DOI:10.2214/AJR.06.0983
AJR 2007; 189:S21-S23
© American Roentgen Ray Society
AJR Teaching File: Weight Lifter with Swelling in the Upper Arm
Deepa Sheth1,
Hector Ferral and
Nilesh H. Patel2,3
1 College of Medicine, University of Illinois at Chicago, 1740 W. Taylor St.,
Chicago, IL 60612.
2 Department of Radiology, Diagnostic Radiology and Nuclear Medicine, Rush
University Medical Center, Chicago, IL.
3 Vascular and Interventional Program, Central DuPage Hospital, Winfield,
IL.
Received July 26, 2006;
accepted after revision October 4, 2006.
Address correspondence to D. Sheth
(dsheth2{at}uic.edu).
Keywords: deep vein thrombosis • Paget-Schroetter syndrome • thrombolytic therapy • thrombosis
Clinical History
A 35-year-old male weight lifter presents with swelling in the left
arm.
Radiologic Description
Left upper extremity venogram (Fig.
1A) obtained through a median cubital vein access shows extensive
filling defects in the subclavian, axillary, and basilic veins. No antegrade
flow into the brachiocephalic vein or superior vena cava is seen.
Differential Diagnosis
The diagnosis is deep vein thrombosis. Vascular tumor invasion is extremely
rare. The cause of axillosubclavian vein thrombosis is either primary or
secondary. Primary axillosubclavian vein thrombosis may be due to anatomic
venous compression at the thoracic outlet (Paget-Schroetter syndrome) or upper
limb immobility, whereas secondary axillosubclavian vein thrombosis may be due
to venous catheterization (catheters, ports); hemodialysis conduits and
fistulas; infusate-related (sclerosants, vesicants); pacemaker wires; IV drug
abuse; radiation; fibrosis; cardiac failure; shoulder trauma; amyloidosis;
sarcoidosis; oral contraceptive use; or local compression by tumor, metastatic
disease, or lymphadenopathy.
Diagnosis
The diagnosis in this patient is Paget-Schroetter syndrome.
Commentary
Primary axillosubclavian vein thrombosis is due to extrinsic compression by
surrounding bone and soft-tissue structures found in the costoclavicular
space. The subclavian vein may be compressed as it passes between the clavicle
and subclavius muscle anteriorly and the first rib and anterior scalene muscle
posteriorly. The axillary vein may be compressed by the pectoralis minor
muscle and the rib cage. In addition, a cervical rib, muscle hypertrophy,
callus from a past clavicular fracture, and congenital fibromuscular bands may
also compress the subclavian or axillary vein. Emphasized in this particular
case study was axillosubclavian vein thrombosis as a result of anatomic
impingement and excessive physical use.
A schematic diagram shows the anatomic relationship of the subclavian
artery, subclavian vein, anterior scalene muscle, clavicle, and first rib with
the arm in the adducted (neutral) position and abducted position (Figs.
2A and
2B).
The clinical presentation is usually quite dramatic and unexpected, often
occurring in otherwise healthy, young, active individuals. Patients clinically
present with swelling, pain, and a history of excessive physical activity or
unusual arm positioning of the affected arm. A venogram of the affected arm
will show the exact location and extent of the compression of the
subclavian-axillary vein. MDCT angiography of the dynamic compression may
differentiate first-rib compression from scalenus anterior compression of the
subclavian vein.
In patients with primary axillosubclavian vein thrombosis,
catheter-directed thrombolytic therapy is the preferred method over a surgical
thrombectomy [1]. Figures
1B–1D
depict this process.
A Unifuse catheter (Angiodynamics Inc.) was embedded in the clot burden,
and catheter-directed thrombolytic therapy with recombinant tissue-type
plasminogen activator (Alteplase, Genentech Inc.) was initiated at 0.5 mg/h.
