The British Childhood Visual Impairment and Blindness Study 2 (BCVIS2)

PURPOSE: Representative epidemiological data on childhood visual impairment in the U.K are lacking. The BCVIS2 study is a comprehensive national study to determine the incidence, distribution and characteristics of all-cause childhood visual disability, encompassing for the first time, visual impairment (VI) alongside severe visual impairment (SVI) and blindness (BL). METHODS: All children newly diagnosed as VI or SVI/BL using the WHO classification (VI: 0.5-1.0 LogMAR, SVI: 1.01-1.30 LogMAR and BL: <1.30 LogMAR in the better eye) were identified in the U.K. within a 1 year period (October 2015 – November 2016). Cases were reported on a monthly basis simultaneously but independently through two established, national active surveillance schemes; the British Ophthalmological Surveillance Unit (BOSU, consisting of over 1,300 attending/consultant ophthalmologists), and the British Paediatric Surveillance Unit (BPSU, including over 3,300 attending/consultant paediatricians). Validated postal questionnaires were completed by the reporting clinician at initial case identification and 12 month follow up. RESULTS: nterim analyses are reported here based on the current complete dataset from 748 (of 983) ascertained cases. Analysis of SVI/BL cases (n=463) shows that: - children of low birth weight, of ethnic minority origin, or from the most socially deprived group are over-represented - most children (79%) have additional major non-ophthalmic disorders - disorders of the visual pathway/cortex remain predominant with increased contribution from 48% to 57%. Children with VI (n=285), in comparison to SVI/BL are: - diagnosed later (36% vs 60% diagnosed aged <1 year) - less likely to have additional non-ophthalmic conditions (51% vs 79%) - more likely to have a retinal disorder such as retinal dystrophy (23% vs 11%) or oculocutaneous albinism (12% vs 3%). CONCLUSIONS: Forthcoming analysis on the full cohort will investigate (a) the changing epidemiology of SVI/BL in the past 15 years and (b) provide novel data on the epidemiology of childhood VI. These should address current gaps in the evidence base in childhood visual disability required for the commissioning and delivery of national health (NHS) services and formulation of public health policies

UNICORNS: Uveitis in childhood prospective national cohort study protocol [version 1; peer review: 1 approved, 1 approved with reservations]

Background: Childhood uveitis is a rare inflammatory eye disease which is typically chronic, relapsing-remitting in nature, with an uncertain aetiology (idiopathic). Visual loss occurs due to structural damage caused by uncontrolled inflammation. Understanding of the determinants of long term outcome is lacking, including the predictors of therapeutic response or how to define disease control. Aims: To describe disease natural history and outcomes amongst a nationally representative group of children with non-infectious uveitis, describe the impact of disease course on quality of life for both child and family, and identify determinants of adverse visual, structural and developmental outcomes. Methods: UNICORNS is a prospective longitudinal multicentre cohort study of children newly diagnosed with uveitis about whom a core minimum clinical dataset will be collected systematically. Participants and their families will also complete patient-reported outcome measures annually from recruitment. The association of patient (child- and treatment- dependent) characteristics with outcome will be investigated using logistic and ordinal regression models which incorporate adjustment for within-child correspondence between eyes for those with bilateral disease and repeated outcomes measurement. Discussion: Through this population based, prospective longitudinal study of childhood uveitis, we will describe the characteristics of childhood onset disease. Early (1-2 years following diagnosis) outcomes will be described in the first instance, and through the creation of a national inception cohort, longer term studies will be enabled of outcome for affected children and families

Long-term visual and treatment outcomes of whole-population pre-school visual screening (PSVS) in children:a longitudinal, retrospective, population-based cohort study

