The term hydrocephalus is derived from the Greek words "hydro" meaning water and

"cephalus" meaning head. As the name implies, it is a condition in which the primary
characteristic is excessive accumulation of fluid in the brain. 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. The excessive
accumulation of CSF results in an abnormal widening of spaces in the brain called
ventricles. This widening creates potentially harmful pressure on the tissues of the
brain.
The ventricular system is made up of four ventricles connected by narrow passages.
Normally, CSF flows through the ventricles, exits into cisterns (closed spaces that serve
as reservoirs) at the base of the brain, bathes the surfaces of the brain and spinal cord,
and then reabsorbs into the bloodstream.
CSF has three important life-sustaining functions: 1) to keep the brain tissue buoyant,
acting as a cushion or "shock absorber"; 2) to act as the vehicle for delivering nutrients
to the brain and removing waste; and 3) to flow between the cranium and spine and
compensate for changes in intracranial blood volume (the amount of blood within the
brain).
The balance between production and absorption of CSF is critically important. Because
CSF is made continuously, medical conditions that block its normal flow or absorption
will result in an over-accumulation of CSF. The resulting pressure of the fluid against
brain tissue is what causes hydrocephalus.
hat are the different types of hydrocephalus?
Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is present at
birth and may be caused by either events or influences that occur during fetal
development, or genetic abnormalities. Acquired hydrocephalus develops at the time of
birth or at some point afterward. This type of hydrocephalus can affect individuals of all
ages and may be caused by injury or disease.
Hydrocephalus may also be 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. One of the most common causes of
hydrocephalus is "aqueductal stenosis." In this case, hydrocephalus results from a
narrowing of the aqueduct of Sylvius, a small passage between the third and fourth
ventricles in the middle of the brain.
There are two other forms of hydrocephalus which do not fit exactly into the categories
mentioned above and primarily affect adults: hydrocephalus ex-vacuo and normal
pressure hydrocephalus.
Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the
brain. In these cases, brain tissue may actually shrink. Normal pressure hydrocephalus
can happen to people at any age, but it is most common among the elderly. It may
result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications
of surgery. However, many people develop normal pressure hydrocephalus even when
none of these factors are present for reasons that are unknown.
Who gets this disorder?
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.
What causes hydrocephalus?
The causes of hydrocephalus are still not well understood. 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). Other possible causes include
complications of premature birth such as intraventricular hemorrhage, diseases such as
meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage, which block the
exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF into
the cisterns.
What are the symptoms of hydrocephalus?
Symptoms of hydrocephalus vary with age, disease progression, and individual
differences in tolerance to the condition. For example, an infant's ability to compensate
for increased CSF pressure and enlargement of the ventricles differs from an adult's.
The infant skull can expand to accommodate the buildup of CSF because the sutures
(the fibrous joints that connect the bones of the skull) have not yet closed. In infancy,
the most obvious indication of hydrocephalus is often a rapid increase in head
circumference or an unusually large head size. Other symptoms may include vomiting,
sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and
seizures. Older children and adults may experience different symptoms because their
skulls cannot expand to accommodate the buildup of CSF. Symptoms may include
headache followed by vomiting, nausea, papilledema (swelling of the optic disk which is
part of the optic nerve), blurred or double vision, sunsetting of the eyes, problems with
balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of
developmental progress, lethargy, drowsiness, irritability, or other changes in
personality or cognition including memory loss. Symptoms of normal pressure
hydrocephalus include, problems with walking, impaired bladder control leading to
urinary frequency and/or incontinence, and progressive mental impairment and
dementia. An individual with this type of hydrocephalus may have a general slowing of
movements or may complain that his or her feet feel "stuck." Because some of these
symptoms may also be experienced in other disorders such as Alzheimer's disease,
Parkinson's disease, and Creutzfeldt-Jakob disease, normal pressure hydrocephalus is
often incorrectly diagnosed and never properly treated. Doctors may use a variety of
tests, including brain scans (CT and/or MRI), a spinal tap or lumbar catheter,
intracranial pressure monitoring, and neuropsychological tests, to help them accurately
diagnose normal pressure hydrocephalus and rule out any other conditions. The
symptoms described in this section account for the most typical ways in which
progressive hydrocephalus manifests itself, but it is important to remember that
symptoms vary significantly from one person to the next.
How is hydrocephalus diagnosed?
Hydrocephalus is diagnosed through clinical neurological evaluation and by using
cranial imaging techniques such as ultrasonography, computed tomography (CT),
magnetic resonance imaging (MRI), or pressure-monitoring techniques. A physician
selects the appropriate diagnostic tool based on an individual's age, clinical
presentation, and the presence of known or suspected abnormalities of the brain or
spinal cord.

