Surgical treatment of Budd-Chiari syndrome: analysis of 221 cases.

BACKGROUND: Budd-Chiari syndrome (B-CS) refers to post-hepatic portal hypertension and/or inferior vena cava hypertension caused by obstruction of blood flow at the portal cardinal hepatic vein. The treatments of B-CS include operations on pathological membrane lesions, shunting and combined operations. Studies have shown that China, Japan, India and South Africa have a high incidence of B-CS. In China, the Yellow River Basin in Henan, Shandong, Jiangsu and Anhui Provinces also have a high incidence, around 10 per 100 000. METHODS: The clinical data of 221 B-CS patients were analyzed retrospectively. We focused on pathological types, surgical methods, effectiveness and complications of treatment, and follow-up. RESULTS: Based on imaging findings such as color ultrasonography, angiography or magnetic resonance angiography, the 221 patients were divided into 3 types (five subtypes): type Ia (72 patients), type Ib (20), type II (72), type IIIa (33), and type IIIb (24). Surgical procedures included balloon membranotomy with or without stent (65 patients), improved splenopneumopexy (18), radical resection of membrane and thrombus (17), inferior vena cava bypass [29, with cavocaval transflow (13) and cavoatrial transflow (16)], mesocaval shunt (41), splenocaval shunt (25), splenoatrial shunt (12), splenojugular shunt (6), and combined methods (8). The complication rate was 9.05% (20/221) and the perioperative death rate was 2.26% (5/221). All of the patients were followed up from 6 months to 5 years. The success rate was 84.6% (187/221), and the recurrence rate was 8.9% (9/101) and 13.5% (13/96) after 1- and 5-year follow-up, respectively. CONCLUSION: The rational choice of surgical treatment based on B-CS pathological typing may increase the success rate and decrease the recurrence.

Necessity and indications of invasive treatment for Budd-Chiari syndrome.

BACKGROUND: The development of collaterals in Budd-Chiari syndrome has been described and these collaterals play an important role in the presentation of this disease. These collaterals are diagnostic and their use in management strategy has never been evaluated. This study aimed to investigate the indications, feasibility and necessity of invasive treatment for patients with Budd-Chiari syndrome and to determine whether such a strategy is necessary for optimal management. METHODS: Twenty-nine patients who had been treated at our unit were enrolled in this study. Based on physical and biochemical examination, and hemodynamic compensation by collaterals, 18 patients underwent radiological intervention (group A), while the other 11 had no invasive treatment (group B). The related hemodynamic parameters were acquired when percutaneous angiography was performed. RESULTS: In group A, all patients underwent successfully inferior vena cava (IVC) balloon angioplasty with or without stenting. Four patients also underwent hepatic vein angioplasty. In these patients, the mean IVC pressure before and after treatment was statistically different (29.3+/-9.2 vs 15.1+/-4.6 mmHg, P<0.01). The mean IVC pressure was much lower in group B than in group A (12.9+/-2.4 vs 29.3+/-9.2 mmHg, P<0.01), but there was no difference from that of the patients after radiological treatment (12.9+/-2.4 vs 15.1+/-4.6 mmHg, P>0.05). Median follow-up was 32.3 months (mean 21.3 months; range 3-61 months). In the course of follow-up, the patients in group A survived with good systemic status except for re-stenosis in one patient who underwent re-canalization of the IVC. In group B, 10 patients had good systemic status except one patient who had a meso-caval shunt because of deterioration. CONCLUSIONS: The rationale of "early diagnosis and early treatment" is not suitable for all patients with Budd-Chiari syndrome. Satisfactory survival can be achieved in some patients without invasive treatment, who are completely compensated by rich collaterals. Nonetheless, a positive treatment procedure should be performed if the patient's situation worsens in the course of regular follow-up.

[Diagnosis and managements of seventeen patients with hepatic veno-occlusive disease].

OBJECTIVE: To summarize the clinical experiences in the diagnosis and managements of hepatic veno-occlusive disease (HVOD). METHODS: The clinical and pathologic data of 17 patients with hepatic veno-occlusive disease were analyzed retrospectively. RESULTS: According to the results of imaging examination, clinical data and pathological data, 17 patients HVOD were divided into acute progressive HVOD and chronic HVOD. 2 cases out of the 11 acute progressive cases got improved, 2 cases died after medical treatment and 2 cases died after shunt operation. The 6 chronic HVOD, including 1 case with medical treatment and 5 cases with shunt operation, were cured. CONCLUSION: Liver biopsy was an efficient method for the diagnosis of hepatic veno-occlusive disease. Acute progressive cases of hepatic veno-occlusive disease should be managed with medical treatment and the chronic cases could be treated with shunt surgery if medical treatment were inefficient.

