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.
- malignancy (~90% of cases), most commonly 5:
- central venous catheters
- pacemaker wires
- fibrosing mediastinitis
- luetic aneurysm
- Behcet disease
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.
Indirect signs on chest x-ray, such as superior mediastinal widening and right hilar prominence that may indicate the presence of mediastinal mass.
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 and prognosis
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.
- 1. Sheth S, Ebert MD, Fishman EK. Superior vena cava obstruction evaluation with MDCT. AJR Am J Roentgenol. 2010;194 (4): W336-46. doi:10.2214/AJR.09.2894 - Pubmed citation
- 2. Lawler LP, Fishman EK. Multi-detector row CT of thoracic disease with emphasis on 3D volume rendering and CT angiography. Radiographics. 2001;21 (5): 1257-73. Radiographics (full text) - Pubmed citation
- 3. Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am. J. Crit. Care. 1993;1 (1): 54-64. Pubmed citation
- 4. Lepper PM, Ott SR, Hoppe H et-al. Superior vena cava syndrome in thoracic malignancies. Respir Care. 2011;56 (5): 653-66. Respir Care (full text) - doi:10.4187/respcare.00947 - Pubmed citation
- 5. Katabathina VS, Restrepo CS, Betancourt Cuellar SL et-al. Imaging of oncologic emergencies: what every radiologist should know. Radiographics. 2013;33 (6): 1533-53. doi:10.1148/rg.336135508 - Pubmed citation
- 6. Fagedet D, Thony F, Timsit JF et-al. Endovascular treatment of malignant superior vena cava syndrome: results and predictive factors of clinical efficacy. Cardiovasc Intervent Radiol. 2013;36 (1): 140-9. doi:10.1007/s00270-011-0310-z - Pubmed citation
- 7. Nunnelee JD. Superior vena cava syndrome. J Vasc Nurs. 2007;25 (1): 2-5. doi:10.1016/j.jvn.2006.09.004 - Pubmed citation