Bleeding 'downhill' esophageal varices associated with benign superior vena cava obstruction: case report and literature review

Abstract

Background: Proximal or 'downhill' esophageal varices are a rare cause of upper gastrointestinal hemorrhage. Unlike the much more common distal esophageal varices, which are most commonly a result of portal hypertension, downhill esophageal varices result from vascular obstruction of the superior vena cava (SVC). While SVC obstruction is most commonly secondary to malignant causes, our review of the literature suggests that benign causes of SVC obstruction are the most common cause actual bleeding from downhill varices. Given the alternative pathophysiology of downhill varices, they require a unique approach to management. Variceal band ligation may be used to temporize acute variceal bleeding, and should be applied on the proximal end of the varix. Relief of the underlying SVC obstruction is the cornerstone of definitive treatment of downhill varices.

Case presentation: A young woman with a benign superior vena cava stenosis due to a tunneled internal jugular vein dialysis catheter presented with hematemesis and melena. Urgent upper endoscopy revealed multiple 'downhill' esophageal varices with stigmata of recent hemorrhage. As there was no active bleeding, no endoscopic intervention was performed. CT angiography demonstrated stenosis of the SVC surrounding the distal tip of her indwelling hemodialysis catheter. The patient underwent balloon angioplasty of the stenotic SVC segment with resolution of her bleeding and clinical stabilization.

Conclusion: Downhill esophageal varices are a distinct entity from the more common distal esophageal varices. Endoscopic therapies have a role in temporizing active variceal bleeding, but relief of the underlying SVC obstruction is the cornerstone of treatment and should be pursued as rapidly as possible. It is unknown why benign, as opposed to malignant, causes of SVC obstruction result in bleeding from downhill varices at such a high rate, despite being a less common etiology of SVC obstruction.

Keywords: Bleeding varices; Case report; Esophagus; Proximal esophageal varices; Superior vena cava; Vascular obstruction.

Fig. 1

a Esophagogastroduodenoscopy in a patient with superior vena cava obstruction demonstrating varices in the proximal esophagus (white arrows), with overlying red wales (black arrowhead) and a fibrin plug (‘nipple sign’) (white arrowhead), indicating recent hemorrhage. b Venography of the superior vena cava showing a tunneled dialysis catheter (white arrow) with an adjacent superior vena cava stenosis (black arrow)