Children's Hospital of Orange County

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Hydrocephalus

We know that as parents you want the best possible care for your child, especially when they have a complex condition. CHOC is the region’s leader in treating complex multicystic hydrocephalus. Our neurosurgeons have pioneered two unique procedures to ensure the most cutting-edge treatment possible for kids with hydrocephalus. We are here for your child and your family through every step of hydrocephalus treatment and are committed to delivering care tailored to your needs.

Diagram showing a normal brain and a brain with hydrocephalus

What is hydrocephalus?

Hydrocephalus is a condition in which excessive fluid accumulates in and around the brain. It occurs from a lack of absorption, blockage of flow, or overproduction of the cerebral spinal fluid (CSF) that’s made inside the ventricles. The ventricles are fluid-filled areas of the brain. CSF is a clear, watery fluid that disperses from the ventricles to form a cushion around the brain and spinal cord and provide protection from injury. However, too much CSF may result in a buildup of fluid that can cause the pressure inside of the head to increase. In a child, this causes the bones of the skull to expand and separate to a larger-than-normal appearance.

Hydrocephalus symptoms

The following are the most common symptoms of hydrocephalus in babies. However, each baby may experience symptoms differently. Symptoms of hydrocephalus may include:

  • A full or bulging fontanel (soft spot located on the top of the head)
  • Increasing head circumference (size)
  • Seizures
  • Bulging eyes and an inability of the baby to look upward with the head facing forward
  • Prominent scalp veins
  • Increased irritability
  • High-pitched cry
  • Poor feeding
  • Projectile vomiting
  • Sleepiness or less alert than usual
  • Developmental delays

Hydrocephalus causes

The imbalance of CSF production and absorption that causes hydrocephalus in children can be due to any of the following:

  • Blockage. A blockage of the flow of cerebrospinal fluid is the most common problem that causes a CSF imbalance. The blockage can occur from one ventricle to another or from ventricles to other spaces in the brain.
  • Absorption Issue. The CSF imbalance can less commonly be due to problems absorbing cerebrospinal fluid. Absorption problems are usually a result of inflammation of brain tissue.
  • Overproduction. Even rarer, overproduction of CSF can cause the imbalance. In these cases, cerebrospinal fluid is produced more quickly than it is absorbed.

Hydrocephalus types

Types of hydrocephalus include congenital, acquired, normal-pressure and ex-vacuo. A person’s condition is classified into one of the four types based on how/when the condition develops. The two types of hydrocephalus that primarily occur in children are:

Congenital hydrocephalus: At birth

Hydrocephalus occurs in approximately one out of 500 births. In some babies the condition is genetic, such as in babies with congenital aqueductal stenosis. Other conditions, such as neural tube defects (like spina bifida), are also associated with hydrocephalus.

Acquired hydrocephalus: After birth

Acquired hydrocephalus occurs after the baby is born, usually as a result of injury or illness that affects the brain, such as other neurological conditions. Causes of acquired hydrocephalus in babies may include:

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

Hydrocephalus Diagnosis & Testing

Hydrocephalus might be diagnosed before birth by prenatal ultrasound. In many cases, though, hydrocephalus doesn’t develop until the third trimester of the pregnancy and may not be seen on ultrasounds performed earlier in pregnancy. The Fetal Care Center of Southern California can confirm a hydrocephalus diagnosis and our team of pediatric specialists will start treatment planning for your baby. Request a fetal care consultation today.

Some congenital hydrocephalus is not diagnosed until after birth using diagnostic procedures. Diagnostic tests that may be performed to confirm the diagnosis of hydrocephalus include:

  • Doctor examination.The doctor obtains a complete prenatal and birth history of the baby. They may ask about family history of hydrocephalus or other medical issues, as well as about developmental milestones in older babies, as hydrocephalus can be associated with developmental delay. Hydrocephalus can result in a larger than average head size, so they may also measure your baby’s head circumference.
  • Ultrasound. This diagnostic procedure uses sound waves to create an image of an internal portion of the body and can be used to determine the size of the ventricles in the womb and in neonates.
  • Magnetic resonance imaging (MRI). This diagnostic procedure uses a combination of large magnets, radio frequencies, and a computer to produce detailed images of organs and structures within the body.
  • Computed tomography (CT) scan. This diagnostic imaging procedure uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays.

Hydrocephalus Treatment and Surgery at CHOC

The goal of hydrocephalus treatment is to reduce the pressure in the baby’s head and to properly drain the excess build-up of cerebral spinal fluid (CSF). Occasionally, medications or procedures to draw off the extra CSF may be used.

Surgery may be needed for some cases of hydrocephalus. Surgery usually involves placing a mechanical shunting device into the baby’s head to help drain the extra CSF from the brain and redirect the extra fluid to another part of the body to be absorbed. A common type of shunt is the ventriculoperitoneal shunt. This directs the fluid into the abdominal cavity.

Hydrocephalus Shunt Treatment

Diagram showing the parts of a hydrocephalus shunt, including the shunt, the tube and the valve

The shunt consists of three parts:

  • A tube that’s placed inside the ventricular space
  • A reservoir and valve to control the flow of CSF
  • Tubing that’s directed under the skin to the abdomen, or less commonly to the heart or lung area

The shunt redirects the CSF out of the head through the tubing to a location elsewhere in the body where it can be absorbed. The shunt usually runs behind the ear and the tubing is tunneled under the skin to the area of the abdomen, heart or lung.

Potential complications from the shunts or surgery can include:

  • Infection
  • Shunt malfunction that results in underdrainage or overdrainage of the CSF
  • Bleeding

These symptoms require prompt medical evaluation. Following surgery, you’ll receive instructions on how to care for your baby at home and information about signs or symptoms requiring immediate medical attention.

What are the lifelong considerations for hydrocephalus?

Hydrocephalus can affect the brain and a baby’s development. The extent of the problem is dependent on the severity of the hydrocephalus, and the presence of brain or other organ system problems.

The key to treating hydrocephalus is early detection, proper treatment, and prevention of infection. A baby with hydrocephalus requires frequent medical evaluations to ensure proper shunt function. The medical team works closely with the family to provide education and guidance as the baby grows and develops.

Genetic counseling may be recommended by the baby’s doctor to discuss the risk for recurrence in future pregnancies, as well as prenatal testing for hydrocephalus.