Budd-Chiari Syndrome Research Articles

Hemodynamic and Hydrodynamic Pathophysiology in Chiari Type 1 Malformations: Towards Understanding the Genesis of Syrinx.

The pathophysiology of this association of type 1 Chiari malformation (CM1) and syrinxes is still unknown. There is an alteration in the dynamics of neurofluids (cerebrospinal fluid, arterial and venous blood) during the cardiac cycle in CM1. Our objective is to quantify CSF or arterial blood or venous blood flow in patients with Chiari syndrome (CS) with and without syrinxes using phase-contrast MRI (PCMRI). We included 28 patients with CM1 (9 with syrinxes, 19 without). Morphological MRI with complementary PCMRI sequences was performed. We analyzed intraventricular CSF, subarachnoid spaces CSF, blood, and tonsillar pulsatility. There is a highly significant correlation (p < 0.001) between cerebral blood flow, cerebral vascular expansion volume and venous drainage distribution. Venous drainage distribution is significantly inversely correlated with oscillatory CSF volume at the level of the foramen magnum plane [-0.37 (0.04)] and not significantly correlated at the C2C3 level [-0.37 (0.05)] over our entire population. This correlation maintained the same trend in patients with syrinxes [-0.80 (<0.01)] and disappeared in patients without a syrinx [-0.05 (0.81)]. The distribution of venous drainage is an important factor in intracranial homeostasis. Impaired venous drainage would lead to greater involvement of the CSF in compensating for arterial blood influx, thus contributing to syrinx genesis.

Direct oral anticoagulants for the treatment of splanchnic vein thrombosis: A state of art.

Splanchnic vein thrombosis (SVT) is a manifestation of venous thromboembolism in an unusual site. Portal, mesenteric, and splenic veins are the most common vessels involved in SVT which occurs mainly in patients with liver cirrhosis, although non-cirrhotic patients could be affected as well. Thrombosis of hepatic veins, also known as Budd-Chiari syndrome, is another manifestation of SVT. Prompt diagnosis and intervention are mandatory in order to increase the recalization rate and reduce the risk of thrombus progression and hypertensive complications. Traditional anticoagulation with heparin and vitamin-K antagonists is the treatment of choice in these cases. However, recent studies have shown promising results on the efficacy and safety of direct oral anticoagulants (DOACs) in this setting. Available results are mainly based on retrospective studies with small sample size, but first clinical trials have been published in the last years. This manuscript aims to provide an updated overview of the current evidence regarding the role of DOACs for SVT in both cirrhotic and non-cirrhotic patients.

Interventions in cerebrovascular emergencies among patients with Paroxysmal nocturnal haemoglobinuria - A word of caution

IntroductionParoxysmal nocturnal haemoglobinuria (PNH) is a clonal hematopoietic disorder, where there is deficiency of glycosylphosphatidylinositol (GPI) anchored proteins in the cell membrane, leading to increased complement sensitivity of red blood cells, intravascular hemolysis and vascular inflammation. Arterial and venous strokes in patients with PNH are a rarity posing significant diagnostic and therapeutic challenges. We report our experience with management of PNH patients with cerebrovascular emergencies. MethodsWe report 2 patients with PNH, one who was previously diagnosed with PNH and had arterial stroke, the other had an index presentation of cerebral venous sinus thrombosis (CVT) and was subsequently diagnosed with PNH. We also present the systematic review of literature reporting similar cases, highlighting the challenges in management. ResultsBoth patients presented to our centre with cerebrovascular emergency. The first patient was a diagnosed with PNH, and presented with left hemispheric infarction caused by thrombosis of middle cerebral artery. He was thrombolysed and underwent mechanical thrombectomy, which was unsuccessful in view of repeated re – thrombosis of the vessel. The patient survived with significant disability. The second patient had severe cerebral venous sinus thrombosis with large right hemispheric hemorrhagic venous infarction. She underwent emergency decompressive hemicraniectomy complicated by massive blood loss and disseminated intravascular coagulation. She subsequently had recurrent life threatening intracranial bleed secondary to platelet transfusions, thrombocytopenia, and use of contrast agents. She progressed to develop Budd Chiari syndrome and was initiated on Eculuzimab. She became transfusion independent, however remained in minimally conscious state and succumbed to sepsis. ConclusionsManagement of arterial and venous strokes is complex in patients with PNH. Invasive procedures and platelet transfusions are to be avoided in acute thrombosis, till robust evidence is available establishing the safety of the same in patients with PNH. Eculuzimab is a promising option, but far from reach for patients in developing countries.

