m


an abnormal expansion of cavities

(ventricles) within the brain that is
caused by the accumulation of
cerebrospinal fluid.
Hydrocephalus comes from two Greek
words: Ú
 means water and
Ú
 means head.
M 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.
M These cells line chambers called ventricles that
are located within the brain.
M 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.
M 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.
  



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



1. Non-communicating - 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.
2. Communicating - a reduction in the
absorption rate is caused by damage to
the absorptive tissue.
M 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.
M 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.
M Both types of elevated-pressure
hydrocephalus may occur from infancy
to adulthood.
Y. "normal pressure hydrocephalus,"
is marked by ventricle enlargement
without an apparent increase in
CSF pressure. This type affects
mainly the elderly
   
M The primary site of CSF formation is
believed to be the choroid plexusus of
the lateral ventricles. CSF flows from
the lateral ventricles through the
foramen of Monro to the third ventricle,
then through the aqueduct of Sylvius
into the fourth ventricle through the
foramen of Luschka and the midline
foramen of Magendie into the cisterna
magna. From there it flows to the
cerebral and cerebellar subarachnoid
spaces where it is absorbed.
Hydrocephalus has a variety of causes
including:
M congenital brain defects
M hemorrhage, either into the ventricles or
the subarachnoid space
M infection of the central nervous system
(syphilis, herpes, meningitis,
encephalitis, or mumps)
M tumor
M 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.
M Hydrocephalus that is congenital
(present at birth) is thought to be caused
by a complex interaction of genetic and
environmental factors.
M Aqueductal stenosis, an obstruction of
the cerebral aqueduct, is the most
frequent cause of congenital
hydrocephalus.
M As of 2001, the genetic factors are not
well understood. According to the
British Association for Spina Bifida and
Hydrocephalus, in very rare
circumstances, hydrocephalus is due to
hereditary factors, which might affect
future generations.
Signs and symptoms of elevated-
pressure hydrocephalus include:
M headache
M nausea and vomiting, especially in
the morning
M lethargy
M disturbances in walking (gait)
M double vision
M subtle difficulties in learning and
memory
M delay in children achieving
developmental milestones
M Irritability is the most common sign of
hydrocephalus in infants. If this is not
treated, it may lead to lethargy. Bulging
of the fontanelles, or the soft spots
between the skull bones, may also be an
early sign. When hydrocephalus occurs
in infants, fusion of the skull bones is
prevented. This leads to abnormal
expansion of the skull.
M Symptoms of normal pressure
hydrocephalus include dementia, gait
abnormalities, and incontinence
(involuntary urination or bowel
movements).
Ô
M Imaging studies²x ray, computed
tomography scan (CT scan), ultrasound,
and especially magnetic resonance
imaging (MRI)²are used to assess the
presence and location of obstructions,
as well as changes in brain tissue that
have occurred as a result of the
hydrocephalus. Lumbar puncture (spinal
tap) may be performed to aid in
determining the cause when infection is
suspected.
 
M The primary method of treatment for both
elevated and normal pressure hydrocephalus is
surgical installation of a shunt.
M A shunt is a tube connecting the ventricles of
the brain to an alternative drainage site, usually
the abdominal cavity.
M A shunt contains a one way valve to prevent
reverse flow of fluid. In some cases of non-
communicating hydrocephalus, a direct
connection can be made between one of the
ventricles and the subarachnoid space,
allowing drainage without a shunt.
M Installation of a shunt requires lifelong
monitoring by the recipient or family members
for signs of recurring hydrocephalus due to
obstruction or failure of the shunt. Other than
monitoring, no other management activity is
usually required.
M Some drugs may postpone the need for surgery
by inhibiting the production of CSF. These
include acetazolamide and furosemide. Other
drugs that are used to delay surgery include
glycerol, digoxin, and isosorbide.
M Some cases of elevated pressure
hydrocephalus may be avoided by preventing
or treating the infectious diseases which
precede them.
M Prenatal diagnosis of congenital brain
malformation is often possible, offering the
option of family planning.
Surgical correction is the only treatment
M º è 
   Ú

M to transport excess fluid from the brain¶s
lateralventricle into the peritoneal cavity
M º è 
  Ú

M drains fluid from the brain¶s lateral ventricle
into the right atrium of the heart, where the fluid
makes its way into venous circulation
M º Ú 
è 


M elevation of the frontal lobe to expose the third
ventricle for catheter insertion and passes it
into the cisterna chiasmatis of the
subarachnoid space
M º è 
  Ú

M - making a small burr hole in the occipital
region and insertion of catheter into lateral
ventricle, passes it under the dura mater and
into cisterna magna

  
M £ Monitor neurologic status closely and
signs of increase ICP (headache,
vomiting, visual
M disturbances, decreased LOC)
M £  measure head
circumference daily, observe fontanels
for bulging and tenderness
M Post operative care:
M £ Monitor for signs of infection and use
strict aseptic technique when taking
care of the shunt
M and suture
M £ Check the shunt for any malfunction
M £ Pump the shunt as prescribed by the
physician (usually 25 to 50 times, once or twice
daily)
M - Ú  
 

 
 
M £ Gradually elevate the head gradually in
stages, about 20 degrees at a time (to help
adjust in
M the lowered ICP)- Ú
   

  

