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Hydrocephalus
Name of Patient:
Name of neurosurgeon:
Shine has been listening to people with hydrocephalus, their parents and families for over 40 years and we are frequently approached by medical practitioners for information about the management of this condition. It is the combination of
Hydrocephalus
managed?
What is Hydrocephalus? Hydrocephalus occurs when the production of cerebro-spinal flu id (CSF) flowing around the brain and spinal cord is greater than the absorption. If the drainage pathways are obstructed, the fluid accumulates in the cerebral ventricles leading to
Hydrocephalus cannot be cured, but inserting a shunt system to drain away the excess CSF can control it. The shunt consists of a short catheter inserted into the lateral ventricles in the brain and connected to a oneway valve. The distal catheter leads downwards from the valve either into the atrium (ventriculo-atrial
Cerebrum Choroid plexus Choroid plexus 3rd ventricle Pituitary gland Brainstem Lateral ventricles (One each side) containing CSF Superior sagittal sinus (Major vein)
Cerebellum
Spinal cord VA shunt) or, more usually, into the abdominal cavity (ventriculoperitoneal VP shunt). There are now many sorts of valves with varying degrees of sophistication. Some have programmable devices controlling the rate of flow of CSF; some have an anti-syphon device to reduce excessive drainage; some have anti bacterial catheters to prevent infection. Shunts are usually intended to stay in place for life, although alterations or revisions might become necessary from time to time. Although most shunts do function well, complications do sometimes arise. Sometimes the headaches are increased in severity by flexing the neck or by depressing the flushing chamber of the valve. Palpating the valve is generally not useful for diagnosis. Visual acuity or visual fields may be reduced. A new or increasing squint (especially on looking upwards or laterally) is an early sign of raised intracranial pressure. New or increased nystagmus may be evident, but papilloedema may take a long time to develop. A raised blood pressure and slow pulse are seen only in the advanced stages of raised intracranial pressure. If any of these signs are present or if there is sufficient doubt about the diagnosis, the patient should be referred to a neurosurgical unit for further investigation. NB A sudden fever or a minor head injury can upset the CSF balance in a patient with a shunt and lead to signs of an acute raised intracranial pressure.
Hydrocephalus
Over-drainage
In the case of over-drainage,the shunt allows CSF to drain from the ventricles more quickly than it is produced. If this happens suddenly the ventricles in the brain collapse, tearing delicate blood vessels on the outside of the brain and causing subdural haematoma. This can be trivial or it can cause symptoms Similar to those of a stroke. The blood may have to be evacuated and in some cases, if this is not done, it may be a cause of epilepsy later. If the over-drainage is more gradual, the ventricles collapse gradually to become slit-like (slit ventricles). This often interferes with
pressure, it is necessary to remove the valve and insert another. The programmable or adjustable shunt, is intended to allow adjustment of the working pressure of the valve without operation. The valve contains magnets that allow the setting to be changed by laying a second magnetic device on the scalp. This is undoubtedly useful where the need for a valve of a different pressure arises, but the adjustable valve is no less prone to over-drainage than any other and it cannot be used to treat this condition. Patients with programmable shunts need to be aware that exposure to magnetic fields including MRI scanning may adjust the shunt pressure.
Shunt infection
Infection of the shunt is very difficult to diagnose, as the symptoms can be insidious and confusing. They vary according to the causal organism, and the route of insertion of the shunt - whether ventriculoperitoneal or ventriculo-atrial. The infection is introduced at operation, and it is caused by a variety of simple organisms, which are usually present on the skin. Most cases are due to coagulase negative staphylococci.
Hydrocephalus
that occur in children who do not have hydrocephalus. This will include referral to a paediatrician or neurologist. In a patient already treated for fits, an urgent consultation with their specialist should be sought if the fits change in character or frequency. A rise in intracranial pressure due to blockage of a shunt system may sometimes reduce the threshold for an epileptic attack in a patient already known to have epilepsy and as both an epileptic fit and blockage of shunt system may produce a deterioration in conscious level this can sometimes lead to confusion between the two. If in doubt, consult the neurosurgeon treating the hydrocephalus.
be unable to assess the width of a doorway or the height of a kerb, which can take on frightening proportions for them. They may have difficulties with patterns, shapes and words causing specific learning deficits. They often have difficulty in sequencing, with an inability to organise themselves to do simple tasks in the right order, which may make them appear feckless. They may have good long term memory but poor working memory. Information needs to be broken down into small chunks and it may help for it to be written down so that your patient can refer to it later. These are characteristic tendencies but, if they are not understood, they can be exasperating. Someone with hydrocephalus may not have the insight or strategies to cope with these aspects of their disability and consequently families often suffer greatly because this is not always understood. A referral to a neuropsychologist with an interest in hydrocephalus can be very useful to assess the patients difficulties and help find ways to cope. Everyone with hydrocephalus should be encouraged to live as their peers do albeit with some adjustments. So, adults who are in work or people in education
may need help with organisation, motivation or just finding their way around. There are a few sports and activities that they are not advised to participate in - these will include extreme sports such as bungee jumping, deep sea diving and some contact sports eg rugby scrums. A protective helmet should be worn only for those sports that require it. If a child has a head injury, the parents should be reassured that the shunt is unlikely to be damaged: if in doubt, the neurosurgeon should be contacted for advice. Children with hydrocephalus will usually be seen in the neurosurgical clinic on an annual basis: adults may not be seen routinely, but will
have a pathway to follow if they have problems. Both groups of patients need annual eye checks (by an optician). Children with hydrocephalus should receive all the routine vaccinations offered to their peers. As some children with hydrocephalus have feeding problems a check on weight and diet should be part of their routine care.
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Shine relies on peoples generosity and support so we can help our clients who depend on us for help and advice - people with hydrocephalus, spina bifida, their families and carers. To donate to Shine please visit www.shinecharity.org.uk or call 01733 421329. This information has been produced by Shines medical advisers and approved by Shines Medical Advisory Committee of senior medical professionals. Shine - Registered charity no.249338 To see our full range of information sheets and to find out how to donate to Shine please visit www.shinecharity.org.uk