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.

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.

Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis:A case report and review of the literature

BACKGROUND Budd-Chiari syndrome(BCS)is a challenging indication for liver transplantation(LT)due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.

Liver cirrhosis in hepatic vena cava syndrome(or membranous obstruction of inferior vena cava)

Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by high incidence of liver cirrhosis(LC) and moderate to high incidence of hepatocellular carcinoma(HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome(BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction,and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations(AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, portosystemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.

A Case Report and a Descriptive Retrospective Study of Cases of Inferior Vena Cava Thrombosis Diagnosed over a 10-Year Period in a Third Level Hospital

Inferior vena cava thrombosis is an under-recognized entity associated with significant morbidity and mortality. This is the reason why, although the diagnosis is challenging, a high index of suspicion is required. Regarding this condition, we present the case of a 63-year-old man who had repeatedly visited the emergency room suffering from abdominal and back pain and painful lower limb edema. After several tests, including magnetic resonance imaging (MRI), he was diagnosed to have agenesis of left renal vein and inferior vena cava thrombosis, from hypercoagulable state secondary to Antiphospholipid Syndrome. He had anticoagulation treatment with low-molecular-weight heparin with good subsequent evolution. This study sets out a descriptive retrospective study of fifty cases of inferior vena cava thrombosis diagnosed in a third-level hospital in the north of Spain over a ten-year period (2010-2018). The aim of this article is to identify the epidemiology, predisposing factors and symptoms that characterize this entity, in order to be able to achieve an early diagnosis that allows us to initiate immediate treatment, minimizing acute and chronic complications of this disease.

Budd-Chiari syndrome secondary to caval recurrence of renal cell carcinoma

BACKGROUND:Renal cell carcinoma(RCC)involves the inferior vena cava(IVC)in a minority of patients.Less commonly,it presents with Budd-Chiari syndrome.If untreated, the condition progresses towards liver failure and death.METHOD:We report a case of Budd-Chiari syndrome due to infiltration of the IVC and right atrium by recurrence of RCC 7 years after successful treatment by primary resection.RESULTS:Surgery was performed with a combined abdominal and thoracic approach with cardio-pulmonary by-pass and cardioplegia.The tumor was removed and a cadaveric iliac vein graft used to re-establish venous continuity between the right atrium and hepatic veins.CONCLUSIONS:Although it is a complex and high-risk procedure,aggressive surgery performed by an experienced team with liver transplant and cardiothoracic skills may enable resection of apparently advanced caval tumors.The case is discussed in the light of the current literature.

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.

Redefining Budd-Chiari syndrome:A systematic review

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.

Acute iatrogenic Budd-Chiari syndrome following hepatectomy for hepatolithiasis:A report of two cases

Budd-Chiari syndrome(BCS)is defined as hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava(IVC)and the right atrium,regardless of the cause of obstruction.We present two cases of acute iatrogenic BCS and our clinical management of these cases.The first case was a 43-year-old woman who developed acute BCS following the implantation of an IVC stent for the correction of stenosis in the IVC after hepatectomy for hepatolithiasis.The second case was a61-year-old woman with complete obstruction of the outflow of hepatic veins during bilateral hepatectomy for hepatolithiasis.Acute iatrogenic BCS should be con-sidered a rare complication following hepatectomy for hepatolithiasis.Awareness of potential hepatic outflow obstructions and timely management are critical to avoid poor outcomes when performing hepatectomy for hepatolithiasis.

Budd-Chiari syndrome associated with liver cirrhosis: A case report

BACKGROUND Budd-Chiari syndrome(BCS)is a rare heterogeneous liver disease characterized by obstruction of the hepatic venous outflow tract.The incidence of BCS is so low that it is difficult to detect in general practice and difficult to include within the scope of routine diagnosis.The clinical manifestations of BCS are not specific;hence,BCS tends to be misdiagnosed.CASE SUMMARY We report the case of a 33-year-old Chinese woman who presented with progressive distension in the upper abdomen.She was initially misdiagnosed with liver cirrhosis(LC)due to abnormalities on an upper abdominal computed tomography scan.Although she was taking standard anti-cirrhosis therapy,her symptoms did not improve.Magnetic resonance imaging showed caudate lobe hypertrophy;and dilated lumbar and hemiazygos veins.Venography revealed membranous obstruction of the inferior vena cava owing to congenital vascular malformation.A definitive diagnosis of BCS was made.Balloon angioplasty was performed to recanalize the obstructed inferior vena cava and the patient’s symptoms were completely resolved.CONCLUSIONBCS lacks specific clinical features and can eventually lead to LC. Clinicians andradiologists must carefully differentiate BCS from LC. Correct diagnosis andtimely treatment are vital to the patient's health.

