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Hydrocephalus

BY FEBRUARY 3, 2009
Nursing care plan

Anatomy and physiology

Practice Test
Definition:
Hydrocephalus is a condition caused by an imbalance in the
production and absorption of CSF in the ventricular system.
When production exceeds absorption, CSF accumulates,
usually under pressure, producing dilation of the ventricles.
It is a term derived from the Greek words hydro meaning
water, and cephalus meaning head, and this condition is
sometimes known as water on the brain.

People with hydrocephalus have abnormal accumulation of


cerebrospinal fluid (CSF) in the ventricles, or cavities, of the brain. This may cause increased intracranial
pressure inside the skull and progressive enlargement of the head, convulsion, and mental disability.
Usually, hydrocephalus does not cause any intellectual disability if recognized and properly treated. A massive
degree ofhydrocephalus rarely exists in typically functioning people, though such a rarity may occur if onset is
gradual rather than sudden.
Hydrocephalus occurs with a number of anomalies, such as NTDs.
Etiology:
Congenital hydrocephalus usually results from defects, such as Chairi malformations. It is also associated with
spina bifida.

Acquired hydrocephalus usually results from space-occupying lesions, hemorrhage, intracranial infections or
dormant development defects.

Classification of Hydrocephalus:
Hydrocephalus can be caused by impaired
cerebrospinal fluid (CSF) flow, reabsorption, or
excessive CSF production.

