HYDROCEPHALUS

INTRODUCTION
The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning
head. Although hydrocephalus was once known as "water on the brain," the "water" is actually
cerebrospinal fluid (CSF)--a clear fluid that surrounds the brain and spinal cord. About 20 ml of CSF is
produced every hour.
Who gets this hydrocephalus? The number of people who develop hydrocephalus or who are currently
living with it is difficult to establish since there is no national registry or database of people with the
condition. However, experts estimate that hydrocephalus affects approximately 1 in every 500 children.

DEFINITION
The term hydrocephalus implies an increase in the volume of cerebrospinal fluid (CSF) occupying the
cerebral ventricles. This is usually a result of impaired absorption but it may occasionally be due to
excessive secretion.
OR
Hydrocephalus is the build-up of fluid in the cavities (ventricles) deep within the brain.
OR
Hydrocephalus is a condition caused by an imbalance in the production and absorption of CSF in the
ventricular system. When production is greater than absorption, CSF accumulates within the
ventricular system, usually under increased pressure, producing passive dilation of the ventricles.
The balance between production and absorption of CSF is critically important. Ideally, the fluid is almost
completely absorbed into the bloodstream as it circulates; however, there are circumstances which, when
present, will prevent or disturb the production or absorption of CSF, or which will inhibit its normal flow.
When this balance is disturbed, hydrocephalus is the result.

incidence

Incidence and prevalence data are difficult to establish as there is no existing national registry or database
of people with hydrocephalus and closely associated disorders; however, hydrocephalus is believed to
affect approximately 1 in every 500 children. At present, most of these cases are diagnosed prenatally, at
the time of delivery, or in early childhood. Advances in diagnostic imaging technology allow more accurate
diagnoses in individuals with atypical presentations, including adults with conditions such as normal
pressure hydrocephalus.

In the United States, a little over 1 in 1000 births are affected by hydrocephalus.
Hydrocephalus is one of the most common "birth defects" and afflicts in excess of 10,000 babies

each year.
Studies by the World Health Organization show that one birth in every 2,000 result in

hydrocephalus.
There are 70,000 discharges a year from hospitals in the United States with a diagnosis of

hydrocephalus.
More than 50% of hydrocephalus cases are congenital.
As many as 75% of children with hydrocephalus will have some form of motor disability.
Over the past 25 years, death rates associated with hydrocephalus have decreased from 54% to
5%; intellectual disability has decreased from 62% to 30%.
About 80% of hydrocephalus patients are born with other defects. Other medical conditions usually

associated with Hydrocephalus include:

Arachnoid Cysts.
Brain Injury.
Dandy-Walker Syndrome.
Head Trauma.
Meningitis.
Porencephaly.
Tumours.
Spina Bifida.
Hydrocephalus occurs in 70 to 90% of children with the most severe form of Spina Bifida.

ANATOMY & PHYSIOLOGY

Diagram of right lateral view of brain
VENTRICLES OF THE BRAIN: The brain contains four irregular shaped cavities or ventricles, containing cerebrospinal fluid. They are:

Right and left lateral ventricles.

Third ventricles
Fourth ventricles.

THE LATERAL VENTRICLES:

These cavities lie within the cerebral hemisphere, one on each side of the median plane just below
the corpus callosum. They are separated from each other by a thin membrane, the septum lucidum, and are
lined with ciliated epithelium. They communicate with the third ventricle by interventricular foramina.
THE THIRD VENTRICLES:
The third ventricle is a cavity situated below the lateral ventricles, between the two parts of the
thalamus. It communicates with the fourth ventricle by a canal, the cerebral aqueduct.
THE FOURTH VENTRICLE:
The fourth ventricle is a diamond shaped cavity situated below and behind the third ventricle, between
the cerebellum and pons. It is continuous below with the central canal of the spinal cord and communicates
with the sub-arachnoid space by foramina in its roof. Cerebrospinal fluid enters the subarachnoid space
through these openings and through the open distal end of the central canal of the spinal cord.
CEREBROSPINAL FLUID:
Cerebrospinal fluid (CSF) is a clear, colourless liquid composed primarily of water that protects the brain
and spinal cord from chemical and physical injuries. It also carries small amounts of oxygen, glucose, and
other needed chemicals from the blood to neurons and neuroglia. CSF continuously circulates through
cavities in the brain and spinal cord and around the brain and spinal cord in the subarachnoid space (the
space between the arachnoid mater and pia mater). The total volume of CSF is 80 to 150 ml in an adult.
CSF contains small amounts of glucose, proteins, lactic acid, urea, cations (Na+, K+, Ca2+, Mg2+), and
aninos (Cl- and HCO3-); it also contains some white blood cells.
The four CSF- filled cavities within the brain, which are called ventricles. There is one lateral ventricle
in each hemisphere of the cerebrum.
Anteriorly, the lateral ventricles are separated by a thin membrane, the septum pellucidum. The third
ventricle is a narrow slit like cavity along the midline superior to the hypothalamus and between the right
and left halves of the thalamus. The fourth ventricle lies between the brain stem and the cerebellum.
It is secreted into each ventricle of the brain by choroid plexuses. These are vascular areas where there is a
proliferation of blood vessels surrounded by ependymal cells in the lining of ventricle walls. CSF passes

