Miscellaneous
60 
Hydrocephalus in Adults
AHMED TOMA
CLINICAL PEARLS
• Hydrocephalus is defined as disturbances in cerebrospinal fluid
dynamics.
• Acute hydrocephalus and shunt obstruction are neurosurgical
emergencies.
T
he term hydrocephalus is a modern Latin adaptation
from Greek hudrokephalon, from húdōr (“water”) +
kephalē (“head”).1 Hydrocephalus is not a single disease
entity. It is rather a spectrum of conditions where there is a
disturbance in cerebrospinal fluid (CSF) dynamics.2
The practice of hydrocephalus in adults is different from
that of pediatrics. It involves managing patients with newly
developed high-pressure hydrocephalus of various etiologies.
A significant proportion involves patients with normal/low-
pressure hydrocephalic conditions, such as normal pressure
hydrocephalus (NPH), or long-standing overt ventriculomeg-
aly in adults (LOVA). It also includes caring for patients
transiting from pediatrics practice to adult practice with hydro-
cephalus treated during childhood. Although not strictly a
hydrocephalus condition, idiopathic intracranial hypertension
(IIH) is often managed as part of the adult hydrocephalus
practice.
Pathogenesis
Cerebral ventricles are four interconnected cavities of the brain
lined by ependymal cells and filled by the cerebrospinal fluid,
a clear, colorless fluid that also surrounds the brain, spinal cord,
and cauda equina. CSF is not a filtrate of the blood. It is pro-
duced by active secretion mainly at the choroid plexuses of the
cerebral ventricles. It is formed at a rate of about 0.35 mL/
min. The daily volume of CSF produced in adult humans is
about 500 mL. Total CSF space in young adults is about
150 mL—that is, CSF is totally replaced about four times each
day. Only about 25% of CSF volume lies within the ventricles.
The rest resides in the cranial and spinal subarachnoid space.3
CSF circulates from the two lateral ventricles, through
the interventricular foramen of Monro to the third ventricle,
822
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• Cerebrospinal fluid drainage shunts are the main treatment
option of hydrocephalus. However, shunt complications are
relatively common.
• Endoscopic third ventriculostomy is an alternative treatment
option in patients with obstructive hydrocephalus.
then through the cerebral aqueduct of Sylvius to the fourth
ventricle. From the fourth ventricle, CSF passes to the sub-
arachnoid space around the brain and spinal cord through the
foramen of Magendie in the midline and the two foramina
of Luschka laterally (Fig. 60.1). The CSF circulation com-
prises not only a directed flow of CSF but a pulsatile to-and-
fro movement as well. It was thought that the dural venous
sinus’s arachnoid villi and granulations play an important
role in CSF absorption. However, research has disputed that
theory. The currently accepted theory is that CSF is cleared
by bulk flow along sleeves of the subarachnoid space sur-
rounding the olfactory and optic nerves as well as spinal
nerve roots.3,4
CSF functions include protection of the brain acting as a
cushion that lessens the impact of a blow. By keeping the brain
buoyant, the net weight of the brain is reduced from about
1400 gm to about 50 gm, thereby eliminating pressure on the
base of the brain and the important basal cerebral arteries. CSF
also serves as a medium to transport hormones to other areas
of the brain. CSF circulates around blood vessels penetrating
from the subarachnoid space into the Virchow-Robin spaces.
We are starting to understand that the clearance of waste
products from the brain during sleep may depend on CSF
circulation as part of the glymphatic system.5
The relationship between intracranial components’ volume
and intracranial pressure is important in the pathophysiology
of many hydrocephalic syndromes. The Monro-Kellie doctrine
states that the skull is a closed bony box with constant volume.
An increase in volume of one of the cranial constituents, or
the presence of a mass lesion (tumor or hematoma), would
result in raised intracranial pressure (ICP).
v.intracranial (constant ) = v.brain + v.CSF + v.blood + v.mass lesion
 CHAPTER 60  Hydrocephalus in Adults 823

Lateral ventricles

Foramen of Monro
Choroid plexus
Third ventricle
Sylvian
aqueduct

Foramen of Luschka

Foramen of
Magendie

• Figure 60.1  Cerebrospinal fluid flow through the ventricular system.

