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JSM Pediatric Neurology
Review Article *Corresponding author
Iraj Lotfinia, Department of Neurosurgery,

A Review in Pediatric Tabriz University of Medical Science Tabriz, Fax:

009841133340830, Email:

Hydrocephalus: Physiology,
Submitted: 25 January 2017
Accepted: 20 February 2017
Published: 22 February 2017

Classification, Clinical Copyright

© 2017 Lotfinia

Presentation, Imaging and OPEN ACCESS

Treatment
Iraj Lotfinia*
Department of Neurosurgery, Tabriz University of Medical Science Tabriz, Iran

Abstract
Hydrocephalus, increased cerebrospinal fluid (CSF) within the skull, is a common
disease, and it may develop due to various causes, such as congenital anomalies, tumors,
infections, intracranial hemorrhage, trauma and idiopathic disorders. The disease can
occur in all ages, even prior to birth through late old age, and is associated with various
clinical symptoms. Lack of treatment or delays in treatment can lead to permanent
brain damage. Appropriate therapy can prevent the occurrence of this complication
in most cases, and a ventriculoperitoneal (VP) shunt is often used to treat this disease.
However, this method is accompanied with numerous complications and problems, and,
as a result, the patient may require repeated surgeries. Careful selection of patients
for surgical intervention and surgery using proper techniques can reduce these side
effects.

INTRODUCTION
The term hydrocephalus is derived from two Greek words:
hydro, meaning water, and cephalus, meaning head. The disease
has been known since ancient times because of the strange
appearance of these patients; Hippo-crates were the first
person who described decompression and trepanation in these
patients [1]. Hydrocephalus is defined as an increased volume
of intracranial cerebrospinal fluid CSF. Although this fluid
accumulates in the ventricles in most cases, the CSF sometimes
accumulates outside the ventricles and in the subarachnoid space
around the brain, and such cases is called external hydrocephalus
[1].
Prevalence
Hydrocephalus is the most frequent neurosurgical problem
seen in pediatric medicine also, there is no preponderance
between males and females [1]. Although the exact prevalence of
this lesion is unknown, more than half of the surgeries performed
by pediatric neurosurgeons involve embedding or revision
the shunt [2]. Surgery for hydrocephalus is much less frequent
in adults. According to investigations, the annual incidence of
surgery for hydrocephalus is 3.4 per 100,000 in adults undergoing
surgery [1]. Moreover, some cases of hydrocephalus are believed
to be caused by genetic factors [3].
Physiology
Parts of the brain tissue that constitute the cerebral ventricles
and the space surrounding the brain and spinal cord that include
the subarachnoid space contain CSF. This liquid has several
functions of which the most important are a significant reduction
in the weight of the brain and spinal cord floating in the liquid,
and a reduction in the likelihood of damage followed by sudden
motion and impact on the inner surface of the skull. The other
function of CSF is its nutritional role for the brain and spinal cord
surface areas. In addition, CSF is probably the disposal pathway
for excess neurotransmitters and some metabolic waste products
from the central nervous system (CNS). The majority of CSF is
produced by the choroid plexus in the lateral, third, and fourth
ventricles. The CSF is synthesized naturally by the ultrafiltration
through the choroidal capillary endothelium wall. This secretion
is dependent on the sodium-potassium-ATPase pump in the
choroid plexus apex and is an energy-dependent process. No
change is found at the level of CSF secreted from the choroid
plexus following a sharp rise in intracranial pressure (ICP), but
a decrease in the secretion of CSF will occur in chronic increased
ICP due to atrophy created in the choroid plexus [4]. A smaller
amount of CSF is created by ependymal secretion within the
ventricles and the subarachnoid space. The historical treatment
of plexectomy for hydrocephalus has failed because of this fluid

Cite this article: Lotfinia I (2017) A Review in Pediatric Hydrocephalus: Physiology, Classification, Clinical Presentation, Imaging and Treatment. JSM JSM
Pediatr Neurol 1(1): 1002.

