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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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Shenoy A, Davis JPE. Contemporary management of portal vein thromboses in patients with and without cirrhosis. Curr Opin Gastroenterol 2025; 41:97-103. [PMID: 39998941 DOI: 10.1097/mog.0000000000001086] [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: 02/27/2025]
Abstract
PURPOSE OF REVIEW Portal vein thromboses (PVT) is a common clotting disorder that can be seen in patients with and without cirrhosis. There are no current clinical guidelines on management of portal vein thromboses in these two distinct populations given most studies are retrospective and comprised of heterogenous cohorts. RECENT FINDINGS When evaluating PVT, patients must first be stratified into those with cirrhosis and those without cirrhosis. In addition, a novel nomenclature can help categorize specific PVT types and determine the need and response to anticoagulation. The management of PVT in patients with cirrhosis varies and is primarily dependent on whether the PVT is recent or chronic. In contrast, patients without cirrhosis are almost always anticoagulated to avoid complications of PVT. Direct oral anticoagulants, low-molecular weight heparin, and vitamin-K antagonists have all been used in patients with and without cirrhosis, without clear guidance on optimal treatment duration and surveillance. SUMMARY Direct oral anticoagulants are increasingly used for patients with PVT though there is limited data on the safety and efficacy of these medications. The risk/benefit profiles of various anticoagulants must be considered when choosing a therapeutic anticoagulant. There are ongoing studies evaluating outcome measures of different anticoagulants in patients with PVT. Large, multicenter, randomized controlled trials may help elucidate the efficacy of anticoagulants on various outcome measures in PVT, including recanalization, bleeding, and survival.
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Affiliation(s)
- Abhishek Shenoy
- Division of Gastroenterology and Hepatology, Department of Medicine, Central Virginia Veterans Affairs Healthcare System, Richmond, Virginia
| | - Jessica P E Davis
- Division of Gastroenterology and Hepatology, Department of Medicine, Veterans Affairs Medical Center, Washington, District of Columbia, USA
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Kumar S, Batra A, Bansal RK, Sharma ZD, Sisodia S. Portal vein Recanalization via Paraumbilical vein with Direct Intrahepatic Portosystemic Shunt (PVR-DIPS) in Chronic Budd-Chiari Syndrome with Extrahepatic Portal Venous Obstruction. Cardiovasc Intervent Radiol 2025; 48:447-449. [PMID: 40038099 DOI: 10.1007/s00270-025-03999-8] [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: 12/01/2024] [Accepted: 02/15/2025] [Indexed: 03/06/2025]
Abstract
Direct intrahepatic portosystemic shunt (DIPS) with access to the portal vein from the IVC remains a viable option to ameliorate features of portal hypertension in patients of BCS with chronically thrombosed hepatic veins. We present a case of BCS with concurrent extrahepatic portal vein obstruction (EHPVO), in which portal vein recanalization (PVR) was achieved utilizing recanalized paraumbilical venous access. Subsequently PVR-DIPS was performed to ameliorate features of portal hypertension.
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Affiliation(s)
- Saurabh Kumar
- Department of Interventional Radiology, Fortis Memorial Research Institute, Gurugram, Haryana, India.
| | - Apoorva Batra
- Department of Interventional Radiology, Fortis Memorial Research Institute, Gurugram, Haryana, India
| | - Rinkesh Kumar Bansal
- Department of Gastroenterology and Hepatology, Fortis Memorial Research Institute, Gurugram, Haryana, India
| | - Zubin Dev Sharma
- Department of Gastroenterology and Hepatology, Fortis Memorial Research Institute, Gurugram, Haryana, India
| | - Shubham Sisodia
- Department of Gastroenterology and Hepatology, Fortis Memorial Research Institute, Gurugram, Haryana, India
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Gil-Lopez F, Rios-Olais FA, Mercado LA, Harnois DM. Portal Vein Thrombosis in Patients Without Cirrhosis: Current Practical Approaches and Treatment Strategies. Diagnostics (Basel) 2025; 15:721. [PMID: 40150064 PMCID: PMC11941439 DOI: 10.3390/diagnostics15060721] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2025] [Revised: 03/01/2025] [Accepted: 03/10/2025] [Indexed: 03/29/2025] Open
Abstract
Portal vein thrombosis in non-cirrhotic individuals, although uncommon, is an increasingly explored condition that affects mainly young people, consequently representing a significant disease burden. Reports primarily including western European populations have recently shed light regarding the pathophysiology, risk factors, natural history, treatment, and prognosis of this entity. Underlying predisposing conditions are documented in ~70% of cases, encompassing local risk factors, inherited and acquired thrombophilia, cancer, and systemic inflammatory conditions. Non-cirrhotic portal vein thrombosis can cause significant portal hypertension in the acute setting, but, more frequently, significant portal hypertension-related complications arise when the condition becomes chronic and portosystemic collaterals develop, increasing the risk for variceal bleeding and ascites. The diagnostic approach to screen for underlying thrombophilia remains a challenge, and recommendations in this regard, although scarce and backed by scarce evidence, have changed notably in the last years, leaning toward a universal screen in patients who develop this condition without a clear provoking factor. Recently, studies have shown that long-term anticoagulation may be appropriate even in the absence of clear provoking factors or underlying thrombophilia. Future studies should address which patients may benefit from this approach, which patients may not need it, and what the most appropriate strategies are to approach patients who do not recover portal vein patency with anticoagulation to further prevent portal hypertension-related complications.
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Affiliation(s)
- Fernando Gil-Lopez
- Department of Liver Transplant, Mayo Clinic Florida, Jacksonville, FL 32224, USA; (F.G.-L.); (L.A.M.)
| | - Fausto Alfredo Rios-Olais
- Hematology and Oncology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico City C.P. 14080, Mexico;
| | - Lydia A. Mercado
- Department of Liver Transplant, Mayo Clinic Florida, Jacksonville, FL 32224, USA; (F.G.-L.); (L.A.M.)
| | - Denise M. Harnois
- Department of Liver Transplant, Mayo Clinic Florida, Jacksonville, FL 32224, USA; (F.G.-L.); (L.A.M.)
- Department of Gastroenterology and Hepatology, Mayo Clinic Florida, Jacksonville, FL 32224, USA
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Davis JPE, Lim JK, Francis FF, Ahn J. AGA Clinical Practice Update on Management of Portal Vein Thrombosis in Patients With Cirrhosis: Expert Review. Gastroenterology 2025; 168:396-404.e1. [PMID: 39708000 DOI: 10.1053/j.gastro.2024.10.038] [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: 08/13/2024] [Revised: 10/09/2024] [Accepted: 10/21/2024] [Indexed: 12/23/2024]
Abstract
DESCRIPTION Portal vein thromboses (PVTs) are common in patients with cirrhosis and are associated with advanced portal hypertension and mortality. The treatment of PVTs remains a clinical challenge due to limited evidence and competing risks of PVT-associated complications vs bleeding risk of anticoagulation. Significant heterogeneity in PVT phenotype based on anatomic, host, and disease characteristics, and an emerging spectrum of therapeutic options further complicate PVT management. This Clinical Practice Update (CPU) aims to provide best practice advice for the evaluation and management of PVT in cirrhosis, including the role of direct oral anticoagulants and endovascular interventions. METHODS This expert review was commissioned and approved by the American Gastroenterological Association (AGA) Institute CPU Committee and the AGA Governing Board to provide timely guidance on a topic of high clinical importance to the AGA membership, and underwent internal peer review by the CPU Committee and external peer review through standard procedures of Gastroenterology. These Best Practice Advice statements were drawn from a review of the published literature and from expert opinion. Because systematic reviews were not performed, these Best Practice Advice statements do not carry formal ratings regarding the quality of evidence or strength of the presented considerations. Best Practice Advice Statements BEST PRACTICE ADVICE 1: Asymptomatic patients with compensated cirrhosis do not require routine screening for PVT. BEST PRACTICE ADVICE 2: Patients with cirrhosis with PVTs identified on Doppler ultrasound should undergo cross-sectional imaging with computed tomography or magnetic resonance imaging to confirm the diagnosis, evaluate for malignancy, and document the degree of lumen occlusion, clot extent, and chronicity. BEST PRACTICE ADVICE 3: Patients with cirrhosis and PVT do not require a hypercoagulable workup in the absence of additional thromboemboli or laboratory abnormalities or family history suggestive of thrombophilia. BEST PRACTICE ADVICE 4: Patients with cirrhosis and PVT with evidence of intestinal ischemia require urgent anticoagulation to minimize ischemic injury. If available, these patients should be managed by a multidisciplinary team, including gastroenterology and hepatology, interventional radiology, hematology, and surgery. BEST PRACTICE ADVICE 5: Consider observation, with repeat imaging every 3 months until clot regression, in patients with cirrhosis without intestinal ischemia and recent (<6 months) thrombosis involving the intrahepatic portal vein branches or when there is <50% occlusion of the main portal vein, splenic vein, or mesenteric veins. BEST PRACTICE ADVICE 6: Anticoagulation should be considered in patients with cirrhosis without intestinal ischemia who develop recent (<6 months) PVT that is >50% occlusive or involves the main portal vein or mesenteric vessels. Patients who have increased benefit of recanalization include those with involvement of more than 1 vascular bed, those with thrombus progression, potential liver transplantation candidates, and those with inherited thrombophilia. BEST PRACTICE ADVICE 7: Anticoagulation is not advised for patients with cirrhosis with chronic (>6 months) PVT with complete occlusion with collateralization (cavernous transformation). BEST PRACTICE ADVICE 8: Patients with cirrhosis and PVT warrant endoscopic variceal screening if they are not already on nonselective beta-blocker therapy for bleeding prophylaxis. Avoid delays in the initiation of anticoagulation for PVT, as this decreases the odds of portal vein recanalization. BEST PRACTICE ADVICE 9: Vitamin K antagonists, low-molecular-weight heparin, and direct oral anticoagulants are all reasonable anticoagulant options for patients with cirrhosis and PVT. Decision making should be individualized and informed by patient preference and Child-Turcotte-Pugh class. Direct oral anticoagulants may be considered in patients with compensated Child-Turcotte-Pugh class A and Child-Turcotte-Pugh class B cirrhosis and offer convenience as their dosages are independent of international normalized ratio monitoring. BEST PRACTICE ADVICE 10: Patients with cirrhosis on anticoagulation for PVT should have cross-sectional imaging every 3 months to assess response to treatment. If clot regresses, anticoagulation should be continued until transplantation or at least clot resolution in nontransplantation patients. BEST PRACTICE ADVICE 11: Portal vein revascularization with transjugular intrahepatic portosystemic shunting may be considered for selected patients with cirrhosis and PVT who have additional indications for transjugular intrahepatic portosystemic shunting, such as those with refractory ascites or variceal bleeding. Portal vein revascularization with transjugular intrahepatic portosystemic shunting may also be considered for transplantation candidates if recanalization can facilitate the technical feasibility of transplantation.
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Affiliation(s)
- Jessica P E Davis
- Division of Gastroenterology and Hepatology, Department of Medicine, Washington DC Veterans Affairs Medical Center, Washington, District of Columbia.
| | - Joseph K Lim
- Section of Digestive Diseases and Yale Liver Center, Yale School of Medicine, New Haven, Connecticut
| | - Fadi F Francis
- Division of Gastroenterology, Hepatology and Nutrition, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
| | - Joseph Ahn
- Division of Gastroenterology and Hepatology, Department of Medicine, Endeavor Health, Chicago, Illinois
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Akabane M, Imaoka Y, Nakayama T, Esquivel CO, Sasaki K. Effect of TIPS insertion on waitlist mortality and access to liver transplantation in Budd-Chiari syndrome. Liver Transpl 2025; 31:151-160. [PMID: 39177578 DOI: 10.1097/lvt.0000000000000469] [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] [Received: 06/17/2024] [Accepted: 08/15/2024] [Indexed: 08/24/2024]
Abstract
The impact of TIPS on waitlist mortality and liver transplantation (LT) urgency in patients with Budd-Chiari syndrome (BCS) remains unclear. We analyzed patients with BCS listed for LT in the UNOS database (2002-2024) to assess TIPS's impact on waitlist mortality and LT access through competing-risk analysis. We compared trends across 2 phases: phase 1 (2002-2011) and phase 2 (2012-2024). Of 815 patients with BCS, 263 (32.3%) received TIPS at listing. TIPS group had lower MELD-Na scores (20 vs. 22, p < 0.01), milder ascites ( p = 0.01), and fewer Status 1 patients (those at risk of imminent death while awaiting LT) (2.7% vs. 8.3%, p < 0.01) at listing compared to those without TIPS. TIPS patients had lower LT rates (43.3% vs. 56.5%, p < 0.01) and longer waitlist times (350 vs. 113 d, p < 0.01). TIPS use increased in phase 2 (64.3% vs. 35.7%, p < 0.01). Of 426 patients who underwent transplantation, 134 (31.5%) received TIPS, showing lower MELD-Na scores (24 vs. 27, p < 0.01) and better medical conditions (intensive care unit: 14.9% vs. 21.9%, p < 0.01) at LT. Status 1 patients were fewer (3.7% vs. 12.3%, p < 0.01), with longer waiting days (97 vs. 26 d, p < 0.01) in the TIPS group. TIPS use at listing increased from phase 1 (25.6%) to phase 2 (37.7%). From phase 1 to phase 2, ascites severity improved, re-LT cases decreased (phase 1: 9.8% vs. phase 2: 2.2%, p < 0.01), and cold ischemic time slightly decreased (phase 1: 7.0 vs. phase 2: 6.4 h, p = 0.14). Median donor body mass index significantly increased. No significant differences were identified in patient/graft survival at 1-/5-/10-year intervals between phases or TIPS/non-TIPS patients. While 90-day waitlist mortality showed no significant difference ( p = 0.11), TIPS trended toward lower mortality (subhazard ratio [sHR]: 0.70 [0.45-1.08]). Multivariable analysis indicated that TIPS was a significant factor in decreasing mortality (sHR: 0.45 [0.27-0.77], p < 0.01). TIPS group also showed significantly lower LT access (sHR: 0.65 [0.53-0.81], p < 0.01). Multivariable analysis showed that TIPS was a significant factor in decreasing access to LT (sHR: 0.60 [0.46-0.77], p < 0.01). Subgroup analysis excluding Status 1 or HCC showed similar trends. TIPS in patients with BCS listed for LT reduces waitlist mortality and LT access, supporting its bridging role.
