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Synonym: superior vena cava syndrome
Obstruction of the superior vena cava (SVC) can be due to external pressure, involvement of the vessel by tumour tissue, or a blood clot obstructing the lumen. William Hunter first identified the condition in 1757 in a patient with syphilitic aortic aneurysm. Today, the most common cause is superior mediastinal pressure associated with lung cancer (over 85%).[2, 3]
The SVC extends from the junction of the right and left innominate veins to the right atrium. It is surrounded by the sternum, trachea, right bronchus, aorta, pulmonary artery and the perihilar and paratracheal lymph nodes. All these are relatively rigid structures and as the SVC runs along the right side of the mediastinum this thin-walled, low pressure vessel can easily get compressed. The SVC provides venous drainage for the head, the neck, the upper extremities and the upper thorax. When obstructed, collateral routes are provided by four main pathways:
- The azygous venous system, which includes the azygous vein, the hemizygous vein and the connecting intercostal veins.
- The internal mammary venous system plus tributaries and secondary communications to the superior and inferior epigastric veins.
- The long thoracic venous system connection to the femoral veins.
- The long thoracic venous system connection to the vertebral veins.
One American study reviewed the aetiology of 78 patients with SVC obstruction over five years. In adults, bronchogenic carcinoma was the most common cause of SVC obstruction. Small-cell carcinoma (22%) and non-small cell carcinoma (24%) comprised the majority of cases, with lymphoma and germ cell tumours responsible for a smaller but significant proportion. Of note, an intravascular device was the most common cause in benign cases, followed by fibrosing mediastinitis.
In children, the most common causes are T-cell acute lymphoblastic leukaemia and non-Hodgkin's lymphoma (T-cell lymphoblastic lymphoma).
Gender predilection reflects aetiology, with more males than females due to the prevalence of lung cancer. Malignant causes are more common in the 40-60 age group and benign causes in the 30-40 age group.
The onset may be gradual or acute, depending on how quickly the condition proceeds from partial to complete obstruction and the degree of development of a collateral circulation.
One study identified dyspnoea, cough and chest pain at rest as being the most prominent symptoms, particularly in patients with malignancy. Other symptoms can include neck and face swelling, arm swelling, dizziness, headache, disturbed vision, nausea, nasal stuffiness, stupor and syncope. Symptoms tend to be aggravated by postures which increase the venous pressure in the upper part of the body, such as bending over or lying down.
Signs may include:
- Dilated veins over the arms, neck and anterior chest wall.
- Oedema of the upper body, extremities and face.
- Severe respiratory distress.
- Engorged conjunctiva.
- Convulsions and coma.
Signs may become more pronounced when the arms are lifted above the head.
Diagnosis may be made on clinical grounds in patients with overt symptoms and signs. However, in more subtle cases, further investigations are required.
- CXR: this may reveal a widened mediastinum or a mass on the right side of the chest.
- CT scanning: this shows more detail, particularly of surrounding structures such as the bronchi and vocal cords and may be necessary to guide more invasive investigative procedures . Various refinements exist, including the use of several detectors taking images in different planes. Injection of contrast media to highlight collateral circulation improves the sensitivity of CT scanning even further.
- Doppler scanning: Doppler flows correlate well with CT scanning and changes seen during the respiratory cycle can be used to evaluate the severity of obstruction and the effect of therapy.
- MRI scanning: this was thought to have some advantages but these have largely been cancelled out by the technological enhancement of CT. MRI also takes longer and is more expensive.
- Invasive contrast venography: this is useful in some cases, particularly prior to surgery.
- Radionuclide technetium-99m venography and gallium single-proton emission CT scanning: these are additional techniques which may be useful in some cases.
Most cases of SVC obstruction present before the primary cause is elucidated, and there has been some controversy as to whether treatment should be instituted before diagnostic procedures are carried out. Radiotherapy, for example, can destroy histologically useful material. However, the underlying pathology is usually easy to identify.
