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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Facial swelling, headache, and arm edema

  • Prominent venous pattern may be seen over the anterior chest wall

  • Associated conditions may aid in the diagnosis, such as lung cancer, lymphoma, and indwelling catheter or pacemaker

  • Venography is diagnostic

    • – Magnetic resonance imaging, ultrasound, and CT are alternates

GENERAL CONSIDERATIONS

  • A relatively rare condition in which the thin-walled superior vena cava is compressed extrinsically by neoplasms, fibrosis due to pulmonary infections or drug toxicity, or aneurysm of the aortic arches; or it may be thrombosed due to extension from tributary vein thrombosis or indwelling catheters

  • Causative factors are changing (from lung cancer) with increased use of indwelling catheters

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Acute or subacute symptoms

  • Headache, dizziness, visual disturbances, stupor

  • Symptoms accentuated by bending over or lying down

PHYSICAL EXAM FINDINGS

  • Swelling and flushing of the neck and face progressing to cyanosis

  • Dilated cutaneous veins of the upper chest and neck

  • Eventually, brawny edema of the face, neck, and arms develops

  • Laryngeal edema leads to respiratory insufficiency

DIFFERENTIAL DIAGNOSIS

  • Differential diagnosis of causes of superior vena cava syndrome includes:

    • – Lung cancer

    • – Lymphoma

    • – Indwelling catheters and pacemaker leads

    • – Fibrosing mediastinitis

    • – Thoracic outlet syndrome

    • – Retrosternal goiter

DIAGNOSTIC EVALUATION

IMAGING STUDIES

  • Chest x-ray: localizes the site of obstruction

  • CT scan or MRI: delineates the anatomy

  • Phlebography: outlines the venous pattern and collaterals

DIAGNOSTIC PROCEDURES

  • Venous pressure measurements in the neck or arm (greater than in the leg)

TREATMENT

CARDIOLOGY REFERRAL

  • If syndrome is believed to be due to pacemaker catheters

HOSPITALIZATION CRITERIA

  • Marked symptoms

  • Need for corrective procedure such as pacemaker lead change

MEDICATIONS

  • Cautious use of diuretics

  • Heparin or thrombolysis with thrombosis

THERAPEUTIC PROCEDURES

  • Mediastinal irradiation for neoplasm

  • Removal of venous catheters or pacemaker leads

  • Balloon venoplasty and stent placement

SURGERY

  • If mediastinal fibrosis is present, surgical excision may be necessary

  • Some catheters need to be removed surgically

MONITORING

  • Chest x-ray to assess response to therapy

DIET AND ACTIVITY

  • Low-sodium diet until obstruction is resolved

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Resolution of the obstruction

  • Transfer to comfort care

FOLLOW-UP

  • Depends on the cause of the obstruction

COMPLICATIONS

  • Death from increased intracranial pressure ...

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