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Cortical visual impairment

Cortical visual impairment (CVI), also referred to as cerebral visual impairment, is a brain-based disorder that affects the processing and interpretation of visual information despite structurally normal eyes and visual pathways up to the optic nerves.^[1] It manifests as a spectrum of visual deficits, ranging from profound impairment to near-normal functional vision, and is the leading cause of childhood visual impairment in developed countries. In the United States, it is estimated to affect more than 180,000 children.^[2] A study in South India found it accounting for 44% of profoundly visually impaired children under age 3.^[3] The condition arises from damage to the retrogeniculate visual pathways or cortical processing areas in the brain, often resulting from perinatal hypoxic-ischemic encephalopathy, prematurity-related complications such as intraventricular hemorrhage, central nervous system malformations, infections, head trauma, hydrocephalus, metabolic or genetic disorders, seizures, or exposure to toxins.^[3] Premature infants and those with developmental disabilities like cerebral palsy or epilepsy are at higher risk, with the impairment potentially occurring before, during, or after birth.^[1] Unlike ocular conditions, CVI involves inconsistent visual behaviors, such as delayed responses to stimuli, difficulty recognizing faces or objects in cluttered environments, light gazing or aversion, reliance on movement to detect items, and challenges with complex visual scenes.^[3] Diagnosis requires a multidisciplinary approach, including detailed medical history, parent interviews on functional vision, comprehensive eye examinations to rule out ocular issues, brain imaging like MRI, and behavioral assessments by specialists such as pediatric ophthalmologists or neurologists, as no single test confirms CVI.^[1] Management focuses on early intervention through vision rehabilitation, educational accommodations, and support from teachers of the visually impaired, aiming to maximize functional vision since no curative treatment exists, though some children show improvement over time with targeted therapies.^[3]

Introduction and Background

Definition

Cortical visual impairment (CVI), also referred to as cerebral visual impairment, encompasses a spectrum of visual impairments caused by an underlying brain abnormality that affects the development of visual processing pathways posterior to the lateral geniculate nucleus, resulting in deficits of visual function and functional vision that exceed any co-occurring ocular conditions.^[4]^[5] This neurodevelopmental disorder is characterized by neurologic damage to visual pathways, including the optic radiations, occipital cortex, and visual association areas, leading to lower-order or higher-order afferent visual deficits, or both.^[4] The terminology debate between "cortical" and "cerebral" reflects the involvement beyond just the cortex to broader cerebral structures, with "CVI" preferred to unify the concepts and emphasize brain-based processing deficits over ocular structural issues.^[4]^[1] A hallmark of CVI is that the eyes are typically structurally normal, transmitting visual signals intact to the brain, but the brain struggles to interpret and integrate this information, often resulting in challenges with functional vision despite preserved basic acuity in some cases.^[1]^[6] This distinguishes CVI from primary ocular disorders, as the visual dysfunction cannot be fully explained by eye pathology alone, such as amblyopia or retinopathy.^[4] CVI is the leading cause of visual impairment among children in developed economies, driven by improved survival rates for infants with neurologic conditions like hypoxic-ischemic encephalopathy.^[3] The historical context of CVI traces back to the early 19th century, when scientific acceptance emerged that vision is processed in the brain rather than solely by the eyes, laying groundwork for understanding central visual deficits.^[7] However, modern recognition of CVI as a distinct spectrum—particularly in pediatric populations—surged in the 1990s, coinciding with neuroimaging advances that revealed damage to dorsal and ventral visual streams beyond primary cortical lesions.^[8] This shift broadened diagnostic criteria from earlier focuses on profound cortical blindness to include subtler, higher-order impairments.^[9]

Epidemiology

Cortical visual impairment (CVI) is the leading cause of pediatric visual impairment in developed economies, such as the United States, where improved survival rates for children with neurologic deficits have contributed to its prominence.^[3]^[10] Recent estimates indicate that at least 3% of primary school children in industrialized nations exhibit CVI-related visual problems.^[11] In a 2019 study from a tertiary eye care center in South India, CVI accounted for 44% of cases among profoundly visually impaired children under 3 years of age, with 33% attributed to pure CVI and 11% to CVI combined with ocular visual impairment.^[12] A cross-sectional survey in the United Kingdom identified CVI-related vision problems in 31.5% of primary school children (aged 5-11 years) requiring extra educational support.^[13] CVI primarily affects infants and young children, with a notably higher incidence among preterm infants due to complications such as intraventricular hemorrhage. Studies report that 21% to 47% of preterm-born children exhibit CVI characteristics.^[3]^[10] It frequently co-occurs with other neurodevelopmental conditions, including cerebral palsy (in 26% to 83% of CVI cases), epilepsy, autism spectrum disorder, intellectual disability, and developmental delays.^[3] Globally, recognition of CVI is increasing in low-resource settings, where it is emerging as a significant cause of childhood visual impairment, though it remains underestimated among children with special needs. Diagnostic and service disparities persist, particularly among minoritized populations, with higher incidence observed in historically minoritized ethnic groups in the United Kingdom, highlighting equity concerns related to access.^[3]^[10]

