Cystic biliary anomaly in a newborn with features of choledochal cyst and cystic biliary atresia

Abstract

Cystic biliary atresia (CBA) and choledochal cysts (CDC) can present similarly with early onset jaundice, conjugated hyperbilirubinemia, and radiographic cystic biliary dilatations. We present the case of an infant antenatally diagnosed with a congenital biliary dilatation found to have non-atretic intrahepatic and extrahepatic bile ducts leading to a CDC without distal flow past the cyst into the duodenum. While prior literature has differentiated CDC and CBA as separate entities, our case suggests that these two pathologies may arise on a spectrum of obstructive cholangiopathies. An operative approach assessing the fluoroscopic patency of the intrahepatic and extrahepatic bile ducts and exploration of the anatomy can guide the choice of cyst excision versus hepatic portoenterostomy.

Keywords

• Choledochal cyst
• Biliary atresia
• Cystic biliary atresia
• Type 1 cystic biliary atresia
• Obstructive cholangiopathies