Hydrocephalus & ShuntsHydrocephalus & ShuntsSean’s StorySean’s Story

What is hydrocephalous?What is hydrocephalous?

Hydrocephalus is the medical term Hydrocephalus is the medical term for a condition that is commonly for a condition that is commonly called “called “water on the brainwater on the brain.” .” 

It is a combination of the Greek It is a combination of the Greek word “hydro,” which means word “hydro,” which means waterwater and “cephalus” which means and “cephalus” which means headhead. .

However, the liquid involved in However, the liquid involved in hydrocephalus is not really water hydrocephalus is not really water at all, it is cerebrospinal fluid or at all, it is cerebrospinal fluid or CSF.  CSF.  

What is CSF?What is CSF?

CSF looks like water, but it contains CSF looks like water, but it contains proteins, electrolytes, and nutrients proteins, electrolytes, and nutrients that help keep your brain healthy. that help keep your brain healthy.

The most important purpose of CSF is The most important purpose of CSF is to cushion your brain and spinal cord to cushion your brain and spinal cord against injury. against injury. 

Your brain produces about 1 pint of Your brain produces about 1 pint of CSF per day. CSF per day.

It circulates through a network of tiny It circulates through a network of tiny passageways in your brain, and passageways in your brain, and ultimately into your blood stream ultimately into your blood stream where it is absorbed by your body. where it is absorbed by your body. 

Why does hydrocephalous Why does hydrocephalous occur?occur?

Hydrocephalus occurs when the Hydrocephalus occurs when the delicate balance of CSF delicate balance of CSF production and absorption is production and absorption is disrupted and CSF builds up in disrupted and CSF builds up in the brain. the brain. 

This build-up of CSF causes the This build-up of CSF causes the brain to swell, and for pressure brain to swell, and for pressure to increase inside the skull, to increase inside the skull, resulting in nerve damage.resulting in nerve damage.

Congenital Congenital HydrocephalusHydrocephalus

People who are born with hydrocephalus have a People who are born with hydrocephalus have a type of hydrocephalus called congenital type of hydrocephalus called congenital hydrocephalus. hydrocephalus. 

It is usually caused by a birth defect or by the brain It is usually caused by a birth defect or by the brain developing in such a way that the cerebrospinal developing in such a way that the cerebrospinal fluid (CSF) in the brain cannot drain properly. fluid (CSF) in the brain cannot drain properly.

Most cases of hydrocephalus (more than 70%) Most cases of hydrocephalus (more than 70%) occur during pregnancy, at birth, or shortly after occur during pregnancy, at birth, or shortly after birth. birth.

Causes of congenital hydrocephalus include:Causes of congenital hydrocephalus include: Toxoplasmosis (an infection from eating undercooked Toxoplasmosis (an infection from eating undercooked

meat, or by coming in contact with infected soil or an meat, or by coming in contact with infected soil or an infected animal) infected animal)

Cytomegalovirus (CMV, infection by a type of herpes virus) Cytomegalovirus (CMV, infection by a type of herpes virus) Rubella (German measles) Rubella (German measles) A genetic disorder usually passed only from mother to sonA genetic disorder usually passed only from mother to son

Acquired HydrocephalusAcquired Hydrocephalus

Hydrocephalus can also develop later in Hydrocephalus can also develop later in life. This type of hydrocephalus is called life. This type of hydrocephalus is called acquired hydrocephalus, and it can occur acquired hydrocephalus, and it can occur when something happens to prevent the when something happens to prevent the CSF in the brain from draining properly. CSF in the brain from draining properly.

Causes of acquired hydrocephalus Causes of acquired hydrocephalus include: include: Blocked CSF flow Blocked CSF flow Brain tumor or cyst Brain tumor or cyst Bleeding inside the brain Bleeding inside the brain Head trauma Head trauma Infection (such as meningitis)Infection (such as meningitis)

The cause of Sean’s The cause of Sean’s hydrocephalushydrocephalus

Sean suffered a bi-Sean suffered a bi-lateral lateral (both sides)(both sides) grade 4 grade 4 (the most (the most severe)severe) intracranial intracranial hemorrhage hemorrhage (brain (brain bleed)bleed) at 2 days of at 2 days of age due to age due to metabolic acidosis metabolic acidosis caused from PDA caused from PDA (Patent ductus (Patent ductus arteriosus)arteriosus) coupled coupled with the stress from with the stress from the trauma he the trauma he experienced at experienced at birth. birth.