INTRACRANIAL PRESSURE/VOLUME RELATIONSHIP

80
• Figure 60.3  Sagittal T2 MRI scan of a patient with Dandy-Walker
70 syndrome.
Intracranial pressure (mm Hg)

60

50
Decompensation
40
point
30
20
10
0 the ventricular system or more distally at the subarachnoid
Volume
• Figure 60.2  Intracranial pressure-volume relationship. (Adapted from
Toma AK. Hydrocephalus. Surgery—Oxford International Edition. 2015;
33:384–389.)
A physiologic buffer exists, where some compensation is pos-
sible as cerebrospinal fluid CSF and blood move into the spinal
canal and extracranial vasculature, respectively. Beyond this
point, ICP rises dramatically (Fig. 60.2). Increased ICP can
lead to one of the life-threatening herniation syndromes
(coning).6,7
The widely accepted value of normal ICP is 8 to 12 mm Hg
in flat lateral position. This is derived from the CSF opening
pressure on lumbar puncture done in flat lateral position. An
important contributory factor to ICP is the CSF. Pressure is
expected to be the same throughout the system (intracranial
and spinal dural compartments) in supine position, but gravity
will give rise to hydrostatic pressure gradients in upright or
seated positions. Humans are upright most of the time. Normal
ICP is thought to be negative in upright or seated positions.
It is thought that CSF and ICP change in relation to posture
change could be also influenced by posture-related change in
intraabdominal or dural venous sinus pressures, which are, in
turn, related to intrathoracic and spinal epidural venous plexus
pressure.8,9
Etiology and Classification
There are different classification schemes of the hydrocephalic
conditions. A widely used classification is based on CSF
dynamics, where hydrocephalus is classified as obstructive
(noncommunicating) or nonobstructive (communicating)
depending on the presence of a blockage of CSF circulation in
space. This classification has clinical significance in relation to
the safety of caudal CSF drainage; lumbar puncture is contra-
indicated in obstructive hydrocephalus. Hydrocephalus syn-
dromes can also be classified based on age of onset as neonatal,
infantile, pediatric, or adult hydrocephalus. Adult hydrocepha-
lus is often subclassified as high or normal (low) pressure
hydrocephalus.2,10
The underlying causes of hydrocephalus syndromes can be
broadly classified into congenital, acquired, and idiopathic cat-
egories. Congenital hydrocephalus usually presents in the
neonatal period. Chiari malformation (type 2) is commonly
associated with myelomeningocele. Dandy-Walker complex is
a specific entity with hydrocephalus caused by atresia of the
foramina of Luschka and Magendie, with agenesis of cerebellar
vermis (Fig. 60.3). Aqueduct stenosis can present in adulthood
as triventricular hydrocephalus: enlarged third and lateral ven-
tricles with a small fourth ventricle (Fig. 60.4A–B). The under-
lying cause is either forking, septum, true stenosis, or gliosis
of the aqueduct. An X-linked recessive gene is a rare cause of
aqueduct stenosis.
Acquired causes of hydrocephalus include hemorrhage,
infection, or tumors. In adults, communicating hydrocephalus
is a common complication of subarachnoid hemorrhage. Post-
meningitis hydrocephalus is seen, as is postpyogenic or tuber-
culous meningitis.11

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824 PART 9 Miscellaneous

A B
• Figure 60.4  (A) Sagittal CISS sequence MRI scan of a patient with aqueduct stenosis. (B) Axial T2
sequence MRI scan of the same patient showing ventriculomegaly.

