BILIARY TRACT

DISEASE
DR.ALEX MOGERE
CONSULTANT PHYSICIAN
OVERVIEW
• Gallstones
• Bile

• Biliary tract tumours

• Other conditions
• Acute acalculous cholecystitis
• Primary Biliary Cirrhosis(PBC)
• Primary Sclerosing Cholangitis(PSC)
• Biliary tract cysts
• Biliary strictures
BILE
• Bile = bile acids+phospholipids +cholesterol
• Primary bile acids- cholic and chenodeoxycholic acids,
formed in liver from cholesterol and AAs
• Secondary bile acids- bacterial metabolites of primary bile
acids formed in the colon(deoxycholate and lithocholate)
• Helps excrete cholesterol,aid in fat digestion,and fat
absorption, cholesterol and fat soluble vitamins in the
intestine
BILIARY TRACT
Part of the digestive system.
Made up of:
•Intra hepatic ducts

•Extra hepatic ducts

•Gallbladder

•Common Bile Duct

 THE GALLBLADDER
The gallbladder concentrates and stores bile.
Bile:
•Secreted by the liver
•Contains cholesterol, bile pigments and
phospholipids
•Flows from the liver, through the hepatic
ducts, into the gallbladder
•Exits the gallbladder via the cystic duct
•Flows from the cystic duct into the common
bile duct, into the small intestine
•In the small intestine, aids digestion
by breaking down fatty foods and
fat-soluble vitamins
 GALLSTONES –
PATHOPHYSIOLOGY
• If ratio of cholesterol, phospholipids, and bile salts altered,
cholesterol crystals may form

• Gallstone formation involves a variety of factors:

• Cholesterol super-saturation
• Mucin hyper-secretion by the gallbladder mucosa creates a
viscoelastic gel that fosters nucleation.
• Bile stasis
• Occurs in diabetes, pregnancy, oral contraceptive use, and
prolonged fasting in critically ill patients on total parenteral
nutrition.
 GALLSTONES – FREQUENCY
• Gallstone disease is one of the most common and costly of all
digestive diseases
• 9% of those > 60 years
• In USA, 6.3 million men and 14.2 million women aged 20-74
years have gallbladder disease
• Incidence of gallstones is 1 million new cases per year
• Prevalence is 20 million cases in USA
GALLSTONES – TYPES
• Two main types:
• Cholesterol stones (85%):
• 2 subtypes—pure (90-100% cholesterol) or mixed (50-90% cholesterol).
• Pure stones often are solitary, whitish, and larger than 2.5 cm in
diameter.
• Mixed stones usually are smaller, multiple in number, and occur in
various shapes and colours.
• Pigment stones (15%) occur in 2 subtypes—brown and black.
• Brown stones are made up of calcium bilirubinate and calcium-soaps.
Bacteria involved in formation via secretion of beta glucuronidase and
phospholipase
• Black stones result when excess bilirubin enters the bile and polymerizes
into calcium bilirubinate (patients with chronic haemolysis)
GALLSTONES – NATURAL HISTORY
• 80% of patients, gallstones are clinically silent
• 20% of patients develop symptoms over 15-20
years
• About 1% per year
• Almost all become symptomatic before
complications develop
• Biliary-type pain due to obstruction of the bile
duct lumen
GALLSTONES – DIVERSE
SYMPTOMS
• Abdominal pain
• Aching or tightness, typically severe and located in the epigastrium,more
at night, esp after fatty meal
• May develop suddenly, last for 15 minutes to several hours, and then
resolve suddenly
• Referred pain – posterior scapula or right shoulder area
• Nausea and vomiting
• Jaundice
• Pruritus: typically worse at night.
• Fatigue
• Weight loss
• Miscellaneous:
• Fatty food intolerance
• Gas
• Bloating
COMPLICATIONS OF GALLSTONES
• In the gallbladder
• Biliary colic
• Acute and chronic cholecystitis
• Empyema
• Mucocele
• Carcinoma
• In the bile ducts
• Obstructive jaundice
• Pancreatitis
• Cholangitis
• In the Gut
• Gallstone ileus
OBSTRUCTIVE JAUNDICE
• Blockage of the biliary tree by gallstones
• Symptoms
• Pain, Jaundice, dark urine, pale stools
• Signs
• Jaundice.
• Investigations
• Bloods – U&E, FBC, LFT, Amylase, CRP, Hepatitis screen,
Coagulation screen
• Ultrasound of abdomen
• Treatment
• Endoscopic Retrograde Cholangio-pancreatogram
ASCENDING CHOLANGITIS
• Obstruction of biliary tree with bile duct infection