Radical resection of pathological membrane for Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and 1 had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.

Splenocaval versus mesocaval shunt with artificial vascular graft for the treatment of Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (B-CS) is a disease with a poor prognosis, and the results of medication are not satisfactory. Surgical treatments are widely used to depress portal hypertension and hypertension of the inferior vena cava. Splenocaval shunt is usually applied to treat intrahepatic portal hypertension, but we used this method to treat patients with B-CS successfully. METHODS: The clinical data of 72 B-CS patients (type II), including 26 patients treated with splenocaval shunt (splenocaval group) and 46 patients with mesocaval C-shape shunt (mesocaval group) were analyzed retrospectively. RESULTS: The platelet count of the splenocaval group increased significantly after operation (P<0.05). Free portal pressure (FPP) significantly decreased in both groups after operation (P<0.05), but no significant difference was seen between the two groups (P>0.05). Twenty patients in the splenocaval group and 36 in the mesocaval group were followed up for 6 months to 3.5 years, showing the effective rates of 90.0% and 91.7% respectively in the two groups. The occurrence of hepatic encephalopathy was 5.0% and 5.6% respectively in both groups, but there was no recurrent hemorrhage. CONCLUSIONS: Splenocaval shunt can effectively control B-CS, decrease FPP, prevent upper gastrointestinal hemorrhage, and eradicate hypersplenia. Its efficacy is similar to that of mesocaval shunt in treatment of B-CS.

Diagnosis and management of severe Budd-Chiari syndrome.

OBJECTIVE: To assess the diagnostic standards and treatment of severe Budd-Chiari syndrome (BCS). METHODS: The clinical data of 126 patients with severe BCS treated from November 1994 to June 2001 at our hospital were retrospectively analyzed. Percutaneous transhepatic recanalization and dilation and/or stent placement of the main hepatic vein was performed in 10 patients. Mesocaval C type shunt with artificial graft was performed in 68 patients, splenojugular shunt in 33, mesojuglar shunt in 1, and mesocaval shunt or improved splenopneumopexy after percutaneous intraluminal angioplasty and stent placement of the inferior vena cava in 14. RESULT: Six patients died during perioperation. In 120 patients followed up for 6 months to 7 years, 89 had excellent results and 31 good results. CONCLUSIONS: Diagnostic standards of severe BCS are suggested. Proper treatment should be used according to the pathological changes of the inferior vena cava and main hepatic veins.

Treatment of membranous Budd-Chiari syndrome: analysis of 480 cases.

BACKGROUND: Budd-Chiari syndrome (BCS) presents a kind of disease resulted from the occlusion of hepatic vein and/or the intrahepatic inferior vena cava. Its different pathological types were proposed. According to our experience, the membranous type takes a large part of it, and we tried to explore the best treatment of membranous BCS through the analysis of 480 cases retrospectively. METHOD: The operative results of 480 patients with membranous BCS were analysed retrospectively. RESULTS: Patients after Kimura's finger rupture, interventional treatment and membrane resection were followed up with rates of 84.62%, 86.55%, and 87.37%, respectively. The effective rates of the three methods were 61.4%, 91.7%, and 90.4%, respectively, and the recurrence rates of the disease after the 3 procedures were 38.6%, 8.3% and 9.6%, respectively. The long-term effects of interventional treatment and resection were significantly better than those of Kimura's finger rupture (P<0.05). CONCLUSION: Balloon dilatation is the choice for membranous BCS. Patients with extensive lesion, thick membrane or recurrence after percutaneous transhepatic angiography should undergo membrane resection.

Relations of Budd-Chiari syndrome to prothrombin gene mutation.

BACKGROUND: Budd-Chiari syndrome (BCS) is a type of disease characterized by portal hypertension and/or hypertension of the inferior vena cava (IVC) due to the obstruction of the hepatic veins (HV) and/or intrahepatic IVC outlet. Being etiologically complicated and obscure, BCS can be acquired or idiopathic and several gene mutations may be contributable. This study was to explore whether prothrombin gene mutation (FII G20210A) takes part in the pathogenesis of BCS and to investigate their correlativity. METHODS: In 38 proven BCS patients and 70 controls, polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) was used to find FII G20210A mutation. To detect whether there are any mutations, four steps were taken: purification of genome DNA from whole blood, amplification of special fragment by polymerase chain reaction, digestion of the fragment via restriction endonuclease, and analysis of results by polyacrylamide gel electrophoresis. RESULTS: FII G20210A mutation was not detected in all patients and controls. CONCLUSIONS: No FII G20210A mutation exists in Chinese patients with BCS, nor correlativity between the occurrence of BCS and FII G20210A mutation. The etiology of BCS in the Chinese needs further investigation.

