What is hydrocephalus?

The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head. As the name implies, it is a condition in which the primary characteristic is excessive accumulation of fluid in the brain. Although hydrocephalus was once known as "water on the brain," the "water" is actually cerebrospinal fluid (CSF) a clear fluid that surrounds the brain and spinal cord. The excessive accumulation of CSF results in an abnormal widening of spaces in the brain called ventricles. This widening creates potentially harmful pressure on the tissues of the brain. The ventricular system is made up of four ventricles connected by narrow passages.. Normally, CSF flows through the ventricles, exits into cisterns (closed spaces that serve as reservoirs) at the base of the brain, bathes the surfaces of the brain and spinal cord, and then reabsorbs into the bloodstream. CSF has three important life-sustaining functions: 1) to keep the brain tissue buoyant, acting as a cushion or "shock absorber"; 2) to act as the vehicle for delivering nutrients to the brain and removing waste; and 3) to flow between the cranium and spine and compensate for changes in intracranial blood volume (the amount of blood within the brain). The balance between production and absorption of CSF is critically important. Because CSF is made continuously, medical conditions that block its normal flow or absorption will result in an over-accumulation of CSF. The resulting pressure of the fluid against brain tissue is what causes hydrocephalus.
What are the different types of hydrocephalus?

Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is present at birth and may be caused by either events or influences that occur during fetal development, or genetic abnormalities. Acquired hydrocephalus develops at the time of birth or at some point afterward. This type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease. Hydrocephalus may also be communicating or non-communicating. Communicating hydrocephalus occurs when the flow of CSF is blocked after it exits the ventricles. This form is called communicating because the CSF can still flow between the ventricles, which remain open. Non-communicating hydrocephalus - also called "obstructive" hydrocephalus - occurs when the flow of CSF is blocked along one or more of the narrow passages connecting the ventricles. One of the most common causes of hydrocephalus is "aqueductal stenosis." In this case, hydrocephalus results from a narrowing of the aqueduct of Sylvius, a small passage between the third and fourth ventricles in the middle of the brain.

There are two other forms of hydrocephalus which do not fit exactly into the categories mentioned above and primarily affect adults: hydrocephalus ex-vacuo and normal pressure hydrocephalus. Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the brain. In these cases, brain tissue may actually shrink. Normal pressure hydrocephalus can happen to people at any age, but it is most common among the elderly. It may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications of surgery. However, many people develop normal pressure hydrocephalus even when none of these factors are present for reasons that are unknown.
Who gets this disorder?

The number of people who develop hydrocephalus or who are currently living with it is difficult to establish since there is no national registry or database of people with the condition. However, experts estimate that hydrocephalus affects approximately 1 in every 500 children.
What causes hydrocephalus?

The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) or developmental disorders (such as those associated with neural tube defects including spina bifida and encephalocele). Other possible causes include complications of premature birth such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage, which block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF into the cisterns.
What are the symptoms?

Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to the condition. For example, an infant's ability to compensate for increased CSF pressure and enlargement of the ventricles differs from an adult's. The infant skull can expand to accommodate the buildup of CSF because the sutures (the fibrous joints that connect the bones of the skull) have not yet closed. In infancy, the most obvious indication of hydrocephalus is often a rapid increase in head circumference or an unusually large head size. Other symptoms may include vomiting, sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures. Older children and adults may experience different symptoms because their skulls cannot expand to accommodate the buildup of CSF. Symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk which is part of the optic nerve), blurred or double vision, sunsetting of the eyes, problems with balance, poor coordination, gait disturbance,

urinary incontinence, slowing or loss of developmental progress, lethargy, drowsiness, irritability, or other changes in personality or cognition including memory loss. Symptoms of normal pressure hydrocephalus include, problems with walking, impaired bladder control leading to urinary frequency and/or incontinence, and progressive mental impairment and dementia. An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel "stuck." Because some of these symptoms may also be experienced in other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, normal pressure hydrocephalus is often incorrectly diagnosed and never properly treated. Doctors may use a variety of tests, including brain scans (CT and/or MRI), a spinal tap or lumbar catheter, intracranial pressure monitoring, and neuropsychological tests, to help them accurately diagnose normal pressure hydrocephalus and rule out any other conditions. The symptoms described in this section account for the most typical ways in which progressive hydrocephalus manifests itself, but it is important to remember that symptoms vary significantly from one person to the next.
How is hydrocephalus diagnosed?

Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring techniques. A physician selects the appropriate diagnostic tool based on an individuals age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord.
What is the current treatment?

Hydrocephalus is most often treated by surgically inserting a shunt system. This system diverts the flow of CSF from the CNS to another area of the body where it can be absorbed as part of the normal circulatory process.

A shunt is a flexible but sturdy plastic tube. A shunt system consists of the shunt, a catheter, and a valve. One end of the catheter is placed within a ventricle inside the brain or in the CSF outside the spinal cord. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites in the body such as a chamber of the heart or areas around the lung where the CSF can drain and be absorbed. A valve located along the catheter maintains one-way flow and regulates the rate of CSF flow. A limited number of individuals can be treated with an alternative procedure called third ventriculostomy. In this procedure, a neuroendoscope a small camera that uses fiber optic technology to visualize small and difficult to reach surgical areas allows a doctor to view the ventricular surface. Once the scope is guided into position, a small tool makes a tiny hole in the floor of the third ventricle, which allows the CSF to bypass the obstruction and flow toward the site of resorption around the surface of the brain.

What are the possible complications of a shunt system?

Shunt systems are not perfect devices. Complications may include mechanical failure, infections, obstructions, and the need to lengthen or replace the catheter. Generally, shunt systems require monitoring and regular medical follow up. When complications occur, the shunt system usually requires some type of revision.

Some complications can lead to other problems such as overdraining or underdraining. Overdraining occurs when the shunt allows CSF to drain from the ventricles more quickly than it is produced. Overdraining can cause the ventricles to collapse, tearing blood vessels and causing headache, hemorrhage (subdural hematoma), or slit-like ventricles (slit ventricle syndrome). Underdraining occurs when CSF is not removed quickly enough and the symptoms of hydrocephalus recur. In addition to the common symptoms of hydrocephalus, infections from a shunt may also produce symptoms such as a low-grade fever, soreness of the neck or shoulder muscles, and redness or tenderness along the shunt tract. When there is reason to suspect that a shunt system is not functioning properly (for example, if the symptoms of hydrocephalus return), medical attention should be sought immediately.
What is the prognosis?

The prognosis for individuals diagnosed with hydrocephalus is difficult to predict, although there is some correlation between the specific cause of the hydrocephalus and the outcome. Prognosis is further complicated by the presence of associated disorders, the timeliness of diagnosis, and the success of treatment. The degree to which relief of CSF pressure following shunt surgery can minimize or reverse damage to the brain is not well understood.

Affected individuals and their families should be aware that hydrocephalus poses risks to both cognitive and physical development. However, many children diagnosed with the disorder benefit from rehabilitation therapies and educational interventions and go on to lead normal lives with few limitations. Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts is critical to a positive outcome. Left untreated, progressive hydrocephalus may be fatal. The symptoms of normal pressure hydrocephalus usually get worse over time if the condition is not treated, although some people may experience temporary improvements. While the success of treatment with shunts varies from person to person, some people recover almost completely after treatment and have a good quality of life. Early diagnosis and treatment improves the chance of a good recovery.
What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research related to hydrocephalus in laboratories and clinics at the NIH and support additional research through grants to major medical institutions across the country. Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders such as hydrocephalus. The NINDS also conducts and supports a wide range of fundamental studies that explore the complex mechanisms of normal and abnormal brain development.

