What Is Hydrocephalus

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What is hydrocephalus?

The 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 over-accumulation of CSF. The resulting
pressure of the fluid against brain tissue is what causes hydrocephalus.

What are the different types of hydrocephalus?

Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is


present at birth and may be caused by either events or influences 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.

Hydrocephalus ex-vacuo occurs when stroke or traumatic injury


cause damage to the brain. In these cases, brain tissue may actually shrink.
Normal pressure hydrocephalus can happen to people at any age, but it is
most common among the elderly. It may result from a subarachnoid
hemorrhage, head trauma, infection, tumor, or complications of surgery.
However, many people develop normal pressure hydrocephalus even when
none of these factors are present for reasons that are unknown.

Who gets this disorder?

The number of people who develop hydrocephalus or who are currently living
with it is difficult to establish since there is no national registry or database of
people with the condition. However, experts estimate that hydrocephalus
affects approximately 1 in every 500 children.

What causes hydrocephalus?

The 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 into the
cisterns.

What are the symptoms?

Symptoms of hydrocephalus vary with age, disease progression, and


individual differences in tolerance to the condition. For example, an infant's
ability to compensate for increased CSF pressure and enlargement of the
ventricles differs from an adult's. 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 "sunsetting"), 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, papilledema (swelling of the
optic disk which is part of the optic nerve), blurred or double vision, sunsetting
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 Alzheimer's disease, Parkinson's
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 (CT and/or MRI), a spinal tap or lumbar
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 manifests itself, but it is important to
remember that symptoms vary significantly from one person to the next.

How is hydrocephalus diagnosed?

Hydrocephalus is diagnosed through clinical neurological evaluation and by


using cranial imaging techniques such as ultrasonography, computed
tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring
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?

Hydrocephalus 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 placed within 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 hole in 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?

Shunt systems are not perfect 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, the shunt system usually requires
some type of revision.

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. 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?

The 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 complicated 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?

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.

Prepared by: Office of Communications and Public Liaison National Institute of


Neurological Disorders and Stroke National Institutes of Health Bethesda, MD
20892

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