IV heparin was also started at 500 U/h
(Fig. 1B). After 14 hours of
infusion, venography shows significant lysis of the clot burden with narrowing
and irregularity of the left subclavian vein at the level of the thoracic
outlet (Fig. 1C). The narrowing
was dilated with a 6-mm diameter angioplasty balloon and then thrombolytic
infusion was reinitiated. Follow-up venography after a further 8-hour infusion
shows no residual thrombus and focal narrowing of the central aspect of the
left subclavian vein (Fig. 1D).
The patient was discharged with instructions to undergo oral anticoagulation
with warfarin. Two weeks later, the patient underwent resection of the first
rib [1].
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Fig. 1C —35-year-old male weight lifter presents with swelling in the
left arm. After 14 hours of recombinant tissue plasminogen activator
administration, venogram shows lysis of thrombus with narrowing in medial
aspect of left subclavian vein.
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Once the thrombus has been completely removed, venography should be
performed with the affected arm in the adducted and abducted positions to help
delineate the location of the extrinsic compression. In Paget-Schroetter
syndrome, surgical resection of the medial half of the clavicle or
transaxillary first rib resection is performed
[1]. If symptoms recur due to
continued presence of a significant venous stenosis, they can be treated with
balloon angioplasty with or without stenting
[2].The patient may have an
underlying coagulopathy, which can be followed up with a hypercoagulable
workup [3].
In this case, the use of a venogram was pertinent in three important ways:
It identified the extent of the thrombus, it showed the result of the
thrombolytic therapy, and it identified the location and extent of compression
of the subclavian vein.
Paget-Schroetter syndrome was first described by the French pathologist
Léon Jean Baptiste Cruveilhier (1791–1874) in 1816, then by Paget
in 1875, and a patient analysis was presented by von Schrötter in 1884.
Hughes introduced the term "Paget-Schroetter" in 1949
[4,
5]. Paget-Schroetter syndrome
refers to primary axillosubclavian vein thrombosis due to a thoracic outlet
abnormality, often precipitated by strenuous physical activity or unusual arm
positioning. The subclavian vein may be compressed by surrounding bone and
soft tissue found in the costoclavicular space. Clinical presentation is
swelling of the affected arm and pain that is related to use of that arm.
Catheter-directed thrombolytic therapy and systemic anticoagulation is the
preferred method of acute treatment; surgery remains the definitive
therapy.
Objective
The educational objective of this teaching article is to describe common
causes and preferred treatment of Paget-Schroetter syndrome, while
highlighting the importance of venography.
Conclusion
Paget-Schroetter syndrome is a dramatic, unexpected condition present in
otherwise healthy, young individuals. Often, the thrombotic event is
precipitated by strenuous physical activity or unusual positioning of the
affected arm. Patients clinically present with swelling and pain of the
affected arm. A venogram obtained of the affected arm can show the exact
extent and location of the stenosis and can also guide in the removal of the
thrombus. The overall goal of therapy is to restore and maintain patency of
the affected vein and alleviate the symptoms arising from venous
obstruction.
References
- Machleder HL. Thrombolytic therapy and surgery for primary
axillosubclavian vein thrombosis: current approach. Semin Vasc
Surg 1996; 9:46
–49[Medline]
- Lee JT, Karwowski JK, Harris EJ, Haukoos JS, Olcott C 4th.
Long-term thrombotic recurrence after nonoperative management of
Paget-Schroetter syndrome. J Vasc Surg2006; 43:1236
–1243[CrossRef][Medline]
- Hingorani A, Ascher E, Marks N, et al. Morbidity and mortality
associated with brachial vein thrombosis. Ann Vasc
Surg 2006; 20:297
–300[CrossRef][Medline]
- Cruveilhier LJB. Essai sur l'anatomie pathologique en
général et sur les transformations et productions organiques en
particulier. Doctoral thesis. 2 volumes, Paris,1816
- Paget J. On gouty and some other forms of phlebitis. St.
Bartholomew's Hospital Reports. London, 1866;2
: 82–92
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Clinical History
Radiologic Description
