Eye-conserving treatment in massive congenital orbital teratoma

An 8-month-old healthy girl presented with a left orbital mass, which orbital magnetic resonance imaging revealed to be a well-circumscribed, mostly cystic lesion. The patient underwent orbitotomy via inferior fornicial conjunctival approach. Tumour shrinkage was accomplished by aspiration of the intralesional fluid, and the remaining debulked mass was removed by total excisional biopsy. Pathological examination revealed a cystic tumour lined mainly with keratinized stratified squamous epithelium, in addition to small foci of mucinous ciliated epithelium resembling respiratory epithelium. Histopathological findings were consistent with the diagnosis of mature orbital teratoma (hair follicles, adipose tissue, mature glial elements, choroid plexus-like papillary organizations and small foci of cartilage in the cyst wall). Derivatives of all three germ lines were present. At 56-month follow up, uncorrected visual acuity in the affected eye was 6/9. This case demonstrates the importance of decompressing the tumour before dissecting it from the periorbital tissues in an eye-conserving approach to orbital teratoma.