Associations between unilateral amblyopia in childhood and cardiometabolic disorders in adult life: a cross-sectional and longitudinal analysis of the UK Biobank.

Background: Amblyopia is a common neurodevelopmental condition and leading cause of childhood visual impairment. Given the known association between neurodevelopmental impairment and cardiometabolic dysfunction in later life, we investigated whether children with amblyopia have increased risk of cardiometabolic disorders in adult life. Methods: This was a cross-sectional and longitudinal analysis of 126,399 United Kingdom Biobank cohort participants who underwent ocular examination. A subset of 67,321 of these received retinal imaging. Data analysis was conducted between November 1st 2021 and October 15th 2022. Our primary objective was to investigate the association between amblyopia and a number of components of metabolic syndrome and individual cardiometabolic diseases. Childhood amblyopia, dichotomised as resolved or persisting by adulthood, cardiometabolic disease and mortality were defined using ophthalmic assessment, self-reported, hospital admissions and death records. Morphological features of the optic nerve and retinal vasculature and sublayers were extracted from retinal photography and optical coherence tomography. Associations between amblyopia and cardiometabolic disorders as well as retinal markers were investigated in multivariable-adjusted regression models. Findings: Individuals with persisting amblyopia (n = 2647) were more likely to be obese (adjusted odds ratio (95% confidence interval): 1.16 (1.05; 1.28)), hypertensive (1.25 (1.13; 1.38)) and diabetic (1.29 (1.04; 1.59)) than individuals without amblyopia (controls, (n = 18,481)). Amblyopia was also associated with an increased risk of myocardial infarction (adjusted hazard ratio: 1.38 (1.11; 1.72)) and death (1.36 (1.15; 1.60)). On retinal imaging, amblyopic eyes had significantly increased venular caliber (0.29 units (0.21; 0.36)), increased tortuosity (0.11 units (0.03; 0.19)), but lower fractal dimension (-0.23 units (-0.30; -0.16)) and thinner ganglion cell-inner plexiform layer (mGC-IPL, -2.85 microns (-3.47; -2.22)). Unaffected fellow eyes of individuals with amblyopia also had significantly lower retinal fractal dimension (-0.08 units (-0.15; -0.01)) and thinner mGC-IPL (-1.14 microns (-1.74; -0.54)). Amblyopic eyes with a persisting visual deficit had smaller optic nerve disc height (-0.17 units (-0.25; -0.08)) and width (-0.13 units (-0.21; -0.04)) compared to control eyes. Interpretation: Although further research is needed to understand the basis of the observed associations, healthcare professionals should be cognisant of greater cardiometabolic dysfunction in adults who had childhood amblyopia. Differences in retinal features in both the amblyopic eye and the unaffected non-amblyopic suggest generalised versus local processes. Funding: Medical Research Council (MR/T000953/1) and the National Institute for Health and Care Research.

Individual, family, and environmental determinants of vision-related quality of life of children and young people with visual impairment.

Childhood visual impairment can have a significant impact on an individual's development. To improve clinical care and develop appropriate psychosocial interventions of these patients, it is necessary to understand the contributing and modifiable factors that both identify individuals in greater need and could be targeted in interventions. Here we investigate the broader individual, family, and environmental factors associated with vision-related quality of life (VQoL) of children and young people with visual impairment (CYP-VI). Data for this cross-sectional study were collected from September 2014 to May 2017 to develop and validate two vision-specific patient-reported outcome measures (PROMs) for CYP-VI. Patients were recruited from 22 hospitals in the United Kingdom and were aged 7-18 years with visual impairment as per WHO criteria. Participants self-completed the two PROMs, VQoL and Functional Vision Questionnaires. Clinical characteristics were extracted from medical records. Their carers provided information on family sociodemographic backgrounds. Associations between the VQoL scores and other factors were examined using Spearman's correlation, Kruskal-Wallis, Wilcoxon rank-sum tests, and quantile regression models. The sample consisted of 152 CYP-VI (67 females). Better VQoL was significantly associated with better functional vision overall (rSpearman = -0.52), parent-reported absence of additional chronic conditions (dCohen = 0.46), attending mainstream (versus other) school (dCohen = 0.44), higher socio-economic status (rSpearman = 0.17) and higher parental education level (rSpearman = 0.20). No other investigated factors were significantly associated with VQoL. The final quantile regression model included functional vision scores and the presence of additional health condition. Variation in self-reported VQoL in CYP-VI can be partly accounted for by factors relating to the clinical status of the affected child and, more importantly, by non-health-related factors. This needs to be considered in clinical practice when assessing vision-specific outcomes and providing support to CYP-VI, as well as in the development of future interventions.

