Aniridia

Overview

Aniridia is a congenital developmental anomaly of the eye that usually affectsboth eyes.The development of the iris,cornea,lens,angle,optic nerve and retinais disturbed.Aniridia is most often a hereditary disease with an autosomal domi-nant,rarely autosomal recessive inheritance,but sporadic cases are also possible.The vision function in aniridia has been observed to have a wide range fromblindness toa normal visual acuity.Themore serious cases where blindness occurshas been due not specifically to the aniridia but to associated conditions likecataract,glaucoma,foveal hypoplasia,corneal dystrophy,nystagmus.Aniridiacould be associated with the mental retardation.Some of the sporadic casesdevelop Wilms ’tumor,frequently as part of the WAGR syndrome (Wilms ’tumor,aniridia,genitourinary abnormalities and mental retardation)

Key words:
aniridia,WAGR syndrome,secondary glaucoma,Wilms ’tumor