Hydrocephalus: Types, Causes, Symptoms & Treatment

Hydrocephalus is the excessive accumulation of cerebrospinal fluid (CSF) within your brain cavities called ventricles. This excess fluid causes your ventricles to widen, which puts harmful press on the tissues of your brain.

Cerebrospinal fluid usually flows through the ventricles and bathes the brain and spinal column. But the pressure of too much cerebrospinal fluid can damage brain tissues and cause a range of symptoms related to brain function.

Hydrocephalus mainly occurs in children and adults aged over 60 years, but younger adults can get it too. The National Institute of Neurological Disorders and Stroke estimates that 1 to 2 of every 1,000 babies is born with hydrocephalus

Types of hydrocephalus

The four main types of hydrocephalus are communicating hydrocephalus, non-communicating hydrocephalus, normal pressure hydrocephalus and hydrocephalus ex-vacuo.

• Communicating hydrocephalus. Occurs when the flow of CSF is blocked after it leaves your ventricles. This type of hydrocephalus may result from a thickening of membranes at the base of your brain called arachnoid. This blockage prevents the free flow of CSF. This type of hydrocephalus is called communicating because the CSF can still flow between your ventricles, which remain open.
• Non-communicating hydrocephalus. This is also known as obstructive hydrocephalus. Non-communicating hydrocephalus occurs when the flow of CSF is blocked along one or more of the narrow passages connecting your ventricles.
• Normal pressure hydrocephalus (NPH). Happens when CSF buildup causes your ventricles to enlarge, but there’s little to no increase in pressure. The difference between NPH and other types of hydrocephalus is that even though there’s a larger than normal amount of CSF, the pressure inside of your ventricles remains the same. The buildup of CSF in your ventricles happens slowly and symptoms occur over time. NPH happens most often among the elderly.
• Hydrocephalus ex-vacuo. This results from brain damage caused by head injury or stroke. In these cases, brain tissue around your ventricles shrinks. CSF builds up in your ventricles to fill in the extra space. Your ventricles are enlarged, but pressure in your head usually remains normal.

Another word you may see or hear when learning about hydrocephalus is ventriculomegaly. Ventriculomegaly is the term used when the ventricles of a fetus’s brain are enlarged. Hydrocephalus may be the cause of the ventriculomegaly, but there are other reasons this enlargement might happen.

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Symptoms of hydrocephalus

Hydrocephalus can cause permanent brain damage, so it’s important to recognize the symptoms of this condition and seek medical attention. It’s most common in children but can develop in people of any age.

Symptoms in infants

Early signs of hydrocephalus in infants include:

• bulging fontanel, which is the soft spot on the surface of the skull
• a rapid increase in head circumference
• eyes that are fixed downward
• seizures
• extreme fussiness
• vomiting
• excessive sleepiness
• poor feeding
• low muscle tone and strength

Symptoms in toddlers and older children

Symptoms or signs that affect toddlers and older children include:

• short, high-pitched cries
• personality changes
• changes in facial structure
• crossed eyes
• headaches
• muscle spasms
• delayed growth
• trouble eating
• extreme sleepiness
• irritability
• loss of coordination
• loss of bladder control
• larger-than-average head
• trouble staying awake or waking up
• vomiting or nausea
• seizures
• problems concentrating

Symptoms in young and middle-aged adults

Symptoms in young and middle-aged adults include:

• chronic headaches
• loss of coordination
• difficulty walking
• bladder problems
• vision problems
• poor memory
• difficulty concentrating

Normal pressure hydrocephalus (NPH)

This form of the condition usually begins slowly and is more common in adults over the age of 60. One of the earliest signs is falling suddenly without losing consciousness. Other common symptoms of normal pressure hydrocephalus (NPH) include:

• changes in the way you walk
• impaired mental functions, including memory problems
• trouble controlling urine

Causes of hydrocephalus

Hydrocephalus can develop for a number of reasons. Hydrocephalus may be congenital or acquired.

Congenital hydrocephalus. A combination of genetic and environmental factors during fetal development causes congenital hydrocephalus. “Congenital” means present at birth.

The most common causes of congenital hydrocephalus are:

• Spina bifida and other brain and spinal cord (neural tube) defects.
• A narrowing of the small passage between the third and fourth ventricles of your brain (aqueductal stenosis).
• Complications of premature birth, such as bleeding within ventricles.
• Infections during pregnancy, such as rubella, that can cause inflammation in fetal brain tissue.

Acquired hydrocephalus develops at any point after birth and can affect people of all ages.

The most common causes of acquired hydrocephalus are:

• Head trauma.
• Stroke
• Brain or spinal cord tumors.
• Meningitis or other infections of your brain or spinal cord.

In addition, hemorrhage or complications of surgery may cause normal pressure hydrocephalus. Many people develop NPH without an obvious cause.

Too much cerebrospinal fluid in the ventricles can occur for one of the following reasons:

• Obstruction. Partial blockage of the flow of cerebrospinal fluid is the most common cause of too much cerebrospinal fluid in the ventricles. A blockage may happen from one ventricle to another or from the ventricles to other spaces around the brain.
• Poor absorption. Less common is a problem with absorbing cerebrospinal fluid. This is often related to inflammation of brain tissues from disease or injury.
• Overproduction. Rarely, cerebrospinal fluid is created more quickly than it can be absorbed.

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Risk factors of hydrocephalus

Sometimes, the cause of hydrocephalus is not known. However, developmental or medical problems can contribute to or trigger hydrocephalus.

