Department of Paediatric Radiology and Neuroradiology, V. Buzzi Children's Hospital, 32 Castelvetro Street, 20154, Milan, Italy.
Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Saclay, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
Pediatr Radiol. 2021 Jul;51(8):1545-1554. doi: 10.1007/s00247-021-05034-7. Epub 2021 May 11.
We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. In this second part, we discuss the roles of magnetic resonance (MR) cholecystopancreatography, hepatobiliary scintigraphy, percutaneous biopsy and percutaneous cholecysto-cholangiography. Among imaging techniques, ultrasound (US) signs have a high specificity, although a normal US examination does not rule out biliary atresia. Other imaging techniques with direct opacification of the biliary tree combined with percutaneous liver biopsy have roles in equivocal cases. MR cholecystopancreatography and hepatobiliary scintigraphy are not useful for the diagnosis of biliary atresia. We propose a decisional flowchart for biliary atresia diagnosis based on US signs, including elastography, percutaneous cholecysto-cholangiography or endoscopic retrograde cholangiopancreatography and liver biopsy.
我们旨在介绍一种疑似胆道闭锁的影像学实用方法,胆道闭锁是一种婴儿期的炎症性胆管病,导致肝外和肝内胆管进行性纤维化和闭塞。如果不进行治疗或 Kasai 手术失败,胆道闭锁会进展为胆汁性肝硬化、终末期肝功能衰竭,并在生命的头几年内死亡。由于没有单一的方法可以诊断胆道闭锁,并且这种疾病的临床、实验室和影像学特征与其他新生儿胆汁淤积症的原因重叠,因此区分胆道闭锁与其他非手术性新生儿胆汁淤积症是困难的。在第二部分中,我们讨论了磁共振(MR)胆囊胰胆管成像、肝胆闪烁显像、经皮肝活检和经皮胆囊胆管造影的作用。在影像学技术中,超声(US)征象具有很高的特异性,尽管正常的 US 检查不能排除胆道闭锁。其他具有胆管直接显影作用的影像学技术结合经皮肝活检在诊断不明确的病例中有一定作用。MR 胆囊胰胆管成像和肝胆闪烁显像对胆道闭锁的诊断没有帮助。我们根据 US 征象(包括弹性成像)、经皮胆囊胆管造影或经内镜逆行胰胆管造影和肝活检提出了胆道闭锁诊断的决策流程图。