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PURPOSE: After infantile cataract surgery, axial elongation, induces a myopic shift that cannot be fully compensated by corneal flattening and the rate is unpredictable owing to the non-linear growth of the eye. The current prospective study assesses the myopic shift and visual outcomes in children undergoing cataract surgery in infancy over a follow-up period of 5 years. MATERIALS AND METHODS: A prospective study conducted at a tertiary eye care center to evaluate the five-year myopic shift, refractive and visual outcomes in infants, who underwent surgery for congenital cataract in infancy. The visual acuity, myopic shift and biometric changes are compared between the aphakia and pseudophakia group. RESULTS: The mean best-corrected visual acuity (BCVA) recorded in logMAR at 5 years for aphakia group was 0.92±0.44 and for pseudophakia group was 0.66±0.42. (pvalue: 0.002102). The myopic shift was noted to be -5.9+/-5.16 in the aphakia group whereas it was -9.01+/- 3.11 in the pseudophakia group (P value= 0.002101) at 5 years after surgery for infantile cataract. CONCLUSION: IOL implantation in eyes of infants undergoing cataract surgery is feasible in eyes that strictly satisfy the pre-operative inclusion criteria and the visual outcomes in these eyes are better compared to aphakia group at 5 years follow up. Eyes with primary IOL implantation had a higher myopic shift compared to ones without primary IOL implantation. Eyes undergoing primary IOL implantation, need higher under correction compared to the current available formulae.

Purpose: There is insufficient literature reporting the concurrent occurrence of retinal ischemic lesions with optic neuritis. In this case report, we present a distinctive instance of Optic Neuritis with a positive Myelin Oligodendrocyte glycoprotein (MOG) antibody, accompanied by retinal ischemia manifesting as paracentral acute middle maculopathy (PAMM) lesions. Observations: Our patient is a 25-year-old female who tested positive for MOG antibodies and exhibited retinal PAMM lesions without any apparent underlying ischemic cause. She received intravenous pulse steroid therapy, and her symptoms and signs completely resolved one month later. Conclusion: PAMM can serve as an initial manifestation of Myelin Oligodendrocyte glycoprotein Antibody Associated Disease (MOGAD). This case has the potential to contribute to the existing literature, facilitating a deeper exploration of the pathophysiology of retinal ischemia in MOG associated optic neuritis.

PURPOSE: To study the clinical presentations, visual, and refractive profiles of children with congenital ectopia lentis in a large cohort of patients from a tertiary eye care network in India. MATERIALS AND METHODS: A retrospective review of electronic medical records from December 2012 to December 2020 was conducted. Two hundred and ninety-seven consecutive children ≤18 years of age at presentation were identified and analyzed for demographic details, patient distribution, lens subluxation, visual, and refractive profiles before and after the interventions. RESULTS: Five hundred and ninety-four eyes of 297 (male 56%; n = 166) patients were analyzed. The mean age at presentation was 8.74 ± 3.89. Best-corrected visual acuity (BCVA) at presentation ranged from 0.3 logMAR to 3.5 logMAR; (Snellen: 6/9 – close to face [CF]) (mean 0.89 ± 0.68). High myopia (n = 201; 33.83%) and mild astigmatism (n = 340; 57.23%) were more frequent. Temporal (n = 108; 18.18%) subluxation was most common followed by superior. Lensectomy with limited vitrectomy was performed in 243 eyes of 127 patients (40.90%). Median preoperative BCVA was 1.0 (range: 0.3–3.5 logMAR; 20/40 - CF). Median postoperative BCVA was 0.5 logMAR (6/18) in the pseudophakic group and 0.6 logMAR (6/24) in the aphakic group. Spherical equivalent in myopic children reduced from −12.06 ± 6.84D to −1.57D (−0.25D to − 5.5D) in the pseudophakic group and +9.3D (+5.5D to 15.5D) in the aphakic group. CONCLUSION: This study is a large cohort of children presenting with ectopia lentis. Following intervention, an improvement in the median BCVA and refractive correction was noted in the entire cohort.

Abstract Neuroscientific research has consistently shown more extensive non-visual activity in the visual cortex of congenitally blind humans compared to sighted controls; a phenomenon known as crossmodal plasticity. Whether or not crossmodal activation of the visual cortex retracts if sight can be restored is still unknown. The present study, involving a rare group of sight-recovery individuals who were born pattern vision blind, employed visual event-related potentials to investigate persisting crossmodal modulation of the initial visual cortical processing stages. Here we report that the earliest, stimulus-driven retinotopic visual cortical activity (

Myopia is a major public health problem worldwide, including India, with the global prevalence of myopia increasing rapidly over decades. The clinical and socioeconomic impact of myopia is also expected to rise with rising prevalence. Therefore, the focus has now been shifted to prevent the incidence and progression of myopia. However, there is lack of any standardized guidelines for myopia management. This document aims to generate a national-level expert consensus statement on the management of childhood myopia in the Indian scenario. The expert panel of pediatric ophthalmologists consisted of 63 members who met in a hybrid meeting. A list of topics deliberating discussion in the meeting was provided to the experts in advance and they were instructed to provide their opinions on the matter during the meet. The panel of experts then gave their views on each of the items presented, deliberated on different aspects of childhood myopia, and reached a consensus regarding the practice patterns in the Indian scenario. In case of opposing views or lack of a clear consensus, we undertook further discussion and evaluated literature to help arrive at a consensus. A written document is prepared based on recommendations explaining definition of myopia, refraction techniques, components and methods of workup, initiation of anti-myopia treatment, type and timing of interventions, follow-up schedule, and indications for revised or combination treatment. This article formulates evidence-based guidelines for progressing myopes and pre-myopes and also establishes uniformity in the management of childhood myopia in the country.

