RESUMO
Hepatic sinus obstruction syndrome (HSOS) is easy to be misdiagnosed or missed, and there is no unified and effective treatment for it. A patient was considered to have Budd-Chiari syndrome. He underwent a transjugular liver biopsy, and pathological examination revealed HSOS without liver cirrhosis. After the failure of anticoagulation therapy, he successfully received a transjugular intrahepatic portosystemic shunt (TIPS). After discharge, he was followed-up for four years with a good prognosis. G. segetum-induced HSOS can be easily overlooked, especially in patients with underlying liver diseases. When medical therapy fails, TIPS can control ascites and portal hypertension, and the long-term prognosis is optimistic.
Assuntos
Hepatopatias Alcoólicas , Derivação Portossistêmica Transjugular Intra-Hepática , Humanos , Masculino , Hepatopatias Alcoólicas/complicações , Hepatopatia Veno-Oclusiva/diagnóstico , Hepatopatia Veno-Oclusiva/complicações , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Pessoa de Meia-IdadeRESUMO
Budd-Chiari syndrome is one of the post-hepatic causes of portal hypertension and a potential obstruction causes liver fibrosis. In pregnancy, obstruction of hepatic veins could occur due to stenosis or thrombosis. Variceal bleeding is the most fatal complication in pregnancy with co-existing Budd-Chiari syndrome, with 29.4% incidence of abortion and 33.3% perinatal mortality. The aim of this case report was to present the management of non-cirrhotic variceal bleeding in pregnant women with Budd-Chiari syndrome in the early second trimester. We report a pregnant female at 13-14 weeks gestation presented to the hospital with profuse hematemesis. Doppler ultrasonography (USG) was utilized to confirm the diagnosis of Budd-Chiari syndrome-hepatic vein occlusion type in pregnancy. Abdominal USG revealed hepatomegaly with hepatic veins dilation, while endoscopy showed grade IV esophageal varices and grade IV gastric varices. Laboratory results indicated disseminated intravascular coagulation due to hemorrhage. The patient was given strict fluid resuscitation and three packed red cells transfusion to stabilize the hemodynamic. Bleeding was successfully managed by intravenous octreotide, tranexamic acid, and vitamin K. The case highlights that the management of non-cirrhotic variceal bleeding in pregnancy with Budd-Chiari syndrome requires a multidisciplinary approach and regular fetal monitoring to ensure optimal outcomes.
Assuntos
Síndrome de Budd-Chiari , Varizes Esofágicas e Gástricas , Hemorragia Gastrointestinal , Segundo Trimestre da Gravidez , Humanos , Feminino , Síndrome de Budd-Chiari/terapia , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Gravidez , Varizes Esofágicas e Gástricas/terapia , Varizes Esofágicas e Gástricas/complicações , Varizes Esofágicas e Gástricas/etiologia , Hemorragia Gastrointestinal/terapia , Hemorragia Gastrointestinal/etiologia , Adulto , Complicações Cardiovasculares na Gravidez/terapia , Complicações Cardiovasculares na Gravidez/diagnóstico por imagemRESUMO
Myeloproliferative neoplasms (MPNs) are the leading causes of unusual site thrombosis, affecting nearly 40% of individuals with conditions like Budd-Chiari syndrome or portal vein thrombosis. Diagnosing MPNs in these cases is challenging because common indicators, such as spleen enlargement and elevated blood cell counts, can be obscured by portal hypertension or bleeding issues. Recent advancements in diagnostic tools have enhanced the accuracy of MPN diagnosis and classification. While bone marrow biopsies remain significant diagnostic criteria, molecular markers now play a pivotal role in both diagnosis and prognosis assessment. Hence, it is essential to initiate the diagnostic process for splanchnic vein thrombosis with a JAK2 V617F mutation screening, but a comprehensive approach is necessary. A multidisciplinary strategy is vital to accurately determine the specific subtype of MPNs, recommend additional tests, and propose the most effective treatment plan. Establishing specialized care pathways for patients with splanchnic vein thrombosis and underlying MPNs is crucial to tailor management approaches that reduce the risk of hematological outcomes and hepatic complications.
