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Malakar S, Mathur A, Agarwal A, Mishra P, Shirol VV, Yadav RR, Ghoshal UC. Budd Chiari Syndrome with Portal Vein Thrombosis Managed Successfully with Direct Intrahepatic Portosystemic Shunt: A Case Report and Review of the Literature. J Clin Exp Hepatol 2023; 13:917-920. [PMID: 37693264 PMCID: PMC10482988 DOI: 10.1016/j.jceh.2023.06.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2023] [Accepted: 06/14/2023] [Indexed: 09/12/2023] Open
Abstract
Budd -Chiari syndrome (BCS) is a hepatic vascular disorder which affects hepatic veins or inferior vena cava. Portal vein thrombosis (PVT) occurs in around 15%-25% of patients with BCS. The presence of PVT in patients with BCS makes it more difficult to intervene radiologically. We present a case of a BCS-related chronic liver disease that presented with a history of variceal upper gastrointestinal bleeding and worsening ascites. The patient had thrombosed hepatic veins (HV) and partial right portal vein thrombosis. He was started on anticoagulation, and treatment for portal hypertension was initiated. Given the inaccessibility of all the HVs for trans-jugular intrahepatic portosystemic shunts (TIPS), the patient underwent direct intrahepatic portosystemic shunts (DIPS). Next-generation sequencing identified the factor V Leiden mutation. Following DIPS, the patient's ascites disappeared, and liver function tests improved. On a nine-month follow-up, the patient was symptom-free with a patent DIPS. DIPS has been widely used in patients with BCS with thrombosed hepatic veins, but there are only a few case reports on the feasibility of DIPS in BCS patients with PVT. This is one of the very few case reports where a patient with BCS-PVT was successfully managed with DIPS.
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Affiliation(s)
- Sayan Malakar
- Department of Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Akash Mathur
- Department of Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Ayushi Agarwal
- Department of Interventional Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Piyush Mishra
- Department of Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Vivek V. Shirol
- Department of Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Rajanikant R Yadav
- Department of Interventional Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Uday C. Ghoshal
- Department of Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Găman MA, Cozma MA, Manan MR, Srichawla BS, Dhali A, Ali S, Nahian A, Elton AC, Simhachalam Kutikuppala LV, Suteja RC, Diebel S, Găman AM, Diaconu CC. Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature. World J Clin Oncol 2023; 14:99-116. [PMID: 37009527 PMCID: PMC10052333 DOI: 10.5306/wjco.v14.i3.99] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2022] [Revised: 02/10/2023] [Accepted: 03/01/2023] [Indexed: 03/19/2023] Open
Abstract
Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e., polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g., portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations (e.g., the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.
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Affiliation(s)
- Mihnea-Alexandru Găman
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
- Department of Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest 022328, Romania
| | - Matei-Alexandru Cozma
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
| | | | - Bahadar S Srichawla
- Department of Neurology, University of Massachusetts Chan Medical School, Worcester, Massachusetts 01655, United States
| | - Arkadeep Dhali
- Department of Internal Medicine, Nottingham University Hospitals NHS Trust, Nottingham NG7 2UH, United Kingdom
| | - Sajjad Ali
- Department of Medicine, Ziauddin University, Karachi 75600, Pakistan
| | - Ahmed Nahian
- California Baptist University-Lake Erie College of Osteopathic Medicine, Riverside, CA 92504, United States
| | - Andrew C Elton
- University of Minnesota Medical School, Minneapolis, MN 55455, United States
| | - L V Simhachalam Kutikuppala
- Department of General Surgery, Dr NTR University of Health Sciences, Andhra Pradesh, Vijayawada 520008, India
| | - Richard Christian Suteja
- Department of Undergraduate Medicine, Faculty of Medicine, Udayana University Denpasar, Bali 80232, Indonesia
| | - Sebastian Diebel
- Department of Family Medicine, Northern Ontario School of Medicine Timmins, Ontario 91762, Canada
| | - Amelia Maria Găman
- Department of Pathophysiology, University of Medicine and Pharmacy of Craiova, Romania & Clinic of Hematology, Filantropia City Hospital, Craiova 200143, Romania
| | - Camelia Cristina Diaconu
- Department of Internal Medicine, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Romania & Internal Medicine Clinic, Clinical Emergency Hospital of Bucharest, Bucharest 105402, Romania
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Venous thrombosis of the liver: current and emerging concepts in management. Transl Res 2020; 225:54-69. [PMID: 32407789 DOI: 10.1016/j.trsl.2020.04.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Revised: 04/11/2020] [Accepted: 04/16/2020] [Indexed: 02/08/2023]
Abstract
Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.
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Inchingolo R, Posa A, Mariappan M, Tibana TK, Nunes TF, Spiliopoulos S, Brountzos E. Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review. World J Gastroenterol 2020; 26:5060-5073. [PMID: 32982109 PMCID: PMC7495032 DOI: 10.3748/wjg.v26.i34.5060] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2020] [Revised: 06/06/2020] [Accepted: 08/25/2020] [Indexed: 02/06/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.
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Affiliation(s)
- Riccardo Inchingolo
- Interventional Radiology Unit, "F. Miulli" Regional Hospital, Acquaviva delle Fonti 70021, Italy
- Department of Radiology, King´s College Hospital, London SE5 9RS, United Kingdom
| | - Alessandro Posa
- Department of Radiology, Gemelli Hospital, Roma 00135, Italy
| | - Martin Mariappan
- Interventional Radiology Department, Aberdeen Royal Infirmary Hospital, Aberdeen AB25 2ZN, United Kingdom
| | - Tiago Kojun Tibana
- Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
| | - Thiago Franchi Nunes
- Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
| | - Stavros Spiliopoulos
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
| | - Elias Brountzos
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
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Haque LYK, Lim JK. Budd-Chiari Syndrome: An Uncommon Cause of Chronic Liver Disease that Cannot Be Missed. Clin Liver Dis 2020; 24:453-481. [PMID: 32620283 DOI: 10.1016/j.cld.2020.04.012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.
