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.

Chinese multidisciplinary collaborative expert consensus for the diagnosis and treatment of Budd-Chiari syndrome (2021 version) / 中华外科杂志

Budd-Chiari syndrome (B-CS) is a complicated hepatic vascular disease caused by hepatic venous outflow obstruction.There are significant differences in the pathogenesis and treatment of B-CS between China and Western countries.Given the characteristics of B-CS in our country,Budd-Chiari Syndrome and Hepatic Vascular Diseases Professional Committee of Chinese Research Hospital Association organizes domestic experts in this field to formulate the "Chinese multidisciplinary collaborative expert consensus for the diagnosis and treatment of Budd-Chiari syndrome（2021 version）".This consensus elaborates the research status of epidemiology,pathogenesis,disease classification,clinical manifestations,diagnosis and treatment of B-CS in China.Conducting basic research on pathogenesis and clinical research with high level evidence are important work direction in the future.This consensus is expected to provide guidance for clinicians to make optimal therapeutic schedules,so as to further standardize and improve the comprehensive diagnosis and treatment and basic research level of B-CS in China.

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.

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.

Manejo de pacientes con síndrome de Budd-Chiari. Revisión de tema / Treatment of patients with Budd-Chiari syndrome. Literature review

El síndrome de Budd-Chiari (SBC), descrito en 1845, se define como la obstrucción del flujo venoso hepático en ausencia de enfermedad cardíaca o pericárdica. En Colombia no se tienen datos epidemiológicos claros de esta patología, la cual alrededor del mundo se considera poco frecuente. Se diagnostica al demostrar la obstrucción del flujo de las venas hepáticas. Tiene diversas manifestaciones clínicas como fiebre, ascitis, dolor abdominal y circulación colateral, entre otras. En ciertos casos es asintomática y en su gran mayoría se acompaña de patologías protrombóticas. El manejo inicial depende de la condición del paciente; sin embargo, se ha propuesto el manejo escalonado, donde se inicia con anticoagulación, se continúa con angioplastia, luego con desvío portosistémico intrahepático transyugular (TIPS), y se termina con trasplante hepático. El pronóstico depende de un diagnóstico precoz y un tratamiento adecuado. En las mejores circunstancias se alcanza una sobrevida a cinco años en el 90% de los casos, mientras que en ausencia de manejo, la tasa de mortalidad a un año alcanza el mismo porcentaje.

Budd-Chiari syndrome (SBC), described in 1845, is defined as the obstruction of hepatic venous flow in the absence of heart or pericardial disease. In Colombia there are no clear epidemiological data of this pathology, that around the world is considered rare. It is diagnosed by demonstrating the obstruction of the flow of the hepatic veins. It has various clinical manifestations such as fever, ascites, abdominal pain and collateral circulation, among others. In certain cases, it is asymptomatic but in the great majority it is accompanied by prothrombotic pathologies. Initial management depends on the patient's condition; however, staggered management has been proposed, beginning with anticoagulation, continuing with angioplasty, then with transjugular intrahepatic portosystemic shunt (TIPS), and finally, with liver transplantation. The prognosis depends on an early diagnosis and proper treatment. In the best circumstances, a five-year survival is achieved in 90% of cases, while in the absence of treatment, the one-year mortality rate reaches the same percentage.

Síndrome de Budd-Chiari de aparición en el puerperio: reporte de caso y revisión de la literatura / Case report and literature review of Budd-Chiari syndrome during the puerperium

Resumen El síndrome de Budd-Chiari es una entidad definida como la obstrucción al flujo sanguíneo en el tracto de salida hepático. Esta obstrucción se puede localizar desde las pequeñas venas hepáticas hasta la entrada de la vena cava inferior a la aurícula derecha. En la mayoría de los casos, se ocasiona por una trombosis endoluminal conocida como Budd-Chiari primario y secundario, cuando ocurre por una compresión extrínseca asociada a las lesiones que ocupan espacio como malignidad. Por tanto, los estados trombofílicos hereditarios se conocen como los principales factores de riesgo. El embarazo y el puerperio, al ser estados hipercoagulables, se pueden asociar al síndrome de Budd-Chiari, sin embargo, la prevalencia reportada en la literatura es muy variable dependiendo de la población estudiada. En Colombia no hay estudios de incidencia o prevalencia de la enfermedad; existen varios reportes de caso, pero no relacionados con el embarazo. Reportamos el caso de una paciente, que 12 semanas posparto presentó un síndrome de Budd-Chiari; además presentamos el manejo ofrecido, la evolución clínica y una revisión de la literatura de los casos asociados con el embarazo.

