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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2003  |  Volume : 14  |  Issue : 2  |  Page : 186-189
The Superior Vena Cava Syndrome: Late Presentation after Hemodialysis Catheter Removal

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

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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 2021 Apr 16];14:186-9. Available from: https://www.sjkdt.org/text.asp?2003/14/2/186/33027

   Introduction Top

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 Top

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 Top

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 Top

1.Schwab SJ, Quarles D, Middleton J. Hemodialysis associated subclavian vein stenosis. Kidney Int 1988;33:l156-9.  Back to cited text no. 1    
2.Vanholder R, Lameire N, Verbanck J, Van Rattinghe R, Kunnen M, Ringoir S. complications of subclavian catheter hemodialysis: a 5-year prospective study in 257 conse-cutive patients. Int J Artif Organs 1982;5:297-303.  Back to cited text no. 2  [PUBMED]  
3.Aujla N, McCauley J, Sorkin M. Superior vena cava syndrome due to subclavian hemodialysis catheters. Mil Med 1990;155:274-7.  Back to cited text no. 3  [PUBMED]  
4.Banalagay E, Perlmutter J, Palevsky P. Central venous hemodialysis catheter related superior vena cava syndrome. Clin Nephrol 1993;40:59-60.  Back to cited text no. 4  [PUBMED]  
5.Seelig MH, Oldenburg WA, Klingler PJ, Odell JA. Superior vena cava syndrome caused by chronic hemodialysis catheters: autologous reconstruction with a pericardial tube graft. J Vasc Surg 1998;28:556-60.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Haqqie SS, Roth M, Low CL, Bailie GR. Superior vena cava syndrome from central venous hemodialysis. Clin Nephrol 1994;42:341-2.  Back to cited text no. 6  [PUBMED]  
7.Khanna S, Sniderman K, Simons M, Beseley M, Uldall R. Superior vena cava stenosis associated with hemodialysis catheters. Am J Kidney Dis 1993;21:278-81.  Back to cited text no. 7    
8.Stamatakis JD, Kakkar VV, Sagar S, Lawrence D, Nairn D, Bentley PG. Femoral vein thrombosis and total hip replacement. Br Med J 1977;2:223-5.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Davies GS, Salzman EW. Venous and arterial thrombosis: pathogenesis, dia­gnosis, prevention and therapy. The pathogenesis of deep vein thrombosis. In: Joist HJ, Sherman LA, eds. New York, NY. Grune & Stratton 1979:1-22.  Back to cited text no. 9    
10.Oschos CB, Khan MI, Regan TJ. Thrombogenic properties of blood during early ischemic and nonischemic injury. Am J Physiol 1971;220:1882-4.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Bjorklid E, Giercksky KE, Prydz H. An immunoradiometric assay for factor III (tissuethromboplastin). Br J Haematol 1978; 39:445-58.  Back to cited text no. 11    
12.Grote J, Lufft V, Nikutta P, Vander Lieth H, Bahlmann J, Danniel WG. Transeso­phageal chocardiographic assessment of superior vena cava thrombosis in patients with long-term central venous hemodialysis catheters. Clin Nephrol 1994;42:183-8.  Back to cited text no. 12    
13.McDonough JJ, Altemeier WA. Subclavian venous thrombosis secondary to indwelling catheters. Surg Gynecol Obstet 1971; 133:397-400.  Back to cited text no. 13  [PUBMED]  
14.Markman M. Diagnosis and manage-ment of superior vena cava syndrome. Cleve Clin J Med 1999;66: 59-61.  Back to cited text no. 14  [PUBMED]  
15.Patel V, Igwebe T, Mast H, Karetzky MS. Superior vena cava syndrome: current concepts of management. N J Med 1995; 92:245-8.  Back to cited text no. 15  [PUBMED]  
16.Demers HG, Siebold G, Schielke DJ, Mueller W, Niemeyer R, Hoeffler D. Soft right atrial catheter for temporary or perma­nent vascular access. Dial Transplant 1989; 18:130-2.  Back to cited text no. 16    
17.Donnelly PK, Hoenich NA, Lennard TW, Proud G, Taylor RM. Surgical management of long-term central venous access in uraemic patients. Nephrol Dial Transplant 1988;3:57-65.  Back to cited text no. 17  [PUBMED]  [FULLTEXT]
18.Huraib SO, Wijeyesinghe CR, Brady HR, Holtzer C, Watson AR, Uldall R. The WBW jugular Catheter as long-term vascular access for hemodialysis. Int J Artif Organs 1988;11:99-101.  Back to cited text no. 18    
19.Bour ES, Weaver AS, Yang HC, Gifford RR. Experience with the double lumen silastic catheter for hemoaccess. Surg Gynecol Obstet 1990;171:33-9.  Back to cited text no. 19  [PUBMED]  
20.Alimi YS, Gloviczki P, Vrtiska TJ, et al. Reconstruction of the superior vena cava: benefits of postoperative surveillance and secondary endovascular interventions. J Vasc Surg 1998;27:287-301.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]

Correspondence Address:
Mangalathillam RN Nampoory
P.O. Box 1427, Hawally 32015
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