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Histologic Identification of Prominent Intrapulmonary Anastomotic Vessels in Severe Congenital Diaphragmatic Hernia.
[congenital diaphragmatic hernia]
To
determine
whether
prominent
intrapulmonary
anastomotic
vessels
(
IPAVs
)
or
bronchopulmonary
"
shunt
"
vessels
can
be
identified
in
lungs
from
infants
with
fatal
congenital
diaphragmatic
hernia
(
CDH
)
.
We
performed
histology
with
immunostaining
for
CD
31
(
endothelium
)
and
D
2
-
40
(
lymphatics
)
,
along
with
high
-precision
3
-
dimensional
(
3
D
)
reconstruction
on
lung
tissue
from
9
patients
who
died
with
CDH
.
E
ach
patient
with
CDH
required
mechanical
ventilation
,
cardiotonic
support
,
and
pulmonary
hypertension
(
PH
)
-
targeted
drug
therapy
.
All
patients
were
diagnosed
with
severe
PH
by
echocardiography
,
and
5
received
extracorporeal
membrane
oxygenation
therapy
.
Death
occurred
at
a
median
age
of
24
days
(
range
,
10
-
150
days
)
from
refractory
hypoxemia
with
severe
PH
,
pneumonia
,
or
tension
pneumothorax
.
Histology
showed
decreased
alveolarization
with
pulmonary
vascular
disease
.
In
each
patient
,
prominent
IPAVs
were
identified
as
engorged
,
thin
-walled
vessels
that
connected
pulmonary
veins
with
microvessels
surrounding
pulmonary
arteries
and
airways
in
lungs
ipsilateral
and
contralateral
to
the
CDH
.
Prominent
anastomoses
between
pulmonary
arteries
and
bronchial
arteries
were
noted
as
well
.
The
3
D
reconstruction
studies
demonstrated
that
IPAVs
connect
pulmonary
vasculature
to
systemic
(
bronchial
)
vessels
both
at
the
arterial
and
venous
side
.
Histology
and
3
D
reconstruction
identified
prominent
bronchopulmonary
vascular
anastamoses
in
the
lungs
of
infants
who
died
with
severe
CDH
.
We
speculate
that
IPAVs
connecting
pulmonary
and
bronchial
arteries
contribute
to
refractory
hypoxemia
in
severe
CDH
.
Diseases
Validation
Diseases presenting
"airways in lungs ipsilateral and contralateral to the cdh"
symptom
congenital diaphragmatic hernia
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