Budd-Chiari syndrome and portal vein thrombosis due to essential thrombocytosis

Budd-Chiari syndrome secondary to essential thrombocytosis has been described in a few reports in the English literature. Associated portal vein thrombosis occurs very rarely. Herein, we report a case presented with ascites and finally diagnosed with hepatic and portal vein thrombosis, and essential thrombocytosis. We discussed the therapeutic approaches in the light of pertinent literature.     

Aged Budd-Chiari syndrome attributed to chronic deep venous thrombosis with alcoholic liver cirrhosis

Budd-Chiari syndrome is a rare disease, but there are many known causes. Recent studies showed that it can be an acquired lesion resulting from thrombosis in some elderly patients. We report a 74-year-old man with Budd-Chiari syndrome attributed to chronic deep venous thrombosis and alcoholic liver cirrhosis. When he was aged 45 years, stasis ulcers of the lower extremities appeared. Cerebral infarction and left hemiparesis occurred at age 71. Ultrasonography, venacavography, and three-dimensional-magnetic resonance imaging on admission demonstrated total obstruction of the inferior vena cava with several massive thrombi and developed collateral vessels. Although the etiology of the thrombosis remained obscure, we made some speculative assumptions that chronic disseminated intravascular coagulation (which is frequently observed in cirrhosis) or hereditary coagulopathy could be involved, from his familial history of thrombotic phenomena and a severe deficiency of clotting inhibitors. Despite the high mortality of untreated Budd-Chiari syndrome reported in previous studies, this patient had been alive for about 30 years from the suspected onset. Received: September 14, 1998 / Accepted: April 16, 1999     

Etiology and portal vein thrombosis in Budd-Chiari syndrome

AIM： To research the etiology, portal vein thrombosis and other features of Budd-Chiari syndrome （BCS） patients prospectively.
METHODS： A total of 75 patients （40 female, 35 male） who were diagnosed between January 2002 and July 2004 as having BCS were studied prospectively. Findings from on physical examination, ultrasonography, duplex ultrasonography and venography were analyzed. Hemogram and blood chemistry were studied at the time of diagnosis and on each hospital visit. Bone marrow examination and immune phenotyping were performed by a hematologist when necessary. Protein C, S, antithrombin Ⅲ, activated protein C resistance, and anticardiolipin antibodies, antinuclear antibodies, and anti ds-DNA were studied twice. The presence of ascite, esophageal varices, and portal thrombosis were evaluated at admission and on every visit.
RESULTS： At least one etiological factor was determined in 54 （72%） of the patients. The etiology could not be defined in 21 （28%） patients. One etiological factor was found in 39, 2 factors in 14 and 3 factors in 1 patient. The most common cause was the web （16%）, the second was Hydatid disease （11%）, the third was Behcet’s disease （9%）. Portal vein thrombosis was present in 11 patients and at least one etiology was identified in 9 of them （82%）.
CONCLUSION： Behcet’s disease and hydatid disease are more prominent etiological factors in Turkey than in other countries. Patients with web have an excellent response to treatment without signs of portal veinthrombosis while patients having thrombofilic factors more than one are prone to develop portal vein thrombosis with worse clinical outcome.     

布加综合征与肝癌

布加综合征(Budd-Chiari syndrome,BCS)是由肝静脉流出道阻塞所引起的一组异质性的症候群,阻塞部位可发生于从肝小静脉(hepatic vein,HV)到肝后段下腔静脉(inferior vena cava,IVC)至右心房入口处的任何部位,同时排除由心脏疾病引起的肝静脉流出道阻塞及肝窦阻塞综合征[1].BCS多见于中国、日本、印度、尼泊尔、南非等;在我国,则以黄淮流域为主,如河南、河北、江苏、山东、安徽等地[2-3].     