Mass lesions including tumors blocking CSF pathways
result in obstructive hydrocephalus (Fig. 60.5A–C). The first
presentation of posterior fossa tumors (eg, astrocytoma or
metastatic tumors) is often that of raised intracranial pressure
caused by hydrocephalus. A colloid cyst of the third ventricle
results in intermittent hydrocephalus or acute hydrocephalus.
Pineal tumors cause aqueduct stenosis and triventricular
hydrocephalus. Choroid plexus papillomas or carcinomas are
rare entities that produce hydrocephalus by excessive CSF
production.
Normal pressure hydrocephalus is a disease of the elderly
population. The underlying cause is not fully understood. It
is thought that the chronic impairment of the Windkessel
effect caused by cerebrovascular disease causes increased brain
pulsation and compression of periventricular tissue produc-
ing ventriculomegaly, with possible stretching of periven-
tricular white matter tracts and subependymal microvascular
ischemia.10,12–14
Epidemiology
A large proportion of adult hydrocephalus patients represent
those who had shunt insertion during childhood. The inci-
dence of pediatric hydrocephalus is less than 1 in 1000 life
births. In the developed countries, there has been a decline in
hydrocephalus caused by congenital malformation or infec-
tion. On the other hand, the incidence of posthemorrhagic
hydrocephalus in prematurely born infants has increased with
improving survival rates.13
Population-based studies estimate the prevalence of normal
pressure hydrocephalus in the elderly population to be 1.4%
to 2.9%, and the incidence has been estimated to be
5.5/100,000 per year. Several studies have estimated the preva-
lence of NPH in the dementia population to be between 1.6%
and 5.4%.15,16
Diagnosis
The clinical presentation of hydrocephalus varies depending
on the etiology. In adults, high-pressure hydrocephalus pre-
sents with features of raised intracranial pressure: Headache is
classically worse in the morning (this is due to relative hyper-
capnia during sleep and subsequent vasodilation). Vomiting
will often relieve the headache as a result of hyperventila-
tion clearing CO2 and hence improve intracranial pressure.
Diplopia is caused by sixth nerve palsy resulting from dis-
tortion of this relatively long slender cranial nerve. Other
symptoms include lethargy and behavioral disturbances.
Signs include papilledema and sixth cranial nerve palsy. If
untreated, impaired consciousness will follow. A further rise in
intracranial pressure will result in brain herniation (coning).
Impending coning is associated with hypertension, bradycar-
dia, and irregular breathing. The patient will have decerebrate
posture and start to have pupillary changes. Without urgent
intervention, irreversible coning and death will follow within
minutes.12,11
NPH is a disease of the elderly population. It could be
either idiopathic or secondary to brain hemorrhage, tumor,
or previous trauma. The classical presentation of NPH is that
of gait and balance impairment, cognitive impairment, and
incontinence.
Impairment of gait is the most readily recognized feature of
NPH. The pattern of gait seen in NPH patients has been vari-
ably described as apractic, glue footed, magnetic, parkinsonian,

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 CHAPTER 60  Hydrocephalus in Adults 825

A C

B
• Figure 60.5  (A) CT scan with contrast of a patient with acute hydrocephalus caused by thalamic lesion
blocking the third ventricle. (B) Lateral skull x-ray showing the bolt EVD inserted for the same patient. (C)
CT scan of the brain of the same patient following an emergency external ventricular drain insertion. Tip
of the EVD is visible at the right foramen of Monro.

short stepped, and shuffling. Freezing is a common feature.
Weakness of the legs is not usually evident on neurologic
examination.
Increased frequency and urgency without actual urinary
incontinence may be seen in early stages of the disorder. Pro-
gression to frank urinary incontinence usually occurs with
disease progression.
The principal cognitive symptoms seen in idiopathic normal
pressure hydrocephalus are suggestive of a subcortical process,
mainly involving frontal lobe functions, such as attention,
psychomotor speed, verbal fluency, and executive functions.
Recognition memory and orientation are relatively preserved
compared with Alzheimer disease.17
LOVA is a form of hydrocephalus that is thought to develop
during childhood (compensated hydrocephalus) and manifests
symptoms during adulthood (decompensated hydrocephalus).
Brain imaging reveals severe ventriculomegaly (Fig. 60.6A–B).
Although symptoms and signs could be of high-pressure
hydrocephalus, many LOVA patients present with symptoms
of low-pressure hydrocephalus, in the form of dizziness, head-
ache, cognitive impairment, gait disturbance, and urinary
incontinence.18

