Hand muscle atrophy and digital ischemia as an unusual presentation of an occluded aberrant right subclavian artery: Endovascular or open approach?

Abstract

Introduction:
An aberrant right subclavian artery (ARSA) or lusorian artery is one of the most common variations of the aortic arch. Although usually without symptoms, some ARSA's occasionally become symptomatic.

Presentation of case:
A 51-year old woman presented with a painful right middle finger. Clinical examination showed thenar muscle hypotrophy and blue discoloration of the distal phalanx suggestive of embolization. Magnetic resonance angiography revealed a non-aneurysmal proximally occluded ARSA. A venous common carotid artery to subclavian artery bypass was combined with ARSA ligation proximal to the right vertebral artery.

Discussion:
Occlusive symptomatic ARSA disease without aneurysmal dilatation is uncommon. Treatment may include bypass grafting by open surgery or angioplasty with stenting.

Conclusion:
Treatment for occlusive non-aneurysmal ARSA must be tailored to the individual. Whether an endovascular or surgical approach is preferred depends on localization of the lesion in relation to the esophagus and on the general condition of the patient.

Full text

CASE
REPORT
–
OPEN
ACCESS
International
Journal
of
Surgery
Case
Reports
4 (2013) 565–
567
Contents
lists
available
at
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ScienceDirect
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Journal
of
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Case
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Hand
muscle
atrophy
and
digital
ischemia
as
an
unusual
presentation
of
an
occluded
aberrant
right
subclavian
artery:
Endovascular
or
open
approach?
B.
Leenders∗,
G.
den
Butter,
M.R.
Scheltinga,
G.
Debonnaire
Department
of
Vascular
Surgery,
Máxima
Medical
Center,
Veldhoven,
The
Netherlands
a
r
t
i
c
l
e
i
n
f
o
Article
history:
Received
11
October
2012
Accepted
13
October
2012
Available online 18 April 2013
Keywords:
Arteria
lusoria
Aberrant
right
subclavian
artery
Treatment
a
b
s
t
r
a
c
t
INTRODUCTION:
An
aberrant
right
subclavian
artery
(ARSA)
or
lusorian
artery
is
one
of
the
most
common
variations
of
the
aortic
arch.
Although
usually
without
symptoms,
some
ARSA’s
occasionally
become
symptomatic.
PRESENTATION
OF
CASE:
A
51-year
old
woman
presented
with
a
painful
right
middle
finger.
Clinical
examination
showed
thenar
muscle
hypotrophy
and
blue
discoloration
of
the
distal
phalanx
suggestive
of
embolization.
Magnetic
resonance
angiography
revealed
a
non-aneurysmal
proximally
occluded
ARSA.
A
venous
common
carotid
artery
to
subclavian
artery
bypass
was
combined
with
ARSA
ligation
proximal
to
the
right
vertebral
artery.
DISCUSSION:
Occlusive
symptomatic
ARSA
disease
without
aneurysmal
dilatation
is
uncommon.
Treat-
ment
may
include
bypass
grafting
by
open
surgery
or
angioplasty
with
stenting.
CONCLUSION:
Treatment
for
occlusive
non-aneurysmal
ARSA
must
be
tailored
to
the
individual.
Whether
an
endovascular
or
surgical
approach
is
preferred
depends
on
localization
of
the
lesion
in
relation
to
the
esophagus
and
on
the
general
condition
of
the
patient.
© 2013 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
1.
Introduction
An
aberrant
right
subclavian
artery
(ARSA),
also
known
as
a
luso-
rian
artery,
is
one
of
the
common
aortic
arch
anatomic
variations.
This
variant
occurs
in
0.6–2.5%
of
general
population
whereas
its
prevalence
is
even
higher
in
patients
with
congenital
abnormal-
ities
of
the
heart
and
greater
vessels.
An
ARSA
is
almost
always
asymptomatic
and
is
usually
identified
after
routine
thorax
or
aortic
arch
imaging.
However,
an
ARSA
may
sporadically
become
symptomatic.
The
complex
of
symptoms
is
related
to
tracheal
or
esophageal
compression
(dysphagia
lusoria),
either
by
reduced
blood
flow
due
to
occlusion
or
by
aneurysmal
dilatation.1–3 We
present
a
patient
with
an
unusual
complex
of
symptoms
includ-
ing
digital
ischemia
and
hand
muscle
atrophy
due
to
an
occluded
non-aneurysmal
ARSA.
2.
Case
report
A
51-year
old
woman
presented
with
pain
in
the
third
digit
of
the
right
hand.
She
had
a
two-year
history
of
arm
claudication
∗Corresponding
author
at:
Department
of
Surgery,
Máxima
Medical
Center,
De
Run
4600,
5504
DB
Veldhoven,
The
Netherlands.
Tel.:
+31
40
8888000.
E-mail
addresses:
b.leenders@mmc.nl,
bart
leenders@hotmail.com
(B.
Leenders).
that
was
previously
diagnosed
as
Raynaud’s
disease.
Her
pre-
vious
medical
history
included
smoking,
hypertension
and
a
laparoscopic
cholecystectomy.
Hand
inspection
demonstrated
sub-
stantial
hypotrophy
of
the
right
thenar
eminence
(Fig.
1)
and
blue
discoloration
of
the
distal
phalanx
of
the
middle
finger.
Capillary
refill
was
slow
(>4
s).
Brachial,
ulnar
and
radial
pulsations
were
absent
in
contrast
to
the
left
arm.
Blood
pressure
was
unrecordable
compared
to
180/90
mmHg
on
the
left
side.
An
occluded
brachial
artery
due
to
emboli
based
on
cardiac
disease
was
hypothesized.
However,
a
transthoracic
cardiac
ultra-
sound
excluded
the
heart
as
the
source
of
embolization
while
an
electrocardiogram
showed
normal
sinus
rhythm.
Magnetic
resonance
angiography
(MRA)
revealed
the
presence
of
a
non-
aneurysmal
ARSA
with
a
3
cm
occlusion
at
the
point
where
the
vessel
crossed
the
esophagus
in
the
posterior
plane
(Fig.
2A).
The
recent
literature
on
ARSA
was
reviewed.
As
hand
tissue
loss
was
deemed
impending,
it
was
decided
to
perform
an
open
revas-
cularization.
Following
general
anesthesia
and
under
continuous
electroencephalographic
monitoring,
the
ARSA
was
approached
via
a
supraclavicular
incision
allowing
a
clear
view
on
structures
including
the
right
common
carotid
artery,
ARSA,
phrenic
and
recurrent
nerves.
A
saphenous
vein
conduit
was
used
as
a
bypass
running
from
the
right
common
carotid
artery
to
the
right
sub-
clavian
artery.
The
lusorian
artery
proximal
to
the
right
vertebral
artery
was
ligated
(Fig.
2B).
The
patient
remains
well
18
months
postoperatively
with
an
open
bypass
and
normal
hand
function.
2210-2612/$
–
see
front
matter ©
2013 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijscr.2012.10.026

