Hydrocephalus

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:
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.
Neural Tube Defects
Definition

anencephaly

Neural tube defects (NTDs) are birth defects of either the brain or spinal cord. This group of
disorders is characterized by an opening in the brain or spinal cord occurring at an early stage of
human development.

Incidence
NTDs are one of the most common birth defects.

 Female predominance is observed. About 60-70% of all cases are females.

 The incidence of neural tube disorders have fallen dramatically in recent years from 3 cases
in every 1000 births to only 0.6 cases in every 1000 births.

Risk Factors
 Cigarette smoking and maternal exposure to cigarette smoke
 Maternal diabetes
 Maternal obesity

Causes
 Polygenic inheritance pattern
 Poor maternal nutrition
 Maternal diet deficient in folic acid
 Intake of folate antimetabolites (methotrexate)
 Use of teratogens
Review of the related anatomy and physiology
The central nervous system is the first observable structure in a human embryo. This embryo passes
through 23 stages of development after conception. Two processes work in order to form the CNS:

1. Primary neurolation
2. Secondary neurolation
Primary neurolation is the formation of the neural structures into a tube. This forms the brain and
the spinal cord. Secondary neurolation is the formation of the lower spinal cord. This gives rise to the
lumbar and sacral elements.

Types of Neural Tube disorders

 Anencephaly – absence of the cerebral hemispheres. Closure of the anterior neuropore
occurs at stage 11 at about 23-26 days age of gestation. Failure of this point to close results to
anencephaly. Children with this disorder cannot survive because of the absence of cerebral
function. These children may only survive for a number days because the respiratory and
cardiac centers are located in the intact medulla.
 Microcephaly – characterized by a slow brain growth. Generally, infants with this disorder
are cognitively challenged because of the lack of functioning brain tissue. The head
circumference of affected neonates is decreased.
 Spina Bifida Occulta – occurs when the posterior laminae of the vertebra fail to fuse at 26-
28 days age of gestation. The most common site is the fifth lumbar or sacral level though it may
occur at any point along the spinal canal.
 Meningocele – herniation of the meninges through the unformed vertebrae of the spinal
cord. Generally, it occurs at the lumbar area but it can be present anywhere along the spinal
canal. It is characterized by an appearance of an orange-shaped mass at the center of the back.
 Myelomeningocele – herniation of the spinal cord and meninges through the unformed
vertebrae. The spinal cord ends at the pint of protrusion, thus, motor and sensory function
beyond the point of herniation is absent.