Whole-genome sequencing unravels novel genetic determinants and regulatory pathways associated with triamcinolone acetonide-induced ocular hypertension.

Glucocorticosteroids commonly used to treat certain ocular inflammatory conditions cause an unwarranted elevation in intraocular pressure (IOP) leading to steroid-induced ocular hypertension (OHT). This study aims to identify novel genetic variants in the Indian population associated with steroid responsiveness, specifically to that of intravitreal Triamcinolone acetonide (TA) injections, which leads to OHT in 27% of the TA-treated Indian subjects. Genetic determinants and pathways regulating TA-OHT progression were investigated by applying whole-genome sequencing (WGS) on DNA extracted from 53 blood samples that included TA responders and non-responders. Sequencing analysis yielded 45 intronic and 49 exonic variants to be associated with TA-OHT, which are known to play a vital role in eye, heart, brain, and bone deformities. Of these, the most significant genetic variant associated with TA-OHT was further considered for molecular dynamics (MD) simulation studies. Variants in the CRPPA, PLOD1, ARHGAP1, TIMELESS and TNFSF4 genes were found to be directly implicating TA-OHT. Furthermore, these genes were enriched in pathways associated with cardiomyopathy, focal adhesion, extracellular matrix, and actin cytoskeleton reorganization. MD simulation studies revealed that the top significant variant (rs141625803) in the CRPPA gene possesses a high pathogenic and structurally destabilizing effect. Thus, novel genetic variants that could be significantly associated with the TA-OHT progression were identified in this study. Validation of these targets in a larger cohort of patients along with their functional analysis would inform on the disease, thereby adding to the existing knowledge on the pathophysiology of TA-OHT.

Incidence, Risk Factors, Treatment, and Outcome of Ocular Hypertension following Intravitreal Steroid Injections: A Comparative Study.

PURPOSE: To compare the incidence, risk factors, treatment, and outcomes associated with intravitreal triamcinolone-acetonide (TA) and dexamethasone-implant (Dex)-induced ocular hypertension (OHT). METHODS: This retrospective study reviewed 1,549 TA and Dex administrations in 1,075 eyes of 897 patients. Intraocular pressure (IOP) values were monitored for a period of 6 months following intravitreal injection(s) and patients were categorized as steroid-responders (SR): IOP≥21 mm Hg, and steroid non-responders (NR): IOP≤20 mm Hg. Glaucoma patients, glaucoma suspects, uveitis, trauma, and less than 1 month of IOP follow-up cases were excluded from the study. The incidence of IOP rise, time and magnitude of IOP rise, and its management procedures were studied. Ocular and systemic associations with OHT incidence were investigated. Statistical analysis was performed using SPSS.23 and p < 0.05 was considered significant. RESULT: Twenty-eight percent of TA and 17% of Dex administered eyes developed OHT. Male subjects and elderly people (greater than 40 years of age) are at higher risk for OHT following steroid treatment. A high percentage of IOP rise was observed on day 1 (41%) for TA-SR, and after 1 month (50%) for Dex-SR. IOP rise was found to be more severe (>30 mm Hg) for TA-SR compared to Dex-SR (p = 0.006). 6% TA-SR required trabeculectomy with medically uncontrollable IOP. Myopia is a risk factor for secondary OHT, whereas diabetes mellitus and hypercholesterolemia are protective of it. CONCLUSION: Twenty-eight percent of TA and 17% of Dex administrations developed OHT. Early and severe IOP rises were more common in TA than among Dex administrations. Myopia is a risk for Dex-OHT.

Choroidal neovascularization in 111 eyes of children and adolescents.

