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Sabu J, Madapat KJ, Baby NK, Subramanian S. Obstruction of the hepatic veins-a rare case of Budd-Chiari syndrome: A case report. SAGE Open Med Case Rep 2025; 13:2050313X251324986. [PMID: 40290352 PMCID: PMC12033598 DOI: 10.1177/2050313x251324986] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2024] [Accepted: 02/14/2025] [Indexed: 04/30/2025] Open
Abstract
Budd-Chiari syndrome is a disorder that is characterized by obstruction of hepatic venous outflow, with thrombosis being the primary cause of the obstruction. This case report describes a 47-year-old Indian male presenting with distension of the abdomen and umbilical swelling for 1 year. Investigations revealed hepatic venous outflow obstruction and a partial web with focal calcification in the Inferior Vena Cava. Despite using standard medical therapeutic options such as diuretics, beta-blockers, and anticoagulation, his ascites remained uncontrolled. Interventional radiology with inferior vena cava venoplasty using 10 and 14 mm angioplasty balloons was performed, leading to the resolution of inferior vena cava stenosis and improved condition. Post-procedure, the patient was put on anticoagulation therapy and was discharged in good condition. This case highlights the successful management of Budd-Chiari syndrome with inferior vena cava occlusion using a multidisciplinary approach combining interventional radiology and medical therapy.
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Affiliation(s)
- Joel Sabu
- Internal Medicine, Father Muller Medical College, Mangaluru, India
| | | | - Namitha K Baby
- Internal Medicine, Kasturba Medical College, Mangaluru, India
| | - Supraja Subramanian
- Department of Paediatric Surgery, Institute of Child Health, Madras Medical College, Chennai, India
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Nigam N, Yadav R, Pandey G, Hashim Z, Bihari C, Vishwadeep R, Kumar N, Mishra P. The histological grading of fibrosis in Budd-Chiari syndrome: A chronic liver disease, different from others. Indian J Gastroenterol 2025; 44:188-197. [PMID: 39546183 DOI: 10.1007/s12664-024-01690-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2024] [Accepted: 09/10/2024] [Indexed: 11/17/2024]
Abstract
INTRODUCTION Budd-Chiari syndrome (BCS) is an uncommon disease caused by hepatic venous outflow obstruction. They can result in centrilobular fibrosis, nodular regenerative hyperplasia and cirrhosis. Assessing liver fibrosis is crucial for determining the stage of BCS, predicting disease progression and guiding treatment decisions. Although this pathology has been known for decades, no useful grading system was assigned. This study aims to introduce a histologic fibrosis grading system for BCS patients. METHODOLOGY All patients from 2017 to 2022 (Sanjay Gandhi Postgraduate Institute of Medical Sciences [SGPGIMS]), Lucknow diagnosed with BCS for whom liver biopsy was performed were included in the study. The Budd-Chiari syndrome-Hepatic Fibrosis system (BCS-HFS) was implemented to grade fibrosis. The fibrosis grade was compared with the fibrosis percentage area and a correlation was found with the hemodynamic variables (hepatic venous pressure gradient [HVPG]) and the prognostic scores. RESULTS There were 56 patients with BCS. The median age was 27 years, with a male-female ratio of 1.8:1. There was a significant difference in the fibrosis percentage, hemorrhage percentage and model for end-stage liver disease (MELD) score among the BCS-HFS grades (p < 0.05). There was a significant correlation between BCS-HFS and HVPG (ρ = 0.699, p < 0.001) and the MELD prognostic score (ρ = 0.474, p < 0.001). CONCLUSION BCS-HFS is applicable for grading fibrosis in BCS. It can help in uniform histopathology reporting and for further prospective and comparative studies.
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Affiliation(s)
- Neha Nigam
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India.
| | - Rajanikant Yadav
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Gaurav Pandey
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Zia Hashim
- Department of Pulmonary Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Chhagan Bihari
- Department of Pathology, Institute of Liver and Biliary Sciences, New Delhi, 110 070, India
| | - Rana Vishwadeep
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Nirbhay Kumar
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Prabhakar Mishra
- Department of Biostatistics and Health Informatics, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
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Hirayama D, Manabe S, Yuge N. Surgical Management of Infective Endocarditis Complicated by Budd-Chiari Syndrome. Cureus 2024; 16:e70979. [PMID: 39507170 PMCID: PMC11539025 DOI: 10.7759/cureus.70979] [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] [Accepted: 10/06/2024] [Indexed: 11/08/2024] Open
Abstract
Budd-Chiari syndrome (BCS) is a relatively rare comorbidity, particularly in patients undergoing cardiac surgery. The difficulty arises when we try to drain blood from the obstructed lower body circulation to establish extracorporeal circulation. Herein, we describe a patient who developed a persistent fever after undergoing neurosurgery for a head arteriovenous fistula, wherein blood cultures confirmed Staphylococcus aureus infection. The patient exhibited hyperbilirubinemia, hyperammonemia, and transient loss of consciousness. Transthoracic echocardiography showed moderate mitral regurgitation and 3 cm vegetation on the mitral valve. Imaging identified a thrombus in the right hepatic vein, stenosis of both the inferior vena cava and left hepatic vein, and esophagogastric and splenic varices. These findings led to establishing a diagnosis of infective endocarditis and BCS. The patient subsequently underwent mitral valve replacement with a mechanical valve. There are only a few case reports describing successful cardiac surgery in patients with BCS. Hence, we would like to report our surgical treatment of infective endocarditis complicated with BCS.
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Affiliation(s)
- Daiki Hirayama
- Cardiac Surgery, International University of Health and Welfare, Narita Hospital, Chiba, JPN
| | - Susumu Manabe
- Cardiac Surgery, International University of Health and Welfare, Narita Hospital, Chiba, JPN
| | - Norihisa Yuge
- Cardiac Surgery, International University of Health and Welfare, Narita Hospital, Chiba, JPN
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Berg T, Aehling NF, Bruns T, Welker MW, Weismüller T, Trebicka J, Tacke F, Strnad P, Sterneck M, Settmacher U, Seehofer D, Schott E, Schnitzbauer AA, Schmidt HH, Schlitt HJ, Pratschke J, Pascher A, Neumann U, Manekeller S, Lammert F, Klein I, Kirchner G, Guba M, Glanemann M, Engelmann C, Canbay AE, Braun F, Berg CP, Bechstein WO, Becker T, Trautwein C. [Not Available]. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2024; 62:1397-1573. [PMID: 39250961 DOI: 10.1055/a-2255-7246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/11/2024]
Affiliation(s)
- Thomas Berg
- Bereich Hepatologie, Medizinischen Klinik II, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Niklas F Aehling
- Bereich Hepatologie, Medizinischen Klinik II, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Tony Bruns
- Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Deutschland
| | - Martin-Walter Welker
- Medizinische Klinik I Gastroent., Hepat., Pneum., Endokrin. Universitätsklinikum Frankfurt, Frankfurt, Deutschland
| | - Tobias Weismüller
- Klinik für Innere Medizin - Gastroenterologie und Hepatologie, Vivantes Humboldt-Klinikum, Berlin, Deutschland
| | - Jonel Trebicka
- Medizinische Klinik B für Gastroenterologie und Hepatologie, Universitätsklinikum Münster, Münster, Deutschland
| | - Frank Tacke
- Charité - Universitätsmedizin Berlin, Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Campus Virchow-Klinikum (CVK) und Campus Charité Mitte (CCM), Berlin, Deutschland
| | - Pavel Strnad
- Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Deutschland
| | - Martina Sterneck
- Medizinische Klinik und Poliklinik I, Universitätsklinikum Hamburg, Hamburg, Deutschland
| | - Utz Settmacher
- Klinik für Allgemein-, Viszeral- und Gefäßchirurgie, Universitätsklinikum Jena, Jena, Deutschland
| | - Daniel Seehofer
- Klinik für Viszeral-, Transplantations-, Thorax- und Gefäßchirurgie, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Eckart Schott
- Klinik für Innere Medizin II - Gastroenterologie, Hepatologie und Diabetolgie, Helios Klinikum Emil von Behring, Berlin, Deutschland
| | | | - Hartmut H Schmidt
- Klinik für Gastroenterologie und Hepatologie, Universitätsklinikum Essen, Essen, Deutschland
| | - Hans J Schlitt
- Klinik und Poliklinik für Chirurgie, Universitätsklinikum Regensburg, Regensburg, Deutschland
| | - Johann Pratschke
- Chirurgische Klinik, Charité Campus Virchow-Klinikum - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Andreas Pascher
- Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Münster, Münster, Deutschland
| | - Ulf Neumann
- Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Essen, Essen, Deutschland
| | - Steffen Manekeller
- Klinik und Poliklinik für Allgemein-, Viszeral-, Thorax- und Gefäßchirurgie, Universitätsklinikum Bonn, Bonn, Deutschland
| | - Frank Lammert
- Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
| | - Ingo Klein
- Chirurgische Klinik I, Universitätsklinikum Würzburg, Würzburg, Deutschland
| | - Gabriele Kirchner
- Klinik und Poliklinik für Chirurgie, Universitätsklinikum Regensburg und Innere Medizin I, Caritaskrankenhaus St. Josef Regensburg, Regensburg, Deutschland
| | - Markus Guba
- Klinik für Allgemeine, Viszeral-, Transplantations-, Gefäß- und Thoraxchirurgie, Universitätsklinikum München, München, Deutschland
| | - Matthias Glanemann
- Klinik für Allgemeine, Viszeral-, Gefäß- und Kinderchirurgie, Universitätsklinikum des Saarlandes, Homburg, Deutschland
| | - Cornelius Engelmann
- Charité - Universitätsmedizin Berlin, Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Campus Virchow-Klinikum (CVK) und Campus Charité Mitte (CCM), Berlin, Deutschland
| | - Ali E Canbay
- Medizinische Klinik, Universitätsklinikum Knappschaftskrankenhaus Bochum, Bochum, Deutschland
| | - Felix Braun
- Klinik für Allgemeine Chirurgie, Viszeral-, Thorax-, Transplantations- und Kinderchirurgie, Universitätsklinikum Schlewswig-Holstein, Kiel, Deutschland
| | - Christoph P Berg
- Innere Medizin I Gastroenterologie, Hepatologie, Infektiologie, Universitätsklinikum Tübingen, Tübingen, Deutschland
| | - Wolf O Bechstein
- Klinik für Allgemein- und Viszeralchirurgie, Universitätsklinikum Frankfurt, Frankfurt, Deutschland
| | - Thomas Becker
- Klinik für Allgemeine Chirurgie, Viszeral-, Thorax-, Transplantations- und Kinderchirurgie, Universitätsklinikum Schlewswig-Holstein, Kiel, Deutschland
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Kimura K, Osaki A, Hirata Y, Egawa H, Kogiso T, Nakamura G, Hashidate H, Wakabayashi T, Sato M, Waguri N. A case of acute liver failure caused by Budd-Chiari syndrome salvaged by brain-dead donor liver transplantation. Clin J Gastroenterol 2024; 17:84-92. [PMID: 37773425 DOI: 10.1007/s12328-023-01863-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2023] [Accepted: 09/11/2023] [Indexed: 10/01/2023]
Abstract
A 24-year-old man was admitted to our hospital with abdominal distension. He was found to have acute liver failure and diagnosed with Budd-Chiari syndrome based on angiography and liver biopsy. Liver transplantation was deemed necessary when angiography showed extensive thrombotic occlusion of the hepatic veins and liver biopsy revealed submassive hepatic necrosis. The patient was found to have the JAK2V617F mutation, indicating a myeloproliferative neoplasm as the background disease. He developed hepatic encephalopathy but remained conscious on on-line hemodiafiltration. Brain-dead donor liver transplantation was performed on hospital day 30. Since then, the patient has remained well.
