Descemet Stripping Endothelial Keratoplasty in Pediatric Patients with Congenital Hereditary Endothelial Dystrophy

Area of Science:

• Ophthalmology
• Regenerative Medicine
• Pediatric Surgery

Background:

• Congenital hereditary endothelial dystrophy (CHED) is a rare genetic disorder affecting corneal clarity.
• Endothelial keratoplasty, specifically Descemet stripping endothelial keratoplasty (DSEK), is a potential treatment for CHED.
• Evaluating long-term outcomes in pediatric CHED patients is crucial for optimizing surgical strategies.

Purpose of the Study:

• To assess the long-term results of DSEK with suture-assisted donor lenticule insertion in pediatric CHED patients.
• To compare outcomes between infant and child age groups undergoing DSEK for CHED.
• To identify factors influencing visual acuity and complications after DSEK in this population.

Main Methods:

• A retrospective case series design was employed.
• Pediatric patients diagnosed with CHED who underwent DSEK between 2010 and 2016 were included.
• Patients were stratified into infant and child groups for comparative analysis of clinical outcomes and complications.

Main Results:

• Thirty eyes from 16 pediatric patients were analyzed, with follow-up averaging over 4 years.
• Infant patients achieved significantly better postoperative best-corrected visual acuity (BCVA) compared to the child group (logMAR 0.32 vs. 0.54, P=.01).
• While corneal transparency was similar, higher rates of achieving target BCVA were observed in infants (86% vs. 33%). Lenticule detachment occurred in 3 cases within the child group.

Conclusions:

• DSEK with suture-assisted lenticule insertion can lead to improved visual outcomes in infant patients with CHED.
• The technique, including Descemet membrane stripping and careful postoperative management, is considered safe and effective for pediatric CHED.
• Age at the time of surgery appears to be a significant factor in achieving optimal visual results.