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: una rara presentación de un cáncer renal / Budd-Chiari syndrome: a rare presentation of renal cancer

El Cáncer Renal se presenta habitualmente como un hallazgo de las imágenes abdominales y cuando se manifiesta clínicamente lo hace a través de los efectos que produce una gran masa abdominal o las metástasis a distancia. En este trabajo se presenta un caso de cáncer renal cuyas manifestaciones clínicas fueron secundarias a la obstrucción de las venas supra hepáticas, es decir un síndrome de Budd-Chiari florido por la presencia de un trombo tumoral en la cava retro hepática. El diagnóstico oportuno y una evaluación adecuada con imágenes permitieron la ejecución de un plan de tratamiento que incluyó un abordaje combinado abdominal y torácico que permitió un tratamiento no solo sintomático sino que potencialmente curativo de un cáncer renal con esta rara presentación.

Renal Cancer usually occurs as a finding of abdominal imaging and when it clinically manifests itself it does so through a large abdominal mass or distant metastases. In this paper we present a case of renal cancer whose clinical manifestations were secondary to obstruction of hepatic veins, a Budd-Chiari syndrome because of the presence of a tumor thrombus in the retro hepatic vena cava. Early diagnosis and appropriate imaging evaluation allowed the implementation of a treatment plan that included a combined abdominal and thoracic approach allowing not only a symptomatic treatment but potentially curative treatment of renal cancer with this rare presentation.

Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report / Doença de Behçet em associação com Síndrome de Budd-Chiari e tromboses múltiplas - Relato de caso

Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in Behçet's disease. The frequency of vascular lesions in Behçet's disease, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Budd-Chiari syndrome is a rare and serious complication of Behçet's disease and implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. We report a case of a 25-year-old man with Behçet's disease that developed Budd-Chiari syndrome. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis.

Doença de Behçet é uma doença inflamatória crônica de etiologia desconhecida, caracterizada clinicamente por ulcerações aftosas orais e genitais recorrentes, uveíte, lesões cutâneas e outras afecções multissistêmicas associadas à vasculite. Diferentes tipos de vasos, predominantemente veias, podem ser afetados na doença de Behçet, causando tromboses venosas superficiais e profundas, aneurismas arteriais e oclusões, com uma frequência em torno de 7 a 29%. Síndrome de Budd-Chiari é uma rara e grave complicação da SB e implica trombose das veias hepáticas e/ou da veia cava inferior intra ou suprahepática. Nós reportamos um caso de paciente masculino com Doença de Behçet que apresentou Síndrome de Budd-Chiari e tromboses múltiplas, cujo diagnóstico foi favorecido pela correlação entre aspectos dermatológicos, histopatológicos, radiológicos e laboratoriais.

Superior sagittal sinus thrombosis and Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria managed with transjugular intrahepatic portosystemic shunt: a case report.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH), caused by somatic mutation of hematopoietic cells, is associated with complement-mediated hemolysis and a hypercoagulable state. Thrombotic complications in this disease are associated with reduced survival. We report a patient with PNH complicated by intracranial venous thrombosis and Budd-Chiari syndrome, who was managed with transjugular intrahepatic portosystemic shunt. CASE PRESENTATION: A 26-year-old man presented with thrombosis of the superior sagittal and right sigmoid sinuses. Initial investigations did not reveal any underlying cause. Nine months later, he developed hepatic venous thrombosis. At this time, Ham test was positive. Flow cytometry confirmed the diagnosis of PNH. The patient was treated with transjugular intrahepatic portosystemic shunt; one episode of stent blockage one month later was managed successfully with balloon dilatation and restenting. CONCLUSION: PNH should be considered in patients with unexplained venous thrombosis. Thrombosis in these patients needs to be managed with prolonged anticoagulation. For Budd-Chiari syndrome in patients with underlying PNH, transjugular intrahepatic portosystemic shunt may be a good option but caution is needed to prevent stent occlusion.

Budd-Chiari syndrome due to antithrombin III deficiency.

Budd-Chiari syndrome is a disease complex with varied etiology and is one of the causes of post-hepatic portal hypertension. We report a 2 year-old boy who presented with Budd-Chiari syndrome due to congenital antithrombin III deficiency, who was managed with an expandable metal stent placed in the inferior vena cava and oral anticoagulation.

Cholangiocarcinoma presenting with recurrent venous thrombosis.

A 38 years female presented with three episodes of venous thrombosis over one year--first in left femoral vein, next in splenic vein causing haematemesis and malena and lastly in inferior vena cava causing Budd-Chiari syndrome. During third episode, endoscopic cholangiopancreatography and guided biopsy established a diagnosis of cholangiocarcinoma at the mid portion of common bile duct. The disease was far advanced with cervical lymph node metastasis.

Budd chiari syndrome: experience at king faisal specialist hospital and research center, Riyadh, Saudi Arabia

Budd-chiari syndrome [BCS] is rare disorder, varied in etiology, inconsistent in presentation, unpredictable in progression, and challenging in therapy. Aim of study: The aim of this study is to review experience of Budd- Chiari syndrome at KFSH, Riyadh; over at ten-year period. In a retrospective study using a computer data search of the medical records from May 1990 to May 2000, 43 patients' charts suspected of BCS were reviewed. 29 patients were found to have BCS, diagnosed by Doppler ultrasound, venography, MRI, or CT with or without liver biopsy. Findings: Of the 29 patients, 18 were male [62.1%] and 11 were female [37.9%]; mean age was 35.4 [range 17-69 years]. Nine patients had Behect's disease seven patients had malignancy [five HCC, one melanoma and one hypernephroma]. Eight patients had antiphospholipid syndrome, two patients one melanoma and one hypernephroma]. Eight patients had antiphospholipid syndrome, two patients had no known cause and three [one related to trauma, the other related to protein C deficiency and the third related nephrotic syndrome]. Nineteen patients presented acutely with abdominal pain, progressive ascites and hepatomegaly. Ten patients had subacute presentation mimicking cirrhosis of liver. Five patients had web in the intrahepatic IVC or ostium HV and the remaining 24 patients had thrombosis of hepatic veins. Five patients received surgical shunts, three had balloon dilatation of webs, 11 had anticoagulant therapy and seven patients had symptomatic treatment. Eleven patients died, six of them had malignancy, two died while awaiting liver transplantation and three died with fulminant hepatic failure. Fourteen patients are still on follow-up. Budd Chiari syndrome in a Saudi Arabia is related to a defined cause in majority of patients [92%]. Behcet's disease is a dominant cause of BCS in Saudi Arabia. This is in contrast to world literature where around 50% of patients are of unknown etiology

