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Shimizu T, Yoshioka M, Ueda J, Kawashima M, Irie T, Kawano Y, Matsushita A, Taniai N, Mamada Y, Yoshida H. Stenting of Inferior Right Hepatic Vein in a Patient with Budd-Chiari Syndrome: A Case Report. J NIPPON MED SCH 2024; 91:119-123. [PMID: 37271547 DOI: 10.1272/jnms.jnms.2023_90-603] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/06/2023]
Abstract
A Japanese man in his 20s was referred to our hospital with a two-month history of abdominal fullness and leg edema. Abdominal computed tomography revealing massive ascites and ostial blockage of the main hepatic veins, and angiographic evaluation demonstrating obstruction of the main hepatic veins yielded a diagnosis of Budd-Chiari syndrome (BCS). Diuretic agents were prescribed for the ascites but failed to provide relief. The patient was referred to our department for further evaluation and treatment. Angiography showed ostial obstruction of the main hepatic veins, with most of the portal hepatic flow draining from an inferior right hepatic vein (IRHV) into the inferior vena cava (IVC) thorough an intrahepatic portal venous and venovenous shunt. Access between the main hepatic veins and IVC was impossible, but cannulation between the IRHV and IVC was achieved. Because of the venovenous connection between the main hepatic vein and the IRHV, metallic stents were placed into two IRHVs to decrease congestion in the hepatic venous outflow. After stent placement followed by balloon expansion, the gradient pressure between the hepatic vein and IVC improved remarkably. The ascites and lower leg edema improved postoperatively, and long-term stent patency (6 years) was achieved.
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Affiliation(s)
- Tetsuya Shimizu
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Masato Yoshioka
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Junji Ueda
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Mampei Kawashima
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Toshiyuki Irie
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Yoichi Kawano
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Akira Matsushita
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Nobuhiko Taniai
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Yasuhiro Mamada
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
| | - Hiroshi Yoshida
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School
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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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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: 8] [Impact Index Per Article: 4.0] [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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Mancuso A. Budd-Chiari Syndrome Management: Controversies and Open Issues. Diagnostics (Basel) 2022; 12:2670. [PMID: 36359513 PMCID: PMC9689902 DOI: 10.3390/diagnostics12112670] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2022] [Revised: 10/26/2022] [Accepted: 11/01/2022] [Indexed: 07/03/2024] Open
Abstract
Budd-Chiari Syndrome (BCS) is due to thrombosis of hepatic veins (HVs), inferior vena cava (IVC) or both, leading to impaired hepatic venous outflow [...].
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Affiliation(s)
- Andrea Mancuso
- Centro di Riferimento Regionale Malattie Rare, Sindrome di Budd-Chiari e Teleangectasia Emorragica Ereditaria, Medicina Interna 1, ARNAS Civico-Di Cristina-Benfratelli, Piazzale Leotta 4, 90100 Palermo, Italy
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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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Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An Update on the Management of Budd-Chiari Syndrome. Dig Dis Sci 2021; 66:1780-1790. [PMID: 32691382 DOI: 10.1007/s10620-020-06485-y] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Accepted: 07/04/2020] [Indexed: 12/17/2022]
Abstract
Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.
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Affiliation(s)
- A Sharma
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India
| | - S N Keshava
- Department of Interventional Radiology, Christian Medical College, Vellore, India
| | - A Eapen
- Department of Radiodiagnosis, Christian Medical College, Vellore, India
| | - E Elias
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.,Liver Unit, University Hospitals Birmingham, Birmingham, UK
| | - C E Eapen
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.
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Sharma A, Goel A, Moses V, Keshava SN, Zachariah UG, Elias E, Eapen CE. Anticoagulating Budd-Chiari syndrome patients presenting with variceal bleed: A retrospective study. J Gastroenterol Hepatol 2020; 35:1397-1403. [PMID: 31900982 DOI: 10.1111/jgh.14971] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/15/2019] [Revised: 12/21/2019] [Accepted: 01/02/2020] [Indexed: 12/09/2022]
Abstract
BACKGROUND AND AIM This aims to study incidence of re-bleeding on anticoagulation and survival of Budd-Chiari syndrome (BCS) patients presenting with variceal bleeding. METHODS Budd-Chiari syndrome patients presenting with variceal bleed between 01/01/2007 and 01/05/2019 were retrospectively studied. Patients underwent endoscopic treatment ± endovascular therapy, followed by anticoagulation. Variceal re-bleed (on anticoagulation) and survival were studied. RESULTS Of 376 BCS patients diagnosed during the study period, 40 (10.7%) patients, presenting with variceal bleed (age 33 [25-40] years; male patients 70%; Rotterdam score 1.13 [0.63-1.22]), Group 1 were compared with 40 randomly selected age-matched BCS patients presenting with ascites, no bleeds (40 [23-42] years; male patients 42.5%; Rotterdam score 1.11 [1.09-1.16]), Group 2. The commonest site of obstruction was hepatic vein (65%) in Group 1 and combined hepatic veins and inferior vena cava (57.5%) in Group 2 (P < 0.01). Thirty-six Group 1 patients underwent endoscopic intervention (variceal ligation, 33; sclerotherapy, 2; glue injection, 1). Endovascular intervention was performed in 30 Group 1 patients (angioplasty ± stent, 22; endovascular shunt, 8) and in 34 Group 2 patients (angioplasty ± stent, 26; endovascular shunt, 8). All 80 patients were started on anticoagulation. Variceal bleed on anticoagulation occurred in five patients in Group 1 and three patients in Group 2. One-year and 5-year survival were 94.2% and 87.5%, respectively, in Group 1 and 100% and 80%, respectively, in Group 2. CONCLUSIONS About one-tenth of BCS patients present with variceal bleed. On management with endoscopic ± endovascular therapy, followed by anticoagulation, variceal re-bleed in these patients were comparable with those in BCS patients presenting with ascites and survival was excellent at 1 and 5 years.
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Affiliation(s)
- Anand Sharma
- Department of Hepatology, Christian Medical College, Vellore, India
| | - Ashish Goel
- Department of Hepatology, Christian Medical College, Vellore, India
| | - Vinu Moses
- Department of Radiology, Christian Medical College, Vellore, India
| | | | | | - Elwyn Elias
- Department of Hepatology, Christian Medical College, Vellore, India.,Liver Unit, University Hospital Birmingham, Birmingham, UK
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Mukund A, Taunk A. Percutaneous Recanalization for Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:147-156. [DOI: 10.1007/978-981-32-9232-1_11] [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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9
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Mancuso A. Controversies in the Management of Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:245-252. [DOI: 10.1007/978-981-32-9232-1_19] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Jiménez Jiménez C, Randial Tagliapetra L, Silva I, Alférez F. Tratamiento endovascular del síndrome de Budd-Chiari, a propósito de un caso. REVISTA COLOMBIANA DE CIRUGÍA 2019; 34:386-393. [DOI: 10.30944/20117582.518] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2025] Open
Abstract
El síndrome de Budd-Chiari consiste en la oclusión de la circulación de salida venosa del hígado, desde las sinusoides hepáticas hasta la unión de la vena cava inferior con la aurícula derecha. Esta entidad es muy rara. Las causas son primarias, obstrucción congénita de la vena cava o trombosis espontánea, o secundarias, compresión extrínseca. Si no se trata la obstrucción esta enfermedad, lleva a cirrosis hepática.
Se presenta un caso del síndrome de Budd-Chiari de causa primaria por obstrucción membranosa, tratado por vía endovascular de manera exitosa y con resolución de todos los síntomas. Es el primer reporte en Colombia de este tipo de tratamiento.