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Affiliation(s)
- Kiwamu Kimura
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan.
| | - Akihiko Osaki
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
| | - Yoshihiro Hirata
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Hiroto Egawa
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Tomomi Kogiso
- Department of Internal Medicine, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Gen Nakamura
- Department of Nephrology and Rheumatology, Niigata City General Hospital, Niigata, Japan
| | - Hideki Hashidate
- Department of Diagnostic Pathology, Niigata City General Hospital, Niigata, Japan
| | - Takuya Wakabayashi
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
| | - Munehiro Sato
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
| | - Nobuo Waguri
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
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Samanta A, Sen Sarma M, Yadav R. Budd-Chiari syndrome in children: Challenges and outcome. World J Hepatol 2023; 15:1174-1187. [PMID: 38075006 PMCID: PMC10698347 DOI: 10.4254/wjh.v15.i11.1174] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2023] [Revised: 10/08/2023] [Accepted: 10/30/2023] [Indexed: 11/24/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.
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Affiliation(s)
- Arghya Samanta
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India.
| | - Rajanikant Yadav
- Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
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7
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Shingina A, Mukhtar N, Wakim-Fleming J, Alqahtani S, Wong RJ, Limketkai BN, Larson AM, Grant L. Acute Liver Failure Guidelines. Am J Gastroenterol 2023; 118:1128-1153. [PMID: 37377263 DOI: 10.14309/ajg.0000000000002340] [Citation(s) in RCA: 64] [Impact Index Per Article: 32.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2022] [Accepted: 04/04/2023] [Indexed: 06/29/2023]
Abstract
Acute liver failure (ALF) is a rare, acute, potentially reversible condition resulting in severe liver impairment and rapid clinical deterioration in patients without preexisting liver disease. Due to the rarity of this condition, published studies are limited by the use of retrospective or prospective cohorts and lack of randomized controlled trials. Current guidelines represent the suggested approach to the identification, treatment, and management of ALF and represent the official practice recommendations of the American College of Gastroenterology. The scientific evidence was reviewed using the Grading of Recommendations, Assessment, Development and Evaluation process to develop recommendations. When no robust evidence was available, expert opinions were summarized using Key Concepts. Considering the variety of clinical presentations of ALF, individualization of care should be applied in specific clinical scenarios.
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Affiliation(s)
- Alexandra Shingina
- Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA
| | - Nizar Mukhtar
- Department of Gastroenterology, Kaiser Permanente, San Francisco, California, USA
| | - Jamilé Wakim-Fleming
- Department of Gastroenterology, Hepatology & Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic Foundation, Cleveland Ohio, USA
| | - Saleh Alqahtani
- Division of Gastroenterology and Hepatology, Johns Hopkins University, Baltimore, Maryland, USA
- Liver Transplantation Unit, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
| | - Robert J Wong
- Division of Gastroenterology and Hepatology, Stanford University School of Medicine, Palo Alto, California, Gastroenterology Section, Veterans Affairs Palo Alto Healthcare System, Palo Alto, California, USA
| | | | - Anne M Larson
- Division of Gastroenterology and Hepatology, University of Washington, Seattle, Washington, USA
| | - Lafaine Grant
- Division of Digestive and Liver Diseases, UT Southwestern Medical Center, Dallas, Texas, USA
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8
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Bova C, De Bartolo T, Pellegrini R, De Vuono A, Fiaschi E. Budd-Chiari syndrome mimicking autoimmune hepatitis. Radiol Case Rep 2023; 18:2311-2313. [PMID: 37153486 PMCID: PMC10159811 DOI: 10.1016/j.radcr.2023.03.053] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2023] [Revised: 03/22/2023] [Accepted: 03/29/2023] [Indexed: 05/09/2023] Open
Abstract
Budd-Chiari syndrome is a rare disorder characterized by obstruction of hepatic venous outflow involving either the hepatic veins or the inferior vena cava. Risk factors include thrombophilia and myeloproliferative disorders, and symptoms are often unspecific. We present a case of a 60-year-old woman admitted to our medical unit for ascites and abdominal pain. Although her medical history significant for a mixed connective tissue disease and a mild increase of the transaminases had raised the suspicion of autoimmune hepatitis, the demonstration on computed tomography of the absence of outflow in the terminal tract of the supra-hepatic veins allowed a correct diagnosis. The contribution of radiological imaging is fundamental in the diagnosis of this rare and elusive pathology.
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Affiliation(s)
- Carlo Bova
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
- Corresponding author.
| | | | - Roberta Pellegrini
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
| | - Andrea De Vuono
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
| | - Elio Fiaschi
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
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Rössle M. Interventional Treatment of Budd-Chiari Syndrome. Diagnostics (Basel) 2023; 13:diagnostics13081458. [PMID: 37189559 DOI: 10.3390/diagnostics13081458] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2023] [Revised: 04/13/2023] [Accepted: 04/15/2023] [Indexed: 05/17/2023] Open
Abstract
Medical treatment is regarded as the primary course of action in patients with Budd-Chiari syndrome (BCS). Its efficacy, however, is limited, and most patients require interventional treatment during follow-up. Short-segment stenosis or the occlusion (the so-called web) of hepatic veins or the inferior vena cava are frequent in Asian countries. An angioplasty with or without stent implantation is the treatment of choice to restore hepatic and splanchnic blood flow. The long-segment thrombotic occlusion of hepatic veins, common in Western countries, is more severe and may require a portocaval shunting procedure to relieve hepatic and splanchnic congestion. Since it was first proposed in a publication in 1993, the transjugular intrahepatic portosystemic shunt (TIPS) has gained more and more attention, and in fact it has been so successful that previously utilized surgical shunts are only used for few patients for whom it does not work. Both interventional treatment options can be performed successfully in about 95% of patients even after the complete obliteration of the hepatic veins. The long-term patency of the TIPS, a considerable problem in its early years, has been improved with PTFE-covered stents. The complication rates of these interventions are low and the survival rate is excellent with five- and ten-year survival rates of 90% and 80%, respectively. Present treatment guidelines recommend a step-up approach indicating interventional treatment after the failure of medical treatment. However, this widely accepted algorithm has several points of contention, and early interventional treatment is proposed instead.
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Affiliation(s)
- Martin Rössle
- Department of Gastroenterology, University Hospital, 79106 Freiburg, Germany
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10
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Găman MA, Cozma MA, Manan MR, Srichawla BS, Dhali A, Ali S, Nahian A, Elton AC, Simhachalam Kutikuppala LV, Suteja RC, Diebel S, Găman AM, Diaconu CC. Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature. World J Clin Oncol 2023; 14:99-116. [PMID: 37009527 PMCID: PMC10052333 DOI: 10.5306/wjco.v14.i3.99] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2022] [Revised: 02/10/2023] [Accepted: 03/01/2023] [Indexed: 03/19/2023] Open
Abstract
Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e., polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g., portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations (e.g., the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.
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Affiliation(s)
- Mihnea-Alexandru Găman
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
- Department of Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest 022328, Romania
| | - Matei-Alexandru Cozma
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
| | | | - Bahadar S Srichawla
- Department of Neurology, University of Massachusetts Chan Medical School, Worcester, Massachusetts 01655, United States
| | - Arkadeep Dhali
- Department of Internal Medicine, Nottingham University Hospitals NHS Trust, Nottingham NG7 2UH, United Kingdom
| | - Sajjad Ali
- Department of Medicine, Ziauddin University, Karachi 75600, Pakistan
| | - Ahmed Nahian
- California Baptist University-Lake Erie College of Osteopathic Medicine, Riverside, CA 92504, United States
| | - Andrew C Elton
- University of Minnesota Medical School, Minneapolis, MN 55455, United States
| | - L V Simhachalam Kutikuppala
- Department of General Surgery, Dr NTR University of Health Sciences, Andhra Pradesh, Vijayawada 520008, India
| | - Richard Christian Suteja
- Department of Undergraduate Medicine, Faculty of Medicine, Udayana University Denpasar, Bali 80232, Indonesia
| | - Sebastian Diebel
- Department of Family Medicine, Northern Ontario School of Medicine Timmins, Ontario 91762, Canada
| | - Amelia Maria Găman
- Department of Pathophysiology, University of Medicine and Pharmacy of Craiova, Romania & Clinic of Hematology, Filantropia City Hospital, Craiova 200143, Romania
| | - Camelia Cristina Diaconu
- Department of Internal Medicine, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Romania & Internal Medicine Clinic, Clinical Emergency Hospital of Bucharest, Bucharest 105402, Romania
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11
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Rana A, Jaganathan S, Ray B, Krishnan V. Improving Outcomes in Catheter-Directed Thrombolysis for the Management of Acute Budd-Chiari Syndrome: A Case Report. Cureus 2023; 15:e35976. [PMID: 37041895 PMCID: PMC10083000 DOI: 10.7759/cureus.35976] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/10/2023] [Indexed: 03/12/2023] Open
Abstract
Traditionally catheter-directed thrombolysis is performed for recanalization of hepatic vein thrombosis in acute Budd-Chiari syndrome. Successful recanalization of the hepatic veins requires a continuous infusion of the thrombolytic agent for an adequate duration due to increased resistance to blood flow in the setting of luminal thrombosis. Here, we describe a case of acute Budd-Chiari syndrome in a young female in whom prolonged catheter-directed thrombolysis of the right hepatic vein was performed for a duration of 84 hours using alteplase as the thrombolytic agent. This was followed by angioplasty and stent placement. We observed that prolonged catheter-directed thrombolysis was associated with a progressive reduction in clot burden with improved luminal patency of the hepatic vein and improved outcome of subsequent angioplasty and stenting. There was a rapid improvement in liver function tests after the procedure and liver enzymes returned to baseline within a week. A follow-up ultrasound scan showed normal blood flow and a patent lumen of the right hepatic vein. In the absence of complications, prolonged catheter-directed thrombolysis in acute Budd-Chiari syndrome can achieve adequate recanalization of the hepatic veins and improved long-term clinical outcomes. This may obviate the need for other invasive procedures like TIPS (transjugular intrahepatic portosystemic shunt)/DIPS (direct intrahepatic portosystemic shunt) and liver transplantation.
