This leaflet is to help you understand what a congenital orbital teratoma is, and what the implication of being diagnosed is for your baby.

What is a Congenital Orbital Teratoma?

Congenital Orbital Teratoma is a very rare tumor located in the eye socket (orbit) of babies. It is a benign malformation that often can be diagnosed in prenatal life by an ultrasound study. 

How does a Congenital Orbital Teratoma happen?

It is a very rare condition that happens in 0.8% of all the babies with teratomas of the head and neck. It is not clear why a congenital orbital teratoma occurs, but the most accepted theory consists of a disorganized growth of the cells during the embryonic life. 

What are the things to watch for during the pregnancy?

Once your obstetrician finds the mass in the orbit by ultrasound, your baby should be followed by a multidisciplinary medical group (including a maternal-fetal specialist, pediatrician, and pediatric eye surgeon). Serial ultrasounds are going to be made to follow the growth of the mass and assess whether other structures of the face and head of the baby are involved. These assessments will help to establish an appropriate delivery plan and what treatment may be required for the infant after birth.  

Could my baby have another associated malformation? 

Yes. Orbital teratoma can be associated with alterations of the optical nerve and retina, and can affect the growth of the eye; in severe cases, it can affect the development of underlying brain structures. 

If I have another baby, is it likely to have a Congenital Orbital Teratoma too?

No. The probability of having another baby with congenital orbital teratoma is very low. There is no evidence that shows this tumour has a genetic predisposition. 

What does it mean for my baby after birth?
Your doctor may advise you that the baby should be delivered by caesarean to avoid complications, such as rupture of the teratoma. The tumour can rapidly grow after delivery, so the ideal treatment consists of a complete surgical removal in the first days of life. When the tumour can be excised, results are generally good with respect to the baby’s vision and the cosmetic result.  In severe cases with larger masses, more extensive surgery could be necessary, and it may not be possible to conserve the eye of the baby. This intervention may require very complex care in a specialised hospital for weeks after birth.  

Will it happen again?

If the tumour is limited to the orbital area and has not extended into deeper tissue, it is likely that complete surgical removal will be successful. In this case   it is highly unlikely that, the tumour will not grow again. 

What other questions should I ask?

  • Where should I deliver?
  • If the complete resection of the tumor is possible, is going my baby to have good vision in the future?
  • What happens if the tumor involves the structures inside the head of my baby?
  • How is the procedure to extract the complete eye of my baby performed?
  • Where will the baby receive the best care after it is born?
  • Can I meet the team of doctors that will be assisting my baby when it is born in advance?

Last updated May 2023