Treatment Of Budd-Chiari Syndrome Research Articles

TEN-YEAR OUTCOMES OF TIPS FOR BUDD-CHIARI SYNDROME: SYSTEMATIC REVIEW AND META-ANALYSIS.

Budd-Chiari syndrome (BCS) results from the obstruction of the hepatic venous flow, usually at the level of the hepatic vein or inferior vena cava. When left untreated, it can progress with several complications, including liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) appears to be effective in a subgroup of BCS patients. To perform a systematic review and meta-analysis of TIPS effectiveness in BCS treatment, considering the survival rate, reduction in portosystemic pressure, need for liver transplantation, technical failure, and shunt dysfunction for up to 10 years of follow-up. We evaluated 17 studies published in PubMed, Science Direct, Web of Science, and SCOPUS databases, which used TIPS as a treatment for BCS, comprising 618 subjects between 18 and 78 years old. We assessed the bias risk by the NOS, NHI, and JBI scales for cohort stu-dies, before-after studies, and case series, respectively. We conducted the meta-analyses by extracting the number of events and the total patients evaluated to perform the proportion meta-analyses using the R software ("meta" package - version 4.9-6). The pooled results (95%CI) showed a 19% (25.9-12.5%) rate of portosystemic pressure reduction, 6% (1-12%) rate for the need for liver transplants despite the use of TIPS, 2% (1-6%) technical failure rate, 30% (18-46%) shunt dysfunction rate, and 88% (81-93%) for the mean frequency of patients alive between 1 and 10 years after the procedure. We stratified survival rate and found an 86% (74-93%) prevalence of living subjects during less than five years, 92% (83-97%) at five years, and a 77% frequency (68-83%) of patients alive ten years after the TIPS placement. TIPS is an effective treatment for BCS, providing a high 10-year frequency of living patients and a significant decrease in portosystemic pressure. The need for liver transplants after TIPS and the technical failure rate is low.

Budd-Chiari syndrome in children: Challenges and outcome

Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.

The change in the liver volume after direct intrahepatic portocaval shunt in patients with Budd-Chiari syndrome with diffuse hepatic vein occlusion.

To evaluate the changes in the liver volume and function after direct intrahepatic portocaval shunt (DIPS) in patients with Budd-Chiari syndrome (BCS) with diffuse hepatic vein (HV) occlusion. The clinical data of 29 patients with BCS who underwent DIPS for intractable ascites caused by diffuse hepatic vein occlusion in the Affiliated Hospital of Xuzhou Medical University were analysed retrospectively; the patients included 8 males and 21 females, with an average age of 33.3 ± 6.3years. The patients underwent abdominal CT scanning and liver function examinations before DIPS, and 1week, 3months, 6months and 12months after DIPS. The changes of the liver volume and liver function before and after DIPS were compared. All 29 patients underwent DIPS successfully. 28 patients survived during the follow-up of 12-33months, with a median follow-up of 16months. The patients' liver function were significantly improved at 3, 6 and 12months after the operation compared to before the operation (P < 0.05). The liver volumes measured before the operation and 1week, 3months, 6months and 12months after the operation were 2124.586 ± 420.889 cm3, 1926.263 ± 372.268 cm3, 1480.592 ± 183.061 cm3, 1461.904 ± 153.027 cm3 and 1469.286 ± 148.549 cm3, respectively. Compared with the preoperative liver volume, the liver volume had decreased significantly at 1week, 3months, 6months and 12months after the operation (P < 0.05). However, there was no significant difference in the liver volumes at 6 and 12months after the operation (P = 0.35). Direct intrahepatic portocaval shunt has achieved satisfactory clinical results in the treatment of BCS with diffuse hepatic vein occlusion. The congestive hepatomegaly was gradually reduced after the operation. The liver volume (which was defined as the clinical efficacy in this study) remained stable after 6months.

Behçet's Disease-related Budd-Chiari Syndrome Successfully Managed with anti-TNF Antibody: A Case Report and Review of the Literature.

