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.

Budd-Chiari syndrome with multiple large regenerative nodules

No abstract available.

Hemorragia digestiva alta como complicación de un síndrome de Budd-Chiari: reporte de un caso / High digestive hemorrhage resulting from a Budd-Chiari syndrome complication: case report

INTRODUCCIÓN: El Síndrome de Budd-Chiari es una obstrucción del drenaje venoso hepático. Las principales etiologías son los síndromes mieloproliferativos y anti-fosfolípido. La clínica que predomina deriva insuficiencia hepática crónica igual que sus complicaciones. El diagnóstico es mediante imágenes o biopsia hepática. El tratamiento va a depender de la causa y tiene una supervivencia de 75 por ciento a diez años. PRESENTACIÓN DEL CASO Mujer de 26 años con antecedentes de Policitemia Vera, Síndrome de Budd-Chiari y aborto espontáneo secundario a trombosis placentaria, consulta en Servicio de Urgencia del Hospital San Juan de Dios de San Fernando por vómitos rojizos asociados a melena. A su ingreso se plantearon los siguientes diagnósticos: hemorragia digestiva alta, anemia severa, anasarca, síndrome de Budd-Chiari, Policitemia Vera e insuficiencia hepática crónica; además de Child-Pugh C. La paciente se hospitaliza en Unidad de Cuidados Intermedios con tratamiento en base a suero fisiológico, transfusiones sanguíneas, omeprazol, vitamina K y furosemida. Endoscopia digestiva alta revela signos de hipertensión portal y várices esofágicas medianas con signo rojo presente. Luego de ocho días de hospitalización cede la hemorragia digestiva y se decide dar el alta, con control en siete días. DISCUSIÓN: El sangrado gastrointestinal es una complicación que alcanza un 10 por ciento a 15 por ciento en pacientes con síndrome de Budd-Chiari, y puede ocurrir en los que están recibiendo terapia anticoagulante, así como en aquellos con hipertensión portal secundaria a insuficiencia hepática crónica.

INTRODUCTION: Budd-Chiari syndrome is an obstruction of hepatic venous drainage. The main causes are myeloproliferative and anti-phospholipid syndromes. The predominant clinical results are from chronic liver failure as well as its complications. Diagnosis is by imaging or liver biopsy. Treatment will depend on the cause and have a survival of 75 percent at ten years. CASE REPORT: A 26 year old woman with history of polycythemia vera, Budd-Chiari syndrome and spontaneous abortion secondary to placental thrombosis, consulted in the Emergency Service in Hospital San Juan de Dios of San Fernando because of red vomits associated with melena. On admission were raised the following diagnoses: Upper gastrointestinal tract hemorrhage, severe anemia, anasarca, Budd Chiari Syndrome, Polycythemia Vera and Chronic Liver failure; Child-Pugh C. The patient was hospitalized on intermediate care unit with treatment based on physiological saline, blood transfusions, omeprazole, vitamin K and furosemide. Upper gastrointestinal endoscopy revealed signs of portal hypertension and esophageal medium varices with red sign. After eight days hospitalized, gastrointestinal bleeding stops, the patient is discharged and control is decided in seven more days. DISCUSSION: Gastrointestinal bleeding is a complication that achieves 10 percent to 15 percent in patients with Budd-Chiari syndrome, and can occur in the ones who are receiving anticoagulant therapy, as well in those with portal hypertension secondary to chronic liver failure.

Acute Hepatitis Like Presentation of Budd Chiari Syndrome in a Child.

The patient presented with progressively increasing ascites and pain abdomen but without any bleeding tendency, or encephalopathy. The initial diagnosis of acute hepatitis was proved wrong by contrast enhanced CT scan of abdomen which showed atretic hepatic veins. Atretic hepatic veins causing Budd Chiari Syndroem (BCS) is a rarity in literature.

Budd-Chiari syndrome in association with Behçet's disease: review of the literature / Síndrome de Budd-Chiari em associação com a doença de Behçet: revisão de literatura

The risk that patients with Behçet's disease may develop various thrombotic complications has been previously described. Although vascular complications from Budd-Chiari syndrome associated with Behçet's disease have been described, the pathogenic mechanisms are still unknown. Severe vascular complications present in Budd-Chiari syndrome associated with Behçet's disease are very common among young male adults. The objective of this study was to review the literature and present the association of Budd-Chiari syndrome with Behçet's disease.

