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Superior Vena Cava Obstruction
Aka: Superior Vena Cava Obstruction, Superior Caval Vein Obstruction, Superior Vena Cava Occlusion, Superior Vena Cava Syndrome, SVC Syndrome- See Also
- Pathophysiology
- Superior Vena Cava Obstruction to flow
- Internal Vascular Invasion (e.g. Vena cava thrombosis , tumor infiltration
- External Compression (e.g. right lung or Mediastinal Mass)
- Mediastinal Mass gradually compresses superior vena cava, blocking entry into right atrium
- Results in retrograde flow and regional edema
- Superior Vena Cava Obstruction Compensation
- SVC is a thin walled, low pressure large vein 2 cm in width and 4-6 cm in diameter
- High venous pressures within SVC may be compensated with collateral circulation over 1-2 weeks
- Example: Azygous vein may provide some collateral drainage
- Superior Vena Cava Obstruction to flow
- Causes
- Malignancy (60% of all cases)
- Bronchogenic Carcinoma (80% of malignancy causes)
- Malignant Lymphoma (15% of malignancy causes)
- Metastatic Disease (to lung, mediastinum or Lymph Nodes)
- Breast adenocarcinoma
- Testicular seminoma
- Vena Cava thrombosis (majority of non-malignant cases)
- Long-term venous catheters (40% of thrombosis cases)
- Idiopathic
- Behcet's Syndrome
- Polycythemia Vera
- Paroxysmal Nocturnal Hemoglobinuria
- Long-term shunts
- Long-term Pacemakers
- Other Benign Disease (Rare)
- Mediastinal fibrosis
- Post-Radiation Therapy
- Idiopathic
- Histoplasmosis
- Actinomycosis
- Tuberculosis
- Benign Mediastinal tumor
- Aortic aneurysm
- Dermoid tumor
- Goiter
- Sarcoidosis
- Mediastinal fibrosis
- Malignancy (60% of all cases)
- Symptoms
- Dyspnea (50%)
- Neck and facial swelling (40-82%)
- Head fullness or pressure Sensation
- Swelling of trunk and upper extremities (40%)
- Local Airway Compression
- Headache
- Common presenting complaint
- Secondary to Increased Intracranial Pressure
- Referred Pain
- Miscellaneous
- Lacrimation
- Visual changes
- Syncope
- Signs
- Thoracic vein distention (65%)
- Neck vein distention (55%)
- Facial edema (55%)
- Tachypnea (40%)
- Plethora of the face and Cyanosis (15%)
- Edema of upper extremities (10%)
- Paralysis of Vocal Cords (3%)
- Horner's Syndrome (3%)
- Distended antecubital veins
- Associated Conditions
- Imaging
- Chest XRay
- Mass identified in 90%
- Right superior mediastinum widening (75%)
- Hilar Adenopathy (50%)
- Right Pleural Effusion (25%)
- Chest CT with IV Contrast
- Identifies mass lesions and sites of SVC obstruction
- Collateral vessel development is a sensitive and specific marker of SVC Syndrome
- Test Specificity: 96%
- Test Sensitivity: 92%
- Other Imaging modalities
- MR Venogram
- Cavogram
- Ultrasound
- Chest XRay
- Management
- Severity of airway and vascular compromise varies by timing of progression
- Rapid progression may not allow for compensation, resulting in acute airway compromise
- Supportive care for airway compromise
- ABC Management
- High-Dose Corticosteroids
- Elevate the head of the bed
- Avoid Diuretics
- Identify mass etiology
- Reduction in mass size
- Emergent Intervention Radiology
- Other modalities
- Radiation Therapy (e.g. Non-Small Cell Lung Cancer)
- Chemotherapy
- Surgical decompression
- Consult Oncology and Cardiothoracic Surgery acutely
- Palliative CareConsultation once stabilized
- Anticoagulation with Heparin
- Indicated for venous thrombosis
- Intravenous stenting
- Common acute management
- Improvement is seen within days
- Effective even when tumor mass is not reducible
- Severity of airway and vascular compromise varies by timing of progression
- Prognosis
- Poor prognostic sign
- Predicts 90% mortality in 3 years
- Median survival: 6 months
- Poor prognostic sign
- References
- Long, Long and Koyfman (2020) Crit Dec Emerg Med 34(11): 17-24
- Higdon (2018) Am Fam Physician 97(11):741-8 [PubMed]