were male. Nyctalopia (n = 35, 53.8%) and blurred vision (n = 29, 44.6%) were the most common symptoms. Positive family history was reported in 32.3% of patients. Most eyes were myopic (69.8% <-3 diopters). Posterior subcapsular cataracts were documented in 36.4% of eyes. The highest frequency of retinal area affected was zone 1 (57.14%), followed by zone 2 (33.33%) and zone 3 (9.52%), correlating with the age at presentation. Foveoschisis was observed in 57.7% of eyes, with a higher prevalence in eyes with zone 1 disease. Elevated serum ornithine levels (>163 μmol/L) were found in 77.14% of patients. The ERG showed nonrecordable (n = 32) or severely reduced (n = 8) responses in scotopic and photopic phases. Genetic analysis of 18 patients identified mutations in the OAT gene, including a novel missense variant (c.290T>C)., Conclusions: This large cohort of patients with gyrate atrophy revealed symmetrical involvement, predominantly in zone 1. Most patients presented between the first and third decades, experienced nyctalopia, vision reduction, early posterior subcapsular cataracts, and varying degrees of myopia. Zone 1 involvement was strongly associated with foveoschisis and visual compromise., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To analyse baseline imaging characteristics and factors affecting poor visual acuity in diabetic papillopathy., Methods: This was a retrospective, observational study conducted at a tertiary eye care centre in eyes with a diagnosis of diabetic papillopathy. Demographic data including age, gender, duration of diabetes, type of diabetes, HbA1c values and other systemic co-morbidities were recorded. Baseline best corrected visual acuity (BCVA), and various imaging characteristics were noted., Results: 37 eyes of 22 patients with diagnosis of diabetic papillopathy were included [mean age of 46.6 ± 13.5 years, five (22.7%) females and 17 (77.3%) males]. Bilateral involvement was seen in 15 (68.2%) patients. The mean baseline BCVA was 0.51 ± 0.49 logMAR (Snellen equivalent 20/60). Two eyes (5.4%) had features of concurrent mild NPDR, 9 eyes (24.3%) had moderate NPDR, 10 eyes (27.03%) had severe NPDR while 16 (43.2%) other had PDR. All eyes had optic disc edema while 4 had disc pallor at presentation. Telangiectatic vessels/ neovascularisation of disc was present in 17 eyes (45.9%) detected either clinically or on optical coherence tomography (OCT) angiography. 11 (29.7%) eyes had a peripapillary cuff of subretinal fluid accompanying the disc edema. Sixteen eyes (43.2%) had centre involving macular edema seen on OCT. In eyes with a macular edema, the mean central macular thickness was 407.4 ± 71.42 microns at baseline. On assessing the baseline factors that contribute to the final visual acuity, presenting visual acuity was the only variable found to be statistically significant., Conclusion: Although described to have good visual outcome, eyes presenting with poor visual acuity tend to have worse vision on follow up. This may aid in prognosticating and guiding management plan., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

This review summarizes the impact of systemic and ocular inflammatory disorders on diabetes mellitus (DM) and diabetic retinopathy (DR). Local inflammation is a key pathology in diabetic retinopathy (DR) and is also an evolving target for clinical therapy. The legacy effects of local inflammation at the intracellular level make DR a persistent self-driven vicious process. Ocular inflammation is accompanied as well as incited by systemic inflammation due to diabetes mellitus (DM) itself. Over the years, a multitude of studies have evaluated the impact of systemic inflammatory disorders (SIDs, like rheumatoid arthritis, lupus, psoriasis, etc.) and anti-inflammatory drugs prescribed for managing them on manifestations of DM. Recent studies have indicated increased insulin resistance to be a result of chronic inflammation, and the anti-inflammatory drugs to have a protective effect towards DM. Very few studies have evaluated the impact of SIDs on DR. Furthermore, the evidence from these studies is conflicting, and while local anti-inflammatory therapy has shown a lot of clinical potential for use in DR, the results of systemic anti-inflammatory therapies have been inconsistent. The impact of local ocular inflammation due to uveitis on DR is a crucial aspect that has not been evaluated well at present. Initial pre-clinical studies and small-sized clinical reports have shown a strong and positive relationship between the presence of uveitis and the severity of DR as well as its progression, while larger cross-sectional patient surveys have refuted the same. The long term impact of ocular inflammation due to uveitis on DR needs to be studied while adjusting for confounders.

Background: Diabetic eye disease is a highly prevalent and sight-threatening disorder. It is a disease of neuro-vascular unit of the retina, if left untreated can cause blindness. Therapeutic approaches followed for its treatment can only restrict the progression of the disease with highly variable results. There is no known biomarker for an early diagonsis of this disease, therefore by the time it is detected it goes beyond repair. This creates a massive demand for development of such biomarkers that help detect disease in its earlier stages., Methods: PUBMED (https://pubmed.ncbi.nlm.nih.gov/) was searched for articles relevant to the topic published till November 2023. The search was made using keywords such as Diabetic Retinopathy, inflammation, tear, biomarker, proteomics etc. The studies providing relevant information to prove the importance of biomarker discovery were chosen. After compiling the data, the manuscript writing was planned under relevant headings and sub-headings., Results: The review provides a comprehensive overview of all the tear protein biomarker studies in the field of DR and DME. Briefly, their potential in other diseases is also elucidated. While there are many studies pertaining to DR biomarkers, the identified markers lack validations which has restricted their usage in clinics. In case of DME, there was no such study towards biomarker discovery for its diagnosis and prognosis., Conclusions: The review highlights major studies and their lacunae in the field of biomarkers discovery for DR and DME.

