Research advances in the diagnosis and treatment of hepatic vein occlusion-type Budd-Chiari syndrome / 临床肝胆病杂志

Budd-Chiari syndrome is a rare liver vascular disease and can be classified into hepatic vein occlusion type， inferior vena cava occlusion type， and mixed type according to the location of hepatic outflow tract obstruction. This article reviews the hepatic vein occlusion type from the aspects of epidemiology， subtypes， clinical diagnosis， and treatment strategies.

Rare Association of Budd-Chiari Syndrome with COVID-19: A Case Report

Introduction: Severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) is a virus that can cause respiratory infections and pose a risk to patients' lives. While it primarily affects the airways, it can also lead to extrapulmonary clinical manifestations, such as hypercoagulable states, resulting in conditions like cerebrovascular disease, acute myocardial infarction, and, in rare cases, Budd-Chiari syndrome. Clinical case: This case involves a patient who was admitted to the emergency room with dyspnea on moderate exertion, progressive functional class deterioration, lower extremity edema, cough, fever, and weakness. The patient had a history of coronavirus disease 2019 (COVID-19) infection one month prior. Abdominal CT scan revealed subacute Budd-Chiari syndrome involving the middle sub-hepatic vein, along with right ventricle thrombosis, and a positive test for SARS-CoV-2. Conclusions: Budd-Chiari syndrome has multiple causes, primarily associated with hypercoagulable states or the presence of neoplasms that disrupt liver function or obstruct hepatic venous drainage. However, the understanding of the relationship between COVID-19 hypercoagulability and Budd-Chiari syndrome is still limited. Further research is needed to explore the heterogeneity of its pathogenesis in the context of the SARS-CoV-2 pandemic.

Introducción: el coronavirus del síndrome respiratorio agudo grave de tipo 2 (SARS-CoV-2) es un virus que puede causar infecciones respiratorias y poner en riesgo la vida de los pacientes. Aunque afecta principalmente las vías respiratorias, se pueden presentar manifestaciones clínicas extrapulmonares como estados de hipercoagulabilidad, lo que causa patologías como enfermedad cerebrovascular e infarto agudo de miocardio, y en casos menos frecuentes, síndrome de Budd-Chiari. Caso clínico: se presenta el caso de una paciente que ingresó al servicio de urgencias por disnea de medianos esfuerzos, deterioro progresivo de la clase funcional y edema en extremidades inferiores asociado con tos, fiebre y adinamia. Refirió el antecedente de infección por enfermedad por coronavirus de 2019 (COVID-19) un mes antes y la tomografía de abdomen contrastada reveló síndrome de Budd-Chiari subagudo de la vena subhepática media asociada a trombosis del ventrículo derecho con prueba positiva para SARS-CoV-2. Conclusiones: El síndrome de Budd-Chiari es de origen multicausal, principalmente por estados de hipercoagulabilidad o presencia de neoplasias que ocasionan disrupción de la función del hígado o causan la ocupación de espacio sobre el drenaje venoso hepático; sin embargo, en el contexto de la pandemia de SARS CoV-2, aún se conoce muy poco la heterogeneidad de su patogénesis, como es la hipercoagulabilidad de la COVID-19 y el síndrome de Budd-Chiari, por lo que inferimos que están altamente relacionados.

Discussion of issues related to Budd-Chiari syndrome / 国际外科学杂志

Budd-Chiari syndrome (B-CS) is a rare disease caused by hepatic vein outflow obstruction, and its etiology is complex and inconclusive. Current studies suggest that vascular dysplasia, gut microbiota and trace element imbalance may be related to the pathogenesis of B-CS, and the development of high-throughput sequencing technology may help to clarify the exact pathogenesis of B-CS. The symptoms of B-CS are not specific and rely mainly on imaging methods to establish the diagnosis, so there is an urgent need to find new noninvasive biological diagnostic markers. In addition, there are many pathological types and different criteria of B-CS, which mostly can′t fully reflect the pathophysiological changes of B-CS patients and guide clinical treatment. Therefore, we recommend pathophysiological classification according to the hemodynamic changes and collateral circulation compensation of B-CS, and then develop personalized treatment strategies for stratified management different from the traditional early diagnosis and treatment protocols. This article summarizes and discusses the above contents.

Alternativas de manejo intervencionista en el síndrome de Budd-Chiari. Serie de casos y revisión de la literatura / Interventional management alternatives in Budd-Chiari syndrome. Series of cases and review of the literature

La obstrucción en el tracto de salida del flujo venoso hepático, también conocida como síndrome de Budd-Chiari, es una condición infrecuente que causa congestión hepática, hipertensión portal, ne-crosis de los hepatocitos y, eventualmente, falla hepática aguda o crónica. Actualmente, el manejo de esta condición representa un reto para el médico, quien debe estar preparado para determinar la mejor alternativa entre las diferentes opciones terapéuticas disponibles. Este artículo pretende ilus-trar las alternativas del manejo intervencionista de esta enfermedad, a través de una serie de casos de pacientes tratados en el servicio de Radiología Intervencionista de un hospital de referencia de la ciudad de Medellín, entre 2011 y 2017.

