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SVC Syndrome

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  • • 80-90% caused by malignant tumors

    • -Lung cancer (90%)

      -Thymoma

      -Hodgkin disease

      -Lymphosarcoma

      -Metastatic melanoma

      -Breast or thyroid cancer

    • Benign tumors unusual

    • -Substernal goiter

      -Large benign mediastinal masses

      -Atrial myxoma

    • The following thrombotic conditions are unusual causes:

    • -Polycythemia

      -Mediastinal infection

      -Indwelling catheters

    • Trauma may produce acute obstruction

    • Clinical presentation varies with: abruptness of onset, extend of occlusion, collateral pathways

    • Venous pressure in arms/head: 200- 500 mm H2O

    • Severity of symptoms correlates with pressure

    • Cerebral edema can occur with complete obstruction

    • Symptoms milder with patent azygous vein

    • Azygous venous flow can increase to 35% of venous return (normal is 11%)

    • Thrombus can propagate proximally to innominate and axillary veins

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Epidemiology

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  • • 80-90% caused by malignant tumors

    • Lung cancer 90% of malignant tumors

    • Incidence is 3-5% in lung cancer patients

    • Male:female ratio is 5:1

    • Malignant SVC obstruction: 35% have thrombosis of innominate or axillary vein, 15% have complete caval obstruction without thrombosis, 50% have partial SVC obstruction

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Symptoms and Signs

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  • • Nasal congestion often earliest symptom

    • Swelling in face, arms, shoulders

    • Blue/purple discoloration of skin

    • Headache, nausea, dizziness, vomiting, vision changes, drowsiness, stupor

    • Cough, hoarseness, dyspnea (edema of vocal cords)

    • Symptoms may be worse when patient lies on back or bends forward

    • Esophageal varices may develop: GI bleeding

    • Neck, arm veins may be visibly distended

    • Fibrosing mediastinitis: Early morning edema of face and head

    • Unilateral symptoms suggest ipsilateral occlusion

    • Effort thrombosis or axillary vein and innominate vein obstruction from elongation and buckling of innominate artery in unilateral cases

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Imaging Findings

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  • Chest film: May show right upper lobe lung lesion or right paratracheal mass

    Venography: Determines location and extent of obstruction

    Interosseous azygography: Useful to determine patency of azygous vein

    Aortography or CT scan: Excludes aortic aneurysm

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  • • Measure upper extremity venous pressure: > 350 mm H2O

    • Location/extent of obstruction determined by venography

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Rule Out

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  • • Angioneurotic edema

    • Congestive heart failure

    • Constrictive pericarditis

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  • • Physical exam

    • Chest x-ray

    • Chest CT scan

    • Venography

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  • • Etiology of obstruction determines treatment

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Cancer

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  • • Diuretics, restriction, head elevation prompt radiation or chemotherapy

    • Often subsides at 7-10 days of treatment

    • Fibrinolytics or anticoagulation can be considered but rule out metastatic cerebral metastases with CT/MRI

    • Percutaneous stents allows good immediate drainage; long-term results unknown

    • Tissue diagnosis often needed for therapy (via fine-needle aspiration, bronchoscopy, mediastinoscopy, or thoracotomy)

    • Avoid operation if acutely obstructed due to high bleeding rate

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Benign Tumors

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  • • Surgical excision for incomplete obstruction

    For complete obstruction: Many improve without treatment, others require SVC bypass

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