What is Hydrocephalus?

Hydrocephalus, also known as "water on the brain," is a condition in which there is extra cerebrospinal fluid around the brain and spinal cord. Cerebrospinal fluid acts as a cushion for the brain and spinal cord, supplies nutrients, and takes away waste products. 

Hydrocephalus can be present at birth (congenital) or can develop later (acquired). 

• Congenital hydrocephalus is present at birth, although it may not be detected until later in life. It forms when the brain and surrounding structures develop abnormally. The exact cause is usually unknown, but contributing factors may include genetics and certain infections during pregnancy. 
• Acquired hydrocephalus results from injuries or illnesses that occur at birth or later, including infections in the brain and spinal column (meningitis), bleeding (hemorrhage) of blood vessels in the brain, severe head injury, brain tumors or cysts. Hydrocephalus also can occur when there is no known injury or illness to cause it. 

Hydrocephalus can be classified according to its cause: 

• Obstructive (non-communicating) hydrocephalus is caused by a blockage in the system of cavities (ventricles) in the brain. The blockage prevents the cerebrospinal fluid from flowing (or "communicating") with the area that surrounds the brain and spinal cord (subarachnoid space), as it normally should. Blockages can be present at birth or can occur later. One of the most common types is aqueductal stenosis, which occurs because of narrowing of the aqueduct of Sylvius, a channel that connects two of the ventricles together. 

• Non-obstructive (communicating) hydrocephalus results from problems with cerebrospinal fluid being produced or absorbed. One of the most common causes is bleeding into the subarachnoid space. Communicating hydrocephalus can be present at birth or can occur later.

Another type of hydrocephalus, called normal pressure hydrocephalus is an acquired type of communicating hydrocephalus in which the ventricles are enlarged but not under high pressure. Normal pressure hydrocephalus is seen in older adults. It may be the result of injury or illness, but in the majority of cases the cause is unknown. 

Another type is called hydrocephalus ex-vacuo. A stroke or traumatic injury causes damage to the brain, which may cause brain tissue to shrink. 

Premature infants born before 34 weeks or weighing less than 4 pounds have a higher risk of blood vessels bleeding in the brain. Severe bleeding can lead to acquired hydrocephalus, communicating or non-communicating, depending on the site and extent of the bleeding.

Symptoms

The most common symptoms of hydrocephalus vary depending on the age of onset. 

In infants, symptoms include: 

• A large head that is growing too rapidly 
• A bulging "soft spot" on top of head (anterior fontanelle) 
• Irritability 
• Seizures 
• Delays in development 

In older children and adults, symptoms include: 

• Headache 
• Difficulty walking 
• Loss of athletic abilities 
• Change in personality 
• Decrease in mental abilities 

At any age, symptoms include: 

• Vomiting
• Lethargy

Diagnosis

Congenital hydrocephalus may be diagnosed during a routine prenatal ultrasound, but often is discovered during infancy or early childhood. A doctor may suspect hydrocephalus before other symptoms appear if an infant has a head that seems to be getting larger too rapidly or is larger than would be expected given the baby's current weight or length. If the soft spot (anterior fontanelle) is still open, an ultrasound of the head can determine if the head is getting larger due to hydrocephalus. If the results of that ultrasound are abnormal, more evaluation is necessary. 

The doctor will ask questions about the person's medical history, and he or she will perform a physical and neurological examination. Detailed pictures of the brain may be obtained with computed tomography (CT) or magnetic resonance imaging (MRI). If these pictures reveal hydrocephalus or other problems, the child or adult usually is referred to a neurosurgeon for further evaluation and treatment. 

Expected Duration

Although the symptoms may subside with treatment, the duration varies according to the type and cause of the hydrocephalus. 

Prevention

A few of the causes of hydrocephalus can be prevented: 

• To help avoid severe head injury, wear appropriate protective gear when participating in contact sports or other activities, such as skiing, riding a bike and sledding, with a risk of head trauma. 
• If you are pregnant, visit your doctor as soon as you know you are pregnant and frequently throughout the pregnancy. Your doctor will screen you for infections and other problems that may occur during pregnancy. Regular visits also will reduce the risk of premature birth. Women of child-bearing age should have folate regularly in their diet before becoming pregnant to help prevent the development of neural tube defects, some with hydrocephalus. 

Treatment

Treatment of hydrocephalus depends on its cause and the severity. Occasionally, medications are used to slow the rate of cerebrospinal fluid production temporarily. However, this has not been successful for long-term treatment.  

Most cases require drainage of the excess fluid. A tube called a shunt is inserted into one of the cavities in the brain through a hole in the skull. The shunt is tunneled under the skin and carries the cerebrospinal fluid into another area of the body, such as the abdomen, where it can be absorbed.  

The shunt may need to be replaced periodically as a child grows or if an infection of it develops, or the shunt becomes blocked. 

Some people with obstructive hydrocephalus can be treated with a technique called ventriculostomy in which a neurosurgeon makes an incision at the site of the blockage to allow cerebrospinal fluid to drain. In some cases, surgeons may recommend surgical repair of congenital hydrocephalus while the fetus is still inside the mother's womb (uterus). 

Treatment options

The following list of medications are in some way related to or used in the treatment of this condition.

• acetazolamide

When To Call a Professional

Always make an appointment with a obstetrical provider if you become pregnant to arrange for prenatal care. Call your doctor if you or your child develops symptoms of hydrocephalus or has a severe head injury. 

Prognosis

The outlook depends on the cause of hydrocephalus and usually is better if the problem is diagnosed and treated early. If hydrocephalus gets worse rapidly or lasts a long time, it can cause brain damage and death. 

Children with hydrocephalus are more likely to have various developmental disabilities. Even after treatment, many children with hydrocephalus have lower intelligence, memory problems and visual problems, including strabismus (abnormally aligned eyes). Children with hydrocephalus also may enter puberty earlier than normal. 

With appropriate treatment, most infants with hydrocephalus survive and approximately half have normal intelligence.

Additional Info

National Institute of Neurological Disorders and Stroke
https://www.ninds.nih.gov/