Despite its prevalence, CVI remains widely misdiagnosed and misunderstood worldwide. Often invisible or masked behind other co-existing conditions, this brain-based visual impairment does not affect the function of the eyes, but that of the brain. It causes damage to the brain’s visual processing pathways, making it difficult for the individual to interpret what they see. For these reasons, CVI is considered a disability of access, in which children with CVI are often unable to make sense of the visual world around them.
The working definition of CVI from the National Eye Institute (NEI) and National Institutes of Health (NIH)1 refers to five key elements of CVI, including the presence of an underlying neurologic insult or injury affecting the visual processing pathways in the brain, functional vision that cannot be explained by co-existing ocular conditions (the individual’s eyes are often healthy), and the presence of characteristic visual behaviors (such as those in Table 1). The definition also recognizes common comorbidities such as cerebral palsy (CP), as up to 83% of individuals with CP also have CVI, and other neurodevelopmental disorders, such as autism and dyslexia. Critical next steps in the NEI’s strategic plan is to determine the reliability and validity of functional vision assessments.2
Pediatric VIEW, founded in 1999 and now housed by The Children’s Home of Pittsburgh and Lemieux Family Center, was developed in response to an overwhelming request from families searching for CVI-specific assessment and direction for intervention. The practice, serving individuals birth to 23, their families, and teams, has continued to grow beyond Pittsburgh, PA. The primary mission is that the needs of individuals with CVI and the priorities of their families come first. Families and children are met every day at different stages of their educational journey. Parents and caregivers witness, firsthand, how their child’s vision can be unreliable – a mesh of ambiguous form and color – especially when environments and materials are visually complex. Families report the ways that their child struggles to access learning inside and outside of the classroom or therapy center. The struggles can include recognizing classmates, engaging in opportunities for interactive play and social development, feeling safe and secure in their environment, recognizing objects and activities around them, interpreting materials in their curricula, and building independence in daily routines. Families are searching for specific, effective intervention and support from educators, early interventionists, therapists, and advocates. It’s important to bring sense to this otherwise senseless visual world.
Table 1 : The 10 Characteristics of CVI
- Need for or attention to light
- Need for or attention to color
- Need for or attention to movement or objects with reflective properties
- Difficulty with visual complexity (objects, array, sensory environment, faces)
- Difficulty visually interpreting novelty (people, environments, objects)
- Visual latency
- Visual field preferences
- Difficulty with distance viewing
- Difficulty with visually guided reach (visual-motor)
- Abnormal responses to visual threat and touch
With research breakthroughs in visual neuroplasticity and the expectation for functional vision to improve in individuals with CVI, the primary responsibility rests on timely and appropriate early intervention (EI). Dealing with age-dependent neuroplasticity, “the assessment and management of children with CVI require a multidisciplinary approach to facilitate access to appropriate services and accommodations throughout childhood and beyond.”3 It is inherently challenging to provide appropriate EI services for a condition that is often missed or misdiagnosed. EI service provision will vary from state to state. Children with CVI and their families often find themselves on a long and difficult search for adequate and appropriate services that can address their unique visual needs. Some may never find it.
“In my family’s experience,” shares Mara LaViola, an advocate and CVI parent, “the greatest hurdles include lack of awareness, dismissal from medical professionals, systemic neglect and misdiagnosis (often related to autism), a lack of appropriately trained professionals, and a failure to act proactively.” To further complicate matters, even when families are promptly aligned with services, “many children are met with professionals who lack the training necessary to recognize and address brain-based visual impairments. This gap leads to missed opportunities for timely intervention and hinders the child’s overall progress.”
TABLE 2 : Common Causes of CVI
If your child has experienced any of the below, they should be screened for CVI.
- hydrocephalus
- intraventricular hemorrhage (IVH)
- periventricular leukomalacia (white matter damage) in prematurity
- hypoxic-ischemic encephalopathy
- metabolic and genetic disorders
- infections such as meningoencephalitis
- seizures and infantile spasms
- drug exposure
- non-accidental head trauma, near-drowning incidents
- rare genetic conditions
- central nervous system malformations
As Mara describes, without CVI-specific EI, children with CVI fall behind on critical opportunities for functional vision development. EI team members may not have received CVI-specific training, or the team does not include a vision professional to lead CVI-specific intervention. Whatever the reason, by age 3, when many states transition children out of EI services and into school-aged services, children with CVI are already playing catch-up. Instead of intervening in a way that sets these individuals up for success – the core purpose of this federally mandated program – the very opposite may be done unintentionally.