Data saves lives: optimising routinely collected clinical data for rare disease research.

Necessity driven organisational change in the post-pandemic landscape has seen health care providers adopting innovations to manage and process health data. These include the use of 'real-world' datasets of routinely collected clinical information, enabling data-driven delivery. Rare disease risks being 'left-behind' unless our clinical and research communities engage with the challenges and opportunities afforded by the burgeoning field of health data informatics. We address the challenges to the meaningful use and reuse of rare disease data, and, through a series of recommendations around workforce education, harmonisation of taxonomy, and ensuring an inclusive health data environment, we highlight the role that those who manage rare disease must play in addressing them.

Trends in the long-term impact of childhood visual impairment on health and social outcomes in the UK: a cross-cohort study across three decades of disability-related legislation and policy implementation.

BACKGROUND: Childhood vision impairment (VI) can adversely impact health and social outcomes and limit life chances. We investigated whether its adverse impacts into adult life changed during a period in which legislation, policy and services to address inequalities relating to disability were implemented. METHODS: Cross-cohort study comprising 14 247 participants from the 1946, 1958 and 1970 British birth cohorts (BC). Participants dichotomized as VI at age 15/16 (distance visual acuity was 6/12 or worse in the better-seeing eye) or normally sighted. Associations of childhood VI with health, well-being, socioeconomic and social participation outcomes in mid-adult life were investigated using regression models adjusted for participants' early life socioeconomic markers and sex. Change in adjusted odds ratios of >10% in the same direction in successive cohorts, or a > 20% difference between 1970BC and one older cohort were considered meaningful. RESULTS: Trends over time in impacts of childhood onset VI into mid-adult life were complex. This included worsening of odds of poorer physical health (odds ratio 1.47; 95% confidence interval 1.02-2.14), living in unsatisfactory (1.54; 1.03-2.29) or overcrowded (2.34; 1.26-4.06) households, being unemployed (2.19; 1.19-3.97) and not gaining additional educational qualifications during mid-adult life (1.61; 1.08-2.47). By contrast the odds of not participating in some social activities (e.g. seeing friends) improved over time. Associations with other outcomes were unchanged. CONCLUSIONS: Many adverse impacts of childhood VI do not appear ameliorated over time by legislation, policies and provision that would have been expected to reduce inequalities. Moreover, some were increased. Childhood VI continues to cast a life-long shadow.

Delayed diagnosis of congenital cataract in preterm infants: Findings from the IoLunder2 cohort study.

BACKGROUND AND OBJECTIVES: Early detection is critical to achieving optimal outcomes in children with congenital cataract. We hypothesized that detection of congenital cataract in preterm infants would be delayed compared with term/post-term peers due to delayed delivery of whole population child health interventions. METHODS: Secondary analysis of data using a nested case-control study approach in a prospective population-based cohort study. Inclusion criteria comprised children diagnosed with congenital cataract requiring surgical intervention during the first two years of life in UK and Ireland in 2009 and 2010. Association between late detection (after eight weeks post-natal age, ie outside the neonatal and infant eye national screening programme) of cataract and preterm birth (gestational age less than 37 weeks) was assessed using multivariable logistic regression. RESULTS: Of 186 children with congenital cataract, 17 children were born preterm (9%, gestational age range 24-37weeks). Neonatal detection occurred in 64/186 (34%), and late detection in 64 children (34%). Late detection was independently associated with premature birth, specifically moderate/late preterm birth (adjusted odds ratio 3.0, 95%CI 1.1 to 8.5). CONCLUSIONS: Our findings suggest that, despite enhanced eye surveillance being recommended for those born moderate/late preterm (32+ weeks gestational age, ie not eligible for retinopathy of prematurity screening), congenital cataract is not being effectively detected through the routine screening programme for this vulnerable group. It is necessary to improve the effectiveness of the screening programme, and care must be taken to ensure that competing health care needs of preterm children do not prevent universal child health interventions.

Educational attainment and trajectories at key stages of schooling for children with amblyopia compared to those without eye conditions: Findings from the Millennium Cohort Study.

