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153 Cards in this Set

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What is the term for gallbladder inflammation?
Cholecystitis
What are the clinical manifestations of Acute Cholecystitis?
- Prolonged (>4-6 hours), steady RUQ pain / epigastric pain
- Pain may radiate to the shoulder or back
- Fever
- Leukocytosis
- Gallbladder inflammation
- Abdominal guarding
- Murphy's sign
What is Acute Cholecystitis typically associated with?
- Gallstones (calculous and xanthogranulomatous)
- Sometimes acalculous
What is Chronic Cholecystitis typically associated with?
Almost always associated w/ gallstones
What happens in Chronic Cholecystitis?
- Chronic inflammatory cell infiltration
- Almost always associated w/ gallstones
- No correlation w/ symptoms
- Mechanical irritation or recurrent acute cholecystitis → fibrosis
What is one type of Chronic Cholecystitis?
Porcelain gallbladder
What is the pathogenesis of Acute Cholecystitis?
- Cystic duct obstruction in addition to additional irritant → release of inflammatory mediators (prostaglandins?)
What pain occurs in Acute Cholecystitis?
- Prolonged (>4-6 hours), steady RUQ and epigastric pain
- Pain may radiate to the shoulder or back
What abdominal physical exam findings are there in Acute Cholecystitis?
- Abdominal guarding: associated w/ local parietal peritoneal inflammation
- Murphy's sign: increased discomfort when patient takes a deep breath in while examiner palpates RUQ
What lab findings are not common in Acute Cholecystitis?
Elevated bilirubin and elevated alkaline phosphatase are not common
What imaging is done to diagnose Acute Cholecystitis?
- Abdominal ultrasound
- Cholescintigraphy / HIDA scan
- CT
What are you looking for on abdominal ultrasound to diagnose Acute Cholecystitis? How sensitive / specific is this test?
- Cholelithiasis (gallstones in gallbladder)
- Gallbladder wall thickening >4-5 mm or edema
- Sonographic Murphy's sign (increased discomfort when patient takes deep breath while palpating RUQ)
- 88% sensitive and 80% specific
- Cholelithiasis (gallstones in gallbladder)
- Gallbladder wall thickening >4-5 mm or edema
- Sonographic Murphy's sign (increased discomfort when patient takes deep breath while palpating RUQ)
- 88% sensitive and 80% specific
What are you looking for on Cholescintigraph / HIDA scan to diagnose Acute Cholecystitis?
- Labeled HIDA injected intravenously → taken up by hepatocytes → excreted in bile
- If there is no visualization of the gallbladder it is d/t cystic duct obstruction
- Labeled HIDA injected intravenously → taken up by hepatocytes → excreted in bile
- If there is no visualization of the gallbladder it is d/t cystic duct obstruction
What are you looking for on CT to diagnose Acute Cholecystitis? How sensitive / specific is this test?
- Gallbladder wall edema
- Pericholecystic stranding and fluid
- High-attenuation bile
- Can be particularly useful when complicated cholecystitis is suspected
- Not a good modality to detect gallstones
What is CT not good at detecting related to the gallbladder?
Poor at detecting gallstones
What is the most common complication of Acute Cholecystitis?
Gangrene
What are the possible complications of Acute Cholecystitis?
- Gangrene
- Perforation (may lead to abscess)
- Cholecystoenteric fistula
- Emphysematous cholecystitis
When do perforations usually occur in Acute Cholecystitis?
- After development of gangrene
- May result in an abscess
Where can a Cholecystoenteric Fistula (complication of Acute Cholecystitis) lead to?
- Usually leads to the duodenum or jejunum
- A gallstone could pass through the fistula ("gallstone ileus") which could cause mechanical bowel obstruction in terminal ileum
What is gallstone ileus?
- Passage of gallstone through fistula
- Leads to a mechanical bowel obstruction usually in the terminal ileum

