Surgical treatment for Behcet's disease with acute superior vena cava syndrome

Behcet's disease (BD) is a multisystem vasculitis with unknown etiology. The involvement of superior vena cava (SVC) is reported in less than 2% of patients with BD.

We report a patient with acute edema of neck and face associated with dyspnea as the primary manifestation. So a diagnosis of superior Vena Cava syndrome (SVCS) was made and the thickening wall of SVC was resected. An Operation was performed under cardiopulmonary bypass to remove the mass and thrombus for avoiding for pulmonary embolism.

The diagnosis of Behcet's disease (BD) didn't not be made until the recurrent oral and genital ulceration occurred 2 weeks later. The patient taked aspirin and prednisolone orally as prescribed and no recurrence were observed during the 30 months follow-up.

BD should be suspected in patients presenting with SVCS, when there is thickening of SVC, whether thrombosis or not. Early diagnosis and treatment are essential for management of BD.