A Review of Endoscopic Treatment of Hydrocephalus in Paediatric and Review

Review
A Review of Endoscopic Treatment of Hydrocephalus in Paediatric and
Adult Patients
Ji Min Ling, MB.ChB (UK), MMed Surgery (NUS), Rajendra Tiruchelvarayan, MBBS, FRCS (Neurosurgery) (Ireland)
Department of Neurosurgery, National Neuroscience Institute, Singapore
Abstract
Endoscopic treatment for hydrocephalus started in the early 20th century, but could not thrive due to poor
illumination and magnification of the scope. In the 1950s, ventriculoperitoneal (VP) shunt became widely
acceptable as standard treatment for hydrocephalus owing to the invention of well-designed valves and discovery
of silicone, a biocompatible material for manufacturing shunt catheters. However, shunting is still far from being
an ideal treatment because of its associated complications such as catheter malposition, blockage, and over- or
under-drainage of cerebrospinal fluid. The shunt revision rates remained high in recent series. At the same time,
endoscopy has undergone tremendous improvement in the latter half of the century and has emerged as an
attractive alternative since the early 1990s. The article described the usage of endoscopy in the treatment of
hydrocephalus, such as endoscopic third ventriculostomy, fenestration of multi-loculated hydrocephalus, and
fenestration of septum pellucidum prior to VP shunting.
Keywords: Endoscopy, Hydrocephalus, Shunt, Third ventriculostomy
HISTORY OF NEUROENDOSCOPY AND
VENTRICULOSCOPY IN THE TREATMENT OF
HYDROCEPHALUS
Endoscopy has been employed by neurosurgeons
since the early 20th century. In 1918, Walter
Dandy was one of the first surgeons who used an
endoscope to perform choroid plexectomy in four
patients with communicating hydrocephalus1.The
results were poor, as three out of four patients died.
The disappointing result led Dandy to develop
a new technique of fenestrating the floor of the
third ventricle via a subfrontal approach2. However,
this technique did not become popular because
of the need to sacrifice an optic nerve to afford
the exposure. In 1923, Fay and Grant were able to
visualise and successfully photograph the interior
of the ventricles of a child with hydrocephalus by
using a cystoscope3. That same year, William Mixter,
a urologist, became the first surgeon to perform
an endoscopic third ventriculostomy (ETV). He
used a urethroscope to examine the ventricles
of a child with obstructive hydrocephalus, and
fenestrated the floor of the third ventricle during
this procedure4. In 1934, Putnam reattempted
cauterisation of the choroid plexus with an
endoscopic device for seven patients. The procedure
was successful in at least three cases, and resulted
in two deaths5. Nine years later Putnam reported
his series of endoscopic choroid plexectomy in
42 patients. There were 10 perioperative deaths
(25%) and 15 patients failed to respond, although
17 had successful relief of increased intracranial
pressure6,7. Despite the various efforts that showed
the potential utility of endoscope in the treatment
of hydrocephalus, endoscopy did not gain favour in
general neurosurgical practice because of the poor
magnification and illumination, which made this
technique unreliable and frustrating to perform.
The beginning of the1950s marked the decline
of endoscopic treatment of hydrocephalus when
Nulsen and Spitz published their landmark report of
cerebrospinal fluid (CSF) shunting with a ball-andcone valve as a viable treatment for hydrocephalus8.
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Subsequent developments of improved valve
designs and discovery of biocompatible and
fatigue-free silicone as constructional material for
the tubing resulted in higher success rates and
lower mortality. Eventually, ventriculo-peritoneal
(VP) shunting became the standard treatment for
hydrocephalus. There was therefore no perceived
need to develop neuroendoscopy for the treatment
of hydrocephalus.
While the utility of endoscopy in neurosurgery
became sparse from the 1960s onwards, a few key
technological advances have occurred that further
improved the quality of the endoscope. In 1966,
Hopkins and Storz developed a rigid endoscope
that used a new type of lens, the SELFOC lens. This
new technology obviated the need for the relay
lenses while preserving light transmission9,10. These
lenses also created a wider effective field of vision.
The advent of charge-coupled devices (CCDs)
marked another technological breakthrough. In
1969, George Smith and Willard Boyle invented
the first CCDs at Bell Laboratories11. The CCDs
are solid-state devices, usually a silicon chip,
which are capable of converting optical data into
electrical current, resulting in both improved
quality of the transmitted images and decreased
size of the endoscopic systems. Another important
technological breakthrough was the development
of fibre optics. Fibre optics allowed the light source
to be separated from the rest of the endoscope12.
