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Biliary Atresia

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. The cause of BA is not known Hypotheses

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Biliary Atresia

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  1. Biliary Atresia

  2. Introduction • A cholangiodestructive disease affecting all parts of biliary tract. • Untreated :cirrhosis,liver failure and death

  3. Epidemiology • 1 in 10,000 – Japan and China • 1 in 17,000 – UK, USA, and Europe • F > M

  4. Etiology The cause of BA is not known Hypotheses Developmental Perinatal acquired Immunological overreaction

  5. Classification Classification of Biliary Atresia

  6. Classification

  7. Clinical Features Jaundice Pale stools Dark urine Failure to thrive

  8. Presentation of biliary atresia

  9. 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

  10. Differential Diagnosis Obstructed choledochal malformations Spontaneous perforation of the bile duct others

  11. Management • Kasai-type portoenterostomy • Laparoscopic-assisted portoenterostomy has been described and is clearly possible although a true comparison with the open procedure

  12. If fails, or the child develops develops significant complications of chronic liver disease, then liver transplantation will be required if available

  13. Complications Cholangitis (40%) Portal hypertension Hepatopulmonary syndrome Malignancy

  14. Outcome Prognosis post-KPE can be affected by 1. Age at surgery 2. Experience of surgeon/center 3. BASM – poor prognosis

  15. Thank you !

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