Cognitive visual impairment with good visual acuity in children with posterior periventricular white matter injury: A series of 7 cases

Abstract

We describe a series of term and preterm children with occipito-parietal periventricular white matter abnormalities on magnetic resonance imaging (MRI) who manifest common clinical features of cognitive visual dysfunction. We also describe a strategy for taking clinical history that highlights the symptom complex and assists with management. A retrospective observational case series study of seven children with cognitive visual difficulties despite good (20/32 or better) visual acuities and MRI findings of periventricular white matter pathology in the territory subserving visual function. Structured history taking was used to identify and characterize the visual difficulties. Objective clinical findings (visual acuity, color vision, stereoacuity, visual fields, ocular motility, refraction, and fundoscopy) were recorded. Seven children with visual acuities of 20/32 (0.200 logMAR) or better had symptoms of cognitive visual difficulties consistent with dorsal stream dysfunction. Four had strabismus of different types and were identified on the basis of parental concern expressed at the ocular motility clinic. The other three children presented to the general ophthalmology clinic. All seven children had various degrees of focal periventricular white matter pathology in a similar distribution on neuroimaging. Children born prematurely are susceptible to periventricular white matter pathology. Such pathology can also occur in children born at term. This case series demonstrates that cognitive visual impairment due to periventricular white matter injury can occur despite good central visual function. A range of strategies to help affected children is described.