Hydrocephalus

What is hydrocephalus?
The brain and spinal cord are surrounded by cerebral spinal fluid. Small chambers in the brain are
called ventricles.  Normally, cerebral spinal fluid is produced primarily in the two lateral
ventricles. Then, the cerebral spinal fluid drains through the third and fourth ventricles and is
circulated around the brain and spinal cord. This cerebral spinal fluid acts as a cushion for the brain
and is reabsorbed by the membrane covering the brain. With hydrocephalus, the cerebral spinal fluid
backs up in the ventricles, causing them to expand and push on the surrounding brain. This increased
size in the ventricles may be referred to as ventriculomegaly. Ventriculomegaly occurs when the two
lateral ventricles are greater than 1 cm in width but less than 1.5 cm. Sometimes ventriculomegaly
will regress or return to normal size and there is no long-term problem. Hydrocephalus occurs when
the two lateral ventricles are greater that 1.5 cm in width.  The ventricles can fill to such an extent
that the head size becomes enlarged in the fetus. The major causes of hydrocephalus include:
 The flow of cerebral spinal fluid is blocked.
 Abnormal circulation of cerebral spinal fluid causes inadequate absorption by the
membranes covering the brain. 
 The brain size is small (cerebral atrophy or there is a localized injury) and the fluid volume
appears large as it fills in space. 
 Very rarely, it may be inherited.
Cerebral spinal fluid production normally increases late in gestation so the ventricles and head size
need close monitoring to determine whether there is really a concern. Ventriculomegaly may be the
first sign of another anomaly, either with the central nervous system or outside the central nervous
system. It is estimated that 50 to 80 percent of fetuses with hydrocephalus will have another anomaly
outside the central nervous system and approximately a third will have an associated anomaly within
the central nervous system. Hydrocephalus occurs more frequently in males (64 percent males versus
36 percent females). Hydrocephalus occurs in approximately 1 out of every 2,000 live births. We do
not know the cause of hydrocephalus. We know it is not related to anything the mother did or did not
do during pregnancy.
 
Prenatal diagnosis of hydrocephalus:
Hydrocephalus may be diagnosed on a routine ultrasound. The ventricles within the head will measure
larger than normal.  The head circumference may remain within the normal limits or it may become
enlarged as the pregnancy progresses. Your obstetrician will likely refer you to a specialist that
handles high-risk pregnancies. These doctors are called perinatologists.  The perinatologist will
perform a targeted ultrasound to examine the anatomy of the brain to confirm
ventriculomegaly.  They will also be looking for any associated anomalies. 
 
Hydrocephalus is occasionally associated with chromosomal abnormalities. The perinatologist typically
will offer an amniocentesis to look for chromosomal issues. Another test that may be recommended is
a fetal MRI. Ultrasound imaging can be limited by the mother's body habitus, the surrounding amniotic
fluid and the position of the fetus. Fetal MRI is a non-invasive diagnostic test that produces better
images of soft tissue, and bone or dense tissue does not interfere with the image. The biggest
limitation of fetal MRI is that the best images are obtained when the fetus stays still. 
 
How does hydrocephalus affect my baby?
The amount of pressure on the brain from the increased size of the
ventricles can impact the affect hydrocephalus has on your baby. Most
children with simple hydrocephalus (no associated anomalies) that is
diagnosed and treated early will function normally. Approximately 75
percent of children with hydrocephalus will have some form of motor
disability. Many will have some type of learning disability.   
 
How does hydrocephalus affect my pregnancy?
With the finding of ventriculomegaly on a routine ultrasound, your physician may refer you to a
specialist who manages high-risk pregnancies. These physicians are called perinatologists. The
perinatologist will perform a targeted ultrasound to examine the anatomy of the brain to confirm
ventriculomegaly.  the perinatologist will also look for any associated anomalies, which have been
reported in 54 to 84 percent of hydrocephalus cases. These associated anomalies may be disorders
within the brain (intracranial) or disorder outside of the brain (extracranial).  Between 12 and 25
percent of cases also have an associated genetic disorder.
 