The prevalence of type 2 diabetes (T2D), associated systemic disorders, diabetic retinopathy (DR) and current health policies in south Asian countries were analysed to assess country-specific preparedness to meet the 2030 Sustainable Development Goals. The south Asian countries were classified by human development index, socio-demographic index, multidimensional poverty indices, and eye health resources for epidemiological resource-level analysis. In south Asia, the prevalence of diagnosed and undiagnosed T2D in adults aged 40 years or above, was higher in Pakistan (26.3%) and Afghanistan (71.4%), respectively; India has the highest absolute number of people with DR, and Afghanistan has the highest prevalence of DR (50.6%). In this region, out-of-pocket spending is high (∼77%). This Health Policy is a situational analysis of data available on the prevalence of DR and common eye diseases in people with T2D in south Asia and available resources to suggest tailored health policies to local needs. The common issues in the region are insufficient human resources for eye health, unequal distribution of available workforce, and inadequate infrastructure. Addressing these challenges of individuals with T2D and DR, a 10-point strategy is suggested to improve infrastructure, augment human resources, reduce out-of-pocket spending, employ targeted screening, and encourage public-private partnerships., Competing Interests: TD received funding from Hyderabad Eye Research Foundation, Hyderabad, India (2024) for this paper. SS reported receiving financial support not related to this manuscript from AbbVie, Amgen, Apellis, Bayer, Biogen, Boehringer Ingelheim, Novartis, Eyebiotech, Eyepoint Pharmaceuticals, Janssen Pharmaceuticals, Nova Nordisk, Optos, Ocular Therapeutix, Kriya Therapeutics, OcuTerra, Roche, Stealth Biotherapeutics and Sanofi. All authors declare no other conflicts of interest., (© 2024 The Author(s).)

The response of retinal pathology to interventions in diabetic retinopathy (DR) is often independent of the glycated hemoglobin (HbA1c) values at the point of care. This is despite glucose control being one of the strongest risk factors for the development and progression of DR. Previous preclinical and clinical research has indicated metabolic memory, whereby past cumulative glucose exposure may continue to impact DR for a prolonged period. Preclinical studies have evaluated punitive metabolic memory through poor initial control of DM, whereas clinical studies have evaluated protective metabolic memory through good initial control of DM. In this narrative review, we evaluate the preclinical and clinical evidence regarding metabolic memory and discuss how this may form the basis of preventive care for DR by inducing "metabolic amnesia" in people with a history of uncontrolled diabetes in the past. While our review suggested mitochondrial biology may be one such target, research is still far from a possible clinical trial. We discuss the challenges in such research., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Purpose: To characterize hemi-retinal vein occlusion (HRVO) in patients presenting to a multi-tier ophthalmology hospital network., Methods: This retrospective, hospital-based study analyzed 2,834,616 new patients between August 2010 and June 2021. Patients with a clinical diagnosis of HRVO in at least one eye were included as cases. Data were collected using an electronic medical record system. Data were compared to the findings noted in branch RVO (BRVO) and central RVO (CRVO) patients., Results: HRVO constituted 0.9% ( n = 191) of all the retinal vein occlusions (RVOs), with the mean age being 60.55 ± 10.14 years. Most patients were male (125, 65.45%) with unilateral (92.67%) affliction. Majority presented during the sixth (31.41%) or seventh (32.46%) decade of life. Most patients reported mild (37.07%) or moderate (27.32%) visual impairment, with vision < 20/200 being less common in HRVO (25.8%) and BRVO (17.2%) compared to CRVO (44.1%) ( P < 0.00001). Glaucoma was diagnosed and treated in 49 (23.90%) eyes, which was much higher than CRVO (11.45%) and BRVO (5.04%) ( P < 0.001), though neovascular glaucoma was much less than CRVO (2.9% vs. 9.2%) ( P = 0.0037). On follow-up, HRVO eyes (12.2%) had lesser vision loss compared to CRVO eyes (13.7%) (this difference does not look very significant to me), though BRVO had the least (9.1%) vision loss., Conclusion: HRVO is a rare RVO, presenting more in males. It causes less-severe visual impairment compared to CRVO. Large majority of patients with HRVO do not have identifiable systemic risk factors other than age. Preexisting glaucoma was more associated with HRVO compared to other RVOs., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Purpose: To present the successful application of fibrin glue as a surgical adjunct in the management of complex rhegmatogenous retinal detachment (RRD). Methods: In this retrospective case series, fibrin glue was used as a surgical adjunct in 5 cases of complex RRD. In each case, standard pars plana vitrectomy and laser retinopexy were performed by the same surgeon. Fibrin glue was used intraoperatively as a tamponade to seal the breaks because the isolated use of conventional tamponade agents was not feasible given the variable nature of the complex RRDs, the anatomy of the eye, or an inability to maintain postoperative positioning. Results: In 1 patient previously treated for a large corneoscleral tear, fibrin glue was used to seal a large iatrogenic retinal break caused by a fragmatome-related surge that led to a quadrantic RD. In 2 patients treated for combined RRD, fibrin glue was used with silicone oil to manage recurrent RRD with incompletely drained thick subretinal fluid and blood. In 2 other cases, fibrin glue was applied to manage RRD in congenital aniridia with advanced glaucoma and aphakia. In all cases, retinal attachment without serious adverse effects was attained over a follow-up ranging from 4 to 6 months. Conclusions: Fibrin glue is an effective, safe surgical adjunct in complex RRD. It can be used to transiently seal a retinal break when use of a conventional tamponade agent is not possible or not sufficient alone., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Purpose: To determine macular pigment (MP) density scores in healthy Indians and examine correlations with demographic and lifestyle variables., Methods: We observed 484 Indians without an ocular pathology. Body mass index (BMI) and self-reported lifestyle factors (sunglasses usage, physical activity, and smoking) were noted. MP density was assessed as the threshold of perception of the shadow of their macular pigments on their retina using a new MP assessment tool (MP-eye). Lutein and zeaxanthin intake was assessed using a prevalidated questionnaire regionally designed for the Indian diet. Clusters of participants were created for statistical analysis based on MP-eye scores secondarily to detect any relevant effects in very low, low, medium, and high ranges of MPs., Results: Data analyzed included 235 males and 249 females with mean age of 36.1 ± 12.9 years (range, 14-72). The median MP-eye score was 6 (range, 0-10, with 10 being high). Most were non-smokers (413, 85.3%) and did not use sunglasses (438, 90.5%), and 314 (64.9%) had low physical activity. Diabetes was present in 62 participants (12.8%) and hypertension in 53 (10.9%). Advancing age (r = -0.209; P < 0.000) and BMI (r = -0.094; P = 0.038) had weak negative correlation with MP-eye scores. Hypertension was less prevalent (7/88) in the cluster with the highest median MP-eye score (P = 0.033). Dietary intake of MPs and other lifestyle factors did not correlate significantly with MP-eye score overall or when analyzed in clusters., Conclusions: MP-eye scores of an Indian population were normally distributed. Higher age, high BMI, and presence of hypertension were weakly associated with lower MP-eye scores. The impact of diet on MPs requires further evaluation., Translational Relevance: This normative regional database enables risk stratification of macular degeneration.

