HPB Flashcards
at what level of billirubin does jaunice occur?
above 50micromol/L
what is billirubin the breakdown product of?
red blood cells -> haem -> billirubin
how is billirubin excreted?
billirubin is conjugated by the liver so it becomes water soluble, then it is excreted via the vile into the GI tract through the duodenum. it is egested in the faeces as stercobilin and urobilinogen but 10% of urobilinogen is reabsorbed into blood and excreted via kidneys
what are the three types of jaundice?
pre hepatic - excess haemolysis…forms unconjugated hyperbillirubinaemia (liver can not cope with quantity to conjugate)
intrahepatic - dysfunction of hepatic cells and can become cirrhotic and compresses billary tree …both conjugated and unconjugated
post hepatic - obstruction of billary drainage …already conjugated by liver
give 3 causes of pre hepatic jaundice
haemolytic anaemia
gilbert’s syndrome
criggler-naijjar syndrome
give 3 causes of intrahepatic jaundice
alcoholic liver disease
hepatitis
primary sclerosing cholangitis
hepatocellular carcinoma
give 3 causes of post hepatic jaundice
intra luminal - gallstones
mural - strictures
extra mural - pancreatic cancerqq
how does post hepatic jaundice clinically present?
conjugated billirubin can be excreted via urine as water soluble —-dark urine (can also be present in intra hepatic)
and due tn obstruction, reduced stercobilin entering Gi tract which normally colours the stools - pale stools
what investigations are required for a patient presenting with jaundice?
LFTS
coag screen - PT is marker of liver synthesis function
FBC - anaemia, raised MCV and thrombocytopenia - liver disease
UandE’s
specialist markers
what does each LFT mean?
billirubin - degree of jaundice
albumin - liver synthesis function
AST and ALT - if ratio >2 then alcoholic liver disease, if around 1 then viral hepatitis
ALP - billary obstruction, bone disease, pregnancy, malignancies
gamma GT - more specific for billary obstruction
what are the causes of acute liver injury and what markers would test?
hep A, B, C, E
CMV
EBV
-paracetamol lieve, caeruloplasmin, antinuclear antibody and IgG
what are the causes of chronic liver injury and what markers would test?
hep B and C
- caeruloplasmin, ferritin and transferrin saturation, tissue transglutaminase antibody, alpha 1 antitrypsin, autoantibodies (AMA, anti SMA, ANA - autoimmune)
what investigations are required for a patient with jaundice?
USS abdomen
MRCP - visualise bil
liver biopsy
how is a gallstone managed
removal through ERCP or stenting of common bile duct
how is hyperbillirubinaemia symptomatically treated
pruritus - cholestyramine to increase billary drainage
- anti histamines
what complications of hyperbillirubinaemia need to monitored for
coagulopathy - with vit K or fresh frozen plasma
hypoglyceamia - give glucose or dextrose
decompensating chronic liver disease (hepatic encephalopathy) - laxatives +/- neomycin to reduce ammonia producing bacteria
why do gallstones form?
formed from cholesterol, phospholipids, and bile pigments (products of haemoglobin metabolism). It is stored in the gallbladder, before passing into the duodenum upon gallbladder stimulation..gallstones form as a result of supersaturation of bile
what are the types of gallstones?
Cholesterol stones – composed purely of cholesterol, from excess cholesterol production
There is a well recognised link between poor diet, obesity, and cholesterol stones
Pigment stones – composed purely of bile pigments, from excess bile pigments production
Commonly seen in those with known haemolytic anaemia
Mixed stones – comprised of both cholesterol and bile pigments
what are the risk fx for gallstones?
5 F’S - fat, female, fertile, forty, family hx
pregnancy, COCP, haemolytic anaemia, malabsorption
what are the clinical fx of gallstones?
billary colic - impacted gallstone, no inflammation - sudden, dull, colicky pain radiates RUQ to epigastrium, precipitated by fatty foods
N+V
acute cholecystitis - constant pain in RUQ or epigastrium, signs of inflammation, tender in RUQ, positive murphy’s sign, guarding, sepsis
what is murphy’s sign?
