Penetrating Keratoplasty in Congenital Hereditary Endothelial Dystrophy

The purpose of the study is to determine the outcome of penetrating keratoplasty in congenital hereditary endothelial dystrophy.Records of 40 patients (13 males, 27 females) who underwent penetrating 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).In 35 (62.5%) of 56 eyes that underwent 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 was 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 older than 30 years (P = 0.02), and for patients who kept follow-up appointments versus those who were delinquent (P0.03). Visual acuity was 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 graft rejection (six eyes) and bacterial keratitis (four eyes).Penetrating keratoplasty in congenital hereditary endothelial dystrophy is moderately successful, and graft survival is better in cases of delayed onset compared with that of congenital onset. Early surgical intervention is recommended to prevent development or progression of amblyopia.