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BILIARY TRACT

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Bile System Diseases Textbook of Medicine

Gallbladder primary disease

Biliary tract obstruction

Drugs

Other gastro

Neural disorders

Metabolic disorders

Gallstones, Cholecystitis

Cystic duct, Common Duct, Pancreas Head, Oddi dysfunction, Stenosis

Narcotics, Anticholinergic Agents

Irritable Bowel Syndrome

Spinal Cord Injury, Truncal Vagotomy, Achalasia

Obesity, Diabetes, Sexual Hormones, Pregnancy, Somatostatin therapy, Sprue, Vipoma, Sickle Hemoglobinopathy

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Valves of Heister

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Variation is common

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Gallbladder and Bile Ducts Function

Bile storage (~30ml) Bile concentration (water & electrolytes absorption) & mucus secretionBile release

Fasting State:

High pressure Oddi

Prevents duodenal reflux

Promote gallbladder filling

AminoA

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Composition of Hepatic and Gallbladder BileMeq/l Hepatic Gallbladder

Na 160 270

K 5 10

Cl 90 15

HCO3 45 10

Ca 4 25

Bilirubin 1.4 15

Protein 150 -

Bile acids 50 150

Phospholipids 8 40

Cholesterol 4 18

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Bacteriology

Bile is normally sterile Bactibilia present in 11% to 30% in

chronic biliary disease, 50% in acute cholecystitis, 60% in common bile duct stones and 95% in cholangitis

Highest in elderly (decreased motility and clearance)

Mainly gram-neg aerobe single organism, polymicrobial infection in cholangitis

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Antibiotic Selection

Antibiotics should be used prophylactically in elective biliary tract surgery or manipulations

Not mandatory in low-risk patients as wound infection is low (1%) following laparoscopic cholecystectomy

Single dose of first generation cephalosporin provides good coverage in high risk patients

Bile infections are treated with second generation cephalosporins, aminoglycoside and fluoroquinolones

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Imaging

US UltrasonographyEUS Endoscopic ultrasonographyHIDA Hepatobiliary iminodiacetic acid scanERCP Endoscopic retrograde cholangio

pancreatog.PTC Percutaneous transhepatic

cholangiographyIOC Intraoperative cholangiography (open or

lap)CT MRCPCholedocoscopy (IO, ER, TH)

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Diseases of the Gallbladder

Congenital Anomalies Gallstones Acute and Chronic Cholecystitis Hyperplastic Cholecystoses

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Congenital Anomalies

Anomalies in number, size and shape Agenesis Duplication Rudimentary or giant gallbladder Diverticula

Anomalies of position or suspension Left-sided Intrahepatic Floating predisposing to torsion

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Gallbladder stasis, sludge & stone formation

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Cholelithiasis- type of gallstones

Type of Stone Cholesterol Black Pigment Brown Pigment

Location Gallbladder Gallbladder Bile Ducts

Pathogenesis 80% Infectious

Cholesterol monohydrate

>50% <20% ++

Calcium & Bilirubin

+ +++ calcium bilirubinate

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Obesity, weight loss, estrogens

Chronic hemolysis, alcoholic cirrhosis, pernicious anemia, infections, ileal disease

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Natural History of Gallstone Disease Silent

over 20y period, 2/3 remain symptoms free Biliary pain by cystic duct obstruction

2/3 have further episodes within 1 yeardelaying treatment contribute to high prevalence of complications (44% of cholecystectomies performed for complications)

Complications include cholecystitischoledocolithiasis with or without cholangitisgallstone pancreatitisgallstone ileusgallbladder carcinoma

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Inflammation Obstruction

Severe steady ache or fullness in epigastrium or ruq with frequent radiation to right scapula

Migration Distension Biliary “colic” Nausea vomiting, precipitated by eating

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Acute Cholecystitis

Etiology: cystic duct obstruction with secondary bacterial infection (75%)

Mechanical inflammationincreased intraluminal pressure, distension, ischemia of gallbladder mucosa and wall

Chemical inflammationrelease of lysolecithin and other tissue factors

Bacterial inflammationescherichia coli, klebsiella, streptococcus, clostridium spp

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Acute Cholecystitis

Clinical presentation women 30-80y old, prior episodes of biliary colic

or previous cholecystitis (60% recurrent within 5y)fever , nausea, vomitingright upper quadrant pain following a fatty mealMurphy sign, palpable mass

