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Ultrasound Detection of Superior Vena Cava Thrombus

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Superior vena cava (SVC) syndrome is most commonly the insidious result of decreased vascular flow through the SVC due to malignancy, spontaneous thrombus, infections, and iatrogenic etiologies. Clinical suspicion usually leads to computed tomography to confirm the diagnosis. However, when a patient in respiratory distress requires emergent airway management, travel outside the emergency department is not ideal. With the growing implementation of point-of-care ultrasound (POCUS), clinicians may make critical diagnoses rapidly and safely. We present a case of SVC syndrome due to extensive thrombosis of the deep venous system cephalad to the SVC diagnosed by POCUS. [West J Emerg Med. 2014;15(6):715-718]
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Volume XV, NO. 6 : September 2014 715 Western Journal of Emergency Medicine
Case RepoRt
Ultrasound Detection of Superior Vena Cava Thrombus
Aaron Birch, MD
David Um, DO
Brooks Laselle, MD
Supervising Section Editor: Rick McPheeters, DO
Submission history: Submitted October 9, 2012; Revision received May 29, 2014; Accepted June 10, 2014
Electronically published July 21, 2014
Full text available through open access at http://escholarship.org/uc/uciem_westjem
DOI: 10.5811/westjem.2014.6.14006
Superior vena cava (SVC) syndrome is most commonly the insidious result of decreased vascular
ow through the SVC due to malignancy, spontaneous thrombus, infections, and iatrogenic
etiologies. Clinical suspicion usually leads to computed tomography to conrm the diagnosis.
However, when a patient in respiratory distress requires emergent airway management, travel
outside the emergency department is not ideal. With the growing implementation of point-of-care
ultrasound (POCUS), clinicians may make critical diagnoses rapidly and safely. We present a case
of SVC syndrome due to extensive thrombosis of the deep venous system cephalad to the SVC
diagnosed by POCUS. [West J Emerg Med. 2014;15(6):715-718]
INTRODUCTION
Superior vena cava (SVC) syndrome, rst described
by William Hunter in 1757 in a patient with syphilitic
aneurysm,
1
is dened as a constellation of symptoms that
result from the decrease in vascular ow within the SVC.
Traditionally, this has been understood to mean a drop
in blood ow due to extrinsic compression of the SVC,
usually from a thoracic tumor or other rigid structure
within the thorax. Bronchogenic carcinoma, typically small
or squamous cell, is responsible for nearly 80% of all cases
of SVC syndrome, and lymphoma accounts for 15% of
cases; yet spontaneous thrombus, infections, and iatrogenic
etiologies are also described.
2-4
The formation of venous
thrombi secondary to pacemaker leads and central venous
catheters is also a well-documented phenomenon.
5,6
Several different anatomic factors contribute to
the development of SVC syndrome. Principally, it is
a relatively thin-walled vessel with low intravascular
pressure, making it easy to compress. Subsequent
compression impedes ow, which may contribute to
thrombus formation, particularly if there is a thrombogenic
foci already present, such as a central venous catheter.
Additionally, there are a number of rigid and semi-rigid
structures abutting the SVC, including vertebrae, ribs, and
the aorta, which can cause compression.
7,8
Imaging modalities, such as computed tomography
(CT), are rapid, standardized, and give a detailed view of
large areas. However, they nearly always require transport
Department of Emergency Medicine, Madigan Army Medical Center, Tacoma,
Washington
away from the resuscitation area and its resources. Point-
of-care ultrasound (POCUS) is a versatile imaging modality
that is instantly available at the bedside, can be performed
real-time in conjunction with resuscitation efforts, and
can be performed serially without harmful ionizing
radiation exposure to the patient or provider. Diagnosis
of SVC syndrome with POCUS has not been previously
described. This diagnosis is usually conrmed via traditional
angiography, CT angiography, or magnetic resonance
imaging (MRI).
9-11
As such, the following is a case presentation of SVC
syndrome due to extensive thrombosis of the deep venous
system cephalad to the SVC diagnosed by POCUS.
Figure 1. Patient demonstrating sharp line of demarcation inferior
to nipples separating pallor (inferior) and plethora (superior).
Western Journal of Emergency Medicine 716 Volume XV, NO. 6 : September 2014
Ultrasound Diagnosis of Superior Vena Cava Thrombus Birch et al
CASE REPORT
A 71-year-old male with a past medical history of acute
promyelocytic leukemia (PML) and diffuse large B-cell
lymphoma currently undergoing chemotherapy, presented to
the emergency department (ED) via ambulance with a chief
complaint of progressively worsening shortness of breath over
the past three days. He experienced an acute worsening of
this symptom accompanied by a gradual onset of upper body
cyanosis beginning that morning.
