Clinical and Surgical Management of Cataract in Congenital Aniridia

Aniridia consists in a complex malformation of the eye with congenital absence of iris. This genetic rare disease can cause severe visual impairment occurring from various mechanisms. Ocular clinical signs in aniridia may associate glaucoma, most common complication with limbal insufficiency leading to keratopathy, cataract, ptosis, foveal aplasia or a microphthalmia. The cataract in aniridia must be identified with its specificities in order to adjust the treatment according with other ocular signs and complications of the disease. In aniridia, cataract is usually reduced in infancy to mild opacities or partial lens opacification. The main treatment aims to correct ametropia, potentially induced strabismus, nystagmus and amblyopia in case of anisometropia or cataract asymmetry. When visual acuity becomes low, time of cataract surgery must be discussed. The assessment of the low vision due to an occlusive cataract must be confirmed. Other factors as foveal aplasia, corneal opacities, glaucoma may influence visual acuity and will not be corrected by cataract surgery. Different techniques of cataract surgeries are available and adapted for cataract in aniridia however the surgeon must be aware of high rate of complications as glaucoma, fibrosis and ocular surface impairment. Phacoemulsification and classical intraocular lens, artificial iris or combinated intraocular lens with diaphragm can be performed very carefully. A regular follow-up of the patient must be performed in order to detect complications.