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826 PART 9 Miscellaneous
A B
• Figure 60.6  (A) Axial T2 and (B) sagittal MRI scan of a patient with LOVA.
Differential Diagnosis
Any condition causing raised intracranial pressure could present
in a similar way to hydrocephalus. The differential diagnosis
includes brain tumors, cerebral abscess, intracranial hemor-
rhage, migraines, and idiopathic intracranial hypertension.
Because NPH is a disease of the elderly population,
where gait difficulties, dementia, and urinary incontinence are
common, the differential diagnosis includes a wide range of
conditions:
• Disorders affecting gait may occur, such as peripheral neu-
ropathy, cervical or lumbar stenosis, arthritis, vestibular
diseases, and Parkinson disease.
• The cognitive impairment observed in NPH has some simi-
larity to other subcortical dementias, including Parkinson
disease, diffuse Lewy body disease, and vascular dementia.
The absence of apraxia, agnosia, and aphasia can help dif-
ferentiate NPH from cortical dementias, like Alzheimer
disease.
• Urinary symptoms might be caused by prostate disease in
men or chronic urinary tract infections in women.19
Investigations
Neuroimaging is essential to diagnose hydrocephalus. A com-
puted tomography (CT) scan of the brain is the main investi-
gation method in emergency situations. It can be done quickly
and gives useful information with regard to ventricular size,
the presence of hemorrhage or calcifications, or intracranial
mass lesions. Contrast administration is useful in the differen-
tial diagnosis of such lesions.
Radiologic features of hydrocephalus include features of
ventriculomegaly, ballooning of the frontal horns and third
ventricle, temporal horns greater than 2 mm in size, periven-
tricular edema, crowded cerebral sulci, and Evans index (the
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ratio of the frontal horns to the greater biparietal diameter)
greater than 0.3.10
The main drawback of CT scan is that of radiation expo-
sure. Hydrocephalus is a chronic condition, and patients are
likely to need multiple images over their lifetime. Accordingly,
MRI scan should be used as an alternative in nonemergency
situations. MRI has greater spatial resolution and has multi-
planar projections, thus could help in delineating an underly-
ing lesion. MRI CSF flow studies can help in assessing flow
through different parts of the CSF circulation (eg, cerebral
aqueduct).13
Several imaging features have been found to be associated
with positive response to shunt insertion in normal pressure
hydrocephalus (Fig. 60.7), particularly the presence of tight
high-convexity and medial surface subarachnoid spaces with
concomitant expanded Sylvian fissures (disproportionately
enlarged subarachnoid-space hydrocephalus [DESH]).20 Other
features include narrow callosal angle (the angle of the roof of
the lateral ventricles measured on the coronal MRI of the
posterior commissure perpendicular to the anteroposterior
commissure plane) and focal impingement and thinning of
corpus callosum.
Continuous Intracranial Pressure Monitoring
Continuous ICP monitoring has the advantage of detecting
transient ICP problems that can be missed by the momentary
ICP pressure estimates during lumbar puncture—for example,
posture-related shunt overdrainage. This is done through the
implantation of an intracranial probe, mostly intraparenchy-
mal, through a bolt fixed using a twist drill hole. The probe
monitor is connected to a computer where raw data are stored
and then analyzed for average minute-by-minute systolic and
diastolic values as well as pulse amplitude. Indirect compliance
is estimated from pulse amplitude. Posture-related changes in

