Congenital Orbital Teratoma

Teratomas account for only 6% of tumours in the head and neck. These tumours contain ectoderm, endoderm, and mesoderm layers. Congenital orbital teratomas are especially rare and clinically present with rapid growth, unilateral proptosis, and palpebral retraction without intracranial involvement. (1- 6)

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Teratomas are the most common intracranial tumors in newborns, and they are commonly found in the midline. These tumors are uncommon in the head and neck andare found in the orbital area injust 0.8% of cases. It is most frequently in the left orbit, and the female: male ratio is 2:1. Approximately 70 cases have been reported in the world. (2-4)

This tumour originates from disorganized growth of pluripotent cells. (1) It contains all three germ cell lines (ectoderm, mesoderm, and endoderm). (1, 2) The histological types of teratomas can be mature, immature, and malignant. Most of the congenital orbital teratomas are mature, and cystic architecture is predominant. 

Although the precise etiology underlying the development of congenital orbital teratoma is not clear, disorganized growth of pluripotent cells is postulated. (1- 3) These tumours are divided into four groups: 1. Complete orbital fetus- in- fetu, 2. Incomplete fetus in the orbit, 3. True orbital teratoma, and 4. A mass with only two germ cell lines. (6)
The relationship between congenital orbital teratomas and inheritance has not been described. (3, 4, 6) In addition, no history of teratogenic influence has been associated with orbital teratoma. (6)

Three categories of intracranial teratomas have been described: small intracranial teratomas that produce hydrocephalus, intracranial teratomas that replace the brain, and intracranial teratomas extending into the orbit or neck.

Congential orbital teratoma is rare. These lesions, located in the infratentorial region, grow rapidly in the postnatal life leading to proptosis, palpebral retraction, and exposure keratopathy. (1)  In affected fetuses the eyes are  sually normally formed but displaced. (4) On occasion orbital teratomas ican be associated with microphthalmia and a destroyed, enlarged, and remodeled orbit. (5)

This tumour may have rapid growth and cause unilateral proptosis, palpebral retraction, keratosis (because of the exposure), destruction of the orbit bone, and even intracranial involvement. (1- 4)
 

Though generally a benign tumour, orbital teratomas can recur as a malignant germ-cell massor undergo malignant degeneration. (2)
 

The prenatal detection of orbital teratomas is made by a routine ultrasound examination. The earliest diagnosis described in the literature was made at 16th week of gestation. (4) The ultrasound can detect characteristics like calcifications, multicystic components, extension of the mass into deeper structures, and other findings like polyhydramnios. (1) A heterogeneous and hyperechoic tumour inside the orbital area with cystic components is sufficient to suspect this disease. (1, 2) In addition, the ocular globe can be displaced anteriorly. (2, 4) It is important to perform colour Doppler ultrasound and evaluate any extension to intracranial structures. (5, 6)
Nuclear magnetic resonance imaging can be useful to determine the extent of the tumor and the appearance and integrity of brain structures  and  the ventricular system. (2)
The definitive diagnosis of congenital orbital teratoma is made by histopathology. (6)

Congenital orbital teratomas should be included in the differential diagnosis of proptosis in the neonate. (6) In the prenatal life the differential diagnoses include dermoid cyst, microphthalmos with cyst, hemangioma, lymphangioma, cephalocele, rhabdomyosarcoma, neuroblastoma, and retinoblastoma. Dermoid cyst is the most common differential diagnosis because is frequently seen in the orbit. (4, 6) Retinoblastoma, the most common intraocular malignancy of infancy can be suspected by ultrasound by the finding of a mass more echogenic than the vitreous, exhibiting  calcifications. (5)

Once congenital orbital teratoma has been diagnosed, follow-up by a multidisciplinary group of  maternal fetal specialists, ophthalmologists, pediatric neurosurgeons, and maxillofacial surgeons with pediatric surgical expertise to create a  comprehensive delivery plan and postnatal treatment plan is recommended. (3)
Cesarean section has been advocated for delivery in order to avoid significant complications such as rupture of  the teratoma and  enhanced damage to the exposed eye. (3, 4)

