Benign Biliary Tree Disease Flashcards

1
Q

What is the definition of Primary Sclerosing Cholangitis (PSC)?

A

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.

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2
Q

What is the male-to-female ratio for Primary Sclerosing Cholangitis?

A

2:1

PSC is more common in males than females.

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3
Q

At what age is Primary Sclerosing Cholangitis commonly diagnosed?

A

40 years old

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4
Q

What is the estimated incidence of Primary Sclerosing Cholangitis?

A

1 in 100,000

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5
Q

What is the aetiology of Primary Sclerosing Cholangitis?

A

Unknown (? Autoimmune)

PSC is often associated with inflammatory bowel disease (IBD) and other conditions.

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6
Q

Which conditions are associated with Primary Sclerosing Cholangitis?

A
  • Inflammatory Bowel Disease (CD, UC)
  • Reidel’s thyroiditis
  • Retroperitoneal fibrosis
  • Lymphoplasmacytic sclerosing pancreatitis
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7
Q

What are the main pathological processes involved in PSC?

A

Inflammation in the biliary tree leading to sclerosis and narrowing of bile ducts, causing biliary obstruction and chronic cholestasis.

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8
Q

What are the early symptoms of Primary Sclerosing Cholangitis?

A

Asymptomatic (↑ LFT)

Patients may have elevated liver function tests without other symptoms.

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9
Q

What are the late symptoms of Primary Sclerosing Cholangitis?

A
  • Pruritus
  • Pain
  • Fever
  • Jaundice
  • Weight loss
  • Liver failure
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10
Q

What is the diagnostic criterion for Primary Sclerosing Cholangitis?

A

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)

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11
Q

What are common investigations for Primary Sclerosing Cholangitis?

A
  • Obstructive LFT
  • ANCA +ve (80%)
  • MRCP/ERCP – Diffusely strictured intrahepatic bile duct
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12
Q

What is the definitive treatment for Primary Sclerosing Cholangitis?

A

Liver transplantation

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13
Q

What are the complications associated with Primary Sclerosing Cholangitis?

A
  • ↑ Risk of malignancy (cholangiocarcinoma, GB cancer)
  • Pancreatitis
  • Liver failure/cirrhosis
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14
Q

What is the prognosis for patients with Primary Sclerosing Cholangitis?

A

Poor prognosis (9.5 year survival)

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15
Q

What is a choledochal cyst?

A

Abnormal cystic dilatation of bile ducts.

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16
Q

What is the estimated incidence of choledochal cysts?

A

1 in 200,000

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17
Q

In which population are choledochal cysts more prevalent?

A

Asia (F>M – 4:1)

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18
Q

What is the aetiology of choledochal cysts?

A

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.

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19
Q

What classification system is used for choledochal cysts?

A

Classification of Todani

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20
Q

What presentations are common for choledochal cysts?

A

Triad of mass, jaundice, pain.

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21
Q

What is the primary investigation for choledochal cysts?

A

Cholangiogram (CT cholangiogram, MRCP, PTC, ERCP)

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22
Q

What is the risk associated with choledochal cysts?

A

High rate of malignancy - adenocarcinoma, squamous carcinoma, cholangiocarcinoma

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23
Q

What is cholangiohepatitis?

A

Cholangiohepatitis = hepatolithiasis = oriental cholangitis = recurrent pyogenic cholangitis.

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24
Q

What are common aetiologies of cholangiohepatitis?

