Purpose or Case Report: Biliary atresia is a progressive, fibro-obliterative disease of the intra- and extrahepatic bile ducts in infancy. This exhibit outlines the pathophysiology, diagnostic pathways, and current and emerging management strategies for biliary atresia in the pediatric age group.
Methods & Materials: Through our exhibit, we will demonstrate the classic imaging appearances of different types of biliary atresia and emphasize strategies for achieving an accurate diagnosis. We will illustrate the Kasai classification for biliary atresia and briefly discuss the pathophysiology behind these congenital anomalies to better understand and become accustomed to identifying their salient imaging features to ensure prompt diagnosis and treatment. In Kasai type I, the distal common bile duct (CBD) is obliterated. In type IIa, the common hepatic duct (CHD) is obliterated, whereas in type IIb CBD, CHD, and cystic ducts are obliterated. In type III, the entire extrahepatic biliary tree is obliterated. Surgical management strategies will also be discussed, along with an intraoperative cholangiogram.
Results: Ultrasound is the initial modality for evaluating neonatal jaundice. Biliary atresia must be ruled out in all infants with progressive jaundice and cholestasis. Ultrasound features such as absent or abnormal gallbladder, triangular cord sign, or enlarged hepatic artery are associated with biliary atresia. Hepatic scintigraphy with a technetium-labeled iminodiacetic acid analog (HIDA) demonstrates progressive hepatic uptake of the radiotracer but no biliary excretion. Magnetic Resonance Imaging (MRI) has excellent soft tissue contrast and resolution ability. MR cholangiopancreatography (MRCP) is ideal for differentiating type I biliary atresia from type I choledochal cyst, thereby playing a vital role in influencing surgical management and patient outcome. We present an imaging spectrum of cases demonstrating different types of biliary atresia in the pediatric population.
Conclusions: Understanding the pathophysiology and accurately recognizing the imaging findings of biliary atresia is essential to ensure timely intervention of this potentially life-threatening entity. We will review the imaging spectrum of biliary atresia and briefly discuss its management.