Síndrome de Budd-Chiari tratada por angioplastia transjugular da veia supra-hepática / Budd-Chiari Sindromy treated by transjugular angioplasty of the supra-hepatic vein

A Síndrome de Budd-chiari é caracterizada pela oclusão das veias supra-hepáticas e apresenta inúmeras causas, mas nenhuma delas é identificável em cerca de 60-70% dos pacientes. O diagnóstico é realizado pela suspeita clínica e confirmado por exames complementares, sendo a ultrassonografia com Doppler a mais sensível (85%). A terapia varia desde medicamentos, cirurgias como anastomoses portossistêmicos e transplante hepático até métodos menos invasivos como derivação intra-hepática portossistêmica transjugular (TIPS) e angioplastia. O objetivo desse trabalho é apresentar um paciente portador da SBC submetido à angioplastia transjugular da veia supra-hepática, utilizando via anterógrada (punção através da veia cava inferior trans-hepática, atingindo a veia supra-hepática e recanalizando anterogradamente a veia supra-hepática).

The Budd-chiari syndrome is characterized by occlusion of the supra-hepatic veins and has any causes, but none of them are identifiable by about 60-70% of patients. The diagnosis is made by clinical suspicion and confirmed by additional tests, and the Doppler ultrasound with the most sensitive (85%). The therapy varies from medications, surgeries such as liver transplantation and anastomosis portossistêmicos even less invasive methods like bypass intra-hepatic portosystemic transjugular (TIPS) and angioplasty. The aim of this article is to present a patient of SBC submitted to angioplasty transjugular the above hepatic vein using antegrade route (puncture through the inferior vena cava reaching the above-hepatic vein and the vein recanalizando anterograde above-liver).

MDCT venography in the evaluation of inferior vena cava in Budd-Chiari syndrome.

Objective To assess the role of multidetector computed tomography (MDCT) venography in the evaluation of the inferior vena cava (IVC) in Budd-Chiari syndrome (BCS), its accuracy as compared to digital subtraction venography (DSV) and the potential of this technique to replace venography for the defi nitive diagnosis of BCS. Methods Twenty-fi ve suspected cases of BCS were prospectively enrolled in this study and underwent both MDCT venography and DSV. Two observers independently evaluated and graded both the axial and reformatted MDCT images for the presence, site, degree and length of IVC narrowing. The collateral pathways and the hepatic veins were also assessed in all cases. The degree of correlation between MDCT venography and DSV was expressed using Spearman’s rank correlation coeffi cient (Rs). Results There was excellent correlation between MDCT venography and DSV in predicting the presence of stenosis and in grading the degree and length of IVC stenosis (Rs=0.58, p<0.05). Four patients had presence of a web within the IVC and the reconstructed MDCT venography images detected the fl ap of the membrane in all of them. In three cases of complete obstruction the cranial extent of the obstruction could be determined on the reconstructed MDCT venography images, while double catheter access through the femoral and jugular routes was needed to determine the same on DSV. MDCT venography was signifi cantly more informative in depicting the presence and site of both intrahepatic and extrahepatic collaterals as compared to DSV. Conclusion MDCT venography, in the present study, accurately provided information of both conventional CT and IVCgraphy, in the evaluation of the IVC in a non-invasive way. It helped overcome the shortcomings of CT in the evaluation of IVC and was better than DSV for the evaluation of collaterals, calcifi cation and complete IVC obstruction. We suggest that CT venography can be used as a frontline investigation for the diagnosis of IVC obstruction and for planning surgery or percutaneous endovascular intervention.

Management of the Budd-Chiari syndrome by balloon cavoplasty.

BACKGROUND: Obstruction of the suprahepatic inferior vena cava (IVC) by a membrane or stricture is the commonest cause of Budd-Chiari syndrome in the eastern hemisphere. We present our experience with the outcome of balloon cavoplasty in such cases. METHODS: We followed up 40 consecutive cases of Budd-Chiari syndrome over seven years. Doppler study of hepatic venous outflow tract (in all cases), liver biopsy (30 cases) and necropsy (two cases) were performed. Balloon cavoplasty was done in selected cases. RESULTS: Of 40 patients with BCS (mean age 35.2 [SD 8.7] years; 26 men) 5, 5 and 30 had fulminant, acute and chronic presentation, respectively. Inferior vena cavography was performed in 32 cases, and showed membranous obstruction of the IVC in 12, segmental occlusion of the IVC in 11 cases, and block in both the IVC and the main hepatic veins in the rest. Successful balloon cavoplasty was done in 18 cases with obstruction of the IVC (membrane or stricture); 15 of them are well over a mean follow up of 56 (14.6) months. Three patients developed restenosis; two of them, treated with redilatation, are doing well, and one died of septicemia and hepatic failure following a surgical bypass. Pressure gradient between the IVC and right atrium decreased significantly after cavoplasty (15.4 [2.8] vs 6.6 [2.0] mmHg; p< 0.001). CONCLUSION: Balloon cavoplasty gave encouraging results in the management of Budd-Chiari syndrome due to membranous obstruction or stricture of the IVC.