Clinical and visual electrophysiological characteristics of vitelliform macular dystrophies in the first decade of life.

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.

Sympathetic ophthalmia: A comprehensive update.

Sympathetic ophthalmia is a rare, bilateral, granulomatous, panuveitis following penetrating trauma or surgery to one eye. Clinical presentation commonly occurs within the first year of trauma occurrence but can be delayed by several years. It manifests as acute/chronic granulomatous uveitis with yellowish-white choroidal lesions or Dalen-Fuchs nodules. Initially, patients respond rapidly to corticosteroid therapy, but a majority require long-term use of corticosteroid-sparing agents to prevent recurrences. The purpose of this review is to elaborate on the current understanding of the pathophysiology, the importance of multimodal imaging in early diagnosis, and the role of newer immunomodulatory and biological agents in recalcitrant cases.

Vogt Koyanagi Harada Disease In Paediatric Age Group: Clinical Characteristics, Remission, Recurrences and Complications in Asian Indian Population.

PURPOSE: To describe disease characteristics and outcomes of Vogt-Koyanagi-Harada (VKH) syndrome in paediatric patients. STUDY DESIGN: Retrospective chart analysis. METHODS: A RETROSPECTIVE: Analysis of all patients ≤16 years with VKH syndrome was done. Clinical presentations, complications, recurrences and outcomes in cases of paediatric VKH were reviewed. RESULTS: 72 eyes of 36 patients with a mean age at presentation of 13.7 ± 2.34 years were assessed. Mean duration of symptoms and follow up were 9.88 ± 17.3 weeks and 55 months respectively. Clinical signs at presentation included anterior chamber cells >2+(34/72eyes, 47.2%), granulomatous keratic precipitates (6 eyes, 8.3%), posterior synechiae (35 eyes,48.6%), disc edema (46 eyes, 63.8%), neurosensory retinal detachments (44 eyes, 61.1%) and 'sunset-glow' fundus (9 eyes, 12.5%). Best corrected visual acuity (BCVA) at the time of presentation was 1.3logMAR or a Snellens equivalent of 20/400 which improved to 0.51logMAR (Snellens equivalent of 20/63) at last follow up. Remission was achieved in 61.1% cases. More than half of our patients developed one or more complications. CONCLUSION: VKH in paediatric patients poses a challenge due to a delayed presentation and paediatric VKH patients have a worse visual acuity at the time of presentation as compared to adult age groups. Rates of remission may be low along with high risk of complications and hence there is a need for prolonged immunosuppression.

Toxic Posterior Segment Syndrome Presenting as Occlusive Retinal Vasculitis following Vitreoretinal Surgery.

PURPOSE: To report a rare retinal manifestation of toxic posterior segment syndrome following vitreoretinal surgery. METHODS: In this case series, we report three cases of rhegmatogenous retinal detachment for which pars plana vitrectomy with silicone oil injection was done. All three patients developed an intense anterior chamber reaction along with occlusive vasculitis-like fundus picture. RESULTS: The three patients were started on topical and systemic steroids, and there was a dramatic improvement in vision and clinical signs at postoperative week 1. CONCLUSION: Toxic posterior segment syndrome is a sight-threatening complication after vitreoretinal surgery, but responds well to topical and systemic steroids.

Sight-threatening intraocular infection in patients with COVID-19 in India.

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

Cytomegalovirus retinitis in patients of non-Hodgkin's lymphoma: clinical presentations and outcomes.

