Granular Corneal Dystrophy

ABSTRACT

Granular corneal dystrophy is characterized by the presence of milk-white spots in the superficial stroma typically lying underneath Bowman's membrane in the axial region of the cornea. This corneal dystrophy was first described by Groenouw in 1890. Since then, not only this dystrophy but also many other types of familial corneal dystrophies have been described. In 1938 Bucklers classified the corneal dystrophy into three types; granular, lattice and macular corneal dystropy. Granular corneal dystrophy was inherited as an autosomal dominant characteristics. It begins in the first decade of life becoming obious at about the age of puberty. The early leasions are small, discrete, grayish-white spots in the superficial stroma of both corneas, and as the condition advances, the lesions are more evident and of various sizes and shapes. This opacities are confined mostly to the axial portion of the cornea. There is no decrease in coreal sensitivity nor any vascularization. Gradually the opacities enlarge, thicken and coalesce into irregular granules hooks, rings and streaks of whitish color and glassy structure. The progress of the disease is usually slow, and moderately good vision often remains in the fourth or fifth decade. The main histopathologic feature is the deposition of a hyalin-like material in the corneal stroma. When the opacification or the irritative epis:xles become disabling, corneal grafting may be indicated. The cases reported here are granu'lar dystrophies which involved four daughters of one family.