Ultrasound Obstet Gynecol 2015; 46: 111

Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/uog.14687

Editorial
Etiological diagnostic tools to elucidate
isolated ventriculomegaly
L. GUIBAUD* and A. LACALM
Departement dImagerie Pediatrique et Ftale, Centre
Pluridisciplinaire de Diagnostic Prenatal, Hopital Femme M`ere
Enfant, Lyon-Bron, France
*Correspondence. Universite Claude Bernard Lyon I, Imagerie
Pediatrique et Ftale, Hopital
Femme M`ere Enfant, 59, Boulevard

Pinel, 69677 Lyon-Bron, France

(e-mail: [email protected])

infections, cerebral vascular insults or other fetal

cerebral (and extracerebral) anomalies6 . To overcome this
difficulty, we have constructed a systematic approach for
patients referred to our institution due to a diagnosis of
isolated or unexplained ventriculomegaly, as we present
in this Editorial. Our approach is based on a set of
etiological diagnostic tools and its application should
always be guided by the clinical context. These tools
were initially developed for ultrasound examination but
can also be used for MRI analysis, the latter often

Introduction
Ventriculomegaly is the most common abnormal finding
that leads to a diagnosis of fetal central nervous system
(CNS) pathology and is the most common indication
for second-level neurosonography and fetal magnetic
resonance imaging (MRI)1 4 . Determination of its
severity is based on strict criteria derived from the width
of the atrium of the lateral ventricle5 : ventriculomegaly is
considered to be mild when the atrial width is 1012 mm,
moderate when it is 1215 mm and severe when it
is > 15 mm1,6,7 .
The finding of ventriculomegaly should prompt careful
analysis of the fetal brain to investigate systematically
the possibility of malformative, clastic, tumoral and
syndromic pathologies7 9 . When no cause is found,
ventriculomegaly is referred to as isolated, indicating that
both the underlying pathophysiology and the etiology are
undetermined. This represents, by definition, a provisional
diagnosis of exclusion6 . As stated by Melchiorre et al.6 ,
counseling in such a setting is particularly difficult, as
isolated ventriculomegaly can be a benign finding, but
it can also be associated with aneuploidies, congenital

d = d

Figure 1 Standardization of fetal cerebral ventricular measurement:

the five criteria of our image-scoring method5 (Table 1) illustrated
in a normal fetus at 22 gestational weeks. Criterion 1: midline (thin
dot-dash line) is perpendicular to ultrasound beam and equidistant
from calvarial margins (i.e. d = d ; double-headed long arrows).
Criterion 2: anterior (cavum septi pellucidi, dotted circle) and
posterior (V shape of ambient cistern, dashed line) anatomical
landmarks are clearly identified. Criterion 3: measurement is
performed opposite deepest part of internal parieto-occipital sulcus
(small arrow). Caliper placement and image magnification are
performed according to Criteria 4 and 5, respectively.

Table 1 Criteria to establish the presence of ventriculomegaly: image-scoring method (from Guibaud5 )
Criterion

Score

Anatomical/technical requirements

Primary criteria
1. Strict axial plane

02

1a. Midline structures equidistant from proximal and distal calvarial

margins
1b. Midline perpendicular to ultrasound beam
Anterior landmark: cavum septi pellucidi or fornix columns
Posterior landmark: fluid-filled triangular V-shape of the ambient cistern
Measurement performed opposite the internal parieto-occipital sulcus

2. Adequate anatomical level

01

3. Location of the atrium

01

Secondary criteria
4. Caliper placement

5. Adequate image size

02

01

4a. Measurement perpendicular to inner and outer borders of the ventricle

4b. On to on measurement (junction of ventricular lumen and
ventricular wall)
Axial transventricular plane occupying the whole screen with visualization
of both proximal and distal calvarial margins

Maximum total score is 7. Criteria 15 are illustrated in Figure 1.

