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[Esophageal strictures in children with epidermolysis bullosa].
[dystrophic epidermolysis bullosa]
Esophageal
stricture
is
a
rare
but
often
severe
complication
of
recessive
dystrophic
epidermolysis
bullosa
in
children
.
The
purpose
of
the
study
was
to
review
this
digestive
complication
with
emphasis
on
diagnostic
modalities
and
therapeutic
management
.
This
was
a
retrospective
study
of
two
pediatric
cases
of
esophageal
stenosis
that
occurred
during
generalized
recessive
dystrophic
epidermolysis
bullosa
of
the
Hallopeau-
Siemens
type
.
The
2
patients
were
aged
8
years
8
months
and
11
years
5
months
,
respectively
.
Dysphagia
was
of
early
onset
,
before
the
age
of
10
years
in
both
cases
.
Esophageal
opacification
led
to
the
diagnosis
of
esophageal
stenosis
located
in
the
upper
1
/
3
of
the
esophagus
in
1
case
and
at
the
junction
between
the
middle
and
the
lower
1
/
3
of
the
esophagus
in
the
other
case
.
None
of
the
2
patients
received
medical
treatment
,
and
pneumatic
dilation
was
the
treatment
method
that
was
advocated
.
Esophageal
endoscopy
showed
the
stenosis
and
helped
guide
the
positioning
of
the
balloon
catheter
.
These
patients
underwent
2
and
3
sessions
of
dilation
,
respectively
,
at
intervals
of
2
months
and
1
year
.
Balloon
dilation
has
allowed
the
patients
to
have
a
more
comfortable
life
with
decreased
dysphagia
and
a
substantial
improvement
in
nutritional
status
.
However
,
this
improvement
was
transient
(
1
patient
had
symptomatic
recurrence
of
stenosis
after
3
years
)
,
which
shows
that
monitoring
of
the
patients
and
the
resumption
of
dilatation
sessions
may
be
necessary
.
Esophageal
strictures
in
dystrophic
epidermolysis
bullosa
of
the
Hallopeau-
Simens
type
are
severe
and
difficult
to
support
.
Pneumatic
dilatation
is
the
treatment
of
choice
for
the
fragile
esophagus
.
It
gives
satisfactory
results
and
can
be
repeated
without
significant
risk
.
Diseases
Validation
Diseases presenting
"often severe complication"
symptom
dystrophic epidermolysis bullosa
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