The Indian Journal of Pediatrics (October 2019) 86(10):952–960

https://doi.org/10.1007/s12098-019-02962-z

REVIEW ARTICLE

Neuroimaging in Pediatric Hydrocephalus

Pradeep Krishnan 1 & Charles Raybaud 1 & Sunitha Palasamudram 2 & Manohar Shroff 3

Received: 2 December 2018 / Accepted: 10 April 2019 / Published online: 10 May 2019
# Dr. K C Chaudhuri Foundation 2019

Abstract
The objective of this review is to discuss the role of neuroimaging in evaluating pediatric and fetal hydrocephalus based on
possible pathophysiologic mechanisms and in the context of differing etiology. Although conventional brain imaging with
ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI) is commonly used to assess for ventricular
enlargement, however, the underlying mechanisms and management of hydrocephalus is a challenge in pediatric population and
fetal hydrocephalus. MRI helps define the possible nature of the obstruction, and provides useful functional and anatomic
information. MR imaging, in both pediatric and fetal hydrocephalus, thus may help in better understanding of the possible
pathophysiologic mechanisms of the varied causal factors. The article focuses on the usage of MRI sequences in both diagnosis
and follow-up of pediatric and fetal hydrocephalus, to be able to investigate all possible etiopathogenesis through the CSF
pathway and to assess the efficacy of treatment in a non-invasive standardized manner.

Keywords Pediatric . Hydrocephalus . Fetal . MRI

Introduction intracranial pressure. Evaluation of the precise pathogenesis

Hydrocephalus is a common pediatric condition predom-
inantly resulting from an imbalance between the produc-
tion and absorption of cerebral spinal fluid (CSF) which
may be isolated or associated with several neurological
conditions and diseases.
Rekate defined hydrocephalus as Bactive distension of the
ventricular system of the brain resulting from the inadequate
passage of CSF from its point of production within the cere-
bral ventricles to its point of absorption into the systemic
circulation^ [1–3].
The onset of hydrocephalus can be acute or chronic. The
imbalance between the production and absorption of CSF re-
sults in an increased volume of cerebrospinal fluid, causing
dilatation of the ventricular system and often increased
* Pradeep Krishnan
pradeep.krishnan@sickkids.ca
1 This review contains a brief overview of the current concepts
Department of Neuroradiology, The Hospital for Sick Children, 555
University Avenue, Toronto M5G1X8, Canada
2 The review discusses the utility of various cross-sectional im-
The Hospital for Sick Children, 555 University Avenue,
Toronto M5G1X8, Canada
3 alus from present day conventional techniques to advanced
Department of Diagnostic Imaging, The Hospital for Sick Children,
555 University Avenue, Toronto M5G1X8, Canada
of hydrocephalus in each case may be complicated, since the
possible causal processes are varied. Additionally, in the pe-
diatric brain, depending on the timing and duration of the
insult, the process may interfere with healthy brain develop-
ment. The resultant changes may lead to ependymal damage
and microstructural white matter alteration, altered cerebral
blood flow and metabolism, cortical gliosis, capillary com-
pression, and axonal damage [4]. MR imaging provides the
opportunity to evaluate the causal disease and to assess the
effects of hydrocephalus on the developing brain [5].
Hydrocephalus is the most common disease treated by pe-
diatric neurosurgeons. The prevalence of infant hydrocepha-
lus is roughly one case per 1000 births, probably more signif-
icant in developing countries. The most common causal
mechanisms in developed countries are post-hemorrhagic hy-
drocephalus of prematurity, congenital aqueduct stenosis,
myelomeningocele, and brain tumors. However, in develop-
ing countries, postinfectious hydrocephalus is the predomi-
nant causal factor, likely due to the higher incidence of neo-
natal sepsis [6].
of CSF physiology and the understanding of hydrocephalus.
aging modalities for the diagnosis and treatment of hydroceph-
magnetic resonance imaging (MRI) techniques and analyses.
