Cerebral Embolic Stroke and Arm
Ischemia in a Teenager With Arterial
Thoracic Outlet Syndrome:
A Case Report

Vascular and
Endovascular Surgery
Volume 41 Number 3
June/July 2007 254-257
©2007 Sage Publications
10.1177/1538574407299780
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Thomas S. Lee and George L. Hines, MD
A rare presentation of arterial thoracic outlet syndrome (TOS) is described in a young woman. Arterial
TOS caused by a cervical rib produced acute upper
extremity ischemia due to subclavian artery aneurysm
formation. Clinical presentation also included left
hemiparesis caused by right subclavian artery thrombosis and retrograde embolization of thrombus via the
common carotid artery to the right middle cerebral
artery distribution. Surgical repair of the subclavian

artery was performed, but permanent neurologic
deficit remained. Acute thrombosis of the right subclavian artery can produce cerebrovascular complication.
The assessment of such risk in patients with arterial
TOS is warranted and the arterial lesion corrected
surgically.

T

Case Report

horacic outlet syndrome (TOS) is a condition
caused by the compression of brachial plexus
elements or subclavian vessels in the cervicoaxillary region. It typically involves 3 regions,
which are the interscalene triangle, costoclavicular
triangle, and subcoracoid space.1 Unfortunately, the
diagnostic criteria especially for the neurologic form
of TOS, as well as the treatment, still remain to be
topics of much debate. As a result, even the incidence of TOS is debatable, ranging from 3 to 80
cases per 1000 people.2,3
This case report describes an unusual cerebral
vascular complication secondary to TOS in a patient
with bilateral cervical ribs. In the process, we hope
to highlight the importance of assessing the risk of
cerebrovascular complication when right subclavian
artery involvement is suspected.

From the Division of Vascular Surgery, Winthrop University
Hospital, Mineola, NY (GLH), and the School of Medicine, State
University of New York Stony Brook, Stony Brook, NY (TSL).
Address correspondence to: George L. Hines, MD, Winthrop
University Hospital, 259 First Street, Mineola, NY 11501; e-mail:
mepstein@winthrop.org.

Keywords: thoracic outlet syndrome; arterial thoracic outlet syndrome; subclavian artery

A 15-year-old female with past medical history of
TOS diagnosed 3 months prior at an outside institution came to the hospital emergency room. The
patient reported a sudden onset of left facial drooling and left-sided weakness associated with sudden
blurry vision, nausea, vomiting, and severe headache
located behind the right eye that began 5 hours prior
to arrival. She stated that she woke up from a nap
with the left-sided weakness. There was no history
of trauma, seizure disorder, or loss of consciousness.
The patient stated that 1 week earlier her right arm
was temporarily locked with a bent elbow. She also
described a 2- to 3-month history of intermittent
right index finger tingling, numbness, and pinprick
sensation. Previous MRI and venogram revealed the
diagnosis of TOS. She has been under the care of
orthopedists and was treated conservatively with
osteopathic manipulations, including increasing
range of motion in shoulder, cervical neck massage,
and spinal relaxations.
The examination on admission revealed an alert
and oriented patient with significant left facial
droop with mildly slurred speech. Extraocular movements were intact with equal and reactive pupils

254
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Arterial Thoracic Outlet Syndrome / Lee, Hines