Behçet’s disease (BD) is multisystemic vasculitis with heterogeneous clinical manifestations. We describe the case of a 26-year-old man who presented with Budd–Chiari syndrome (BCS) related to BD. The patient received infliximab (IFX) due to the severity of vascular involvement. Subsequently, after IFX therapy, hospital-acquired pneumonia, trapped lung, and fungal infection of the lung and central nervous system developed as complications. The patient benefited from a second course of IFX and clinical remission was achieved following early identification and treatment of complications. Data on the presentation and prognosis of BCS related to BD are extremely limited. Our case report supports the growing evidence that anti-TNF antibody is a promising treatment for BD-related BCS.LEARNING POINTSBehçet’s disease-related Budd–Chiari syndrome is a rare form of vascular involvement that severely affects mortality.Behçet’s disease-related Budd–Chiari syndrome is frequently confused with idiopathic thrombosis and may be underdiagnosed.Infliximab could be a therapeutic option for refractory Behçet’s disease with major vascular involvement, but the increased risk of opportunistic infections should be kept in mind.

Chinese multidisciplinary collaborative expert consensus for the diagnosis and treatment of Budd-Chiari syndrome (2021 version)

Budd-Chiari syndrome (B-CS) is a complicated hepatic vascular disease caused by hepatic venous outflow obstruction.There are significant differences in the pathogenesis and treatment of B-CS between China and Western countries.Given the characteristics of B-CS in our country,Budd-Chiari Syndrome and Hepatic Vascular Diseases Professional Committee of Chinese Research Hospital Association organizes domestic experts in this field to formulate the "Chinese multidisciplinary collaborative expert consensus for the diagnosis and treatment of Budd-Chiari syndrome（2021 version）".This consensus elaborates the research status of epidemiology,pathogenesis,disease classification,clinical manifestations,diagnosis and treatment of B-CS in China.Conducting basic research on pathogenesis and clinical research with high level evidence are important work direction in the future.This consensus is expected to provide guidance for clinicians to make optimal therapeutic schedules,so as to further standardize and improve the comprehensive diagnosis and treatment and basic research level of B-CS in China.

Analysis of curative efficacy of transjugular intrahepatic portosystemic shunt using viabahn stent in the treatment of Budd-Chiari syndrome with extensive hepatic vein occlusion

Objective: To investigate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) using viabahn stent in patients with Budd-Chiari syndrome (BCS) with extensive hepatic vein occlusion. Methods: Technical success rates, portal pressure gradient (PPG), complications and shunt patency rate of 25 cases with BCS with extensive hepatic vein occlusion treated by TIPS using viabahn stent were retrospectively analyzed. Color Doppler ultrasound was used for postoperative follow-up. Patency rates were evaluated by Kaplan-Meier curve. Results: The technical success rate of TIPS was 100%. Mean PPG decreased from (22.7 ± 6.2) mmHg preoperatively to (9.7 ± 2.6) mmHg postoperatively (t = 9.58, P < 0.05). Hepatic encephalopathy incidence was 8.0% (2/25), and there were no complications such as intra-abdominal hemorrhage. Twenty-five cases were followed up for 1 to 39 ( mean 19.0 ± 11.9) months, and one patient died of liver failure three months after interventional treatment. The primary patency rates at 1, 2, and 3 years after interventional treatment were 91.3%, 91.3%, and 78.2%, respectively, while the repatency rates were 100%, 92.9%, and 92.9%, respectively. Conclusion: TIPS using viabahn stent can bring better short-term and mid-term curative efficacy in treatment of BCS with extensive hepatic vein occlusion.