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Affiliation(s)
- Lamia Y K Haque
- Section of Digestive Diseases, Yale University School of Medicine, 333 Cedar Street, LMP 1080, New Haven, CT 06520, USA
| | - Joseph K Lim
- Section of Digestive Diseases, Yale Liver Center, Yale University School of Medicine, 333 Cedar Street, LMP 1080, New Haven, CT 06520, USA.
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Radiation exposure during transjugular intrahepatic portosystemic shunt creation in patients with complete portal vein thrombosis or portal cavernoma. Radiol Med 2020; 125:609-617. [PMID: 32072390 DOI: 10.1007/s11547-020-01155-5] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2019] [Accepted: 02/06/2020] [Indexed: 02/07/2023]
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Wang Q, Han G. Budd-Chiari Syndrome and Inferior Vena Cava Obstruction: The Asian Perspective. DIAGNOSTIC METHODS FOR CIRRHOSIS AND PORTAL HYPERTENSION 2018:257-269. [DOI: 10.1007/978-3-319-72628-1_17] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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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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Cansu DU, Temel T, Erturk A, Kasifoglu T, Acu B, Korkmaz C. The Long-Term Outcomes for Patients with Budd-Chiari Syndrome Caused by Behcet's Disease: A Case Series on the Results, from Cirrhosis to Death. HEPATITIS MONTHLY 2016; 16:e32457. [PMID: 27882061 PMCID: PMC5116124 DOI: 10.5812/hepatmon.32457] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/15/2015] [Revised: 08/16/2016] [Accepted: 08/17/2016] [Indexed: 12/11/2022]
Abstract
BACKGROUND Budd-Chiari syndrome, which is a rare complication of Behcet's disease, carries a high mortality rate. OBJECTIVES The aim of the study was to present our long-term follow up experience with patients suffering from Budd-Chiari syndrome due to Behcet's disease. METHODS The records of 402 patients with Behcet's disease were evaluated retrospectively. To facilitate detection of the long-term complications caused by Budd-Chiari syndrome, the patients were evaluated via physical examinations, laboratory tests, imaging modalities, and endoscopy results. RESULTS The data for 402 patients diagnosed with Behcet's disease, who were followed up at our hospital over 16 years, were analyzed retrospectively. Five of these 402 patients (1.2%) were diagnosed as having Budd-Chiari syndrome. The patients with Budd-Chiari syndrome were aged between 23 and 54, and all five were male. The interval between the onset of Behcet's disease and the development of Budd-Chiari syndrome ranged from 1 to 8 years. All the patients had combined venous occlusion (affecting the hepatic vein and inferior vena cava). Portal venous thrombosis was detected in only one patient (Case 1), who died 1 month after the diagnosis of Budd-Chiari syndrome. The survival time for the other four patients after the diagnosis of Budd-Chiari syndrome ranged from 4 to 16 years. During the long-term follow-up, hepatic masses were detected via radiological surveillance in Case 3 (in the form of large regenerative nodules) and Case 4 (nodular regenerative hyperplasia and cirrhosis). CONCLUSIONS In our study, portal venous thrombosis was detected in the patient who died during the acute period only. A study including large numbers of Budd-Chiari-syndrome patients with Behcet's disease and portal venous thrombosis would be helpful to determine the prognostic significance of portal venous thrombosis in Budd-Chiari-syndrome patients with Behcet's disease. In addition, patients should be monitored regularly for the development of hepatic masses via a long-term surveillance program.
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Affiliation(s)
- Dondu Uskudar Cansu
- Division of Rheumatology, Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey
- Corresponding Author: Dondu Uskudar Cansu, Department of Rheumatology, School of Medicine, Eskisehir Osmangazi University Eskisehir, Turkey. Tel: +90-2222392979-2931, Fax: +90-2222393772, E-mail:
| | - Tuncer Temel
- Division of Gastroenterology, Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Adem Erturk
- Division of Rheumatology, Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Timucin Kasifoglu
- Division of Rheumatology, Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Berat Acu
- Department of Radiology, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Cengiz Korkmaz
- Division of Rheumatology, Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey
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Townsend SA, Karkhanis S, Tripathi D, Mueisan P, Zia Z, Elsharkawy AM. Rescue from liver transplantation: TIPSS and thrombectomy successfully treat a case of acute Budd-Chiari syndrome complicated by portal vein thrombosis. BJR Case Rep 2016; 3:20160059. [PMID: 30363345 PMCID: PMC6159308 DOI: 10.1259/bjrcr.20160059] [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: 03/03/2016] [Accepted: 06/07/2016] [Indexed: 11/06/2022] Open
Abstract
We report the rare case of a female who presented with fulminant liver failure secondary to acute Budd-Chiari syndrome and complete portal vein thrombosis. She met the criterion for liver transplant and was transferred to our care for assessment and further management. Transplant was deemed a too-high risk and so rescue therapy was undertaken using mechanical thrombectomy and transjugular intrahepatic portosystemic shunt insertion to decompress the portal system. The patient made a full recovery. This is a rare case report of a patient meeting liver transplant criteria secondary to acute Budd-Chiari syndrome and complete portal vein thrombosis, which was managed successfully entirely by radiological means; this technique could be used to avoid or act as a bridge to liver transplantation in the future.
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Affiliation(s)
| | - Salil Karkhanis
- Radiology Department, Queen Elizabeth Hospital, Birmingham, UK
| | | | - Paolo Mueisan
- Liver Unit, Queen Elizabeth Hospital, Birmingham, UK
| | - Zergham Zia
- Radiology Department, Queen Elizabeth Hospital, Birmingham, UK
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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; 9:865-875. [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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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
GOALS We aimed to present our long-term surveillance experience in patients with Budd-Chiari syndrome (BCS), and we retrospectively evaluated the natural history, results of thrombophilia studies, and the factors related to mortality. BACKGROUND Primary BCS is a rare form of vascular disease, secondary to underlying thrombophilia. Because of its rarity and heterogeneous nature, there is a scarcity of knowledge about the natural history of the disease. STUDY AND RESULTS In 22 years, a total of 62 patients with primary BCS were followed in our tertiary hospital. We identified an acquired cause of BCS in 40 out of 62 patients (64.5%), whereas in 6 patients (9.7%), we found no identifiable cause. One or more thrombophilia causes were identified in 56 patients (90.3%). In 19 patients with myeloproliferative disease, 15 had Janus tyrosine kinase 2 mutation analysis and Janus tyrosine kinase 2 positivity was found in 10 patients. In regression analysis, portal vein thrombosis was found to be the only indicator of mortality, with an estimated instantaneous risk of 8.4. CONCLUSIONS In this study, we present one of the largest series of BCS in the English literature. We have shown that the multifactorial nature of underlying thrombophilia should be thoroughly investigated. In a patient with BCS, a clinician should be alert for the development or coexistence of portal vein thrombosis due to its deleterious effect on mortality.
