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Hydrocephalus

 

The multidisciplinary team at the Mischer Neuroscience Institute (MNI) has developed a comprehensive, coordinated program for the care of hydrocephalus patients. From specially trained neurosurgeons and neurologists to our experienced and compassionate nursing staff, our team is dedicated to providing exceptional care.

This guide has been provided to help you navigate the journey to optimal recovery for hydrocephalus. Along with learning about treatment options and expectations for hydrocephalus patients, you will find important resources, clinical details and helpful suggestions for rehabilitation.

What is Hydrocephalus?

Hydrocephalus is a condition that occurs when an abnormal amount of cerebrospinal fluid (CSF) accumulates in the brain. Cerebrospinal fluid is a clear, water-like fluid that is vital to protecting the brain and spinal cord. When there is too much of this fluid, it will pool in  the brain and cause increased pressure which leads to age-dependent symptoms. Because of the increased amount of fluid visible in imaging studies of the brain, a term often used to describe hydrocephalus is "water on the brain."

In addition to providing cushioning for the brain and spinal cord, CSF delivers important nutrients while also transporting waste which is eventually absorbed into the bloodstream. When the brain is functioning normally, CSF flows through channels called ventricles, circulates around the brain and spinal cord, and then is absorbed into the bloodstream via large veins called sinuses.

Hydrocephalus occurs when there is a blockage within the ventricles or the pathway back into the bloodstream. Rarely, it can also happen when the brain produces too much CSF.

If left untreated, hydrocephalus can lead to a myriad of health problems, including a loss in physical and mental abilities, brain damage and even death. Thankfully, the prognosis for patients can be very good with early diagnosis
and treatment.

Causes of Hydrocephalus

Congenital

Aqueductal stenosis is a narrowing or blockage in one of the passageways of CSF in the brain and can be caused by infection, hemorrhage or tumors.

Neural tube defects are birth defects of the brain and spinal cord. Spina bifida is the most common neural tube defect.

Arachnoid cysts are fluid-filled sacs that can occur anywhere in the brain.

Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum and the fluid-filled spaces around it. Chiari malformation is a brain abnormality that causes the cerebellum to protrude into the space normally occupied by the spinal cord.

Acquired

Intraventricular hemorrhage is bleeding in the ventricles, the major fluid-filled space of the brain.

Meningitis is an inflammation of the meninges (the membranes that cover the brain and spinal cord) and is typically caused by a virus or bacteria.

Head trauma occurs when an injury damages the brain’s tissue, nerves or blood vessels.

Brain tumor is any abnormal growth within the brain.

Types of Hydrocephalus

There are two types of hydrocephalus – Communicating and Noncomunicating.

Communicating hydrocephalus means that the flow of fluid is blocked after it exits the ventricles. It’s called “communicating” because the ventricles remain open and the CSF can still flow freely. This type of hydrocephalus is most often caused by a deficiency in the body’s ability to absorb CSF into the bloodstream after it circulates around the brain and spinal cord. In rare cases, communicating hydrocephalus may occur as a result of an overproduction of CSF.

Non-communicating hydrocephalus (also referred to as “obstructive” hydrocephalus) occurs when CSF cannot properly flow through the ventricles, generally indicating a blockage at a site within the ventricular system or between it and the subarachnoid space. The most common type of non-communicating hydrocephalus is aqueductal stenosis.

Hydrocephalus Signs and Symptoms

Symptoms of hydrocephalus vary depending on age and the progression of the condition.

Prenatal hydrocephalus is most often diagnosed between 20 and 24 weeks of gestation, when an ultrasound shows the abnormal dilation of the fetus’s ventricles and CSF pooling.

Infants and babies who are younger than one year may appear to have an abnormally shaped head that is unusually large. Because the bones in the skull of babies have not yet fused together, the skull may expand and protrude to accommodate the excess buildup of CSF. Other symptoms may include bulging at the “soft spots” and/or exaggerated swollen veins on the head, vomiting, sleepiness, irritability, a downward cast of the eyes and seizures.

Older children and adults often have different symptoms because their skulls have long since closed and are no longer able to expand to accommodate the buildup of CSF. Since their heads do not become enlarged, hydrocephalus is not as visibly recognizable. Symptoms in older children most often include the sudden onset of severe headaches accompanied by vomiting.

They may also exhibit loss of bladder control, irritability, sleepiness, seizures, and loss of balance, motor skills and memory. Visual difficulties such as decreased visual acuity, a downward cast of the eyes, double vision or repetitive eye movements may also be noted.

What to Expect Initial Evaluation

Your healthcare team may use a number of tools to detect or confirm the diagnosis of hydrocephalus, depending on your child’s age and symptoms. Generally, temperature, pulse and blood pressure will be measured. Blood tests may be necessary if surgery is being considered.

The physical examination may include measuring the size of the head, feeling the fontanelle (soft spot) of the head in babies, and assessing eye movements and motor function. Older children may also be tested for speech, coordination, attention and memory difficulties. An eye exam by an ophthalmologist may be ordered, as a careful exam of the eyes may provide evidence of increased pressure in the brain.

