Budd-Chiari Syndrome Patients Research Articles

Recanalization of accessory hepatic vein for hepatic vein-type Budd-Chiari syndrome.

To evaluate the clinical efficacy and long-term outcomes associated with the treatment of hepatic vein (HV)-type Budd-Chiari syndrome (BCS) via accessory HV (AHV) recanalization. In total, 26 HV-type BCS patients underwent AHV recanalization between July 2014 and December 2019 at our hospital, while 73 HV-type BCS patients without compensatory AHV underwent main HV (MHV) recanalization and served as controls in the present study. Short- and long-term clinical outcomes were compared. AHV and MHV recanalization approaches were both associated with 100% technical success rates, with one recanalization procedure being performed per patient. Respective clinical success rates for the AHV and MHV recanalization approaches were 96.2% and 94.5% (P = 0.744). Re-obstruction rates were comparable between these two approaches at 20% and 34.8%, respectively (P = 0.17). Primary cumulative 1-, 2-, and 5-year patency rates in the AHV group were 96.0%, 91.6%, and 76.3%, respectively, whereas in the MHV group, these three respective rates were 87.0%, 78.6%, and 58.6% (P = 0.048). Secondary cumulative 1-, 2-, and 5-year patency rates in the AHV group were 96.0%, 96.0%, and 96.0%, respectively, whereas in the MHV group, they were 97.1%, 97.1%, and 81.8%, respectively (P = 0.289). Cumulative 1-, 2-, and 5-year survival rates for AHV group patients were 96.0%, 96.0%, and 96.0%, respectively, while for the MHV group, these respective rates were 98.6%, 95.2%, and 89.7% (P = 0.462). HV-type BCS can be safely and effectively treated via AHV recanalization, which may achieve longer patency relative to MHV recanalization.

Outcomes of status 1 liver transplantation for Budd-Chiari Syndrome with fulminant hepatic failure.

There is a paucity of data on the outcome of liver transplantation (LT) in Budd-Chiari Syndrome (BCS) patients who are listed as status 1. The objective of our study was to determine patient or graft survival following LT in status 1 BCS patients. We utilized United Network for Organ Sharing (UNOS) database to identify all adult patients (> 18years of age) listed as status 1 with a primary diagnosis of BCS in the United States from 1998 to 2018, and analyzed their outcomes and compared it to non-status 1 BCS patients. Four hundred and forty-six patients with BCS underwent LT between 1998 and 2018, and of these 55 (12.3%) were listed as status 1. There was no difference in long-term post-liver transplant or "intention-to-treat" survival from the time of listing to death or the last day of follow-up between status 1 and non-status 1 groups. Graft and patient survival at 5years for status 1 patients were 75% and 82%, respectively. Cox regression analysis showed that patients listed as status 1 (aHR: 0.45, p<.02) were associated with a better survival. BCS patients listed as status 1 have excellent survival following emergency LT.

Clinical outcomes of large diameter balloon angioplasty for Budd-Chiari syndrome with inferior vena cava involvement

ObjectiveWe compared the long-term outcomes of small and large diameter balloon angioplasty for Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) involvement in a retrospective cohort study. MethodsOf 119 patients with BCS and IVC involvement, 23 had undergone small diameter balloon angioplasty (diameter, 14-20 mm; group A) and 96 had undergone large diameter balloon angioplasty (diameter, 24-30 mm; group B). The patients were considered cured clinically if the IVC was patent with no symptom or signs evident. ResultsFrom January 2010 to December 2016, 119 BCS patients with IVC involvement had undergone balloon angioplasty, with angioplasty successful in all 119 patients. One patient died of pulmonary embolism after dilation in group A. Abdominal pain was the most common complication during dilation and was experienced by significantly more patients in group B (77.1%) than in group A (47.8%; P = .009). Of the 119 patients, 91 (76.5%) were considered cured, with significantly more patients in group B (82.3%) than in group A (52.2%; P = .005). The mean follow-up period was 41.3 ± 2.2 months. In group B, the 1-, 3-, and 5-year primary and secondary patency rates were 82.2% ± 4.2%, 69.8% ± 5.4%, and 54.0% ± 7.0% and 97.5% ± 1.7%, 92.5% ± 3.3%, and 90.2% ± 3.9%, respectively. The patency rates at all follow-up intervals were lower in group A than in group B. The clinical response rate in group B was also significantly greater than that in group A (96.9% vs 78.3%; P = .007). The 1-, 3-, and 5-year survival rates were 100%, 88.7% ± 7.6%, and 88.7% ± 7.6% and 96.5% ± 2.0%, 92.5% ± 3.0%, and 86.0% ± 5.4% in groups A and B, respectively. ConclusionsLarge diameter balloon angioplasty is safe and effective for BCS patients with IVC involvement, yielding better patency rates and long-term outcomes compared with small diameter balloon angioplasty.