Indian J Pediatr (October 2019) 86(10):952–960
Mechanisms of CSF Secretion
The embryonic neural tube closes at the end of the fourth
week of gestation and is nourished by the amniotic fluid.
After closure, the neural tube is covered by the primitive
meninges. There is proliferation of primitive capillaries
and nutrients which diffuse into the neuroepithelium.
The fluid produced by the neuroepithelium fills the devel-
oping rostral brain vesicles and the meningeal mesen-
chyme projects dorsally into their lumen. This forms early
choroid plexuses at around five to seven gestational
weeks, which start secreting ventricular CSF. The choroid
plexus is a vascular network of fenestrated capillaries
which are leaky, and, in contrast to cerebral endothelium,
readily allow the passage of ions and other small mole-
cules. This facilitates transport of various neurotrophic
growth factors, oxygen and proteins to the periventricular
germinal zone till around 26–28 wk [4]. The choroid plex-
uses also play a role in the removal of endogenous and
exogenous substances from the CSF.
The ventricles are lined by germinative ventricular ep-
ithelium. The ependyma develops late and is complete at
about 26–28 wk with differentiation of the radial glia
when the ventricular zone regresses. Ependymal cells are
ciliated cuboidal cells which help orient the diffusion of
neurotrophic factors and guidance molecules, remove de-
posits, and facilitate CSF flow in narrow channels.
Additionally, the ependyma appears to play a role in
supporting stem cells of the subventricular zone.
Ependymal denudation has been associated with the de-
velopment of nodular heterotopia. The extracerebral CSF
spaces develop while the meningeal layers become differ-
entiated and become generalized by about 8 wk [4].
Recent studies have indicated that the choroid plexus
generates approximately 60–90% of CSF, whereas the re-
maining 10–40% is derived from brain and cord parenchy-
ma [7]. Choroid plexuses secrete CSF by passive exudation
from the vessels into the choroid Interstitial space fluid
(ISF), and regulate transport across the epithelial layer into
the ventricle. The CSF secretion is also regulated by vari-
ous vasoactive neuropeptides. The total volume of CSF in
the adult brain (i.e., within the cerebral ventricles and the
subarachnoid spaces) is estimated to be approximately
150 ml and about 100–120 ml for the spinal spaces with
around 500–600 ml produced every 24 h, which corre-
sponds to turnover of 3–4 times daily. The total CSF vol-
ume in children is approximately 60–100 ml, and 50 ml in
newborns. The ventricular volume is around 10–15 ml in
neonates and increases in children. The absorption rate is
in the range of 0.35 ml/min with variations due to physio-
logic activities. Choroid plexus ablation does not suppress
the production of CSF, there is ongoing exchange of water
molecules diffusely across the brain surfaces. The
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extracerebral CSF includes Virchow Robin Spaces (VRS)
and Interstitial Space Fluid (ISF) and forms a single brain
extracellular fluid compartment. ISF content of the central
nervous system is estimated to be 100–300 ml. This high-
lights the presence of additional factors that influence CSF
homeostasis [4, 8].
The flow of ISF is dynamic with a preferentially
perivascular route and can contribute to both net CSF produc-
tion and reabsorption. The constituents of this dynamic pro-
cess have been called the Bg-lymphatic system.^ These para
vascular channels are bound by astrocytic end feet containing
aquaporin 4 (AQP4). These function as a route for trans
ependymal, extracellular movement of water into CSF spaces.
The recent hypothesis proposes that arterial and venous VRS
together would form a loop with the pulsating wave of the
arterial VRS driving water toward the parenchyma and the
venous VRS draining it toward the arachnoid space (Fig. 1).
However, the role of the g-lymphatic system in the adaptation
of CSF secretion is still unknown.