BACKGROUND: Amblyopia is a neurodevelopmental condition resulting in reduced vision for which whole population child vision screening is undertaken. Cross-sectional studies have established an association between amblyopia and lower academic self-concept, slower reading speed. No difference has been found in educational performance in adolescence whilst there are mixed associations with educational attainment in adults. Educational trajectories and intentions have not been studied previously. We analyse if those treated for amblyopia have different educational performance and trajectories for core subjects during statutory schooling, or subsequent higher education (university) intentions than their peers without eye conditions. METHODS AND FINDINGS: Data from the Millennium Cohort Study of children born in the United Kingdom in 2000-01 and followed-up to age 17 years (n = 9989). Using a validated approach drawing on parental self-report on eye conditions and treatment coded by clinical reviewers, participants were grouped into mutually exclusive categories: no eye conditions, strabismus alone, refractive amblyopia, strabismic/mixed (refractive plus strabismic) amblyopia. The outcomes were levels and trajectories of passing English, Maths, Science at ages 7-16 years, passing national exams at age 16, and intentions at ages 14-17 to pursue higher (university) education. Adjusted analyses showed that amblyopia status was not associated with performance in English, Maths, and Science at any key stage, attainment in national exams, or intending to go to university. Similarly, the age-related trajectories of performance in core subjects and higher education intentions did not differ between the groups. There were no significant differences in main reason for having or not having university intentions. CONCLUSIONS: We found no associations between a history of amblyopia and either adverse performance or age-related attainment trajectories in core subjects during key stages of statutory schooling as well as the absence of an association with intentions for higher education. These results should be reassuring to affected children and young people, and their families, teachers and physicians.

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.

The role of screening and surveillance in the detection of childhood vision impairment and blindness in the UK.

OBJECTIVE: Understanding pathways to detection for childhood visual impairment (VI) is critical for planning services. We aimed to describe patterns of detection for childhood VI. DESIGN AND SETTING: Cross-sectional study using data from British Childhood Visual Impairment and Blindness Study 2. PATIENTS: Children newly diagnosed with VI, severe vision impairment or blindness (SVI/BL)-that is, visual acuity worse than logMAR 0.5 in both eyes-were identified through active surveillance, with data collection at diagnosis and 1 year later. OUTCOME MEASURE: Method of detection of vision/eyes problem. RESULTS: 784 children (45%, 356 girls) were identified, of whom 313 (40%) had VI, 471 (60%) had SVI/BL. Additional non-ophthalmic disorders or impairments (VI/SVI/BL 'plus'), were diagnosed in 72% (559/784). Of the 784, 173 children were detected through routine screening (22%), 248 through targeted examinations (32%) and 280 through family self-referral (36%). Parents and carers had only reported symptoms in 55% of children who manifested them, with evidence that families living in socioeconomically deprived areas were less likely to report concerns. Paediatricians were the professionals most likely to raise initial suspicion of visual disability. CONCLUSIONS: Our findings show that targeted screening and surveillance is important for the detection of full spectrum childhood visual impairment (VI/SVI/BL), as a significant proportion of children will not have symptoms, or their parents or carers will not report symptoms. As paediatricians were the professionals most commonly involved in detection, it would be helpful if their core competencies included the skills needed to undertake simple assessments of vision.

Is amblyopia associated with school readiness and cognitive performance during early schooling? Findings from the Millennium Cohort Study.

BACKGROUND: Amblyopia is a neurodevelopmental condition causing reduced vision, for which programmes of whole population child vision screening exist throughout the world. There is an ongoing debate about the value of screening due to the lack of evidence about meaningful functional impacts of amblyopia. Our objective was to determine whether amblyopia is associated with school readiness and early cognitive performance. METHODS AND FINDINGS: Data from the prospective Millennium Cohort Study of children born in the United Kingdom in 2000-01 and followed-up to age 7 years (n = 13,967). Using parental self-report on eye conditions and treatment coded by clinical reviewers, participants were grouped into no eye conditions, strabismus alone, refractive amblyopia, or strabismic/mixed (refractive plus strabismic) amblyopia. The outcomes were poor school readiness using Bracken School Readiness Assessment <25th percentile (age 3); and cognitive tests and their age-related trajectories using British Ability Scale II Naming Vocabulary (ages 3/5) and Pattern Construction (ages 5/7). Multivariable analyses showed that compared to children without any eye conditions, only those with strabismic/mixed amblyopia had an increased risk of poor school readiness (OR = 2.04, 95%CI 1.09-3.82). Small differences in mean scores for NV and PC of children with amblyopia (all types) compared to those without any eye condition were not clinically significant (>10 points) irrespective of whether treatment had already started. The age-related cognitive trajectories of children with amblyopia did not differ from those without any eye conditions for either NV (p = 0.62) or PC (p = 0.51). These associations are at population rather than individual level, so it might be that some individuals with amblyopia did experience significant adverse outcomes that are not captured by summary statistics. CONCLUSIONS: Amblyopia is not significantly associated with adverse cognitive performance and trajectories in early schooling and there is no evidence that this is due to a mediating effect of treatment. Although amblyopia combined with strabismus is associated with poor school readiness, this is not translated into poor cognitive performance. These novel findings may explain the lack of association reported between amblyopia and educational outcomes in adult life and suggest that the impact of amblyopia on education is not of itself a justification for whole population child vision screening aimed at detecting this disorder.

