AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Superior vena cava syndrome (SVCS) is characterized by gradual, insidious compression/obstruction of the superior vena cava (SVC). Although the syndrome can be life threatening, its presentation is often associated with a gradual increase in symptomatology.

Pathophysiology

Extrinsic compression of the SVC is possible because it has a thin wall coupled with a low intravascular pressure. Because the SVC is surrounded by rigid structures, it is relatively easy to compress. The low intravascular pressure also allows for the possibility of thrombus formation, such as catheter-induced thrombus.

The subsequent obstruction to flow causes an increased venous pressure, which results in interstitial edema and retrograde collateral flow.

Frequency

United States

SVCS is associated chiefly with malignancy. Currently, more than 90% of patients with SVCS have an associated malignancy as the cause. This contrasts with studies in the early 1950s in which a large proportion of cases were nonmalignant. Infectious causes (eg, syphilis, tuberculosis) have decreased because of improvements in antibiotic therapy. Of the nonmalignant causes of SVCS, thrombosis from central venous instrumentation (catheter, pacemaker, guidewire) is an increasingly common event, especially as these procedures become more common.

International

In developing countries, nonmalignant causes of SVCS continue to constitute a significant percentage. Still, SVCS occurs infrequently in the general population.

Mortality/Morbidity

Bronchogenic carcinoma (CA) accounts for more than 80% of cases of SVCS. Even when treated with radiation, only 10% of these patients are alive 30 months after presentation. However, patients with SVCS due to a malignant cause survive only 30 days without radiation.

Race

SVCS has no racial predilection. However, because of poorer access to adequate health care, some socioeconomic groups have a disproportionately greater representation.

Age

• Because the majority of SVC syndromes are caused by bronchogenic carcinoma, the age distribution is skewed strongly toward elderly persons.
• Nonmalignant causes, as well as lymphoma, tend to affect younger people more than malignancy-associated SVCS.
• The age range reported in one study was 18-76 years, with a mean age of 54 years (Chen, 1990).

CLINICAL

Section 3 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

• In the early clinical course, few, if any, signs or symptoms of superior vena cava syndrome (SVCS) may be manifested.
• Typically, symptoms accelerate as the underlying malignancy increases in size and/or invasiveness.
• Dyspnea is the most common symptom, followed by trunk or extremity swelling.
• Facial swelling
• Cough
• Orthopnea
• Headache
• Nasal stuffiness
• Light-headedness

Physical

• Physical examination often reveals facial or upper extremity edema. The degree of facial edema has been described as facial engorgement (see Image 1).
• The degree of jugular venous distention is variable.
• Other markers of lung malignancy, such as Horner syndrome, paralysis of the vocal cords, and paralysis of the phrenic nerve, are rarely present.

Causes

• Today, the most common etiology of SVCS is related to malignancy.
• Prior to modern antibiotics, infectious causes including syphilis, tuberculosis, and fungi occurred with almost equal frequency.
• The most common cause of malignancy-related SVCS is bronchogenic carcinoma, which accounts for nearly 80% of cases.
• Lymphoma accounts for approximately 15% of cases.
• Other cases have a variety of causes, including infectious and catheter-related etiologies. Increasingly, dialysis catheters and pacemaker leads are becoming associated with SVCS due to thrombosis.

DIFFERENTIALS

Section 4 of 11  

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Other Problems to be Considered

Airway obstruction

WORKUP

Section 5 of 11  

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• Follow-up
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Lab Studies

• The diagnosis of superior vena cava syndrome (SVCS) is often made on clinical grounds alone, combining clinical presentation with an often-obtained history of thoracic malignancy.

Imaging Studies

• Plain radiographs are often helpful, showing a mediastinal mass in most patients (see Image 2).
• When in doubt, venography can aid in the diagnosis, but this is usually not necessary.
• Thoracic CT scanning is helpful, but remember that the histologic diagnosis is important in initiating therapy.

TREATMENT

Section 6 of 11  

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• Follow-up
• Miscellaneous
• Multimedia
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Prehospital Care

• Prehospital caregivers are aware of the superior vena cava syndrome (SVCS) diagnosis only on occasion.
• The usual attention to airway, breathing, and circulation (ABCs) is required.
• SVCS only rarely manifests as a life-threatening entity; therefore, other causes for the symptomatology must be sought.

Emergency Department Care

• SVCS only rarely manifests as an acute emergency.
• Attention to the ABCs is essential.
• If patients are allowed to sit upright, they may experience some relief of the usual dyspnea.
• Stabilize the airway, as needed, and consider steroids.
• If cerebral/airway edema is present, consider diuretics; however, diuretics have not shown consistent benefit in the emergency department (ED).
• Endovascular shunts are being used increasingly often, as are thrombolytics if a thrombotic cause is present.
• After a tissue diagnosis, radiation and chemotherapy may be initiated.

Consultations

Over the last 10 years, considerable experience with endovascular stenting of superior vena cava syndrome has been achieved. At many centers, endoprostheses have become the initial choice for palliative treatment of superior vena cava syndrome.

• Emergent consultation with radiation therapy may be necessary, depending upon the acuteness of the presentation.
• Because most causes of SVCS are related to lung cancer, a pulmonary consultation is essential.
• Generally, considering the diagnosis in the ED is important.
• • If the diagnosis is made de novo in the ED, only rarely is emergent consultation necessary.
  • Exceptions include sudden airway compromise or acute SVC thrombosis, which may occur from an indwelling catheter.

