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Xue W, Xu B, Wang H, Zhu X, Qin J, Zhou G, Yu P, Li S, Jin Y. Development and validation of a machine learning model based on multiple kernel for predicting the recurrence risk of Budd-Chiari syndrome. Front Physiol 2025; 16:1589469. [PMID: 40519787 PMCID: PMC12162280 DOI: 10.3389/fphys.2025.1589469] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2025] [Accepted: 05/12/2025] [Indexed: 06/18/2025] Open
Abstract
Background Budd-Chiari syndrome (BCS) is a rare global condition with high recurrence rates. Existing prognostic scoring models demonstrate limited predictive efficacy for BCS recurrence. This study aims to develop a novel machine learning model based on multiple kernel learning to improve the prediction of 3-year recurrence in BCS patients. Methods Data were collected from BCS patients admitted to the Affiliated Hospital of Xuzhou Medical University between January 2015 and July 2022. The dataset was divided into training, validation, and test sets in a 6:2:2 ratio. Models were constructed by evaluating all combinations of four kernel functions in the training set. Hyperparameters for each model were optimized using the particle swarm optimization (PSO) algorithm on the validation set. The test set was used to compare kernel function combinations, with the area under the curve (AUC), sensitivity, specificity, and accuracy as evaluation metrics. The optimal model, identified through the best-performing kernel combination, was further compared with three classical machine learning models. Result A kernel combination integrating all four basic kernels achieved the highest average AUC (0.831), specificity (0.772), and accuracy (0.780), along with marginally lower but more stable sensitivity (0.795) compared to other combinations. When benchmarked against classical machine learning models, our proposed MKSVRB (Multi-Kernel Support Vector Machine Model for Three-Year Recurrence Prediction of Budd-Chiari Syndrome) demonstrated superior performance. Additionally, it outperformed prior studies addressing similar objectives. Conclusion This study identifies risk factors influencing BCS recurrence and validates the MKSVRB model as a significant advancement over existing prediction methods. The model exhibits strong potential for early detection, risk stratification, and recurrence prevention in BCS patients.
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Affiliation(s)
- Weirong Xue
- School of Public Health, Xuzhou Medical College, Xuzhou, China
| | - Bing Xu
- Department of Otorhinolaryngology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu, China
| | - Hui Wang
- Department of Hepatobiliary Surgery, Xuzhou Central Hospital, Xuzhou, Jiangsu, China
| | - Xiaoxiao Zhu
- School of Public Health, Xuzhou Medical College, Xuzhou, China
| | - Jiajia Qin
- School of Public Health, Xuzhou Medical College, Xuzhou, China
| | | | - Peilin Yu
- School of Public Health, Xuzhou Medical College, Xuzhou, China
| | - Shengli Li
- Clinical Research Institute, The Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Yingliang Jin
- Department of Biostatistics, Center for Medical Statistics and Data Analysis, School of Public Health, Key Laboratory of Human Genetics and Environmental Medicine Xuzhou Medical College, Xuzhou, China
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Tripathy TP, Patel R, Behera S, Pattnaik B, Dutta T, Mohakud S, Gupta S, Mohapatra AK, Sahoo D, Naik S, Nayak HK, Mohanty RR, Panigrahi MK. The Change in Liver Volume After Inferior Vena Cava and/or Hepatic Vein Venoplasty in Patients With Budd Chiari Syndrome With at Least One Patent Hepatic Vein Presenting With Ascites. J Clin Exp Hepatol 2025; 15:102486. [PMID: 39868011 PMCID: PMC11754519 DOI: 10.1016/j.jceh.2024.102486] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2024] [Accepted: 12/04/2024] [Indexed: 01/28/2025] Open
Abstract
Objective To assess the effects of inferior vena cava and/or hepatic vein (IVC±HV) venoplasty on liver volumetry and function in individuals with Budd Chiari syndrome (BCS) who present with ascites and at least one patent hepatic vein. Methods A retrospective analysis was conducted on the clinical data of 17 patients with BCS (6 males and 11 females, average age of 42.3 ± 11.9 years) who underwent IVC venoplasty for ascites caused by IVC blockage and at least one patent HV, either pre- or post-venoplasty. Liver function tests and abdominal CT scans were performed before the procedure and at three and six months post-venoplasty. The changes in liver function and volume before and after venoplasty were analyzed. Results Each of the 17 patients successfully underwent IVC±HV venoplasty. During the median follow-up period of six months, all patients survived. Comparisons with preoperative conditions showed significant improvements in ascites and liver function three and six months after the procedure (P < 0.05). The liver volumes measured before and at three- and six-months post-procedure were 2077.06 ± 185.53 cm³, 1742.00 ± 124.62 cm³, and 1632.71 ± 108.29 cm³, respectively. There was a significant decrease in liver volume between the pre-operative measurements and the three-month follow-up, as well as between the three-month and six-month follow-ups (P < 0.05). Conclusions IVC±HV venoplasty produced satisfactory clinical results in BCS patients. Following the intervention, there was a progressive decrease in hepatic congestion and an improvement in liver function which correlated with decrease in liver volume.
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Affiliation(s)
| | - Ranjan Patel
- Department of Radiodiagnosis, AIIMS, Bhubaneswar, India
| | - Srikant Behera
- Department of General Medicine, AIIMS, Bhubaneswar, India
| | | | - Tanmay Dutta
- Department of Surgical Gastroenterology, AIIMS, Bhubaneswar, India
| | | | - Sunita Gupta
- Department of Surgical Gastroenterology, AIIMS, Bhubaneswar, India
| | | | | | - Suprava Naik
- Department of Radiodiagnosis, AIIMS, Bhubaneswar, India
| | - Hemant K. Nayak
- Department of Medical Gastroenterology, AIIMS, Bhubaneswar, India
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Zahid M, Es-Salim M, Moursi MO, Mahmood N, Elzouki AN. Hepatic vena cava syndrome: a case report and literature review. Oxf Med Case Reports 2025; 2025:omaf035. [PMID: 40443867 PMCID: PMC12118078 DOI: 10.1093/omcr/omaf035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2024] [Revised: 02/04/2025] [Accepted: 02/06/2025] [Indexed: 06/02/2025] Open
Abstract
BACKGROUND Hepatic vena cava syndrome (HVCS) is an uncommon condition, with no prior reports from the Gulf area. In regions with limited resources, where this disease is found, there is a lack of data due to underdiagnosis. HVCS results from recurrent bacterial thrombophlebitis of the hepatic portion of the inferior vena cava (IVC), causing intimal thickening and obstruction, eventually leading to complications like liver cirrhosis and hepatocellular carcinoma. CASE PRESENTATION A 32-year-old Afghan refugee lady, presented with progressive abdominal distention. Initial investigations showed anemia, splenomegaly, and massive ascites. Further investigations revealed cirrhotic liver, varices, and a stenotic segment of the IVC. She was diagnosed with HVCS, underwent IVC stenting, and commenced on clopidogrel and dabigatran. Conclusion Often misdiagnosed, HVCS requires a high index of suspicion and should be considered in patients with liver cirrhosis with low socioeconomic backgrounds. Early diagnosis can prevent liver cirrhosis and hepatocellular carcinoma.
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Affiliation(s)
- Muhammad Zahid
- Department of Internal Medicine, Hamad General Hospital, Doha, Qatar
- College of Medicine, QU Health, Qatar University, Doha, Qatar
- Weill Cornell Medical College, Qatar
| | - Mohammad Es-Salim
- Department of Internal Medicine, Hamad General Hospital, Doha, Qatar
| | - Moaz O Moursi
- Department of Internal Medicine, Hamad General Hospital, Doha, Qatar
- College of Medicine, QU Health, Qatar University, Doha, Qatar
| | - Nabil Mahmood
- Weill Cornell Medical College, Qatar
- Department of Radiology, Hamad General Hospital, Doha, Qatar
| | - Abdel-Naser Elzouki
- Department of Internal Medicine, Hamad General Hospital, Doha, Qatar
- College of Medicine, QU Health, Qatar University, Doha, Qatar
- Weill Cornell Medical College, Qatar
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Husieva SA, Osyodlo GV, Husiev AV, Savichan KV. A Clinical Case of Timely Diagnosis and Successful Treatment of Budd-Chiari Syndrome With Fulminant Cytolysis in the Setting of a First-time Diagnosed Myeloproliferative Disease. Mil Med 2025; 190:1292-1299. [PMID: 38935394 DOI: 10.1093/milmed/usae331] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2024] [Revised: 05/03/2024] [Accepted: 06/23/2024] [Indexed: 06/28/2024] Open
Abstract
The article presents a clinical case of peculiarities of clinical manifestations, diagnostic and therapeutic approaches of undiagnosed chronic myeloproliferative disease, on the background of which Budd-Chiari syndrome (BCS) developed. The results of clinical course, examination, and treatment of a patient with BCS as a manifestation of the hidden course of primary myelofibrosis with the presence of somatic mutation (V617F) in Janus-tyrosine kinase-2 (JAK2) gene in myeloid cells are presented. Standard clinical and laboratory examinations, and cytomorphologic and histologic examination of bone marrow were used. The diagnosis of BCS was confirmed by ultrasound (US) Doppler examination of the portal system vessels. Symptomatic therapy resulted in insignificant positive results. The analysis of this clinical case showed that the development of BCS was due to a chronic myeloproliferative disease that was not diagnosed before the development of thrombosis. Hepatic vein thrombosis was accompanied by the development of fulminant cytolytic syndrome. Along with symptomatic therapy, patient K., female, 32 years old, underwent transjugular intrahepatic portosystemic shunting 1 month after the first symptoms of BCS appeared, which contributed to a significant clinical effect. Seven years after the installation of 4 transjugular intrahepatic portosystemic shunts, the patient's condition remains satisfactory. The uniqueness of this clinical case lies in the presence of 2 serious diseases at the same time: myeloproliferative pathology (primary myelofibrosis) JAK2-positive variant and BCS. Timely diagnosis of both hematological diseases and their complication in the form of hepatic vein thrombosis with fulminant cytolytic syndrome allowed timely prescription of adequate treatment with a good clinical response.
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Affiliation(s)
- Svitlana A Husieva
- Military Therapy Department, Ukrainian Military Medical Academy, Kyiv 01015, Ukraine
| | - Galyna V Osyodlo
- Military Therapy Department, Ukrainian Military Medical Academy, Kyiv 01015, Ukraine
| | - Andrii V Husiev
- Department of Liver Surgery and Transplantation, Shalimov National Scientific Center of Surgery and Transplantation of the National Academy of Medical Sciences of Ukraine, Kyiv 03680, Ukraine
| | - Kyrylo V Savichan
- Military Therapy Department, Ukrainian Military Medical Academy, Kyiv 01015, Ukraine
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Nigam N, Yadav R, Pandey G, Hashim Z, Bihari C, Vishwadeep R, Kumar N, Mishra P. The histological grading of fibrosis in Budd-Chiari syndrome: A chronic liver disease, different from others. Indian J Gastroenterol 2025; 44:188-197. [PMID: 39546183 DOI: 10.1007/s12664-024-01690-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2024] [Accepted: 09/10/2024] [Indexed: 11/17/2024]
Abstract
INTRODUCTION Budd-Chiari syndrome (BCS) is an uncommon disease caused by hepatic venous outflow obstruction. They can result in centrilobular fibrosis, nodular regenerative hyperplasia and cirrhosis. Assessing liver fibrosis is crucial for determining the stage of BCS, predicting disease progression and guiding treatment decisions. Although this pathology has been known for decades, no useful grading system was assigned. This study aims to introduce a histologic fibrosis grading system for BCS patients. METHODOLOGY All patients from 2017 to 2022 (Sanjay Gandhi Postgraduate Institute of Medical Sciences [SGPGIMS]), Lucknow diagnosed with BCS for whom liver biopsy was performed were included in the study. The Budd-Chiari syndrome-Hepatic Fibrosis system (BCS-HFS) was implemented to grade fibrosis. The fibrosis grade was compared with the fibrosis percentage area and a correlation was found with the hemodynamic variables (hepatic venous pressure gradient [HVPG]) and the prognostic scores. RESULTS There were 56 patients with BCS. The median age was 27 years, with a male-female ratio of 1.8:1. There was a significant difference in the fibrosis percentage, hemorrhage percentage and model for end-stage liver disease (MELD) score among the BCS-HFS grades (p < 0.05). There was a significant correlation between BCS-HFS and HVPG (ρ = 0.699, p < 0.001) and the MELD prognostic score (ρ = 0.474, p < 0.001). CONCLUSION BCS-HFS is applicable for grading fibrosis in BCS. It can help in uniform histopathology reporting and for further prospective and comparative studies.
