Congenital orbital teratoma: a clinicopathologic case report

A 5-day-old infant boy was noted to have severe left proptosis at birth. The left eye protruded superotemporally through the palpebral fissure and had exposure keratopathy. There was frank left afferent pupillary defect. Computerized tomography (CT) showed a left orbital soft tissue mass with foci of calcification. Magnetic resonance imaging (MRI) studies revealed a left orbital mass with solid and cystic portions without intracranial extension. As the eye was considered to be nonsalvagable, a lid-sparing type, modified exenteration was performed. Histopathologic examination demonstrated various mature tissues of all three embryonic germinal cell lines. This case represents one of the rare examples of true congenital orbital teratoma, which is an uncommon cause of hideous proptosis in the neonate. MRI may prove useful in differentiating this tumor from more common conditions.