It is important to rule out an intrauterine infection such as cytomegalovirus, toxoplasmosis, syphilis,
intracranial hemorrhage and tumor of the central nervous system. These causes of hydrocephalus
typically lead to increased size in the lateral ventricles but not an increase in the head size. This is
because the cerebral spinal fluid is filling in space from damage caused to the brain from the infection
or injury. Often, these babies will have microcephaly or a smaller than average size head.
   
Extracranial abnormalities have been seen in as many as two-thirds of babies with hydrocephalus. The
major extracranial defect associated with hydrocephalus is mylomeningocele. 
 
Your baby's head growth and ventricle size will be monitored closely throughout the remainder of the
pregnancy. The method of delivery is determined by the baby's head size and his or her well-being at
the time of labor and delivery, as with any pregnancy. Hydrocephalus does not, by itself, necessitate a
Caesarian section delivery.
 
How is hydrocephalus treated?
Treatment will be necessary if the ventricles continue to expand and the head circumference grows
too fast. Your baby will be evaluated after birth for the need of a shunt. If a shunt is deemed
necessary, your baby will need surgery. A shunt is a thin tube. One end of this tube is placed in the
ventricle and the other end is passed underneath the skin and drains into the abdominal cavity where
the body can reabsorb the cerebral spinal fluid. As the baby grows, the shunt will need to be replaced
approximately every two to four years.
 
If the ventricles are thought to be large because of severe brain damage, we can openly discuss other
treatment options such as palliative care.
 
What about after surgery?
If it is determined a shunt is necessary, your baby will need surgery. After surgery the baby may need
some help breathing. A special tube is placed in surgery and may stay in place until your baby is able
to breath effectively on his or her own. 
 
Some special IV lines may also by placed. The umbilical cord normally has two arteries and one
vein. A line may be placed in the vein as a means to provide nourishment until the baby is able to
eat.  Another line may be placed in one of the arteries.  This is called an arterial line. Fluids and
medication can be given through this line as well as blood pressure monitored and blood removed for
lab work.
Until the baby is able to eat it will receive total parenteral nutrition. This is an IV solution that contains
protein, fats, sugar, vitamins, and mineral. It will supply all your baby's nutritional requirements until
he or she is able to take food by mouth.
Your baby will be observed for infection, proper shunt drainage and the head circumference will be
monitored. You will be able to start learning how to care for, feed and monitor your baby in
preparation for when he or she goes home.  Sometimes the shunt can become clogged or may stop
draining effectively, which causes an increase in the pressure inside your baby's head. You will be
trained to recognize signs and symptoms of this and other potential complications.
 
Will I be able to care for my baby?
Yes. Please ask your baby's nurse about ways to interact with and care for your baby. If you had
planned to breastfeed your baby, you can begin to pump and freeze breast milk while you are still in
the hospital. A lactation consultant can assist in answering your questions. Your milk will be frozen
and stored in the Neonatal Intensive Care Unit until your baby is ready for it. The NICU has breast
pumps and private rooms available to you when you are visiting. You can bring in pictures, small toys,
booties and blankets for your baby while he or she is in the NICU.
 
When can my baby go home?
Your baby will go home when he or she is eating and tolerating enough food to maintain and allow
them to grow and gain weight.  A baby with simple hydrocephalus that requires shunt placement may
be home within two weeks. If there are any other complicating defects, the stay would be prolonged
according to the severity of those anomalies.
If you have decided to pursue palliative care for your baby, you also have the option of taking your
infant home with the support of hospice services. This option will be discussed with you.
 