Abstract Budd-Chiari syndrome is defined as obstruction of hepatic blood outflow. This obstruction can be located anywhere from the small hepatic veins at the entrance of the inferior vena cava to the right atrium. Most cases are primary Budd-Chiari syndrome which is caused by endoluminal thrombosis. Secondary Budd-Chiari syndrome occurs as the result of extrinsic compression associated with space-occupying lesions such as malignant tumors. Hereditary thrombophilic states are the main risk factors, but since pregnancy and the puerperium are hypercoagulable states, they can be associated with Budd-Chiari syndrome. Nevertheless, the prevalence of this type of case in the literature varies according to the population studied. There have been no studies on the incidence or prevalence of this disease in Colombia. The small number of case reports here have not been related to pregnancy. We report the case of a patient who developed Budd-Chiari syndrome 12 weeks postpartum. Our report includes management and clinical evolution as well as a review of the literature of cases associated with pregnancy.

Síndrome de Budd-Chiari asociado a síndrome antifosfolípido / Budd-Chiari syndrome associated to antiphospholipid syndrome / Síndrome de Budd-Chiari associado à síndrome antifosfolípidica

Introducción: el síndrome de Budd-Chiari es una entidad rara definida por la obstrucción al flujo de salida venoso hepático. Se asocia frecuentemente a un estado protrombótico. El diagnóstico y tratamiento oportuno representan un reto para el médico clínico. El objetivo del presente trabajo es reportar un caso clínico de síndrome de Budd-Chiari secundario a síndrome antifosfolípido, asociación infrecuente en la literatura. Caso clínico: mujer de 31 años. Ascitis de seis años de evolución, actualmente refractaria. Repercusión general en el último año. Examen físico: lúcida, ictericia universal, hepatomegalia dolorosa, esplenomegalia y ascitis a tensión. Laboratorio: hepatograma con patrón colestásico. Colinesterasa y tasa de protrombina descendidas. Ecografía Doppler abdominal: ascitis severa, hepatomegalia irregular, ecogenicidad aumentada. Esplenomegalia. Obstrucción de vena suprahepática media, estrechamiento de vena suprahepática derecha. Flujo escasamente fásico de baja velocidad, invertido en algunas ramas, hepatófugo. Fibrogastroduodenoscopía: várices esofágicas grado III. Se planteó ascitis secundaria a hipertensión portal, probable síndrome de Budd-Chiari. Los anticuerpos IgM beta2 glicoproteína 1 fueron positivos. Diagnóstico de síndrome antifosfolípido. Se realizó trasplante hepático con buena evolución posterior. Discusión y conclusiones: el síndrome de Budd-Chiari es más frecuente en mujeres entre los 30 y 50 años. Debe considerarse como causa de enfermedad hepática, fundamentalmente cuando no existe otra causa evidente. Los estados protrombóticos que más se asocian al síndrome de Budd-Chiari en Occidente son las enfermedades hematológicas. El tratamiento debe ser individualizado según la presentación clínica. El trasplante hepático se plantea como medida de salvataje cuando el resto de los tratamientos han fracasado.

Introduction: [/sectitle][p]Budd-Chiari is a rare condition characterized by the occlusion of the hepatic vein flow out. It is frequently associated to a pro-thrombotic state. Diagnosis and treatment constitute a challenge for clinical doctors. This study aims to report a clinical case of Budd-Chiari syndrome secondary to an antiphospholipid syndrome, a rather unusual association in literature.[/p] Clinical case: 31 year old woman, with 6 years of evolution ascites, evidencing a general affection in the last year. Clinical examination: lucid, universal hyperbilirubinemia (jaundice), painful hepatomegaly, splenomegaly and tension ascites. Laboratory tests: hepatogram with cholestatic pattern, cholinesterase, and reduced prothrombin rate. Abdominal Doppler revealed: severe ascites, irregular hepatomegaly, increased echogenicity. Splenomegaly. Occlusion of the middle suprahepatic vein, narrowing of the right suprahepatic vein. Llow speed scarcely phasic flow, inverse in a few branches, hepatofugal. Fibrogastroduodenoscopy: esophagic varices grade III. Ascites secondary to portal hypertension was suspected, probable Budd Chiari syndrome. Antibodies IgM â2glicoprotein 1 were positive. Diagnosis of antiphospholipid syndrome. Liver transplant was performed with a good evolution of the patient. Discussion and conclusions:Budd-Chiari syndrome is more frequent in women between 35 and 50 years old. It needs to be regarded as a cause of liver conditions, in particular when there is no other evident cause. Hematologic diseases are the prothrombotic states more frequently associated to Budd-Chiari syndrome in the western world. Treatment must be based on the clinical presentation. Liver transplant is a rescue/salvage measure when all other treatments fail.

