Budd-Chiari Syndrome in Childhood Secondary to Inferior Vena Caval Obstruction

PEDIATRICS ◽  
1979 ◽  
Vol 63 (5) ◽  
pp. 808-812
Author(s):  
Arvind Taneja ◽  
S. K. Mitra ◽  
P. D. Moghe ◽  
P. N. Rao ◽  
N. Samanta ◽  
...  
Keyword(s):  
Inferior Vena ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Venous Outflow ◽  
Massive Ascites ◽  
Inferior Vena Caval ◽  
Uncommon Disease ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow

Budd-Chiari syndrome is an uncommon disease caused by an obstruction to hepatic venous outflow either at the level of the hepatic veins or in the hepatic part of the inferior vena cava. Clinically, it presents with ascites, abdominal pain, hepatomegaly, edema, and occasionally jaundice. The syndrome was first recognised by Lamboran1 in 1842 and later described by Budd2 in 1846 and Chiari3 in 1899. The syndrome is caused by obstruction to the hepatic veins. In the Fig 1. Photograph showing massive ascites and dilated superficial abdominal veins. majority of cases, the obstruction is ascribed to obliterative thrombophlebitis of unknown cause.4

scholarly journals Budd-Chiari Syndrome

2017 ◽  
Vol 118 (2-3) ◽  
pp. 69-80 ◽  
Author(s):  
Tomáš Grus ◽  
Lukáš Lambert ◽  
Gabriela Grusová ◽  
Rohan Banerjee ◽  
Andrea Burgetová
Keyword(s):  
Inferior Vena Cava ◽  
Structural Changes ◽  
Inferior Vena ◽  
Survival Rates ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Venous Outflow ◽  
Secondary Pathology ◽  
Chiari Syndrome

Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment. Without treatment, 90% of patients die within 3 years, mostly due to complications of liver cirrhosis. BCS can be classified according to etiology (primary, secondary), clinical course (acute, chronic, acute or chronic lesion), and morphology (truncal, radicular, and venooclusive type). The diagnosis is established by demonstrating obstruction of the venous outflow and structural changes of the liver, portal venous system, or a secondary pathology by ultrasound, computed tomography, or magnetic resonance. Laboratory and hematological tests are an integral part of the comprehensive workup and are invaluable in recognizing hematological and coagulation disorders that may be identified in up to 75% of patients with BCS. The recommended therapeutic approach to BCS is based on a stepwise algorithm beginning with medical treatment (a consensus of expert opinion recommends anticoagulation in all patients), endovascular treatment to restore vessel patency (angioplasty, stenting, and local thrombolysis), placement of transjugular portosystemic shunt (TIPS), and orthotopic liver transplantation as a last resort rescue treatment.

scholarly journals Budd-Chiari syndrome due to giant hydatid cyst: a case report and brief literature review

2013 ◽  
Vol 7 (06) ◽  
pp. 489-493 ◽  
Author(s):  
Sami Akbulut ◽  
Mehmet Yilmaz ◽  
Aysegul Kahraman ◽  
Sezai Yilmaz
Keyword(s):  
Inferior Vena Cava ◽  
Hydatid Cyst ◽  
Hydatid Disease ◽  
Inferior Vena ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow ◽  
The Right

Budd-Chiari syndrome is an uncommon disorder characterized by the thrombotic or non-thrombotic obstruction of hepatic venous outflow anywhere along the venous course from the hepatic venules to the junction of the inferior vena cava and the right atrium. The etiology of Budd-Chiari syndrome is classified as primary, attributable to intrinsic intraluminal thrombosis or the development of venous webs; or secondary, caused by intraluminal invasion by a parasite or malignant tumor or extraluminal compression by an abscess, solid tumor, or cyst, such as a hydatid cyst. In this study, we present a case of a giant hydatid cyst manifesting Budd-Chiari syndrome symptoms and signs by compressing the inferior vena cava and hepatic veins. In brief, the case demonstrates that hydatid disease should be considered in the differential diagnosis of Budd-Chiari Syndrome in areas such as Turkey, where hydatid disease is endemic.

