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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
"mental retardation"
symptom
achondroplasia
alexander disease
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
classical phenylketonuria
coats disease
cohen syndrome
cowden syndrome
cystinuria
dentin dysplasia
familial hypocalciuric hypercalcemia
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
kallmann syndrome
lamellar ichthyosis
lymphangioleiomyomatosis
monosomy 21
phenylketonuria
primary hyperoxaluria type 1
proteus syndrome
pyruvate dehydrogenase deficiency
sneddon syndrome
triple a syndrome
wolf-hirschhorn syndrome
zellweger syndrome
This symptom has already been validated