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Manieri VM, Offidani M, Capelli D, Marzioni M, Maroni L, Filosa A, Rupoli S, Morsia E, Poloni A, Morè S. Hepatic, gastric and bone marrow AL amyloidosis that began with Budd-Chiari syndrome: a case report. Ann Hematol 2024; 103:3783-3786. [PMID: 38871930 DOI: 10.1007/s00277-024-05837-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2023] [Accepted: 06/06/2024] [Indexed: 06/15/2024]
Abstract
Amyloid Light Chain (AL) Amyloidosis is a rare disorder of protein misfolding and metabolism characterized by insoluble fibrils deposition in various tissues and organs, which could quickly progress and become fatal. The most frequently affected organ is heart being its involvement the most adverse prognostic feature. Kidney and liver could be other organ localizations, defining AL Amyloidosis as a multisystem disorder. Being Budd-Chiari syndrome (BCS) an uncommon congestive hepatopathy caused by blockage of hepatic veins in the absence of cardiac disorders, it could be rarely caused by a massive deposition of amyloid proteins into hepatic sinusoidal spaces, giving an uncommon clinical presentation of AL Amyloidosis.
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Affiliation(s)
| | - Massimo Offidani
- Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Debora Capelli
- Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Marco Marzioni
- Clinica di Gastroenterologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
- Università Politecnica delle Marche, Facoltà Medicina e Chirurgia, Ancona, Italia
| | - Luca Maroni
- Clinica di Gastroenterologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Alessandra Filosa
- Anatomia Patologica, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Serena Rupoli
- Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Erika Morsia
- Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Antonella Poloni
- Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy
| | - Sonia Morè
- Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy.
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Custo S, Tabone E, Aquilina A, Gatt A, Riva N. Splanchnic Vein Thrombosis: The State-of-the-Art on Anticoagulant Treatment. Hamostaseologie 2024; 44:242-254. [PMID: 38354834 DOI: 10.1055/a-2232-5480] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/16/2024] Open
Abstract
Splanchnic vein thrombosis (SVT) is a rare type of venous thromboembolism occurring within the splanchnic venous system. Portal vein thrombosis is the most common presentation, while Budd-Chiari syndrome is the least common. Liver cirrhosis and abdominal solid cancer are the main local risk factors for SVT, whereas myeloproliferative neoplasms are the predominant systemic risk factors. Signs and symptoms of SVT are nonspecific and include abdominal pain, gastrointestinal bleeding, and ascites. Asymptomatic SVT is not uncommon, and the majority would be detected incidentally on routine abdominal imaging performed for the follow-up of liver diseases and tumors. The management of SVT aims to prevent thrombus progression, promote vessel recanalization, and prevent recurrent venous thromboembolism. Anticoagulation should be started early in order to increase the chances of vessel recanalization and reduce the risk of portal hypertension-related complications. Direct oral anticoagulants have been included in recent guidelines, as alternatives to vitamin K antagonists, after clinical stability has been reached; however, caution is required in patients with liver or kidney dysfunction. Treatment duration is based on the presence (or absence) and type (transient vs. permanent) of risk factors. This narrative review aims to summarize the latest evidence on SVT, with a particular focus on the anticoagulant treatment in special categories of patients (e.g., liver cirrhosis, solid cancer, myeloproliferative neoplasms, pancreatitis, incidentally detected SVT, Budd-Chiari syndrome, and chronic SVT).
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Affiliation(s)
- Scott Custo
- Medical School, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Emma Tabone
- Medical School, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Alexia Aquilina
- Medical School, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Alex Gatt
- Department of Pathology, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Nicoletta Riva
- Department of Pathology, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
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Hu X, Jiang X, Li J, Zhao N, Gan H, Hu X, Li L, Liu X, Shan H, Bai Y, Pang P. Identification of potential genetic Loci and polygenic risk model for Budd-Chiari syndrome in Chinese population. iScience 2023; 26:107287. [PMID: 37539039 PMCID: PMC10393737 DOI: 10.1016/j.isci.2023.107287] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2023] [Revised: 05/19/2023] [Accepted: 07/02/2023] [Indexed: 08/05/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction, posing life-threatening risks in severe cases. Reported risk factors include inherited and acquired hypercoagulable states or other predisposing factors. However, many patients have no identifiable etiology, and causes of BCS differ between the West and East. This study recruited 500 BCS patients and 696 normal individuals for whole-exome sequencing and developed a polygenic risk scoring (PRS) model using PLINK, LASSOSUM, BLUP, and BayesA methods. Risk factors for venous thromboembolism and vascular malformations were also assessed for BCS risk prediction. Ultimately, we discovered potential BCS risk mutations, such as rs1042331, and the optimal BayesA-generated PRS model presented an AUC >0.9 in the external replication cohort. This model provides particular insights into genetic risk differences between China and the West and suggests shared genetic risks among BCS, venous thromboembolism, and vascular malformations, offering different perspectives on BCS pathogenesis.
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Affiliation(s)
- Xiaojun Hu
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Xiaosen Jiang
- BGI-Shenzhen, Shenzhen, China
- College of Life Sciences, University of the Chinese Academy of Sciences, Beijing, China
| | - Jia Li
- BGI Genomics, BGI-Shenzhen, Shenzhen, China
- Hebei Industrial Technology Research Institute of Genomics in Maternal & Child Health, Shijiazhuang BGI Genomics Co., Ltd, Shijiazhuang, China
| | - Ni Zhao
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Hairun Gan
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Xinyan Hu
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Luting Li
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Xingtao Liu
- Changfeng Hospital of Jinjiang District, Chengdu, China
| | - Hong Shan
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | | | - Pengfei Pang
- Center for Interventional Medicine, Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
- Guangdong Provincial Key Laboratory of Biomedical Imaging, Fifth Affiliated Hospital, Sun Yat-sen University, Zhuhai, China
- Guangdong Provincial Engineering Research Center of Molecular Imaging, Fifth Affiliated Hospital, Sun Yat-sen University, Zhuhai, China
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Giuli L, Pallozzi M, Venturini G, Gasbarrini A, Ponziani FR, Santopaolo F. Molecular Mechanisms Underlying Vascular Liver Diseases: Focus on Thrombosis. Int J Mol Sci 2023; 24:12754. [PMID: 37628933 PMCID: PMC10454315 DOI: 10.3390/ijms241612754] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2023] [Revised: 08/07/2023] [Accepted: 08/11/2023] [Indexed: 08/27/2023] Open
Abstract
Vascular liver disorders (VLDs) comprise a wide spectrum of clinical-pathological entities that primarily affect the hepatic vascular system of both cirrhotic and non-cirrhotic patients. VLDs more frequently involve the portal and the hepatic veins, as well as liver sinusoids, resulting in an imbalance of liver homeostasis with serious consequences, such as the development of portal hypertension and liver fibrosis. Surprisingly, many VLDs are characterized by a prothrombotic phenotype. The molecular mechanisms that cause thrombosis in VLD are only partially explained by the alteration in the Virchow's triad (hypercoagulability, blood stasis, and endothelial damage) and nowadays their pathogenesis is incompletely described and understood. Studies about this topic have been hampered by the low incidence of VLDs in the general population and by the absence of suitable animal models. Recently, the role of coagulation imbalance in liver disease has been postulated as one of the main mechanisms linked to fibrogenesis, so a novel interest in vascular alterations of the liver has been renewed. This review provides a detailed analysis of the current knowledge of molecular mechanisms of VLD. We also focus on the promising role of anticoagulation as a strategy to prevent liver complications and to improve the outcome of these patients.
