A Case of Bilateral Diffuse Uveal Melanocytic Proliferation Followed by Massive Unilateral Uveal Proliferation.

OBJECTIVE: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) followed by massive unilateral uveal proliferation. METHODS: Retrospective case report. RESULTS: A 47-year-old female with history of metastatic ovarian carcinoma initially presented with bilateral vision loss and multifocal red patches on posterior poles consistent with BDUMP. Five years later, she presented with bilateral neovascular glaucoma and unilateral iris and ciliary body mass concerning for malignancy. Enucleation revealed diffuse uveal growth involving almost the entirety of the uveal tract. CONCLUSIONS: BDUMP can rarely be associated with uveal proliferation. Routine examinations are recommended to monitor for any changes concerning malignancy.

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).

The scope of artificial intelligence in retinopathy of prematurity (ROP) management.

Artificial Intelligence (AI) is a revolutionary technology that has the potential to develop into a widely implemented system that could reduce the dependence on qualified professionals/experts for screening the large at-risk population, especially in the Indian scenario. Deep learning involves learning without being explicitly told what to focus on and utilizes several layers of artificial neural networks (ANNs) to create a robust algorithm that is capable of high-complexity tasks. Convolutional neural networks (CNNs) are a subset of ANNs that are particularly useful for image processing as well as cognitive tasks. Training of these algorithms involves inputting raw human-labeled data, which are then processed through the algorithm's multiple layers and allow CNN to develop their own learning of image features. AI systems must be validated using different population datasets since the performance of the AI system would vary according to the population. Indian datasets have been used in AI-based risk model that could predict whether an infant would develop treatment-requiring retinopathy of prematurity (ROP). AI also served as an epidemiological tool by objectively showing that a higher ROP severity was in Neonatal intensive care units (NICUs) that did not have the resources to monitor and titrate oxygen. There are rising concerns about the medicolegal aspect of AI implementation as well as discussion on the possibilities of catastrophic life-threatening diseases like retinoblastoma and lipemia retinalis being missed by AI. Computer-based systems have the advantage over humans in not being susceptible to biases or fatigue. This is especially relevant in a country like India with an increased rate of ROP and a preexisting strained doctor-to-preterm child ratio. Many AI algorithms can perform in a way comparable to or exceeding human experts, and this opens possibilities for future large-scale prospective studies.

Strabismus management in retinoblastoma survivors.

PURPOSE: To report the result of strabismus surgery in eye-salvaged retinoblastoma (Rb) patients. METHODS: A retrospective case series including 18 patients with Rb and strabismus who underwent strabismus surgery after completing tumor treatment by a single pediatric ophthalmologist. RESULTS: A total of 18 patients (10 females and 8 males) were included with a mean age of 13.3 ± 3.0 (range, 2-39) months at the time tumor presentation and 6.0 ± 1.5 (range, 4-9) years at the time of strabismus surgery. Ten (56%) patients had unilateral and 8(44%) had bilateral involvement and the most common worse eye tumor's group was D (n = 11), C (n = 4), B (n = 2) and E (n = 1). Macula was involved by the tumors in 12 (67%) patients. The tumors were managed by intravenous chemotherapy (n = 8, 47%), intra-arterial chemotherapy (n = 7, 41%) and both (n = 3, 17%). After complete treatment, the average time to strabismus surgery was 29.9 ± 20.5 (range, 12-84) months. Except for one, visual acuity was equal or less than 1.0 logMAR (≤ 20/200) in the affected eye. Seven (39%) patients had exotropia, 11(61%) had esotropia (P = 0.346) and vertical deviation was found in 8 (48%) cases. The angle of deviation was 42.0 ± 10.4 (range, 30-60) prism diopter (PD) for esotropic and 35.7 ± 7.9 (range, 25-50) PD for exotropic patients (P = 0.32) that after surgery significantly decreased to 8.5 ± 5.3 PD in esotropic cases and 5.9 ± 6.7 PD in exotropic cases (P < 0.001). The mean follow-up after surgery was 15.2 ± 2.0 (range, 10-24) months, in which, 3 (17%) patients needed a second surgery. CONCLUSION: Strabismus surgery in treated Rb is safe and results of the surgeries are acceptable and close to the general population. There was not associated with tumor recurrence or metastasis.

Diagnosis of primary vitreoretinal lymphoma masquerading infectious retinitis by retinal biopsy.

