Case Nine - Case One Flashcards
1
Q
what is Cholangitis
A
inflammation of the bile duct
2
Q
what is cholangitis typically the result of
A
a bacterial infection (often secondary to gallstones), but can also occur in other conditions, such as primary sclerosising cholangitis and Caroli’s syndrome
3
Q
in cases where bacterial infection is the cause, what is it called
A
ascending cholangitis
4
Q
what is acute ascending cholangitis presentation
A
rigors, fever, abdominal pain and jaundice
it has a high mortality and morbidity, especially in old people
5
Q
what is the treatment for acute ascending cholangitis
A
- IV antibiotics
- urgent biliary drainage
- stenting
- surgical drainage
6
Q
what are the IV antibiotics used in acute ascending cholangitis
A
cephalosporin - cefotaxime
7
Q
how is urgent biliary drainage carried out
A
endoscopically - usually access to the biliary tree is gained by sphincterotomy and then stones removed with a balloon catheter.
successful in 90% of patients
8
Q
what is primary sclerosing cholangitis
A
a condition where the intrahepatic and extra hepatic bile ducts become inflamed and damaged, developing strictures that obstruct the flow of bile out of the liver, and into the intestines
9
Q
what does sclerosis refer to
A
the stiffening and hardening of the bile ducts
10
Q
what does cholangitis refer to
A
the inflammation of the bile ducts
11
Q
what does chronic bile obstruction eventually lead to
A
liver inflammation (hepatitis), fibrosis and cirrhosis
12
Q
what is thought to be the cause of primary sclerosing cholangitis
A
combination of genetic and environmental factors. there is a strong association with ulcerative colitis, with around 70% of the cases occurring alongside pre-existing ulcerative colitis
13
Q
what are the key risk factors for primary sclerosing cholangitis
A
male
aged 30-40
ulcerative colitis
family history
14
Q
what is the presentation of primary sclerosing cholangitis
A
often patients are asymptomatic at diagnosis, with the problem picked up on abnormal liver function tests, however they may present with:
- abdominal pain in RUQ
- pruitis
- jaundice
- hepatomegaly
- splenomegaly
15
Q
what are the investigations carried out for primary sclerosing cholangitis
A
liver function tests, that will show:
- raised alkaline phosphatase
- other liver enzymes and bilirubin are raised later in the disease
16
Q
what is the most notable liver enzyme as with the most ‘obstructive’ pathology
A
alkaline phosphatase
17
Q
what are the main antibodies, however they are not helpful in diagnosis or assessment
A
Perinuclear antineutrophil cytoplasmic antibody (p-ANCA)
Antinuclear antibodies (ANA)
Anti-smooth muscle antibodies (anti-SMA)
18
Q
what is the diagnostic imaging investigation
A
MRCP
magnetic resonance cholangiopancreatography
19
Q
what does MRCP involve
A
an MRI scan that gives a detailed view of the bile ducts, showing bile duct strictures in primary sclerosing cholangitis
20
Q
what should be performed to assess for ulcerative colitis
A
colonoscopy
21
Q
what is the management for primary sclerosing cholangitis
A
there are no treatments proven to be effective for primary sclerosing cholangitis
22
Q
however, what may be used to treat dominant strictures
A
ERCP - endoscopic retrograde cholangio-pancreatography
23
Q
what does ERCP involve
A
inserting an endoscope down the oesophagus, past the stomach, to the duodenum and the opening of the common bile duct.