Hydrocephalus may be present at or before birth, known as congenital hydrocephalus. Or it can occur shortly after birth. Any of the following incidents may cause hydrocephalus in newborns:

• The central nervous system developed in a way that blocks the flow of cerebrospinal fluid.
• Bleeding occurred within the ventricles. This is a possible complication of premature birth.
• There was an infection in the uterus during pregnancy, such as rubella or syphilis. An infection can cause swelling in the brain tissues of an unborn baby.

Other factors that can contribute to hydrocephalus among any age group include:

• Tumors of the brain or spinal cord.
• Central nervous system infections, such as bacterial meningitis or mumps.
• Bleeding in the brain from a stroke or head injury.
• Other traumatic injury to the brain.

Complications of hydrocephalus

In most cases, hydrocephalus worsens. Without treatment, it can leads to complications such as:

• Learning disabilities
• Developmental and physical disabilities
• Death

When hydrocephalus is mild and it’s treated, there may be few, if any, serious complications.

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Prevention of hydrocephalus

You can’t prevent hydrocephalus, but you can lower your risk and your child’s risk for developing the condition in the following ways:

• Prenatal care. Make sure you get prenatal care during pregnancy. This can help reduce your chance of going into premature labor, which can lead to hydrocephalus.
• Vaccinations. Getting vaccinations can help prevent illnesses and infections that are linked to hydrocephalus. Having regular screenings can also ensure that you get prompt treatment for illnesses or infections that could increase your risk of hydrocephalus.
• Safety equipment. Use safety equipment, like helmets, to prevent head injuries when doing activities like riding a bike. You can also lower your risk of head injuries by always wearing a seat belt.
• Buying safe gear for younger children. It’s essential to secure young children in a car seat. You can also prevent head injuries by making sure your baby equipment, like strollers, meets safety standards.

By taking steps to hydrocephalus, you can reduce your risk for permanent disability, as well as that of your child.

Diagnosis of hydrocephalus

A diagnosis of hydrocephalus is usually based on:

• Your symptoms.
• A general physical exam.
• A neurological exam.
• Brain-imaging tests.

Hydrocephalus is diagnosed through a neurological evaluation and imaging techniques. These includes:

• Neurological exam. The type of neurological exam depends on a person’s age. A health care professional might ask questions and conduct simple tests to judge muscle condition, movement, well-being and function of sensory abilities.
• Ultrasound scan. Your doctor may also use an ultrasound to get a closer look at the brain. These tests use high-frequency sound waves to create images of the brain. This type of ultrasound can only be done in babies whose fontanel (soft spot) is still open.
• Magnetic resonance imaging (MRI) scans. These can indicate signs of excess CSF. MRIs use a magnetic field and radio waves to make a cross-sectional image of the brain.
• Computerized tomography (CT) scans. These can also help diagnose hydrocephalus in children and adults. CT scans use several different X-rays to form a cross-sectional image of the brain. These scans can show enlarged brain ventricles that result from too much CSF.

Other tests are often performed in adults to diagnose the condition. These tests may include:

• Spinal tap (lumbar puncture).
• Intracranial pressure monitoring (ICP), which uses a small pressure monitor inserted into your brain to measure pressure.
• Fundoscopic exam, which uses a special device to view your optic nerve at the back of your eye.

Treatment of hydrocephalus

Hydrocephalus can be fatal without treatment. Interventions may not reverse all brain damage that’s already occurred, but its goal is to restore the flow of CSF and prevent further brain damage. Your doctor may explore either of the following surgical options.

• Shunt. The most common treatment for hydrocephalus is the surgical insertion of a drainage system, called a shunt. It consists of a long, flexible tube with a valve that keeps fluid from the brain flowing in the right direction and at the proper rate. One end of the tubing is usually placed in one of the brain’s ventricles. The tubing is then tunneled under the skin to another part of the body such as the stomach or a heart chamber. This allows excess fluid to be more easily absorbed. People who have hydrocephalus usually need a shunt system for the rest of their lives. They require regular monitoring.
• Endoscopic third ventriculostomy (ETV). With this surgery, a small hole is made in the floor of your third ventricle. This creates a pathway for CSF to flow in and around your brain as it would normally. This procedure is generally performed in children over the age of 2.

Other treatments options

Some people with hydrocephalus, particularly children, might need supportive therapies. Need for these therapies depends on the long-term complications of hydrocephalus. Children’s care teams might include:

• Pediatrician or physiatrist, who oversees the treatment plan and medical care.
• Pediatric neurologist, who specializes in diagnosing and treating neurological conditions in children.
• Occupational therapist, who specializes in therapy to develop everyday skills.
• Developmental therapist, who specializes in therapy to help your child develop age-appropriate behaviors, social skills and interpersonal skills.
• Mental health professional, such as a psychologist or psychiatrist.
• Social worker, who helps the family get needed services and plan for transitions in care.

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Possible complications after treatments

Many people go decades without complications, but things can change quickly. People with shunts must get regular medical checkups. Shunts can break, fail or become infected. If this happens, another brain surgery is required. An ETV can close at any time and put a person in danger.

You should seek help from your healthcare provider if symptoms of a shunt failure or EVT closure develop. These symptoms may include those similar with hydrocephalus, such as:

• Headache.
• Vision problems.
• Nausea or vomiting.
• Feeling tired.
• Soreness of your neck or shoulder muscles.
• Seizures.
• Redness or tenderness along the shunt area.
• Low fever.

Children who are in school may need special education. Special education teachers address learning disabilities, determine educational needs and help find needed resources.

Adults with more serious complications might need the services of occupational therapists or social workers. Or they may need to see specialists in dementia care or other medical specialists.

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