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We describe an investigation of temporal trends in the incidence of childhood uveitis-related hospital admissions, hospitalization being a robust indicator of disease severity. A population-based retrospective study using a hospitalization database, the Hospital Episode Statistics (HES) dataset, capturing data on care between 2013 and 2020. Primary outcomes were rates of pediatric hospital admissions for uveitis-related disorders. During the study period, there were 3,258 reported uveitis-related hospital admissions of children aged 0 to 14 years, comprising 19% of all-age uveitis-related admissions. Anterior uveitis was the most common diagnosis. The annual incidence of childhood uveitis-related hospitalizations declined year on year from a peak incidence of 5.4 per 100,000 children (95% confidence interval 5.0–5.9) in 2015–2016 to 3.3 per 100,000 (95% CI 3.0–3.6) in 2019–2020. Over the same period, the national incidence of juvenile arthropathy-related admissions stayed stable. The decline in admissions nationally may reflect reduced incidence of uveitis complications with increasing use of immunosuppressive therapies. [ABSTRACT FROM AUTHOR]

Objective: Children with juvenile idiopathic arthritis (JIA) need regular examinations for uveitis to avoid visual morbidity from the most common extraarticular manifestation of disease. This study was undertaken to investigate the feasibility, acceptability, and performance of optical coherence tomography (OCT) imaging‐based diagnosis of uveitis. Methods: This observational cross‐sectional study included children with and those without uveitis. The children underwent routine clinical examinations and anterior segment OCT scanning of intraocular inflammatory cells. Acceptability of image acquisition was assessed using a visual analog scale and length of time needed to acquire images. Interobserver and intraobserver variability of manual counting of acquired images (Bland‐Altman limits of agreement), correlation between imaging and routine assessment, and sensitivity and specificity of anterior segment OCT detection of active inflammation were assessed. Results: Of the 26 children ages 3–15 years (median age 8 years) who underwent imaging, 12 had active inflammation. All patients rated the acceptability of image acquisition as at least 8.5 on a scale of 0–10. Time taken to acquire images ranged from 1.5 minutes to 22 minutes (median time 8 minutes). There was good positive correlation between clinical assessment and image‐based cell quantification (R2 = 0.63, P = 0.002). Sensitivity of anterior segment OCT manual image cell count for diagnosis of active inflammation was 92% (95% confidence interval [95% CI] 62–99%), specificity was 86% (95% CI 58–98%), and negative predictive value (ruling out uveitis) was 92% (95% CI 65–99%). Conclusion: Non‐contact, high‐resolution imaging for JIA uveitis surveillance is feasible, acceptable to patients, and holds the promise of transforming pediatric practice. Further work is needed to determine the analytic and clinical validity of anterior segment OCT quantification of active inflammation, and the clinical utility and cost‐effectiveness of imaging‐based disease monitoring. [ABSTRACT FROM AUTHOR]

Purpose: Visual fields are key functional outcome measures in children with a variety of ophthalmologic disorders. However, reliably assessing fields in children is challenging. We report the findings of a survey of current practices of perimetry in children in the United Kingdom and Ireland. Methods: An electronic questionnaire was sent to Orthoptic Service Heads in July 2008. Respondents were asked for comments regarding visual field testing in children as well as details of the volume and type of perimetry performed in their units, over a 1-year period. Results: Of the 98 (62%) completed questionnaires, 16 departments reported not testing visual fields in children. In total 3675 subjects under 16 years of age were reported to have undergone perimetry in 1 year, most in units with a ≥ 50% pediatric caseload for orthoptics. A total of 42% of units used static perimetry alone, 11% kinetic, and 47% used a combination of both. Conclusion: High numbers of visual field tests are carried out in children in the UK and Ireland annually. Automated perimetry is used predominantly, despite the underlying algorithms having been developed for adult populations. Thus there is a clear need for more research, to ensure that evolving management practices are informed by understanding of the diagnostic accuracy and value of perimetry in children. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the hypothesis that the excessive growth of the eye in myopia is associated with general growth and thus influenced by early life biological and social factors, and that these associations underlie recent secular trends of increasing prevalence and severity of myopia. Design: Cohort study. Participants: A total of 2487 randomly selected 44-year-old members of the 1958 British birth cohort (27% subsample). Methods: Diverse and detailed biological, social, and lifestyle data have been collected by following members since birth through a series of clinical examinations or face-to-face interviews carried out by trained examiners. At 44 years, cohort members underwent autorefraction using the Nikon Retinomax 2 (Nikon Corp., Tokyo, Japan) under non-cycloplegic conditions. A lifecourse epidemiologic approach, based on 4 sequential multivariable “life stage” models (preconceptional; prenatal, perinatal, and postnatal; childhood; and adult), was used to examine the influence of early life biological, social and lifestyle factors, growth patterns, and “eye-specific” factors on myopia. Main Outcome Measures: Myopia severity (all, mild/moderate: spherical equivalent −0.75 to −5.99 diopters [D]; severe: ≥−6.00 D extreme vs. emmetropia −0.74 to +0.99 D) and myopia onset (early [<16 years] vs. later). Results: A total of 1214 individuals (49%; 95% confidence interval, 48.8–50.8) were myopic (late onset in 979 [80.6%]). Myopia was positively associated with low birthweight for gestational age, gender, greater maternal age, higher paternal occupational social class, and maternal smoking in early pregnancy. Myopia was independently associated with proxy markers of near work and educational performance, with some differences by onset and severity. In adults, greater height and higher educational attainment and socioeconomic status were associated with myopia. Conclusions: Trends in the key influences on child health and growth identified as novel putative risk factors in this study are consistent with global trends of increasing myopia: increasing births to older mothers, increasing rates of intrauterine growth retardation and survival of affected children, increasing persistence of smoking in pregnancy, and changing socioeconomic status. Prospects for prevention of myopia would be improved by a paradigm shift in myopia research, with lifecourse and genetic epidemiologic approaches applied in tandem in large unselected populations. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

Objective: To describe a child-centered approach to identifying content for a novel self-report questionnaire for assessing vision-related quality of life (QoL) of visually impaired (VI) or blind (BL) children and young people. Design: Questionnaire development. Participants: A stratified random patient sample of children and young people who are VI/BL (visual acuity in the better eye Snellen <6/18; logarithm of the minimum angle of resolution <0.51) as the result of any visual disorder, but in the absence of any other significant impairment, aged 10 to 15 years (N=49); and a convenience school-based sample of children and young people who are VI/BL and aged 10 to 17 years (N=29). Methods: Individual interviews were conducted with a stratified random sample of 32 children and young people, aged 10 to 15 years, who were VI/BL. The interviews followed a topic guide based on vision-related issues identified from a focus group of affected children and young people, combined with a literature review and consultations with professionals. Collaborative qualitative thematic analysis was undertaken and used to derive draft items of the instrument, using the children''s own language wherever possible. Items were reduced, rephrased, and refined through individual consultation, as well as an expert reference group of children and young people who were VI/BL, and supplemented by the research team''s consensus. Main Outcome Measures: A draft 47-item instrument. Results: A total of 874 potential questionnaire items were initially generated spanning the following domains: social relations, acceptance, and participation; independence and autonomy; psychological and emotional well-being; future—aspirations and fears; functioning—home, school, and leisure; and treatment of eye condition. This was eventually reduced to a 47-item instrument with each item presented as a vignette describing a QoL issue from an “illustrative” child''s perspective. Thus, the responding child reports on how much he/she is presently like and how much he/she wishes to be like that child, using a 4-point Likert-type scale. Conclusions: We demonstrate that a child-centered approach to identifying the content for a self-report vision-related QoL questionnaire is feasible. We suggest this approach is critical to accurately capturing children and young peoples'' subjective perspectives on the impact of living with impaired vision. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