Hepatic venous outflow tract obstruction, also known as Budd-Chiari syndrome, is a rare condition that causes hepatic congestion, portal hypertension, hepatocyte necrosis and eventually acute or chronic liver failure. Currently, the management of this condition represents a challenge for the physi-cian, who must be prepared to determine the best alternative among the different therapeutic options available. This article aims to illustrate the alternatives of interventional management of this disease, through a series of cases of patients treated in the Interventional Radiology service of a referral hos-pital in the city of Medellin, between 2011 and 2017.

Transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation for Budd-Chiari syndrome complicated with liver cancer: a case report with surgical video / 器官移植

Objective To summarize clinical experience of transabdominal pericardial anastomosis of suprahepatic vena cava of the donor and right atrium of the recipient in liver transplantation for Budd-Chiari syndrome (BCS) complicated with liver cancer. Methods Clinical data of a BCS patient complicated with liver cancer undergoing transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation were retrospectively analyzed. Results The hepatic vein and suprahepatic vena cava were partially occluded in the patient. Liver transplantation was completed by transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium with beating-heart. In addition, due to pathological changes of the recipient's hepatic artery, splenic artery of the recipient was cut off, distal ligation was performed, and the proximal end was reversed and anastomosed with the common hepatic artery of the donor liver, and the reconstruction of hepatic artery was completed. The surgery was successfully performed. At approximately postoperative 1 week, the function of the liver allograft was gradually restored to normal, and no major complications occurred. The patient was discharged at postoperative 25 d. No signs of BCS recurrence was reported after 8-month follow-up. Conclusions It is safe and feasible to treat BCS by liver transplantation with transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium. BCS patients complicated with liver cancer obtain favorable prognosis.

Síndrome de Budd-Chiari secundario asociado a hepatitis alcohólica grave: reporte de caso / Secondary Budd-Chiari syndrome associated with severe alcoholic hepatitis: A case report

Resumen La hepatitis alcohólica es la inflamación aguda del hígado secundaria al consumo de alcohol en cantidades hepatotóxicas, su fisiopatología está influida por diversos factores asociados. El diagnóstico tiene tres escenarios: la hepatitis alcohólica probable, posible y definitiva. La hepatitis alcohólica probable se basa en el cumplimiento de criterios diagnósticos clínicos y laboratoriales; la hepatitis alcohólica posible corresponde a casos de cumplimiento de estos criterios, pero con presencia de factores potencialmente confusores del diagnóstico, y la definitiva se sustenta sobre una base histopatológica. El síndrome de Budd-Chiari es una entidad poco frecuente, heterogénea y potencialmente letal, caracterizada por la presencia de trombos a nivel de las venas suprahepáticas, así como de la vena cava inferior. El diagnóstico definitivo está basado en pruebas de imagen. Por su parte, el síndrome de Budd-Chiari secundario es una entidad aún más infrecuente, poco estudiada, cuyo diagnóstico es difícil debido a su gran similitud al síndrome de Budd-Chiari primario, tanto clínica como en imágenes diagnósticas, por lo que se tiene que acudir a métodos diagnósticos de mayor complejidad e incluso invasivos. A continuación, se presenta un caso inusual de un paciente con consumo crónico de alcohol que presenta síndrome de Budd-Chiari secundario asociado a hepatitis alcohólica grave confirmada mediante biopsia hepática y sometido a múltiples estudios de imagen que descartaron la presencia de trombosis a nivel de las venas suprahepáticas.

Abstract Alcoholic hepatitis is an acute inflammation of the liver secondary to the consumption of alcohol in hepatotoxic amounts; various associated factors influence its pathophysiology. The diagnosis has three scenarios: probable, possible, and definite alcoholic hepatitis. Probable alcoholic hepatitis is based on compliance with clinical and laboratory diagnostic criteria; possible alcoholic hepatitis corresponds to cases that meet these criteria but with potentially confounding factors in the diagnosis, and the definite one is based on histopathology. Budd-Chiari syndrome is a rare, heterogeneous, potentially lethal entity characterized by thrombi in the suprahepatic veins and the inferior vena cava. The final diagnosis relies on imaging tests. Moreover, secondary Budd-Chiari syndrome is an even rarer entity, little studied, whose diagnosis is difficult due to its remarkable similarity to primary Budd-Chiari syndrome, both clinically and in diagnostic images, for which more complex and even invasive diagnostic methods must be used. Then, we describe an unusual case of a male patient with chronic alcohol consumption presenting with secondary Budd-Chiari syndrome associated with severe alcoholic hepatitis confirmed by liver biopsy and subjected to multiple imaging studies that ruled out thrombosis in the suprahepatic veins.

