Corneal transplantation in young children with congenital hereditary endothelial dystrophy

Abstract

Abstract Purpose: To describe the surgical success rate and visual results of penetrating keratoplasty in a series of young children with congenital hereditary endothelial dystrophy and to summarize the current literature on outcomes of keratoplasty for congenital hereditary endothelial dystrophy, with particular attention to the timing of surgery. Methods: The authors conducted a retrospective study of children aged 12 years and younger who underwent penetrating keratoplasty between 1975 and 1994 at four participating eye centers, and who were followed for at least 6 months postoperatively. For this report, 21 corneal transplants performed in 16 eyes of nine patients with congenital hereditary endothelial dystrophy were studied. Patients’ median age at the time of first keratoplasty was 40 months (range, 3 months to 10 years). Results: During a mean follow-up period of over 70 months (range, 6 to 240 months), 11 (69%) of 16 eyes retained full graft clarity. The 2-year survival rate of first grafts was 71% (95% confidence interval, 47% to 95%). Postoperative visual acuity improvement of 1 or more Snellen lines was seen in five of 10 eyes in which the patients were old enough for accurate assessment of visual acuity; however, just four of these 10 eyes attained a visual acuity of 20/200 or better. Conclusions: Penetrating keratoplasty for congenital hereditary endothelial dystrophy in children has a reasonable chance of surgical success when performed at a young age; however, the prognosis for improved visual acuity in children appears to be more guarded. Decisions on the timing of surgical intervention for congenital hereditary endothelial dystrophy should be made on a case-by-case basis. Although the threat of irreversible amblyopia in untreated eyes and good surgical success rates even among very young children argue for the consideration of relatively early surgical intervention in the most severely affected cases, there is evidence to support delaying surgery in some cases.