- Caused by cholecystoenteric fistula (a complication of Acute Cholecystitis)
What is Emphysematous Cholecystitis?
- Secondary infection of gallbladder wall w/ gas-forming organisms
- Usually leads to gangrene and perforation
- Complication of Acute Cholecystitis
How do you treat Acute Cholecystitis?
- May ablate (surgically remove) in 7-10 days if not treated
- Antibiotics
- Pain control: NSAIDs, opioids
- Gallbladder drainage (percutaneous cholecystostomy, endoscopy)
- Surgery
When should you do surgery for treating Acute Cholecystitis?
- Immediate cholecystectomy for patients w/ complications or who are low risk
- Delayed cholecystectomy in high risk patients (eg, severe chronic illness, low-risk patient w/ sepsis)
What is the prognosis of Acute Cholecystitis?
~3% mortality
- <1% in young healthy patients
- Up to 10% in high-risk patients or those w/ complications
What can cause Acalculous Cholecystitis?
Gallbladder stasis and ischemia → local inflammatory response → secondary infection
What are the clinical manifestations of Acalculous Cholecystitis?
- Unexplained fever
- Leukocytosis
- Abdominal pain
- Non-specific liver test elevations

- May present similarly to calculous cholecystitis
Who is at risk for Acalculous Cholecystitis?
Typically seen in hospitalized, critically ill patients
What lab tests can you do to diagnose Acalculous Cholecystitis?
- Abdominal US: no cholelithiasis (gallstones), gallbladder wall thickening > 3mm, sonographic Murphy's sign, pericholecystic fluid
- HIDA scan: lack of gallbladder visualization
- CT
How do you treat Acalculous Cholecystitis?
- Antibiotics
- Percutaneous cholecystostomy
- Cholecystectomy (not typically required once underlying problem is addressed, only if the cholecystostomy does not lead to improvements or is contraindicated)
When would a cholecystectomy be indicated for treating Acalculous Cholecystitis?
- If cholecystostomy does not result in clinical improvement
- If cholecystostomy is contraindicated

- Typically not required once underlying problem is addressed
What is the prognosis for Acalculous Cholecystitis?
- High mortality (75%) w/ delayed treatment
- Overall mortality of 30%
What is the term for extravasation of bile into the gallbladder wall?
Xanthogranulomatous Cholecystitis
What happens in Xanthogranulomatous Cholecystitis?
- Extravasation of bile into the gallbladder wall → inflammatory reaction (fibroblasts and macrophages phagocytose biliary lipids in bile) → xanthoma cells
- Gallstones present in ALL patients
What kinds of cells phagocytose biliary lipids in bile in Xanthogranulomatous Cholecystitis? What does this lead to?
- Fibroblasts and Macrophages phagocytose biliary lipids in bile
- Leads to xanthoma cells
What is the clinical presentation of Xanthogranulomatous Cholecystitis?
- History suggestive of Acute Cholecystitis
- Can mimic gallbladder cancer
- High rate of complications
What kind of complications can occur in Xanthogranulomatous Cholecystitis?
- Perforation
- Fistulas into adjacent structures
- Abscess
How do you diagnose Xanthogranulomatous Cholecystitis?
- Abdominal US: hypoechoic nodules or bands in gallbladder wall most characteristic
- CT: intramural hypodense nodules
How do you treat Xanthogranulomatous Cholecystitis?
- Cholecystectomy (usually open)
- Pre-operative cholangiogram to exclude bile duct cancer
What does Xanthogranulomatous Cholecystitis mimic?
Gallbladder cancer
What causes "Porcelain Gallbladder"?
Chronic cholecystitis w/ intramural calcification of gallbladder wall
How common is Porcelain Gallbladder? Who is more likely to get it?
- Uncommon (0.06-0.08%)
- More common in females (5:1)
What is there increased risk of in Porcelain Gallbladder? What increases the risk?
Gallbladder cancer (0-62%) - incomplete calcification of gallbladder wall associated w/ higher risk than complete calcification
What is the clinical presentation of Porcelain Gallbladder?
- Asymptomatic
- Biliary type pain
- Palpable gallbladder
How do you diagnose Porcelain Gallbladder?
- Plain abdominal x-ray
- CT
- Abdominal ultrasound
- Plain abdominal x-ray
- CT
- Abdominal ultrasound
How do you treat Porcelain Gallbladder?
- Cholecystectomy for incomplete calcification or symptomatic patients w/ complete calcification
- Consider cholecystectomy for asymptomatic patients w/ complete calcification
When is Cholecystectomy definitely indicated for Porcelain Gallbladder? When should you only consider it?
- Indicated: incomplete calcification or for complete calcification in symptomatic patients

