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BILIARY TRACT DISEASE
Dr Julia Epstein
Gastroenterology department
Hadassah Medical center
Biliary disease
• Gallstones
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Congenital anomalies
Extrinsic compression of the bile ducts
Hepatobiliary parasitism
Noncancerous strictures
Biliary tract anatomy
Ampullary anatomy
Biliary duct sphincter
Papilla of
Vater
Pancreatic duct
sphincter
Sphincter of
Oddi
‫כיס מרה‬
‫מבנה אגסי ‪ 7.5-10‬ס"מ‬
‫• תכולה ‪ 30-50‬ס"מ•‬
‫• מרה מיוצרת←כבד•‬
‫• מרה נאגרת←כיס מרה•‬
‫• אוכל שומני←תרסריון←הפרשת ‪•CCK‬‬
‫מרה מתכווץ ‪+‬‬
‫ספינקטר ע"ש אודי נפרה ← מעבר מיצי •‬
‫מרה לתריסריון‬
‫• ספיגה מחדש לוריד הפורטלי←חזרה •‬
‫‪Enterohepatic Circulation :‬לכבד‬
‫כבד←מרה←מעי‬
‫•‬
‫מיצי מרה‬
‫מכיל‪ :‬מים‪ ,‬אלקטרוליטיים ‪-‬‬
‫‪Na, K, Ca, Cl, HCO3‬‬
‫לציטין‪,‬חומצות שומן‪ ,‬כולסטרול‪,‬‬
‫בילירובין ומלחי מרה‬
‫מאיפה מגיע בילירובין?‬
‫תפקיד‪:‬‬
‫– ספיגה של השומנים לאחר תהליך אמולסיפיקציה‬
‫– שפעול מיצי הלבלב‬
‫– המרה (בסיסית) סותרת את מיץ הקיבה החומצי‬
‫המגיע לתריסריון‪.‬‬
‫מבנה מרה‬
‫מלחי המרה ← מסיסים במים •‬
‫פוספוליפידים ‪ +‬הכולסטרול ← לא מסיסים במים •‬
‫הופכים למסיסים שנוצרות מיצלות •‬
‫היחס בין שלושת המרכיבים עדין •‬
‫כל חריגה מהיחס המסוים ← •‬
‫מרה רוויה ‪ +‬שקיעה של •‬
‫כולסטרול (יצירת אבנים) •‬
Principal pathways of cholesterol
metabolism in liver
Gallstones – Pathophysiology
• Cholesterol, ordinarily insoluble in water, comes
into solution by forming vesicles with
phospholipids
• If ratio of cholesterol, phospholipids, and bile salts
altered, cholesterol crystals may form
• Gallstone formation involves a variety of factors:
– Cholesterol supersaturation
– Mucin hypersecretion by the gallbladder mucosa
creates a viscoelastic gel that fosters nucleation.
– Bile stasis
Gallstone pathogenesis
Cholesterol
supersaturation of
bile
Nucleation
Motility
stasis
of cholesterol
molecules to
crystals
Gallstones
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Risk factors:
Obesity
Female gender
Parity
Maternal family history ( ABCB4 , CYP7A1)
Ethnic predilection (Hispanic, Native americans)
Increasing age
Rapid weight loss
Gallstones
• Risk factors:
Ileal disease
Lipid abnormalities (high TG, low LDL)
Medications (contraceptives, postmenopausal
estrogens, lipid lowering agents
fibric acid derivated)
• TPN
5 F’s
• Fair
• Fat
• Fertile
• Female
• Forty
‫מצבים שכיחים‬
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Cholelithiasis
Biliary colic
Hydrops
Empyema
Choledocholithiasis
Acute Cholecystitis
Ascending Cholangitis
Gallstones – Types
• Two main types:
– Cholesterol stones (85%)
– Pigment stones (15%) occur in 2 subtypes—
brown and black.