Purpose: To investigate how visual function in mid-adult life is associated with health and social outcomes and, using life-course epidemiology, whether it is influenced by early life biological and social factors. Design: Population-based cohort study. Participants: Nine thousand three hundred thirty members of the 1958 British birth cohort at age 44 or 45 years. Methods: Distance, near, and stereo vision were assessed as part of a broader biomedical examination. Logistic, multinomial, and proportional odds ordinal logistic regression were used, as appropriate, to assess the association between these vision functions and both key early life influences and health and social outcomes in mid-adult life. Main Outcome Measures: Distance, near, and stereo acuities and health and social outcomes. Results: In mid-adult life, vision function (across the full spectrum of both type and level of function) is associated with unemployment resulting from permanent sickness, lower socioeconomic status, and poorer general health (for example, for blindness; odds ratios were 2.5, 2.6, and 1.2, respectively). Also, impaired visual functions in mid-adult life are associated with a low birthweight, being small for gestational age, maternal smoking in pregnancy, and markers of socioeconomic deprivation in childhood (for example, for impaired distance acuity; odds ratios were 1.4, 1.3, 1.02, and 1.1, respectively). Conclusions: Although relatively uncommon in working-age adults, impaired vision can have important adverse consequences, which highlights the value of investigating visual function in the broader context of health and social functioning. In addition, visual function in adult life may be influenced directly by key prenatal and childhood biological and social determinants of general health. Thus, application of life-course epidemiology to complex chronic ophthalmic diseases of adult life such as glaucoma or macular degeneration is likely to prove valuable in elucidating whether and how biological, social, and lifestyle factors contribute to the cause. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

Purpose: To describe the prevalence of impaired vision and its relative burden, together with the prevalence of impaired vision-related quality of life (VRQOL), and investigate associations with social outcomes in a contemporary and nationally representative population of working age adults. Design: Population-based cross-sectional study. Participants: We included 9330 members of the 1958 British birth cohort at age 44 and 45 years. Methods: “Habitual” and “best achieved” distance visual acuity in each eye, binocular near vision acuity and stereoacuity (three dimensional/depth perception) were tested during a broader biomedical examination. VRQOL was assessed using the Vision-related Quality of Life Core Measure 1 (VCM1), a validated, 10-item, self-complete instrument. Logistic and proportional odds ordinal logistic regression were used to calculate odds ratios (ORs) of the association of VRQOL with visual acuities and social outcomes. Main Outcome Measures: Distance, near, and stereo acuities and VRQOL and social outcomes. Results: Of the 1.3% (124) of those with visual loss that precluded driving, a further 0.75% (70) were visually impaired or severely visually impaired and 0.15% (14) blind, the latter accounting for 19% total population (all ages) burden of blindness. Impairment of VRQOL is strongly associated with impaired distance, near, and stereo vision, as well as with adverse occupational and other social outcomes. However, VRQOL impairment is also sometimes reported with unilateral or mild bilateral visual loss. Conclusions: Although impaired vision in working age adults is relatively uncommon, it confers important adverse consequences for the “health and wealth” of the public. This may be captured best by assessment of VRQOL in addition to objective visual function. Ophthalmic disorders occurring or impacting in middle life should be given a higher priority than currently in national and international strategies against avoidable visual disability. Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. [Copyright &y& Elsevier]

Objective. Key worker programs for families of children with disabilities, to promote information provision, emotional support, and liaisons among different agencies, have long been advocated but not extensively implemented. We report the impact on the experiences of parents and the practices of health care professionals of a novel, hospital-based, key worker service (Community Link Team [CLT]), implemented in the pediatric ophthalmology department of Great Ormond Street Hospital (London, United Kingdom). Design, Setting, and Participants. The CLT included 2 members, 1 of whom was present during the first out-patient assessment by the consultant ophthalmologist of any child newly diagnosed as visually impaired (corrected acuity of 6/18 or worse in the better eye) and accompanied the family during other assessments performed during that visit. A dedicated room was used by the CLT members to spend time with each family after completion of the clinical assessments. The CLT members reiterated and/or clarified clinical information already provided, specifically advised the families about visual stimulation programs and the benefits and purpose of visual impairment certification, and provided information about educational and social services. The same CLT member met the family at subsequent visits to the department and acted as the first point of contact for parents. Parents of children newly diagnosed with visual impairment and/or ophthalmic disorders at Great Ormond Street Hospital participated in a 2-stage study to assess their needs, their views about the processes of care, and their overall satisfaction. The study included a questionnaire survey with 2 standard instruments, ie, the Measure of Processes of Care, specifically developed and used to assess parents' views of the degree to which health services for a range of childhood disorders are family-centered, and the short form of the Client Satisfaction Questionnaire, used to assess overall parental satisfacti... [ABSTRACT FROM AUTHOR]

Background Prevention of visual impairment and blindness in childhood is an international priority. However, many countries do not have contemporary information about incidence and causes, from which the scope and priorities for prevention and treatment can be identified. Methods In the UK, children aged younger than 16 years newly diagnosed with severe visual impairment or blindness SVI/BL, WHO criteria) during 2000 were identified through national active surveillance schemes in ophthalmology and paediatrics. From these data, we calculated yearly age-group specific incidence and cumulative incidence. Causes were classified by the anatomical site or sites affected and by timing of the insult or insults and causal factors, where known. Findings Of 439 newly diagnosed children, 336 (77%) had additional non-ophthalmic disorders or impairments (SVI/BL plus). Total yearly incidence was highest in the first year of life, being 4.0 (95% CI 3.6--4.5) per 10 000, with a cumulative incidence by 16 years of age of 5.9 (5.3--6.5) per 10 000. 10% (44) of all children died within 1 year of diagnosis of blindness. Prenatal causal factors affected 61% 268) of children, with perinatal or neonatal and childhood factors each affecting 18% (77). Incidence and causes varied with presence of non-ophthalmic impairments or disorders, birthweight, and ethnic orgin. At least 75% (331) of children had disorders that were neither potentially preventable nor treatable, with current knowledge. Interpretation Severe visual impairment and blindness in childhood in the UK is more common, occurs more frequently in the context of complex non-ophthalmic impairments, and has greater associated mortality, than previously assumed. An increased rate in children of low birthweight and from ethnic minority groups, together with the observed diversity and complexity of the causes, reflect recent secular changes in the population at risk and specific risk factors. [ABSTRACT FROM AUTHOR]

