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Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
We
investigated
small
-
and
large
-
bowel
specimens
of
three
newborn
infants
presenting
with
the
clinical
and
radiological
symptoms
of
megacystis-
microcolon
-
intestinal
hypoperistalsis
syndrome
(
MMIHS
)
.
Conventional
histological
staining
revealed
marked
thinning
of
the
longitudinal
muscle
layer
.
Electron-microscopic
investigations
showed
typical
"
central
core
"
vacuolic
degeneration
of
smooth-
muscle
-cells
combined
with
proliferation
of
col
lagen
fibres
.
The
expression
of
alpha-smooth-
muscle
actin
was
absent
or
markedly
reduced
in
the
circular
and
longitudinal
muscle
layers
and
muscularis
mucosae
compared
to
the
normal
controls
.
These
findings
suggest
that
the
intestinal
obstruction
in
MMIHS
is
due
to
an
abnormality
of
the
smooth-
muscle
cells
.
