Visual Axis Opacity after Intraocular Lens Implantation in Children in the First 2 Years of Life: Findings from the IoLunder2 Cohort Study

Objective/ Purpose: Appropriate correction of aphakia is key to good outcomes. There may be clinical settings and populations where accessing or managing aphakic contact lenses is challenging. Strategies to target the increased risk of visual axis opacity (VAO) following primary IoL implantation in infancy are necessary. We describe the predictors of VAO following primary IoL implantation for unilateral or bilateral congenital or infantile cataract in children aged under 2 years. / Design: Population based (UK and Ireland) prospective inception cohort study undertaken through a national clinical network. / Participants: 105 children (57 bilateral cataract, 48 unilateral, total 162 eyes) undergoing primary IoL implantation in the first two years of life between January 2009 and December 2010. / Methods: Observational longitudinal study with multilevel, multivariable modelling to investigate associations between outcome of interest, and child and treatment specific factors including age, axial length, socioeconomic status, IoL model, and post operative steroid use. / Main outcome measures: Post operative proliferative and / or inflammatory visual axis opacity (VAO) requiring surgical correction. / Results: Visual axis opacity occurred in 67 eyes (45%), typically within the first post-operative year. Use of a three piece IoL model (odds ratio/OR 0.3, 95% confidence interval/CI 0.09 – 0.99, p=0.03), and increasing age at surgery (OR 0.97, 95% CI 0.95-0.99, p=0.02), were each independently protective against the development of proliferative VAO. Inflammatory VAO was independently associated with socioeconomic deprivation (OR 5.39, 95%CI 1.46 – 19.89, p=0.01). / Conclusions: Visual axis opacification is common following IoL implantation in early childhood. The findings of this prospective cohort study suggest that the use of three piece IoL models may reduce the risk of pseudophakic VAO in children aged under 2 years

Epidemiology of blindness in children

An estimated 1.4 million of the world’s children are blind. A blind child is more likely to live in socioeconomic deprivation, to be more frequently hospitalised during childhood and to die in childhood than a child not living with blindness. This update of a previous review on childhood visual impairment focuses on emerging therapies for children with severe visual disability (severe visual impairment and blindness or SVI/BL). For children in higher income countries, cerebral visual impairment and optic nerve anomalies remain the most common causes of SVI/BL, while retinopathy of prematurity (ROP) and cataract are now the most common avoidable causes. The constellation of causes of childhood blindness in lower income settings is shifting from infective and nutritional corneal opacities and congenital anomalies to more resemble the patterns seen in higher income settings. Improvements in maternal and neonatal health and investment in and maintenance of national ophthalmic care infrastructure are the key to reducing the burden of avoidable blindness. New therapeutic targets are emerging for childhood visual disorders, although the safety and efficacy of novel therapies for diseases such as ROP or retinal dystrophies are not yet clear. Population-based epidemiological research, particularly on cerebral visual impairment and optic nerve hypoplasia, is needed in order to improve understanding of risk factors and to inform and support the development of novel therapies for disorders currently considered ‘untreatable’

Visual impairment, severe visual impairment, and blindness in children in Britain (BCVIS2): a national observational study

