[Keratoglobus]

Abstract

Keratoglobus is a bilateral corneal disease characterised by thinning and protrusion of the entire corneal surface. The cornea is of normal size and usually transparent. Acute corneal edema due to rupture of Descemet's membrane and perforation even from minimal trauma are the most frequent complications. Keratoglobus may be associated with blue sclera, hyperextensibility of the joints or auditory problems. Most often it is a congenital disease with autosomal recessive transmission, but it may be secondarily acquired to an advanced keratopathy (keratoconus), to trauma or to exophthalmos. We report 5 cases of keratoglobus. 4 patients underwent penetrating keratoplasty. The anatomical abnormalities of congenital keratoglobus (3 cases) are constants : an absent Bowman's membrane, a disorganized stroma containing granular material, a thickened, striated Descemet's membrane with breaks, folds and verrucosities. On the other hand, acquired keratoglobus (1 case) shows localised breaks in Bowman's membrane resembling those of keratoconus. The anatomopathological differences are discussed.