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Findings: There is narrowing and tapering of the extrahepatic duct inferiorly. There is also slight beading with areas of narrowing seen in the intrahepatic ducts.
Diagnosis: Primary sclerosing cholangitis
Discussion: Primary sclerosing cholangitis is a progressive, inflammatory process of unknown etiology involving the bile duct system. The disease affects men more often with a male to female ratio of two to one. About 70% are younger than 45 years of age at the time of diagnosis. Certain conditions are associated with primary sclerosing cholangitis, most notably ulcerative colitis. Other associated conditions are sicca complex, Riedel's stroma, retropertioneal fibrosis, and mediastinal fibrosis.
Clinically, primary sclerosing cholangitis can cause fatigue, pleuritis, jaundice, right upper quadrant pain, and hepatosplenomegaly. There may also be elevations in serum alkaline phosphatase, serum billirubin, and serum spartate transamylase levels. Patients with primary sclerosing cholangitis have a five year survival of 88% and a median survival of 11.9 years from the time of diagnosis. Liver transplant is the only effective therapy at this time.
Pathologically, primary sclerosing cholangitis is characterized by fibrosing inflammation of the biliary tree. Tissue samples may show areas of cholangiectasis in combination with fibrous obliteration of large intrahepatic bile ducts. There are four histologic stages of primary sclerosing cholangitis. 1) portal hepatitis or cholangitis; 2) periportal hepatitis or fibrosis; 3) septal fibrosis bridging necrosis; 4) cirrhosis.
Radiographically, the most important findings are found on cholangiography. Multiple segmental strictures involving the intrahepatic and extrahepatic bile ducts are the hallmark of primary sclerosing cholangitis. Normal duct segments alternating with segmental stricutres produce a "beaded" appearance. Strictures can vary in length from 1 to 2 mm to several cm. There can also be a "pruned tree" appearance when the peripheral ducts are completely obliterated. Diverticular out-pouchings can also be seen in 25% of patients. About 50% of patients have mucosal irregularities ranging from a fine brush-border appearance to a coarse, shaggy or nodular appearance. Some differential diagnostic considerations include: cholangiocarcinoma (especially when there is metastatic spread involving the biliary tree diffusely), primary biliary cirrhosis, parasitic infestations, allograft rejection after liver transplantation.
References:
1. Gore RN, Levine NS, Laufer I: Textbook of Gastrointestinal Radiology,
1994, WB Saunders Company, Philadelphia.
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