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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
"dysphagia"
symptom
alexander disease
cadasil
cushing syndrome
dedifferentiated liposarcoma
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
familial hypocalciuric hypercalcemia
inclusion body myositis
kindler syndrome
liposarcoma
locked-in syndrome
neuralgic amyotrophy
oligodontia
triple a syndrome
well-differentiated liposarcoma
This symptom has already been validated
