The Functional Vision for Communication Questionnaire (FVC-Q): Exploring Parental Report of Non-Speaking Children's Fixation Skills Using a Structured History-Taking Approach.

This paper explores whether a structured history-taking tool yields useful descriptions of children's looking skills. Parents of 32 children referred to a specialist communication clinic reported their child's looking skills using the Functional Vision for Communication Questionnaire (FVC-Q), providing descriptions of single object fixation, fixation shifts between objects and fixation shifts from object to person. Descriptions were compared with clinical assessment. 24/32 children were reported to have some limitation in fixation. Limitation was subsequently seen in 30/32 children. Parental report and assessment agreed fully in 23/32 (72%). The largest area of discrepancy was object-person fixation shifts, with five children not observed to show this behavior despite its being reported. Findings indicate a structured questionnaire yields description of fixations, which correspond well with clinical assessment. Descriptions supported discussion between parents and clinicians. It is proposed that the FVC-Q is a valuable tool in supporting clinicians in eliciting information about fixation skills.

Incidence, aetiology and neurodisability associated with severe microcephaly: a national surveillance study.

OBJECTIVE: To determine the incidence, causes and neurodevelopmental impact of severe microcephaly (head circumference <-3SD) up to age 2 years. DESIGN: Binational active paediatric surveillance study undertaken in 2017-2018 to identify and characterise new diagnoses of severe microcephaly. SETTING: UK and Ireland. PARTICIPANTS: Infants aged under 12 months at diagnosis. INTERVENTIONS: Observational study. MAIN OUTCOME MEASURES: Incidence, aetiology and neurodevelopmental outcomes at age 2 years. RESULTS: Fifty-nine infants met the case definition, of whom 30 (51%) were girls; 24 (41%) were born preterm (<37 weeks' gestation); and 34 (58%) were of 'white' ethnicity. Eight (14%) children died before 12 months of age. Incidence of severe microcephaly was 5.5 per 100 000 infants (95% CI 4.0 to 7.3). Higher relative risk (RR) was associated with preterm birth (RR 7.7, 95% CI 3.8 to 15.1) and British Asian ethnicity (RR 3.6, 95% CI 1.6 to 7.8). Microcephaly was mainly due to genetic causes (59%), brain ischaemia/hypoxia (10%) and congenital infection (8%), and 19% remained undetermined. Each child was referred on average to eight specialists, and 75% had abnormal brain imaging. By 2 years of age, 55 children experienced neurodevelopmental abnormalities, including feeding problems (68%), motor delay (66%), visual impairment (37%), hearing loss (24%) and epilepsy (41%). CONCLUSIONS: Although severe microcephaly is uncommon, it is associated with high mortality, complex multimorbidity and neurodisability, thus representing a significant ongoing burden for families and healthcare services. Potentially preventable causes include preterm birth, hypoxic/ischaemic brain injury and congenital infections. Clinical guidelines are essential to standardise aetiological investigation and optimise multidisciplinary management.

Avoidable childhood blindness in a high-income country: findings from the British Childhood Visual Impairment and Blindness Study 2.

BACKGROUND/AIMS: Addressing childhood visual disability is an international priority, with data on causes needed to plan, implement and evaluate public health and clinical care. We have examined the contribution of 'avoidable' blinding disorders to childhood visual impairment, severe visual impairment and blindness (VI/SVIBL) in the UK. METHODS: National prospective observational longitudinal study, the British Childhood Visual Impairment and Blindness Study 2 (BCVIS2), of children (aged 18 years or under) newly diagnosed with vision worse than 0.48 logMAR (logarithm of the minimum angle of resolution) or equivalent in both eyes. Proportions of children with an 'avoidable' disorder comprising either a potentially preventable (isolated disorders with an effective intervention which reduces disease incidence) or treatable (isolated eye or vision disorders for which there are routinely available effective interventions able to improve vision or halt progressive visual loss) are reported. RESULTS: Of the 784 children within BCVIS2, isolated potentially preventable disorders were present in only 17% (132/784) and treatable disorders in an additional 13% (99/784). The most common treatable causes were cataract, retinopathy of prematurity and glaucoma. Of the 132 children with potentially preventable disease, 64 had hypoxic-ischaemic encephalopathy. Non-accidental injury accounted for almost two-thirds (11/16, 69%) of those with VI/SVIBL due to injury. CONCLUSION: Despite significant progress in the past decades in high-income countries, there remains a need to be vigilant about implementing preventive strategies and treatments. Attention to disorders that are currently neither preventable nor treatable remains a priority in these settings and will become increasingly important in lower-income and middle-income countries undergoing economic transition.