Reliability of Transient Elastography as a non-Invasive Technique for Detection of Fibrosis in Budd Chiari Syndrome Patients after Endovascular Intervention

Abstract Aim of the work To test the reliability of fibroscan in detection of fibrosis in patients with Budd Chiari syndrome before and after endovascular intervention (after elimination of hepatic congestion). Background transient elastography (TE) is a noninvasive methodology that has been used to monitor liver stiffness in patients with chronic viral hepatitis. One of the limitations for accurate assessment of liver fibrosis by TE is the liver congestion. Liver congestion can result from Budd Chiari syndrome (BCS).The treatment of BCS is through restoring the flow of the blood between the portal vein to the inferior vena cava, which will lead to decongestion of the liver.TE, will be tested after liver decongestion for proper detection of liver fibrosis. Patients and methods This was a prospective cohort study conducted on 25 Egyptian patients with confirmed diagnosis of primary Budd-Chiari Syndrome (BCS) in the period from June 2017 to September 2019. TE was performed three days before endovascular intervention and three months after it. Liver biopsy was taken during the intervention for assessment of METAVIR score. Comparison was done between TE assessments before and after intervention in detection of the degree of liver fibrosis in comparison to METAVIR score measured in liver biopsy. Results FVLM was the most common hypercoagulable cause in the involved patients. There was significant drop in Liver Stiffness Measurements (LSM) measured three months post-intervention indicating improvement of liver fibrosis after relieving liver congestion but still not correlated to the METAVIR scores measured in the liver biopsy. Conclusion Liver congestion has high impact on Liver stiffness measurement giving overestimation which improves significantly after decongestion of the liver by the endovascular intervention.

The Albumin-bilirubin Score Detects Changes in the Liver Function during Treatment for Budd-Chiari Syndrome: A Retrospective Observational Study.

Objective Mapping the long-term prognosis of Budd-Chiari syndrome (BCS) is difficult, as the prognosis is associated with changes in the liver function. The present study evaluated the time course changes in the liver function in a treatment group with percutaneous old balloon angioplasty (POBA) and a non-treatment group using the albumin-bilirubin score (ALBI) and Child-Pugh score during long-term follow-up. Methods In this retrospective study, 13 consecutive patients diagnosed with BCS at our hospital between 2007 and 2020 were categorized into a treatment group (n=8), which received POBA, and a non-treatment group (n=5). Differences in the liver function in the ALBI and Child-Pugh scores between the initial visit and one- and three-year follow-up were calculated and statistically evaluated. We investigated the changes in the liver function during the long-term follow-up, including events such as re-stenosis and re-treatment. Results While the Child-Pugh scores in the treatment group did not differ significantly between the initial visit and 1- or 3-year follow-up, the ALBI scores in this group improved significantly between the initial visit and the 1- or 3-year follow-up visit (p=0.0078 and 0.0156, respectively). The liver function according to the ALBI score in the treatment group showed gradual improvement from the initial value but gradual worsening in the non-treatment group. The ALBI scores also revealed that the liver function varies according to re-stenosis and re-POBA in BCS patients. Conclusion Unlike the Child-Pugh score, the ALBI score was able to capture changes in the liver function of BCS patients during the long-term course of BCS.

Application of ex vivo liver resection and autotransplantation in treating Budd-Chiari syndrome secondary to end-stage hepatic alveolar echinococcosis: A case series.

Background:Secondary Budd-Chiari syndrome (BCS) occurs due to a blockage in the liver caused by invasion or compression by a large lesion. Conventional treatments for BCS do not solve practical problems, wherease liver transplantation has been only applied as a last-resort therapy and as the only opportunity for a radical cure. We explored the feasibility of applying ex vivo liver resection and autotransplantation (ELRA) for the new indications of treating patients with end-stage hepatic alveolar echinococcosis (HAE). Our center has firstly proposed the idea and successfully treated the 49 patients with HAE. This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE.Methods:According to the degree of lesion invasion and surgical options, 11 patients were divided into 4 types. These 11 patients had large lesions that invaded the second and third hilum of the caudate lobe and involved the confluence of the hepatic vein and the inferior vena cava, suprahepatic vena cava, or at least 2 hepatic veins and led to secondary BCS. The aim of the present work was to report 11 patients with life-threatening diseases who underwent ELRA (ex vivo liver resection and autotransplantation) for secondary BCS, to propose a classification system for secondary BCS, and to suggest that secondary BCS is an indication for ELRA.Results:Eleven patients successfully underwent ELRA without intraoperative mortality. The median autograft weight was 690 g (440–950 g); operative time was 12.5 hours (9.4–16.5 hours); Postoperative hospital stay was 15 days (7–21 days). Clavien-Dindo grade IIIa or higher postoperative complications occurred in only 5 patients.Conclusions:This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE, not only provides new ideas for alternative treatments of secondary BCS, but also provides a classification system for secondary BCS. This article describes the technical process of outflow tract reconstruction and the experience for expanding the indications for ELRA. Our study demonstrated that ELRA is well feasible for treatment of BCS secondary to advanced HAE.