MEDICATION

Section 7 of 11  

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Steroids and diuretics have been the mainstays of ED management. However, superior vena cava syndrome (SVCS) rarely presents as an acute life-threatening emergency. As such, considering the diagnosis may be more important than the actual definitive care when making therapeutic decisions.

Drug Category: Glucocorticoids

These agents decrease the inflammatory response to tumor invasion and edema surrounding the tumor mass. They have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Drug Name	Prednisone (Deltasone, Orasone, Sterapred)
Description	Useful in treatment of inflammatory and autoimmune reactions. By reversing increased capillary permeability and suppressing polymorphonuclear neutrophil (PMN) activity, may decrease inflammation.
Adult Dose	5-60 mg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolve
Pediatric Dose	4-5 mg/m2/d PO; alternatively, 1-2 mg/kg PO qd; taper over 2 wk as symptoms resolve
Contraindications	Documented hypersensitivity; viral disease; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections
Interactions	Estrogens may decrease clearance; digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur

Drug Category: Diuretics

These agents may decrease venous return to the heart by decreasing preload, relieving the increased pressure in the SVC.

FOLLOW-UP

Section 8 of 11  

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• Follow-up
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• References

Complications

• Total SVC obstruction
• • Fortunately, this is rare.
  • Potential causes include indwelling catheters.
  • Thrombolysis must be considered.
• Airway compromise is unusual but may result from extrinsic compression of the SVC or the trachea by the tumor mass.

Prognosis

• Superior vena cava syndrome (SVCS) is associated with malignancy.
• • The prognosis for relief of SVCS symptoms is good with radiation therapy.
  • Symptoms usually decrease within 1 month of the onset of radiation therapy.
  • However, the ultimate prognosis is associated with the underlying malignancy itself.
• The prognosis for SVCS not associated with malignancy is excellent because most of these causes are infectious and respond to appropriate antibiotic therapy.
• Recently, management of SVCS by internal jugular to femoral vein bypass has been described (Dhaliwal, 2006). This may help improve symptoms of patients with malignancy.

MISCELLANEOUS

Section 9 of 11  

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• Introduction
• Clinical
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• Workup
• Treatment
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• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Potential medical/legal pitfalls include not considering the diagnosis in patients with known lung cancer. Their symptoms of increasing shortness of breath may be from SVCS. Symptoms may be relieved with radiation therapy.
• In patients in whom the diagnosis of lung cancer is not known, patients' sensations of arms swelling, facial fullness, and related symptoms may tend to be discounted. An excellent opportunity may exist to positively affect a patient's prognosis if the diagnosis of SVCS is entertained and the underlying cause of cancer is considered.

Special Concerns

• Special concerns include the need to be sensitive to patients' probable diagnosis of lung cancer.
• • In patients in whom the diagnosis has not been made, this represents a unique occasion for the clinician to approach the patient with care.
  • Special care must be taken to ensure that the explanation of the patient's symptoms is not rushed.

MULTIMEDIA

Section 10 of 11  

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• Miscellaneous
• Multimedia
• References

Media file 1:  Patient with a 4-week history of increasing facial edema and known lung cancer.
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Media type:  Photo

Media file 2:  Chest radiograph of a patient with known superior vena cava syndrome and bronchogenic carcinoma.
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Media type:  X-RAY

Media file 3:  Chest radiograph of a 50-year-old woman with complaint of shortness of breath and facial swelling. No previous history of cancer but 30 pack-year history.
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Media type:  X-RAY

Media file 4:  CT scan of the same 50-year-old woman in Image 3 with an initial presentation of shortness of breath and facial swelling. This shows a large tumor mass in the right mediastinum nearly surrounding the right main stem bronchus and partially occluding the superior vena cava.
	View Full Size Image
Media type:  CT

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• Abner A. Approach to the patient who presents with superior vena cava obstruction. Chest. Apr 1993;103(4 Suppl):394S-397S. [Medline].
• Armstrong BA, Perez CA, Simpson JR, Hederman MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys. Apr 1987;13(4):531-9. [Medline].
• Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care. Jul 1992;1(1):54-64. [Medline].
• Bauset R. Pacemaker-induced superior vena cava syndrome: a case report and review of management strategy. Can J Cardiol. Nov 2002;18(11):1229-32. [Medline].
• Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg. Aug 1990;160(2):207-11. [Medline].
• Courtheoux P, Alkofer B, Al Refai M, et al. Stent placement in superior vena cava syndrome. Ann Thorac Surg. Jan 2003;75(1):158-61. [Medline].
• Dhaliwal RS, Das D, Luthra S, et al. Management of superior vena cava syndrome by internal jugular to femoral vein bypass. Ann Thorac Surg. Jul 2006;82(1):310-2. [Medline].
• Lanciego C, Chacon JL, Julian A, et al. Stenting as first option for endovascular treatment of malignant superior vena cava syndrome. AJR Am J Roentgenol. Sep 2001;177(3):585-93. [Medline].
• Link MS, Pietrzak MP. Aortic dissection presenting as superior vena cava syndrome. Am J Emerg Med. May 1994;12(3):326-8. [Medline].
• Madan AK, Allmon JC, Harding M, et al. Dialysis access-induced superior vena cava syndrome. Am Surg. Oct 2002;68(10):904-6. [Medline].
• Queen JR, Berlin J. Superior vena cava syndrome. J Emerg Med. Aug 2001;21(2):189-91. [Medline].

Article Last Updated: Jan 16, 2007