A Case of Downhill Esophageal Varices in a Patient With Superior Vena Cava Obstruction

Abstract

Downhill varices are a rare cause of acute upper gastrointestinal bleed and are generally a result of superior vena cava (SVC) obstruction. Here, we present a case of a young man who presented multiple times with bleeding esophageal varices. A 56 year old man with hypertension, diabetes mellitus, rheumatoid arthritis, end stage renal disease on intermittent hemodialysis (HD), had first presented to our hospital in 2015 with complaint of hematemesis. An endoscopy done at that time showed esophageal varices for which he underwent band ligation. However, in subsequent months, he had several admissions to hospital for difficult HD catheter access due to thrombosis of AV graft and deep venous thrombosis around dialysis catheter. A revision of AV-Graft was planned; however a CT angiogram showed an occlusive SVC thrombus extending into right brachio-cephalic vein with an extensive collateral circulation involving internal mammary vessels, azygous vein and varices around thoracic and abdominal viscera. Over the next two years, patient had multiple episodes of hematemesis. Patient presented to our hospital once again for chronic infected venous stasis ulcer. He was treated with multiple debridements and ultimately required BKA. Repeat imaging showed persistent occlusive SVC thrombosis. A collateral pathway involving azygous veins and dilated esophageal veins was identified with the esophageal varices forming part of this system. His hospital course was complicated by two episodes of hematemesis requiring endoscopic band ligation. Patient was evaluated by surgery for possibility of thrombectomy of SVC to decompress the collateral veins and prevent further episodes of hematemesis; however he was deemed to be at high surgical risk for the procedure. Patient was discharged on oral anticoagulation. The etiology of the extensive thrombus remains obscure after extensive hematological workup. SVC obstruction can occur secondary to malignancies, rheumatic heart disease, central venous catheter etc. SVC obstruction diverts venous return through collaterals such as azygous and innominate veins, and the increased pressure and collateralization results in development of esophageal varices. Therapy involves achieving hemostasis endoscopically and treating the underlying cause. High index of suspicion and investigation of alternative causes of varices when pertinent is prudent; as prompt diagnosis and appropriate treatment can significantly improve the long term outcome.