PURPOSE: To describe the clinical features, management, and outcomes of choroidal neovascularization (CNV) in children less than 18 years of age. METHODS: This was a retrospective, case control study of 111 eyes of 96 patients. CNV was clinically diagnosed in all patients. Eyes were classified as those that were observed (Controls; Group 1) or those that had treatment (Cases; Group 2). CNV was categorized as regressed, persistent, or recurrent in order to evaluate the anatomical outcomes. RESULTS: Of 96 patients, 68(71%) were male. Mean presenting age was 11.4 ± 3.4 years (median = 11 years, range = 1-17 years). CNV was bilateral in 15(16%) patients. Of 111 eyes, 38 eyes had treatment (Cases) and 73 eyes did not (Natural history group or Controls). Subfoveal CNV was seen in majority of cases (59%). Most common etiology was post-inflammatory (38%), followed by trauma (16%). Eyes were classified as those that were observed (controls; Group 1) or those that had treatment (cases; Group 2). In group 1, spontaneous regression of CNV was seen in 26(36%) eyes and there was no recurrence in this group. In group 2 following treatment, 25(66%) of 38 eyes achieved complete regression at mean 4.9 months and was persistent in 5 eyes. CNV recurrence was seen in 10 eyes with the mean time to first recurrence being 9 months. At presentation, mean best-corrected visual acuity (BCVA) of eyes with subfoveal CNV was logMAR 0.99 that improved to logMAR 0.63 with treatment. Mean follow-up was 17 months. CONCLUSION: CNV results in significant visual decline in children; most commonly of post-inflammatory etiology. Treatment achieves high regression rates, albeit with limited visual improvement.

A prospective, interventional study comparing the outcomes of macular hole surgery in eyes randomized to C3F8, C2F6, or SF6 gas tamponade.

PURPOSE: Analysis of outcomes of macular hole (MH) surgery using 12% C3F8, 16% C2F6, or 18% SF6 as randomized gas tamponading agent. METHODS: This is a prospective, randomized, interventional study of 159 eyes with idiopathic MH undergoing 23/25G pars-plana vitrectomy with internal limiting membrane peeling and gas tamponade. Eyes were stratified into two groups: Group I (MH < 800 µ) and group II (MH > 800 µ) according to MH apical diameter. Eyes in group I were randomized to receive either 18% SF6, 16% C2F6, or 12% C3F8. Eyes in group II were randomized to either 16% C2F6 or 12% C3F8. Clinical details, MH parameters on optical coherence tomography, surgical details and complications were analyzed. Outcome measures were type of hole closure (I/II), best corrected visual acuity, intraocular pressure, and cataract progression. RESULTS: In Group I (n = 139), type 1 closure was achieved in 107 (77%) eyes. Type 1 closure rates in group I per gas tamponade were as follows: SF6 (70%), C2F6 (80%), C3F8 (78%) (p = 0.503, chi-square test for independence). There was no statistical difference in MH closure rates between SF6 and C2F6 (p = 0.134), SF6 and C3F8 (p = 0.186), and C2F6 and C3F8 (p = 0.373). In Group II (n = 20), type 1 closure was achieved in 12 (60%) eyes. Type 1 closure rates in group II per gas tamponade were as follows: C2F6 (75%), C3F8 (50%) (p = 0.132, Two proportion Z test). Mean follow-up after surgery was 2.66 ± 2.74 months. CONCLUSION: Given the similar outcomes of using 18% SF6, 16% C2F6, or 12% C3F8 in idiopathic macular hole surgery, the advantage of using a shorter acting tamponade translates into earlier recovery and rehabilitation.

ANATOMICAL AND VISUAL OUTCOMES IN STAGE 5 RETINOPATHY OF PREMATURITY WITH MICROINCISION VITRECTOMY SURGERY.

