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Purpose: To report the clinical profile and donor characteristics of post-optical keratoplasty adverse events notified at an eye bank. Methods: Between January 2013 and December 2022, 37,041 donor corneas were utilized for keratoplasty, of which 16,531 were used for penetrating keratoplasty (PK), 12,171 for endothelial keratoplasty (EK), and 1356 for anterior lamellar keratoplasty (ALK). EK included 10,956 Descemet stripping automated endothelial keratoplasty (DSAEK) and 1215 Descemet membrane endothelial keratoplasty (DMEK). The adverse events reported within the first 6 weeks of optical keratoplasty were analyzed for donor-related parameters. Results: A total of 41 (0.11%) recipients had post-keratoplasty infections. Of these, 33 occurred after EK (29 after DSAEK, and four after DMEK), two after ALK, and six after PK. The clinical presentation was keratitis alone in 16 eyes and associated with endophthalmitis in 25 eyes. The organisms isolated were gram-negative bacteria in 26 eyes, fungus in eight eyes, gram-positive bacteria in four eyes, mixed infection in five eyes, and microbiology inconclusive in seven eyes. The majority (78%) of the gram-negative infections were due to multidrug-resistant organisms. Most (88%) donor corneas were harvested from hospital premises. The most common cause of donor mortality was trauma. The median duration of presentation from surgery was 4.46 (range: 1–30) days. The death to preservation time was 4.18 (1.5–7.65) hours. The death to utilization time was 3 (2–4.7) days. Conclusion: The overall risk of infectious adverse events after keratoplasty was 0.11%, ranging from 0.08% to 0.36%. Most (80.4%) of the adverse events occurred after EK. The majority (78.9%) of the adverse events were of bacterial etiology, of which gram-negative infections (68.4%) were the most common. The trends and microbiological spectrum of organisms associated with infections should be thoroughly documented in eye banks to gain insights and formulate guidelines on the management of adverse events.

Abstract The clinical success of CRISPR therapies hinges on the safety and efficacy of Cas proteins. The Cas9 from Francisella novicida (FnCas9) is highly precise, with a negligible affinity for mismatched substrates, but its low cellular targeting efficiency limits therapeutic use. Here, we rationally engineer the protein to develop enhanced FnCas9 (enFnCas9) variants and broaden their accessibility across human genomic sites by ~3.5-fold. The enFnCas9 proteins with single mismatch specificity expanded the target range of FnCas9-based CRISPR diagnostics to detect the pathogenic DNA signatures. They outperform Streptococcus pyogenes Cas9 (SpCas9) and its engineered derivatives in on-target editing efficiency, knock-in rates, and off-target specificity. enFnCas9 can be combined with extended gRNAs for robust base editing at sites which are inaccessible to PAM-constrained canonical base editors. Finally, we demonstrate an RPE65 mutation correction in a Leber congenital amaurosis 2 (LCA2) patient-specific iPSC line using enFnCas9 adenine base editor, highlighting its therapeutic utility.

This study aims to investigate the relationship between pachychoroid spectrum disorders and retinitis pigmentosa (RP) or rod-cone dystrophy through a comprehensive literature review. The purpose is to explore the association between these disorders, understand their underlying mechanisms, and summarize the existing hypotheses and opinions. A thorough review of the literature was conducted using PubMed, focusing on articles related to central serous chorioretinopathy (CSC), RP, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, focal choroidal excavation, peripapillary pachychoroid neovasculopathy, and peripheral exudative hemorrhagic chorioretinopathy. Relevant studies were selected for a detailed narrative review and analysis. Several studies have reported the coexistence of CSC and RP, indicating a potential association between the two conditions. The dysfunction of the retinal pigment epithelium is proposed as a common factor. Choroidal thinning is observed in RP, but conflicting results exist regarding choroidal thickness (CT). While some studies support choroidal thinning in RP, others suggest preserved or increased thickness. Additionally, cases of pachychoroid neovasculopathy and polypoidal choroidal vasculopathy in RP have been reported, suggesting an overlap between these conditions. The literature suggests conflicting reports on CT changes in RP. Future research should focus on large-scale studies using comprehensive imaging techniques, genetic analysis, and long-term follow-up to uncover the underlying mechanisms and determine the prevalence of pachychoroid spectrum disorders in RP patients.

The Stargardt’s Disease, Type 1 (STGD1) is associated with the loss of function mutations in ABCA4. This gene codes for a retina-specific, ATP-binding cassette (ABC) family transporter, involved in the transport of the key visual cycle intermediate, all-trans-retinaldehyde (atRAL), across the photoreceptor cell membranes. Here, we report the establishment of a patient-specific, iPSC line (LVPEIi008-A), that carries a homozygous nonsense mutation at (c.6088C > T) position, within exon 44 of ABCA4. The patient-specific skin fibroblasts were reprogrammed using episomal plasmids and the stably expanding iPSC line expressed the key stemness and pluripotency markers, maintained its chromosomal integrity and tested negative for mycoplasma.

Mutations in ABCA4 gene leads to the most common form of an inherited retinal disease namely, the Stargardt disease, type 1. Here, we report the generation of two different patient-specific induced pluripotent stem cell lines (LVPEIi007-B and LVPEIi008-B), carrying an identical homozygous mutation, (c.6088C>T) within the exon 44 of ABCA4 gene. These lines were generated by the reprogramming of patient-specific dermal fibroblasts, using the integration-free, Sendai viral vectors. Both lines were stably expanded and expressed the stemness and pluripotency markers, differentiated into cell types of all three germ layers, and maintained a normal karyotype.