Reversion of liver cirrhosis after endovascular treatment in Chinese patients with Budd-Chiari syndrome.

To investigate the impact of endovascular (EV) treatment on liver cirrhosis in Chinese patients with Budd-Chiari syndrome (BCS). 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. 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±8.89cm/s vs. 17.51±6.67cm/s, p<0.001) and decreased liver stiffness (30.37±6.39kPa vs.23.70±7.99kPa, p<0.001), portal vein diameter (14.97±3.42mm vs. 13.36±2.89mm, p<0.001), and spleen volume (870.00±355.61cm3 vs. 771.36±277.45cm3 , 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. 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.

Unraveling the Connection: PNH Induced Budd-Chiari Syndrome - A Case Study and Early Detection Emphasis

Unraveling the Connection: PNH Induced Budd-Chiari Syndrome - A Case Study and Early Detection Emphasis

Determination of the JAK2 V617F mutation in thrombosis patients

Background: Janus kinase 2 (JAK2) gene mutation causes uncontrolled myeloproliferation independent of cytokines and abnormal formation of the endogenous erythroid colony. JAK2 mutations were frequently observed in myeloproliferative neoplasms (MPNs), especially in polycythemia vera (PV) and essential thrombocythemia (ET). MPNs represent a risk factor for the development of thrombosis that is a significant cause of morbidity and mortality in patients. Materials and methods: We aimed to study the correlation between JAK2 mutation and thrombosis. Thirty-nine patients with a clinical diagnosis of thrombocytosis and Budd-Chiari syndrome were collected to determine the JAK2 V617F mutation. Genomic DNA from all specimens was amplified and detected the presence of JAK2 V617F mutation by AS-PCR. Results: We demonstrated JAK2 V617F mutation in both patients with a clinical diagnosis of thrombocytosis and Budd-Chiari syndrome. We found that 11 of 37 (29.7%) thrombocytosis patients had a JAK2 V617F mutation. Moreover, one of two patients who represented as Budd-Chiari syndrome was positive for JAK2 V617F mutation. In addition, JAK2 V617F mutation was associated with thrombosis. However, further study in large series is needed to support this finding. Conclusion: Determination of the JAK2 V617F mutation may be helpful for screening latent or occult MPNs patients who have an occurrence of thrombosis to adjust the appropriate treatment for good patient outcomes.

A remarkable presentation of a massive Budd-Chiari syndrome.

A 59-year-old female presented to the emergency department with malaise, significant weight loss, abdominal discomfort, dyspnoea and severely swollen peripheral extremities. She had a past medical history of sickle cell anaemia, a latent tuberculosis infection and a chronic hepatitis B that was treated with PEG-interferon alpha-2 in 2008. Since 2018, she was lost to follow-up. The patient was critically ill, sarcopenic and lethargic. Clinical examination revealed icteric sclerae and a markedly distended and diffusely tender abdomen and peripheral oedemas. Her vital signs included a blood pressure of 105/64 mmHg, tachycardia of 130 bpm, hypothermia of 35.6°C and a SpO2 of 100% in ambient air. Laboratory workup demonstrated a total bilirubin of 12.9 mg/dL, AST 500 U/L, ALT 218 U/L, ALP 178 U/L and GGT 126 U/L, a thrombocytopenia of 34 x10E3/ μL, 16,01 x10E3/μL leukocytes, a CRP of 94.8 mg/L and a lactate of 10,12 mmol/L. The serum creatinine was 1.04 mg/dL, eGFR 54.1 ml/min. The INR was 2.77 and there was a severe hypoalbuminemia (22 g/L). D-dimers were &gt;20 000 ng/ml. The patient presented also with spontaneous hypoglycaemia. A multiphasic contrast-enhanced thoracic and abdominal computed tomography (CT) was performed (figure 1). Given the results of the CT, a 2D echocardiogram was urgently requested (figure 2).