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secretion from areas other than the choroid plexus. According to
recent studies, secretion of CSF and interstitial fluid is the result
of water filtration through the vessel walls. This is associated
with the maintenance of electrolytes and therefore osmotic
pressure in the vessels, which causes reuptake of liquid to the
capillary venous [5]. This theory posits that the formation and
absorption of fluid take place in different areas of the CNS, and
sagittal sinus granulations play little role in fluid uptake due to
low cross-sectional area [5]. Water constitutes more than 99% of
CSF; all the water does not pass through the entire CSF route in
the subarachnoid space, but rather is reabsorbed in the adjacent
vessels. The distribution of other substances in the subarachnoid
space occurs to a greater extent when the reuptake of them to the
microvessels is slower [5].
The liquid enters from the lateral ventricles through the
foramina of Monro into the third ventricle, and then into the
fourth ventricle through the aqueduct of Sylvius. The CSF from
the fourth ventricle flows into the subarachnoid space through
a central foramen of Magendie and two lateral foramina of
Luschka. According to the traditional hypothesis, this flow is
unidirectional [5] and after flowing around the brain and spinal
subarachnoid space, the fluid can be reabsorbed through the
arachnoid villi around the superior sagittal sinus into the systemic
circulation. The CSF formation requires energy consumption, but
its absorption is passive [6]. This absorption is dependent on the
CSF pressure and can be increased significantly with higher intra
cranial pressure. On the other hand, when pressure in the sagittal
sinus increases, the ICP must increase too for CSF to be absorbed
[7]. In patients with subarachnoid hemorrhage and trauma,
fibrosis along the route of CSF flow can induce hydrocephaly
[8]. ICP has been defined as the hydrostatic pressure within CSF
and is dependent on certain factors, such as active CSF secretion,
resistance to exit routes, and passive CSF absorption into the
dural sinuses. Due to the above factors, hydrocephalus may be
caused by three mechanisms: increased production, obstruction
in the route, and elevated venous pressure in the dural sinuses,
which reduce CSF absorption [1]. For normal ICP, there must
be balance and coordination among the production, storage,
and absorption of CSF, and the occurrence of any imbalance in
this area can lead to clinical symptoms. According to the site of
obstruction and disruption in the CSF flow, hydrocephalus can be
divided into two groups: the obstructive (non-communicating)
type where there is blockage in the CSF flow through the
ventricles, and which may occur in any part of the ventricular
system such as the lateral, third, and fourth ventricles; and the
non-obstructive (communicating) type where there is a normal
ventricular CSF flow path and impaired CSF absorption through
the arachnoid villi and, thus, non-normal absorption [9]. The non-
obstructive type is uncommon and develops due to factors such
as recurrent subarachnoid hemorrhage or meningitis, which
disrupt the function of absorption. The CSF overproduction from
the choroid plexus papilloma has been described as a cause of
communicating hydrocephalus, but this concept is controversial
[10]. The increase in CSF volume inside the skull results in
enlargement of the ventricles and increased pressure inside
the skull. Increased intracranial pressure can lead to neuronal
loss and brain damage. The ventricles may not be dilated after
hydrocephalus and CSF blockage in the early stages [11]. The
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enlargement of the ventricles requires time, which depends on
the severity and velocity of hydrocephalus formation. In newborn
infants with open sutures that are not surrounded by rigid skull,
increased ICP in the early stages may be compensated for by
bulging fontanelle and diastasis of sutures, and so ventricular
dilatation may not be observed [11]. In other groups of patients,
a new balance may be established between production and
absorption of CSF following enlargement of the ventricles. In this
case, the ICP is normal, and the infants probably will not benefit
from the insertion of a shunt [11]. Da Silva et al. [12], showed that
cerebral blood flow in acute hydrocephalus was reduced globally
and the decrease in blood flow in chronic hydrocephalus was
dominantly around the ventricles. This hypoperfusion can lead
to brain damage due to the reduction of oxygen and glucose in the
brain [11]. The increased fluid pressure also can cause significant
oxidative changes and may lead to gliosis in all of the brain [13].
Some white matter abnormalities seen in patients with untreated
hydrocephalus, such as ependymal rupture, periventricular
edema, and collapse of periventricular capillaries, will be
reversible with early treatment, but axonal degeneration and
gliosis are signs of permanent damage in the white matter [11]
and remain following the treatment of hydrocephalus.
Classification
Various types of hydrocephalus have been described. As
mentioned earlier, hydrocephalus can be classified into two
main forms based on the site of blockage: obstructive and non-
obstructive. Instead of these two words, non-communicating
and communicating can also be used. Simple classification of
hydrocephalus into the obstructive and non-obstructive types is
not enough if it is possible to identify the actual site of obstruction
[7]. Studies have shown that acute obstruction of the Sylvius
aqueduct is unable to produce a change in intraventricular
pressure. This finding suggests a balance between production
and absorption of CSF within the ventricles [5]; several factors
besides obstruction, therefore, are likely involved in the
development of hydrocephalus. Hence, it is better to modify the
classification of obstructive and non-obstructive hydrocephalus
to reflect patient age, the presence of lesions and rapidity and
chronicity of lesions [7]. Various factors can affect the CSF
system and cause communicating hydrocephalus, including
tumor surgery, brain trauma, infections and subarachnoid and
intraparenchymal hemorrhage within the brain. Nevertheless,
according to the clinical experience of Xu [14], a large percentage
of these patients do not suffer from hydrocephalus because of the
compensatory CSF absorption capacity within a certain range.
Another form of classification used in hydrocephalus is
based on time of onset of the lesion, which is divided into two
types: congenital and acquired. In congenital hydrocephalus, the
lesion is present in the infant prior to birth, such as a narrowing
of the cerebral aqueduct and hydrocephaly associated with
neural tube defects. In some cases, congenital hydrocephalus has
idiopathic etiology. The incidence of congenital hydrocephalus
is about 0.5−0.8 per 1,000 live births [15] and most commonly
involves aqueductal stenosis [16]. It should be considered
that hydrocephalus in all cases of the congenital form may not
exist at birth, but may appear later in life. Other terms used
in the classification of hydrocephalus are normal pressure
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hydrocephalus (NPH) and high-pressure hydrocephalus (HPH).

