The Superior Vena Cava Syndrome

Abstract

The superior vena cava syndrome (SVCS) refers to a group of symptoms caused by obstruction or compression of the superior vena cava. Malignant causes represents the majority of all cases of SVCS (bronchogenic carcinoma, lymphoma, metastatic breast carcinoma, primary mediastinal tumors, acute leukemias). SVCS may be due to iatrogenic causes such as presence of intravascular devices (e.g., permanent central venous access catheters, pacemaker/defibrillator leads). Syphilis and tuberculosis have also been known to cause SVCS.

The clinical presentation of SVCS may be acute or subacute. The most common presenting symptoms and signs are dyspnea, facial edema, jugular venous distention, upper body plethora, cough, orthopnea, stridor, chest pain, cyanosis, positive Pemberton’s sign, dysphagia, visual impairment, lethargy, and headache.

Diagnostic studies include chest X-ray, CT and MRI scans, venography, and nuclear flow studies. Invasive procedures, including bronchoscopy, percutaneous needle biopsy, mediastinoscopy, and even thoracotomy, can be performed.

Several methods of treatment are available. In an acute setting with severe symptoms, endovascular stenting by an interventional radiologist may provide relief of symptoms. Radiotherapy is the primary treatment for SVCS caused by non-small cell lung cancer and other metastatic solid tumors. Chemotherapy is effective in small cell carcinoma of the lung, lymphoma, and germ cell tumor. Surgery is helpful for patients in whom a benign process is the cause. Glucocorticoids may be useful at lymphomas but they have no benefit in lung cancer. Diuretics with a low-salt diet and head elevation can be effective.