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Rasheed K. Hydatid cyst of liver complicated with budd-Chiari syndrome and portal vein thrombosis. NORTH AMERICAN JOURNAL OF MEDICAL SCIENCES 2013; 5:242-4. [PMID: 23626964 PMCID: PMC3632032 DOI: 10.4103/1947-2714.109206] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Affiliation(s)
- Khalid Rasheed
- Department of Internal Medicine, University of Alabama at Birmingham, Health Center Montgomery, Montgomery, Alabama, USA. E-mail:
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Vascular disorders of the liver: recommendations from the Italian Association for the Study of the Liver (AISF) ad hoc committee. Dig Liver Dis 2011; 43:503-14. [PMID: 21185794 DOI: 10.1016/j.dld.2010.11.006] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2010] [Accepted: 11/23/2010] [Indexed: 02/07/2023]
Abstract
This review summarizes the document elaborated by the Italian Association for the Study of the Liver (AISF) ad hoc committee "Vascular disorders of the liver" on the primary circulatory liver diseases, which include Budd-Chiari syndrome, obstruction of the hepatic portion of the inferior vena cava, portal vein thrombosis, sinusoidal obstruction syndrome (veno-occlusive disease) and hereditary hemorrhagic telangiectasia. A characteristic of the primary circulatory liver diseases is that portal hypertension usually precedes liver dysfunction. Significant overlap exists amongst the diseases and risk factors that predispose patients to the primary circulatory liver diseases, though the pathogenesis of individual diseases varies. Management of the different vascular disorders is very peculiar and often multidisciplinary and patients should be referred to a tertiary referral centre for optimal care.
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Abstract
Doppler ultrasonography (US) is usually the first-line modality for evaluating flow in native liver vessels and transjugular intrahepatic portosystemic shunts (TIPS). Waveforms, which represent flow in each of the major vessels and in TIPS, have been well described. The appearance of these waveforms should be recognized, and the mechanisms behind their generation should be understood by those interpreting these examinations. Understanding how waveforms are formed--that is, their mechanisms for generation--is predicated on knowing basic vascular Doppler concepts and established nomenclature. This article is a review of these basic concepts and nomenclature as applied to the interpretation of liver Doppler US waveforms.
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Affiliation(s)
- Dean Alexander McNaughton
- Department of Radiology, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, Iowa City, IA 52242, USA.
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Abstract
This guideline has been approved by the American Association for the Study of Liver Diseases (AASLD) and represents the position of the association.
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Affiliation(s)
- Laurie D DeLeve
- Division of Gastrointestinal and Liver Diseases and the Research Center for Liver Diseases, University of Southern California Keck School of Medicine, Los Angeles, CA, USA
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Rajani R, Melin T, Björnsson E, Broomé U, Sangfelt P, Danielsson A, Gustavsson A, Grip O, Svensson H, Lööf L, Wallerstedt S, Almer SHC. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience. Liver Int 2009; 29:253-9. [PMID: 18694401 DOI: 10.1111/j.1478-3231.2008.01838.x] [Citation(s) in RCA: 94] [Impact Index Per Article: 5.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/13/2023]
Abstract
BACKGROUND The exact incidence and prevalence of Budd-Chiari syndrome (BCS) is unknown in the general population. Published reports differ in terms of the clinical characteristics, effects of therapy and survival. AIMS To investigate the epidemiology, clinical presentation and survival in patients with BCS. METHODS Retrospective multicentre study in Sweden reviewing the medical records of all patients with BCS 1986-2003, identified from the computerised diagnosis database of 11 hospitals, including all university hospitals and liver transplantation centres. RESULTS Forty-three patients with BCS were identified, of whom nine (21%) had concomitant portal vein thrombosis. The mean age-standardised incidence and prevalence rates in 1990-2001 were calculated to be 0.8 per million per year and 1.4 per million inhabitants respectively. Myeloproliferative disorders (38%), thrombophilic factors (31%) and oral contraceptives (30%) were common aetiological factors. Two or more risk factors were present in 44%. In 23%, no risk factor was evident. The median follow-up time was 2.7 years. Seventy-two percent were on anticoagulant therapy during follow-up. Transjugular intrahepatic portosystemic shunting, surgical shunting procedures and liver transplantation were performed in 4, 6 and 18 patients respectively. Nineteen patients died. The overall transplantation-free survival at 1, 5 and 10 years was 47, 28 and 17% respectively. CONCLUSIONS Budd-Chiari syndrome is a rare disorder; the mean age-standardised incidence and prevalence rates in Sweden in 1990-2001 were calculated to be 0.8 per million per year and 1.4 per million inhabitants respectively. The presence of a myeloproliferative disorder was a common aetiological factor in our cohort and about half of the patients had a multifactorial aetiology. The transplantation-free survival was poor.