Congenital blindness leads to profound changes in electroencephalographic (EEG) resting state activity. A well-known consequence of congenital blindness in humans is the reduction of alpha activity which seems to go together with increased gamma activity during rest. These results have been interpreted as indicating a higher excitatory/inhibitory (E/I) ratio in visual cortex compared to normally sighted controls. Yet it is unknown whether the spectral profile of EEG during rest would recover if sight were restored. To test this question, the present study evaluated periodic and aperiodic components of the EEG resting state power spectrum. Previous research has linked the aperiodic components, which exhibit a power-law distribution and are operationalized as a linear fit of the spectrum in log-log space, to cortical E/I ratio. Moreover, by correcting for the aperiodic components from the power spectrum, a more valid estimate of the periodic activity is possible. Here we analyzed resting state EEG activity from two studies involving (1) 27 permanently congenitally blind adults (CB) and 27 age-matched normally sighted controls (MCB); (2) 38 individuals with reversed blindness due to bilateral, dense, congenital cataracts (CC) and 77 age-matched sighted controls (MCC). Based on a data driven approach, aperiodic components of the spectra were extracted for the low frequency (Lf-Slope 1.5 to 19.5 Hz) and high frequency (Hf-Slope 20 to 45 Hz) range. The Lf-Slope of the aperiodic component was significantly steeper (more negative slope), and the Hf-Slope of the aperiodic component was significantly flatter (less negative slope) in CB and CC participants compared to the typically sighted controls. Alpha power was significantly reduced, and gamma power was higher in the CB and the CC groups. These results suggest a sensitive period for the typical development of the spectral profile during rest and thus likely an irreversible change in the E/I ratio in visual cortex due to congenital blindness. We speculate that these changes are a consequence of impaired inhibitory circuits and imbalanced feedforward and feedback processing in early visual areas of individuals with a history of congenital blindness.

Objective To study the visual, refractive and surgical outcomes of intraocular lens (IOL) implantation versus aphakia in children with microspherophakia.Design Retrospective, comparative, non-randomised interventional study.Methods All consecutive children with microspherophakia who satisfied the inclusion criteria were included. The eyes that underwent in-the-bag IOL implantation and those that were left aphakic were included in groups A and B, respectively. The postoperative visual outcomes, IOL stability and complications during the follow-up period were studied.Results 22 eyes (13 patients, male 76%), of which 12 eyes were in group A and 10 eyes in group B. The mean±SE of age at surgery was 9.4±1.4 and 7.3±0.9 years in group A and group B, respectively (p value 0.18). The mean follow-up of group A was 0.9±0.4 years (median 0.5 years; Q1 0.04, Q3 2.16) and group B was 1.3±0.9 years (median 0.147 years; Q1 0.08, Q3 0.39) (p value 0.76). All the baseline biometric variables including best-corrected visual acuity (BCVA) were comparable in each group. The final BCVA in logMAR adjusted for follow-up was comparable in both group A (0.29±0.06) and group B (0.52±0.09) (p value 0.06). Mean predictive error of IOL power in microspherophakia was 0.17±0.43.The most common complication in group A was visual axis opacification of two eyes (16.7%, 95% CI 2.9% to 49.1%), of which one eye (8.3%, 95% CI 0.4% to 40.2%) needed membranectomy. Vitreous in anterior chamber was the most common complication in group B, seen in two eyes (20%, 95% CI 3.5% to 55.8%), of which one eye (10%, 95% CI 0.5% to 45.9%) underwent YAG laser vitreolysis. The survival analysis (p value 0.18) was comparable in each group.Conclusion In-the-bag IOL is an option, which can be considered in selected cases of microspherophakia in developing nations where regular follow-up and economic constraints are a major concern.

Purpose: To study the profile, risk factors, and management outcomes of infantile strabismus at a tertiary eye care center. Methods: We prospectively analyzed the data of infants (children less than 1 year of age) who presented at our institute from August 2018 to December 2019. We excluded infants who did not complete a minimum follow-up of 6 months. Detailed meticulous history based on a set of standardized questionnaires was obtained and a comprehensive ophthalmological examination of the child was performed. Data were collected regarding refractive error (astigmatism; myopia; hyperopia; anisometropia [1.0 DS]; astigmatism [1.0 DS]) and the type of strabismus. Results: During this period, we saw 4,773 infants, out of which 123 infants were diagnosed to have infantile-onset strabismus (hospital prevalence of 2.6%). Boys and girls were equally affected. Sixty-two patients had esotropia, 37 had exotropia, 2 had hypotropia, and 22 had pseudo strabismus. Prematurity, hypermetropia, and anisometropia had increased odds of developing esotropia, whereas delivery by cesarean section, delayed cry at birth, infantile seizures, parental consanguinity, delayed development of milestones, and myopia had increased odds of developing exotropia. Twenty-nine patients underwent a surgical correction. The mean deviation at the first visit was 42.59 ± 15.40 PD and 8.25 ± 12.70 PD at the last visit. For all patients who underwent a squint surgery, the change in ocular deviation was clinically and statistically significant (P-value

Purpose: Head tilt associated with infantile nystagmus syndrome (INS) can be corrected by (a) operating the oblique muscles, (b) horizontally transposing the vertical rectus muscles, or (c) vertically transposing the horizontal rectus muscles. We report three cases of INS with head tilt corrected by vertically transposing the horizontal rectus muscles in both the eyes. Methods: Three cases of head tilt with INS from an institutional practice operated by a single surgeon were retrospectively reviewed and analyzed. The intervention included full tendon width transposition (upward or downward) of all four horizontal rectus muscles to induce cyclotorsion in the direction of head tilt. The primary outcome measure was the correction of head tilt in the primary position. Results: Three patients (boys) of ages ranging from 4 to 7 years with a pre-operative head tilt of 30° were operated upon. Although one patient's oblique muscles had been operated on to correct head tilt, another patient had an unmasked face turn after the surgery, which was corrected with a modified Anderson's procedure. Post-operatively, all patients had a reduction of head tilt to a range of 0–10°. Conclusion: Vertical transposition of horizontal rectus muscles is a simple surgical option to correct head tilt in INS. However, the results may vary based on individual cases.

