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Hydrocephalus

Cerebrospinal fluid serves as a protective cushion around the brain and spinal
cord. The fluid is secreted by the choroid plexuses of the ventricles. It flows from the
lateral ventricles into the third ventricle, through the cerebral aqueduct (aqueduct of
Sylvius) into the fourth ventricle and then out into the subarachnoid space through three
small openings in the roof of the fourth ventricle. The fluid circulates around the cord and
over the convexity of the brain and is resorbed into the large venous sinuses in the dura.
Secretion of the CSF continues even if the flow of fluid through the ventricular system is
blocked. Obstruction to the normal circulation of spinal fluid distends the ventricles
proximal to the site of obstruction, with associated compression atrophy of the brain
tissue around the dilated ventricles. Such condition is called hydrocephalus.
Hydrocephalus is an excessive accumulation of cerebrospinal fluid (CSF) within
the ventricular spaces of the brain. In infants, hydrocephalus enlarges the head; infants
and adults, resulting compression can cause damage brain tissue. With early detection
and surgical intervention, the prognosis improves but remains guarded. Even after
surgery, such complications as mental retardation, impaired motor function, and vision
loss can persist. Without surgery, the prognosis is poor: Mortality may result from
increased intracranial pressure (ICP); infants may also die prematurely of infection and
malnutrition.

Cause and Incidence


Hydrocephalus may result from an obstruction in CSF flow (noncommunicating
hydrocephalus) or from faulty absorption of CSF (communicating hydrocephalus). In non
communicating hydrocephalus, the obstruction occurs most frequently between the third
and fourth ventricles, at the aqueduct of Sylvius, but it can also occur at the outlets of
fourth ventricles (foramina of Luschka and Magendie) or, rarely, at the foramen of Monro.
This obstruction may result from faulty fetal development, infection (syphilis,
granulomatous diseases, meningitis), a tuimor, cerebral aneurysm, or a blood clot (after
intracranial hemorrhage).
Communicating hydrocephalus occurs as the result of impaired reabsorption of
CSF from the arachnoid villi into the venous system. Decreased absorption can result
from a block in the CSF pathway to the arachnoid villi or a failure of the villi to transfer
the CSF to the venous system. It can occur if too few villi are formed, if postinfective
(meningitis) scarring occludes them, or if the villi become obstructed with fragments of
blood or infectious debris. Normal-pressure hydrocephalus seen in older adults, it is
accompanied by ventricular enlargement with compression of cerebral tissue but normal
CSF pressure.
Hydrocephalus occurs most commonly in neonates but also occur in adults as
result of injury or disease. It affects 1 of every 1000 people.

Complications
Mental retardation, impaired motor function, vision loss, death

Signs and Symptoms


In infants, the unmistakable sign of hydrocephalus is rapidly increasing head
circumference, clearly disproportionate to infant’s growth. Other characteristic changes
include widening and bulging of the fontanels; distended scalp veins; thin, shiny, and
fragile-looking scalp skin; and under developed neck muscles that can’t support the
head. In severe hydrocephalus, the roof of the orbit is depressed, the eyes are displaced
downward, and the scleras are prominent. Sclera seen above the iris is called the
“setting-sun sign.” A high-pitched, shrill cry, abnormal muscle tone of the legs, irritability,
anorexia, and projectile vomiting commonly occur. In adults and older children, indicators
of hydrocephalus include decreased level of consciousness (LOC), ataxia, incontinence,
loss of coordination, and impaired intellect.
The clinical manifestations include papilledema, spasticity, and ataxia affecting
the extremities and a progressive decline in judgement and reasoning aith a
characteristic “empty chatter” type of speech pattern.