O risco de pacientes com doença de Behçet desenvolverem várias complicações trombóticas já foi descrito. Apesar de complicações vasculares na síndrome de Budd-Chiari associada à doença de Behçet terem sido descritas, os mecanismos patogenéticos ainda são desconhecidos. Complicações vasculares graves presentes na síndrome de Budd-Chiari associada à doença de Behçet são muito mais comuns no adulto jovem do sexo masculino. O objetivo deste trabalho é o de revisar a literatura e apresentar a associação da síndrome de Budd-Chiari com a doença de Behçet.

A Case of Budd-Chiari Syndrome and Superior Mesenteric Vein Thrombosis in Ulcerative Colitis / 대한소화기학회지

Patients with inflammatory bowel disease are at increased risk for thromboembolic complications. Its prevalence rate ranges from 1% to 7% in clinical studies and reaches 39% in autopsy series. The cause of thrombotic complications in inflammatory bowel disease is generally considered to be associated with hypercoagulability. We experienced a case of ulcerative colitis associated with Budd-Chiari syndrome and superior mesenteric vein thrombosis. This rare complication of ulcerative colitis was successfully managed by conventional treatment for ulcerative colitis and anticoagulation therapy.

Percutaneous balloon angioplasty of membranous obstruction of the inferior vena cava.

BACKGROUND: Membranous obstruction of the inferior vena cava is common in African and Asian countries. METHODS AND RESULTS: Between January 1999 and January 2002, 19 patients were prospectively studied. The mean age of the patients was 38+/-6.9 years. All of them had swelling of the abdomen and ankle edema. Five patients (26.3%) had jaundice, 9 (47.3%) had hepatomegaly, and 5 (26.3%) splenomegaly. Ultrasonography could detect the site of obstruction in 18 patients (94.7%). Vena cavography demonstrated obstruction of the inferior vena cava at the level of the diaphragm, with 2 patients (10.5%) having additional intrahepatic obstruction. The mean pressure gradient was 22+/-3.5 mmHg. Seventeen patients underwent balloon angioplasty using a Joseph balloon. The procedure was successful in 15 patients (88.2%). The post-angioplasty mean pressure gradient was 5+/-1.4 mmHg. On follow-up, 3 patients (20%) developed features of restenosis: out of them, 2 underwent successful redilatation. CONCLUSIONS: Balloon angioplasty of membranous obstruction of the inferior vena cava is feasible with a high success rate, without any rupture of the inferior vena cava.

Rapidly Progressing Budd-Chiari Syndrome Complicated by Hepatocellular Carcinoma

Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.

The Usefullness of Percutaneous Transluminal Balloon Angioplasty in the Management of Budd-Chiari Syndrome / 대한간학회지

BACKGROUND/AIMS: Membranous obstruction is the most common cause of Budd-Chiari syndrome in Orientals. Recently, percutaneous transluminal balloon angioplasty (PTBA) has been successfully applied as a treatment of membranous obstruction. We evaluated etiologies and clinical manifestations in our cases and the usefulness of PTBA. METHODS: Twelve cases of Budd-Chiari syndrome were analyzed. RESULTS: 50.3 years was the average age of the cases (ranging from 37 to 67 years). Major symptoms or signs were superficial collateral vessels on the chest or the abdomen in 6 cases, ascites in 3, abdominal pain in 4, hepatomegaly in 4, splenomegaly in 3, melena or hematemesis in 2, and leg edema in 2. Upper gastrointestinal endoscopy showed esophageal varices in 6 cases and two of these 6 cases had gastric varices. Of 8 cases with liver cirrhosis, 4 were classified as Child-Pugh class A and 4 as B. Four patients with cirrhosis had concurrent hepatocellular carcinoma including 1 patient who was HBs Ag positive. Etiologies were membranous obstruction in 11 cases and protein C deficiency in 1 case. The main site of obstruction was IVC in 8 and hepatic vein in 4. PTBA was successfully performed in 8 cases of membranous obstruction. During the mean follow-up period of 27.6 months (12-40 months), there were no reobstructions except in 2 cases. CONCLUSIONS: The most common cause of Budd-Chiari syndrome in our cases was membranous obstruction of IVC. Percutaneous transluminal balloon angioplasty is a very useful treatment method.

