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| | __NOTOC__ |
| {{DiseaseDisorder infobox | | | {{DiseaseDisorder infobox | |
| Name = Superior vena cava syndrome | | | Name = Superior vena cava syndrome | |
| ICD10 = {{ICD10|I|87|1|i|80}} |
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| ICD9 = {{ICD9|459.2}} |
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| ICDO = |
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| Image = Svc-syndrome.jpg| | | Image = Svc-syndrome.jpg| |
| Caption = |
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| OMIM = |
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| OMIM_mult = |
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| MedlinePlus = 001097 |
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| eMedicineSubj = emerg |
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| eMedicineTopic = 561 |
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| DiseasesDB = 12711 |
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| MeshID = D013479 |
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| }} | | }} |
| {{SI}} | | {{Superior vena cava syndrome}} |
| {{WikiDoc Cardiology Network Infobox}}
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| {{CMG}}
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| {{Editor Help}} | | '''For patient information, click [[{{PAGENAME}} (patient information)|here]].''' |
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| '''Synonyms and Related Keywords:''' SVC syndrome | | '''For pediatric considerations, click [[Superior vena cava syndrome pediatric considerations|here]].''' |
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| == Overview ==
| | {{CMG}};{{AE}}{{CZ}}{{MV}} |
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| '''Superior vena cava syndrome''' (SVCS) is an array of symptoms caused by the impairment of blood flow through the superior vena cava (SVC) to the right atrium. Symptoms that prompt suspicion of this syndrome include dyspnea, coughing, and swelling of the face, neck, upper trunk, and extremities. In rare instances, patients may complain of hoarseness, chest pain, dysphagia, and hemoptysis. Physical signs that may be noted on presentation are neck vein distention, thoracic vein distention, edema of the face or upper extremities, plethora, and tachypnea. Rarely, cyanosis, Horner syndrome, and a paralyzed vocal cord may also be present.
| | {{SK}} SVC syndrome; superior vena cava obstruction; SVC obstruction; superior mediastinal syndrome; SVCS; SVCO |
| | ==[[Superior vena cava syndrome overview|Overview]]== |
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| SVCS is usually a sign of locally advanced bronchogenic carcinoma. Survival depends on the status of the patient’s disease. When small cell bronchogenic carcinoma is treated with chemotherapy, the median survival times with or without SVCS are almost identical (42 weeks or 40 weeks, respectively). The 24-month survival rate is 9% in patients without SVCS and 3% in those with the syndrome. When the malignancy is treated with radiation therapy, 46% of patients who have non-small cell lung cancer experience relief of symptoms compared with 62% of patients who have small cell bronchogenic carcinoma. The 2-year survival rate of 5% is almost the same for both groups.
| | ==[[Superior vena cava syndrome historical perspective|Historical Perspective]]== |
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| Most non-Hodgkin lymphoma patients with SVCS respond to appropriate chemotherapy or to combined modality regimens.
| | ==[[Superior vena cava syndrome pathophysiology|Pathophysiology]]== |
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| ==Etymology== | | ==[[Superior vena cava syndrome causes|Causes]]== |
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| The Scottish obstetrician and anatomist, William Hunter, first described the entity in 1757, noting it as a complication of a syphilitic aortic aneurysm.
| | ==[[Superior vena cava syndrome differential diagnosis|Differentiating Superior Vena Cava Syndrome from other Diseases]]== |
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| == Epidemiology and Demographics == | | ==[[Superior vena cava syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
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| Most SVC syndromes in the present day are related to malignancy. An underlying malignancy is found in approximately 90% of patients.
| | ==[[Superior vena cava syndrome risk factors|Risk Factors]]== |
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| == Pathophysiology & Etiology== | | ==[[Superior vena cava syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| Since SVCS was first described by William Hunter in 1757, the spectrum of underlying conditions associated with it has shifted from tuberculosis and syphilitic aneurysms of the ascending aorta to malignant disorders. Almost 95% of SVCS cases described in published modern series are due to cancer; the most common cause is small cell bronchogenic carcinoma, followed by squamous cell carcinoma of the lung, adenocarcinoma of the lung, non-Hodgkin lymphoma, and large cell carcinoma of the lung. A nonmalignant cause of SVCS in cancer patients is thrombosis that is associated with intracaval catheters or pacemaker wires. A rare cause of SVCS is fibrosing mediastinitis, either idiopathic or associated with histoplasmosis. Additional rare causes of SVCS include metastatic germ cell neoplasms, metastatic breast cancer, colon cancer, Kaposi sarcoma, esophageal carcinoma, fibrous mesothelioma, Behçet syndrome, thymoma, substernal thyroid goiter, Hodgkin lymphoma, and sarcoidosis.