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Affiliation(s)
- Neha Nigam
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India.
| | - Rajanikant Yadav
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Gaurav Pandey
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Zia Hashim
- Department of Pulmonary Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Chhagan Bihari
- Department of Pathology, Institute of Liver and Biliary Sciences, New Delhi, 110 070, India
| | - Rana Vishwadeep
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Nirbhay Kumar
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
| | - Prabhakar Mishra
- Department of Biostatistics and Health Informatics, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India
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Horner G, Luger G, McGrath M, Sharma A, Bloom DA, Shakhin V. Unilateral Leg Swelling and Liver Enzyme Elevation in an Adolescent. Pediatrics 2025:e2024067246. [PMID: 39965644 DOI: 10.1542/peds.2024-067246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2024] [Accepted: 12/16/2024] [Indexed: 02/20/2025] Open
Abstract
A previously healthy 15-year-old female developed sudden onset right lower extremity swelling, pain, and erythematous linear streaking from the ankle to mid-thigh. Duplex venous ultrasound revealed multiple superficial and deep venous thrombi in the right lower extremity. Incidentally, the patient was also noted to have elevated transaminases and a microcytic anemia with significant iron deficiency. Additional evaluation ultimately led to the diagnosis of 2 distinct but interconnected chronic conditions, one of which progressed to requiring liver transplantation.
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Affiliation(s)
- Gabrielle Horner
- University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan
| | - Grace Luger
- University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan
| | - Mary McGrath
- Department of Pediatric Hematology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan
| | - Anita Sharma
- Department of Pediatric Gastroenterology and Hepatology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan
| | - David A Bloom
- Department of Pediatric Radiology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan
| | - Victoria Shakhin
- Department of Pediatric Gastroenterology and Hepatology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan
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Ma H, Gou Y, Wang C, Xiao J, Shen B, Liu H, Huang Q, Xu H, Zu M, Zhang Q. Feasibility and long-term outcomes of hepatic vein recanalization in Budd-Chiari syndrome through intrahepatic collateral pathways. Eur J Gastroenterol Hepatol 2024; 36:1230-1237. [PMID: 39012650 DOI: 10.1097/meg.0000000000002822] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 07/17/2024]
Abstract
BACKGROUND AND AIMS To investigate the feasibility and long-term outcomes of hepatic vein (HV) recanalization using intrahepatic collateral pathways in patients with Budd-Chiari syndrome (BCS) with HV obstruction. METHODS Clinical data of 29 BCS patients with HV obstruction and intrahepatic collateral pathways were reviewed. All patients underwent HV recanalization through the intrahepatic collaterals. Follow-up was performed at 1, 3, 6, and 12 months after treatment and annually thereafter. Cumulative patency and survival rates were assessed using Kaplan-Meier curves. The independent predictors of patency were determined using a Cox regression model. RESULTS HV recanalization was successful in 28 of the 29 patients (96.6%), with no complications. Of the 28 cases, simultaneous recanalization of the accessory HV and right HV was achieved in 11 patients, accessory HV and middle HV in six, accessory HV and left HV in three, right HV and middle HV in five, and left HV and middle HV in three. Twenty-eight patients were followed from 4 to 87 (mean, 53.6 ± 26.7) months after treatment, and six patients developed reocclusion. The overall cumulative 1-, 3-, 5-, and 7-year primary HV patency rates were 96.3, 82.9, 74.6, and 59.7%, respectively. The cumulative 1-, 3-, 5-, and 7-year survival rates were 100, 95.8, 95.8, and 86.3%, respectively. CONCLUSION Interventional treatment of HV obstruction in BCS patients through intrahepatic collateral approaches is well tolerated and feasible and can result in excellent long-term patency and survival rates.
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Affiliation(s)
- Hongru Ma
- Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
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Joueidi F, Alhanaee A, Alsuhaibani H, Albenmousa A, Joueidi A, Elhassan A, Nasir AN, Marquez KAH, Alghamdi S, Al Hamoudi W, Abualganam S, Broering D, Bzeizi KI. Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: A Single-Centre Experience. J Clin Med 2024; 13:5858. [PMID: 39407918 PMCID: PMC11478255 DOI: 10.3390/jcm13195858] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2024] [Revised: 08/08/2024] [Accepted: 09/14/2024] [Indexed: 10/20/2024] Open
Abstract
Background: Despite several challenges in clinical management, there has been significant progress in understanding the aetiology, natural history and outcomes of Budd-Chiari syndrome (BCS) treatments. This study aims to evaluate the outcomes of transjugular intrahepatic portosystemic shunt (TIPS) using covered stent in management of BCS. Methods: We conducted a retrospective analysis of 70 BCS patients who underwent TIPS using covered stents between January 2010 and December 2022 at a single tertiary liver transplant centre. Patients' clinical features, laboratory parameters, and imagine findings were collected before and after TIPS. The primary endpoint was overall survival. Results: TIPS was performed on 70 patients with BCS out of a total of 88 patients. The remaining patients (18) underwent liver transplantation. The mean age was 37.7 ± 11.2 years at time of diagnosis and the majority were female (64.35). The most common symptoms and signs at presentation were abdominal pain, jaundice, ascites, and variceal bleeding. Over a median followup of 76 months, the survival rates at 1, 3, and 5 years were 98.8%, 97.9%, and 97.7%, respectively. Patients who underwent TIPS alone had better survival that patients with BCS who required liver transplantation (LTx) (p = 0.003). Conclusions: In our study TIPS provided a highly effective treatment option for BCS patients. The long-term favourability of the outcome was not impacted by the need for repeated TIPS revision. Use of covered stents was instrumental in reducing shunt dysfunction rates. Prospective and larger studies are needed to further optimize therapeutic strategies in this challenging population.
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Affiliation(s)
- Faisal Joueidi
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Amnah Alhanaee
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Hamad Alsuhaibani
- Radiology Department, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (H.A.); (S.A.)
| | - Ali Albenmousa
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Ahmad Joueidi
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Ahmed Elhassan
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Abdallah Nabeel Nasir
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Kris Ann Hervera Marquez
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Saad Alghamdi
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Waleed Al Hamoudi
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Saad Abualganam
- Radiology Department, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (H.A.); (S.A.)
| | - Dieter Broering
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Khalid Ibrahim Bzeizi
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
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Lu Q, Zhu R, Zhou L, Zhang R, Li Z, Xu P, Wang Z, Wu G, Ren J, Jiao D, Song Y, Li J, Wang W, Liang R, Ma X, Sun Y. Gut dysbiosis contributes to the development of Budd-Chiari syndrome through immune imbalance. mSystems 2024; 9:e0079424. [PMID: 39166878 PMCID: PMC11406926 DOI: 10.1128/msystems.00794-24] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2024] [Accepted: 07/17/2024] [Indexed: 08/23/2024] Open
Abstract
UNLABELLED Budd-Chiari syndrome (B-CS) is a rare and lethal condition characterized by hepatic venous outflow tract blockage. Gut microbiota has been linked to numerous hepatic disorders, but its significance in B-CS pathogenesis is uncertain. First, we performed a case-control study (Ncase = 140, Ncontrol = 63) to compare the fecal microbiota of B-CS and healthy individuals by metagenomics sequencing. B-CS patients' gut microbial composition and activity changed significantly, with a different metagenomic makeup, increased potentially pathogenic bacteria, including Prevotella, and disease-linked microbial function. Imbalanced cytokines in patients were demonstrated to be associated with gut dysbiosis, which led us to suspect that B-CS is associated with gut microbiota and immune dysregulation. Next, 16S ribosomal DNA sequencing on fecal microbiota transplantation (FMT) mice models examined the link between gut dysbiosis and B-CS. FMT models showed damaged liver tissues, posterior inferior vena cava, and increased Prevotella in the disturbed gut microbiota of FMT mice. Notably, B-CS-FMT impaired the morphological structure of colonic tissues and increased intestinal permeability. Furthermore, a significant increase of the same cytokines (IL-5, IL-6, IL-9, IL-10, IL-17A, IL-17F, and IL-13) and endotoxin levels in B-CS-FMT mice were observed. Our study suggested that gut microbial dysbiosis may cause B-CS through immunological dysregulation. IMPORTANCE This study revealed that gut microbial dysbiosis may cause Budd-Chiari syndrome (B-CS). Gut dysbiosis enhanced intestinal permeability, and toxic metabolites and imbalanced cytokines activated the immune system. Consequently, the escalation of causative factors led to their concentration in the portal vein, thereby compromising both the liver parenchyma and outflow tract. Therefore, we proposed that gut microbial dysbiosis induced immune imbalance by chronic systemic inflammation, which contributed to the B-CS development. Furthermore, Prevotella may mediate inflammation development and immune imbalance, showing potential in B-CS pathogenesis.
