Development of a prognostic scoring system for hepatic vena cava Budd-Chiari syndrome with hepatocellular carcinoma

Background:Hepatocellular carcinoma(HCC)is a serious complication of hepatic vena cava Budd-Chiari syndrome(HVC-BCS)that significantly reduces the survival time of patients.Our study aimed to analyze the prognostic factors influencing the survival of HVC-BCS patients with HCC and to develop a prognostic scoring system.Methods:The clinical and follow-up data of 64 HVC-BCS patients with HCC who received invasive treatment at the First Affiliated Hospital of Zhengzhou University between January 2015 and December 2019 were retrospectively analyzed.Kaplan-Meier curves and log-rank tests were used to analyze the survival curve of patients and the difference in prognoses between the groups.Univariate and multivariate Cox regression analyses were performed to analyze the influence of biochemical,tumor,and etiological characteristics on the total survival time of patients,and a new prognostic scoring system was developed according to the regression coefficients of the independent predictors in the statistical model.The prediction efficiency was evaluated using the time-dependent receiver operating characteristics curve and concordance index.Results:Multivariate analysis showed that serum albumin level<34 g/L[hazard ratio(HR)=4.207,95%confidence interval(CI):1.816-8.932,P=0.001],maximum tumor diameter>7 cm(HR=8.623,95%CI:3.771-19.715,P<0.001),and inferior vena cava stenosis(HR=3.612,95%CI:1.646-7.928,P=0.001)were independent predictors of survival.A prognostic scoring system was developed according to the above-mentioned independent predictors,and patients were classified into grades A,B,C and D.Significant differences in survival were found among the four groups.Conclusions:This study successfully developed a prognostic scoring system for HVC-BCS patients with HCC,which is helpful for clinical evaluation of patient prognosis.

A Budd-Chiari Syndrome Due to C Protein Deficiency: A Case Report at YaoundéGeneral Hospital (Cameroon)

Primary Budd-Chiari syndrome (BCS) is a spontaneously fatal disease characterized by an obstruction of the hepatic venous outflow tract due to thrombosis or a primary disease of the venous wall. The primary form of BCS is extremely rare. This is a disease mainly affecting young adults of both sexes. Clinical manifestations are variable;they can be asymptomatic, acute, or subacute but mostly chronic. Several causes have been identified, such as myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, and inherited thrombotic disorders. Data on primary BCS in Sub-Saharan Africa is rare as most publications available are case reports. In these reports, the causes are unknown with poor prognosis in most cases often leading to patient death. We herein present a case report of a male patient diagnosed with a primary BCS at Yaoundé General Hospital (Cameroon) caused by a Protein C deficiency who presented with ascites decompensating liver cirrhosis. Treatment was based on anticoagulants, diuretics and laxatives administration. Two years after the diagnosis, the patient is alive with clinical and paraclinical improvement.

Fracture of inferior vena cava stent after endovascular treatment for Budd-Chiari syndrome:A case series and literature review

Budd-Chiari syndrome(BCS)is a rare condition characterized by hepatic venous outflow obstruction.Balloon angioplasty,with or without stenting,is the recommended first-line treatment modality in Asian countries.As a supplement to balloon angioplasty,expandable metallic Z-stent deployment can effectively improve long-term inferior vena cava(IVC)patency.Although stent placement is a standard and frequently performed treatment,very few IVC stent-related complications,such as stent fractures,have been reported.Here we present a case series and a comprehensive review of IVC stent fractures in patients with BCS.The most common characteristic of IVC stent fractures is a protrusion of the proximal segment of the IVC stent into the right atrium and its systolic and diastolic movements along with heart rhythms.Accurate stent deployment,large-diameter balloon dilation,patient breath-holding training,preferential selection of a triple stent,and the use of an internal jugular vein approach to stent deployment may ensure precise stent localization and avoid postoperative complications.