Long-term outcomes of individualized interventional treatment for primary Budd-Chiari syndrome complicated by inferior vena cava thrombosis.

Various endovascular treatments were used for Budd-Chiari syndrome (BCS) patients complicated by inferior vena cava (IVC) thrombosis. The best treatment for this disease remains unknown. To evaluate safety and efficacy of individualized interventional treatment for primary BCS with IVC thrombosis. Forty-seven consecutive patients with IVC involvement BCS complicated by IVC thrombosis between June 2002 and August 2020 were analyzed retrospectively. They were treated with individualized interventional treatment based on thrombus type and size. Agitation thrombolysis, transcatheter thrombus aspiration, and catheter-directed thrombolysis were initially used for fresh and mixed thrombus (n = 20), then stent implantation for compressing thrombus and IVC recanalization were performed according to the size of the residual thrombus. Direct balloon angioplasty was used for old thrombus (n = 27). Median follow-up duration was 109 (5-223) months (average 114 ± 60 months). IVC recanalization were attempted in forty patients and failed in one. The technical successful rate of IVC recanalization was 97.5%. Thrombus was completely lysed in eight patients with fresh thrombus (40%, 8/20), partially lysed in 11 patients with mixed thrombus (55%, 11/20), and no response in one patient with mixed thrombus (5%, 1/20). Thrombolytic-related complications occurred in one patient (5%, 1/20). No symptomatic pulmonary embolism occurred. Among the 11 patients with thrombus partially lysed, five patients underwent stent implantation for compressing thrombus, six patients received conservative treatment. Old thrombus was completely lysed in 15 patients (55.6%, 15/27) during follow-up. Restenosis occurred in six patients (15.4%, 6/39). Primary patency rates at 1, 5, 10 and 15 years were 92, 92, 86 and 80%, respectively. Hepatocellular carcinoma (HCC) occurred in four patients (8.5%, 4/47). One patient died of HCC. The survival rate was 97.9%. The individualized interventional treatment based on thrombus type and size for primary BCS complicated by IVC thrombosis patients is safe and effective with long-term patency and survival.

Restenosis after Balloon Dilatation with versus without Stent Placement for Hepatic Vein Involvement Budd-Chiari Syndrome.

Balloon angioplasty with or without stent placement has become the mainstream treatment of Budd-Chiari syndrome (BCS). Restenosis of hepatic vein (HV) is a tough problem. The aim of this study was to perform a meta-analysis to compare the restenosis in HV involvement type BCS patients treated by balloon dilatation with versus without stent. Meta-analysis was used to calculate the combined effect size and their 95% confidence intervals (CI), based on random effect; and calculate the risk ratio (RR) and its 95% CI based on fixed effect. The publication bias was assessed by funnel plot and Begg's test. Sixteen studies were selected for meta-analysis. One thousand and eighty-two patients (1,019 from Asian, 63 from non-Asian countries) were included. Seven hundred and five of 1,019 (69%) Asian patients received HV balloon dilatation alone. RR value (RR=0.85, 95% CI 0.68-1.08) of the two groups was obtained through meta-analysis, which meant that the risk of restenosis in balloon dilatation alone group was 15% lower than that in combined with stent placement group; although there was no significant statistical difference between two groups (p=0.178). The current meta-analysis indicated that balloon dilatation alone is first preferred approach in Asian BCS patients compared to the non-Asian patients. Balloon dilatation combined with stent placement does not reduce restenosis risk over balloon dilation alone in the treatment of BCS with HV occlusion. It is suggested that stent should be used cautiously for such patients. Key Words: Balloon dilatation, Budd-Chiari syndrome, Stent, Restenosis, Meta-analysis.