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12
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Biswas S, Sheikh S, Vaishnav M, Elhence A, Farooqui N, Anand A, Gamanagatti S, Shalimar. Pregnancy outcomes in patients with Budd-Chiari syndrome: A tertiary care experience. Indian J Gastroenterol 2023; 42:96-105. [PMID: 36738382 DOI: 10.1007/s12664-022-01307-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2021] [Accepted: 10/27/2022] [Indexed: 02/05/2023]
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) is associated with infertility and adverse pregnancy outcomes in affected females. Scant literature is available on the effect of an endovascular intervention on fertility and the outcome of future pregnancies in these patients. AIMS To assess the infertility rates, maternal and fetal outcomes of pregnancy and effect of endovascular intervention in women with BCS. METHODS In this retrospective analysis, 121 female patients with BCS attending our liver clinic from 2017 to 2020 were included. Demographic details, intervention details, pregnancies - pre- and post-intervention - and fetal outcomes were noted. RESULTS BCS was diagnosed pre-conception in 58 women (group 1; median age: 22 years), during/after pregnancy, but before completion of family in 39 (group 2; median age: 27 years), and after completion of family in 24 (group 3; median age: 34 years). Median Child-Turcotte-Pugh (CTP) and model for end-stage liver disease (MELD) scores were 7 and 12, respectively. The primary infertility rate was 19.8% (24/121). In group 1, 15 women with primary infertility underwent endovascular intervention with 5/15 (33%) women conceiving subsequently, resulting in four live births and seven abortions. In group 2, five women developed BCS during pregnancy and 11 postpartum; 11/39 had a history of one or more abortions. Overall, 8/34 (23.5%) who underwent endovascular intervention had 4/8 (50%) successful pregnancies. In group 3, no patient had any major complications during past pregnancies. The mode of delivery was vaginal in 88% of cases. No congenital anomaly/major bleeding episodes/decompensation/maternal mortality occurred. CONCLUSIONS Infertility is common in patients with BCS. Pregnancy is well-tolerated in those with compensated liver disease.
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Affiliation(s)
- Sagnik Biswas
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 110 029, India
| | - Sabreena Sheikh
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 110 029, India
| | - Manas Vaishnav
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 110 029, India
| | - Anshuman Elhence
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 110 029, India
| | | | - Abhinav Anand
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 110 029, India
| | - Shivanand Gamanagatti
- Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, 110 029, India
| | - Shalimar
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 110 029, India.
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13
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Mukhiya G, Jiao D, Han X, Zhou X, Pokhrel G. Survival and clinical success of endovascular intervention in patients with Budd-Chiari syndrome: A systematic review. J Clin Imaging Sci 2023; 13:5. [PMID: 36751561 PMCID: PMC9899460 DOI: 10.25259/jcis_130_2022] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2022] [Accepted: 01/14/2023] [Indexed: 01/26/2023] Open
Abstract
Budd-Chiari syndrome is a complex clinical disorder of hepatic venous outflow obstruction, originating from the accessory hepatic vein (HV), large HV, and suprahepatic inferior vena cava (IVC). This disorder includes both HV and IVC obstructions and hepatopathy. This study aimed to conduct a systematic review of the survival rate and clinical success of different types of endovascular treatments for Budd-Chiari syndrome (BCS). All participant studies were retrieved from four databases and selected according to the eligibility criteria for systematic review of patients with BCS. The survival rate, clinical success of endovascular treatments in BCS, and survival rates at 1 and 5 years of publication year were calculated accordingly. A total of 3398 patients underwent an endovascular operation; among them, 93.6% showed clinical improvement after initial endovascular treatment. The median clinical success rates for recanalization, transjugular intrahepatic portosystemic shunt (TIPS), and combined procedures were 51%, 17.50%, and 52.50%, respectively. The median survival rates at 1 and 5 years were 51% and 51% for recanalization, 17.50% and 16% for TIPS, and 52.50% and 49.50% for combined treatment, respectively. Based on the year of publication, the median survival rates at 1 and 5 years were 23.50% and 22.50% before 2000, 41% and 41% in 2000‒2005, 35% and 35% in 2006‒2010, 51% and 48.50% in 2010‒2015, and 56% and 55.50% after 2015, respectively. Our findings indicate that the median survival rate at 1 and 5 years of recanalization treatment is higher than that of TIPS treatment, and recanalization provides better clinical improvement. The publication year findings strongly suggest progressive improvements in interventional endovascular therapy for BCS. Thus, interventional therapy restoring the physiologic hepatic venous outflow of the liver can be considered as the treatment of choice for patients with BCS which is a physiological modification procedure.
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Affiliation(s)
- Gauri Mukhiya
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Dechao Jiao
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xinwei Han
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.,Corresponding author: Xinwei Han, Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
| | - Xueliang Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Gaurab Pokhrel
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
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14
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Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis. Sci Rep 2022; 12:16166. [PMID: 36171454 PMCID: PMC9519873 DOI: 10.1038/s41598-022-20399-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2021] [Accepted: 09/13/2022] [Indexed: 11/19/2022] Open
Abstract
This study was performed to evaluate the outcome of endovascular intervention therapy for Budd-Chiari syndrome (BCS) and compare recanalization, transjugular intrahepatic portosystemic shunt (TIPS)/direct intrahepatic portosystemic shunt (DIPS), and combined procedure treatment. For the meta-analysis, 71 studies were identified by searching four databases. The individual studies’ samples were used to calculate a confidence interval (CI 95%), and data were pooled using a fixed-effect model and random effect model. The pooled measure and an equal-weighted average rate were calculated in all participant studies. Heterogeneity between the studies was assessed with I2, and T2 tests, and publication bias was estimated using Egger’s regression test. A total of 4,407 BCS patients had undergone an endovascular intervention procedure. The pooled results were 98.9% (95% CI 97.8‒98.9%) for a technical success operation, and 96.9% (95% CI 94.9‒98.9%) for a clinical success operation. The re-intervention rate after the initial intervention procedure was 18.9% (95% CI 14.7‒22.9%), and the survival rates at 1 and 5 years after the initial intervention procedure were 98.9% (95% CI 96.8‒98.9%) and 94.9% (95% CI 92.9‒96.9%), respectively. Patients receiving recanalization treatment (98%) had a better prognosis than those with a combined procedure (95.6%) and TIPS/DIPS treatment (94.5%). The systematic review and meta-analysis further solidify the role of endovascular intervention treatment in BCS as safe and effective. It maintains high technical and clinical success and long-term survival rates. The recanalization treatment had a better prognosis and outcome than the combined procedures and TIPS/DIPS treatment.
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15
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Elkilany A, Alwarraky M, Denecke T, Geisel D. Percutaneous transluminal angioplasty for symptomatic hepatic vein-type Budd-Chiari syndrome: feasibility and long-term outcomes. Sci Rep 2022; 12:14095. [PMID: 35982064 PMCID: PMC9388522 DOI: 10.1038/s41598-022-16818-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2022] [Accepted: 07/15/2022] [Indexed: 11/09/2022] Open
Abstract
For management of Budd-Chiari syndrome (BCS), a step-wise therapeutic approach starting with medical treatment, followed by endovascular recanalization, transjugular intrahepatic portosystemic shunt, and finally liver transplantation has been adopted. We retrospectively analyzed 51 patients with symptomatic short segment (≤ 30 mm) hepatic vein (HV)-type BCS who underwent percutaneous transluminal balloon angioplasty (PTBA) with/without stenting to determine the feasibility, clinical effectiveness, and long-term outcomes. The intervention was technically successful in 94.1% of cases (48/51)—32 patients underwent PTBA and 16 patients underwent HV stenting. Procedure-related complications occurred in 14 patients (29.1%). The clinical success rate at 4 weeks was 91.7% (44/48). Nine patients underwent reintervention, six patients due to restenosis/occlusion and three patients with clinical failure. The mean primary patency duration was 64.6 ± 19.9 months (CI, 58.5–70.8; range, 1.2–81.7 months). The cumulative 1-, 2-, and 5-year primary patency rates were 85.4, 74.5, and 58.3%, respectively. The cumulative 1-, 2-, and 5-year secondary patency rates were 93.8, 87.2, and 75%, respectively. The cumulative 1-, 2-, and 5-year survival rates were 97.9, 91.5, and 50%, respectively. Percutaneous transluminal angioplasty with and without stenting is effective and achieves excellent long-term patency and survival rates in patients with symptomatic HV-type BCS. With its lower incidence of re-occlusion and higher clinical success rate, HV angioplasty combined with stenting should be the preferred option especially in patients with segmental HV-type BCS.
