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Objective: Assess safety and effectiveness of subretinal gene replacement therapy at 18 months post treatment., Design: Retrospective, longitudinal study conducted at the Hospital for Sick Children in Toronto, Canada., Participants: Patients with bi-allelic RPE65 variants, early onset retinal degeneration, and residual viable retina who underwent voretigene neparvovec r-zyl gene replacement therapy., Methods: Data collected included demographic information, molecular genetic results, and comprehensive ocular assessment results from preoperataive and postoperative visits up to 18 months. To assess the treatment's efficacy, postoperative best corrected visual acuity, full-field stimulus test (FST), visual field (VF) area, optical coherence tomography, and global satisfaction were compared to preoperative findings., Results: The procedures were safe with no complications. There was no significant improvement in visual acuity. Three eyes showed a slight reduction in VF area. All showed a reduction in ellipsoid zone thickness and area, but the outer nuclear layer thickness and area were stable. All had a significant improvement in retinal sensitivity, as per FST, allowing better navigation in a dim environment. All 3 patients reported being "very satisfied"., Conclusion: Following a safe gene replacement therapy, the 3 first Canadian cases had an improvement in retinal sensitivity as per FST, and our patients described their experience as positively life changing., (Copyright © 2025 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objective: Biallelic RPE65 pathogenic variants may cause Leber congenital amaurosis (LCA). Voretigene neparvovec-rzyl (VN, Luxturna) is the only approved subretinal gene therapy that demonstrated benefit and safety. The eligibility criteria are vague and variable between centres. This is the first comprehensive outcome report of RPE65-LCA patients with World Health Organization blindness criteria vision treated with VN., Design: Multicentre retrospective case series., Participants: Patients meeting the treatment criteria for VN who had best-corrected visual acuity (BCVA) <20/400 or visual field (VF)III4e isopter <10°., Methods: Patients were followed for a mean of 11.1 ± 4.7 months. Age, sex, BCVA, central retinal thickness (CRT), retinal atrophy, VF, full-field stimulus testing (FST), and subjective impressions were assessed., Results: Nine patients met the inclusion criteria (mean: BCVA 1.89 LogMAR, range: 1.4 - 2.7 LogMAR, mean age: 28.7-years-old, range: 17-59 years). Though VF area did not improve, FST improved in patients with better baseline FST (-8.83 dB vs -0.56 dB; p = 0.010), and better VFV4e (7245 vs 341 o2 ; p < 0.001) and III4e (596.1 vs 24.8 o2 ; p = 0.011) area. VA improved in younger (20 vs 32 years; p = 0.011) patients with thinner CRT 1mm (155 vs 193 µm; p = 0.038). VFV4e loss occurred in older (38 vs 19 years; p = 0.001) patients with worse baseline V4e area (1728 vs 8159 o2 ; p < 0.001). Subjective improvement in dim light navigation skills occurred in younger patients (20.3 vs 45.3 years; p < 0.001)., Conclusions: Blindness is not a contraindication to VN treatment for RPE65-LCA. Superior results correlated with greater baseline FST but not with CRT 1mm , provided that measurable outer retinal structures persist., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

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.)

Background : Inherited retinal diseases (IRDs) are a genetically complex group of disorders, usually resulting in progressive vision loss due to retinal degeneration. Traditional imaging methods help in structural assessments, but limitations exist in early functional cellular-level detection that are crucial for guiding new therapies. Methods : This review includes a systematic search of PubMed and Google Scholar for studies on advanced imaging techniques for IRDs. Results : Key modalities covered are adaptive optics, fluorescence lifetime imaging ophthalmoscopy, polarization-sensitive optical coherence tomography, optoretinography, mitochondrial imaging, flavoprotein fluorescence imaging, and retinal oximetry. Each imaging method covers its principles, acquisition techniques, data from healthy eyes, applications in IRDs with specific examples, and current challenges and future directions. Conclusions : Emerging technologies, including adaptive optics and metabolic imaging, offer promising potential for cellular-level imaging and functional correlation in IRDs, allowing for earlier intervention and improved therapeutic targeting. Their integration into clinical practice may significantly improve IRD management and patient outcomes.

Introduction: Mutations in BEST1 gene have been linked to the development of refractory angle closure glaucoma (ACG). This study aims to delineate the clinical characteristics, genetic mutations, and disease progression in patients with autosomal recessive bestrophinopathy (ARB) and autosomal dominant Best vitelliform macular dystrophy (BVMD) who are presented with treatment-resistant ACG., Methods: This retrospective analysis encompasses a comprehensive ophthalmic assessment, retinal imaging, and mutational profiling of six patients diagnosed with bestrophinopathy and concurrent ACG, with a particular emphasis on the risk of post-glaucoma filtration surgery malignant glaucoma (MG). Exome sequencing was conducted utilizing a next-generation sequencing (NGS) based gene panel., Results: The cohort included five patients with ARB and one with BVMD, with a mean (±SD) age at ACG diagnosis of 35.1 ± 6.9 years. NGS analysis revealed homozygous BEST1 variants in four patients (ARB; cases 1-4) and a heterozygous BEST1 variant in one patient (BVMD; case 5). One patient (ARB; case 6), despite a recessive pedigree, showed a single heterozygous variant, suggesting the presence of an undetected heterozygous variant indicative of compound heterozygous autosomal recessive inheritance. A novel non-frameshift deletion (c.841&#95;843delTTC; p.Phe281del) was identified in case 2. Surgical intervention was required due to uncontrolled glaucoma in all cases except case 4. All five cases that underwent glaucoma filtration surgery developed MG, which was effectively managed with combined iridozonulo-hyaloido-vitrectomy (IZHV) and pars plana vitrectomy (PPV). Cases 5 and 6, harboring a heterozygous pathogenic variant (c.241 G>A; p.Val81Met), experienced refractory MG and corneal decompensation necessitating multiple interventions., Conclusion: Genomic analysis plays a pivotal role in the management of bestrophinopathies with ACG. Characterization of mutational types facilitates prognostication and enables timely interventions. IZHV with PPV emerges as a promising standalone or adjunctive procedure for the management of glaucoma among patients with BEST1 mutations and ACG.