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Nakatsuka T, Soroida Y, Nakagawa H, Okura N, Sato J, Akahane M, Sato M, Yatomi Y, Abe O, Tateishi R, Koike K. Utility of hepatic vein waveform and transient elastography in patients with Budd-Chiari syndrome who require angioplasty: Two case reports. Medicine (Baltimore) 2019; 98:e17877. [PMID: 31702657 PMCID: PMC6855573 DOI: 10.1097/md.0000000000017877] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
RATIONALE Budd-Chiari syndrome (BCS), which causes congestive hepatopathy and aggravates cirrhosis, is typically treated by interventional angioplasty to ameliorate blood flow. X-ray venography is useful for the evaluation of inferior vena cava (IVC) stenosis and determination of treatment timing, but it is invasive and thus unsuitable for repeated examinations. The development of a simple method for the prediction of IVC stenosis would reduce the burden on patients with BCS. PATIENT CONCERNS We report here our experience of 2 patients with BCS who underwent percutaneous transluminal angioplasty (PTA). The first patient was a 39-year-old male who underwent PTA to expand his stenotic IVC. The second patient was a 19-year-old male who underwent PTA 3 times due to restenosis of his IVC. DIAGNOSES Both patients were diagnosed with BCS with severe obstruction of the IVC. INTERVENTIONS We evaluated the hepatic vein (HV) waveform by Doppler ultrasonography and measured liver stiffness (LS) using transient elastography (TE) before and after PTA. OUTCOMES In case 1, the phasic oscillation of the HV waveform recovered and the LS value decreased after PTA. Both improvements were maintained for ∼3 years, reflecting the long-term patency of the IVC. In case 2, the HV waveform and the LS value improved temporarily after PTA, but then deteriorated gradually. Monitoring of the HV waveform and LS value allowed retreatment prior to total occlusion of the IVC and abrogated the risk of intravascular needle puncture. LESSONS Monitoring of the HV waveform and the LS value enables safe management of patients with BCS who may require PTA.
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Affiliation(s)
- Takuma Nakatsuka
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Yoko Soroida
- Department of Clinical Laboratory Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Hayato Nakagawa
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Naoki Okura
- Department of Radiology, International University of Health and Welfare, School of Medicine, Minato-Ku
| | - Jiro Sato
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Masaaki Akahane
- Department of Radiology, International University of Health and Welfare, School of Medicine, Minato-Ku
| | - Masaya Sato
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
- Department of Clinical Laboratory Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Yutaka Yatomi
- Department of Clinical Laboratory Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Osamu Abe
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Ryosuke Tateishi
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Kazuhiko Koike
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
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Kageyama K, Yamamoto A, Jogo A, Nota T, Murai K, Ogawa S, Nakano MM, Sohgawa E, Hamamoto S, Hamuro M, Kaminou T, Nishida N, Takahashi K, Yamamoto K, Miki Y. Usefulness of Venous Pressure Measurement in Endovascular Treatment of Budd-Chiari Syndrome: A Retrospective Cohort Study. Intern Med 2019; 58:2923-2929. [PMID: 31243212 PMCID: PMC6859396 DOI: 10.2169/internalmedicine.2704-19] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023] Open
Abstract
Objective Therapeutic predictors derived from the venous pressure before therapy have not been identified for Budd-Chiari syndrome (BCS). The aim of this study was to determine whether or not measuring the distal pressure or pressure gradient was useful for predicting treatment efficacy in BCS. Methods We retrospectively analyzed seven consecutive patients diagnosed with symptomatic BCS at our hospital between 2008 and 2017. Distal and proximal venous pressures at occlusion sites of BCS were measured before treatment in all cases. The pressure gradient was defined as the difference between distal and proximal venous pressures. A receiver operating characteristics (ROC) analysis was performed for venous pressures. Results Percutaneous old balloon angioplasty (POBA) was performed in seven cases, with technical success achieved in all cases (100%). No complications were encountered. The median primary patency was 574 (interquartile range, 439.5-1,056.5) days. The 1-year primary patency rate was 71.73%. Six cases (85.7%) showed resolution of symptoms, representing clinical success. The ROC analysis revealed a high distal pressure (area under the ROC curve = 0.83, cut-off=12 mmHg) as a predictor of treatment efficacy of POBA for symptomatic BCS. In addition, the pressure gradient was considered significant from a clinical perspective, because the 6 successful cases with resolution of symptoms showed a large pressure gradient (range, 8-21 mmHg) before treatment, whereas the failed case showed a relatively small pressure gradient (7 mmHg). Conclusion High distal pressure and a large pressure gradient might predict the treatment efficacy of balloon angioplasty for symptomatic BCS.
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Affiliation(s)
- Ken Kageyama
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Akira Yamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Atsushi Jogo
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Takehito Nota
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Kazuki Murai
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Satoyuki Ogawa
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Mariko M Nakano
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Etsuji Sohgawa
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Shinichi Hamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Masao Hamuro
- Department of Radiology, Izumiotsu Municipal Hospital, Japan
| | - Toshio Kaminou
- Department of Radiology, National Hospital Organization Osaka Minami Medical Center, Japan
| | - Norifumi Nishida
- Department of Radiology, Osaka Saiseikai Nakatsu Hospital, Japan
| | - Kanae Takahashi
- Department of Medical Statistics, Osaka City University Graduate School of Medicine, Japan
| | - Kouji Yamamoto
- Department of Medical Statistics, Osaka City University Graduate School of Medicine, Japan
| | - Yukio Miki
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
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Josyabhatla R, Hsu D, McGuire M, D'Mello S. Thrombotic Storm With Budd-Chiari Syndrome in a Pediatric Patient With Ulcerative Colitis. ACG Case Rep J 2019; 6:e00159. [PMID: 31832449 PMCID: PMC6855525 DOI: 10.14309/crj.0000000000000159] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2018] [Accepted: 07/10/2019] [Indexed: 11/30/2022] Open
Abstract
Ulcerative colitis is associated with an increased risk of thromboembolic phenomena. Thrombotic storm defined by the development of multiple thrombi in multiple locations within a short period of time is a rare condition that is potentially life threatening. We present a 14-year-old adolescent boy with an ulcerative colitis flare complicated by Budd-Chiari syndrome and thrombotic storm.
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Affiliation(s)
| | - Diane Hsu
- Department of Pediatrics, Rutgers NJMS, Newark, NJ
| | - Michael McGuire
- Division of Pediatric Radiology, Hackensack University Medical Center, Hackensack, NJ
| | - Sharon D'Mello
- Division of Pediatric Gastroenterology, Hackensack University Medical Center, Hackensack, NJ
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Wang Q, Li K, He C, Yuan X, Luo B, Qi X, Guo W, Bai W, Yu T, Fan J, Wang Z, Yuan J, Li X, Zhu Y, Han N, Niu J, Lv Y, Liu L, Li J, Tang S, Guo S, Wang E, Xia D, Wang Z, Cai H, Wang J, Yin Z, Xia J, Fan D, Han G. Angioplasty with versus without routine stent placement for Budd-Chiari syndrome: a randomised controlled trial. Lancet Gastroenterol Hepatol 2019; 4:686-697. [PMID: 31279647 DOI: 10.1016/s2468-1253(19)30177-3] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/01/2019] [Revised: 04/18/2019] [Accepted: 04/23/2019] [Indexed: 02/07/2023]
Abstract
BACKGROUND Angioplasty recanalisation is recommended as the first-line interventional procedure for Budd-Chiari syndrome, but subsequent restenosis is common. We aimed to test whether use of routine, non-selective stenting in angioplasty could improve patency and treatment efficacy with adequate safety in Budd-Chiari syndrome. METHODS We did a randomised controlled trial, for which patients aged 18-75 years with Budd-Chiari syndrome with membranous obstruction or short-length stenosis (≤4 cm), and a Child-Pugh score of less than 13 were considered eligible. Patients were excluded if they had obstruction not amenable to angioplasty, were recommended to be treated with transjugular intrahepatic portosystemic shunt or liver transplantation, or had contraindications for angioplasty. Eligible patients were randomly assigned (1:1) to an angioplasty-only group or an angioplasty plus routine stenting group, with use of a web-based allocation system (Pocock and Simon's minimisation method, stratified by obstruction features and Child-Pugh score). Recanalisation procedures were done within 24 h of randomisation. The statistician and investigators responsible for data collection data and endpoint assessment were masked to group allocation. The primary outcome was the proportion of patients free of restenosis, analysed in the intention-to-treat population. The study is registered on ClinicalTrials.gov (NCT02201485) and is completed. FINDINGS Between July 28, 2014, and Sept 29, 2017, 88 (59%) of 150 screened patients were enrolled and assigned either the angioplasty-only group (n=45) or the angioplasty plus routine stenting group (n=43). During a median follow-up period of 27 months (IQR 19-41), the angioplasty plus routine stenting group had significantly higher proportion of patients free of restenosis (42 [98%] of 43 patients) than did the angioplasty-only group (27 [60%] of 45 patients; p<0·0001). In the survival analysis, 3-year restenosis-free survival was 96·0% (95% CI 88·6-100·0) in the routine stenting group versus 60·4% (46·4-78·7) in the angioplasty-only group (log-rank p<0·0001). The hazard ratio for restenosis was 0·04 (95% CI 0·01-0·31) in favour of routine stenting, with an absolute risk reduction of 35·6% (95% CI 24·2-55·0). Two (5%) patients in the angioplasty plus routine stenting group and one (2%) patient in the angioplasty-only group died during follow-up. One (2%) patient from the angioplasty plus routine stenting group had puncture site haematoma, which was not related to stenting. No stent fracture or migration occurred. Anticoagulation-related adverse events occurred in five (11%) patients from angioplasty alone group and five (12%) patients from angioplasty plus routine stenting group. INTERPRETATION Routine stenting with angioplasty is superior to angioplasty alone for preventing restenosis in patients with Budd-Chiari syndrome with short-length stenosis and is safe to use as part of first-line invasive treatment. Further validation is needed in similar settings and other regions in which different characteristics of Budd-Chiari syndrome are more prevalent. FUNDING National Natural Science Foundation of China, National Key Technology R&D Programme, Optimised Overall Project of Shaanxi Province, Boost Programme of Xijing Hospital.
