Hydrocephalus & Shunts
Sean’s Story
What is hydrocephalous?
Hydrocephalus is the medical term for
a condition that is commonly called
“water on the brain.”
 It is a combination of the Greek word
“hydro,” which means water and
“cephalus” which means head.
 However, the liquid involved in
hydrocephalus is not really water at
all, it is cerebrospinal fluid or CSF.

What is CSF?




CSF looks like water, but it contains
proteins, electrolytes, and nutrients that
help keep your brain healthy.
The most important purpose of CSF is to
cushion your brain and spinal cord against
injury.
Your brain produces about 1 pint of CSF
per day.
It circulates through a network of tiny
passageways in your brain, and ultimately
into your blood stream where it is absorbed
by your body.
Why does hydrocephalous
occur?
Hydrocephalus occurs when the
delicate balance of CSF production
and absorption is disrupted and CSF
builds up in the brain.
 This build-up of CSF causes the brain
to swell, and for pressure to increase
inside the skull, resulting in nerve
damage.

Congenital Hydrocephalus




People who are born with hydrocephalus have a type
of hydrocephalus called congenital hydrocephalus.
It is usually caused by a birth defect or by the brain
developing in such a way that the cerebrospinal fluid
(CSF) in the brain cannot drain properly.
Most cases of hydrocephalus (more than 70%) occur
during pregnancy, at birth, or shortly after birth.
Causes of congenital hydrocephalus include:




Toxoplasmosis (an infection from eating undercooked meat,
or by coming in contact with infected soil or an infected
animal)
Cytomegalovirus (CMV, infection by a type of herpes virus)
Rubella (German measles)
A genetic disorder usually passed only from mother to son
Acquired Hydrocephalus


Hydrocephalus can also develop later in
life. This type of hydrocephalus is called
acquired hydrocephalus, and it can occur
when something happens to prevent the
CSF in the brain from draining properly.
Causes of acquired hydrocephalus include:





Blocked CSF flow
Brain tumor or cyst
Bleeding inside the brain
Head trauma
Infection (such as meningitis)
The cause of Sean’s
hydrocephalus

Sean suffered a bilateral (both sides)
grade 4 (the most
severe) intracranial
hemorrhage (brain
bleed) at 2 days of
age due to metabolic
acidosis caused from
PDA (Patent ductus
arteriosus) coupled
with the stress from
the trauma he
experienced at birth.
Kaitlyn & Sean
The cause of Sean’s
hydrocephalus

PDA is a heart condition seen in premies (Sean and
Kaitlyn were born 10 weeks early) in which the
newborn’s DA does not close after birth and there is an
irregular transmission of blood between two of the
most important arteries in close proximity to the heart.

A PDA allows that portion of the oxygenated blood from
the left heart to flow back to the lungs.

This caused acidic levels to build up in body and, in
Sean’s case, caused a severe brain hemorrhage.

The large quantity of blood matter mixed with the CSF,
slowing its re-absorption and causing hydrocephalus.
What is a shunt?

A shunt is a piece of soft, flexible
plastic tubing that is about 1/8-inch
(3mm) in diameter.

It allows excess cerebrospinal fluid
(CSF) that has built-up inside the
skull to drain out into another part of
the body, such as the heart or
abdomen.

To drain excess CSF, shunts are
inserted into an opening or pouch
inside the brain called a ventricle,
just above where the blockage is
that is preventing the CSF from
flowing properly.
How a shunt works
All shunts perform two functions.
 They allow CSF to flow in only one
direction, to where it is meant to
drain.
 They all have valves, which
regulate the amount of pressure
inside the skull.

When the pressure inside the skull
becomes too great the valve opens,
lowering the pressure by allowing
excess CSF to drain out.
Types of shunts

Shunts are named according to where they are
inserted in the brain and where they are
inserted to let the excess CSF drain out.



A ventriculo-peritoneal (VP) shunt
drains into the abdomen or
peritoneum (belly). Most shunts,
including Sean’s, are VP shunts.
A ventriculo-pleural shunt drains
into the space surrounding the
lung.
A ventriculo-atrial (VA) shunt
drains into the atria of the heart.
The 4 parts of a shunt


Ventricular (Upper) CatheterThis is the top-most part of the
shunt. It is a small, narrow tube
that is inserted into the ventricle (a
small opening or pouch) inside the
brain that contains the
cerebrospinal fluid (CSF).
Reservoir-This is where the excess
CSF is collected until it drains into
the bottom portion of the shunt.
The reservoir also lets the doctor
remove samples of CSF for testing,
and to inject fluid into the shunt to
test for flow and to make sure the
shunt is working properly.
The 4 parts of a shunt

Valve-This controls how much
CSF is allowed to drain from the
brain.



