When Your Baby Is Born With Part of the Brain Outside of the Skull (Encephalocele): What to Know

An encephalocele is a congenital problem. This means your baby is born with it. It happens when an opening in the skull allows part of the brain and its coverings (meninges) to bulge out into a small pouch.

• Most of these pouches stick out from the back of the skull.
• Some can't be seen because they're in the forehead or sinus area.

An encephalocele is a type of neural tube defect. This tube is what forms the baby's brain and spinal cord during pregnancy.

An encephalocele can be linked to other health problems, like:

• A cleft lip or cleft palate.
• Extra fingers or toes.
• Kidney problems.
• Genital problems.

Often, a group of problems can point to a certain syndrome. Children who had encephaloceles may need therapy to help them manage new problems.

What are the causes?

The cause isn't known.

The opening in the skull forms very early in pregnancy. It may be genetic. This means that it can be passed down in families. Other causes may be:

• Things in the environment.
• Certain infections during pregnancy.

What are the signs or symptoms?

The pouch can usually be seen at birth as a bulge or lump. Signs and symptoms may include:

• Slow growth or development.
• Learning problems.
• Jerky movements that can't be controlled (seizures).
• Problems with eyesight.
• A head that's smaller than usual.
• Weakness, loss of use, or stiffness of the arms and legs.
• Problems with movements made when walking and reaching (ataxia).
• Trouble breathing.

Signs and symptoms depend on many things, like:

• Where and how big the bulge or lump is.
• If there are any other health problems.

How is this diagnosed?

Your unborn baby may be diagnosed while you're getting an ultrasound.

You may also need an amniocentesis. This is done if it's thought that the baby may have a congenital problem. For this, a small amount of the fluid in your uterus is removed for testing.

An encephalocele may also be diagnosed at birth, when the pouch can be seen. In rare cases, a pouch in the forehead or sinus area isn't found until there are symptoms. If the health care provider thinks your baby has a pouch that can't be seen, your baby may have imaging tests, like:

• Ultrasound.
• CT scan.
• MRI.
• X-ray.

How is this treated?

Treatment usually involves surgery. Depending on how bad the pouch is, surgery may be done:

• Right after your child is born.
• A few months after birth.

Sometimes, many surgeries are needed. Surgery includes:

• Taking the pouch off.
• Taking out any injured brain tissue.
• Placing the brain tissue back into the skull.
• Fixing problems in the skull.

This will help to avoid a life-threatening infection or harm to the brain tissue.

Other problems may also be fixed through surgery. If your child has extra fluid in the brain (hydrocephalus), a tube may be placed in the skull. This helps drain the fluid and prevent pressure from harming the brain.

Follow these instructions at home:

• Learn as much as you can about encephaloceles. This can help you make treatment decisions and know what to expect.
• If your baby doesn't have surgery right after birth, follow the provider's instructions about how to:
  • Keep your baby safe.
  • Protect your baby's head.
  • Feed your baby.
  • Comfort your baby.
• Give your child medicines only as told.
• Keep all follow-up visits. Your child will need to be checked at times for issues with their brain and development.

Where to find more information

To learn more:

1. Go to the Centers for Disease Control and Prevention (CDC) at cdc.gov.
2. Click "Search" and type "encephalocele."
3. Find the link you need.

Contact a health care provider if:

• Your child has repeated infections of the upper airways.
• Your child has a fever.
• Your child throws up.

Contact your child's provider right away if:

• Your baby is younger than 3 months old and has a temperature of 100.4°F (38°C) or higher.
• Your child is 3 months old or older and has a temperature of 102.2°F (39°C) or higher.
• Your child has a fever, and they look or act sick in a way that worries you.

If you can't reach the provider, go to an urgent care or emergency room.

Get help right away if:

• Your child's behavior changes for no known reason.
• Your child has a seizure.