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Affiliation(s)
- Qiuhe Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Kai Li
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Chuangye He
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Xulong Yuan
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Bohan Luo
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Xingshun Qi
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Wengang Guo
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Wei Bai
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Tianlei Yu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jiahao Fan
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Zhengyu Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jie Yuan
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Xiaomei Li
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Ying Zhu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Na Han
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jing Niu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Yong Lv
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Lei Liu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jing Li
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Shihao Tang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Shuai Guo
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Enxing Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Dongdong Xia
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Zhexuan Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Hongwei Cai
- Department of Medical Statistics, School of Preventive Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China; Information Technology Department, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
| | - Jianhong Wang
- Department of Ultrasound, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Zhanxin Yin
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jielai Xia
- Department of Medical Statistics, School of Preventive Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Daiming Fan
- State Key Laboratory of Cancer Biology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Guohong Han
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, 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] [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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Bi Y, Chen H, Ding P, Ren J, Han X. Comparison of retrievable stents and permanent stents for Budd-Chiari syndrome due to obstructive inferior vena cava. J Gastroenterol Hepatol 2018; 33:2015-2021. [PMID: 29851172 DOI: 10.1111/jgh.14295] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/09/2018] [Revised: 05/13/2018] [Accepted: 05/18/2018] [Indexed: 12/09/2022]
Abstract
BACKGROUND AND AIM The aim of this study is to compare long-term outcomes of retrievable stents (RSs) and permanent stents (PSs) for Budd-Chiari syndrome (BCS) due to long-segment obstructive inferior vena cava (IVC). METHODS Between July 2000 and August 2016, 42 patients with BCS due to long-segment obstructive IVC were treated with RSs, and 41 patients were treated with PSs. The RSs were removed eventually after thrombus disappeared. Patients were subsequently followed up by color Doppler sonography or computed tomography scanning. RESULTS All RS placements were successful, and 37 RSs were retrieved 8 to 29 days later. Forty-two stents were implanted in PS group. One failure retrieval of RSs occurred, and two failures of cannulations were found in PS group. Two deaths may be procedure related and died from acute pulmonary thromboembolism perioperatively. One patient developed acute cerebral infarction and recovered after treatment. In PS group, minor complications were found in three patients. The length of IVC lesion segment and length and thickness of IVC thrombus decreased significantly, and diameter of retrocaval IVC and diaphragm IVC increased significantly in both groups. During follow-up, three patients died from liver failure in RS group, and two patients died in PS group. RS group showed a significantly higher primary patency rate than PS group. Cumulative 1-, 3-, and 5-year secondary patency rates were 95.2%, 89.6%, and 89.6% in RS group and 100%, 96.6%, and 96.6% in PS group (P = 0.7109). CONCLUSIONS Retrievable stents are effective for BCS because of long-segment obstructive IVC, with a higher primary patency rate.
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Affiliation(s)
- Yonghua Bi
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - 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, Zhengzhou, China
| | - Jianzhuang Ren
- 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
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18
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Yagi T, Takagi K, Yoshida R, Umeda Y, Nobuoka D, Kuise T, Fujiwara T, Takaki A. New Left Lobe Transplantation Procedure with Caval Reconstruction Using an Inverted Composite Graft for Chronic Budd-Chiari Syndrome in Living-Donor Liver Transplantation-A Case Report. Transplant Proc 2018; 50:1192-1195. [PMID: 29731092 DOI: 10.1016/j.transproceed.2017.11.078] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2017] [Accepted: 11/21/2017] [Indexed: 02/07/2023]
Abstract
When the Budd-Chiari syndrome (BCS) lesion extends to the inferior vena cava (IVC) or the orifices of the hepatic vein, the thickened IVC and/or hepatic vein wall must be removed and IVC reconstruction is required in living-donor liver transplantation (LDLT). In various reports about IVC resection in LDLT for BCS, there are none about left lobe liver transplantation with reconstruction of the retrohepatic IVC (rhIVC). To overcome removal and reconstruction of the rhIVC in LDLT for BCS, we introduced a composite IVC graft that is applicable to both right and left lobe partial liver grafts for LDLT for BCS. Pathogenic IVC was removed together with the native liver between the lower edge of the right atrium and 5 cm above the renal vein junction with the use of venovenous bypass. The e-polytetrafluoroethylene graft was anastomosed to the suprarenal intact IVC. Then the native part was detached at the level of just above the renal junction. The composite graft was inverted and a half rim of the native part of the graft was anastomosed to the posterior wall of the right atrium. Next, the common venous orifice of the left lobe graft was anastomosed to the wall defect which was composed of the anterior wall of the right atrium and the distal end of the native part of the composite graft. In conclusion, our inverted composite graft technique will overcome the weak points of LDLT for BCS, such as incomplete removal of the pathogenic caval wall and reconstruction of the rhIVC.
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Affiliation(s)
- T Yagi
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan.
| | - K Takagi
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - R Yoshida
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - Y Umeda
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - D Nobuoka
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - T Kuise
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - T Fujiwara
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - A Takaki
- Departments of Gastroenterology, Transplant Surgery, and Surgical Oncology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
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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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Wang HW, Shi HN, Cheng J, Xie F, Luo YK, Tang J. Real-time shear wave elastography (SWE) assessment of short- and long-term treatment outcome in Budd-Chiari syndrome: A pilot study. PLoS One 2018; 13:e0197550. [PMID: 29847588 PMCID: PMC5976180 DOI: 10.1371/journal.pone.0197550] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2016] [Accepted: 04/19/2018] [Indexed: 12/12/2022] Open
Abstract
Purpose The first aim of this study was to analyze the relationships between liver stiffness measurement, hepatic venous pressure and liver fibrosis. The second aim was to demonstrate the utility of real-time shear wave elastography for evaluation of Budd-Chiari syndrome patients before and after balloon hepatic venous angioplasty. Materials and methods A total of 32 patients with Budd-Chiari syndrome slated for successful balloon angioplasty met the inclusion and exclusion criteria. Shear wave elastography was used to generate dynamic liver stiffness measurement 2 days before angioplasty and 2 days, 3 months, and 6 months after angioplasty. Hepatic venous pressures were measured during balloon angioplasty. Correlations among liver stiffness, hepatic venous pressure, and fibrosis were assessed. Result Mean liver stiffness was 35.17 ± 10.60 kPa, 20.15 ± 5.47 kPa, 15.36 ± 4.34 kPa and 15.68 ± 5.58 kPa at baseline and 2 days, 3 months, and 6 months after angioplasty, respectively. Liver stiffness measured at 2 days and 3 months after angioplasty was significantly decreased (P < 0.001); liver stiffness measured at 6 months after angioplasty was not significantly different from that measured at 3 months after angioplasty (P = 0.636). Analysis of liver stiffness measurement and hepatic venous pressure before balloon angioplasty yielded a coefficient of correlation r = 0.701 (P < 0.001). Before and 2d after angioplasty, liver stiffness measurement did not correlated with fibrosis (r = − 0.170, P = 0.22), (r = 0.223, P = 0.220), respectively, while the LSM difference before and 2 days after angioplasty negatively correlated with stiffness severity (r = − 0.502, P = 0.003). Liver stiffness measured at 2 days and 3 months after angioplasty was significantly decreased (P < 0.001), remaining stable at 3 months, though still in the cirrhotic range. Conclusions The liver stiffness of Budd-Chiari syndrome patients, measured by shear wave elastography, decreased considerably after hepatic venous recanalization, and significantly correlated with hepatic venous pressure though not with degree of fibrosis. Shear wave elastography may be effective in monitoring short- and long-term treatment outcomes in Budd-Chiari syndrome.