Diagnosis: ultrasound, HIDA, CBC, LFT, Amylase , ECG , Chest x-ray

Treatment: Cholecystectomy – immediate or delayed

Perioperative antibioticsCholecystostomy in high risk patients (diabetes,

elderly) Emphysematous cholecystitis

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Acute

Cholecystitis

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Acute Cholecystitis

Complications occur mainly in not-treated patientsEmpyema and hydropsGangrenous and Perforated cholecystitis with localized / generalized peritonitisBilio-enteric and Cholecysto-choledocal fistulaGallstone ileusCalcium deposition: Limey, PorcelainCarcinoma of gallbladderSepsis

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Acalculous Cholecystitis 5-10%

Absence of gallstonesUsually complicate the outcome of

severeburns , sepsis , trauma or collagen

diseaseEtiology: thrombosis of cystic art

sphincter spasmprolonged fastingdehydration

systemic disease, sepsis

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Hyperplastic Cholecystoses

Adenomyomatosis Cholesterolosis

Strawberry gallbladder Cholesterol “polyps”

Cholecystectomy when symptomatic or gallstones

Gallbladder polyp Cholecystectomy when symptomatic or

over 50 y or polyps > 10mm or gallstones or polyp growth on serial US

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Diseases of the Bile Ducts

Congenital anomalies Choledocolithiasis Trauma, strictures, hemobilia (95%

iatrogenic) Extrinsic compression Hepatobiliary parasitism Sclerosing cholangitis

Increased risk for cholangiocarcinoma

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Obstructive Jaundice, diagnosis Increased production unconjugated

multiple transfusion, transfusion reaction, sepsis, burns, hemolysis, congenital hemoglobinopathies

Impaired uptake or conjugation unconjugated

Gilbert’s disease, neonatal jaundice, sepsis, viral hepatitis, drug inhibition, Crigler-Najjar syndrome

Impaired transport and excretion conjugated

cirrhosis, amyloidosis, cancer, pregnancy, hepatitis, Dubin-Johnson syndrome

Biliary obstruction conjugated

choledocolithiasis, periampullary cancer, chronic pancreatitis, primary sclerosing cholangitis, cholangiocarcinoma

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Obstructive Jaundice, goals of imaging

Confirmation of intrahepatic and/or extrahepatic bile duct dilation

Identification of site and cause of the obstruction

Selection of treatment modalitypreoperative biliary drainage?anticipate!

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Obstructive Jaundice: Additive Operative Risk Factors

Alterations in hepatic and pancreatic functiongastrointestinal barrierimmune functionhemostatic mechanismswound healing

Malnutrition (hypoalbuminemia), cholangitis and renal insufficiency are associated with increased morbidity and mortality

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Congenital anomalies = Risk!

Biliary Atresia and Hypoplasia 10% treatable with Kasai procedure

within 1m Chronic cholangitis, hepatic fibrosis,

liver tx Choledocal Cysts

>50% develop symptoms after 10y of age Increased risk for cholangiocarcinoma

Congenital Biliary Ectasia Caroli’s disease Congenital hepatic fibrosis

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Treatment is surgical by Complete Resection and biliary-enteric anastomosis

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Choledocolithiasis

Asymptomatic, found in about 10 % of patients undergoing Cholecystectomy

Associated with gallstones in general

Can cause obstructive jaundice, cholangitis, or pancreatitis

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Endoscopic view of sphincterotomy and basket extraction

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Choledocolithiasis Complications

Cholangitis Acute cholangitis (suppurative / non

suppurative) Chronic cholangitis, hepatic abcesses

Obstructive Jaundice In association of cholecystitis

Pancreatitis Secondary Biliary Cirrhosis

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Acute Cholangitis

- Infection of bile ducts by obstruction (choledocal stones, benign stricture, sclerosing cholangitis and tumors of CBD or periampullary neoplasm)

- Enteric flora most common (E coli)- Charcot’s triad of right upper quadrant

pain, fever and jaundice (70% of patients)

- Relapse if cause not treated, life threatening with generalized sepsis, MOF. Chronic cholangitis may lead to secondary biliary liver cirrhosis

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Cholangiopancreatography in acute cholangitis

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Indications for Cholecystectomy

Asymptomatic gallstones?18% cumulative risk at 15 y for development of symptoms or complications in male silent gallstones patients.-cumulative risk of death on expectant management = small-prophylactic cholecystectomynot warrantedexcept in young age, diabetics,large stones, anomalies

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Choledocolithiasisdiagnosed during Cholecystectomy

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Postcholecystectomy complications

Overlooked non-biliary disorder Biliary strictures Retained biliary calculi Cystic duct stump syndrome (?) “Papillary dysfunction, papillary

stenosis, sphincter of Oddi spasm, biliary dyskinesia”

Bile salt – induced diarrhea (5%, cholestyramine)

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Operative Complications of Laparoscopic Cholecystectomy

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Retained common bile duct stone after Cholecystectomy