SUMMARY BACKGROUND Screening for amblyopia in early childhood is done in many countries to ensure that affected children are detected and treated within the critical period, and achieve a level of vision in their amblyopic eye that would be useful should they lose vision in their non-amblyopic eye later in life. We aimed to investigate the risk, causes, and outcomes of visual impairment attributable to loss of vision in the non-amblyopic eye. METHODS For 24 months from July, 1997, national surveillance was done to identify all individuals in the UK with unilateral amblyopia (acuity worse than 6/12) who had newly acquired vision loss in the non-amblyopic eye, resulting in acuity of worse than 6/12 or visual-field restriction precluding driving. Information about participants was obtained at presentation and 1 year later. Participants were categorised as having socially significant visual impairment, or visual impairment, severe visual impairment, or blindness, in accordance with WHO taxonomy. FINDINGS Of 370 eligible individuals, at presentation 104 (28%) had socially significant visual impairment, 180 (49%) visual impairment, and 86 (23%) severe visual impairment or blindness. The minimum risk of permanent visual impairment by age 95 years was 32.9 (95% CI 29.1-36.9) per 100 000 total population. The projected lifetime risk of vision loss for an individual with amblyopia was at least 1.2% (95% CI 1.1-1.4). Only 36 (35%) of 102 people previously in paid employment were able to continue. INTERPRETATION In the UK, where screening for amblyopia is under review, risk of serious vision loss affecting the non-amblyopic eye and its results are greater than that previously assumed. Thus, in addition to the benefits of improved vision in the amblyopic eye, treatment of amblyopia during childhood is a potentially valuable strategy to prevent incapacitating vision loss later in life. [ABSTRACT FROM AUTHOR]

The author discusses the primary objective of vision screening among children in Great Britain. Topics discussed include determinant of the cost-effectiveness of vision screening, visual function and acuity testing, and the conclusion of a major systematic review which revealed the absence of robust evidence of the effectiveness of amblyopia treatment. Other issues explained include the benefit of timely treatment and other screening recommendation of the National Screening Committee.

Objectives: This study aimed to investigate associations between persisting amblyopia into adulthood and its "real-life" impacts and inform the current debate about the value of childhood vision screening programs.Methods: Associations between persisting amblyopia and diverse socioeconomic, health, and well-being outcomes were investigated in multivariable-adjusted (sex, age, ethnicity, deprivation) regression models, with 126 400 participants (aged 40-70 years) of the UK Biobank with complete ophthalmic data. Analysis by age group (cohort 1, 60-70 years; cohort 2, 50-59 years; cohort 3, 40-49 years) assessed temporal trends.Results: Of 3395 (3%) participants with confirmed amblyopia, overall 77% (2627) had persisting amblyopia, declining from 78% in cohort 1 to 73% in cohort 3. The odds of persisting amblyopia were 5.91 (5.24-6.66) and 2.49 (2.21-2.81) times greater in cohort 1 and cohort 2, respectively, than cohort 3. The odds were also higher for more socioeconomically deprived groups and for white ethnicity. Reduced participation in sport, adverse general and mental health, and well-being were all independently associated with persisting amblyopia, with the strongest associations in the youngest cohorts. Associations with lower educational attainment and economic outcomes were only evident in the oldest cohort.Conclusions: There has been a decline in the overall frequency of persisting amblyopia since the introduction of universal child vision screening in the United Kingdom. Nevertheless, most adults treated for amblyopia in childhood have persisting vision deficits. There was no evidence that persisting amblyopia has vision-mediated effects on educational, employment-related, or economic outcomes. The observed adverse outcomes were largely those not directly mediated by vision. Patients undergoing treatment should be counseled about long-term outcomes. [ABSTRACT FROM AUTHOR]

Background: Childhood visual impairment (VI) has a profound impact on many aspects of childhood and adolescence. This is well-documented in cross-sectional and/or quantitative studies utilizing self-report instruments which compare children with and without VI. Young people's views on the experience of growing up with VI as a developmental, change-driven process remain largely unexplored. Methods: As part of our broader research programme on quality of life of visually impaired children and young people in the United Kingdom, in-depth, semi-structured interviews were conducted between March and June 2015, with a stratified sample of 17 young people with VI, aged 16–19 years. An age-sensitive, empirically-based topic guide encouraged retrospective reflections on participants' experiences of growing up with VI, including age-normative and vision-specific challenges. Results: Descriptions of growing up with VI largely centered on an overarching higher-order theme labelled becoming me. Four themes representing everyday activities, attitudes, preferences and perceptions in relation to i) social relationships, ii) independence and responsibilities, iii) the future, and iv) rising to challenges emerged and were used by participants in their description of three stages in which they developed a sense of self: i) laying the foundations, ii) testing the waters, and iii) this is me. Differences in manifestation of VI influenced how young people made sense of their experiences and their sense of self. Conclusions: Findings are discussed in relation to normative and vision-specific changes in psychosocial development during adolescence, including the development of identity. They highlight the need for ongoing monitoring of subjective well-being in a clinical population with a unique early life course trajectory. [ABSTRACT FROM AUTHOR]

Background: Evidence on the socioeconomic burden associated with childhood visual impairment, severe visual impairment and blindness (VI/SVI/BL) is needed to inform economic evaluations of existing and emerging interventions aimed at protecting or improving vision. This study aimed to evaluate the quantity and quality of literature on resource use and/or costs associated with childhood VI/SVI/BL disorders. Methods: PubMed, Web of Science (Ovid), the National Health Service (NHS) Economic Evaluation Database and grey literature were searched in November 2020. The PubMed search was rerun in February 2022. Original articles reporting unique estimates of resource use or cost data on conditions resulting in bilateral VI/SVI/BL were eligible for data extraction. Quality assessment (QA) was undertaken using the Drummond checklist adapted for cost-of-illness (COI) studies. Results: We identified 31 eligible articles, 27 from the peer-reviewed literature and four from the grey literature. Two reported on resource use, and 29 reported on costs. Cerebral visual impairment and optic nerve disorders were not examined in any included studies, whereas retinopathy of prematurity was the most frequently examined condition. The quality of studies varied, with economic evaluations having higher mean QA scores (82%) compared to COI studies (77%). Deficiencies in reporting were seen, particularly in the clinical definitions of conditions in economic evaluations and a lack of discounting and sensitivity analyses in COI studies. Conclusions: There is sparse literature on resource use or costs associated with childhood visual impairment disorders. The first step in addressing this important evidence gap is to ensure core visual impairment outcomes are measured in future randomised control trials of interventions as well as cohort studies and are reported as a discrete health outcome. [ABSTRACT FROM AUTHOR]