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Affiliation(s)
- Hong-Wei Wang
- Department of Ultrasound, Chinese PLA General Hospital, Beijing, China
- Department of Ultrasound, Yong Liang Hospital, Baoding, China
| | - Hua-Ning Shi
- Department of Ultrasound, Affiliated Hospital of Chengde Medical College, Chengde, China
| | - Jia Cheng
- Department of Ultrasound, Chinese PLA General Hospital, Beijing, China
| | - Fang Xie
- Department of Ultrasound, Chinese PLA General Hospital, Beijing, China
| | - Yu-Kun Luo
- Department of Ultrasound, Chinese PLA General Hospital, Beijing, China
- * E-mail: (YKL); (JT)
| | - Jie Tang
- Department of Ultrasound, Chinese PLA General Hospital, Beijing, China
- * E-mail: (YKL); (JT)
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Long-term Outcome of Recoverable stents for Budd-Chiari syndrome Complicated with Inferior Vena Cava Thrombosis. Sci Rep 2018; 8:7393. [PMID: 29743653 PMCID: PMC5943331 DOI: 10.1038/s41598-018-25876-w] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2017] [Accepted: 04/26/2018] [Indexed: 11/12/2022] Open
Abstract
This study aimed to present long-term results of a 12-year patient follow-up of recoverable stents for BCS complicated by inferior vena cava (IVC) thrombosis. Forty consecutive patients with BCS complicated by IVC thrombosis were treated with recoverable stents. The median duration of symptoms was 24 months. Recoverable stents was placed after predilation of the obstructed IVC, and then agitation thrombolysis or catheter-directed thrombolysis of IVC was performed. The recoverable stents was removed eventually after thrombus disappeared. Clinical patency was defined as absence or improvement of symptoms. Patients were subsequently followed-up by color Doppler ultrasound. Recoverable stents placement, balloon angioplasty and thrombolysis were technically successful in all patients. Stents were successfully removed in 92.1% of patients. A few serious related complications including one acute pulmonary thromboembolism, one stent migration, and one failure retrieval stents occurred. The median follow-up was 43.7 months. The long-term results were satisfactory except 2 patients who presented with a restenosis or re-obstruction and underwent additional therapy. There were 5 deaths owing to pulmonary embolism or underlying malignant disease 0.4–101.8 months after the procedures, including one procedure-related death. In conclusion, Recoverable stents treatment is safe and effective for BCS complicated by IVC thrombosis, with a good long-term outcome.
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Mukund A, Mittal K, Mondal A, Sarin SK. Anatomic Recanalization of Hepatic Vein and Inferior Vena Cava versus Direct Intrahepatic Portosystemic Shunt Creation in Budd-Chiari Syndrome: Overall Outcome and Midterm Transplant-Free Survival. J Vasc Interv Radiol 2018; 29:790-799. [PMID: 29705227 DOI: 10.1016/j.jvir.2018.01.781] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/24/2017] [Revised: 01/23/2018] [Accepted: 01/24/2018] [Indexed: 02/07/2023] Open
Abstract
PURPOSE To assess overall outcome and midterm transplant-free survival of patients with Budd-Chiari syndrome (BCS) undergoing radiologic interventions including anatomic recanalization of the hepatic vein (HV) and inferior vena cava (IVC) and direct intrahepatic portosystemic shunt (DIPS) creation, both as combined and as independent groups. MATERIALS AND METHODS From November 2010 to October 2014, 136 patients with BCS were treated with HV/IVC recanalization (group 1) or DIPS creation (group 2). Both groups were periodically analyzed for stent patency on Doppler ultrasound, clinical outcome, biochemical parameters, and survival until death, liver transplantation, or last clinical evaluation. RESULTS Actuarial transplant-free survival for the entire cohort was 94% at 1 year and 5 years with no significant difference in overall survival. There was significant biochemical improvement in group 1 with decrease in mean serum bilirubin level (1.8 mg/dL to 1.4 mg/dL, P < .011), mean serum aspartate aminotransferase (48.6 IU/L to 33.2 IU/L, P < .05), and mean serum alanine aminotransferase (38.7 IU/L to 28.5 IU/L) and increase in mean serum albumin level (3.2 g/dL to 3.45 g/dL, P < .001) after 3 and 24 months. There were 4 deaths in each group at 1-year follow-up; all 4 patients had acute fulminant BCS at presentation. CONCLUSIONS Radiologic interventions for BCS lead to remarkable improvement of liver function and a good overall outcome and midterm transplant-free survival. Patients receiving anatomic recanalization show improved liver synthetic functions compared with patients treated with DIPS.
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Affiliation(s)
- Amar Mukund
- Department of Radiology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi 110070, India.
| | - Kartik Mittal
- Department of Radiology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi 110070, India
| | - Aniket Mondal
- Department of Radiology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi 110070, India
| | - Shiv Kumar Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi 110070, India
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23
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Mancuso A. Timing of Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: An Italian Hepatologist's Perspective. J Transl Int Med 2017; 5:194-199. [PMID: 29340275 PMCID: PMC5767708 DOI: 10.1515/jtim-2017-0033] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident. Recent data support that in BCS liver fibrosis could arise from chronic microvascular ischemia. A reasoning model of BCS physiopathology is that impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development and causes hepatic fibrosis and liver failure through chronic ischemic damage. On this assumption is the concept that relieving liver congestion could ameliorate liver function and prevent development of BCS complications. Recently, early interventional treatment with TIPS for BCS has been reported to be effective. Early TIPS seems to be the best option for BCS management. Future multicenter controlled studies should compare the outcome of BCS treated with early interventional treatment compared with stepwise strategy.
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Affiliation(s)
- Andrea Mancuso
- Medicina Interna 1, ARNAS Civico - Di Cristina - Benfratelli, Piazzale Leotta 4, Palermo, Italy
- Epatologia e Gastroenterologia, Ospedale Niguarda Ca’ Granda, Piazza Ospedale Maggiore 3, 20162Milano, Italy
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Abstract
AIM Outcomes of endovascular intervention in Budd-Chiari syndrome (BCS) have been reported with varied results. Clinical outcomes of endovascular interventions in BCS and role of various prognostic scores were critically evaluated in this study. METHODS This study retrospectively analyzed consecutive patients of BCS who underwent endovascular intervention between January 2007 and May 2016 at our center. Technical, clinical successes and complications were documented. The role of the prognostic scores such as Child-Turcotte-Pugh (CTP), model for end-stage liver disease (MELD), Rotterdam index, and original Clichy score in predicting mortality, clinical success, and need for re-interventions were also assessed. RESULTS A total of 88 patients were analyzed. The median follow up was 12 months (range 1-96 months). Thirteen (14.8%) patients had combined inferior vena cava (IVC) and hepatic vein (HV) obstruction; HV obstruction in 33 (37.5%) and inferior vena cava IVC obstruction in 42 (47.7%) patients. The following interventions were done: IVC angioplasty alone (n = 11), IVC angioplasty with stenting (n = 36), HV angioplasty with stenting (n = 26), combined HV and IVC stent (n = 2), and direct intrahepatic porto-systemic shunt (DIPS) (n = 13). Overall technical success was 87/88 (98.86%), and clinical success was 76/88 (86.36%). Immediate complications were noted in 8 patients (10%). The 1-, 2-, 3-, and 4-year stent patency rates were 90.91%, 81.08%, 74.59%, and 70.45%, respectively. Re-interventions were required in 15 (17%). Overall mortality was 6 (6.8%). Apart from MELD >14, none of the other prognostic score could predict mortality, clinical success, and need for re-interventions. CONCLUSION Endovascular interventions play an important role in the management of BCS, in properly selected patients, even if prognostic score is unfavorable.