Percutaneous hepatic vein recanalization in pediatric Budd-Chiari syndrome: Comment.

Percutaneous hepatic vein recanalization in pediatric Budd-Chiari syndrome: Comment.

Factor V Leiden (FVL), Prothrombin G20210A and MTHFR C677T Mutations among Patients of Budd-Chiari Syndrome

Abstract Background Many studies ask whether screening Factor V Leiden (FVL), Prothrombin G20210A and Methyl Tetra Hydro Folate Reductase (MTHFR) C677T Mutations should be routinely performed in patients with Budd–Chiari syndrome (BCS). However, the prevalence of these mutations in such patients is substantially variable. This is the first study in clinical pathology department in Ain Shams University to evaluate the importance of this mutational screening in patients with Budd-Chiari syndrome to detect occult genetic susceptibility to thrombophilia. Aim of the Work This study aims to detect Factor V Leiden (FVL), Prothrombin G20210A and MTHFR C677T Mutations among Patients with Budd-Chiari Syndrome. Patients and Methods This study was a cross sectional study conducted on 35 newly of Budd Chiari syndrome patients who were attending tropical department at Ain Shams University Hospitals during the period from April 2020 to January 2021. Informed consent was obtained from all patients in the study. Results results of our study revealed that JAK2 mutation it was detected in 17.1% of patients (6 patients), While PGM was detected in 2.9% of our patients. Furthermore Factor V Leiden Mutation was detected in 65.7%of patients and MTHFR mutation was detected in 48.6% of patients. The present study found no significant statistical difference between patients with Factor V Leiden Mutation &amp; patients with normal Factor V Leiden regarding CBC parameters, levels of ANA, ACL IgM, ACL IgG, PC Deficiency, PS Deficiency and AT III Deficiency. In addition, our study revealed that there was no effect of MTHFR Mutation &amp; JAK2 Mutation on levels of ANA, ACL IgM, ACL IgG, PC Deficiency, PS Deficiency and AT III Deficiency. Also there was no effect for MTHFR Mutation &amp; JAK2 Mutation on outcome of patients, survival and response to treatment. Conclusion BCS is associated usually with genetic thrombophilia. Most commonly Factor V Leiden Mutation in 65.7%, followed by MTHFR mutation in 48.6% of our patients. Although, only Factor V Leiden Mutation has an effect on patients Outcome, Survival and Response to treatment through its effect on CBC parameters, levels of ANA, ACL IgM, ACL IgG, PC Deficiency, PS Deficiency and AT III Deficiency. Otherwise, JAK2 Mutation have significant effect on hemopoiesis.

Polymorphism Detection of VKORC1 (Vitamin K Epoxide Reductase Complex, Subunit 1) Gene for Warfarin Dose Adjustment in Egyptian Patients with Budd Chiari and/or Portal Vein Thrombosis: A Single Center Experience

