2 Biliary Disease Flashcards
1
Q
What is Hartmann’s pouch?
A
Infundibulum of gallbladder; sometimes described as abnormal pouching at the gallbladder neck
2
Q
Bile is secreted by the ______ and concentrated in the _______
A
Liver
Gallbladder
3
Q
What is bile made of?
A
Water Bile salts Lecithin/traces of phospholipids Bilirubin Cholesterol
4
Q
What is the function of bile?
A
Digestion and absorption of fats
Vehicle for excretion of bilirubin, excess cholesterol, and metabolic byproducts
5
Q
Inflammation of the gallbladder
A
Cholecystitis
6
Q
Stones in the gallbladder
A
Cholelithiasis
7
Q
Inflammation of the bile ducts
A
Cholangitis
8
Q
Stones in the common bile duct
A
Choledocholithiasis
9
Q
Disruption of bile flow
A
Cholestasis
10
Q
In the US, gallstones are seen in approx ___ of men and ____ of women
A
6%
9%
11
Q
Risk factors for cholelithiasis
A
The Four F’s (female, forty, fluffy, fertile): • Females • Age ≥40 • Obesity • Pregnancy • Estrogen (OCPs, HRT)
Other RFs: • Rapid weight loss (esp low cal diets or post bariatric surgery) • Family Hx or genetics • Ethnicity (Native Americans • Diabetes
12
Q
80% of cholelithiasis involves stones made of …
A
Cholesterol
13
Q
20% of cholelithiasis in the US involves ______ stones
A
Pigment stones
Either black or brown pigment stones
14
Q
Clinical manifestation of cholelithiasis for the majority of patients
A
Asymptomatic, incidental gallstones
15
Q
What differentiates uncomplicated gallstone disease from cholelithiasis?
A
SYMPTOMATIC biliary colic in the absence of gallstone-related complications
(Compared to ASYMPTOMATIC cholelithiasis - the stones are there but don’t hurt)
16
Q
What is considered complicated gallstone disease?
A
Gallstone related complications:
• Acute cholecystitis
• Choledocholithiasis with or w/o acute cholangitis
• Gallstone pancreatitis
17
Q
What is the initial diagnostic test of choice for cholelithiasis?
A
Ultrasound
95% sensitivity for detection of gallstones
Inexpensive and non-invasive
18
Q
Abdominal plain films are only positive in ______ of patients with cholesterol stones
A
~10-15%
B/c it takes a lot of cholesterol content to make them radiopaque
19
Q
Why aren’t CT’s preferred imaging for cholelithiasis?
A
Less sensitive than US (only 55-80%)
Increased expense
Radiation exposure
Used more if trying to eval for other conditions
20
Q
What do you do for someone with incidental gallstones?
A
Majority of patients do not require treatment
Manage expectantly - reassurance, pt ed for warning signs
Refer for cholecystectomy if Sx develop, unless increased risk of gallbladder cancer or hemolytic disorders (ie sickle cell)
21
Q
Biliary colic in the absence of gallstone-related complications
A
Uncomplicated gallstone disease
22
Q
How does biliary colic occur?
A
NO inflammation of the gallbladder
Gallbladder contracts, forcing stone against the outlet or cystic duct opening
—> increased intra-gallbladder pressure
—> Increase in pressure results in pain
—> as GB relaxes, obstruction is relieved and pain slowly subsides
23
Q
What will your patient report to you that will make you suspect biliary colic?
A
Constant, intense RUQ pain radiating to R shoulder blade
Pain lasts at least 30 min, plateaus within 1 hour, usually lasts <5-6 hours
Postprandial, esp if fatty/greasy meal
Associated N/V and diaphoresis
Not exacerbated by movement
Not relieved by squatting, BM, or flatus
Nocturnal pain common
24
Q
Physical exam findings for biliary colic
A
Normal vitals (no fever, tachycardia)
Not ill appearing, no evidence of jaundice, anicteric
Abdominal exam:
• Often benign, possible RUQ TTP
• No peritoneal signs
• (-) Murphy’s sign (b/c no inflammation)
All labs normal
25
What will US show for biliary colic?
