Gallbladder and Biliary tract disorders Flashcards Preview

GI > Gallbladder and Biliary tract disorders > Flashcards

Flashcards in Gallbladder and Biliary tract disorders Deck (60)

What stimulates the gallbladder to release bile into the duodenum? Fxn of the bile?

- hormone cholecystokinin (CCK)
- CCK released after food is ingested causes the sphincter of oddi to contract and relax which sends a bolus of bile into the duodenum
- bile is used to emulsify fats and assist with the excretion of cholesterol


What is cholelithiasis?

- formation of gallstones which are solid concentrations of varying quantities of cholesterol, Ca2+, and bilirubin - which usually form in GB, but may form in bile ducts (choledocholithiasis)
- supersaturation of bile with cholesterol and GB hypomotility leads to formation of cholesterol stones


Most common digestive disease?

- gallstone disease - also most costly
- 10-15% of all Americans have gallstones, most are asx
- over 500,000 cholecystectomies are done in US each year due to stones


Diff types of gallstones?

- cholesterol stones (90%)
- pigmented (10%)
black stones: contain Ca bilirubinate, assoc with cirrhosis and hemolysis
brown stones: a/q biliary tract stasis and infection


The 4 F's to remember as RFs?

- fat
- fertile
- female
- forty


Major RFs for development of gallstones?

- age
- female
- genetic: native americans, chileans
- pregnancy
- obesity
- rapid wt loss
- cirrhossi
- hemolytic anemias
- hyperTG
- meds: ceftriaxones, estrogen, OCP
- gallbladder stasis: DM, TPN
- reduced physical activity (in men)


Signs and sxs of cholelithiasis?

- most pts with stones are asx, but 20% will become sx during up to 15 yrs of f/u
- cardinal sx: biliary colic, steady RUQ pain radiates to back and R shoulder, may be accompanied by nausea. Pain may be brought on after ingestion of fatty foods
- if uncomplicated may have normal PE and normal labs
- Can see stones on US (US is dx imaging of choice for RUQ pain)


Tx of cholelithiasis?

- 60-80% asx: observation
- sx: cholecystectomy
- consider prophylactic cholecystectomy:
porcelain gallbladder
sickle cell disease
heriditary spherocytosis (not usually recommended)
gastric bypass - usually recommended


Complications of gallstones?

- gallstone ileus: stone erodes through GB wall and develops a cholecystoenteric fistula leading to obstruction of narrowest segemnent of bowel casuing ileus
- gallstone pancreatitis
- acute cholecystitis: 10-20% pts with sx gallstones:
GB gangrene
GB perf
GB empyema (pus in GB)
emphysematous cholecystitis ( a/w GB vascular compromise, stones, impaired immune system, infection with gas - forming organisms - Clostridium, E. coli, klebsiella)
- choledocholithiasis: 8-15% of pts with sx gallstones
- cholangitis
- bile duct injuries


Protective factors - prevent GB stones?

- statins
- ascorbic acid
- coffee -caffeinated
- veggie protein


What is choledocholithiasis? What labs will be elevated, sxs?

- gallstones within common bile duct
- may be asx in up to 30% of pts
- if sx typically present with RUQ or epigastric pain, N/V
- LFTs (ALT, AST) are elevated, bili and AP may be elevated
- complicated choledocholithiasis: acute cholangitis, acute pancreatitis, hepatic abscesses


Imaging for choledocholithiasis?

- transabdominal U/S
- abdominal CT
- intraoperative cholangiography or US


What is gold std for dx of CBD stones and sphincter of Oddi dysfxn?

- advantage: therapeutic option when CBD stone ID'd
stone retrieval and sphincterotomy
- disadvantage: complications - pancreatitis, cholangitis, perf of duodenum or bile duct, bleeding


Advantages and disadvantages of using MRCP? Indications?