Diagnosis
In infants, abnormally large head size for the patient’s age strongly suggests
hydrocephalus. Measurement of head circumference is a most important diagnostic
technique. Skull x-rays show thinning of the skull with separation of sutures and
widening of fontanels.
Other diagnostic tests for hydrocephalus, including arteriography, computed
tomography scan, and magnetic resonance imaging (MRI), can differentiate between
hydrocephalus and intracranial lesions and can also demonstrate the Arnold-Chiari
deformity, which may occur in an infant with hydrocephalus.
Treatment/ Medical-Surgical Management
Hydrocephalus can often be treated successfully by inserting a plastic tube into
one of the dilated ventricles and rerouting (shunting) the fluid into another part of the
body where it can be absorbed. The fluid can be shunted into the right atrium
(ventriculoatrial shunt) or into the peritoneal cavity (ventriculoperitoneal shunt). A small
opening is made in the skull to allow insertion of a plastic drainage tube through cerebral
hemisphere into one of the dilated lateral ventricles. The other end of the tube is passed
through the subcutaneous tissues behind the ear. In a ventriculoatrial shunt, the tube is
inserted into the jugular vein and threaded down the vein so that the tip is positioned in
the right atrium. In the more commonly used ventriculoperitoneal shunt the tube is
passed through the subcutaneous tissues of the neck, chest, and upper abdomen and
introduced into the abdominal cavity through a small incision in the peritoneum.
Whatever type of shunt is used, a one-way valve is incorporated in the tube to prevent
any reflux of blood or peritoneal fluid into the ventricles.

Nursing Management
Observation is essential, as is conscientious collection of data, including daily
measurement of the head circumference and check of the size and fullness of the
anterior fontanel. Noting any change in the infant’s behavior is also important.
Nurses have a direct responsibility for the nutritional requirements of these
newborns, but feeding may be a particular time-consuming activity for the staff. When
the infant is irritable or vomiting, various techniques should be attempted to provide
adequate nutrients and fluids. Techniques that are successful for a particular infant
should be shared with all persons involved in feeding him, including the parents. Feeding
times should be flexible, and small feedings at frequent intervals may prove more
successful. The increased head size makes positioning a potential problem, especially
when the head circumference is increasing rapidly. Hydrocephalic neonates may
develop decubiti if they are not turned often. Frequent linen changes and the use of
lamb’s wool also help deter skin breakdown. The infant should be turned cautiously, for
the increased head size places an additional strain on the neck.
The primary nursing goal for an infant after a shunting procedure for
hydrocephaly is the promotion of CNS intactness. The vital signs are frequently
monitored, neurological assessments are performed, and the shunt pathways are closely
examined.
The shunting of CSF extracranially has many technical problems. The sudden
release of ICP with a shunt insertion may precipitate a seizure. The infant’s immature
system may not readily adapt to the rapid release of pressure. The shunt tubing may
occlude or separate. Close attention is therefore given to vital signs, palpation of the
anterior fontanel when the infant is quiet and in an upright position, feeding and
behavioral patterns and the signs and symptoms of increased ICP and cerebral
irritability.
The physician may request that the infant be placed in semi-Fowler’s position to
assist in the draining of the ventricles through the shunt. The shunt pathways are
observed for any infectious or inflammatory process. The insertion point of the shunt into
the ventricles, the valve on the side of the head, and the extracranial shunt tubing paths
are all examines for redness, swelling, or drainage. Tension on the tissue covering the
shunt may cause skin erosion with the potential for a serious infection. With
ventriculoperitoneal shunts, abdominal girths need to be measures. Pooling of CSF may
occur because of the inability of the peritoneal cavity to absorb the relatively large
volume of CSF. The physician or nurse may test the patency of the tube by pressing on
the valve. The valve is functioning when it easily depresses and refills with CSF and
returns to its original position. If the valve does not depress and refill easily, either the
shunt is malfunctioning or the pressure of the CSF within the ventricles is inadequate.
These neonates also have emotional needs which should not be dismissed. They
enjoy being held or cuddled. Although some nurses may be hesitant about handling the
baby, it is important to remember that dexterity comes through experience. Such an
endeavor on the part of one health team member may support and encourage others to
do likewise.
Bibliography;

Abercrombie, Diane Dixon, MA, MMSc, PA-C. Professional Guide to Diseases 9th ed.
Lippincott Williams and Wilkins. 323 Norristown Road, Suite 200, Ambler,
Philadelphia, PA 19002-2756. pp 176-179

