Downhill Esophageal Varices Caused by Catheter-Related Thrombosis

3 55-year-old woman with end-stage renal disease on hemodialysis presented with hematemesis and melena after a routine dialysis session. wo months prior she had been diagnosed with a non–catheter-related left pper-extremity deep vein thrombosis and was placed on anticoagulation with arfarin. Her past medical history was significant for human immunodefiiency virus (last CD4 cell count, 168/mm3) and ischemic cardiomyopathy ejection fraction, 40%). On examination, she was hemodynamically unstable. he had a swollen face with puffy eyes, bulging neck veins, and an edematous ight breast and right arm that had developed over the previous 3 months. A unneled right internal jugular central venous catheter was in place since 5 onths prior. Laboratory testing revealed a hemoglobin level of 7.4 g/dL, ecreased from 12.5 g/dL, and an international normalized ratio of 2.29. An esophagogastroduodenoscopy showed grade III varices in the proximal sophagus with an overlying protuberant blood clot (Figure A). The rest of the sophagus was normal. Endoscopic variceal ligation was performed with placeent of 3 bands, resulting in successful hemostasis and decompression of the arices. Magnetic resonance angiography and venography revealed an abrupt ut off in the superior vena cava (SVC) with an intraluminal obstructing hrombus, likely related to her central venous catheter (Figure B). Radiographic vidence of numerous breast and abdominal collaterals suggested a chronic bstruction. The patient underwent angiography, which confirmed the abence of blood flow beyond the thrombus. Balloon dilation with stent placeent was performed with successful recannulization of the SVC, shown by eturn of physiologic blood flow from the SVC to the right atrium on angioram (Figure C). Her internal jugular central venous catheter was removed. ecovery was uneventful and the patient was discharged home off anticoaglation. Downhill varices form in the proximal esophagus and provide an alternate oute for blood flow when systemic flow is obstructed. Obstruction of the SVC bove the level of the azygous vein causes blood to return toward the heart via he azygous vein and mediastinal collaterals, resulting in the formation of arices in the upper third of the esophagus. This leads to the downward irection of blood flow along the esophagus, hence the name downhill varices. n contrast, obstruction of the SVC below the level of the azygous vein causes lood to return to the heart via the inferior vena cava through the hemiazygos, sophageal, and portal veins and results in formation of varices along the ntire length of the esophagus. Downhill varices are less common than uphill istal esophageal varices caused by portal hypertension. Downhill varices rarely leed and it is estimated that 7.6% are associated with hemorrhage.1,2 Downhill varices are associated most often with upper-body venous obtruction from a variety of causes including primary and metastatic mediastial tumors, mediastinal fibrosis, substernal goiters, venulitis, indwelling cathters, muscular constriction of the hypopharyngeal veins, and, rarely, astleman disease.3 Diagnosis should be suspected when patients present with pper gastrointestinal bleeding and symptoms of SVC syndrome, including yspnea, facial swelling, and head fullness. Management of downhill varices hould be targeted at obtaining hemostasis through endoscopic therapy and reatment of the underlying vascular occlusion. At the 3-month follow-up evaluation this patient had significant improveent in her facial swelling and breast and arm edema, and no further bleeding fter removal of her central venous catheter and recannulization of her SVC.