View source for Superior vena cava syndrome overview


<div style="-webkit-user-select: none;">
{| class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=RDHO3FxNT3s|350}}
|-
|}
__NOTOC__
{{Superior vena cava syndrome}}
{{CMG}}{{AE}}{{MV}}

==Overview==

'''Superior vena cava syndrome''' (also known as '''SVCS'''), is a group of [[Symptom|symptoms]] caused by [[obstruction]] of the [[superior vena cava]]. More than 60% of cases of [[superior vena cava]] [[obstruction]] are caused by [[malignant]] causes, typically a [[tumor]] outside the [[Blood vessel|vessel]] compressing the [[Blood vessel|vessel wall]]. However, in 20% 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 circulation|collateral veins]] on the front of the [[Thoracic wall|chest wall]]. [[Dyspnea|Shortness of breath]] and [[coughing]] are quite common [[Symptom|symptoms]]; [[Dysphagia|difficulty swallowing]] is reported  in 11% of cases, [[headache]] in 6%, and [[stridor]] in 4%. The [[prognosis]] is generally poor and the [[survival rate]] of [[Patient|patients]] with [[superior vena cava syndrome]] is approximately 10-20% at 6 months. In less than 5% of cases of [[superior vena cava syndrome]], severe [[Neurological|neurological symptoms]] or [[airway]] compromise are reported. [[Treatment IND|Treatment]] mainly consists of careful [[Medical therapy template|medical therapy]] ([[glucocorticoids]]) and [[surgery]].

==Historical Perspective==
Superior vena cava syndrome was first discovered by William Hunter (1718-1783), a Scottish [[obstetrician]], in 1757.<ref name="wikihistory">William Hunter. https://en.wikipedia.org/wiki/William_Hunter_%28anatomist%29 Accessed on December 11, 2016</ref>

==Pathophysiology==
[[Superior vena cava]] 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 [[Thorax|upper thorax]] to the [[heart]]. [[Superior vena cava syndrome]] is a complication of 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 [[Lymph nodes|nodes]], enlarged [[ascending aorta]],  or by extrinsic pressure of a [[tumor]] mass against the thin-walled [[superior vena cava]]  which leads to the development of [[superior vena cava syndrome]]. [[Superior vena cava syndrome]] is associated with a number of conditions that include [[malignant]] [[Tumor|tumors]], [[tuberculosis]], [[histoplasmosis]], and [[syphilis]].

==Causes==
[[Superior vena cava syndrome]] may be caused by [[obstruction]] of the [[superior vena cava]] by [[neoplastic]] [[Invasive (medical)|invasion]] of the [[Venous|venous wall]], [[intravascular]] [[thrombosis]], enlarged [[Lymph node|lymph nodes]], enlarged [[ascending aorta]], or by extrinsic [[pressure]] of a [[tumor]] against the thin-walled [[superior vena cava]].<ref name="wiki1">Superior vena cava syndrome. https://en.wikipedia.org/wiki/Superior_vena_cava_syndrome Accessed on January, 11 2016</ref>

==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]].<ref name="pmid26458355">{{cite journal |vauthors=Menon A, Gupta A |title=Superior vena cava syndrome |journal=Indian J. Med. Res. |volume=142 |issue=3 |pages=350 |year=2015 |pmid=26458355 |pmc=4669875 |doi=10.4103/0971-5916.166606 |url=}}</ref>

==Epidemiology and Demographics==

[[Superior vena cava syndrome]] is a common [[Oncologic emergencies|oncologic emergency]]. The [[incidence]] rate in the United States is approximately 15,000 individuals each year. The incidence of [[superior vena cava syndrome]] increases with age; the median age of diagnosis is between 40-60 years. [[Male|Males]] are more commonly affected with [[superior vena cava syndrome]] than [[Female|females]].<ref name="pmid17168344">{{cite journal |vauthors=Higdon ML, Higdon JA |title=Treatment of oncologic emergencies |journal=Am Fam Physician |volume=74 |issue=11 |pages=1873–80 |year=2006 |pmid=17168344 |doi= |url=}}</ref>

==Risk Factors==

The most potent risk factor in the development of [[superior vena cava syndrome]] is [[intrathoracic]] [[malignancies]]. Other common risk factors include [[thrombosis]] (due to intravascular devices), [[Fibrosis|postradiation fibrosis]], and [[mediastinitis]].<ref name="pmid1307879">{{cite journal |vauthors=Baker GL, Barnes HJ |title=Superior vena cava syndrome: etiology, diagnosis, and treatment |journal=Am. J. Crit. Care |volume=1 |issue=1 |pages=54–64 |year=1992 |pmid=1307879 |doi= |url=}}</ref>

