WordNet

a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
the 19th letter of the Roman alphabet (同)s

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
sulfurの化学記号 / {略}South[ern]

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/03/28 12:13:35」(JST)

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Superior vena cava syndrome (SVCS), is a group of symptoms caused by obstruction of the superior vena cava (a short, wide vessel carrying circulating blood into the heart). More than 90% of cases of superior vena cava obstruction (SVCO) are caused by cancer - most commonly bronchogenic carcinoma, typically a tumor outside the vessel compressing the vessel wall - but it can, sometimes, have a benign cause. 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 (a high-pitched wheeze) in 4%. The condition is rarely life-threatening, though edema of the epiglottis can make breathing difficult, and edema of the brain can cause reduced alertness, and in less than 5% of cases of SVCO, severe neurological symptoms or airway compromise are reported.^[1]

Signs and symptoms

Shortness of breath is the most common symptom, followed by face or arm swelling.^[2]

Following are frequent symptoms:

Difficulty breathing^[3]
Headache^[3]
Facial swelling^[3]
Venous distention in the neck and distended veins in the upper chest and arms^[3]
Upper limb edema^[3]
Lightheadedness^[2]
Cough^[2]
Edema (swelling) of the neck, called the collar of Stokes^[4]
Pemberton's sign^[3]

Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.^[2]

Cause

Thrombosis of the superior vena cava caused by an indwelling central venous catheter which caused superior vena cava syndrome

Approximately 90% of cases are associated with a cancerous tumor that is compressing the superior vena cava,^[2] such as bronchogenic carcinoma including small cell and non-small cell lung carcinoma, Burkitt's lymphoma, lymphoblastic lymphomas, pre-T-cell lineage acute lymphoblastic leukemia (rare), and other acute leukemias.^[2] Syphilis and tuberculosis have also been known to cause superior vena cava syndrome.^[2] SVCS can be caused by invasion or compression by a pathological process or by thrombosis in the vein itself, although this latter is less common (approximately 35% due to the use of intravascular devices).^[2]

Diagnosis

A CXR of a person with lung cancer which was causing superior vena cava syndrome.

A CT image showing compression of the right hilar structures by cancer.

The main techniques of diagnosing SVCS are with chest X-rays (CXR), CT scans, transbronchial needle aspiration at bronchoscopy and mediastinoscopy.^[3] CXRs provide the ability to show mediastinal widening and may show the presenting primary cause of SVCS.^[3] CT scans should be contrast enhanced and be taken on the neck, chest, lower abdomen and pelvis.^[3] They may also show the underlying cause and the extent to which the disease has progressed.^[3]

Treatment

Several methods of treatment are available, mainly consisting of careful drug therapy and surgery.^[2] Glucocorticoids (such as prednisone or methylprednisolone) decrease the inflammatory response to tumor invasion and edema surrounding the tumor.^[2] Glucocorticoids are most helpful if the tumor is steroid-responsive, such as lymphomas. In addition, diuretics (such as furosemide) are used to reduce venous return to the heart which relieves the increased pressure.^[2]

In an acute setting, endovascular stenting by an interventional radiologist may provide relief of symptoms in as little as 12-24 hours with minimal risks.

Should a patient require assistance with respiration whether it be by bag/valve/mask, BiPAP, CPAP or mechanical ventilation, extreme care should be taken. Increased airway pressure will tend to further compress an already compromised SVC and reduce venous return and in turn cardiac output and cerebral and coronary blood flow. Spontaneous respiration should be allowed during endotracheal intubation until sedation allows placement of an ET tube and reduced airway pressures should be employed when possible.

Prognosis

Symptoms are usually relieved with radiation therapy within one month of treatment.^[2] However, even with treatment, 90% of patients die within two and a half years.^[2] This relates to the cancerous causes of SVC that are 90% of the cases. The average age of onset of disease is 54 years of age.^[2]

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.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j ^k ^l ^m ⁿ emedicine > Superior Vena Cava Syndrome. Author: Michael S Beeson, MD, MBA, FACEP, Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine and Pharmacy; Attending Faculty, Summa Health System. Updated: Dec 3, 2009 Cite error: Invalid <ref> tag; name "emedicine" defined multiple times with different content (see the help page).
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j Parker, Robert; Catherine Thomas; Lesley Bennett (2007). Emergencies in Respiratory Medicine. Oxford. pp. 96–7. ISBN 978-0-19-920244-7.
^ define:collar of Stokes at open-resource-project.org. Retrieved Mars 2011

External links

Superior vena cava syndrome at eMedicine
Wilson LD, Detterbeck FC, Yahalom J (May 2007). "Clinical practice. Superior vena cava syndrome with malignant causes". N Engl J Med. 356 (18): 1862–9. doi:10.1056/NEJMcp067190. PMID 17476012.
Randolph HL Wong, Joshua Chai, Calvin SH Ng, et al. Transvenous pacing lead-induced Superior Vena Cava Syndrome: What do we know? (2009). Surgical Practice 13 (4): 125–126. doi:10.1111/j.1744-1633.2009.00462.x. Missing or empty |title= (help)