Objective: To describe the demographics, clinical, and imaging characteristics, and visual outcomes in young patients with full-thickness traumatic macular hole (TMH)., Methods: This retrospective hospital-based study included patients with full-thickness TMH who presented between August 2010 and June 2021. Demographic data, clinical findings, and imaging characteristics were extracted from an electronic medical record system. Regression analyses were performed to determine significant associations among variables and to identify predictors of visual outcomes., Results: 144 (0.005%) patients among 2,834,616 were diagnosed with Full thickness TMH. The majority of them were male (89.58%; odds ratio [OR] = 6.71) and the holes were unilateral. The mean age at presentation was 23.37 ± 8.19 years. Ball were the most common cause of injuries (22.22%), followed by stick (14.58%) and firecracker (12.50%). The mean LogMAR visual acuity (VA) at presentation was 1.18 ± 0.72, with 25.69% of eyes having VA < 20/400. The mean minimum hole diameter was 619.34 ± 336.16 μm. Sub-retinal fluid was present in 44.44%, followed by intraretinal fluid in 34.03% of eyes. Macular holes closed after vitrectomy in 66.67% of eyes, with mean final VA of 1.07 ± 0.85. Baseline VA was a strong predictor of final VA (R 2  = 0.677; p = 0.000168)., Conclusion: Traumatic macular hole is a unilateral condition with significant visual impairment that is mainly seen in males during the third decade of life. Surgery is successful in most cases but improvements in VA are modest., (© 2024. The Author(s).)

Background: To evaluate the clinical profile, outcomes and antibiotic resistance in bacterial endophthalmitis., Methods: This was a post-hoc analysis of a study conducted at a tertiary centre, where 60 consecutive cases of culture-proven bacterial endophthalmitis were included prospectively. Group 1 included coagulase-negative Staphylococcus endophthalmitis (CNSE), while group 2 included the remaining cases. Clinical features, antibiotic resistance and visual outcomes were compared. Visual acuity >3/60 at six months of follow-up was defined as a good visual outcome., Results: Group 1 had 31 cases, while group 2 had 29. Group 2 included 12 gram-positive and 17 gram-negative isolates. Among the groups, group 2 had more patients with presenting visual acuity below hand motions close to the face (25 vs. 12, p<0.001), poor visual outcomes (26 vs. 3, p<0.001) and retinal detachment (RD) (10 vs. 2, p=0.007). Pseudomonas was most commonly resistant to antibiotics, and ceftazidime (p=0.005) and cefazolin (p=0.009) resistance were higher in group 2 isolates. In group 1, five isolates were resistant to any one of the antibiotics, whereas in group 2, 13 isolates were resistant to any one of the antibiotics (p=0.024)., Conclusions: In the current study, eyes in the group of endophthalmitis caused by CNSE achieved better visual acuities at the last follow-up compared to eyes with endophthalmitis caused by other bacteria. Antibiotic resistance in isolates other than CNSE is a cause of concern., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Gaur et al.)