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

PURPOSE: Appropriate correction of aphakia is key to good outcomes. There may be clinical settings where and populations in whom accessing or managing aphakic contact lenses is challenging. Strategies to target the increased risk of visual axis opacity (VAO) after primary intraocular lens (IOL) implantation in infancy are necessary. We describe the predictors of VAO after primary IOL implantation for unilateral or bilateral congenital or infantile cataract in children younger than 2 years of age. DESIGN: Population-based (United Kingdom and Ireland), prospective, inception cohort study undertaken through a national clinical network. PARTICIPANTS: A total of 105 children (57 with bilateral cataract, 48 with unilateral cataract, total 162 eyes) undergoing primary IOL implantation in the first 2 years of life between January 2009 and December 2010. METHODS: Observational longitudinal study with multilevel, multivariable modeling to investigate associations between outcome of interest and child- and treatment-specific factors, including age, axial length, socioeconomic status, IOL model, and postoperative steroid use. MAIN OUTCOME MEASURES: Postoperative proliferative or inflammatory visual axis opacity (VAO) requiring surgical correction. RESULTS: Visual axis opacity occurred in 67 eyes (45%), typically within the first postoperative year. Use of a 3-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 after IOL implantation in early childhood. The findings of this prospective cohort study suggest that the use of 3-piece IOL models may reduce the risk of pseudophakic VAO in children younger than 2 years of age.

Self-Reported Health Experiences of Children Living with Congenital Heart Defects: Including Patient-Reported Outcomes in a National Cohort Study.

BACKGROUND: Understanding children's views about living with congenital heart defects (CHDs) is fundamental to supporting their successful participation in daily life, school and peer relationships. As an adjunct to a health and quality of life outcomes questionnaire, we asked school-age children who survived infant heart procedures to describe their experiences of living with CHDs. METHODS: In a UK-wide cohort study, children aged 10 to 14 years with CHDs self-completed postal questionnaires that included an open question about having a 'heart problem'. We compared the characteristics of children with more and less severe cardiac diagnoses and, through collaborative inductive content analysis, investigated the subjective experiences and coping strategies described by children in both clinical severity groups. RESULTS: Text and/or drawings were returned by 436 children (246 boys [56%], mean age 12.1 years [SD 1.0; range 10-14]); 313 had less severe (LS) and 123 more severe (MS) cardiac diagnoses. At the most recent hospital visit, a higher proportion of the MS group were underweight (more than two standard deviations below the mean for age) or cyanosed (underweight: MS 20.0%, LS 9.9%; cyanosed: MS 26.2%, LS 3.5%). Children in the MS group described concerns about social isolation and feeling 'different', whereas children with less severe diagnoses often characterised their CHD as 'not a big thing'. Some coping strategies were common to both severity groups, including managing health information to avoid social exclusion, however only children in the LS group considered their CHD 'in the past' or experienced a sense of survivorship. CONCLUSIONS: Children's reported experiences were not dependent on their cardiac diagnosis, although there were clear qualitative differences by clinical severity group. Children's concerns emphasised social participation and our findings imply a need to shift the clinical focus from monitoring cardiac function to optimising participation. We highlight the potential for informing and evaluating clinical practice and service provision through seeking patient-reported outcomes in paediatric care.

Patient-reported outcome measures (PROMs) in paediatric ophthalmology: a systematic review.

