Superior vena cava syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Overview
Superior vena cava syndrome (also known as, SVCS), is a group of symptoms caused by obstruction of the superior vena cava. More than 90% of cases of superior vena cava obstruction are caused by malignant causes, typically a tumor outside the vessel compressing the vessel wall. However, in 10% of the cases, the cause is benign. Characteristic features are edema (swelling due to excess fluid) of the face and arms and development of swollen collateral veins on the front of the chest wall. Shortness of breath and coughing are quite common symptoms; difficulty swallowing is reported in 11% of cases, headache in 6% and stridor in 4%. The condition is rarely life-threatening, in less than 5% of cases of SVCO, severe neurological symptoms or airway compromise are reported.[1] Treatment mainly consists of careful medical therapy (glucocorticoids) and surgery.[2]
Historical Perspective
Superior vena cava syndrome was first discovered by William Hunter (1718-1783), a Scottish obstetrician, in 1757 following as a complication of a syphilitic aortic aneurysm.[3]
Pathophysiology
Superior vena cava (SVC) syndrome arises from the obstruction of venous blood drainage of the superior vena cava, which is normally involved in the major blood flow return from head, neck, upper extremities, and upper thorax to the heart. Superior vena cava syndrome is a complication from a partial or complete obstruction due to malignant causes (60%) or benign causes (20%). This syndrome consists on the invasion of the venous wall associated with intravascular thrombosis, enlarged nodes, enlarged ascending aorta or by extrinsic pressure of a tumor mass against the thin-walled superior vena cava (SVC) which leads to the development of SVC syndrome. SVC syndrome is associated with a number of conditions that include malignant tumors, tuberculosis, histoplasmosis, and syphilis.[4]
Causes
Superior vena cava syndrome may be caused by obstruction of the superior vena cava (SVC) by neoplastic invasion of the venous wall associated with intravascular thrombosis, enlarged nodes, enlarged ascending aorta, or by extrinsic pressure of a tumor mass against the thin-walled superior vena cava (SVC).[5]
Differentiating Superior Vena Cava Syndrome from other Diseases
Superior vena cava syndrome should be differentiated from other causes of dyspnea and jugular venous distention, such as, cardiac tamponade, chronic obstructive pulmonary disease, mediastinitis, pneumonia, acute respiratory distress syndrome, and syphilis.[4]
Epidemiology and Demographics
Superior vena cava syndrome is a common oncologic emergency. The incidence rate in the United States of SVCS is approximately 15,000 individuals each year. The incidence of superior vena cava syndrome increases with age; SVCS affects adults with more frequency, and it is less common in children and young adults. The median age of diagnosis is between 40-60 years. Males are slightly more affected than women.[6]
Risk Factors
The most potent risk factor in the development of SVCS is intrathoracic malignancies. Other common risk factors include thrombosis (due to intravascular devices), postradiation fibrosis and mediastinitis.[7]
Natural History, Complications and Prognosis
If left untreated, patients with superior vena cava syndrome may progress to develop a complete blood flow obstruction and a decreased cardiac output with hypotension, leading to heart failure and death. Common complications of superior vena cava syndrome include airway obstruction, increased ICP, laryngeal edema and cerebral edema. The prognosis will vary depending on the cause of the syndrome, and the amount of blockage that has already occurred. Prognosis is generally poor, and the survival rate of patients with SVCS is approximately 10-20% at 6 months.[8]
Diagnosis
History and Symptoms
The hallmark of superior vena cava syndrome is elevated jugular pressure and dyspnea. A positive history of cancer and intra-vascular devices are suggestive of superior vena cava syndrome. The most common symptoms of superior vena cava syndrome include upper body swelling, dyspnea, and cough.[9]
Physical Examination
Common physical examination findings of superior vena cava syndrome include the pemberton's sign, facial swelling, and jugular venous distension.[9][10]
Chest X Ray
On chest x-ray, indirect signs such as superior mediastinal widening and right hilar prominence that may indicate the presence of mediastinal mass. [11]
CT
On enhanced CT scan, findings include location and severity of the SVC obstruction, superimposed thrombosis, a mediastinal mass or lymphadenopathy, collateral vessels and associated lung masses.[11]
MRI
Blockage of the SVC may be visible precisely on MRI of the chest and it is also useful in evaluating source and extent of a neoplasm.
