Management of Superior Vena Cava Occlusion Causing Bleeding “Downhill” Esophageal Varices

Introduction

The incidence of central vein occlusion in hemodialysis patients is reported to be 10% to 40% and is a known complication of tunneled dialysis catheters. The health care system in Peru treats approximately 15,000 dialysis patients and 45% undergo treatment via tunneled catheters. The incidence of superior vena cava (SVC) stenosis in hemodialysis patients with catheters has been estimated to be 9.4% ¹ to 24%. ² Hemodialysis patients with catheter-induced SVC occlusion may be asymptomatic due to collateral venous circulation. However, in the presence of an arteriovenous fistula, venous hypertension develops, and SVC syndrome may occur. SVC occlusion may also affect adequate function of the dialysis access and may even cause loss of the access. Rarely, these patients can present with esophageal bleeding caused by “downhill” varices due to SVC occlusion. There are currently no established guidelines for the management of this condition. ³ We present the case of a patient on hemodialysis complicated by SVC occlusion and resultant esophageal varices who underwent treatment with SVC recanalization and stenting in a hospital in Lima, Peru.

Case Report

The patient is an 18-year-old woman with end-stage renal disease secondary to urological complications of myelomeningocele who had been on hemodialysis for 3 years. She initially received dialysis for a year through a left internal jugular central venous catheter and subsequently via a left upper arm brachiocephalic fistula.

She presented to Hospital Nacional Arzobispo Loayza, in Lima, Peru, with hematemesis and general weakness. Her vital signs were stable. She had edema of her face, neck, and left arm with visible venous collaterals in neck, chest, and proximal upper arm. Her left brachiocephalic fistula was pulsatile and dilated. She had hemoglobin of 10 mg/dL and normal liver function tests.

She underwent upper endoscopy notable for 3 columns of grade 3 varices in the mid to distal esophagus with multiple red patches and strips (Figure 1). Given evidence of active bleeding, the varices were banded. Ten days later, a repeat endoscopy was completed due to recurrent hematemesis. Given this persistent finding and no other apparent etiology for her varices, she was evaluated with upper extremity fistulography that revealed left brachiocephalic vein occlusion at the junction with the SVC draining into a large azygous vein (Figure 2). The right arm venogram was notable for right subclavian and brachiocephalic vein occlusion. SVC venogram from a femoral vein approach revealed occlusion of the SVC 1 cm above the right atrium (Figure 3).

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Figure 1.

Endoscopic images of bleeding esophageal varices with banding (arrow).

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Figure 2.

Occlusion of the left brachiocephalic vein (large arrow) with large azygous vein (small arrow).

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Figure 3.

Superior vena cavogram shows occlusion above the right atrium (larger arrow). Notice the sheath in the left brachiocephalic vein (small arrow).

Given her repeat episodes of bleeding, she was considered for an advanced procedure, either fistula ligation with femoral dialysis access placement or endovascular repair of the SVC occlusion. In an attempt to preserve her dialysis access, endovascular repair was chosen. The case was planned and completed in a hybrid endovascular suite at Hospital Nacional Dos de Mayo Hospital with monitored anesthesiology support and cardiothoracic surgeons on standby.

The planned procedure was intrathoracic vein sharp recanalization. ⁴ First, using the left arm arteriovenous fistula, a 7-Fr destination sheath (Destination Guiding Sheath, Terumo, Leuven, Belgium) was placed in the brachiocephalic next to the junction with the SVC. The next step was common femoral vein placement of an 8-F sheath (Swartz Braided SL Transseptal Guiding Introducer Sheath, St Jude Medical, St Paul, MN, USA) in the SVC just below the occlusion. A Brockenbrough (BRK) transseptal needle (St Jude Medical, St Paul, MN, USA) was placed in the sheath and advanced toward the brachiocephalic vein using the upper extremity 7-F destination sheath as a reference point. After successful puncture across the occlusion, an 0.018-inch wire was advanced into the brachiocephalic vein (Figure 4). The transseptal needle was removed and the wire was replaced with a 0.035-inch Amplatz Super Stiff guidewire (Boston Scientific, Marlborough, MA, USA). This was followed by angioplasty of the occluded tract and deployment of a 14 × 60-mm self-expanding Lifestar stent (Tempe, AZ, USA) from the SVC to the left brachiocephalic vein, posted with a 10-mm balloon.

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Figure 4.

Brockenbrough (BRK) needle inside the sheath and wire in the left jugular vein across the occlusion (arrow).

Postprocedure venogram revealed excellent flow from left brachiocephalic vein to the SVC and right atrium without filling of the azygous vein (Figure 5). No anticoagulation was used during the procedure and the patient remained on her preoperative medications. The patient was discharged home the following day after dialysis. Outpatient follow-up included 6-week and 12-week endoscopies notable for improved grade II esophageal varices without evidence of bleeding. The patient continued to be dialyzed using her left arm fistula without gastrointestinal bleeding, although she developed recurrent arm edema. Fistulography completed 9 months after the procedure revealed 90% stenosis of the SVC, which was then treated with angioplasty (Figure 6).

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Figure 5.

Venogram after stenting shows excellent flow into the right atrium.

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Figure 6.

Venogram post stent angioplasty after 9 months.

Discussion

We present a dialysis patient with a serious complication of SVC occlusion causing SVC syndrome, dysfunction of vascular access and bleeding downhill esophageal varices.

Although superior vena cava occlusion is often asymptomatic in dialysis patients, the presence of a functional upper extremity vascular access may trigger symptoms of SVC syndrome. Rarely, “downhill” esophageal varices may occur.