The outcomes of interventional treatment for Budd-Chiari Syndrome complicated by inferior vena cava thrombosis: Systematic review and meta-analysis

PurposeThe aim of this study was to perform a systematic review and meta-analysis to assess the safety and efficacy of interventional treatment for Budd-Chiari syndrome (BCS) complicated by Inferior Vena Cava thrombosis (IVCT) patients. MethodsWe evaluated the published studies on interventional treatment for BCS complicated by IVCT. Meta-analysis was used to calculate the combined effect size and their 95% confidence intervals (CI) based on random effect. The publication bias was assessed by Begg's test. ResultsSixteen studies on interventional treatment for BCS complicated by IVCT patient were selected for meta-analysis, a total of 767 BCS complicated by IVCT patients were included. The combined effect size (95% CI) were 99% (98–100%) for the total successful rate of IVC recanalization, 15% (11–21%) for the rate of IVC restenosis after initial operation, 92.0% (86–97%) for the rate of clinical improvement, 76% (68–84%) for the rate of thrombus clearance and 0.00% (0–1%) for the incidence of pulmonary embolism (PE). Through subgroup meta-analysis about the rate of thrombus clearance, we got the pooled results (95% CI) of individualized treatment strategy (ITS) group and non-individualized treatment strategy (non-ITS) group, were 81% (71–92%) and 73% (63–83%), respectively. ConclusionsThe interventional treatment for BCS complicated by IVCT patients is safe and effective with low incidence of PE, high thrombus clearance rate, high technically successful rate, good patency, and high clinical improvement rate. Moreover, subgroup analysis indicated that management based on the type and extent of the thrombus is proposed.

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review.

Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.

Budd-Chiari Syndrome: Anticoagulation, TIPS, or Transplant

Budd-Chiari syndrome (BCS) is a rare vascular liver disease caused by the obstruction of the hepatic venous outflow tract that may be fatal and is not adequately treated. We aim to review the current management of BCS. Analysis of recent literature by using PubMed, EMBASE, and Medline databases. Recommended treatment of BCS is based in an stepwise progressively invasive management strategy that includes anticoagulation and treatment of the underlying prothrombotic risk factors and percutaneous revascularization in short hepatic or inferior vena cava stenosis as first step then, if failure, transjugular intrahepatic portosystemic shunt reserving liver transplantation to those patients that do not respond to previous steps. Early diagnosis and treatment have improved the outcome of patients with BCS. However, BCS management remains a challenge, requiring multidisciplinary teams in referral centers with BCS expertise.

Long-term outcomes of interventional treatment for Budd-Chiari syndrome with chronic accessory hepatic vein obstruction.

Interventional treatment is the main therapy for Budd-Chiari syndrome (BCS) with hepatic vein obstruction. The aim of this study was to investigate the long-term outcomes of endovascular management for BCS with chronic accessory hepatic vein (AHV) obstruction. In total, 68 patients with primary BCS who underwent AHV dilation with or without stenting from January 2008 to December 2018 were included in this retrospective study. The technical success rate and complications were recorded. Cumulative patency rates were estimated by the Kaplan-Meier curves and compared using the log-rank test. Technical success was achieved in all 68 patients, and AHV patency was confirmed by postoperative angiography. Fifty-two patients underwent balloon dilation alone and 16 underwent adjunctive stent implantation. During a mean follow-up period of 60.2 ± 30.5 months, AHV reocclusion occurred in 14 patients in the balloon group and four patients in the stent group (χ2 = 0.034, P = 0.855). The overall cumulative 1-, 3-, 5-, and 7-year primary and secondary AHV patency rates were 90.6, 76.6, 72.0, and 68.2% and 98.4, 91.4, 89.2, and 89.2%, respectively. The cumulative 1-, 3-, 5-, and 7-year primary patency rates of the AHV were 89.8, 73.8, 71.2, and 71.2% in the balloon group and 93.3, 86.2, 75.4, and 60.3% in the stent group, respectively. There was no statistically significant difference between the two groups (P = 0.934). Interventional treatment of BCS with chronic AHV obstruction has good long-term outcomes. Both balloon dilation alone and adjunctive stent implantation can be used for AHV recanalization.

