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Background: Cerebral visual impairment (CVI) is a common sequala of early brain injury, damage, or malformation and is one of the leading individual causes of visual dysfunction in pediatric populations worldwide. Although patients with CVI are heterogeneous both in terms of underlying etiology and visual behavioural manifestations, there may be underlying similarities in terms of which white matter pathways are potentially altered. This exploratory study used diffusion tractography to examine potential differences in volume, quantitative anisotropy (QA), as well as mean, axial, and radial diffusivities (mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD), respectively) focusing on the dorsal and ventral visual stream pathways in a cohort of young adults with CVI compared to typically sighted and developing controls. Methods: High angular resolution diffusion imaging (HARDI) data were acquired in a sample of 10 individuals with a diagnosis of CVI (mean age = 17.3 years, 2.97 standard deviation (SD), range 14–22 years) and 17 controls (mean age = 19.82 years, 3.34 SD, range 15–25 years). The inferior longitudinal fasciculus (ILF), inferior fronto-occipital fasciculus (IFOF), vertical occipital fasciculus (VOF), and the three divisions of the superior longitudinal fasciculus (SLF I, II, and III) were virtually reconstructed and average tract volume (adjusted for intracranial volume), MD, AD, and RD were compared between CVI and control groups. As a secondary analysis, an analysis of variance (ANOVA) was carried out to investigate potential differences based on etiology (i.e., CVI due to periventricular leukomalacia (CVI-PVL) and CVI due to other causes (CVI-nonPVL)). Results: We observed a large degree of variation within the CVI group, which minimized the overall group differences in tractography outcomes when examining the CVI sample as a unitary group. In our secondary analysis, we observed significant reductions in tract volume in the CVI-PVL group compared to both controls and individuals with CVI due to other causes. We also observed widespread significant increases in QA, MD, and AD in CVI-PVL compared to the control group, with mixed effects in the CVI-nonPVL group. Conclusions: These data provide preliminary evidence for aberrant development of key white matter fasciculi implicated in visual perceptual processing skills, which are often impaired to varying degrees in individuals with CVI. The results also indicate that the severity and extent of the white matter changes may be due in part to the underlying cause of the cerebral visual impairments. Additional analyses will need to be done in a larger sample alongside behavioural testing to fully appreciate the relationships between white matter integrity, visual dysfunction, and associated causes in individuals with CVI.

An estimated 14 million of the world’s children are blind. A blind child is more likely to live in socioeconomic deprivation, to be more frequently hospitalised during childhood and to die in childhood than a child not living with blindness. This update of a previous review on childhood visual impairment focuses on emerging therapies for children with severe visual disability (severe visual impairment and blindness or SVI/BL).For children in higher income countries, cerebral visual impairment and optic nerve anomalies remain the most common causes of SVI/BL, while retinopathy of prematurity (ROP) and cataract are now the most common avoidable causes. The constellation of causes of childhood blindness in lower income settings is shifting from infective and nutritional corneal opacities and congenital anomalies to more resemble the patterns seen in higher income settings. Improvements in maternal and neonatal health and investment in and maintenance of national ophthalmic care infrastructure are the key to reducing the burden of avoidable blindness. New therapeutic targets are emerging for childhood visual disorders, although the safety and efficacy of novel therapies for diseases such as ROP or retinal dystrophies are not yet clear. Population-based epidemiological research, particularly on cerebral visual impairment and optic nerve hypoplasia, is needed in order to improve understanding of risk factors and to inform and support the development of novel therapies for disorders currently considered ‘untreatable’.

In clinical practice Cerebral Visual Impairment (CVI) is typically diagnosed by observation of abnormal visually guided behaviors which indicate higher visual function deficits (HVFDs) suggesting abnormal brain development or brain damage in a child with a suitable clinical history. HVFDs can occur even in the presence of good visual acuity and may remain undiagnosed because the good visual acuity does not prompt further investigation. This leads to a lack of understanding of the child’s visual perceptual difficulties. In a prospective study, we determined the spectrum of HVFDs in a group of children with history suggestive of brain damage or disruption of brain development and an independent diagnosis of CVI in comparison with typically developing children with a structured 51 question inventory, the Higher Visual Function Question Inventory (HVFQI-51) adapted from the Cerebral Vision Impairment Inventory, CVI-I. Here, we show that the HVFQI-51 can detect a range of HVFDs in children with CVI with good visual acuity and clearly distinguishes these children from typically developing children. HVFDs in our study group could mostly be attributed to dorsal stream visual processing dysfunction though the spectrum varied between children. We report on the inclusion of the “not applicable” response option in analysis providing a picture of HVFDs more in tune with the overall disability of each child. We also propose a subset of 11 questions (Top-11) which discriminate between children with CVI vs. behaviors seen in typical children: this provides both a potential screening tool for initial assessment of HVFDs and a measure of CVI-related impairment, and needs further validation in a secondary independent sample.

