Primary sclerosing cholangitis

Case contributed by Andrew Lawson
Diagnosis almost certain

Presentation

Deranged LFT's.

Patient Data

Age: 30 years
Gender: Female

The liver demonstrates marked surface nodularity and diffusely abnormal parenchymal architecture throughout both lobes with regions of capsular retraction. Segments 4A/B, 3 and 8 are largely spared. The gallbladder is distended. The extrahepatic duct is not enlarged or severely strictured. No intrahepatic biliary ductal dilatation but there are multiple typically beaded appearance of the visualized biliary tree. No filling defects or convincing dominant stricture identified. Evaluation of the extrahepatic biliary confluence is obscured by motion artefact. The pancreatic duct and pancreas unremarkable. The spleen is markedly enlarged measuring 19 cm in the craniocaudal dimension. The splenic vein is enlarged and multiple hilar varices are visible. The inferior mesenteric vein is dilated.

Comment: Findings typical of advanced PSC without an obvious dominant stricture.

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