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BILIARY ATRESIA
Sameh Shehata
BILIARY ATRESIA
Progressive obliterative
cholangiopathy
incidence 1:10,000 to 1:15,000
Girls > boys
Etiology
1. Reovirus 3 infection.
2. Congenital malformation.
3. Congenital cytomegalovirus (CMV).
4. Autoimmune
5. Genetic
Types
ASSOCIATED ANOMALIES
Associated anomalies in 10 to 20 % of cases.
 Polysplenia syndrome:
− Cardiac malformations
− Polysplenia
− Situs inversus
− Absent vena cava
− A preduodenal portal vein
-- Bilobed symmetric liver
-- Bilobed lungs
BASM
Syndrome
Direct hyperbilirubinemia
Biliary atresia simplified
Main DD
Investigations
• Liver function tests.
• US
• HIDA Isotope study.
• Percutaneous liver biopsy.
• Cholangiography.
Biliary USG
to exclude other etiologies like Choledochal cyst
GB absent / contracted / distended.
Triangular cord sign: Presence of fibrous cone of bile
duct remnant at porta hepatis
Triangular Cord Sign
Triangular Cord Sign Micky Mouse Sign
ISOTOPE SCAN HIDA
Normal uptake / No excretion
Poor uptake / Good excretion
NNT hepatitis
PERCUTANEOUS CHOLANGIOGRAPHY
Biliary atresia
(showing absent intrahepatic ducts /patent distal bile ducts
normal biliary tree with contrast draining
Biliary atresia simplified
Percutaneous liver biopsy:
• bands of fibrous tissue
• bile duct proliferation.
• Giant cells.
Kasai ( porto-enterostomy)
Before 60 days
Biliary atresia simplified
Post Kasai
1/3rd of pts remain asymptomatic No transplant
1/3 never have bile flow and require Early transplant
1/3 initially have good bile flow but subsequently
develop cirrhosis Late transplant
2/3 will need: Liver Tx
Prognosis
Without surgery or liver transplant life span – 19
months
Death is due to liver failure, bleeding esophageal
varices and sepsis
Biliary Atresia - Mindmap
Biliary atresia simplified

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Biliary atresia simplified