Also, other transdural drainage routes including dural chan-
nels, venous plexuses of the cavernous sinuses around the
internal carotid and in the sellar region, nasal mucosa and
along the olfactory nerves, optic nerve sheaths and other cra-
nial and spinal nerve outlets, with their arachnoid villi, also
contribute to CSF absorption.
Hydrocephalus and CSF Production
The standard bulk flow model of CSF physiology based on
the concepts formulated by Dandy and Blackfan was the par-
adigm used for the description of pathogenesis of hydroceph-
alus [9]. According to this model, CSF from the choroid plex-
us passes down from the lateral ventricles into the subarach-
noid space over the cerebral convexity and spinal cord and is
finally resorbed by arachnoid granulations into the cerebral
venous system. Hydrocephalus results from obstruction to
CSF flow anywhere along this pathway with upstream dilata-
tion as per this hypothesis.
The free exchange of water molecules across the
ependyma with the ISF and therefore, with the blood
has been known and recognized by Bering. The potential
role for pulsating choroid plexuses in ventricular enlarge-
ment had also been suggested. Further improved under-
standing of water transport channels; aquaporins and rec-
ognition of aquaporin-4 (AQP4) expression in ependymal
cells and astrocytic endfeet strengthens the hypothesis of
transport of water from ventricles to the interstitial and the
leptomeningeal surface as an alternative hydrodynamic
model of hydrocephalus [7]. In this model, the role of
abnormal intracranial pulsations in disease pathogenesis
is emphasized. This addresses inherent deficiencies with
the bulk flow model (Fig. 2).
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Fig. 1 Model of g-lymphatic
sytem
CSF Hydrodynamics
There is improved understanding and evidence supporting the
presence of multiple sites of CSF production and absorption,
as well as potential sites of bulk CSF flow within the lateral
and third ventricles, fourth ventricle, convexity cisternal
spaces, and spinal canal.
Systolic pulse pressure in CSF hydrodynamics, which
originates from the arterial system including cerebral and
choroidal vessels accounts for the larger volume of the
CSF displaced across the tentorial incisura and upper spine
as compared to the displacement observed at the level of the
aqueduct [10]. The movement is easily detected across the
aqueduct (oscillatory flow volume of about 1.7 ml/min), the
incisura (oscillatory volume of about 14.5 ml/min), the
Fig. 2 Models of CSF circulation: bulk flows. a Traditional model
(vertical bulk flow). This model postulates that the CSF, secreted by
choroid plexuses, would flow along the ventricles, then into the
cisterns, and then toward the convexity to be absorbed at the level of
arachnoid villi. b Modern model (transverse bulk flows). The present
Indian J Pediatr (October 2019) 86(10):952–960
upper cervical canal (oscillatory volume of about 39 ml/
min), and along the spinal cord, as it produces the MRI T2
SE flow void artifacts. Compared to these, the amount of
fluid displaced through the absorption sites, about 0.35 ml/
min, is far less than at C2–C3. The prominent flow voids in
the partially enclosed prepontine, suprasellar, and Sylvian
cisterns reflect local turbulences due to basilar and carotid
pulsations and pulsations due to branch arteries in these
regions. The lack of aqueductal flow void in normal neo-
nates can be explained by the peripheral pulsations of elastic
sutures / fontanels compensating for the small size of the
spine relative to the cranial cavity.
