Redefining Budd-Chiari syndrome: A systematic review.

AIM: To re-examine whether hepatic vein thrombosis (HVT) (classical Budd-Chiari syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) are the same disorder. METHODS: A systematic review of observational studies conducted in adult subjects with primary BCS, hepatic vein outflow tract obstruction, membranous obstruction of the inferior vena cava (IVC), obliterative hepatocavopathy, or HVT during the period of January 2000 until February 2015 was conducted using the following databases: Cochrane Library, CINAHL, MEDLINE, PubMed and Scopus. RESULTS: Of 1299 articles identified, 26 were included in this study. Classical BCS is more common in women with a pure hepatic vein obstruction (49%-74%). HVC-BCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins (14%-84%). Classical BCS presents with acute abdominal pain, ascites, and hepatomegaly. HVC-BCS presents with chronic abdominal pain and abdominal wall varices. Myeloproliferative neoplasms (MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F mutation in 2%-5%. Classical BCS responds well to medical management alone and 1(st) line management of HVC-BCS involves percutaneous recanalization, with few managed with medical management alone. CONCLUSION: Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.

Budd-Chiari Syndrome in Young Chinese: Clinical Characteristics, Etiology and Outcome of Recanalization from a Single Center.

PURPOSE: We aimed to characterize the clinical profile, etiology, and outcomes of young Chinese patients with Budd-Chiari syndrome treated with recanalization. METHODS: A total of 35 consecutive young patients (≤25 years of age) with primary Budd-Chiari syndrome treated with recanalization at our center were enrolled in this study between March 2011 and December 2014. Data on baseline information, etiology tests, therapeutic recanalization strategies, and follow-up were collected. RESULTS: The most common clinical feature was ascites, present in 33 cases (94%). Hepatic vein obstruction was present in 60% (21/35) of patients, inferior vena cava obstruction in 3% (1/35), and combined obstruction in 37% (13/35). The most common risk factor for thrombosis was hyperhomocysteinemia (14/35, 40%). Recanalization was technically successful in 32 of 35 patients (91%), and clinically successful in 28 of these 32 patients (88%). The cumulative 1- and 3-year primary patency rates were 75.2 and 54.3%, respectively. The cumulative 1- and 3-year secondary patency rates were 89.3 and 89.3%, respectively. The cumulative 1- and 3-year survival rates were 96.9 and 93.8%, respectively. CONCLUSION: In this study, the most common type of lesion was hepatic vein obstruction, the most common thrombotic risk factor was hyperhomocysteinemia, and recanalization resulted in good mid-term outcomes in young Chinese patients with Budd-Chiari syndrome.

The Significance of Serum CA-125 Elevation in Chinese Patients with Primary Budd-Chiari Syndrome: A Multicenter Study.

Objective. To investigate the serum level of CA-125 and its corresponding clinical significance in Chinese patients with primary BCS. Methods. Serum CA-125 was measured in 243 patients with primary BCS receiving interventional treatment in the participating hospitals and in 120 healthy volunteers. The correlation between serum CA-125 levels and ascites volume, liver function, and prognosis was analyzed. Results. Serum CA-125 was significantly elevated in BCS patients compared to healthy volunteers (P < 0.001). Higher levels of CA-125 were found in BCS patients with abnormal hepatic function and low serum albumin levels and in patients with high volume of ascites compared to patients without these abnormalities. Serum CA-125 levels significantly correlated with ascites volume, serum level of alanine aminotransferase, aspartate aminotransferase, albumin, and Rotterdam BCS scores. The follow-up study indicated that the survival rate and asymptomatic survival rate after interventional treatment were lower in BCS patients with serum CA-125 > 175 U/mL (P < 0.05). Conclusion. Serum CA-125 was significantly higher in patients with primary BCS and had a positive correlation with the volume of ascites, severity of liver damage, and poor prognosis. Thus the serum CA-125 levels may be used to estimate the severity and prognosis of BCS in Chinese patients.

