Superior vena cava obstruction

Radswiki et al.

Superior vena cava (SVC) obstruction can occur from extrinsic compression, intrinsic stenosis or thrombosis. Malignancies are the main cause and are considered an oncologic emergency. Superior vena cava syndrome (SVCS) refers to the clinical syndrome with symptoms that results from this obstruction.

Clinical presentation depends on the speed, severity and location of superior vena cava obstruction 5. Collateral drainage may develop with slow obstruction and patients may have no or only mild symptoms.

With acute superior vena cava obstruction, symptoms include facial and neck swelling, facial flushing, bilateral upper extremity swelling, neurological signs, dyspnoea, headache and cough.

Causes
Pathophysiology

In long-standing cases with 60% or more stenosis, collateral channels are formed to restore venous return. Various collaterals are formed depending on the site of the obstruction:

  • pre azygos: in this conditions mainly the right superior intercostal veins serves as the collateral pathway to drain into the azygos vein.
  • azygos: when the azygos vein is also obstructed the collateral circulation establishes between SVC and IVC via minor communicating channels i.e. internal mammary veins, superior and inferior epigastric veins to iliac veins and finally into the IVC.
  • post azygos: in this case, the blood from the SVC is distributed into the azygos and hemiazygos and then into the IVC tributaries i.e. ascending lumbar and lumbar veins.

The most efficient collateral system is right superior intercostal and azygos circulation. For this reason, most of the patients with pre azygos obstruction of SVC remain asymptomatic for a long period of time.

Plain radiograph

Indirect signs on chest x-ray, such as superior mediastinal widening and right hilar prominence that may indicate the presence of mediastinal mass. 

CT

Is the imaging modality of choice. Enhanced CT shows the location and severity of the SVC obstruction, superimposed thrombosis, a mediastinal mass or lymphadenopathy, collateral vessels and associated lung masses. 

See: superior vena cava obstruction: grading 

Treatment of SVCS will depend on the cause of the compression. Thrombolysis and anticoagulation may be indicated on thrombosis. In cases of compression, endovascular treatment with self-expandable bare stents is an effective SVCS therapy 6. With carcinoma or infection, specific drugs or radiation may be used 7.  

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Article information

rID: 19760
Sections: Syndromes, Pathology
Synonyms or Alternate Spellings:
  • SVC syndrome
  • Superior vena cava compression
  • SVC obstruction
  • Obstruction of the superior vena caval
  • Obstruction of superior vena caval
  • Superior vena caval compression
  • SVC compression
  • Superior vena cava syndrome
  • Superior vena cava syndrome (SVCS)

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Cases and figures

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    Case 1: with SVC syndrome
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    Case 2
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    Case 3
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    Case 4: tumour thrombus
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    Case 5
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    Case 6: treated with stent
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    Case 7: with internal mammary vein collaterals
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    Case 8
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    Prominent right s...
    Case 9: pre azygos obstruction of SVC and resultant right superior intercostal-azygos collaterals.
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    Case 10: likely chronic
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