Causes and Pathophysiology

Etiology

Cortical visual impairment (CVI) arises from damage to the brain's visual processing areas, with the most common etiology being hypoxic-ischemic encephalopathy (HIE), which involves oxygen deprivation to the brain during the perinatal period.^[3]^[1] HIE accounts for a significant proportion of cases, particularly in term and preterm infants, where it disrupts cerebral blood flow and leads to ischemic injury in visual pathways.^[3] Perinatal causes are predominant, especially in premature infants. Complications of prematurity, such as intraventricular hemorrhage (IVH), occur in approximately 34% of very low birth weight (VLBW) infants weighing less than 1,500 grams and can extend to periventricular leukomalacia, contributing to CVI through bleeding and subsequent white matter damage.^[14]^[3] Prolonged neonatal intensive care unit stays often exacerbate these risks due to associated vulnerabilities like respiratory distress or mechanical ventilation.^[6] Acquired causes typically occur postnatally and include traumatic brain injury from accidents or non-accidental trauma, such as shaken baby syndrome.^[15] Infections like meningitis, meningoencephalitis, or congenital cytomegalovirus can inflame brain tissue and impair visual centers.^[3]^[16] Hydrocephalus, resulting from cerebrospinal fluid buildup, and strokes in older children further contribute by exerting pressure or causing focal ischemia in the occipital regions.^[1] Genetic and congenital etiologies involve central nervous system malformations, such as lissencephaly or schizencephaly, which disrupt cortical development and visual processing from birth.^[6]^[3] Metabolic disorders, including hypoglycemia or leukodystrophies, can lead to neuronal damage affecting vision.^[3] Rare genetic conditions like Joubert syndrome, characterized by midbrain-hindbrain malformations, are associated with CVI through cerebellar and brainstem involvement impacting visual integration.^[17] Key risk factors include low birth weight, particularly in VLBW infants, which heightens susceptibility to perinatal insults and IVH.^[6]^[14] Although less common in pediatric populations, adult-onset CVI can stem from strokes or trauma, but the focus remains on early-life vulnerabilities.^[16] CVI often has a multifactorial nature, with multiple insults—such as combined HIE and prematurity—contributing cumulatively, as outlined in the 2024 American Academy of Pediatrics guidelines.^[3] This interplay underscores the need for comprehensive evaluation of potential overlapping causes in affected children.^[3]

Neurological Mechanisms

Cortical visual impairment (CVI) arises from damage to brain structures posterior to the lateral geniculate nucleus, primarily affecting the occipital lobe, including the primary visual cortex (V1), as well as parietal and temporal lobes involved in higher-order visual processing.^[18] The optic radiations and subcortical structures like the thalamus are also commonly impacted, leading to disruptions in the transmission and integration of visual signals relayed from the intact retinogeniculate pathway.^[6] Unlike ocular visual impairments, which stem from issues in the eyes or anterior visual pathways, CVI features preserved retinal and geniculate function but impaired cortical integration, resulting in visual deficits that exceed any coexisting ocular abnormalities.^[19] Key mechanisms involve selective vulnerabilities in the two major visual processing streams originating from V1. The dorsal stream, projecting to the parietal lobe via pathways like the superior longitudinal fasciculus, handles motion detection, spatial orientation, and visuomotor guidance; its disruption often predominates in CVI, causing difficulties in perceiving movement and navigating space.^[18] In contrast, the ventral stream, extending to the temporal lobe through the inferior longitudinal fasciculus, supports object recognition and form perception; damage here leads to challenges in identifying complex visual stimuli.^[19] White matter injuries, such as periventricular leukomalacia, further contribute by causing delays in neural response latency and reduced signal integrity, impairing the synchronization required for efficient visual processing.^[20] CVI manifests in progressive phases as described in the Roman-Lantzy framework, reflecting evolving neural recovery and adaptation. Phase I involves severe basic visual deficits, with inconsistent attention and reliance on salient cues like light due to profound disruptions in early cortical activation.^[21] Phase II features emerging integration of vision with motor function, as partial reconnection in association areas allows functional use amid ongoing stream imbalances.^[21] Phase III entails refinement of complex interpretation, with improved but residual higher-order processing issues from persistent white matter and stream vulnerabilities.^[21] Neuroimaging provides evidence of these mechanisms, with structural MRI frequently revealing periventricular leukomalacia and optic radiation thinning in affected individuals.^[22] Functional MRI studies demonstrate reduced activation in visual cortical areas during stimuli presentation, correlating with dorsal stream deficits and delayed responses.^[23] Recent research, including 2024 National Eye Institute initiatives, highlights diffusion tensor imaging's role in mapping white matter tract integrity, underscoring impaired connectivity as a core pathophysiological feature.^[3]

