John Libbey Eurotext

Hépato-Gastro & Oncologie Digestive


Hepatolithiasis Volume 21, issue 8, Octobre 2014


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Hôpital Saint-Antoine,
service d’hépatologie,
184 rue du Faubourg Saint Antoine,
75571 Paris Cedex 12, France ;
INSERM, UMR_S 938, CDR Saint-Antoine,
F-75012, Paris, France ;
Sorbonne Universités, UPMC Univ Paris 06,
UMR_S 938, CDR Saint-Antoine,
F-75005, Paris, France
* Tirés à part

Hepatolithiasis is defined as the presence of gallstones in the bile ducts proximal to the confluence of the right and left hepatic ducts. Gallstones in hepatolithiasis are typically of two types: brown pigment stones (mainly made of calcium bilirubinate following formation of unconjugated bilirubin in the setting of biliary stasis or bacterial infection) and cholesterol stones (due to a decrease in biliary phospholipids secretion and subsequent impaired cholesterol solubility secondary to ABCB4 gene defect with mutations identified in 50% of cases). Despite some common features and the occurrence of intermediate forms, these two types usually differ in terms of clinical presentation, prognosis and management. Cholesterol hepatolithiasis has been described in western countries as LPAC (low phospholipid associated cholelithiasis) syndrome and is typically characterized by recurrence of biliary symptoms after cholecystectomy; stones are usually (very) small without any associated biliary tract abnormalities and diagnosis is based on ultrasonography; ABCB4 genotyping is mandatory and therapy with ursodeoxycholic acid is highly effective. Pigment hepatolithiasis is more frequent in East Asian countries and is associated with dilatations (sometimes congenital) or stenosis (of various causes) of intrahepatic ducts ; stones are bigger, symptoms may vary from none to severe acute cholangitis or liver abscess and MRCP is the most useful tool in the pre-therapeutic work-up; ursodeoxycholic acid may have some potential but symptomatic patients require a complex management (clearance of stones and elimination of bile stasis) based on a multidisciplinary approach (endoscopic, percutaneous, surgical).