To restrict indication for stenting of the inferior vena cava and liver transplantation in patients with Budd-Chiari syndrome

The Budd-Chiari syndrome(BCS)used to be a very rare disorder and lacked a means of relieving.However,various shuntings and radical procedures have emerged in the last three decades with quite encouraging outcomes.Recent minimally invasive therapies,such as stenting of the inferior vena cava(IVC),are simple and easy to handle.However,it has been realized that the IVC stent may compromise the hepatic veins and cause catastrophic consequences,thus the indication for IVC stenting should be reevaluated and especially not to be overused.Instead,it should be applied by percutaneous transangiography(PTA)only,which may be repeated when necessary.The BCS in China predominantly belongs to the IVC type rather than the intrahepatic type in Western;the outcome from its conventional therapy,at least for the moment,is better than that of liver transplantation.It is thus suggested that,before liver transplantation is decided,the conventional means for managing BCS be considered.The final suggestion is to restrict the indication both for stenting of the IVC and liver transplantation.In this paper,ten examples are given with figures.

分期递增式球囊扩张术治疗布加综合征合并下腔静脉血栓的临床疗效及安全性分析

目的探讨分期递增式球囊扩张术治疗布加综合征(BCS)合并下腔静脉血栓的临床疗效及安全性。方法62例BCS合并下腔静脉血栓患者,均使用分期递增式球囊扩张术进行治疗。分析治疗结果(小球囊预开通成功率、血栓溶解率、并发症发生情况及死亡情况);比较手术前后实验室指标[白细胞计数(WBC)、红细胞计数(RBC)、血红蛋白(Hb)、白蛋白(ALB)、血小板计数(PLT)、谷丙转氨酶(ALT)、谷草转氨酶(AST)、碱性磷酸酶(ALP)、直接胆红素(DBil)、总胆固醇(TC)、高密度脂蛋白胆固醇(HDL-C)]。结果62例患者中,小球囊预开通成功率为61例(98.39%),有1例因下腔静脉长段闭塞而反复扩张无法开通;其中,新鲜血栓25例,均行经导管溶栓后清除,其余为陈旧性血栓,在1个月内完全溶解率为93.44%(57/61);所有患者围术期均未发生无症状性肺栓塞、死亡病例。患者术后PLT(131.65±37.02)×10^(9)/L、HDL-C(1.15±0.21)mmol/L均高于术前的(108.94±46.36)×10^(9)/L、(0.93±0.28)mmol/L,ALP(88.19±40.63)U/L、DBil(21.02±7.59)μmol/L均低于术前的(111.57±57.48)U/L、(24.89±9.98)μmol/L(P<0.05);手术前后WBC、RBC、Hb、ALB、ALT、AST、TC水平相比无明显差异(P>0.05)。结论在BCS合并下腔静脉血栓患者的治疗中,分期递增式球囊扩张术的临床疗效显著,手术成功率及血栓溶解率高,并发症发生率低,能有效调节PLT、HDL-C等水平,对改善预后有积极意义。

Budd-Chiari综合征:预开通治疗下腔静脉内陈旧性血栓

目的探讨预开通技术治疗Budd-Chiari综合征(BCS)合并下腔静脉内陈旧性血栓的临床应用。方法收集2006年12月-2009年8月采用预开通技术治疗的BCS合并下腔静脉内陈旧性血栓9例,先行下腔静脉造影,而后使用直径12～16mm的球囊导管预扩张下腔静脉以恢复下腔静脉正向血流;术后给予抗凝、溶栓治疗促进血栓溶解;血栓溶解完全后使用直径25mm/30mm的球囊导管扩张下腔静脉以充分开通下腔静脉。术后采用彩色多普勒超声探查血栓溶解情况及下腔静脉通畅情况。结果 9例BCS合并下腔静脉内陈旧性血栓患者均为下腔静脉膜性阻塞,预开通下腔静脉后,除1例因再次阻塞下腔静脉而改行可回收支架治疗外,余8例患者血栓逐渐溶解消失,未出现肺栓塞症状,成功实施下腔静脉球囊扩张成形。术后随访除1例下腔静脉再次阻塞外,余8例下腔静脉血流通畅,无血栓再次形成。结论预开通技术治疗BCS合并下腔静脉内陈旧性血栓,安全、有效。

长期口服利伐沙班联合介入治疗Budd-Chiari综合征合并下腔静脉陈旧性血栓1例

BCS合并IVC血栓 A.IVC造影+置管溶栓术前MR延迟期增强扫描冠状位图像;B.IVC造影+置管溶栓术中DSA,溶栓效果不佳;C.IVC造影+置管溶栓术后口服利伐沙班6个月,复查MR增强扫描冠状位图像;D.口服利伐沙班6个月后IVC血管成形术中DSA患者男,47岁,因“反复双下肢水肿伴色素沉着5年余,加重1个月”入院;既往无肝病史及长期饮酒史。查体:肝肋下3 cm,脾肋下4 cm;胸腹壁可见纵行曲张静脉,血流方向由下至上,双下肢及阴囊肿胀,双下肢色素沉积。实验室检查:血清总胆红素12.0μmol/L,白蛋白26.9 g/L,凝血酶原时间11.30 s。肝功能Child-Pugh分级为B级。MR:下腔静脉(inferior vena cava,IVC)近膈面处可见膜性带孔狭窄,IVC肾静脉开口以上水平可见血栓形成,约4.0 cm×2.0 cm×1.5 cm(图1A),奇静脉、副奇静脉等IVC侧支血管开放、扩张,同时可见肝叶比例失调,脾脏体积增大及少量腹腔积液。