PURPOSE: To report anatomical and visual outcomes and potential prognostic factors with microincision vitrectomy surgery in Stage 5 retinopathy of prematurity. METHODS: The medical records of premature babies who underwent microincision vitrectomy surgery for Stage 5 retinopathy of prematurity using 23G, 25G, or 27G instrumentation and had a minimum follow-up of 6 weeks were, retrospectively, analyzed. Primary outcome measures were anatomical success at last follow-up defined as retinal attachment at the posterior pole and visual outcomes. Potential risk factors and complications influencing anatomical outcomes were also analyzed. RESULTS: One hundred seventy eyes of 115 infants underwent lensectomy and vitrectomy with microincision vitrectomy surgery. After a mean follow-up of 30.59 ± 33.24 weeks, anatomical success was achieved in 56 eyes (33.7%) of 166 eyes that had a minimum follow-up of 6 weeks. Occurrence of vitreous hemorrhage was more with 23 gauge (62.27%) as compared to 25 gauge (37.73%) (P = 0.024). With increase in age with each week, the probability of achieving anatomical success was found to be significantly more (odds ratio 1.030; confidence interval = 1.010-1.060; P = 0.008). Presence of anterior segment pathology was associated with poor anatomical outcomes (odds ratio 2.480; confidence interval = 1.190-5.160; P = 0.010). Seventeen children with attached retina had a follow-up of 14 months-5 years, of which ambulatory vision was recorded in five eyes and the ability to identify objects close to face in 12 eyes. CONCLUSION: Although surgery for Stage 5 retinopathy of prematurity is challenging, anatomical success can be seen in one-third of cases with microincision vitrectomy surgery. Visual prognosis may be limited but still beneficial.

Multimodal imaging characteristics in eyes with vitreoretinal lymphoma treated with intravitreal rituximab.

PURPOSE: To characterize the imaging features in eyes with vitreoretinal lymphoma (VRL) using ultra-widefield fundus photography (UWF-FP), swept-source optical coherence tomography (SSOCT) and fundus autofluorescence (FAF) that are correlated to ongoing treatment with intravitreal Rituximab(IVR). METHODS: Retrospective observational imaging-based study of 15 treatment-naive eyes with VRL treated with IVR. All patients with primary VRL underwent vitreous biopsy using 23/25G microincision vitrectomy system for confirmation of diagnosis. All eyes received monthly IVR (1 mg/0.1 mL) injections till disease remission. Baseline clinical characteristics, treatment details, outcomes, and sequential imaging features on UWF-FP, FAF, and SSOCT were analyzed. OUTCOME MEASURES: Baseline features and changes in UWF-FP, FAF patterns, and SSOCT features in response to treatment RESULTS: Clinically, patients presented with sub-RPE deposits (n = 15), superficial retinal hemorrhages (n = 2), 'giant' RPE (retinal pigment epithelium) holes (n = 2), and anterior segment reaction (n = 1). Eyes were treated with mean 5.7 IVR injections (median: 5; range 1-13) over a mean 7.2 ± 4.9 months. During the course of treatment, two eyes developed superficial retinal hemorrhages with spontaneous resolution, 2 eyes developed CME, and 4 eyes developed characteristic 'leopard skin' pigmentation. Hyper-autofluorescence corresponding to areas of active lesions decreased with each treatment cycle and was finally replaced by hypo-autofluorescence. Serial OCTs showed regression of sub-RPE/subretinal deposits (n = 15), ellipsoid zone disruption (n = 9), and its resolution with treatment (n = 3), epiretinal membrane (ERM; n = 6), choroidal hyperreflective foci (HRF; n = 4), disorganization of retinal inner layers (DRIL; n = 3), RPE-rip (n = 2), cystoid macular edema (CME; n = 2), and hyperreflective lesions in the choroid (n = 1). Complete resolution was observed in all eyes with extensive hypo-AF. The central foveal thickness decreased from 237 ± 113 µ to 182 ± 114 µ (p = 0.1) and subfoveal choroidal thickness decreased from 258 ± 66 µ to 220 ± 64 µ (p = 0.12) at final follow-up. The mean baseline BCVA was logMAR 0.9 ± 0.9 that deteriorated to mean logMAR 1 ± 1 final visit (p = 0.7). The mean recurrence-free follow-up was 5.9 ± 5.1 months CONCLUSION: Multimodal imaging provides novel insights into features of VRL, a better understanding of regression patterns, and prognostication of outcomes when treated with intravitreal rituximab. Larger, multicentric studies with longer follow-up will help unravel imaging biomarkers to understand these aspects better.

Intravitreal rituximab monotherapy for management of eyes with vitreoretinal lymphoma: initial experience from India.