Kaitlyn & Sean

The cause of Sean’s The cause of Sean’s hydrocephalushydrocephalus

PDA is a heart condition seen in premies (Sean and PDA is a heart condition seen in premies (Sean and Kaitlyn were born 10 weeks early) in which the Kaitlyn were born 10 weeks early) in which the newborn’s DA does not close after birth and there is newborn’s DA does not close after birth and there is an irregular transmission of blood between two of an irregular transmission of blood between two of the most important arteries in close proximity to the most important arteries in close proximity to the heart. the heart.

A PDA allows that portion of the oxygenated blood A PDA allows that portion of the oxygenated blood from the left heart to flow back to the lungs.from the left heart to flow back to the lungs.

This caused acidic levels to build up in body and, in This caused acidic levels to build up in body and, in Sean’s case, caused a severe brain hemorrhage.Sean’s case, caused a severe brain hemorrhage.

The large quantity of blood matter mixed with the The large quantity of blood matter mixed with the CSF, slowing its re-absorption and causing CSF, slowing its re-absorption and causing hydrocephalus.hydrocephalus.

What is a shunt?What is a shunt?

A shunt is a piece of soft, A shunt is a piece of soft, flexible plastic tubing that is flexible plastic tubing that is about 1/8-inch (3mm) in about 1/8-inch (3mm) in diameter.diameter.

It allows excess cerebrospinal It allows excess cerebrospinal fluid (CSF) that has built-up fluid (CSF) that has built-up inside the skull to drain out into inside the skull to drain out into another part of the body, such another part of the body, such as the heart or abdomen.as the heart or abdomen.

   To drain excess CSF, shunts are To drain excess CSF, shunts are

inserted into an opening or inserted into an opening or pouch inside the brain called a pouch inside the brain called a ventricle, just above where the ventricle, just above where the blockage is that is preventing blockage is that is preventing the CSF from flowing properly.the CSF from flowing properly.

How a shunt worksHow a shunt works

All shunts perform two functions. All shunts perform two functions. They allow CSF to flow in only They allow CSF to flow in only

one direction, to where it is one direction, to where it is meant to drain. meant to drain.

They all have valves, which They all have valves, which regulate the amount of regulate the amount of pressure inside the skull. pressure inside the skull. When the pressure inside the When the pressure inside the

skull becomes too great the valve skull becomes too great the valve opens, lowering the pressure by opens, lowering the pressure by allowing excess CSF to drain out. allowing excess CSF to drain out.

Types of shuntsTypes of shunts

A ventriculo-peritoneal (VP) A ventriculo-peritoneal (VP) shunt drains into the abdomen shunt drains into the abdomen or peritoneum (belly). Most or peritoneum (belly). Most shunts, including Sean’s, are VP shunts, including Sean’s, are VP shunts. shunts.

A ventriculo-pleural shunt drains A ventriculo-pleural shunt drains into the space surrounding the into the space surrounding the lung. lung.

A ventriculo-atrial (VA) shunt A ventriculo-atrial (VA) shunt drains into the atria of the heart. drains into the atria of the heart.

Shunts are named according to where Shunts are named according to where they are inserted in the brain and where they are inserted in the brain and where they are inserted to let the excess CSF they are inserted to let the excess CSF drain out. drain out.

The 4 parts of a shuntThe 4 parts of a shunt

Ventricular Ventricular (Upper)(Upper) CatheterCatheter-This is the top-most -This is the top-most part of the shunt. It is a small, part of the shunt. It is a small, narrow tube that is inserted narrow tube that is inserted into the ventricle (a small into the ventricle (a small opening or pouch) inside the opening or pouch) inside the brain that contains the brain that contains the cerebrospinal fluid (CSF).cerebrospinal fluid (CSF).