• Figure 60.7  Coronal T2 MRI scan of a normal pressure hydrocephalus
patient with narrow callosal angle and DESH sign.
pressure could be deducted by comparing overnight and
daytime data. Telemetric (implantable) ICP monitors have
become part of routine hydrocephalus practice. They have
particular value in managing IIH patients. In NPH, study of
indirect compliance/pulsatility could help in predicting shunt
responsiveness.21
Cerebrospinal Fluid Infusion Studies
A CSF infusion study is a test of pressure response to active
infusion or withdrawal of CSF. It aims to assess the adequacy
of the patient’s CSF absorptive ability. This test is based on a
mathematical model of CSF pressure–volume, where hydro-
cephalus results from a disturbance of CSF drainage—that is,
elevated resistance to CSF outflow. An abnormal and sustained
rise in CSF pressure in the face of the challenge is indicative
of reduced absorptive capacity. The calculations are based on
Davson’s equation:
ICP = CSF formation × resistance to CSF outflow
+ pressure in sagittal sinus
The main value of a CSF infusion test is the CSF outflow
resistance (Rcsf ). Normal Rcsf is considered to be 4 to
10 mm Hg/(mL/min), and values above > 13 mm Hg/(mL/
min) are generally considered abnormal.
There are three different types of CSF infusion studies:
• Constant flow infusion: CSF is infused at a constant rate
with a consequent rise in ICP until a steady state level
(plateau) is reached, where external input plus the forma-
tion rate will equal the absorption rate. Rcsf is calculated as
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CHAPTER 60  Hydrocephalus in Adults 827
the difference between the value of the plateau pressure
during infusion and the resting pressure divided by the
infusion rate.
• Constant pressure infusion: Several predetermined pressure
levels are reached by infusing artificial CSF. The inflow of
artificial CSF needed to maintain each pressure level is
measured. Flow should be linearly dependent on ICP.
• Bolus infusion: This type involves the fast injection of a
small volume of artificial CSF (usually around 4 mL) and
the study of the ICP response to that injection.22,23
In normal pressure hydrocephalus, shunt insertion based on
the clinical picture and the presence of ventriculomegaly will
result in an improvement in 50% of patients. Therefore various
tests are often used to predict shunt responsiveness in suspected
NPH patients. These are based on either physiologic testing
(such as CSF infusion, studies to determine CSF outflow resis-
tance, and ICP monitoring to assess the indirect intracranial
compliance) or functional testing (such as the CSF tap test and
extended lumbar drainage [ELD]).15
Diagnostic Tap Test
This test is used to communicate low-pressure hydrocephalus,
do a lumbar puncture and drain a relatively large volume of
CSF, and assess the response in terms of improvement in
walking speed or neuropsychological assessment. It has a high
positive predictive value but a low negative predictive value
of shunt responsiveness in normal pressure hydrocephalus
(Fig. 60.8).
Extended Lumbar Drainage Protocol
This test involves inserting a lumbar drain, continuously drain-
ing CSF for 72 hours, and assessing the response in terms of
improvement in walking speed or neuropsychological assess-
ment. It has a high positive and negative predictive value.
Management
Surgery is the main treatment for hydrocephalus. Diuretics like
acetazolamide and furosemide can reduce CSF production.
However, the effect is minimal, and these medications have
side effects.
Temporary Measures
The following procedures can be used as temporary measures
to prevent/delay the need for shunt surgery by maintaining
intracranial pressure within normal limits. They are usually
used in emergency situations, particularly in cases of acute
hydrocephalus post-subarachnoid hemorrhage. About 50% of
these patients will not require permanent CSF diversion with
shunt. Shunt insertion in the context of a heavy CSF blood
load could result in early shunt blockage. These measures are
also used in cases of acute hydrocephalus with suspected infec-
tion to allow for CSF sampling and facilitate intrathecal anti-
biotics administration (in patients with an external ventricular
drain).
828 PART 9 Miscellaneous

Degree of certainty for

improvement
sensitivity Clinical exam CT/MRI

Ventriculomegaly
Triad element present
Evans index >0.3
No Yes Yes No

50% Follow Follow

Evaluate surgical
candidacy

LP CSF bolus
withdrawal
Improved

ICP >18 ICP 5 – 18

70%
Probable secondary CSF dynamics test
hydrocephalus
Ro ↑ Ro ↓

>80% Drainage
protocol

+ –

Shunt Follow

• Figure 60.8  Schema for predicting shunt responsiveness. The initial diagnosis of NPH is based solely
on clinical examination coupled with CT/MRI scans. With evaluation of surgical candidacy, proceeding
with shunt without further testing will result in a sensitivity of 46% to 61% (scale at left). A positive response
to a CSF tap test of 40 to 50 mL is highly predictive of a favorable shunt response (72%–100%); however,
the sensitivity is low (26%–61%). Proceeding with an infusion test directly or after the tap test will provide
a high sensitivity, ranging from 57% to 100%, and is associated with a positive predictive value (PPV) of
75% to 92%. The highest sensitivity (50%–100%) and highest PPV (80%–100%) are associated with
external drainage, which requires hospital admission, and improvement of symptoms is seen shortly after
drainage is stopped. LP, lumbar puncture. (From Marmarou A, Bergsneider M, Klinge P, et al. The value
of supplemental prognostic tests for the preoperative assessment of idiopathic normal pressure hydro-
cephalus. Neurosurgery. 2005;57(3 Suppl):S17–28; discussion ii–v.)