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.)

Introduction: Physicians need an accurate understanding of diabetic retinopathy (DR) severity to optimally manage patients. The aim of this prospective study is to correlate the severity of macular and peripheral retinal vascular abnormalities seen on widefield (WF) optical coherence tomography angiography (OCTA) with DR grading based on WF fundus photography., Methods: The study included 150 eyes from 82 patients with treatment-naïve DR. All patients were imaged with WF fundus photography and swept-source WF OCTA. Quantitative and qualitative analyses of the foveal avascular zone (FAZ) size and shape, and measurement of capillary nonperfusion (CNP) areas, were performed from the OCTA images. The mixed-effects model was used to compare the DR grading from WF photography with the vascular changes seen on WF-OCTA, and Bonferroni correction was applied to the gradings., Results: The mean [± standard deviation (SD)] age of patients was 55.5 (± 9.4) years. The WF-OCTA showed that an increasing size of the FAZ (from 0.442 (± 0.059) µm to 0.933 (± 0.086) µm) correlated with increasing severity of the DR (as determined with WF photography). The deep capillary plexus, FAZ size, and CNP areas in eyes with proliferative diabetic retinopathy (PDR) differed from those with mild nonproliferative diabetic retinopathy (NPDR) (p < 0.001). Most eyes with severe nonproliferative DR were found to have CNP in four quadrants [superficial capillary plexus (SCP) 60%, deep capillary plexus (DCP) 50%]. The WF-OCTA detected subtle neovascularization of the disc (NVD) in 7 eyes (10%) and neovascularization elsewhere (NVE) in 13 eyes (18%) that had been diagnosed with only moderate NPDR on WF photography., Conclusions: FAZ and CNP areas as measured by WF-OCTA correlate with DR severity. WF-OCTA can also detect subtle NVE and NVD that cannot be seen with fundus photography., (© 2024. The Author(s).)

Purpose: To study the inner and outer retinal functions using a full-field electroretinogram (ERG) before and after intravenous chemotherapy (IVC) in children with retinoblastoma (RB)., Methods: Of the 11 eyes, seven had RB and four were normal. All children were examined under anesthesia using a handheld ERG machine with a standard protocol - light-adapted single-flash ERG (fERG), photopic single-flash 3.0- and 30-Hz flickers, and photopic negative response (PhNR) amplitudes at 72 ms (P72). The amplitudes and peak times were compared before and after IVC., Results: Post-chemotherapy tumor regressed in all seven eyes. Of the seven eyes, the fERG peak time (a-wave) was delayed in two eyes (29%), whereas the b-wave was delayed in six eyes (86%). The fERG amplitude height for a- and b-waves decreased in five eyes (71%) and six eyes (86%), respectively. In addition, photopic flicker 30-Hz b-wave peak time delayed in five eyes (71%), whereas the b-wave amplitude height decreased in six eyes (86%). Simultaneously, the P72 amplitude height decreased in six eyes (86%), whereas the P-ratio increased in all seven eyes (100%). In comparison, the ERG responses improved in three of the four contralateral normal eyes. Overall, the cone function improved in two eyes (29%), whereas cone bipolar cell and retinal ganglion cell (RGC) function improved in one eye (14%) each., Conclusion: Comparison of light-adapted ERG changes before and after IVC showed reduced amplitudes and delayed peak times for both a and b waveforms, as well as reduced PhNR amplitude attributable to bipolar and RGC injury., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Purpose: To report a case of active retinochoroiditis in a neonate treated with intravitreal clindamycin as an adjuvant therapy., Case Report: A 10-day-old infant presented with active retinochoroiditis lesions in both eyes along with hepatosplenomegaly, abdominal distension, and thrombocytopenia. The mother had a history of fever in the third trimester for which she received symptomatic treatment. The infant was treated with systemic anti-toxoplasma therapy along with intravitreal clindamycin in the severely affected eye. There was a significant faster resolution in the retinochoroiditis lesions, which eventually lead to better anatomical and visual outcomes., Conclusion and Importance: Active screening and timely intervention of the neonates who are born to mothers with fever during pregnancy can go a long way in preventing unlikely outcomes due to congenital toxoplasma retinochoroiditis. Intravitreal clindamycin can be considered as an adjuvant therapy in selected cases.

Introduction: Mutations in the retinal pigment epithelial 65 kilodalton protein ( RPE65 ) gene are associated with various inherited retinal diseases (IRDs), including Leber congenital amaurosis (LCA), early-onset severe retinal dystrophy (EOSRD), and retinitis pigmentosa (RP). We screened for mutations in RPE65 in a series of Indian patients with these IRDs to determine the frequency/types of mutations and to describe the associated phenotypes., Materials and Methods: Diagnosis of LCA, EOSRD, and RP was made by standard and pre-defined criteria. Patients were evaluated by clinical, retinal imaging, and electrophysiological parameters. Genomic DNA from patients and available family members were used for identifying mutations by direct Sanger sequencing of the RPE65 gene or targeted NGS gene panel for IRDs covering 260+ genes. Variations detected were tested in healthy control populations and for co-segregation with the disease in available family members., Results: Mutations were found in eight patients, out of 220 total cases screened, all homozygous for the respective mutant alleles. Seven patients had mutations leading to premature termination codons and one patient had a missense change. The onset of visual loss ranged from birth to <2 years of life. At presentation, RPE mottling in the background retina was present in all cases with macular involvement in five cases with or without vascular attenuation and optic disc pallor., Conclusion: RPE65 mutations in this series were found in 3.6% of cases associated with severe, early-onset disease, with consistent RPE mottling and variable manifestations with regard to the extent of disc pallor, arteriolar attenuation, and appearance of the macula.

