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Budd chiari syndrome liver transplantation

Susana Nobre, Rajeev Khanna, Natalie Bab, Eirini Kyrana, Sue Height, John Karani, Pauline Kane, Nigel Heaton, Anil Dhawan
Primary Budd-Chiari syndrome (BCS) is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary BCS presenting from Jan 2001 to Nov 2015 to our hospital. Seven children were identified. Their presentation was mostly chronic. All had predisposing factors for thrombosis and were started on anticoagulation. Radiological interventions (2 transjugular intrahepatic portosystemic shunts (TIPSS) and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2 and 1 patients, respectively; one child underwent bone-marrow transplantation following TIPSS, and one child was managed only medically...
March 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
Syed Muhammad Raza, Saima Zainab, Ali Reza Shamsaeefar, Saman Nikeghbalian, Seyed Ali Malek Hosseini
OBJECTIVES: Budd-Chiari syndrome can lead to fulminant hepatic failure and cirrhosis. The treatment depends on the severity of disease. Liver transplant is a successful treatment option for those with advanced-stage disease. MATERIALS AND METHODS: In this retrospective study, we analyzed all liver transplants conducted for Budd-Chiari syndrome at the organ transplant unit of Shiraz University of Medical Sciences, Iran, from 1993 to January 2016. Overall, 3201 liver transplant procedures were performed...
February 7, 2017: Experimental and Clinical Transplantation
Konrad Kobryń, Rafał Paluszkiewicz, Krzysztof Dudek, Urszula Ołdakowska-Jedynak, Michał Korba, Joanna Raszeja-Wyszomirska, Piotr Remiszewski, Michał Grąt, Piotr Milkiewicz, Waldemar Patkowski, Marek Krawczyk
BACKGROUND: This report presents a case of a 57- year old female with advanced Hepatic Alveolar Echinococcosis causing a secondary Budd-Chiari Syndrome due to infiltration of the suprahepatic inferior vena cava treated successfully by liver transplantation. CASE PRESENTATION: A temporary veno-venous bypass was introduced, but a typical end to end cavo-caval anastomosis wasn't possible in this case. In order to access a disease free part of the inferior vena cava, an oval window of the diaphragm was excised, providing communication between the peritoneum and pericardium...
January 13, 2017: BMC Surgery
Francisco Pereira da Silva, Paulo Donato, Filipe Caseiro-Alves
We report the approach to a 29 year old patient presenting with the diagnosis of a Budd-Chiari Syndrome, with clinical deterioration after initial anticoagulation treatment. The patient was proposed to endovascular treatment. Through intra-hepatic shunting seen at angiography, and from a left to right liver lobe, a guide-wire was passed retrogadely and allowed sufficient support to perform hepatic vein angioplasty and stenting. The patient responded favourably and liver transplant was avoided.
2016: European Journal of Radiology Open
H Pahari, R J Chaudhary, S Thiagarajan, V Raut, R Babu, P Bhangui, S Goja, A Rastogi, V Vohra, A S Soin
BACKGROUND: Living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) has been reported with <10 inferior vena cava (IVC) replacements with vascular/synthetic graft. The goal of this study was to review outcomes of LDLT for BCS at our center, with an emphasis on surgical techniques and postoperative anticoagulation therapy. METHODS: Between October 2011 and December 2015, a total of 1027 LDLTs were performed. Nine of these patients had BCS. We analyzed their etiologies, operative details, postoperative complications, and outcomes...
October 2016: Transplantation Proceedings
Cengiz Ara, Sami Akbulut, Volkan Ince, Serdar Karakas, Adil Baskiran, Sezai Yilmaz
BACKGROUND: The aim of the study was to report the detailed surgical techniques of living donor liver transplantation (LDLT) in patients with Budd-Chiari syndrome (BCS). METHODS: Demographic and surgical techniques characteristics of 39 patients with BCS who underwent LDLT were retrospectively reviewed. Thirty-two of them had native vena cava inferior (VCI) preservation and 6 had retrohepatic VCI resection with venous continuity established by cryopreserved VCI (n = 4) or aortic graft (n = 2)...
