Visual and Orthoptic Development After Descemet Stripping Automated Endothelial Keratoplasty for Congenital Hereditary Endothelial Dystrophy in Children Younger Than 8 Years: Case Series and Literature Review.

Purpose: Congenital hereditary endothelial dystrophy (CHED) impairs the sensitive phase of visual development. We examined results of Descemet stripping automated endothelial keratoplasty (DSAEK) for CHED regarding the critical period for amblyogenic factors.

Methods: Retrospective analysis of 11 eyes of 6 consecutive patients with CHED younger than 8 years treated with DSAEK and a PubMed-based literature search on management and optimal timing of the intervention.

Results: Our series encompasses 11 eyes of 6 children who received 6 DSAEKs and 5 non- or partial DSAEKs. Mean age at surgery was 3.45 years (0.59-7.44). In 3 eyes, further interventions were necessary, 2 were Re-DSAEKs for early graft failure. At final visit, all 10 transplants with a follow-up >1 month were clear, and 2 corneas showed slight stromal opacification. Final visual acuity was 0.8-0.2 logMAR (mean 0.49) in 9 eyes with sufficient follow-up time in cooperative patients. Mean follow-up was 47 months. Six eyes showed postoperative hyperopia > 5D. We found literature reports on 58 DSAEK/non-DSAEK procedures in 36 children with CHED under 8 years, including 11 children under 2 years. Data show a trend for better visual outcomes of DSAEK in CHED at younger age. Non-DSAEKs seem to be associated with more graft complications.

Conclusions: DSAEK for CHED in young children is an option for achieving corneal transparency with a trend toward better outcomes of surgery at younger age. The special features of amblyopia in CHED make studies with follow-up times to reach age for reliable acuity testing and recovery from amblyopia worthwhile.