The most common cause of hydrocephalus


is CSF flow obstruction, hindering the free
passage of cerebrospinal fluid through the ventricular system and subarachnoid space (e.g., stenosis of
the cerebral aqueduct or obstruction of the interventricular foramina foramina of Monro secondary to
tumors, hemorrhages, infections or congenital malformations).
Hydrocephalus can also be caused by overproduction of cerebrospinal fluid (relative obstruction) (e.g.,
papilloma of choroid plexus).
Based on its underlying mechanisms, hydrocephalus can be classified into communicating, and non-
communicating(obstructive). Both forms can be either congenital, or acquired.
Communicating
Communicating hydrocephalus, also known as non-obstructive hydrocephalus
It is caused by impaired cerebrospinal fluid resorption in the absence of any CSF-flow obstruction.
It has been theorized that this is due to functional impairment of the arachnoid granulations, which are
located along the superior sagittal sinus and is the site of cerebrospinal fluid resorption back into the
venous system.
Various neurologic conditions may result in communicating hydrocephalus, including
subarachnoid/intraventricular hemorrhage, meningitis, Chiari malformation, and congenital absence of
arachnoidal granulations (Pacchionis granulations).
Normal pressure hydrocephalus (NPH) is a particular form ofcommunicating hydrocephalus,
characterized by enlarged cerebral ventricles, with only intermittently elevated cerebrospinal fluid
pressure. The diagnosis of NPH can be established only with the help of continuous intraventricular
pressure recordings (over 24 hours or even longer), since more often than not, instant measurements
yield normal pressure values. Dynamic compliance studies may be also helpful. Altered compliance
(elasticity) of the ventricular walls, as well as increased viscosity of the cerebrospinal fluid, may play a
role in the pathogenesis of normal pressure hydrocephalus.
Hydrocephalus ex vacuo also refers to an enlargement of cerebral ventricles and subarachnoid spaces,
and is usually due to brain atrophy (as it occurs in dementias), post-traumatic brain injuries and even in
some psychiatric disorders, such as schizophrenia. As opposed to hydrocephalus, this is acompensatory
enlargement of the CSF-spaces in response tobrain parenchyma loss it is not the result of increased
CSF pressure.
Non-communicating
Non-communicating hydrocephalus, or obstructive hydrocephalus, is caused by a CSF-flow obstruction (either
due to external compression or intraventricular mass lesions).
Foramen of Monro obstruction may lead to dilation of one or, if large enough (e.g., in colloid cyst), both
lateral ventricles.
The aqueduct of Sylvius, normally narrow to begin with, may be obstructed by a number of genetically or
acquired lesions (e.g., atresia, ependymitis, hemorrhage, tumor) and lead to dilatation of both lateral
ventricles as well as the third ventricle.
Fourth ventricle obstruction will lead to dilatation of the aqueduct as well as the lateral and third
ventricles.
The foramina of Luschka and foramen of Magendie may be obstructed due to congenital failure of
opening (e.g., Dandy-Walker malformation).
The subarachnoid space surrounding the brainstem may also be obstructed due to inflammatory or
hemorrhagic fibrosing meningitis, leading to widespread dilatation, including the fourth ventricle.
Congenital
The cranial bones fuse by the end of the third year of life. For head enlargement to occur, hydrocephalus
must occur before then. The causes are usually genetic but can also be acquired and usually occur within
the first few months of life, which include 1) intraventricular matrix hemorrhages in premature infants, 2)
infections, 3) type II Arnold-Chiari malformation, 4) aqueduct atresia and stenosis, and 5) Dandy-Walker
malformation.
In newborns and toddlers with hydrocephalus, the head circumference is enlarged rapidly and soon
surpasses the 97th percentile. Since the skull bones have not yet firmly joined together, bulging, firm
anterior and posterior fontanelles may be present even when the patient is in an upright position.
The infant exhibits fretfulness, poor feeding, and frequent vomiting. As the hydrocephalus progresses,
torpor sets in, and the infant shows lack of interest in his surroundings. Later on, the upper eyelids
become retracted and the eyes are turned downwards (due to hydrocephalic pressure on the
mesencephalic tegmentum and paralysis of upward gaze). Movements become weak and the arms may
become tremulous. Papilledema is absent but there may be reduction of vision. The head becomes so
enlarged that the child may eventually be bedridden.
About 80-90% of fetuses or newborn infants with spina bifidaoften associated with meningocele or
myelomeningoceledevelop hydrocephalus.
Acquired
This condition is acquired as
a consequence of CNS
infections, meningitis, brain
tumors, head trauma,
intracranial hemorrhage
(subarachnoid or
intraparenchymal) and is
usually extremely painful.
Pathophysiology of
Hydrocephalus:
Clinical Manifestations:
1. Abnormal rate of head
growth
2. Bulging fontanelle
3. Tense anterior fontanelle (often bulging and nonpulsatile)
4. Dilated scalp veins
5. Macewens sign (cracked pot)
6. Frontal bossing
7. Setting sun sign
8. Sluggish and unequal pupils
9. Irritability and lethargy with varying LOC
10. Abnormal infantile reflexes
11. Possible cranial nerve damage
Manifestations in children include possible signs of increased ICP, which include headache on awakening with
improvement following emesis, papilledema, strabismus, ataxia, irritability, lethargy, apathy and confusion.

Laboratory and Diagnostic Study Findings:


