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 of hydrocephalus 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 NTD’s.
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:
Abnormal rate of head growth
Bulging fontanelle
Tense anterior fontanelle (often bulging and nonpulsatile)
 Dilated scalp veins
Macewen’s sign (“cracked pot”)
Frontal bossing
Setting sun sign
Sluggish and unequal pupils
Irritability and lethargy with varying LOC
Abnormal infantile reflexes
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:
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).
CT scan will diagnose most cases postnatally.
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.

pathophysiology
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 ti is absorbed.
Causes of Hydrocephalus are varied but result in either impaired absorption of CSF
within the arachnoid space (formerly referred to as communicating hydrocephalus) or
obstruction to the flow of CSF through the ventricular system (formerly referred as
noncommunicating hydrocephalus.
Most cases of obstruction are the result of developmental malformations; other causes
include neoplasms, infection and trauma. Obstruction to the normal flow can occur at any
point in the CSF pathway, which produces increased pressure and dilation of the
pathways proximal to the site of obstruction.
Impaired absorption can result form meningitis, prenatal maternal infections, meningeal
malignancy (secondary to leukemia or lymphoma), an arachnoid cyst, and tuberculosis.