What is my baby's long term prognosis?
Prognosis is dependent on any associated defects that may be related to the cause of hydrocephalus
as well as the amount of pressure exerted on the brain tissue prior to shunting. The affects of
hydrocephalus vary and is partly dependent on the cause and treatment required, as well as your
baby's response to treatment. The key to a good prognosis is early detection and treatment and the
prevention of infections. Most of the newborns born with hydrocephalus will have a normal lifespan
and approximately 40 to 50 percent will have normal intelligence. Seizure disorders have been
diagnosed in about 10 percent of these children. The mortality rate for infants with hydrocephalus is
approximately 5 percent. 
Studies have shown that the risk of shunt failure in the infant's first year is 30 percent. Shunts, on
average, are revised about two times in the first 10 years. The risk of intellectual disability is
approximately 35 percent. Your baby's pediatrician and pediatric neurologist will provide follow-up
care once your baby is home. 

HYDROCEPHALUS
Frequently Asked Questions (FAQ)  

1. What is it ?
2. How frequent is Hydrocephalus?
3. When does Hydrocephalus appear ?
4. What can cause Hydrocephalus ?
5. How is Hydrocephalus detected in a child ?
6. What does the doctor do when he suspects that a child has Hydrocephalus ?
7. How is Hydrocephalus treated ?
8. What is a shunt ?
9. How does a shunt work ?
10. How do you take care of a child with a shunt ?
11. What are the symptoms of shunt malfunction ?
12. Can shunt malfunction be prevented or detected beforehand ?
13. What causes a shunt malfunction ?
14. What do you do when you suspect that a shunt is not working properly ?
15. Can a child with Hydrocephalus lead a normal life ?
16. How often does a shunt have to be replaced ?
17. Can a child stop needing a shunt ?
18. What do parents have to know about the shunt placed on their child ?
19. Are there any options other than a shunt ?
20. What is a Third Ventricular Cysternostomy ?
1_ What is it? 
 

Normally within the brain there are some cavities

named ventricles, where a liquid known as
Cerebrospinal Fluid (CSF) is produced. The purpose of
this is to protect the brain and spinal cord, acting as a
shock absorber. It also carries away disposed
materials. The CSF circulates from the ventricles
towards a space that exists between the brain and the
membranes (meninges) that surround it, from where it
is "eliminated", into the blood stream.

When for any reason the volume of CSF

increases within the head and the brain, the
size of the ventricles increases and this is
known as Hydrocephalus. This translates into
an increase of head pressure, and therefore
there is suffering of the brain.
  
 

  
2_ How frequent is Hydrocephalus?

It is found in 1 to 3 of every 1000 born children.

3_ When does Hydrocephalus appear ?

1- When there is over production of CSF.

2- When there is a blocking in the paths of the CSF circulation.
3- When not all the CSF produced is "eliminated".
4_ What can cause Hydrocephalus ?

 Congenital defects in the developing of the CSF circulation pathways is the most frequent cause.
  It can also be caused by other diseases affecting the brain, like meningitis, hemorrhage or tumors that block
the CSF circulation.

5_ How is Hydrocephalus detected in a child ?

In children under 1 year of age the head can have an accelerated and disproportionate growth and the fontanel could
bulge. This is why in a routine medical check the doctor has to measure the head and be certain that the size and
growth rhythm are adequate. In older children the symptoms could be headache, vomit, double vision, walking
problems or tiredness.

6_ What does the doctor do when he suspects that a child has Hydrocephalus ?

First is has to be corroborated with special tests as the Ultrasound or Computerized Axial Tomography (CT scanner).
If the diagnosis is confirmed, he will try to Determine the cause.

7_ How is Hydrocephalus treated ?

The objective of the treatment is to diminish the volume of accumulated CSF and this is done by means of a shunt.

8_ What is a shunt ?

It is a drainage system which will transport the excess of CSF collected in the head to other parts of the body where it
can be reabsorbed. The most frequent places are the abdomen and the heart.

9_ How does a shunt work ? 

 

Each shunt has 3 parts:

1- Ventricular catheter: a small flexible tube which goes

in the brain, in one of the cavities where the CSF is
being retained.