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Affiliation(s)
- Lucia Giuli
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Maria Pallozzi
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Giulia Venturini
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Antonio Gasbarrini
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
- Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
| | - Francesca Romana Ponziani
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Francesco Santopaolo
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
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Hyodo R, Takehara Y, Naganawa S. 4D Flow MRI in the portal venous system: imaging and analysis methods, and clinical applications. Radiol Med 2022; 127:1181-1198. [PMID: 36123520 PMCID: PMC9587937 DOI: 10.1007/s11547-022-01553-x] [Citation(s) in RCA: 14] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2022] [Accepted: 08/29/2022] [Indexed: 02/07/2023]
Abstract
Thus far, ultrasound, CT, and 2D cine phase-contrast MRI has been adopted to evaluate blood flow and vascular morphology in the portal venous system; however, all these techniques have some shortcomings, such as limited field of view and difficulty in accurately evaluating blood flow. A new imaging technique, namely 3D cine phase-contrast (4D Flow) MRI, can acquire blood flow data of the entire abdomen at once and in a time-resolved manner, allowing visual, quantitative, and comprehensive assessment of blood flow in the portal venous system. In addition, a retrospective blood flow analysis, i.e., "retrospective flowmetry," is possible. Although the development of 4D Flow MRI for the portal system has been delayed compared to that for the arterial system owing to the lower flow velocity of the portal venous system and the presence of respiratory artifacts, several useful reports have recently been published as the technology has advanced. In the first part of this narrative review article, technical considerations of image acquisition and analysis methods of 4D Flow MRI for the portal venous system and the validations of their results are described. In the second part, the current clinical application of 4D Flow MRI for the portal venous system is reviewed.
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Affiliation(s)
- Ryota Hyodo
- Department of Radiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
| | - Yasuo Takehara
- Department of Radiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan
- Department of Fundamental Development for Advanced Low Invasive Diagnostic Imaging, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Shinji Naganawa
- Department of Radiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan
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Kageyama K, Yamamoto A, Jogo A, Sohgawa E, Hagihara A, Fujii H, Uchida-Kobayashi S, Kawada N, Miki Y. The Albumin-bilirubin Score Detects Changes in the Liver Function during Treatment for Budd-Chiari Syndrome: A Retrospective Observational Study. Intern Med 2022; 61:959-967. [PMID: 34544949 PMCID: PMC9038474 DOI: 10.2169/internalmedicine.8020-21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
Abstract
Objective Mapping the long-term prognosis of Budd-Chiari syndrome (BCS) is difficult, as the prognosis is associated with changes in the liver function. The present study evaluated the time course changes in the liver function in a treatment group with percutaneous old balloon angioplasty (POBA) and a non-treatment group using the albumin-bilirubin score (ALBI) and Child-Pugh score during long-term follow-up. Methods In this retrospective study, 13 consecutive patients diagnosed with BCS at our hospital between 2007 and 2020 were categorized into a treatment group (n=8), which received POBA, and a non-treatment group (n=5). Differences in the liver function in the ALBI and Child-Pugh scores between the initial visit and one- and three-year follow-up were calculated and statistically evaluated. We investigated the changes in the liver function during the long-term follow-up, including events such as re-stenosis and re-treatment. Results While the Child-Pugh scores in the treatment group did not differ significantly between the initial visit and 1- or 3-year follow-up, the ALBI scores in this group improved significantly between the initial visit and the 1- or 3-year follow-up visit (p=0.0078 and 0.0156, respectively). The liver function according to the ALBI score in the treatment group showed gradual improvement from the initial value but gradual worsening in the non-treatment group. The ALBI scores also revealed that the liver function varies according to re-stenosis and re-POBA in BCS patients. Conclusion Unlike the Child-Pugh score, the ALBI score was able to capture changes in the liver function of BCS patients during the long-term course of BCS.
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Affiliation(s)
- Ken Kageyama
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Akira Yamamoto
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Atsushi Jogo
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Etsuji Sohgawa
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Atsushi Hagihara
- Department of Hepatology, Graduate School of Medicine, Osaka City University, Japan
| | - Hideki Fujii
- Department of Hepatology, Graduate School of Medicine, Osaka City University, Japan
| | | | - Norifumi Kawada
- Department of Hepatology, Graduate School of Medicine, Osaka City University, Japan
| | - Yukio Miki
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
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Liu D, Ren Q, Ye T, Zheng C, Su Y, Xia X. Evaluation of dangerous collateral vessels and thrombus in Budd-Chiari syndrome patients with inferior vena cava obstruction. Abdom Radiol (NY) 2022; 47:869-877. [PMID: 34888706 DOI: 10.1007/s00261-021-03354-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2021] [Revised: 11/12/2021] [Accepted: 11/15/2021] [Indexed: 11/24/2022]
Abstract
PURPOSE To evaluate the diagnostic accuracy of preoperative imaging in defining inferior vena cava (IVC) obstruction characteristics, in identifying the presence of a thrombus and dangerous venous collateral. The other goal is to explore the clinical implication of these data in the designing the treatment strategy in Budd-Chiari patients. METHODS This study included 112 patients with IVC obstruction who underwent endovascular treatment between July 2009 and June 2019. Two radiologists independently assessed MSCT and/or MRI imaging data with a 5-point scale to evaluate the diagnostic accuracies relating to obstructive characteristics, dangerous collateral vessels, and thrombus within IVC. RESULTS The diagnostic sensitivities for obstructive characteristics, as determined by the two independent assessors, ranged from 81.25 to 100%. The areas under the receiver operating characteristic curve (ROC) for judging thrombus ranged from 0.87 to 0.975 for the two assessors. Inter-assessor agreement was substantial or excellent with regards to diagnostic accuracy (κ = 0.745-0.927). Twelve cases involving dangerous collateral vessels were identified in the MSCT group of 82 patients (κ = 1); six were identified by digital subtraction venography (DSV) imaging. Eight cases involving dangerous collateral vessels were reported in the MRI group of 32 patients (κ = 1); three were identified by DSV imaging. CONCLUSION Preoperative MSCT and MRI can accurately reveal the obstructive characteristics and risk factors of patients with IVC obstruction and can therefore be used to guide interventional planning so as to minimize complications.
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Affiliation(s)
- Dehan Liu
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277#, Jiefang Road, Wuhan, 430022, Hubei, China
| | - Qianqian Ren
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277#, Jiefang Road, Wuhan, 430022, Hubei, China
| | - Tianhe Ye
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277#, Jiefang Road, Wuhan, 430022, Hubei, China
| | - Chuansheng Zheng
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277#, Jiefang Road, Wuhan, 430022, Hubei, China
| | - Yangbo Su
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277#, Jiefang Road, Wuhan, 430022, Hubei, China
| | - Xiangwen Xia
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277#, Jiefang Road, Wuhan, 430022, Hubei, China.
