The ocular pathology in Leber's congenital amaurosis

T. J. Sullivan, J. G. Heathcote, S. M. Brazel, M. A. Musarella

Research output: Contribution to journalArticlepeer-review

14 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)25-31
Number of pages7
JournalAustralian and New Zealand Journal of Ophthalmology
Volume22
Issue number1
DOIs
Publication statusPublished - 1994
Externally publishedYes

ASJC Scopus Subject Areas

  • Ophthalmology

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