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

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.

Utility of Transabdominal Ultrasonography Enhanced by Oral Cellulose-Based Contrast Agent in Depicting Varices at Cardia and Fundus

“…Of note, the TUS-OCCA technique implemented herein was not applied to the full range of esophageal varices. This would include proximal or so-called "downhill" esophageal varices, caused by superior vena cava obstruction, and distal or "uphill" esophageal varices, caused largely by portal hypertension (Loudin et al 2016). Patients with cirrhosis may develop esophageal, gastroesophageal and isolated gastric varices; and varices of the esophagus typically manifest "uphill" drainage of the left gastric vein, which merges at the cardia with a branch of the esophageal vein (Gaba et al 2015).…”

Are Downhill Varices an Overlooked Entity of Upper Gastrointestinal Bleedings?

“…Regarding the size of the varices, there was not found any difference between the patients' groups with and without superior vena cava syndrome. There are publications relating to a downsizing of the varices after interventional procedures to a stenosis of the vena cava superior in patients with superior vena cava syndrome [ 4 , 6 – 8 ]. The bleeding incidences showed up in literature are generally mild and exceptionally life threatening.…”

“…Because of lacking gastrointestinal bleeding among the screened cases, accordingly, there were not any therapeutical interventions done. The necessary interventions to solve the superior vena cava obstruction were performed by the concerned departments (interventional radiology, cardiology, vascular, and thorax surgery); for example, there was performed a stent implantation into the superior vena cava [ 4 , 6 ]. In cases of primer downhill varices without upper gastrointestinal bleeding, treatment is not necessary.…”

“…Therapeutic means are the banding therapy as a safe and effective option and sclerotherapy [ 8 ]. Other specific therapies targeting the cause of obstruction of the superior vena cava are thyroidectomy, chemoradiotherapy, and surgical resection [ 6 – 12 ].…”

Hematemesis, a Rare Presentation for Downhill Esophageal Varices

“…Less than 0.1% of patients present with hematemesis [ 5 , 6 ]. Those who have a long-term venous catheter complicated by SVC narrowing, due to endothelial injury or thrombosis, make up the population with the highest DEV bleeding risk (around 27%) [ 1 , 7 ]. Only 14% of DEV cases secondary to malignancy are complicated by bleeding [ 1 , 7 ].…”