Abstract

A 4 1/2-year-old boy with congenital hereditary endothelial dystrophy underwent successful bilateral penetrating keratoplasties. Visual acuity in both eyes was restored to 6/18 (20/60). Correlative microscopic analysis of the corneal button obtained from the first transplant showed an irregular epithelium, a continuously thickened Bowman's layer, fragmentation and homogenization of the anterior stromal lamellae, a mildly enlarged stromal fibril diameter, abnormal collagen layers posterior to Descemet's membrane, atrophic paracentral corneal endothelium, and absent central endothelium. The endothelial cells from the second corneal button were placed in tissue culture and failed to demonstrate any growth characteristics of differentiation into normal appearing cells after 14 days. We concluded that the endothelial cells in congenital hereditary endothelial dystrophy are functionally and morphologically abnormal. Early penetrating keratoplasty can be successful in such cases.