Children with brain-based visual impairments (some of whom have a diagnosis of Cerebral Visual Impairment, or 'CVI') represent a growing and underserved population within vision services. These children often have more complex needs than those with ocular visual impairments and benefit from specialist support from multiple disciplines. This study aimed to understand the perspectives of these specialists in terms of their goals, views on collaboration, and understanding of the term 'CVI'. We invited a range of specialists who work with children with brain-based visual impairments, including educators, rehabilitation staff, clinicians, and family members, to complete an online survey between April 2023 and April 2024. The analysis included 94 respondents: 51 educators, 30 rehabilitation staff, 7 clinicians, and 6 family members. Respondents shared common goals of connecting with the child (87/94, 93%) and fostering their learning and development (82/94, 93%). However, respondents also noted some specific and divergent goals, which can be at odds with each other. Professional staff frequently identified family members as the most valuable source of information about their child's vision (36/88, 41%), though family members expressed feeling under-valued. Transdisciplinary clinics were highlighted as a helpful model to provide quality child-centered care. Of the 73 professional staff who reported being familiar with the term 'CVI' (73/88, 83%), most (61/73, 84%) thought it was underdiagnosed, but respondents had different perspectives on what a diagnosis meant. Only 73% of professionals familiar with CVI reported receiving formal training about it. The varied goals and different perspectives on CVI create challenges to providing cohesive support for children with brain-based visual impairments. Increasing the availability of complementary formal training across disciplines and adopting transdisciplinary models of care are promising approaches to improve the quality of services.

The association between the dietary inflammatory index (DII) and visual impairment remains unclear. This study aimed to investigate the relationship between the DII and non-refractive visual impairment among US populations. A cross-sectional analysis was conducted using data from the National Health and Nutrition Examination Survey (NHANES) 2005–2008, including dietary information and visual impairment assessment. Participants with presenting visual impairment, defined as presenting visual acuity in the better-seeing eye worse than 20/40, were included. Participants whose visual acuity in the better-seeing eye could be corrected to 20/40 or better through automated refraction, were classified as having uncorrected refractive error, while others were considered to have non-refractive visual impairment. Logistic regression models, restricted cubic spline (RCS) analysis, subgroup analyses, and propensity score matching (PSM) were performed to assess the association between DII and the prevalence of non-refractive visual impairment. After adjusting for potential confounding factors, a positive association was observed between DII scores and the prevalence of non-refractive visual impairment (odds ratio [OR] = 1.277, 95% confidence interval [CI] = 1.017–1.603, P  < 0.05). RCS analysis demonstrated that there was no nonlinear relationship between them ( P for nonlinear > 0.05). Furthermore, sensitivity analysis by PSM indicated the robustness of this positive association. This study revealed a positive correlation between the DII and the prevalence of non-refractive visual impairment among those with presenting visual impairment in the United States. Further prospective studies are warranted to confirm a causal relationship and elucidate the underlying mechanisms involved.

The inconsistency in terminology for Cortical Visual Impairment or Cerebral Visual Impairment presents challenges: (1) different levels of changes in visual pathway and other cerebral areas do not allow discrimination; (2) different visual and oculomotor aspects are not adequately considered. We open a debate to consider a more appropriate diagnosis.