http://www.ninds.nih.gov/disorders/hydrocephalus/detail_hydrocephalus.htm HYDROCEPHALUS AND SHUNTS: WHAT THE NEUROLOGIST SHOULD KNOW Ian K Pople T he second most common reason for being sued for negligence in neurosurgery is a problem related to hydrocephalus management (the rst being spinal surgery!). However, the good news is that the overall standard of care for patients with hydrocephalus appears to have greatly improved over the last 10 years with the advent of better facilities for investigation, new approaches to treatment, and a greater awareness of the need for adequate follow up. In the possible absence of a local neurosurgeon with an interest in hydrocephalus, a neurologist who is faced with the ongoing care of a patient with hydrocephalus should ideally have a clear idea of what exactly constitutes appropriate follow up and which clinical and radiological warning signals of shunt problems to look out for. c DEFINITIONS Hydrocephalus is an excessive accumulation of cerebrospinal uid (CSF) within the head caused by a disturbance of formation, ow or absorption. Hydrocephalus ex vaccuo is a misnomer. It refers to asymptomatic ventricular enlargement caused by generalised loss of cerebral tissue, fromsevere head injury, infarction or cerebral hypoxia. Normal pressure hydrocephalus is also a misnomer. It describes a condition in older adults of low grade hydrocephalus with intermittently raised intracranial pressure (ICP) (usually at night) causing the classic Adams triad of symptomsgait apraxia, incontinence, dementia. BASIC HYDROCEPHALUS PATHOPHYSIOLOGY IN ADULTS The normal CSF production rate in an adult is 0.35 ml/min (20 ml/hour or 500 ml/24 hours). The

capacity of normal lateral and third ventricles is approximately 20 ml, whereas the total CSF volume in an adult is 120150 ml. Hence, in normal circumstances CSF is recycled over three times each day. ICP rises if production of CSF exceeds absorption, but CSF production will fall as ICP rises to high levels, and compensation (stabilisation of hydrocephalus at a new steady state) may occur through transventricular absorption of CSF.Dural absorptionmay also be important through nerve root sleeves and unrepaired meningocoeles. Of vital signicance is the fact that compensated hydrocephalus (for example, in patients with a longstanding non-functional shunt) is not necessarily permanent because of the sometimes precarious nature of the balance between production and absorption of CSF. Clinicians involved in the care of patients with so called stable hydrocephalus should always be alert to the possibility of insidious subclinical progression or late decompensation of this condition, that may occur spontaneously or after a minor head injury. As hydrocephalus develops the temporal and frontal horns dilate rst, often asymmetrically. There is then elevation of the corpus callosum and stretching of the white matter tracts followed by thinning of the convexity grey matter of the brain (g 1). CAUSES OF HYDROCEPHALUS The causes of hydrocephalus are listed in table 1. CLINICAL FEATURES The clinical features of hydrocephalus are notoriously variable, depending on the rapidity of onset of the condition (see box). Themost rapid deteriorations are seen in young adults with colloid cysts of the third ventricle where the acute rise in ICP caused by the ball valve plugging of the third ventricle can lead to sudden death. The least rapid presentations occur in older patients with soft compliant brains where the only clue to the presence of progressive hydrocephalus may be a subtle slowing of gait or mentation. This slow presentation also characteristically occurs after severe head injury or subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 2002;73(Suppl I):i17i22 *i17 Correspondence to:

Mr Ian K Pople, Department of Neurosurgery, Frenchay Hospital, Frenchay Park Road, Bristol BS16 1LE, UK: ikpople@hotmail.com www.jnnp.com
J Neurol Neurosurg Psychiatry 2002;73:i17-i22 doi:10.1136/jnnp.73.suppl_1.i17

HYDROCEPHALUS AND SHUNTS: WHAT THE NEUROLOGIST SHOULD KNOW

1. 1. Ian K Pople Correspondence to: Mr Ian K Pople, Department of Neurosurgery, Frenchay Hospital, Frenchay Park Road, Bristol BS16 1LE, UK: ikpople@hotmail.com The second most common reason for being sued for negligence in neurosurgery is a problem related to hydrocephalus management (the first being spinal surgery!). However, the good news is that the overall standard of care for patients with hydrocephalus appears to have greatly improved over the last 10 years with the advent of better facilities for investigation, new approaches to treatment, and a greater awareness of the need for adequate follow up. In the possible absence of a local neurosurgeon with an interest in hydrocephalus, a neurologist who is faced with the ongoing care of a patient with hydrocephalus should ideally have a clear idea of what exactly constitutes appropriate follow up and which clinical and radiological warning signals of shunt problems to look out for.

DEFINITIONS
Hydrocephalus is an excessive accumulation of cerebrospinal fluid (CSF) within the head caused by a disturbance of formation, flow or absorption. Hydrocephalus ex vaccuo is a misnomer. It refers to asymptomatic ventricular enlargement caused by generalised loss of cerebral tissue, from severe head injury, infarction or cerebral hypoxia. Normal pressure hydrocephalus is also a misnomer. It describes a condition in older adults of low grade hydrocephalus with intermittently raised intracranial pressure (ICP) (usually at night) causing the classic Adams triad of symptomsgait apraxia, incontinence, dementia.