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Benson CB, Frates MC. Color Duplex Scanning of the Hepatoportal Circulation. NONINVASIVE VASCULAR DIAGNOSIS 2022:933-960. [DOI: 10.1007/978-3-030-60626-8_48] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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Hyodo R, Takehara Y, Mizuno T, Ichikawa K, Yokoyama S, Ishizu Y, Naganawa S. Assessing the Complicated Venous Hemodynamics and Therapeutic Outcomes of Budd-Chiari Syndrome with Respiratory-gated 4D Flow MR Imaging During the Expiratory and Inspiratory Phases. Magn Reson Med Sci 2021; 22:1-6. [PMID: 34880192 PMCID: PMC9849410 DOI: 10.2463/mrms.ici.2021-0110] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023] Open
Abstract
A man in his 50s with Budd-Chiari syndrome diagnosed with the suprahepatic inferior vena cava (IVC) obstruction on CT was assessed using 4D Flow MRI before and after balloon angioplasty. 4D Flow MRI acquired in two respiratory phases, depicted complex hemodynamic and respiratory variability, and a jet stream at the narrowed channel of the membranous IVC. Post-interventional 4D Flow MRI showed that the IVC blood flow increased with corrected flow directions in the infrarenal IVC.
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Affiliation(s)
- Ryota Hyodo
- Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan,Corresponding author: Department of Radiology, Nagoya University Graduate School of Medicine, 65, Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8550, Japan. Phone: +81-52-744-2327, Fax: +81-52-744-2335, E-mail:
| | - Yasuo Takehara
- Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan,Department of Fundamental Development for Advanced Low Invasive Diagnostic Imaging, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan
| | - Takashi Mizuno
- Department of Medical Technology, Nagoya University Hospital, Nagoya, Aichi, Japan
| | - Kazushige Ichikawa
- Department of Medical Technology, Nagoya University Hospital, Nagoya, Aichi, Japan
| | - Shinya Yokoyama
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan
| | - Yoji Ishizu
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan
| | - Shinji Naganawa
- Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan
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Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An Update on the Management of Budd-Chiari Syndrome. Dig Dis Sci 2021; 66:1780-1790. [PMID: 32691382 DOI: 10.1007/s10620-020-06485-y] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Accepted: 07/04/2020] [Indexed: 12/17/2022]
Abstract
Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.
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Affiliation(s)
- A Sharma
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India
| | - S N Keshava
- Department of Interventional Radiology, Christian Medical College, Vellore, India
| | - A Eapen
- Department of Radiodiagnosis, Christian Medical College, Vellore, India
| | - E Elias
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.,Liver Unit, University Hospitals Birmingham, Birmingham, UK
| | - C E Eapen
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.
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Lyu L, Xu P, Ping J, Hu C, Xu K. Changes and significance of hydrodynamic parameters in Budd-Chiari syndrome with obstruction of the inferior vena cava prior to and after interventional therapy. Exp Ther Med 2021; 21:178. [PMID: 33488788 PMCID: PMC7812571 DOI: 10.3892/etm.2021.9609] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2019] [Accepted: 04/17/2020] [Indexed: 01/01/2023] Open
Abstract
In recent years, the role of computational fluid dynamics for Budd-Chiari syndrome evaluation has become the focus of certain studies. The purpose of the present study was to evaluate the role of computational fluid dynamics in Budd-Chiari syndrome with obstruction of the inferior vena cava (IVC). Magnetic resonance venous angiography was used to obtain original IVC and hepatic venous blood flow images from patients with Budd-Chiari syndrome. The computational fluid dynamics method was used to establish a three-dimensional model and simulate the blood flow velocity, wall shear stress and wall pressure. The results revealed that the hemodynamic parameters of Budd-Chiari syndrome were successfully simulated by computational fluid dynamics. The hemodynamic parameters of the IVC stenosis varied with the cardiac cycle. Vascular flow velocity (pre-operative, 1.64±0.10 m/sec; post-operative, 0.34±0.14 m/sec; t=34.97, P<0.001) and wall shear stress (pre-operative, 25.69±2.85 Pa; post-operative, 3.51±1.70 Pa; t=29.86, P<0.001) at the area of stenosis decreased after interventional therapy and the wall pressure increased (pre-operative, -119.33±251.50 Pa; post-operative, 1,128.42±207.70 Pa; t=17.10, P<0.001). In conclusion, the computational fluid dynamics method was able to effectively simulate the hemodynamic parameters of Budd-Chiari syndrome with obstruction of the IVC and may provide an effective quantitative method for the evaluation of vascular function post-treatment.
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Affiliation(s)
- Lulu Lyu
- The First School of Clinical Medicine, Nanjing Medical University, Nanjing, Jiangsu 210029, P.R. China.,Department of CT and MRI, Xuzhou Central Hospital, Xuzhou, Jiangsu 221009, P.R. China
| | - Peng Xu
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221002, P.R. China
| | - Jie Ping
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221002, P.R. China
| | - Chunfeng Hu
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221002, P.R. China
| | - Kai Xu
- The First School of Clinical Medicine, Nanjing Medical University, Nanjing, Jiangsu 210029, P.R. China.,Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221002, P.R. China
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Niknam R, Hajizadegan N, Mohammadkarimi V, Mahmoudi L. A study of the different parameters in acute and chronic Budd–Chiari syndrome. EGYPTIAN LIVER JOURNAL 2020; 10:48. [DOI: 10.1186/s43066-020-00058-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2020] [Accepted: 09/21/2020] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Budd–Chiari syndrome (BCS) is a rare and potentially life-threatening vascular disease of the liver. There are a few studies on the differences between acute and chronic BCS in clinical and laboratory characteristics, as well as the outcomes, so we designed this research to study the different parameters in acute and chronic BCS. Diagnosis of BCS was made using Doppler ultrasound, magnetic resonance imaging, and venography. Patients with BCS were then divided into chronic and acute groups based on both imaging characteristics and disease duration. Finally, the outcomes, clinical features, and laboratory parameters of acute and chronic BCS were compared.
Results
In total, 60 patients were included in this study, of whom 28.3% and 71.7% had acute and chronic BCS, respectively. According to clinical features, spontaneous bacterial peritonitis (PR 1.289; 95% CI 1.115–1.489; P = 0.001) and jaundice (PR 1.308; 95% CI 1.148–1.490; P < 0.001) were significantly associated with chronic than acute BCS. According to laboratory parameters, the levels of international normalized ratio (INR) (PR 0.953; 95% CI 0.918–0.989; P = 0.012), blood urea nitrogen (BUN) (PR 0.996; 95% CI 0.993–1.000; P = 0.039), and model for end-stage liver disease (MELD) score (PR 1.024; 95% CI 1.012–1.037; P < 0.001) in chronic group were significantly higher than in the acute BCS. The hospital length of stay (LOS) and mortality rate between the two groups did not differ significantly.
Conclusions
This study showed that the spontaneous bacterial peritonitis, jaundice, increased levels of MELD score, INR, and BUN were significantly associated with the chronic group compared with the acute group. The hospital LOS and mortality rate between the two groups did not differ significantly. Further research is recommended to clarify this issue.
