HYDROCEPHALUS

Hydrocephalus is a condition caused by an imbalance in the productionand 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.

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 (Pacchioni’s granulations).

 Normal pressure hydrocephalus (NPH) is a particular form of communicating 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 a compensatory enlargement of the CSF-spaces in response
to brain 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 bifida—often associated with
meningocele or myelomeningocele—develop 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. Macewen’s 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.