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.

Capturing the experiences of patients with inherited optic neuropathies: a systematic review of patient-reported outcome measures (PROMs) and qualitative studies.

PURPOSE: To identify and comprehensively evaluate studies capturing the experience of individuals affected by an inherited optic neuropathy (ION), focusing on patient-reported outcome measures (PROMs) and qualitative studies where the health status and quality of life (QoL) of these individuals have been explored. METHODS: Systematic review of five databases using a search strategy combining four concepts: (1) ION; (2) QoL and health status; (3) PROMs; and (4) qualitative research. Studies assessing the impact of ION on any QoL domain using a PROM or qualitative methodology were included and appraised, using criteria based on the COSMIN checklist (for PROM studies) and the CASP checklist (for qualitative studies). RESULTS: Of 1326 unique articles identified, six studies were included. Five PROMs were identified: Visual Function Index (VF-14); Hospital Anxiety and Depression Scale (HADS); a novel graphical online assessment tool (NGOAT) for reporting emotional response to vision loss; a new PROM informed by the DSM-V Criteria for Major Depressive Disorder; and an interpersonal and career 'impact rating' PROM. The psychometric performance of included PROMs were poorly described. Qualitative studies found that vision loss resulted in psychosocial losses including loss of social and communication skills and loss of independence and freedom. Factors that modified the response to vision loss were also identified. CONCLUSION: The current PROMs used by individuals with ION have poor content coverage, primarily measuring activity limitation and emotional well-being, and insufficient reporting of psychometric performance. There is a need to develop a PROM for individuals ION to report their experiences of living with their condition.

This is me: A qualitative investigation of young people's experience of growing up with visual impairment.

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.

Differences in Self-Rated Versus Parent Proxy-Rated Vision-Related Quality of Life and Functional Vision of Visually Impaired Children.

PURPOSE: To investigate disagreement between children's self-reported vision-related quality of life (VQoL) and functional vision (FV) and their parents' proxy-reports. DESIGN: Cross-sectional study. METHODS: A total of 152 children aged 7-18 years with visual impairment (VI) (defined by the World Health Organization), and their parents, were recruited from 22 National Health Service (NHS) ophthalmology departments in the United Kingdom. Age-appropriate versions of 2 vision-specific instruments, capturing VQoL and FV, were administered to children alongside modified versions for completion by parents on behalf of their child (ie, parent proxy-report). Disagreement between self-report and parent proxy-report was examined using the Bland-Altman (BA) method and a threshold of disagreement based on 0.5 standard deviation. Disagreement was analyzed according to participants' age, sex, and clinical characteristics, using logistic regression analyses. RESULTS: Children rated themselves as having better outcomes than their parents did, although parents both under- and overestimated their child's VQoL (mean score difference = 7.7). With each year of increasing age, there was a 1.18 (1.04-1.35) higher odds of children self-rating their VQoL better than their parents (P = .013). Although parents consistently underestimated their child's FV (mean score difference = -4.7), no characteristics were significantly associated with differences in disagreement. CONCLUSIONS: Disagreement between child self-report on the impact of VI and their parents' proxy-reports varies by age. This implies that self-report from children must remain the gold standard. Where self-reporting is not possible, parent proxy-reports may provide useful insights, but they must be interpreted with caution.

Feasibility of using patient-reported outcome measures with visually impaired children/young people attending paediatric ophthalmology clinics.

OBJECTIVE: To explore feasibility of using child/young person patient-reported outcome measures (PROMs) routinely in practice, using vision-specific instruments and paediatric ophthalmology as the exemplar. METHODS: Participants comprised patients aged 8-17 years, with visual impairment or low vision (visual acuity of the logarithm of the minimum angle of resolution (logMAR) worse than 0.3 in the better eye), attending the Department of Ophthalmology at Great Ormond Street Hospital, London, UK. All participants completed age-appropriate PROMs before attending their outpatient appointment. Half were randomly assigned to completion at home, with the choice of paper-and-pencil or electronic format. The other half were invited to complete PROMs during their hospital appointment, and randomly assigned to completion format. All participants completed a face-to-face survey exploring their attitudes and preferences. Analysis comprised survival analysis, and direct comparisons of proportions, with complementary qualitative data analysis. RESULTS: 93 patients participated. 48 (98%) completing PROMs at home chose the paper-and-pencil format. Completion at home took longer than at hospital (median=20, vs 14 min, p<0.001). Visual acuity was associated with completion time (p=0.007) and missing data (p=0.03). Overall, 52 (60%) reported a preference for completion at home but there was no clear preference for format (37 (43%) preferred either format). CONCLUSION: PROM completion at home ahead of hospital appointments may be preferable for collecting complete, high-quality datasets. Despite equipoise on preference for format, the majority of those completing at home chose the traditional paper-and-pencil format, despite impaired sight. These findings should inform implementation of child/young person PROMs into routine practice.

Attitudes, experiences, and preferences of ophthalmic professionals regarding routine use of patient-reported outcome measures in clinical practice.

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.

A Patient-reported Outcome Measure of Functional Vision for Children and Young People Aged 8 to 18 Years With Visual Impairment.

