Biliary Tree Flashcards
What is primary biliary cholangitis?
An autoimmune condition where T cells attack small bile ducts in the liver causing bile to leak into the interstitial space and cause chronic inflammation of bile ducts
This can lead to cirrhosis
Who presents with primary biliary cholangitis?
Women in 40-50s
Patients present late
What are diseases associated with primary biliary cholangitis?
Sjogren’s
Rheumatoid arthritis
Hypothyroidism
Other autoimmune conditions
What are the signs and symptoms of primary biliary cholangitis?
Jaundice Xanthoma (cholesterol in skin) Xanthelasma (cholesterol around eyes) Pruritis (itch) Joint pain
What are the investigations for primary biliary cholangitis?
Anti-Mt antibodies (AMA) High cholesterol High IgM LFT abnormalities USS (rule out structural abnormality)
Why is there hypercholesterolaemia in primary biliary cholangitis?
Cholesterol leaks out of bile and deposits in skin
What are the complications of primary biliary cholangitis?
Osteoporosis (bile doesn’t function properly and calcium is not absorbed)
Portal hypertension
Ascites (from liver damage)
Fat soluble vitamin deficiencies (ADEK) (also not absorbed)
What is the management for primary biliary cholangitis?
Lifestyle (smoking, weight, alcohol, NSAIDs) Ursodeoxycholic acid Obeticholic acid Vit ADEK supplements Antipruritics (cholestryamine) Biphosphates Liver transplant
What is secondary biliary cholangitis?
Small bile ducts in the liver are damaged due to obstruction, no AMA detected in blood
What is primary sclerosing cholangitis?
Autoimmune condition that causes progressive inflammation and fibrosis of the bile duct
How does primary sclerosing cholangitis progress to cirrhosis?
- Inflammation of bile duct
- Fibrosis
- Cholestasis
- Liver cirrhosis
Who presents with primary sclerosing cholangitis?
Middle aged men
Associated with UC
Patients have ANCA antibodies
What are the symptoms of primary sclerosing cholangitis?
Can be asymptomatic
Pruritis (itch)
Jaundice
RUQ pain
How does primary sclerosing cholangitis present when it is acute?
Hepatitis like infection with fever, jaundice and cholangitis
What investigations are done for primary sclerosing cholangitis?
ANCA antibodies LFTs US MRCP (diagnosis - beaded appearance) ERCP (biopsy - onion skin)
What are the characteristic features of primary sclerosing cholangitis seen on an MRCP and ERCP?
MRCP - beaded appearance
ERCP - onion skin on biopsy
What looks similar to primary sclerosing cholangitis on MRCP and is important to rule out?
Cholangiocarcinoma
What is the management for primary sclerosing cholangitis?
Liver transplant definitive
Manage symptoms
Diet and nutrition
Endoscopic interventions (stenting, balloon dilation)
What can primary sclerosing cholangitis progress to?
Secondary biliary cholangitis Gallstones Strictures Duct cannulation Cholangiocarcinoma
How and where is bile secreted?
When chyme enters the duodenum, CCK stimulates the gallbladder to contract and release bile
Bile secreted via sphincter of Oddi at D2
What makes up the majority of bile?
Bile salts and acids
What are the main types of gallstone and which makes up the majority of gallstones, and what are they associated with?
Cholesterol (majority)
Pigmented (bilirubin) black stones (associated with haemolytic anaemia)
Brown stones (associated with parasitic infection)
What is cholelithiasis?
The presence of gallstones
What is cholecystolithiasis?
Gallstones in the gallbladder
What is choledocholithiasis?
Gallstones in the bile duct
What are the factors precipitating cholesterol gallstone formation?
Too much cholesterol
Not enough salt/acid/phospholipid
Gallbladder stasis
What are the risk factors for cholesterol stones?
Obesity Rapid weight loss High fat, low fibre diet Family history Female 40s Hormone replacement therapy (5 Fs)
Are most people with gallstones asymptomatic?
Yes
What is biliary colic?
Temporary obstruction of the CBD or cystic duct by a gallstone
How does biliary colic present?
RUQ/epigastric colicky pain that might radiate to right shoulder
Pain may last 2-6 hours and crescendos then plats
Associated with indigestion and over-indulgence with high fat foods
Onset mid-evening or early morning
Nausea and vomiting can occur
What causes the pain in biliary colic?
Peristaltic contraction around the stone
What investigation is done for biliary colic?
Diagnosis is clinical but can do USS
What is the management for biliary colic?
Lifestyle
If mild-moderate pain - paracetamol/NSAIDs
iIf severe pain - diclofenac IM
What is cholecystitis?
Obstruction of the cystic duct by gallstone causes inflammation of the gallbladder
How does cholecystitis present?
RUQ/epigastric pain and tenderness
Fever
Murphey’s sign (pain on deep breath when examiner’s fingers are over RUQ)
Obstructive jaundice
What is obstructive jaundice?
Jaundice of the skin, sclera
Pale stools
Dark urine
What are the complications of cholecystitis?