Purpose: To evaluate the factors influencing the refractive outcomes following silicone oil tamponade (SOT) and silicone oil removal (SOR) in different lens statuses post-vitreoretinal surgery., Design: Retrospective analysis of three different lens statuses., Materials and Methods: This was a descriptive study that included 150 eyes of 147 patients who had undergone pars plana vitrectomy with SOT and SOR between January 2017 and June 2021. Demographic profile, spherical equivalent refraction (SER), and its association with clinical features were evaluated with SOT and post-SOR., Results: The mean (±standard deviation [SD]) age was 47 ± 17.8 years, including all three groups. SER was represented in diopters (D). The mean ± SD refraction with SOT in phakic, pseudophakic, and aphakic was 4.28 ± 2.59 D, 2.94 ± 2.58 D, and 3.98 ± 4.82 D. The mean SER post-SOR in phakic, pseudophakic, and aphakic was -2.72 ± 2.03 D, -1.12 ± 1.41 D, and 8.22 ± 3.70 D. The diagnosis of rhegmatogenous retinal detachment (RRD) among 96 eyes (64%) is the common indicator to perform vitreoretinal (VR) surgery. A minority of subjects were managed with retinal lasers before VR surgery (14%). The macula was attached in 100 eyes (67.6%), the belt buckle was done in 37 eyes (24.7%), and the silicone oil viscosity with 1000 centistoke was chosen in 129 eyes (86%)., Conclusion: SOT was used as a tamponade in VR surgeries irrespective of lens status. The significant predictor for post-SOR refraction in phakic and aphakic is post-SOT refraction. In pseudophakic, gender and diagnosis of RRD are the predictors of SOR refraction., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Oman Ophthalmic Society.)

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.)

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: X-linked retinoschisis (XLRS) is a rare inherited bilateral retinal degeneration caused by mutations in RS1 gene, occurring exclusively in men. Various ocular complications associated with XLRS are reported, and angle closure glaucoma in these eyes is one such complication that is refractory and needs surgery for intraocular pressure control. Glaucoma surgery in these eyes often results in refractory malignant glaucoma with its serious sequelae. Several surgical modifications to prevent this complication have been tried with no or limited success., Methodology: In this report, we present a case of XLRS in a young male with a 22-year follow-up. We have described the natural history and progression of retinal disease and glaucoma., Results: Refractory angle closure glaucoma in our patient was treated with core vitrectomy, phacoemulsification with intraocular lens implantation, and irido-zonulo-hyaloido-vitrectomy. This helped in successful deepening of anterior chamber, good IOP control, and preventing malignant glaucoma., Conclusion: Our case highlights the role of vitrectomy in managing the secondary angle closure glaucoma in eyes with X-LRS.

Purpose: LAMA 1 gene as a pathologic variant leading to cerebellar dysplasia and cysts, nonprogressive ataxia, language, and motor developmental delay without any muscular involvement was recently described as Poretti-Boltshauser syndrome (PBS). Ocular involvement is a common associated feature in this neurodegenerative disorder. In this case report, we describe the retinal changes associated with Poretti-Boltshauser syndrome., Methods, Patient, and Results: A 4-year-old female child presented with the progressive decreased vision for the past 6 to 8 months. Ophthalmic examination revealed mild myopia and ocular motor apraxia with retinal disruptions appearing as holes that were confined only to inner retinal layers. The child also had motor and speech developmental delays. Magnetic resonance imaging of the brain showed vermis hypoplasia with cerebellar dysgenesis and multiple cystic spaces in both cerebellar hemispheres. Whole exome sequencing revealed a homozygous pathogenic variant of exon 2-63 deletion in the LAMA 1 gene, which was confirmatory for Poretti-Boltshauser syndrome., Conclusion: Oculomotor apraxia and retinal changes can lead to visual disturbances in Poretti-Boltshauser syndrome. Identification of these features and prompt rehabilitative measures can improve the quality of life of these children.

Background: Ultrasound biomicroscopy (UBM) is a noninvasive imaging modality that enables in-vivo visualization of the structures of the anterior segment of the eye. Unlike routine ophthalmic diagnostic ultrasound which uses frequencies of 5-10 MHz, UBM utilizes ultrasound frequencies in the range of 50-100 MHz. The high-frequency probes in UBM allows for higher resolution and better visualization of subsurface ocular structures, even in the presence of anatomic or pathological obscuration. UBM has qualitative as well as quantitative applications in various disorders affecting the anterior segment of the eye. Despite its huge importance, many clinicians lack in knowledge about the technique and its clinical usefulness. The current educational video aims to address this gap in knowledge by highlighting the technique and various clinical indications of UBM., Purpose: The purpose of this video is to demonstrate the technique of UBM and showcase its quantitative and qualitative implications and importance through various clinical cases., Synopsis: UBM is an imaging technique that assesses the depth of tissue structures by measuring the time delay of the returning ultrasound signal. This modality is capable of measuring the size of various structures within the eye, such as the cornea, iris, ciliary body, sclera, and the depth of the anterior and posterior chamber. To perform a UBM, a transducer is inserted into a specially designed eye cup filled with distilled water, creating a water bath environment. Axial and longitudinal scans can be performed in a similar fashion as in routine diagnostic B-scan ultrasound. Quantitative indications for UBM depicted in this video include measurements of corneal thickness, depth of the anterior chamber, and the width of the angle. The video also showcases how UBM can aid in the diagnosis and management of various anterior segment disorders like angle-closure glaucoma, plateau iris configuration, secondary glaucoma, and anterior uveitis with complicated cataract. Qualitative indications for UBM highlighted in this video include its role in intermediate uveitis, ocular hypotony, ocular surface tumors, cystic lesions of iris, and identifying the location and type of intraocular foreign bodies in the anterior segment based on the type of artifact seen. Additionally, the video shows the applications of UBM in scleral and episcleral pathologies., Highlights: This video will educate clinicians about the technique of UBM and showcase a bouquet of UBM findings in various case scenarios, helping one to better understand the potential of this modality in clinical practice., Video Link: https://youtu.be/F626TMbJXoU., Competing Interests: None

