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[Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case of prolonged survival].
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis-
microcolon
-
intestinal
hypoperistalsis
syndrome
is
a
multisystemic
disorder
in
which
impaired
intestinal
motor
activity
causes
recurrent
symptoms
of
intestinal
obstruction
in
the
absence
of
mechanical
occlusion
,
associated
with
bladder
distention
without
distal
obstruction
of
the
urinary
tract
.
It
is
an
uncommon
disorder
with
high
morbidity
and
mortality
,
and
long
-term
total
parenteral
nutrition
may
be
required
.
We
describe
an
18
-
year
-old
girl
with
long
-term
survival
,
followed-up
by
our
service
since
birth
.
With
medical
,
surgical
and
nutritional
management
she
currently
presents
a
good
oral
tolerance
and
good
quality
of
life
,
only
requiring
parenteral
nutrition
during
periods
of
decompensation
.