Variceal Hemorrhage as Complication of Vascular Access for Total Parenteral Nutrition

ABSTRACT

Downhill varices, a rare manifestation of venous hypertension, arise from superior vena cava (SVC) obstruction. We present a unique case of downhill varices secondary to SVC syndrome in a 69-year-old man on chronic total parenteral nutrition who presented with large volume melena and facial swelling. In this case, chronic central venous catheter use contributed to thrombotic occlusion of the SVC, leading to collateral vessel formation and variceal development. Management involves addressing the underlying cause of venous obstruction. This case highlights the importance of considering downhill varices as a complication in patients with chronic vascular access presenting with symptoms of SVC syndrome.

INTRODUCTION

Most esophageal varices are seen in the lower esophagus in the setting of portal hypertension. Although rare, accounting for only 0.5% of varices seen endoscopically, esophageal varices can also be seen in the upper to middle esophagus.¹ When these varices are seen, they are known as downhill varices and they present in approximately 30% of cases where there is an obstruction in the superior vena cava (SVC).² In this report, we will describe a case of facial swelling and upper gastrointestinal bleeding from downhill varices in the setting of SVC obstruction from chronic central venous access for total parenteral nutrition (TPN).

CASE REPORT

Patient is a 69-year-old man with a history of short bowel syndrome with prior small bowel resection due to small bowel lymphoma, now on chronic TPN for over 20 years, who initially presented to the hospital for large volume melena and facial swelling.

The patient was initially diagnosed with short bowel syndrome after large surgical resection secondary to small bowel lymphoma, leaving small portion of the jejunum anastomosed to 70 cm of colon. Since the initiation of TPN over 20 years ago, the patient has diligently maintained his central venous catheter (CVC) with daily saline runs and EtOH locks 3 times per week. CVC was exchanged 6 months before hospitalization due to line malfunction.

On hospital admission, hemoglobin had decreased from prior baseline of 12.8 to 6.3, and he received 3 red blood cell transfusions. He underwent esophagogastroduodenoscopy (EGD), which was remarkable for grade III esophageal varices in the middle and lower esophagus with stigmata of recent bleeding (Figure 1). Seven bands were placed (Figure 2), melena resolved, and hemoglobin stabilized to 9.7. Given endoscopic findings, in addition to lack of clinical and biochemical evidence of advanced liver fibrosis or cirrhosis, downhill varices secondary to SVC stenosis was suspected. The patient then underwent computed tomography of his chest, which was remarkable for high-grade stenosis or occlusion involving the central right subclavian vein, right brachiocephalic vein, and SVC, as well as possible age-indeterminate thrombosis and multiple thoracic venous collaterals. Interventional radiology ultimately performed venogram with evidence of right internal jugular vein occlusion, distal innominate vein occlusion, and no discernible flow to SVC (Figure 3). The patient underwent angioplasty, with successful dilation and revascularization of SVC, and his CVC was exchanged. After angioplasty, facial swelling resolved and he was then restarted on prior antithrombotic therapy with aspirin and enoxaparin sodium. One month after the initial banding, repeat EGD revealed 2 residual large esophageal varices seen in upper esophagus tracking down into mid esophagus without any stigmata of recent bleeding, which were subsequently banded. EGD performed 10 weeks after the variceal bleed showed near-complete resolution of varices without any additional episodes of bleeding.

Figure 1.

Downhill varices seen tracking from proximal-to-distal esophagus on endoscopy. Evident platelet plug noted on proximal varix, suggestive of recent variceal bleed.

Figure 2.

Varix after endoscopic banding.

Figure 3.

Digital subtraction venogram of right subclavian vein demonstrating central occlusion immediately adjacent to tunneled central line in right internal jugular vein. Arrows in both figures contrast draining in retrograde fashion through high external jugular vein.