Light could also be emitted from the tip of the
endoscope without significant heating through
one set of cables; while other specialised and
coherently arranged cables could be constructed
to conduct images without a loss of luminescence.
The combination of brighter light sources
and improved camera resolution significantly
advanced the quality of endoscopy, which led
to the subsequent reconsideration of its usage
in neurosurgery.
On the other hand, the dramatic success of
CSF shunting was later on met with various
complications such as hardware infection, improper
placement of catheter, obstruction, and hydraulic
mismanagement, i.e. over- or under-drainage13,14.
Shunt failure rates remained high even in modern
series. In recent publications with long follow-up
periods of six to twenty years, shunt revision rates
ranged from 32.5% in adult patients15 to 84.5% in
the paediatric population16. This has resulted in a
renewed interest in ETV in the 1990s, a then viable
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option owing to the improved imaging capabilities
of the endoscope17. A successful ETV negates the
need for a permanent implant and all its associated
problems mentioned above.
ENDOSCOPIC THIRD VENTRICULOSTOMY
Endoscopic third ventriculostomy (ETV) aimed
to create a new communication between the
ventricular system and the subarachnoid space,
by fenestrating the floor of the third ventricle.
Below is an example of a patient with obstructive
hydrocephalus secondary to a pineal tumour
treated with ETV.
A middle aged lady presented with headache and
unsteadiness for one month. Physical examination
revealed papilloedema and an unsteady gait.
In Figure 1 (please see overleaf ), the sagittal T1
post-contrast magnetic resonance imaging (MRI)
showed an enhanced tumour in the pineal region
causing obstructive hydrocephalus. The lateral and
third ventricles were dilated but the forth ventricle
was of normal size. The diagnosis was obstructive
hydrocephalus secondary to a pineal region
tumour. The dilated third ventricle and patency
of the prepontine cistern made this case suitable
for third ventriculostomy. The patient underwent
ETV. Her symptoms improved after the operation.
An MRI CSF flow study done on post-operative day
three demonstrated net forward motions through
the ventriculostomy, thus confirming its patency.
Indications for ETV
The pioneers of third ventriculostomy such as
Hirsh18, Hoffman19, Patterson and Bergland20,
recommended that the procedure should only
be reserved for patients with obstructive or
non-communicating hydrocephalus, in whom
the normal mechanism of CSF absorption still
exists at the arachnoid granulations. Indeed, the
literature showed that ETV is more effective if the
hydrocephalus is due to aqueductal stenosis21,22
or compression of aqueduct or fourth ventricle
by tumours23–25. Causes of hydrocephalus such
as meningitis, subarachnoid haemorrhage,
and intraventricular haemorrhage have been
used by some to exclude patients from ETV.
However, these have recently become relative
contraindications in view of the reduction of
the morbidity and mortality of ETV. Recent
publications have shown acceptable results of
ETV compared to VP shunt when its indication was
extended to patients with CSF infection26, normal
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Endoscopic Third Ventriculostomy and Fenestration
Fig. 1. A: Sagittal MRI, T1 sequence post-contrast showing an enhancing tumour in the pineal region causing
obstructive hydrocephalus. The lateral and third ventricles were dilated but the forth ventricle was of normal
size. B: Coronal MRI, T1 post-contrast showing similar findings. C: Axial MRI, FLAIR sequence, showing dilated
lateral ventricles and CSF transudation. D: Axial MRI, FLAIR sequence, showing dilated third ventricle which
made third ventriculostomy possible.
MRI: Magnetic resonance imaging
CSF: Cerebrospinal fluid
FLAIR: Fluid attenuated inversion recovery
Fig. 2. Snapshot of the navigation screen showing the selection of an entry point (lateral/
superficial crosshair) at the frontal region and a trajectory that will bring the endoscope to
the foramen of Monro (medial/deep crosshair) and then to the floor of the third ventricle with
minimum angulation.
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Review
pressure hydrocephalus (NPH)27, intraventricular
haemorrhage28, subarachnoid haemorrhage29, and
other communicating hydrocephalus30. Gangemi
et al31 published ETV success rates of 73.4% (33/45)
in patients with NPH and 60% (12/20) in patients
with hydrocephalus secondary to infection or
haemorrhage. The latest preliminary result of a
randomised controlled trial in Brazil where patients
with NPH were randomised to VP shunt (with
a fixed pressure valve) or ETV, showed that the
12-months improvement rate for VP shunt was
77%, whereas only 50% of patients who underwent
ETV showed improvement. The difference was
statistically significant32. Therefore at this point, it
remains difficult to advocate ETV as a standard or
routine treatment for NPH. More work still needs to
be done to refine the selection criteria and improve
the understanding of the pathophysiology of
communicating hydrocephalus. Apart from the
aetiology of hydrocephalus, the success rate of
ETV is also dependent on the age of patients.