Accessory hepatic vein recanalization for hepatic vein-type Budd–Chiari syndrome

Purpose To explore the clinical efficacy and long-term outcomes of accessory hepatic vein (AHV) recanalization as a means of treating hepatic vein (HV)-type Budd–Chiari syndrome (BCS). Methods Between January 2011 and December 2018, a total of 46 symptomatic HV-type BCS patients were treated by AHV recanalization in our hospital. The technical and clinical success of this treatment, as well as associated long-term patient prognosis was assessed herein. Results The AHV recanalization approach was technically successful in 100% of patients, without any instances of complications associated with the operation. This procedure was 95.7% (44/46) clinically successful and resultant. AHV re-obstruction occurred in 12 patients. The cumulative primary one-, two-, and five-year patency rates were 77.3%, 71.7%, and 71.7%, respectively. The secondary cumulative one-, two-, and five-year patency rates were 97.7, 87.1, and 87.1%, respectively. The five-year patency rates did not differ significantly between patients treated with balloons and stents (p = .674). Based on Cox-regression analysis, younger age was an independent predictor of re-obstruction (p = .005). The cumulative one-, two-, and five-year survival rates were 97.7, 92.2, and 92.2%, respectively. Conclusions AHV recanalization is a safe and effective treatment for HV-type BCS.

Is There Still a Role for Surgical Shunts in the Treatment of Budd-Chiari Syndrome? A 25-Year Experience

PurposeTo investigate the long-term results of shunt surgery in the treatment of Budd-Chiari Syndrome. MethodsMedical records of patients treated with Budd-Chiari Syndrome between 1993 and 2006 were reviewed. ResultsThirty-seven patients (26 female, 11 male) were identified, with a median age of 30 years (range 14–51). Median duration of symptoms was 3 months (range 1 month to 10 years). Twenty-five patients, all in acute or subacute stages of disease, were treated surgically. Constructed shunts were mesoatrial in 17, portocaval in five (one was converted from a failed portorenal shunt) and mesocaval in three. Median portal pressure decreased from 44 cm H2O (range 31–55) to 20 cm H2O (range 5–27). Seven patients (28%) died in the perioperative period. Eighteen patients (72%) were followed up for a median of 186 months (24–241 months). Seven patients died during follow-up, five due to reasons related to the underlying cause and treatment. Remaining 11 patients (61%) were alive at a median of 18 years (13–25 years) with patent shunts. One-, 5-, and 10-year survival rates in patients undergoing shunt surgery were 78%, 72%, and 66%, respectively. ConclusionPortosystemic shunts may still be considered when expertise for transjugular intrahepatic portosystemic shunt or liver transplantation is not available.

1297 Cheers to Budd: Pyogenic Liver Abscess Complicated by Acute Budd-Chiari Syndrome

INTRODUCTION: Amebic liver abscess (ALA) is the most common extraintestinal manifestation of amebiasis with high mortality rates (20%). Complications include rupture; superinfection; anemia; and rarely vascular thrombosis. Budd-Chiari syndrome is characterized by post-sinusoidal portal hypertension caused by obstruction of either major hepatic veins or the inferior vena cava. CASE DESCRIPTION/METHODS: A 54-year-old alcoholic male, with a history of recent travel to India, presented with 2 weeks of fever, vomiting and abdominal pain. On exam, he was febrile, icteric, with generalized abdominal tenderness. Chemistries revealed WBCs 12000/mm3, total/direct bilirubin 6.5/3 mg/dL, AST/ALT 410/318 U/L, and ALP 310 U/L. USG RUQ showed hepatomegaly with multiple, thick-walled areas of central low attenuation, suspicious for LA. CT confirmed the presence of multiple ALAs along with rupture and delayed filling in the left and middle hepatic veins. Under USG guidance, brownish fluid (anchovy sauce-like) was aspirated and a pigtail catheter was placed. He was started on ceftriaxone and metronidazole for the treatment of pyogenic LA. Fluid serology was positive for Entamoeba histolytica and only metronidazole was continued for 21 days, along with rivaroxaban for six months for the treatment of Budd Chiari syndrome. His liver enzymes normalized in 21 days and a follow-up USG after 48 days of treatment with rivaroxaban showed no evidence of thrombosis in hepatic veins and IVC. DISCUSSION: ALA should be suspected in patients with fever &amp; jaundice with a history of recent travel to an endemic area. The rates of complications are high, commonest being an abscess rupture. Thrombosis of IVC, hepatic and sometimes right atrium, are rare complications, caused by either direct spread of inflammation or external mechanical compression by the abscess. A diagnosis of Budd-Chiari syndrome should be considered in cases of prolonged fever unresponsive to treatment or ascites with leg edema and collateral abdominal venous circulation. Underlying prothrombotic affection must be excluded. To conclude, identifying a treatable cause of Budd-Chiari syndrome, such as liver abscess, can result in early, effective, and life-saving treatment.