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Seif HMAH, Abu Rahma MZ, Zaky S, Swifee YM. Transjugular intrahepatic porto-systemic shunt in bleeding esophageal varices and refractory ascites. The first 4years experience in Assiut University Hospital. THE EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE 2016. [DOI: 10.1016/j.ejrnm.2016.05.016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022] Open
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Outcome of the Z-expandable metallic stent for Budd-Chiari syndrome and segmental obstruction of the inferior vena cava. Eur J Gastroenterol Hepatol 2016; 28:972-9. [PMID: 27172449 DOI: 10.1097/meg.0000000000000640] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
OBJECTIVE Treatment of segmental obstruction of the inferior vena cava (SOIVC) with Z-expandable metallic stents (Z-EMS) is controversial and data on long-term follow-up are lacking. We aimed to evaluate the long-term outcomes of the use of a Z-EMS for Budd-Chiari syndrome (BCS) patients with SOIVC. MATERIALS AND METHODS Between August 2004 and December 2014, 37 consecutive BCS patients with SOIVC were referred for Z-EMS treatment and subsequently underwent follow-up in our department. Data were collected retrospectively and follow-up observations were made 1, 2, 2-5, and 5-10 years postoperatively. RESULTS Percutaneous transluminal balloon angioplasty and Z-EMS placement were technically successful in all patients. Major procedure-related complications occurred in four of 37 patients (10.81%). Follow-up for 61.89±41.45 months in 37 patients indicated portal hypertension in one patient 4 months after stent placement and symptoms were resolved by transjugular intrahepatic portosystemic stent shunting. Hepatocellular carcinoma was observed in four patients and five patients died during follow-up. Reocclusion of the inferior vena cava occurred in four patients (10.81%, 4/41) and all reocclusions were managed by percutaneous transluminal balloon angioplasty. Cumulative 1-, 2-, 2-5-, and 5-10-year primary patency rates were 94.60% (35/37), 93.33% (28/30), 88.89% (24/27), and 85.0% (17/20), respectively. Cumulative 1-, 2-, 2-5-, and 5-10-year secondary patency rates were 100% at all time-points. CONCLUSION These data suggest that Z-EMS implantation is an efficacious, safe, and curative approach for BCS with SOIVC because satisfactory long-term outcomes were achieved. Long-term follow-up is required to ascertain stent patency and hepatocellular carcinoma occurrence.
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Li WD, Yu HY, Qian AM, Rong JJ, Zhang YQ, Li XQ. Risk factors for and causes and treatment of recurrence of inferior vena cava type of Budd-Chiari syndrome after stenting in China: A retrospective analysis of a large cohort. Eur Radiol 2016; 27:1227-1237. [DOI: 10.1007/s00330-016-4482-4] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2016] [Revised: 06/07/2016] [Accepted: 06/21/2016] [Indexed: 12/18/2022]
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Shalimar, Kumar A, Kedia S, Sharma H, Gamanagatti SR, Gulati GS, Nayak B, Thakur B, Acharya SK. Hepatic venous outflow tract obstruction: treatment outcomes and development of a new prognostic score. Aliment Pharmacol Ther 2016; 43:1154-67. [PMID: 27060876 DOI: 10.1111/apt.13604] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2016] [Revised: 01/29/2016] [Accepted: 03/10/2016] [Indexed: 12/20/2022]
Abstract
BACKGROUND Results of endovascular interventions in hepatic venous outflow tract obstruction (HVOTO) have been reported from limited studies. Treatment outcomes and prognostic scores need further validation. AIM To evaluate treatment outcomes and prognostic scores for hepatic venous outflow tract obstruction in an Indian population. METHODS Consecutive patients with hepatic venous outflow tract obstruction diagnosed at a tertiary centre were included. Technical success and clinical response after endovascular interventional therapy were documented. Predictors of survival were assessed with Cox-proportional model. A new score was derived from the factors significant on multivariate analysis and compared with Child-Turcotte-Pugh, model for end-stage liver disease (MELD), Rotterdam prognostic index (PI) and Budd-Chiari syndrome-transjugular intrahepatic portosystemic shunt ( BCS-TIPSS) PI. RESULTS Three hundred and thirty-four patients (56.6% males), median age 24 (3-62) years were included. Hepatic vein was the commonest site of block-isolated hepatic vonous block in 48%, combined hepatic venous-inferior vena cava block in 46%. Endovascular interventional therapy was performed in 233/334 (70%) with 90% technical success. Clinical response was complete in 166 (71.2%), partial in 58 (24.9%) and no response in nine (3.9%). Majority of cases with HV block did not require TIPSS and could be treated with angioplasty (with/without stenting). On Cox-proportional multivariate analysis, Child class C and response to intervention were independent predictors of outcome and used to derive the All India Institute of Medical Sciences (AIIMS) hepatic venous outflow tract obstruction score. The 5-year survival was 92% (95% CI, 81-97%) for score ≤3, 79% (95%CI, 63-88%) for score >3 and ≤4, and 39% (95% CI, 21-57%) for score >4. The performance of AIIMS hepatic venous outflow obstruction score was superior to other prognostic indices. CONCLUSIONS Advanced Child class and no response to intervention are associated with poor outcomes. The All India Institute of Medical Sciences hepatic venous outflow tract obstruction score predicts survival better than other prognostic scores.
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Affiliation(s)
- Shalimar
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - A Kumar
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - S Kedia
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - H Sharma
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - S R Gamanagatti
- Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India
| | - G S Gulati
- Department of Cardiovascular and Interventional Radiology, All India Institute of Medical Sciences, New Delhi, India
| | - B Nayak
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
| | - B Thakur
- Department of Biostatistics, All India Institute of Medical Sciences, New Delhi, India
| | - S K Acharya
- Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
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Ding PX, Zhang SJ, Li Z, Fu MT, Hua ZH, Zhang WG. Long-term safety and outcome of percutaneous transhepatic venous balloon angioplasty for Budd-Chiari syndrome. J Gastroenterol Hepatol 2016; 31:222-8. [PMID: 26102208 DOI: 10.1111/jgh.13025] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/24/2015] [Revised: 05/21/2015] [Accepted: 06/15/2015] [Indexed: 12/13/2022]
Abstract
BACKGROUND AND AIMS The restenosis following percutaneous transluminal balloon angioplasty (PTBA) is high for Budd-Chiari syndrome (BCS) patients with hepatic venous obstruction (HVO). We aim to evaluate the safety and long-term outcome of PTBA with a large balloon catheter in a large series of patients with HVO. METHODS Between January 2005 and December 2013, 93 consecutive BCS patients with HVO were referred for PTBA and subsequently underwent color Doppler ultrasonography or angiography follow-up. Data were retrospectively collected, and follow-up observations were performed at 1-, 2-, 2- to 5-, and 5- to 8-years postoperatively. RESULTS Percutaneous transluminal balloon angioplasty was technically successful in all patients. Ninety-one patients (97.85%) were treated with PTBA and two with PTBA and stent. Major procedure-related complications occurred in six of the 93 patients (6.45%). The cumulative 1-, 2-, 2- to 5-, and 5- to 8-year primary patency rates were 97.5%, 92.9%, 90%, and 86.5%, respectively. Cumulative 1-, 2-, 2- to 5-, and 5- to 8-year secondary patency rates were 100%, 100%, 98.6%, and 97.3%, respectively. Mean and median primary patency rates were 51.50 ± 3.01 months and 55.0 ± 3.63 months, respectively. Cumulative 1-, 2-, 2- to 5-, and 5- to 8-year survival rates were 98.75%, 98.6%, 100%, and 100%, respectively. Mean and median survival times were 53.10 ± 3.04 months and 55.0 ± 3.64 months, respectively. CONCLUSION Percutaneous transluminal balloon angioplasty with a large balloon is a safe and effective treatment that could provide excellent rates of long-term patency and survival for the majority of Chinese patients with BCS and HVO.