• Serial lumbar punctures or lumbar drain insertion: Could
be used in communicating hydrocephalus only.
• External ventricular drain (EVD): A silicone tube is passed
surgically through a frontal burr hole or twist-drill hole to
the frontal horn of the lateral ventricles, then it is tunneled
subcutaneously and connected to a drainage bag in a closed
system. More recently, bolt EVDs have been used, where
the silicone tube is introduced through a hollow bolt fixed
to a skull twist-drill hole.
Definitive Measures
• Removal of obstructive lesion: An example is the surgical
removal of a posterior fossa tumor compressing the fourth
ventricle causing obstructive hydrocephalus. Surgical exci-
sion of the tumor could relieve the hydrocephalus and avoid
the need for an alternative drainage through a shunt surgery.
A temporizing measure, like EVD insertion, is usually
needed.
• Endoscopic third ventriculostomy (ETV): This operation is
used in obstructive hydrocephalus at or distal to the level
of the aqueduct of Sylvius. An endoscope is advanced
through a frontal burr hole into the lateral ventricle and
then through the foramen of Monro into the third ventricle.
Using a balloon catheter, an opening is created in the floor
of the third ventricle, establishing a communication with
the subarachnoid space at the basal cisterns. The success rate
of ETV in adult patients with obstructive hydrocephalus is
reported to be around 75%.
• CSF shunts: These are the most commonly used surgical
option for the treatment of hydrocephalus. They serve to
divert the CSF from the ventricles or lumbar thecal sac to
an alternative body cavity where CSF will be absorbed.