Gallstones and/or gallbladder sludge
26
DDx for biliary colic
PUD
Acute cholecystitis
Choledocholithiasis
Functional gallbladder disorder
Sphincter of Oddi dysfunction
27
How to manage uncomplicated gallstone disease
Patient with biliary colic but normal PE, normal labs who has US (+) for gallstones or sludge —> cholecystectomy recommended
Why? Prevent recurrent sx and complications
28
Biliary-type pain in the absence of gallstones, sludge, microlithiasis, or microcrystal disease
Functional Gallbladder Disorder
29
Functional Gallbladder Disorder occurs as a result of...
Gallbladder dysmotility - something is impairing GB’s ability to eject bile
30
What do labs/imaging look like in functional GB disorder?
Normal labs (CBC, LFTs, amylase/lipase)
Normal imaging (no stones on US, normal EGD)
31
How is functional gallbladder disorder diagnosed?
Diagnosis of exclusion in a patient with typical biliary-type pain
1st exclude other causes for patient’s pain (PUD, dyspepsia, ischemic heart disease)
If no other causes identified —> Cholecystokinin (CCK)-stimulated cholescintigraphy (AKA: HIDA scan)
32
HIDA scan is the test of choice for...
Functional gallbladder disorder, after you’ve ruled out other causes of biliary type pain
33
How does a HIDA scan w/ CCK work?
CCK given to stimulate GB to contract
GB ejection fraction calculated
A GBEF <35-40% is considered low and supportive of diagnosis
34
What is the Rome IV criteria for functional gallbladder disorder?
Required:
• Biliary Pain
• Absence of gallstones or other structural pathology
Supports Dx but not required:
• Low EF on scintigraphy (HIDA scan)
• Normal liver enzymes, conjugated bilirubin, and amylase/lipase
35
How does the Rome IV criteria define biliary pain?
Pain in the epigastrium and/or RUQ
Builds up to a steady level and lasts at least 30 min
Occurs at variable intervals (not daily)
Is severe enough to interrupt ADL or lead to ER visit
Not significantly relieved by BM, postural changes, or acid suppression
Other criteria supportive of biliary pain but not required:
Pain with N/V
Pain that radiates to the back or right subscapular region
Pain that awakens patient from sleep
36
How do you manage functional gallbladder disorder?
Education and reassurance
| Referral
37
When is a cholecystectomy recommended for patients with functional gallbladder disorder?
Typical biliary-type pain and a GBEF <40%
38
Acute inflammation of the gallbladder together with cystic duct obstruction
Acute (calculous) cholecystitis
Predominantly a complication of gallstone disease
~5-10% are acalculous
39
How does acute cholecystitis present?
Usually begins as an attack of biliary pain that progressively worsens (most have had previous episodes of biliary pain)
Prolonged (>4-6 hours), steady, severe RUQ/Epigastric pain
May radiate to right shoulder or back
Fever, N/V, anorexia
Often a Hx of fatty food ingestion
40
PE findings for acute cholecystitis
Vitals: FEVER, TACHYcardia
Gen: ILL appearing, might be lying still on exam table
Skin: NO jaundice
Eyes: anicteric
Abd: RUQ TTP, +/- guarding, (+) MURPHY’S SIGN
41
What will labs show in acute cholecystitis
LEUKOCYTOSIS W/ LEFT SHIFT
May see mild elevation in serum AST/ALT
Elevated serum total bilirubin and alk phos are NOT common
• If present, should raise concern for biliary obstruction (cholangitis, choledocholithiasis)
Serum amylas usually normal (if elevated, suggests pancreatitis)
42
Most common complication of acute cholecystitis
Gangrene (up to 20% of cases)
More common in older patients, patients with DM, those that delay seeking treatment
Sepsis-like presentation, can lead to perforation, peritonitis
43
Complications of acute cholecystitis
Gangrene (20%)***
Perforation (10%)*** - usually 2˚ to gangrene
Cholecystoenteric FISTULA
Gallstone ILEUS
Emphysematous cholecystitis, empyema, hydros (from gas-producing infection)
MIRIZZI SYNDROME (1%)
44
A stone in the cystic duct or Hartmann’s pouch of the gallbladder that compresses the common hepatic duct and causes jaundice
Mirizzi Syndrome - uncommon but good to know about
45
How do you manage Acute Cholecystitis?
