Hydrocephalus

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What is Hydrocephalus?

Hydrocephalus is a condition that results when there is an abnormality in the circulation of the cerebrospinal fluid that causes an accumulation of the cerebrospinal fluid in the ventricles of the brain increasing the pressure on the brain. The ventricles are spaces and cavities that are present in the brain which has a fluid within them called the cerebrospinal fluid (CSF) which circulates the brain, spinal cord. The accumulation of the CSF results in the loss of several vital activities of the body apart from the symptoms of increased pressure inside the skull.

Is Hydrocephalus a Medical emergency?

Hydrocephalus can present as a medical emergency. In babies, symptoms like a high-pitched cry, not wanting to feed, repeated vomiting, not moving the head, episodes of fits, indicate the need for emergency medical help. Similarly, in adults, uncontrolled headache fits, confusion, should prompt emergency medical attention.

Types

Hydrocephalus could be one of the following types:

  • Communicating Hydrocephalus: All the brain spaces (ventricles) and the space in the spinal cord (spinal canal) are not blocked, yet, there is an accumulation of CSF due to poor absorption of CSF back into the bloodstream.
  • Non-communicating Hydrocephalus: This is when there is a blockage in the brain spaces. This is also called obstructive Hydrocephalus.
  • Normal Pressure Hydrocephalus NPH: There is an accumulation of the cerebrospinal fluid in the ventricles of the brain without an increase in intracranial pressure.

Each type can be further classified as congenital (birth defect) and acquired (during life).

Causes

An accumulation of Cerebro Spinal Fluid (CSF) happens due to either excess production or due to poor removal of the CSF from the system. CSF is produced by a specialized tissue that lines the inner surface of the brain’s ventricles. It is absorbed by the bloodstream. These two events happen simultaneously, thereby maintaining a balance of the CSF. Apart from these, there could be a blockage in the connections between the ventricles in the brain or the connections between the ventricles and the spinal cord. This blockage could result in a build-up of pressure inside the brain.

Risk factors

Factors that contribute to the risk of developing Hydrocephalus are:

  • In newborns:
    • Abnormal growth of the central nervous system. This creates a blockage to the normal flow of CSF.
    • Bleeding into the brain spaces. A common cause of this in a newborn is the premature birth of the baby.
    • When a woman is pregnant, if infections like Syphilis or Rubella infect the baby in the womb, the baby’s brain tissue might become inflamed.
  • In all age groups:
    • Tumors developing in the brain or the spinal cord
    • Serious injury to the head
    • Infections of the brain like bacterial meningitis, viral meningitis, etc
    • Bleeding into the brain. This could happen due to a major injury or due to rupture of blood vessels

Signs & symptoms

The symptoms and signs of Hydrocephalus are different in different age groups.

  • In infants:
    • The head grows in size very rapidly and becomes unusually large. An infant’s head has some soft spots as the skull bones don’t fuse. These soft spots are called ‘Fontanelle’. These are found to be bulging and feel very tense.
    • The baby might not feed properly and might frequently vomit
    • The baby appears very restless and might have fitted as well
    • The muscles might be lacking strength
  • In slightly older children:
    • Headache
    • Vomiting
    • The eyes might deviate downwards always
    • The child might feel drowsy for most of the day
    • The child might not be able to balance the body and might not be able to hold back urine
    • Episodes of fits are also common
    • A marked change can be noticed in the child’s behavior
    • Poor performance at school
    • An unusual loss or decrease in the abilities which the child previously acquired
  • In adults, the following features may be noticed:
    • Headache
    • Feeling weak for most of the day
    • Disturbances in vision
    • Inability to maintain body balance
    • Unable to hold back urine
    • Poor performance at work due to memory problems, inability to focus, decrease inability to plan and execute tasks, etc.

Investigations

Initially, a doctor makes a note of all the symptoms a person is experiencing. Then, a detailed neurological examination is done by the doctor. This includes testing the strength of the muscles, the sensations, the level of body balance and coordination, the sharpness of reflexes, and the vision. The commonly employed tests are

  • Ultrasonography: This works on the principle of producing sound waves with which images are created. This is particularly used in newborns and to detect brain abnormalities while the baby is in the womb.
  • Magnetic Resonance Imaging (MRI): An MRI creates 3-D images of the brain by creating a magnetic field around the body.
  • Computerized Tomography (CT): This produces X-Rays to visualize the brain.

Both CT and MRI scans require the person to remain unmoving while the test is being performed. Thus, when they are used for children, a sedative has to be given to put the child to sleep for some time.

Diagnosis

A diagnosis of Hydrocephalus is established based on medical history, clinical evaluation, and results of the investigations done.

Treatment options

Medical treatment

Medications have almost no role in the management of Hydrocephalus.

Interventional treatment including surgery and indications for surgery/ Surgical treatment

Surgical interventions are required for the management of Hydrocephalus. Two approaches are commonly followed:

  • Shunt Surgery: A shunt is a flexible tube that has a valve. One end of this tube will be placed in the ventricles of the brain. The shunt passes underneath the skin to finally reach a space where the excess CSF can be drained. Such spaces include the abdomen and the heart chambers.
  • Endoscopic Third Ventriculostomy: When a small opening is made at the bottom of the ventricle or in between the ventricles, it is called Ventriculostomy. This allows the excess CSF to flow out of the ventricles. This surgery is done by making very tiny openings in the skull to pass a video camera into the brain.

Role of diet/ Exercise/ Lifestyle changes/ Preventive measures

Rehabilitation is necessary for people who are on the recovery path,

  • Physical therapy: This includes training to achieve the best possible physical efficiency
  • Occupational therapy: The person is offered training to return to normality at the workplace

Complications

The possible complications include:

  • Defects in the development of a child. These defects could be in terms of brain growth, physical growth, or both. This risk is highest when the Hydrocephalus has developed while the baby was in the womb and has worsened by the time of birth.
  • Adults also develop defects in their brain function. Such individuals may not recover properly and might have permanent disabilities.

Prognosis

The outcome of treatment depends on factors like:

  • Severity of symptoms
  • Early diagnosis
  • The person’s response to treatment

It has been observed that most people who receive appropriate therapy at the right time recover well. The quality of life, in the long run, is quite good. However, delays in diagnosis and treatment or a completely neglected Hydrocephalus result in a bad outcome. It may even result in death.

When to contact the doctor? / How to identify the complications?

Whenever a person experiences symptoms suggestive of a brain disease or if a baby or a child shows such features, immediate medical attention is required.

Indications for hospitalization if required

Hospitalization will be required for the surgical management of Hydrocephalus. The tests can, however, be done on an outpatient basis. Complications due to lack of treatment or due to surgery will also need hospitalization.

Suggested clinical specialists/ Departments to consult for this condition

  • Neurology
  • Neurosurgery
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