Management of severe Budd-Chiari syndrome: report of 147 cases.

BACKGROUND: Budd-Chiari syndrome (BCS) is an uncommon disorder caused by the obstruction of hepatic venous outflow and/or the inferior vena cava. Major therapeutic approaches include operation and radiological intervention. This study was conducted to investigate the treatment of severe BCS. METHODS: The clinical data of 147 patients with severe BCS who had been treated at our hospital from November 1994 to December 2003 were retrospectively analyzed. RESULTS: One hundred twenty-one patients with BCS underwent surgery, including mesocaval C type shunt with artificial graft (82 patients), splenojugular shunt (37), mesojugular shunt (2), percutaneous transhepatic recanalization and dilatation and/or stent placement of the main hepatic vein (MHV) (12), and combined percutaneous transhepatic angioplasty (PTA) and stent placement of the inferior vena cava and mesocaval shunt (14). Follow-up for 6-108 months showed excellent results in 102 patients (69.4%), good results in 40 (27.2%), and 5 deaths. CONCLUSION: Good results could be obtained in most of patients with BCS after different surgical treatments according to the pathological changes of the IVC and MHV.

Triplex operation for portal hypertension with esophageal variceal bleeding: report of 140 cases.

BACKGROUND: Portal hypertension is a common disease. The surgical therapy of this disease focuses on the resultant upper digestive tract bleeding, which can imperil patients' life directly. This study was to evaluate the effect of triplex operation (mesocaval C shunt with artificial graft, ligation of the coronary vein and splenic artery) on portal hypertension and its associated upper digestive tract bleeding. METHODS: A retrospective study was made on clinical data of 140 patients undergoing triplex operation, who had suffered from portal hypertension and upper digestive tract bleeding. RESULTS: Postoperative portal pressure was 25-43 cmH2O(preoperative portal pressure 27-45 cmH2O) with the average reduction of 10 cmH2O. One patient (0.7%) died of cerebrovascular disease. Five patients (3.5%) suffered from mild hepatic encephalopathy, which was ameliorated through conservative treatment. Lymphatic fistula occurred in 3 patients (2.1%) who recovered without treatment 5, 10 days and 3 months after operation respectively. One hundred patients were followed up for 1 month to 6 years without recurrent hemorrhage or hepatic encephalopathy. Hypersplenism and ascites disappeared in 70 patients (70%) and 80 patients (80%) respectively. A significant reduction of ascites was seen in 12 patients(12%). The artificial vessels remained unblocking detected by B type ultrasonography and Doppler sonography in 95 patients(95%). CONCLUSION: Triplex operation is suitable for patients with the following portal hypertensions: portal hypertension caused by simple occlusion of the hepatic vein (a pathological type of Budd-Chiari syndrome); thrombosis of the portal vein or prehepatic portal hypertension because of cavernous transformation; intrahepatic portal hypertension with rebleeding after splenectomy or non-operation, and those patients with liver function in grade A or B according to the Child-Pugh classification.

Diagnosis and treatment of insulinoma: report of 105 cases.

OBJECTIVE: To study the methods for diagnosis and treatment of insulinoma. METHODS: Clinical data from 105 patients with insulinoma who had been admitted to our hospital from July 1966 to December 1999 were retrospectively reviewed. RESULTS: Fasting blood glucose values were less than 2.75 mmol/L in all the patients. Fasting serum insulin values in 60 patients were higher than 25 mU/L, average 65 mU/L. Before operation, carcinoma was detected in 2 of 45 patients by ultrasound scan, and in 10 of 35 by CT. Enucleation of insulinoma was performed in 60 patients. Operations included insulinoma resection (35 patients), distal resection of the pancreas (8), and biopsy (2). CONCLUSION: Whipple's triad and the index of insulin release >0.3 are the major variables for diagnosis. Intraoperative exploration and ultrasound scan are the methods for the localization of insulinoma. Enucleation of benign insulinoma is preferred, but proximal or distal resections of the pancreas are required only for large, deep or multiple tumors.

Surgical treatment of 1360 cases of Budd-Chiari syndrome: 20-year experience.

BACKGROUND: Budd-Chiari syndrome (BCS) is a disease 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 analyzed 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.