Introdução: a síndrome de Budd-Chiari é uma entidade rara definida pela obstrução do fluxo de saída venoso hepática. Frequentemente está associado a um estado protrombótico. O diagnóstico e o tratamento oportuno são um desafio para o médico clínico. O objetivo deste trabalho é descrever um caso clínico de síndrome de Budd-Chiari secundário à síndrome antifosfolípidica, uma associação pouco frequente na literatura. Caso clínico: mulher de 31 anos. Ascite com seis anos de evolução, atualmente refrataria. Repercussão geral no último ano. Exame físico: lúcida, icterícia universal, hepatomegalia dolorosa, esplenomegalia e ascite sob tensão. Laboratório: hepatograma com padrão colestásico. Colinesterase e taxa de protrombina diminuídas. Ultrassonografia Doppler abdominal: ascite severa, hepatomegalia irregular, ecogenicidade aumentada. Esplenomegalia. Obstrução de veia supra-hepática média, estreitamento de veia supra-hepática direita. Fluxo escassamente fásico de baixa velocidade, invertido em algumas ramas, hematófago. Fibrogastroduodenoscopia: varizes esofágicas de grau 3. Diagnóstico presuntivo: ascite secundaria à hipertensão portal, provável síndrome de Budd-Chiari. Anticorpos IgM alfa 2glicoproteína 1 positivos. Diagnóstico de síndrome antifosfolípido. Foi realizado um transplante hepático com boa evolução.

Tratamiento endovascular del síndrome de Budd-Chiari, a propósito de un caso / Endovascular management for Budd-Chiari syndrome: A case report

El síndrome de Budd-Chiari consiste en la oclusión de la circulación de salida venosa del hígado, desde las sinusoides hepáticas hasta la unión de la vena cava inferior con la aurícula derecha. Esta entidad es muy rara. Las causas son primarias, obstrucción congénita de la vena cava o trombosis espontánea, o secundarias, compresión extrínseca. Si no se trata la obstrucción esta enfermedad, lleva a cirrosis hepática. Se presenta un caso del síndrome de Budd-Chiari de causa primaria por obstrucción membranosa, tratado por vía endovascular de manera exitosa y con resolución de todos los síntomas. Es el primer reporte en Colombia de este tipo de tratamiento (AU)

Budd-Chiari syndrome refers to the occlusion of the venous outflow of the liver, from the hepatic sinusoids to the junction of the inferior vena cava with the right atrium. This entity is very rare. The causes can be divided in primary (congenital vena cava obstruction or spontaneous thrombosis), and secondary (extrinsic compression). If the obstruction is not treated, leads to liver cirrhosis. In this article, we present a case of Budd-Chiari syndrome, primary due to membranous obstruction, successfully managed by the endovascular route with resolution of all symptoms. To our knowledge, this is the first case reported in Colombia utilizing this type of treatment (AU)

Segmental Liver Stiffness Evaluated with Magnetic Resonance Elastography Is Responsive to Endovascular Intervention in Patients with Budd-Chiari Syndrome

OBJECTIVE: To assess segmental liver stiffness (LS) with MRI before and after endovascular intervention in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: Twenty-three patients (13 males and 10 females; mean age, 42.6 ± 12.6 years; age range, 31–56 years) with BCS as a primary liver disease were recruited for this study. Two consecutive magnetic resonance elastography (MRE) examinations were performed before the endovascular treatment. Fifteen patients who underwent endovascular intervention treatment also had follow-up MRE scans within three days after the procedure. LS was measured in three liver segments: the right posterior, right anterior, and left medial segments. Inter-reader and inter-exam repeatability were analyzed with intraclass correlation coefficients (ICCs) and Bland-Altman analysis. Segmental LS and clinical characteristics before and after the intervention were also compared. RESULTS: Within three days of the endovascular intervention, all three segmental LS values decreased: LS of the right posterior segment = 7.23 ± 0.88 kPa (before) vs. 4.94 ± 0.84 kPa (after), LS of the right anterior segment = 7.30 ± 1.06 kPa (before) vs. 4.77 ± 0.85 kPa (after), and LS of the left medial segment = 7.22 ± 0.87 kPa (before) vs. 4.87 ± 0.72 kPa (after) (all p = 0.001). There was a significant correlation between LS changes and venous pressure gradient changes before and after treatments (r = 0.651, p = 0.009). The clinical manifestations of all 15 patients significantly improved after therapy. The MRE repeatability was excellent, with insignificant variations (inter-reader, ICC = 0.839–0.943: inter-examination, ICC = 0.765–0.869). Bland-Altman analysis confirmed excellent agreement (limits of agreement, 13.4–19.4%). CONCLUSION: Segmental LS measured by MRE is a promising repeatable quantitative biomarker for monitoring the treatment response to minimally invasive endovascular intervention in patients with BCS.