• Black stones result when excess bilirubin enters the bile and
polymerizes into calcium bilirubinate (chronic hemolysis)
• Brown stones are made up of calcium bilirubinate and calciumsoaps. Bacteria involved in formation via secretion of beta
glucuronidase and phospholipase
Gallstones – Natural History
• 80% of patients, gallstones are clinically
silent
• 20% of patients develop symptoms over
15-20 years (1-2% per year)
• 50-70% continued to have symptoms
and complications
• More than 90% of complications are
preceded by biliary colic
Biliary Colic
• Intermittent obstruction of the cystic duct, no
inflammation of GB
• Severe epigastric/ RUQ pain growing over 15 min and
remaining constant up to 3h
• Frequency of attacks varies
• Normal examination
• Lab tests usually normal
• DS: US, EUS
• Natural history: 30% have no further symptoms
50% continue symptoms in 2y
Acute Calculous Cholecystitis
• Impacted stone in the cystic duct
• 75% are preceded by attacks of biliary colic
• Visceral epigastric pain – mod to severe, irradiated to RUQ,
back, shoulder, chest and lasting > 6 h
• Fever, Right subcostal tenderness with inspiratory arrest
( Murphy’s sign) , palpable GB
• Leucocytosis, mild elevation of BIL, Amylase
• 50% resolve spontaneously in 7-10 days without surgery
• DS: US, EUS, CT
• 10% are complicated by perforation.
Empyema / Mucocoele
• Mucocele refers to an
overdistended gallbladder filled
with mucoid or clear and watery
content.
• Empyema refers to a gallbladder
filled with pus due to acute
cholecystitis
Choledocholithiasis
• Intermittent obstruction of CBD
• Often symptomatic – indistinguishable from biliary
colic
• Predisposed to acute cholangitis and pancreatitis
• Signs : jaundice with pain.
• Investigations
– Elevated BIL, transient spike in Tranaaminases / Amylase,
– US, EUS, CT
• Treatment
– ERCP - Endoscopic Retrograde CholangioPancreatography
Ascending Cholangitis
•Impacted stone in CBD causing bile stasis
•Bacterial superinfection
•Charcot’s triad : pain, jaundice, fever – 70%
•Mental confusion, hypotension, RUQ tenderness
•Jaundice (>80%)
•Peritoneal signs (15%)
•Elevated WBC, BIL, APH (blood cult usually pos)
•Emergent decompression of the CBD
(ERCP, PTC)
Acute biliary pancreatitis
•
• Pancreatic duct obstruction or chemical inflammation
• Signs - Variable – None to Sepsis
(Severe pain, fever, tachycardia, low BP),
Jaundice, acute abdomen
Investigations
• Bloods – U&E, FBC, LFT, Amylase, CRP
• Ultrasound of abdomen
• MRCP
• CT Pancreas
• Treatment Supportive / 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/rt colon
• Symptoms : SBO - vomiting, abdominal pain,
distension, obstructive bowel sounds
• Investigations: X-ray, US/CT - air in CBD
• Treatment : Laparotomy and removal of stone from
small bowel and cholecystectomy.
Mirizzi’s Syndrome
Inflammatory phenomenon secondary to a pressure
ulcer caused by an impacted gallstone at the
gallbladder infundibulum
The impacted gallstone causes first external
obstruction of the CBD
Eventually erodes into the bile duct evolving into a
cholecystocholedochal fistula with different
degrees of communication between the GB and
CBD
Mirizzi’s Syndrome
Lemmmel’s Syndrome
Duodenal diverticula syndrome
Secondary to extrinsic compression by
periampullary diverticula in the absence of
additional pathology (cholelithiasis, tumor)
• The hypothesized mechanisms:
• Alterations of the papillary motility
• Bacterial contamination
• Extrinsic compression of the Main biliary tract
Lemmmel’s Syndrome
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 (eg,
severe burns, multiple traumas, lengthy postoperative care,
prolonged intensive care)
• Accounts for 5% of cholecystectomies
• Etiology is thought to have ischemic basis, and gangrenous
gallbladder may result
• Increased rate of complications and mortality
• An uncommon subtype known as acute emphysematous
cholecystitis generally is caused by infection with clostridial
organisms and occlusion of the cystic artery associated with
atherosclerotic vascular disease and, often, diabetes.
