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Restorative proctocolectomy with J-pouch ileoanal anastomosis for total colonic aganglionosis among neonates and infants.
[hirschsprung disease]
No
consensus
exists
on
the
optimal
surgical
management
of
total
colonic
aganglionosis
(
TCA
)
.
Outcomes
after
restorative
proctocolectomy
(
RPC
)
as
the
initial
reconstructive
procedure
among
neonatal
and
infant
TCA
patients
have
not
been
evaluated
previously
.
Medical
records
of
patients
with
Hirschsprung
disease
(
HD
)
who
underwent
RPC
during
infancy
between
1997
and
2012
(
n
=
8
)
were
reviewed
.
Bowel
function
and
satisfaction
with
operative
results
were
assessed
in
a
follow-up
interview
.
Median
age
at
RPC
was
1
.
1
months
,
and
covering
loop
ileostomies
were
closed
3
.
7
months
later
.
No
operative
complications
occurred
.
Hospitalizations
for
enterocolitis
and
obstruction
occurred
each
in
50
%
of
patients
postoperatively
.
Enterocolitis
-associated
outlet
obstruction
occurred
in
one
third
of
patients
,
most
of
whom
responded
well
to
intersphincteric
botulinum
toxin
(
botox
)
injections
.
No
pouchitis
or
elevated
fecal
calprotectin
levels
(
median
51
μg
/
g
)
were
observed
.
At
last
follow-up
3
.
2
years
after
ileostomy
closure
,
the
median
24
-
hour
stooling
frequency
was
3
.
5
.
None
had
socially
limiting
fecal
incontinence
or
problems
in
holding
back
defecation
.
Parent
satisfaction
with
operative
results
was
high
.
The
rate
of
postoperative
enterocolitis
was
similar
to
other
procedures
,
but
a
better
functional
outcome
was
achieved
.
Botox
injections
were
effective
for
postoperative
functional
outlet
obstruction
.
Short
-term
results
following
RPC
among
neonates
and
infants
are
promising
.