The effect of visual support strategies on the quality of life of children with cerebral palsy and cerebral visual impairment/perceptual visual dysfunction in Nigeria: study protocol for a randomized controlled trial.

BACKGROUND: Cerebral visual impairment (CVI), including perceptual visual dysfunction (PVD), is common in children with cerebral palsy (CP). Inventories of questions relating to practical aspects of visual perception in everyday life, in particular the closed-ended Insight Questions Inventory (IQI), can be used to assess CVI/PVD. Studies linking responses to the inventory with specific visual support strategies, aimed at modifying the child's environment and/or behaviour to minimize the impact of the CVI/PVD, have been piloted. The IQI and tailored strategies have not been used in an African population, nor have they been tested in a controlled trial. This trial will compare the effectiveness of the IQI and linked visual support strategies versus general supportive treatments on the quality of life of children with CVI/PVD and CP through a randomized controlled trial. METHODS/DESIGN: This is a prospective, double-blind, parallel-arm, randomized controlled trial. The primary outcome is change in quality of life scores between the two arms of the trial at 6 weeks, assessed using the Paediatric Quality of Life Inventory (PedsQL) generic 4.0 and CP 3.0 module. All children will undergo baseline assessment including the Open Questions Inventory, IQI, PedsQL 3.0, PedsQL 4.0 generic, and the Strengths and Difficulties Questionnaire (SDQ). Eligible children with CP aged 4 years to < 16 years will be stratified and blocked by the age groups 4-9 and 10 to < 16 years and by Gross Motor Function Classification System (GMFCS) levels 1-3 and 4-5. Families in the intervention arm will receive tailored insight visual support strategies and telephone calls during the 6-week trial period. The control arm will receive standard treatment and the intervention after the 6-week trial period. Follow-up interviews will be performed in both arms at 6 weeks with a repeat administration of the PedsQL CP 4.0 and 3.0, the IQI and the SDQ. Secondary outcomes include a change in functional vision. DISCUSSION: This randomized controlled trial will provide evidence of the effectiveness of this intervention for children with CP in a resource-poor setting. TRIAL REGISTRATION: Pan African Clinical Trials Registration, PACTR201612001886396 . Registered on 3 December 2016

Clinical assessment, investigation, diagnosis and initial management of cerebral visual impairment: a consensus practice guide

Cerebral Visual Impairment (CVI) is a common condition in the UK. Patients with conditions associated with CVI are frequently seen in paediatric ophthalmology clinics offering eye care professionals an opportunity to identify children proactively. In most cases CVI occurs as part of a neurodevelopmental condition or as a feature of multiple and complex disabilities. However, CVI can also be seen in children with apparently typical development. In some cases, high contrast visual acuity is normal and in other cases severely impaired. As such, identification of CVI requires evaluation of aspects of visual performance beyond high contrast acuity and consideration that visual function of those with CVI may fluctuate. Few paediatric ophthalmologists have received formal training in CVI. The detection and diagnosis of CVI varies across the UK and patients report hugely different experiences. A diagnosis of CVI is made based on professional clinical judgement and it is recognised that individual perspectives and local practice in the specific methodologies of assessment will vary. A systematic review and survey of professionals is underway to attempt to reach agreement on diagnostic criteria. Nonetheless, established pathways and published protocols can offer guidance on how a paediatric ophthalmology service can approach assessment of the child with suspected CVI. The purpose of this paper is to present a summary of research and clinical practice methods for detecting and diagnosing CVI in a paediatric ophthalmology outpatient setting. It represents current understanding of the topic and acknowledges the evolving nature of both practice and the evidence-base. A rapid literature review was undertaken to identify articles relating to clinical investigation of children with CVI. A focus group of QTVI and subject matter experts from sight loss charities was undertaken to address areas which were not covered by the literature review

Behavioural features of cerebral visual impairment are common in children with down syndrome

It is widely recognised that children with Down syndrome have a broad range and a high prevalence of visual deficits and it has been suggested that those with Down syndrome are more likely to exhibit visual perception deficits indicative of cerebral visual impairment. This exploratory study aims to determine the prevalence of behavioural features suggestive of cerebral visual impairment (CVI) occurring with Down syndrome and whether the visual problems can be ascribed to optometric factors. A cohort of 226 families of children with Down syndrome (trisomy 21), aged 4–17, were invited to participate in a validated question inventory, to recognise visual perception issues. The clinical records of the participants were then reviewed retrospectively. A five-question screening instrument was used to indicate suspected CVI. The majority of the 81 families who responded to the questionnaire reported some level of visual perceptual difficulty in their child. Among this cohort, the prevalence of suspected CVI as indicated by the screening questionnaire was 38%. Only ametropia was found to have a significant association with suspected CVI, although this increased the correct prediction of suspected CVI outcome by only a small amount. Results suggest that children with Down syndrome are more likely to experience problems consistent with cerebral visual impairment, and that these may originate from a similar brain dysfunction to that which contributes to high levels of ametropia and failure to emmetropise. It is important that behavioural features of CVI are recognised in children with Down syndrome, further investigations initiated and appropriate management applied.V. Vinuela-Navarro and R. England were funded by Action Medical Research for Children GN2338.Peer ReviewedPostprint (author's final draft

