HYDROCEPHALUS

water in the brain is characterized by an accumulation of CSF in the

ventricles of the brain. Hydrocephalus may result from overproduction of
CSF, obstruction of the flow of CSF within the ventricular system or a
decrease in the obstruction of the flow of CSF within the ventricular system,
or a decrease in the absorption of CSF out of the ventricles. Hydrocephalus
can be apparent with a sonogram before birth or it may develop in
adulthood. In adults, hydrocephalus may develop suddenly after a head
injury or slowly in response to a growing tumor.

Risk Factors

Genetic defect - Congenital hydrocephalus id associated with dysfunctional

motile cilia represent the underlying pathogenetic mechanism in 8 of the 12
lines. Other researchers suggested that Although hydrocephalus is usually
a sporadic disorder, there exist some rare but well delineated syndroms, in
which recurrence is to be expected. The here presented Bickers-Adams-
Syndrome of sex linked hydrocephalus is caused by congenital aqueductal
stenosis.

Meningitis - Hydrocephalus is associated with the complications of

tuberculous meningitis (TBM) occurred in up to 85% of children with the
disease. It is more severe in children than in adults. It could be either of the
communicating type or the obstructive type with the former being more
frequently seen.

Neural tube defect - Congenital hydrocephalus is a relatively uncommon

abnormality in population malformation surveys accounting for between
four and ten out of every 10,000 births. In the review of Sixty cases of 28
with NTD (group 1) and 32 without NTD (group 2). The groups were similar
in terms of maternal and child variables at birth and hospitalization days
during the 1st year of life.

Intraventricular hemorrhage - Intraventricular hemorrhage and post

hemorrhagic hydrocephalus are common causes of neonatal morbidity and
mortality among preterm and low-birth weight infants (PT-LBWIs), but
Intraventricular hemorrhage in PT-LBWIs remains a significant problem,
particularly when it is associated with PHH leading to long-term
neurological impairment and decreased survival.

PTB Deficiency - Polypyrimidine tract-binding protein (PTB) is a well-

characterized RNA-binding protein and known to be preferentially
expressed in neural stem cells (NSCs) in the central nervous system. Dr.
Shibasaki T and the research team at the Center for Experimental Medicine
and Systems Biology. showed that PTB depletion in the dorsal
telencephalon is causally involved in the development of HC and that PTB
is important for the maintenance of AJs in the NSCs of the dorsal
telencephalon.

Tumors - Tumor-associated hydrocephalus is common in primary pediatric

brain tumors. In the study of a total of 56.7% of cases
presented hydrocephalus, including hydrocephalus that occurred at tumor
diagnosis (51.5%), and hydrocephalus developed after tumor diagnosis
(5.1%). At tumor diagnosis, the hydrocephalus was mainly obstructive type
(98%) and rarely communicating type (1.9%). Definite shunting procedures
in this series comprised of ventriculoperitoneal (VP) shunt in 54.4%,
endoscopic third ventriculostomy (ETV) in 10.9%, subduroperitoneal (SP)
shunt in 4.8%, septostomy in 0.7%, lumboperitoneal shunt in 0.6%, and
ventriculoatrial shunt in 0.1% of patients with hydrocephalus.

Traumatic head injury - Post-traumatic hydrocephalus (PTH) is considered

a frequent complication after severe head injury (HI). External
hydrocephalus is a well-established entity in infants which is benign and
usually resolves without shunting. The term External Hydrocephalus has
also been used to describe the presence of extra ventricular cerebrospinal
fluid (CSF) collections accompanied by hydrocephalus, particularly in cases
of adults suffering from aneurysmal subarachnoid hemorrhage and severe
head injuries Several other terms have been used to describe this entity [7]
which has led to confusion about this disease.

