Biliary atresia. A surgical perspective.

The combination of portoenterostomy with subsequent liver transplantation is the treatment of choice for patients with biliary atresia. It is important, however, to attempt to keep the patient's own organ by continuing efforts to achieve the best possible results with portoenterostomy. Additional basic research, perhaps concerning on the role of cytokines and apoptosis in the control of biliary atresia, may provide insight into possible new medical strategies for treating patients with biliary atresia. For example, in addition to portoenterostomy, control of apoptosis at various cellular levels and of bile duct cell proliferation and maturation by manipulation of the growth factors and cytokines may become part of future treatment modalities. Another direction of research should be the control of fibrogenesis, which might be accomplished by blocking TGF-beta 1 and platelet-derived growth factor and by HGF gene therapy. The author's current strategy for surgical treatment for patients with biliary atresia include (1) early diagnosis, including prenatal diagnosis and broader use of mass screening programs, (2) hepatic portoenterostomy, without stoma formation; (3) close postoperative care, especially for prevention of postoperative cholangitis; (4) revision of portoenterostomy only in selected cases; (5) early liver transplantation in patients with absolutely failed portoenterostomy; (6) avoidance of laparotomy for the treatment of esophageal varices and hypersplenism; (7) consideration of exploratory laparotomy or primary liver transplantation for patients with advanced liver disease at the time of referral. The development of new treatment modalities based on the understanding of the pathogenesis of the disease, and especially on the biology of intrahepatic bile ducts and hepatic fibrosis, is essential.