LAUSR

PURPOSE To report the outcomes of medical and surgical management for congenital aniridia-associated keratopathy (AAK) over a long follow-up period. DESIGN Retrospective, comparative case series METHODS: 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 (appETDRS, 26(20-35) to 35(26-35), Snellen, 20/300 to 20/200,P>0.05). The appETDRS VAE was significantly improved from 20(0-35) to 30(20-55), Snellen 20/400 to 20/250, following LSCT (P=0.021) and from 2(0-20) to 2(0-41) after KPro, same 20/20000 Snellen VAE, but with improved 95% CI after follow-up (P=0.019). CONCLUSIONS With proper characterization and staging of AAK, individualized medical and advanced surgical interventions preserves and improves visual acuity.