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Affiliation(s)
- Aboelyazid Elkilany
- Department of Diagnostic and Interventional Radiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, Berlin Institute of Health, Augustenburger Platz 1, 13353, Berlin, Germany. .,Department of Diagnostic Medical Imaging and Interventional Radiology, National Liver Institute, Menoufia University, Menoufia, Egypt.
| | - Mohamed Alwarraky
- Department of Diagnostic Medical Imaging and Interventional Radiology, National Liver Institute, Menoufia University, Menoufia, Egypt
| | - Timm Denecke
- Department of Diagnostic and Interventional Radiology, Leipzig University Hospital, Leipzig, Germany
| | - Dominik Geisel
- Department of Diagnostic and Interventional Radiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, Berlin Institute of Health, Augustenburger Platz 1, 13353, Berlin, Germany
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16
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De Bruyne R, De Bruyne P. Vascular Disorders of the Liver. TEXTBOOK OF PEDIATRIC GASTROENTEROLOGY, HEPATOLOGY AND NUTRITION 2022:931-951. [DOI: 10.1007/978-3-030-80068-0_70] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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17
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Gonçalves A. Primary Budd-Chiari Syndrome With Right Atrial Extension: A Rare Presentation of Intrahepatic Cholangiocarcinoma. Cureus 2021; 13:e18935. [PMID: 34812319 PMCID: PMC8604428 DOI: 10.7759/cureus.18935] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/20/2021] [Indexed: 11/05/2022] Open
Abstract
Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction and can be classified as primary when the obstruction is due to a predominantly venous process, caused by multiple risk factors that lead to a prothrombotic state. We report a case of a primary BCS, with an exuberant thrombus extending from the supra-hepatic vein, via the inferior vena cava, to the right atrium, a rare form of presentation of intrahepatic cholangiocarcinoma (ICC).
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Affiliation(s)
- Ana Gonçalves
- Internal Medicine, Hospital Fernando Fonseca, Amadora, PRT
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18
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Cravo M, Oliveira DG, Guimas A, Vita P, Rego AL, Alves R, Valadares D, Carvalheiras G, Ricardo M, Pinto A, Pessegueiro H, Nery F. The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari Syndrome: Case Series. J Med Cases 2021; 12:190-194. [PMID: 34434456 PMCID: PMC8383533 DOI: 10.14740/jmc3659] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2021] [Accepted: 02/01/2021] [Indexed: 11/11/2022] Open
Abstract
Budd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them presenting with acute liver injury. Progression to ALF was seen in three patients, two of whom died, with one being successfully submitted to liver transplantation. IntCUs allow a 24-h patient surveillance and a prompt management of BCS, with less economic impact when compared to ICUs. Mortality was related to the presence of associated comorbidities that limited therapeutic approach.
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Affiliation(s)
- Marcia Cravo
- Servico de Medicina Interna, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Daniel G Oliveira
- Servico de Medicina Interna, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Arlindo Guimas
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Pedro Vita
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Ana Luisa Rego
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Rute Alves
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Diana Valadares
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Graziela Carvalheiras
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Miguel Ricardo
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Alexandre Pinto
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal.,Instituto de Ciencias Biomedicas Abel Salazar-Universidade do Porto, Porto, Portugal
| | - Helena Pessegueiro
- Unidade de Transplante Hepato-Pancreatica, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Filipe Nery
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal.,Instituto de Ciencias Biomedicas Abel Salazar-Universidade do Porto, Porto, Portugal.,EpiUnit, Instituto de Saude Publica da Universidade do Porto, Porto, Portugal
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19
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Sebastian JJ, Raju R, Mathur K, Ayyappan MK. Persistent hiccups - A rare complication of suprahepatic inferior vena cava stenting. BMJ Case Rep 2021; 14:14/7/e242707. [PMID: 34285021 DOI: 10.1136/bcr-2021-242707] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Persistent hiccups has not been reported previously with suprahepatic inferior vena cava (IVC) stenting. Persistent hiccups after palliative oesophageal stenting has been reported. We present a case of a 41-year-old male patient diagnosed with primary Budd-Chiari syndrome who underwent IVC stenting for suprahepatic IVC stenosis. Patient developed transient hiccups post procedure which settled immediately with medications. Patient developed a recurrence of symptoms after a year due to stent migration which led to redo stenting. Post-procedure, the patient developed persistent hiccups which could not be controlled by physiological methods or chlorpromazine and needed baclofen at a dose of 10 mg three times a day. The patient has been symptom-free for the past 2 years. Persistent hiccups may present as a rare complication of suprahepatic IVC stenting due to extrinsic phrenic nerve compression or by direct irritation of the diaphragm. Baclofen has been effective in terminating persistent hiccups in our case.
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Affiliation(s)
- Jithin Jagan Sebastian
- Department of Vascular Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Radhakrishnan Raju
- Department of Vascular Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Kapil Mathur
- Department of Vascular Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
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20
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Asl AA, Lankarani KB, Nikeghbalian S, Kazemi K, Shamsaieefar A, Alizade-Naini M, Fattahi MR, Taghavi SA, Niknam R, Ejtehadi F, Dehghan M, Sivandzadeh G, Ghahramani S, Malek-Hosseini SA. Post liver transplant complications of Budd-Chiari syndrome. Indian J Gastroenterol 2021; 40:281-286. [PMID: 33743161 DOI: 10.1007/s12664-020-01139-3] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/18/2020] [Accepted: 12/14/2020] [Indexed: 02/05/2023]
Abstract
BACKGROUND/PURPOSE Budd-Chiari syndrome (BCS) is a rare, life-threatening disease characterized by hepatic venous outflow obstruction. Liver transplantation (LT) is widely accepted as an effective therapeutic measure for irreversible liver failure due to BCS. There is debate on differences in the post LT course and complications in patients with BCS as compared to non-Budd-Chiari (NBC) patients. METHOD In this retrospective study, data on all patients who received a liver transplant for BCS at the Shiraz Organ Transplantation Center between January 1996 and September 2017 were reviewed and compared to data of a control group who had received liver transplants over the same period but due to other causes (NBC). RESULTS Out of 4225 patients who received liver transplants in the study period, 108 had BCS and an age- and gender-matched control group consisted of 108 NBC cases. The mean ± standard deviation (SD) of model for end-stage liver disease (MELD) scores were 19.1 ± 3 and 20 ± 3 for BCS and NBC groups, respectively (p = 0.33). One-, 3-, 5-, and 10-year survival rates in the BCS group were as follows: 82%, 78%, 76%, and 76% compared with the NBC rates of 83%, 83%, 83%, and 76%, respectively (p = 0.556). There was no difference between the two groups in complication rates after 6 months. In the later period, vascular thrombosis was more common in BCS. CONCLUSIONS Whole-organ LT from deceased donors in patients with BCS had comparable outcomes with LT due to other causes of end-stage liver disease. In most instances, these patients should receive lifelong anticoagulation.
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Affiliation(s)
- Ali Ansari Asl
- Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Kamran B Lankarani
- Health Policy research center, School of Medicine, Institute of Health, Shiraz University of Medical Sciences, Shiraz, Islamic Republic of Iran.
| | - Saman Nikeghbalian
- Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Koroush Kazemi
- Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Alireza Shamsaieefar
- Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Mahvash Alizade-Naini
- Gastroenterohepatology Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Mohamad Reza Fattahi
- Gastroenterohepatology Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Seyed Alireza Taghavi
- Gastroenterohepatology Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Ramin Niknam
- Gastroenterohepatology Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Fardad Ejtehadi
- Gastroenterohepatology Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Masood Dehghan
- Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Gholamreza Sivandzadeh
- Gastroenterohepatology Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
| | - Sulmaz Ghahramani
- Health Policy research center, School of Medicine, Institute of Health, Shiraz University of Medical Sciences, Shiraz, Islamic Republic of Iran
| | - Seyed Ali Malek-Hosseini
- Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran
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21
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Li D, Shi C, Ding Z, Li X. Budd-Chiari syndrome as a complication of eosinophilic granulomatosis with polyangiitis in a young Chinese man: a case report. J Int Med Res 2021; 48:300060520964352. [PMID: 33115313 PMCID: PMC7645410 DOI: 10.1177/0300060520964352] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/18/2023] Open
Abstract
Hepatic vein thrombosis is a rare occurrence in the clinical course of eosinophilic granulomatosis with polyangiitis (EGPA). The major mechanism of thrombosis has been postulated to involve the release of toxic proteins from eosinophils. A 36-year-old man with EGPA was admitted to our hospital in July 2018 with hematemesis and melena. Findings on physical examination included ascites and pigmentation of the lower extremities. Ultrasonography of the hepatic vein and inferior vena cava showed an obstruction of the hepatic vein. Magnetic resonance imaging showed low enhancement in the right hepatic vein region. At 34 years of age, the patient's EGPA had initially presented as asthma with eosinophilia (white blood cell count of 11.46 × 1012/L with 14.6% eosinophils). His skin biopsy showed infiltration of inflammatory cells and eosinophils, especially around medium-sized vessels, which was consistent with EGPA. The patient was thus diagnosed with Budd-Chiari syndrome associated with EGPA.
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Affiliation(s)
- Dezhao Li
- Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin Province, China
| | - Chunyu Shi
- Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin Province, China
| | - Zhongyang Ding
- Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin Province, China
| | - Xu Li
- Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin Province, China
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22
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Finotti M, Auricchio P, Vitale A, Gringeri E, Cillo U. Liver transplantation for rare liver diseases and rare indications for liver transplant. Transl Gastroenterol Hepatol 2021; 6:27. [PMID: 33824931 DOI: 10.21037/tgh-19-282] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2019] [Accepted: 06/12/2020] [Indexed: 12/13/2022] Open
Abstract
Liver transplantation (LT) is currently considered the gold standard treatment for end-stage liver failure. Compared to the first decades of its use, LT is associated with lower comorbidity and mortality, with a 5-year survival over 70%. Worldwide, liver cirrhosis and hepatocellular carcinoma represent the major indications to LT. However, almost 1% of LT is performed for rare diseases or rare indications, which include non-hepatocellular malignancy, vascular disorders, metabolic and congenital liver disorders. These diseases can lead to hepatocellular necrosis, biliary tree abnormality and/or hepatomegaly. Most of these diseases are not associated with liver failure but in highly selected patients, LT represent an effective therapy improving the overall survival and quality of life. Rare indications for LT often overlap with rare diseases. However, rare LT indications for non-rare diseases are rising in the last decades, especially for benign primary liver tumor, colon rectal liver metastasis, neuroendocrine liver metastasis, and cholangiocarcinoma (CCA). Non-rare diseases with rare indication for LT and rare adult disease with an indication for LT are categorized and discussed in detail, focusing on some disorders for which the literature provides a more definitive evidence base. Early referral to a transplant center is encouraged to provide an effective therapeutic option in these non-standard indications for LT.
