Liver Disorders: Biliary Atresia Flashcards
In how many live births does biliary atresia occur?
1 in 15000
What is biliary atresia?
Progressive fibrosis and obliteration of the extrahepatic and intrahepatic biliary tree, resulting in an obstruction of the flow of bile
Without intervention, what occurs?
Chronic liver failure develops and death occurs in 2 years
What causes it?
Exact cause unknown
Congenital cause e.g problem during development
Inflammation of bile duct due to a viral or toxic substance
How does it present?
Mild jaundice
Pale stools - may fluctuate but becomes increasingly pale as the disease progresses
Dark urine
Normal birth weight followed by faltering growth
Hepatomegaly often present initially
Splenomegaly develops (due to portal hypertension)
Is extrahepatic biliary atresia more common in females or males?
Females
Is it unique to neonatal children?
Yes
Describe the different types
Type 1 - the proximal ducts are patent, but the common duct obliterated
Type 2 - there is atresia if cystic duct and cystic structures are found in the porta hepatis
Type 3 - atresia of left and right ducts to the level of the porta hepatis (this occurs in > 90% of cases of biliary atresia
What does the serum bilirubin show?
Conjugated bilirubin abnormally high
Total bilirubin may be normal
Will there be normal LFTs?
No - will be raised but cannot differentiate from biliary atresia and other causes of neonatal cholestasis
Why should a sweat chloride test be done?
Cystic fibrosis often involves the biliary tract
What can an fasting abdominal ultrasound demonstrate?
A contracted or absent gallbladder (although may be normal)
Triangular sign - common bile duct not visualised, only portal vein and hepatic artery visualised
How is the diagnosis confirmed?
A cholangiogram: ERCP - endoscopic retrograde cholangiopancreatography or operative. Failure to outline a normal biliary tree.
Liver biopsy initially demonstrates neonatal hepatitis with features of extrahepatic biliary obstruction developing with time
How is it managed?
Surgical intervention is the only definitive treatment for biliary atresia
What increases the success rate?
Early surgery
What surgery procedure is done?
A Kasai hepatoportoenterostomy = a loop of jejunum anastomosed to the cut surface of the porta hepatis - bypasses the fibrotic ducts and facilitates drainage of bile from any remaining patent ductules
Even with successful clearance of the jaundice after Kasai surgery, does the disease progress?
Yes in most children - to develop cholangitis and cirrhosis with portal hypertension
What supplementation is essential?
Nutrition and fat soluble vitamin supplementation A,D,E,K
If the Kasai is unsuccessful, what is considered?
Liver transplant
Is biliary atresia the most common indication for liver transplant in paediatric age group?
Yes