Crowley, Leonard V., M.D. An Intoduction to Human Diseases: Pathology and


Pathophysiology Correlations 7th ed. Jones and Bartlett Publishers. Sudbury,
Massachusets. Pp 719-721

Porth, Carol mattson, RN, MSN, PhD. Essentials of Pathophysiology: Concepts of


Altered Health States. Lippincott Williams and Wilkins. 530 Walnut St.,
Philadelphia, PA 19106. pp 829-830

Scipien, Gladys M., RN, MS. Pediatric Nursing Care. The C.V. Mosby Company. 11830
Westline Industrial Drive, St. Louis, Missouri 63146. pp 403- 404
Outlook (Prognosis)

Untreated hydrocephalus has a 50-60% death rate, with the survivors having varying degrees of
intellectual, physical, and neurological disabilities.

The outlook for treated hydrocephalus depends on the cause. Hydrocephalus that is caused by
disorders not associated with infection has the best outlook. Persons with hydrocephalus caused
by tumors usually do very poorly.

Most children with hydrocephalus that survive for 1 year will have a fairly normal life span.
Approximately a third will have normal intellectual function, but neurological difficulties may
persist.

Possible Complications

Shunt placement may lead to blockage. Symptoms of such a blockage include headache and
vomiting. Surgeons may be able to help the shunt open without having to replace it.

There may be other problems with the shunt, such as kinking, tube separation, or infection in the
area of the shunt.

Other complications may include:

• Complications of surgery
• Infections such as meningitis or encephalitis
• Intellectual impairment
• Nerve damage (decrease in movement, sensation, function)
• Physical disabilities

Symptoms

The symptoms depend on the cause of the blockage, the person's age, and how much brain
tissue has been damaged by the swelling.

In infants with hydrocephalus, CSF fluid builds up in the central nervous system, causing the
fontanelle (soft spot) to bulge and the head to expand. Early symptoms may also include:

• Separated sutures
• Vomiting

Symptoms of continued hydrocephalus may include:

• Irritability, poor temper control


• Muscle spasticity (spasm)

Symptoms that occur later in the disease may include:

• Brief, shrill, high-pitched cry


• Decreased mental function
• Delayed development
• Difficulty feeding
• Excessive sleepiness
• Loss of bladder control (urinary incontinence)
• Slow growth (child 0-5 years)
• Slow or restricted movement

Symptoms in older babies and children may include:

• Changes in facial appearance and eye spacing


• Confusion or psychosis
• Crossed eyes
• Headache
• Loss of coordination
• Poor gait (walking pattern)
• Uncontrolled eye movements
• Vision changes
• Vomiting

Exams and Tests

When a health care provider taps fingertips on the skull, there may be abnormal sounds that
indicated thinning and separation of skull bones. Scalp veins may appear stretched or enlarged.

Part or the entire head may be larger than normal. Enlargement is most commonly seen in the
front part of the head. Head circumference measurements, repeated over time, may show that
the head is getting bigger.

The eyes may look "sunken in." The white part of the eye may appear above the colored part of
the eye, given the eyes a "setting-sun" appearance. Reflexes may be abnormal.

A head CT scan is one of the best tests for identifying hydrocephalus. Other tests that may be
done include:

• Arteriography
• Brain scan using radioisotopes
• Cranial ultrasound (an ultrasound of the brain)
• Lumbar puncture and examination of the cerebrospinal fluid (rarely done)
• Skull x-rays
Definition

Hydrocephalus is an abnormal expansion of cavities (ventricles) within the brain


that is caused by the accumulation of cerebrospinal fluid. Hydrocephalus comes
from two Greek words: hydros means water and cephalus means head.

There are two main varieties of hydrocephalus: congenital and acquired. An


obstruction of the cerebral aqueduct (aqueductal stenosis) is the most frequent
cause of congenital hydrocephalus. Acquired hydrocephalus may result from
spina bifida, intraventricular hemorrhage, meningitis, head trauma, tumors, and
cysts.

