An unusual presentation of primary sclerosing cholangitis

Abstract

This case report describes the unusual presentation of a patient who had findings which were initially suggestive of a type Ⅳ choledochal cyst. Her liver biopsy demonstrated biliary cirrhosis. She was treated with endoscopic retrograde cholangiopancreatography and biliary stent exchanges over one year. Her cholangiogram one year later demonstrated resolution of the biliary cystic dilation which led to her initial diagnosis, with beading and stricturing of the hepatic ducts consistent with primary sclerosing cholangitis. Liver-associated enzymes and physical findings also improved. A liver biopsy one year later demonstrated a marked improvement in hepatic fibrosis with no evidence of cirrhosis.

Primary Sclerosing Cholangitis: Diagnostic Criteria

“…Lastly, the occurrence of biliary cystic dilatation (illustrated in Figure 3 ) within the intrahepatic bile ducts was recorded in 21 patients diagnosed with PSC [ 41 , 42 , 43 , 44 , 45 , 46 , 47 ] as well as in five livers that underwent explantation [ 48 ].…”

“…Lastly, the occurrence of biliary cystic dilatation (illustrated in Figure 3) within the intrahepatic bile ducts was recorded in 21 patients diagnosed with PSC [41][42][43][44][45][46][47] as well as in five livers that underwent explantation [48]. Lastly, the occurrence of biliary cystic dilatation (illustrated in Figure 3) within the intrahepatic bile ducts was recorded in 21 patients diagnosed with PSC [41][42][43][44][45][46][47] as well as in five livers that underwent explantation [48].…”