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Asymmetric, disproportionate craniofacial hyperostoses of mosaic pattern in a patient not meeting Proteus syndrome criteria.
[proteus syndrome]
A
34
-
year
-old
patient
with
asymmetric
hyperostosis
of
the
craniofacial
skeleton
much
more
pronounced
on
the
right
side
is
presented
.
A
long
-term
follow-up
of
16
years
showed
progression
of
overgrowth
even
after
skeletal
maturity
and
despite
repeated
surgical
corrections
focusing
on
regional
reduction
of
the
hyperostoses
.
Clinical
situation
during
infancy
,
adolescence
,
and
adulthood
is
documented
.
Although
the
craniofacial
hyperostosis
in
the
patient
was
indicative
of
the
Proteus
syndrome
,
the
applicable
criteria
were
not
met
.
Proportionate
mild
hemihypertrophia
/
hyperplasia
of
the
patient
's
right
extremities
also
exclude
an
oligosymptomatic
variant
of
the
Proteus
syndrome
limited
to
the
skull
.
Both
surgical
strategies
and
difficulty
of
a
diagnostic
classification
are
discussed
.
Diseases
Validation
Diseases presenting
"despite repeated surgical corrections focusing on regional reduction of the hyperostoses"
symptom
proteus syndrome
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