Yonago Acta medica 1996;37:99-106

Histopathology of Microspherophakia in the Weill-Marchesani Syndrome

Masao Nagata, Shigeru Takagi, Atsushi Yamasaki, Sayo Tsunematsu, Takeshi Kumagami, Chiemi Itamochi and Akihiko Tamai

Department of Ophthalmology, Faculty of Medicine, Tottori University, Yonago 683, Japan

We examined a surgically obtained lens from a 66-year-old male with the Weill-Marchesani syndrome 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 ribers were well preserved, and ran circularly in the cortex and elliptically in the fetal nucleus. They had undergone hyaloid degeneration occupying 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 ribers suggested that microspherophakia developed postnatally. Changes in the shape of the lens would later affect the lens fibers and induce hyaloid degeneration.

Key words: histopathology; hyaloid degeneration; Iens fibers; microspherophakia; Weill-Marchesani syndrome

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