AIM: To identify patient-reported outcome measures (PROMs) specifically developed and used to assess the impact of ophthalmic disorders in children and to systematically assess their quality as a basis for recommendations about their use in clinical and research settings. METHODS: A systematic review of the literature was performed in MEDLINE, EMBASE, PsychINFO, CINAHL and AMED, supplemented by a grey literature search. Papers reporting development and validation of questionnaire instruments for assessing patient-reported outcomes of an ophthalmic disorder in patients aged 2-18 years were included. Quality was assessed by examining the purpose and psychometric properties of the instruments. Strengths and limitations were summarised with recommendations regarding use. RESULTS: Search identified 17 instruments. Of these, 11 were condition-specific and six were intended for a broader population of children and young people with visual impairment regardless of the ophthalmic condition. Three were developed for use in a specific trial and two are still in development. CONCLUSIONS: Paediatric ophthalmology PROM development and application is a developing field and new instruments are needed. There is scope for improvement in this area through (a) clarity of definitions of the underlying constructs intended to be measured at the onset of development of new instruments, (b) application of child-centred approaches and (c) adherence to extant guidance and best practice in questionnaire instrument development.

The health-related quality of life of children with hereditary retinal disorders and the psychosocial impact on their families.

PURPOSE: Childhood-onset hereditary retinal disorders comprise a group of visually disabling conditions with variable onset and progression of visual impairment. Their impact on the health-related quality of life (HRQoL) of affected individuals, as well as the broader impact on their families has not been investigated previously. METHODS: In a cross-sectional study, a generic age-appropriate instrument, the PedsQL, was used to assess self-reported HRQoL in a subsample of a representative group of children with hereditary retinal disorders and their siblings as well as parental (proxy) assessment of HRQoL of their affected children. In addition, parents reported the broader impact and effect on functioning of the family using the PedsQL Family Impact Module. RESULTS: Affected children (n = 44) reported worse HRQoL than their unaffected siblings (n = 34) and notably, also worse scores than those reported by children with various serious chronic systemic disorders. On average, parents assessed their child's HRQoL to be worse than that self-reported by the child. There was an overall adverse impact on the family and its functioning, although siblings did not report impaired HRQoL themselves. CONCLUSIONS: This study demonstrates the significant impact, on both affected children and their families, of living with an untreatable, often progressive, and sometimes blinding ophthalmic disorder. It highlights the importance of support for affected individuals and their families, which may be targeted through use of generic or vision-related quality-of-life instruments for children as the latter become more widely available. Assessment of HRQoL would also be an important outcome measure in clinical trials of novel therapies for hereditary retinal disorders.

'Silent voices' in health services research: ethnicity and socioeconomic variation in participation in studies of quality of life in childhood visual disability.

Purpose. To investigate patterns of participation of visually impaired (VI) children and their families in health services research. Methods. The authors compared clinical and sociodemographic characteristics of children and their families who participated with those who did not participate in two studies of quality of life (QoL) of VI children. In Study 1, the authors interviewed VI children and adolescents, aged 10 to 15 years, about their vision-related quality of life (VRQoL) as the first phase of a program to develop a VRQoL instrument for this population. One hundred seven children with visual impairment (visual acuity in the better eye LogMar worse than 0.51) were invited to participate in the interviews. Study 2 investigated health-related quality of life (HRQoL) of VI children using an existing generic instrument, administered in a postal survey. 151 VI children and adolescents, aged 2 to 16 years, with hereditary retinal disorders were invited to participate in the survey. Results. The overall participation level was below 50%. In both studies, participants from white ethnic and more affluent socioeconomic backgrounds were overrepresented. Participation did not vary by age, sex, or clinical characteristics. Conclusions. The authors suggest that there are barriers to participation in child- and family-centered research on childhood visual disability for children from socioeconomically deprived or ethnic minority groups. They urge assessment and reporting of participation patterns in further health services research on childhood visual disability. Failure to recognize that there are "silent voices" is likely to have important implications for equitable and appropriate service planning and provision for VI children.

Incidence of and factors associated with glaucoma after surgery for congenital cataract: findings from the British Congenital Cataract Study.

PURPOSE: To report the incidence of and factors associated with postoperative open-angle glaucoma in a nationally representative group of children undergoing surgery for congenital or infantile cataract. DESIGN: Noncomparative interventional cohort study. PARTICIPANTS: All children in the United Kingdom who were newly diagnosed with congenital or infantile cataract in a 12-month period in 1995 and 1996 (the British Congenital/Infantile Cataract Study) were eligible for this study. One hundred sixty-five children with congenital or infantile cataract underwent cataract surgery. METHODS: All the children were traced through their managing ophthalmologists. Standardized outcome data were collected at least 6 years after diagnosis. For children undergoing cataract extraction, Cox regression analysis was performed to determine incidence of postoperative open-angle glaucoma and the effect of key factors considered, a priori, potentially to be associated with it (i.e., age at detection and surgery, type of cataract surgery, primary intraocular lens implantation, severe postoperative uveitis, and microphthalmia). MAIN OUTCOME MEASURES: Development of open-angle glaucoma after cataract surgery. RESULTS: Postoperative glaucoma developed in 27 of 275 eyes of 165 children who underwent cataract surgery. The overall annual incidence of postoperative glaucoma was 5.25 per 100 cataract operations. The median time to development of postoperative glaucoma was 1.34 years (range, 0.39 months-6.73 years). Younger age at detection of cataract was the only factor independently associated with the development of glaucoma when all other factors of interest (which were all statistically associated with age at detection) were accounted for. A 10-fold increase in the age at detection (for example, 30 days compared with 3 days) was associated with a 64% decrease in the hazard ratio (95% confidence interval, 41%-79%; P<0.001). CONCLUSIONS: Median time to development of postoperative open-angle glaucoma in the present study was lower than that reported previously, emphasizing the need for vigilance from the early postoperative period. Earlier detection of cataract was the only significant factor associated with the development of glaucoma after surgery for congenital cataract.

