Flashcards in 17. Biliary Tract: Gallbladder and Biliary Disease Deck (110):
What are the three main problems of the gallbladder that were discussed?
Cholecystitis: calculous cholecystitis, acalculous, xanthogranulomatous
What is cholecystitis?
What does acute choecystitis present with?
What is the term for when cholecystitis is NOT associated with a gallstone?
**calculus and xanthogranulomatous are associated with gallstones
Chronic cholecystitis is almost always associated with:
What happens in chronic cholecystitis?
Mechanical irritation or recurrent acute cholecystitis --> fibrosis
What is the pathogenesis of acute cholecystitis?
Cystic duct obstruction in addition to irritant (lysolecithin) --> Release of inflammatory mediators (prostaglandins)
What are the clinical manifestations of acute cholecystitis?
1. Prolonged (over 4-6 hr) RUQ/epigastric pain with radiation to the shoulder or back
3. Abdominal guarding: local parietal peritoneal inflammation
4. Murphy's sign
What is murphy's sign for acute cholecystitis?
Increased discomfort when the patient takes a deep breath in while the examiner palpates RUQ
Is there elevated bilibrubin and ALP with acute cholecystitis?
What imaging is done for acute cholecystitis?
What abdominal ultrasound findings suggest acute chol?
Cholelithiasis, wall thickening over 4-5 mm or edema, sonographic Murphy's sign
What happens in cholescintigraphy/99mTc-hepatic imindiacetic acid (HIDA) scans?
Labelled HIDA injected IV
Taken up by hepatocytes
Excreted in the bile
**no visualization of the gallbladder due to cystic duct obstruction
What will a CT show with acute chol?
Gallbladder wall edema
Pericholecystic stranding and fluid
**not a good modality to detect gallstones
What is the most common complication of acute cholecystitis?
What can happen after the development of gangrene from acute chol?
Perforation; localized, resulting in abscess
4 complications of acute chol?
What happens in a cholecystoenteric fistula from acute chol?
Fistula into duodenum or jejunum allows passage of gallstone, mechanical bowel obstruction, usually in the terminal ileum (gallstone ileus)
What is emphysematous cholecystitis? (complication of acute chol)
Secondary infection of the gallbladder wall with gas forming organisms
--usually leads to gangrene and perforation
Tx of acute cholecystitis
May abate in 7-10 days if not treated
Pain control: NSAIDs and opioids
Gallbladder drainage (percutaneous, endoscopic)
When is immidiate surgery advised for acute chol? Delayed cholecystectomy?
Patients with complications or low risk
High risk patients: severe chronic illness, low-risk patients with sepsis
Prognosis of acute cholecystitis?
Mortality of approximately 3%
- less than 1% in young healthy patients
- up to 10% in high-risk patients or those with complications
What happens in acalculous cholecystitis (pathogenesis)?
Gallbladder stasis and ischemia ->
Local inflammatory response ->
Who typically gets acalculous cholecystitis?
Hospitalized, critically ill patients
What is the clinical presentation of acalculous chol?
Similar to calculous--fever, leukocytosis, abdominal pain
**non-specific liver enzyme tests
Diagnosis of acalculous chol
Abdominal ultrasound: no cholelithiasis, wall thickening over 3 mm, sonographic Murphy's sign, percholecystic fluid
HIDA scan: lack of gallbladder visualization
Three components of tx for acalculous cholecystitis?
2. Percutaneous cholecystostomy
What is the prognosis of acalculous cholecystitis?
High mortality with delayed tx 75%
Overall mortality of 30%
What is xanthogranulomatous cholecystitis?
Extravastion of bile into the gallbladder wall -->
Inflammatory reaction (fibroblasts and PMNs phagocytose biliary lipids in bile) -->
**gallstones present in all patients
What is the clinical presentation of xanthogranulomatous cholecystitis?
Hx suggestive of acute cholecystitis
Can mimic gallbladder cancer
High rate of complications--perforation, fistulas, abscess
Diagnosis of xanthogranulomatous cholecystitis
Abdominal ultrasound: hypoechoic nodules or bands in the gallbladder wall most characteristic
CT: intramural hypodense nodules
Tx for xanthogranulomatous cholecystitis
Preop cholangiogram to exclude bile duct cancer
What is porcelain gallbladder?