Neurocognitive findings in adults who played youth football

Chronic Traumatic Encephalopathy (CTE) has been linked to contact sports, most notably boxing and American football, due to their propensity for repetitive head impacts. Concerns in the community for the safety of athletes in all contact sports has driven a significant amount of research into concussions, their long term effects, and strategies for treatment and prevention. Knowledge of long term brain health in response to neurotrauma is limited, a gap especially noticeable in the literature on non-catastrophic brain injuries sustained as a child. Concussion is a common injury that is often self-resolving with no lasting neurologic or cognitive deficits. Although repetitive brain trauma is hypothesized to be necessary and sufficient to lead to CTE, no human or animal models have definitively demonstrated the pathophysiologic connection or confirmed the mechanism of symptoms. The research to date has been case based, lacking prospective cohorts, with data complicated by convenience sampling. These factors limit the generalizability of conclusions. CTE is neuropathologically defined with variable symptoms; however, it is only diagnosable at postmortem autopsy making the etiology and prevalence difficult to understand. As more research is published to understand if there is an association between a neurocognitive degenerative disease and contact sports, the concentration is on professional athletes. Yet professional athletes do not represent the overwhelming majority of all contact sport participants. The proposed study will compare adults who participated in youth football, but not beyond the high school level, to a control group of adults who did not play contact sports. Evaluating their cognitive function with an online assessment, the Behavior Rating Inventory of Executive Function – Adult Version (BRIEF-A), data will be analyzed for signs of clinical cognitive impairment. The objective is to measure adults who represent the high percentage of youth football players who do not continue to the advanced levels. Data obtained from this study will help communities make informed decisions, and create the foundation for future studies on long term benefits and risks of contact sports for children

The CVI practice framework : an effective approach to supporting children with cerebral visual impairment (CVI) : a thesis presented in partial fulfilment of the requirements for the degree of Doctor of Philosophy in Education at Massey University, Manawatū, Aotearoa New Zealand

Cerebral visual impairment (CVI) is the most common cause of visual impairment affecting children in the economically developed world, with the prediction that numbers will continue to rise with continued advancements in medical care for neonates. Despite this increase, many children are still being supported with approaches that have been developed for children with ocular visual impairments, as there are limited evidence-based approaches for supporting children with CVI. This thesis by publication therefore proposed a CVI practice framework to help meet the specific needs of children with CVI that can be used within an education or habilitation/rehabilitation context. The framework was based on the researcher’s own experience of developing a successful rehabilitation programme to improve her visual and overall functioning following a late CVI diagnosis. From this programme, the researcher identified three main components that led to the improvements in her quality of life. These included the development of an individual CVI profile, an individualised programme, and empowerment through the attainment of knowledge. To ascertain whether a similar approach could be effective for children with CVI, the researcher conducted a number of different research projects to assess the effectiveness of each individual component and the overall framework. Results from the different research projects show that, firstly, the Austin Playing Card Assessment has the potential to be an effective tool to detect visual perceptual difficulties related to clutter and can be used as part of a wider assessment process to develop a child’s CVI profile. Secondly, there are a number of generic strategies that can be implemented within a child’s programme to support their visual, emotional and behavioural needs. Thirdly, information at the time of a child’s diagnosis helps to empower parents and leads to them being more involved in the services their child receives. Finally, the CVI practice framework has the potential to be an effective approach for supporting children with CVI. Further research on the three components of the framework and the overall framework is needed to corroborate these findings

Understanding Mother’s Perceptions on Assessment And Educational Programming for Their Children With Cerebral Visual Impairment

Brain-based visual impairment is the leading cause of visual impairment in early childhood populations in the developed world and its contribution to childhood visual impairment is increasing (Bosch et al., 2014; Dutton & Bax, 2010; Hoyt, 2007; Kong et al., 2012; Kran et al., 2019). In order to meet the needs of this population, comprehensive assessment that includes information from families and caregivers, pediatric ophthalmologists, neurodevelopmental specialists, vision professionals, and education teams is crucial for academic and functional success (Lueck & Dutton, 2015). Family input during the special education process and the transfer of knowledge from the clinical to the educational setting that the parent provides is also vital for this population. While parent participation is a mandated feature of special education programming development, parents of children with special needs (including those with visual disability) do not always participate to the extent the law presumes. The purpose of this qualitative multiple case study was to examine the experiences of 3 participants who are mothers of children with this visual impairment, bounded together by their shared experiences at a large children’s hospital in a city in the United States and participation in their children’s special education programming. The researcher explored the experiences of participants in both the clinical and educational environments and ultimately their feelings on the adequacy and effectiveness of their children’s educational programming relative to their visual disability. The results from this study revealed valuable information on the multitude of roles that mothers play across the physical, social, and emotional spaces in the lives of their children. Since comprehensive assessment and specific programming is crucial for the success of students with CVI, the results of this study helped to construct a more comprehensive picture of the outcomes of clinical and educational assessment and opportunities for collaboration with parents and families of children with CVI. It also provided a better understanding of the challenges families face, lack of resources on CVI, and a lack of qualified personnel in the field

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