Budd-Chiari syndrome due to segmental obstruction of the inferior vena cava successfully managed with endovascular stenting

MedPharmRes ◽  
2018 ◽  
Vol 2 (3) ◽  
pp. 22-26
Author(s):  
Uyen Vo ◽  
Duc Quach ◽  
Luan Dang ◽  
Thao Luu ◽  
Luan Nguyen
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Laboratory Data ◽  
Transaminase Level ◽  
Vena Cava ◽  
Budd Chiari Syndrome ◽  
Endovascular Stenting ◽  
Massive Ascites ◽  
Chiari Syndrome ◽  
History Of

Budd–Chiari syndrome (BCS), a rare and life-threatening disorder due to hepatic venous outflow obstruction, is occasionally associated with hypoproteinemia. We herein report the first case of BCS with segmental obstruction of the intrahepatic portion of inferior vena cava (IVC) and hepatic veins (HVs) successfully treated by endovascular stenting in Vietnam. A 32-year-old female patient presented with a 2-month history of massive ascites and leg swelling. She refused history of oral contraceptives use. Hepatosplenomegaly without tenderness was noted. Laboratory data showed polycythemia, mild hypoalbuminemia and hypoproteinemia, slightly high total bilirubin and normal transaminase level. The serum ascites albumin gradient was 1.9 g/dL and ascitic protein level was 1.1 g/dL. The other data were normal. BCS was suspected because of the discrepancy between mild liver failure and massive ascites; and the presence of hepatosplenomegaly and polycythemia. On abdominal magnetic resonance imaging, the segmental obstruction of three HVs and IVC was 2-3 cm long without thrombus. Cavogram revealed the severe segmental stenosis of intrahepatic portion of IVC with no visualized HV and extensive collateral veins. A Protégé stent was deployed to IVC. Leg swelling and ascites were completely resolved within 3 days after stenting. During 1-year follow-up, edema was not recurred and repeated laboratory results were all normal.

Recanalization and Reconstruction of a Chronically Occluded Inferior Vena Cava Through an Existing Transjugular Intrahepatic Portosystemic Shunt in the Setting of Budd-Chiari Syndrome

2021 ◽  
pp. 153857442110020
Author(s):  
Reza Talaie ◽  
Hamed Jalaeian ◽  
Nassir Rostambeigi ◽  
Anthony Spano ◽  
Jafar Golzarian
Keyword(s):  
Inferior Vena Cava ◽  
Transjugular Intrahepatic Portosystemic Shunt ◽  
Inferior Vena ◽  
Vena Cava ◽  
Portosystemic Shunt ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome ◽  
Intrahepatic Portosystemic Shunt ◽  
The Right

Budd-Chiari syndrome (BCS) results from the occlusion or flow reduction in the hepatic veins or inferior vena cava and can be treated with transjugular intrahepatic portosystemic shunt when hepatic vein recanalization fails.1-3 Hypercoagulable patients with primary BCS are predisposed to development of new areas of thrombosis within the TIPS shunt or IVC. This case details a patient with BCS, pre-existing TIPS extending to the right atrium, and chronic retrohepatic IVC thrombosis who underwent sharp recanalization of the IVC with stenting into the TIPS stent bridging the patient until his subsequent hepatic transplantation.

scholarly journals Acute Budd–Chiari syndrome caused by inferior vena cava compression from a congenital diaphragmatic hernia

2020 ◽  
Vol 102 (8) ◽  
pp. e202-e204
Author(s):  
KM Wcislo ◽  
CE Hall ◽  
N Abbassi-Ghadi
Keyword(s):  
Inferior Vena Cava ◽  
Diaphragmatic Hernia ◽  
Inferior Vena ◽  
Vena Cava ◽  
Rare Condition ◽  
Acute Onset ◽  
Budd Chiari Syndrome ◽  
Extrinsic Compression ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow

Acute Budd–Chiari syndrome is a rare condition characterised by obstruction of hepatic venous outflow. We describe the case of a 52-year-old man, with a congenital Morgagni diaphragmatic hernia, who presented with acute onset abdominal pain, shortness of breath, lactic acidosis, hyperbilirubinaemia and transaminasaemia. Computed tomography revealed strangulation of the diaphragmatic hernia and extrinsic compression of the inferior vena cava from the herniated viscera. Emergency surgery was carried out to repair the hernia with a biosynthetic mesh, with complete resolution of the Budd–Chiari syndrome.

Numerical Study of Blood Flow in the Hepatic Portion of the Inferior Vena Cava

2007 ◽  
Author(s):  
M. Matsubara ◽  
M. Watanabe ◽  
S. Watanabe ◽  
K. Konishi ◽  
S. Yamaguchi ◽  
...  
Keyword(s):  
Blood Flow ◽  
Inferior Vena Cava ◽  
Vessel Wall ◽  
Inferior Vena ◽  
Numerical Study ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Partial Occlusion ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome

Budd-Chiari syndrome (BCS) is complete or partial occlusion in the hepatic veins and the hepatic portion of the inferior vena cava (IVC). The cause of BCS is not well known yet, however: abnormal vessel wall shear stress caused by blood flow is thought to increase the likelihood of developing BCS. In order to reveal the formation mechanism of BCS, we construct several vessel models of the IVC and hepatic veins from medical images and study the characteristics of the blood flow in the vicinity of the junctions of the hepatic veins with the IVC numerically.

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