Prevalence of cerebral visual impairment in developmental and Epileptic Encephalopathies: a systematic review protocol.

BACKGROUND: Developmental and Epileptic Encephalopathies (DEEs) are defined by drug-resistant seizures and neurodevelopmental disorders. Over 50% of patients have a genetic cause. Studies have shown that patients with DEEs, regardless of genetic diagnosis, experience a central visual function disorder known as Cerebral (cortical) Visual Impairment (CVI). The prevalence of CVI in DEE patients is currently unknown. A quantitative synthesis of existing data on the prevalence rates of this condition would aid in understanding the magnitude of the problem, outlining future research, and suggesting the need for therapeutic strategies for early identification and prevention of the disorder. METHODS: The protocol followed the PRISMA-P statement for systematic review and meta-analysis protocols. The review will adhere to the JBI Manual for Evidence Synthesis (Systematic Reviews of Prevalence and Incidence) and use the CoCoPop framework to establish eligibility criteria. We will conduct a comprehensive search of several databases, including MEDLINE, EMBASE, Science Direct, Scopus, PsychINFO, Wiley, Highwire Press, and Cochrane Library of Systematic Reviews. Our primary focus will be determining the prevalence of cerebral visual impairments (Condition) in patients with developmental and epileptic encephalopathy (Population). To ensure clarity, we will provide a narrative summary of the risk of bias in the studies we include. The Cochrane Q statistic will be used to assess heterogeneity between studies. If the quantitative synthesis includes more than 10 studies, potential sources of heterogeneity will be investigated through subgroup and meta-regression analyses. Meta(bias)es analysis will also be performed. The quality of evidence for all outcomes will be evaluated using the Grading of Recommendations Assessment Development and Evaluation (GRADE) working group methodology. DISCUSSION: This protocol outlines a systematic review and meta-analysis to identify, collect, evaluate, and integrate epidemiological knowledge related to the prevalence of CVI in patients with DEEs. To the best of our knowledge, no other systematic review and meta-analysis has addressed this specific issue. The results will provide useful information for understanding the extent of the problem, outlining future research, and suggesting the need for early identification strategies. SYSTEMATIC REVIEW REGISTRATIONS: This Systematic Review Protocol was registered in PROSPERO (CRD42023448910).

Motor-Based Application Process for Cerebral Visual Impairment-Related Questionnaires for School-Age Children with Cerebral Palsy.

This study aims to examine the psychometric properties of questionnaires related to cerebral visual impairment (CVI) in school-age children with cerebral palsy (CP). Additionally, it proposes an application process based on motor functionality for occupational therapists. A total of 288 children with CP were recruited for the study and administered the CVI Motor Questionnaire (CVI-MQ). The children's daily visual performance was assessed using the CVI Inventory and the Functional Vision Questionnaire (FVQ) considering the children's ambulatory status. The FVQ and the two factors extracted from the CVI Inventory significantly predicted visual functioning. Specific questionnaires related to CVI, applied with gross motor function in mind, can be valuable tools for occupational therapists to assess daily visual performance.

Eyes on CVI: Eye movements unveil distinct visual search patterns in Cerebral Visual Impairment compared to ADHD, dyslexia, and neurotypical children.

Visual search problems are often reported in children with Cerebral Visual Impairment (CVI). To tackle the clinical challenge of objectively differentiating CVI from other neurodevelopmental disorders, we developed a novel test battery. Visual search tasks were coupled with verbal and gaze-based measurements. Two search tasks were performed by children with CVI (n: 22; mean age (SD): 9.63 (.46) years) ADHD (n: 32; mean age (SD): 10.51 (.25) years), dyslexia (n: 28; mean age (SD): 10.29 (.20) years) and neurotypical development (n: 44; mean age (SD): 9.30 (.30) years). Children with CVI had more impaired search performance compared to all other groups, especially in crowded and unstructured displays and even when they had normal visual acuity. In-depth gaze-based analyses revealed that this group searched in overall larger areas and needed more time to recognize a target, particularly after their initial fixation on the target. Our gaze-based approach to visual search offers new insights into the distinct search patterns and behaviours of children with CVI. Their tendency to overlook targets whilst fixating on it, point towards higher-order visual function (HOVF) deficits. The novel method is feasible, valid, and promising for clinical differential-diagnostic evaluation between CVI, ADHD and dyslexia, and for informing individualized training.