The term normal pressure is not appropriate, and attacks of
increased ICP can be observed in these patients [17,18]. Another
classification is applied in this case: internal hydrocephalus in
which the ventricles are dilated and the CSF accumulates in the
ventricles (Figure 1), and external hydrocephalus in which the
CSF accumulates in the subarachnoid space surrounding the
ventricles (Figure 2). Hydrocephalus due to clinical symptoms is
divided into two types: active hydrocephalus in which the patient
shows clinical features, and occult hydrocephalus in which
the patient has an increase in intracranial CSF in imaging, but
without clinical features. Arrested hydrocephalus implies a halt
in the growth of the lesions and no increase in ventricular volume
over time. The hydrocephalus based on the appearance rate of
clinical symptoms is divided into three types: acute (within days),
subacute (within weeks), and chronic (within months).

Clinical features
Symptoms: The clinical symptoms will vary depending on
the primary cause of lesions leading to hydrocephalus, age, and
acute or chronic condition of the lesion. Prior to closure of the

Figure 1c Sagittal T2 weighted MRI of a patient with hydrocephaly.

Figure 1a CT scan of a patient with very severe internal hydrocephalus, nearly

almost all of the skull filled with CSF.

Figure 2 Three-month-old patient with birth trauma history and severe

external hydrocephaly in which CSF accumulated outside the brain you can also
see a bridging vein under sever tension.

cranial sutures and the fontanels at an early age, the dominant

manifestations of the disease are a progressive increase in head
circumference and bulging of the fontanels (Figure 3). Different
symptoms may be observed at an early age, including poor
feeding, vomiting, general irritability, drowsiness, seizures,
impaired eye movements, bradycardia, hypertension, and
prominence of scalp veins. After the closure of the cranial
sutures, the dominant clinical symptoms are related to increase
ICP, and the patient presents predominantly with headache,
nausea, vomiting, irritability, drowsiness, blurred vision, and
double vision, loss of bladder control and changes in personality
and memory. Headache, nausea, and vomiting are usually worse
Figure 1b Cronal T1 weighted MRI of another patient with hydrocephaly.
in the morning, and there are two reasons for this. The ICP will