Leber Congenital Amaurosis 2 is an early onset retinal dystrophy that occurs due to mutation in RPE65 gene. Here, we report the generation of two patient specific induced pluripotent stem cell lines harboring nonsense mutations in exon 7 (c.646A > T) and exon 9 (c.992G > A) of RPE65 gene, respectively, which leads to premature translational termination and formation of defective protein. These lines were generated by the reprogramming of human dermal fibroblast cells using integration-free, episomal constructs expressing stemness genes. The stable lines maintained a normal karyotype, expressed the key stemness factors, underwent trilineage differentiation, and maintained their genetic identity and genomic integrity.

Leber congenital amaurosis (LCA) is a congenital, early onset, autosomal recessive inherited retinal disease (IRD). This report describes an LCA12 patient-specific iPSC line (LVPEIi006-A), generated by the reprogramming of dermal fibroblasts using integration-free episomal plasmids.This disease-specific iPSC model carries a homozygous point mutation in RD3, within the donor splice site at the end of exon 2 (c.296 + 1G > A). The stable line at passage 15 has displayed a normal colony morphology, expressed multiple stemness and pluripotency markers, lost all transgenes, differentiated into cell types of all three germ layers, and maintained a normal karyotype.

Purpose: To evaluate the feasibility and outcome of a real-time retinopathy of prematurity (ROP) telescreening strategy using videoconferencing in a community setting in India. Method: In a prospective study, trained allied ophthalmic personnel obtained the fundus images in the presence of the parents and local childcare providers. Analysis of images and parental counseling were done in real time by an ROP specialist located at a tertiary center using videoconferencing software. A subset of babies was also examined using bedside indirect ophthalmoscopy by an ROP care-trained ophthalmologist. The data were analyzed using descriptive statistics, sensitivity, specificity, positive and negative predictive values, and correlation coefficient. Results: Over 9 months, we examined 576 babies (1152 eyes) in six rural districts of India. The parents accepted the model as they recognized that a remotely located specialist was evaluating all images in real time. The strategy saved the travel time for ROP specialists by 477 h (47.7 working days) and for parents (47,406 h or 1975.25 days), along with the associated travel cost. In a subgroup analysis (100 babies, 200 eyes), the technology had a high sensitivity (97.2%) and negative predictivity value (92.7%). It showed substantial agreement (k = 0.708) with the bedside indirect ophthalmoscopy by ROP specialists with respect to the detection of treatment warranting ROP. Also, the strategy helped train the participants. Conclusion: Real-time ROP telescreening using videoconferencing is sensitive enough to detect treatment warranting ROPs and saves skilled workforce and time. The real-time audiovisual connection allows optimal supervision of imaging, provides excellent training opportunities, and connects ophthalmologists directly with the parents.

Abstract 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.

Avascular peripheral retina in an infant is a common characteristic of numerous pediatric retinal vascular disorders and often presents a diagnostic challenge to the clinician. In this review, key features of each disease in the differential diagnosis, from retinopathy of prematurity, familial exudative vitreoretinopathy, Coats disease, incontinentia pigmenti, Norrie disease, and persistent fetal vasculature, to other rare hematologic conditions and telomere disorders, will be discussed by expert ophthalmologists in the field.

Advent of pediatric handheld fundus cameras like RetCam, 3netra Forus, and Phoenix ICON pediatric retinal camera has aided in effective screening of retinopathy of prematurity (ROP), especially in countries with limited number of trained specialists. Recent advent of various smartphone-based cameras has made pediatric fundus photography furthermore affordable and portable. Future advances like ultra-wide field fundus cameras, trans-pars-planar illumination pediatric fundus camera, artificial intelligence, deep learning algorithm, and handheld SS-OCTA can help in more accurate imaging and documentation. This article summarizes the features of existing and upcoming imaging modalities in detail, including their features, advantages, challenges, and effectiveness, which can help in implementation of telescreening as a standard screening protocol for ROP across developing as well as developed countries.

Purpose: To assess the incidence, visual impairment, and blindness due to retinitis pigmentosa (RP) in a rural southern Indian cohort. Methods: This is a population-based longitudinal cohort study of participants with RP from the Andhra Pradesh Eye Disease Study (APEDS) cohorts I and III, respectively. The study included participants with RP of APEDS I who were followed until APEDS III. Their demographic data along with ocular features, fundus photographs, and visual fields (Humphrey) were collected. Descriptive statistics using mean ± standard deviation with interquartile range (IQR) were calculated. The main outcome measures were RP incidence, visual impairment, and blindness as per the World Health Organization (WHO) definitions. Results: At baseline (APEDS I), 7771 participants residing in three rural areas were examined. There were nine participants with RP with a mean age at baseline of 47.33 ± 10.89 years (IQR: 39–55). There was a male preponderance (6:3), and the mean best-corrected visual acuity (BCVA) of 18 eyes from nine participants with RP was 1.2 ± 0.72 logarithm of minimum angle of resolution (logMAR; IQR: 0.7–1.6). Over a mean follow-up duration of 15 years, 5395/7771 (69.4%) were re-examined, which included seven RP participants from APEDS 1. Additionally, two new participants with RP were identified; so, the overall incidence was 370/ million in 15 years (24.7/million per year). The mean BCVA of 14 eyes of seven participants with RP who were re-examined in APEDS III was 2.17 ± 0.56 logMAR (IQR: 1.8–2.6), and five of these seven participants with RP developed incident blindness during the follow-up period. Conclusion: RP is a prevalent disease in southern India that warrants appropriate strategies to prevent this condition.

