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 "Hydrocephalus Known as "water on the brain"rain."

 
 

About Hydrocephalus

The Hydrocephalus Awareness Ribbon is recognized by a light blue, dark blue ribbon with a water drop reprsenting CSF 

Hydro Angels launched the "This Ribbon Matters" campaign in 2012-17.

 

  

 

There is hope

The diagnosis can be hard and prognosis uncertain. But, hydrocephalus is not a one size fits all condition. Some people live very productive lives, others struggle a little more, and some sadly do not survive long after birth. But, we all fight on, together. It is important to understand that many doctors give grave diagnoses at the 1st known ultrasound sighting of fluid on the brain. However, many women have gone on to deliver babies that beat the odds and unfarvorable prognosis. We encourage you to seek a second opinion and expert advice of trained and qualified doctors who have advnaced experience of the medical condition known as hydrocephalus.

What is hydrocephalus?

Hydrocephalus, also called Water on the Brain is a condition in which there is an abnormal build up of CSF (cerebrospinal fluid) in the cavities (ventricles) of the brain. The buildup is often caused by an obstruction which prevents proper fluid drainage. The fluid buildup can raise intracranial pressure inside the skull which compresses surrounding brain tissue, possibly causing progressive enlargement of the head, convulsions, and brain damage. Hydrocephalus can be fatal if left untreated.

Who can have hydrocephalus?

Babies: In fact it is estimated that 1 in every 500 babies born will have or aquire hydrocephalus. Hydrocephalus may develop in the womb or after birth as a result of a congenital defect. This defect is not necessarily hereditary. Hydrocephalus may also result from complications associated with premature birth.

Children, young and middle-aged adults: Hydrocephalus may develop during these years as a result of intracranial bleeding, (stroke), brain injury, tumor growth, meningitis or other factors. Hydrocephalus that occurs after birth as a result of one of these factors is called “acquired hydrocephalus.”

Seniors: When it is diagnosed during these years, hydrocephalus is typically called “adult onset hydrocephalus.” It may take one of two forms: the common form of hydrocephalus which involves high intracranial pressure or Normal Pressure Hydrocephalus (NPH).

 

Causes, incidence, and risk factors

Hydrocephalus is due to a problem with the flow of a water-like fluid that surrounds the brain. This fluid is called the cerebrospinal fluid, or CSF. It surrounds the brain and spinal cord, and helps cushion the brain.

CSF normally moves through the brain and the spinal cord, and is soaked into the bloodstream. CSF levels in the brain can rise if:

  • The flow of CSF is blocked
  • It does not get absorbed into the blood properly
  • Your brain makes too much of it

Too much CSF puts pressure on the brain. This pushes the brain up against the skull and damage brain tissue.

Understanding the Ventricular System and CSF

The ventricular system is made up of four ventricles connected by narrow pathways. Normally, CSF flows through the ventricles, exits into cisterns (closed spaces that serve as reservoirs) at the base of the brain, bathes the surfaces of the brain and spinal cord, and then is absorbed into the bloodstream.
CSF has three important life-sustaining functions. It:
  • Keeps the brain tissue buoyant
  • Acts as a cushion or "shock absorber"
  • Acts as the vehicle for delivering nutrients to the brain and removing waste
  • Flows between the cranium and spine to compensate for changes in intracranial blood volume (the amount of blood within the brain).
The balance between production and absorption of CSF is critically important. Ideally, the fluid is almost completely absorbed into the bloodstream as it circulates; however, there are circumstances that, when present, will prevent or disturb the production or absorption of CSF, or that will inhibit its normal flow. When this balance is disturbed, hydrocephalus is the result.

 

  


Hydrocephalus in Infants & Small Children

1 in every 500 children born will have hydrocephalus, or develope hydrocephalus as a secondary condition.

 

 Hydrocephalus may begin while the baby is growing in the womb. It is common in babies who have a myelomeningocele, a birth defect in which the spinal column does not close properly. This woull be known as Congenital—Definition: A condition that is congenital is one that is present at birth.

