Multirecurrence of Corneal Posterior Polymorphous Dystrophy

Abstract

Posterior polymorphous corneal dystrophy (PPD) is a rare bilateral, autosomal-dominant disease. The presence of the epitheliumlike endothelium and the thickening of Descemet's membrane by the posterior collagenous layer (PCL) has been reported in the majority of published cases. Reepithelialization of the posterior cornea in donor tissue has been reported only once. Therefore, to examine this process we examined, by light and electron microscopy, three corneal buttons (an original and two subsequent keratoplasties after 3 and 4 years, respectively) from the left eye of a patient with bilateral disease. Our study showed a repopulation of the posterior surface of the donor corneas by the host epitheliumlike endothelium, which was of identical morphology in each case. In contrast to the previously published work, a posterior collagenous layer in the failed grafts was absent from the axial cornea, where the epitheliumlike endothelium was in direct contact with donor Descemet's membrane. A PCL at the periphery in the failed transplants was of the fibrocellular type and differed from the fibrillar PCL in the first keratoplasty specimen. The fibroblastlike cells within the fibrocellular PCL were distinct from the underlying epithelial-like cells and contained numerous tertiary phagolysosomes. We suggest that the major contributor to the fibrocellular PCL in the repeat keratoplasties was a fibrous ingrowth from the host/graft junction and not the epitheliumlike endothelium. It appears that the synthetic capacity of the epitheliumlike endothelium was similar to that of the normal epithelium and was much lower than that of normal native endothelium.