What is hydrocephalus?
Hydrocephalus is a neurological condition in which fluid accumulates in the brain due to a blockage. The ventricles swell and put pressure on the brain. This condition can cause serious damage to your brain and its ability to process information.
How common is hydrocephalus?
It is estimated that 120,000 Canadians are living with hydrocephalus. It is more common than Down’s Syndrome or brain tumours. 90% of people with spina bifida, also have hydrocephalus. It is the most common reason for brain surgery in children.
What causes hydrocephalus?
Hydrocephalus can be present at birth or be acquired later in life as a result of a tumor, head injury, meningitis or hemorrhage. Congenital hydrocephalus can occur in isolation or may be associated with other conditions such as spina bifida, dandy walker syndrome, aqueductal stenosis, arachnoid cysts and porencephaly.
Who is at risk for developing hydrocephalus?
Anyone can develop hydrocephalus at any time during their lifespan. The most common causes of hydrocephalus in children are brain bleeds as a result of prematurity, spina bifida, brain tumors, infection and head injury. Acquired hydrocephalus can also result due to a concussion or head injury, brain tumor or cyst, intracranial bleeding (stroke), meningitis and other unknowns. Those aged 55 and older are at risk of developing Normal Pressure Hydrocephalus as it is often associated with the aging process. It is frequently misdiagnosed as dementia or Parkinson’s or Alzheimer’s.
What challenges are associated with Hydrocephalus?
Hydrocephalus is a very serious condition that, in most cases, requires medical attention. In some instances, if left untreated, it can cause permanent brain damage and even death. For some, delaying treatment can cause lasting injury to the brain and cause a wide range of issues ranging from difficulty breathing, poor muscle coordination and mobility challenges to difficulties with vision, fatigue, headaches, seizures, incontinence and hormonal imbalances. Challenges with learning, social skills, memory and problem solving are also common.
How Does Hydrocephalus Affect a Person?
It differs from person to person depending 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.
Is there a cure for hydrocephalus?
Hydrocephalus is a chronic neurological condition. That means there is no cure and, in most cases, the person affected will live with the condition for life. In some instances, if hydrocephalus develops due to a blockage like a brain tumour, sometimes removing the blockage or tumour will allow the cerebral spinal fluid flow to return to normal.
What is the treatment for hydrocephalus?
The most common treatment for hydrocephalus is the surgical placement of a shunt, a device that drains excess cerebral spinal fluid (CSF) from the brain to another part of the body. A shunt is a flexible piece of tubing that is attached to a valve that controls the flow of CSF.
Endoscopic Third Ventriculostomy (ETV) surgery is an alternative to shunting for some individuals. An ETV consists of making an opening in the floor of the ventricle at the base of the brain. The CSF is then no longer blocked and can flow around the brain. An ETV can also fail or become blocked. Not everyone with hydrocephalus is a candidate for ETV surgery.
What causes shunt failure?
In some cases, the shunt may totally cease to function without any adverse consequences. Most often shunts get blocked by tissue, blood cells or bacteria growths. Obstructions may occur in any of the shunt parts. The shunt may also be blocked if the parts become separated or if the position of the shunt changes. Overdrainage and underdrainage can also occur.
How common are shunt revisions?
Shunts can stop working or malfunction at any time. Some individuals may need few, if any revisions. Others may have ongoing problems requiring many revisions. It cannot be predicted how many revisions a person may undergo.
How long is the hospital stay after surgery and what follow-up is required?
Time spent in hospital can range from 1-2 days to a week or more depending on the individual. After discharge, a follow-up appointment is usually made for six weeks post-op. There may be staples or stitches that need to be taken out and your doctor or a nurse will discuss this before leaving the hospital. Upon returning home an individual should monitor their recovery and be aware of the signs of shunt malfunction and infection. Seek medical attention immediately, if you notice any of those signs or symptoms.
What is Normal Pressure Hydrocephalus (NPH)?
NPH is a type of hydrocephalus that usually occurs in people aged 55 and older. It is different than other types of hydrocephalus because it develops slowly over time. The cerebral spinal fluid (CSF) is blocked gradually, and the excess fluid builds up slowly. This means the fluid pressure in the brain may not be as high as in other types of hydrocephalus. The ventricles still become enlarged and press on the brain causing the triad of symptoms loss of bladder control, altered walking gait and a decline in cognitive processes such as memory and problem solving.
How is NPH diagnosed?
In most cases if NPH is suspected, your family doctor will refer you to a neurologist. The neurologist may do an exam that includes evaluation of how you walk, process information, speak and inquire about your bladder function. They may also order an MRI, CT scan or a lumbar puncture.
The symptoms of NPH are similar to Alzheimer’s and Parkinson’s diseases, however, the combination of dementia-like symptoms, altered walking (shuffling feet, short steps, a feeling like one’s feet are stuck to the floor) and urinary incontinence should alert your health care provider to consider NPH. If Normal Pressure Hydrocephalus is determined to be likely, then a referral to a neurosurgeon is the next step to discuss treatment options.