A case report of Budd-Chiari syndrome with cardiac complications and low SPO2 successfully managed by an integrated-approach

Budd-Chiari syndrome [BCS] is a rare, potentially life-threatening condition characterized by the triad of abdominal pain, ascites, and hepatomegaly (with or without jaundice). There will be an underlying disorder in most cases. The diagnosis, as well as management of the case, requires a multidisciplinary approach. The treatment should aim at reducing the presenting symptoms as well as removing the underlying pathology. Here we explain a case report of a 21-year-old male patient in the subacute stage of BCS with its complications and considered as a candidate for liver transplantation. His liver enzymes, PT/INR, D- dimer, and homocysteine values were above normal levels. The arterial oxygen saturation level was subnormal, and he was on supportive oxygen supplement. Inferior venacava [IVC] Doppler revealed a non-obstructive intrahepatic thrombus. The patient was treated with Homoeopathic medicine Arsenicum album and Arnica montana, given as an adjuvant to conventional treatment.

Síndrome de Budd Chiari secundario al síndrome antifosfolipídico y lupus eritematoso sistémico en una adolescente: reporte de un caso

El síndrome de Budd-Chiari es causado por una obstrucción del flujo sanguíneo del hígado. Los casos publicados del síndrome antifosfolípido asociado a SBC son limitados en la población pediátrica. Reportamos el caso de una adolescente de 15 años que presentó fiebre, ascitis y hepatoesplenomegalia. En la ecografía Doppler hepática se observó ausencia de flujo en la vena hepática derecha y media, y en la vena cava inferior. En la tomografía abdominal se observó una extensa trombosis de la vena cava inferior. Durante la hospitalización se le diagnosticó SAF y lupus eritematoso sistémico. Se le administró tratamiento con heparina no fraccionada, heparina de bajo peso molecular y anticoagulantes. El síndrome de Budd-Chiari secundario al síndrome antifosfolípido es una enfermedad potencialmente mortal. El diagnóstico y el tratamiento oportunos permiten mejorar la calidad de vida del paciente.

Budd-Chiari syndrome is caused by an obstruction of blood flow to the liver. Published cases of the antiphospholipid syndrome associated with BCS are limited in the pediatric population. We report a 15-year-old adolescent who presented with fever, ascites, and hepatosplenomegaly. Hepatic Doppler ultrasound revealed no flow in the right and middle hepatic veins and in the inferior vena cava. Abdominal tomography revealed extensive thrombosis of the inferior vena cava. During hospitalization, she was diagnosed with antiphospholipid syndrome and systemic lupus erythematosus. She was given treatment with unfractionated heparin, low molecular weight heparin, and anticoagulants. Budd-Chiari syndrome secondary to the antiphospholipid syndrome is a life-threatening disease. Timely diagnosis and treatment improve the quality of life of the patient.

Post-COVID Pediatric Budd-Chiari Syndrome

Introduction: Budd-Chiari syndrome (BCS) is rare in children. Underlying etiologies, natural history and management differ in children and adults. Treatment options like liver transplantation and transjugular intrahepatic portosystemic shunt have also been less well-studied in children. Presented here is the case of a male child with BCS after coronavirus disease 2019 (COVID-19) infection. Case presentation: A 3-year-old male child presented with complaints of gradually increasing abdominal distension, constipation, decreased urine output, visible veins over abdomen and swelling in lower limbs and periorbital edema since last 15 days. He was diagnosed as BCS after multiple imaging investigations, including triple phase contrast-enhanced computed tomography (CECT) abdomen. His COVID antibody tested positive. Conclusion: Practice guidelines for children with BCS should be formulated, expert group recommendations should be reviewed and a consensus statement should be issued. Underlying etiology remains obscure despite extensive work-up in most of the children. Association of COVID-19 with BCS may be incidental but should be studied further as COVID is known to cause thrombotic complications

Analysis of risk factors of Budd-Chiari syndrome associated with hepatocellular carcinoma in patients who underwent endovascular recanalization / 中华肝胆外科杂志