Risk factors
Premature infants have an increased risk of severe bleeding within the ventricles of the
brain (intraventricular hemorrhage), which can lead to hydrocephalus.
Certain problems during pregnancy may increase an infant's risk of developing
hydrocephalus, including:
• An infection within the uterus
• Problems in fetal development, such as incomplete closure of the spinal column
Congenital or developmental defects not apparent at birth also can increase older
children's risk of hydrocephalus.
Other factors that increase your risk of hydrocephalus include:
• Lesions or tumors of the brain or spinal cord
• Central nervous system infections
• Bleeding in the brain
• Severe head injury

What is the current treatment for hydrocephalus?

Hydrocephalus is most often treated by surgically inserting a shunt system. This system
diverts the flow of CSF from the CNS to another area of the body where it can be
absorbed as part of the normal circulatory process. A shunt is a flexible but sturdy
plastic tube. A shunt system consists of the shunt, a catheter, and a valve. One end of
the catheter is placed within a ventricle inside the brain or in the CSF outside the spinal
cord. The other end of the catheter is commonly placed within the abdominal cavity, but
may also be placed at other sites in the body such as a chamber of the heart or areas
around the lung where the CSF can drain and be absorbed. A valve located along the
catheter maintains one-way flow and regulates the rate of CSF flow. A limited number of
individuals can be treated with an alternative procedure called third ventriculostomy. In
this procedure, a neuroendoscope - a small camera that uses fiber optic technology to
visualize small and difficult to reach surgical areas - allows a doctor to view the
ventricular surface. Once the scope is guided into position, a small tool makes a tiny
hole in the floor of the third ventricle, which allows the CSF to bypass the obstruction
and flow toward the site of resorption around the surface of the brain.
What are the possible complications of a shunt system?
Shunt systems are not perfect devices. Complications may include mechanical failure,
infections, obstructions, and the need to lengthen or replace the catheter. Generally,
shunt systems require monitoring and regular medical followup. When complications
occur, the shunt system usually requires some type of revision.
Some complications can lead to other problems such as overdraining or underdraining.
Overdraining occurs when the shunt allows CSF to drain from the ventricles more
quickly than it is produced. Overdraining can cause the ventricles to collapse, tearing
blood vessels and causing headache, hemorrhage (subdural hematoma), or slit-like
ventricles (slit ventricle syndrome). Underdraining occurs when CSF is not removed
quickly enough and the symptoms of hydrocephalus recur. In addition to the common
symptoms of hydrocephalus, infections from a shunt may also produce symptoms such
as a low-grade fever, soreness of the neck or shoulder muscles, and redness or
tenderness along the shunt tract. When there is reason to suspect that a shunt system
is not functioning properly (for example, if the symptoms of hydrocephalus return),
medical attention should be sought immediately.
What is the prognosis for hydrocephalus?
The prognosis for individuals diagnosed with hydrocephalus is difficult to predict,
although there is some correlation between the specific cause of the hydrocephalus and
the outcome. Prognosis is further complicated by the presence of associated disorders,
the timeliness of diagnosis, and the success of treatment. The degree to which relief of
CSF pressure following shunt surgery can minimize or reverse damage to the brain is
not well understood. Affected individuals and their families should be aware that
hydrocephalus poses risks to both cognitive and physical development. However, many
children diagnosed with the disorder benefit from rehabilitation therapies and
educational interventions and go on to lead normal lives with few limitations. Treatment
by an interdisciplinary team of medical professionals, rehabilitation specialists, and
educational experts is critical to a positive outcome. Left untreated, progressive
hydrocephalus may be fatal. The symptoms of normal pressure hydrocephalus usually
get worse over time if the condition is not treated, although some people may
experience temporary improvements. While the success of treatment with shunts varies
from person to person, some people recover almost completely after treatment and
have a good quality of life. Early diagnosis and treatment improves the chance of a
good recovery.
MANAGEMENT:

Medical treatment has been largely unsuccessful, at least in chronic and progressive
cases. Diuretics may help in acute transient cases. Carbonic anhydrase inhibitor has
been claimed to reduce CSF production. Adrenocorticosteroids may diminish the CSF
flow.