1. Level II ultrasonography of the fetus will allow a prenatal diagnosis. (Transuterine placement of
ventriculoamniotic shunts during late pregnancy is still being developed as a treatment modality).
2. CT scan will diagnose most cases postnatally.
3. MRI can be used if a complex lesion is suspected.
Nursing Management:
1. Teach the family about the management required for the disorder
a. Treatment is surgical by direct removal of an obstruction and insertion of shunt to provide primary
drainage of the CSF to an extracranial compartment, usually peritoneum (ventriculoperitoneal
shunt)
1. The major complications of shunts are infections and malfunction
2. Other complications include subdural hematoma caused by a too rapid reduction of CSF,
peritonitis, abdominal abscess, perforation of organs, fistulas, hernias and ileus.
b. A third ventriculostomy is a new nonshunting procedure used to treat children with hydrocephalus.
2. Provide preoperative nursing care
a. Assess head circumference, fontanelles, cranial sutures, and LOC; check also for irritability, altered
feeding habits and a high-pitched cry.
b. Firmly support the head and neck when holding the child.
c. Provide skin care for the head to prevent breakdown.
d. Give small, frequent feedings to decrease the risk of vomiting.
e. Encourage parental-newborn bonding.
3. Provide Postoperative nursing care (nursing interventions are the same as those for increased ICP)
a. Assess for signs of increased ICP and check the following; head circumference (daily), anterior
fontanelle for size and fullness and behavior.
b. Administer prescribed medications which may include antibiotics to prevent infection and analgesics
for pain.
c. Provide shunt care
1. Monitor for shunt infection and malfunction which may be characterized by rapid onset of
vomiting, severe headache, irritability, lethargy, fever, redness along the shunt tract, and
fluid around the shunt valve.
2. Prevent infection (usually from Staphylococcus epidermis or Staphylococcus aureus)
3. Monitor for shunt overdrainage (headache, dizziness and nausea). Overdrainage may lead to
slit ventricle syndrome whereby the ventricle become accustomed to a very small or slitlike
configuration, limiting the buffering ability to increased ICP variations.
4. Teach home care
a. Encourage the child to participate in age-appropriate activities as tolerated. Encourage the parents
to provide as normal lifestyle as possible. Remind both the child and parents that contact sports are
prohibited.
b. Explain how to recognize signs and symptoms of increased ICP. Subtle signs include changes in
school performance, intermittent headache, and mild behavior changes.
c. Arrange for the child to have frequent developmental screenings and routine medical checkups.
Myelomeningocele?

A view of a myelomeningocele or spina bifida, a formerly


common birth defect
A myelomeningocele (pronounced my-e-lo-MENING-o-seal) is a defect of the backbone (spine) and spinal
cord. Before birth, the baby's backbone, spinal cord and the structure they float in (spinal canal) do not form
or close normally.

A myelomeningocele is the most serious form of spina bifida. In babies with a myelomeningocele, the bones of
the spine (vertebrae) don't form properly. This lets a small sac extend through an opening in the spine. The sac
is covered with a membrane. It holds cerebrospinal fluid (CSF) and tissues that protect the spinal cord
(meninges). The sac may also contain portions of the spinal cord and nerves. The sac itself may be opened up
either before birth or during the birth.

A myelomeningocele can occur anywhere along the spinal cord. It is most common in the lower back (lumbar
and sacral areas). Babies lose function below the level of the problem. So, the higher the myelomeningocele is
on the baby's back, the more loss of function occurs.

Myelomeningocele in Children
Myelomeningoceles are present when a baby is born (congenital). About one to five babies in every 1,000
born in the United States have a myelomingocele. The condition develops during the third week of a woman's
pregnancy.

Doctors don't know exactly what causes myelomeningoceles. But there probably is a genetic component. If a
woman has one child with a myelomeningocele, there is a 3% to 5% chance that other children she has will
also have the condition.

While we don't know the exact cause of myelomeningoceles, doctors do know what can help prevent them.
Early in pregnancy, it is very important for women to get enough folic acid in their diets. This vitamin helps the
baby's neural tube develop properly. The neural tube develops into the baby's brain and spinal cord.

Myelomeningocele at Seattle Children's


Neurosurgeons at Seattle Children's treat many children with myelomeningoceles. These children often have
other complex problems. In our multidisciplinary clinic, our neurosurgeons work closely with experts from
other medical fields to make sure your child gets the care they need as they grow.
Doctors in our community usually find myelomeningoceles during exams before the baby is born. They refer
about 10 to 20 babies with myelomeningocele to Seattle Children's each year. The babies usually are
transferred from the hospital where they are born to Seattle Children's shortly after their birth. Our
neurosurgeons are on hand to close the hole in the baby's back, usually within 24 to 48 hours of birth.

Most children with a myelomeningocele develop hydrocephalus, or too much cerebrospinal fluid in parts of
the brain. Neurosurgeons at Seattle Children's have a great deal of experience putting in shunts, a common
treatment for hydrocephalus. We also have a lot of experience treating children with the related problems
of spinal cord tethering,Chiari malformation and syringomyelia.