Objective:To study the risk factors of Budd-Chiari syndrome (BCS) associated with hepatocellular carcinoma in patients who underwent endovascular recanalization.Methods:The data of 340 patients with BCS who underwent endovascular recanalization at the Affiliated Hospital of Xuzhou Medical University between January 2015 and June 2021 were retrospectively collected. Using propensity score matching, a total of 57 patients (40 males and 17 females) were enrolled into this study, with the age of (50.4±8.7) years. Patients were divided into the hepatocellular carcinoma group ( n=19) and the control group ( n=38) according to whether occurrence of hepatocellular carcinoma after cardovascular recanalization. Preoperative indicators including gender, age, BCS type, and model for end-stage liver disease (MELD) score, and postoperative indicators including alpha fetoprotein, intrahepatic nodule formation, vascular restenosis, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were compared between the two groups after propensity score matching. Multivariate logistic regression analysis was used to analyze the risk factors of BCS associated with after endovascular recanalization in these patients. Results:There were no significant differences in gender, age, BCS type, MELD score and other preoperative data between the two groups (all P>0.05). The proportions of patients with postoperative alpha fetoprotein>9.0 μg/L, AST>40 U/L, ALT>50 U/L, intrahepatic nodules and vascular restenosis after endovascular treatment in the hepatocellular carcinoma group were significantly higher than those in the control group (all P<0.05). Multivariate analysis showed postoperative alpha fetoprotein >9.0 μg/L ( OR=46.778, 95% CI: 3.310-661.140), AST>40 U/L ( OR=36.307, 95% CI: 1.317-1 001.009), intrahepatic nodule formation ( OR=66.254, 95% CI: 4.225-1 038.974) and vascular restenosis ( OR=16.276, 95% CI: 1.712-154.773) to have an increased risk of being associated with hepatocellular carcinoma in these BCS patients (all P<0.05). Conclusion:Postoperative alpha fetoprotein>9.0 μg/L, AST>40 U/L, intrahepatic nodule formation and vascular restenosis were independent risk factors of BCS associated with hepatocellular carcinoma in patients who underwent endovascular recanalization.

Impact of left hepatic vein classification on hepatic vein reconstruction and prognosis after pediatric living-donor liver transplantation using left lateral liver segments / 中华肝胆外科杂志

Objective:To study the impact of donor left hepatic vein classification and the reconstruction methods on hepatic venous outflow obstruction (HVOO) after pediatric living-donor liver transplantation using left lateral liver segments.Methods:A retrospective study was performed on the clinical data of 653 children recipients who underwent living-donor liver transplantation with left lateral liver segments from January 2014 to December 2020 at Tianjin First Central Hospital. There were 309 males and 344 females, aged 7.0 (6.0, 10.0) months, with an age range of 3-121 months. Based on the left hepatic vein on preoperative donor enhancement CT as well as the intraoperative reconstruction methods, the recipients were divided into 3 groups: type Ⅰ group ( n=514), anastomosis using a single opening was performed directly between the donor and the recipient; type Ⅱ group ( n=118), angioplasty was performed on two adjacent recipient venous orifices before anastomosis, and type Ⅲ group ( n=21), an interposition vessel was anastomosed to two widely spaced openings or the two veins were anastomosed separately. The preoperative general status of the patient, postoperative HVOO incidences, and graft and recipient survival rates were compared among the three groups. The patients were followed up by outpatient reexamination or telephone. Results:Graft to recipient weight ratio in the type Ⅲ group was smaller than that in the type Ⅰ group and the type Ⅱ group ( P<0.05). For all the 653 patients, the incidence of postoperative HVOO was 4.59% (30/653), with the incidences of HVOO in the 3 groups of patients were 4.1% for the type Ⅰ group (21/514), 5.1% for the type Ⅱ group (6/118), and 14.3% for the type Ⅲ group (3/21), respectively. There was no significant difference among the groups ( P>0.05). The recipient cumulative survival rates at 1 and 3 years after surgery in the type I group were 97.8% and 97.0%, and the corresponding rates in the type Ⅱ group were 96.5% and 94.2%, and in the type Ⅲ group were 94.1% and 86.9%, respectively. There was a significant difference between the type Ⅰ and type Ⅲ groups ( P=0.048). The graft cumulative survival rates at 1 and 3 years in the type Ⅰ group were 97.4% and 96.9%, and the corresponding rates in the type Ⅱ group were 94.9% and 92.5%, and in the type Ⅲ group were 94.1% and 86.9%, respectively. The difference in the postoperative graft cumulative survival rates between the type Ⅰ group and type Ⅱ group was significant ( P=0.044). Conclusions:The anatomy of the left hepatic vein supplying the left lateral liver segment was highly variable, and the majority of the variations could be reconstructed. A reasonable reconstructive method could reduce the incidence of postoperative HVOO and improved the outcomes of the graft.

Effect of metformin on liver fibrosis in a mouse model of Budd-Chiari syndrome / 临床肝胆病杂志

