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PURPOSE To report our early experiences with Descemet membrane endothelial keratoplasty (DMEK) in congenital hereditary endothelial dystrophy (CHED). METHODS Retrospective analysis of medical records of eyes with CHED that underwent DMEK between January 1, 2018, and April 30, 2019, and had a minimum of 1-year follow-up. RESULTS Three eyes of 2 CHED patients (a 10-year-old girl and a 22-year-old man) were included. Both had decreased vision and hazy corneas from birth and underwent DMEK during the study period. Surgery was performed by a single surgeon and was uneventful in all 3 eyes. Within 1 month of surgery, the corneal edema cleared, and vision improved significantly. The mean decimal visual acuity improved from 0.15 ± 0.08 (baseline) to 0.33 ± 0.19 at 3 months. The mean central corneal thickness improved from 928 ± 2.3 μm (baseline) to 555.3 ± 25.8 μm at 3 months. The final visual outcome was less in 1 eye because of dense amblyopia. In all 3 eyes, a subtle stromal haze persisted even after resolution of edema. One eye had graft rejection 7 months after surgery because of discontinuation of medications, effectively managed by increasing the frequency of topical steroids. CONCLUSIONS DMEK may be a viable option in phakic eyes with CHED.