Biliary Atresia

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Definition

-  Progressive idiopathic fibro-obliterative disease of extrahepatc biliary tree

 

Features

-  1:10,000 births

-  Most common reason for liver transplant in kids

-  Usually thrive initially

-  90%+ survive to adulthood

 

Associated S/S

-  Neonatal jaundice anytime from birth to 8weeks

-  <30% also ass with malformations

-  +/- acholic stools

 

Ix

-  Abdo US

-  DISIDA

-  Liver biopsy

-  Cholangiogram

-  Gold standard for Dx

-  Intra-operative

 

Mx

-  Surgery

-  Hepatoportojejunestomy (Kasai procedure)

-  Liver transplant 60-80% pts