Clinical Characteristics

Visual Symptoms

Cortical visual impairment (CVI) is characterized by core visual processing deficits that arise from damage to the brain's visual pathways, rather than the eyes themselves. Individuals with CVI often experience difficulty recognizing faces, places, or objects, even when visual acuity appears intact. They may struggle particularly with static objects or objects presented against cluttered or patterned backgrounds, often relying on movement to aid detection, as these increase the complexity of visual processing. Inconsistent visual attention is common, such as staring at lights or bright areas while ignoring nearby toys or familiar items.^[16]^[1]^[24] Specific deficits further highlight the neurological basis of CVI. Visual latency manifests as delayed responses to stimuli, including slow fixation on objects or delayed reactions to hand gestures and facial expressions. Photophobia or paradoxical light gazing can occur, where individuals are either sensitive to light or unusually attracted to bright sources for prolonged periods. Eccentric or peripheral viewing preferences may lead to reliance on side vision rather than central gaze, and visual fatigue often sets in after brief tasks, making sustained focus challenging.^[16]^[1]^[25] The severity of CVI spans a broad spectrum, from mild forms involving subtle recognition issues to profound cortical blindness, where individuals may exhibit no purposeful visual responses but retain potential for partial recovery over time. In severe cases, vision can improve with neuroplasticity, particularly in children, though persistent challenges may remain.^[16]^[24]^[6] Age-specific manifestations are evident in early development. Infants with CVI typically show poor visual tracking, failing to follow moving objects smoothly from birth or early months. In toddlers, avoidance of complex visuals becomes apparent, such as reluctance to engage with busy scenes or multiple items, as noted in recent clinical reviews.^[26]^[16] A key differentiation from ocular blindness is that in CVI, the eyes and pupils function normally—pupils react appropriately to light—yet the brain cannot effectively process or utilize the visual input, rendering any present vision unusable for recognition or navigation.^[1]^[6]^[27]

Behavioral Manifestations

Children with cortical visual impairment (CVI) frequently demonstrate a preference for tactile or auditory cues over visual input, using touch or sound to interact with objects and their surroundings more reliably than sight alone. Inconsistent responses to familiar items are common, where a child might recognize a toy or caregiver on one occasion but fail to do so in another due to fluctuating visual processing efficiency influenced by factors like fatigue or environmental complexity. Navigation challenges manifest as hesitation or clumsiness in moving through spaces, particularly unfamiliar ones, often resulting in bumping into objects or avoiding certain areas. Social interactions can be affected by reduced eye contact and difficulty interpreting facial expressions, behaviors that are sometimes mistaken for autism spectrum disorder symptoms.^[28]^[3]^[29] Developmental milestones may be delayed, such as postponing crawling or walking because visual disorientation on patterned surfaces creates uncertainty and fear of movement. The intense cognitive effort required for visual processing often leads to rapid fatigue, manifesting as irritability, withdrawal, or meltdowns during prolonged visual tasks. To compensate, children might adopt strategies like head tilting to optimize peripheral vision or relying on auditory predictions to anticipate events.^[28]^[29]^[3] CVI often overlaps with ADHD-like inattention and anxiety, where visual processing demands contribute to distractibility, difficulty sustaining focus, and heightened anxiety from sensory overload in cluttered or busy settings; such co-occurring traits are noted in a significant portion of cases, exacerbating behavioral challenges.^[30]^[29] Key observational signs include fixating on the edges or lights around objects rather than their centers, as well as improved performance and engagement in familiar, simplified environments compared to novel or complex ones. These behaviors can lead to misdiagnosis as cognitive delays or primary behavioral disorders, underscoring the need for specialized visual assessments.^[1]^[28]^[3]

Diagnosis and Assessment

Diagnostic Process

The diagnostic process for cortical visual impairment (CVI) begins with a comprehensive medical history to identify potential risk factors, such as birth complications including preterm delivery or hypoxic-ischemic encephalopathy, and neurological events like pediatric stroke or hydrocephalus.^[3] Caregivers are interviewed to describe the child's visual behaviors, such as delayed responses to visual stimuli or inconsistent visual attention, which may prompt further evaluation.^[31] This step is crucial for distinguishing CVI from other developmental delays and guiding referrals.^[3] A thorough pediatric eye examination follows to rule out ocular causes, confirming normal eye structure, refractive status, and anterior segment health, as CVI requires visual deficits that exceed any identified eye-related issues.^[3] If ocular pathology is absent but visual function remains impaired, referral to a neuro-ophthalmologist, neurologist, or pediatric ophthalmologist is recommended for specialized assessment.^[31] Behavioral observations in natural settings, such as the child's response to toys, lights, or familiar faces, help evaluate functional vision and atypical patterns like field loss or latency in fixation.^[3] Neuroimaging, typically via MRI or CT scan, is employed to detect brain lesions contributing to CVI, such as periventricular leukomalacia or damage to visual processing pathways in preterm infants.^[3] If seizures are suspected—common in CVI cases—electroencephalography (EEG) is indicated per American Academy of Pediatrics guidelines for epilepsy evaluation in children.^[3] Diagnosis relies on multidisciplinary consensus among pediatricians, ophthalmologists, neurologists, and vision specialists, as no single test confirms CVI; it is established when deficits in visual function and functional vision cannot be explained by ocular conditions alone, aligning with the 2024 NIH working definition of CVI as a neurodevelopmental disorder involving brain-based visual pathway abnormalities.^[19] Challenges in diagnosis include significant delays, averaging 1 to 2 years from symptom onset, due to symptom variability, overlapping comorbidities like cerebral palsy, and the absence of standardized protocols.^[32] This process incorporates assessment of CVI phases, from basic visual skills to complex integration, to characterize the extent of impairment without relying on formal tools.^[19] Early identification is essential, as untreated CVI can hinder learning and development.^[3]

