Superior Vena Cava Syndrome (SVCS) is caused by lung cancer, especially small cell, and lymphoma in approximately 90% of cases. A minority of cases are caused by fibrosing mediastinitis and thrombosis of indwelling central venous devices. Syphilitic aortic aneurysm and tuberculosis use to be common causes in developed countries. Symptoms include: facial or neck swelling (82%), arm swelling (68%), dyspnea (66%), cough (50%), dilated chest veins (38%), and orthopnea is commonly noted. Facial swelling and plethora are exacerbated when the patient is supine and cyanosis can be dramatic. Chest xray will show a lesion in 84% of cases, with mediastinal widening in 64% and pleural effusion in 26%. Computed tomography with IV contrast is the definitive imaging study as it evaluates the extent of blockage and evaluates collateral pathways of drainage. Survival ranges from 1.5-9.5 months.
SVCS is now known not to be a major threat to life in most clinical scenarios. Emergent intervention will be needed in cases with respiratory or neurologic compromise. It is recommended that staging and tissue diagnosis be completed before initiating chemotherapy, radiotherapy or steroid treatment if possible. Initial management should include supportive care, such as elevation of the head of bed, oxygen support, diuretics, and possibly steroids for severe obstruction, although none have been proven to be of benefit. Intravascular stenting and anticoagulation my be needed urgently for patients with severe symptoms or thrombosis, respectively. Radiation, chemotherapy, or stent placement, or a combination will play a role as the definitive intervention.
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