Ultrasound
Doppler ultrasound may be valuable in assessing the site and nature of the obstruction in SVC syndrome. Venous patency and the presence of thrombi can also be assessed by using contrast and rapid scanning techniques.
Other Imaging Findings
SVC syndrome may also affect the findings of radionuclide ventriculography and liver scan.
Treatment
Medical Therapy
The treatment of SVC syndrome depends on the etiology of the obstruction, the severity of the symptoms, the prognosis of the patient, and patient preferences and goals for therapy.
Surgery
Surgical options include stent placement and surgical bypass.
Radiation Therapy
If the obstruction of the SVC is caused by a tumor that is not sensitive to chemotherapy, radiation therapy should be given. Treatment with larger fractions of radiation is thought to be beneficial in developing a rapid response. One study shows, however, that there is no obvious need for large radiation fraction sizes for the first few radiation treatments as was previously believed. Many fractionation schemes have been used, with doses ranging from 30 Gy in 10 fractions to 50 Gy in 25 fractions. Relief of symptoms in small cell lung cancer is reported to be 62% to 80%, whereas in non-small cell lung cancer, approximately 46% of the patients experienced symptomatic relief. In one study, more than 90% of the patients achieved a partial or complete response with a 3-week regimen of 8 Gy given once a week for a total dose of 24 Gy.
Primary Prevention
Prompt treatment of other medical disorders may reduce the risk of developing SVC obstruction.
References
- ↑ Kent, MS; Port, JL (2007). "Superior Vena Cava Syndrome". In Chang, AE; Ganz, PA; Hayes, DF; et al. Oncology – An Evidence-based Approach. Springer Science & Business Media. pp. 1291–9. ISBN 0387310568.
- ↑ Superior vena cava syndrome https://en.wikipedia.org/wiki/Superior_vena_cava_syndrome Accessed on December 11, 2016
- ↑ William Hunter. https://en.wikipedia.org/wiki/William_Hunter_%28anatomist%29 Accessed on December 11, 2016
- ↑ 4.0 4.1 Menon A, Gupta A (2015). "Superior vena cava syndrome". Indian J. Med. Res. 142 (3): 350. doi:10.4103/0971-5916.166606. PMC 4669875. PMID 26458355.
- ↑ Superior vena cava syndrome. https://en.wikipedia.org/wiki/Superior_vena_cava_syndrome Accessed on January, 11 2016
- ↑ Higdon ML, Higdon JA (2006). "Treatment of oncologic emergencies". Am Fam Physician. 74 (11): 1873–80. PMID 17168344.
- ↑ Baker GL, Barnes HJ (1992). "Superior vena cava syndrome: etiology, diagnosis, and treatment". Am. J. Crit. Care. 1 (1): 54–64. PMID 1307879.
- ↑ Wilson LD, Detterbeck FC, Yahalom J (2007). "Clinical practice. Superior vena cava syndrome with malignant causes". N. Engl. J. Med. 356 (18): 1862–9. doi:10.1056/NEJMcp067190. PMID 17476012.
- ↑ 9.0 9.1 Uberoi R (2006). "Quality assurance guidelines for superior vena cava stenting in malignant disease". Cardiovasc Intervent Radiol. 29 (3): 319–22. doi:10.1007/s00270-005-0284-9. PMID 16502166.
- ↑ Superior Vena Cava Syndrome. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/diagnosis-and-treatment/managing-side-effects/superior-vena-cava-syndrome/?region=bc Accessed on January 13, 2016
- ↑ 11.0 11.1 Superior Vena Cava Syndrome.Dr Amir Rezaee and Radswiki et al. Radiopedia http://radiopaedia.org/articles/superior-vena-cava-obstruction Accessed on January 13, 2016
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