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Affiliation(s)
- Miho Akabane
- Division of Abdominal Transplant, Department of Surgery, Stanford University Medical Center, Stanford, California, USA
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Elkrief L, Denecheau-Girard C, Magaz M, Praktiknjo M, Colucci N, Ollivier-Hourmand I, Dumortier J, Simon Talero M, Tellez L, Artru F, Meszaros M, Verhelst X, Tabchouri N, Beires F, Andaluz I, Leo M, Diekhöner M, Dokmak S, Fundora Y, Vidal-Gonzalez J, Toso C, Plessier A, Carlos Garcia Pagan J, Rautou PE. Abdominal surgery in patients with chronic noncirrhotic extrahepatic portal vein obstruction: A multicenter retrospective study. Hepatology 2025; 81:152-167. [PMID: 38683626 DOI: 10.1097/hep.0000000000000901] [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] [Received: 11/14/2023] [Accepted: 03/22/2024] [Indexed: 05/01/2024]
Abstract
BACKGROUND AND AIMS In patients with noncirrhotic chronic extrahepatic portal vein obstruction (EHPVO), data on the morbimortality of abdominal surgery are scarce. APPROACH AND RESULTS We retrospectively analyzed the charts of 76 patients (78 interventions) with EHPVO undergoing abdominal surgery within the Vascular Disease Interest Group network. Fourteen percent of the patients had ≥1 major bleeding (unrelated to portal hypertension) and 21% had ≥1 Dindo-Clavien grade ≥3 postoperative complications within 1 month after surgery. Fifteen percent had ≥1 portal hypertension-related complication within 3 months after surgery. Three patients died within 12 months after surgery. An unfavorable outcome (ie, ≥1 abovementioned complication or death) occurred in 37% of the patients and was associated with a history of ascites and with nonwall, noncholecystectomy surgical intervention: 17% of the patients with none of these features had an unfavorable outcome, versus 48% and 100% when one or both features were present, respectively. We then compared 63/76 patients with EHPVO with 126 matched (2:1) control patients without EHPVO but with similar surgical interventions. As compared with control patients, the incidence of major bleeding ( p <0.001) and portal hypertension-related complication ( p <0.001) was significantly higher in patients with EHPVO, but not that of grade ≥3 postoperative complications nor of death. The incidence of unfavorable postoperative outcomes was significantly higher in patients with EHPVO than in those without (33% vs. 18%, p =0.01). CONCLUSIONS Patients with EHPVO are at high risk of major perioperative or postoperative bleeding and postoperative complications, especially in those with ascites or undergoing surgery other than wall surgery or cholecystectomy.
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Affiliation(s)
- Laure Elkrief
- Faculté de médecine et service d'hépato-gastroentérologie, CHRU de Tours, ERN RARE-LIVER, France
- Inserm, Centre de recherche sur l'inflammation, UMR, Paris, France
| | | | - Marta Magaz
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Departament de Medicina i Ciències de la Salut, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Universitat de Barcelona
| | | | - Nicola Colucci
- Service de chirurgie viscérale, Hôpitaux Universitaires de Genève, Geneva, Switzerland
| | | | - Jérôme Dumortier
- Service d'Hépatogastroentérologie, Hôpital Edouard Herriot, Lyon
| | - Macarena Simon Talero
- Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Universitat Autònoma de Barcelona, Barcelona, Spain
| | - Luis Tellez
- Departamento de Gastroenterología y Hepatología Hospital Universitario Ramon y Cajal, Madrid, Spain
| | - Florent Artru
- Service d'hépato-gastroentérologie, CHUV, Lausanne, Switzerland
| | | | - Xavier Verhelst
- Department of Gastroenterology and Hepatology, Ghent University Hospital, Belgium
| | - Nicolas Tabchouri
- Service de chirurgie digestive et de transplantation hépatique, CHRU de Tours, France
| | - Francisca Beires
- AP-HP, Service d'Hépatologie, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Irene Andaluz
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Departament de Medicina i Ciències de la Salut, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Universitat de Barcelona
| | - Massimo Leo
- AP-HP, Service d'Hépatologie, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Mara Diekhöner
- Department of Internal Medicine I, University Hospital Bonn, Bonn, Germany
| | - Safi Dokmak
- AP-HP, Service de chirurgie hépato-biliaire et pancréatique, Hôpital Beaujon, DMU DIGEST, Clichy, France
| | - Yliam Fundora
- Department of General & Digestive Surgery, Institut de Malalties Digestives I Metabòliques, Hospital Clínic, University of Barcelona, IDIBAPS, Spain
| | - Judit Vidal-Gonzalez
- Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Universitat Autònoma de Barcelona, Barcelona, Spain
| | - Christian Toso
- Service de chirurgie viscérale, Hôpitaux Universitaires de Genève, Geneva, Switzerland
| | - Aurélie Plessier
- Inserm, Centre de recherche sur l'inflammation, UMR, Paris, France
- AP-HP, Service d'Hépatologie, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Juan Carlos Garcia Pagan
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Departament de Medicina i Ciències de la Salut, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Universitat de Barcelona
| | - Pierre-Emmanuel Rautou
- Inserm, Centre de recherche sur l'inflammation, UMR, Paris, France
- AP-HP, Service d'Hépatologie, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
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Shimizu T, Yoshioka M, Matsushita A, Ueda J, Kawashima M, Ono T, Kawano Y, Yoshida H. Esophagogastric Varix Caused by Extrahepatic Portal Vein Obstruction with Essential Thrombocythemia: A Case Report. J NIPPON MED SCH 2024; 91:541-547. [PMID: 37558426 DOI: 10.1272/jnms.jnms.2024_91-601] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/11/2023]
Abstract
Extrahepatic portal vein obstruction (EHPVO) is a rare disease-causing form of portal hypertension. Myeloproliferative neoplasm (MPN) including essential thrombocythemia (ET) is a reported risk factor for EHPVO due to underlying persistent thrombophilia. A Japanese woman in her 40s was referred to our hospital with a 1-month history of gastric variceal bleeding due to EHPVO. Laboratory investigation showed thrombocytosis despite portal hypertension. She had a mutation in clonal marker JAK2V617F with EHPVO, which prompted us to consult a hematologist. A bone marrow biopsy revealed megakaryocyte lineage proliferation, which confirmed a diagnosis of ET. Esophagogastroduodenoscopy revealed esophagogastric varices (LsF2CbRC2, Lg-cF1RC1), and abdominal computed tomography and angiography revealed splenomegaly and portal vein thrombosis with cavernous transformation, which suggested EHPVO. The patient had a history of ruptured esophagogastric varices and required prophylaxis against further variceal bleeding before antithrombotic therapy for EHPVO with ET. We performed laparoscopic Hassab's operation followed by endoscopic variceal ligation (EVL) and hematological cytoreduction therapy. Laparoscopic Hassab's operation and three bi-monthly EVL procedures improved the esophagogastric varix (LmF0RC0, Lg-f F0RC0) at 6 months after surgery. Cytoreduction therapy reduced platelet count to 60.1 × 104/uL, and the patient was very healthy at 7 months after surgery. Patients with EHPVO are traditionally referred to a gastroenterologist for abdominal pain, intestinal bleeding, or refractory ascites; however, hypercoagulative disease may be occult in such patients and require the attention of a hematologist. When treating patients with EHPVO, gastroenterologists should screen for hematological disease, including MPN.
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Affiliation(s)
- Tetsuya Shimizu
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Masato Yoshioka
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Akira Matsushita
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Junji Ueda
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Mampei Kawashima
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Takashi Ono
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Yoichi Kawano
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Hiroshi Yoshida
- Department of Gastroenterological Surgery, Nippon Medical School
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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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10
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Ollivier-Hourmand I, Lebedel L, Alabau BB, Goria O, Bureau C, Dumortier J, Heurgué A, Silvain C, De-Ledinghen V, Rautou PE, Payancé A, Ballester TG, Alvarado-Tapias E, Hernández-Gea V, Valla D, Zekrini K, Nga Nguyen TT, Dao T, Garcia Pagan JC, Morello R, Plessier A. Recurrent splanchnic and extrasplanchnic thrombotic events in patients with non-cirrhotic portal vein thrombosis associated with local factors. J Hepatol 2024; 81:451-460. [PMID: 38679069 DOI: 10.1016/j.jhep.2024.04.016] [Citation(s) in RCA: 3] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/12/2023] [Revised: 04/04/2024] [Accepted: 04/13/2024] [Indexed: 05/01/2024]
Abstract
BACKGROUND & AIMS One-third of non-cirrhotic portal vein thrombosis (NCPVT) cases are associated with local factors. The risk of rethrombosis after anticoagulation withdrawal is unknown. We aimed to determine factors associated with new splanchnic or extrasplanchnic thrombotic events in this setting. METHODS We performed a retrospective study including cases of recent NCPVT associated with local factors. High- and low-risk prothrombotic factors, prespecified according to RIPORT study criteria, were assessed. Univariate and multivariate Cox models assessed the influence of different variables on the occurrence of new thrombotic events. RESULTS At baseline, 83/154 (53.9%) patients had at least one prothrombotic factor including 50 (32.5%) with a high-risk and 33 (21.4%) with a low-risk prothrombotic factor. Oestrogen-containing contraception was discontinued in all patients. During follow-up, 63/140 (45%) patients had at least one prothrombotic factor, including 47 (33.6%) with a high-risk and 16 (11.4%) with a low-risk prothrombotic factor. Seventeen new thrombotic events occurred after a median follow-up of 52 (IQR 14-62) (min-max 3.0-69.0) months. New thromboses were associated with high-risk factors (hazard ratio [HR] 3.817, 95% CI 1.303-11.180, p = 0.015), but were inversely related to recanalization (HR 0.222, 95% CI 0.078-0.635, p = 0.005) and anticoagulation (HR 0.976, 95% CI 0.956-0.995, p = 0.016). When a high-risk factor was present a new thrombotic event occurred in 7.4%, 14.6%, 14.6% and 28.8% of patients at 1, 3, 5 and 7 years under anticoagulants, respectively, compared to 21.2%, 21.2%, 58% and 58% without anticoagulants, respectively. CONCLUSIONS In cases of recent NCPVT associated with local factors, high-risk factors for thrombosis are associated with new thrombotic events. Permanent anticoagulation appears beneficial in this high-risk situation. IMPACT AND IMPLICATIONS In non-cirrhotic portal vein thrombosis (NCPVT) associated with local factors, systematic screening for prothrombotic factors is recommended, but the prevalence of the latter is not clearly established, and the risk of recurrent intra or extrasplanchnic thromboembolism is poorly described. Thus, interest in permanent anticoagulation remains. NCPVT associated with local factors is a matter of concern for hepatologists, gastroenterologists and digestive surgeons. Due to a lack of knowledge, practices are heterogeneous. Our findings highlight that systematic screening for prothrombotic factors in NCPVT is needed even when associated with local factors, as it may justify long-term anticoagulation for the prevention of new intra or extrasplanchnic thrombotic events in at least one-third of cases. The interest in long-term anticoagulation should be investigated prospectively in the absence of high-risk prothrombotic factors. CLINICAL TRIAL NUMBER NCT0536064.
