Superior Vena Cava Syndrome

Approximately 50% of bronchogenic carcinomas involve the mediastinal lymph nodes. Severe, extensive involvements may encase, compress, or invade the superior vena cava,resulting in superior vena cava syndrome. Note the venous congestion due to obstruction to blood drainage from the head and upper extremities. This patient is marked for the purposes of surgical resection. Select the button below to see the surgical view. Note the extensive infiltration of mediastinal structures.

Superior vena cava (SVC) syndrome was first described in 1757 by William Hunter. SVC syndrome results from the partial or complete obstruction of the SVC. It occurs most often in men between the ages of 50 and 70 years. The major signs and symptoms of SVC include facial fullness or flushing, headache, dyspnea, and cough. Less common complaints include edema of the upper extremities, pain, dysphagia, and syncope. Physical findings may include prominent distended and tortuous venous systems in the face, neck and upper trunk, papilledema, facial cyanosis and pleural effusion .

The most common causes of this syndrome are extrinsic compression of the superior vena cava and intraluminal venous thrombosis . Up to 82% of cases result from obstruction caused by bronchogenic carcinoma. Less common causes include granulomas (Histoplasmosis and tuberculosis), lymphoma, and thrombosis of the SVC due to intravascular foreign bodies (pacemaker leads, central venous catheters).

Because of the risks associated with SVC obstruction (central edema, airway compromise), it is important to obtain a diagnosis and institute therapy in a timely fashion. SVC obstruction is sometimes suggested on plain radiographs as a widened mediastinum. The use of contrast enhanced CT is much more specific and can demonstrate collateral veins. Depending on the clinical presentation, tissue diagnosis can be made by bronchoscopy, fine needle aspiration biopsy (FNAB) or mediastinotomy. The procedure of choice is based on the clinical situation and risks. Caution is necessary when performing venipuncture, lymph node biopsy, and bronchoscopy because there may be profuse bleeding due to the high venous pressures in the head and neck.

When SVC syndrome is associated with malignancy, is usually fatal within 6-7 months without treatment. Overall survival and symptom resolution depend on the ability to treat the underlying disease. Symptom relief can be obtained through simple measures such as supplemental oxygen, diuretics, and steroids. Chemotherapy, radiation therapy or both can effectively relieve the signs and symptoms when arising from malignant disease. Surgical intervention is not recommended except in selected individuals with fibrosing mediastinitis.

superior vena cava syndrome. Note the distended neck veins and purple discoloration

The most common causes of superior vena cava syndrome are extrinsic compression of the superior vena cava and intraluminal venous thrombosis

Selected radiographic images (x-ray and CT) from a patient with superior vena cava syndrome

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