Biliary Atresia Flashcards
What is biliary atresia?
A arre condition where the bile ducts of an infant are progressively fibrosed and destroyed
What is the issue with the bild ducts in someone with biliary atresia?
They are progressivelly fibrosed and destroyed leading to obstruction in the flow of bile
What does biliary atresia result in?
Cholestasis in the first few weeks of life.
What can biliary atresia cause?
Conjugated hyperbilirubinaemia
Liver failure
Death (if not treated)
How can biliary atresia present?
Jaundice extending beyond the physiological two weeks
Dark urine and pale stools
Appetite and growth disturbance
What are the signs of biliary obstruction?
Pale stools
Dark urine
What is cholestasis?
Bile cannot be transported from the liver to the bowel.
What are the main signs of biliary atresia?
Jaundice
Hepatomegaly with splenomegaly
Abnormal growth
What are the main investigations for biliary atresia?
Bloods
A hepatic scintigraphy
Abdominal ultrasound
Cholangiography
What would you find in the bloods of someone with biliary atresia?
Raised conjugated bilirubin and deranged liver function tests
What is used for the definitive diagnosis of biliary atresia?
Cholangiography
What would you see on a cholangiography of someone with biliary atresia?
It will fail to show normal architecture of the biliary tree
What would an abdominal US of someone with biliary atresia show?
Echogenic fibrosis
What is the management of biliary atresia?
Kasai procedure
What is kasai procedure?
hepatoportoenterostomy- creates a new pathway from the liver to the gut to bypass fibrosed ducts