| | ==Diagnosis== |
| | [[Superior vena cava syndrome history and symptoms|History and Symptoms]] | [[Superior vena cava syndrome physical examination|Physical Examination]] | [[Superior vena cava syndrome laboratory findings|Laboratory Findings]] | [[Superior vena cava syndrome chest x ray|Chest X Ray]] | [[Superior vena cava syndrome CT|CT]] | [[Superior vena cava syndrome MRI|MRI]] | [[Superior vena cava syndrome ultrasound|Ultrasound]] | [[Superior vena cava syndrome other imaging findings|Other Imaging Findings]] | [[Superior vena cava syndrome other diagnostic studies|Other Diagnostic Studies]] |
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| Knowledge of the anatomy of the SVC and its relationship to the surrounding lymph nodes is essential to understanding the development of the syndrome. The SVC is formed by the junction of the left and right brachiocephalic veins in the mid third of the mediastinum. The SVC extends caudally for 6 to 8 cm, coursing anterior to the right mainstem bronchus and terminating in the superior right atrium, and extends anteriorly to the right mainstem bronchus. The SVC is joined posteriorly by the azygos vein as it loops over the right mainstem bronchus and lies posterior to and to the right of the ascending aorta. The mediastinal parietal pleura is lateral to the SVC, creating a confined space, and the SVC is adjacent to the right paratracheal, azygous, right hilar, and subcarinal lymph node groups. The vessel itself is thin-walled, and the blood flowing therein is under low pressure. Thus, when the nodes or ascending aorta enlarge, the SVC is compressed, blood flow slows, and complete occlusion may occur.
| | ==Treatment== |
| | [[Superior vena cava syndrome medical therapy|Medical Therapy]] | [[Superior vena cava syndrome surgery|Surgery]] | [[Superior vena cava syndrome radiation therapy|Radiation Therapy]] | [[Superior vena cava syndrome primary prevention|Primary Prevention]] | [[Superior vena cava syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Superior vena cava syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| The severity of the syndrome depends on the rapidity of onset of the obstruction and its location. The more rapid the onset, the more severe the symptoms because the collateral veins do not have time to distend to accommodate an increased blood flow. If the obstruction is above the entry of the azygos vein, the syndrome is less pronounced because the azygous venous system can readily distend to accommodate the shunted blood with less venous pressure developing in the head, arms, and upper thorax. If the obstruction is below the entry of the azygos vein, more florid symptoms and signs are seen because the blood must be returned to the heart via the upper abdominal veins and the inferior vena cava, which requires higher venous pressure.
| | ==Case Studies== |
| | [[Superior vena cava syndrome case study one|Case #1]] |
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| One study suggested that the general recruitment of venous collaterals over time may lead to remission of the syndrome, although the SVC remains obstructed
| | ==Related Chapters== |
| | | *[[Pemberton's sign]] |
| == Natural History ==
| | *[[Cough causes]] |
| In the past, SVC syndrome was a medical emergency and empiric radiation was given to shrink the tumor. With the advent of better medical therapy for some lung cancers and lymphoma and the low morbidity associated with diagnostic procedures, this approach has fallen out of favor.
| | *[[Oncologic emergencies]] |
| | | *[[Mediastinal tumor]] |
| ==Signs and symptoms== | |
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| The most common symptoms are these:
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| *[[Dyspnea]] | |
| * Cough
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| * Swollen face, neck, upper body, and arms.
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| Less common symptoms include the following:
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| * Hoarse voice.
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| * Chest pain.
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| * Problems swallowing and/or talking.
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| * Coughing up blood.
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| * Swollen veins in the chest or neck.
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| * Bluish color to the skin.
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| * Drooping eyelid.