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., Competing Interests: None

Purpose: To describe the demographics, clinical profile, and outcomes of ocular siderosis in patients presenting to a multi-tier ophthalmology hospital network in India., Methods: This cross-sectional and hospital-based study included 3,082,727 new patients who presented between August 2010 and December 2021. Patients with a clinical diagnosis of ocular siderosis in at least one eye were included., Results: Overall, 58 eyes of 57 patients (0.002%) were diagnosed with ocular siderosis. The majority were men (96.49%) and had unilateral (98.25%) affliction. The most common age group at presentation was during the third decade of life with 24 patients (42.11%). A clear history of ocular trauma was documented in 47 patients (81.03%). Major clinical signs included corneal pigment deposition in nearly half of the eyes (27/58 eyes, 46.55%), corneal scar (20/58 eyes, 34.48%), cataract (22/58 eyes, 37.93%) and retinal detachment (11/58 eyes, 18.96%). The intraocular foreign body (IOFB) was anatomically localized in a majority of the eyes (i.e., 45/58 eyes, 77.59%). The most common location of the IOFB was in the posterior segment (22/58 eyes, 37.93%). The eyes that underwent a vitreoretinal surgery with removal of IOFB had a slightly better BCVA (1.0 ± 1.01) when compared to eyes with non-removal of IOFB (1.58 ± 1.00)., Conclusion: Ocular siderosis is a rare sight-threatening entity, with half of the affected eyes exhibiting severe visual impairment. Majority of the eyes in ocular siderosis will have a detectable IOFB. Surgical removal of IOFB may lead to a better visual gain when compared to non-removal., Competing Interests: None

Purpose: To report the disease pattern, progression and imaging characteristics in eyes with bilateral central serous chorioretinopathy (CSCR)., Methods: This was a retrospective case review of bilateral CSCR patients with active disease in at least one eye. Multimodal imaging including fundus photography, fundus autofluorescence, optical coherence tomography (OCT), fluorescein and indocyanine angiography (FA/ICGA) was done at baseline and follow-up visits. Disease classification was done using recently described classification criteria. The degree of asymmetry in the disease distribution pattern at baseline and disease progression during follow-up visit with a minimum duration of 12 months was studied., Results: Among 103 CSCR patients, 36 patients (34.95%) with mean age of 53.6 ± 10.5 years had bilateral CSCR at baseline. Five patients (13.9%) had asymmetrical disease i.e. simple in one eye and complex in fellow eye. The remaining 31 patients had symmetric disease (simple, 2; complex 29). Mean duration of follow up was 17.58 ± 13.84 months. There was no significant difference between both eye parameters at last follow up (best corrected visual acuity, BCVA; central macular thickness, CMT; and subfoveal choroidal thickness, SFCT) (all p > 0.05). At last follow up, 22 eyes (2 simple and 20 complex) remained active whereas none of the eyes converted from simple to complex CSCR., Conclusion: Bilateral disease was more commonly seen with complex CSCR in contrast to simple CSCR. Moreover, disease distribution in complex CSCR had symmetric pattern if bilateral disease was present. None of the simple CSCR eyes converted to complex type., (© 2021. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

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) and 6 had hyperopia with 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., Competing Interests: None

Identification of ocular manifestations in patients with sensorineural hearing loss (SNHL) can have a large impact on the outcome and treatment of pediatric patients. Due to the common co-incidence of ocular manifestations and SNHL in children, both ophthalmologic and hearing loss screening and routine examinations must be conducted to minimize adverse outcomes and worsening of pathology. Early evaluation and diagnosis is imperative for intervention and further development of the patient. Co-incidence requires a thorough evaluation that includes a comprehensive history, examination, and diagnostic testing. In this article, a literature review was conducted to analyze the presentations of various diseases and syndromes, such as Alport Syndrome, Waardenburg Syndrome, Norrie Disease, Usher Disease, Stickler Syndrome, Marfan Syndrome, Congenital Rubella, and Hereditary Optic Neuropathies. We divided the various ocular pathologies into anterior and posterior segment presentations and associated systemic findings for better understanding. Additionally, this review aims to include an update on the management of patients with both ocular and hearing loss manifestations., Competing Interests: The authors declare no potential conflicts of interest for this article's research, authorship, and/or publication., (Copyright © 2022 Zaheer et al.)

Purpose: To assess the clinical characteristics of comorbid retinal dystrophies and primary angle closure disease., Design: Retrospective study from January 1992 to June 2020., Methods: This descriptive study included 92 eyes of 46 patients with comorbid retinal dystrophies and primary angle closure disease (PACD) that included eyes with primary angle closure suspect, primary angle closure and primary angle closure glaucoma. Demographic profile, clinical characteristics of PACD and its association with retinal dystrophies are described., Results: The study included 46 patients (92 eyes). Males were majority, 63%. Mean (± standard deviation) age when retinal dystrophy was diagnosed was 29.6 ± 9.4 years and PACD was diagnosed at 32.23 ± 7.92 years. Mean BCVA at presentation was 1.07 ± 0.87 log MAR [95% confidence interval (CI) 0.87, 1.26]. Mean Intraocular pressure at diagnosis of glaucoma was 27 ± 16 mmHg (95% CI 23.5, 31.5 mmHg). The most common retinal dystrophy associated with PACD was retinitis pigmentosa (RP) followed by RP with retinoschisis. The hospital-based prevalence of PACD among all patients with RP and retinoschisis was 0.19% and 0.15% respectively. Laser peripheral iridotomy was performed in 74 eyes (80.5%). Glaucoma was managed medically in majority of the eyes (58 eyes, 63.04%) and minority required surgical management with trabeculectomy (11, 11.9%)., Conclusion: Retinitis pigmentosa is the most common retinal dystrophy associated with PACD. Comorbid PACD in eyes with retinal dystrophies was observed in second to third decade of life. This calls for screening for angle closure in eyes with retinal dystrophies from second decade onwards to identify the comorbid PACD and treat or refer them appropriately., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

