Superior Vena Cava Syndrome Airway Edema: Assessment and Management

Significant Statement

Superior vena cava (SVC) syndrome can be a life-threatening complication that requires close airway monitoring by otolaryngologists while the patient undergoes interventions to address the underlying cause. In this article, we present a unique case of upper airway edema secondary to thrombosis-related SVC syndrome and provide an overview of the condition’s presentation and management.

Case Description

A 19-year-old male undergoing hemodialysis for end-stage renal disease presented to the emergency department with a cough and a nonfunctioning right internal jugular permanent catheter. He had no stridor or dyspnea at the time of admission. Vitals were significant for hypotension, tachycardia, tachypnea, and fever. He underwent an infectious workup, started supportive measures and antibiotics, and was hospitalized for sepsis. Three days after admission, the patient experienced difficulty breathing and throat pain. A noncontrast computed tomography (CT) scan, which was performed given his poor renal function, showed a retropharyngeal fluid collection (Figure 1). The otolaryngology service conducted a rapid airway assessment via bedside flexible laryngoscopy, revealing an edematous posterior pharyngeal wall and moderate edema of the supraglottic structures. The airway was otherwise patent. The otolaryngology team recommended transfer to the intensive care unit for close airway monitoring and treatment with intravenous dexamethasone. Twelve hours later, the patient developed noticeable facial swelling and worsening dyspnea. The airway was reevaluated via flexible laryngoscopy, which showed an unchanged edematous posterior pharyngeal wall. The leading diagnosis at this time was superior vena cava (SVC) syndrome, secondary to venous thrombosis near the site of the permanent catheter tip. The patient underwent a CT angiogram the following morning, which showed the catheter ending in the SVC with multiple thromboses surrounding the tip. The imaging also demonstrated worsened diffuse subcutaneous and deep fascial edema (Figure 1). The patient underwent removal and replacement of the permanent catheter and balloon angioplasty of the SVC, brachiocephalic, and right internal jugular vein. Two days later, he underwent a mechanical thrombectomy of these veins.

Figure 1.

CT scan of neck revealing a retropharyngeal effusion measuring 24.1 mm. CT, computed tomography.

The swelling of the face and neck initially improved. However, the next day, the patient experienced acute neck swelling suspicious for recurrent thrombosis and SVC syndrome. Repeat scope examination showed no worsened upper airway edema. A repeat CT scan of neck confirmed a reocclusion of the SVC. A decision was made to keep the patient on anticoagulation and monitor his status. He experienced gradual improvement in edema and respiratory status without any requirement of further surgical intervention. The patient remained without any airway complaints and was discharged home 8 days later.

Discussion

SVC syndrome results from obstruction of flow through the SVC, which causes increased venous pressure and swelling of the tissues of which venous flow drains into the SVC. Common symptoms include swelling of the neck or face, distended neck veins and chest veins, dyspnea, and cough.^1,2 Retropharyngeal effusion, typically caused by an infectious process, is a rarely reported sign of SVC syndrome.^{3 -5} However, SVC syndrome should lead the evaluating clinician to assess for swelling or effusion of the upper airway. While SVC syndrome is most frequently caused by malignancy, the most common benign causes include central venous catheters, pacemakers, and indwelling hemodialysis catheters. Treatment generally consists of addressing the underlying cause of venous congestion. In the case of this patient, obstruction of the SVC from the thromboses caused a pressure increase in the jugular veins and decreased venous drainage of the neck, which caused fluid to effuse in the retropharyngeal space.

Airway monitoring plays a crucial role in the management of SVC syndrome. SVC syndrome due to thrombosis and a hypercoagulable state is susceptible to recurrence as shown by our case discussion.^1,5 Endovascular approaches and anticoagulation may alleviate the obstruction but definitive treatment can be challenging. Therefore, patients with airway symptoms should be evaluated by an otolaryngologist with flexible laryngoscopy to assess airway patency and obstruction progression while definitive therapies are sought. A repeat flexible laryngoscopy should be conducted immediately if the patient develops worsening dyspnea or voice changes, and patients may require close monitoring in an intensive care unit. Worsening airway edema or swelling indicates treatment with short-course, high-dosed steroids. Additional doses can be considered if symptoms recur. SVC syndrome-induced airway edema can be complex and involve a collaborative treatment plan involving otolaryngologists.