Cerebral Visual Impairment In Children Research Articles

Expert consensus on the definition, diagnosis, assessment and intervention of cerebral visual impairment in children(2023)

Expert consensus on the definition, diagnosis, assessment and intervention of cerebral visual impairment in children(2023)

Visual Function and Neuropsychological Profile in Children with Cerebral Visual Impairment.

Cerebral Visual Impairment (CVI) has become the leading cause of children’s visual impairment in developed countries. Since CVI may negatively affect neuropsychomotor development, an early diagnosis and characterization become fundamental to define effective habilitation approaches. To date, there is a lack of standardized diagnostic methods to assess CVI in children, and the role of visual functions in children’s neuropsychological profiles has been poorly investigated. In the present paper, we aim to describe the clinical and neuropsychological profiles and to investigate the possible effects of visual functions on neuropsychological performance of a cohort of children diagnosed with CVI. Fifty-one children with CVI were included in our retrospective analysis (inclusion criteria: verbal IQ > 70 in Wechsler scales; absence of significant ocular involvement). For each participant, we collected data on neuropsychological assessment (i.e., cognitive, cognitive visual, and learning abilities), basic visual functions (e.g., Best Corrected Visual Acuity—BCVA, contrast sensitivity, and ocular motor abilities) and global development features (e.g., neurological signs and motor development delay) based on standardized tests, according to patients’ ages. The results showed that oculomotor dysfunction involving saccades and smooth pursuit may be a core symptom of CVI and might have a significant impact on cognitive visual and other neuropsychological abilities. Furthermore, visual acuity and contrast sensitivity may influence cognitive, cognitive visual, and academic performances. Our findings suggest the importance of a comprehensive assessment of both visual and neuropsychological functions in children when CVI is suspected, which is needed to provide a more comprehensive functional profile and define the best habilitation strategy to sustain functional vision.

Age-Related Effects on the Spectrum of Cerebral Visual Impairment in Children With Cerebral Palsy.

BackgroundCerebral Visual Impairment (CVI) is a very common finding in children affected by Cerebral Palsy (CP). In this paper we studied the characteristics of CVI of a large group of children with CP and CVI, describing their neurovisual profiles according to three different age subgroups (subgroup 1: infants 6 months–2 years; subgroup 2: pre-school age 3–5 years; subgroup 3: school age ≥ 6 years).MethodsWe enrolled 180 subjects (104 males, mean age 66 ± 42.6 months; range 6–192 months) with CP and CVI for the study. We carried out a demographic and clinical data collection, neurological examination, developmental or cognitive assessment, and a video-recorded visual function assessment including an evaluation of ophthalmological characteristics, oculomotor functions, and basic visual functions. In school-aged children, we also performed an evaluation of their cognitive-visual profiles.ResultsThere were signs of CVI in all the three subgroups. Subgroup 1 (62 children) and subgroup 2 (50 children) were different for fixation (p = 0.02), visual acuity (p = 0.03) and contrast sensitivity (p < 0.01), being more frequently impaired in younger children. Comparing subgroup 2 with subgroup 3 (68 children), the older children presented more frequently myopia (p = 0.02) while the younger ones esotropia (p = 0.02) and alteration in smooth pursuit (p = 0.03) and saccades (p < 0.01). Furthermore, fixation, smooth pursuit, visual acuity, contrast sensitivity and visual filed (p < 0.01) were more frequently impaired in younger children (subgroup 1) compared to the older ones. Multiple correspondence analysis (MCA) confirmed the different neurovisual profiles according to age: younger children with CP showed more signs of CVI compared to the older ones. 34 out of 68 children belonging to subgroup 3 underwent the cognitive visual evaluation; an impairment of cognitive visual skills was detected in 21 subjects.ConclusionYounger children with CP showed more signs of CVI compared to the older ones, likely for the physiological maturation of visual system and mechanisms of neuroplasticity. In this direction, we suggest an early neurovisual evaluation to detect any weak visual functions.

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.

Current practice used by therapists to screen and assess cerebral visual impairment in children with cerebral palsy in the South African setting

Current practice used by therapists to screen and assess cerebral visual impairment in children with cerebral palsy in the South African setting

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

PurposeCerebral 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.MethodsMedline, 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.ResultsOf 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.ConclusionThere 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.

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

To develop a data-driven subgrouping method to identify and profile subtypes of early-onset childhood cerebral visual impairment (CVI). 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. 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. 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. 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.

Cerebral visual impairment in children: the importance of classification

This commentary is on the original article by Sakki et al. on pages 303–312 of this issue.

