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Foot or hand malformations related to deep venous system anomalies of the lower limb in Klippel-Trénaunay syndrome.
[proteus syndrome]
Klippel-
Trénaunay
syndrome
(
KTS
)
is
a
capillary-lymphatic-
venous
malformation
associated
with
soft
tissue
and
skeletal
hypertrophy
of
one
or
more
limbs
.
Deep
venous
system
(
DVS
)
anomalies
are
reported
to
be
present
in
8
%
to
18
%
of
patients
with
KTS
;
approximately
25
%
of
patients
with
KTS
have
hand
or
foot
malformations
.
We
sought
to
assess
whether
the
presence
of
hand
or
foot
malformations
in
KTS
is
a
predictor
of
DVS
anomalies
.
Retrospective
data
were
collected
from
51
consecutive
patients
with
KTS
seen
in
a
university
hospital
between
January
2000
and
February
2008
.
Patients
with
possible
Proteus
syndrome
were
not
included
.
The
presence
and
patency
of
the
DVS
was
studied
using
conventional
venography
,
multidetector
computed
tomography
,
or
fast
3
-
dimensional
magnetic
resonance
imaging
venography
.
Seventeen
hand
or
foot
malformations
were
present
in
9
patients
,
consisting
of
:
toe
macrodactyly
in
5
patients
(
two
bilateral
and
one
with
plantar
expansion
)
;
toe
microdactyly
in
one
patient
;
finger
macrodactyly
in
one
patient
;
finger
macrodactyly
and
ectrodactyly
in
one
patient
;
syndactyly
in
4
patients
;
and
clinodactyly
with
camptodactyly
of
the
hand
of
one
patient
with
lower
limb
KTS
.
Eleven
patients
had
DVS
anomalies
(
one
with
aplasia
of
entire
DVS
;
one
with
duplication
of
the
superficial
femoral
vein
;
7
with
hypoplasia
of
femoral
vein
;
and
7
with
aplasia
of
the
popliteal
vein
)
.
All
patients
with
hand
or
foot
malformations
also
had
DVS
anomalies
(
P
<
.
001
)
.
Small
sample
size
was
a
limitation
.
The
presence
of
hand
or
foot
malformations
in
KTS
may
predict
the
presence
of
DVS
anomalies
.
Diseases
Validation
Diseases presenting
"small sample size"
symptom
lamellar ichthyosis
proteus syndrome
systemic capillary leak syndrome
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