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Affiliation(s)
- Michele Finotti
- Department of Surgery, Oncology and Gastroenterology, Hepatobiliary Surgery and Liver Transplantation Unit, Padova University Hospital, Padova, Italy
| | - Pasquale Auricchio
- Department of Surgery, Oncology and Gastroenterology, Hepatobiliary Surgery and Liver Transplantation Unit, Padova University Hospital, Padova, Italy
| | - Alessandro Vitale
- Department of Surgery, Oncology and Gastroenterology, Hepatobiliary Surgery and Liver Transplantation Unit, Padova University Hospital, Padova, Italy
| | - Enrico Gringeri
- Department of Surgery, Oncology and Gastroenterology, Hepatobiliary Surgery and Liver Transplantation Unit, Padova University Hospital, Padova, Italy
| | - Umberto Cillo
- Department of Surgery, Oncology and Gastroenterology, Hepatobiliary Surgery and Liver Transplantation Unit, Padova University Hospital, Padova, Italy
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23
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Elhence A, Gamanagatti S, Das P. Budd Chiari Syndrome and Intrahepatic Cholangiocarcinoma, An Unusual Combination: Case Report and Review of the Literature. Perm J 2021; 24:1-3. [PMID: 33482951 DOI: 10.7812/tpp/19.204] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
INTRODUCTION Intrahepatic cholangiocarcinoma arising in the setting of Budd Chiari syndrome is uncommon and its prognostic and management implications differ from hepatocellular carcinoma. CASE PRESENTATION We report a case of intrahepatic cholangiocarcinoma in a patient with primary Budd Chiari syndrome. Hepatocellular carcinoma is known to occur with Budd Chiari syndrome. It was difficult to differentiate from hepatocellular carcinoma in the presence of increased alfa-fetoprotein levels. The contrast imaging showed features of progressive enhancement in the arterial, portal, and venous phases. A targeted liver biopsy showed histological features typical for cholangiocarcinoma. Immunostaining for cytokeratin 7 and cytokeratin 20 were positive, whereas that for arginase was negative, suggesting an intrahepatic cholangiocarcinoma. The patient was planned for inferior vena cava angioplasty followed by resection for intrahepatic cholangiocarcinoma. CONCLUSION Previously, only secondary Budd Chiari syndrome developing in the background of primary liver tumor has been described; no report exists of intrahepatic cholangiocarcinoma arising in background of primary Budd Chiari syndrome.
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Affiliation(s)
- Anshuman Elhence
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - Shivanand Gamanagatti
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - Prasenjit Das
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
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- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
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24
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Obed M, Othman MI, Hammoudi S, Chattab MA, Jarrad A, Bashir A, Obed A. Living Donor Liver Transplant in Patients With Budd-Chiari Syndrome: A Single-Center Experience at Our University Hospital. EXP CLIN TRANSPLANT 2020; 18:796-802. [PMID: 33349208 DOI: 10.6002/ect.2020.0331] [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 is an infrequent, but potentially fatal, hepatic condition with the clinical manifestation of obstructed venous drainage. This may lead to progressive hepatic congestion, portal hypertension, and, ultimately, liver failure. If medical, interventional, and surgical approaches are not effective, liver transplant offers a rescue modality. The primary objective of this study was to report the perioperative and, above all, the vascular challenges associated with living donor liver transplant in patients with Budd-Chiari syndrome. MATERIALS AND METHODS We retrospectively reviewed demographic and clinical characteristics of 6 patients with Budd-Chiari syndrome who underwent living donor liver transplant at our transplant center from April 2004 to July 2020. We also evaluated all data regarding perioperative course, surgical outcome, and the postoperative follow-up period. RESULTS All patients displayed advanced liver disease with a Child-Pugh score C. The mean calculated Model for End-Stage Liver Disease score was 32. The causes of Budd-Chiari syndrome were factor V Leiden thrombophilia in 1 patient, myeloproliferative disorder in 3 patients, antiphospholipid antibody syndrome in 1 patient, and a protein C deficiency in 1 patient. The mean age of patients was 40 years. One of the 6 patients was female. All patients had living donor liver transplant from immediate kin according to Jordanian allocation rules. The mean graft-to-recipient weight ratio was 0.9, and the median follow-up period was 89 months. Cumulative 1-, 3-, and 5-year-survival rates were 84%, 67%, and 67%, respectively. CONCLUSIONS Good survival rates are achievable with living donor liver transplant for patients with advanced Budd-Chiari syndrome, particularly by means of posterior cavoplasty for enlargement of the cava orifice. Therefore, in countries with insufficient deceased donor programs, such as Jordan, living donor liver transplant may be a lifesaving therapeutic possibility.
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Affiliation(s)
- Mikal Obed
- From the Hepatology, Gastroenterology, and Hepatobiliary/Transplant Unit Jordan Hospital, Amman, Jordan
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McGinty K. Acute Abdomen in the Oncology Patient. Semin Roentgenol 2020; 55:400-416. [PMID: 33220786 DOI: 10.1053/j.ro.2020.07.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Katrina McGinty
- Department of Radiology, UNC School of Medicine, Chapel Hill, NC.
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Budd-Chiari Syndrome: The “inferior vena cava reverse-flow” sign and “jet-blood” sign on CT and MRI. Eur J Radiol 2020; 132:109288. [DOI: 10.1016/j.ejrad.2020.109288] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2020] [Revised: 08/22/2020] [Accepted: 09/13/2020] [Indexed: 11/18/2022]
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Madhusudhan KS, Sharma S. Ultrasonography in pediatric Budd-Chiari syndrome. Pediatr Radiol 2020; 50:1768-1780. [PMID: 32870359 DOI: 10.1007/s00247-020-04753-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/03/2020] [Revised: 04/25/2020] [Accepted: 06/03/2020] [Indexed: 02/06/2023]
Abstract
Budd-Chiari syndrome is a vascular pathology of the liver, commonly seen in adults and uncommon in children. Most children with Budd-Chiari syndrome present with ascites, and an etiology is found in only about 50%. Ultrasonography (US) with color Doppler is the main modality used in the diagnosis. US imaging additionally guides radiologic interventions and follow-up after recanalization or shunt procedure. In this pictorial review, we illustrate the findings in pediatric Budd-Chiari syndrome as seen on B-mode and color Doppler US and describe the role of US in guiding percutaneous radiologic interventions, with a brief description of the role of US contrast agent and sonoelastography in this setting.
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Affiliation(s)
| | - Sanjay Sharma
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
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28
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Strozzi M, Besic KM, Ivana KS, Darko A. Endovascular treatment of an obstructive membrane between inferior vena cava and right atrium in an unrecognized Budd-Chiari syndrome. CVIR Endovasc 2020; 3:76. [PMID: 33048283 PMCID: PMC7554274 DOI: 10.1186/s42155-020-00168-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Budd-Chiari syndrome is defined as a hepatic venous outflow track obstruction of various etiology, which appears at different levels. The inferior vena cava outflow membrane is an unusual, but a potentially treatable cause. The percutaneous treatment has emerged as a very promising management mode for such patients. Follow-up results are favorable for balloon angioplasty and/or stenting, with minimal re-stenosis rates. CASE PRESENTATION We report a case of a young woman, earlier operated on congenital heart defect and with previous pulmonary embolic incident after childbirth, with no evidence of thrombophilia. She was admitted to our institution for a suspected right atrial tumor. After the diagnosis of Budd-Chiari syndrome caused by membranous inferior vena cava obstruction, a percutaneous treatment of a thick membrane was successfully performed, using an unusual technique. CONCLUSION Balloon angioplasty should be considered in cases of membranous obstruction of vena cava, where a focal obstruction is causing the symptoms. In our patient, the anatomy was not suitable for stenting, and balloon dilatation was successful just after the membrane was pulled apart with a big balloon in a "Rashkind-like" procedure.
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Affiliation(s)
- Maja Strozzi
- University Clinic for Cardiovascular Diseases, Clinical Hospital Center Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia. .,Department for Adult Congenital Heart Disease, University Clinic for Cardiovascular Diseases, Clinical Hospital Center Zagreb, Kispaticeva 12, 10000, Zagreb, Croatia.
| | - Kristina Maric Besic
- University Clinic for Cardiovascular Diseases, Clinical Hospital Center Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia
| | - Knezevic Stromar Ivana
- Department of Gastroenterology, University Clinic for Internal Medicine, University Hospital Center, University of Zagreb School of Medicine, Zagreb, Croatia
| | - Anić Darko
- University Clinic for Heart Surgery, Clinical Hospital Center Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia
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Zeng YW, Liu C, Yin MP, Zhao Y, Wang ZW, Zhou PL, Ma YZ, Li CX, Wu G. Sequential interventional therapy for Budd-Chiari syndrome associated with fresh inferior vena cava thrombosis. J Vasc Surg Venous Lymphat Disord 2020; 8:945-952. [PMID: 32418826 DOI: 10.1016/j.jvsv.2020.03.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2019] [Accepted: 03/09/2020] [Indexed: 12/29/2022]
Abstract
OBJECTIVE Our study aimed to evaluate the safety and efficacy of sequential interventional therapy for Budd-Chiari syndrome (BCS) caused by obstruction of the inferior vena cava (IVC) with fresh thrombus in the IVC. METHODS Full medical records were obtained for 20 patients with BCS associated with fresh IVC thrombus who received sequential interventional therapy from 2014 to 2019 at our hospital. All patients underwent small-diameter percutaneous transluminal angioplasty (PTA) balloon catheter predilation combined with sequential catheter-directed thrombolysis and large-diameter PTA balloon dilation. Ultrasound examinations were performed at 1 week, 1 month, 3 months, and every 6 months thereafter. Therapeutic effects and perioperative and postoperative adverse effects were recorded to assess the safety of the treatment. RESULTS All 20 patients were treated with small PTA balloon catheters (diameter, 10-14 mm) to predilate the occlusive segment of the IVC. Urokinase 400,000 to 600,000 (465,000 ± 93,000) units was administered to patients through the catheter for 6 to 20 (9.7 ± 4.2) consecutive days postoperatively. Ultrasound re-examination showed that the IVC thrombus disappeared completely in 14 patients (70.0%), and a small amount of the old thrombus remained in 6 patients (30.0%). After thrombolysis, all 20 patients received PTA balloon dilation (diameter, 26-30 mm) in the stenosed IVC segment, and blood flow recovered subsequently. No pulmonary embolism or death occurred in the perioperative course. The perioperative survival rate was 100.0%. CONCLUSIONS Sequential interventional therapy for BCS associated with fresh IVC thrombus is safe and effective.