==Screening==
According to the the National Cancer Institute, there is insufficient evidence to recommend routine screening for [[superior vena cava syndrome]].<ref name="screening">National Cancer Institute. Cardiopulmonary Syndromes. http://www.cancer.gov/about-cancer/treatment/side-effects/cardiopulmonary-pdq#section/_AboutThis_1 Accessed on January 13, 2016</ref>

==Natural History, Complications and Prognosis==

If left untreated, [[Patient|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 [[superior vena cava syndrome]] is approximately 10-20% at 6 months.<ref name="pmid17476012">{{cite journal |vauthors=Wilson LD, Detterbeck FC, Yahalom J |title=Clinical practice. Superior vena cava syndrome with malignant causes |journal=N. Engl. J. Med. |volume=356 |issue=18 |pages=1862–9 |year=2007 |pmid=17476012 |doi=10.1056/NEJMcp067190 |url=}}</ref>

==Diagnosis==
===Staging===
According to the Journal of Thoracic Oncology, there are 5 stages of [[superior vena cava syndrome]] based on the severity and [[incidence]]. Each stage is assigned a grade and a description that designate disease severity.<ref name="pmid17476012">{{cite journal |vauthors=Wilson LD, Detterbeck FC, Yahalom J |title=Clinical practice. Superior vena cava syndrome with malignant causes |journal=N. Engl. J. Med. |volume=356 |issue=18 |pages=1862–9 |year=2007 |pmid=17476012 |doi=10.1056/NEJMcp067190 |url=}}</ref><ref name="pmid18670297">{{cite journal |vauthors=Yu JB, Wilson LD, Detterbeck FC |title=Superior vena cava syndrome--a proposed classification system and algorithm for management |journal=J Thorac Oncol |volume=3 |issue=8 |pages=811–4 |year=2008 |pmid=18670297 |doi=10.1097/JTO.0b013e3181804791 |url=}}</ref>

===History and Symptoms===
The hallmark of [[superior vena cava syndrome]] is elevated [[jugular venous pressure]]. A positive history of [[cancer]] and intra-vascular devices are suggestive of [[superior vena cava syndrome]]. The most common [[Symptom|symptoms]] of [[superior vena cava syndrome]] include upper body [[swelling]], [[dyspnea]], and [[cough]].<ref name="pmid16502166">{{cite journal |vauthors=Uberoi R |title=Quality assurance guidelines for superior vena cava stenting in malignant disease |journal=Cardiovasc Intervent Radiol |volume=29 |issue=3 |pages=319–22 |year=2006 |pmid=16502166 |doi=10.1007/s00270-005-0284-9 |url=}}</ref>

===Physical Examination===

Common [[physical examination]] findings of [[superior vena cava syndrome]] include the [[pemberton's sign]], facial [[swelling]], and [[jugular venous distension]].<ref name="pmid16502166">{{cite journal |vauthors=Uberoi R |title=Quality assurance guidelines for superior vena cava stenting in malignant disease |journal=Cardiovasc Intervent Radiol |volume=29 |issue=3 |pages=319–22 |year=2006 |pmid=16502166 |doi=10.1007/s00270-005-0284-9 |url=}}</ref><ref name="caca">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</ref>

===Laboratory findings===

There are no [[Diagnosis|diagnostic]] [[Laboratory findings template|laboratory findings]] associated with [[superior vena cava syndrome]]. 

===Chest X Ray===
On [[Chest X-ray|chest x-ray]], indirect [[signs]] such as [[Superior mediastinal syndrome|superior mediastinal widening]] and right [[Hilar|hilar prominence]] may indicate the presence of a [[mediastinal mass]].<ref name="radio">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</ref>

===CT===
On enhanced [[Computed tomography|CT scan]], findings include location and severity of the [[superior vena cava]] [[obstruction]], superimposed [[thrombosis]], a [[mediastinal mass]] or [[lymphadenopathy]], [[Vessels|collateral vessels]], and associated [[Lung mass|lung masses]]. [[Computed tomography|CT scan]] is the [[Imaging|imaging modality]] of choice.<ref name="radio">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</ref>