Assessment Tools

Observational tools play a central role in evaluating cortical visual impairment (CVI), focusing on behavioral responses to visual stimuli. The CVI Range Assessment, developed by Christine Roman-Lantzy, is a widely used functional vision tool that evaluates the impact of CVI across 10 key characteristics, including color preference, latency of visual attention, visual novelty, and complexity of visual stimuli.^[33] This assessment scores each characteristic on a 0-10 scale, delineating three phases of visual skill development—Phase I (limited vision with strong impact from CVI characteristics), Phase II (intermediate integration), and Phase III (approaching typical vision)—to guide intervention planning.^[34] Parental input is incorporated through questionnaires like the Dutch CVI Questionnaire, which gathers observations on daily visual behaviors in preschool children to support early screening and identification of CVI-related difficulties.^[35] Functional assessments adapt traditional vision testing for the brain-based nature of CVI, emphasizing behavioral responses over ocular exams. Preferential looking cards, such as Teller Acuity Cards, measure grating acuity by observing an infant's or child's eye preference for patterned stimuli, providing estimates of visual acuity in non-verbal individuals with CVI.^[15] Behavioral checklists aligned with Roman-Lantzy's phases I-III further assess functional vision by documenting responses to environmental factors like movement, lighting, and object familiarity, helping to quantify how CVI affects daily visual engagement.^[36] Advanced methods offer insights into neural underpinnings of CVI. Functional MRI (fMRI) evaluates neural activation in visual pathways, revealing atypical cortical responses to stimuli in children with CVI, as demonstrated in studies of infants with periventricular leukomalacia.^[37] Eye-tracking technology measures fixation duration, saccades, and visual attention patterns, distinguishing CVI profiles from typical development; recent National Eye Institute (NEI)-funded research has validated its use for classifying CVI subtypes through oculomotor analysis.^[38]^[4] Despite these tools, CVI assessment lacks a gold standard, with no single method fully capturing the heterogeneous impacts on visual function.^[39] Tools often vary by age group—preferential looking for infants versus phase-based checklists for school-age children—and require cultural adaptations for global applicability to account for environmental and linguistic differences.^[40]

Management and Intervention

Therapeutic Approaches

Therapeutic approaches for cortical visual impairment (CVI) emphasize rehabilitative and supportive strategies to optimize residual visual abilities, as no curative medical interventions exist. These methods leverage neuroplasticity, particularly in children, to enhance visual processing and functional participation in daily activities through targeted therapies and adaptations. Rehabilitative therapies form the cornerstone of management, with vision therapy programs utilizing high-contrast, single-object exposure to build visual attention and recognition skills. For example, early intervention visual training, delivered in sessions of 45 minutes three times per week for six months, has been shown to enhance visual processing in pediatric cases. Occupational therapy complements this by focusing on motor-visual integration, employing techniques such as pairing touch with vision using 3D high-contrast objects to improve reaching and object manipulation.^[41]^[42] Environmental modifications play a vital role in supporting visual function by minimizing distractions and enhancing salience. Strategies include reducing visual clutter with solid backgrounds and simple layouts, incorporating colored lighting or backlit materials, and integrating auditory cues to direct attention. Per established guidelines, these adaptations should progress gradually in complexity—from isolated, preferred objects to more integrated scenes—to prevent overload and promote skill development.^[16]^[43] No pharmacological agents specifically target CVI, though treatment of comorbidities is critical; for instance, anti-epileptic medications are used to manage associated seizures, which frequently accompany the condition due to underlying brain injury.^[3] Early intervention, initiated in infancy through programs under Part C of the Individuals with Disabilities Education Act (IDEA), is essential for leveraging developmental windows of plasticity. These services involve multidisciplinary teams, including occupational therapists for visual-motor skills, physical therapists for mobility, and speech therapists for communication, with individualized plans developed within 45 days of referral to foster overall development.^[3] The evidence supporting these approaches is derived primarily from observational studies and case series, as randomized controlled trials remain limited. Such research demonstrates functional improvements in visual acuity, processing, and motor integration with consistent therapy, outperforming outcomes in untreated cases.^[41]^[44]