In particular, children who undergo ETV at less
than six months of age are shown to have higher
failure rate33–35.
Surgical Anatomy and Technique
Familiarity with the ventricular anatomy is essential
in performing an ETV. A burr hole is made on
a point at the frontal region that will project a
straight line linking the foramen of Monro and the
tuber cinereum, the part of the floor of the third
ventricle to be perforated. Stereotactic navigation
is often employed to select the entry point and
determine the trajectory of the endoscope. Figure
2 is a snapshot of the navigation screen showing
the selection of an entry point at the frontal region
and a trajectory that will bring the endoscope to
the foramen of Monro and then to the floor of
the third ventricle with minimum angulation. The
foramen of Monro is usually the first structure
visualised after entry of the endoscope into the
frontal horn of the lateral ventricle. The standard
view of the foramen of Monro is illustrated in
Figure 3. The choroid plexus of the lateral ventricle
projects forward to the foramen, through which
it passes before turning posteriorly to lie under
the roof of the third ventricle. The septal vein
is located anteromedially along the septum
pellucidum. It joins the thalamostriate vein, located
posterolaterally, at the posterior rim of the foramen
of Monro to form the internal cerebral vein36. The
scope is then advanced through the foramen of
Monro. The endoscopic view of the floor of the
third ventricle is illustrated in Figure 4 (please see
overleaf ). The paired mammillary bodies marked
the posterior limit of the floor of the third ventricle.
The target of fenestration is the tuber cinereum,
the area between the mammillary bodies and the
infundibulum of the pituitary gland. It is important
to note that the basilar artery and the oculomotor
nerves are situated directly under the tuber
cinereum. A probe is then passed through the
working channel of the endoscope. Once the tip of
the probe appears in view of the endoscope, it is
Fig. 3. Standard endoscopic view of the right foramen of Monro.
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Endoscopic Third Ventriculostomy and Fenestration
Fig. 4. Endoscopic view of the floor of the third ventricle.
Table 1. Selected Publications of the Success Rates of ETV from 1990 to 2013.
^ success rate at 36 months; * 65% at one year, 52% at five years; f only included patients with tumour-related obstructive hydrocephalus.
guided to the tuber cinereum where a fenestration
is made. This has to be done carefully in order to
avoid bleeding from the basilar artery or damage
to the oculomotor nerve. The hole is then enlarged
with a 3 mm Fogarty balloon. Figure 4 showed
the fenestration being made by the probe.
Once haemostasis is achieved, the endoscope is
withdrawn gently.
Success Rates According to Literature
When Jones37 published his ETV series in 1990,
the success rate was only 50%. Success is defined
as adequate treatment of hydrocephalus without
the need for further VP shunt. The subsequent
two decades saw the improvement of success
rates ranging from 65% to 78% due to better
patient selection and improved techniques. Table
1 summarises some of the selected publications of
ETV success rates from 1990 to 2013. As a result, ETV
has become an attractive alternative to VP shunt.
It obviates the complications associated with an
implant such as infection, blockage, and overor under-drainage. The senior author’s personal
experience in performing ETV is from 2007 to 2013.
The commonest indications for surgery were brain
tumours causing obstructive hydrocephalus. In
the 10 adult patients who underwent ETV, there
was a success rate of 70%. Surgical aids such as
computer-based neuro-navigation were used to
enhance the safety of surgery. In his experience
with eight paediatric patients, the success rate
was 75%. Importantly, there were no ETV related
complications of haemorrhage or infection. Thus,
ETV has been found to be a viable alternative to VP
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Review
shunting in carefully selected cases.
fenestration followed by VP shunt.
COMPLICATIONS OF ENDOSCOPIC THIRD
VENTRICULOSTOMY
The common complications associated with ETV
include bleeding and infection. Bleeding could
occur from a number of sources, such as injury
of the basilar artery, intraventricular vessels, and
brain parenchyma. Minor bleeding will usually stop
with irrigation. Heavy bleeding may necessitate
abandonment of the procedure and placement
of an external ventricular drain. Infection can be
subdivided into superficial skin infection and
meningitis. The latter requires treatment with a
longer duration of intravenous antibiotic. The rate
of infection is approximately 2%38. The less common
complications associated with ETV include leakage
of cerebrospinal fluid, seizure, and hypothalamic
injury leading to diabetes insipidus. According to
a series of 193 ETVs published by Schroeder et al39,
the mortality rate was 1%, permanent morbidity
rate of 1.6%, and transient morbidity rate was
7.8%. In this series, the mortality and permanent
morbidity occurred in the author’s early experience,
thus indicating the steep learning curve associated
with this procedure.
