A 21-year-old woman with a history of G6PD deficiency presented with three to four weeks of shortness of breath. She had swelling, pain, and pressure to the face, neck, and chest, and also reported hemoptysis, dyspnea on exertion, and orthopnea. She was seen three times at her home clinic, which originally suspected viral upper respiratory infection. She had a negative CBC, chemistry panel, TSH, and HIV labs, and experienced no relief from antibiotics.
Her vitals were notable for tachycardia at 119 bpm, a temperature of 36.1°C, tachypnea at 25 bpm, and oxygen saturation of 95% on room air. She appeared in respiratory distress, with shallow, diminished breath sounds. She had perioral cyanosis, and was diaphoretic. She felt a hard cord on the right side of her neck, and had visible swelling around the neck, which made it painful for her to perform range-of-motion testing. Pictures of the patient a month before her visit revealed dramatic facial swelling.
What is this patient's diagnosis? Find it and a case discussion on p. 29.
Diagnosis: Superior Vena Cava Syndrome
Superior vena cava syndrome (SVCS) occurs when the superior vena cava is obstructed, and about 15,000 cases occur in the United States each year. (Int J Angiol 2008;17:43.) Obstruction is mainly the result of a pathological process of adjacent structures, which includes thrombosis, masses, and untreated infection before the widespread use of antibiotics.
SVCS was initially thought of as an infectious process and was first described in 1757 in a patient with an aortic aneurysm secondary to syphilis, but today malignant masses are the most common cause of SVCS and account for more than 90 percent of cases. (Int J Angiol 2008;17:43.) The most prevalent causes of the malignancies are non-small cell lung cancer, small cell lung cancer, and non-Hodgkin lymphoma. (N Engl J Med 2007;356:1862.) Thrombotic causes of SVCS, however, are increasing because of the rising number of catheters and pacemakers in use. (Int J Angiol 2008;17:43.)
As the SVC becomes occluded, venous collaterals develop alternative pathways to bring blood back to the heart. Collateral veins dilate and produce the characteristic signs and symptoms of this syndrome over several weeks, including dyspnea (most common) and edema to the head and neck that can compress superficial and deep structures. (J Clin Oncol 1984;2:260.) Other symptoms seen in patients with SVCS include cough, chest pain, dysphagia, headache, confusion, coma, and discoloration of the neck, face, and upper extremities. Cerebral edema can occur as a result of venous congestion, and can lead to ischemia, herniation, or death. Diagnosis of SVCS is mostly based on clinical signs and symptoms, though chest x-rays, CT scans, and venography can assist.
Treatment is often based on the cause of SVCS. Chemotherapy and radiation can help reduce the tumor burden if it is cancer-related. Intravenous stents (percutaneous) are also increasing in use. Steroids, diuretics, anticoagulation medications, keeping the patient sitting up, and thrombolytics have also been used as adjuvant therapy while waiting for more definitive therapy to start working. Sixty percent of patients with SVCS have had pleural effusions. (Int J Angiol 2008;17:43.) Patients with SVCS usually have advanced malignancies, and mortality is high.
Imaging in the emergency department revealed that our patient had a left-sided mass in the mediastinum causing deviation of the upper trachea and hypopharynx. She was diagnosed during admission with mediastinal B-cell lymphoma causing superior vena cava syndrome. She was treated with chemotherapy.
This patient was also found to have a rapidly enlarging pericardial effusion with tamponade physiology and bilateral brachiocephalic deep vein thrombosis revealed on bedside ultrasound prior to admission. This was managed immediately through admission to the cardiac ICU for image-guided paracentesis before further workup of the mediastinal mass that was later diagnosed as a B-cell lymphoma causing SVCS.
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