• Symptoms
• Unwell, pain, jaundice, dark urine, pale stools
• Charcot triad (ie, fever, right upper quadrant pain, jaundice) occurs in only
20-70% of cases

• Signs
• Sepsis (Fever, tachycardia, low BP), Jaundice.
• Investigations
• Bloods – U&E, FBC, LFT, Amylase, CRP, Coagulation screen
• Ultrasound of abdomen
• Treatment
• Intravenous antibiotics
• Endoscopic Retrograde Cholangio-Pancreatogram (ERCP)
GALLSTONE ILEUS
• Obstruction of the small bowel by a large gallstone
• A stone ulcerates through the gallbladder into the duodenum and causes
obstruction at the terminal ileum

• Symptoms
• Small bowel obstruction (vomiting, abdominal pain, distension, nil PRr)
• Signs
• Abdominal distension, obstructive bowel sounds.
• Investigations
• Bloods – U&E, FBC, LFT, Amylase, CRP, Hepatitis screen, Coagulation
screen
• Plain film of abdomen – Air in CBD, small bowel fluid levels and stone
• Treatment
• Laparotomy and removal of stone from small bowel.
 MANAGEMENT OF
GALLSTONES
• Abdominal U/S, CT scan Abdomen

When to treat
• Asymptomatic vs. symptomatic

How to treat
• Surgery: open vs. laparoscopic cholecystectomy
• Medical therapy:
• Ursodeoxycholic acid to dissolve stones; very slow up to years, very
ill patients and surgically unfit
• Chemical dissolution
• ON the stones. Experimental
• Lithotripsy
ACUTE ACALCULOUS CHOLECYSTITIS
• Presence of an inflamed gallbladder in the absence of an
obstructed cystic or common bile duct
• Typically occurs in the setting of a critically ill patient (e.g.,
severe burns, multiple traumas, lengthy postoperative care,
prolonged intensive care)
• Accounts for 5% of cholecystectomies
• Aetiology is thought to have ischemic basis, and gangrenous
gallbladder may result
• Increased rate of complications and mortality
 PRIMARY SCLEROSING CHOLANGITIS
• Chronic cholestatic biliary disease characterized by non-
suppurative inflammation and fibrosis of the biliary ductal system
• Cause is unknown but is associated with autoimmune
inflammatory diseases, such as chronic ulcerative colitis and
Crohn’ colitis, and rare conditions, such as Riedel thyroiditis and
retroperitoneal fibrosis
• Most patients present with fatigue and pruritus and, occasionally,
jaundice
• Natural history is variable but involves progressive destruction of
the bile ducts, leading to cirrhosis and liver failure
• Clinical features of cholangitis (i.e., fever, right upper quadrant
pain, jaundice) are uncommon unless the biliary system has been
instrumented.
 PRIMARY SCLEROSING
CHOLANGITIS
Medical Care
•Chronic progressive disease with no curative medical therapy
•Goals of medical management are to treat the symptoms and to
prevent or treat the known complications
•Liver transplantation is the only effective therapy and is indicated
in end-stage liver disease.

Surgical Care
•Indications for liver transplantation include variceal bleed or
portal gastropathy, intractable ascites, recurrent cholangitis,
progressive muscle wasting, and hepatic encephalopathy.
•Recurs in 15-20% of patients after transplantation.
PRIMARY BILIARY CIRRHOSIS
• Progressive cholestatic biliary disease that presents with fatigue
and itching or asymptomatic elevation of the alkaline
phosphatase.
• Jaundice develops with progressive destruction of bile ductules
that eventually leads to liver cirrhosis and hepatic failure.
• Autoimmune illness has a familial predisposition
• Anti-mitochondrial antibodies (AMA) are present in 95% of
patients
• Goals of treatment are to slow the progression rate of the
disease and to alleviate the symptoms (e.g. pruritus,
osteoporosis, sicca syndrome)
• Liver transplantation appears to be the only life-saving
procedure.
BILIARY TRACT CYSTS
• Choledochal cysts
• Consist of cystic dilatations of the extra-hepatic
biliary tree
• Uncommon abnormality
• 50% present with combination of jaundice,
abdominal pain, and an abdominal mass.
• ? Due to anomalous union of the pancreatic
and biliary ductal system.
• Classified into 5 types
• Treatment for choledochal cysts is surgical
(excision of the cyst with construction).
BILIARY TRACT TUMOURS:

1. CHOLANGIOCARCINOMA

2. CANCER OF THE GALL

BLADDER
BILIARY TREE NEOPLASMS
• Clinical symptoms:
• Weight loss (77%)
•Fever (21%)
• Nausea (60%)
•Malaise (19%)
• Anorexia (56%)
•Diarrhoea (19%)
• Abdominal pain (56%)
•Constipation (16%)
• Fatigue (63%)
•Abdominal fullness (16%).
• Pruritus (51%)
• Symptomatic patients usually have advanced disease, with spread to
hilar lymph nodes before obstructive jaundice occurs
• Associated with a poor prognosis.
CHOLANGIOCARCINOMA
• Adenocarcinoma of the bile ducts
• May occur without associated risk factors
• Associated with chronic cholestatic liver disease such as:
• Primary Sclerosing Cholangitis
• Choledochal cysts
• Asbestos.
• Accounts for 25% of biliary tract cancers
• Presentation:
• Jaundice
• Vague upper or right upper quadrant abdominal pain
• Anorexia, weight loss
• Pruritus.
Cont. CHOLANGIOCARCINOMA
• Slow growing malignancy of biliary tract which tend to infiltrate
locally and metastasize late.
• Gall Bladder cancer = 6,900/year
• Bile duct cancer = 3,000/year
• Hepatocellular Cancer = 15,000/year
CHOLANGIOCARCINOMA
DIAGNOSIS AND INITIAL WORKUP
• Jaundice
• Weight loss, anorexia, abdominal pain,
fever
• US – bile duct dilatation
• Quadruple phase CT
• MRCP/MRI
• ERCP with Stent and Brush Biopsy
• Percutaneous Cholangiogram with
Internal Stent and Brush Biopsy
MRCP: CHOLANGIOCARCINOMA AT THE BIFURCATION

Klatskin tumour = Cholangiocarcinoma of junction of right & left hepatic

ducts
ERCP: DISTAL CBD CANCER
GALLBLADDER CANCER
• 6th decade
• 1:3, Male : Female
• Highest prevalence in Israel, Mexico, Chile,
Japan, and Native American women.

• Risk Factors: Gallstones, porcelain

gallbladder, polyps, chronic typhoid and
some drugs
 Cont. GALLBLADDER CANCER
• Uncommon malignancy
• 2.5 per 100,000 population
• Represents 54% of biliary tract cancers.
Cont. GALL BLADDER CANCER
PRESENTATION

• RUQ pain, jaundice, weight loss

• Biopsy yields adenocarcinoma consistent with GB primary
• Biliary Decompression
• Chemo/Radiation
• Median survival with chemo-radiotherapy is 9 months.
BILIARY STRICTURE

Biliary stricture is an abnormal

narrowing of the bile duct.
Among biliary strictures:
•90% are malignant
•Pancreatic cancer is the most
common malignant cause, followed
by cancers of the gallbladder, bile
duct, liver, and large intestine.
 BILIARY STRICTURE – NON CANCEROUS
CAUSES
Noncancerous causes of bile duct stricture include:
•Injury to the bile ducts during surgery for gallbladder removal
(accounting for 80% of nonmalignant strictures)
•Pancreatitis (inflammation of the pancreas)
•Primary Sclerosing cholangitis (an inflammation of the bile ducts
that may cause pain, jaundice, itching, or other symptoms)
•Gallstones
•Radiation therapy
•Blunt trauma to the abdomen
Biliary Stricture – Patient Symptoms

Patients with biliary strictures may present with:

•Jaundice (yellow skin color)
•Abdominal pain
•Fever
•Vomiting
BILIARY STRICTURE – DIAGNOSTIC
TESTS
Common diagnostics for biliary stricture are:
• Ultrasound
• CT
• MRI
• Biopsy
• Cholangiography
A Cholangiogram is an X-ray of the bile ducts
• Can be performed:
• Endoscopicaly
• Percutaneously
STENT PLACEMENT - ENDOSCOPIC APPROACH

The Endoscope is
positioned in the
duodenum at the
opening of the bile
duct.
Summary
• Gallstones
• In the bile ducts
• In the gallbladder
• Obstructive jaundice
• Biliary colic
• Pancreatitis
• Acute and chronic cholecystitis
• Cholangitis
• Empyema
• In the Gut
• Gallstone ileus

• Biliary tract tumours

• Other conditions
• Acute acalculous cholecystitis
• Mirizzi’s syndrome
• Primary Biliary Cirrhosis
• Primary Sclerosing Cholangitis
• Biliary tract cysts
• Biliary strictures
HEPATOCELLULAR
CARCINOMA
• General points to remember
• Increasing incidence
• Prognosis improving with screening. Early diagnosis, more
therapies for HBV & HCV, more therapies for cancer itself
• Can be diagnosed without biopsy
• Can be cured by transplant, surgery or in some cases local
ablation
• Some patients can live several years without treatment:
THE END