BASIC HYDROCEPHALUS PATHOPHYSIOLOGY IN ADULTS

The normal CSF production rate in an adult is 0.35 ml/min (20 ml/hour or 500 ml/24 hours). The capacity of normal lateral and third ventricles is approximately 20 ml, whereas the total CSF volume in an adult is 120 150 ml. Hence, in normal circumstances CSF is recycled over three times each day. ICP rises if production of CSF exceeds absorption, but CSF production will fall as ICP rises to high levels, and compensation (stabilisation of hydrocephalus at a new steady state) may occur through transventricular absorption of CSF. Dural absorption may also be important through nerve root sleeves and unrepaired meningocoeles. Of vital significance is the fact that compensated hydrocephalus (for example, in patients with a longstanding non-functional shunt) is not necessarily permanent because of the sometimes precarious nature of the balance between production and absorption of CSF. Clinicians involved in the care of patients with so called stable hydrocephalus should always be alert to the possibility of insidious subclinical progression or late decompensation of this condition, that may occur spontaneously or after a minor head injury. As hydrocephalus develops the temporal and frontal horns dilate first, often asymmetrically. There is then elevation of the corpus callosum and stretching of the white matter tracts followed by thinning of the convexity grey matter of the brain (fig 1).

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Figure 1
Lateral ventricles in coronal section showing the gross stretching and thinning of white matter tracts after chronic hydrocephalus.

CAUSES OF HYDROCEPHALUS
The causes of hydrocephalus are listed in table 1.
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Table 1
Causes of hydrocephalus

CLINICAL FEATURES
The clinical features of hydrocephalus are notoriously variable, depending on the rapidity of onset of the condition (see box). The most rapid deteriorations are seen in young adults with colloid cysts of the third ventricle where the acute rise in ICP caused by the ball valve plugging of the third ventricle can lead to sudden death. The least rapid presentations occur in older patients with soft compliant brains where the only clue to the presence of progressive hydrocephalus may be a subtle slowing of gait or mentation. This slow presentation also characteristically occurs after severe head injury or subarachnoid haemorrhage.

INVESTIGATIONS
Computerised tomography
A computerised tomographic (CT) scan should be undertaken to assess the overall size of the ventricles, and to determine if periventricular oedema or lucency is present. A CT scan is also useful to assess the size of the fourth ventricleif large, this suggests a communicating hydrocephalus, whereas a relatively small fourth ventricle implies obstructive hydrocephalus that might be best treated by endoscopic third ventriculostomy rather than a ventriculoperitoneal shunt.

Magnetic resonance imaging

Chiari malformations and cerebellar or periaqueductal tumours, sometimes not visible on CT, can be detected with magnetic resonance imaging (MRI). This imaging technique is also useful for detecting flow voids in the third ventricle and aqueduct. Various radiological features (angle of the frontal horns on coronal view, downward bowing of the floor of the third ventricle, elevation of the corpus callosum) may imply active hydrocephalus, but these must not be heavily relied upon because of their poor sensitivity. Differentiating normal pressure hydrocephalus from cerebral atrophy is still often more of an art than a science.

ICP monitoring/CSF infusion studies

ICP monitoring and CSF infusion studies are now being used more frequently in young patients with mild symptoms and older patients with possible low grade hydrocephalus. ICP monitoring may reveal B waves either at night time alone or throughout the day and night. An ICP above 15 mm Hg at frequent intervals during the night or day while asleep or resting is abnormal, and patients with functioning shunts should normally have an ICP below or near to zero while 45 head up in bed.

