Hydrocephalus

U.S. DEPARTMENT OF HEALTH

AND HUMAN SERVICES
Public Health Service
National Institutes of Health

Hydrocephalus

What is hydrocephalus?

he term hydrocephalus is derived from

the Greek words hydro meaning water
and cephalus meaning head. As the name
implies, it is a condition in which the primary
characteristic is excessive accumulation of
fluid in the brain. Although hydrocephalus
was once known as water on the brain,
the water is actually cerebrospinal fluid
(CSF) a clear fluid that surrounds the brain
and spinal cord. The excessive accumulation
of CSF results in an abnormal widening
of spaces in the brain called ventricles.
This widening creates potentially harmful
pressure on the tissues of the brain.
The ventricular system is made up of four
ventricles connected by narrow passages.
Normally, CSF flows through the ventricles,
exits into cisterns (closed spaces that serve
as reservoirs) at the base of the brain, bathes
the surfaces of the brain and spinal cord,
and then reabsorbs into the bloodstream.
CSF has three important life-sustaining
functions: 1) to keep the brain tissue buoyant,
acting as a cushion or shock absorber;
2) to act as the vehicle for delivering
nutrients to the brain and removing waste;
and 3) to flow between the cranium and

spine and compensate for changes in

intracranial blood volume (the amount
of blood within the brain).
The balance between production and
absorption of CSF is critically important.
Because CSF is made continuously,
medical conditions that block its normal
flow or absorption will result in an overaccumulation of CSF.The resulting
pressure of the fluid against brain
tissue is what causes hydrocephalus.
What are the different types of
hydrocephalus?

ydrocephalus may be congenital or

acquired. Congenital hydrocephalus
is present at birth and may be caused by
either events orinfluences that occur during
fetal development, or genetic abnormalities.
Acquired hydrocephalus develops at the
time of birth or at some point afterward.
This type of hydrocephalus can affect
individuals of all ages and may be caused
by injury or disease.
Hydrocephalus may also be communicating
or non-communicating. Communicating
hydrocephalus occurs when the flow of CSF
is blocked after it exits the ventricles. This
form is called communicating because the
CSF can still flow between the ventricles,
which remain open. Non-communicating
hydrocephalus also called obstructive
hydrocephalus occurs when the flow of
CSF is blocked along one or more of the
narrow passages connecting the ventricles.

One of the most common causes of

hydrocephalus is aqueductal stenosis.
In this case, hydrocephalus results from
a narrowing of the aqueduct of Sylvius,
a small passage between the third and
fourth ventricles in the middle of the brain.
There are two other forms of hydrocephalus
which do not fit exactly into the categories
mentioned above and primarily affect adults:
hydrocephalus ex-vacuo and Normal Pressure
Hydrocephalus (NPH).
Hydrocephalus ex-vacuo occurs when stroke
or traumatic injury causedamage to the
brain. In these cases, brain tissue may
actually shrink. NPH is an abnormal increase
of cerebrospinal fluid in the brains ventricles
that may result from a subarachnoid
hemorrhage, head trauma, infection, tumor,
or complications of surgery. However, many
people develop NPH when none of these
factors are present. An estimated 375,000
older Americans have NPH.
Who gets this disorder?

he number of people who develop

hydrocephalus or who are currently
living with it is difficult to establish since
the condition occurs in children and adults,
and can develop later in life. A 2008 data
review by the University of Utah found that,
in 2003, hydrocephalus accounted for 0.6
percent of all pediatric hospital admissions
in the United States. Some estimates report
one to two of every 1,000 babies are born
with hydrocephalus.