Upon initial examination, the patient was in severe
respiratory distress, speaking in three-word sentences. Initial
vital signs were the following: blood pressure 124/94 mmHg,
pulse 100 beats per minute, respiratory rate 28, temperature
97.9 F, SpO2 97% on 15 L/min non-rebreather mask. His
head and upper torso had a generalized cyanotic appearance
with a remarkably sharp demarcation line approximately
at the nipples (Figure 1). Neck exam showed jugular
venous distension without thyromegaly. The pulmonary
exam revealed tachypnea, bilateral wheezing and the use
of intercostal and supraclavicular accessory muscles. The
abdomen was soft, mildly tender, distended, without masses,
and with diminished bowel sounds. Extremity and neurologic
exams were unremarkable.
Initial attempts at obtaining peripheral intravenous
access proved exceedingly difcult despite obviously visible
vasculature. After gaining peripheral access, several nurses
noted difculty in drawing blood and ushing medications and
saline. POCUS was immediately performed to evaluate for the
cause of his acute dyspnea and to locate vessels for vascular
access, including a limited trans-thoracic echocardiogram,
extended focused assessment with sonography for trauma
(EFAST), evaluation of the inferior vena cava for preload and
bilateral internal jugular veins (IJV) assessments, for projected
central venous access.
The echocardiogram showed a small pericardial
effusion without evidence of tamponade with a grossly
normal ejection fraction. The EFAST revealed absence of
pneumothorax and a moderately large amount of free uid
in the abdomen consistent with previously known ascites.
The vascular ultrasound (US) examinations (Figures 2-5)
showed an inferior vena cava that collapsed more than 50%
with each respiration, suggesting a central venous pressure
of less than 8 mm Hg
12
; assessment of the IJVs demonstrated
bilateral thromboses with extension into the subclavian and
axillary veins bilaterally. There was no thrombosis in the right
femoral vein and because of lack of functional IV access, a
triple lumen central line was then placed under US guidance
to allow resuscitation and treatment. Normal saline was
administered with improvement in the patients’ symptoms.
The electrocardiogram was without ischemia, and a chest
radiograph was without acute pathology. Once the patient was
stabilized, a CT of the head, neck and chest showed thrombus
within the left innominate, right and left subclavians, and
superior vena cava with total occlusion of the distal superior
vena cava at the level of the cavo-atrial junction (Figure 6).
These correlated with the POCUS ndings and conrmed the
cause of SVC syndrome. After discussion with the patient’s
primary oncologist and his family, the decision was made to
transition the patient to comfort care measures and he was
transferred to the medical ward. He expired later that evening
due to respiratory arrest.
DISCUSSION
Our case represents a novel report in the medical
literature demonstrating the utility of POCUS in the ED as
the diagnostic modality of bilateral IJV thromboses. An added
benet of POCUS was demonstrated in our case – the directed
guidance of venous access to the appropriate, patent femoral
vein, which allowed treatment and resuscitation of the patient.
Upper extremity IV access was non-functional in this patient
due to obstruction from the SVC thrombosis, but the IVC was
found to be patent on US as well as the femoral vein distal,
which was cannulated using real-time ultrasound guidance.
SVC syndrome with unilateral IJV thrombosis is a rare
occurrence, with bilateral IJV thromboses being rarer still.
13
Literature searches via PubMed, Google Scholar, Cochrane
Figure 2. Ultrasound image demonstrating clot in right IJ (star).
IJ, internal jugular
Figure 3. Ultrasound image demonstrating clot in left IJ (star).
IJ, internal jugular
Volume XV, NO. 6 : September 2014 717 Western Journal of Emergency Medicine
Birch et al Ultrasound Diagnosis of Superior Vena Cava Thrombus
Review, and Ovid using the terms “bilateral internal jugular
vein thrombus,” “bilateral internal jugular vein thrombosis,”
and “bilateral internal jugular vein thromboses” revealed
multiple case reports demonstrating diagnosis of the thrombus
via CT, MRI and US; however, none of the articles reported
ED POCUS as the diagnostic modality. Few cases mentioned
US as the initial diagnostic modality and these were
radiologist-performed inpatient studies.