Cholecystectomy
• Laparoscopic
cholecystectomy
standard of care
• Timing
– Early vs interval operation
• Patient consent
– Conversion to open
procedure 10%
– Bleeding
– Bile duct injury
– Damage to other organs
Biliary disease
• Gallstones
• Congenital anomalies
• Extrinsic compression of the bile ducts
• Hepatobiliary parasitism
• Noncancerous strictures
Biliary Tract Cysts
• Choledochal cysts
• Consist of cystic dilatations of the extrahepatic biliary tree
• Uncommon abnormality and 90% diagnosed
before age 30
• Infantile form presentation identical to biliary
atresia
• 50% present with combination of jaundice,
abdominal pain, and an abdominal mass
Choledochal cysts
• Classified into 5 types
• Can occur in the presence of pancreaticobiliary maljunction (PBM)
• Treatment for choledochal cysts is surgical
excision of the cyst with construction
• Most ominous complication is malignancy
Choledochal cysts
PBM
Caroli disease
Biliary ductopenic disorders
• Paucity of interlobular bile ducts 2 types: syndromic and non syndromic
Syndromic ( Alagille’s syn)
ADD d/t JAG1 gene
Intrahepatic cholestasis and biliary hypoplasia
Pruritus and hepatomegaly
Extrahepatic manifeatations - congenital heart
defects, eye defects, triangular face
Biliary ductopenic disorders
• interlobular ductopenia as result of
inflammatory condition:
• PSC
• PBC
• GVHD
• Liver allograft resection
• Drug induced liver disease
• idiopathic
Biliary disease
• Gallstones
• Congenital anomalies
• Extrinsic compression of the bile ducts
• Hepatobiliary parasitism
• Noncancerous strictures
Extrinsic compression of the bile
ducts
•
•
•
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•
Biliary tract tumor
Carcinoma of the head of pancreas
Acute and chronic pancreatitis
Lymph nodes – lymphoma or metastasis
Benign stricture of biliary ducts
Biliary Tract Tumor
Cholangiocarcinoma
Cancer of the Gall Bladder
Cholangiocarcinoma
• Slow growing malignancy of biliary tract which
tend to infiltrate locally and metastasize late
• 90% adenocarcinoma
• 60-70% at the bifurcation ofhepatic ducts
• 20-30% - in the distal CBD
• 5-10% - arise within the liver (peripheral)
Biliary Tree Neoplasms
• Clinical symptoms:
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–
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Weight loss (77%)
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Nausea (60%)
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Anorexia (56%)
Abdominal pain (56%) •
•
Fatigue (63%)
Pruritus (51%)
Fever (21%)
Malaise (19%)
Diarrheoa (19%)
Constipation (16%)
Abdominal fullness (16%).
• Symptomatic patients usually have advanced
disease, with spread to hilar lymph nodes before
obstructive jaundice occurs
• Associated with a poor prognosis
Risk factors
• Liver flukes (Opistorchis viverrini, Chlonorchis
sinensis)
• Chemial exposition (Asbestosis)
• Congenital predisposition (PBM, Choledochal
cysts)
• Intrahepatic biliary stones
• PSC
Cholangiocarcinoma
Diagnosis and Initial Workup
• Jaundice
• Weight loss, anorexia, abdominal pain, fever
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US – bile duct dilatation
3-phase CT
MRCP/MRI
ERCP with Brush biopsy
Percutaneous Cholangiography 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
Surgical Removal – only 25%
resectable at the time of diagnosis
• Node Dissection in Bile
Duct Excision
• Roux-en-Y
Hepaticojejunostomy
Cholangiocarcinoma
Palliative therapy :
• Stent
• Chemotherapy +/- Radiation Therapy
• Survival with surgery and chemo/radiation is 24
to 36 months
• With chemotherapy / radiation alone survival is
12 to 18 months
• Liver transplantation – 80% 5-y survival rate
• In selected patients who complete chemoradiation protocol
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, Salmonella typhi
carrier state, some drugs
Gall Bladder Cancer
• Uncommonly diagnosed preoperatively
• >80% with gallstones
• Clinical manifestation from abdominal pain to
unexplained weight loss and jaundice
• Palpable RUQ mass
• Jaundice suggests local extention with ductal
obstruction
• DS: US, EUS, CT
Gall Bladder Cancer
• Discovered on pathology after a routine
cholecystectomy (1-2%)
• If negative for metastasis:
– Radical cholecystectomy with nodal dissection,
central hepatectomy, w or w/o bile duct excision
– Excise port sites
– Followed by Chemo/Radiation
• 5 year survival = 60%
Gallbladder Cancer
• Cholecystectomy should not be offered for all
patients with stones for fear of cancer
• Cholecystectomy should be considered for
calcified GB and for growths (adenomyoma,
polyps > 15 mm)
Pancreatic cancer
• Pancreatic cancer is
the most common
malignant cause,
followed by cancers of
the gallbladder, bile
duct, liver, and large
intestine.