Educational and Support Services

Educational accommodations for individuals with cortical visual impairment (CVI) are primarily delivered by Teachers of the Visually Impaired (TVIs), who provide specialized instruction in alternative formats such as braille instruction, preparation of enlarged print materials, and training on assistive technologies including screen readers and magnification software to enhance access to educational content.^[45] Under the Individuals with Disabilities Education Act (IDEA), students with CVI qualify for Individualized Education Programs (IEPs) that incorporate tailored accommodations like extended time for assignments, preferential seating to minimize visual distractions, and access to auditory learning aids, ensuring participation in the general curriculum.^[46] Section 504 plans, governed by the Rehabilitation Act, offer similar accommodations for students who do not require the intensive services of an IEP but still need modifications such as large-print textbooks or verbal descriptions of visual information to support academic success.^[47] Support services extend beyond the classroom to include family training programs that empower parents and caregivers with practical strategies for daily visual support, as provided through resources from the Perkins School for the Blind, which offer workshops on home adaptations and communication techniques.^[48] Transition planning, mandated by IDEA to begin at age 16, focuses on preparing individuals with CVI for adulthood by addressing employment barriers such as limited access to adaptive job training; programs like Career Launch at Perkins provide intensive career services, including resume building and workplace simulations tailored for visually impaired young adults.^[49] School integration for children with CVI involves creating sensory-friendly classroom environments with adaptations such as organized layouts to reduce visual clutter, consistent lighting to avoid glare, and color-coded materials to improve navigation and focus, which not only aid the student with CVI but also benefit the entire class.^[50] Peer education initiatives, where classmates learn about CVI through simple discussions or demonstrations, foster empathy and inclusion by highlighting how visual processing differences affect participation.^[29] These efforts are particularly important given the frequent overlap in behavioral manifestations, such as challenges in social interaction, which can lead to misdiagnosis of CVI as autism spectrum disorder.^[51] Community resources play a vital role in advocacy and support, with organizations like Prevent Blindness offering informational toolkits, webinars, and connections to local services for families affected by CVI.^[52] FamilyConnect, an online platform by the American Printing House for the Blind, provides articles, forums, and expert advice to help families navigate educational and daily challenges associated with visual impairment. September is designated as CVI Awareness Month, during which groups like the Perkins School for the Blind and the Pediatric Cortical Visual Impairment Society (PCVIS) run campaigns, social media toolkits, and events to promote early diagnosis and policy changes for better recognition of CVI.^[53] PCVIS specifically advocates for enhanced quality-of-life improvements through research funding and professional training initiatives.^[54] Access to educational and support services for CVI remains uneven, with significant disparities in rural and minority communities stemming from shortages of specialized providers, transportation challenges, and limited insurance coverage for vision-related aids. These inequities exacerbate vision loss burdens, as social determinants like poverty and geographic isolation hinder timely interventions, underscoring the need for targeted policies to ensure equitable distribution of resources.^[55]

Prognosis and Research

Long-term Outcomes

The prognosis for cortical visual impairment (CVI) is highly variable, with many children experiencing some degree of visual recovery over time, though full restoration is rare. Studies indicate that approximately 60% of children with brain damage affecting the visual cortex demonstrate visual recovery, often manifesting as improvements in visual acuity, fixation, and visual field awareness by school age. However, persistent higher-order visual processing deficits, such as difficulties with reading, object recognition, or simultaneous visual perception, contribute to ongoing challenges in daily functioning.^[7]^[6] Several factors influence these recovery patterns, including the timing of early diagnosis and intervention, as well as the severity and location of underlying brain damage. Children with milder hypoxic-ischemic events or localized lesions tend to have better outcomes compared to those with diffuse brain injury or periventricular leukomalacia, where recovery is more limited. Neuroplasticity in the developing brain plays a key role, allowing for adaptation and partial compensation through alternative neural pathways, particularly when interventions begin early.^[6]^[56] Lifelong effects of CVI often extend beyond vision, with affected individuals facing increased risks of unemployment and mental health challenges. Employment rates for adults with visual impairments, including those from CVI, stand at about 46%, compared to 79% in the general population, reflecting a roughly 40% higher unemployment risk due to barriers in education and workplace adaptation. Additionally, visual impairments are associated with elevated rates of anxiety and depression, imposing a significant psychological burden that can exacerbate social isolation. In cases of adult-acquired CVI, such as from stroke, recovery is generally poorer due to reduced neuroplasticity, with persistent visual field defects and slower rehabilitation progress.^[57]^[58] Despite these challenges, positive outcomes are possible, particularly in pediatric cases where neuroplasticity enables adaptation and functional independence for some individuals. According to the National Eye Institute, early therapeutic support can help children with CVI maximize their remaining vision and achieve greater autonomy in learning and daily activities. Lifelong monitoring by specialists, such as ophthalmologists and neurodevelopmental teams, is essential to adjust interventions for evolving needs, including changes during puberty that may alter visual behaviors or cognitive demands.^[1]^[6]

