Sorsby's fundus dystrophy: a light and electron microscopic study

The light and electron microscopic changes in both eyes of a patient with Sorsby's fundus dystrophy are reported. The most striking finding was a 30-microns thick deposit present within Bruch's membrane that stained positive for lipids. The deposit has several unique features when compared with those found in other retinal dystrophies and in aged eyes. In addition, there was gross loss of the outer retina, the retinal pigment epithelium was discontinuous, and there was atrophy of the choriocapillaris. These findings are discussed in relation to the published clinical observations of other members of this pedigree.