Eye-conserving treatment in massive congenital orbital teratoma.
ABSTRACT 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.
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ABSTRACT: Teratomas are rare germ cell tumors that comprise approximately 1% of orbital tumors in childhood. Review of the world literature revealed only 51 well-documented patients with true congenital orbital teratomas. We present a newborn girl with a massive orbital teratoma that caused significant orbital enlargement with inferior and lateral displacement of the zygoma and a thinning of the orbital roof. The ipsilateral maxilla and palate were depressed inferiorly. No bony invasion was seen despite its massive size. A craniofacial approach was used to safely and completely extirpate this tumor. Histological sections demonstrated derivatives of all three germ cell layers. Recommendations for definitive treatment and a review of the literature are presented.Annals of Plastic Surgery 12/1993; 31(5):453-8. · 1.38 Impact Factor
Article: Massive congenital orbital teratoma.[show abstract] [hide abstract]
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.Ophthalmic Plastic and Reconstructive Surgery 12/2005; 21(6):445-7. · 0.67 Impact Factor
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ABSTRACT: A newborn baby had an orbital-intracranial benign teratoma radically excised with preservation of the eye. Three years later she developed recurrent proptosis, and reoperation showed an invasive orbital tumour that histologically was a malignant germ-cell neoplasm. The relationship of the two tumours is discussed. Malignant change in a benign orbital-intracranial teratoma of the newborn has not been previously reported.British Journal of Ophthalmology 03/1986; 70(2):111-3. · 2.73 Impact Factor