“Downhill”ʼ Esophageal Varices: Unusual Cause of Dysphagia

Abstract

Introduction: “Downhill” varices refer to proximal esophageal varices which typically result from superior vena cava (SVC) obstruction resulting reversal of blood flow in the upper esophageal venous plexus. The commonest etiologies of SVC obstruction include lung and thyroid carcinoma, mediastinal fibrosis and metastatic disease. Complication of central venous catheter is becoming a significant cause of SVC obstruction. In contrast to “uphill” varices associated with portal hypertension, downhill varices are usually asymptomatic with only few cases of bleeding reported. Case Description: A 48-year-old African American male with past medical history of AIDS on HAART, ESRD on hemodialysis (for 4 years), hypothyroidism and hypertension presented with progressively worsening dysphagia of 4 weeks duration. The dysphagia is mainly to solid food, which gets stuck at the base of the neck and behind his sternum. This has resulted in loss of appetite and 15 lb unintentional weight loss. He denies pain on swallowing, abdominal pain, nausea, vomiting, regurgitation or hematemesis. He also complained of left upper arm swelling. Physical exam was significant for diffuse left upper arm swelling and left arm arteriovenous graft (AVG). No oral lesions or thrash were seen. Laboratory studies are unremarkable except CD4 count of 131cells/mm3. Endoscopy (EGD) showed several columns of small-medium varicosities in proximal-mid esophagus with normal distal esophagus. No strictures, mass, adherent lesions were seen and biopsy result was unremarkable. The finding is consistent with “downhill” esophageal varices of the upper 1/3 of the esophagus, a condition commonly seen with SVC stenosis. Neck and chest and CT were unremarkable. Fistulogram and superior venacavogram showed patent AV- fistula but narrowed SVC. The SVC had stenotic segment measuring 9.9 mm in diameter above the point of entry of the azygous vein with extensive collateral formation. Angioplasty dilated the narrowing to 13 mm diameter which led to resolution of left arm swelling and dysphagia. Repeat EDG a week later revealed near complete resolution of the downhill varices. The patient continued to improve clinically during his hospital stay and started to eat without difficulty. Discussion: Although bleeding from downhill varices is well described, to our knowledge no case of downhill varices presenting with dysphagia is reported. Our patient's SVC stenosis was a complication of a prior indwelling hemodialysis catheter he had for two years before placement of AV fistula graft. The management is aimed at correcting the cause of the obstruction through angioplasty or stent placement as in our case.