Long-term visual and neurodevelopmental outcomes in children with Congenital Zika Syndrome after undergoing strabismus surgery.

Purpose: To assess long-term visual and neurodevelopmental outcomes in children with congenital Zika syndrome (CZS) after strabismus surgery. Methods: A consecutive sample of five children with CZS who underwent strabismus surgery was enrolled. All children underwent a standardized pre- and postoperative protocol including binocular best-corrected visual acuity (BCVA) using the Teller Acuity Cards II (TAC II), ocular alignment, functional vision using the functional vision developmental milestones test (FVDMT), and neurodevelopmental milestone evaluation using the Bayley Scales of Infant Development-Third Edition (BSID-III). Scores of the FVDMT outcomes considering the child's developmental age based on the BSID-III score were compared with scores from postoperative assessment. Results: Five children with CZS (3 girls, 2 boys) were enrolled with a mean age at baseline (preoperative) of 35.0 ± 0.7 months (range, 34-36 months) and at final assessment of 64.4 ± 0.5 months (range, 64-65 months). Preoperative BCVA was 1.2 ± 0.5 logMAR and at final assessment 0.7 ± 0.1 logMAR. Successful strabismus surgery outcome was maintained in 4/5 (80.0%) of children at final assessment. The children's BSID-III scores showed significant neurodevelopment delay at the initial assessment (corresponding developmental mean age was 4.7 months) and at their final assessment (corresponding developmental mean age was 5.1 months). There was improvement or stability in 34/46 items evaluated in the FVDMT (73.9%) when comparing baseline with 2-year follow-up. Conclusions: Strabismus surgery resulted in long-term ocular alignment in the majority of children with CZS. All the children showed improvement or stability in more than 70.0% of the functional vision items assessed. Visual and neurodevelopmental dysfunction may be related to complex condition and associated disorders seen in CZS including ocular, neurological, and skeletal abnormalities.

Cerebral versus cortical visual impairment: eliminating the conflict and renewing the terminology.

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.

Visuospatial processing in early brain-based visual impairment is associated with differential recruitment of dorsal and ventral visual streams.

Visuospatial processing impairments are prevalent in individuals with cerebral visual impairment (CVI) and are typically ascribed to "dorsal stream dysfunction" (DSD). However, the contribution of other cortical regions, including early visual cortex (EVC), frontal cortex, or the ventral visual stream, to such impairments remains unknown. Thus, here, we examined fMRI activity in these regions, while individuals with CVI (and neurotypicals) performed a visual search task within a dynamic naturalistic scene. First, behavioral performance was measured with eye tracking. Participants were instructed to search and follow a walking human target. CVI participants took significantly longer to find the target, and their eye gaze patterns were less accurate and less precise. Second, we used the same task in the MRI scanner. Along the dorsal stream, activation was reduced in CVI participants, consistent with the proposed DSD in CVI. Intriguingly, however, visual areas along the ventral stream showed the complete opposite pattern, with greater activation in CVI participants. In contrast, we found no differences in either EVC or frontal cortex between groups. These results suggest that the impaired visuospatial processing abilities in CVI are associated with differential recruitment of the dorsal and ventral visual streams, likely resulting from impaired selective attention.

Aberrant White Matter Development in Cerebral Visual Impairment: A Proposed Mechanism for Visual Dysfunction Following Early Brain Injury.

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.

Long-Term Visual Outcomes in Prematurely Born Children.

PURPOSE: Prematurely born children are at risk of numerous complications that affect the visual system. Retinopathy of prematurity (ROP) and cerebral visual impairment (CVI) are among two major causes of childhood blindness and visual impairment in industrialized nations, and large countries with emerging economies are seeing increasing childhood blindness from ROP alone, adding to the burden of disease worldwide. The purpose of this paper is to review the long-term impacts of prematurity, ROP and CVI on vision in children who were born preterm. METHOD: The topics in this review of the literature include the burden of vision loss in prematurely born children world-wide, a description of ROP and CVI, effects on visual acuity, refractive errors, strabismus and binocularity, visual fields and contrast sensitivity, and risk factors for visual complications. RESULTS: Children who are most at risk of visual complications are those with the smallest gestational age at birth and birth weight in general. Although ROP severity and the presence of neurological impairments including CVI play a large role in the development of poor visual outcomes, premature birth alone without CVI or severe ROP increases the risk of future visual complications. Awareness of signs and symptoms of CVI are important in the management of affected children. CONCLUSION: Children born preterm are at increased risk of reduced visual acuity, refractive errors, strabismus and amblyopia, complications of ROP, CVI, visual field abnormalities and reduced contrast sensitivity. Awareness of risk factors warranting close monitoring and signs and symptoms of CVI are critical to optimize the visual outcomes and overall development.