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Affiliation(s)
- Rupesh Rajani
- Department of Medicine, Ryhov County Hospital, Jönköping, Sweden
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Bertolotto M, Martinoli C, Migaleddu V, Cernic S, Zappetti R. Color Doppler sonography of intrahepatic vascular shunts. JOURNAL OF CLINICAL ULTRASOUND : JCU 2008; 36:527-538. [PMID: 18693256 DOI: 10.1002/jcu.20519] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/26/2023]
Abstract
PURPOSE To illustrate the sonographic and Doppler features of the different types of intrahepatic vascular shunts. METHODS Patients presenting in our department with intrahepatic vascular shunts with healthy livers or with a variety of liver pathologies underwent color Doppler interrogation and spectral analysis. RESULTS Intrahepatic vascular shunts may involve all liver vessels. Arterioportal and arteriosystemic venous connections can be recognized in normal and cirrhotic livers, following trauma, within tumors, and within nontumoral arteriovenous malformations. Portosystemic intrahepatic venous shunts are categorized into 4 morphologic types. Systemic-to-systemic shunts are more often recognized in patients with chronic hepatic venous congestion or with Budd-Chiari syndrome. Portal-to-portal intrahepatic venous shunts are rare, with few cases described. CONCLUSION In general, all the liver vessels can be associated with formation of arterioportal, arteriosystemic, and portosystemic venous shunts and, rarely, systemic venous shunts and portal-to-portal communications. Sonography allows characterization of the different intrahepatic shunts.
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Affiliation(s)
- Michele Bertolotto
- Department of Radiology, University of Trieste, Ospedale di Cattinara, Strada di Fiume 449, 34149 Trieste, Italy
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Senzolo M, Patch D, Miotto D, Ferronato C, Cholongitas E, Burroughs AK. Interventional treatment should be incorporated in the algorithm for the management of patients with portal vein thrombosis. Hepatology 2008; 48:1352-3. [PMID: 18821588 DOI: 10.1002/hep.22503] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
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de Buys Roessingh AS, Portier-Marret N, Tercier S, Qanadli SD, Joseph JM. Combined endovascular and surgical recanalization after central venous catheter-related obstructions. J Pediatr Surg 2008; 43:E21-4. [PMID: 18558160 DOI: 10.1016/j.jpedsurg.2008.01.076] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2007] [Revised: 01/10/2008] [Accepted: 01/29/2008] [Indexed: 11/19/2022]
Abstract
Central venous occlusion in children is a challenging problem that can occur after a central venous catheter insertion. Long-term catheter-related complications include sepsis and venous thrombosis with consequent loss of central access. We describe 2 cases of children younger than 1 year who were dependent on a central venous catheter for total parenteral nutrition. They developed a chronic extensive obstruction of the right and left brachiocephalic veins with a superior vena cava syndrome. The patients' survival was dependent on the restoration of central venous access until the planned intestinal transplantation could be performed. Retrograde recanalization of the superior vena cava was successfully achieved using a pathway created under general anesthesia from the femoral vein to, respectively, the right thyroid vein and the right subclavian vein.
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Affiliation(s)
- Anthony S de Buys Roessingh
- Department of Pediatric Surgery, University Hospital Center of the Canton of Vaud CHUV, Lausanne, Switzerland.
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Uskudar O, Akdogan M, Sasmaz N, Yilmaz S, Tola M, Sahin B. Etiology and portal vein thrombosis in Budd-Chiari syndrome. World J Gastroenterol 2008; 14:2858-62. [PMID: 18473410 PMCID: PMC2710727 DOI: 10.3748/wjg.14.2858] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To research the etiology, portal vein thrombosis and other features of Budd-Chiari syndrome (BCS) patients prospectively.
METHODS: A total of 75 patients (40 female, 35 male) who were diagnosed between January 2002 and July 2004 as having BCS were studied prospectively. Findings from on physical examination, ultrasonography, duplex ultrasonography and venography were analyzed. Hemogram and blood chemistry were studied at the time of diagnosis and on each hospital visit. Bone marrow examination and immune phenotyping were performed by a hematologist when necessary. Protein C, S, antithrombin III, activated protein C resistance, and anticardiolipin antibodies, antinuclear antibodies, and anti ds-DNA were studied twice. The presence of ascite, esophageal varices, and portal thrombosis were evaluated at admission and on every visit.
RESULTS: At least one etiological factor was determined in 54 (72%) of the patients. The etiology could not be defined in 21 (28%) patients. One etiological factor was found in 39, 2 factors in 14 and 3 factors in 1 patient. The most common cause was the web (16%), the second was Hydatid disease (11%), the third was Behcet’s disease (9%). Portal vein thrombosis was present in 11 patients and at least one etiology was identified in 9 of them (82%).
CONCLUSION: Behcet’s disease and hydatid disease are more prominent etiological factors in Turkey than in other countries. Patients with web have an excellent response to treatment without signs of portal vein thrombosis while patients having thrombophilic factors more than one are prone to develop portal vein thrombosis with worse clinical outcome.
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Abstract
Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100 000 in the general population. Hypercoagulable state could be identified in 75% of the patients; more than one etiologic factor may play a role in 25% of the patients. Primary myeloproliferative diseases are the leading cause of the disease. Two of the hepatic veins must be blocked for clinically evident disease. Liver congestion and hypoxic damage of hepatocytes eventually result in predominantly centrilobular fibrosis. Doppler ultrasonography of the liver should be the initial diagnostic procedure. Hepatic venography is the reference procedure if required. Additionally liver biopsy may be helpful for differential diagnosis. The prognosis of the chronic form is acceptable compared to other chronic liver diseases.
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Affiliation(s)
- Musa Aydinli
- Gastroenterology Department, Hacettepe University School of Medicine, Ankara, Turkey
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Senzolo M, Burra P, Cholongitas E, Lodato F, Marelli L, Manousou P, Patch D, Sturniolo GC, Burroughs AK. The transjugular route: the key hole to the liver world. Dig Liver Dis 2007; 39:105-16. [PMID: 17196894 DOI: 10.1016/j.dld.2006.06.038] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2006] [Revised: 06/20/2006] [Accepted: 06/27/2006] [Indexed: 02/07/2023]
Abstract
Portal hypertensive complications are major causes of morbidity and mortality in patients with liver cirrhosis. The advent of the transjugular route with its minimal access allows non-surgical management of portal hypertension, therapy of venous complications of liver transplantation, monitoring of therapy for portal hypertension, hepatic venous pressure gradient and is also the major route to treat hepatic venous obstruction syndromes. In addition, the transjugular route is a safe route to perform a liver biopsy (transjugular liver biopsy) and allows retrograde evaluation of the portal vein. All these procedures can be combined in the same session. These hepatic interventional radiological skills should be incorporated into the expertise of the liver team in specialised hepatological centres, particularly in liver transplant centres as they are especially useful in improving outcomes of cirrhotic patients on the liver transplantation waiting list. A limitation in achieving this goal, could be the number of experienced radiologists, but hepatologists can be trained, at least for the most simple procedures (transjugular liver biopsy and hepatic venous pressure gradient). This would allow wider applicability and use of these diagnostic and therapeutic techniques, all through a 2 mm hole in the neck--the key hole to the liver world.