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Affiliation(s)
- Qinwei Lu
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
| | - Rongtao Zhu
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
| | - Lin Zhou
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
- Department of Gastroenterology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Ruifang Zhang
- Department of Ultrasound Diagnosis, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Zhen Li
- Department of Endovascular Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Peng Xu
- Department of Interventional Radiology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Zhiwei Wang
- Department of Interventional Radiology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Gang Wu
- Department of Vascular Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Jianzhuang Ren
- Department of Vascular Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Dechao Jiao
- Department of Vascular Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yan Song
- Department of Vascular Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Jian Li
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
| | - Weijie Wang
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
| | - Ruopeng Liang
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
| | - Xiuxian Ma
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
| | - Yuling Sun
- Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Key Lab of Hepatobiliary and Pancreatic Diseases, Zhengzhou, China
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10
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Berg T, Aehling NF, Bruns T, Welker MW, Weismüller T, Trebicka J, Tacke F, Strnad P, Sterneck M, Settmacher U, Seehofer D, Schott E, Schnitzbauer AA, Schmidt HH, Schlitt HJ, Pratschke J, Pascher A, Neumann U, Manekeller S, Lammert F, Klein I, Kirchner G, Guba M, Glanemann M, Engelmann C, Canbay AE, Braun F, Berg CP, Bechstein WO, Becker T, Trautwein C. [Not Available]. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2024; 62:1397-1573. [PMID: 39250961 DOI: 10.1055/a-2255-7246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/11/2024]
Affiliation(s)
- Thomas Berg
- Bereich Hepatologie, Medizinischen Klinik II, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Niklas F Aehling
- Bereich Hepatologie, Medizinischen Klinik II, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Tony Bruns
- Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Deutschland
| | - Martin-Walter Welker
- Medizinische Klinik I Gastroent., Hepat., Pneum., Endokrin. Universitätsklinikum Frankfurt, Frankfurt, Deutschland
| | - Tobias Weismüller
- Klinik für Innere Medizin - Gastroenterologie und Hepatologie, Vivantes Humboldt-Klinikum, Berlin, Deutschland
| | - Jonel Trebicka
- Medizinische Klinik B für Gastroenterologie und Hepatologie, Universitätsklinikum Münster, Münster, Deutschland
| | - Frank Tacke
- Charité - Universitätsmedizin Berlin, Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Campus Virchow-Klinikum (CVK) und Campus Charité Mitte (CCM), Berlin, Deutschland
| | - Pavel Strnad
- Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Deutschland
| | - Martina Sterneck
- Medizinische Klinik und Poliklinik I, Universitätsklinikum Hamburg, Hamburg, Deutschland
| | - Utz Settmacher
- Klinik für Allgemein-, Viszeral- und Gefäßchirurgie, Universitätsklinikum Jena, Jena, Deutschland
| | - Daniel Seehofer
- Klinik für Viszeral-, Transplantations-, Thorax- und Gefäßchirurgie, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Eckart Schott
- Klinik für Innere Medizin II - Gastroenterologie, Hepatologie und Diabetolgie, Helios Klinikum Emil von Behring, Berlin, Deutschland
| | | | - Hartmut H Schmidt
- Klinik für Gastroenterologie und Hepatologie, Universitätsklinikum Essen, Essen, Deutschland
| | - Hans J Schlitt
- Klinik und Poliklinik für Chirurgie, Universitätsklinikum Regensburg, Regensburg, Deutschland
| | - Johann Pratschke
- Chirurgische Klinik, Charité Campus Virchow-Klinikum - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Andreas Pascher
- Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Münster, Münster, Deutschland
| | - Ulf Neumann
- Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Essen, Essen, Deutschland
| | - Steffen Manekeller
- Klinik und Poliklinik für Allgemein-, Viszeral-, Thorax- und Gefäßchirurgie, Universitätsklinikum Bonn, Bonn, Deutschland
| | - Frank Lammert
- Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
| | - Ingo Klein
- Chirurgische Klinik I, Universitätsklinikum Würzburg, Würzburg, Deutschland
| | - Gabriele Kirchner
- Klinik und Poliklinik für Chirurgie, Universitätsklinikum Regensburg und Innere Medizin I, Caritaskrankenhaus St. Josef Regensburg, Regensburg, Deutschland
| | - Markus Guba
- Klinik für Allgemeine, Viszeral-, Transplantations-, Gefäß- und Thoraxchirurgie, Universitätsklinikum München, München, Deutschland
| | - Matthias Glanemann
- Klinik für Allgemeine, Viszeral-, Gefäß- und Kinderchirurgie, Universitätsklinikum des Saarlandes, Homburg, Deutschland
| | - Cornelius Engelmann
- Charité - Universitätsmedizin Berlin, Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Campus Virchow-Klinikum (CVK) und Campus Charité Mitte (CCM), Berlin, Deutschland
| | - Ali E Canbay
- Medizinische Klinik, Universitätsklinikum Knappschaftskrankenhaus Bochum, Bochum, Deutschland
| | - Felix Braun
- Klinik für Allgemeine Chirurgie, Viszeral-, Thorax-, Transplantations- und Kinderchirurgie, Universitätsklinikum Schlewswig-Holstein, Kiel, Deutschland
| | - Christoph P Berg
- Innere Medizin I Gastroenterologie, Hepatologie, Infektiologie, Universitätsklinikum Tübingen, Tübingen, Deutschland
| | - Wolf O Bechstein
- Klinik für Allgemein- und Viszeralchirurgie, Universitätsklinikum Frankfurt, Frankfurt, Deutschland
| | - Thomas Becker
- Klinik für Allgemeine Chirurgie, Viszeral-, Thorax-, Transplantations- und Kinderchirurgie, Universitätsklinikum Schlewswig-Holstein, Kiel, Deutschland
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11
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Narne KG, K I S N V, Kakumani J, T V, A GS. Revealing an Exceptional Case of Budd-Chiari Syndrome. Cureus 2024; 16:e69949. [PMID: 39445237 PMCID: PMC11496205 DOI: 10.7759/cureus.69949] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2024] [Accepted: 09/22/2024] [Indexed: 10/25/2024] Open
Abstract
Budd-Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction, leading to substantial effects, which include liver congestion, ascites, and liver failure. This is an unusual but very important form of secondary BCS caused by extraluminal compression from structures such as abscesses, tumors, or cysts. This case study exemplifies a 46-year-old female with no previous medical history who developed BCS due to the encasement of the IVC and hepatic veins secondary to a hydatid cyst, which is an uncommon presentation. Distension, ascites, and pain in the abdomen are the chief complaints. Investigations were positive for a hydatid cyst in the liver on imaging and serology, as well as features commensurate with BCS. The patient received conservative management with albendazole and anticoagulation. However, she refused cyst surgical resection and thus died three weeks after her discharge because of a cardiorespiratory arrest possibly associated with complications of her hydatid cyst like anaphylaxis secondary to rupture of the hydatid cyst. Therefore, this case brings out the significance of considering hydatid cysts when making a differential diagnosis on patients suffering from BCS, especially in endemic areas, and also underscores the importance of early recognition, treatment measures, and education to avoid fatal conditions.
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Affiliation(s)
- Krishna Geetha Narne
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND
| | - Vaishnavi K I S N
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND
| | - Jagadeswar Kakumani
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND
| | - Vivekanandan T
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND
| | - Gowri Shankar A
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND
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12
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Custo S, Tabone E, Aquilina A, Gatt A, Riva N. Splanchnic Vein Thrombosis: The State-of-the-Art on Anticoagulant Treatment. Hamostaseologie 2024; 44:242-254. [PMID: 38354834 DOI: 10.1055/a-2232-5480] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/16/2024] Open
Abstract
Splanchnic vein thrombosis (SVT) is a rare type of venous thromboembolism occurring within the splanchnic venous system. Portal vein thrombosis is the most common presentation, while Budd-Chiari syndrome is the least common. Liver cirrhosis and abdominal solid cancer are the main local risk factors for SVT, whereas myeloproliferative neoplasms are the predominant systemic risk factors. Signs and symptoms of SVT are nonspecific and include abdominal pain, gastrointestinal bleeding, and ascites. Asymptomatic SVT is not uncommon, and the majority would be detected incidentally on routine abdominal imaging performed for the follow-up of liver diseases and tumors. The management of SVT aims to prevent thrombus progression, promote vessel recanalization, and prevent recurrent venous thromboembolism. Anticoagulation should be started early in order to increase the chances of vessel recanalization and reduce the risk of portal hypertension-related complications. Direct oral anticoagulants have been included in recent guidelines, as alternatives to vitamin K antagonists, after clinical stability has been reached; however, caution is required in patients with liver or kidney dysfunction. Treatment duration is based on the presence (or absence) and type (transient vs. permanent) of risk factors. This narrative review aims to summarize the latest evidence on SVT, with a particular focus on the anticoagulant treatment in special categories of patients (e.g., liver cirrhosis, solid cancer, myeloproliferative neoplasms, pancreatitis, incidentally detected SVT, Budd-Chiari syndrome, and chronic SVT).
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Affiliation(s)
- Scott Custo
- Medical School, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Emma Tabone
- Medical School, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Alexia Aquilina
- Medical School, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Alex Gatt
- Department of Pathology, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
| | - Nicoletta Riva
- Department of Pathology, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
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13
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Saad Eddin A, Ramzan U, Saad Aldin E. Budd-Chiari syndrome treated with direct intrahepatic portocaval shunt: A case report. Radiol Case Rep 2024; 19:2608-2612. [PMID: 38645960 PMCID: PMC11026531 DOI: 10.1016/j.radcr.2024.03.036] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2023] [Revised: 03/09/2024] [Accepted: 03/13/2024] [Indexed: 04/23/2024] Open
Abstract
Budd-Chiari syndrome is an uncommon disorder characterized by occlusion of hepatic veins. It can lead to portal hypertension. Most common causes of this syndrome are hypercoagulability states. Transjugular intrahepatic portosystemic shunt is often not possible given the portal venous thrombosis. In these cases, direct intrahepatic portocaval shunt, involving the creation of an access between the portal vein and the systemic circulation via the intrahepatic inferior vena cava has proven to be a feasible alternative, and can improve portal hypertension in these patients. Herein, we present a case of a 37-year-old woman diagnosed with Budd-Chiari syndrome that was successfully treated with percutaneous ultrasound (US)-guided direct intrahepatic portocaval shunt.
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Affiliation(s)
- Assim Saad Eddin
- University of Iowa Hospitals and Clinics, Department of Radiology, 200 Hawkins Dr, Iowa City, IA 52242, USA
| | - Umar Ramzan
- Northwestern University Feinberg School of Medicine, 420 E Superior St, Chicago, IL 60611, USA
| | - Ehab Saad Aldin
- University of Nebraska Medical Center, Department of Radiology, 42nd and Emile St, Omaha, NE 68198, USA
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14
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Moreno MOA, Paz CLDSL, Dezan MGF, Cavalcante LN, Lyra AC. TEN-YEAR OUTCOMES OF TIPS FOR BUDD-CHIARI SYNDROME: SYSTEMATIC REVIEW AND META-ANALYSIS. ARQUIVOS DE GASTROENTEROLOGIA 2024; 61:e23171. [PMID: 38896573 DOI: 10.1590/s0004-2803.24612023-171] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/12/2023] [Accepted: 02/23/2024] [Indexed: 06/21/2024]
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) results from the obstruction of the hepatic venous flow, usually at the level of the hepatic vein or inferior vena cava. When left untreated, it can progress with several complications, including liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) appears to be effective in a subgroup of BCS patients. OBJECTIVE To perform a systematic review and meta-analysis of TIPS effectiveness in BCS treatment, considering the survival rate, reduction in portosystemic pressure, need for liver transplantation, technical failure, and shunt dysfunction for up to 10 years of follow-up. METHODS We evaluated 17 studies published in PubMed, Science Direct, Web of Science, and SCOPUS databases, which used TIPS as a treatment for BCS, comprising 618 subjects between 18 and 78 years old. We assessed the bias risk by the NOS, NHI, and JBI scales for cohort stu-dies, before-after studies, and case series, respectively. We conducted the meta-analyses by extracting the number of events and the total patients evaluated to perform the proportion meta-analyses using the R software ("meta" package - version 4.9-6). RESULTS The pooled results (95%CI) showed a 19% (25.9-12.5%) rate of portosystemic pressure reduction, 6% (1-12%) rate for the need for liver transplants despite the use of TIPS, 2% (1-6%) technical failure rate, 30% (18-46%) shunt dysfunction rate, and 88% (81-93%) for the mean frequency of patients alive between 1 and 10 years after the procedure. We stratified survival rate and found an 86% (74-93%) prevalence of living subjects during less than five years, 92% (83-97%) at five years, and a 77% frequency (68-83%) of patients alive ten years after the TIPS placement. CONCLUSION TIPS is an effective treatment for BCS, providing a high 10-year frequency of living patients and a significant decrease in portosystemic pressure. The need for liver transplants after TIPS and the technical failure rate is low.
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Affiliation(s)
| | | | - Maria Gabriela Fernandes Dezan
- Escola Bahiana de Medicina e Saúde Pública; Salvador, BA, Brasil
- Hospital São Rafael, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
- Universidade Federal da Bahia, PPgMs, Salvador, BA, Brasil
| | - Lourianne Nascimento Cavalcante
- Hospital São Rafael, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
- Universidade Federal da Bahia, PPgMs, Salvador, BA, Brasil
- Hospital Universitário Professor Edgard Santos, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
| | - Andre Castro Lyra
- Hospital São Rafael, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
- Universidade Federal da Bahia, PPgMs, Salvador, BA, Brasil
- Hospital Universitário Professor Edgard Santos, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
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15
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Perini MV, Ischia J, Woon D, Bhaskar J, Starkey G, Qi S, Wetherell D, Ellard L, McCall P, Miles LF, Seevanayagam S. A novel two-stage approach to the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome. BJU Int 2024; 133:480-486. [PMID: 38102752 DOI: 10.1111/bju.16257] [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: 12/17/2023]
Abstract
OBJECTIVE To present the early results of a new technique for the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome. PATIENTS AND METHODS The first stage involves transdiaphragmatic debulking of the right heart, inferior vena cava (IVC) and hepatic veins via median sternotomy, followed by a purse-string suture placed in the IVC below the hepatic veins. The second stage is performed separately and involves en bloc resection of the affected kidney, and IVC and vascular reconstruction via an abdominal incision. RESULTS Three of five patients presented with clinical Budd-Chiari syndrome; two had radiological features only. The median time between surgical procedures was 12 days (IQR 13 days). Four of the five patients had a R0 resection. While all five patients successfully completed both operative stages, one patient died 22 days after the second stage. Of the remaining four, all survive with no disease recurrence. CONCLUSION While we continue to compile longer-term data for a larger follow-up series, these preliminary findings show the feasibility of this technique and support the development of this programme of surgery.