Background/Objectives: Routine use of patient-reported outcome measures (PROMs) to assess quality of health care systems is mandated in many countries and has been implemented successfully in many specialities. Ophthalmology currently lags behind. To support and inform future implementation, we investigated paediatric ophthalmic clinicians' experience of, and future training needs for, using child-appropriate vision PROMs and their views about the barriers and enablers to future routine implementation in clinical practice. Methods: We conducted a pilot study, using an online survey to elicit the experience, attitudes, training needs and perceptions of barriers and enablers to routine PROMs use of ophthalmic health professionals in the Paediatric Ophthalmology Department at Great Ormond Street Hospital, London. A focus-group was undertaken to discuss survey results and preferences regarding presentation of PROM data. Analysis comprised descriptive statistics, presented alongside complementary qualitative data. Results: Eighteen clinicians in the department completed the survey. Twenty-seven took part in the focus group. Clinicians had limited experience of using PROMs but high confidence in the potential positive impact on communication with patients, monitoring chronic conditions and clinical decision-making. Clinicians identified operational issues (collection and analysis of data) and impact (interpretation and application of data) as the two key areas for consideration. Training and information requirements before implementation were clearly articulated, alongside the benefits of using digital/electronic data capture ahead of consultations to allow efficiency and automated analysis, and presentation in an appropriate visual format alongside clinical data to ensure meaningful use. Conclusion: The findings of this pilot study of ophthalmic clinicians working in a specialist paediatric ophthalmology department, suggest that ophthalmic clinicians recognise the potential benefits of routine PROMs use in clinical practice. Together with existing literature outside ophthalmology relating to overcoming barriers and exploiting enablers to routine implementation, findings may be applicable in planning routine PROM implementation in paediatric ophthalmology. [ABSTRACT FROM AUTHOR]

Amblyopia arises from abnormal visual experiences in early childhood. Improved function of the amblyopic eye after visual loss in the non-amblyopic eye could be a model for residual neural plasticity. We aimed to establish the likelihood of, and predictive factors for, this improvement in function. We identified 254 individuals aged 11 years or older with unilateral amblyopia who were visually impaired after loss of vision in their non-amblyopic eye but had no other disorder affecting their amblyopic eye. 25 (10%) of 254 people had improved visual acuity in their amblyopic eye. These findings suggest there is some plasticity in the visual system of a few visually mature individuals with amblyopia, which warrants further study. Children should remain the focus of detection and treatment. [ABSTRACT FROM AUTHOR]

Comments on two studies to determine the effectiveness of pre-school screening for amblyopia, an eye disorder. Details of the studies conducted in Rotterdam, the Netherlands and Bristol, England; Indication that screening is more accurate at older ages and when undertaken by ophthalmic professionals; Questions about long term benefits of screening.

Editorial. Talks about the need for better research on which to base policy decisions regarding the screening of preschool children for the related conditions of amblyopia, refractive error and strabismus. Serious deficiencies in research evidence identified by the National Health Service; Main purpose of screening and surveillance for visual defects throughout childhood.

Background: Currently, about 2 per 1000 children in the industrialised world are severely visually impaired or blind (SVI/BL) due to diverse uncommon conditions that are usually present from early infancy. The impact of SVI/BL is lifelong and life-changing. Thus, children are a priority in the WHO-led global initiative against avoidable blindness. The aim of this scoping review is to assess the current evidence base on interventions to prevent or treat the major causes of childhood SVI/BL, specifically the degree of alignment between robust interventional research (RCTs) and the burden (relative frequency) of the key causative disorders, identifying gaps in the evidence base for tackling childhood blindness. Methods/design: We will perform a scoping review of the published literature of randomised controlled trials (RCTs) for clinical interventions that prevent or treat eye and vision diseases in children (<18 years old). Major electronic databases MEDLINE (PUBMED), EMBASE and the Cochrane CENTRAL will be searched to identify published trials using a comprehensive paediatric specific strategy informed by previous searches. The outcome of our study, randomised clinical trial activity, will be measured by the total number of RCTs and total paediatric participants randomised. The quantity and distribution of activity across diseases will be classified in the broad categories of anatomical site affected (per WHO taxonomy). The degree of alignment between paediatric trial activity and burden of SVI/BL disease (relative proportion) will be measured using a test of association (Spearman's correlation coefficient). Discussion: Despite the global public health importance of childhood blindness, there has been no assessment of the completeness of the evidence base regarding clinical interventions to prevent or treat the causative disorders. This scoping review will measure the degree of alignment between the published evidence and the burden of disease to identify gaps in current knowledge and consider the underlying reasons, informing clinicians, policy makers and funders about research priorities. [ABSTRACT FROM AUTHOR]

An abstract of the article "Improving Detection of Blindness in Childhood: The British Childhood Vision Impairment Study," by Jugnoo S. Rahi, Phillippa M. Cumberland and Catherine S. Peckham is presented.

Presents a reply by Jugnoo Rahi and colleagues to letters to the editor about their article "Impact of congenital colour vision deficiency on education and unintentional injuries: finding from the 1958 British birth cohort," in the November 6, 2004 issue.

Purpose: Genetic variants identified through population-based genome-wide studies are generally of high frequency, exerting their action in the central part of the refractive error spectrum. However, the power to identify associations with variants of lower minor allele frequency is greatly reduced, requiring considerable sample sizes. Here we aim to assess the impact of rare variants on genetic variation of refractive errors in a very large general population cohort. Methods: Genetic association analyses of non-cyclopaedic autorefraction calculated as mean spherical equivalent (SPHE) used whole-exome sequence genotypic information from 50,893 unrelated participants in the UK Biobank of European ancestry. Gene-based analyses tested for association with SPHE using an optimised SNP-set kernel association test (SKAT-O) restricted to rare variants (minor allele frequency < 1%) within protein-coding regions of the genome. All models were adjusted for age, sex and common lead variants within the same locus reported by previous genome-wide association studies. Potentially causal markers driving association at significant loci were elucidated using sensitivity analyses by sequentially dropping the most associated variants from gene-based analyses. Results: We found strong statistical evidence for association of SPHE with the SIX6 (p-value = 2.15 x 10−10, or Bonferroni-Corrected p = 4.41x10-06) and the CRX gene (p-value = 6.65 x 10−08, or Bonferroni-Corrected p = 0.001). The SIX6 gene codes for a transcription factor believed to be critical to the eye, retina and optic disc development and morphology, while CRX regulates photoreceptor specification and expression of over 700 genes in the retina. These novel associations suggest an important role of genes involved in eye morphogenesis in refractive error. Conclusion: The results of our study support previous research highlighting the importance of rare variants to the genetic risk of refractive error. We explain some of the origins of the genetic signals seen in GWAS but also report for the first time a completely novel association with the CRX gene. [ABSTRACT FROM AUTHOR]

Amblyopia is a neurodevelopmental disorder that affects at least 2% of most populations and can lead to permanently reduced vision if not detected and treated within a specific period in childhood. Whole-population screening of children younger than 5 years is applied in many countries. The substantial diversity in existing programmes reflects their heterogeneous implementation in the absence of the complete evidence base that is now a pre-requisite for instituting screening. The functional importance of amblyopia at an individual level is unclear as data are scarce, but in view of the high prevalence the population-level effect might be notable. Screening of all children aged 4-5 years (eg, at school entry) confers most benefit and addresses inequity in access to timely treatment. Screening at younger ages is associated with increased risk of false-positive results, and at older ages with poor outcomes for children with moderate to severe amblyopia. We suggest that the real-life adverse effects of amblyopia should be characterised and screening and diagnosis should be standardised. [ABSTRACT FROM AUTHOR]