Budd-Chiari syndrome in myeloproliferative neoplasms:A review of literature

Myeloproliferative neoplasms(MPNs)are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs.Classical,Philadelphia-negative MPNs,i.e.,polycythemia vera,essential thrombocythemia and primary myelofibrosis,exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites,e.g.,portal,splanchnic or hepatic veins,the placenta or cerebral sinuses.The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury,stasis,elevated leukocyte adhesion,integrins,neutrophil extracellular traps,somatic mutations(e.g.,the V617F point mutation in the JAK2 gene),microparticles,circulating endothelial cells,and other factors,to name a few.Herein,we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs,with a particular focus on its epidemiology,pathogenesis,histopathology,risk factors,classification,clinical presentation,diagnosis,and management.

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.

Selection of treatment modalities for Budd-Chiari Syndrome in China:A preliminary survey of published literature

AIM:To evaluate the frequency of use of various treatment modalities for Budd-Chiari syndrome(BCS)in China by conducting a preliminary survey of relevant literature.METHODS:All papers regarding the treatment of BCS in Chinese patients were identified by searching PubMed,Chinese Scientific and Technological Journal,and China National Knowledge Infrastructure databases.Data regarding the number of BCS patients treated with different treatment modalities over time were collected.The proportions of BCS patients undergoing various treatment modalities were calculated.RESULTS:Overall,300 of 3005 papers initially retrieved were included.These papers included 23352BCS patients treated with different treatment modalities.The treatment modalities include surgery(n=8625),interventional treatment(n=13940),surgery combined with interventional treatment(n=363),medical therapy(n=277),other treatments(n=91),and no treatment(n=56).After 2005,the number of BCS patients treated with surgery was drastically decreased,but the number of BCS patients who underwent interventional treatment was almost maintained.Shunt surgery was the most common type of surgery(n=3610).Liver transplantation was rarely employed(n=2).Balloon angioplasty with or without stenting was the most common type of interventional treatment(n=13747).Transjugular intrahepatic portosystemic shunt was rarely employed(n=81).CONCLUSION:Selection of treatment modalities for BCS might be different between China and Western countries.Further work should be necessary to establish a unanimous therapeutic strategy for BCS in China.

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.

Transjugular intrahepatic portosystemic shunt for severe jaundice in patients with acute Budd-Chiari syndrome

AIM:To evaluate the feasibility of transjugular intrahepatic portosystemic shunt(TIPS)for severe jaundice secondary to acute Budd-Chiari syndrome(BCS).METHODS:From February 2009 to March 2013,37patients with severe jaundice secondary to acute BCS were treated.Sixteen patients without hepatic venule,hepatic veins(HV)obstruction underwent percutaneous angioplasty of the inferior vena cava(IVC)and/or HVs.Twenty-one patients with HV occlusion underwent TIPS.Serum bilirubin,liver function,demographic data and operative data of the two groups of patients were analyzed.RESULTS:Twenty-one patients underwent TIPS and the technical success rate was 100%,with no technical complications.Sixteen patients underwent recanalization of the IVC and/or HVs and the technical success rate was 100%.The mean procedure time for TIPS was 84.0±12.11 min and angioplasty was44.11±5.12 min(P<0.01).The mean portosystemic pressure in the TIPS group decreased significantly from 40.50±4.32 to 16.05±3.50 mm Hg(P<0.01).The mean portosystemic pressure gradient decreased significantly from 33.60±2.62 to 7.30±2.21 mm Hg(P<0.01).At 8 wk after the procedures,in the TIPS group,total bilirubin(TBIL)decreased significantly from 266.24±122.03 before surgery to 40.11±3.52μmol/L(P<0.01)and direct bilirubin(DBIL)decreased significantly from 194.22±69.82μmol/L to 29.82±3.10μmol/L(P<0.01).In the angioplasty group,bilirubin returned to the normal range,with TBIL decreased significantly from 258.22±72.71μmol/L to 13.33±3.54μmol/L(P<0.01)and DBIL from175.08±39.27 to 4.03±1.74μmol/L(P<0.01).Liver function improved faster than TBIL.After 2 wk,in the TIPS group,alanine aminotransferase(ALT)decreased significantly from 50.33±40.61 U/L to 28.67±7.02U/L(P<0.01)and aspartate aminotransferase(AST)from 49.46±34.33 U/L to 26.89±8.68 U/L(P<0.01).In the angioplasty group,ALT decreased significantly from 51.56±27.90 to 14.22±2.59μmol/L(P<0.01)and AST from 60.66±39.89μmol/L to 8.18±1.89μmol/L(P<0.01).After mean follow-up of 12.6 mo,there was no recurrence of jaundice in either group.CONCLUSION:Severe jaundice is not a contraindication for TIPS in patients with acute BCS and TIPS is appropriate for severe jaundice due to BCS.

Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center

AIM To investigate 30-year treatment outcomes associated with Budd-Chiari syndrome(BCS) at a tertiary hospital in China.METHODS A total of 256 patients diagnosed with primary BCS at our tertiary hospital between November 1983 and September 2013 were followed and retrospectively studied. Cumulative survival rates and cumulative mortality rates of major causes were calculated by Kaplan-Meier analysis, and the independent predictors of survival were identified using a Cox regression model. RESULTS Thirty-four patients were untreated; however, 222 patients were treated by medicine, surgery, or interventional radiology. Forty-four patients were lost to follow-up; however, 212 patients were followed, 67 of whom died. The symptom remission rates of treated and untreated patients were 81.1%(107/132) and 46.2%(6/13), respectively(P = 0.009). The cumulative 1-, 5-, 10-, 20-, and 30-year survival rates of the treated patients were 93.5%, 81.6%, 75.2%, 64.7%, and 58.2%, respectively; however, the 1-, 5-, 10-, 20-, and 30-year survival rates of the untreated patients were 70.8%, 70.8%, 53.1%, 0%, and unavailable, respectively(P = 0.007). Independent predictors of survival for treated patients were gastroesophageal variceal bleeding(HR = 3.043, 95%CI: 1.363-6.791, P = 0.007) and restenosis(HR = 4.610, 95%CI: 1.916-11.091, P = 0.001). The cumulative 1-, 5-, 10-, 20-, and 30-year mortality rates for hepatocellular carcinoma were 0%, 2.6%, 3.5%, 8%, and 17.4%, respectively.CONCLUSION Long-term survival is satisfactory for treated Chinese patients with BCS. Hepatocellular carcinoma is a chronic complication and should be monitored with long-term follow-up.

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.

Budd-Chiari syndrome management:Lights and shadows

Budd-Chiari syndrome (BCS) is a rare disease whose management should follow a step by step strategy.Anticoagulation and medical therapy should be the first line treatment.Revascularization or TIPS are indicated in case of no response to medical therapy.OLT should be indicated as a rescue therapy and anticoagulation be started soon after OLT.However,no clear indica-tion can actually be given about the timing of different treatments.Moreover,there is some concern about treatment of some subgroup of patients,especially regarding the risk of recurrence after liver transplanta-tion.The topic of this paper is to critically review the actual knowledge of BCS management.

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.

Imaging and interventions in Budd-Chiari syndrome

Budd-Chiari syndrome(BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension.Clinically,two forms of disease(acute and chronic) are recognized.Mostly the patients present with ascites,hepatomegaly,and portal hypertension.In acute disease the liver is enlarged with thrombosed hepatic veins(HV) and ascites,whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava(IVC),or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC.Due to advances in radiological interventional techniques and hardware,there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation.The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.

Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins

BACKGROUND: Collateralized intraand extra-hepatic routes in patients with Budd-Chiari syndrome (BCS) were important. This study aimed to investigate the feasibility and clinical outcomes of the staged management of BCS based on the degree of compensation provided by intraor extra-hepatic collateral circulations. METHODS: A total of 103 adult patients with BCS caused by co-obstruction of the inferior vena cava (IVC) and main hepatic veins (MHVs) between March 2001 and October 2009 were enrolled in this study. Based on the pathological classification and degree of hemodynamic compensation by collateral circulations, treatment priority for IVC hypertension was determined in the first-stage treatment. Patients were deemed eligible for second-stage treatment when the first-stage treatment failed to relieve. RESULTS: Imaging results revealed that most patients had collateral circulations to different extents. Based on the degree of compensation provided by these collateral circulations, 74 patients underwent single-stage treatment for IVC hypertension, i.e., radiologic intervention (RI) for 61 patients and surgical procedures (SPs) for 13. One patient was treated for portal hypertension. Twenty-nine patients underwent second-stage treatment (25 underwent RI and SP, and 4 only SP). The general morbidity and mortality after all procedures were 8.3% and 1.5%, respectively. After a median follow-up of 35 months, 4 patients underwent second-stage treatment and 7 underwent recanalization of the IVC/MHVs. Two patients died of hepatocellular carcinoma and 1 died of graft obstruction. CONCLUSION: Staged management produces excellent outcomes for patients with BCS caused by co-obstruction of the IVC and MHVs.

Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt

AIM:To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre.METHODS:Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men,median age 40 years (range 17-66 years)].Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals.Management consisted of tests for underlying haematological,endocrinological,or hypercoagulative disorders parallel to initiation of specific treatment of BCS.RESULTS:BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders.Fortythree percents had symptoms for less than one week with ascites as the most prevalent finding.Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics.The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo),and none required subsequent liver transplantation.Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics.A total of 14 TIPS revisions were needed,mostly of uncovered stents.Two died during follow-up:One non-TIPS patient worsened after 6 mo and died in relation to transplantation,and one TIPS patient died 4 years after the TIPS-procedure,unrelated to BCS.CONCLUSION:In our BCS cohort TIPS-treated patients have near-complete survival,reduced need for diuretics and compared to historical data a reduced need for liver transplantation.

The application and efficacy of stent place for Budd-Chiari syndrome

Objective To evaluate the application value and efficacy on stent place for Budd-Chiari syndrome(BCS). Methods From January 1990 to May 2017, 2228 patients with BCS were admitted to our institution. The mean age was 43.3 years. Stents were placed in inferior vena cava(IVC), hepatic vein(HV), or both after balloon dilation. During follow-up period, the patency of stent was evaluated by ultrasound regularly and the clinic sign was surveyed by letter, telephone or clinic visit. The restenosis of stent were treated with balloon dilatation and thrombolysis to restore the its function. Results IVC type was diagnosed in 1492 cases, HV type in 510 cases, and mixed type in 226 cases. Eighteen patients aborted treatment because of economic reasons, advanced liver cancer, severe scoliosis, or both bilateral iliac veins and total IVC occlusion. Among the other 2210 cases who underwent endovascular therapy, stents were implanted into IVC in 339 cases, HV in 97 cases, mixed type in 64 cases. The rate of restenosis in IVC stent was 11.50%(39/339). After repeat angioplasty, the long-term patency rate reached to 98.12%. The incidence of HV occlusion caused by IVC stent was 12.09%(n = 41). Restenosis occurred in 47 cases(48.45%) after HV stent placement. However, the 5-year patency rate was 91.75%(89/97) after repeat dilatation and stent re-implantation. The incidence of IVC obstruction caused by HV was 3.33%(3 cases). Conclusion IVC stent placement appears to be an effective treatment for the cases of IVC segmental occlusion, and at the same time, the stent has the dual role of compression and fixation of thrombus and support of lumen. The HV and accessory hepatic vein obstruction could happen when the IVC stent crossed these veins ostium. The incidence of the stent restenosis in the HV was higher than that in the IVC.

