Hydrocephalus & ShuntsHydrocephalus & ShuntsSean’s StorySean’s Story

What is hydrocephalous?What is hydrocephalous?

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

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

However, the liquid involved in However, the liquid involved in hydrocephalus is not really water at hydrocephalus is not really water at all, it is cerebrospinal fluid or CSF. all, it is cerebrospinal fluid or 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 that proteins, electrolytes, and nutrients that help keep your brain healthy. help keep your brain healthy.

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

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

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

Why does hydrocephalous Why does hydrocephalous occur?occur?

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

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

Congenital HydrocephalusCongenital Hydrocephalus

People who are born with hydrocephalus have a type People who are born with hydrocephalus have a type of hydrocephalus called congenital hydrocephalus. of hydrocephalus called congenital 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 fluid developing in such a way that the cerebrospinal fluid (CSF) in the brain cannot drain properly. (CSF) in the brain cannot drain properly.

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

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

or by coming in contact with infected soil or an infected or by coming in contact with infected soil or an infected animal) 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 include: Causes of acquired hydrocephalus 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 metabolic age due to metabolic acidosis caused from acidosis caused from PDA 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 birth. experienced at 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 an newborn’s DA does not close after birth and there is an irregular transmission of blood between two of the irregular transmission of blood between two of the most important arteries in close proximity to the heart. most important arteries in close proximity to the heart.

A PDA allows that portion of the oxygenated blood from A PDA allows that portion of the oxygenated blood from the left heart to flow back to the lungs.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 CSF, The large quantity of blood matter mixed with the CSF, slowing its re-absorption and causing hydrocephalus.slowing its re-absorption and causing hydrocephalus.

What is a shunt?What is a shunt?

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

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

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

inserted into an opening or pouch inserted into an opening or pouch inside the brain called a ventricle, inside the brain called a ventricle, just above where the blockage is just above where the blockage is that is preventing the CSF from that is preventing the CSF from flowing properly.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 one They allow CSF to flow in only one

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

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

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

Types of shuntsTypes of shunts

A ventriculo-peritoneal (VP) A ventriculo-peritoneal (VP) shunt drains into the abdomen or shunt drains into the abdomen or peritoneum (belly). Most shunts, peritoneum (belly). Most shunts, including Sean’s, are VP shunts. including Sean’s, are VP 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 they are 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 drain out. inserted to let the excess CSF drain out.

The 4 parts of a shuntThe 4 parts of a shunt

Ventricular Ventricular (Upper)(Upper) Catheter Catheter--This is the top-most part of the This is the top-most part of the shunt. It is a small, narrow tube shunt. It is a small, narrow tube that is inserted into the ventricle (a that is inserted into the ventricle (a small opening or pouch) inside the small 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 of drains into the bottom portion of the shunt. The reservoir also lets the shunt. The reservoir also lets the doctor remove samples of CSF the doctor remove samples of CSF for testing, and to inject fluid into for testing, and to inject fluid into the shunt to test for flow and to the shunt to test for flow and to make sure the shunt is working make sure the shunt is working properly.properly.

The 4 parts of a shuntThe 4 parts of a shunt

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

a specific pressure (a fixed a specific pressure (a fixed pressure valve) or 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 person individual needs of the person with hydrocephalus (a with hydrocephalus (a programmable valve).programmable valve).

Lower CatheterLower Catheter-This is the -This is the bottom-most part of the shunt. bottom-most part of the shunt. It is a small, narrow tube that It is a small, narrow tube that carries the excess CSF into the carries the excess CSF into the part of the body where it will be part of the 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 the 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 through neurosurgeon to pass the shunt's tubing through the fatty tissue just under the skin. the fatty tissue just under the skin.

A small hole is made in the skull, opening the A small hole is made in the skull, opening the membranes between the skull and brain to allow membranes between the skull and brain to allow the upper catheter to be passed through the brain the upper catheter to be passed through the brain and into the ventricle. 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 eventually be abdomen where the excess CSF will eventually be absorbed. absorbed.

The incisions are then closed and sterile bandages The incisions are then closed and sterile bandages are applied. 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 drain It worked as an external drain to remove excess CSF and to to remove excess CSF and to relieve pressure on the brain.relieve pressure on the brain.

It also helped to remove the It also helped to remove the blood matter trapped in blood matter trapped in Sean’s head from the 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 more malfunction, requiring more surgery.surgery.

ReservoirReservoir

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

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

A syringe was then used to A syringe was then used to pull excess CSF from 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 of 2 months after the insertion of the reservoir and going through the reservoir and going through taps 2-3 times a week, Sean had taps 2-3 times a week, Sean had a second surgery to place a VP a second surgery to place a VP shunt.shunt.

This internal system will allow for This internal system will allow for constant and continual pressure constant and continual pressure control inside his head without control inside his head without the risk of infection present with the 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 surgeryOutcomes of shunt surgery

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

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

After effects of shunt surgeryAfter effects of shunt surgery

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

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

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

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

In children, a shunt may need to be replaced as the child In children, a shunt may need to be replaced as the child grows to lengthen the catheter. 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 persistent Frequent or persistent headaches with headaches with increased severity increased severity

Difficulty walking Difficulty walking Numbness on one side Numbness on one side

of the body 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 skin shunt is under the skin

Coma Coma Difficulty breathing Difficulty breathing Abnormal heart rateAbnormal heart rate

Shunt Revision and Relocation #1Shunt Revision and Relocation #1February 2009February 2009

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

February 1, 2009February 1, 2009 February 8, 2009February 8, 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 persistent Frequent or persistent headaches with headaches with increased severity increased severity

Difficulty walking Difficulty walking Numbness on one side Numbness on one side

of the body 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 skin shunt is under the skin

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

(rapid)(rapid)

Surgery #1Surgery #1

Before SurgeryBefore SurgeryNotice swelling in head, including temples. Even Notice swelling in head, including temples. Even

eye sockets swollen, forcing eyes down.eye sockets swollen, forcing eyes down.(Cranial sutures of skull opened up allowing (Cranial sutures of skull opened up allowing

expansion & relieving pressure)expansion & relieving pressure)

After SurgeryAfter SurgeryShunt removed, EVD placed. Shunt removed, EVD placed.

(Swelling already gone!)(Swelling already gone!)40 rounds of IV antibiotics40 rounds of IV antibiotics

20 rounds of oral antibiotics20 rounds of oral antibiotics

EVD: External Ventricular EVD: External Ventricular DrainDrain

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

200 cc (200 mL) of CSF is 200 cc (200 mL) of CSF is drained every day – no drained every day – no wonder he had so much wonder he had so much swelling & a headache!swelling & a headache!

Surgery #2Surgery #2

24 hours post-op - New shunt in place 24 hours post-op - New shunt in place (Reservoir now in the back of his head)(Reservoir now in the back of his head)

Much happier baby!!!! Much happier baby!!!!

For more information…For more information…

Visit Visit www.hydrokids.comwww.hydrokids.com