Histopathology of Keratoconus Posticus Circumscriptus

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Abstract
A central corneal button from one eye of a patient with familial bilateral keratoconus posticus circumscriptus is studied histologically. Thinning of Descemet's membrane with small breaks, proliferation of endothelial cells, and formation of hyaline excrescences as well as splitting of Descemet's membrane and a superficial spot of whirl-like scarring in the thinned stroma are demonstrated in the corneal lesion. This seems to be the first report of successful penetrating keratoplasty and histopathological study of the corneal lesion in this rare corneal entity. Keratoconus posticus circumscriptus is characterized by a normal anterior corneal curvature with a loss of substance in a circumscribed area of the posterior central corneal stroma. This causes an increase of the posterior corneal curvature in a circumscribed central area. In a paper by Butler,1 a very good description of the condition is cited from a letter of P. L. Stallard: "It looked as though a marble

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