NEUROSURGERY
Hydrocephalus
Robert Corns
Andrew Martin
Abstract
Hydrocephalus is a common condition in both adult and paediatric neuro-
surgery. We outline how an understanding of its cause in an individual
patient helps to guide treatment. The clinical features, investigation
and treatment of hydrocephalus are described.
Keywords Endoscopic third ventriculostomy (ETV); hydrocephalus;
ventriculo-peritoneal shunt (VP shunt)
Definitions
Hydrocephalus is an abnormal accumulation of cerebrospinal
fluid (CSF) within the ventricles of the brain.
Normal anatomy and physiology
The majority of the CSF is produced actively by the choroid
plexus within the ventricles, but the flow of interstitial fluid from
the brain parenchyma also contributes to the total CSF volume.
Production by the choroid plexus is independent of intracranial
pressure (ICP) e that is, when there is raised ICP, CSF produc-
tion does not fall provided the ICP is not so high as to impair
arterial perfusion of the plexus. The total CSF volume is
approximately 40e50 ml in neonates, 65e140 ml in children and
140e170 ml in adults. The normal rate of production is 0.35 ml/
minute or approximately 500 ml/day in both adults and children.
Figure 1 depicts the normal flow of CSF. In summary, CSF
originates in the lateral ventricles and flows through the foramen
of Monro into the third ventricle. It then drains via the cerebral
aqueduct (of Sylvius) into the fourth ventricle, and out into the
subarachnoid space (SAS) of the basal cisterns via the foraminae
of Magendie (medial, single) and Lushka (bilateral).
CSF is absorbed principally at the arachnoid granulations,
which are out-pouchings of the subarachnoid space into the
venous sinuses (Figure 2). There is also a small amount of
absorption around the nerve roots exiting the spine.
Causes of hydrocephalus
Hydrocephalus is most usefully classified according to the loca-
tion of the obstruction to CSF flow. Hydrocephalus is almost
Robert Corns MRCS is a Specialist Registrar in Neurosurgery at the
Atkinson Morley Wing, St George’s Hospital, London, UK. Conflicts of
interest: none declared.
Andrew Martin FRCS(SN) is a Consultant Neurosurgeon at the Atkinson
Morley Wing, St George’s Hospital, London, UK. Conflicts of interest:
none declared.
SURGERY 30:3
never caused by an overproduction of CSF, although this may
contribute to the hydrocephalus associated with choroid plexus
papilloma, a rare benign tumour of the plexus itself. Following
the flow of CSF in Figure 1, the obstruction can occur at any point
along this path. Common locations and examples of common
pathology found are given in Table 1.
A so-called ‘communicating hydrocephalus’ is one where the
CSF of the ventricles communicates freely with the SAS e that is,
the obstruction is beyond the fourth ventricle and the foraminae
of Lushka and Magendie (Figure 2). By contrast, an ‘obstructive
hydrocephalus’ is one where there is discreet lesion obstructing
the flow of CSF before it enters the SAS. This distinction is useful
as communicating hydrocephalus may be treated by way of
a lumbar puncture (LP) or lumbo-peritoneal shunt (see below)
since the ventricular and subarachnoid pressures should be
approximately equal. An LP in obstructive hydrocephalus is
dangerous and should be avoided because lowering the CSF
pressure in the spine may produce a pressure gradient across the
foramen magnum leading to downward herniation of the cere-
bellar tonsils, brainstem compression and death.
Epidemiology
The most likely cause of hydrocephalus is dependent on
a patient’s age.
Premature infants are susceptible to parenchymal and intra-
ventricular haemorrhage from the so-called germinal matrix,
a highly vascular and metabolically active area deep in each
cerebral hemisphere from which neurons migrate into the
developing cortex. These bleeds are usually managed conserva-
tively with less than 25% requiring surgical intervention for CSF
drainage. Infants born at term are likely to have hydrocephalus
arising from a congenital malformation, such as aqueduct
stenosis, the DandyeWalker malformation (a cystic expansion of
the fourth ventricle with absent cerebellar vermis) or from the
hindbrain abnormalities (the Chiari 2 malformation) associated
with spinal myelomeningocoele. In addition, a significant
proportion of hydrocephalus in young children is idiopathic.
Older children are more likely to develop hydrocephalus from
tumours and these remain a possible cause of hydrocephalus
throughout life. Middle aged adults are in the highest risk group
for subarachnoid haemorrhage (SAH) which often results in
transient hydrocephalus. This usually only needs treatment in
the short term by way of external CSF drainage (see below), but
approximately 15% of cases later require definitive treatment
with a ventriculo-peritoneal (VP) shunt.
The elderly may develop so-called normal pressure hydro-
cephalus (NPH). NPH is a chronic form of hydrocephalus, which
can present insidiously with the triad of gait disturbance, urinary
incontinence and dementia. Clearly these are not uncommon
symptoms and there is some overlap with the clinical and patho-
logical features of Alzheimer’s disease and cerebrovascular
disease. However, NPH sufferers may show symptomatic
improvement following the insertion of a VP shunt.
In a review1 of 1000 adults with hydrocephalus, 34% of cases
were idiopathic. Where a cause was found, 51% were due to SAH,
16% were post-traumatic, 9% were due to tumours, 7% followed
neurosurgery, 5% were due to aqueductal stenosis and 5% followed
meningitis.
142 Ó 2012 Elsevier Ltd. All rights reserved.
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Normal production and flow of cerebrospinal fluid (CSF)

