The Clinical Outcomes of Keratoplasty in Irreversible Corneal Decompensation Secondary to Axenfeld-Rieger Syndrome
Abstract Porpose To evaluate the clinical outcomes of penetrating keratoplasty (PK) and Descemet’s stripping automated endothelial keratoplasty (DSAEK) in eyes with irreversible corneal decompensation secondary to Axenfeld-Rieger syndrome (ARS).Methods In this retrospective case series, a total of 4 eyes undergoing PK and 7 eyes undergoing DSAEK, including 1 eye requiring 1 repeat DEASK, between 2014 and 2021 were enrolled. Postoperative complications, graft survival, glaucoma treatment before and after keratoplasty, visual outcomes, and endothelial cell density were recorded.Results The mean follow-up duration was 33.4 ± 16.8 months. Before keratoplasty, the mean BCVA was 2.0 ± 0.4 LogMAR, and the mean IOP was 21.6 ± 8.1 mmHg. 63.6% of eyes (7/11) received glaucoma treatment, including 5 eyes with glaucoma surgeries. After keratoplasty, 27.3% of eyes (3/11) exhibited secondary graft failure. The mean BCVA reached a maximum of 0.7 ± 0.5 LogMAR at 8.9 ± 7.5 months, with no significant difference between the PK and DSAEK groups (P1=1.00, P2=0.12). Four eyes with previous glaucoma surgeries exhibited markedly high IOP. 72.7% of eyes (8/11) required additional glaucoma treatments. The mean endothelial cell loss (ECL) rates at 1, 6, 12 and 24 months were 43%, 49%, 63% and 54%, respectively, with no significant difference between the PK and DSAEK groups (P1=0.64, P2=1.00, P3=0.57, and P4=0.44).Conclusion Both PK and DSAEK can successfully treat corneal decompensation secondary to ARS, resulting in similar outcomes with regard to IOP control, BCVA and ECL. IOP control is essential for postoperative management, especially for eyes with previous glaucoma surgeries.