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Affiliation(s)
- Marcos V Perini
- Department of Surgery (Austin Health), The University of Melbourne, Melbourne, Victoria, Australia
- Victorian Liver Transplant Unit, Austin Health, Melbourne, Victoria, Australia
| | - Joseph Ischia
- Department of Surgery (Austin Health), The University of Melbourne, Melbourne, Victoria, Australia
- Urology Unit, Austin Health, Melbourne, Victoria, Australia
| | - Dixon Woon
- Urology Unit, Austin Health, Melbourne, Victoria, Australia
| | - Jayapadman Bhaskar
- Brian F. Buxton Cardiac and Thoracic Aortic Surgery Unit, Austin Health, Melbourne, Victoria, Australia
| | - Graham Starkey
- Victorian Liver Transplant Unit, Austin Health, Melbourne, Victoria, Australia
| | - Sara Qi
- Vascular Surgery Unit, Austin Health, Melbourne, Victoria, Australia
| | | | - Louise Ellard
- Department of Critical Care, The University of Melbourne, Melbourne, Victoria, Australia
- Department of Anaesthesia, Austin Health, Melbourne, Victoria, Australia
| | - Peter McCall
- Department of Critical Care, The University of Melbourne, Melbourne, Victoria, Australia
- Department of Anaesthesia, Austin Health, Melbourne, Victoria, Australia
| | - Lachlan F Miles
- Department of Critical Care, The University of Melbourne, Melbourne, Victoria, Australia
- Department of Anaesthesia, Austin Health, Melbourne, Victoria, Australia
| | - Siven Seevanayagam
- Brian F. Buxton Cardiac and Thoracic Aortic Surgery Unit, Austin Health, Melbourne, Victoria, Australia
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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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17
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Rostami S, Fickert J, Morris C, Samuel M, Vu D, Ray CE, Kord A. Advanced Imaging Techniques Used in Direct Portosystemic Shunt Creation in Budd-Chiari Syndrome with Complex Venous Anatomy. Semin Intervent Radiol 2024; 41:97-102. [PMID: 38495262 PMCID: PMC10940039 DOI: 10.1055/s-0044-1779712] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/19/2024]
Affiliation(s)
- Sara Rostami
- Division of Interventional Radiology, Department of Radiology, University of Cincinnati, Cincinnati, Ohio
| | - Jaclyn Fickert
- Division of Interventional Radiology, Department of Radiology, University of Cincinnati, Cincinnati, Ohio
| | - Connor Morris
- Division of Interventional Radiology, Department of Radiology, University of Cincinnati, Cincinnati, Ohio
| | - Michael Samuel
- Division of Interventional Radiology, Department of Radiology, University of Cincinnati, Cincinnati, Ohio
| | - Doan Vu
- Division of Interventional Radiology, Department of Radiology, University of Cincinnati, Cincinnati, Ohio
| | - Charles E. Ray
- Division of Interventional Radiology, Department of Radiology, University of Illinois College of Medicine, Chicago, Illinois
| | - Ali Kord
- Division of Interventional Radiology, Department of Radiology, University of Cincinnati, Cincinnati, Ohio
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18
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Kimura K, Osaki A, Hirata Y, Egawa H, Kogiso T, Nakamura G, Hashidate H, Wakabayashi T, Sato M, Waguri N. A case of acute liver failure caused by Budd-Chiari syndrome salvaged by brain-dead donor liver transplantation. Clin J Gastroenterol 2024; 17:84-92. [PMID: 37773425 DOI: 10.1007/s12328-023-01863-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2023] [Accepted: 09/11/2023] [Indexed: 10/01/2023]
Abstract
A 24-year-old man was admitted to our hospital with abdominal distension. He was found to have acute liver failure and diagnosed with Budd-Chiari syndrome based on angiography and liver biopsy. Liver transplantation was deemed necessary when angiography showed extensive thrombotic occlusion of the hepatic veins and liver biopsy revealed submassive hepatic necrosis. The patient was found to have the JAK2V617F mutation, indicating a myeloproliferative neoplasm as the background disease. He developed hepatic encephalopathy but remained conscious on on-line hemodiafiltration. Brain-dead donor liver transplantation was performed on hospital day 30. Since then, the patient has remained well.
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Affiliation(s)
- Kiwamu Kimura
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan.
| | - Akihiko Osaki
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
| | - Yoshihiro Hirata
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Hiroto Egawa
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Tomomi Kogiso
- Department of Internal Medicine, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Gen Nakamura
- Department of Nephrology and Rheumatology, Niigata City General Hospital, Niigata, Japan
| | - Hideki Hashidate
- Department of Diagnostic Pathology, Niigata City General Hospital, Niigata, Japan
| | - Takuya Wakabayashi
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
| | - Munehiro Sato
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
| | - Nobuo Waguri
- Department of Gastroenterology and Hepatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata, 950-1197, Japan
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19
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20
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Valla DC. Hepatic vein thrombosis and PVT: A personal view on the contemporary development of ideas. Clin Liver Dis (Hoboken) 2024; 23:e0246. [PMID: 38988821 PMCID: PMC11236412 DOI: 10.1097/cld.0000000000000246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2024] [Accepted: 05/10/2024] [Indexed: 07/12/2024] Open
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21
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Pescia C, Lopez G, Cattaneo D, Bucelli C, Gianelli U, Iurlo A. The molecular landscape of myeloproliferative neoplasms associated with splanchnic vein thrombosis: Current perspective. Leuk Res 2024; 136:107420. [PMID: 38016412 DOI: 10.1016/j.leukres.2023.107420] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/26/2023] [Revised: 10/30/2023] [Accepted: 11/03/2023] [Indexed: 11/30/2023]
Abstract
BCR::ABL1-negative myeloproliferative neoplasms (MPNs) are classically represented by polycythemia vera, essential thrombocythemia, and primary myelofibrosis. BCR::ABL1-negative MPNs are significantly associated with morbidity and mortality related to an increased risk of thrombo-hemorrhagic events. They show a consistent association with splanchnic vein thrombosis (SVT), either represented by the portal, mesenteric or splenic vein thrombosis, or Budd-Chiari Syndrome. SVT is also a frequent presenting manifestation of MPN. MPNs associated with SVT show a predilection for younger women, high association with JAK2V617F mutation, low JAK2V617F variant allele frequency (generally <10 %), and low rates of CALR, MPL, or JAK2 exon 12 mutations. Next-Generation Sequencing techniques have contributed to deepening our knowledge of the molecular landscape of such cases, with potential diagnostic and prognostic implications. In this narrative review, we analyze the current perspective on the molecular background of MPN associated with SVT, pointing as well future directions in this field.
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Affiliation(s)
- Carlo Pescia
- Unit of Anatomic Pathology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
| | - Gianluca Lopez
- Unit of Anatomic Pathology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Biomedical, Surgical and Dental Sciences, University of Milan, Italy
| | - Daniele Cattaneo
- Hematology Division, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy
| | - Cristina Bucelli
- Hematology Division, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Umberto Gianelli
- Department of Health Sciences, University of Milan, Milan, Italy; Unit of Anatomic Pathology, ASST Santi Paolo e Carlo, Milan, Italy
| | - Alessandra Iurlo
- Hematology Division, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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22
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Mann JP, Ikram F, Modin L, Kelgeri C, Sharif K, Olliff S, McGuirk S, Gupte GL. Budd-Chiari Syndrome-A Single Center Experience From the United Kingdom. J Pediatr Gastroenterol Nutr 2023; 77:455-459. [PMID: 37314703 DOI: 10.1097/mpg.0000000000003860] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 06/15/2023]
Abstract
Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.
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Affiliation(s)
- J P Mann
- From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK
| | - F Ikram
- From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK
| | - L Modin
- From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK
| | - C Kelgeri
- From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK
| | - K Sharif
- From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK
| | - S Olliff
- Interventional Radiology Department, Queen Elizabeth Hospital Birmingham, Birmingham, UK
| | - S McGuirk
- the Radiology Department, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK
| | - G L Gupte
- From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK
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23
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Zeng Y, Zhang J, Jian W, Zhang Y, Yang Y, Li R, Fu Q. Rhabdoid meningioma with a history of Budd-Chiari syndrome: a case report and review of the literature. Front Oncol 2023; 13:1209244. [PMID: 37503325 PMCID: PMC10370419 DOI: 10.3389/fonc.2023.1209244] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2023] [Accepted: 06/21/2023] [Indexed: 07/29/2023] Open
Abstract
Background Rhabdoid meningioma and Budd-Chiari syndrome are both extremely rare, and there is no report describing the two diseases occurring in the same patient thus far. Herein, we showed an unusual case of rhabdoid meningioma with a history of Budd-Chiari syndrome. Case presentation The man was found to have abnormal liver function during physical examination in 2016 at 36 and was not paid attention to it. In 2019, he went to Beijing YouAn Hospital Affiliated to Capital Medical University for the decompensation of cirrhosis and was diagnosed with Budd-Chiari syndrome, subsequent angiography of the inferior vena cava combined with balloon dilatation were performed, the anticoagulation and hepatoprotective therapy were performed for a long time. When he turned 40 who had magnetic resonance imaging (MRI) that showed a left frontotemporal lobe space-occupying lesion, and postoperative pathological examination confirmed rhabdoid meningioma. He underwent surgery and postoperative adjuvant radiotherapy, but then he developed severe psychiatric symptoms and eventually succumbed to a lung infection two months after treatment. Conclusions Budd-Chiari syndrome and Rhabdoid meningiomas are both extremely rare diseases. To the best of our knowledge, there is no report that the two rare diseases occurred in the same patient, and this is the first case. However, whether there is any link between the two diseases is unclear, more researches are needed to confirm it in the future.
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Affiliation(s)
- Ying Zeng
- Department of Radiation Oncology, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Jing Zhang
- Department of Radiation Oncology, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Wei Jian
- Department of Radiation Oncology, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Yong Zhang
- Department of Radiation Oncology, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Ying Yang
- Department of Pathology, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Rongqing Li
- Department of Radiation Oncology, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Qiaofen Fu
- Department of Radiation Oncology, First Affiliated Hospital of Kunming Medical University, Kunming, China
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24
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Bova C, De Bartolo T, Pellegrini R, De Vuono A, Fiaschi E. Budd-Chiari syndrome mimicking autoimmune hepatitis. Radiol Case Rep 2023; 18:2311-2313. [PMID: 37153486 PMCID: PMC10159811 DOI: 10.1016/j.radcr.2023.03.053] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2023] [Revised: 03/22/2023] [Accepted: 03/29/2023] [Indexed: 05/09/2023] Open
Abstract
Budd-Chiari syndrome is a rare disorder characterized by obstruction of hepatic venous outflow involving either the hepatic veins or the inferior vena cava. Risk factors include thrombophilia and myeloproliferative disorders, and symptoms are often unspecific. We present a case of a 60-year-old woman admitted to our medical unit for ascites and abdominal pain. Although her medical history significant for a mixed connective tissue disease and a mild increase of the transaminases had raised the suspicion of autoimmune hepatitis, the demonstration on computed tomography of the absence of outflow in the terminal tract of the supra-hepatic veins allowed a correct diagnosis. The contribution of radiological imaging is fundamental in the diagnosis of this rare and elusive pathology.