back into the blood through tiny diverticula of arachnoid mater, called arachnoid villi, which projects into
the venous sinuses. The movement of CSF from the subarachnoid space to venous sinuses depends upon
the difference in pressure on each side of the walls of the arachnoid villi, which act of one-way valves.
When CSF pressure is higher than venous pressure, CSF passes into the blood and when the venous
pressure is higher the arachnoid villi collapse, preventing the passage of blood constituents into the CSF.
There may also be some reabsorption of CSF by cells in the walls of the ventricles.
From the roof of the fourth ventricle CSF flows through foramina into the subarachnoid space and
completely surrounds the brain and spinal cord. There is no intrinsic system of CSF circulation but its
movement is aided by pulsating blood vessels, respiration and changes of posture.
It is secreted at a rate of about 0.5 ml/min i.e. 720 ml per day. The volume remains fairly constant
at about 150 ml, as absorption keeps pace with tube attached to a lumbar puncture needle inserted into the
subarachnoid space above or below the 4th lumbar vertebrae. The pressure remains fairly constant at about
10 cm H2O when the individual is lying on his side and about 30 cm H2O when sitting up. If the brain is
enlarged by, e.g., haemorrhage or tumour, some compensation is made by a reduction in the amount of
CSF. When the volume of brain tissue is reduced, such as in degeneration, the volume of CSF is increased.
FUNCTIONS OF CSF
The CSF has three basic functions:
1. Mechanical protection: - CSF serves as a shock-absorbing medium that protects the delicate tissue
of the brain and spinal cord from jolts that would otherwise cause them to hit the bony walls of the
cranial cavity and vertebral canal. The fluid also buoys the brain so that it floats in the cranial
cavity.
2. Homeostatic function: - The pH of the CSF affects pulmonary ventilation and cerebral blood flow,
which is important in maintaining homeostatic controls for brain tissue. CSF also serves as a
transport system for polypeptide hormones secreted by hypothalamic neurons that act at remote sites
in the brain.
3. Circulation: - CSF is a medium for minor exchange of nutrients and waste products between the
blood and adjacent nervous tissue.
FORMATION OF CSF IN THE VENTRICLES
The majority of CSF production is from the choroid plexuses, networks of blood capillaries in the walls of
the ventricles. Ependymal calls joined by tight junctions over the capillaries of the choroid plexus. Selected
substances (mostly water) from the blood plasma, which are filtered from the capillaries, are secreted by

the ependymal cells to produce the cerebrospinal fluid. This secretory capacity is bidirectional and accounts
for continuous production of CSF and transport of metabolites from the nervous tissue back to the blood.
Because of the tight junction between ependymal cells, materials entering CSF from choroid
capillaries cannot leak between these cells; instead, they must pass through the ependymal cells. This
blood-cerebrospinal fluid barrier permits certain substances to enter the CSF but excludes other, protecting
the brain and spinal to the blood-brain barrier, which is formed mainly by tight junctions of brain capillary
endothelial cells, the blood-cerebrospinal fluid barrier is formed by tight junctions of ependymal cells.
CIRCULATION OF CSF
The CSF formed in the choroid plexuses of each lateral ventricle flows into the third ventricle through two
narrow, oval openings, the interventricular foramina. More CSF is added by the choroid plexus in the roof
of the third ventricle. The fluid then flows through the aqueduct of the midbrain, which passes through the
midbrain, into the fourth ventricle. The choroid plexus of the fourth ventricle contributes more fluid. CSF
enters the subarachnoid space through three openings in the roof of the fourth ventricle; a single median
aperture and paired lateral aperture, one on each side. CSF then circulates in the central canal of the spinal
cord and in the subarachnoid space around the surface of the brain and spinal cord.
CSF is gradually reabsorbed into the blood through arachnoid villi, finger like extensions of the
arachnoid that project into the dural venous sinuses, especially the superior sagittal sinus (A cluster of
arachnoid villi is called an arachnoid granulation). Normally, CSF is reabsorbed as rapidly as it it formed
by the choroid plexuses, at a rate of about 20 ml/hr. (480ml/day). Because the rates of formation and
reabsorption are the same, the pressure of CSF normally is constant. For the same reason, the volume of
CSF remains constant.
Cerebrospinal fluid plays an important role in brain function by:
Keeping the brain buoyant, allowing the relatively heavy brain to float within the skull
Cushioning the brain to prevent injury
Removing waste products of the brain's metabolism
Flowing back and forth between the brain cavity and spinal column to maintain a constant pressure within
the brain compensating for changes in blood pressure in the brain
Excess cerebrospinal fluid in the ventricles occurs for one of the following reasons:

 Obstruction: - The most common problem is a partial obstruction of the normal flow of
cerebrospinal fluid, either from one ventricle to another or from the ventricles to other spaces
around the brain.
Poor absorption: - Less common is a problem with the mechanisms that enable the blood vessels
to absorb cerebral spinal fluid. This is often related to inflammation of brain tissues from disease or
injury.
Overproduction: - Rarely, the mechanisms for producing cerebrospinal fluid create more than
normal and more quickly than it can be absorbed.

Types of hydrocephalus
Hydrocephalus can be identified in one of two ways - Congenital and Acquired.
Congenital Hydrocephalus:
Congenital Hydrocephalus results from a complex interaction of genetic and environmental factors and is
present at birth. It is important to remember that the term genetic does not imply that it is hereditary.
Often the exact cause of congenital Hydrocephalus cannot be determined. Though it
might not be recognised and diagnosed immediately, congenital Hydrocephalus is often diagnosed before
birth through routine ultrasound.
Acquired Hydrocephalus:
Acquired Hydrocephalus develops after birth as a result of neurological conditions. This type of
Hydrocephalus can affect individuals of all ages and may be caused by head trauma, brain tumour, cyst,
intraventricular haemorrhage or infection of the central nervous system.
Within both of these areas, congenial and acquired, Hydrocephalus can be described as
communicating or non-communicating. Communicating Hydrocephalus occurs when the flow of CSF is
blocked after it exits the ventricles. This form is called communicating because the CSF can still flow
between the ventricles, which remain open. Non-communicating Hydrocephalus - also called "obstructive"
Hydrocephalus occurs when the flow of CSF is blocked along one or more of the narrow passages
connecting the ventricles.
There are two other forms of Hydrocephalus which do not fit exactly into the categories mentioned above
and primarily affect adults: Benign External Hydrocephalus and Normal Pressure Hydrocephalus.
1. Congenital Hydrocephalus

This means that Hydrocephalus is present at birth. It is important to remember that this term does not imply
that it is hereditary. Often the exact cause of Congenital Hydrocephalus cannot be determined but known
causes can include:

Aqueductal Stenosis (non-communicating): - The most common cause of congenital

Hydrocephalus is an obstruction called aqueductal stenosis. When the long, narrow passageway
between the third and fourth ventricles (see What is Hydrocephalus diagram) is narrowed or
blocked, perhaps because of infection, haemorrhage or a tumour. Fluid accumulates "upstream"
from the obstruction, producing Hydrocephalus.

Neural Tube Defect or NTD (communicating)

An open NTD, where the spinal cord is exposed at birth and is often leaking CSF, is often referred
to as Spina Bifida (see What is Spina Bifida). This kind of NTD causes part of the cerebellum and
the fourth ventricle to push downward through the opening at the base of the skull into the spinal
cord area, blocking CSF's flow out of the fourth ventricle and producing Hydrocephalus.

Arachnoid Cysts (non-communicating)

Arachnoid Cysts may occur anywhere in the brain. In children, they're often located at the back of
the brain and in the area of the third ventricle. These cysts are filled with CSF and lined with the
arachnoid membrane. Some arachnoid cysts are self-contained, while others are connected with the
ventricles or the subarachnoid space. The fluid trapped by the cysts may block the CSF pathways,
producing Hydrocephalus.

Dandy-Walker Syndrome (non-communicating)

In Dandy-Walker syndrome the fourth ventricle becomes enlarged because its outlets are partly or
completely closed, and part of the cerebellum fails to develop. Dandy-Walker syndrome may also
be associated with irregular development in other parts of the brain and sometimes leads to
aqueductal stenosis. In some instances, two shunts are placed in the child's ventricles - one in the
lateral ventricle and another in the fourth ventricle to manage the Hydrocephalus.