Neurological and Visual Outcomes in Infants and Toddlers Following Therapeutic Hypothermia for Neonatal Hypoxic-Ischemic Encephalopathy.

BACKGROUND: The majority of studies have investigated neurodevelopmental outcomes, whereas visual impairment is less explored in children with a history of neonatal (hypoxic-ischemic) encephalopathy. Our aim was to perform a detailed neurological and visual assessment and also to investigate the presence of cerebral visual impairment in infants and toddlers with neonatal encephalopathy. METHODS: Thirty participants with a history of neonatal encephalopathy, who had been hospitalized for therapeutic hypothermia, underwent a detailed neurological examination at age five to 36 months. Age-matched, 30 healthy children were also enrolled as a control group. All children in the study and control groups received neurological and a comprehensive ophthalmologic examination, including visual field and visual acuity. Presence of cerebral visual impairment was also evaluated clinically. RESULTS: Rates of cerebral palsy, severe motor impairment, cognitive impairment, epilepsy, and cerebral visual impairment were found to be 20%, 10%, 15.3%, 10%, and 20%, respectively. When compared with healthy controls, oculomotor functions, pupillary light response, refractive parameters, anterior/posterior segment examinations, ocular visual impairment rates, and last, visual acuities were found similar. However, we found a statistically significant increase in visual field defects in our study group. CONCLUSIONS: It could be better to perform a comprehensive ophthalmologic examination including visual field, visual acuity, and oculomotor functions by a pediatric ophthalmologist to accurately diagnose neurovisual deficits in infants following therapeutic hypothermia. Early identification and rehabilitation of the visual deficits might improve the neurodevelopment in these children.

Childhood cerebral visual impairment subtype classification based on an extensive versus a limited test battery.

Purpose: To classify CVI subtypes and compare the added value of an extensive test battery over a limited test battery in subtype classification of cerebral visual impairment (CVI) in children. Methods: Seventy-five children with a clinical diagnosis of CVI (median [IQR] age: 9 [7-12] years) were identified from the medical records. The extensive test battery included visual acuity, contrast sensitivity, ocular alignment, eye movement analysis, visual field analysis, optic nerve head evaluation, and evaluation of visual perception. The limited test battery included visual acuity, contrast sensitivity, ocular alignment, and evaluation of visual perception. Principal component analysis (PCA) followed by cluster analysis was done, for both test batteries separately, to determine the optimum subtype classification for CVI. Results: Fifty-one participants with an extensive test battery with mild to moderate visual impairment were included in the main analysis. This resulted in four CVI subtypes for the extensive test battery (subtle characteristics, higher-level visual function deficits, lower-level visual function deficits, and higher- and lower- level visual function deficits) and three CVI subtypes for the limited test battery (subtle characteristics, higher-level visual function deficits, and higher- and lower- level visual function deficits). There were significant differences between the subtypes for 9 out of 10 measures of the extensive and all 4 measures of the limited test battery (p < 0.05). The subtle characteristics subtype (extensive n = 19, limited n = 15) showed near normal lower and higher-level visual functions in both test batteries. The higher-level visual function deficits subtype (extensive n = 18, limited n = 24) showed near normal visual acuity combined with significant visual perceptual deficits in both test batteries; accompanied by visual pathways defects and abnormal eye movement behavior in the extensive test battery. The higher- and lower- level visual function deficits subtype (extensive n = 4, limited n = 12) showed both higher and lower-level visual function deficits in both test batteries, but application of the extensive test battery revealed additional visual pathways defects and abnormal eye movement behavior. The lower-level visual function deficits CVI subtype (extensive n = 10) was a new subtype identified by the extensive test battery. This subtype showed lower-level visual function deficits together with abnormal eye movement measures. Conclusion: This data-driven study has provided meaningful CVI subtype classifications based on the outcomes of various key functional and structural measures in CVI diagnosis. Comparison of the extensive test battery to the limited test battery revealed the added value of an extensive test battery in classifying CVI. The outcomes of this study, therefore, have provided a new direction in the area of CVI classification.