Intracranial compliance (ICC) refers to the change in vol-
ume per unit change in pressure. In normal conditions (normal
intracranial volumes and ICP), the degree of compliance
day model stipulates that the CSF secreted in the lateral, third and
fourth ventricles is absorbed locally at the corresponding ependymal
levels, allowing specific signalling molecules to be secreted and reach
their local sub ependymal targets at each level. The pericerebral CSF
produced by the brain is also absorbed transdurally at the periphery
Indian J Pediatr (October 2019) 86(10):952–960
determines the ability of the intracranial compartment to ac-
commodate an increase in volume without an associated in-
crease in intracranial pressure (ICP). The loss of compliance is
an important factor in hydrocephalus either by restriction of
the dampening force or by increased impedance to CSF dis-
placement [7]. However, in young children, the brain volume
may remain stable until increased pressures compress the
capillary-venular bed and compromise ISF drainage by
ependymal absorption, resulting in the accumulation of fluid
in the ventricles. Combining failure of absorption with loss of
compliance may offer a plausible explanation in cases of
chronic (compensated) hydrocephalus with acute decompen-
sation, because the ventriculomegaly reaches a threshold be-
yond which absorption cannot adjust anymore. This may be
facilitated by the progressive closure of sutures, or precipitated
by trauma or infection. Casual etiologies like tumor and infec-
tion may simultaneously compromise both compliance and
absorption (Fig. 3) [7].
The process of facilitation of CSF flow by the cilia of
the ependymal cells is less well understood. This is be-
lieved to play a role in the diffusion of nutrients and
signaling molecules and distribution of neuroendocrine
signals. Ciliary activity is also believed to protect ventric-
ular walls from debris and help maintain proper homeo-
stasis along ventricular walls [4].
The traditional concept that hydrocephalus is the result of
an imbalance between the production and absorption of CSF,
also known as the bulk flow theory has been challenged espe-
cially with regard to communicating hydrocephalus, with
studies demonstrating routes of CSF absorption in capillaries
and not only arachnoid granulations as once believed.
However the consensus classification of hydrocephalus re-
cently proposed reflects the surgical approach to hydrocepha-
lus, which is to divert the CSF between the site of production
and the location of the obstruction, based on the concept of
points of restriction of flow [3].
Fig. 3 Case of high pressure,
obstructive hydrocephalus in a
17-d-old child who presented
with group B streptococcal (GBS)
meningitis. MRI axial T2 (a) and
coronal T2 (b) images demon-
strate poor absorption of CSF
with rapid ventriculomegaly
(arrows) on day 5 of birth. Also
visible is the immature cranial
vault that gives way
955
Imaging Tools
Computed Tomography (CT)
CT is commonly utilized in the assessment of hydrocephalus,
being a fast, reliable technique with no need for metal screen-
ing, making it extremely useful in the emergency setting [11].
Also, due to the short duration of the test, children often do not
need to be sedated. CT is more reliable for catheter tip local-
ization than MR. However, one of the concerns with repeated
use of CT in pediatric patients for hydrocephalus, is the po-
tential for long-term effects of ionizing radiations. Although
low-dose CT protocols have been established to reduce dose
accumulation, repeated exposure due to multiple examina-
tions may have harmful effects on the developing brain and
associated lifetime oncologic risk.
Additionally, CT for initial and subsequent evaluation
of pediatric hydrocephalus may not provide sufficient an-
atomic detail or information to aid in neurosurgical man-
agement such as endoscopic third ventriculostomy (ETV)
procedures. Children with hydrocephalus often require
long-term follow-up and multiple investigations to assess
shunt function, thus requiring many imaging studies over
the course of their lifetime.
Ultrasound
Ultrasound is extremely helpful in the assessment of brain and
ventricles during the first few months of life when the anterior
fontanelle is patent. Ultrasound is used as a primary bedside
screening test to evaluate intraventricular hemorrhage and hy-
drocephalus in neonates [12]. Even though the test is adequate
for gross assessment of ventricles, it can be challenging to
visualize details related to the adjacent parenchyma and the
third and fourth ventricle. Besides, it is limited in its role for
evaluation of the posterior fossa and is highly user-dependent.
956
Hence, ultrasound is rarely used as the only diagnostic test for
evaluation of hydrocephalus. Ultrasound is best used as a
screening or surveillance test and for follow-up of pediatric
hydrocephalus before or after treatment typically early in life
when the anterior fontanelle is patent.