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Affiliation(s)
- M Senzolo
- Liver Transplantation and Hepatobiliary Unit, Royal Free Hospital, London, Pond Street, London NW3 2QG, UK
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Vilgrain V, Condat B, Bureau C, Hakimé A, Plessier A, Cazals-Hatem D, Valla DC. Atrophy-hypertrophy complex in patients with cavernous transformation of the portal vein: CT evaluation. Radiology 2006; 241:149-55. [PMID: 16908681 DOI: 10.1148/radiol.2411051102] [Citation(s) in RCA: 55] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
PURPOSE To retrospectively evaluate the morphologic changes in the liver associated with cavernous transformation of the portal vein. MATERIALS AND METHODS This study was institutional review board approved. Informed patient consent was not required. The computed tomographic (CT) results for 22 patients (14 male, eight female; mean age, 54 years) with cavernous transformation of the portal vein and no evidence of chronic liver disease at liver biopsy were retrospectively reviewed and compared with the CT results for 36 control subjects. Various morphologic changes in the hepatic lobes were qualitatively and quantitatively assessed by using the Student t test for unpaired data. RESULTS Qualitative analysis revealed the atrophy-hypertrophy complex in most (n = 20, 91%) of the patients with cavernous transformation and in no control subjects. Atrophy of the left lateral segment and right liver lobe was seen in 16 (73%) and seven (32%) patients, respectively. Hypertrophy of the caudate lobe and liver segment IV was identified in 19 (86%) and 11 (50%) patients, respectively. All mean caudate lobe volume index values and mean caudate lobe-to-right lobe ratio values were significantly greater (P < .05) in the cavernous transformation group than in the control group. The mean segment IV diameter was significantly greater (41.6 vs 28.1 mm, P < .001) in the patients with cavernous transformation. Hepatic nodules and hepatic contour nodularity were not seen in the patients with cavernous transformation. CONCLUSION The atrophy-hypertrophy complex is frequently observed in patients with cavernous transformation of the portal vein. Some findings, such as hypertrophy of the caudate lobe, mimic chronic liver disease or signs of portal hypertension, but left lateral segment atrophy and a normal or enlarged segment IV are distinctive findings of cavernous transformation.
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Affiliation(s)
- Valérie Vilgrain
- Departments of Radiology and INSERM Unité 773 CRB3, Assistance Publique des Hôpitaux de Paris, Hôpital Beaujon, 100 bld Général Leclerc, 92110 Clichy, France.
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Senzolo M, Tibbals J, Cholongitas E, Triantos CK, Burroughs AK, Patch D. Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation. Aliment Pharmacol Ther 2006; 23:767-75. [PMID: 16556179 DOI: 10.1111/j.1365-2036.2006.02820.x] [Citation(s) in RCA: 178] [Impact Index Per Article: 9.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
BACKGROUND Treatment options for patients with portal vein thrombosis are limited. AIM To evaluate the feasibility and efficacy of transjugular intrahepatic portosystemic shunt for portal vein thrombosis with/without cavernomatous transformation. METHODS A survey of such patients, referred for transjugular intrahepatic portosystemic shunt between 1994 and 2005, was performed. Success rates, complications, transjugular intrahepatic portosystemic shunt patency and clinical progression were examined. RESULTS Transjugular intrahepatic portosystemic shunt was attempted in 28 patients (13 cirrhotics). Indications were: presurgery/transplantation (2), worsening of ascites (2), variceal bleeding (15 - 8 elective), refractory ascites (3), portal biliopathy (3) and portal vein thrombosis complicating Budd-Chiari syndrome (2). Transjugular intrahepatic portosystemic shunt was placed successfully in 19 of 28 (73%); 23 of 28 had complete portal vein thrombosis and 9 of 23 had cavernous transformation and transjugular intrahepatic portosystemic shunt was successfully placed in six of these. In the 19 patients with transjugular intrahepatic portosystemic shunt, the mean follow-up was 18.1 months (range 5-70): six patients had stent revisions; three had liver transplantation, one died of bleeding. Most cirrhotic patients had an improvement in the Child-Pugh score. In the failed transjugular intrahepatic portosystemic shunt group, two of nine died, and three had further bleeding. CONCLUSIONS Transjugular intrahepatic portosystemic shunt should be considered for selected patients with symptomatic complete portal vein thrombosis with/without cavernous transformation, as clinical improvement and less rebleeding occur when transjugular intrahepatic portosystemic shunt placement is successful.