The health-related quality of life of children with congenital cataract: findings of the British Congenital Cataract Study.

BACKGROUND: There is increasing emphasis on patient-based assessment of healthcare outcomes, but evaluation of the impact of ophthalmic disorders on quality of life is undertaken infrequently. AIM: To report on the health-related quality of life (HRQOL) of children with congenital cataract. METHODS: At least 6 years after diagnosis, 41 children (representative of a national cohort) and their parents completed the child and proxy (parental) versions of PedsQL 4.0, a generic multidimensional paediatric HRQOL instrument, independently. This provided a physical health summary score, a psychosocial health summary score and an overall score (range 0-100, higher scores indicating better HRQOL). RESULTS: Questionnaires were completed by 33 parent-child pairs and by 8 parents alone. The mean (SD) total score self-reported by children was 75.85 (15.56) and that reported by parents was 75.91(16.79). The mean (SD) physical health score self-reported by children was 80.76 (8.61), which was higher than the psychosocial health score of 72.93 (16.06). There was considerable variation in agreement of scores reported by individual child-parent pairs (the largest difference was 56.3 points). Notably, the PedsQL scores of children with congenital cataract were comparable with those reported for some children with severe systemic diseases such as rheumatological disease and some cancers. CONCLUSIONS: These findings serve to characterise the significant broader impact of congenital cataract on patients and their families. The PedsQL 4.0 proved to be an acceptable instrument for assessing generic HRQOL, permitting valuable reporting by children and their parents. However, to capture the subjective experience of ophthalmic disease in childhood fully, specific vision-related quality of life measures for children are needed.

Long-term visual acuity and its predictors after surgery for congenital cataract: findings of the British congenital cataract study.

PURPOSE: To report long-term postoperative visual acuity in a nationally representative group of children with congenital/infantile cataract and to investigate the factors associated with poor vision. METHODS: All children aged less than 16 years in the United Kingdom who had newly diagnosed congenital/infantile cataract in a 12-month period during 1995-1996 (the British Congenital/infantile Cataract Study) were traced through their managing ophthalmologists. Outcome data were collected at least 6 years after diagnosis, by using specifically designed questionnaires. Ordinal regression analysis identified factors associated with postoperative acuity. RESULTS: Of 153 children who had surgery, complete data were available in 122 (85%). Median age at follow-up was 7 and 6.91 years, respectively, for bilateral and unilateral disease. Median age at surgery was 4.57 months in bilateral and 2.99 months in unilateral cases, with 40% and 45%, respectively, of children operated on by 3 months. Median (range) postoperative acuity was 6/18 (6/5, no perception of light) in bilateral and 6/60 (6/5, no perception of light) in unilateral disease. Poor compliance with occlusion was the factor most strongly associated with poorer acuity in both unilateral and bilateral disease: the odds of worse vision in unilateral cataract were 7.92 times greater with <50% versus 100% compliance (95% CI 1.68-37.26). In bilateral disease, odds of worse vision were reduced with each month of decreasing age at surgery (0.98, 95% CI 0.94-0.99), but increased by the presence of additional medical conditions (3.53, 95% CI 1.08-11.44) and the presence of postoperative ocular complications (2.94, 95% CI 1.38-6.51). CONCLUSIONS: These findings support a secular improvement in postoperative acuity in bilateral, and to a lesser extent, unilateral disease. Nevertheless early detection of congenital cataract through effective newborn screening and improving concordance with occlusion both remain priorities. Further improvements in outcomes in unilateral disease are necessary before parents can be advised universally that treatment will achieve a functionally useful "spare" eye.