Development and testing of the eye-pointing classification scale for children with cerebral palsy.

PURPOSE: The aim of this study was to develop and test a new classification scale to describe looking behaviours (gaze fixations and gaze shifts) in relation to eye-pointing. METHODS: The Eye-pointing Classification Scale (EpCS) was developed and tested following established procedures for the construction and evaluation of equivalent scales, and involved 2 phases: Drawing on research literature, Phase 1 involved initial drafting of the scale through a series of multi-disciplinary group discussions; evaluation of the scale through a survey procedure, and subsequent expert group evaluation. Phase 2, was an examination of scale reliability and relationships between child characteristics and level of EpCS classification. RESULTS: In Phase 1, an initial draft of the scale was developed and then evaluated by 52 participants in 10 countries, leading to its refinement. Subsequent expert evaluation of content, style and structure indicated that no further refinement was required. In Phase 2, the scale achieved excellent levels of reliability in clinical testing. A significant relationship was identified between level of child motor ability and EpCS classification, and level of child language understanding and EpCS classification.Implications for rehabilitationNon-speaking children with severe bilateral cerebral palsy who have limited upper limb movement may communicate by using controlled looking behaviours to point to objects and people, referred to as eye-pointing.However, there is little consensus as to which looking behaviours represent eye-pointing and which do not.The Eye-pointing Classification Scale (EpCS) was developed to describe looking behaviours related to eye-pointing in this population of childrenThe EpCS provides a new robust tool for clinical management and research with children with cerebral palsy.

Exploratory Investigation of Brain MRI Lesions According to Whole Sample and Visual Function Subtyping in Children With Cerebral Visual Impairment.

Background: There is limited research on brain lesions in children with cerebral visual impairment (CVI) of heterogeneous etiologies and according to associated subtyping and vision dysfunctions. This study was part of a larger project establishing data-driven subtypes of childhood CVI according to visual dysfunctions. Currently there is no consensus in relation to assessment, diagnosis and classification of CVI and more information about brain lesions may be of potential diagnostic value. Aim: This study aimed to investigate overall patterns of brain lesions and associations with level of visual dysfunction and to compare the patterns between the classification subgroups in children with CVI. Methods: School-aged children with CVI received ophthalmological and neuro-psychological/developmental assessments to establish CVI-related subtyping. Other pediatric information was collected from medical records. MRI scans were coded according to a semi-quantitative template including brain regions (right hemisphere, left hemisphere, visual pathways) and summed for total scores. Non-parametric analyses were conducted. Results: 28 children had clinical brain MRI scans available [44% of total sample, Group A (lower severity of visual dysfunctions) n = 16, Group B (higher severity) n = 12]. Total brain scores ranged between 0 and 18 (Group A mdn = 7, IQR = 0.8-10.0, Group B mdn = 10, IQR = 6.5-11.8) and were widespread across regions. 71 per cent had post-geniculate visual pathway damage. The median total brain and hemisphere scores of Group B were higher than subgroup A but differences did not reach statistical significance. No statistically significant associations were found between brain scores and vision variables (acuity, contrast sensitivity). Conclusion: This study found a spread of lesions across all regions on the brain scans in children with congenital CVI. The majority had damage in the postgeniculate visual pathways and visual cortex region suggesting this is an area of interest and potentially informative for diagnosis. However the subtyping classification did not show differences in number or region of lesions though the trend was higher toward Group B. This study confirms the complex diffuse and variable nature of brain lesions in children with congenital CVI, many of whom have other neurological impairments.