- Consider: complete calcification in asymptomatic patients
How commonly are gallbladder polyps found when a patient undergoes a gallbladder US?
1.5-4.5%
What are the types of benign gallbladder polyps?
- Cholesterol
- Adenomyomatosis
- Inflammatory
- Adenomas
What is found in benign cholesterol gallbladder polyps?
Abnormal deposits of TGs, cholesterol precursors, and cholesterol esters into the gallbladder mucosa
What is found in benign adenomyomatosis gallbladder polyps?
- Overgrowth of mucosa
- Thickening of muscle wall
- Intramural diverticula
Adenomyomatosis gallbladder polyps (benign) are associated with what? More common in what? Risk for cancer?
- Associated w/ cholelithiasis (gallstones)
- More common in women
- No conclusive evidence of increased risk of GB cancer
What is found in benign inflammatory gallbladder polyps?
Granulation and fibrous tissue w/ plasma cells and lymphocytes
What is found in benign adenoma gallbladder polyps? Relationship to cancer?
- Benign glandular tumors w/ potential for malignancy
- Likelihood of malignant transformation related to size: none less than 12 mm in one series
What is the clinical presentation of gallbladder polyps?
- Asymptomatic / incidental finding
- Biliary pain
- Possibly associated w/ dyspepsia w/ cholesterolosis and adenomyomatosis
How do you diagnose gallbladder polyps?
- Transabdominal US
- Endoscopic US
- CT (most useful in GB cancer)
What is the most useful imaging tool for diagnosing gallbladder cancer?
CT
How do you manage gallbladder polyps? What are the criteria for this procedure?
Cholecystectomy
- Cholelithiasis irrespective of size of gallbladder polyps
- Primary Sclerosing Cholangitis (PSC) irrespective of size of GB polyps
- Biliary cholic (form of pain which starts and stops abruptly) or pancreatitis
- Polyps >10 mm
What is Acute Cholangitis?
- Infection of the bile duct (cholangitis)
- Usually caused by bacteria ascending from its junction with the duodenum
- It tends to occur if the bile duct is already partially obstructed by gallstones
Bacteria entering the small intestine or portal system via a disruption in the sphincter of Oddi causes what?
Acute Cholangitis
What infections are responsible for Acute Cholangitis? How does this infection occur?
G- and G+ colonic bacteria:
- E. coli (25-50%)
- Klebsiella (15-20%)
- Enterococcus species (10-20%)
- Enterobacter species (5-10%)

*Bacteria most likely enter via the Sphincter of Oddi
What are the symptoms of Acute (Ascending) Cholangitis?
Charcot's Triad (50-75%):
- Fever
- Jaundice
- Abdominal pain

Reynolds' Pentad:
- Charcot's Triad +
- Confusion
- Hypotension
What is the term for fever, abdominal pain, and jaundice in Acute Cholangitis? How commonly do these symptoms present?
Charcot's Triad (50-75%)
What is the term for fever, abdominal pain, jaundice, confusion, and hypotension in Acute Cholangitis? Implications?
Reynolds' Pentad (high morbidity and mortality)
What lab tests are seen in Acute Cholangitis?
- Cholestatic liver test elevations
- Leukocytosis
How do you diagnose Acute Cholangitis?
- Clinical signs
- Imaging: dilated biliary system, choledocholithiasis
How do you treat Acute Cholangitis?
- Antibiotics
- Biliary drainage (ERCP, PTC - percutaneous transhepatic cholangiography, or surgery)
How does Biliary Atresia present?
- Biliary obstruction exclusively in the neonatal period
- Progressive, idiopathic, fibro-obliterative disease of the extra-hepatic biliary tree