October 2016: Medicine (Baltimore)
L Liu, X-S Qi, Y Zhao, H Chen, X-C Meng, G-H Han
Budd-Chiari syndrome (BCS) is a rare disorder caused by hepatic venous outflow obstruction with a wide spectrum of etiologies. Clinical manifestations are so heterogeneous that the diagnosis should be considered in any patients with acute or chronic liver disease. Therapeutic modalities for BCS have improved dramatically during the last few years. The concept of a step-wise treatment strategy has been established, including anticoagulation, thrombolysis, percutaneous recanalization, transjugular intrahepatic portosystemic shunt, surgery and liver transplantation...
July 2016: European Review for Medical and Pharmacological Sciences
Masaaki Hidaka, Susumu Eguchi
Budd-Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Europe and North America countries. Multistep management is required for such patients. Interventional revascularization and transjugular intrahepatic portosystemic shunt procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC...
June 1, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
Saima Karim, Victor Lucas, Anil Verma, Nigel Girgrah, Stephen Ramee, Fausto Castriota, Antonio Micari, Giuseppe Roscitano, Francesco Spinelli, Sameer Gafoor, Abdul Haseeb, Asad Khan, Jennifer Franke, Predrag Matic, Markus Reinartz, Stefan Bertog, Laura Vaskelyte, Ilona Hofmann, Horst Sievert
No abstract text is available yet for this article.
May 17, 2016: EuroIntervention
Y M Shao, W T Wang, Aji Tuerganaili, T Li, T M Jiang, R Q Zhang, Y B He, M Guo, L N Yan, H Wen
OBJECTIVE: To report the first clinical experience of living donor liver transplantation(LDLT) in patient with complicated hepatic cystic echinococcosis in China. METHODS: First Affiliated Hospital of Xinjiang Medical University successfully implemented LDLT in the treatment of one patient with complicated hepatic cystic echinococcosis after four times liver surgery and secondary congestive cirrhosis on May 9, 2015. The clinical data of the patient has been retrospectively analyzed...
April 26, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Gil-Chun Park, Gi-Won Song, Deok-Bog Moon, Sung-Gyu Lee
Living donor liver transplantation (LDLT) has become an inevitable procedure in Asia due to its shortage of deceased donor under the influence of the religion and native cultures. Through a broad variety of experience, LDLT has been evolved and extended its indication. Although there have been many surgical and ethical efforts to prevent donor risk, concerns of donor's safety still are remaining questions due to its strict selection criteria. Therefore, dual grafts LDLT or ABO incompatible (ABO-I) LDLT may be effective means in its application and safety aspect...
April 2016: Hepatobiliary Surgery and Nutrition
Sameer Zaman, Stefan Wiebe, William Bernal, Julia Wendon, Julia Czuprynska, Georg Auzinger
BACKGROUND: Budd-Chiari syndrome (BCS) is a rare, potentially fatal disease characterized by hepatic venous outflow tract obstruction. Heparin-induced thrombocytopenia (HIT) is an immune-mediated complication of heparin therapy, with mortality approaching 10%. The reported prevalence of HIT in adults is 0.2-5.2%. Expert consensus through case reports is the only existing evidence of HIT in BCS. To our knowledge, this is the first study to formalize this anecdotal evidence. METHODS: A retrospective analysis was carried out of patients presenting at a tertiary liver centre with acute liver failure because of BCS or BCS as the primary indication for liver transplantation between 2000 and 2013...
August 2016: European Journal of Gastroenterology & Hepatology
Mitchell Smith, Janette Durham
Transjugular intrahepatic portosystemic shunt creation is a well-established therapy for refractory variceal bleeding and refractory ascites in patients who do not tolerate repeated large volume paracentesis. Experience and technical improvements including covered stents have led to improved TIPS outcomes that have encouraged an expanded application. Evidence for other less frequent indications continues to accumulate, including the indications of primary prophylaxis in patients with high-risk acute variceal bleeding, gastric and ectopic variceal bleeding, primary treatment of medically refractory ascites, recurrent refractory ascites following liver transplantation, hepatic hydrothorax, hepatorenal syndrome, Budd-Chiari syndrome, and portal vein thrombosis...