14
Risk factors for thrombosis of the SVC include a history
of malignancy, trauma, recent surgery, central venous access,
retropharyngeal or deep space neck infections, hypercoagulability
or known existing thrombi, and polycythemia. The more rapid the
compression of the vessel, the more quickly symptoms occur due
to lack of collateral vessel development. Physical exam ndings
of SVC syndrome most commonly include facial swelling with
venous engorgement of the neck, trunk and upper extremities,
dyspnea, orthopnea, cough, headache, nausea, and dizziness. Other
complaints include a palpable cord or mass, fever, chest pain, visual
disturbances, or seizures.
15-19
Upper body cyanosis tends to be a
Figure 4. Ultrasound image demonstrating clot in right axillary
vein (star).
Figure 5. Ultrasound image demonstrating clot in left axillary vein
(star).
late and ominous sign. Possible complications include pulmonary
embolus, thrombophlebitis, airway edema, pseudotumor cerebri,
superior sagittal sinus thrombosis, coma, and death.
Many diagnostic modalities exist including US, CT,
magnetic resonance imaging, and contrast venography. CT
is the gold standard and is the conrmatory radiographic
modality of choice.
20
As seen in this case, in the hands of
an appropriately trained emergency physician, POCUS
identied the extensive thromboses and helped plan the
course of action for vascular access in this critically ill patient
(femoral venous access). This information was timely and
invaluable in the resuscitation of this patient who was too sick
to travel to radiology for consultative imaging. Additionally,
a number of other bedside ultrasounds were performed
including an echocardiogram and an EFAST exam, giving
critical information about cardiac function, volume status,
and abdominal free uid – all within minutes of arrival. In our
patient, POCUS proved to be an invaluable tool.
Treatment typically consists initially of supportive care,
head elevation, rest, supplemental oxygen, anticoagulation with
the consideration of thrombolytics, clot retrieval. Some have
advocated the use of diuretics and glucocorticoids. Identifying
and treating the underlying cause, such as radiation and/or
chemotherapy for malignancy, or antibiotics for underlying
infection or thrombophlebitis, is also critical.
15-19
All unnecessary
catheters or other thrombogenic foci should be removed.
In summary, the rare occurrence of SVC syndrome due
to thrombosis of the SVC is most commonly associated
with vein cannulation, surgery, and malignancy. Although
the SVC itself may be difcult to image with US, the
diagnosis can be inferred readily at the bedside in the hands
of the emergency physician, as evidenced in this case by
identication of bilateral IJ and axillary thromboses. Though
Figure 6. Computed tomography image demonstrating lling
defect in SVC distal to central venous port tip (arrow).
SVC, superior vena cava
Western Journal of Emergency Medicine 718 Volume XV, NO. 6 : September 2014
Ultrasound Diagnosis of Superior Vena Cava Thrombus Birch et al
operator-dependent, POCUS is becoming an increasingly
available and used resource. As more providers become
adept at performing these exams in both stable and unstable
patients, our hope is that patient safety and outcomes
correspondingly improve.
Address for Correspondence: Aaron Birch, MD, Department
of Emergency Medicine, Madigan Army Medical Center, 9040
Fitzsimmons Ave, Tacmoa, WA 98431. Email: asbirch@gmail.
com.
Conicts of Interest: By the WestJEM article submission
agreement, all authors are required to disclose all afliations,
funding sources and nancial or management relationships that
could be perceived as potential sources of bias. The authors
disclosed none. The views expressed herein are solely those
of the author and do not represent the ofcial views of the
Department of Defense or Army Medical Department.
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... Imaging the superior vena cava (SVC) should be a part of any comprehensive two-dimensional (2D) transthoracic echocardiographic examination [1,2]. In patients with indwelling catheters used for hemodialysis, chemotherapy, or parenteral nutrition and those with pacemaker leads, imaging of the SVC and mainly its lower (juxta-atrial) part is a crucial noninvasive test to look for complications like vegetations and thrombus formation [3,4]. Also in patients with a mediastinal mass invading the SVC, ultrasound imaging of the SVC is imperative during echocardiographic examination [5]. ...
... Ultrasound evaluation of the superior vena cava during the 2D transthoracic echocardiographic examination will lead to an increase in diagnostic yield: from central catheter thrombosis to superior vena cava syndrome and invasion of the SVC by a tumor [3][4][5]; furthermore, the point-of-care ultrasound (POCUS) can allow clinicians, by evaluating the SVC from different windows during transthoracic echocardiography, to make a rapid diagnosis for example of a SVC thrombosis at the bedside in a patient in respiratory distress with SVC syndrome [4]. ...
