What assessments are currently used to investigate and diagnose cerebral visual impairment (CVI) in children? A systematic review

Abstract

Purpose Cerebral visual impairment (CVI) is the leading cause of childhood visual impairment in the developed world. Despite this, there are no agreed clinical guidelines for the investigation and diagnosis of the condition. Before development of such guidelines can commence, it is important to recognise which approaches are currently employed. This systematic review evaluated the literature to identify which methods of assessment are currently used to investigate and diagnose childhood CVI. Methods Medline, Embase, CINAHL, Scopus and the Cochrane Library databases were systematically searched in January 2020 using defined search terms. Articles were included if they: (i) were research papers, conference abstracts or research protocols published in peer‐reviewed scientific journals, or relevant textbooks; (ii) included a clinical investigation of CVI in children; (iii) provided an explanation or criteria to diagnose CVI and (iv) were specifically investigating cerebral/cortical visual impairment. Methods used to a) assess and b) diagnose CVI were extracted from included articles. ‘Assessment scores’ were assigned for each method employed by researchers to investigate and diagnose CVI to quantify and compare approaches between articles. A quality grading was also applied to each article. Results Of 6454 identified articles, 45 met the inclusion criteria. From these, 10 categories of assessment utilised within included articles were identified: (1) Medical history, (2) Vision assessment/ophthalmologic examination, (3) Neuroimaging, (4) Visual behaviour and direct observation, (5) Structured history‐taking, (6) Visual perception tests, (7) Ocular movement and posture assessment, (8) Intelligence/IQ assessment, (9) Clinical electrophysiology and (10) Neurodevelopmental tests. In terms of diagnostic criteria, the most commonly reported approach was one of exclusion, i.e., CVI was diagnosed when visual dysfunction could not be attributed to abnormalities detected in the anterior visual pathway. Conclusion There is a lack of common practice in the approaches used by clinicians to investigate and diagnose CVI in children. At present, a ‘diagnosis of exclusion’ remains the most common means to diagnose CVI. Development of clinical guidelines for assessment and diagnosis are necessary to ensure consistency in the diagnosis of CVI and the timely implementation of support to alleviate the impact of CVI on the child’s daily living.

Cortical Visual Impairments and Learning Disabilities

“…The neuropsychological approach combined with structured history taking for CVI allows us to finely describe visual function disorders as well as to characterize their deleterious effect on cognitive, social and motor development. Optimal management (that we are not covering here) is founded on this finely profiled description, and aims at truly enhancing all the capacities of detection, discrimination, analysis, memory, and visual attention, as well as the processes involved in the mental organization and representation of space ( Zihl and Dutton, 2015 ; Chokron, 2018 ; Chang and Borchert, 2020 ; McConnell et al, 2021 ), complemented by skilled teaching of parents and teachers about the unique visual difficulties of each child, and the salient actions that they need to take. Future research in this field will aim to standardize both assessment and management, tenable collection of comprehensive data on the subject, and dissemination of diagnostic and rehabilitative methodologies for the dynamic assessment and management of CVI to bring about optimal learning and development.…”

“…Indeed, parents of a child with ophthalmologic visual dysfunctioning are warned of future visual difficulties at an early stage and can adapt their behavior accordingly, by using auditory or haptic modalities instead of visual ones. On the contrary, in the child with a CVI, lack of knowledge of the disorder by the medical profession, the family and the child herself, does not allow the stakeholders to interpret the child’s particular behavior in terms of a potential visual cause, and therefore take appropriate action to cater for the causative visual disabilities ( McConnell et al, 2021 ).…”

“…It therefore seems essential to be able to search early and systematically for CVIs in at risk children, in order to be able to treat them as quickly as possible, and avoid the occurrence of interaction and/or cognitive and/or behavioral disorders ( Lueck and Dutton, 2015b ; McConnell et al, 2021 ). The ability to make the best possible differential diagnosis between CVI and autism would also help to identify the most appropriate targeted intervention for each child, to bring about salient school adaptations, while providing useful parental guidance to optimally stimulate and teach these children as effectively as possible ( Fazzi et al, 2019 ).…”

“…The situation for children with additional associated neurological or ophthalmological disorders is equally problematic ( West et al, 2021 ). In particular, the visual pathology can be “the tree that hides the forest.” In this scenario, the child who is already being followed-up for ophthalmic disorders, may not necessarily be subject to a complementary neurovisual assessment ( Ego et al, 2015 ; Lueck and Dutton, 2015b ; Chang and Borchert, 2020 ; McConnell et al, 2021 ). The diagnosis of CVI is crucial, as this can impact the child’s whole development and their future, because the diagnosis can be confused with other conditions, thereby delaying or even preventing appropriate care.…”

“…The diagnostic approach includes in-depth structured history taking, precise visual assessment and regular evaluation of those affected to identify the condition, assess the evolution and best adapt management to cater for specific needs at school ( Ysseldyke et al, 2009 ; Salvia et al, 2013 ; Chang and Borchert, 2020 ). The assessment process thus varies according to the goal, to identify and characterize other disorders, implement targeted interventions, and make decisions concerning the provision of optimal appropriate educational or vocational services ( McConnell et al, 2021 ).…”