Purpose: To report retinal nerve fiber layer thickness (RNFLT) in eyes with amblyopia compared with contralateral healthy eyes. Methods: In this cross-sectional study, we included patients with anisometropic amblyopia, strabismic amblyopia, and mixed amblyopia. All subjects underwent complete ophthalmic examination, including RNFLT measurement with time-domain OCT (Stratus OCT) and scanning laser polarimeter (GDX VCC). A paired “t” test was used to compare average and quadrant-wise RNFL thickness between the amblyopic and contralateral normal eyes. In addition, an analysis of variance test was used to compare various RNFL thickness parameters between the three groups. Results: A total of 33 eyes of 33 subjects with anisometropic amblyopia, 20 eyes of 20 subjects with strabismic amblyopia, and 38 eyes of 38 subjects with mixed amblyopia were included. In the anisometropic amblyopia group, the average RNFLT in the amblyopic eye was 98.2 μm and 99.8 μm in the fellow normal eye (P = 0.5), the total foveal thickness was 152.82 μm (26.78) in the anisometropic eye and 150.42 μm (23.84) in the fellow eye (P = 0.38). The difference between amblyopic and contralateral normal eye for RNFL and macular parameters was statistically insignificant in all three groups. The RNFL thickness in four quadrants was similar in the amblyopic and non-amblyopic eye between all three groups and statistically non-significant. Conclusion: Our study showed that RNFL thickness was similar in amblyopic and non-amblyopic eyes between all three amblyopia groups.

We piloted an innovation in teaching by conducting live virtual bedside clinics and evaluated the effectiveness compared to conventional bedside clinics. The purpose is to report the methodology and survey results of this innovation in teaching. A virtual bedside clinic was set up utilizing multiple audio-visual aids at a tertiary eye care facility. The bedside clinic was conducted and streamed live to pre-registered participants across the globe using the Zoom platform. The online survey was conducted comparing its effectiveness with conventional bedside clinics. A total of five sessions were conducted. A total of 2058 participants registered (411/session), of which 938 (45.57%) attended (187/session). A total of 287 participants (30.6%) responded to the survey. The respondents included ophthalmology residents (43.4%), fellows (19%), sub-specialty ophthalmologists (15.4%), general ophthalmologists (12%), and optometrists (9%). More than 95% of the respondents felt that these clinics were equally effective/better in imparting the following: physical examination 97%, clinical knowledge 99.3%, clinical reasoning 98.3%, procedural skills 95%, and communication skills 96.5%. Respondents suggested that these clinics were better/equally effective in the following techniques: general examination (96%), ocular motility (93.3%), nystagmus evaluation (93.3%), and anterior (80%) and posterior segment examination (73.3%). The hybrid mode presentation (97.3%) and discussion with the panel (100%) were reported to be equally effective/much better. Live virtual bedside clinics are a novel and effective way of continuing quality teaching and impactful learning. Most of the bedside manners, procedural skills, and examination techniques can be effectively taught through this virtual platform with a scope to improve anterior and posterior segment examination skills.

Congenital aniridia is a pan ocular disorder characterized by partial or total loss of iris tissue as the defining feature. Classic aniridia, however, has a spectrum of ocular findings, including foveal hypoplasia, optic nerve hypoplasia, nystagmus, late-onset cataract, glaucoma, and keratopathy. The latter three are reasons for further visual compromise in such patients. This entity is often due to mutations in the PAX6 (Paired box protein Pax-6) gene. Recently, aniridia-like phenotypes have been reported due to non-PAX6 mutations as in PITX2, FOXC1, FOXD3, TRIM44, and CYP1B1 as well wherein there is an overlap of aniridia, such as iris defects with congenital glaucoma or anterior segment dysgenesis. In this review, we describe the various clinical features of classic aniridia, the comorbidities and their management, the mutation spectrum of the genes involved, genotype-phenotype correlation of PAX6 and non-PAX6 mutations, and the genetic testing plan. The various systemic associations and their implications in screening and genetic testing have been discussed. Finally, the future course of aniridia treatment in the form of drugs (such as ataluren) and targeted gene therapy has been discussed.

Persistent visual impairments after congenital blindness due to dense bilateral cataracts have been attributed to altered visual cortex development within a sensitive period. Occipital alpha (8–14 Hz) oscillations were found to be reduced after congenital cataract reversal, while participants performed visual motion tasks. However, it has been unclear whether reduced alpha oscillations were task-specific, or linked to impaired visual behavior in cataract-reversed individuals. Here, we compared resting-state and stimulus-evoked alpha activity between individuals who had been treated for dense bilateral congenital cataracts (CC, n = 13, mean duration of blindness = 11.0 years) and age-matched, normally sighted individuals (SC, n = 13). We employed the visual impulse response function, adapted from VanRullen and MacDonald (2012), to test for the characteristic alpha response to visual white noise. Participants observed white noise stimuli changing in luminance with equal power at frequencies between 0 and 30 Hz. Compared to SC individuals, CC individuals demonstrated a reduced likelihood of exhibiting an evoked alpha response. Moreover, stimulus-evoked alpha power was reduced and correlated with a corresponding reduction of resting-state alpha power in the same CC individuals. Finally, CC individuals with an above-threshold evoked alpha peak had better visual acuity than CC individual without an evoked alpha peak. Since alpha oscillations have been linked to feedback communication, we suggest that the concurrent impairment in resting-state and stimulus-evoked alpha oscillations indicates an altered interaction of top-down and bottom-up processing in the visual hierarchy, which likely contributes to incomplete behavioral recovery in individuals who experienced transient congenital blindness.

Abstract Lower resting-state functional connectivity (RSFC) between ‘visual’ and non-‘visual’ neural circuits has been reported as a hallmark of congenital blindness. In sighted individuals, RSFC between visual and non-visual brain regions has been shown to increase during rest with eyes closed relative to rest with eyes open. To determine the role of visual experience on the modulation of RSFC by resting state condition—as well as to evaluate the effect of resting state condition on group differences in RSFC—, we compared RSFC between visual and somatosensory/auditory regions in congenitally blind individuals (n = 9) and sighted participants (n = 9) during eyes open and eyes closed conditions. In the sighted group, we replicated the increase of RSFC between visual and non-visual areas during rest with eyes closed relative to rest with eyes open. This was not the case in the congenitally blind group, resulting in a lower RSFC between ‘visual’ and non-‘visual’ circuits relative to sighted controls only in the eyes closed condition. These results indicate that visual experience is necessary for the modulation of RSFC by resting state condition and highlight the importance of considering whether sighted controls should be tested with eyes open or closed in studies of functional brain reorganization as a consequence of blindness.

