What is the recommended endoscopic treatment for primary sclerosing cholangitis (PSC) with dominant strictures?

Endoscopic Treatment of Primary Sclerosing Cholangitis with Dominant Strictures

Balloon dilation alone should be the first-line endoscopic treatment for dominant strictures in PSC, with short-term stenting (1-2 weeks) reserved only for strictures that fail to respond adequately to balloon dilation. 1, 2

Initial Diagnostic Workup Before Intervention

Before any endoscopic therapy, you must exclude cholangiocarcinoma, which occurs in 26% of PSC patients with dominant strictures and often presents within 4 months of PSC diagnosis 3:

• Perform MRCP or contrast CT and review with a hepatopancreaticobiliary multidisciplinary team before high-risk interventions 1
• Conduct biliary brush cytology at ERCP (sensitivity 43%, specificity 97% for cholangiocarcinoma) 1
• Consider cholangioscopy with direct visualization if available (sensitivity 50-62% in PSC) 1
• Suspect malignancy with worsening cholestasis, weight loss, elevated tumor markers, or progressive dominant stricture 2

Prophylactic Measures

Administer prophylactic antibiotics before every ERCP in PSC patients - this is a strong recommendation given the high risk of cholangitis 1, 2

First-Line Treatment: Balloon Dilation Technique

The preferred approach is balloon dilation without routine stenting 1, 2:

Balloon Selection and Technique

• Select balloon diameter up to 6-8 mm for hepatic ducts and up to 8 mm for common bile duct, matching the caliber of ducts delimiting the stricture 1, 2
• Perform stepwise dilation to avoid exceeding upstream/downstream duct diameter 1
• Repeat dilations at 1-4 week intervals until technical success, typically requiring 2-3 sessions 1, 2
• Technical success = complete balloon inflation with no waist on fluoroscopy plus unobstructed contrast passage to duodenum 1, 2

Safety Profile

Balloon dilation has superior safety compared to stenting 1, 2:

• Bile duct perforation: 0.2% of procedures (versus 3.5% with aggressive dilation) 1
• Overall complications: 15% versus 54% with stenting 2
• Cholangitis: 18% versus 50% with stenting 1

Long-Term Outcomes

A large prospective study (n=96 patients, 500+ dilations over median 7 years) demonstrated 1:

• 5-year transplant-free survival: 81%
• 10-year transplant-free survival: 52%
• Low complication rates: 2.2% pancreatitis, 1.4% cholangitis

Second-Line Treatment: Short-Term Stenting

Reserve stenting only for strictures refractory to balloon dilation alone 1, 2:

Stent Selection

• Single 10-Fr stent for extrahepatic dominant strictures 1
• Two 7-Fr stents for hilar strictures extending into left or right hepatic duct 1
• Use stepwise approach: start with 7-Fr stent for 1 week if 10-Fr cannot be placed initially 1

Duration

Remove stents after 1-2 weeks (mean 9-11 days) - short-term stenting shows similar efficacy to standard 8-12 week stenting but with lower complication rates 1:

• Resolution of jaundice/cholestasis: 81% versus 57% with standard stenting 1
• Stents clog rapidly in PSC, making prolonged stenting problematic 1
• The European DILSTENT trial was stopped early due to significantly higher serious adverse events with stenting versus balloon dilation 2

Role of Biliary Sphincterotomy

Do not perform routine biliary sphincterotomy - use it selectively 2:

• Consider only after difficult cannulation in patients requiring multiple ERCPs 2
• PSC patients have increased short-term adverse events with sphincterotomy, though it may be protective for subsequent procedures 2

Follow-Up Strategy

Multiple interventions are typically required over months to years 1:

• Median of 3 interventions per patient with dominant strictures (versus 0 in those without) 3
• Repeat dilation for relapsing dominant strictures if: (1) the stricture causes recurrent symptoms (cholangitis, pruritus) or significant cholestasis increase, AND (2) previous dilations showed satisfactory response 1
• Regular endoscopic follow-up at 3,6,12,18, and 24 months, then yearly 4

Expected Clinical Outcomes

Endoscopic therapy typically improves 1, 4:

• Serum bilirubin: 73% reduction
• Alkaline phosphatase: 46% reduction
• Gamma-glutamyl-transpeptidase: 55% reduction
• Pruritus and cholangitis symptoms

However, survival remains worse in patients with dominant strictures (mean 13.7 years) compared to those without (23 years), largely due to 26% cholangiocarcinoma risk 3.

Critical Pitfalls to Avoid

• Never ignore new or worsening dominant strictures - half of cholangiocarcinomas present within 4 months of PSC diagnosis 3
• Avoid long-term stenting - associated with high rates of stent occlusion and cholangitis within 3 months 1
• Do not use aggressive balloon sizes - one study using 4-12 mm balloons reported 3.5% perforation rate versus 0.2% with conservative sizing 1
• Always obtain tissue sampling before therapeutic intervention to exclude malignancy 2

When to Consider Alternative Approaches

• Refractory strictures despite repeated endoscopic therapy: consider percutaneous transhepatic drainage or surgical intervention in non-cirrhotic patients 2
• Recurrent bacterial cholangitis despite endoscopic therapy: evaluate for liver transplantation 2
• Development of cholangiocarcinoma: liver transplantation is contraindicated in the UK but some centers consider it for biliary dysplasia 1