PURPOSE: To evaluate treatment outcomes and complications of intravitreal rituximab (IVR) monotherapy for eyes with vitreoretinal lymphoma (VRL). METHODS: Patients diagnosed with 'isolated primary VRL' or 'VRL with remission of systemic disease' and treated with IVR (1 mg/0.1 ml) between June 2014 and June 2019 were included in this retrospective, interventional case series. Injections were repeated at monthly intervals until complete resolution. All patients signed a written informed consent form. Institutional review board approval was obtained. RESULTS: Twelve eyes of 7 patients with VRL were treated with 77 IVR injections at mean 6.42 injections per eye (median = 5; range = 2-13) for complete resolution at mean 8.16 ± 4.62 months (median = 6.97 months; range = 1.97-14.33 months). Mean age at presentation was 53.3 years (median = 54 years; range = 34-74 years). Patients were co-managed with medical oncologist and periodically evaluated. Complications included anterior uveitis (n = 6), raised intraocular pressure (n = 3), posterior synechiae (n = 2), vitreous haemorrhage (n = 1), pre-retinal haemorrhage (n = 1), retinal detachment (n = 1), posterior subcapsular cataract (n = 2) and sectoral iris atrophy (n = 1). Recurrences were seen in 3 eyes (25%), which eventually achieved complete resolution with treatment. None of the patients had systemic involvement or death during follow-up. Mean follow-up was 18.73 ± 8.83 months (median = 21.60 months; range = 7.37-32.67 months). CONCLUSION: Intravitreal rituximab monotherapy is effective in management of vitreoretinal lymphoma in patients with isolated ocular disease.

Outcome of sutured scleral fixated intraocular lens in Marfan syndrome in pediatric eyes.

PURPOSE: To report the clinical outcomes and complications in eyes with Marfan syndrome with subluxated/dislocated lens undergoing sutured scleral fixated intraocular lens (SSFIOL) implantation. METHODS: A retrospective review was done for a consecutive series of 73 eyes of 43 patients with Marfan syndrome, with age less than 18 years who underwent SSFIOL from July 2000 to June 2017. Postoperative best corrected visual acuity, intraocular pressure and refractive error, intraoperative and postoperative complications and time for the development of these complications were analyzed. RESULTS: Visual acuity improved significantly to 0.43 ± 0.30 (p = 0.01), 0.40 ± 0.3 (p = 0.00) and 0.39 ± 0.3 (p = 0.00) logMAR units at 6 weeks, 1 year and at last follow-up, respectively. The refractive error reduced significantly (p = 0.056, 0.039 and 0.031) at 6 weeks, 1 year and final follow-up following surgery. Intraoperative complications included iatrogenic retinal break (n = 3) and surgical trauma to the iris (n = 1). Postoperative complications included increased intraocular pressure in 3 eyes (4.1%), intraocular lens dislocation/subluxation in 5 eyes (6.8%), retinal detachment in 3 eyes (4.1%), choroidal detachment in 1 eye (1.3%), pupillary capture in 15 eyes (20.5%), vitreous hemorrhage in 1 eye (1.3%), pre-retinal hemorrhage in 1 eye (1.3%) and hyphema in 1 eye (1.3%). CONCLUSION: SSFIOL provides good visual outcomes in eyes with ectopia lentis associated with Marfan syndrome. Complications can be seen in some cases, and the parents need to be counseled regarding the same before surgical intervention.

Vitreoretinal Complications and Outcomes in 92 Eyes Undergoing Surgery for Modified Osteo-Odonto-Keratoprosthesis: A 10-Year Review.