Evaluation of upper endoscopic findings before and after Transjugular Intrahepatic Portosystemic Shunt in Egyptian Patients with Budd-Chiari Syndrome

Background and study aim: Successful transjugular intrahepatic portosystemic shunt (TIPS) insertion effectively lowers the portosystemic pressure gradient, resolves ascites and improves esophageal varices, we studied the effect of TIPS on the grades of gastroesophageal varices and portal hypertensive gastropathy (PHG) in Egyptian patients with Budd-Chiari Syndrome (BCS). Patients and Methods: This prospective study was conducted at the Tropical Medicine Department; Ain Shams University on 20 patients with confirmed BCS with esophageal, gastric varices and PHG. Upper GI endoscopy was conducted before TIPS. Patients were followed up by upper GIT endoscopy at one and six months after TIPS. Results: After TIPS, there was a highly significant improvement of portal pressure, (before TIPS: Mean ±SD =24.9±1.9; after TIPS: 8.3±1.5, p-value <0.001) and esophageal varices (EV). In two of three patients with large EV within one-month post-TIPS and in all 3 patients within 6 months post TIPS. 15 patients with medium-sized EV markedly improved to 7 patients reduced to small EV and one reduced to non EV in one month and 9 patients to small EV and 6 reduced to non EV after 6 months and all 2 patients with small EV reduced to non EV after one and six months. After TIPS, there was a highly significant improvement of PHG in most cases. Conclusion: Successful TIPS insertion in BCS patients leads to improvement in esophageal varices & PHG.

Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study

AbstractIn a collaborative multicenter case-control study, we investigated the effect of factor V Leiden mutation, prothrombin gene mutation, and inherited deficiencies of protein C, protein S, and antithrombin on the risk of Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT). We compared 43 BCS patients and 92 PVT patients with 474 population-based controls. The relative risk of BCS was 11.3 (95% CI 4.8-26.5) for individuals with factor V Leiden mutation, 2.1(95% CI 0.4-9.6) for those with prothrombin gene mutation, and 6.8 (95% CI 1.9-24.4) for those with protein C deficiency. The relative risk of PVT was 2.7 (95% CI 1.1-6.9) for individuals with factor V Leiden mutation, 1.4 (95% CI 0.4-5.2) for those with prothrombin gene mutation, and 4.6 (95% CI 1.5-14.1) for those with protein C deficiency. The relative risk of BCS or PVT was not increased in the presence of inherited protein S or antithrombin deficiency. Concurrence of either acquired or inherited thrombotic risk factors was observed in 26% of the BCS patients and 37% of the PVT patients. We conclude that factor V Leiden mutation and hereditary protein C deficiency appear to be important risk factors for BCS and PVT. Although the prevalence of the prothrombin gene mutation was increased, it was not found to be a significant risk factor for BCS and PVT. The coexistence of thrombogenic risk factors in many patients indicates that BCS and PVT can be the result of a combined effect of different pathogenetic mechanisms.