In 2017, before her son was 9 months old, Kathryne Hart discovered Dr. Roman-Lantzy’s research, identifying the 10 Characteristics of CVI. When she discussed her observations and concerns with her son’s ophthalmologist, he was diagnosed with CVI, and immediately scheduled to receive a functional vision assessment for CVI, the CVI Range Assessment©. With clear assessment results describing her son’s functional vision, Kathryne had a plan she could bring to his team. “I ended up having to train our early interventionists myself, and yet, I’d still consider myself one of the lucky ones. I had the education and resources to not settle for what I was given.” EI, when driven by an individual who considers the unique manifestations of CVI, can change an individual’s course to access for the rest of their educational career.
“We need a systemic shift in how CVI is identified and addressed, starting with better education for professionals and parents. Increased training for personnel across all disciplines, and a commitment to early and proactive intervention is needed,” notes Mara. “Collaboration and specialization should become the standard to ensure children like my son receive the care and support they deserve.”
Structured Collaboration and Best Practices
Certainly, no one team member can tackle this multi-pronged condition alone. Teams will look different, depending on the needs of the individual. A team may require specialists in AAC (Augmentative and Alternative Communication), orientation and mobility (COMS), or assistive technology (CATIS), while others may have different priorities.
Early identification through infant/NICU screening: The earlier individuals at-risk for CVI can be identified, the sooner they can be referred for further examination by neurology, ophthalmology, optometry and ultimately, initiate EI services in their state.
CVI-specific functional vision assessment: The CVI Range is an activities-based behavioral assessment of functional vision administered by certified examiners, typically teachers for the visually impaired. Through parent interview, observation, and direct assessment, the examiner scores the child’s performance regarding the 10 characteristics of CVI (Table 1), determining if a child is in Phase I, II, or III.2 While there are a variety of CVI inventories and questionnaires available to clinical and educational teams, the CVI Range Assessment provides a critical element, a quantitative measure of the impact of CVI on a child’s functional vision. The score, when determined by a skilled assessor, offers families and teams a baseline from which to measure the progress and effectiveness of interventions, over time. It is used to guide accommodations and CVI-specific instructional methods, and directly inform the development of an Individualized Family Service Plan (IFSP) and Individualized Educational Plan (IEP) goals, supporting the team through strategic and data-based early intervention, and beyond.4
Collaboration in CVI-specific EI services: Once an individual has been identified as having CVI, they should receive a referral to the state department, providing EI services and a functional vision assessment that can determine the degree of impact on their functional vision. Services beyond that point should include a plan, routed in CVI principles, that address the individual’s visual needs. It can be used to initiate an ongoing and collaborative partnership between the EI team and individuals who specialize in CVI services.
Strategic educational planning for individuals with CVI, beyond EI: Once a child turns 3 or 5 years old (depending on the state) and transitions out of EI services, that intervention should not end. On the contrary, as an individual continues to build functional vision and utilize vision as a reliable learning channel, IFSP and IEP-mandated services may need to increase to meet the growing demands of curricula and daily routines. “Children with CVI have a range of challenges that require long-term intervention and monitoring. Improved public health policies and education are important to optimally support children with CVI, their families, and those who provide care for them.”3
Routine evaluation of progress to inform ongoing team collaboration: As functional vision is expected to improve with strategic and thoughtful approaches to intervention, it’s critical to conduct annual assessment and progress monitoring. IFSP and IEP teams often meet once a year. Consistent, routine collaboration amongst team members can directly impact the effectiveness of service time.
Ongoing professional development for those working with individuals with CVI: Professionals can be better prepared across all disciplines if robust coursework on CVI is integrated into graduate level and medical school curricula. Before they enter their respective fields, and meet children with CVI in the exam room, classroom, or therapy center, professionals should be familiar with the heterogeneity of the condition, recognize red flags for diagnosis, and understand their state’s policies on next steps for referral and intervention. This can create more supportive networks and direct, hassle-free paths toward appropriate services.
Continually evolving to meet the unique needs of children with CVI: Currently, CVI-specific programs with skilled professionals can be found across the US, but the number of service providers remains gravely disproportionate to the number of children requiring services. To meet this overwhelming demand, new and existing programs must be willing to dig in and do the hard work. In return, they will see immeasurable rewards in their student or client’s progress.
