Superior Vena Cava Obstruction: A Rare Cause of Recurrent Esophageal Variceal Bleeding

Abstract

“Downhill” esophageal varices are formed in upper two-thirds of the esophagus as a consequence of a superior vena cava obstruction. We present a case of 55-year-old African-American female with a medical history of multiple comorbidities, including end-stage renal disease, who presented with an upper gastrointestinal bleed and was found to have distended neck veins on physical examination. She gave a history of the insertion of an intravenous central line in her neck area for hemodialysis purposes about six years previously. An endoscopy showed the presence of esophageal varices and computed tomography (CT) of the abdomen showed the presence of a superior vena cava (SVC) obstruction. The patient was managed supportively. This case represents a rare cause of acute upper gastrointestinal bleeding in an individual with a central line for dialysis leading to SVC thrombosis.

Hematemesis, a Rare Presentation for Downhill Esophageal Varices

“…DEV are a rare form of varices first described in 1964 by Felson and Lessure [ 8 ]. Another form called uphill esophageal varices (UEV) are found in the setting of portal hypertension [ 1 , 3 , 7 , 9 ]. UEV tend to have a higher risk for bleeding compared to DEV for a couple of reasons.…”

“…UEV are found in the superficial sub-epithelium near the gastroesophageal junction, are damaged frequently from refluxed acid, and tend to be found in the setting of coagulopathy secondary to liver dysfunction [ 1 , 3 , 7 , 9 ]. DEV have a lower risk for bleeding as they are found in the submucosa of the proximal esophagus which provides protection from gastric acid exposure [ 1 , 3 , 7 , 9 ]. Once DEV are diagnosed on EGD, it is important to evaluate for the underlying etiology.…”

“…To assist in ruling out portal hypertension as an underlying etiology, imaging of the portal vein should be undertaken, for example with vascular duplex ultrasonography [ 1 ]. To better evaluate for extrinsic compression and intrinsic disease of the SVC, the gold standard imaging test is direct venography [ 3 ]. CT angiography of the chest can also be used if there is a contrast injection protocol instituted to minimize flow artifacts [ 3 ].…”

“…To better evaluate for extrinsic compression and intrinsic disease of the SVC, the gold standard imaging test is direct venography [ 3 ]. CT angiography of the chest can also be used if there is a contrast injection protocol instituted to minimize flow artifacts [ 3 ].…”

“…DEV are caused by either extrinsic compression of the SVC or an intrinsic venous disease process in the SVC. Extrinsic compression can occur from a mass effect such as from surrounding malignancy and mediastinal fibrosis [ 3 , 4 ]. Intrinsic venous disease can occur from SVC thrombosis, complications with hemodialysis catheters, and Behcet's disease, for example [ 3 , 4 ].…”