Challenges in pediatric endothelial keratoplasty.

We performed endothelial keratoplasty (EK) in three eyes of two siblings (2.5 years, male and 3.5 years, female) with congenital hereditary endothelial dystrophy (CHED) and report the intraoperative and postoperative difficulties. Repeated iris prolapse, apprehension of crystalline lens touch due to positive vitreous pressure, and need for frequent air injections to attach the graft were intraoperative challenges in all three eyes. These were addressed by use of Sheet’s glide instead of Busin’s glide during graft insertion and suturing of main and side ports before air injection. One eye had graft dislocation on second postoperative day due to eye rubbing by the child. Graft was repositioned with air and a venting incision was created. Postoperative examination required repeated general anesthesia. Corneal edema resolved completely in all three eyes. Present case series highlights the possible intraoperative and postoperative challenges and their solutions in pediatric EK for CHED.

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

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.