Arachnoid villus
Choroid plexus of lateral ventricle
Superior sagittal sinus
Choroid plexus of third ventricle
Transverse cerebral fissure
Lateral ventricle

Anterior choroidal artery

Tentorium cerebelli

Fourth ventricle

Median aperture Internal carotid

artery
Pontine cistern

Cisterna magna Lateral aperture

Figure 1

Clinical features  Before sutures close (<2 years): macrocephaly, head

circumference increasing and crossing normal centile
The presentation of hydrocephalus depends very much on the
lines, conjugate down-deviation (‘sun setting’) of the eyes,
age of the patient and the rate of progression of the hydroceph-
distended scalp veins, developmental delay or regression,
alus, with an important difference between cases arising before
and poor feeding (Figure 3).
and after the closure of the cranial sutures.

Normal cerebrospinal fluid (CSF) absorption from the subarachnoid space into
the venous sinuses
Superior sagittal sinus Arachnoid villus

Dura
mater

Subarachnoid
space Arachnoid

Pia mater
CNS

Falx cerebri

Ventricle

Figure 2

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 NEUROSURGERY

imaging, which may also indicate the underlying cause. In utero,

Causes of hydrocephalus and location of obstruction and in babies with open fontanelles, cranial ultrasound scanning
is a useful screening test. It can measure ventricular size with
Location Cause
precision and is sensitive in identifying parenchymal and intra-
ventricular haemorrhage.
Lateral, 3rd and Intraventricular haemorrhage
CT scanning is an excellent tool for assessing ventricular size
4th ventricles
and morphology from which it is often possible to infer the level
3rd ventricle at foramen Colloid cyst
of obstruction to CSF flow. Furthermore, acute blood (Figure 5),
of Monro
and most tumours and other space occupying lesions can easily
3rd ventricle Tumours
be seen on CT.
Cerebral aqueduct Aqueduct stenosis
MRI scanning will provide the most detailed pictures of
Tumours
anatomy and is very useful in characterizing posterior fossa
4th ventricle DandyeWalker malformation
lesions (Figure 6). Special MRI sequences can also provide
Tumours
dynamic information on CSF flow (Figure 7).
Impaired absorption at the Subarachnoid haemorrhage
When a picture of communicating hydrocephalus has been
arachnoid granulations Trauma
established, an LP can be useful to measure the pressure of the
Meningitis
CSF and examine its constituents. Normal CSF pressure varies
Table 1
considerably depending on a patient’s size and body habitus,
their position, whether they are anxious or in pain, and whether
 After closure of sutures, acute presentation: headache, vom- they are coughing or straining. A CSF pressure greater than 18
iting, drowsiness, papilloedema (Figure 4). In extremis, coma. cm H2O is abnormal for an adult lying flat and comfortably the
 Chronic presentation in adults, similar to NPH with any of lateral position.
the following: cognitive impairment, incontinence and gait
disturbance. Treatment
 ‘Compensated’ hydrocephalus: some patients can adapt to
the over-accumulation of CSF and may be asymptomatic. The treatment of hydrocephalus depends on cause, patient age
They may also ‘decompensate’ at a later stage and present and rapidity of onset of the symptoms. There can be no exag-
with features of either acute or chronic hydrocephalus. geration of the urgency with which acute obstructive hydro-
cephalus must be treated in a patient with deteriorating
Diagnosis conscious level.

