A Clinical Result of PKP performed on the Family with Congenital Hereditary Endothelial Dystrophy

Lee, Jae Hyeun; Chung, Eui Sang; Kim, Woo Jung

Abstract

PURPOSE: Congenital hereditary endothelial dystrophy is a rare disease and is usually characterized by diffuse, bilaterally symmetric corneal opacity and edema inherited by autosomal dominant or recessive pattern. We experienced this rare disease and performed PKP. We report the result with a review of the literature.
METHODS
We performed PKP on 7 eyes with congenital corneal opacity inherited by autosomal dominant type, and histopathologically confirmed. We checked preoperative visual acuity, potential visual acuity, intraocular pressure and corneal thickness. Then postoperatively visual acuity, intraocular pressure, corneal thickness and endothelial cell count was checked.
RESULTS
After the 1 year follow up, all transplanted cornea survived and there were no rejection. Postoperative visual acuities all increased to potential acuity and the postoperative corneal thickness and endothelial cell count all maintained their normal levels. There was no recurrence of the disease at the 1 year follow up, however secondary glaucoma developed in 2 patients(27.5%), and they were treated by glaucoma surgery.
CONCLUSION
The PKP performed on patients of congenital hereditary endothelial dystrophy occurred in one family tree, which has not yet been reported in korea, showed good postoperative results.