Whilst applying pressure in the RUQ, ask the patient to inspire. Murphy’s sign is positive when there is a halt in inspiration due to pain, indicating an inflamed gallbladder
what are the ddx for gallstones?
gastro-oesophageal reflux disease, peptic ulcer disease, acute pancreatitis, or inflammatory bowel disease.
which investigations are required for gallstones?
FBC and CRP – assess for the presence of any inflammatory response, which will be raised in cholecystitis
LFTs – biliary colic and acute cholecystitis are likely to show a raised ALP (indicating ductal occlusion), yet ALT and bilirubin should remain within normal limits (unless a Mirizzi syndrome, discussed below)
Amylase (or lipase) – to check for any evidence of pancreatitis
A urinalysis, including a pregnancy test
imaging -trans-abdo USS - The presence of gallstones or sludge (the start of gallstone formation)
Gallbladder wall thickness (if thick walled, then inflammation is likely)
Bile duct dilatation (indicates a possible stone in the distal bile ducts)
MRCP if not found on USS
how is billary colic managed?
analgesia
low fat diet, weight loss, increased exercise
high chance of recurrence and may require elective laprascopic cholectomy within 6 weeks of first presentation
how is acute cholecystitis managed?
IV abx
analgesia
antiemetics
laprascopic cholecystectomy within 1 week but should be within 72 hrs
if have pain post cholecytsectomy may be a retained CBD
what is a complication of gallstones?
mirizzi syndrome - stone located in hartmanns pouch (an outpouching of gallbladder wall at junction with the cystic duct or in cystic duct itself causing compression of CHD..obstructive jaundice…requires laprascopic cholecystectomy
gallbaldder empyema = gallbladder full of pus..laprascopic cholecystectomy
chronic cholecystitis - recurrent or unreated cholecystitis - CT…elective cholecytectomy
bouveret’s syndrome - inflammation of gallbladder can cause a fistula from wall to small bowel..cholecystoduodenal fistula sp gallstones passes and impacts in duodenum
gallstone ileus - ^ stone impacts at terminal ileum..SBO
define ascending cholangitis
biliary outflow obstruction* and biliary infection. During an obstruction, stasis of fluid combined with elevated intraluminal pressure allows the bacterial colonisation of the biliary tree to become pathological.
what are the causes of ascending cholangitis?
gallstones, ERCP, cholangiocarcinoma
pancreatitis, primary sclerosing cholangitis
usually e.coli, klebsiella species, enterococcus
what are the clinical fx of ascending cholangitis?
charcot’s - RUQ, fever, jaundice
reynolds pentad - jaundice, fever, RUQ, hypotension and confusion
pruritus, pale stools, dark urine
PMH - gallstones, recent ERCP, or previous cholangitis
MHX- COCP, fibrates, lipid rich diet
pyrexia, rigors, confusion, hypotension, tachycardia
which investigations are required for ascending cholangitis?
FBC - leukocytosis, LFT’s - raised ALP nad gamma Gt and billirubin
blood cultures
USS - bile duct dilation >6mm, or gallstones
ERCP
how is ascending cholangitis managed?
sepsis 0 IV access for fluids, bloods, blood cultures, broad spectum IV abx
endoscopic biliary decompression
ERCP - with or without sphincterotomy and stenting
what are the complications of ERCP?
pancreatitis, bleeding, perforation
define cholangiocarcinoma
malignancy of billiary tree
high incidence in south east asia - chronic endemic parasitic infection from liver flukes can cause
usually adenocarcinomas then rest are squamous cell, sarcomas, lymphomas
where is the most common site for a bile duct tumour?
bifurcation of right and left hepatic ducts - klatsin tumour
what are the risk fx for cholangiocarcinoma?