A
  • Parasites (e.g., liver fluke, Clonorchis Sinensis)
  • Bacterial
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25
What is Type 1 in the classification of Todani?
Single fusiform or cystic dilatation of CBD ## Footnote Type 1 accounts for 80% of the cases in this classification.
26
What characterizes Type 2 in the classification of Todani?
Supraduodenal diverticulum ## Footnote Type 2 occurs in less than 1% of the cases.
27
What is Type 3 in the classification of Todani also known as?
Intraduodenal diverticulum ('choledochocoele') ## Footnote Type 3 represents about 5% of the cases.
28
What does Type 4 in the classification of Todani involve? (4a and 4b)
Multiple intra/extra-hepatic duct dilatation. 4a = IHD and EHD. 4b = EHD. ## Footnote Type 4 accounts for 30-40% of the cases.
29
What is Type 5 in the classification of Todani characterized by?
Multiple intrahepatic duct dilatations with hepatic fibrosis ('Caroli's disease') ## Footnote Type 5 represents approximately 10% of the cases.
30
What is the management approach for choledochal cyst?
High rate of malignancy, so all fit pts need to be treated
31
What surgical procedure is performed for Type 1 choledochal cyst?
Complete excision with Roux-en Y hepaticojejunostomy and cholecystectomy
32
What is the treatment for Type 2 choledochal cyst?
Simple cyst excision (CBD reconstruction over T-tube) + cholecystectomy
33
What procedure is indicated for Type 3 choledochal cyst?
ERCP, sphincterotomy, sphincteroplasty
34
What is the management for Type 4 choledochal cyst in the case of cirrhosis or portal hypertension?
Need liver transplantation. If unilateral, requires hepatic and CBD resection and hepaticojejunostomy
35
What is the treatment for Type 5 choledochal cyst?
Liver transplantation. If unilateral, hepatic resection and hepaticojejunostomy
36
What is biliary spasm?
Structural or functional abnormality of the sphincter of Oddi. ## Footnote This condition affects the flow of bile from the gallbladder into the intestine.
37
Which demographic is most commonly affected by biliary spasm?
Young women. ## Footnote Epidemiological studies suggest a higher prevalence in this group.
38
What is the primary aetiology of biliary spasm?
Cause unknown, but associated with diffuse enteric motility disorder. ## Footnote This suggests a functional aspect rather than a clear structural cause.
39
What can cause secondary biliary spasm?
Damage and fibrosis from: * Previous gallstone migration * Sphincterotomy * Pancreatitis * Trauma ## Footnote These causes lead to physical changes that affect the sphincter.
40
What are the typical symptoms of biliary spasm?
Typical biliary type pain, recurrent and does not improve with cholecystectomy. ## Footnote Pain is often worse on eating and located in the RUQ or epigastric area.
41
What percentage of patients with biliary spasm experience recurrent pancreatitis?
30%. ## Footnote This is a significant complication of the condition.
42
What blood tests might indicate biliary spasm?
Increased amylase and GGT. ## Footnote These tests help identify issues with bile and pancreatic function.
43
What ultrasound finding is associated with biliary spasm?
CBD >12mm. ## Footnote This indicates potential dilation of the common bile duct.
44
What is the response to CCK in biliary spasm?
Dilatation of CBD. ## Footnote CCK (cholecystokinin) is a hormone that stimulates bile release.
45
What is the response to secretin in biliary spasm?
Dilatation of pancreatic duct. ## Footnote Secretin helps regulate pancreatic secretions.
46
What does delayed excretion of contrast on ERCP indicate?
Biliary spasm. ## Footnote ERCP (endoscopic retrograde cholangiopancreatography) is used to visualize the bile ducts.
47
What sphincter manometry finding is associated with biliary spasm?
Resting pressure >40mmHg. ## Footnote This indicates abnormal sphincter function.
48
What are the management options for biliary spasm?
Management includes: * Antispasmodics * Endoscopic sphincterotomy * Surgical transduodenal sphincteroplasty and trans-ampullary septotomy. ## Footnote These treatments aim to relieve symptoms and improve bile flow.
49
What are potential complications of biliary spasm?
Complications include: * Recurrent pancreatitis * Cholangitis ## Footnote Both complications can lead to further health issues.
50
What are the outgrowths of the mucosal wall in the gallbladder called?
Polyps ## Footnote Polyps can be classified into several types including pseudotumours, adenomas, and other benign tumors.
51
What is a cholesterol polyp?
An accumulation of lipid in gallbladder wall mucosa ## Footnote Also known as cholesterolosis, these are typically pedunculated echogenic lesions.
52
What is the typical size of cholesterol polyps?
Usually <1cm ## Footnote They are often multiple lesions.
53
What are adenomyomas?
Overgrowth of mucosa, thickening of muscle wall ## Footnote These lesions are usually sessile and often >1cm in diameter, typically located in the gallbladder fundus.
54