Ultrasonic Evaluation of Spontaneous Portosystemic Shunts in Patients with Budd-Chiari Syndrome

Background: Spontaneous portosystemic shunts (SPSS) are one of the hallmarks of Budd-Chiari syndrome (BCS). Ultrasound can accurately show the location and type of portosystemic collaterals. Objectives: To study the sonographic feature of SPSS in patients with BCS and to evaluate differences in the main portal vein diameter among multiple types of portosystemic shunts. Patients and Methods: Ultrasonographies of 44 patients with SPSS among 352 BCS patients between June 2000 and November 2015 were reviewed retrospectively. The SPSS in 44 BCS patients were first detected by ultrasound and then confirmed via digital subtraction angiography (DSA), computed tomography angiography (CTA) or magnetic resonance venography (MRV). The location, course, diameter and hemodynamics of the spontaneous portosystemic shunts were observed by ultrasound. In addition, one-way analysis of variance (ANOVA) was performed to evaluate the difference in the main portal vein diameter between the different shunt types. Results: The blood drainage patterns of SPSS in 44 of 352 patients with BCS were classified as the following five types: portal-umbilical shunts (15/44), portal-hepatic shunts (11/44), portal-accessory hepatic shunts (6/44) (the accessory hepatic veins included the inferior right hepatic vein and the caudate lobe vein), splenorenal shunts (8/44) and main portal vein-inferior vena cava shunts (4/44). The corresponding hemodynamics of the five types mentioned above were obtained. Main portal vein-inferior vena cava shunts had a significantly larger mean portal trunk diameter compared with all other types (P &lt; 0.05 for all comparisons). In addition, the mean portal trunk diameters in portal-umbilical shunts and portal-hepatic shunts were obviously larger than that of splenorenal shunts (P &lt; 0.05), while there were no statistically significant differences between the other types. Conclusion: Spontaneous portosystemic shunts are not rare in patients with BCS. Ultrasound is a reliable means for their diagnosis and it offers substantial information for use in clinical treatment.

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.

Restenosis after recanalization for Budd-Chiari syndrome: Management and long-term results of 60 patients.

BACKGROUNDBudd-Chiari syndrome is defined as hepatic venous outflow tract obstruction. For Asian Budd-Chiari syndrome patients, the major treatment modality is recanalization (percutaneous transluminal angioplasty with or without stent implantation). The cumulative 1-, 5-, and 10-year primary patency rates and survival rates are reported to be excellent or satisfactory, but the long-term outcome of patients with restenosis (the most common complication after recanalization) is unknown.AIMTo explore the treatment strategy for restenosis in patients with Budd-Chiari syndrome after interventional therapy and to evaluate the long-term follow-up results.METHODSThe clinical data and follow-up results of 60 patients with restenosis after interventional therapy from November 1983 to December 2013 were retrospectively analyzed.RESULTSSixty patients with restenosis were retrospectively divided into a percutaneous transluminal angioplasty (PTA) group (40 patients) and a PTA + stent group (20 patients) according to the primary recanalization method. For the patients with restenosis in the PTA group, 13 refused treatment, and 27 received further treatment; among these patients, five had a second restenosis, two had a third restenosis, and one had a fourth restenosis. For the patients with restenosis in the PTA + stent group, nine refused treatment, ten received PTA alone, and the other received PTA + stent implantation. Among the patients who received further treatment, five had a second restenosis, three had a third restenosis, and one had a fourth restenosis. The 1-, 5-, 10-, 20-, and 25-year cumulative survival rates of the 38 patients who received further treatment after restenosis were 100%, 78.3%, 78.3%, 70.5%, and 70.5%, respectively; however, for the 22 patients who refused treatment, the survival rates were 72.7%, 45.9%, 30.6%, 10.2%, and unavailable, respectively (P < 0.001).CONCLUSIONLong-term follow-up after interventional therapy is very important. Active treatment for patients with restenosis can improve prognosis, and minimally invasive treatment strategies for restenosis allows to obtain satisfactory results.

An Update on the Management of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.