Magnetic Resonance Imaging (MRI)
MRI is the current modality of choice for assessing pediatric
hydrocephalus. MRI is used for surveillance of ventricular
size, identification of the underlying etiology of hydrocepha-
lus, assessment for ETV patency, and assessment of parenchy-
mal changes and peri-cerebral spaces amongst others.
When using MRI to evaluate hydrocephalus, apart from the
radiological assessment of the size of the ventricles, additional
features including the presence or absence of periventricular
interstitial edema, stretching of the corpus callosum (CC),
effacement of cerebral sulci, and absence of the aqueductal
CSF flow void phenomenon need to be evaluated. Several
specific sequences are tailored to obtain additional informa-
tion for this purpose [13].
T1-3D (MPRAGE, SPGR), coronal and axial T2-FSE are
of help in the assessment of septal dehiscence, effacement of
pericerebral spaces and stretching of the hippocampal com-
missure along the midline. Thin sagittal T2-FSE sequences,
also help determine ventricular, aqueductal and cisternal CSF
flow voids, bowing of the corpus callosum and bulging of the
supra-optic and infundibular recesses of the tuber cinereum,
and the supra-pineal recess. The aqueduct can be assessed for
the presence and degree of the aqueductal CSF flow void,
which can be complementary to phase contrast CSF flow im-
aging. However, aqueductal flow void is somewhat attenuated
in healthy young infants. The FLAIR sequence may be useful
for evaluating periventricular interstitial edema secondary to
the hydrocephalus or subsequent white matter changes in
long-standing pediatric hydrocephalus [13].
Sagittal steady-state T2 imaging (FIESTA/CISS sequence)
and 3D SPACE techniques are beneficial for preoperative as-
sessment when considering an endoscopic third ventriculostomy
for the treatment of hydrocephalus. These are helpful, for ascer-
taining post-operative patency of the third ventriculostomy, eval-
uating ETV failures including webs and membranes in the cis-
terns, thin membranes in the ventricles or aqueduct (Fig. 4). This
combination of imaging sequences with high spatial resolution
delineates tissue-fluid interface, enabling visualization of fine
details of CSF pathways and associated membranes. Along with
thin sagittal T2FSE sequences, these may be useful to demon-
strate the anatomic ETV site as well as to confirm functional
adequacy of the procedure (flow turbulence across the ETV
defect in the floor of the third ventricle) (Fig. 5). MR
Cisternography has been recommended and is considered safe,
however has failed to gain real acceptance as the trend in brain
imaging is toward minimal invasiveness [4].
Indian J Pediatr (October 2019) 86(10):952–960
Several non-invasive imaging parameters have been eval-
uated for determining the patency of an ETV, including a
change in ventricular size, flow void signal intensity,
aqueductal velocity and velocity ratios and MRI phase con-
trast (PC) studies. Although hydrocephalus is known to be
associated with increased aqueductal flow pulsatility, there is
a lack of clear association between MR pulsatile flow values
and outcomes from surgical interventions, hence limiting the
ability to predict success with ETV or shunting.
Phase contrast imaging is used to demonstrate flow and
allows flow quantification. PC-MRI is a non-invasive tech-
nique that uses flow-sensitive gradient echo (GRE) sequence
to assess CSF flow velocity and its directionality during a
cardiac cycle. In combination, 3D-CISS sequences and PC-
MRI can provide detailed information about CSF pathways,
the directionality of flow, potential areas of obstruction and
targets for intervention. Other MR pulse sequences such as
time-spatial labeling inversion pulse technique (Time-SLIP)
have been studied for the evaluation of CSF in physiological
conditions, including, to demonstrate CSF motion [14].
Diffusion tensor imaging (DTI) has been used to quantify
microstructural changes in the white matter. Several studies
have shown neurocognitive outcomes to be independent of
ventricular volume, further suggesting that using traditional
parameters such as improvement in ventricular size or volume
as indices of improvement are not adequate. DTI helps provide
further insight into the underlying mechanisms of white matter
(WM) damage and its reversibility with surgical intervention.