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Affiliation(s)
- Carlo Bova
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
- Corresponding author.
| | | | - Roberta Pellegrini
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
| | - Andrea De Vuono
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
| | - Elio Fiaschi
- Unit of Internal Medicine, Azienda Ospedaliera, Via Migliori 1, 87100 Cosenza, Italy
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25
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Erden A, Kuru Öz D, Adıgüzel M, Özalp Ateş FS. Quantitative liver magnetic resonance imaging: correlation between conventional magnetic resonance imaging, laboratory values, and prognostic indices in Budd-Chiari syndrome. Diagn Interv Radiol 2023; 29:428-436. [PMID: 36960638 PMCID: PMC10679617 DOI: 10.4274/dir.2022.221462] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2021] [Accepted: 09/29/2022] [Indexed: 01/15/2023]
Abstract
PURPOSE In Budd-Chiari syndrome (BCS), unevenly distributed parenchymal changes and perfusion abnormalities occur due to hepatic venous outflow obstruction. This study aimed to evaluate the changes in the liver parenchyma in BCS using the quantitative magnetic resonance (MR) techniques of MR elastography, T1 and T2 mapping, and diffusion imaging and correlate the quantitative MR parameters through biochemical results and prognostic indices. METHODS Fourteen patients with BCS (seven men and seven women) were examined retrospectively. Liver stiffness (kPa), T1 relaxation times (ms) were achieved using the modified Look-Locker inversion recovery (MOLLI) 3(2)3(2)5 sequence and B1-corrected variable flip angle methods, T2 relaxation times (ms), and apparent diffusion coefficient (ADC) values (mm2/s) were measured using regions of interest placed in the same region in all quantitative methods. Measurements were repeated at the precontrast and postcontrast hepatobiliary phases. The reduction rate (RR; %) and adjusted postcontrast T1 (%) were calculated. The values obtained from different liver parenchyma areas (whole liver, caudate lobe, pathological T2 hyperintense tissue, and relatively preserved normal-appearing tissue) were compared using the Wilcoxon signed-rank test. Spearman's correlation coefficient was used to investigate the correlation between quantitative MR parameters and biochemical parameters/ prognostic scores (Child-Pugh score, Clichy score, and Rotterdam index). RESULTS The parenchymal stiffness and precontrast T1 values of the caudate lobe were significantly lower than those of the remainder of the parenchyma, whereas the adjusted postcontrast T1 percentages (MOLLI) were significantly higher (P ≤ 0.027). The parenchymal stiffness value, T1 and T2 values, percentages of RR (MOLLI), and adjusted postcontrast T1 values for the pathological tissue and relatively normal tissue were significantly different (P < 0.028). No significant difference was found in terms of ADC values between any of the distinct regions of the liver. A strong correlation was detected between the Child-Pugh score, Clichy score, and precontrast T1 values obtained through the MOLLI sequence (r = 0.867, P = 0.012, r = 0.821, P = 0.023, respectively). No correlation was found between the whole liver stiffness values and the laboratory parameters, fibrosis markers, prognostic indices, or MR parameters. A significant correlation was identified between creatinine levels and several T1 parameters and the T2 relaxation time (r ≥ 0.661, P ≤ 0.052). CONCLUSION Tissue stiffness and T1 relaxation values are high in the areas identified as fibrosis compared with those in the relatively preserved parenchyma. The T1 relaxation time can offer quantitative information for assessing segmental functional changes and prognosis in BCS.ion for assessing segmental functional changes and prognosis in BCS.
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Affiliation(s)
- Ayşe Erden
- Department of Radiology, Ankara University Faculty of Medicine, Ankara, Turkey
| | - Diğdem Kuru Öz
- Department of Radiology, Ankara University Faculty of Medicine, Ankara, Turkey
| | - Mehmet Adıgüzel
- Department of Radiology, Ankara University Faculty of Medicine, Ankara, Turkey
| | - Funda Seher Özalp Ateş
- Department of Biostatistics and Medical Informatics, Manisa Celal Bayar University, Faculty of Medicine, Manisa, Turkey
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26
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Michieletti E, Bodini FC, Morelli N, Rossi B, Bossalini M, Colombi D. Acute Budd-Chiari Syndrome with Complete Portal Vein Thrombosis Complicated by Hepato-Renal Syndrome Treated Successfully by Emergent TIPS with Rheolytic Thrombectomy. J Clin Exp Hepatol 2023; 13:549-551. [PMID: 37250886 PMCID: PMC10213869 DOI: 10.1016/j.jceh.2022.11.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2022] [Accepted: 11/12/2022] [Indexed: 11/21/2022] Open
Abstract
We described a case of a 73-year-old female admitted to the emergency department with acute hepatic and renal failure (hepato-renal syndrome, HRS) due to acute Budd-Chiari syndrome associated with complete portal vein thrombosis (BCS-PVT) for an unknown cause. Despite the initial therapy with anticoagulants, a sudden impairment of the renal function requiring hemodialysis was observed. The hepatic transplant was excluded for patient age and clinical conditions. Thus, the patient was successfully treated by emergent transjugular intrahepatic portosystemic shunt (TIPS) previous rheolytic thrombectomy of the PVT with AngioJet Ultra PE Thrombectomy System (Boston Scientific, Marlborough, MA, USA). After the procedure, the sudden resolution of the HRS was observed, and the patient is alive 13 months after hospital dismission with no TIPS dysfunction. In conclusion, emergent extended TIPS with the usage of rheolytic thrombectomy device in patient with acute BCS-PVT complicated by HRS is feasible by experienced operators and provide resolution of the HRS.
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Affiliation(s)
- Emanuele Michieletti
- Department of Radiological Functions, Azienda USL Piacenza, Via Taverna 49, Postal Code 29121, Piacenza, Italy
| | - Flavio C Bodini
- Department of Radiological Functions, Azienda USL Piacenza, Via Taverna 49, Postal Code 29121, Piacenza, Italy
- Department of Radiology, Fondazione Poliambulanza Istituto Ospedaliero, Via Leonida Bissolati, 57, Postal Code 25124, Brescia, Italy
| | - Nicola Morelli
- Department of Radiological Functions, Azienda USL Piacenza, Via Taverna 49, Postal Code 29121, Piacenza, Italy
| | - Beatrice Rossi
- Department of Radiological Functions, Azienda USL Piacenza, Via Taverna 49, Postal Code 29121, Piacenza, Italy
| | - Margherita Bossalini
- Department of Radiological Functions, Azienda USL Piacenza, Via Taverna 49, Postal Code 29121, Piacenza, Italy
| | - Davide Colombi
- Department of Radiological Functions, Azienda USL Piacenza, Via Taverna 49, Postal Code 29121, Piacenza, Italy
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27
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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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28
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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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Mukhiya G, Jiao D, Han X, Zhou X, Pokhrel G. Survival and clinical success of endovascular intervention in patients with Budd-Chiari syndrome: A systematic review. J Clin Imaging Sci 2023; 13:5. [PMID: 36751561 PMCID: PMC9899460 DOI: 10.25259/jcis_130_2022] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2022] [Accepted: 01/14/2023] [Indexed: 01/26/2023] Open
Abstract
Budd-Chiari syndrome is a complex clinical disorder of hepatic venous outflow obstruction, originating from the accessory hepatic vein (HV), large HV, and suprahepatic inferior vena cava (IVC). This disorder includes both HV and IVC obstructions and hepatopathy. This study aimed to conduct a systematic review of the survival rate and clinical success of different types of endovascular treatments for Budd-Chiari syndrome (BCS). All participant studies were retrieved from four databases and selected according to the eligibility criteria for systematic review of patients with BCS. The survival rate, clinical success of endovascular treatments in BCS, and survival rates at 1 and 5 years of publication year were calculated accordingly. A total of 3398 patients underwent an endovascular operation; among them, 93.6% showed clinical improvement after initial endovascular treatment. The median clinical success rates for recanalization, transjugular intrahepatic portosystemic shunt (TIPS), and combined procedures were 51%, 17.50%, and 52.50%, respectively. The median survival rates at 1 and 5 years were 51% and 51% for recanalization, 17.50% and 16% for TIPS, and 52.50% and 49.50% for combined treatment, respectively. Based on the year of publication, the median survival rates at 1 and 5 years were 23.50% and 22.50% before 2000, 41% and 41% in 2000‒2005, 35% and 35% in 2006‒2010, 51% and 48.50% in 2010‒2015, and 56% and 55.50% after 2015, respectively. Our findings indicate that the median survival rate at 1 and 5 years of recanalization treatment is higher than that of TIPS treatment, and recanalization provides better clinical improvement. The publication year findings strongly suggest progressive improvements in interventional endovascular therapy for BCS. Thus, interventional therapy restoring the physiologic hepatic venous outflow of the liver can be considered as the treatment of choice for patients with BCS which is a physiological modification procedure.
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Affiliation(s)
- Gauri Mukhiya
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Dechao Jiao
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xinwei Han
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.,Corresponding author: Xinwei Han, Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
| | - Xueliang Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Gaurab Pokhrel
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
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30
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Chia YC, Siti Asmaa MJ, Ramli M, Woon PY, Johan MF, Hassan R, Islam MA. Molecular Genetics of Thrombotic Myeloproliferative Neoplasms: Implications in Precision Oncology. Diagnostics (Basel) 2023; 13:163. [PMID: 36611455 PMCID: PMC9818412 DOI: 10.3390/diagnostics13010163] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2022] [Revised: 11/24/2022] [Accepted: 11/28/2022] [Indexed: 01/06/2023] Open
Abstract
Classical BCR-ABL-negative myeloproliferative neoplasms (MPN) include polycythaemia vera, essential thrombocythaemia, and primary myelofibrosis. Unlike monogenic disorders, a more complicated series of genetic mutations are believed to be responsible for MPN with various degrees of thromboembolic and bleeding complications. Thrombosis is one of the early manifestations in patients with MPN. To date, the driver genes responsible for MPN include JAK2, CALR, MPL, TET2, ASXL1, and MTHFR. Affords have been done to elucidate these mutations and the incidence of thromboembolic events. Several lines of evidence indicate that mutations in JAK2, MPL, TET2 and ASXL1 gene and polymorphisms in several clotting factors (GPIa, GPIIa, and GPIIIa) are associated with the occurrence and prevalence of thrombosis in MPN patients. Some polymorphisms within XRCC1, FBG, F2, F5, F7, F12, MMP9, HPA5, MTHFR, SDF-1, FAS, FASL, TERT, ACE, and TLR4 genes may also play a role in MPN manifestation. This review aims to provide an insightful overview on the genetic perspective of thrombotic complications in patients with MPN.
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Affiliation(s)
- Yuh Cai Chia
- Department Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia
| | - Mat Jusoh Siti Asmaa
- School of Health Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia
| | - Marini Ramli
- Department Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia
| | - Peng Yeong Woon
- Department of Molecular Biology and Human Genetics, Tzu Chi University, Hualien 97004, Taiwan
| | - Muhammad Farid Johan
- Department Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia
| | - Rosline Hassan
- Department Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia
| | - Md Asiful Islam
- Department Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
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Alshehhi MK, Nazir A. Microbial management of nonalcoholic fatty acid liver diseases. MICROBIOME THERAPEUTICS 2023:139-161. [DOI: 10.1016/b978-0-323-99336-4.00010-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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32
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Wang Z, Wang Z, Zhang Z, Li J, Pan Z, Liu A, Lu J, Guo J, Zu M, Xu H. Establishment and validation of a prediction model for the first recurrence of Budd-Chiari syndrome after endovascular treatment: a large sample size, single-center retrospective study. Hepatol Int 2022; 17:159-169. [PMID: 36567373 PMCID: PMC9895038 DOI: 10.1007/s12072-022-10464-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/18/2022] [Accepted: 11/24/2022] [Indexed: 12/27/2022]
Abstract
OBJECTIVE To investigate the independent risk factors for the first recurrence after endovascular management in patients with Budd-Chiari syndrome (BCS), and to establish a prediction model for predicting recurrence in target patients. METHODS BCS patients who underwent endovascular treatment in the Affiliated Hospital of Xuzhou Medical University from January 2010 to December 2015 were retrospectively examined, with their clinical, laboratory test, and imaging data collected and analyzed. Independent risk factors for recurrence were identified, and a prediction model was established and validated. RESULTS A total of 450 patients met the filtering criteria, and 102 recurred during the follow-up. The median follow-up time was 87 months, ranging from 1 to 137 months. The 1-, 3-, 5- and 10-year cumulative recurrence rate was 9.11% (6.41-11.73%), 17.35% (13.77-20.78%), 20.10% (16.30-23.72%), and 23.06% (18.86-27.04%), respectively. Liver cirrhosis, ascites, thrombosis, and all the main intrahepatic drainage veins obstructed (obstructed HV + AHV) are independent risk factors, while age is an independent protective factor. The prediction model was named MRBET. Based on the model, the risk score of each patient equals (-0.385981 * Age/10) + (0.0404184 * PT) + (0.0943423 * CRE/10) + (0.0157053 * LDH/10) + (0.592179 * LC) + (0.896034 * Ascites) + (0.691346 * Thrombosis) + (0.886741 * obstructed HV + AHV), and those in the high-risk group (risk score ≥ 1.57) were more likely to recur than those in the low-risk group (HR = 6.911, p < 0.001). The MRBET model is also available as a web tool at https://mrbet.shinyapps.io/dynnomapp . CONCLUSION Liver cirrhosis, ascites, thrombosis, and obstructed HV + AHV are independent risk factors for the first recurrence; age is an independent protective factor. The prediction model can effectively and conveniently predict the risk of recurrence and screen out patients at a high recurrence risk.
