Superior Vena Cava Syndrome - Etiology & Diagnosis

 1/22/03 (Desai)

Question: What are common and uncommon causes of superior vena cava syndrome (or obstruction) and what are some clinical diagnostic clues?

<8>

Unique Identifier:1307879

Authors: Baker GL. Barnes HJ.

Institution: Kansas University Medical Center, Kansas City 66103.

Title: Superior vena cava syndrome: etiology, diagnosis, and treatment. [Review] [40 refs]

Source: American Journal of Critical Care. 1(1):54-64, 1992 Jul.

Abstract: Superior vena cava (SVC) syndrome is a critical condition in which an intrathoracic mass lesion compresses the SVC and promotes the development of head and upper body edema and cyanosis. SVC syndrome develops in 10% of patients with a right-sided malignant intrathoracic mass lesion. Diagnostic evaluation and emergency therapy are always necessary to assess and alleviate airway obstruction, cerebral venous hypertension and symptoms secondary to mediastinal compression. Radiation therapy and venous bypass of the obstructed SVC are both used successfully as early treatment. Although radiation therapy to the malignant process may provide initial decompression, a more sustained decrease in venous pressure occurs in patients who also undergo decompressive SVC surgical bypass. SVC bypass should be considered early in the course of patients with profound cerebral or laryngeal edema, patients with extensive thrombosis of the SVC, and in rare patients afflicted with severe venous hypertension and in whom a tissue diagnosis requires a mediastinal exploration. [References: 40] CAS Registry/EC Number 0 (Adrenal Cortex Hormones). 0 (Anticoagulants). 0 (Diuretics).

<15>

Unique Identifier:8462332

Authors: Abner A.

Institution: Joint Center for Radiation Therapy and Harvard Medical School, Boston.

Title: Approach to the patient who presents with superior vena cava obstruction. [Review] [28 refs]

Source: Chest. 103(4 Suppl):394S-397S, 1993 Apr.

Abstract: Obstruction of the superior vena cava (SVC), a thin-walled vessel that carries blood at low pressure, may result from compression by tumor or intraluminal thrombus formation. The SVC syndrome that ensures may be the cause of significant morbidity. This article reviews the pathogenesis of SVC syndrome, appropriate radiologic imaging procedures, and the issue of obtaining a tissue diagnosis. The management of SVC syndrome with chemotherapy, radiotherapy, and surgery is also discussed. [References: 28]

<18>

Unique Identifier:7253702

Authors: Parish JM. Marschke RF Jr. Dines DE. Lee RE.

Title: Etiologic considerations in superior vena cava syndrome.

Source: Mayo Clinic Proceedings. 56(7):407-13, 1981 Jul.

Abstract: The Mayo Clinic experience with superior vena cava obstruction during the last 20 years was reviewed. The diagnosis of superior vena cava obstruction is often made at the bedside. Typical symptoms include suffusion, dyspnea, cough, and, less commonly, pain, syncope, dysphagia, and hemoptysis. The most important physical findings are the increased collateral veins covering the anterior chest wall and the dilated neck veins with edema of the face, arms, and chest. The chest x-ray film usually shows widening of the superior mediastinum. Of our 86 cases of superior vena cava obstruction, 67 (78%) were due to malignancy and 19 (22%) to benign causes. The cause of obstruction is usually established by bronchoscopy, open lung biopsy, or biopsy of the superficial lymph node. Radiotherapy remains the standard approach for the treatment of superior vena cava obstruction due to malignant disease. It is of particular interest to note that of the six benign cases resulting from thrombosis of the superior vena cava, three were due to the use of central venous catheters. Physicians should be aware of this association.

<21>

Unique Identifier:1149526

Authors: Mahajan V. Strimlan V. Ordstrand HS. Loop FD.

Title: Benign superior vena cava syndrome.

Source: Chest. 68(1):32-5, 1975 Jul.

Abstract: Benign superior vena cava (SVC) obstruction is an uncommon entity. However, it is important to recognize that a small percentage of SVC syndromes are due to benign diseases such as mediastinal granulomas. The insidious onset and slow progression of symptoms allow for development of an efficient collateral venous circulation compatible with long-term survival. Surgical intervention to bypass the obstruction is often unsuccessful and should be avoided in most cases. We review the English literature on the subject, classify the various causes of benign SVC syndrome, and report our experience with 16 documented cases.

 1.6922.1.83

<1>

Unique Identifier:9385120

Authors: Anders H. Keller C.

Institution: Medizinische Poliklinik, Klinikum Innenstadt, Ludwig-Maximilians-University Munich, Pettenkoferstr. 8a, Munich, D-80336 Germany.

Title: Pemberton's maneuver - a clinical test for latent superior vena cava syndrome caused by a substernal mass.

Source: European Journal of Medical Research. 2(11):488-90, 1997 Nov 28.

Abstract: A 62-year-old white male presented with facial erythema and jugular vein distension in upright position which progressed to cyanosis and facial edema while keeping both arms elevated (Pemberton sign). A CT scan revealed a large substernal goiter as the cause of superior vena cava syndrome. Symptoms and signs disappeared after thyroid surgery. First reported in 1946 Pemberton's maneuver is a useful but unrecognized clinical sign for oligosymptomatic superior vena cava syndrome caused by a substernal mass.

<3>

Unique Identifier:1626963

Authors: Hirschmann JV. Raugi GJ.

Institution: Veterans Affairs Medical Center, University of Washington School of Medicine, Seattle 98108.

Title: Dermatologic features of the superior vena cava syndrome.

Source: Archives of Dermatology. 128(7):953-6, 1992 Jul.

Abstract: BACKGROUND--The superior vena cava syndrome occurs when extrinsic compression or intraluminal occlusion impedes blood flow through this vessel. The most common underlying cause is a malignant neoplasm, especially a bronchogenic carcinoma. This article describes the cutaneous findings of this disorder. OBSERVATIONS--Among the earliest and most prominent features are numerous, dilated, vertically oriented, and tortuous cutaneous venules or veins above the rib cage margin. Recognition of this cutaneous sign allowed us to make a diagnosis of lung cancer in several patients. Other features include upper body edema and ruddiness or cyanosis, distended neck veins, proptosis, and conjunctival suffusion. CONCLUSIONS--Detecting the characteristic cutaneous features can lead to an early diagnosis of the superior vena cava syndrome. These skin changes usually represent indirect dermatologic signs of an underlying malignant neoplasm; for most patients, this syndrome is the initial manifestation of their cancer. The most common cause is a bronchogenic carcinoma, especially the small-cell variety, but others include lymphomas, primary mediastinal tumors, and metastases to the mediastinal lymph nodes from extrathoracic primary tumors, especially breast cancer. Treatment of the underlying malignant neoplasm and relief of the obstruction produce prompt improvement in the dermatologic findings.