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13
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Venous thrombosis of the liver: current and emerging concepts in management. Transl Res 2020; 225:54-69. [PMID: 32407789 DOI: 10.1016/j.trsl.2020.04.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Revised: 04/11/2020] [Accepted: 04/16/2020] [Indexed: 02/08/2023]
Abstract
Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.
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Inchingolo R, Posa A, Mariappan M, Tibana TK, Nunes TF, Spiliopoulos S, Brountzos E. Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review. World J Gastroenterol 2020; 26:5060-5073. [PMID: 32982109 PMCID: PMC7495032 DOI: 10.3748/wjg.v26.i34.5060] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2020] [Revised: 06/06/2020] [Accepted: 08/25/2020] [Indexed: 02/06/2023] Open
Abstract
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.
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Affiliation(s)
- Riccardo Inchingolo
- Interventional Radiology Unit, "F. Miulli" Regional Hospital, Acquaviva delle Fonti 70021, Italy
- Department of Radiology, King´s College Hospital, London SE5 9RS, United Kingdom
| | - Alessandro Posa
- Department of Radiology, Gemelli Hospital, Roma 00135, Italy
| | - Martin Mariappan
- Interventional Radiology Department, Aberdeen Royal Infirmary Hospital, Aberdeen AB25 2ZN, United Kingdom
| | - Tiago Kojun Tibana
- Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
| | - Thiago Franchi Nunes
- Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
| | - Stavros Spiliopoulos
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
| | - Elias Brountzos
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
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Xu P, Lyu L, Ge H, Sami MU, Liu P, Hu C, Xu K. Segmental Liver Stiffness Evaluated with Magnetic Resonance Elastography Is Responsive to Endovascular Intervention in Patients with Budd-Chiari Syndrome. Korean J Radiol 2020; 20:773-780. [PMID: 30993928 PMCID: PMC6470085 DOI: 10.3348/kjr.2018.0767] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2018] [Accepted: 01/18/2019] [Indexed: 12/12/2022] Open
Abstract
OBJECTIVE To assess segmental liver stiffness (LS) with MRI before and after endovascular intervention in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS Twenty-three patients (13 males and 10 females; mean age, 42.6 ± 12.6 years; age range, 31-56 years) with BCS as a primary liver disease were recruited for this study. Two consecutive magnetic resonance elastography (MRE) examinations were performed before the endovascular treatment. Fifteen patients who underwent endovascular intervention treatment also had follow-up MRE scans within three days after the procedure. LS was measured in three liver segments: the right posterior, right anterior, and left medial segments. Inter-reader and inter-exam repeatability were analyzed with intraclass correlation coefficients (ICCs) and Bland-Altman analysis. Segmental LS and clinical characteristics before and after the intervention were also compared. RESULTS Within three days of the endovascular intervention, all three segmental LS values decreased: LS of the right posterior segment = 7.23 ± 0.88 kPa (before) vs. 4.94 ± 0.84 kPa (after), LS of the right anterior segment = 7.30 ± 1.06 kPa (before) vs. 4.77 ± 0.85 kPa (after), and LS of the left medial segment = 7.22 ± 0.87 kPa (before) vs. 4.87 ± 0.72 kPa (after) (all p = 0.001). There was a significant correlation between LS changes and venous pressure gradient changes before and after treatments (r = 0.651, p = 0.009). The clinical manifestations of all 15 patients significantly improved after therapy. The MRE repeatability was excellent, with insignificant variations (inter-reader, ICC = 0.839-0.943: inter-examination, ICC = 0.765-0.869). Bland-Altman analysis confirmed excellent agreement (limits of agreement, 13.4-19.4%). CONCLUSION Segmental LS measured by MRE is a promising repeatable quantitative biomarker for monitoring the treatment response to minimally invasive endovascular intervention in patients with BCS.
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Affiliation(s)
- Peng Xu
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Lulu Lyu
- Department of CT and MRI, Xuzhou Central Hospital, Xuzhou, China.,The First School of Clinical Medicine, Nanjing Medical University, Nanjing, China
| | - Haitao Ge
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Muhammad Umair Sami
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Panpan Liu
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Chunfeng Hu
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Kai Xu
- Department of Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China.,The First School of Clinical Medicine, Nanjing Medical University, Nanjing, China.
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Kageyama K, Yamamoto A, Jogo A, Nota T, Murai K, Ogawa S, Nakano MM, Sohgawa E, Hamamoto S, Hamuro M, Kaminou T, Nishida N, Takahashi K, Yamamoto K, Miki Y. Usefulness of Venous Pressure Measurement in Endovascular Treatment of Budd-Chiari Syndrome: A Retrospective Cohort Study. Intern Med 2019; 58:2923-2929. [PMID: 31243212 PMCID: PMC6859396 DOI: 10.2169/internalmedicine.2704-19] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023] Open
Abstract
Objective Therapeutic predictors derived from the venous pressure before therapy have not been identified for Budd-Chiari syndrome (BCS). The aim of this study was to determine whether or not measuring the distal pressure or pressure gradient was useful for predicting treatment efficacy in BCS. Methods We retrospectively analyzed seven consecutive patients diagnosed with symptomatic BCS at our hospital between 2008 and 2017. Distal and proximal venous pressures at occlusion sites of BCS were measured before treatment in all cases. The pressure gradient was defined as the difference between distal and proximal venous pressures. A receiver operating characteristics (ROC) analysis was performed for venous pressures. Results Percutaneous old balloon angioplasty (POBA) was performed in seven cases, with technical success achieved in all cases (100%). No complications were encountered. The median primary patency was 574 (interquartile range, 439.5-1,056.5) days. The 1-year primary patency rate was 71.73%. Six cases (85.7%) showed resolution of symptoms, representing clinical success. The ROC analysis revealed a high distal pressure (area under the ROC curve = 0.83, cut-off=12 mmHg) as a predictor of treatment efficacy of POBA for symptomatic BCS. In addition, the pressure gradient was considered significant from a clinical perspective, because the 6 successful cases with resolution of symptoms showed a large pressure gradient (range, 8-21 mmHg) before treatment, whereas the failed case showed a relatively small pressure gradient (7 mmHg). Conclusion High distal pressure and a large pressure gradient might predict the treatment efficacy of balloon angioplasty for symptomatic BCS.
