Related Experiment Video
Updated: Sep 23, 2026
Corneal Donor Tissue Preparation for Descemet's Membrane Endothelial Keratoplasty
Published on: September 17, 2014
Penetrating keratoplasty in young children with congenital hereditary endothelial dystrophy
Mohammad Ali Javadi1, Ali Reza Baradaran-Rafii, Mitra Zamani
1Ophthalmic Research Center, Labbafinejad Medical Center, Shaheed Beheshti University of Medical Sciences, Tehran, Iran. ma_javadi@yahoo.com
Insights
Penetrating keratoplasty (PKP) improved vision in 94.7% of children with congenital hereditary endothelial dystrophy (CHED). Graft survival was 88% at 3 years, but rejection was common, necessitating careful consideration for pediatric corneal transplants.
Area of Science:
- Ophthalmology
- Corneal Surgery
- Pediatric Ophthalmology
Background:
- Congenital hereditary endothelial dystrophy (CHED) is a rare genetic disorder affecting corneal clarity in infants and children.
- Early diagnosis and intervention are crucial for visual development, but pediatric corneal transplantation presents unique challenges.
Purpose of the Study:
- To evaluate the visual outcomes and graft survival rates of penetrating keratoplasty (PKP) in young children diagnosed with CHED.
- To assess the relationship between age at surgery and final visual results in this pediatric cohort.
Main Methods:
- A retrospective analysis of 24 eyes from 15 pediatric patients (<12 years) who underwent PKP for CHED.
- Data collected included patient demographics, surgical indications, visual acuity (Snellen chart), graft clarity, and complication rates (e.g., allograft rejection).
- Statistical analysis included McNemar's test, Kaplan-Meier survival analysis, and Mann-Whitney U test.
Main Results:
- Postoperative visual acuity improved in 94.7% of eyes, though 47.4% remained below 20/80.
- Graft clarity was achieved in 79.1% of grafts at the last examination.
- Allograft rejection occurred in 43.4% of eyes, with endothelial rejection being the primary cause of graft failure. Graft survival was 88% at 3 years and 74% at 5 years.
Conclusions:
- PKP can significantly improve vision in children with CHED, but challenges like allograft rejection persist.
- No significant relationship was found between age at PKP and final visual outcome.
- A conservative approach with careful risk-benefit assessment is recommended for pediatric CHED patients undergoing keratoplasty.
Purpose:
To report the visual results and success rate of penetrating keratoplasty (PKP) in a series of young children with congenital hereditary endothelial dystrophy (CHED).
Methods:
This is a retrospective study on twenty-four eyes of 15 patients (seven male and eight female) operated on for CHED. Children less than 12 years of age at the time of surgery who were followed for at least 6 months were recalled. Characteristics of the patients, indications for PKP, final visual outcome, and graft clarity were evaluated. The following tests were employed: McNemmar test for evaluating visual results, Kaplan-Meyer analysis for determination of graft survival, and Mann-Whitney U test for evaluating the relationship between visual outcome and age at PKP.
Results:
Patients' age at diagnosis and at initial PKP was 6.5 +/- 3.6 and 8.1 +/- 2.5 years, respectively. Follow-up period was 35.5 +/- 36.2 months. Visual acuity could be evaluated by Snellen chart in 19 eyes. Preoperative visual acuity was less than 20/80 in all of these. Postoperatively, visual acuity was less than 20/80 in nine eyes (47.4%) (P < 0.002). Visual acuity improved in 18 (94.7%) of 19 eyes. There was no relationship between age at initial PKP and final visual outcome (P = 0.35). At the last examination (24 grafts), 19 were clear (79.1%), two were hazy (8.3%), and three were opaque (12.5%). Allograft rejection was seen in 10 eyes (43.4%), seven of which were endothelial. Excluding one case of trauma, all graft failures resulted from endothelial rejection. The probability of primary graft survival was 88% at 3 years and 74% at 5 years.
Conclusion:
Regarding the difficulties in pediatric keratoplasty and the absence of a relationship between postoperative visual outcome and age at keratoplasty, a conservative approach and careful risk-benefit ratio evaluation are recommended in patients with CHED.