Amblyopia is a neurodevelopmental disorder that affects at least 2% of most populations and can lead to permanently reduced vision if not detected and treated within a specific period in childhood. Whole-population screening of children younger than 5 years is applied in many countries. The substantial diversity in existing programmes reflects their heterogeneous implementation in the absence of the complete evidence base that is now a pre-requisite for instituting screening. The functional importance of amblyopia at an individual level is unclear as data are scarce, but in view of the high prevalence the population-level effect might be notable. Screening of all children aged 4–5 years (eg, at school entry) confers most benefit and addresses inequity in access to timely treatment. Screening at younger ages is associated with increased risk of false-positive results, and at older ages with poor outcomes for children with moderate to severe amblyopia. We suggest that the real-life adverse effects of amblyopia should be characterised and screening and diagnosis should be standardised. [ABSTRACT FROM AUTHOR]

As visual impairment ( VI) due to adverse drug reactions ( ADR) is rare in adults and children, there is an incomplete evidence base to inform guidance for screening and for counseling patients on the potential risks of medications. We report on suspected drugs and the eye conditions found in a national study of incidence of diagnosis of visual impairment due to suspected ADR. Case ascertainment was via the British Ophthalmological Surveillance Unit ( BOSU), between March 2010 and February 2012, with follow-up after 6 months. Case definition: any child or adult with bilateral or unilateral visual impairment due to a suspected ADR, using distance acuity worse than Snellen 6/18 (log MAR 0.48) in the better eye (bilateral) or affected eye (unilateral). Anonymized patient information on potential cases was provided by managing ophthalmologists, comprising visual status before and after suspected ADR, ophthalmic condition attributable to the ADR, preexisting eye disease and prescribed medications at the time of the ADR. Permanency and causality of the visual impairment were confirmed by the managing clinician, after 6 months, using the WHO Uppsala Monitoring Committee criteria. Over 2 years, 36 eligible cases were reported of whom 23 had permanent VI. While most cases were due to drugs known to have adverse side-effects, some were unanticipated sporadic cases. Visual impairment due to ADRs is rare. However, with for example, increasing polypharmacy in the elderly, monitoring of ocular ADRs, although challenging, is necessary. [ABSTRACT FROM AUTHOR]

Background A prospective, national population-based cross-sectional study to enable understanding of the burden and management in the UK of hereditary retinal disorders presenting in childhood. Methods Children aged <16 years with a new diagnosis of an inherited retinal disorder made between September 2006 and February 2008 in the UK were identified through two national active surveillance schemes. Clinical and socio-demographic information was collected on each child at diagnosis and 9 months later using standardised questionnaires. Results 241 patients were reported with 24 distinct diagnoses. 14% had additional systemic disorders and 13% had dual sensory impairment. Annual incidence was 1.4/100 000 children (aged 0-15 years) and the cumulative incidence by age 16 years was 22.3/100 000 children. The most common mode of inheritance was autosomal recessive. A significantly higher rate was seen in males than females (relative rate (RR) 1.53), in children of Asian compared with White ethnicity (RR 7.12) and in those in the worst quintile of socio-economic deprivation compared with those in the best (RR 1.43). Parents most commonly detected a problem with their child's vision. Up to seven different health professionals were involved in a child's early management, and variations were noted in the proportion of eligible children having assessments for low vision aids, statement of educational needs and certification as sight-impaired. Conclusions These findings illustrate the highly heterogeneous nature of childhood retinal dystrophies and provide previously unavailable data on disease incidence, distributions and management, which are important for service provision and for planning future treatment programmes, particularly as novel therapies become available. [ABSTRACT FROM AUTHOR]

This study investigated temporal trends in the epidemiology of primary myopia and associations with key environmental risk factors in a UK population. Data were collected at recruitment (non-cycloplegic autorefraction, year of birth, sex, ethnicity, highest educational attainment, reason and age of first wearing glasses and history of eye disease) from 107,442 UK Biobank study participants aged 40 to 69 years, born between 1939 and 1970. Myopia was defined as mean spherical equivalent (MSE) ≤-1 dioptre (D). Temporal changes in myopia frequency by birth cohort (5-year bands using date of birth) and associations with environmental factors were analysed, distinguishing both type (childhood-onset, <18 years versus adult-onset) and severity (three categories: low -1.00 to -2.99D, moderate -3.00 to -5.99D or high ≥-6.00D). Overall myopia frequency increased from 20.0% in the oldest cohort (births 1939–1944) to 29.2% in the youngest (1965–1970), reflecting a relatively higher increase in frequency of adult-onset and low myopia. Childhood-onset myopia peaked in participants born in 1950–54, adult-onset myopia peaked in the cohort born a decade later. The distribution of MSE only shifted for childhood-onset myopia (median: -3.8 [IQR -2.4, -5.4] to -4.4 [IQR -3.0, -6.2]). The magnitude of the association between higher educational attainment (proxy for educational intensity) and myopia overall increased over time (adjusted Odds Ratio (OR) 2.7 [2.5, 2.9] in the oldest versus 4.2 [3.3, 5.2] in the youngest cohort), being substantially greater for childhood-onset myopia (OR 3.3 [2.8, 4.0] to 8.0 [4.2, 13]). Without delineating childhood-onset from adult-onset myopia, important temporal trends would have been obscured. The differential impact of educational experience/intensity on both childhood-onset and high myopia, amplified over time, suggests a cohort effect in gene-environment interaction with potential for increasing myopia frequency if increasing childhood educational intensity is unchecked. However, historical plateauing of myopia frequency does suggest some potential for effective intervention. [ABSTRACT FROM AUTHOR]

Aim: To report the usefulness of uncorrected distance visual acuity (DVA) at 16 years to "screen" for myopia status and to assess the lifetime risk of myopia, based on a national birth cohort. Methods: 1867 members of the 1958 British birth cohort for whom there were data on acuity at 16 years had autorefraction, as part of a biomedical survey, at 45 years. Reduced uncorrected DVA at age 16 years (6/12 or worse in both eyes) was compared with adult refraction (spherical equivalent). Results: Only a quarter of individuals in the population studied who had developed myopia by 45 years of age had reduced acuity at 16 years of age. Notably, half of all adults with moderate myopia (-2.99 to -5.99) and 31% (11/35) with severe myopia (≥ -6) had good uncorrected DVA in both eyes at 16 years of age. Thus, sensitivities were low, ranging from 16% for all myopia (cut-off point spherical equivalent -0.5) to 69% for severe myopia (cut-off point spherical equivalent -6). However, a high (91%) lifetime probability of primary myopia (spherical equivalent ≥ -0.5) given a reduced uncorrected DVA at 16 years was found. Conclusion: In this population, reduced uncorrected DVA in childhood is an inaccurate and inappropriate intermediate "phenotype" for capturing adult myopia status. However, our findings support assessment of DVA in secondary school children as an effective method of identifying refractive error (both myopia and hypermetropia). [ABSTRACT FROM AUTHOR]

