Department of Radiology, Hôpital Beaujon, HUPNVS, 100, Bd du Général Leclerc, Clichy, France.
Department of Hepatology, Hôpital Beaujon, HUPNVS, Clichy, France.
Abdom Radiol (NY). 2018 Aug;43(8):1896-1907. doi: 10.1007/s00261-017-1447-2.
Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition. Acute BCS is rare, and patients present with hepatomegaly, ascites, and hepatic insufficiency. The diagnosis is based on imaging. Imaging features include (1) direct signs, in particular occlusion or compression of the hepatic veins and/or the inferior vena cava and venous collaterals and (2) indirect signs, in particular morphological changes in the liver with hypertrophy of the caudate lobe and delayed nodule formation. Ultrasound and magnetic resonance imaging are the gold standard for diagnosis. The aim of this review is to provide an overview of the role of imaging in the diagnosis of BCS.
布加综合征（BCS）定义为临床和实验室征象，伴有肝静脉流出道部分或完全受损，而无右心衰竭或缩窄性心包炎。原发性 BCS 是最常见的类型，是高凝状态的并发症，特别是骨髓增生性肿瘤。继发性 BCS 涉及肿瘤侵犯或外在压迫。大多数患者表现为慢性 BCS，包括非肝硬化、变形、慢性肝病，伴有不同程度的纤维沉积。急性 BCS 很少见，患者表现为肝肿大、腹水和肝功能不全。诊断基于影像学。影像学特征包括（1）直接征象，特别是肝静脉和/或下腔静脉闭塞或受压以及静脉侧支循环；（2）间接征象，特别是肝脏形态改变，尾状叶肥大和结节形成延迟。超声和磁共振成像（MRI）是诊断的金标准。本文旨在概述影像学在 BCS 诊断中的作用。
