Downhill esophageal varices: a therapeutic dilemma

Abstract

Esophageal varices can cause life-threatening complications and are most often a sequela of liver disease. Although a rare cause of gastrointestinal bleeding, downhill variceal bleeding secondary to superior vena cava (SVC) obstruction should be considered in the differential diagnosis for patients with upper gastrointestinal hemorrhage. We discuss two such cases of downhill esophageal varices presenting with hematemesis in patients with end stage renal disease and no history of cirrhosis. These varices were thought to be secondary to SVC occlusion caused by complications from previous dialysis catheters. However, their difficult anatomy posed a significant challenge to the therapeutic interventions.

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.…”

“…When there is obstruction of the SVC, venous flow makes its way back to the right atrium via collateral pathways to the inferior vena cava. One collateral pathway it can take is through the azygos and hemiazygos veins that then flow into the esophageal venous plexus, thus creating DEV [ 1 , 2 ]. When the SVC obstruction is above the azygos vein, varices form in the upper third of the esophagus, whereas if obstruction is at the level of or below the azygos vein, varices form throughout the entire esophagus [ 1 ].…”

“…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 ].…”

“…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 ]. The case presented here highlights the rare presentation of hematemesis secondary to DEV in the setting of extrinsic SVC compression from metastatic renal cancer.…”

Color Doppler Ultrasound in Portal Hypertension

“…1,3,4 Esophagogastroduodenoscopy is considered the reference standard technique for the diagnosis of esophageal varices, allowing their direct visualization, assessment of severity, and risk stratification for rupture, and for its interventional capabilities. 5 Endoscopy, however, is an invasive procedure with possible complications, such as perforation. Therefore, it should be performed in a dedicated center, making it unsuitable for close patients' follow-up.…”