Primary sclerosing cholangitis (lifetime risk of 10-20%)
Ulcerative colitis
Infective (Liver flukes, HIV, hepatitis virus)
Toxins (Chemicals in rubber and aircraft industry)
Congenital (Caroli’s disease, choledochal cyst)
Alcohol excess
Diabetes mellitus
what are the clinical fx of cholangiocarcinoma?
asx
jaundice
pruritus
steatorrhoea
non speciifc abdo pain
dark urine
weifht loss
lethargy
cachexia
define courvoisier’s law
in the presence of jaundice and an enlarged or palpable gallbladder, malignancy of the biliary tree or pancreas should be strongly suspected as the cause is unlikely to be gallstones
which investigations are required for cholangiocarcinoma?
LFT - billirubin, ALP, gamma GT
tumour markers - CEA, CA19-9
Ultrasound scanning may initially show dilated biliary ducts, however CT imaging or MRI imaging is required for diagnosis.
Endoscopic retrograde cholangiopancreatography (ERCP) is required to obtain a tissue sample, which will delineate the exact location of the lesion and take brushings of the area.
Staging of the disease is best done via CT imaging. Angiography may be used in pre-operative planning to image the hepatic arteries and portal vein.
how is cholangiocarcinoma managed?
complete surgical resection
if intrahepatic or klatsin tumours - partial hepatectomy or reconstruction of biliary tree
if distal common duct tumour - pancreaticiduodenectomy
radiotherapy
palliative - stenting, surgical bypass, chemo and radio
what is the prognosis of cholangiocarcinomas ?
12-18months from diagnosis as mostly unresectable disease
5 yr survival rates after surgery - 10-40%
define simple liver cysts
fluid-filled epithelial-lined sacs within the liver, most commonly occurring in the right lobe. They are relatively common, with a prevalence of 2.5-18%, increasing in incidence with age
due to congenitally malformed bile duct cells, failing to connect to the extrahepatic ducts, which leads to a local dilatation filled with a bile-like fluid, of varying size.
what are the clinical fx of simple liver cysts?
normally asx
around 10-15% will have abdo pain, nausea, early satiety
which investigations are required for simple liver cysts?
LFTs normal but may have raised gamma GT
tumour markers - CEA, CA19-9 elevated
USS - well defined, thin walled, oval with no septations
how are simple liver cysts managed?
mostly no intervention, if >4cm follow up USS, if size unchanged for 2-3 yrs, no further scans until asx
if asx - USS guided aspiration or laprascopic deroofing
define polycystic liver disease
presence of ≥20 cysts within the liver parenchyma, each of which are ≥1cm in size.
what are the genetic causes of polycystic liver disease?
utosomal dominant polycystic kidney disease (ADPKD)
Caused by mutations in the PKD1 (chromosome 16) and PKD2 (chromosome 4) genes, whereby 10-60% of patients will also develop have liver cysts (as the most common extra-renal manifestation of ADPKD)
Autosomal dominant polycystic liver disease (ADPLD)
Caused by mutations in the PRKCSH (chromosome 19) or SEC63 (chromosome 6) genes; patients with this genotype will not have any renal involvement.
what are the clinical fx of polycystic liver disease?
asx
may have abdo pain as cysts grow, and hepatomegaly
can cause liver cirrhosis and portal hypertension
which investigations are required for polycystic liver disease?
normal LFT’s
USS
how are polycstic liver cysts managed?
asx left alone
short term benefit of somatostatin analogues
surgery indicated if -(1) intractable symptoms (2) inability to rule out malignancy on imaging alone (3) prevention of malignancy.
US guided aspiration of laprascopic deroofing
define cystic neoplasms
rare
common subtype are cystadenomas (non-invasive mucinous cystic neoplasms). These are premalignant lesions, developing as a result of abnormal proliferation of biliary epithelium and can undergo malignant transformation into cystadenocarcinomas in around 10% of case
what are the clinical fx of cystic neoplasms?
asx
if do = abdo pain and anorexia
which investigations are required for cystic neoplasms?
LFTS normal
ALP, CEA, CA19-9 can be elevated
CT with contrast
aspiration or biopsy should be avoided as can lead to peritoneal seeding
liver lobe resection