A Wider View : CVI Resources
Resources serving individuals with CVI, their families, and teams around the US include:
Pediatric VIEW Program
Children's Home Of Pittsburgh
www.childrenshomepgh.org/our-services/pediatric-view-program
Pediatric Cortical Visual Impairment Society
https://pcvis.vision
The Bridge School
https://cvi.bridgeschool.org
Connections Beyond Sight and Sound
https://marylanddb.org
Paths to Literacy
www.pathstoliteracy.org/learning-center/cvi
Perkins School for the Blind
www.perkins.org/our-work/cvi
Texas School for the Blind and Visually Impaired
www.tsbvi.edu
West Virginia Department of Education
wvde.us/special-education/resources-sp-page/low-incidence-disabilities/cvi-special-topics
It’s what is done during early intervention in the classroom, home therapies, medical exam rooms, the community, at times of transition, in professional development coursework, during IEP meetings, when service time and critical goals are determined, that make the difference. The earliest stage possible that intervention happens can change an individual’s course.
References
1. Chang, Melinda Y.Borchert, Mark et al. Special Commentary: Cerebral/Cortical Visual Impairment Working Definition. Ophthalmology, Volume 131, Issue 12, 1359 – 1365 https://www.aaojournal.org/article/S0161-6420(24)00565-7/fulltext
2. Melinda Chang, Christine Roman-Lantzy, Sharon H O’Neil, Mark W Reid, Mark S Borchert. Validity and reliability of CVI Range Assessment for Clinical Research (CVI Range-CR): a longitudinal cohort study: BMJ Open Ophthalmology 2022;7:e001144.
3. Sharon S. Lehman, Larry Yin, Melinda Y. Chang, Section On Ophthalmology, Council On Children With Disabilities; American Association For Pediatric Ophthalmology And Strabismus, American Academy Of Ophthalmology; American Association Of Certified Orthoptists; Diagnosis and Care of Children With Cerebral/Cortical Visual Impairment: Clinical Report. Pediatrics 2024; e2024068465. 10.1542/peds.2024-068465
4. Roman-Lantzy, C. (2018). Cortical Visual Impairment: An Approach to Assessment and Intervention. 2nd ed., New York, NY: AFB Press.
5. Nielsen, L.S. et al. (2007). Visual dysfunctions and ocular disorders in children with developmental delay. I. prevalence, diagnoses and aetiology of visual impairment. Acta Ophthalmol Scand, 85: 149-56.
6. Hatton, D.D. et al. (2007). Babies Count: the national registry for children with visual impairments, birth to 3 years. JAAPOS, 11: 351-5.
7. Matsuba, C.A., Jan, J.E.. (2006). Long-term outcome of children with cortical visual impairment. Dev Med Child Neurol, 48: 508-12.
8. Swaminathan, M. (2011). Cortical visual impairment in children — A new challenge for the future? Oman Journal of Ophthalmology, 4(1), 1–2. http://doi.org/10.4103/0974-620X.77654
9. Mass. Eye and Ear Communications (2018, Feb 12). Blindness of the Brain: Explaining CVI. Retrieved from https://focus.masseyeandear.org/blindness-brain-explaining-cvi/
10. The Laboratory for Visual Neuroplasticity (2019). Neuroplasticity Associated With Cortical/Cerebral Visual Impairment. Retrieved from https://scholar.harvard.edu/merabetlab/research
11. Martín MB, Santos-Lozano A, Martín-Hernández J, López-Miguel A, Maldonado M, Baladrón C, Bauer CM, Merabet LB. Cerebral versus Ocular Visual Impairment: The Impact on Developmental Neuroplasticity. Front Psychol. 2016 Dec 26;7:1958. doi: 10.3389/fpsyg.2016.01958. PMID: 28082927; PMCID: PMC5183596.
ABOUT THE AUTHOR:
Francesca Crozier-Fitzgerald, MS, MEd, is a Certified Teacher of Students with Visual Impairment and a Practitioner at Pediatric VIEW of The Children’s Home of Pittsburgh and Lemieux Family Center. She has devoted her career to providing assessment, instruction and support to individuals with CVI, their families and their teams. Francesca is a Co-President of the Board of Pediatric Cortical Visual Impairment Society.
Read the article here.