this gives the operator access to the biliary system
strictures can be dilated. stents can be iterated to keep the ducts open. a
24
Q
what are given alongside ERCP to reduce the risk of infection (bacterial cholangitis)
A
antibiotics are given to reduce the risk of infection
25
what is used in advanced disease, and what is the survival rate
liver transplant is used in advanced disease, with around d 80% survival in five years post transplantation
26
what are the other aspects of management
colestyramine for symptoms of pruritus (a bile acid sequestrant that reduces intestinal absorption of bile acids)
replacement of fat soluble vitamins
monitoring for complications such as cholangiocarcinoma, cirrhosis and oesophageal varies
27
what are the complications of primary sclerosing Cholangitis
Biliary strictures
Acute bacterial cholangitis
Cholangiocarcinoma develops in 10-20% of cases
Cirrhosis and the related complications (e.g., portal hypertension and oesophageal varices)
Fat-soluble vitamin deficiency (A, D, E and K)
Osteoporosis
wColorectal cancer in patients with ulcerative colitis
28
what line is commonly tested in exams
the association between ulcerative colitis, primary sclerosing cholangitis and cholangiocarcinoma
29
what is IgG4 related sclerosing cholangitis
is similar to primary sclerosing cholangitis. elevated IgG4 levels in the blood are the distinguishing feature. unlikely primary sclerosing cholangitis, IgG4- related sclerosing cholangitis responds well to treatment with steroids
30
what is IgG4 related sclerosing cholangitis associated with
autoimmune pancreatitis
31
what is primary biliary cholangitis
an autoimmune condition where the immune system attacks the small bile ducts in the liver, resulting in obstructive jaundice and liver disease
previously known as primary biliary cirrhosis
32
what does primary biliary cholangitis affect
the small bile ducts inside the liver (intrahepatic ducts)
33
where does the inflammation affect and what does this lead to over time
there is inflammation and damage to the epithelial cells of the bile ducts (the cholangiocytes)
overtime, this can lead to obstruction of bile flow through these ducts.
34
what is reduced flow of bile called
cholestasis
35
what do the back-pressure of bile and the overall disease process lead to
liver fibrosis, cirrhosis and failure
36
what are excreted through the bile ducts into the intestines
bile acids, bilirubin and cholesterol
37
what do raised bile acids and bilirubin cause
raised bile acids cause itching and raised bilirubin causes jaundice
38
what does raised cholesterol cause and where is this seen
causes cholesterol deposits in the skin called xanthelasma. Xanthomas are larger nodular deposits of cholesterol in the skin or tendons. Raised cholesterol increases the risk of atherosclerosis and cardiovascular disease.
39
what is responsible for the darker colour of stools
bilirubin, a lack of this results in pale stools
excretion of bilirubin via the urine causes dark urine
40
what is the typical presentation of primary biliary cholangitis
white woman, aged 40-60 years
often patients are asymptomatic at diagnosis, with the problem being picked up on abnormal liver function tests
41
what are the symptoms that they may present with
fatigue
pruitus (itching)
gastrointestinal symptoms and pain
jaundice
pale, greasy stools
dark urine
42
what may be found on examination with primary biliary cholangitis
Xanthoma and xanthelasma (cholesterol deposits)
Excoriations (scratches on the skin due to itching)
Hepatomegaly
Signs of liver cirrhosis and portal hypertension in end-stage disease (e.g., splenomegaly and ascites)
43
what will liver function tests show
Raise alkaline phosphatase (the most notable liver enzyme as with most “obstructive” pathology)
Other liver enzymes and bilirubin are raised later in the disease
44
what are the autoantibodies relevant to primary biliary cholangitis
Anti-mitochondrial antibodies (AMA) are the most specific to PBC and form part of the diagnostic criteria
Anti-nuclear antibodies are present in about 35% of patients
45
what is a non-specific blood result finding
Raised immunoglobulin M (IgM) is a non-specific blood result finding.
46
what are the two results for primary biliary cholangitis to remember
The two results for primary biliary cholangitis to remember are anti-mitochondrial antibodies and alkaline phosphatase. In your exams, a middle-aged white woman presenting with itching, a positive AMA and a raised alkaline phosphatase almost certainly has primary biliary cholangitis.
