What is Hydrocephalus?
Hydrocephalus is a condition in which there has been an excess build up of CSF “Cerebrospinal Fluid” a clear fluid surrounding the brain. The excessive accumulation of CSF results in an abnormal dilation of the spaces in the brain called “Ventricles”, this dilation causes harmful pressure to build up on the brain and connective tissues.
- Hydrocephalus “Water on the Brain”, in Greek literally means “watery head”, “Hydro” meaning water, and “Cephalus” referring to the head.
- Hydrocephalus can happen at ANY age
- Hydrocephalus is life threatening and has a 75% chance of causing long lasting brain damage and motor disability, and a 30% mental disability rate.
- More than 50% of hydrocephalus is congenital “present at birth”
What are Ventricles?
The ventricular system is a set of four interconnected cavities (ventricles) in the brain, where the cerebrospinal fluid (CSF) is produced. Within each ventricle is a region of choroid plexus, a network of ependymal cells involved in the production of CSF. The ventricular system is continuous with the central canal of the spinal cord (from the fourth ventricle) allowing for the flow of CSF to circulate. All of the ventricular system and the central canal of the spinal cord are lined with ependyma, a specialized form of epithelium.
What is CSF “Cerebrospinal Fluid”?
Cerebrospinal Fluid, the clear fluid surrounding the brain and spinal cord has three critical functions:
- It acts as a “Shock absorber” for the brain and spinal cord to protect the brains connective tissue from harmful impact damage and excess movement.
- It acts as a river to flow nutrients to the brain and remove waste
- It flows between the cranium and spine to regulate changes in pressure within the brain.
Common Causes
A variety of medical problems can cause hydrocephalus. In many children the problem is there at “birth”, this kind of hyrocephalis is referred to as “congenital”. Most cases of congenital hydrocephalus are thought to be caused by a complex interaction of genetic and environmental factors “Exposure to radiation, mould, virus, bacteria, genetic defect, impact damage in utero, Car accident and so on”
- Aqueductal Obstruction “Stenosis” – The most common cause of congenital hydrocephalus is obstruction “narrowing or blockage” of the cerebral aqueduct “a long, narrow passageway between the third and fourth ventricle in the brain.” NO FEVER”
- Neural Tube Defects, or Myelomeningocele – Spina Bifida, meaning “Open spine”, actually refers to the condition in which the structures of vertebrae, muscles, ligaments, etc… supporting and protecting the spinal cord are impaired, not the spinal cord itself.
- Intraventricular Haemorrhage – Intraventricular haemorrhage is an acquired form of hydrocephalus and most frequently affects premature newborns. It occurs when small blood vessels lying alongside the ventricular lining of the brain rupture. This can lead to scarring of the ventricles or plug the arachnoid villi sites of CSF absorption.
- Meningitis & Bacterial / Viral Exposure – Meningitis is an inflammation of the membranes “Meninges” of the brain and spinal cord. It may be caused by bacterial infections or, less frequently, viral infections. FEVER
- Head Trauma – A head trauma can damage the brains tissues, nerves or blood vessels. Blood from these ruptured vessels may enter the CSF pathways and cause blockage or restricted flow.
- Tumours – In children, brain tumors most commonly occur in the back of the brain “posterior fossa”. as a tumor grows it may fill or compress the ventricles blocking the flow of CSF.
- Cysts – Arachnoid cysts are congenital in origin and may occur anywhere in the brain. In children, they are often located in the back of the brain and in the region of the third ventricle.
- Dandy-Walker Syndrome – Dandy-Walker Syndrome is where the fourth ventricle “last ventricle to the spine” is enlarged because of partial or complete closure of its outlets “no drainage of CSF”. Associated with developmental defects.
Symptoms “How to recognise it”
“If you suspect Hyrdocephalus DO NOT HESITATE, the Childs life is in Danger!”
In Infant age 0-1: Abnormal Enlargement of Babe’s head, soft spot “fontanel” is tense and bulging: scalp can appear thin “Stretched due to the swelling” prominent scalp veins, NO FEVER in congenital hydrocephalus, vomiting, drowsiness, irritability, downward deviation of baby’s eyes “Sun-setting eyes”, seizure, poor appetite.
In Child age 1-8: Headache “Rubbing at head or ears“, Nausea, vomiting, “NO FEVER in congenital hydrocephalus” lethargy, limp movements, unable to stay awake, poor coordination, change in personality, loss of motor functions, seizure and poor appetite, From Birth-Abnormal Enlargement of Babe’s head.
In Adult age 8+: Headache, difficulty remaining awake or waking up, loss of coordination or balance, bladder control problems, impaired “double vision”, cognitive impairment
Elderly – Loss of coordination or balance, shuffling gait, memory loss, headache, bladder control loss, forgetfulness, migraine like symptoms. Nausea, vomiting, drowsiness.
What can you do?
Take them to the Hospital Immediately! Hydrocephalus can easily be misdiagnosed as a viral infection and be prescribed antibiotics “This will not help in congenital hydrocephalus” NO FEVER and lead to potential for increased brain damage. Hydrocephalus needs immediate surgery to minimize brain damage and potential for death.
