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Typical ocular coloboma is caused by defective closure of the embryonal fissure. The occurrence of coloboma can be sporadic, hereditary (known or unknown gene defects) or associated with chromosomal abnormalities. Ocular colobomata are more often associated with systemic abnormalities when caused by chromosomal abnormalities. The ocular manifestations vary widely. At one extreme, the eye is hardly recognisable and non-functional—having been compressed by an orbital cyst, while at the other, one finds minimalistic involvement that hardly affects the structure and function of the eye. In the fundus, the variability involves the size of the coloboma (anteroposterior and transverse extent) and the involvement of the optic disc and fovea. The visual acuity is affected when coloboma involves disc and fovea, or is complicated by occurrence of retinal detachment, choroidal neovascular membrane, cataract, amblyopia due to uncorrected refractive errors, etc. While the basic birth anomaly cannot be corrected, most of the complications listed above are correctable to a great extent. Current day surgical management of coloboma-related retinal detachments has evolved to yield consistently good results. Cataract surgery in these eyes can pose a challenge due to a combination of microphthalmos and relatively hard lenses, resulting in increased risk of intra-operative complications. Prophylactic laser retinopexy to the border of choroidal coloboma appears to be an attractive option for reducing risk of coloboma-related retinal detachment. However, a majority of the eyes have the optic disc within the choroidal coloboma, thus making it difficult to safely administer a complete treatment.

Aim To report serious adverse events and long-term outcomes of initial experience with intraocular bevacizumab in retinopathy of prematurity (ROP). Methods Consecutive vascularly active ROP cases treated with bevacizumab, in addition to laser and surgery, were analysed retrospectively from a prospective computerised ROP database. Primary efficacy outcome was regression of new vessels. Secondary outcomes included the anatomic and visual status. Serious systemic and ocular adverse events were documented. Results 24 ROP eyes in 13 babies, received single intraocular bevacizumab for severe stage 3 plus after failed laser (seven eyes), stage 4A plus (eight eyes), and stage 4B/5 plus (nine eyes). Drug was injected intravitreally in 23 eyes and intracamerally in one eye. New vessels regressed in all eyes. Vision salvage in 14 of 24 eyes and no serious neurodevelopmental abnormalities were noted up to 60 months (mean 30.7 months) follow-up. Complications included macular hole and retinal breaks causing rhegmatogenous retinal detachment (one eye); bilateral, progressive vascular attenuation, perivascular exudation and optic atrophy in one baby, and progression of detachment bilaterally to stage 5 in one baby with missed follow-up. One baby who received intracameral injection developed hepatic dysfunction. One eye of this baby also showed a large choroidal rupture. Conclusions Though intraocular bevacizumab, along with laser and surgery salvaged vision in many otherwise progressive cases of ROP, vigilance and reporting of serious adverse events is essential for future rationalised use of the drug. We report one systemic and four ocular adverse events that require consideration in future use of the drug.

Recent decade has seen a shift in the causes of childhood blinding diseases from anterior segment to retinal disease in both developed and developing countries. The common retinal disorders are retinopathy of prematurity and vitreoretinal infections in neonates, congenital anomalies in infants, and vascular retinopathies including type 1 diabetes, tumors, and inherited retinal diseases in children (up to 12 years). Retinal imaging helps in diagnosis, management, follow up and prognostication in all these disorders. These imaging modalities include fundus photography, fluorescein angiography, ultrasonography, retinal vascular and structural studies, and electrodiagnosis. Over the decades there has been tremendous advances both in design (compact, multifunctional, tele-consult capable) and technology (wide- and ultra-wide field and noninvasive retinal angiography). These new advances have application in most of the pediatric retinal diseases though at most times the designs of new devices have remained confined to use in adults. Poor patient cooperation and insufficient attention span in children demand careful crafting of the devices. The newer attempts of hand-held retinal diagnostic devices are welcome additions in this direction. While much has been done, there is still much to do in the coming years. One of the compelling and immediate needs is the pediatric version of optical coherence tomography angiography. These needs and demands would increase many folds in future. A sound policy could be the simultaneous development of adult and pediatric version of all ophthalmic diagnostic devices, coupled with capacity building of trained medical personnel.

