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The problematic Soave cuff in Hirschsprung disease: manifestations and treatment.
[hirschsprung disease]
Following
a
Soave
pull-through
for
Hirschsprung
disease
(
HD
)
,
some
children
struggle
with
obstructive
symptoms
.
We
hypothesized
that
these
symptoms
could
result
from
a
functional
obstruction
of
the
pull
through
caused
by
the
Soave
cuff
,
and
that
cuff
resection
might
improve
bowel
emptying
.
We
reviewed
patients
referred
to
our
center
from
2008
to
2012
with
obstructive
problems
following
a
Soave
pull-through
for
HD
(
CCHMC
IRB
#
2011
-
2019
)
.
Only
patients
with
an
obstructing
Soave
cuff
were
analyzed
.
Patients
with
other
reasons
for
obstruction
(
anastomotic
stricture
,
transition
zone
,
aganglionic
segment
)
were
excluded
.
Thirty
-
six
patients
underwent
reoperation
at
our
center
for
obstructive
symptoms
after
an
initial
Soave
pull-through
.
Seventeen
of
these
patients
had
a
Soave
cuff
only
as
the
potential
source
of
obstruction
.
Pre-operative
symptoms
included
enterocolitis
(
10
)
,
constipation
(
6
)
,
and
failure
to
thrive
(
1
)
.
Nine
patients
(
53
%
)
required
irrigations
to
manage
distension
or
enterocolitis
pre-operatively
.
14
/
17
patients
(
82
%
)
had
a
palpable
cuff
on
rectal
exam
.
Eight
patients
(
47
%
)
had
radiographic
evidence
of
a
cuff
demonstrated
by
distal
narrowing
(
4
)
or
a
prominent
presacral
space
(
4
)
.
Four
children
(
23
%
)
underwent
excision
of
the
cuff
only
.
Thirteen
(
76
%
)
had
removal
of
the
cuff
and
proximally
dilated
colon
[
(
average
length
7
.
2
cm
)
(
12
performed
transanally
,
and
five
needed
laparotomy
as
well
.
)
]
Post-operatively
,
episodes
of
enterocolitis
were
reduced
to
zero
,
and
need
for
irrigation
to
treat
distension
was
reduced
by
50
%
.
Nine
patients
have
voluntary
bowel
movements
,
and
five
are
clean
on
enemas
.
3
/
6
patients
with
pre-operative
constipation
or
impaction
now
empty
without
enemas
.
(
Follow
up
1
-
17
months
,
mean
7
months
.
)
Recurrent
enterocolitis
,
constipation
,
or
failure
to
thrive
can
indicate
a
functional
obstruction
due
to
a
Soave
cuff
when
no
other
pathologic
cause
exists
.
Physical
exam
or
contrast
enema
can
identify
a
problematic
cuff
.
Reoperation
with
cuff
resection
can
dramatically
improve
bowel
emptying
.
Diseases
Validation
Diseases presenting
"enterocolitis"
symptom
congenital diaphragmatic hernia
hirschsprung disease
megacystis-microcolon-intestinal hypoperistalsis syndrome
omenn syndrome
phenylketonuria
sneddon syndrome
This symptom has already been validated