An endoscopic finding in patients with primary sclerosing cholangitis: retraction of the main duodenal papilla into the duodenum wall.

Background: The diagnosis of primary sclerosing cholangitis (PSC) is reached by typical cholangiographic findings and liver biopsy. The characteristic cholangiographic abnormalities consist of multifocal strictures and dilatations in the intrahepatic and/or extrahepatic bile ducts. Patients may develop cirrhosis and portal hypertension. Endoscopy may reveal esophageal/gastric varices and portal hypertensive gastropathy.

Objective: To define a novel endoscopic finding in patients with PSC.

Design: Case series.

Setting: Single tertiary referral center in Turkey.

Patients: Ten patients with PSC, 16 with liver cirrhosis, and 10 with PSC-like cholangiogram.

Interventions: Inspection of papilla with duodenoscope and ERCP.

Main Outcome Measurements: Retraction of papilla into the duodenum wall.

Results: Ten patients with PSC (8 male, 2 female, mean age 38 y) underwent ERCP at our institution. Retraction of papilla into the duodenum wall was observed in 7 patients (70%). The mean time elapsed between the retraction of the papilla and onset of PSC was 5.1 years (range 2-7 y). In patients with retraction of the papilla, both of the intrahepatic and extrahepatic bile ducts were involved; however, only the intrahepatic bile ducts were involved in patients with no retraction of papilla. Of 7 patients with retraction of papilla, 5 had a history of sphincterotomy procedure 5.4 years previously (range 5-6 y). Two patients had native papilla. None of the patients had end stage liver disease. Retraction of papilla was observed in none of the 16 patients with cirrhosis of the liver and in 10 with PSC-like cholangiogram.

Limitations: Only observational; absence of surgical and/or pathologic evidence.

Conclusions: Papilla may be embedded in the duodenum wall in some patients with PSC. Extrahepatic involvement seems to be necessary for the occurrence of this finding.