Purpose: To describe the clinical profile and treatment outcomes of infants with retinopathy of prematurity (ROP) presenting with isolated exudative retinal detachment (ERD). Methods: Retrospective interventional case series. Preterm infants diagnosed with ROP with ERD at presentation were included. All demographic details, clinical findings, and treatment given were documented. The anatomical outcome was categorized as good, fair, and poor. Refractive outcome was classified into mild, moderate, and severe according to spherical equivalent at the last visit. Results: Fifteen eyes (8 patients) were included. Mean gestational age was 31.3 weeks, and birth weight was 1462.6 g. All eyes presented with aggressive ROP. Patches of retinal edema in avascular retina were seen in all eyes. A total of 86.6% of eyes had vascular sclerosis while 86.6% of eyes had subretinal exudates. The anatomical outcome was good in all eyes. In addition, 40% of eyes had a mild refractive error. Conclusion: Exudative retinal detachment in ROP is rare. The use of unregulated oxygen can be a contributory factor. Vascular sclerosis is consistent with hyperoxia-induced retinopathy models. Retinal edema and subretinal exudates indicate disrupted inner and outer blood-retinal barrier. Treatment outcomes are good when diagnosed and treated in time.

Albinism describes a heterogeneous group of genetically determined disorders characterized by disrupted synthesis of melanin and a range of developmental ocular abnormalities. The main ocular features common to both oculocutaneous albinism (OCA), and ocular albinism (OA) include reduced visual acuity, refractive errors, foveal hypoplasia, congenital nystagmus, iris and fundus hypopigmentation and visual pathway misrouting, but clinical signs vary and there is phenotypic overlap with other pathologies. This study reviews the prevalence, genetics and ocular manifestations of OCA and OA, including abnormal development of the optic chiasm. The role of visual electrophysiology in the detection of chiasmal dysfunction and visual pathway misrouting is emphasized, highlighting how age-associated changes in visual evoked potential (VEP) test results must be considered to enable accurate diagnosis, and illustrated further by the inclusion of novel VEP data in genetically confirmed cases. Differential diagnosis is considered in the context of suspected retinal and other disorders, including rare syndromes that may masquerade as albinism.

Purpose: To measure the proportion of people with major ophthalmic and retinal disorders in the tribal and non-tribal people presenting to a community eye hospital in an Indian state with a sizeable tribal population. Methods: Hospital-based cross-sectional retrospective study of all new adult patients, examined between September 2015 and June 2020. A tribal was defined as per the Indian ethnic classification. Blindness and visual impairment were defined as per the WHO standards. Diabetes and hypertension were defined as per Indian standards. The proportion of common ophthalmic and retinal disorders between the tribal and non-tribal community were compared. Results: This cohort consisted of 76,166 people (45.8%; n = 34,869, tribal); 39.4% (n = 29,989; non-tribal 23.6% and tribal 15.8%) people had ophthalmic disorders. In the examined people 2.3% were blind (higher in tribal community 4.7% versus 0.8%; P < 0.001) and 8.4% had moderate-to-severe visual impairment (higher in tribal community 14.4% versus 4.4%; P < 0.001). Refractive error (64.4%; higher in non-tribal community, 77.3% versus 44.6%, P < 0.001) and operable cataract (23.9%; higher in tribal community, 40.9% versus 11.8%, P < 0.001) were the principal ophthalmic disorders. Retinal disorders were higher in non-tribal people (5.9% vs. 2.9%; P < 0.001), but the tribal group had higher proportion of retinitis pigmentosa (20% vs. 6.4%; P < 0.001) and lower proportion of diabetic retinopathy (8% vs. 40.7%; P < 0.001). Conclusion: The health-seeking behavior of the tribal community in India is low. A tribal person in India apparently visits the hospital when vision is grossly affected. It calls for greater advocacy, increased access to healthcare, and a larger population-based study.

