Predescemetic deep anterior lamellar keratoplasty as a treatment modality in severe granular corneal dystrophy type 2: a study with a long-term follow-up period

Abstract

This study was designed to assess the long term surgical outcomes of predescemetic deep anterior lamellar keratoplasty (preDALK) in Korean patients with severe granular corneal dystrophy type 2 (GCD2).Retrospective review of the medical charts of patients with GCD2 who had undergone preDALK was performed. DNA analysis was done to confirm the transforming growth factor-β induced gene (TGFBI) R124H mutation. After preDALK was performed, visual acuity, refractive error, keratometry values, complications, and the presence of rejection and recurrence were evaluated.Ten eyes of nine patients were followed up for more than 30 months, and the mean follow-up period was 48.9 ± 14.6 months. Two eyes showed homozygous R124H mutations and the other eyes had heterozygous R124H mutation. The mean best corrected visual acuity after surgery was 0.14 ± 0.11 (logMAR). The two eyes with homozygous mutations showed recurrence.However, all the eyes with heterozygous mutations showed no recurrence during the follow-up period(45.1 ± 13.9 months).The outcome of preDALK which was conducted on the GCD2 patients seems to have at least an equivalent recurrence-free period in comparison to that of penetrating keratoplasty (PKP) and deep anterior lamellar keratoplasty (DALK). Furthermore, preDALK was predicted to provide a longer-term graft survival than that of PKP.Reoperation using thepreDALK procedure may cause fewer complications than the DALK procedure. Therefore, preDALK should be considered as a primary surgical option for GCD2 patients with severe visual impairment who needsto undergo PKP and DALK.