Hydrocephalus is a buildup of fluid in the brain, or more precisely, cerebrospinal fluid in the ventricular system, which causes potentially damaging increased pressure in the head. Most children who have it are born with it. It occurs in one or two per 1,000 live births.

The ventricular system in the brain is made up of four chambers that hold most of the cerebrospinal fluid that is in the head. The two upper chambers are called the left and right lateral ventricles. They connect to the third chamber or ventricle, which drains into the fourth.

Cerebrospinal fluid is a water-like substance that acts to cushion the brain within the skull. It also filters waste from tissue in and around the brain.

The cerebrospinal fluid is made in the walls of the ventricles. The fluid flows from the upper ventricles down to the lower ventricles and then over the surface of the brain and around the spinal cord. The cerebrospinal fluid is absorbed over the surface of the brain into the bloodstream.

What are the Causes?

Hydrocephalus occurs when the body makes more cerebrospinal fluid than the brain absorbs. A child may be born with this, but it can happen to a person at any age. Older children or adults with an abnormal growth in the head (tumor) or a head injury can develop hydrocephalus.

Hydrocephalus has two base causes: the bloodstream is not fully absorbing the fluid due to a damaged filter in the brain and / or the flow of fluid within the ventricles is blocked.

Any significant block in the ventricular system will cause hydrocephalus since cerebrospinal fluid is made there. This blockage can occur due to a buildup of debris that blocks the flow of fluid or can be from a smaller than normal opening in the ventricular system.

This narrow opening in the ventricular system may happen during the baby’s growth in the womb. The debris which collects and creates a blockage can be from an infection or from bleeding (hemorrhage) after birth. A tumor in the head may also block the flow of cerebrospinal fluid.

This buildup of fluid causes increased pressure in the head. If untreated, over time it can cause damage to the brain.

Signs and Symptoms of Hydrocephalus

• Your infant’s doctor may order a screening for hydrocephalus if your infant’s head is growing more quickly than others his age.
• Headaches and vomiting are signs of increased intracranial pressure, which can result from hydrocephalus.

Treatment for Hydrocephalus

The most common treatment for hydro is inserting a ventricular shunt. A shunt is a tube that drains the CSF from the ventricles to another space in the body, which then absorbs the fluid.

A second type of treatment for hydrocephalus is endoscopic third ventriculostomy (ETV). This procedure has proven more effective at managing hydrocephalus caused by the ventricular system. It is not used for children who have a damaged filter in the brain that does not allow cerebrospinal fluid absorption into the bloodstream.

Third Type of treatment option is Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization.

What is a Shunt?

A shunt is a tube that drains the cerebrospinal fluid (CSF) from the ventricles in the brain to another space in the body, which reabsorbs the fluid. Most often the abdominal cavity (the space around the stomach and intestines) is used. The shunt’s small, soft tubing is placed, or tunneled, under the skin, and the end of the tubing is placed in the abdominal space. This is done in the operating room.

A valve is attached to the shunt tubing. It controls the direction the cerebrospinal fluid flows and keeps a normal amount of cerebrospinal fluid in the ventricles. The valve adjusts the amount of cerebrospinal fluid that flows from the ventricles and prevents the ventricles from over-draining, which is called over-shunting.

Shunt Malfunctions

A shunt malfunctions when something blocks the flow of cerebrospinal fluid within the shunt. This may be caused by the buildup of the tissue, proteins and chemicals in the cerebrospinal fluid that clog the tubing. It may also be caused by tissue within the abdominal space that blocks the flow of the tubing. Over time, the shunt tubing may also wear out or pull apart and need to be replaced.

The signs of a shunt malfunction are similar to the signs of hydrocephalus. The symptoms are a result of pressure again building up within the head. Call your child’s doctor immediately if and when these symptoms develop.

Preparing for Surgery

Whether your child is getting a shunt or is having endoscopic third ventriculostomy, to help us prepare for a successful surgery, please share with us your child’s full medical history.

During the Surgery

Prior to surgery, your child’s surgeon will choose the most appropriate surgery, either the shunt or endoscopic third ventriculostomy. The neurosurgeon will discuss the procedure in detail with you before your child’s scheduled surgery date, but here is a brief description.

Shunt − The first step in the shunting procedure is placing a ventricular catheter through the skull into the ventricular system. A ventricular catheter is a tube with tiny holes. This catheter is connected to a pressure valve that serves to regulate the amount of spinal fluid drainage.