The term “downhill” varices was first coined in 1964 by Felson and Lessure ⁵ when they reported SVC occlusion secondary to fibrous mediastinitis in one patient and caused by metastatic lung cancer in 2 additional patients. The etiology of SVC obstruction causing bleeding downhill esophageal varices is related to a venous catheter in 27% of cases, and malignancy is responsible for 14%. Other benign etiologies include mediastinal fibrosis, Behcet’s, goiter, thrombosis, or postsurgical complications. ⁶

Pop and Cutler ⁷ were the first to describe bleeding downhill esophageal varices in a dialysis patient in 1996, a 52-year-old woman with SVC occlusion, SVC syndrome, and a right thigh arteriovenous graft. After failed attempts for endovascular therapy, she underwent right internal jugular vein to right atrial bypass successfully improving symptoms. ⁷

Esophageal veins involve a submucosal plexus and an adventitial plexus connected by perforating veins through the muscularis layer. Veins from the upper two-thirds of the esophagus drain via multiple veins (azygous, hemiazygos, vertebral, internal mammary, inferior thyroid, bronchia, and brachiocephalic) to the SVC and right atrium. The lower esophagus venous drainage is through the left gastric vein to the portal system. In SVC obstruction above the entrance of the azygous veins, blood from the head and neck will traverse the esophageal veins on its way to the azygous veins and SVC. Varices will be limited to the upper esophagus. If the obstruction is at or below the azygous vein, esophageal vein flow will be toward the portal system and inferior vena cava and varices may involve the entire thoracic esophagus.^5,8 Evaluation of computed tomography scans of patients with SVC obstruction have, however, revealed that most common drainage patterns were systemic-to-systemic collateral veins that drain into the inferior vena cava without traversing the portal system. ⁸

Downhill varices represent 0.4% to 10% of esophageal varices but less than 0.1% of patients present with hematemesis.^3,9 The most common etiology of bleeding downhill varices is a complication related to a venous catheter (27%). Although bleeding varices are rare, nonbleeding downhill varices have been reported to occur in 30% of patients with SVC obstruction undergoing screening contrast enhanced computed tomography. Most of these patients had end-stage renal disease.^6,10 Downhill varices bleed less than distal esophageal varices because proximal varices are submucosal as opposed to the more superficial epithelial venous plexus of distal esophageal varices. In addition, distal varices have more exposure to gastric acid and patients are commonly associated with coagulopathy of liver disease. Currently, there are no standard guidelines regarding the management of these varices. Traditional medical management such as octreotide and proton pump inhibitors are not effective, as varices are not affected by portal system pressures and are less influenced by gastric pH. Direct control of bleeding with varices has been reported with banding being preferred over sclerotherapy, although both techniques are associated with complications.^3,6

Diagnosis of downhill varices is made by upper endoscopy, which allows treatment if necessary. Etiology of SVC obstruction can be ascertained with computed tomography scan. magnetic resonance imaging/magnetic resonance venography can also diagnose varices and demonstrate reversal of flow in the azygous system. ¹¹ Diagnosis can also be made with venogram and in dialysis patients the best study may be a fistulogram. ¹²

The treatment of bleeding downhill varices with benign etiologies should be directed to alleviating the venous obstruction. Endovascular intervention reestablishes venous drainage through the SVC, achieving decompression of esophageal varices and preventing or controlling bleeding. In cases involving dialysis patients, such as in this case report, it also preserves function of upper extremity access.

Upper endoscopy with varices banding is effective as a temporary measure but definitive treatment of central vein occlusion is recommended to prevent recurrence of bleeding, preserve dialysis access function, and improve SVC syndrome symptoms. Patients who fail traditional endovascular recanalization techniques have limited alternatives such as upper arm access ligation and femoral catheters and grafts, choices that have increased risks of complications.^13,14 Morbidity associated with open chest atrial bypass procedures is higher than with endovascular procedures and has been reported at 19% in one study. ¹⁵ We elected to use a more aggressive method of recanalization using a sharp needle like a transseptal needle to cross the occlusion of the SVC allowing successful revascularization with angioplasty and stent placement. Cohen et al ⁴ have reported the use of this technique to treat chronic central vein occlusions successfully with pericardial bleeding complication rate of 5.1%. To minimize the risk of this complication, special attention should be made to maintaining the guiding sheath and transseptal needle in a central position toward the target, in this case, the brachiocephalic vein. Lateral projections and angiography should be obtained. Stenotic lesions or occlusions near the cavo-atrial junction should be considered of a higher risk because of its location within the intrapericardial portion of the SVC where wire and catheter manipulation may cause SVC or atrial injury.

After successful initial SVC endovascular reconstruction, adequate patency and long-term clinical success can be obtained with regular follow-up and secondary interventions. This patient returned for follow-up after 9 months and venography revealed stent stenosis, which was treated with balloon angioplasty to restore patency and to prevent future bleeding. She had not experienced recurrence of gastrointestinal bleeding.

Conclusion

Bleeding from “downhill” esophageal varices should be considered in the differential diagnosis of dialysis patients exposed to central venous catheters. This diagnosis requires a high degree of suspicion. In the absence of any evidence of liver disease, evaluation should be directed to rule out SVC obstruction. In this case, the diagnosis was initially established by upper endoscopy and evaluation with fistulography and central venography from femoral vein approach confirmed the diagnosis of SVC occlusion. Endovascular treatment should be considered, even with aggressive techniques such as sharp central vein recanalization. Successful endovascular SVC recanalization and stenting controlled esophageal bleeding and maintained arm vascular access function in this patient.