Advances in the surgical technique of Kasai portoenterostomy

Abstract

Biliary atresia is a rare, degenerative, and obliterative inflammatory disease of the bile ducts in neonates, affecting both the intra- and extrahepatic biliary tract. With an unknown etiology and no parallel condition in adults or older children, biliary atresia affects 1 in 10,000–18,000 live births and, if untreated, progresses to liver cirrhosis and death by the age of two. The Kasai portoenterostomy, introduced in 1950, remains the primary palliative procedure to restore bile drainage and delay the need for liver transplantation, which is required in cases of biliary flow failure. Biliary atresia continues to be the leading cause of pediatric liver transplants, accounting for up to 75 % of procedures in children under two years. While the Kasai technique has undergone numerous modifications; surgeons at Juntendo University Hospital, Japan, have modernized the procedure while preserving Dr. Kasai's original portoenterostomy principles. Since 2009, the hospital has been a pioneer in laparoscopic Kasai portoenterostomy; this article presents a detailed description of the state-of-the-art approaches and postoperative management, along with analysis of the latest outcomes. These advancements highlight the hospital's role as a leader in treatment of biliary atresia.