Elective-Stenting-in-Superior Vena 
Cava Syndrome Caused by Idiopathic 
Fibrosing Mediastinitis:Use of 
Self-Expandable Wallstent

AS Mullasari, Rohit Mody, Ulhas Pandurangi, V Lakshmi

Institute of Cardiovascular Diseases, 
Madras Medical Mission, Mogappair, Chennai

We present a case of superior vena cava obstruction caused by idiopathic fibrosing mediastinitis treated with a self-expandable Wallstent. A Gortex jump graft had been used previously, which was totally occluded. This procedure relieved symptoms and alleviated the need for re-operation. (Indian Heart J 2002; 54: 425–427)

Key Words: Stents, Superior vena cava syndrome, Idiopathic fibrosing mediastinitis

The majority of the superior vena cava (SVC) obstructions are due to extrinsic compression by mediastinal malignancy,1–3 lung carcinoma being the leading cause. Localized fibrosing mediastinitis is one of the most important benign etiologies.4 Although the long-term results of stenting for malignant lesions have been well studied, results in benign lesions are less well established.5 We report a case of SVC obstruction caused by fibrosing mediastinitis, treated with a self-expanding Wallstent in a patient with an occluded Gortex graft. 

Case Report

A 44-year-old male presented with a history of headache, neck fullness and distension of the neck veins in March 1998. He was diagnosed to have SVC obstruction caused by idiopathic fibrosing mediastinitis by open thoracic biopsy and a cavoatrial Gortex jump graft was performed in April 1998 in another hospital. An MRI scan 15 days later revealed graft occlusion, compression and narrowing of the SVC and irregularity in the lumen of the distal right subclavian vein. The left innominate vein was totally occluded, hence it was possible that this drainage was routed via collaterals to the right innominate vein (Fig. 1).He was treated with urokinase, anticoagulants and oral aspirin for graft occlusion which was unsuccessful. He presented to our institute in January 2001 with the same symptoms. On examination, he was found to have a facial swelling and nonpulsatile distension of neck veins. 

In view of the acute presentation due to graft occlusion and the possibility of chronic occlusion of the left innominate vein, percutaneous transluminal angioplasty and stenting to the SVC were planned. The right femoral vein and left femoral artery were cannulated under local anesthesia. An SVC angiogram was done using a 5 F pigtail catheter; this showed fibrous narrowing extending from the right innominate vein to the SVC with marked (70%) concentric stenosis (8.3 mm diameter and 38 mm length) in the lower part of the SVC (Fig. 2). The portion of the SVC above the narrowing measured 14.7 mm on QCA. The lesion was crossed using a 0.035×260 cm Teflon guidewire and predilated with a 15×40 mm Maxi LD Cordis balloon at 4 atm. A 16×50 mm Schneider Wallstent was deployed (Fig. 3) and intrastent dilatation was done using the same balloon sequentially at 6 atm. Check angiography showed no residual stenosis and good antegrade flow in the SVC (Fig. 4). The patient was put on aspirin and clopidogrel for one month post procedure. A Doppler scan at 8-month follow-up shows a patent stent with good drainage into the right atrium. On clinical follow-up after 1 year, the patient remained asymptomatic.

Obstruction of the SVC, a thin-walled vessel carrying blood at low pressure, may result from compression by tumor or intraluminal thrombus formation. This promotes edema and cyanosis ⁶ of the head and upper body and is called the SVC syndrome. In 80%–97% cases, the etiology of SVC obstruction is attributed to malignant causes such as lung carcinoma, lymphoma, malignant thymoma, malignant primary and metastatic cardiac tumors.⁷ Fibrosing mediastinitis is one of the important infiltrative benign etiologies causing SVC obstruction.⁴ Other important noninfiltrative causes of SVC obstruction are substernal goiter and Riedel’s thyroiditis.⁷ Injury to the SVC due to the catheterization is becoming an important cause.⁸ Most studies in the literature use endovascular stents for SVC obstruction caused by malignancy.^9–11 Stenting of the SVC in benign cases, where longevity is more, has been less well studied.⁸ The reported experience is primarily with Gianturco, Wall and Palmaz–Schatz stents. The rate of restenosis following stenting in various series ranges from 68% to 100%. In one series, recurring symptoms occurred in 4%–45% of patients but could be treated with anticoagulation, angioplasty or repeat stenting.¹² Palmaz–Schatz stents lack flexibility and have relatively shorter lengths. Similarly, the Gianturco stent is a self-expanding one but gaps between the stent wires make it more prone to tumor ingrowth. However, the Wallstent with its self-expanding design of woven stainless steel wires is flexible, available in various lengths and has a tightly woven pattern,⁸ which makes it more suitable for use in infiltrative benign lesions such as fibrosing mediastinitis. 

