Congenital Hydrocephalus Content from the guide to life, the universe and everything

Congenital Hydrocephalus

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Hydrocephalus is Latin for 'fluid on the brain'; it is a birth defect commonly associated with another congenital condition called spina bifida. With hydrocephalus, cerebral-spinal fluid1 is unable to drain effectively and builds up in the cranium.

In infants whose bones have not yet reached mature size or hardened, this causes the skull to swell and enlarge unless immediate treatment is sought, otherwise there is an increasing likelihood of the swelling becoming fatal. The swelling creates pressure on the brain, which becomes compressed and shrunken unless the pressure is released, and can cause brain damage which may not be reversible. In older adolescents and adults who develop hydrocephalus, the skull has by now hardened and the pressure will be directed inwards onto the brain. The fluid can also collect in the ventricles and hollows of the brain, forcing the tissues of the brain outwards against the inside of the cranium. Some symptoms may include intense headaches or migraines, memory problems, blurred vision and loss of consciousness.

Treatments for Hydrocephalus

The treatment for hydrocephalus is usually a shunt. In this context, a shunt is a tube that begins in the spina bifidant's skull which trails down into the chest or stomach cavity, where excess spinal fluid can drain and be harmlessly absorbed back into the body. A button is accessible outside the skull underneath the scalp which is used to activate the shunt valve. This way, infection and excessive drainage are prevented.

In older patients, headaches or dizziness may signal a build up in cerebral-spinal fluid pressure, and they will generally be aware that they need to 'press the button' themselves, which makes the shunt convenient and safe to use. Pressing the button when there is no build up is harmless to the individual - this should not be attempted without the express permission of the individual, and even then, ideally only done by a trained physician. Many individuals with hydrocephalus who have shunts will need to have the shunt removed or replaced due to infection or physical growth at some point in their lives.

Up to 80% of all patients who have childhood hydrocephalus and have a shunt placed in their bodies will develop clinical depression by adulthood, but the cause of this has yet to be determined. It is important that if standard treatment for depression in a patient with a shunt does not improve their mental health, that the patient see their physician who handled their birth defect matters as quickly as possible, as the shunt may need to be removed or replaced. Some children who have hydrocephalus will experience a period of 'remission' from the defect. Every individual, however, who has had hydrocephalus in childhood is likely to experience a recurrence after they have reached adulthood. If the previous shunt has not already been replaced or removed altogether, it is likely that it will need to be replaced when the hydrocephalus returns.

Further Information

1This is the fluid which normally lubricates, cushions and protects the brain, as well as aiding the proper functioning of the brain's neurotransmitters.

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