- detects choledocholithiasis, neoplasms, strictures, biliary dilations
- sensitivity of 81-100%, specificity of 92-100% of choledocholithiasis
- minimally invasive - avoid invasive procedure in 50% of pts

- can't sample bile, test cytology, remove stone
CIs: pacemaker, implants, prosthetic valves, metal in the eye

indications: if cholangitis isn't severe, and risk of ERCP high - MRCP useful


Tx of choledocholithiasis?

- remove the stone:
ERCP (#1 tx)
lithotripsy (may not work)
laparoscopic CBD exploration (used if ERCP fails)


What is acute cholecystitis?

- syndrome of RUQ pain, fever, and leukocytosis assoc with gallbladder inflammation usually caused by cystic duct obstuction
- 90% caused by impacted gallstone
- 10% caused by bile stasis or acalculus cholecystitis: occurs in critically ill pts, and assoc with high morbidity and mortality


H&P findings for acute cholecystitis?

- RUQ or epigastric pain may have radiation to right shoulder, often occurs after fatty meal.
- anorexia, N/V, fever
- positive murphy's sign: pain in RUQ on inspiration
- palpable enlarged gallbladder: 1/3 pts
- jaundice in about 10%


Dx - labs of acute cholecystitis?

- LFTs and bili elevated
- CBC: leukocytosis with left shift
- CRP elevated more than 3 mg/dl
- US: stones or sludge, pericholecystic fluid, distended GB, thickened GB wall
- HIDA scan: failure of GB filling


Imaging modality of choice for gallbladder issue?

- U/S
- fast, real-time, non-invasive, doesn't utilize ionizing radiation
- 95% sensitivity for detection of cholelithiasis. Dx based on visualization of mobile, hyperechoic, intraluminal mass with acoustic shadowing
- greater than 90% sensitivity for detection of acute cholecystitis, dx based on presence of cholelithiasis, gallbladder wall thickening, pericholeecystic fluid and sonographic murphy sing
- limited by skill of operator, and pt's body habitus


Indications for a HIDA scan?

- fxnl assessment of hepatobiliary system
- integrity of hepatobiliary tree:
eval of susp acute cholecystitis, susp chronic biliary tract disorders, eval of common bile duct obstruction, detection of bile extravasation, eval of congenital abnormalities of biliary tree


Normal uptake time of HIDA? Other abnormal findings?

- inject HIDA by IV, taken up by hepatocytes and excreted into bile
- uptake by liver, GB, CBD, duodenum w/in 1 hr: this is normal
- slow uptake: hepatic parenchymal disease
- filling of GB/CBD w/ delayed or absent filling of CBD and duodenum - cystic duct obstruction and acute cholecystitis (95% sensitive and specific), give CCK to calc GB ejection fraction and can reproduce sxs


Requirements for HIDA scan?

- pt prep: fasted for 2-4 hrs, otherwise delayed or non-visualization
- fasted for over 24 hours or on TPN, a false positive may occur

- need to know hx of previous surgeries esp biliary and GI
- time of most recent meal
- current meds: opioid compounds - wait 4 hours to do study
- bili and liver enzyme levels
- results of US


Tx of acute cholecystitis?

- admit to hospital - supportive care, NPO, IV fluids, analgesia, close monitoring of vitals and urinary output
- abx: cipro or levo, and flagyl or cefuroxime
- early laparoscopic cholecystectomy preferred tx - eventually will need surgery
- emergency surgery for advanced disease or sepsis
- if fail medical managment and poor surgical candidate - percutaneous cholecystostomy tube + abx


Complications of acute cholecystitis?

- emphysematous cholecystitis
- gangrenous cholecystitis
- pericholecystic abscess formation
- sepsis
- peritonitis
- ascending cholangitis
- cholecystoenteric fistula
- perf: elderly, diabetic, recurrent cholecystitis
- 10% present with complications
- look for: fever, shaking chills, high WBC, increased abdominal pain, persistent sxs


When does chronic cholecystitis occur?