PURPOSE: To report a case of primary vitreoretinal lymphoma masquerading as infectious retinitis that was diagnosed via a retinal biopsy. OBSERVATIONS: A 72-year-old female patient was referred to our ophthalmology clinic for evaluation of retinitis and vasculitis in the right eye (OD). On examination, best-corrected visual acuities (BCVAs) were hand motions OD and 20/20 in the left eye (OS). Fundus examination revealed optic disc edema and diffuse retinal whitening superior to the superotemporal arcade OD. Given the high suspicion of infectious retinitis, the patient was treated with intravitreal foscarnet, systemic acyclovir, and oral prednisone and underwent a comprehensive uveitis workup, which was unremarkable for viral and autoimmune entities. Given the patient's history of diffuse large B cell lymphoma with cutaneous involvement, vitreoretinal lymphoma was suspected, prompting pars plana vitrectomy with a retinal biopsy. Biopsy and immunohistochemistry results were consistent with B-cell lymphoma, and the patient was treated with high-dose methotrexate and rituximab. At 5-month follow-up, BCVAs were hand motions OD and 20/30 OS, and fundus examination demonstrated disc edema with resolution of retinal whitening OD. She responded well to the treatment with regression of vitreoretinal lymphoma on examination and is being monitored closely for lymphoma recurrence. CONCLUSIONS AND IMPORTANCE: Although uncommon, patients with vitreoretinal lymphoma may masquerade as infectious retinitis, and vitreoretinal lymphoma should be suspected when refractory to antiviral therapy and in the setting of a negative workup for viral etiologies. Vitrectomy with retinal biopsy may be considered to aid the diagnosis of vitreoretinal lymphoma although careful consideration of the risks and benefits is warranted.

Multimodal Imaging Study of Patients With Vitamin A Deficiency Retinopathy.

OBJECTIVES: To describe multimodal imaging findings of vitamin A deficiency retinopathy. METHODS: A retrospective study of patients with serum retinol < 0.3 mg/L. Fundus color photos, spectral domain-optical coherence tomography (SD-OCT), and fundus autofluorescence (FAF) were reviewed and, when available, electrophysiological tests were analyzed. RESULTS: Forty-five eyes (63.9 ± 15.7 years) were included. Ultra-widefield fundus photography showed drusen-like deposits (53.3%) and macular retinal pigment epithelium (RPE) mottling (40%). The deposits were hypoautofluorescent, and a perifoveal hyperautofluorescent ring was present in 8.9%. By SD-OCT, the ellipsoid zone had an irregular appearance (100%) and conical deposits anterior to the RPE (33.3%). Electroretinogram (ERG) (66.7%) showed a decrease in b-wave in the scotopic registers, and microperimetry (4.4%) showed decreased foveal sensitivity. After vitamin A supplementation, SD-OCT and FAF showed resolution of all findings. Forty percent of eyes had restoration of the scotopic registers in ERG and improved macular sensitivity by microperimetry (4.4%). CONCLUSIONS: Vitamin A deficiency causes a mild cone dysfunction in addition to the more severe absent rod response. [Ophthalmic Surg Lasers Imaging Retina 2023;54:330-336.].

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.

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.

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.

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.

A case of primary vasoproliferative tumor with full-thickness macular hole.

A 53-year-old female presented with defective vision in the left eye of one week duration with a best-corrected visual acuity (BCVA) of 6/9. Fundus examination showed a primary retinal vasoproliferative tumor (RVPT) at the inferotemporal quadrant and a small full-thickness macular hole (MH). The patient underwent cryotherapy for RVPT, followed later by vitrectomy with internal limiting membrane peeling and gas tamponade. The tumor regressed and the MH was closed with BCVA of 6/9. RVPT can present with remote macular complications like epiretinal membrane, cystoid macular edema, macular exudates, and rarely full-thickness MH. Management should be directed towards the tumor as well as the complication.

All India Ophthalmological Society (AIOS) Task Force guidelines to prevent intraocular infections and cluster outbreaks after cataract surgery.

Infectious endophthalmitis is a serious and vision-threatening complication of commonly performed intraocular surgeries such as cataract surgery. The occurrence of endophthalmitis can result in severe damage to the uveal and other ocular tissues even among patients undergoing an uncomplicated surgical procedure. If the infections result from common factors such as surgical supplies, operative or operation theater-related risks, there can be a cluster outbreak of toxic anterior segment syndrome (TASS) or infectious endophthalmitis, leading to several patients having an undesirable outcome. Since prevention of intraocular infections is of paramount importance to ophthalmic surgeons, the All India Ophthalmological Society (AIOS) has taken the lead in the formation of a National Task Force to help ophthalmic surgeons apply certain universal precautions in their clinical practice. The Task Force has prepared a handy checklist and evidence-based guidelines to minimize the risk of infectious endophthalmitis following cataract surgery.

