Massive congenital orbital teratoma

Abstract

Orbital teratomas are rare embryonic tumors composed of a wide diversity of tissues derived from the three germinal layers. The presenting features include, a healthy newborn with extreme unilateral proptosis; marked stretching of the eyelids over a tense, fluctuating mass, with elongation of the palpebral fissure; enlargement of the bony orbit (two to three times normal size) with subsequent nasal and malar deformities; and transillumination of all or part of the orbital mass. Commonly the eye is normally developed but often vision is not preserved either due to exposure or secondary optic atrophy. The objective in the management of orbital teratoma is to save the eye to encourage orbitofacial development, maintain cosmesis and retain some vision. We report a case of massive congenital orbital teratoma successfully removed by an eyelid-sparing exenteration technique.