Histopathological study of microspherophakia in the Weill-Marchesani syndrome

A surgically obtained lens from a 66-year-old man with the Weill-Marchesani syndrome was examined histopathologically by light microscopy, transmission electron microscopy and scanning electron microscopy. The lens was diffusely opaque and brown. It was microspherophakic in appearance, 6.0 mm in equatorial diameter and 4.8 mm in anteroposterior diameter. The lens fibers were well preserved, and ran circularly in the cortex and elliptically in the fetal nucleus. The lens fibers had undergone hyaloid degeneration in the area from the deep cortex to the superficial portion of the adult nucleus. Hyaloid degeneration also extended from the anterior to the posterior pole and was more marked in the equator. The distribution of lens fibers suggested that microspherophakia had developed postnatally. It is surmised that later changes in the shape of the lens affected the lens fibers, inducing hyaloid degeneration.