PATHOLOGY OF IRIDECTOMY SPECIMENS IN GYRATE ATROPHY OF THE RETINA AND CHOROID
- 1 February 1983
- journal article
- research article
- Published by Wiley in Acta Ophthalmologica
- Vol. 61 (1) , 9-19
- https://doi.org/10.1111/j.1755-3768.1983.tb01389.x
Abstract
Gyrate atrophy of the retina and choroid is an autosomal recessive disease characterized by progressive retinal degeneration and ornithine aminotransferase deficiency. We report here the new histological findings and ultrastructural changes in 3 iridectomy specimens from 2 Finnish patients with gyrate atrophy. The iridectomy specimens were removed during routine cataract extraction and studied with a transmission electron microscope. The dilator muscle showed atrophy, abnormall mitochondria, and tubular aggregate type structures similar to those found in skeletal muscle. Degenerative changes such as extracted cellular matrix, dropout of cellular organelles, and dilated intercellular spaces were observed in the pigmented posterior epithelium and the anterior iris epithelium.Keywords
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