Successful Recanalization and Stenting of Central Thrombosis Including Superior Vena Cava Occlusion Secondary to Behcet Disease

Purpose: Behcet disease is a rare disorder with typical symptoms including aphthous oral ulcers, genital lesions, and recurrent eye inflammation; however, because of the underlying vasculitis, these patients are also at high risk for venous thrombotic events. Vascular involvement is seen in nearly 50% of patients with Behcet disease, most commonly involving the lower extremities. Caval thrombosis is a rare complication that is generally seen in patients who have already experienced venous thrombosis in other locations.

Materials and Methods: This is a case of a 33-year-old woman with a history of Behcet disease, recurrent deep vein thrombosis (DVT), and chronic nausea requiring long-term chest port access secondary to idiopathic intracranial hypertension complicated by prior line-associated DVT who presented with dyspnea, orthopnea, chest pain, and facial and arm swelling with cyanosis. Her imaging workup was compatible with the clinical picture of superior vena cava (SVC) syndrome with a chronically occluded right internal jugular vein, thrombosis of the left subclavian and innominate veins, and total SVC occlusion.

Results: The patient’s chest port was removed, and two endovascular thrombectomies were performed with initial attempts to avoid stent placement because of the patient’s young age; however, 3 months after initial presentation, she presented again with recurrent thrombosis. We performed mechanical thrombectomy with the ClotTriever system (Inari Medical, Irvine, CA) and suction thrombectomy with the CATD system (Penumbra, Alameda, CA) and placed an SVC stent (Gore Medical, Flagstaff, AZ). Completion venography and intravascular ultrasound both demonstrated a widely patent stent with restoration of flow in the left subclavian and innominate veins. The clot removed appeared to be a mixture of acute, subacute, and chronic.

Conclusions: The patient’s swelling and cyanosis improved quickly and completely resolved by the following morning. Because of recurrent thrombosis while compliant with therapeutic Lovenox, the patient was discharged on an increased dose of Lovenox 1.2 mg/kg twice a day. Follow-up chest computed tomography at 3 months postprocedure demonstrated continued patency of all vessels intervened upon with no in-stent stenosis.