The L V Prasad Eye Institute (LVPEI) was established in 1987 at Hyderabad by Gullapalli Nageswara Rao as a not-for-profit, non-government eye care institution. The mission of LVPEI is to provide "equitable and efficient eye care to all sections of society." The institute was founded by Dr. Gullapalli Nageswara Rao.
PURPOSE:To report the presentation, management and outcomes of atypical cases presenting with granulomatous diseases of the lacrimal drainage system. METHODS:Retrospective analysis of biopsy-proven cases presenting with atypical lacrimal drainage system granulomatous diseases over a study period of 2 years (August 2023 to September 2025) was performed. Demographics, clinical presentations, radiological features (CT-DCG), treatment, histopathology, immunohistochemistry, final diagnosis, and outcomes were analysed. RESULTS:During the study period, five such cases were found in the database with the average age of patients being 62.4 years (range: 43-70 years). The diagnosis of the atypical lacrimal sac granulomatous diseases includes xanthogranuloma, sclerosing, and non-sclerosing variants of non-specific orbital inflammatory disease, and fungal granulomas. CONCLUSIONS:Atypical presentations of lacrimal sac granulomatous lesions are an uncommon occurrence and sparsely reported in the literature. They can be differentiated from malignancy based on clinical presentations, a high index of suspicion, and a confirmatory histopathological examination.
To describe previously unrecognized inner retinal cavitations in patients with Enhanced S-cone syndrome (ESCS) carrying specific NR2E3 mutations, and to evaluate their structural characteristics using multimodal imaging. Three patients with molecularly confirmed ESCS, each harboring homozygous NR2E3 variants (c.290G > A [p.Arg97Cys/His] and c.229C > T [p.Arg77Trp]), were evaluated. Detailed clinical assessments included fundus examination, full-field electroretinography (ERG), and multimodal retinal imaging, including swept-source optical coherence tomography (SS-OCT), fundus autofluorescence (FAF), and red-free photography. All patients exhibited classical ESCS functional features: nyctalopia, hyperopia with full-field ISCEV-standard ERGs demonstrating pathognomonic changes consistent with ESCS and additional S-cone ERGs of greater amplitude than standard light-adapted (LA 3.0) ERGs. Bilateral, perhaps oblong like, hypopigmented retinal cavitations were observed along the vascular arcades and nasal to the optic disc. SS-OCT localized these lesions primarily to the ganglion cell and inner plexiform layers, occasionally extending to the outer plexiform layer. Red-free imaging delineated cavitations more clearly than FAF. Hyperautofluorescent foci partially overlapped with the cavitations but did not match their shape. In this case series, inner retinal cavitations were observed in NR2E3-associated enhanced S-cone syndrome and may represent a previously underrecognized structural feature. Larger studies are needed to establish the broader prevalence of this finding across NR2E3 genotypes.
The light-rise of the electro-oculogram (EOG) is used as a clinical marker for a collection of disorders known as the ‘bestrophinopathies.’ This review provides an overview of these conditions including Best Vitelliform Macular Dystrophy (BVMD, Autosomal Recessive Bestrophinopathy (ARB), Adult Onset Vitelliform Macular Dystrophy (AVMD) and Autosomal Dominant Vitreoretinalchoriodopathy (ADVIRC) and potential future therapies. One drawback of the EOG is the time to administer the test and shortened protocols that have been developed to improve the clinical testing of the EOG which include incorporating measures during recordings of the ERG or shortening the period of dark and light adaptation. The companion paper summarizes the cellular mechanism of the EOG, and this review is focused on the clinical applications of the EOG.
AIM:To conduct an analysis of ophthalmic manifestations in leukaemic patients and evaluate their impact on visual outcomes and disease remission. METHODS:This retrospective, comparative study included 244 patients (378 eyes) diagnosed with leukaemias between January 2016 and 2024. Data included demography, leukaemia type, ophthalmic manifestations, imaging modalities, treatment modalities (ocular and systemic), treatment outcomes (relapses or remission) and final visual acuity. Univariate and multivariate regression analyses were performed to identify correlations and predictive features associated with visual outcomes. RESULTS:The mean age of patients was 34.9±22.1 years (range, 1-88 years). Acute lymphocytic leukaemia was the most common subtype (58%), followed by chronic myeloid leukaemia (CML; 20%). Acute forms manifested earlier in life and were more aggressive. The presence of posterior segment manifestations (64%) was more than anterior segment manifestations (52%). Myeloid leukaemia types were more often associated with haemorrhagic manifestations, whereas lymphoid types were associated with non-haemorrhagic manifestations. Univariate regression analysis showed that the presence of epiretinal membrane (OR 0.110, 95% CI 0.012 to 0.951, p=0.04), submacular haemorrhage (OR 0.091, 95% CI 0.010 to 0.763, p=0.027) and type of leukaemia (acute myeloid leukaemia (AML) (OR 3.195, 95% CI 1.697 to 6.012, p<0.000) or CML (OR 6.39, 95% CI 3.130 to 13.041, p<0.000)) had significant association with poor visual outcome. Multivariate regression analysis revealed AML (OR 2.769, 95% CI 1.392 to 5.507, p=0.004) and CML (OR 7.313, 95% CI 3.326 to 16.074, p<0.000) as independent predictors of poor visual outcome. CONCLUSION:Ophthalmic manifestations are major diagnostic and prognostic clues for leukaemias. Pattern recognition facilitates timely recognition, risk stratification, management and disease remission prediction.