Purpose: Outcomes of retinal detachment (RD) have been discussed in detail in many reports of patients with retinitis pigmentosa (RP). This study tries to understand the outcomes of vitreoretinal (VR) surgery for indications other than RD in the eyes with RP., Methods: This is a retrospective study that includes clinical data from January 2013 to December 2021. Patients with RP who were treated with a VR surgical intervention were included in the study. The primary outcome of the study was to assess the changes in best-corrected visual acuity., Results: Forty-four eyes of 40 patients with RP were included in the study. Nearly half of the eyes (43%, 19/44) presented from 1 month to 1 year after the onset of diminished vision, with or without floaters. The mean ± standard deviation (SD) best-corrected visual acuity (BCVA) at presentation was 1.30 ± 0.79 logMAR (20/400 ± 20/125). The major surgical indications were vitreous opacities (43.2%, 19/44) and subluxated/dislocated cataractous lenses (25%, 11/44). The median follow-up duration was 8 months (interquartile range (IQR): 1.5-27). Approximately 77% (34/44) of the eyes had improvement in vision. The mean postoperative BCVA at the last follow-up was 0.95 ± 0.73 logMAR (p-value: 0.03)., Conclusions: Most eyes with RP recovered well after VR surgical interventions, with short-term improvements in visual acuity. It may be crucial to address the vitreous opacities and membranes as they hinder the residual central island of vision in RP. However, appropriate counselling is required regarding the progressive nature of retinal neuronal degeneration., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To describe the demographics and clinical profile of parafoveal telangiectasia (PFT) and compare risk factors for diabetic retinopathy (DR) among PFT and non-PFT patients., Methods: This cross-sectional hospital-based study included 2,834,616 new patients presenting to a multi-tier ophthalmology hospital network in India between August 2010 and June 2021. Patients with a clinical diagnosis of PFT in at least one eye were included as cases. The data were collected using an electronic medical record system., Results: Overall, 2,310 (0.081%) patients were diagnosed with PFT. Most of the patients were female (62.42%) with (odds ratio [OR] = 2.08), and had bilateral (84.85%) affliction. The most common age group at presentation was during the sixth decade of life with 825 (35.71%) patients. The overall prevalence was higher in patients from an upper socio economic status (0.242%) presenting from the metropolitan geography (0.113%; OR = 2.37). Systemic history of diabetes mellitus (DM) with a mean duration of 122.03 ± 95.59 months was seen in 849 (36.75%) and hypertension in 609 (26.36%) patients. Of the 4,270 eyes, 2,441 (57.17%) eyes had a visual impairment of mild or no visual impairment (<20/70) followed by moderate visual impairment (>20/70-20/200) in 1022 (23.93%) eyes. The risk of sight threatening diabetic retinopathy (STDR) among PFT patients was higher (OR = 1.43) compared to non-PFT cohort. Choroidal neovascular membrane (CNVM) was observed in 481 (11.26%) eyes., Conclusion: PFT is more common in females and is predominantly bilateral. PFT is more common in upper socio economic status and majority of the eyes had mild or moderate visual impairment. Diabetes and Hypertension are associated risk factors in PFT.

Clinically, hemorrhages at the vitreoretinal interface have been termed as 'pre-retinal' in location. However, there is a careful distinction to be made between sub-hyaloid and sub-internal limiting membrane (ILM) planes of blood collection. In the past half-century, a body of literature has accrued on sub-internal limiting membrane hemorrhage. We characterize the etiopathological, clinical, anatomical, and imaging characteristics of this entity (often misconstrued as sub-hyaloid hemorrhage). Management decisions are briefly described, and a unifying term of sub-internal limiting membrane macular hemorrhage is proposed to aid in further research., (Copyright © 2023 Copyright: © 2023 Indian Journal of Ophthalmology.)

This comprehensive review provides a thorough examination of inherited retinal diseases (IRDs), encompassing their classification, genetic underpinnings, and the promising landscape of gene therapy trials. IRDs, a diverse group of genetic conditions causing vision loss through photoreceptor cell death, are explored through various angles, including inheritance patterns, gene involvement, and associated systemic disorders. The focal point is gene therapy, which offers hope for halting or even reversing the progression of IRDs. The review highlights ongoing clinical trials spanning retinal cell replacement, neuroprotection, pharmacological interventions, and optogenetics. While these therapies hold tremendous potential, they face challenges like timing optimization, standardized assessment criteria, inflammation management, vector refinement, and raising awareness among vision scientists. Additionally, translating gene therapy success into widespread adoption and addressing cost-effectiveness are crucial challenges to address. Continued research and clinical trials are essential to fully harness gene therapy's potential in treating IRDs and enhancing the lives of affected individuals., (© 2023. The Author(s).)

Background: To analyze the clinical profile, presentation, possible pathophysiology, and outcomes of central retinal artery occlusion (CRAO) following blunt trauma in pediatric subjects., Methodology: The medical charts of subjects aged 18 years or less with a diagnosis of CRAO following blunt ocular trauma were analyzed retrospectively for demography, details of the trauma, ocular findings, additional imaging reports if any, and final outcome. A Medline search was done (key words like central retinal artery occlusion, blunt trauma, children, pediatric subjects, and adolescents) to gather information available in the literature on the subject., Results: A total of 11 patients (11 eyes), mean age of 14.3 ± 3.4 years, and 100% male preponderance, with an average time duration from trauma to presentation to the hospital of 8.1 days were included. Visual acuity ranged from no light perception (four eyes) to finger count at a 1 m distance. Intraocular pressure was raised in three patients, of which two were suffering from sickle cell disease. In two eyes, the CRAO coexisted with optic nerve avulsion and the cilioretinal artery was spared. Disk pallor was seen in six eyes as early as 12 days from the trauma. None of the cases revealed any bony fracture in the CT scan., Conclusion: CRAO was observed to be an important primary or contributory cause of visual loss in children following blunt trauma, reflex vasospasm being the most common etiology. Early onset disk pallor could suggest an underlying vascular compromise of both retinal and optic disk circulation in addition to direct disk damage., (Copyright © 2023 Copyright: © 2023 Indian Journal of Ophthalmology.)

