Guidelines for General Practitioners

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Many people find it difficult or painful to absorb medical information at the emotionally traumatic time of their, or their child‘s, diagnosis of hydrocephalus and looking to the future can seem frightening when a shunt is inserted. For these reasons, many patients choose to discuss their situation with their more familiar general practitioner once they or their child are discharged from hospital.

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Guidelines for General Practitioners

  1. 1. shinecharity.org.uk info@shinecharity.org.uk 42 Park Road Peterborough PE1 2UQ 01733 555988HydrocephalusGuidelines for General PractitionersName of Patient: these two factors that has led to our Medical Advisory Committee producing this leaflet. Only General Practitioners who are responsibleDate of Birth: / / for the care of someone with hydrocephalus will receive thisAddress: information, and it is sent only with the permission of the patient, or his or her parents. Problems encountered in a general practice may includeName of neurosurgeon: subtle symptoms of a shunt malfunction. Other symptoms can be moreNeurosurgeon unit attended: obvious indications of a possible malfunction They may include: Vomiting, headache, dizziness, photophobia and other visualUnit telephone no. disturbances, drowsiness and fits. Or there may be symptoms of a possible chronic shunt malfunction. These include:Shine has been listening to peoplewith hydrocephalus, their parentsand families for over 40 years and Fatigue, general malaise, visuo-we are frequently approached by perceptual problems, behaviouralmedical practitioners for information changes, decline in academicabout the management of this performance, being ‘not right’ fromcondition. It is the combination of the carer’s point of view.
  2. 2. HydrocephalusGuidelines for General PractitionersMany people find it difficult or painful A typical ventricular shunt in situto absorb medical information atthe emotionally traumatic time oftheir, or their child‘s, diagnosis ofhydrocephalus and looking to the catheter future can seem frightening when a brain shunt is inserted. For these reasons, CSF many patients choose to discuss their situation with their more Valve familiar general practitioner once they or their child are dischargedfrom hospital. heart In some case,Research findings show that parents tubing maynever forget how news is broken lead to theand explanations are given, and that heart a successful relationship between a family and general practitioner tubing to thecan develop the confidence to face abdominalthe future and the security to live cavitycomfortably in the present. raised intracranial pressure, withA good understanding of their own compression of the brain tissue. Inor their child’s condition enables a babies the head will enlarge but infamily or individual to live life with adults and older children, when thethe minimum of distress caused by cranial sutures have fused, the headuncertainty. size cannot increase.When people contact Shine they How is Hydrocephalus usually want to know: managed?What is Hydrocephalus? Hydrocephalus cannot be cured, but inserting a shunt system to drainHydrocephalus occurs when the away the excess CSF can control it.production of cerebro-spinal flu The shunt consists of a short catheterid (CSF) flowing around the brain inserted into the lateral ventricles inand spinal cord is greater than the the brain and connected to a one-absorption. If the drainage pathways way valve. The distal catheter leadsare obstructed, the fluid accumulates downwards from the valve eitherin the cerebral ventricles leading to into the atrium (ventriculo-atrial
  3. 3. CerebrumChoroid plexus Lateral ventricles (One eachChoroid plexus side) containing CSF3rd ventricle Superior sagittal sinus (Major vein)Pituitary gland CerebellumBrainstem Spinal cordVA shunt) or, more usually, into Sometimes the headaches arethe abdominal cavity (ventriculo- increased in severity by flexing theperitoneal VP shunt). There are neck or by depressing the flushingnow many sorts of valves with chamber of the valve. Palpatingvarying degrees of sophistication. the valve is generally not useful forSome have programmable devices diagnosis. Visual acuity or visualcontrolling the rate of flow of CSF; fields may be reduced. A new orsome have an anti-syphon device increasing squint (especially onto reduce excessive drainage; looking upwards or laterally) is ansome have anti bacterial catheters early sign of raised intracranialto prevent infection. Shunts are pressure. New or increasedusually intended to stay in place for nystagmus may be evident, butlife, although alterations or revisions papilloedema may take a long timemight become necessary from time to develop. A raised blood pressureto time. Although most shunts do and slow pulse are seen only infunction well, complications do the advanced stages of raisedsometimes arise. intracranial pressure.Acute blockage of the shunt If any of these signs are present or if there is sufficient doubt about theThe patient with an acute diagnosis, the patient should bemalfunction of the shunt usually referred to a neurosurgical unit forpresents with headaches, vomiting further investigation. NB A suddenand dizziness, but these symptoms fever or a minor head injury canare variable. He may also have upset the CSF balance in a patientvisual disturbances and diplopia, with a shunt and lead to signs of anfits or losses of consciousness. acute raised intracranial pressure.