Long-term outcomes after stent insertion in patients with early and late hepatic vein outflow obstruction after living donor liver transplantation

PURPOSE: The purpose of this study was to describe the long-term effects of stenting in patients with hepatic venous outflow obstruction (HVOO), who underwent living donor liver transplantation (LDLT). METHODS: Between January 2000 and December 2009, 622 adult patients underwent LDLT at our hospital, and of these patients, 21 (3.3%) were diagnosed with HVOO; among these patients, 17 underwent stenting. The patients were divided into early or late groups according to the time of their HVOO diagnoses (cutoff: 60 days after liver transplantation). RESULTS: The median follow-up period was 54.2 months (range, 0.5–192.4 months). Stent insertion was successful in 8 of 10 patients in the early group and 6 of 7 in the late group. The 5-year primary patency rates were 46% and 20%, respectively. In both groups, patients with recurrent HVOO at the beginning showed kinking confirmed by venography. Patients who carried their stents for more than 3 years maintained long-term patency. There was no significant difference in spleen size between groups; however, when the groups were compared according to whether they maintained patency, spleens tended to be smaller in the patency-maintained group. CONCLUSION: Unlike stenosis, if kinking is confirmed on venography, stenting is not feasible in the long term for patients with LDLT.

Magnetic Resonance Venography Findings of Obstructed Hepatic Veins and the Inferior Vena Cava in Patients with Budd-Chiari Syndrome

OBJECTIVE: This study aimed to illustrate the magnetic resonance venography (MRV) manifestations of obstructed hepatic veins (HVs), the inferior vena cava (IVC), and accessory hepatic veins (AHVs) in patients with Budd-Chiari syndrome (BCS) and to evaluate the visualization capacity of MRV in the diagnosis of BCS. MATERIALS AND METHODS: Fifty-two patients with chronic BCS were included in this study. All patients were examined via MRV performed with a 3T system following injections of gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA) or Gd-ethoxibenzyl-DTPA. HV and IVC lesions were classified, and their characteristics were described. HV cord-like occlusions detected via MRV were compared using ultrasonography (US). Digital subtraction angiography (DSA) was performed as a contrast in the MRV detection of IVC lesions. The HVs draining collaterals, mainly AHVs, were carefully observed. HV lesions were classified as segmental stenosis, segmental occlusion, membranous stenosis, membranous occlusion, cord-like occlusion, or non-visualized. Except for patent IVCs, IVC lesions were classified as segmental occlusion, segmental stenosis, membranous occlusion, membranous stenosis, and hepatomegaly-induced stenosis. RESULTS: All patients (52/52, 100%) showed HV lesions of different degrees. MRV was inferior to US in detecting cord-like occlusions (6 vs. 19, χ2 = 11.077, p < 0.001). Dilated AHVs, including 50 (50/52, 96.2%) caudate lobe veins and 37 (37/52, 71.2%) inferior HV and AHV lesions, were well-detected. There were no significant differences in detecting segmental lesions and thrombosis between MRV and DSA (χ2 = 0.000, p1 = 1.000, p2 = 1.000). The capacity of MRV to detect membranous lesions was inferior to that of DSA (7 vs. 15, χ2 = 6.125, p = 0.013). CONCLUSION: In patients with BCS, MRV can clearly display the lesions in HVs and the IVC, as well as in AHVs, and it has diagnostic and therapeutic value.

Stasis Dermatitis Associated with Budd-Chiari Syndrome / 대한피부과학회지

No abstract available.