Purpose: To describe the clinical features and treatment outcomes in spontaneous uveal effusion syndrome (UES). Methods: A 10-year retrospective chart review of UES patients from a tertiary eye center was carried out. Optical coherence tomography (OCT), fundus fluorescein angiography, and ultrasound biomicroscopy (UBM) scans were performed. UES was managed based on presenting best-corrected visual acuity (BCVA), symptoms, and fundus findings. Patients with secondary causes of uveal effusion were excluded. Results: Twenty-five eyes of 16 patients were included. Of the 16 patients, 14 (88%) were male and 9 (56%) had bilateral disease. Fifteen of 25 affected eyes had nanophthalmos (axial length (AL) 20.5 mm. The presenting mean distance BCVA was 0.74 ± 0.64 logMAR (mean Snellen: 20/100). Eleven eyes had exudative retinal detachment, and 4 also had exudative choroidal detachment (CD). Choroidal thickness (CT) was increased in 11 eyes on B-scan ultrasonography, and the mean CT was 1.74 ± 0.38 mm. Sub-retinal fluid (SRF) and retinal folds were the most common OCT findings. UBM findings included shallow angles, peripheral CD, and supra-ciliary effusion. A combination of local and systemic corticosteroids was used to successfully treat 12 eyes, 6 needed surgery, and 7 were observed. Partial sclerectomy with anterior chamber maintainer-assisted SRF drainage was the favored surgery. The median period of follow-up was 6.5 months (0.1–76 months), and the mean distance BCVA at the last follow-up was 0.58 ± 0.42 logMAR (mean Snellen: 20/80). Conclusion: UES can be suitably managed both medically and surgically based on clinical presentation.

Physicians have to play the role of a team leader and counselor and take written informed consent for high-risk surgeries in many cases. This is the first step toward initiating a mutually trustworthy relationship with the patient and family. The situation is more sensitive when vulnerable patients like small premature babies or the elderly are under consideration. In the event of a death, leadership and processes in place become extremely critical. We share our experience and practice pattern during this process, especially suited to India, but the broad principles would apply to most human situations. Hopefully, some of these can be incorporated into the existing training curriculum for team building and the art of effective physician-patient communication that should be intricately woven into the curriculum for the Science of Ophthalmic care.

Purpose: To describe the clinical presentation and demographic distribution of retinitis pigmentosa (RP) in patients with Usher syndrome (USH). Methods: This is a cross-sectional observational hospital-based study including patients presenting between March 2012 and October 2020. In total, 401 patients with a clinical diagnosis of USH and RP in at least one eye were included as cases. The data were retrieved from the electronic medical record database. For better analysis, all 401 patients were reclassified into three subtypes (type 1, type 2, and type 3) based on the USH criteria. Results: In total, there were 401 patients with USH and RP, with a hospital-based prevalence rate of 0.02% or 2/10,000 population. Further, 353/401 patients were subclassified, with 121 patients in type 1, 146 patients in type 2, and 86 patients in the type 3 USH group. The median age at presentation was 27 years (IQR: 17.5–38) years. There were 246 (61.35%) males and 155 (38.65%) females. Males were more commonly affected in all three subtypes. Defective night vision was the predominant presenting feature in all types of USH (type 1: 43 (35.54%), type 2: 68 (46.58%), and type 3: 40 (46.51%) followed by defective peripheral vision. Patients with type 2 USH had more eyes with severe visual impairment. Conclusion: RP in USH is commonly bilateral and predominantly affects males in all subtypes. Patients with USH and RP will have more affection of peripheral vision than central vision. The key message of our study is early visual and hearing rehabilitation in USH patients with prompt referral to otolaryngologists from ophthalmologists and vice versa.

Purpose: To analyze the impact of a revised care plan for retinopathy of prematurity (ROP) during SARS-CoV-2 pandemic in a tertiary eye care facility in eastern India. Methods: In a retrospective study, we analyzed the medical records of babies managed for ROP during the peak of the SARS-CoV-2 pandemic, with particular reference to the challenges, and the revised strategies addressing travel restrictions for five months, from April to August 2020. The strategy included selective referral (babies with higher treatment probability), longer follow-up intervals (babies with non-alarming findings), use of locally available workforce, and teleconsultation whenever feasible. Results: In the given period, 222 babies were examined versus 624 in the preceding year (P = 0.001). The average gestational age, birth weight, and postmenstrual age at presentation were 30.4 weeks, 1.31 kg, and 37.7 weeks, respectively. The first examination was on time in 40.1% of babies but was delayed by a median of 23 days in the remaining babies. In the cohort, 56.7% of babies had any ROP, and 27.9% required treatment (versus 8.8% in the previous year; P < 0.001). The intravitreal anti–vascular growth factor (anti-VEGF) injection was more often used than in the previous year (n = 72 vs 36; P < 0.0001). The treatment outcome was comparable before and after the SARS-CoV-2 lockdown period. There was no report of health issues among the care providers attributable to ROP care. Conclusion: The revised strategy resulted in a smaller pool of babies screened but a larger proportion of babies treated for ROP. This strategy could be used more profitably in future ROP care.

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.

Purpose: To describe the clinical profile and treatment outcomes of infants with retinopathy of prematurity (ROP) presenting with isolated exudative retinal detachment (ERD). Methods: Retrospective interventional case series. Preterm infants diagnosed with ROP with ERD at presentation were included. All demographic details, clinical findings, and treatment given were documented. The anatomical outcome was categorized as good, fair, and poor. Refractive outcome was classified into mild, moderate, and severe according to spherical equivalent at the last visit. Results: Fifteen eyes (8 patients) were included. Mean gestational age was 31.3 weeks, and birth weight was 1462.6 g. All eyes presented with aggressive ROP. Patches of retinal edema in avascular retina were seen in all eyes. A total of 86.6% of eyes had vascular sclerosis while 86.6% of eyes had subretinal exudates. The anatomical outcome was good in all eyes. In addition, 40% of eyes had a mild refractive error. Conclusion: Exudative retinal detachment in ROP is rare. The use of unregulated oxygen can be a contributory factor. Vascular sclerosis is consistent with hyperoxia-induced retinopathy models. Retinal edema and subretinal exudates indicate disrupted inner and outer blood-retinal barrier. Treatment outcomes are good when diagnosed and treated in time.

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.