bilaterally. The examination of extremities revealed
pale and cool right upper extremity with nonpalpable
radial and brachial pulses, but bounding left upper
extremity radial and brachial pulses. Left upper and
lower extremities displayed 3/5 strength, whereas
right upper and lower extremities exhibited 5/5
strength. Deep tendon reflexes were significantly
decreased on the left side with positive Babinski’s
sign. Lastly, sharp and dull sensory tests were intact
bilaterally.
The admission chemistry, complete blood count,
and head CT were all normal. The patient was
admitted to the pediatric ICU with the working
diagnosis of left hemiplegia. Within the next 12
hours, left-sided weakness worsened progressively
from 3/5 to 0/5 in strength but with unaffected sensation. Subsequent MRI and MRA revealed the
occlusion of the right middle cerebral artery (MCA)
and right MCA infarct. Digital subtraction angiography showed the occlusion of right subclavian artery
at the level of the mid-clavicle (Figure 1) and at the
distal end of the right internal carotid artery.
Although the right common carotid artery and common carotid bifurcation were normal, there was
absence of flow to the right anterior or middle cerebral arteries due to an abnormal right internal carotid
artery (Figure 2). Right posterior cerebral artery flow
was preserved. Upper arterial duplex scan showed
total occlusion of the right subclavian and brachial
arteries, whereas cerebrovascular duplex scan
demonstrated abnormal Doppler waveform in the
right common carotid artery without obvious signs
of stenosis or occlusion. Cervical x-ray revealed bilateral cervical ribs. Negative echocardiogram ruled out
cardiac source of embolus. Lastly, hypercoagulation
tests revealed factor V Leiden heterozygous status
and a positive antinuclear antibody of 80. However,
the tests for anti-double strand DNA antibody, antismooth muscle antibody, anticardiolipin, C3, C4,
anti-thrombin III, protein C, protein S, and homocystinemia were all negative.
Upon diagnosis of arterial thrombus, heparin
was administered with partial thromboplastin time
in the range of 55 to 70. Requested by neurology, a
combination of carbidopa and levodopa (Sinemet,
Merck & Co, Inc, Whitehouse Station, NJ) was also
started. On hospital day 8, the patient underwent a
surgical intervention, including right subclavian,
axillary, and brachial thrombectomy and resection of
right cervical rib, using the supraclavicular approach.
A segment of the right subclavian artery was resected

255

Figure 1. Digital subtraction angiography depicts the obstruction of flow in the right subclavian artery, whereas right common carotid artery flow is maintained. The left common carotid
and subclavian arteries are shown to be patent.

Figure 2. Sagittal view of the patient's head reveals occlusion
of distal right internal carotid artery and the absences flow to
right middle cerebral artery and right anterior cerebral artery.

owing to significant intimal damage, aneurysm formation, and arterial sclerosis. Right subclavian to
right axillary artery bypass was performed using right
great saphenous vein graft. The procedure was welltolerated by the patient. Postoperatively, the patient’s
right upper extremity became warm and thin radial
pulses were present. Since then, no cyanotic events
of her right upper extremity have been reported. The

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Vascular and Endovascular Surgery / Vol. 41, No. 3, June/July 2007

patient was discharged with an intense physical
therapy regimen. At 3 months follow-up, the patient
was able to ambulate without assistance but had
residual left upper extremity weakness.

Discussion
TOS can involve the brachial plexus, subclavian vein,
or subclavian artery. Reportedly, most TOS cases
involve the brachial plexus (94%-96%) and result in
neurological symptoms as initial complaints.4,5 Pain,
paresthesia, or weakness in ulnar distribution,
involving C8 and T1, are the most frequent initial
neurologic symptoms.1 Rarer forms of TOS involve the
subclavian vein (4%-6%) or subclavian artery (1%).4,5
The signs of subclavian vein involvement include
thrombosis, edema, and cyanosis of the involved
upper extremity as well as the distension of superficial veins of the shoulder and chest.6 Contrastingly,
subclavian artery compression may present with pallor, pulselessness, coolness of the involved upper
extremity, as well as antegrade or retrograde propagation of a thrombus or embolus.6
The subclavian vessel’s involvement in TOS is
often due to neck trauma or anatomic abnormalities
such as cervical ribs, abnormal first rib, enlarged
transverse process of seventh vertebra, or congenital
fibromuscular bands.7 In our patient’s case, the most
likely cause of TOS in the absence of neck trauma
history was the presence of cervical ribs. Cervical
ribs induce pathologic conditions by narrowing the
passageway through which the brachial plexus and
the subclavian artery pass in the interscalene triangle, which is defined by the anterior scalene muscle
anteriorly, middle scalene muscle posteriorly, and
medial surface of the first rib inferiorly.1,8 The
reported incidence of cervical ribs is about 1% in the
general population.8,9 Cervical ribs are found twice
as frequently in females (68%) than in males (32%)
and are bilateral in greater than 50% of involved
cases.10 Whereas cervical ribs are asymptomatic in
90% of cases, symptomatic cervical ribs typically
result in neurogenic TOS and, less commonly, arterial TOS.8 The pattern of involvement differs between
incomplete and complete cervical ribs. Complete
cervical ribs are associated with neurogenic or arterial TOS. However, only neurogenic TOS is typically
observed with incomplete cervical ribs.8,11 Furthermore, the onset of TOS in the absence of neck
trauma was higher with complete cervical ribs (50%)
than with incomplete cervical ribs (15%).8 Better