Background Budd–Chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the level of the hepatic venules, the large hepatic veins, the inferior vena cava till its junction with the right atrium. The classic triad of abdominal pain, ascites, and hepatomegaly is observed in the vast majority of patients with Budd-Chiari syndrome, but it is nonspecific. Warfarin was introduced into clinical practice in the 1950s, and has been the most widely prescribed oral anti-coagulant for prevention and treatment of thrombotic diseases despite its significant side effects as (bleeding tendency, drug drug interaction). Vitamin K epoxide reductase complex subunit1 (VKORC1), a membrane protein located in hepatocytes, has an important role in the anabolism of vitamin K, which is essential for blood coagulation. Objectives The aim beyond this study is to evaluate effect of the allelic frequency of the VKORC1 (G1639A) polymorphic genes by real time PCR on response of Egyptian patients with Budd–Chiari syndrome or PVT to Warfarin. Patients and Methods The studied patients were selected from patients presented to Budd chiari study group clinic.tropical medicine department in Ain Shams University hospitals. Within 6 months from july 2020 to December 2020, total number of cases during the study period was 50 cases fulfilling the inclusion criteria Results The mean age of the patients was (32.40 ± 9.69) slightly more common in males. The chronic presentation represented 60% of the patients and the acute represented 32%.The most common cause of budd chiarri syndrome was MTHFRD (52%) followed by FVLM (36%) then Primary ant phospholipid antibody syndrome (26%). Patients with the −1639 promoter polymorphism GG genotype had lower dose requirements, whereas the AG/AA genotypes had higher dose. Conclusion Identifying these polymorphisms in patients prior to starting the therapy may help clinicians choose the appropriate warfarin dose &amp; decrease its adverse effects.

Prothrombotic states in portal vein thrombosis and Budd-Chiari syndrome in India: A systematic review and meta-analysis.

Both Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT) have been linked to various prothrombotic (PT) conditions. The PT profile in Asians is different from the west and there are no nationwide epidemiological surveys from India. Hence, the present meta-analysis was aimed at analyzing the prevalence of acquired and hereditary thrombophilia among Indian patients with non-cirrhotic PVT and BCS. A comprehensive literature search of Embase, Medline and Scopus was conducted from January 2000 to February 2022 for studies evaluating the prevalence of various PT conditions in Indian patients with PVT and BCS. Pooled prevalence rates across studies were expressed with summative statistics. Thirty-five studies with 1005 PVT patients and 1391 BCS patients were included in the meta-analysis. At least one PT condition was seen in 46.2% (28.7-63.7) of the PVT patients and 44.9% (37.3-60.7) of the BCS patients. Multiple PT conditions were seen in 13.0% (4.2-21.8) of the PVT patients and 7.9% (3.5-12.4) of the BCS patients. Among PVT patients, hyperhomocysteinemia was the commonest prothrombotic condition (21.6%) followed by protein C (PC) deficiency (10.7%), Janus kinase 2(JAK-2) mutation (8.5%) and antiphospholipid antibodies (APLA) (7.5%). Among patients with BCS, PC deficiency was the commonest prothrombotic condition (10.6%) followed by methylenetetrahydrofolate reductase (MTHFR) mutation (9.8%), APLA (9.7%) and JAK-2 mutation (9.1%). The PT profile in Indian patients with abdominal vein thrombosis is different from that of the western data with a lower prevalence of PT conditions in patients with BCS.

Risk Factors and Predictors of Occlusion of Stent of Trans Jugular Intrahepatic Porto-Systemic Shunt (TIPSS) in Egyptian Patients with Primary Budd Chiari Syndrome: A Single Center Experience

Background Budd–Chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the level of the hepatic venules, the large hepatic veins, the inferior vena cava till its junction with the right atrium. The classic triad of abdominal pain, ascites, and hepatomegaly is observed in the vast majority of patients with Budd-Chiari syndrome. The diagnosis of BCS is made by clinical presentation and confirmatory investigations. Standard laboratory investigations are rarely helpful in establishing the diagnosis, because abnormal liver function tests are not specific for BCS, real time Gray-scale sonography combined with colour flow is considered by some to be the initial imaging technique. The recommended stepwise therapeutic algorithm of BCS is mainly by medical treatment with anticoagulation, angioplasty with or without stenting, TIPS of surgical shunting and liver transplantation if indicated. TIPS is a common teqnique used in BCS and TIPS stent occlusion is major complication. Objectives To identify the possible risk factors and predictors of stent occlusion in primary BCS patients who underwent TIPSS. Patients and Methods The studied patients were selected from patients presented to Budd Chiari outpatient clinic. Tropical medicine department in Ain Shams University hospitals. total number of cases during the study period was 76 cases fulfilling the inclusion criteria, they were divided in 2 groups according to patency of TIPS stent and comparing both groups regarding their pre and post TIPS data. Results The mean age of the patients was (29.29 ± 7.92) with female predominance. FVLM (69.7%) &amp; MTHFRD (67.1%) were the most common causes of BCS in the studied patients. positive and higher CRP results were found in the occluded stent patients than patent groups. also, higher TLC levels were found in the occluded TIPS stent patients. Conclusion Identifying these risk factors may help predict the outcome of TIPS stent patency.

