Corneal transplantation may enable a few years of improved visual function in patients severely affected with aniridia-related keratopathy (ARK), but any visual acuity gains for these patients are modest at best, according to research published in Acta Ophthalmologica.
The retrospective registry-study included 36 eyes with ARK from 26 patients who underwent corneal transplantation during 2001 to 2016 in Sweden and Denmark. At the time of transplantation, the median age of the cases included was 42 years and 63.9% were women. All participants had severe vision impairment before surgery.
Penetrating keratoplasty (PK) was the most common surgical method used, chosen in 58.3% (n=21) of the eyes, followed by a combination of PK and limbal stem cell transplantation in 13.9% (n=5), keratolimbal allograft in 13.9% (n=5), boston keratoprosthesis in 8.3% (n=3), and anterior lamellar keratoplasty in 5.6% (n=2). A total of 13 procedures (36.1%) were retransplantations.
At 2 year follow-up exams, grafts were functioning in 61.5% of the 26 eyes (n=16). Recurrence of ARK during 2 years following surgery was noted in 19.2% (n=5) of the 26 eyes, infection in the graft in 19.2% (n=5), rejection of the graft in 11.5% (n=3) and glaucoma was observed in 34.6% (n=9).
“The visual acuity gain was modest at best, but might enable a few years of improved visual function in a severely affected patient group,” according to the researchers. “The decision to perform corneal transplantation in ARK patients is difficult and must be considered individually in each case due to its specific factors, still this study can be helpful in that process, not least by pointing out precautions.”
Study limitations include its registry-based design, small sample size, and relatively short-follow up time.
Viberg A, Vicente A, Samolov B, Hjortdal J, Byström B. Corneal transplantation in aniridia-related keratopathy with a two-year follow-up period, an uncommon disease with precarious course. Acta Ophthalmol. Published online August 9, 2022. doi:10.1111/aos.15229