  4. 4. HydrocephalusGuidelines for General Practitioners shunt function causing the oppositeChronic malfunction of the problem, high CSF pressure, toshunt reappear, but unfortunately the slit ventricles do not always increase inChronic malfunction of the shunt size again, producing the situationcan be almost symptom-free. Signs where there is very high CSFof a slow increase in intracranial pressure with headache, vomitingpressure can occur gradually over etc but very small ventricles ona period of weeks, months or years scan. The symptoms of over-and cause insidious changes, drainage can be very similar tofatigue, and general malaise. The those of under-drainage, thoughpatient may experience intellectual there are important differences.deterioration, an increase in Headaches, dizziness and faintingvisuospatial perceptual problems, occur and are often worse afterand disturbances of gait and motor getting up from lying down,function, which can progress to whereas the headaches, caused bydementia and death. If your patient high CSF pressure are often worsecomplains of slowly progressive on waking, before rising in thedeterioration, chronic shunt morning. However, the best wayblockage should be considered. to diagnose the problem, having recognised that one exists, is for a neurosurgeon to monitor the CSFOver-drainage pressure over 24 hours.In the case of over-drainage,the Over-drainage can be a difficultshunt allows CSF to drain from the problem. There is no clearventricles more quickly than it is relationship between the type ofproduced. If this happens suddenly valve (high or low pressure) orthe ventricles in the brain collapse, the brand, and over-drainage.tearing delicate blood vessels on A change of valve to a higherthe outside of the brain and causing pressure cannot be relied uponsubdural haematoma. This can be to cure it, though it appears to dotrivial or it can cause symptoms so in some cases. Studies haveSimilar to those of a stroke. The shown the use of an ‘anti-syphonblood may have to be evacuated device’, a small button inserted intoand in some cases, if this is not the shunt tubing, will often solvedone, it may be a cause of epilepsy the problem, but this does notlater. If the over-drainage is more always work. Some shunts havegradual, the ventricles collapse these built-in, but neurosurgicalgradually to become slit-like (‘slitventricles’). This often interferes with opinion varies as to whether they should be used. To change a valve
  5. 5. pressure, it is necessary to remove the valve and insert another. The Ventriculo-peritoneal shunt‘programmable’ or adjustable infectionshunt, is intended to allowadjustment of the working pressure Infection of the ventriculo-peritonealof the valve without operation. The shunt usually presents withinvalve contains magnets that allow two to three months but almostthe setting to be changed by laying always within 6-8 months after aa second magnetic device on the shunt operation. It can present asscalp. This is undoubtedly useful a blockage of the distal catheterwhere the need for a valve of a with signs of a raised intracranialdifferent pressure arises, but the pressure rather than as an infection.adjustable valve is no less proneto over-drainage than any other There can be intermittent fever,and it cannot be used to treat this vomiting and anorexia, withcondition. abdominal pain and tenderness over the distal catheter, whichPatients with programmable shunts may imitate an acute appendicitis.need to be aware that exposure Tenderness, swelling and erythemato magnetic fields including MRI can extend up the catheter track. scanning may adjust the shuntpressure. Ventriculo-atrial shunt infectionShunt infection Ventriculo-atrial shunt infection,Infection of the shunt is very difficult presents as a very different conditionto diagnose, as the symptoms from that of ventriculo-peritonealcan be insidious and confusing. shunt infection.They vary according to the causalorganism, and the route of insertion The patient can become ill withinof the shunt - whether ventriculo- a few days of the shunt operation,peritoneal or ventriculo-atrial. with signs of infection, but often theThe infection is introduced at symptoms may be minimal and gooperation, and it is caused by a unnoticed for many months or evenvariety of simple organisms, which years. There may be general malaise,are usually present on the skin. fatigue, anorexia, intermittent fevers,Most cases are due to coagulase anaemia and splenomegaly, but nonegative staphylococci. signs of blockage of the shunt.