Purpose: To describe the demographics and clinical profile of Stargardt disease in patients presenting a multitier ophthalmology hospital network in India., Methods: This cross-sectional hospital-based study was performed among 2,834,616 new patients presenting between August 2010 and June 2021 in our network. Patients with a clinical diagnosis of Stargardt disease in at least one eye were included as cases. The data were collected using an electronic medical record system., Results: Overall, 1,934 (0.069%) patients were diagnosed with Stargardt disease. Most of the patients were male (63.14%). The most common age group at presentation was during the second decade of life, with 626 (31.87%) patients. The overall prevalence was higher in patients from a higher socioeconomic status (0.077%), in those presenting from the urban geography (0.079%), and in students (0.197%). Systemic history of hypertension was seen in 56 (2.85%) patients, while diabetes mellitus was seen in (2.49%) patients. Of the 3,917 eyes, 1,910 (48.76%) eyes had moderate visual impairment (>20/70-20/200) followed by severe visual impairment (>20/200 to 20/400) in 646 (16.49%) eyes. The most commonly associated retinal signs were retinal flecks in 1,260 (32.17%) eyes, followed by RPE changes in 945 (24.13%) eyes. The most documented investigations were autofluorescence (39.85%), followed by optical coherence tomography (23.90). Cataract surgery was the commonest performed surgical intervention in (0.66%) eyes, followed by intravitreal injection in 4 (0.10%) eyes. The family history of parent consanguinity marriage was reported by 212 (10.79%) patients., Conclusion: Stargardt disease was seen more commonly in males presenting during the second decade of life. It is predominantly a bilateral disease, with the majority of the eyes having moderate visual impairment., Competing Interests: None

Competing Interests: Competing interests: None declared.

Introduction: The objective of this study was to compare the microbiome in the aqueous humour and gut of people with diabetes mellitus (DM) with and without diabetic retinopathy (DR)., Methods: This was a prospective controlled study. The study included 17 people undergoing intraocular surgery in their naïve eyes. Stool samples were obtained in the perioperative period; aqueous humour samples of sufficient quantity were obtained in 12 people during intraocular surgery. Dietary information was obtained using a previously validated questionnaire. The gut and aqueous humour samples were assessed for microbiome using 16S rRNA gene sequencing coupled with QIIME and R software., Results: Aqueous humour was analysed in 12 people: 4 each healthy controls, people with DM, and people with DR. There were minor differences at the phyla levels, but the aqueous humour microbiomes of healthy controls, DM, and DR formed three distinct clusters on heat map analysis with discriminatory genera. This genera-level clustering was more apparent for the intraocular than the gut microbiome. In people with DM and DR, we identified genera unique to the eye or the gut. There was a consistent reduction in the abundance of anti-inflammatory bacteria in people with DR than DM., Conclusions: There is a difference in intraocular and gut microbiome regardless of disease or health. Our preliminary findings indicate distinctive features of the intraocular microbiome in people with DR compared with those without it. While this distinctiveness appears more evident in aqueous humour than in the gut, it needs further confirmation with larger studies., (© 2023. The Author(s).)

Introduction: The photopic ON pathway defect is associated with nocturnal vision loss. However, the measurement of ON function to detect a rod-dominated disease (rods affected more than cones) has not been explored. We evaluated whether the psychophysical evaluation of ON/OFF pathways can be used to distinguish cone-dominated from rod-dominated diseases., Methods: Thirty-seven patients with inherited retinal diseases were tested using the 'EyeSpeed' [iOS application] on an iPad. The test displayed a random number (1-3) of light or dark targets on a black-and-white noise background. Participants responded on a touch screen indicating the correct number of targets displayed. The outcome variables-reaction time, accuracy and performance index (speed [1/reaction time] * accuracy) to both light and dark targets were assessed for diagnostic ability using standard receiver-operating characteristic (ROC) analysis., Results: Mean ± standard deviation age and visual acuity for the cone- and rod-dominated groups were 25.15 ± 11.74 years, 0.80 ± 0.25 logMAR and 28.3 ± 14.29 years, 0.48 ± 0.26 logMAR, respectively. The median reaction time to light targets in rod-dominated disease [interquartile range] was 5.28 s [3.17], significantly greater than for patients with cone-dominated disease (2.07 s [0.93]; Mann-Whitney U test, p < 0.001). Amongst all of the outcome variables evaluated, the reaction time to light targets (criterion of ≥2.98 s) exhibited the highest area under the ROC curve (area = 0.89 ± 0.11; p < 0.001), with a sensitivity and specificity of 82.4% and 85% respectively., Conclusions: Reaction time to light targets using the ON/OFF pathway paradigm is a valid marker to differentiate between rod- and cone-dominated retinal dystrophies. ON pathway function measured using a tablet-based test could act as a supplemental test in the diagnosis of challenging photoreceptor-specific inherited retinal diseases., (© 2022 College of Optometrists.)

Pythium insidiosum is a parasitic oomycete that can cause keratitis and closely resembles fungus, both clinically and morphologically. It requires a trained microbiologist for its differentiation from fungal filaments and has poor response to antifungal therapy. We present a case of primary isolation of the organism from the vitreous cavity in a case of endophthalmitis. The infection spread quickly and involved all the ocular tissues. The eye had poor visual outcome despite a sequence of rapidly completed retinal and corneal surgeries along with initiation of anti-Pythium therapy.

