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budd-chiari syndrome: A case with a combination of hepatic vein and superior vena cava occlusion

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World Journal of Gastroenterology 2005年第24期11卷 3797-3799页

作者： Yoshio Araki Chikara Sakaguchi Izumi Ishizuka Masaya Sasaki Tomoyuki Tsujikawa Shigeki Koyama Akira Furukawa Yoshihide Fujiyama Department of Internal Medicine Department of Radiology

We here report a recent, rare case of budd-chiari syndrome, associated with a combination of hepatic vein and superior vena cava occlusion. A young female, who had been ingood health, was admitted to our hospital because of massive ascites. The patient had used no oral contraceptives. Tests for coagulation disorders, hematological disorders, and antiphospholipid syndrome were all negative. buddchiari syndrome was diagnosed by radiographic examination. The patient was suffering from a combination of hepatic vein and superior vena cava occlusion. In particular, the venous flow returned from the liver mainly through a right accessory hepatic vein, and stenosis was recognized at the orifice of this collateral vein into the vena ***, the patient underwent percutaneous balloon dilatation therapy for this stenosis. After this treatment, the massive ascites was gradually reduced, and she was discharged from our hospital. It has now been one year since discharge, and the patient has been doing well. If deteriorating liver function or intractable ascites occur again, a liver transplantation may be anticipated. This is the first case report of budd-chiari syndrome associated with a superior vena cava occlusion.

关键词： budd-chiari syndrome Hepatic vein occlusion Superior vena cava ocdusion Percutaneous balloon dilatation

budd-chiari syndrome associated with liver cirrhosis: A case report

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World Journal of Clinical Cases 2021年第12期9卷 2937-2943页

作者： Qiao-Bo Ye Qin-Feng Huang Yao-Chang Luo Yi-Lei Wen Zi-Kun Chen Ai-Ling Wei Basic Medical College Chengdu University of Traditional Chinese MedicineChengdu 611137Sichuan ProvinceChina Department of Oncology The First Affiliated HospitalGuangxi University of Chinese MedicineNanning 530023Guangxi Zhuang Autonomous RegionChina Department of Intervention The First Affiliated HospitalGuangxi University of Chinese MedicineNanning 530023Guangxi Zhuang Autonomous RegionChina Department of Pathology The First Affiliated HospitalGuangxi University of Chinese MedicineNanning 530023Guangxi Zhuang Autonomous RegionChina Graduate School Guangxi University of Chinese MedicineNanning 530200Guangxi Zhuang Autonomous RegionChina Department of Liver Disease The First Affiliated HospitalGuangxi University of Chinese MedicineNanning 530023Guangxi Zhuang Autonomous RegionChina

BACKGROUND budd-chiari syndrome(BCS)is a rare heterogeneous liver disease characterized by obstruction of the hepatic venous outflow *** incidence of BCS is so low that it is difficult to detect in general practice and difficult to include within the scope of routine *** clinical manifestations of BCS are not specific;hence,BCS tends to be *** SUMMARY We report the case of a 33-year-old Chinese woman who presented with progressive distension in the upper *** was initially misdiagnosed with liver cirrhosis(LC)due to abnormalities on an upper abdominal computed tomography *** she was taking standard anti-cirrhosis therapy,her symptoms did not *** resonance imaging showed caudate lobe hypertrophy;and dilated lumbar and hemiazygos *** revealed membranous obstruction of the inferior vena cava owing to congenital vascular malformation.A definitive diagnosis of BCS was *** angioplasty was performed to recanalize the obstructed inferior vena cava and the patient’s symptoms were completely *** 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.

关键词： budd-chiari syndrome Liver cirrhosis Venography Balloon angioplasty Inferior vena cava Case report

budd-chiari syndrome associated with coeliac disease:case report and literature review

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Gastroenterology Report 2018年第4期6卷 308-312,I0002页

作者： Khaled Ali Jadallah Enas Walid Sarsak Yara Mohammad Khazaleh Rawan Mohammad Khair Barakat Department of Internal Medicine King Abdullah University HospitalIrbidJordan

We report a case of budd-chiari syndrome occurring in a patient with coeliac disease,who presented with symptoms of increased abdominal girth,right upper quadrant pain and shortness of breath for three weeks prior to *** assessment revealed the presence of moderate ascites,hepatosplenomegaly and right-sided pleural *** diagnostic work-up established a diagnosis of chronic budd-chiari ***,complete screening for pro-thrombotic factors was negative.A review of the literature on this association disclosed only 28 similar cases,with the majority of them describing individuals of North African ***,in the majority of cases no specific thrombotic factor could be identified,suggesting that coeliac disease may play a role in this thrombotic disorder.

关键词： coeliac disease budd-chiari syndrome hepatic vein thrombosis

budd-chiari syndrome:A single-center experience

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World Journal of Gastroenterology 2014年第43期20卷 16236-16244页

作者： Tanya M Pavri Alan Herbst Rajender Reddy Kimberly A Forde Division of Gastroenterology and Hepatology Perelman School of Medicine at the University of PennsylvaniaPhiladelphiaPA 19104United States Center for Clinical Epidemiology and Biostatistics Perelman School of Medicine at the University of PennsylvaniaPhiladelphiaPA 19104United States

AIM: To investigate challenges, risk factors, prognostic indicators, and treatment outcomes associated with budd-chiari syndrome (BCS) at a tertiary care center.