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Affiliation(s)
- Zhongkai Wang
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, 221006 Jiangsu China ,Center of Interventional Radiology and Vascular Surgery, Department of Radiology, Zhongda Hospital, Medical School, Southeast University, Nanjing, 210009 Jiangsu China
| | - Ziwei Wang
- Department of Urology, Changhai Hospital, Naval Medical University, 168 Changhai Road, Shanghai, 200433 China
| | - Zhiyuan Zhang
- Department of Interventional Oncology, The Second Affiliated Hospital of Xuzhou Medical University, 32 Meijian Road, Xuzhou, 221006 Jiangsu China
| | - Jiandong Li
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, 221006 Jiangsu China
| | - Zhiyang Pan
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, 221006 Jiangsu China
| | - Ang Liu
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, 221006 Jiangsu China ,Department of Structural Heart Disease, Cardiovascular Institute and Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Road, Beijing, 100037 China
| | - Jian Lu
- Center of Interventional Radiology and Vascular Surgery, Department of Radiology, Zhongda Hospital, Medical School, Southeast University, Nanjing, 210009 Jiangsu China
| | - Jinhe Guo
- Center of Interventional Radiology and Vascular Surgery, Department of Radiology, Zhongda Hospital, Medical School, Southeast University, Nanjing, 210009 Jiangsu China
| | - Maoheng Zu
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, 221006 Jiangsu China
| | - Hao Xu
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, 221006 Jiangsu China
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Allaoui A, Echchilali K, Fares M, Belabbes FZ, Jabbouri R, Naitlho A, Moudatir M, Alaoui FZ, Elkabli H. Budd-Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco. Medicine (Baltimore) 2022; 101:e31308. [PMID: 36343052 PMCID: PMC9646571 DOI: 10.1097/md.0000000000031308] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
Budd-Chiari syndrome (BCS) is considered a rare but serious complication of Behçet's disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the medical records of 1578 patients fulfilling the international diagnostic criteria for BD, including those with BSC. Eighteen male and 3 female patients, with a mean age of 36 ± 8.6 years. The inferior vena cava was involved in 81% (n = 17) of cases. All forms of BCS were noted: the chronic form in 52.4% (n = 11), the subacute form in 38% (n = 8), and the fulminant form (2 cases). Ascites was the main clinical sign and was present in 62% of patients (n = 13). Other venous thromboses (superior vena cava and lower limbs) were associated with BSC in 52.4% of patients (n = 11). Arterial involvement was noted in 28.6% (n = 6). Cardiac manifestations were present in 19% (n = 4) of the patients. All the patients received anticoagulants associated with corticosteroids. Immunosuppressants were used in 95% (n = 20). One patient received infliximab. Severe complications were noted in 38% (n = 8) of patients (digestive bleeding, confusion, infections and liver failure). Four patients have died during the study period. BCS in patients with BD is not uncommon and can be life threatening. It is frequently associated with other vascular manifestations that can be difficult to treat, particularly in the presence of pulmonary artery aneurysms. Prognosis improved with the use of immunosuppressants. Biologics can be promising in the early stages.
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Affiliation(s)
- Abire Allaoui
- Department of Internal Medicine, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, Morocco
- Laboratory of Clinical Immunology, Inflammation and Allergy, Faculty of Medicine and Pharmacy of Casablanca, Hassan II University of Casablanca, Casablanca, Morocco
- * Correspondence: Abire Allaoui, Department of Internal Medicine, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, 30, Rue Buzancy Bd Emile Zola, Bélvédère, Casablanca, Morocco (e-mail: )
| | - Khadija Echchilali
- Department of Internal Medicine, Ibn Rochd UHC, Hassan II University of Casablanca, Casablanca, Morocco
| | - Manal Fares
- Department of Internal Medicine, Ibn Rochd UHC, Hassan II University of Casablanca, Casablanca, Morocco
| | - Fatim-Zahra Belabbes
- Department of Internal Medicine, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, Morocco
| | - Rajaa Jabbouri
- Department of Internal Medicine, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, Morocco
| | - Abdelhamid Naitlho
- Department of Internal Medicine, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, Morocco
| | - Mina Moudatir
- Department of Internal Medicine, Ibn Rochd UHC, Hassan II University of Casablanca, Casablanca, Morocco
| | - Fatim Zohra Alaoui
- Department of Internal Medicine, Ibn Rochd UHC, Hassan II University of Casablanca, Casablanca, Morocco
- Faculty of Medicine and Pharmacy of Laayoune, Ibn Zohr University, Laayoune, Morocco
| | - Hassan Elkabli
- Department of Internal Medicine, Ibn Rochd UHC, Hassan II University of Casablanca, Casablanca, Morocco
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Elhence A, Shalimar. Treatment Options for Hepatic Venous Outflow Tract Obstruction: Is the Scale Tipping in Favor of TIPS? J Clin Exp Hepatol 2022; 12:1403-1405. [PMID: 36340299 PMCID: PMC9630011 DOI: 10.1016/j.jceh.2022.09.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2022] [Accepted: 09/26/2022] [Indexed: 12/12/2022] Open
Affiliation(s)
- Anshuman Elhence
- Department of Gastroenterology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India
| | - Shalimar
- Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, Delhi, India
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Giri S, Kale A, Shukla A. Efficacy and Safety of Transjugular Intrahepatic Portosystemic Shunt Creation for Budd-Chiari Syndrome: A Systematic Review and Meta-Analysis. J Vasc Interv Radiol 2022; 33:1301-1312.e13. [PMID: 35940361 DOI: 10.1016/j.jvir.2022.07.022] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2022] [Revised: 07/04/2022] [Accepted: 07/22/2022] [Indexed: 12/15/2022] Open
Abstract
PURPOSE To assess the critical role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of Budd-Chiari syndrome (BCS), as the data with respect to the safety and outcome of TIPS in patients with BCS are scarce because of the rarity of the disease. MATERIALS AND METHODS A comprehensive search of literature of various databases from 2000 to October 2021 was conducted for studies evaluating the outcome of TIPS in patients with BCS. The primary outcomes of the analysis were technical and clinical success, adverse events and mortality associated with TIPS, dysfunction of TIPS, need for TIPS revision, need for liver transplantation (LT), and 1-year survival. RESULTS A total of 33 studies (1,395 patients) were included in this meta-analysis. The pooled rates and 95% confidence intervals of various outcomes were 98.6% (97.6-99.7) for technical success, 90.3% (86.0-94.6) for clinical success, 10.0% (6.5-13.6) for major adverse events, 0.5% (0.2-1.0) for TIPS-related mortality, 11.6% (7.8-15.4) for post-TIPS hepatic encephalopathy (HE), 40.1% (32.5-47.7) for TIPS dysfunction, 8.6% (4.9-12.4) for the need for TIPS revision, 4.5% (2.8-6.2) for the need for LT, and 94.6% (93.1-96.1) for 1-year survival. Publication bias was seen with all outcomes except for post-TIPS HE, TIPS dysfunction, and the need for LT. CONCLUSIONS The existing literature supports the feasibility, safety, and efficacy of TIPS in the treatment of BCS. Deciding the optimal timing of TIPS in BCS needs further studies.
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Affiliation(s)
- Suprabhat Giri
- Department of Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
| | - Aditya Kale
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
| | - Akash Shukla
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
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Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis. Sci Rep 2022; 12:16166. [PMID: 36171454 PMCID: PMC9519873 DOI: 10.1038/s41598-022-20399-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2021] [Accepted: 09/13/2022] [Indexed: 11/19/2022] Open
Abstract
This study was performed to evaluate the outcome of endovascular intervention therapy for Budd-Chiari syndrome (BCS) and compare recanalization, transjugular intrahepatic portosystemic shunt (TIPS)/direct intrahepatic portosystemic shunt (DIPS), and combined procedure treatment. For the meta-analysis, 71 studies were identified by searching four databases. The individual studies’ samples were used to calculate a confidence interval (CI 95%), and data were pooled using a fixed-effect model and random effect model. The pooled measure and an equal-weighted average rate were calculated in all participant studies. Heterogeneity between the studies was assessed with I2, and T2 tests, and publication bias was estimated using Egger’s regression test. A total of 4,407 BCS patients had undergone an endovascular intervention procedure. The pooled results were 98.9% (95% CI 97.8‒98.9%) for a technical success operation, and 96.9% (95% CI 94.9‒98.9%) for a clinical success operation. The re-intervention rate after the initial intervention procedure was 18.9% (95% CI 14.7‒22.9%), and the survival rates at 1 and 5 years after the initial intervention procedure were 98.9% (95% CI 96.8‒98.9%) and 94.9% (95% CI 92.9‒96.9%), respectively. Patients receiving recanalization treatment (98%) had a better prognosis than those with a combined procedure (95.6%) and TIPS/DIPS treatment (94.5%). The systematic review and meta-analysis further solidify the role of endovascular intervention treatment in BCS as safe and effective. It maintains high technical and clinical success and long-term survival rates. The recanalization treatment had a better prognosis and outcome than the combined procedures and TIPS/DIPS treatment.
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Zhou ZW, Wang SH, Xu CA, Wu WH, Hui TC, Yin QQ, Zheng W, Pan HY. Three-years misdiagnosis of Niemann Pick disease type B with novel mutations in SMPD1 gene as Budd-Chiari syndrome. BMC Med Genomics 2022; 15:196. [PMID: 36114502 PMCID: PMC9482227 DOI: 10.1186/s12920-022-01353-2] [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: 06/17/2021] [Accepted: 09/09/2022] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
The chronic visceral subtype of acid sphingomyelinase deficiency, commonly known as Niemann Pick disease type B (NPDB), is a relatively rare autosomal recessive genetic disorder that is caused by mutations in the SMPD1 gene. NPDB with sea-blue histiocytes (SBH) clinically mimics Budd-Chiari syndrome (BCS), as it lacks specific clinical characteristics. This makes its diagnosis difficult.
Case presentation
Here, we report a case of NPDB with SBH that was misdiagnosed as BCS for three years. A 20-year-old female with abdominal distension, hepatosplenomegaly, and haematological anomalies was initially diagnosed with BCS based on her imaging finding of a thin hepatic vein and rapid blood flow at the confluence of the hepatic vein and inferior vena cava. Her bone marrow cytology found sea-blue histiocytes. Liver biopsy showed foamy cytoplasm in hepatocytes surrounded by numerous Kupffer cells. Sequencing analysis of the SMPD1 gene led to the finding of two missense mutations in the heterozygous state: C.829 T > C (p.Trp277Arg) in exon 2 (novel) and c.1805G > A (p.Arg602His) in exon 6 (already described). These findings established the diagnosis of NPDB.
Conclusion
The patient presented with hepatosplenomegaly, haematological anomalies, and dyslipidaemia. Thus, NPDB should be considered following the exclusion of related diseases. The diagnosis of NPDB was suspected by clinical symptoms and routine laboratory tests and was confirmed by liver biopsy and gene sequencing. The novel mutation c.829 T > C in exon 2 of the SMPD1 gene has never been reported and needs to be further investigated.
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Wiest I, Teufel A, Ebert MP, Potthoff A, Christen M, Penkala N, Dietrich CF. [Budd-Chiari syndrome, review and illustration]. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2022; 60:1335-1345. [PMID: 34820810 DOI: 10.1055/a-1645-2760] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Budd-Chiari syndrome is a rare vascular disorder characterized by obstruction of the hepatic venous outflow. Various diseases causing coagulopathy play a role in aetiology, such as myeloproliferative disorders. Acute vascular occlusion may lead to acute phlebitis with fever. The classic triad of acute liver failure may be present with ascites, hepatomegaly, and abdominal pain. However, subacute courses of disease were also observed. Because of the variable symptoms and severity extent, depending on the acuity of the course and the extent of the affected vessels, diagnosis is often difficult. Sonography, as a ubiquitously available and cost-effective diagnostic tool, plays a leading role. Doppler ultrasonography can be used to visualize hemodynamics in particular. In acute thrombotic occlusion, the affected hepatic veins usually cannot or only partially be visualized. In non-occluding thrombi, turbulent flow patterns may develop in the area of venous outflow obstruction, and flow velocity is then increased in the area of stenosis. Contrast enhanced ultrasound offers even better specificity of diagnosis. Computed tomography and magnetic resonance imaging can directly visualize thrombi and the cause of obstruction. Once the diagnosis is confirmed, anticoagulation must be initiated, but therapy of the underlying disease must also be started. If symptom-controlling measures are not sufficient, angioplasty/stenting to reopen short-segment stenoses or implantation of a TIPSS device may be considered. Liver transplantation remains ultima ratio. As studies on the precision of diagnostic methods are controversial, the characteristics of imaging for BCS are therefore summarized in this review on the basis of several illustrating case reports.