Description

Hydrocephalus is the result of an imbalance between the formation and drainage


of cerebrospinal fluid (CSF). Approximately 500 milliliters (about a pint) of CSF is
formed within the brain each day, by epidermal cells in structures collectively
called the choroid plexus. These cells line chambers called ventricles that are
located within the brain. There are four ventricles in a human brain. Once formed,
CSF usually circulates among all the ventricles before it is absorbed and returned
to the circulatory system. The normal adult volume of circulating CSF is 150 ml.
The CSF turn-over rate is more than three times per day. Because production is
independent of absorption, reduced absorption causes CSF to accumulate within
the ventricles.

There are three different types of hydrocephalus. In the most common variety,
reduced absorption occurs when one or more passages connecting the ventricles
become blocked. This prevents the movement of CSF to its drainage sites in the
subarachnoid space just inside the skull. This type of hydrocephalus is called
"noncommunicating." In a second type, a reduction in the absorption rate is
caused by damage to the absorptive tissue. This variety is called "communicating
hydrocephalus."

Both of these types lead to an elevation of the CSF pressure within the brain.
This increased pressure pushes aside the soft tissues of the brain. This
squeezes and distorts them. This process also results in damage to these
tissues. In infants whose skull bones have not yet fused, the intracranial pressure
is partly relieved by expansion of the skull, so that symptoms may not be as
dramatic. Both types of elevated-pressure hydrocephalus may occur from infancy
to adulthood.

A third type of hydrocephalus, called "normal pressure hydrocephalus," is marked


by ventricle enlargement without an apparent increase in CSF pressure. This
type affects mainly the elderly.
Hydrocephalus has a variety of causes including:

• congenital brain defects


• hemorrhage, either into the ventricles or the subarachnoid space
• infection of the central nervous system (syphilis, herpes, meningitis, encephalitis,
or mumps)
• tumor
• Hydrocephalus is believed to occur in approximately one to two of every 1,000
live births. The incidence of adult onset hydrocephalus is not known. There is no
known way to prevent hydrocephalus.

— L. Fleming Fallon, Jr., MD, PhD, DrPH

Definition

The word hydrocephalus derives from the Greek words hydro, meaning water,
and cephalus, meaning head. Hydrocephalus is the result of the excessive
accumulation of fluid in the brain. Traditionally, hydrocephalus has been
described as a disease characterized by increased intracranial pressure (ICP),
increased cerebrospinal fluid (CSF) volume, and dilatation of the CSF spaces
known as cerebral ventricles.

Description

Hydrocephalus is the result of an imbalance between the formation and drainage


of cerebrospinal fluid. This imbalance appears when an injury or illness alters the
circulation of CSF; one or more of the ventricles of the brain become enlarged as
CSF accumulates. However, hydrocephalus is not a single disease entity, as a
wide number of underlying diseases are responsible for causing retention of CSF,
resulting in ventricular dilatation and increased intracranial pressure (ICP). In
infants and children, for example, hydrocephalus usually results from a birth
defect, viral infection, head injury, hemorrhage, meningitis, or tumor.

In adults, the causes of hydrocephalus include brain damage due to stroke or


injury, Alzheimer's disease, or obstruction of the ventricles. Often, the cause is
unknown. Conditions responsible for hydrocephalus in a fetus include infantile
congenital (present at birth) hydrocephalus, hydrocephalus associated with
encephalocele or myelomeningocele, posthemorrhagic hydrocephalus in
newborns, and postmeningitic hydrocephalus. Conditions responsible for
hydrocephalus in adults include hydrocephalus following subarachnoid
hemorrhage, idiopathic adult hydrocephalus, and posttraumatic hydrocephalus.
Tumors can also result in hydrocephalus in both children and adults. Based on
the different kind of CSF circulation in the brain, hydrocephalus can be divided
into two types: communicating and non-communicating. In communicating
hydrocephalus, the CSF circulation pathways are competent from the ventricles
inside of the brain to the fluid spaces just below the third ventricle. Non-
communicating (obstructive) hydrocephalus refers to hydrocephalus that
develops from a blockage of the normal circulation of CSF within the brain. In
most cases, it refers to a blockage between the third and fourth ventricles.

