Biliary atresia

Question 1

What is biliary atresia?

Answer 1

Biliary atresia is a rare condition where the bile ducts of an infant are progressively fibrosed and destroyed, leading to:
=> conjugated hyperbilirubinaemia,
=> liver failure
=> death if not treated

Question 2

What are the 3 types of biliary atresia?

Answer 2

Type 1: The proximal ducts are patent but the common duct is obliterated

Type 2: Atresia of the cystic duct and cystic structures are found in the porta hepatis

Type 3: Atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia

Question 3

What are the signs and symptoms of biliary atresia?

Answer 3

Typically presents in the first few weeks of life with:

=> Jaundice beyond the physiological 2 weeks

=> dark urine and pale stools

=> appetite & growth disturbances

=> hepatomegaly with splenomegaly

=> abnormal growth

=> cardiac murmurs if assoc. cardiac abnormalities present

Question 4

What are the investigations carried out in biliary atresia?

Answer 4

1. Serum bilirubin: => total bilirubin may be normal, conjugated bilirubin is abnormally high
2. Liver function tests
   => serum bile acids and aminotransferases are usually raised but cannot differentiate between biliary atresia and other causes of neonatal cholestasis
3. Serum alpha 1-antitrypsin:
   => deficiency may be a cause of neonatal cholestasis
4. Sweat chloride test:
   => cystic fibrosis often involves the biliary tract
5. Ultrasound of the biliary tree and liver:
   => may show distension, echogenic fibrosis and tract abnormalities
6. Percutaneous liver biopsy with intraoperative cholangioscopy

Question 5

How is biliary atresia managed?

Answer 5

Surgery => Hepatoportoenterostomy (Kasai procedure) => creates a new pathway from the liver to the gut bypass fibrosed ducts

Question 6

What are the complications of biliary atresia?

Answer 6

=> Unsuccessful anastomosis formation

=> Progressive liver disease

=> Cirrhosis with eventual hepatocellular carcinoma

Question 7

What is the prognosis for biliary atresia?

Answer 7

Good if surgery is successful

If surgery fails, liver transplantation may be required in the first two years of life