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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2011  |  Volume : 22  |  Issue : 2  |  Page : 381-386
Superior vena cava syndrome in hemodialysis patient

Prince Salman Center for Kidney Disease, Riyadh, Kingdom of Saudi Arabia

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Date of Web Publication18-Mar-2011


Obstruction of blood flow in the superior vena cava (SVC) results in symptoms and signs of SVC syndrome. SVC obstruction can be caused either by invasion or external compression of the SVC by contagious pathologic processes involving the right lung, lymph nodes, and other mediastinal structures, or by thrombosis of blood within the SVC. Occasionally, both mechanisms co-exist. We hereby report a case of a 28-year-old male, Saudi patient who was diagnosed with end­stage renal disease and was maintained on regular hemodiaysis via right jugular vein dual lumen catheter for ten months. Three years later, the patient presented with signs and symptoms suggestive of SVC obstruction that was successfully managed with SVC stenting.

How to cite this article:
Molhem A, Sabry A, Bawadekji H, Al Saran K. Superior vena cava syndrome in hemodialysis patient. Saudi J Kidney Dis Transpl 2011;22:381-6

How to cite this URL:
Molhem A, Sabry A, Bawadekji H, Al Saran K. Superior vena cava syndrome in hemodialysis patient. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2022 Jan 17];22:381-6. Available from: https://www.sjkdt.org/text.asp?2011/22/2/381/77652

   Case Report Top

A 28-year-old Saudi male patient presented in 2003 with severe hypertension and impaired renal functions. Renal biopsy revealed totally sclerosed glomeruli. Diagnosis of chronic kidney disease (CKD) stage 5 was established, but no vascular access was created at that time. On No­vember 2004, hemodiaysis was started via right jugular dual-lumen polyurethane permicatheter and continued for approximately ten months. During this period, a left brachial-cephalic arterio-venous fistula (AVF) was created, and after AVF maturation in September 2005, we started using this access for hemodialysis.

On August 2008, the patient started to com­plain of facial swelling, dyspnea, and evident visible dilated collateral veins over the chest wall. Physical exam revealed a well-oriented pa­tient who was afebrile. His blood pressure was 143/85 mmHg, pulse rate: 82 b/m with tachyp­nea: 30 b/m. Mild peripheral cyanosis, neck and facial swelling, and visible dilated collateral veins over the chest wall were observed. Laboratory investigations showed hemoglobin: 13.3 g/dL, total leucocyte count: 12600/mm 3 , differential leucocyte count: polymorph 68%, lymphocyte 29%, eosinophils 3%, ESR: 45 mm/hr, Ca: 2.18 mmol/L, P: 1.86 mmol/L, and PTH: 87.5 pmol/L.

A coagulation study showed anti-thrombin III at 40% (normal levels being 50-150%), protein C activity at 19% (normal levels being 50- 150%) and protein S activity at 47% (normal levels being 58-127%).

A chest computed tomography (CT) scan re­vealed occluded superior vena cava (SVC) with many dilated collaterals [Figure 1] and [Figure 2]. The diagnosis of superior vena cava (SVC) throm­bosis was established and the patient was main­tained on oral anticoagulant (warfarin), aiming for a target INR between 2 and 2.5.
Figure 1: CT scan at the level of the chest shows subcutaneous venous collaterals (short arrows) and paravertebral collaterals (long black arrows). Note also the enhancement within a thoracic vein (head arrows).

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Figure 2: CT scan at the level of the diaphragm shows anterior thoracoabdominal wall, venous collaterals (short arrows) and large diaphragmatic collaterals (long arrows), Note also enhancement within an intercostal vein (head arrows).

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On October 2008, the patient was transferred to the Emergency Room because of increased neck and facial swelling that was associated with dysphagia and orthopnea. There was no history of vomiting or fever. An ENT surgeon was con­sulted and flexible laryngoscopy was carried out, which was negative for upper airway obs­truction. Ultrasound revealed sluggish flow in both internal jugular veins, with obscure sub­clavian veins. A chest and neck CT scan was requested. However, unfortunately, the patient was severely orthopnic and could not tolerate to lie down to perform the procedure. SVC veno­graphy was carried out, which revealed obstruc­tion of the SVC [Figure 3].
Figure 3: Venography shows collaterals; venous circulation due to SVC stenosis. Note enhancement of the internal mammary vein (long black arrows).

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The patient was treated with anticoagulant the­rapy, which was not successful to prevent the SVC obstruction. Also, because of the chronic onset and severe complaints, it was very late to use thrombolytic therapy. Therefore, a radiolo­gist intervention was consulted and a stent was placed to restore the blood flow [Figure 4], [Figure 5] and [Figure 6]. This was followed by marked improvement of the patient's clinical condition.
Figure 4: Balloon angioplasty.

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Figure 5: Venography after SVC stenting.

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Figure 6: CT scan at the level of the chest shows enhancement within an azygos vein (long white arrows).

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The patient was discharged in good condition, continued on Warfarin treatment with target INR between 2 and 2.5. Currently, the patient continues to receive hemodiaysis three-times weekly using the left AVF.