Ventricular shunting:

In transient hydrocephalus, and in patients with high CSF protein or when CSF infection
is suspected external ventricular drainage is usually the first line of treatment. External
drainage introperatively helps prior to certain tumor removal. Normally, the drainage is
not maintained for longer than two weeks to avoid infection. Studies suggest that, if the
drainage tube is tunneled well away from the insertion site, the infection rate is low.

Established and progressive hydrocephalus is treated with shunt insertion. Newborns

with hydrocephalus with cerebral mantle less than<2cm, should be treated within 5
months to maximize the mantle thickness, associated with normal IQ.
The shunts drain CSF from the ventricles to a site of of superior absorptive capacity.
Many sites, including vessels, peritoneum, gall bladder, urinary bladder have been
used. Peritoneum has been proven to have the best absorptive capacity. Ventriculo
peritoneal, and ventriculo atrial shunts are widely employed.

Implantable shunts are composed of a silicone elastomer and are often impregnated
with barium. The shunts consists of a proximal catheter, a valve, and distal tubing.
Evolution of shunts has been guided by the need to reduce the incidence of
complications, some of which are trivial and self-limiting whereas others are
occasionally fatal. Financial aspects are also important, as an episode of shunt
infection can be extremely expensive. Lately, antibiotic impreganated shunts are being
marketed.
There is no perfect shunt. In general, there are three types of valves:
1) A pressure regulating valve opens at a preset pressure and maintains its pressure
across the valve, regardless of flow rate.
Slit valves, e.g.. Hotler,Upadyaya, Chabra: flow decreases gradually as differential
pressure decreases until closing pressure of valve (low pressure - 2-5cm water, medium
pressure - 5-10cm water, high pressure - 10-15cm water) is reached.
Diaphragm valves, e.g.: Pudenz, Ceredrain: flow remains roughly constant until closing
pressure is reached.
Ball valves, e.g.: Hakim: similar pattern to diaphragm valves.
Programmable valves allow the closing pressure to be adjusted externally using
magnet.
2) Flow regulating valve maintains a constant flow at different pressures to overcome
the complications of over drainage. The flow is regulated by increasing valve resistance
as pressure increases.

3) Siphon resistant valves act by increasing the opening pressure of the system in direct
proportion to the vertical distance between the proximal and distal ends of the distal
tubing. This allows correction of hydrostatic pressure, which changes when the patient
changes his position. Positive ICP is maintained, despite the position of the patient.

Insertion of a shunt must be regarded as a lifetime commitment from the surgeon to the
patient. meticulous measures should be taken owing to the high frequency of potential
complications. Small skin incisions to avoid contact with skin, small bony opening to
prevent egress of CSF are recommended. The ideal position for the ventricular catheter
is in the frontal horn or in the occipital horn to catheter blockage due to choroid plexus.
The peritoneal catheter should be positioned over the liver in the retrohepatic space to
avoid distal occlusion by omental fat.

Complications of shunts:
A baby with a new shunt would be expected on average to undergo 2 shunt revisions
for blockage during first 10 years. Approximately 30% of infants with newly inserted
shunts will have a shunt complication within the first year.
The common shunt complications are, obstruction, over drainage, and infection, and
less commonly, seizures.

Obstruction:
A shunt can occlude at any site, but the most common site is the ventricular end,
usually by the choroid plexus.
The distal end can become occluded with fat and with an abdominal pseudocyst.
Precise endoscopic placement of the ventricular end in the frontal horn to avoid the
choroid plexus and abdominal end at the retrohepatic space may minimize the chance
of obstruction.
RBCs, tumor cells, high protein level in CSF have also been the cause of proximal tube
obstruction. Body growth, adhesions (associated with low-grade infection), and
pregnancy may the cause in distal end.
A shunt can occlude at any site, but the most common site is the ventricular end,
usually by the choroid plexus. The distal end can become occluded with fat and with an
abdominal pseudocyst. Precise endoscopic placement of the ventricular end in the
frontal horn to avoid the choroid plexus and abdominal end at the retrohepatic space
may minimize the chance of obstruction. Body growth, adhesions (associated with low-
grade infection), and pregnancy may the cause in distal end obstruction.

Patient presents with features of raised ICT, and CT reveals ventriculomegaly.

If there is no spontaneous CSF flow through a needle inserted into the shunt reservoir,
the proximal is the one that is blocked.
A shunt-o-gram, by injecting a radioisotope into the reservoir and imaging both ends,
may help to detect the blocked end. ICP monitoring and CSF infusion studies will
document more precisely the shunt’s hydrodynamic properties.
The blocked end may be revised. Alternatively, a new shunt system is established on
the opposite side.