Treatment strategies of Budd-Chiari syndrome during the epidemic period of 2019 coronavirus disease

Prevention and control about the situation of 2019 coronavirus disease (COVID-19) are grim at present. In addition to supporting the frontline actively, medical workers in general surgery spare no efforts in making good diagnosis and treatment of specialized diseases by optimizing treatment process, providing medical advice online, mastering indications of delayed operation and emergency operation reasonably, etc. Budd-Chiari syndrome is a complex disorder, and severity of the disease varies, serious cases can be life threatening. While fighting the epidemic, medical workers should also ensure the medical needs of patients. However, instead of continuing the traditional treatment, a new management system should be developed. Based on the characteristics of Budd-Chiari syndrome patients in China and our experience, patients are divided into ordinary cases and critical cases, and treatment strategies suitable for the epidemic period of COVID-19 are put forward for reference and discussion by physicians.

Inferior vena cava obstruction in Budd-Chiari syndrome: a comparative study of rapid quantitative phase-contrast MRI and MRV.

The purpose of this study was to analyze inferior vena cava (IVC) obstruction in Budd-Chiari syndrome (BCS) using rapid quantitative phase-contrast (PC) magnetic resonance (MR) imaging and compare the diagnostic efficacy of PC and MR venography (MRV) to explore the clinical prospects of applying PC quantitative diagnosis in IVC obstruction in BCS. PC quantitative data and MRV qualitative data obtained in 35 DSA-recognized inferior vena cava obstruction in BCS patients and 35 patients with liver focal lesions found in physical examination were analyzed and compared. The areas under the ROC curve for diagnostic parameters related to PC quantitative parameters, including stroke volume, forward flow volume, absolute stroke volume, mean flux, stroke distance, mean velocity (MV), and peak velocity, were 0.7767, 0.7788, 0.7747, 0.7665, 0.9143, 0.9159, and 0.8947, respectively. The sensitivity, specificity, and accuracy of MV in the diagnosis of IVC obstruction were 88.57%, 85.71%, and 87.14%, respectively. For IVC obstruction in BCS, there was no significant difference between the diagnostic efficacy of MV (one of the quantitative parameters of PC) and that of MRV (P = 0.0768). PC can be used to diagnose IVC obstruction in BCS and improve the understanding of hemodynamics of IVC obstruction in BCS.

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.

Anticoagulating Budd-Chiari syndrome patients presenting with variceal bleed: A retrospective study.

This aims to study incidence of re-bleeding on anticoagulation and survival of Budd-Chiari syndrome (BCS) patients presenting with variceal bleeding. Budd-Chiari syndrome patients presenting with variceal bleed between 01/01/2007 and 01/05/2019 were retrospectively studied. Patients underwent endoscopic treatment±endovascular therapy, followed by anticoagulation. Variceal re-bleed (on anticoagulation) and survival were studied. Of 376 BCS patients diagnosed during the study period, 40 (10.7%) patients, presenting with variceal bleed (age 33 [25-40] years; male patients 70%; Rotterdam score 1.13 [0.63-1.22]), Group 1 were compared with 40 randomly selected age-matched BCS patients presenting with ascites, no bleeds (40 [23-42] years; male patients 42.5%; Rotterdam score 1.11 [1.09-1.16]), Group 2. The commonest site of obstruction was hepatic vein (65%) in Group 1 and combined hepatic veins and inferior vena cava (57.5%) in Group 2 (P<0.01). Thirty-six Group 1 patients underwent endoscopic intervention (variceal ligation, 33; sclerotherapy, 2; glue injection, 1). Endovascular intervention was performed in 30 Group 1 patients (angioplasty±stent, 22; endovascular shunt, 8) and in 34 Group 2 patients (angioplasty±stent, 26; endovascular shunt, 8). All 80 patients were started on anticoagulation. Variceal bleed on anticoagulation occurred in five patients in Group 1 and three patients in Group 2. One-year and 5-year survival were 94.2% and 87.5%, respectively, in Group 1 and 100% and 80%, respectively, in Group 2. About one-tenth of BCS patients present with variceal bleed. On management with endoscopic±endovascular therapy, followed by anticoagulation, variceal re-bleed in these patients were comparable with those in BCS patients presenting with ascites and survival was excellent at 1 and 5years.