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

EDITORIAL

Guibaud and Lacalm

Figure 2 Patient referred at 32 gestational weeks due to unexplained mild ventriculomegaly. Axial (a,b) and sagittal (c) images confirming
mild ventriculomegaly associated with normal cephalic biometry and demonstrating a fluid-filled cystic anatomical structure ( ) which was
connected directly to the lumen of the posterior and superior borders of the third ventricle, and which was identified as dilatation of the
suprapineal recess. Three weeks after referral, follow-up examination demonstrated, on axial (d,e) and sagittal (f ) sonography and axial and
sagittal T2-weighted magnetic resonance (MRI) (g,h) imaging, a major increase of ventriculomegaly involving both lateral and third
ventricles and associated with increased cephalic biometry and reduction of the pericerebral spaces. The aqueduct was not identified
(arrows), suggesting aqueductal stenosis, as shown on midsagittal T2-weighted fetal MRI (h). The diagnosis was confirmed on midsagittal
T2-weighted postnatal MRI (i) (arrow).

being included in the imaging work-up of undetermined

ventriculomegaly, especially for moderate and severe
forms.
Prior to using this etiological approach, the most
important issue, when faced with a finding of isolated
mild ventriculomegaly, is to establish whether or not
ventriculomegaly is in fact present. As discussed in our
article on standardization of fetal cerebral ventricular
measurement, a large number of fetuses referred to
our department due to mild or moderate isolated
ventriculomegaly in fact have normal ventricular measurements when these are performed using strict criteria5 .
We have proposed an image-scoring method using five
criteria (three primary and two secondary) (Table 1). The

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

primary criteria identify, based on anatomical landmarks,

a reference image for measurement of atrial width,
ensuring a strict axial plane, an appropriate anatomical
level and location of the atrium using the landmark
of the internal parieto-occipital sulcus (Figure 1). The
secondary criteria ensure high quality of measurement by
appropriate caliper placement on an optimally magnified
image5 . This standardization is important, since many
patients can be reassured if, when these strict criteria
are applied to its measurement, the atrial width is
below the threshold of 10 mm5,10 . If ventriculomegaly
is confirmed, our set of etiological diagnostic tools
may be helpful. Note that several tools can be used in
combination.

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Editorial

Etiological diagnostic tools for complete analysis of the

ventricular system following identification
of ventriculomegaly
Investigation of obstructive pattern
An obstructive pattern refers to ventriculomegaly related
to an obstruction, regardless of the cause of the obstruction (e.g. malformative, tumoral, vascular origin)1,7,11 . In
some cases, the origin of the obstruction is obvious, in
other cases the etiology of the ventriculomegaly can be
overlooked, underlining the importance of recognizing an
obstructive pattern as the pathophysiological mechanism
of the ventricular dilatation. This pattern includes four
parameters: increased cephalic biometry (head circumference), partial or complete (barotraumatic) destruction of
the cavum septi pellucidi (CSP), decrease or absence of
pericerebral spaces and, in some cases, if the obstruction
is downstream of the third ventricle, presence of a dilated
suprapineal recess (SPR)12 . In the case of long-term and
major obstruction of cerebrospinal fluid (CSF) flow, lamination of the cerebral parenchyma is also associated with
severe ventriculomegaly and is therefore an additional
finding suggestive of an obstructive pattern. One should
note that this pattern can be incomplete in three different settings: in cases of early obstruction (in the first
trimester), in cases with partial obstruction and at onset of
the obstructive process12 . Indeed, normal cephalic biometry, or even decreased biometry, may be encountered
in early obstructive ventriculomegaly in the first or early
second trimester, since an early increase in pressure in the
ventricular system may cause reduced production of the
progenitor cells in the periventricular germinal matrix,
leading to a slowing-down of cephalic growth13 15 .
Moreover, both decrease in size of pericerebral spaces
and barotraumatic destruction of the leaflets of the CSP
require a significant increase of pressure in the ventricular
system, accounting for the fact that these findings may be
absent in the early stages of the obstructive process.
In contrast, we have shown that identification of
a dilated SPR may suggest a diagnosis of obstructive
ventriculomegaly prenatally, even if the ventriculomegaly
is mild and the obstructive pattern is absent at the time of
initial examination (Figure 2)12 . This recess was initially
termed pressure diverticulum of the third ventricle16 .
Such dilatation of the SPR should therefore prompt
careful imaging and clinical follow-up, particularly
for ventriculomegaly of undetermined origin in which
obstruction is located downstream of the third ventricle12 .
Identifying an obstructive pattern is important for
prenatal counseling, since in such cases the severity of
prognosis is related more to the cause of obstruction than
to the severity of ventriculomegaly.
Investigation of vascular clastic pattern when facing
unilateral/asymmetrical ventriculomegaly
Special attention should be paid to unilateral and
asymmetrical ventriculomegaly; these should prompt