BACKGROUND: Cytomegalovirus (CMV) retinitis in patients with Non-Hodgkin's Lymphoma (NHL) can occur even in the presence of high CD 4 counts and can behave differently when compared to CMV retinitis in human immunodeficiency (HIV) patients. It, therefore, becomes important to understand its varied presentations and the challenges in management of these cases. The aim of this study was to analyse the various patterns of presentations and outcomes of CMV Retinitis in patients with NHL. STUDY DESIGN: A retrospective chart review of seven eyes of four patients of NHL presenting with CMV retinitis between June 2017 and May 2020 was done. METHODS: Clinical patterns of CMV Retinitis, CD4 counts at the time of presentation and the duration of treatment along with recurrences and time for recurrence of retinitis were assessed. RESULTS: Granular or indolent retinitis (6 out of 7 eyes) was the commonest form of CMV retinitis in patients of NHL. Three patients had a presenting CD4 count above 150 cells/mm3 and none of them were below 50 cells/mm3. Floaters were the commonest presenting complaint. All patients had vitritis and majority of the patients (3 out of 4) had anterior chamber (AC) inflammation. Two out of the 4 patients had a recurrence (mean time 33.8 days) after stopping the maintenance phase of ganciclovir and one patient had significant myelosuppression related to oral valganciclovir which required discontinuation of the drug. CONCLUSION: CMV retinitis in NHL patients is usually of an indolent or granular type and can occur even in the presence of high CD4 counts as compared to patients with HIV. These patients may require a long term maintenance in view of frequent recurrences after discontinuation of treatment.

Topiramate induced bilateral hypopyon uveitis and choroidal detachment: a report of two cases and review of literature.

BACKGROUND: Topiramate (TPM) is a drug commonly used by neurophysicians and psychiatrists for a plethora of indications. Topiramate has been reported to induce acute angle closure glaucoma as an adverse effect. However, there is limited literature on Topiramate causing hypopyon uveitis and intense ocular inflammation. It is imperative for ophthalmologists as well as physicians to be aware of the potential sight threatening ocular adverse effects of Topiramate. We report 2 rare consecutive cases of severe hypopyon uveitis and choroidal detachments after using Topiramate. CASE PRESENTATION: Two patients presented with sudden onset of angle closure, bilateral hypopyon uveitis and choroidal detachments. On reassessing a detailed treatment history, it was found that both patient were taking oral Topiramate which had been started 2 weeks before the onset of ocular symptoms. The bilateral hypopyon and angle closure were considered to be induced by Topiramate and the drug was discontinued. The patients were started on oral and topical steroids which led to resolution of hypopyon uveitis and choroidal detachments. The visual acuity improved and the intraocular pressure also got normalised in both the cases. CONCLUSIONS: Topiramate can lead to a bilateral hypopyon uveitis and severe ocular inflammation. An urgent cessation of topiramate along with topical and systemic steroids is required to prevent serious complications.

Cytomegalovirus retinitis in a patient of granulomatosis with polyangiitis on long-term immunosuppressants.

A 69-year-old male patient presented to the retina clinic with a sudden decrease in vision in his right eye since 1 day. He was a known case of granulomatosis with polyangiitis and was on systemic immunosuppression for the past 3 years. The best-corrected visual acuity (BCVA) in his right eye was 20/60 and he has no perception of light in the left eye. Fundus examination revealed the presence of retinitis lesions in the right eye and total optic atrophy in the left eye. A vitreous biopsy was done and the PCR was found to be positive for cytomegalovirus (CMV). He was treated with intravitreal ganciclovir injections. Subsequently, the retinitis lesions regressed and BCVA in the right eye improved to 20/40.This case report elaborates on the risks of the development of opportunistic ocular infections in patients receiving long-term systemic immunosuppressants and the need for regular ocular examinations in such cases.

Virus containment box for retinopathy of prematurity screening and laser.

PURPOSE: Health care workers are susceptible to contracting infection with COVID-19 by aerosol transmission. This is a risk while examining and/or treating an un-sedated neonate in retinopathy of prematurity (ROP) screening and treatment. But screening for neonates for ROP and treating with laser, when required, should not be delayed to avoid the blindness. We describe a cost-effective method of containing aerosols generated during such a procedure in an un-sedated baby. METHODS: An acrylic transparent containment box was prepared to accommodate an average-sized infant. The box had four walls and a roof. The floor was open to place the container box over the baby. The walls have two types of openings, large ones to allow passage of hands to examine the baby, small ones to enable passage of oxygen tubing when required. A simulation was created to examine the impact of aerosol spray on examining healthcare personnel. RESULTS: The cost of the acrylic box was negligible. It could be assembled locally with available acrylic sheets and craftsmen. It was not difficult to examine the baby inside the box, and the simulation demonstrated that it protected the health personnel from the aerosol contamination. CONCLUSION: The described method is likely to increase healthcare personnel's confidence not to delay or deny ROP screening and laser treatment and save the babies from blindness.