Current Research Directions

Recent milestones in cortical visual impairment (CVI) research include the NIH CVI Working Group's 2024 consensus, which defines CVI as a neurodevelopmental disorder characterized by deficits in visual function and functional vision caused by neurologic damage to visual pathways, emphasizing its spectrum nature and distinction from ocular impairments.^[19] Longitudinal studies have demonstrated neuroplasticity windows extending up to age 8, with early interventions like vision therapy showing significant improvements in CVI severity grades, particularly when initiated before this period, as age exerts a small positive effect on recovery (0.01 unit per month).^[59] Key research areas focus on developing standardized screening tools, such as AI-based methods combining eye tracking with generative models like SegCLIP to assess visual saliency for lower- and higher-order processing deficits in children as young as 12 months, enabling objective early detection and personalized interventions.^[60] Efficacy trials for vision rehabilitation, supported by the National Eye Institute (NEI) as a priority in its 2021 strategic plan, explore environmental adaptations and early intervention leveraging neuroplasticity, though no evidence-based treatments exist yet, with ongoing efforts to evaluate outcomes through prospective registries.^[19] Advances in neuroimaging, including functional MRI and resting-state connectivity mapping, aim to predict recovery by identifying abnormalities in visual pathways like the optic radiations and occipital cortex, with early interhemispheric functional connectivity in the visual cortex correlating with improved visual field outcomes over time.^[19]^[61] Genetic research investigates susceptibility factors, such as associations with metabolic disorders and perinatal hypoxic-ischemic encephalopathy, using models to uncover novel causes like mutations in genes affecting brain development, informing risk stratification in post-hypoxia cases.^[19]^[62] Addressing research gaps includes tackling disparities in diagnosis and treatment for underserved and minoritized populations, where higher CVI risk and limited access hinder early identification, prompting calls for equitable universal screening.^[3] Transition care from pediatric to adult services remains underdeveloped, with the American Academy of Pediatrics (AAP) 2024 priorities urging early planning to ensure continuity of accommodations and specialized vision needs into adulthood.^[3] Future potential lies in pharmacotherapies targeting neural repair, though current management relies on non-pharmacologic approaches, with NIH initiatives prioritizing studies on neuroplasticity timing and intervention efficacy to develop such options.^[19] Global epidemiology databases, such as the NEI-led CVI registry in development as of 2024, will collect longitudinal data to track prevalence—now the leading cause of pediatric visual impairment in industrialized nations—and inform worldwide research priorities. In 2025, a review of CVI interventions highlighted the need for more evidence-based therapies and experimental approaches to support children's vision and learning.^[19]^[63]

