PENETRATING KERATOPLASTY IN CONGENITAL HEREDITARY ENDOTHELIAL DYSTROPHY

Purpose: The purpose of the study is to determine the outcome of penet rating keratoplasty in congenital hereditary endothelia[ dystrophy. Me thods: Records of 40 patients (13 males, 27 females) who underwent pen etrating keratoplasty (56 eyes) were reviewed. The mean age at surgery was 11.8 years (range, 2 months-35 years). The mean follow-up was 37 months (range, 6-136 months). Results: In 35 (62.5%) of 56 eyes that u nderwent primary penetrating keratoplasty, the grafts survived, Graft survival analysis showed the probability of obtaining a clear graft is 92% at 1 year, 72% at 2 years, and 56.5% at 5 years. Graft survival w as statistically better in eyes where onset of the disease is delayed (P = 0.02), if the graft donor age is between 5 and 30 years versus ol der than 30 years (P = 0.02), and for patients who kept follow-up appo intments Versus those who were delinquent (P < 0.03), Visual acuity wa s 20/40 in 1.9%, 20/50 to 20/80 in 18.9%, 20/100 to 20/300 in 49%, and less than 20/400 in 30.2%. The main causes of graft failure were graf t rejection (six eyes) and bacterial keratitis (four eyes). Conclusion s: Penetrating keratoplasty in congenital hereditary endothelia[ dystr ophy is moderately successful, and graft survival is better in cases o f delayed onset compared with that of congenital onset. Early surgical intervention is recommended to prevent development or progression of amblyopia.