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Surgical treatment for kyphoscoliosis in Cohen syndrome.
[cohen syndrome]
Cohen
syndrome
is
a
very
rare
disease
.
Complication
by
spinal
deformity
has
been
reported
,
but
management
and
surgery
for
spinal
deformity
in
Cohen
syndrome
has
not
been
previously
described
.
The
objective
of
this
study
was
to
examine
the
outcome
of
surgical
treatment
for
kyphoscoliosis
of
Cohen
syndrome
with
a
literature
review
.
The
patient
was
a
14
-
year
-old
male
with
the
characteristics
of
Cohen
syndrome
:
truncal
obesity
,
mental
retardation
,
arachnodactyly
,
microcephalia
,
and
a
facial
malformation
.
Scoliosis
was
conservatively
treated
with
a
brace
at
13
years
of
age
,
but
the
spinal
deformity
rapidly
progressed
within
a
year
.
Plain
radiographs
before
surgery
showed
scoliosis
of
47
degrees
(
T
5
-
T
11
)
and
79
degrees
(
T
11
-
L
3
)
,
and
kyphosis
of
86
degrees
(
T
7
-
L
1
)
.
One
-
stage
anteroposterior
corrective
fusion
of
T
4
-
L
3
was
scheduled
after
2
-
week
Halo
traction
.
Postoperative
respiratory
management
was
carefully
performed
because
of
Cohen
syndrome
-associated
facial
malformation
,
obesity
,
and
reduced
muscle
tonus
.
Respiration
was
managed
with
intubation
until
the
following
day
and
no
respiratory
problems
occurred
.
After
surgery
,
thoracolumbar
scoliosis
was
28
degrees
(
correction
rate
:
65
%
)
.
Kyphosis
was
markedly
improved
from
86
degrees
to
20
degrees
,
achieving
a
favorable
balance
of
the
trunk
.
The
outcome
is
favorable
at
6
.
5
years
after
surgery
.
In
conclusion
,
Cohen
syndrome
is
often
complicated
by
spinal
deformity
,
particularly
kyphosis
,
that
is
likely
to
progress
even
in
adulthood
.
In
our
patient
,
spinal
deformity
progressed
within
a
short
period
,
even
with
brace
treatment
.
Surgery
should
be
required
before
progression
to
the
severe
spinal
deformity
with
careful
attention
to
general
anesthesia
.
Diseases
Validation
Diseases presenting
"cohen syndrome-associated facial malformation"
symptom
cohen syndrome
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