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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
"failure to thrive"
symptom
22q11.2 deletion syndrome
alexander disease
child syndrome
congenital diaphragmatic hernia
cystinuria
familial hypocalciuric hypercalcemia
hirschsprung disease
homocystinuria without methylmalonic aciduria
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
krabbe disease
neonatal adrenoleukodystrophy
omenn syndrome
papillon-lefèvre syndrome
pyruvate dehydrogenase deficiency
triple a syndrome
wolf-hirschhorn syndrome
zellweger syndrome
This symptom has already been validated