   Discussion Top

The SVC is a thin-walled blood vessel with low-intravascular pressure, enclosed in a tight compartment. It can be compressed easily be­cause the chest has no room for expansion. Extra luminal compression of the SVC can be caused by tumors or enlarged lymph nodes. Intraluminal obstruction of the SVC can be caused by infiltration by tumor, although thrombosis is a more common cause.

Malignancy is the most common cause of SVC syndrome. It accounts for more than 78% of the cases, and bronchogenic carcinoma is the most common malignancy. [1],[2]

Non-malignant causes of SVC syndrome in­clude granulomatous infections secondary to tu­berculosis, actinomycosis, aspergillosis, blasto­mycosis, nocardiosis, goiter, aortic aneurysms, and sarcoidosis. [3],[4],[5]

The incidence of SVC syndrome arising from benign etiologies is increasing. It is now more commonly associated with indwelling central venous devices that create a nidus for SVC thrombosis. [2],[6],[7]

Iatrogenic causes of SVC syndrome include venous thrombosis as a consequence of central venous catheters or pacemaker catheters and also fibrosis, caused by radiation therapy of the mediastinum. [7]

Thrombi in the SVC were detected by trans­esophageal echocardiography in 30% of the patients who had single-lumen silicone rubber hemodialysis catheters. [8]

Polyvinyl chloride, polyethylene and teflon catheters are associated with increased throm­bogenisity as compared with silicone rubber. [9],[10] The catheter that was used for hemodiaysis in our patient was dual-lumen polyurethane per­micatheter.

Patients with hypercoagulable disorders who need central venous access may require a daily low dose of warfarin to help prevent thrombus formation. [11]

Lab investigations, which were done for this patient, revealed hypercoagulable disorders (anti­thrombin III deficiency-low protein S and pro­tein C activity) and, thus, it was necessary to use Warfarin as a prophylactic anticoagulant therapy at the time of starting hemodiaysis through the permanent permicatheter.

The development of clinical manifestations of the SVC syndrome depends on the degree and rapidity of obstruction of the SVC and the cli­nical signs and symptoms of the underlying causative pathophysiologic process.

If onset of SVC syndrome is gradual, symp­toms may be subtle. However, a rapid onset of SVCS, in the absence of collateral circulation, will cause a more dramatic and life-threatening presentation, often with neurologic and respire­tory squeals resulting from cerebral and laryn­geal edema.

The onset was gradual in this patient and there were many collateral circulations as demonstra­ted by chest CT [Figure 1] and [Figure 2] Therefore, the manifestations were late for three years after the catheter was removed.

Early symptoms of SVC syndrome include swelling of the face, arms, fingers, neck, and breast. Often, the first symptom is dyspnea, which is the most common symptom, and a non-productive cough. [6],[7] Also, feelings of full­ness of the head, difficulty buttoning shirt col­lars (a stoke sign), dysphagia, hoarseness, and chest pain.

Late symptoms of SVC syndrome include life­threatening symptoms of respiratory distress, e.g. orthopnea, headache, visual disturbances, dizziness, syncope, lethargy, irritability, mental status changes, and the formation of esophageal varices. [12]

The first manifestation of SVC in this patient was neck and face swelling, while severe dys­phagia and orthopnea were the late manifes­tations, which progressed because of lateness in prompt treatment.

The choice of diagnostic procedures with sus­pected SVC syndrome depends on the patients' status.

The preferred diagnostic tools to confirm the diagnosis of SVC syndrome are a chest CT scan with IV contrast and a chest magnetic reso­nance imaging (MRI) scan. [6],[13],[14] CT and MRI scans are non-invasive, accurate in distingui­shing between tumor mass and thrombosis as causes of SVC syndrome, and able to document the extent and location of involvement.

In malignancy, accurate, definitive histologic diagnosis by biopsy or cytology specimen is necessary to provide appropriate treatment of SVC syndrome. [13]

CT and ultrasound were enough to establish the correct diagnosis for this patient, but SVC venography, which was done before stenting, was well tolerable.

The four main treatment modalities for SVC syndrome are radiation therapy (non-small cell lung cancer), chemotherapy (small cell lung cancer and lymphoma), pharmacologic therapy (steroids, diuretics, and thrombolytic therapy), and surgery.

Thrombolytic therapy may be used when SVC syndrome is caused by catheter-induced intra­luminal thrombosis. Thrombolytic therapy or ti­ssue plasminogen activators are used to treat catheter-induced thrombosis and can effectively lyse clots. [6],[15] It should be noted that treatment with thrombolytics should be initiated within five to seven days of the onset of symptoms for maximum effectiveness. [5]

Pharmacologic management of SVC syndrome with anticoagulant therapy is controversial. [13],[16] One potential preventive measure for catheter­induced thrombosis is prophylactic administra­tion of warfarin. [6],[17]

Surgical intervention of SVC syndrome in­cludes stent placement or SVC bypass, and it is used occasionally when the SVC syndrome is chronic or recurrent. [18]

It was necessary to start aggressive treatment by endovascular intervention for this patient as soon as the diagnosis was established, because the response to anticoagulant therapy was not appropriate and lead to progress in symptoms, which was a life-threatening condition.