Prevalence of prothrombotic factors in patients with Budd-Chiari syndrome or non-cirrhotic nonmalignant portal vein thrombosis: A hospital-based observational study.

Comprehensive investigations on the prothrombotic factors of splanchnic vein thrombosis (SVT), including Budd-Chiari syndrome (BCS) and non-cirrhotic nonmalignant portal vein thrombosis (PVT), in Eastern patients are scarce. Between March 2012 and July 2017, 812 consecutive patients, including 418 BCS and 394 non-cirrhotic nonmalignant PVT patients, were admitted to Xijing Hospital (a Chinese tertiary academic hospital) and screened for prothrombotic factors. Odds ratios (ORs), 95% confidence intervals (CIs), and P-trends were calculated by using conditional logistic regression. The prevalence of myeloproliferative neoplasms (MPNs) was only 6.3% among BCS patients but 28.3% among PVT patients. Notably, the presence of MPNs was associated with a higher risk of hepatic vein-type BCS (OR 9.9, 95% CI 3.6-26.7, P-trend <0.001) and extensive thrombosis in PVT (OR 4.1, 95% CI 1.9-8.9, P-trend <0.001). Calreticulin mutations existed in 2.7% of SVT patients. Furthermore, the prevalence of antiphospholipid antibody syndrome and protein C, protein S, or antithrombin deficiency in BCS patients was 7.3% and 22.5%, respectively, similar to that in patients with PVT (7.4% and 25.7%). In addition, factor V Leiden mutation, prothrombin G20210A mutation, and paroxysmal nocturnal hemoglobinuria were identified in <1% of both BCS and PVT patients. There is a significant positive association between MPNs and hepatic vein-type BCS or non-cirrhotic nonmalignant PVT with extensive thrombosis. Additionally, calreticulin mutations should be tested in JAK2V617F -negative SVT patients in China. However, screening for factor V Leiden mutation, prothrombin G20210A mutation, and paroxysmal nocturnal hemoglobinuria may be unnecessary.

Dabigatran as an oral anticoagulant in patients with Budd–Chiari syndrome post‐percutaneous endovascular intervention

Anticoagulants play an important role in the management of Budd-Chiari syndrome. There is a paucity of data on the efficacy and safety of direct-acting oral anticoagulants-dabigatran, among patients with Budd-Chiari syndrome. In a retrospective analysis of prospectively maintained data, the stent patency rates, major bleeding episode, and a composite endpoint of major bleed and/or mortality rates were compared between Budd-Chiari syndrome patients treated with dabigatran (n=36) or vitamin K antagonists (n=62) following endovascular intervention. The baseline characteristics, including sites of block and types of interventions, were similar between the two groups. The mean duration of follow-up in the dabigatran and vitamin K antagonist groups was 10.5±6.7 and 14.1±6.9months (P=0.006), respectively. The endovascular stent patency rates were comparable between the dabigatran and vitamin K antagonist groups at 6months (91% vs 96.5%) and 12months (91% vs 93%), P=0.296 (log-rank test), respectively. Major bleeding events were comparable between the dabigatran and vitamin K antagonist groups at 6months (3.5% vs 2%) and 12months (3.5% vs 6.5%), P=0.895 (log-rank test), respectively. The composite endpoint of mortality and major bleed was comparable between dabigatran and vitamin K antagonists at 6months (4% vs 5%) and 12months (4% vs 8%), P=0.875 (log-rank test), respectively. Dabigatran, as compared with vitamin K antagonists, is associated with similar stent patency rates and complications among patients with Budd-Chiari syndrome post-endovascular intervention.

