Biliary atresia (BA) is the end result of a destructive, inflammatory
process that affects intra- and extrahepatic bile duets, leading to fi
brosis and obliteration of the biliary tract with the development of b
iliary cirrhosis. It is the commonest cause of chronic cholestasis in
infants and children, and therefore is the most frequent indication fo
r li ver transplantation in this age group. The disease occurs worldwi
de, affecting an estimated 1 in 8,000 to 12,000 live births. At presen
t, there is no specific therapy for BA; however, sequential surgical t
herapy begins with creation of a hepatoportoenterostomy (HPE); in thos
e with end-stage liver disease, liver transplantation is indicated. Si
nce most candidates are young children of small size, there is a short
age of size-matched donors for liver transplantation. At present, an i
ncreased awareness to ensure early diagnosis and development of method
s to prevent progressive fibrosis are needed. These considerations are
dependent on detailed studies of the pathogenesis of BA Recent studie
s have focused on normal and altered bite duct morphogenesis and the r
ole of various factors (infectious or toxic agents and metabolic insul
ts) in isolation or in combination with a genetic or immunologic susce
ptibility in the etiology of BA.