Abstract Inflammation plays a key role in the pathogenesis of retinal vascular diseases. We have shown earlier an increase in the activity of matrix metalloproteinases in the vitreous and tears of preterm born babies with retinopathy of prematurity (ROP) compared to those with no-ROP leading to a shift in the balance of angiogenic (vascular endothelial growth factor [VEGF], matrix metalloproteinase [MMPs], complement component [C3]) and anti-angiogenic (opticin, thrombospondin) in ROP eyes. We now confirmed that tear MMP levels in premature infants perfectly correlates with disease severity. Next, we demonstrated that a reduced opticin levels in ROP vitreous are regulated by MMPs secreted by activated microglia. Upon exposing the human microglia cell line (CHME3) to hypoxia, an increased expression of inflammatory proteins (MMP9, VEGF) was noticed while opticin reduced significantly (p = 0.005). Further, the reduced opticin’s expression by microglial cells under hypoxia could be rescued by inhibiting the MMP activity using doxycycline and EDTA. The inhibition of MMP activity altered the expression of other key signaling molecules under hypoxia. Our study clearly explains that increased activity of MMPs under hypoxia regulates the expression of opticin as seen in the vitreous humor of ROP and could serve as a potential target for ROP management.

AIM: To summarize the experience of response to COVID-19 outbreak at a tertiary eye care institute and its network of health facilities in India. METHODS: Our responses are based on the principles of social distancing, hand hygiene, respiratory etiquettes, surface disinfection protocol, and rational use of appropriate personal protective equipment (PPE). We describe our response in terms of administrative controls, clinical protocols, staff protection, environmental controls, and social distancing measures. We also discuss our communication strategies and monitoring systems, to ensure compliance to protocols. RESULTS: Administrative control is mainly related to formation of task force and its functions. Clinical protocols are related to patient triaging methods and clinical examination guidelines in Outpatient, Inpatient and Operating Room. Staff protection is focused on training staff on the protocols to be followed in hospital as well as at home, and use of PPE. Environmental protocol is focused on cleaning and disinfectant methods to be used in the hospital. In addition, there are systems for communication as well as monitoring compliance to protocols. CONCLUSION: We hope that these protocols and our experience would help the ophthalmic community globally and serve as a guide to protect ophthalmologists and ophthalmic care personnel, and their patients across the world.

Recent decade has seen a shift in the causes of childhood blinding diseases from anterior segment to retinal disease in both developed and developing countries. The common retinal disorders are retinopathy of prematurity and vitreoretinal infections in neonates, congenital anomalies in infants, and vascular retinopathies including type 1 diabetes, tumors, and inherited retinal diseases in children (up to 12 years). Retinal imaging helps in diagnosis, management, follow up and prognostication in all these disorders. These imaging modalities include fundus photography, fluorescein angiography, ultrasonography, retinal vascular and structural studies, and electrodiagnosis. Over the decades there has been tremendous advances both in design (compact, multifunctional, tele-consult capable) and technology (wide- and ultra-wide field and noninvasive retinal angiography). These new advances have application in most of the pediatric retinal diseases though at most times the designs of new devices have remained confined to use in adults. Poor patient cooperation and insufficient attention span in children demand careful crafting of the devices. The newer attempts of hand-held retinal diagnostic devices are welcome additions in this direction. While much has been done, there is still much to do in the coming years. One of the compelling and immediate needs is the pediatric version of optical coherence tomography angiography. These needs and demands would increase many folds in future. A sound policy could be the simultaneous development of adult and pediatric version of all ophthalmic diagnostic devices, coupled with capacity building of trained medical personnel.

Purpose: The aim of this study was to report the use and the impact of a point-of-care rapid antigen test (PoC-RAT) at a tertiary eye care facility in facilitating commencement of elective surgeries, contact tracing of exposed health care professionals (HCPs) and ancillary hospital staff, and implementation of back-to-work (BTW) policy for them. Methods: Retrospective analysis of subjects undergoing PoC-RAT for COVID-19 infection at a tertiary level dedicated eye care facility. Decision making with regard to commencement of elective surgeries post COVID-19 related discontinuation of services, contact tracing of HCPs and ancillary hospital staff exposed to known COVID-19 cases and implementation of back-to-work policy for all staff based upon the results of PoC-RAT were studied. Results: A total of 311 subjects (224 patients and 87 hospital staff) were tested. Overall positivity rate was around 7%. Asymptomatic patients who were screened preoperatively had a lower positivity rate at around 3% compared to the staff (who were either known contacts or were symptomatic) at around 17%. Contact tracing found three-quarters of the staff at low risk and only one quarter at medium or high risk. Among patients, 97% of those followed up for at least 2 weeks after the test remained healthy. For staff, this was around 65%. Conclusion: Based on our preliminary results, we suggest that PoC-RAT may be considered routinely for indication-based preoperative screening of asymptomatic patients, and for on-campus screening, contact tracing and implementation of BTW policies for HCPs and ancillary hospital staff at a tertiary level eye care facility.