2952 Liver Transplant in Patients With Budd-Chiari Syndrome: A National Study

INTRODUCTION: Budd-Chiari syndrome (BCS) involves obstruction of hepatic venous outflow tracts at various levels from small hepatic veins to the inferior vena cava and is the result of thrombosis or its fibrous sequelae. The clinical presentation and clinical manifestations are so varied that it should be suspected in any patient with acute or chronic liver dysfunction. Liver transplantation (LT) may be indicated as a rescue treatment or for fulminant cases with promising results. Living donor liver transplantation (LDLT) can also be used for patients with BCS if deceased donor livers are scarce. The Aim of this study is to assess the mortality rate and review the safety of liver transplant in patients with Budd-Chiari Syndrome. METHODS: The National Inpatient Sample (NIS) is the largest all-payer inpatient database consisting of approximately 20% of all inpatient admissions to nonfederal hospitals in the United States. We collected and reviewed data records of new liver transplant patients from the NIS database from the year 2009 to 2014. IBM Statistical Package for the Social Sciences (SPSS) version 25 was used for statistical analysis. RESULTS: A total of 5,592 patients were discharged between 2009-2014 with a new liver transplant. Of these patients, there were 37 patients (0.66%) with BCS. The overall mortality rate for a new liver transplant was substantially higher for BCS patients (10.8%) when compared to non-BCS patients (4.0%) with an odds ratio (OR) of 2.92 (CI 1.03-8.32). However, liver transplant complications were not significantly higher in BCS patients (27.0%) when compared with non-BCS patients (15.3%) with OR of 2.06 (CI 0.99-4.26). The mean length of stay was higher in BCS patients at 32 days as compared to non-BCS patients at 20 days (P = 0.001). Results of the statistical analysis are presented in Tables 1 and 2. CONCLUSION: LT can be indicated as a rescue treatment or for fulminant cases with promising results. LDLT can also be used for patients with BCS, but it involves a difficult procedure particularly regarding venous outflow reconstruction. Our research indicates that liver transplant is riskier in patients with BCS and we encourage more research to be done regarding BCS patients regarding liver transplantation. Limitations of the study are that the NIS is an administrative database which predisposes to errors from coding inaccuracies as well as the inability to distinguish temporal relationships between variables. Future prospective studies can expand on these conclusions.

2209 Abdominal Arterial Aneurysms in a Patient With Budd-Chiari Syndrome

INTRODUCTION: Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction. Chronic BCS eventually leads to post-sinusoidal hypertension resulting in the symptoms of the disease. There have been case reports of BCS leading to intra-abdominal bleeding from ectopic varices, however, these are cases of venous bleeding. Here, we report a unique case of a Chinese woman with BCS presenting with bleeding from arterial aneurysms. CASE DESCRIPTION/METHODS: A 61-year-old woman with BCS requiring multiple prior porto-systemic shunting procedures was brought by EMS for coffee ground emesis. At the ED, she was stuporous and developed bloody emesis and hypotension. She was intubated and transferred to the ICU. Exam was remarkable for abdominal distention (AD) with a fluid wave. Labs showed leukocytosis; prolonged coags, elevated troponins, lactate, LDH and decreased fibrinogen and haptoglobin. FAST showed intra-abdominal free fluid. EGD showed grade II varices in the esophagus that were banded. However, the patient continued to deteriorate with increasing AD. CT-AP revealed a cirrhotic liver with occluded left and middle hepatic veins and extravasation of contrast near the greater curvature of the stomach into a LUQ hematoma (Figure 1). IR embolized the left gastric artery and identified aneurysms along a branch of the SMA (Figure 2) in addition to an aneurysm at the celiac artery origin (Figure 3). On the 2nd post-op day, the patient again developed hypotension with AD. Attempted embolization of the SMA branch was unsuccessful. On the 5th post-op day, the family agreed to withdrawal of life support and the patient was extubated and vasopressors were stopped. The patient passed away in a few hours. DISCUSSION: Chronic BCS presents with complications of cirrhosis; i.e., ascites and rupture of esophageal varices, like in our patient. On CTA, both the hepatic veins and part of the IVC were occluded. In fact, 31% of BCS patients have outflow occlusion at both these locations. However, in our patient, CTA revealed aneurysms in the celiac artery and distal portions of an SMA branch along with left gastric artery bleeding. Multiple arterial aneurysms have been associated with disorders such as PAN, Behcet's, Ehler-Danlos etc. By comparison, there have not been many reports of multiple idiopathic arterial aneurysms in adults. Several of these possible causes were unable to be worked up for due to the acute presentation, although our patient’s history was not typical for these diseases.