  6. 6. HydrocephalusGuidelines for General PractitionersIf the infection remains undiagnosed, there may be early evidence Endoscopic Third of immune complex disease, Ventriculostomy (ETV)with arthralgia and transientrashes. If a shunt infection is still This procedure is not suitable forunsuspected, this may lead to loin all patients with hydrocephalus andpain, and, occasionally, to oedema, is not available in all neuro-surgicalhaematuria (with proteinuria), signs centres. If ETV fails, symptoms ofof glomerulonephritis, or ‘shunt raised ICP will be demonstrated.nephritis’. (see shunt malfunction). For further information about this procedure, please contact Shine.Diagnosis of shunt infections and prevention of complications Your local district general hospital may not be familiarDiagnosis of infection in VP with the management ofshunts rest upon the timing of hydrocephalus, and a referralpresentation after operation, and to a neurosurgical unit oron any signs or symptoms as telephone contact with thedescribed above. Generally, if signs neurosurgical team will be theof shunt obstruction appear within most appropriate line of action6-8 months of operation, infection when faced with shunt orshould be suspected, but in any ventriculostomy malfunction.case the patient should be referredto a neurosurgeon for further investigation. Features of VA shuntinfection are often misleading and Hydrocephalus and fitsnot infrequently result in referral toan inappropriate specialist such Patients with Hydrocephalusas rheumatology, haematology or sometimes have fits. The fits are notnephrology. A simple antibody test due to the hydrocephalic processis available for those with suspected itself but are usually associated withVA (but not VP) shunt infections. an underlying cause (meningitis, abnormal development of theFurther advice is available from brain, neonatal haemorrhage etc).Shine about where tests can be As a general rule, fits in patientsdone. with hydrocephalus should be treated in the same way as those
  7. 7. that occur in children who do be unable to assess the width ofnot have hydrocephalus. This will a doorway or the height of a kerb,include referral to a paediatrician or which can take on frighteningneurologist. proportions for them. They may have difficulties with patterns,In a patient already treated for fits, shapes and words causing specifican urgent consultation with their learning deficits. They often havespecialist should be sought if the fits difficulty in sequencing, with anchange in character or frequency. inability to organise themselves to do simple tasks in the rightA rise in intracranial pressure due order, which may make themto blockage of a shunt system may appear feckless. They may havesometimes reduce the threshold good long term memory but poorfor an epileptic attack in a patient working memory. Informationalready known to have epilepsy and needs to be broken down intoas both an epileptic fit and blockage ‘small chunks’ and it may help forof shunt system may produce a it to be written down so that yourdeterioration in conscious level this patient can refer to it later. Thesecan sometimes lead to confusion are characteristic tendencies but, ifbetween the two. they are not understood, they can be exasperating.If in doubt, consult the neurosurgeontreating the hydrocephalus. Someone with hydrocephalus may not have the insight or strategies to cope with these aspects of theirHydrocephalus - the hidden disability and consequently familiesproblems often suffer greatly because this is not always understood. A referralPeople with hydrocephalus often to a neuropsychologist with anappear very verbose. Unfortunately, interest in hydrocephalus can bethis can mask any brain damage and very useful to assess the patient’ssubtle learning difficulties caused difficulties and help find ways toby the hydrocephalus. These are cope.difficult to understand and easy toignore or miss. Everyone with hydrocephalus should be encouraged to live asThey may have spatial awareness their peers do albeit with someproblems which means that they adjustments. So, adults who aremay see things differently, and may in work or people in education
  8. 8. may need help with organisation, have a pathway to follow if theymotivation or just finding their way have problems.around. Both groups of patients needThere are a few sports and annual eye checks (by an optician).activities that they are not advisedto participate in - these will include Children with hydrocephalus should‘extreme sports’ such as bungee receive all the routine vaccinationsjumping, deep sea diving and some offered to their peers. As somecontact sports eg rugby scrums. A children with hydrocephalus haveprotective helmet should be worn feeding problems a check ononly for those sports that require it. weight and diet should be part of their routine care.If a child has a head injury, theparents should be reassured thatthe shunt is unlikely to be damaged:if in doubt, the neurosurgeon shouldbe contacted for advice.Children with hydrocephalus willusually be seen in the neurosurgicalclinic on an annual basis: adultsmay not be seen routinely, but willHelp usShine relies on people’s generosity and support so we can help our clientswho depend on us for help and advice - people with hydrocephalus,spina bifida, their families and carers. To donate to Shine please visitwww.shinecharity.org.uk or call 01733 421329.This information has been produced by Shine’s medical advisers andapproved by Shine’s Medical Advisory Committee of senior medicalprofessionals.Shine - Registered charity no.249338To see our full range of information sheets and to find out how to donateto Shine please visit www.shinecharity.org.uk

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