Common Diagnostic Tests for Hydrocephalus

While the physical examination provides your child’s physician with much needed information, additional tests may be necessary to confirm the diagnosis as well as the degree of hydrocephalus. These diagnostic tests provide more detailed information regarding treatment. The following tests are available 24 hours a day and on weekends. Some common tests include:

Ultrasound

An ultrasound is an imaging test that uses sound waves to detect structures within the body and then translates those sound waves into pictures. This test measures the size of the ventricles of infants whose soft spots have not yet fused. During the test, the technician slowly moves a probe around the outside of the infant’s head. Ultrasound is also commonly used to take images of a fetus inside a mother’s womb and abdomen. It generally takes around 15 minutes, requires no sedation or radiation exposure, and is painless.

Computed Tomography (CT Scan)

A CT, or CAT scan uses a computer system to give detailed, cross-sectional views of brain tissue for physicians to analyze. During the test, the child lies on a table with his or her head in a large, donut-shaped machine that takes pictures of the brain. Occasionally, contrast dye may be injected into the body. Contrast helps certain areas of the brain show up better on X-rays. A CT scan generally takes five minutes to complete and is painless.

Magnetic Resonance Imaging (MRI)

An MRI is a test that uses a strong magnetic field and radio waves to give physicians a 3-D picture of the brain. An MRI can be used for a more in-depth look of the brain than other imaging methods. During an MRI, which is painless, the child may require sedation because he or she must lie completely still within the MRI scanner for approximately 45 minutes or longer depending upon the specific sequences ordered by your physician. In order to obtain the best quality image, some patients, especially young children, may require general anesthesia. It is important to be aware that some types of shunts must be reprogrammed after the MRI scan and before the child leaves the hospital.

Quick Brain MRI

A Quick Brain MRI, or Fast Brain MRI, is an abbreviated MRI in which only a handful of images of the brain are taken. It can be performed within 5 minutes, requires no sedation or radiation exposure, and is painless. Children’s Memorial Hermann Hospital is one of the only facilities in the region with the capability and equipment to perform this test. It was created for children who require multiple scans during childhood with the goal of reducing overall radiation exposure. In some cases, a standard MRI may still be necessary.

Shunt Series

A Shunt Series is a set of X-rays (skull, chest and abdomen) that can reveal a break or disconnect in the shunt tubing and is painless.

Advanced Treatment of Hydrocephalus

Shunt Surgically implanting a shunt is the most common treatment for hydrocephalus. A shunt is a device that contains a flexible tube and valve system that drains CSF from the brain into another part of the body such as the abdomen or chest cavity where it can be absorbed into the bloodstream.

Shunting can be a highly effective treatment option. However, shunt malfunctions may occur after placement for a variety of reasons. Most children with shunts will have to undergo one or more subsequent surgeries when the shunt malfunctions. Shunts can also become infected, most commonly within the first three months after placement. Strict protocols have been implemented to minimize the risk of shunt infection.

There are three basic components of a shunt:

  • A catheter (or tube) that is inserted into the brain ventricles
  • A valve that regulates the flow of CSF
  • A longer catheter that carries the CSF to another part of the body

Endoscopic Third Ventriculostomy (ETV)

Some children may be candidates for a minimally invasive surgical procedure called ETV. The best candidates have non-communicating (obstructive) hydrocephalus and most commonly have an obstruction preventing fluid from passing between the third and fourth ventricles of the brain.

An ETV creates an alternate channel for the flow of CSF and is an alternative to shunting. During this procedure, the neurosurgeon uses a very small camera and tiny surgical tools to create an opening which provides an alternative route for CSF and bypasses the fluid obstruction. The surgery takes approximately 30 minutes and most children recover quickly with no additional surgical intervention required. In some cases, the opening will close and the procedure must be performed again or a shunt may be required.

Other Endoscopic Treatments

Our expert team of affiliated physicians is nationally recognized for their innovative endoscopic treatments for pediatric hydrocephalus that go beyond ETV. Pediatric neurosurgeons at Mischer Neuroscience Institute and Children’s Memorial Hermann Hospital are among only a handful of physicians in the country who are extensively experienced in using minimally-invasive techniques with every possible case, including the following:

  • Septostomy
  • Aqueductoplasty
  • Choroid plexus coagulation
  • Cyst fenestration

Septostomy is a procedure in which the surgeon makes a tiny incision in the septum pellucidium, which is the membrane that separates the two lateral ventricles. This procedure may be performed in place of a shunt in some cases or may be utilized in place of adding a second shunt for children with non-communicating ventricles.

Aqueductoplasty is a procedure to expand the opening of a narrowed aqueduct of Sylvius, the channel which connects the third and fourth ventricles of the brain. The most common cause of congenital hydrocephalus is aqueductal stenosis. This occurs when the passageway between the third and fourth ventricles — the aqueduct of Sylvius - is narrowed or blocked. When a child has an isolated fourth ventricle, CSF becomes trapped and creates pressure on the brain stem. In these cases, an aqueductoplasty may be performed to enable communication between the third and fourth ventricles of the brain.

Choroid plexus coagulation is a groundbreaking technique that is performed to decrease the production of CSF. The surgeon places a small cautery device into the endoscope to obliterate the choroid plexus, the substance in the brain which produces the majority of CSF.

Cyst fenestration may be performed in children with arachnoid cysts or other types of cysts. This endoscopic procedure also eliminates the need for a shunt, as the surgeon makes tiny incisions in the cyst walls to create channels for CSF flow.

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