Diagnosing Hydrocephalus
Parents should remember that this is “Not” your fault”
Your Doctor will recommend a course of treatment based on what they find, they will review medical history and perform examinations including a complete neurological examination with diagnostic testing. “Do not be afraid to ask questions regarding symptoms / options / treatments”, anything that you can think of to help narrow down the cause can impact the treatments available. The neurological examination will also help to determine the severity of the child’s condition. Further tests such as ultrasound “if the patient is an infant”, computed tomography “CT or CAT scan”, or a magnetic resonance imaging “MRI” may be ordered. The more your neurosurgeon knows the more likely the chance to minimize brain damage and death. They will also ask for information regarding head circumference at birth along with birth weight, health, defects and head development.
Treatments?
Hydrocephalus can be treated in only three or four ways with current medical technology “2018“. The problem area may be treated directly “removing the cause of CSF obstruction”, or indirectly by diverting the fluid somewhere else. In some cases, two procedures are performed, one to divert the CSF temporarily, and another on a later stage to remove the cause of the obstruction.
- Shunt – Often in the emergency setting hydrocephalus is treated at the beginning with a “Shunt”. A shunt is a flexible but sturdy silastic tube. A shunt system consist of the shunt, a catheter, and a valve. One end of the catheter is placed within a ventricle inside the brain, but also may be placed potentially within a cyst or site close to the spinal cord. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites within the body so excess CSF can be absorbed. “Shunt systems are not perfect devices” Complications may include mechanical failure, infections, obstructions, length, improper flow “too much or too little CSF” or deterioration. Each time the Shunt has a malfunction potential for brain damage increases exponentially.
- Obstruction Removal – Neurosurgery is performed to remove the blockage, cyst, tumor that is causing the blockage of CSF flow. CT – Cat scans and MRI’s are needed to see if this is an option, your Neurosurgeon will know once they have all the data and information for them to assess weather removing the obstruction is possible.
- Endoscopic Third Ventriculostomy “ETV” – This surgery involves making a hole in the floor of the third ventricle to allow free flow of spinal fluid into the basil cisterns for absorption of CSF. Many neurosurgeons “do not” perform ETV on children below the age of 2 due to the failure rate of up to 40%. However, ETV has the benefit of consistent pressure, flow and the lack of non natural parts within the body, this reduces the chance of malfunction leading to brain damage or death. Depending on the hydrocephalus ETV has a patency rate for up to five years with a 50%-80% no malfunction rate. “Candidacy” – ETV is clearly appropriate for treating “obstructive non-communication hydrocephalus” It is controversial as to weather it is effective in treating non-obstructive communication hydrocephalus. Although some neurosurgeons have used it successfully in these cases with a 1.0% infection rate.
- Endoscopic Third Ventriculostomy & Choriod Plexus Cauterization “ETV & CPC” – This technique combines ETV with a procedure called Choroid Plexus Cauterization “CPC”. CPC is where your neurosurgeon in the middle of the ETV inserts a specialized tool “wire” to cauterize the CP tissue beginning at the right foramen of Monro, and following it back to the typically mobile glomus choroideum in the atrium. Your neurosurgeon will have more details to explain the procedure, however by combining ETV and CPC the chances of successfully holding proper Intracranial CSF fluid pressure increase, infection rates decrease to 1.0% or less and raise the success rate of the procedures in general.
Communication VS Non-Communicating Deffinition
- Communicating – Communicating Hydrocephalus is when the flow of CSF is blocked after exiting the ventricles, however CSF is still able to flow between the ventricles but is blocked from draining out.
- Non-Communicating – Non-Communicating also known as “Obstructive” Hydrocephalus occurs from “Aqueductal stenosis” a narrowing of the tube “aquaduct of Sylvius” that flows between the 3rd ventricle and 4th ventricle before exiting to the spine. “This is the most common cause of Hydrocephalus”
Long Term Outcome
“There is Hope” – The prognosis for patients diagnosed with hydrocephalus is difficult to predict, although there is some correlation between the specific “cause” of the hydrocephalus, the time between when symptoms began, diagnosis & treatment, as well as the age of the patients can influence the outcome. Brain Damage and complications are further compounded by the presence of associated disorders, the degree to which decompression “relief of CSF pressure or build-up” following shunt placement can also further complicate risks. Parents and guardians should be aware that up to 70% of hydrocephalus patience suffer from both cognitive and physical development disorders.
However “There is Hope” – Those affected by hydrocephalus benefit from rehabilitation therapies and educational interventions, many children go on to lean normal lives with few limitations. Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts are critical to a positive outcome.
Although most children with hydrocephalus are within the normal range of intelligence, most experience “specific” learning difficulties. Immediate memory and auditory/verbal information may be intact, however, there is a rapid loss of information over time and difficulty in retrieving the appropriate bit of information due to brain tissue damage. Most students find it hard to organize themselves, plan ahead and think flexibly. In addition, some may experience difficulty in understanding the passage of time or understand when matters are urgent.
There are many resources out there to help you guide your child through this difficult emergency. For more information contact your physician and don’t be afraid to ask questions or seek support groups.
“This material is for information purposes only and is taken from the Hydrocephalus Association and Hydrocephalus Foundation. It should not be used in place of medical advice, instruction, and/or treatment. If you have questions, speak to your doctor or appropriate healthcare provider.”
In any emergency just remember to Protect Yourself!!! Call 911!!! Don’t Waste Time!!!
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