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., Competing Interests: None

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

We report a case of a 65-year-old female who presented to us with diminution of vision in the right eye. She was only able to perceive light in the right eye, and the left eye had a vision of 20/20, N6. Anterior segment examination in both eyes was unremarkable except for senile cataract in the left eye. Posterior segment examination revealed features of choroidal metastasis in both eyes and exudative retinal detachment in the right eye. Multimodal imaging helped in the further confirmation of metastatic lesions. Right-eye fundus autofluorescence showed hyperautofluorescent lesions, ultrasound B-scan showed an elevated mass lesion in the choroid with moderate to high internal echogenicity, and optical coherence tomography showed a lumpy-bumpy appearance of the retinal pigment epithelium as well as an elevated choroidal mass lesion beneath it. On detailed systemic evaluation, the primary site of cancer was found to be the lungs. The patient was referred to a pulmonologist and an oncologist for chemotherapy and further management., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2022 Jain et al.)

AimTo describe the clinical presentation and demographic distribution of retinitis pigmentosa in patients presenting to a multi-tier ophthalmology hospital network in India and implications for potential treatments.DesignCross-sectional hospital-based study.MethodsThis study included 2,541,810 patients presenting between March 2012 and October 2020. Patients with a clinical diagnosis of retinitis pigmentosa in at least one eye were included as cases. The data were collected using an electronic medical record system.ResultsOverall, 15,062 (0.59%) new patients were diagnosed with retinitis pigmentosa and were included for analysis. The median age was 35 (IQR: 21-49) years and adult age (84.56%) presented more often. The most common presenting age group was between 21 and 30 years (20%). Majority of patients were male (61.68%) and had bilateral (96.39%) affliction. There was a family history in a tenth of the patients (10.46%) and a history of consanguinity in a minority (5.53%). The majority of the eyes had mild or no central visual acuity impairment of <20/70 (34.25%) followed by blindness <20/400 to 20/1200 (21.26%). The predominant retinal signs included disc pallor (76.43%), attenuated arterioles (82.61%) and bony spicule pigmentation (90.15%), and almost all had retinal pigmentary changes. A minority of the eyes underwent a surgical procedure (4.96%).ConclusionRetinitis pigmentosa is commonly bilateral and predominantly affects males. Most patients present in the third decade of life with severe visual acuity impairment. More than half of the eyes from our study are potential candidates for newer therapies. These results have implications for upcoming treatment choices and planning.

Purpose: To assess the influence of fellow eye information on diagnosis and classification of central serous chorioretinopathy (CSCR) using multimodal imaging-based classification., Methods: This was a retrospective, observational study including patients with CSCR with unilateral or bilateral involvement. Multimodal images of both eyes of each patient were compiled and presented to two masked retina specialists subdivided into three groups: (1) both eye data, (2) right eye data and (3) left eye data. The masked observers graded the CSCR as per the new CSCR classification into simple and complex CSCR in three different scenarios as subdivided above. Interobserver and intraobserver agreement was assessed using Cohen's kappa (95% confidence intervals)., Results: A total of 206 eyes of 103 patients with unilateral or bilateral CSCR were graded. We found a "strong" intraobserver and interobserver agreement when one eye versus both eye data was provided in terms of "simple CSCR" or "complex CSCR" (kappa value = 0.77 and 0.87, p < 0.001, and kappa value = 0.85 and 0.76, p < 0.001, respectively). Forty-three eyes (10.55%) showed discrepancy in classification between observers for individual eyes, whereas only 13 eyes (6.53%) showed discrepancy between observers when both eye data was provided., Conclusion: We conclude that fellow eye information was helpful in solving diagnostic dilemmas and reached 85% consensus in the diagnosis of CSCR between the observers. We found that information of fellow eyes led to a discrepancy only in 6.53% cases with 2.42% cases that had a controversial diagnosis of CSCR. Multimodal imaging-based CSCR classification provides objective approach to diagnose and classify CSCR., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Conventional cataract surgeries can be a challenge in eyes with microcorneas due to difficult manipulation in a crowded anterior chamber (AC) and can lead to a high occurrence of corneal edema and an increased rate of complications. Similarly, in cases of dense brunescent cataracts even a lensectomy through posterior approach may prove to be difficult because of inadequate visualization. An endoscopyassisted pars plana lensectomy can help in obviating these difficulties. This study aims to describe the surgical technique of endoscopy-assisted pars plana lensectomy in eyes with brunescent cataract, microcornea, and microphthalmos., Methods: Retrospective review of two cases where endoscopy-assisted pars plana vitrectomy and lensectomy was performed for patients with dense cataract and microcornea and microphthalmos., Result: Complete clearance of the cataract was achieved without having to resort to a sclerocorneal incision. Postoperatively the cornea was clear and there was no postoperative corneal edema., Conclusion: Endoscopy-assisted pars plana lensectomy can help in overcoming the challenges of conventional anterior and posterior approaches of cataract extraction in cases of microcornea with reduced intraoperative and postoperative complications.