PURPOSE: To develop age-appropriate extensions of a patient-reported outcome measure for capturing the functional impact of visual impairment on daily activities of children and young people aged 8 up to 18 years. DESIGN: Questionnaire development and validation study. METHODS: Pediatric Ophthalmology departments at Great Ormond Street Hospital and Moorfields Eye Hospital, and, in the final study phase, 20 further UK hospitals. Children and young people (aged 6-19 years) with visual impairment (acuity of the logarithm of the minimum angle of resolution (LogMAR) worse than 0.50 in the better eye) due to any cause but without significant non-ophthalmic impairments. We used our prototype FVQ_CYP for 10-15 year olds as the foundation. Twenty-nine semi-structured interviews confirmed relevance of existing, and identified new, age-specific items. Twenty-eight cognitive interviews captured information regarding comprehensibility and format. The FVQ_Child (8-12 years) and FVQ_Young Person (13-18 years) were evaluated with a national sample of 113 children and 96 young people using Rasch analysis. RESULTS: Issues emerging from interviews with children and young people were largely congruent with those elicited originally with 10-15 year olds. The 28-item FVQ_Child and 38-item FVQ_Young Person versions have goodness-of-fit statistics within the interval 0.5, 1.5 and person separation values of 5.87 and 6.09 respectively. Twenty-four overlapping "core" items enabled their calibration on the same measurement scale. Correlations with acuity (r = 0.47) demonstrated construct validity. CONCLUSIONS: The FVQ_C and FVQ_Young Person are robust age-appropriate versions of the FVQ_CYP which can be used cross-sectionally or sequentially/longitudinally across the age range of 8 up to 18 years in clinical practice and research.

An Age- and Stage-Appropriate Patient-Reported Outcome Measure of Vision-Related Quality of Life of Children and Young People with Visual Impairment.

PURPOSE: 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. DESIGN: Questionnaire development. PARTICIPANTS: 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. METHODS: 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. MAIN OUTCOME MEASURES: Psychometric indices of validity and reliability of the instrument versions. RESULTS: 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). CONCLUSIONS: 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.

UNICORNS: Uveitis in childhood prospective national cohort study protocol.

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.

Cross-cultural validation of the Functional Vision Questionnaire for Children and Young People (FVQ_CYP) with visual impairment in the Dutch population: challenges and opportunities.

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.

Do visually impaired children and their parents agree on the child's vision-related quality of life and functional vision?

AIMS: To investigate agreement between children with visual impairment (VI) and their parents on their ratings of the child's vision-related quality of life (VQoL) and functional vision (FV) using two novel self-report patient-reported outcome measures developed for this population. METHODS: 99 children aged 10-15 years (mean age=12.2, SD=1.9) with VI (best corrected acuity (logarithm of the minimum angle of resolution) 0.50 or worse in better eye) and their parents participated in a national postal survey, completing the child and proxy versions of our novel instruments assessing VQoL and FV of children with VI-the vision-related quality of life instrument for children and young people (VQoL_CYP) and the functional vision questionnaire for children and young people (FVQ_CYP), respectively. Parent-child agreement was investigated using the Bland-Altman (BA) method. Variation across key sociodemographic and clinical characteristics was examined using the Intraclass Correlation Coefficient. RESULTS: Average parental ratings of their child's VQoL and FV were significantly lower than the children's own ratings, but the range of disagreement was wide, with parents both overestimating and underestimating their child's VQoL (mean score difference=5.7, BA limits of agreement (LOA): lower -22.10 (CI 95% -24.61 to 19.59) and upper 33.50 (CI 95% 30.99 to 36.01)), but more consistently underestimating the child's FV (mean score difference=-11.8, BA LOA: lower -39.60 (CI 95% -42.12 to 37.08) and upper 16 (CI 95% 13.48 to 18.52)). There was variation in agreement by some child characteristics, including vision level, time of onset and course of VI progression. CONCLUSIONS: Visually impaired children and their parents perceive the broader impact of living with VI very differently. There is value in routine capture of information independently from children and their parents for comprehensively gauging the impact of childhood VI and tailoring appropriate interventions.

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.

Measuring the Quality of Life of Visually Impaired Children: First Stage Psychometric Evaluation of the Novel VQoL_CYP Instrument.

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.

Story discourse and use of mental state language between mothers and school-aged children with and without visual impairment.

BACKGROUND: Lack of sight compromises insight into other people's mental states. Little is known about the role of maternal language in assisting the development of mental state language in children with visual impairment (VI). AIMS: To investigate mental state language strategies of mothers of school-aged children with VI and to compare these with mothers of comparable children with typically developing vision. To investigate whether the characteristics of mother-child discourse were associated with the child's socio-communicative competence. METHODS & PROCEDURES: Mother-child discourse with twelve 6-12-year-old children with VI was coded during a shared book-reading narrative and compared with 14 typically sighted children matched in age and verbal ability. OUTCOMES & RESULTS: Mothers of children with VI elaborated more and made significantly more references to story characters' mental states and descriptive elaborations than mothers of sighted children. Mental state elaborations of mothers in the VI group related positively with the level produced by their children, with the association remaining after mothers' overall verbosity and children's developmental levels were controlled for. Frequency of maternal elaborations, including their mental state language, was related to socio-communicative competence of children with VI. CONCLUSIONS & IMPLICATIONS: The findings offer insights into the potential contribution of maternal verbal scaffolding to mentalistic language and social-communicative competences of children with VI.

Development of the functional vision questionnaire for children and young people with visual impairment: the FVQ_CYP.

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.

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.