Understanding Hydrocephalus in Youth

Your brain contains billions of cells and is the control centre of your mind and body. It is protected by your skull, cushiony membranes, and an essential liquid called cerebrospinal fluid, or CSF.

We all need CSF. It delivers important nutrients and chemicals from the blood to the brain, removes waste products from the brain, and protects this complex, essential organ.

CSF is continuously produced inside four ventricles, or chambers, in the brain. Normally, CSF flows freely from one ventricle to the next before it exits the brain. However, when the flow of CSF is interrupted or blocked, or too much CSF accumulates, this causes the ventricles to swell. That puts pressure on the brain and can cause serious damage. This excess accumulation of CSF is called hydrocephalus.

Because it affects the brain, hydrocephalus can cause a wide range of symptoms ranging from difficulty breathing, poor muscle coordination and mobility challenges to problems with vision, fatigue, headaches, seizures, incontinence and hormonal imbalances. Challenges with learning, social skills, memory and problem solving are among the most common complications of hydrocephalus. Individuals with the condition may require modifications to the way they are taught, especially when it comes to learning new things at school or work.

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Hydrocephalus is a very serious condition that, in most cases, requires medical attention. Delaying treatment may cause lasting injury to the brain, so getting an accurate diagnosis and the appropriate treatment as soon as possible is critical.

Who is at risk of developing hydrocephalus?

Hydrocephalus can affect anyone at any time. It is a chronic condition which means there is no cure and, in most cases, the person affected will have to live with the condition for life.

How hydrocephalus affects people differs from person to person. The resulting challenges depend on:

  • what caused the hydrocephalus
  • how long the condition has been present
  • if there is any brain damage, and how extensive the damage is
  • if there are any other associated complications, and
  • what treatment was provided

When the condition is present in a newborn baby, it is called congenital hydrocephalus. It may be caused by genetic conditions or influences that affect development in the womb or by blockages, cysts or spina bifida.

Hydrocephalus that develops after birth, is called acquired hydrocephalus. Babies born prematurely, those who suffer a head injury, stroke, brain tumor, or meningitis may be at a higher risk of developing the condition.

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Recognizing the signs of hydrocephalus in children

It is important that we all learn to watch for symptoms of hydrocephalus in people of any age and seek medical attention when we think someone may have the condition. A child with hydrocephalus may develop some or all of the symptoms listed below.

INFANTS with hydrocephalus may show the following symptoms:

  • head enlargement
  • fontanel (soft spot) bulging when baby is upright and quiet
  • prominent scalp veins appearing unnaturally full
  • fever
  • vomiting (especially projectile)
  • irritability and sleepiness
  • seizures
  • downward deviation of the eyes (sunset eyes)

TODDLERS with hydrocephalus may show the following symptoms:

  • head enlargement
  • headaches and seizures
  • fever
  • vomiting (especially projectile)
  • irritability, sleepiness
  • lethargy/listlessness
  • blurred or double vision
  • unable to concentrate
  • loss of previous cognitive or motor abilities, delayed development in walking and talking
  • bladder and bowel incontinence
  • change in personality
  • poor coordination or balance

 CHILDREN with hydrocephalus may show the following symptoms:

  • headaches and seizures
  • vomiting (especially projectile)
  • irritability, tiredness or difficulty staying awake
  • difficulty waking up from sleep
  • blurred or double vision
  • loss of coordination or balance
  • bladder and bowel incontinence
  • change in personality
  • impairment of cognitive or motor performance
  • decline in academic or work performance

   PLEASE TAKE NOTE: people with hydrocephalus who experience headaches often feel pain at the front and on both sides of the head. The headaches are generally severe upon waking in the morning, or following a nap, and may be relieved by sitting up. 

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Diagnosis and Treatment

Hydrocephalus is diagnosed using imaging techniques such as ultrasonography, CT, MRI, or pressure-monitoring techniques to understand what is happening with your brain. The tests that will be done may depend on the person’s age, the symptoms they have been experiencing, and known or suspected abnormalities of the brain or spinal cord.

Although there is currently no cure for hydrocephalus, there are treatments options. There are important factors to consider before choosing the treatment that is right for you. You and your physician should speak openly about your health history and personal factors that may affect your care plan. It is important that you understand the benefits and risks associated with each treatment option.

Wait and Watch

In some cases, treatment may not be necessary or can be delayed. These individuals are carefully monitored throughout their lives to detect any changes that may indicate damaging effects of hydrocephalus that require medical attention. It is possible that treatment may become necessary as time goes on.

Shunt Implant

For most people, hydrocephalus is managed by surgically implanting a shunt system in the brain to manage the flow of CSF. These systems include:

  • a catheter (a flexible tube made of sturdy plastic) which diverts excess CSF away from the brain. One end is placed inside a ventricle and the other ends in another area of the body where the CSF can drain and be absorbed such as the belly, heart or lungs, and
  • a one-way valve that controls the rate at which the CSF drains.

While shunts can be life-saving, they can also be problematic. They tend to be subject to mechanical failure, infection and blockages and catheters sometimes need to be adjusted or replaced. Some people will many brain surgeries to manage shunt challenges.

Shunt surgery is the most prevalent surgery in children. Today, as many as 40% of shunts placed in children fail. When this happens, symptoms of hydrocephalus may return along with the risk of brain damage. It is important to watch for symptoms of hydrocephalus and seek medical attention when we think a child may have a shunt that is malfunctioning.

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ETV (Endoscopic Third Ventriculostomy)

For some children, an alternative to inserting a shunt is another surgical procedure called endoscopic third ventriculostomy (ETV). During this procedure, an endoscope (a device surgeons use to see inside the body) creates a small hole in the floor of the third ventricle in the brain. This creates an alternate path for CSF to flow out of the blocked ventricle and into the other cavities of the brain and then elsewhere in the body so there is no accumulation in the brain.

Children who have ETV are carefully monitored throughout their lives and must be watched diligently. If symptoms of hydrocephalus return, medical attention must be sought.

ETV/CPC (Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization)

There is a third option for treating hydrocephalus in infants that involves the addition of choroid plexus cauterization to the ETV. During this procedure, the neurosurgeon cauterizes, or burns, tissue in the choroid plexus in the ventricles of the brain where CSF is produced.

It is very important that parents and patients understand that ETV may not be a permanent cure for hydrocephalus and that ETV/CPC may not be recommended because the chances for success could be low.

Is ETV the Prefered Treatment for Hydrocephalus: Dr. Abhaya Kulkarni provides an overview of the procedure, who is most likely to benefit from the procedure, and the risks and benefits associated with the procedure.

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