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Affiliation(s)
- Isabella Wiest
- II. Medizinische Klinik, Sektion Hepatologie, Medizinische Fakultät Mannheim, Universität Heidelberg, Mannheim, Germany
| | - Andreas Teufel
- II. Medizinische Klinik, Sektion Hepatologie, Medizinische Fakultät Mannheim, Universität Heidelberg, Mannheim, Germany
- Klinische Kooperationseinheit Healthy Metabolism, Zentrum für Präventivmedizin und Digitale Gesundheit Baden-Württemberg, Universität Heidelberg, Mannheim, Germany
| | - Matthias Philip Ebert
- Klinische Kooperationseinheit Healthy Metabolism, Zentrum für Präventivmedizin und Digitale Gesundheit Baden-Württemberg, Universität Heidelberg, Mannheim, Germany
- II. Medizinische Klinik, Universitätsklinikum Mannheim, Medizinische Fakultät Mannheim, Universität Heidelberg, Mannheim
| | - Andrej Potthoff
- Gastroenterology, Hepatology and Endocrinology, Medizinische Hochschule, Hannover, Germany
| | - Michael Christen
- Allgemeine Innere Medizin (DAIM), Kliniken Beau Site, Salem und Permanence, Bern, Switzerland
| | - Nadine Penkala
- Allgemeine Innere Medizin (DAIM), Kliniken Beau Site, Salem und Permanence, Bern, Switzerland
| | - Christoph F Dietrich
- Allgemeine Innere Medizin (DAIM), Kliniken Beau Site, Salem und Permanence, Bern, Switzerland
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Keshav N, Ohliger MA. Imaging Vascular Disorders of the Liver. Radiol Clin North Am 2022; 60:857-871. [PMID: 35989049 DOI: 10.1016/j.rcl.2022.05.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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Mukund A, Rana S, Mohan C, Kalra N, Baijal SS. Indian College of Radiology and Imaging Evidence-Based Guidelines for Interventions in Portal Hypertension and Its Complications. Indian J Radiol Imaging 2022; 31:917-932. [PMID: 35136505 PMCID: PMC8817816 DOI: 10.1055/s-0041-1740235] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/10/2022] Open
Abstract
Portal hypertension is a complication of chronic liver disease. Various radiological interventions are being done to aid in the diagnosis of portal hypertension; further, an interventional radiologist can offer various treatments for the complications of portal hypertension. Diagnosis of portal hypertension in its early stage may require hepatic venous pressure gradient measurement. Measurement of gradient also guides in diagnosing the type of portal hypertension, measuring response to treatment and prognostication. This article attempts to provide evidence-based guidelines on the management of portal hypertension and treatment of its complications.
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Affiliation(s)
- Amar Mukund
- Department of Interventional Radiology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Shaleen Rana
- Department of Interventional Radiology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Chander Mohan
- Department of Interventional Radiology, BLK Superspecialty Hospital, New Delhi, India
| | - Naveen Kalra
- Department of Radiology, PGIMER, Chandigarh, India
| | - Sanjay Saran Baijal
- Department of Diagnostic and Interventional Radiology, Medanta—The Medicity, Gurugram, Haryana, India
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41
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De Bruyne R, De Bruyne P. Vascular Disorders of the Liver. TEXTBOOK OF PEDIATRIC GASTROENTEROLOGY, HEPATOLOGY AND NUTRITION 2022:931-951. [DOI: 10.1007/978-3-030-80068-0_70] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Abou-Ismail MY, Prchal JT, Deininger MW, Parker CJ, Lim MY. Anticoagulation management post-transjugular intrahepatic portosystemic shunt in portal hypertension associated with myeloproliferative neoplasms. Blood Coagul Fibrinolysis 2021; 32:578-583. [PMID: 34608880 DOI: 10.1097/mbc.0000000000001087] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Portal hypertension (pHTN) complicates myeloproliferative neoplasms (MPNs), and usually occurs due to Budd-Chiari syndrome or splanchnic vein thrombosis. Current management modalities for MPN-associated pHTN include anticoagulation, transjugular intrahepatic portosystemic shunt (TIPS), and orthotopic liver transplant. Data on the thrombotic and bleeding outcomes of this practice is of poor quality, and whether direct oral anticoagulants (DOACs) are effective in this setting is unknown. We describe failure of DOACs to prevent post-TIPS complications in two case reports of patients with MPN-associated pHTN and review the associated literature. We conducted a comprehensive search in Embase (embase.com), Scopus (scopus.org), and PubMed for existing data on MPN-associated pHTN post-TIPS procedure. Four studies (n = 251) of patients with pHTN post-TIPS were eligible (MPN, n = 143). A review of the literature suggests that patients with MPN-associated pHTN may be at higher risk for post-TIPS complications including stent thrombosis and stenosis, compared with other causes of thrombotic pHTN. DOAC use has not been studied in this setting. While further studies to guide optimal management of MPN-associated pHTN post-TIPS are needed, available evidence suggests that life-long anticoagulation is warranted. DOACs should not be considered standard of care because of lack of evidence of efficacy.
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Affiliation(s)
- Mouhamed Yazan Abou-Ismail
- Division of Hematology and Hematologic Malignancies, Department of Internal Medicine, The University of Utah, Salt Lake City, Utah, USA
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Hoffmann T, Voigtländer H, Fröhlich E, Debove I, Pauluschke-Fröhlich J. Single-center study: evaluation of sonography in Budd-Chiari syndrome. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2021; 60:1111-1117. [PMID: 34781388 DOI: 10.1055/a-1550-3141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
PURPOSE Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction. The study aimed to evaluate the diagnostic utility of ultrasound in confirming the diagnosis of BCS and to provide an overview of the clinical picture. MATERIALS AND METHOD In this retrospective single-center study, patients with an initial diagnosis of BCS were included. The files were analyzed concerning the ultrasound images and compared to computed tomography (CT) and magnetic resonance imaging (MRI). Main clinical signs of BCS were collected. RESULTS Data of 25 patients were analyzed. Doppler sonography showed the highest sensitivity (78.9%) with the highest specificity 97.4 (%) in confirming the correct diagnosis of BCS. Main imaging signs were obstruction in the hepatic veins (68.0%, 17/25 thrombotic), collaterals (91.7%, 11/12 intrahepatic), inhomogeneous liver parenchyma (7/21), and a hypertrophied lobus caudatus (18/21) (p < 0.01). All imaging signs could be detected with sonography. Hypertrophied lobus caudatus was seen exclusively in BCS. Furthermore, portal hypertension (9/25), liver cirrhosis (9/25), and ascites (19/25) can be diagnosed as non-specific signs of BCS (p < 0.01).The main clinical findings were elevated γ-GT levels in the laboratory (92.0%, 23/25, p < 0.01) and esophageal varices in endoscopy (12/25 p < 0.01). An association with myeloproliferative neoplasia (MPN) was frequently seen (10/25) (p < 0.01). CONCLUSION The present study demonstrates that sonography is an appropriate tool for the diagnosis of BCS and should be used as the first imaging procedure.
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Affiliation(s)
- Tatjana Hoffmann
- Department of Internal Medicine 1 (Gastroenterology, Hepatology, Infectious Diseases), University Hospital Tübingen, Germany, Germany
| | - Hendrik Voigtländer
- Department of Internal Medicine 1 (Gastroenterology, Hepatology, Infectious Diseases), University Hospital Tübingen, Germany, Germany
| | - Eckhart Fröhlich
- Department of Internal Medicine 1 (Gastroenterology, Hepatology, Infectious Diseases), University Hospital Tübingen, Germany, Germany
| | - Ines Debove
- Department of Neurology, Inselspital Bern University Hospital, University of Bern, Switzerland
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Aktas H, Ozer A, Yilmaz TU, Keceoglu S, Can MG, Emiroglu R. Liver transplantation for Budd-Chiari syndrome: A challenging but handable procedure. Asian J Surg 2021; 45:1396-1402. [PMID: 34688524 DOI: 10.1016/j.asjsur.2021.09.007] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2021] [Revised: 08/26/2021] [Accepted: 09/26/2021] [Indexed: 11/25/2022] Open
Abstract
BACKROUND Establishing venous outflow in liver transplantation for patients with Budd-Chiari syndrome is crucial and requires various surgical techniques. The outcomes of these patients exibits distinct problems including vasculary thrombosis and biliary complications. METHODS In this single center study, the outcomes and surgical features of 33 patients with Budd-Chiari syndrome who were carried out liver translantation (27 patients from living donor). Another group was formed among patients underwent liver transplantation due to other etiologies and the outcomes were compared. RESULTS The most-seen type was the classical type of Budd-Chiari syndrome (25, 75.8%). For twenty-six patients inherited or acquired prothrombotic disorders were identified (78.2%) in Budd-Chiari group. Average follow-up was 29.7 ± 15.5 months. We have observed no recurrence of disease in our BCS patients. When the two groups was compared in terms of thrombotic complications, there was a significantly increased risk in BCS group (p = 0.014). Our 1 and 3-year survival rates for the BCS group were 81.8% and 78.8%, respectively. In the control group, 1 and 3-year survival rates were 93.3% and 88.9%, respectively. Log-rank test analyses showed no statistically significant results. CONCLUSION Liver transplantation with individual surgical and postoperative treatment strategy for patients with Budd-Chiari syndrome provides comparable outcomes.
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Affiliation(s)
- Hikmet Aktas
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Ali Ozer
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey.
| | - Tonguc Utku Yilmaz
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Selim Keceoglu
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Meltem Guner Can
- Department of Anaesthesiology and Reanimation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Remzi Emiroglu
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
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45
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Gamanagatti S, Hemachandran N. Hepatic Venous Outflow Tract Obstruction: Causes, Natural History, and Treatment. Clin Liver Dis (Hoboken) 2021; 18:117-121. [PMID: 34691397 PMCID: PMC8518338 DOI: 10.1002/cld.1126] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2021] [Revised: 03/29/2021] [Accepted: 04/17/2021] [Indexed: 02/04/2023] Open
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Ninalowo H, Oluyemi A, Olowoyeye O, Ajayi A. A Case Series of Budd-Chiari Syndrome in Nigerian Patients: Diagnosis, Associations, Management, and Outcomes. JOURNAL OF GASTROINTESTINAL AND ABDOMINAL RADIOLOGY 2021. [DOI: 10.1055/s-0041-1731975] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/20/2022] Open
Abstract
AbstractBudd-Chiari syndrome (BCS) is defined as hepatic outflow obstruction regardless of the cause or level of obstruction—from small hepatic veins to the opening of the inferior vena cava in the right atrium. BCS could be primary or secondary. Very few cases of this syndrome have been reported in Nigeria and there is no local clinical series documenting the noninvasive and invasive imaging findings and importance of interventional radiology techniques in its diagnosis and management.We report three cases of patients diagnosed with BCS in Lagos, Nigeria. We hope this work will raise awareness about the condition and its associations and show that much can be done to establish diagnosis and care with minimally invasive techniques in our resource-limited environment.
