Similarities between Posterior Polymorphous and Congenital Hereditary Endothelial Dystrophies

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Abstract
Histopathologic study of 14 keratoplasty specimens in 11 cases of posterior corneal dystrophy was performed by light and electron microscopy. Prior clinical diagnosis was congenital endothelial dystrophy (CED) in nine specimens, posterior polymorphous dystrophy (PPD) in three specimens, and posterior corneal dystrophy (PCD) in two specimens. Retrocorneal fibrous tissue proliferation and multilaminated basement membrane were observed in 13 of the 14 specimens. Two or more types of cells lined the posterior surface of the cornea in 12 of the 14 specimens. These cells included: normal-appearing endothelial cells, embryonic-like endothelial cells, degenerated endothelial cells, fibrocyte-like cells, epithelial-like cells, and melanocyte-like cells. Total absence of a cellular layer was observed in one specimen. Because some of the ultrastructural features of CED and PPD are the same, it is possible that these two entities represent a spectrum of the same process.

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