Inferior Vena Cava Rupture Caused by Balloon Angioplasty During the Treatment of Budd-Chiari Syndrome.

Percutaneous transluminal balloon angioplasty (PTA) is the main treatment option for Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) obstruction. However, IVC rupture caused by PTA has never been reported. Between August 2004 and December 2016, a consecutive cohort of 617 BCS patients with obstructed IVC who underwent PTA with or without stent placement were reviewed retrospectively to identify IVC rupture. Pre- and post-procedural imaging data, clinical and procedural technical data were analyzed. Of the 617 BCS patients, five patients had IVC rupture caused by PTA (0.81%). Four of these patients had retroperitoneal, intra-hepatic IVC rupture without extravasation into abdominal cavity which was successfully managed conservatively. One patient had supra-hepatic IVC rupture into the pericardial cavity which was surgically treated. IVC rupture is a rare complication of PTA treatment of BCS and most commonly located at the intra-hepatic IVC caused by oversized balloons which does not require additional treatment.

Effectiveness and Postoperative Prognosis of Using Preopening and Staged Percutaneous Transluminal Angioplasty of the Inferior Vena Cava in Treating Budd-Chiari Syndrome Accompanied with Inferior Vena Cava Thrombosis

Budd-Chiari syndrome (BCS) is a rare disorder that has relatively high prevalence in the Huang-Huai area of China. Effective treatment of BCS accompanied with inferior vena cava thrombosis is challenging. This study retrospectively analyzed the clinical effectiveness and safety of traditional open operations versus preopening and staged percutaneous transluminal angioplasty (PTA) of the inferior vena cava in treating BCS accompanied with inferior vena cava thrombosis. Data from patients hospitalized and treated for BCS accompanied with inferior vena cava thrombosis between January 1997 and December 2017 were retrospectively analyzed. Thirty-two patients received traditional open operation (open group). Fifty-six patients received preopening and staged PTA of the inferior vena cava (PTA group). Baseline and clinical data were compared between groups. The patients were followed for up to 60months. Postoperative recurrence rates and restenosis degree were recorded. Eighty-eight patients were included (47 males and 41 females), aged 41.82±10.12years (range 29-65). In the open group, no pulmonary arterial embolism was found during and after the operation, and the technique success rate was 100%. One patient died of intrathoracic bleeding. In the PTA group, 2 patients had shifting of thrombus in the inferior vena cava that blocked the blood flow restored by the preopening, one resulted in treatment failure, while the other had blood flow restored by dilation with a 12-mm balloon; the success rate was 55/56 (98.21%). The median follow-up time was 32months (range 3-60). Two patients in the open group developed restenosis 2years after operation (recurrence rate: 6.25%), and were successfully treated by balloon PTA. Seven patients in the PTA group had severe restenosis 18-42months after operation (recurrence rate: 12.96%). No thrombosis was found in these 7 patients, and normal blood flow was restored in the inferior vena cava after balloon PTA. Preopening and staged PTA of the inferior vena cava is a safe and simple method for the treatment of BCS accompanied with inferior vena cava thrombosis, with satisfactory treatment effectiveness that could be applied in clinical practice.