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

Figure 3 Patient referred at 31 gestational weeks for asymmetrical

ventriculomegaly associated with cephalic biometry > 95th centile.
Axial (a,b) and parasagittal (c) sonographic images confirming
asymmetrical ventriculomegaly associated with increased
echogenicity of the ventricular wall, echogenic material in the
ventricular lumen and frontal horn (clot), highly suggestive of a
clastic pattern related to ischemohemorrhagic insult. Note, on the
parasagittal image (c), irregular ventricular borders suggestive of
extension of the ischemohemorrhagic process to the periventricular
parenchyma, resulting in liquefaction and opening in the
ventricular lumen.

Ultrasound Obstet Gynecol 2015; 46: 111.

Guibaud and Lacalm

Figure 4 Patient referred at 27 gestational weeks for unilateral ventriculomegaly. Oblique (towards the proximal hemisphere) sonographic
image (a) showing unilateral ventriculomegaly, located on the hemisphere close to the transducer, associated with multiple echogenic foci
suggestive of small clots (arrows) in the ventricular lumen lying on the ventricular wall. Axial fetal magnetic resonance imaging confirmed
unilateral ventriculomegaly (b) associated with hyposignal ( ) (c) and absence of signal ( ) (d) on T2- and T2*-weighted images, respectively,
which confirmed subependymal hemorrhage.

consideration of an underlying clastic vascular pathophysiological mechanism and lead to an investigation for
a clastic pattern suggestive of an underlying ischemohemorrhagic insult. This pattern can be complete or
incomplete. In its complete form, the vascular clastic pattern includes asymmetrical ventriculomegaly, increased
echogenicity of the ventricular wall and echogenic deposits
within the ventricular lumen related to intraventricular
hemorrhage1 . When ischemohemorrhagic insult extends
to the parenchyma, the borders of the ventricle can be
irregular, related to focal liquefaction and destruction of
the periventricular parenchyma (Figure 3).
In routine practice, ischemohemorrhagic insult is
strongly suggested when associated with a typical
clastic pattern, but it may be overlooked in cases of
long-term and pure subependymal hemorrhage, requiring
systematic use of T2* gradient echo MR sequences

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

during imaging work-up of isolated ventriculomegaly,

especially if the dilatation is asymmetrical or unilateral
(Figure 4)1,17 .
Unilateral ventriculomegaly should not be considered
to be specific to clastic lesions, since it can be encountered
also in association with malformative entities such as
hemimegalencephaly, which is characterized by both
unilateral ventriculomegaly and dysmorphism of the
dilated ventricle (Figure S1)18,19 , and exceptional cases
of congenital stenosis of the foramen of Monroe
(Figure S2)20 .
CSF flow should be followed from the diencephalon
to the posterior fossa
Sonographic work-up of isolated ventriculomegaly
should include a complete analysis of the ventricular

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Editorial

Figure 5 Patient referred at 23 gestational weeks for severe ventriculomegaly. Axial sonographic images (ac) showing fusion of the thalami
( ) associated with dilatation of the proximal part of the third ventricle (diencephalosynapsis) (b) in combination with absence of
identification of the aqueduct and cerebellar hypoplasia (c). Absence of the cavum septi pellucidi, presence of cerebral parenchymal
lamination and reduction of the pericerebral spaces were highly suggestive of an obstructive pattern (a). Microscopic examination confirmed
thalamic fusion ( ) (d).

