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Assessment and management of the orthopedic and other complications of Proteus syndrome.
[proteus syndrome]
A
multidisciplinary
workshop
was
convened
at
the
National
Institutes
of
Health
(
NIH
)
to
discuss
the
management
of
the
orthopedic
and
other
complications
of
Proteus
syndrome
(
PS
)
,
a
progressive
,
disproportionate
overgrowth
disorder
.
While
PS
poses
many
complex
challenges
,
the
focus
of
the
workshop
was
the
management
of
the
asymmetric
and
disorganized
skeletal
overgrowth
that
characterizes
this
multisystem
disorder
.
Workshop
participants
developed
recommendations
for
clinical
research
and
patient
management
and
surveillance
to
maximize
the
benefits
and
reduce
the
risks
of
surgical
and
other
interventions
.
Recommendations
for
clinical
care
and
management
included
assessments
of
skeletal
overgrowth
and
its
progression
with
modalities
such
as
X-
ray
,
magnetic
resonance
imaging
(
MRI
)
,
dual-energy
X-
ray
absorptiometry
,
and
computerized
tomography
(
CT
)
imaging
.
The
recommendations
also
cover
the
assessment
of
non-orthopedic
complications
of
PS
that
significantly
impact
surgical
risk
,
such
as
pulmonary
embolism
and
lung
bullae
.
Surgical
considerations
in
PS
include
assessment
of
the
contribution
of
contractures
to
deformities
and
prophylactic
soft
-tissue
release
,
aggressive
and
early
use
of
epiphysiodesis
and
epiphysiostasis
,
amputation
,
and
spinal
bracing
.
Decisions
on
the
timing
of
orthopedic
procedures
in
children
with
PS
are
challenging
because
they
entail
balancing
the
risks
of
intervention
in
this
high
-risk
and
complex
population
against
the
increasing
morbidity
that
patients
experience
with
progressive
bony
overgrowth
.
If
surgery
is
delayed
too
long
,
the
condition
may
become
inoperable
.
We
hope
that
these
recommendations
will
help
clinicians
gather
appropriate
data
and
assist
their
patients
in
making
timely
treatment
decisions
.
Diseases
Validation
Diseases presenting
"ps that significantly impact surgical risk"
symptom
proteus syndrome
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