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Affiliation(s)
- Hammed Ninalowo
- Interventional Radiology, IRDOC Interventional Radiology Consulting Limited, Euracare Multispecialty Hospital, Victoria Island, Lagos, Nigeria
| | - Aderemi Oluyemi
- Department of Gastroenterology, Endoscopy and Hepatology, ReMay Consultancy and Medical Services, Ikeja, Lagos State, Nigeria
| | - Omodele Olowoyeye
- Department of Radiodiagnosis, Lagos University Teaching Hospital, Idi-Araba, Lagos, Nigeria
| | - Abisoye Ajayi
- Department of Radiodiagnosis, Lagos University Teaching Hospital, Idi-Araba, Lagos, Nigeria
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Prince D, Popuri R, Majumdar A. An essential accessory. BMJ Open Gastroenterol 2021; 8:bmjgast-2021-000770. [PMID: 34588177 PMCID: PMC8483049 DOI: 10.1136/bmjgast-2021-000770] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2021] [Accepted: 09/07/2021] [Indexed: 11/03/2022] Open
Abstract
A young adult male was referred for a second opinion of deranged liver biochemistry. He initially presented two years prior with abdominal pain, lethargy and fevers due to a segment two pyogenic liver abscess. He received empirical antibiotic therapy to resolution. Computed tomography for abscess follow-up revealed an intrahepatic inferior vena cava thrombus. He was anti-coagulated with warfarin. He was lupus anticoagulant positive and had a highly positive beta-2 glycoprotein antibody on serial measurement and was diagnosed with anti-phospholipid syndrome. On current review, the patient had no clinical stigmata of chronic liver disease. There were dilated veins on the supraumbilical abdominal and chest walls. There was mild hepatomegaly but no splenomegaly. Laboratory investigations revealed mildly cholestatic liver function tests with hyperbilirubinaemia (40μmol/L) but no liver synthetic dysfunction. Serological screening did not reveal any cause of chronic liver disease. The patient underwent multiphase abdominal CT and formal hepatic venography. What is the diagnosis and describe the hepatic venous outflow?
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Affiliation(s)
- David Prince
- AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
| | - Radha Popuri
- Department of Radiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
| | - Avik Majumdar
- AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia .,Central Clinical School, The University of Sydney, Sydney, New South Wales, Australia
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48
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Utility of Hepatic 2D Shear-Wave Elastography in Monitoring Response to Image-Guided Intervention in Children With Chronic Budd-Chiari Syndrome: A Prospective Study. AJR Am J Roentgenol 2021; 218:534-543. [PMID: 34585610 DOI: 10.2214/ajr.21.26547] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Background: Image-guided interventions for Budd-Chiari syndrome (BCS) reduce hepatic congestion, thereby reducing liver stiffness. Liver stiffness measurements (LSM) by ultrasound may provide a noninvasive marker for assessing treatment response. Objective: To assess the utility of 2D shear-wave elastography (SWE) for monitoring response to image-guided intervention in children with BCS, with attention to changes in LSM in patients with disease recurrence. Materials and Methods: This prospective study included children with chronic BCS and planned image-guided intervention. Color Doppler ultrasound (CDUS) and 2D SWE were performed at baseline; at 24 hours, one month, and three months after intervention; and thereafter every three months or at the time of clinically suspected recurrence. Eighteen children underwent liver biopsy at the time of intervention for fibrosis staging using METAVIR criteria. Disease recurrence was diagnosed by CDUS. Statistical evaluation was performed using non-parametric tests. Results: A total of 32 children (28 boys, 4 girls; mean age: 9.0 years; range: 3-14 years) were included. Median LSM was at baseline 43.7 kPa [interquartile range (IQR): 33.0-65.4), at 24 hours 22.5 kPa (IQR: 16.8-32.0), at one month 18.7 kPa (IQR: 14.2-32.0), and at three months 16.7 kPa (IQR: 11.5-22.5) (p<.05 for all post-intervention time points vs baseline). Nine (28.1%) patients developed recurrence after intervention at mean of 4.4 months. In one patient with recurrence, LSM was higher at 24 hours (52.3 kPa) than at baseline (44.2 kPa). In the remaining eight patients with recurrence, LSM increased at the time of recurrence compared with the prior post-intervention LSM measurement [median absolute increase of 11.0 kPa (IQR: 6.1-24.4 kPa)]. Fibrosis stage was not significantly correlated with baseline LSM (r=0.11, 95% CI: -0.37, 0.54; p=.51) or 24-hour post-intervention LSM (r=0.39, 95% CI: -0.11, 0.73; p=.11). Conclusion: LSM decreased significantly after image-guided intervention for chronic BCS in children, showing a maximal decrease at 24 hours post-intervention. Disease recurrence was typically associated with an increase in LSM compared with the patient's prior measurement. Clinical Impact: LSM obtained by 2D SWE may serve as a useful quantitative adjunct to CDUS in monitoring children with chronic BCS for disease recurrence after percutaneous interventional treatment.
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49
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Cravo M, Oliveira DG, Guimas A, Vita P, Rego AL, Alves R, Valadares D, Carvalheiras G, Ricardo M, Pinto A, Pessegueiro H, Nery F. The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari Syndrome: Case Series. J Med Cases 2021; 12:190-194. [PMID: 34434456 PMCID: PMC8383533 DOI: 10.14740/jmc3659] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2021] [Accepted: 02/01/2021] [Indexed: 11/11/2022] Open
Abstract
Budd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them presenting with acute liver injury. Progression to ALF was seen in three patients, two of whom died, with one being successfully submitted to liver transplantation. IntCUs allow a 24-h patient surveillance and a prompt management of BCS, with less economic impact when compared to ICUs. Mortality was related to the presence of associated comorbidities that limited therapeutic approach.
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Affiliation(s)
- Marcia Cravo
- Servico de Medicina Interna, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Daniel G Oliveira
- Servico de Medicina Interna, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Arlindo Guimas
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Pedro Vita
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Ana Luisa Rego
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Rute Alves
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Diana Valadares
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Graziela Carvalheiras
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Miguel Ricardo
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Alexandre Pinto
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal.,Instituto de Ciencias Biomedicas Abel Salazar-Universidade do Porto, Porto, Portugal
| | - Helena Pessegueiro
- Unidade de Transplante Hepato-Pancreatica, Centro Hospitalar Universitario do Porto, Porto, Portugal
| | - Filipe Nery
- Unidade Intermedia Medica, Servico de Cuidados Intensivos, Centro Hospitalar Universitario do Porto, Porto, Portugal.,Instituto de Ciencias Biomedicas Abel Salazar-Universidade do Porto, Porto, Portugal.,EpiUnit, Instituto de Saude Publica da Universidade do Porto, Porto, Portugal
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50
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Akyol L, Toz B, Bayındır Ö, Zengin O, Cansu D, Yiğit M, Çetin GY, Omma A, Erden A, Küçükşahin O, Altuner MŞ, Çorba B, Ünal AU, Küçük H, Küçük A, Balkarli A, Gönüllü E, Tufan AN, Bakırcı S, Öner SY, Balcı MA, Kobak Ş, Yazıcı A, Özgen M, Şahin A, Koca SS, Erer B, Gül A, Aksu K, Keser G, Onat AM, Kısacık B, Kaşifoğlu T, Çefle A, Kalyoncu U, Sayarlıoğlu M. Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study. Clin Rheumatol 2021; 41:177-186. [PMID: 34368908 DOI: 10.1007/s10067-021-05878-2] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2021] [Revised: 07/17/2021] [Accepted: 08/01/2021] [Indexed: 02/07/2023]
Abstract
OBJECTIVE To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS). METHODS This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. RESULTS Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). CONCLUSION To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.
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Affiliation(s)
- Lütfi Akyol
- Department of Internal Medicine, Division of Rheumatology, Health Sciences University, Gazi Yaşargil Training and Research Hospital, Diyarbakır, Turkey.
| | - Bahtiyar Toz
- Department of Medicine, Icahn School of Medicine At Mount Sinai, Queens Hospital Center, New York, NY, USA
| | - Özün Bayındır
- Department of Internal Medicine, Division of Rheumatology, Katip Çelebi University School of Medicine, İzmir, Turkey
| | - Orhan Zengin
- Department of Internal Medicine, Division of Rheumatology, Dr Ersin Arslan Training and Research Hospital, Gaziantep, Turkey
| | - DöndüÜsküdar Cansu
- Department of Internal Medicine, Division of Rheumatology, Osmangazi University School of Medicine, Eskişehir, Turkey
| | - Murat Yiğit
- Department of Internal Medicine, Division of Rheumatology, Pamukkale University School of Medicine, Denizli, Turkey
| | - Gözde Yıldırım Çetin
- Department of Internal Medicine, Division of Rheumatology, Sütçü İmam University School of Medicine, Kahramanmaraş, Turkey
| | - Ahmet Omma
- Department of Internal Medicine, Division of Rheumatology, Ankara City Hospital, Ankara, Turkey
| | - Abdulsamet Erden
- Department of Internal Medicine, Division of Rheumatology, Ankara City Hospital, Ankara, Turkey
| | - Orhan Küçükşahin
- Department of Internal Medicine, Division of Rheumatology, Ankara City Hospital, Ankara, Turkey
| | - Mehmet Şakir Altuner
- Department of Internal Medicine, Division of Rheumatology, Malatya Training and Research Hospital, Malatya, Turkey
| | - BurçinŞeyda Çorba
- Department of Statistics, Ondokuz Mayıs University School of Medicine, Samsun, Turkey
| | - Ali Uğur Ünal
- Department of Internal Medicine, Division of Rheumatology, Medıcal Park Hospital, Ordu, Turkey
| | - Hamit Küçük
- Department of Internal Medicine, Division of Rheumatology, Gazi University School of Medicine, Ankara, Turkey
| | - Adem Küçük
- Department of Internal Medicine, Division of Rheumatology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Ayşe Balkarli
- Department of Internal Medicine, Division of Rheumatology, Antalya Training and Research Hospital, Antalya, Turkey
| | - Emel Gönüllü
- Department of Internal Medicine, Division of Rheumatology, Sakarya University School of Medicine, Sakarya, Turkey
| | - Ayşe Nur Tufan
- Department of Internal Medicine, Division of Rheumatology, Health Sciences University, İstanbul Haseki Training and Research Hospital, İstanbul, Turkey
| | - Sibel Bakırcı
- Department of Internal Medicine, Division of Rheumatology, Antalya Training and Research Hospital, Antalya, Turkey
| | - Sibel Yılmaz Öner
- Department of Internal Medicine, Division of Rheumatology, Kartal Dr. Lütfi Kırdar Training and Research Hospital, İstanbul, Turkey
| | - Mehmet Ali Balcı
- Department of Internal Medicine, Division of Rheumatology, Health Sciences University, Gazi Yaşargil Training and Research Hospital, Diyarbakır, Turkey
| | - Şenol Kobak
- Department of Internal Medicine, Division of Rheumatology, Liv Hospital, Istanbul, Turkey
| | - Ayten Yazıcı
- Department,of Internal Medicine, Division of Rheumatology, Kocaeli University School of Medicine, Kocaeli, Turkey
| | - Metin Özgen
- Department of Internal Medicine, Division of Rheumatology, Ondokuz Mayıs University School of Medicine, Samsun, Turkey
| | - Ali Şahin
- Department of Internal Medicine, Division of Rheumatology, Cumhuriyet University School of Medicine, Sivas, Turkey
| | - Süleyman Serdar Koca
- Department of Internal Medicine, Division of Rheumatology, Fırat University School of Medicine, Elazığ, Turkey
| | - Burak Erer
- Department of Internal Medicine, Division of Rheumatology, Memorial Şişli Hospital, İstanbul, Turkey
| | - Ahmet Gül
- Department of Internal Medicine, Division of Rheumatology, İstanbul University School of Medicine, İstanbul, Turkey
| | - Kenan Aksu
- Department of Internal Medicine, Division of Rheumatology, Ege University School of Medicine, İzmir, Turkey
| | - Gökhan Keser
- Department of Internal Medicine, Division of Rheumatology, Ege University School of Medicine, İzmir, Turkey
| | - Ahmet Mesut Onat
- Department of Internal Medicine, Division of Rheumatology, Medıcal Park Hospital, Gaziantep, Turkey
| | - Bünyamin Kısacık
- Department of Internal Medicine, Division of Rheumatology, Medıcal Park Hospital, Gaziantep, Turkey
| | - Timuçin Kaşifoğlu
- Department of Internal Medicine, Division of Rheumatology, Osmangazi University School of Medicine, Eskişehir, Turkey
| | - Ayşe Çefle
- Department,of Internal Medicine, Division of Rheumatology, Kocaeli University School of Medicine, Kocaeli, Turkey
| | - Umut Kalyoncu
- Department of Internal Medicine, Division of Rheumatology, Hacettepe University School of Medicine, Ankara, Turkey
| | - Mehmet Sayarlıoğlu
- Department of Internal Medicine, Division of Rheumatology, Ondokuz Mayıs University School of Medicine, Samsun, Turkey
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