system, from the supratentorial structures to the infracerebellar cisterna. Pathologies generating ventriculomegaly
between the diencephalon and posterior fossa, including
the aqueduct of Sylvius at the level of the mesencephalon,
are often overlooked21 . Scrutinization of the third ventricle, the thalami, the aqueduct and the posterior fossa
is crucial, especially in the case of an obstructive pattern associated with severe ventriculomegaly in early
second trimester. In such a setting, partial atresia of the
third ventricle with upstream dilatation of the ventricular system associated with partial fusion of the thalami,
leads to a diagnosis of diencephalosynapsis (Figure 5)21 .
Such obstructive pathology may occur in combination
with stenosis of the aqueduct of Sylvius. Aqueductal
stenosis can be either complete, as in the case of atresia, or partial. The degree of obstruction accounts for
early or late presentation of pathology during pregnancy.
Therefore, we emphasize the importance of carefully

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

examining the lumen of the aqueduct, which is identified clearly in an oblique axial plane in the normal
fetus, but is not seen in cases of complete stenosis
or in partial tapering stenosis associated with upward
dilatation of the proximal part of the aqueduct and
absence of its distal part. Both diencephalosynapsis and
aqueductal stenosis can be isolated, but they can also
be combined with posterior fossa anomalies, including cerebellar hypoplasia and rhombencephalosynapsis
(Figure 5)21 .
Sylvian fissure as the main landmark of gyration
Gyration should be scrutinized, particularly when facing
unexplained ventriculomegaly. The main landmark of
the gyration process is Sylvian fissure operculization;
this should be integrated systematically in the imaging work-up of the fetal brain22 25 . This important

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Guibaud and Lacalm

20 weeks
(open angle)

22

(45 angle)
24

(90 angle)

26

Temporal lobe covers of the

posterior insula
28

Temporal lobe covers of the

posterior insula
32

Temporal lobe completely covers the

posterior insula

Figure 6 Fetal Sylvian fissure development. (Adapted from

Guibaud et al.27 )

diagnostic etiological tool requires knowledge regarding

the shape of the Sylvian fissure in the axial plane, according to gestational age. We previously published a reliable
subjective method with which to assess the shape of the
Sylvian fissure between 22 and 32 weeks of gestation
using a simple score-based evaluation of operculization of
the posterior part of the Sylvian fissure on a standardized
view in an axial cerebral plane (Figure 6)25 . Using this
method in routine prenatal imaging can help in the
early diagnosis of anomalies of operculization, reflecting
underlying cortical dysplasia such as polymicrogyria
(Figure 7) or lissencephaly (Figure S3)24,26,27 . Moreover,
we have shown that anomalies of operculization can
also be related to extracortical factors such as abnormal
cerebral volume; examples include frontal hypoplasia
and other developmental anomalies of the CNS27 .
Ventricular wall, ventricular lumen and periventricular
area
As discussed above, identifying changes in the ventricular
wall and ventricular lumen can aid in the diagnosis of a
vascular clastic pattern related to ischemohemorrhagic

