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Perioperative Risk Factors in Patients With 22q11.2 Deletion Syndrome Requiring Surgery for Velopharyngeal Dysfunction.
[22q11.2 deletion syndrome]
Objective
:
 
To
determine
the
prevalence
of
cardiac
,
cervical
spine
,
and
carotid
artery
abnormalities
in
patients
with
22
q
11
.
2
deletion
syndrome
(
22
q
11
.
2
DS
)
undergoing
surgery
for
velopharyngeal
dysfunction
(
VPD
)
,
associations
between
the
presence
of
these
abnormalities
,
and
whether
these
abnormalities
caused
changes
in
surgical
management
or
perioperative
complications
.
Design
:
 
Retrospective
review
.
Setting
:
 
Tertiary
pediatric
hospital
.
Patients
:
 
Seventy
patients
with
22
q
11
.
2
DS
with
complete
preoperative
cervical
vascular
and
spine
imaging
and
cardiac
evaluation
between
1998
and
2011
.
Main
Outcome
Measures
:
 
Incidence
of
cardiac
,
cervical
spine
,
and
vascular
abnormalities
;
related
perioperative
complications
;
and
resulting
changes
in
surgical
,
anesthetic
,
or
perioperative
management
plan
.
Results
:
 
Cardiac
abnormalities
occurred
in
45
patients
(
64
.
3
%
)
,
and
8
patients
required
cardiac
anesthesia
.
Thirty
-
eight
patients
(
54
.
3
%
)
had
at
least
one
vascular
abnormality
of
the
neck
,
and
14
%
had
medial
deviation
of
the
internal
carotid
artery
.
Surgery
was
not
performed
in
one
patient
,
and
the
surgical
plan
was
altered
in
three
patients
because
of
carotid
anomalies
.
Cervical
spine
abnormalities
were
found
in
24
patients
(
34
.
3
%
)
;
8
patients
demonstrated
radiographic
evidence
of
cervical
instability
and
were
treated
with
spinal
precautions
during
surgery
.
The
presence
of
one
anomaly
was
not
predictive
of
any
other
finding
,
and
there
were
no
complications
related
to
the
heart
,
cervical
spine
,
or
carotid
arteries
.
Conclusions
:
 
Anomalies
of
the
heart
,
cervical
spine
,
and
cervical
vasculature
occur
frequently
in
22
q
11
.
2
DS
,
vary
drastically
in
severity
,
and
are
impossible
to
predict
based
on
other
features
of
the
syndrome
.
Preoperative
diagnosis
of
these
comorbidities
with
routine
imaging
can
minimize
the
risk
of
avoidable
surgical
complications
.
Diseases
Validation
Diseases presenting
"cervical spine abnormalities"
symptom
22q11.2 deletion syndrome
This symptom has already been validated