Clinical features of hydrocephalus

o o o

Young adults: Symptomsheadache, vomiting, failing vision, drowsiness, muzziness of the head, fatigue Signspapilloedema, enlarged blind spots on visual field analysis or reduced visual acuity, failure of upward gaze, general clumsiness, dyspraxic gait, large head Older adults/elderly: Symptomsslowing of mental capacity, unsteady on feet/frequent falls, incontinence, drowsiness, headaches less frequently Signsgait dyspraxia (slow, hesitant shuffling gait), dementia (reduced mini-mental score), rarely papilloedema
Lumbar CSF infusion tests measure CSF outflow resistance, which in simple terms represents the overall compliance of the intracranial and spinal CSF compartment. During this test saline or artificial CSF is constantly infused via a lumbar puncture needle or catheter, and the subsequent gradient of rise in the ICP with time is recorded. A low outflow resistance corresponds to high cerebral compliance and vice versa. Normal values are 5 10 mm Hg/ml/minute and a value > 18 mm Hg/ml/minute appears to be the approximate cut off point for diagnosing active hydrocephalus in the elderly. Other compliance monitors have recently been developed that are placed as bolts through small twist drill holes in the skull. These tests can be used to guide treatment of patients with newly diagnosed ventricular enlargement; they can also be useful in patients with possible blockage of their shunts or delayed occlusion of their third ventriculostomy site.

Transcranial Doppler
Transcranial Doppler involves the non-invasive measurement of the middle cerebral artery flow velocities and pulsatility index. The latter index appears to correlate fairly well with ventricular distension and cerebrovascular impedance. The quality of information obtained is very much operator dependent, but can be useful for monitoring patients in an outpatient setting.

Tympanic membrane displacement

Measurement of tympanic membrane displacement is an indirect non-invasive technique for assessing ICP. It is based on a direct communication of pressure waves from the intracranial space to the middle ear via the endolymphatic system of the inner ear. There must be a clear ear canal, intact tympanic membrane, and skilled, experienced operators to give dependable results. In order to diagnose a blocked shunt the patient should preferably already have a baseline reading while well with an obviously functioning shunt.

CSF sample

In post-subarachnoid and post-meningitic hydrocephalus, CSF samples are useful for cell counts, protein concentration, and to exclude residual infection. A protein concentration greater than 4 g/l will clog up most ventriculo-peritoneal shunt valves.

Psychometric analysis
Although it is highly unlikely that any patients with possible pressure from hydrocephalus would ever stand a chance of seeing a neuropsychologist during an investigational work up, the right posterior hemisphere has been shown to be most susceptible to functional deterioration from raised ICP. Deterioration in hand eye coordination and visuospatial skills may precede classic symptoms of shunt blockage.

WHICH TREATMENT OF HYDROCEPHALUS IS BEST: ENDOSCOPIC OR SHUNT?

Since the 1960s the standard treatment of hydrocephalus has been the insertion of a valved ventricular shunt either into the peritoneum or right atrium of the heart. Most neurosurgeons have used ventriculo-peritoneal (VP) shunts mainly because of the potentially life threatening nature of some of the complications of ventriculo-atrial (VA) shunts (multiple pulmonary emboli, shunt nephritis). Over the past 10 years or so there has been a huge resurgence of interest in ways of avoiding CSF shunts for hydrocephalus by utilising endoscopic techniques, such as third ventriculostomy. During this period smaller endoscopes have been developed that have better optics and brighter light sources, and these changes have undoubtedly enabled neurosurgeons to perform these techniques with a greater degree of confidence and safety for the patient. The attraction of giving patients the chance of escaping the numerous inherent complications of CSF shunts has led to a rapid expansion of enthusiastic neurosurgeons offering a neuroendoscopic alternative for the treatment of hydrocephalus. Techniques such as endoscopic septostomy (making a hole in the septum pellucidum for a trapped ventricle), cyst fenestration or choroid plexus coagulation have been used with reasonable long term success, and colloid cysts and pineal tumours are often ideally treated by skilled neuroendoscopy.

ENDOSCOPIC THIRD VENTRICULOSTOMY

In the past the main indication for third ventriculostomy has been non-communicating (obstructive) hydrocephalus, demonstrated by CT or ventriculography. There would typically be dilatation of the lateral ventricles, ballooning of the third ventricle, and a relatively small fourth ventricle. The aetiology of hydrocephalus was generally congenital aqueduct stenosis, and a history of meningitis would have been regarded a contraindication. However, these traditional criteria are no longer adhered to by an increasing number of neuroendoscopists, who have greatly extended their indications for third ventriculostomy to include patient groups previously regarded as inappropriate. The technique consists of the creation of a single burrhole in the frontal region followed by ventricular cannulation and insertion of a 3 or 4 mm wide neuroendoscope into the lateral ventricle. The third ventricle is then negotiated via the foramen of Monro and a hole is then made in the floor of the third ventricle between the infundibulum of the pituitary gland and the mammillary bodies (fig 2). This creates a CSF fistula between the third ventricle and the subarachnoid space in front of the brainstem. The hole is made with a small electrode and enlarged with a balloon dilator, such that a very exciting close up view of the basilar artery is unveiled.