Subarachnoid hemorrhage - Acute hydrocephalus as a consequence

of subarachnoid hemorrhage is a relatively frequent problem. Dr. Suarez-
Rivera O. at the National Institute of Neurology and Neurosurgery, Mexico
City, indicated that that acute hydrocephalus is present in 20% of patients
with subarachnoid hemorrhage. One third of them may be asymptomatic on
admission; 50% of those who have clinical hydrocephalus recover
spontaneously within the first 24 h.

Pathophysiology

In noncommunicating hydrocephalus, the obstruction occurs most

frequently between the third and fourth ventricles, at the aqueduct of
Sylvius, but it can also occur at the outlets of the fourth ventricles (foramina
at Luschka and Magendie) or rarely, at the foramen of Monro. This
obstruction may result from faulty fetal development, infection (syphilis,
granulomatous diseases, meningitis), a tumor, a cerebral aneurysm or a
blood clot (after intracranial hemorrhage).

In communicating hydrocephalus, faulty absorption of CSF may result

from surgery to repair a myelomeningocele, adhesions between meninges
at the base of the brain, or meningeal hemorrhage. Rarely, a tumor in the
choroid plexus causes overproduction of CSF and consequent
hydrocephalus.

In either type, both CSF pressure and volume increase. Obstruction

in the ventricles causes dilation, stretching. Underlying and disruption of the
lining. Underlying white matter atrophies. Compression of brain tissue and
cerebral blood vessels lead to ischemia and eventually cell death.

Signs and symptoms

Enlargement of the head clearly disproportionate to the infants

growth from the increased CSF volume.
Distended scalp veins from increased CSF pressure.
 Thin shiny, fragile Looking scalp skin from the increase CSF
pressure.
Underdeveloped neck muscle from increase weight of the head
Depressed orbital roof with downward displacement of eyes and
prominent sclerae from increased pressure.
High pitched, shrill cry, irritability and abnormal muscle tone in legs
from neurologic compression.
Projectile vomiting from increased ICP.
Skull widening to accommodate increased pressure
Decreased LOC from increasing ICP
Ataxia from compression of the motor areas.
Complications
Mental retardation, Impaired motor function, Vision loss, Brain
herniation, Death from increased ICP, Infection, Malnutrition, Shunt
infection, Septicemia, Paralytic Ileus, Adhesions, Peritonitis, and
Intestinal perforation.

Treatment

The only treatment for hydrocephalus is surgical correction by

insertion of ventriculoperitoneal shunt, which transport excess fluid from the
lateral ventricle into the peritoneal cavity. Ventriculoatrial (less common)
which drains fluid from the brains lateral ventricle into the right atrium of
the heart, where the fluid makes its way into the venous circulation.
Temporary external ventricular or lumbar drain for head trauma or
intracranial bleeding. Supportive care is also warranted.

VP shunting is a surgical procedure that primarily treats a condition called

hydrocephalus. This condition occurs when excess cerebrospinal fluid
(CSF) collects in the brains ventricles. CSF cushions your brain and
protects it from injury inside your skull. The fluid acts as a delivery system
for nutrients that your brain needs, and it also takes away waste products.
Normally, CSF flows through these ventricles to the base of the brain. The
fluid then bathes the brain and spinal cord before its reabsorbed into the
blood. When this normal flow is disrupted, the buildup of fluid can create
harmful pressure on the brains tissues, which can damage the brain.
Doctors surgically place VP shunts inside one of the brains ventricles to
divert fluid away from the brain and restore normal flow and absorption of
CSF.

Ventriculoatrial shunt placement enables cerebrospinal fluid (CSF) to flow

from the cerebral ventricular system to the atrium of the heart.
Ventriculoatrial shunt placement is indicated for hydrocephalus, which is
among the most common conditions encountered in neurosurgical practice.

Nursing Diagnosis

Ineffective cerebral perfusion related to increase intracranial

pressure.
Goal

Adequate central tissue perfusion.