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Affiliation(s)
- Yan-Wei Zeng
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Chao Liu
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Mei-Pan Yin
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yue Zhao
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Zhi-Wei Wang
- Department of Vascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Peng-Li Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yao-Zhen Ma
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Chun-Xia Li
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Gang Wu
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
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Dongaonkar RM, Quick CM, Laine GA, Uray K, Cox CS, Stewart RH. Adaptation of the hepatic transudation barrier to sinusoidal hypertension. Am J Physiol Regul Integr Comp Physiol 2020; 318:R722-R729. [PMID: 32023079 DOI: 10.1152/ajpregu.00178.2019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
Abstract
The role of the hepatic transudation barrier in determining ascites volume and protein content in chronic liver disease is poorly understood. Therefore, the purpose of the present study was to characterize how chronic sinusoidal hypertension impacts hepatic transudation barrier properties and the transudation rate. The suprahepatic inferior vena cava was surgically constricted, and animals were exposed to either short-term (SVH; 2-3 wk) or long-term venous hypertension (LVH; 5-6 wk). Compared with SVH, LVH resulted in lower peritoneal fluid pressure, ascites volume, and ascites protein concentration. The transudation barrier protein reflection coefficient was significantly higher, and the transudation barrier hydraulic conductivity, transudation rate, and transudate-to-lymph protein concentration ratio were significantly lower in LVH animals compared with SVH animals. The sensitivity of transudation rates to acute changes in interstitial fluid pressures was also significantly lower in LVH animals compared with SVH animals. In contrast, there was no detectable difference in hepatic lymph flow rate or sensitivity of lymph flow to acute changes in interstitial fluid pressures between SVH and LVH animals. Taken together, these data suggest that decreased hepatic transudation barrier permeability to fluid and protein and increased reflection coefficient led to a decrease in the hepatic contribution to ascites volume. The present work, to the best of our knowledge, is the first to quantify an anti-ascites adaptation of the hepatic transudation barrier in response to chronic hepatic sinusoidal hypertension.
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Affiliation(s)
- Ranjeet M Dongaonkar
- Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Department of Veterinary Physiology and Pharmacology, Texas A & M University, College Station, Texas
| | - Christopher M Quick
- Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Department of Veterinary Physiology and Pharmacology, Texas A & M University, College Station, Texas
| | - Glen A Laine
- Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Department of Veterinary Physiology and Pharmacology, Texas A & M University, College Station, Texas
| | - Karen Uray
- Department of Medical Chemistry, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
| | - Charles S Cox
- Department of Pediatric Surgery, University of Texas Medical School, Houston, Texas
| | - Randolph H Stewart
- Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Department of Veterinary Physiology and Pharmacology, Texas A & M University, College Station, Texas
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Payancé A, Plessier A. Anticoagulation for Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:131-145. [DOI: 10.1007/978-981-32-9232-1_10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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32
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Malik I, Bhatia V, Kumar K, Sibal A, Goyal N. Pediatric Hepatic Venous Outflow Tract Obstruction: Experience from a Transplant Center. Indian Pediatr 2019. [PMID: 31729328 DOI: 10.1007/s13312-019-1654-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
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Cheng DL, Xu H, Li CL, Lv WF, Li CT, Mukhiya G, Fang WW. Interventional Treatment Strategy for Primary Budd-Chiari Syndrome with Both Inferior Vena Cava and Hepatic Vein Involvement: Patients from Two Centers in China. Cardiovasc Intervent Radiol 2019; 42:1311-1321. [PMID: 31218407 DOI: 10.1007/s00270-019-02267-w] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/28/2018] [Accepted: 06/11/2019] [Indexed: 01/18/2023]
Abstract
OBJECTIVE This retrospective study evaluated interventional treatments (recanalization, balloon dilation, and/or stent placement) for Budd-Chiari syndrome (BCS), caused by combined obstruction of the inferior vena cava (IVC) and hepatic veins (HVs). METHODS Before and after interventional therapy, patients with BCS (n = 162; asymptomatic 105.2 ± 103.3 mo; follow-up 15 [6-24] mo) underwent imaging studies (color Doppler ultrasound, CT, or MRI), and inferior vena cavography and manometry. Venous lesions were characterized by occlusion features, and presence of thrombosis and peripheral collateral vessels. RESULTS One, 2, and 3 main HV occlusions were observed, respectively, in 25 (15.4%), 61 (37.7%), and 76 (46.9%) patients. Eighty-three (51.2%), 98 (60.5%), and 104 (64.2%) patients had, respectively, large accessory HVs, venous collaterals formed between the HVs, or venous communicating branches between the HV and the peritoneal veins. The middle, left, and right HV was patent in 32 (19.8%), 35 (21.6%), and 44 (27.2%) patients. Recanalization of both hepatic and caval occlusions was successful in 96% (51/53) of those attempted; recanalization of IVC occlusion was successful in 97% (106/109). Among 157 patients successfully treated, 146 were cured and 11 showed clinical improvement. Clinical symptoms were relieved in 82.4% after the initial intervention, and 94.2% after the second intervention. CONCLUSION Recanalization and balloon angioplasty was effective for the management of BCS with concurrent HV and IVC occlusions. The majority of patients required only IVC recanalization. The outcome of patients treated only by IVC intervention was similar to that of patients given combined HV and IVC intervention.
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Affiliation(s)
- De-Lei Cheng
- Department of Interventional Radiology, The First Affiliated Hospital of USTC (Anhui Provincial Hospital), Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, 230001, Anhui, People's Republic of China.,Shandong Medical Imaging Research Institute, Shandong University, No. 324, Jing Five Road, Jinan, 250021, Shandong, People's Republic of China
| | - Hao Xu
- Department of Interventional Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, 221006, Jiangsu, People's Republic of China
| | - Cheng-Li Li
- Shandong Medical Imaging Research Institute, Shandong University, No. 324, Jing Five Road, Jinan, 250021, Shandong, People's Republic of China
| | - Wei-Fu Lv
- Department of Interventional Radiology, The First Affiliated Hospital of USTC (Anhui Provincial Hospital), Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, 230001, Anhui, People's Republic of China
| | - Chuan-Ting Li
- Shandong Medical Imaging Research Institute, Shandong University, No. 324, Jing Five Road, Jinan, 250021, Shandong, People's Republic of China.
| | - Gauri Mukhiya
- Zhengzhou University, Zhengzhou, 450001, Henan, People's Republic of China
| | - Wei-Wei Fang
- Department of Interventional Radiology, The First Affiliated Hospital of USTC (Anhui Provincial Hospital), Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, 230001, Anhui, People's Republic of China
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Khan F, Armstrong MJ, Mehrzad H, Chen F, Neil D, Brown R, Cain O, Tripathi D. Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome. Aliment Pharmacol Ther 2019; 49:840-863. [PMID: 30828850 DOI: 10.1111/apt.15149] [Citation(s) in RCA: 34] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2018] [Revised: 11/06/2019] [Accepted: 12/29/2018] [Indexed: 12/13/2022]
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. AIM To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. METHODS Analysis of recent literature by using Medline, PubMed and EMBASE databases. RESULTS Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma. CONCLUSIONS With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome.
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Affiliation(s)
- Faisal Khan
- Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Matthew J Armstrong
- Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
- NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, UK
- Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
| | - Homoyon Mehrzad
- Imaging and Interventional Radiology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Frederick Chen
- NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, UK
- Department of Clinical Haematology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Desley Neil
- Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Rachel Brown
- Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Owen Cain
- Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Dhiraj Tripathi
- Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
- NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, UK
- Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
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Castro L, Nazal L, Silva G, Brahm J, Segovia R, Ferrario M, Buckel E, Uribe M, Puelma F, Regueira T, Reccius A, Fardella P, Rodríguez MDLÁ, Blamey R. MANEJO DE LA FALLA HEPÁTICA FULMINANTE: PROPUESTA DE PROTOCOLO EN CLÍNICA LAS CONDES. REVISTA MÉDICA CLÍNICA LAS CONDES 2019. [DOI: 10.1016/j.rmclc.2019.03.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
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Shukla A, Jain A, Kahalekar V, Bendkhale S, Gogtay N, Thatte U, Bhatia S. Mutations in CYP2C9 and/or VKORC1 haplotype are associated with higher bleeding complications in patients with Budd-Chiari syndrome on warfarin. Hepatol Int 2019; 13:214-221. [PMID: 30617764 DOI: 10.1007/s12072-018-9922-6] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/29/2018] [Accepted: 12/18/2018] [Indexed: 12/12/2022]
Abstract
INTRODUCTION Anticoagulation is universally recommended in Budd-Chiari syndrome [BCS]. Vitamin K epoxide reductase complex 1 (VKORC1) and CYP2C9 are involved in the metabolism of warfarin. The present study was done to assess whether these mutations are associated with the risk of bleeding in patients with BCS receiving warfarin. PATIENTS AND METHODS Patients diagnosed with BCS underwent genotyping for three single nucleotide polymorphisms [SNPs]-two for the CYP2C9 and one for the VKORC1 haplotype. The patients were followed up for at least 12 months and all bleeding episodes were recorded. Patients with and without mutations were compared for bleeding complications and a crude odds ratio [crude OR] was derived for the association between bleeding and presence or absence of mutant alleles. RESULTS Eighty patients [mean (SD) age 27.47 (8.93) years, 35 male] with BCS underwent genetic testing. 37/80 (46.2%) patients had mutation of CYP2C9 and/or VKORC1; 22/80 (27.5%) had either of the mutant alleles of CYP2C9 and, similarly, 22/80 (27.5%) had the VKORC mutation. Over a median follow-up of 20 (range 12-96) months, 21/80 (26.3%) patients had bleeding complications. Patients with mutant SNPs had a higher risk of bleeding than those without [14/37 vs. 7/43, p = 0.04, crude OR (95% CI) 3.13 (1.1-8.9)]. CONCLUSION The presence of mutations in VKORC1 or CYP2C9 is associated with increased risk of bleeding in patients with BCS on warfarin. Such patients with SNPs of CY2C9 or VKORC1 haplotype should be monitored intensively while receiving warfarin.