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Figure 2
View of foramen of Monro via a neuroendoscope in the lateral ventricle. The cause of hydrocephalus undoubtably influences the chance of long term success. There is clearly a better success rate in patients with aqueduct stenosis, spina bifida, and tectal, pineal, and posterior fossa tumours. The risk of failure appears to increase with a past or recent history of intracranial infection, presumably as a result of obliteration of cerebrospinal fluid pathways. Long term results have been questioned as follow up is often not more than five years in the literature, but most studies quote an overall success rate between 6575%. The overall complication rates reported in published series varies widely from 4 30%, but the overall rate of serious complications is 9.4% and this includes an average 3% infection rate, 2.3% haemorrhage rate, and 1.3% risk of a permanent neurological deficit. Fortunately the rate of life threatening complications appears to be low and postoperative deaths are rare (0.1%, or 1 per 1000 third ventriculostomies).

CEREBROSPINAL FLUID SHUNTS

Most shunting systems drain according to the differential pressure gradient between the ventricle and the tip of the distal catheter. These valves have been shown to be effective in the majority of patients and a typical valve is shown in fig 3. Most neurosurgeons use medium pressure valves, that will drain CSF continuously if the differential pressure is over about 10 mm Hg. The ventricular catheter of a shunt is normally inserted through a burrhole in the right parieto-occipital region and the valve will sit usually behind the right ear. The distal catheter is tunneled subcutaneously down to another incision in the abdomen where it is then placed into the peritoneal cavity. It is not usually helpful for non-neurosurgeons to palpate or flush the shunt valve, as their contours and characteristics are so variable as to make interpretation notoriously inaccurate.

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Figure 3
Structure of a typical CSF shunt valve.

Clinical features of shunt malfunction

Headaches Vomiting Drowsiness Papilloedema with or without failing vision Occasionally failure of upward gaze Neck stiffness Thoracic back pain in patients with spina bifida

Investigations of shunt malfunction

CT scan (enlargement of ventricles) Plain x ray of shunt system (lateral skull, anteroposterior (AP) chest and AP abdomen) Palpation of shunt reservoirunreliable Peripheral blood for C reactive protein, white cell count if there has been any recent surgery Shunt reservoir tap ICP monitoring/lumbar infusion test

In selected patients a ventricular access device (otherwise known as an Ommaya reservoir) is placed in the right frontal region for ICP monitoring or treatment of infections (fig 4). These cannot be flushed or assessed in any way by palpation, but provide the facility for potentially life saving percutaneous aspiration of CSF in the event of acutely raised ICP. This can be done by any clinician in this situation and simply involves the passing of a butterfly needle through the skin perpendicular to the surface of the skin at the apex of the dome of the reservo ir, until a pop is felt. Elective sampling of reservoirs or shunts should preferably be carried out by a neurosurgeon, unless the clinician looking after the patient has had previous experience in the technique.

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Figure 4
Ventricular access reservoir and catheter for percutaneous sampling of CSF. Differential pressure valves allow the siphoning effect in the upright position and this may lead to excessive CSF drainage from the ventricles. Some systems now incorporate an anti-siphon device to ameliorate this effect. Programmable or adjustable valves allow the closing pressure to be altered externally using a special magnetic adjusting device. Although this is sometimes extremely useful in selected shunted patients with intractable headaches, it can lead to problems following inadvertent change of pressurefor example, by having an MRI scan or, less obviously, by using headphones and certain cordless phones. Flow controlled valves, such as the Orbis-Sigma valve, have a more physiological CSF drainage pattern, but they do not appear to be effective in normal pressure hydrocephalus or where brain compliance is poor (so called brittle ventricles).

COMPLICATIONS OF SHUNTING
Shunt obstruction
Shunt obstruction may occur proximally in the ventricular catheter as a result of choroid plexus, red cells, tumour cells, or a high protein concentration in the CSF. Blockage of the distal catheter can occur as a result of body growth (if the shunt was placed during childhood), adhesions within the abdominal cavity, especially when associated with a low grade infection, pregnancy, and occasionally constipation.