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| * [[Headache]] | |
| * Facial [[plethora]] | |
| * [[Lightheadedness]] | |
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| Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.<ref name="emedicine">{{cite web | last = Beeson | first = Michael S | title = Superior Vena Cava Syndrome | url=http://www.emedicine.com/emerg/topic561.htm | accessdate = 2008-03-24 }}</ref>
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| == Diagnosis ==
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| Once SVCS is recognized, prompt clinical attention is important. A diagnosis should be established prior to initiating therapy for the following reasons:
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| * 75% of patients have symptoms and signs for longer than 1 week before seeking medical attention.
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| * Cancer patients diagnosed with SVCS do not die of the syndrome itself but rather from the extent of their underlying disease.
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| * 3% to 5% of the patients diagnosed with SVCS do not have cancer.
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| In the absence of tracheal obstruction, SVCS is unlikely to be a life-threatening oncologic emergency, and treatment prior to definitive diagnosis is not justified.
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| The initial evaluation of the patient should include a chest radiograph to look for mediastinal masses and associated findings, such as pleural effusion, lobar collapse, or cardiomegaly. Computed tomography (CT) scanning of the thorax yields the most useful diagnostic information and can define the anatomy of the involved mediastinal nodes. Venous patency and the presence of thrombi are assessed by using contrast and rapid scanning techniques. Depending on local expertise, contrast or nuclear venography, magnetic resonance imaging, and ultrasound may be valuable in assessing the site and nature of the obstruction.
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| If bronchogenic carcinoma is suspected, a sputum specimen should be obtained. If the sputum specimen is negative, a biopsy specimen should be taken from the most accessible site that is clinically involved with disease. The biopsy approach depends on the working diagnosis, the location of the tumor, the physiologic status of the patient, and the expertise available at the facility. It may include bronchoscopy, biopsy of palpable cervical or supraclavicular lymph nodes, needle biopsy of a lung mass or mediastinal nodes using either CT or ultrasound guidance, mediastinoscopy, mediastinotomy, median sternotomy, video-assisted thoracoscopy, and conventional thoracotomy. The biopsy findings will help the clinician plan appropriate treatment
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| === History and Symptoms ===
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| The presentation depends on the degree of occlusion and the rapidity with which it develops. The most common symptoms are [[dyspnea]], facial swelling/fullness (suffusion) and [[cough]]. The most common signs are venous distention of the neck and chest wall and facial edema. Less commonly, [[chest pain]], [[dysphagia]], [[proptosis]], [[hemoptysis]], glossal edema, [[hoarseness]], and [[headache]] are described. Symptoms get worse with leaning forward, coughing or lying down. Typically symptoms are present for >3 months before diagnosis.
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| === Physical Examination ===
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| ==== Appearance of the Patient ====
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| Pemberton’s Sign: suffusion, plethora, or duskiness that develop upon elevation of the arms above the head in patients with SVC syndrome.
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| ====Vital Signs====
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| ====Skin====
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| <div align="left">
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| <gallery heights="175" widths="175">
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| Image:Svc-syndrome.jpg|Distension of veins on the torso of a patient with SVC syndrome
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| </gallery>
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| </div>
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| The skin of the face may have plethora.
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| There may be distension of veins on the torso.
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| ==== Ear Nose and Throat ====
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| There is swelling of the face.
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| === Laboratory Findings ===
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| ==== Chest X Ray ====
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| This is a useful test to exclude lung cancer
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| ==== MRI and CT ====
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| Useful in evaluating source and extent of a neoplasm.
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| <div align="left">
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| <gallery heights="175" widths="175">
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| Image:Svc syndrome collaterals.jpg|right|thumb|Collaterals on CT scan in a patient with SVC syndrome
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| </gallery>
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| </div>
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| === Other Diagnostic Studies ===
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| Biopsy may be neccessary to evaluate the underlying cause.
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| == Risk Stratification and Prognosis==
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| SVC syndrome is rarely fatal, and the prognosis is generally related to the prognosis of the underlying malignancy rather than the presence/absence of venous obstruction.
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| == Differential Diagnosis ==
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| The leading cancers associated with SVC syndrome are [[bronchogenic lung cancer]] (particularly [[small cell lung cancer]]), [[breast cancer]], and [[lymphoma]].