Splanchnic vein thrombosis associated with SARS-CoV-2 infection: A VALDIG case–control study

Whether severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is a risk factor for splanchnic vein thrombosis (SVT) is unknown. This study aims to assess the impact of SARS-CoV-2 infection on the presentation and prognosis of recent SVT and to identify specific characteristics of SARS-CoV-2-associated SVT. This is a retrospective study collecting health-related data of 27 patients presenting with recent SVT in the context of SARS-CoV-2 infection in 12 Vascular Liver Disease Group (VALDIG) centres and in comparison with 494 patients with recent SVT before the SARS-CoV-2 pandemic. Twenty-one patients with SARS-CoV-2 had portal vein thrombosis with or without thrombosis of another splanchnic vein, two had superior mesenteric vein thrombosis, one had splenic vein thrombosis, and three had hepatic vein thrombosis. Diagnosis of SVT was made 10 days (95% CI 0-24 days) after the diagnosis of SARS-CoV-2 infection. Fever (52 vs. 15%; p <0.001) and respiratory symptoms (44 vs. 0%; p <0.001) were more frequent, and median lymphocyte count was lower (1.1×103/mm3vs. 1.6×103/mm3; p= 0.043) in patients with infection than in those without SARS-CoV-2 infection. A prothrombotic condition was identified in 44 and 52% of patients with and without SARS-CoV-2 infection, respectively (p= 0.5). All patients with SARS-CoV-2 received anticoagulation therapy. During a median follow-up of 250 days, three SARS-CoV-2-infected patients (11%) required intestinal resection for infarction 1 to 3 months after diagnosis of SVT compared with 13 (2.6%) controls (p= 0.044). Partial or complete recanalisation of the thrombosed splanchnic vein was performed in 33% of patients with SARS-CoV-2. SARS-CoV-2 infection can be associated with recent SVT. Intestinal infarction leading to intestinal resection might be more frequent in patients with SARS-CoV-2. SARS-CoV-2 infection can be associated with recent SVT. SVT occurring during SARS-CoV-2 infection is characterised by a higher frequency of respiratory symptoms and a lower lymphocyte count. Intestinal infarction leading to intestinal resection appears to occur more frequently in patients with SARS-CoV-2.

MRI Using Gadoxetic Acid in the Work-Up of Liver Nodules Not Conclusively Benign in Budd-Chiari Syndrome: A Prospective Long-Term Follow-Up

Introduction: The incidence of hepatocellular carcinoma (HCC) in Budd-Chiari syndrome (BCS) is unknown and there is no validated diagnostic work-up to define the liver nodules with arterial phase hyperenhancement (APHE), suggesting malignancy. This prospective study evaluates HCC incidence in a Western cohort of patients with BCS and assesses the performance of MRI with hepatobiliary contrast (HB-MRI) for nodule characterization. Methods: Patients with BCS followed in our hospital were prospectively evaluated by MRI with extracellular contrast (EC-MRI). Nodules with APHE categorized as non-conclusively benign by 2 radiologists were studied by HB-MRI and reviewed by 2 radiologists blinded to the EC-MRI results. A new EC-MRI 1 year later and clinical, analytical, and sonographic follow-up every 6 months for a median of 10 years was performed. Results: A total of 55 non-conclusively benign nodules with APHE were detected at EC-MRI in 41 patients. While 32 of them were suggestive of HCC by EC-MRI, all the 55 nodules showed increased uptake of hepatobiliary contrast. An unequivocal central scar was seen in 12/55 nodules at HB-MRI regardless of it was not detected on the EC-MRI. None of the nodules was hypointense in the hepatobiliary phase (HBP). HCC was not detected during a median of 10 years of follow-up. Conclusions: Detection of nodules with APHE is frequent in patients with BCS, but HCC is rare in Western patients with BCS. While EC-MRI may detect nodules suggesting malignancy, the identification of contrast uptake in the HBP at HB-MRI may help categorize them as benign.

