SYMPOSIUM: NEUROLOGY
Management of neonatal
hydrocephalus
Neil Buxton
Abstract
Neonatal hydrocephalus is a complex disorder due to many different
causes. This review seeks to encapsulate the management of neonatal
hydrocephalus in the term neonate. The current treatments are explored
and explained.
Keywords hydrocephalus; intraventricular haemorrhage; neonatal
meningitis; neonates; shunts
Introduction
Neonatal hydrocephalus (NHC) is increasingly becoming the
most difficult management problem in paediatric neurosurgery
but survival from the hydrocephalus has improved. There are
many problems associated with aetiology, body weight and
immaturity, including unfused sutures, relating to risks of
infection and controversies with actual treatment protocols. This
review is intended to give an overview of current thoughts on the
management of hydrocephalus in the term neonate. The
management of hydrocephalus (HC) in the premature child will
not be covered.
Neonatal hydrocephalus occurs in approximately 1 in 1000
live births. It is secondary to full term intraventricular haemor-
rhage (IVH), infection or congenital causes such as tumours,
aqueduct stenosis, DandyeWalker syndrome and its variants or,
of course, it can be truly idiopathic.
Where possible, treatment of the cause is the first priority but
in many cases the treatment of the hydrocephalus takes prece-
dence. For example, in meningitis with HC, draining an enlarged
ventricular system may be necessary even before the infection
has been completely cleared.
Birth weight influences treatment choices as well. There is
a reluctance to introduce any permanent shunt systems into
a child less than 2 kg in weight because below this weight there is
a substantially increased risk of shunt failure due to infection.
Fortunately, most term babies exceed this weight.
Treatment choices
All authorities in Western countries agree that, except in the most
devastating of circumstances, the HC should be treated. Difficulty
arises in choosing the best treatment option for a particular
aetiology and is discussed below.
Neil Buxton MB ChB DMCC FRCS(Ed) FRCS(Neuro Surg) is a Consultant Paediatric
Neurosurgeon at the Royal Liverpool Children’s Hospital, Liverpool, UK.
Conflict of interest: none.
PAEDIATRICS AND CHILD HEALTH 21:11
Intraventricular haemorrhage
Full term IVH probably occurs more often than we think but is
more common at lower birth weights. IVH does not invariably
cause HC. There is controversy as to how HC follows IVH. Some
have suggested that proteinaceous material blocks the arachnoid
granulations, thus resulting in excess cerebrospinal fluid (CSF)
due to reduced reabsorption. In some cases there must be some
kind of block to CSF passage at the exit foraminae of the fourth
ventricle, as this would explain why endoscopic third ven-
triculostomy (ETV) works in some patients with IVH (18%).
However, the majority need to have shunts inserted and, despite
many recently advocating the use of ETV this is still considered
by most to be the treatment of first option.
ETV has also been used to washout heavy protein loaded CSF
and to enable shunting to be implemented somewhat earlier or
even avoided. In heavy blood/protein loaded CSF, drainage may
be needed for some time before shunting to reduce the risk of
shunt blockage, although lumbar drainage has been successful in
avoiding shunting in IVH (see below).
A study into intraventricular washout looks promising in this
age group and may reduce the risk of subsequent hydrocephalus.
Neonatal meningitis
Meningitis can also lead to HC. This may be due to a heavy
protein load causing problems at the arachnoid granulations or
discrete blockages of exit foraminae by debris or membranes.
Hence, again, ETV has been shown to be effective in some post-
meningitic HC cases but the majority will require shunt insertion.
During the acute, infective phase, and whilst there is heavy
protein load in the CSF, it may be necessary to drain the HC with
an external ventricular drain (EVD) in order to reduce the intra-
cranial hypertension. Obviously, such a device can be used to
drain excess CSF, but is also useful for obtaining CSF samples for
serial cultures and for the administration of intraventricular
antibiotics (a technique restricted to specialist neurosurgical
units). Draining the CSF in this way will allow it to return to its
normal constituent levels, so allowing shunting to be imple-
mented. It is generally believed that the higher the protein load of
the CSF the more likely it is that the shunt will fail due to
blockage by debris; this is not the case with HC secondary to
tuberculous meningitis. In these circumstances, the protein load
is much less important and shunting can take place earlier.
Non-communicating hydrocephalus
The terminology communicating and non-communicating
hydrocephalus is becoming controversial, partly because of
issues with post-infectious and post-haemorrhagic HC, as briefly
mentioned above. It is clear that tumours, aqueduct stenosis and
DandyeWalker syndrome and its variants can have physical
blocks to the passage of the CSF, and thus can lead to truly non-
communicating HC. In these types of HC, seemingly in all ages,
ETV is the treatment of choice.
Unfortunately, ETV in the truly non-communicating hydro-
cephalus in some younger children will still fail. There are no
accepted theories for this but it may be that the pressure of CSF
required to initiate CSF reabsorption via the arachnoid granula-
tions exceeds the pressure needed to expand the cranium in those
with unfused sutures; in such a situation, ETV is certain to fail
and a shunt is required. Whilst seemingly very simple, shunting
510 Ó 2012 Published by Elsevier Ltd.
 SYMPOSIUM: NEUROLOGY
is controversial too and in order to address this small number of
children and their requirements an international randomized trial
is looking at the efficacy of shunting versus ETV.