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| Benign causes of SVC syndrome include [[mediastinal fibrosis]], [[histoplasmosis]], [[radiation therapy complications]], retrosternal [[goiter]], [[Behcet’s]] syndrome, and thrombosis due to indwelling catheters or pacemakers. In the past, [[Tuberculosis]] and [[Syphilis]] were major players.
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| == Treatment ==
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| The treatment of SVCS depends on the etiology of the obstruction, the severity of the symptoms, the prognosis of the patient, and patient preferences and goals for therapy. Radiation therapy or chemotherapy should be withheld until the etiology of the obstruction is clear. The treatments discussed here focus on SVC obstruction caused by a malignant tumor. Since the treatment of malignant obstruction may depend on tumor histology, a histologic diagnosis, if not made earlier, should be made prior to initiation of treatment. Unless there is airway obstruction or cerebral edema, there appears to be no detriment in outcome when treatment is delayed for the assessment. <ref> Nieto AF, Doty DB. Superior vena cava obstruction: clinical syndrome, etiology, and treatment. Curr Probl Cancer 1986; 10:441-84. </ref> <ref>Yellin A, Rosen A, Reichert N, Lieberman Y. Superior vena cava syndrome. The myth--the facts. Am Rev Respir Dis 1990; 141:1114-8. </ref> <ref> Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990; 160:207-11. </ref>
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| ===Medical Management===
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| A patient with sufficient collateral blood flow and minimal symptoms may not need treatment. If the lesion is above the azygous vein or if the onset of SVC occlusion is slow enough to allow sufficient collateral circulation, the symptoms and signs may stabilize and the patient may be comfortable enough to forego further therapy. Short-term palliation of a symptomatic patient who does not want aggressive treatment may be achieved by elevating the head and using corticosteroids and diuresis. There are no definitive studies that prove the effectiveness of steroids, although they are potentially useful to treat respiratory compromise. Diuretics may give symptomatic relief of edema but can ultimately cause systemic complications, such as dehydration.
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| ===Radiation Therapy===
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| 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.
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| ===Chemotherapy===
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| Chemotherapy is the treatment of choice for sensitive tumors such as lymphoma or small cell lung cancer. SVCS does not appear to be an independent prognostic factor, and its presence should not change the treatment approach. Rapid initiation of chemotherapy can result in complete and partial response rates of the SVCS of more than 80% in small cell lung cancer patients.
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| ===Thrombolysis===
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| It has been suggested that SVCS arises when a thrombus forms in a partially occluded vein. In patients with a documented thrombus in the SVC, treatment may include thrombectomy, with or without tissue plasminogen activator or other thrombolytic agents such as streptokinase or urokinase.
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| ===Stent Placement===
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| There have been numerous small studies using an intravascular expandable stent to reopen the occluded SVC; however, no prospectively designed comparative studies have been published. The reported response rates have been about 90% or greater. There is no agreement on the need for anticoagulant therapy after stent placement. In one series that used anticoagulant therapy for patients as part of the treatment protocol, there were reports of reocclusion after this therapy was stopped. However, in another study, 17 cancer patients who were treated with stents and who did not have anticoagulant therapy had no occlusions.
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| ===Surgery===
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| Surgical bypass of an obstructed SVC is more appropriate for patients with a benign obstruction than with a malignant obstruction, although surgical bypass has also been used for patients with malignant obstructions.
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| ===Psychosocial Considerations===
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| Patients and family members are often frightened and anxious because of the symptoms produced by SVCS, particularly swelling, dysphagia, coughing, and hoarseness. It is important to provide information to patients and family members on the cause of the symptoms and on short-term measures for palliation, especially during the diagnostic period. When aggressive treatment is declined because of the terminal nature of the underlying disease, it may be necessary to teach symptom management approaches to patients and family members.
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| Because most adult patients who develop SVCS have lung cancer, the treatment and psychologic support approaches that are developed for SVCS should take into account the patient’s prognosis and psychologic condition and other symptoms caused by the malignancy
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| == References ==
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| {{reflist|2}}
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| ==See also==
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| * [[Pemberton's sign]]
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| == Acknowledgements ==
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| Source of Initial Content: Morning report notes prepared by Dr. Duane Pinto, C. Michael Gibson, M.S., M.D.
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