Interventions

Assess factors related to individual situation for decreased cerebral

perfusion and potential for increased ICP
Closely assess and monitor neurological status frequently and
compare with baseline.
Maintain bedrest, provide quiet and relaxing environment, restrict
visitors and activities. Cluster nursing interventions and provide rest
periods between care activities. Limit duration of procedures.
Monitor status of fluid and I%O.
Give medication as prescribed.

Medications

Isosorbide Increase CSF.

Acetazolamide and Furosemide decrease CSF.

Nursing Diagnosis

Impaired gas exchange.

Goal
Oxygen saturation will be in 95%

Interventions

Elevated head of bed/ position client appropriately, provide airway

adjuncts and suction as indicated.
Encouraged frequent deep breathing/ coughing exercises.
auscultated breath sounds noting crackles, wheezes
Observe for nail beds; cyanosis in skin.

Nursing Diagnosis

Acute pain related to ICP.

Goal

Decrease pain.

Interventions

Perform pain assessment (location, duration, intensity, quality and

characteristic.
Position the client: HOB elevated with body in central position
Control environmental factors that may influence patients discomfort.
 Provide comfort measures.
Instruct the client to relax.
Encourage diversional activities
Instruct the client to avoid going to bathroom
Instruct the client to avoid activities that may contribute to Increase
Intracranial pressure.
SPINA BIFIDA

Is a congenital neural tube defect characterized by a failure of the

vertebral arches to close. This results in a cyst like protrusion of the
meninges alone or of the meninges and the spinal cord out of the vertebral
column. In the case of a meningocele, neural tissue is unexposed, and thus
neural deficits are absent or minor. With a myelomeningocele, the spinal
cord, in a cyst like protrusion with its nerves, suffers injury, inflammation
and scarring. The result is some loss of neural function, often including
paralysis. Another type of spina bifida is one in which minor irregularities in
the vertebral arches exist that are not obvious at birth. This is called spina
bifida occulta.

Risk Factors

Race. Spina bifida is more common among whites and Hispanics.

Sex. Girls are affected more often.

Family history of neural tube defects. Couples who've had one
child with a neural tube defect have a slightly higher chance of having
another baby with the same defect. That risk increases if two previous
children have been affected by the condition.
In addition, a woman who was born with a neural tube defect, or who
has a close relative with one, has a greater chance of giving birth to a
child with spina bifida. However, most babies with spina bifida are born
to parents with no known family history of the condition.

Folate deficiency. Folate (vitamin B-9) is important to the healthy

development of a baby. Folate is the natural form of vitamin B-9. The
synthetic form, found in supplements and fortified foods, is called folic
acid. A folate deficiency increases the risk of spina bifida and other
neural tube defects.

Some medications. Anti-seizure medications, such as valproic acid

(Depakene), seem to cause neural tube defects when taken during
pregnancy, perhaps because they interfere with the body's ability to use
folate and folic acid.

Diabetes. Women with diabetes who don't control their blood sugar
well have a higher risk of having a baby with spina bifida.

Obesity. Pre-pregnancy obesity is associated with an increased risk

of neural tube birth defects, including spina bifida.

Increased body temperature. Some evidence suggests that

increased body temperature (hyperthermia) in the early weeks of
pregnancy may increase the risk of spina bifida. Elevating your core
body temperature, due to fever or the use of saunas or hot tubs, has
been associated with increased risk of spina bifida.
Pathophysiology

Neural tube defects are the result of a teratogenic process that

causes failed closure and abnormal differentiation of the embryonic neural
tube. Neural tube defects occur between the 17th and 30th day of
gestation, at a time when the mother may not be aware that she is
pregnant and the fetus is estimated to be about the size of a grain of rice.

The most common neural tube defects are anencephaly and

myelomeningocele. Anencephaly results from failed closure of the rostral
end of the neural tube, resulting in incomplete formation of the brain and
skull.

