Superior Vena Cava Syndrome

A 42-year-old woman complained of facial puffiness, fullness, and redness for 2 weeks, which were gradually becoming more severe. She also noticed a sensation of “heaviness” in her head. The patient had no significant past medical history. She denied having a cough, shortness of breath, hoarseness, allergies of any kind, and neurologic deficits. Her weight and appetite were unchanged. She had smoked a pack of cigarettes every day for the past 20 years.

Significant physical examination findings were the facial swelling and plethora and fullness of the neck veins. Dilated and tortuous veins were present on the anterior chest wall. A chest film revealed a right parahilar and apical mass; these findings were confirmed on a CT scan of the chest. A diagnosis of superior vena cava syndrome (SVCS) was made, and treatment with intravenous corticosteroids was initiated.

Bronchoscopy was performed, and a biopsy of a specimen from the right parahilar mass showed small cell carcinoma of the lung. Chemotherapy was instituted.

Drs Navin Verma, Terence M. Brady, and Sonia Arunabh of Queens, NY, write that SVCS occurs when the superior vena cava is obstructed by extrinsic compression, tumor invasion, or a thrombus that impairs venous drainage of the head, neck, and upper extremities. Onset usually is gradual and progresses to cause the classic signs and symptoms of SVCS: varying degrees of edema of the head, neck, and upper torso; dyspnea; orthopnea; hoarseness; headache; dizziness; and chest pain. The symptoms improve during the course of the day as gravity facilitates venous drainage.

If you suspect SVCS, examine the patient with facial plethora for dilatation of the superficial venous channels of the upper torso. CT or MRI will define the anatomy and show the extent of occlusion and the presence of collateral circulation or thrombus formation. MRI will provide greater detail of mediastinal structures in multiple planes. Biopsy of a specimen obtained during bronchoscopy or mediastinoscopy helps determine whether chemotherapy or radiation therapy is indicated.

Drs Verma, Brady, and Arunabh add that small cell lung carcinoma is the most common cause of SVCS. Other causes include lymphoma and thrombus formation from a central venous catheter in the superior vena cava.

Corticosteroids and diuretics are used initially to relieve inflammation, soft-tissue edema, and respiratory distress. Thrombolytics and anticoagulants can be tried to treat thrombus formation.

Expandable wire stents also can be placed into the obstructed or stenosed portion of the vena cava. Surgical bypass is used only if chemotherapy, radiation therapy, and stent placement fail or are not therapeutic options for the patient.