A 92-year-old woman with a history of hypertension, atrial fibrillation, prior DVT, and thyroid nodule was brought to the ED for a cough producing yellow sputum, shortness of breath, facial swelling, rhinorrhea, and mild headache. She had no sore throat, hoarseness, fever, or chills.
Her blood pressure was 168/88 mm Hg, and her other vital signs were within normal limits. Her lungs were clear to auscultation, her pulse was irregular without any murmurs, and she had distended veins to the chest wall. The patient's CBC and chemistry panel were all within normal limits except for a sodium of 144 mmol/L. She had an NT-proBNP of 1,827 pg/mL. A two-view chest x-ray was done, and the ECG revealed atrial fibrillation and left ventricular hypertrophy.
Find the diagnosis and case discussion on the next page.
Diagnosis: Superior Vena Cava Syndrome
Superior vena cava syndrome (SVCS) occurs from extraluminal or intraluminal compression of the superior vena cava. The most common symptom of SVCS is neck swelling. One report found that all patients with SVCS presented with this complaint. Other symptoms include dyspnea, headache, blurred vision, hoarseness, and extremity, facial, and tongue swelling. (SpringerPlus. 2016;5:229, http://bit.ly/2vN91mz; Semin Intervent Radiol. 2017;34:398.)
More than 15,000 patients are diagnosed with SVCS annually. (Emerg Med Clinics N Am. 2018;36:577, http://bit.ly/2HarSOU; Semin Intervent Radiol. 2017;34:398.). Approximately 90 percent of cases of SVCS are caused by malignancy, 75 percent by lung cancer and 15 percent by non-Hodgkin lymphoma. Breast cancer has been associated with SVCS to a lesser degree. Benign causes of SVCS include implanted devices involving veins with associated thrombosis, infections, vascular disease, benign tumors of the mediastinum, cardiac conditions, and mediastinal fibrosis. (Hematol Transfus Cell Ther. 2018;40:75; http://bit.ly/2WwaWYc.)
SVCS is diagnosed with an appropriately high index of suspicion, typically in a patient with known lung cancer, non-Hodgkin lymphoma, risk for intravascular thrombosis, and neck or face swelling. A chest x-ray rarely adds to the diagnosis, though at times, as in this patient, a mass in the mediastinum may be identified. A CT scan with IV contrast is typically the fastest, easiest, and most available imaging modality for assessing patients with suspected SVCS and can also provide information about the possible causes. (Emerg Med Clinics N Am. 2018;36:577, http://bit.ly/2HarSOU.) Ultrasound, though unable to visualize the SVC directly, may identify a thrombus distal to the superior vena cava or dampening and loss of pulsatility in the brachiocephalic and subclavian veins, suggestive of SVC obstruction. MRI can be used in patients who have allergies to IV contrast.
Airway obstruction secondary to laryngeal and cerebral edema is one of the most serious complications of SVCS. These patients are rarely hypotensive, and appropriate resuscitation is indicated before symptomatic treatment.
Following the assessment of mental status and airway, elevating the head of the bed, supplemental oxygen, and parenteral steroids may be helpful. Anticoagulation or thrombolysis may also be indicated in patients with thrombosis. (Emerg Med Clinics N Am. 2018;36:577, http://bit.ly/2HarSOU.)
Radiation therapy with or without steroids has been an established treatment for SVCS patients with malignancy causing external compression of the SVC. Recent advances in percutaneous stenting have shown symptom relief within 72 hours, and provide the option of treating the obstruction at the targeted source. (SpringerPlus. 2016;5:229, http://bit.ly/2vN91mz.)
Our patient was admitted to the hospital after a CT scan revealed a large nodular thyroid with substernal extension, causing mass effect on the trachea with deviation to the right and moderate narrowing and compression of the brachiocephalic and subclavian veins. Her TSH, T3, and free T4 were all within normal limits, and otolaryngology was consulted. The tracheal deviation and narrowing were thought not to be severe enough to require intervention, and she was treated conservatively. Her symptoms improved, and she was discharged home for outpatient workup and treatment with her primary care physician, otolaryngology, and endocrinology.
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Dr. Queenis associate staff andDr. Smalleyis associate staff and the associate ultrasound director at the Emergency Services Institute at the Cleveland Clinic. Follow her on Twitter@SmallsSono. Read past Quick Consult columns athttp://bit.ly/EMN-QuickConsult.