Objective To investigate the effect of metformin on liver fibrosis in a mouse model of Budd-Chiari syndrome and its mechanism. Methods A total of 30 male C57 mice were randomly divided into sham-operation group (SHAM group) with 6 mice, sham operation+ metformin group (SHAM+M group) with 5 mice, Budd-Chiari model group (BCS group) with 10 mice, and Budd-Chiari model+metformin group (BCS+M group) with 9 mice. The mice in the model group were treated with partial ligation of the inferior vena cava, those in the SHAM group were not treated with ligation, and those in the metformin group were given 0.1% metformin in drinking water besides modeling. The mice were sacrificed after 6 weeks. HE staining and picrosirius red staining were used to observe liver histopathology and collagen deposition; immunohistochemistry was used to measure the expressions of α-smooth muscle actin (α-SMA) and fibrinogen; quantitative real-time PCR was used to measure the mRNA expression of hypoxia-inducible factor 1α (HIF-1α) and type Ⅰ collagen (collagen 1), and Western blot was used to measure the relative protein expression levels of HIF-1α, vascular endothelial growth factor (VEGF), fibrinogen, α-SMA, and collagen 1. A one-way analysis of variance was used for comparison of continuous data between multiple groups, and the least significant difference t -test was used for further comparison between two groups. Results Pathological staining showed that compared with the SHAM group, the BCS group had significant liver fibrosis, disordered arrangement of hepatocytes near the central vein, sinusoidal expansion with red blood cell deposition and a small amount of inflammatory cell infiltration, and collagen deposition. The BCS group had significant increases in the mRNA expression levels of HIF-1α and collagen 1 and the protein expression levels of α-SMA, collagen 1, HIF-1α, VEGF, and fibrinogen (all P < 0.05); compared with the BCS group, the BCS+M group had significant alleviation of liver fibrosis, red blood cell deposition, and collagen deposition and significant reductions in the mRNA expression levels of HIF-1α and collagen 1 and the protein expression levels of α-SMA, collagen 1, HIF-1α, VEGF, and fibrinogen (all P < 0.05). Conclusion Metformin can improve congestive liver fibrosis caused by Budd-Chiari syndrome, possibly by reducing microthrombus in hepatic sinusoid and inhibiting the HIF-1α/VEGF pathway.

Platelet-albumin-bilirubin score in assessing short-term prognosis of patients with Budd-Chiari syndrome presenting with upper gastrointestinal bleeding / 中华肝胆外科杂志

Objective:To study the factors influencing short-term prognosis of patients with Budd-Chiari syndrome (B-CS) presenting with upper gastrointestinal bleeding and to assess the predictive value of platelet-albumin-bilirubin score (PALBI) on death within 30 d in these patients.Methods:A retrospective study was conducted on 74 patients with B-CS who presented with upper gastrointestinal bleeding and were treated at the First Affiliated Hospital of Zhengzhou University from January 2014 to February 2020. There were 51 males and 23 females, with age of (46.5±11.1) years old. These patients were divided into the survival group ( n=58) and the death group ( n=16) according to the disease outcomes up to 30 d of follow-up. Factors influencing short-term deaths of these patients were analyzed, and the predictive values of PALBI, ALBI, CTP and MELD scores on short-term prognosis of the patients were assessed. The receiver operating characteristic (ROC) curves were plotted, and the areas under the curve (AUC) were calculated and compared. Results:The differences between patients in the survival and death groups for white blood cell, platelet, PALBI score, PALBI classification, ALBI score, CTP score, MELD score, and presence or absence of hepatic encephalopathy were significantly different (all P<0.05). Multivariate logistic regression analysis showed that CTP score≥10 or CTP grade C ( OR=1.669, 95% CI: 1.048-2.661), and PALBI score >-2.09 or PALBI grade 3 ( OR=5.245, 95% CI: 2.128-12.924) were independent risk factors for predicting death within 30 days. The areas under the ROC curves for PALBI, ALBI, CTP and MELD score were 0.89, 0.72, 0.77 and 0.76, with the cut-off values of -1.92, -1.60, 8.50 and 13.60, respectively. The differences between the PALBI score and ALBI, CTP scores were significantly different ( P<0.05). Conclusion:The PALBI score showed a positive predictive value on short-term prognostic assessment of patients with B-CS presenting with upper gastrointestinal bleeding. It was comparable to the effect of the MELD score but was significantly better than the ALBI and CTP scores.

Study on the mechanism of mTOR/HIF-1α signaling pathway in Budd-Chiari syndrome liver fibrosis / 国际外科学杂志