PURPOSE: To analyze vitreoretinal (VR) complications and treatment outcomes in eyes undergoing modified osteo-odonto-keratoprosthesis (OOKP) surgery. DESIGN: Retrospective case series. PARTICIPANTS: All patients who underwent modified OOKP (mOOKP) surgery at a tertiary eye-care center from March 2003 to February 2013 were included. METHODS: Medical records were reviewed for relevant medical history, best-corrected visual acuity (BCVA), slit-lamp examination, ultrasound scan, oral examination findings, and VR complications. MAIN OUTCOME MEASURES: The BCVA at the last visit. Optimal anatomic outcome was attached retina with a normal intraocular pressure at the last visit. RESULTS: A total of 92 eyes of 90 patients were included. Indications for OOKP included Stevens-Johnson syndrome (n = 53), chemical injury (n = 36), and ocular cicatricial pemphigoid (n = 3). A total of 41 eyes of 39 patients developed VR complications, including vitritis (n = 21), retinal detachment (RD) (n = 12; primary RD = 5), retroprosthetic membrane (RPM) (n = 10; primary RPM = 2), endophthalmitis (n = 8), vitreous hemorrhage (VH) (n = 5; primary VH = 1), serous choroidal detachment (n = 5), hemorrhagic choroidal detachment (n = 2), and leak-related hypotony (n = 1). Mean interval from mOOKP surgery to occurrence of VR complication(s) was 43.8 months (median, 41.9 months; range, 0.2-95.5 months). After treatment of VR complication, visual improvement was seen in 17 eyes (42%) (mean improvement = 1.2 logarithm of the minimum angle of resolution [logMAR]; median, 0.8 logMAR; range, 0.1-2.5 logMAR), visual decline in 7 eyes (14%) (mean decline in BCVA = 0.6 logMAR; median, 0.4 logMAR; range, 0.3-1.8 logMAR), and no change in BCVA in 17 eyes (42%). However, BCVA ≥6/60 was retained in 19 eyes and ≥6/18 was retained in 9 eyes after final VR treatment. CONCLUSIONS: Vitreoretinal complications constitute a significant cause of visual morbidity in eyes undergoing mOOKP surgery and pose a challenging situation to manage. However, appropriate and timely intervention can achieve encouraging results.

VITREORETINAL COMPLICATIONS IN EYES WITH BOSTON KERATOPROSTHESIS TYPE I.

PURPOSE: To describe the spectrum of vitreoretinal complications in eyes with Boston keratoprosthesis type I and evaluate the treatment outcomes. METHODS: This was a retrospective interventional case series of 23 of 45 eyes that underwent Boston keratoprosthesis from April 2003 to December 2013 and developed vitreoretinal complications. Types of vitreoretinal complications, surgical techniques, and anatomical and visual outcomes were analyzed. RESULTS: Vitreoretinal complications in eyes with Boston keratoprosthesis included retroprosthetic membranes (n = 11), retinal detachment (n = 6), endophthalmitis (n = 4), epiretinal membrane (n = 4), vitreous hemorrhage (n = 2), and choroidal detachment (n = 1). Twenty of 23 eyes (87%) underwent surgical intervention. Retinal reattachment was achieved in 5 eyes (83%) with significant visual improvement in 3 (50%). Mean preoperative visual acuity improved from 1.84 ± 0.89 logMAR to 1.5 ± 0.87 logMAR (P = 0.01) at the last follow-up. Mean 1.4 surgical procedures were performed per eye. Mean follow-up was 28 months (median: 28 months, range: 5-57 months). CONCLUSION: Retroprosthetic membrane and retinal detachment are most common vitreoretinal complications in eyes with Boston keratoprosthesis. Vitreoretinal complications can be managed by appropriate intervention in such eyes with encouraging anatomical and functional results.

Bone augmentation of the osteo-odonto alveolar lamina in MOOKP--will it delay laminar resorption?