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.

Endophthalmitis in Eyes with Osteo-odonto Keratoprosthesis.

Aim: To analyze the incidence, microbiological profile, risk factors, and outcomes in eyes with osteo-odonto keratoprosthesis (OOKP) developing endophthalmitis.Methods & material: Retrospective review of medical records of patients presenting between 2003 and 2013 with OOKP and endophthalmitis was undertaken after obtaining IRB approval. Patient demographics, clinical features, microbiological evaluation, treatment details, and outcomes were analyzed.Results: Of 92 eyes with OOKP, 8 (9%) had endophthalmitis. Indications for OOKP included Steven-Johnson syndrome (SJS; n = 6; 75%) and chemical injuries (n = 2; 25%). The mean duration from OOKP surgery to endophthalmitis was 49.4 ± 32.3 months. Seven of eight eyes had associated laminar resorption. Endophthalmitis resolved with conservative management in three eyes. Lamina removal was required in three eyes; one eye underwent evisceration. At presentation, mean BCVA was 2.76 ± 0.24 logMAR that improved to 2.48 ± 1.0 logMAR after intervention. Mean follow-up was 12.6 ± 13.2 months.Conclusion: Endophthalmitis portends a grave prognosis for eyes with OOKP. The most important risk factors are SJS (75%) and lamina resorption (88%).

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.

Indocyanine green angiography imaging findings in artery occlusions.

PURPOSE: To describe the multimodal imaging features including indocyanine green angiography (ICGA) in cases diagnosed clinically as central retinal artery occlusion (CRAO) at its different disease stages. METHODS: In this retrospective observational study, patients diagnosed clinically as CRAO or hemi-CRAO were included. All patients underwent multimodal imaging with optical coherence tomography (OCT), fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were studied. Analysis of ICGA images in different stages of artery occlusions and its correlation with accompanying FFA and OCT images was done. RESULTS: Eight such studies in five patients were available for analysis. The most important observation noted on ICGA was the presence of hypercyanescent spots seen during the acute stages of the disease in four of the five cases. The spots were accompanied by retinal vessel staining on FFA in the corresponding region. As the disease showed signs of resolution, the hypercyanescent spots on ICGA and retinal vessel staining on FFA disappeared. The hypercyanescent spots seen on the ICGA were noted due to the red blood cell aggregation or 'rouleaux' formation. In addition, choroidal perfusion abnormalities were noted on ICGA in all five cases in the acute stage. CONCLUSION: Choroidal perfusion changes can be identified in acute phase of retinal artery occlusion. Rouleaux formation in the retinal circulation occurs due to the slowing of the blood flow following artery occlusion. These are seen as hypercyanescent spots in the late phase on ICGA.

Intravitreal Ozurdex has no short term influence on choroidal thickness and vascularity index in eyes with diabetic macular edema: A pilot study.

AIM: To analyze choroidal parameters in eyes with diabetic macular edema (DME) treated with intravitreal Ozurdex. PATIENTS AND METHODS: Twenty eyes of 14 patients were included in this prospective study. Optical coherence tomography images were obtained before and 8-10 weeks after intravitreal Ozurdex injection; binarized and subfoveal choroidal thickness (SFCT) and choroidal vascularity index (CVI) were calculated. RESULTS: Mean SFCT (treatment naïve; 242.22 ± 32.87 reduced to 218.10 ± 22.10, P = 0.158 and previously treated; 330.4 ± 56.72 reduced to 328.93 ± 50.55, P = 0.833) and mean CVI (treatment naïve; 0.64 ± 0.03 changed to 0.65 ± 0.04, P = 0.583 and previously treated; 0.65 ± 0.05 reduced to 0.64 ± 0.03, P = 0.208) showed no significant change. CONCLUSION: Intravitreal Ozurdex showed no significant effects on SFCT and CVI in eyes with DME over short term. Larger studies with longer follow-up may allow a better understanding.

Miliary tuberculosis presenting as bilateral pseudo-retinoblastoma.

Objective: To describe an interesting case of miliary tuberculosis mimicking retinoblastoma. Method: A retrospective case report. Result: The twin brother of a known case of retinobastoma presented with headache. On fundus examination, multiple yellowish-white lesions were noted in both eyes. Magnetic resonance imaging of the brain showed multiple enhancing lesions. A diagnosis of miliary tuberculosis was made and anti-tubercular therapy was started. Conclusion: Ocular tuberculosis can mimic retinoblastoma and lead to diagnostic dilemma especially in cases with family history of retinoblastoma.