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Neuroependymal denudation is in progress in full-term human foetal spina bifida aperta.
[hydrocephalus with stenosis of the aqueduct of sylvius]
In
human
spina
bifida
aperta
(
SBA
)
,
cerebral
pathogenesis
[
hydrocephalus
,
Sylvius
aqueduct
(
SA
)
stenosis
and
heterotopias
]
is
poorly
understood
.
In
animal
models
,
loss
of
ventricular
lining
(
ependymal
denudation
)
causes
SA
stenosis
and
hydrocephalus
.
We
aimed
to
investigate
whether
ependymal
denudation
also
takes
place
in
human
foetal
SBA
.
Considering
that
ependymal
denudation
would
be
related
to
alterations
in
junction
proteins
,
sections
through
SA
of
five
SBA
and
six
control
foetuses
(
gestational
ages
ranged
between
37
and
40
weeks
)
were
immunostained
for
markers
of
ependyma
(
caveolin
1
,
βIV-tubulin
,
S
100
)
,
junction
proteins
(
N-
cadherin
,
connexin-
43
,
neural
cell
adhesion
molecule
(
NCAM
)
,
blood
vessels
(
Glut-
1
)
and
astrocytes
[
glial
fibrillary
acidic
protein
(
GFAP
)
]
.
In
control
foetuses
,
ependymal
denudation
was
absent
.
In
SBA
foetuses
different
stages
of
ependymal
denudation
were
observed
:
(
i
)
intact
ependyma
/
neuroepithelium
;
(
ii
)
imminent
ependymal
denudation
(
with
abnormal
subcellular
location
of
junction
proteins
)
;
(
iii
)
ependymal
denudation
(
with
protrusion
of
neuropile
into
SA
,
formation
of
rosettes
and
macrophage
invasion
)
;
(
iv
)
astroglial
reaction
.
It
is
suggested
that
abnormalities
in
the
formation
of
gap
and
adherent
junctions
result
in
defective
ependymal
coupling
,
desynchronized
ciliary
beating
and
ependymal
denudation
,
leading
to
hydrocephalus
.
The
presence
of
various
stages
of
ependymal
denudation
within
the
same
full-term
SBA
foetuses
suggests
continuation
of
the
process
after
birth
.
Diseases
Validation
Diseases presenting
"ependymal denudation"
symptom
hydrocephalus with stenosis of the aqueduct of sylvius
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