*Complete obstruction of bile flow caused by destruction or absence of all or part of the extra-he...
- Biliary obstruction exclusively in the neonatal period
- Progressive, idiopathic, fibro-obliterative disease of the extra-hepatic biliary tree

*Complete obstruction of bile flow caused by destruction or absence of all or part of the extra-hepatic bile ducts
Which part of the biliary tree is affected by Biliary Atresia?
Extra-hepatic region
Extra-hepatic region
What are the types of Biliary Atresia?
- Biliary Atresia: 70-85%
- Biliary Atresia Splenic Malformation (BASM): 10-15%
- Biliary Atresia: 70-85%
- Biliary Atresia Splenic Malformation (BASM): 10-15%
How often does Biliary Atresia occur with other congenital malformation? Which ones?
5-10% associated w/ other congenital abnormalities:
- Intestinal atresia
- Imperforate anus
- Kidney anomalies
- Heart malformations
What are the possible causes of Biliary Atresia?
- Viral
- Toxic
- Genetic (possibly in BASM subtype)
- Immune dysregulation
What is the clinical presentation of Biliary Atresia?
- Infant usually born full term, w/ normal birth weight, and initially they thrive
- Jaundice: birth to 8 weeks
- Acholic stools
- Dark urine
How do you diagnose Biliary Atresia?
- Abdominal US
- Liver biopsy
- Cholangiogram (intraoperative, PTC, endoscopic - ERCP)
How do you treat Biliary Atresia?
How do you treat Biliary Atresia?
- Kasai procedure: perform ASAP, jejunum attached to liver to drain bile and duodenum is attached lower down on jejunum 
- Liver transplantation
- Kasai procedure: perform ASAP, jejunum attached to liver to drain bile and duodenum is attached lower down on jejunum
- Liver transplantation
When should a liver transplant be done for treatment of Biliary Atresia?
- 60-80% of patients w/ Biliary Atresia will eventually require a transplant despite optimal management
- Should be deferred as long as possible d/t improved outcomes w/ weights > 10 kg
How does survival / prognosis improve when Biliary Atresia is treated with a liver transplantation?
Without transplant:
- 30-55% 5 year survival
- 30-40% 10 year survival
- 20-40% 20 year survival

With transplant:
- 92% 1 year survival
- 70-80% 5-10 year survival

Vastly improves prognosis!
What are the characteristics of Biliary Cysts? Location?
Cystic dilations that may occur singly or in multiple throughout the biliary tree
What are most Biliary Cysts associated with?
70% associated w/ Abnormal Pancreaticobiliary junction (APBJ)
- Pancreatic and bile duct join outside the duodenal wall
70% associated w/ Abnormal Pancreaticobiliary junction (APBJ)
- Pancreatic and bile duct join outside the duodenal wall
What is an Abnormal Pancreaticobiliary Junction (APBJ)? What is it associated with?
- Pancreatic and bile ducts join together outside the duodenal wall
- Associated with biliary cysts
- Associated with increased risk of gallbladder cancer independent of biliary cysts
- Pancreatic and bile ducts join together outside the duodenal wall
- Associated with biliary cysts
- Associated with increased risk of gallbladder cancer independent of biliary cysts
How common are Biliary Cysts? In whom are they more common?
- 1:100,000 to 150,000
- More common (1:1000) in some Asian countries
- More common in women (3-4:1)
- Equal numbers in children and adults
What are the types of Biliary Cysts? How common is each type? Location?
Type I:
- 50-85%
- Extra-hepatic only

Type IV: 
- 15-35%
- Multiple cysts
- Extrahepatic ± intrahepatic

Type V: 
- 20%
- Intrahepatic only
- Assoc. w/ Caroli's disease
Type I:
- 50-85%
- Extra-hepatic only

