This leaflet is to help you understand what epignathus is, what tests you need, and the implication of being diagnosed for your baby after it is born.

What is an Epignathus?

Epignathus is a rare tumour that protrudes from the oral cavity. It is a benign malformation that can be diagnosed in the prenatal life by an ultrasound study. 

How does a Epignathus happen?

It is not clear why an epignathus occurs but in most cases, it is thought to occur due to a disorganised growth of the cells during the embryonic life. It is a very rare condition that happens in about 1 in 35.000 to 1 in 200.000 babies. 

What are the things to watch for during the pregnancy?

Babies with epignathus are at risk of some problems, especially during the delivery time. The tumour that is formed on the mouth, can be small causing some feeding issues or may reach a large size, causing problems with the breathing of the newborn. The ultrasound will help identify if the tumour is compromising other structures of the baby’s head and in this way to establish an appropriate delivery plan. Many women will also accumulate extra amniotic fluid around the baby. This condition is called polyhydramnios. It can stretch the uterus too much and cause early labour well before the due date. 

Could my baby have another associated malformation? 

Yes. Although is considered rare, epignathus may be associated with other findings that could affect the formation of the palate, nose, and tongue. 

If I have another baby, is it going to have an epignathus too?

No. The probability of having another baby with epignathus is very low. There is no evidence that shows this tumor has a genetic predisposition. 

What does it mean for my baby after it is born?
Sometimes, babies that have been diagnosed with epignathus may need a special delivery plan. A specific procedure called an EXIT procedure may be proposed if the mass is compromising the airway and limiting the correct breathing of your baby. This is the reason why once the diagnosis is made by ultrasonography your baby must be examined by a maternal-fetal specialist who will follow the growth of this mass and help formulate a plan for delivery. 

The baby often requires a surgical procedure to remove the tumour from the oral cavity. This intervention may require very complex care in a specialised hospital for weeks after birth.

Will it happen again?

If the surgical procedure is successful with total removal of the mass, the tumour will not grow again. 

What other questions should I ask?

  • Where should I deliver?
  • How is the procedure to secure the breathing of my baby performed during delivery?
  • 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