A young girl presented with acute left hemiparesis
associated with a headache. On physical
examination, her power was 3/5. Computed
tomography of the brain showed a large right
basal ganglia haemorrhage which also discharged
into the ventricle resulting in intraventricular
haemorrhage. She underwent decompressive
craniectomy, evacuation of the haematoma,
and insertion of an external ventricular drain.
Unfortunately, she developed secondary bacterial
infection which finally led to a frank ventriculitis. As
a result, she developed loculated hydrocephalus.
In Figure 5, the MRI on the left shows a loculation
within the right frontal horn. Doing a VP shunt
required insertion of three intracranial catheters:
one in the left lateral ventricle, one in the right
lateral ventricle, and one in the loculated collection
within the right frontal horn. This surgical option
was not ideal, as more catheters would expose the
patient to a higher risk of catheter malposition,
blockage, and infection. Therefore, the patient
underwent an endoscopic fenestration of the right
frontal loculation and the septum pellucidum
followed by placement of a ventricular catheter at
the right frontal horn. In Figure 5, the MRI on the
right taken post-operatively shows that both the
lateral ventricles were adequately decompressed.
UTILITY OF ENDOSCOPE IN MULTILOCULATED
HYDROCEPHALUS
Neuroendoscopy is also a useful treatment for
multiloculated hydrocephalus. Endoscopy allowed
fenestration of the loculations with minimal
invasion. Below is an example of a patient with
loculated hydrocephalus treated with endoscopic
The
common
causes
of
multiloculated
hydrocephalus are ventriculitis and intraventricular
haemorrhage. Tumours that grow in the CSF
pathway may also result in obstruction of CSF
Fig. 5. Left: T2 sequence axial magnetic resonance imaging (MRI) showing loculation of the
right frontal horn. Right: MRI taken after the endoscopic fenestration of the right frontal
loculation and septum pellucidum. Both lateral ventricles were adequately decompressed.
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Endoscopic Third Ventriculostomy and Fenestration
flow and isolated hydrocephalus. It is a difficult
condition to treat and in most patients multiple
surgical revisions are required. Historically,
multiloculated hydrocephalus was usually treated
with multiple intracystic and intraventricular
catheters, and occasionally with microsurgical open
cyst fenestration49–52. The placement of multiple
catheters is associated with many problems.
The ventricular anatomy is frequently distorted
in patients with multiloculated hydrocephalus.
Malposition of the catheter is therefore common,
especially without the aid of computerised
surgical navigation tools. Even when navigation
is employed, drainage of CSF after insertion of
the first catheter may result in a significant shift
of the intracranial structures, thus rendering the
navigation unreliable for subsequent catheters
insertion. The cost of implants also increases
significantly when more shunt tubings and valves
are required. When a patient with multiple shunts
presents with infection, all the shunts need to
be removed. It becomes especially complicated
when a patient with multiple shunts present with
a blocked shunt, as it is very difficult to diagnose
which catheter is blocked.
It minimises the size of skull opening; a burr hole
is usually sufficient. It reduces brain retraction and
trauma and facilitates deep access mainly through
preformed and existing cavities53. Combined with
navigation, fenestration of the membrane could
be made at the appropriate locations and the
ventricular catheter could also be guided into
a good position. In patients with tumour in the
third ventricle causing a blockage of the bilateral
foramen of Monro, fenestration of the septum
pellucidum could re-establish the communication
of both lateral ventricles, thus avoiding placement
of two shunts.
Multiple shunts can be avoided if the
multiloculated hydrocephalus is converted to a
unilocular hydrocephalus. Open microsurgical
fenestration is an option, but it is invasive with
associated complications. A craniotomy is required
and a large corticectomy is frequently needed to
access the ventricles. The advantages of using an
endoscope to examine the ventricle are obvious.
A middle aged lady presented with headache
associated with features of increased intracranial
pressure. An MRI showed a large thalamic tumour
causing an obstruction of the bilateral foramen of
Monro. She underwent endoscopic fenestration
of the septum pellucidum followed by insertion
of VP shunt. Figure 6 shows the steps involved in
the insertion of a ventriculoscope for fenestration
ENDOSCOPIC FENESTRATION OF THE SEPTUM
PELLUCIDUM
In cases where both the foramina of Monro are
blocked, endoscopic fenestration of the septum
pellucidum could be performed prior to shunting,
thus avoiding the insertion of two intraventricular
catheters. Below is an example of a patient who
has obstructive hydrocephalus secondary to a
large thalamic tumour, treated with endoscopic
fenestration of the septum pellucidum followed by
VP shunt.