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Affiliation(s)
- Peng-Xu Ding
- Department of Vascular and Endovascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China
| | - Shui-Jun Zhang
- Department of Vascular and Endovascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China
| | - Zhen Li
- Department of Vascular and Endovascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China
| | - Ming-Ti Fu
- Department of Vascular and Endovascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China
| | - Zhao-Hui Hua
- Department of Vascular and Endovascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China
| | - Wen-Guang Zhang
- Department of Vascular and Endovascular Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China
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Goel RM, Johnston EL, Patel KV, Wong T. Budd-Chiari syndrome: investigation, treatment and outcomes. Postgrad Med J 2015; 91:692-7. [PMID: 26494427 DOI: 10.1136/postgradmedj-2015-133402] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2015] [Accepted: 09/22/2015] [Indexed: 12/17/2022]
Abstract
Budd-Chiari syndrome is a rare disorder characterised by hepatic venous outflow obstruction. It affects 1.4 per million people, and presentation depends upon the extent and rapidity of hepatic vein occlusion. An underlying myeloproliferative neoplasm is present in 50% of cases with other causes including infection and malignancy. Common symptoms are abdominal pain, hepatomegaly and ascites; however, up to 20% of cases are asymptomatic, indicating a chronic onset of hepatic venous obstruction and the formation of large hepatic vein collaterals. Doppler ultrasonography usually confirms diagnosis with cross-sectional imaging used for complex cases and to allow temporal comparison. Myeloproliferative neoplasms should be tested for even if a clear causative factor has been identified. Management focuses on anticoagulation with low-molecular-weight heparin and warfarin, with the new oral anticoagulants offering an exciting prospect for the future, but their current effectiveness in Budd-Chiari syndrome is unknown. A third of patients require further intervention in addition to anticoagulation, commonly due to deteriorating liver function or patients identified as having a poorer prognosis. Prognostic scoring systems help guide treatment, but management is complex and patients should be referred to a specialist liver centre. Recent studies have shown comparable procedure-related complications and long-term survival in patients who undergo transjugular intrahepatic portosystemic shunting and liver transplantation in Budd-Chiari syndrome compared with other liver disease aetiologies. Also, the optimal timing of these interventions and which patients benefit from liver transplantation instead of portosystemic shunting remains to be answered.
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Affiliation(s)
- Rishi M Goel
- Department of Gastroenterology, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| | - Emma L Johnston
- Department of Gastroenterology, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| | - Kamal V Patel
- Department of Gastroenterology, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| | - Terence Wong
- Department of Gastroenterology, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
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31
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Qi X, Ren W, Wang Y, Guo X, Fan D. Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies. Expert Rev Gastroenterol Hepatol 2015; 9:865-75. [PMID: 25754880 DOI: 10.1586/17474124.2015.1024224] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
This paper aimed to systematically review the survival of Budd-Chiari syndrome and to identify the most robust prognostic predictors. Overall, 79 studies were included. According to the treatment modalities, the median 1-, 5- and 10-year survival rate was 93, 83 and 73% after interventional radiological treatment; 81, 75 and 72.5% after surgery other than liver transplantation; 82.5, 70.2 and 66.5% after liver transplantation and 68.1, 44.4% and unavailable after medical therapy alone. According to the publication years, the median 1-, 5- and 10-year survival rate was 68.6, 44.4% and unavailable before 1990; 75.1, 69.5 and 57% during the year 1991-1995; 77, 69.6 and 65.6% during the year 1996-2000; 86.5, 74 and 63.5% during the year 2001-2005 and 90, 82.5 and 72% after 2006. Bilirubin, creatinine and ascites were more frequently identified as significant prognostic factors in univariate analyses. But their statistical significance was less frequently achieved in multivariate analyses.
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Affiliation(s)
- Xingshun Qi
- Department of Gastroenterology, General Hospital of Shenyang Military Area, Shenyang, 110840, China
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Taslakian B, Faraj W, Khalife M, Al-Kutoubi A, El-Merhi F, Saade C, Hallal A, Haydar A. Assessment of surgical portosystemic shunts and associated complications: The diagnostic and therapeutic role of radiologists. Eur J Radiol 2015; 84:1525-1539. [PMID: 25963504 DOI: 10.1016/j.ejrad.2015.04.021] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2015] [Revised: 04/16/2015] [Accepted: 04/21/2015] [Indexed: 02/07/2023]
Abstract
Surgical portosystemic shunting, the formation of a vascular connection between the portal and systemic venous circulation, has been used as a treatment to reduce portal venous pressure. Although the use of portosystemic shunt surgery in the management of portal hypertension has declined during the past decade in favour of alternative therapies, and subsequently surgeons and radiologists became less familiar with the procedure, it remains a well-established treatment. Knowledge of different types of surgical portosystemic shunts, their pathophysiology and complications will help radiologists improve communication with surgeons and enhance their understanding of the diagnostic and therapeutic role of radiology in the assessment and management of these shunts. Optimal assessment of the shunt is essential to determine its patency and allow timely intervention. Both non-invasive and invasive imaging modalities complement each other in the evaluation of surgical portosystemic shunts. Interventional radiology plays an important role in the management of complications, such as shunt thrombosis and stenosis. This article describes the various types of surgical portosystemic shunts, explains the anatomy and pathophysiology of these shunts, illustrates the pearls and pitfalls of different imaging modalities in the assessment of these shunts and demonstrates the role of radiologists in the interventional management of complications.
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Affiliation(s)
- Bedros Taslakian
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Walid Faraj
- Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Mohammad Khalife
- Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Aghiad Al-Kutoubi
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Fadi El-Merhi
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Charbel Saade
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Ali Hallal
- Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
| | - Ali Haydar
- Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020-PO Box: 11-0236, Beirut, Lebanon.
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Hoehne SN, Milovancev M, Hyde AJ, deMorais HA, Scollan KF, Nemanic S. Placement of a caudal vena cava stent for treatment of Budd-Chiari-like syndrome in a 4-month-old Ragdoll cat. J Am Vet Med Assoc 2015; 245:414-8. [PMID: 25075825 DOI: 10.2460/javma.245.4.414] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
CASE DESCRIPTION A 16-week-old 1.5-kg (3.3-lb) sexually intact male Ragdoll kitten that had a 9-week history of marked modified transudate ascites was evaluated. A membranous obstruction of the caudal vena cava at the cranial aspect of the liver was identified via CT angiography. CLINICAL FINDINGS Physical examination findings included a markedly distended abdomen and panting. Testing for circulating FIV antibody and FeLV antigen, a PCR assay for feline coronavirus performed on a sample of peritoneal fluid, and fecal flotation yielded negative results. A diagnosis of Budd-Chiari-like syndrome secondary to a membranous obstruction of the caudal vena cava was made. TREATMENT AND OUTCOME The cat was anesthetized, and the subhepatic portion of the caudal vena cava was identified and accessed via median celiotomy and direct venipuncture. A 6F 8 × 24-mm balloon-expandable nitinol biliary stent was placed across the stenotic area under fluoroscopic guidance. The patient remained free of clinical signs at the last follow-up 13 months following the procedure. CLINICAL RELEVANCE Budd-Chiari-like syndrome is a rare phenomenon in veterinary medicine, and congenital malformations should be considered in young feline patients with ascites. Computed tomography angiography proved to be a helpful adjunctive imaging technique to establish a diagnosis in this case. To the authors' knowledge, this is the first report of successful treatment of a congenital caudal vena cava obstruction by means of stent placement in a juvenile cat.
