Hydrocephalus - What Is It?

Spina bifida and hydrocephalus:

Spina bifida is often associated with hydrocephalus, which occurs when structural abnormalities develop before birth in parts of the brain, preventing proper drainage of the CSF, and further compressing the brain. When a baby has open spina bifida, the CSF may have nowhere to go after the surgery to close the spina bifida lesion, even though the baby may not have enlarged ventricles before birth. The CSF accumulates in the ventricles, resulting in hydrocephalus. Early after back surgery, the baby's head circumference can usually be checked for this, and it can be treated accordingly. As the CSF flow is disrupted by Chiari II, some babies with spina bifida develop hydrocephalus in the womb.

Which types of hydrocephalus are there?

People of all ages can develop hydrocephalus, but it is more common among infants and older adults. The two major types of hydrocephalus are communicating hydrocephalus and non-communicating hydrocephalus. Some of these cases may be associated with abnormalities in the brain and spinal cord during pregnancy.

The communicating hydrocephalus occurs when the flow of CSF between the ventricles is blocked after it leaves them. The passages between the ventricles remain open even when the flow of CSF is blocked. It is also possible for CSF to build up in the ventricles and communication hydrocephalus if the flow and absorption of CSF to specialized blood vessels called arachnoid villi is reduced. 

Whenever a narrow passage connecting the ventricles is blocked, non-communicating hydrocephalus occurs.

Symptoms of the condition?

In order to receive proper diagnosis and treatment for hydrocephalus, it is important to see a doctor. Hydrocephalus symptoms vary significantly from person to person and mostly depend on age.

Infants with hydrocephalus may show the following signs and symptoms:

• Increased head size in a short period of time

• Head that is unusually large

• On the top of the head, there is a bulge in the soft spot (fontanel)

• vomiting

• Feeding or sucking problems

• Tiredness

• Feeling irritable

• Fixation of the eyes downwards (also referred to as "sun setting") or inability to turn the eyes outwards

• seizures.

Older children, young adults, and middle-aged adults may experience symptoms such as:  

• headache

• Double vision or blurry vision

• Vomiting or nausea

• Balance issues

• The development of certain skills such as walking and talking is slowed or stopped

• Problems with vision

• Performance declines at school or at work

• Coordination problems

• Frequent urination and/or loss of bladder control

• Sleep disturbances or difficulty waking up

• Tiredness

• Feeling irritable

• Loss of memory or changes in personality.

Elderly adults may experience symptoms such as:

• Feet feel "stuck" when walking, often described as "problems"

• The progression of dementia and mental impairment

• Movements are generally slower

• Inability to control bladder function and/or frequent urination

• Balance and coordination are poor.

Currently, what treatment is available?

Surgery can be used to treat hydrocephalus in two ways:

• An intracranial shunt (tube) drains excess fluid into the chest cavity or abdomen through a flexible tube inserted under the skin. 

• An endoscopic Third Ventriculostomy (ETV) improves the flow of CSF from the brain by opening a tiny hole in the third ventricle. In order to reduce CSF production, it can sometimes be combined with choroid plexus cauterization. A choroid plexus cauterisation burns the choroid plexus (i.e., the tissue that produces CSF) in the lateral ventricles of the brain, resulting in less CSF being produced.

As a general rule, shunt systems work well, but they can fail to drain CSF properly due to mechanical failure or infection. When this happens, CSF begins to build up again in the brain, causing earlier symptoms to return. In order to reduce the buildup of CSF, a clogged shunt system is replaced to allow it to drain properly. Shunts require regular medical checks and monitoring. Throughout the lifetime of a person, multiple surgeries may be necessary to repair or replace a shunt.  Seek medical help immediately if symptoms develop that suggest the shunt system is not working properly.
Signs and symptoms of shunt malfunction may include:

• headache

• Light sensitivity or double vision

• Feeling sick or nauseated

• Muscle pain in the neck or shoulders

• seizures

• Tenderness or redness along the shunt tract

• Fever of low intensity

• Exhaustion or sleepiness

• Recurrence of symptoms associated with hydrocephalus.