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Affiliation(s)
| | - Louise Lebedel
- Hepatology unit, University Hospital Côte de Nacre, Caen, France.
| | - Berta Bartroli Alabau
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain; Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Spain
| | - Odile Goria
- Hepatology unit, University Hospital Charles-Nicolle, Rouen, France
| | | | - Jérome Dumortier
- Hepatology unit, University Hospital Edouard Herriot, Lyon, France
| | | | | | - Victor De-Ledinghen
- Hepatology unit, University Hospital Haut Levêque & INSERM U1312; Université de Bordeaux, Bordeaux, France
| | - Pierre-Emmanuel Rautou
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Audrey Payancé
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Teresa García Ballester
- Cardiology Department, Hospital Clínico Universitario, Universitat de Valencia, INCLIVA, Valencia, Spain
| | - Edilmar Alvarado-Tapias
- Centro De Investigación Biomédica Red De Enfermedades Hepáticas y Digestivas (CIBERehd)), Spain; Servei De Patologia Digestiva, Hospital De La Santa Creu I Sant Pau, Spain
| | - Virginia Hernández-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain; Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Spain
| | - Dominique Valla
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Kamal Zekrini
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | | | - Thong Dao
- Hepatology unit, University Hospital Côte de Nacre, Caen, France
| | - Juan Carlos Garcia Pagan
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain; Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Spain
| | - Rémy Morello
- Statistics Department, Hospital Côte de Nacre, Caen, France
| | - Aurélie Plessier
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
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11
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Elkrief L, Hernandez-Gea V, Senzolo M, Albillos A, Baiges A, Berzigotti A, Bureau C, Murad SD, De Gottardi A, Durand F, Garcia-Pagan JC, Lisman T, Mandorfer M, McLin V, Moga L, Nery F, Northup P, Nuzzo A, Paradis V, Patch D, Payancé A, Plaforet V, Plessier A, Poisson J, Roberts L, Salem R, Sarin S, Shukla A, Toso C, Tripathi D, Valla D, Ronot M, Rautou PE. Portal vein thrombosis: diagnosis, management, and endpoints for future clinical studies. Lancet Gastroenterol Hepatol 2024; 9:859-883. [PMID: 38996577 DOI: 10.1016/s2468-1253(24)00155-9] [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] [Received: 02/21/2024] [Revised: 04/27/2024] [Accepted: 05/08/2024] [Indexed: 07/14/2024]
Abstract
Portal vein thrombosis (PVT) refers to the development of a non-malignant obstruction of the portal vein, its branches, its radicles, or a combination. This Review first provides a comprehensive overview of all aspects of PVT, namely the specifics of the portal venous system, the risk factors for PVT, the pathophysiology of portal hypertension in PVT, the interest in non-invasive tests, as well as therapeutic approaches including the effect of treating risk factors for PVT or cause of cirrhosis, anticoagulation, portal vein recanalisation by interventional radiology, and prevention and management of variceal bleeding in patients with PVT. Specific issues are also addressed including portal cholangiopathy, mesenteric ischaemia and intestinal necrosis, quality of life, fertility, contraception and pregnancy, and PVT in children. This Review will then present endpoints for future clinical studies in PVT, both in patients with and without cirrhosis, agreed by a large panel of experts through a Delphi consensus process. These endpoints include classification of portal vein thrombus extension, classification of PVT evolution, timing of assessment of PVT, and global endpoints for studies on PVT including clinical outcomes. These endpoints will help homogenise studies on PVT and thus facilitate reporting, comparison between studies, and validation of future studies and trials on PVT.
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Affiliation(s)
- Laure Elkrief
- Faculté de médecine de Tours, et service d'hépato-gastroentérologie, Le Centre Hospitalier Régional Universitaire de Tours, Tours, France; Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France
| | - Virginia Hernandez-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, Institut de Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
| | - Marco Senzolo
- Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, Italy
| | - Agustin Albillos
- Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departamento de Gastroenterología y Hepatología, Instituto Ramón y Cajal de Investigación Sanitaria, Hospital Universitario Ramon y Cajal, Madrid, Spain
| | - Anna Baiges
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, Institut de Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
| | - Annalisa Berzigotti
- Department of Visceral Surgery and Medicine, Inselspital, Bern University Hospital, Bern, Switzerland
| | - Christophe Bureau
- Service d'Hépatologie Hôpital Rangueil, Université Paul Sabatier, Toulouse, France
| | - Sarwa Darwish Murad
- Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center, Rotterdam, Netherlands
| | - Andrea De Gottardi
- Gastroenterology and Hepatology Department, Ente Ospedaliero Cantonale Faculty of Biomedical Sciences of Università della Svizzera Italiana, Lugano, Switzerland
| | - François Durand
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Juan-Carlos Garcia-Pagan
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, Institut de Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
| | - Ton Lisman
- Department of Surgery, University Medical Center Groningen, Groningen, Netherlands
| | - Mattias Mandorfer
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
| | - Valérie McLin
- Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland
| | - Lucile Moga
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Filipe Nery
- Immuno-Physiology and Pharmacology Department, School of Medicine and Biomedical Sciences, University of Porto, Portugal
| | - Patrick Northup
- Transplant Institute and Division of Gastroenterology, NYU Langone, New York, NY, USA
| | - Alexandre Nuzzo
- Intestinal Stroke Center, Department of Gastroenterology, IBD and Intestinal Failure, AP-HP Hôpital Beaujon, Clichy, France; Laboratory for Vascular and Translational Science, INSERM UMR 1148, Paris, France
| | - Valérie Paradis
- Department of Pathology, AP-HP Hôpital Beaujon, Clichy, France
| | - David Patch
- Department of Hepatology and Liver Transplantation, Royal Free Hospital, London, UK
| | - Audrey Payancé
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | | | - Aurélie Plessier
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Johanne Poisson
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service de Gériatrie, Hôpital Corentin Celton (AP-HP), Paris, France
| | - Lara Roberts
- Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK
| | - Riad Salem
- Northwestern Memorial Hospital, Northwestern University, Chicago, IL, USA
| | - Shiv Sarin
- Institute of Liver and Biliary Sciences, New Delhi, India
| | - Akash Shukla
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, India
| | - Christian Toso
- Service de Chirurgie Viscérale, Hôpitaux Universitaires de Genève, Geneva, Switzerland
| | - Dhiraj Tripathi
- Department of Liver and Hepato-Pancreato-Biliary Unit, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham, UK; Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
| | - Dominique Valla
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Maxime Ronot
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service de Radiologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Pierre-Emmanuel Rautou
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France.
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12
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Dongelmans E, Erler N, Adam R, Nadalin S, Karam V, Yilmaz S, Kelly C, Pirenne J, Acarli K, Allison M, Hakeem A, Dhakshinamoorthy V, Fedaruk D, Rummo O, Kilic M, Nordin A, Fischer L, Parente A, Mirza D, Bennet W, Tokat Y, Faitot F, Antonelli BB, Berlakovich G, Patch D, Berrevoet F, Ribnikar M, Gerster T, Savier E, Gruttadauria S, Ericzon BG, Valdivieso A, Cuervas-Mons V, Perez Saborido B, Croner RS, De Carlis L, Magini G, Rossi R, Popescu I, Razvan L, Schneeberger S, Blokzijl H, Llado L, Gomez Bravo MA, Duvoux C, Mezjlík V, Oniscu GC, Pearson K, Dayangac M, Lucidi V, Detry O, Rotellar F, den Hoed C, Polak WG, Darwish Murad S. Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers. Hepatology 2024; 80:136-151. [PMID: 38358658 DOI: 10.1097/hep.0000000000000778] [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] [Received: 08/22/2023] [Accepted: 12/06/2023] [Indexed: 02/16/2024]
Abstract
BACKGROUND AND AIMS Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (post-LT) outcomes in Europe. APPROACH AND RESULTS Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). CONCLUSIONS LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.
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Affiliation(s)
- Edo Dongelmans
- Department of Gastroenterology and Hepatology, Erasmus MC Transplant Institute, Erasmus MC University Medical Center, Rotterdam, the Netherlands
| | - Nicole Erler
- Department of Biostatistics, Erasmus University Medical Center, Rotterdam, The Netherlands
| | - Rene Adam
- Department of Hepato-Biliary Surgery, Cancer and Transplantation Unit, Hospital Paul Brousse, Villejuif, France
| | - Silvio Nadalin
- Department of General, Visceral and Transplant Surgery, Universitätsklinik Tübingen, Tubingen, Germany
| | - Vincent Karam
- Department of Hepato-Biliary Surgery, Cancer and Transplantation Unit, Hospital Paul Brousse, Villejuif, France
| | - Sezai Yilmaz
- Department of Surgery, Liver Transplant Institute, Turgut Özal Medical Center, Malatya, Turkey
| | - Claire Kelly
- Institute of Liver Studies, King's College Hospital, London, UK
| | - Jacques Pirenne
- Department of Abdominal Transplant Surgery, Universitaire Ziekenhuizen Leuven, Belgium
| | - Koray Acarli
- Department of Liver and Biliary Tract Surgery, Memorial Hospital, Istanbul, Turkey
| | - Michael Allison
- Liver Unit, Cambridge University Hospitals NHS Foundation Trust, Cambridge NIHR Biomedical Research Center, Cambridge, UK
| | - Abdul Hakeem
- Department of HPB Surgery and Liver Transplantation, Leeds Teaching Hospitals NHS Trust, Leeds, UK
| | | | - Dzmitry Fedaruk
- Department of Transplantation, Minsk Scientific and Practical Center for Surgery, Transplantology and Hepatology, Minsk, Belarus
| | - Oleg Rummo
- Department of Transplantation, Minsk Scientific and Practical Center for Surgery, Transplantology and Hepatology, Minsk, Belarus
| | - Murat Kilic
- Department of Surgery, Kent Hospital, Izmir, Turkey
| | - Arno Nordin
- Transplantation and Liver Surgery Unit, Helsinki University Hospital, Helsinki, Finland
| | - Lutz Fischer
- Department of Surgery, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | | | - Darius Mirza
- Liver Unit, Queen Elizabeth Hospital, Birmingham, UK
| | - William Bennet
- Department of Surgery, Sahlgrenska University Hospital, Gothenburg, Sweden
| | - Yaman Tokat
- Department of General Surgery, International Liver Center and Acibadem Health Care Hospitals, Istanbul, Turkey
| | - Francois Faitot
- Department of HPB Surgery and Transplantation, C.H.R.U. de Strasbourg, Strasbourg, France
| | - Barbara B Antonelli
- General and Liver Transplant Surgery Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Gabriela Berlakovich
- Department of Transplantation Surgery, Medical University of Vienna, Wien, Austria
| | - David Patch
- Department of Hepatology and Liver Transplantation, Royal Free Hospital, London, UK
| | - Frederik Berrevoet
- Department of General and HPB Surgery and Liver Transplantation, University Hospital Gent, Ghent, Belgium
| | - Marija Ribnikar
- Department of Gastroenterology, University Medical Center Lubljana, Ljubljana, Slovenia
| | - Theophile Gerster
- Department of Gastroenterology and Hepatology, C.H.U. de Grenoble, Grenoble, France
| | - Eric Savier
- Department of Digestive Surgery and Liver Transplantation, Pitie Salpetriere university hospital, Sorbonne University, Paris, France
| | - Salvatore Gruttadauria
- Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, IRCCS-ISMETT (Istituto di Ricovero e Cura a Carattere Scientifico-Istituto Mediterraneo per i Trapianti e Terapie ad alta specializzazione), UPMC (University of Pittsburgh Medical Center), Palermo, Italy
- Department of Surgery and Medical and Surgical Specialties, University of Catania, Catania, Italy
| | - Bo-Göran Ericzon
- Department of Transplantation Surgery, Karolinska University Hospital, Huddinge, Sweden
| | - Andrés Valdivieso
- Department of HBP Surgery and Liver Transplantation, Cruces University hospital, Bilbao, Spain
| | | | - Baltasar Perez Saborido
- Department of General and Digestive Surgery, Hospital Universitario "Rio Hortega", Valladolid, Spain
| | - Roland S Croner
- Department of General-, Visceral-, Vascular- and Transplant Surgery, University Hospital Magdeburg, Germany
| | - Luciano De Carlis
- Department of General Surgery and Transplantation, ASST Grande Ospedale Metropolitano Niguarda, University of Milano-Bicocca, Milan, Italy
| | - Giulia Magini
- Department of Surgery, Hôpital Universitaire de Genève, Geneve, Switzerland
| | - Roberta Rossi
- Department of Gastroenterology and Transplantation, Università Politecnica delle Marche, Ancona, Italy
| | - Irinel Popescu
- Department of Surgery, University of Medicine "Carol Davila", Bucharest, Romania
| | - Laze Razvan
- Department of Surgery, University of Medicine "Carol Davila", Bucharest, Romania
| | - Stefan Schneeberger
- Department of Visceral, Transplant and Thoracic Surgery, University Hospital, Innsbruck, Austria
| | - Hans Blokzijl
- Department of Gastroenterology and Hepatology, University Medical Center Groningen, Groningen, the Netherlands
| | - Laura Llado
- Department of Surgery, Hospital Universitari de Bellvitge, Barcelona, Spain
| | | | - Christophe Duvoux
- Department of Medical Liver Transplant Unit and Liver, Hôpital Henri Mondor, Creteil, France
| | - Vladimír Mezjlík
- Department of Transplantation, Center of cardiovascular surgery and transplantations, Brno, Czech Republic
| | - Gabriel C Oniscu
- Edinburgh Transplant Center, Royal Infirmary of Edinburgh, Edinburg, UK
| | - Kelsey Pearson
- Edinburgh Transplant Center, Royal Infirmary of Edinburgh, Edinburg, UK
| | - Murat Dayangac
- Center for Organ Transplantation, Medipol University Hospital, Istanbul, Turkey
| | - Valerio Lucidi
- Department of abdominal surgery, Unit of Hepato-biliary surgery and Liver Transplantation, Hôpital Erasme, Cliniques Universitaires de Bruxelles, Brussels, Belgium
| | - Olivier Detry
- Department of Abdominal Surgery and Transplantation, CHU Liege, Liege, Belgium
| | - Fernando Rotellar
- Department of General and Digestive Surgery, Clinica Universitaria de Navarra, Pamplona, Spain
| | - Caroline den Hoed
- Department of Gastroenterology and Hepatology, Erasmus MC Transplant Institute, Erasmus MC University Medical Center, Rotterdam, the Netherlands
| | - Wojciech G Polak
- Department of Surgery, Division of HPB and Transplant Surgery, Erasmus MC Transplant Institute, Erasmus MC University Medical Center, Rotterdam, the Netherlands
| | - Sarwa Darwish Murad
- Department of Gastroenterology and Hepatology, Erasmus MC Transplant Institute, Erasmus MC University Medical Center, Rotterdam, the Netherlands
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13
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Willington AJ, Tripathi D. Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis. World J Hepatol 2024; 16:751-765. [PMID: 38818283 PMCID: PMC11135268 DOI: 10.4254/wjh.v16.i5.751] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2023] [Revised: 02/20/2024] [Accepted: 04/08/2024] [Indexed: 05/22/2024] Open
Abstract
Non-cirrhotic non-malignant portal vein thrombosis (NCPVT) is an uncommon condition characterised by thrombosis of the portal vein, with or without extension into other mesenteric veins, in the absence of cirrhosis or intra-abdominal malignancy. Complications can include intestinal infarction, variceal bleeding and portal biliopathy. In this article, we address current concepts in the management of NCPVT including identification of risk factors, classification and treatment, and review the latest evidence on medical and interventional management options.
