The superior vena cava syndrome constitutes a constellation of signs and symptoms resulting from either extrinsic or intrinsic obstruction of the superior vena cava and causing congestion of venous outflow from the head, neck, and upper extremities. A resulting increase in venous pressure leads to dramatic signs and symptoms. According to Wilson¹⁰⁰ and Weinberg,⁹⁷ important factors that determine the nature of the clinical presentation include the rate with which the obstruction progresses, its completeness, and the location of the superior vena cava obstruction relative to the azygos vein. The most common presenting symptoms, best described by Yellin and associates,¹⁰⁴ include swelling of the face, neck, arms, and upper chest, often accompanied by venous distention. The eyes are often affected first. The patient may complain of tearing, proptosis, and conjunctival edema.⁸⁴ Retinoscopy may reveal edema and venous engorgement, as reported by Leys and associates.⁶³ The face may appear cyanotic or have a rubescent flush, as described by Parish,⁷⁸ Armstrong,⁸ and Chen¹⁹ (Fig. 177-1). Dilated venous channels often appear over the anterior chest wall. These signs and symptoms are greatly exaggerated when the azygos vein is also obstructed. Headache, dizziness, tinnitus, and a “bursting” sensation in the head on bending forward all follow in short succession. Venous hypertension can lead to the dire consequences of jugular venous and cerebrovascular thrombosis. Blindness may result from retinal venous thrombosis.

Since most cases of superior vena cava obstruction are caused by carcinoma of the lung, structures contiguous with the superior vena cava may also be involved. Respiratory complaints are the second most common presenting symptoms.¹⁰⁴ Symptoms ranging from a mild irritating cough to dyspnea and, with severe airway obstruction, even respiratory arrest may result from compression of the trachea or right main bronchus. The phrenic, vagus, and sympathetic nerves lie within the superior mediastinum; involvement of these structures can lead to paralysis of the right hemidiaphragm, hoarseness, pain, or Horner’s syndrome. The superior vena cava syndrome in children, unlike that in adults, often constitutes a medical emergency. Severe airway compromise is common owing to the tight thoracic compartment and markedly pliable tracheobronchial tree, as reported by Neuman⁷³ and Issa.⁵³

During the first half of the twentieth century, most reported cases of superior vena cava syndrome were the result of nonmalignant mediastinal disease. Syphilitic aneurysms accounted for nearly half of the reported cases. Successful treatment and prevention of this disease has now made syphilitic aneurysms and their sequelae a rarity. The author and others—Banker,¹³ Lochridge,⁶⁵ Ahmann,³ Helms,⁵¹ and Yellin¹⁰⁴—previously reported that malignancy accounted for more than 90% of all superior vena cava obstructions. These earlier studies indicated that 80% to 97% of cases of superior vena cava syndrome resulted from vena cava obstruction by mediastinal malignancy (Fig. 177-2). The rise in lung cancer was the most common cause of superior vena cava syndrome in the latter half of the twentieth century, paralleling the increased tobacco use following World War II. Most such carcinomas are of bronchial origin. Malignancy now remains the leading cause of superior vena cava syndrome. However, Rice and associates⁸² have found that malignancy now accounts for only 60% of the cases because of a
sharp rise in the use of intravascular devices related to benign obstructions. The superior vena cava syndrome occurs in approximately 3% to 15% of lung cancer patients, and— according to Weinberg,⁹⁷ Urban,⁹⁴ Elias,³⁴ Rice,⁸² and Wilson¹⁰⁰—small-cell lung cancer is the most frequent cell type associated with this syndrome. Lymphoma, following lung cancer, is the next most frequent malignancy causing this syndrome. A variety of other malignant neoplastic conditions have also been implicated in causing superior vena cava syndrome: Airan and associates⁴ reported malignant thymoma, Bishop¹⁶ and Masuda⁶⁷ described malignant thymoma, Liu⁶⁴ acknowledged myeloid leukemia, Osawa⁷⁶ cited gastric carcinoma, Kew⁵⁵ named hepatocellular carcinoma, Munjal⁷² reported liposarcoma, Aggarwal and coworkers² described mediastinal seminoma, Wakabayashi⁹⁶ identified metastic ependymoma, Dirix³¹ mentioned osteogenic sarcoma, and Davis²⁷ cited intrathoracic plasmacytoma as unusual examples of this syndrome. Common nonmalignant conditions predisposing to superior caval obstruction are substernal goiter, which was reviewed by Wesseling⁹⁸ and reported by de Perrot,²⁸ and fibrosing mediastinitis, reviewed by Esquivel,³⁶ Kulpati,⁵⁷ and Peters⁷⁹ (Fig. 177-3). The syndrome has also been described in association with Riedel’s thyroiditis by Abet.¹

The last few decades have ushered a greater use of diagnostic procedures, invasive monitoring, and therapeutic techniques performed intravenously, which have resulted in complications leading to the superior vena cava syndrome. Prolonged transvenous hemodynamic monitoring with pulmonary arterial catheters or injury to the superior vena cava at the time of cardiac catherization have led to caval obstruction and superior vena cava syndrome, as noted by Chetty²¹ and Ansari.⁶
Indwelling intravascular devices such as Quinton and Hickman catheters for long-term cancer chemotherapy, antibiotic therapy, and renal dialysis have been reported by Preston,⁸⁰ Guijarro-Escribano,⁴⁹ Ozcinar,⁷⁷ and Greenwell.⁴⁷ Numerous episodes of superior vena cava syndrome have resulted from scarring and thrombosis of the superior vena cava from transvenous cardiac pacing and defibrillator leads, as reported by Furman,³⁹ Koike,⁵⁶ Antonelli,⁷ Goudevenos,⁴⁴ Santangelo,⁸⁶ and Aryana¹⁰ and their associates. Intravascular devices are now the most common cause of superior vena cava syndrome in benign cases, accounting for 71% of benign obstructions according to Rice.⁸²

Vascular anomalies, aortic aneurysms, and aneurysms of the brachiocephalic vessels, once common in the 1950s and 1960s, are reported by Yavuzer¹⁰¹ to be important but rare causes of superior vena cava obstruction today. Other unusual vascular-related causes such as pulmonary artery aneurysm in Behçet’s disease and giant coronary artery aneurysm have been reported by Kajiya⁵⁴ and Kumar⁵⁸ respectively. The superior vena cava syndrome has also resulted from aortic pseudoaneurysms, as reported by Vydt⁹⁵ and Baldari¹² and their associates.

Superior vena cava syndrome may also occur after open cardiac surgical procedures, as reported by Garcia-Delgado,⁴⁰ Sze,⁹¹ and Blanche.¹⁷ The present author⁶⁸ reported a patient who developed the syndrome as a result of narrowing and thrombosis of the superior vena cava following orthotopic cardiac transplantation (Fig. 177-1). Surgical procedures for congenital heart disease that require intracardiac baffles, such as the Mustard operation for transposition of the great vessels, have been reported by Cumming and Ferguson²³ to result in superior vena cava syndrome because of scarring.

Miscellaneous causes of superior vena cava syndrome include thoracic trauma, reported by Shah⁸⁸; sarcoidosis, reported by Fincher³⁷; radiation-induced obstruction, reported by Lee⁶⁰; and amebic abscess, reported by Gupta.⁵⁰