Purpose: To analyze the weekly rate of retinal vascular growth in treatment-naïve babies with various stages of retinopathy of prematurity (ROP) and validate if this could be a predictor of treatment need. Methods: Retrospective review of medical charts and retinal images of babies with various stages of ROP. The images were enhanced using red-green image enhancement software. Using the length of the horizontal disc diameter (DD) of each eye, the vessel growth was measured from the disc margin up to the vessel tip in fixed quadrants. The rate of vessel growth was the ratio of vessel length to the number of weeks it took to reach this length. The babies were divided into treatment warranting ROP (group 1), low-risk pre-threshold (type II) ROP (group 2,), and no-ROP (group 3) for analysis. The \"no-ROP\" group acted as normal control. Group 1 was further subdivided into 1A (threshold ROP), IB (aggressive posterior ROP), 1C (hybrid ROP), and ID (high-risk pre-threshold ROP). Results: Out of 436 eyes, groups 1, 2, and 3 had 238, 108, and 90 eyes, respectively. The mean rate of vascular outgrowth along with 95% confidence interval (CI) was 0.490 [0.487,0.520], 0.612 [0.599, 0.638], and 0.719 [0.703, 0.740] DD/week, respectively, for babies with \"treatment warranting,\" \"low risk pre-threshold\" and \"no ROP\" groups, respectively. In our estimate, more than 80% of eyes with a vessel growth rate of 0.54 DD/week or less required treatment Conclusion: A rate of retinal vascular growth less than 0.54 DD/week can be used to determine treatment requirements in babies with ROP.

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.

Purpose: To analyze the causes for late presentation in a series of patients with advanced retinopathy of prematurity (ROP) in a tertiary eye care institute in Eastern India. Methods: We analyzed our medical records and ROP database retrospectively from 2007 to 2015 and prospectively thereafter till 2017 to identify the factors for late presentation in babies with advanced ROP (stages 4 and 5). Results: A total of 71 eligible subjects were analyzed. The mean chronological age was 15.1 months (2 months to 14 years). The three important barriers were: (1) the system and neonatal care policy failure (n = 45; 63.3%), (2) parental negligence and ignorance (n = 19; 26.7%), and (3) ophthalmologist's misdiagnosis or unavailability (n = 7; 10%). Majority of the babies (63.3%) were admitted in the neonatal care unit when they were due for ROP screening with an average duration of stay of 35.5 days. Conclusion: The main barriers to early screening for ROP were related to availability of trained human resources, ignorance of \"parents and health care personnel,\" and distance from the point of care. This calls for training of ophthalmologists, advocacy with neonatologists and parents, and create systems for better coordination and compliance of the care providers.

Purpose: To describe clinical and imaging characteristics of the outer retinal folds (ORF) in cases of retinitis, retinochoroiditis, and chorioretinitis. Methods: Retrospective review of retinitis cases with presence of ORFs either at presentation or during follow up. Results: ORFs were seen adjacent to retinitis lesions in 16 eyes of 14 cases (retinitis post-febrile illness n = 10, toxoplasma retinochoroiditis n = 2, fungal chorioretinitis n = 2) either at presentation (n = 2) or during follow up (n = 14). Optical coherence tomography (OCT) appearance was outer retinal vertical stout lesions involving ellipsoid, external limiting membrane, and outer nuclear layer. All the cases had a presence of past or concurrent subretinal fluid and/or subretinal hyperreflective material when ORF was seen. ORF resolved with variable outer retinal atrophy over a mean period of 2.86 months. Conclusion: ORF is observed in cases of retinitis with subretinal fluid either at presentation or during resolution. It is not specific to any etiological disease. Differentiation of this sign from vertical outer retinal stripes in viral retinitis on OCT is important to avoid misinterpretation.