To evaluate the visual prognosis in aniridia and the influence of keratoplasty in one large family.Methods.
The medical charts of 30 affected members of this family were studied retrospectively. Their eyes were divided in keratoplasty and nonkeratoplasty groups. In the keratoplasty group, 42 keratoplasties (26 lamellar and 16 penetrating) were performed (20 eyes), with a median follow-up of 18.5 years. In the nonkeratoplasty group, 18 eyes with sufficient follow-up were included. Best corrected visual acuity ever measured (BCVA-ever), BCVA at the last visit (end-BCVA), and the difference between BCVA-ever and end-BCVA (long-term Δ-BCVA) were compared in those two groups. Best postoperative VA (post-BCVA), the difference between pre- and post-BCVA (short-term Δ-BCVA), and its duration and outcomes of the grafts were evaluated in the keratoplasty group. Possible side effects of other procedures and incidents related to aniridia were compared.Results.
The median BCVA-ever was 1/10 in both groups. The median end-BCVA was 2/300 in the keratoplasty group and 1.5/300 in the nonkeratoplasty group; the long-term Δ-BCVA was comparable in the two groups. There was a temporary improvement of BCVA in 36 of 42 eyes after corneal transplantation, with a median of 8 logMAR and duration of 12 months. Most grafts failed because of recurrence of aniridic keratopathy. In the keratoplasty group, there were more corneal infections and more retinal detachments. The occurrence of glaucoma was comparable in both groups. In the nonkeratoplasty group, more eyes (five versus three) lost light perception.Conclusion.
Corneal transplantation in aniridia provided only a temporary improvement in VA in this family. The long-term visual outcome was not affected by keratoplasty.