Type IV:
- 15-35%
- Multiple cysts
- Extrahepatic ± intrahepatic

Type V:
- 20%
- Intrahepatic only
- Assoc. w/ Caroli's disease
Which type of Biliary Cyst is only found extra-hepatically? How common is it?
Type I (50-85%)
Type I (50-85%)
Which type of Biliary Cyst is found both extra-hepatically and intra-hepatically? How common is it?
Type IV (15-35%)
- Multiple cysts
Type IV (15-35%)
- Multiple cysts
Which type of Biliary Cyst is only found intra-hepatically? How common is it?
Type V (20%)
- Caroli's disease
Type V (20%)
- Caroli's disease
What is the cause / pathogenesis of Biliary Cysts?
- Possibly genetically or environmentally predisposed
- Can be associated w/ developmental anomalies
- Can be congenital or acquired (from APBJ)
What can Biliary Cysts be acquired from?
APBJ: Abnormal Pancreaticobiliary Junction
APBJ: Abnormal Pancreaticobiliary Junction
What are the clinical manifestations of Biliary Cysts?
- Majority present before age 10
- Infants: jaundice, failure to thrive, abdominal mass
- Patients > age 2: chronic intermittent abdominal pain, pancreatitis, intermittent jaundice, cholangitis
How do you diagnose Biliary Cysts?
- Abdominal US
- Cholangiography: ERCP, PTC, intraoperative, MRCP
- CT
What type of cancer is a patient with Biliary Cysts at increased risk for? How much is their risk increased?
20-30 fold increased risk for Cholangiocarcinoma
What types of Biliary Cysts are at increased risk for Cholangiocarcinoma?
- Type I and Type IV biliary cysts
- Increased risk confined to patients only w/ APBJ in one study
What disease is caused by progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts?
Primary Sclerosing Cholangitis (PSC)
What can cause Secondary Sclerosing Cholangitis?
- Recurrent pyogenic cholangitis
- Choledocholithiasis
- Cholangitis
- AIDS cholangiopathy
How common is Primary Sclerosing Cholangitis (PSC)? Who is more likely to get it?
- 1 in 100,000 persons
- 70% men
- Mean age: 40 years
- Women generally diagnosed later than men
What is Primary Sclerosing Cholangitis (PSC) associated with?
Inflammatory Bowel Disease:
- Ulcerative Colitis (UC) > Crohn's Disease
- Up to 90% of patients w/ PSC have UC
- < 10% of patients w/ UC have PSC
What is the cause / pathogenesis of Primary Sclerosing Cholangitis (PSC)?
- Immune activation: humoral and cellular abnormalities
- Genetic factors
- Cystic fibrosis transmembrane conductance regulator mutations
What are the clinical manifestations of Primary Sclerosing Cholangitis (PSC)?
- 50% asymptomatic
- Fatigue
- Pruritus
- Jaundice
What are the lab results of a patient with Primary Sclerosing Cholangitis (PSC)?
- Elevated liver tests in a cholestatic pattern
- 30% Hypergammaglobulinemia
- 40-50% increased IgM
- 30-80% P-ANCA positive
What areas of the bile ducts are affected by Primary Sclerosing Cholangitis (PSC)?
Classic PSC:
- Intrahepatic and extrahepatic (87%)
- Intrahepatic alone (11%)
- Rarely extrahepatic alone (2%)

Small duct PSC:
- Normal cholangiogram
- Involves small caliber bile ducts
How do you diagnose Primary Sclerosing Cholangitis (PSC)?
- CT
- Abdominal US
- Cholangiography: MRI, ERCP