- after repeated episodes of mild attacks
- usually due to presence of stones
- sxs from transient obstruction of cystic duct by gallstones
- mild histologic inflammation can lead to shrinking, scarring, thickened walls, mucosal atrophy, and fibrosis of GB wall


Signs and sxs of chronic cholecystitis?

- pain: RUQ that comes and goes, may be referred to R shoulder or scapula, subsides in 30 min. Usually precipitated by fatty meal
- may have N/V
- usually no fever, WBC normal
- gallbladder not palpable
- trigger: ingestion of fatty foods
- episodes variable intervals: days to years


Tx of chronic cholecystitis?

- by laparotomy or laparoscopy
- look for concomitant choledocholithiasis
- prevents future complications: acute cholecystitis, choledocholithiasis, cholangitis


What is a porcelain gallbladder?

- extensive Ca2+ encrustation of gallbladder wall - blue discoloration and brittle consistency
- thought to be uncommon manifestation of chronic cholecystitis
- pts with this are usually asx, and condition is found incidentally on plain abdominal radiographs, sonograms, or CT


Porcelain gallbladder increases risk of what? Tx?

- GB adenocarcinoma
- surgery shouldn't be delayed even if the pt is asx, b/c assoc of porcelain gb with carcinoma - reported incidence highly variable (5-60%)


What is acute cholangitis? Most impt predisposing factor?

- inflammation or infection of the bile duct system - caused primarily by bacterial infection. Organisms typically ascend from duodenum. hematogenous spread from portal vein is rare source of infection
- most impt predisposing factor for acute cholangitis is biliary obstruction and stasis secondary to biliary calculi (CBD stone) or benign stricture


Charcot's triad? Reynold's pentad in cholangitis?

- Charcot's triad: 50-70%
RUQ pain
fever and chills
- can start mild then quickly progress to severe and include sepsis, and shock
- in severe cases, bacteremia and sepsis may lead to hypotension and altered mental status ( reynold's pentad)


Septic cholangitis prognosis?

- 50% mortality rate, usually life threatening sepsis, bacterial infection superimposed on obstruction of biliary tree most commonly from a gallstone but may assoc with neoplasm or stricture


Dx cholangitis?

- labs: leukocytosis, elev LFTs, elev CRP, blood cultures usually gram - but may be gram + or anaerobes. ABGs in severe cases will be metabolic acidosis, may be in renal failure with elevated BUN and creatinine
- US: dilated bile duct, CBD stones
- ERCP: bile duct stone or obstruction or stricture, also is therapeutic as it can extract stone


Tx of cholangitis?

- hospitalize, tx infection with IV abx
- remove stones - ERCP
- blood cultures should be performed in all pts in whom cholangitis is suspected
- biliary drainage: 80% of pts with acute cholangitis will respond to conservative management and abx therapy. Biliary drainage can then be performed on elective basis. In 15-20% of cases, cholangitis fails to settle over the first 24 hrs with conservative therapy alone, requiring urgent biliary decompression


What is primary sclerosing cholangitis? Assoc with what other GI problems?

- chronic inflammatory cholestatic disease: characterized by diffuse inflammation of biliary tract
- etiology is unknown
- fibrosis and strictures develop
- progressive destruction of bile ducts may progress to cirrhosis, end stage liver disease
- more common in men: 20-40
- closely assoc with ulcerative colitis
- increased risk for cholangiocarcinoma, gallbladder CA, colon CA, pts with cirrhosis, and HCC


Presentation of PSC?

- progressive, obstructive jaundice
- malaise, fatigue, pruritus, anorexia, dyspepsia
- 44% asx but most will develop sxs over time
- late presentation will develop sxs of cirrhosis and portal HTN
- elev AP (3-10x normal), ALT, AST (2-3x normal)


How do you dx PSC?