Purpose: Vitrectomy with brilliant blue G (BBG) assisted internal limiting membrane (ILM) peeling is the standard operational technique in macular hole surgeries. However, BBG dye, though considered safe and nontoxic, can also occasionally lead to macular toxicity. This study aims to describe the clinical features and characteristics of four eyes who developed macular toxicity after following surgery for macular hole repair., Methods: Retrospective review of four consecutive cases of macular toxicity after conventional BBG assisted ILM peeling. All the cases reviewed, their operative surgical notes were retrieved and analyzed. The ILM was stained twice during surgery with prolonged intraoperative surgical time., Results: All four cases had a prolonged surgical time and the ILM was stained twice during surgery in all cases. The area of macular toxicity was corresponding to the area of ILM peeling which had been exposed to repeated staining by BBG dye. By the end of one month, all four cases had foveal thinning along with choriocapillary atrophy. The mean BCVA was 20/80 before surgery and the final mean visual acuity was <20/800., Conclusion: This report highlights the occurrence of macular and choriocapillary atrophy due to prolonged focal endoillumination and the increased risk of toxicity with repeated dye staining.

Purpose: To present clinical profile and risk factors of sight-threatening diabetic retinopathy (STDR) among people with age of onset of diabetes (AOD) <25 versus ≥25 years., Methods: A retrospective chart analysis of consecutive patients with diabetic retinopathy (DR) n = 654) treated at 14 eye care centers across India between 2018 and 2019 was performed. Patients were divided into two groups, Group 1: AOD <25 years and Group 2: AOD ≥25 years. DR and diabetic macular edema (DME) were classified using the International Clinical Classification of DR severity scale. STDR included severe nonproliferative DR (NPDR), proliferative DR (PDR), and moderate to severe DME. A multilevel mixed-effects model was used for comparison between two groups: 1) Patients with DR and AOD <25 years and 2) Patients with DR and AOD ≥25 years. Bivariate and multivariate regression analyses were used to evaluate risk factors between the two groups., Results: A total of 654 patients were included, 161 (307 eyes) in AOD <25 and 493 (927 eyes) in AOD >25 group. There was a higher prevalence of PDR with high-risk characteristics in AOD <25 group (24% vs. 12%) at baseline and 12-month follow-up (25% vs. 6%); P < 0.001. Systolic hypertension and poor glycemic control were risk factors in both groups, with no difference in these modifiable risk factors between groups., Conclusion: People with youth-onset DM are likely to present with severer form of STDR despite similar modifiable risk factors. Therefore, strict control of systolic blood pressure, glycemic status, and regular screening for DR are recommended to reduce the risk of STDR irrespective of the age of onset of diabetes., Competing Interests: None

We report a case of a 57-year-old man, who underwent right eye silicone oil injection as a part of his surgical treatment for rhegmatogenous retinal detachment. Following this, on the first postoperative day, he developed acute postoperative intraocular inflammation. There was circumciliary congestion, diffuse corneal oedema, anterior chamber (AC) reaction, hypopyon and a pupillary membrane. The AC had not been entered during the surgery. No patients, who had been operated on the same day, or with the materials having the same batch number, developed similar reactions. We started the patient on hourly topical steroids and cycloplegics after which the patient's condition showed gradual improvement. At the 2 weeks follow-up appointment, his pupillary membrane had completely contracted and the AC was quiet. This case highlights a clinical picture of acute postoperative silicone oil-induced ocular inflammation, which was resolved through medical management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To describe optical coherence tomography (OCT) characteristics of central serous chorioretinopathy (CSCR) without any hyperfluorescent leakage on fundus fluorescein angiography (FFA)., Methods: This was a multicentric, retrospective, observational study of ten eyes of ten patients with CSCR without any hyperfluorescence leakage on FFA. Baseline patient characteristics, best corrected visual acuity, and OCT parameters like relative retinal pigment epithelium (RPE) reflectivity at the presumed leak site and control site were measured., Results: Increased macular thickness, neurosensory detachment, and choroidal thickness were seen at the site of maximum subretinal fluid (SRF). Out of ten eyes, nine had photoreceptor outer segment (PROS) disruption (46% ± 26.33%) at the site of SRF pocket, and five had presumed former leak site characterized by PROS thinning. The presumed leak site demonstrated higher RPE reflectivity compared to the control site (0.92 ± 0.04 vs. 0.87 ± 0.04; P = 0.0058)., Conclusion: CSCR without hyperfluorescent leakage on FFA may have PROS damage and changes in RPE hyperreflectivity., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Current Ophthalmology.)

Background: To report the clinical and angiographic characteristics of choroidal neovascularization in patients with diabetic retinopathy., Methods: Patients of type 2 diabetes mellitus with presence of choroidal neovascularization in at least one eye were retrospectively analyzed. The study eyes were divided into three groups based on presence (active or scarred) or absence of choroidal neovascularization (fellow eyes). Imaging characteristics of active choroidal neovascularization were recorded using optical coherence tomography, fluorescein, and indocyanine angiography. Central macular thickness, subfoveal choroidal thickness, and large choroidal vessel layer thickness were compared at baseline and final visit., Results: Our study reports the prevalence rate of choroidal neovascularization in eyes with diabetic retinopathy (0.27%; 36 out of 13,382 eyes). A total of 64 eyes of 32 patients (age, mean ± standard deviation: 68.5 ± 9.3 years) with baseline visual acuity of 0.69 ± 0.69 logarithm of minimum angle of resolution (Snellen equivalent 20/100) were included. Nonproliferative diabetic retinopathy (57 eyes) comprised the majority followed by proliferative diabetic retinopathy (7 eyes). Eyes with choroidal neovascularization (36, 56.25%) included both active (25) and scarred (11) choroidal neovascularization, with bilateral choroidal neovascularization in 4 patients. Type 1 choroidal neovascularization was the most common subtype of choroidal neovascularization on optical coherence tomography. Common etiologies for active choroidal neovascularization included age-related macular degeneration (3; 12%), myopia (1; 4%), and inflammatory choroidal neovascularization secondary to chorioretinitis (1; 4%). In the remaining 20 eyes, choroidal neovascularization formation was primarily due to diabetic choroidopathy., Conclusion: The prevalence of choroidal neovascularization in eyes with diabetic retinopathy is very low, with a lower prevalence of age-related macular degeneration. Diabetic choroidopathy plays a significant role in formation of choroidal neovascularization in eyes with diabetic retinopathy.

