Hydrocephalus is the build-up of cerebrospinal fluid (CSF) in areas called ventricles situated deep within the brain.

CSF has three important functions:

• it protects the brain from damage
• it removes waste products from the brain
• it provides the brain with the nutrients it needs to function properly. (Source: NHS)

The brain continually produces CSF. Old CSF is released from the brain and absorbed into the blood vessels. Hydrocephalus disrupts this normal process.

Types of Hydrocephalus

There are two types of Hydrocephalus: Communicating and Obstructive. Both lead to the accumulation of CSF within the brain, causing pressure inside to become raised. This is dangerous if left untreated.

Obstructive Hydrocephalus involves a physical blockage within the brain, preventing CSF from circulating normally.

In Communicating Hydrocephalus, CSF is able to circulate, but it doesn’t get absorbed properly. This type of Hydrocephalus often develops at a much slower rate.

Causes

In infants and children, the cause of hydrocephalus can be something they are either born with (congenital) or from events/factors occurring after birth (acquired).

Obstructive Hydrocephalus can be caused by developmental anomalies with a genetic cause or cranial anomalies. Acquired causes include tumours, infection, or haemorrhage.

Communicating Hydrocephalus can similarly be caused by haemorrhage, particularly in pre-term babies. Other causes include trauma, surgery, infections including TORCH and inflammation of the brain (chemical arachnoiditis).

Finally, it can occur in individuals born with spina bifida due to the disruption of CSF flow caused by the condition.

Presentation and symptoms

Babies born with hydrocephalus often have distinct physical features. These include:

• a large head or one that is growing quickly and crossing centiles
• a tense or bulging fontanelle (the soft spot on top of a baby’s head)
• downward looking eyes (also known as sunsetting)
• tiredness and not waking for feeds
• severe vomiting
• irritability
• poor feeding and swallowing.

Symptoms in older children include:

• head and neck pain
• nausea and vomiting
• drowsiness and irritability
• blurred/double vision
• difficulty walking, poor coordination and lower limb weakness
• urinary and bowel incontinence.

(Source: NHS)

Diagnosis

Brain scans (CT and MRI) can be used to diagnose Hydrocephalus. Doctors will look for enlarged ventricles in addition to other features of elevated pressure.

Treatment

Treatment usually involves inserting a thin tube known as a shunt into the brain to drain away the excess fluid.

A procedure known as an endoscopic third ventriculostomy, or ETV, can sometimes be used as an alternative. This involves the surgical opening of the third ventricle in order to allow trapped fluid to escape to the surface, where it can then be absorbed.

In obstructive types of hydrocephalus, the causative lesion may also be removed.

Importance of timely treatment

Hydrocephalus can lead to permanent brain damage, developmental delays and other complications, including blindness. If left untreated, the condition can be fatal.

Although the condition itself is often unavoidable, timely treatment is essential.

The severity and duration of the elevated pressure within the brain are two vital factors that significantly influence the resulting impact and prognosis.

Support

For those affected by hydrocephalus, there are a number of charities that may be able to provide advice and support.

• The Brain Charity provides free support for carers, friends and family of people with any form of neurological condition, including hydrocephalus, from anywhere in the UK.
• The Charity SHINE can offer advice and support for parents of children with Hydrocephalus. Call them on 01733 555 988 or visit their website. They also have a useful Resource Hub for hydrocephalus.
• Hydrocephalus UK is a UK charity focusing solely on the condition and its impact. You can find more information on their website.

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