Skip to Main Content

Key Features

  • A rare disorder caused by partial or complete obstruction of the superior vena cava

  • Most frequent causes

    • Neoplasms, such as lymphomas, primary malignant mediastinal tumors, or carcinoma of the lung with direct extension (over 80%)

    • Chronic fibrotic mediastinitis, either of unknown origin or secondary to tuberculosis, histoplasmosis, pyogenic infections, or drugs (especially methysergide)

    • Deep venous thrombosis

    • Aneurysm of the aortic arch

    • Constrictive pericarditis

Clinical Findings

  • Swelling of the neck and face and upper extremities

  • Headache

  • Dizziness, stupor, syncope

  • Visual disturbances

  • Bending over or lying down accentuates symptoms

  • Dilated anterior chest wall veins and/or collateral veins

  • Facial flushing

  • Brawny edema and cyanosis of the face, neck, and arms

  • Cerebral and laryngeal edema

  • Venous pressure

    • Elevated in the arm (often > 20 cm H2O)

    • Normal in the leg

Diagnosis

  • Venous pressure

    • Elevated (often more than 20 cm H2O) in the arm

    • Normal in the leg

  • Bronchoscopy is often performed, since lung cancer is a common cause

  • Transbronchial biopsy is relatively contraindicated because of venous hypertension and the risk of bleeding

  • Chest radiographs and a CT scan will define the location and often the nature of the obstructive process

  • Contrast venography or magnetic resonance venography (MRV) will map out the extent and degree of the venous obstruction and the collateral circulation

  • Brachial venography or radionuclide scanning following intravenous injection of technetium Tc-99m pertechnetate demonstrates a block to the flow of contrast material into the right heart and enlarged collateral veins

  • These techniques also allow estimation of blood flow around the occlusion as well as serial evaluation of the response to therapy.

Treatment

  • Conservative measures such as elevation of the head of the bed and lifestyle modification to avoid bending over are useful

  • Occasionally, anticoagulation is needed, while thrombolysis is rarely needed

  • Balloon angioplasty of the obstructed caval segment combined with stent placement

    • Procedure of choice

    • Provides prompt relief of symptoms

  • Urgent treatment for neoplasm consists of

    • Cautious use of intravenous diuretics

    • Mediastinal irradiation

  • Chemotherapy added for lymphoma or small-cell carcinoma

  • In cases where the thrombosis is secondary to an indwelling catheter, thrombolysis may be attempted

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.