Arnold-Chiari Malformation (communicating)

There are two types of Arnold-Chiari malformation. Both types occur in the bottom of the brain
stem where the brain and spinal cord join. The lowest portion of the brain is displaced and is lower
than normal pushing down into the spinal column. (For further information see What is Chiari
Malformation)

2. Acquired Hydrocephalus
This means that Hydrocephalus has occurred after birth and can be caused by:

Intraventricular Hemorrhage (communicating)

An intraventricular haemorrhage, which most frequently affects premature new-borns, may cause
an acquired form of Hydrocephalus. When small blood vessels alongside the ventricular lining
rupture, blood may block or scar the ventricles or plug the arachnoid villi. The arachnoid villi is
located in the second layer covering the brain which allows CSF to be absorbed. When the CSF
can't be absorbed, Hydrocephalus results.

Meningitis (communicating)
Meningitis is an inflammation of the membranes of the brain and spinal cord. Caused by a bacterial
or (less frequently) viral infection, meningitis can scar the delicate membranes (meninges) that line
the CSF pathway. An acquired form of Hydrocephalus may develop if this scarring obstructs the
flow of CSF as it passes through the narrow ventricles or over the surfaces of the brain in the
subarachnoid space.

Head Injury (communicating)

A head injury can damage the brain's tissues, nerves, or blood vessels. Blood from ruptured vessels
may enter the CSF pathway, causing inflammation. Sites of CSF absorption might then be blocked
by scarred membranes (meninges) or by blood cells. The CSF flow is restricted and Hydrocephalus
develops.

Brain Tumours (non-communicating)

In children, brain tumours most commonly occur in the back of the brain (posterior fossa). As a
tumour grows, it may fill or compress the fourth ventricle, blocking the flow of CSF and causing
Hydrocephalus. A tumour somewhere else in the brain might also block or compress the ventricular
system.

Ventriculitis (non-communicating)
Ventriculitis is a disease causing inflammation and or infection of the ventricles. It is most common
in infants and is often an extension of meningitis.

Choroid plexus papilloma (communicating)

A Choroid plexus papilloma (CPP) is a rare, slow-growing, tumour that is commonly located in the
ventricular system of the choroid plexus (see What is Hydrocephalus diagram). It may obstruct the
cerebrospinal fluid flow, causing increased intracranial pressure and Hydrocephalus.

Prematurity (communicating)
Babies born prematurely are at risk of Hydrocephalus as their brain is still developing. The area
which lies just beneath the lining of the ventricles in the brain is particularly important in this
development as the activity in this area has a plentiful blood supply. The blood vessels are very

fragile at this time and can easily burst if the baby suffers too large a swing in blood pressure or
becomes severely ill from other causes. If these complications occur, then the baby may be at risk
of developing a haemorrhage. This can lead to a blood clot developing, which in some cases is big
enough to break through the wall of the ventricle. Should the clot block the flow of CSF, the baby
will develop Hydrocephalus. The blockage may be temporary or permanent. Even if a blood clot
does not develop, the blood cells from the haemorrhage can cause blockage and Hydrocephalus can
occur.
Benign External Hydrocephalus (communicating)
Benign External Hydrocephalus (also referred to as External Hydrocephalus) occurs when an
accumulation of CSF is found outside the brain, which usually presents itself at birth or soon
thereafter. The infants head size will increase, but scans show no international difficulties in the
ventricles or pathways. This condition usually corrects itself within 18 months of age.
Normal Pressure Hydrocephalus (Non-communicating)
Normal Pressure Hydrocephalus can happen to people at any age, but it is most common among the
elderly. It may result from a subarachnoid haemorrhage, head trauma, infection, tumour, or
complications of surgery. However, many people develop Normal Pressure Hydrocephalus even
when none of these factors are present for reasons that are unknown. (For further information see
What is Normal Pressure Hydrocephalus)

ETIOLOGY
Hydrocephalus is caused by an imbalance between how much cerebrospinal fluid is produced and how
much is absorbed into the bloodstream.
Hydrocephalus may result from

Inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) or
Developmental disorders (such as those associated with neural tube defects including spina bifida

and encephalocele).
Complications of premature birth such as intraventricular haemorrhage,
Diseases such as meningitis, tumours, traumatic head injury, or subarachnoid hemorrhage, which
block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF within
the cisterns.

