SPR Unknown # 94 -- FINAL

Agustin Cardenas, MD

UCSF Benioff Children's Hospital

Abstract


Publication Date: 2013-08-08

History

17-year-old woman with chronic right upper quadrant and epigastric pain. The patient had a previous history of autoimmune hepatitis and nonspecific cholangiopathy.

Findings

Fig. A: MRCP. MIP (Maximum Intensity Projection) showing multiple strictures and a “string of beads” appearance of the intrahepatic bile ducts (arrow).

Fig. B: MRCP. Volume-rendering reconstruction showing a dilated common bile duct. No gallstones or ductal masses were present. The pancreatic duct was not dilated and the gallbladder was normal.

Fig. C: Coronal T2 SSFSE acquisition of the upper abdomen. The common bile duct is moderately dilated and the intrahepatic branches show multiple strictures. There are no radiologic findings that suggest underlying cirrhosis, such as irregular contour of the liver nor presence of ascites.

Discussion

The findings shown on the MRCP are highly suggestive of PSC (primary sclerosing cholangitis). Pathology showed an autoimmune cholangiopathy. The final diagnosis was primary sclerosing cholangitis.

This rare condition is characterized by diffuse cholangitis and progressive fibrosis of the extra- and intrahepatic bile ducts. Differential diagnosis includes ascending cholangitis, AIDS cholangiopathy, sclerosing cholangiocarcinoma, cirrhosis and chemotherapy-induced cholangitis.

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