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Figure 3 A hydrocephalic patient before suture closing. You can see the large
head circumference, inappropriate size of head to face, dilated scalp veins, and
sunset sign.
increase further at night because of the lying position, resulting
in some venous stasis. Moreover, carbon dioxide retention occurs
briefly during sleep due to mild respiratory depression, which
causes dilation of cerebral blood vessels and, thus, the increase
in blood volume within the skull, and, consequently, ICP will
increase further. Because the corticospinal fibers of the lower
extremities which pass along the lateral ventricle may be more
prone to stretching and tension, their dysfunction will lead to
weakness in the lower limbs, impaired gait, and frequent falls.
The patient may complain of neck pain that can be caused
by the herniation of the cerebellar tonsils. The patient may
also complain of transient attacks of blurred vision, indicating
impaired blood flow and ischemic optic neuropathy. In these
cases, prompt medical and surgical measures should be taken.
In rare cases, endocrinological symptoms, such as delayed
growth, obesity, and early puberty, may occur due to the
enlargement of the third ventricle and the pressure on the
hypothalamus.
Signs: In the lower ages and prior to closure of the cranial
sutures, the most important sign is excessive abnormal head
circumference. Usually, there is no papilledema in these ages,
but often it is seen after the closure of the cranial sutures. Spastic
weakness may be seen in the lower limbs due to the stretching of
corticospinal fibers. The patient may have the failure of upward
gaze due to pressure on the tectal plate through the suprapineal
recess of the dilated ventricle. This condition can lead to sunset
eye sign in infants (Figure 3). Unilateral or bilateral sixth nerve
palsy also can be seen, which is often the result of high ICP.
Diagnostic techniques
Plain radiography: This technique is less frequently used
to diagnose hydrocephalus. Increased skull size and open
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sutures can be seen in infants. The increase in skull size will
lead to a disproportion between the sizes of the skull and face.
The pathologic calcification, such as that due to brain tumor or
neonatal infection (toxoplasmosis, rubella, cytomegalovirus, and
herpes simplex) may be detectable by this method. Also, signs
of increased ICP, such as destruction of dorsum sellae and silver
beaten appearance in the skull, may be seen in plain radiography.
Ultrasound: This is a noninvasive method that does not have
any proven side effects. Although it is able to detect prenatal
hydrocephalus, the etiology is not usually diagnosed by this
method. It can be used in infants with open fontanelle, and can
determine the size of the ventricles very well. Fluid accumulation
in the brain and the presence of lesions, such as cerebral
hemorrhage and space occupying lesions can be detected by
ultrasound.
Computerized tomographic scan (CT-Scan): CT-Scan
is able to show the size of the ventricles and lesions, such as
bleeding, pathologic calcification, vascular lesions and tumors,
as well as some of the signs of increased ICP, such as edema
surrounding the ventricles, flattened cerebral gyri, and reduced
subarachnoid space.
Magnetic resonance imaging (MRI): MRI can diagnose
hydrocephalus and pathologic factors, without the complications
associated with radiation. In addition, phase-contrast cine MRI
application can be used in patients with suspected hydrocephalus
to assess how CSF flows and to locate possible obstruction sites.
Due to the time required and the need for immobilization of the
patient during the examination, sedation may be needed in young
children and infants.
Transcranial doppler ultrasonography (TCD): TCD
ultrasonography can be used as a noninvasive method at the
bedside, which indirectly measures ICP [11]. In this way, the ICP
status can be assessed with regard to systolic and diastolic blood
flow in the brain and its changes. This method is useful, especially
in young babies, whose signs and symptoms of increased ICP are
often uncertain [11].
Treatment
The treatment process is specified after the necessary studies
to determine the underlying causes of the hydrocephalus. In
cases where other lesions, such as tumors, vascular lesions, or
infection are present, relevant therapeutic measures should
be done. In the following section, we will primarily discuss the
treatment of hydrocephalus.
Non-surgical treatment:
Drug therapy: There are no compelling non-surgical
therapies for hydrocephalus [19], but, in cases like cerebral
hemorrhage leading to hydrocephalus, since hydrocephalus may