Vernal keratoconjunctivitis (VKC) is an underdiagnosed and underrecognized ocular surface disease with limited epidemiological data in Asia. It is more prevalent in warm, dry, and windy climates, and often has a substantial impact on a patient’s quality of life. In rare cases, VKC can be associated with vision loss, either through corticosteroid overuse or inadequate treatment of persistent inflammation. As a potentially severe and complex disease, there is variability with how VKC is managed across Asia and among the various allergic eye diseases. Diagnosis and treatment of patients with VKC is a challenge for many ophthalmologists, since no precise diagnostic criteria have been established, the pathogenesis of the disease is unclear, and anti-allergic treatments are often ineffective in patients with moderate or severe disease. In addition, the choice of treatment and management strategies used for patients varies greatly from country to country and physician to physician. This may be because of a lack of well-defined, standardized guidelines. In response, the Management of Vernal Keratoconjunctivitis in Asia (MOVIA) Expert Working Group (13 experts) completed a consensus program to evaluate, review, and develop best-practice recommendations for the assessment, diagnosis, and management of VKC in Asia. The expert-led recommendations are summarized in this article and based on the currently available evidence alongside the clinical expertise of ophthalmologists from across Asia with specialism and interest in the ocular surface, VKC, and pediatric ophthalmology.

Summary: To clarify the role of sensory experience during early development for adult multisensory learning capabilities, we probed audiovisual spatial processing in human individuals who had been born blind because of dense congenital cataracts (CCs) and who subsequently had received cataract removal surgery, some not before adolescence or adulthood. Their ability to integrate audio-visual input and to recalibrate multisensory spatial representations was compared to normally sighted control participants and individuals with a history of developmental (later onset) cataracts. Results in CC individuals revealed both normal multisensory integration in audiovisual trials (ventriloquism effect) and normal recalibration of unimodal auditory localization following audiovisual discrepant exposure (ventriloquism aftereffect) as observed in the control groups. In addition, only the CC group recalibrated unimodal visual localization after audiovisual exposure. Thus, in parallel to typical multisensory integration and learning, atypical crossmodal mechanisms coexisted in CC individuals, suggesting that multisensory recalibration capabilities are defined during a sensitive period in development.

Abstract Inflammation plays a key role in the pathogenesis of retinal vascular diseases. We have shown earlier an increase in the activity of matrix metalloproteinases in the vitreous and tears of preterm born babies with retinopathy of prematurity (ROP) compared to those with no-ROP leading to a shift in the balance of angiogenic (vascular endothelial growth factor [VEGF], matrix metalloproteinase [MMPs], complement component [C3]) and anti-angiogenic (opticin, thrombospondin) in ROP eyes. We now confirmed that tear MMP levels in premature infants perfectly correlates with disease severity. Next, we demonstrated that a reduced opticin levels in ROP vitreous are regulated by MMPs secreted by activated microglia. Upon exposing the human microglia cell line (CHME3) to hypoxia, an increased expression of inflammatory proteins (MMP9, VEGF) was noticed while opticin reduced significantly (p = 0.005). Further, the reduced opticin’s expression by microglial cells under hypoxia could be rescued by inhibiting the MMP activity using doxycycline and EDTA. The inhibition of MMP activity altered the expression of other key signaling molecules under hypoxia. Our study clearly explains that increased activity of MMPs under hypoxia regulates the expression of opticin as seen in the vitreous humor of ROP and could serve as a potential target for ROP management.

Abstract Visual deprivation in childhood can lead to lifelong impairments in multisensory processing. Here, the Size-Weight Illusion (SWI) was used to test whether visuo-haptic integration recovers after early visual deprivation. Normally sighted individuals perceive larger objects to be lighter than smaller objects of the same weight. In Experiment 1, individuals treated for dense bilateral congenital cataracts (who had no patterned visual experience at birth), individuals treated for developmental cataracts (who had patterned visual experience at birth, but were visually impaired), congenitally blind individuals and normally sighted individuals had to rate the weight of manually explored cubes that differed in size (Small, Medium, Large) across two possible weights (350 g, 700 g). In Experiment 2, individuals treated for dense bilateral congenital cataracts were compared to sighted individuals in a similar task using a string set-up, which removed haptic size cues. In both experiments, indistinguishable SWI effects were observed across all groups. These results provide evidence that early aberrant vision does not interfere with the development of the SWI, and suggest a recovery of the integration of size and weight cues provided by the visual and haptic modality.

Purpose: The aim of this study was to report the proportion and patterns of eye diseases observed among infants seen at two rural eye care centers in South India. Methods: A retrospective review of case records of infants seen between January 1, 2017 and December 31, 2017 at two rural secondary eye care centers attached to L V Prasad Eye Institute, Hyderabad. Data were collected regarding their demographic profile, the pattern of eye problems observed, management at the facility itself, and need for referrals. Results: During this period, a total of 3092 children were seen. Among them, 141 were infants (4.56%, 71 boys: 70 girls, median age: 8 months). Twenty-five percent of infants were less than 6 months of age. The most common eye problem was congenital nasolacrimal duct obstruction (n = 76, 53.90%), followed by conjunctivitis (n = 33, 23.40%), retinopathy of prematurity (n = 4, 2.84%) and strabismus (n = 3, 2.13%). One case each of congenital cataract and suspected retinoblastoma were identified. Majority of the cases (58.8%) belonged to the oculoplastic and orbital surgery sub-specialty. Sixteen percent of the infants (n = 23) had sight-threatening eye problems. Twenty percent (n = 28) were referred to tertiary care hospital for further management. Conclusion: Profile of eye disease in infants in secondary or rural eye care centers ranged from simple to complex, including sight-threatening diseases. While our study concluded that nearly 4/5th of these eye problems were simple and could be managed by a well-trained comprehensive ophthalmologist, 20% of these cases required a referral to a tertiary care center.

