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Internal Medicine

Superior Vena Cava Syndrome (SVCS)

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). | By Herbert L. Fred, MD and Hendrik A. van Dijk - http://cnx.org/content/m14895/latest/, Attribution, https://commons.wikimedia.org/w/index.php?curid=11079126
Cover image: 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). | By Herbert L. Fred, MD and Hendrik A. van Dijk - http://cnx.org/content/m14895/latest/, Attribution, https://commons.wikimedia.org/w/index.php?curid=11079126

Gradual compression of the SVC, leading to edema and retrograde flow, but it can also be caused more abruptly in thrombotic cases.

History:

First described by William Hunter in 1757 in a patient with a large syphilitic aortic aneurysm compressing the SVC.

Etiology

Malignant (>85%):

  • Non-small cell lung cancer (NSCLC) (50%)
  • Small cell lung cancer (SCLC) (25%)
  • Non-Hodgkin’s lymphoma (10%)
  • Others causes (<10%): Solid tumors with mediastinal lymph node metastasis, thymomas, mesothelioma, mediastinal germ cell tumors 

Benign (3-15%):

  • Indwelling catheters
  • Cystic hygroma
  • Tuberculosis
  • Histoplasmosis
  • Thyroid goiter
  • Aortic aneurysm

Presentation

Typically dyspnea, facial swelling, neck distension and cough developed over a period of 10 days.

  • Dyspnea (M/C reported symptom, (54-83% cases)
  • Swelling of neck (100%), trunk and/or upper extremeties (38-75%), face (48-82%)
  • Head fullness
  • Neck distension (exacerbated by bending forward or lying down)
  • Cough (secondary to functional compromise of the upper airways, 22-58%)
  • Chest pain (15%)
  • Dilated chest vein collaterals (38%)
  • Weight loss (10-31%)
  • Jugular venous distension (27%)
  • Phrenic nerve paresis (16.2%)
  • Plethora (13%)
  • Dysphagia (10-13%)
  • Rare symptoms: Hoarseness, headache, confusion, dizziness, night sweats, hypoxia, hyponatremia, and syncope
The Pemberton maneuver is a physical examination tool used to demonstrate the presence of latent pressure in the thoracic inlet. The maneuver is achieved by having the patient elevate both arms until they touch the sides of the face. A positive Pemberton’s sign is marked by the presence of facial congestion and cyanosis, as well as respiratory distress after approximately one minute. A positive Pemberton’s sign is indicative of superior vena cava syndrome (SVC), commonly the result of a mass in the mediastinum. | Wallace, C. and Siminoski, K. (1996) ‘THe pemberton sign’, Annals of Internal Medicine, 125(7), pp. 568–569. Available at: http://dx.doi.org/10.7326/0003-4819-125-7-199610010-00006.

Diagnosis

Chest X-ray:

  • Widening of the superior mediastinum (64% cases)
  • Pleural effusion (26% cases)
A CXR of a person with lung cancer which was causing superior vena cava syndrome. | By James Heilman, MD – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=14634345

CECT:

Differential diagnosis:

  • Cardiac tamponade
  • Mediastinitis
  • Thoracic Aortic aneurysm
  • Tuberculosis

Management

Management algorithm for superior vena cava syndrome (SVCS) | Lepper, P. M., Ott, S. R., Hoppe, H., Schumann, C., Stammberger, U., Bugalho, A., Frese, S., Schmücking, M., Blumstein, N. M., Diehm, N., Bals, R. and Hamacher, J. (2012) ‘Superior Vena Cava Syndrome in Thoracic Malignancies’, Respiratory Care, 56(5), p. 653 LP-666. Available at: http://rc.rcjournal.com/content/56/5/653.abstract.
Stent placement for superior vena cava (SVC) syndrome. a Pre-stenting: SVC occluded by large tumor. White arrow SVC. Black Chevron tumor occluding SVC. b Post-stenting: SVC now patent. White arrow stent in SVC. Black Chevron tumor surrounding SVC | Straka, C., Ying, J., Kong, F.-M., Willey, C. D., Kaminski, J., & Kim, D. W. N. (2016). Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome. SpringerPlus, 5, 229. https://doi.org/10.1186/s40064-016-1900-7

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