Assuntos
Síndrome de Budd-Chiari , Transtornos Mieloproliferativos , Neoplasias , Trombose , Trombose Venosa , Humanos , Veia Porta , Neoplasias/patologia , Trombose Venosa/genética , Trombose Venosa/complicações , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/genética , Transtornos Mieloproliferativos/complicações , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Trombose/patologia , Mutação , Janus Quinase 2/genéticaAssuntos
Síndrome de Behçet , Síndrome de Budd-Chiari , Hipertensão Pulmonar , Humanos , Síndrome de Behçet/complicações , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/complicações , Hipertensão Pulmonar/etiologia , Feminino , Masculino , Adulto , Fatores de Risco , Pessoa de Meia-IdadeRESUMO
A Japanese man in his 20s was referred to our hospital with a two-month history of abdominal fullness and leg edema. Abdominal computed tomography revealing massive ascites and ostial blockage of the main hepatic veins, and angiographic evaluation demonstrating obstruction of the main hepatic veins yielded a diagnosis of Budd-Chiari syndrome (BCS). Diuretic agents were prescribed for the ascites but failed to provide relief. The patient was referred to our department for further evaluation and treatment. Angiography showed ostial obstruction of the main hepatic veins, with most of the portal hepatic flow draining from an inferior right hepatic vein (IRHV) into the inferior vena cava (IVC) thorough an intrahepatic portal venous and venovenous shunt. Access between the main hepatic veins and IVC was impossible, but cannulation between the IRHV and IVC was achieved. Because of the venovenous connection between the main hepatic vein and the IRHV, metallic stents were placed into two IRHVs to decrease congestion in the hepatic venous outflow. After stent placement followed by balloon expansion, the gradient pressure between the hepatic vein and IVC improved remarkably. The ascites and lower leg edema improved postoperatively, and long-term stent patency (6 years) was achieved.
Assuntos
Síndrome de Budd-Chiari , Masculino , Humanos , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/cirurgia , Veias Hepáticas/diagnóstico por imagem , Veias Hepáticas/cirurgia , Ascite/diagnóstico por imagem , Ascite/etiologia , Ascite/terapia , Stents/efeitos adversos , Edema/complicaçõesRESUMO
INTRODUCTION: In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction. CASE PRESENTATION: We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank. The living donor underwent a laparoscopic-assisted left lateral hepatectomy. The reconstruction of the vena cava took place on the back table and the liver was implanted en bloc with the reconstructed IVC in the recipient. Anticoagulation was immediately restarted after the surgery because of her pro-thrombotic state. Her postoperative course was complicated by a biliary anastomotic leak and an infected biloma. The patient recovered progressively and remained well on outpatient clinic follow-up 32 weeks after the procedure. CONCLUSION: IVC reconstruction using a cryopreserved pulmonary vein graft is a valid option during LDLT for pediatric patients with BCS where reconstruction of the IVC entails considerable challenges. Early referral to a pediatric liver transplant facility with a multidisciplinary team is also important in the management of pediatric patients with BCS.
Assuntos
Síndrome de Budd-Chiari , Transplante de Fígado , Veias Pulmonares , Feminino , Humanos , Criança , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/métodos , Veias Hepáticas/cirurgia , Doadores Vivos , Veia Cava Inferior/cirurgiaRESUMO
Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 µmol/L, direct bilirubin 14 µmol/L, and indirect bilirubin 39 µmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.