Purpose: To report the etiology, clinical presentation, and morphology of congenital cataract in a tertiary care center. Methods: It is a prospective cohort study conducted at L V Prasad Eye Institute, Hyderabad. All children with congenital cataract ≤ 12 months of age that required surgical intervention between August 2015 and July 2016 were included in the study. 109 such patients were subjected to meticulous history taking, pedigree charting, ocular, and systemic examination, B-scan, TORCH testing, clinical photographs, pediatrician consult and blood tests, which included serum calcium, serum phosphorous and urine for reducing sugars. Results: The mean age of presentation was 4.1 months (±2.6 months) and both the genders were equally affected (P = 0.49). Eighty-five patients (77.9%) presented with bilateral cataracts while 24 patients had a unilateral presentation (22.1%). The common morphological presentation was either a total or a nuclear cataract, both variants noticed in 47 patients (43.1%). TORCH infections were responsible for a maximum (37 patients, 33.4%) number of cases followed by familial (20 patients, 18%) and developmental anomalies (11 patients, 10.1%) while the total number of idiopathic cases were 24% (27 patients). Eighteen patients (16.5%) had congenital heart defects and the majority (16 patients, 88.9%) of these had positive TORCH titres. Conclusion: Familial cataract and those possibly due to TORCH are still the predominant cause of congenital cataract in this series-highlighting the role of vaccination and preventive measures.

Pediatric optic neuritis (PON) is one of the commonest causes of acute vision loss in children. Although it might often be postinfectious or postvaccination, recent understanding and available evidence suggest that it can be the first manifestation of a neuro-inflammatory syndrome such as multiple sclerosis, neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis or myelin oligodendrocyte glycoprotein associated optic neuritis. Therefore, neuroimaging, serological testing, cerebrospinal fluid analysis, testing for various systemic autoimmune conditions become a part of the workup. However, this can be exhaustive and expensive, especially in countries with limited access to health insurance. Many recent studies suggest that neuroimaging and few clinical features can provide clues to the underlying etiology. However, serological tests can provide a confirmatory evidence. Therefore, in this mini-review, we propose a balanced approach to the evaluation of PON, based on the available literature emanating in the last decade.

Purpose: The purpose of this study is to describe the demographic profile, clinical features, visual outcomes, and follow-up patterns after successful cataract surgery in children from the tribal community in Odisha, India. Methods: We retrospectively reviewed records of tribal children aged 4 months–16 years, who underwent public health financed cataract surgery at our institute from January 1, 2015, to December 31, 2019. Collected data included demographic profile, clinical features, outcomes, and follow-up. Univariate and multivariate linear regression identified factors affecting the visual outcome at a 6-week follow-up.Results: During this period, a total of 352 children [536 eyes; mean age: 9.11 ± 4.4 years, 219 boys (62%)] underwent cataract surgery. The most common etiology and presenting complaints were idiopathic congenital cataract and decreased vision, respectively. In 304 children (86%), presenting best-corrected visual acuity (BCVA) was

Purpose: To report clinical profile, diagnostic challenges, and outcomes in cases of subacute/chronic cerebral sinus venous thrombosis (CSVT) presenting to neuro-ophthalmologists/neurologists. Methods: This was a multicentric, retrospective, observational study. Records of patients with neuroimaging proven subacute/chronic CSVT seen the from January 1, 2016 to March 31, 2020 were analyzed. Data collected included duration of symptoms, diagnosing physician, ophthalmological vs. focal/generalized neurological symptoms, optic disc examination, perimetry, and neuroimaging findings. Statistical analysis was performed using STATA software. Results: Forty-three patients with subacute (30)/chronic (13) CSVT were identified (32 males, 11 females). Median age was 37 (IQR 27–47) years. The presenting complaints were blurred vision 34 (79%), headaches in 25 (58%), vomiting 12 (28%), and diplopia 11 (26%). Eleven patients had associated sixth cranial nerve palsy. All but two patients had either disc edema/optic atrophy; four had unilateral disc edema at presentation. Ophthalmologists and neurologists diagnosed/suspected CSVT correctly in 13/29 (45%) and 11/14 (78.5%) patients, respectively. Most common initial alternate diagnosis was idiopathic intracranial hypertension in 12 (28%). Female gender, age ≤36, unilateral papilledema, not obtaining venogram at initial workup increased chances of initial alternate diagnosis. Median follow-up duration was 21 days. Average visual function remained stable in majority of patients at last follow-up. In total, 47.6% of patients had best-corrected visual acuity ≥20/30 at the final follow-up. Conclusion: In our series, subacute or chronic CSVT presented presented primarily with symptoms of intracranial hypertension. Unilateral papilledema, middle-aged patients, female gender, lack of focal/generalized neurological symptoms created diagnostic dilemma. Visual function remained stable in majority of patients.

Purpose: The objective is to analyse and report the data of teleconsultations provided to paediatric ophthalmology and strabismus patients during COVID-19 times and to elaborate our experience for guiding future teleconsultation practices to General, paediatric Ophthalmologists and Strabismologists. Methods: Retrospective analysis of electronic medical record data of teleconsultations provided in the department of Strabismus, Paediatric and Neuro-ophthalmology was done. Patients with optic nerve related disorders were excluded. Study period was one month. Statistical analysis of collected data was done using Microsoft excel. Results: A total of 198 patients were provided teleconsultations (an average of seven teleconsultations/day). The final analysis included 161 patients after excluding optic nerve related disorders. The median age was seven years. We had a near equal gender distribution (53% males and 47% females) of whom a third were new cases. Video calling was used in 14%, review of clinical photos shared was used in 53%. Rest of the 33% were given telephonic advice. Allergic conjunctivitis (14%), pseudophakia (9%), strabismus (12%), status post strabismus surgery (8%), cranial nerve palsies (11%) were common diagnoses. 19% (n = 30) were advised/needed visit in emergency services on same or next day. Conclusion: Our experience of teleconsultation during COVID-19 times for paediatric ophthalmology and strabismus patients was very encouraging. Pivoting teleconsultation platform can provide primary eye care to most of the patients and work as essential forward triage for rest. As we continue to further explore the currently available avenues in multimedia such as video conferencing and web/mobile based applications, we believe that tele-ophthalmology platform can provide a reliable service in patient care.

