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Biliary Atresia

This is a congenital narrowing/absence of the bile duct, leading to cholestasis.

“Operative view of complete extrahepatic biliary atresia.” © Chardot C CC BY 2.0 (https://creativecommons.org/licenses/by/2.0/)

It presents with:

  • Prolonged jaundice (14+ days)

  • Signs of biliary obstruction – Dark urine, Pale stools, Itchiness


Investigations:

  • Conjugated and Unconjugated bilirubin levels – High proportion of conjugated bilirubin suggests post-hepatic jaundice

  • Bloods - FBC, LFT, Clotting screen

  • Ultrasound

  • Cholangiography


Management:

  • Surgery - Kasai procedure (Hepatoportoenterostomy) – Section of small intestine attached to opening of the liver

  • Ursodeoxycholic acid to prevent cholangiocyte damage




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