Abstract
Purpose :
The outcomes of keratoplasty for Aniridia Associated Keratopathy (AAK) are generally poor, although may still be helpful adjunctive for restoring corneal clarity. This study aims to evaluate the characteristics and outcomes of patients undergoing corneal transplantation to treat AAK in the United Kingdom (UK).
Methods :
A retrospective registry study in the UK, including all patients who underwent corneal transplantation to treat AAK between 1 April 2000 and 31 March 2021. Data were collected from UK Transplant Registry by NHS Blood and Transplant at time of transplant, one year and two years post-transplant. Outcomes measured were graft survival, best-corrected visual acuity (BCVA), incidence/type of rejection and cause of failure.
Results :
Sixty-five AAK patients received corneal transplants in the UK within the study period. The majority were female (n=37, 57%), white (n=59, 95%) and aged between 41-60 years (n=32, 49%). Reported risk factors were inflammation, infection, ocular surface disease (n=55, 85%) and glaucoma (n=33, 53%, p=0.61). Preoperatively, most common BCVA was 6/60 or worse (n=61, 97%). Amongst AAK patients with one-year follow-up data (n=46, 69%), 4 (9%) grafts rejected, primarily due to endothelial rejection and 9 (20%) grafts failed at one year which equated to 75% (95% Confidence Interval (CI): 59% - 85%) one-year graft survival using Kaplan Meier methods. At two years, graft survival fell to 62% (95% CI: 45% - 76%, N=36). BCVA was progressively worse as years post transplantation increased, with 19 (46%) and 12 (48%) more AAK cases having CF or worse at one- and two-years post transplantation, respectively.
Conclusions :
AAK is a rare disease in the UK, therefore small number of corneal transplants collected seriously limited accuracy of data analysis to determine benefit of keratoplasty in AAK. There was no overall difference in graft survival when considering donor, recipient and transplant factors. Visual gain post corneal transplantation was modest with 92% of patients had 6/60 vision or worst at 2 years. The decision to perform keratoplasty in AAK patients is difficult and must be individually considered. Longer follow-up and more patients are required to evaluate functional graft outcome.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.