Background: A successful outcome in pediatric cataract surgery is determined by an intact, curvilinear anterior capsulotomy which is dependent on the type and density of cataract, the morphology of the anterior capsule, and associated anterior segment pathologies. Purpose: This video highlights 10 different techniques which can be used for capsulorhexis in pediatric cataract. Synopsis: The choice of technique for capsulorhexis in pediatric cataract is on case basis, namely the gold standard manual capsulotomy aided by rhexis forceps (1. Standard capsulorhexis/2. Vitrector, Vitrectorhexis), with an assistance from capsular staining (3. Blue-rhexis), or by coaxial illumination (4. Coaxial-rhexis) or by just the sheen of capsule (5. Sheen-rhexis). The anterior chamber can be maintained using ophthalmic visco-elastic device (Visco-rhexis) or by irrigation fluid (6. Hydro-rhexis). A speed-breaker in the routine capsulotomy is the presence of plaque which is managed by rhexis forceps (7. Plaque-rhexis) or by a vitrector (vitrectorhexis) or a pair of micro-scissors (8. Scissor rhexis). Above all, the technology of femto-second-laser-assisted (9. Femto-rhexis) and zepto-pulse-precision capsulotomy (10. Zepto-rhexis) is also illustrated. Highlights: This video highlights the 10 different techniques of capsulorhexis in pediatric cataract surgery. Video Link: https://youtu.be/TgDrk5RYdbI

Background: Ocular morbidity with an early onset can have a significant impact on the long-term development of an individual. Hence, careful assessment of visual functions early on is very important. However, testing infants always poses a challenge. Standard tools to assess infants' visual acuity, ocular motility, and so on rely on the clinician's quick subjective judgments of an infant's looking behavior. Eye movements are usually observed from head rotations or spontaneous eye movements in infants. Judging eye movements in the presence of strabismus is even more challenging. Purpose: This video shows a 4-month-old infant's viewing behavior captured during a visual field screening study. The recorded video aided in the examination of this infant that was referred to a tertiary eye care clinic. The additional information captured through the perimeter testing is discussed. Synopsis: The Pediatric Perimeter device was developed to address visual field extent and gaze reaction time assessment in the pediatric population. As a part of a large-scale screening study, infants' visual fields were tested. During this screening, a 4-month-old infant presented with a ptosis in the left eye. The infant was consistently missing the light stimuli presented in the left upper quadrant in the binocular visual field testing. The infant was referred to a tertiary eye care center to a pediatric ophthalmologist for further examination. During clinical examination, the infant was suspected to either have congenital ptosis or monocular elevation deficit. But the diagnosis of the eye condition was unsure owing to the poor cooperation of the infant. With the aid of Pediatric Perimeter, the ocular motility was consistent with limitation of elevation in abduction, indicating a possible monocular elevation deficit with congenital ptosis. The infant was also noted to have Marcus Gunn jaw-winking phenomenon. The parents were assured and asked for a review in 3 months. In the subsequent follow-up, the Pediatric Perimeter testing was performed, and the recording showed a full extraocular motility in both eyes. Hence, the diagnosis was changed to only congenital ptosis. The probable explanation for missing the target in the left upper quadrant in the first visit is postulated further. The left upper quadrant is the superotemporal visual field of the left eye and the superonasal visual field of the right eye. As the left eye had ptosis, the superotemporal visual field could have been obstructed and hence the stimuli missed. The normative extent for the nasal and superior visual field is just about 30° for a 4-month-old infant. Hence, the right eye also perhaps missed the stimuli in its superonasal visual field extent. This video highlights the utility of the Pediatric Perimeter device in providing a magnified view of the infant's face along with greater visibility of ocular features from the infrared video imaging. This can potentially help the clinician to easily observe different ocular/facial abnormalities such as extraocular motility disorders, lid functions, and in identifying unequal pupil size, media opacities, and nystagmus. Highlights: The presence of congenital ptosis in younger infants might predispose as superior visual field defect and could also masquerade as a limitation in elevation. Video link: https://youtu.be/Lk8jSvS3thE

Hyperphosphatemia in the absence of renal failure is an unusual occurrence, particularly in children, but is a common primary feature of familial hyperphosphatemic tumor calcinosis. We report a child with hyperphosphatemia who presented with multiple episodes of neurologic dysfunction involving lower motor neuron facial nerve palsy along with sequential visual loss. He also had an episode of stroke. There was an extensive metastatic calcification of soft tissue and vasculature. Hyperphosphatemia with normal serum alkaline phosphatase, calcium, parathyroid hormone, and renal function was noted. He was managed with hemodialysis and sevelamer (3 months) without much success in reducing serum phosphate level, requiring continuous ambulatory peritoneal dialysis (3 years). Intact fibroblast growth factor 23 (FGF23) was undetectable, with C-terminal FGF23 fragments significantly elevated (2575 RU/ml, normal A (p.N162K) mutation in FGF23 exon 3, confirming the diagnoses of primary FGF23 deficiency, the first case to be reported from India.

Early presentation for childhood cataract surgery is an important first step in preventing related visual impairment and blindness. In the absence of neonatal eye screening programmes in developing countries, the early identification of childhood cataract remains a major challenge. The primary aim of this study was to identify potential barriers to accessing childhood cataract services from the perspective of parents and carers, as a critical step towards increasing the timely uptake of cataract surgery. In-depth interviews were conducted using a pre-designed topic guide developed for this study to seek the views of parents and carers in nine geographic locations across eight states in India regarding their perceived barriers and enablers to accessing childhood cataract services. A total of 35 in-depth interviews were conducted including 30 at the hospital premises and 5 in the participants’ homes. All interviews were conducted in the local language and audio taped for further transcription and analysis. Data were organised using NVivo 11 and a thematic analysis was conducted utilising the Theoretical Domains Framework (TDF), an integrative framework of behavioural theories. The themes identified from interviews related to 11 out of 12 TDF domains. TDF domains associated with barriers included: ‘Environmental context and resources’, ‘Beliefs about consequences’ and ‘Social influences’. Reported enablers were identified in three theoretical domains: ‘Social influences’, ‘Beliefs about consequences’ and ‘Motivations and goals’. This comprehensive TDF approach enabled us to understand parents’ perceived barriers and enablers to accessing childhood cataract services, which could be targeted in future interventions to improve timely uptake.

