Outcomes of Boston keratoprosthesis in aniridia: a retrospective multicenter study.
OBJECTIVE
To evaluate the long-term outcomes of keratoprosthesis as an alternative surgical procedure in the management of aniridic keratopathy.


METHODS
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.
Akpek
Esen Karamursel
EK
Wilmer Eye Institute, The Johns Hopkins School of Medicine, Baltimore, MD 21287, USA. esakpek@jhmi.edu
Harissi-Dagher
Mona
M
Petrarca
Robert
R
Butrus
Salim I
SI
Pineda
Roberto
R
Aquavella
James V
JV
Dohlman
Claes H
CH
D000328:Adult; D000368:Aged; D015783:Aniridia / Q000601:surgery*; D003315:Cornea; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D019736:Prostheses and Implants; D011474:Prosthesis Design; D019919:Prosthesis Implantation / Q000295:instrumentation*; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014792:Visual Acuity