Background: The WHO VISION 2020 global initiative against blindness, launched in 2000, prioritised childhood visual disability by aiming to end avoidable childhood blindness by 2020. However, progress has been hampered by the global paucity of epidemiological data concerning childhood visual disability. The British Childhood Visual Impairment and Blindness Study 2 (BCVIS2) was done to address this evidence gap. Methods: BCVIS2 was a prospective UK-wide, cross-sectional, observational study to establish an inception cohort of children newly diagnosed with visual impairment. Ophthalmologists and paediatricians reported cases from 89 hospitals and community centres across the UK. We included children aged 18 years or younger who were newly diagnosed with any condition causing impaired visual acuity to a level of 0·5 logMAR or worse (worse than 6/18 Snellen) in each eye, or equivalent vision as assessed by standard qualitative measures, between Oct 1, 2015, and Nov 1, 2016. Eligible children were notified simultaneously but independently by their managing ophthalmologists and paediatricians via the two national active surveillance schemes, the British Ophthalmological Surveillance Unit and the British Paediatric Surveillance Unit. Standardised detailed demographic, socioeconomic, and clinical data about detection, management, and treatment were collected at diagnosis and 1 year later. We calculated incidence estimates and relative rates by key sociodemographic factors. We did descriptive analyses of underlying ophthalmic disorders and non-ophthalmic comorbidities. Findings: 61 (7%) of 845 eligible children initially notified were ineligible at follow-up because of improved vision after treatment. Thus, the study sample comprised 784 children with permanent newly-diagnosed all-cause visual impairment, severe visual impairment, or blindness. 559 (72%) of 778 children had clinically significant non-ophthalmic impairments or conditions. 28 (4%) of 784 children died within a year after diagnosis of visual disability (all had underlying systemic disorders). Incidence of visual disability in the first year of life was 5·19 per 10 000 children (95% CI 4·71–5·72), almost ten times higher than among 1-to-4-year-olds and between 20 times and 100 times higher than in the older age groups. The overall cumulative incidence (or lifetime risk) of visual impairment, severe visual impairment, or blindness was 10·03 per 10 000 children (9·35–10·76). Incidence rates were higher for those from any ethnic minority group, the lowest quintile of socioeconomic status, and those born preterm or with low birthweight. 345 (44%) of 784 children had a single affected anatomical site. Disorders of the brain and visual pathways affected 378 (48%) of 784 children. Interpretation: BCVIS2 provides a contemporary snapshot of the heterogeneity, multi-morbidity, and vulnerability associated with childhood visual disability in a high-income country. These findings could facilitate developing and delivering health care and planning of interventional research. Our findings highlight the importance of including childhood visual disability as a sentinel event and metric in global child health initiatives. Funding: Fight for Sight, National Institute for Health Research, and Ulverscroft Foundation

Paediatric autoimmune and autoinflammatory conditions associated with uveitis

Childhood uveitis comprises a collection of heterogenous ocular phenotypes which are associated with a diverse range of childhood autoimmune and autoinflammatory disorders. Of these genetic and/or acquired disorders, juvenile idiopathic arthritis is the most common, affecting 30-80% of children with uveitis. Up to a third of children with uveitis have ‘isolated’ idiopathic disease and do not have an associated systemic disease which manifests in childhood. However, uveitis may be the presenting manifestation of disease; thus, the apparently well child who presents with uveitis may have isolated idiopathic disease, but they may have an evolving systemic disorder. The diagnosis of most of the associated disorders is reliant on clinical features rather than serological or genetic investigations, necessitating detailed medical history taking and systemic examination. Adequate control of inflammation is key to good visual outcomes, and multidisciplinary care is key to good broader health outcomes

Glaucoma following cataract surgery in the first 2 years of life: frequency, risk factors and outcomes from IoLunder2

BACKGROUND: We investigated glaucoma related adverse events, predictors and impact at 5 years following surgery in the IoLunder2 cohort // METHODS: Population based observational cohort study of children undergoing cataract surgery aged 2 years or under between January 2009 and December 2010. Glaucoma was defined using internationally accepted taxonomies based on the consequences of elevated intraocular pressure (IOP). Glaucoma related adverse events were any involving elevated IOP. Multivariable analysis was undertaken to investigate potential predictors of secondary glaucoma with adjustment for within-child correlation in bilateral cataract. Unilateral and bilateral cataract were analysed separately. // RESULTS: Complete follow-up data were available for 235 of 254, 93% of the inception cohort. By 5 years after primary cataract surgery, 20% of children with bilateral cataract and 12% with unilateral had developed secondary glaucoma. Glaucoma related complications had been diagnosed in 24% and 36% of children, respectively. Independent predictors of glaucoma were younger age at surgery (adjusted OR for reduction of week in age: 1.1, 95%C I 1.1 to 1.2, p<0.001); the presence of significant ocular comorbidity (adj OR 3.2, 95% CI 1.1 to 9.6, p=0.01); and shorter axial length (adj OR for each mm 1.7, 95% CI 10.0 to 1, p=0.05) for bilateral cataract. Shorter axial length was the single independent factor in unilateral disease (adj OR 9.6, 95% CI 1.7 to 52, p=0.009) // CONCLUSIONS: Both younger age at surgery (the strongest marker of ocular 'immaturity') and smaller ocular size (a marker of both immaturity and developmental vulnerability) can be used to identify those at greatest risk of glaucoma due to early life cataract surgery

Impaired vision and physical activity in childhood and adolescence: findings from the Millennium Cohort Study