The Wallstent has been used to relieve SVC obstructions due to malignant causes ¹³ as well as benign lesions, but none of the patients had fibrosing mediastinitis or other infiltrative benign lesions as a cause.¹⁴ In a large series of patients with the SVC syndrome, which included 16 patients with benign etiology, only one had SVC obstruction due to fibrosing mediastinitis.¹⁵ In our present case, a Wallstent was used to relieve SVC obstruction due to fibrosing mediastinitis as the bypass graft was totally occluded.

Conclusions: Stenting with a self-expandable Wallstent for SVC obstruction due to an infiltrative, benign cause such as fibrosing mediastinitis is technically feasible and is a reasonable option for primary treatment or secondary to occlusion of bypass grafts.

1. Helms SR, Carlson MD. Cardiovascular emergencies. Semin Oncol 1989; 16: 463–470

2. Banker VP, Maddison FE. Superior vena cava syndrome secondary to aortic disease. Dis Chest 1967; 51: 656–662 

3. Lewis RJ, Sisler GE, Mackenzie JW. Mediastinoscopy in advanced superior vena cava obstruction. Ann Thorac Surg 1981; 32: 458–462

4. Kulpati DD, Gupta R, Saha MM, Khastgir T, Jain N. Fibrosing mediastinitis—a rare cause of superior vena caval obstruction. Indian J Chest Dis Allied Sci 1989; 31: 291–294

5. Laing AD, Thomson KR, Vrazas JI. Stenting in malignant and benign vena caval obstruction. Australas Radiol 1998; 42: 313–317

6. Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care 1992; 1: 54–64 

7. Mc Fadden PM, Jamplis RW. Superior vena cava syndrome. In: Thomas W Shields (ed). General Thoracic Surgery. 4th ed. Chicago: Williams & Wilkins Publishers; 2000. pp. 1716–1717

8. Schindler N, Vogelzang RL. Superior vena cava syndrome. Experience with endovascular stents and surgical therapy. Surg Clin North Am 1999; 79: 683–694

9. Uchita S, Hata T, Tsushima Y, Matsumoto M, Hina K, Moritani T. Primary cardiac angiosarcoma with superior vena caval syndrome: review of surgical resection and interventional management of venous inflow obstruction. Can J Cardiol 1998; 14: 1283–1285 

10. Chin DH, Petersen BD, Timmermans H, Rosch J. Stent-graft in the management of superior vena cava syndrome. Cardiovasc Intervent Radiol 1996; 19: 302–304

11. Crowe MT, Davies CH, Gaines PA. Percutaneous management of superior vena cava occlusions. Cardiovasc Intervent Radiol 1995; 18: 367–372

12. Yim CD, Sane SS, Bjarnason H. Superior vena cava stenting. Radiol Clin North Am 2000; 38: 409–424 

13. Stock KW, Jacob AL, Proske M, Bolliger CT, Rochlitz C, Steinbrich W. Treatment of malignant obstruction of the superior vena cava with the self-expanding Wallstent. Thorax 1995; 50: 1151–1156 

14. Qanadli SD, El Hajjam M, Mignon F, de Kerviler E, Rocha P, Barre O, et al. Subacute and chronic benign superior vena cava obstructions: endovascular treatment with self-expanding metallic stents. AJR Am J Roentgenol 1999; 173: 159–164

15. Kee ST, Kinoshita L, Razair MK, Nyman UN, Semba CP, Dake MD. Superior vena cava syndrome: treatment with catheter directed thrombolysis and endovascular stent placement. Radiology 1998; 20: 187–193