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Imaging findings in megacystis-microcolon-intestinal hypoperistalsis syndrome.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis-
microcolon
-
intestinal
hypoperistalsis
syndrome
(
MMIHS
)
is
a
rare
and
frequently
lethal
form
of
severe
functional
intestinal
obstruction
more
commonly
found
in
girls
.
Imaging
features
characteristic
of
this
disease
include
a
large
dilated
bladder
,
microcolon
and
intestinal
dysmotility
.
Additional
imaging
findings
may
include
intestinal
malrotation
,
hydronephrosis
and
vesicoureteral
reflux
.
It
is
usually
fatal
in
the
first
year
of
life
.
Because
presenting
clinical
and
imaging
features
can
mimic
other
causes
of
neonatal
bowel
obstruction
,
we
compiled
examples
of
this
disorder
to
help
the
pediatric
radiologist
recognize
imaging
findings
associated
with
MMIHS
and
aid
in
the
development
of
a
long
-term
management
plan
and
in
counseling
the
family
regarding
implications
of
this
disorder
.
We
reviewed
recent
and
historical
literature
relevant
to
MMIHS
and
present
the
imaging
and
clinical
features
of
four
patients
with
MMIHS
treated
at
our
institution
as
examples
of
this
uncommon
disorder
.
Diseases
Validation
Diseases presenting
"large dilated bladder"
symptom
megacystis-microcolon-intestinal hypoperistalsis syndrome
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