```
Admit to hospital
NPO
IV fluids
IV pain control (Ketorolac, Morphine, Meperidine)
IV Abx (empiric)
CHOLECYSTECTOMY*** mainstay of treatment
```
46
RUQ Pain
Fever
Leukocytosis
GB Inflammation
What you think?
Acute Cholestasis!
47
Chronic inflammation of the gallbladder, almost always associated with gallstones
Chronic cholecystitis
48
Chronic cholecystitis results from...
Repeated acute/subacute cholecystitis or prolonged mechanical irritation of the gallbladder wall by stones
49
How do patients with chronic cholecystitis present?
May only have minimal Sx
May be asymptomatic for years, may progress to symptomatic GB disease or acute cholecystitis, or may present with complications
You Just. Don’t. Know.
50
Acute necroinflammatory disease of the gallbladder in the absence of gallstones
Acalculous cholecystitis
Associated with high morbidity and mortality rates (much more sick)
51
What is the pathogenesis of acalculous cholecystitis?
GB stasis and ischemia
—> Local inflammatory response in the GB wall
—> Secondary bacterial infection is common
—> Perforation in severe cases
More common in hospitalized and critically ill patients (trauma/burn patients, postpartum after prolonged labor, ortho surgery)
52
How do patients with acalculous cholecystitis present?
May present similar to acute (calculous) cholecystitis
May have a clinical picture of sepsis-related cholestasis and jaundice
53
What diagnostic approach do you take for acalculous cholecystitis?
Clinical suspicion in critically ill patients with sepsis w/o clear source or jaundice
Work up includes imaging (US, CT, HIDA scan), liver lasts, CBC, CMP, pancreatic enzymes, UA
Prompt treatment is imperative
54
How do you treat acalculous cholecystitis?
Treat to manage risk of GB gangrene and perforation
Obtain blood cultures and initiate abx (b/c already septic)
Cholecystectomy
If too sick for surgery, possible GB drainage (up to the surgeon)
55
Gallstones in the common bile duct
Choledocholithiasis
In patients with cholelithiasis, passage of gallstones in CBD occurs in about 10-15%
56
Incidence of choledocholithiasis increases with ...
Age
57
In choledocholithiasis, the gallstones can block the flow of _____ and cause _______
Bile
Jaundice
58
How does choledocholithiasis present?
Biliary-type pain, often more prolonged than seen with typical biliary colic
Reports of JAUNDICE
59
PE findings for choledocholithiasis
Vital signs typically WNL
RUQ or epigastric TTP
May appear JAUNDICED
Courvoisier sign**** (palpable GB)
60
What is the name for a palpable GB, typically noted on PE in choledocholithiasis?
Courvoisier sign
61
Lab findings indicative of choledocholithiasis
No leukocytosis on CBC
Elevated liver tests (due to cholestasis)
• ALT and AST elevated early in course
• Cholestatic pattern later - inc bilirubin, alk phos, and gamma-glutamyl transpeptidase (GGT) exceeding elevations in ALT/AST
Normal amylase and lipase (no pancreatic involvement
62
What imaging do you want to order for your poor patient with choledocholithiasis?
US - initial imaging of choice
• Cholelithiasis with CBD stone and CBD dilation >6mm
If diagnosis uncertain, consider MRCP or endoscopic US
63
MRCP is great for showing...
Biliary tree and pancreatic ducts
Used to confirm diagnosis of common bile duct stone/obstruction
64
How do you manage choledocholithiasis?
Removal of the CBD stone
Identify and treat complications
• Acute cholangitis
• Acute pancreatitis
ERCP = therapeutic and diagnostic
Cholecystectomy to follow if appropriate
65
Inflammation of the biliary duct system caused primarily by a bacterial infection in a patient with biliary obstruction
Acute cholangitis
66
Biliary obstruction causing cholangitis can be from...
Choledocholithiasis***
Benign biliary stricture
S/P ERCP
Malignancy
67
Where do the bacteria come from in acute cholangitis?