Background: Untreated congenital blindness through cataracts leads to lasting visual brain system changes, including substantial alterations of extrastriate visual areas. Consequently, late-treated individuals (> 5 months of age) with dense congenital bilateral cataracts (CC) exhibit poorer visual function recovery compared to individuals with bilateral developmental cataracts (DC). Reliable methods to differentiate between patients with congenital and developmental cataracts are often lacking, impeding efficient rehabilitation management and introducing confounds in clinical and basic research on recovery prognosis and optimal timing of surgery. A persistent reduction of the P1 wave of visual event-related potentials (VERPs), associated with extrastriate visual cortical activity, has been reported in CC but not in DC individuals. Using two experiments, this study developed and validated P1-based biomarkers for diagnosing a history of congenital blindness in cataract-reversal individuals. Methods: Congenital and developmental cataract-reversal individuals as well as typically sighted matched controls took part in a first experiment used for exploring an electrophysiological biomarker (NCC = 13, NDC = 13, NControl = 26). Circular stimuli containing gratings were presented in one of the visual field quadrants while visual event-related potentials (VERPs) were recorded. Two biomarkers were derived from the P1 wave of the VERP: (1) The mean of the normalized P1 amplitude at posterior electrodes, and (2) a classifier obtained from a linear support vector machine (SVM). A second experiment with partially new CC/DC individuals and their matched controls (NCC = 14, NDC = 15, NControl = 29) was consecutively used to validate the classification based on both biomarkers. Performance of the classifiers were evaluated using receiver operating characteristic (ROC) curve analyses. All cataract-reversal individuals were tested after at least one year of vision recovery. Findings: The normalized P1 amplitude over posterior electrodes allowed a successful classification of the CC from the DC individuals and typically sighted controls (area under ROC curve, AUC = 0.803 and 0.929 for the normalized P1 amplitude and the SVM-based biomarker, respectively). The validation for both biomarkers in experiment 2 again resulted in a high classification success (AUC = 0.800 and 0.883, respectively for the normalized P1 amplitude and the SVM-based biomarker). In the most conservative scenario involving classification of CC from DC individuals in a group of only cataract-reversal individuals, excluding typically sighted controls, the SVM-based biomarker was found to be superior to the mean P1 amplitude based biomarker (AUC = 0.852 compared to 0.757 for the mean P1 based biomarker in validation). Minimum specificity obtained was 80% across all biomarkers. Interpretation: A persistent reduction of the P1 wave provides a highly specific method for classifying cataract patients post-surgically as having suffered from bilateral congenital vs. bilateral developmental cataracts. We suggest that using the P1 based non-invasive electrophysiological biomarker will augment existing clinical classification criteria for individuals with a history of bilateral congenital cataracts, aiding clinical and basic research, recovery prognosis, and rehabilitation efforts. Funding: German Research Foundation (DFG) and the European Research Council (ERC).

Typical human perception features stable biases such as perceiving visual events as later than synchronous auditory events. The origin of such perceptual biases is unknown. To investigate the role of early sensory experience, we tested whether a congenital, transient loss of pattern vision, caused by bilateral dense cataracts, has sustained effects on audio-visual and tactile-visual temporal biases and resolution. Participants judged the temporal order of successively presented, spatially separated events within and across modalities. Individuals with reversed congenital cataracts showed a bias towards perceiving visual stimuli as occurring earlier than auditory (Expt. 1) and tactile (Expt. 2) stimuli. This finding stood in stark contrast to normally sighted controls and sight-recovery individuals who had developed cataracts later in childhood: both groups exhibited the typical bias of perceiving vision as delayed compared to audition. These findings provide strong evidence that cross-modal temporal biases depend on sensory experience during an early sensitive period.

Purpose: To study the long-term safety profile and visual outcomes of primary intraocular lens (IOL) implantation in infants

Purpose: The purpose of this study is to detect the optic nerve head (ONH) and peripapillary perfusion in eyes with acute nonarteritic anterior ischemic optic neuropathy (NAION) compared to the fellow normal eyes using optical coherence tomography angiography (OCTA) and to compare with nonischemic disc edema (papilledema). Methods: Retrospective analysis of patients with unilateral NAION who underwent OCTA was performed. All patients underwent comprehensive ocular examination including visual field testing. ONH was imaged using 6 mm × 6 mm scan by Topcon DRI Triton® OCT system. Vascularity loss was analyzed using ImageJ software in diseased eyes in comparison to normal fellow eyes and eyes with papilledema. Results: Twenty-one patients (15 males, 6 females) with unilateral NAION and 9 patients (18 eyes) with papilledema were included in the study. In eyes with NAION, two distinct patterns of loss of vasculature were noted – (a) diffuse loss of microvasculature cuff and vascular network around the optic disc in all the patients (100%) and (b) additional area of sectoral loss of vasculature extending from the disc in 12 of the 21 eyes (57.14%). All 18 eyes with papilledema showed loss of the microvasculature cuff; however, none showed the focal pattern of vascular defect. The mean area of the peripapillary vascular zone in eyes with NAION was significantly lesser than that in normals. Of the 12 eyes with NAION with focal loss of vasculature, 11 correlated with visual field defects (91.6%). Conclusion: Deficient peripapillary choroidal vasculature is present in NAION and has a different pattern than in nonischemic disc edema and can cause corresponding visual field deficits.

Cyclic esotropia, a rare condition of obscure etiology characterized by regularly alternating periods of esotropia and orthophoria. We present a case of a 7-year-old boy who underwent surgical correction for intermittent exotropia elsewhere and developed esotropia with cyclic pattern post-operatively. Initially the cycle was of half-day orthophoria and half-day esotropia, which later became one full day of esotropia and one day of orthophoria. During re-surgery, right lateral rectus was found to have a stretched scar which was resected and then advanced and medial rectus was recessed. Post-surgery the child was orthophoric without cyclic pattern.

