Education and Support

When confronted with a chronic medical condition it is not uncommon to feel helpless and
overwhelmed. Please know that vital resources are available to you. The Association publishes
educational materials and provides one-on-one support through phone and email to help you and
your family to understand and manage this aspect of your lives.

Hydrocephalus is a chronic neurological condition characterized by an increased volume of

cerebrospinal fluid (CSF) within spaces inside the brain. While there's no known cure for
hydrocephalus, the future holds promise. Recent research gives great hope that we may one day
find a cure. In the meantime, gradual advances in technology as well as diagnostic and treatment
protocols are helping more and more people with hydrocephalus to lead full and active lives.

Although hydrocephalus (hi-dro-sef-a-lus) is not yet a household word, it is NOT uncommon and
you are not alone. Over a million people of all ages currently live with hydrocephalus in the
United States, though it occurs all over the world. Hydrocephalus knows no socioeconomic,
gender or ethnic boundaries. Of every 1,000 babies born in this country, one or two will have
hydrocephalus. It is the most common reason for brain surgery in children, but it can also be
diagnosed in any decade of life. Experts estimate that hundreds of thousands of older adults
have normal pressure hydrocephalus (NPH), which often goes undetected or is misdiagnosed as
untreatable dementia, Alzheimer’s or Parkinson’s.

What You Can Do: The Power of Knowledge and

Community

Often the most important thing people living with hydrocephalus can do is become informed.
You may feel resistant to learning more about hydrocephalus — afraid of what you may discover
and upset that this condition has touched your life — but it is important to recognize that
knowledge is power and to persevere through that resistance. What causes hydrocephalus? What
are its symptoms? How is it treated? Who can help? Knowledge of the causes and symptoms of
hydrocephalus will give you confidence and peace of mind. Knowledge of treatment options and
medical terminology will help you make informed decisions and better manage your health care
team.

The Hydrocephalus Association empowers people with such knowledge, helping them find the
medical care, programs and resources that will work best for them, now and in the future. Please
take advantage of all the resources we offer, from personal support, to educational materials, to
tools and knowledge to access to high-quality medical care.

The Hydrocephalus Association also empowers people by creating a community of individuals,

families and medical professionals — all focused on providing support and medical care for
people with hydrocephalus. Community is essential to physical and emotional well-being: you
are not alone. We encourage you to explore our resources and become informed and connected.
Two good places to start:
What research is being done for hydrocephalus?

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the
National Institutes of Health (NIH) conduct research related to hydrocephalus in laboratories and
clinics at the NIH and support additional research through grants to major medical institutions
across the country. Much of this research focuses on finding better ways to prevent, treat, and
ultimately cure disorders such as hydrocephalus. The NINDS also conducts and supports a wide
range of fundamental studies that explore the complex mechanisms of normal and abnormal
brain development.

Where can I get more information about hydrocephalus?

For more information on neurological disorders or research programs funded by the National
Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and
Information Network (BRAIN) at:

I was able to the client a couple of sites she could visit!

BRAIN
P.O. Box 5801
Bethesda, MD 20824
(800) 352-9424
http://www.ninds.nih.gov

Information also is available from the following organizations:

Guardians of Hydrocephalus Research Foundation

2618 Avenue Z
Brooklyn, NY 11235-2023
GHRF2618@aol.com
http://ghrf.Homestead.com/ghrf.html
Tel: 718-743-GHRF (4473)
Fax: 718-743-1171

Hydrocephalus Association
870 Market Street
Suite 705
San Francisco, CA 94102
info@hydroassoc.org
http://www.hydroassoc.org
Tel: 415-732-7040 888-598-3789
Fax: 415-732-7044
Hydrocephalus Support Group, Inc.
P.O. Box 4236
Chesterfield, MO 63006-4236
hydrodb@earthlink.net
Tel: 636-532-8228
Fax: 314-251-5871

National Hydrocephalus Foundation

12413 Centralia Road
Lakewood, CA 90715-1623
debbifields@nhfonline.org
http://nhfonline.org
Tel: 562-924-6666 888-857-3434
Fax: 562-924-6666

SOURCE: National Institute of Neurological Disorders and Stroke, National Institutes of Health

