Unilateral diplophthalmos

Abstract

In a child born of a full-term pregnancy, unilateral diplophthalmos without proboscis was observed along with other craniocervical abnormalities (ipsilateral temporoparietal porencephaly, supernumerary teeth, and cervical cyst). A globe almost normal in shape and size was observed within the left orbit. The upper quadrant of this globe was attached to a smaller, pear-shaped, supernumerary eye containing a small lens, normal vitreous humor, ciliary body structures, avascular retina, choroid, and a rudimentary optic disk. When a two-peaked and enlarging intraocular mass was observed clinically, the eye was enucleated because of suspected neoplasm. The enlarging prominence had been caused by the growing second globe. Our findings indicated that a single primary optic vesicle was formed (globe I with an optic fasciculus), and some unknown damage was caused when this single primary optic vesicle induced the lens formation by contacting the ectoderm. Although the conception and early embryonal life of this child had taken place during a period of increased radiation exposure caused by the explosion of the nuclear plant at Chernobyl, U.S.S.R., in the spring of 1986, it seemed unlikely that the abnormality could have been caused by radioactive fallout in the mountains of Austria.