results from surgical intervention were observed
with complete cervical ribs than with incomplete
cervical ribs.8
Although subclavian artery involvement is the
rarest form of TOS, such involvement can carry
significant prognostic risks. Three major pathologic changes in arterial TOS include partial or
complete thrombosis of the subclavian artery, poststenotic dilatation of the subclavian artery, and
poststenotic subclavian artery aneurysm.7 All 3
entities can result in distal embolization of the
involved upper extremity as well as acute hand
ischemia, claudication, vasomotor phenomena,
and digital gangrene. In addition, as in our patient,
the more uncommon complication of retrograde
thrombus propagation from subclavian artery to
vertebral or carotid arteries can result in cerebrovascular embolism.
The diagnosis of vascular TOS should begin with
careful history and physical examination. Arterial
TOS occurs typically in young adults with vigorous
arm activity. Patients with arterial TOS may present
initially with mild symptoms due to extensive arterial
collateralization of upper extremities. As a result,
patients may not seek medical attention until
ischemic events have occurred, such as ulceration of
digits, gangrene, absent pulses, or Raynaud’s phenomenon.12 Physical examination may reveal signs
of arterial compromise such as pallor, pulselessness,
and coolness of the involved limb. One should auscultate for bruits. In addition, blood pressure measurements of both arms should be evaluated. If the
blood pressure in the affected limb is markedly
reduced and such difference in the blood pressures
is greater than 20 mm Hg from the contralateral
limb, arterial involvement is likely.1 However,
provocative maneuver tests, such as Adson’s and
Halsted, are unreliable.10
Further imaging studies can be useful in the
establishment of diagnosis and understanding the
extent of pathology. Chest radiographs and cervical
spine x-rays are useful tools in detecting abnormal
bony structures, such as cervical rib and anomalous
first rib. However, soft tissue abnormality causing
TOS may require MRI or MRA. Color flow duplex
and plethysmography are helpful noninvasive studies. Arteriography, venography, MRI, MRA/magnetic
resonance venography (MRV), or cytotoxic assay can
be used to confirm the diagnosis. Minimally, 4-vessel
view arch aortogram should be conducted if a patient
is suspicious for vascular TOS.

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Arterial Thoracic Outlet Syndrome / Lee, Hines

Surgical intervention is indicated in vascular
TOS as it is typically resistant to conservative management.1 The surgical treatment of arterial TOS
includes the excision of the cervical and/or first rib,
followed by reconstructive vascular procedure, such
as resection and anastomosis or replacement with
vein or prosthetic grafts.7 Two common approaches
to TOS are the supraclavicular and transaxillary
approaches. The advantage of the supraclavicular
approach is that it allows good exposure of the
brachial plexus, medial two thirds of the first rib,
and the cervical rib, if present.1,12 As a result, first rib
resection or anterior and middle scalenectomies can
be done with this approach. The transaxillary approach
provides access to the first rib through a hidden incision. However, there is limited exposure of neural
structures and the cervical rib with the transaxillary
approach.1 The success rates of the supraclavicular
and transaxillary approaches defined as complete to
partial resolution of symptoms are 87.5% to 89%
and 81% to 93%, respectively.8,9,13,14 Nonetheless,
Sanders reported comparable success rates for anterior and middle scalenectomy, transaxillary first rib
resection, and combined supraclavicular scalenectomy and first rib resection.15 Potential complications from TOS decompression surgeries include
major neurovascular injuries, Horner’s syndrome,
phrenic nerve, long thoracic nerve, supraclavicular
nerve, and intercostal brachial nerve injuries.10,12
There are limited reported cases of cerebral
complication as a result of retrograde embolic event
from subclavian artery involvement in TOS.16-18
However, all those reports involved the right subclavian artery with the propagation of thrombus to the
right brachiocephalic trunk and had a latent period
of 3 months to 3 years from the onset of upper limb
symptoms and major cerebral ischemic event.16
Although an embolic event leading to cerebrovascular accident is a rare occurrence, vigorous diagnostic
workup appears to be warranted to prevent such a
devastating complication. Furthermore, when such
risk is found, surgical treatment should be carefully
considered, instead of conservative treatment.

257

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