Bronchoscopy has a reported diagnostic yield of >70%, whilst mediastinoscopy or mediastinotomy has a yield of >90%. The latter has a high anaesthetic and complication risk; ultrasound-assisted biopsies have been found to be safer with a similar yield.
- Elevation of the head, and oxygen, may help to provide symptomatic relief.
- Corticosteroids and diuretics may be indicated in the emergency situation (eg, reduced cardiac output, cerebral or laryngeal oedema) although evidence for their efficacy is lacking.
- Radiotherapy is indicated in some lung cancer cases. The use of radiotherapy in SVC obstruction before a diagnosis of malignancy has been established is controversial but may occasionally be justified if symptoms are rapidly deteriorating or there have been multiple attempts to obtain a tissue diagnosis without success.
- Chemotherapy may be indicated in chemo-sensitive tumours (eg, small-cell lung cancer).
- Anticoagulation may be required in cases where venography has demonstrated central vein thrombosis, to prevent pulmonary embolism. It may also be indicated in central venous stenosis to prevent thrombosis.
- Stenting and/or angioplasty (also known as endovascular surgery) are particularly hopeful in patients requiring long-term venous access (eg, haemodialysis, total parenteral nutrition), or severe SVC obstruction symptoms. An increasing body of evidence suggests it should be considered as first-line treatment for all benign disease, as it is as effective but even less invasive than angioplasty.
- Surgical bypass of the obstruction is technically difficult but may be indicated in some cases. There are no large trials comparing this approach with stenting in benign disease.
- Selected patients may benefit from resection of the blockage, and reconstruction.
- Stenting is useful in relieving obstruction and a National Institute for Health and Care Excellence (NICE) review concluded that it was more effective and produced a quicker response than chemotherapy or radiotherapy. Thrombolysis and angioplasty may also be indicated in individual patients.
- Reconstructive surgery is possible and may be assisted by the use of autologous tissue.
- Insertion of a prosthesis does not increase the overall mortality from SVC resection and is a feasible option where reconstructive surgery is not possible.
The prognosis for the relief of SVC obstruction is good with radiation therapy or stenting. However, the ultimate prognosis is of course associated with the underlying condition.
Further reading and references
; The Many Facets of Dr William Hunter Historical Sciences, xxxii, 1994
; Endovascular stenting to treat obstruction of the superior vena cava, 1 BMJ 2008336:1434
; NICE Interventional Procedure Guidance, July 2004
; The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore). 2006 Jan85(1):37-42.
; Superior vena cava syndrome. Indian J Pediatr. 2002 Apr69(4):293-7.
; The superior vena cava syndrome caused by malignant disease. Imaging with multi-detector row CT. Eur J Radiol. 2006 Jul59(1):93-103. Epub 2006 Feb 14.
; Doppler superior vena cava flow evolution and respiratory variation in superior Echocardiography. 2008 Apr25(4):360-5.
; MRI versus CT and US in the evaluation of a patient presenting with superior vena cava syndrome. Case report. Clin Imaging. 1992 Oct-Dec16(4):269-71.
; Superior vena cava obstruction: diagnosis, management and outcome. East Afr Med J. 2008 Mar85(3):129-36.
; Malignant superior vena cava syndrome: is this a medical emergency? Ann Thorac Cardiovasc Surg. 2009 Apr15(2):89-92.
; Diagnostic yield and safety of ultrasound-assisted biopsies in superior vena cava Eur Respir J. 2009 Jun33(6):1389-95. Epub 2009 Feb 12.
; Benign superior vena cava syndrome: stenting is now the first line of treatment. J Vasc Surg. 2008 Feb47(2):372-80.
; Review of superior vena cava resection in the management of benign disease and Ann Thorac Surg. 2009 Aug88(2):392-7.
; Superior vena cava (SVC) reconstruction using autologous tissue in two cases of World J Surg Oncol. 2009 Oct 137:75.
; Superior vena cava resection in thoracic malignancies: does prosthetic Eur J Cardiothorac Surg. 2009 Nov 23.