A long-term follow-up of three patients with biliary atresia

This report reviews the courses of three children who underwent surgery for biliary atresia. They include a patient who is now 25 years of age who underwent correction of an "operable" biliary atresia in the neonatal period. This patient is alive and well without jaundice. The second is a 21-year-old woman who had a Kasai operation, also performed in the neonatal period; she is well and attending school but does have an elevated bilirubin and signs of liver dysfunction. The third patient, who underwent surgery performed by Dr Willis Potts in 1963 for what appeared to be "inoperable" biliary atresia, finally underwent a portal duodenostomy after her third operation. She had satisfactory bile drainage but had numerous bouts of cholangitis early in her life. She survived until she had several major episodes of gastrointestinal hemorrhage from varices and finally died after a portal systemic shunt. These three patients illustrate that prolonged survival is possible after a variety of operations performed for biliary atresia. Furthermore, even though these patients were severely jaundiced early on in life and at least two of them had complicated postoperative courses, they are perfectly normal from an intellectual standpoint and have functioned in a normal fashion.