Budd-Chiari syndrome:A single-center experience

AIM:To investigate challenges,risk factors,prognostic indicators,and treatment outcomes associated with Budd-Chiari syndrome(BCS)at a tertiary care center.METHODS:A retrospective cohort study was conducted at the University of Pennsylvania in patients with a diagnosis of BCS or hepatic vein thrombosis.All patients receiving care at the University of Pennsylvania,and who had at least 2 clinical encounters in the University of Pennsylvania Health system from January1,2008 to September 10,2013 were eligible for study inclusion.Data were extracted from the electronic medical record of each patient,and recorded in a secure Research Electronic Data Capture database.Logistic regression analyses were applied to identify predictors of outcome of liver transplant(LT)or death.RESULTS:Between January 1,2008 and September10,2013,forty-seven patients were identified.Median age was 42.4 years.Thirty-one(66.0%)were women.A majority were Caucasian(68.1%).At diagnosis,43(91.5%)patients had ascites,27(57.4%)patients had a hematologic disorder associated with a hypercoagulable state and 26(55.3%)had cirrhosis.Forty(85.1%)patients were on anticoagulation(AC),30(63.8%)of whom were maintained on warfarin.Two patients(4.3%)underwent thrombolytic therapy.A transjugular intrahepatic portosystemic shunt(TIPS)was placed in21(44.7%)patients,19(90.5%)of whom were also on AC.Twenty-one(44.7%)received AC alone.Over a median of 974 d,8(17.0%)patients received LT,and10(21.3%)died.The median time from listing to death was 26 mo[interquartile range(IQR)=16,65)].TIPS with AC was utilized more frequently in younger patients(P=0.02).Age,cirrhosis and chronic kidney disease(CKD)were significant predictors of LT or death.CONCLUSION:AC alone was employed as frequently as TIPS with AC,though the latter was used more frequently in younger patients with polycythemia vera.There were no significant differences in treatment outcome regardless of the therapeutic intervention employed.Significant predictors of poor prognosis included age,cirrhosis and CKD.

Budd-Chiari syndrome in a patient with ulcerative colitis and no inherited coagulopathy

We report a case of 27 year old female patient who was admitted to the hospital with an acute flare up of ulcerative colitis.The patient presented with complaints of persistent abdominal pain and bloody diarrhea despite aggressive therapy for her ulcerative colitis.A CT scan of the abdomen on admission revealed hepatic vein thrombosis,suggesting a diagnosis of Budd-Chiari syndrome.Significantly,an associated thrombosis of the inferior mesenteric vein was also detected.Based on imaging data and clinical assessment,the patient was started on anticoagulation therapy and an extensive work-up for hypercoagulability was initiated.Up to the time of publication,no significant findings suggesting this patient has an underlying coagulation disorder have been found.Based on our search of PUBMED,this report is one of only five reported adult cases of Budd-Chiari Syndrome associated with ulcerative colitis in the English literature in living patients without evidence of a co-existing coagulation disorder.This case highlights the potential for thrombosis at unusual sites in ulcerative colitis patients even in the absence of classical coagulation abnormalities.In addition to the case presented,we provide a brief review of previously reported cases of Budd-Chiari Syndrome occurring in patients with inflammatory bowel disease.

Large regenerative nodules in a patient with Budd-Chiari syndrome after TIPS positioning while on the liver transplantation list diagnosed by Gd-EOB-DTPA MRI

BACKGROUND:Large regenerative nodules (LRNs) are hyperplastic benign nodules most commonly associated with Budd-Chiari syndrome (BCS),caused by outflow obstruction of the hepatic veins or vena cava.To our knowledge,no cases of LRNs arising in BCS after transjugular intrahepatic portosystemic shunt (TIPS) positioning and detected by GdEOB-DTPA MRI have been reported in the literature.METHODS:A 58-year-old woman with BCS,on the liver transplantation (LT) list,underwent a follow-up enhanced MRI.Two years earlier,a TIPS had been placed.In 2008,recurrent hepaticoencephalopathy resistant to medical treatment fulfilled the LT criteria for BCS treated with TIPS and the patient was therefore added to the LT list.CT performed before TIPS had not detected any hepatic lesions.CT performed six months after TIPS showed its complete patency but documented two indeterminate hypervascular liver lesions.RESULTS:MRI performed with Gd-EOB-DTPA revealed additional hypervascular lesions with uptake and retention of the medium in the hepatobiliary phase,thus reflecting a benign behavior of hepatocellular composition.These MRI features were related to LRNs as confirmed by histopathologic analysis.CONCLUSIONS:Gd-EOB-DTPA-enhanced MRI is potentially superior to standard imaging using gadolinium chelates or spiral CT,especially for the differential diagnosis of hypervascular lesions.Gd-EOB-DTPA MRI may become the imaging method of choice for evaluating LT list patients with BCS after TIPS placement.