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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}} |
  ICD9          = {{ICD9|459.2}} |
  ICDO          = |
   Image          = Svc-syndrome.jpg|
   Image          = Svc-syndrome.jpg|
  Caption        =  |
  OMIM          = |
  OMIM_mult      = |
  MedlinePlus    = 001097 |
  eMedicineSubj  = emerg |
  eMedicineTopic = 561 |
  DiseasesDB    = 12711 |
  MeshID        = D013479 |
}}
}}
{{SI}}
{{Superior vena cava syndrome}}
{{WikiDoc Cardiology Network Infobox}}
{{CMG}}


{{Editor Help}}
'''For patient information, click [[{{PAGENAME}} (patient information)|here]].'''


== Overview ==
'''For pediatric considerations, click [[Superior vena cava syndrome pediatric considerations|here]].'''


Also known as: SVC syndrome
{{CMG}};{{AE}}{{CZ}}{{MV}}


'''Superior vena cava (SVC) syndrome''' is the obstruction of the superior vena cava resulting in diminished venous return from the head, neck and upper extremities. The Scottish obstetrician and anatomist, William Hunter, first described the entity in 1757, noting it as a complication of a syphilitic aortic aneurysm.
{{SK}} SVC syndrome; superior vena cava obstruction; SVC obstruction; superior mediastinal syndrome; SVCS; SVCO                                                                                                                                                                                                     
==[[Superior vena cava syndrome overview|Overview]]==


== Epidemiology and Demographics ==
==[[Superior vena cava syndrome historical perspective|Historical Perspective]]==


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 pathophysiology|Pathophysiology]]==


== Pathophysiology & Etiology==
==[[Superior vena cava syndrome causes|Causes]]==


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.
==[[Superior vena cava syndrome differential diagnosis|Differentiating Superior Vena Cava Syndrome from other Diseases]]==


== Natural History ==
==[[Superior vena cava syndrome epidemiology and demographics|Epidemiology and Demographics]]==
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.


==Signs and symptoms==
==[[Superior vena cava syndrome risk factors|Risk Factors]]==
*[[Dyspnea]]
*[[Headache]]
*Facial [[plethora]]
*Venous distension in the neck and distended veins in the upper chest and arms
*Upper limb [[oedema]]
*[[Lightheadedness]]<ref name="emedicine"/>
*[[Cough]]<ref name="emedicine"/>


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>
==[[Superior vena cava syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


== Diagnosis ==  
==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]]


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.
==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]]


=== Differential Diagnosis ===
==Case Studies==
[[Superior vena cava syndrome case study one|Case #1]]


The leading cancers associated with SVC syndrome are [[bronchogenic lung cancer]] (particularly [[small cell lung cancer]]), [[breast cancer]], and [[lymphoma]]. 
==Related Chapters==
 
*[[Pemberton's sign]]
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.
*[[Cough causes]]
 
*[[Oncologic emergencies]]
=== History and Symptoms ===
*[[Mediastinal tumor]]
 
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.
 
=== Physical Examination ===
 
==== Appearance of the Patient ====
 
Pemberton’s Sign:  suffusion, plethora, or duskiness that develop upon elevation of the arms above the head in patients with SVC syndrome.
 
====Vital Signs====
 
====Skin====
 
<div align="left">
<gallery heights="175" widths="175">
Image:Svc-syndrome.jpg|Distension of veins on the torso of a patient with SVC syndrome
</gallery>
</div>
 
The skin of the face may have plethora.
 
There may be distension of veins on the torso.
 
==== Ear Nose and Throat ====
There is swelling of the face.
 
=== Laboratory Findings ===
 
==== Chest X Ray ====
This is a useful test to exclude lung cancer
 
==== MRI and CT ====
Useful in evaluating source and extent of a neoplasm.
 
<div align="left">
<gallery heights="175" widths="175">
Image:Svc syndrome collaterals.jpg|right|thumb|Collaterals on CT scan in a patient with SVC syndrome
</gallery>
</div>
 
=== Other Diagnostic Studies ===
Biopsy may be neccessary to evaluate the underlying cause.
 
== Risk Stratification and Prognosis==
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.
 
== Treatment ==
=== Pharmacotherapy ===
 
==== Acute Pharmacotherapies ====
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>
 
=== Surgery and Device Based Therapy ===
Stenting may be neccessary to preserve patency.
 
== References ==
{{reflist|2}}
 
==See also==
* [[Pemberton's sign]]
 
== Acknowledgements ==
Source of Initial Content: Morning report notes prepared by Dr. Duane Pinto, C. Michael Gibson, M.S., M.D.
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== Suggested Reading and Key General References ==
 
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== For Patients ==
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Latest revision as of 17:02, 27 November 2017

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Superior Vena Cava Syndrome Microchapters

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Patient Information

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Superior Vena Cava Syndrome from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

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Medical Therapy

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Primary Prevention

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]Maria Fernanda Villarreal, M.D. [3]

Synonyms and keywords: SVC syndrome; superior vena cava obstruction; SVC obstruction; superior mediastinal syndrome; SVCS; SVCO

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Superior Vena Cava Syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Radiation Therapy | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

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