Once a clinical picture of hydrocephalus has been observed, it is Emergency

necessary to confirm the diagnosis with some form of brain
In an emergency, often the quickest and easiest treatment is an
external ventricular drain (EVD). This is placed usually through
a frontal burr hole, with a silicone rubber catheter passed
through the frontal lobe into the lateral ventricle and connected
to an external collection system (Figure 8). This system is
designed to drain CSF when the intracranial pressure (ICP)
exceeds a specified preset pressure.

Figure 3 Historical picture of an infant with hydrocephalus and classic
signs: a large head with ‘sun setting’ of the eyes.
Shunts
The commonest form of treatment is a ventriculo-peritoneal (VP)
shunt. Much the same as an EVD, a catheter is passed into the
lateral ventricle, usually through the parietal or occipital lobes,
although a frontal catheter is also a common alternative
(Figure 9). Rather than draining externally, the catheter is con-
nected to a valve and a second catheter tunnelled to reach the
peritoneal cavity where the CSF is reabsorbed. There are many
varieties of VP shunt valve. Most are manufactured to open and
drain at a pre-fixed pressure or flow rate, but some can be adjusted
through intact skin by means of a magnet to open at different
pressure settings, and this can be an advantage in certain complex
patients. Of note is that these ‘programmable’ valves may be reset
by the intense magnetic field in an MRI scanner. Yet other valves
incorporate an ‘anti-siphon device’ to limit over-drainage when
the patient is upright but there is no good evidence in the literature
to favour the use of one sort of valve over any others. A recent and
far more important development which has reduced the infection
rate is to impregnate the shunt tubing with antibiotics.

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Figure 4 Normal optic disc (left) and papilloedema with blurring of the disc margins (right).

Figure 5 A CT scan showing widespread subarachnoid haemorrhage (seen as hyper-density with the subarachnoid space (SAS), arrowed) and commu-
nicating hydrocephalus. Note the enlarged lateral ventricular temporal horns and blood within the occipital horns.

Figure 6 MRI of a large posterior fossa tumour, which caused obstructive hydrocephalus.

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 NEUROSURGERY

Figure 7 Sagittal T2-weighted ‘DRIVE’ MRI sequences showing stenosis of the cerebral aqueduct (left e arrow) and post-endoscopic third ventriculostomy
(ETV) (right) where the arrow shows a pulsatile jet of CSF passing through the floor of the third ventricle.

The ventricles can be drained to other cavities when the
peritoneal cavity is no longer favourable. Common alternatives
are the right atrium and pleural space, although most cavities
have been tried including gallbladder and ureters! One can also
drain CSF form the lumbar spinal canal in cases of communi-
cating hydrocephalus. A narrow catheter is inserted in a similar
fashion to an LP and can then be tunnelled to the peritoneal
cavity.
The complications of VP shunts are well-recognized. In
addition to the usual risks of any operation e general
anaesthesia, pulmonary embolism and deep vein thrombosis,
bleeding, chest infection e shunts may block and need to be
replaced or ‘revised’, over-drain CSF to produce ‘low-pressure’
headaches when the patient is upright, and the insertion of the
ventricular catheter may cause intracerebral or intraventricular
haemorrhage. There is also a small risk of epilepsy from cortical
injury and, in the UK, the insertion of an EVD or VP shunt carries
an automatic 6-month driving ban for private car owners. Heavy
goods vehicle (HGV) and public service vehicle (PSV) drivers are
usually banned for life. In addition, there is a tiny risk of bowel

Typical arrangement of an external ventricular drain

Collection system
cmsH20
mmHg

Black arrow on
chamber indicates
Ventricles of the brain pressure level as
set by surgeon
Clamp

Zero-line is
placed at the Slide chamber
level of the ear

Clamp
Drain

Clamp

Collection
bag

Figure 8

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 NEUROSURGERY

(ventricular catheter, valve or peritoneal catheter). In young

Common configuration for a ventriculo-peritoneal children, the failure rate has been noted to be 38% after 1 year
shunt and 59% after 4 years2 but the long-term survival of shunts is
better in older children and adults.
Lateral ventricle