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REPORT
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566 B.
Leenders
et
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/
International
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Case
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4 (2013) 565–
567
Fig.
1.
Hypotrophy
of
the
right
thenar
muscle.
3.
Discussion
An
aberrant
right
subclavian
artery
(ARSA)
typically
originates
distally
to
the
left
subclavian
artery
and
runs
posteriorly
to
the
aor-
tic
arch.
On
its
course,
it
may
cross
either
behind
the
esophagus
(80–84%),
between
the
esophagus
and
the
trachea
(12.7–15%),
or
in
front
of
the
trachea
(4.2–5%).
Two-thirds
of
ARSAs
remain
asymptomatic
during
life.
How-
ever,
three
types
of
symptoms
may
occur
in
the
remaining
one-third.4A
first
type
is
related
to
compression
to
the
esopha-
gus
or
trachea
presenting
as
difficulty
with
swallowing
(dysphagia
lusoria).
Symptoms
of
the
second
type
of
symptomatic
ARSAs
are
due
to
aneurysm
formation.
Dilatation
preferably
occurs
at
the
base
of
the
vessel,
leading
to
a
Kommerell’s
diverticulum.
A
third
type
of
symptom
is
related
to
stenotic
alterations
due
to
atheroscle-
rotic
or
fibromuscular
changes.
Dysphagia
lusoria,
dyspnea
and
chronic
coughing,
respectively
due
to
esophageal
or
tracheal
com-
pression,
are
thus
by
far
the
most
common
presenting
symptoms
accounting
for
up
to
90%
of
symptomatic
ARSA
patients.5Rare
man-
ifestations
are
right
arm
ischemia
due
to
embolization,
esophageal
fistulization,
local
compression
causing
thoracic
outlet
syndrome
or
superior
vena
cava
syndrome
and
chest
pain.
Hemorrhagic
shock
from
rupture
has
also
been
reported.6–8
Kieffer,
reporting
on
the
largest
cohort
of
33
cases,
proposed
a
classification
based
on
anatomy.9The
first
group
consists
of
patients
with
dysphagia
caused
by
non-aneurysmal
ARSA.
Group
2
comprises
patients
with
symptomatic
occlusive
disease
of
non-
aneurysmal
ARSA.
Group
3
harbors
patients
with
an
aneurysmal
ARSA
without
aortic
lesions,
with
or
without
esophageal
compres-
sion
or
arterial
thrombo-embolism.
Patients
with
an
aortic
(usually
aneurysmal)
lesion
involving
the
origin
of
the
ARSA
form
Group
4.
The
ARSA
of
the
present
patient
belonged
to
the
second
group.
Once
an
ARSA
is
suspected
or
deemed
symptomatic,
imag-
ing
using
MRA
or
computed
tomography
angiography
(CTA)
may
visualize
arteries
including
the
aortic
arch
thorax
and
arm.
CTA
is
preferred
if
dilatation,
dissection
or
arteritis
is
suspected.10
Angiography
using
a
percutaneous
technique
allows
for
optimal
visualization
of
the
arteries
but
fails
to
show
vessel
topography
in
relation
to
other
tissues
such
as
the
esophagus
and
trachea.
Treatment
of
ARSA
must
be
tailored.
An
aneurysmal
ARSA
is
associated
with
high
mortality
and
morbidity
rates
as
44–57%
evolves
toward
fatal
rupture
or
fistulization.
Aneurysmal,
stenotic
or
symptomatic
disease
has
typically
been
treated
by
open
repair
with
the
first
procedure
reported
by
Gross
in
a
pediatric
group
in
1946.11 However,
an
open
repair
involves
a
sternotomy
or
left
pos-
terolateral
thoracotomy
to
control
the
origin
of
the
artery.
Hence,
a
hybrid
first
stage
approach
in
which
the
ARSA
is
occluded
distal
to
the
aneurysm
combined
with
a
covered
stent
at
the
origin
at
the
aorta
is
advised
in
recent
literature,
even
in
acute
settings.12,13
In
a
second
stage,
revascularization
of
the
right
arm
by
a
bypass
or
re-implantation
is
obtained
using
an
open
approach
via
a
right
supraclavicular
incision.
Patients
presenting
with
dysphagia
lusoria
due
to
a
non-
aneurysmatic
and
non-stenotic
ARSA
were
previously
treated
by
open
repair.
The
approach
was
through
a
right
supraclavicular
incision
with
proximal
ARSA
ligation
and
direct
re-implantation
onto
the
carotid
artery.
Recently,
Morris
et
al.
reported
a
combined
hybrid
procedure
in
which
an
occluder
is
placed
at
the
origin
of
the
artery
in
a
percutaneous
fashion
and
blood
flow
to
the
right
arm
is
restored
in
the
same
manner
as
mentioned
above.14
Treatment
for
embolizing
non-aneurysmal
ARSA
has
also
devel-
oped
from
an
open
toward
an
endovascular
approach.
Two
cases
on
endovascular
treatment
in
occlusive
diseased
ARSA
were
iden-
tified
in
the
literature.
Azakie
published
on
an
ARSA
patient
with
arm
claudication
and
dizziness
receiving
endovascular
treat-
ment
using
angioplasty
and
stenting.
A
possible
disadvantage
of
stenting
is
an
increased
vessel
diameter
possibly
leading
to
Fig.
2.
(A)
MRA
showing
proximal
total
occlusion
of
a
aberrant
right
subclavian
artery.
(B)
MRA
two
months
postoperatively
showing
patent
carotico-subclavian
bypass
and
right
vertebral
artery
and
occluded
aberrant
right
subclavian
artery.