Last Editorial Review: 5/8/2008

What is Hydrocephalus? A. Hydrocephalus comes from Greek words: hydro means

water, cephalus means head. Hydrocephalus is an abnormal accumulation of
fluid—cerebrospinal fluid, or CSF—within cavities called ventricles inside the
brain.

http://www.hydroassoc.org/education_support/learning/diagnosis/retrieved
november 8th

http://www.hydroassoc.org/docs/PrenatalHydrocephalus-A_Book_for_Parents.pdf

Diagnosis
If you think that you or someone you care about may have hydrocephalus, we recommend you
learn about the symptoms and diagnostic tests for this condition. We also encourage you to trust
your instincts. If you notice changes or have an intuitive feeling that something is not right with
the person you are concerned about, act on that feeling and seek medical attention.

Only a medical doctor can make a diagnosis of hydrocephalus. The information here is intended
to help you understand and navigate through the process of seeking medical advice. In children,
it is often a pediatrician who first suspects hydrocephalus. A pediatric neurosurgeon will
typically make the final diagnosis. In adults, hydrocephalus is often diagnosed by a neurologist
working with a neurosurgeon.
If you have already received a diagnosis, the information below will help you make sense of the
classifications and causes of hydrocephalus.

Symptoms and Diagnostic Tests

Symptoms of untreated hydrocephalus are variable. During pregnancy, routine ultrasound can
detect enlarged ventricles (spaces) within the baby’s brain. In children the symptoms tend to be
related to high pressure and may include nausea, vomiting, headache and vision problems. In
young and middle aged adults symptoms run the gamut of those seen in children and in older
adults and most often include dizziness and vision problems. In older adults with NPH the
symptoms are more likely to be loss of function in three main areas: walking, thinking and
bladder control.

Infants and Children Young & Middle Aged Adults Older Adults (NPH)
Abnormal Head Enlargement Difficulty Walking Difficulty Walking

Tense, Bulging Fontanel Cognitive Challenges Cognitive Challenges

Urinary Urgency or Urinary Urgency or

Prominent Scalp Veins
Incontinence Incontinence

Skull bones may feel

Chronic Headaches
seperated

Vomiting, Sleepiness,
Irritability

Headache, Nausea, Vomiting,

Vision

Downward Deviation of Eyes

The most common initial diagnostic test to determine hydrocephalus at any age is an image of
the brain (CT Scan or MRI) to identify the enlarged ventricles (spaces) within the brain that are
typical of hydrocephalus. More tests are often performed in adults. The following links take you
to detailed information about the signs, symptoms and diagnostic tests for hydrocephalus in
various age groups.

• PreNatal
• Infants, Children and Teenagers
• Adults Diagnosed in Young and Middle Age
• Older Adults with Normal Pressure Hydrocephalus (NPH)

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Classifications & Causes

Doctors often identify hydrocephalus in one of three ways. Congenital hydrocephalus results
from a complex interaction of genetic and environmental factors and is present at birth. Though
it might not be recognized and diagnosed immediately, congenital hydrocephalus is now often
diagnosed before birth through routine ultrasound. Hydrocephalus diagnosed in adulthood may
have existed since birth and can still be considered congenital and may be referred to as
compensated hydrocephalus. Acquired hydrocephalus develops after birth as a result of
neurological conditions such as head trauma, brain tumor, cyst, intraventricular hemorrhage or
infection of the central nervous system. If the cause of hydrocephalus is unknown, it is referred
to as idiopathic which is most commonly true of normal pressure hydrocephalus (NPH).

Another set of terms you may hear from a doctor to describe hydrocephalus are
“communicating” and “non-communicating”. If the cerebrospinal fluid (CSF) flows freely
throughout the brain’s ventricular system and the subarachnoid space, it is referred to as
communicating hydrocephalus. If there is a physical blockage such as a tumor somewhere in
that system, it is called non-communicating hydrocephalus.

Causes

The most common causes of congenital and acquired hydrocephalus are listed below.