How is NPH treated?
The most common treatment for NPH is the surgical placement of a shunt, a device that drains excess cerebral spinal fluid (CSF) from the brain to another part of the body. A shunt is a flexible piece of tubing that is attached to a valve that controls the flow of CSF.
Endoscopic third ventriculostomy (ETV) surgery is an alternative for some individuals to shunting. An ETV involves making an opening in the floor of the third ventricle at the base of the brain. The CSF can flow around the brain through the new pathway. An ETV can also fail or become blocked. Not everyone with hydrocephalus is a candidate for ETV surgery. A neurosurgeon will make the determination of the best course of treatment on a case-by-case basis.
Can NPH be cured?
It is a long-term condition, however, if diagnosed early and with treatment, many of the symptoms of NPH can be alleviated and in some cases reversed.
What kind of lifestyle can I expect to have while I have NPH?
The symptoms of abnormal gait, dementia and bladder control may improve within days of shunt surgery, or may take weeks to months to abate. Generally, individuals with a shunt are not restricted in their daily activities, except those involving great physical exertion. Most people can look forward to a normal future. But because hydrocephalus is an ongoing condition, follow-up and long-term care by a neurosurgeon or neurologist is sensible and encouraged. Especially if symptoms come back.
SPINA BIFIDA FAQ’s
What is spina bifida?
Spina bifida is a neural tube defect that occurs within the first four weeks after conception. It is a neurological condition that people are born with. A portion of the neural tube does not develop or close properly. Bone, muscle and skin cannot form around the spinal cord where the opening occurred and nerves surrounding the area may be damaged or improperly formed.
How common is spina bifida?
Approximately 26 in 100,000 babies in Canada are born with some form of spina bifida.
What causes spina bifida?
The exact cause is not known. Development of spina bifida is the result of a number of factors. These may include genetics, the mother’s diet, a lack of folic acid and several unidentified environmental factors present before and during conception and in early pregnancy may contribute to the development of the condition.
Is spina bifida preventable?
There is still much to learn about the prevention of spina bifida. However, medical research has proven that folic acid can reduce the risk of having a neural tube defect (NTDs) affected pregnancy, by as much as 70%. Folic acid is essential for the development of a baby’s spine, brain and skull in the early weeks of pregnancy. Any woman who can become pregnant risks having a baby with an NTD. It is recommended that all women who can become pregnant take a multivitamin with at least 0.4 mg of folic acid every day.
Breakthrough treatment of in-utero fetal surgery to close the baby’s back while they are still in the womb may prevent further damage and offer better results than a traditional postnatal repair. It may also reduce the development of hydrocephalus and improve mobility outcomes.
How does spina bifida affect a person and what challenges associated with the condition?
Spina bifida is a life-long chronic neurological condition that can result in mild to severe paralysis depending on the extent of nerve damage and location of the cele. The impact may include partial or total paralysis, loss of sensation in lower extremities, challenges with bowel and bladder function, difficulty with hand skills, vision and hearing, learning, social skills, memory and problem solving are also common.
What is the treatment for spina bifida?
People with spina bifida require life-long medical care. Treatment occurs shortly after birth for babies who have not had the in-utero fetal surgery and whose spinal cord and nerves are exposed. Surgery is performed by a neurosurgeon to close the opening and reduce the risk of further damage to the spinal cord and nerves. A person with spina bifida, will likely require bowel, bladder and orthopedic treatment throughout their life-span. Many while also need physical therapy, mobility supports such as braces, crutches, walkers or wheelchairs at various stages of life.
Are people with spina bifida at increased risk of developing osteoporosis?
50% of people with spina bifida will develop osteoporosis. Research shows that it occurs at a younger than the general population. Children and adults with spina bifida have lower bone density in their lumbar spine and in their hips than the general population. This is true for those who ambulate and those who use wheelchairs, but is higher in those that don’t walk. Decreased bone density may also be due to medical conditions, medications or surgeries.
Latex Allergy and spina bifida-What do I need to know?
Studies indicate that up to 73% of individuals with spina bifida have a latex sensitivity and/or allergy. Latex is the sap from the rubber tree. It is used in a variety of common items like balloons, erasers, surgical gloves, contraceptives and elastic fabrics. Sensitivity to latex may cause symptoms such as watery eyes, runny nose, sneezing, rashes, hives, swelling and itching on contact and in severe cases, respiratory distress. Unfortunately, as with most allergies, the only effective treatment is avoidance. Using latex free products as much as possible is recommended. Consult an allergist for further information.
How can I get help for symptomatic spina bifida occulta when my primary care provider says that it does not cause pain?
Occulta is a minor deformity of one or two vertebrae that doesn’t get much attention, yet it can significantly decrease quality of life and cause pain for those who develop secondary neurological issues such as tethered cord. This can become a serious issue and requires a thorough neurosurgical assessment of symptoms and most likely an MRI or CT scan to determine what is causing the pain and what treatments are available.