Hydrocephalus may also be due to:

  • Genetic defects
  • Certain infections during pregnancy

In young children, hydrocephalus may be due to:

  • Infections that affect the central nervous system (such as meningitis or encephalitis), especially in infants
  • Bleeding in the brain during or soon after delivery (especially in premature babies)
  • Injury before, during, or after childbirth, including subarachnoid hemorrhage
  • Tumors of the central nervous system, including the brain or spinal cord
  • Injury or trauma

 

Symptoms of hydrocephalus depend on:   

  • Age
  • Amount of brain damage
  • What is causing the buildup of CSF fluid

Parents and older children need to be taught the signs and symptoms of shunt failure. They also need to have a management plan about who to contact and when to go to hospital. It can be useful for them to carry a protocol for managing a shunt failure in case the doctor they see is not familiar with signs of a shunt failure or with how to manage a shunt failure.

In infants with hydrocephalus, it causes the fontanelle (soft spot) to bulge and the head to be larger than expected. Early symptoms may also include:

  • Eyes that appear to gaze downward
  • Irritability
  • Seizures                                                                                                                                                   
  • Separated sutures
  • Sleepiness
  • Vomiting

Symptoms that may occur in older children can include:

  • Brief, shrill, high-pitched cry
  • Changes in personality, memory, or the ability to reason or think
  • Changes in facial appearance and eye spacing
  • Crossed eyes or uncontrolled eye movements
  • Difficulty feeding
  • Excessive sleepiness
  • Headache
  • Irritability, poor temper control
  • Loss of bladder control (urinary incontinence)
  • Loss of coordination and trouble walking                                                                                  
  • Muscle spasticity (spasm)
  • Slow growth (child 0 - 5 years)
  • Slow or restricted movement
  • Vomiting

Signs and tests

The doctor or nurse will examine the child. This may show:

  • Stretched or swollen veins on the baby's scalp
  • Abnormal sounds when the health care provider taps lightly on the skull, suggesting a problem with the skull bones
  • All or part of the head may be larger than normal, usually in the front part
  • Eyes that look "sunken in"
  • White part of the eye appears over the colored area, making it look like a "setting sun"
  • Reflexes may be normal

Head circumference measurements, repeated over time, may show that the head is getting bigger.

A head CT scan is one of the best tests for identifying hydrocephalus. Other tests that may be done include:

  • Arteriography
  • Brain scan using radioisotopes
  • Cranial ultrasound (an ultrasound of the brain)
  • Lumbar puncture and examination of the cerebrospinal fluid (rarely done)
  • Skull x-rays

Treatment

The goal of treatment is to reduce or prevent brain damage by improving the flow of CSF.

Surgery may be done to remove a blockage, if possible.

If not, a flexible tube called a shunt may be placed in the brain to re-route the flow of CSF.  The shunt sends CSF to another part of the body, such as the belly area, where it can be absorbed.

Other treatments may include:

  • Antibiotics are given if there are signs of infection. Severe infections may require the shunt to be removed.
  • A procedure called endoscopic third ventriculostomy (ETV), which relieves pressure without replacing the shunt.
  • Removing or burning away (cauterizing) the parts of the brain that produce CSF.

The child will need regular check-ups to make sure there are no further problems. Tests are regularly done to check the child's developmental and for intellectual, neurological, or physical problems.

Visiting nurses, social services, support groups, and local agencies can provide emotional support and assist with the care of a child with hydrocephalus who has significant brain damage.

 

Expectations (prognosis)

Without treatment, up to 6 in 10 people with hydrocephalus will die. Those who survive have different amounts of  intellectual, physical, and neurological disabilities.

The outlook depends on the cause. Hydrocephalus that is not due to an infection has the best outlook.

Complications

The shunt may become blocked. Symptoms of such a blockage include headache and vomiting. Surgeons may be able to help the shunt open without having to replace it.There may be other problems with the shunt, such as kinking, tube separation, or infection in the area of the shunt.

Other complications may include:

  • Complications of surgery
  • Infections such as meningitis or encephalitis
  • Intellectual impairment
  • Nerve damage (decrease in movement, sensation, function)
  • Physical disabilities
  • Constipation can put pressure on the end of the shunt, stopping it draining completely and it might be a factor in the development of a shunt malfunction. A doctor might recommend a high fiber diet and use laxatives to maintain regular bowel movements.