Extracellular vesicles-transmitted long non-coding RNA MTUS2-5 promotes proliferation and vascularization of human vascular endothelial cells in patients with Budd-Chiari syndrome.

The high rates of misdiagnosis and untreated mortality with regard to Budd-Chiari syndrome (BCS) indicated the need to screen effective biomarkers. The aim of this study was to explore the function of extracellular vesicles (EVs) in patients with BCS as well as associated mechanisms. First, differentially expressed long non-coding RNAs (lncRNAs) from EVs separated from serum between BCS and healthy controls were screened using microarray analysis. Second, the proliferation, migration and tube formation of human vascular endothelial cells (HUVECs) were detected after EVs treatment, along with vascular endothelial growth factor (VEGF) levels and inflammatory factors from the cell supernatant. Last, the overexpressed lncRNA was transfected into the cells to further explore the mechanisms involved. Extracellular vesicles of BCS patients have significantly higher levels of lncRNA MTUS2-5 than healthy controls. Apparently, treatment with EVs from BCS or the ones transfected with plasmids that overexpress lncRNA MTUS2-5 enhances proliferation, migration and angiogenesis capacity. The results were considerably better than those obtained from treatment with EVs from healthy controls or transfection with the normal control plasmid, which also elevated the level of VEGF and inflammatory factors. Furthermore, FOS and PTGS2 were potentially regulated by the lncRNA MTUS2-5 transmitted by EVs. The lncRNA MTUS2-5 in EVs plays an important role in angiogenesis in the Budd-Chiari syndrome.

Exploring safety and efficacy of rivaroxaban after living donor liver transplantation: a retrospective study.

Thromboembolic complications remain a significant concern in postoperative patients, particularly those who have undergone liver transplantation. Warfarin has been the standard oral anticoagulant. Direct oral anticoagulants (DOACs) have several advantages over warfarin, including rapid onset of action and standardized dose guidelines. We aimed to assess the safety of rivaroxaban in living donor liver transplantation (LDLT) recipients. This study was a single-center, retrospective descriptive analysis of LDLT recipients who received rivaroxaban between December 2020 and April 2022. A total of 27 recipients received rivaroxaban postoperatively. Liver function tests, immunosuppression levels, serum creatinine, and INR were recorded before the initiation of rivaroxaban and then on post-therapy days 1, 7, 14, 28, 90, and 180. Among the 27 recipients receiving rivaroxaban postoperatively, portal venous thrombosis was the most prevalent indication for anticoagulation (44.4%), followed by Budd-Chiari syndrome (29.6%). Nine patients had a twofold increase in either ALT or AST values, two of whom were treated for biliary strictures and the others for rejection. Eighteen patients were given tacrolimus, and eight were on cyclosporine, with one patient switched from tacrolimus to cyclosporine due to insufficient therapeutic levels. There were no incidents of bleeding or re-thrombosis during the 180-day follow-up period. Rivaroxaban may be a safe and effective alternative in LDLT recipients with no significant adverse incidents. Further studies with larger sample sizes are needed to confirm these findings and determine this population's optimal dose and duration of rivaroxaban therapy.