Fig. 6. A: Picture showing a rigid ventriculoscope. The patient has been positioned and draped for
a ventriculoperitoneal shunt surgery. B: A burr hole has been made. The lateral ventricle was first
cannulated with a Dandy needle. C: The ventriculoscope was introduced through the same path
created by the Dandy needle. D: The camera was introduced.
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Fig. 7. The ventriculoscope has been registered to the navigation system. The
axial and coronal views of the navigation screen showed that the structure
seen from the scope camera was the septum pellucidum.
Fig. 8. Endoscopic picture showing fenestration of the septum pellucidum.
After the septal fenestration was done, the thalamic tumour was endoscopically
biopsied.
of septum pellucidum. We preferred to use the
rigid ventriculoscope because of superior image
quality. The patient was positioned and draped
as for VP shunt insertion. A frontal burr hole was
made. The lateral ventricle was first cannulated
by a Dandy needle. The rigid ventriculoscope
was then introduced, following the same path
created by the Dandy needle. The camera was
then introduced. The endoscopic view confirmed
the successful placement of the scope within
the frontal horn of the lateral ventricle. Prior to
210
inserting the ventriculoscope, the scope was
registered to the navigation system. In Figure 7,
the axial and coronal MRI on the navigation screen
showed that the structure seen on the endoscopic
camera was the septum pellucidum. The septum
was then fenestrated with a monopolar probe and
dilated with the scope itself. Figure 8 shows the
fenestration made at the septum pellucidum. After
the septal fenestration was done, the thalamic
tumour was biopsied endoscopically. Her headache
resolved after the surgery.
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Endoscopic Third Ventriculostomy and Fenestration
CHALLENGES IN PROMOTING THE USE
OF ENDOSCOPY IN THE TREATMENT OF
HYDROCEPHALUS
The current recognised indications of endoscopic
treatment of hydrocephalus are ETV for obstructive
hydrocephalus, and endoscopic fenestration in
multiloculated hydrocephalus. Recent publications
have shown acceptable results of ETV compared
to VP shunt when its indication was extended to
patients with CSF infection26, NPH27, intraventricular
haemorrhages28, subarachnoid haemorrhage29,
and other communicating hydrocephalus30. With
a shunt free rate of 65% to 75%, ETV has shown
superior results compared to VP shunt, when used
for good indications.
The volume of endoscopic work in a typical
neurosurgery department is relatively low. It is
also associated with a steep learning curve. As
a result, endoscopic surgery is only practiced
by a few neurosurgeons who have developed
subspecialised interest. Most neurosurgeons
would still prefer to treat hydrocephalus with VP
shunt, an established surgery with relatively simple
technique. However, as the literature on endoscopy
usage and its advantages are further disseminated,
it is likely that more neurosurgeons will consider it
as an option.
Capital investment is needed in the purchase of
the endoscopic system, including the endoscopic
camera, telescope, light source, and recording
device. The huge sum of money required often
serves as an obstacle for small neurosurgical
departments
to
employ
neuroendoscopy.
Nevertheless, the capital investment could be offset
by the money saved from not using additional
implants. Potential savings could be derived from
the avoidance of treatment complications of VP
shunt such as infection, and revision surgeries
required for blockage and over- or under-drainage.
CONCLUSION
The improvement of the image quality of endoscope,
as well as the endoscopic tools such as diathermy
and scissors have revived the use of endoscopy
in treatment of hydrocephalus. The aim is to treat
hydrocephalus without the use of an implant,
such as a shunt, thus avoiding the complications
associated with a permanent implant such as
infection and malfunction. Endoscopic surgeries
employed in the treatment of hydrocephalus are
ETV, fenestration of multi-loculated hydrocephalus,
and fenestration of septum pellucidum before
shunting. Endoscopic treatment of hydrocephalus
may become more widely accepted and practiced
with the increase in success rate, reduction of
morbidity and mortality, and emergence of new
evidences supporting its role in treatment of
communicating hydrocephalus. Further research
is required to refine the selection criteria in
order to identify patients with communicating
hydrocephalus who will likely respond to ETV.
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Proceedings of Singapore Healthcare  Volume 22  Number 3  2013
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