Orthotopic liver transplant, recurrent primary sclerosing cholangitis

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Presentation transcript:

Orthotopic liver transplant, recurrent primary sclerosing cholangitis Chapter 12 Liver Transplantation 1 Orthotopic liver transplant, recurrent primary sclerosing cholangitis Case 12.8

Clinical Presentation 12 Liver Transplantation 2 Clinical Presentation A 66-year-old man with Crohn disease and coexisting primary sclerosing cholangitis (PSC) was transplanted for end-stage disease. Post-operative complications included bile duct strictures necessitating stent placement with eventual stent removal. Persistent elevations of the alkaline phosphatase values led to MRCP and cholangiogram showing bile duct dilatation with multifocal areas of duct narrowing without significant obstruction A transhepatic cholangiogram showed an anastomotic stricture.

Laboratory Values Course in Hospital Alk Phos: 899 12 Liver Transplantation 3 Laboratory Values Alk Phos: 899 Total Bilirubin: 1.2 AST: 112 ALT: 138 Course in Hospital The possibility of duct damage to either duct ischemia versus recurrent PSC was considered and a liver biopsy was performed approximately 5 years post-transplant.

12 Liver Transplantation 4 Pathology Moderate portal lymphocytic infiltrates were seen with prominent periductal fibrosis involving most of the interlobular bile ducts (a, b); some of the ducts also showed cytologic atypia of the duct epithelium (a). Figure 12.8(a) Figure 12.8(b)

12 Liver Transplantation 5 Pathology In addition some of the portal tracts were devoid of interlobular bile ducts (c). Figure 12.8(c)

12 Liver Transplantation 6 Diagnosis Orthotopic liver transplant (OLT), recurrent primary sclerosing cholangitis with focal bile duct loss (approximately 5 years post-transplant)

12 Liver Transplantation 7 Comment When the native liver disease is PSC which can recur post-transplant in up to one-third of patients, sometimes it is very difficult to distinguish periductal fibrosis due to post-transplant bile duct strictures versus recurrent PSC itself. When bile duct loss is also seen, recurrent PSC is more likely, as duct loss is a characteristic feature of PSC as it progresses, while duct loss is very unusual in bile duct obstruction due to post-surgical strictures. In addition multifocal both intra- and extrahepatic strictures can favor recurrent PSC, although ischemia can also at times show multifocal lesions and even duct loss long term.