Objective:To explore the mechanism of mTOR/HIF-1α signaling pathway in Budd-Chiari syndrome (B-CS) liver fibrosis.Methods:Twenty male C57 mice were randomly divided into Sham operation group (Sham), sham operation+ rapamycin (Sham+ Ra) group, B-CS group, B-CS+ rapamycin (B-CS+ Ra) Group, 5 in each group. The B-CS mouse model was constructed by partial ligation of the inferior vena cava(IVC) at the posterior segment of the liver; IVC was not ligated in the Sham group. Mice in Sham+ Ra and B-CS+ Ra groups were intraperitoneally injected with rapamycin (2 mg/kg, 5% DMSO solution preparation) every other day, Sham group and B-CS group were injected with the same dose of 5% DMSO solution.After 6 weeks, samples were taken, and part of the liver tissue was used to make paraffin sections for hematoxylin-eosin (HE) and Sirus Red staining to observe the pathological changes, and immunohistochemical staining to detect the expression of α-SMA and Fibrinogen in liver tissues; Protein and RNA were extracted from fresh liver tissue, and Western-blot was used to detect α-SMA, Fibrinogen, p-mTOR, mTOR, HIF-1α, Collagen I, and VEGF protein levels. Real-time fluorescent quantitative PCR was used to detect mTOR, HIF-1α, CollagenⅠ, VEGF mRNA levels.Measurement data were expressed as mean±standard deviation ( ± s), and the comparison between groups was performed by one-way ANOVA test. Results:The results of pathological staining showed that in the B-CS group, there was severe congestion around the central vein of the liver and sinusoids, widening of the sinus space, and increased collagen deposition, indicating that this study successfully established a mouse B-CS liver fibrosis model. The expression levels of fibrosis indicators α-SMA and Collagen I protein, mTOR pathway related indicators p-mTOR and HIF-1α protein, and microthrombus indicator Fibrinogen protein in the Sham group were 0.027±0.012, 0.337±0.008, 0.138±0.024, 0.296±0.113, 0.733±0.192; B-CS group were 0.986±0.001, 0.927±0.055, 0.936±0.044, 1.693±0.443, 1.612±0.068, and the differences were statistically significant ( P<0.05). The expression levels of B-CS+ Ra group were 0.707±0.078, 0.311±0.024, 0.332±0.094, 0.254±0.117, 0.569±0.075, which were statistically significant compared with B-CS group ( P<0.05). Conclusions:The mTOR/HIF-1α signaling pathway is significantly activated in mouse B-CS liver fibrosis. This pathway may participate in the development of liver fibrosis by regulating microthrombosis.

Ascitis en paciente con colitis ulcerativa como presentación de síndrome de Budd-Chiari: reporte de caso y revisión de la literatura / Ascites in a patient with ulcerative colitis presenting Budd-Chiari syndrome: case report and literature review

La enfermedad inflamatoria intestinal es una patología crónica en la que participa el sistema inmune, que puede acompañarse de manifestaciones extraintestinales en el hígado y vías biliares. El síndrome de Budd-Chiari se ha descrito previamente como una complicación rara de la colitis ulcerativa. Se describe el caso de una paciente joven con antecedente de colitis ulcerativa y trombosis venosa de miembro inferior, en manejo crónico con aminosalicilatos y anticoagulada durante 6 meses, quien debutó con cuadro clínico de ascitis de dos meses de evolución, con líquido ascítico de características hipertensivas, documentación imagenológica y biopsia hepática compatibles con síndrome de Budd-Chiari. Se presenta reporte de caso y revisión breve de la literatura.

Inflammatory bowel disease is a chronic disease involving the immune system, which can be accompanied by extraintestinal manifestations in the liver and biliary tract. Budd-Chiari syndrome has previously been described as a rare complication of ulcerative colitis. We describe the case of a young patient with a history of ulcerative colitis and venous thrombosis of the lower limb, under chronic management with aminosalicylates, and anticoagulation therapy for the past 6 months, who consulted with a 2-month history of ascites, with hypertension-related ascitic fluid, imaging analysis and liver biopsy compatible with Budd-Chiari syndrome. A case report and a brief literature review are presented.

Síndrome de Budd-Chiari por obstrução da via de saída da veia hepática por membrana: tratamento percutâneo por angioplastia com balão / Budd-Chiari syndrome due to hepatic venous web outflow obstruction: percutaneous treatment with balloon angioplasty

Resumo A síndrome de Budd-Chiari é uma doença venosa hepática rara, mais incidente em adultos jovens, podendo se apresentar na forma aguda, subaguda ou crônica, o que resulta em hipertensão portal. O tratamento tradicional consiste em técnicas de trombólise e de shunts portossistêmicos intra-hepáticos, como pontes para o transplante hepático. Recentemente, técnicas de angioplastia com balão ou stents têm sido relatadas para o tratamento dessa afecção. Neste artigo, é relatado e discutido um caso de síndrome de Budd-Chiari por obstrução membranosa da via de saída da veia supra-hepática com trombose da veia hepática média em uma paciente de 24 anos. O tratamento estabelecido foi a angioplastia transjugular com balão, que obteve resultados satisfatórios e boa evolução clínica.

Abstract The Budd-Chiari syndrome is a rare hepatic venous disease. It is more prevalent in young adults and may present in acute, subacute, or chronic forms, causing portal hypertension. Traditional treatment consists of thrombolysis techniques and transjugular intrahepatic portosystemic shunt, as a bridge to liver transplantation. Recently, use of balloon or stent angioplasty techniques has been reported for treatment of this condition. In this article, we report and discuss a case of BCS by membranous obstruction in the hepatic vein outflow tract, with middle hepatic vein thrombosis, in a 24-year-old patient. The treatment chosen and employed was transjugular balloon angioplasty, which achieved satisfactory results and good clinical evolution.

