Purpose-To report the outcomes of medical and surgical management for congenital aniridiaassociated keratopathy (AAK) over a long follow-up period. Design-Retrospective, comparative case seriesMethods-Medical records of patients diagnosed with congenital aniridia were retrospectively reviewed. Age, gender, ethnicity, follow-up time, AAK stage, non-corneal abnormalities, ocular surgeries, and complications were recorded. The visual acuity equivalent (VAE), approximate Early Treatment Diabetic Retinopathy Study (appETDRS) letter score, was calculated using recorded Snellen visual acuities.Results-Ninety-two eyes of 47 patients (31 females) with mean age of 48.0±18.0 years, and mean follow-up of 78.6±42.2 months were included. At the initial visit, 12 eyes (13%) were classified as stage I AAK, 33 eyes (35.9%) stage II, 25 eyes (27.2%) stage III, 17 eyes (18.5%) stage IV and 5 eyes (5.4%) stage V. Limbal stem cell transplantation (LSCT) and Boston Keratoprosthesis (KPro) were frequently performed on eyes with stages III-V. These advanced corneal surgeries significantly improved the median (95% CI) of calculated appETDRS scores from 2(0-20) to 26(15-41) (Snellen, 20/20000 to 20/300, P=0.0004). Patients with earlier stages (I-II) of AAK were managed medically and had stable visual acuity through their final visits *

Despite improved retention and reduced complication rates paving the way for the current expansion of applications and surge in prevalence for the Boston type I Keratoprosthesis (KPro), the most frequent indication for its implantation today remains prior graft failure. The purpose of this study is to evaluate the long-term outcomes of primary KPro and compare to secondary implantation in a matched cohort study. This study included patients who underwent KPro implantation in a single center by two surgeons between July 2008 and October 2014. All eyes with KPro implantation as the primary procedure with a minimum follow up of 12 months were matched with eyes with same preoperative diagnoses that underwent secondary KPro implantation. Main outcomes included visual acuity and device retention. A total of 56 eyes were included with 28 eyes in each group. Mean follow up was 5.0 years for both groups. Twenty-nine percent (8) of the eyes in the primary group had a diagnosis of chemical or thermal injuries, 25% (7) aniridia, 18% (5) autoimmune disease, 4% (1) infectious keratitis/neurotrophic cornea, 7% (2) gelatinous corneal dystrophy, 7% (2) ectrodactyly ectodermal dysplasia/limbal stem cell deficiency, and 11% (3) uveitis/hypotony. Sixty-one percent (17) of the eyes in the primary group and 39% (11) in the secondary group maintained a final best-corrected visual acuity of 20/200 or better at a mean follow up of 5.0 years; the probability of maintaining best-corrected vision is 0.83 and 0.49 for primary and secondary groups at 5.0 years (p = 0.02). There is no statistically significant difference between groups in device retention (p = 0.22) or postoperative complication rates (p >0.05). This study demonstrates that Boston KPro implantation may be successful as a primary procedure in patients at high risk of failure with traditional penetrating keratoplasty. The device has a good long-term retention rate and visual outcomes are promising however a larger study is needed for more definitive results.

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