- difficult dx to make, but is demonstrated by characteristic multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts on ERCP
- ANCA: (anti-neutrophil cytoplasmic abs): found in 70%. A number of other autoabs may also be present, but of uncertain clinical significance
- MRCP: may be an alt to ERCP will see multifocal stricturing and dilation of bile ducts as well, less invasive


Tx of PSC?

- immunosuppressive and anti-inflammatory agents and direct injection of steroids into biliary tree - INEFFECTIVE!
- balloon dilation or stenting can improve clinical, biochemical and cholangiographic appearances
- with balloon dilation there are some reports of survival advantages and delay to liver transplantation: many strategies tried but only liver transplantation shown to improve survival


What is primary biliary cirrhosis (PBC)? Peak incidence? What can it lead to?

- slowly progressive autoimmune liver disease
- 90% females
- peak incidence in 40s
- portal inflammation and autoimmune destruction of intrahepatic bile ducts
- leads to cirrhosis and liver failure
- 90-95% have antimitochondrial ab (AMA)


Clinical features of PBC?

- 50% asx at dx
- fatigue and pruritus most common sxs - 20%
- skin hyperpigmentation due to melanin deposition
- rheumatic sxs, musculoskeletal complaints, frequently due to an inflammatory arthropathy
- hyperlipidemia, hypothyroidism, osteopenia, autoimmune diseases
- portal HTN, liver failure


PE findings in PBC?

- dry hyperpigmented skin, excoriations (from scratching so much)
- xanthelasmas (yellow plaques near the innner canthus of the eyelid)
- hepatomegaly 70%
- jaundice, spider nevi, temporal and proximal limb muscle wasting, ascites, and edema are all late manifestations of disease and imply cirrhosis


How do you dx PBC?

- need 2 of 3 criteria:
positive AMA
abnorm LFT - typically AP and GGTP very high
compatible bx shows destruction of interlobular bile ducts


Tx of PBC?

Ursodeoxycholic acid (UDCA - ursodiol):
- reduces bili, LFTs, cholesterol, and IgM
- delays fibrosis and varices
- doesn't work in advanced disease
- reduced risk of need for liver transplantation and death over 4 yrs

- other drugs: if there is sig inflammatory component - colchicine
- end stage liver disease: liver transplant


Carcinoma of biliary tract? Known RFs?

- cholangiocarcinoma often in an advanced stage by the time sxs develop, which may limit tx options

known RFs for cholangiocarcinoma include:
- congenital liver malformations
- infection with parasitic flukes
- exposure to thorotrast (thorium dioxide) - chemicaly formerly used in medical imaging


Dx testing for a cholangiocarcinoma?

- US, CT, MRI:
both US and CT can detect the actual tumor and extent to which it has spread. Dilation of gallbladder or bile ducts can be seen
- CT adds the ability to detect enlarged lymph nodes throughout the abdominal cavity. MRI has also been used to determine the involvement of bile ducts and blood vessels
- cholangiography via ERCP: allows access to biliary tree in cases wehre ERCP has been unsuccessful


Tx of cholangiocarcinoma?

- majority present as inoperable disease due to its advanced stage in which pts tx generally with palliative chemo, with or w/o radiation
- cholangiocarcinoma considered to be incurable and rapidly lethal disease unless all of its tumors cna be fully resected
- pts may receive adjunctive chemo or radiation therapy after surgery to improve the chances of cure


Prognosis of cholangiocarcinoma?

- surgical resection offers only potential chance of a cure. For non-resectable cases, 5 yr survival rate is 0%, disease is inoperable b/c of distal lymph nodes mets, and less than 5% in general
- overall median duration of survival is less than 6 months in inoperable, untx, otherwise healthy pts with tumors involving the liver by way of the intrahepatic bile ducts and hepatic portal vein


Gallbladder cancer: how common? Highest incidence?

- GBC is uncommon but highly fatal malignancy
- epidemiology: less than 5000 cases/yr in US
- women more than men
- high incidence in South America (Chile) maybe due to high prevalence of gallstones and/or salmonella infection
- majority are found incidentally in pts undergoing expoloration for cholelithiasis


RFs for GBC?