Demographics

Overall incidence of infantile hydrocephalus is approximately one to two per


1,000 live births. The overall prevalence of hydrocephalus in the United States is
about 0.5%. When cases of spina bifida are included, congenital hydrocephalus
occurs in two to five births per 1,000 births. The incidence of acquired
hydrocephalus in adults is not known because it occurs as a result of injury,
illness, or environmental factors. Normal pressure hydrocephalus was found to
be significantly more prevalent in males, and can occur in adults of any age
group. The age distribution in children and teenagers is disputed.

Causes and symptoms

Approximately 16 oz (500 ml) of CSF are formed within the brain each day, by
cells located on the wall of the four ventricles in the brain. Once formed, CSF
circulates among all the ventricles before it is absorbed. The normal adult volume
of circulating CSF is about 2 oz (150 ml). The CSF turnover rate is more than
three times per day. Because production is independent of absorption, reduced
absorption causes CSF to accumulate within the ventricles.

Hydrocephalus can be subdivided into three forms, involving the following:

• Disorders of cerebrospinal fluid circulation. Tumors, hemorrhages, congenital


malformations, and infections can cause such obstructions in the circulation of
cerebrospinal fluid.
• Disorders of cerebrospinal fluid absorption, resulting from diseases such as the
superior vena cava syndrome and sinus thrombosis.
• Disorders of cerebrospinal fluid production: This is the less common form of
hydrocephalus resulting from tumors that secrete cerebrospinal fluid in excess of
its absorption.

Congenital hydrocephalus is thought to be caused by a complex interaction of


genetic and environmental factors. The origin of hydrocephalus in congenital
cases is unknown. Very few cases (less than 2%) are inherited (X-linked
hydrocephalus). The most common causes of hydrocephalus in acquired cases
are tumor obstruction, trauma, intracranial hemorrhage, and infection.

The two most common adult forms of hydrocephalus are hydrocephalus ex-
vacuo and normal pressure hydrocephalus. Hydrocephalus ex-vacuo occurs
when a stroke or injury damages the brain, yielding a brain substance. Although
there is more CSF than usual, the CSF pressure may or may not be elevated.
Normal pressure hydrocephalus is an abnormal increase of CSF in the brain's
ventricles due to the gradual blockage of the CSF-draining pathways. This may
result from a subarachnoid hemorrhage, head trauma, infection, tumor, or
complications of surgery. The ventricles enlarge to handle the increased volume
of the CSF, and the compression of the brain from within by the fluid-filled
ventricles destroys or damages brain tissue. Fluctuation of CSF pressure from
high to normal to low can also be present.

For congenital-onset hydrocephalus, early symptoms include enlargement of the


head (increased head circumference), bulging fontanelles (soft spots) with or
without enlargement of the head size, separation of sutures (the flexible and
fibrous joints between the skull bones of an infant), and vomiting. Symptoms of
continued hydrocephalus include irritability and muscle spasticity. Late symptoms
of congenital-onset hydrocephalus seen in children up to five years of age
include decreased mental function, delayed development, slow or restricted
movement, difficulty feeding, lethargy, and delayed growth.

In children, symptoms depend on the amount of damage caused by ICP.


Symptoms may be similar to many of those in infants or may include headache,
vomiting, vision changes such as crossed eyes, uncontrolled eye movements,
loss of coordination, poor gait (walking pattern), mental confusion, or psychosis.
For adult-onset hydrocephalus, headaches and nausea are the most common
symptoms. Other signs of the condition include difficulty focusing the eyes,
unsteady gait, weakness of the legs, sudden falls, and a distinctive inability to
walk forward. As hydrocephalus progresses, decreased mental activity appears,
including lethargy, apathy, impaired memory, and speech problems. Urinary and
bowel incontinence can also occur. During the final stage, dementia involving
loss of movement, sensory functions, and cognitive abilities may result.

