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| Year : 2003 | Volume
: 14
| Issue : 2 | Page : 186-189 |
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| The Superior Vena Cava Syndrome: Late Presentation after Hemodialysis Catheter Removal |
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Nabieh Al-Hilali1, Mangalathillam RN Nampoory1, Vadakethu T Ninan1, Fawzia MY Hussein2, Jaber H Ali1, Mahmoud Samhan3, Kaivilayil V Johny4
1 Department of Medicine, Mubarak AI-Kabeer Hospital, Kuwait
2 Department of Radiology, Mubarak AI-Kabeer Hospital, Kuwait
3 Hamed Al Essa Organ Transplant Center, Kuwait
4 Department of Medicine, Faculty of Medicine, Kuwait University, Kuwait
Click here for correspondence address and email
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 Abstract | | |
The superior vena cava (SVC) syndrome is due to obstruction of the SVC and may present by dyspnea, chest pain, cough, headache, dysphasia, and symptoms of increased intracranial pressure; however, the affected patients can be asymptomatic. Numerous collateral veins are often seen on the upper chest, arms and neck. The syndrome may be caused by prolonged use of indwelling catheters, but is an infrequently reported complication in the hemodialysis patients. We report two patients who developed SVC syndrome several months after removal of hemodialysis indwelling catheters. The causes of this syndrome in our patients were stenosis in one patient and thrombosis in the other; venous endothelial injury and subnormal levels of protein C and S were possible contributory factors. These cases illustrate that SVC syndrome is a possible late complication after removal of hemodialysis indwelling catheters. Keywords: Superior vena cava syndrome, Hemodialysis catheter, Late presentation.
How to cite this article:
Al-Hilali N, Nampoory MR, Ninan VT, Hussein FM, Ali JH, Samhan M, Johny KV. The Superior Vena Cava Syndrome: Late Presentation after Hemodialysis Catheter Removal. Saudi J Kidney Dis Transpl 2003;14:186-9 |
How to cite this URL:
Al-Hilali N, Nampoory MR, Ninan VT, Hussein FM, Ali JH, Samhan M, Johny KV. The Superior Vena Cava Syndrome: Late Presentation after Hemodialysis Catheter Removal. Saudi J Kidney Dis Transpl [serial online] 2003 [cited 2019 Dec 13];14:186-9. Available from: http://www.sjkdt.org/text.asp?2003/14/2/186/33027 |
| Introduction | |  |
Double lumen subclavian and internal jugular dialysis catheters are commonly used as immediate vascular access for patients requiring urgent hemodialysis. Thrombosis and stenosis are frequent complications, particularly in the cannulation of subclavian vein. [1],[2],[3] However, superior vena cava (SVC) syndrome due to stenosis or thrombosis caused by hemodialysis indwelling catheters is infrequently reported in these patients. [4],[5],[6],[7]
We describe in this report two patients who presented with SVC syndrome related to long-term use of hemodialysis indwelling catheters. Notably, these patients developed features of SVC syndrome several months after catheter removal.
| Case Report | | |
Case 1:
A 43-years-old woman presented in 1995 with advanced chronic renal failure due to chronic tubulointerstitial disease. She had several attempts for permanent vascular access including a left radiocephalic arteriovenous fistula a left straight brachio-axillary polytetrafluoroethylene graft (PTFE), and right internal radiocephalic arteriovenous fistula which all failed. In the meanwhile, the patient was maintained on hemodialysis using a right subclavian dual lumen polyurethane catheter as a vascular access for 87 days followed by a right internal jugular dual lumen silicone rubber permanent catheter for 233 days. At last, a successful brachiocephalic arteriovenous fistula was created on the right arm. After 213 days from last catheter removal, the patient developed puffy face, facial flushing, and multiple tortuous dilated veins on the anterior chest wall and upper abdomen.
The magnetic resonance angiography revealed severe stenosis involving the proximal SVC [Figure 1]. The inferior vena cava was patent. Studies for hypercoagulable state showed antithrombin III: 78% (normal 70-120%), Protein C activity: 55% (normal 60-150%), Protein S activity: 88% (normal 55-130%) and lupus anticoagulant: negative. The patient was maintained on systemic anticoagulation with warfarin. At present she continues to receive hemodialysis using the right arm brachiocephalic arteriovenous fistula.