Effects of patent accessory hepatic vein in the treatment of Budd-Chiari syndrome with hepatic vein occlusion / 中华普通外科杂志

Objective:To evaluate the clinical significance of patent accessory hepatic vein (AHV) in treatment of Budd-Chiari syndrome (BCS) with hepatic vein occlusion.Methods:The clinical data of 21 BCS patients treated from Jan 2010 to June 2019 were retrospectively analyzed. All patients underwent AHV and IVC venography.Results:Angiography showed that the diameter of AHV was 6-13mm after the procedure, the angle between AHV and the distal end of IVC was (106.9±27.7)°, and 57.1% of the AHV were opened at the right side of IVC, 9.5% at the front, and 1.4% at the left side, respectively. The technical success rate was 100%. Thirteen patients with AHV disease underwent balloon dilatation angioplasty, and their preoperative AHV pressure dropped from (41.6±6.4) cm H 2O to (22.2±5.5) cm H 2O ( t=11.966, P<0.01). The preoperative AHV and intrahepatic IVC pressure decreased from (29.1±3.3) cm H 2O to (19.1±8.8) cm H 2O ( t=8.136, P<0.01) and from (25.5±6.1) cm H 2O to (13.8±4.0) cm H 2O ( t=5.536, P<0.01), respectively. All patients were of no symptom during follow up for 6 months and ultrasound showed that the patency rate of original lesion was 100%. Conclusion:A patent AHV helps alleviate the symptoms and blood congestion of BCS patients with hepatic vein obstruction.

Use of three-dimensional visualization technique in diagnosis and interventional treatment of Budd-Chiari syndrome presenting with inferior vena cava obstruction and dangerous collateral branches / 中华肝胆外科杂志

Objective:To study the use of three-dimensional (3D) visualization in diagnosis and interventional treatment of patients with Budd-Chiari syndrome (BCS) presenting with inferior vena cava obstruction and dangerous collateral branches.Methods:The data of 28 patients with BCS presenting with inferior vena cava obstruction and dangerous collateral branches treated at the Affiliated Hospital of Xuzhou Medical University from September 2018 to January 2021 were retrospectively analyzed. There were 11 males and 17 females with a mean age of 49.0 years. Enhanced MR images of these 28 patients were used to build 3D visualization of inferior vena cava. Anteroposterior and left lateral digital subtraction angiography (DSA) of inferior vena cava were performed. The inferior vena cava of these patients was recanalized under guidance of 3D visualization, and patency of inferior vena cava was determined on follow up.Results:3D visualization of inferior vena cava was successfully constructed in all the 28 patients, and 51 dangerous collateral branches were displayed. One, 2, 3 and 4 dangerous collateral branches were found in 13, 8, 6 and 1 patients, respectively. The average angle between the preoperative planning puncture route and the long axis of the proximal end of inferior vena cava was 22.2°. The orifices and courses of the dangerous collaterals and the shape of inferior vena cava could be clearly displayed on 3D visualization in all the 28 patients (100.0%), which were significantly better than the 6 patients (21.4%) using DSA obtained in the anteroposterior and left lateral positions (χ 2=20.045, P<0.05). The inferior vena cava was successfully recanalized in all the 28 patients without complications. On follow up of these patients for 2 to 30 months (mean 18.4 months), the inferior vena cava was patent in 25 patients. Three patients developed inferior vena cava re-obstruction at 3, 4 and 14 months after interventional treatment, respectively. Conclusion:3D visualization was useful in the diagnosis and interventional treatment of patients with BCS presenting with inferior vena cava obstruction and dangerous collateral branches.

Effect of hepatic volume change on clinical practice in patients with extensive hepatic vein occluded Budd-Chiari Syndrome treated with TIPS / 中华肝胆外科杂志

Objective:To investigate the changes of liver volume and liver function in patients with extensive hepatic vein occluded Budd-Chiari syndrome (BCS) treated with transjugular intrahepatic portosystem shunt (TIPS).Methods:The clinical data of 29 BCS patients from Affiliated Hospital of Xuzhou Medical University during March 2016 to June 2019 were retrospectively collected and analyzed. The BCS was caused by extensive hepatic vein occlusion and patients were treated with TIPS. Pre- and postoperative abdominal CT/MRI images were collected and analyzed, and hepatic volume was measured with 3D-reconstruction. The liver volume and liver function during before and post the surgery were also collected and analyzed with preoperative value.Results:Patients including 8 males and 21 females, aged (33.3±6.3) years, were enrolled in this study. TIPS was successfully performed in all patients, with a technical success rate 100%. No serious complications related to TIPS occurred. Patients were followed up for 12-33 months (median, 16 months). Compared with preoperative [(2 124.6±420.9) cm 3] , the hepatic volume of time points after operation [1 week: (1 926.3±372.3) cm 3; 3 months: (1 480.6±183.1) cm 3; 6 months: (1 461.9±153.0) cm 3; 12 months: (1 469.3±148.5) cm 3] were all significantly reduced, and the differences were statistically significant ( P<0.05). Compared with preoperative values, the hepatic function indexes at each time point after operation were significantly improved ( P<0.05). The complete remission rate of ascites was 96.4% (27/28), 100.0% (28/28) and 100.0% (28/28) at 3, 6 and 12 months, respectively. Conclusion:The extensive hepatic vein occlusive BCS patients were benefit from TIPS therapy. Six months after operation, the hepatic volume and the hepatic function returned to normal level.