Diagnosis

Ultrasound can be used to diagnose prenatal hydrocephalus. Although fetal


hydrocephalus may be an isolated finding, it is more frequently found along with
other cerebral anomalies, including neural tube defects. Diagnosis after birth may
be suggested by symptoms; however, imaging studies of the brain are the
mainstay of diagnosis. Computed tomography (CT) and magnetic resonance
imaging (MRI) reveal enlarged ventricles and may indicate a specific cause of
hydrocephalus, such as a tumor or hemorrhage. The presence of papilledema
(elevation or swelling of the optic disc) also indicates that hydrocephalus that is
well developed. In rare cases, long-standing hydrocephalus causes blindness.

Small abnormalities that may not be seen with CT scanning, such as cysts and
abscesses, are often seen with MRI. These studies can also help the
neurosurgeon differentiate between communicating and non-communicating
hydrocephalus. In cases of suspected normal pressure hydrocephalus, a lumbar
puncture (spinal tap) may help determine CSF pressure. Also, a cisternagram
can be useful to evaluate the dynamics of CSF flow in the brain and spinal chord.
Cisternography can reveal CSF concentration, obstruction, leakage, and
pressure. Also, certain biochemical markers in the blood have been described in
the disease. They include increased neurofilament light protein (NFL) and tau
protein, both markers of neuronal degeneration; increased myeline basic protein
(a marker of demyelination; and albumin); and a marker of the blood-brain barrier
function.

Treatment team

Treatment of hydrocephalus for children or adults will likely involve a neurologist,


neurosurgeon, obstetrician, pediatrician, and specialty nurses and physical
therapists.

Treatment

Medical treatment is first aimed at reducing intracranial pressure, while the need
for a more permanent solution is determined. Reduction of fluid intake and
administration of drugs such as mannitol, glycerol, urea (drugs with an osmotic
effect), or furosemide (a diuretic) are able to reduce ICP and CSF production.

External drainage of the CSF is useful for urgent reduction of intracranial


pressure, as well as of ventricular or subarachnoid hemorrhage. Complications
include overdrainage, blocked tube, or bacterial contamination. The placement of
a permanent ventricular shunt (internal shunting) is a common procedure. Around
33,000 shunts are placed in the United States each year; almost half of them to
replace previous shunt devices. CSF from the ventricles in the brain is usually
shunted to the peritoneum, pleura, ureter, bladder, or vascular spaces such as
the jugular or subclavian veins. Most shunts are connected to the peritoneum.
Some shunts operate according to intracranial pressure by using a valve system
able to regulate the flow at a pressure close to the normal values of ICP. Others
are programmable and can be adjusted to open at a given ICP. Complications
include overdrainage that may cause intracranial hypotension, subdural
hematoma, shunt occlusion, and infection. The risk of shunt failure is greater
within the first year (between 25–40% of shunts must be replaced). The
subsequent failure rate is around 5% for each year.

Other surgical procedures include, in some cases, choroid plexectomy, third


ventriculostomy, and ventricular reservoir. Ventricular reservoir is basically a
catheter inserted into a ventricle of the brain to draw CSF. This procedure is
much simpler than placing a full shunt system and is used to provide temporary
control of ICP until a full shunt can be placed.

Recovery and rehabilitation

Hydrocephalus is a chronic condition, and clinical symptoms are based on the


time of insurgence of the disease. With appropriate, early treatment, a normal
lifespan with few limitations can be reached. After surgery, specially trained
medical professionals carefully monitor the patient. Some symptoms such as
headaches may disappear immediately due to the release of excess pressure.
The symptoms associated with normal pressure hydrocephalus (walking
difficulties, mild dementia, poor bladder control) may improve quickly, or may take
weeks to months to improve. In some patients, little or no improvement is also
possible.