Purpose: Intraocular infection in patients with COVID-19 could be different in the presence of treatment with systemic corticosteroid and immunosuppressive agents. We describe the epidemiology and microbiological profile of intraocular infection in COVID-19 patients after their release from the hospital. Methods: We analyzed the clinical and microbiological data of laboratory-confirmed COVID-19 patients from April 2020 to January 2021 presenting with features of endogenous endophthalmitis within 12 weeks of their discharge from the hospital in two neighboring states in South India. The data included demography, systemic comorbidities, COVID-19 treatment details, time interval to visual symptoms, the microbiology of systemic and ocular findings, ophthalmic management, and outcomes. Results: The mean age of 24 patients (33 eyes) was 53.6 ± 13.5 (range: 5–72) years; 17 (70.83%) patients were male. Twenty-two (91.6%) patients had systemic comorbidities, and the median period of hospitalization for COVID-19 treatment was 14.5 ± 0.7 (range: 7–63) days. Infection was bilateral in nine patients. COVID-19 treatment included broad-spectrum systemic antibiotics (all), antiviral drugs (22, 91.66% of patients), systemic corticosteroid (21, 87.5% of patients), supplemental oxygen (18, 75% of patients), low molecular weight heparin (17, 70.8% of patients), admission in intensive care units (16, 66.6% of patients), and interleukin-6 inhibitor (tocilizumab) (14, 58.3% of patients). Five (20.8%) patients died of COVID-19-related complications during treatment for endophthalmitis; one eye progressed to pan ophthalmitis and orbital cellulitis; eight eyes regained vision >20/400. Fourteen of 19 (73.7%) vitreous biopsies were microbiologically positive (culture, PCR, and microscopy), and the majority (11 patients, 78.5%) were fungi. Conclusion: Intraocular infection in COVID-19 patients is predominantly caused by fungi. We suggest a routine eye examination be included as a standard of care of COVID-19.

Purpose: To report the clinical presentation, microbiology, and management outcome of endogenous endophthalmitis in Indian patients. Methods: Retrospective chart review of culture-positive (vitreous/urine/blood) endogenous endophthalmitis cases treated in tertiary eye care facility in India was done. Results: The study included 173 eyes of 117 patients. Mean patient age was 25.41 ± 20.46 years (median 24 years). Pre-disposing systemic illness could not be elicited in 79 (67.63%) patients. Commonest predisposing systemic condition in patients where it could be detected (n = 79) was pyrexia of unknown origin (25/79 = 32.0%). Following treatment, 45 out of 173 (26.0%) eyes regained vision of ≥20/400. Commonest isolated organism from vitreous was Streptococcus pneumoniae (36 eyes, 20.8%) and fungi were isolated in 24 (13.8%) eyes, the commonest being Candida spp. (8/24, 33.33%). Favorable functional outcome was seen in 26% eyes and favorable anatomic outcome in 43% eyes. Those with an underlying systemic illness were older (P = 0.02), had greater urine culture positivity (P = 0.003), lesser vitreous culture positivity (0.001), greater gram negative etiology (P = 0.0006), and greater fungal etiology (P = 0.01) as compared to those cases without underlying systemic illness. Conclusion: Endogenous endophthalmitis in India often presents in young immunocompetent individuals without any underlying systemic illness and with negative blood or urine microbiologic work up. Underlying systemic illness leads to greater gram-negative and fungal etiology. Overall visual outcome is poor inspite of prompt management.

Retinitis pigmentosa is one of the major forms of inherited retinal dystrophy transmitted in all Mendelian and non-Mendelian forms of inheritance. It involves the loss of retinal photoreceptor cells with severe loss of vision or blindness within the first 2 decades of life. RP occurs at a relatively high prevalence in India and is often associated with consanguinity in certain South Asian communities where this practice is customary. This review describes the studies that have been published with regard to genetics of retinitis pigmentosa in India and neighboring South Asian countries. These populations have been understudied in these aspects although to a variable degree from one country to another. Genetic studies on RP in India have been carried out with a range of methods aimed at detecting specific mutations, to screening of candidate genes or selected genomic regions, homozygosity mapping to whole genome sequencing. These efforts have led to a molecular genetic characterization of RP in Indian families. Similar studies on large extended families from Pakistan have provided insight into several novel genes underlying the pathogenesis of these diseases. The extreme degree of clinical and genetic heterogeneity of RP renders it challenging to identify the associated genes in these populations, and to translate the research output towards better management of the disease, as there are no unifying genetic features that are characteristic of any population so far.

Purpose A simple psychophysical paradigm is available as a digital application in iOS devices such as iPad to measure the function of ON and OFF visual pathways. However, an age-matched normative database is not readily available. The purpose of the study is to evaluate the response of ON and OFF visual pathways as a function of age. Methods 158 normal healthy adults (84 males and 74 females) whose age ranged 18–80 years participated in the study. None of them had any ocular disease (except cataract of grade II or less) and visual acuity of ≤ 20/25. Monocular testing (only one eye) was performed on the ‘EyeSpeed’ application on an iPad at 40cm distance. The targets ranged between 1 to 3 light or dark squares presented randomly in a noise background and participants responded by indicating the number of squares by touching the screen as fast as possible. The main outcome variables are reaction time, accuracy and performance index (1 / speed * accuracy). Results The median reaction time was shorter (Median (IQR): 1.53s (0.49) [dark] Vs 1.76s (0.58) [light], p < 0.001) and accuracy was higher (97.21% (3.30) [dark] Vs 95.15% (5.10) [light], p < 0.001) for dark targets than the light targets. Performance index and reaction time for both target types significantly correlated with age (ρ = -0.41 to -0.43; p < 0.001). Conclusions This normative database will be useful to quantify disease-specific defects. More importantly, the ON pathway function can potentially serve as a surrogate for rod photoreceptor function.