RISK FACTORS

In many cases, the exact event leading to hydrocephalus is unknown. However, a number of developmental
or medical problems can contribute to or trigger hydrocephalus.
New born
Hydrocephalus present at birth (congenital) or shortly after birth may occur because of any of the
following:
Abnormal development of the central nervous system that can obstruct the flow of cerebral spinal fluid
Bleeding within the ventricles, a possible complication of premature birth
Infection in the uterus during a pregnancy, such as rubella or syphilis that can cause inflammation in foetal
brain tissues
Other contributing factors
Other factors that can contribute to hydrocephalus among any age group include:
Lesions or tumours of the brain or spinal cord
Central nervous system infections, such as bacterial meningitis or mumps
Bleeding in the brain from stroke or head injury
Other traumatic injury to the brain

PATHOPHYSIOLOGY of hydrocephalus
Due to etiological factors such as imbalance of secretion and absorption causes an increased accumulation
of CSF in the ventricles.

Ventriculomegaly (dilation)

Compress the brain substance against the surrounding rigid bony cranium
It occurs before fusion of the cranial sutures

Causes enlargement of the skull, Frontal enlargement , Depressed eyes

CLINICAL MANIFESTATION
Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to
the condition.
The signs and symptoms of hydrocephalus vary generally by age of onset.
Infants
1. Infant (Early)
Abnormality rapid head growth
Bulging fontanels (especially anterior) sometimes without head enlargement
Dilated scalp veins
Separated sutures
Macewen sign (cracked-pot sound on percussion)
Thinning of skull bones
2. Infancy (Later)
Frontal enlargement
Depressed eyes
Setting-sun sign (sclera visible above the iris)
Pupils sluggish, with unequal response to light
3. Infancy (General)
Irritability
Lethargy
Infant cries when picked up or rocked and quiets when allowed to lie still
Early infantile reflex acts may persist
Normally expected responses fail to appear
May display:

Change in level of consciousness

Lower extremity spasticity
Vomiting

Advanced cases:
Difficulty in sucking and feeding
Shrill, brief, high pitched cry
CHILDHOOD

Headache on awakening improvement following emesis or upright posture

Papilledema
Strabismus
Extrapyramidal tract signs (e.g. ataxia)
Irritability
Lethargy
Apathy
Confusion
Incoherence
Vomiting

Toddlers and older children

Among toddlers and older children, signs and symptoms may include:
Physical symptoms
Headache
Blurred or double vision
Physical signs
Abnormal enlargement of a toddler's head
Sleepiness
Difficulty remaining awake or waking up
Nausea or vomiting
Unstable balance
Poor coordination
Poor appetite
Seizures
Behavioural and cognitive changes
Irritability
Change in personality
Problems with attention

Decline in school performance

Delays or problems with previously acquired skills, such as walking or talking

DIAGNISTIC EVALUATION
Hydrocephalus is a disorder, not a disease, and therefore it is important to investigate the cause of
hydrocephalus.

1. Medical history
Accurate serial recording of the head circumference is essential for early diagnosis of hydrocephalus and
should be supported by serial USG. Increase in head circumference in first 3 months of life, more than 1
cm every 15 days and persistent widening of squamo-parietal sutures should arouse suspicion of
hydrocephalus.
2. Skull X-ray- Anteroposterior and lateral view reveals size, sutural separation, thinning of skull
3.
4.
5.
6.
7.

bone, shape of head, ossification, etc.

MRI/CT scan to determine the site of obstruction.
Ultrasonography: Hydrocephalus can be diagnosed at 10-20 weeks of intrauterine life.
CSF dynamic scan.
Angiography done to identify the obstruction.
Ventriculography may also be done to more clearly define the site of blockage to the flow of

cerebrospinal fluid.
8. Laboratory studies on the cerebrospinal fluid should also be done in order to determine whether
any infection is present.

Management
General principles
Management depends on how acute the deterioration is.
Drugs are usually used as a holding measure until the appropriate intervention can be carried out.
A lumbar puncture may be used in the management of acute deterioration if there is a
communicating hydrocephalus. Repeated lumbar punctures may avoid neurosurgery if the condition
is likely to resolve spontaneously.
Gradual deterioration allows for a more ample assessment and careful consideration of options.
Underlying causes such as tumours need to be identified and addressed.