Purpose: Our study aimed to evaluate the utility of the anterior segment morphometry for objectively assessing anterior segment architectural changes of corneal clouding in the mucopolysaccharidoses (MPS) cohort and to investigate whether these measurements correlate with the slit-lamp findings on the cornea and early diagnosis of glaucoma., Methods: This retrospective study involved 70 eyes of 35 children with cloudy cornea due to MPS variants. Anterior segment architectural alterations were measured using anterior segment imaging and biometry in MPS children and compared with controls., Results: Mean age of the cohort at the time of assessment was 7.9 ± 4.5 years. Males constituted two-thirds of the cohort. Variants of MPS with cloudy cornea were as follows: Type I (62%), Type IV (11%), and Type VI (22%). Morphometric measurements were available in 22 eyes of 11 MPS children and an age-matched healthy control group. There were significant differences between MPS cohort and controls in refraction in Diopters (5.03 ± 0.39 and 0.01 ± 0.04; P < 0.0001), axial length (AXL) in mm (21.39 ± 0.28 and 23.04 ± 0.28; P = 0.0002), average keratometry in Diopters (40.67 ± 0.44 and 42.83 ± 0.44; P < 0.0001), anterior chamber depth (ACD) in mm (2.92 ± 0.07 and 3.65 ± 0.07; P < 0.0001), and intraocular pressure (IOP) in mmHg (25.2 ± 2.0 and 14.1 ± 2.3; P = 0.0003). Secondary glaucoma was observed in 28% of the MPS cohort., Conclusion: The anterior segment morphometry in the cloudy cornea due to MPS provides an objective measurement of anterior segment architectural changes, thus diagnosing early-onset secondary glaucoma. These findings highlight that cloudy cornea due to MPS variants merits close monitoring throughout life., Competing Interests: None

Diabetic retinopathy (DR) is an important microvascular complication of diabetes mellitus (DM), causing significant visual impairment worldwide. Current gold standards for retarding the progress of DR include blood sugar control and regular fundus screening. Despite these measures, the incidence and prevalence of DR and vision-threatening DR remain high. Given its slowly progressive course and long latent period, opportunities to contain or slow DR before it threatens vision must be explored. This narrative review assesses the recently described unconventional strategies to retard DR progression. These include gut-ocular flow, gene therapy, mitochondrial dysfunction-oxidative stress, stem cell therapeutics, neurodegeneration, anti-inflammatory treatments, lifestyle modification, and usage of phytochemicals. These therapies impact DR directly, while some of them also influence DM control. Most of these strategies are currently in the preclinical stage, and clinical evidence remains low. Nevertheless, our review suggests that these approaches have the potential for human use to prevent the progression of DR., Competing Interests: Disclosures None of the authors have any conflicting interests to declare., (Copyright © 2022. Published by Elsevier Inc.)

Purpose: To report a rare ocular presentation of unilateral anterior uveitis with hypopyon in a 5-year-old child, suspected as masquerade., Methods: Retrospective report., Results: A 5-year-old boy presented with hypopyon-uveitis. Detailed systemic work-up was negative for masquerade and uveitis entities. He was started on oral and topical steroids but had reactivation on taper. Real-time polymerase chain reaction (PCR) test of aqueous fluid was positive for herpes simplex virus (HSV)-1 DNA. The inflammation resolved completely on oral acyclovir and topical corticosteroids. He subsequently underwent pars plana lensectomy, primary posterior capsulotomy and vitrectomy for complicated cataract. Post-operatively, vision improved to 20/400 with aphakic correction. Maintenance oral acyclovir was stopped after 3 months with no reactivation and amblyopia therapy was continued., Conclusion: This is a rare presentation of hypopyon uveitis due to HSV-1 in a child. The role of real-time PCR in establishing the diagnosis is crucial in such cases of diagnostic dilemma.

Background: Regular screening for retinopathy and timely intervention reduces blindness from diabetes by 90%. Screening is currently dependent on the interpretation of images captured by trained technicians. Inherent barriers of accessibility and affordability with this approach impede widespread success of retinopathy screening programs. Herein, we report our observations on the potential of a novel approach, Selfie Fundus Imaging (SFI), to enhance diabetic retinopathy screening., Methods: The study was undertaken over a two-month period during COVID 19 lockdown. 60 diabetic patients participated in the study. Retinal images were captured using three different approaches, handheld smartphone-based photographs captured by patients themselves after a short video-assisted training session (SFI group), and smartphone-based photographs captured by a trained technician and photographs taken on desktop conventional digital fundus camera (Gold standard). Sensitivity and kappa statistics was determined for retinopathy and macular oedema grading., Findings: Mean age of the study participants was 52.4 years ± 9.8 years and 78% were men. Of 120 images captured using SFI, 90% were centred-gradable, 8% were decentred-gradable and 2% were ungradable. 82% patients captured the image within a minute (majority by 31-45 s). The sensitivity of SFI to detect diabetic retinopathy was 88.39%. Agreement between SFI grading and standard fundus photograph grading was 85.86% with substantial kappa (0.77). For the detection of diabetic macular oedema, the agreement between SFI images and standard images was 93.67, with almost perfect kappa (0.91)., Conclusion: Fundus images were captured by patients using SFI without major difficulty and were comparable to images taken by trained specialist. With greater penetrance, advances, and availability of mobile photographic technology, we believe that SFI would positively impact the success of diabetic retinopathy screening programs by breaking the barriers of availability, accessibility, and affordability. SFI could ensure continuation of screening schedules for diabetic retinopathy, even in the face a highly contagious pandemic., (© 2021. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Ocular cysticercosis is a sparsely reported condition, requiring urgent management. The gold standard for diagnosis is an in toto extraction of the cyst with subsequent histopathology. The procedure can be demanding in contrast to the frequently adopted practice of in vivo cyst lysis. The latter, however, obviates a conventional biopsy. We reviewed published optical coherence tomography (OCT) images of ocular cysticercosis for their suitability to surrogate a conventional biopsy and identified commonly reported features. We also used triple masking and ascertained the observer agreement on identification of these features. We found that the features of the parasite are much more clearly discernible as compared with features of the involved ocular tissue itself. The hyperreflective cyst wall and scolex and the hyporeflective cyst cavity had the highest frequency and observer agreement among all the analyzed features, suggesting their use for diagnosis. We could match many of the OCT features with the previously reported histopathological findings, supporting the role of OCT as a diagnostic adjunct and a substitute for conventional biopsy. Conversely, features of the ocular tissue could be judged poorly with low observer agreement, suggesting poor prognostic ability of OCT. [ Ophthalmic Surg Lasers Imaging Retina 2022;53:446-454.] .