Background: The long-term treatment outcomes in Coat’s disease – particularly in the era of newer pharmacotherapies such as anti-vascular endothelial growth factor (VEGF) agents and depot steroids – are poorly understood. Aim: To describe the clinical features and treatment outcomes of 148 eyes with Coats’ disease assessed in a referral centre over 30 years. Materials and methods: We conducted a retrospective chart review of patients diagnosed with Coats’ disease between 1 June 1987 and 31 July 2017. The demographic, clinical and treatment data were collected and long-term functional and anatomical outcomes were analysed based on the treatment either with conventional therapy (cryo/laser) or along with adjuvants like intravitreal steroids or anti-VEGFs. Results: The mean age at presentation was 15.22 years (median 11). Familial exudative vitreoretinopathy was the most common referral diagnosis, 76/148 (51.5%), followed by Coats’ disease, 37/148 (25%), and retinoblastoma, 35/148 (23.6%). Stage 3B was most common at presentation (31.8%), followed by 2B (22.3%) and 2A (16.9%). A total of 107 patients were treated either with conventional therapy or in combination with adjuvants. The mean follow-up period was 24.95 months. The visual acuity improved from baseline logMAR 2.17 (Snellen-20/2958) to logMAR 1.88 (Snellen-20/1517) at final follow-up ( p = 0.004). The improvement in visual acuity was better when the presenting BCVA was

Purpose: The sub-retinal injections are not very commonly performed procedures in vitreoretina, but form a crucial step in any cell replacement therapy for retinal diseases. The purpose of this study is to describe the learning curve of a trained vitreo-retinal surgeon in sub-retinal injections in a rat model and its implications in future clinical trials. Methods: This is an in-vivo retrospective animal study using Wistar rats. All ARVO guidelines regarding animal handling were followed. After anesthetization, aspectic preparation and dilating the pupils with 1% tropicamide eye drops, subretinal injection of 10 μl saline was done via a limbal entry. Data recorded included time taken for the procedure, success of injection, associated complications, post-operative infections and complications. The rats were followed up for 1 month post procedure. A trend analysis was done for the above factors to look for improvement in ease of procedure, reduction in procedure time and reduction in complications for the clinician using a novel objective scale. Results: About 20 eyes were studied. Mean weight of the rats was 188 ± 12.82 gram. Mean time taken for the procedure was 14.1 ± 5.07 minutes. There was a significant inverse co-relation between the serial number of the eye and time taken for the procedure (r = −0.89, P < 0.0001). Comparative complications noted between the first ten and the last ten eyes were: conjunctival tear 30% versus 10% (P = 0.27), lens touch 50% versus 10% (P = 0.05), subretinal hemorrhage 40% versus 0% (P = 0.13), vitreous loss 30% versus 0% (P = 0.06). The successful subretinal injection without intraocular complications was achieved in 40% versus 90% (P = 0.02). There was a significant co-relation between the serial number of the eye and ease of the procedure (r = 0.87, P < 0.0001). Post operatively none of the eyes had any infection. Six eyes (12%) developed cataract and 3 eyes (6%) had non-resolving retinal detachment at the last examination visit. Conclusion: Subretinal injections in rats have a definite learning curve even for a trained vitreoretinal surgeon. This should be accounted for and resources allocated accordingly to achieve good technical comfort and negate confounding by the surgeon factor in the results of future clinical trials

Purpose: To evaluate the factors influencing timely versus delayed presentation of preterm babies for ROP evaluation. Methods: Preterm babies (≤35 weeks gestational age, ≤2000 g birth weight) were prospectively included in the study. Timely presentation was defined as babies who presented for the first ROP screening within 30 days of birth and Delayed as more than 30 days of birth. An event survey to assess factors influencing timely vs delayed presentation was administered to parents/guardian of babies after obtaining informed consent. Results: Data of 278 preterm babies (n = 139 timely vs n = 139 delayed presentation) collected in the event surveys were analyzed. The delayed presenters came at a median duration of 6.3 weeks (1st and 3rd quartiles: 5.3 and 9.1) after birth. The odds of any stage of ROP was 2.6 times and the odds of sight threatening ROP was 6.8 times in those presenting delayed compared to those presenting timely. Major Reasons for delayed presentation were not asked to do so/no referral from pediatrician in 64 (46%) participants and unaware of the importance by 46 (33%) participants. Conclusion: Deviation from screening protocol is an important modifiable risk factor in ROP screening. The study findings suggest the need for creating awareness about timely screening and referral guidelines among the pediatricians involved in “care” of preterm infants at risk of developing ROP.

Purpose: To analyze the causes for late presentation in a series of patients with advanced retinopathy of prematurity (ROP) in a tertiary eye care institute in Eastern India. Methods: We analyzed our medical records and ROP database retrospectively from 2007 to 2015 and prospectively thereafter till 2017 to identify the factors for late presentation in babies with advanced ROP (stages 4 and 5). Results: A total of 71 eligible subjects were analyzed. The mean chronological age was 15.1 months (2 months to 14 years). The three important barriers were: (1) the system and neonatal care policy failure (n = 45; 63.3%), (2) parental negligence and ignorance (n = 19; 26.7%), and (3) ophthalmologist's misdiagnosis or unavailability (n = 7; 10%). Majority of the babies (63.3%) were admitted in the neonatal care unit when they were due for ROP screening with an average duration of stay of 35.5 days. Conclusion: The main barriers to early screening for ROP were related to availability of trained human resources, ignorance of “parents and health care personnel,” and distance from the point of care. This calls for training of ophthalmologists, advocacy with neonatologists and parents, and create systems for better coordination and compliance of the care providers.

PurposeTo report the clinical presentation and management outcomes of glaucoma in the "Indian Twin cities retinopathy of prematurity (ROP) Screening database."MethodsAll children with diagnosis of ROP and glaucoma between 1997 and 2016 from a prospective database were included. Glaucoma was classified as open when anterior chamber (AC) was deep, closed when AC was shallow or flat and neovascular when there was extensive iris neovascularization. ROP was classified based on International classification of ROP.ResultsThe prevalence of secondary glaucoma in our cohort was 1.36% (82 eyes of 6000 children). Eighty-two eyes of 54 children with secondary glaucoma due to ROP where included in this study. The distribution of glaucoma among the ROP stages included, stage V (58.5%), stage 1V (24.3%), stage III (2.4%) and stage II (1.2%) eyes. Median (interquartile range) duration from birth to glaucoma diagnosis was 7.8 (4.2, 24.9) months. Type of glaucoma was angle closure in 39 (47.6%), open angle in 35 (42.7%) and neovascular in 8 (9.8%) eyes. Retinal interventions included vitreoretinal surgery in 59 (72%), retinal laser in 14 (17%) and intravitreal bevacizumab injection in 19 (23.1%) eyes. The mean (±standard deviation) IOP at presentation was 22.6 ±11.8 mm Hg. Glaucoma was managed medically in 66 (76%) and surgically in 16 (19.5%) eyes. The mean follow up for the entire cohort was 1.14±2.24 years. At final visit, 37% eyes with ROP and glaucoma had ambulatory vision with mean IOP of 16.0±8.1 mm Hg and 56 eyes (68.2%) needed glaucoma medications.ConclusionIn this large ROP cohort, 1.36% eyes developed secondary glaucoma. Majority of them had stage V or IV ROP and 1/5 of them needed glaucoma surgery. Around 1/3rd of the ROP eyes with glaucoma had ambulatory vision.