47
what is the most essential treatment to remember in primary biliary cholangitis
ursodeoxycholic acid
it is a non-toxic, hydrophilic bile acid that protects the cholangiocytes form inflammation and damage. it makes the bile less harmful to the epithelial cells of the bile ducts
48
how does Ursodeoxycholic acid work
slows disease progression and improves outcomes
49
what is given if UDCA is inadequate or not tolerated
obeticholic acid
50
what is given for the symptoms of pruitus
colestyramine
a bile acid sequestrant that reduces intestinal absorption of bile acids
51
what is the most crucial result of disease progression
liver cirrhosis with the associated complications e,g portal hypertension and hepatocellular carcinoma
52
what do the other complications of primary biliary cholangitis include
Fat-soluble vitamin deficiency (A, D, E and K)
Osteoporosis
Hyperlipidaemia (raised cholesterol)
Sjögren’s syndrome (dry eyes, dry mouth and vaginal dryness)
Connective tissue diseases (e.g., systemic sclerosis)
Thyroid disease
53
what does gallstones refer to
cholelithiasis - scientific name for gallstones
refer to the formation of hard stones in the gallbladder - a process which typically takes Years to occur
54
how do we separate gallstones and biliary disease
it can be confusing, with overlap
however, essentially, gallstones themselves are not problematic in the vast majority of cases, but can predispose the other problems with the biliary tree
55
what percentage of patients will have an acute prevention
what percentage of these are biliary colic and what percentage of these are acute cholecystitis
1-4%
about 60% of these are biliary colic and 40% of these are acute cholecystitis
56
what does bile contain
bile pigments (from Hb breakdown products). cholesterol and other lipids
57
how are stones generally classified
as cholesterol stones or pigment stones; however, in reality they are usually mixed.
58
in Europe and the USA, what percentage of gallstones are cholesterol stones
75% of gallstones are cholesterol stones
59
what are the features of pure cholesterol stones
usually solitary and large
the red flags are female, age and obesity
stones of about 70% or more of cholesterol are usually smaller and more numerous. the rest of the stone is made up of calcium compounds and protein
60
what are the two categories of pigment stones
brown and black
brown stones are softer, and contain a mixture of pigment, cholesterol and calcium salts.
black stones are much harder, and made of pure pigment
61
what is a massive factor in gallstones formation and how does this relate to women
cholesterol secretion, particularly the amount of cholesterol secreted in relation to the concentration of bile salts
women naturally secrete a nigher proportion of cholesterol than men, and thus they have a higher incidence of gallstones
62
where are gallstones the most common
Racial differences: More common in Scandinavia, and Native North and South American Populations.
63
what are the red flags for gall stones
Weight
Obesity
Sudden weight loss
Family History
Oestrogen
Female gender
Oral contraceptive pill
Diet (high fat, low fibre)
Increasing age
Stones take time to form
Diabetes
‘Fair, fat, female, forty’ used to be a term used to describe the typical patient with gallstones – but as noted above, that is only a selection of the risk factors.
64
what are the three factors needed for the formation of cholesterol stones
high concentration of cholesterol in the gallbladder
gallbladder stasis
products that promote the crystallisation of cholesterol - some lipoproteins found in bile do this
65
when do cholesterol stones form
when the concentration of micelles is not great enough to hold all the cholesterol in the micelles
66
when is formation of stones increased
during fasting - particularly extended fasting as this increases the concentration of cholesterol in the gallbladder relative to other solutes
67
what do patients with cholesterol tones generally have
a smaller bile pool which circulates more often
68
how do cholesterol stones form
initially, cholesterol crystals will form in bile that is supersaturated with cholesterol. this results in the production of sludge.
69
what is the formation of cholesterol stones inhibited by
caffeine, NSAIDs and bile salt
70
what is the formation of cholesterol salts exacerbated by
mucin, rapid weight loss, pregnancy, increased serum cholesterol
71
what does this sludge do
goes on to form stones or it can be reabsorbed. only in about 15% of cases will it go on to form stones
72
what are pigments in bile from
bilirubin breakdown
73
what are the three main causes that can lead to pigment stones
An increase in bilirubin load, as a result of haemolytic anaemia.
40-60% of patients with haemolytic disease have pigment stones, but the vast majority of pigment stones patients do not have haemolytic disease.
Pigments become less water soluble once in the bile as a result of the action of glucuronidases. It is thought that most cases of pigment stones result from the subclinical bacterial colonisation of the gallbladder. This is particularly common in East Asia, and associated with E. coli. These stones tend to be softer and brown, and combined with calcium carbonate. The other two types of stone tend to be smaller, blacker and harder, and more commonly encountered in the west.
Cirrhosis – with this there will be depletion of glucuronidase inhibitors in the bile.