Combined thrombus aspiration and recanalization in treating Budd-Chiari syndrome with inferior vena cava thrombosis.

PURPOSE: To investigate the feasibility, safety, and effectiveness of combined thrombus aspiration and inferior vena cava (IVC) recanalization for Budd-Chiari syndrome (BCS) patients with IVC thrombosis. MATERIALS AND METHODS: From March 2011 to October 2014, 17 consecutive BCS patients with IVC thrombosis [male 13, female 4 (mean age 52.6 ± 8.4 years, range 43-72 years)] treated by combined thrombus aspiration and IVC recanalization were enrolled in this retrospective study. An 8F guiding catheter was used as the aspiration catheter. During the treatment, the aspiration catheter was placed from the right femoral vein to the IVC thrombi, and a 20-ml syringe was connected with the aspiration catheter for thrombus aspiration. IVC recanalization was performed after thrombus aspiration. Data on technical success, clinical success, and follow-up were analyzed. RESULTS: Technical success was achieved in all patients. After thrombus aspiration, 12 patients had no visible thrombi on IVC venography, while 5 patients were shown to have the IVC mural thrombi. Afterwards, 13 patients were treated by IVC balloon dilation, and 4 patients were treated by IVC stent insertion. No patient experienced dyspnea after treatment. The average IVC pressure decreased from 29.8 ± 3.4 cmH2O to 8.6 ± 2.1 cmH2O (P < 0.001). Clinical success was achieved in all patients. The average follow-up period was 15.3 ± 11.6 months (range 2-44 months). Long-term IVC patency was achieved in 15 of 17 patients. CONCLUSION: Combined thrombus aspiration and IVC recanalization can be a safe and effective method for BCS patients with IVC thrombosis.

Use of Accessory Hepatic Vein Intervention in the Treatment of Budd-Chiari Syndrome.

PURPOSE: To evaluate the clinical value of accessory hepatic vein (AHV) intervention in the treatment of Budd-Chiari syndrome (BCS). PATIENTS AND METHODS: From August 2008 to July 2014, consecutive patients with BCS caused by obstruction of three hepatic veins (HVs) with or without obstruction of inferior vena cava (IVC) were treated by recanalization or transjugular intrahepatic portosystemic shunt in our center. Patients who had the compensatory AHV and successfully underwent recanalization of AHV outflow were enrolled in this retrospective study. The clinical response to AHV drainage was analyzed. RESULTS: Compensatory AHV was found in 69 of 97 (71.1%) patients, and 66 patients successfully underwent recanalization of AHV outflow (IVC recanalization, n = 49; AHV recanalization, n = 15; both, n = 2). In total, 78 AHVs were used instead of HV as the hepatic drainage vein after treatment. Fifty-five patients had one AHV, 10 patients had two AHVs, and 1 patient had three AHVs. The average diameter of all AHV stems was 8.0 ± 2.6 mm (range 5-21 mm). Clinical response to AHV drainage was positive in all patients. Patients' symptoms and liver function improved progressively after treatment. During the follow-up of 3-74 months (average 39.4 ± 11.0 months), 11 patients experienced reobstruction at 6 to 36 months (average 16.8 ± 9.8 months) after treatment. CONCLUSION: Compensatory AHV can be effectively used instead of HV for drainage of hepatic blood in patients with BCS. AHV intervention can help to simplify the BCS treatment procedure.

Accessory hepatic vein recanalization for treatment of Budd-Chiari syndrome due to long-segment obstruction of the hepatic vein: initial clinical experience.