===MRI===
[[MRI]] may be helful to provide images without the use of contrast (useful in patients with [[renal failure]]).<ref name="pmid11305067">{{cite journal |vauthors=Marcy PY, Magné N, Bentolila F, Drouillard J, Bruneton JN, Descamps B |title=Superior vena cava obstruction: is stenting necessary? |journal=Support Care Cancer |volume=9 |issue=2 |pages=103–7 |year=2001 |pmid=11305067 |doi= |url=}}</ref>

===Ultrasound===
[[Doppler ultrasound]] may be valuable in assessing the site and nature of the [[obstruction]] in [[superior vena cava syndrome]]. [[Venous]] patency and the presence of [[thrombi]] can also be assessed by using [[contrast]] and rapid scanning techniques.<ref name="pmid25247051">{{cite journal |vauthors=Birch A, Um D, Laselle B |title=Ultrasound detection of superior vena cava thrombus |journal=West J Emerg Med |volume=15 |issue=6 |pages=715–8 |year=2014 |pmid=25247051 |pmc=4162737 |doi=10.5811/westjem.2014.6.14006 |url=}}</ref>

===Other Imaging Findings===
Other imaging finding is the radionuclide technetium-99m venography.<ref name="pmid7924107">{{cite journal |vauthors=Muramatsu T, Miyamae T, Mashimo M, Suzuki K, Kinoshita S, Dohi Y |title=Hot spots on liver scans associated with superior or inferior vena caval obstruction |journal=Clin Nucl Med |volume=19 |issue=7 |pages=622–9 |year=1994 |pmid=7924107 |doi= |url=}}</ref>

===Other Diagnostic Studies===

Other diagnostic studies in the evaluation of superior vena cava syndrome include sputum tests, bronchoscopy, and biopsy. In addition, invasive contrast venography is also a diagnostic tool in the diagnosis of superior vena cava syndrome. It may be useful on the etiology of obstruction and exact location of the obstruction, also helpful in the surgical management of the obstructed vena cava.<ref name="pmid8790193">{{cite journal |vauthors=Mahmud AM, Isawa T, Teshima T, Hirano T, Anazawa Y, Miki M, Nukiwa T |title=Radionuclide venography and its functional analysis in superior vena cava syndrome |journal=J. Nucl. Med. |volume=37 |issue=9 |pages=1460–4 |year=1996 |pmid=8790193 |doi= |url=}}</ref>

==Treatment==

===Medical Therapy===

Superior vena cava syndrome is a medical emergency and requires prompt treatment. The treatment of superior vena cava syndrome depends on the etiology of the obstruction, the severity of the symptoms, the prognosis of the patient, patient preferences, and goals for therapy.<ref name="pmid17476012">{{cite journal |vauthors=Wilson LD, Detterbeck FC, Yahalom J |title=Clinical practice. Superior vena cava syndrome with malignant causes |journal=N. Engl. J. Med. |volume=356 |issue=18 |pages=1862–9 |year=2007 |pmid=17476012 |doi=10.1056/NEJMcp067190 |url=}}</ref>

===Surgery===

Elective stent placement and surgical bypass is recommended for all patients who develop superior vena cava syndrome.<ref name="pmid17476012">{{cite journal |vauthors=Wilson LD, Detterbeck FC, Yahalom J |title=Clinical practice. Superior vena cava syndrome with malignant causes |journal=N. Engl. J. Med. |volume=356 |issue=18 |pages=1862–9 |year=2007 |pmid=17476012 |doi=10.1056/NEJMcp067190 |url=}}</ref>

===Radiation Therapy===
Rapid radiotherapy  is recommended for all patients who develop superior vena cava syndrome secondary to malignant tumor. The feasibility of radiation therapy depends on the stage of cancer at diagnosis. Radiation dosage will depend on clinical features.<ref name="pmid17476012">{{cite journal |vauthors=Wilson LD, Detterbeck FC, Yahalom J |title=Clinical practice. Superior vena cava syndrome with malignant causes |journal=N. Engl. J. Med. |volume=356 |issue=18 |pages=1862–9 |year=2007 |pmid=17476012 |doi=10.1056/NEJMcp067190 |url=}}</ref>

===Primary Prevention===

There are no primary preventive measures available for superior vena cava syndrome.

==References==
{{Reflist|2}}

[[Category:Disease]]
[[Category:Hematology]]
[[Category:Cardiology]]
[[Category:Up-To-Date cardiology]]
[[Category:Up-To-Date]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Best pages]]

{{WH}}
{{WS}}