J Korean Ophthalmol Soc.
2000 Jan;41(1):282-287.
Three Cases of Congenital Aniridia in One Family
- Affiliations
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- 1Department of Ophthalmology, Taegu Fatima Hospital.
Abstract
- Aniridia is a relatively rare congenital anomaly and its incidence is about 1:100, 000.Main features of aniridia include congenitally partial or nearly complete absence of the iris and hypoplasia of optic disc and fovea. It is almost bilateral and occurred as an autosomal dominant trait.However, some patients develop both sporadic nonfamilial aniridia and Wilms 'tumor, and another group of patients is transmitted as an autosomal recessive trait. We observed aniridia in two generations, as mother and two daughters. They had cataract, nystagmus, neovascularization of corneal margin and pannus formation, as well as aniridia.We also found hypoplasia of optic disc and fovea in mother. We have operated on mother with phacoemulsified lens aspiration and posterior chamber IOL implantation in both eyes consecutively.The postoperative visual acuity following lens extraction improved, but was not corrected. In addition, intraocular pressure decreased.
