30 years' experience in the management of biliary atresia and CPAM: latest advances and controversies

Abstract

Purpose: Despite affecting different organ systems, both biliary atresia and congenital pulmonary airway malformation are two indexed conditions in our specialty. Our unit has been taking active interests in these two conditions for many years and recent research has resulted in some exciting data. Here, we aim to provide an up-to-date review of current understanding of the two diseases. Patients and Methods: The basis of this talk is based on a review of the experience in our centre in the clinical management and also data from basic and clinical research of biliary atresia and CPAM, in combination with Medline searches of the latest findings worldwide. Results: The evolution of surgical technique and the addition of adjuvant post-operative measures have contributed to an increase of long-term success rate of Kasai procedure. Genetic studies have further provided clues to the disease pathogenesis. For CAPM, we have gathered all available information and have provided a recommended clinical management algorithm. Conclusions: With advances in clinical and basic knowledge, we strive to provide the best care for these patients and exciting times lie ahead.