... Ultrasound evaluation of the superior vena cava during the 2D transthoracic echocardiographic examination will lead to an increase in diagnostic yield: from central catheter thrombosis to superior vena cava syndrome and invasion of the SVC by a tumor [3][4][5]; furthermore, the point-of-care ultrasound (POCUS) can allow clinicians, by evaluating the SVC from different windows during transthoracic echocardiography, to make a rapid diagnosis for example of a SVC thrombosis at the bedside in a patient in respiratory distress with SVC syndrome [4]. ...
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Imaging the superior vena cava (SVC) during two-dimensional (2D) transthoracic echocardiographic examination is challenging and should be performed routinely. Here, we present a case where a lower (juxta-atrial) SVC mass was seen prolapsing into the right atrium by 2D transthoracic echocardiography; in this case, the imaging of the lower (juxta-atrial) SVC was done from the subcostal window. It was not possible to image the SVC from the suprasternal, right supraclavicular, left parasternal, or apical windows. CT scan of the chest with intravenous contrast was done in this case and showed an anterior mediastinal mass invading the SVC and prolapsing into the right atrium. CT-guided biopsy proved the mass to be a type B2 thymoma.
... Due to the accessibility and non-invasive nature, PoCUS has gained popularity in assessing SVC conditions in recent years. A case report published by Birch et al. demonstrated the utility of PoCUS in rapidly identifying an SVC syndrome associated with bilateral IJ vein thrombosis, facilitating timely clinical interventions in a critically ill patient [13]. Another study conducted by Blanco et al. underscored the utility of PoCUS in detecting mediastinal masses, assessing venous flow abnormalities, and guiding diagnostic interventions, thereby showcasing its potential as a valuable tool in the evaluation of patients presenting with SVC syndrome [5]. ...
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Background Superior vena cava (SVC) syndrome is an urgent condition arising from restricted blood flow through the SVC, often linked to factors like malignancy, thrombosis, or infections. Typically, confirmation of the diagnosis involves computed tomography. However, many patients experience respiratory distress and cannot lie supine. Given the increasing integration of point-of-care ultrasound in emergency medicine, it is important to be familiar with findings that are suggestive of this important condition. Case report In this case report, we highlight a young patient presenting to the emergency department with superior vena cava syndrome symptoms, successfully diagnosed using point-of-care ultrasound. Conclusion This case highlights the utility of point-of-care ultrasound based diagnosis of SVC syndrome and upper arm deep venous thrombosis in a patient with underlying malignancy which ultimately led to early involvement of relevant speciality for initiation of treatment.
... Although the SVC itself may be difficult to image with US, the diagnosis can be suspected indirectly at the bedside in the hands of a skilled emergency physician. Operator-dependent point-of-care US is becoming an increasingly available resource [13]. Contrast-enhanced CT venogram is the main diagnostic imaging modality and helps define the degree and cause of SVC obstruction, in addition to the collateral venous supply. ...
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Superior vena cava (SVC) syndrome represents clinical manifestations from obstruction of the SVC. It is an uncommon medical emergency that is commonly missed. SVC obstruction is usually caused by extraluminal compression by an intrathoracic mass or intraluminal obstruction from a thrombus. The latter is more common in patients with indwelling central venous catheters and pacemaker leads. Here, we present the case of a 53-year-old woman who presented to the emergency department (ED) with clinical features which were initially diagnosed as anaphylaxis and subsequently diagnosed as a case of SVC syndrome. In this case, diagnostic challenges in the ED and the potential role of cognitive bias are highlighted. In addition, we discuss the clinical manifestations and management of SVC syndrome.
... The superior vena cava syndrome is the clinical manifestation of gradual obstruction of blood flow in the superior vena cava [1,2]. Approximately 70% of these cases are in the context of malignancies [3,4]. ...
... This finding was attributed to mediastinal malignancy (37.5%) or the presence of central vein catheters (62.5%). In contrast, Birch et al. [11], stated that SVC syndrome resulting from SVC and internal jugular vein (IJV) thrombosis is rare. Rice et al. [12], in their study reported that, malignancy is the most common cause of the superior vena cava syndrome (60%); however, they reported an increasing incidence of benign etiologies of SVC syndrome due to the increasing use of intravascular devices [12]. ...
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... 2 The diagnosis of superior vena cava syndrome is built on clinical suspicion that needs to be confirmed with a CT scan or magnetic resonance imaging. 3 The POCUS could also be used for initial screening, 4 ...
... Diagnosis can be made indirectly by detection of thrombosis in the internal jugular vein or through flow voidance and nonpulsatile, non-phasic flow in the upper central veins [12][13][14]. Early detection is considered important because of the risk for pulmonary emboli when particles of the thrombus detach. ...
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