Visual function subtyping in children with early-onset cerebral visual impairment.

AIM: To develop a data-driven subgrouping method to identify and profile subtypes of early-onset childhood cerebral visual impairment (CVI). METHOD: Sixty-three children with suspected or diagnosed congenital CVI were recruited (28 males, 35 females, median age=8y, range=5-16y). Cognitive, basic, and higher-order vision functions were assessed and quality of life, functional vision questionnaire, neurodevelopmental, and ophthalmological data were collected. Cluster analysis and other statistical analyses were undertaken to determine and validate the subgrouping. RESULTS: Forty-three participants completing the full test battery were included in cluster analysis, revealing two subgroups. Group A1 (n=15) showed selective visual perception and visuomotor deficits. Group A2 (n=28) showed more severe and broader visual perception and visuomotor deficits, and variable visual acuity. A third, lower-functioning group, Group B (n=20), was differentiated and showed significant visual acuity reduction compared with Group A (p<0.001, V=0.69). External validation showed significant cooccurring ophthalmological (e.g. strabismus p<0.001, V=0.59) and motor impairment differences (χ2 =16.26, p<0.001, V=0.51) between the three groups. All groups had lowered parent-reported quality of life and everyday functional vision. INTERPRETATION: Statistical analyses revealed three subgroups with differentiated vision function characteristics on a gradient of severity. The subgrouping method provides the first steps in developing a novel classification system to underpin future clinical diagnostics and profiling of early-onset CVI. WHAT THIS PAPER ADDS: Three data-driven subgroups of vision function deficits were identified. A similar severity gradient was shown in cooccurring cognitive and neurodevelopmental deficits. Reported quality of life and functional vision difficulties were low across all groups.

Is there consensus in defining childhood cerebral visual impairment? A systematic review of terminology and definitions.

The childhood condition of visual difficulties caused by brain damage, commonly termed cortical or cerebral visual impairment (CVI), is well established but has no internationally accepted definition. Clarification of its core features is required to advance research and clinical practice. This systematic review aimed to identify the definitions of childhood CVI in the original scientific literature to describe and critically appraise a consensual definition of the condition. MEDLINE, EMBASE, PsychINFO, CINAHL and AMED databases were searched in January 2017. Studies were included if they (1) were published original research, (2) contained a childhood CVI sample, (3) contained a definition of CVI and (4) described their CVI identification/diagnostic method. Thematic analysis identified concepts within definitions and narrative synthesis was conducted. Of 1150 articles, 51 met inclusion criteria. Definitions were subdivided according to detail (descriptive definition, description not reaching definition status and diagnostic/operationalising criteria). Three themes concerning visual deficits, eye health and brain integrity were identified (each containing subthemes) and analysed individually across definitions. The most common themes were 'visual impairment' (n=20), 'retrochiasmatic pathway damage'(n=13) and 'normal/near normal eye health' (n=15). The most consensual definition identified here may not be the best quality for advancing our understanding of CVI. We argue for the alternative definition: CVI is a verifiable visual dysfunction which cannot be attributed to disorders of the anterior visual pathways or any potentially co-occurring ocular impairment. We propose reporting guidelines to permit comparison across studies and increase the evidence base for more reliable clinical assessment and diagnosis.

Fifteen-minute consultation-the child with a developmental disability: is there an ocular or visual abnormality?

OBJECTIVE: To present a structured approach for an outpatient consultation for a child with developmental disability who may have an ocular or visual disorder. METHOD: Review of relevant literature and description of the approach to ocular and visual assessment which could be used by any paediatrician. CONCLUSION: A systematic approach to history, observation and examination of a child with a developmental disability will assist in identifying a possible visual problem. A structured referral letter will ensure that the child will receive the most appropriate assessment to clarify the problem and appropriate management in the eye clinic.

