Superior Vena Cava Syndrome Clinical Presentation

  • Author: Todd A Nickloes, DO, FACOS; Chief Editor: Vincent Lopez Rowe, MD   more...
 
Updated: Aug 13, 2010
 

History

  • Early in the clinical course, partial superior vena cava (SVC) obstruction may be asymptomatic, but more often, minor symptoms and signs are overlooked.
  • As the syndrome advances toward total superior vena cava (SVC) obstruction, the classic symptoms and signs become more obvious.
    • Dyspnea is the most common symptom and is observed in 63% of patients with superior vena cava syndrome (SVCS).[7, 10]
    • Other symptoms include facial swelling, head fullness, cough, arm swelling, chest pain, dysphagia, orthopnea, distorted vision, hoarseness, stridor, headache, nasal stuffiness, nausea, pleural effusions, and light-headedness.[7, 10, 11]
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Physical

  • The characteristic physical findings of superior vena cava syndrome (SVCS) include venous distension of the neck and chest wall, facial edema, upper extremity edema, mental changes, plethora, cyanosis, papilledema, stupor, and even coma.
  • Bending forward or lying down may aggravate the symptoms and signs.
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Causes

  • More than 80% of cases of superior vena cava syndrome (SVCS) are caused by malignant mediastinal tumors.[12, 13, 14]
    • Bronchogenic carcinomas account for 75-80% of all these cases, with most of these being small-cell carcinomas.[3]
    • Non-Hodgkin lymphoma (especially the large cell type) represents 10-15% of cases.
  • Causes of superior vena cava syndrome (SVCS) appear similar to the relative incidence of primary lung and mediastinal tumors.
  • Rare malignant diagnoses include Hodgkin disease, metastatic cancers,[15] primary leiomyosarcomas of the mediastinal vessels, and plasmocytomas.[16, 17, 18]
  • Nonmalignant conditions causing superior vena cava syndrome (SVCS) include mediastinal fibrosis; vascular diseases such as aortic aneurysm, vasculitis, and arteriovenous fistulas; infections such as histoplasmosis, tuberculosis, syphilis, and actinomycosis; benign mediastinal tumors such as teratoma, cystic hygroma, thymoma, and dermoid cyst; cardiac causes, such as pericarditis and atrial myxoma; and thrombosis related to the presence of central vein catheters. These account for approximately 22% of the causes of superior vena cava syndrome (SVCS).[16, 19, 20, 21]
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Contributor Information and Disclosures
Author

Todd A Nickloes, DO, FACOS  Assistant Professor, Department of Surgery, Division of Trauma/Critical Care, University of Tennessee Medical Center-Knoxville

Todd A Nickloes, DO, FACOS is a member of the following medical societies: American College of Osteopathic Surgeons, American Medical Association, American Osteopathic Association, Association for Academic Surgery, Eastern Association for the Surgery of Trauma, Society of Critical Care Medicine, Society of Laparoendoscopic Surgeons, Southeastern Surgical Congress, and Southern Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

LaMar O Mack, MD  Resident Physician, Department of Surgery, University of Tennessee Medical Center

LaMar O Mack, MD is a member of the following medical societies: American Urological Association, National Medical Association, and Student National Medical Association

Disclosure: Nothing to disclose.

Andre M Kallab, MD  Clinical Associate Professor of Oncology, Medical College of Georgia; Consulting Staff, Department of Oncology, Northeast Georgia Diagnostic Clinic

Andre M Kallab, MD is a member of the following medical societies: American College of Physicians, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Allan Bernard Dunlap, MD  Fellow in Trauma/Surgical Critical Care, University of Tennessee Health Science Center College of Medicine

Allan Bernard Dunlap, MD is a member of the following medical societies: American College of Surgeons and Society for Vascular Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard M Stillman†, MD, FACS  Honorary Medical Staff, Northwest Medical Center; Former Chief of Staff and Medical Director, Wound Healing Center, Department of Surgery, Northwest Medical Center

Richard M Stillman†, MD, FACS is a member of the following medical societies: American College of Angiology, American College of Surgeons, Association for Academic Surgery, and Society of University Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Vincent Lopez Rowe, MD  Associate Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

Paolo Zamboni, MD  Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Vincent Lopez Rowe, MD  Associate Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

References

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Superior vena cava syndrome (case 1). The patient was a 35-year-old man with a 3-year history of progressive upper-extremity and fascial swelling. The patient had undergone treatment for histoplasmosis in the past. CT scan shows a narrowed superior vena cava with adjacent calcified lymph nodes and posterior soft tissue thickening.
Superior vena cava syndrome (case 1, cont'd). Sonogram shows markedly damped venous waveform with complete loss of normal venous pulsatility and minimal respiratory variation.
Superior vena cava syndrome (case 1, cont'd). Venogram shows almost complete occlusion of the superior vena cava with dramatic collateral drainage through the left superior intercostal vein.
Superior vena cava syndrome (case 1, cont'd). A Palmaz P308 stent mounted on a 12-mm balloon was deployed in the superior vena cava after it was predilated to 8 mm. The stent was subsequently dilated to 14 mm.
Superior vena cava syndrome (case 1, cont'd). Venogram obtained after stenting shows a widely patent superior vena cava with no collateral drainage. Pressure measurements after stenting showed a 1- to 2-mm residual gradient.
Superior vena cava syndrome (case 1, cont'd). Sonogram obtained 1 year after stenting shows near-normal venous pulsatility and respiratory phasicity. The patient experienced a complete resolution of symptoms.
 
 
 
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