Hepatic artery infusion chemotherapy for advanced hepatocellular carcinoma

Objective:This study’s purpose was to evaluate the response, safety and overall survival of trans-arterial infusion chemotherapy in patients with advanced hepatocellular carcinoma with preserved hepatic function.Methods:This study was carried out on 25 patients, diagnosed with hepatocellular carcinoma (HCC) combined with portal vein tumor thrombosis (PVTT) and underwent hepatic artery infusion chemotherapy (HAIC). Radiological investigations as Triphasic CT or dynamic MRI liver assessment pre and post therapy were acquired. Intra-Arterial chemotherapeutic agent infusion using only doxorubicin was performed.Results:Neither of the patients who underwent HAIC developed complete or partial response. Only one patient (4.8%) from 21 patients under HAIC had stable disease. 20 patients (95%) had progressive disease. Progressive disease was in form of progression at the primary tumor site in form of increased focal lesion size, number or vascular invasion. Vascular invasion was seen in one patient (4.8%) in the form of hepatic vein thrombosis. Mean progression free survival was about 2.24 ± 0.88 months. Mean overall survival was about 5.72 ± 0.89 months.Conclusion:Our study demonstrated lower clinical efficacy and lower disease control rate of repeated HAIC using doxorubicin only infusion in case of advanced HCC with PVT as compared to combined doxorubicin and cisplatin in previous studies as well as the standard therapy with sorafenib.

Hepatic venous outflow obstruction after adult living donor liver transplantation.

Hepatic venous outflow obstruction (HVOO) is a rare but critical vascular complication after adult living donor liver transplantation. We categorized HVOOs according to their morphology (anastomotic stenosis, kinking, and intrahepatic stenosis) and onset (early-onset < 3mo vs. late-onset ≥ 3mo). Overall, 16/324 (4.9%) patients developed HVOO between 2000 and 2020. Fifteen patients underwent interventional radiology. Of the 16 hepatic venous anastomoses within these 15 patients, 12 were anastomotic stenosis, 2 were kinking, and 2 were intrahepatic stenoses. All of the kinking and intrahepatic stenoses required stent placement, but most of the anastomotic stenoses (11/12, 92%) were successfully managed with balloon angioplasty, which avoided stent placement. Graft survival tended to be worse for patients with late-onset HVOO than early-onset HVOO (40% vs. 69.3% at 5y, p = 0.162) despite successful interventional radiology. In conclusion, repeat balloon angioplasty can be considered for simple anastomotic stenosis, but stent placement is recommended for kinking or intrahepatic stenosis. Close follow-up is recommended in patients with late-onset HVOO even after successful treatment.

Unveiling a Rare Association: Budd-Chiari Syndrome as a Consequence of Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus

Unveiling a Rare Association: Budd-Chiari Syndrome as a Consequence of Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus

Perceptions of surgical difficulty in liver transplantation: A European survey and development of the Pitié-Salpêtrière classification

BackgroundSignificant variations exist regarding the definition of difficult liver transplantation. The study goals were to investigate how liver transplant surgeons evaluate the surgical difficulty of liver transplantation and to use the identified factors to classify liver transplantation difficulty. MethodsA Web-based online European survey was presented to liver transplant surgeons. The survey was divided into 3 parts: (1) participant demographics and practices; (2) various situations based on recipient, liver disease, tumor treatment, and technical factors; and (3) 8 real-life clinical vignettes with different levels of complexity. In part 3 of the survey, respondents were asked whether they would perform liver transplantation but were not aware that these patients eventually underwent liver transplantation. ResultsA total of 143 invites were sent out, and 97 (67.8%) participants completed the survey. Most participants considered previous spontaneous bacterial peritonitis, previous supra-mesocolic surgery, hypertrophy of segment I, and obesity to be recipient factors for high-difficulty liver transplantation. Most participants considered liver transplantation to be challenging in patients with Budd–Chiari syndrome, Kasai surgery, polycystic liver disease, diffuse portal vein thrombosis, and a history of open hepatectomy. The proportion of participants indicating that liver transplantation was warranted varied across the 8 cases, from 69% to 100%. Our classification of the surgical difficulty of liver transplantation employed these recipient-related, surgical history–related, and liver disease–related variables and 3 difficulty groups were identified: low, intermediate, and high difficulty groups. ConclusionThis survey provides an overview of the surgical difficulty of various situations in liver transplantation that could be useful for further benchmark and textbook outcome studies.