Caroli's disease and liver transplantation

✍ Scribed by Shahid Habib; Obaid S. Shaikh

• Publisher: John Wiley and Sons
• Year: 2007
• Tongue: English
• Weight: 57 KB
• Volume: 14
• Category: Article
• ISSN: 1527-6465
• DOI: 10.1002/lt.21379

✦ Synopsis

Caroli's disease is a rare indication for liver transplantation that accounts for 0.13% of all liver transplants performed in the United States. 1 It was first described in 1958 by the French physician Jacques Caroli (1902-1979). 2 The disease is characterized by segmental dilatation of the intrahepatic biliary tree, and it has an autosomal recessive inheritance pattern. In patients with coexistent congenital hepatic fibrosis, it is termed Caroli's syndrome. The disease often develops in association with autosomal recessive polycystic kidney disease. Caroli's disease belongs to the group of fibrocystic liver diseases that develop as a result of biliary ductal plate malformation. In the human embryo, liver development begins in the third week of gestation in the form of a hollow endodermal outgrowth from the ventral foregut or future duodenum into the mesodermal septum transversum. 3 The progenitor hepatic parenchymal cells or hepatoblasts soon align themselves as doublelayered sheets in proximity with the vitelline capillary plexus to create what is termed the ductal plate. At 12 weeks of gestation, the ductal plate starts to remodel to form tubular and cylindrical structures accompanied by resorption of excess epithelial cells. The ductal plate remodeling persists throughout the fetal life, with a gradient directed from the hepatic hilum to the periphery that results in the formation of a network of bile ducts within the portal tracts. The gallbladder, cystic duct, and extrahepatic biliary tree develop from the caudal part of the embryonic endodermal projection. 3 Defective ductal plate remodeling may involve all levels of the intrahepatic biliary tree, with large duct involvement in Caroli's disease and smaller portal bile duct malformation in congenital hepatic fibrosis.