Congenital Acquired

• Aqueductal stenosis • Intraventricular

• Neural tube defect hemorrhage
• Arachnoid cysts • Meningitis
• Dandy-Walker syndrome • Head injury

• Chiari malformation • Brain tumor

Aqueductal Stenosis
The most common cause of congenital hydrocephalus is an obstruction called aqueductal
stenosis. When the long, narrow passageway between the third and fourth ventricles (the
aqueduct of Sylvius) is narrowed or blocked, perhaps because of infection, hemorrhage, or a
tumor. Fluid accumulates "upstream" from the obstruction, producing hydrocephalus.

Back to Causes

Neural Tube Defect

Another common cause is a neural tube defect (NTD). An open NTD, where the spinal cord is
exposed at birth and is often leaking CSF, is called a myelomeningocele. This condition is often
referred to as spina bifida. This kind of NTD usually leads to the Chiari II malformation, which
causes part of the cerebellum and the fourth ventricle to push downward through the opening at
the base of the skull into the spinal cord area, blocking CSF's flow out of the fourth ventricle and
producing hydrocephalus. Please see the websites of the Spina Bifida Association and the
International Association for Spina Bifida and Hydrocephalus for more information on neural
tube defects.

Back to Causes

Arachnoid Cysts
Congenital hydrocephalus can also be caused by arachnoid cysts, which may occur anywhere in
the brain. In children, they're often located at the back of the brain (posterior fossa) and in the
area of the third ventricle. These cysts are filled with CSF and lined with the arachnoid
membrane (one of the three meningeal coverings). Some arachnoid cysts are self-contained,
while others are connected with the ventricles or the subarachnoid space. The fluid trapped by
the cysts may block the CSF pathways, producing hydrocephalus.

Back to Causes

Dandy-Walker Syndrome
In Dandy-Walker syndrome, another cause of congenital hydrocephalus, the fourth ventricle
becomes enlarged because its outlets are partly or completely closed, and part of the cerebellum
fails to develop. Dandy-Walker syndrome may also be associated with abnormal development in
other parts of the brain and sometimes leads to aqueductal stenosis. In some instances, two
shunts are placed in the child's ventricles — one in the lateral ventricle and another in the fourth
ventricle to manage the hydrocephalus. Please see the Dandy-Walker Alliance website for
further information.

Back to Causes

Chiari Malformation
There are two types of Chiari malformation. Both types occur in the bottom of the brain stem
where the brain and spinal cord join. The lowest portion of the brain is displaced and is lower
than normal pushing down into the spinal column. Chiari II is explained under Neural Tube
Defects. To learn more about Chiari I, please visit NeurosurgeryToday.org.

Back to Causes

Intraventricular Hemorrhage
An intraventricular hemorrhage, which most frequently affects premature newborns, may
cause an acquired form of hydrocephalus. When small blood vessels alongside the ventricular
lining rupture, blood may block or scar the ventricles or plug the arachnoid villi, which allow
CSF to be absorbed. When the CSF can't be absorbed, hydrocephalus results.

Back to Causes

Meningitis
Meningitis is an inflammation of the membranes of the brain and spinal cord. Caused by a
bacterial or (less frequently) viral infection, meningitis can scar the delicate membranes
(meninges) that line the CSF pathway. An acquired form of hydrocephalus may develop if this
scarring obstructs the flow of CSF as it passes through the narrow ventricles or over the surfaces
of the brain in the subarachnoid space.

Back to Causes

Head Injury
A head injury can damage the brain's tissues, nerves, or blood vessels. Blood from ruptured
vessels may enter the CSF pathway, causing inflammation. Sites of CSF absorption might then
be blocked by scarred membranes (meninges) or by blood cells. The CSF flow is restricted, and
hydrocephalus develops.

Back to Causes

Brain Tumors
In children, brain tumors most commonly occur in the back of the brain (posterior fossa). As a
tumor grows, it may fill or compress the fourth ventricle, blocking the flow of CSF and causing
hydrocephalus. A tumor somewhere else in the brain might also block or compress the
ventricular system. For more information, please see the National Brain Tumor Foundation
website.

Treatment of Hydrocephalus
While there is no known cure, there are two forms of surgical treatment currently used to manage
hydrocephalus. In the last 50 years, there have been incremental advancements in the diagnosis
and treatment of hydrocephalus, however, long term health outcomes for individuals with
hydrocephalus remain unpredictable. Often repeated neurosurgical operations are necessary to
treat hydrocephalus. And it is important to recognize when treatment is failing and to stay current
with doctors and developments even when things are going well. For further information on
treatment complications and what to be alert for, please click here.

