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The ocular pathology in Leber's congenital amaurosis
T J Sullivan1, J G Heathcote, S M Brazel
1Department of Ophthalmology, Hospital for Sick Children, Toronto, Canada.
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.