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.

DIAGNOSTIC VALUE OF JAK2 V617F MUTATIONAL SCREENING IN PATIENTS WITH BUDD-CHIARI SYNDROME

Background: The diagnosis of underlying myeloproliferative neoplasms (MPNs) is often problematic in patients with Budd Chiari syndrome (BCS). A clonal mutation in JAK2 tyrosine kinase (JAK2V617F) occurs in a high proportion of patients with MPNs and is of use in the characterization of occult MPNs in BCS. Aim of the work: Detection of JAK2 V617F mutation in patients with BCS and its value in detection of occult MPNs. Patients and Methods: This study was carried out on fifty seven newly diagnosed Budd Chiari syndrome patientswho were attending tropical department in Ain Shams University Hospitals during the period from July 2017 to July 2018.Detection of JAK2V617F mutation was done by real time polymerase chain reaction. Results: Out of the studied 57 BCS patients, JAK2 V617F mutation was detected in 12 patients (21.1%) {10 (83.3%) were heterozygous and 2(16.7%) were homozygous for mutation}, while 45 patients (78.9%) were negative.On comparing JAK2 V617F positive and negative groups, there was a highly statistically significant relation regarding MPNs diagnosis, where all JAK2 V617F positive patients were diagnosed as MPNs of whom 7 (58.3%) had overtpresentation and 5(41.7%) had occult presentation, while in JAK2 V617F negative patients only 2 were diagnosed overt MPNs (p=0.001). Conclusion: In conclusion the JAK2 V617F mutation is an acquired mutation that can be used for diagnosis of latent MPNs presenting with thrombotic events, thus it is recommended to include JAK2 V617F gene analysis in the research panel for BCS patients.

Preliminary application of three-dimensional visualization in interventional treatment of Budd-Chiari syndrome

Objective To analyze the application of three-dimensional visualization in interventional treatment of Budd-Chiari syndrome (BCS) with the hepatic vein occlusion type. Methods A retrospective study was conducted on 15 patients with BCS of the hepatic vein occlusion type who were admitted to the Affiliated Hospital of Xuzhou Medical University from January 2017 to June 2018. The study included 8 males and 7 females with a mean age of (36.7±14.4) years. All patients were examined with MRI enhanced scanning and the original MRI data of the DICOM format were extracted. The digital data were then extracted and reconstructed by the Mimics software to obtain a three-dimensional visualization model. The hepatic vein was recanalized under the guidance of the three-dimensional visualization model. Results The three-dimensional visualization model was successfully constructed. Of all the 15 three-dimensional visualization models, right hepatic vein ostial stenosis occurred in 4 patients and occlusion in 11 patients. The middle hepatic vein and the left hepatic vein formed a common trunk in 15 patients, and ostial stenosis and occlusion of the common trunk occurred in 3 and 12 patients, respectively. Communicating branches between the right and middle hepatic veins occurred in 6 patients. Accessory hepatic veins occurred in 3 of 15 patients, and the accessory hepatic vein ostium was stenosed in 3 patients. Communicating branches between the accessory hepatic vein and the right hepatic vein occurred in 2 patients, and communicating branches between the accessory hepatic vein and the middle hepatic vein occurred in 1 patient. All patients were treated successfully with interventional treatment without any complications. One, 2, and 3 hepatic veins were recanalized in 7, 5 and 3 patients, respectively. Conclusion The three-dimensional visualization was valuable in displaying the location, pattern and collateral vessels of the hepatic veins in BCS patients with the hepatic vein occlusion type. It was helpful for accurate preoperative assessment and to make individualized interventional treatment plans. Key words: Budd-Chiari syndrome; Hepatic vein; Imaging, three-dimensional; Interventional treatment