Hepatolithiasis, or the presence of intrahepatic stones, is prevalent
in East Asia and is characterized by the finding of stones within the
intrahepatic bile ducts proximal to the confluence of the right and le
ft hepatic ducts. Bile stasis and bacterial infection have been incrim
inated as the major aetiopathogenic factors. Clinical features include
recurrent pyogenic cholangitis, multiple liver abscesses, secondary b
iliary cirrhosis and cholangiocarcinoma. The goals of management inclu
de accurate localization of pathologies, control of biliary sepsis and
the elimination of stones and stasis. Ultrasonography, computed tomog
raphy and direct cholangiography complement each other in defining the
stones, strictures and degree of liver damage. Non-operative biliary
decompression by endoscopy and interventional radiology is effective i
n controlling the infection, but surgery remains the mainstay for the
treatment of stones and strictures. Intra-operative ultrasound and fle
xible choledochoscopy, combined with percutaneous transhepatic cholang
ioscopy and intraductal lithotripsy, facilitate stone removal. Balloon
dilatation and biliary stenting serve to open the bile duct stricture
s. The creation of a hepaticocutaneous jejunostomy after conventional
surgery allows atraumatic access to the biliary system for the removal
of recurrent stones. The management of biliary parasites begins with
conservative measures, including analgesics and anti-helminthic therap
y. In refractory cases or patients with acute cholangitis, endoscopic
biliary drainage and the extraction of worms may be necessary. Improve
ment in sanitation plays a crucial role in the epidemiological control
of these biliary diseases.