Clinical Presentation

Superior Vena Cava Syndrome

Mita Sanghavi Goel, M.D. July 17, 2002

Clinical Presentation

Symptoms

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  May be mild if obstruction is proximal to the azygous vein
  

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  Usually neck, upper extremity, and/or facial swelling, especially around the eyes.
  
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  Dyspnea, cough
  
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  Hoarseness, tongue swelling, headaches, nasal congestion, epistaxis, hempoptysis, dysphagia, pain, epistaxis, dizziness, syncope, and lethargy
  
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  Symptoms exacerbated by lying down or leaning forward
  

Physical signs

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  Dilated neck veins
  
• 
  Cyanosis
  
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  Increased collateral blood vessels of the anterior chest wall
  
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  Edema of the face, arms and chest
  
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  Non pitting edema of the neck “Stoke’s Collar”
  
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  Severe cases include glossal and laryngeal edema, proptosis, and obtundation
  
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  Stridor
  
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  Pleural and pericardial effusion
  

Causes

Anything causing substantial reduction of venous return from the head, neck, or upper extremities. Can result from either intrinsic (10%) or extrinsic (90%) compression of the superior vena cava.

Malignant Causes

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  Malignant tumors cause >90% of all cases of SVC syndrome
  
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  Lung cancer, particularly small cell and squamous cell lung cancer, cause 85% of all malignancy-associated SVC syndrome, usually bronchogenic carcinoma (70%)
  
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  Lymphoma is the second most common malignant cause
  
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  Metastatic tumors to the mediastinum, including testicular and breast cancers.
  

Benign Causes

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  Aortic aneurysm
  
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  Enlarged thyroid
  
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  Thrombosis
  
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  Fibrosing mediastinitis from prior irradiation, methysergide ingestion, or infection (Histoplasmosis, M. tuberculosis)
  

Diagnosis

Largely clinical diagnosis

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  CXR may show mass, widening of the mediastinum (generally right- sided), or pleural effusion (present in 25% of patients with SVC syndrome and are generally right-sided)
  
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  CT Scan provides the most reliable definition of mediastinal anatomy. Will show diminished or absent opacification of central venous structures and prominent collateral venous circulation
  
• 
  MRI offers no advantage over CT scan
  
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  Upper extremity venography complements CT or MRI with defining level if obstruction
  

Treatment

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  Emergent therapy indicated if:
  
  • 
    Tracheal obstruction
    
  • 
    CNS abnormalities such as stupor or seizures are present because may indicate increased intracranial pressure
    
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  If otherwise stable, treatment depends on the cause of SVC syndrome
  
  • 
    XRT– primary treatment for SVC caused by non-small cell lung cancer and other metastatic tumors; however important to have tissue diagnosis before beginning XRT because XRT will obfuscate histology
    
  • 
    Chemotherapy – effective when underlying tumor is chemosensitive, such as small cell lung cancer or lymphoma
    
  • 
    Intravascular stents – effective palliative treatment. Often reserved for recurrent SVC syndrome (which is 10-30% of patients)
    
  • 
    Surgery – immediate relief if SVC syndrome from a benign cause
    
  • 
    Removal of foreign body and anticoagulation – if etiology is thrombus secondary to central venous catheter placement. Anticoagulation helps prevent embolization. If detected early enough, fibrinolytic therapy without catheter removal may be sufficient. Additionally low-dose coumadin (1-2 mg/day) reduces thrombus incidence
    
  • 
    Steroids – decreases cerebral or pharyngeal edema
    
  • 
    Diuretics– may provide temporary relief if respiratory symptoms present
    

Natural history

Most (75-90%) improve symptomatically within one week of initiating treatment.

Mortality depends on underlying etiology of SVC syndrome


References

Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990; 160:207-211.

Cecil Textbook of Medicine. Last accessed via www.mdconsult.com July 15, 2002.

Harrison’s Online. www.harrisonsonline.com. Last accessed July 13, 2002.

Markman M. Diagnosis and management of superior vena cava syndrome. Clev Clin J Med 1999; 66:59-61.

Roberts JR, Bueno R, Sugarbaker DJ. Multimodality treatment of malignant superior vena caval syndrome. Chest 1999; 116:835-837.

Beth Israel Deaconess Medical Center Residents’ Report