关键词： budd-chiari syndrome Transjugular intrahepatic portosystemic shunt Liver transplantation Anticoagulation Hepatic vein thrombosis

budd-chiari syndrome secondary to caval recurrence of renal cell carcinoma

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Hepatobiliary & Pancreatic Diseases International 2010年第3期9卷 321-324页

作者： Gabriele Marangoni Adrian O'Sullivan Amir Ali Walid Faraj Nigel Heaton Institute of Liver Studies King's College London School of Medicine at King's College Hospital

BACKGROUND:Renal cell carcinoma(RCC)involves the inferior vena cava(IVC)in a minority of *** commonly,it presents with budd-chiari *** untreated, the condition progresses towards liver failure and ***: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 ***:Surgery was performed with a combined abdominal and thoracic approach with cardio-pulmonary by-pass and *** tumor was removed and a cadaveric iliac vein graft used to re-establish venous continuity between the right atrium and hepatic ***: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 *** case is discussed in the light of the current literature.

关键词： inferior vena cava renal cell carcinoma recurrence budd-chiari syndrome

budd-chiari syndrome in children:Challenges and outcome

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World Journal of Hepatology 2023年第11期15卷 1174-1187页

作者： Arghya Samanta Moinak Sen Sarma Rajanikant Yadav Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia Department of Radiology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia

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

关键词： budd-chiari syndrome Radiological endovascular intervention Transjugular intrahepatic porto-systemic shunt Direct intrahepatic porto-systemic shunt Liver stiffness Splenic stiffness Shear-wave elastography

Hepatocellular carcinoma in budd-chiari syndrome:A single center experience with long-term follow-up in South Korea

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World Journal of Gastroenterology 2012年第16期18卷 1946-1952页

作者： Hana Park Jin Young Yoon Kyeong Hye Park Do Young Kim Sang Hoon Ahn Kwang-Hyub Han Chae Yoon Chon Jun Yong Park Department of Internal Medicine Yonsei University College of MedicineSeoul 120-752South Korea Institute of Gastroenterology Yonsei University College of MedicineSeoul 120-752South Korea Liver Cirrhosis Clinical Research Center Seoul 120-752South Korea Brain South Korea 21 Project for Medical Science Seoul 120-752South Korea

AIM:To evaluate long-term clinical course of buddchiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and ***:We analyzed 67 patients with BCS between June 1988 and May *** diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography,computed tomography,magnetic resonance imaging,or *** median follow-up period was 103 ± 156 [interquartile range(IQR)] ***:The median age of the patients was 47 ± 16(IQR) *** diagnosis,54 patients had cirrhosis,25(37.3%) Child-Pugh class A,23(34.3%) Child-Pugh class B,and six(9.0%) patients Child-Pugh class *** the follow-up period,HCC was developed in 17 patients,and the annual incidence of HCC in patients with BCS was 2.8%.Patients in HCC group(n = 17) had higher hepatic venous pressure gradient(HVPG) than those in non-HCC group(n = 50)(21 ± 12 mmHg vs 14 ± 7 mmHg,P = 0.019).The survival rate of BCS patients was 86.2% for 5 years,73.8% for 10 years,and 61.2% for 15 *** patients with BCS and HCC,survival was 79% for 5 years,43.1% for 10 years,and 21.5% for 15 ***:The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South *** HVPG is expected to provide additional information for predicting HCC development in BCS patients.

关键词： budd-chiari syndrome Hepatocellular carcinoma Prognosis

Distant skeletal muscle metastasis from intrahepatic cholangiocarcinoma presenting as budd-chiari syndrome

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World Journal of Gastroenterology 2007年第22期13卷 3141-3143页

作者： Oh Sung Kwon Dae Won Jun Sang Heum Kim Mee Yeon Chung Nam In Kim Moon Hee Song Han Hyo Lee Seung Hwan Kim Yoon Ju Jo Young Sook Park Jong Eun Joo Department of Internal Medicine Eulji University Hospital Seoul Korea Department of Radiology Eulji University Hospital Seoul Korea Department of Pathology Eulji University Hospital Seoul Korea

Intrahepatic cholangiocarcinoma is a malignant neoplasm arising from the biliary epithelium, which frequently invades adjacent organs or metastasizes to other visceral organs such as the lungs, bones, adrenals, and brain. However, distant skeletal muscle metastasis of cholangiocarcinoma has never been described before to the best of our knowledge and, furthermore, budd-chiari syndrome secondary to intrahepatic cholangiocarcinoma is also extremely rare. Here we present the first case overall of distant muscle metastasis from intrahepatic cholangiocarcinoma presenting as budd-chiari syndrome. A 44-year-old man admitted to the hospital with complaints of abdominal distension, edema of both legs, back pain and anorexia of 30 d' duration. Computed tomography and ultrasonography-guided percutaneous muscle biopsy established intrahepatic cholangiocarcinoma with disseminated thrombosis from inferior vena cava to bilateral iliac and femoral veins, and multiple skeletal muscle metastases in bilateral buttock and erector spinal muscle.

关键词： Intrahepatic Cholangiocarcinoma Metastasis Skeletal muscle budd-chiari syndrome

Etiology and portal vein thrombosis in budd-chiari syndrome

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World Journal of Gastroenterology 2008年第18期14卷 2858-2862页

作者： Oguz Uskudar Meral Akdogan Nurgul Sasmaz Sevinc Yilmaz Muharrem Tola Burhan Sahin Turkiye Yuksek htisas Hospital clinics of gastroenterology Ankara 06010 Turkey Turkiye Yuksek htisas Hospital hematology unit Ankara 06010 Turkey Turkiye Yuksek htisas Hospital clinics of radiology Ankara 06010 Turkey

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.

关键词： budd-chiari syndrome Etiology Web Behcet's disease Portal thrombosis