Treatment of Congenital Aniridia Associated with Subluxated Infantile Cataract

Abstract

A 5 year old boy was presented at Eye clinic University clinical center Tuzla with congenital aniridia in both eyes. Clinical examination revealed visual acuity of 0,08 without correction in right and 0.7 with -5.0 Dsph and -1.0 Dcyl Axx 109° in left eye. Opthalmologic examination showed bilateral aniridia associated with moderate cataract in the right and incipient cataract in the left eye. In the right eye, zonular weakness with incipient capsular displacement and esotropia of Δ6º, were noted. The patient underwent phacoemulsification, implantation of capsular tension ring and Artificial Iris implant in the capsular bag. Phacoemulsification went uneventful and early postoperative recovery was successful with no signs of aniridia-associated keratopathy development and normal values of intra ocular pressure. Patient was not motivated for operation of the left eye and it was corrected with soft contact lens. Six month after the operation visual acuity in the right eye improved to 0.9 with +1.25Dsph and maintained stable in left eye, with complete elimination of esotropia and signs of binocular vision restoration. Small incision cataract extraction with IOL and Artificial Iris implantation in one procedure can be used to correct congenital aniridia and cataract with significant visual function improvement.

Novel surgical technique of sutureless artificial iris and intraocular lens scleral fixation using Yamane technique

“… 22 For children, even smaller AI diameters of 8.0mm should be considered as Jusufovic et al report. 23 In another publication Mayer et al give the advice of trimming the AI diameter to WTW minus 0.5mm and point out that a removal of the residual iris in sutureless sulcus implantation of the AI might have a positive effect in the prevention of secondary glaucoma. 11 Gooi et al recommend to assure that there is enough clearance of the outer AI rim to the IOL haptics to avoid iris and ciliary body chafing.…”

Congenital Aniridia with Ectopia Lentis

“…Aniridia is a rare congenital disorder, characterized by profound hypoplasia of the iris, in association with multiple ocular anomalies such as ectopia lentis, corneal defects, glaucoma, strabismus and optic nerve hypoplasia [1][2][3], which can lead to glare, photophobia and other visual disturbances. This condition may be associated with systemic defects consisting of mental retardation, genital anomalies, and Wilms' tumour (WAGR syndrome) [1].…”

Familial Congenital Aniridia with Subluxated Lens and Glaucoma

“…The latest innovation is using an artificial iris implant, which is fully customized with handmade color composition, structurally matches the appearance of a natural iris and restores the aesthetic appearance. [13] Lens dislocation was observed in 42% of patients with aniridia, and it is often displaced superiorly. [10], [14] The risk of lens dislocation may be caused by altered molecular structure in the zonules.…”