Abstract
BackgroundProximal 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 presentationA 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.ConclusionDownhill 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.
Highlights
Proximal or ‘downhill’ esophageal varices are a rare cause of upper gastrointestinal hemorrhage
While malignancy is described as the most common underlying etiology of superior vena cava (SVC) obstruction (60 %), based on a review of the available literature, malignancy is the reported etiology for only 14 % of SVC obstruction resulting in downhill variceal bleeding (Table 1)
Other benign etiologies of SVC obstruction such as mediastinal fibrosis, behcet’s, goiter, thrombus or post-surgical complications account for the majority of the remaining reported cases of benign obstruction resulting in bleeding
Summary
Proximal or ‘downhill’ esophageal varices are a rare cause of upper gastrointestinal hemorrhage. 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. While malignancy is described as the most common underlying etiology of SVC obstruction (60 %), based on a review of the available literature, malignancy is the reported etiology for only 14 % of SVC obstruction resulting in downhill variceal bleeding (Table 1).
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