What Is Hydrocephalus and How Is It Treated?

Hydrocephalus is a condition in which there is a lack of absorption, blockage of flow or overproduction of the cerebral spinal fluid (CSF) that is found inside the ventricles of the brain. This may result in a buildup of fluid that can cause pressure inside the head to increase and the skull bones to expand to a larger-than-normal appearance.

What causes hydrocephalus?

Hydrocephalus occurs in approximately 1 out of every 500 births. It usually occurs due to:

  • Blockage of CSF flow inside of the head
  • Problems with the body absorbing CSF
  • Overproduction of CSF

Hydrocephalus can be congenital (present at birth) or it can be acquired later in life. If not caused by genetics, doctors think many factors—both genetic and environmental—contribute to the condition. In a small percentage of children, a single-gene defect on the X chromosome, or another chromosome, causes hydrocephalus. In these cases, the chance for recurrence is higher.

Causes of acquired hydrocephalus may include:

  • Tumor
  • Infection
  • Prematurity
  • Bleeding inside the head
  • Birth injury
  • Abnormal blood vessel formation inside the head
  • Trauma

What are the symptoms of hydrocephalus?

Common symptoms of hydrocephalus in younger children may include:

  • A full or bulging fontanelle (soft spot located on the top of the head)
  • Increasing head circumference
  • Seizures
  • Bulging eyes and an inability to look up while the head is facing forward
  • Very noticeable scalp veins
  • Increased irritability
  • High-pitched cry
  • Poor feeding
  • Projectile vomiting
  • Sleepiness or decrease in alertness
  • Developmental delays

In cases for which hydrocephalus is not congenital but is due to a tumor, trauma, infection or other causes, symptoms seen in older children may include:

  • Complaints of severe headaches
  • Nausea and vomiting
  • Changes in personality, behavior or school performance
  • Problems with bowel or bladder control
  • Increased movement in the arms or legs
  • Seizures

Symptoms of hydrocephalus may resemble other conditions or medical problems. Always consult your child’s doctor for a diagnosis.

How is hydrocephalus diagnosed?

Hydrocephalus may be diagnosed before birth during a prenatal ultrasound. However, in many cases, hydrocephalus does not develop until the third trimester of the pregnancy, so it may not be seen on ultrasounds performed earlier in pregnancy.

The diagnosis of congenital hydrocephalus may also be made at birth, even though it is sometimes diagnosed in older children who have developmental delays.

Diagnostic tests that may be done to confirm hydrocephalus include:

How is hydrocephalus treated?

Specific treatment for hydrocephalus will be determined by your child’s doctor based on his age, overall health and medical history, as well as the cause of the condition.

The goal of treatment at Children’s Healthcare of Atlanta is to reduce the pressure in a child’s head and drain the CSF. Sometimes, medicines or procedures to draw off the extra CSF may be used. Surgery may be needed for some cases and usually involves placing a mechanical shunting device into the child’s head to help drain extra CSF from the brain.

Potential complications from shunts or surgery may include:

  • Infection
  • Shunt malfunction that results in under-drainage or over-drainage of the CSF
  • Bleeding

Other complications may include fever, nausea or vomiting, irritability, headache, redness and swelling along the area of the tubing, and decreased alertness or complaints of being tired. Following surgery, you will receive instructions on how to care for your child at home and information about signs or symptoms requiring medical care.

Lifelong considerations for hydrocephalus patients

Hydrocephalus can affect the brain and a child’s development. The key to treating hydrocephalus is early detection, treatment and prevention of infection. Children with hydrocephalus require frequent medical tests to make sure their shunts are working. Our team at Children’s will work with your family to provide education and guidance as your child grows and develops.

Your child’s doctor may also recommend genetic counseling to discuss the risk of hydrocephalus recurring in future pregnancies, as well as prenatal testing for hydrocephalus.