A 38-year-old man presented with sudden decreased vision in the right eye 3 years ago due to vitreous haemorrhage. During follow-up, right eye fundus showed evidence of vasculitis, non-perfusion areas and neovascularisation elsewhere. Systemic evaluation findings of positive Mantoux test, QuantiFERON Gold test and right apical pleuroparenchymal fibrosis observed on high-resolution CT of the chest were suggestive of postinfection probable tubercular aetiology. He was treated with oral steroids, antitubercular therapy, intravitreal bevacizumab and anterior retinal cryopexy, leading to resolution of vasculitis and vitreous haemorrhage. Later he developed peripheral retinal flap and posterior retinal breaks at 8-month and 11-month follow-up, respectively, which were managed by barrage laser. He maintained a stable visual acuity of 20/20, N6 for the next 2 years. He then presented with sudden decreased vision in the right eye (20/50, N10). Right eye fundus showed posterior pole retinal detachment with lifting of previously barraged posterior retinal breaks. He underwent vitreoretinal surgery with gas tamponade. Recent 1-month postoperative visit showed successful retinal reattachment and visual recovery of 20/20, N6., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

A 44-year-old woman presented with decreased vision in both eyes. The retina in both eyes had drusen distributed along vascular arcades, central macula and in peripapillary region. Macula had pigmented scarring and exudation. Fundus autofluorescence showed drusen. Optical coherence tomography showed drusen, subretinal and intraretinal fluid. Fundus fluorescein and indocyanine green angiography showed drusen, retinal pigment epithelial atrophy and vascular network. Younger age at presentation, bilateral symmetry, typical distribution of drusen along the arcades in a radiating pattern, peripapillary involvement, scarring and atrophy at macula were suggestive of doyne honeycomb retinal dystrophy. The reduced vision was due to macular atrophy and an active choroidal neovascular membrane. The patient was treated with antivascular endothelial growth factor injections for choroidal neovascular membrane. Our case highlights the importance of pattern recognition and multimodal imaging for diagnosing the type of macular dystrophy as doyne honeycomb retinal dystrophy, while simultaneously managing choroidal neovascular membrane., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To describe retinal pigment epithelium (RPE) disease detected by fundus autofluorescence (FAF) imaging in eyes with idiopathic choroidal neovascularization (ICNV)., Methods: A retrospective review of patients seen during a 14-month period with the diagnosis of ICNV was performed to identify patients with RPE disease, defined as hypo or hyperautofluorescent lesions on FAF. The presence of ICNV was confirmed by clinical history, ophthalmoscopic examination, fluorescein angiography (FA), and spectral domain-optical coherence tomography (SD-OCT). The clinical diagnosis of an underlying inflammatory condition was based on the FAF appearance of multiple punched-out hyper or hypoautofluorescent spots in the retinal fundus., Results: The mean age was 27 years (range, 21-33 years). Best-corrected visual acuity ranged from 20/25 to 20/200 with a median visual acuity of 20/80. Ten eyes of 8 patients presented RPE abnormalities on FAF. Of the 10 study eyes, ICNV was observed in 8 eyes. ICNV appeared as a type 2 neovascular membrane at the macular area on FA, and SD-OCT revealed neurosensory detachment in all study eyes. FAF demonstrated abnormalities of the RPE that were not appreciated on clinical examination or by other imaging modalities., Conclusions: FAF may reveal an underlying inflammatory condition in patients diagnosed as ICNV, modifying the diagnosis and management., Competing Interests: None., (© 2020 Published by Elsevier Inc.)

Purpose: To study the early anatomic choroidal alterations in eyes with chronic central serous chorioretinopathy (CSCR) undergoing photodynamic therapy (PDT)., Design: Multicenter retrospective cohort study., Methods: A total of 77 patients and 81 eyes with chronic CSCR treated with PDT and 64 untreated fellow eyes were evaluated. Central macular thickness (CMT) and choroidal features including subfoveal choroidal thickness (SFCT), total choroidal area (TCA), luminal choroidal area (LCA), and stromal choroidal area (SCA) were analyzed. Choroidal vascularity index (CVI) was calculated in all study eyes at baseline and at 1- and 3-months post-PDT., Results: In eyes receiving PDT, Snellen visual acuity (VA) significantly improved at months 1 and 3 (P < .001). CMT and SFCT showed a significant reduction from baseline at months 1 and 3 (P < .001), whereas TCA and LCA showed a significant decrease only at the 1-month follow-up visit. Baseline mean TCA and LCA were 2.30 ± 1.41 mm 2 and 1.23 ± 0.73 mm 2 , respectively, and decreased to 2.07 ± 1.21 mm 2 and 1.08 ± 0.63 mm 2 at the 1-month follow-up visit, respectively (P = .01). No significant changes were recorded for SCA and CVI. In the fellow eye group, VA, CMT, and all choroidal parameters showed no differences between baseline and any follow-up visits (all P > .05)., Conclusions: After PDT for chronic CSCR we observed sustained reductions in CMT and SFCT, while reductions in TCA and LCA were only noted at the 1-month follow-up interval. These choroidal parameters may provide additional quantitative biomarkers to evaluate the anatomic response to therapy but await further prospective validation., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: To compare the vascular lesion size using optical coherence tomography angiography and indocyanine green angiography in eyes with polypoidal choroidal vasculopathy., Methods: Treatment-naïve cases (46 eyes of 44 patients) with polypoidal choroidal vasculopathy were retrospectively analyzed. The comparison of mean area of branching vascular network and polyp detection rate was done between indocyanine green angiography and optical coherence tomography angiography and correlated with various optical coherence tomography features., Results: The mean age of the study patients was 62.33 ± 10.74 years. The mean branching vascular network size was 7.47 ± 5.74 and 7.51 ± 5.69 mm² in indocyanine green angiography and optical coherence tomography angiography, respectively, with an excellent correlation (r = 0.997). Optical coherence tomography angiography overestimated (mean ± SD: 0.28 ± 0.19 mm²) and underestimated branching vascular network area (0.36 ± 0.33 mm²) in 23 eyes each as compared to indocyanine green angiography. However, the difference in branching vascular network size was not statistically significant (p = 0.53). Indocyanine green angiography and optical coherence tomography angiography could identify polyps in 43 of 46 (93.48%) and 32 of 46 (69.57%) patients, respectively., Conclusion: Branching vascular network size measurements with indocyanine green angiography and optical coherence tomography angiography were comparable and showed significant correlation, albeit the polyp identification rate was lower with optical coherence tomography angiography. Optical coherence tomography angiography may serve as a useful substitute to indocyanine green angiography in measurements of branching vascular network for photodynamic therapy and follow-up of polypoidal choroidal vasculopathy eyes.

