Long-term results of corneal graft survival in infants and children with peters anomaly.

OBJECTIVE: To determine the long-term results of corneal graft survival after penetrating keratoplasty for Peters anomaly and to identify risk factors for graft failure. DESIGN: Noncontrolled interventional case series: a single-center retrospective review of a consecutive surgical series. PARTICIPANTS: The records of all children 12 years of age or younger who underwent penetrating keratoplasty for Peters anomaly between January 1971 and December 1992 were reviewed. All study eyes had completed a minimum of 3 years of follow-up from the date of first keratoplasty and had undergone most of their corneal surgery at Emory University. INTERVENTION: Characteristics of the recipient, the eye, the donor, and the surgical procedure were analyzed for their influence on survival of the first graft. Survival probabilities were estimated using the Kaplan-Meier method. Multivariate regression analysis was performed to estimate relative risks and adjusted survival probabilities. MAIN OUTCOME MEASURE: Graft clarity. RESULTS: One hundred forty-four penetrating keratoplasties were performed in 72 eyes of 47 patients. The median age at first keratoplasty was 4.4 months. The median follow-up was 11.1 years. Fifty-four percent of eyes received one graft, 18% received two grafts, and 28% received three or more grafts. The overall probability of maintaining a clear first graft was 56% at 6 months, 49% at 12 months, 44% at 3 years, and 35% at 10 years. The probability of second or subsequent grafts surviving for 3 years was less than 10%. Thirty-nine percent of eyes had a clear graft at the time of review; 36% of eyes had a clear first graft. Multivariate analysis identified disease severity, donor cornea size, coexisting central nervous system abnormalities, and quadrants of anterior synechiae as the strongest risk factors for graft failure. Supplemental multivariate analysis, restricted to observable preoperative variables, identified stromal vessels, total limbal opacification, and preoperative glaucoma as independent preoperative predictors of graft failure. Allograft rejection was the most frequently identified cause of graft failure. Major complications after keratoplasty were phthisis, retinal detachment, cataract, and glaucoma. CONCLUSIONS: The overall long-term probability of maintaining a clear graft after initial penetrating keratoplasty for Peters anomaly is 35% +/- 0.06%, with subsequent grafts rarely surviving. Eyes with severe disease, larger donor corneas, coexisting central nervous system abnormalities, and anterior synechiae have significantly poorer outcomes than eyes without these factors. These data should be carefully considered before recommending corneal transplantation for Peters anomaly, particularly after previous graft failure.