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, 3 (2), 106-117

Biliary Endoscopy in the Management of Primary Sclerosing Cholangitis and Its Complications

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Biliary Endoscopy in the Management of Primary Sclerosing Cholangitis and Its Complications

Brian M Fung et al. Liver Res.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic, idiopathic, cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts. It can affect individuals of all age groups and gender, has no established pharmacotherapy, and is associated with a variety of neoplastic (e.g. cholangiocarcinoma) and non-neoplastic (e.g. dominant strictures) hepatobiliary complications. Given these considerations, endoscopy plays a major role in the care of patients with PSC. In this review, we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of PSC. Where evidence is limited, we suggest pragmatic approaches based on currently available data and expert opinion.

Keywords: Balloon dilation; Biliary tract disease; Cholangiocarcinoma (CCA); Cholangioscopy; Dominant stricture; Endoscopic retrograde; Primary sclerosing cholangitis (PSC); cholangiopancreatography (ERCP).

Conflict of interest statement

Conflict of interest The authors declare that they have no conflict of interest.

Figures

Fig. 1.
Fig. 1.. ERCP in a patient with PSC.
ERCP with balloon occlusion cholangiogram demonstrating diffusely irregular intrahepatic bile ducts consistent with PSC. Abbreviations: ERCP, endoscopic retrograde cholangiopancreatography; PSC, primary sclerosing cholangitis.
Fig. 2.
Fig. 2.. MRCP in a patient with PSC.
MRCP demonstrating multifocal perihilar and intrahepatic ductal strictures consistent with PSC. Abbreviations: MRCP, magnetic resonance cholangiopancreatography; PSC, primary sclerosing cholangitis.
Fig. 3.
Fig. 3.. Schematic representation of diameter criteria for the diagnosis of a dominant stricture.
A dominant stricture is generally defined as a stenosis with a diameter of: (i) ≤ 1.5mm in the common bile duct or (ii) ≤ 1.0mm in the hepatic ducts within 2 cm of the hepatic ductal confluence.
Fig. 4.
Fig. 4.. Dominant stricture during ERCP.
A dominant stricture in the region of the hepatic duct is seen on ERCP. Abbreviation: ERCP, endoscopic retrograde cholangiopancreatography.
Fig. 5.
Fig. 5.. Evaluation of dominant strictures in patients with PSC: A multimodal undertaking.
Of note, ancillary modalities such as cholangioscopy and endoscopic ultrasound may be implemented in addition to ERCP with biliary brushings/biopsies, as discussed in the text, and serum liver tests including CA 19-9 should be monitored. Abbreviations: CA 19-9, carbohydrate antigen 19-9; CCA, cholangiocarcinoma; ERCP, endoscopic retrograde cholangiopancreatography; FISH, fluorescence in situ hybridization; MRCP, magnetic resonance cholangiopancreatography.
Fig. 6.
Fig. 6.. Simplified overall management algorithm of dominant strictures in patients with PSC.
The overall management of dominant strictures depends on whether malignancy is found. Balloon dilation is the preferred initial treatment modality for benign strictures, while palliative stenting is the preferred initial treatment for (unresectable) malignant strictures or for benign dominant strictures that are refractory to balloon dilation. Abbreviations: CA 19-9, carbohydrate antigen 19-9; ERCP, endoscopic retrograde cholangiopancreatography; PDT, photodynamic therapy.
Fig. 7.
Fig. 7.. Treatment of a dominant stricture in a patient with PSC.
Description: (A) Endoscopic balloon dilation of a dominant stricture. (B) Placement of a self-expandable metallic stent (SEMS) in a patient who experienced rapid stricture recurrence following balloon dilation alone and also following balloon dilation with plastic stent placement. Abbreviation: PSC, primary sclerosing cholangitis.

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