Budd-Chiari syndrome secondary to catheter-associated inferior vena cava thrombosis / Síndrome de Budd-Chiari secundária a trombose de veia cava inferior associada a cateter

Abstract Introduction: Patients with chronic kidney disease (CKD) are at increased risk for thrombotic complications. The use of central venous catheters as dialysis vascular access additionally increases this risk. We describe the first case of Budd-Chiari syndrome (BCS) secondary to central venous catheter misplacement in a patient with CKD. Case report: A 30-year-old female patient with HIV/AIDS and CKD on hemodialysis was admitted to the emergency room for complaints of fever, prostration, and headache in the last six days. She had a tunneled dialysis catheter placed at the left jugular vein. The diagnosis of BCS was established by abdominal computed tomography that showed a partial thrombus within the inferior vena cava which extended from the right atrium to medium hepatic vein, and continuing along the left hepatic vein. Patient was treated with anticoagulants and discharged asymptomatic. Discussion: Budd-Chiari syndrome is a rare medical condition caused by hepatic veins thrombosis. It can involve one, two, or all three of the major hepatic veins. It is usually related to myeloproliferative disorders, malignancy and hypercoagulable states. This case calls attention for inadvertent catheter tip placement into hepatic vein leading to this rare complication. Conclusion: Assessment of catheter dialysis tip location with radiological image seems to be a prudent measure after each procedure even if the tunneled dialysis catheter has been introduced with fluoroscopy image.

Resumo Introdução: Pacientes com doença renal crônica (DRC) apresentam risco aumentado de complicações trombóticas e o uso de cateter venoso central para realização de hemodiálise aumenta este risco. Nós descrevemos um caso de síndrome de Budd-Chiari (SBC) causado pelo mal posicionamento de um cateter de diálise em um paciente com DRC e, para nosso conhecimento, este é o primeiro caso relatado na literatura. Caso clínico: Paciente feminina, 30 anos, com diagnóstico de HIV/SIDA e DRC em hemodiálise foi admitida na emergência com queixas de febre, prostração e cefaleia há 6 dias. Ela apresentava um cateter de diálise tunelizado implantado 7 dias antes na veia jugular esquerda. O diagnóstico de SBC foi realizado por tomografia computadorizada abdominal que mostrava um trombo no interior da veia cava inferior o qual estendia-se desde o átrio direito até a veia hepática esquerda. O cateter foi removido e a paciente foi anticoagulada. A paciente estava assintomática no momento da alta hospitalar. Discussão: SBC é uma condição clínica rara causada por trombose das veias hepáticas, podendo envolver desde uma até todas as três principais veias. Esta síndrome é em geral associada a desordens mieloproliferativas, a malignidades e a situações de hipercoagulabilidade. Este caso demonstra que o mal posicionamento da ponta do cateter no interior da veia hepática causou esta rara complicação. Conclusão: Realização de exame radiológico para avaliar localização da ponta do cateter de diálise é uma medida prudente após cada procedimento, mesmo nos casos de implante de cateter de diálise tunelizados com fluoroscopia.

Síndrome de Budd-Chiari agudo secundario a cirrosis alcohólica. Manejo intervencionista y descripción de una complicación / Acute Budd-Chiari Syndrome Secondary to Alcoholic Cirrhosis. Interventional Management and Description of a Complication

El síndrome de Budd-Chiari (SBC) es una patología poco frecuente y potencialmente fatal si no se diagnostica y se trata a tiempo. Se describe un caso de SBC secundario a obstrucción de la vena cava inferior intrahepática y venas suprahepáticas izquierda y media, con manejo intervencionista (colocación de endoprótesis en la vena cava) y posterior complicación con migración de la endoprótesis a la arteria pulmonar

Budd-Chiari syndrome (BCS) is an infrequent and potentially fatal disease if not diagnosed and treated early. We describe a case of BCS secondary to obstruction of intrahepatic inferior cava vein and left and middle suprahepatic veins, with interventional management (stent placement in the cava vein) and subsequent complication with stent migration to the pulmonary artery.

Budd-Chiari syndrome with antiphospholipid syndrome and systemic lupus erythematosus in a patient with Klinefelter's syndrome / 영남의대학술지

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.

The first case of paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome treated with complement inhibitor eculizumab in Korea

No abstract available.

Percutaneous transhepatic hepatic venous stenting after extracorporeal hepatic resection and autotransplantation: A case report

We report a case of percutaneous transhepatic stent placement for the treatment of hepatic venous outflow obstruction after extracorporeal hepatic resection and autotransplantation. A 63-year-old woman with a large mass in the liver was asymptomatic with no hepatic virus infection. Because the tumor was unresectable by conventional means, we used extracorporeal hepatic resection and autotransplantation for operation. Two days after surgery, hepatic venous outflow obstruction of the right and right inferior hepatic veins was suspected on computed tomography. After failure of the transjugular approach, hepatic venous stenting was performed successfully via the percutaneoustranshepatic approach.

Budd-Chiari syndrome with antiphospholipid syndrome and systemic lupus erythematosus in a patient with Klinefelter's syndrome / 영남의대학술지

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.