Superior vena cava syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Also known as: SVC syndrome

Overview

Superior vena cava (SVC) syndrome is the obstruction of the superior vena cava resulting in diminished venous return from the head, neck and upper extremities.

Etymology

The Scottish obstetrician and anatomist, William Hunter, first described the entity in 1757, noting it as a complication of a syphilitic aortic aneurysm.

Epidemiology and Demographics

Most SVC syndromes in the present day are related to malignancy. An underlying malignancy is found in approximately 90% of patients.

Pathophysiology & Etiology

Collaterals form over time via the mammary, vertebrals, azygous and lateral thoracic veins, so despite almost or complete occlusion of the SVC venous drainage still can occur. Cerebral edema and obtundation with raised intracranial pressure is, therefore, rare. However, if this rare presentation is noted or if the airway is compromised, SVC syndrome represents a medical emergency.

Natural History

In the past, SVC syndrome was a medical emergency and empiric radiation was given to shrink the tumor. With the advent of better medical therapy for some lung cancers and lymphoma and the low morbidity associated with diagnostic procedures, this approach has fallen out of favor.

Signs and symptoms

The most common symptoms are these:

• Dyspnea
• Cough
• Swollen face, neck, upper body, and arms.

Less common symptoms include the following:

• Hoarse voice.
• Chest pain.
• Problems swallowing and/or talking.
• Coughing up blood.
• Swollen veins in the chest or neck.
• Bluish color to the skin.
• Drooping eyelid.
• Headache
• Facial plethora
• Lightheadedness

Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.

Diagnosis

The current strategy is to embark on a search for the primary source of the tumor. Start with a chest x-ray as a screening tool followed by a CT scan with contrast to further define the anatomy, extent of compression and to guide biopsy if a diagnosis cannot be made less invasively, e.g. thoracentesis, sputum, lymph node, and bone marrow biopsy.

Differential Diagnosis

The leading cancers associated with SVC syndrome are bronchogenic lung cancer (particularly small cell lung cancer), breast cancer, and lymphoma.

Benign causes of SVC syndrome include mediastinal fibrosis, histoplasmosis, radiation therapy complications, retrosternal goiter, Behcet’s syndrome, and thrombosis due to indwelling catheters or pacemakers. In the past, Tuberculosis and Syphilis were major players.

History and Symptoms

The presentation depends on the degree of occlusion and the rapidity with which it develops. The most common symptoms are dyspnea, facial swelling/fullness (suffusion) and cough. The most common signs are venous distention of the neck and chest wall and facial edema. Less commonly, chest pain, dysphagia, proptosis, hemoptysis, glossal edema, hoarseness, and headache are described. Symptoms get worse with leaning forward, coughing or lying down. Typically symptoms are present for >3 months before diagnosis.

Physical Examination

Appearance of the Patient

Pemberton’s Sign: suffusion, plethora, or duskiness that develop upon elevation of the arms above the head in patients with SVC syndrome.

Skin

• 

  Distension of veins on the torso of a patient with SVC syndrome

The skin of the face may have plethora.

There may be distension of veins on the torso.

Ear Nose and Throat

There is swelling of the face.

Laboratory Findings

Chest X Ray

This is a useful test to exclude lung cancer

MRI and CT

Useful in evaluating source and extent of a neoplasm.

• 

  Collaterals on CT scan in a patient with SVC syndrome

Other Diagnostic Studies

Biopsy may be neccessary to evaluate the underlying cause.

Risk Stratification and Prognosis

SVC syndrome is rarely fatal, and the prognosis is generally related to the prognosis of the underlying malignancy rather than the presence/absence of venous obstruction.

Treatment

Pharmacotherapy

Acute Pharmacotherapies

The goal of therapy is to relieve the obstructive symptoms and address the underlying process. Removal of nonessential central lines and upright posture are initial measures. Diuretics, steroids, radiation and chemotherapy are used depending on clinical scenario. In the case of compression, anticoagulation and stent placement are accepted means of attempting to assure long-term patency and prevent propagation.

Surgery and Device Based Therapy

Stenting may be neccessary to preserve patency.

See also

• Pemberton's sign

Acknowledgements

Source of Initial Content: Morning report notes prepared by Dr. Duane Pinto, C. Michael Gibson, M.S., M.D.