Biliary Disease Flashcards
Function of bile
Digestion and absorption of fats
Vehicle for excretion of bilirubin, excess cholesterol and metabolic byproducts
Stones in the gallbladder
Cholelithiasis
Inflammation of the gallbladder
Cholecystitis
Stones in the common bile duct
Choledocholithiasis
Inflammation of the bile ducts
Cholangitis
Disruption of bile flow regardless of cause
Cholestasis
Risk factors for cholelithiasis
Four Fs (female, fluffy, forty, fertile)
Females, Age 40, Obese, Pregnant, Estrogen (OCTs, HRT)
Also rapid weight loss, family hx, ethnicity (Native Americans), diabetes
Types of stones
Cholesterol mostly
Can be pigment (black or brown)
Types of presentation of cholelithiasis
Asymptomatic incidental stones (MOST)
Uncomplicated disease (biliary colic and symptomatic in the absence of gallstone related complications)
Complicated gall stone disease with complications
Complications that can arise in complicated gallstone disease
Aute cholecystitis
Choledocholithiasis with or without acute cholangitis
Gallstone pancreatitis
Initial test of choice for cholelithiasis
Ultrasound (very sensitive to detect stones)
Other diagnostics for cholelithiasis
Abd plain film (only positive in some pts) CT abdomen (less sensitive)
Tx for asymptomatic gallstones
Mostly don’t need tx, manage expectantly
When to refer for cholecystectomy with asymptomatic gallstones
Increased risk of gallbladder cancer Hemolytic disorders (sickle cell, G6PD etc)
Why does biliary colic/uncomplicated gallstone disease occur?
Gall bladder contracts forcing stone against outlet or cystic duct opening–increased intra gall bladder pressure resulting in pain–when relaxes, obstruction is relieved and pain subsides
**no inflammation of the gall bladder!
Classic description of biliary type pain in biliary colic
Intense RUQ/epigastric pain that may radiate to R shoulder blade
Constant and steady
Pain lasting at least 30 min then plateaus (<5-6 hrs)
Postprandial pain (fatty or greasy foods)
N/v, diaphoresis
Not worse with movement
Not relieved with squatting, bowel movements or gas
Noctural pain waking pt
PE for biliary colic
Often normal vitals (NO fever or tachycardia) Not ill appearing NO evidence of jaundice Anicteric eyes No peritoneal signs, - Murphys
Labs in biliary colic
Normal CBC, LFTS, amylase and lipase
Preferred diagnostic test for biliary colic
US (seeing gallstones or gallbladder sludge)
Management for uncomplicated gallstones/biliary colic
Cholecystectomy (prevents recurrence and complications)
What is functional gall bladder disorder/biliary dyskinesia?
Biliary type pain in ABSENCE of gallstones, sludge, microlithiasis or microcrystal disease
Results from gall bladder dysmotility
Labs in functional gall bladder disorder
Normal CBC, LFTS, amylase, lipase
Normal imaging and upper endoscopic exam
How to diagnose functional gall bladder disorder
Diagnosis of exclusion when have biliary type pain (make sure not PUD, functional dyspepsia or ischemia heart)
If no other causes, order CCK-stimulated cholescintigraphy (HIDA scan)
How HIDA scan is used in functional gallbladder disorder
CCK is given to stimulate GB to contract
Calculate GB ejection fraction
GBEF <35-40% is considered low and supports
Criteria to diagnose functional gallbladder disorder
Rome IV criteria REQUIRES
Biliary pain
Absence of gallstones of other structural pathology
Other criteria supportive but not required
Low ejection fraction on scintigraphy
Normal liver enzymes, conjugated bilirubin and amylase/lipase
How to exactly define biliary pain
Pain in epigastrium and or RUQ
Builds up to steady level and lasts at least 30 min
Occurs at variable intervals (not daily)
Is severe enough to interrupt daily activities or lead to an ED visit
Is not significantly (<20%) relieved by BMs, postural changes or acid suppression
Other things supportive:
Pain associated with n/v
Pain radiates to back and or right subscap region
Pain awakens pt from sleep
Management for functional gallbladder disorder
Cholecystectomy recommended for those with typical biliary type pain and low GBEF (<40%)
What is acute calculous cholecystitis?
Acute inflammation of the GB
-mostly complication of gallstone disease
Occurs with cystic duct obstruction and gallbladder inflammation (must have both!!)
Presentation of acute calculous cholecystitis
Begins as attack of biliary pain that progressively worsens (usually have had a previous experience)
Prolonged (>4-6 hrs) steady, severe RUQ or epigastric pain
May radiate to right shoulder or back
Fever, n/v, anorexia
Often hx of fatty food ingestion
What is seen on PE for acute calculous cholecystitis?