- If cholangiogram is diagnostic, don't need liver biopsy
- Consider liver biopsy for small-duct PSC
What characteristic finding is seen on liver biopsy of Primary Sclerosing Cholangitis (PSC)?
Onion skin pattern around bile ducts
Onion skin pattern around bile ducts
What are the complications of Primary Sclerosing Cholangitis (PSC)?
- Progressive hepatic fibrosis → cirrhosis and portal HTN
- Decreased bile acids → steatorrhea and fat-soluble vitamin malabsorption
- Osteoporosis (not d/t vitamin D malabsorption usually)
- Dominant biliary strictures
- Acute cholangitis
- Cholelithiasis
- Hepatobiliary and colon cancer
What does the progressive hepatic fibrosis in Primary Sclerosing Cholangitis (PSC) lead to?
- Cirrhosis
- Portal HTN
What does the decreased bile acids in Primary Sclerosing Cholangitis (PSC) lead to?
- Steatorrhea
- Malabsorption of fat-soluble vitamins
What cancers are you at increased risk for if you have Primary Sclerosing Cholangitis (PSC)?
Hepatobiliary:
- Cholangiocarcinoma (10-15% lifetime risk)
- Gallbladder (3-14% prevalence)
- Hepatocellular in setting of cirrhosis

Colon:
- Approximately 4-fold increased risk w/ PSC and UC compared to UC alone
How do you treat Primary Sclerosing Cholangitis (PSC)?
- Medical therapy not recommended
- ERCP for dominant extra-hepatic strictures
- Surgery: biliary reconstruction (avoid if possible) or liver transplantation

*Liver transplantation has better long-term outcomes, biliary reconstruction can complicate future liver transplantation surgery
What gallbladder disease is associated with AIDS?
AIDS Cholangiopathy
What causes AIDS Cholangiopathy?
Biliary obstruction resulting from infection-related (classically Cryptosporidium parvum) strictures of the biliary tract
What AIDS patients are at risk for AIDS Cholangiopathy?
AIDS patients w/ CD4 count < 100 / mm3
What are the symptoms of AIDS Cholangiopathy?
- RUQ pain
- Epigastric pain
- Diarrhea
What lab tests are seen in AIDS Cholangiopathy?
Cholestatic liver enzyme elevations
How do you diagnose AIDS Cholangiopathy?
- Transabdominal US (high negative predictive value)
- MRCP
- ERCP if ultrasound is positive
How do you treat AIDS Cholangiopathy?
- Biliary sphincterotomy (cutting the biliary sphincter during ERCP)
- Stenting of dominant extra-hepatic strictures
- Anti-microbial treatment NOT effective
- Ursodeoxycholic acid may be helpful in small series
What parasites can infect the biliary tree?
- Ascaris lumbricoides
- Echinococcus granulosus
- Clonorchis sinensis (Chinese Liver Fluke)
- Opistohorchiasis
- Fasciola hepatica (Sheep Liver Fluke)
What parasites can affect the biliary tree? Where are they found?
Ascaris lumbricoides
- Roundworm that is found world-wide
- Adult worms inhabit human small intestine

Echinococcus granulosus
- Tapeworm found in S. America, Middle East, E. Mediterranean, some sub-Saharan countries, China, and former Soviet Union
- Canines are hosts

Clonorchis sinensis (Chinese Liver Fluke)
- Far East and far eastern Russia
- Dogs and cats are reservoirs

Opistohorchiasis
- Liver fluke in SE Asia and Central / Eastern Europe
- Cats, dogs, and fish-eating mammals

Fasciola hepatica (Sheep Liver Fluke)
Which biliary parasites are:
- Roundworms?
- Tapeworms?
- Liver Flukes?
- Roundworm: Ascaris lumbricoides
- Tapeworm: Echinococcus granulosus
- Liver Fluke: Clonorchis sinensis (Chinese), Opisthorchiasis (cats, dogs), Fasciola hepatica (sheep)
How do you treat the biliary parasites?
Ascaris lumbricoides:
- ERCP for removal
- Anti-helminthic therapy

Echinococcus granulosus:
- Surgical resection
- Percutaneous injection of scolicidal agents
- Anti-helminthic therapy