Perforation
Fistula formation
Peritonitis
Sepsis
What are the investigations for cholecystitis?
High ALP or GGT
Abdominal USS (detects stones and thickened GB)
MRCP
ERCP (diagnosis and can treat)
What is the management for cholecystitis?
Supportive measures (fluids, analgesia, IV antibiotics while waiting for stone to dislodge itself)
ERCP to remove stones
Cholecystectomy
Percutaneous cholecystotomy (drain pus)
What is acalculous cholecystitis?
Inflammation of the gallbladder in the absence of a gallstone
Occurs due to gallbladder stasis and bile stagnation
Who tends to get acalculous cholecystitis?
Very ill patients e.g. sepsis, ICU
What are the signs of acalculus cholecystitis?
Obstructive jaundice
Fever
Would be the same as calculous cholecystitis but patients are likely to be sedated or intubated to history wont obtained and signs wont be obvious
What are the investigations for acalculous cholecystitis?
Raised ALP
High CRP
USS - thickened gallbladder with absence of stones
What is the management for acalculous cholecystitis?
Cholecystectomy
Percutaneous cholecystotomy
What is ascending cholangitis?
Bacterial infection superimposed on an obstruction of the biliary tree
What is the pathogenesis of ascending cholangitis?
Obstruction of the bile ducts causes bile stagnation.
This means bacteria are not being washed out so are able to grow and cause infection
Obstruction also increases pressure in the bile ducts, widening the spaces between cholangiocytes
This causes bile to leak into the blood stream, can cause sepsis
What can cause ascending cholangitis?
Gallstones (most common)
Stricture
Tumour
What is the complication of ascending cholangitis?
Sepsis
What are the signs of ascending cholangitis?
Charcot’s triad (fever, jaundice, RUQ pain)
Reynold’s pentad (fever, jaundice, RUQ pain, hypotension, confusion)
What are the investigations for ascending cholangitis?
Bloods (raised WCC, CRP) LFTs (raised ALP, bilirubin, GGT) USS Blood culture MRCP ERCP
What is the gold standard investigation for ascending cholangitis?
ERCP
What is the management for ascending cholangitis?
IV fluids
Antibiotics
Remove obstruction via ERCP
Cholecystectomy
What is gallstone ileus?
Obstruction caused by a gallstone stuck permanently in the lumen of the small bowel
What is the pathogenesis of gallstone ileus?
The gallstone causes repeated bouts of inflammation which can cause thinning of the gallbladder wall
Erosion causes the gallbladder to stick to the duodenum and a fistula forms
This allows gallstones to pass straight to the duodenum
If the gallstone is big it will get stuck in the small bowel, usually the terminal ileus which is narrowest, causing obstruction
What are the signs of gallstone ileus?
Abdominal distension
Nausea and vomiting
Recurrent RUQ pain
Dehydration
What are the investigations for gallstone ileus?
Rigler’s triad is diagnostic
Pneumobilia
Evidence of small bowel obstruction
Evidence of gallstone outside gallbladder
What is the management for gallstone ileus?
IV fluid resuscitation
NG tube
Gallstone removal
What are the causes of biliary strictures?
Post-op
Pancreatitis
Primary sclerosing cholangitis
Cholangiocarcinoma
What are the complications of biliary strictures?
Biliary colic
Cholangitis
Obstructive jaundice
Can just be asymptomatic
What are the investigations for biliary strictures?
Related to the complication caused
Rule out malignancy
What is the management for biliary strictures?
Treat complication
Stenting
What is congenital biliary atresia and what does it lead to?
Congenital condition characterised by an absence of deficiency of extra-hepatic biliary tree
Leads to cholestasis and liver cirrhosis from the back pressure of bile
What are the symptoms of congenital biliary atresia?
Jaundice
Dark urine, pale stools
Growth and development delay
When does congenital biliary atresia present?
Within 3 months of birth
What are the investigations for biliary atresia?
LFTs
USS
HIDA scan
What is the management for biliary atresia?
Excision of fibrous tissue
Liver transplant
What is cholangiocarcinoma?
Cancer of the bile duct
What is the most common form of cholangiocarcinoma?
Hilar cholagniocarcinoma - tumour forms at the point where the left and right bile duct of the liver join together
Who tends to get cholangiocarcinoma?
> 65
South East Asia
Previous biliary disease
What are the symptoms of cholangiocarcinoma?
Can be asymptomatic Jaundice (with dark urine, pale stools) Abdominal pain Weight loss, fatigue, anorexia Fever, RUQ pain, other symptoms of cholangitis
What investigations are done for cholangiocarcinoma?
LFTs
Contrast MRI is diagnostic
CT
ERCP (sometimes for biopsy)
What is the management for cholangiocarcinoma?
Surgical resection
Stenting
Adjuvant chemotherapy and radiotherapy
What is carcinoma of the Ampulla of Vater?
Rare cancer that forms in the junction of the common bile duct and the main pancreatic duct
Prognosis is bad as there are many structures it can invade into and management is hard