What causes hydrocephalus?

he causes of hydrocephalus are still not

well understood. Hydrocephalus may
result from inherited genetic abnormalities
(such as the genetic defect that causes
aqueductal stenosis) or developmental
disorders (such as those associated with
neural tube defects including spina bifida
and encephalocele). Other possible causes
include complications of premature birth
such as intraventricular hemorrhage, diseases
such as meningitis, tumors, traumatic head
injury, or subarachnoid hemorrhage, which
block the exit of CSF from the ventricles to
the cisterns or eliminate the passageway for
CSF within the cisterns.
What are the symptoms?

ymptoms of hydrocephalus vary with

age, disease progression, and individual
differences in tolerance to the condition. For
example, an infants ability to compensate
for increased CSF pressure and enlargement
of the ventricles differs from an adults. The
infant skull can expand to accommodate
the buildup of CSF because the sutures (the
fibrous joints that connect the bones of the
skull) have not yet closed.
In infancy, the most obvious indication of
hydrocephalus is often a rapid increase in
head circumference or an unusually large
head size. Other symptoms may include
vomiting, sleepiness, irritability, downward
deviation of the eyes (also called sun
setting), and seizures.

Older children and adults may experience

different symptoms because their skulls
cannot expand to accommodate the buildup
of CSF. Symptoms may include headache
followed by vomiting, nausea, blurred
or double vision, sun setting of the eyes,
problems with balance, poor coordination,
gait disturbance, urinary incontinence,
slowing or loss of developmental progress,
lethargy, drowsiness, irritability, or other
changes in personality or cognition including
memory loss.
Symptoms of normal pressure hydrocephalus
include problems with walking, impaired
bladder control leading to urinary frequency
and/or incontinence, and progressive mental
impairment and dementia. An individual
with this type of hydrocephalus may have
a general slowing of movements or may
complain that his or her feet feel stuck.
Because some of these symptoms may also
be experienced in other disorders such as
Alzheimers disease, Parkinsons disease,
and Creutzfeldt-Jakob disease, normal
pressure hydrocephalus is often incorrectly
diagnosed and never properly treated.
Doctors may use a variety of tests, including
brain scans such as computed tomography
(CT) and magnetic resonance imaging (MRI),
a spinal tap or lunbar catheter, intracranial
pressure monitoring, and neuropsychological
tests, to help them accurately diagnose
normal pressure hydrocephalus and rule
out any other conditions.

The symptoms described in this section

account for the most typical ways in which
progressive hydrocephalus is noticeable, but
it is important to remember that symptoms
vary significantly from person to person.
How is hydrocephalus diagnosed?

ydrocephalus is diagnosed through

clinical neurology evaluation and by
using cranial imaging techniques such as
ultrasonography, CT, or MRI, or pressuremonitoring techniques. A physician selects
the appropriate diagnostic tool based on an
individuals age, clinical presentation,
and the presence of known or suspected
abnormalities of the brain or spinal cord.
What is the current treatment?

ydrocephalus is most often treated

by surgically inserting a shunt system.
This system diverts the flow of CSF from
the CNS to another area of the body where
it can be absorbed as part of the normal
circulatory process.
A shunt is a flexible but sturdy plastic
tube. A shunt system consists of the shunt,
a catheter, and a valve. One end of the
catheter is placedwithin a ventricle inside
the brain or in the CSF outside the spinal
cord. The other end of the catheter is
commonly placed within the abdominal
cavity, but may also be placed at other sites
in the body such as a chamber of the heart
or areas around the lung where the CSF
can drain and be absorbed. A valve located

along the catheter maintains one-way flow

and regulates the rate of CSF flow.
A limited number of individuals can be
treated with an alternative procedure called
third ventriculostomy. In this procedure, a
neuroendoscope a small camera that uses
fiber optic technology to visualize small and
difficult to reach surgical areas allows a
doctor to view the ventricular surface. Once
the scope is guided into position, a small tool
makes a tiny holein the floor of the third
ventricle, which allows the CSF to bypass
the obstruction and flow toward the site of
resorption around the surface of the brain.
What are the possible complications of a
shunt system?

hunt systems are imperfect devices.