DISCUSSION

With patent vasculature, blood from the upper two-thirds of the esophagus drains into the SVC by multiple tributaries, and the lower third of the esophagus drains into the left gastric vein to the portal vein. In SVC syndrome, obstruction within the SVC forces retrograde blood flow into the right atrium through collateral vessels, including drainage into the azygous/hemiazygous system.³ If the obstruction is above the level of the azygous vein, blood can collect in the upper esophagus, leading to downhill variceal formation. Often these downhill varices are seen in the setting of SVC syndrome secondary to thrombosis, severe pulmonary hypertension, thyroid or mediastinal masses, and/or catheter-related complications. In a systematic review of documented case reports describing downhill varices, 29 of 41 reported cases of downhill varices were seen in the setting of SVC syndrome.³ Most commonly, in the setting of hemodialysis catheter use for patients with end-stage renal disease, however, one reported case was associated with vascular access for chronic TPN use.

SVC syndrome is thought to occur in patient populations with chronic vascular access lines given risk for endothelial injury, central line stenosis, turbulent blood flow, and thrombosis.⁴ For chronic TPN patients, catheter-related thrombosis is a common cause of line occlusion that can predispose to the development of SVC syndrome. While a known and a severe thrombotic complication of TPN, SVC syndrome had an incidence of 0.7 episodes per year in patient with chronic CVC use for home TPN.⁵ According to the results of this retrospective study, patients at the highest risk for thrombotic events were those with catheter-related sepsis, infrequent line exchange (notably a line in place for more than 7 months), and patients with prior thrombotic events not on chronic anticoagulation. Most thrombotic disease associated with catheter use occurs around catheter site, rather than inside of the line. Ideal catheter placement requires catheter tip to terminate within SVC due to its relatively high rate of blood flow.⁶ Vessels with slower blood flow are predisposed to sclerosis from TPN solution, leading to higher risk of thrombosis. Occlusion can also occur secondary to nonthrombotic etiologies, such as administration of noncompatible medications through central line, high calcium or phosphate content in TPN solution, or lipid deposition from lipid emulsion administered with TPN. Catheters should be routinely flushed and not used for any other medication administration to avoid line occlusion.⁶ In the current literature, there is not sufficient evidence to determine benefit for surveillance imaging to prevent recurrent episodes. While some observational studies may indicate benefit to routine surveillance imaging, a prior randomized control trial from Ram et al investigated benefit for regular surveillance in comparison with clinical monitoring in patients with chronic vascular access. Patients who underwent routine surveillance with ultrasound every 3 months and pre-emptive angioplasty for stenosis >50% were found to have lower rates of thrombosis. However, there was no statistical difference in overall survival or graft survival in comparison with patients who were clinically monitored.⁷

In contrast to lower esophageal varices seen with cirrhosis, bleeding is a rare presenting symptom for patients with downhill varices. Approximately only 7.6% of downhill varices are complicated by hemorrhage.⁸ It is suspected that bleeding is less common in the upper esophagus because the submucosa is less susceptible to bleeding in comparison with the superficial subepithelium of the distal esophagus, in addition to decreased gastric acid exposure in the proximal esophagus.⁹ When bleeding does occur, treatment involves endoscopic band ligation, sclerotherapy, and balloon tamponade to control bleeding. Variceal band ligation is preferred over sclerotherapy, due to higher rates of complications associated with sclerotherapy including spinal cord paralysis and pulmonary embolism.⁴ The primary goal of treatment for patients with downhill varices in the setting of SVC syndrome was to relieve underlying obstruction. In the setting of SVC thrombosis, mechanical or chemical thrombolysis, venoplasty, and stenting are all viable treatment options.¹⁰ In addition, those who develop SVC syndrome from chronic catheter use should have catheter site examined for accurate positioning and/or have line exchanged. It is reasonable to consider periodic endoscopic surveillance to screen for and eradicate downhill varices after an episode of SVC syndrome with catheter-related thrombosis or variceal bleeding, though evidence is currently limited.¹¹ Given that the risks and benefits are not yet delineated, high quality research is needed to better characterize this practice.

Although rare, chronic vascular access from TPN can result in SVC obstruction with development of downhill varices. In a patient with melena or hematemesis, and risk factors for the development of SVC syndrome, downhill varices should be considered as potential etiology of bleeding. Furthermore, the cause for underlying obstruction should be investigated and appropriately treated.

DISCLOSURES

Author contributions: C. Chiodi is the primary author who performed literature review on topic, in addition to drafting case report. D. Liu assisted in editing case report. E. Omer was the primary attending physician who managed this patient while in hospital, including performing procedural intervention as well as obtaining patient consent for publication of this case. C. Chiodi is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.