neoplasms of biliary tract & liver Flashcards
what is cholestasis
- systemic retention of bilirubin/ other solutes eliminated in bile, caused by impaired bile formation and bile flow
presentation of cholestasis
jaundice
- yellow discolouration of skin
icterus
- yellow discolouration of sclera
other symptoms
- pruritus, skin xanthomas, intestinal malabsorption / vit ADEK deficiencies
types of jaundice
Pre-hepatic:
* excess production of bilirubin e.g. haemolysis, ineffective erythropoiesis
Hepatic:
* reduced hepatic uptake e.g. drugs
* impaired bilirubin conjugation e.g. physiologic / neonatal jaundice, genetic deficiency, diffuse hepatocellular disease
* impaired bile flow e.g. AI cholangiopathies
Post-hepatic:
* impaired bile flow / large duct obstruction
what does post hepatic jaundice commonly present with
- hyperbilirubinaemia
unconjugated vs conjugated hyperbilirubinaemia
unconjugated
- before conjugation, pre hepatic
- excess cannot be excreted in urine
conjugated
- after conjugation in liver
- water soluble -> excess CAN be excreted in urine
Biliary tract diseases
- large bile duct obstruction
- primary heptolithiasis
- neonatal cholestasis & biliary atresia
- structural anomalies of biliary tree
what is large bile duct obstruction associated with (2)
- GALLSTONES
- MALIGNANCIES of biliary tree
others:
- inflammation of bile duct strictures
- porta hepatis lymphadenopathy
- bile duct malformations in children
large bile duct obstruction symptoms
- acute: reversible with correction of obstruction
- subtotal/ intermittent obstruction: increase risk of ascending cholangitis -> abscess & sepsis
- chronic: biliary cirrhosis
what is primary hepatolithiasis
- intrahepatic biliary stone formation
- lead to repeated bouts of ascending cholangitis, progressive inflammatory destruction -> collapse & scarring of hepatic parenchyma
what does primary hepatolithiasis predispose pt to
- Biliary Intraepithelial Neoplasia (BilIN) and cholangiocarcinoma
neonatal cholestasis
- prolonged conjugated hyperbilirubinaemia in neonate BEYOND 14 DAYS after birth
most common cause of neonatal cholestasis
- cholangiopathies (extrahepatic biliary atresia)
what is extrahepatic biliary atresia
- complete/ partial obstruction of lumen of extrahepatic biliary tree within FIRST 3 MONTHS of life -> can extend to involve intrahepatic ducts
types of structural anomaly of biliary tree
- choledochal cyst
- fibropolycystic disease
choledochal cyst
- developmental malformation of biliary tree, usually CBD -> predispose to stones, stenosis, strictures, pancreatitis and risk of bile duct cancer
fibropolycystic disease
- Heterogeneous group of lesions in which the primary abnormalities are congenital malformations of the biliary tree (ductal plate malformations)
diseases of gallbladder
- cholelithiasis
- cholecystitis (acute & chronic)
clinical features of cholelithiasis
- usually asymptomatic
- RUQ/ epigastric pain ->