Urgent help from the on-call neurosurgeon should be sought for all suspected cases of acute shunt malfunction as patients with little remaining compensatory reserve may deteriorate suddenly as a result of a respiratory arrest, seizures, or simple coning. Shunt blockage may cause death and blindness if there is a combination of sudden onset and delay in treatment. Uncomplicated shunt revisions do not affect long term outcome. However, revision surgery on patients with blocked shunts is occasionally complicated by serious secondary ventricular or intraparenchymal haemorrhage, and any patient who is not quite right soon after a shunt revision should have a follow up CT scan immediately. Mos t young patients with shunts require a revision operation once or twice every 10 years as the shunt tubing degenerates gradually over the years and flakes of silicone break off (causing subcutaneous granulomas), weakening the wall of the tube. Eventually the tube may fracture or obstruct. Some unlucky patients run into multiple problems with their shunt, usually as a result of one problem leading to another (fig 5). There is little to be done in such circumstances, other than commiserate with the patient and approach each hurdle in a positive and objective manner. However, when the situation becomes unduly complicated or intractable it may be prudent for the neurologist or neurosurgeon to seek the opinion of a neurosurgeon with a special interest in hydrocephalus.

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Figure 5
Multiple shunt catheters caused by intractable shunt failurea nightmare scenario for the on-call neurosurgical team.

Infection
Shunt infections are usually caused by the patients own skin organisms (most common is Staphylococcus epidermidis), which gain access to shunt tubing during the shunt procedure. Typically this contamination will cause an internal

shunt colonisation where the bacteria settle and grow on the internal wall of the shunt catheter and valve, establishing adherent colonies. However, some bacteria set up a ventriculitis without full colonisation of the shunt, and others (for example, Staphylococcus aureus) cause an external shunt infection (deep wound infection). The most important clinical features of a shunt infection are as follows:

general malaise pyrexia headaches, vomiting, neck stiffness abdominal tenderness or distension recurrent lower end shunt obstruction occasionally pain and erythema around the shunt pulmonary hypertension or shunt nephritis in chronic VA shunt infections recent shunt operation 90% of VP shunt infections present within three months of a shunt operation raised C reactive protein high peripheral and CSF white cell count culture of organism from CSF.
Patients should be reassessed urgently by the relevant neurosurgical team should any of the above symptoms develop within the first few months after a shunt operation. After six months a VP shunt will not become infected unless intra-abdominal sepsis occurs (for example, appendicitis, diverticulitis or postgastrostomy feeding tube insertion). The current incidence of shunt infection in most neurosurgical units is about 5 8%, but many units are now achieving better results as a result of preventative measures and protocols. Details, including infection rates, of most shunt operations performed in the UK are now submitted to the UK Shunt Registry that was set up by the Medical Devices Agency a few years ago. It is hoped that information derived by analysing this huge data set will lead to further improvements in the standards of shunt surgery in the future. Infected shunts will often have to be removed and then replaced after two weeks of antibiotics and temporary drainage. Infections with low grade pathogens can sometimes be treated with intraventricular and intravenous antibiotics alone.

Over-drainage
The perfect shunt valve has yet to be designed and many current models allow over-drainage of CSF due to the siphoning effect. The hydrostatic pressure (2575 cm CSF) caused by the weight of the column of CSF within the distal catheter leads to fluid being sucked out of the ventricles in the upright position. The valve pressure may be set too low for an individual patient leading to over-drainage; this can be remedied by adjustments of the valve pressure either by revision of the valve or by using a programmable valve. ICP monitoring may be required in those patients presenting with possible low pressure headaches without evidence of subdurals on the CT scan. Subdural haematoma can occur during the first six months after a shunt insertion and has been shown to be related to the amount of CSF released at operation. Small collections occur in up to 30% of patients after shunt insertion in the elderly, but symptomatic collections requiring surgery affect only 1015%. The symptoms of a shunt related subdural collection include headaches, confusion, hemiparesis, and drowsiness.