Metastasis
• 1st category involves local extension into the hilum by a
tumor arising in an adjacent structure, such as the
gallbladder, cholangio CA
• The 2nd - includes metastases from a distant primary site,
most often from solid tumors, such as carcinoma of the
breast, colon, ovaries or lymphoma, melanoma
• Mechanical cholestasis caused by the stricture,
mostly of the common hepatic or common bile duct
Patients have severe jaundice and associated symptoms
such as pruritis, recurrent cholangitis and malaise.
Metastasis
Biliary disease
• Gallstones
• Congenital anomalies
• Extrinsic compression of the bile ducts
• Hepatobiliary parasitism
• Noncancerous strictures
Hepatobiliary parasitism
• Pyogenic cholangitis &hepatic abscess, ductal
stones, biliary obstruction
• Clonorchis sinensis, Opisthorchis viverrini, O.
felineus, Fasciola hepatica
• Ascaris lumbricoides, Echinococcus spp.
Clinical manifestation of hydatid
cyst
• Most patients with hepatic uncomplicated hydatid cyst are
asymptomatic
 Possible symptoms and signs are: RUQ pain , epigastric
pain , fever , fatigue , nausea and dyspepsia ,hepatomegaly
and abdominal mass
 Complications:
 Superinfection of hydatid cysts
 Rupture to adjacent structures (peritoneal spillage,
 cholangitis, pancreatitis, anaphylaxis)
 Rare – portal HTN, hepatic vein thrombosis,
secondary biliary cirrhosis
Diagnosis
 History of exposure
 Chest and abdominal X-ray
 Ultrasound (diagnostic method of choice)
 CT (better information about location, depth , mandatory
before planning operation)
 MRI (for NS, venous system and biliary complications )
 Serology (positive – confirms infection, negative test
does not exclude)
Gharbi’s US classification
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
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
Type I Type II Type III Type IV Type V -
Pure fluid collection
Fluid collection with a detached membrane
Fluid collection with multiple septa and or daughter cysts
Hyperechoic with high internal echoes
Cysts with reflecting, calcified walls
CT
ERCP
Histology
Treatment of hepatic hydatid
cyst
 The treatment of hepatic hydatid cysts is
strongly indicated in order to prevent cyst
complications
 The therapeutic options are:
1. Surgical intervention – remains the
cornerstone of radical treatment
2. Percutaneous drainage
3. Drug therapy
Surgical options
Open surgical techniques
 Radical removal of pericystic membrane and parasitic
content
 Marsupialization (partial cysto-pericystectomy)
Contraindications: severe comorbidity
Complications:
biliary fistula, cyst infection, pleural
effusion, peritonitis, abscess, anaphylactic shock
 Laparoscopic surgery
Contraindications: Deep intraparenchimal cysts
> 3 cysts, with thick calcified wall
Complications: intra-abdominal seeding due to
pneumoperitoneum
Paliative procedures
 PAIR - puncture-aspiration-injection-reaspiration
Indicated for type I , II and III cysts
Inoperable patients, pregnant women
Multiple disseminated cysts
 Contraindications:
IV, V types of cysts,
ruptured cysts into biliary tree or peritoneum
 ERCP – Naso-biliary drainage – biliary endoprosthesis
 Combined therapy with albendazole is an effective and safe
alternative to surgery for uncomplicated hydatid cysts
(Khuroo et al, 1998)
Indications for drug therapy:
WHO Guidelines 1996
 Inoperable primary liver or lung echinococcosis
 Multiple echinococcal multiorgan and peritoneal
cysts
 Preoperative or pre-drainage (at least 4 days
before surgery and 1 m (ABZ) or 3 m (MBZ) after)
 Poor patient status
