Superior vena cava syndrome

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Synonyms and Related Keywords: SVC syndrome

Overview

Superior vena cava syndrome (SVCS) is an array of symptoms caused by the impairment of blood flow through the superior vena cava (SVC) to the right atrium. Symptoms that prompt suspicion of this syndrome include dyspnea, coughing, and swelling of the face, neck, upper trunk, and extremities. In rare instances, patients may complain of hoarseness, chest pain, dysphagia, and hemoptysis. Physical signs that may be noted on presentation are neck vein distention, thoracic vein distention, edema of the face or upper extremities, plethora, and tachypnea. Rarely, cyanosis, Horner syndrome, and a paralyzed vocal cord may also be present.

SVCS is usually a sign of locally advanced bronchogenic carcinoma. Survival depends on the status of the patient’s disease. When small cell bronchogenic carcinoma is treated with chemotherapy, the median survival times with or without SVCS are almost identical (42 weeks or 40 weeks, respectively). The 24-month survival rate is 9% in patients without SVCS and 3% in those with the syndrome. When the malignancy is treated with radiation therapy, 46% of patients who have non-small cell lung cancer experience relief of symptoms compared with 62% of patients who have small cell bronchogenic carcinoma. The 2-year survival rate of 5% is almost the same for both groups.

Most non-Hodgkin lymphoma patients with SVCS respond to appropriate chemotherapy or to combined modality regimens.

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

Since SVCS was first described by William Hunter in 1757, the spectrum of underlying conditions associated with it has shifted from tuberculosis and syphilitic aneurysms of the ascending aorta to malignant disorders. Almost 95% of SVCS cases described in published modern series are due to cancer; the most common cause is small cell bronchogenic carcinoma, followed by squamous cell carcinoma of the lung, adenocarcinoma of the lung, non-Hodgkin lymphoma, and large cell carcinoma of the lung. A nonmalignant cause of SVCS in cancer patients is thrombosis that is associated with intracaval catheters or pacemaker wires. A rare cause of SVCS is fibrosing mediastinitis, either idiopathic or associated with histoplasmosis. Additional rare causes of SVCS include metastatic germ cell neoplasms, metastatic breast cancer, colon cancer, Kaposi sarcoma, esophageal carcinoma, fibrous mesothelioma, Behçet syndrome, thymoma, substernal thyroid goiter, Hodgkin lymphoma, and sarcoidosis.

Knowledge of the anatomy of the SVC and its relationship to the surrounding lymph nodes is essential to understanding the development of the syndrome. The SVC is formed by the junction of the left and right brachiocephalic veins in the mid third of the mediastinum. The SVC extends caudally for 6 to 8 cm, coursing anterior to the right mainstem bronchus and terminating in the superior right atrium, and extends anteriorly to the right mainstem bronchus. The SVC is joined posteriorly by the azygos vein as it loops over the right mainstem bronchus and lies posterior to and to the right of the ascending aorta. The mediastinal parietal pleura is lateral to the SVC, creating a confined space, and the SVC is adjacent to the right paratracheal, azygous, right hilar, and subcarinal lymph node groups. The vessel itself is thin-walled, and the blood flowing therein is under low pressure. Thus, when the nodes or ascending aorta enlarge, the SVC is compressed, blood flow slows, and complete occlusion may occur.

The severity of the syndrome depends on the rapidity of onset of the obstruction and its location. The more rapid the onset, the more severe the symptoms because the collateral veins do not have time to distend to accommodate an increased blood flow. If the obstruction is above the entry of the azygos vein, the syndrome is less pronounced because the azygous venous system can readily distend to accommodate the shunted blood with less venous pressure developing in the head, arms, and upper thorax. If the obstruction is below the entry of the azygos vein, more florid symptoms and signs are seen because the blood must be returned to the heart via the upper abdominal veins and the inferior vena cava, which requires higher venous pressure.

One study suggested that the general recruitment of venous collaterals over time may lead to remission of the syndrome, although the SVC remains obstructed

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.[1]

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.

Vital Signs

Skin

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.

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. [2] [3] [4]

Surgery and Device Based Therapy

Stenting may be neccessary to preserve patency.

References

  1. Beeson, Michael S. "Superior Vena Cava Syndrome". Retrieved 2008-03-24.
  2. Nieto AF, Doty DB. Superior vena cava obstruction: clinical syndrome, etiology, and treatment. Curr Probl Cancer 1986; 10:441-84.
  3. Yellin A, Rosen A, Reichert N, Lieberman Y. Superior vena cava syndrome. The myth--the facts. Am Rev Respir Dis 1990; 141:1114-8.
  4. Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990; 160:207-11.

See also

Acknowledgements

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

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