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Affiliation(s)
- Adam J Willington
- Department of Hepatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, United Kingdom
| | - Dhiraj Tripathi
- Department of Hepatology, University Hospitals Birmingham, Birmingham B15 2TH, United Kingdom.
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14
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Shimizu T, Yoshida H, Taniai N, Yoshioka M, Kawano Y, Matsushita A, Ueda J, Iwai T, Murokawa T, Ono T, Hamaguchi A. Clinical features of extrahepatic portal vein obstruction: Myeloproliferative neoplasms eliminate hypersplenic hematologic changes in extrahepatic portal vein obstruction. Intractable Rare Dis Res 2024; 13:63-68. [PMID: 38404733 PMCID: PMC10883843 DOI: 10.5582/irdr.2023.01106] [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: 11/19/2023] [Revised: 01/10/2024] [Accepted: 01/12/2024] [Indexed: 02/27/2024] Open
Abstract
Extrahepatic portal vein obstruction (EHPVO) is a rare disease. Most EHPVO patients are usually referred to a gastroenterologist for intestinal bleeding and hypersplenic thrombocytopenia; however, hypercoagulative diseases may be occult in these patients and require anticoagulation. The purpose of this study was to elucidate the clinical characteristics of EHPVO. We conducted a retrospective analysis of the hospital database, evaluating the medical records of 15 patients (7 males, 8 females, mean age of onset 42.0 years, range 5-74 years). Thirteen of 15 EHPVO patients (86.7%) had intestinal varices. These included 10 esophageal (66.7%), 12 gastric (80.0%), and 6 ectopic varices (40.0%). Nine (60.0%) of 15 had a history of intestinal bleeding. Regarding comorbidities, 5 of 15 (33.3%) suffered from vascular diseases, including acute myocardial infarction, cerebral infarction, pulmonary embolism, Budd-Chiari syndrome, and mesenteric vein thrombosis. The former 3 vascular commodities manifested at less than 32 years of age. Four patients (26.7%) with JAK2V617F mutation were diagnosed as myeloproliferative neoplasm (MPN). 72.3% of EHPVO patients without MPN experienced thrombocytopenic state. No EHPVO patients with MPN experienced thrombo-leukocytopenia. The elevation of white blood cell and platelet counts, and decrease of protein S were seen in EHPVO with MPN, compared with EHPVO without MPN. EHPVO is frequently associated with underlying hypercoagulative factors, causing a dilemma between thrombotic complications and portal hypertensive bleeding. Most EHPVO patients experience an evident thrombocytopenic state due to severe hypersplenism; however, hypersplenic hematologic changes are eliminated in EHPVO with MPN. MPN should be suspected in EHPVO patients negative for thrombo-leukocytopenia.
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Affiliation(s)
- Tetsuya Shimizu
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Hiroshi Yoshida
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Nobuhiko Taniai
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Masato Yoshioka
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Yoichi Kawano
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Akira Matsushita
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Junji Ueda
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Takuma Iwai
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Takahiro Murokawa
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Takashi Ono
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Akira Hamaguchi
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
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15
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Riescher-Tuczkiewicz A, Elkrief L, Rautou PE. [Splanchnic vein thrombosis]. Rev Med Interne 2024; 45:17-25. [PMID: 37838484 DOI: 10.1016/j.revmed.2023.07.005] [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: 06/15/2023] [Accepted: 07/25/2023] [Indexed: 10/16/2023]
Abstract
Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis, and is associated with a worse course of cirrhosis. Indications for anticoagulation in patients with cirrhosis are increasing. Transjugular intrahepatic portosystemic shunt is a second-line procedure in this setting. Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.
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Affiliation(s)
- A Riescher-Tuczkiewicz
- Université Paris-Cité, Inserm, centre de recherche sur l'inflammation, UMR 1149, Paris, France.
| | - L Elkrief
- Université de Tours, service d'hépato-gastro-entérologie, CHRU de Tours, Tours, France
| | - P-E Rautou
- Université Paris-Cité, Inserm, centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d'hépatologie, AP-HP, hôpital Beaujon, DMU DIGEST, centre de référence des maladies vasculaires du foie, FILFOIE, ERN RARE-LIVER, Clichy, France
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16
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Wang C, Li T, Chen K, Niu H, Bai Y, Liu J, Wang Y, Ju S, Yao W, Zhao G, Xiong B, Zhou G. Reversion of liver cirrhosis after endovascular treatment in Chinese patients with Budd-Chiari syndrome. Hepatol Res 2023; 53:1198-1212. [PMID: 37632703 DOI: 10.1111/hepr.13960] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/31/2023] [Revised: 08/14/2023] [Accepted: 08/16/2023] [Indexed: 08/28/2023]
Abstract
AIMS To investigate the impact of endovascular (EV) treatment on liver cirrhosis in Chinese patients with Budd-Chiari syndrome (BCS). METHODS From September 2011 to March 2022, 97 patients from four hospitals in China who were diagnosed with primary BCS complicated with liver cirrhosis and received EV treatment were retrospectively enrolled in this study for clinical analysis. In addition, liver tissues for basic research were acquired from 25 patients between June 2022 and March 2023, including six with benign liver tumors, 11 with BCS before EV treatment, and eight with EV-treated BCS. Liver cirrhosis was assessed by clinical outcomes, histological studies, and the expression of related genes at the mRNA and protein levels. RESULTS The patients with BCS had better liver function after EV treatment, evidenced by an increased albumin level and reduced total bilirubin, ALT, and AST. The imaging findings suggested an amelioration of liver cirrhosis and portal hypertension, including increased portal vein velocity (13.52 ± 8.89 cm/s vs. 17.51 ± 6.67 cm/s, p < 0.001) and decreased liver stiffness (30.37 ± 6.39 kPa vs. 23.70 ± 7.99 kPa, p < 0.001), portal vein diameter (14.97 ± 3.42 mm vs. 13.36 ± 2.89 mm, p < 0.001), and spleen volume (870.00 ± 355.61 cm3 vs. 771.36 ± 277.45 cm3 , p < 0.001). Furthermore, histological studies revealed that EV treatment resulted in a restoration of liver architecture with reduced extracellular matrix deposition. Meanwhile, hepatic angiogenesis and inflammation, which have a close relationship with cirrhosis, were also inhibited. In addition, the state of hepatocytes switches from apoptosis to proliferation after EV treatment. CONCLUSIONS BCS-induced liver cirrhosis could be reversed by EV treatment from macroscopic to microscopic dimensions. Our study may provide further insights into understanding BCS and treating cirrhosis.
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Affiliation(s)
- Chaoyang Wang
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Tongqiang Li
- Department of Interventional Radiology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China
| | - Kequan Chen
- Department of Gastroenterology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China
| | - Huanzhang Niu
- Department of Interventional Radiology, The First Affiliated Hospital of Henan University of Science and Technology, Luoyang, China
| | - Yaowei Bai
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jiacheng Liu
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yingliang Wang
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Shuguang Ju
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Wei Yao
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Guorui Zhao
- Department of Infectious Disease, Henan Infectious Disease Hospital, Zhengzhou, China
| | - Bin Xiong
- Department of Interventional Radiology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China
| | - Guofeng Zhou
- Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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17
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Fernandes R, Curralo E, Cunha S, Ferreira F. Conservatively Treated Mesenteric Vein Thrombosis in a 48-Year-Old Obese Female: A Case Report. Cureus 2023; 15:e49966. [PMID: 38058525 PMCID: PMC10697179 DOI: 10.7759/cureus.49966] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/05/2023] [Indexed: 12/08/2023] Open
Abstract
Mesenteric vein thrombosis (MVT) is a rare pathological entity that results in compromised venous return from the intestine due to involvement, in most cases, of the superior mesenteric vein. Its diagnosis is not straightforward, since the findings on physical examination are often disproportionate to the patient's pain complaints, leading to it being undervalued by clinicians. The patient is a 48-year-old female with a medical history of essential arterial hypertension, dyslipidemia, class II obesity, and Hashimoto's thyroiditis. She also had a family history of gastric and colon cancer, with an age at diagnosis of over 70 years. She went to an appointment at a primary care facility for abdominal pain located in the left hypochondrium and flank, with ipsilateral lumbar irradiation and no other accompanying symptoms. Physical examination revealed a globose, depressible abdomen, painful on palpation of the left quadrants, with no other associated signs of peritoneal irritation. Due to suspicion of acute diverticulitis, the patient was referred to the emergency department (ED) for assessment by general surgery. In the emergency department, given the patient's body type and the fact that the physical examination findings were disproportionate to her symptoms, an abdominal and pelvic computed tomography (CT) scan was ordered, which revealed complete thrombosis of the entire length of the inferior mesenteric vein, with a focal extension of the thrombus, partially obstructing the confluence with the superior mesenteric and portal veins. Various complementary diagnostic tests were requested, which revealed no clinically significant findings, and obesity was therefore identified as the only risk factor. In this context, the patient started anticoagulation with warfarin, with the indication that it should be ad aeternum. To date, the patient remains asymptomatic, and there have been no new thrombotic events. Given the high morbidity and mortality rates of this pathological entity, it is imperative that clinicians are trained to recognize the typical signs of mesenteric venous thrombosis, in the characteristic epidemiological context, in order to establish a timely diagnosis and carry out early targeted therapeutic intervention.
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Affiliation(s)
- Rita Fernandes
- General Practice, Unidade Local de Saúde (ULS) do Alto Minho, Viana do Castelo, PRT
| | - Estefania Curralo
- Family Medicine, Unidade Local de Saúde (ULS) do Alto Minho, Viana do Castelo, PRT
| | - Silvia Cunha
- Family Medicine, Unidade Local de Saúde (ULS) do Alto Minho, Viana do Castelo, PRT
| | - Fabíola Ferreira
- Family Medicine, Unidade Local de Saúde (ULS) do Alto Minho, Viana do Castelo, PRT
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18
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Samanta A, Sen Sarma M, Yadav R. Budd-Chiari syndrome in children: Challenges and outcome. World J Hepatol 2023; 15:1174-1187. [PMID: 38075006 PMCID: PMC10698347 DOI: 10.4254/wjh.v15.i11.1174] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2023] [Revised: 10/08/2023] [Accepted: 10/30/2023] [Indexed: 11/24/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.
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Affiliation(s)
- Arghya Samanta
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India.
| | - Rajanikant Yadav
- Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
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19
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Rabiee A, Cornman-Homonoff J, Kunstman JW, Garcia-Tsao G, Taddei TH. Interventional Radiology and Surgical Treatment Options for Non-Cirrhotic Portal Hypertension. CURRENT HEPATOLOGY REPORTS 2023; 22:269-275. [DOI: 10.1007/s11901-023-00617-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 09/08/2023] [Indexed: 01/04/2025]
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20
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Giuli L, Pallozzi M, Venturini G, Gasbarrini A, Ponziani FR, Santopaolo F. Molecular Mechanisms Underlying Vascular Liver Diseases: Focus on Thrombosis. Int J Mol Sci 2023; 24:12754. [PMID: 37628933 PMCID: PMC10454315 DOI: 10.3390/ijms241612754] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2023] [Revised: 08/07/2023] [Accepted: 08/11/2023] [Indexed: 08/27/2023] Open
Abstract
Vascular liver disorders (VLDs) comprise a wide spectrum of clinical-pathological entities that primarily affect the hepatic vascular system of both cirrhotic and non-cirrhotic patients. VLDs more frequently involve the portal and the hepatic veins, as well as liver sinusoids, resulting in an imbalance of liver homeostasis with serious consequences, such as the development of portal hypertension and liver fibrosis. Surprisingly, many VLDs are characterized by a prothrombotic phenotype. The molecular mechanisms that cause thrombosis in VLD are only partially explained by the alteration in the Virchow's triad (hypercoagulability, blood stasis, and endothelial damage) and nowadays their pathogenesis is incompletely described and understood. Studies about this topic have been hampered by the low incidence of VLDs in the general population and by the absence of suitable animal models. Recently, the role of coagulation imbalance in liver disease has been postulated as one of the main mechanisms linked to fibrogenesis, so a novel interest in vascular alterations of the liver has been renewed. This review provides a detailed analysis of the current knowledge of molecular mechanisms of VLD. We also focus on the promising role of anticoagulation as a strategy to prevent liver complications and to improve the outcome of these patients.
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Affiliation(s)
- Lucia Giuli
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Maria Pallozzi
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Giulia Venturini
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Antonio Gasbarrini
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
- Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
| | - Francesca Romana Ponziani
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
| | - Francesco Santopaolo
- Hepatology Unit, CEMAD Centro Malattie Dell’Apparato Digerente, Medicina Interna e Gastroenterologia, Fondazione Policlinico Universitario Gemelli IRCCS, 00168 Rome, Italy; (L.G.); (M.P.); (G.V.); (F.R.P.); (F.S.)
