Figure - available from: Case Reports in Ophthalmological Medicine
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(a) Histological examination showing respiratory tissue. (b) Histological examination showing a cluster of neuroganglion cells and nerve cells. (c) Histological examination showing cartilage tissue (top left) and bone tissue (bottom). (d) Histological examination showing a cartilaginous lobule in the middle of an adipose lobule. (e) Histological examination showing keratinized malpighian epithelium.
Source publication
Purpose:
Teratomas are congenital tumors of stem cells derived from the three germ layers. They are frequently located in the sacrococcygeal region. Orbital teratoma is rare with less than 70 cases reported until 2016. We report the case of prenatal exophthalmia discovered by ultrasound exam which turned out to be a teratoma postnatally. The newbo...
Citations
... Although rare, they may thus occur in the orbital region. [1][2][3] Orbital teratomas are extremely rare tumors, always congenital and benign, with less than seventy cases reported in the English literature until 2016. Accounting for less than 1% of all childhood orbital tumors, [2] they result from ectopia or disorientation of germinative cells, involving neonates with no other comorbidities. ...
... [1][2][3] Orbital teratomas are extremely rare tumors, always congenital and benign, with less than seventy cases reported in the English literature until 2016. Accounting for less than 1% of all childhood orbital tumors, [2] they result from ectopia or disorientation of germinative cells, involving neonates with no other comorbidities. [1][2][3] These tumors appear to have a 2:1 preponderance for females, with a slight preference for the left orbit. ...
... Accounting for less than 1% of all childhood orbital tumors, [2] they result from ectopia or disorientation of germinative cells, involving neonates with no other comorbidities. [1][2][3] These tumors appear to have a 2:1 preponderance for females, with a slight preference for the left orbit. [1,3] Characteristically, orbital teratoma presents with severe unilateral proptosis, sometimes enormous enough to cause dystocia, thus constituting a clear indication for a mandatory cesarean section. ...
To report a rare case of orbital teratoma diagnosed in utero by ultrasound examination at 24 + 4 weeks of gestation. The fetus was delivered by cesarean section, by terminative medical indication, at 39 weeks of pregnancy, due to the possibility of dystocia caused by the size of the tumoral mass, or the risk of tumor rupture during vaginal delivery, with risk to fetal survival. The neonate, a 3 day‑old female patient was examined in our Department with a large tumoral mass in the right orbit, severe proptosis, producing severe disfigurement. The diagnosis of orbital teratoma was confirmed by clinical, image, and histopathological exams. To the authors best knowledge this is the first case of orbital teratoma in Brazilian literature reporting a case where the diagnosis was made in utero, at 24 + 4 weeks of gestation.
... Although rare, they may thus occur in the orbital region. [1][2][3] Orbital teratomas are extremely rare tumors, always congenital and benign, with less than seventy cases reported in the English literature until 2016. Accounting for less than 1% of all childhood orbital tumors, [2] they result from ectopia or disorientation of germinative cells, involving neonates with no other comorbidities. ...
... [1][2][3] Orbital teratomas are extremely rare tumors, always congenital and benign, with less than seventy cases reported in the English literature until 2016. Accounting for less than 1% of all childhood orbital tumors, [2] they result from ectopia or disorientation of germinative cells, involving neonates with no other comorbidities. [1][2][3] These tumors appear to have a 2:1 preponderance for females, with a slight preference for the left orbit. ...
... Accounting for less than 1% of all childhood orbital tumors, [2] they result from ectopia or disorientation of germinative cells, involving neonates with no other comorbidities. [1][2][3] These tumors appear to have a 2:1 preponderance for females, with a slight preference for the left orbit. [1,3] Characteristically, orbital teratoma presents with severe unilateral proptosis, sometimes enormous enough to cause dystocia, thus constituting a clear indication for a mandatory cesarean section. ...
To report a rare case of orbital teratoma diagnosed in utero by ultrasound examination at 24 + 4 weeks of gestation. The fetus was delivered by cesarean section, by terminative medical indication, at 39 weeks of pregnancy, due to the possibility of dystocia caused by the size of the tumoral mass, or the risk of tumor rupture during vaginal delivery, with risk to fetal survival. The neonate, a 3 day-old female patient was examined in our Department with a large tumoral mass in the right orbit, severe proptosis, producing severe disfigurement. The diagnosis of orbital teratoma was confirmed by clinical, image, and histopathological exams. To the authors best knowledge this is the first case of orbital teratoma in Brazilian literature reporting a case where the diagnosis was made in utero, at 24 + 4 weeks of gestation.
... Teratomas are congenital tumours derived from the three germ layers. Although rare, the orbit is a recognised location for congenital teratoma [1]. ...
Congenital orbital teratomas are rare entities with few case reports detailing their prenatal and perinatal imaging features. We present the case of a congenital orbital teratoma initially detected as cystic lesion on prenatal ultrasound, with foetal and postnatal imaging showing evolution of characteristic MRI appearances. Knowledge of these appearances and the ability to diagnose these rare entities in foetal life can aid management and operative planning in the immediate postnatal period.
Congenital orbital teratomas are rare entities with few case reports detailing their prenatal and perinatal imaging features. We present the case of a congenital orbital teratoma initially detected as cystic lesion on prenatal ultrasound, with fetal and postnatal imaging showing evolution of characteristic MRI appearances. Knowledge of these appearances and the ability to diagnose these rare entities in fetal life can aid management and operative planning in the immediate postnatal period.
Background
Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration.
Case presentation
We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up.
Conclusion
Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.
