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