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Affiliation(s)
- Ken Kageyama
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Akira Yamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Atsushi Jogo
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Takehito Nota
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Kazuki Murai
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Satoyuki Ogawa
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Mariko M Nakano
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Etsuji Sohgawa
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Shinichi Hamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Masao Hamuro
- Department of Radiology, Izumiotsu Municipal Hospital, Japan
| | - Toshio Kaminou
- Department of Radiology, National Hospital Organization Osaka Minami Medical Center, Japan
| | - Norifumi Nishida
- Department of Radiology, Osaka Saiseikai Nakatsu Hospital, Japan
| | - Kanae Takahashi
- Department of Medical Statistics, Osaka City University Graduate School of Medicine, Japan
| | - Kouji Yamamoto
- Department of Medical Statistics, Osaka City University Graduate School of Medicine, Japan
| | - Yukio Miki
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
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Bansal V, Gupta P, Sinha S, Dhaka N, Kalra N, Vijayvergiya R, Dutta U, Kochhar R. Budd-Chiari syndrome: imaging review. Br J Radiol 2018; 91:20180441. [PMID: 30004805 PMCID: PMC6319835 DOI: 10.1259/bjr.20180441] [Citation(s) in RCA: 55] [Impact Index Per Article: 7.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2018] [Revised: 06/13/2018] [Accepted: 07/09/2018] [Indexed: 12/16/2022] Open
Abstract
Budd-Chiari syndrome (BCS), also known as hepatic venous outflow tract obstruction includes a group of conditions characterized by obstruction to the outflow of blood from the liver secondary to involvement of one or more hepatic veins (HVs), inferior vena cava (IVC) or the right atrium. There are a number of conditions that lead to BCS-ranging from hypercoagulable states to malignancies. In up to 25% patients, no underlying disorder is identified. Diagnosis of BCS is based on a combination of clinical and imaging features. A major part of the literature in BCS has been devoted to interventions; however, a detailed description of various imaging manifestations of BCS is lacking. In this review, we highlight the importance of various imaging modalities in the diagnosis of BCS.
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Affiliation(s)
- Varun Bansal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute ofMedical Imaging and Research (PGIMER), Chandigarh, India
| | - Pankaj Gupta
- Deapartment of Gastroenterology, Postgraduate Institue of Medical Education and Research, Chandigarh, India
| | - Saroj Sinha
- Deapartment of Gastroenterology, Postgraduate Institue of Medical Education and Research, Chandigarh, India
| | - Narender Dhaka
- Deapartment of Gastroenterology, Postgraduate Institue of Medical Education and Research, Chandigarh, India
| | - Naveen Kalra
- Department of Radiodiagnosis and Imaging, Postgraduate Institute ofMedical Imaging and Research (PGIMER), Chandigarh, India
| | - Rajesh Vijayvergiya
- Department of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Usha Dutta
- Deapartment of Gastroenterology, Postgraduate Institue of Medical Education and Research, Chandigarh, India
| | - Rakesh Kochhar
- Deapartment of Gastroenterology, Postgraduate Institue of Medical Education and Research, Chandigarh, India
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Li Q, Zhang T, Wang D, Li W, Zhang X, Zhang X. Radical surgical treatment of Budd-Chiari syndrome through entire exposure of hepatic inferior vena cava. J Vasc Surg Venous Lymphat Disord 2018; 7:74-81. [PMID: 30341019 DOI: 10.1016/j.jvsv.2018.02.007] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2017] [Accepted: 02/19/2018] [Indexed: 11/28/2022]
Abstract
OBJECTIVE Therapies for Budd-Chiari syndrome (BCS) can be divided into three main categories: medical, endovascular, and surgical. Surgery is applicable to the disease when other therapeutic options have failed. We introduce a surgical method of recanalization through exposure of the entire hepatic inferior vena cava (IVC) and hepatic vein (HV) outflow tract for BCS and investigate the long-term outcomes. METHODS From July 2002 to December 2015 in our center, 83 consecutive symptomatic BCS patients with failure of endovascular therapy were treated by radical surgical recanalization. IVC recanalization was the first goal for all patients, and recanalization of at least one HV was the second goal for selected patients at the same surgical operation. Patients were followed up, and data on technical and clinical success, survival, and patency of target vessels were analyzed. RESULTS Technical success of surgical recanalization was achieved in 80 patients (96.4%), with relief of clinical symptoms and improvement of liver function. During a mean follow-up of 84 ± 25.9 months, the cumulative 1-, 3-, and 5-year primary patency rates of the HV were 96.7%, 90.0%, and 83.3%, respectively. The cumulative 1-, 3-, and 5-year primary patency of the IVC was 86.7%, 71.7%, and 68.3%, respectively. No factor demonstrated significant association with recurrence of obstruction. During follow-up, 10 patients died, 8 of end-stage hepatic disease and 2 of unknown causes. The cumulative 1-, 3-, and 5-year all-cause survival rates were 91%, 90%, and 87%, respectively. Female sex, encephalopathy, severe ascites, and hypersplenism had an impact on survival in univariate analysis. With Cox regression, encephalopathy was the only independent determining factor for surgical survival. CONCLUSIONS Surgical recanalization through exposure of the entire hepatic IVC for BCS is suitable for most primary BCS patients after failure of endovascular therapies.
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Affiliation(s)
- Qingle Li
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
| | - Tao Zhang
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
| | - Dashuai Wang
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
| | - Wei Li
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
| | - Xuemin Zhang
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
| | - Xiaoming Zhang
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China.
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Van Wettere M, Bruno O, Rautou PE, Vilgrain V, Ronot M. Diagnosis of Budd-Chiari syndrome. Abdom Radiol (NY) 2018; 43:1896-1907. [PMID: 29285598 DOI: 10.1007/s00261-017-1447-2] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition. Acute BCS is rare, and patients present with hepatomegaly, ascites, and hepatic insufficiency. The diagnosis is based on imaging. Imaging features include (1) direct signs, in particular occlusion or compression of the hepatic veins and/or the inferior vena cava and venous collaterals and (2) indirect signs, in particular morphological changes in the liver with hypertrophy of the caudate lobe and delayed nodule formation. Ultrasound and magnetic resonance imaging are the gold standard for diagnosis. The aim of this review is to provide an overview of the role of imaging in the diagnosis of BCS.
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20
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Das CJ, Soneja M, Tayal S, Chahal A, Srivastava S, Kumar A, Baruah U. Role of radiological imaging and interventions in management of Budd-Chiari syndrome. Clin Radiol 2018; 73:610-624. [PMID: 29549997 DOI: 10.1016/j.crad.2018.02.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2017] [Accepted: 02/08/2018] [Indexed: 02/06/2023]
Abstract
Budd-Chiari syndrome (BCS) is a clinical condition resulting from impaired hepatic venous drainage, in which there is obstruction to the hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium leading to hepatic congestion. The diagnosis of BCS is based on imaging, which can be gathered from non-invasive investigations such as ultrasonography coupled with venous Doppler, triphasic computed tomography (CT) and magnetic resonance imaging (MRI). Apart from diagnosis, various interventional radiology procedures aid in the successful management of this syndrome. In this article, we present various imaging features of BCS along with various interventional procedures that are used to treat this diverse condition.
