superior vena cava (SVC) syndrome
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Introduction
Obstruction of blood flow through the superior vena cava.
Etiology
- lung cancer, especially small cell carcinoma (75%)
- Hodgkin's lymphoma
- mediastinal fibrosis
- radiation fibrosis
- other mediastinal tumors
- thrombosis[6]
- goiter compressing subclavian vein & jugular vein[9]
Pathology
- 3 collateral venous pathways
- azygous vein
- internal thoracic vein
- long thoracic vein
- when venous pathways overwelmed, venous pressures become elevated
Clinical manifestations
- insidious onset
- dyspnea is the most common presenting symptom
- dysphagia
- cough
- chest pain
- prominent collateral veins (venous distension) over the chest
- jugular venous distension
- facial edema, Pemberton's Sign[9]
- fullness of the head, pethora
- headache
- arm swelling
- stridor (tracheal obstruction)
Diagnostic procedures
- bronchoscopy has a high yield & is generally safe
- not safe [NEJM knowlege+]
- mediastinoscopy may be associated with excessive hemorrhage due to obstructed venous return but may have a higher diagnostic yield
- complications 5%[2]
- thorcentesis if pleural effusion
- biopsy of peripheral lymphadenopathy
Radiology
- computed tomography (CT of chest) with IV contrast (diagnostic test of choice)
- mediastinal widening
- pleural effusion may be present
Complications
- tracheal obstruction
Management
- generally NOT regarded as a medical emergency
- generally MORE IMPORTANT to establish tissue diagnosis
- biopsy tissue external to obstructing mass
- if no external tissue to biopsy, biopsy mediastinal mass
- if tissue biopsy unsuccessful at establishing diagnosis, bronchoscopy, mediastinoscopy, thoracotomy
- treatment directed at specific etiology (type of cancer)
- radiation therapy (mainstay of treatment[2])
- palliation in 70% of patients with lung cancer & in 95% of patients with lymphoma
- appropriate even with unstaged NSCLC[6]
- chemotherapy: small cell lung cancer, germ cell neoplasms, NSCLC
- continuous infusion of heparin for thrombosis[6]
- radiation therapy (mainstay of treatment[2])
- endovascular stenting
- refractory disease, hempdynamic instability [10]
- rapid symptomatic improvement
- warfarin 1 mg daily with goal of INR < 1.6 ??[3]
- does not worsen prognosis of otherwise curable malignancies[2]
More general terms
Additional terms
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 721
- ↑ 2.0 2.1 2.2 2.3 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2021.
- ↑ 3.0 3.1 UpToDate 14.1 http://www.utdol.com
- ↑ Wan JF, Bezjak A. Superior vena cava syndrome. Hematol Oncol Clin North Am. 2010 Jun;24(3):501-13 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20488350
- ↑ Kumar B and Hosn NA Superior Vena Cava Syndrome N Engl J Med 2014; 371:1142. September 18, 2014 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25229918 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1311911
- ↑ 6.0 6.1 6.2 6.3 Wilson LD et al Superior Vena Cava Syndrome with Malignant Causes. N Engl J Med 2007; 356:1862-1869. May 3, 2007 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/17476012 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcp067190
- ↑ NEJM Knowledge+/ Question of the Week. July 26, 2016 http://knowledgeplus.nejm.org/question-of-week/936/
- ↑ DeFilippis EM, Vaidya A, Braun D et al A Shocking Turn of Events N Engl J Med. 2018 May 24;378(21):e29. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29791827 https://www.nejm.org/doi/full/10.1056/NEJMimc1710576
- ↑ 9.0 9.1 9.2 Abu-Shama Y, Cuny T. Pemberton's Sign in a Patient with a Goiter. N Engl J Med 2018; 378:e31. May 31, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29847764 https://www.nejm.org/doi/full/10.1056/NEJMicm1712263
- ↑ 10.0 10.1 NEJM Knowledge+ Hematology
- ↑ Superior Vena Cava Syndrome (PDQ) http://www.cancer.gov/cancertopics/pdq/supportivecare/superior-vena-cava/HealthProfessional