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Although orthotopic liver transplantation (OLT) is a well-established curative option for hepatocellular carcinoma, transplantation for CCA has historically been unsuccessful.
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Transplantation for intrahepatic CCA remains controversial because most studies report inferior survival of OLT for intrahepatic CCA compared with hilar CCA.
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Specific challenges associated with transplantation in the setting of neoadjuvant therapy for CCA include radiation and inflammatory changes to the porta hepatis and
Role of Transplant in Biliary Disease
Section snippets
Key points
Orthotopic liver transplantation for cholangiocarcinoma
Cholangiocarcinoma (CCA) is a malignant tumor of the biliary system consisting of adenocarcinoma 95% of the time, which is subtyped based on the location of the lesion as intrahepatic or extrahepatic (perihilar or distal).1 The incidence of CCA is estimated to be 3000 to 4000 cases per year, with perihilar representing approximately 60% to 70% of cases, intrahepatic approximately 10% of cases, and distal 20% to 30% of cases.2, 3, 4 The ductal distribution of CCA is depicted in Fig. 1. Surgical
Orthotopic liver transplantation for primary biliary cirrhosis
Primary biliary cirrhosis (PBC) is characterized by immune-mediated inflammation of bile ducts leading to granuloma formation, chronic destructive cholangitis, and subsequent cirrhosis. Patients may progress to liver failure and ultimately death without liver transplantation. Despite the established association with antimitochondrial antibodies (AMAs) the exact cause of PBC remains unclear. There are several theories for the relationship including cross reactivity with infectious sources and
Orthotopic liver transplantation for primary sclerosing cholangitis
PSC is a cholestatic disease of unknown cause characterized by recurrent multifocal strictures and dilations of the intrahepatic and extrahepatic bile ducts. An example of the classic “chain of lakes” appearance in PSC is depicted in Fig. 3. In 60% to 80% of cases PSC is associated with chronic inflammatory bowel disease (IBD), predominantly ulcerative colitis. Males are more commonly affected and the course is progressive in nature.35 The diagnosis is established based on typical
Orthotopic liver transplantation in the setting of complex biliary injuries
Both open and laparoscopic cholecystectomy are associated with common bile duct injury. The incidence of bile duct injury with the laparoscopic approach is 0.4% to 0.6%. This is higher than the previously reported incidence (0.2%–0.3%) of bile duct injury in open cholecystectomy.47, 48 During laparoscopic cholecystectomy, in contrast to open, the biliary tree is more often injured at the proximal level and likely to have an associated vascular injury. The pattern of injury is well described and
Orthotopic liver transplantation for biliary atresia
Biliary atresia (BA) is the most common pediatric liver disease leading to liver transplantation during infancy or childhood. The exact cause of BA remains unknown; however, it is an occlusive panductular cholangiopathy affecting intrahepatic and extrahepatic bile ducts.58 It typically presents in the first few weeks of life and without early recognition and surgical intervention it progresses to biliary cirrhosis, leading to liver transplantation or death by 2 years of age.
BA is divided into
Caroli disease
Caroli disease is characterized by intrahepatic cystic dilation of the bile ducts that leads to intrahepatic stones, recurrent cholangitis, eventual liver failure, and potentially CCA.76, 77 It is a rare autosomal-recessive disorder of biliary cystic diseases, and is included as type V in Todani classification of choledochal cysts. When combined with hepatic fibrosis, it is known as Caroli syndrome. Remodeling of the larger intrahepatic bile ducts results in destructive inflammation and
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Disclosure Statement: The authors have nothing to disclose.
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