Biliary atresia

Question 1

Define biliary atresia

Answer 1

Rare but serious condition where bile ducts in newborn are progressively fibrosed and destroyed
Causes conjugated hyperbilirubinemia, liver failure and death.

Question 2

What is the relevant epidemiology of biliary atresia?

Answer 2

Commonly affects newborn -> most common neonatal cholestasis
UK -> 1 in 8,000/18,000 births
Globally most common reason for paediatric liver transplant.

Question 3

What is the underlying aetiology of biliary atresia?

Answer 3

Genetic and environmental
Potential - aberrant immune response to viral infections of bile ducts
20% existing congenital anomalies

Question 4

What are the typical signs and symptoms of biliary atresia?

Answer 4

Prolonged jaundice (14d or 21 if prem)
Signs of biliary obstruction such as dark urine and pale or chalky white stool

Question 5

What are the relevant investigations for biliary atresia?

Answer 5

Blood - raised conj BR and deranged LFTs (GGT high)
Hepatic scintigraphy - poor excretion into bowel
Abdo US - echogenic fibrosis
Cholangiography - definitive diagnostic test
Gold standard - liver histology by percutaneous biospy

Question 6

What is the management for biliary atresia?

Answer 6

Hepatoportoenterostomy - kasia procedure - bypass between liver and gut
If advanced liver failure - transplantation

Question 7

What are the common complications of biliary atresia?

Answer 7

Ascending cholangitis following surgery
Cirrhosis, portal hypertension and liver failure
Osteomalacia or biliary rickets