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21
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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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22
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Krishnan A, Schneider CV, Schattenberg JM, Alqahtani SA. Risk of severe disease and mortality of COVID-19 in patients with Budd-Chiari syndrome: A population-based matched cohort study. Liver Int 2023; 43:1141-1144. [PMID: 36825357 DOI: 10.1111/liv.15553] [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: 10/18/2022] [Revised: 02/15/2023] [Accepted: 02/17/2023] [Indexed: 02/25/2023]
Abstract
BACKGROUND & AIMS Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract. It is unknown whether patients with BCS represent a high risk for severe disease and mortality from coronavirus disease 2019 (COVID-19). Thus, we aimed to assess hospitalization rates, severe disease, all-cause mortality, intensive care unit (ICU) requirement and acute kidney injury (AKI) from COVID-19 diagnoses. METHODS & RESULTS We identified 467 patients with BCS with COVID-19, 96 427 non-chronic liver disease (CLD) and 9652 non-BCS CLD. The BCS and non-CLD cohorts (n = 467 each) and BCS and non-BCS CLD (n = 440 each) were well balanced after propensity matching. When compared to the non-CLD cohort, the BCS group had a higher risk of all-cause mortality (5.1% vs. 2.4%, HR 2.18; 95% CI, 1.08-4.40), severe disease (6.0% vs. 2.4%, HR 2.20; 95% CI, 1.09-4.43), hospitalization (24.6% vs. 13.1%, HR 1.77; 95% CI, 1.30-2.42) and AKI (7.9% vs. 2.8%, HR 2.57; 95% CI, 1.37-4.85), but no significant differences in ICU requirements (2.4% vs. 2.1%, HR 0.75; 95% CI, 0.27-2.08) at 60-days time points. When compared to the non-BCS CLD cohort, the BCS group had a higher risk of all-cause mortality (3.6% vs. 2.5%, HR 3.94; 95% CI, 1.31-11.79), hospitalization (29.8% vs. 21.6%, HR 1.43; 95% CI, 1.09-1.86), but differences in ICU requirements (HR 0.90 (0.38-2.12)), AKI (HR 1.41 (0.86-2.30)) or severe disease (HR 1.92 (0.99-3.71)) did not reach statistical significance at 60-day follow up. CONCLUSION In conclusion, COVID-19 infection in patients with BCS is associated with poor outcomes. Patients with BCS infected with COVID-19 carry a significantly higher risk of hospitalization and all-cause mortality and a possible effect on severe disease and AKI compared with COVID-19 patients without CLD or with non-BCS-CLD.
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Affiliation(s)
- Arunkumar Krishnan
- Section of Gastroenterology and Hepatology, West Virginia University School of Medicine, Morgantown, West Virginia, USA
| | - Carolin V Schneider
- Department of Medicine III, Gastroenterology, Metabolic diseases and Intensive Care, University Hospital RWTH Aachen, Aachen, Germany
| | - Jörn M Schattenberg
- Metabolic Liver Research Program, Department of Medicine I, University Medical Center Mainz, Mainz, Germany
| | - Saleh A Alqahtani
- Liver Transplant Center, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
- Division of Gastroenterology and Hepatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
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23
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Nadinskaia MY, Kodzoeva KB, Gulyaeva KA, Khen MDE, Koroleva DI, Privalov MA, Tekaeva AK, Fedorov VR, Prokofev SG. Risk Factors of Portal Vein Thrombosis in Patients with Different Child-Pugh Classes Liver Cirrhosis. RUSSIAN JOURNAL OF GASTROENTEROLOGY, HEPATOLOGY, COLOPROCTOLOGY 2023; 33:45-59. [DOI: 10.22416/1382-4376-2023-33-2-45-59] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
Abstract
Aim: to evaluate the frequency of portal vein thrombosis (PVT) and build predictive models of the development of PVT for patients with liver cirrhosis (LC) of A and B/C classes by Child-Pugh.Materials and methods. Research design is a case-control. The Case group included 130 patients with newly diagnosed PVT not caused by invasive hepatocellular carcinoma (HCC); 29 patients were assigned to class A, 101 patients were assigned to class B/C. From the database of cirrhotic patients without PVT 60 Controls for class A and 205 for B/C were selected using sratified randomization by sex, age and etiology of cirrhosis. The Mann-Whitney U-test and Pearson's chi-squared test were used to compare the groups. Odds ratios (OR) and 95 % confidence intervals (95 % CI) were calculated. Logistic regression models are constructed with the separation of the sample into training and test (0.7; 0.3). The operational characteristics of the models were calculated on the test sample; ROC analysis was carried out, the area under the ROC curve (AUC) was calculated.Results. The overall frequency of PVT was 4.1 % (95 % CI 2.7-5.8 %) in class A and 10.4 % (95 % CI 8.5-12.5 %) class B/C. Patients with class A and B/C PVT differed from the corresponding controls by more severe portal hypertension: the frequency of bleeding / number of interventions on varices compared with the control were 41/45 % vs. 7/8 % (p < 0.001) for class A and 25.7/30.7 % vs. 16.1/16.1 % (p < 0.05) for class B/C, ascites frequency was 24 % vs. 8 % (p < 0.05) for class A and 89.1 % vs. 68.3 % (p < 0.001) for class B/C. The cutoff by the portal vein diameter was the same for both classes — 13.4 mm; the spleen length was similar and amounted 17.5 mm for class A, 17.1 mm for class B/C. Patients with PVT differed from the corresponding controls by neutrophil-to-lymphocyte ratio: class A 2.33 (1.82; 3.61) vs. 1.76 (1.37; 2.20), p < 0.01, class B/C 2.49 (1.93; 3.34) vs. 2.15 (1.49; 3.26), p < 0.05. Patients of class B/C had a higher incidence of newly diagnosed malignant tumors - 23.8% (primarily HCC that does not invade the portal vein), compared with control and cases of class A - 6.3 % and 3 % (p < 0.05), respectively. The best model for class A included variceal bleeding, ascites, portal vein diameter, absolute number of neutrophils, for class B — ascites, spleen length, portal vein diameter, malignant tumors / local factors; sensitivity, specificity, accuracy and AUC were 79.3 %, 90 %, 86.5 %, 0.897 and 73.3 %, 68.3 %, 69.9 %, 0.789, respectively.Conclusion. Independently of the Child-Pugh class of LC, the main risk factor for PVT is severe portal hypertension.
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Affiliation(s)
- M. Yu. Nadinskaia
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - Kh. B. Kodzoeva
- I.M. Sechenov First Moscow State Medical University (Sechenov University); V.I. Shumakov National Medical Research Center of Transplantology and Artificial Organs
| | - K. A. Gulyaeva
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - M.-D. E. Khen
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - D. I. Koroleva
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - M. A. Privalov
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - A. Kh. Tekaeva
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - V. R. Fedorov
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
| | - S. G. Prokofev
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
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24
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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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25
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Elkrief L, Payancé A, Plessier A, d’Alteroche L, Ronot M, Paradis V, Valla D, Rautou PE. Management of splanchnic vein thrombosis. JHEP Rep 2023; 5:100667. [PMID: 36941824 PMCID: PMC10023986 DOI: 10.1016/j.jhepr.2022.100667] [Citation(s) in RCA: 21] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2022] [Revised: 12/11/2022] [Accepted: 12/23/2022] [Indexed: 01/04/2023] Open
Abstract
The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
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Key Words
- BCS, Budd-Chiari syndrome
- CALR, calreticulin
- Cavernoma
- DOACs, direct-acting oral anticoagulants
- Direct oral anticoagulants
- EHPVO, extrahepatic portal vein obstruction
- GFR, glomerular filtration rate
- JAK2, Janus kinase 2
- LMWH, low-molecular-weight heparin
- MPN, myeloproliferative neoplasm
- MTHFR, methylene-tetrahydrofolate reductase
- PNH, paroxysmal nocturnal hemoglobinuria
- PVT, portal vein thrombosis
- Portal biliopathy
- Portal vein recanalisation
- SVT, splanchnic vein thrombosis
- TIPS, transjugular intrahepatic portosystemic shunt
- VKAs, vitamin K antagonists
- Vascular liver diseases
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Affiliation(s)
- Laure Elkrief
- Service d’Hépato-Gastroentérologie CHU de Tours, France
| | - Audrey Payancé
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Aurélie Plessier
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | | | - Maxime Ronot
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service de radiologie, Hôpital Beaujon APHP.Nord, Clichy, France
| | - Valérie Paradis
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d’anatomie et cytologie pathologique, Hôpital Beaujon APHP.Nord, Clichy, France
| | - Dominique Valla
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Pierre-Emmanuel Rautou
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
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26
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Semmler G, Reiberger T, Scheiner B. Reply. Clin Gastroenterol Hepatol 2023; 21:1117-1119. [PMID: 35842122 DOI: 10.1016/j.cgh.2022.06.014] [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: 06/07/2022] [Accepted: 06/08/2022] [Indexed: 02/07/2023]
Affiliation(s)
- Georg Semmler
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network for Rare Hepatological Diseases, Medical University of Vienna, Vienna, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network for Rare Hepatological Diseases, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network for Rare Hepatological Diseases, Medical University of Vienna, Vienna, Austria
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27
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Kiladjian JJ, Cassinat B. Myeloproliferative neoplasms and splanchnic vein thrombosis: Contemporary diagnostic and therapeutic strategies. Am J Hematol 2023; 98:794-800. [PMID: 36869873 DOI: 10.1002/ajh.26896] [Citation(s) in RCA: 12] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2023] [Revised: 02/15/2023] [Accepted: 02/20/2023] [Indexed: 03/05/2023]
Abstract
Myeloproliferative neoplasms (MPNs) are the most common etiologies of primary splanchnic vein thrombosis, present in almost forty percent of patients with Budd-Chiari syndrome or portal vein thrombosis. Diagnosis of MPNs can be difficult in these patients because key characteristics, such as elevated blood cell counts and splenomegaly, are confounded by portal hypertension or bleeding complications. In recent years, diagnostic tools have improved to provide more accurate diagnosis and classification of MPNs. Although bone marrow biopsy findings remain a major diagnostic criterion, molecular markers are playing an increasing role not only in diagnosis but also in better estimating prognosis. Therefore, though screening for JAK2V617F mutation should be the starting point of the diagnostic workup performed in all patients with splanchnic vein thrombosis, a multidisciplinary approach is needed to accurately diagnose the subtype of myeloproliferative neoplasm, recommend the useful additional tests (bone marrow biopsy, search for an additional mutation using targeted next-generation sequencing), and suggest the best treatment strategy. Indeed, providing a specific expert care pathway for patients with splanchnic vein thrombosis and underlying myeloproliferative neoplasm is crucial to determine the optimal management to reduce the risk of both hematological and hepatic complications.
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Affiliation(s)
- Jean-Jacques Kiladjian
- Centre d'Investigations Cliniques, Université Paris Cité, AP-HP, Hôpital Saint-Louis, Paris, France.,INSERM UMR 1131, Institut de Recherche Saint-Louis, Paris, France
| | - Bruno Cassinat
- INSERM UMR 1131, Institut de Recherche Saint-Louis, Paris, France.,Laboratoire de Biologie Cellulaire, AP-HP, Hôpital Saint-Louis, Paris, France
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28
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Leoni FG, Magnano San Lio P, De Molo C, Bakken S, Ferronato M, Dietrich CF, Serra C. Budd-Chiari syndrome (BCS): a challenging diagnosis not to be overlooked-single center report and pictorial essay. J Ultrasound 2023; 26:249-254. [PMID: 36180766 PMCID: PMC10063764 DOI: 10.1007/s40477-022-00723-x] [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: 04/30/2022] [Accepted: 08/22/2022] [Indexed: 10/14/2022] Open
Abstract
Budd-Chiari syndrome (BCS) is a rare disease with a variable clinical presentation and often late diagnosis. Doppler ultrasonography (DUS) permits to determine the site of the obstructed venous tracts, the thrombotic or non-thrombotic nature of the obstruction with its morphologic features and the flow-pattern alterations. Other non-specific findings, which are seen in most of the other liver diseases, include ascites, hepatosplenomegaly and caudate hypertrophy. The aim of this study is to show our experience in BCS reporting retrospectively 15 cases referred to our hepatology center between 2017 and 2021. Four selected cases depict the extreme heterogeneous behaviour of BCS and highlight the importance of DUS as a diagnostic tool when there is a clinical suspicion. In patients, mainly young, who present with ascites and abdominal pain, BCS has to be considered and DUS is the first imaging technique to be performed to rule it out.
