Corneal tranplantation in young children with congenital hereditary endothelial dytrophy Original Reearch Article

Purpoe: To decribe the urgical ucce rate and viual reult of penetrating keratoplaty in a erie of young children with congenital hereditary endothelial dytrophy and to ummarize the current literature on outcome of keratoplaty for congenital hereditary endothelial dytrophy, with particular attention to the timing of urgery. Method: The author conducted a retropective tudy of children aged 12 year and younger who underwent penetrating keratoplaty between 1975 and 1994 at four participating eye center, and who were followed for at leat 6 month potoperatively. For thi report, 21 corneal tranplant performed in 16 eye of nine patient with congenital hereditary endothelial dytrophy were tudied. Patient’ median age at the time of firt keratoplaty wa 40 month (range, 3 month to 10 year). Reult: During a mean follow-up period of over 70 month (range, 6 to 240 month), 11 (69%) of 16 eye retained full graft clarity. The 2-year urvival rate of firt graft wa 71% (95% confidence interval, 47% to 95%). Potoperative viual acuity improvement of 1 or more nellen line wa een in five of 10 eye in which the patient were old enough for accurate aement of viual acuity; however, jut four of thee 10 eye attained a viual acuity of 20/200 or better. Concluion: Penetrating keratoplaty for congenital hereditary endothelial dytrophy in children ha a reaonable chance of urgical ucce when performed at a young age; however, the prognoi for improved viual acuity in children appear to be more guarded. Deciion on the timing of urgical intervention for congenital hereditary endothelial dytrophy hould be made on a cae-by-cae bai. Although the threat of irreverible amblyopia in untreated eye and good urgical ucce rate even among very young children argue for the conideration of relatively early urgical intervention in the mot everely affected cae, there i evidence to upport delaying urgery in ome cae.