The most common treatment for hydrocephalus—and the most common procedure performed by
pediatric neurosurgeons in the United States—is surgical implantation of a device called a shunt.
A flexible tube and valve system, a shunt drains cerebrospinal fluid from the brain to another
part of the body. For more information on shunting systems, click here.

Shunt Operation Statistics

• About 40,000 shunt-related operations are performed every year in the US for
hydrocephalus—that averages out to one every 13 minutes and over 100
every day.
• Shunt operations for hydrocephalus cost over $1 billion a year in the US.
• 50% of shunted individuals require a revising operation within two years.
A second treatment option is a surgical procedure called endoscopic third ventriculostomy
(ETV). An endoscope is used to puncture a membrane in the floor of the third ventricle creating
a pathway for CSF flow within the cavities in the brain. ETV is an important alternative to
shunting for obstructive hydrocephalus and may be useful in other cases as well. For more
information on ETVs click here.

Unlike many other operations in which the risks are highest during the surgical procedure, most
problems associated with shunting occur weeks or even years afterward. Shunt obstruction and
malfunction occur in approximately one third of children in the first year after a shunt-related
operation and studies have shown that ETVs can close up after time. We cannot overstate the
importance of learning the signs and symptoms of treatment failure. Staying in close contact with
neurosurgeons if you have a shunt or an ETV is critical. Please click here to read about serious
complications that you should be informed about.

Life-Threatening Complications
Hydrocephalus is almost always treated successfully with surgical placement of a shunt or an
ETV; but rarely does either treatment last a lifetime without complications. A critical aspect of
managing hydrocephalus is being well informed and staying vigilant about potential life-
threatening complications. Most problems associated with shunting or ETV occur weeks or even
years after the surgery. When things are going well, it is easy to forget about hydrocephalus and
having a shunt or an ETV. We encourage you to stay alert and informed. Feel free to call or
email the Hydrocephalus Association for more information about any of the complications listed
here.

Knowing what symptoms to watch for will help you become more at ease. Although the early
symptoms of shunt malfunction or infection in children—fever, vomiting and irritability—are
similar to many childhood illnesses, you will learn to determine the symptoms associated with
shunt failure in a particular individual. Adults tend to exhibit the symptoms they experienced
before treatment when there is a problem. If you have any doubt about symptoms, don’t hesitate
to contact a doctor. If you suspect there is a problem with the shunt, you are wise to have it
checked by the neurosurgeon rather than ignore it. It is better to have a false alarm checked than
to leave it unattended. Remember, although shunt complications can be very serious and become
life threatening, they can almost always be treated successfully when they are discovered early.

An estimated 50% of shunts fail within two years and 20-50% of ETVs close up within five
years. Either treatment can fail at any time. Infections are less common, but still not infrequent.
Be informed and vigilant. Be prepared to act quickly. Mere hours can mean the difference
between a resolvable complication and brain damage or even death, especially in children.

• Shunt Malfunction
• Shunt Infection
• Other Shunt Complications
• Important Related Conditions
 • Signs & Symptoms of Shunt Malfunction or Sudden Closure of ETV

• Signs & Symptoms of Shunt Infection

Shunt malfunction is usually a problem with a partial or complete blockage of the shunt. The
fluid backs up from the site of the obstruction and, if the blockage is not corrected, almost
always results in recurrent symptoms of hydrocephalus. Shunt obstruction can occur in any part
of the shunt. Most commonly in children, the ventricular catheter (the one in the brain) becomes
obstructed by tissue from the choroid plexus or ventricles. In adults it is more often the distal
catheter (the one that drains the fluid to another part of the body) that becomes blocked. The
catheters or the valve may become blocked with blood cells or bacteria. Shunts are very durable,
but the components of the shunt can become disengaged or fractured as a result of wear or as a
child grows, and occasionally they move from where they originally were placed. More rarely, a
valve will fail because of mechanical malfunction.