Australian children with cerebral visual impairment: using what we know now to improve future approaches.

PURPOSE: Little has been reported on Australian children with Cerebral Vision Impairment (CVI). This paper aims to present the outcome of an audit focussed on children with the primary diagnosis of CVI, using findings from the Australian Childhood Vision Impairment Register (ACVIR). METHODS: Records on 132 children (49% girls, 51% boys) from ACVIR data gathered from both the child's parent/guardian and their eye health professional were reviewed. The child's demographics, level of vision impairment, birth history, diagnostic journey, secondary ocular diagnoses, comorbidities and low vision support were analyzed. Several correlations were investigated using a Kendall's tau-b analysis including the relationship between vision and age of diagnosis; level of vision and developmental delay; and age of suspicion of visual impairment and age of diagnosis. RESULTS: The most common level of visual impairment was blindness (39%), and most children were suspected and diagnosed of visual impairment in the first 6 months of life. The majority of children were born full term (72%), weighing >2000gms (84%). Nearly half of the cohort of children (48%) had a secondary ocular diagnosis with 44% having nystagmus. The majority of children (80%) had additional health problems, and 85% of children had additional disabilities, with 79% having developmental delay. CONCLUSION: While the findings of this audit cannot be generalized to a wider population of Australian children with CVI, the outcome encourages continued discussion on CVI, to explore comprehensive assessment approaches which facilitate timely and accurate diagnosis.

Ophthalmic manifestations in children with tuberous sclerosis complex.

PURPOSE: To report on the ophthalmic findings in children with tuberous sclerosis complex (TSC) in southern Sweden, and to investigate the frequency of refractive errors, strabismus and cerebral visual impairment associated with this condition. METHODS: This was a retrospective cohort study including all paediatric patients with TSC in southern Sweden born between 1983 and 2020. Medical records were reviewed regarding retinal findings, visual acuity, refractive error, strabismus, full-field electroretinography results and cerebral visual impairment. RESULTS: Ophthalmological records were available for 50 of the 52 children in the region diagnosed with TSC. The mean age at the last visit was 12.4 (SD 7.2) years. Monocular visual acuity had been measured in 38 patients, and the median value did not deviate from that expected for their age in the better eye, but by -0.2 Snellen decimal acuity in the worse eye. Refractive errors were found in 62% of the patients, and strabismus in 16%. Retinal astrocytic hamartomas were found in 34% and achromatic patches in 34%. Ten of the patients on medication with vigabatrin were examined with full-field electroretinography and treatment had to be stopped or lowered in three (30%), due to a reduced response. Investigation of cerebral visual impairment had not been conducted in any of the children. CONCLUSION: Refractive errors and strabismus were common among children with TSC. None of the patients in this cohort had undergone investigation for cerebral visual impairment. The general awareness of cerebral visual impairment among ophthalmologists is poor and constitutes an important area for improvement.

Current perspective: Cerebral visual impairment-The impending doom.

Cerebral visual impairment (CVI) has emerged as an important cause of morbidity in young children. CVI children often have a large number of visual symptoms along with motor abnormalities. It is the need of the hour to build an integrated approach towards their management. This article aimed to provide a comprehensive view of the manifestations, evaluation, and management of children with CVI.

Altered neural oscillations underlying visuospatial processing in cerebral visual impairment.

Visuospatial processing deficits are commonly observed in individuals with cerebral visual impairment, even in cases where visual acuity and visual field functions are intact. Cerebral visual impairment is a brain-based visual disorder associated with the maldevelopment of central visual pathways and structures. However, the neurophysiological basis underlying higher-order perceptual impairments in this condition has not been clearly identified, which in turn poses limits on developing rehabilitative interventions. Using combined eye tracking and EEG recordings, we assessed the profile and performance of visual search on a naturalistic virtual reality-based task. Participants with cerebral visual impairment and controls with neurotypical development were instructed to search, locate and fixate on a specific target placed among surrounding distractors at two levels of task difficulty. We analysed evoked (phase-locked) and induced (non-phase-locked) components of broadband (4-55 Hz) neural oscillations to uncover the neurophysiological basis of visuospatial processing. We found that visual search performance in cerebral visual impairment was impaired compared to controls (as indexed by outcomes of success rate, reaction time and gaze error). Analysis of neural oscillations revealed markedly reduced early-onset evoked theta [4-6 Hz] activity (within 0.5 s) regardless of task difficulty. Moreover, while induced alpha activity increased with task difficulty in controls, this modulation was absent in the cerebral visual impairment group identifying a potential neural correlate related to deficits with visual search and distractor suppression. Finally, cerebral visual impairment participants also showed a sustained induced gamma response [30-45 Hz]. We conclude that impaired visual search performance in cerebral visual impairment is associated with substantial alterations across a wide range of neural oscillation frequencies. This includes both evoked and induced components suggesting the involvement of feedforward and feedback processing as well as local and distributed levels of neural processing.