Colocación de stent en VCI en síndrome de Budd Chiari

En pacientes con síndrome de Budd-chiari de corta evolución sin signos de cirrosis hepática o fibrosis, se busca disminuir la hipertensión portal, manejar el síndrome de vena cava inferior si está presente y conservar la función hepática brindándole al paciente la posibilidad de un transplante hepático. Se colocaron stents de Gianturco en la vena cava inferior a una paciente con síndrome de Budd-chiari de etiología desconocida, utilizando la técnica percutánea y simultaneamente se dilató con balón de angioplastia la vena suprahepática derecha la cual se encontraba estenosada y de aspecto membranoso. Después del tratamiento hubo disminución inmediata de la ascitis, del edema de miembros inferiores y genitales externos, desaparición del flujo colateral con una evolución satisfactoria y sin complicaciones posteriores al procedimiento. sin embargo posteriormente hubo reaparición de la ascitis la cual fue refractaria al tratamiento

Enfermedad hepática veno-oclusiva asociada a la ingestión de humanrripa (senecio tephrosioides) / Hepatic veno-oclusive disease associated to ingetion of huamanrripa (senecio tephrosioides)

Se reporta un caso de enfermedad veno-oclusiva del hígado (EHVO) en una mujer de 38 años de edad que había consumido ocasionalmente "huamanrripa" (Senecio tephrosioides) durante algunos años como antitusígeno. Fue hospitalizada por presentar dolor abdominal difuso, ictericia y anasarca durante 10 semanas. El estudio histológico de la biopsia hepática mostró marcada congestión a predominio centrolobulillar, focos de necrosis y en algunas áreas, inversión de la arquitectura del lobulillo hepático. En los siguientes 13 meses ha sido hospitalizada en 4 ocasiones por complicaciones de hipertensión portal. Este es el primer caso reportado de enfermedad hepática veno-oclusiva asociada a la ingesta de S. tephrosioides. Es muy probable que esta planta usada en la medicina tradicional peruana contenga alcaloides pirrolizidina, sustancias hepato- y nefrotóxicas que estan presentes en otras especies del género Senecio. Debido a la popularidad actual de las medicinas alternativas se espera un aumento en la incidencia de EHVO

Percutaneous transluminal balloon angioplasty in suprahepatic IVC obstruction--Budd-Chiari syndrome.

Encouraging results of Transluminal Angioplasty for Budd-Chiari Syndrome due to obstruction of Suprahepatic inferior vena cava have been reported in the last decade. We present our experience with two such patients, treated with balloon dilatation producing dramatic clinical improvements and control of disease on follow-up.

Budd Chiari syndrome and pericaval filariasis.

A young girl presented with Budd-Chiari syndrome (BCS) with narrowing of the retrohepatic segment of inferior vena cava (VC). Dorsal cavoatrial bypass (DCAB) was unsuccessful due to thrombotic occlusion of the graft. A limited autopsy revealed occlusion of the retrohepatic segment of IVC and the terminal parts of the three major hepatic veins. Caval occlusion was just above the level of the superior hepatic veins, and caused by a transverse fibrous shelf. An adult filarial worm was found amidst pericaval fibrosis. Filariasis should be included as a possible aetiological factor in chronic BCS.

Operación de blanco Benavides en niños / Blanco Benavides procedure in children

De 1987 a 1990 en el Instituto Nacional de Pediatría (INP) se efectuó la operación de Blanco Benavides en seis niños para eñ tratamiento de várices esofágicas sangrantes con clasificación A, B y C de child Turcotte, en quienes fracasaron la derivación porto sistémica y la escleroterapia, o no estaban indicadas. Hubo cuatro niños y dos niñas entre dos y quince años de edad. La hipertención portal se debió a degeneración cavernomatosa de la porta en cuatro, fibrosis hepática congénita en uno, síndrome de Budd-Chiari en otro. Cinco pacientes presentaron complicaciones postoperatorias, de poca importancia y su evolución posterior fue satisfactoria. Cuatro pacientes viven asintomáticos y sin recurrencia del sangrado. Se les controla con estudios radiológicos y endoscópicos y han disminuido o sesaparecido las várices. Dos pacientes fallecieron, uno en el postoperatorio inmediato y otro por sepsis, un año después. La operación de Blanco Benavides es una alternativa de manejo para niños con hipertensión portal de difícil tratamiento quirúrgico

Portal vein thrombosis in a child with homozygous sickle-cell disease

A 13-year old boy with homozygous sickle-cell (SS) disease died suddenly at home folllowing a short history of abdominal pain. Autopsy revealed venous thrombosis of the hepatic, portal, superior mesenteric and splenic veins. Venous thrombosis is rare in SS disease and thrombosis of mesenteric vessels is most frequently seen in chronic myeloproliferative disorders. Its occurrence in SS disease raises the possibility of a common pathogenesis and adds another pathology to the causes of abdominal painful crisis.