PURPOSE: We aimed to describe a new technique and analyse the early outcomes of augmenting the canine tooth using a mandibular bone graft in an attempt to delay or retard the process of laminar resorption following the modified osteo odonto keratoprosthesis (MOOKP) procedure. DESIGN: This was a retrospective case series. PATIENTS AND METHODS: Eyes that underwent the bone augmentation procedure between December 2012 and February 2014 were retrospectively analysed. The procedure, performed by the oromaxillofacial surgeon, involved securing a mandibular bone graft beneath the periosteum on the labial aspect of the canine tooth chosen to be harvested for the MOOKP procedure. This procedure was performed simultaneously with the Stage 1 A of the MOOKP. Three months later, the tooth was harvested and fashioned into the osteo-odonto alveolar lamina similar to the method described in the Rome-Vienna Protocol. RESULTS: The bone augmentation procedure was performed in 11 eyes (five SJS/ six chemical injuries). The mean follow-up after Stage 2 of MOOKP procedure in these eyes was 7.45 months (2 to 20 months). Complications noted were peripheral laminar exposure (three eyes-SJS) and bone graft exposure and necrosis in the mouth (nine-SJS). No evidence of clinical laminar resorption was noted in any of the eyes. CONCLUSION: Laminar resorption in MOOKP can lead to vision and globe threatening complications due to the consequent cylinder instability and chances of extrusion. Augmenting the bone on the labial aspect of the canine tooth might have a role to play in delaying or preventing laminar resorption.

Phacoemulsification in patients with diabetic macular edema - A real-world data of 75 eyes.

PURPOSE: The purpose of this study was to evaluate the outcomes of phacoemulsification with intravitreal triamcinolone acetonide (IVTA) injection in patients with cataract and diabetic macular edema (DME) across the grades of diabetic retinopathy (DR). MATERIALS AND METHODS: It was a retrospective observational study. The study included 70 patients (75 eyes) with cataract and clinically significant macular edema in diabetics with nonproliferative (61 eyes) or proliferative (14 eyes) DR who underwent phacoemulsification with IVTA. Visual outcome and progression of DME over 3 months were studied in terms of the best-corrected visual acuity (BCVA) and central macular thickness (CMT), respectively. RESULTS: The case records of 70 patients (75 eyes) were studied. The mean baseline BCVA (n = 75) in the logMAR scale ± SD was 0.92 ± 0.53. 3 weeks after the surgery, the mean BCVA improved to 0.39 ± 0.26 and the mean CMT (n = 40) was 382.57 ± 192.30 compared with the mean preoperative CMT of 480.57 ± 163.25 in the same set (P = 0.01). The mean CMT decreased at 3 weeks in 22 (55% of the eyes). An improvement of ≥2 lines was seen at 3 weeks in 52 (69.33%) eyes and in 49 (65.33%) eyes at 3 months. The mean CMT at 3 months was 445.51 ± 222.99 (n = 70) compared to the preoperative mean CMT of 470.72 ± 176.15 in the same set (P = 0.29). The mean CMT decreased at 3 months in 34 (48.5%) eyes. CONCLUSIONS: Phacoemulsification with IVTA injection improved the mean visual acuity at 3 weeks and 3 months in two-thirds of cases and decreased the mean CMT at 3 weeks in nearly half the numbers.

Posterior segment manifestations of Takayasu arteritis: A narrative review.

Ocular symptoms can be the presenting manifestation of Takayasu arteritis (TA) or could be indicative of disease reactivation. A review of published literature related to posterior segment manifestations of TA by using the keywords "Takayasu arteritis," "ophthalmic manifestations," "retina," "retinopathy," "ocular," "optic nerve," and "optic neuropathy" was performed. In total, 62 case reports and 12 case series were included. The majority of the articles were from Asia (n = 47, 64%). Females outnumbered males in the ratio of 7:1. The mean age of patients was 33 years (range: 8-78 years, SD: 13.5 years). In 58% (n = 41 out of 71) cases, ocular symptoms were the presenting manifestation of the underlying disease. Hypotensive retinopathy was found in 70% of eyes, and hypertensive retinopathy was found in 27%. The mean presenting visual acuity (VA) was +1.03 logMAR (range: -0.12 to 3, SD: 1.07), and at the final follow-up was +1.02 logMAR (range: -0.12 to 3, SD 1.17). VA improved in 34% (n = 29/86), remained stable in 45% (39/86), and worsened in 21% (18/86). The mean follow-up was 9 months (range: 0.5-204, SD: 16 months).

Acute postoperative Bacillus cereus endophthalmitis mimicking toxic anterior segment syndrome.

