Purpose: To present the ocular pathology of a three-year-old child with Leber's congenital amaurosis (LCA) who died from an unrelated episode of presumed viral meningitis.
Methods: Autopsy was performed, and in addition the globes were harvested for pathological examination. Before the child's death, known systemic disorders with infantile retinal dystrophy were excluded.
Results: The outer nuclear layer and photoreceptor layers are the primary site of retinal pathology in LCA.
Conclusion: This report of the pathological findings in LCA, early in the natural history of the disease, indicates that process appears to be a degeneration rather than an agenesis.