be transient, drug treatment can be considered for a short time.
For this purpose, acetazolamide (50-100 mg/kg/day) can be
used alone or in combination with furosemide (1 mg/kg/day).
The effect of these drugs on CSF secretion is rapid and reversible
[20].
The mechanism of action of acetazolamide is the inhibition
of carbonic anhydrase enzyme, which thereby reduces CSF
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secretion. The mechanism of action of furosemide that reduces
ICP is unclear, but its impact is likely due to a decrease in
interstitial fluid in the brain.
Lumbar puncture: Similar to medical treatment, in cases
where the cause of hydrocephalus is temporary, lumbar puncture
can be attempted repeatedly and for a limited time to reduce
ICP. It should be noted that lumbar puncture is allowed to be
performed only in communicating hydrocephalus. This method
can lead to death if used for the obstructive type due to the
occurrence of brain herniation and its complications.
Surgical treatment:
CSF diversion with shunt placement: For this purpose, a
ventriculoperitoneal shunt is commonly used [21]. In cases where
there is no possibility of using this method because of factors
such as abdominal infection or previous surgical adhesions, a
ventriculoatrial or ventriculopleural shunt can be used. With the
ventriculoatrial shunt, the distal portion of the shunt is inserted
through the jugular vein and its tip must be parallel to the junction
of the superior vena cava and right atrial. For this purpose, the
shunt tip should be controlled by intraoperative radiography;
the perfect position of shunt tip placement is between the sixth
and seventh thoracic vertebrae. In the case of communicating
hydrocephalus, and when the ventricular size is small, a
lumboperitoneal shunt can be used. The ventriculoperitoneal
shunt is preferred due to several factors, the most important
of which is related to the unique ability of the peritoneum to
easily localize infection and prevent its spreading. This is also
the easiest method of shunt surgery [21]. Moreover, with this
method, a substantial length of the peritoneal catheter can be
placed inside the abdomen to avoid disruption of shunt function
by child development and shunt peritoneal catheter stretching
upwards, which avoids the need for repeated surgery. In these
cases, use of antibiotic-impregnated catheters will be associated
with a significant reduction in infection rate [22].
These shunts basically consist of three parts: ventricular
catheter, pump, and peritoneal, lumbar, or arterial catheter
(depending on the site of shunt insertion). There are many types
of shunts which have fundamental differences in the pump. The
shunt pump acts as a one-way valve, and, depending on the
pressure set for it, the CSF comes out of the brain when the CSF
pressure is higher than the set pressure, and no fluid will be
discarded from the system at lower pressure. The cerebral shunt
pump is manufactured in two forms. The first type is adjustable,
and its settings can be changed after surgery depending on the
CSF volume required to drain from the brain. With the changes,
intracranial pressure will be reduced or increased. The changes
in the pump take place through the skin, using a special magnetic
field. The second type of pump has a constant pressure and allows
draining CSF from the brain at a constant pressure setting. These
pumps are made in low, medium, and high pressure settings. The
pump valve opens at the pressures of 5, 10, and 15 cm water,
respectively. The type used is selected depending on the medical
condition, the lesion causing hydrocephalus, and the volume
required to drain the CSF. The pressure required for these
pumps to drain CSF from the brain is not changeable. However,
most of the time the medium pressure setting is used. Despite a
few complications reported with programmable valve [23], and
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its large artifacts In MRI [24] it is efficient and safe tool in the
treatment of high pressure and normal pressure hydrocephaly
[25,26].
In this way, the ventricular catheter is inserted into the
lateral ventricle through surgery and is connected to the pump
that is placed under the skull skin. The peritoneal catheter is
also connected to other side of the pump and is inserted in the
peritoneal cavity via subcutaneous tunneling. The ventricular
part is embedded by frontal entry point (10 cm above the nasion
and 2.5 cm on the right) or occipital entry point (6 cm above the
inion and 3 cm off the midline on the right side). The catheter
is conducted toward the lateral ventricle frontal horn. Maximum
catheter entry is 6 cm from the frontal region and 11 cm from
the occipital region. For the peritoneal region, the right upper
