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Focal cerebral mantle disruption in fetal hydrocephalus.
[hydrocephalus with stenosis of the aqueduct of sylvius]
A
variety
of
developmental
brain
anomalies
have
been
described
in
individuals
with
fetal
hydrocephalus
,
regardless
of
etiology
.
Examples
include
callosal
dysgenesis
,
periventricular
gray
matter
heterotopia
,
hippocampal
and
white
matter
hypoplasia
,
and
cortical
polygyration
.
The
present
report
draws
attention
to
another
anomaly
not
reported
in
previous
case
series
of
fetal
hydrocephalus
:
focal
cerebral
mantle
disruption
.
Neonatal
imaging
findings
(
where
available
)
and
post-shunt
,
stable-
state
magnetic
resonance
imaging
,
or
pathological
findings
were
reviewed
in
77
subjects
with
fetal
hydrocephalus
(
55
myelomeningocele
,
16
sporadic
aqueductal
stenosis
,
6
miscellaneous
)
.
Of
these
,
12
subjects
(
15
.
6
%
)
demonstrated
a
combination
of
absence
of
the
septum
pellucidum
and
severe
thinning
or
absence
of
the
posteromesial
cerebral
mantle
.
On
axial
sequences
,
this
combination
created
the
illusion
of
a
common
ventricle
,
as
in
lobar
holoprosencephaly
.
All
12
subjects
had
massive
hydrocephalus
at
birth
,
accompanied
in
7
by
posteromesial
ventricular
diverticula
.
Two
subjects
,
and
one
other
subject
with
distinct
lateral
ventricles
,
demonstrated
unilateral
or
bilateral
mantle
clefts
suggestive
of
schizencephaly
.
Close
radiological
(
n
=
2
)
or
pathological
(
n
=
1
)
inspection
showed
that
the
clefts
were
only
partially
lined
with
gray
matter
and
contained
a
transverse
gliotic
membrane
.
These
findings
are
consistent
with
the
hypothesis
that
massive
early
fetal
hydrocephalus
may
completely
disrupt
cerebral
mantle
formation
,
particularly
in
the
posteromesial
hemispheres
.
Diseases
Validation
Diseases presenting
"severe thinning or absence of the posteromesial cerebral mantle"
symptom
hydrocephalus with stenosis of the aqueduct of sylvius
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