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

changes (Figures 3 and 4)1 . However, ventriculomegaly

can also be associated with another clastic pattern
related to infectious fetopathy, cytomegalovirus infection
in particular. This infectious clastic pattern includes
periventricular echogenic halo in the subependymal zone,
which may be associated with germinolysis cysts, periventricular or parenchymal calcification and pathognomonic
cystic changes posterior to the occipital horn or anterior to
the temporal horn (Figures 8 and S4)28 30 . This infectious
clastic pattern can also be associated with an abnormal
Sylvian fissure, suggestive of polymicrogyria. One should
note that cytomegalovirus infection is, in the vast majority
of cases, associated with minor or moderate ventriculomegaly, which can easily be considered as isolated
when the infectious clastic pattern is overlooked29 .
Systematic investigation of the ventricular wall may suggest diagnosis of subependymal heterotopia, presenting as
nodular irregularities of the ventricular wall, which can
be either isolated or combined with more obvious cerebral
anomalies1 . Isolated subependymal heterotopia is very
often associated with mild or moderate ventriculomegaly,
which can be symmetrical, asymmetrical or unilateral
(Figure 9). An irregular square-shaped appearance of the
lateral ventricle has been reported as a finding suggestive
of subependymal heterotopia31 .
Analysis of the periventricular zone is useful not only to
diagnose periventricular halo related to cytomegalovirus
infection, but also to depict periventricular changes related
to neuronal migration anomalies, such as in lissencephaly
(Figure S3).
It should be noted that changes involving the ventricular wall, ventricular lumen and periventricular area
of the proximal hemisphere close to the transducer are
overlooked on axial sonographic examination, since the
proximal hemisphere is obscured by bony artifacts of
the proximal vault. In such cases, it can be useful to use
an oblique view, by tilting the transducer from axial to
oblique plane, in order to explore the entire lateral ventricle of the proximal hemisphere (Figure 10). Although this
plane may be of help when investigating ventricular and
periventricular changes of the proximal ventricle, it should
be emphasized that the use of this plane is inadequate
for proximal ventricular atrial measurement and may
generate a false-positive diagnosis of ventriculomegaly
when used inappropriately for ventricular measurement.
Systematic analysis of the pericerebral spaces
Pericerebral spaces, anatomical structures that are largely
overlooked, should in fact be included systematically
in the imaging analysis. Unlike with MRI, analysis of
the pericerebral spaces with ultrasound is limited to the
hemisphere distal to the transducer, particularly to those
spaces facing the Sylvian fissure. Even so, such analysis is
mandatory when facing undetermined ventriculomegaly
and/or biometric changes (micro or macrocephaly).
Ventriculomegaly combined with macrocephaly can be
associated either with an obstructive pattern, in which
pericerebral spaces are often reduced (as illustrated

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Editorial

Figure 7 Patient referred at 28 gestational weeks for unexplained mild ventriculomegaly. Retrospective analysis of gyration on the axial
plane, from routine sonographic examination performed at 23 weeks, demonstrated abnormal, wide-open Sylvian fissure (for comparison,
refer to Figure 6). Axial sonography (b) and axial T2-weighted fetal magnetic resonance imaging (c,d) at 28 weeks confirmed abnormal
operculization and showed abnormal undulated cortical ribbon, suggestive of polymicrogyria, which was confirmed on pathological
examination.

in Figures 2d, e and g), or with benign familial

macrocephaly (Figure S5), in which pericerebral spaces
are increased32,33 . In contrast, ventriculomegaly combined
with microcephaly can be associated with a clastic pattern,
especially in cases of cytomegalovirus infection, in which
the pericerebral spaces may be significantly increased, due
to encephaloclastic tropism of the cytomegalovirus.
One should note that, even though there is no norm for
pericerebral space measurement, subjective evaluation of
the pericerebral spaces can be of great help in elucidating
isolated ventriculomegaly.
Clinical context
Last but not least, the clinical context should always
be kept in mind when facing isolated ventriculomegaly.

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

Clinical data, such as consanguinity, unexplained fever

during pregnancy, risk factors for vascular clastic
lesions (e.g. drug use, alcohol consumption, coagulation
disorders, monochorionic twin pregnancy), cephalic
biometry of both siblings and parents (Figure S5) and
familial conditions should always be scrutinized and
integrated into the work-up.

Conclusion
In conclusion, following confirmation of the presence
of ventriculomegaly, we propose a diagnostic algorithm
based on a set of etiological diagnostic tools to
elucidate isolated or undetermined ventriculomegaly
(Figure 11). These tools can be divided into two groups,
the first focusing on anatomical structures, and the

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Guibaud and Lacalm

Figure 8 Patient referred at 28 gestational weeks for mild

unexplained asymmetrical ventriculomegaly. Axial (a) and oblique
axial (towards the proximal hemisphere) sonographic imaging
showed periventricular echogenic halo (arrows) and cystic lesion ( )
posterior to the occipital horn (b), as well as small parenchymal
calcifications (not shown), which were highly suggestive of an
infectious clastic pattern related to cytomegalovirus infection.
Biological work-up using polymerase chain reaction confirmed the
diagnosis.