Management of children and young people with vision impairment: diagnosis, developmental challenges and outcomes.

Vision impairment (VI) has a significant impact on an individual's ability to engage with everyday tasks. Severe early-onset VI presents different challenges to adult onset visual loss since reduced visual input presents a major obstacle to the acquisition and development of fundamental developmental skills in early and later childhood. Early referral of the child with reduced vision is vital to ensure accurate diagnosis and prompt treatment of any modifiable aspects of the condition. Guidance to ensure optimisation of developmental opportunity through adaptation of play and care is vital from the earliest stages. Cognitive and social challenges occur throughout education and need to be understood and addressed through skilled support. Multiagency input, particularly from health and education, is vital to minimise the risk of adverse outcomes and promote successful transition to independent adulthood. VI may also arise in a variety of neurological disorders, and diagnosis and assessment to ensure appropriate adaptations are made is also crucial for this group of children. This review provides the paediatrician with information on diagnosis, assessment, long-term support needs and outcomes.

Common visual problems in children with disability.

Children with disability are at a substantially higher risk of visual impairment (VI) (10.5% compared with 0.16%) but also of ocular disorders of all types, including refractive errors and strabismus. The aetiology of VI in children with disability reflects that of the general population and includes cerebral VI, optic atrophy, as well as primary visual disorders such as retinal dystrophies and structural eye anomalies. VI and other potentially correctable ocular disorders may not be recognised without careful assessment and are frequently unidentified in children with complex needs. Although assessment may be more challenging than in other children, identifying these potential additional barriers to learning and development may be critical. There is a need to develop clearer guidelines, referral pathways and closer working between all professionals involved in the care of children with disability and visual disorders to improve our focus on the assessment of vision and outcomes for children with disability.

Use of eye-pointing by children with cerebral palsy: what are we looking at?

BACKGROUND: Children with cerebral palsy often show significant communication impairment due to limited or absent speech. Further, motor impairment can restrict the use of movement, including pointing, to signal interest and intent. For some children, controlled gaze can be an effective 'point-substitute': such 'eye-pointing' can be used to request items, establish mutual interest in an event, or select vocabulary within an alternative or augmentative communication (ACC) system. However, in clinical practice there is a lack of clarity about how the term 'eye-pointing' is used, how 'eye-pointing' is recognized or how it relates to social development. AIMS: To present a clinical description of the term 'eye-pointing' with reference to children with severe cerebral palsy who cannot speak or finger-point. To consider this description within a wider discussion of the importance of gaze in communication development. METHODS & PROCEDURES: Cumulative clinical observations during assessment of children referred to a specialist multidisciplinary communication clinic have provoked discussion between the authors on what factors precipitate use of the term 'eye-pointing' in young children with severe cerebral palsy. In particular, discussion has centred on whether use of the term is appropriate in individual cases and whether guidance is available about how gaze should be observed in this developmentally vulnerable group of children. A literature search was also conducted in order to explore whether the use and meaning of the term is established. CONCLUSIONS & IMPLICATIONS: In interactions with non-speaking children, determining whether a child is using eye-gaze communicatively requires observation and interpretation of several factors. These processes will be informed by reflection on what is known about other aspects of the child's communication and interaction skills. Within the literature, the term 'eye-pointing' is sometimes used when describing the communication functions of individuals using augmentative and alternative communication (AAC) systems, and is occasionally qualified by a definition. No papers have been found that set out a clinical description universally applicable to children with severe motor impairment. Moreover, guidance is lacking on how possible episodes of 'eye-pointing' might be confidently distinguished from other episodes of directed gaze in young, developing communicators. The discussion of the term makes reference to the importance of gaze in early communication development, and explores factors that might influence gaze and its interpretation in young children with cerebral palsy. A description of eye-pointing for this group is offered. The authors suggest that this will bring practical benefits to those supporting the communication development of children with severe cerebral palsy.