Encephaloceles are frequently associated with other cranial (head, skull or brain) and/or facial abnormalities. 

In the United States, encephaloceles occur in approximately  one to four per 10,000 live births. The presence of an encephalocele is associated with an increased incidence of death in utero. Experts estimate that only half of the children with encephaloceles survive to birth. They are seen more commonly in females than males. 

Doctors diagnose most encephaloceles during a routine ultrasound. An alpha-fetoprotein blood test usually does not help to diagnose encephaloceles. This is because AFP levels are not typically elevated with this defect because the defect is covered by skin. 

If your child is diagnosed with encephalocele, we recommend a thorough examination of them to look for other anomalies.

The prognosis for an encephalocele depends on a number of things, including:

• The size of the defect(s) and the amount of brain tissue that has pushed out into the sac
• The location of the defect
• The presence of other birth defects or anomalies

Encephaloceles may be associated with brain malformations that can have an effect on the prognosis. 

The location of the encephalocele greatly affects the prognosis. Those located in the front have a 100% survival rate, while those located in the back have a 55% survival rate. In the United States, the most common type of encephalocele is in the back, while in Southeast Asia a frontal type is more common. 

Approximately 13-44% of babies with encephaloceles have a chromosomal abnormality, and approximately 75% of babies who survive will have some degree of mental deficit. 

If your baby has been diagnosed with encephalocele, we recommend that you deliver them at a tertiary care center (a hospital that is capable of caring for critically ill infants). 

If your baby has been diagnosed with an encephalocele, you are at greater risk for some complications and problems during pregnancy, including:

• Possible cesarean section

If the encephalocele is large, your baby's head may be too big to fit through the birth canal. In this case, you may have to have a cesarean section to deliver your baby. 

However, minimizing maternal risk is most important when the encephalocele is large and there are associated anomalies because of the poor prognosis for the baby.

• Miscarriage 

Fetuses with an encephalocele are likely to die before birth. Approximately 21% are born alive. Of those born alive, only 50% will survive. 

Fetuses with a front-type encephalocele are much more likely to survive than those who have an encephalocele on the back of the head. The absence of brain tissue within the sac is the single most favorable prognostic indicator. Presence of associated malformations is another indicator of prognosis.

Once an accurate diagnosis of the defect and any associated anomalies has been made, you will be counseled regarding your baby's probable outcome or prognosis. If the prognosis is poor, but you desire is to continue the pregnancy, palliative care may be offered as an option of care for your baby when they are born. 

After surgery, recovery will vary depending on the age of your baby, how extensive of a repair was required and surgical time. 

• Breathing assistance

Your baby likely will need assistance with breathing for a while. During surgery, we will pass an endotracheal tube between the two vocal cords and into the trachea or windpipe. This tube is attached to a ventilator, which can deliver oxygen and help your baby's lungs inflate. 

• IV lines

Your baby will also have IV lines. If your baby is a newborn at the time of surgery, these lines may be placed in the umbilical cord. The umbilical cord normally has two arteries and one vein. The arterial line provides access for blood to be drawn for lab work rather than having to perform a needle stick. We can also monitor blood pressure via an arterial line and administer fluids and medication.

• Nutrition

The vein in the umbilical cord will also be threaded with a thin flexible catheter or tubing. Through this line, we can give nourishment in the form of an IV solution called TPN, or total parenteral nutrition, if your baby is not able to eat. TPN includes two types of solutions. One contains all the calories, vitamins and minerals necessary for normal daily intake. This is a yellow solution. Another type of nourishment provided through this line is fats. This solution appears white and milky. 

• Peripheral IV

Your baby may also have a peripheral IV. These lines can be in the arms, hands, feet or scalp.

We will explain each procedure to you as it is done unless it is an emergency situation. 

Your baby will go home when they have recovered from surgery and is able to take in enough food to maintain and gain weight. Your baby's respiratory status or breathing also must be stable. If it is necessary to send your baby home with equipment, we will train you how to care for your baby and the equipment, and you will be trained in emergency procedures. Babies with encephalocele often require follow-up with a variety of specialists once they go home.

If you have decided against aggressive therapy, your baby can go home with you with support from a hospice service.

Babies with a frontal encephalocele, no associated syndrome or defects, and no brain tissue herniating into the sac have a good chance of survival. 

Babies with an encephalocele at the back of the head have a 55% survival rate. Long-term prognosis for survival becomes less likely if there are other complications, such as associated defects or syndromes or if brain tissue protrudes into the sac. Approximately 75% of these infants who do survive have varying degrees of mental deficit. 

Learn more about the services at Children's Wisconsin:

• Craniofacial Clinic
• Eye Clinic
• Neurology Program
• Neurosurgery Program