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Affiliation(s)
- F. G. Leoni
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - P. Magnano San Lio
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - C. De Molo
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - S. Bakken
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - M. Ferronato
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - C. F. Dietrich
- University Clinic for Visceral Surgery and Medicine, INSELSPITAL, University Hospital of Bern, Bern, Switzerland
| | - C. Serra
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
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29
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Stevens-Haas C, Maniar V, Dietrich P, Langenstroer P, Joyce L, White S, Kilari D, Hong J, Johnson S. Management of Acute Liver Dysfunction Due to Budd-Chiari Syndrome in the Setting of Tumor Thrombus and Advanced Renal Cell Carcinoma. Urology 2023; 173:32-33. [PMID: 36535364 DOI: 10.1016/j.urology.2022.12.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2022] [Revised: 11/26/2022] [Accepted: 12/04/2022] [Indexed: 12/23/2022]
Affiliation(s)
- Claire Stevens-Haas
- Department of Urology, Froedtert and the Medical College of Wisconsin, Froedtert Hospital. Milwaukee, WI.
| | - Viraj Maniar
- Department of Urology, Froedtert and the Medical College of Wisconsin, Froedtert Hospital. Milwaukee, WI
| | - Peter Dietrich
- Department of Urology, Froedtert and the Medical College of Wisconsin, Froedtert Hospital. Milwaukee, WI
| | - Peter Langenstroer
- Department of Urology, Froedtert and the Medical College of Wisconsin, Froedtert Hospital. Milwaukee, WI
| | - Lyle Joyce
- Department of Cardiothoracic Surgery, Froedtert and the Medical College of Wisconsin, Medical College of Wisconsin Hub for Collaborative Medicine, Milwaukee, WI
| | - Sarah White
- Department of Vascular & Interventional Radiology, Froedtert and the Medical College of Wisconsin, Milwaukee, WI
| | - Deepak Kilari
- Department of Hematology and Oncology, Froedtert and the Medical College of Wisconsin, Milwaukee, WI
| | - Johnny Hong
- Department of Transplant Surgery, Froedtert and the Medical College of Wisconsin, Medical College of Wisconsin Transplant Surgery, Milwaukee, WI
| | - Scott Johnson
- Department of Urology, Froedtert and the Medical College of Wisconsin, Froedtert Hospital. Milwaukee, WI
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30
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Multiple hemangiomas (hepatic small vessel neoplasia) in the liver with Budd-Chiari syndrome. Virchows Arch 2023:10.1007/s00428-023-03505-w. [PMID: 36757499 DOI: 10.1007/s00428-023-03505-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2022] [Revised: 01/26/2023] [Accepted: 01/28/2023] [Indexed: 02/10/2023]
Abstract
Hepatic small vessel neoplasia (HSVN) is a recently recognized hemangioma of the liver with uncertain malignant potential. Almost all the patients are asymptomatic. Budd-Chiari syndrome (BCS) is a rare disorder characterized by noncardiogenic hepatic venous outflow obstruction. Benign hepatocellular nodules have been acknowledged for a long time in the liver with the chronic BCS. However, there has been no case report of BCS associated with HSVN. The patient was diagnosed with BCS 13 years ago. The imaging test initially displayed multiple hepatic nodules that were suspected of benign hepatocellular nodules. They gradually increased in size and number in the course of the disease. At an autopsy, these nodules were confirmed to be multifocal HSVN. The tumor of the present case could not be proved to have GNAQ and GNQ14 mutations. We describe the case focusing on the chronological imaging changes and discuss on the relationship between BCS and HSVN.
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31
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Luo X, Nicoară-Farcău O, Magaz M, Betancourt F, Soy G, Baiges A, Turon F, Hernández-Gea V, García-Pagán JC. Obstruction of the liver circulation. CARDIO-HEPATOLOGY 2023:65-92. [DOI: 10.1016/b978-0-12-817394-7.00004-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Perez Ruiz de Garibay A, Kortgen A, Leonhardt J, Zipprich A, Bauer M. Critical care hepatology: definitions, incidence, prognosis and role of liver failure in critically ill patients. Crit Care 2022; 26:289. [PMID: 36163253 PMCID: PMC9511746 DOI: 10.1186/s13054-022-04163-1] [Citation(s) in RCA: 39] [Impact Index Per Article: 13.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2022] [Accepted: 09/10/2022] [Indexed: 01/11/2023] Open
Abstract
AbstractOrgan dysfunction or overt failure is a commonplace event in the critically ill affecting up to 70% of patients during their stay in the ICU. The outcome depends on the resolution of impaired organ function, while a domino-like deterioration of organs other than the primarily affected ones paves the way for increased mortality. “Acute Liver Failure” was defined in the 1970s as a rare and potentially reversible severe liver injury in the absence of prior liver disease with hepatic encephalopathy occurring within 8 weeks. Dysfunction of the liver in general reflects a critical event in “Multiple Organ Dysfunction Syndrome” due to immunologic, regulatory and metabolic functions of liver parenchymal and non-parenchymal cells. Dysregulation of the inflammatory response, persistent microcirculatory (hypoxic) impairment or drug-induced liver injury are leading problems that result in “secondary liver failure,” i.e., acquired liver injury without underlying liver disease or deterioration of preexisting (chronic) liver disease (“Acute-on-Chronic Liver Failure”). Conventional laboratory markers, such as transaminases or bilirubin, are limited to provide insight into the complex facets of metabolic and immunologic liver dysfunction. Furthermore, inhomogeneous definitions of these entities lead to widely ranging estimates of incidence. In the present work, we review the different definitions to improve the understanding of liver dysfunction as a perpetrator (and therapeutic target) of multiple organ dysfunction syndrome in critical care.
Graphic Abstract
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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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Impact of SARS-CoV-2 Pandemic on Vascular Liver Diseases. Clin Gastroenterol Hepatol 2022; 20:1525-1533.e5. [PMID: 34968728 PMCID: PMC8710430 DOI: 10.1016/j.cgh.2021.12.032] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/13/2021] [Revised: 12/14/2021] [Accepted: 12/16/2021] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Vascular liver diseases (VLDs) are represented mainly by portosinusoidal vascular disease (PSVD), noncirrhotic splanchnic vein thrombosis (SVT), and Budd Chiari syndrome (BCS). It is unknown whether patients with VLDs constitute a high-risk population for complications and greater coronavirus disease 2019 (COVID-19)-related mortality from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Our objective was to assess the prevalence and severity of SARS-CoV-2 infection among patients with VLDs, as well as to assess its impact on hepatic decompensation and survival. METHODS This is an observational international study analyzing the prevalence and severity of SARS-CoV-2 infection in VLDs between March 2020 and March 2021, compared with the general population (GP). Patients from Spain (5 centers; n = 493) and France (1 center; n = 475) were included. RESULTS Nine hundred sixty-eight patients were included: 274 with PSVD, 539 with SVT, and 155 with BCS. Among them, 138 (14%) were infected with SARS-CoV-2: 53 with PSVD, 77 with SVT, and 8 with BCS. The prevalence of SARS-CoV-2 infection in patients with PSVD (19%) and SVT (14%) was significantly higher than in the GP (6.5%; P < .05), whereas it was very similar in patients with BCS (5%). In terms of infection severity, patients with VLDs also presented a higher need of hospital admission (14% vs 7.3%; P < .01), intensive care unit admission (2% vs 0.7%; P < .01), and mortality (4% vs 1.5%; P < .05) than the GP. Previous history of ascites (50% vs 8%; P < .05) and post-COVID-19 hepatic decompensation (50% vs 4%; P < .05) were associated with COVID-19 mortality. CONCLUSIONS Patients with PSVD and SVT could be at higher risk of infection by SARS-CoV-2 and at higher risk of severe COVID-19 disease.
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Jeyanesan D, Balachandrakumar VK, Hogan B. Guideline review: transjugular intrahepatic portosystemic stent-shunt in the management of portal hypertension-a BSG guideline. Frontline Gastroenterol 2022; 13:531-534. [PMID: 36250168 PMCID: PMC9555133 DOI: 10.1136/flgastro-2022-102151] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2022] [Accepted: 05/03/2022] [Indexed: 02/04/2023] Open
Abstract
The decision-making around transjugular intrahepatic portosystemic shunt (TIPSS) placement in the management of patients with chronic liver disease and portal hypertension (PH) is a regular challenge for hepatologists. In the UK, access has improved, with more than 35 hospitals now offering this service. However, its role in acute variceal bleeding, refractory ascites and other complications of PH continues to be redefined and expanded. In particular, the role of pre-emptive TIPSS has become more established and requires re-evaluation of pathways to enable equitable access for patients. Here, we summarise the key recommendations from the recently published British Society of Gastroenterology guidelines and expand on the challenges posed.
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Affiliation(s)
- Dhaarica Jeyanesan
- Institute of Liver Studies, King's College Hospital Liver Unit, London, UK
| | | | - Brian Hogan
- Institute of Liver Studies, King's College Hospital Liver Unit, London, UK
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Artru F, Vietti-Violi N, Sempoux C, Vieira Barbosa J, Becce F, Sah N, Marot A, Deltenre P, Moschouri E, Fraga M, Hocquelet A, Duran R, Moradpour D, Rautou PE, Denys A. Portal vein recanalisation alone to treat severe portal hypertension in non-cirrhotic patients with chronic extrahepatic portal vein obstruction. JHEP REPORTS : INNOVATION IN HEPATOLOGY 2022; 4:100511. [PMID: 35801087 PMCID: PMC9253474 DOI: 10.1016/j.jhepr.2022.100511] [Citation(s) in RCA: 17] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/14/2021] [Revised: 04/26/2022] [Accepted: 05/04/2022] [Indexed: 11/16/2022]
Abstract
Background & Aims We aimed to evaluate long-term outcome of patients with chronic non-cirrhotic extrahepatic portal vein obstruction (CNC-EHPVO) who underwent portal vein recanalisation (PVR) without transjugular intrahepatic portosystemic shunt (TIPS) insertion and to determine factors predicting PVR failure and stent occlusion. Methods This retrospective monocentric study included all patients who underwent PVR without TIPS insertion in the context of CNC-EHPVO between the years 2000 and 2019. Primary patency was defined by the absence of a complete stent occlusion on follow-up imaging. Results A total of 31 patients underwent PVR with a median follow-up of 52 months (24–82 months). Indications were gastrointestinal bleeding (n = 13), abdominal pain attributed to CNC-EHPVO (n = 7), prior to abdominal surgery (n = 4), and others (n = 7). Technical success was obtained in 27 patients. PVR failure was associated with extension within the intrahepatic portal veins (p = 0.005) and recanalisation for abdominal pain (p = 0.02). Adverse events occurred in 6 patients with no mortality. Anticoagulation was administered in 21 patients after technical success of PVR. In patients with technical success, 5-year primary patency was 73% and was associated with improved muscle mass (p = 0.007) and decreased spleen volume (p = 0.01) at 1 year. Furthermore, 21 (78%) patients with PVR technical success were free of portal hypertension complication at 5 years. Conclusions PVR without TIPS insertion was feasible and safe in selected patients with CNC-EHPVO and portal hypertension with past or expected complications. Primary patency at 5 years was obtained in 3 of 4 patients with technical success of PVR and was associated with a control of complications of CNC-EHPVO. PVR was associated with improvement of sarcopenia and decreased spleen volume at 1 year. Lay summary Patients with chronic obstruction of the portal vein and without cirrhosis or malignancy can develop complications related to the high pressure in the venous system. The present study reports long-term favourable outcome of patients in whom the obstruction was treated with stents.
CNC-EHPVO with severe portal hypertension can be treated with PVR alone. After technical success of PVR, the 5-year primary patency is above 70%. After technical success of PVR, 78% of patients had complete resolution of symptoms. Intrahepatic extension of obstruction is associated with failure of PVR. Indication of PVR for abdominal pain is associated with poorer outcome.
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Affiliation(s)
- Florent Artru
- Service of Gastroenterology and Hepatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
- Institute of Liver Studies, King’s College Hospital, London, UK
| | - Naik Vietti-Violi
- Service of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Christine Sempoux
- Service of Clinical Pathology, Institute of Pathology Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Joana Vieira Barbosa
- Service of Gastroenterology and Hepatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Fabio Becce
- Service of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Nelly Sah
- Service of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Astrid Marot
- Department of Gastroenterology and Hepatology, CHU UCL Namur, Université Catholique de Louvain, Yvoir, Belgium
| | - Pierre Deltenre
- Department of Gastroenterology, Hepatopancreatology and Digestive Oncology, CUB Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium
- Department of Gastroenterology and Hepatology, Clinique St Luc, Bouge, Belgium
| | - Eleni Moschouri
- Service of Gastroenterology and Hepatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Montserrat Fraga
- Service of Gastroenterology and Hepatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Arnaud Hocquelet
- Service of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Rafael Duran
- Service of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Darius Moradpour
- Service of Gastroenterology and Hepatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Pierre-Emmanuel Rautou
- Université de Paris, AP-HP, Hôpital Beaujon, Service d’Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l’inflammation, Inserm, UMR 1149, Paris, France
- Service d’Hépatologie, Hôpital Beaujon, 100 boulevard du General Leclerc, 92100 Clichy, France. Tel.: +331-40-87-52-83; Fax: +331-40-87-44-35.
| | - Alban Denys
- Service of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
- Corresponding authors. Address: Service de radiodiagnostic et de radiologie interventionnelle, BH 10-119, Bugnon 46, CH-1011 Lausanne, Switzerland. Tel.: +41-21-314-97687; Fax: +41-21-314-4554.
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Pérez-González A, Argibay A, Lorenzo-Castro R, Martín-Granizo I, Rivera-Gallego A. Budd-Chiari syndrome: epidemiological and clinical characteristics of a case series in Northwest Spain. EGYPTIAN LIVER JOURNAL 2022. [DOI: 10.1186/s43066-022-00192-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Introduction
Budd-Chiari syndrome (BCS) is a rare vascular disease of the liver, characterised by occlusion of the venous outflow tract. Cancer, pyogenic liver infection, and prothrombotic haematological conditions are the most frequent causes of BCS. The treatment and prognosis of the disease are closely related to the underlying cause.
Methods
This is a retrospective case-series study performed in Spain, in a health area of around 523,000 inhabitants. Cases were identified in the discharge database of the hospital between 2000 and 2020. Epidemiological, clinical, therapeutic, and prognosis data were obtained from the patient medical records.
