Outcomes of Boston keratoprosthesis in aniridia: a retrospective multicenter study.

PURPOSE: To evaluate the long-term outcomes of keratoprosthesis as an alternative surgical procedure in the management of aniridic keratopathy.

DESIGN: Retrospective, multicenter case series.

METHODS: Fifteen adult patients (16 eyes) with aniridia who were deemed at high risk for regular donor corneal transplantation underwent a Boston type I keratoprosthesis procedure for visual rehabilitation. Device retention rate, preoperative and postoperative visual acuity, and intraoperative and postoperative complications were studied.

RESULTS: The mean age of the patients was 45 (median 50) years. Six of the patients were females. Eleven patients had previously undergone donor corneal transplantations (average two grafts), with failure. In 10 patients, the keratoprosthesis surgery was combined with other procedures such as cataract extraction, tube shunt implantation, vitrectomy, and intraocular lens removal. No intraoperative complications were encountered. The follow-up ranged from two months to 85 months, with a median of 17 months. All devices remained in situ throughout the entire follow-up period. The visual acuity improved in all but one patient from a median of counting fingers (light perception to 20/300) to 20/200 (hand motions to 20/60). Comorbid preoperative conditions particularly optic nerve and foveal hypoplasia limited the final postoperative vision. No endophthalmitis or extrusion of the device occurred. One device required repair procedure without necessitating a removal.

CONCLUSIONS: Keratoprosthesis offers significant vision benefits in this patient group.