| Background and ObjectiveBudd-Chiari syndrome (B-CS) refers to posthepatic portal hypertension (PHT) and/or inferior vena cava (TVC) hypertension caused by obstruction of blood flow in outlet of major hepatic veins(MHV) and/or posthepatic inferior vena cava. Since 3 cases were reported respectively by George Budd from England in 1845 and Hans Chiari from Germany in 1899, with the improvement of diagnosis and therapy, a lot of cases were discovered.From May 1983 to October 2003, Professor Xu Peiqin and his colleagues had treated more than 1 300 patients with B-CS in the First Affiliated Hospital of Zhengzhou University. Severe B-CS mostly happens in those B-CS patients whose conditions develop both rapidly and progressively and spontaneous prognosis are often bad. The main manifestations are hepatomegaly , injury of hepatic and nephrotic function , intractable ascites, bleeding esophagogastric varices and hepatic encephalopathy; Main hepatic veins(MHV) are competely occlusive while no dilated compensable sub-HV exists in diagnostic imageology, and/or IVC is occlusive or narrow . The patients will die of serious complications if treated improperly. But under sufficient preoperative preparation and definitive diagnosis ofthe pathological changes, satisfactory effect can also be achieved after appropriate operation. This article aimed at further discuss the current diagnosis criterion and reasonable therapy scheme through a retrospective analysis of common conditions, clinical characteristics, diagnosis and classification, treatments and results together with data of liver function, coagulation function , blood routine comparing with normal matched controlled group and the changes of free portal pressure(FPP) after shunting.Object and Method174 patients with severe B-CS, 96 males, 78 females, range 18-46 years, mean 32.67 8.29 years. Normal matched controlled group were 35 cases, 20 males , 15 females, range 28~53 years. According to Xu Peiqin classification, 110 cases were II type, 64 cases were Illb type.Clinical characteristics: both hepatomegaly and ascites in 174 cases(100%), inertia in 104 cases(59.77%), edema in 91 cases(52.30%), splenomegaly in 82 cases(47.13%), anorexia in 73 cases(41.95%), anuria or oliguria in 71 cases(40.80%), varices on chest and abdominal wall in 68 cases(39.08%), abdominal distension or pain in 54 cases(31.03%), bleeding esophagogastric varices in 33 cases(18.97%), jaundice in 31 cases(17.82%), varices of lower extremity and pigmentation in 30 cases( 17.24%), hepatoencephalopathy in 7 cases(4.23%).Preoperative treatment: Urokinase and heparin sodium were used to prevent from thromboembolism. Propranolol was used to avoiding rebleeding. Albumin was applied to preserve liver function, artificial liver was used when necessary. Branched-chain amino acid and Arginine lessened hepatoencephalopathy. Ascites was pumped into "blood circulation after purification and concintration by using a hemodialysis machine. TPN was necessary to severe malnutrition cases. Splenic pulp pressure was measured and portovenogram was used to learn the calibre of vessels and collateral circulation. Ringed PTFE grafts(Gore-Tex) were applied to all operative cases (10030030L).Surgical procedures: Percutaneous hepatic vein angioplasty was applied to 12cases(9.77%), with metallic stent placement in 5 cases; mesocaval shunt was performed hi 93 cases(53.45%), splenocaval shunt in 5 cases(2.87%); splenojugular shunt hi 48 cases(27.59%), mesojugular shunt hi 2 cases(1.15%); mesocaval shunt after percutaneous intraluminal angioplasty with metallic stent placement hi the inferior vena cava was achieved in 14 cases(8,05%).Pressure measured during operation: To measure FPP before and after shunting by using a glass tube paracentesis of omentum vessel.Postoperative treatment: Anticoagulation therapy was persisted. Heparin sodium subcutaneous injection and low molecular dextran perfusion were performed during early postoperative period, life-long anticoagulation was maintained with oral aspirin and agacore. |
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