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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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| Also known as: 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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| Collaterals form over time via the mammary, vertebrals, azygous and lateral thoracic veins, so despite almost or complete occlusion of the SVC venous drainage still can occur. Cerebral edema and obtundation with raised intracranial pressure is, therefore, rare. However, if this rare presentation is noted or if the airway is compromised, SVC syndrome represents a medical emergency.
| | ==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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| == Natural History == | | ==Treatment== |
| 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.
| | [[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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| ==Signs and symptoms== | | ==Case Studies== |
| | [[Superior vena cava syndrome case study one|Case #1]] |
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| The most common symptoms are these:
| | ==Related Chapters== |
| | | *[[Pemberton's sign]] |
| *[[Dyspnea]] | | *[[Cough causes]] |
| * Cough
| | *[[Oncologic emergencies]] |
| * Swollen face, neck, upper body, and arms.
| | *[[Mediastinal tumor]] |
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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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| The current strategy is to embark on a search for the primary source of the tumor. Start with a chest x-ray as a screening tool followed by a CT scan with contrast to further define the anatomy, extent of compression and to guide biopsy if a diagnosis cannot be made less invasively, e.g. thoracentesis, sputum, lymph node, and bone marrow biopsy.
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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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| === 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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| == Treatment ==
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| === Pharmacotherapy ===
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| ==== Acute Pharmacotherapies ====
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| The goal of therapy is to relieve the obstructive symptoms and address the underlying process. Removal of nonessential central lines and upright posture are initial measures. Diuretics, steroids, radiation and chemotherapy are used depending on clinical scenario. In the case of compression, anticoagulation and stent placement are accepted means of attempting to assure long-term patency and prevent propagation. <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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| === Surgery and Device Based Therapy ===
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| Stenting may be neccessary to preserve patency.
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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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