Bleeding ‘downhill’ esophageal varices associated with benign superior vena cava obstruction: case report and literature review

‘Downhill’ esophageal varices are an uncommon etiology of gastrointestinal bleeding, estimated to account for approximately 0.1 % of all cases of variceal hemorrhage [1, 2]. The most common reported cause of SVC compression is from mediastinal malignancy such as thymoma, lymphoma or lung cancer, accounting for approximately 60 % of cases [3]. Although bleeding ‘downhill’ varices are rare, non-bleeding varices have been reported to occur in 30 % of patients with benign or malignant SVC obstruction undergoing screening upper endoscopy [1]. SVC obstruction diverts venous return from the head and upper torso through collaterals such as the azygous or innominate veins to bypass the obstruction. The proximal and mid esophageal veins drain into the azygous and innominate veins, and the increased pressure and collateralization result in the development of esophageal varices supplied from the superior aspect of the esophagus and extending distally [4].

The treatment of bleeding ‘downhill’ esophageal varices involves a multidisciplinary team including thoracic or vascular surgery, interventional radiology, and the endoscopist. When possible, correction of the underlying cause of SVC obstruction is the cornerstone of management, and may involve the angiographic dilation of the narrowed SVC segment, surgical reconstruction or resection of the involved SVC, or cancer therapies such as chemotherapy or external beam radiation [6, 7]. Endoscopic therapy with variceal band ligation or sclerotherapy (at the proximal end of the varix from which blood flow is supplied) or balloon tamponade can be attempted when bleeding is severe to temporize bleeding prior to definitive therapy. Endoscopic approaches are technically limited by the proximity of the varices to the larynx and may be painful due to the somatic innervation of the proximal esophagus.

In this paper we report the 10^th case of bleeding downhill varices secondary to complications from a central venous catheter, confirming this as the most commonly reported underlying etiology of bleeding downhill varices. It remains uncertain why benign, as opposed to malignant, causes of SVC obstruction result in bleeding from downhill varices at such a high rate.

A 22 year-old woman presented with acute hematemesis, tachycardia, and hypotension after 3 days of melenic stools. Her only medical history was end-stage kidney disease due to Henoch-Schönlein purpura, and she underwent chronic hemodialysis using a tunneled right internal jugular venous catheter due to prior complications with her right arm fistula. Her current hemodialysis catheter had been in place for approximately 14 months. She had no history of prior liver disease or gastrointestinal bleeding and denied NSAID use. Physical exam was notable for facial edema and erythema (plethora), abdominal and chest wall varices, and tachycardia, without stigmata of chronic liver disease (ascites, splenomegaly, palmar erythema, or spider telangiectasias). Laboratory evaluation revealed an acute anemia (hemoglobin 4.65 mmol/L) with normal platelets, liver function, and coagulation studies. Upper endoscopy was urgently pursued and revealed three columns of large varices in the proximal esophagus with stigmata of recent hemorrhage (Fig. 1a) and a normal distal esophagus, stomach, and duodenum. CT angiogram showed a stenosis in the superior vena cava adjacent to the distal aspect of her hemodialysis catheter with a dilated azygous vein bypassing the stenotic SVC segment to supply the proximal esophageal varices in a retrograde direction.

The patient experienced a second episode of hematemesis and urgently underwent balloon dilation of the stenotic SVC segment under angiography (Fig. 1b). She had no further episodes of gastrointestinal bleeding and her vital signs normalized immediately after the procedure. She was discharged shortly thereafter and underwent a repeat balloon dilation of the stenotic SVC segment 1 week after discharge. She did not experience recurrent gastrointestinal hemorrhage over a 12-month follow up period after her hospitalization.

Providers should be vigilant for bleeding “downhill” varices in patients with upper gastrointestinal bleeding and clinical evidence of SVC obstruction because the pathophysiology of this disorder mandates a unique management compared to esophageal varices occurring in the usual setting of portal hypertension and cirrhosis. Though data is lacking, traditional medical management would be unlikely to be of benefit in this population. Octreotide, as a splanchnic dilator would not decrease the pressure in “downhill” varices as they do not communicate directly with the portal system. Proton pump inhibitors would be unlikely to play a role as the upper esophagus is less likely to be influenced by gastric pH. The current literature only provides guidance for therapy by means of case reports and while firm recommendations cannot be made as to ideal therapy in this patient population, several methods of temporization seem to have been successful in halting bleeding until definitive decompression of the affected vessels can be performed. Further investigation is required to determine 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 in the general population.