The valve can be set to open at
a specific pressure (a fixed
pressure valve) or
It can be set by the
neurosurgeon to meet the
individual needs of the person
with hydrocephalus (a
programmable valve).
Lower Catheter-This is the
bottom-most part of the shunt.
It is a small, narrow tube that
carries the excess CSF into the
part of the body where it will be
absorbed, such as into the
abdomen or the heart.
Shunt Surgery


Small incisions are made on the head and in the
abdomen (in case of a VP shunt) to allow the
neurosurgeon to pass the shunt's tubing through
the fatty tissue just under the skin.
A small hole is made in the skull, opening the
membranes between the skull and brain to allow
the upper catheter to be passed through the brain
and into the ventricle.
Shunt Surgery


The lower catheter is passed into the belly through
a small opening in the lining of the abdomen
where the excess CSF will eventually be absorbed.
The incisions are then closed and sterile bandages
are applied.
Baby’s 1st Shunt




Sean’s first shunt was a
reservoir.
It worked as an external drain
to remove excess CSF and to
relieve pressure on the brain.
It also helped to remove the
blood matter trapped in
Sean’s head from the
hemorrhage.
If left, this blood could clog
the shunt and cause it to
malfunction, requiring more
surgery.
Reservoir

It contained only the upper
portions of the shunt:




Ventricular Catheter
Reservoir
A small needle was used to
pierce the skin and tap into
the reservoir (a plastic
bulb).
A syringe was then used to NeedleRESERVOIR:
inserted here to drain
pull excess CSF from
ANCHOR
around the brain and to suture
in place
relieve pressure.
VENTRICULAR
CATHETER
VP Shunt

2 months after the insertion of
the reservoir and going through
taps 2-3 times a week, Sean had
a second surgery to place a VP
shunt.

This internal system will allow for
constant and continual pressure
control inside his head without
the risk of infection present with
constant needle pricks.
Sean’s Shunt Line
RESERVOIR
END HERE
with excess coiling
for growth
Outcomes of shunt surgery
Shunt surgery is the most effective
treatment for hydrocephalus.
 By draining excess cerebrospinal fluid
(CSF) from the brain, shunt surgery
reduces pressure inside the
skull lowers the risk of central
nervous system damage, and relieves
the symptoms associated with
hydrocephalus.

After effects of shunt surgery
Children may need physical or
occupational therapy after shunt
surgery.
 Adults may have trouble
remembering some things that
happened recently (short-term
memory).

A Lifetime Commitment

Once you have a shunt, you always have a shunt.

On average, shunts last about 10 years, although
they can last for a much longer or much shorter
amount of time.



A shunt may need to be replaced because of an infection or
blockage, or because the shunt valve stops working
properly.
Fixed pressure shunts, which are preset to a fixed pressure
pressure, may need to be replaced if the fixed pressure
setting no longer matches the person’s needs.
In children, a shunt may need to be replaced as the child
grows to lengthen the catheter.
Signs that a shunt needs
replacing:








Loss of appetite
Nausea and vomiting
Abdominal pain or
cramps
Changes in mood,
including being
irritable
Frequent or persistent
headaches with
increased severity
Difficulty walking
Numbness on one side
of the body
Muscle tension








Sudden, constant, or
extreme tiredness
Difficulty thinking
clearly or
remembering
Difficulty seeing or
speaking
Persistent fever
Redness, swelling, or
tenderness where the
shunt is under the skin
Coma
Difficulty breathing
Abnormal heart rate
Shunt Revision and Relocation #1
February 2009
The difference
a week can make…
February 1, 2009
February 8, 2009
Signs of a problem
with Sean’s shunt








Loss of appetite
Nausea and vomiting
Abdominal pain or
cramps
Changes in mood,
including being
irritable
Frequent or persistent
headaches with
increased severity
Difficulty walking
Numbness on one side
of the body
Muscle tension








Sudden, constant, or
extreme tiredness
Difficulty thinking
clearly or
remembering
Difficulty seeing or
speaking
Persistent fever
Redness, swelling, or
tenderness where the
shunt is under the skin
Coma
Difficulty breathing
Abnormal heart rate
(rapid)
Surgery #1
Before Surgery
Notice swelling in head, including temples. Even
eye sockets swollen, forcing eyes down.
(Cranial sutures of skull opened up allowing
expansion & relieving pressure)
After Surgery
Shunt removed, EVD placed.
(Swelling already gone!)
40 rounds of IV antibiotics
20 rounds of oral antibiotics
EVD: External Ventricular
Drain

The EVD must be kept
level with the drain’s end
inside Sean’s head.

200 cc (200 mL) of CSF is
drained every day – no
wonder he had so much
swelling & a headache!
Surgery #2
24 hours post-op - New shunt in place
(Reservoir now in the back of his head)
Much happier baby!!!!
For more information…

Visit www.hydrokids.com