What is Hydrocephalus?

A diagnosis of Hydrocephalus can be extremely worrying. However, with the right treatment and careful clinical care, you can still lead a relatively normal life for years to come. Here, we explain exactly what your diagnosis means, and what kind of treatment you can expect. At Sophysa, our aim is to provide you with valuable information that you can discuss with your medical team, and what kind of prognosis you may have as a result of new and innovative treatments that are now available, including surgery and implanted shunt systems.

What is happening to me?

An introduction to Hydrocephalus

You have been diagnosed with Hydrocephalus which is a disease which results from the accumulation of Cerebrospinal Fluid (CSF) into the brain ventricles. There are several types and forms of Hydrocephalus, as well as different therapies and treatments which can include everything from drugs to surgery.

Hydrocephalus is an incurable disease which must be treated permanently to relieve pain and minimize the effect of disorders. Surgery and implantation of a valve (also called a shunt) is one possible treatment.

Before explaining to you what a shunt is, let’s look closer at what Hydrocephalus really means.

What is Hydrocephalus ?

The word hydrocephalus comes from two classical Greek words: húdôr (hydro) which means “water” and kephalě (cephalus) which means “head”.

Today, the term hydrocephalus indicates an excess of fluid within the cranium. This is a pathological condition which occurs as a result of an imbalance between the production and absorption of this fluid, the Cerebrospinal Fluid – CSF.

Under normal conditions, circulation and absorption of the CSF create a perfect equilibrium between secretion and absorption of CSF. The average CSF pressure (often called intracranial pressure (ICP)) in adults when lying down is 80-200 mmH2O (6 to 15 mmHg). The pressure is almost zero or even negative when standing up.

But this equilibrium can be disrupted, causing Hydrocephalus. The condition only develops if the CSF is unable to leave the ventricular cavities or if its absorption is disturbed. The excess CSF will remain in the ventricles, which become much bigger.

As the size of the cranium is fixed (except for children up to around 18 months of age in whom the fontanelles are still open), any increase in the volume of fluid within the ventricles affects the brain. This can lead to the development of neurological symptoms due to an increase in the intracranial pressure (ICP), depending on brain compliance for instance.

Symptoms are different between adults and infants, and depend on the patient’s age. Infants and young children, whose cranial bones have not yet completely fused together, will have symptoms different from those of adults.

The clinical manifestations of hydrocephalus occur as a result of ventricular dilatation and of the increased pressure within the cranium.

The usual symptoms of hydrocephalus in infants include:

  • abnormal increase in head circumference and bulging fontanelles
  • downward shift in gaze (sunset eyes)
  • dilatation of the veins on the surface of the cranium
  • vomiting and behavioural difficulties (irritability, drowsiness, etc.) or even seizures.

Depending on the cause, raised intracranial pressure may produce different signs in older children and adults. The major signs are:

  • Headaches and vomiting
  • Visual disturbance (blurred vision, double vision, etc.) With papilloedema (a swelling of the optic disc), which is seen when the fundus of the eye is examined
  • Consciousness disorders (drowsiness, progressive lethargy or even coma).

Other signs may be found and are systematically looked for by doctors. These include bradycardia or seizures.

The characteristic Hakim’s Triad may be seen in normal pressure hydrocephalus, which is found mostly in adults:

  • Psychiatric disorders, which can mimic some symptoms of dementia. This involves mostly slow and poor quality ideation and activity, with apathy and indifference, serious memory loss and orientation disturbance, particularly in time, loss of attention and unawareness of reality.
  • Gait disorders, with instability. This is due to static abnormalities, which may develop into titubation. The person moves around slowly with care and may, occasionally, walk on the spot. Turning around, or either starting or stopping suddenly leads to imbalance.
  • Sphincter disturbance (incontinence). On occasions, the patient may become incontinent. It is not clear whether this is due to inattention, reduced awareness, or urgency of micturition.

Why do I have Hydrocephalus?

There are two different forms of hydrocephalus:
  • Congenital, if it develops during pregnancy, at birth, or later because of a birth condition that prevents the CSF from circulating and getting drained properly. Congenital hydrocephalus may be caused by a fault during foetal development, a pregnancy disease such as toxoplasmosis or cytomegalovirus (a type of herpes virus), or genetic inheritance.
  • Acquired, if it develops after birth. It can affect any patient of all ages and is diagnosed, for example, after a head injury, meningitis, cerebral haemorrhage, or neoplastic disease.
Hydrocephalus is commonly classified into two types:
  • Non-communicating or obstructive hydrocephalus: it is caused by the blockage of CSF circulation in the ventricular cavities. This blockage usually occurs at the level of the aqueduct of Sylvius and may also be seen in the foramina of Monro and other foramina. This type of hydrocephalus is commonly associated with clinical signs of raised intracranial pressure. The major causes are congenital stenosis of the aqueduct of Sylvius, Arnold Chiari Syndrome (Spina Bifida) or tumours of the posterior fossa.
  • Communicating or non-obstructive hydrocephalus: it occurs when the CSF circulation around the brain is disturbed or if the absorption sites are non-functioning. The major causes of communicating hydrocephalus may be grouped into two categories:
    • Hydrocephalus due to excessive production of CSF caused by a choroid plexus papilloma, a very rare tumour,
    • Hydrocephalus due to impaired re-absorption of CSF

What are the available treatments?

Hydrocephalus is an unpredictable disease, but it can be controlled to relieve pain and disorders. Two medical therapies exist but they are only short-term treatments, which means pain and disorders will be relieved only temporarily and treatment has to be assessed on an ongoing basis.

To control communicating hydrocephalus, surgery is the only truly effective long-term treatment. One of the most effective therapies available is CSF shunting. These have been performed for decades and still represent the most important advance made to date in the treatment of hydrocephalus. A CSF shunt involves establishing an alternative pathway for the movement of CSF in order to bypass an obstruction of the natural pathways.


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