UpToDate Contents

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1. 悪性腫瘍関連上大静脈症候群 malignancy related superior vena cava syndrome
2. 肺癌の危険因子、病理、および臨床症状の概要 overview of the risk factors pathology and clinical manifestations of lung cancer
3. 小児癌患者の救急治療 emergencies in pediatric cancer patients
4. 線維性縦隔炎 fibrosing mediastinitis
5. カテーテル関連の上肢静脈血栓症 catheter related upper extremity venous thrombosis

English Journal

Before and After: Superior Vena Cava Syndrome Treated by Fibrinolysis.

Dias-Neto M1, Ramos JF2.
European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery.Eur J Vasc Endovasc Surg.2016 Feb 23. pii: S1078-5884(16)00047-2. doi: 10.1016/j.ejvs.2016.01.012. [Epub ahead of print]
PMID 26919934

Superior Vena Cava Syndrome with Cardiac Device-related Infective Endocarditis Secondary to Pacemaker Infection.

Sakurai M1, Tsurukiri J1.
The Journal of emergency medicine.J Emerg Med.2016 Feb 15. pii: S0736-4679(16)00003-2. doi: 10.1016/j.jemermed.2016.01.002. [Epub ahead of print]
PMID 26899510

Vertebral pseudometastases secondary to collateral circulation in superior vena cava syndrome.

Paniagua González M1, Alarcón Rodríguez JJ2.
Radiologia.Radiologia.2016 Feb 3. pii: S0033-8338(16)00011-4. doi: 10.1016/j.rx.2015.12.009. [Epub ahead of print]
PMID 26852377

Japanese Journal

Surgical Outcomes after Superior Vena Cava Reconstruction with Expanded Polytetrafluoroethylene Grafts

Nakano Tomoyuki,Endo Shunsuke,Kanai Yoshihiko,Otani Shinichi,Tsubochi Hiroyoshi,Yamamoto Shinichi,Tetsuka Kenji
Annals of Thoracic and Cardiovascular Surgery, 2013
… Objectives: Graft occlusion is a problem after superior vena cava (SVC) reconstruction for thoracic malignancy. … Expanded polytetrafluoroethylene (ePTFE) is considered to be an optimal material for venous reconstruction.Methods: We reviewed the hospital records of 13 patients who underwent complete resection of thoracic malignancy invading the SVC, including SVC reconstruction with ePTFE grafts. …
NAID 130003377905

特発性線維性縦隔炎による上大静脈症候群に合併したDownhill esophageal varicesの1例

青木耕平,澤藤誠,神山育男,江口圭介,大森泰,成松芳明
日本呼吸器外科学会雑誌 27(5), 642-649, 2013
症例は62歳，男性．特発性線維性縦隔炎による上大静脈狭窄に伴って発症した上大静脈症候群に対して，上大静脈内ステントを挿入し症状の改善を得たが，ステント挿入30ヵ月後に閉塞し，上大静脈症候群の再発が認められた．同時期に施行された上部消化管内視鏡検査にて食道全域に静脈の拡張と蛇行を認め，上大静脈症候群に伴い発症したDownhill esophageal varicesと診断した．内視鏡所見より経過観察 …
NAID 130003368812

Chronic Amiodarone Therapy Impairs the Function of the Superior Sinoatrial Node in Patients With Atrial Fibrillation

Mun Hee-Sun,Shen Changyu,Pak Hui-Nam,Lee Moon-Hyoung,Lin Shien-Fong,Chen Peng-Sheng,Joung Boyoung
Circulation Journal, 2013
… Only the amiodarone group had sick sinus syndrome (n=4, 22%, P=0.03) and abnormal (>550ms) corrected SAN recovery time (n=5, 29%; … The median distance from the junction of the superior vena cava (SVC) and RA to the most cranial earliest activation site (EAS) was longer in the amiodarone group than in the no-amiodarone group at baseline (20.5 vs. 10.6mm, P=0.04) and during isoproterenol infusion (12.8 vs. 6.3mm, P=0.03). …
NAID 130003361700

Related Pictures

★リンクテーブル★

リンク元	「上大静脈症候群」
関連記事	「SV」「S」「SVC」「syndrome」

「上大静脈症候群」

Superior vena cava syndrome (Mediastinal syndrome)
	Superior vena cava syndrome in a person with bronchogenic carcinoma. Note the swelling of his face first thing in the morning (left) and its resolution after being upright all day (right).
Classification and external resources
Specialty	cardiology
ICD-10	I87.1
ICD-9-CM	459.2
DiseasesDB	12711
MedlinePlus	001097
eMedicine	emerg/561
MeSH	D013479
	[edit on Wikidata]