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Gallbladder adenocarcinoma: risk factorsIsrael & Chile: highest incidence worldwideChronic Gallstones: 70-90% presence

Estrogens / Female sex / Obesity

75% older than 65y

Choledocal cyst

Salmonella typhi carriers

Porcelain gallbladder (neglected cholecystitis)

Carcinogens

Gallbladder polyps

Smoking, Alcohol consumption

PSC

Repetitive epithelial repair (dysplasia to invasive carcinoma, ~15y)

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Gallbladder AdenoCarcinoma: Spread & Staging

TNM* Tumor Location

Tis Carcinoma in situ

T1a GB wall: mucosa

T1b GB wall: muscle

T2 Perimuscular tissue

T3 Serosa,organ, liver <2 cm

T4 >2 organs, liver >2 cm

N1a Hepatoduodenal nodes

N1b Other regional nodes

M0 No distant metastases

M1 Distant metastases

Early lymphatic spread: hepatoduodenal, retroperitoneal, celiac. Direct invasion of the liver, extrahepatic biliary ducts. Intraperitoneal seeding.

Stage 0 Tis N0 M0

Stage I T1 N0 M0

Stage II T2 N0 M0

Stage IIIT1-2T3

N1N0-1

M0M0

Stage IVA T4 N0-1 M0

Stage IVBT1-4T1-4

N2N0-2

M0M1

44intraluminal mass enhancing heterogeneously after IV contrast

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Porcelain gallbladder: 60y old woman

carcinoma in 25% of patients with "porcelain" GB

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Gallbladder adenocarcinoma: Presentation

Fifth most common GI malignancy Incidental finding in lap. excised

gallbladders Patients in early stages show signs and

symptoms that mimic cholelithiasis and/or cholecystitis.

Patients in later stages present with weight loss, hepatomegaly, and jaundice, which are considered poor prognostic signs.

Duodenal or colonic obstruction or cholecystenteric fistula may signal GB carcinoma

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Gallbladder adenocarcinoma: Presentation

Typically unresectable at presentation Prognostic is poor with the exception of

early-stage cases. Overall mean survival rate of 6 months, and the 5-year survival rate is 5%

In locally advanced gallbladder cancer, radical surgery with negative tumor margins is an accepted treatment; if negative margins were seen, patients achieved a 5-year survival rate Stage I, 95% Stage II, 75% Stage III, 25% Stage IV, 15% Stage V, 2%

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Chronic Cholecystitis Cholelithiasis Small Cystic Duct Papillary Tumor

Prevention of gallbladder carcinoma by cholecystectomy?

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Cholangiocarcinoma: risk factors

Primary sclerosing cholangitis: 30% of CC in patients with PSC and UC (lifetime risk 10-15%).

· Choledochal cysts: related to duration of disease. 15% per year after first two decades.

· Parasitic infections: Liver flukes (Clonorchis and Opisthorchis)

· Hepatolithiasis :Asian patients with gallstones.

· Toxin exposures: auto, rubber, chemical, wood-finishing occupations

· Genetics: “cancer family" syndromes (eg, Lynch, Li-Fraumeni), Caroli’s syndrome (congenital dilation of intrahepatic ducts), multiple biliary papillomatosis

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Cholangiocarcinoma: presentation2000-3000 US cases per year. 3% of GI

malignancies.

Symptoms: pruritus 60%, abdominal pain 30-50%, weight loss 30-50%, fever 20%, fatigue, clay stools , dark urine.

Signs: jaundice (often intermittent) 90%, hepatomegaly 25-40%

Lab test: increased bilirubin, increased alkaline phosphatase.

CEA elevated in some but not sensitive or specific.

CA 19-9 elevated in 80% and most helpful in pts with PSC.

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Cholangiocarcinoma: imaging

Ultrasound: segmental dilatation, nonunion of R and L ducts, polypoid intraluminal masses, nodular smooth masses with mural thickening.

Doppler: assess vascular invasion

CT: contracted gallbladder in Klatskin tumor, Courvoisier in CBD tumor.

Cholangiography (ERCP or PTC) with cytology and biopsy.

MRCP

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Perihilar cholangiocarcinoma: Klatskin

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Cholangiocarcinoma: treatment

Surveillance of congenital disease and PSC!

Prognosis: 5 year survival without surgery 5-10%. With surgery 10-30% or higher.

Surgery is best option in resectable tumor

Adjuvant therapy: post-operative radiation may provide benefit in incomplete resections.

Chemotherapy combined with radiation therapy may improve survival time by up to 10 months in pts with unresectable tumors.

Palliative therapy: Stenting of biliary tree

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Intra-pancreatic cholangiocarcinoma

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