Laparoscopic cholecystectomy in patient with portal cavernoma and portal hypertension.

Successful laparoscopic cholecystectomy has been reported in patients with cirrhosis of liver with portal hypertension; the procedure has, however, not been reported in patients with portal vein thrombosis, portal cavernoma and portal hypertension. We report an 18-year-old man with portal hypertension due to portal vein thrombosis and portal cavernoma who had symptomatic gallstone disease and was successfully treated with laparoscopic cholecystectomy.

Síndrome de Budd Chiari como expresión de carcinoma renal / Budd Chiari syndrome how expression of the renal carcinoma

El síndrome de Budd Chiari es la manifestación clínica de la construcción del flujo venoso hepático. Existen diversas causas que producen este síndrome, que dependiendo de la localización y de su forma de presentación será la expresión clínica. El tratamiento se condiciona de acuerdo con los resultados hemodinámicos y los procedimientos por imágenes son de gran ayuda en el diagnóstico. Se presenta el caso de pacientes femenino de 76 años de edad con manifestaciones clínicas de síndrome de Budd Chiari por obstrucción de la vena cava inferior, secundario a la trombosis tumoral de un carcinoma renal

Balloon dilatation of inferior vena cava stenosis in Budd-Chiari syndrome.

Percutaneous balloon angioplasty was used to dilate inferior vena cava (IVC) stenosis in 12 patients with Budd Chiari syndrome. There were seven men and five women, aged 32.8 +/- 8.5 years. Angioplasty was performed using balloon 18-20 mm in diameter. In eleven (91.6%) patients, IVC could be successfully dilated. In these eleven patients, the caval diameter at the site of stenosis increased from 2.3 +/- 1.5 to 13.1 +/- 2.8 mm (p < 0.001), the mean IVC pressure decreased from 28.2 +/- 4.1 to 10.5 +/- 3.4 mmHg (p < 0.001) and the gradient across the stenosis decreased from 23.1 +/- 2.2 to 4.2 +/- 1.9 mmHg (p < 0.001). There was appreciable clinical improvement after angioplasty. On a mean followup of 10.8 (3-18) months four (36.4%) patients had restenosis which could be successfully dilated again. These results suggest that balloon dilatation of inferior vena cava stenosis is safe and effective, however, recurrence is common and needs redilatation.

A case of Budd-Chiari syndrome with high antiphospholipid antibody in a patient with systemic lupus erythematosus

Antiphospholipid syndrome is characterized by recurrent episodes of arterial and venous thrombosis, spontaneous fetal losses, thrombocytopenia and persistently elevated levels of antiphospholipid antibodies. We experienced a case of Budd-Chiari syndrome in a 32-year old female lupus patient who was presented with left leg edema, ascites and esophageal varix. The clinical and laboratory findings were compatible with the cirteria for systemic lupus erythematosus (SLE) and she was found to have anticardiolipin antibody, thrombocytopenia and prolonged partial thromboplastin time. Initially, she was treated with intravenous heparin and uroki nase and she was followed up with warfarin, baby aspirin and steroids.

Pileflebitis séptica criptogénica / Cryptogenic septic pylephlebitis

Introducción. La piliflebitis séptica con trombosis es un proceso infeccioso grave, poco frecuente en Pediatría, generalmente secundario a procesos inflamatorios abdominales y raramente criptogénico. Caso clínico. Se describe el caso de un paciente masculino de diez años de edad, que presentó dolor abdominal difuso, fiebre, vómito, hepatoesplenomegalia dolorosa a la palpación, leucocitosis con neutrofilia y bandemia. Se aislaron Bacteroides spp y Peptostreptococcus spp de hemocultivo y mielocultivo. Por estudios de ultrasonido abdominal, tomografía axial computada y resonancia magnética se diagnosticó trombosis portal y abcesos hepáticos piógenos. No se encontró foco infeccioso que pudiera ser catalogado como el sitio primario de infección que hubiese condicionado la complicación portal y hepática por lo que se consideró el proceso como criptogénico. Conclusiones. La pileflebitis séptica es una patología poco frecuente que debe ser considerada en el diagnóstico diferencial de absceso hepático y en los casos de hepato-esplenomegalia más fiebre en la etapa escolar