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Primary sclerosing cholangitis

Case contributed by Jan Frank Gerstenmaier
Diagnosis almost certain

Presentation

Known Crohn disease presented with abnormal liver enzymes.

Patient Data

Age: 55 years
Gender: Male

MRCP

mri

There is atrophy of the right lobe of the liver. The intrahepatic biliary tree demonstrates multifocal stricturing and beading. There is a short stricture just central to the confluence with a short segment of mild duct dilatation at 7mm with a normal caliber CBD distally at 4mm. The pancreatic duct is normal. Appearances are in keeping with early PSC.

Case Discussion

The association between primary sclerosing cholangitis and inflammatory bowel disease is strong (in 70% of cases), as in this patient. Caudate hypertrophy is often seen in advanced disease (not present here). The most feared complication is cholangiocarcinoma which develops in up to 15% of patients.,

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