Multiloculated Hydrocephalus:
The term “multiloculated hydrocephalus” refers to the presence of an isolated CSF compartment or compartments within the ventricular system that may become enlarged despite a functioning shunt. It is most often caused by birth trauma, neonatal intraventricular hemorrhage, ventriculitis, shunt related infection or overdrainage. Because the condition is usually in infants and children who are already neurologically compromised, it can be difficult to recognize. Among several of the operative treatments are multiple shunt placement, multiperforated ventricular catheters, craniotomy and fenestration of intraventricular septations

Calling your health care provider

Seek immediate medical care if your child has any symptoms of this disorder. Go to the emergency room or call 911 if emergency symptoms occur, which include:

  • Breathing problems
  • Extreme drowsiness or sleepiness
  • Feeding difficulties
  • Fever
  • High-pitched cry
  • No pulse (heart beat)
  • Seizures
  • Severe headache
  • Stiff neck
  • Vomiting

You should also call your health care provider if the child has been diagnosed with hydrocephalus and the condition gets worse and you are unable to care for him or her at home.

 

Effects of hydrocephalus on learning and behaviour

Sometimes children with hydrocephalus can be seen as 'naughty' or 'disruptive', but often at least some of the difficulties they have can be due to their underlying learning difficulties, or to the cause of the hydrocephalus.                                                                                                                                       

Each child will also have 'good' and 'bad' days, like other children.

Effects can include:                                                                                                                                                                                                             

  • Fine motor skills
    • May have impaired dexterity affecting drawing and sports.
    • Handwriting can often be untidy
  • Spatial awareness
    • Poor eye hand co ordination, poor sense of direction, unsure balance.                                                                           
  • Short term memory impairment
    • Difficulty following detailed sequence of instructions
    • Difficulties learning new information
    • Forgetting things they have learned
    • Difficulties with comprehension
  • Social
    • Talking a lot and repetitive speech
    • Difficultly making and keeping friends
    • Content of conversation is superficial but well articulated
    • Use social jargon and words without fully understanding their meaning
    • Over-familiar manner
    • Inappropriate introduction of personal experience during conversation
  • Short attention span
    • May experience difficulty keeping to task.
    • They may not complete tasks, daydream and have difficulty following things when there is lots going on.
  • Diminished motivation/initiation
    • May sit and wait for instructions. Often this is misinterpreted as laziness.
  • Executive functions
    • Not thinking before acting, poor planning and organisation
  • Inability to generalise
    • Difficulty transferring concepts from one situation to a different one, especially in mathematics.
    • Difficulty transferring problem solving experiences from one situation to another.
  • Altered concept of time
    • No inclination to hurry.                                                                                      

Prevention

Protect the head of an infant or child from injury. Prompt treatment of infections and other disorders associated with hydrocephalus may reduce the risk of developing the disorder.


Hydrocephalus in Teens & Young Adults

1 Million known people are living with hydrocephalus
 

Hydrocephalus may develop during these years as a result of intracranial bleeding, (stroke), brain injury, tumor growth, meningitis or other factors. Hydrocephalus that occurs after birth as a result of one of these factors is called “acquired hydrocephalus.”

When the child is older and the fontanel and other sutures between the skull bones are closed, you no longer have the same warning signs.

Older children and adults may experience:

  • Headaches
  • vomiting
  • irritability
  • Swelling along the shunt tract occurs less frequently
  • Tired
  • Seizures
  •  Difficulty waking up and staying awake
  • Experience personality changes 

In the event of an abrupt malfunction, a person may develop symptoms very rapidly, in a matter of hours or days.Unless treated promptly, may go into a coma, and even death, may result.

The same testing techniques are used to diagnose hydrocephalus and malfunctions as in infants and small children.

A head CT scan is one of the best tests for identifying hydrocephalus. Other tests that may be done include:

  • Arteriography
  • Brain scan using radioisotopes
  • Cranial ultrasound (an ultrasound of the brain)
  • Lumbar puncture and examination of the cerebrospinal fluid (rarely done)
  • Skull x-rays

Complications

The shunt may become blocked. Symptoms of such a blockage include headache and vomiting. Surgeons may be able to help the shunt open without having to replace it.There may be other problems with the shunt, such as kinking, tube separation, or infection in the area of the shunt.

Other complications may include:

  • Complications of surgery
  • Infections such as meningitis or encephalitis
  • Intellectual impairment
  • Nerve damage (decrease in movement, sensation, function)
  • Physical disabilities
  • Short-term Memory issues

Living with a shunt

  • Avoid contact sports that may cause injury to the shunt valve or head injury. Football, lacrosse or other contact sports are not recommended. Encourage low impact sports such as tennis or swimming. A helmet should be worn for skiing or other sports that may cause head injury.

  • If a person suffers head injury they require close observation for signs of neurological changes and must be taken to a hospital if these develop. (For example they may develop a headache, start vomiting or become more drowsy or irritable than usual.)

