This leaflet is to help you understand what frontal encephalocele is, what it means for my baby after it is born, and what treatments are available.

What is a Frontal Encephalocele?

An encephalocele is an opening in the skull of your baby through which skin-covered brain contents (meninges and cerebrum) bulge out. Encephaloceles can develop in different parts of the head of your baby. Frontal encephalocele is the defect that is formed specifically in the forehead of the baby.  

How does a Frontal Encephalocele happen?

There have been various theories proposed about the origin of encephalocele. It is thought that encephaloceles develop due to a failure in the closure of an embryonic structure called the neural tube in early embryonic life. This alteration affects the normal formation of the skull of the baby. 

Could my baby have another associated malformation? 

Yes. Up to 40% of babies can have associated anomalies in the head, eyes, face, health, and even limbs. The commonest associated malformations are the finding of a small head (microcephaly) and excess liquid inside the head (ventriculomegaly). However, encephaloceles can also be found as an isolated anomaly. 

If I have another baby, is it going to have a Frontal Encephalocele too?

If your baby has only an encephalocele without other defects, the probability of having another baby with this disease is low. However, if the disease is caused by a genetic anomaly, this risk may be increased. This is the reason why early evaluation with ultrasound is important when planning a future pregnancy.

What does it mean for my baby after it is born?
The delivery plan can change depending on the size of the defect. Unless the lesion is considered very small Caesarean section is often chosen to avoid further damage. Once the diagnosis is made during pregnancy, your baby should be followed by a group of perinatal specialists (pediatricians, neonatologists, maternal fetal specialists, neurosurgeons, neurologists, geneticists). These professionals will create a delivery plan and a surgical repair strategy to help your baby to have the best possible outcome. 

What is the treatment of Frontal Encephalocele?

The treatment of frontal encephalocele consists of surgical resection of the abnormal neural tissue within the herniated sac, followed by the repair of the defect in the head.  The prognosis of these babies is sometimes poor. Some of these patients can not survive after a few days of life and some of the babies that do survive may have significant alterations in their neurological development. This is the reason why the option of termination of the pregnancy may be presented to you after the diagnosis is made (according to your country’s laws).  

Will it happen again?

If the surgical procedure is successful, the lesion will not recur on your baby’s face.

What other questions should I ask?

  • Where should I deliver?
  • What happens if my baby has other malformations in its head?
  • How is the procedure to repair the complete lesion 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