Case Presentation:

A 56-year old woman with history of ESRD on hemodialysis presented with loss of consciousness. She had an AV fistula placed in her left forearm 5 days prior to presentation. She started feeling discomfort and swelling of the left side of her neck during a transcontinental flight, associated with severe lightheadedness. She continued to feel her neck swell upon exiting the plane and felt increasingly lightheaded before losing consciousness. She was alert upon awakening after a few minutes and immediately took a return flight for evaluation.

On arrival, she was awake and alert with normal vital signs. Her left upper extremity appeared mildly swollen and her left external jugular vein appeared full. Her AV fistula had no thrill or bruit. She had a clean and intact hemodialysis catheter in the right chest. Her exam was otherwise unremarkable. Values from her complete metabolic panel and complete blood count were at baseline and consistent with her ESRD. She had received hemodialysis 2 days prior to presentation. Duplex of the left arm showed a thrombus in the outflow cephalic vein of her fistula. A chest CT did not show signs of a pulmonary embolus, however, it showed venous collaterals in the mediastinum suggestive of central vein stenosis. Electrocardiogram, troponin, orthostatic vitals and a transthoracic echocardiogram were normal.

The patient had persistent symptoms of fullness in her left neck associated with lightheadedness. She underwent a fistulogram and venogram by IR. Her fistula thrombus was treated by balloon angioplasty. The venogram revealed moderate stenosis of the left brachiocephalic vein, which was treated by balloon angioplasty. The patient reported immediate improvement in her symptoms after the procedure and no longer experienced pre-syncope. Her left arm edema and engorgement of her left external jugular vein appeared improved the following day. Her AV fistula regained a bruit and thrill. She was subsequently discharged home. 

Discussion:

Central vein stenosis is a common finding in hemodialysis patients, especially in those with a history of having a central venous catheter. They are usually asymptomatic and found incidentally on imaging. When they are symptomatic, typical symptoms include ipsilateral arm edema and development of collateral veins. A variety of atypical symptoms have been reported including hearing loss and upper airway obstruction. This case represents another atypical presentation of symptomatic brachiocephalic vein stenosis. Her syncope and persistent pre-syncope thereafter were likely a result of the venous hypertension triggering either a vasovagal response or stimulation of her carotid sinus. Balloon angioplasty proved to be a successful intervention in this case. 

Conclusions:

Hospitalists should be familiar with the symptoms and evaluation of central vein stenosis given its high prevalence in ESRD patients.