Chronic cholecystitis with intramural calcification of the gallbladder wall
Possible causes of porcelain gallbladder
1. Gallbladder wall injury from stone irritation
2. Bile stagnation and mucosal precipitation of calcium carbonate salts
3. Deposition of lime salts from chronic inflammation
Prevalence of porcelain gallbladder
Increased risk for:
Gallbladder cancer; incomplete calcificaiton more risky than complete
Possible clinical presentations of porcelain gallbladder
Biliary type pain
Diagnosis of porcelain gallbladder
Tx for porcelain gallbladder
Cholecystectomy for incomplete calc or symptomatic complete calc
What are the four classes of benign gallbladder polyps *found in 1.5-4.5% of patients undergoing gallbladder ultrasonography
What are cholesterol gallbladder polyps
Abnormal deposits of triglycerides, cholesterol precursors, and cholesterol esters into the gallbladder mucosa
What is a adenomyomatosis? (gallbladder polyp)
Overgrowth of the mucosa, thickening of the muscle wall, and intramural diverticula
**associated with cholelithiasis
M/F in adenomyomatosis? Cancer?
More common in women
No conclusive evidence of increased risk of gallbladder cancer
What are inflammatory gallbladder polyps?
Granulation and fibrous tissue with plasma cells and lymphocytes
What are adenomas (gallbladder polyps)?
Benign glandular tumors with the potential for malignancy
What is the relationship between likelihood of adenoma malignant transformation to size?
Larger has more risk of transforming
What is the clinical presentation of gallbladder polyps?
possible association of dyspepsia with cholestrolosis and adenomyomatosis
Diagnosis of gallbladder cancer
CT--most useful in gallbladder cancer
When should you do a cholecystectomy for gallbladder polyps?
1. With cholelithiasis
2. With primary sclerosing cholangitis
3. Biliary colic or pancreatitis
4. Polyps > 10 mm
What is acute (ascending) cholangitis
Fever, jaundice, and abdominal pain that develops as a result of stasis and infection in the biliary tract
What is the pathogenesis of acute cholangitis?
Bacteria enter from the small intestine or protal system
- disruption of the sphincter of oddi
- nidus for bacterial colonization
WHat are the more common bacteria involved in acute cholangitis?
E. coli (25-50%) > Klebsiella (15-20%) > Enterococcus (10-20%) > Enterobacter species (5-10%)
What is Charcot's triad?
**associated with acute cholangitis 50-75% of the time
What is Reynolds' pentad?
Confusion and hypotension witht Charcot's triad
**high morbidity and mortality, assoicated with acute cholangitis
How is acute cholangitis diagnosed?
Imaging: dilated biliary system, cholegocholithiasis
How is acute cholangitis treated?
Biliary drainage: ERCP, percutaneous transhepatic cholangiography (PTC), and surgery
What is biliary atresia?
Progressive, idiopathic, fibroobliterative disease of the extrahepatic biliary tree
How does biliary atresia present?
WIth biliary obstruction exclusively in the neonatal period
Infants born at full term with normal birth weight
Jaundice birth to 8 weeks with acholic stools and dark urine
What are the two types of biliary atresia?
1. Biliary atresia 70-85%
2. Biliary atresia splenic malformation (BASM) 10-15%
3. BIliary atresia in association with other congenital malformations
What are the findings in biliary atresia splenic malformation?
Asplenia or polysplenia
What is associated in 'biliary atresia in association with other congenital malfromations'?
Possible pathogenesis of biliary atresia
Genetic--possibly BASM subtype
Dx of biliary atresia
1. Abdominal ultrasound
2. Lier biopsy
3. Cholangiogram: intraoperative, PTC, endoscopic (ERCP)
Treatment of biliary atresia
Kasai procedure: surgical reconstruction of the extrahepatic biliary tract. Not curative, buys time for--
Liver transplantation (want to wait till weight is over 10 kg)
What are biliary cysts?
Cystic dilations that may occur singly or multiply thoughout the biliary tree
What are 70% of biliary cysts associated with?
Abnormal pancreaticobiliary junction (APBJ): pancreatic and bile duct join outside the duodenal wall
**associated with increased risk of gallbladder cancer independent of biliary cysts
Who gets biliary cysts
More common in asian populations
Equal numbers in children and adults
What are the types of biliary cysts?
Type I: 50-80%--> extrahepatic only
TYpe IV: 15-35%--> multiple cysts, extrahepatic +/- intrahepatic
Type V: 20% --> intrahepatic only, Caroli's disease
Pathogenesis of biliary cysts
Genetic or environmental predisposition
Associated with developmental anomalies
Congenital or acquired (from APBJ)
The majority of biliary cysts present before the age of
Infants with biliary cysts present with:
Patients over 2 with biliary cysts present with:
Jaundice, FTT, abdominal mass
Chronic intermittant abdominal pain, pancreatitis, intermittant jaundice, cholangitis
How are biliary cysts diagnosed?