Myelomeningocele
Spina bifida cystica causes a problem when the meningeal cyst
(meningocele) includes cord tissue extending into the cyst (in which case, it
is a myelomeningocele). The condition is also of particular concern when
the neural tube is completely open and the ependymal layer is exposed as
a myelocele (or myeloschisis). A meningocele alone may cause no
neurologic problems if the cord is confined to the vertebral canal.
Myelomeningocele results from failed closure of the caudal end of the
neural tube, resulting in an open lesion or sac that contains dysplastic
spinal cord, nerve roots, meninges, vertebral bodies, and skin (see the
image below). The anatomic level of the myelomeningocele sac roughly
correlates with the patient's neurologic, motor, and sensory deficits.

Myelomeningocele is associated with abnormal development of the

cranial neural tube, which results in several characteristic CNS anomalies.
The Chiari type II malformation is characterized by cerebellar hypoplasia
and varying degrees of caudal displacement of the lower brainstem into the
upper cervical canal through the foramen magnum. This deformity impedes
the flow and absorption of cerebrospinal fluid (CSF) and
causes hydrocephalus, which occurs in more than 90% of infants with
myelomeningocele.

Cerebral cortex dysplasia, including heterotopias, polymicrogyria,

abnormal lamination, fused thalami, and corpus callosum abnormalities,
also occurs frequently. In addition, mesodermal structures surrounding the
neural tube, such as the vertebra and ribs, may be malformed.

Signs and symptoms

Spina Bifida Occulta

Occulta is the mildest form of the disease. Vertebrae are malformed but the
spinal cord and its covering are undamaged. The condition typically doesn't
cause disability or symptoms. About 5 to 10 percent of the population may
have spina bifida occulta, but are unaware of it. In many cases, the only
visible signs are an abnormal tuft of hair, birthmark or dimple at the defect.
Spina Bifida Meningocele
Meningocele is more severe. The meninges membranes that cover and
protect the spinal cord protrude from the spinal opening and form a fluid-
filled sac visible on a baby's back. The sac may be covered by a thin layer
of skin. Some babies have few or no symptoms, while others experience
complications, such as partial paralysis and urinary and bowel dysfunction.
Spina Bifida Myelomeningocele
Myelomeningocele is the most severe form of the disease. The meninges
and spinal cord are exposed through the opening in the spine. Babies born
with this condition may develop a variety of health problems, including
partial or complete paralysis below the spinal cord, urinary and bowel
dysfunction, hydrocephalus or excessive fluid in the brain and mental
retardation, as well as impaired educational, social and psychological
development.
Women who are pregnant with a fetus affected with spina bifida don't
experience symptoms. However, there are several tests available to detect
spina bifida before a baby is born.

Diagnostic Test
Maternal Serum alpha-fetoprotein test
Ultrasound
Amniocentesis
Medical Management
Anticholinergics
Tricydic Depressants
Alpha_adrenergic antagonists
Nursing Diagnosis

Impaired physical mobility r/t motor paralysis

Goal

Client can participate in an exercise program, increase muscle

strength and client indicates action to improve mobility.

Interventions

Teach parents to know about prevent children from dangerous

objects.
Demonstrate parents that some games that do not cause injury.
Provide support to children in order not to feel inferior to his condition.

Nursing Diagnosis

Urinary incontinence r/t Visceral paralysis

Goal
Client urination normal in number and frequency.

Interventions

Assess the level of incontinence and voiding patterns.

Provide care to the client's skin wet with urine (wipe warm water then
wipe dry and give the powder).
Instruct the client's mother to check diapers often, if wet immediately
replaced.
Collaboration with the medical team in giving drugs (eg
anticholinergics).

Nursing Diagnosis

Risk for injury related to spastic paralysis

Goal

the patient's parents know about the things that lead to injury.

Interventions

Teach or suggest to parents to prevent children from dangerous

objects that could cause injury
Demonstrate to parents that some games do not cause injury.
Provide health education to parents regarding drugs or handling of
the first case of injury in children.
Provide support to children in order not to feel inferior to his condition.