Medical management
For mild hydrocephalus, acetazolamide 50-100 mg/kg/day in two or three divided doses is effective.
It reduces the secretion of CSF from choroid plexus.
Glycerol is used for the same purpose.
Diuretics such as furosemide 1mg/kg/day and mannitol are used to reduce the CSF production
Medication may help to defer surgery in order to stabilise the patient but medical treatment alone is
generally unsuccessful in long-term control of ICP. Furosemide and acetazolamide inhibit secretion
of CSF by the choroid plexus. Isosorbide promotes reabsorption.

Surgical management
Direct removal of an obstruction such as tumour.
Shunt Procedure
A shunt is a tube that drains fluid from one area to another space in the body, where the fluid is then
reabsorbed.
There are 4 different kinds of shunts:
1. Ventriculo-Peritoneal (V-P) shunt: Drains cerebrospinal fluid (CSF) from the ventricles into the
peritoneum (the space in the abdomen around the stomach and intestines).
There are three parts to a V-P shunt:
The proximal catheter: the part of the tube that is placed directly into the ventricle (fluid space) of the
brain.
A one-way valve is attached to the end of the proximal catheter. It controls the flow of CSF, keeping a
normal amount of fluid in the ventricle. This valve only allows fluid to leave the ventricle, preventing backflow.
The distal catheter is the end part of the shunt. The shunt is passed under the skin in the neck and chest,
and the end is placed in the peritoneum. Extra length is left to allow for growth.
2. Lumbo-Peritoneal (L-P) Shunt: This shunt is placed into the CSF fluid space below the spinal
cord, and drains into the abdomen.
3. Ventriculo-Atrial (V-A) shunt: Drains from the ventricle, into the right atrium of the heart.
4. Ventriculo-Pleural (V-Pleural) shunt: Drains from the ventricle into the lining of the lung.

If hydrocephalus is diagnosed, treatment will depend on the age of the child, the cause of the cerebral
spinal fluid build-up (whether from a blockage, overproduction of fluid, or another problem), and the
child's overall health.
Commonly used shunt is VP shunt (Ventriculoperitoneal shunt).
A shunt system consists of ventricular catheter, a flush pump, unidirectional flow valve and a distal
catheter. The valve is designed in such a way that it opens at a predetermined intraventricular pressure and
closes when the pressure falls below that level, thus the backflow of CSF is prevented. It only allows the
flow of CSF from ventricles to peritoneum not in the reverse direction.
Ventricular end of the tube is put in the lateral ventricle and the shunt tube is put in a skin
tunnel from scalp, behind the ear to upper abdomen. A small incision is put in the right hypochondrium,
peritoneum is opened and lower end of shunt is placed between diaphragm and liver.
Before the surgery:Following tests should be done:
CT scan: - This donut-shaped x-ray scanner shows us the amount of fluid inside the ventricles of the brain.
Ultrasound of the head (infants only): - sound waves are used to look at the amount of fluid in the
ventricles.
MRI (Magnetic Resonance Imaging): - radio waves and a magnetic field (created by a large magnet),
give a detailed picture of the brain.
Shunt survey: - a series of x-rays of the shunt tubing, which shows if the tubing is in the right position, is
disconnected or broken.
Shunt-o-gram: - this test uses a nuclear tracer to show movement of CSF through the shunt. The
radiologist will inject a small amount of tracer into the valve with a sterile needle. Pictures are then taken
as the fluid moves through the shunt.
A blood test: - All children having surgery must have a blood test before the surgery
Shunt procedures, which have been the standard of care for decades, involve surgically implanting one end
of a catheter (flexible tube) into a ventricle of the brain and placing the other end in the abdominal cavity,
chambers of the heart, or space around the lungs where fluid is drained and absorbed by the bloodstream. A
valve in the shunt system regulates flow to prevent over-draining and under-draining.

Infections are another side effect of shunting, and occur in 5%-10% of shunt operations. Kids will develop
typical signs of infection, like fever and neck stiffness, and may feel tenderness along the shunt or belly
pain. Most infections develop within the first several months after a shunt procedure and require temporary
removal of the device while a child receives intravenous antibiotics for up to 2 weeks.
Ventriculostomy
A second, increasingly more common treatment for hydrocephalus is an endoscopic third ventriculostomy.
During this procedure, a small opening is made in the bottom of the third ventricle (one of four ventricles
in the brain) to allow fluid to exit the brain.
This minimally invasive approach involves placing an endoscope (small lighted camera) inside the brain to
provide surgeons with a view of the surgical site on a computer monitor. Then, using very small
instruments, the doctor will make a tiny hole in the bottom of the third ventricle, where a thin membrane
separates the inside and outside of the brain. This new "evacuation route" permits fluid to drain normally
into the spaces outside the brain while bypassing any obstructions that are causing a backup, so the body
can reabsorb the CSF back into the bloodstream as it normally would.
When deemed appropriate, third ventriculostomies are the procedure of choice for kids older than 6 months
of age due to a higher efficacy rate and lower risk of infection than shunting. Those who undergo the
procedure have up to a 90% chance of long-term success, with little need for follow-up procedures.
Third ventriculostomies also have shown promise in new-borns and may be offered as a treatment to these
younger patients in the near future.
Complications of surgery
Both surgical procedures can result in complications. Shunt systems can stop draining cerebrospinal fluid
or poorly regulate drainage because of mechanical malfunctions, blockage or infections. Complications of
ventriculostomy include bleeding and infections.
Any failure requires prompt attention, surgical revisions or other interventions. Signs and symptoms of
problems may include:

Fever
Irritability
Drowsiness
Nausea or vomiting
Headache
Vision problems
Redness, pain or tenderness of the skin along the path of the shunt tube

Abdominal pain when the shunt valve is in the abdomen

Recurrence of any of the initial hydrocephalus symptoms

Nursing management
ASSESSMENT: Assess the head circumference of child.
Assess the amount of CSF fluid.
Assess the nutritional status of the child.
Assess neurologic status.
Assess knowledge of disorder, causes, treatment, and expected outcome.

NURSING DIAGNOSIS
1. Nursing Diagnosis: - Risk for injury related to increased intracranial pressure.
Goal: - To maintain normal intracranial pressure.
Intervention:1. Check the head circumference (occipitofrontal) daily.
2. Gently palpate the fontanelle and suture lines for size, signs of bulging, tenseness and
separation.
3. Monitor vital signs frequently
4. Observe for signs and symptoms of increased ICP such as irritability, lethargy, vomiting and
5.
6.
7.
8.

poor feeding.
Inform the parents about the importance of surgery (shunt) if indicated.
Prepare the child adequately for the shunt procedure.
Offer small frequent feeds.
Administer anti-seizure prophylaxis as prescribed.

2. Nursing Diagnosis: - Imbalanced the nutritional status of the child.

Goals: - To maintain adequate nutritional status.
Intervention: 1.
2.
3.
4.
5.
6.
7.
8.

Observe the nutritional status of the child.

Observe for signs of dehydration.
Provide small frequent feeds.
Serve food which the child likes.
Administer antiemetic half an hour before feeds.
Provide food which contains high calorie and protein.
Administer medications as ordered at correct time.
Encourage solid food then liquid food.

9. Handling the baby for diagnostic procedures just before or after feed may induce vomiting. So
schedule feed accordingly.
POSTOPERATIVE NURSING CARE
3. Nursing Diagnosis: - Risk for injury related to increased intracranial pressure.
Goal: - To maintain normal intracranial pressure
Intervention: 1.
2.
3.
4.
5.
6.

Position the child on the unoperated side.

Keep the child flat on the bed.
Avoid hyperextension and hyperflexion of the neck.
If there is increased ICP elevate the head end.
Observe for signs of increased ICP, observe the functioning of the shunt.
Provide oral feeds only after determining the presence of bowel sounds.

4. Nursing Diagnosis: - Deficient knowledge of parents regarding the care of shunt.

Goal: - To facilitates adequate knowledge.
Intervention:1. Explain to the parents about the shunt procedure done to the child.
2. Make them feel for the shunt behind the ear.
3. Teach them how to pump the shunt if necessary.
4. Teach them the signs of shunt malfunction or shunts infection.
5. Ask to report any rise in body temperature.
6. Tell them to prevent fall of the child.
7. Advise them to take extra care while transporting the child.
8. Explain to come for follow-up regularly and shunt revisions as necessary.
9. Enough to wear an ID stating the name of shunt etc.
10. Refer to community agencies for support and guidance.
5. Nursing Diagnosis: - risk for injury related to shunt complications.
Goal: - To remain free from complications.
Intervention: 1. Observe signs of increased intracranial pressure.
2. Check pupil reaction.
3. Observe for sign of meningitis, wound infection or any other infection.
4. Inspect the incision site for leakage.
5. Check vital signs.
6. Check the shunt functioning.
7. Observe for abdominal distension.
8. Monitor intake & output.
9. If abdominal distension is present, stop oral feeds and start intravenous fluids.
10. Administer IV fluids.
11. Provide meticulous skincare.