Developmentally sensitive measures of vision-related quality of life (VQoL) are needed to capture age-specific concerns about the impact of living with visual impairment (VI) in children and young people. Our objective was to use our validated VQoL instrument for children and young people 10 to 15 years of age (the VQoL_CYP) as the foundation for development of age-specific extensions. Questionnaire development. A representative sample of children and young people 6 to 19 years of age with VI, defined as visual acuity worse than 0.50 logarithm of the minimum angle of resolution in the better eye. They were recruited from pediatric ophthalmology clinics at Great Ormond Street Hospital and Moorfields Eye Hospital and, in the final phase of the study, from 20 additional United Kingdom hospitals. Standard instrument development processes were followed across 4 phases. Twenty-nine semistructured interviews with children and young people permitted draft age-appropriate extensions. Twenty-eight cognitive interviews informed items and response options. Age-appropriate extensions were prepiloted with 49 participants to ensure feasibility and administered via a postal survey to a national sample of 160 participants for psychometric evaluation using Rasch analysis. Construct validity was evaluated through correlations with the Pediatric Quality of Life Inventory. Psychometric indices of validity and reliability of the instrument versions. Interviews confirmed that the existing VQoL_CYP content and format were relevant across a wider age range. Age-appropriate extensions were drafted for children (8–12 years) and young people (13–17 years). Psychometric item reduction produced 20-item child and 22-item young person versions, each with acceptable fit values, no notable differential item functioning, good measurement precision, ordered response categories and acceptable targeting, and no notable differential item functioning on items common to both. Construct validity was demonstrated through correlations with health-related quality of life (r = 0.698). Using an efficient child- and young person–centered approach, we developed 2 robust, age-appropriate versions of an instrument capturing VQoL that can be used cross-sectionally or sequentially across the age range of 8 to 17 years in research and clinical practice. This approach may be applicable in other rare childhood ophthalmic disorders. [ABSTRACT FROM AUTHOR]

The article discusses vulnerabilities in diabetic eye screening for children and young people in England. Topics include the National Pediatric Diabetes Audit using data reported routinely by pediatricians caring for children living with diabetes, and the medical care of children living with diabetes involves a diverse range of professionals and services spanning primary care.

Strabismus refers to an abnormal alignment of the eyes leading to the loss of central binocular vision. Concomitant strabismus occurs when the angle of deviation is constant in all positions of gaze and often manifests in early childhood when it is considered to be a neurodevelopmental disorder of the visual system. As such, it is inherited as a complex genetic trait, affecting 2–4% of the population. A genome-wide association study (GWAS) for self-reported strabismus (1345 cases and 65,349 controls from UK Biobank) revealed a single genome-wide significant locus on chromosome 17q25. Approximately 20 variants across the NPLOC4–TSPAN10–PDE6G gene cluster and in almost perfect linkage disequilibrium (LD) were most strongly associated (lead variant: rs75078292, OR = 1.26, p = 2.24E−08). A recessive model provided a better fit to the data than an additive model. Association with strabismus was independent of refractive error, and the degree of association with strabismus was minimally attenuated after adjustment for amblyopia. The association with strabismus was replicated in an independent cohort of clinician-diagnosed children aged 7 years old (116 cases and 5084 controls; OR = 1.85, p = 0.009). The associated variants included 2 strong candidate causal variants predicted to have functional effects: rs6420484, which substitutes tyrosine for a conserved cysteine (C177Y) in the TSPAN10 gene, and a 4-bp deletion variant, rs397693108, predicted to cause a frameshift in TSPAN10. The population-attributable risk for the locus was approximately 8.4%, indicating an important role in conferring susceptibility to strabismus. [ABSTRACT FROM AUTHOR]

Purpose: To report piloting and initial validation of the VQoL_CYP, a novel age-appropriate vision-related quality of life (VQoL) instrument for self-reporting by children with visual impairment (VI). Methods: Participants were a random patient sample of children with VI aged 10–15 years. 69 patients, drawn from patient databases at Great Ormond Street Hospital and Moorfields Eye Hospital, United Kingdom, participated in piloting of the draft 47-item VQoL instrument, which enabled preliminary item reduction. Subsequent administration of the instrument, alongside functional vision (FV) and generic health-related quality of life (HRQoL) self-report measures, to 101 children with VI comprising a nationally representative sample enabled further item reduction and evaluation of psychometric properties using Rasch analysis. Construct validity was assessed through Pearson correlation coefficients. Results: Item reduction through piloting (8 items removed for skewness and individual item response pattern) and validation (1 item removed for skewness and 3 for misfit in Rasch) produced a 35-item scale, with fit values within acceptable limits, no notable differential item functioning, good measurement precision, ordered response categories and acceptable targeting in Rasch. The VQoL_CYP showed good construct validity, correlating strongly with HRQoL scores, moderately with FV scores but not with acuity. Conclusions: Robust child-appropriate self-report VQoL measures for children with VI are necessary for understanding the broader impacts of living with a visual disability, distinguishing these from limited functioning per se. Future planned use in larger patient samples will allow further psychometric development of the VQoL_CYP as an adjunct to objective outcomes assessment. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Vision impairment and dual sensory problems in middle age" by P. Dawes, C. Dickinson, R. Emsley, and colleagues that was published in the previous issue.

Background/aims To investigate outcomes following cataract surgery with and without primary intraocular lens (IoL) implantation in children under 2 years of age with congenital or infantile cataract. Method Prospective population based cohort study undertaken through the British Isles Congenital Cataract Interest Group, with systematic data collection on children undergoing surgery in UK and Ireland between January 2009 and December 2010. ORs for the association between IoL implantation and visual acuity, postoperative glaucoma and reoperation at 1 year after surgery were estimated using multivariable regression analysis to control for potential confounders. Results Of 221 children, 56/131 with bilateral and 48/ 90 with unilateral cataract underwent primary IoL implantation. IoL implantation was independently associated with better visual outcome in bilateral (OR 4.6, 95% CI 1.6 to 13.1, p=0.004) but not unilateral disease. IoL use increased the odds of reoperation requiring repeat general anaesthetic (bilateral OR 5.5, p<0.01; unilateral OR 16.7, p<0.01). IoL implantation did not reduce the odds of postoperative glaucoma. Conclusions The use of IoLs in cataract surgery in young children should be critically reassessed, particularly used in settings/communities where close, long-term follow-up is challenging. The absence of visual benefit and the lack of a previously postulated protective effect against postoperative glaucoma serve to question the value of IoLs in unilateral disease. The potential association between IoL use and better early visual outcomes in bilateral disease needs to be balanced against the risk of reoperation and exposure to additional general anaesthetics during a key period of neurodevelopment. [ABSTRACT FROM AUTHOR]

Purpose: To report the methodology and findings of a large scale investigation of burden and distribution of refractive error, from a contemporary and ethnically diverse study of health and disease in adults, in the UK. Methods: U K Biobank, a unique contemporary resource for the study of health and disease, recruited more than half a million people aged 40–69 years. A subsample of 107,452 subjects undertook an enhanced ophthalmic examination which provided autorefraction data (a measure of refractive error). Refractive error status was categorised using the mean spherical equivalent refraction measure. Information on socio-demographic factors (age, gender, ethnicity, educational qualifications and accommodation tenure) was reported at the time of recruitment by questionnaire and face-to-face interview. Results: Fifty four percent of participants aged 40–69 years had refractive error. Specifically 27% had myopia (4% high myopia), which was more common amongst younger people, those of higher socio-economic status, higher educational attainment, or of White or Chinese ethnicity. The frequency of hypermetropia increased with age (7% at 40–44 years increasing to 46% at 65–69 years), was higher in women and its severity was associated with ethnicity (moderate or high hypermetropia at least 30% less likely in non-White ethnic groups compared to White). Conclusions: Refractive error is a significant public health issue for the UK and this study provides contemporary data on adults for planning services, health economic modelling and monitoring of secular trends. Further investigation of risk factors is necessary to inform strategies for prevention. There is scope to do this through the planned longitudinal extension of the UK Biobank study. [ABSTRACT FROM AUTHOR]

