Congenital zonular cataract. Clinicopathologic correlation with electron microscopy and review of the literature

A whole subluxated lens with a congenital zonular cataract was obtained after intracapsular extraction from an 83-year-old patient for histopathologic examination. The first electron microscopic description of a congenital zonular cataract revealed the presence of many globules ranging in diameter from 0.1 to 2.5 microns, occasionally accompanied by enlarged intercellular spaces and a few multilamellar bodies. Most of the globules were arranged concentrically around the nucleus like a shell, and only a few globules were concentrated in nodular aggregates in the nucleus. Ultrastructural indications of the relationship between globules and lens fiber membranes have substantiated the idea of the transformation from normal lens fibers into cataractous fibers by primary breakdown of the lens fiber membranes and secondary degeneration or liquefaction of the fiber contents.