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Affiliation(s)
- M Senzolo
- Liver Transplantation and Hepatobiliary Unit, Royal Free & University College Medical School, London, UK
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28
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Kori I, Bar-Zohar D, Carmiel-Haggai M, Samuels D, Nakache R, Oren R, Kessler A, Szold O, Ben-Haim M. Budd-Chiari syndrome and acute portal vein thrombosis: management by a transjugular intrahepatic portosystemic shunt (TIPS) and portal vein interventions via a TIPS. J Gastrointest Surg 2006; 10:417-21. [PMID: 16504889 DOI: 10.1016/j.gassur.2005.07.019] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2005] [Accepted: 07/27/2005] [Indexed: 01/31/2023]
Abstract
Acute portal vein thrombosis (PVT) is a devastating complication of Budd-Chiari syndrome (BCS). Conservative approach, anticoagulation, systemic or transarterial thrombolysis, and urgent liver transplantation were applied in this scenario but with poor results. We present and discuss an approach to treat BCS complicated by acute PVT. Two young female patients presented with acute liver failure, rapidly progressive tense ascites, renal- and respiratory failure. The diagnosis of chronic BCS complicated by acute PVT was confirmed with ultrasound Doppler. Initial treatment was supportive. Right portal vein localization was by transarterial portogram or by computed tomography-guided microcoil placement. Transjugular intrahepatic portosystemic shunt (TIPS) was performed and included Wallstents and a Jograft in one case and Viatorr stentgraft that was extended later with a Hemobahn stentgraft in another. Mechanical clot removal from the portal system was performed in the primary procedure and in a revision procedure in the following few days. Stents were placed precisely with no extension into the inferior vena cava or deeply into the main portal vein. Patients were fully anticoagulated and patency was assessed by ultrasound Doppler. The procedures were performed on days 5 and 10 following admission. In both cases, successful thrombectomies were revised and maintained. Partial occlusion of the TIPS and reaccumulation of ascites were reversed with repeated procedure. Both patients were discharged without ascites and normal liver function. In conclusion, urgent TIPS and portal vein thrombectomy via TIPS are emerging therapeutic options that offer a safe and effective treatment to patients with BCS complicated by acute portal vein thrombosis.
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Affiliation(s)
- Isaac Kori
- Interventional Radiology, Tel-Aviv Sourasky Medical Center, 6 Weitzman Street, Tel-Aviv 64239, Israel
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Abstract
The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymptomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.
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Affiliation(s)
- Yusuf Bayraktar
- Hacettepe University Faculty of Medicine, Department of Gastroenterology, 06100 Sihhiye, Ankara, Turkey.
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Darwish Murad S, Valla DC, de Groen PC, Zeitoun G, Haagsma EB, Kuipers EJ, Janssen HLA. Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis. Am J Gastroenterol 2006; 101:83-90. [PMID: 16405538 DOI: 10.1111/j.1572-0241.2006.00353.x] [Citation(s) in RCA: 63] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Combined Budd-Chiari syndrome and Portal Vein Thrombosis (BCS-PVT) is a challenging clinical condition with as yet unknown outcome. The aim of the present study was to investigate etiology, treatment options, and prognosis of patients with BCS-PVT. METHODS Patients diagnosed with nonmalignant BCS between 1984 and 2001 were identified in a large international study and classified into isolated BCS (n = 204), BCS-PVT without spleno-mesenteric vein thrombosis (SMVT; n = 15), and BCS-PVT with SMVT (n = 18). RESULTS Multifactorial etiology was present in 58% of patients with combined BCS-PVT. Number of etiological factors increased significantly with the extent of thrombosis (p = 0.002). Main treatment options included anticoagulation and portosystemic shunting, of which extended TIPS showed the most beneficial results. Five-year survival was 59% (95% CI 39-80%) in BCS-PVT versus 85% (95% CI 76-88%) in isolated BCS (p = 0.11). Survival tended to be worse in BCS-PVT patients with SMVT as compared to patients without SMVT (RR = 3.47, p = 0.11). CONCLUSIONS In BCS, extension of thrombosis into the splanchnic venous bed was significantly related to the number of etiological factors, and was associated with poor outcome. These results strongly support a liberal use of anticoagulants, which so far had been widely debated. Alternatively, derivative shunt procedures appear difficult, yet not impossible.
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Affiliation(s)
- Sarwa Darwish Murad
- Department of Gastroenterology and Hepatology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands
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Murray KF, Carithers RL. AASLD practice guidelines: Evaluation of the patient for liver transplantation. Hepatology 2005; 41:1407-32. [PMID: 15880505 DOI: 10.1002/hep.20704] [Citation(s) in RCA: 516] [Impact Index Per Article: 25.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Affiliation(s)
- Karen F Murray
- Division of Gastroenterology, University of Washington School of Medicine, Seattle, WA 98195-6174, USA
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Senzolo M, Cholongitas EC, Patch D, Burroughs AK. Update on the classification, assessment of prognosis and therapy of Budd–Chiari syndrome. ACTA ACUST UNITED AC 2005; 2:182-90. [PMID: 16265183 DOI: 10.1038/ncpgasthep0143] [Citation(s) in RCA: 66] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2004] [Accepted: 03/07/2005] [Indexed: 02/08/2023]
Abstract
Budd-Chiari syndrome (BCS) occurs as a result of obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCS--as fulminant, acute or chronic--is not prognostically useful. This makes assessing the benefit of therapy difficult, especially as there is no evidence from randomized studies. This article highlights advances in the prognosis and therapy of BCS. Identification of the site of venous obstruction has a major effect on prognosis. Portal-vein thrombosis occurs in 20-30% of cases, and acute presentation of BCS reflects an acute or chronic syndrome in 60% of BCS cases. BCS can be diagnosed and treated on a single occasion in the setting of the radiology department, with hepatic venography, transjugular liver biopsy, retrograde CO2 portography and inferior vena cava pressure measurements performed simultaneously with therapies such as dilation or stenting of webs in the inferior vena cava or hepatic veins, and placement of transjugular intrahepatic portosystemic shunts. Disruption of a portal vein thrombus can also be done during the same session. Surgical shunts have been superseded by the use of transjugular intrahepatic portosystemic shunts. Liver transplantation is reserved for fulminant and progressive chronic forms of BCS. Anticoagulation therapy must be used routinely, before and after specific therapy, regardless of whether a thrombophilic disorder is diagnosed.