M £ Position client on nonoperative side (to
avoid pressure on the suture line and prevent
M dependent edema)
M £ Anticonvulsant medications may be
prescribed for 6 ± 12 months (to prevent
seizure)
Home Health Education:
M  Instruct family to report sings of infection
and increased ICP
M  Advise patient/family not to lie over the
catheter¶s course for a prolonged period
M  Teach client/family on how to pump the
shunt (caution not to pump excessively)
M  Emphasize compliance of anticonvulsant
drug therapy
M  Inform family that shunt requires periodic
surgery as the child grows older
M  Check the child¶s growth and development
periodically and help the parents set goals
M consistent with child¶s ability and potentials
M  Help parents focus on their child¶s strength,
not with his weakness
À
Ú   

M elongation or tongue-like downward
projection of the cerebellum
M medulla extends through the
foramen magnum into the cervical
portion of the spinal canal
(impairing the CSF drainage from
fourth ventricle)
M patient may have 
Ú  
 
        
  
  
Ú  Ú

M The prognosis for elevated-pressure
hydrocephalus depends on a wide
variety of factors, including the cause,
age of onset, and the timing of surgery.
M Studies indicate that about half of all
children who receive appropriate
treatment and follow-up will develop IQs
greater than 85.
M Those with hydrocephalus at birth do
better than those with later onset due to
meningitis.
M For individuals with normal pressure
hydrocephalus, approximately half will
benefit by the installation of a shunt.
ë


M Cerebral tissue herniation through a congenital
or acquired defect in the skull.
M The majority of congenital encephaloceles
occur in the occipital or frontal regions. Clinical
features include a protuberant mass that may
be pulsatile.
M The quantity and location of protruding neural
tissue determines the type and degree of
neurologic deficit. Visual defects, psychomotor
developmental delay, and persistent motor
deficits frequently occur.
M Encephaloceles are rare neural tube defects
characterized by sac-like protrusions of the
meninges (the membranes that cover the brain)
and brain.
M These defects are caused by failure of the
neural tube to close completely during fetal
development.There have been studies and
evidence linking NTD's to folic acid deficiency.
The severity of encephalocele varies,
depending on the location. Currently, the only
effective treatments are reparative surgeries
following birth. The degree to which they can
be corrected varies greatly on where and how
big the encephalocele is.
M In normal fetal development, the neural tube
forms by the closure of the neural structure.
When this does not occur in the case of an
encephalocele, the result is a groove.
M The groove can form down the middle region of
the upper part of the skull, or between the
forehead and the nose, or down the back of the
skull.
M The incomplete closure also creates areas
where the brain and its overlaying membrane
can bulge outward in sac-like protrusions.
M The larger deformities, in particular those that
occur at the back of the skull, are readily
evident and are recognized very soon after
birth. These deformities are also associated
with abnormal structure and functioning of the
brain. Some encephaloceles are less evident,
even to the point of being undetectable at birth.
Defects in the region of the forehead and nose
are examples.
{

  
M The exact cause of encephaloceles is not yet
known.
M The disorder is passed on from generation to
generation, and is more prevalent in families
where there is a history of spina bifida.
M It is clear that one or more genetic
abnormalities lie at the heart of the condition.
However, fetal development is an extremely
complex process, with interactions between
various genes, and influence of the external
environment determining which genes are
activated at which time.
M Thus, pinning down the crucial genes whose
expression or changed activity produces
abnormal neural tube formation is a difficult
task.
M Research using animal models has
shown that teratogens, compounds
like x rays, trypan blue, and
arsenic, which can damage the
developing fetus, cause
encephaloceles in the animals.
Whether exposure of a human fetus
to such agents contributes to
encephalocele formation in humans
is not known.
Most often, the symptoms of
encephaloceles are not difficult to
recognize.
M These include the excessive build-up of
cerebrospinal fluid in the brain (a
condition called hydrocephalus),
M paralyzed arms and legs (spastic
quadriplegia),
M an abnormally small head
(microcephaly),
M difficulty in tasks like walking and
reaching because of a lack of
coordination (ataxia),
M delayed or impaired mental and physical
development (although intelligence is
not always affected),
M problems with vision, and seizures.
M If the bulging portion contains only
cerebrospinal fluid and the
overlaying membrane, the malady
can also be called a cranial
meningocele or a meningocele.
M If brain tissue is also present, the
malady can also be referred to as
an encephalomeningocele.
Diagnosis
M based at the discovery of the
physical abnormalities at birth or
sometime later, and on the failure
to attain the various physical and
mental developmental milestones
that are a normal part of early life.
Treatment
M Treatment typically involves surgery. The
surgery is usually accomplished soon after
birth and re-positions the bulging brain back
into the skull, removes any of the sac-like
protrusions, and corrects the skull deformities.
M Often, shunts are placed during surgery to
drain excess cerebrospinal fluid from the brain.
M While delicate, the operation typically relieves
the pressure that would otherwise impede
normal brain development.
M Other treatment involves dealing with specific
symptoms and producing as comfortable and
satisfying everyday life as is possible.
Recovery and rehabilitation
M Prospects for recovery are difficult
to predict prior to surgery.
Nonetheless, if surgery is
successful, and other
developmental difficulties have not
occurred, an individual can develop
normally.
M Where neurological and
developmental damage has
occurred, the focus shifts from
recovery to maximizing mental and
physical abilities.
Prognosis
M As for recovery and rehabilitation,
the prognosis is varies and cannot
be predicted beforehand. In
general, when the bulging material
consists of mainly cerebrospinal
fluid, a complete recovery can
occur 60±80% of the time. However,
the presence of brain tissue in the
protruding material can reduce the
chances of a complete recovery
considerably.
Special concerns
M Folic acid, a B vitamin, has been
shown to help prevent neural tube
defects when taken before and in
early pregnancy. The March of
Dimes organization and the United
States Public Health Service
recommend that all women who
may become pregnant take a multi-
vitamin that contains 400
micrograms of folic acid every day.