We thank Prof. Soleimani and colleagues for their correspondence and insightful comments on our publication, “Outcomes of Penetrating Keratoplasty and Boston type 1 Keratoprosthesis in Aniridia-Associated Keratopathy: A Systematic Review and Meta-Analysis”. In their letter, they raised important considerations regarding the reported anatomical superiority of keratoprosthesis (KPro) implantation compared with penetrating keratoplasty (PK) in patients with aniridia-associated keratopathy (AAK). ,
Firstly, we agree that limbal stem cell transplantation (LSCT) is an important option in AAK management prior to corneal transplantation and requires prolonged systemic immunosuppression; however, low success rates in congenital aniridia question its long-term efficacy and favor careful patient selection over routine use. Future therapies for AAK may involve restoring or enhancing PAX6 activity, miRNA modulation, locally delivered biologics targeting inflammatory mediators such as IL-17 or TNF, normalization of apoptotic signaling, and personalized reprogramming of aniridic limbal stem cells to re-establish function and prevent recurrence.
AAK often affects young patients. Based on current literature, KPro appears superior to PK in terms of anatomical success; however, we disagree with several interpretations presented by Soleimani et al. Importantly, anatomical success does not equate to functional success, and as we reported, postoperative visual improvement was comparable between KPro and PK. At no point did we advocate routine primary KPro implantation in AAK. Furthermore, in our meta-analysis, mean follow-up for KPro ranged from 9 to 70.7 months, and our conclusions were confined to this short- to mid-term period. Longer-term data suggest a different perspective: Bernstein et al. reported progressive visual decline over time, with the proportion of patients achieving BSCVA > 20/200 decreasing from 49% at 5 years to 37% at 10 years and 30% at 15 years after KPro implantation. Although overall complication rates were similar for PK and KPro, KPro carries a higher risk of severe anatomical failure and irreversible vision loss—an especially important concern in younger patients, in whom long-term safety appears less favorable. Younger age at implantation is linked to more frequent serious, vision-threatening complications and a greater likelihood of KPro exchange, whereas PK can be repeated multiple times while preserving globe integrity.
Glaucoma is a recognized complication after both PK and KPro, likely related to anterior synechiae and prolonged corticosteroid therapy. Although the difference was not statistically significant, glaucoma developed or progressed in 17.5% of eyes after KPro vs 4.0% after PK during mid-term follow-up. Notably, no cases of no light perception (NLP) occurred after PK, whereas 9% of KPro eyes progressed to NLP. Long-term data are more concerning: in a large cohort, new-onset glaucoma reached 63.6% at 15 years after KPro, and eyes with pre-existing glaucoma showed greater progression than those with newly developed disease.
In conclusion, in patients with AAK, KPro demonstrates superior anatomical success compared with graft survival after PK. Postoperative visual acuity gains and overall complication rates are similar between the procedures. However, long-term safety—particularly in younger individuals and those with pre-existing glaucoma—remains a concern. Further long-term comparative studies are needed to clarify its role, especially in combination with LSCT.
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