Two Cases of Congenital Hereditary Stromal Dystrophy of the Cornea.
- Author:
Mi Ra PARK
1
;
Man Soo KIM
Author Information
1. Department of Ophthalmology, Kangnam St. Mary's Hospital, The Catholic University of Medical College, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Alternating patterns;
Autosomal dominant;
Changes of stromal lamellae;
Congenital hereditary stromal dystrophy
- MeSH:
Cornea*;
Diagnosis;
Endothelial Cells;
Genetic Diseases, Inborn;
Humans;
Keratoplasty, Penetrating;
Microscopy, Electron
- From:Journal of the Korean Ophthalmological Society
1998;39(9):2182-2186
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital hereditary stromal dystrophy of the cornea is a rare hereditary disease which is inherited in autosomal dominant fashion. This disorder is not related to abnormal endothelial cell architecture and function. It presents as bilateral, congenital opacifications of the cornea and has unique histopathologic changes of the stroma such as alternating patterns of densely packed lamellae and loose lamellae. We experienced a family with congental corneal opacifications and performed penetrating keratoplasties in 2 patients. The obtained corneal buttons were examined by light and electron microscopy. The characteristic changes of stromal lamellae were consistent with a diagnosis of congenital hereditary stromal dystrophy of the cornea.