Shunt infection usually is caused by a person’s own bacterial organisms; it is not acquired from
exposure to other children or adults who are ill. The most common organism to produce infection
is Staphylococcus Epidermidis, which is normally found on the surface of the person's skin and
in the sweat glands and hair follicles deep within the skin. Infections of this type are most likely
to occur one to three months after surgery but may occur up to six months after the placement of
a shunt. People with ventriculo-peritoneal (VP) shunts are at risk of developing a shunt infection
secondary to abdominal infection, whereas people with ventriculo-atrial (VA) shunts may
develop generalized infection, which can quickly become serious. In either case, the shunt
infection must be treated immediately to avoid life-threatening illness or possible brain damage.

Other Shunt Complications may include the shunt system draining fluid at the wrong rate.
Overdrainage of the ventricles can cause the ventricle to decrease in size to the point where the
brain and its meninges pull away from the skull or the ventricles become like slits. If blood from
broken vessels in the meninges becomes trapped between the brain and skull, resulting in a
subdural hematoma, further surgery is required. This is most common in older adults with
normal pressure hydrocephalus (NPH). Slit-like ventricles, sometimes called slit-ventricle
syndrome (SVS), are most commonly a problem in young adults who have been shunted since
childhood. Underdrainage of the ventricles can fail to relieve the symptoms of hydrocephalus.
To restore a balanced flow of CSF it may be necessary to place a new shunt containing a more
appropriate pressure valve. For those who have externally adjustable or programmable valves,
the balance of flow can often be restored by re-setting the opening pressure.

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Slit Ventricles:
When the ventricles get too small, usually due to too much fluid drainage over time, they become
like slits. This only tends to happen in those shunted since early childhood and can manifest
either in childhood or young adulthood. Symptoms include severe intermittent headaches,
characteristically lasting 10-90 minutes that are often relieved when lying down, and smaller
than normal ventricles on imaging studies. Some doctors refer to this as slit ventricle syndrome
(SVS). Patients may be asymptomatic for prolonged periods. Most often the condition responds
to intervention and most shunt manufacturers have shunt hardware designed to address the
problem.

Multiloculated Hydrocephalus:
The term “multiloculated hydrocephalus” refers to the presence of an isolated CSF compartment
or compartments within the ventricular system that may become enlarged despite a functioning
shunt. It is most often caused by birth trauma, neonatal intraventricular hemorrhage, ventriculitis,
shunt related infection or overdrainage. Because the condition is usually in infants and children
who are already neurologically compromised, it can be difficult to recognize. Among several of
the operative treatments are multiple shunt placement, multiperforated ventricular catheters,
craniotomy and fenestration of intraventricular septations.

Seizures and Hydrocephalus:

Seizures are not an uncommon occurrence in people with hydrocephalus. However, no
correlations have been found between the number of shunt revisions or the site of shunt
placement and the risk of developing seizures. Past studies have shown:

• Children who have been shunted for hydrocephalus and who have significant
cognitive delay or motor disability are more likely to develop seizures than
those without cognitive or motor delays.
• Seizures are not likely to occur at the time of shunt malfunction.
• The most likely explanation for the development of seizure disorder is the
presence of associated malformations of the cerebral cortex.

Abdominal Complications:
The peritoneum (belly area) is the most popular site for the distal catheter implantation.
Although ventriculo- peritoneal (VP) shunts do not have fewer complications than ventriculo-
atrial shunts, the complications are less severe and associated with a lower mortality rate.
However the peritoneum is not immune to specific complications. Abdominal problems
represent a good number of VP shunt complications including peritoneal pseudocysts, lost distal
catheters, bowel perforations and hernias that require special attention.

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Signs & Symptoms of Shunt Malfunction or Sudden

Closure of ETV

Symptoms of shunt malfunction vary considerably from person to person, but tend to be similar
each time for a particular person. Often the symptoms that existed before treatment return when
something is going wrong.