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Affiliation(s)
- C J Das
- Department of Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India.
| | - M Soneja
- Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India
| | - S Tayal
- Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India
| | - A Chahal
- Department of Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India
| | - S Srivastava
- Department of Gastroenterology, GB Pant Hospital, New Delhi - 110002, India
| | - A Kumar
- Department of Gastroenterology, GB Pant Hospital, New Delhi - 110002, India
| | - U Baruah
- Department of Anaesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India
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Li G, Huang Y, Tang S, Song Y, Liang H, Liu D, Yang L, Hou X. A single-center retrospective study: Clinical features of different types of Budd-Chiari syndrome in Chinese patients in the Hubei area. Vascular 2018; 26:80-89. [PMID: 28738751 DOI: 10.1177/1708538117718638] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Background The characteristics and prevalence of Budd-Chiari syndrome in China remain unclear. This study aimed to analyze the clinical features of Budd-Chiari syndrome in Chinese patients in the Hubei area. Methods One-hundred and thirty patients with Budd-Chiari syndrome, admitted to Union Hospital from January 2002 to January 2011, were included in this retrospective study. Clinical features, laboratory data, imaging characteristics, and cumulative patency rates were analyzed. Results Of the 130 patients with Budd-Chiari syndrome, 77 were men (59.2%) and 53 women (40.8%). Budd-Chiari syndrome was more commonly associated with inferior vena cava block (56.9%, 74/130) than hepatic vein block (19.2%, 25/130) and combined inferior vena cava/hepatic vein block (23.9%, 31/130). The clinical features of Budd-Chiari syndrome varied based on the location of the obstruction. The incidence of bilirubin abnormality, elevated alkaline phosphatase, and γ-glutamyl peptide transferase levels was common in patients with Budd-Chiari syndrome. Liver injury was more severe in cases with combined inferior vena cava/hepatic vein block than in the other two types of Budd-Chiari syndrome. Color Doppler ultrasound imaging was better for the diagnosis of hepatic vein obstruction, while computed tomography and magnetic resonance imaging were superior in diagnosing inferior vena cava obstruction. The cumulative 1-, 5-, and 10-year patency rates were 97%, 69%, and 59%, respectively. Univariate analysis indicated that liver cirrhosis was an independent risk factor of recurrence. Conclusion The most prevalent type of Budd-Chiari syndrome is inferior vena cava obstruction in Chinese patients in the Hubei area. Different types of Budd-Chiari syndrome have diverse clinical and biochemical features, which may assist clinicians in diagnosing Budd-Chiari syndrome. Liver cirrhosis was found as an independent risk factor of recurrence.
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Affiliation(s)
- Gangping Li
- 1 Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Ying Huang
- 1 Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Shunyu Tang
- 1 Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yuhu Song
- 1 Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Huimin Liang
- 2 Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Dehan Liu
- 2 Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Ling Yang
- 1 Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xiaohua Hou
- 1 Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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Wang Q, Han G. Budd-Chiari Syndrome and Inferior Vena Cava Obstruction: The Asian Perspective. DIAGNOSTIC METHODS FOR CIRRHOSIS AND PORTAL HYPERTENSION 2018:257-269. [DOI: 10.1007/978-3-319-72628-1_17] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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Malikowski TM, Podboy AJ, Sweetser S. 57-Year-Old Woman With Abdominal Pain. Mayo Clin Proc 2017. [PMID: 28647225 DOI: 10.1016/j.mayocp.2016.10.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Affiliation(s)
- Thomas M Malikowski
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Alexander J Podboy
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Seth Sweetser
- Advisor to residents and Consultant in Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN.
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Hatzidakis A, Galanakis N, Kehagias E, Samonakis D, Koulentaki M, Matrella E, Tsetis D. Ultrasound-guided direct intrahepatic portosystemic shunt in patients with Budd–Chiari syndrome: Short- and long-term results. Interv Med Appl Sci 2017. [DOI: 10.1556/1646.9.2017.14] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Affiliation(s)
- Adam Hatzidakis
- Interventional Radiology Unit, Department of Medical Imaging, University Hospital of Heraklion, Heraklion, Greece
| | - Nikolaos Galanakis
- Interventional Radiology Unit, Department of Medical Imaging, University Hospital of Heraklion, Heraklion, Greece
| | - Elias Kehagias
- Interventional Radiology Unit, Department of Medical Imaging, University Hospital of Heraklion, Heraklion, Greece
| | - Dimitrios Samonakis
- Department of Gastroenterology, University Hospital of Heraklion, Heraklion, Greece
| | - Mairi Koulentaki
- Department of Gastroenterology, University Hospital of Heraklion, Heraklion, Greece
| | - Erminia Matrella
- Department of Gastroenterology, University Hospital of Heraklion, Heraklion, Greece
| | - Dimitrios Tsetis
- Interventional Radiology Unit, Department of Medical Imaging, University Hospital of Heraklion, Heraklion, Greece
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25
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Working Subgroup for Clinical Practice Guideline for Aberrant Portal Hemodynamics. Diagnosis and treatment guidelines for aberrant portal hemodynamics: The Aberrant Portal Hemodynamics Study Group supported by the Ministry of Health, Labor and Welfare of Japan. Hepatol Res 2017; 47:373-386. [PMID: 28058764 DOI: 10.1111/hepr.12862] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/06/2016] [Revised: 12/29/2016] [Accepted: 01/04/2017] [Indexed: 12/18/2022]
Abstract
Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.
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Dou JP, Yu J, Han ZY, Liu FY, Cheng ZG, Liang P. Microwave ablation for hepatocellular carcinoma associated with Budd-Chiari syndrome after transarterial chemoembolization: an analysis of ten cases. Abdom Radiol (NY) 2017; 42:962-968. [PMID: 27688061 DOI: 10.1007/s00261-016-0923-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
PURPOSE To evaluate the feasibility, efficacy, and safety of microwave ablation (MWA) in the treatment of hepatocellular carcinoma associated with Budd-Chiari syndrome (BCS) after transarterial chemoembolization (TACE). METHODS A total of 10 patients (mean 50.0 ± 7.5 years) with 15 BCS-associated HCC lesions were retrospectively evaluated. All patients received MWA treatment for residual tumors after 1 to 3 sessions of TACE. The diagnosis of residual tumors was confirmed by at least two types of enhanced imaging. CEUS images were performed to confirm the residual lesions and guide the placement of antenna before MWA. Thermal monitoring and artificial pleural effusion or ascites were used to guarantee ablative accuracy and safety for patients with tumors adjacent to vital structures. Technical success, technique efficacy, local tumor progression, survival rate, and the incidence of complications were comprehensively analyzed. RESULTS Technical success and technique effectiveness were achieved in all patients. Thirteen lesions achieved complete ablation for the first time, and 2 lesions needed two sessions. Thermal monitoring was used in 2 patients, artificial pleural effusion was used in 1 patient, and artificial ascites in 2 patients. In a median follow-up of 34.5 months (range 21-52 months), no LTP was founded in all patients. Intrahepatic recurrence was found in 5 patients. 1-, 2-, 3-, and 4-year survival rates were 100%, 100%, 74.1%, and 37.0%, respectively. No major or minor complications were observed. CONCLUSION Microwave ablation is a feasible and effective way to treat residual tumors after TACE treatment in patients with BCS-associated HCC.
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Affiliation(s)
- Jian-Ping Dou
- Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China
| | - Jie Yu
- Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China
| | - Zhi-Yu Han
- Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China
| | - Fang-Yi Liu
- Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China
| | - Zhi-Gang Cheng
- Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China
| | - Ping Liang
- Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China.