Relationship between Sensory Processing Skills and Feeding Behaviors in Children Aged 3-6 Years with Cerebral Palsy with Cerebral Visual Impairment.

The current study aimed to examine the relationship between sensory processing skills and feeding behavior in cerebral palsy (CP) children aged 3-6 years with cerebral visual impairment (CVI). A total of ninety mothers participated in the study in three groups: thirty mothers of children with CP with CVI, thirty mothers of children with CP without CVI, and thirty mothers of children with typical development (TD). The sensory processing skill of the children was evaluated with the Sensory Profile (SP), and feeding behavior was evaluated with the the Behavioral Pediatric Feeding Assessment Scale (BPFAS). In the triple comparison, a significant difference was found between the groups in all SP parameter and BPFAS scores (p < 0.001). Post hoc analysis revealed statistically significant differences between the groups in all parameters (p < 0.001). Feeding problems were detected in 65% of all groups. In the correlation analysis, a significant relationship was found between all parameters of the SP and the BPFAS (p < 0.05). In terms of sensory processing skills and feeding status, it was determined that children with CVI with CP had more problems than children with CP without CVI, and children with CP without CVI had more problems than children with TD. With these results, it was concluded that sensory processing problems affect feeding status, and visual impairment causes both sensory problems and feeding problems.

Visual Behaviours (ViBes) in Cerebral Visual Impairment: Validating a Descriptive Tool to Support Diagnosis and Monitoring.

Introduction: Cerebral visual impairment (CVI) is the most common cause of visual impairment in children in the UK. Diagnosis is based on identification of visual behaviours (ViBes) relating to visual dysfunction. Examination techniques and inventories have been developed to elicit these in children with a developmental age of two years or more. The absence of a structured approach to recording visual behaviours in children with complex needs is a barrier to diagnosis. The aim of the study was to develop a matrix of visual behaviours seen in pre-verbal and pre-motor children with visual impairment and establish its content validity and inter-rater reliability. Methods: ViBe content validation:: Visual behaviour descriptors relating to visual function were collated and categorised by expert consensus of vision professionals into a matrix composed of three functions (attention, field/fixation, motor response) and five levels (0 = no awareness; 1 = visual awareness; 2 = visual attention; 3 = visual detection; 4 = visual understanding).ViBe inter-rater reliability:: The participants (two orthoptists, an optometrist, an ophthalmologist and two qualified teachers of the visually impaired) used the ViBe matrix to independently score each of 17 short video clips of children demonstrating visual behaviours seen in CVI. Results: The ViBe matrix will be presented. Cohen's kappa for the matrix was 0.67, demonstrating moderate-to-strong inter-rater reliability. Conclusion: The development of standardised descriptors can support clinicians and teachers in identifying areas of concern for children with complex needs. In addition, the ViBe matrix could be utilised in research, clinical and diagnostic reports to clearly communicate the areas of visual dysfunction and track progress resulting from interventions. Key Points: The absence of a structured approach to recording visual behaviours in children with complex needs is a barrier to diagnosis.The ViBe matrix offers descriptors relating to visual behaviours and has demonstrated acceptable inter-rater reliability.The tool may support the identification and diagnosis of cerebral visual impairment in a population of children who cannot access standard testing.

A Scoping Review of Intervention for Pediatric Cerebral Visual Impairment: Calling All Pediatric Occupational Therapists.

Cerebral visual impairment is the leading cause of low vision in children, and functional gains can be achieved with intervention. To date there exists no evidence-based intervention protocol to guide rehabilitation therapists. This scoping review was conducted to synthesize the evidence currently available and explore current interventions in order to guide future research. This review identified five types of interventions for cerebral visual impairment; habilitation, visual stimulation, video game, color tent, and medical and also evidenced the need for standardized, objective measures of function for this population.

