Management of Budd–Chiari syndrome: what is the best approach?

Abstract

Abstract Objective: Exploring the proper management of a formerly rare and life‐threatening entity, hepatocaval syndrome or the Budd–Chiari syndrome (BCS). Methods: From four hospitals, 2677 patients with BCS were collected since 1981, among them, 2546 cases underwent surgery or intervention including 170 membranotomies, 560 shunts, 232 radical resections, 1289 PTA or stents, and 295 miscellaneous procedures. Results: Ninety‐five patients in this series were caused by lesions in the inferior vena cava (IVC). Patients treated in the first decade had an effective rate of 77.7%, patency for various approaches was approximately 60–90% at 7 years. In our early experience, 55.7% patients underwent shunts, 26.1% had membranotomy and only 0.8% the interventional modality. Recently this has shifted to 16.2%, 5.2%, and 55.6%, respectively ( P < 0.01). In the early phase, 11.6% of patients were classified as stage I (less clinical severity), and 28.6% patients in stage IV (most severe). In contrast, recent patients, they were 56.1% and 4.8% ( P < 0.01). Conclusion: Therapy has evolved from the relatively simple to the more complex (such as variety of shunts and radical correction) procedures, and finally, the interventional approaches chronologically. Regarding the best approach however, it depends on various factors. For those with Type I lesion (localized disorder in the suprahepatic IVC), interventional method is the means of choice, radical corrective surgery follows. For those with type II (long segment stenosis or occlusion of the IVC), major procedures, such as the mesocavoatrial shunt, maintain an essential role. For those with type III (the hepatic venous lesions), porto‐systemetic shunt remains the procedure of choice. The increased use of interventional method is a reflection of greater awareness and more frequent detection of much earlier cases. In contrast, the number of advanced cases declines, signaling a remarkable breakthrough in the management of the BCS.