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February 1984

Aicardi's Syndrome: A Clinicopathologic Study

Author Affiliations

From the Departments of Ophthalmology (Drs Bell and McMahon) and Pathology (Drs Moore and Ludwin), Queen's University and Affiliated Hospitals, Kingston, Ontario.

Arch Ophthalmol. 1984;102(2):250-253. doi:10.1001/archopht.1984.01040030200026

• A 9-week-old female infant was seen with typical clinical features of Aicardi's syndrome. At autopsy (at 21 months of age), examination of the brain disclosed polymicrogyria and agenesis of the corpus callosum. Histopathologic studies of the posterior segments showed areas of intact but attenuated and depigmented retinal pigment epithelium, and atypical colobomatous defect of both posterior segments, and ectatic scleral channels lined by a retinal pigment epithelial choriocapillarislike complex in the left eye. The pathogenesis of the ocular defects is discussed.

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