What is the size range for inflammatory polyps?
5-10 mm ## Footnote They can be sessile or pedunculated and may contain granulation and fibrous tissue with plasma cell/lymphocyte infiltrate.
55
What characterizes adenomas in the gallbladder?
Benign growths that are difficult to distinguish from adenocarcinoma on ultrasound ## Footnote They are considered premalignant and are rarer than adenocarcinoma.
56
What are the malignant lesions associated with gallbladder polyps?
Adenocarcinoma, squamous cell carcinoma, mucinous cystadenomas, and adenoacanthomas ## Footnote These are serious conditions that require prompt medical attention.
57
What percentage of patients undergoing ultrasound have polyps?
3-6% ## Footnote The majority of these are adenomyosis or cholesterol polyps.
58
What is the prevalence of cholesterolosis?
9-26% ## Footnote It has similar risk factors to gallstone disease.
59
What is adenomyomatosis associated with?
Gallstones ## Footnote It has a prevalence of 1% and is more common in females.
60
What is the incidence of adenomas?
0.5% ## Footnote These are benign epithelial cell tumors that can progress to malignancy.
61
What is a key pathological feature of cholesterolosis?
Abnormal deposit of TGs, cholesterol precursors, and cholesterol esters in gallbladder mucosa ## Footnote This condition is characterized by yellow deposits within the gallbladder, known as 'strawberry gallbladder.'
62
What types of structures can adenomyomatosis form?
Diffuse, segmental, or localized structures ## Footnote The diffuse type forms cystic structures with pockets of mucus, while segmental forms rings dividing the gallbladder.
63
What size of polyp is considered always malignant?
>2cm ## Footnote This size is associated with advanced cancer.
64
What is the management for symptomatic patients with gallbladder polyps?
Cholecystectomy ## Footnote This is indicated regardless of polyp size or symptoms if gallstones are present.
65
What should be done for asymptomatic patients with polyps >20mm?
Operation ## Footnote This includes preoperative staging with CT and EUS.
66
What is the follow-up procedure for polyps 5-10mm in size?
Ultrasound surveillance at 3, 6 months, then yearly until stable in size ## Footnote This monitoring is crucial for early detection of potential malignancy.
67
What is the typical follow-up for polyps <5mm?
Follow-up at 12 months, nil further if stable ## Footnote These polyps are usually benign, often cholesterolosis.
68
What is Mirizzi syndrome?
Extrahepatic biliary stricture due to cholelithiasis ## Footnote Mirizzi syndrome occurs in 0.5% of cholecystectomies.
69
How is Mirizzi syndrome classified?
Classification includes: * Type 1 – external compression of CHD due to impacted stone in gallbladder neck/infundibulum * Type 2 – fistula <1/3 circumference of CBD * Type 3 – involvement 1/3 – 2/3 circumference of CBD * Type 4 – destruction of entire CBD wall ## Footnote CHD refers to the common hepatic duct, and CBD refers to the common bile duct.
70
What are the common presentations of Mirizzi syndrome?
Same symptoms as cholecystitis, abnormal LFTs, jaundice ## Footnote LFTs refer to liver function tests.
71
When is the diagnosis of Mirizzi syndrome often made?
Diagnosis is often made at the time of cholecystectomy.
72
What investigations are used for Mirizzi syndrome?
Investigations include: * US * MRCP * ERCP ## Footnote US is used to distinguish mirizzi vs gallbladder cancer, MRCP defines anatomy, and ERCP is for jaundice or decompression.
73
What is the management for Type 1 Mirizzi syndrome?
Laparoscopic (or traditionally open) cholecystectomy + IOC ## Footnote HJ may be required if there is a persistent stricture.
74
What is the management for Type 2, 3, and 4 Mirizzi syndrome?
Subtotal cholecystectomy and suture of bile duct OR drainage procedure, including: * Hepaticojej * Cholecysto/choledochoduodenostomy * Cholecysto/choledochojejunostomy
75
What is the management for Type 5 Mirizzi syndrome?
Division and suture of enteric fistula, drainage of biliary tree/gallbladder depending on severity of mirizzi.
76
True or False: Mirizzi syndrome can present with jaundice.
True
77
What is Rigler’s triad?
SBO, pneumobilia, ectopic gallstone (usually at vitillointestinal duct remnant in distal ileum) ## Footnote SBO stands for small bowel obstruction, pneumobilia refers to air in the biliary tree, and ectopic gallstone indicates a gallstone located outside its usual position.
78
What does SBO stand for in the context of Rigler's triad?
Small bowel obstruction ## Footnote SBO is a condition where there is a blockage in the small intestine.
79
What is pneumobilia?
Air in the biliary tree ## Footnote Pneumobilia can indicate an abnormal connection between the gastrointestinal tract and the biliary system.
80
Where is the ectopic gallstone usually found in Rigler’s triad?
At vitillointestinal duct remnant in distal ileum ## Footnote This location is significant as it relates to the embryological development of the gastrointestinal tract.