Biliary atresia
definition
characterised by obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction to bile flow
aetiology
incidence
pathophysiology
type 1
obliteration of common duct, proximal ducts are patent
type 2
atresia of hepatic duct, with cystic structures found in porta hepatitis
type 3 (>90%)
atresia of right and left hepatic ducts to the level of porta hepatis
?
congenital malformation of biliary ductular system
toxic/infectious cause of bile duct obliteration
progressive inflammatory lesions
?
infectious agents
highest in Asian population
1 per 10,000
prognosis
complications
disease
surgery
biliary cirrhosis
cholangitis
portal HTN
HCC
F>M
neonate period
clinical
symptoms
signs
dark urine
jaundice
light stool
jaundice
poor feeding
failure to thrive
hepatomegaly
splenomegaly
hyperbilirubinaemia
cardiac murmur
genetic factors
diagnosis
ancillary
serum bilirubin
conjugated
ALP, GGT
serum alpha1-antitrypsin
sweat chloride
USS
percutaneous liver biopsy
intraoperative cholangiography
hepatobiliary scintiscanning
treatment
surgery