Corneal transplantation in children with Peters anomaly and mesenchymal dysgeneses

Objective: The purpose of the study is to describe graft and visual outcomes of penetrating keratoplasty among young children with Peters anomaly and associated mesenchymal dysgeneses. Design: The design was a multicenter retrospective analysis of the indications and outcome in pediatric keratoplasty. Participants: The records of all children aged 12 years and younger who underwent penetrating keratoplasty for mesenchymal dysgenesis between January 1975 and May 1993 at the participating centers were reviewed. Measures: The data were analyzed regarding graft survival and postoperative visual acuity. Results: Forty-seven corneal transplants in 36 eyes of 29 patients with mesenchymal dysgenesis were studied. The majority of eyes operated on (30) had Peters anomaly (83%). Patients' mean age at the time of keratoplasty was 7 months. After a mean follow-up period of 38 months, 61% of eyes retained full graft clarity. One and 3-year survival rates were 79% (95% confidence interval [CI] = 65%-93%) and 62% (95% CI = 45%-79%), respectively. Postoperative corneal ulcers/nonhealing epithelial defects (P = 0.03), and additional noncorneal surgical procedures at the time of transplantation (P = 0.05) were associated with graft failure. Provision of postoperative optical aids (P = 0.01) was associated with better postoperative visual acuity levels. Conclusions: Penetrating keratoplasty for Peters anomaly and related mesenchymal dysgeneses in young children has a reasonable chance of success during the critical years of visual maturation and is associated with satisfactory visual results in one third to half the cases. The data suggest that complicated cases requiring additional surgical procedures have a worse prognosis.