Cerebral visual impairment captured with a structured history inventory in extremely preterm born children aged 6.5 years

To investigate whether a questionnaire can identify cerebral visual impairment (CVI) in a group of 6.5-year-old children born extremely preterm (EPT) as accurately as direct assessments. This prospective population-based study included 120 children born before 27weeks' gestational age (66 males; mean, 25.4±1.0weeks) and 97 full-term controls (56 males; mean, 39.9±1.1weeks) at the age of 6.5years, as part of the Extremely Preterm Infants in Sweden Study (EXPRESS). A questionnaire for detection of CVI was evaluated and compared with visual, perceptual, and cognitive assessments. Parents of children born EPT reported more CVI features than the parents of control children, with median sum scores of 25 (95% CI, 18.1-31.9) and 11 (95% CI, 8.8-13.2), respectively (P<0.001), and a median difference of 14 (95% CI, 6.6-21.4). Low rates of reported CVI features were significantly associated with better results from direct assessments within the EPT group and with less pronounced differences compared to controls. The questionnaire discriminated well between children born EPT and controls, and the scores were congruent with other evidence of visual, perceptual, and cognitive deficits. The easily used questionnaire compared favorably with direct assessment in identifying CVI in children born EPT and also provides valuable information to clinicians, and parents about the daily life problems associated with CVI.

Cerebral visual impairment in children

Cerebral visual impairment has gained importance due to increased survival of extremely premature babies. It encompasses visual processing dysfunctions due to disorders of the retrochiasmal pathways and visual association areas. This review describes the physiology of visual processing, clinical approach to children with Cerebral Visual Impairment, and principles of intervention. Functional vision assessment and vision intervention strategies are given emphasis. We have to ensure that these children are able to use vision for learning, for social interaction, and mobility. A literature review was undertaken using Medline and PubMed.

The progress and prospect of ophthalmological research on cerebral visual impairment in children

With the rapid development of global perinatal medicine and neonatal medicine, the survival rate of high-risk neonates (premature, and those who suffer neurologic lesion during or shortly after birth, et al.) improved significantly, however the incidence of cerebral visual impairment (CVI) also rose, which has become the most common cause of visual impairment in children in developed countries. Studies found that visual abnormalities of patients with CVI can be various. Since children's cognition and motor development are inseparable from visual functions, children with CVI are usually characterized by abnormalities in sensory perception, cognition and even movement, other than visual impairment itself. Due to the characteristics of CVI, such as early onset, complex etiology, difficulty in diagnosis and treatment, and involvement with ophthalmology, pediatrics, rehabilitation medicine, genetic epidemiology and other multi-disciplinary content, current domestic research on CVI is limited. From the perspective of ophthalmologist, this paper reviews the progress of ophthalmology diagnosis and treatment in children with CVI in recent years, aiming to have better early recognition and individualized intervention, so as to help pediatrician and rehabilitation physicians to improve survival skills for CVI children and their quality of life. (Chin J Ophthalmol, 2019, 55:469-474).

Ganglion Cell Topography Indicates Pre- or Postnatal Damage to the Retro-Geniculate Visual System, Predicts Visual Field Function and May Identify Cerebral Visual Impairment in Children – A Multiple Case Study

ABSTRACTIn this paper, we quantify the degree of ganglion cell layer thinning due to retrograde trans-synaptic degeneration (RTSD) from retro-geniculate damage in six cases who had homonymous visual field defects known since childhood. Three had prenatal injuries, occurring close to mid-gestation and in the first parts of the early and late third trimester, respectively, and representing injuries at different early developmental stages. Three had later acquired injuries, at age 1.5, 4 and 13 years. The impact of the injury to the optic radiations was revealed by fibre tractography. The ganglion cell thinning corresponded with the visual field defects and the extent and location of the primary brain damage. The most important sign of RTSD was asymmetry of the ganglion cell topography within the macular area.

Cerebral Visual Impairment in Children

Cerebral visual impairment (CVI) is vision loss as a result of damage to the retrogeniculate pathway of the visual system in the absence of any ocular pathology and with the improvement of perinatal care and increased survival of the preterm babies, it accounts for the leading cause of visual impairment today. Perinatal hypoxic ischemic encephalopathy has been the most common cause of CVI. Morphology of lesion due to hypoxic ischemic injuries to the retrogeniculate visual pathways varies with the timing of the event and the mechanism. Injury during the first trimester results in congenital malformations. Liquefaction necrosis and tissue resorption without any gliotic changes are the major pathological changes. However, damage during the late second to third trimester causes periventricular leukomalacia from ischemic damage followed by gliosis of the subcortical areas. A careful assessment of functional vision as well as level of visual acuity is the first and foremost step in managing these children. Rehabilitation strategies form the backbone of complete management of children with CVI. Every child needs different and special services based on their functional visual and neurological deficit. A multidisciplinary approach is always the mainstay of treatment. Nevertheless, parental education and their cooperation optimises the management outcomes in children with cerebral visual impairment. Prompt diagnosis and timely intervention in terms of neurological, ophthalmological and rehabilitation services always has a rewarding result for children with CVI and their parents.

Cerebral visual impairment in children: Causes and associated ophthalmological problems.

