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[General anesthesia in Cohen syndrome. Report of a clinical case].
[cohen syndrome]
The
case
of
a
23
years
old
woman
,
affected
by
the
Cohen
syndrome
,
who
underwent
general
anesthesia
for
extensive
dental
surgery
,
is
reported
.
The
Cohen
syndrome
is
an
autosomal
recessive
syndrome
that
causes
mental
retardation
,
obesity
,
short
stature
as
well
as
oral
,
ocular
,
and
limb
anomalies
.
The
problems
the
anesthesiologist
could
deal
with
include
the
capacity
of
the
patient
to
cooperate
;
difficult
intubation
because
of
maxillary
hypoplasia
,
micrognathia
,
narrow
and
high-arched
palate
,
and
prominent
maxillary
central
incisors
;
generalized
muscular
hypotonia
;
moderate
leukopenia
,
that
could
theoretically
increase
the
risk
of
infection
:
and
,
finally
,
possible
associated
mitral
valve
prolapse
or
hiatus
hernia
.
In
the
case
reported
the
presence
of
mitral
valve
prolapse
or
hiatus
hernia
was
ruled
out
echographically
.
The
patient
was
premedicated
with
diazepam
and
atropine
i
.
m
.
;
general
anesthesia
was
carried
out
by
propofol-fentanyl
association
and
myorelaxation
was
obtained
with
atracurium
.
Nasotracheal
intubation
was
performed
easily
in
spite
of
oral
anomalies
so
that
the
usefulness
of
thyromental
distance
,
which
was
7
cm
long
,
as
a
clinical
test
to
evaluate
a
potentially
difficult
intubation
was
confirmed
.
Noteworthy
,
the
thyromental
distance
was
the
only
test
which
was
suitable
for
the
uncooperative
patient
.
At
the
end
of
surgery
muscular
tone
recovered
promptly
and
the
endotracheal
tube
could
be
regularly
removed
.
No
complication
was
registered
postoperatively
.
Diseases
Validation
Diseases presenting
"muscular tone"
symptom
cohen syndrome
focal myositis
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