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Penetrating keratoplasty (PK) was the procedure of choice in 58.3% (<jats:italic>n<\/jats:italic>\u00a0=\u00a021) of the eyes, followed by a combination of PK and limbal stem cell transplantation in 13.9% (<jats:italic>n<\/jats:italic>\u00a0=\u00a05) and keratolimbal allograft in 13.9% (<jats:italic>n<\/jats:italic>\u00a0=\u00a05). Boston keratoprosthesis was used in 8.3% (<jats:italic>n<\/jats:italic>\u00a0=\u00a03), and anterior lamellar keratoplasty in 5.6% (<jats:italic>n<\/jats:italic>\u00a0=\u00a02). Thirteen of the procedures (36.1%) were retransplantations. Two years after surgery 26 cases were available to follow\u2010up of which 16 of the grafts were functioning (61.5%). The median visual acuity showed a trend of improvement from hand motion to counting fingers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>A majority of the ARK cases (61.5%) had a graft providing useful vision for the patient 2\u2009years after corneal transplantation, but the visual gain was modest at best. Longer follow\u2010up time is required to evaluate functional graft outcomes. Despite the introduction of limbal stem cell transplantation as a suitable treatment, PK was the most common surgical method in the present study.<\/jats:p><\/jats:sec>","DOI":"10.1111\/aos.15229","type":"journal-article","created":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T03:38:51Z","timestamp":1660102731000},"page":"222-228","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Corneal transplantation in aniridia\u2010related keratopathy with a two\u2010year follow\u2010up period, an uncommon disease with precarious course"],"prefix":"10.1111","volume":"101","author":[{"given":"Andreas","family":"Viberg","sequence":"first","affiliation":[{"name":"Department of Clinical Sciences, Ophthalmology Ume\u00e5 University Ume\u00e5 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