The length of the patient's hospital stay will be determined by the rate of
recovery. If neurological problems persist, rehabilitation may be required to
further the patient's improvement. However, recovery may be limited by the
extent of the damage already caused by the hydrocephalus. Because
hydrocephalus is an ongoing condition, patients do require long-term follow up.
Follow-up diagnostic tests, including CT scans, MRI, and x rays, may be
performed to determine if the shunt is working correctly.

Clinical trials

Ventricular shunts are the most common surgical treatment for hydrocephalus
and appear to be the safest. It is possible that choroid plexectomy and third
ventriculostomy may become more feasible in the future if better procedures and
equipment are developed.

As of mid-2004, several clinical trials to study hydrocephalus were underway,


including a trial to evaluate the efficacy and safety of endoscopic choroid plexus
coagulation with third ventriculostomy in the treatment of idiopathic normal
pressure hydrocephalus, sponsored by the Frenchay Hydrocephalus Research
Fund. The National Institute of Neurological Disorders and Stroke is sponsoring a
study to establish the physiology of syringomyelia. Updated information on these
and other ongoing clinical trials may be found at the National Institutes of Health
website for clinical trials at .

Prognosis

Untreated hydrocephalus has a survival rate of 40–50%, with the survivors


having varying degrees of intellectual, physical, and neurological disabilities.
Prognosis for treated hydrocephalus varies, depending on the cause. If the child
survives for one year, more than 80% will have a fairly normal lifespan.
Approximately one-third will have normal intellectual function, but neurological
difficulties may persist. Hydrocephalus not associated with infection has the best
prognosis, and hydrocephalus caused by tumors has a very poor prognosis.
About 50% of all children who receive appropriate treatment and follow up will
develop IQs in the near-normal or normal range.

Resources

BOOKS

Matsumoto, Satoshi. Hydrocephalus: Pathogenesis and Treatment. New York:


Springer-Verlag, 1991.
The Official Parent's Sourcebook on Hydrocephalus: A Revised and Updated
Directory for the Internet Age. San Diego: Icon Group International, 2002.

Toporek, Chuck, and Kellie Robinson. Hydrocephalus: A Guide for Patients,


Families and Friends. Sebastopol, CA: Patient-Centered Guides, 1999.

PERIODICALS

Arriada, N., and J. Sotelo. "Review: Treatment of Hydrocephalus in Adults." Surg


Neurol. (2002) Dec 58 (6): 377–84.

Davis, G. H. "Fetal Hydrocephalus." Clin Perinatol. (2003) Sep 30 (3): 531–9.

Meier, U., and C. Miethke. "Predictors of Outcome in Patients with Normal-


Pressure Hydrocephalus." J Clin Neurosci. (2003) Jul 10 (4): 453–9.

OTHER

"NINDS Hydrocephalus Information Page." National Institute of Neurological


Disorders and Stroke. May 15, 2004 (May 22, 2004).
http://www.ninds.nih.gov/health_and_medical/disorders/hydrocephalus.htm.

"What is Hydrocephalus?" Hydrocephalus Foundation, Inc. May 15, 2004 (May


22, 2004). http://www.hydrocephalus.org/.

ORGANIZATIONS

Hydrocephalus Association. 870 Market Street, Suite 705, San Francisco, CA


94102. (415) 732-7040 or (888) 598-3789; Fax: (415) 732-7044.
info@hydroassoc.org. http://www.hydroassoc.org.

National Hydrocephalus Foundation. 12413 Centralia Road, Lakewood, CA


90715-1623. (562) 402-3523 or (888) 857-3434; Fax: (562) 924-6666.
hydrobrat@earthlink.net. http://nhfonline.org.

Antonio Farina, MD, PhD


Prognosis

Statistics vary regarding the success of shunt surgery. Not all patients with normal pressure
hydrocephalus experience significant improvements from shunt surgery. When the cause of
NPH has been determined, however, shunt surgery has a high success rate.

Untreated, NPH symptoms will generally worsen and likely lead to death over time. Individuals
who are advised not to undergo shunt surgery may see improvements with periodic lumbar
punctures and/or other treatments. When shunt surgery is successful, patients experience a
reversal or lessening of their symptoms and an improved quality of life.