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Barrault C, Plessier A, Valla D, Condat B. [Non surgical treatment of Budd-Chiari syndrome: a review]. ACTA ACUST UNITED AC 2004; 28:40-9. [PMID: 15041809 DOI: 10.1016/s0399-8320(04)94839-7] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
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Barakat M. Unusual hepatic-portal-systemic shunting demonstrated by Doppler sonography in children with congenital hepatic vein ostial occlusion. JOURNAL OF CLINICAL ULTRASOUND : JCU 2004; 32:172-178. [PMID: 15101077 DOI: 10.1002/jcu.20019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/24/2023]
Abstract
PURPOSE This report describes unusual changes in the hepatic vasculature in 3 children presenting with upper gastrointestinal hemorrhage. METHODS The study included 3 children (ages 5-8 years) who presented with hematemesis. All had mild hepatosplenomegaly and normal liver function. Esophageal varices were demonstrated in all on upper endoscopy. Color and spectral Doppler sonography was performed to assess the hepatic vasculature, including the hepatic veins (HVs), portal vein (PV), hepatic artery (HA), and inferior vena cava (IVC). RESULTS The HVs were all patent but with ostial occlusion at the point of their communication with the IVC. Complete flow reversal was shown inside the HVs, with blood draining into collateral vessels at the liver surface and paraumbilical vein. In one patient, the paraumbilical vein could be traced to its communication with the right external iliac vein. In all children, the direction of flow in the PV, HA, and IVC was normal. After endoscopic sclerotherapy, all children were shown to be in good general condition and to have normal liver function for a follow-up period of 15-36 months. CONCLUSIONS Ostial occlusion of the HV is a rare cause of hepatic outflow obstruction in children. Doppler sonography is a valuable, noninvasive imaging technique for evaluation of the hepatic vasculature and the accompanying shunting pathways in such cases.
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Affiliation(s)
- Maha Barakat
- Department of Tropical Medicine and Gastroenterology, Faculty of Medicine, Assiut University, Assiut, Egypt
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36
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Sharma S, Texeira A, Texeira P, Elias E, Wilde J, Olliff SP. Pharmacological thrombolysis in Budd Chiari syndrome: a single centre experience and review of the literature. J Hepatol 2004; 40:172-80. [PMID: 14672630 DOI: 10.1016/j.jhep.2003.09.028] [Citation(s) in RCA: 63] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/14/2023]
Abstract
BACKGROUND/AIMS To review our experience of thrombolytic therapy in patients with acute Budd Chiari syndrome (BCS). METHODS Records of 10 patients with BCS, treated by thrombolysis over a 12-year period were retrospectively analysed for demographics, clinical presentation/duration, primary disease, thrombolytic regimen, and follow-up. The same characteristics were also studied in previously reported patients. The agent used was recombinant tissue plasminogen activator (tPA) in all patients. RESULTS Thrombolysis was used 12 times in 10 patients. Infusion was made systemically in three patients, into the hepatic artery in one patient, locally into a hepatic vein and/or IVC in four patients and locally within TIPS/portal vein in two patients. Only one infusion made systemically was partially successful. Adjunctive balloon angioplasty and/or stent insertion was undertaken for all eight procedures (in six patients) where local infusion was into the hepatic vein or TIPS. Six of these were ultimately successful (in five patients) and two were unsuccessful. Thrombolysis was more likely to be successful in the presence of a short history of thrombosis, when the thrombolytic agent was locally infused and when it was combined with a successful radiological procedure. Mean follow-up was 4.5 years (range 1-10 years). No serious bleeding complication occurred. CONCLUSIONS We observed no benefit from thrombolysis when delivered systemically or arterially except in one case. Thrombolysis was useful in adjunctive management of BCS when the drug was infused locally into recently thrombosed veins that had appreciable flow following partial recanalisation. Thrombolysis was clearly of benefit in the repermeation of occluded/partially occluded hepatic veins/TIPS when early detection of new thrombus followed interventional procedures such as balloon angioplasty or stenting of hepatic veins.
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Affiliation(s)
- S Sharma
- Department of Radiology, Queen Elizabeth Hospital, University Hospital Birmingham NHS Trust, The Liver Unit, Birmingham B15 2TH, UK
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38
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Abstract
Shunting and transplantation are satisfactory methods of treating Budd-Chiari syndrome (BCS). Selection of treatment is based on the degree of hepatic injury (clinical settings), liver biopsy results, potential for parenchymal recovery, and pressure measurements. Shunting is recommended in cases of preserved hepatic function and architecture. In the presence of fulminant forms of BCS, in cases of established cirrhosis or frank fibrosis, or for patients with defined hepatic metabolic defects (e.g., protein C or protein S deficiency), liver transplantation is the treatment of choice. Nonsurgical alternatives, although encouraging, have limited long-term outcome results at the present time. In most cases of BCS, a thrombophilic disorder can be identified. However, it is important to note that postoperative vascular thrombosis has been identified in patients with BCS who do not have a definable hypercoagulable predisposition. It therefore is our practice to recommend early (<24 hours postoperatively) initiation of intravenous heparin therapy in all patients with BCS, who then undergo life-long anticoagulation with coumadin.
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Affiliation(s)
- Andrew S Klein
- Department of Surgery, Division of Transplantation, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
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Mancuso A, Fung K, Mela M, Tibballs J, Watkinson A, Burroughs AK, Patch D. TIPS for acute and chronic Budd-Chiari syndrome: a single-centre experience. J Hepatol 2003; 38:751-4. [PMID: 12763367 DOI: 10.1016/s0168-8278(03)00118-1] [Citation(s) in RCA: 91] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
BACKGROUND/AIMS Transjugular intrahepatic portosystemic shunt (TIPS) is a technically challenging but feasible treatment for Budd-Chiari syndrome (BCS). However, information about the outcome, particularly in patients with liver failure, is scarce. We report our experience of TIPS for BCS. METHODS Fifteen patients with BCS underwent TIPS. Eight had hepatic failure and seven underwent TIPS for BCS uncontrolled by medical treatment. RESULTS Fourteen out of 15 had successful TIPS placement. Out of the eight hepatic failure patients, four died soon after TIPS: one liver rupture, one portal vein rupture, one liver failure and one pulmonary oedema. Another patient had a significant intrahepatic haematoma, which resolved with conservative management. TIPS was successfully placed in all of the seven patients with chronic BCS, in whom there was an average follow-up of 20 months. Ascites resolved and liver function improved in all. One patient died after 18 months from the original hepatic metastatic disease. Four patients have had evidence of TIPS dysfunction requiring three balloon dilatations and one restenting. No patient has required liver transplantation. CONCLUSIONS TIPS should be the first line treatment for BCS uncontrolled by medical therapy. However, mortality in BCS with hepatic failure is high and liver transplantation could be a better option.
