Biliary Atresia: the Timing Needs a Changin'

Biliary atresia (BA), a uniquely pediatric liver disease, is the leading cause of liver-related death in children and the most frequent indication for liver transplantation in the pediatric population. Early intervention with a Kasai procedure (KP) is the current standard of care for this condition. The single most important and well-established prognostic factor for the KP outcome is the patient's age at the time of the KP. The older the infant, the less successful the operation and the less favourable is the post-KP survival with native liver. There remains in Canada, and throughout the world, a problem of late referral, delayed diagnosis and older age at surgery. Early disease detection and intervention has been hampered by the lack of an effective screening strategy for BA. Recently, however, novel programs for the early identification of BA in the first month of life, but after two weeks of age, have been successfully implemented and evaluated in some countries, with significantly improved outcomes for affected infants. Whether any of these programs should be adopted to improve the timing of referral and treatment for Canadian infants affected with this devastating liver disease deserves consideration and study.