Factors influencing the prognosisinclude extension of the mass, gender, histopathological components, degree of tissue differentiation, and the compromise of other intracranial structures.  Close follow-up by ultrasound is imperative to evaluate growth and possible extension to intracranial structures. Rapid enlargement  may predict spontaneous rupture (during pregnancy or delivery). (4)
Congenital ocular teratomas exhibit rapid growth postnatally (2) and can be associated with proptosis, palpebral retraction, and keratopathy because of the exposure of the eye. (2)
Some reports have suggested that a benign teratoma may recur as a malignant tumor. (2) Tumours displaying benign biologic behaviorcan being managed conservatively with good prognosis for vision. (2) Complete surgical removal is curative, but some transient or permanent complications can be  encountered. For example, nerve palsies, amblyopia, and vision impairment secondary to nerve compression, can result. Follow-up by an ophthalmologist after surgical  reatment is crucial in these patients. (3)

Once the prenatal diagnosis is made, care by a multidisciplinary group of specialists is key to management planning. In the postnatal life, management depends on the degree of extension of the tumor. A localized tumour is likely amenable to complete excision of the mass,  preserving the eye bulb and maintaining vision. The symmetric reconstruction of the orbital bone vault is important to restore binocular vision.  It may be necessary to use bone grafts from parietal calvaria and/or dermis fat graft. Also, it is important to prescribe antibiotics and constant padding of the eye. Sometimes it is necessary to perform an exenteration of the affected eye because of the severe proptosis and irreversible damage of the eye. (2- 7) The severe cases are very malformative lesions and very invasive and mortality can be high. 

1.    Moon Y-J, Hwang H-S, Kim Y-R, Park Y-W, Kim Y-H; Prenatally detected congenital orbital teratoma. Ultrasound Obstet Gynecol 2008; 31: 106–110
2.    Oladigbolu, K., Abdullahi, H. & Abdullahi, M. (2021). Congenital orbital teratoma: A case report. Journal of West African College of Surgeons, 11(2), 28. https://doi.org/10.4103/jwas.jwas_27_22
3.    Reinhold, A., Meyer, P., Bruder, E., Soleman, J., von der Weid, N., Mueller, A. A. & Savic, M. (2022, junio). Congenital orbital teratoma: A clinicopathologic case report. American Journal of Ophthalmology Case Reports, 26, 101420. https://doi.org/10.1016/j.ajoc.2022.101420
4.    Yang, Z., Du, L. & Zhang, H. (2021, 14 marzo). Congenital orbital teratoma: A clinicopathologic case report. Journal of Obstetrics and Gynaecology Research, 47(6), 2225-2229. https://doi.org/10.1111/jog.14744
5.    Chen, X., Yang, J., He, G., Cheng, C., Zhang, C., Wang, H., He, L., Yang, Z., Chen, L. & Zhao, J. (2020, 19 octubre). Orbital teratoma in the foetus: a rare case without proptosis. BMC Ophthalmology, 20(1). https://doi.org/10.1186/s12886-020-01681-w
6.    Pellerano, F., Guillermo, E., Garrido, G. & Berges, P. (2016, 7 septiembre). Congenital Orbital Teratoma. Ocular Oncology and Pathology, 3(1), 11-16. https://doi.org/10.1159/000448144}
7.    Aiyub, S., Chan, W., Szetu, J., Sullivan, L., Pater, J., Cooper, P. & Selva, D. (2013). Congenital orbital teratoma. Indian Journal of Ophthalmology, 61(12), 767. https://doi.org/10.4103/0301-4738.111219
8.    Onyekwe, L., Onwuegbuna, A. & Emejulu, J. (2010, septiembre). CONGENITAL ORBITAL TERATOMA. Nigerian Journal of Clinical Practice, 13(3), 338-340.

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