ReservoirReservoir-This is where the -This is where the excess CSF is collected until it excess CSF is collected until it drains into the bottom portion drains into the bottom portion of the shunt. The reservoir also of the shunt. The reservoir also lets the doctor remove lets the doctor remove samples of CSF for testing, and samples of CSF for testing, and to inject fluid into the shunt to to inject fluid into the shunt to test for flow and to make sure test for flow and to make sure the shunt is working properly.the shunt is working properly.

The 4 parts of a shuntThe 4 parts of a shunt

ValveValve-This controls how -This controls how much CSF is allowed to much CSF is allowed to drain from the brain. drain from the brain. The valve can be set to The valve can be set to

open at a specific pressure open at a specific pressure (a fixed pressure valve) or (a fixed pressure valve) or

It can be set by the It can be set by the neurosurgeon to meet the neurosurgeon to meet the individual needs of the individual needs of the person with hydrocephalus person with hydrocephalus (a programmable valve).(a programmable valve).

Lower CatheterLower Catheter-This is the -This is the bottom-most part of the bottom-most part of the shunt. It is a small, narrow shunt. It is a small, narrow tube that carries the excess tube that carries the excess CSF into the part of the CSF into the part of the body where it will be body where it will be absorbed, such as into the absorbed, such as into the abdomen or the heart. abdomen or the heart.

Shunt SurgeryShunt Surgery

Small incisions are made on the head and in Small incisions are made on the head and in the abdomen (in case of a VP shunt) to allow the abdomen (in case of a VP shunt) to allow the neurosurgeon to pass the shunt's tubing the neurosurgeon to pass the shunt's tubing through the fatty tissue just under the skin. through the fatty tissue just under the skin.

A small hole is made in the skull, opening A small hole is made in the skull, opening the membranes between the skull and brain the membranes between the skull and brain to allow the upper catheter to be passed to allow the upper catheter to be passed through the brain and into the ventricle. through the brain and into the ventricle.

Shunt SurgeryShunt Surgery

The lower catheter is passed into the belly The lower catheter is passed into the belly through a small opening in the lining of the through a small opening in the lining of the abdomen where the excess CSF will abdomen where the excess CSF will eventually be absorbed. eventually be absorbed.

The incisions are then closed and sterile The incisions are then closed and sterile bandages are applied. bandages are applied.

Baby’s 1Baby’s 1stst Shunt Shunt

Sean’s first shunt was a Sean’s first shunt was a reservoir.reservoir.

It worked as an external It worked as an external drain to remove excess drain to remove excess CSF and to relieve CSF and to relieve pressure on the brain.pressure on the brain.

It also helped to remove It also helped to remove the blood matter trapped the blood matter trapped in Sean’s head from the in Sean’s head from the hemorrhage.hemorrhage.

If left, this blood could clog If left, this blood could clog the shunt and cause it to the shunt and cause it to malfunction, requiring malfunction, requiring more surgery.more surgery.

ReservoirReservoir

It contained only the It contained only the upper portions of the upper portions of the shunt:shunt: Ventricular Catheter Ventricular Catheter ReservoirReservoir

A small needle was used A small needle was used to pierce the skin and to pierce the skin and tap into the reservoir (a tap into the reservoir (a plastic bulb).plastic bulb).

A syringe was then used A syringe was then used to pull excess CSF from to pull excess CSF from around the brain and around the brain and relieve pressure.relieve pressure.

ANCHORto suture in place

RESERVOIR:Needle inserted here to drain

VENTRICULARCATHETER

VP ShuntVP Shunt

2 months after the insertion 2 months after the insertion of the reservoir and going of the reservoir and going through taps 2-3 times a through taps 2-3 times a week, Sean had a second week, Sean had a second surgery to place a VP shunt.surgery to place a VP shunt.

This internal system will This internal system will allow for constant and allow for constant and continual pressure control continual pressure control inside his head without the inside his head without the risk of infection present with risk of infection present with constant needle pricks.constant needle pricks.

Sean’s Shunt LineSean’s Shunt Line

END HEREwith excess coiling

for growth

RESERVOIR

Outcomes of shunt Outcomes of shunt surgerysurgery

Shunt surgery is the most effective Shunt surgery is the most effective treatment for hydrocephalus. treatment for hydrocephalus.

