THE OCULAR PATHOLOGY IN LEBERS CONGENITAL AMAUROSIS

Citation
Tj. Sullivan et al., THE OCULAR PATHOLOGY IN LEBERS CONGENITAL AMAUROSIS, Australian and New Zealand journal of ophthalmology, 22(1), 1994, pp. 25-31
Citations number
25
Categorie Soggetti
Ophthalmology
ISSN journal
08149763
Volume
22
Issue
1
Year of publication
1994
Pages
25 - 31
Database
ISI
SICI code
0814-9763(1994)22:1<25:TOPILC>2.0.ZU;2-Y
Abstract
Purpose: To present the ocular pathology of a three-year-old child wit h Leber's congenital amaurosis (LCA) who died from an unrelated episod e of presumed viral meningitis. Methods: Autopsy was performed, and in addition the globes were harvested for pathological examination. Befo re the child's death, known systemic disorders with infantile retinal dystrophy were excluded. Results: The outer nuclear layer and photorec eptor layers are the primary site of retinal pathology in LCA. Conclus ion: This report of the pathological findings in LCA, early in the nat ural history of the disease, indicates that process appears to be a de generation rather than an agenesis.