quadrant of the abdomen is used. The catheter is placed about 20-
30 cm [27] into the peritoneum. In these cases, use of antibiotic
impregnated catheters will be associated with a significant
reduction in infection rate [22].
One of the shunt complications is over drainage in the
standing or sitting positions as a result of gravity. To deal with
this problem, an anti-siphon device is added to the shunt system.
Endoscopic third ventriculostomy (ETV): Although the
insertion of a ventriculoperitoneal shunt is one of the most
common neurosurgery procedures in our daily practice [28] and
often children with hydrocephalus are treated with it [9], other
treatments should also be considered due to the high prevalence
of the complications of this method. In the last two decades,
there have been shifts in the therapeutic pattern from the
ventriculoperitoneal shunt toward the ETV [29]. In cases where
there is a certain obstruction in the CSF pathway in the third or
fourth ventricular system, the ETV may be a useful approach [30].
The use of this method is effective in both children and adults
[29]. Also, ETV seems to be the first-line therapy in the narrowing
of the cerebral aqueduct due to its low rate of complications
[21,31].
ETV is a routine and safe method for treating obstructive
hydrocephalus [32]. The use of this technique has been on
the rise in the past two decades [33], and today it is the first
choice for treatment of obstructive hydrocephalus [32]. In this
approach, age is a determining factor for prognosis [34]. The
success rate with this method is higher in children over one-year-
old and patients who have no history of surgery [35]. Because
there is no need for an implant in ETV, the incidence of long-term
complications is reduced [36].
ETV can be used in the patients with non-communicating
hydrocephalus. This method is therefore appropriate for
treatment of hydrocephalus secondary to obstruction due to
tumor, aqueductal stenosis, myelomeningocele, postinfectious
hydrocephalus and intraventricular hemorrhage [35]. In this way,
an endoscope is inserted through a small burr hole in the skull
into the lateral ventricle and then the third ventricle through the
foramina of Monro and creates foramina in the floor of the third
ventricle. As a result, the CSF will be able to exit from the third
ventricle into the subarachnoid space and can continue its normal
flow. In the patients who are appropriately selected and where
the CSF absorption in the subarachnoid space is appropriate, the
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acceptable result will be obtained. However, the basilar artery
status in these patients should be evaluated prior to surgery. In
cases where an artery is lower than the ventricular floor, the risk
of intraoperative damage increases, and this method should not
be used in the surgery [37]. ETV is a preferred method for the
treatment of the narrowing of an aqueduct in most neurosurgical
centers [31]. According to Warf [38], the result will be better
using bilateral ventricle choroid plexus cauterization during this
surgery.
It should be considered that only the presence of
ventriculomegaly in ultrasound, CT-Scan, or MRI cannot be
considered as an indication for surgery [11]. In cases with normal
or slightly elevated ICP, even if the size of the ventricles is large,
the CSF drainage will be contraindicated [11]. In this case, on the
one hand, the surgery is not associated with a beneficial result,
and on the other hand, the shunt complications will be added.
Complications
Following the insertion of a ventriculoperitoneal shunt,
several complications have been reported, including shunt
malfunction, infection, subdural hematoma, seizures, migration
to different organs or shunt kinking, and disconnection of the
shunt components [21].
Following surgery, a small amount of blood is often seen
within the ventricles, brain parenchyma, and catheter passing
tract, but the occurrence of clinically significant symptoms is
uncommon in these lesions [28]. Also, symptomatic intracerebral
hemorrhages often occur shortly after surgery [39]. Different
risk factors have been described for the occurrence of an
intracerebral hemorrhage following a cerebral shunt insertion,
including disseminated intravascular coagulation (DIC) caused
by the shunt, vascular damage caused by the shunt passing
through brain tissue, bleeding from the tumor or hidden vascular
lesion, coagulation disorders, and brain trauma shortly after
shunt insertion [40].
In the delayed hemorrhages, most probably the mechanism
of bleeding is vascular injury and its wall erosion due to the
pulsation transmitted by the shunt tube [28].
Other possible complications of using a shunt are over
