Congenital Orbital Teratoma

Abstract

We report a case of congenital orbital teratoma. A 3-day-old male, born at 39 weeks' gestation without relevant prenatal history, presented with a large vascularized proptotic mass distorting the left midface. Laboratory studies showed elevated serum alpha-fetoprotein (12,910 ng/ml). Computed tomography showed a multiloculated heterogeneous lesion composed of hypodense and hyperdense calcified areas encompassing the whole orbital cavity with expansion of the bony walls, as well as forward displacement and compression of the eyeball without extension to surrounding structures. Clinical, imaging and laboratory features were consistent with congenital orbital teratoma. Due to pronounced proptosis with exposure keratopathy and corneal perforation, no motility of the globe and no vision in the affected eye in a resource-limited setting, the patient underwent orbital exenteration. Histopathological examination confirmed the diagnosis of mature cystic teratoma. We describe the clinical course, radiographic and histopathological findings of this rare orbital tumor.

Prenatal Exophthalmia Revealing a Postnatal Orbital Teratoma

“…Orbital teratoma is an extremely rare tumor developed from primitive germ cells [8]. In fact, Pellerano et al reported only 70 cases of such tumor throughout English literature until 2016 [9]. The present report is the first documented case of the disease in Burkina Faso.…”

“…Massive exophthalmia may lead to exposure keratopathy and its complication such as abscess formation and eye perforation [ 9 , 11 , 12 , 15 ]; this is what happened with our patient.…”

Congenital orbital teratoma: a case report with preservation of the globe and 18 years of follow-up

“…Since then, there have been 70 published cases that fulfill the characteristics of a true congenital orbital teratoma [ 4 ]: presentation at birth, being primarily intraorbital and comprising of all 3 germ cell layers. Some authors have found a female predominance (F/M:2/1) [ 5 ] or left orbit predominance [ 6 ] but others have failed to show this discrepancy [ 3 ]. Heredity and teratogens have not been shown to play a role in the incidence as there is no family history of congenital deformities with nonconsanguineous parents, there is a history of normal pregnancy and delivery and no history of teratogenic influences to the mother [ 7 ].…”

“…The most common presentation of orbital teratomas is that of a well circumscribed rapidly growing mass causing massive unilateral axial proptosis, chemosis, exposure keratopathy and markedly distended eyelids with elongation of the palpebral fissure. In primary congenital orbital teratomas, the orbital enlargement is not accompanied by bony destruction, lysis or intracranial or sinus involvement and the optic nerve is usually either encased within the tumor, adherent or severely compressed leading to severe ischemia and atrophy [ 2 , 6 , 11 ].…”

Congenital orbital teratoma: A case report

“…[6] Orbit is a rare (0.8%) site of extra-gonadal teratoma with over 70 cases have been reported in literature. [7] Only one case of orbital teratoma has been reported from Pakistan in 2017. [8] The clinical suspicion in that case was retinoblastoma, although no radiological studies were performed.…”