Biliary/Pancreatic Disease Flashcards
Primary sclerosing cholangitis is
a biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.
Primary sclerosing cholangitis associations?
ulcerative colitis
Crohn’s (much less common association than UC)
HIV
?% of patients with UC have PSC, ?% of patients with PSC have UC
4% of patients with UC have PSC, 80% of patients with PSC have UC
PSC bloods
raised bilirubin + ALP
PSC sx
cholestasis
jaundice, pruritus
right upper quadrant pain
fatigue
PSC ix
endoscopic retrograde cholangiopancreatography (ERCP)
or magnetic resonance cholangiopancreatography (MRCP)
are the standard diagnostic investigations, showing multiple biliary strictures giving a ‘beaded’ appearance
PSC which ANCA might be positive
p-ANCA may be positive
PSC always use liver biopsy
false
limited role for liver biopsy,
PSC what would liver biopsy show
fibrous, obliterative cholangitis often described as ‘onion skin’
PSC complications
cholangiocarcinoma (in 10%)
increased risk of colorectal cancer
Primary biliary cholangitis is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1)
true
Primary biliary cholangitis aetiology is not fully understood although it is thought to be an autoimmune condition
true
Primary biliary cholangitis classic presentation
itching in a middle-aged woman
Primary biliary cholangitis pathophysiology?
Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis
Primary biliary cholangitis associations
Sjogren’s syndrome (seen in up to 80% of patients)
rheumatoid arthritis
systemic sclerosis
thyroid disease
PBC sx
early: may be asymptomatic or fatigue, pruritus
cholestatic jaundice
hyperpigmentation, especially over pressure points
around 10% of patients have right upper quadrant pain
xanthelasmas, xanthomata
also: clubbing, hepatosplenomegaly
late: may progress to liver failure
PBC LFTs
raised ALP on routine LFTs
PBC diagnosis?
anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
smooth muscle antibodies in 30% of patients
raised serum IgM
PBC mx
first-line: ursodeoxycholic acid
slows disease progression and improves symptoms
pruritus: cholestyramine
fat-soluble vitamin supplementation
PBC liver is transplanted is indicated when?
if bilirubin > 100 (PBC is a major indication)
recurrence in graft can occur but is not usually a problem
PBC complications
cirrhosis → portal hypertension → ascites, variceal haemorrhage
osteomalacia and osteoporosis
significantly increased risk of hepatocellular carcinoma (20-fold increased risk)
Viral hepatitis common symptoms include:
nausea and vomiting, anorexia
myalgia
lethargy
right upper quadrant (RUQ) pain
Viral hepatitis Questions may point to risk factors such as
foreign travel or intravenous drug use.
Congestive hepatomegaly sx
The liver only usually causes pain if stretched
One common way this can occur is as a consequence of congestive heart failure.
Biliary colic sx
RUQ pain, intermittent, usually begins abruptly and subsides gradually. Attacks often occur after eating. Nausea is common.
Acute cholecystitis sx
Pain similar to biliary colic but more severe and persistent. The pain may radiate to the back or right shoulder.
The patient may be pyrexial and Murphy’s sign positive (arrest of inspiration on palpation of the RUQ)
Ascending cholangitis sx
An infection of the bile ducts commonly secondary to gallstones. Classically presents with a triad of:
fever (rigors are common)
RUQ pain
jaundice
Gallstone ileus sx
Abdominal pain, distension and vomiting are seen.
Cholangiocarcinoma sx
Persistent biliary colic symptoms, associated with anorexia, jaundice and weight loss. A palpable mass in the right upper quadrant (Courvoisier sign), periumbilical lymphadenopathy (Sister Mary Joseph nodes) and left supraclavicular adenopathy (Virchow node) may be seen
Acute pancreatitis sx
Usually due to alcohol or gallstones
Severe epigastric pain
Vomiting is common
Examination may reveal tenderness, ileus and low-grade fever
Periumbilical discolouration (Cullen’s sign) and flank discolouration (Grey-Turner’s sign) is described but rare
Pancreatic cancer sx
Painless jaundice is the classical presentation of pancreatic cancer. However pain is actually a relatively common presenting symptom of pancreatic cancer. Anorexia and weight loss are common
Amoebic liver abscess sx
Typical symptoms are malaise, anorexia and weight loss. The associated RUQ pain tends to be mild and jaundice is uncommon.
