A Rare Case of Superior Vena Cava Syndrome caused by long-term indwelling Hemodialysis Catheter Placement

Title: A Rare Case of Superior Vena Cava Syndrome caused by long-term indwelling Hemodialysis Catheter Placement First Authors: Dr. Som Aftabizadeh, DO, PGY-1 Co-authors: Dr. Thao Nguyen, DO, PGY-2; Dr. Aman Patel, DO, PGY-3; Dr. Prashanth Reddy, MD, PGY-1 Attendings: Dr. Ikponmwosa Iyamu, MD; Dr. Machaiah Madhrira, MD; Dr. Balamurugan Sankarapandian, MD Name of Institution- Medical City Fort Worth Name of institutional department, division or other administrative unit- Internal Medicine City, State, Zip Code: Fort worth, Texas, 76104 Background/Abstract: End-stage renal disease (ESRD) cases continue to rise at approximately 21,000 cases per year in the US.1 During the past decade there has been a trending increase in use of central venous catheters (CVC) for hemodialysis (HD).2-3 When inserted into the superior vena cava (SVC), these catheters have been shown to be associated with thrombosis. While malignancy is the most common cause of superior vena cava syndrome (SVCS), an increasing incidence of benign causes are appearing. A portion of these benign causes are associated with the increasing use of intravascular catheters.2,4 Case Report: A 51-year-old african american female with past medical history of ESRD on HD, type II diabetes mellitus, coronary artery disease, paroxysmal atrial fibrillation who presented to our emergency department with acute neck swelling associated with hoarseness and headache. She states she woke up on the morning of admission with diffuse bilateral neck swelling that increased in size throughout the day. She denied difficulty with swallowing solids or liquids. She also denied difficulty with respiratory effort, chest pain, or shortness of breath. Her Vitals were stable. Clinical exam showed minimal facial edema but prominent superficial veins of the chest wall and neck region. Chest x-ray showed widening of the mediastinum. Our initial clinical impression was cellulitis, angioedema, or allergic reaction. Chest CT showed a thrombus partially occluding the SVC with severe stenosis at the cavoatrial junction. The patient was diagnosed with SVCS and underwent recanalization with angioplasty and stenting of SVC. The symptoms of superior vena cava syndrome began to improve immediately after the angioplasty and she continued to be free of symptoms and was subsequently discharged home. Of note, the patient had a long history of CVC access with repeated and prolonged placement after failed AVGs. Discussion/Conclusion: SVC syndrome is a medical emergency and can be fatal if resulting in severe cerebral edema and cerebellar herniation. A slower development of SVC is often better tolerated as many patients develop collateralization of vessels.4 It is thought that approximately half of central vein stenosis remain asymptomatic with clinical manifestations presenting due to eventual local upstream hypertension. In addition to risk of thrombosis of all CVCs, hemodialysis related CVCs are believed to have increased risk of thrombosis due to having longer, thicker lumens and longer length of placement.5-6 Therapy is generally endovascular or surgical with the former fairing better in hemodialysis patients with multiple comorbidities.7 This case illustrates the unusual manner in which SVC can present along with the potential for iatrogenic causes of SVC syndrome. Given the increasing frequency of intravascular catheter placement, it’s important to identify iatrogenic causes. Avoiding long term use of hemodialysis catheters and timely creation of AVFs may help prevent these complications. References:
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Quaretti P, Galli F, Moramarco LP, et al. Dialysis catheter-related superior vena cava syndrome with patent vena cava: long term efficacy of unilateral Viatorr stent-graft avoiding catheter manipulation. Korean J Radiol. 2014;15(3):364-9.
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