Biliary Tree Flashcards

1
Q

What is primary biliary cholangitis?

A

An autoimmune condition where T cells attack small bile ducts in the liver causing bile to leak into the interstitial space and cause chronic inflammation of bile ducts
This can lead to cirrhosis

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2
Q

Who presents with primary biliary cholangitis?

A

Women in 40-50s

Patients present late

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3
Q

What are diseases associated with primary biliary cholangitis?

A

Sjogren’s
Rheumatoid arthritis
Hypothyroidism
Other autoimmune conditions

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4
Q

What are the signs and symptoms of primary biliary cholangitis?

A
Jaundice
Xanthoma (cholesterol in skin)
Xanthelasma (cholesterol around eyes)
Pruritis (itch)
Joint pain
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5
Q

What are the investigations for primary biliary cholangitis?

A
Anti-Mt antibodies (AMA)
High cholesterol
High IgM
LFT abnormalities
USS (rule out structural abnormality)
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6
Q

Why is there hypercholesterolaemia in primary biliary cholangitis?

A

Cholesterol leaks out of bile and deposits in skin

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7
Q

What are the complications of primary biliary cholangitis?

A

Osteoporosis (bile doesn’t function properly and calcium is not absorbed)
Portal hypertension
Ascites (from liver damage)
Fat soluble vitamin deficiencies (ADEK) (also not absorbed)

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8
Q

What is the management for primary biliary cholangitis?

A
Lifestyle (smoking, weight, alcohol, NSAIDs)
Ursodeoxycholic acid
Obeticholic acid
Vit ADEK supplements
Antipruritics (cholestryamine)
Biphosphates
Liver transplant
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9
Q

What is secondary biliary cholangitis?

A

Small bile ducts in the liver are damaged due to obstruction, no AMA detected in blood

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10
Q

What is primary sclerosing cholangitis?

A

Autoimmune condition that causes progressive inflammation and fibrosis of the bile duct

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11
Q

How does primary sclerosing cholangitis progress to cirrhosis?

A
  1. Inflammation of bile duct
  2. Fibrosis
  3. Cholestasis
  4. Liver cirrhosis
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12
Q

Who presents with primary sclerosing cholangitis?

A

Middle aged men
Associated with UC
Patients have ANCA antibodies

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13
Q

What are the symptoms of primary sclerosing cholangitis?

A

Can be asymptomatic
Pruritis (itch)
Jaundice
RUQ pain

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14
Q

How does primary sclerosing cholangitis present when it is acute?

A

Hepatitis like infection with fever, jaundice and cholangitis

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15
Q

What investigations are done for primary sclerosing cholangitis?

A
ANCA antibodies
LFTs
US
MRCP (diagnosis - beaded appearance)
ERCP (biopsy - onion skin)
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16
Q

What are the characteristic features of primary sclerosing cholangitis seen on an MRCP and ERCP?

A

MRCP - beaded appearance

ERCP - onion skin on biopsy

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17
Q

What looks similar to primary sclerosing cholangitis on MRCP and is important to rule out?

A

Cholangiocarcinoma

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18
Q

What is the management for primary sclerosing cholangitis?

A

Liver transplant definitive
Manage symptoms
Diet and nutrition
Endoscopic interventions (stenting, balloon dilation)

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19
Q

What can primary sclerosing cholangitis progress to?

A
Secondary biliary cholangitis
Gallstones
Strictures
Duct cannulation
Cholangiocarcinoma
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20
Q

How and where is bile secreted?

A

When chyme enters the duodenum, CCK stimulates the gallbladder to contract and release bile
Bile secreted via sphincter of Oddi at D2

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21
Q

What makes up the majority of bile?

A

Bile salts and acids

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22
Q

What are the main types of gallstone and which makes up the majority of gallstones, and what are they associated with?

A

Cholesterol (majority)
Pigmented (bilirubin) black stones (associated with haemolytic anaemia)
Brown stones (associated with parasitic infection)

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23
Q

What is cholelithiasis?

A

The presence of gallstones

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24
Q

What is cholecystolithiasis?

A

Gallstones in the gallbladder

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25
Q

What is choledocholithiasis?

A

Gallstones in the bile duct

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26
Q

What are the factors precipitating cholesterol gallstone formation?

A

Too much cholesterol
Not enough salt/acid/phospholipid
Gallbladder stasis

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27
Q

What are the risk factors for cholesterol stones?

A
Obesity
Rapid weight loss
High fat, low fibre diet
Family history
Female
40s
Hormone replacement therapy
(5 Fs)
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28
Q

Are most people with gallstones asymptomatic?

A

Yes

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29
Q

What is biliary colic?

A

Temporary obstruction of the CBD or cystic duct by a gallstone

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30
Q

How does biliary colic present?

A

RUQ/epigastric colicky pain that might radiate to right shoulder
Pain may last 2-6 hours and crescendos then plats
Associated with indigestion and over-indulgence with high fat foods
Onset mid-evening or early morning
Nausea and vomiting can occur

31
Q

What causes the pain in biliary colic?

A

Peristaltic contraction around the stone

32
Q

What investigation is done for biliary colic?

A

Diagnosis is clinical but can do USS

33
Q

What is the management for biliary colic?

A

Lifestyle
If mild-moderate pain - paracetamol/NSAIDs
iIf severe pain - diclofenac IM

34
Q

What is cholecystitis?

A

Obstruction of the cystic duct by gallstone causes inflammation of the gallbladder

35
Q

How does cholecystitis present?

A

RUQ/epigastric pain and tenderness
Fever
Murphey’s sign (pain on deep breath when examiner’s fingers are over RUQ)
Obstructive jaundice

36
Q

What is obstructive jaundice?

