ARRS 2022 Abstracts


E1735. Congenital Anomalies of Hepatobiliary System: A Pictorial Review
  1. Ankita Chauhan; Le Bonheur Children's Hospital; University of Tennessee Health Science Center
  2. Vijetha Maller; Le Bonheur Children's Hospital; University of Tennessee Health Science Center
Congenital anomalies of the hepatobiliary system are a group of fibropolycystic liver disorders resulting from embryonic ductal plates malformation. These include biliary atresia, biliary cysts (choledochal cyst), congenital hepatic fibrosis, and biliary hamartomas. Congenital hepatobiliary anomalies commonly present with neonatal cholestasis (conjugated hyperbilirubinemia). This exhibit outlines the etiologies, diagnostic pathways, and current and emerging management strategies for congenital hepatobiliary anomalies in the pediatric age group.

Educational Goals / Teaching Points
Through our exhibit, we will demonstrate the classic imaging appearances of congenital hepatobiliary anomalies in the pediatric age group (biliary atresia, choledochal cyst, and congenital hepatic fibrosis) and emphasize strategies for achieving an accurate diagnosis. We will 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. We will illustrate the Kasai classification for biliary atresia and Todani et al. classification for choledochal cysts. Surgical management strategies will also be discussed, along with an intraoperative cholangiogram.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Ultrasound is the initial modality for evaluating patients presenting with abdominal pain, jaundice, or an abdominal mass in children, often as part of a targeted exam at the site of symptoms. Biliary atresia must be excluded in all cholestatic infants. Ultrasound features such as absent or abnormal gallbladder, triangular cord sign, hepatic artery–portal vein ratio, or increased hepatic subcapsular blood flow are typically associated with biliary atresia. Hepatobiliary scintigraphy with a technetium-labeled iminodiacetic acid analog by confirming the patency of bile ducts excludes biliary atresia. Choledochal cysts are congenital dilation of the biliary tree and are classified based on their location according to the Todani system. Diagnosis is usually made at an ultrasound, followed by MRCP for surgical planning. When the presentation is in neonatal life or infancy with progressive cholestasis, choledochal cysts must be differentiated from cystic biliary atresia. In infants with choledochal cysts, the intrahepatic bile ducts are normal or dilated rather than sclerosed. Management depends on the type of choledochal cyst and the presence of biliary obstruction. Most cases of choledochal cysts will require surgical resection rather than the Kasai procedure used for patients with biliary atresia.

Understanding the pathophysiology and accurately recognizing the imaging findings of congenital hepatobiliary anomalies is essential to ensure timely intervention in these potentially life-threatening entities (such as biliary atresia). For other anomalies, prompt diagnosis is crucial as a delay in diagnosis will result in increased morbidity and mortality. We will review the imaging spectrum of congenital hepatobiliary anomalies and briefly discuss their and management.