Original Article: Clinical Endoscopy
Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-term treatmentDisclosure,

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Background

Primary sclerosing cholangitis is characterized by progressive fibrotic inflammation and obliteration of intra- and/or extrahepatic bile ducts. Total or subtotal stenoses of major bile ducts are associated with reduced survival.

Objective

To evaluate the outcome after long-term endoscopic treatment.

Design

Prospective, single-center study.

Setting

Tertiary care academic medical center.

Patients

A total of 171 patients treated with ursodeoxycholic acid were followed for as long as 20 years. At entry, 20 patients had dominant stenoses, and during a median follow-up period of 7.1 years, dominant stenosis developed in another 77.

Interventions

Ninety-six patients with dominant stenoses were treated by repeated balloon dilation; 5 patients with complete obstruction with bacterial cholangitis were stented.

Main Outcome Measurements

Survival free of liver transplantation, number of procedures, complications.

Results

In total, 500 balloon dilations were performed and 5 stents were placed. Complications were pancreatitis (2.2%), bacterial cholangitis (1.4%), and bile duct perforation (0.2%); there were no deaths. Repeated endoscopic interventions allowed the preservation of a functioning common bile duct and of at least 1 hepatic duct up to 2 cm above the bifurcation in all patients. Progression of intrahepatic bile duct and liver disease led to the need for liver transplantation in 22 of 96 patients. Five years after the first dilation of a dominant stenosis, the survival free of liver transplantation rate was 81%, and after 10 years, it was 52%.

Limitations

Single-center study, no control group, primary end-stage liver disease excluded.

Conclusion

Repeated endoscopic balloon dilations of dominant stenoses allow the preservation of a functioning common bile duct for many years.

Section snippets

Study design

This prospective study began in May 1987 and continued until July 2006. The outcomes of all patients in the study were followed until June 2007.

Patient selection and characteristics

The selection criteria for enrolling patients with PSC in the study included typical endoscopic retrograde cholangiography (ERC) findings, serum alkaline phosphatase activity of at least twice the normal range, negative antimitochondrial antibody, and liver biopsy findings compatible with the diagnosis of PSC. Routine laboratory values were determined

Results

Total or subtotal stenoses of major bile ducts were found in 97 patients (1 with choledochojejunostomy had a cholangiocarcinoma) (Table 1). Of 96 carcinoma-free patients, 37 were symptomatic, 32 had jaundice with a serum bilirubin level greater than 2 mg/dL, 6 patients had jaundice as well as pruritus, and 5 had pruritus without jaundice; 41 patients were subicteric and had a serum bilirubin level of 1.0 to 2.0 mg/dL. Of the 23 patients with normal serum bilirubin levels, 10 had intermittently

Discussion

In PSC, dominant stenoses of major bile ducts may lead to severe cholestasis, are associated with reduced survival,24 and in general require endoscopic opening.13, 15, 16, 17, 18, 19, 20, 21, 22, 23 Originally it was thought that short-segment stenoses may be opened endoscopically, whereas there was little experience with the opening of long-segment stenoses. In recent years, it became clear that long-segment stenoses of the common duct more than 2 cm in length may be dilated and kept open for

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      Among endoscopic therapies, biliary endoscopic balloon dilation (EBD) is generally preferred over endoscopic biliary stenting (EBS) in the management of long PSC strictures, whenever malignancy is excluded.26 Gotthardt and colleagues in 2010 reported single-center 20-year experience of their cohort of patients with PSC (n = 171), of which 20 had DS at initiation period, and 77 more patients developed DS over time.27 500 serial EBD were performed over the years, with reported adverse events of pancreatitis (2.2%), bacterial cholangitis (1.4%), and bile duct perforation (0.2%), whereas only 5 patients with complete obstruction with bacterial cholangitis were stented.27

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    Disclosure

    All authors disclosed no financial relationships relevant to this publication.

    See CME section; p. 596

    Current affiliations: Department of Medicine (D.G., G.R., P.K.-P., A.S.), University of Heidelberg, Heidelberg, Germany, Department of Medicine (H.K.), University of Ulm, Ulm, Federal Republic of Germany.

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