Purpose We sought to define normative visual field (VF) values for children using common clinical test protocols for kinetic and static perimetry. Design Prospective, observational study. Subjects We recruited 154 children aged 5 to 15 years without any ophthalmic condition that would affect the VF (controls) from pediatric clinics at Moorfields Eye Hospital. Methods Children performed perimetric assessments in a randomized order using Goldmann and Octopus kinetic perimetry, and Humphrey static perimetry (Swedish Interactive Thresholding Algorithm [SITA] 24-2 FAST), in a single sitting, using standardized clinical protocols, with assessment by a single examiner. Unreliable results (assessed qualitatively) were excluded from the normative data analysis. Linear, piecewise, and quantile mixed-effects regression models were used. We developed a method to display age-specific normative isopters graphically on a VF plot to aid interpretation. Main Outcome Measures Summary measures and graphical plots describing normative VF data for 3 common perimetric tests. Results Visual field area increased with age on testing with Goldmann isopters III4e, I4e, and I2e (linear regression; P < 0.001) and for Octopus isopters III4e and I4e (linear regression; P < 0.005). Visual field development occurs predominately in the inferotemporal field. Humphrey mean deviation (MD) showed an increase of 0.3 decibels (dB; 95% CI, 0.21–0.40) MD per year up to 12 years of age, when adult MD values were reached and thereafter maintained. Conclusions Visual field size and sensitivity increase with age in patterns that are specific to the perimetric approach used. These developmental changes should be accounted for when interpreting perimetric test results in children, particularly when monitoring change over time. [ABSTRACT FROM AUTHOR]

Purpose: To investigate feasibility, reliability and repeatability of perimetry in children. Methods: A prospective, observational study recruiting 154 children aged 5–15 years, without an ophthalmic condition that affects the visual field (controls), identified consecutively between May 2012 and November 2013 from hospital eye clinics. Perimetry was undertaken in a single sitting, with standardised protocols, in a randomised order using the Humphrey static (SITA 24–2 FAST), Goldmann and Octopus kinetic perimeters. Data collected included test duration, subjective experience and test quality (incorporating examiner ratings on comprehension of instructions, fatigue, response to visual and auditory stimuli, concentration and co-operation) to assess feasibility and reliability. Testing was repeated within 6 months to assess repeatability. Results: Overall feasibility was very high (Goldmann=96.1%, Octopus=89% and Humphrey=100% completed the tests). Examiner rated reliability was ‘good’ in 125 (81.2%) children for Goldmann, 100 (64.9%) for Octopus and 98 (63.6%) for Humphrey perimetry. Goldmann perimetry was the most reliable method in children under 9 years of age. Reliability improved with increasing age (multinomial logistic regression (Goldmann, Octopus and Humphrey), p<0.001). No significant differences were found for any of the three test strategies when examining initial and follow-up data outputs (Bland-Altman plots, n=43), suggesting good test repeatability, although the sample size may preclude detection of a small learning effect. Conclusions: Feasibility and reliability of formal perimetry in children improves with age. By the age of 9 years, all the strategies used here were highly feasible and reliable. Clinical assessment of the visual field is achievable in children as young as 5 years, and should be considered where visual field loss is suspected. Since Goldmann perimetry is the most effective strategy in children aged 5–8 years and this perimeter is no longer available, further research is required on a suitable alternative for this age group. [ABSTRACT FROM AUTHOR]

Objective: To develop a novel age-appropriate measure of functional vision (FV) for self-reporting by visually impaired (VI) children and young people. Design: Questionnaire development. Participants: A representative patient sample of VI children and young people aged 10 to 15 years, visual acuity of the logarithm of the minimum angle of resolution (logMAR) worse than 0.48, and a school-based (nonrandom) expert group sample of VI students aged 12 to 17 years. Methods: A total of 32 qualitative semistructured interviews supplemented by narrative feedback from 15 eligible VI children and young people were used to generate draft instrument items. Seventeen VI students were consulted individually on item relevance and comprehensibility, instrument instructions, format, and administration methods. The resulting draft instrument was piloted with 101 VI children and young people comprising a nationally representative sample, drawn from 21 hospitals in the United Kingdom. Initial item reduction was informed by presence of missing data and individual item response pattern. Exploratory factor analysis (FA) and parallel analysis (PA), and Rasch analysis (RA) were applied to test the instrument's psychometric properties. Main Outcome Measures: Psychometric indices and validity assessment of the Functional Vision Questionnaire for Children and Young People (FVQ_CYP). Results: A total of 712 qualitative statements became a 56-item draft scale, capturing the level of difficulty in performing vision-dependent activities. After piloting, items were removed iteratively as follows: 11 for high percentage of missing data, 4 for skewness, and 1 for inadequate item infit and outfit values in RA, 3 having shown differential item functioning across age groups and 1 across gender in RA. The remaining 36 items showed item fit values within acceptable limits, good measurement precision and targeting, and ordered response categories. The reduced scale has a clear unidimensional structure, with all items having a high factor loading on the single factor in FA and PA. The summary scores correlated significantly with visual acuity. Conclusions: We have developed a novel, psychometrically robust self-report questionnaire for children and young people—the FVQ_CYP—that captures the functional impact of visual disability from their perspective. The 36-item, 4-point unidimensional scale has potential as a complementary adjunct to objective clinical assessments in routine pediatric ophthalmology practice and in research. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [ABSTRACT FROM AUTHOR]

Purpose: To report the prevalence and distribution of eye conditions and visual impairment and associations with early social and biological factors using parental report of diagnosed eye conditions and additional chronic illnesses. Design: Population-based, cross-sectional study. Participants: We included 14 981 children, aged 3 years, participating in the United Kingdom Millennium Cohort Study. Methods: Data on demographic, socioeconomic, and maternal and child health factors were obtained by maternal report through structured interview and verbatim reports of diagnosed eye problems and additional chronic illnesses were recorded. Multinomial regression analyses were used to calculate risk ratios of the association of eye disease (with or without associated visual impairment), with socioeconomic and early life factors. Main Outcome Measures: Parental report of diagnosed eye conditions and other chronic illnesses. Results: Overall, at 3 years, 5.7% (95% confidence interval, 5.2–6.3%; n = 881) of children had ≥1 eye condition with 0.24% (0.15–0.3%; n = 45) reported to have associated visual impairment. In the majority, time of onset was reported to be the first year of life. Eye disorders without report of visual impairment were independently associated with lower socioeconomic status, decreasing birth weight, and prematurity. Visual impairment was more likely in those of low birthweight for gestational age and from an ethnic minority group. Maternal illnesses during pregnancy were associated with eye disease without reported visual impairment, as was white ethnicity. Conclusions: Good research opportunities exist within the context of population-based general health surveys to use parental report to estimate minimum prevalence, investigate associations of eye disease with a broad range of environmental factors, and as a mechanism for “flagging” individuals with eye disease in a population sample for further study. Our findings regarding the association of parentally reported childhood eye disease with early life factors such as modest degrees of prematurity, ethnicity and maternal ill-health warrant further investigation. Financial Disclosure(s): The authors have no proprietary or commercial interest in any of the materials discussed in this article. [Copyright &y& Elsevier]