MR perfusion using the arterial spin labeling technique (ASL)
may be used to investigate blood flow in the parenchyma [13].
Imaging Morphology Based
on the Pathogenesis of Hydrocephalus
There are many quantitative imaging assessment tools de-
scribed for hydrocephalus, mainly applied in the adult
population like: Evans’ index, widening of the third ven-
tricular recesses, decreased mamillopontine distance and
frontal horn angle. Following successful surgical manage-
ment, most of these parameters approach near normal, but
often remain significantly different from normal values. It
is also recommended to use the combination of these pa-
rameters in the context of relevant imaging findings;
aware of their potential pitfalls [15].
Functional magnetic resonance imaging (fMRI) provides
an opportunity for a more global pathophysiologic approach
to pediatric hydrocephalus. One can incorporate several path-
ogenetic mechanisms of CSF hydrodynamics discussed earli-
er; obstructive vs. non-obstructive, compliance, secretion/
absorption balance, chronic vs. acute presentation, duration
and parenchymal changes, specifics of causal pathologies,
and vulnerability of developing brain and repair process [4].
Indian J Pediatr (October 2019) 86(10):952–960
Fig. 4 Sagittal steady-state T2
MR imaging (FIESTA/CISS se-
quence) shows (a) normal ap-
pearance (b) a thin membrane at
the level of aqueduct (arrow) with
resultant hydrocephalus
Obstructive hydrocephalus associated with raised intracra-
nial pressure, is commonly caused by midline tumors due to
their location. The raised venous pressure and cerebral swell-
ing further impede CSF absorption by the ventricular
ependyma. Additionally, compression of the subependymal
veins results in periventricular interstitial edema, a feature of
acute obstructive hydrocephalus. This is identified as
periventricular FLAIR hyperintense rim which is more appar-
ent in the very young, as the peripheral white matter and
cortical medullary venous territory matures later in gestation.
Diffuse leptomeningeal disease may also contribute to the
development of hydrocephalus in these cases.
Post-Infectious and Hemorrhagic Causes
of Obstructive Hydrocephalus
Infectious etiologies are one of the most common causes
of hydrocephalus, especially in developing countries with
bacterial or tubercular meningitis being associated with
Fig. 5 Thin sagittal T2W MR
images (a and b) demonstrate the
anatomic ETV site as the
functional analysis (flow
turbulence across the ETV defect
in the floor of the third ventricle)
(arrow) for assessing the
adequacy of the procedure
957
hydrocephalus. Tuberculous meningitis is pathologically
characterized by the occurrence of thick basilar exudates.
Most of the complications of tuberculous meningitis, in-
cluding hydrocephalus, occur because of thick copious
ex ud at es th at a re p red omi na ntly pre se nt in th e
interpeduncular, suprasellar and Sylvian cisterns.
Optochiasmatic arachnoiditis is a devastating form of tu-
berculous meningitis and often associated with profound
vision loss and hydrocephalus [16].
Diffuse edema with impaired absorption and flow of
purulent CSF contributes to early changes of hydroceph-
alus (Fig. 6). In older children with a rigid calvarium, the
resulting acute hydrocephalus with raised pressures may
present with minimally dilated ventricles. In younger in-
fants with open fontanelles and sutures, the infection may
result in early ventricular dilatation. Associated extensive
parenchymal injury secondary to cerebritis or ependymal
inflammation and abscess formation may be seen. In the
later stages, post-infectious sequelae such as obstruction
of the CSF pathways and loss of compliance, may co-
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Fig. 6 Coronal T2W (a) and axial
FLAIR (b) images show diffuse
edema with impaired absorption
and flow of the purulent CSF
(arrow) that contributes to the
early changes of hydrocephalus
exist with post-inflammatory impaired peripheral absorp-
tion with resultant hydrocephalus [4].