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Shunts are made of inert silicone tubes that are tunneled
subcutaneously between the proximal and distal ends. They
have three main components: a proximal catheter, a valve to
control the amount of CSF drained, and a distal catheter.
There is some evidence that antibiotic and silver-impregnated
catheters use results to reduce acute shunt infection. There are
different types of shunt surgeries, depending on the location
of the proximal and distal shunt catheters:
• Ventriculoperitoneal shunt: This is the most common type.
A proximal catheter is placed through a burr hole into
cerebral ventricles, and a distal catheter is tunneled subcu-
taneously to a minilaparotomy to the peritoneal cavity.
These are subdivided depending on the entry site of the
proximal catheter into frontal, parietal, or occipital.
• Ventriculoatrial shunt: The distal catheter is placed at the
right atrium through the internal jugular vein.
• Ventriculopleural shunt: The distal catheter is placed within
the pleural cavity.
• Lumboperitoneal shunts: These can only be used in com-
municating hydrocephalus or in idiopathic intracranial
hypertension in the absence of a Chiari malformation. A
proximal catheter is passed into the lumbar theca and a
distal catheter in the peritoneal cavity.
• Examples of less common types of shunts include the syrin-
gopleural shunt (draining spinal cord syrinx into pleural
cavity) and the lumbopleural shunt (lumbar theca to pleural
cavity).
Types of Shunt Valves
There are two major types:
• Flow-regulated valves are built into the mechanism to sta-
bilize the flow rather than pressure.
• Differential pressure regulated valves include the majority
of CSF shunts valves used. The valve opens when there is
differential pressure between the proximal and distal ends.
Valve design mechanisms include ball and cone, slit mem-
brane, or spring varieties. The differential pressure regulated
valves are of two subtypes:
• Simple fixed-pressure valves: These have fixed opening
pressure: low, medium, or high (depending on the
opening pressure).
• Adjustable (programmable) valves: The opening pressure
of the valve can be adjusted noninvasively by manipulat-
ing a built-in magnetic component to achieve the best
functional outcome tailored to the patient’s needs. Earlier
generation adjustable valves were sensitive to strong
magnets, where valve opening pressure could change
inadvertently (eg, when the patient undergoes an MRI
scan). Newer generation adjustable valves have a built-in
safety mechanism to prevent an inadvertent change in
opening pressure. These valves are called MRI condi-
tional. There is no evidence that adjustable valves are
superior to simple fixed pressure valves in the manage-
ment of hydrocephalus. However, their use could add
flexibility in the management of patients’ over- or under-
drainage symptoms noninvasively. They have particular
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CHAPTER 60  Hydrocephalus in Adults 829
value in the management of low- and normal pressure
hydrocephalus conditions.
• Shunt reservoirs: These are silicone chambers that could be
a built-in part of the shunt valve or could be an added
component. They act as an access point to aspirate CSF
using a small needle (shunt tap).
• Antisiphon and antigravity shunt components: These
devises are being increasingly used in shunt surgery to
prevent posture-related overdrainage (siphoning). They
could be a built-in part of the shunt valve or could be an
added component. They are particularly useful in reducing
overdrainage complications in low- and normal pressure
hydrocephalus conditions. Adjustable antigravity valves are
also available. Special consideration is needed in surgical
implantation to ensure that the antigravity component is
parallel to the body’s gravitational axis so that it will be
activated fully when the patient is in an erect position and
deactivated when he or she is in a supine position.
Postoperative Management and Follow-up
Postoperative routine brain imaging and shunt series x-rays are
advisable to detect and correct misplacement and serve as a
baseline for future comparison. True shunt independence is
rare, and therefore lifelong neurosurgical follow-up with annual
review is advisable.
On discharge, patients should be fully informed about the
signs and symptoms of shunt malfunction and should be
advised to seek urgent medical advice when they suspect that
a malfunction has occurred. It is advisable that shunted patients
are provided with a “shunt identity card” that specifies the type
of shunt, valve type and setting, and whether or not the valve
is MRI conditional.10,12
Normal pressure hydrocephalus requires regular follow-up
to monitor the patient’s walking speed and cognitive function.
Sudden deterioration could result from shunt malfunction. In
these situations, shunt revision could restore the functional
benefit.
Complications
In adults, shunt-related problems are less frequent when com-
pared with the pediatrics age group.
Infection
Shunt infections occur during implantation and usually present
within a few weeks of surgery. Patients could present with
either superficial or deep incisional wound infections in the
form of inflammation, collection, or a discharging wound,
with or without fever, meningism, or signs of raised intra-
cranial pressure. More commonly, the shunt infection is that
of CSF or peritoneal infection where patients present with
fever, malaise, meningism, or shunt malfunction. Other sources
of infection should be ruled out (eg, urinary tract or chest
infection). A CSF sample is essential for the diagnosis; the
sample can be obtained by doing a lumbar puncture in patients
830 PART 9 Miscellaneous
with communicating hydrocephalus. Alternatively, the shunt
reservoir could be tapped for CSF sampling under strict aseptic
technique. CSF should be tested for pleocytosis, Gram stain
as well as culture. Chronic low-grade infection could present
late in the form of a shunt malfunction. Acute shunt infection
is a neurosurgical emergency. Failure of early diagnosis could
have devastating consequences.
Prevention of shunt infection involves strict aseptic sur-
gical techniques and prophylactic antibiotics administration,
including intrathecal antibiotic treatment during ventricular
catheter placement and use of antibiotics or silver-impregnated
shunt catheters. The use-of-care bundle regimen or standard-
ized surgical protocol has been shown to reduce the rate of
infection. Treatment of shunt infection entails surgery plus
antibiotic therapy. In shunt-dependent patients, surgery could
be either in the form of removal of the shunt system with
insertion of an EVD or externalization of the existing shunt
as a temporary measure with a plan to insert a new shunt
once the infection has cleared. CSF and shunt tubes are sent
for microbiologic tests. Antibiotics should be started imme-
diately, Gram-positive organisms, particularly Staphylococcus
epidermidis, are the most common pathogens implicated. Less
common pathogens include Staphylococcus aureus and gram-
negative bacteria. The combination of intrathecal antibiotics
with systemic antibiotics will depend on the clinical situation
and infecting organism.
Shunt Obstruction
Obstruction is the most common cause of shunt malfunction.
Clinically, patients present with features similar to untreated
hydrocephalus with a brain scan showing features of acute
hydrocephalus. In shunt-dependent high-pressure hydroceph-
alus, shunt obstruction is a neurosurgical emergency. Patients
could deteriorate quickly and die of raised intracranial pressure
unless shunt revision surgery is done. In low-pressure hydro-
cephalus, shunt obstruction presents with a recurrence of pre-
senting symptoms. Diagnosis often requires the repetition of
tests used to make the diagnosis, either in the form of CSF
drainage or infusion tests.
Obstruction could occur at different parts of the shunt:
proximal catheter obstruction by choroid plexus, valve mal-
function or blockage, or distal catheter blockage or withdrawal/
migration. Shunt obstruction could be related to misplacement
of proximal or distal catheters.
Disconnection or Breakage
Particularly in older shunts, disconnection or breakage is
usually evident on shunt series x-rays. An intermittently patent
shunt tract could result in intermittent symptoms of shunt
blockage. Treatment is surgical.
Shunt Under- or Overdrainage
In high-pressure hydrocephalus patients, underdrainage pre-
sents with clinical features of high intracranial pressure, like
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headache. Diagnosis involves brain-imaging features of persis-
tently enlarged ventricles. In low-pressure hydrocephalus, on
the other hand, underdrainage presents with persistent or par-
tially improved symptoms. Diagnosis also requires the repeti-
tion of tests used to make the diagnosis, either in the form of
CSF drainage or infusion tests.
Overdrainage could result in low intracranial pressure head-
ache, dizziness, and nausea. Brain imaging features include
collapsed ventricles, subdural hygroma, or hematoma. Treat-
ment is by shunt valve readjustment, if an adjustable valve is
in place. Alternatively, shunt valve revision or the addition of
an antigravity component is needed.
Other Complications
Other less common complications include seizures, intra-
cranial hemorrhage, shunt tip migration, endocarditis and
nephritis (with atrial shunts), and secondary Chiari malforma-
tion (with lumboperitoneal shunts).10,12
Idiopathic Intracranial Hypertension
This is not hydrocephalus as such. However, because treatment
often involves CSF drainage with shunt insertion, it is fre-
quently discussed in conjunction with hydrocephalus.
Intracranial hypertension (IIH) is a neurologic condition
characterized by increased ICP in the absence of any central
nervous system disease, structural abnormality, and normal
CSF composition. Table 60.1 lists the diagnostic criteria. IIH
is a rare but potentially blinding condition. Therefore it is not
entirely benign. Alternative nomenclature includes benign
intracranial hypertension (BIH) or pseudotumor cerebri.24
The prevalence of IIH in the general population is reported
as 1 to 3 per 100,000 patients per year. However, it is higher
among obese patients as well as women of childbearing age.25
IIH is managed with medical measures in the first instance.
This consists of ICP-lowering medications (eg, acetazolamide)
and weight loss. Lumbar punctures are commonly used as a
temporizing measure. Opening pressure is often high. CSF
should be drained up to a volume of 20 mL or to half the
opening pressure. In some patients, surgical management is
TABLE Diagnostic Criteria for Idiopathic Intracranial
60.1  Hypertension
Diagnostic criteria for idiopathic intracranial hypertension
Symptoms of raised intracranial pressure
Signs representing elevated intracranial pressure or
papilledema
Elevated CSF opening pressure
Normal CSF composition
Imaging to exclude hydrocephalus, mass, or structural lesion
No secondary cause of elevated intracranial pressure
identified