drainage, which may lead to a slit ventricle, and, in tumor cases,
metastasis of tumor cells through the shunt tube may occur.
Over 50% of patients within two years after
ventriculoperitoneal shunt placement experience shunt
failure [17,41]. Most of these cases are caused by obstruction
that occurs most frequently in the proximal part of the shunt
(proximal to the pump) [9]. The shunt is usually inserted into
the lateral ventricle through the burr hole created in the right
parietal bone. The shunt tip is best placed in the lateral ventricle
anterior to the foramen of Monro that lacks the choroid plexus.
This consequently reduces the likelihood of growth and sticking
of the choroid plexus to the holes in the shunt tube and, thus,
prevents its obstruction; however, these adhesions lead to the
occurrence of intraventricular hemorrhage during the removal
and replacement of the shunt. The shunt tip placed anterior to
the foramen of Monro reduces these problems.
Shunt dysfunction can lead to headache, nausea and vomiting,
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drowsiness, abdominal pain, irritability, and seizures. In children
with a history of meningomyelocele repair, swelling and pain
may be seen in the surgical site. In infants, whose sutures and
fontanelle are still not closed, the bulging fontanelle and increased
size of head circumference may be observed. To examine the
shunt function, observation of the emptying and filling of the
shunt pump after finger pressing will be very helpful to determine
dysfunction and also to locate the disorder. In normal mode by
pressing on the pump, the pump can be easily emptied and slowly
filled afterward. If the pump is not emptied by hand pressure or
is emptied by high pressure, this means that the blockage has
occurred in the distal or abdominal parts of the shunt. If the
shunt pump is emptied easily and is filled with delay or is not
filled, this means that the blockage may occur in the proximal or
intracranial parts of the shunt. In such cases, all aspects of the
shunt need to be checked in terms of kinking and disconnection
of the shunt components. Given that the shunt tubes are visible
on plain radiography; plain radiography can be employed for this
purpose. Also, a brain CT scan can confirm shunt dysfunction by
showing changes in ventricular size and their enlargement, as
well as increased ICP symptoms, such as periventricular edema
and the narrowing of subarachnoid cisterns. Further, MRI can
help to diagnose increased ICP symptoms, intracranial bleeding
or infection. The cine MRI application can determine CSF flow
and possible obstruction.
Radioisotope methods can be recruited to evaluate shunt
function. Technetium is used for this purpose. However, the
injection should be done in the proximal tube of the shunt, and the
injection in the pump cannot evaluate the proximal obstruction
of the shunt because of the one-way valve in the pump [9].
The ventriculoatrial shunt and lumboperitoneal shunt are
rarely used. Ventriculoatrial shunt infection can lead to life-
threatening complications, such as septic endocarditis, septic
pulmonary emboli, and immune complex glomerulonephritis [9].
The lumboperitoneal shunt is associated with a high incidence of
herniation of the cerebellar tonsils, brain stem compression, and
syringomyelia, as well as the possibility of leading to scoliosis and
nerve-root deficit [9].
To reduce the complications associated with the use of
shunts, the surgery should only be performed on patients with
hydrocephalus who also have symptoms of increased ICP. Mild or
moderate dilation of ventricles and mild ventricular enlargement
following surgical repair of meningomyelocele can be controlled
without shunt placement [42].
Prognosis
Fundamentally, prognosis depends on the underlying
condition causing hydrocephalus and duration of symptoms and
treatment-related complications.
Increased intracranial pressure can lead to neuronal loss and
brain damage [43], according to Laurence and Coates [44], if the
natural course of hydrocephalus is not treated, the disease will
lead to death within 10 years in 54% of the cases. They also noted
that 62% of children who did survive had cognitive impairments.
In child-ren who were treated, the results are far better. Shurtleff
et al. [45], showed that the 10-year survival rate of these children
was 90% and only 30% of them had cognitive impairments.
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Cite this article

Lotfinia I (2017) A Review in Pediatric Hydrocephalus: Physiology, Classification, Clinical Presentation, Imaging and Treatment. JSM JSM Pediatr Neurol 1(1):
1002.

JSM Pediatr Neurol 1(1): 1002 (2017)

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