2- Reservoir : a small pump which regulates the

amount of fluid that goes out. Through this the doctor
can also check the working state of the shunt, as well
as take CSF samples, when necessary, with a needle.

 
3- Distal catheter: another flexible tube that will take the fluid to the
place where it is going to be absorbed. It is usually left with
sufficient length, thinking in the child's growth..

The shunts regulates the draining pressure. There are different levels of pressure,
as high, medium and low. There are also some differences in the design but the
means is always the same. 
 

10_ How do you take care of a child with a shunt ?

- Do not manipulate the shunt unless indicated by the doctor.

- Do not leave a small child lying on the side of the shunt, as the pressure could weaken the skin. Once the child
turns on his own he will move the head freely to where he feels more comfortable.

 Frequently check for any redness or secretion on the skin over the shunt.
 Check the integrity of the skin over the shunt, wounds or scratches may conduct to infection.
 Be punctual with the normal pediatric checks.
 Check that the fontanel is leveled or depressed below the level of the bone. However, when the baby cries it
could bulge.
 Observe for symptoms of shunt malfunction.

11_ What are the symptoms of shunt malfunction ?

  One or some of these are:

 persistent headache
 vomiting without diarrhea
 double vision
 irritability
 tiredness
 fits
 bulging of the fontanel, if this is still open.

12_ Can shunt malfunction be prevented or detected beforehand ?

No.  Unfortunately, it can happen at any moment, or never.

13_ What causes a shunt malfunction ?

Remember that a shunt is a "foreign body" within the patient and the tubing is made from synthetic materials;
therefore, malfunction could be due to one of the following:

 it's blocked with byproducts of the CSF

  some of its components get disconnected
 it breaks (usually the distal end)
 any of the catheters come out of their place
 the shunt drains less fluid than it is supposed to
 it gets infected

14_ What do you do when you suspect that a shunt is not working properly ?

Take the child to the doctor, he will determine if the symptoms are due to shunt malfunction or to other disease.If
there is a malfunction probably it will need to be corrected surgically.

15_ Can a child with Hydrocephalus lead a normal life ?

Once Hydrocephalus is compensated with the use of a shunt the child could have a normal developing. However, you
have to consider that the origin of it could have left damages in the brain. The promptness of the diagnosis is also
very important, the earlier the shunt is placed, the better the prognosis.

If there is not an associated disease and depending on the child's condition, he could go to school, practice sports or
arts, the same as any child of the same age, there is no reason for segregation.

16_ How often does a shunt have to be replaced ?

In about 6 of every 10 children, at some stage in their lives a shunt revision or change will have to be made.

As long as a shunt is working properly it is not necessary to change it. Sometimes the distal catheter breaks with the
child's growth and if the shunt is being needed malfunction symptoms will appear. Other times, infection could
account for a shunt replacement.

17_ Can a child stop needing a shunt ?

Sometimes it happens, the CSF blocking disappears. However this could be overlooked and there is no problem for
the child and it is not necessary to operate on him only to remove the shunt. As far as there are no complications the
shunt can stay in its place forever and produce no harm.

18_ What do parents have to know about the shunt placed on their child ?

 The day the shunt was placed.

 The kind or type of shunt.
 The opening pressure of the shunt.

19_ Are there any options other than a shunt ?

20_ What is a Third Ventricular Cysternostomy ?

In some kinds of Hydrocephalus known as "non
communicating" (because there is no
communication between the ventricles and the
spaces around the brain, where the CSF is
absorbed), a Third Ventricular Cisternostomy
can be done. This operation is done with an
endoscope (instrument connected to a video
camera which allows to see the depths of the
brain) and instruments passed through it to
make an opening in the floor of the third
ventricle, which will allow the passage of CSF
to a place where it can be absorbed
(subarachnoid space).

If this procedure is indicated and is effective, the patient

could live without a shunt.