Descemet-Stripping Automated Endothelial Keratoplasty for Congenital Hereditary Endothelial Dystrophy

Objective: To describe the results of Descemet-stripping automated endothelial keratoplasty (DSAEK) for congenital hereditary endothelial dystrophy (CHED). Methods: The medical records of all patients with CHED who underwent DSAEK at our institution were reviewed. A standard DSAEK was performed in all cases with the exception of the Descemet membrane not being removed in patients younger than 12 months. A thorough ophthalmic examination was performed preoperatively and at each postoperative visit in all patients. Results: Fifteen eyes of 8 patients with phakic eyes (4 male and 4 female) were included. The mean age was 9 years (range, 6 months to 30 years). The average follow-up was 15.9 months (range, 3 to 48 months). There were 4 cases of graft detachment, all of which were managed by rebubbling. All corneas were clear within 1 week after surgery. Two of the three infants (6 eyes) could fix and follow preoperatively, while all 3 could do so as early as 1 week following surgery on the second eye. In older patients (9 eyes), preoperative best-corrected visual acuity was 20/200 or less in 6 eyes. Postoperatively, 8 eyes achieved a best-corrected visual acuity of 20/40 or better. Endothelial cell loss (= 7 eyes) averaged 30.0% (range, 8.3% to 43.0%). Conclusions: DSAEK performed in eyes with CHED allows rapid restoration of corneal clarity while minimizing intraoperative and postoperative complications. Our data suggest performing surgery at an earlier age, thus providing opportunity for improved visual development and potentially avoiding amblyopia.