Hydrocephalus FAQs

What is hydrocephalus?

Hydrocephalus is a condition in which fluid accumulates in the brain, typically in young children, enlarging the head and sometimes causing brain damage. 

How common is hydrocephalus?
Hydrocephalus occurs in two out of every 1,000 births in the United States. It is not known how many people develop it after birth. Approximately 125,000 persons are living with cerebrospinal fluid (CSF) shunts, and 33,000 shunts are placed annually in the United States.

What causes hydrocephalus?
Hydrocephalus can be present at birth or be acquired later in life as a result of a tumor, head injury, meningitis or hemorrhage.  Congenital hydrocephalus can occur in isolation or may be associated with other conditions such as spina bifida or Dandy Walker syndrome.

Which children are most at risk for hydrocephalus?
The most common causes of pediatric hydrocephalus in children in the United States are brain bleeds as a result of prematurity, spina bifida, brain tumors, infection and head injury. Acquired hydrocephalus is caused by head injury or an obstruction in the brain, such as a tumor.

What are the different types of hydrocephalus?
There are two main types of hydrocephalus. Non-communicating hydrocephalus is where the blockage of cerebrospinal fluid (CSF) is at the level of the fluid spaces of the brain. Communicating hydrocephalus where the blockage of CSF is at the surface of the brain.

Is hydrocephalus serious?
Hydrocephalus can be very serious, and even fatal, if left untreated. Fifty percent of those who fail to have their hydrocephalus treated will die. The other half survive with what is called arrested hydrocephalus. Those who are not treated and survive may have serious brain damage and physical disabilities. 

How does hydrocephalus affect children differently than adults?
In small children and infants, hydrocephalus can affect the head by increasing its size to accommodate the excess fluid buildup. Hydrocephalus may also slow growth in children as well have an impact on facial formation and eye spacing.

What are possible complications of hydrocephalus?
Hydrocephalus complications include headaches, hearing loss, muscle weakness, seizures an hormonal imbalances. Many of the complications, like nerve damage, seizure and impaired growth, arise from pressure placed on parts of the brain by the excess fluid. Other complications come from surgical treatments.

Why does congenital hydrocephalus run in families?
Congenital hydrocephalus can run in families. It is thought that congenital hydrocephalus can be caused by genetic defects that can be passed from one or both parents to a child, but the direct links to hereditary disorders are still being investigated. However, experts have found a connection between a rare genetic disorder called L1 syndrome and hydrocephalus. L1 syndrome is the result of mutated gene. It can cause a condition known as aqueductal stenosis, which involves an obstruction of the flow of cerebrospinal fluid (CSF), which is a fluid that is necessary for the normal functioning of the brain and spinal cord. This obstruction of the CSF can cause hydrocephalus. Researchers estimate that L1 syndrome occurs in one in 25,000 to 60,000 males and rarely occurs in females.

Is there a cure for hydrocephalus?
Hydrocephalus has no cure, but there are treatments that allow those affected to lead fairly normal lives with the condition. In some cases, it is possible for the blockage that is causing the fluid to build in the brain to be surgically removed. If the blockage is successfully removed, the child will require follow-up appointments to monitor his or her cerebrospinal fluid (CSF) levels and the possibility exists that they will need more surgeries later on in life.

What is the treatment for hydrocephalus?
The most common treatment for hydrocephalus is the surgical placement of a shunt in the area of the brain with excess fluid. A shunt consists of flexible tubing that drains the fluid into another area of the body that can properly absorb it. A more modern treatment of hydrocephalus uses a small camera to make an internal connection in the brain to allow the fluid to be absorbed without the need to place a shunt. This procedure is sometimes an excellent alternative treatment to a shunt.

How common are shunt revisions?
The typical child that receives a shunt requires about 2 to 5 operations over the course of their life to maintain it.  There are however some children who will require only a single operation while other children may have numerous shunt revisions, sometimes several in one year.

What is the life expectancy of a child who has hydrocephalus?
Children often have a full life span if hydrocephalus is caught early and treated. Infants who undergo surgical treatment to reduce the excess fluid in the brain and survive to age 1 will not have a shortened life expectancy due to hydrocephalus.