Fever and tachycardia Ill appearing, lying still No jaundice Anicteric Abd exam: RUQ tenderness, voluntary or involuntary guarding, + Murphy's sign
Labs seen with acute calculous cholecystitis
Leukocytosis with a left shift
Mild elevation in serum AST/ALT
Elevated serum total bilirubin and alk phos NOT COMMON (think biliary obstruction)
Normal serum amylase
Preferred imaging for acute calculous cholecystitis
US (see gallstones, wall thickening >4-5 mm or edema, pericholecystic fluid, + sonographic Murphys sign)
HIDA for acute calculous cholecystitis
Use if uncertain of diagnose
+ if failure to visualize GB in setting of cystic duct obstruction
Most common complication of acute calculous cholecystitis
Gangrene (older pts, DM, those delaying therapy-sepsis like pic)
Other complications of acute calculous cholecystitis
Perforation (often after developing gangrene)
Cholecystoenteric fistula
Gallstone ileus
Emphysematous cholecystitis, empyema, hydrops
Mirizzi syndrome
What is Mirizzi syndrome?
Stone in the cystic duct or Hartmann’s pouch of the GB may compress common hepatic duct and cause jaundice!
Management of acute calculous cholecystitis
Admit, NPO, IVF
IV pain (Ketorolac, Morphine, Meperidine)
IV abx (empiric)
Mainstay is cholecystectomy
What is chronic cholecystitis?
Chronic inflammation of the GB almost always associated with gallstones (repeated acute/subacute cholecystitis or prolonged medical irritation of GB wall)
Presentation of chronic cholecystitis
May have minimal sxs (asymptomatic for yrs and may progress to symptomatic GB disease of acute cholecystitis or just present with complications)
What is acalculous cholecystitis?
Acute necroinflammatory disease of GB in absence of gallstones (high morbidity and mortality)
Pathogenesis of acalculous cholecystitis
GB stasis and ischemia (local inflammatory response in GB wall) and secondary bacterial infection and maybe perf
When is acalculous cholecystitis common?
Hospitalized and critically ill pts (trauma, burn, postpartum, ortho surgery)
Presentation of acalculous cholecystitis
May be similar to acute calculous or may have picture of sepsis related cholestasis and jaundice (so think with critically ill pt with sepsis without clear source or jaundice)
Management for acalculous cholecystitis
Must do quick to start abx (get cultures)
Cholecystectomy vs GB drainage
Labs for acalculous cholecystitis
US, CT, HIDA
LFTs, CBC, lytes, pancreatic enzymes, UA
What is choledocholithiasis?
Gallstones in common bile duct (increasing incidence with age)
Can block flow of bile and cause jaundice!!!
History for choledocholithiasis
Usually with biliary type pain (more prolonged than with typical biliary colic)
Can be asymptomatic tho
Report jaundice
PE for choledocholithiasis
Vital signs WNL when uncomplicated
RUQ or epigastric tenderness
May be jaundiced
Courvoisier signs (palpable gallbladder maybe)
Labs for choledocholithiasis
No leukocytosis
Elevated LFTs (cholestasis)
Normal amylase and lipase
Elevated liver tests in choledocholithiasis
Elevated ALT and AST early on with biliary obstruction
Later on, elevated in cholestatic pattern (increase in serum bilirubin, alk phos, gamma-glutamyl transpeptidase that are higher than AST and ALT)
Initial imaging for choledocholithiasis
US (cholelithiais, CBD stone and CBD dilation)–MRCP or endoscopic us if unsure
Why is MRCP used in choledocholithiasis?
Great to see biliary tree and pancreatic ducts
Can confirm dx of CBD stone
Management of choledocholithiasis
Remove CBD stone and treat complications if not gotten out in time
ERCP is therapeutic and diagnositc
Cholecystectomy if appropriate
Complications of choledocholithiasis
Acute cholangitis or acute pancreatitis
What is acute cholangitis?
Inflammation of biliary duct system
Cause of acute cholangitis
Bacterial infection in pt with biliary obstruction (choledolithiasis, benign biliary stricture, s/p ERCP, malignancy)
Typically ascending from the duodenum
What must be remembered with acute cholangitis?
