Three Cases of Congenital Aniridia in One Family.

Bae, Jae Hak; Cho, Young Wook; Kwak, Mi Sun

Case Report

Journal of the Korean Ophthalmological Society 2000;41(1):282-287.
Published online January 1, 2000.

Three Cases of Congenital Aniridia in One Family.

Jae Hak Bae, Young Wook Cho, Mi Sun Kwak

Department of Ophthalmology, Taegu Fatima Hospital.

한 가족 3 명에서 발생한 선천성 무홍채증

배재학(Jae Hak Bae),곽미선(Mi Sun Kwak),조영욱(Young Wook Cho)

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.

Key Words: Aniridia;Cataract;Posterior chamber IOL