Congenital mature teratoma of the right orbit

BACKGROUND: Teratomas are congenital embryonic tumours composed of a wide diversity of tissues derived from the three germinal layers (ectoderm, mesoderm, endoderm). Located in the orbit they endanger the eye and the bony structures. The main goal of treatment is preservation of the eye. In most cases this will not be feasible. CASE REPORT: A newborn boy was admitted to our hospital because of a prenatally known protrusion of the right globe. On examination the baby had a large retrobulbar orbital tumour, which displaced the globe ventrally. On MRI the tumour had an extent of 5 cm. It compromised the frontal bone and reached intracranially. No other malformations were found. The tumour was transorbitally resected and the eye exenterated. A congenital mature teratoma was diagnosed. To preserve orbital volume an implant was inserted (Hema globe, 18 mm). FOLLOW-UP: The healing course was uneventful. Illig implants were changed without difficulties. After 10 months an individual prosthesis was inserted. Control CT scans showed no signs of tumour. CONCLUSIONS: The case presented underlines the possible extent and impact of congenital mature teratomas. Despite the extent of the tumour and the very young age of the patient a satisfactory treatment result was achieved.