Etiology,treatment,and classification of Budd-Chiari syndrome

Since Professor WANG Zhong-gao＇s creative work on thesystemic treatment of the Budd-Chiari syndrome（BCS）, this debilitating disease has been more and more widely recognized in China. Several large-scale studies of surgery or intervention strategies for treating BCS have been reported. However, much controversy still remains regarding many aspects of this disease, including its etiology, treatment, and classification. This review explores these controversies with emphasis on areas that merit further study.

布加综合征:搅拌溶栓治疗下腔静脉内新鲜血栓形成

目的探讨搅拌溶栓技术在布加综合征合并下腔静脉内新鲜血栓形成的临床应用。方法收集2004年8月-2009年3月布加综合征合并下腔静脉内新鲜血栓形成5例,首先经股静脉途径行下腔静脉造影,而后开通下腔静脉,再采用搅拌溶栓技术溶解血栓,直至血栓完全消失,最后行腔内球囊扩张血管成形术。术后采用多普勒超声随访下腔静脉通畅情况。结果5例布加综合征合并下腔静脉内新鲜血栓形成患者均为下腔静脉膜性阻塞,采用搅拌溶栓后血栓均完全消失,球囊扩张血管成形术后均未出现肺栓塞症状。术后随访下腔静脉血流通畅,无血栓再次形成,无一例下腔静脉再阻塞。结论搅拌溶栓技术可用于治疗布加综合征合并下腔静脉内新鲜血栓,能一次性完成整体治疗,安全有效。

血管内支架放置治疗下腔静脉闭塞伴血栓形成

为了使下腔静脉闭塞伴血栓形成,在作成形手术时既能有效地保持下腔静脉再通,又能避免肺栓塞发生,我们对3例下腔静脉闭塞伴血栓形成的患者实施了 PTA 和 stent 置入术,均取得满意效果。支架置入后,下腔静脉通畅,无肺栓塞发生。支架具有支撑血管和压迫固定血栓的双重作用。

球囊扩张辅助经导管直接溶栓导致下腔静脉滤器栓塞及其处理

目的探讨球囊扩张辅助经导管直接溶栓(CDT)治疗急性下肢深静脉血栓形成(DVT)过程中下腔静脉滤器(VCF)血栓阻塞的发生率及其处理。方法 2009年9月至201 1年5月收治44例急性下肢DVT患者,作下腔静脉VCF保护下行球囊扩张辅助CDT治疗。结果 27.3%(12/44)患者因VCF拦截大量血栓导致下腔静脉阻塞(完全闭塞8例,部分闭塞4例),经CDT治疗后1 1例完全通畅,1例部分通畅(造影表现为VCF远端充盈缺损)。治疗前后下腔静脉通畅度评分差异有统计学意义(X^2=48.2,P=0.000)。88.64%(39/44)的患者放置可取出VCF,治疗结束后均全部顺利取出。主要并发症包括穿刺点出血13.6%(6/44)、肉限血尿4.5%(2/44)、痰中带血4.5%(2/44),未发生症状性肺栓塞。结论球囊扩张辅助CDT治疗急性下肢DVT可能发生血栓脱落,由此导致的急性VCF血栓阻塞经CDT治疗效果良好。

可回收支架联合搅拌溶栓、球囊扩张治疗布加综合征合并下腔静脉血栓

目的:分析采用可回收支架联合搅拌溶栓、球囊扩张治疗布加综合征(BCS)合并下腔静脉(IVC)血栓形成的效果。方法:针对40例BCS合并IVC血栓形成的患者,对闭塞的IVC采用球囊预扩张部分恢复IVC管腔,以顺利放置可回收支架,然后进行搅拌溶栓。血栓消失后取出支架,必要时采用大球囊扩张。术后采用彩色多普勒超声检查观察IVC通畅程度。结果:所有患者均成功进行可回收支架置入、搅拌溶栓和球囊扩张,支架置入后IVC均立即恢复通畅。1例支架放置6 h后死于急性肺栓塞,1例出院0.4个月后死于冠心病,1例出院0.8个月后死于肝功能衰竭。37例支架放置8~29 d后取出,取出技术成功率为97.3%(36/37),1例因支架断裂改用外科手术取出。34例成功随访,初次通畅率和二次通畅率分别为94.1%(32/34)和97.1%(33/34),术后5 a和10 a生存率分别为89.5%和67.1%。结论:可回收支架联合搅拌溶栓、球囊扩张治疗BCS合并IVC血栓形成安全、有效,远期疗效良好。