Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

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.

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.

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.

Terapia endovascular de enfermedades trombóticas hepáticas / Endovascular management of thrombotic liver disease

Budd-Chiari syndrome and porto-spleno-mesenteric thrombosis are less prevalent patologies, but related to high morbimortality, that present in context of chronic hepatopathies, myeloproliferative neoplasms and other prothrombotic conditions. Review of local experience and of current available literature of endovascular management of last five years was made. In relation to Budd-Chiari syndrome, early anticoagulation with low molecular weight heparines and transition to oral anticoagulation with K vitamin antagonists was recomended, also managing subyacent pathology, identifying and treating portal hypertension signs and endovascular therapy with angioplasty or Transjugular Intrahepatic Portosystemic Shunt (TIPS), depending on the thrombus extension. For portal vein thrombosis the same scheme was recommended, with emphasis in treating portal hypertension signs in chronic cases. Endovascular approach includes localized thrombolisis, thrombectomy and TIPS in previous non-effective therapy. We propose a management algorithm of these diseases.

El síndrome de Budd-Chiari y la trombosis venosa porto-espleno-mesentérica son patologías de baja prevalencia, pero de gran morbimortalidad asociada, que se presentan en contexto de hepatopatías crónicas, neoplasias mieloproliferativas y otras condiciones protrombóticas. Se revisó casuística local y literatura disponible del abordaje endovascular de estas patologías en los últimos cinco años. En relación al síndrome de Budd-Chiari, se recomienda la anticoagulación precoz con heparinas de bajo peso molecular y traslape con antagonistas de vitamina K, tratar la patología subyacente, identificar y tratar signos de hipertensión portal y realizar terapia endovascular con angioplastia o instalación de Shunt portosistémico transyugular (TIPS) según la extensión del compromiso. Se recomienda un esquema similar para la trombosis porto-espleno-mesentérica, con énfasis en manejo de hipertensión portal en los casos crónicos. El abordaje endovascular incluye la trombolisis localizada, trombectomía y TIPS en caso de fracaso del tratamiento previo. Se propone un algoritmo de manejo de dichas patologías.

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.

Tratamento da síndrome de Budd-Chiari / Treatment of the Budd-Chiari syndrome

Supressão da drenagem venosa hepática com ou sem o comprometimento da veia cava resulta na Síndrome de Budd-Chiari (SBC). Neste artigo os autores discutem o tratamento e aspectos controversos publicados na literatura médica

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

Hipertensión cava-inferior por empiema tabicado / Inferior vena cava hypertension due to an empyema thoracis

Hemos presentado una rara observación de compresión de la vena cava inferior en sus segmentos inter-hepato-cardíaco y retro-hepático por empiema pleural derecho tabicado; que determinó la existencia de un Sindrome de Budd-Chiari agudo reversible. Los procedimientos de estudio efectuado (Rx de torax, neumoperitoneo y Rx de abdomen, ecografía y T.A.C.) no permitieron establecer el diagnóstico etiológico, sin embargo la T.A.C. mostró la compresión de la vena cava en el sector indicado. Fue la laparotomía exploradora que determinó la existencia de una hepatomegalia de éstasis basculada hacia la izquierda, asi como la existencia de un diafragma convexo. La toracotomía permitió drenar un importante empiema tabicado a presión que ocupaba la mitad inferior de la cavidad pleural. El paciente tuvo buen post operatorio. A propósito del caso presentado y como no se encontraron citas bibliográficas al respecto, se analizaron todos los procesos descriptos que afectan a la vena cava inferior en el sector mencionado