Results
A total of 15 cases were identified. Most of them were male patients (n = 8, 53.3%) with a median age of 52 years. The most common cause of BCS was cancer (n = 6, 40.0%) followed by liver abscesses (n = 4, 26.7%). The most frequent clinical course was subacute hepatitis (n = 8, 53.3%); 12 of the 15 patients (80%) received anticoagulant treatment, and interventional treatment was carried out in 4 patients (26.7%). Seven patients died within 6 months (46.7%), 6 of them due to progression of the underlying disease, most often cancer; 2 patients (13.3%) developed liver cirrhosis after BCS.
Discussion
The incidence of BCS was low but higher than in other European studies. In addition, this current research showed a different aetiology than previously described. The mortality rate was extremely high and closely related to the underlying disease. The involvement of classic prothrombotic haematological factors was less common than previously described.
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The Pathophysiology of Portal Vein Thrombosis in Cirrhosis: Getting Deeper into Virchow's Triad. J Clin Med 2022; 11:jcm11030800. [PMID: 35160251 PMCID: PMC8837039 DOI: 10.3390/jcm11030800] [Citation(s) in RCA: 28] [Impact Index Per Article: 9.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2021] [Revised: 01/21/2022] [Accepted: 01/28/2022] [Indexed: 02/07/2023] Open
Abstract
Portal vein thrombosis (PVT) is a common complication among patients with cirrhosis. However, its pathophysiology is not well established and there are currently very few predictive factors, none of which are actually useful, from a clinical perspective. The contribution of each of the vertices of Virchow’s triad, e.g., blood hypercoagulability, blood flow, and portal vein endothelial damage in the development of PVT is not clear. In this review, we aim to recapitulate the latest studies on the field of PVT development in order to understand its mechanisms and discuss some of the future directions in the study of this important complication of cirrhosis.
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Meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults (with video). Hepatobiliary Pancreat Dis Int 2022; 21:25-32. [PMID: 34426078 DOI: 10.1016/j.hbpd.2021.08.003] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2021] [Accepted: 08/02/2021] [Indexed: 02/07/2023]
Abstract
BACKGROUND Extrahepatic portal vein obstruction (EHPVO) results in severe portal hypertension (PHT) leading to severely compromised quality of life. Often, pharmacological and endoscopic management is unable to solve this problem. Restoring hepatic portal flow using meso-Rex bypass (MRB) may solve it. This procedure, uncommon in adult patients, is considered the treatment of choice for EHPVO in children. METHODS From 1997 to 2018, 8 male and 6 female adults, with a median age of 51 years (range 22-66) underwent MRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels, Belgium. Symptoms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings, sepsis due to portal biliopathy, and/or severe abdominal discomfort. The surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal vein. RESULTS Median operative time was 500 min (range 300-730). Median follow-up duration was 22 months (range 2-169). One patient died due to hemorrhagic shock following percutaneous transluminal intervention for early graft thrombosis. Major morbidity, defined as Clavien-Dindo score ≥ III, was 35.7% (5/14). Shunt patency at last follow-up was 64.3% (9/14): 85.7% (6/7) of pure venous grafts and only 42.9% (3/7) of prosthetic graft. Symptom relief was achieved in 85.7% (12/14) who became asymptomatic after MRB. CONCLUSIONS Adult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening complications. In such patients, MRB represents the only and last resort to restore physiological portal vein flow. Although successful in a majority of patients, this procedure is associated with major morbidity and mortality and should be done in tertiary centers experienced with vascular liver surgery to get the best results.
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De Broucker C, Plessier A, Ollivier-Hourmand I, Dharancy S, Bureau C, Cervoni JP, Sogni P, Goria O, Corcos O, Sartoris R, Ronot M, Vilgrain V, de Raucourt E, Zekrini K, Davy H, Durand F, Payancé A, Fidouh-Houhou N, Yazdanpanah Y, Valla D, Rautou PE. Multicenter study on recent portal venous system thrombosis associated with cytomegalovirus disease. J Hepatol 2022; 76:115-122. [PMID: 34563580 DOI: 10.1016/j.jhep.2021.09.011] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2021] [Revised: 08/23/2021] [Accepted: 09/09/2021] [Indexed: 12/15/2022]
Abstract
BACKGROUND & AIMS Recent non-malignant non-cirrhotic portal venous system thrombosis (PVT) is a rare condition. Among risk factors for PVT, cytomegalovirus (CMV) disease is usually listed based on a small number of reported cases. The aim of this study was to determine the characteristics and outcomes of PVT associated with CMV disease. METHODS We conducted a French multicenter retrospective study comparing patients with recent PVT and CMV disease ("CMV positive"; n = 23) to patients with recent PVT for whom CMV testing was negative ("CMV negative"; n = 53) or unavailable ("CMV unknown"; n = 297). RESULTS Compared to patients from the "CMV negative" and "CMV unknown" groups, patients from the "CMV positive" group were younger, more frequently had fever, and had higher heart rate, lymphocyte count and serum ALT levels (p ≤0.01 for all). The prevalence of immunosuppression did not differ between the 3 groups (4%, 4% and 6%, respectively). Extension of PVT was similar between the 3 groups. Thirteen out of 23 "CMV positive" patients had another risk factor for thrombosis. Besides CMV disease, the number of risk factors for thrombosis was similar between the 3 groups. Heterozygosity for the prothrombin G20210A gene variant was more frequent in "CMV positive" patients (22%) than in the "CMV negative" (4%, p = 0.01) and "CMV unknown" (8%, p = 0.03) groups. Recanalization rate was not influenced by CMV status. CONCLUSIONS In patients with recent PVT, features of mononucleosis syndrome should raise suspicion of CMV disease. CMV disease does not influence thrombosis extension nor recanalization. More than half of "CMV positive" patients have another risk factor for thrombosis, with a particular link to the prothrombin G20210A gene variant. LAY SUMMARY Patients with cytomegalovirus (CMV)-associated portal venous system thrombosis have similar thrombosis extension and evolution as patients without CMV disease. However, patients with CMV-associated portal venous system thrombosis more frequently have the prothrombin G20210A gene variant, suggesting that these entities act synergistically to promote thrombosis.
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Affiliation(s)
- Chloé De Broucker
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Aurélie Plessier
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Isabelle Ollivier-Hourmand
- Service d'Hépato-Gastroentérologie et Nutrition, Centre Hospitalo-Universitaire Côte de Nacre, Caen, France
| | - Sébastien Dharancy
- Service d'Hépatologie et de Gastroentérologie, Hôpital Huriez, Centre Hospitalo-Universitaire de Lille, Lille, France
| | - Christophe Bureau
- Service d'Hépatologie, Centre Hospitalo-Universitaire de Toulouse, Université Paul Sabatier Toulouse 3, Toulouse, France
| | - Jean-Paul Cervoni
- Service d'hépatologie et de soins intensifs digestifs, Centre Hospitalo-Universitaire Régional Jean-Minjoz, Besançon, France
| | - Philippe Sogni
- Université de Paris, APHP, Service d'Hépatologie, Hôpital Cochin, Paris, France
| | - Odile Goria
- Service d'Hépatologie et de Gastroentérologie, Hôpital Charles Nicolle, Centre Hospitalo-Universitaire de Rouen, Rouen, France
| | - Olivier Corcos
- Université de Paris, AP-HP, Hôpital Beaujon, Service de Gastroentérologie Assistance Nutritive, DMU DIGEST, Paris, France
| | - Riccardo Sartoris
- Service de radiologie, CHU Paris Nord-Val de Seine - Hôpital Beaujon, Clichy, France
| | - Maxime Ronot
- Service de radiologie, CHU Paris Nord-Val de Seine - Hôpital Beaujon, Clichy, France
| | - Valérie Vilgrain
- Service de radiologie, CHU Paris Nord-Val de Seine - Hôpital Beaujon, Clichy, France
| | - Emmanuelle de Raucourt
- Service d'hématologie biologique, CHU Paris Nord-Val de Seine - Hôpital Beaujon, Clichy, France
| | - Kamal Zekrini
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Hortense Davy
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - François Durand
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Audrey Payancé
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Nadira Fidouh-Houhou
- Université de Paris, Department of Virology Unit, APHP, Bichat-Claude Bernard University Hospital, Paris, France
| | - Yazdan Yazdanpanah
- Université de Paris, APHP, Bichat-Claude Bernard University Hospital, Department of Infectious and Tropical Diseases, IAME, Inserm, Umr 1137, Paris, France
| | - Dominique Valla
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Pierre-Emmanuel Rautou
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France.
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Mehta S, Malhotra S, Panwar A, Sibal A. Complicated pylephlebitis secondary to perforated appendicitis in an adolescent. J Indian Assoc Pediatr Surg 2022; 27:115-117. [PMID: 35261528 PMCID: PMC8853607 DOI: 10.4103/jiaps.jiaps_291_20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2020] [Revised: 11/22/2020] [Accepted: 01/09/2021] [Indexed: 11/04/2022] Open
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Hernández-Gea V, Baiges A, Turon F, Garcia-Pagan JC. Budd-Chiari Syndrome: Hepatic Venous Outflow Tract Obstruction. VASCULAR DISORDERS OF THE LIVER 2022:79-92. [DOI: 10.1007/978-3-030-82988-9_6] [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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Li J, Chen XM, Zhou CZ, Fang WW, Lv WF, Cheng DL. Novel roles of lipopolysaccharide and TLR4/NF-κB signaling pathway in inflammatory response to liver injury in Budd-Chiari syndrome. World J Gastrointest Surg 2021; 13:1448-1462. [PMID: 34950433 PMCID: PMC8649562 DOI: 10.4240/wjgs.v13.i11.1448] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2021] [Revised: 08/19/2021] [Accepted: 10/31/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. To date, the exact mechanism underlying hepatic injury derived from the hepatic venous outflow obstruction in BCS remains largely unknown.
AIM To assess the role of NF-κB-mediated inflammation in BCS-induced liver injury in humans and rats.
METHODS A total of 180 rats were randomly assigned into nine groups, including four BCS model groups (1, 3, 6 and 12 wk), four sham-operated groups (1, 3, 6 and 12 wk), and a control group. Lipopolysaccharide (LPS) levels in each group were detected by the Tachypleus Amebocyte Lysate assay. The mRNA and protein levels of TLR4, NF-κB, tumor necrosis factor (TNF)-α, interleukin (IL)-2 and interferon (IFN)-γ were quantified. In addition, 60 patients with BCS and 30 healthy controls were enrolled, and their blood samples were analyzed.
RESULTS Hepatic and plasma LPS levels were significantly increased in rats. The mRNA and protein expression levels of TLR4, NF-κB and inflammatory cytokines (TNF-α, IL-2 and IFN-γ) in liver tissues were significantly higher in the BCS model groups compared with the other two groups. In addition, the model groups (1, 3, 6 and 12 wk after BCS induction) showed significant differences in the levels of LPS, TLR4, NF-κB, TNF-α, IL-2 and IFN-γ. Notably, there was a significant correlation between the LPS concentrations and mRNA and protein levels of TLR4, NF-κB and inflammatory cytokines. Importantly, it was revealed that the levels of LPS, TLR4, NF-κB and inflammatory cytokines were significantly greater in chronic BCS patients than healthy controls and acute BCS patients.
CONCLUSION LPS level is markedly elevated in BCS, in turn activating the TLR4/NF-κB signaling pathway, leading to induction of inflammatory cytokines (TNF-α, IL-2 and IFN-γ) in response to BCS-induced liver injury.
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Affiliation(s)
- Jie Li
- Department of Interventional Radiology, The First Affiliated Hospital of University of Science and Technology of China, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Xiao-Ming Chen
- Department of Gastroenterology, The Second Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China
| | - Chun-Ze Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of University of Science and Technology of China, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Wei-Wei Fang
- Department of Radiology, The Third People’s Hospital of Hefei, Hefei 230022, Anhui Province, China
| | - Wei-Fu Lv
- Department of Interventional Radiology, The First Affiliated Hospital of University of Science and Technology of China, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - De-Lei Cheng
- Department of Interventional Radiology, The First Affiliated Hospital of University of Science and Technology of China, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
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Quan X, Tai Y, Wei B, Tong H, Wang Z, Yang Y, Wu H. Angioplasty With Stent Implantation for Portal Venous Stenosis Caused by Abdominal Tuberculosis: A Case Report and Literature Review. Front Med (Lausanne) 2021; 8:778672. [PMID: 34778328 PMCID: PMC8581033 DOI: 10.3389/fmed.2021.778672] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2021] [Accepted: 10/06/2021] [Indexed: 02/05/2023] Open
Abstract
Abdominal tuberculosis is one of common forms of extra-pulmonary tuberculosis. However, portal vein involvement leading to portal venous stenosis and portal hypertension is a rare complication in abdominal tuberculosis. Because of the non-specific presentations and insensitive response to anti-tuberculosis therapy of the lesions involving portal vein, it continues to be both a diagnostic and treatment challenge. We have reported a 22-year-old woman presented with massive ascites and pleural effusion, which was proved to be TB infection by pleural biopsy. After standard anti-tuberculosis therapy, her systemic symptoms completely resolved while ascites worsened with serum-ascites albumin gradient >11 g/L. Contrast-enhanced computed tomography and portal venography showed severe main portal vein stenosis from compression by multiple calcified hilar lymph nodes. Finally, the patient was diagnosed with portal venous stenosis due to lymphadenopathy after abdominal tuberculosis infection. Portal venous angioplasty by balloon dilation with stent implantation was performed and continued anti-tuberculosis therapy were administrated after discharge. The ascites resolved promptly with no recurrence occurred during the six-month follow-up. Refractory ascites due to portal venous stenosis is an uncommon vascular complication of abdominal tuberculosis. Portal venous angioplasty with stent placement could be a safe and effective treatment for irreversible vascular lesions after anti-tuberculosis therapy.
