Outcomes of Descemet's membrane endothelial keratoplasty for congenital hereditary endothelial dystrophy.

Purpose: To report outcomes of Descemet's membrane endothelial keratoplasty (DMEK) in eyes with congenital hereditary endothelial dystrophy (CHED).
Methods: The medical records of patients with CHED who underwent DMEK were retrospectively reviewed. Pre- and postoperative visual acuity, corneal thickness, corneal clarity, and graft survival, were analyzed, and intra- and postoperative complications were assessed.
Results: A total of 14 eyes of 8 patients were included. Surgery was performed at a mean age of 10 ± 7 years (range, 2-23). DMEK was successfully performed in all eyes, with 13 of 14 eyes (93%) maintaining a clear cornea at final follow-up (mean, 16.9 ± 8.1 months). Following surgery, corrected distance visual acuity improved from 0.9 ± 0.3 logMAR (Snellen 20/158) to 0.4 ± 0.2 (20/50), and pachymetry improved from 932 ± 57 μm to 642 ± 93 μm. Endothelial cell loss was 33%, and average cell counts were 1767 ± 281 cells/mm ² at final follow-up. Intraoperative aqueous misdirection occurred in both eyes of a 2-year-old child, requiring pars plana vitrectomy. Postoperative donor Descemet's membrane detachment occurred in 4 eyes. Rebubbling was performed in 3 eyes (21%); 1 eye had spontaneous reattachment. One eye (8%) had possible graft failure during follow-up, requiring repeat DMEK surgery.
Conclusions: In our study cohort of eyes with CHED, DMEK was performed with good visual outcome. Rebubbling was necessary for donor reattachment in the early postoperative period in some cases.
(Copyright © 2020 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)