Clinical manifestations and outcomes of ocular syphilis in Asian Indian population: Analysis of cases presenting to a tertiary referral center.

PURPOSE: To describe disease manifestations and outcomes of ocular syphilis in Asian Indian population. METHODS: Retrospective analysis of patients diagnosed with ocular syphilis at a tertiary referral center in India. Demographics, history, extraocular and ocular manifestations, ocular and systemic investigations, treatment and visual acuity outcomes were noted. All patients were diagnosed after necessary laboratory investigations including HIV ELISA (Human immunodeficiency virus, enzyme-linked immunosorbent assay), VDRL (venereal disease research laboratory), and TPHA (treponema pallidum hemagglutination). RESULTS: Totally, 20 patients with mean age at presentation 38.25 ± 9.76 were analyzed. 9/20 patients had bilateral involvement. 8/20 had concurrent HIV at presentation with an average CD4 counts of 592.25 ± 411.34 cells/microliter. The mean duration of symptoms at time of presentation was 15.45 ± 35.15 weeks. VDRL test was reactive in 45% (9/20) patients whereas, all patients had a reactive TPHA test. Clinical manifestations included outer retinal placoid chorioretinitis lesions (8/20, 40%), followed by retinitis mimicking acute retinal necrosis as the second most common phenotype (4/20, 20%). Other presenting manifestations noted were panuveitis, miliary retinitis lesions, retinal vasculitis, intermediate uveitis, and anterior uveitis. The clinical phenotypes in immunocompromised included panuveitis, acute retinal necrosis and isolated anterior uveitis. Mean follow up duration was 6.32 ± 6.15 months. An improvement in mean best corrected visual acuity (BCVA) of (0.63 LogMAR, approximately 6 Snellen lines, P < 0.02) was noted at last follow-up. CONCLUSION: Phenotypic manifestations of ocular syphilis are varied. Non-treponemal tests like VDRL may be unreliable when compared with treponemal tests in diagnosing ocular syphilis. Syphilitic uveitis is considered equivalent to neurosyphilis and is treated similar to neurosyphilis.

Enterobacter endophthalmitis: Clinical settings, susceptibility profile, and management outcomes across two decades.

Purpose: To describe the clinical presentation and management of Enterobacter endophthalmitis and compare with previous in-house published literature. Methods: This was a retrospective interventional comparative case series involving 44 cases with culture proven Enterobacter endophthalmitis from April 2006 to August 2018 who underwent vitrectomy/vitreous biopsy, intravitreal antibiotics with or without additional procedures as appropriate. The current outcomes were compared to the outcomes previously reported a decade back from our center. The mean age at presentation, predisposing factor, number of interventions, interval between inciting event and presentation, type of intravitreal antibiotic used, anatomic, and the functional outcomes were analyzed and compared to the previous series. Results: There were 30 males. Mean age was 22.73 ± 21.35 years (median 14 years). Inciting event was open globe injury in 34 (77.27%) eyes, 4 (9.09%) eyes following cataract surgery, 3 (6.81%) eyes with endogenous endophthalmitis, 2 (4.54%) eyes following keratoplasty, and 1 eye (2.27%) following trabeculectomy. Presenting visual acuity was favorable (≥20/400) in 2 eyes (4.54%), at the final visit it was in 11 eyes (25%). The organisms were most sensitive to ciprofloxacin (95.12%), amikacin (90.47%), and ceftazidime (85.36%). A comparison of the current study with previous in-house study showed that number of eyes with presenting vision ≥20/400 as well as final vision ≥20/400 were comparable. Susceptibility was highest to ciprofloxacin 39 (95.12%) (previous series) and 33 (92%) (current series). Conclusion: Enterobacter organisms show susceptibility to ciprofloxacin, amikacin, and ceftazidime. Susceptibility profile, clinical presentations, and management remain largely similar over many years. Final outcome is unfavorable.