BACKGROUND: This study reports the long-term visual and treatment outcomes in a whole-population, orthoptic-delivered pre-school visual screening (PSVS) programme in Scotland and further examines their associations with socioeconomic backgrounds and home circumstances. METHODS: Retrospective case review was conducted on 430 children who failed PSVS. Outcome measures included best corrected visual acuity (BCVA), severity of amblyopia (mild, moderate and severe), binocular vision (BV) (normal, poor and none), ophthalmic diagnosis and treatment modalities. Parameters at discharge were compared to those at baseline and were measured against the Scottish index of multiple deprivation (SIMD) and Health plan indicator (HPI), which are indices of deprivation and status of home circumstances. RESULTS: The proportion of children with amblyopia reduced from 92.3% (373/404) at baseline to 29.1% (106/364) at discharge (p < 0.001). Eighty percent (291/364) had good BV at discharge compared to 29.2% (118/404) at baseline (p < 0.001). Children from more socioeconomically deprived areas (OR 2.19, 95% CI 1.01–4.30, p = 0.003) or adverse family backgrounds (OR 3.94, 95% CI 1.99–7.74, p = 0.002) were more likely to attend poorly and/or become lost to follow-up. Children from worse home circumstances were five times more likely to have residual amblyopia (OR 5.37, 95% CI 3.29–10.07, p < 0.001) and three times more likely to have poor/no BV (OR 3.41, 95% CI 2.49–4.66, p < 0.001) than those from better home circumstances. CONCLUSIONS: Orthoptic-delivered PSVS is successful at screening and managing amblyopia. Children from homes requiring social care input are less likely to attend and are more likely to have poorer visual outcomes

One size doesn’t fit all: time to revisit patient-reported outcome measures (PROMs) in paediatric ophthalmology?

The purpose of this article is to summarise methodological challenges and opportunities in the development and application of patient reported outcome measures (PROMs) for the rare and complex population of children with visually impairing disorders. Following a literature review on development and application of PROMs in children in general, including those with disabilities and or/chronic condition, we identified and discuss here 5 key issues that are specific to children with visual impairment: (1) the conflation between theoretically distinct vision-related constructs and outcomes, (2) the importance of developmentally appropriate approaches to design and application of PROMs, (3) feasibility of standard questionnaire formats and administration for children with different levels of visual impairment, (4) feasibility and nature of self-reporting by visually impaired children, and (5) epidemiological, statistical and ethical considerations. There is an established need for vision-specific age-appropriate PROMs for use in paediatric ophthalmology, but there are significant practical and methodological challenges in developing and applying appropriate measures. Further understanding of the characteristics and needs of visually impaired children as questionnaire respondents is necessary for development of quality PROMs and their meaningful application in clinical practice and research

Under-utilisation of reproducible, child appropriate or patient reported outcome measures in childhood uveitis interventional research.

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

Cataract management in children: a review of the literature and current practice across five large UK centres

Congenital and childhood cataracts are uncommon but regularly seen in the clinics of most paediatric ophthalmology teams in the UK. They are often associated with profound visual loss and a large proportion have a genetic aetiology, some with significant extra-ocular comorbidities. Optimal diagnosis and treatment typically require close collaboration within multidisciplinary teams. Surgery remains the mainstay of treatment. A variety of surgical techniques, timings of intervention and options for optical correction have been advocated making management seem complex for those seeing affected children infrequently. This paper summarises the proceedings of two recent RCOphth paediatric cataract study days, provides a literature review and describes the current UK ‘state of play’ in the management of paediatric cataracts.</p

Non-invasive Instrument-Based Tests for Quantifying Anterior Chamber Flare in Uveitis: A Systematic Review.

: Anterior chamber (AC) flare is a key sign for anterior uveitis. New instrument-based techniques for measuring AC flare can offer automation and objectivity. This review aims to identify objective instrument-based measures for AC flare.: In this systematic review, we identified studies reporting correlation between instrument-based tests versus clinician AC flare grading, and/or aqueous protein concentration, as well as test reliability.: Four index tests were identified in 11 studies: laser-flare photometry (LFP), optical coherence tomography, ocular flare analysis meter (OFAM) and the double-pass technique. The correlation between LFP and clinician grading was 0.40-0.93 and 0.87-0.94 for LFP and protein concentration. The double-pass technique showed no correlation with clinician grading and insufficient information was available for OFAM.: LFP shows moderate to strong correlation with clinician grading and aqueous protein concentration. LFP could be a superior reference test compared to clinician AC flare grading for validating new index tests