March 2016: Techniques in Vascular and Interventional Radiology
Kerstin Rosenqvist, Reza Sheikhi, Lars-Gunnar Eriksson, Rupesh Rajani, Fredrik Rorsman, Per Sangfelt, Rickard Nyman
INTRODUCTION: Treatment of Budd-Chiari syndrome (BCS) has shifted from mainly medical treatment, with surgical shunt and orthotopic liver transplantation (OLT) as rescue, to medical treatment combined with an early endovascular intervention in the past two decades. PURPOSE: To assess the safety and efficiency of endovascular treatment of symptomatic patients with BCS and to compare mortality with symptomatic BCS patients in the same region treated with only sporadic endovascular techniques...
June 2016: European Journal of Gastroenterology & Hepatology
Varun K Sharma, Prajakta R Ranade, Shaji Marar, Fazal Nabi, Aabha Nagral
OBJECTIVES: Budd-Chiari syndrome (BCS) is an uncommon cause of chronic liver disease in children. The literature on the management of pediatric BCS is scarce. Our aim was to determine the long-term outcome of patients undergoing a radiological intervention for the treatment of BCS. METHODS: Thirty-two children diagnosed with BCS between 2004 and 2014 were included. Data on the course of disease, medical management, response, and complications related to radiological interventions and outcome were collected...
May 2016: European Journal of Gastroenterology & Hepatology
Mark R Pedersen, Peter Molloy, David Wood, Anil Seetharam
Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction that in severe cases can lead to acute liver failure prompting consideration of revascularization or transplantation. Here, a 22 year old female with angiographically proven BCS secondary to JAK2/V617F positive Polycythemia vera on therapeutic warfarin presented with acute liver failure (ALF). Imaging revealed a new, near complete thrombotic occlusion of the main portal vein with extension into the superior mesenteric vein. An emergent direct intrahepatic portocaval shunt (DIPS) was created and liver function promptly normalized...
January 2016: Annals of Hepatology
Moran Ki, Hwa Young Choi, Kyung-Ah Kim, Bo Hyun Kim, Eun Sun Jang, Sook-Hyang Jeong
BACKGROUND & AIMS: The population-based epidemiology of Budd-Chiari syndrome (BCS), a rare disease of hepatic venous outflow obstruction, is largely unknown. This study aimed to elucidate the nationwide population-based incidence, prevalence, complications, case fatalities and direct medical cost of BCS in South Korea from 2009 to 2013. METHODS: Using two large data sources, the Health Insurance Review and Assessment Service Claims database and Rare Intractable Disease registration program database in Korea, we identified all patients with BCS who were registered under International Classification of Diseases 10 (code I82...
July 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Shashank Kraleti
OBJECTIVE: To describe a patient with Budd-Chiari syndrome (BCS) found to have multiple hypercoagulopathies. CASE SUMMARY: A 33-year-old man who presented with abdominal distension, hematemesis, shortness of breath was found to have liver cirrhosis, portal hypertension and Budd-Chiari Syndrome. He was evaluated for hypercoagulability and was found to be heterozygous for Factor V Leiden mutation and his protein C level was 38% of normal. He was started on oral anticoagulation and underwent elective liver transplantation within 3 months of diagnosis...
October 2015: Journal of the Arkansas Medical Society
Julie C Wigton, Jean M Tersak
A 12-year-old female with Budd-Chiari syndrome underwent liver transplant and subsequent splenectomy. Her platelet count began to rise postoperatively after previous normal values. JAK2V617F-positive essential thrombocythemia (ET) was diagnosed. This case demonstrates that the diagnosis of ET should be considered in the face of normal platelet counts and included on the differential diagnosis for pediatric patients. With this population in mind, we review the current literature on long-term use of platelet-lowering agents...
January 2016: Journal of Pediatric Hematology/oncology
Rishi M Goel, Emma L Johnston, Kamal V Patel, Terence Wong
Budd-Chiari syndrome is a rare disorder characterised by hepatic venous outflow obstruction. It affects 1.4 per million people, and presentation depends upon the extent and rapidity of hepatic vein occlusion. An underlying myeloproliferative neoplasm is present in 50% of cases with other causes including infection and malignancy. Common symptoms are abdominal pain, hepatomegaly and ascites; however, up to 20% of cases are asymptomatic, indicating a chronic onset of hepatic venous obstruction and the formation of large hepatic vein collaterals...
December 2015: Postgraduate Medical Journal
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