FOLLOW UP AFTER ENDOSCOPIC THIRD VENTRICULOSTOMY

There is a clear need for continued follow up of patients after apparently initially successful third ventriculostomy, as late failure occurs in up to 40% and sudden death has been recently reported as little as two years following apparently successful third ventriculostomy. Most patients are followed up primarily by monitoring their clinical symptoms, optic fundi, and visual acuity. Midline sagittal T2 weighted MRI sequences combined with cine phase contrast MRI flow measurements provide a reliable tool for ascertaining the patency of the stoma during follow up evaluation. The important sign is flow voids through the ostomy; change in ventricular size is less important after third ventriculostomy. However, a 33% reduction in the size of the third ventricle on follow up CT scans occurs in patients whose symptoms have been successfully controlled. Lateral ventricles will only decrease in size on average by 16%. ICP measurements from reservoirs may be useful in selected younger patients, as the value of ICP monitoring in shunted children by means of a reservoir or by parenchymal devices has been previously well documented. Reservoirs with an integral telesensor to measure ICP non-invasively may prove useful after third ventriculostomy where the preoperative pressures have been substantially raised. Other workers have advocated the use of CSF infusion studies to monitor cerebral compliance, or transcranial Doppler indices before and after operation as an indirect measure of compliance.

HOW SHOULD WE FOLLOW UP SHUNTED PATIENTS?

Arrangements for follow up of adult patients with shunts have been extremely variable, with some patients receiving careful regular annual outpatient assessments and others being completely discharged and left to contact their general practitioners in the event of symptoms. A number of patients have gone blind or died as a result of undetected chronic shunt malfunction, and efforts by the Association of Spina Bifida and Hydrocephalus have led to improvements in standards of follow up care. The principle components of good long term care of patients with shunted hydrocephalus are outlined below. All patients should have a baseline CT brain scan 612 months after their initial shunt insertion, while they are well. They should preferably retain a copy of this baseline scan if they travel far from their base neurosurgical unit, and it is our experience that most patients are willing to pay for this copy. Although it is not always possible to detect shunt blockage on a CT scan as a result of a patient having non-compliant ventricles, this is the exception rather than the rule. All patients and carers should be given clear instructions (preferably written) as to what symptoms to look out for and when to contact their doctor. Some documentation of exactly which type of valve has been implanted should be given to the patient. Those with programmable/adjustable valves should always have their shunt re-programmed, or at least checked by their neurosurgeon after any MRI scan has been carried out. They should be made aware of the possible problems of inadvertent valve pressure change from extraneous magnetic sources. All younger (under 60 years) patients should have an annual visual acuity check by an optician or an ophthalmologist if there have been particular concerns about vision. All younger patients with a shunt should probably be encouraged to seek a neurosurgical check up at least every three years, ideally at a dedicated hydrocephalus follow up clinic.

SUMMARY
Modern trends in surgical treatment of hydrocephalus are moving towards the greater use of minimally invasive endoscopic procedures and away from routine shunting wherever feasible. Patients with isolated hydrocephalus should have a normal life expectancy, as long as prompt detection and treatment of complications is provided through maintaining appropriate arrangements for long term follow up.

KEY REFERENCES
1. Adams RD, Fisher CM, Hakim S, et al. Symptomatic occult hydrocephalus with `normal cerebrospinal fluid pressure N Engl J Med 1965;273:11726. A landmark paper in the clinical assessment of hydrocephalus in the older age group. 2. Hebb AO, Cusimano MD. Idiopathic normal pressure hydrocephalus: a systematic review of diagnosis and outcome. Neurosurgery2001;49:116686. A general overview of diagnostic criteria and treatment of older patients with hydrocephalus.

[Medline][Web of Science] 3. Boon AJ, Tans JT, Delwel EJ, et al. Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid. J Neurosurg1997;87:68793. An evaluation of the use of lumbar infusion tests in the diagnosis of active hydrocephalus in older adults. A widely quoted paper on the assessment of normal pressure hydrocephalus. [Medline][Web of Science] 4. Pople IK, Edwards RE, Aquilina C. Endoscopic management of hydrocephalus treatment.Neurosurg Clin N Am2001;12:71935. An overview of current endoscopic techniques in hydrocephalus treatment. [Medline] 5. Drake JM, Kestle JR, Tuli S. CSF shunts 50 years on past, present and future. Childs Nervous System 2000;16:8004. A useful overview of ventricular shunts for hydrocephalus. [CrossRef][Medline][Web of Science]