Biliary Atresia Introduction A cholangiodestructive disease affecting all parts of biliary tract. Untreated :cirrhosis,liver failure and death Epidemiology 1 in 10,000 – Japan and China 1 in 17,000 – UK, USA, and Europe F>M Etiology 1. 2. The cause of BA is not known Hypotheses Developmental Perinatal acquired Immunological overreaction Classification Classification of Biliary Atresia Type Incidence (%) Description 1 ∼5% Level of obstruction within the common bile duct. 2 ∼2% Level of obstruction within the common hepatic duct. 3 >90% Obstruction is within the porta hepatis with no visible bilecontaining proximal lumen. Classification Clinical Features Jaundice Pale stools Dark urine Failure to thrive Presentation of biliary atresia Diagnosis It is possible to make the correct diagnosis in >80% of cases before a laparotomy Ultrasound Liver biopsy In Japan, use duodenal intubation and measuement of intralumenal bile ERCP，percutaneous cholangiography Differential Diagnosis Obstructed choledochal malformations Spontaneous perforation of the bile duct others Management Kasai-type portoenterostomy Laparoscopic-assisted portoenterostomy has been described and is clearly possible although a true comparison with the open procedure If fails, or the child develops develops significant complications of chronic liver disease, then liver transplantation will be required if available Complications Cholangitis (40%) Portal hypertension Hepatopulmonary syndrome Malignancy Outcome Prognosis post-KPE can be affected by 1. Age at surgery 2. Experience of surgeon/center 3. BASM – poor prognosis Thank you !