Case 2:
A 41-year-old woman with end-stage renal failure, due to chronic glomerulonephritis, was started on hemodialysis in 1998.. The patient had a left arm radiocephalic arteriovenous fistula followed by a left arm straight radiocephalic PTFE graft but both failed. In the meanwhile she was maintained on hemodialysis using a right subclavian dual lumen polyurethane catheter as a vascular access for 45 days followed by a right internal jugular permanent dual lumen silicone rubber catheter for 36 days. A second successful left straight brachioaxillary PTFE graft was inserted. After 275 days of the permanent catheter removal, the patient presented with puffiness of the face, fascial flushing, sense of suffocation, swelling around the neck and upper chest wall, the swelling was more remarkable on the predialysis day.
Doppler study of the internal jugular vein did not reveal evidence of thromboses or stenosis. The magnetic resonance angiography showed occlusion of the SVC and right subclavian vein. Numerous collateral veins in the upper limbs and chest were noted. The left subclavian and left innominate veins were outlined [Figure 2]. Hypercoagulability study showed anti-thrombin III: 88% (normal 70-120%), Protein C activity: 25% (normal 60-150), Protein S activity: 45% (normal 55-130%) and lupus anticoagulant: negative. The patient was maintained on systemic anticoagulation with warfarin. Presently, she continues to receive hemodialysis using the left brachioaxillary graft.
| Discussion | | |
The SVC syndrome is caused by either significant narrowing or occlusion of the SVC. Vascular damage contributes to the genesis of venous thrombosis through either direct trauma [8],[9] or activation of endothelial cells by cytokines released as a result of tissue injury and inflammation. [10],[11]
Thrombi in the SVC were detected by transesophageal echocardiography in 30% of patients who had single lumen silicone rubber hemodialysis catheters. [12] Our patients received right-sided subclavian catheters made of polyurethane and right internal jugular vein catheters made of silicone. Polyvinyl chloride, polyethylene and teflon catheters are associated with increased thrombogenisity as compared to silicone rubber. [7],[13] Furthermore, the SVC stenosis may be induced by persistent trauma of the endothelium by the catheter tip and the higher blood flow during dialysis hours.
In our patients, the SVC stenosis (Case 1) and thrombosis (Case 2) occurred in association with the subnormal level of protein S and C, which are the possible contributing factor in the development of the syndrome. The fact that both patients had multiple peripheral access failures due to thrombosis suggests the presence of a hypercoagulale state. The late presentation was probably due to the gradual progression of the obstruction despite the initial compensation by the development of numerous collaterals.
Clinically, the common symptoms of the SVC syndrome include dyspnea, chest pain, cough, headache, dysphagia, and symptoms of increased intracranial pressure; however, the affected patients can be asymptomatic. [14],[15]
Inadequate blood flow rate during dialysis is often present in these patients due to obstruction of the catheter tip by either the caval vein thrombus sucking the displaced catheter tip against the vessel wall or the thrombotic occlusion of the lumen of the catheter. [16],[17],[18],[19]
In chronic obstruction of the SVC, collateral routes are necessary for venous return to the heart. [20] The veins of the upper extreemities are distended in 60 to 70% of the patients with SVC syndrome. The swelling of the upper extremity and face is found in 40 to 45%, whereas cyanosis is less common; found only in 15% of the patients. [18]
In summary, the SVC syndrome may present late after the removal of the subclavian or internal jugular catheters used for hemodialysis. Stenosis and or thrombosis of the SVC should be suspected in patients with long-term catheterization of the subclavian or jugular veins; hypercoagulability may increase the risk of the stenosis and/or thrombosis. Once the process is initiated, the removal of the catheter may not prevent the occurrence of thrombosis or stenosis.
| References | | |
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Correspondence Address:
Mangalathillam RN Nampoory
P.O. Box 1427, Hawally 32015
Kuwait
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PMID: 18209444 
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