Background/aims: Investigate if impaired vision is associated with reduced levels and differences in types of physical activity (PA) to identify barriers or enablers to achieving healthy PA levels. / Methods: Data from the Millennium Cohort Study of children born in the UK in 2000–2001 and followed-up to age 14 years (n=11 571). Using parental report on eye conditions coded by clinicians, children were categorised as having no, unilateral or bilateral impaired vision. Outcomes included objective accelerometer-derived time spent in moderate-to-vigorous physical activity (MVPA), and 16 PA types reported by parents, teachers and/or participants, covering physical education (PE), organised sports, self-organised sports and hobbies. / Results: Overall, 50% of 7-year-olds and subsequently 41% as 14-year-olds achieved the internationally recommended level of ≥60 MVPA min/day, irrespective of vision status, and mainly attributable to PE and organised sports. Bilateral impaired vision (vs none) was associated with parent-reported difficulties with PE (adjusted OR, 4.67; 95% CI, 2.31 to 9.41), self-rated poor ability in PE (3.21; 1.44 to 7.15) and not enjoy indoor PA (0.48; 0.26 to 0.88). Unilateral impaired vision was associated with both parent-rated difficulties (1.80; 1.26 to 2.59) and teachers’ perception of low ability in PE (2.27; 1.57 to 3.28), and reduced odds of high participation in organised sports (0.77; 0.59 to 0.99). Age-related trajectories showed suboptimal PA in childhood tracked into adolescence, with no difference by vision status. / Conclusion: Population-wide programmes to increase PA levels in children should pay special attention to those with impaired vision and include early interventions to encourage participation and confidence in PE and organised sports, starting in primary school and maintained afterwards

Long Term Outcomes of Pediatric Idiopathic Intermediate Uveitis

Purpose: To describe the course of childhood-onset intermediate uveitis without associated systemic disease, and investigate determinants of outcomes. / Design: A retrospective clinical cohort study. / Setting: Institutional. / Patients: 125 children (221 eyes) (aged 16 years and under). / Main Outcomes and Measures: Outcomes of interest were visual acuity, severity of inflammation, and the occurrence of sight-threatening complications. Variables examined included age and clinical findings at presentation, treatment, and duration of follow-up. Multivariable analysis was undertaken to investigate potential predictors of outcomes. / Results: Median follow-up duration was 57 months. At presentation, best-corrected visual acuity worse than 20/160 was recorded in 11 (4.4%) eyes, and significant vitreous haze (≥2+SUN) in 35 (14%) eyes. Corticosteroid-sparing agents were used in 41 children (33%), with methotrexate most commonly used (27 children, 21.6%). The most frequent complications were raised intraocular pressure n=65 (29.4%), cataract n=41 (18.5%), and cystoid macular edema n=29 (13.1%). At the last visit, 116 (92.8%) patients achieved best-corrected vision of 20/40 or better with quiescent uveitis. The absence of the use of a steroid-sparing immunomodulatory agent was the strongest predictive factor for the development of new macular edema (OR 6.3, 95% CI 2.3 – 16.9, p<0.001) or glaucoma (OR, 6.6, 95% CI 2.5 – 17.9, p<0.001) over the period of observation. / Conclusions: The visual outcomes of childhood-onset idiopathic intermediate uveitis are favorable. The frequency of sight-threatening sequelae of inflammation, which confer a life-long risk of further visual loss, is high. The use of immunomodulatory therapy is associated with a lower risk of developing macular edema and ocular hypertension

Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

PURPOSE: To describe the frequency, characteristics, and treatment outcome of persistent fetal vasculature (PFV) in children undergoing surgery for congenital and infantile cataract in the first 2 years of life. PATIENTS AND METHODS: Observational population-based cohort study with case identification through active surveillance and standardised data collection via a national clinical network, the British Isles Congenital Cataract Interest Group (BCCIG). RESULTS: The IoLunder2 cohort comprises 246 children undergoing surgery for bilateral and unilateral congenital and infantile cataract in the first 2 years of life. A total of 58/246 (24%) children had PFV (%): overall, 46/95 (46%) with unilateral cataract, and 12/141 (8%) with bilateral disease. Anterior segment vascular remnants were more common in bilateral than unilateral disease (75 vs 11%, P=0.01). At 1 year after surgery, 20% of children with bilateral PFV and 24% with unilateral had achieved normal vision for age within the operated eye. The prevalence of post-operative glaucoma was 9% (of children with bilateral disease) and 4% (unilateral). CONCLUSION: PFV is significantly more common than previously reported, and outcomes are comparable to that for congenital and infantile cataract overall