A

Jaundice of the skin, sclera
Pale stools
Dark urine

37
Q

What are the complications of cholecystitis?

A

Perforation
Fistula formation
Peritonitis
Sepsis

38
Q

What are the investigations for cholecystitis?

A

High ALP or GGT
Abdominal USS (detects stones and thickened GB)
MRCP
ERCP (diagnosis and can treat)

39
Q

What is the management for cholecystitis?

A

Supportive measures (fluids, analgesia, IV antibiotics while waiting for stone to dislodge itself)
ERCP to remove stones
Cholecystectomy
Percutaneous cholecystotomy (drain pus)

40
Q

What is acalculous cholecystitis?

A

Inflammation of the gallbladder in the absence of a gallstone
Occurs due to gallbladder stasis and bile stagnation

41
Q

Who tends to get acalculous cholecystitis?

A

Very ill patients e.g. sepsis, ICU

42
Q

What are the signs of acalculus cholecystitis?

A

Obstructive jaundice
Fever
Would be the same as calculous cholecystitis but patients are likely to be sedated or intubated to history wont obtained and signs wont be obvious

43
Q

What are the investigations for acalculous cholecystitis?

A

Raised ALP
High CRP
USS - thickened gallbladder with absence of stones

44
Q

What is the management for acalculous cholecystitis?

A

Cholecystectomy

Percutaneous cholecystotomy

45
Q

What is ascending cholangitis?

A

Bacterial infection superimposed on an obstruction of the biliary tree

46
Q

What is the pathogenesis of ascending cholangitis?

A

Obstruction of the bile ducts causes bile stagnation.
This means bacteria are not being washed out so are able to grow and cause infection
Obstruction also increases pressure in the bile ducts, widening the spaces between cholangiocytes
This causes bile to leak into the blood stream, can cause sepsis

47
Q

What can cause ascending cholangitis?

A

Gallstones (most common)
Stricture
Tumour

48
Q

What is the complication of ascending cholangitis?

A

Sepsis

49
Q

What are the signs of ascending cholangitis?

A

Charcot’s triad (fever, jaundice, RUQ pain)

Reynold’s pentad (fever, jaundice, RUQ pain, hypotension, confusion)

50
Q

What are the investigations for ascending cholangitis?

A
Bloods (raised WCC, CRP)
LFTs (raised ALP, bilirubin, GGT)
USS
Blood culture
MRCP
ERCP
51
Q

What is the gold standard investigation for ascending cholangitis?

A

ERCP

52
Q

What is the management for ascending cholangitis?

A

IV fluids
Antibiotics
Remove obstruction via ERCP
Cholecystectomy

53
Q

What is gallstone ileus?

A

Obstruction caused by a gallstone stuck permanently in the lumen of the small bowel

54
Q

What is the pathogenesis of gallstone ileus?

A

The gallstone causes repeated bouts of inflammation which can cause thinning of the gallbladder wall
Erosion causes the gallbladder to stick to the duodenum and a fistula forms
This allows gallstones to pass straight to the duodenum
If the gallstone is big it will get stuck in the small bowel, usually the terminal ileus which is narrowest, causing obstruction

55
Q

What are the signs of gallstone ileus?

A

Abdominal distension
Nausea and vomiting
Recurrent RUQ pain
Dehydration

56
Q

What are the investigations for gallstone ileus?

A

Rigler’s triad is diagnostic
Pneumobilia
Evidence of small bowel obstruction
Evidence of gallstone outside gallbladder

57
Q

What is the management for gallstone ileus?

A

IV fluid resuscitation
NG tube
Gallstone removal

58
Q

What are the causes of biliary strictures?

A

Post-op
Pancreatitis
Primary sclerosing cholangitis
Cholangiocarcinoma

59
Q

What are the complications of biliary strictures?

A

Biliary colic
Cholangitis
Obstructive jaundice
Can just be asymptomatic

60
Q

What are the investigations for biliary strictures?

A

Related to the complication caused

Rule out malignancy

61
Q

What is the management for biliary strictures?

A

Treat complication

Stenting

62
Q

What is congenital biliary atresia and what does it lead to?

A

Congenital condition characterised by an absence of deficiency of extra-hepatic biliary tree
Leads to cholestasis and liver cirrhosis from the back pressure of bile

63
Q

What are the symptoms of congenital biliary atresia?

A

Jaundice
Dark urine, pale stools
Growth and development delay

64
Q

When does congenital biliary atresia present?

A

Within 3 months of birth

65
Q

What are the investigations for biliary atresia?

A

LFTs
USS
HIDA scan

66
Q

What is the management for biliary atresia?

A

Excision of fibrous tissue

Liver transplant

67
Q

What is cholangiocarcinoma?

A

Cancer of the bile duct

68
Q

What is the most common form of cholangiocarcinoma?

A

Hilar cholagniocarcinoma - tumour forms at the point where the left and right bile duct of the liver join together

69
Q

Who tends to get cholangiocarcinoma?

A

> 65
South East Asia
Previous biliary disease

70
Q

What are the symptoms of cholangiocarcinoma?

A
Can be asymptomatic
Jaundice (with dark urine, pale stools)
Abdominal pain
Weight loss, fatigue, anorexia
Fever, RUQ pain, other symptoms of  cholangitis
71
Q

What investigations are done for cholangiocarcinoma?

A

LFTs
Contrast MRI is diagnostic
CT
ERCP (sometimes for biopsy)

72
Q

What is the management for cholangiocarcinoma?

A

Surgical resection
Stenting
Adjuvant chemotherapy and radiotherapy

73
Q

What is carcinoma of the Ampulla of Vater?

A

Rare cancer that forms in the junction of the common bile duct and the main pancreatic duct
Prognosis is bad as there are many structures it can invade into and management is hard