Bile-acid malabsorption is a cause of
chronic diarrhoea
Bile-acid malabsorption may be primary, due to
excessive production of bile acid, or secondary to an underlying gastrointestinal disorder causing reduced bile acid absorption.
Bile-acid malabsorption leads to
steatorrhoea and vitamin A, D, E, K malabsorption.
Bile-acid malabsorption secondary causes?
patients with ileal disease, such as with Crohn’s. Other secondary causes include:
cholecystectomy
coeliac disease
small intestinal bacterial overgrowth
Bile-acid malabsorption ix
the test of choice is SeHCAT
nuclear medicine test using a gamma-emitting selenium molecule in selenium homocholic acid taurine or tauroselcholic acid (SeHCAT)
scans are done 7 days apart to assess the retention/loss of radiolabelled 75SeHCAT
Bile-acid malabsorption mx
bile acid sequestrants e.g. cholestyramine
Raised levels of unconjugated bilirubin may occur as a result of
haemolysis, which is to say a pre-hepatic source, for example, autoimmune-mediated haemolytic anaemia. Red blood cell breakdown exposes heme-containing proteins and, as discussed above, these are then processed to form unconjugated bilirubin.
Raised levels of conjugated bilirubin can result from
defective excretion of bilirubin, for example, Dubin-Johnson Syndrome, or cholestasis.
Jaundice starts to appear when bilirubin reaches an excess of
35umol/l
Gallstones ix
abdominal ultrasound and liver function tests
Biliary colic mx
If imaging shows gallstones and history compatible then laparoscopic cholecystectomy
Acute cholecystitis mx
Imaging (USS) and cholecystectomy (ideally within 48 hours of presentation) (2)
Gallbladder abscess mx
Imaging with USS +/- CT Scanning
Ideally, surgery although subtotal cholecystectomy may be needed if Calot’s triangle is hostile
In unfit patients, percutaneous drainage may be considered
Cholangitis mx
Fluid resuscitation
Broad-spectrum intravenous antibiotics
Correct any coagulopathy
Early ERCP
Gallstone ileus mx
Laparotomy and removal of the gallstone from small bowel, the enterotomy must be made proximal to the site of obstruction and not at the site of obstruction. The fistula between the gallbladder and duodenum should not be interfered with.
Acalculous cholecystitis mx
If patient fit then cholecystectomy, if unfit then percutaneous cholecystostomy
Risks of ERCP(1)
Bleeding 0.9% (rises to 1.5% if sphincterotomy performed)
Duodenal perforation 0.4%
Cholangitis 1.1%
Pancreatitis 1.5%
Pancreatic cancer is often diagnosed late as
it tends to present in a non-specific way
Over 80% of pancreatic tumours are ?
adenocarcinomas
Over 80% of pancreatic tumours typically occur where
at the head of the pancreas.
Pancreatic cancer assoc
increasing age
smoking
diabetes
chronic pancreatitis (alcohol does not appear an independent risk factor though)
hereditary non-polyposis colorectal carcinoma
multiple endocrine neoplasia
Pancreatic cancer genetics
BRCA2 gene
KRAS gene mutation
Pancreatic cancer sx
classically painless jaundice
pale stools, dark urine, and pruritus
patients typically present in a non-specific way with anorexia, weight loss, epigastric pain
loss of exocrine function (e.g. steatorrhoea)
loss of endocrine function (e.g. diabetes mellitus)
atypical back pain is often seen
cholestatic liver function tests seen in pancreatic cancer
true
Trousseau sign
pancreatic cancer migratory thrombophlebitis (Trousseau sign) is more common than with other cancers
Pancreatic cancer ix
ultrasound has a sensitivity of around 60-90%
high-resolution CT scanning is the investigation of choice if the diagnosis is suspected
imaging may demonstrate the ‘double duct’ sign - the presence of simultaneous dilatation of the common bile and pancreatic ducts
Pancreatic cancer mx
less than 20% are suitable for surgery at diagnosis
a Whipple’s resection (pancreaticoduodenectomy) is performed for resectable lesions in the head of pancreas. Side-effects of a Whipple’s include dumping syndrome and peptic ulcer disease
adjuvant chemotherapy is usually given following surgery
ERCP with stenting is often used for palliation
