What is Hydrocephalus?

If your doctor tells you your baby or young child has hydrocephalus, you probably have a lot of questions.

Hydrocephalus is not a disease. Rather, it’s a condition that happens when your child has too much fluid in the brain.

Hydrocephalus is a serious condition. But with the right diagnosis and treatment, your child has a good chance of leading a full life.

Here’s what you need to know about hydrocephalus.

What Is Hydrocephalus?

Hydrocephalus is a condition where extra cerebrospinal fluid (CSF) builds up in the cavities of the brain. People used to call hydrocephalus “water on the brain” but that’s not an accurate description.

CSF isn’t water. It’s an organic liquid that absorbs shocks and delivers nutrients to the brain. It regulates changes in pressure between the cranium and spine.

Excess CSF puts pressure on the brain. That pressure can harm brain tissue and keep the brain from working right. Left untreated, it can cause brain damage and even death.

Hydrocephalus is most common in infants and people over 60. The National Institute of Neurological Disorders and Stroke estimates it affects one to two in every 1,000 children born in the U.S. It’s the most common reason for children to have brain surgery.

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What Causes Hydrocephalus?

You can be born with hydrocephalus. You can also get it later in childhood as the result of an accident or injury. Older adults may get it after a stroke or other brain trauma.

There are two types of hydrocephalus. They have different causes.

Congenital hydrocephalus

This condition is present at birth. It is the result of some other problem with the nervous system, such as:

  • Genetic abnormalities such as Chiari malformation.
  • Birth defects in the brain, spine, or spinal cord, such as spina bifida.
  • Complications of premature birth, such as bleeding in the brain.
  • An infection during pregnancy, such as mumps or rubella.

Acquired hydrocephalus

This type of hydrocephalus can happen in childhood or adulthood. It develops over time because of injury or disease. Causes include:

  • Brain or spinal cord tumors.
  • Bacterial meningitis or other infections of the central nervous system.
  • Stroke.
  • An injury that causes bleeding in the brain.

Hydrocephalus Symptoms

Hydrocephalus symptoms in babies include:

  • An unusually large head.
  • A rapid increase in head size.
  • A fontanel (the soft spot on top of a baby’s head) that bulges outward.
  • Fussiness.
  • Being unusually tired.
  • A poor appetite.
  • Vomiting.
  • Eyes that look downward all the time (doctors call this “sun setting”).
  • Eyes that can’t turn outward.
  • Slowed development.
  • Seizures.

In older children, symptoms may include:

  • Headache.
  • Blurred or double vision.
  • Slow development.
  • Vision problems.
  • Nausea and vomiting.
  • Balance problems.
  • Decline in school performance.
  • Loss of bladder control.
  • Coordination problems.
  • Having a hard time waking up.
  • Changes in personality.
  • Memory loss.
  • Irritability.

Complications of Hydrocephalus

Some babies born with congenital hydrocephalus suffer permanent brain damage. They might have long-term complications, both physically and mentally. Those problems may include:

  • Learning disabilities.
  • Speech and vision problems.
  • Memory and organizational problems.
  • A short attention span.
  • Problems with physical coordination.
  • Epilepsy.

If your child has complications from hydrocephalus, it’s important to get extra support. Your child may benefit from physical and occupational therapy, or an early intervention program.

Diagnosing Hydrocephalus

Doctors diagnose hydrocephalus in several ways. The earlier doctors catch hydrocephalus, the better chance your child has of a successful plan of treatment. Here are some ways doctors diagnose hydrocephalus.

  • Prenatal ultrasound. Sometimes doctors catch early signs of hydrocephalus before birth. One sign is that the baby’s head begins to grow rapidly.
  • A detailed family history. Hydrocephalus can run in families, because some of the birth defects that cause it run in families.
  • A complete medical history. If your child is older, your doctor will want to know about any injuries or accidents. They will also want to know if your child had a recent bout with a disease that could have affected their brain.
  • A neurological exam. Depending on your child’s age, doctors may be able to test their muscle strength and reflexes. They can also test coordination, balance, vision, hearing, and mental functioning in older children.
  • Ultrasound. This is often the first test doctors use, because it is low-risk and simple.
  • Magnetic resonance imaging (MRI). This test can tell doctors if the brain cavities seem enlarged. It helps them see the CSF flow.
  • Computed tomography (CT). This test shows doctors if the cavities seem enlarged, and if there is something obstructing the CSF.
  • Lumbar puncture (spinal tap). Doctors can estimate CSF pressure and analyze spinal fluid from this test. They insert a needle in the lower back to remove and test some fluid.
  • Intracranial pressure monitoring (ICP). Doctors use a pressure monitor inserted into the brain or brain cavities. It measures the amount of swelling; if the pressure is too high, they may drain the CSF.
  • Fundoscopic exam. Doctors use a device to view the optic nerve. Swelling suggests abnormal pressure in the brain.

Hydrocephalus Treatment

Treatment for hydrocephalus may depend on your child’s age, and what caused the hydrocephalus.

Your child may also need therapy or other follow-up care and monitoring throughout their lifetime.

Surgical treatment

There are two main surgeries for hydrocephalus:

  • Ventriculoperitoneal (VP) shunt surgery. Doctors place a piece of tubing from the brain cavities to the peritoneal cavity (where the stomach and bowels are). The excess cerebrospinal fluid gets absorbed into the bloodstream.
  • Endoscopic third ventriculostomy (ETV) surgery. Doctors make a tiny hole in the bottom of the affected brain cavity. The CSF drains out of the brain and gets absorbed into the bloodstream. Sometimes an ablation of
    the part of the brain that makes CSF, called the choroid plexus, is performed
    in the same procedure.

As your child grows, they may need more surgeries and ongoing medical care.

Non-surgical treatment

After surgery, your child will need follow-up care. Children with hydrocephalus often have developmental delays. Your child may need:

  • Physical therapy.
  • Occupational therapy.
  • An early intervention program.

With continued medical care and therapy, your child has the best chance of living a full, active life.

Sources

National Institute of Neurological Disorders and Stroke, Hydrocephalus Fact Sheet, Link

American Association of Neurological Surgeons, Hydrocephalus, Link

NHS, Hydrocephalus, Link

National Library of Medicine, Hydrocephalus, Link

Hydrocephalus Association, Hydrocephalus in Infants and Children, Link

Kidshealth.org, Hydrocephalus, Link

About Pediatrics

From nutrition to illnesses, from athletics to school, children will face many challenges growing up. Parents often will make important health care decisions for them. We hope to help guide both of you in that journey. UPMC Children’s Hospital of Pittsburgh is a national leader in pediatric care, ranking consistently on U.S. News & World Report’s Best Children’s Hospitals Honor Roll. We provide expert treatment for pediatric diseases, along well-child visits, urgent care, and more. With locations across Pennsylvania, Maryland, and West Virginia, you can find world-class care close to home. We also work closely with UPMC Magee-Womens Hospital, a national leader in care for newborns and their mothers. Our goal is to provide the best care for your children, from birth to adulthood and beyond. Visit our website to find a doctor near you.