Intraventricular hemorrhage (IVH) is a common cause of
hydrocephalus especially in premature infants. There is sig-
nificant ventricular enlargement in more than 50% of the pa-
tients with IVH and a significant proportion of these require
CSF diversion procedures. The intraventricular blood leads to
subependymal gliosis/ fibrosis and adhesive arachnoiditis
resulting in ventricular distension.
Compensated Obstructive Hydrocephalus
Compensated hydrocephalus with loss of compliance and ef-
fective ventricular absorption can be seen associated with
common conditions like aqueduct stenosis. Other etiologies
including arachnoid, suprasellar or quadrigeminal cistern
cysts, tectal plate tumors, Chiari malformation and posterior
fossa cystic malformations can also be associated with com-
pensated hydrocephalus.
Communicating Hydrocephalus
(Non-Obstructive)
The exact mechanism of communicating non-obstructive hy-
drocephalus is unclear and the underlying mechanisms of
pathogenesis including poor absorption, decreased compli-
ance and unrecognized obstruction are difficult to ascertain
in individual cases (Fig. 7).
However, two conditions observed in pediatric com-
municating non-obstructive hydrocephalus, including be-
nign idiopathic external hydrocephalus and choroid plex-
us papilloma associated hydrocephalus, are widely attrib-
uted to disturbances in CSF secretion-absorption. Benign
idiopathic external hydrocephalus (benign enlargement of
Indian J Pediatr (October 2019) 86(10):952–960
the subarachnoid spaces in infancy) is usually seen before
two years of age and is characterized by macrocephaly,
enlarged subarachnoid spaces, including the anterior in-
terhemispheric fissure, and mild prominence of lateral
ventricles (Fig. 8). Imaging studies like ultrasound or
MRI are useful in these infants to rule out other causes
of macrocephaly such as hydrocephalus, subdural or
extradural collections, and space-occupying lesions in
the brain. Benign enlargement of subarachnoid spaces
can be differentiated from a subdural hygroma on MRI
by the demonstration of bridging veins traversing the flu-
id spaces in cases of benign enlargement of the spaces
and can also be further differentiated from parenchymal
atrophy by the ‘head circumference’ tool which may be
more than the 95th percentile on regular follow-up clin-
ical examination in cases of benign enlargement [17].
Choroid plexus papilloma-associated hydrocephalus is
believed to result from an imbalance between secretion
and absorption [4].
Though the exact pathophysiology of brain injury in
hydrocephalus is not well understood, there are neuro-
pathological changes especially within the white matter
that are thought to be contributory. Injury and vulnerabil-
ity of the developing brain results in impaired cerebral
blood flow and ischemia. It also leads to axonal and my-
elin damage, resulting in impaired conduction and paren-
chymal loss. The ependyma may be damaged with
subependymal proliferative gliosis.
In early-onset hydrocephalus, abnormal migration and ab-
normal gyration have been shown to occur.
The main factors affecting outcome and prognosis for
hydrocephalus, in addition to the intracranial pressure,
are, duration, severity and age at the time of insult.
Severity and duration of hydrocephalus influence paren-
chymal changes including chronic ischemia, and
ependymal and white matter injury. The age at the time
Indian J Pediatr (October 2019) 86(10):952–960
Fig. 7 Case of communicating
hydrocephalus (non-obstructive)
with presence of dilated lateral
ventricles (arrows) on sagittal
T2W (a) and coronal T2W
(b) MRI
of insult modifies the impact of the causal disease and
extent of the plasticity of the developing brain.
