Biliary Atresia with Portal Vein Anomaly: Surgical Challenges and Implications during Kasai Portoenterostomy

A BSTRACT Biliary atresia (BA) is a progressive, fibro-obliterative cholangiopathy of infancy that constitutes the leading cause of neonatal cholestasis and remains the primary indication for pediatric liver transplantation. While the Kasai portoenterostomy (KPE) is the standard initial surgical intervention, anatomical variations, particularly in the vascular architecture at the porta hepatis, can present considerable technical difficulties. This paper details a case of type IIa BA associated with a type III portal vein anomaly, identified intraoperatively during the KPE procedure. The case highlights the importance of recognizing vascular variants to facilitate precise dissection, minimize the risk of vascular injury, and optimize both immediate surgical and long-term transplant outcomes.