ARRS 2022 Abstracts

RETURN TO ABSTRACT LISTING


E1408. Adult and Pediatric Congenital and Developmental Diseases of the Biliary System: Cross-Sectional Imaging Spectrum
Authors
  1. Sammar Ghannam; UT Health San Antonio
  2. Dhiraj Kamireddy; UT Health San Antonio
  3. Anil Dasyam; University of Pittsburgh Medical Center
  4. Deepa Biyyam; Phoenix Children's Hospital
  5. Venkata Katabathina; UT Health San Antonio
Background
Congenital and developmental anomalies of the biliary system are often clinically significant. Knowledge of the embryologic development of the biliary system provides a basis for understanding developmental pathology. Bile duct congenital diseases are organized based on the involved biliary ducts. Knowledge of congenital cystic diseases will help the radiologist identify predisposing factors for the development of pancreatitis, cholangitis, stones, and malignancy. Recognition of bile duct anomalies and normal variants will help the radiologist avoid diagnostic errors, advise surgical planning, and prevent injury to surrounding structures. Radiologists need to be familiar with congenital and developmental diseases of the biliary system, the imaging tests available for their study, and their imaging findings.

Educational Goals / Teaching Points
The goals of this exhibit are as follows. Review embryology of biliary system with special focus on ductal plate, molecular, and cellular mechanisms that may help to understand the origin of congenital and developmental biliary diseases. Discuss imaging findings of cystic and noncystic congenital biliary diseases on US, CT, and MRI/MRCP. We also discuss the imaging spectrum of gallbladder and aberrant biliary ductal anomalies. Finally, we review the role of imaging in diagnosis and management of complications and associated conditions.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Key points of this exhibit are as follows. Embryologic development of bile ducts includes morphogenesis of ductal plate, cholagiocytes, and intrahepatic ducts and extrahepatic bile ducts growth. Diseases of intrahepatic bile ducts includes cystic (Von Meyenburg complex, congenital hepatic fibrosis, Caroli disease, simple/complex cysts, and polycystic liver disease) and noncystic disease (non-syndromic paucity of interlobular bile ducts and Alagille syndrome). Diseases of extrahepatic bile ducts includes cystic (choledochal cysts) and noncystic disease (biliary atresia) Gallbladder issues include agenesis, intrahepatic, floating, double, and septate. The exhibit also covers anomalous pancreaticobiliary junction, aberrant cystic duct insertion, and aberrant biliary ducts, and other associated conditions. Complications include infection and malignancy.

Conclusion
Congenital and developmental anomalies of the biliary system are often clinically significant. Bile duct congenital diseases are organized based on the involved biliary ducts. Recent knowledge of molecular and cellular mechanisms involved in biliary system development has improved our understanding of pathogenesis of congenital and developmental biliary diseases. Imaging of biliary anomalies often requires a multimodality imaging approach; MRI with MRCP plays a pivotal role in the diagnosis and management. Radiologists should know the basic concepts including embryology of the biliary system and normal variants; this provides an opportunity for improved diagnosis, prognosis, treatment, appropriate follow-up, and specialized imaging surveillance.