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Affiliation(s)
- Sabrina N Hoehne
- Department of Clinical Sciences, College of Veterinary Medicine, Oregon State University, Corvallis, OR 97331
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Sun Z, Jiang J, Li W, Zhang X, Zhang X, He C. New approach to dilation of stenotic lesions through the accessory hepatic vein in Budd-Chiari syndrome. J Vasc Surg Cases 2015; 1:42-45. [PMID: 31724632 PMCID: PMC6849980 DOI: 10.1016/j.jvsc.2014.10.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2014] [Accepted: 10/25/2014] [Indexed: 11/28/2022] Open
Abstract
We investigated a new approach to dilation of stenotic lesions through the femoral vein-accessory hepatic vein-intrahepatic communicating branched vein-hepatic vein-inferior vena cava loop in two cases of Budd-Chiari syndrome. For some selected patients, this approach represents an additional method to increase technical success and to decrease complications.
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Affiliation(s)
- Zhanguo Sun
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
| | - Jingjun Jiang
- 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
| | - Changshun He
- Department of Vascular Surgery, Peking University People's Hospital, Beijing, China
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An update on the management of Budd-Chiari syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol 2015; 27:200-3. [PMID: 25590783 DOI: 10.1097/meg.0000000000000282] [Citation(s) in RCA: 35] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Because of the rarity of Budd-Chiari syndrome (BCS), the flow chart of management comes from expert opinion and is not evidence based. To report an update on the management of BCS. I performed a review on published papers on BCS in an attempt to speculate in particular on the timing and the choice of treatment. Some authors suggest that the management of BCS should follow a step-wise strategy. Anticoagulation and medical therapy should be the first-line treatment. Revascularization or transjugular intrahepatic portosystemic shunt should be performed in case of no response to medical therapy. Orthotopic liver transplant should be used as a rescue therapy. The biggest criticism of this flow chart is that it is based on the assumption that patients with BCS should receive further treatment only when hemodynamic effects on portal hypertension become clinically evident, thus paying little attention to the chronic ischemic liver damage effects on hepatic function and to the possibility of preventing liver failure by relieving impaired hepatic veins outflow. Recently, I presented a proposal of a new algorithm for the management of BCS, in which medical therapy alone is suggested only for patients without any sign of portal hypertension, irrespective of whether early interventional treatment is suggested when either any symptoms or signs of portal hypertension appear, with the aim of preventing hepatic fibrosis development, disease progression, and finally improving outcome. Given that the benefit of treatments for BCS is not under debate, guidelines for the management of BCS should be re-evaluated and updated, with particular attention to both the timing and the choice of treatment.
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Akamatsu N, Sugawara Y, Kokudo N. Budd-Chiari syndrome and liver transplantation. Intractable Rare Dis Res 2015; 4:24-32. [PMID: 25674385 PMCID: PMC4322592 DOI: 10.5582/irdr.2014.01031] [Citation(s) in RCA: 42] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2014] [Revised: 12/23/2014] [Accepted: 12/25/2015] [Indexed: 12/13/2022] Open
Abstract
Budd-Chiari syndrome involves obstruction of hepatic venous outflow tracts at various levels from small hepatic veins to the inferior vena cava and is the result of thrombosis or its fibrous sequelae. There is a conspicuous difference in its etiology in the West and the East. Myeloproliferative disease predominates in the West and obstruction of the vena cava predominates in the East. The clinical presentation and clinical manifestations are so varied that it should be suspected in any patient with acute or chronic liver dysfunction. It should be treated with step-wise management. First-line therapy should be anticoagulation with medical treatment of the underlying illness, and interventional revascularization and TIPS are indicated in the event of a lack of response to medical therapy. Liver transplantation may be indicated as a rescue treatment or for fulminant cases with promising results. This step-by-step strategy has achieved a 5-year transplant-free survival rate of 70% and a 5-year overall survival rate of 90%. Living donor liver transplantation can also be used for patients with Budd-Chiari syndrome if deceased donor livers are scarce, but it requires a difficult procedure particularly with regard to venous outflow reconstruction.
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Affiliation(s)
- Nobuhisa Akamatsu
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yasuhiko Sugawara
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
- Address correspondence to: Dr. Yasuhiko Sugawara, Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. E-mail:
| | - Norihiro Kokudo
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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Qi X, Fan D, Han G. Timing of transjugular intrahepatic portosystmic for Budd-Chiari syndrome: still an open issue. Liver Int 2014; 34:1288-9. [PMID: 24684318 DOI: 10.1111/liv.12554] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Affiliation(s)
- Xingshun Qi
- Department of Liver Disease and Digestive Interventional Radiology, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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Budd-Chiari syndrome in children: clinical features, percutaneous radiological intervention, and outcome. Eur J Gastroenterol Hepatol 2014; 26:1030-8. [PMID: 25003745 DOI: 10.1097/meg.0000000000000144] [Citation(s) in RCA: 46] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/27/2023]
Abstract
OBJECTIVES 'Radiological intervention' to restore venous patency is the preferred therapy in adults with Budd-Chiari syndrome (BCS). The published literature on pediatric BCS is scarce. This study evaluated the clinical profile and role of a therapeutic radiological intervention in children with BCS. PATIENTS AND METHODS Forty-six BCS children [29 boys, median age 10.5 (2-16) years] were enrolled. Standard medical therapy was administered to all. A radiological intervention, angioplasty [hepatic vein (HV) (n=3)], stenting [HV (n=18), inferior vena cava (IVC) (n=5)], transjugular intrahepatic portosystemic shunt (TIPS) (n=3), was performed in 25 cases. Clinical, biochemical, and radiological follow-up was carried out. RESULTS Doppler ultrasonography was diagnostic in 95% of cases. All patients had chronic BCS, with ascites in 82.6%, hepatomegaly in 84.8%, splenomegaly in 69.6%, prominent abdominal veins in 69.6%, and variceal bleed in 34.8% cases. The most common site of block was HV (n=33), followed by combined HV and IVC block (n=11), and isolated IVC block (n=2). Eight of 12 (75%) cases had abnormal procoagulant workup. Radiological intervention was technically successful in 100%. Clinical and biochemical improvement was observed in the intervention group. Complications included neck hematoma and hemorrhagic ascites in one patient each. One child in the intervention group (post-TIPS sudden cardiac event) and two children in the nonintervention group [end-stage liver disease (n=1), head injury (n=1)] died. Stent was patent in 15/20 (75%) children over a median follow-up of 6.5 months. CONCLUSION HV block and a chronic presentation are most common in BCS children. Doppler ultrasonography establishes the diagnosis in 95% of cases. Radiological intervention is an effective and safe therapeutic modality for children with BCS.
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Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B. Diagnosis and management of Budd Chiari syndrome: an update. Cardiovasc Intervent Radiol 2014; 38:1-12. [PMID: 24923240 DOI: 10.1007/s00270-014-0919-9] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2014] [Accepted: 04/23/2014] [Indexed: 12/16/2022]
Abstract
Imaging plays a crucial role in the early detection and assessment of the extent of disease in Budd Chiari syndrome (BCS). Early diagnosis and intervention to mitigate hepatic congestion is vital to restoring hepatic function and alleviating portal hypertension. Interventional radiology serves a key role in the management of these patients. The interventionist should be knowledgeable of the clinical presentation as well as key imaging findings, which often dictate the approach to treatment. This article concisely reviews the etiology, pathophysiology, and clinical presentation of BCS and provides a detailed description of imaging and treatment options, particularly interventional management.
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Affiliation(s)
- Alexander Copelan
- Diagnostic Radiology Department, William Beaumont Hospital, 3601 W 13 Mile Rd., Royal Oak, MI, 48073, USA,
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Abstract
In the 25 years since the first TIPS intervention has been performed, technical standards, indications, and contraindications have been set up. The previous considerable problem of shunt failure by thrombosis or intimal proliferation in the stent or in the draining hepatic vein has been reduced considerably by the availability of polytetrafluoroethylene (PTFE)-covered stents resulting in reduced rebleeding and improved survival. Unfortunately, most clinical studies have been performed prior to the release of the covered stent and, therefore, do not represent the present state of the art. In spite of this, TIPS has gained increasing acceptance in the treatment of the various complications of portal hypertension and vascular diseases of the liver.
