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A corneal button excised from a 2-month-old infant with congenital posterior polymorphous dystrophy of the cornea, a rare disease affecting Descemet's membrane and endothelium, was examined by electron microscopy. We observed irregularly arranged, sometimes multilayered cells with marked epithelial features, lining the posterior surface of the cornea in place of the endothelium, and Descemet's membrane with focal alterations sometimes involving all of its layers. We interpreted these abnormal cells as epithelial-like cells. As these findings were in a very young patient, which is unusual, we concluded that the onset of the disease may take place in the early period of intrauterine life, corresponding to the beginning of Descemet's membrane production.

Four members of a family were affected with lattice dystrophy of the cornea. One of them showed recurrence of the disease in the corneal graft in one eye 9 years after a keratoplasty, and a very advanced stage of the disease in the fellow eye. Therefore a keratoplasty was done in this eye. The corneal button was thoroughly investigated by means of histochemistry, electron microscopy and X-ray electron microanalysis. Two young members of the family showing early signs of the disease were followed up for 2 years. It has been possible to observe the evolution of their lesions. Histopathological examinations revealed the presence of two different types of abnormal deposits under the epithelium and in the stroma. X-ray electron microanalysis of the stromal deposits revealed a substantial increase in sulphur content.