Purpose: Gut dysbiosis has been identified and tested in human trials for its role in diabetes mellitus (DM). The gut-retina axis could be a potential target for retardation of diabetic retinopathy (DR), a known complication of DM. This study reviews the evidence suggesting gut dysbiosis in DR., Methods: The published literature in the past 5 years was reviewed using predetermined keywords and articles. The review intended to determine changes in gut microbiome in DR, the hypothesized mechanisms linking to the gut-retina axis, its predictive potential for progression of DR, and the possible therapeutic targets., Results: The gut microbiota of people with DM differ from those without it, and the gut microbiota of people with DR differ from those without it. The difference is more significant in the former (DM versus no DM) and less significant in the latter (DM without DR versus DM with DR). Early research has suggested mechanisms of the gut-retina axis, but these are not different from known changes in the gut microbiome of people with DM. The current evidence on the predictive value of the gut microbiome in the occurrence and progression of DR is low. Therapeutic avenues targeting the gut-retina axis include lifestyle changes, pharmacologic inhibitors, probiotics, and fecal microbiota transplantation., Conclusions: Investigating the therapeutic utility of the gut ecosystem for DM and its complications like DR is an emerging area of research. The gut-retina axis could be a target for retardation of DR but needs longitudinal regional studies adjusting for dietary habits.

Purpose: To describe the clinical presentation and demographic distribution of retinitis pigmentosa (RP) in Laurence-Moon-Bardet-Biedl (LMBB) syndrome patients., Methods: This is a cross-sectional observational hospital-based study wherein 244 patients with RP in LMBB syndrome presenting to our hospital network between March 2012 and October 2020 were included. An electronic medical record database was used for data retrieval., Results: There were 244 patients in total, with a hospital-based prevalence rate of 0.010% or 1000/100,000 population. The mean and median age of patients was 15.22 ± 7.56 and 14 (IQR: 10-18.5) years, respectively, with the majority being in the age group of 11-20 years (133/244 patients; 54.50%). Males were more commonly affected (164 patients; 67.21%), and the majority (182 patients; 74.59%) were students. All 244 patients (100%) complained of defective central vision at presentation. More than one-fourth of the patients had severe visual impairment to blindness at presentation. Prominent retinal feature at presentation was diffuse or widespread retinal pigment epithelial degeneration in all patients., Conclusion: Patients with RP in LMBB syndrome present mainly in the first to second decade of life with severe visual acuity impairment to blindness early in life. It is important to rule out LMBB syndrome in early-onset RP with central visual acuity impairment. On the contrary, all patients diagnosed or suspected with LMBB syndrome systemic features at physician clinic should also be referred for ophthalmic evaluation, low vision assessment, rehabilitation, and vice versa., Competing Interests: None

Competing Interests: None

Competing Interests: None

Purpose: To evaluate patterns of pediatric vitelliform macular dystrophy (PVMD)., Methods: This is a retrospective analysis of Indian children with vitelliform macular dystrophy (VMD) presenting within the first decade of life. Records were evaluated for clinical findings, family screening, and investigative findings including optical coherence tomography (OCT), fundus autofluorescence (FAF), full-field electroretinogram (ERG) and electrooculogram (EOG). Electrophysiology was scrutinized and audited for acquisition and interpretation errors. Findings on follow-up were also recorded., Results: 46 eyes of 24 patients were included. Mean age at presentation was 7.17 ± 2.17 years. Mean follow-up duration was 1.55 ± 1.69 years. Best disease was the commonest type of VMD detected (21 patients), while autosomal recessive bestrophinopathy was seen in three cases. Mean logMAR BCVA was 0.364 which decreased to 0.402 on follow-up. Hyperopia was noted in 29 out of 46 eyes (mean being +3.87 D, range ebing +0.75 to +8.75 D). Four eyes of four children had choroidal neovascular membrane at presentation, while another child developed while in follow-up. Solid type subretinal deposit was the commonest OCT finding (n = 29/38) and central hyper FAF was the commonest pattern (n = 18/32). EOG was available for review in 32 eyes, but was unreliable in 11 eyes. Seven eyes demonstrated complete absence of light rise on EOG., Conclusion: PVMD can present in advanced forms. Progression to complications with loss of visual acuity can happen within the first decade of life. EOG shows grossly suppressed waveforms in the light phase in a large number of such children., Competing Interests: None

