Encephalocele

Encephalocele is an open neural tube defect (NTD) that is most often present at birth. Normally, the neural tube folds and closes to form the brain and spinal cord around the third or fourth week of pregnancy, before most women even know they are pregnant.

Encephalocele happens when the tube does not fully close during pregnancy. This causes the brain, and the membranes that cover it, to poke out through openings in the skull. The Centers for Disease Control and Prevention (CDC) reports that each year about 375 or one out of every 10,000 babies born in the U.S. will have encephalocele.

Types of encephalocele

Encephalocele can range from minor to severe based on where it occurs and what it involves. Encephalocele is are most often described as:

  • Nasofrontal—in the nose and forehead
  • Nasoethmoidal—in the nose and ethmoid sinus
  • Naso-orbital—in the nose and the eye
  • Meningocele—when the bulging area contains only cerebrospinal fluid (CSF) and the overlaying membrane
  • Encephalomeningocele—when brain tissue is involved

Encephaloceles can also be classified as:

  • Primary—present at birth
  • Secondary—as a result of a problem, such as trauma

Symptoms

Symptoms of encephalocele may include:

  • A lump on the head or neck
  • Headache
  • Drainage of CSF from the encephalocele
  • Drainage from the nose
  • Vision problems
  • Trouble learning
  • Abnormally small head 
  • Seizures
  • CSF buildup in the brain 
  • Paralysis of the limbs

Treatment

At Children’s, we provide a multidisciplinary approach to treat encephalocele. Our neurosurgery, craniofacial surgery, plastic surgery and pediatric intensive care unit (PICU) teams provide a unique treatment plan for your child.

The treatment includes surgery, which will most likely occur within the first few months after birth. The surgery may involve:

  • Moving the bulging area back into the skull.
  • Removing extra tissue that protrudes from the skull.
  • Correcting other deformities that result from the encephalocele.