- gallstones
- GB polyps
- chronic salmonella infection
- abnorm pancreatobiliary duct jxn
- porcelain gallbladder
- diabetes
- obesity


Presentation of GBC? Imaging?

- presentation is non-specific, usually incidental finding on imaging or intraoperatively

3 presentations:
-GB CA discovered during or after lap/open cholecystectomy for assumed benign disease
- GB CA suspected after dx eval and found on imaging
- GB CA in advanced stage at presentation


Pathology of GBC?

- adenocarcinoma 80-90%
- small cell neuroendocrine tumors 7%
- adenosquamous or SCC 6%
- lymphoma, sarcoma 5%


Tx of GBC?

- only 10-30% resectable at time of dx
- simple cholecystectomy
- radical cholecystectomy
- radical cholecystectomy w/ anatomic liver resection
- radical cholecystectomy with whipple procedure (pancreaticoduodenectomy)


Tumors of ampulla of vater? How common? Avg age and most common tumor?

- hepatopancreatic duct tumor
- cancer of intestinal mucosa occurs more commonly near the ampulla than at any other site in small intestine
- both benign and malignant tumors of ampulla of vater occur
- avg age at dx of ampullary carcinomas is 60-70
- adenomas and adenocarcinomas most common malignant tumors


Clinical features of hepatopancreatic duct tumors? Imaging used?

- present most commonly with obstructive jaundice (80%)
- wt loss, anorexia, fatigue, abdominal pain
- occult gi bleeding is common (1/3 of pts)
- positive fecal occult blood test result
- imaging:
US or CT: CT more sensitive, but US more readily available
EUS (endoscopic US)


Tx/prognosis of hepatopancreatic duct tumors?

- surgery: whipple is considered standard approach for ampullary cancer
- survival in pts with unresectable ampullary adenocarcinoma is 5-9 months
- overall 5 year survival rate for pts with resected ampullary cancer ranges from 25-55%


Normal phys of bilirubin?

- bili formed by breakddown of heme in hemoglobin
- unconjugated bili transported to liver - bound to albumin because insoluble in water
- next transported into hepatocyte and conjugated with glucuronic acid and is now water soluble
- secreted into the bile
- in the ileum and colon: converted to urobilinogen - 10-20% reabsorbed into portal circulation and re-excreted into bile or into urine by kidneys


Causes of hyperbilirubinemia - increased production of bili?

hemolytic disease:
- isoimmune mediated hemolysis (ABO or Rh incompatibility)
- hereditary spherocytosis, elliptocytosis
- erythrocyte enzymatic defects (G6PD) deficiency, pyruvate kinase deficiecny and congenital erythropoietic porphyria
- sepsis
- polycythemia
- sequestration of blood within a closed space, which occurs in cephalohematoma


Causes of hyperbilirubinemia - decreased clearance of bili?

- inherited defects in UGT1A1 gene, which catalyzes the conjugation of bili with glucuronic acid, decrease in bili conjugation, this reduces hepatic bili clearance and increases total bili levels (known as gilbert's syndrome)


What is Gilbert's syndrome? Presentation? Cause?

- most common hereditary cause of increased bili (5-10% of pop)
- produces an elevated level of unconjugated bili in bloodstream, but normally causes no serious consequences
- mild jaundice may appear under conditions of exertion, stress, fasting and infections, but otherwise pts are usually asx
- caused by 70-80% reduction in glucuronidation activity of UGT1A1 enzyme (also responsible for some of liver's ability to detoxify certain drugs)


Dx and tx of gilbert's syndrome?

- lab work will show predominantly elevated unconjugated bili while conjugated bili is usually WNL and all other LFTs will be normal
- no specific therapy is reqd
- most impt aspect of care is recognition of disorder and its benign nature
- its mode of inheritance should also be discussed to prevent unnecessary testing in family members