Introduction Cerebral Visual Impairment (CVI) is an important cause of visual impairment in western countries. Perinatal hypoxic-ischemic damage is the most frequent cause of cerebral visual impairment but CVI can also be the result of a genetic disorder. The majority of children with CVI have cerebral palsy and/ or developmental delay. Early diagnosis is crucial; however, there is a need for consensus on evidence based diagnostic tools and referral criteria. The aim of this study is to develop guidelines for diagnosis and referral in CVI according to the grade method. Patients and methods We developed the guidelines according to the GRADE method 5 searches on CVI (children, developmental age ≤18 years) were performed in the databases Medline, Embase and Psychinfo, each with a distinct topic. Results Based on evidence articles were selected on five topics: 1 Medical history and CVI-questionnaires 23 (out of 1007) 2 Ophthalmological and orthoptic assessment 37 (out of 816) 3 Neuropsychological assessment 5 (out of 716) 4 Neuroradiological evaluation and MRI 9 (out of 723) 5 Genetic assessment 5 (out of 458) Conclusions In medical history taking, prematurity low birth weight and APGAR (Appearance, Pulse, Grimace, Activity, Respiration) Scores (<5) are important. Different questionnaires are advised for children under the age of 3 years, older children and for specific risk groups (extremely preterm). In ophthalmological examination, eye movements, specially saccades, accommodation, crowding, contrast sensitivity and visual fields should be evaluated. OCT can show objective signs of transsynaptic degeneration and abnormalities in fixation and saccades can be measured with eye tracking. Screening of visual perceptive functioning is recommended and can be directive for further assessment. MRI findings in CVI in Cerebral Palsy can be structured in five groups (brain maldevelopment, white and grey matter lesions, postnatal lesions and a normal MRI). In children with CVI and PVL (periventricular leukomalacia), brain lesion severity correlates with visual function impairment. A differentiation can be made between cortical and subcortical damage and related visual function impairment. Additional assessments (neurological or genetic) can be necessary to complete the diagnosis of CVI and/or to reveal the etiology.

Introduction: Cerebral visual impairment (CVI) results from damage to cerebral visual processing structures. There is currently limited understanding on how neurologic, developmental, and ophthalmic factors predict outcome for pediatric CVI. Method: A retrospective manual chart review of pediatric CVI patients seen at a tertiary pediatric hospital between 2010-2019 was conducted. Patients were stratified into severity groups (based on a custom CVI grading score). Collected baseline characteristics included perinatal, genetic, developmental and neurologic history, neuroimaging and fundoscopic findings. Longitudinal data were collected and a linear mixed-effect models were used for CVI outcome analysis. Results: 249 individuals spanning 779 patient visits were identified. Mean age at diagnosis was 18.8 ± 16.8 months (2-108 months). 64.3% were born at term age. Perinatal history revealed hypoxic ischemic encephalopathy (HIE) in 16.5%, intraventricular hemorrhage (IVH) in 11.6%, and seizures in 21.7%. At presentation, 60.3% had a diagnosis of cerebral palsy and 84.7% had developmental delay. Among all subjects, 78.6% had epilepsy; 33.8% had an epileptic encephalopathy. Abnormal neuroimaging was present in 93.8%. Genetic anomalies were present in 26.9%. Baseline visual examination revealed no blink-to-light (BTL) in 24.5%; only BTL in 34.5%, fixation/tracking in 26.5%, and optokinetic drum follow in 14.4%. Longitudinal data analysis showed that perinatal history of HIE, a positive epilepsy history, multiple (≥3) epilepsy medications, cerebral palsy, and abnormal fundoscopic findings were all negatively associated with CVI grade change over time. After controlling for significant confounders, receiving any type of therapy (early childhood intervention [ECI], physical and occupational therapy [PT/OT], refractive error correction or glasses) was significantly associated with longitudinal improvement in CVI grade compared to patients who did not receive any therapy. Conclusions: This study offers extensive insights into neurologic, developmental and ophthalmologic features in patients with moderate to severe CVI. Aspects of perinatal history and epilepsy/seizure control may help inform severity/prognosis in the general neurology or ophthalmology clinic. Moreover, genetic and specific epilepsy traits may alert vision health care providers in the clinic to pursue visual evaluation in at-risk individuals. Longitudinal follow-up of CVI patients showed that interventional therapies demonstrated vision function improvement greater than no therapy and maturational development.

The symptoms that characterize children with cerebral visual impairments (CVI) are diverse, ranging from extensive behavioral or physical disabilities to subtle changes that can easily be missed. A correct diagnosis of CVI is therefore difficult to make, but having a wide variety of tests available can be helpful. This study aims to determine if the developmental eye movement test (DEM) can be one of those tests. In this test, a fixed set of numbers has to be read aloud, first in vertical columns and then in horizontal lines. In order to measure differences between children with CVI compared to normally sighted age-matched controls and children with a visual impairment (VI), we determined DEM times, crowding intensities and the reaction time to a large visual stimulus for all three groups. We found that children with CVI or VI need significantly more time to read the DEM numbers than age-matched controls. Additionally, children with CVI need more time than children with VI to read the horizontal DEM, but not the vertical DEM. We also found a significant difference between the children with CVI and the other two groups in the relationship between horizontal DEM performance and crowding intensity. However, for the relationship between DEM performance and visual detection time, no group-differences were found. We conclude that the DEM can be a useful addition in the diagnosis of CVI, especially in combination with information about crowding.