Retinopathy of prematurity (ROP) is a neurovascular complication in preterm babies, leading to severe visual impairment, but the underlying mechanisms are yet unclear. The present study aimed at unraveling the molecular mechanisms underlying the pathogenesis of ROP. A comprehensive screening of candidate genes in preterms with ROP (n = 189) and no-ROP (n = 167) was undertaken to identify variants conferring disease susceptibility. Allele and genotype frequencies, linkage disequilibrium and haplotypes were analyzed to identify the ROP-associated variants. Variants in CFH (p = 2.94 × 10−7), CFB (p = 1.71 × 10−5), FBLN5 (p = 9.2 × 10−4), CETP (p = 2.99 × 10−5), and CXCR4 (p = 1.32 × 10−8) genes exhibited significant associations with ROP. Further, a quantitative assessment of 27 candidate proteins and cytokines in the vitreous and tear samples of babies with severe ROP (n = 30) and congenital cataract (n = 30) was undertaken by multiplex bead arrays and further validated by western blotting and zymography. Significant elevation and activation of MMP9 (p = 0.038), CFH (p = 2.24 × 10−5), C3 (p = 0.05), C4 (p = 0.001), IL-1ra (p = 0.0019), vascular endothelial growth factor (VEGF) (p = 0.0027), and G-CSF (p = 0.0099) proteins were observed in the vitreous of ROP babies suggesting an increased inflammation under hypoxic condition. Along with inflammatory markers, activated macrophage/microglia were also detected in the vitreous of ROP babies that secreted complement component C3, VEGF, IL-1ra, and MMP-9 under hypoxic stress in a cell culture model. Increased expression of the inflammatory markers like the IL-1ra (p = 0.014), MMP2 (p = 0.0085), and MMP-9 (p = 0.03) in the tears of babies at different stages of ROP further demonstrated their potential role in disease progression. Based on these findings, we conclude that increased complement activation in the retina/vitreous in turn activated microglia leading to increased inflammation. A quantitative assessment of inflammatory markers in tears could help in early prediction of ROP progression and facilitate effective management of the disease, thereby preventing visual impairment.

Background: Optic nerve atrophy is an important ophthalmological sign that may be associated with serious systemic conditions having a significant bearing on the overall morbidity of the child. Studies specific to etiology of childhood optic atrophy are scarce, this being the first such study from India to the best of our knowledge. Aim: The aim was to analyze the clinical features and etiology of diagnosed cases of optic nerve atrophy in children

Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypoplasia or optic disc cupping and affection of the posterior visual pathway leads to cortical visual impairment (CVI). Other ocular associations include strabismus, nystagmus, and ocular motor abnormalities such as tonic down gaze and defective saccades and pursuits. Cortical and subcortical involvement also manifests as defects in functional vision and these have not yet been completely understood. Children with CVI may have visual field defects, photophobia, defective visual processing, and deficient color vision. Since most of these children also suffer from additional systemic disabilities, evaluation, and management remains a challenge. However, early diagnosis and initiation of rehabilitation therapy can prove to be of significant benefit in these children.

A 2-year 7-month-old girl born out of a consanguineous marriage, presented at our facility with clinical features characterized by the eyelid triad of blepharophimosis, blepharoptosis and epicanthus inversus in association with hypertelorism, cleft palate and craniosynostosis. This constellation of features is suggestive of Michels syndrome. At the time of writing this report, there were only ten reported cases worldwide and to the best of our knowledge, there have been no published reports from India.

Purpose: To evaluate the outcome of strabismus surgery for congenital superior oblique palsy (SOP) in relation to correction of head tilt and hypertropia. The cohort of patients mainly involved very young children. This is the first study to use a standardized instrument to objectively measure torticollis before and after surgery. Materials and Methods: A non-comparative interventional case series of 13 cases of congenital superior oblique palsy with head tilt, who underwent simultaneous superior oblique tuck and inferior oblique recession between Jan 2000 and Dec 2008, were studied. Results: The mean duration of SOP until surgery was 36.8 months. Of the 12 unilateral cases, 8 were right-sided. Mean follow-up period was 17 months (range 7-36). The outcome was determined at the last follow-up. Mean pre-and post-operative hypertropia (p.d.) in forced primary position was 19 ± 7 and 2 ± 6, respectively ( P < 0.0001). The head tilt reduced from mean of 17 ± 9 to 2 ± 2 degrees ( P < 0.0001). Success, defined as hypertropia

Neurofibromatosis Type I if associated with aplasia of greater wing of sphenoid may be associated with a pulsatile exophthalmos. However, very rarely it may be associated with a pulsatile enophthalmos. This clinical image describes a rare presentation with pulsatile enophthalmos, esotropia and kinking of the optic nerve due to neurofibomatosis type I.

Ophthalmic artery occlusion usually presents as a sudden onset profound decrease in vision in the middle-aged and elderly patients following periocular procedures (retrobulbar injection/glabellar fat injection), embolism from the heart or after prolonged systemic surgery. In this report, we describe three children with spontaneous ophthalmic artery occlusion who presented with unilateral loss of vision and diagnosed elsewhere as optic atrophy whose detailed history and examination were suggestive of ophthalmic artery occlusion. Detailed systemic and laboratory evaluation revealed hyperhomocysteinemia as the only potential risk factor. To the best of our knowledge, this is the first report of the association of hyperhomocysteinemia and spontaneous ophthalmic artery occlusion.

Background: The management of Duane retraction syndrome (DRS) is challenging and may become more difficult if an associated accommodative component due to high hyperopia is present. The purpose of this study is to review clinical features and outcomes in patients with partially accommodative esotropia and DRS. Setting and Design: Retrospective, non-comparative case series. Materials and Methods: Six cases of DRS with high hyperopia were reviewed. Results: Of the patients studied, the mean age of presentation was 1.3 years (range: 0.5-2.5 years). The mean amount of hyperopia was + 5D (range: 3.50-8.50) in both eyes. The mean follow up period was 7 years (range: 4 months-12 years). Five cases were unilateral while one was bilateral. Four cases underwent vertical rectus muscle transposition (VRT) and one had medial rectus recession prior to presentation; all were given optical correction. Two (50%) of the four patients who underwent vertical rectus transposition cases developed consecutive exotropia, one of whom did not have spectacles prescribed pre-operatively. All other cases (four) had minimal residual esotropia and face turn at the last follow-up with spectacle correction. Conclusion: Patients with Duane syndrome can have an accommodative component to their esotropia, which is crucial to detect and correct prior to surgery to decrease the risk of long-term over-correction. Occasionally, torticollis in Duane syndrome can be satisfactorily corrected with spectacles alone.