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[Megacystis-microcolon-intestinal hypoperistalsis syndrome. A case report].
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
A
case
of
intestinal
pseudo-obstruction
in
the
neonatal
period
is
presented
.
It
is
known
as
Megacistis-
microcolon
-
intestinal
Hypoperistalsis
Syndrome
and
is
a
rare
cause
of
intestinal
dismotility
.
A
white
female
newborn
weighing
3
,
110
g
was
admitted
to
the
Neonatal
Surgical
Unit
of
the
Hospital
de
Base
with
a
small
omphalocele
and
a
history
of
biliary
vomit
.
Abdominal
X-
rays
revealed
complete
absence
of
intestinal
gas
.
Exploratory
laparotomy
was
carried
out
,
and
the
findings
were
great
distension
of
the
bladder
,
a
short
poorly
developed
and
malrotated
bowel
with
a
non-fixed
cecum
.
Surgical
treatment
consisted
of
gastrostomy
,
sigmoid
colostomy
,
vesicostomy
and
repair
of
the
omphalocele
.
Multiple
biopsies
were
done
in
the
small
and
large
bowel
.
Ganglion
cells
were
present
in
all
specimens
.
The
patient
did
not
tolerate
enteral
feeding
,
and
total
parenteral
nutrition
was
readily
initiated
.
Nonetheless
,
death
occurred
on
the
51
st
postoperative
day
.
This
is
a
rare
syndrome
which
presents
itself
at
birth
as
chronic
intestinal
obstruction
.
A
postmortem
examination
confirmed
the
diagnosis
.