History and exam

Key diagnostic factors

common

history of smoking, pacemaker, or central venous catheter

Lung cancer is the most common overall cause of superior vena cava syndrome, while multiple pacemaker leads and central venous catheters are becoming increasingly frequent benign causes of the condition.

localized edema of the face and upper extremities

Present in 80% of cases.[9]

If edema is localized to upper extremities and face, obstruction of the superior vena cava should be considered.

dyspnea

Present in 60% of cases.[20]

Usually made worse by bending forward or lying down (orthopnea).

May suggest lung malignancy or chronic infection.

facial plethora

Due to venous engorgement and edema.

cough

Present in 54% of cases.[10]

Can be related to underlying etiology or laryngeal edema.

distended neck veins

Seen in 63% of cases and due to increased venous pressure.[1]

Bending forward usually worsens venous engorgement and is a helpful clinical sign.

distended chest veins

Seen in 53% of cases and due to increased venous pressure.[1]

Prominent collateral veins covering the anterior chest wall may be visible.

Bending forward usually worsens venous engorgement and is a helpful clinical sign.

hoarseness of voice

Present in 17% of cases.[6]

Can be related to underlying etiology or laryngeal edema.

lymphadenopathy

Lymphoma is a possibility if lymphadenopathy is outside of the chest.

uncommon

blurred vision

Present in 2% of cases.[6]

stridor

Present in 4% of cases.[6]

Related to laryngeal edema or direct compression.

confusion/stupor

Present in 4% of cases and due to cerebral edema.

Has been described with severe obstruction.[1][6]

Other diagnostic factors

common

anorexia

May suggest lung malignancy or chronic infection.

weight loss

May suggest lung malignancy or chronic infection.

hemoptysis

May suggest lung malignancy or chronic infection.

uncommon

headache

Present in 9% of cases.[6]

Due to increased cerebral venous pressure.

chest pain

Usually pleuritic; related to pleural involvement from malignancy, infection, or autoimmune diseases.

mental changes

Has been described with severe obstruction.[1][6]

fever

May be indicative of collagen-vascular disease.

skin rash

May be indicative of collagen-vascular disease.

arthralgia

May be indicative of collagen-vascular disease.

laryngeal edema

Has been described with severe obstruction.[1][6]

cyanosis

Has been described with severe obstruction.[1][6]

papilledema

Has been described with severe obstruction.[1][6]

coma

Has been described with severe obstruction.[1][6]

Risk factors

strong

smoking

Strong relationship to lung cancer, the most common overall cause of superior vena cava syndrome.

multiple pacemaker leads

Becoming an increasingly frequent benign cause of superior vena cava syndrome.

central venous catheters/ports

Becoming an increasingly frequent benign cause of superior vena cava syndrome.[7]

weak

age >50 years

Lung malignancy should be considered as the most likely etiology in patients >50 years of age.

radiation

Excess radiation to the mediastinum can lead to fibrosis causing superior vena cava obstruction.

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