3.8 Biliary Atresia

Question 1

How does biliary atresia present?

Answer 1

• jaundice persisting longer than the physiological 2 weeks (3 weeks in prem)
• dark urine and pale stools
• appetite and growth disturbance (may be normal in some cases)

Question 2

What is your initial investigation for possible biliary atresia?

Answer 2

conjugated and unconjugated bilirubin levels

will be conjugated in atresia as liver is processing it, but it cannot escape

Question 3

How do you manage biliary atresia?

Answer 3

surgery is the only option to create duct anastomoses

liver transplant may be required

Question 4

What is the name of the biliary atresia operation?

Answer 4

“Kasai portoenterostomy” uses a section of small intestine into the opening of the liver.

Question 5

6 investigations you might do during a biliary atresia diagnosis?

Answer 5

Serum bilirubin: total bilirubin may be normal, whereas conjugated bilirubin is abnormally high

Liver function tests (LFTs): bile acids and aminotransferases are usually raised but cannot differentiate between biliary atresia and other causes of neonatal cholestasis

Serum alpha 1-antitrypsin: Deficiency may be a cause of neonatal cholestasis

Sweat chloride test: Cystic fibrosis often involves the biliary tract

Ultrasound of the biliary tree and liver: distension and tract abnormalities

Percutaneous liver biopsy with intraoperative cholangioscopy