By draining excess cerebrospinal By draining excess cerebrospinal fluid (CSF) from the brain, shunt fluid (CSF) from the brain, shunt surgery reduces pressure inside surgery reduces pressure inside the skull  lowers the risk of central the skull  lowers the risk of central nervous system damage, and nervous system damage, and relieves the symptoms associated relieves the symptoms associated with hydrocephalus. with hydrocephalus.

After effects of shunt After effects of shunt surgerysurgery

Children may need physical or Children may need physical or occupational therapy after shunt occupational therapy after shunt surgery. surgery. 

Adults may have trouble Adults may have trouble remembering some things that remembering some things that happened recently (short-term happened recently (short-term memory). memory). 

A Lifetime CommitmentA Lifetime Commitment

Once you have a shunt, you always have a Once you have a shunt, you always have a shunt.shunt.

On average, shunts last about 10 years, On average, shunts last about 10 years, although they can last for a much longer or although they can last for a much longer or much shorter amount of time. much shorter amount of time. A shunt may need to be replaced because of an A shunt may need to be replaced because of an

infection or blockage, or because the shunt valve infection or blockage, or because the shunt valve stops working properly. stops working properly.

Fixed pressure shunts, which are preset to a fixed Fixed pressure shunts, which are preset to a fixed pressure pressure, may need to be replaced if the pressure pressure, may need to be replaced if the fixed pressure setting no longer matches the fixed pressure setting no longer matches the person’s needs. person’s needs.

In children, a shunt may need to be replaced as the In children, a shunt may need to be replaced as the child grows to lengthen the catheter. child grows to lengthen the catheter.

Signs that a shunt needs Signs that a shunt needs replacing:replacing:

Loss of appetite Loss of appetite Nausea and vomiting Nausea and vomiting Abdominal pain or Abdominal pain or

cramps cramps Changes in mood, Changes in mood,

including being including being irritable irritable

Frequent or Frequent or persistent headaches persistent headaches with increased with increased severity severity

Difficulty walking Difficulty walking Numbness on one Numbness on one

side of the body side of the body Muscle tension Muscle tension

Sudden, constant, or Sudden, constant, or extreme tiredness extreme tiredness

Difficulty thinking Difficulty thinking clearly or clearly or remembering remembering

Difficulty seeing or Difficulty seeing or speaking speaking

Persistent fever Persistent fever Redness, swelling, or Redness, swelling, or

tenderness where tenderness where the shunt is under the shunt is under the skin the skin

Coma Coma Difficulty breathing Difficulty breathing Abnormal heart rateAbnormal heart rate

Shunt Revision and Relocation Shunt Revision and Relocation #1#1

February 2009February 2009

Signs of a problemSigns of a problemwith Sean’s shuntwith Sean’s shunt

Loss of appetite Loss of appetite Nausea and vomiting Nausea and vomiting Abdominal pain or Abdominal pain or

cramps cramps Changes in mood, Changes in mood,

including being including being irritable irritable

Frequent or Frequent or persistent headaches persistent headaches with increased with increased severity severity

Difficulty walking Difficulty walking Numbness on one Numbness on one

side of the body side of the body Muscle tension Muscle tension

Sudden, constant, or Sudden, constant, or extreme tiredness extreme tiredness

Difficulty thinking Difficulty thinking clearly or clearly or remembering remembering

Difficulty seeing or Difficulty seeing or speaking speaking

Persistent fever Persistent fever Redness, swelling, or Redness, swelling, or

tenderness where the tenderness where the shunt is under the shunt is under the skin skin

Coma Coma Difficulty breathing Difficulty breathing Abnormal heart rate Abnormal heart rate

(rapid)(rapid)

The differenceThe differencea week can make…a week can make…

February 1, February 1, 20092009

February 8, February 8, 20092009

Surgery #1Surgery #1

Before SurgeryBefore Surgery After SurgeryAfter SurgeryShunt removedShunt removed

EVD placedEVD placed

EVD: External Ventricular EVD: External Ventricular DrainDrain

The EVD must The EVD must be kept level be kept level with the drain’s with the drain’s end inside end inside Sean’s head.Sean’s head.

Surgery #2Surgery #2

24 hours post-op24 hours post-op

New shunt in place New shunt in place (now in the back of his head)(now in the back of his head)

For more information…For more information…

Visit Visit www.hydrokids.com