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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.
On this page:
Article:
Clinical presentation
Pathology
Radiographic features
Treatment and prognosis
References
Images:
Clinical presentation
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.
Pathology
Causes
malignancy (responsible for SVC syndrome in about ~90% of cases, lung carcinoma and lymphoma
are the most common neoplasms associated with this condition) 5
central venous catheters
pacemaker wires
fibrosing mediastinitis
luetic aneurysm
Behcet disease
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.
Radiographic features
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.
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.
References
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Article information
rID: 19760
System: Chest, Vascular, Oncology
Section: Syndromes
Tag: oncology
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)
Cases and figures
Case 2Case 2
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Case 3Case 3
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Case 4: tumour thrombusCase 4: tumour thrombus
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Case 5Case 5
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Case 8Case 8
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Case 9: pre azygos obstruction of SVC and resultant right superior intercostal-azygos collaterals.Case 9: pre
azygos obstruction of SVC and resultant right superior intercostal-azygos collaterals.
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Case 10: likely chronicCase 10: likely chronic
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