Purpose: To determine the predictive values of baseline optical coherence tomography (OCT) abnormalities on 12-month visual acuity changes in eyes with macular edema (ME) caused by branch retinal vein occlusions (BRVO). Methods: We performed a post hoc analysis of data from 75 participants in the 12-month MARVEL trial. OCT abnormalities at baseline, including ganglion cell layer cystoid spaces (GCL), intraretinal hyper-reflective dots, and central subfield thickness (CST), were correlated with improvements in visual acuity and the number of anti-vascular endothelial growth factor injections required using a multivariate regression model. Results: Eyes with baseline CST > 500 μm had greater visual gains compared to those with CST

Purpose: Retinopathy of prematurity (ROP) is now emerging as one of the major causes of preventable childhood blindness. The proportion of preterm babies has increased dramatically over the past decade. Our study aims to emphasize the need for ROP screening and management services in these preterm infants. Methods: ICD-coded medical records of children less than 10 years of age presenting to a subspecialty eye hospital from 2000 to 2017 were reviewed. ROP, congenital cataract, congenital glaucoma, and vitamin A deficiency were the most common diagnoses. We evaluated the trend of these diseases from 2000 to 2017. Results: Our data suggested a 20-fold increase in the attendance of children with a diagnosis of ROP who now make over 2% of outpatient children. Vitamin A deficiency has declined over time whereas cataract and glaucoma have remained stable. Conclusion: Our data indicate a need to scale up ROP screening integrated with neonatal care, as well as to build capacity for the treatment of acute and late-stage ROP in India.

Two siblings aged 9 and 15 years with unexplained visual loss had normal pupillary reactions, unremarkable anterior and posterior segment, normal fluorescein angiography, visual evoked potential, and flash electroretinogram (ERG). Spectral domain optical coherence tomography (OCT) showed loss of normal inner segment-outer segment (IS-OS) junction line bump at fovea in one and absent IS-OS junction line at fovea in the other. Characteristic hypovoltaged responses from central macula in multifocal ERG (mfERG) confirmed the diagnosis of occult macular dystrophy (OMD) in both siblings. Marked difference in OCT findings despite same visual acuity indicate that structural changes in OCT might not always correlate with the extent of functional loss. Obvious mfERG changes and very subtle OCT defect in the younger one suggests that functional changes probably appear much earlier than the structural changes. OMD is often underdiagnosed because of lack of high index of suspicion and detailed work up. The patients presented here represent first OMD report from India, one of them being the second youngest reported so far (medline search).

Retinopathy of prematurity (ROP) is a neurovascular complication in preterm babies, leading to severe visual impairment, but the underlying mechanisms are yet unclear. The present study aimed at unraveling the molecular mechanisms underlying the pathogenesis of ROP. A comprehensive screening of candidate genes in preterms with ROP (n = 189) and no-ROP (n = 167) was undertaken to identify variants conferring disease susceptibility. Allele and genotype frequencies, linkage disequilibrium and haplotypes were analyzed to identify the ROP-associated variants. Variants in CFH (p = 2.94 × 10−7), CFB (p = 1.71 × 10−5), FBLN5 (p = 9.2 × 10−4), CETP (p = 2.99 × 10−5), and CXCR4 (p = 1.32 × 10−8) genes exhibited significant associations with ROP. Further, a quantitative assessment of 27 candidate proteins and cytokines in the vitreous and tear samples of babies with severe ROP (n = 30) and congenital cataract (n = 30) was undertaken by multiplex bead arrays and further validated by western blotting and zymography. Significant elevation and activation of MMP9 (p = 0.038), CFH (p = 2.24 × 10−5), C3 (p = 0.05), C4 (p = 0.001), IL-1ra (p = 0.0019), vascular endothelial growth factor (VEGF) (p = 0.0027), and G-CSF (p = 0.0099) proteins were observed in the vitreous of ROP babies suggesting an increased inflammation under hypoxic condition. Along with inflammatory markers, activated macrophage/microglia were also detected in the vitreous of ROP babies that secreted complement component C3, VEGF, IL-1ra, and MMP-9 under hypoxic stress in a cell culture model. Increased expression of the inflammatory markers like the IL-1ra (p = 0.014), MMP2 (p = 0.0085), and MMP-9 (p = 0.03) in the tears of babies at different stages of ROP further demonstrated their potential role in disease progression. Based on these findings, we conclude that increased complement activation in the retina/vitreous in turn activated microglia leading to increased inflammation. A quantitative assessment of inflammatory markers in tears could help in early prediction of ROP progression and facilitate effective management of the disease, thereby preventing visual impairment.