Purpose:The aim of this study is to identify common causes, associated ophthalmological abnormalities, and systemic comorbidities in children in Andhra Pradesh, India, with cerebral visual impairment (CVI).Methods:A retrospective review of case records of all children aged <16 years with diagnosis of CVI seen between January 2016 and December 2016 was carried out. Data were collected for their age, gender, cause of CVI, refraction, accommodation, anterior and posterior segment examination findings, and systemic problems.Results:A total of 124 patients were identified and studied (80 boys and 44 girls, mean age 5.23 years, 44.8% aged <2 years). The most common causes of CVI were hypoxic–ischemic encephalopathy (HIE) (34.4%), undetermined etiology (32.8%), neonatal seizures, and infantile spasms (16% each). The most common presenting complaints were poor vision (76%) and squint (11.2%). Profound visual impairment was seen in 88.8%, and 11.2% had high functioning CVI. Fifty-eight (46.4%) patients had significant refractive errors, 40 (32.25%) had strabismus, 4 (3.2%) had visually significant cataract, and 40 (32%) had optic atrophy. Motor delay was observed in 39.5%, speech delay was evident in 22.4%, and cognitive delay in 16%.Conclusion:HIE is the most common cause (one-third) of CVI in our population, and the majority of them presented at age <2 years (44.8%) with profound visual impairment (88.8%). A significant number of them have treatable ophthalmic conditions such as refractive errors (46.4%), accommodative insufficiency (12.1%), and cataract (3.2%), and more than one-third of them also have delay in other areas of development.

Profile of Cerebral Visual Impairment in Children with Cerebral Palsy at a Tertiary Care Referral Center in Southern India

Profile of Cerebral Visual Impairment in Children with Cerebral Palsy at a Tertiary Care Referral Center in Southern India

“I See Something You Don’t See...”: Cerebral Visual Impairment in Children with Cerebral Palsy

“I See Something You Don’t See...”: Cerebral Visual Impairment in Children with Cerebral Palsy

Book Review: Vision and the Brain: Understanding Cerebral Visual Impairment in Children

Book Review: Vision and the Brain: Understanding Cerebral Visual Impairment in Children

The relationship between visual orienting responses and clinical characteristics in children attending special education for the visually impaired.

We recently introduced a method based on quantification of orienting responses toward visual stimuli to assess the quality of visual information processing in children. In the present study, we examined the relationship between orienting responses and factors that are associated with visual processing impairments in current clinical practice. Response time and fixation quality to visual features such as form, contrast, motion, and color stimuli were assessed in 104 children from 1 to 12 years attending special education for the visually impaired. Using regression analysis, we investigated whether these parameters were affected by clinical characteristics of children. Response times significantly depended on stimulus type. Responses to high-contrast cartoons were significantly slower in children with a clinical diagnosis of cerebral visual impairment. Fixation quality was significantly affected by visual acuity and nystagmus. The results suggest that the quantitative measurement of orienting responses is strongly related to cerebral visual impairment in children.

Cross-sectional study on childhood cerebral visual impairment in New Zealand

To determine the prevalence, etiology, and avoidable causes of childhood cerebral visual impairment (CVI) in New Zealand. The clinical and educational records of blind and low vision children enrolled in the Blind and Low Vision Education Network, New Zealand (BLENNZ), a national referral center, were retrospectively analyzed. The WHO Program for Prevention of Blindness (WHO/PBL) Eye Examination Record for Children with Blindness and Low Vision was used to record data from children ≤16 years of age diagnosed with CVI and visual acuity ≤6/18 enrolled with BLENNZ. Data analyzed included demographics, etiology, visual acuity, visual fields, educational setting, and rehabilitation plan. A total of 182 children (blind, 143; low vision, 39) were included. The calculated prevalence of childhood CVI in New Zealand was 0.02%. Of these, only 21% required low vision aids. Principle causes of CVI blindness were perinatal hypoxia/asphyxia (25%), nonaccidental injury (7%), and prematurity (7%). Approximately 50% of all cases of CVI blindness were potentially avoidable; of these, 52% were caused by perinatal hypoxia and 14% by nonaccidental injury. The conservative calculated prevalence of CVI, responsible for 30% of all childhood blindness in New Zealand, was 0.02%. The most common cause of CVI blindness in New Zealand, perinatal asphyxia, is also an avoidable cause.

The spectrum of cerebral visual impairment as a sequel to premature birth: an overview

In children born prematurely, impairment of vision due to damage to the brain is more common than due to retinopathy of prematurity. Yet, the diagnosis of cerebral visual impairment may be missed. The subject of cerebral visual impairment in children is reviewed in order to explain and draw attention to the types of visual deficits and behaviours that may result as a sequel to premature birth. A wide range of sources of data has been employed to assemble this overview. The principal reference source is PubMed. The material presented highlights the origin and range of visual deficits that result from damage to the brain, related to premature birth. Deficits of primary visual functions, perceptual dysfunction, simultanagnostic visual disorders and impaired visual guidance of movement (optic ataxia), as well as disorders of visual attention and memory, can occur in a variety of combinations and degrees. The resulting behavioural outcomes are described. Identification and characterisation of impaired vision, due to prematurity associated damage to the brain, are essential. This is required so as to ensure that affected children are not inappropriately disadvantaged on account of the diagnosis being missed or inadequately acted upon, but instead, they are managed optimally, both at home and at school, so that their development is enhanced to the greatest advantage.