Benign Biliary Tree Disease Flashcards
What is the definition of Primary Sclerosing Cholangitis (PSC)?
Progressive inflammation and obliterative fibrosis of intrahepatic and extrahepatic biliary tree.
PSC is characterized by the hardening and narrowing of bile ducts, leading to biliary obstruction.
What is the male-to-female ratio for Primary Sclerosing Cholangitis?
2:1
PSC is more common in males than females.
At what age is Primary Sclerosing Cholangitis commonly diagnosed?
40 years old
What is the estimated incidence of Primary Sclerosing Cholangitis?
1 in 100,000
What is the aetiology of Primary Sclerosing Cholangitis?
Unknown (? Autoimmune)
PSC is often associated with inflammatory bowel disease (IBD) and other conditions.
Which conditions are associated with Primary Sclerosing Cholangitis?
- Inflammatory Bowel Disease (CD, UC)
- Reidel’s thyroiditis
- Retroperitoneal fibrosis
- Lymphoplasmacytic sclerosing pancreatitis
What are the main pathological processes involved in PSC?
Inflammation in the biliary tree leading to sclerosis and narrowing of bile ducts, causing biliary obstruction and chronic cholestasis.
What are the early symptoms of Primary Sclerosing Cholangitis?
Asymptomatic (↑ LFT)
Patients may have elevated liver function tests without other symptoms.
What are the late symptoms of Primary Sclerosing Cholangitis?
- Pruritus
- Pain
- Fever
- Jaundice
- Weight loss
- Liver failure
What is the diagnostic criterion for Primary Sclerosing Cholangitis?
Based on having 2 out of 3 of the following:
* ALP > 1.5x the upper limit of normal
* Cholangiography demonstrating biliary strictures or irregularity
* Liver histology (if available)
What are common investigations for Primary Sclerosing Cholangitis?
- Obstructive LFT
- ANCA +ve (80%)
- MRCP/ERCP – Diffusely strictured intrahepatic bile duct
What is the definitive treatment for Primary Sclerosing Cholangitis?
Liver transplantation
What are the complications associated with Primary Sclerosing Cholangitis?
- ↑ Risk of malignancy (cholangiocarcinoma, GB cancer)
- Pancreatitis
- Liver failure/cirrhosis
What is the prognosis for patients with Primary Sclerosing Cholangitis?
Poor prognosis (9.5 year survival)
What is a choledochal cyst?
Abnormal cystic dilatation of bile ducts.
What is the estimated incidence of choledochal cysts?
1 in 200,000
In which population are choledochal cysts more prevalent?
Asia (F>M – 4:1)
What is the aetiology of choledochal cysts?
During week 5, abnormal creation of biliary tree -> leads to CBD + pancreatic duct sharing a LONG COMMON DUCT (few cm) -> reflux of pancreatic juice into biliary tree -> chronic damage/ inflammation -> cystic dilatation.
What classification system is used for choledochal cysts?
Classification of Todani
What presentations are common for choledochal cysts?
Triad of mass, jaundice, pain.
What is the primary investigation for choledochal cysts?
Cholangiogram (CT cholangiogram, MRCP, PTC, ERCP)
What is the risk associated with choledochal cysts?
High rate of malignancy - adenocarcinoma, squamous carcinoma, cholangiocarcinoma
What is cholangiohepatitis?
Cholangiohepatitis = hepatolithiasis = oriental cholangitis = recurrent pyogenic cholangitis.
What are common aetiologies of cholangiohepatitis?
- Parasites (e.g., liver fluke, Clonorchis Sinensis)
- Bacterial