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A computer-assisted diagnostic and treatment concept to increase accuracy and safety in the extracranial correction of cranial vault asymmetries.
[proteus syndrome]
Proteus
syndrome
is
described
as
a
progressive
,
asymmetric
,
disproportional
overgrowth
of
various
parts
of
the
body
.
The
theory
of
somatic
mosaicism
is
widely
accepted
to
be
the
cause
of
this
disease
.
Affected
patients
present
very
heterogeneous
symptoms
,
but
in
about
30
%
craniofacial
deformities
are
the
leading
clinical
features
.
Because
no
causal
therapy
exists
,
treatment
options
are
limited
to
surgical
improvement
of
functional
constraints
.
A
computer-assisted
method
was
used
to
increase
the
accuracy
and
safety
of
bone
removal
in
the
extracranial
correction
of
cranial
vault
asymmetries
.
Descriptions
of
the
diagnosis
,
preoperative
planning
,
and
intraoperative
management
of
craniofacial
dysmorphia
caused
by
Proteus
syndrome
in
a
6
-
year
-old
boy
are
presented
.
After
computed
tomography-based
generation
of
a
virtual
3
-
dimensional
(
3
D
)
model
of
the
patient
and
a
haptic
stereolithographic
model
to
display
the
special
pathology
,
flow-sensitized
4
-
dimensional
magnetic
resonance
imaging
was
performed
to
clarify
the
properties
of
vascular
formation
inside
the
hyperostosis
.
To
transfer
the
mathematically
optimized
preoperative
planning
of
a
new
skull
shape
to
the
patient
,
a
surgical
guide
was
fabricated
by
rapid
manufacturing
.
Intraoperative
3
D
real-time
navigation
was
installed
as
an
additional
visualization
and
security
feature
.
The
surgery
could
be
performed
safely
and
quickly
.
Postoperative
imaging
showed
that
the
surgical
plan
was
realized
with
high
accuracy
.
This
newly
developed
and
validated
method
can
be
successfully
implemented
in
the
operating
room
environment
.
Diseases
Validation
Diseases presenting
"hyperostosis"
symptom
erdheim-chester disease
familial hypocalciuric hypercalcemia
focal myositis
proteus syndrome
well-differentiated liposarcoma
This symptom has already been validated