Contraindications:
 Large cysts that are at risk of rupture
 Pregnancy ,chronic liver disease or depressed BM
Biliary disease
•
•
•
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Gallstones
Congenital anomalies
Extrinsic compression of the bile ducts
Hepatobiliary parasitism
• Noncancerous strictures
Biliary Stricture – Non Cancerous
Causes
Noncancerous causes of bile duct stricture
include:
• Injury to the bile ducts during surgery for
gallbladder removal
• Pancreatitis (inflammation of the pancreas)
• Primary sclerosing cholangitis
• Gallstones (benign CBD stricture, papillary
stenosis
• Blunt trauma to the abdomen
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’s colitis, and
rare conditions, such as Riedel thyroiditis and
retroperitoneal fibrosis
• Most patients present with fatigue and pruritus
and, occasionally, jaundice
PSC
•Natural history is variable but involves
progressive destruction of the bile ducts,
leading to cirrhosis and liver failure
•Clinical features of cholangitis (ie, fever, right
upper quadrant pain, jaundice) are uncommon
unless the biliary system has been
instrumented.
PSC
PSC
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
PBC
Antimitochondrial 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 (eg, pruritus, osteoporosis, sicca
syndrome)
Liver transplantation appears to be the only lifesaving procedure.
ERCP
Endoscopic retrograde
cholangiopancreatography (ERCP)
• Endoscopic tube is placed into the
patient’s mouth, through the
stomach, and into the duodenal
portion of the small intestine.
• Contrast is introduced into the
biliary tract through the
endoscope, in a retrograde
manner.
• X-rays taken
‫‪ERCP‬‬
‫ספינקטרוטומיה •‬
‫הוצאת אבנים (בלון‪ ,‬בסקט)‪ ,‬ריסוק אבנים •‬
‫הכנסת סטנטם •‬
‫‪PTC‬‬
‫הזרקת חומר ניגוד דרך עור לדרכי מרה‬
‫התוך כבדיים תחת סונר והדמיה ברנטגן‬
‫‪PCC‬‬
‫‪• PerCutaneous Cholecystostomy‬‬
‫במקרים קשים בהם ניתוח עלול לסכן את החולה – ניקוז •‬
‫מרה בשיטה מלעורית‬
Indications For Biliary Stenting
Indications for stent insertion include:
• Ampullary Stenosis
• Bile duct injury
• Benign or malignant biliary obstruction
• Prevention of obstruction where stone
extraction is not possible at that time
• Pancreatic duct strictures, stones and
sphincter of Oddi dysfunction
Stent Placement -Endoscopic Approach
• A catheter is inserted through
the endoscope into the ostium
of the common bile duct.
• While maintaining the
endoscope position in the
duodenum, a wire is inserted
through the catheter into the
bile duct.
• The stent delivery system is
then inserted over the wire
to the site of obstruction,
where the stent is deployed.
Stent Placement – Endoscopic
Approach
Success rate of ERCP 90-95%
Complication rate of approximately 3-5%.
Complications:
•
•
•
•
•
Pancreatitis
Bleeding
Perforation
Infection
Cardiopulmonary depression from conscious sedation.
Biliary Stent - Percutaneous transhepatic
approach PTC
For biliary stent placement
using a percutaneous
approach:
•A fine needle is inserted
between the 4th and 5th rib on
the patient’s right side
•The puncture is through the
liver
•The needle is inserted into an
intrahepatic duct under image
guidance.
Photo on file at Medtronic
Biliary Stent - Percutaneous Approach
Success rate 95% when ducts are dilated
• 5-10% rate of major complications which include:
•Sepsis
•Bile leak
•Intraperitoneal haemorrhage, Haemobilia
•Hepatic and perihepatic abscess, Pneumothorax
•Skin infection and granuloma at the catheter entry site
• Contraindicated in patients with bleeding diatheses and
significant ascites.