OBJECTIVE: To study the clinicomicrobiologic characteristics and treatment outcomes in eyes with acute postoperative endophthalmitis (APE) owing to Bacillus cereus from a tertiary eye-care center. DESIGN: Retrospective, interventional case series. PARTICIPANTS: Case records of all eyes with culture-proven APE attributable to B cereus from January 2000 to May 2011 were identified from a computerized database and evaluated. METHODS: Clinical features at time of presentation, microbiological characteristics, and treatment measures were recorded. A thorough literature search using PubMed and the Cochrane Library databases was done to identify all cases of APE owing to Bacillus species reported to date and clinical characteristics of these eyes was compared with our series. MAIN OUTCOME MEASURES: Structural (globe salvage) and functional (visual rehabilitation) outcomes at last follow-up visit. RESULTS: We found 6 sporadic cases that experienced APE during the study period. All eyes had a fulminant onset within the first 24 hours of cataract surgery with extremely high intraocular pressure (IOP) and corneal edema similar to toxic anterior segment syndrome (TASS). However, these eyes progressed rapidly to develop corneal infiltrates, scleral and uveal tissue necrosis with hyphema, brownish exudates in anterior chamber and necrotizing retinitis within hours despite immediate initiation of intravitreal pharmacotherapy and vitrectomy. All eyes demonstrated gram-positive bacilli from the aqueous and B cereus was isolated, which was sensitive to conventional antibiotics except penicillin. Two eyes required therapeutic keratoplasty, combined with a scleral patch graft in 1 eye, 1 eye was eviscerated after 48 hours of onset of symptoms, and 2 eyes experienced phthisical changes within 10 days of onset. CONCLUSIONS: We found that APE owing to B cereus has an onset within 12 to 24 hours of intraocular surgery and simulates TASS in the first few hours. The clinical course is marked by rapidly worsening necrotizing infection, leading to very poor outcomes despite early institution of appropriate therapy. One must closely observe every case of TASS that presents with intense pain and extremely high IOP and rule out APE owing to B cereus with microbiologic testing. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Emerging Pathogenic Viral Infections of the Eye.

Global health security threats and the public health impact resulting from emerging infectious diseases including the ongoing COVID-19 pandemic and recent Ebola virus disease outbreaks continuously emphasize the need for a comprehensive approach to preparedness, management of disease outbreaks, and health sequelae associated with emergent pathogens. A spectrum of associated ophthalmic manifestations, along with the potential persistence of emerging viral pathogens in ocular tissues, highlight the importance of an ophthalmic approach to contributing to efforts in the response to public health emergencies from disease outbreaks. This article summarizes the ophthalmic and systemic findings, epidemiology, and therapeutics for emerging viral pathogens identified by the World Health Organization as high-priority pathogens with epidemic potential.

Lightning injuries of the posterior segment of the eye.

Lightning causes serious injuries and deaths worldwide every year. Ophthalmic injuries due to lightning are due to direct or indirect transmission of electric current, resistance-induced heat, and heat-induced shock wave. PubMed search of articles related to posterior segment injuries caused by lightning using keywords (lightning injury, ophthalmic manifestations, ocular injuries, and posterior segment) was conducted, and 19 case reports in 17 articles including 29 eyes with lightning injury to the posterior segment of the eye from 1984 to 2019 were reviewed. The majority of case reports (n = 10, 53%) were from North America. Eleven patients (58%) were in the age group of 10-30 years. Most patients (n = 10, 53%) had bilateral injury. The macula was the most common site of involvement with retinal pigment epithelial changes (n = 14, 48%) being the most common manifestation. A variety of other retinal, vitreous, and electrophysiological abnormalities have also been reported. We conclude that although lightning injuries are usually mild injuries, with vision remaining, either stable or showing some improvement in the majority of cases, severe visual loss due to optic atrophy and maculopathy may occur in long term.

Hypotrichosis with Juvenile Macular Dystrophy in a Patient with Cadherin 3 (CDH3) Mutation.