Purpose: To determine the etiology, clinical features and outcomes of bilateral vitreous hemorrhage (VH) in children. Methods: This retrospective chart review was performed on patients with bilateral VH under the age of 18 at a tertiary eye care center in India. Data included demographics, details of history and ocular examination, reports of investigations, surgeries or other interventions performed, and final anatomical and visual outcomes. Patients were divided into two groups i.e., traumatic and non-traumatic (spontaneous). Results: The traumatic group was comprised of 37 patients including 27 male and 10 female subjects with mean age of 13.47 ± 5.31 years, the most common complaint was decreased vision (96.45%) and the most prevalent etiology was firecracker injury in 16 (43.2%) patients. Mean baseline visual acuity (VA) was 2.34 ± 1.31 logMAR which was significantly improved to 1.08 ± 0.23 logMAR (P = 0.042). The mean number of surgeries was 2.72 ± 1.43 in the traumatic VH and mean follow up period was 23.14 ± 6.54 months. The spontaneous group included 48 subjects comprised of 27 male and 21 female cases with mean age of 14.48 ± 2.03 years. The most common cause was vasculitis in 21 (43.75%) subjects including four patients with tuberculosis. Mean baseline VA was 1.97 ± 1.13 logMAR which showed a significant improvement to 0.82 ± 0.24 logMAR (P = 0.012) after mean follow up of 34.2 ± 11.2 months. Eleven patients required at least one major surgery. Conclusion: Vasculitis was the most common cause of spontaneous bilateral VH; traumatic VH most prevalently occurred due to firecracker injury. Final VA was better in the spontaneous group.

Purpose: To evaluate changes in choroidal thickness (CT) in inherited retinal diseases and its relationship with age, spherical equivalent, visual acuity, and macular thickness. Methods: Retrospective analysis of 51 eyes with features of retinal dystrophy of 26 subjects, who underwent enhanced depth imaging using spectral domain (SD) optical coherence tomography (OCT), were included. The CT measurements were made at the fovea and at 5 points with an interval of 500 microns in both directions, nasal and temporal from the fovea and were compared with age-matched healthy subjects. Step-wise regression was used to find the relationship between age, spherical equivalent, best-corrected visual acuity (BCVA), central macular thickness (CMT), and subfoveal CT. Results: Disease distribution was as follows: Stargardt′s disease 18 eyes (9 subjects); Best disease 5 eyes (3 subjects); cone-rod dystrophy 26 eyes (13 subjects); and Bietti′s crystalline dystrophy 2 eyes (1 subject). Mean subfoveal CT was 266.33 ± 76 microns. On regression analysis, no significant correlation was found between subfoveal CT and any other variable such as age (P = 0.9), gender (P = 0.5), CMT (P = 0.1), spherical equivalent (P = 0.3) and BCVA (P = 0.6). While comparing with age-matched healthy subjects, no significant statistical difference was noted (P < 0.05) among all age groups. Conclusion: Our study reports quantitative changes in CT in various common inherited retinal diseases seen in Indian populations. To validate changes in choroid, a longitudinal study with larger sample size is warranted.

Knowing how to classify retinopathy of prematurity is essential as it provides information on the prognosis and guides decision making about screening and treatment.

Blunt ocular trauma in the elderly can result in anterior dislocation of the crystalline lens into the subconjunctival space (phacocele). Although rare, this presentation can be missed, especially if the patient presents several days after the injury and if the lid is not everted on examination. While a careful clinical examination is adequate in the diagnosis, imaging techniques can be put to use for the accurate location of the associated sclera rupture. We report three cases of post-traumatic phacocele wherein ultrasound biomicroscopy (UBM) was compared to the anterior segment optical coherence tomography (AS-OCT) and B-scan ultrasonography (B-scan), in order to establish the best imaging tool for this condition. We concluded, based on image quality, that UBM could be the imaging modality of choice to aid in the diagnosis of phacocele.

The article reports a case and review of the literature of endophthalmitis presenting as isolated retinal vasculitis. A 26-year-old male was observed to have white-centered retinal hemorrhages and retinal vasculitis following an occult scleral perforation. At presentation, the visual acuity was 20/60. With clinical suspicion of early endophthalmitis, he underwent wound exploration, scleral tear repair, vitreous biopsy and administration of intravitreal antibiotics. Microbiology evaluation revealed significant presence of methicillin-resistant coagulase-negative Staphylococcus epidermidis. Final visual acuity improved to 20/20 at 6 weeks postoperatively. Literature search revealed eight similar cases, all of them due to Staphylococcus species. Retinal vasculitis and white-centered retinal hemorrhages can be a presenting sign of early endophthalmitis, especially with non-fulminant pathogens like S. epidermidis.

Retinopathy of prematurity can develop when babies are born before their retinal blood vessels are fully formed.

Purpose. We investigated acute endophthalmitis incidence following cataract surgery vis-a-vis the current technological and postoperative care changes in higher and lower socioeconomic categories of patients in South India. Methods. In a retrospective case control study, we analyzed 62 cases of acute endophthalmitis and 5 controls for each endophthalmitis case from 46,095 cataract surgeries done between years 1993 and 1998. The time period covered the transition of surgical technique and after care. In addition, we analyzed systemic diseases, surgeon factor, habitat, and socioeconomic status. Results. Clinical and culture positive endophthalmitis incidence were 0.13% and 0.07%, respectively. Differential incidence of 0.10% and 0.17% for in- and ambulatory care surgeries, respectively, was close to statistical significance (𝑃=0.054). Lower economy category ambulatory patients had higher risk of infection. Conclusion. Ambulatory cataract surgery carried additional risk for post-operative infection in lower socioeconomic group. Improved health education could ensure greater safety.

Detachment of the retina is a serious event, which may result in complete blindness. The outer segments of the photoreceptors receive oxygen and nutrition from the choroid. If the retina is detached from the choroid, the photoreceptors will fail. The fovea has no retinal blood vessels and depends wholly on the choroid for its oxygen, so detachment of the macula leads to permanent damage to the cones and rods at the posterior pole, and loss of vision. If the macula is not detached, then good vision can be retained if the retina is re-attached promptly.