Endoscopic third ventriculostomy

Burr hole A more elegant way of treating hydrocephalus in appropriate
patients is to use an endoscope to make a new drainage route out
Proximal of the ventricular system. This procedure is called an endoscopic
conventional third ventriculostomy (ETV) and is best suited to patients with
catheter a discreet lesion, such as aqueduct stenosis or a posterior fossa
Shunt tumour, causing obstructive hydrocephalus. An ETV involves
fashioning a frontal burr hole, passing an endoscope through the
Distal frontal lobe into the lateral ventricle and on through the foramen
conventional of Munro into the third ventricle. The third ventricular floor is
catheter typically thinned in chronic hydrocephalus with functional
hypothalamic nuclei displaced laterally. The anatomy of the floor
of the third is inspected and a hole made in the midline just in
front of the mammilary bodies using a blunt probe e often the tip
and then balloon of a Fogarty catheter e or diathermy. This then
To peritoneal cavity allows the CSF to drain into the basal cisterns and to be reab-
sorbed in the normal way (Figure 10).
ETV has the advantage of reducing infection rates, of avoiding
Figure 9 the long-term risks of a shunt, and leaving the patient with CSF
physiology that is far closer to normal. However, there are risks:
injury at the time of insertion of the distal catheter to the peri- the ventriculostomy can block, usually within the first 6 months,
toneal cavity. The most feared complication is infection, typically and there is the possibility of damaging the basilar artery when
by skin commensals such as Staphylococcus epidermidis, which making the ventriculostomy, which, although rare, may have
usually presents within 6 months of shunt insertion. Infection catastrophic consequences. Although reported, the incidence of
almost always necessitates removal of the entire shunt system neuro-endocrine complications from hypothalamic injury and
and a period of external drainage, often with the use of systemic amnesia from operative damage to the fornix (the structure at the
and intrathecal antibiotics until the CSF clears. Blockages require superior and anterior margins of the foramen of Monro) are very
surgical exploration and replacement of the defective component unusual. The chances of success increase with patient age, with

Catheter passing
Choroid plexus of Fornix through the foramen
the right lateral of Monro
ventricle

Floor of III ventricle

Thalamostriate vein

Figure 10 The approach to an endoscopic third ventriculostomy (ETV) and the endoscopic view of a probe passing through the foramen of Monro and into
the third ventricular floor.

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 NEUROSURGERY
ETVs less frequently performed in children under 6 months of
age due to a higher failure rate in this age group.
What to do if you see a patient with a shunt
Patients with shunts tend to need them for life. When shunts fail
many patients present in a stereotyped way, though this is not
necessarily like their original presentation with hydrocephalus.
Many parents are all too familiar with the way their child
manifests signs of shunt dysfunction and are usually correct in
their judgement. They must never be ignored and most shunted
patients have open access to their local neurosurgical unit.
A concise history is important including when they were first
shunted, what was the indication, when and where the shunt
was last ‘revised’, and whether there is any evidence of inter-
current illness such as urinary tract infection (UTI) or upper
respiratory tract infection (URTI). A CT scan is usually obtained
and compared with old scans at the neurosurgical unit, and if the
patient is well and the CT reassuring then usually admission for
observation is all that is required. If doubt persists, then the
patient can be admitted to their neurosurgical unit for either
shunt ‘tapping’ or ICP monitoring. Shunt tapping involves the
aseptic placement of a needle into the shunt valve reservoir to
measure CSF pressure and drainage, and obtain a sample for cell
count and culture. ICP monitoring is more invasive, and involves
a small burr hole through which a monitor is placed into the
brain which then records the ICP for 1 or 2 days.
SURGERY 30:3
Beware of the unwell patient with very small ventricles on
scan! Patients who have been shunted from early childhood and
are unwell but have small ventricles may well have a blocked
shunt. ‘Slit ventricle syndrome’ occurs as a result of overdrainage
and loss of ventricular compliance (elasticity). Any unwell
patient with a shunt should at least be discussed with a neuro-
surgeon who sees previous and current scans.
What to do if you see a shunt
As a general surgeon, one may come across the peritoneal end of
a shunt when opening the abdomen for other reasons. It is
important to leave it alone by packing it off, preferably with saline-
soaked swabs. It should be protected from any contamination
which may arise from their abdominal surgery. Should it become
contaminated (for example, by faecal matter from a perforated
bowel) then the local neurosurgeons should be contacted and
the distal end can be externalized until sterile conditions are
re-established at which point it can be re-inserted. A
REFERENCES
1 Katzman R. Low pressure hydrocephalus. In: Wells CE, ed. Dementia.
Philadelphia: FA Davis, 1977.
2 Kestle J, Drake J, Milner R, et al. Long-term follow-data from the Shunt
Design Trial. Pediatr Neurosurg 2000; 33: 230e6.
148 Ó 2012 Elsevier Ltd. All rights reserved.