Overdrainage:
When there is excessive drainage, the ventricles may collapse around the shunt, as in
the 'slit ventricle' syndrome. Siphoning effect of the shunt (hydrostatic pressure-25-75
cm CSF) caused by column of CSF within peritoneal or atrial catheter sucks fluid out of
ventricles in upright position. Differential valve, even high pressure 15 cm CSF, may not
stop siphoning.

'Slit ventricle' syndrome: Some patients will develop decreased transependymal flow
and decreased intracranial compliance. When a shunt malfunction occurs in these
patients, the ventricles fail to expand. Chronic low pressure within the ventricle and
intermittent shunt malfunction due to ventricular catheter abutting against ventricular
wall have been blamed
Clinical features include attacks of headaches, vomiting, drowsiness and pallor
associated with slit-like ventricles on CT scan.
It is a difficult problem to treat. Revision of shunt with a Siphon resistant valve, if
available or a high pressure valve may help. Occasionally subtemporal decompression
or other cranial expansion technique is needed.

Subdural Hematoma: Many post-operative sub dural collection are asymptomatic and
do not require treatment. Others may cause reduced conscious level and focal deficit,
and require evacuation, and at times shunt removal and reinsertion of shunt with a
Siphon resistant valve, if available or a high pressure valve as a second stage
procedure.
Recurrent symptomatic sub dural collections may need sub duro-peritoneal shunt.
Infections:
3-20% of the patients develop shunt infection, and have increased mortality and
increased seizures and may affect long-term outcome in children. Staphylococcus
epidermidis is the causative bacteria in two thirds of shunt infections. Staph. aureus and
gram negative bacilli are also common. In neonates, Escherichia coli and
Streptococcus hemolyticus predominate.
Presenting symptoms include nausea, vomiting, fever, lethargy, anorexia, irritability, and
abdominal pain. In addition, shunt may get blocked with features of raised ICT. CSF
cultures may be negative in 40% and one must have a high level of suspicion. On rare
occasions, 'shunt nephritis' may develop, secondary to chronic low level infection of a
shunt with subsequent immune complex deposition into renal glomeruli.

Strict aseptic precautions, and avoidance of shunt surgery in the presence of CSF leak
or intercurrent infection prevention.
In most cases of shunt infection, the shunt may need to be removed. External
ventricular drainage may be required. Appropriate antibiotic is mandatory. Intravenous
Vancomycin is used widely while awaiting bacteriological studies. Intrathecal/reservoir
antibiotics do not provide additional benefit. When the CSF culture is sterile for 3
consecutive days, it is recommended that the antibiotics may be continued for about 10
days and a fresh shunt is inserted
Some centers are successful in treating shunt infections in situ without removing the
shunt.

Other rare complications:

Secondary sagittal synostosis may occur as a result of shunting in infants, particularly

premature babies with severe post-hemorrhagic hydrocephalus.
Miscellaneous complications of V-P shunts (Operative misplacement of the shunt
tubing, erosion of shunt tubing through wall of abdominal organs, disconnections) occur
sporadically.
Miscellaneous complications of V-A shunts (cardiac arrhythmias, cardiac tamponade,
mural thrombus and pulmonary emboli, detachment of distal catheter during shunt
revision) are uncommon, but more life-threatening than those in V-P shunts.

Endoscopic third ventriculostomy:

Internal decompression, by 'by passing' the obstruction, restores normal CSF flow. If the
obstruction is in the ventricles or at the outlets of the third or fourth ventricles, internal
decompression may be possible; third ventricle should not be small. The decrease in
ventriculomegaly, is almost always less than that achieved after a V-P shunt.
Lately, internal decompression by rerouting CSF flow through the floor of the third
ventricle using neuroendoscopic techniques has
become dramatically refined.Third ventriculostomy is claimed to be an effective
alternative to shunt in experienced hands.

OUTCOME:

20% of untreated children survive to adulthood. Severely damaged babies with

hydrocephalus are best treated by shunting to prevent excessive head growth and its
associated nursing problems. 70% of babies with treated non tumoral hydrocephalus
would be expected to attend a normal school, with a normal IQ. Most become shunt
dependent and remain so for the rest of their lives.

About 50% of children with communicating hydrocephalus may retain the potential to be
independent of shunt.