Assuntos
Síndrome de Budd-Chiari , Síndrome Hepatopulmonar , Masculino , Humanos , Criança , Adolescente , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/terapia , Síndrome Hepatopulmonar/complicações , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/terapia , Estudos Retrospectivos , Hipóxia/complicações , Oxigênio , Dispneia/complicações , Cianose/complicações , BilirrubinaRESUMO
We report the case of a male patient who had a history of early-onset protein-losing enteropathy, chronic diarrhea, and repeated thrombotic events since early childhood. He developed Budd-Chiari syndrome with consequent acute liver failure that required liver transplantation when he was 12 years old. The initial graft failed to function and he required retransplantation. Steroid-resistant rejection complicated the clinical course after the second transplant. Treatment with antithymocyte globulin stabilized graft function but abdominal symptoms and enteral protein loss persisted. The patient remained dependent on intravenous albumin and immunoglobulin. Extended work-up for thrombophilia was unremarkable. Flow cytometry analysis of the peripheral blood cells revealed an unexplained CD55 deficiency. By sequencing of CD55 and, later, exclusion of alternative rare diseases by whole-exome sequencing, we discovered a novel, likely pathogenic homozygous splice-site variant in CD55 c.578 + 5G>A, NM_000574.4, OMIM 125240. The staining of liver and colon biopsies revealed a lack of CD55 protein expression. After initiation of treatment with eculizumab, the patient achieved and has maintained a complete clinical remission throughout 56 months of follow-up. We recommend testing for CD55 deficiency in patients with protein-losing enteropathy. In addition, CD55 deficiency should be considered in the differential diagnosis of patients with Budd-Chiari syndrome in whom an underlying cause is uncertain.
Assuntos
Síndrome de Budd-Chiari , Transplante de Fígado , Enteropatias Perdedoras de Proteínas , Criança , Humanos , Masculino , Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/tratamento farmacológico , Síndrome de Budd-Chiari/cirurgia , Enteropatias Perdedoras de Proteínas/complicaçõesRESUMO
Splanchnic vein thrombosis (SVT) is an unusual-site venous thromboembolism that includes portal, mesenteric, and splenic vein thrombosis as well as the Budd-Chiari syndrome. SVT is a relatively rare disease (portal vein thrombosis and Budd-Chiari syndrome are, respectively, the most and the least common presentations); roughly onethird of the cases are detected incidentally, and liver cirrhosis and solid cancer represent the main risk factors. Once SVT is diagnosed, careful patient evaluation should be performed to assess the stage, grade, and extension of the thrombosis, as well as the risks and benefits of the anticoagulation regimen. Anticoagulant therapy is effective in SVT treatment and is associated with high rates of vein recanalization, low rates of thrombosis progression or recurrence, and an acceptable rate of bleeding complications. Most available data come from observational studies in patients with liver cirrhosis-related SVT receiving lowmolecularweight heparin or vitamin K antagonists. Data on the use of direct oral anticoagulants are increasing and promising. In selected patients and in specialized centers, interventional procedures may be considered in adjunction to anticoagulation in the cases of mesenteric or extensive SVT, intestinal ischemia, or in the patients whose condition deteriorates despite adequate anticoagulant therapy. In this narrative review, we summarize the available data regarding anticoagulation in patients with SVT, identify specific subgroups of patients who may achieve the greatest benefits from anticoagulant therapy, and provide practical advice for clinicians caring for these patients.
Assuntos
Síndrome de Budd-Chiari , Trombose Venosa , Humanos , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Anticoagulantes/efeitos adversos , Cirrose Hepática/complicações , Fatores de RiscoRESUMO
BACKGROUND AND AIM: Our primary objective is to report the results of the ultrasound (US)-guided revision technique of transhepatic shunt in patients in whom intrahepatic portosystemic shunt was created by the percutaneous or conventional route. Our secondary objective is to investigate whether there is an association between the indication for a portosystemic shunt and the need for post-shunt revision. METHODS: Data from 117 consecutive patients who had a transjugular intrahepatic portosystemic shunt placed percutaneously or conventionally were extracted from hospital electronic medical records and examined those who underwent revision within 11 years and those who did not. US-guided transhepatic shunt revision technique was evaluated in terms of technical success, complications, and patency. In addition, the relationship between etiology and the need for revision was also examined using the chi-square test in three groups. RESULTS: Forty six point two percent of patients who underwent transjugular intrahepatic portosystemic shunt required one or more revisions within 11 years. While patency of the shunt could be established via the transjugular route in 83.3% of revision patients, it was necessary to use the transhepatic route in 16.7%. The technical success rate for the US-guided transhepatic shunt revision method was 100%, and the pressure gradient between the portal and hepatic venous systems decreased below 10 mmHg in all patients at the end of the procedure. CONCLUSION: US-guided transhepatic shunt revision is a safe and effective method where transjugular revision cannot be performed. In addition, the revision rate is significantly higher in patients who have undergone transjugular intrahepatic portosystemic shunt due to Budd-Chiari syndrome compared with other groups.