Síndrome de Budd-Chiari: etiología, manejo y resultados en una cohorte de 35 pacientes del Hospital Pablo Tobón Uribe / Budd-Chiari Syndrome: Etiology, management, and outcomes in a study of 35 patients at the Hospital Pablo Tobón Uribe

Resumen El síndrome de Budd-Chiari (Budd-Chiari syndrome, BCS) es una entidad rara, definida por la obstrucción del flujo venoso del hígado y corresponde a una condición heterogénea tanto en presentación como en manejo terapéutico. A continuación, presentamos un estudio retrospectivo de pacientes con diagnóstico de BCS, atendidos por el grupo de hepatología del el Hospital Pablo Tobón Uribe. Los resultados se expresan como proporciones en las variables categóricas y como medias y rangos para las variables continuas. La edad promedio de presentación fue de 37,7 años y no hubo preponderancia por el género. Los principales síntomas fueron dolor abdominal (91,4 %), ascitis (71,4 %) y hepatomegalia (60 %). Asimismo, 11 pacientes (31,4 %) presentaron hipercoagulabilidad primaria, mientras que en 7 (20 %) se encontró algún síndrome mieloproliferativo. Además, 6 (17,1 %) tenían neoplasias extrahepáticas: 3 con compresión mecánica y 3 por hipercoagulabilidad asociada; solo en 1 caso se documentaron membranas en la vena cava inferior. Y en 5 casos no fue posible determinar la causa de trombosis. De igual forma, 32 pacientes (91,4 %) recibieron anticoagulación como primer manejo; solo 4 sujetos fueron remitidos a procedimientos: 2 a angioplastia, 1 a derivación portosistémica intrahepática transyugular (Transjugular Intrahepatic Portosystemic Shunt, TIPS) y 1 a trasplante ortotópico hepático (TOH). Luego de ello, 17 (48,5 %) recibieron procedimientos invasivos como segunda línea: 8 (22,8 %) se condujeron a angioplastia y endoprótesis (stent), 5 (14,29 %) a TIPS y 4 necesitaron TOH. Así, los 6 pacientes que se llevaron a TIPS no necesitaron trasplante hepático. El BCS continúa siendo una condición poco frecuente que afecta la salud de personas jóvenes, tanto hombres como mujeres, con una presentación clínica heterogénea. Sin embargo, en la mayoría de los casos ocurre por una causa desencadenante clara, entre las cuales predominan las trombofilias y los síndromes mieloproliferativos. El uso de medidas terapéuticas invasivas, especialmente de la selección temprana de los enfermos que se benefician de TIPS, ha cambiado la historia natural y el pronóstico de este grupo de pacientes.

Abstract Budd-Chiari syndrome (BCS) is a rare disorder characterized by the obstruction of the veins of the liver. Both its presentation and therapeutic management are heterogenous. The following is a retrospective study of patients diagnosed with BCS treated by the hepatology service at the Hospital Pablo Tobón Uribe. The results of the categorical variables are expressed as proportions and the continuous variables as means and ranges. The average age of onset of this disease was 37.7 years and it was not predominant in neither sex. The most common symptoms were abdominal pain (91.4%), ascites (71.4%) and hepatomegaly (60%). Out of 35 patients, 11 (31.4%) had primary hypercoagulability, 7 (20%) had some myeloproliferative disorder, 6 (17.1%) had extrahepatic malignancies -3 with mechanical compression and 3 due to associated hypercoagulability-, and 1 case had a membranous obstruction of the inferior vena cava. 32 patients (91.4%) received anticoagulation as first-line therapy and only 4 were taken to surgery: 2 to angioplasty, 1 to transjugular intrahepatic portosystemic shunt (TIPS) and 1 to orthotopic liver transplantation (OLT). On the other hand, 17 (48.5%) individuals underwent invasive procedures as a second-line therapy, of which 8 (22.8%) underwent angioplasty and stenting, 5 (14.29%) underwent TIPS and 4 needed OLT. The 6 patients who were taken to TIPS did not need liver transplants. Budd-Chiari syndrome remains a rare condition affecting the health of young men and women alike, with a heterogeneous clinical presentation but, in most cases, with a clear trigger where thrombophilic and myeloproliferative disorders predominate. The use of invasive therapeutic measures, especially the early selection of patients who may benefit from TIPS, has changed the natural course and prognosis of this group of patients.