PURPOSE: We aimed to investigate the feasibility and effectiveness of accessory hepatic vein recanalization (balloon dilatation/stent insertion) for patients with Budd-Chiari syndrome (BCS) due to long-segment obstruction of the hepatic vein. METHODS: From March 2010 to December 2013, 20 consecutive patients with BCS, due to long-segment obstruction of three hepatic veins, treated with accessory hepatic vein recanalization (11 males, 9 females; mean age, 33.4±10.9 years; range, 22-56 years) were included in this retrospective study. Data on technical success, clinical success, and follow-up were collected and analyzed. RESULTS: Technical and clinical success was achieved in all patients. Each patient was managed with a single accessory hepatic vein recanalization procedure. No procedure-related complications occurred. The diameter of the accessory hepatic vein was 8.45±1.47 mm (6-11 mm) at the stem, and there were many collateral circulations between the hepatic vein and the accessory hepatic vein. The mean pressure of accessory hepatic vein decreased from 47.50±5.59 cm H2O before treatment to 28.80±3.47 cm H2O after treatment (P < 0.001). Abnormal levels of total bilirubin, albumin, aspartate aminotransferase, and alanine transaminase improved after the treatment. During the follow-up, three patients experienced restenosis or stenting of the accessory hepatic vein. CONCLUSIONS: In BCS due to long-segment obstruction of the hepatic veins, it is important to confirm whether there is a compensatory accessory hepatic vein. For patients with a compensatory but obstructed accessory hepatic vein, recanalization is a simple, safe, and effective treatment option.

[Budd-Chiari syndrome in children and adolescents: therapeutic radiological intervention].

OBJECTIVE: Due to its minimal-invasive approach, endovascular procedure had replaced surgery in treating Budd-Chiari syndrome (BCS). The interventional therapy was a safe and effective treatment in adults with BCS and the cure rate was high. However Budd-Chiari syndrome in children and adolescents is rare. Published literature on interventional procedure for Budd-Chiari syndrome in children and adolescents is scarce. The aim of the study was to present results of percutaneous transluminal angioplasty (PTA) and stents placement in children and adolescents with BCS and to evaluate the efficacy and safety in these patients of this approach. METHOD: Twenty-five patients [16 boys and 9 girls; average age of (14.5 ± 3.4) years old; age ranged from 5 to 17 years] with Budd-Chiari syndrome who were hospitalized from December 1990 to August 2012 were presented. All of them were diagnosed by color Doppler ultrasound scan while 12 of them had magnetic resonance venography (MRV) scan. All of the patients had undergone angiographic examination. Four cases with membranous obstruction of the inferior vena cava (IVC) were treated with PTA. One case with segmental block of IVC was treated with PTA and stent placement. Five cases with membranous obstruction of IVC and hepatic vein (/and accessory hepatic vein) were treated with PTA. Among 8 cases with membranous obstruction of hepatic veins, 6 cases were treated with PTA and the others with PTA and stent placement. Among 4 cases with blocks of 3 hepatic veins (HVs), one was treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement and the other one was unsuccessful. Three cases with obstruction of HV and accessory HV (AHV) were treated with PTA. Totally, 24 patients were treated with interventional approach and followed up. RESULT: The procedure was successful in 24 patients. The involved veins (hepatic veins or IVC) were patented after interventional procedure. The pressure of hepatic vein was (42.1 ± 4.2) cm H2O (37-50 cm H2O) (1 cm H2O = 0.098 kPa) before the interventional therapy, while it was (17.3 ± 3.3) cm H2O (14-26 cm H2O) after it. The pressure of IVC was (30.6 ± 2.9) cm H2O (26-36 cm H2O) before the interventional therapy, while it was (18.8 ± 4.2) cm H2O (15-26 cm H2O) after it. The symptoms and signs vanished instantly after interventional procedure. There were no procedure-related complications. The rate of overall initial cure was 96%. The patients were followed up for a mean of 25.8 months (range 6 months to 8 years). Seven cases developed restenosis after first procedure. Five of them were treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement. All of the involved veins were patented again. Clinical symptoms were relieved. There were no procedure-related complications as well. CONCLUSION: The interventional procedure in children and adolescents with BCS is the same as in adults. Radiological therapeutic intervention is efficacious and safe in children and adolescents with BCS.