References

[1]
Cerebral Visual Impairment (CVI) - National Eye Institute - NIH
Dec 4, 2024 · Symptoms: Low vision, trouble seeing things like faces or moving objects · Diagnosis: Medical history, eye exam, brain scans, other tests that ...
[2]
Diagnosis and Care of Children With Cerebral/Cortical Visual ...
Nov 19, 2024 · Children with CVI can have profound visual impairment or have near-normal functional vision. It is important to query families about the child's ...Background · Definition · Etiology and Comorbidities · Recommendations...Missing: symptoms | Show results with:symptoms
[3]
[PDF] 2024-Chang-Merabet-CVI-working-definition.pdf
The NIH CVI Working Group proposes that CVI is a neu- rodevelopmental disorder characterized by deficits of visual function and functional vision that are ...
[4]
What Is Cerebral/Cortical Visual Impairment and Why Do We Need a ...
This new definition describes CVI as a spectrum of visual impairment caused by an underlying structural or functional brain abnormality that impacts the ...Missing: symptoms | Show results with:symptoms
[5]
Cerebral Visual Impairment - EyeWiki
Aug 26, 2025 · Cortical / Cerebral visual impairment (CVI) is defined as vision loss resulting from damage to the retrogeniculate pathway, in the absence ...
[6]
Visual function in the brain-damaged child | Eye - Nature
Apr 30, 2003 · It was not until the early 19th century that many scientists began to ... Most studies of cortical visual impairment have described the ...
[7]
Assessment of cerebral visual impairment with the L94 visual ...
Feb 19, 2009 · The term CVI was introduced in the 1990s since – in contrast with earlier findings – visual impairment was found to arise more often from ...
[8]
Cerebral/Cortical Visual Impairment Working Definition
In the first descriptions of CVI, children demonstrated (often profound) decreased visual acuity associated with damage to the occipital cortex, optic ...<|control11|><|separator|>
[9]
Diagnosis and Care of Children With Cerebral/Cortical Visual ...
In 2019, a study from a tertiary eye care center in South India reported that 44% of children younger than 3 years with profound visual impairment were ...
[10]
NIH-led scientific team defines elements of brain-based visual ...
Nov 19, 2024 · Brain Involvement: CVI encompasses a spectrum of visual impairments that involve an underlying brain abnormality affecting the development of ...
[11]
Cerebral visual impairment is a major cause of profound visual ...
We found that the prevalence of CVI is similar among children belonging to rural 34 (42.3%) and urban 56 (50.5%) areas (P = 0.10).
[12]
Cerebral visual impairment-related vision problems in ... - PubMed
Feb 3, 2021 · We identified 78 out of 248 children (31.5% of those examined, 3.4% of the total sample), who had at least one CVI-related vision problem. The ...
[13]
Occurrence and Time of Onset of Intraventricular Hemorrhage in ...
Dec 30, 2024 · A 2022 meta-analysis by Lai et al found that the incidence of any IVH was 34.3%, whereas the incidence of severe IVH was 15.0% for infants with ...
[14]
Cortical Visual Impairment | Boston Children's Hospital
Cortical visual impairment is when children show abnormal visual responses that aren't caused by the eyes. Learn more from Boston Children's.
[15]
Cortical Visual Impairment (CVI): Symptoms & Treatment
Co-occurring conditions. Kids with this condition often have other brain-related diagnoses, like: Autism spectrum disorder · Cerebral palsy · Developmental ...Symptoms And Causes · Management And Treatment · Additional Common Questions
[16]
Review of Ocular Manifestations of Joubert Syndrome - PMC
Dec 4, 2018 · Joubert syndrome is a group of rare disorders that stem from defects in a sensory organelle, the primary cilia.
[17]
Neuroplasticity in Cerebral Visual Impairment (CVI) - PubMed Central
The behavioral characteristics and visual dysfunctions observed in CVI (first described by Jan and colleagues) are markedly different compared to children with ...Missing: citation | Show results with:citation<|control11|><|separator|>
[18]
https://pmc.ncbi.nlm.nih.gov/articles/PMC6949360/
[19]
Neural correlates associated with impaired global motion perception ...
Sep 21, 2021 · Overall, our results suggest that global motion processing deficits in CVI may be associated with impaired signal integration and segregation ...
[20]
Examining degree of impact and CVI phases
Christine Roman-Lantzy's CVI Phases · Phase I, Building consistent visual attention · Phase II, Integrating vision with function · Phase III, Refinement of CVI ...
[21]
MR correlation. Cerebral visual impairment in periventricular
CONCLU-. SION: This study clearly establishes a relationship between specific MR findings and visual impairment in children with periventricular leukomalacia.
[22]
Assessment of Cortical Visual Impairment in Infants with ... - NIH
We wanted to investigate the usefulness of event-related (ER) functional MRI (fMRI) for the assessment of cortical visual impairment in infants with ...
[23]
Cortical Visual Impairment (CVI) - Prevent Blindness
trouble responding to visual cues · trouble noticing details in a busy, cluttered, or moving image · trouble recognizing faces or objects while viewing them.
[24]
The 10 Characteristics of CVI | Pediatric Cortical Visual Impairment ...
Christine Roman-Lantzy, PhD, defines 10 characteristics of visual behavior that are quantifiable using the CVI Range.<|control11|><|separator|>
[25]
Cerebral Visual Impairment Characterized by Abnormal ... - PMC
May 13, 2021 · Children with these conditions present with poor visual tracking in infancy but have normal visual ... infants. Invest Ophthalmol Vis Sci ...
[26]
Cortical blindness: Symptoms, causes, and treatment
Feb 16, 2024 · Unchanged pupil reflexes: With cortical blindness, the pupils typically react in the usual way to light. This is not usually the case in people ...
[27]
What does CVI look like? - Perkins School For The Blind
Manifestations of CVI in children · 1. Trouble with direct eye contact and social gaze. This is often the behavior that parents spot first. · 2. Clumsiness.
[28]
Cortical Visual Impairments and Learning Disabilities - PMC
Oct 13, 2021 · Cortical or cerebral visual impairments (CVIs) include a wide range of visual dysfunctions that can impair learning and social interaction.The Differences Between... · Definitions, Epidemiology... · Behavioral Expressions Of...
[29]
ADHD and CVI explained - Perkins School For The Blind
CVI can look like ADHD and even overlap with it, but each condition needs different support. Meanwhile, CVI is still too often misdiagnosed.
[30]
How is CVI diagnosed? - Perkins School For The Blind
A CVI diagnosis is typically made by a pediatric ophthalmologist, optometrist, neuro-ophthalmologist, clinical low vision specialist, or sometimes a neurologist ...Cvi Is The Leading Cause Of... · How Is Cvi Diagnosed? · Next Steps After A Cvi...
