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Superior Vena Cava Syndrome
Updated: Dec 3, 2009
Introduction
Background
Superior vena cava (SVC) syndrome (SVCS) is characterized by gradual, insidious compression/obstruction of the superior vena cava. Although the syndrome can be life threatening, its presentation is often associated with a gradual increase in symptomatology. For this reason, diagnosis is often delayed until significant compression of the superior vena cava has occurred.
Pathophysiology
Extrinsic compression of the superior vena cava is possible because it has a thin wall coupled with a low intravascular pressure. Because the superior vena cava 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
Superior vena cava syndrome is chiefly associated with malignancy. Currently, more than 90% of patients with superior vena cava syndrome 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 superior vena cava syndrome, 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 superior vena cava syndrome continue to constitute a significant percentage. Still, superior vena cava syndrome occurs infrequently in the general population.
Mortality/Morbidity
Bronchogenic carcinoma (CA) accounts for more than 80% of cases of superior vena cava syndrome. Even when treated with radiation, only 10% of these patients are alive 30 months after presentation. However, patients with superior vena cava syndrome due to a malignant cause survive only 30 days without radiation.
Race
Superior vena cava syndrome has no racial predilection. However, because of poorer access to adequate health care, some socioeconomic groups have a disproportionately greater representation.
Age
Because most superior vena cava 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 superior vena cava syndrome. The age range reported in one study was 18-76 years, with a mean age of 54 years.1
Clinical
History
Early in the clinical course of superior vena cava (SVC) syndrome (SVCS), few, if any, signs or symptoms may be observed. 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. Other symptoms include the following:
- 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 (as is seen in the image below).
Patient with a 4-week history of increasing facial edema and known lung cancer.
- The degree of jugular venous distention varies.
- 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 superior vena cava syndrome 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 superior vena cava syndrome is bronchogenic carcinoma, which accounts for nearly 80% of cases.
- Lymphoma accounts for approximately 15% of cases.
- Other cases have various causes, including infectious and catheter-related etiologies. Increasingly, dialysis catheters and pacemaker leads are becoming associated with superior vena cava syndrome due to thrombosis.
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References
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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].
Leggio L, Abenavoli L, Vonghia L, et al. Superior vena cava thrombosis treated by angioplasty and stenting in a cirrhotic patient with peritoneovenous shunt. Ann Thorac Cardiovasc Surg. Feb 2008;14(1):60-2. [Medline].
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Madan AK, Allmon JC, Harding M, et al. Dialysis access-induced superior vena cava syndrome. Am Surg. Oct 2002;68(10):904-6. [Medline].
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Further Reading
Keywords
superior vena cava syndrome, SVCS, bronchogenic carcinoma, endoprostheses, malignancy, obstruction of superior vena cava, compression of superior vena cava, treatment, diagnosis, symptoms
