Light and electron microscopic study of gelatinous drop-like corneal dystrophy with deeper stromal involvement
Fujita, S.; Sameshima, M.; Hirashima, S.; Nakao, K.
Nippon Ganka Gakkai Zasshi 92(11): 1744-1757
1988
ISSN/ISBN: 0029-0203 PMID: 3265852 Document Number: 314747
A 56-year-old man with gelatinous drop-like corneal dystrophy had severe diffuse corneal opacities with gelatinous white-yellowish granules interspersed by dense white opacities in the subepithelial to deep stroma of both corneas. The corneal buttons obtained by 7.0 mm penetrating keratoplasty were studied histochemically and electron microscopically. The whole epithelium showed irregularity and occasional atrophy, and basal cells in particular exhibited a variety of alterations. The basal lamina and Bowman's membrane disappeared and were replaced by an amyloid substance. Amyloid materials occupied the anterior stroma, and were also deposited in some areas of the deeper stroma. Keratocytes embedded in the amyloid masses showed variable changes. Most of the posterior stromal collagen lamellae, although appearing normal light microscopically, manifested numerous small spherical or elliptical vacuoles containing occasional granular materials of high electron density, a finding similar to the histological description of lattice corneal dystrophy. In such regions, amyloid materials were frequently deposited beside keratocytes showing various alterations; occasional elongated amyloid deposits with cellular debris occurred between the collagen lamellae. Descemet's membrane appeared normal, but some endothelial cells were degenerated. Corneal nerves were not detected throughout the tissue. These results suggest that both corneal epithelial cells and keratocytes were involved in amyloid production with resultant posterior stromal involvement, and that penetrating keratoplasty is recommended for treatment of gelatinous drop-like corneal dystrophy.