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Affiliation(s)
- Martin Rössle
- Praxiszentrum and University Hospital, Freiburg, Germany.
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Budd-Chiari Syndrome with Multiple Thrombi due to a Familial Arg42Ser Mutation in the Protein C Gene. Case Rep Med 2013; 2013:270419. [PMID: 24250338 PMCID: PMC3821949 DOI: 10.1155/2013/270419] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2013] [Accepted: 09/09/2013] [Indexed: 01/11/2023] Open
Abstract
A 34-year-old Japanese woman was admitted to our hospital complaining of developing bilateral pedal edema. Imaging studies led to a diagnosis of Budd-Chiari syndrome combined with internal jugular vein thrombus. We investigated the cause of thrombosis and found that the anticoagulant activity of protein C was decreased. Genetic analysis showed the presence of a c.125C>A (Arg42Ser) substitution in the protein C gene (PROC) of the proband, which generates an Arg42Ser mutation that replaces the scissile bond Arg42-Ala43 normally cleaved by a furin-like processing protease. Her father and younger brother also carried this mutation, although they had no evidence of thrombosis.
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Mancuso A, Martinelli L, De Carlis L, Rampoldi AG, Magenta G, Cannata A, Belli LS. A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction. World J Hepatol 2013; 5:292-295. [PMID: 23717741 PMCID: PMC3664288 DOI: 10.4254/wjh.v5.i5.292] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/13/2013] [Accepted: 05/10/2013] [Indexed: 02/06/2023] Open
Abstract
Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.
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Han G, Qi X, Zhang W, He C, Yin Z, Wang J, Xia J, Xu K, Guo W, Niu J, Wu K, Fan D. Percutaneous recanalization for Budd-Chiari syndrome: an 11-year retrospective study on patency and survival in 177 Chinese patients from a single center. Radiology 2013; 266:657-67. [PMID: 23143028 DOI: 10.1148/radiol.12120856] [Citation(s) in RCA: 92] [Impact Index Per Article: 7.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
PURPOSE To evaluate the long-term outcomes of percutaneous recanalization and determine the predictors of patency and survival in a large case series of Chinese patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS This retrospective study was approved by the institutional ethics committee. Informed consent for the procedure was obtained from all patients. Between July 1999 and August 2010, 177 consecutive Chinese patients with primary BCS were treated with percutaneous recanalization and followed up until death or their last clinical evaluation. Recanalization therapeutic strategy and complications were recorded. Cumulative patency and survival rates were assessed with Kaplan-Meier curves. Independent predictors of patency and survival were calculated with the Cox regression model. RESULTS Percutaneous recanalization was technically successful in 168 of the 177 patients (95%). Fifty-one of the 168 patients (30%) were treated with percutaneous transluminal angioplasty (PTA) alone and 117 (70%) were treated with a combination of PTA and stent placement. Procedure-related complications occurred in seven of the 168 patients (4%). The cumulative 1-, 5-, and 10-year primary patency rates were 95%, 77%, and 58%, respectively. Independent predictors of reocclusion included increased white blood cell count and use of PTA alone. The cumulative 1-, 5-, and 10-year secondary patency rates were 97%, 90%, and 86%, respectively. Twenty-two patients died during a median follow-up of 30 months (range, 0.25-137 months). The cumulative 1-, 5-, and 10-year survival rates were 96%, 83%, and 73%, respectively. Independent predictors of survival included variceal bleeding, increased alkaline phosphatase and blood urea nitrogen levels, and reocclusion. CONCLUSION Percutaneous recanalization could achieve excellent long-term patency and survival in most Chinese patients with BCS. PTA combined with stent placement should be recommended to decrease the frequency of reocclusion and its associated mortality.
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Affiliation(s)
- Guohong Han
- Department of Digestive Interventional Radiology, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, 15 West Changle Rd, Xi'an 710032, China.
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Percutaneous recanalization of an occluded hepatic vein in a difficult subset of pediatric Budd-Chiari syndrome. Pediatr Cardiol 2012; 33:806-10. [PMID: 22349663 DOI: 10.1007/s00246-012-0188-9] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/02/2011] [Accepted: 11/17/2011] [Indexed: 10/14/2022]
Abstract
We present a 3-year-old child with Budd Chiari syndrome having idiopathic complete occlusion of all three major hepatic veins and small hepatic venules. Adequate antegrade flow in right hepatic vein was established by transfemoral balloon angioplasty followed by stenting of the same. Long term antiplatelet therapy was instituted. Medium term follow up reveals satisfactory antegrade flow and regression of symptoms. This strategy highlights an effective nonsurgical approach of restoring physiological pattern of hepatic sinusoidal blood flow in Budd Chiari syndrome.
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Wang YL, Ding PX, Li YD, Han XW, Wu G. Comparative study of predilation with stent filter for Budd-Chiari syndrome with old IVC thrombosis: A nonrandomized prospective trial. Eur J Radiol 2012; 81:1158-64. [DOI: 10.1016/j.ejrad.2011.03.021] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2011] [Accepted: 03/04/2011] [Indexed: 11/26/2022]
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Mancuso A. Budd-Chiari syndrome management: Lights and shadows. World J Hepatol 2011; 3:262-4. [PMID: 22059108 PMCID: PMC3208178 DOI: 10.4254/wjh.v3.i10.262] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2011] [Revised: 07/19/2011] [Accepted: 09/20/2011] [Indexed: 02/06/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is a rare disease whose management should follow a step by step strategy. Anticoagulation and medical therapy should be the first line treatment. Revascularization or TIPS are indicated in case of no response to medical therapy. OLT should be indicated as a rescue therapy and anticoagulation be started soon after OLT. However, no clear indication can actually be given about the timing of different treatments. Moreover, there is some concern about treatment of some subgroup of patients, especially regarding the risk of recurrence after liver transplantation. The topic of this paper is to critically review the actual knowledge of BCS management.
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Affiliation(s)
- Andrea Mancuso
- Andrea Mancuso, Epatologia e Gastroenterologia, Ospedale Niguarda Ca' Granda, Milano 20162, Italy
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Chen H, Cheng Y, Ning S, Chen Y. Budd--Chiari syndrome caused by multiple membranous obstruction of the inferior vena cava in a young man. Ann Vasc Surg 2011; 25:1139.e5-7. [PMID: 21835582 DOI: 10.1016/j.avsg.2011.06.004] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2011] [Revised: 06/01/2011] [Accepted: 06/14/2011] [Indexed: 11/18/2022]
Abstract
Membranous obstruction of the inferior vena cava is a frequent cause of Budd--Chiari syndrome in Eastern countries. The study reports the case of a young male patient with Budd--Chiari syndrome caused by extremely rare multiple membranous obstruction of inferior vena cava. A percutaneous transluminal angioplasty with stent placement was performed successfully, accompanied with satisfactory resolution of clinical symptoms and signs. The graft was reported to be patent 2 years postoperatively.
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Affiliation(s)
- Hongqiang Chen
- Department of Hepato-Biliary, Qilu Hospital, Shandong University, Ji'nan, China
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Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol 2011; 3:169-77. [PMID: 21860712 PMCID: PMC3158894 DOI: 10.4329/wjr.v3.i7.169] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/28/2011] [Revised: 07/13/2011] [Accepted: 07/20/2011] [Indexed: 02/06/2023] Open
Abstract
Budd-Chiari syndrome (BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension. Clinically, two forms of disease (acute and chronic) are recognized. Mostly the patients present with ascites, hepatomegaly, and portal hypertension. In acute disease the liver is enlarged with thrombosed hepatic veins (HV) and ascites, whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava (IVC), or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC. Due to advances in radiological interventional techniques and hardware, there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation. The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.
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