Cholangiography: ERCP, PTC, intraoperative, MRCP
What do biliary cysts increase the risk for?
20-30 fold increased risk for cholangiocarcinoma
**most occur with types I and IV cysts
What is primary sclerosing cholangitis
Progressive inflammation, fibrosis, and stricture of the intrahepatic and extrahepatic bile ducts
What causes secondary sclerosing cholangitis?
Recurrent pyogenic cholangitis, choledocholithiasis, cholangitis, AIDS cholangiopathy
What does PSC have a strong association with?
Ulcerative colitis > crohns
**up to 90% of patients with PSC have UC
Who gets PSC?
Mean age of dx 40 yo **women dx later
What is the pathogenesis of PSC?
Genetic factors--CFTR mutations
?Ischemia duct injury
What are the clinical manifestations of PSC?
Elevated liver tests in a cholestatic pattern
What are the lab manifestations of PSC?
What are the subtypes of classic PSC?
Intrehepatic and extrahepatic
What is the presentation of small-duct PSC?
Involves small caliber bile ducts
Diagnosis of PSC?
CT, abdominal ultrasound
Liver biopsy (not needed if the cholangiogram is diagnostic, consider for small-duct PSC
**will see onion skin pattern around bile ducts
Complications of PSC
Cirrhosis and portal HTN due to hepatic fibrosis
Steatorrhea and fat-soluble vitamin malabsorption from decreased bile acids
Dominant biliary structures
Treatment of PSC
Medical therapy NOT recommended
ERCP for dominant extrahepatic strictures
Surgery: biliary reconstruction or liver transplant
What is AIDS cholangiopathy?
Biliary obstruction resulting from infection related strictures of the biliary tract
Classically cryptosporidium parvum
Who gets AIDS cholangiopathy?
Seen in AIDs patients with CD4 count <100/mm3
What is the presentation of AIDS cholangiopathy?
Diagnosis of AIDS cholangiopathy?
Elevation of cholestatic liver enzymes
Treatment of AIDS cholangiopathy
BIliary sphincterotomy during ERCP
Stending of dominant extrahepatic strictures
Sometimes ursodeoxycholic acid
What parasites are involved in biliary parasitosis?
Ascaris lumbricoides (roundworm)*
Echinococcus granulosus (tapeworm)*
Fasciola hepatica (sheep liver fluke)*
What is the roundworm that is found worldwide and inhabits human small intestine?
What will be seen on ultrasound with ascarid lumbricoides?
Long, linear, parallel echogenic structures without acoustic shadowing
Dx and tx of ascaris lumbricoides?
ERCP of dx and removal
Tx with anti-helminthic therapy
What is the tapeworm that has a dog as a host and is found in S. america, middle east, e. mediterranian, china, etc
What happens with echinococcus granulosus?
Rupture of hepatic cyst into biliary system causing jaundice and hepatomegaly
Tx of echinococcus granulosus related cyst?
Surgical resection or percutaneous injection of scolicidal agents as well as anti-helminth therapy
What is a liver fluke found in the far east and russie, with dog and cat reservoir?
What does clonorchis sinensis cause?
Chronic infection associated with cholangiocarcinoma
Dx and tx of clonorchis sinensis
ERCP for acute cholangitis
Tx with anti-helminth therapy
What is a liver fluke of cates in SE asia and central and eastern europe with a similar presentation as clonorchis sinensis?
What is a sheep liver fluke that causes human infection when eating raw veggies infected with metacercariae?
What happens upon fasciola hepatica infection?
Penetrate duodenal wall
Migrate across peritoneum
Enter the biliary system
Tx for Fasciola hepatica
ERCP for acute cholangitis
Tx with anti-helminthic therapy
What is recurrent pyogenic cholangitis?
Pigment stone formation in the intrahepatic biliary system resulting in intrahepatic stricturing and biliary obstruction with recurrent bouts or acute cholangitis
Who gets recurrent pyogenic cholangitis?
Patients from SE Asia
What causes recurrent pyogenic cholangitis?
What is the key clinical manifestation of recurrent pyogenic cholangitis?
Dx of recurrent pyogenic cholangitis
Tx of recurrent pyogenic cholangitis
Treat acute cholangitis
Stone clearance--ERCP, PTC, surgical
Consider ursodeoxycholic acid
Hepatic resection and reanastomosis