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Affiliation(s)
- Xin Quan
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Yang Tai
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China.,Laboratory of Gastroenterology and Hepatology, West China Hospital, Sichuan University, Chengdu, China
| | - Bo Wei
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Huan Tong
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Zhidong Wang
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Yuhang Yang
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Hao Wu
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
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Lu Q, Xu H, Zhou L, Zhang R, Li Z, Xu P, Bai T, Wang Z, Wu G, Ren J, Jiao D, Song Y, Zhu R, Li J, Wang W, Liang R, Li L, Ma X, Zu M, Sun Y. Alterations in Faecal Metagenomics and Serum Metabolomics Indicate Management Strategies for Patients With Budd-Chiari Syndrome. Front Cell Infect Microbiol 2021; 11:730091. [PMID: 34746022 PMCID: PMC8567795 DOI: 10.3389/fcimb.2021.730091] [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: 07/09/2021] [Accepted: 09/29/2021] [Indexed: 11/13/2022] Open
Abstract
We investigated the effects of gut microbiota and serum metabolite levels in patients with Budd-Chiari syndrome (B-CS) and their importance for guiding clinical management strategies. In total, 214 B-CS patients (93 untreated and 121 treated) and 41 healthy controls were enrolled. Gut microbiota and serum metabolome were analysed using shotgun metagenomics and liquid chromatography-mass spectrometry. The gut microbiota of the patients showed abundance of Campylobacter and low levels of Saccharomyces, Deinococcus, and Thiomonas (P < 0.05). Thirty metabolites, including taurocholate and (R)-3-hydroxybutyric acid, were identified in the patients (VIP > 1, P < 0.05 and FC > 1.2 or FC < 0.83). Random forest (RF) models showed that serum metabolome could effectively identify B-CS from healthy controls and RF-metabolomics exhibited perfect discrimination (AUC = 100%, 95% CI: 100% - 100%), which was significantly higher than that achieved by RF-metagenomics (AUC = 58.48%, 95% CI: 38.46% - 78.5%). Campylobacter concisus and taurocholate showed significant positive correlation in patients with clinical manifestations (P < 0.05). Actinobacteria levels were significantly higher in untreated patients than in treated patients (P < 0.05). Campylobacter and Veillonella levels were significantly higher in treated patients than in healthy controls (P < 0.05). We identified major alterations in the gut microbiota and serum metabolome of patients with B-CS. Faecal metagenomics- and serum metabolomics-guided management strategies are required for patients with B-CS.
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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
| | - Hao Xu
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, Xuzhou, China
| | - Lin Zhou
- Institute of Hepatobiliary and Pancreatic Diseases, Zhengzhou University, Zhengzhou, China
- Department of Digestive, 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
| | - Tao Bai
- 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
| | - 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
| | - 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
| | - 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
| | - 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
| | - Lin Li
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, Xuzhou, 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
| | - Maoheng Zu
- Department of Interventional Radiology, The Affiliated Hospital of Xuzhou Medical University, Xuzhou, 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
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Ju C, Li X, Gadani S, Kapoor B, Partovi S. Portal Vein Thrombosis: Diagnosis and Endovascular Management. ROFO-FORTSCHR RONTG 2021; 194:169-180. [PMID: 34649289 DOI: 10.1055/a-1642-0990] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Portal vein thrombosis (PVT) is a rare but severe entity that can cause clinically significant sequela such as worsening portal hypertension or mesenteric ischemia. Those cases refractory to medical management may be referred for endovascular intervention. Several technical considerations have been described in the literature, but a cohesive comparison of these multiple techniques is lacking. METHODS The purpose of this article is to review the diagnosis and endovascular management of PVT, including areas in which further research is warranted. RESULTS Cases of PVT can be readily diagnosed using ultrasound, computed tomography, or magnetic resonance imaging. Treatment often begins with systemic anticoagulation and endovascular interventions may be used in selected cases. Determining the optimal approach to accessing the portal venous system depends on the underlying disease and chronicity of the thrombus and the degree of occlusion. Once access to the portal venous system is established, catheter-directed therapy may be performed to achieve recanalization. CONCLUSION Despite the heterogeneity in patient presentation, cases of PVT can be readily diagnosed across several imaging modalities. Strategizing interventional approaches involves evaluation of the underlying disease and the chronicity of the thrombus. KEY POINTS · This review will enable interventionalists to establish a framework for treating portal vein thrombosis by identifying patient risk factors and thrombus characteristics that determine patient management.. · The unique risks and benefits for transhepatic, transsplenic, and transmesenteric approaches for establishing portal venous access will be discussed.. · Advantages and complications of thrombolysis, thrombectomy, and transjugular intrahepatic portosystemic shunt creation for treating portal vein thrombosis will be reviewed in detail based on our extensive institutional experience.. CITATION FORMAT · Ju C, Li X, Gadani S et al. Portal Vein Thrombosis: Diagnosis and Endovascular Management. Fortschr Röntgenstr 2021; DOI: 10.1055/a-1642-0990.
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Affiliation(s)
- Connie Ju
- Radiology, University of California Los Angeles Health System, Los Angeles, United States
| | - Xin Li
- Radiology, University of Pennsylvania Health System, Philadelphia, United States
| | - Sameer Gadani
- Interventional Radiology, Cleveland Clinic Foundation, Cleveland, United States
| | - Baljendra Kapoor
- Interventional Radiology, Cleveland Clinic Foundation, Cleveland, United States
| | - Sasan Partovi
- Interventional Radiology, Cleveland Clinic Foundation, Cleveland, United States
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Coe TM, Tanaka M, Bethea ED, D'Alessandro DA, Kimura S, Yeh H, Markmann JF. Liver transplantation with suprahepatic caval anastomosis including inferior vena cava stent. TRANSPLANTATION REPORTS 2021; 5. [PMID: 34485756 DOI: 10.1016/j.tpr.2020.100062] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
Abstract
Budd Chiari syndrome (BCS) results from hepatic outflow obstruction. Endovascular management to restore venous patency, including inferior vena cava (IVC) angioplasty with stenting, and transjugular intrahepatic shunt (TIPS) placement to decompress liver congestion, have become standard of care. Herein, we describe a patient with BCS requiring liver transplantation and the surgical technique of suprahepatic IVC anastomosis including thoracic extension of an IVC stent with a review of the relevant literature. A 29-year-old female with BCS due to polycythemia vera, who had been previously managed with TIPS and IVC stent placement, was taken for liver transplantation. Preoperative imaging confirmed stent extension into the thoracic IVC and the stent was unable to be removed intraoperatively. The thoracic IVC was clamped through the diaphragm at the level of the right atrium and the stent was left in place and incorporated within the suprahepatic anastomosis with good vascular outcome at one year follow up. Diligent preoperative preparation is essential with adequate imaging and cardiac surgical consultation in patients with malpositioned stents. Review of the literature shows four cases in which performing the suprahepatic anastomosis including an embedded stent is a viable alternative that allows for avoidance of a thoracotomy.
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Affiliation(s)
- Taylor M Coe
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
| | - Mari Tanaka
- Harvard Medical School, Boston, MA, United States.,Department of Radiology, Massachusetts General Hospital, Boston, MA, United States
| | - Emily D Bethea
- Harvard Medical School, Boston, MA, United States.,Division of Gastroenterology, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States
| | - David A D'Alessandro
- Harvard Medical School, Boston, MA, United States.,Division of Cardiac Surgery, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States
| | - Shoko Kimura
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
| | - Heidi Yeh
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
| | - James F Markmann
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
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49
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A Scoring Model to Predict In-Hospital Mortality in Patients With Budd-Chiari Syndrome. Am J Gastroenterol 2021; 116:1905-1912. [PMID: 33900212 DOI: 10.14309/ajg.0000000000001273] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2020] [Accepted: 03/18/2021] [Indexed: 12/11/2022]
Abstract
INTRODUCTION A model that can predict short-term mortality in patients with the Budd-Chiari syndrome (BCS) with a high degree of accuracy is currently lacking. The primary objective of our study was to develop an easy-to-use in-hospital mortality prediction model in patients with BCS using easily available clinical variables. METHODS Data were extracted from the National Inpatient Sample to identify all adult patients with a listed diagnosis of BCS from 2008 to 2017 using ICD-9 or ICD-10 codes. After identifying independent risk factors of in-hospital mortality, we developed a prediction model using logistic regression analysis. The model was built and validated in a training and a validation data set, respectively. Using the model, we risk stratified patients into low-, intermediate-, and high-risk groups. RESULTS Between 2008 and 2017, we identified a total of 5,306 (weighted sample size 26,110) discharge diagnosis of patients with BCS, with an overall in-hospital mortality of 7.14%. The independent risk factors that predicted mortality were age of 50 years or older, ascites, sepsis, acute respiratory failure, acute liver failure, hepatorenal syndrome, and cancers. The mortality prediction model that incorporated these risk factors had an area under the receiver operating characteristic curve of 0.87 (95% CI 0.85-0.95) for the training data and 0.89 (95% CI 0.86-0.92) for the validation data. Patients with low-, intermediate-, and high-risk scores had a predicted in-patient mortality of 4%, 30%, and 66%, respectively. DISCUSSION Using a national administrative database, we developed a reliable in-patient mortality prediction model with an excellent accuracy. The model was able to risk stratify patients into low-, intermediate-, and high-risk groups.
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50
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Semmler G, Pomej K, Bauer DJM, Balcar L, Simbrunner B, Binter T, Hartl L, Becker J, Pinter M, Quehenberger P, Trauner M, Mandorfer M, Lisman T, Reiberger T, Scheiner B. Safety of direct oral anticoagulants in patients with advanced liver disease. Liver Int 2021; 41:2159-2170. [PMID: 34152697 PMCID: PMC8456813 DOI: 10.1111/liv.14992] [Citation(s) in RCA: 42] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2021] [Revised: 05/28/2021] [Accepted: 06/18/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUND & AIMS While direct oral anticoagulants (DOACs) are increasingly used in patients with liver disease, safety data especially in advanced chronic liver disease (ACLD) are limited. METHODS Liver disease patients receiving DOAC treatment (ACLD: n = 104; vascular liver disease: n = 29) or vitamin K antagonists (VKA)/low-molecular-weight heparin (LMWH; ACLD: n = 45; vascular: n = 13) between January 2010 and September 2020 were retrospectively included. Invasive procedures and bleeding events were recorded. Calibrated anti-Xa peak levels and thrombomodulin-modified thrombin generation assays (TM-TGAs) were measured in a subgroup of 35/28 DOAC patients. RESULTS Among patients receiving DOAC, 55 (41.3%) had advanced liver dysfunction (Child-Pugh-stage [CPS] B/C) and 66 (49.6%) had experienced decompensation. Overall, 205 procedures were performed in 60 patients and procedure-related bleedings occurred in 7 (11.7%) patients. Additionally, 38 (28.6%) patients experienced spontaneous (15 minor, 23 major) bleedings during a median follow-up of 10.5 (IQR: 4.0-27.8) months. Spontaneous bleedings in ACLD patients were more common in CPS-B/C (at 12 months: 36.9% vs CPS-A: 15.9%, subdistribution hazard ratio [SHR]: 3.23 [95% CI: 1.59-6.58], P < .001), as were major bleedings (at 12 months: 22.0% vs 5.0%, SHR: 5.82 [95% CI: 2.00-16.90], P < .001). Importantly, CPS (adjusted SHR: 4.12 [91% CI: 1.82-9.37], P < .001), but not the presence of hepatocellular carcinoma or varices, was independently associated with major bleeding during DOAC treatment. Additionally, ACLD patients experiencing bleeding had worse overall survival (at 12 months: 88.9% vs 95.0% without bleeding; P < .001). Edoxaban anti-Xa peak levels were higher in patients with CPS-B/C (345 [95% CI: 169-395] vs CPS-A: 137 [95% CI: 96-248] ng/mL, P = .048) and were associated with lower TM-TGA. Importantly, spontaneous bleeding rates were comparable to VKA/LMWH patients. CONCLUSIONS Anticoagulants including DOACs should be used with caution in patients with advanced liver disease due to a significant rate of spontaneous bleeding events.
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Affiliation(s)
- Georg Semmler
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Katharina Pomej
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - David J. M. Bauer
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Lorenz Balcar
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Benedikt Simbrunner
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria,Christian Doppler Laboratory for Portal Hypertension and Liver FibrosisMedical University of ViennaViennaAustria
| | - Teresa Binter
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Lukas Hartl
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Jeannette Becker
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Matthias Pinter
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Peter Quehenberger
- Department of Laboratory MedicineMedical University of ViennaViennaAustria
| | - Michael Trauner
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria
| | - Mattias Mandorfer
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
| | - Ton Lisman
- Surgical Research Laboratory and Section of Hepatobiliary Surgery and Liver TransplantationDepartment of SurgeryUniversity of Groningen, University Medical Center GroningenGroningenthe Netherlands
| | - Thomas Reiberger
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria,Christian Doppler Laboratory for Portal Hypertension and Liver FibrosisMedical University of ViennaViennaAustria
| | - Bernhard Scheiner
- Division of Gastroenterology and HepatologyDepartment of Internal Medicine IIIMedical University of ViennaViennaAustria,Vienna Hepatic Hemodynamic LabMedical University of ViennaViennaAustria
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