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Raza SM, Zainab S, Shamsaeefar AR, Nikeghbalian S, Malek Hosseini SA. Experience of Liver Transplant in Patients Diagnosed with Budd-Chiari Syndrome. EXP CLIN TRANSPLANT 2017; 16:177-181. [PMID: 28176618 DOI: 10.6002/ect.2016.0129] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
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. Among these, 68 presented with Budd-Chiari syndrome. RESULTS The median age was 31 years among 27 male and 41 female patients. Five patients received pretransplant interventions, with 2 treated with inferior vena cava stenting and 3 having transjugular intrahepatic portosystemic shunts. Sixty-five patients with Budd-Chiari syndrome received deceased-donor grafts and 3 received living-donor grafts. Among the Budd-Chiari transplant patients, 6 patients died. Five deaths occurred in the early posttransplant period, and 1 patient retransplanted after 2 years for recurrence of disease died due to graft failure. The five-year survival rate was 89% among patients with Budd-Chiari syndrome. CONCLUSIONS Liver transplant along with posttransplant anticoagulation therapy can improve the survival of patients with advanced-stage Budd-Chiari syndrome.
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Affiliation(s)
- Syed Muhammad Raza
- From the Sindh Institute of Urology and Transplantation, Karachi, Pakistan
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Alcaraz G, Meineri M, Dattilo K, Wąsowicz M. Intraoperative Transesophageal Echocardiographic Diagnosis of Acute Budd-Chiari Syndrome After Extended Right Hepatectomy. ACTA ACUST UNITED AC 2017; 7:13-5. [PMID: 27166743 DOI: 10.1213/xaa.0000000000000326] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Budd-Chiari syndrome (BCS) is a congestive hepatopathy caused by hepatic venous outflow obstruction. Torsion of the remnant liver after extended right hepatectomy is a potential cause of acute BCS, and it can lead to acute liver failure or death. We present a case of intraoperative transesophageal echocardiographic (TEE) diagnosis of acute BCS after extended right hepatectomy. TEE allowed timely detection of acute BCS and consequent inferior vena cava obstruction and decreased right atrial filling as the cause of sudden life-threatening hemodynamic collapse unresponsive to intravascular volume therapy and inotropic support. TEE constituted a stepped-up level of monitoring, prompting an immediate surgical reexploration, and resolution of hemodynamic instability.
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Affiliation(s)
- Gabriela Alcaraz
- From the Toronto General Hospital, University Health Network, Toronto, Ontario, Canada
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Percutaneous Transluminal Angioplasty for Complete Membranous Obstruction of Suprahepatic Inferior Vena Cava: Long-Term Results. Cardiovasc Intervent Radiol 2016; 39:1392-9. [PMID: 27272713 DOI: 10.1007/s00270-016-1394-2] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/06/2016] [Accepted: 05/26/2016] [Indexed: 10/21/2022]
Abstract
PURPOSE To determine the long-term results of percutaneous transluminal angioplasty (PTA) for a complete membranous obstruction of the suprahepatic inferior vena cava. METHODS Patients (n = 65) who were referred to the interventional unit for PTA for a complete membranous obstruction of the suprahepatic inferior vena cava between January 2006 and October 2014 were included in the study. Thirty-two patients (18 males, 14 females, mean age 35 ± 10.7, range 20-42 years) were treated. The patients presented with symptoms of ascites (88 %), pleural effusion (53 %), varicose veins (94 %), hepatomegaly (97 %), abdominal pain (84 %), and splenomegaly (40 %). Transjugular liver access set and re-entry catheter were used to puncture and traverse the obstruction from the jugular side. PTA balloon dilations were performed. The mean follow-up period was 65.6 ± 24.5 months. The objective was to evaluate technical success, complications, primary patency, and clinical improvement in the symptoms of the patients. RESULTS The technical success rate was 94 %. In two patients, obstruction could not be traversed. These patients underwent cavoatrial graft bypass surgery. There were no procedure-related complications. Clinical improvements were achieved in all patients within 3 months. The primary patency rate at 4 years was 90 %. There was no primary assisted patency. There was no need for metallic stent deployment in the cohort. The secondary patency rate at 4 years was 100 %. CONCLUSIONS Percutaneous transluminal angioplasty for a complete membranous obstruction of the suprahepatic inferior vena cava is safe and effective, and the long-term results are excellent.
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Endovascular treatment of symptomatic Budd-Chiari syndrome - in favour of early transjugular intrahepatic portosystemic shunt. Eur J Gastroenterol Hepatol 2016; 28:656-60. [PMID: 26958788 DOI: 10.1097/meg.0000000000000621] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
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. METHODS This was a retrospective review of clinical data, treatment and survival in 14 patients diagnosed with BCS and treated with endovascular methods from 2003 to 2015. A national epidemiology study of BCS from 1986 to 2003 was used for comparison. RESULTS Thirteen of the 14 patients eventually had transjugular intrahepatic portosystemic shunt (TIPS), four after previous liver vein angioplasty. TIPS were performed with polytetrafluoroethylene-covered stents and technical success was 100%. Calculated preinterventional prognostic indices indicated a high risk of TIPS dysfunction, OLT and death. However, only one patient died and one had an OLT, and the 1- and 2-year primary TIPS-patency was 85 and 67%, respectively. Episodes of de-novo hepatic encephalopathy occurred in three patients. Overall 1- and 5-year transplantation-free survival was 100 and 93% compared with 47 and 28%, respectively, in 1986 to 2003. CONCLUSION TIPS seems to be a safe and effective treatment for symptomatic BCS and there is an obvious improvement in transplantation-free survival compared with conservatory medical treatment. It should, therefore, be considered early, as first-line intervention, in patients with insufficient response to medical treatment.
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Wang CY, Liao CY, Huang SC, Yeh YC. Budd-Chiara syndrome and chylothorax. QJM 2016; 109:211-2. [PMID: 26574499 DOI: 10.1093/qjmed/hcv216] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
Affiliation(s)
- C-Y Wang
- Internal Medicine Department, Kaohsiung Armed Forces General Hospital, Kaohsiung City 802, Taiwan, Republic of China.
| | - C-Y Liao
- Internal Medicine Department, Kaohsiung Armed Forces General Hospital, Kaohsiung City 802, Taiwan, Republic of China.
| | - S-C Huang
- Internal Medicine Department, Kaohsiung Armed Forces General Hospital, Kaohsiung City 802, Taiwan, Republic of China.
| | - Y-C Yeh
- Internal Medicine Department, Kaohsiung Armed Forces General Hospital, Kaohsiung City 802, Taiwan, Republic of China.
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Huang Q, Shen B, Zhang Q, Xu H, Zu M, Gu Y, Wei N, Cui Y, Huang R. Comparison of Long-Term Outcomes of Endovascular Management for Membranous and Segmental Inferior Vena Cava Obstruction in Patients With Primary Budd–Chiari Syndrome. Circ Cardiovasc Interv 2016; 9:e003104. [PMID: 26908849 DOI: 10.1161/circinterventions.115.003104] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Affiliation(s)
- Qianxin Huang
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Bin Shen
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Qingqiao Zhang
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Hao Xu
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Maoheng Zu
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Yuming Gu
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Ning Wei
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Yanfeng Cui
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
| | - Rui Huang
- From the Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou, China
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Bell BM, Cura M, Shaw CJ, Rees CR. Transjugular intrahepatic portosystemic shunt creation using a three-dimensional fluoroscopy guidance system in patients with the Budd-Chiari syndrome. Proc (Bayl Univ Med Cent) 2015; 28:484-7. [PMID: 26424949 DOI: 10.1080/08998280.2015.11929316] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022] Open
Abstract
When performed for Budd-Chiari syndrome (BCS), transjugular intrahepatic portosystemic shunt (TIPS) creation can be technically difficult due to hepatic congestion and asymmetric hypertrophy. We present three female patients with decompensated BCS in whom TIPS were created using a three-dimensional fluoroscopy guidance system. On a dedicated workstation using three-dimensional volumes of computed tomography imaging, a virtual needle path was created by the operator extending from the needle entry point (hepatic vein stump or inferior vena cava) to the target portal vein. Subsequently, the virtual needle path was overlaid on the fluoroscopy image for guidance of portal venous cannulation. This technology can be used for TIPS procedures in patients with BCS and other complex TIPS cases, as it may help delimit the trajectory of the needle pass and optimally result in more efficient procedures with decreased radiation dose.