74
what is the presentation of 90% of pigment gallstones
asymptomatic and discovered incidentally
75
what do gallstones not usually cause
Gallstones usually do NOT cause flatulence, dyspepsia, fat intolerance or other vague upper abdominal symptoms, unless they are causing an acute flare-up of biliary colic or other acute manifestations
76
how can symptomatic gallstones present
either as biliary colic, cholecytsitis, pancreatitis
77
what is biliary colic described as
intermittent RUQ pain/epigastric pain associated with a blockage in the bile duct. often it is caused by a stone migrating down the duct
78
what does the word colic mean
wave of pain.
79
describe how colic works
it is associated with a tubular structure surrounded by smooth muscle
the wave nature of pain is related to the fact that smooth muscle has its own pacemaker that causes contraction at certain intervals
80
what are the features of pain in biliary colic
usually sporadic and is associated with eating - particularly fatty foods
pain will subside with analgesia, and is usually reduced with modification of diet
81
what're the clinical features of pain in biliary colic
pain after eating a large fatty meal. typically the pain will appear in the mid evening and last until the early hours of the morning
82
where may this pain radiate
to right shoulder tip
83
what are the other clinical features seen
Nausea and vomiting (in severe attacks)
More severe prolonged pain suggests association with an underlying condition such as cholecystitis, cholangitis or gallstone induced pancreatitis.
LFT’s often normal.
Typically, a recurring condition: over a 5-year period 100% of patients will experience it again. 20% of patients go on to develop something more serious; cholecystitis, obstructive jaundice or pancreatitis.
84
what are the differential diagnosis of biliary colic
GORD
Peptic ulcer
IBS
Pancreatitis
Tumour
85
stones in the gallbladder or cystic duct that cause biliary colic are unlikely to produce what
unlikely to produce abnormal test results, however stones lying in the common bile duct are most likely to account for symptoms
86
what are the LFT's that may be very slightly raised in the presence of cholecystitis, even without current duct obstruction
↑bilirubin, ↑ALP and ↑ALT
87
what is an OBSTRUCTIVE pattern of LFT's
ALP and bilirubin raised higher than ALT is an obstructive pattern and tends to signify biliary duct problems
88
what does ALT being raised higher than ALP and bilirubin usually indicate
an intra-hepatic pathology
89
what may a plain AXR show
gallstones (only show up in 10-15% of cases, as they need to be calcified to be seen on radiograph)
90
what is the gold standard test for gallstones
ultrasound as it is 95% effective at detecting but is operator dependent
91
how can you tell the different between gallstones and polyps on an USS
On a USS, you can sometimes mistake gallstones for polyps and vice versa. You can tell the difference because gallstones will cast a ‘shadow’ underneath them on the USS screen, but polyps will not.
92
how may cholecystitis be detectable
by a thickening of the gallbladder wall to more than 4mm
there may also be a halo effect around the gallbladder which shows oedema surrounding the bladder. this may also indicate hypoalbuminaemia, portal hypertension or acute viral hepatitis
93
what is oral cholecystography
the patient takes a dose of an oral dye that is absorbed and concentrated by the liver and then secreted in the bile. This should enable you to see the gallbladder on radiograph. If you can’t see the gallbladder, then there could be a blockage in the cystic duct, or there may have been recent cholecystitis. Even when this test works correctly, some stones are too small to see, and so we get a false negative 5% of the time. Used in conjunction with an USS, the false negative rate is reduced to 2%.
94
what is Courvoisier's sign
palpable gallbladder
a palpable gallbladder in the presence of painless jaundice is unlikely to be gallstones
95
what to gallstones usually result in (gallbladder)
typically result in a fibrotic shrunken gallbladder, which is not usually distended and thus not palpable
96
what are the complications
Acute and chronic cholecystitis
Acute cholangitis
Pancreatitis
Increased risk of adenocarcinoma of the wall of the gallbladder (only very small)
Fistulation – Gallstones may perforate the gallbladder and form a fistula, often to the colon or small intestine. A gallstone passing into the small intestine may cause a blockage in the ileum – gallstone ileus.