Fetal Hydrocephalus
Fetal hydrocephalus is a complex entity yet not completely
understood and distinct from postnatal hydrocephalus as
discussed earlier in the chapter whereas postnatal hydroceph-
alus is presumed as a disorder of the secretion-absorption bal-
ance of the CSF, the pathophysiological aspects of the CSF
dynamics in the fetus are still largely unknown [18]. The fluid
is present within the lumen of the neural tube before the for-
mation of the choroid plexuses. A choroid plexus CSF secre-
tion presumably is present only after wk 7–8 when AQP1
become expressed. The fetal circulation pattern is associated
Fig. 8 Axial contrast enhanced CT image with diagnosis of ‘benign
enlargement of the subarachnoid spaces in infancy’ showing features of
macrocephaly, enlarged subarachnoid spaces (arrows), including the
anterior interhemispheric fissure (block arrow), and mild prominence of
lateral ventricles
959
with both a low arterial pressure and a high venous pressure,
the arterio-venous gradient necessarily is small throughout the
gestation. There are no definitive established morphologic
criteria yet for fetal hydrocephalus.
The fetal ventriculomegaly is currently accepted with
the atrium of the lateral ventricle larger than 10 mm; the
presence of a fetal ventriculomegaly suggests that brain
development may be abnormal and is perhaps one of the
most common reasons to do a fetal MR scan [19]. In au-
thors’ experience with fetal ventriculomegaly, a relative
increase of the parietal diameter of two gestational weeks,
and the presence of a causal lesion would suggest obstruc-
tive fetal hydrocephalus. The ventricular morphology in
obstructive fetal hydrocephalus presents with typical
rounded configuration of the ventricles, effaced
pericerebral spaces, preserved layering of the cerebral
mantle (ventricular zone, intermediate zone, sub plate, cor-
tex) (unlike cases of destructive ventriculomegaly) and dis-
ruption of septum pellucidum (Fig. 9). The partial dehis-
cence of the mantle seems to be a feature of the severe fetal
hydrocephalus. Ependymal denudation is a characteristic
feature in fetal hydrocephalus, which affects the progenitor
cells of the sub ventricular zone; believed to result in the
development of sub ependymal nodules/heterotopia [20].
The recent new understanding of role of water channels
aquaporin’s, provides new insight and perspective on the com-
plex pathophysiology of the hydrocephalus caused by a myr-
iad of etiologies. The arterial pulsating force is believed to be
significant factor in CSF flow and transport. It is now well
understood that hydrocephalus does not result simply from the
interruption of a global bulk flow. Depending on the etiolo-
gies, hydrocephalus may result from a loss of compliance,
from impaired absorption, or from a combination of the two.
These may also affect diversely the different processes of re-
pair. This must be taken into consideration when hydroceph-
alus is assessed in an individual patient, as it may affect the
therapeutic approach. Current treatment options are limited
and invasive: cerebrospinal fluid (CSF) diversion via shunt
catheter or endoscopic third ventriculostomy [21].
960
Fig. 9 Sagittal (a) and axial T2W
(b) MR images in obstructive
fetal hydrocephalus showing the
typical rounded configuration of
the ventricles (arrows), effaced
pericerebral spaces, preserved
layering of the cerebral mantle
and disruption of septum
pellucidum
Summary
Neuroimaging techniques in pediatric hydrocephalus have
evolved with newer methods of treatment. Currently, routine
CT and MRI imaging studies continue to be used to assess and
follow-up. Advanced MRI and ultrasound techniques will
continue to provide more comprehensive tools for the diagno-
sis, treatment, and prognosis of pediatric hydrocephalus. For
example, newer techniques including DTI and elastography
are increasingly used to evaluate microstructural white matter
abnormalities related to hydrocephalus and predictive out-
comes in pediatric hydrocephalus [4]. Further research to im-
prove our understanding of the causal mechanisms and path-
ophysiology is necessary to optimize treatment and outcomes.
Authors’ Contribution PK: Manuscript revision and providing images;
CR: Concept, providing images and editing the manuscript; SP:
Providing images and references; MS: Concept editing and revision of
the manuscript. MS is the guarantor for this paper.
Compliance with Ethical Standards
Conflict of Interest None.
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