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Affiliation(s)
- Akash Shukla
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India.
| | - Abhinav Jain
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India
| | - Vinit Kahalekar
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India
| | - Sheetal Bendkhale
- Department of Clinical Pharmacology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India
| | - Nithya Gogtay
- Department of Clinical Pharmacology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India
| | - Urmila Thatte
- Department of Clinical Pharmacology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India
| | - Shobna Bhatia
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, 400012, India
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Patients With Budd-Chiari Syndrome Have Variable Coagulation Status on Thromboelastography at Diagnosis. J Clin Exp Hepatol 2019; 9:460-467. [PMID: 31516262 PMCID: PMC6728540 DOI: 10.1016/j.jceh.2018.10.002] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2017] [Accepted: 10/18/2018] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND & AIMS Budd-Chiari Syndrome (BCS) is considered a thrombophilic state, and most patients with BCS have thrombophilic disorder. Liver dysfunction-related coagulopathy makes coagulation function unpredictable in BCS. Thromboelastography (TEG) assesses the dynamics, strength, and stability of clot formation. We conducted a pilot study using TEG to evaluate coagulation status in patients with BCS. METHODS Fifty-one patients with newly diagnosed BCS (age 32.3 [10.7] years; 23 men) underwent TEG (TEG®5000 Hemostasis Analyzer®, USA), and its components were analyzed and correlated with clinical profile and thrombophilic disorders. Patients who had received anticoagulation, antiplatelet drugs, or radiological intervention were excluded. RESULTS Twenty-nine patients had normal TEG, 11 had procoagulant TEG, and 11 had hypocoagulant TEG. Among patients with hypocoagulant TEG, Coagulation Index (CI) was < -3 in 11 patients, R was >8 min in 6 patients, K was >3 min in 9 patients, alpha <55 in 9 patients, and MA <51 in 7 patients; among those with hypercoagulant TEG, CI was >3 in 3 patients, R < 2 min in 2 patients, K <1 min in 2 patients, alpha >78 in none, and MA >69 mm in 7 patients. TEG findings were similar in patients with and without thrombophilic disorder. The mean platelet count (1.75, 2.22, and 1.79 × 105/mm3; P = 0.13) and international normalized ratio (1.27, 1.34, and 1.28, P = 0.69) were similar in those with procoagulant, normal, and hypocoagulant TEG. Two patients in Rotterdam class-III had abnormal LY30. Other clinical parameters did not correlate with TEG findings. CONCLUSION Patients with BCS are heterogeneous with respect to coagulation status, with one-fifth of patients are hypocoagulant on TEG. Patients with advanced disease may have accelerated fibrinolysis.
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Key Words
- BCS, Budd-Chiari Syndrome
- CT, Computed Tomography
- CTP, Child-Turcotte-Pugh
- INR, International Normalized Ratio
- IVC, Inferior Vena Cava
- JAK-2, Janus Kinase-2
- MELD, Model for End-Stage Liver Disease
- MRI, Magnetic Resonance Imaging
- MTHFR, Methylene tetrahydrofolatereductase
- PNH, Paroxysmal Nocturnal Hemoglobinuria
- PT, Prothrombin Time
- TEG, Thromboelastography
- aPTT, Partial Thromboplastin Time
- cirrhosis
- gastrointestinal hemorrhage
- hepatic venous outflow tract obstruction
- portal hypertension
- varices
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Dongaonkar RM, Stewart RH, Quick CM, Uray KL, Cox CS, Laine GA. Hepatic transudation barrier properties. Microcirculation 2018; 25. [PMID: 29047195 DOI: 10.1111/micc.12424] [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: 12/06/2016] [Accepted: 10/12/2017] [Indexed: 01/22/2023]
Abstract
OBJECTIVE Fluid and protein continuously transude from the surface of the liver. Despite a common understanding that transudation plays a critical role in hepatic interstitial and peritoneal fluid balance, transudation from the entire liver has not been studied. Therefore, the goal of the present work was to provide the first direct measurement of the hepatic transudation rate and transudation barrier properties. METHODS Transudation rates were determined by collecting transudate from the entire liver. Hydraulic conductivity, and fluid transudation and protein reflection coefficients of the transudation barrier (formed by the subscapular interstitial matrix, capsule, and peritoneum) were determined from changes in fluid and protein transudation rates in response to hepatic venous pressure elevation. RESULTS Following hepatic venous pressure elevation from 6.1 ± 0.9 to 11.1 ± 0.6 mm Hg, transudation rate increased from 0.13 ± 0.03 to 0.37 ± 0.03 mL/min·100 g. Transudation barrier hydraulic conductivity, fluid transudation and protein reflection coefficients (3.9 × 10-4 ± 5.7 × 10-5 mL/min·mm Hg·cm2 , 0.36 ± 0.04 mL/min·mm Hg, and 0.09 ± 0.03, respectively) were comparable to those reported for hepatic sinusoids. CONCLUSIONS Taken together, these findings suggest that the hepatic transudation barrier is highly permeable at elevated sinusoidal pressures. These fundamental studies provide a better understanding of the hepatic transudation barrier properties and transudation under conditions that are physiologically and clinically relevant to ascites formation.
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Affiliation(s)
- Ranjeet M Dongaonkar
- Department of Veterinary Physiology & Pharmacology, Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Texas A&M University, College Station, TX, USA
| | - Randolph H Stewart
- Department of Veterinary Physiology & Pharmacology, Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Texas A&M University, College Station, TX, USA
| | - Christopher M Quick
- Department of Veterinary Physiology & Pharmacology, Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Texas A&M University, College Station, TX, USA
| | - Karen L Uray
- Department of Medical Chemistry, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
| | - Charles S Cox
- Department of Pediatric Surgery, The University of Texas Medical School, Houston, TX, USA
| | - Glen A Laine
- Department of Veterinary Physiology & Pharmacology, Michael E. DeBakey Institute for Comparative Cardiovascular Science and Biomedical Devices, Texas A&M University, College Station, TX, USA
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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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Bi Y, Chen H, Ding P, Zhou P, Han X, Ren J. Excellent long-term outcomes of endovascular treatment in budd-chiari syndrome with hepatic veins involvement: A STROBE-compliant article. Medicine (Baltimore) 2018; 97:e12944. [PMID: 30412110 PMCID: PMC6221688 DOI: 10.1097/md.0000000000012944] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2018] [Accepted: 10/01/2018] [Indexed: 12/23/2022] Open
Abstract
This study aimed to evaluate the long-term efficacy and safety of percutaneous transhepatic balloon angioplasty (PTBA) and transjugular intrahepatic portosystemic stent-shunt (TIPSS) in the treatment of Budd-Chiari syndrome (BCS) with hepatic veins involvement. Between June 2008 and August 2016, a total of 60 BCS patients with hepatic vein involvement in our department were enrolled in this study. Thirty-three cases underwent hepatic vein balloon angioplasty in PTBA Group and 27 cases underwent TIPSS. Data were retrospectively collected, and follow-up observations were performed. TIPSS Group showed significantly higher thrombotic/segmental obstruction and peripheral stenosis/obstruction compared with PTBA Group. The success rates were 93.9% and 100.0% in PTBA Group and TIPSS Group, respectively. The mean portal vein pressure decreased significantly after stenting. Except for 1 patient died from repeated hemorrhage, other sever complications had not been observed in both group. Twenty-six patients and 21 patients were clinically cured in PTBA Group and TIPSS Group, respectively. The primary patency rates were 89.7%, 79.3%, and 79.3% for short-term, mid-term and longterm in PTBA Group, which were significantly higher than TIPSS Group for long-term follow up. The second patency rates were 100.0%, 96.6% and 96.6% for short-term, mid-term and long-term in PTBA Group, which were similar to TIPSS Group (P = 1.0000). In conclusion, PTBA and TIPSS are safe and effective in the treatment of BCS with hepatic veins involvement, with an excellent long-term patency rate of hepatic vein and TIPSS shunt. TIPSS can be used to treat patients with all 3 hepatic veins lesion and failure PTBA.
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Affiliation(s)
- Yonghua Bi
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Hongmei Chen
- Department of Ultrasound, Zhengzhou Central Hospital Affiliated to Zhengzhou University, Zhengzhou, China
| | - Pengxu Ding
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Pengli Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Xinwei Han
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Jianzhuang Ren
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
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Image-guided treatment of Budd-Chiari syndrome: a giant leap from the past, a small step towards the future. Abdom Radiol (NY) 2018; 43:1908-1919. [PMID: 28988356 DOI: 10.1007/s00261-017-1341-y] [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] [Indexed: 02/06/2023]
Abstract
Budd-Chiari syndrome (BCS) is a relatively rare vascular disease characterized by hepatic outflow tract obstruction, and image-guided endovascular treatment, namely percutaneous angioplasty, stenting, and transjugular intrahepatic portosystemic shunt (TIPS), has proven to be effective treatment modalities to alleviate symptoms and markedly improve the prognosis of the disease. Specifically, a step-wise approach is recommended, i.e., angioplasty and stenting are the prioritized choice for patients with membranous obstruction and short-length stenosis, whereas TIPS is the option for patients who fail this treatment. Currently, 5-year survival with the step-wise approach is about 75%, and the most promising way to further improve this value is to identify candidates who are at high risk of failing angioplasty, and perform pre-emptive TIPS in these patients.