Typically ascend from the duodenum
68
“Pus under pressure”
Acute cholangitis
It’s a surgical emergency - early intervention is imperative
69
How does acute cholangitis present?
Charcot’s Triad:
• Fever
• Abdominal Pain
• Jaundice
If Charcot’s Triad + AMS + Hypotension = Reynold’s Pentad (significant morbidity and mortality)
70
Fever + Abdominal Pain + Jaundice
Charcot’s Triad
71
Fever + Abdominal Pain + Jaundice + AMS + Hypotension
Reynolds Pentad
72
What lab findings are indicative of acute cholangitis?
LEUKOCYTOSIS W/ LEFT SHIFT
Elevated CRP (or ESR)
```
Liver tests show evidence of cholestasis
• Inc bilirubin
• Inc alk phos
• Inc GGT
• May see Inc AST/ALT
```
Serum amylase or lipase can be 3-4x normal (suggests associated pancreatitis)
(+) blood cultures in 25-40%
73
What imaging do you want for acute cholangitis?
Ultrasound
CT
MRCP (or EUS)
• CBD Dilation
• CBD Stone
74
How do you manage acute cholangitis?
Admit to hospital
Monitor for and treat sepsis (empiric abx)
Emergent consult with GI and surgery
• Establishing biliary drainage (typically with ERCP)
75
What it the treatment of choice for acute cholangitis?
Biliary Drainage
For relief of biliary obstruction imperative (“pus under pressure”)
76
How is biliary drainage performed?
ERCP w/ sphincterotomy and stone extraction (and/or stent insertion)
Follow with cholecystectomy if appropriate
77
Biliary Colic or Acute Cholecystitis?
Temporary RUQ pain
No fever
No leukocytosis
No jaundice
Biliary Colic
78
Biliary Colic or Acute Cholecystitis?
Persistent RUQ pain
Fever
Leukocytosis
No Jaundice
Acute Cholecystitis
79
Choledocholithiasis or Acute Cholangitis?
RUQ/epigastric pain
Jaundice
No fever
No leukocytosis
Choledocholithiasis
80
Choledocholithiasis or Acute Cholangitis?
RUQ/epigastric pain
Jaundice
Fever
Leukocytosis
Acute cholangitis
81
Autoimmune destruction of the intrahepatic bile ducts which causes cholestasis
Primary Biliary Cholangitis (PBC)
May result in cirrhosis and liver failure
82
Clinical presentation of primary biliary cholangitis
May be asymptomatic
```
FATIGUE, intense PRURITIS, +/- Jaundice
RUQ discomfort
Skin hyperpigmentation
Xanthelasmas
Hepatomegaly
```
83
Primary Biliary Cholangitis is associated with...
Other autoimmune conditions
| ie Sjogren syndrome and autoimmune thyroid disease
84
Incidence of PBC is higher in...
Women
86
Lab findings for Primary Biliary Cholangitis
Liver tests = cholestatic pattern
• Elevated Alk Phos, GGT, 5-NT (5’-nucleotidase)
• ALT/AST may be normal or slightly elevated
• Inc serum bilirubin concentration (later in disease)
Antimitochondrial antibodies (AMA) in 95%****
ANA (70% - non specific)
Hyperlipidemia (LDL and HDL may be markedly elevated)
87
Serologic hallmark of PBC
Antimitochondrial antibodies (AMA)
Seen in 95% of PBC cases
88
Complications of Primary Biliary Cholangitis
```
Cirrhosis
Hepatocellular carcinoma
Malabsorption (and steatorrhea)
Fat-soluble vitamin deficiences
Metabolic bone disease, incl osteopenia and osteoporosis
```
89
How do you diagnose Primary Biliary Cholangitis?
No extrahepatic biliary obstruction
No comorbidities affecting the liver
At least 2 of the following present:
• Elevated Alk Phos (1.5x ULN)
• (+) AMA
• Histologic evidence of PBC (liver bx if needed)
90
What should you do for patients you suspect have PBC?
Liver bx is helpful for prognosing and staging
Refer to GI for:
Medication management
Treatment of complications
91
Sclerosing, inflammatory, and obliterative process involving the intrahepatic and/or extrahepatic biliary tree
Primary Sclerosing Cholangitis (PSC)
Chronic/progressive disease of unknown etiology
92
Incidence of PSC is higher in ...
