A 75-year-old woman with hypertension, dyslipidemia, thalassemia trait, osteoarthritis of the knees, and hyperthyroidism presented with bloating, abdominal discomfort, and change in stool caliber underwent an esophagogastroduodenoscopy. The esophagogastroduodenoscopy revealed varices at the middle of esophagus, which were diagnosed to be downhill esophageal varices (DEVs). DEVs occur mainly from the superior vena cava (SVC) obstruction; however, in the absence of obstruction, angiogenesis and thrombosis could be the etiology. In our patient, computed tomography showed a pulmonary nodule located at periphery of the right upper lung without SVC contact nor obstruction. The nodule was later proven to be an adenocarcinoma. Thus, our patient showed the possibility that adenocarcinoma of the lung might associated with the DEV through a mechanism other than SVC obstruction. Recognition and differentiation of DEV from other benign venous blebs of the esophagus are important, and once detected, further investigation with computed tomography of the chest is recommended.

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.

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.

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.

Behçet's disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD.

Tunneled hemodialysis catheters are well-documented causes of benign central vein stenosis, which can be associated with proximal or downhill esophageal varices due to shunting of blood flow from the upper portion of the body through the esophageal venous plexuses. A majority of these cases remain asymptomatic. As a result, studies are largely limited to symptomatic patients, with incidence rates ranging from 16% to 29%. Recently, Hemodialysis Reliable Outflow (HeRO) graft has been introduced as an effective alternate hemodialysis access in catheter-dependent patients, especially in the presence of significant central venous occlusion. It differs from a conventional arteriovenous graft (AVG) by the fact that its venous outflow end is in the right atrium via one of the central veins, bypassing any significant occlusion upstream. Lower intervention rates and reduced incidence of bacteremia make it comparable to conventional tunneled catheters. However, the incidence of central vein occlusion and associated complications with HeRO grafts is unknown. We present the first case of gastrointestinal bleeding from downhill esophageal varices secondary to HeRO-graft-related SVC occlusion.

Proximal or "downhill" esophageal varices are a rare complication of superior vena caval (SVC) obstruction. Few reports describe downhill varices in dialysis patients with catheter-related SVC occlusion.

We studied a case of downhill esophageal varices in a dialysis patient from our center and reviewed the published literature on presentation, evaluation and treatment in other dialysis patients (MEDLINE database search).

Including our current case, we identified eight reports of dialysis patients with downhill varices. All cases were recognized after presentation with gastrointestinal bleeding, in contrast to low reported bleeding rates of downhill varices in non-dialysis patients. Localized edema and superficial venous engorgement (signs of SVC occlusion) were each observed in four of eight patients. The duration of hemodialysis dependence ranged from 2.5 to 23 years, and dialysis access history included multiple central venous catheters when described (seven cases). Central venous imaging by direct, magnetic resonance or computerized tomographic venography documented SVC stenosis in all cases. Management included percutaneous transluminal angioplasty of the SVC with or without stenting in five of eight patients, three of whom developed restenosis during observation. Successful surgical venous bypass was performed in one patient after failed percutaneous venoplasty. Varices were treated with band ligation in four of eight cases without reported complications.

Although rare, downhill esophageal varices should be considered in the differential diagnosis of upper gastrointestinal hemorrhage in dialysis patients exposed to central venous catheters. Diagnosis should prompt radiographic evaluation of SVC patency. Treatment requires timely and coordinated care by specialists in endovascular interventions and gastrointestinal endoscopy.