Congenital Aniridia in a Family for Three Generations.
- Author:
Jeong Im LEE
1
;
Yong Hwa KIM
;
Chung Sook AHN
Author Information
1. Department of Ophthalmology, College of Medicine, Ewha Womans University, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Anirida;
Congenital;
Hereditary
- MeSH:
Aniridia*;
Capsule Opacification;
Family Characteristics*;
Glaucoma;
Humans;
Iris;
Optic Nerve;
Penetrance;
Phenotype;
Strabismus
- From:Journal of the Korean Ophthalmological Society
1990;31(3):383-389
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Aniridia is a congenital, often hereditary, usually bilateral absence of iris in whole or in part. And aniridia occurs mainly as an autosomal dominant condition with almost complete penetrance. In the usual phenotype, aniridia is associated with nystagmus, foveal and optic nerve hypoplasia, corneal pannus, cataract, secondary glaucoma and strabismus. The author experienced 4 cases of aniridia in a family for three generations. So the author reports these cases with the review of literathure.
