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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.
Complications
Mental retardation, impaired motor function, vision loss, death
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
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.
• 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
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
Description
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.
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
Demographics
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.
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.
Diagnosis
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
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.
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.
Prognosis
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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.