Clonorchis sinensis AND Opisthorchiasis AND Fasciola hepatica (all liver flukes):
- Anti-helminthic therapy
- ERCP for acute cholangitis
Ascaris lumbricoides:
- Type
- Location
- Reservoir
- Diagnosis
- Treatment
- Roundworm
- Worldwide
- Inhabits human small intestine
- Diagnose w/ US (long, linear, parallel echogenic structure w/o acoustic shadowing) or w/ ERCP
- Treat by removing w/ ERCP or w/ anti-helminthic therapy
Echinococcus granulosus:
- Type
- Location
- Reservoir
- Symptoms
- Treatment
- Tapeworm
- S. America, Middle East, E. Mediterranean, some Sub-Saharan countries, China, former Soviet Union
- Canines are hosts
- Rupture of hepatic cyst into biliary system, jaundice and hepatomegaly
- Treat by surgically resecting or percutaneous injection of scolicidal agents in addition to anti-helminthic therapy
Clonorchis sinensis:
- Type
- Location
- Reservoir
- Symptoms
- Treatment
- Chinese liver fluke
- Far East and far eastern Russia
- Reservoirs are cats and dogs
- Asymptomatic, cholangitis, chronic infection associated w/ cholangiocarcinoma
- Treat w/ anti-helminthic therapy or ERCP for acute cholangitis
Opisthorchiasis:
- Type
- Location
- Reservoir
- Symptoms
- Treatment
- Liver fluke
- SE Asia and C/E Europe
- Found in cats, dogs, and fish-eating mammals
- Asymptomatic, cholangitis, chronic infection associated w/ cholangiocarcinoma
- Treat w/ anti-helminthic therapy or ERCP for acute cholangitis
Fasciola hepatica:
- Type
- Cause
- Reservoir
- Symptoms
- Treatment
- Sheep liver fluke
- Eating raw vegetables infected w/ metacercariae
- Human infection
- Penetrates duodenal wall, migrates across peritoneum, and enters biliary system
- Treat w/ anti-helminthic therapy and ERCP for acute cholangitis
Which biliary disease is found almost exclusively in patients from SE Asia?
Recurrent Pyogenic Cholangitis
What causes Recurrent Pyogenic Cholangitis?
- Pigment stone formation in intra-hepatic biliary system
- Results in intra-hepatic stricturing and biliary obstruction
- Leads to recurrent bouts of acute cholangitis
Which patients are affected by Recurrent Pyogenic Cholangitis?
Almost exclusively in patients from SE Asia
What are the clinical manifestations of Recurrent Pyogenic Cholangitis?
Acute cholangitis
- Infection of the bile duct (cholangitis)
- Usually caused by bacteria ascending from its junction with the duodenum
- It tends to occur if the bile duct is already partially obstructed by gallstones
How do you diagnose Recurrent Pyogenic Cholangitis?
- Abdominal US
- MRI
- CT
- PTC
- ERCP
How do you treat Recurrent Pyogenic Cholangitis?
- Treat acute cholangitis (antibiotics and biliary drainage)
- Stone clearance (ERCP, PTC, surgical)
- Consider Ursodeoxycholic acid (limited data)
- Hepatic resection and reanastomosis
What are the potential complications of Recurrent Pyogenic Cholangitis?
- Cirrhosis from secondary sclerosing cholangitis
- Increased risk for cholangiocarcinoma
What is the term for gallstones?
Cholelithiasis
What are the symptoms of Cholelithiasis?
- Often asymptomatic (up to 90%)
- Approximately 20% will become symptomatic
- Higher likelihood of continued symptoms or complications (eg, pancreatitis or cholecystitis) w/ symptomatic gallstones
What does the term Cholelithiasis indicate?
Gallstones or sludge in the gallbladder
What are the potential complications of Cholelithiasis?
- Pancreatitis
- Cholecystitis
What are the risk factors for Cholelithiasis?
- Pregnancy and estrogen and oral contraceptives
- Terminal ileal resection
- Gallbladder stasis (DM, total parenteral nutrition)
- Reduced physical activity (men)
- Age (increased risk w/ age)
- Gender (females)
