Riggio Oliviero, Marzano Chiara, Papa Alessia, Pasquale Chiara, Gasperini Maria Ludovica, Gigante Antonietta, Valla Dominique Charles, Plessier Aurélie, Amoroso Antonio
Gastroenterology, Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
J Thromb Thrombolysis. 2014 May;37(4):536-9. doi: 10.1007/s11239-013-0959-z.
Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms of this disease where the obstruction is limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this cases only a liver biopsy can demonstrate the presence of a small vessels outflow block. We report two cases of small hepatic veins Budd-Chiari syndrome.
布加综合征是一种罕见的疾病,其特征为从小肝静脉至腔静脉交界处的任何水平出现肝静脉流出道梗阻,且不存在心力衰竭或缩窄性心包炎。有多种影像学检查方法可用于研究肝脏的大体血管解剖结构,但这种疾病存在罕见类型,其梗阻仅限于肝内小静脉,影像学检查显示大肝静脉外观正常。在这种情况下,只有肝活检才能证实存在小血管流出道阻塞。我们报告两例肝小静脉布加综合征病例。