Clinical spectrum of chronic Budd-Chiari syndrome and surgical relief for 'coarctation' of the inferior vena cava

This report presents the clinical features of 78 cases of the chronic Budd-Chiari syndrome encountered over a period of 13 years. The diagnosis of hepatic venous outflow obstruction was confirmed by venographic studies in all cases. In 20 patients there was hepatic vein occlusion without inferior vena caval (IVC) obstruction (Group A). In 17 patients there was constriction of the IVC above the drainage site of the right hepatic vein which was patent (Group B). In 13 patients there was short segmental obstruction of the hepatic segment of the IVC along with blockage of the hepatic venous orifices (Group C). In 28 patients there was hepatic venous obstruction with long segment involvement of the IVC extending to varying lengths of the infrahepatic segment (Group D). Of particular interest are the operative findings in 12 of 17 patients of Group B of hour glass constriction of the IVC, which can be labelled as ‘coarctation of the IVC'. Dorsal cavoatrial bypass using a polytetra fluoroethylene graft has proved useful in Group B. Interesting histopathological findings of the liver in some of the cases are also described. The possible aetiology of the Group B cases is discussed.     

New approaches to the Budd-Chiari syndrome

Abstract  Recent research has led to an improved understanding of the aetiology of Budd-Chiari syndrome in some patients. Fresh approaches and technical developments within methods of radiological intervention have added more effective options to its treatment. In this editorial we aim to summarize our understanding of the role of new aetiologies and new therapeutic approaches in the Budd-Chiari syndrome.     

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.     

布-加综合征自愈：个例报道

1病例资料患儿女,4岁,宁夏人。一年前因＂过敏性紫癜＂在当地医院治疗,住院期间出现下肢水肿、蛋白尿,诊断为＂紫癜性肾炎＂,给予口服强的松治疗,症状缓解。一个月前无诱因突发腹胀、上腹疼痛、发热。患儿全腹膨隆,腹壁静脉曲张,主要分布于脐水平以上,血流自下至上,腹围67 cm。腹软无压痛,     

Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation

A 22‐year‐old Japanese woman was found to have severe esophageal varices and then suffered from hepatic encephalopathy. She was diagnosed with Budd‐Chiari syndrome (BCS) due to hepatic vein (HV) thrombosis accompanied by portal vein thrombosis without inferior vena cava (IVC) obstruction. Latent myeloproliferative neoplasm (MPN) lacking the JAK2‐V617F mutation was considered to be the underlying disease. Liver transplantation was strikingly effective for treating the clinical symptoms attributable to portal hypertension. Although thrombosis of the internal jugular vein occurred due to thrombocythemia, which manifested after transplantation despite anticoagulation therapy with warfarin, the thrombus immediately disappeared with the addition of aspirin. Neither thrombosis nor BCS has recurred in more than 4 years since the amelioration of the last thrombotic event, and post‐transplant immunosuppression with tacrolimus has not accelerated the progression of MPN. In Japan, IVC obstruction, which was a predominant type of BCS, is suggested to have decreased in incidence with recent improvements in hygiene. The precise diagnosis of BCS and causative underlying diseases should be made with attention to the current trend of the disease spectrum, which fluctuates with environmental sanitation levels. Because the stepwise strategy, including liver transplantation, has been proven effective for patients with pure HV obstruction in Western countries, this strategy should also be validated for utilization in Japan and in developing countries where HV obstruction potentially predominates.     

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.     

经腹翻肝入路非体外循环直视下布-加综合征根治术——单纯开腹手术根治是否可行？

1临床资料1.1病例男,45岁,主因＂腹胀半年,双下肢水肿伴色素沉着5年＂以布-加综合征入院。查体腹部明显膨隆,胸腹壁、背部可见静脉曲张,血流向上,双下肢指凹性水肿,双下肢静脉曲张,膝以下色素沉着。彩超肝左、肝中静脉近心段闭塞,肝右静脉未显示;     

布-加综合征模型的构建及进展

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞引起的门静脉高压伴或不伴有下腔静脉高压的临床综合征,中国人群主要以下腔静脉隔膜形成和(或)阶段性合并肝静脉梗阻为特征。目前人们对布-加综合征的病因和发病机制所知甚少,所做研究多为诊治策略方面的临床研究,针对其发病机制的实验探索相对稀缺,而稳定模型往往是疾病机制研究的必要条件。就目前布-加综合征模型的构建方法、各种模型的特点和研究进展作一综述和分析,以便为该病病因和发病机制的探索提供可靠的实验基础。     

Budd-Chiari syndrome, portal vein and mesenteric vein thrombosis in a patient homozygous for factor V Leiden mutation treated by TIPS and thrombolysis

We present the first case of Budd-Chiari syndrome in association with portal and mesenteric vein thrombosis in a patient homozygous for the factor V Leiden mutation. She was treated by transjugular intrahepatic portosystemic stent (TIPS) placement followed by local thrombolytic therapy. Venous outflow from the liver was established and the thrombi in the portal and mesenteric veins were lysed completely. This therapeutic approach may be used for such patients with this severe thrombotic event, who generally have a poor prognosis.     

Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis

Summary A 22-year-old female with active ulcerative colitis developed massive ascites, hypoalbuminemia, and hepatomegaly compatible with thrombosis of the hepatic veins. The diagnosis of Budd-Chiari syndrome was confirmed by ultrasonography, computed tomography, and by liver biopsy. A search of the literature disclosed only three previous reports of Budd-Chiari syndrome occurring in patients with ulcerative colitis. All patients have been young females with active colitis and no other known risk factor for the development of hepatic vein thrombosis. Our patient, unlike the previously reported patients who died, recovered sufficiently to be discharged from the hospital.     

布-加综合征的进展与展望

本文叙述了布-加综合征的演变,自1845年和1899年由Budd和Chiari报道的不同肝静脉阻塞引起的门脉高压,在病因和流行病学方面开始研究。我国、印度、南非等国的病变大多涉及下腔静脉,今后以改名为肝腔静脉阻塞综合征更为恰当。在病变分类的基础上,出现了一系列治疗方法。在急性期,纤溶疗法是指征。慢性病例采用的方法：各种腔内治疗法、间接减压术、断流术、各种促进侧枝循环的手术、直接减压术,包括各型肠系膜上静脉或下腔静脉或水母头与右心房或颈内静脉之间的转流术、根治性矫治术和肝移植术,后者指征要严格。今后至少要把下腔静脉隔膜性的病因查清,要做到预防和早期诊断,要对复杂性病变的介入和手术方法加以探讨。     

Platelet ultra-structure study in Budd-Chiari syndrome

Abstract: Morphologic characteristics of circulating platelets were studied in 20 patients with primary Budd-Chiari syndrome (BCS) without any known etiology, using transmission electron microscopy (TEM). Significant platelet ultrastructural changes were observed in all the patients (in 10←90% platelets) as compared to 20 normal healthy controls (in up to 4% of their platelets). The prominent changes in the platelets were paucity or absence of alpha granules, hypertrophy of the open canalicular systems (OCS) and clumping and fusion of the granules and other organelles in the centre of platelets. Some other changes observed in platelets were dilated channels of OCS, pseudopodial protrusion of cytoplasm and presence of prominent masses of glycogen particles. Platelets from 20 normal controls processed along with the patients' platelets showed only a few such abnormalities. Most of these changes observed in patients' platelets were akin to the changes observed in platelets undergoing activation. Assay of plasma beta-thromboglobulin showed significantly higher levels in all the patients (p< 0.001) further confirming on-going in vivo platelet activation with morphologic changes most likely reflecting the thrombotic process present in BCS patients.     

Suprahepatic Budd-Chiari syndrome treated with thrombectomy and cavoplasty

The case of a 42-year-old female with Budd-Chiari syndrome (BCS) and lupus anticoagulant is reported. This patient, who had been on chronic anticoagulants for her lupus anticoagulant, presented with abdominal pain, dyspnea on exertion, engorged abdominal wall venous collaterals, and hepatomegaly. A dynamic computerized tomography of the abdomen showed complete suprahepatic inferior vena caval occlusion at the junction with the right atrium. IVC venogram confirmed the diagnosis and also demonstrated patency of the hepatic veins. Free hepatic venous pressure was 25-26 mm Hg. Histopathologic examination of the liver showed marked central venous congestion with significant bridging fibrosis. The total caval occlusion and overt calcification of the clot precluded radiologic angioplasty, and the patient underwent a successful surgical thrombectomy with cavoplasty utilizing an autologous venous patch. Several weeks following surgery, she was free of symptoms and resumed her usual daily activities. Follow-up venography showed a widely opened cava with normal free hepatic vein pressures. Repeat liver biopsy at 6 months showed complete resolution of the hepatic venous congestion and a decrease in the degree of fibrosis.