Purpose: To determine and validate retinal vascular caliber measurements by using the confocal scanning laser ophthalmoscopy system. Retinal vasculature changes are often regarded as clinical markers for systemic disease., Methods: It was a prospective observational study conducted on 600 eyes of 300 normal subjects with no systemic or ocular illness from January 1, 2016 to June 30, 2017 in a tertiary referral eye center. Non-mydriatic infrared reflectance, blue reflectance, and blue peak blue autofluorescence fundus imaging were done on the confocal scanning laser ophthalmoscopy system. The dimensions of the retinal vessels were measured using inbuilt calipers at 1800 μm from the center of the optic disc. Internal and external dimensions were measured. Observer variation and its comparison using Image J software were assessed., Results: The median age was 29 years (18-50 years). Mean internal and external diameters for arterioles were 85.1 ± 12.4 μm and 105.0 ± 12.0 μm, and for venules were 133.8 ± 16.6 μm and 145.4 ± 16.1 μm, respectively. The mean internal and external wall thicknesses were 19.7 ± 8.0 μm and 11.0 ± 5.6 μm, and wall thickness-to-lumen ratios were 0.3 ± 0.1 and 0.1 ± 0.1, respectively. Arteriolar-to-venular ratio for lumen and vessel was 0.66 ± 0.1 and 0.74 ± 0.1, respectively. There was no statistically significant difference between age groups. Both inter- and intra-observer reproducibility was >95%. The Bland-Altman plot showed that the difference between measurements using both confocal scanning laser ophthalmoscopy and Image J software lies within the limits of agreement approximately 95% of the time., Conclusion: This is the first effort to develop a normative database by using a simple non-invasive confocal scanning laser ophthalmoscopy system with high observer reproducibility., Competing Interests: None

Purpose: To demonstrate the usefulness of ultrawide-field fundus (UWF) photography for documentation in retinopathy of prematurity (ROP)., Methods: The medical records of infants with ROP who underwent at least one sitting of UWF fundus photography in addition to binocular indirect ophthalmoscopy (BIO) from April 2018 to September 2020 were analyzed retrospectively. BIO was conducted by a trained ROP specialist, and final diagnosis and treatment were based solely on BIO findings. All fundus photographs were captured on Optos UWF camera (Dunfermline, UK) in a flying baby position. Demographic details and fundus findings on BIO and fundus photographs were analyzed., Results: Of the 187 infants who met inclusion criteria for successful imaging, 22 (11.7%) had findings that were discordant with BIO. Although no posterior disease was missed, 4 infants who received treatment would not have been treated based solely on UWF photography findings. Of the 60 babies whose images did not meet the inclusion criteria for successful imaging, 41 had ROP that required intervention., Conclusions: In our patient cohort, UWF photography proved useful in documenting the initial and follow-up findings of preterm babies with ROP., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the causes of jet stream injury (JSI)-related iatrogenic retinal breaks (IRBs) during vitreoretinal surgery (VRS)., Methods: The precise surgical environment, which includes the indication and type of surgical procedure, retina status, details of instrumentation and fluidic parameters, and characteristics of the jet responsible for the IRB, was noted from case records. The nature of IRB and its healing and impact on anatomical and visual outcomes were analyzed., Results: Five eyes of five patients with complete documentation of both the JSI and the IRB were included. Two cases were operated for macular hole, and one each for vitreous hemorrhage, retinal detachment, and endophthalmitis. One case had infusion-fluid-related JSI, while four developed it because of injection of surgical adjuncts (drugs, PFCL, and dye). JSI developed in two cases when the vitreous cavity was filled with fluid, while it was air-filled in three cases. In four cases, the fluid migrated into subretinal space, necessitating further maneuvers following which the breaks healed, but were directly responsible for vision loss in two cases., Conclusion: JSI related IRBs are rare but may be directly responsible for vision loss if they impact the macula. The balance between jet stream velocity, its distance from the retinal surface, the intervening media (vitreous cavity), and retinal health play an important role. It can occur because of both infusion as well as injection jets. Precautions must be taken in cases vulnerable to complications with suggested modifications in the surgical technique., Competing Interests: None

Purpose: The proportion of axial length (AL) occupied by vitreous chamber depth (VCD), or VCD:AL, consistently correlates to ocular biometry in the general population. Relation of VCD:AL to ocular biometry in high myopia is not known. The purpose of this study is to evaluate the relation of VCD and VCD:AL to ocular biometry of highly myopic eyes., Methods: This was a cross-sectional retrospective study of records of 214 myopic eyes (<-1 D SE, aged 20-40 years) attending the refractive surgery services. High axial myopia was defined as AL >26.5 mm. Eyes with posterior staphyloma and myopic maculopathy were excluded. Records were assessed for measurements of AL, central corneal thickness (CCT), anterior chamber depth (ACD), lens thickness (LT), white to white diameter (WTW), and vitreous chamber depth (VCD). Groups were formed based on increasing AL, while the sum of CCT, ACD, and LT was recorded as anterior segment depth (AS). The main outcome measure was the correlation of VCD and VCD:AL to ocular biometry. A comparison was also performed based on of degree of axial myopia., Results: Mean age of the patients was 27.0 ± 5.2 years. VCD showed a very strong correlation with AL (R = 0.98, P < 0.001) but did not correlate to any anterior parameter. VCD:AL showed moderate negative relation with AS (R = -0.43, P < 0.001) and ACD (R = -0.3, P < 0.001), while it had a weakly negative relation with LT (R = -0.18, P = 0.006). VCD:AL showed strong negative relation (R > ~0.7) with AS in all individual groups of AL. Among anterior parameters, WTW showed the most consistent relation with ocular biometry., Conclusion: VCD:AL is a better correlate of ocular biometry in high myopia as compared to VCD. However, the correlation is weaker than that noted by previous studies done on the general population. Longitudinal studies of VCD:AL in the younger age group is recommended., Competing Interests: None