Chapter 53: Biliary Tract- Sclerosing Cholangitis

Question 1

What is it?

Answer 1

Multiple inflammatory fibrous thickenings of bile duct walls resulting in biliarystrictures

Question 2

What is its natural history?

Answer 2

Progressive obstruction possibly leading to cirrhosis and liver failure; 10% of patients will develop cholangiocarcinoma

Question 3

What is the major risk factor?

Answer 3

Inflammatory bowel disease

Question 4

What type of IBD is the most common risk factor?

Answer 4

Ulcerative colitis (≈66%)

Question 5

What are the signs and symptoms of sclerosing cholangitis?

Answer 5

Same as those for obstructive jaundice:

• Jaundice
• Itching (pruritus
• )Dark urine
• Clay-colored stools
• Loss of energy
• Weight loss

(Many patients are asymptomatic)

Question 6

How is it diagnosed?

Answer 6

• Elevated alkaline phosphatase
• AND PTC or ERCP revealing “beads on a string”appearance on contrast study

Question 7

What are the management options?

Answer 7

1. Hepatoenteric anastomosis
   
   • if primarily extrahepatic ducts are involved and resection of extrahepatic bile ducts because of the risk of cholangiocarcinoma
2. Transplant
   
   • if primarily intrahepatic disease or cirrhosis
3. Endoscopic balloon dilations