In infants, signs include a full and tense fontanel (soft spot), bulging of the scalp veins and
swelling or redness along the shunt tract. Also watch for symptoms like unusual vomiting,
irritability, sleepiness and decreased interest in eating. Be aware that medication with a side
effect of drowsiness can mimic or mask signs of shunt malfunction and should be used with
caution in those with hydrocephalus, especially infants and young children.
When the child is older and the fontanel and other sutures between the skull bones are closed,
you no longer have the same warning signs. Children and adults may experience headaches,
vomiting, irritability and tiredness. Swelling along the shunt tract occurs less frequently. In the
event of an abrupt malfunction, a child may develop symptoms very rapidly, in a matter of hours
or days. Without treatment, coma, and even death, may result.

Older children and young adults may also become increasingly tired, have difficulty waking up
and staying awake, experience personality changes and, unless treated promptly, may go into a
coma.

People who were diagnosed and treated in adulthood, including those with NPH, tend to revert to
the symptoms they experienced before initial treatment during a malfunction.

Sudden closure of ETV:

While there are a number of reports that describe the benefits following ETV, there are others
that raise concerns that the pathway created for CSF flow can close up. It can be sudden and life-
threatening. It is critical that parents and patients understand that ETV is not a cure for
hydrocephalus. Be alert to signs and symptoms that may signal failure of the ETV, which are
similar to those described for shunt malfunction. Neurosurgeons should keep a watchful eye on
their ETV patients just as they do with their shunted patients.

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Signs & Symptoms of Shunt Infection

Shunt infection frequently results in fever and may happen with or without a shunt obstruction.
Sometimes shunt infection also produces reddening or swelling along the shunt tract. If an
infection is suspected it is critical to notify the neurosurgeon immediately or go to the
emergency room. A shunt infection must be treated immediately to avoid life-threatening illness
or possible brain damage.

Ongoing Management
Surgical treatment of hydrocephalus saves lives - but saving lives is not enough; it is important to
attend to the quality of those lives. We encourage you to focus on what you can do at each stage
of life to assure the highest possible quality of life for yourself or the person you care about who
has hydrocephalus. Empower yourself - explore the social, emotional, educational and advocacy
issues facing people with hydrocephalus and learn from those who’ve come before you.

Hydrocephalus can affect cognitive, social and physical development and abilities in different
ways in different people. For some of the challenges that may occur with hydrocephalus, and
suggestions on how to proactively manage them, we have created Fact Sheets. For a more in-
depth look at issues including headaches, vision problems, learning disabilities, education
strategies, hospitalization challenges, and other ongoing management issues, see our list of
downloadable Fact Sheets.
Brain Physiology
Hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF)
within cavities inside the brain called ventricles.

The brain and spinal cord form the central nervous system (CNS). They're surrounded and
protected by the bones of the skull and the vertebral column. Between the brain and skull are
three other protective coverings. These are the meninges — special membranes that completely
surround the brain and spinal cord. CSF flows between these membranes in an area called the
subarachnoid space. CSF cushions the brain and spinal cord against forceful blows, distributes
important substances and carries away some waste products.

CSF is believed to be primarily produced within the ventricles by delicate tufts of specialized
tissue called the choroid plexus. The ventricles can be thought of as chambers filled with fluid.
There are four ventricles in all: the two lateral ventricles, the third ventricle, and the fourth
ventricle. The ventricles are connected by narrow passageways.
Produced mainly in the lateral and third ventricles, CSF flows from the lateral ventricles through
two narrow passageways into the third ventricle. From the third ventricle, it flows down another
long passageway (the aqueduct of Sylvius) into the fourth ventricle. From the fourth ventricle, it
passes through three small openings (foramina) into the subarachnoid space surrounding the
brain and the spinal cord.

It has traditionally been thought that CSF is primarily absorbed through tiny, specialized cell
clusters (arachnoid villi) near the top and midline of the brain. CSF then passes through the
arachnoid villi into a large vein (the superior sagittal sinus) and is absorbed into the bloodstream.
Once in the bloodstream, it's carried away and filtered by the kidneys and liver in the same way
as other bodily fluids. Small amounts of CSF are also absorbed into lymphatic channels.

Our bodies produce approximately a pint of CSF every day, continuously replacing it as it's
absorbed. Under normal conditions, a delicate balance exists between the amount of CSF
produced and the rate at which it is absorbed. Recent research raises the possibility that the
rhythm of cerebral blood flow and CSF pulsations may have bearing on this delicate balance.