Idiopathic
So-called idiopathic HC is best treated by treating the underlying
anatomical precedence. If there is evidence of flow obstruction,
then ETV may well work; otherwise it is likely that shunting will
be required.
Tumours
In the presence of third or fourth ventricular tumours or tectal plate
or brainstem tumours causing HC, the HC can easily be treated by
ETV and in some, biopsies obtained. In successful resection of
a cerebellar tumour, for example, CSF flow may be restored and no
longer requires diversion. ETV or shunt is often needed, however,
although temporary EVD may ‘buy enough time’ for definitive
tumour treatment. Arachnoid cysts in or near the third ventricle
behave like tumours and can be successfully managed by ETV.
Low birth weight
If the birth weight is less than 2 kg, then shunting tends not to be
recommended. This is because of concerns about the anaesthetic,
neonatal care, risk of infection, operating on someone so small,
etc, with infection of the shunt being the most worrisome. The
HC can be ameliorated until the baby gains weight by serial
lumbar punctures, serial ventricular taps, an EVD (which can be
used up to 3 weeks without changing, with care) or a more
permanent Ommaya reservoir (an implanted ventricular tube
with an injection part). Intuitively there is concern about intro-
ducing a permanent or semi-permanent foreign body into the
child, just as there is concern over definitive shunting; however,
this seems to be safe in cases of low birth weight and post-
haemorrhagic hydrocephalus.
Congenital problems
Many patients with hydrocephalus will have other problems
such as spina bifida and chiari malformations. Usually the
overwhelming problem is to make sure that the hydrocephalus is
managed effectively and the other problems dealt with subse-
quently. ETV is an option in many of these cases.
Which shunt?
The type of shunt largely depends on the individual surgeon, their
experience with a particular model and their own biases. Whilst
this approach is not scientifically sound, a surgeon will get ‘used’
to a particular model, understand its idiosyncrasies and become
confident with its use. This is perhaps more important than any
other consideration such as cost, ‘newness’, etc, as the surgeon
uses their own experience to decide what is best for a particular
patient. This is where experience counts and, dare it be said, some
of the art in the science remains. Notwithstanding all of the above,
most would agree that the smaller the child the less bulky the
shunt and the quicker it should be to insert, with fewer compo-
nents to increase surgery time (hence infection risk) and in the
long run with fewer options for malfunction. Unfortunately, the
answer to the problem of deranged physiology is difficult to
identify when we have mechanical devices with fixed tolerances
for, literally, a fluid system.
PAEDIATRICS AND CHILD HEALTH 21:11
Where does the distal end go? Obviously in treating HC we are
inserting the upper end into the lateral ventricle. This tends to be
on the right (the non-dominant hemisphere). This is connected to
a one-way valve device to the distal catheter. The distal end is
placed into the peritoneal cavity by choice, although common
alternatives include the right atrium via a neck vein and the
superior vena cava (the historical site of choice) and also the
pleural space. The pleura is used as a last resort in those in whom
there has been extensive abdominal surgery, peritonitis or
necrotizing enterocolitis, and whose neck veins have been
damaged by central lines. Pleural shunts always cause effusions
and, if there is a significant CSF volume, then the effusion
resulting may embarrass lung function. A balance must be
struck. Similarly, in the abdomen there can be a bulging tense
abdominal wall and the development of CSF hydroceles.
Shunt failure
This is almost inevitable in the lifetime of a patient with a shunt,
with the greatest number, approximately 20%, occurring in the
first year after insertion. Failure manifests itself in many ways,
e.g. increased head circumference, tense fontanel, drowsiness,
vomiting, squint, CSF tracking alongside the shunt tubing, signs
of infection, and banging the head with the hands or against
something, which can indicate headache. In these circumstances
shunt revision is usually required. It is a neurosurgical rule that if
the primary carer says that the child is ‘not right’ and that they
‘think it’s the shunt’, it is a brave and foolhardy person to ignore
the warning.
In some in whom a shunt subsequently fails, an ETV may well
work and paediatric neurosurgeons will always assess a ‘new’
shunt failure for anatomical suitability for the procedure.
Conclusion
The numbers of children surviving to term in the West with HC
are increasing as better obstetric care, earlier antenatal diagnosis
and better awareness lead to more informed decisions. In the last
20 years, paediatric neurosurgery has evolved into a distinct
subspeciality on a par, for example, with spinal neurosurgery.
More aggressive, better targeted treatment for paediatric HC, no
longer in isolation from other children’s specialists, provided by
surgeons with expertise and training in the management of these
difficult clinical scenarios is improving the situation for these
patients. Treatment in the West has moved out of the hands of
paediatric general surgeons but their historical contribution
cannot be underestimated as without their skills and expertise
there would be no paediatric neurosurgery at all. With the
development of paediatric neurosurgical centres there is no
longer an excuse to dabble in the management of such complex
problems and such an approach is to be discouraged. A
FURTHER READING
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Buxton N, Macarthur D, Robertson I, et al. Neuroendoscopic third ven-
triculostomy for failed shunts. Surg Neurol 2003; 60: 201e3.
511 Ó 2012 Published by Elsevier Ltd.
 SYMPOSIUM: NEUROLOGY
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PAEDIATRICS AND CHILD HEALTH 21:11
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Practice points
C Neonatal hydrocephalus has varied aetiology
C Choice of method of treatment for the hydrocephalus can
depend heavily on the aetiology
C Early consultation with a paediatric neurosurgeon is essential
512 Ó 2012 Published by Elsevier Ltd.