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Affiliation(s)
- Andrea Mancuso
- Scuola di Specializzazione in Gastroenterologia ed Endoscopia Digestiva, Reparto di Medicina, Ospedale V. Cervello, Universita' di Palermo, Via Trabucco 180, 90144, Palermo, Italy
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Mancuso A, Watkinson A, Tibballs J, Patch D, Burroughs AK. Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins. Gut 2003; 52:438. [PMID: 12584231 PMCID: PMC1773544 DOI: 10.1136/gut.52.3.438] [Citation(s) in RCA: 36] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Affiliation(s)
- A Mancuso
- Universita’ di Palermo, Scuola di Specializzazione in Gastroenterologia ed Endoscopia Digestiva, Reparto di Medicina, Ospedale V Cervello, Via Trabucco 180, 90144, Palermo, Italy
| | - A Watkinson
- Department of Radiology, Royal Free Hospital, London, UK
| | - J Tibballs
- Department of Radiology, Royal Free Hospital, London, UK
| | - D Patch
- Liver Transplantation Unit and Hepatobiliary Medicine, Royal Free Hospital, London, UK;
| | - A K Burroughs
- Liver Transplantation Unit and Hepatobiliary Medicine, Royal Free Hospital, London, UK;
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Cazals-Hatem D, Vilgrain V, Genin P, Denninger MH, Durand F, Belghiti J, Valla D, Degott C. Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers. Hepatology 2003; 37:510-9. [PMID: 12601347 DOI: 10.1053/jhep.2003.50076] [Citation(s) in RCA: 130] [Impact Index Per Article: 5.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Hepatic parenchymal changes associated with Budd-Chiari syndrome (BCS) have been tentatively explained by combined arterial and portal perfusion disturbances in addition to the complete occlusion of hepatic veins. The aim of this study was to correlate pretransplant course and vascular imaging with pathologic findings in livers explanted for BCS. Seventeen consecutive white patients who underwent transplantation for severe classic BCS were retrospectively analyzed. Pretransplant course was 1 year or less in 8 patients and more than 1 year in 9 patients. Thrombophilia was found in 16 patients (94%). Imaging showed decreased portal perfusion in 16 patients (94%) and increased arterial perfusion in 9 patients. Histology showed obstructive portal venopathy and nodular regenerative hyperplasia (NRH) aspects in all cases, large regenerative nodules resembling focal nodular hyperplasia (FNH) in 9 cases, and cirrhosis in 2 cases. Patients with increased arterial inflow had large regenerative nodules and a protracted pretransplant course. Patients with acute thrombi in portal veins had parenchymal infarcts (2 cases) and a short pretransplant course. In conclusion, patients with severe BCS have a constant impaired perfusion inflow unrelated to progression of cirrhosis but related to the outcome. An early decrease in portal perfusion is observed in the short term and is responsible for NRH or infarcts if complicated with large thrombi. An increase in arterial perfusion compensates impaired portal flow in chronic BCS. Arterial hyperemia contributes to the development of large regenerative nodules that are FNH-like. This pathologic situation offers an interesting vascular model to further understand the parenchymal response to changes in hepatic blood flow.
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Affiliation(s)
- Dominique Cazals-Hatem
- Service d'Anatomie-Pathologique, Laboratoire d'Hématologie et d'Immunologie, Hôpital Beaujon, Clichy, France.
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Janssen HLA, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003; 38:364-71. [PMID: 12586305 DOI: 10.1016/s0168-8278(02)00434-8] [Citation(s) in RCA: 324] [Impact Index Per Article: 14.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Affiliation(s)
- Harry L A Janssen
- Department of Gastroenterology and Hepatology, University Hospital Rotterdam, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands.
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Malkowski P, Michalowicz B, Pawlak J, Nyckowski P, Wroblewski T, Grzelak I, Najnigier B, Paczek L, Szczerbań J, Krawczyk M. Liver transplantation in patients with Budd-Chiari syndrome. Transplant Proc 2002; 34:629-30. [PMID: 12009646 DOI: 10.1016/s0041-1345(01)02869-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023]
Affiliation(s)
- Piotr Malkowski
- Department of General and Liver Surgery, Medical University of Warsaw, 1A Banacha Street, 02-097 Warsaw, Poland.
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Pfammatter T, Benoit C, Cathomas G, Blum U. Budd-Chiari syndrome with spleno-mesenteric-portal thrombosis: treatment with extended TIPS. J Vasc Interv Radiol 2000; 11:781-4. [PMID: 10877427 DOI: 10.1016/s1051-0443(07)61641-8] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Affiliation(s)
- T Pfammatter
- Institute of Diagnostic Radiology, University Hospital, Zurich, Switzerland.
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Leebeek FW, Lameris JS, van Buuren HR, Gomez E, Madretsma S, Sonneveld P. Budd-Chiari syndrome, portal vein and mesenteric vein thrombosis in a patient homozygous for factor V Leiden mutation treated by TIPS and thrombolysis. Br J Haematol 1998; 102:929-31. [PMID: 9734642 DOI: 10.1046/j.1365-2141.1998.00860.x] [Citation(s) in RCA: 39] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
We present the first case of Budd-Chiari syndrome in association with portal and mesenteric vein thrombosis in a patient homozygous for the factor V Leiden mutation. She was treated by transjugular intrahepatic portosystemic stent (TIPS) placement followed by local thrombolytic therapy. Venous outflow from the liver was established and the thrombi in the portal and mesenteric veins were lysed completely. This therapeutic approach may be used for such patients with this severe thrombotic event, who generally have a poor prognosis.
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Affiliation(s)
- F W Leebeek
- Department of Haematology, University Hospital Rotterdam Dijkzigt, The Netherlands
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