In conclusion, central venous catheters in he­modiaysis patients may result in SVC syndrome. And, this complication can be presented after years of catheter removal.

With the increasing use of these catheters, the SVC syndrome is likely to become common a­mong hemodiaysis patients. The syndrome is serious and requires early recognition and treat­ment, and alternate approaches for renal replace­ment therapy should be considered.

Thrombolysis constitutes the cornerstone of therapy. If insufficient, endovascular interven­tions such as percutaneous transluminal balloon angioplasty or intravascular stent placement should be considered.

We think that creating an early arteriovenous fistula should be kept in mind to minimize the use of dialysis catheter.

   Questions and Answers Top

Dr. Ebadur Rahman (Chairman of the Club, Armed Forces Hospital, Riyadh): Thank you, the presentation is now open for discussion. And, the first question will be, where the SVC radiology and stent procedure were performed?

Dr. Azeb Molhem (Presenter, Prince Salman Center for Kidney Disease): The SVC radio­logy and stent procedure were performed at the King Abdulaziz Medical City in Riyadh since our unit (Prince Salman Center for Kidney Disease) is a satellite center for dialysis only.

Audience: Is the size of the central venous catheter play a role in developing the SVC thrombosis or obstruction?

Dr. Molhem: To my knowledge, there is no published data about correlation between size of catheter and complications but the number of insertions could play a role in thrombosis com­plication.

   References Top

1.Parish JM, Marschke RF Jr, Dines DE, Lee RE. Etiologic considerations in superior vena cava syndrome. Mayo Clin Proc 1981;56:407.  Back to cited text no. 1
2.Escalante C. Causes and management of supe­rior vena cava syndrome. Oncology 1993;7: 61­-8.  Back to cited text no. 2
3.Aurora R, Milite F, Vander Els N. Respiratory emergencies. Semin Oncol 2000;27:256-69.  Back to cited text no. 3
4.Haapoja I, Blendowski C. Superior vena cava syndrome. Semin Oncol Nursing 1999;15: 183­-9.  Back to cited text no. 4
5.Yahalom J. Oncologic emergencies. In DeVita V, Hellman S, Rosenberg S, (Eds.), Cancer: Principles and practice of oncology. Phila­delphia: Lippincott-Raven, 4th ed. 1993;2370­-85.  Back to cited text no. 5
6.Bertrand M. Presant CA, Klein L, Scott E. Iatrogenic superior vena cava syndrome. A new entity. Cancer 1984;54:376.  Back to cited text no. 6
7.Markman M. Diagnosis and management of superior vena cava syndrome. Cleve Clin J Med 1999;66:59.  Back to cited text no. 7
8.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 hemodiaysis catheters. Clin Nephrol 1994;42:183-8.  Back to cited text no. 8
9.Khanna S, Sniderman K, Simons M, Beseley M, Uldall R. Superior vena cava stenosis asso­ciated with hemodiaysis catheters. Am J Kidney Dis 1993;21:278-81.  Back to cited text no. 9
10.McDonough JJ, Altemeier WA. Subclavian venous thrombosis secondary to indwelling catheters. Surg Gynecol Obstet 1971;133:397­-400.  Back to cited text no. 10
11.Carrico R, ed. APIC Text Book of Infection Control and Epidemiology, 2nd ed. Washington, DC: Association for Professionals in Infection Control and Epidemiology; 2005.  Back to cited text no. 11
12.Greenwell MW, Basye SL, Dhawan SS, Parks FD, Acchiardo SR. Dialysis catheter induced superior vena cava syndrome and downhill esophageal varices. Clin Nephrol 2007;67(5): 325-30.  Back to cited text no. 12
13.DeMichele A, Glick J. Cancer-related emergen­cies. In Lenhard R, Osteen R, Gansler T, (Eds.), Clinical oncology. Atlanta, GA: American Cancer Society; 2001;733-64.  Back to cited text no. 13
14.Chen J, Bongard F, Klein S. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990;160:207-11.  Back to cited text no. 14
15.Greenberg S, Kosinski R, Daniels J. Treat-ment of superior vena cava thrombosis with recom­binant tissue type plasminogen activator. Chest 1991;99:1298-301.  Back to cited text no. 15
16.Nomori H, Nara S, Morinaga S, Soejima K. Primary malignant lymphoma of superior vena cava. Ann Thorac Surg 1998;66:1423-4.  Back to cited text no. 16
17.Bern M, Lokich J, Wallach S, et al. Very low dose of warfarin can prevent thrombosis in central venous catheters. Ann Intern Med 1990;112:423-8.  Back to cited text no. 17
18.Schafer S. Oncologic complications. In Otto S (Ed.), Oncology Nursing. St. Louis, MO: Mosby Yearbook; 3rd ed. 1997;406-74.  Back to cited text no. 18

Correspondence Address:
Khalid Al Saran
Prince Salman Center for Kidney Disease, Riyadh
Kingdom of Saudi Arabia
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PMID: 21422652

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