Acute Budd-Chiari Syndrome with Suspicious Behçet’s Disease: Successful Catheter-Directed Thrombolysis after the Failure of Anticoagulation

Budd-Chiari syndrome (BCS) with occlusion of the major hepatic veins is a rare and serious complica- tion of Behcet's disease. We report the case of a 38-year-old female who presented with abdominal pain recently. Computerized tomography of the abdomen revealed acute thrombus in the supra- to intrahe- patic IVC and thrombotic occlusion of the middle hepatic vein, which is a cardinal sign of acute BCS. The patient had suffered from recurrent oral ulcers, multiple arthralgia, and erythema nodosum, but was not diagnosed with Behcet's disease based on the international diagnostic criteria. We also tested for other autoimmune diseases, hereditary thrombophilia, antiphopholipid syndrome, myeloproliferative neoplasm and paroxysmal nocturnal hematuria, the results of which were all negative. Therefore, Behcet's disease was suspected clinically and was also thought to be the cause of BCS. She was treated with heparin for a week, but edema of lower extremities and the abdominal wall were aggravated. We performed cathe- ter-directed thrombolysis and thrombectomy followed by insertion of a stent. Abdominal distension and edema of the legs were improved immediately after the procedure. The patient has exhibited no signs of recurred BCS during 3 months of follow-up with warfarin treatment.