CASE
REPORT
–
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Leenders
et
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/
International
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of
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Case
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4 (2013) 565–
567 567
symptomatic
esophageal
compression.15 Angioplasty
and
stenting
of
ostial
lesions
does
not
lead
to
compression
and
can
be
conducted
through
trans-brachial
access.16
Although
a
minimal
invasive
approach
in
the
present
case
was
considered,
it
was
decided
upon
an
open
repair
for
several
reasons.
First,
our
patient
was
young
and
fit
for
surgery.
Second,
percuta-
neous
manipulations
probably
induced
additional
embolization
as
a
highly
unstable
plaque
was
feared.
Thirdly,
additional
stenting
may
have
harmed
the
integrity
of
the
esophagus
that
was
located
in
close
proximity.
In
conclusion,
treatment
of
a
non-aneurysmatal,
stenotic
or
occluded
ARSA
depends
on
patient
status,
anatomical
relation-
ship
to
neighboring
organs
and
characteristics
of
the
lesion
within
the
ARSA.
An
endovascular
approach
may
be
preferred
in
high-risk
patients
with
stenotic
diseased
ARSA
with
low
proba-
bility
of
embolism
and
a
reasonable
distance
to
the
esophagus.
In
contrast,
open
surgery
using
a
bypass
or
re-implantation
is
justified
in
young
healthy
patients
with
unstable
stenotic
disease.
Conflict
of
interest
statement
None.
Funding
None.
Contributions
Written
consent
obtained.
Author
contributions
All
authors
contributed.
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