Sight Unseen: The Importance of Recognizing Cerebral Visual Impairment in Pediatric Patients

Over 19 million children worldwide are affected by visual impairment.⁵ In the United States, cerebral visual impairment (also known as cortical visual impairment, or CVI) is a leading cause of vision loss among children.¹ Moreover, 10.5 percent of all children with developmental challenges have CVI.¹ With its unique characteristics, CVI is often under or misdiagnosed.

As pediatric therapists, it is likely that, at some point in our careers, we will encounter a child with CVI. Without proper diagnosis guided by the CVI range, opportunities for intervention can easily be missed. Typically, a child with CVI has a normal eye exam or has an eye condition that cannot account for the abnormal visual behavior, making familiarity with CVI and other common vision diagnoses essential for every pediatric therapist.

Emily’s Story

Years ago, I received an early intervention (EI) physical therapy referral from hospice for a child named Emily. At birth, Emily was not expected to live beyond a few days. When referred to our EI program at one month of age, she had been diagnosed with hydrocephaly and significant white matter loss/damage. Upon evaluation, Emily was challenged by every skill common in newborns, apart from breathing and cardiac regulation. She did not visually recognize anything, including light, and did not have an active blink-to-threat reflex when something came close to her face.

Three years after Emily’s referral to our EI program, Emily could see more than 40 feet, name letters, shapes, and colors, walk with a gait trainer, sing, and crack jokes. Each year following Emily’s departure from our program, her mother gave me a framed photo of her brain MRI. Each successive photo displayed thickening of Emily’s brain cortex. She became one of the first of my many patients with CVI, and her success propelled my journey as therapist and life-long learner around the concept of how essential vision can be to early learning.

Emily’s story demonstrates the significance of recognizing CVI as a roadblock to natural development. For all intents and purposes, she initially presented as a fully visually impaired child. However, with targeted intervention from a team of therapists and physicians who understood her CVI diagnosis and tailored her treatments accordingly, she experienced neuroplastic changes and the roadblock of her visual impairment did not stand in her way.

What Is Cerebral Visual Impairment?

CVI is a congenital or acquired brain-based visual impairment with onset in childhood, unexplained by an ocular disorder, and associated with unique visual and behavioral characteristics.⁶ In other words, CVI is the name for vision problems that stem from the brain rather than the eyes. The brain struggles to interpret and process what the child is trying to visualize, which then affects visual abilities.

Who Is at Risk for Cerebral Visual Impairment?

Patients who have suffered an injury to the brain are at highest risk for CVI. Often, these injuries happen before, during, or shortly after birth. Common causes of CVI in babies and young children include:

• Premature births
• Intraventricular hemorrhage
• Lack of oxygen/blood supply to the brain, such as with a stroke
• Hydrocephalus
• Infections that reach the brain
• Head injury
• Certain genetic conditions

Common Characteristics of Cerebral Visual Impairment

Visual characteristics common to children with CVI include:

• A distinct color preference (commonly red, orange, yellow)
• A preference for lighted and moving objects
• Avoidance of visually complex things (i.e., faces and objects or pictures with complex backgrounds)
• Reaching for toys or objects using peripheral vision (looking away) rather than central vision
• Delayed visual responses
• Difficulty with novel objects or settings
• Light gazing

Screening for Cerebral Visual Impairment

A comprehensive visual history and screening is essential to the pediatric assessment and diagnosis of children with CVI.² Communicating findings with other team members including a pediatric optometrist or ophthalmologist, neurologist, or neuro-ophthalmologist is also critical. Pediatric therapists often have a front row seat to functional vision within a child’s natural environment and can be a bridge of meaningful communication between the patient and eye specialist. If you have access to a vision specialist, many are trained to assess CVI using the CVI Range.⁴ Co-treatment with them is an exceptional pathway to providing appropriate treatment.

Guidance for Therapists Treating Cerebral Visual Impairment

Unfortunately, there is not yet a cure for CVI. However, with EI that includes therapy, educational support, and other special services, young patients with CVI can learn essential vision rehabilitation skills to help them develop and make the most of their vision. Once a therapist understands the characteristics, needs, and ongoing assessment tools unique to CVI, they can tailor treatment using environment, materials, and therapeutic activities to promote both visual and developmental progress.

In our course, Roadblocks to Natural Development Part 1, Lacy Morise and I discuss common pediatric roadblocks, such as undetected vision loss, that significantly impact natural development. This problem-and-solution style lecture will empower you with greater knowledge about CVI, as well as other common vision impairments, and strengthen your ability to effectively detect and treat these diagnoses.