second on pathophysiological mechanisms. This second

group includes the obstructive and clastic patterns,
the latter with their associated ischemohemorrhagic or
infectious origins, the patterns being recognized based
on a combination of specific sonographic morphological
and biometric parameters. One should note that these
two patterns can be incomplete but also combined
in some cases, especially in cases of hemorrhagic
parenchymal insult (clastic vascular pattern) resulting
in intraventricular hemorrhage, which can lead to CSF
obstruction (obstructive pattern).
For elucidation of the etiological origin of so-called
isolated ventriculomegaly, we believe that this systematic
approach represents an essential checklist, from initial
confirmation of ventriculomegaly to analytical focus of
specific anatomical supratentorial structures, recognition
of specific pathophysiological patterns and overall
analysis within the medical context. As a direct result

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

Figure 9 Patient referred at 32 gestational weeks for unexplained

moderate unilateral ventriculomegaly. Oblique (towards the
proximal hemisphere) sonographic image (a) showing irregular
border of the ventricular wall with small nodules protruding into
the ventricular lumen (arrows). Axial T2-weighted magnetic
resonance imaging confirmed focal dilatation of the posterior part
of the lateral ventricle as well as slightly hypointense nodules
protruding into the dilated horn (arrows), suggestive of focal
subependymal heterotopia. The diagnosis was confirmed on
postnatal imaging.

of using this approach in routine practice over recent

years, as illustrated by some cases in this article, the
number of cases of apparently isolated or unexplained
ventriculomegaly encountered in our department has
decreased dramatically.

ACKNOWLEDGMENTS
We would like to thank all the people who contributed
in helping us to develop fetal neuroimaging in our fetal
medicine department in Lyon over the last 20 years. This
article is also dedicated to Dr Pierre Droulle who was a
true pioneer in prenatal ultrasound and was our mentor
in performing neurosonography.

Ultrasound Obstet Gynecol 2015; 46: 111.

Editorial

(b)

Distal H

Proximal H

Proximal H
Axial plane
LV

LV

Distal H
Oblique plane

Cerebellum

Figure 10 Oblique sonographic view in a normal fetus at 21 gestational weeks. This sonographic view (a), acquired in an oblique plane
towards the proximal hemisphere, as shown in the diagram (b), demonstrates clearly the wall of the proximal lateral ventricle, which is
smooth, the periventricular area, which shows the same echogenicity as the adjacent parenchyma, and the ventricular lumen, which is
homogeneously hypoechogenic. Note that this view should not be used for ventricular measurement. H, hemisphere; LV, lateral ventricle.

Confirmation of presence
of ventriculomegaly
Clinical context
(e.g. risk factors, pregnancy-related disorders, familial conditions)

Tools focusing on pathophysiological mechanisms

Tools focusing on anatomical structures

Complete analysis of ventricular system
-Lateral ventricles (symmetrical/asymmetrical VM),
3rd ventricle, aqueduct, 4th ventricle
-CSF flow should be followed, especially at level of
diencephalon, mesencephalon and rhombencephalon
Careful inspection of ventricular lumen, ventricular wall
and periventricular zone
Gyration should be scrutinized
-Systematic sonographic analysis of Sylvian fissure
Systematic analysis of pericerebral spaces

Investigation of an obstructive pattern

Includes analysis of HC, PC spaces, CSP, presence of dilated SPR
-Either complete or incomplete pattern according to timing
or severity of obstruction
Investigation of a clastic pattern of imaging findings,
highly suggestive of clastic origin related to:
Vascular ischemohemorrhagic insult
-Asymmetrical ventriculomegaly, echogenic ventricular wall,
echogenic contents within ventricular lumen, irregular
ventricular borders
-Vascular clastic pattern can be combined with an
obstructive pattern when hemorrhagic deposits reduce CSF flow
-T2* MR sequence should be used systematically to diagnose an
overlooked subtle long-term subependymal hemorrhage
Infectious insult
-Periventricular echogenic halo, germinolysis cysts, parenchymal
calcifications, cystic changes anterior to temporal horn
or posterior to occipital horn

NEGATIVE RESULTS
Including relevant biological work-up
Confirmed isolated ventriculomegaly

Figure 11 Diagnostic algorithm based on a set of etiological diagnostic tools to elucidate isolated ventriculomegaly (VM). CSF,
cerebrospinal fluid; CSP, cavum septi pellucidi; HC, head circumference; MR, magnetic resonance; PC, pericerebral; SPR, suprapineal recess.

Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd.

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10

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SUPPORTING INFORMATION ON THE INTERNET

The following supporting information may be found in the online version of this article:
Figure S1 Patient referred at 30 gestational weeks for unilateral unexplained ventriculomegaly. Axial
sonographic image (a) showing unilateral ventriculomegaly with severe dysmorphism of the dilated ventricle.
Axial T2- weighted (b,c) and T1- weighted (d) fetal magnetic resonance imaging confirmed sonographic data
but also showed abnormal unilateral cortical surface and abnormal intensity of the adjacent cerebral
parenchyma (hypointense on T2- and hyperintense on T1-weighted sequences), suggestive of underlying large
neuroglial heterotopia. Imaging findings were consistent with diagnosis of hemimegalencephaly.
Figure S2 Patient referred at 30 gestational weeks for severe unilateral isolated ventriculomegaly associated
with increased cephalic biometry (80th centile). Axial sonography (a) and axial and coronal T2-weighted fetal
magnetic resonance (MRI) (b,c) images confirming severe unilateral ventriculomegaly (24 mm) without any
other findings, which would have suggested hemorrhage, especially on T2*-weighted images (sequence not
shown). A mass effect was evident on the only identified leaflet of the cavum septi pellucidi, suggestive of
obstruction at the level of the foramen of Monroe and barotraumatic destruction of the missing leaflet.
Diagnosis of unilateral foramen of Monroe obstruction was confirmed on postnatal MRI (d).

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Ultrasound Obstet Gynecol 2015; 46: 111.

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Figure S3 Patient referred at 28 gestational weeks for moderate ventriculomegaly associated with
microcephaly. Axial sonographic image (a) showing echogenic periventricular area (arrows) and abnormal
operculization of the Sylvian fissure, as well as moderate ventriculomegaly. Coronal T2-weighted magnetic
resonance (MR) image (b) confirming suspicion of gyral anomalies, demonstrating typical eight-shaped
appearance of the brain with smooth cortical surface and layered appearance of the parenchyma (arrows)
suggestive of diffuse migration disorder, in particular lissencephaly type 1. Note that the echogenic
periventricular area on sonographic examination was related to an abnormal wave of neuronal migration, as
shown by MR examination.
Figure S4 Patient referred at 32 gestational weeks for isolated mild ventriculomegaly. Axial (a) and coronal (b)
sonographic images showing bilateral symmetrical echogenic periventricular areas, with microcysts on the
mid-lateral border of the lateral ventricles (arrows) (a), and bulging slightly into the frontal horns on coronal
imaging (b). (c) Coronal T2-weighted magnetic resonance image showing hyperintense lesions located in the
germinal matrix zone, bulging into the lateral ventricle (arrows). Biological work-up confirmed
cytomegalovirus fetopathy.
Figure S5 Patient referred at 28 gestational weeks for isolated mild ventriculomegaly associated with increased
cephalic biometry. Axial sonographic (a) and T2-weighted magnetic resonance (MR) (b) images confirming
isolated ventriculomegaly with normal cerebral organization without any clastic lesion and demonstrating
increase of the pericerebral spaces. Note that pericerebral spaces are seen more clearly on MR examination
than on sonography due to artifact of the vault on the proximal hemispheres. The familial context, with a
paternal head circumference of 64 cm, was suggestive of familial benign macrocephaly, in which the lateral
ventricles are often enlarged.

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Ultrasound Obstet Gynecol 2015; 46: 111.