Object identification in cerebral visual impairment characterized by gaze behavior and image saliency analysis.

Individuals with cerebral visual impairment (CVI) have difficulties identifying common objects, especially when presented as cartoons or abstract images. In this study, participants were shown a series of images of ten common objects, each from five possible categories ranging from abstract black & white line drawings to color photographs. Fifty individuals with CVI and 50 neurotypical controls verbally identified each object and success rates and reaction times were collected. Visual gaze behavior was recorded using an eye tracker to quantify the extent of visual search area explored and number of fixations. A receiver operating characteristic (ROC) analysis was also carried out to compare the degree of alignment between the distribution of individual eye gaze patterns and image saliency features computed by the graph-based visual saliency (GBVS) model. Compared to controls, CVI participants showed significantly lower success rates and longer reaction times when identifying objects. In the CVI group, success rate improved moving from abstract black & white images to color photographs, suggesting that object form (as defined by outlines and contours) and color are important cues for correct identification. Eye tracking data revealed that the CVI group showed significantly greater visual search areas and number of fixations per image, and the distribution of eye gaze patterns in the CVI group was less aligned with the high saliency features of the image compared to controls. These results have important implications in helping to understand the complex profile of visual perceptual difficulties associated with CVI.

Development of an integrated telerehabilitation model in children with cerebral visual impairment in South India during the COVID-19 pandemic - A pilot study.

Purpose: Coronavirus disease 2019 (COVID-19) pandemic affected the in-person rehabilitation/habilitation services in families with children with cerebral visual impairment (CVI) in India. This study aimed to develop a structured and family-centered telerehabilitation model alongside conventional in-person intervention in children with CVI to observe its feasibility in the Indian population. Methods: This pilot study included 22 participants with a median age of 2.5 years (range: 1-6) who underwent a detailed comprehensive eye examination followed by functional vision assessment. The visual function classification system (VFCS) was administered to the children and the structured clinical question inventory (SCQI) to the parents. Every participant underwent 3 months of telerehabilitation including planning, training, and monitoring by experts. At 1 month, the parental care and ability (PCA) rubric was administered to the parents. After 3 months, in an in-person follow-up, all the measures were reassessed for 15 children. Results: After 3 months of Tele-rehabilitation there were significant improvements noted in PCA rubric scores (P<0.05). Also, statistically significant improvements were noted in functional vision measured using SCQI and VFCS scores (P<0.05) compared to baseline. Conclusion: The outcomes of the study provide the first steps towards understanding the use of a novel tele-rehabilitation model in childhood CVI along-side conventional face-to-face intervention. The added role of parental involvement in such a model is highly essential.

Case report: Bilateral damage to the immature optic radiation and secondary massive loss of retinal ganglion cells causing tunnel vision.

We describe the case of a 30-year-old woman, who needed a formal report on her visual impairment to seek support from society. She was born preterm, and during her neonatal period, she suffered from bilateral intraventricular hemorrhage (IVH) grade 3, a condition that can cause cerebral visual impairment (CVI) due to damage to the retro-geniculate visual pathways. Individuals with such brain damage of this severity are often restricted by cerebral palsy (CP) and intellectual disability, and thus have a limited ability to cooperate in the assessment of visual function. However, our patient was capable of providing reliable test results, and she manifested only a small island of central vision in each eye, with additional reduced visual acuities. She cooperated well in examinations involving MRI of the brain, optical coherence tomography (OCT) of retinal ganglion cells, and multi-focal visual evoked potentials, with each test providing information about potential limitations in the structural prerequisites for visual function. What distinguishes our case is the severity of the damage to the optic radiations and the massive secondary loss of most of her retinal ganglion cells (GCs). However, there is some measurable visual function, which may be due to developmental neuroplasticity during early development, when surviving GCs prioritize the central visual field. Despite her visual difficulties, she is a keen portrait painter. Our patient may be representative of, and a spokesperson for, other individuals with extensive brain damage of the same etiology, who are unable to perform perimetric tests and therefore run the risk of not being recognized as severely visually impaired, and consequently, not being given the best conditions for habilitation. OCT may serve as a helpful diagnostic tool. Aim: This study aims to describe visual behavior and practical applications of visual function in relation to structural prerequisites for visual function.