- Ethnicity (50-75% prevalence of cholesterol gallstones in Pima Indians and certain other Native Americans)
- Obesity
- Rapid weight loss
- Cirrhosis
- Hemolytic anemias
- Hypertriglyceridemia
What is the term for intermittent cystic duct obstruction? Symptoms?
Biliary Colic:
- Crescendo steady pain in RUQ that can radiate to back and right shoulder
- Nausea
- After ingestion of fatty foods
- Lasts < 4 hours before abating completely
What causes biliary colic?
- Intermittent cystic duct obstruction
- Brought on after ingestion of fatty foods
How long does Biliary Colic last?
Less than 4 hours at a time before abating completely
How do you diagnose Cholelithiasis? Sensitivity / specificity?
- Transabdominal US: sensitivity 84% and specificity 99%
- Endoscopic US: sensitivity 96% and specificity 86%
Which method of diagnosing Cholelithiasis is more sensitive? Specific?
- Sensitive: Endoscopic Ultrasound
- Specific: Transabdominal Ultrasound
How do you treat asymptomatic Cholelithiasis?
Prophylactic cholecystectomy ONLY for patients with risk factors
What makes a cholecystectomy for asymptomatic Cholelithiasis indicated?
- Biliary cysts
- Caroli's disease
- APBJ
- Gallbladder adenomas
- Porcelain gallbladder
- Patients w/ Sickle Cell Disease, hereditary spherocytosis, or undergoing gastric bypass surgery
How do you treat a patient with Cholelithiasis and Biliary Cholic?
- Cholecystectomy
- Non-surgical: Ursodeoxycholic acid, extracorporeal shockwave lithotripsy
What is the term for gallstones or sludge in the common bile duct?
Choledocholithiasis
What is Choledocholithiasis?
Stones or sludge in the common bile duct
What causes most Choledocholithiasis?
- Mostly secondary to passage of Cholelithiasis
- Primary is less common (eg, cystic fibrosis, periampullary diverticulum large bile ducts)
What are the clinical manifestations of Choledocholithiasis?
- Asymptomatic
- Intermittent RUQ pain (more prolonged than biliary cholic and resolves w/ stone passage, removal, or ball-valve effect (does not obstruct opening)
- Nausea / vomiting
- Elevated liver tests typically in cholestatic patient
How do you diagnose Choledocholithiasis?
* Endoscopic US: 94% sensitivity and 95% specificity
- Abdominal US has poor sensitivity (38-42%) b/c overlying bowel gas, negative study does not exclude
- Cholangiography: ERCP, MRCP, intra-operative
* Endoscopic US: 94% sensitivity and 95% specificity
- Abdominal US has poor sensitivity (38-42%) b/c overlying bowel gas, negative study does not exclude
- Cholangiography: ERCP, MRCP, intra-operative
How do you treat Choledocholithiasis?
- ERCP
- Intra-operative bile duct exploration
What is the syndrome that causes common hepatic duct obstruction from extrinsic compression by an impacted stone in the cystic duct or Hartmann's pouch of the gallbladder?
Mirizzi Syndrome
What are the features of Mirizzi Syndrome?
- Common hepatic duct obstruction from extrinsic compression
- May be by an impacted stone in the cystic duct or Hartmann's pouch of the gallbladder
- Common hepatic duct obstruction from extrinsic compression
- May be by an impacted stone in the cystic duct or Hartmann's pouch of the gallbladder
What are the clinical manifestations of Mirizzi Syndrome?
- Jaundice
- RUQ pain
- Fever
- Elevated liver tests in cholestatic pattern
What is Mirizzi Syndrome associated with?
Gallbladder cancer
How do you diagnose Mirizzi Syndrome?
How do you diagnose Mirizzi Syndrome?
- Abdominal US
- Cholangiography (ERCP > PTC, MRCP)
How do you treat Mirizzi Syndrome?
How do you treat Mirizzi Syndrome?
- Surgery: cholecystectomy ± bilioenteric anastomsis
- Endoscopic: limited role