INTRODUCTION: Hypotrichosis with juvenile macular dystrophy (HJMD) is an autosomal recessive disease with progressive macular degeneration leading to blindness in the first three decades of life along with hypotrichosis. CASE: We herein report a case of a five year old boy with hypotrichosis with juvenile macular dystrophy diagnosed with multi-modal imaging which was later confirmed by genetic testing by whole genome sequencing. OBSERVATIONS: Fundus examination of both eyes revealed symmetrical hypopigmentation in peripapillary retinal pigment epithelium (RPE) involving posterior pole and surrounded by a mottled hyperpigmented border. Fundus autofluorescence showed central hypo autofluorescence with surrounding hyper autofluorescence corresponding to RPE atrophy and a faint hypo autofluorescence at the junction of normal retina. SD-OCT showed segmental outer retinal and choriocapillaris atrophy temporal to fovea with interdigitation zone and ellipsoid zone loss and RPE irregularities with hyperreflective subretinal deposits at the fovea. Electroretinogram showed normal waves but a slight reduction of b wave amplitude in both eyes. He had sparse scalp-hair. CONCLUSION: Children with reduced vision not falling into a typical macular degeneration should be examined systemically and may just have sparse scalp hair and still have a genetic disease. A regular follow-up should be emphasized in view of progressive nature of the disease.

Analysis of choroidal structure and vascularity indices with image binarization of swept source optical coherence tomography images: A prospective study of 460 eyes.

PURPOSE: To evaluate choroidal vascularity index (CVI) among normal subjects using image binarization of swept source optical coherence tomography (SS-OCT). MATERIALS AND METHODS: Four hundred and sixty eyes of 230 normal participants were included. Total circumscribed choroidal area, luminal area, stromal area (SA), and CVI were derived from SS-OCT scans using open-source software (ImageJ) with the modified Niblack method. Both CVI and subfoveal choroidal thickness (SFCT) were correlated with age, refractive error, intraocular pressure, and mean ocular perfusion pressure (MOPP) using mixed linear model analysis. Pearson's correlation coefficient was used to determine the relationship between age and each dependent factor. Analyses were performed using the SPSS software version 20.0 (IBM Corp., Armonk USA) and statistical significance was tested at 5%. RESULTS: The mean age was 42.1 (±17.6) years. Mean SFCT was 307 ± 79 µm. Mean CVI was 66.80 (±3.8)%. There was statistically significant positive correlation between CVI and increasing age (r = 0.259, P < 0.0001) and statistically significant negative correlation between SFCT and age (r = -0.361, P < 0.0001). There was positive linear correlation between refractive error and CVI (r = 0.220, P < 0.0001) and negative correlation between SFCT and refractive error. There was no significant effect of MOPP on both CVI (P = 0.07) and SFCT (P = 0.7). CONCLUSION: CVI and SFCT are significantly correlated with age and refractive error in normal Indian eyes.

Diagnostic Value of Optical Coherence Tomography in the Early Diagnosis of Macular Complications in Chronic Vogt-Koyanagi-Harada Disease.

PURPOSE: To describe the role of optical coherence tomography (OCT) in detecting subclinical choroidal inflammation and early diagnosis of macular complications in chronic Vogt-Koyanagi-Harada (VKH) disease with sunset glow fundus. METHODS: Retrospective observational study. Clinical features, OCT images, treatment, and visual outcome were analyzed. RESULTS: Fourteen patients (9 females and 5 males) were included in the study. Mean age was 39 years (range 7-67 years). Mean duration of disease was 5.25 years (range 1-15 years). Anterior uveitis was seen in 14 eyes (52%). Fundus examination showed sunset glow fundus in all patients with no obvious macular pathology. OCT showed macular edema in 16 eyes (59%), choroidal neovascular membrane in 8 eyes (30%), and macular hole in 3 eyes (11%). CONCLUSION: OCT should be regularly used to detect subclinical inflammation and early macular complications in chronic VKH disease where sunset glow fundus may delay the clinical diagnosis thus causing permanent damage.