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Bi Y, Chen H, Ding P, Zhou P, Ren J, Han X. Percutaneous Transhepatic Hepatic Vein Angioplasty in Budd-Chiari Syndrome After Transvenous Failure. J Laparoendosc Adv Surg Tech A 2018; 28:1346-1351. [PMID: 29851361 DOI: 10.1089/lap.2018.0156] [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] [Indexed: 12/12/2022] Open
Abstract
PURPOSE To evaluate the safety and efficacy of percutaneous transhepatic route creation for hepatic vein (HV) angioplasty in Budd-Chiari syndrome (BCS). PATIENTS AND METHODS Between April 2012 and August 2016, a total of 19 BCS patients underwent percutaneous transhepatic route creation for HV angioplasty after transvenous catheterization failure in this study. Color Doppler ultrasonography was required in all patients after admission and during follow-up. Data were retrospectively collected, and follow-up observations were performed. RESULTS Technical and clinical success was achieved in 18 patients. Except for 1 failure of route creation, 19 routes were successfully created in 18 patients, with a technology success rate of 95.0%. Twenty-two balloon angioplasties were performed in 18 patients, with a mean balloon diameter of 13.6 ± 0.5 mm. Blood pressure and length of occlusive HV decreased significantly, and blood flow velocity and diameter of HV increased significantly after procedure. Abdominal distension/pain and ascites decreased significantly after procedure. One procedure-related death occurred, who died of gastrointestinal bleeding 6 days later. Except for the failure case, the rest of 18 patients were successfully followed up. The remaining 18 patients survived during follow-up, with a 5-year survival rate of 94.9%. One patient had a restenosis of HV after 47 days, and had undergone successful dilation. The 5-year primary and second patency rates were 94.1% and 100%, respectively. CONCLUSION Percutaneous transhepatic route creation is safe and effective for HV angioplasty, and can be used to treat BCS patients after transvenous catheterization failure.
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Affiliation(s)
- Yonghua Bi
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Hongmei Chen
- 2 Department of Ultrasound, Zhengzhou Central Hospital Affiliated to Zhengzhou University , Zhengzhou, China
| | - Pengxu Ding
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Pengli Zhou
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Jianzhuang Ren
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Xinwei Han
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
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Angioplasty for Budd-Chiari Syndrome in a Child with 26-Year Follow-up. Ann Vasc Surg 2018; 51:328.e7-328.e12. [PMID: 29777844 DOI: 10.1016/j.avsg.2018.03.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2017] [Revised: 02/10/2018] [Accepted: 03/05/2018] [Indexed: 11/23/2022]
Abstract
Budd-Chiari syndrome (BCS) is a rare but life-threatening disease. If not treated promptly, it is almost always lethal with progressive liver failure, severe nutritional depletion, and renal failure at the late stage. We report the successful treatment of a 5-year-old boy with BCS due to a stenosis of the inferior vena cava (IVC) with a 26-year follow-up. We performed a percutaneous transluminal angioplasty, which resulted in a great improvement of BCS and the disappearance of ascites. Restenosis occurred at 25 years after the initial angioplasty, for which balloon angioplasty was repeatedly performed. The IVC was patent at 1-year follow-up after the second angioplasty.
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Abstract
Acute liver failure (ALF) is a rare but highly fatal condition. The most common causes include drug-induced and viral hepatitis, but other less common etiologies, especially autoimmune hepatitis, Budd-Chiari syndrome, and Wilson disease, need to be considered. Because diagnosis is frequently tied to potential for reversibility of ALF and prognosis, early identification in a timely manner is crucial. Other causes of ALF are more easily recognizable based on specific circumstances, such as ALF in pregnancy or ischemic hepatitis. Ultimately, maintaining a wide differential diagnosis in patients with ALF is essential to identifying the proper treatment and prognosis.
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Affiliation(s)
- Russell Rosenblatt
- Division of Gastroenterology and Hepatology, Weill Cornell Medical College, 1305 York Avenue, 4th Floor, New York, NY 10021, USA
| | - Robert S Brown
- Division of Gastroenterology and Hepatology, Weill Cornell Medical College, 1305 York Avenue, 4th Floor, New York, NY 10021, USA.
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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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Buechter M, Manka P, Gerken G, Canbay A, Blomeyer S, Wetter A, Altenbernd J, Kahraman A, Theysohn JM. Transjugular Intrahepatic Portosystemic Shunt in Patients with Portal Hypertension: Patency Depends on Coverage and Interventionalist's Experience. Dig Dis 2018; 36:218-227. [PMID: 29316565 DOI: 10.1159/000486030] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/13/2017] [Accepted: 11/29/2017] [Indexed: 02/02/2023]
Abstract
BACKGROUND AND AIMS Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in decompensated portal hypertension. TIPS revision due to thrombosis or stenosis increases morbidity and mortality. Our aim was to investigate patient- and procedure-associated risk factors for TIPS-revision. PATIENTS AND METHODS We retrospectively evaluated 189 patients who underwent the TIPS procedure. Only patients who required TIPS revision within 1 year (Group I, 34 patients) and patients who did not require re-intervention within the first year (Group II [control group], 54 patients) were included. RESULTS Out of 88 patients, the majority were male (69.3%) and mean age was 56 ± 11 years. Indications for TIPS were refractory ascites (68%), bleeding (24%), and Budd-Chiari syndrome (8%). The most frequent liver disease was alcohol-induced cirrhosis (60%). Forty-three patients (49%) received bare and 45 patients (51%) covered stents, thus resulting in reduction of hepatic venous pressure gradient (HVPG) from 19.0 to 9.0 mm Hg. When comparing patient- and procedure-related factors, the type of stent (p < 0.01) and interventionalist's experience (number of performed TIPS implantations per year; p < 0.05) were the only factors affecting the risk of re-intervention due to stent dysfunction, while age, gender, indication, Child-Pugh, and model of end-stage liver disease score, platelet count, pre- and post-HVPG, additional variceal embolization, stent diameter, and number of stents did not significantly differ. CONCLUSION Patients undergoing TIPS procedure should be surveilled closely for shunt dysfunction while covered stents and high-level experience are associated with increased -patency.
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Affiliation(s)
- Matthias Buechter
- Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany
| | - Paul Manka
- Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.,Regeneration and Repair, Institute of Hepatology, Division of Transplantation Immunology and Mucosal Biology, Faculty of Life Sciences and Medicine, King's College London, London, United Kingdom
| | - Guido Gerken
- Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany
| | - Ali Canbay
- Department of Gastroenterology, Hepatology and Infectious Diseases, Otto-von-Guericke University, Magdeburg, Germany
| | - Sandra Blomeyer
- Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany
| | - Axel Wetter
- Department of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany
| | - Jens Altenbernd
- Department of Radiology and Neuroradiology, Klinikum Vest Knappschaftskrankenhaus Recklinghausen, Recklinghausen, Germany
| | - Alisan Kahraman
- Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany
| | - Jens M Theysohn
- Department of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany
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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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Living donor liver transplantation for adult Budd Chiari syndrome - Resection without replacement of retrohepatic IVC: A case report. Int J Surg Case Rep 2017; 42:50-54. [PMID: 29216531 PMCID: PMC5724988 DOI: 10.1016/j.ijscr.2017.11.050] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2017] [Revised: 11/23/2017] [Accepted: 11/28/2017] [Indexed: 01/03/2023] Open
Abstract
Budd-Chiari syndrome is characterized by hepatic venous outflow obstruction. Liver transplantation is a definitive treatment of BCS, indicated in failure of other treatment modalities or end stage liver diseases. The Budd-Chiari syndrome patients with IVC obstruction can receive liver transplantation with IVC removal and without IVC replacement. Living donor liver transplant with refined techniques can be performed safety with stable hemodynamics peri-and post-liver transplantation. Introduction Suprahepatic caval resection and replacement of inferior vena cava (IVC) is standard procedure in deceased donor liver transplantation for patients with Budd-Chiari syndrome (BCS). However, replacement of IVC in living donor liver transplantation (LDLT) is difficult. We report a case of BCS successfully treated by LDLT without replacement of IVC. Presentation of case A 52-years-old female with a primary BCS due to IVC thrombosis. A vena cava (VC) stent placed after angioplasty without improvement of the hepatic, portal venous flow and liver functions, Transjugular intrahepatic portosystemic shunt was considered and the patient had a rapid deterioration and increased ascites. The patient was scheduled for living donor liver transplantation (LDLT). Her Child-Paugh and MELD scores were 11, 18, respectively at time of transplantation. Left lobe was obtained from her son. Preservation of the native suprarenal IVC was impossible due to massive fibrosis and thrombosed. The suprahepatic IVC was also fibrotic and unsuitable for anastomosis with hepatic vein. The retrohepatic IVC resected include suprahepatic IVC together with the liver. The supradiaphragmatic IVC was reached and encircled through opening the diaphragm around the IVC and a vascular clamp applied on the right atrium with subsequent anastomosis with hepatic vein of the graft. The hemodynamic stability of the patient was maintained throughout the operation without IVC replacement due to developed collateral vessels. Conclusion Patients with Budd-Chiari syndrome with obstructive IVC are successfully treated with living donor liver transplantation without replacement of IVC.
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Martín-Llahí M, Albillos A, Bañares R, Berzigotti A, García-Criado MÁ, Genescà J, Hernández-Gea V, Llop-Herrera E, Masnou-Ridaura H, Mateo J, Navascués CA, Puente Á, Romero-Gutiérrez M, Simón-Talero M, Téllez L, Turon F, Villanueva C, Zarrabeitia R, García-Pagán JC. Enfermedades vasculares del hígado. Guías Clínicas de la Sociedad Catalana de Digestología y de la Asociación Española para el Estudio del Hígado. GASTROENTEROLOGIA Y HEPATOLOGIA 2017; 40:538-580. [PMID: 28610817 DOI: 10.1016/j.gastrohep.2017.03.011] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/13/2017] [Accepted: 03/29/2017] [Indexed: 12/11/2022]
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