A 36-year-old female from Tunisia presented with slow-onset, moderate SVCS. She was diagnosed with nodal and oesophageal tuberculosis in 2012 and treated with a 6-month course of anti-tubercular treatment. One year prior she had a mediastinal mass that regressed with corticoids. She presented with symptoms including cough, weakness, weight loss (15 kg), thoracic pain, distended collateral venous circulation of the thorax, and significant oedema for 2 years (moderate SVCS and Kishi score = 5). She had no history of central venous device placement or thrombophilic risk. Lymphoma or recurrence of tuberculosis was suspected. Bronchoscopy with biopsy, analysis of bronchoalveolar fluid, positron emission tomography (PET) CT, and 2 endobronchial ultrasounds ruled out infection or malignant cause. Pathology demonstrated inflammatory tissue without granuloma. Streptococcus parasanguinis was found on bacterial culture of bronchoalveolar fluid and was treated by antibiotics. A contrast-enhanced CT scan with 3-dimensional reconstruction (Figure 1) revealed significant superior vena cava compression secondary to MF. Intraoperative venograms performed via the right and left arms confirmed pre-occlusive stenosis of the right internal jugular (IJ) and innominate veins and SVC with extensive collateral veins and type 4 SVCS according to the Standford Classification (Figure 2). We decided not to perform mediastinoscopy because of prior negative biopsies on endobronchial ultrasounds.
Primary endovascular repair was discussed and planned by a multidisciplinary team including a thoracic surgeon, interventional radiologist, pulmonologist, and oncologist. The ethical committee approved the informed patient consent, procedure, and report.
Under local anaesthesia, a percutaneous venous access was achieved through the right common femoral vein. A 12F sheath was inserted, and two 0.035-in hydrophilic guidewires (Glidewire Terumo, Somerset, NJ, USA) were manoeuvred in parallel past the venous obstruction (Figure 3). Pre-dilation was performed using a 6-mm Armada balloon (Abbott Vascular, Lakeside Drive, Santa Clara, CA, USA) inflated in the confluence of SVC, right IJ and innominate veins. A 10 mm x 37 mm balloon-expandable covered stent (Begraft Bentley InnoMed GmbH, Hechingen, Germany) was deployed at the beginning of the SVC. Complementary simultaneous kissing stents were deployed in the SVC bifurcation, with a 8 mm x 29 mm stent in the right IJ vein and a 8 mm x 39 mm balloon-expandable bare metal stent (Omnilink Elite; Abbott Vascular) in the innominate vein. The parallel stents extended beyond the initial part of the stent in the SVC (Figure 4). A venogram demonstrated complete coverage of the SVC bifurcation and patent right IJ and innominate veins and SVC without any extravasation (Figure 5). The pressure gradient across the occlusions before angioplasty was 20 mm Hg and it decreased to 8 mm Hg on both sides. There were no procedural complications. There was a marked improvement in symptoms. Dual anticoagulation therapy with acetylsalicylic acid (160 mg once daily) and therapeutic low-molecular-weight heparin was started immediately after the intervention. The patient was discharged on the fourth day post-surgery. Her drug regimen at discharge included long-term anticoagulation (vitamin K inhibitors) and antiplatelet therapy. A CT scan confirmed stent patency at discharge and 1 year (Figure 6).