Maurice F. Rabb » Intraocular Teratoid Medulloepithelioma

A three week old white girl, the product of a normal pregnancy, labor, and delivery, was found by her pediatrician to have a "cloudiness" of the anerior segment of the right eye. During the next two weeks, the parents noted a progressive white color and oval shape to the pupil and the chils was referred to a pediatric ophthalmologist at age five weeks.

Ocular examination revealed a completely normal left eye. The right had a normal intraocular pressure to finger touch. The cornea was clear and equal in size to that of the fellow eye. The anterior chamber was shallow but formed. The pupil was small and irregular but reacted to light. Persistent vascularized pupillary membranes obscured a view of the lens which had a "milky" appearance. This iris was lighter in color in the involved eye but there was no iris neovascularization.

The diagnostic considerations at that time included congenital cataract, persistent hyperplastic primary vitreous (PHPV), and possible intraocular tumor. B-scan ultrasonography revealed a normal posterior segment with no evidence of intraocular tumor.