Complications may include mechanical
failure, infections, obstructions, and the need
to lengthen or replace the catheter. Generally,
shunt systems require monitoring and regular
medical follow up. When complications occur,
subsequent surgery to replace the failed part
or the entire shunt system may be needed.
Some complications can lead to other
problems such as overdraining or
underdraining. Overdraining occurs when
the shunt allows CSF to drain from the
ventricles more quickly than it is produced.
Overdraining can cause the ventricles to
collapse, tearing blood vessels and causing
headache, hemorrhage (subdural hematoma),
or slit-like ventricles (slit ventricle syndrome).

Underdraining occurs when CSF is not

removed quickly enough and the symptoms
of hydrocephalus recur. Overdrainage
and underdrainage of CSF are addressed
by adjusting the drainage pressure of the
shunt valve; if the shunt has an adjustable
pressure valve these changes can be made
by placing a special magnet on the scalp
over the valve. In addition to the common
symptoms of hydrocephalus, infections from
a shunt may also produce symptoms such
as a low-grade fever, soreness of the neck or
shoulder muscles, and redness or tenderness
along the shunt tract. When there is reason to
suspect that a shunt system is not functioning
properly (for example, if the symptoms of
hydrocephalus return), medical attention
should be sought immediately.
What is the prognosis?

he prognosis for individuals diagnosed

with hydrocephalus is difficult to predict,
although there is some correlation between
the specific cause of the hydrocephalus and
the outcome. Prognosis is further clouded
by the presence of associated disorders, the
timeliness of diagnosis, and the success of
treatment. The degree to which relief of
CSF pressure following shunt surgery can
minimize or reverse damage to the brain is
not well understood.
Affected individuals and their families
should be aware that hydrocephalus
poses risks to both cognitive and physical
development. However, many children
diagnosed with the disorder benefit from

rehabilitation therapies and educational

interventions and go on to lead normal
lives with few limitations. Treatment
by an interdisciplinary team of medical
professionals, rehabilitation specialists, and
educational experts is critical to a positive
outcome. Left untreated, progressive
hydrocephalus may be fatal.
The symptoms of normal pressure
hydrocephalus usually get worse over time
if the condition is not treated, although
some people may experience temporary
improvements. While the success of
treatment with shunts varies from person
to person, some people recover almost
completely after treatment and have a
good quality of life. Early diagnosis
and treatment improves the chance
of a good recovery.
What research is being done?

he National Institute of Neurological

Disorders and Stroke (NINDS) and

other institutes of the National Institutes
of Health (NIH) conduct research related
to hydrocephalus and support additional
research through grants to major medical
research 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.

The Hydrocephalus Clinical Research

Network (HCRN, www.hcrn.org) is a multicenter collaborative research effort that
was borne out of the first NIH workshop
on hydrocephalus. NINDS supported the
work of HCRN through the Challenge
Grant process to advance their studies.
HCRN consists of seven pediatric centers
that pool their hydrocephalus patient
population to more rapidly study the
potential for improved treatments. HCRN
conducts multiple, simultaneous studies at
all of its centers and maintains a substantial
registry of patients and procedures.
Where can I get more information?

or more information on neurological

disorders or research programs funded
by the National Institute of Neurological
Disorders and Stroke, contact the Institutes
Brain Resources and Information Network
(BRAIN) at:
BRAIN
P.O. Box 5801
Bethesda, MD 20824
800-352-9424
www.ninds.nih.gov
Information also is available from the
following organizations:
Hydrocephalus Association
4340 East-West Highway
Suite 905
Bethesda, MD 20814
301-202-3811
www.hydroassoc.org

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National Hydrocephalus Foundation

12412 Centralia Road
Lakewood, CA 90715-1652
562-924-6666
888-598-3789
www.nfonline.org
Pediatric Hydrocephalus Foundation
2004 Green Hollow Drive
Inselin, NJ 08830
732-634-1283
www.hydrocephaluskids.org

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NIH . . . Turning Discovery Into Health

Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological
Disorders and Stroke
National Institutes of Health
Department of Health and Human Services
Bethesda, Maryland 20892-2540

NIH Publication No. 13-385

February 2013