BACKGROUND: Budd-Chiari syndrome  (BCS)  is a di\|sease caused by blood flow obstruction of the main hepatic veins (MHVs) and/or the outlet of the inferior vena cava (IVC), characterized by retrohepatic portal hypertension (PHT) and/or IVC hypertension. In the past decade, over 3000 cases of BCS have been reported in China. This study was to sum up our 20-year experience in surgical treatment of BCS and to investigate its pathological classification and principles of surgery.
METHODS: The data from 1360 BCS patients were analy\|zed retrospectively.
RESULTS: Four types (6 subtypes) were classified according to IVC angiography and hepatovenography: type Ⅰa (594 patients), type Ⅰb  (123),  type Ⅱ  (292),  type Ⅲa  (237),  type Ⅲb  (112),  and type Ⅳ  (2).  Surgical procedures included: improved splenopneumopexy  (265   cases),  finger or balloon membranotomy  (407),  radical resection of membrane and thrombus  (275),  IVC bypass (88: cavocaval transflow 71 cases, and cavoatrial transflow 17 cases), mesocaval C-shape shunt  (192),  splenocaval shunt  (32),  splenoatrial shunt  (23),  splenojugular shunt  (57),  mesoatrial shunt  (8),  and combined methods (6), including plenal-cavoatrial shunt  (4),  and mesocavoatrial shunt  (2),  splenorenal shunt  (4),  mesojugular shunt  (2),  and other  methods   (1).  The perioperative death rate and the complication rate after operation was  3.09% (42/1360) and  14.8% (201/1360) respectively. 885 cases were followed up from 9 months to 15 years  (average   6.8±1.2  years. The 791  (89.4%)  of 885 patients were successfully treated, 61 patients  (6.89%)  had a recurrence, and 33 died.
CONCLUSION: Surgical treatment of BCS is dependent on a correct diagnosis and classification of the disease.