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Megacystis-microcolon-intestinal hypoperistalsis and prune belly: overlapping syndromes.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis-
microcolon
-
intestinal
hypoperistalsis
syndrome
(
MMIHS
)
is
a
rare
,
often
fatal
condition
.
Infants
present
with
a
functional
obstruction
of
the
gastrointestinal
tract
(
GI
)
,
malrotation
,
microcolon
,
and
a
large
nonobstructed
bladder
.
Several
features
common
to
both
MMIHS
and
Eagle
-
Barrett
or
prune
belly
syndrome
(
PBS
)
include
hydronephrosis
,
bladder
distension
and
laxity
of
the
abdominal
wall
musculature
.
Additionally
,
MMIHS
and
PBS
have
been
reported
in
the
same
family
,
suggesting
the
possibility
of
a
common
pathogenesis
.
MMIHS
usually
presents
in
female
infants
.
We
present
a
male
infant
diagnosed
with
both
MMIHS
and
PBS
.
This
is
a
unique
case
in
which
both
MMIHS
and
true
PBS
are
present
in
the
same
infant
.
Diseases
Validation
Diseases presenting
"bladder distension and laxity of the abdominal wall musculature"
symptom
megacystis-microcolon-intestinal hypoperistalsis syndrome
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