A 55-year-old male presents with face and arm swelling that he says is worst when he wakes in the morning. He also notes a 10 pound unintentional weight loss over the past three months, plus new-onset exertional dyspnea. His past medical history is significant for hypertension, for which he takes hydrochlorothiazide. He has smoked cigarettes for the past 40 years and drinks alcohol occasionally. On physical examination, he is afebrile and his vital signs are within normal limits. His face is plethoric and dark-appearing. The veins on the anterior chest wall appear engorged. His abdomen is soft, non-tender, and non-distended, and there is no organomegaly. The lower extremities are non-edematous. What is the most appropriate next step in the management of this patient?
This patient's signs and symptoms are consistent with superior vena cava (SVC) syndrome, a condition where obstruction of the SVC impedes venous return from the head, neck and arms to the heart. Signs and symptoms include dyspnea, venous congestion, and swelling of the head, neck and arms. Malignancy is the most common cause of obstruction (i.e. lung cancer, non-Hodgkin lymphoma), accounting for >60% of cases. This patient's 40 year smoking history and recent 10 pound weight loss make lung cancer a likely diagnosis. Chest x-ray (Choice C) can identify the cause of SVC syndrome in >80% of cases. Abnormalities on chest x-ray warrant follow-up with chest CT and histology to determine the tumor type and to guide therapy.
(Choice A) Echocardiogram is not part of the work-up for SVC syndrome. Echocardiography can be helpful in suspected cardiac tamponade, which presents with Beck's triad of jugular venous distention, distant heart sounds and hypotension. Tamponade does not cause arm or face swelling or engorgement of the anterior chest wall veins.
(Choice B) Unexplained unilateral arm swelling warrants a Doppler to rule out DVT. However, simultaneous swelling of the face and bilateral arms indicates venous obstruction in the SVC, which upper extremity Doppler would not demonstrate.
(Choices D & E) Measurement of the 24-hour urinary protein excretion can help assess for nephrotic syndrome, and measurement of the serum albumin level can help assess for liver disease. These conditions can cause generalized edema, but not edema isolated to the upper half of the body.
Educational objective:
Malignancy is the most common cause of SVC syndrome. Lung cancer (particularly small cell lung cancer) and NHL are often implicated. Other possible causes include fibrosing mediastinitis (secondary to histoplasmosis or Tb infection) or thrombosis secondary to indwelling central venous devices. When the history and physical examination are suggestive, chest x-ray is warranted.