Special Commentary: Cerebral/Cortical Visual Impairment Working Definition

Abstract

Cerebral/cortical visual impairment (CVI), a brain-based condition, has emerged as a leading cause of pediatric visual impairment in the United States and other industrialized nations. The National Eye Institute (NEI) recognized CVI as a priority area for research as part of their 2021 NEI Vision for the Future Strategic Plan and partnered with the Eunice Kennedy Shriver National Institute of Child Health and Human Development and the National Institute of Neurologic Disorders and Stroke within the National Institutes of Health (NIH) to sponsor a CVI Workshop in November 2023. A panel consisting of a group of clinicians with expertise in diagnosing CVI convened to draft a working definition for this condition. Five key elements were identified: (1) CVI encompasses a spectrum of visual impairments caused by an underlying brain abnormality that affects the development of visual processing pathways and is characterized by deficits in visual function and functional vision; (2) the visual dysfunction in CVI is greater than expected by any comorbid ocular conditions alone; (3) the visual dysfunction in CVI may manifest as lower-order or higher-order afferent visual deficits, or both, leading to characteristic behaviors in affected individuals; (4) although CVI may be comorbid with other neurodevelopmental disorders, CVI is not primarily a disorder of language, learning, or social communication; and (5) the underlying neurologic insult of the developing brain may go unrecognized or undiagnosed until later in life. Future work is needed to achieve consensus on nomenclature, diagnostic criteria, and strategies for early identification and intervention. The NIH is developing a CVI registry to collect relevant demographic and clinical data prospectively and longitudinally to help inform future research questions and to provide insight into considerations for future clinical trials in the field of CVI.