Another long, thin tube called a distal catheter is attached to the valve. This is the tube that drains the cerebral spinal fluid. The distal catheter and valve are located underneath your child’s skin.

Endoscopic Third Ventriculostomy (ETV) − For this procedure, the surgeon makes a small hole in the bottom of the third ventricle so that cerebral spinal fluid bypasses the obstruction and flows into the natural pathways.

Both of these surgeries are performed under general anesthesia and will be scheduled for at least one hour and a half.

After Surgery

Your child will go to the Post Anesthesia Recovery Unit after surgery, and the surgeon will update you on how well the surgery went. When you get a call from the Recovery Unit, you may join your child.

Your child will spend one to three nights with us, and we will take care of special dietary needs (general anesthesia may cause an upset stomach). Your child may go home when he or she:

• Is eating and drinking well
• Is walking around without problems
• Is keeping pain under control
• Has had a bowel movement

Caring for Your Child at Home

Here is some basic information to help you care for your child at home after surgery.

Pain − If your child is under 4 years of age, give regular children’s Paracetamol as directed for his or her pain. Children older than 4 years may be sent home with a prescription for Paracetamol.

Diet − Once your child is home, he or she will be on a regular diet. There are some restrictions right after surgery, but since your child will be spending several nights with us, we will restrict the diet as needed during that time.

Wound care − Wash your child’s incision each day with a mild shampoo.

Activity − Your child may slowly resume regular activities. Your child may return to school or daycare three to five days after surgery, depending on how he or she feels.

Medication − No medicines are given routinely after hydrocephalus surgery. If your child complains of pain at the site of the incision, give him or her Tylenol.

Bathing – Wash the wound daily with a mild soap or shampoo and rinse with clear water. A shower is fine if your child typically takes showers. Do not let the wound soak in water such as a bathtub or swimming pool until the skin is completely healed.

Follow-up care − About 14 days after surgery, your child will have a follow-up appointment with the Neurosurgery Division for a wound check. During that visit, we may also take more images of your child’s brain. If your child becomes ill in the future, this will help us figure out if hydrocephalus is causing the problem.

Watch your child for any signs of hydrocephalus. These would typically be the same symptoms that prompted the surgery.

What Are Signs of Shunt Malfunction?

• Headaches
• Vomiting
• Lethargy (sleepiness)
• Irritability
• Swelling or redness along the shunt tract
• Decreased school performance
• Periods of confusion
• Seizures

Shunt Malfunctions

• If your child becomes very ill (persistent vomiting, extreme sleepiness, severe headache), seek medical assistance immediately.
• Call your child’s doctor if you think that your child is having a shunt malfunction based on the symptoms described above.
• Remember that you may see only one or two warning signs at a time. You will not see all of the signs at one time. Call your child’s doctor if you notice one or more of these signs in your child.
• Call your doctor if vomiting and lethargy occur without fever or diarrhea. You may be instructed to first be seen by your primary medical doctor to assess for another illness.
• DO NOT PUSH ON OR PUT PRESSURE ON THE SHUNT.
• You may be sent for a head CT before seeing a member of the neurosurgery team.
• When a shunt is not working well, all or part of it must be replaced. This is done in the operating room. Your child’s neurosurgeon will decide where the problem is within the shunt system. The doctor will replace the tubing in the head (the proximal tubing), the tubing to the abdominal space (the distal tubing), and / or the valve.
• The location of the blockage will determine where the surgeon needs to operate. If it is a blockage in the proximal system (valve or ventricular catheter), a small patch of hair will be shaved and a “C” shaped incision will be made.
• If the blockage is a distal malfunction, there will be an abdominal incision. Sometimes it is unclear where the malfunction is occurring, so both the head and abdomen need to be explored.

What is Shunt Revision?

When a shunt is not working well, all or part of it must be replaced. This is done in the operating room. Your child’s neurosurgeon will decide where the problem is within the shunt system. The doctor will replace the tubing in the head (the proximal tubing), the tubing to the abdominal space (the distal tubing), and / or the valve.

The location of the blockage will determine where the surgeon needs to operate. If it is a blockage in the proximal system (valve or ventricular catheter), a small patch of hair will be shaved and a “C” shaped incision will be made.

If the blockage is a distal malfunction, there will be an abdominal incision. Sometimes it is unclear where the malfunction is occurring, so both the head and abdomen need to be explored.