Hydrocephalus: Raising Awareness and Increasing Education

Ruth E. Bristol, MD
Associate Program Director, Pediatric Neurosurgery

One of Dr. Bristol’s hydrocephalus patients, Zane (right), and his brother, Ryker (left).

Close up of where Zane had his shunt surgically implanted.

Aaaar! Pirates these Panceroff boys be (Zane, right, and Ryker, left)!

Last month was Hydrocephalus Awareness Month, but still, we find that too many families have never heard of hydrocephalus or how it can be treated. Here at BARROW Neurological Institute at Phoenix Children’s Hospital, our team of neurosurgeons and researchers are dedicated to providing the best care and furthering treatment options for children with hydrocephalus.

So, first, let’s identify hydrocephalus. Our brains are protected by a fluid called cerebrospinal fluid (CSF). Most CSF is found in fluid-filled areas, called ventricles, inside the brain. The fluid’s main purpose is to cushion and protect the brain and the spinal cord. Some babies have extra CSF in and around the brain. Excess CSF can increase the pressure in your baby’s head. This causes the bones in your baby’s skull to expand and separate. Subsequently, the baby’s head may look larger than normal.

Who is affected?

The number of people who develop hydrocephalus or who are currently living with it is difficult to establish since the condition occurs in children and adults, and can develop later in life. Some estimates report one to two of every 1,000 babies are born with hydrocephalus. There are, however, currently more than 1 million people living with hydrocephalus in the US today.

What causes hydrocephalus?

It may be caused by the following:

  • The fluid is blocked from flowing through your baby’s head.
  • Your baby has problems absorbing the fluid.
  • Your baby makes too much fluid. This happens in rare cases.

This condition can be congenital. This means that your baby is born with it. Hydrocephalus can also happen later in life. Causes of this condition include:

  • Congenital aqueductal stenosis (narrowing of the aqueduct)
  • Neural tube defects, such as spina bifida
  • Premature birth
  • Infections
  • Tumors
  • Bleeding inside your baby’s brain
  • Birth injuries
  • Blood vessels in your baby’s head that aren’t formed correctly
  • Head Injuries

Symptoms

Symptoms can occur a bit differently in each child. They may include:

  • A full or bulging soft spot on the top of your baby’s head (fontanel)
  • Increasing head size (circumference)
  • Seizures
  • Bulging eyes and not being able to look up when facing forward
  • Visible scalp veins
  • Irritability
  • High-pitched cry
  • Poor feeding
  • Projectile vomiting
  • Sleepiness or being less alert than usual
  • Developmental delays

The symptoms of hydrocephalus may seem like symptoms of other conditions, so it’s very important to make sure your child sees his or her healthcare provider for a diagnosis.

Diagnosis

A healthcare provider may first spot this condition in your baby during an ultrasound in pregnancy. In many cases, hydrocephalus doesn't develop until the third trimester of the pregnancy. Ultrasounds done earlier in pregnancy may not show this condition.  

Your child may be diagnosed with this condition after birth. Your child’s healthcare provider will examine your child and ask you about your child’s prenatal, birth, and family history. If your baby is older, your child’s provider may ask if he or she is meeting milestones. Children with this condition may be likely to have developmental delays. If your child has a delay, his or her healthcare provider may check for underlying problems.

Your baby’s head may be larger than normal. Your child’s healthcare provider will measure his or her head. If your baby’s head size isn’t in the normal range, he or she will have tests such as:

Ultrasound

This test uses sound waves to create an image of the inside of the body. During pregnancy, this test can show the size of the ventricles inside of your baby’s head.  After birth, ultrasounds can be used to see inside the head as long as the soft spot is open.

MRI

This test uses large magnets, radio frequencies (like those coming from a radio antenna), and a computer. Together, these show detailed images of organs and structures inside your baby’s body. This test does not involve radiation like x-rays.  It is very good for looking at brain and fluid.

CT scan

This test uses X-rays and computer technology to make detailed images of any part of your baby’s body. It is very good for looking at bones, and also shows muscles, fat, and organs. CT scans are more detailed than X-rays.

Treatment

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.

The goal of treatment is to reduce the pressure inside your baby's head. This can be done by draining the fluid. Your child may need medicine to remove the extra fluid. Some children need surgery.

In surgery, a doctor usually places device called a shunt in your baby’s head. There are three parts to this system: a tube that goes inside the head to the ventricle, a valve that sits underneath the skin on the outside of the skull, and a tube that runs down to another part of your baby’s body, where it can be absorbed.

The shunt usually runs behind your baby’s ear. The tubing goes under your baby’s skin to the belly (abdomen), heart, or lung. Your baby's healthcare provider will decide the drainage location. This will be based on your baby’s condition, age, and other factors. The belly is generally the first choice. A VP (ventriculoperitoneal) shunt is the most common system used to direct fluid into the abdomen.

Shunt complications

Sometimes surgery and shunts can cause problems. Possible complications include:

  • Infection
  • Bleeding
  • Malfunction (may drain too much or not enough fluid) or breakage

After surgery, your child’s healthcare team will tell you how to care for your baby at home. They’ll also tell you symptoms that are an emergency. If your child has these symptoms, call his or her healthcare provider right away.

Research

As mentioned above, hydrocephalus is typically treated by surgical placement of a shunt. However, they do experience high failure rates due to mechanical malfunctions, clogging, infection and over-draining; greater than 50% of such shunts fail within the first 2 years. For this reason, the doctors at Phoenix Children's Hospital are now using a scope technique for some babies called endoscopic third ventriculostomy with choroid plexus coagulation (ETV+CPC).  This procedure can prevent the need for shunts in some babies.

At BARROW Neurological Institute at Phoenix Children's Hospital, we are committed to providing excellence in research. Collaborations and strategic partnerships enable us to provide children with access to the best care, diagnostic tools and treatments available.

So, knowing the possible complications from shunts, our research team is collaborating with engineers at Arizona State University to develop a novel microvalve with a passive and simple design/implantation that may result in lower failure rates. While the research is still very early, these microvalves have the potential to open the door to a new standard in hydrocephalus treatment.

Prevention

Your baby’s healthcare provider may suggest genetic counseling if you plan to have more children. Through counseling, you can learn about the risk for this condition in future pregnancies. You may also need testing during pregnancy to check for hydrocephalus.

How can I help my child live with hydrocephalus?

Hydrocephalus can affect your baby’s brain and development. Your child’s outlook depends on how severe his or her condition is. It also depends on other brain and health problems your baby has.  

The key to treating this condition is getting it diagnosed and treated early, and avoiding infections. Your baby will need regular checkups to make sure his or her shunt is working right. Your child’s healthcare team will work closely with you as your baby grows.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

  • Know the reason for the visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
  • Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
  • Ask if your child’s condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if your child does not take the medicine or have the test or procedure.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.

The Walk

We are sponsoring the Hydrocephalus Association’s WALK to End Hydrocephalus on November 4 at 8:30AM at the Scottsdale Sports Complex. The walk helps to raise money for research, education, advocacy and community support networks. It’s also the organization’s largest annual fundraising event, raising nearly $1.6 million last year thanks to more than 12,000 participants in 38 locations across the U.S. You can register for the walk at: http://support.hydroassoc.org/site/TR?fr_id=1111&pg=entry. We hope to see you there!

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