  • Discourage the teen/adult from wearing handbags, shoulder bags, or backpacks on the side where the shunt tubing passes down the side of the neck, as pressure on the tubing may cause a break or kink in the tubing resulting in shunt malfunction.

  • Constipation can put pressure on the end of the shunt, stopping it draining completely and it might be a factor in the development of a shunt malfunction. A doctor might recommend a high fiber diet and use laxatives to maintain regular bowel movements.

 

Adults - Normal Pressure Hydrocephalus (NPH)

Normal pressure hydrocephalus (NPH) is an abnormal accumulation of cerebrospinal fluid (CSF) that causes the ventricles in the brain to become enlarged, sometimes with little or no increase in intracranial pressure (ICP).

NPH primarily affects people in their 60s and 70s. Scientists aren't certain how many older adults have this disorder because common symptoms of NPH are also common in other brain disorders.

We now know that the phrase “normal pressure” is misleading, because many patients have fluctuations in CSF pressure ranging from high to normal to low. However, normal pressure hydrocephalus, or NPH, continues to be the common name for the condition.

 

Diagnosis

There is no single test to determine if someone has normal pressure hydrocephalus. And even though the three hallmark symptoms listed above are considered the "classic" signs of this disorder, not everyone with NPH has all of these symptoms.

Brain imaging to detect enlargement of the ventricles, often with magnetic resonance imaging (MRI), plays a key role in diagnosing NPH. Several brain disorders, including Alzheimer's disease, can cause overall brain tissue shrinkage that makes the ventricles look larger than normal. In NPH, although the ventricles are enlarged, brain tissue may not appear shrunken.

Because the symptoms of NPH may overlap with those of Alzheimer's and other dementias, experts recommend that a person with suspected NPH undergo examination by a neurologist with extensive experience evaluating brain disorders that affect movement, thinking skills and physical functions.

If symptoms and an MRI strongly suggest NPH, a large-volume spinal tap may be used to identify those who may benefit from a shunt. In this procedure, doctors remove a larger-than-usual amount of spinal fluid, and then observe the person for 30 to 60 minutes to note any improvements in walking or thinking and reasoning. Most people originally suspected of having NPH do not improve following a CSF removal test.

It is most commonly seen in older adults, and is accompanied by some or all of the following triad of symptoms:

Symptoms

The following symptoms are considered hallmarks of normal pressure hydrocephalus:

  • Difficulty walking that's sometimes compared to the way a person walks "on a boat," with the body bent forward, legs held wide apart and feet moving as if they're "glued to the deck."

  • Decline in thinking skills that includes overall slowing of thought processes, apathy, impaired planning and decision-making, reduced concentration and changes in personality and behavior.

  • Loss of bladder control, which tends to appear somewhat later in the disease than difficulty walking and cognitive decline

These symptoms may not occur all at the same time, at the same level of seriousness and sometimes only one or two symptoms are present.


Causes and risks

In some cases, normal pressure hydrocephalus is caused by other brain disorders such as hemorrhages, infections or inflammation. But in most cases, the fluid buildup happens for unknown reasons

 

Is there any treatment?

Researchers have not found effective nonsurgical treatments for normal pressure hydrocephalus. Drugs that remove excess fluid throughout the body, such as diuretics, haven't been shown to help.

NPH can sometimes be treated with surgical insertion of a shunt, a long, thin tube that drains excess CSF from the brain to the abdomen. Difficulty walking is the symptom most likely to improve after surgery. Thinking changes and bladder control are less likely to get better. Shunting doesn't help everyone with NPH, and there's uncertainty about how best to identify those most likely to benefit.

More research is needed to:

  • Understand the prevalence of NPH
  • Show how the excess CSF involved in NPH causes symptoms affecting movement, thinking and bodily functions
  • Clarify the possible benefits of shunt insertion and who is most likely to benefit

The effectiveness of shunting in NPH has never been demonstrated in a randomized clinical trial. Most of these studies were small and followed people for a limited time. Available data suggest that difficulty walking is the symptom most likely to improve. Several studies found a significant rate of postsurgical complications. Findings also showed that short-term benefits of shunt insertion tended to decline over time.

 

What is the prognosis?

The symptoms of NPH usually get worse over time if the condition is not treated, although some people may experience temporary improvements. While the success of treatment with shunts varies from person to person, some people recover almost completely after treatment and have a good quality of life. Early diagnosis and treatment improves the chance of a good recovery. Without treatment, symptoms may worsen and cause death.