Men (70%)
Mean age at diagnosis is ~40 years
93
PSC is closely associated with ...
IBD (UC > CD)
94
Primary Sclerosing Cholangitis ultimately leads to complications of...
Cholestasis
End-stage liver disease
95
SSx of Primary Sclerosing Cholangitis
Asymptomatic
FATIGUE
PRURITIS
Jaundice
Hepatomegaly
96
Lab tests indicative of PSC
Liver tests demonstrate cholestatic pattern
Increased IgM levels, (+) P-ANCA, Autoantibodies (ANA, ASMA)
(-) AMA
97
How do you diagnose PSC?
Cholestatic pattern on liver test, esp with underlying IBD
Cholangiography
• MRCP or ERCP (or Percutaneous transhepatic cholangiography)
• Multifocal structuring and dilation of intrahepatic and/or extrahepatic bile ducts
Liver bx
• May support diagnosis but is rarely diagnostic
98
Complications of PSC
```
End-stage liver disease
Fat-soluble vitamin deficiencies
Metabolic bone disease
Cholangitis and cholelithiasis
Hepatobiliary cancer
Colon cancer (in pt with UC)
```
99
Inherited deficiency in enzyme for glucuronidation of bilirubin in the liver (conjugation)
Gilbert Syndrome
100
Unconjugated hyperbilirubinemia in the absence of hemolysis
Gilbert Syndrome
Normal CBC, blood smear, and reticulocyte count
Remainder of liver tests normal
101
Clinical presentation of Gilbert Syndrome
Typically presents during post-pubertal adolescence
More common in males
Mild INTERMITTENT episodes of jaundice
Otherwise, most are asymptomatic
No specific treatment required
102
What can trigger jaundice in patients with Gilbert Syndrome?
Dehydration
Fasting
(Menstruation)
103
Most common cancer arising in the biliary tract
Gallbladder Cancer
Uncommon but highly fatal malignancy
104
How is Gallbladder Cancer usually diagnosed?
Often found incidentally during workup for cholelithiasis
105
Gender distribution for Gallbladder Cancer
W>M
Also, increased incidence with age
106
Risk factors for gallbladder cancer
```
Gallstone Disease***
Porcelain Gallbladder
Gallbladder polyps
PSC
Chronic infection (salmonella, Helicobacter)
Obesity
```
107
What is porcelain gallbladder?
Calcified GB due to chronic inflammation
108
How does GB cancer present?
May be asymptomatic
May mimic cholelithiasis or suggestive of malignancy (weight loss)
May see jaundice
109
Cholangiocarcinoma arises from...
Epithelial cells of the bile ducts
Can be intrahepatic, perihilar, or distal (extrahepatic) biliary tree
Exclusive of GB and ampulla of vater
110
What is the incidence of cholangiocarcinoma?
Rare in the US, with slightly higher incidence in men
111
Cholangiocarcinoma is associated with...
PSC and choledochal cysts
112
Presentation of cholangiocarcinoma?
```
Jaundice
Pruritis
Abdominal pain
Anorexia
Weight loss
Palpable gallbladder (Courvoisier sign)
```
Labs may show cholestasis
Increased CA19-9 (screen those with PSC)
113
Cancer arising distal to the bifurcation of the distal CBD and pancreatic duct
Ampullary carcinoma
114
There is an increased incidence of ampullary carcinoma with...
Familiar Adenomatous Polyposis (FAP) and Hereditary Non-Polyposis Colon Cancer (HNPCC)
115
How does ampullary carcinoma present?
```
Obstructive Jaundice (most common)
Occult GI bleed with microcytic anemia
Abdominal pain
```
116
Name that diagnosis:
Women
Pruritis
(+) AMA titer
PBC
117
Name that diagnosis:
Men
IBD
Risk for cholangiocarcinoma
PSC
118
Name that diagnosis:
Men
Post-puberty
Elevated unconjugated hyperbilirubinemia
Gilbert Syndrome
119
How do you manage PSC?
Referral to GI for med management and treatment of complications
