Optic nerve tfratoma associated with orbital dhrmoid and microPimialmia

Purpose. Teratomas and dermoid cysts are clionstomas, tumors composed o: normal tissue elements in an abnormal location. A paiient vith ail opiic nerve teratoma associated wiih an orbital dermoid cyst and microphthalmia is reported. Methods. The patient's medical records were reviewed, including ophthalmologic and systemic examinations and results of radiologie and pathologic studies. BssuJlj. A 5-day-old paiient presented with microphthalmia of the right globe. The patien underwent a lensectomy-vitrectomy procedure for treatment of presumed persistent hyperplastic primary vitreous. At age 2 months a cyst was noted in the inferior right orbit. Compute-1 tomography and magnetic resonance imaging scans showed a right inferior orbital cyst witr. calcifications. A separate mass involving the mtracramal portion of the right optic nerve v as identified. 1-xcisional biopsy of die orbital mass revealed a dermoid that contained odoritogenic rests, dental papillae, and two formed leeth. Subtotal neurosurgical excision of the intracraniaJ mass revealed cartilage, smooth muscle, and gastrointestinal mucosa consistent with mature teratoma. A complete systemic evaluation revealed no other teratomatous lesions. The patient remains in excellent health at age 14 months. Conclusion: The orbital demoid in our patienl was very unusual because it contained odontogenic tissue. Although the optic nerve teratoma and the orbital dermoid were not anatomically linked, the association ind close proximity of diese lesions suggests a common origin. To our knowledge, this is uv first reported case of an optic nerve teratoma associated with an orbital dermoid containing lental structures and microphlhalmia.