Effects from right hepatic vein and inferior vena cava position on mechanism of Budd-Chiari syndrome / 医用生物力学

Objective To investigate the effects from various angles between inferior vein cava (IVC) and right hepatic vein (RHV) on pathogenesis of IVC membranous obstruction for patients with Budd-Chiari syndrome (BCS). Methods The normal 3D solid model of IVC and hepatic veins was reconstructed using MRI angiograms, and the angle between IVC and RHV was 56°. The two models with IVC-RHV angle of 30° and 120° were established, respectively, based on the reconstructed model. The distributions of wall shear stress, static pressure and blood velocity of the 3 models were calculated by numerical simulation. Results The wall shear stresses, static pressure and blood velocity of the 3 models displayed significantly differences. Compared with the normal 56° model, the 30° model showed a higher wall pressure and lower blood velocity, while the 120° model presented a lower wall pressure and blood velocity with turbulence of blood flowing, and such hemodynamic changes would increase the risk of thrombosis. The 56° model had the fastest blood velocity. Conclusions Numerical simulation of the flow in IVC and RHV can promote to discover the pathogenesis of BCS, and help to predict risk of IVC membranous obstruction, and provide theoretical references for BCS treatment.

Síndrome de Budd Chiari: Reporte de tres casos y revisión de la literatura / Budd Chiari Syndrome: Three Case Reports and a Literature Review

Se describen los casos de tres pacientes de sexo femenino a quienes se les hizo diagnóstico de síndrome de Budd Chiari. En una paciente la presentación del síndrome fue subaguda, pudiendo ser manejada exitosamente con la colocación de TIPS. Otra con mutación del factor V Leyden asociada desarrolló disfunción hepática progresiva y requirió de trasplante hepático. En dos de los tres casos se identificó una enfermedad hematológica como trastorno de base, y en uno el uso de anticonceptivos orales como factor de riesgo. Las tres pacientes fueron sometidas a terapia anticoagulante y el manejo quirúrgico fue definido de acuerdo a su condición clínica. Sin embargo, en un caso la presentación fue aguda con falla hepática y muerte.

This article describes the cases of three female patients who were diagnosed with Budd-Chiari syndrome. One patient was subacute and could be successfully managed by placement of a transjugular intrahepatic portosystemic stent (TIPS). Another patient who had the Factor V Leiden mutation developed associated progressive liver dysfunction and required liver transplantation. A hematologic disease was identified as the underlying disorder in two of the three cases. For one patient, the use of oral contraceptives was a risk factor. Since all three patients were undergoing anticoagulant therapy, surgical management was determined according to each patient’s clinical condition. Nevertheless, the one patient who that presented acute hepatic failure did not survive.

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.