Purpose: To describe the clinical features of children with anophthalmos, microphthalmos, and typical coloboma (AMC). Design: Descriptive, observational, cross-sectional study of the United Kingdom. Participants: A total of 135 children with AMC newly diagnosed over an 18-month period beginning in October 2006. Methods: Cases were identified using active surveillance through an established ophthalmic surveillance system. Eligible cases were followed up 6 months after first notification. Main Outcome Measures: Phenotypic characteristics, both ocular and systemic, clinical investigations, causes, and interventions. Results: A total of 210 eyes (of 135 children) were affected by AMC, of which 153 had isolated coloboma or coloboma with microphthalmos. The most common colobomatous anomaly was a chorioretinal defect present in 109 eyes (71.2%). Some 44% of children were bilaterally visually impaired. Systemic abnormalities were present in 59.7% of children, with craniofacial anomalies being the most common. Children with bilateral disease had a 2.7 times higher odds (95% confidence interval, 1.3–5.5, P = 0.006) of having systemic involvement than unilaterally affected children. Neurologic imaging was the most frequent investigation (58.5%) performed. Less than one third (30.3%) of the children with microphthalmos had ocular axial lengths measured. Eight children had confirmed genetic mutations. Approximately half (49.2%) of the children required ocular intervention. Conclusions: Colobomatous defects were the most common phenotype within this spectrum of anomalies in the United Kingdom. The high frequency of posterior segment colobomatous involvement means that a dilated fundal examination should be made in all cases. The significant visual and systemic morbidity in affected children underlines the importance of a multidisciplinary approach to management. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [ABSTRACT FROM AUTHOR]

Background: Prevention of visual impairment and blindness in childhood is an international priority. However, many countries do not have contemporary information about incidence and causes, from which the scope and priorities for prevention and treatment can be identified. Methods: In the UK, children aged younger than 16 years newly diagnosed with severe visual impairment or blindness (SVI/BL, WHO criteria) during 2000 were identified through national active surveillance schemes in ophthalmology and paediatrics. From these data, we calculated yearly age-group specific incidence and cumulative incidence. Causes were classified by the anatomical site or sites affected and by timing of the insult or insults and causal factors, where known. Findings: Of 439 newly diagnosed children, 336 (77%) had additional non-ophthalmic disorders or impairments (SVI/BL plus). Total yearly incidence was highest in the first year of life, being 4.0 (95% CI 3.6-4.5) per 10000, with a cumulative incidence by 16 years of age of 5.9 (5.3-6.5) per 10000. 10% (44) of all children died within 1 year of diagnosis of blindness. Prenatal causal factors affected 61% (268) of children, with perinatal or neonatal and childhood factors each affecting 18% (77). Incidence and causes varied with presence of non-ophthalmic impairments or disorders, birthweight, and ethnic origin. At least 75% (331) of children had disorders that were neither potentially preventable nor treatable, with current knowledge. Interpretation: Severe visual impairment and blindness in childhood in the UK is more common, occurs more frequently in the context of complex non-ophthalmic impairments, and has greater associated mortality, than previously assumed. An increased rate in children of low birthweight and from ethnic minority groups, together with the observed diversity and complexity of the causes, reflect recent secular changes in the population at risk, specific risk factors, and strategies available for treatment. [ABSTRACT FROM AUTHOR]

Background: To assess cross-cultural validity between Dutch and English versions of the FVQ_CYP, a patient-reported outcome measure developed in the United Kingdom (UK) for children and adolescents with (severe) visual impairment or blindness (VI for brevity) to measure functional vision.Methods: The 36-item FVQ_CYP was translated and adapted into Dutch using standard guidelines. The questionnaire was administered to Dutch children and adolescents aged 7-17 years (N = 253) with impaired vision (no restrictions regarding acuity). Data were compared to existing UK data of children and adolescents aged 10-15 years (N = 91) with VI (acuity LogMar worse than 0.48). As with the original UK FVQ_CYP validation, a rating scale model (RSM) was applied to the Dutch data.Results: Minor adaptations were needed in translation-rounds. Significant differences in item responses were found between the Dutch and UK data. Item response theory assumptions were met, but fit to the RSM was unsatisfactory. Therefore, psychometric properties of the Dutch FVQ_CYP were analysed irrespective of the original model and criteria used. A graded response model led to the removal of 12 items due to missing data, low information, overlapping content and limited relevance to Dutch children. Fit indices for the remaining 24 items were adequate.Conclusions: Differences in population characteristics, distribution of responses, non-invariance at the model level and small sample sizes challenged the cross-cultural validation process. However, the Dutch adapted FVQ_CYP showed high measurement precision and broad coverage of items measuring children's functional vision. The underlying reasons for differences between countries in instrument performance are discussed with implications for future studies. [ABSTRACT FROM AUTHOR]

Background: Childhood uveitis is a collection of chronic rare inflammatory eye disorders which result in visual loss in at least one eye of one fifth of affected children. Despite the introduction of novel systemic immunochemotherapies, it remains a blinding disease. We have undertaken a systematic review of outcome measures used in interventional trials of children with, or at risk of uveitis, in order to investigate metric quality and heterogeneity, as possible barriers to the translation of clinical research into improved outcomes.Methods: Systematic review of trials registered within databases approved by the International Committee of Medical Journal Editors (ICMJE). Eligible trials for were those which involved participants aged under 18 years with or at risk of non-infectious uveitis. Data on date of study commencement, uveitis site, inclusion age criteria, and outcome measure characteristics including type, dimension and quality were extracted independently by two authors. Quality was determined using the reproducibility, validity and age-appropriateness of the metric.Results: Of 917 identified trials, 57 were eligible for inclusion. Twenty different domains across five dimensions were used as primary outcome measures. The structure most commonly used was multiple separate outcome measures. In a quarter of studies, outcomes were assessed less than 3 months following the intervention. Disease activity was the most commonly assessed dimension, with only 30 studies (60%) using reproducible methodologies to assess activity. Only 2/12 (18%) studies on intermediate or posterior uveitis used reproducible activity grading schemes. Of 18 studies involving children aged under 6 years old which used outcome measures related to visual function, only 8/18 (44%) described the use of age-appropriate acuity assessment measures. None of the studies used a vision related quality of life metrics which had been validated for use in childhood.Conclusion: This review of outcome measures in childhood uveitis interventional trials has identified under-utilisation of reproducible or child appropriate measures, and considerable heterogeneity in metric type, and structure. Clinicians and researchers interested in improving outcomes for affected children must identify a patient and family centred core outcome set, and work to validate both objective and patient (or proxy) reported disease age appropriate outcome measures. [ABSTRACT FROM AUTHOR]