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Affiliation(s)
- Bruce M Bell
- Department of Radiology, Baylor University Medical Center at Dallas
| | - Marco Cura
- Department of Radiology, Baylor University Medical Center at Dallas
| | - Cathryn J Shaw
- Department of Radiology, Baylor University Medical Center at Dallas
| | - Chet R Rees
- Department of Radiology, Baylor University Medical Center at Dallas
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Falcão CK, Fagundes GCF, Lamos GC, Felipe-Silva A, Lovisolo SM, Martines JA, de Campos FPF. Budd-Chiari Syndrome: an unnoticed diagnosis. AUTOPSY AND CASE REPORTS 2015; 5:17-25. [PMID: 26484330 PMCID: PMC4584664 DOI: 10.4322/acr.2015.009] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2015] [Accepted: 05/28/2015] [Indexed: 01/30/2023] Open
Abstract
Budd–Chiari syndrome (BCS) encompasses a group of disorders caused by the obstruction to the hepatic venous outflow at the level of the small or large hepatic veins, the inferior vena cava, or any combination thereof. Clinical manifestation of the subacute form is characterized by supramesocolic abdominal discomfort, abdominal distension, fever, and lower limbs edema. Imaging work-up with hepatic Doppler ultrasound and abdominal computed tomography (CT) enables the diagnosis in the majority of cases. Treatment comprises long-term anticoagulation associated with measures that attempt to re-establish the flow in the thrombosed vessel (thrombolysis or angioplasty) or through the venous blood flow bypasses (transjugular intrahepatic portosystemic shunt or surgical bypass); however, the outcome is often dismal. The authors report the case of a 37-year-old woman presenting a 2-month history of dyspeptic complaints and abdominal distention. Fever was present at the beginning of symptoms. The laboratory work-up disclosed mild hepatic dysfunction, and the ultrasound showed evidence of chronic liver disease. Despite a thorough etiologic investigation, diagnosis was missed and, therefore, management could not be directed towards the physiopathogenetic process. The outcome was characterized by portal hypertension and esophageal varices bleeding. The patient died and the autopsy findings were characteristic of BCS, although an abdominal CT, close to death, had showed signs consistent with this diagnosis. The authors highlight the importance of knowledge of this entity, the diagnostic methods, and the multidisciplinary approach. BCS should be considered whenever investigating etiology for chronic or acute hepatopathy.
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Affiliation(s)
- Camila Kruschewsky Falcão
- Internal Medicine Department - Hospital das Clínicas - Faculdade de Medicina -Universidade de São Paulo, São Paulo/SP - Brazil
| | - Gustavo C Freitas Fagundes
- Internal Medicine Department - Hospital das Clínicas - Faculdade de Medicina -Universidade de São Paulo, São Paulo/SP - Brazil
| | - Gustavo Checolli Lamos
- Internal Medicine Department - Hospital das Clínicas - Faculdade de Medicina -Universidade de São Paulo, São Paulo/SP - Brazil
| | - Aloisio Felipe-Silva
- Anatomy Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil
| | - Silvana Maria Lovisolo
- Anatomy Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil
| | - João Augusto Martines
- Radiology Department - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil
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Taslakian B, Faraj W, Khalife M, Al-Kutoubi A, El-Merhi F, Saade C, Hallal A, Haydar A. Assessment of surgical portosystemic shunts and associated complications: The diagnostic and therapeutic role of radiologists. Eur J Radiol 2015; 84:1525-1539. [PMID: 25963504 DOI: 10.1016/j.ejrad.2015.04.021] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2015] [Revised: 04/16/2015] [Accepted: 04/21/2015] [Indexed: 02/07/2023]
Abstract
Surgical portosystemic shunting, the formation of a vascular connection between the portal and systemic venous circulation, has been used as a treatment to reduce portal venous pressure. Although the use of portosystemic shunt surgery in the management of portal hypertension has declined during the past decade in favour of alternative therapies, and subsequently surgeons and radiologists became less familiar with the procedure, it remains a well-established treatment. Knowledge of different types of surgical portosystemic shunts, their pathophysiology and complications will help radiologists improve communication with surgeons and enhance their understanding of the diagnostic and therapeutic role of radiology in the assessment and management of these shunts. Optimal assessment of the shunt is essential to determine its patency and allow timely intervention. Both non-invasive and invasive imaging modalities complement each other in the evaluation of surgical portosystemic shunts. Interventional radiology plays an important role in the management of complications, such as shunt thrombosis and stenosis. This article describes the various types of surgical portosystemic shunts, explains the anatomy and pathophysiology of these shunts, illustrates the pearls and pitfalls of different imaging modalities in the assessment of these shunts and demonstrates the role of radiologists in the interventional management of complications.
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Affiliation(s)
- Bedros Taslakian
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Walid Faraj
- Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Mohammad Khalife
- Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Aghiad Al-Kutoubi
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Fadi El-Merhi
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Charbel Saade
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Ali Hallal
- Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Ali Haydar
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
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Akamatsu N, Sugawara Y, Kokudo N. Budd-Chiari syndrome and liver transplantation. Intractable Rare Dis Res 2015; 4:24-32. [PMID: 25674385 PMCID: PMC4322592 DOI: 10.5582/irdr.2014.01031] [Citation(s) in RCA: 42] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2014] [Revised: 12/23/2014] [Accepted: 12/25/2015] [Indexed: 12/13/2022] Open
Abstract
Budd-Chiari syndrome involves obstruction of hepatic venous outflow tracts at various levels from small hepatic veins to the inferior vena cava and is the result of thrombosis or its fibrous sequelae. There is a conspicuous difference in its etiology in the West and the East. Myeloproliferative disease predominates in the West and obstruction of the vena cava predominates in the East. The clinical presentation and clinical manifestations are so varied that it should be suspected in any patient with acute or chronic liver dysfunction. It should be treated with step-wise management. First-line therapy should be anticoagulation with medical treatment of the underlying illness, and interventional revascularization and TIPS are indicated in the event of a lack of response to medical therapy. Liver transplantation may be indicated as a rescue treatment or for fulminant cases with promising results. This step-by-step strategy has achieved a 5-year transplant-free survival rate of 70% and a 5-year overall survival rate of 90%. Living donor liver transplantation can also be used for patients with Budd-Chiari syndrome if deceased donor livers are scarce, but it requires a difficult procedure particularly with regard to venous outflow reconstruction.
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Affiliation(s)
- Nobuhisa Akamatsu
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yasuhiko Sugawara
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Norihiro Kokudo
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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