12. Administer antibiotics as ordered.

6. Nursing Diagnosis: - Ineffective family coping related to life threatening problem of infant.
Goal: - To make effective family coping.
Intervention: 1. Strengthening family coping by teaching daily care of V-P shunt, as needed for the particular
shunt, like pumping the shunt, positioning the child as directed( initially flat to prevent
excessive CSF drainage then gradual elevation of head of childs bed to 30 to 45 degree) and
assessing for excessive drainage of CSF.
2. Parent should also be informed about the signs of increased ICP, which indicate shunt
malfunctions.
7. Nursing Diagnosis: - Altered cerebral tissue perfusion related to increase ICP.
Goal: - To maintain cerebral tissue perfusion.
Intervention: 1. Assess for and report signs and symptoms of decreased cerebral tissue perfusion:

dizziness
visual disturbances (e.g. blurred or dimmed vision, diplopia, change in visual field)
aphasia
irritability and restlessness
decreased level of consciousness
paresthesias, weakness, paralysis.

2. Implement measures to maintain adequate cerebral tissue perfusion:

A. Perform actions to prevent and treat increased intracranial pressure.
B. If client is hypotensive, perform actions to improve cerebral blood flow (e.g. administer
prescribed sympathomimetic agents)
C. Administer calcium-channel blockers (e.g. nimodipine) if ordered to reduce cerebral
vasospasm (the calcium that is released by the injured neural cells can cause vasospasm)
D. Prepare client for surgical intervention (e.g. evacuation of hematoma, ligation of bleeding
vessels) if planned.

3. Consult physician if signs and symptoms of decreased cerebral tissue perfusion persist or worsen.

Complication
Hydrocephalus can be complicated with seizures, herniation of brain, persistent increased ICP,
developmental delay, infections, neurological deficits, motor and intellectual handicaps, visual problems,
aggressive and delinquent behaviour.
Shunt complication are found as:

Shunt block at ventricular or peritoneal end due to cell debris.

Malposition of ventricular end.
Displacement of shunt tube.
Psuedocysts in the abdomen.
Shunt infection: septicaemia, wound infection, meningitis, ventriculitis, and peritonitis. If infection

occur, it is treated with massive doses of antibiotics

Seizures.
Acute subdural hygroma.

Summary
Current treatment for hydrocephalus is endoscopic third ventriculostomy or ventriculoperitoneal shunt. The
widely applicable method is the ventriculoperitoneal shunt. Despite this, the shunt infection is the most
common post-operative complication. A proper nursing assessment leads to the timely identification of
complications and their prompt treatment. Meanwhile, parents should be informed that monitoring of the
childs condition by a neurologist (for life) will assist in the full assessment of the childs neurological
status. Parents should always be vigilant about the development of the child, since there should be a change
in the catheters length, after a few years.

Bibliography
BOOKS:1. Gerard J. Tortora & Bryan Derrickson, Principles of anatomy & physiology (organization, support
2.
3.
4.
5.
6.

and movement, and control systems of the human body), Volume 1, page no: 531-533.
Susamma Varghese, Textbook of Pediatric Nursing, page no: - 449-454
Parul Datta, Pediatric Nursing, page no:- 402-405
Dorothy R. Marlow & Barbara A. Redding, Textbook of Pediatric Nursing, page no: - 522-527
OP Ghai, Essential Pediatric, page no:-548-550
Ross and Wilson, Anatomy & Physiology in Health and illness, page no: - 145-148.

WEBSITE:

http://www.ninds.nih.gov/disorders/hydrocephalus/detail_hydrocephalus.htm
http://www.medicinenet.com/hydrocephalus/page6.htm
http://www.patient.co.uk/doctor/Hydrocephalus.html
http://www.mayoclinic.org/diseases-conditions/hydrocephalus/basics/causes/con-

20030706
http://kidshealth.org/parent/medical/brain/hydrocephalus.html#
www.neurosurg.cam.ac.uk/pages/brainphys/16-Shunts. pdf

psyc.jmu.edu/school/documents/Hydrocephalus.pdf
eradiology.bidmc.harvard.edu/LearningLab/central/edwards.pdf
www.cheo.on.ca/uploads/Shunt%20Surgery/Shunt%20surgery.pdf
www.sbhi.ie/wp-content/uploads/2014/02/Types-of-Hydrocephalus.pdf
http://neurosurgery.ucla.edu/body.cfm?id=164
www1.us.elsevierhealth.com/MERLIN/Gulanick/.../gulanick02.html
www.internationaljournalofcaringsciences.org/.../vol4_issue2_02_athana