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
What is choledocholithiasis?
Gallstones in the bile duct
26
What are the factors precipitating cholesterol gallstone formation?
Too much cholesterol Not enough salt/acid/phospholipid Gallbladder stasis
27
What are the risk factors for cholesterol stones?
``` Obesity Rapid weight loss High fat, low fibre diet Family history Female 40s Hormone replacement therapy (5 Fs) ```
28
Are most people with gallstones asymptomatic?
Yes
29
What is biliary colic?
Temporary obstruction of the CBD or cystic duct by a gallstone
30
How does biliary colic present?
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
What causes the pain in biliary colic?
Peristaltic contraction around the stone
32
What investigation is done for biliary colic?
Diagnosis is clinical but can do USS
33
What is the management for biliary colic?
Lifestyle If mild-moderate pain - paracetamol/NSAIDs iIf severe pain - diclofenac IM
34
What is cholecystitis?
Obstruction of the cystic duct by gallstone causes inflammation of the gallbladder
35
How does cholecystitis present?
RUQ/epigastric pain and tenderness Fever Murphey's sign (pain on deep breath when examiner's fingers are over RUQ) Obstructive jaundice
36
What is obstructive jaundice?
Jaundice of the skin, sclera Pale stools Dark urine
37
What are the complications of cholecystitis?
Perforation Fistula formation Peritonitis Sepsis
38
What are the investigations for cholecystitis?
High ALP or GGT Abdominal USS (detects stones and thickened GB) MRCP ERCP (diagnosis and can treat)
39
What is the management for cholecystitis?
Supportive measures (fluids, analgesia, IV antibiotics while waiting for stone to dislodge itself) ERCP to remove stones Cholecystectomy Percutaneous cholecystotomy (drain pus)
40
What is acalculous cholecystitis?
Inflammation of the gallbladder in the absence of a gallstone Occurs due to gallbladder stasis and bile stagnation
41
Who tends to get acalculous cholecystitis?
Very ill patients e.g. sepsis, ICU
42
What are the signs of acalculus cholecystitis?
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
What are the investigations for acalculous cholecystitis?
Raised ALP High CRP USS - thickened gallbladder with absence of stones
44
What is the management for acalculous cholecystitis?
Cholecystectomy | Percutaneous cholecystotomy
45
What is ascending cholangitis?
Bacterial infection superimposed on an obstruction of the biliary tree
46
What is the pathogenesis of ascending cholangitis?
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
What can cause ascending cholangitis?
Gallstones (most common) Stricture Tumour
48
What is the complication of ascending cholangitis?
Sepsis
49
What are the signs of ascending cholangitis?
Charcot's triad (fever, jaundice, RUQ pain) | Reynold's pentad (fever, jaundice, RUQ pain, hypotension, confusion)
50
What are the investigations for ascending cholangitis?
``` Bloods (raised WCC, CRP) LFTs (raised ALP, bilirubin, GGT) USS Blood culture MRCP ERCP ```
51
What is the gold standard investigation for ascending cholangitis?
ERCP
52
What is the management for ascending cholangitis?
IV fluids Antibiotics Remove obstruction via ERCP Cholecystectomy
53
What is gallstone ileus?
Obstruction caused by a gallstone stuck permanently in the lumen of the small bowel
54
What is the pathogenesis of gallstone ileus?
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
What are the signs of gallstone ileus?
Abdominal distension Nausea and vomiting Recurrent RUQ pain Dehydration
56
What are the investigations for gallstone ileus?
Rigler's triad is diagnostic Pneumobilia Evidence of small bowel obstruction Evidence of gallstone outside gallbladder
57
What is the management for gallstone ileus?
IV fluid resuscitation NG tube Gallstone removal
58
What are the causes of biliary strictures?
Post-op Pancreatitis Primary sclerosing cholangitis Cholangiocarcinoma
59
What are the complications of biliary strictures?
Biliary colic Cholangitis Obstructive jaundice Can just be asymptomatic
60
What are the investigations for biliary strictures?
Related to the complication caused | Rule out malignancy
61
What is the management for biliary strictures?
Treat complication | Stenting
62
What is congenital biliary atresia and what does it lead to?
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
What are the symptoms of congenital biliary atresia?
Jaundice Dark urine, pale stools Growth and development delay
64
When does congenital biliary atresia present?
Within 3 months of birth
65
What are the investigations for biliary atresia?
LFTs USS HIDA scan
66
What is the management for biliary atresia?
Excision of fibrous tissue | Liver transplant
67
What is cholangiocarcinoma?
Cancer of the bile duct
68
What is the most common form of cholangiocarcinoma?
Hilar cholagniocarcinoma - tumour forms at the point where the left and right bile duct of the liver join together
69
Who tends to get cholangiocarcinoma?
>65 South East Asia Previous biliary disease
70
What are the symptoms of cholangiocarcinoma?
``` Can be asymptomatic Jaundice (with dark urine, pale stools) Abdominal pain Weight loss, fatigue, anorexia Fever, RUQ pain, other symptoms of cholangitis ```
71
What investigations are done for cholangiocarcinoma?
LFTs Contrast MRI is diagnostic CT ERCP (sometimes for biopsy)
72
What is the management for cholangiocarcinoma?
Surgical resection Stenting Adjuvant chemotherapy and radiotherapy
73
What is carcinoma of the Ampulla of Vater?
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