[31]
Cerebral visual impairment (CVI) overlooked: A retrospective review ...
Sep 10, 2025 · It took an average of 1 year to diagnose children with CVI in nearly 50% of the current cohort. Some intervals between the first visit and the ...
[32]
Cortical Visual Impairment: An Approach to Assessment and ...
This one-of-a-kind resource provides readers with both a conceptual framework with which to understand working with CVI and concrete strategies to apply ...
[33]
CVI Part Two: Dr. Christine Roman's CVI Range Assessment
May 21, 2014 · The CVI Range has a 0-10 scale, and describes three broad phases of visual processing development. Where the child falls on the range corresponds to the types ...
[34]
https://cprn.org/cvi-part-two-dr-romans-cvi-range-assessment/
[35]
The CVI Range – Christine Roman, PhD - CVI Resources
The first iteration was a checklist. It was a single sheet with statements that described the unique behaviors of CVI. In 1994-6, I conducted research at the ...
[36]
Assessment of Cortical Visual Impairment in Infants with ...
Jul 22, 2011 · We wanted to investigate the usefulness of event-related (ER) functional MRI (fMRI) for the assessment of cortical visual impairment in infants ...
[37]
Cerebral/Cortical Visual Impairment Classification and ...
Cerebral/cortical visual impairment (CVI) is a leading cause of pediatric visual impairment and is frequently associated with abnormal ocular motility.
[38]
Validity and reliability of CVI Range assessment for Clinical ...
Oct 31, 2022 · The CVI Range was developed by Dr Christine Roman-Lantzy as a tool for understanding a child's functional vision and applying targeted ...
[39]
The Multidisciplinary Guidelines for Diagnosis and Referral in ...
Jun 29, 2022 · Perinatal hypoxic-ischemic damage is the most frequent cause of CVI but CVI can also be the result of a genetic disorder. The majority of ...<|separator|>
[40]
A Systematic Review on the Management of Cortical Visual ...
Jun 30, 2024 · General management of CVI includes training in vision, physical, cognitive, and behavioral skills [11-13]. Managing CVI in children involves ...
[41]
[PDF] Occupational Therapy: Cortical Visual Impairment
Dec 7, 2022 · CVI is exceedingly common and is known to be the leading cause of visual impairment in children in the United States and developed countries ( ...
[42]
[PDF] STRATEGIES FOR WORKING WITH CHILDREN WITH CORTICAL ...
The following is a compilation of strategies useful for working with children having Cortical Visual Impairment (CVI). Jeanne Gardier is an educational ...
[43]
Longitudinal neurological analysis of moderate and severe pediatric ...
Aug 15, 2022 · Longitudinal follow-up of CVI patients showed that interventional therapies demonstrated vision function improvement greater than no therapy and ...
[44]
A guide to common CVI IEP accommodations
Here's an overview of general CVI accommodations created by the Perkins CVI Assessment Team and based on multiple perspectives in the field.
[45]
CVI and the IFSP/IEP | Pediatric Cortical Visual Impairment Society
Students with CVI must have a CVI-rich IEP to ensure all services, interventions, and accommodations are meaningful, intentional, and based on the CVI Range© ...
[46]
504 Plans for Students who are Blind or Visually Impaired
Jun 15, 2025 · Accommodations Under Section 504 · Extended time on tests or assignments · Preferential seating (e.g., near the board or away from distractions) ...
[47]
Transition Center Resources - Perkins School For The Blind
Our Transition Resources include. Guides and articles to help families and young adults create a rich and meaningful future after high school. Including a ...
[48]
Transition: Success after secondary education for people with CVI
Check out Career Launch @ Perkins, an innovative training and career services program helping blind and visually impaired young adults land career-track jobs.
[49]
These 9 easy classroom adaptations can help kids with CVI
but all students in all classrooms!Missing: peer misdiagnosis autism
[50]
Autistic-Like Features in Visually Impaired Children - PubMed Central
Aug 1, 2020 · Autistic-like symptoms shown by some children with VI might reflect the influence of the visual deficit, or represent a primary neurodevelopmental condition.Missing: misdiagnosis | Show results with:misdiagnosis
[51]
Cortical Visual Impairment Resources
Promotes independence of people who are blind and visually impaired by providing specialized materials, products, and services needed for education and life.Informative Websites... · Books · Advocacy
[52]
CVI Awareness Month Social Media Toolkit
Help raise CVI awareness during September, CVI Awareness Month, with our social media toolkit!
[53]
Pediatric Cortical Visual Impairment Society - Helping Children with ...
The Pediatric Cortical Visual Impairment Society mission is to advocate for improvement in the quality of life of children with vision loss due to CVI.
[54]
Advancing Health Equity: Social Determinants and Vision Loss - CDC
May 15, 2024 · Outlines the social determinants of health and how disparities affect the burden of vision loss.Missing: cortical rural 2025
[55]
Cortical Visual Impairments and Learning Disabilities - Frontiers
Oct 12, 2021 · Cortical or cerebral visual impairments (CVIs) include a wide range of visual dysfunctions that can impair learning and social interaction.
[56]
Continuity of Employment for People with Visual Impairments - NIH
The most recent American Community Survey (ACS) data indicate that only 46.2% of people with visual impairments were employed in 2019 compared to 78.6% of ...
[57]
Implications of cerebral/cortical visual impairment on life and learning
Dec 9, 2024 · A recent US prevalence study found at least 180,000 kids with CVI or likely CVI, but less than 20% have a CVI diagnosis (Perkins School for the ...
[58]
Longitudinal neurological analysis of moderate and severe pediatric ...
Aug 16, 2022 · Longitudinal follow-up of CVI patients showed that interventional therapies demonstrated vision function improvement greater than no therapy and ...
[59]
Method for assessing visual saliency in children with cerebral ...
In India, CVI was diagnosed in 44% of young children with profound visual ... 3 years: a study from tertiary eye care center in South India. Indian J ...
[60]
Early Functional Connectivity Predicts Recovery from Visual Field ...
The impaired interhemispheric RSFCs in the visual cortex recovered over time, although a lower level of RSFC was still observed at 3 months compared to healthy ...
[61]
Novel genetic causes for cerebral visual impairment - PubMed Central
Sep 9, 2015 · Our findings show that diverse genetic causes underlie CVI, some of which will provide insight into the biology underlying this disease process.