Competing Interests: None

Background: To report retinal pigment epithelium (RPE) apertures as a possible evolution of serous pigment epithelium detachment (PED) in patients with chronic central serous chorioretinopathy (CSC) and to analyze their progression over time., Design: Retrospective case series., Methods: Fifteen patients (17 eyes) with a diagnosis of avascular PED in chronic CSC were retrospectively evaluated based on multimodal imaging. All patients had documented records of clinical examination, best correct visual acuity (BCVA), fundus autofluorescence (FAF), fluorescein and indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography (OCT-A). Primary outcomes measures were RPE aperture imaging analysis along with their temporal course., Results: All eyes showed the RPE aperture as an interruption of the RPE in correspondence of the PED with neither sign of rippling nor retraction. Mean age was 59.4 ± 8.1 years and mean BCVA was 0.34 ± 0.24 logMAR. In all eyes, FAF displayed a well-circumscribed roundish hypoautofluorescence. Multimodal imaging and OCT-A confirmed the absence of any vascular network beneath the aperture in all study eyes. The mean time of follow-up was 19.3 ± 14.3 months, and RPE discontinuities showed an increase in size over time from 0.81 ± 0.39 mm 2 to 0.95 ± 0.45 (P = 0.005)., Conclusions: RPE aperture is a new finding in the setting of chronic CSC and it should be distinguished by RPE tears for the different pathogenesis and evolution in time.

Purpose: To analyze and compare the clinical profile of herpes zoster ophthalmicus (HZO) patients in a South Indian patient population aged <60 years and ≥60 years and determine the risk factors for recurrence of ocular inflammation., Methods: Retrospective study of 249 cases between 2006 and 2016 from two tertiary referral eye centres in south india., Results: Out of 249 cases, 189 cases were <60 years (Group 1) and 60 cases were aged ≥60 years (Group2). Presence of diabetes mellitus, increased intraocular pressure(IOP) at the time of active inflammation, use of topical steroids and recurrences were significantly more common in group 1. Significant risk factors for recurrences included corneal, uveal, scleral involvements and increase in IOP. Good vision at presentation was noted in 67.9% of the patients., Conclusions: Anterior uveitis with or without keratitis was the most common presentation observed in more than 50% cases. The overall visual outcome was good.

Purpose: To report an interesting case of development of tonic pupil in an immunocompetent male with cytomegalovirus (CMV) anterior uveitis., Methods: Retrospective case report Results: A 30-year-old Iranian healthy male was diagnosed to have Posner-Schlossman syndrome (OS). Aqueous tap was positive for CMV by multiplex Polymerase chain reaction (PCR). Adequate control of inflammation and Intraocular pressures (IOP) were achieved with topical steroids, ganciclovir gel, and antiglaucoma medications. On a follow-up visit, he complained of recent onset of blurring of vision (OS) despite appropriate refractive correction, quiet anterior chamber, and normal IOP. Pupillary examination showed anisocoria (larger pupil in OS). Pupillary constriction (OS) on instillation of 0.125% pilocarpine drops confirmed the diagnosis of a tonic pupil., Conclusion: We describe a unique finding of tonic pupil on a follow-up examination in an immunocompetent adult male with CMV anterior uveitis. A prior association has not been described in literature (Medline Search).

Purpose: We report an interesting case of atypical Tolosa Hunt syndrome with bilateral Sclerokeratitis and optic atrophy in a patient with Takayasu's arteritis., Observations: A 31-year- old lady presented with severe retroorbital pain in the right eye and right sided headache of 2 weeks duration. She had a history of 6th and 7th nerve palsies which improved with oral corticosteroids. Her BCVA was no perception of light in OD and 6/6 in OS. Slitlamp examination showed bilateral old sclerokeratitis and fundus examination showed bilateral optic atrophy. Systemic examination revealed BP of 240/100 mm of hg in the right upper limb with absent pulsations in the left radial, bilateral femoral and dorsalis pedis arteries noted. CT aortic angiography confirmed the diagnosis of Takayasu's arteritis. Symptoms improved with oral corticosteroids and azathioprine, which was followed by a renal stenting procedure. At 1 year followup, she is doing well with no recurrences., Conclusions and Importance: This case report presents a unique manifestation of atypical Tolosa Hunt syndrome in Takayasu's arteritis. To our knowledge, atypical Tolosa Hunt syndrome in Takayasu'arteritis has not been described in literature.