Visual development of infants with severe ocular disorders.
Fielder A R, Fulton A B, Mayer D L
AI Summary
Infants with severe ocular disorders often develop measurable vision and guided behavior, suggesting significant visual pathway maturation despite early blindness, offering hope for improved function.
Abstract
Among 11 patients who presented as blind in early infancy, with Leber's congenital amaurosis (5 patients), optic nerve hypoplasia (4 patients), or macular colobomata (2 patients), 8 developed visually guided behavior and measurable grating acuity by age 5 to 46 months. All children with measurable grating acuity demonstrated visually guided mobility. Grating acuity was predictive of later visual performance in 10 of 11 patients by age 12 to 16 months. The best grating acuity attained by 7 months was 1.3 to 3.0 cycles/degrees (20/460 to 20/200) and 0.13 cycles/degrees (20/4700) by month 8. Two patients with Leber's congenital amaurosis and one with optic nerve hypoplasia remained blind. No clinical features existed to differentiate these three patients from the eight whose visual status improved. Posterior visual pathway maturation may underlie the improvement.
MeSH Terms
Shields Classification
Related Articles5
Progressive Optic Neuropathy in Hydrocephalic Ccdc13 Mutant Mice Caused by Impaired Axoplasmic Transport at the Optic Nerve Head.
Basic ScienceOptic Nerve Hypoplasia Is a Pervasive Subcortical Pathology of Visual System in Neonates.
Basic ScienceSporadic bilateral optic neuropathy in children: the role of mitochondrial abnormalities.
Case-Control StudyCauses of childhood low vision and blindness in New Zealand.
Observational StudyPostretinal Structure and Function in Severe Congenital Photoreceptor Blindness Caused by Mutations in the GUCY2D Gene.
Observational StudyIs this article assigned to the wrong chapter(s)? Let us know.