Assuntos
Síndrome de Budd-Chiari , Hipertensão Portal , Derivação Portossistêmica Transjugular Intra-Hepática , Humanos , Derivação Portossistêmica Transjugular Intra-Hepática/métodos , Síndrome de Budd-Chiari/complicações , Resultado do Tratamento , Estudos RetrospectivosRESUMO
BACKGROUND: Budd-Chiari syndrome (BCS) is associated with infertility and adverse pregnancy outcomes in affected females. Scant literature is available on the effect of an endovascular intervention on fertility and the outcome of future pregnancies in these patients. AIMS: To assess the infertility rates, maternal and fetal outcomes of pregnancy and effect of endovascular intervention in women with BCS. METHODS: In this retrospective analysis, 121 female patients with BCS attending our liver clinic from 2017 to 2020 were included. Demographic details, intervention details, pregnancies - pre- and post-intervention - and fetal outcomes were noted. RESULTS: BCS was diagnosed pre-conception in 58 women (group 1; median age: 22 years), during/after pregnancy, but before completion of family in 39 (group 2; median age: 27 years), and after completion of family in 24 (group 3; median age: 34 years). Median Child-Turcotte-Pugh (CTP) and model for end-stage liver disease (MELD) scores were 7 and 12, respectively. The primary infertility rate was 19.8% (24/121). In group 1, 15 women with primary infertility underwent endovascular intervention with 5/15 (33%) women conceiving subsequently, resulting in four live births and seven abortions. In group 2, five women developed BCS during pregnancy and 11 postpartum; 11/39 had a history of one or more abortions. Overall, 8/34 (23.5%) who underwent endovascular intervention had 4/8 (50%) successful pregnancies. In group 3, no patient had any major complications during past pregnancies. The mode of delivery was vaginal in 88% of cases. No congenital anomaly/major bleeding episodes/decompensation/maternal mortality occurred. CONCLUSIONS: Infertility is common in patients with BCS. Pregnancy is well-tolerated in those with compensated liver disease.
Assuntos
Síndrome de Budd-Chiari , Doença Hepática Terminal , Infertilidade , Gravidez , Humanos , Feminino , Adulto Jovem , Adulto , Masculino , Resultado da Gravidez , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/terapia , Estudos Retrospectivos , Doença Hepática Terminal/complicações , Atenção Terciária à Saúde , Índice de Gravidade de Doença , Infertilidade/complicações , Resultado do TratamentoRESUMO
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.
Assuntos
Síndrome de Budd-Chiari , COVID-19 , Humanos , Síndrome de Budd-Chiari/complicações , Estudos de Coortes , COVID-19/complicaçõesRESUMO
BACKGROUND: Budd-Chiari syndrome (BCS) is a rare vascular disorder of the liver, and acute and secondary BCS is even rarer. CASE PRESENTATION: A 62-year-old man with perihilar cholangiocarcinoma of Bismuth type IIIa underwent right hemi-hepatectomy with caudate lobectomy and pancreatoduodenectomy. Adjuvant chemoradiotherapy was performed due to a positive hepatic ductal margin. Subsequently, the disease passed without recurrence. The patient visited for acute onset abdominal pain at the 32nd postoperative month. Multidetector-row computed tomography (MDCT) showed stenosis of the left hepatic vein (LHV) root, which was the irradiated field, and thrombotic occlusion of the LHV. The patient was diagnosed with acute BCS caused by adjuvant radiotherapy. Although anticoagulation therapy was performed, the patient complained of sudden upper abdominal pain again. MDCT showed an enlarged LHV thrombus and hepatomegaly. The patient was diagnosed with exacerbated acute BCS, and stenting for the stenotic LHV root was performed with a bare stent. Although stenting for the LHV root was very effective, restenosis occurred twice due to thrombus in the existing stent, so re-stenting was performed twice. The subsequent clinical course was acceptable without recurrence or restenosis of the LHV root as of 6 months after the last stenting using a stent graft. CONCLUSION: Although no case of BCS caused by radiotherapy has yet been reported, the present case showed that late side effect of radiotherapy can cause hepatic vein stenosis and secondary BCS.