“Pus under pressure” is surgical emergency so must do something immediately
Manifestations of acute cholangitis
Charcots (fever, abd pain, jaundice)
Reynolds (Charcots and AMS and hypotension)
Labs for acute cholangitis
Leukocytosis with a left shift
Elevated CRP or ESR
Liver tests show cholestasis (increased bilirubin, alk phos, GGT and maybe AST/ALT)
Can see increased amylase or lipase with associated pancreatitis
+blood cultures
Imaging for acute cholangitis
U/s
CT
MRCP (or EUS) to see CBD dilation or stone
Management for acute cholangitis
Admit and monitor for sepsis (empiric abx)
Consult GI and surgery immediately (ERCP to do biliary drainage)
Biliary drainage with acute cholangitis
Must relieve the biliary obstruction!!
ERCP with sphincterotomy and stone extraction or stent insertion
Can follow with cholecystectomy if needed
What does increased alk phos, bilirubin or GGT suggest?
Cholestasis (biliary obstruction-choledocho)
Who do you see primary biliary cholangitis in?
Women 30-65
What is primary biliary cholangitis?
Autoimmune destruction of intrahepatic bile ducts which causes cholestasis
May result in cirrhosis or liver failure
Presentation of primary biliary cholangitis
Maybe asymptomatic
Fatigue, pruritus, jaundice, RUQ discomfort, skin hyperpigmentation, xanthelasmas, hepatomegaly
What is primary biliary cholangitis associated with?
Other autoimmune conditions (Sjogrens, thyroid)
Labs in primary biliary cholangitis
Cholestatic patter of liver tests (elevated alk phos, GGT and maybe AST/ALT, increased bilirubin later on)
AMA (antimitochondrial!!!!)
ANA but not specific
Hyperlipidemia
Complications of primary biliary cholangitis
Cirrhosis
Hepatocellular carcinoma
Malabsorption (and steatorrhea)
Metabolic bone disease
How to diagnose primary biliary cholangitis
No extrahepatic biliary obstruction
No comorbidity affecting liver
Must have 2: elevated alk phos (1.5x), +AMA, histologic evidence of PBC
Management of primary biliary cholangitis
Refer to GI for meds and complication tx
What is primary sclerosing cholangitis?
Sclerosing, inflammatory and obliterative process involving intrahepatic and or extra hepatic biliary tree
Who do we see primary sclerosing cholangitis in ?
Men (usually around 40)
Close association of primary sclerosing cholangitis
IBD (mostly UC!!!!)
Ultimate complications of primary sclerosing cholangitis
Cholestasis and end stage liver disease
Presentation of primary sclerosing cholangitis
Asymptomatic, fatigue, pruritus, jaundice, hepatomegaly
Labs for primary sclerosing cholangitis
Cholestatic pattern of liver tests
Increased IgM, + P-ANCA, autoantibodies (ANA,ASMA)
Negative AMA!!!
How to diagnose primary sclerosing cholangitis
Abnormal liver tests especially with IBD
Cholangiography (MRCP or ERCP-multifocal stricturing and dilation of intrahepatic or extrahepatic bile ducts)
Liver bx
Complications of primary sclerosing cholangitis
End stage liver disease Fat soluble vit deficiencies Metabolic bone disease Cholangitis and cholelithiasis Hepatobiliary cancer Colon cancer (pts with UC)
Management of primary sclerosing cholangitis
Refer to GI for meds and complication tx
What is Gilbert syndrome?
Inherited disorder of deficiency in enzyme for glucuronidation of bilirubin in liver
Have unconjugated hyperbilirubinemia in absence of hemolysis (normal CBC, blood smear and retic count)
Presentation of Gilbert syndrome
Present during adolescence (post puberty)
Males
Mild intermittent episodes of jaundice!!!
Otherwise probably asymptomatic
Risk factors for gall bladder cancer
Gallstone disease Gallbladder polyps Porcelain GB PSC Chronic infection Obese
Presentation of GB cancer
May be asymptomaatic, mimic cholelithiais or malignancy or see jaundice
What does cholangiocarcinoma arise from?
Epithelial cells of bile ducts (not GB or ampulla or vater)
Association of cholangiocarcinoma
PSC and choledochal cysts (cystic dilatations of bile ducts)
Presentation of cholangiocarcinoma
Jaundice, pruritus, abd pain, anorexia, weight loss, Courvoisier
Labs are cholestatic pattern
Increased CA 19-9 (Screen when have PSC)
Where does ampullary carcinoma arise?
In ampulla of vater distal to bifurcation of distal CBD and pancreatic duct
When is there and increased risk of ampullary carcinoma?
With familial adenomatous polyposis (FAP) and HNPCC
Presentation of ampullary carcinoma
Obstructive jaundice most, can be occult GI bleed with microcytic anemia and abd pain