Combining angioplasty with percutaneous microwave ablation for treating primary Budd-Chiari syndrome associated with hepatocellular carcinoma in two patients: A case report.

Percutaneous transluminal angioplasty using balloon catheters for Budd-Chiari syndrome (BCS) and transcatheter arterial chemoembolization (TACE) for unresectable hepatocellular carcinoma (HCC) have become increasingly accepted as alternative therapeutic modalities. However, few studies have investigated the clinical efficacy of combining percutaneous microwave ablation with angioplasty for patients with BCS complicated by HCC. In the present study, a safe and effective method for treating BCS associated with HCC is presented. Color Doppler ultrasonography, magnetic resonance imaging (MRI), computed tomography (CT), inferior venacavography, hepatic arteriogram and cytological examinations were used for the diagnosis. A KY2000 microwave system with an emission of 915 MHz was also employed for the treatment. Two patients with BCS associated with HCC that were administered different adjuvant drug treatments underwent percutaneous transluminal angioplasty and percutaneous microwave ablation successfully, with no treatment-related complications. Combining angioplasty with percutaneous microwave ablation may represent an alternative method for the treatment of BCS associated with HCC.

[Study on between magnetic resonance venography and digital subtraction angiography on the inferior vena cava obstructive interface morphology of Budd-Chiari syndrome].

OBJECTIVE: To evaluate magnetic resonance venography (MRV) in diagnosing obstructive interface morphology of Budd-Chiari syndrome(BCS). METHODS: MRV examination was performed on 44 cases of BCS, and the images of obstructive interface morphology of the inferior vena cava were reviewed by two radiologists. RESULTS: In all 44 cases, there were 37 cases with complete obstruction and 7 with incomplete obstruction. MRV showed 4 cases with membrane with hole of incomplete obstruction. The morphologies MRV demonstrated that the proximal part of the 37 cases with complete obstruction were mainly divided into the cone type (36 cases) and the planum type (1 case). Besides, the type of distal end of obstruction were the cone type (30 cases), the planum type (4 cases) and the irregular type (3 cases). The overall sensitivity, specificity, positive and negative predictive values for the diagnosis of MRV were respectively 100%%, 57.1%, 92.5% and 100% as compared to the DSA. CONCLUSION: The examination of MRV is capable of revealing the obstructive interface morphology of the inferior vena cava, especially for the distal end of obstruction. MRV can provide guidelines in interventional treatment of Budd-Chiari syndrome.

[Interventional treatment of idiopathic long-segment occlusion of infrahepatic inferior vena cava complicated with thrombosis].

OBJECTIVE: To evaluate the efficacy of interventional treatment of idiopathic long-segment occlusion of infrahepatic inferior vena cava (IVC) complicated with thrombosis. METHODS: Fourteen patients with idiopathic long-segment occlusion of infrahepatic IVC complicated with thrombosis underwent endovascular recanalization. All procedures were performed under local anesthesia via internal jugular vein in combination with a unilateral femoral approach. First catheter-directed urokinase thrombolysis was performed. Then the occlusion of IVC was treated with balloon angioplasty and/or stent placement. RESULTS: Iliocavogram demonstrated an occlusion of IVC from intrahepatic segment to infrarenal segment in 3 patients and an occlusion of infrahepatic IVC above renal veins in 11 patients. Thrombosis was located in IVC (n = 14) and extended to iliofemoarl veins (n = 12). Technical success was achieved in 12 patients. IVCs were recanalized successfully. Complete or partial thrombus removal was accomplished in 8 and 4 cases, respectively. Recanalization failure occurred in 2 patients. No rethrombosis occurred over a mean follow-up of 12 +/- 6 months (range: 1 - 36). And an asymptomatic restenosis of IVC was diagnosed by duplex scanning. CONCLUSION: Interventional treatment of idiopathic long-segment occlusion of infrahepatic IVC complicated with thrombosis is a safe and effective method.