Outcomes of Descemet-Stripping Automated Endothelial Keratoplasty in Congenital Hereditary Endothelial Dystrophy.

Abstract

To evaluate the outcomes and complications of Descemet-stripping automated endothelial keratoplasty (DSAEK) in children with congenital hereditary endothelial dystrophy (CHED). Eighteen eyes from 10 children with CHED who underwent standard phakic DSAEK were enrolled in this retrospective interventional case series. Medical records including slit-lamp biomicroscopy, cycloplegic refraction, visual acuity, and measurements of central corneal thickness, corneal topography, and endothelial cell density were evaluated. All complications were recorded. The mean age of cases was 8.1±4.2 (range: 3-16) years at the time of surgery. Descemet-stripping automated endothelial keratoplasty was performed successfully in all eyes, which survived in 16/18 (88.9%) of cases during a median follow-up period of 38 (range 19-64) months. In patients with clear graft, best-corrected visual acuity was improved to a range of 20/100 to 20/40 and improved to fix and follow in one younger child. Endothelial cell density decreased by an average of 42.1%, comparing baseline and 6-month follow-up and was relatively stable thereafter. A marked flattening of the anterior corneal curvature (mean change of anterior K reading: +2.56±3.14 diopters) was observed after DSAEK. The only major complication in patients with clear graft was intraocular pressure elevation in 61.1% of the eyes, which was easily controlled with one antiglaucoma medication. None of the eyes developed cataract. Descemet-stripping automated endothelial keratoplasty is a safe and successful procedure in children with an acceptable graft survival in a mid-term follow-up period. A marked hyperopic shift could occur after DSAEK in children with CHED.