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Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis-
microcolon
-
intestinal
hypoperistalsis
syndrome
(
MMIHS
)
is
the
most
severe
form
of
functional
intestinal
obstruction
in
the
newborn
.
To
date
,
multivisceral
transplantation
has
been
the
only
accepted
treatment
modality
for
these
patients
,
and
the
results
have
met
with
marginal
success
.
We
report
the
first
case
of
a
patient
affected
by
MMIHS
and
cholestatic
liver
failure
treated
by
a
combined
living-related
liver
and
intestinal
transplant
(
CLRLITx
)
.
The
patient
was
a
1
-
year
-old
Hispanic
girl
born
with
MMIHS
and
maintained
on
total
parenteral
nutrition
since
birth
.
Once
liver
failure
developed
,
she
was
referred
for
evaluation
for
possible
CLRLITx
.
The
patient
's
mother
volunteered
as
the
donor
.
The
left
lateral
segment
was
used
for
the
liver
transplant
.
The
intestinal
graft
consisted
of
the
terminal
180
cm
of
the
ileum
with
a
single
vascular
pedicle
.
Initially
,
the
patient
continued
to
have
severe
gastroparesis
;
however
,
by
8
months
posttransplant
,
stomach
function
had
returned
to
normal
.
Currently
,
at
2
years
posttransplant
,
she
is
tolerating
an
oral
diet
with
gastric
tube
supplementation
.
Results
of
absorption
studies
are
within
normal
,
and
she
has
shown
catch-up
growth
.
A
CLRLITx
can
be
a
viable
alternative
for
infants
diagnosed
with
MMIHS
.
This
procedure
can
help
avoid
the
25
%
wait-list
mortality
for
children
who
are
in
need
of
a
combined
transplant
.
Diseases
Validation
Diseases presenting
"first case of a patient"
symptom
carcinoma of the gallbladder
kabuki syndrome
megacystis-microcolon-intestinal hypoperistalsis syndrome
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