necessary due to rapidly deteriorating vision or the failure or
intolerance of medical therapy. Surgical options in these
patients include CSF diversion in the form of shunt insertion
and optic nerve sheath fenestration.26
Venous sinus stenosis is increasingly recognized as the caus-
ative factor of this condition. The presence of stenosis with an
associated pressure gradient on a catheter cerebral venogram is
considered to be a possible etiologic factor. The endovascular
insertion of a stent has been shown to result in symptomatic
improvement in IIH patients. However, there is no good-
quality evidence supporting the efficacy of stent insertion in
this condition.27
Selected Key References
Brean A, Eide PK. Prevalence of probable idiopathic normal pressure
hydrocephalus in a Norwegian population. Acta Neurol Scand.
2008;118(1):48-53.
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Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2020. Elsevier Inc. Todos los derechos reservados.
CHAPTER 60  Hydrocephalus in Adults 831
Brinker T, Stopa E. A new look at cerebrospinal fluid circulation. Fluids
Barriers CNS. 2014;1(11):10.
Eklund A, Smielewski P, Chambers I, et al. Assessment of cerebrospinal
fluid outflow resistance. Med Biol Eng Comput. 2007;45(8):719-735.
Higgins JN, Cousins C, Owler BK, et al. Idiopathic intracranial hyper-
tension: 12 cases treated by venous sinus stenting. J Neurol Neurosurg
Psychiatry. 2003;74(12):1662-1666.
Qvarlander S, Sundstorm N, Malm J, et al. Postural effects on intra-
cranial pressure: modeling and clinical evaluation. J Appl Physiol.
2013;115(10):1474-1480.
Relkin N, Marmarou A, Klinge P, et al. Diagnosing idiopathic normal-
pressure hydrocephalus. Neurosurgery. 2005;57(3 suppl):s4-s16.
Wagshul ME, Eide PK, Madsen JR. The pulsating brain: a review of
experimental and clinical studies of intracranial pulsatility. Fluids
Barriers CNS. 2011;18(8):5.
Please go to ExpertConsult.com to view the complete list of references.

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Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2020. Elsevier Inc. Todos los derechos reservados.
CHAPTER 60  Hydrocephalus in Adults 831.e1
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