Stones can pass out of the cystic duct and into the bile duct, causing obstructive jaundice. This is called choledocholithiasis and it is commonly accompanied by cholangitis (bacterial infection of the gallbladder)
97
what is the treatment for asymptomatic stones
they are not normally treated
98
what is the management of biliary colic
morphine IV - 5-10mg/4hr
antiemetic e.g domperidone, cyclizine
99
what surgery can patients with severe colic elect to get
elective cholecystectomy
this is nearly always accompanied by cholangiography
performed under general anaesthetic after four hours of fasting
100
what is an open elective cholecystectomy
Open – there is a 10% chance that the patient will have a gallstone in the bile duct at the time of cholecystectomy and as a result, often during the operation, a catheter is put in the bile duct, and dye squirted up to outline the biliary tree. If there is a stone present here, then it should be removed. After this is done, the gallbladder is removed. Some surgeons also like to leave a drain in place of the gallbladder for a few days, as some people have small ducts that take bile directly to the gallbladder, and these may leak. However, the drain is a cause of post-operative pain, and the evidence to suggest it improves the outcome from bile leak is limited.
101
what is a laparscopic elective cholecystectomy
Laparoscopic – the camera is inserted in the umbilicus and there are three other incisions made for instruments. This is now a more common procedure than the open version, and the techniques used are virtually the same. Once the gallbladder has been removed from its bed, it is particularly important to examine it for leaks (to check for the little ducts described above). Usually the gallbladder is removed via the umbilicus. The peritoneal cavity will then be washed out, and cleaned up.
102
what is post cholecystectomy syndrome
this is a bilary type of pain that occurs months or years after cholecystectomy
patients say this pain feels identical to the pain for which the operation was carried out
103
what does post cholecystectomy pain basically mean
that the procedure was not needed, because the gallbladder were not originally causing the pain. The pain was actually caused by functional colonic disease, where there is spasm at the hepatic flexure
104
what is cholestasis
biliary obstruction
105
what are the intrinsic causes of bile duct obstruction
Common bile duct gallstones
Cholangitis
Carcinoma of the bile duct
Carcinoma of the gallbladder
Benign post-traumatic stricture
Sclerosing cholangitis (primary and secondary)
Haemobilia
106
what are the extrinsic causes of bile duct obstruction
Carcinoma of the pancreas
Carcinoma of the ampulla of Vater
Metastatic carcinoma
Lymphoma
Pancreatitis (acute and chronic)
Pancreatic cysts
Congenital causes
Biliary atresia
Choledochal cyst
Congenital intrahepatic biliary dilatation (Caroli’s disease)
107
what would show on blood test for biliary colic
normal inflammatory markers and LFT's and there is no acute presentation
108
what is a distended gallbladder a normal finding in
someone who has been fasting
109
what is the medical name of gallstones
cholelithiasis
110
what are the risk factors for developing gallstones
female
increasing age
high BMI
diabetes
prolonged fasting or rapid weight loss
hormone replacement therapy
diet high in triglycerides and refined carbohydrates
111
what is the gallbladder stimulated by
cholecystokinin released from I cells that line the duodenum in response to fatty acids and amino acids in the stomach and duodenum
112
why will all the tests be normal (do not learn, just be able to explain)
The gallbladder is stimulated by cholecystokinin released from I-cells that line the duodenum in response to fatty acids and amino acids in the stomach and duodenum. In biliary colic, there is no pain when the gallbladder is not stimulated (hence, its intermittent nature and relationship to food). There is no infection present and therefore no clinical signs of Systemic Inflammatory Response Syndrome (SIRS) or sepsis. Blood inflammatory markers (WCC and CRP) will be normal. Because the gallstones remain in the gallbladder and do not obstruct the Common Bile Duct (CBD), the Liver Function Tests (LFT) are normal and Ultrasound (US) imaging will show a non-obstructing / non-dilated CBD). The US image below demonstrates a gallstone in the gallbladder (white arrow).
113
what is the conservative management of biliary colic
consists of a fat free diet (stops the stimulation of the gallbladder) and simple analgesia for any biliary colic episodes for some patients, this is enough to control their symptoms to avoid surgery
114
what are the more serious conditions that gallbladder stones can cause
acute cholecystitis - 1-3% of patients with symptomatic gallstones will develop an acute infection of the gallbladder
acute cholangitis - about 50% of patients with acute cholangitis have a gallstone aetiology
acute pancreatitis - up to 70% of acute pancreatitis cases are due to gallstone disease
115
